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Fourth Edition

Prof. MRKhan
MBBS, DTM&H, DCH, FCPS, FRCP
National Professor
Director 6 Professor (Honorary) of Child Heahh
Institute of Child Heahh & Shishu Shasthya Foundation Hospital, Dhaka
Wsiting Professor, International Cenne for Diarrheal Disease
Res earc h, B angladzs h Q CD D R, B)

Prof. M Ekhlasur Rahman

MBBS, FCPS, FRCP EdiN
Professor and Head
D ep artrnent of Pediatrics
Dhaha Medical College y'r Hospital, Dhaha

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ELSEVIER
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, Reed Elsevier India Private Limited
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.11

Essence of Pediatrics, 4/e

Khan and Rahman

ELSEVIER
A division of
Reed Elsevier India Private Limited

Mo sby, Saunders, Churchill Liv ingstone, Butterworth-Heinemann and

Hanley 6 Belfus are the Health Science imprints of Elsevier,

@ 201 I Elsevier

All rights are reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in
any form or by any means, electronic, mechanical, photocopying, recording, or otherwise without the prior permission
of the publisher.

ISBN: 978-81-312-2804-3 (Indian Edition)

ISBN: 978-8 1 -3 12 -29 40 -B (Bangladesh Edition)

Medical knowledge is constantly changing. As new information becomes available, changes in treatment, procedures,
equipment and the use ofdrugs become necessary. The authors, editors, contributors and the publisher have, as far as
it is possible, taken care to ensure that the information given in this text is accurate and up-to-date. Howeveg readers
are strongly advised to confirm that the information, especially with regard to drug dosei usage, complies with current
legislation and standards of practice. Please consult fuII prescribing information before issuing prescriptions for any
product mentioned in this publication.

Published by Elsevier, a division of Reed Elsevier India Private Limited.

Registered Ofice:622,Indraprakash Building,2l Barakhamba Road, New Delhi-ll0 001.
Corporate Ofice: l4rh Floor, Building No. 108, DLF Cyber City, Phase II, Gurgaon-l22 002,Haryana, India.

Managing Editor (Developmen f): Shabina Nasim

Development Edlfor: Shravan Kumar
Manager Publishing Operations: Sunil Kumar
Pro ductio n Executiy e: Arvind Booni

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Tlpeset by Chitra Computers, New Delhi.

Printed and bound at RakmoPress.N.Delhi.

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Jaira Khanam
I Ameena Khatoon
For the continued. ffiction (r inspiration they haue flowing non-stop

in the snedm of their blood for their children

Dedicated to our wiues:
Anwara Khan
Reltana Rahman
For their inherent inquisitiueness and intense enthusiasm that had
made the deliuery of this book less painful
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 Contributors

Prof. MN Islam Prof. M QKTirlukder

MBBS, FCPS, FRCP MBBS, DIP NUTR, DCH, PhD, FRCP
Professor and Head, Pediatrics Chairman, Centre for \7omen & Child Health, Dhaka
Ganoshasthya Samajvittic Medical College, Dhaka
Prof. MAMannan
Professor Nazmun Nahar MBBS, FCPS, M Phil, FRCP
MBBS, FCPS^, FRCP Ex. Pro-VC & Professor of Pediatric Hematology &
Professor of Pediatrics Oncology
Additional Director General, BIRDEM, Dhaka Bangabandhu Sheikh Mujib Medical Universiry Dhaka

Prof. Md Moazz,em Hossain Prof. Shafiqul Hoque

MBBS, FCPS, FRCP MBBS, FCPS
Professor of Pediatric Nephrology & Chairman, Department of Pediatric Surgery
Tieasurer, Bangabandhu Sheikh Mujib Medical Universiry, Bangabandhu Sheikh Mujib Medical Universiry Dhaka
Dhaka
Prof. MdAbul Kashem Sarkar
Prof. AFM Selim MBBS, DCH, MRCP
FCPS, PhD Professor of Pediatrics
Professor of Pediatrics Bangladesh Institute of Child Health (BICH), Dhaka
Deputy Director, Institute of Child Health & SSF Hospital,
Dhaka Prof. ARM Luthfrrl Kabir
MBBS, FCPS
Dr. ANAlam Professor of Pediatrics
MBBS, PhD Sir Salimullah Medical College & Mitford Hospital,
Consultant Physician Dhaka
ICDDR,B, Dhaka
Dr. Md Mesbah Uddin Ahmed
Prof. Md Nazrul Islam MBBS, D Ortho, MS
MBBS, FCPS Consultant, Orthopedic Surgery
Professor of Pediatrics Square Hospital, Dhaka
Community Based Medical College & Hospital, Mymensingh
Prof. Soofia Khatoon
Prof. ASM Bazlul Karim MBBS, FCPS
MBBS, FCPS ProFessor & Head, Pediatrics
Professor of Pediatric Gastroenterology & Nutrition Shahid Suhrawardy Medical College & Hospital,
Bangabandhu Sheikh Mujib Medical Universiry Dhaka Dhaka

Prof. Md Habibur Rahman Dr. Sajal Kumar Mazumder

MBBS, FCPS, MD MBBS, MS
Professor of Pediatric Nephrology Assistant Professor of Pediatric Surgery
Bangabandhu Sheikh Mujib Medical University, Dhaka Shahid Suhrawardy Medical College & Hospital, Dhaka
 I

ESSENCE OF PEDIATRICS

Dr. MARouf Dr. Rehana Perveen

MBBS, MD, FCPS MBBS, FCPS
Assistant Professor of Neonatology Associate Professor of Obsterics & Gynaecology
Sylhet MAG Osmani Medical College & Hospital, Sylhet Shahid Suhrawardy Medical College & Hospital,
Dhaka
Prof. MdAbid Hossain Mollah
MBBS, FCPS, FRCB M Med, FCCP
Dr. Ahmed Murtaza Choudhury
Professor of Pediatrics
MBBS, FCPS
Dhaka Medical College & Hospital, Dhaka
Associate Professor of Pediatrics
Mymensingh Medical College & Hospital,
Prof. Afiqul Islam
Mymensingh
MBBS, FCPS, MD
Chairman, Pediatric Hematology and Oncology
Bangabandhu Sheikh Mujib Medical Universiry Dhaka Dr. ShakilAhmed
Assistant Professor of Pediatrics
Dr. Narayan Chandra Saha Shahid Suhrawardy Medical College & Hospital,
MBBS, FCPS Dhaka
Associate Professor of Pediatrics
Dhaka Medical College & Hospital, Dhaka Dr. Monirul Islam Khan Ranzu
MBBS, MD
Dr. Selina Husna Banu Consultant, Pediatrics
MBBS, DCH, PhD Mymensingh Medical College,
Associate Professor of Neuroscience, Department of Pediatrics Mymensingh
Institute of Child Health and Shishu Shasthya Foundation
Hospital, Dhaka

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 Preface to the Fourth Edition

In United of infant & neonatal mortality was identified as two of ten important achievements
States of America, reduction
during the last cenrury. The ten countries of South-East Asia Region (SEAR) contribute to 40o/o of neonatal deaths and 40%
of deaths due to tuberculosis; 30o/o of U5 child deaths occur in SE'AR'
Although globally U5 mortaliry has decreased by almost a third since 1970s, this reduction has not been evenly distributed
\Worid Health reporr, about 10.5 million children die before they reach their 5th
throughou'r ,i. -orid. According to the 2005
birthJay-50%o of them die of malnutrition, 20o/o of pneumonia, 12o/o of diarrhea, and22o/o of perinatal causes.
'S(.
really amazed by the overwhelming acceptance of "Essence of Pediatrics", Jhird Edition by the under- and post-graduate
"r. pediatricians, and teachers in countries of South-East Asia region. \7e are very much thankful and grateful to them.
students,
This edition contains detailed and updated coverage of all the systems. There is also addition of a new chapter named
"Drug
Therapy in Children'. have been thoroughly revised, corrected, and expanded.
All the topics
Ve ir"tefully acknowledge the publications and books from where information has been taken; reference lists have been cited
at th. J.rd of each chapter, but if some have been left out through oversight, we offer our sincere apologies.
\fe hope that the fourth edition of "Essence of Pediatrics" will continue to stimulate the under- and post-graduate medical
srudenrs, do",o.r, and pediatricians of Bangladesh, India, Nepal, Pakistan & other countries, and will prove to be as useful to
readers as the third edition had been.
Our heartiest thanks are due to Dr. Abu Sadat Mohammad Saleh, Dr. UKM Nazmun Ara, and Dr. Nazmul Huq of Department
of pediatrics, Dhaka Medical College & Hospital for their untiring efforts in preparation of manuscript and proof reading; they
worked smilingly to complete this Himalay"., t"rk early. \7e are also thankful to Dr. Mohammad Mohsin, Dr. Abu Sayeed,
Dr. N{ehdi pervez and Dr. Monir Hossain of Department of Pediatrics, Dhaka Medical College & Hospital whose hard work
and co-op€radon have made delivery of this book less painful.
'W'e
ajcnowledge gratefully the contributions made by Dr. Shishir Ranjan Das, Assistant Professor of Neonatology, Dhaka
Medical College *abt lmnul Islam, Assistant Professor of Pediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka.
Any constructive criticism and structured suggestion will be thankfuliy acknowledged.
For the first time, Elsevier, a division of ReJ Ekevier India Pvt. Ltd. has taken the responsibiliry of editing, printing, and
publishing the fourth edition of "Essence of Pediatrics"; we express our heartiest thanks and felicitation to all the staff members
of Elsevier.

Dhaka MR Khan
M Ekhlasur Rahman
luly,2011
 Preface to the First Edition

It has long been felt that a book dealing with practical pediatric problems is urgently needed; in this small volume' we have
discussed Lriefly the recent management of commoner childhood diseases consulting the available current literatures. Protein-
energy -"l.r.rtiitio.r, acure respiratory infections and diarrheal diseases are written a bit elaborately, since these are the major
killei diseases of children here. One chapter, in this book, is devoted for practical procedures and another one for pediatric dose
calculation; so it is presumed that this volume will be very much helpful in pediatric practice. \7e hope this book will meet up
some of the demands of under- and post-graduate medical students, general practitioners, and pediatricians.
References have been cited at the end of each chapter, but if some have been left out through oversight, we offer our sincere
apologies.
^
wJ deeply thankful to prof. Maleka Khatoon, Prof. M QK Thlukder, and Dr. CA Kawser of Department of Pediatrics,
"..
IpGM&R; p.of. ivta Nurul Islam and Dr. Shafiur Rahman of Sir Salimullah Medical College; Prof. Nazmun Nahar, Dr' Munimul
Hoque, and Dr. euazi Monzurul Moula of Dhaka Medical College; Prof. FH Nazir and Dr. ABM Siddique of Rajshahi Medical
Colieg.; Dr. MA n* of Sylhet Medical College, Dr. SA Hamid of Mymensingh Medical College, Prof. Shahadat Hussein and
Dr. ihowdhury B Mahmood of Chittagong Medical College; Dr. Hamidur Rahman of Rangpur Medical College,
Dr. BD Shaha of Sher-e-Bangla Medical College and Prof. MS Akbar, Dr. AFM Salim of Dhaka Shishu Hospital for their
chronic encouragement and inspiration in writing a book on pediatrics.
Our sincere appreciations and thanks are due to Dr. Bazlul Karim, Dr. Azizur Rouf, Dr. ARM Luthful Kabir, Dr. Afiqul
Islam, Dr. Shahnewaz-bin-Thbib, Dr. Manajjir Ali, Dr. Md Ruhul Amin, and Dr. Mohammad Shahidullah for their spontane-
ous help in writing this book.
W'. o*. gr."t-d."1 to Mr. Motior Rahman Khan (Mod) for his frequent persuation in timely completion of this book' and
" 'W'e
wish to thank to the staffs of Messrs BRAC Printers whose hard work and
to Mr. Hawladar for his secretarial assisrance.
co-operation had made this endeavor possible.
Any suggestion or criticism for updating this volume will be thankfully acknowledged.

Dhaka MR Khan
M Ekhlasur Rahman
]anuary 1989

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 I

Contents

Cuaprnn I History-Taking and Physical Examination ............ """"""' I

CHeprBn 2 'Neonatology.......... """'7
Cnaprnn 3 Infant and Young Child Feeding..... """""""'49
CHeprnn 4 Growth and Development....'.' ""'56
Cneprnn 5 Nutritional Problems """"""""""72
CneprnR 6 Community Pediatrics """""""""85
CnaprBn 7 Respiratory Diseases """""""""'104
Crrlprnn 8 Diseases of Gastrointestinal System """""""130
Cnnprnn g Diseases
Cardiovascular """""""152
Cneprnn 10 Renal Diseases """""195
Cneprnn 11 Fluid Electrolytes and Acid-Base Disturbances......... .....2L2
Cneprnn 12 Endocrine and Metabolic Diseases """""""'219
Cneprnn L3 Neurology. """"""""252
Cneprun 14 Muscle Disorders """286
CHaprsn 15 Hematology """""""291
Cnlprnn 16 Oncology.. """"""""303
Cneprnn 17 Connective Tissue Diseases..... ""317
Cneprnn 18 Child Psychiatry and Developmental Disorders .........'."330
Cneprrn 19 Dermatology """"""'347
Crreprnn 20 Poisoning.. """"""""357
e';renrnn 21 Immunization and Infectious Diseases """"'370
CHeprsn 22 Genetic Disorders ""'423
Cnaprnn 23 Common Surgical Problems """'444
Cnaprnn 24 Procedures in Practice. """"""""455
Csaprnn 25 Some Selected Syndromes ......'.'.. """"""""'465
Cnaprnn 26 Laboratory Medicine """""""""469
Cs.xprnn 27 Drug Therapy in Children ......'..... """""""'473
Cn,c"prBn 28 Growth Charts....... "'508
 CHAPTER 1

History-Taking and Physical Examination

Chopler Conlenls
The hist0ry..........,.., ........,,...,,..,,,..,.........,..1 Cardiovascular system ..................... ..........................................2
Ceneral examinaii0n.,..,,...........................................,...,,...,...,,.. I The abdomen ........ ,........................,,........3

Respiratory system .................,...,,.............................................. I Locomotor system...................................,,..,,....................,........4

GENERAT EXAMINATION
Particulars of the patient
r Name c Address (Mobile No) Greet the patient with a socially or regionally appropriate
r Age (date of birth) r Source of informarion greeting (like handshake or salam).
> Sex
o Appearance (ill-looking, o Blood pressure
Presenting complaints: The problem that made the patient pufi', wizened, mongoloid, o Temperature
seek medical help. Chronology of the complaints should be grotesque) . Skin condition
maintained-define the compiaint with duration. o risus . Back of the body
Facies (coarse, moon,
: History ofpresent illness: Story of the disease plus drug sardonicus) . Anthropometry
history (for the present illness) and systemic enquiries o Grades ol xerophthalmia . \Weight for age (WAZ),
(review of systems). Use patient's language, nor even o Pallor \X/eight for height
technically correct terms. o Jaundice (wTrz)
t History of past illness: Diarrhea, pneumonia, measles, o Cyanosis . Height
pertussis, convulsion, tuberculosis, etc. o BCG scars . OFC (occipirofrontal cir-
: Birth bistory: Antenatal, natal, postnatal, neonatal. o Dehydration cumference)
> Feeding (dietetic) history: Exclusive breast feeding, r Edema . MUAC (mid upper arm
cowt milk, formula feeding, complementary feeding. o Clubbing circumference)
> Deuelopmental history: Social smile, neck conrrol, r Koilonvchia . Urine for aibumin in
sitting, standing, walking, speech. o Lvmph nodes MCNS and reducing
) Immunizatian history: BCG, Polio, Penta, Measles, others. o Fontanel substance in DM
) Treatment history: Drugs taken for past illness, drug o Pulse/hean rate . Examination of ear, nose
reaction or allergy. o Respiration rate and throat
Family bistory.. Consanguiniry pedigree chart should be
drawn; familial illness, maternal illness during pregnan-
cy, history of abortions and still births, health of other RESPIRATORY SYSTEM
sibs etc. should be noted.
> Socio-economic bistory.. Occupation of parents, hous- Generol Assessment (Look, Lislen & Feel)
ing, safe water supply, sanitation.
> Personal history: History of aliergic rhinitis, eczema erc.
o Cough o Stridor
in a case of asthma.
r Dvspnea o Grunting
r V4'reeze o Cyanosis
At the end, a summary should be made; it should be put to r Hemoprysis o Clubbing
the patient for correction, confirmation. o Sputum (t8 y.)
 I

ESSENCE OF PEDIATRICS

Respirolory Syslem Proper Auscultate in two stages: compare first the breath sounds,
added sounds, and then the vocal resonance
lnspection
Auscultation is carried out to determine:
o Form of the chest
e Respiratory rate
o Character of breath sounds (vesicular, bronchiai)
r Chest indrawing
r Vocal resonance (by asking one, one, one)
o Movement
o Added sounds (crepitations, rhonchi, pleural rub)
o Chest deforrnity (pectut carinatum, pectus excavatum,
Harrison sulcus, pigeon chest, rachitic rosary)
c CARDIOVASCUTAR SYSTEM
Expansion of the chest
r Cardiac impulse
Generol Assessmenl
o Dyspnea (exertional, orthopnea)
Palpation o Cyanosis
o Movement of the chest o Apex beat o Ascites
o Tiachea o Vocal fremitus o Clubbing
n Chest expansion o Enlarged tender liver
o Edema
o Respiratory sign-crepitations
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Percussion
r Practice percussion only nvice at each ofthe sites suggested Cordiovosculor Syslem Proper
for auscultation alternating right and left.
o Pulselheart rate (rate, rhythm, character, volume, radio-
The positions in which the percussion note in the rwo
femoral delay)
sides should be compared are as follows:
o Blood pressrue: For measurement of blood pressure, normal
c Anterior chest wall: (z) clavicle, (ii) infraclavicular re- auscultatory method is carried out in children over 3 years of
gion, (iii) znd to 6th intercostals spaces age with the manometer at the level of the heart. The palpa-
o Lateral chest wall: 4rn rc 7th intercostal spaces tory method is employed in babies and younger children;
o Posterior chest wall: (l) trapezius, percussing down- where none of these methods is possible, flush method may
wards over the lung apex; (zl) above the level of spine of be used. Doppler method may also be applied.
scapula; (iii) at intewals of 4-5 cm from below the levei o It is rarely practicable in infants
Jugular venous pressure:
of spine of scapula, down to the ll'h rib. because of shortness of neck.
o Determine the upper border of liver dullness.
r Resonance increases in bronchiolitis, asthma, emphysema, The Precordium
and pneumothorax.
o Resonance decreases (e.g., percussion note is dull) in con- lnspection
solidation, collapse, pleural thickening and effusion. \X/ith the patient sitting at 45" to the upright with the shoul-
ders horizontal.
o Precordial bulge
Auscultation
o Apical impulse
Listen for one or two breaths at each of the six sites in the o Other pulsations (Suprasternal/epigastric)
following order: o Veins on the chest wall
o Anteriorly: Below right clavicle, below left clavicle,
Palpation
medial to right nipple, medial to left nipple, right axilla,
left axilla. o Apex beat
o Posteriody: In the supraspinous fossa and over the lower o Other pulsations
ribs (child remains seated with both hands in front). o Left parasternal lift (position, character)
o Thrill
Can also auscultate in positions described above under
o Palpable P,
percussion-auscultate anteriorly from above the clavicle
down to the 6'h rib, laterally from the axilla to the 8'h rib,
Percussion
and posteriorly down to the level of the 11'h rib. Avoid
auscultation within 2-3 cm of the midline. Auscultate the Percussion ofthe heart is now seldom carried out, and has been
rwo sides alternately. superseded by the chest x-ray and echocardiography. 1
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 HISTORY-TAKING AND PHYSICAL EXAMINATION

Auscultation The Abdomen ond Genilqlio

Auscultate all over the precordium, listening in turn the base lnspection
ofthe heart, auscultatory areas, over carotids, axilla and back,
when needed. Roll the patient on the left side and listen at the
r Shape
apex using the bell to detect murmur of MS. Sit the patient
o Visible peristalsis
up, leaning forward and listen over the right 2"d interspace
o Movement of the abdominal wall
o Abdominal distension
and then down the left sternal edge with the diaphragm for
murmur of AR.
o Umbilicus (everted, omphalitis, granuloma, hernia, polyp)
l-
i o Superficial veins (over costal margin, caput medusae, in
l- Auscultatory areas: Auscultation starts with palpation of obstruction of inferior vena cava)
t- apex beat. o Groin (impulse on coughing)
l o Genitalia
I o Mitral area (which corresponds to the apex beat, area is r Anus
I not fixed)
, o Tiicuspid area (which lies just to the left of the lower end Palpation
v
of the sternum)
t r Aortic area (which is to the right of the sternum in the 1. Start palpation gendy from the 1eft iliac region and proceed
anti-clockwise to end in the suprapubic region.
second iCS)
o 2. Feel for left kidney.
Pulmonary area (which is to the left of the sternum in the
J. Feel for spleen (enlarges commonly towards the left iliac
second ICS)
fossa in infancy, towards the right iiiac fossa in older age).
o Other areas (e.g., infraclavicular region, neck, left axilla,
interscapular area, left sternal edge, etc.).
4. Feel for liver, gallbladder.
5. Feel for right kidney (right is lower than left).
Heart sounds and added sounds: Qualify first and second 6. Feel for urinary bladder.
heart sounds, murmut pericardial rub, low frequency sounds 7. lleel for aorta and para-aortic glands and common femoral
in diastole (third or fourth sounds), gallop rhphm, ejection ressels.
systolic clicks. 8. Ila mass is palpable, elicit its features (size, shape, surface,
mobiliry bimanual palpabiliry pulsation).
edge, consistenry,
Murmurs: Timing (systolic or diastolic), relation with c Palpate both groins (expansile impulse on coughing).
respiration, with position of the patient, point of maximum 10. Examine the external genitalia.
intensity, direction of propagation, character, grading (for
o Male: penis, scrotum, testes.
grading of murmur refer Table 1 . I ).
o Female: vagina (imperforate, discharge), ambiguous
genitalia, pubertal changes.
Table 1.1 Crading of a Murmur 11. Per-rectal examination (imperforate anus, anal stenosis,
ffiffi Heard by an expert in optimum condition
rectal pol1p, Hirschsprung disease, anal fissure, patulous
ffi&ffiffi
ffiffi anus).
m *rtldiw Heard by d non-experl in optimum condition
ffi
12. Ascertain direction of flow of the distended veins.
ffi Easily heard, no thrill
ffi A loud murmur with a thrill Remember
@ffigE65$
ffiffi Very loud murmur with a thrill
o In infants, examination may be aided by having the child
ceai{diE&sw
ffi suckling at the breast.
Extremely loud, can be heard without stethoscope
E*d#w*m r In hypertrophic pyloric stenosis, visible gastric peristalsis
Note can'be observed during a test feed, the appearance having
o Close splitting of the second sound in the pulmonary area is common in normal
ch i ldren. been linked to an olive moving under the skin from left
. A venous hum is a continuous murmur heard at the root of the neck and abolishes hypochondrium downwards and to the right. The pyloric
by pressure over the internal jugular vein or placing the child in the head down
position. tumor mass is felt as a smal1, hard tumor at the right of
the midline just belorn' the costal margin with the left
hand from the left side of the patient during the course
THE ABDOMEN of a test feed.
o Testes should be palpated with the child in squatting posi-
Generol Assessmenl tion to differentiate retractile testes from cryptorchidism.
o Pallor a Fetor hepaticus
r \(asting a Jaundice
Percussion
o Koiionychia a Clubbing o Shifting dullness: To elicit shifting dullness, lie the patient
o Stomatitis, gingivitis supine and percuss laterally from the midline keeping your
ESSENCE OF PEDIATRICS

fingers in the longitudinal axis, until dullness is detected o Joints (swelling, redness, deformiry)
(in normal individuals dullness is detected over the lateral r Gower sign
abdominal musculature). Then, keeping your hand on the r Gait
abdomen, ask the patient to roll away from you on the
opposite side. Percuss again on this new position; if the Palpation
previously found dull note has now become resonant then
o Bulk
ascitic fuid is probably present. To confirm its presence,
repeat the maneuver in the opposite side of the abdomen.
r Tone
o o Strength: The quickest and most reliable method of making
Fluid thrill: The padent is laid on his back. Place one hand
a quick assessment of strength of muscles is to watch the
flat over the lumbar region of one side, get an assistant to
patient sitting up from lying position, standing up, walking,
put side of his hand in the midline of the abdomen and
jumping, hopping, dressing, or undressing.
then tap the opposite lumbar region. A fuid thrill is felt.
o Joint (temperature, tenderness, crepitus, efrrsion)
Assistant's hand is used to dampen any thrill that may be
transmitted through the fat of the abdominal wall. It is felt o Range of movement:
when a large amount of ascites under tension is present' Active movement should always be attempted before
- passive movement.
The above two signs are present in half of the cases of
It is impossible to memorize the normal range of
ascites. - movement at every joint, but much can be learnt by
o Upper border of the liver for dullness. comparing left and right limbs or comparing with
that of examiner himself.
a Schober test
Auscultation
a Subcutaneous nodule
o Bowel sounds (stethoscope placed just to the right of the a Eyes (iridocyclitis, uveitis, conjunctivitis)
umbilicus): to decide whether they are normal, increased, a Lymphadenopathy
or absent. a Hepatosplenomegaly
o Bruits in the aorta and main abdominal vessels.
a Muscle power (MRC scale)
o Grade 0 : Complete paralysis
TOCOMOTOR SYSTEM o Grade 1 : Flicker of contraction only
o Grade 2 : Power detectable with graviry eliminated
Generol Assessmenl o Grade 3 : Ihe limb can be held against gravity, not
against examiner's resistance
o Tenderness (bone, .ioints, muscles, tendons) o Grade 4 : There is some degree of weakness, usually
o Impaired movement (limited by pain and stiffness) described as poor, fair, or moderate strength
o Swelling o Grade 5 : Normal power
o Deformity
Joint tenderness
o Grade 1 : Patient says that joint is painful
Locomolor Syslem Proper o Grade 2 : Patient winces on palpation
lnspection o Grade 3 : Patient winces and withdraws on palpation
o Grade 4 : Patientwill not allow the joint to be touched
o Postufe
Examination of the hips in the newborn: This should be
o Muscle wasting
o Chest deformity the last part of assessment, as it is very uncomfortable
r Pseudohypertrophy for the baby. Skin creases on the upper Posterior thigh
r Rash may be asymmetrical. Limitation of abduction may also
o Skull deformity be noted.
o Jaw (micrognathia) o Stage 1 examination: The examiner grasps the thighs at
o Spine deformity (\yphosis, scoliosis, gibbus, spina bifida. the medial aspect of the thigh, and puts the middle fin-
gers over the greater trochanters. The hips are flexed, me-
.
Scoliosis is most evident when the child bends forward to
touch his toes unclothed, and the examiner observing him dially rotated, and pushed posteriorly. This will dislocate
from behind). dislocatable hips. It is associated with a pronounced and i
r Limb deformity (wide carrying angle, enlargement of wrists, palpable snap. a
rhizomelic, mesomelic shortening of limbs, polydactyly, o Stage 2 examination: The hips are abducted apply- I
dinner fork deformiry coxa vara, coxa valga, genu recurva- ing pressure over the greater trochanters. A snap felt in 1
tum, talipes, pes cavus). abduction (Ortolani sign) is suggestive of dislocation.
Ii
{
 HISTORY-TAKING AND PHYSICAL EXAMINATION

NERVOUS SYSTEM o Smooth forehead

o Inabiliry to close the eyelids and tear flows
Menlol Funclions o Loss of nasolabial folds
o Angle of the mouth sags and saliva dribbles
o Appearance and behavior o Clouding of consciousness o Lesion in part I (pons): LNFP + concomitant VI
i-
l"

t-
v
o Emotional state
r Delusion and hallucination
o Orientation in place and
o Memory
r Intelligence
o Speech (dysanhria, aphasia)
nerve involvement. Lesion in part 2 (cerebello-
pontine angle): LNFP + VIII nerve involvement.
Lesion in part 3 (facial canal): LNFP + herpetic
|il
I time r Apraxia eruption of the pinna of ear (geniculate herpes);
I LNFP + hyperacusis (nerve to the stapedius affect-
I Croniql Nerves ed); LNFP + loss of taste of anterior third of tongue
(chorda rympani affected). Lesion in part 4 (ster-
t 1. Olfactory: Identification of odors, disorders of smell
t
nomastoid foramen or beyond): LNFP without
I (anosmia, parosmia).
2. Optic Vsual acuiry visual fields, color vision, fundoscopy, collateral signs.
I), pupillary refexes (direct light reflex, consensual light refex, 8. Vestibulocochlear:
I accommodation refex). A. Cochlear:
t 3. Oculomotor: Ocular movements, strabismus, nystagmus,
v i) Hearing (use of human voice, tuning fork tests-
I pupil (size, shape, pupillary reflexes).
Rinne test, \feber test, audiometric tests).
r 4. Tlochlear: Ocular movement.
ii) Abnormal auditory sensations (tinnitus, hyper-
t 5. Trigeminal:
acusis).
A. Motor: Masseter and temporal muscle prominence
B. Vestibular: Caloric test
on clenching.
B. Sensory: Sensations of face-by testing ophthalmic, 9. Glossopharyngeal: IX and X are considered together.
maxillary and mandibular branches. A. Motor: Palatal reflex.
C. Reflexes: Corneal reflex (afferent - V,, efferent facial B. Sensory: Posterior third of the tongue and the mucous
nerve), jaw jerk (afferent & efferent pathways are by membrane of the pharynx.
V nerve). C. Thste: Posterior third of the tongue.

6. Abducent: Ocular movement. The oculomotor, 10. Vagus:

trochlear, and abducens nerves are tested together for ocular A. {otor: Ability to swallow; speaking clearly, movement
movements. of the palate during phonation 'ali, palatal reflex.
7. FaciaL Observe for any facial asymmetry. B. Sensory: To respiratory passage.
A. Motor 1l. Accessory - motor: Strength of sternocleidomastoid
i) Raise eye-brows, wrinkle the forehead by looking muscle and trapezius.
upwards 12. Hypoglossal - motor: Protrusion of tongue, strength of
ii) Close the eyes, check the strength of eyes; check tongue, deviation, wasting, fasciculation, tremor.
strength of eye closure
iii) Puff out the cheeks Molor Funclions
iv) Vhistle o Bulk of muscles
v) Show the teeth (even these are false) o Tone of muscles
B. Sensory: Sense of taste (sweet, salt, sour, or bitter) of o Strength of muscles
the anterior two-thirds of tongue. r Reflexes:
C. Reflexes: Corneal, pout or snout, glabellar tap. o Superficial: Planter (L 5, S 1), cremasteric (L 1,2), anal
(T 7,8,9,10,11,12).
(S 3,4), abdominal
Consider paralysis of facial nerve in different parts of
its course:
o Deep: Knee jerk (L 3,4), ankle jerk (S 1,2), triceps jerk
(C 6,7), biceps jerk (C 5,6), supinator jerk (C 5,6)'
o Upper neuron facial paralysis (UNFP)
Grading of reflexes
o Only lower part of the face is affected Grade 0 : Absent
o Forehead wrinkling normal Grade 1 : Present (as a normal ankle jerk)
o Eye closure normal Grade 2: Brisk (as a normal knee jerk)
o Cheek is puffed out loosely with each expiration Grade3:Verybrisk
o Angle of the mouth droops Grade 4 : Clonus
o Lower neuron facial paralysis (LNFP) r Co-ordination of movement: In the upper limbs, in the
c Both upper and lower part of the face is affected lower limbs.
ESSENCE OF PEDIATRICS

Gait: Hemiplegic gait, ataxic gait (reeling, stamping), wad- Diagnostic Procedures
dling gait, high-stepping gait, festinating gait. No neurologi-
cal examination is complete, unless gait is observed.
Involuntary movements: Chorea, athetosis, tremor, tetany,

a
tics, dystonia, epilepsy, myoclonus.
Romberg sign
jI"ti":3gs':::J
a Cower sign *
PhysicalExamination $

Sensory Funclions Y
o Thctile sensibiliry (light touch, pressure, tactile localization, I3y::9i3T3"ete:f J
discrimination)
o Position sense *
r Recognition of the size, shape, form, weight, and texture
of objects
lly:llq{irry l
r Vibration t
o Pain ClinicalDiag'nosis
*ff
o Temperature
v
Treatment g

Signs of Meningeol lrritolion @E

o Nuchal rigidiry
*
o Kernig sign
Final Diagnosis fi

o Brudzinski sign
Salient features should be written with summary of symptoms
and signs (with positive and important negative findings).
Correct technical terms may be used.
Each progress note should contain the following four sections:
(i) Subjective data: Usualiy supplied by the patient or
i
1. Gill D, O'Brien N. Paediatric Clinical Examination l," ed. Singapore :
Churchill Livingstone, 1988.
2. Silver HK, Kemple CH, et al. Handbook of Paediatriu i5'h ed.
Singapore: Appleton & Lange, 1987.
Stephenson T, W'allac EIIJ.. Clinical Paediatrics for Postgraduate
Examination 1" ed. Edinburgh: Churchill Livingstone, 1991.
4. Ogilvie C. Chamberlain's Symptoms and Signs in Clinical Medicine
10'h ed. London: ELBS{ohn \Tright & Son Ltd., 1980.

Parents.
Gupta P Clinical Method in Pediatrics. l" ed. New Delhi: CBC
(ii) Objective data: Findings available on physical Publisher, 2009.
6. Hutchison JH. Practical Paediarric Problem.s 6'h ed. Singapore: PG
examination.
Publishing Pte Ltd, 1986.
(iii) fusessment: Assessment should be made considering each
7. Swash M, Mason S. Hutchisonls Clinica/ Methods 20'h ed. Eastbourne:
problem (i.e., diagnosis), ELBS, Bailliere Tindall, 1989.
(iv) Plan: Plan includes investigations, trearmenr, counseling, 8. Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of
and follow-up. Pediatrics 186 ed. Philadelphia: \X{B Saunders Co., 2008.

\
't
1
i
a:

CHAPTER 2
Neonatology
V

v-

v
I
v Chopter Conlenls
t Normal-term newborn infant..... ...........................................,.1
Essential newborn care.............................................................8
L
Care at birth...,....... .........,.........................8
V
Case record in newborn infant.......................,.......................8
V
Thermal protection in newb0rn.,............................................9
V
Fluid requirement in infancy and chi1dh00d....,............... l0
t
Minor developmental pecu1iarities....................................... 10
Iv Common congenital ma1f0rmati0ns..,................................. I I

Laryngomalacia........ ... ........................... ................................. I 2

r
Y
Tracheomalacia...... ................................. 13

Y Perinatal asphyxia. .....,...........................i3

I Low birth-weight neonates ................. ...................................11

!
L

I
?

deeply pigmented and has adequate rugae. The labia minora

The average weight of a normal newborn infant, born after
40 weeks of gestation is around 2800-3000 g. Th. length
and the head circumference (OFC) are approximately 50 cm
and 35 cm, respectively; the chest circumference is usually
3 cm less than OFC. The upper segment to lower segment
ratio is between 1.7 and 1.9 to 1.
The skull may show molding. The parietal bones may slightly
over-ride the occipital and the frontal bones. A contended
baby sleeps around 18 hours a day.
A full-term infant has got physiological photophobia, his
sclera appears slightly bluish. The ear cartilage is firm having
good elastic recoil.
The breast nodule is palpable, usually >5 mm in diameter.
Breast hypertrophy is common, milk may be present (should
not be expressed). Heart rate may vary from 120 to 160
per minute. There may be transitory murmurs. Congenital
heart disease may not initially produce murmurs that will be
present later; only a l:2 chance exists that a murmur heard
at birth represent congenital heart disease. The respiratory
rates stabilizes at about 40-<50 breaths/min.
In the abdomen, the liver is usually palpable, sometimes as
much as 2 cm below the rib margin. Less commonly' the
spleen tip may be felt. The approximate size and location of
each kidney can usually be determined on deep palpation.
Testes are usually in the scrotum, or palpable in the inguinal
canal, at least one testis is descended. The scrotum appears
are covered by the labia majora.
The anterior two-thirds or more of the soles show deep
creases. Peripheral cyanosis (acrocyanosis) may be present for
a short while after birth, especially when limbs are cool.
The skin is pink and is covered with vernix caseosa; this vernix
caseosa is not evident after 40 weeks ofgestation. Lanugo has
usually been lost, or replaced by vellus hair in term infant.
Muscle tone is increased by adult standard. Baby lies in an
attitude of flexion. Glabellar tap, tonic neck refex, rooting,
sucking and swallowing reflexes, grasp reflex, Babinski sign,
Moro reflex, etc. can be elicited in a normai-tetm neonate
when he is lying in a quite awake state.
About 95o/o of term and preterm neonates void within 24
hours. One can wait up to 48 hours for normal urination to
occur.99o/o ofterm neonates and95o/o ofpreterm neonates
will pass meconium within 48 hours of birth.
A healthy newborn has normal vital signs, as well as warm
and pink palms.
Hearing is weli-developed at birth, aiert more readily by a
high-pitch female voice than by a low-pitch male voice.
Smell is well-developed at birth, can recognize motherk
breast milk and odor of mother.
Newborn infants have more taste buds than adult and have
a definite taste preference for sweet.
Retina is well-developed at birth, prefers bright light. Fixa-
tion and tracking through at the visual field are well-devel-
oped by 2 months of age.
 .q

ESSENCE OF PEDIATRICS

o Observe the baby, resuscitate if needed.

a A clean and safe delivery: Clean cord cutting and tying.
a Prevention and management of hypothermia:
o Drying and wrapping
o Skin-to-skin contact
o Delayed bathing
Assessment of breathing status (management of birth
asphyxia):
o Thctile stimulation
o Mouth-to-mouth resuscitation, bag and musk resuscita-
tion.
Initiation of breast-feeding immediately after birth and
no later than t hour.
Appropriate umbilical cord care:
o Umbilical cord is the major entry point for infection.
o Before cord cutting, the caregiver should wash hands;
cord should be tied in at least three places before
cutting.
o The cord should be cut with a sterile scissor or surgical
blade.
o The cord should be left clean and dry after cutting.
o Nothing should be applied to the cord.
Eye care: Appropriate eye drop (chloramphenicol eye drop)
if the eyes are sticky and pus drains from eye.
Special care of the preterm and LB'W neonate:
o Two extra home visits
o Assisted feeding, if needed
Identification, man4gement, and referal for complica-
tions:
o A family member/relative/volunteer oriented on ENC
can take care of neonate. The orientation may take place
during antenatal visit by health workers.
o Drugs and injectables for neonates as per protocol will
be provided by: Community skilled binh attendant
(CSBA), family welfare assistant (F\fA), and health as-
sistant (HA).
CTEAN DETIVERY AND NEONATAT
KrT (CDNK)
Soap, plastic sheet, surgical/sterile blade for cord cutting, sterile
thread for cord tying, two pieces of gauze, two pieces of cloth
o Hold the baby at the same level of the mother, keep the
baby warm.
e Clamp the umbilica.l cord, and cur rhe cord within I minute
about 2 cm from the skin with a sterile blade.
o Put the baby on mother's bare abdomen within half an hour
after delivery; the baby will look for and find the nipple for
breast-feeding on demand,
o Bedding in: Keep the mother and the baby together
24 hours a day in the same bed.
a Give 2 mg of vitamin K, after breast-feeding is initiated.
a Not to bath the baby until the 2'd or 3'd day of life.
a Keep umbilical cord dry, clean, and bare.
a Check for passage of stool and urine.
a Immunize the baby with Hep-B, BCG, OPV-0.
A detailed physical examination of a neonate should be done
by the pediatrician (i) soon after birth, (ii) at 24 hours, and
(iii) l:efore the discharge from nursery. If the patient remains
cool and calm, the palpation of abdomen and auscultation
of heart and lungs should be done before other disturbing
manipulations.
o The History
o Particulars ofthe patienf.'Name, age, sex, address, name
of parents, date and time of admission, and examination
o Presenting conxplaints
o Historl of present illness
o Maternal history of illness
Antenatal: Amenorrhea, maternal age, blood group,
- para, gravida, abortion, weight and height of mother,
nutritional status of mother, LMB EDD, antenatal
period, fatal distress.
Natal: Labor (spontaneous or induced), duration (1"
- stage, 2'd stage), mode of delivery, place of delivery.
Postnatal: Cry, singleor twin, asphyxiated, resusci-
- tation (if any), Apgar score at lst minute and 5'h
minute.
o Feeding history
o Immunization bistory
o Family bistory
o Physical Fxamination :

for drying and wrapping the neonate. t Color, pallor, jaundice, actiaities
t Head: Hair line, microcephaly, cephalhematoma, sub-
aponeurotic hemorrhage, encephalocele.
t Anterior fontanel: Normally flat on sitting position.
Abnormally large fontanel presents cretinism, trisomy,
After a clean and safe delivery:
IUGR, rickets, hypophosphatasia, and osteogenesis im-
o Proper handwashing is a must before touching the baby. perfecta.
Dry and wrap the baby, using rwo pieces of pre-warmed c Face: Facial dysmorphism/asymmetry. 1
clothes. o Eyes: Ptosis, epicanthic fold, cararacr. 1
.l
tt
E

o Jaw: Micrognathia.
() Moutb: Cleft and cleft palate, thrush.
(_) Ear:Malformed.
o Nose.' Choanal atresia.
o Nech: Shon, webbed, sternomastoid tumor.
o Extremities: Upper (Erb palsy, Klumpke paralysis, syn-
dactyly); Iower (deformiry club foot).
c Cbest: Abnormality of nipple, mastitis neonatorum.
o Abdornen: Umbilicus, umbilical hernia, abdominal dis-
tension.
Hip: lnstablliry of the hip joint is detected by modified
Ortolani/Burlow maneuver.
o Orif.ce counting and their patency
o Bacb: Spine (meningocele, meningomyelocele, tuft of
hair, spina bifida, pilonidal sinus).
o Shin: Cyanosis, pallor, purpura, sclerema, birth mark,
erythema toxicum.
o Congenital or deaelopmental malformations: if any
o Antbropbmetr!: Birth weight, occipitofrontal head
circumference (it should be taken after the disappearance
of caput and over-riding of suture, normal 34-36 cm;
in preterm, small-for-date or hydrocephalous baby, head
circumference will be 3 cm bigger than normal), chest
circumference at the nipple, crown heel length, US:LS.
o Gestational age: From history of amenorrhea, NBS
system.
Vital Signs
o Temperature: Axlllary temperature (fever 37.5" C or
above; low body temperature <35.5' C).
o Pube: Normally 120 to <160 beats per minute.
o BP: Normally 60/40 mmHg.
o Breatbingrate:Normally 40 rc <60 breaths per minute.
Systemic Examination
o Neraous system: Physical acdvity, posture, level of con-
sciousness, cranial nerve palsy, tone, movement, knee
jerk, primitive reflexes (Moro, rooting, stepping).
o Cardioaascular system: Radial pulse, femoral pulse, apex
beat, heart rate, heart sounds (Sl, 52, added sounds).
o Respirator! slstem: Cyanosis, respiratory rate, chest in-
drawing, breath sounds and added sounds.
o Abdornen and genitalia.'Shape of abdomen, abdominal
girth, umbilicus, visible peristalsis, ascites, bowel sounds.
Liver (normally 2 cm enlarged). Spleen (easily palpable
is pathologic). Kidney (easily ballotable is pathologic).
Genitalia for hydrocele, penile length (norm ally 2.5 cm) ,
opening ofurethra (for epispadias and hypospadias), in-
guinal hernia, ambiguous genitalia.
o Salient Features
a Provisional Diagnosis
a Investigations
a Clinical Diagnosis
a Tieatment
a Final Diagnosis
NEONATOLOGY
A newborn is more prone to develop hypothermia because
of large surface area per unit of body weight. An LBW' baby
has decreased thermal insulation due to lesser subcutaneous
and brown fat. Brown fat is the site of heat production. It is
located around the adrenal gland, kidneys, nape of the neck,
interscapular and axillary regions. Metabolism of brown fat
results in heat production. Blood fowing through the brown
fat becomes warm and through circulation transfers heat to
other parts of the body (non-shivering thermogenesis).
Newborn loses heat by euaporation (due to evaporation of
amniotic fluid from skin surface) , conduction (by coming in
contact with cold object, cloth, tray, etc.), conuection (by air
current in which cold air replaces warm air around baby), and
radiation (to colder solid objects in viciniry such as walls).
The newborn gains heat by conduction, convection, radia-
tion, and non-shivering thermogenesis.
Normal temperature 36-5-37: C
Mild hypothermia (cold stress) <36.5-36104 C
Moderate hypothermia <36.0-32.00 C
Severe hypothermia <32.A" C '
MEASUREMENT
The neonatet arm should be adducted with the thermometer
bulb deep in the axilla. The thermometer should be kept in
situ for full 3 minutes. Axillary temperature refects rectal tem-
perature when taken properly. Abdominal temperature assessed
by touch is representative of core temperature.
The warm and pink feet indicate that the baby is in thermal
comfort. But when feet are cold and abdomen is warm to touch
it indicates thai the baby is in cold stress. In hypothermia
both feet and abdomen are cold to touch.
WARM CHAIN
Baby must be kept warm at the place of birth, during trans-
portation, or within the hospital.
Ten steps of warm chain maintenance:
1. -Warm deiivery room (>25" C)
2. \7arm resuscitation
3. Immediate drying
4. Skin-to-skin contact between baby and mother
5. Breast-feeding
6. Bathing postponed
7. Appropriate clothing
8. Mother and baby nursed together
9. \farm transportation
10. Tiaining of healthcare providers
ESSENCE OF PEDIATRICS

Kongoroo Molher Core (KMC) Table 2.1: Daily Fluid Requirement (Approximate) in lnfancy
and Childhood
Its key features are (i) early, continuous and prolonged
skin-to-skin contact between the mother and the baby and
(ll) exclusive breast-feeding initiated in the facility and 1't day 60

continued at home. 2id clay BO

3d day 100
e Ail babies <2000 grams are candiciares for KMC.
120
r Place the baby naked with or without a nappy, upright inside
4th day

mothert clothing against skin (a loose blouse, sweater tight 5'h day 140

at the waist holds the baby). 6d'day & 7'h day onwards 150
r Let the baby suckle at the breast as often as he wants, but Up to 9 months 1 50-1 60
at least 2 hourly. 12 months 1 20-1 50
o Keep baby in propped-up position. 2 years 1 00-1 20
o Make sure the baby stays warm all time. 4 years 90*1 00
o \7hen mother wants to bath or to take rest, ask the father
B years 70-90
or another family member to kangaroo the baby or wrap
1 2 years 60-70
the infant in several layers of warm clothing.
1. Daily requirement of fluid for normal-term newborn starts with 60 ml/kg; for
LBW babies, it can be started with B0-100 ml/kg.
2. Daily increment of fluid in normal weighing baby is 20 ml/kgid, and in LBW
baby it is 1 5 ml/kg/d. ln LBW babies, amount of fluid can be raised up to 200 n1l/
kg/d by 1 5'h day.
3. Babies receiving phototherapy will need 1 0-1 5 ml/kg/d of extra fluid.

In a newborn full-term baby, the total body water is approxi-
mately 75o/o of the body weight, while in older children and
adults it is about 60-650/o. Maintenance of fluid in this period
is very important. See Tabie 2.1 for approximate daily iluid
requirement during infancy and childhood.
MITIA
Yellow-white spots on the nose, 1 mm size (pin headed) due
to retention of sebum are present in pracdcally all babies and
disappear spontaneously.

sToRK BTTES (SAIMON PATCHES OR NEVUS

TOXIC ERYTHEMA OR URTICARIA
NEONATORUM
It is an erlthematous rash with central pale papule appearing
on the second or third day in term babies. The rash starts on
the face and spreads to the trunk and extremities in about 24
hours. It disappears spontaneously within a few days without
any specific treatment. The exact cause is not known, but it is
considered as an early marker of atopy. The scrapings from the
skin lesions show eosinophils, no bacteria have been cultured
from the lesions. Increased family history of atopy among
relatives ofinfants suffering from urticaria neonatorum further
supports allergic etiology. The rash should be differentiated
from pyoderma and skin iesions of congenital syphilis.
PEETING SKIN
Dry skin with peeling and exaggerated transverse skin creases
is seen in post-term and some term small-for-date babies.
Application of emollients, creams, or oil provides relief.
srMPrEX)
These are discrete pinkish-gray sparse capillary hemangiomata,
commonly located at the nape of neck, upper eyelids, forehead,
and root of the nose, present in 30-50% of normal newborn.
They invariably disappear after a few months.
MONGOTIAN BLUE SPOTS
In babies of African and Asiatic origin, irregular biue patches of
skin pigmentation are often present characteristically over the
sacral area and buttocks though extremities and rest ofthe trunk
may also be affected. The macule may be as big as 10 cm in
diameter. ,The cause is infiltration of monocytes in deep layer of
dermis. These invariably disappear by age of 6 months.
SU BCONJ U NCTIVAL H EMORRHAGE
Semilunar arcs of subconjunctival hemorrhage, located at the
outer canthus, are a common finding in normal babies. Their
presence is brought to the notice of the physician by the
observant mother. The blood gets reabsorbed after a few days
without leaving any pigmentation.

1
.t
.i
{

EPSTEIN PEARTS
These are white spots, usually one of either side of the median
raphe of the hard palate and are of no significance. They are
possibly epithelial inclusion cysts.
CONGENITAT TEETH
Rarely, the newborn may be born with one or two already
erupted teeth. These need not be extracted unless they interfere
with breast feeding or are loose.
TONGUE.TIE
It may be either in the form of thin broad membrane or thick
fibrous frenulum with a notch at the dp of tongue. Tongue-tie
seldom interferes with sucking or, later, speech development.
The condition is uncommon and is often over-diagnosed. The
genuine tongue-tie may be snipped after 3 months if it is a
source of anxiety to the mother.
NON. RETRACTABTE PREPUCE
The prepuce is normally non-retractable in all male newborn
babies and should not be diagnosed as phimosis. The urethral
opening is often pinpoint and is visualized with difficulty. The
mother should be advised against forcibly retracting the foreskin.
HYMENAI TAGS
Mucosal tags at the margin of hymen are seen in rwo-thirds
of female infants.
PROMIN ENT XIPHISTERN UM
Xiphisternal cartilage may stand out rather prominently and
is of no significance.
UMBITICAI HERNIA
V4ren the cord has fallen ofi umbilical hernia may manifest
after the age of 2 weeks or later. It may be associated with
divarication of recti. Most of these disappear spontaneously
by l-2 years of age. Application of a coin and bandage over
the hernia is not recommended.
DISORDERS DUE TO TRANSPTACENTAT
PASSAGE OF HORMONES
Mostitis Neonolorum
The engorgement of breasts occurs in full-term babies of
both sexes on the third or fourth day and may last for days
NEONATOLOGY
or even weeks. Lack of inactivation of progesterone and
estrogens after birth due to immaturiry of neonatal liver leads
to further rise in their levels; thus resulting in hypertrophy
of breasts. The local massage, fomentation, and attempt to
express the milk should not be made, and mother should
be reassured.
Voginol Bleeding
The development of menstrual-like withdrawal bleeding may
occur in about one-fourth of female babies after 3-5 days of
birth. The bleeding is mild and lasts for 2-4 days. The local
aseptic cleaning of genitals is advised. Additional vitamin K
is unnecessary. No specific treatment.
Mucoid Voginol Secrelions
Most female babies have copious grayish-white mucoid vaginal
secretions due to female sex hormones. These should not be
mistaken for purulent discharge.
ANENCEPHAI.US
It is a lethal congenital malformation in which both cerebral
hemispheres and several other areas of brain (brain stem,
cerebellum, pituitary gland) are absent. Frontal bones and
vault are deficient, and deformed brain is exposed to view. It
can be diagnosed antenatally by detection ofelevated levels of
alpha-fetoproteins in the maternal serum and amniotic fluid
around 14-16 weeks of gestation. Anencephaly in newborn is
not compatible with life, most infants are stillborn, but those
born alive die within few hours or days.
MICROCEPHATY
The head size of less than 3'd percentile for the gestational
age may result either due to craniosynostosis or arrested brain
growth. Intrauterine infections and birth asphlxia are other
important causes of microcephaly.
MENINGOCETE
This is a pedunculated swelling on the skull or spinal column
due to herniation of the meninges; it contains cerebrospinal
fluid. If the sac contains spinal cord tissue, it is called menin-
gomyelocele.
Meningocele may be excised; but meningomyelocele, when
with paralysis of the lower limbs and bladder &
associated
bowel dysfunction, is often inoperable. Shunt operation for
hydrocephalus is required sooner or later.
 5

ESSENCE OF PEDIATRICS

CHOANAT ATRESIA
In choanal atresia, the baby has severe respiratory difficulty
dating from birth, because the posterior nasal air passage is
blocked by a bony or membranous septum. The baby breathes
through the mouth and becomes cyanosed when his mouth
is closed and during feeding. Breathing improves and color
returns to normal as the baby starts crying. Unilateral atresia
may be asymptomatic. The inability to pass a catheter through
the nostrils confirms the diagnosis. Diagnosis can be established
by instilling a small amount of water-soluble contrast agent
into the posterior nares and obtaining a radiograph of the skull
in the lateral position.
Tieatment of choice is surgery; operation may be deferred till
the age of 2-3 months, when the nasal passages are bigger.
CONGENITAT DISTOCATION OF
THE H|PS (CDH)
The exact etiology is unknown. The condition is more common
among first born, post-term, large infants and female babies
delivered following breech presentation. In breech presentation,
the head of the femur may get dislocated upward and baclcvard;
its constant pressure over the dorsal aspect of the ileum may
cause development of a false acetabulum. The dislocation is
bilateral in about 30-40o/o of cases.
Asymmetry of the thigh, inability to abduct the hip fully,
shortness of the affected leg, reduced spontaneous movements,
and a bulge of the femoral head must arouse suspicion. The
routine examination of the hips in all infants for Ortolani click
is essential for early diagnosis. The click may not be audible
in the newborn baby, but the middle finger on the greater
trochanter can be guided to feel the entry of the femoral head
into the acetabulum (Barlow sign).
Tesls for CDH
Ortolani test It is a sign of entry of the dislocated femoral
head into the acetabulum. The infant is placed supine. Vith
one hand, the pelvis is steadied. Vith the other hand, the knee
of the tested side is flexed acutely and the hip is flexed to 90
degree. The tips of the index and middle fingers are placed
over the greater trochanter. The thumb is placed across the
knee. As the hip is gently abducted, the femorai head reduces
with a clunk. \fhen the hip is adducted, the femoral head is
displaced with a clunk.
Barlow test: It is a provocative test of dislocation. The infant
is placed supine. The untested hip is in mid-abduction and
90 degree flexion. The tested hip is in slight adduction and
45 degree flexion. The tips of middle and index fingers
are over the greater trochanter on the lateral aspect of the
upper thigh. The thumb is over the medial aspect of the
lower thigh. The femoral head is pushed posteriorly and
laterally. In the dislocatable hip, the femoral head moves
out with a clunk.
The x-rays should be taken with both hips extended and legs
held in 45 degree abduction and full internal rotation. The
conventional x-rays of the hips may not show any abnormality.
Early management is essential for optimal functional recov-
ery. The legs are maintained in the position of abduction and
external rotation by the use ofVon Rosen splint. The recovery
is generally complete in a period of about 3-4 months. \Tithout
treatment, delay in learning to walk occurs.
TATEPES EQUINOVARUS
It is characterized by plantar flexion (equinus) and inversion
(varus) of the ankle so that dorsiflexion of the foot is limited;
and unlike normal babies, the dorsum of the foot cannot
be made to touch the front of shin. Skin may be puckered
or grooved on the medial side of the foot. In bilateral cases,
spine should be examined for meningocele and any tuft of hair
to suggest dermoid. The other joints should be examined to
exclude arthrogryposis multiplex.
In mild cases, manipulations (eversion and dorsiflexion)
alone are enough to correct the anomaly. In established or
ankylosed cases, early manipulations and plaster applications
are needed. The corrective casts should be applied as soon as
possible after birth. The plaster is changed and foot is manipu-
lated weekly for the first 2 months, then every 2 weeks until
full correction has been achieved.
The stridor is caused by flaccidity or easy collapsibility of
aryepiglottic folds or epiglottis in general. Stridor, usually present
from birth, may not appear until 2 months in some patients.
The stridor is inspiratory, low pitched, may be associated
with dyspnea, inspiratory retractions in the supraclavicular,
intercostal, and subcostal spaces. When retractions are severe,
thoracic deformiry may result. Symptoms may be intermittent,
and are worse when the infant lies on his back. Stridor may
be aggravated by excitement, crying, feeding or sleeping or by
infectioq. The condition disappears spontaneously by 6-18
months of age. These infants are, howevet prone to develop
aspiration of feeds and frequent lung infections.
Laryngomalacia can usually be diagnosed by direct
laryngoscopy. The differential diagnosis includes supraglottic
causes, e.g., micrognathia (Pierre Robin syndrome, Tleacher
Collins syndrome), macroglossia (Beckwith \Tiedmann
syndrome, hypothyroidism); glomic causes, e.g., laryngeal web or
papilloma, vocal cord paralysis, birth injury to recurrent laryngeal
nerve; infraglottic causes, e.g., congenital subglottic stenosis, t
1
tracheal stenosis or tracheomalacia, vascular ring, congenital
1
1
ti
a

goiters, hemangioma. Generalized severe chondromalacia of
larynx and trachea should also be differentiated.
TREATMENT
. Usually no specific therapy is indicated, the condition
resolves spontaneously, so assurance is needed.
o Most patients seem more comfortable or less noisy lying
prone or lateral positions.
o Severe symptoms may require tracheostomy (in one review,
only 4 of 1415 patients required tracheostomy).
!
Y and aorta by the gravid uterus.
The most common anomaly of tracheal structure, although a
t c.
I less common cause of upper airway obstruction, is tracheo-
L malacia.In tracheomalacia, the cartilage rings do not extend
r nearly so far around the circumference, and thus a larger
I portion of the tracheal wall is membranous. Therefore, the
I umbilical cord as a result of knotting of the cord.
lumen of the intrathoracic portion of the trachea tends to
I f.
l' collapse during expiration, and persistent wheezing may be a
prominent symptom; tracheomalacia localized to the cervical
L
I
v
trachea may result in inspiratory obstruction. Tiacheomalacia is
!
)
almost invariably present in children who have had esophageal
I
atresia and a tracheo-esophageal fistula.
t tacheomalacia must be differentiated from extrinsic com-
V pressing lesions.
t.
TREATMENT
a Usually no specific therapy is indicated.
a Some patients may require long-term tracheostomy and
ventilatory support.
Although tracheomalacia usually has resolved by 18 months
of age, some degree of inspiratory obstruction may persist
later in childhood.
Perinatal asphyxia is an insult to the fetus or newborn infant
due to lack of oxygen (hypoxia) and/or a lack of perfusion
(ischemia) to various organs, which will manifest as difficulry
in establishing spontaneous respiration evident by delayed cry
after birth, at least after 1 minute.
ESSENTIAL CRITERIA
o Profound metabolic or mixed acidemia (pH <7) on umbili-
cal cord arterial blood sample, if obtained.
NEONATOLOGY
o Persistence of an Apgar score of <3 for more than 5 minutes.
o Neurologic manifestations in the immediate neonatal
period-seizure, hypotonia, coma, or hypoxic ischemic
encephalopathy.
o Multi-organ system dysfunction in the immediate neonatal
period.
ETIOLOGY
l. Fetal hypoxia
a. Inadequate oxygenation of maternal blood as a result
of hypoventilation during anesthesia, cyanotic heart
disease, pneumonia, respiratory failure.
b. Low maternal blood pressure as a result of hypoten-
sion, e.g., in anesthesia, compression of the vena cava
Inadequate relaxation ofuterus in uterine tetanycaused
by excessive oxFtocin.
d. Premature separation of placenta.
e. Impedance to the circulation of blood through the
Placental insufficiency (in toxemia, postmaturity).
) After birth
a. Severe anemia (in hemorrhage or hemolytic disease)
b. Shock (in severe infection, massive blood loss,
intracranial or adrenal hemorrhage)
c. Failure to breathe adequately (trauma, narcosis,
cerebral defect)
d. Failure of oxygenation (in pulmonary or heart diseases)
e. Unscientific delivery
PHYSIOTOGY
\X/hen babies become asphyxiated (in utero or after delivery),
they undergo a well-defined sequence of events:
o Primary apnea: An initial brief period of rapid breathing
followed by a cessation of respiratory movements; heart rate
begins to fall, muscle tone gradually diminishes, baby enters
a period of apnea, known as primary apnea.
o Secondary apnea: if asphlxia continues following primary
apnea, the infant develops deep gasping respiration
(3-6 breaths/min), heart rate continues to decrease, the
blood pressure begins to fall, and the baby becomes nearly
flaccid. This is secondary apnea.
Clinically, primary apnea and secondary apnea are virtually
indistinguishable. An infant may go through primary and
secondary apnea while in utero. So when an infant is apneic at
binh, it is verv difficult to say whether it is primary or secondary
apnea. A newborn in primary apnea may re-establish breathing
without extensive intervention, but an infant in secondary
apnea will not recover without help. Oxygen is required to
 NEONATOLOGY

Table 2.4= Sarnat & Sarnat Stages of Hypoxic lschemic o Make sure that chest moves up with each press on
Encephalopathy** the bag.
r Ventilation with bag & mask at 40-60 breaths/min.
C. Circulation:
Level of Hyperalert Lethargic Stuporous,
consciousness c0ma
90 compressions coordinated with 30 brea hs per
minutes (3 compressions: 1 breath every rwo si conds).
Muscle tone Normal Hypotonic Flaccid
Place thumbsjust below the line connecting the nipples
Posture Normal Flexion Decerebrate
on the sternum.
Tendon reflexes/ Hyperactive Hyperactlve Absent a Compress 1/3 the A-P diameter of the chest.
clonus
a Inform the guardian about the present condition ofthe
Myoclonus Present Present Absent
baby and what you are doing.
Moro reflex Strong Weak Absent
If after 20 minutes of resuscitation, the baby is not breathing
Pupils Mydriasis Miosis Unequal, poor
light ref lex and pulse is absent, cease efforts. Explain to the mother/father
that the baby has died.
Seizures None Common Decerebration
Electroence- Normal to Lor,r, voltage Burst
Endotrocheql lnlubolion
phalogram isoelet trit changing suppression
(seizure activily) Endotracheal intubation is required in only small proportion
Duration <24 hr il 24 hr to 14 Days to weeks of asphyxiated neonates.
progressesi days
otherwise, may
remain normal
lndicolions
Oulcome Cood Variable Death, severe (z) Vhen tracheal suction is required, (il) when prolonged
delicits positive pressure ventilation is required, (iii) when bag and
*Modified mask ventilation is ineffective, (iu) when diaphragmatic hernia
tThis staging is ior iniants of >36 wk gL-stational age is suspected, and (z) when thick meconium is present.

Technique
PERINATAL TREATMENT
Position the child flat on the back with neck slightly extended.
Monitoring of heart rate, ultrasound, fetal scaip pH, etc. are Use a ro11 under the shoulder, if necessary to maintain the head
valuable information about fetal condition, asphlocia and prog- in correct position. The laryngoscope is designed to be held in left
ress of labor. These will guide the obstetrician to take decision hand for both right- and left-handed persons. The goal in inserting
about to perform cesarean section, augment vaginal delivery, the laryngoscope blade is to slide it over the tongue with the tip of
or to allow progression of labor. the blade resting in the vallecula (the area between the base of the
tongue and the epiglottis). To view the glottis, lift the entire blade
in the direction of the handle (perpendicular to blade). Keep the
DETIVERY ROOM TREATMENT
glottis in view and insert the tip of an endotracheal tube. This wiil
The steps of neonatal resuscitation include: A. establish a position the tube in trachea, approximately half the way between
patent airway by suctioning, if necessary by endotracheai the vocal cords and carina. Appropriate sizes ofendotracheal tubes
intubation; B. initiate breathing by tactiie stimulation or with respect to weight of the baby and gestational age at delivery
PPV with a bag and mask or through endotracheal tube; have been listed in Table 2.5.
C. maintain circulation with chest compression and medica-
tions if needed. The algorithm of resuscitation to be followed Moinlenonce of Body Temperoture
in the delivery room, as suggested by VHO/UNICEF, is Place the newborn under radiant warmer after proper drf ing.
depicted in Fig.2.1, and invertogram ofneonatal resuscitation Hypothermia leads to increased metabolic needs, which lead
has been given in Fig. 2.2.
A. Airway
Table 2.5: Enclotracheal Tube Size rvith Respect to WeiSht of
Suction Airway: If
there is meconium-stained lluid and
the Baby ancl Cestational Age at Delivery
baby is not crying and moving limbs, suck the mouth,
nose, and oropharynx and do not suck right dox'n the
throat as these can cause apnea or bradvcardia. 2.5 <1 000 <28
B. Breathing: 1.0 1000-2000 zo )+
o 2000-3000 l+-J o
Choose mask size 1 for normal weight baby and zero
4.0 >3000 >38
for <2.5 kg baby.
ESSENCE OF PEDIATRICS

Dry baby with clean cloth and place baby where it will
be warm

. Breathing or crying
. Good muscle tone Routine care
. Color pink
30 No
sec
. Position the head of the baby in neutral position to
open the airway
. Clear airway if necessary Breathing & pink Routine care & observe #
. Stimulate, reposition closely g

. Give oxygen, if necessary

Not breathing, cyanosed

Use a correctly fitting mask and give the baby five

slow ventilations with bag

30
sec
. Check position and mask fit
'Adjust position if necessary
o Provide ventilation with bag & mask
. lf chest wall not moving well
'Suction airway

Callfor HELP

Compress the chest

lf HR >60/min

. Continue to bag @ 40 breaths/min

o Mak€ sure lhe chest is moving adequately
. Use oxygen if available
,.l Everv 1-2 min stop and see if the pulse or
breathing has improved
. Stop compressions once the H/R is >100/min
. Stop bagging when R/R is >30/min
. Continue oxygen until pink and active

Fig. 2.1: Delivery room management.

to acidosis, myocardial depression, hypotension, bleeding ten- Inadequate spontaneous respiration

dency, and pulmonary hemorrhage. Continuing hypoxia/hypercarbia

Mqinlenonce of Adequote Oxygenolion Moinlenonce of Adequote Perfusion

o Nasal cannula: preferable (not >2 L/min) r Immediate opening of an IV access.
o Face mask o If the baby is in shock (CRT >3 sec)
o Head box r Volume expander: Blood, normal saline, Ringer lactate
o Assisted ventilation, in case of: (10 ml/kg over 5-10 minutes). This may be repeated if
o Apnea required.

t

Drying wrapping tactile stimulation
Positioning, + suction if meconium
Fig. 2.2: lnvertogram of neonatal resuscitation.
Inotropics:
Dopamine (starting dose 3-5 pg/kg/min, can be
- increased up to 10 pg/kg/min)
Dobutamine: After giving dopamine, if perfusion is
- still poor (same dose)
Mointenonce of Normol Serum
Glucose level
o Immediate capillary glucose estimation should be done
with glucometer.
o Both hypoglycemia and hyperglycemia (>125 mgldl or 7 '5
mmol/L in term and >150 mg or 8.5 mmol/L in preterm
neonate) merits immediate attention.
Control of Seizure
o Any neonatal seizure should be identified immediately and
treated promptly.
o Phenobarbitone is the drug of choice. Loading dose, 20
mg/kg stat over 10 minutes; then, if not controlled, repeat
l0 mg/lg, if still not controlled then again repeat 10 -g/kg-
rctal 40 mg/kg.
o If not controlled with above measures, switch to second-
third-line medications (phenytoin, midazolam, or lidocaine)
o After control of seizure: Maintenance dose of PHB (5 mg/kg/d
in 2 divided doses) is continued until EEG is normal or
no clinical seizure for >2 months.
Corticosteroids have no role in the treatment of asphyxia.
Subsequenl Treolmenl
o Keep the baby nothing per oral for 48-72 hous.
o Give appropriate IV fluid with 30% curtail (10% DA for the
first2448 hours followed by 10% dextrose in baby saiine.)
r Inj. vit K, should be given.
NEONATOLOGY
Treotment of Other Complicotions
r Anuria
o DIC
o Dyselectrolytemia
DISCHARGE CRITERIA
o Baby is on full oral feed
o Vital signs are stable
o Convulsion free with/without anti-convulsive drug
The newborn baby may be LB\f because of prematuriry or
intrauterine growth retardation (iUGR). Approximately one-
third of LB\f neonates are preterm. The second situation that
leads to LB\il/ is IUGR; the gestation may be full term or
preterm, but the baby is malnourished and, therefore, LBV;
such a baby is also called a small-for-date (SFD) baby' Two-
thirds of LB\f neonates fall in this category. At times, an LB'$7
neonate may be both preterm as well as SFD.
CAUSES OF PRETERM BIRTH
o Fetal Fetal distress, multiple gestation, erythroblastosis.
o Placental: Placental dysfunction, placenta previa, abruptio
placentae
o (Jterine: Bicornaute uterus, incompetent cervix (premature
dilatation)
e Maternal: Teenage mother, Preeclampsia, Chronic medical
illness (e.g. cyanotic heart disease, renal disease), infections
(e.g., use of tobacco.
UTI, chorioamnionitis),
o Others: Premature rupture of membranes, polyhydramnios
and iatrogenic (DM and Rh incompatibility).
o l]nknown
CAUSES OF INTRAUTERINE GROWTH
RETARDATION
Fetal: Chromosomal disorders (e.g., trisomies), chronic fetal
infections (e.g., cytomegalic inclusion disease, congenital
rubella, syphllis), radiation, multiple gestations.
Placental:
o Decreased placental weight or cellularity or both
o Villous placentitis (bacterial, viral, parasitic)
o Tumor (chorioangioma, hydatidiform mole)
o Placental separation, infarction
o Twin-rwin transfusion syndrome
Maternal: Toxemia, hypertension or renal disease or both,
hypoxemia (high altitude, cyanotic cardiac or pulmonary
disease), malnutrition or chronic illness or anemia, drugs
(narcotics, alcohol, use of smoking or smokeless tobacco).
 _!

ESSENCE OF PEDIATRICS

DISTINGUISHING PRETERM AND SFD
It is desirable to make clinical distinction between the two rypes
ofLB\X/ babies. A preterm baby is diagnosed on the basis ofthe
period of gestation calculated from the last mensrrual cycle of
tne mot-ner orlrom-NB) score [or)\BS system see ]ig. 2.3).
If it is less than 37 completed weeks, the baby is designated
as preterm. Preterm babies also have distinct physical and
neurological fearures, which help in their recognition. The deep
skin creases over the soles are present only over the anterior
one-third. The external ear or the pinna is soft and devoid of
cartilage, and it does not recoil back qromqtll on beingfolded.
In males, the scrotum does not have rugae and testes may nor
be descended into the scrorum. In female infants, the labia are

Neuromuscular maturity

Score

q
-1 0 1 2 3 4 5

Posture ca< c€E

Square
window
(wrist) r
' >go'
a-,
I on'
f-
' 60' h
t 4s'
h
' 30' 1,"
Arm
recoil & ,8" "& -& a
e
1 80. 140'-1 80' 110"-140' 90'-'11 0' <90"

Popliteal
angle A CC140" C- CO 00" -l
(AJ<90'
--
1 80"

o
1 60'
A
120" 1

"5 90'
Scarf
sign
8, - t)- -& ..-v
H
_- 8i -8
Heel
to ear € 6 d3 c(= @
Physical maturity

Sticky, Gelatinous, Superficial Parcnment,

Smooth pink; peeling Cracking, Leathery,
Skin friable, red, pale areas; deep
visible veins and/or rash; cracked,
transparent translucent cracking;
rare veins wrinkled
few veins no vessels

Lanugo' None Sparse Abundant Thinning Mostly bald

Maturity
Bald areas
rating
Heeltoe
Plantar Anterior Score Weeks
40-50 mm: >50 mm, Faint Creases Creases over
transverse
surface -1 no crease red marks anlerior 213 entire sole -10 20
<40 mm: crease only
-2 -5 22
Barely Stippled Raised U 24
Breast lmperceptible Flat areola, Full areola,
percepiible areola, areola,
no bud 5-10 mm bud 5 26
1-2 mm bud 3-4 mm bud
weil-curved 10 28
Lids fused Lids open: Slightly Formed and
curved pinna: pinna; Thick 15 30
Eye/Ear loosely: -1 pinna flat firm,
soft; soft but cartilage,
tighty: stays folded instant
-2 slow recoil ready recoil ear stiff 20 32
recoil
25 34
Scrotum Testes in Testes Testes
Genitals Scrotum flat, Testes down,
(male) empty, upper canal, descending, pendulous, 30 36
smooth good rugae
faint rugae rare rugae few rugae deep rugae 35
Clitoris Clitoris MaJOra and 40 40
Genitals Clitoris Majora covers
(female) prominent,
prominent,
small
prominent,
enlarging
minora
equally
Majora large,
clitoris and 45 42 I
labia flat minora small t
labia minora minora prominent minora 50 44 i
1
Fig. 2.3: New Ballard Scoring System (modified) for determination of gestational age. 1
tl
I
 NEONATOLOGY

Table 2.6: Physical Features Diiferentiating Preterm and Table 2.7: Problems of Preterm and Term Small-for-Date Babies
Small-for-Date Babies

lntrauterine hvporia + ++
Respiratory diflit ulr ies
Weight for age corresponds to Weight for age less than + ++
Birth asphyxia
geslalional age gestational age (<1 Oth percentile) 0 +
Meconium aspiration
++ 0
2 Small but plump Wasted Hyaline membrane disease
++ 0
Apnelc attack
3 Pi nk White or pale pink
Feeding difficulties
4 Length <50 crn Length >50 cm ++ 0
lnability to suck and swallow
Head circumference <35 cm Head circr-rmference >35 cm
++ (.)
5 Aspiration of feeds
6 Lanugo hair Thick, dark hair Syrnptomatic hypoglycemia + ++
7 Skin: shiny transparent, thin, Skin: dry, loose, thick Hypothermia ++
edematous
Hyperbilirubinemia ++ +
Ears, breast tissue, genitalia-al I Ears, breast tissue, genitalia-all
Liabilities to infection
immature mature ++ +
(necrotizi ng enterocol itis)
9 Hypotonic ({loppy) Cood muscle tone
Congen ital malformations +++
Hemorrhage
ntraventricu ar
+0
widely separated and do not cover the labia minora, resulting I I
+++
Pulmonary
in the prominent appearance of the clitoris. The back of the
preterm babies has abundant growth offine hair called lanugo. Prognosis
lmmediate High mortality Better prognosis
Small-for-date neonates have an emaciated look and loose Future physical and mental Cood if no Poor in hypoplastic
folds of skin because of lack of subcutaneous tissue; these are development perinatal babies
particularly prominenr over rhe buttocks and the thighs. They complication
are undernourished, undersized, and r-rnderweight. They look OCCU TS

alert and often plethoric. In SFD babies, the head circumfer- Note: 0 =:ibscnt; + - common; ++ - more common.
ence exceeds the chest circumference by more than 3 cm. The ln preterm small forclates babics, cornbincd hazarcls oi immaturiry and intr.ruterine
growth retardaiion would be manifested.
SFD babies are often full term or borderline term in gestation.
When their birth weight is plotted on the intrauterine growth
chart, it falls below the tenth percentile. o Treatment of: complicarions
Refer Thble 2.6 for differentiating physical features of preterm
o Monitoring
and small-for-date babies and Table 2.7 for the problems faced Keeping Wqrm
by these babies.
r Infant should be dried
DEFINITIONS o Clothing covering the whole body except the face
o Room temperature: 30-32" C
o Preterm: Baby born before 37 completed weeks. o Humidity: 40-650/o
o Low birth weight (LB\7): Birth weight <2500 g. r \(/arm under heater/radiant warmer
r Very low birth weight (VLBW): Birth weight <1500 g. o Vindows closed
o Extremely low birth werght (FI B\$!r): Birth weight <1000 g. o Cap over the head
o Incredibly LBW: Binh weight <750 g. o Gloves in hands and feet
o Kangaroo mother care for healthy newborn
INDICATION FOR HOSPITALIZATION OF Incubator care
PRETERM LBW INFANTS
a Weight <1500 g
r Birth weight <1800 g a Very sick nervborn
o Gestation <34 weeks a Temp setting:
o Not able to take feed from breast/cup and spoon r Environmental temp: 32-35' C
r Any sick neonate irrespective of birth rveight/gestation r Babv skin ternperature: 36-36.9' C
Hunridin': 50 600/o
MANAGEMENT
o Keeping the baby warm
Fluid & Nutrition Requiremenfs
o Fluid and nutrition Some preterm low birth weight babies may not tolerate oral
o Prevention of infection or naso-gastric tube feeding; they should be kept nothing by
ESSENCE OF PEDIATRICS

Table 2.8; Fluid and Nutritional Requirements of a Low Birth o Problems with feeding
Weight Neonate
o Gastro-intestinal dysmotility Tieat with domperidone,
l
o Gastro-esophageal refux o.z o.s mgikg/dose hourly.
J
o Necrotizing enterocolitis: In LB\fbabies, formula milk
feeding is associated with incidence of necrotizing en-
terocolitis 10 times higher than with breast milk.
Feeding (NC,
lV fluid BF + EBM 'When to stop feeding
cup or spoon) r
NG feeding IV/BF BF r Abdominal distension
Cup & spoon BF BF o Feeding intolerance
NC, nasogastric; BF, Breast-feeding; EBM, expressed breast milk
o Apnea
o Respiratory distress
o Convulsions
mouth, and intravenous fluid should be given as suggested. o Sepsis/necrotizing enterocolitis
Fluid and nutritional requirements of an LB\W neonate have o Surgical problem
been given in Thble 2.8.
r Indication for initiation of oral feeding: No abdominal
On the other hand, feeding should be delayed in case of
distension, bowel sounds are present, meconium passed,
premature newborn:
no frequent vomiting, no blood stained/bilious gastric
r \7ith H/O perinatal asphyxia aspirates, R/R <60/min, no apnea/respiratory distress and
o On mechanical ventilation no convulsions-
o \7ith hemodynamic instability o Nutrition supplement
o \il/ith necrotizing enterocolitis o Vitamin K, within 4hr,4 days, 4 weeks
o \(/ith frequent episode of apnea and bradycardia o Multivitamin from D,,
fluman milk is preferred for feeding term, preterm, and o Folic acid from D,,
sick infants. o Iron from 6 weeks to l-2 year of age (2 mg/kg/d)
o 200 mg/kg of calcium and 100 mg/kg of phosphorus
Types of Fluid

o l"'24 hours: <1000 g-5% DA; >1000 g-10% DA. Prevenlion of lnfeclion
o After 24hours: <1000 g-5% dextrose in 0.225olo NaCl; o Hand washing
>1000 g-10% dextrose in 0.225Vo NaCl. Add potas- o Minimal handling
sium 1-2 mEq/kg/d from day 3 in the newborn who is o Breast feeding
or-r NPO. o Visitors restriction
o Asepsis of instrument
Amount of Fluid o Care of umbilicus
o Day 1: 60-100 ml/kg/d (.1 k, 100; 1-1.5, 80; >1.5, 50)
o Daily increase 20 mllkgld lndication for Antibiotics
o Premature rupture of membrane (PROM) > 18 hours
Feeding o Mother who had infection
o Trophic feed/gut priming o H/O repeated & unclean PV exam of mother
,r <1500 g: 0.5 ml 4 hourly o Invasive inter-vention in baby
,r >1500 g: I ml4 hourly o Resuscitation
o Signs of sepsis
Increment 10-20 ml/kg/d
o Modes of feeding: IV feeding, NG tube feeding, breast- Treqtment of Complicolions
feeding including colostrum. Breast milk is the best food for
an LB\Z baby. Baby should be put on to the bare abdomen
o Eady complications
of the mother within half an hour after birth. In absence o Respiratory distress syndrome (RDS), recurrent apnea,
of breast milk, EBM or banked breast milk or wet nursing PDA
'W'hen o Hypothermia, hypoglycemia, dyselectrolytemia, edema
can be done. baby can take orally, demand-feeding :
or 3-hourly feeding can be started. \X4ren baby passes urine c Infections: Sepsis, necrotizing enterocolitis 1
>6 times/d or gains weight 20-30 g/d, feeding is adequate. o IVH, HIE, retinopathy of prematuriry seizures .,t

Feeding with milk powder or bottle feeding is harmful. r Jaundice. kernicterus, anemia 1
1
1
I
 !

NEONATOLOGY

Late complications 3. Anterior fossa hemorrhage

o Neurodevelopmental disorders: Cerebral palsy, mental a. Subdural
retardation, epilepsy b. Intraparenchymal
t.) Seizures, visual and/or hearing problems 4. Subarachnoid hemorrhage (SAH)
iJ Behavioral problem
() Anemia SUBEPEN DYMAL HEMORRHAGE.
TNTRAVENTRTCU rAR HEMORRHAGE (SEH - rVH)
Monitoring
I o Colot vital signs, activiry, tissue perfusion (capillary refill time)
Bleeding from the subependymal germinal matrix with or
without subsequent rupture into a ventricle occurs before 34
rv o \(/arm and pink hands and feet weeks of gestation.
Y o lJrine output >1-3 ml/kg/hr
t r Feed tolerance Clinicol Feolures
t e Development of complications
i- Clinical symptoms and signs may occur as a result of blood
I'
v o \Teight gain 20-30 gld
volume loss or neurologic dysfunction. The clinical presentation
L
l" depends on the size, site, and rapidity of the hemorrhage. IVH
r DISCHARGE CRITERIA can present as a catastrophic event when blood loss is large
I
Iv o Can take total feeding by mouth and rapid. Presentation can be stuttering, with intermittent
periods of stabilizing when there is a slower evolution of blood
L
i o Maintain nbrmal temperature in open air
loss. A clinically silent presentation may occur in up to 50%
I
I o After attainment of 34 weeks of gestation and weight >14009
of cases, usually wrch smaller hemorrhages. See Thble 2.9 for
o Consistent weight gain for at least 3 days
clinical presentations of SEH-IVFI.

PROGNOSIS
Mortality of LBW babies is inversely related to the gestation
and birth weight, and directly to the complications. More than
90o/o of LB\if babies have no neurodevelopmental handicaps.
The outlook for uncomplicated premature babies is as good as
that for babies born after full maturity. In fact, several renowned
and famous people who were premature grew up to become leaders
and intellectuals. Sir Isaac Newton, the greatest mathematician
genius, weighed merely 3 lbs at birth. Sir Winston Churchill, the
legendary Prime Minister of Britain and Nobel Laureate was born
after 7 months of pregnanry when his mother was participating
in royal dance. The world-renowned artist Pablo Picasso came
into this world a bit too early. The parents of premature children,
therefore, should not feel despondent.
Diognosis
CSF study: Clinical signs and symptoms, combined with
the presence of hemorrhagic cerebrospinal fluid (CSF) were
used to make the diagnosis, but it remains only presumptive
because of hlgh incidence of traumatic lumber puncture in
premature infants.
Ultrasonography is the method of choice in
evaluating
infants for the presence of SEH-I\rH. Periventricular leuko-
malacia (PVI-) occurs in hypoxic ischemia or in IVH and is
the result of necrosis of the periventricular white matter and
damage to the corticospinal fibers of the internal capsule. PVL
is usually asymptomatic until in later infancy. Patient may
present with spastic diplegia.
Table 2.91 Clinical Presentations of SEH-lVH

Intracranial hemorrhage (ICH) occurs in 20o/o to more than

40o/o of infants with birth weights under 1500 g but less
common among more mature newborns. Bleeding within the
. Shock a Low hemaiocrit Bulglng anterior
. Pallor Hypoxemia, fontanel
skull can occur extracerebrally into epidural, subdural and o Respiratory hypercarbia & Excessive
subarachnoid spaces; into parenchyma of the cerebrum or distress respiratory acidosis somnolence
cerebellum; or into ventricles from the subependymal germinal o Disseminated Thrombocytopen ia Hypotonia
matrix or choroid plexus. i ntravascu lar & prolongation of Weakness
coagulation both PT & PTT Seizures
Categories of neonatal intracranial hemorrhage are as follows: . Jaundice Hyperbilirubinemia Temperature
instabi lity
i. Subependymal hemorrhage-intraventricular hemorrhage Brain stem
(sEH-I\'lI) signs: Apnea,
2. Posterior fossa hemorrhage lost extra-ocular
a. Cerebellar movements,
facial weakness
b. Subdural (SDH)
ESSENCE OF PEDIATRICS

Table 2.10: Crading of Subependymal-lntraventricular a Circulatory disorder: Hypotension, con gestive heart failure.
Hemorrhage a Apnea of prematurity-diagnosis by exclusion.

I Isolated SEH or to <10% of ventricles

CLASSIFICATION
ll IVH withoul ventricular dilatation with I0-50'2" filling of
venlric les Based on whether absent air flow is accompanied by continuous
lll IVH >50% involvemenl of ventricles with ventricular inspiratory efforts and upper airway obstruction.
dilalation
o Central apnea: Simultaneous cessation of respiratory effort
lV lll with parenchymal hemorrhage
and air flow at the end of expiration, probably due to ces-
sation of motor output from the respiratory center in the
brainstem.
CT scan defines the pathologic anatomy of SEH-IVH weil. o Obstructive apnea: Cessation of airflow while respiratory
Magnetic resonance imaging (MRI) is both sensitive and spe-
efFort continues.
cific in identifying SEH-IVH after the first several days of life. r Mixed apnea: Cessation of airflow with continued respira-
Grading: For grading of subependymal-intraventricular tory efFort on some occasions precedes or follows central
hemorrhages refer Thbie 2.10.
apnea. Both central and obstructive apneas occur during
the same episode.
Treolment
Apnea should be differentiated from periodic breathing:
The trearmen, oi Sp,u-ntg is supportive and is directed at
avoiding extension of the hemorrhage. No interventions have o Pause in breathing of 3 seconds or longer interrupted by
been shown conclusively to limit the extent of hemorrhage respirations for less than 20 seconds.
once it has occurred. e Normal phenomena in preterm infant.

o Avoid excessive suctioning and manipulations.

o Anemia-shock requires slow transfusion of packed blood INVESTIGATIONS
cells or FFP
Blood glucose, electroly'tes, serum calcium, sepsis screening,
Vitamin K administration may be important for those infants
blood culture, ABG CXR, USG brain.
who have evidence of abnormalities of coagulation.
Treat seizures and hyperbilirubinemia associated with the
break-down of red blood cells from the hemorrhage, and TREATMENT
acidosis with NaHCO,.
Serial LP has no role during acute hemorrhage, but reduces Algorithmic approach for the treatment of apnea has been
the symptoms of post hemorrhagic hydrocephalus. depicted in Fig. 2.4.
a Subdural tap, in subdural effusion.
Drugs to be used:
a VP shunt in hydrocephalus.
1. Aminophylline W (therapeutic blood level 5-10 pg/ml)
o Loading: 4 mg/kg, then
o I.5-3 mg/kg every 8-72 hr (<1500 g, 2 mglkg;
>1500 g, 1.5 mg/kg) or 0.7 mglkglhr
Apnea may be defined as cessation of respiration for >20 seconds 2. Cafieine fV or oral (therapeutic blood level 8-20 pg/ml)
with or without bradycardia (<100 beats/min) and cyanosis; o Loading: 20 mglkg
or for shorter periods (short apnea), if it is associated with o Then 5-8 mg/kg daily
cyanosis or bradycardia. After 30-45 seconds of apnea, pallor
and hypotonia are seen, and infant may be unresponsive to RECURRENT APNEA
tactile stimulation.

CAUSES

r Hypoxemia: Respiratory distress syndrome, pneumonia,

airwav obstruction.
a Infection: Sepsis, necrotizing enterocolitis.
a Metabolic disorder: Hypoglycemia, hypocalcemia,
hypernatremia, hyponatremia, acidosis. Mechanical ventilation
\
CNS disorder: Seizures, intracranial hemorrhage, with minimal setting .i
kernicterus. 1
1
.i
a

Other measures:
o Start antibiotic
o Manage hypoglycemia, if present
o Manage electrol;.te imbalance, if present
r Correct anemia
Check bradycardia, cyanosis, and airway obstruction
breathing, and circulation
suction (don't touch oropharynx;
avoid vigorous sucking)
100% oxygen with head box/nasal
(keep O, saturation 90-95%)
Start caffeinelAminophylline if apnea o{
prematurity (<7 days)
CPAP ) trial of Doxapram > if respond -+ continue for 48 hr
IMV/SNlPPVlHook to artificial ventilation
tig.2.4: Algorithm for treating apnea. CPAP, continuous positive
airway pressure; lMV, intermittent mandatory ventilation; SNIPPV,
synchronized nasal intermittent positive pressure ventilation.
NEONATOLOGY
Respiratory distress syndrome (RDS) is defined as respiratory
difficulty starting shortly after birth, commonly in a preterm
newborn, and is due to deficiency of pulmonary surfactant. It
occurs in 15-30% ofthose berween 32 and 36 week ofgesta-
tional age, in about 5o/o beyond 37 week and rarely ar term.
The major constituents of surfactant are dipalmitoylphos-
phatidylchoiine (lecithin), phosphatidylglycerol, apoproteins,
and cholesterol. With advancing gestational age, increasing
amount of phospholipids are synthesized and stored in type-Il
alveolar cells. These surface-active agents are released into the
alveoli to maintain alveolar stability by reducing surface rension
(prevent collapse).
Deficiency of pulmonary surfactant leads to alveolar ate-
lactasis, edema, and cell injury. Subsequendy, serum proteins
that inhibit surfactant function leak into the alveoli. Majority
of RDS/HMD are self-limiting. Microscopically, there are
eosinophilic membranes in collapsed alveoli (so is the name
HMD) and sometimes pulmonary hemorrhage and interstitial
emphysema. Factors afrecting the incidence of RDS are listed
in Thble 2.11.
CtINICAt FEATURES
The typical presentation is respiratory di{ficulty that develops
shortly after birth (usually within minures of birth), may peak
at 12-24 hr. The respiratory distress may improve in 3-5 days
or may be rapidly fatal within few hours. Expiratory grunting,
tachypnea, intercostal and subcostal recessions, and cyanosis
are the presenting symptoms (Table 2.12). Refer Thble 2.13 for
scoring system to evaluate severity of respiratory distress.
INVESTIGATIONS
Chest x-ray alone almost confirms the diagnosis. The typical
x-ray findings (develop during the first 6 hours) are low iung
volumes, a generalized haziness or reticulogranular "ground
Table 2.11: Factors Affecting the lncidence of Respiratory
Distress Syndrome
IUCR Preterm baby
Prolonged rupture of membranes Multifetal pregnancies
Maternal steroid therapy Perinatal asphyxia in preterm
Cirls Maternal diabetes. precipitous
delivery Thoracic malformation
€ ldiaphragmalic hernia.l
Llective cesarean section without labor
Erythrob lastosis
Second twin, Boys
ESSENCE OF PEDIATRICS

Table 2.12: Diagnostic Criteria for RDS-Early Signs of RDS MANAGEMENT

(After the 1st Hour of Life)*

Tachypnea r>60/min) Prevenlion

Expiratory grunting (by closure of glottis) Assessment of fetal lungs maturity before delivery by
5lernat and intercoslal recession amniotic fuid indices: L/S ratio, PG concentration.
Cyanosis in room air Maternal corticosteroid therapy: If an infant is of <34
These signs must develop before the neonate is 4-hours old and persist beyond weeks' gestation with the evidence of pulmonary immatu-
2,{ hours of age. rity (L/S < 2:7), mother should be given betamethasone or
*Diagnosis: At two of the above signs plus typical chest x-ray (refer
least dexamethasone over 48 hours. It is appropriate to administer
"l nvestigations").
steroid IM to all pregnant women who are likely to deliver
in I week berween 24 and 34 week.
; Dose:
Table 2.13: Scoring System to Evaluate Severity of Respiratory
Distress Itj.betamethasone 12 mg IM every 12 hr,2 doses;
- Or
I.j. dexamethasone 6 mg IM every 12 hr, 4 doses.
-
o First dose of surfactant (cost is high) into trachea of symp-
Respiratory
<60 60-80 >80 tomatic preterm baby immediately after birth or during the
rate/min
first 24 hour of life.
Absent with up Require >40"/o
Cyanosis Absent
to 407o oxygen oxygen
Moderate- TREATMENT
Retraclions Absent Mild
severe

Grunting Absent Audible
Breath sounds Cood Decreased
Mild 0-3; Moderate 4 6; Severe 7 10
glass" appearance of lung fields, and when severe, obscuring
heart boarders, and an air bronchogram, due to air in the major
bronchi being highlighted against rhe white opacified lung.
The typical radiological features of RDS are not evolved
from the beginning and progress according to the severity of
disease. Radiological features may improve considerably soon
after effective treaiment.
Blood: Complete blood count may help to exclude neo-
natal sepsis.
Tests of fetal lung maturity (FLM):
o Test for surfactant function: Shake test (bed side test):
Serial dilutions of ethanol are added to amniotic {luid or
gastric aspirate to allow for removal of non-surfactant foam.
The sample is then shaken for 15 seconds to permit forma-
tion of stable foam layer. Presence of bubbles in presence
of adequate surfactant that persist on the surface for 15
minutes is considered a positive test; implying a very low
risk for RDS.
o Test for surfactant biochemistry and composition: Leci-
thin/sphingomyelin (L/S) ratio testing is the "gold standard"
for FLM. An amniotic fuid L/S ratio of >2:1 is considered
equivaient to fetal lung maturity, and avalue <2:1 indicates
immaturity of fetal lung. Phosphatidylglycerol (PG) is esti-
mated in case of baby of diabetic mother.
Audible with
a Temperature must be carefuliy regulated and maintained.
stethoscope a Oxygen: The lowest concentration of humidified oxygen
Barely audible to maintain the PaO, at about 55-65 mmHg to avoid O,
toxicity.
Surfactant replacement therapy: Early administration of
exogenous surfactant via endotracheal tube to premature
infants significantly reduces severity of RDS. Surfactant is
indicated in all neonates with RDS; the route of admin-
istration is intratracheal. It can either be given as rescue
treatment in neonates or prophylactically in all neonates
<28 weeks of gestation. Even those babies who have been
given surfactants will need ventilatory support.
o Beractant (Suravanta): 4 mllkgvia endotracheal tube q 6
hours ,. 4 doses.
o Colfosceril palmitate (Exosurf): 5 ml/kg via endotracheal
tube q 12 hours x 2-4 doses.
o Follow blood pressure and cardiac function carefully. If
the baby is hypovolemic, infuse normal saline, 570 albumin,
or packed RBCs as appropriate.
o Fluid and electo$te balance should be maintained, but
relative dehydration decreases the incidence and severiry
of RDS.
o Metabolic acidosis should be corrected with bicarbonate
infusion: NaHCO" (-Eq = 0.3 x kg x base deficit) at a
rate of I mEq/kg/min.
o Antibiotic: Antibiotic coverage with ampicillin and an
aminoglycoside, or cefotaxime should be provided, since
pneumonia can mimic the clinical and radiographic appear-
ance of RDS. If the clinical and laboratorv evaluation of
infection is negative, antibiotic can be discontinued after
48-72 hou.rs.

rr

)

Intermittent mandatory ventilation (IMD is required for
severe disease; the baby with moderate disease be managed
with continuous positive airway pressure (CPAP).
Fluid and nutrition: Initially IV fluid should be given,
10olo glucose water should be infused at the rare of 55-75
mllkgl24 hr, subsequently electroly'tes should be added and
fuid volume increased gradually to 120-150 mllkgl24 hr.
In mild illness, NG tube or oral feeding can be started from
day 3 or 4, but later in severe cases.
coMPHCATTONS
Pneumothorax, bronchopulmonary dysplasia, PDA, intraven-
tricular hemorrhage, retinopathy of prematurity.
Aspiration of . meconium contaminated amniotic fluid by
a fetus or a newborn during birth is termed as meconium
aspiration.
Passage of meconium in utero rarely occurs prior to 37
weeks of gestation, but it is more common in post 42 weeks
of gestation, perinatal asphyxia, oligohydramnios, cesarean
section delivery, and in male baby. Meconium is a greenish-
black fetal intestinal content, which may pass into the amniotic
fuid and lead to hypoxia. Gasping by the fetus or newborn
infant can cause aspiration of amniotic fuid contaminated
with meconium, which by obstructing atrway interferes with
gas exchange and causes respiratory distress. Partial obstruc-
tion by particulate meconium could give rise to emphysema
ahd pneumothorax.
CtINICAL FEATURES
The newborn (often post mature) with meconium aspiration
presents with respiratory distress within the first hour, with
varying degrees oftachypnea, retraction, grunting, and cyanosis.
The baby has meconium staining of skin, nail, and umbilical
cord. The asphlxia is a finding in many cases. There may be
distended chest due to air trapping, pneumothorax, extensive
crepitation, and reduced air entry. Auscultation of the chest
reveals diffirse rales and rhonchi. The clinical symproms progress
over 12-24 hours as meconium migrates to the periphery of
the lung. Because meconium must ultimately be removed by
phagocytes, respiratory distress requirements for supplemental
oxygen may persist for days or even weeks after birth. There
may be hypotonia and seizures.
Meconium can cause chemical pne 'monitis; thick meco-
nium can give rise to atelactasis, emphysema that cen
progress to air leak syndrome, like pner -othorax, persis-
tent pulmonary hypertension, then ventilation perfusion
mismatch-respiratory failure.
NEONATOLOGY
INVESTIGATIONS
o Blood: Arterial PO, may be low, and there may be metabolic
acidosis.
o CXR shows patchy infiltrates; coarse, irregular streaking
of both lung fields, increased anreroposrerior diameter,
and flattening of diaphragm. Pneumothorax and
pneumomediastinum may be present.
DI FFERENTIAT DIAGNOSIS
Perinatal asphyxia, bacterial pneumonia, RDS, TTN, congenital
heart disease.
MANAGEMENT
Prevenlion
1. Prevention of passage of meconium in utero:
a. Prompt delivery in fetal distress.
b. Tianscervical amnioinfusion: tanscervical amnioin-
fusion with normal saline solution in cases of thick
meconium and oligohydramnios may reduce the inci-
dence of fetal distress and meconium aspiration.
2. Prevention of meconium aspiration:
a. If thick particulate (pea soup) meconium is present:
i) At delivery, the obstetrician should suction the
oropharynx before the shoulders are delivered.
ii) \Mhen received from the obstetrician: Intubation
and suction under direct laryngoscopy is
mandatory before triggering the first breath by
drying and stimulating the infant.
iii) Intubation and suction should be continued until
the meconium has been cleared.
b. If thick meconium is not present: Bulb suction of the
mouth first then nose can initiate effective respiration.
Treolmenl
r In the absence offetal distress, avigorous infant (Apgar score
>B) may not require treatment. Some may be depressed at
birth and require resuscitation. Oxygen by mask should be
administered as soon as the trachea has been cleared.
o Oropharyngeal suction should be provided to assist pul-
monary toileting.
o Broad-spectrum antibiotic should be started ifa radiological
infiltrate or documented infection is evident.
r Monitor temperarure, blood gas, IV fluid, and eiectrolyte
balance.
o Hypoxia and acidosis may lead to persistent pulmonary
hvpertension and should be treated promptly.
o Some patienrs whose clinical status continues to deterio-
rate and require escalating support may be benefited from
surfactant treatment.
ESSENCE OF PEDIATRICS
o Mechanical ventilation is indicated if PaCO, > 60 mmHg
or persistent hypoxemia (PaO, < 50 mmHg).
o Tiearment of complications.
Meconium aspiration syndrome usually improves by 72 hov;
its potential for mortality is quite high.
TTN (also known as wet lung) is a common, mild, seif-limited
disorder, usually affecting infants who are born at term or near-
term gestation, and is characterized by tachypnea, or expiratory
grunting, retractions and, occasionally, cyanosis that is relieved
by minimal oxygen (<40o/o).
The etiology of TTN is not known, but the risk factors are
premature birth, precipitous birth, operative delivery without
labor, delayed cord clamping or cord milking, macrosomia,
multiple gestation, male sex, and birth to an asthmatic morher.
The association benveen TTN and other obstetric factors such as
excessive maternal sedation, prolonged labor, and large amount
of IV fluid to mother have been less consistent.
TTN represents a transient pulmonary edema that results
from delayed clearance of fetal lung fuid. Potential causes
include conditions (delayed cord clamping or milking of the
cord) that elevate central venous pressure, leading to delayed
clearance of fluid by the thoracic duct or the pulmonary
lymphatic system. Retained fluid accumulates in the peri-
bronchiolar lymphatics and bronchovascular spaces, causing
compression and bronchiolar collapse with areas of air trapping
and hyperinflation. These changes decrease lung compliance,
leading to tachypnea.
CtINICAt FEATURES
The infant usually develops tachypnea (60-1'20 breaths per
minute) within the first 6 hours after delivery. Premature infants
may present with pulmonary edema. TTN usually has mild to
moderate tachypnea, cyanosis, slight subcostal and intercostal
retractions, increased anteroposterior diameter of the upper
thorax, nasal faring, and intermittent expiratory grunting.
These infants usually have good air exchange without crackles
or rhonchi. No other signs of cardiac, CNS, hematologic, or
metabolic diseases are demonstrable. These symptoms rypically
persist for 12-24 hours in infants with mild TTN, but may
persist for longer than 72 hours in infants with severe TTN.
INVESTIGATIONS
Analysis of arterial blood gases may show a mild respiratory
acidosis, which usually resolves within 24 hours.
o CBC t IT ratio will help ruling out an infectious process.
a X-ray chest shows prominent pulmonary vascular markings
(sunburst pattern emanating from the hilum), widened
interlobar fissures, overaeration, and fat diaphragm.
TREATMENT
Recovers rapidly (usually within 3-5 day$ without any
residual pulmonary disability. Assurance.
Minimal oxygen (<40o/o) to keep arterial oxygen saruration
>90o/o.
Feeding. Infants can be fed orally if respiratory rate is <60
breaths/min. If the rate is 60-80 breaths/min, the infant
can be fed via NG tube or IV.
:
Infections are frequent and important cause of morbidity and {
mortality in the neonatal period. About 1 in 50 preterm infants
and 1 in 1000 term infants will develop systemic infection
during the neonatal period. This increased risk occurs because
of both physical and immunological abnormalities. Types
include the following:
Superficial infections: Although infections do not generally
endanger life, and apparently trivial, in the high risk
infant can rapidly lead to systemic sepsis. These include
conjunctivitis, skin sepsis, paronychia, umbilical infection,
candida infection, oral thrush, perineal candidiasis.
Systemic infections include neonatal sepsis, meningitis,
pneumonia, urinary tracr infection, osteomyelitis/septic
arthritis, diarrhea, neonatal tetanus.
Congenital infections (STORCH) include syphilis
(congenital), toxoplasmosis, other agents (varicella zoster, HIV
hepatitis B virus, N. gononhoeae, M. tuberculosis, Chlamydia
trachomati), Rubella, cltomegalovirus, herpes simplex.
SKIN SEPSIS/PYODERMA
Pustules are commoniy seen on the scalp, neck, groin, and axilla
and are due to staphylococcal infection. The spread of infec-
tion may lead to formation of abscess, parotitis, osteomyelitis,
and sepsis. Life-threatening staphylococcal infection may lead
to manifestations of pemphigus neonatorum characterized by
marked erythema, bullous lesions, and exfoliations. Scaled skin
syndrome (futter disease) is due to staphylococcal toxin and
presents with erythema and epidermal separation on friction
(Nikolsky sign).
The isolated lesions should be punctured and sent for
Gram-staining and culture. These lesions need treatment with
topicai antibiotic. Infants who show obvious constitutional
upset should be treated with fucloxacillin with or without
gentamicin.
CONJUNCTIVITIS
Babies with conjunctivitis have purulent discharge, lid edema,
redness, and conjunctival injection. The most important patho- I
gens are N. gonorrhoeae, Chlamydia nachomatis, Staph. 4ureus t
(most common), and Pseudomonas aeruginosa. 1
't
I
t
t

EARry CONJ U NCTtVtTtS (OpHTHALMIA
NEONATORUM)
Purulent discharge from both eyes within 48 hours is frequently
due to gonococcal infection and may co-exisr with chlamydial
infection. Gram-staining of gonococcal cases can immediately
show Gram-negative intracellular diplococci. Urgent treatmenr
is required with Ceftriaxone 50 mg/kg for one dose. Eyes
should be irrigated with saline every 10-30 minures, gradually
increasing to 2 hour interval until the purulent discharge has
cleared. Alternative drug is Cefotaxime.
PURULENT STICKY EYES AFTER 48 HOURS
Bacteriological swab and chlamydial scrapings (diagnosis is
made by examining Giemsa stained epithelial cells from tarsal
conjunctiva showing intracytoplasmic inclusions) should be
taken. The eyes should be washed with saline, and local antibi-
otic (e.g., neomycin or tetracycline) ointment should be given
every 6 hour for 1 month, plus oral eryrhromycin for 14 days
should be given to suspected cases of chlamydial infection.
Staphylococcal coniunctivitis is treated with local saline
irrigation and parenteral flucloxacillin, and pseudomonas con-
junctivitis by saline irrigation and aminoglycoside.
UMBItICAt SEPSIS
Purulent discharge, redness of peri-umbilical area, and foul
smell are indicative of umbilical sepsis. Infection is most often
due to Staph. aureus and less commonly group A streptococci.
In mild cases, frequent cleansing (keeping it open and dry) and
iVo gentian violet is enough. If peri-umbilical redness is marked
or systemic infection is present, parenteral antibiotic therapy
should be started with flucloxacillin with or without gentamicin.
In chronic or neglected cases, a granuloma may develop that
should be cauterized with silver nitrate or copper sulphate crystal.
PARONYCHIA
Single paronychia can be treated with alcohol swab. If there is
multiple paronychia or evidence of local spread, baby should
be treated with systemic flucloxacillin.
CANDIDA INFECTION
Oral: The infection most commonly occurs during passage
through infected birth canal. Feeding bottles, conraminated
breast nipples, and prolonged antibiodc therapy may also result
in candidiasis. \(hite
plaques are present inside the mouth,
which are difficult to remove and may bleed when scrapped.
Treatment is with Nystatin suspension 1 ml (100,000 units)
6 hourly after feed for 2 weeks or Miconazole oral gel 1 mI 4
times daily for 5 days.
Perineal: Red satellite colonies are diagnostic. teatment is with
1olo gentian violet or Nystatin cream 4 times daily. Associated
NEONATOLOGY
dermatitis may be treated with hydrocorrisone cream. Oral
thrush, if present, should be simultaneously treated.
Neonatal sepsis is the term that has been used to describe the
systemic in{lammatory response syndrome (SIRS) resulting from
a suspected or proven infection in the first monrh of life. The
clinical spectrum of sepsis begins when a systemic infection (e.g.,
bacteremia, fungemia, viremia) or localized infection (e.g., menin-
gitis, pneumonia, pyelonephritis) progresses from sepsis to severe
sepsis (the presence of sepsis combined with organ dysfunction),
septic shock (severe sepsis plus the persistence of hypoperfusion
or hypotension for >1 hour despite adequate fuid resuscitation
or requirement for ionotropic agents or vasopressors), multiple
organ dysfunction syndrome (MODS), ultimately death.
Initial presentarion of sepsis may be limited to only one
system, but a full clinical and laboratory evaluation usually
reveals the involvement of other sysrems also. Infants with
suspected sepsis should be investigated for multi-organ system
involvement.
ctAssrFrcATtoN
Eorly-Onsel Sepsis (EOS)
It presents within the first 72 hours of life. In severe cases,
the neonate may be symptomaric at birth. Infants with EOS
usually present with respiratory distress and pneumonia. The
following risk factors seem to be associated with an increased
risk of early-onset sepsis:
o Low birth weight (<2500 grams) or prematurity.
r Febrile illness in the mother with evidence of bacterial
infection within 2 weeks prior to delivery.
o Foul smelling and/or meconium-srained liquor.
o Rupture of membranes >24 hours.
o Single unclean or >3 sterile vaginal examination(s) during
iabor.
o Prolonged labor (sum of I and 2 stage of labor >24 hr)
r Perinatal asphyxia (Apgar score <4 at 1 minute)
Presence of foul smelling liquor or three of the above men-
tioned risk factors warrant initiation of antibiotic trearmenr.
Infants with two risk factors should be investigated and rhen
treated accordingly.
Lole-Onsel Sepsis (LOS)
It usually presents after 72 hours of age. The source of infection
in LOS is either nosocomial (hospital-acquired) or commu-
niry-acquired, and neonates usually present with septicemia,
pneumonia, or meningitis. Various factors that predispose to
an increased risk of nosocomial sepsis include low birth weight,
prematurity, admission to inrensive care unit, mechanical
ventilation, invasive procedures, administration of parenteral
fluids, and use of stock solutions. Factors that might increase
ESSENCE OF PEDIATRICS

the risk of community-acquired LOS include poor hygiene, Table 2.15: The Normal Cerebrospinal Fluid Examination in
poor cord care, bottie-feeding, and prelacteal feeds. in contrast, Neonates

breast-feeding helps in prevention of infections.

Cells/cmm 8 (0-30 cells)

CLINICAL FEATURES PMN (%) 60%

CSF protein (mg/dL) 90 (20,170)
1. Non-specific features: A high index of suspicion is needed
CSF glucose (mg/dL) 52 (34-1 1 9)
for early diagnosis. Neonates with sepsis may present with
CSF/blood glucose (%) 51 (44-248')
one or more of the following symptoms and signs:
a) Hypothermia or fever (former is more common in
preterm low birth-weight infants)
b) Lethargy, poor cry.J refusal to suck be performed once the clinical condition stabilizes. Refer
c) Poor perfusion, prolonged capillarv refili time Table 2.15 for normal range of CSF components.
d) Hypotonia, absent neonatal reflexes o Radiology
e) Brady/tachycardia ,r Chest x-ray should be considered in the presence of
f) Respiratory distress, apnea, and gasping respiration respiratory distress or apnea.
g) Hypo/hyperglvcemia ,r An abdominai x-ray is indicated in the presence of
h) Metabolic acidosis abdominal signs suggestive of necrotizing enterocolitis.
2. Specific features related to various systems: Lr Neurosonogram and computed tomography (CT scan)
a) Central nervous system (CNS): Bulging anterior fon- should be performed in all patients diagnosed to have
tanelle, vacant stare, high-pitched cry, excess irritabiliry meningitis.
stupor/coma, seizures, neck retraction. Presence of these o Urine culture: Urine cuitures obtained by suprapubic Punc-
features should raise a clinical suspicion of meningitis. ture or bladder catheterization have been recommended in
b) Cardiac: Hypotension, poor perfusion, shock' all cases of LOS. Since the procedures are painful and the
c) Gastrointestinal: Feed intolerance, vomiting, diar- yield is often pooq urine culture is not recommended in
rhea, abdominal distension, paralytic ileus, necrotizing neonates with sepsis.
enterocolitis.
d) Hepatic Hepatomegaly, direct hyperbilirubinemia
(especially with urinary tract infections).
MANAGEMENT
e) Renal: Acute renal failure.
Supporlive
f) Hematological: Bleeding, petechiae, purpura.
s) Skin changes: Multiple pustules, abscess,
sclerema, o Tleat hypothermia by maintaining optimum room tem-
mottling, umbilical redness and discharge. perature (>25" C), by wrapping, warming in the radiant
warmer or in the incubator.
a Hypoglycemia should be treated with 10% dextrose.
INVESTIGATIONS a If hemodynamically unstable, IV fluid should be admin-

o istered.
Blood culture
r Septic screen: A practical sePtic screening is given in Volume expander 10-20 ml/kg (normal saline, albumin'
Table 2.14. blood) should be used in shock along with dopamine or
o Lumbar puncture! Lumbar puncture (LP) should be done dobutamine where needed.
in all infants prior to starting antibiotics. Lumbar Puncture In case of DIC, fresh frozen plasma (FFP) 10 mi/kg, vitamin
could be postponed in a critically sick neonate. It should K, platelet infusion, and possible exchange transfusion
should be done.
Assess hvpoxia bv pulse oximetrv and initiate oq/gen therapy
'{able 2.14: Components of Septic Screening and their Abnor- or Yentilator slrpport rvhen needed.
rnal Values a Control seizure u.ith appropriate medication.
a Monitor for SIADH, i.e., urine output, hyponarremia,
Total leukocyte count <5000/cmm serum osnoialiry and urine osmolality
Treat metabolic acidosis with bicarbonate and lluid replace-
nbsolute neutrophil count Low counts
ment
lmmature/total neutrophil (l :T) >0.2
IV nutrition for very sick babies, NG tube feeding and
\4icr o-LSR > l5 mm rn lst nour
breast-feeding (when needed).
t
{--reactive protein (CRP) >1 mg/dl Care of the umbilicus.
I
I
I
 NEONATOLOGY

Anlimicrobiol Theropy Adjunctive therapy:

Indications in neonates at risk of EOS: The indications for o Exchange transfusion: Double volume exchange transfu-
starting antibiotics in neonates at risk of EOS include any sion in septic neonates with sclerema demonstrated a 50o/o
one of the following: reduction in sepsis related mortality in the treated group.
o o Intravenous immunoglobulin (IVIG): Non-specific pooled
Presence of >3 risk factors for early-onset sepsis
IVIG has not been found to be useful.
o Presence of foul-smelling liquor
r Granulocyte-macrophage colony stimulating factor
r Presence of rwo antenatal risk factor(s) and a positive septic
(GM-CSF): Still experimental.
screen
r Strong clinical suspicion of sepsis

Indications for starting antibiotics in LOS:

o Positive septic screen and/or
o Strong clinical suspicion of sepsis Congenital infections are defined as infections that are trans-
mitted vertically from the mother to the fetus in utero.
Refer Tables 2.15 and 2.17 for empirical choice of antibiotics
Perinatal infections are defined as infections that are
and duration of antibiotic therapy, respectively, in the treat-
acquired intrapartum or immediate postpartum period.
ment oF neonaral sepsis.
Classically, congenital infections are known by acronym
Reserve antibiotics: STORCH (S - syphilis, T - toxoplasma, O - others [varicella
o Aztreonam has excellent activity against gram-negative
zoster, hepatitis-B, enterovirus, E coh, malaria, etc.], R - rubella,
C - cytomegalovirus, H - herpes simplex virus).
organisms.
o Meropenem is effective against most bacterial pathogens, Mode of infection of the fetus:
except methicillin-resistant Staphylococcus dureus (MRSA) o Hematogenous through the piacenta.
and Enterococcus. o Direct infection of the placenta.
o Imipenem is generally avoided in neonates, because of the o Ascending infection from the vagina, resulting in amni-
reported increase in the incidence of seizures following its use. onitis.
o Empirical use of these antibiotics should be avoided. o During passage through the birth canal.
o tansmission through the breast rnilk.
Table2.16: Empirical Choice of Antibiotics for Treatment of
Neonatal Sepsis
CONGENITAT SYPHILIS
TTeponema pallidum is the etiological agent, and it results from
First line transplacental transmission of spirochetes. Pregnant women
Penicillin or
Commun ity-acqu ired with primary and secondary syphilis and spirochetemia are
Ampicillin and Add Cefotaxime
(resistant strains are
Centamicin more likely to transmit the infection. tansmission can occur
unlikely)
at any stage of pregnancy.
Second line Ampicillin or
Hospital-acqu ired Cloxacillin and
Add Cefotaxime
(some strains are Centamicin or Clinicol Feqlures
likely to be resistant) Amikacin
Syphilis during pregnancy has a transmission rate approach-
Third line Cefotaxime or
ing 100%. Fetal or perinatai death occurs in 40o/o oi affected
Hospita l-acqu ired Piperaci I I in-
Same (avoid infants. Among survivors, early manifestations develop during
sepsis (Most Tazobactam or
Ciprofloxacin)
strains are likelv to Ciprofloxacin and the first 2 years of life, while late manifestations develop gradu-
be resistant) Amikacin ally during the first rwo decades.
o Early manifestations:
Table 2.17: Duration of Antibiotic Therapy in Neonatal Sepsis
> Mucocutaneous manifestations: A bullous rash may be
present at birth. A pink maculopapular rash that dark-
Meningitis (with or without positive blood and ens and desquamates in course of time may also be pres-
21 da,vs
CSF culture)
ent. Flat, \\'art-like moist condylomata is also seen. Most
Blood culture positive, but no meningitis 1 4 days infants with congenital syphilis present with rhinitis or
Culture negative, sepsis screen positive, and clinical snuffies.
7 10days
course consislent with sepsis > Hepatosplenomegaly, jaundice.
Culture and sepsis screen negative, but clinical
5-7 days r Lymphadenopathy tends to diffuse and resolves sponta-
course ( ompatible wi[h sepsis
neously, shotry nodes may persist.
ESSENCE OF PEDIATRICS

Hematologic manifestations viz., Coombs negative lower extremities (usually the knee), which presents as
hemoll'tic anemia, bleeding diatheses, thrombocytopenia, painless joint swelling with sterile synovial fluid.
and erythroblastemia are not unusual.
Skeletal changes: Characteristic roentgenographic ab- Diognosis
normalities include multiple sites of osteochondritis (at
Based on (z) clinical evaluation, (ll) epidemiological consider-
the wrists, elbows, ankles, and knees) and periostitis of
ations, (iii)
examination of placenta, and (iu) serological tests
the long bones, and rarely the skull. The osteochondritis
in the mother and the infant.
is painful and often results in irritabiliry and refusal to
move the involved extremity (pseudoparalysis of Parrot). o Mother: VDRL and fuorescent treponemal antibody
c Renal lesions: Nephritis or nephrotic syndrome may be absorption (FTA-ABS) tests. Positive tests in mother are
present at birth or may appear within I month. They ap- usually associated with positive test in newborn infant.
pear to be related to glomerular deposition of circulatory o Infant: VDRL, FTA-ABS IgM, CSF VDRL, CSF for
immune complexes. antibody titer. Examination of nasal discharge for DGI.
Central nervous system: There may be acute syphilitic If VDRL and FTA-ABS IgM tests are positive, congenital
leptomeningitis, hydrocephalus, and cranial nerve pal- infection should be suspected strongly.
sies.
O Eye involvement includes glaucoma and chorioretinitis. Treolmenl
O Failure to thrive and intrauterine growth retardation are
constant features. The birth weight is low and the infant
1. Current recommendations for treatment of congenital syph-
ilis include IV crystalline penicillin G 100,000-150,000
presents a wizened ippearance.
Ulkgld given as 50,000 U/kg every 12 hr for the first
Late manifestations: These result primarily from chronic 7 days and every 8 hr thereafter for 10-14 days; or
inflammation of bone, teeth, and CNS. 2. Procaine penicillin G 50,000 U/kg IM daily in a single
o Skeletal changes due to persistent or recurrent periostitis dose for 10-14 davs.
and associated thickening of bone include frontal boss-
ing, a bony prominence of forehead ("olympian brow"), Follow-up
unilateral or bilateral thickening of sternoclavicular por-
These children should be kept under surveillance for a year.
tion of clavicle (Higoumenakis sign), and anterior bow-
Serological tests for syphilis are repeated 4-6 weeks after the
ing of the mid-portion of the tibia (Saber shins) and
therapy.
scaphoid scapula, a convexity along its medial border.
o Dental abnormalities are common and inciude Hutchin-
son teeth, that erupt during the sixth year oflife; abnor- TOXOPTASMOSIS
mal enamel, which results in a notch along the biting
surfaces; mulberry molars, abnormal first lower molars
Tbxoplasma gondii, an obligate intracellular protozoa, causes
toxopiasmosis ofwhich cat is the definitive host. \7hen a mother
characterized by small biting surface and as excessive
acquires the infection during gestation, the organism may
number of cusps. Defects in enamel formation lead to
disseminate hematogenously to the placenta and transmitted
repeated carries and eventual tooth destruction.
o A saddle nose, a depression of nasal root, is a result of to the fetus or may be transmitted during vaginal delivery. In
first trimester, I7o/o of fetuses are infected, usually with severe
syphilitic rhinitis that destroys the adjacent bone and car-
disease; in third trimester 650/o of fetuses are infected, usually
tilage. Painless perforation ofnasal septum also occurs.
c Rhagades, which are linear scars that extend in a spoke-
with disease that is mild or apparent at birth.
like pattern from previous mucocutaneous fissures of the
mouth, anus, and genitalia. Clinicol Feolures
o CNS involvement includes juvenile paresis, rypically o CNS manifestations: Microcephaly, hydrocephaly,
present during adolescence with behavioral changes, convulsions, psychomotor retardation, hypotonia, intracra-
focal seizures, or loss ofintellectual function. Juvenile ta- nial calcification, meningoencephalitis.
bes with spinal cord involvement are rare. o Ocular manifestations: Microphthalmia, strabismus,
o Late hypersensitivity phenomenon includes unilateral chorioretinitis.
or bilateral interstitiai keratitis foilowed by corneal Cuteneous manifestations: Maculopapular rashes, petechiae,
opacification and complete blindness; choroiditis, ecchymoses or large hemorrhages, exfoliative dermatitis,
retinitis, vascular occlusion, and optic atrophy also persistent jaundice. \
occur. Eighth nerve deafness may be unilateral or 1
Systemic signs: Prematuriry/iUGR, remperature instability, 1
bilateral, presents initially as vertigo and hearing loss hepatosplenomegaly, myocarditis, pneumonitis, nephrotic
joint
I
and progresses to permanent deafness. The Clutton syndrome, diarrhea, erythroblastosis fetalis (Coombs a
represents a unilateral or bilateral synovitis involving the negative). t
t
t
I

o Endocrine abnormalities: Secondary to hypothalamic or
pituitary involvement, myxedema, diabetes insipidus, and
sexual precocity.
lnvesiigolions
o Complete blood count: Thrombocytopenia.
o CSF analysis: Cells, glucose, protein, toxoplasma-specific
IgG and IgM antibodies, total amount of IgG, evaluation
for T gondii by PCR.
o Serology: Most useful for diagnosis. Either persistent or
rising titers in the dye (Sabin-Felbman dye test) or IFA
(indirect fluorescent antibody test) or a positive IgM ELISA
is diagnostic of congenital toxoplasmosis.
o Isolation of T, gondiir Placenta, infant's white blood cells
from umbilical cord blood and bufr. coat.
r X-ray skull: Intracranial calcification, especially of basal ganglia.
o CT scan of brain.
Prenolql Diognosis
Fetal ultrasound examinations performed every 2 week during
gestation and PCR analysis of amniotic fluid are used for
prenatal diagnosis.
Treolmenl
o AII infected newborns should be treated, whether or not
they have clinical manifestations of the infections.
o Infants should be treated for 1 year with oral pyrimethamine
(I-2 mglkgld for 2 days, then 1 mg/kgi d for 2 months or
6 months, then 1 mglkgld on Monday, \Tednesday, and
Friday), plus
o Sulfadiazine or triple sulfonamides 100 mg/kg/d (loading
dose), then 100 mg/kg/d in 2 divided doses, plus
o Calcium leucovorin (5-10 mg/kg/d) on Monday, \Tednes-
day, and Friday.
o Folinic acid 10 mg thrice in a week should be given to
prevent hematologic toxicity.
o Prednisolone (1 mg/kg/d orally in divided doses) has been
utilized in addition when active chorioretinitis involves the
macula or the CSF protein is >1000 mg/dl at birth.
Prevenlion
r Avoid consumption of raw and undercooked meat products
by pregnant mothers.
o Avoid handling of the soil potentially contaminated with
cat feces.
o Identify women at risk by serologic testing.
o teatment of acutely infected mother during pregnancy by
Spiramycin.
CYTOMEGATIC INCTUSION DISEASE
Cytomegalovirus (CMD is the most common congenital
infection that occasionally causes the syndrome of C;,tomegalic
NEONATOLOGY
Inclusion Disease (hepatosplenomegaly, jaundice, petechiae,
purpura, and microcephaly). The fetus may become infected as
a consequence of primary (40o/o) and recurrent (<1%) maternal
infection. Perinatal rransmission is common (10--60%) via
genital tract secretion at delivery and breast milk. Infected
infants excrete virus for vears in saliva and urine.
Clinicol Feolures
Only 5o/o of all congenitally infected infants have severe cyto-
megalic inclusion disease, another 5o/o have mild involvement
and 90o/o are born with subclinical but chronic CMV infection.
o CNS: Microcephaiy, meningoencephalitis, intracranial cal-
cification (usually periventricular), sensory neural hearing
loss, psychomotor retardation, and convulsions.
r Ocular: Chorioretinitis, retinopathy.
o Others: Intrauterine growth retardation, prematuriry hepa-
tosplenomegaly, jaundice, thrombocytopenia, purpura,
hemolytic anemia, pneumonitis, and rashes.
lnvesligotiorii
o Complete blood count: Reduced Hb%, thrombocltopenia,
features of hemolysis, arypical lymphocytosis.
o X-rayskull: Intracranial calcification, especially periventricular.
o CSF analysis: Protein more than 120 mg/dl.
o Isolation ofvirus by urine and saliva culture or demonstra-
tion of specific DNA sequence by PCR.
o Liver function tests
r CT scan or MRI of brain
o Hearing test
Prenolql Diognosis
Isolation of CMV from amniotic fluid.
Treolmenl
r Infected infant should be isolated.
o A non-infected infant should not be given breast milk of a
mother with CMV infection.
o There is no specific treatment for congenital or perinatal
CMV infection. Ganciclovir 12 mglkgld for a total of 6
weeks showed hearing improvement in infants.
Prognosis
The prognosis for normal development with symptomatic c1.to-
megalic inciusion disease is poor; more than 90% children
develop CNS and hearing defects in later years. In infants with
subclinical infections, sensorineural hearing loss (5-l0olo), chorio- I
retinitis (3-5o/o), microcephaly, and neurologic defects may occur.
I
I
CoNGENTTAL RUBETH ryN_DROI!,IF I
Infection with rubella virus is transmitted from the mother I
to the fetus. Virulence of the fetal infection depends upon I
il
ESSENCE OF PEDIATRICS

the gestational age at the time of transmission of maternal
infection. Rubella infection (German measles) usually results
in a mild illness in adult and children but can have serious
consequences when fetus is infected.
The risk for congenital defects and disease is greatest with
primary maternal infection during the first rrimester. Congenital
defects occur in about 90%o of infants whose mothers acquire
maternal infection before the I I th week of pregnancy, dimin-
ishing to about 10-20o/oby the end of the first trimester, with
an overall risk for the trimester being about 70o/o. Maternal
infection after the 16th week of pregnancy poses a low risk for
congenital defects, although infection of the fetus may occur.
Clinicol Feotures
PERINATAL HERPES SIMPTEX VIRUS
INFECTION
Most cases of neonatal herpes occur due to infection during
delivery, and75-B0Vo are HSV type-2. About 10% of infants
acquire their infection postpartum, nor necessarily from the
mother, but usually from another close family member shed-
ding HSV (often type-1) from fever blisters, finger infections,
or lesions at other sites.
Clinicol Feqlures
Infection manifests in the first monrh of life with 25o/o on the
first day, and in two-thirds by the first week.

Classically, congenital rubella syndrome is characterized by

the constellation of 2 11i2d-6212ract, sensorineural hearing
loss, and congenital heart disease. Congenital rubella affects
virtually all organ sysrems:
r CNS: Microcephaly, mental retardation, sensorineural deaf-
ness, meningoencephalitis, convulsions, hypotonia.
o Ocular: Microphthalmia; nuclear cataract; bilatera"l or uni-
lateral retinopathy and glaucoma.
o CVS: Patent ductus arteriosus (PDA), pulmonary artery
stenosis, ventricular septal defect (VSD), myocarditis.
o Skeletal: Micrognathia, bone lucencies.
r GIT Esophageal atresia, jejunal atresia, hepatitis.
o Hemopoietic: Anemia, leucopenia, thrombocytopenia,
lymphadenopathy.
o Respiratory: Interstitial pneumonitis, respiratory distress.
o Others: IUGR/LBW and postnatal growth retardation ,
blue berry muffin skin lesions, polycystic kidneys, diabetes
mellitus, thyroid dysfunctions, dental abnormalities, speech
disorders.
o Skin: Vesiculag ulcerative lesions (hallmark).
o CNS: Lethargy, poor feeding, seizures, meningoencepha-
litis.
r Eyes: Keratoconjunctivitis, microphthalmia.
e Others: Fever or hypothermia, hepatitis, pneumonitis,
myocarditis, DIC.
Invesligotions
o Complete blood count: Thrombocytopenia.
o Liver function tests: Raised rransaminases.
o Coagulation defect
o Lesion scraping microscopic examination (Tzanck stain)
reveals multinuclear giant cells and intracellular inclusions.
o Antigen detection by ELISA and immunofluorescence
technique.
o CSF analysis: Moderate pleocytosis with elevated protein
level.
o Virus isolation

Diognosis
lnvesligolions
The diagnosis is based on any rwo of the following:
o Complete blood count: Anemia, thrombocytopenia.
r Serum: SGPT increased. o Compatible clinical partern.
o Serolog;,': A positive rubella-specific IgM antibody or a rise o Isolation of the virus.
in paired IgG titers is indicarive of recent infection. o Development of specific antibodies.
o X-ray: Bone lucencies. o Demonstration of characteristic cells; histological changes;
o Virus isolation: Rubella virus can be cultured from naso- and viral antigen or HSV DNA in scrapings, CSF, or
pharynx and blood. biopsy material.

Treotmenl Treolment
There is no specific antiviral therapy; treatmenr is entirely o Intravenous acyclovir 60 mg/kg/d in three divided doses
supportive, such as administration of blood transfusion for for 14-27 days is the drug of choice.
anemia or active bleeding, seizure control, and phototherapy for o Initially, intensive care is necessary providing ventilatory
hyperbilirubinemia. Long-term care requires a multidisciplinary support, seizure management, and other supportive care.
approach consisting of occupationai physical therapy, close
neurologic and audiologic monitoring, and surgical interven- l
tions as needed for cardiac malformations and cataracts. Proven
Prevenlion \
a
rubella infection before 13 weeks gestation is an indication for A woman with cervical HSV should be delivered by cesarean rl
termination of pregnancy. section to prevent transmission of infection to the baby. ri
t<l
t

VARICELIA ZOSTER VIRUS INFECTION
Also known as congenital varicella syndrome. Vhen pregnant
women contract chicken pox, about 25o/o of the fetuses may
become infected, although not every infected fetus is clinically
affected. lJp to 2o/o of fetuses whose mothers had varicella
benveen 8th and 20th week of pregnancy may demonstrate
varicella zoster virus (VZV) embryopathy.
Stigmata of varicella zoster virus fetopathy:
o Damage to sensory neraes: Cicatricial skin lesions, hypopig-
mentation.
o Damage of optic stalh and lens oesicle: Microphthalmia,
cataracts, chorioretinitis, optic atrophy.
o Damage to brain/ enceqt balitis : Microcephaly, hydrocepha-
lus, calcifications, aplasia of brain.
o Dam.age to ceraical or lumbosacral cord: Hypoplasia of an
extremiry motor and sensory deficits, absent deep tendon
reflexes, Horner syndrome, anai/urinary sphincter dysfunction.
Diognosis
The diagnosis of VZV fetopathy is based mainly on the history
of gestational chickenpox combined with the stigmata seen in
the fetus. Virus cannot be cultured from the affected newborns,
but viral DNA can be detected in tissue samples by PCR.
Demonstration of VZV-sepcific IgM antibody in cord blood
sample. Chorionic villus sampling may also be done to detect
viral DNA or viruses.
Treotmenl
Antiviral treatment is not indicated.
NEONATAL CHICKENPOX
Delivery within 1 week before or after the onset of maternal
varicella frequently results in the newborn developing varicella,
which may be severe. The initial infection is intrauterine, although
the newborn ofien develops clinical chickenpox postpartum.
Treqlmenl
Newborns whose mothers develop varicella 5 days before
to 2 days after delivery should receive 1 vial varicella zoster
immunoglobulin (VZ Ig).
Every premature infant born to a mother with active chicken-
pox at delivery (even if present >1 wk) should receive YZIg.
Perinatally acquired varicella may be life-threatening and
should be treated with acyclovir 10 mg/kg/dose 8 hourlv
IV for 10-14 days.
Neonatal tetanus (by Cl tetani) follows contamination of the
umbilical stump following cutting of the cord with an unclean
NEONATOLOGY
unsterilized blade, knife, or scissor; tying with unsterilized
thread; or application of cow dung etc. on umbilical stumP.
The common age at the onset of symPtoms is 5-15 days.
Excessive unexplained crying followed by refusal of feeds and
apathy (remained mentally clear) are initial symptoms. There is
dysphagia. Lockjaw is followed by spasms of the limbs. There
is generalized rigidiry and opisthotonus. Episodes of apnea
and cyanosis are attributed to spasm of larynx and respira-
tory muscles. Spasms are characteristically induced by stimuli
of touch, noise, and bright light. Umbilical stump may show
evidence of sepsis. Intercurrent infections (aspiration pneumo-
nia), dehydration, cardiovascular problems, and renal failure
may complicate the clinical features.
TREATMENT
r General measures: The infant should be nursed in a quiet
room; noise and light should be avoided. Handling should
be reduced to the barest minimum. IM injections must
be avoided. Temperature should be controlled. Oral secre-
tions should be sucked periodically. Thke care of inflamed
umbilical srump.
r IV infusion: An intravenous line should be established
for providing {luid (with caution, because inappropriate
secretion of ADH may occur), calories, electrolytes, and
medication. Nter 3-4 days of IV treatment' feeding through
nasogastric tube may be started.
o Antitoxin serum: It neutralizes the circulating toxins, but
it has no role in dlslodging the toxin already fixed to the
nerve roots. Tetanus immunoglobulin (TIG) of human
origin 500 units should be given IM; it is preferred over
tetanus antitoxic horse serum (ATS), which is often given
in a single dose of 10,000 units IV.
o Control of tetanic spasm: Diazepam 0.2-0.5 mg/kg/dose
or more should be given IV 4 hourly round the clock or by
continuous infusion. PR diazepam is also found effective. Phe-
nobarbitone may be given to increase the threshold of seizure.
o Antibiotic: IV penicillin or metronidazole.
o Thacheostomy and assisted ventilation may be needed
in severe cases when the infant gets frequent episodes of
laryngeal spasms, apneic attacks with respiratory failure.
PREVENTION
o Neonatal tetanus can be prevented by two injections of
tetanus toxoid 1 month apart to non-immune pregnant
woman,
o To provide education to local 'dah' (TBA) to use sterilized
instruments and thread for cutting and tying the cord.
A seizure is defined clinically paroxysmal alteration in neuro-
as a
logical function, i.e., motor, behavior and/or autonomic function.
ESSENCE OF PEDIATRICS

ctAsstFtcATtoN
DAY OF ONSET
a) Subtle seizures: Occurs both in term and prererm infants.
Subtle means the clinical manifestations are mild and Day 0-3 may be related to perinatal asphlxia, intracranial
frequently missed. They constitute 50o/o of all seizures. hemorrhage, metabolic and developmental defects.
Examples: Day 4-7 may be due to sepsis, meningitis, metabolic causes,
and developmental defects.
1. Ocular: Tonic horizontal deviation of eyes or sustained
eye opening with ocular fixation or cycled fluttering.
2. Oral-facial-lingual movemenrs (chewing, rongue INVESTIGATIONS
thrusting, lip smacking)
3. Limb movements (cycling, paddling, boxing-jabs) Blood sugar; hematocrit; serum elecrrolltes (Na, Ca, Mg); arte,
4. Autonomic phenomena (tachycardia or bradycardia) rial blood gas; anion gap; cerebrospinal fuid (CSF) examination
5. Apnea with accelerated or normal heart rate when (if indicated); ultrasound (USG) examination of the head; and
evaluated 20 seconds after onset. electroencephalography (EEG).
b) Clonic seizures: Characterized by rhlthmic movements of
muscle groups, most commonly associated with abnormal TREATMENT
EEG changes, seen primarily in term babies.
c) Tonic seizures: They are seen in preterm infants. They Steps involved in the acute management of neonatal seizure
are sustained flexion or extension ofaxial or appendicular are depicted in Fig. 2.5.The causes should be identified and
muscle groups, may be focal or generalized. EEG changes treated accordingly.
in generalized tonic seizures are usually absent.
d) Myoclonic seizures: They are single or multiple jerks of
Doses
the upper or lower limbs. EEG changes include burst
suppression pattern, focal sharp waves, and hypsarrhythmia. r Inj. Magnesium sulfate (I"j. G Magsulph 2.47 gl5 ml)
o Inj. Phenobarbitone (Inj. Barbit/Bardinal 200 mg/ml)
o Inj. Fosphenytoin (Inj. Fosfen 150 mgl2 ml)
CAUSES Pyridoxine related 5si2u165-x therapeutic trial of IV 50-100
mg of pyridoxine followed by remission of seizures and
1. Hypoxic-ischemic encephalopathy
normalization of EEG (Inj. Nervin - vit Bu 100 mg amp),
o Commonesr cause (50-650/o of all seizures) maintenance 15 mg/kg.
r Most seizures (50-650/o) due to HIE start within 12 Inj. Midazolam can be started with an infusion rate of 100
hours, remaining have an onser within 24-48 hours. pg/kg/hr and can be increased 3-4 hourly to a maximum of
2. Metabolic causes 1000 pg/kg/hr ifit had been used to control drug-resistant
o Hypoglycemia (blood glucose level <2.5 mmol/L) neonatal seizure. (Inj. Hipnofast 5 mg/5 ml).
r Hypocalcemia (serum calcium level <1.75 mmol/L) Folinic acid deficiency (a course of Folinic acid 2.5 mg
o Hypomagnesemia (Mg level <1.5 mEq/L or <0.70 twice a day, increasing the dose up to 8 mglkgld may be
mmol/L) required). (Inj. calcium leucovorin 3 mglml or 25 mg/ml.
o Hyponatremia, hypernatremia, and rarely pyridoxine Thblet 5 mg or 25 mg.)
deficiency o Lidocaine drip (Inj. Jasocaine 2o/o 120 mg/l mll 50 mg in
each vial) can be started with a loading dose of 2 mglkg
3. Infections
given over 20 minutes followed by 4-6 mg/kg/hr in continu-
o Meningitis (E. coli, Klebsiella, Proteus, Listeria) ous drips. Adverse effects are arrhythmia, hypotension, and
r Viral encephalitis (Herpes simplex, enterovirus) seizure. It should not be administered with Phenvtoin.
r Secondary to inrrauterine infections (TORCH
infection, HIV syphllis)
4. Intracranial hemorrhage Moinlenqnce Theropy :
o Subarachnoid, intraparenchymal, or subdural hemor- o The maintenance is begun 12 hours after the loading dose.
rhage-more in term babies o Phenobarbitone (5 mg/kg/d) can be given IV/IM or per
o Intraventricular hemorrhage (I\GI) occurs in preterms, oral in two divided doses (12 hourly).
benveen 2 and7 days
5. Cerebral infarcts: Both arterial and venous strokes Durolion of Theropy
1
6. Developmental brain defects: Cerebral dysgenesis and o Optimal duration of therapy is determined by underlying t
neuronal migration disorders are rare causes of seizures etiology, recurrence rate, neurological status, and EEG a
in the neonatal period. t
changes.
t
tt
t
 !

NEONATOLOGY

vCorrect
Table 2.18: Rough Cuide to Serum Bilirubin Levels with
Hypoglycemia or
Respet t lo Dermal Slaining
Hypocalcemia or lnvestigation for Face 4-6 mg/dl
hypomagnesemia, specific causes and
clinical features. Chest and upper abdomen B-10 mg/dl
if preseni or
suspected. Lower abdomen and thigh 12-14 mg/cJl
Arms and lower legs I 5 IB mgdl
Palm and sole I 5-20 mgdl

-Consider
ffi

Midazolam/ ffi
physiological photophobia. Eyes and sclera are best examined
ylV PHB 20 mg/kg Lidocaine by holding an infant against diffuse light and without trying
slowly over infusion ffi

ffi
to open the eyelids forcibly. The clinical jaundice manifests
20 min or e@Gffi#ffietry
T on the face (since it is the thinnest part) at a serum bilirubin
1 mglkg/min I
t
level of 5 mg/dl (1 mg/dl = 17 pmollL) (Table 2.18). It is
Seizure ffi
essential that a1l newborn babies must be clinically screened
continues #
rwice a day in good day light to detect the onset and severity
Seizure continues ssryffi
ofjaundice. In a large majoriry jaundice is however physiologic
?
I
!
and is sel[-limitirrg.
*Exclude ffi
Repeat PHB twice Pyridoxine/ ffi CAUSES
1Omg/kg/dose at an Folinic acid ffi

interval of 10 min to Start responsrve g Common causes of neonatal jaundice include the following:
reach a maxirnum Fosphenytoin seizures
of 40 mg/kg. 30mg/kg/dose -******ry ffi
o Physiological
(lV/lM) diluted I o Pathological
in normal I

saline @ 1 Seizure 'r Infections: Sepsis, TORCH

$
Seizure continues mg/kg/min continues ffi
r Hemolytic disease of the newborn: R1l, ABO incompat-
*@w@
rbrlrfv/
v Start lV Phenobarbitone (PHB); simultaneously exclude
r Extravasation: Cephalhematoma, subaponeurotic hem-
metabolic cause (hypoglycemia or Hypocalcemia). orrhage
. Decision for exclusion of Pyridoxine or Folinic acid
,, Cong hypothyroidism
responsive seizure and initiation of Midazolam/Lidocaine r Neonatal hepatitis syndrome, biliary atresia, HS
should proceed simultaneously. Differentiating features of physiological and pathological
jaundice:
Fig. 2.5: Acute management of neonatal seizure.

. Jaundice appears on 2nd day Jaundice starts on day 1

The good practice is to stop anticonvulsant as early as pos- . Bilirubin level rises slowly a Jaundice lasts Ionger than
sible for about within 2 weeks or before discharge.
. Level rarely goes above 5
.l
14 days in term & 21 days in
mg/dl preterm
The common practice is to withdraw anticonvulsants before . Baby remains otherwise A rise in serum bilirubin Ievel
discharge home if the baby is neurologically normal on healthy over 0.5 mg/dl/hr or I0 mg/
examination. o Jaundice clears spontaneously dl/d
If baby is abnorn-ral (clinical and EEG) at the time of within 7-1 0 days of life laundir e r.r ilh signs of cep'is
iaundice extends up to palms
discharge, PB should continue with re-evaluation at 6-12 & soles
weeks interval. Jaundice with pale stool

Based on the time of onset or duration, iaundice can be cat-

egorized as (i) earl,v, (il) prolonged, and (lli) persistent jaundice;
their respective causes have been listed below:
Over two-thirds of newborn babies develop clinical jaundice,
and by adult standards almost all newborn babies are jaundiced l. EarlyJaundice (\J(rithin l0 Days of Age)
during eally days of life. Yellow discoloration is first evident a. Within first 2 days: Hemolytic disease of the newborn
on rhe skin of face, nasolabial folds, and tip of the nose. The (HDN), Rh-incompatibiliry G6PD deficiency, con-
vellow staining of the sclera is difficult to evaluate because of genital spherocyrosis. sepsis.
ESSENCE OF PEDIATRICS
b. Vithin 3-10 days: Physiological jaundice, prematuriry
hypoglycemia, acidosis, sepsis, congenital hemolytic
anemia, Crigler-Najjar and Giibert syndrome, galac-
tosem ia. drugs.
2. ProlongedJaundice (at >10 Days of Age)
a. Prolonged unconjugated hyperbilirubinemia: Breast
milk jaundice, sepsis, hypothyroidism, galactosemia,
infantiie pyloric stenosis, persisting hemolysis, Rh-
incompatibility, G6PD deficiency, congenital hemo-
lytic anemia, drugs.
b. Prolonged conjugated hyperbilirubinemia
i). Intrahepatic cholestasis: Infections (septicemia,
UTI, hepatitis, syphilis); galactosemia, alpha
1-antitrypsin deficiency, MPS, Gaucher disease;
hypothyroidism; idiopathic neonatal hepatitis,
drugs.
ii). Extrahepatic cholestasis: Biliary atresia,
choledochal cyst.
3. Persistent Jaundice
a. Unconjugated hyperbilirubinemia
i) Breast milk jaundice
ii) Hypothyroidism
iii) Crigler-Najjar syndrome
iv) Intestinal obstruction or stasis
v) Ongoing hemolysis (Rh, ABO)
b. Conjugated hyperbilirubinemia (cholestasis)
i) Neonatal hepatitis
ii) Extrahepatic biliary atresia
iii) Inborn errors of metabolism
iv) Total parenteral nutrition
TREATMENT OPTIONS
o Breasr feeding
o Phototherapy
o Exchange transfusion.
o Treatment of underlying causes
PHOTOTHERAPY
Phototherapy or exposure to light is known to cause photo-
isomerization of bilirubin (bilirubin absorbs light maximally
at 420-470 nm) to more polar, water-soluble, harmless com-
pounds that are hardly excreted in tl-re bile, feces, and r-rrine.
Phototherapy units with blue or white tubes or haloger.r lamps
are useful for preventing rapid rise in serum bilirubin levels.
o The naked infant is exposed under phototherapy unit, which
is kept at a distance of about 45 cm from the baby's skin.
o During exposure to light, the eyes must be effectively
shielded ro prevenr retinal damage.
o The position of the infant should be changed frequently so
that maximum skin is exposed to light. The infant is kept
under the light round-the-clock and taken out only for
feeding or changing wet napkins.
Most preterm babies are placed under phototherapv when
their serum bilirubin approaches to 10-12 mg/dl, and tern.r
babies are given phototherapy when their serum bilirubin
approaches to 15 mg/dl.
During phototherapy, the infant should be closely watched
for hydration status, temperature, degree of jaundice, and
anemia. Phototherapy is by and large safe but may produce
loose greenish stools, dehydration, hypothermia, hyper-
thermia, and skin rash. During phototherapy, the clinical
evaluation of the severity of jaundice becomes unreliable,
because the infantt skin gers bleached under light.
24-28 hour exposure is generally long enough to bring
down serum bilirubin level to safe limit.
When lo Slop Phototheropy
o Risk factors are gone
o Bilirubin level <13 mg/dl in rerm and <10 mg/dl in preterm
o Discharge need not be delayed to observe rebound
o Repeat TSB measurement or clinical follow-up 24 hours
after discharge is a clinical oprion
Side Effecls
lmmediate:
Passage of loose green stools, because of transient lactose
intolerance and irritant effect of photocatabolites on intes-
tinal mucosa.
a Dehydration (mild) due to increased insensible water loss.
a Hyperthermia and irritabiliry.
a Skin rashes, usually mild and self-limiting.
a Bronze baby syndrome.
a Retinal damage. Eye injury from bandages is uncomrnon.
Latez
r Damage to intracellular DNA.
o Exposure to light may disturb the circadian lhythm of the
sex hormones; thus, having potential implication regarding
onset of puberty and disturbances in future sex behavior.
Note:
1. Siniple sunlight is usefr.Ll, artificial light sources are better in lowering
r:ncon j ugated hyperbilirubinemia.
2. Phorotherapy should be discontinued as soon as the indirect bilirubin
concentration has been reduced to a level considered safe in view ofthe
infintt age and conclition.
J. Infant r,Lnder photorherapy should leceive additional20-40 mllkgl24
hr fluid to saleguard against dehydration and hemoconcentration.
PHYSIOLOGICAL HYPERBILIRUBINEMIA IN
TERM AND PRETERM BABY
In term baby:
r Full-term infant develops jaundice on or after 2nd or 3rd
day of life.
a

r Unconjugated hyperbilirubinemia: Total serum biiirubin
<12 gldl, the direct fraction is <15o/o of the total.
o Usually the bilirubin peaks to 6-8 mg/dl by 3 days of age
and then falls and resolves before l0 day of life.
o The baby is otherwise healthy.
In preterm baby:
o Preterm infant develops jaundice on or after 2nd or 3rd
day of life, and it resolves by 14 days.
o Unconjugated hlperbilirubinemia: The peak may be 10-12
mg/dl on the 5th day of life and may rise to a maximum 15
mg/dl and then falls, the direct fraction is <l5o/o of the total.
o The baby is otherwise healthy.
Tieatment modalities of hyperbilirubinemia in LBW babies:
12-15
500 5-B
>15
750-1 000 6-1 0
1 000,1 250 B-t 0 15-18
1 250-1 500 10-12 17-20
1 500-2500 15-18 20*25
TSB = Total serum bilirubin
JAUNDICE DUE TO RHESUS INCOMPATIBITITY
Hemolytic disease of the newborn occurs when the mother
(usually Rh-negative) and the fetus (usualiy Rh-positive) are
of different groups. Vhen fetal red cells enter the maternal
circulation, the mother may become sensitized to the Rh-
antigen and form Rh-antibodies (isoimmunization). Later in
pregnancy, these maternal Rh-antibodies cross the placenta in
the opposite direction to act upon the fetal red cells. It is rare
for the first infant of a Rh-positive blood to be afFected, but
this can occur if mother has been previousiy sensitized to the
Rh-antigen by transfusion of Rh-incompatible blood, abortion,
or menstrual regulation.
The genotype of the husband of an Rh-negative wife is
important. If he is homozygous (D/D) with two D genes, all
his children must receive a D gene, so that once hemolytic
disease has made its appearance all subsequent children will be
similarly affected. \fhen, however, the father is heterozygous
Rh-positive (D/d), half his children will inherit d; they will also
have inherited d from their Rh-negative (d/d) mother, they will
be Rh (D) negative and so unaffected by the disease.
Not all "at risk" Rh-negative women become isoimmunized,
and the chance is only I in 12, i.e., affection of the baby due
to Rh incompadbiliry is low considering the increased number
of Rh-positive babies delivered in Rh-negative mothers. The
reasons are:
o Inborn inability of mother to respond to the Rh-antigen
stimulus.
o Associated ABO group incompatibility reduces the incidence
of Rh immunization. The fetal cells (group A, B or AB) in
NEONATOLOGY
such cases are promptly destroyed by the natural anti-A and/or
anti-B isoagglutinins present in maternal circulation, so that
the red cells will not find time to excite Rh isoimmunizadon.
o Volume of fetal blood (<0.1 ml) entering into the maternal
circulation (0. 1 mI is considered as critical sensitizing volume).
Fetol Affeclion by the Rh-Antibody
The antibody formed in the maternal system (IgG) crosses the
placental barrier and enters into the fetal circuiation. The anti-
body will not have any effect on Rh-negative fetus. If the fetus is
Rh-positive, the antibody becomes attached to the antigen sites
on the surface of the fetal erythrocy'tes. The affected cells are
rapidly removed from the circulation by the reticulo-endothelial
system. These are loosely termed as ery'throblastosis fetalis, since
many babies may have a large number of nucleated cells in the
peripheral blood as a result of compensatory erythropoiesis in
response to anemia due to any cause other than Rh factor.
In case of Rh (D) negative women, the serum shouid be
tested at 12, 28, and 36 weeks for Rh-antibodies. The presence
of measurable antibody titer at the beginning of the pregnancy,
a rapid rise in titer, or a titer of l:64 or greater suggests sig-
nificant hemolytic disease. The husband's Rh genotype must
also be determined to heip in more accurate prediction.
Clinicol Feotures
o Hydrops fetalis: This is the most serious form. The exces-
sive destruction of fetal red cells leads to severe anemia,
tissue anoxia, metabolic acidosis, hepatosplenomegaly. As
a result of anemia, there is damage to the liver leading to
hypoproteinemia, which is responsible for generaiized edema,
ascites, and hydrothrorax. Fetal death occurs sooner or later
due to cardiac failure.
o lcterus gravis neonatorum: The baby is born alive without
evidence ofjaundice but soon develops it within 24 hours of
birth. There occurs rapid unconjugated hyperbilirubinemia;
if the bilirubin rises to the critical level of 20 mg/100 ml,
the bilirubin crosses the blood-brain barrier to damage the
basal nuclei ofrhe brain producing kernicterus. These infants
have a significant risk of hypoglycemic hepatic dysfunction,
thrombocytopenia, and DIC.
o Congenital anemia of the newborn: This is the mildest form
of the disease, where the hemolysis is going on slowly. As the
anemia develops slowly within the first few weeks of life, the
jaundice is not usually evident. The destruction of the red
cells continues up to 6 weeks, after which the antibodies are
not availabie for hemolysis. Liver and spleen are enlarged.
lnvesligotions
Specimen of blood should be collected from the maternal end
of the umbiiical cord.
1. Blood groups (Rh & ABO) of baby and mother (group-
ing and genotyping of father, if available). Rh-antibody
titer of mother.
 t

ESSENCE OF PEDIATRICS

2. Serum bilirubin (indirect, direct).
3. Coombs resr.
4. CBC and reticulocyte count.
Sonography during antenatal period would detect polyhydram-
nios, edema, pleural effusion, large placenta. X-ray abdomen
would show "Buddha position" of the fetus with hallow around
the head due to edematous skull.
Treolmenl
Antenatal
The technique of spectrophotometric examination of amni-
otic fuid obtained by amniocentesis from about 28th week
of pregnancy has been used to predict the probable severity
of the disease. Liquor falling into the upper zone of the Liley
graph (zone III) indicates severe fetal disease and impending
intrauterine death. Liquor falling into the middle zone (zone
II) indicates moderately affected disease. Liquor falling into the
lowest zone (zone I) indicates a miidly affected or unaffected
fetus. Thus, cases falling into zone I may safely be allowed to
go to full term.
Cases falling into zone II are the babies with significant
anemia, where premature delivery is necessary; a reasonable
maturity (not less than 34 week) can still be obtained.
The small number of cases falling into zone III will end in
fetal death or hydrops before 34 weeks' gestation. Premature
induction has little to offer in this group; the technique of
intrauterine transfusion has been developed for these babies
(Liley 1965).
Postnatal: Exchange Transfusion
Exchange transfusion is done commonly in hemolytic disease of
the newborn due to Rh incompatibility. The aims of exchange
transfusion in Rh incompatibiliry are (l) to correct anemia,
(il) to remove damaged and antibody-coated RBCs from the
circulation, (iii) rc remove unfixed antibodies, and (iu) to
reduce hyperbilirubinemia.
Indications:
o Cord hemoglobin of 10 g/dl or less.
e Cord biiirubin of 5 mg/dl or more.
o Unconjugated serum bilirubin of >10 mg/dl within 24
hours or rate of rise >0.5 mg/dlihr.
o Exchange at lower bilirubin levels in the presence of peri-
natai distress factors (asphyxia, respiratory distress, sepsis,
hypothermia, etc.).
o Unconjugated serum bilirubin of 20 mg/dl or more in a
term baby.
r In preterm babies, serum bilirubin of >1.0 mgi 100 mg
weight of infant (i.e., 10 mgidl for 1000 g, and 15 mg/dl
for 1500 g and so on).
Exchange transfusion should be repeated as frequently as neces-
sary to keep indirect bilirubin level in the serum under 18-20
mg/dl in full-term infants. A variety of factors may alter this
criterion in either direction in an individual patient.
Blood to be transfused: Blood for exchange transfusion should
be as fresh as possible, should not be more than 4 days old.
Acid-citrate-dextrose (ACD) may be used as an anticoagulant.
The goal should be an exchange of approximately 2 blood
volumes of tire infant (2 x 85 ml/kg). Blood should be gradu-
ally warmed to and maintained at a temperature between 35"
and 37" C throughout the exchange. Whole blood should be
used rather than packed red blood cells, since the former has
suflicient albumin in serum to bind unconjugated bilirubin
(especially when severe anemia is not a problem).
Choice of blood (Thble 2.19):
The donor's blood should be Rh-negative, preferably of
the same ABO group as the infants, and it should also
be compatible with the mothert serum (or baby's serum).
a Group-O, Rh-negative blood is commonly used in pracrice.
a In case ofABO incompatibility, group-O blood of homolo-
gous rhesus group (compatible with mother and infant)
should be used.
In Rh incompatibility, always give negative blood; in ABO
incompatibility, give homologous rhesus group.
Procedure:
r The whole procedure should be done in isolation room with
strict aseptic precautions. An experienced doctor should
conduct the procedure, and an assistant is needed to help,
monitot and tally the volume of blood exchanged. Warmth
of the baby should be maintained. Umbilical catheterization
is needed to be done first.
o Two 3-way stopcocks are attached end-to-end to make
four ways; one way is connected with the distal end of the
umbilical catheter, another (opposite) way is connected with
a20 ml disposable syringe. Of the remaining two sideways,
the distal one (from the operator) is connected with the
donor blood set, and the proximal one is connected with
a saline set leading down to an empty 1000 cc saline bag
being tied with the leg of the cot into which patient's blood
Table 2.19: Choice of Blood Croup in Exchange Transfusion
o O,A,B o
o,B o
A, AB A,O
B O,A o
B B, AB B,O
AB A,O
i
AB B B,O
i
AB AB A, AB, O
I

I
i
t

is expelled out and discarded. Operator should make one
round before starting the exchange to check the valves of
stopcocks and to confirm their functioning. Exchange is
done by push-pull technique.
Blood is removed in aliquots that are tolerated by the infant.
This usually is 5 ml for infants weighing <1500 g 10 ml for
infants 2500 g;15 ml for infants <3500 g; and20 ml for
, infants over 3500 g. The recommended time for exchange
,-
transfusion is <1 hour.
F
The desired amount of blood (say, 1 5 ml) is withdrawn first
? from the patient slowly and steadily; it is then expelled out
I through the proximal sideway outlet, by keeping the inlet
v closed. Then 15 ml of donor's blood is drawn from the
! the infantt own blood forming mechanism may be expected to take over.
suspended bag through the distal sideway outlet by keeping
II
t- the proximal outlet key closed, and the whole amount is
transfused slowly.
As ill infants usually have a metabolic acidosis that can
readily be aggravated by donor's citrated blood with a low
pH (6-7), it has become a common Practice to inject I
ml (1 mmol) o{ 8.4o/o sodium bicarbonate after each 100
rnl of blood exchanged, especially during the first exchange
transfusion. As the citrate is metabolized, there is a later
tendency towards metabolic alkalosis, and so sodium bicar-
bonate should not be given during subsequent exchange(s).
If the patient is found to have features of hypocalcemia
(prolonged QT), then it should be corrected immediately
by 3-5 ml of 10% Ca-gluconate IV
o \Xlhen exchange transfusion is over, phototherapy may be
continued and bilirubin levels are measured 4 hourly.
o At the end of the procedure, blood sample is sent for esti-
mation of post exchange serum bilirubin level. The catheter
may be left in situ when further exchange is expected;
in that case, 5o/o dextrose in aqua at a rate of 4 pdrops/
min should be continued. If there is no need for further
exchange, the umbilical catheter is withdrawn and a dry
dressing is applied.
o An intake-output chart should be maintained. Heart rate,
respiration rate, temperature, color, etc. are to be recorded
on a chart during the whole procedure'
o After exchange transfusion is ovet phototherapy can be
considered.
Possible hazards of exchange transfusion:
Acute:
o Cardiac: Dysrhythmias, arrest) failure from hypervolemia.
o ElectrolJte and metabolic: Hyperkalemia, hypocalcemia,
hyperglycemia.
r Hemorrhage: From cord, internal (e.g., pulmonary cerebral).
o Embolic: Blood clot, air.
o Hypothermia.
o Hazards of blood transfusions: Bacteremia, hepatitis B, HIV
CMV infection, necrotizing enterocolitis, malaria.
o Apnea.
o Oxvgen toxicity may occur at a relatively lower arterial
oxvgen tension because adult hemoglobin (transfused blood)
I
NEONATOLOGY
readily releases oxygen to the tissues by virtue of its low
affinity to bind oxygen.
Late:
a Anemia (hemolytic or hyporegenerative).
a Cholestasis.
a Portal vein thrombosis, portal hypertension.
a Graft versus host reaction.
a Inspissated bile syndrome.
Note:
Ordinary transfusions of compatible Rh-negative blood may be necessary
to cortect anemia ("boost" transfusion, 20 ml/kg) when Hb level falls
below 9 g/100 ml at any stage ofthe disease up to 6-8 weeks ofage, when
Monitoring during exchange transfusion:
o Heart rate, breath sound and added sound, perfusion i.e.
Capillary refill time (CRT), body temperature (hypother-
mia).
o Serum glucose, serum calcium, platelet count, S. electrolyte,
blood culture, S/S of necrotizing enterocolitis, rebound
hyperbilirubinemia.
Prevenlion of Rh lsoimmunizolion
A. Prevent active immunization: Rh anti-D immuno-
globulin (IgG) is administered intramuscularly to the
Rh-negative mother following child birth or abortion.
It should be administered within 72 hours or preferably
earlier following delivery or abortion. It should be given
provided the baby born is Rh-positive and the direct
Coombs test (of mother) becomes negative. In case where
the specified time limit has been exceeded, still it should
be given. Even if it does not protect against sensitization,
it will certainly cause no harm. Similarly, when the Rh
factor of the fetus cannot be determined, it should be
administered without any harm.
Dose: 300 pg of anti-D immunoglobulin is administered
intramuscularly to the mother following delivery; 100 pg
following abortion beyond 20 weeks; 50 pg following
abortion before 20 weeks.
B. Prevent or minimize feto-maternal bleed:
o Precautions should be taken during cesarean section to
prevent blood to spill into the peritoneal cavity.
o Prophylactic ergometrine with the delivery of the
anterior shoulder should preferably be withheld, as it
may facilitate more feto-placental bleed.
o Forcible attempt to perform external version under
anesthesia should be avoided.
o Manual removal of placenta should be done gently'
o To refrain from abdominal palpation as far as possible
in abruptio placentae.
C. Avoid mismatched transfusion: Avoid giving Rh-positive
blood to any Rh-negative female from her birth to the
menopause.
ESSENCE OF PEDIATRICS

ABO HEMOLYTIC DISEASE OF THE NEWBORN

ABO incompatibility occurs in a group O mother with A or
B group infants who become jaundiced in the first 24 hours
of life. The cause is the reaction of marernal anti-A or anri-B
antibodies against the A or B antigen on rhe red cells ofthe fetus
or newborn. It is usually seen only in group A or B infants of
group O mothers, because these mothers have anti-A or anri-B
antibodies of the IgG class, which cross rhe placenta, while
mothers of A or B blood group usually have anti-A or anri-B
antibodies of the IgM class, which do not cross rhe placenta.
The combination of a group O mother and an A or B blood
group infant occurs in 75o/o of pregnancies.
The majority of ABO-incompatible infants have anti-A or
anti-B antibody on their red cells, yet only a small number
has significant ABO hemolytic disease of the newborn. Infants
may have a low concentrarion of antibody on their red cells;
consequently, their antibody will not be demonstrated by a
direct Coombs test. As the antibody concenrrarion increases, rhe
antibody can bb demonstrated by the Coombs test. Although
all ABO-incompatible infants have some degree of hemolysis,
significant hemolysis is usually associared only with a positive
direct Coombs test result on the infant's red cells.
Kernicterus is a neurological syndrome resulting from bilirubin
toxicity within the brain-staining and necrosis of neurons
in the basal ganglia, hippocampal correx, subthalamic nuclei
and cerebellum followed by gliosis of these areas. The cerebral
cortex is usually spared.
ETIOTOGY
o Prematurity
o Hemolytic disease of the newborn
r Congenital familial non-hemolytic jaundice
o Neonatal hepatitis
o Congenital spherocltosis
The precise blood level above which indirect-reacting biliru-
bin or free bilirubin will be toxic for an individual infant is
unpredictable, but kernicterus is rare in healthy term infant
and in the absence of hemolysis if serum level is under
25 mgldl.
CtINICAI FEATURES

Kernicterus is a pathological diagnosis of bilirubin toxicity and

BREAST MILK JAUNDICE
This is of late onset. By day 7, instead of the usual fall in the
serum bilirubin level, the bilirubin level continues to rise and
may reach 20-30 mgldlby 2"d-3'a week of age. If breast-feeding
is continued, the levels will stay elevated and then flll slowly
after the 2n'1-3rd week, returning to normal by 4-12 weeks of
age. The mechanism of true breast-milk jaundice is unknown
but is thought to be due to factors in breast milk, e.g., glu-
coronidase interfering with conjugation or metabolism.
If breast-feeding is stopped, the bilirubin level will fall
rapidly in 48 hours. If nursing is then resumed, the bilirubin
may raise 2-4 mgldl but usually will not reach the previous
high level. These infants show good weight gain, have normal
liver function test results, and show no evidence of hemoly-
sis. There are rare reports of kernicterus on otherwise healthy
breast-fed, term newborns.
EARTY BREAST. FEEDING JAUNDICE
Characterized by early-onset unconjugated hyperbilirubinemia
(>12 mg/dl) in the first week of life. This observation is due
to decreased milk intake or calorie intake. Giving supplements
of glucose warer ro breast-fed infants is associated wirh higher
bilirubin levels owing to reduced intake of higher caloric
density breast milk.
Frequent breast feeding (>10124 hr), rooming-in with night
feeding and discouraging5o/o dextrose or water supplementation
may reduce the incidence of early breast-feeding jaundice.
refers to yellow staining of brain, but the bilirubin toxiciry is
manifested as follows:
1. The early signs may be subtle and indistinguishable from
those of sepsis, asphlxia, hypoglycemia, and intracranial
hemorrhage. Lethargy, poor feeding, and loss of Moro
refex are common initial signs.
2. Acute bilirubin encephalopathy: Early signs and symproms
usually appear 2-5 days after birth in rerm infants and
as late as the 7th day in premarure ones, but hyperbili-
rubinemia may lead to the syndrome ar any time during
the neonatal period.
Three phases:
Phase I : Hypotonia, lethargy, poor suck, and depressed
sensorium.
Phase II : Hypertonia, progressing to opisthoronus,
rigidiry oculogyric crisis.
Phase III : High-pitched cry, convulsions, hypotonia
replaces hypertonia after about 1 week of
age, death.
Many infants who progress to rhese severe neurologic
signs die; the survivors are usually seriously damaged but
mav appear to recover, and lor 2-3 months manifest few
abnormalities. All infants who survive this phase develop
chronic bilirubin encephalopathy.
3. Chronic bilirubin encephalopathy: In the first year of life,
opisthotonus, muscular rigidiry irregular movements, and
convulsions tend to recur. In the 2nd year, opisthotonus
and seizures abate but irregulaq involuntary movemenrs,

1
\
I

I

muscular rigidiry or in some infants, hypotonia increase
steadily. By 3rd year of age, the complete neurologic syn-
drome of chronic bilirubin encephalopathy is often appar-
ent, consisting of choreoathetosis with involuntary muscle
spasm, extrapyramidal signs, seizures, mental deficiency,
dysarthric speech, high-frequency hearing loss, squints,
and defective upward movement of the eyes. Pyramidal
signs and ataxia occur in a few infants. In mildly affected
infants the syndrome may be characterized only by mild
to moderate neuromascular incoordination, partial deaf-
ness, or "minimal brain dysfunction", occurring singly or
in combination.
TREATMENT
Appearance of clinical signs suggesting bilirubin toxicity or
encephalopathy is an indication for exchange transfusion at
any level of serum bilirubin.
Once established kernicterus is irreversible, the management
is only syrrlptomatic.
Neonatal cholestasis is characterized by clinical and laboratory
features of hepatic inflammation and dysfunction, particularly
conjugated hyperbilirubinemia and jaundice, persisting beyond
14 days of age. The urine is yellow and when the stool con-
tains no yellow or green pigment then cholestasis is complete'
ETIOTOGY
Causes of neonatal cholestasis (conjugated hyperbilirubinemia)
are as follows:
1. Intrahepatic disorder
a. Idiopathic neonatal hepatitis
b. Endocrine abnormalities, e.g., hypothyroidism
c. Hemolytic disease-Rh S. ABO incompatibilities
d. Pauciry of interlobular bile ducts
e. Inherited disorder-Galactosemia, alpha-1 antitrypsin
deficiency
2. Extrahepatic disorders
a. Biliary atresia
b. Choledochal cyst
c. Congenital infections-SToRCH
d. Viral hepatitis
e. Cystic fibrosis
f. Infections-Viral, bacterial
The primary pathology may be one of the following three
gT)es:
1. Idiopathic neonatal hepatitis, which can occur in either
a sporadic or a familial form, is a disease of unknown
NEONATOLOGY
cause; most cases are idiopathic. These patients presum-
ably are affiicted with a specific yet undefined metaboiic
or viral disease. This condition and biliary atresia (intra
or extrahepatic) have been described to be the different
manifestations of same disease entity.
2. Infectious hepatitis in a neonate may be shown to be
due to specific virus, such as herpes simplex, enterovirus,
cytomegaloviruses or rarely, hepatitis B.
3. Cases of inrahepatic bile duct paucity form a heterog-
enous subset of cholestatic disease.
IMPORTANT TIPS
1. Complete acholic stool
r <10 d (transitional)-intrahepatic cholestasis
o > 10 d (permanent)-Extrahepatic cholestasis (also have
normal birth weight, Iarge liver)
2. Direct bilirubin >20o/o of total suggests extrahepatic
cholestasis.
3. Absence of biliary tree including gallbladder on USG
indicates extrahepatic biliary atresia.
4. If excretion of radioactive substance occurs, shown by
HIDA scan, in the intestine-intrahepatic cholestasis);
if does not occur-extrahepatic cholestasis.
5, Surgeon can feel the presence or absence of biliary tree
peroperatively. If gallbladder is present, peroperative chol-
angiogram will show no excretion of dye in the intestine
in extrahepatic cholestasis.
5. Liver biopsy could differentiate neonatal hepatitis syndrome
from extrahepatic biliary atresia in about 85% ofcases.
APPROACH TO THE DIAGNOSIS
1. History and physical examination: Size and consistency
of liver and spleen; presence of other anomalies (cardiac,
renal, splenic); stool and urine color.
2. Blood and urine analysis: Fractionated seruin bilirubin,
serum bile acids, prothrombin time, ct-1-antitrypsin, and
metabolic screen-urine/serum amino acids, urine reducing
substances, thyroxine and thyroid stimulating hormone.
3. Blood, urine, spinal fluid cultures (bacteria, herpes simplex,
CMV enteroviruses).
4. Serologic studies for evidence of infection (HBtAg, specific
viral serology, and VDRL).
5. Ultrasonography, hepatobiliary scintigraphy (HIDA).
6. Liver biopsy.
Features differentiating idiopathic neonatal hepatitis from
biliary arresia are listed in Table 2.20.
TREATMENT
Refer Table 2.21
 !

ESSENCE OF PEDIATRICS

Table 2.20: Diiferentiation of Idiopathic Neonatal Hepatitis from Biliary Atresia

Familial incidence 20To Un likely

Associated other abnormalities Absent Present, e.9., polysplenia syndrome, etc.
Associated causes ldentified (infection, metabolic, familial) Not identified
Relationship with Preterm, SCA Full terrn
Organ involvement (abnormal size of liver) Hepatosplenomegaly (less common) Hepatomegaly (usr-ral)

Stool lnconrplete cholestasis (stool with some color) Complete cholestasis (acholic stool)
Bile-stained fluid on duodenal intubation May be Urrlikely
Biliary tree with gallbladder by USC Present May be absent (extrahepatic)

Hepatocyte function is intact, uptake of

Uptal<e slLrggish, excretion into the biliary tract
HIDA scan the agent is unimpaired; excretion into the
and intestine eventually occurs
intestine is absent.
HIDA scan after phenobarbitone Enhances biliary excretion of the isotope No change
Severe diffuse hepatocel u lar disease, distortion
I

of lobular architecture, marked infiltration Bile ductular proliferation, the presence of bile
Liver biopsy with inflammatory cells, focal hepatocellular plugs, portal or perilobular edema and fibrosis,
necrosis; the bile ductules show little the basic hepatic lobular architecture intact.
alteration.

Table 2.21: Treatment of Persistent Cholestasis

A. Mainutrition resulting from malabsorption

Replace with infant formula or supplements containing medium,chain triglycerides.
of dietary long-chain triglyceride
B. Fat-soluble vitamin malabsorption
i. Vitamin E deficiency (neuromuscular Replace with 50-400 lU/d as oral o-tocopherol.
degeneration)
ii. Vitamin D deficiency (metabolic bone
Replace with 5000-8000 lU/d of D2 or rn,ith 1,25 dihydroxycholecalciferol.
disease)
iii. Vitamin K deiiciency
Replace with 2.5 5.0 mg every other day as water-soluble derivative of menadione (K,).
Lhypoprolhrombinemia r

C. Mi( ronutrient defit ient y Calcium, phosphate, or zinc supplementation.

D. Defir iency of water-.oluhle vitaminc Supplement with twice the recommended daily allowance.

E, Relention o{ biliary Lonslituents such as bile

Adrninister ursodeoxycholic acid 15-20 rnglkg/d) or bile acid binder (cholestyramine S,t 6 g1d).
acids and cholesterol (itch or xanthomas).
E
t- Progressive liver disease, portal
hypertension (variceal bleeding, ascites, Interim management (control bleeding, salt restriction, spi ronolactone).
hyperspleni.mr.
C. End-stage liver disease (liver failure) Tra nrpla ntat ion.

common in infants fed human milk. Aggressive enteral feeding

Necrotizing enterocolitis, the most common life-threatening
emergency of the neonatal GIT, is an acquired disorder char-
acterized by various degrees of mucosal or transmural ischemic
necrosis of bowel; the incidence being 5-10% in VLB\W babies.
Both incidence and case fataliry rates increase with decreas-
ing birth weight and gestational age. The triad of intestinal
ischemia (injury), enteral nutrition (metabolic substrate), and
pathogenic organisms has classically been linked to NEC.
The greatest risk factor for NEC is prematurity. NEC rarely
occurs before the initiation of enteral feeding and is much less
may predispose ro rhe development of NEC. Asphp<ia, acute
cardiopulmonary disease, exchange transfusions, and enteric
pathogenic microorganisms are associated risk factors.
Common presentarions are feeding intolerance, abdominal
distention and bloody stools, vomiting (of bile, blood or both),
ileus, abdominal wall erythema or induration, persisrenr local-
ized abdominal mass, or ascites, decreased or absent bowel
sound. The clinical course is variable, but can progress rapidly
to gangrene, perforarion, and shock. The diagnosis is confirmed
by the presence of pneumatosis intestinalis (gas accumulation
in the submucosa of the bowel wall) or gas in the porral vein

a
 NEONATOLOGY

(sign of severity) and pneumoperitoneum indicates a perforation

on the abdominal x-ray. Hepatic ultrasonography may detect
portal venous gas despite normal abdominal x-ray. Thrombo- Post-term infants are those who born after 42 weeks of gesta-
cytopenia, persistent metabolic acidosis, and severe refractory tion, calculated from the mother's LMP, irrespective of weight
hyponatremia constitute the most common triad of signs. at birth. This designation is often used synonymously with the
, term postmature infants whose gestation exceeds the normal
280 days by 7 days or more.
TREATMENT
)- These post-term, postmature infants are often of greater
Medical: birth weight and are characterized by absence of lanugo,
v decreased or absent vernix caseosa, long nails, abundant scalp
, NPO, nasogastric suction, bowel decompression, and admin-
hair, parchment-like or desquamating skin, and increased
r istration of IV fluid or total parenteral nutrition (TPN)
alertness. If placental insufficiency occurs (20o/o), the infants
v for 10_14 days. Enteral feeds are then re-introduced with
p may have a variety of physical signs-desquamation, long
progressive increase in volume and concentration of feeds to
v nails, abundant hair, alertness, pale loose skin especially
I full calories by mouth by 7-14 days. A lactose-free formula
around the thighs and buttocks, growth retardation, wasted,
v may be necessary if lactose intolerance occurs.
meconium-stained skin, vernix, umbilical cord, and placental
iv IV antibiotics for 10-14 days; Ampicillin (50 mglkgl
are used
membranes.
dose bid), Gentamicin (2.5 nglkgldose bid), and Metronida-
I zole (7.5 mg/kg/dose bid) is a suitable regimen. Alternatively,
r TREATMENT
t gentamicin and clindamycin (1540 mg/kg/d) can be used.
I Fluids: Up to 200 ml/kg/d may be needed; volume in the o Preventive treatment is important including induction of
I form of normal saline or fresh frozen plasma or whole blood labor or cesarean section, when needed.
v
F (10 ml/kg) and/or ionotropes (dopamine, 3-5 pg/kg/min) r Meconium aspiration pneumonia or hypoxic encephalopathy
F may be required to maintain blood pressure, urine output, and is treated symptomatically.
rl, tissue perfusion in presence of endotoxic shock and peritonitis.
I
I Infusion of bicarbonate (2 mEq/kg) may be necessary to
L
t correct metabolic acidosis.
)-
t
r Correction of hematologic, metabolic, and electrolyte abnor-
malities are essential to stabilize the infant.
t-
t
o Analgesia with Morphine (0.1 mg/kg) for painful distention Hemorrhagic disease of the newborn occurs in I of every
v and peritonitis may be necessary. 200*400 neonates not given vitamin K prophylaxis. A
f- . Supplemental oxygen and mechanical ventilatory support moderate decrease in factors II, ViI, IX and X normally
r (in apnea or abdominai distension lead to hypoxia and occurs in all newborn infants by 48-72 hour after birth,
It hypercapnia) should be provided as needed. with a gradual return to birth levels by 7-10 days of
age. This transient deficiency (classic form) of vitamin
Surgical:
K-dependent factors is probably due to lack of free vitamin
20o/o infants with NEC undergo surgery in the following
K from the mother and absence of the bacterial intestinal
situations:
flora normally responsible for the synthesis of vitamin K.
o Bowel perforation as evidenced by pneumoperitoneum. Accentuation and prolongation of this deficiency between the
o Full-thickness necrosis of bowel wall with impending perfo- 2"d and 7'h days of life result in spontaneous and prolonged
ration as evidenced by dilated loop of intestine that remains bleeding. Hemorrhagic complications are more frequent in
unchanged in position and shape for >24 hours on serial breast-fed (poor source of vitamin K) than in formula-fed
radiographs. infants. Eady-onset (0-24 hour) life-threatening vitamin
o Peritonitis as suggested by ascites, abdomina.l mass, edema and K deficiency induced bleeding occurs if the mother has
erJ,thema of abdominal wall, and localized abdominal rigidiry. been treated with phenytoin, primidone, methsuximide,
o Failure to respond to medical treatment. or phenobarbital. Late onset (>2 week) is often associated
The necrotic portion of the gut is excised, and end-to-end with vitamin K malabsorption (neonatal hepatitis, biliary
atresia, cystic fibrosis).
anastomosis is established.
The most common form of bleeding is melena (melena
Note: neonatorum); frank blood may be passed per rectum in severe
1. Mortality is approximately 40o/o, w'ith 4o/o recurrence rate.
cases. Hematemesis; hematuria; nasal, subgaleal, intracranial,
2. Strictures occur in 10olo and usualiy present 2-8 weeks after the initial
illness with abdominal obstruction and/or bleeding. Surgically treated
post circumcision, or vaginal bleeding; and hemorrhage from
patients have a higher incidence of rwo or more strictures. the umbilicus or into the skin may also occur. The prothrom-
3. Removal of >70o/o of the bowel results in serious nutritional distur- bin time (PT), clotting time, and partial thromboplastin time
bances with maiabsorption of fats, vit B,r, and bile salts. are prolonged. The levels of vitamin K dependent coagulation
ESSENCE OF PEDIATRICS

factors II, \aII, IX, and X (Thrombotest) are significantly o X-ray and Echocardiography, when respiratory distress is
reduced. Vitamin K facilitates post-transcriptional carboxyla- present.
tion of factors II, \4I, IX, and X. In the absence of carboxyla-
tion, such factors form PIVKA (protein induced in vitamin
TREATMENT
K absence), which is a sensitive marker for vitamin K status.
The platelet level is normal. 1. Hypoglycemia
a. Asymptomatic ltypoglycem.ia:
DIFFERENTIAT DIAGNOSIS i) If the infant is term, treat with early feeding in the
first 5-12 hour of life. Re-check glucose level. If
Factor VII and IX deficiency (only 5-35o/o cases become clini-
cally apparent in the newborn period), DIC, the swallowed
glucose level is <25 mgldl, treat with IV glucose

blood syndrome (blood from marernai source, contains adult

hemoglobin, prompdy change to alkaline hematin with alkali,
Apt test-yellow-brown).
TREATMENT
1. Vitamin K, (phytomenadione) 1-5 mg, IM or IV in a
single dose.
2. If there is actiye bleeding or PT is greatly prolonged, 10
ml/kg fresh frozen plasma with an IV dose of 1 mg of
vitamin K is given.
3. In cases where blood loss is severe and sudden, transfusion
of fresh blood (20 mUkg) is life-saving.
4. Vitamin K,, I mg/wk for the first 3 months of life, may
prevent late hemorrhagic disease of newborn.
If maternal diabetes is not well-controlled, infant of diabetic
mother (IDM) may have the following complications: hypo,
glycemia, hypocalcemia, hypomagnesemia, perinatal asphlxia,
respiratory distress syndrome, hyperbilirubinemia, polycythe-
mia, renal vein thrombosis, macrosomia, birth injury, congenital
malformations.
CTINICAT FEATURES
Infant may be large for gestational age; if mother has vascular
disease, infant may be small. If macrosomia present, birth
injury may be present. Respiratory distress may also occur.
Cardiomegaly with heart failure may also be present. One
should look for congenital anomalies.
TABORATORY STUDIES
Serum glucose level should be checked at delivery and at
Y2, 1,2, 4,8,12,24,36,48 hour ofage.
Serum calcium level should be obtained at 5,24,48 hour
of age; if found low, serum magnesium level should also
be estimated.
a Hematocrit should be checked.
a S. bilirubin level should also be checked. ABG, CBC when
needed.
at 6 mg/kg/min. Infusion may be increased
until normoglycemia occurs. Bolus glucose is
contraindicated.
ii) For infants with Dextrostix value 25-45 mgldl
and there is no risk factor and infanr is clinically
stable, early feeding with 5o/o dextrose in warer
can be given.
b. Symptomatic hypoglycem.ia (transient) :
i) Infuse 2 mllkg of l0o/o Dexrrose srat, rhen give
continuous infusion of glucose 6-8 mg/kg/min,
and increase the rate as needed to maintain a
normal blood glucose (>40-50 mg/dl); the level
should be monitored hourly untill stable. Higher
concentration of glucose that can be given
through peripheral vein is 12.5o/o.
a
i0 If IV iine cannot be started, Inj. glucagon 300
mg/kg SC or IM can be given.
Persistent hypoglycemia.. -When persisting over 7
days.
i) Continue glucose infusion 16-20 mglkglmin.
ii) Consider glucagon 0.3 mg/kg/dose.
iii) Consider a trial of hydrocorrisone 5 mg/kg/d IV
12 hourly or prednisolone 2 mglkgld orally. If
no improvement, make a work-up for specific
diagnosis. May consider the following drugs:
human growth hormone, diazoxide, somatostarin
and surgery to remove part of pancreas.
) Hypocalcemiu I0o/o calcium gluconate should be given IV
tie initial dose, a maintenance dose is given by
siowly. After
continuous IV infusion. Monitor every 12 hour.
3. Magnesium maintenance therapy: Magnesium is usually
added to IV fluid or given orally as magnesium sulphate
50o/o, 0.2 ml/kg/d (4 mEq/ml).
4. Treatment of other problems, when present (perina- \
tal asphlxia, RDS, cardiomyopathy, hyperbilirubinemia,
polycythemia, renal venous thrombosis, blrth injuries-
fracture,Erbpalsy,congenitalmalformations,etc).
a Blood glucose <40 mgldl (2.2 mmol/L) or
a Plasma glucose <45 mgldl
 NEONATOLOGY

CtINICAt FEATURES
Stable VLBW on parenterdl FirstT2hr as high-risk babies,
A. Asymptomatic nutrition then daily
B. Symptomatic: Jitteriness, stuPor, tremor apathy,
Crowing VLBW babies Once a week as FIU
cyanosis, convulsion, apneic spells, tachypnea, weak cry.
High-pitched cry, lumpiness, lethargy, sudden pallor, Asymptomatic, bloocl sugar After t hr of oral feed, then every 6 hr
hypothermia, cardiac arrest. 20-40 mg/dl on screening till 4B hr if Blood sugar >50 mg/dl

Asymptomatic, Lrlood sugar After t hr of starting lV fluid then

HIGH.RISK BABIES <20 mg/dl every hr till blood sugar <50 mg/dl

Asymptomatic, blood sugar

Once the brlood sugar levels are
o LBW (<1800 g), preterm (<32 weeks), SGA birth weight below 40 mg/dl even after
above 50 mfdl, every 6 hr for 48 hr
<10'h percentile, IDM t hr of oral feed
r Infant of Rh hemolytic disease
Symptomatic babies
At any point when exhibiting signs
. Any sick neonate compalible with hypoglYcemia

Babies exhibiting signs compatible with hypoglycemia at any time also need to be
screened.
TREATMENT

fuymptomatic
r Direct breast feeding
o EBM in baby unable to suck Normal total serum calcium: 10-11 mg/dl (2-5 mmollL).
r Add 5 g sugar to 100 ml of milk Hypocalcemia: <7 mgldl in term or ionized calcium
.l Monitor blood sugar after I hour and then 6 hourly <4 mgldl (<1 mmol/L).
Symptomatic or blood sugar <20 mg/dl
o Bolus 107o dextrose 2 ml/kg stat TYPES
,r IV infusion 6 mglkg/min -+ check blood
@ sugar after I
hour and 5 hourly if blood sugar A. Early-onset hJpocalcemia: (First 3-4 days)
1. Prematurity
If Blood sugar <50 mg/dl
i. Premature termination of placental supply
,r Increase glucose infusion rate 2 mglke/min every 15
ii. Increased postnatal drop
minute till euglycemic iii. Decreased target organ resPonse to PTH
,r If in two values aft.er 24 hours >50 mg/dl, taper offinfu- 2. IDM
sion @ 2 mg/kg/min every 6 hour until 4 mglkglmin i. Increased calcium demand
then omit IV 3. Perinatal asphyxia (usually in SPNA)
o Stop monitoring when two values are >50 mg/dl on oral i. Maternal hyperparathyroidism
feeds ii. Renal insufficiency
o Persistent hypoglycemia: Failure to maintain blood sugar iii. Metabolicacidosis
despite infusion 12 mglkglmin or unstable sugar level by iv. Decreased PTH secretion
7 days of age. B. Persistent or late-onset hypocalcemia (end of firstweek)
r> Give hydrocortisone 5 mg/kg/d IV or PO in two divided
doses
INVESTIGATIONS
o Diazoxide 10-25 mglkg/d PO in three divided doses
(not in SGA) a Serum Mg
L
o Glucagon 100 mgikg SC or IM a Serum phosphate
o Octreotide 2-10 Fgikgld SC 2-3 times a day a Alkaline phosphatase
Formula for infusion rate = Dextrose being infused x Rate
o/o a PTH
(ml/hr)/Body weight in kg 6 a ljrine catecholamine
"
SCHEDULE FOR BLOOD GLUCOSE CLINICAT FEATURES
MONITORING
1. Asymptomatic
2. Symptomatic: Neuromuscular irritabiliry myoclonic jertr<s,
exaggerated startle, .iitteriness, seizures, apnea' cyanosis,
At risk neonates 2, 6, 12, 24, 48, 72 hr
tachypnea.
Sepsis, asphyxia, shock Every 6-8 hr
ESSENCE OF PEDIATRICS

TREATMENT Table 2.23: Type of Fluid

Dose: 40 mg/kg/d of elemental calcium (4 mllkgld of 10o/o

< 1000s 5''" DA
First 48 hr
Ca gluconate)-IV infusion or orally (6 hourly) for 3 days >10009 10%DA
<1000 s
o IV preparation can be given orally After 48 hr
5% DA in 0.25% NaCl

o Ca gluconate 1 ml = 10 mg >1000 g 10o,. DA in 0.259" \aCl

fuymptomatic:
Additional fuid for increased IWL in special situation:
o B0 mg/kg/d (8 ml/kg/d of l0o/o Ca gluconate) for 48 hours
o Then 40 mglkgld (4 mllkgld of 70o/o Ca gluconate) for r Radian warmer: Increase 20 mllkgld
24 hours, then stop o Phototherapy: 20 ml/kg/d
o Increased losses from other route - volume for voiume
Symptomatic: (gastric aspiration)
o Bolus 2 mllkg diluted with 1 : 1 with 5o/o DA given over 10 Restrict fluid by 30o/o of maintenance in severe perinatal
minutes under cardiac monitoring asphyxia, RDS, meningitis, intraventricular hemorrhage (I\rFI),
o Then 80 mg/kg/d elemental Ca (B ml/kgld of l0o/o Ca PDA, SIADH.
gluconate) by continuous infusion for 48 hours
o Then 40 mglkgld (4 mVkgld of 70o/o Ca gluconate) for If patient is in shock:
24 hours, then stop o 10-20 ml/kg NS immediately, then
o Assess the deficit by formula (wt in kg x 7o of deficit x 10)
SIDE EFFECTS AND PRECAUTIONS
o Replace half by N/2 saline over 8 hours
o Remaining half by N/2 saline over 16 hours
a Bradycardia and arrythmia
a IV sites where Ca is infused should be checked daiiy for
extravasation to avoid tissue nectosis.
Never give Ca in umbilical artery catheter. Mechanical ventilation is defined as movement of gas in and
out of the lungs by an external source (resuscitation bag, CPAP
device, or Yentilator).
Types of ventilator support:
After birth, there is effiux of water from intracellular fuid o Continuous positiae airuay pressure (CPAP) can be deliv-
(ICF) compartment to extracellular fluid (ECF) compartment
ered by lollowing devices
resulting in an increase in the ECF volume and loss of iCF
in the first week of life. There is also salt and water diuresis ,r Face mask
during 48-72 hours of life. Kidney of neonare has limited o Nasal or nasopharyngeal prongs
capacity to excrete dilute or concentrated urine. So the urine
,r Endotracheal tube
osmolality is narrow; and the capaciry to excrete or conserve o Mechanical aentilator
sodium is limited. o Bag and mask or bag to endotracheal tube
Acceptable urine osmolality is 300-400 mosm/L with daily o Pressure control ventilators
urine output of 2-3 ml/kgid. In addition to mandatory loss of o Synchronized and patient triggered ventilator
water by kidney and GIT (sensibie water loss tS\XaLl), there is o Volume control ventilator
water loss from skin and lungs also (insensible water loss [I\X/L]). o High-frequencyventilator
i\ML is more in the preterm infant due ro thinness of skin. As
the skin matures (cornification) in a preterm baby, the I\WL CONTINUOUS POSITIVE AIRWAY PRESSURE
decreases and become similar to a term baby by the end of first
week. Refer Thble 2.22 for daily fluid requirements of a neonate CPAP is a modaliry of respiratory support in which increased
during the first week of life, and Table 2.23 for type of fluid. pulmonary pressure is provided artificially during the expiratory

Table 2.22:. Daily Fluid Requirement during First Week of Life (ml/kg/d)

<1000 g BO 100 120 130 140 150 160

1Oo0-1500 g BO 95 110 120 130 140 150
.150
>1500 g 60 75 90 105 120 135

I
!
 NEONATOLOGY

CTINICAI FEATURES
Stable VLBW on parenteral First 72 hr as high-risk babies,
A. Asymptomatic nuffition then daily
B. Symptomatic: Jitteriness, stupor, tremor apathy,
Crowing VLBW babies Once a week as F/U
cyanosis, convulsion, apneic spells, tachypnea, weak cry.
High-pitched cry, lumpiness, lethargy, sudden pallor, Asymptomatic, blood sugar After t hr of oral feed, then every 6 hr
hypothermia, cardiac arrest. 2Aa0 m{d1on screening till 48 hr if Blood sugar >50 mg/dl
Asymptomatic, blood sugar After t hr of starting IV fluid then
HIGH.RISK BABIES <20 mg/dl every hr till blood sugar <50 mgidl

Asymptomatic. blood sugar

o LBV (<1800 g), preterm (<32 weeks), SGA birth weight Once the blood sugar levels are
below 40 mg/dl even after
above 50 mfldl, every 6 hr for 48 hr
<10'h percentile, IDM t hr of oral feed
o Infant of Rh hemolytic disease At any point when exhibiting signs
Symptomatic blabies
. Any sick neonate compatible with hypoglycemia

Babies exhibiting signs compatible with hypoglycemia at any time also need to be

TREATMENT screened.

Asymptomatic
, Direct breast Feeding
o EBM in baby unable to suck Normal total serum calcium: l0-11 mg/dl (2-5 mmollL).
o Add 5 g sugar to 100 ml of milk Hypocalcemia: <7 mgl dl in term or ionized calcium
o Monitor blood sugar after t hour and then 6 hourly <4 mgldl (<l mmol/L).
Symptomatic or blood sugar <20 mg/dl
o Bolus 10%o dextrose 2 ml/kg stat TYPES
o IV infusion E 6 mglkglmin -+ check blood sugar after 1

hour and 6 hourly if blood sugar A. Early-onset hypocalcemia: (First 34 days)

1. Prematurity
If Blood sugar <50 mg/dl
i. Premature termination of placental supply
o Increase glucose infusion rate 2 mglke/min every 15 ii. Increased postnatal drop
minute till euglycemic iii. Decreased target organ response to PTH
o If in two values after 24 hours >50 mg/dl, taper off infu- 2. IDM
sion @ 2 mg/kg/min every 6 hour until 4 mglkglmin i. Increased calcium demand
then omit IV 3. Perinatal asphlxia (usually in SPNA)
,r Stop monitoring when two values are >50 mg/dl on oral i. Maternal hyperparathyroidism
feeds ii. Renal insufficiency
. Persistent hypoglycemia: Failure to maintain blood sugar iii. Metaboiicacidosis
despite infusion 12 mglkglmin or unstable sugar level by iv. Decreased PTH secretion
7 days of age. B. Persistent or late-onset hnlocalcemia (end of first week)
o Give hydrocortisone 5 mglkgld IV or PO in mo divided
doses
INVESTIGATIONS
i) Diazoxide 10-25 mglkg/d PO in three divided doses
f- (not in SGA) o Serum Mg
t a)Giucagon 100 mg/kg SC or IM o Serum phosphate
o Octreotide 2-10 pg/kg/d SC 2-3 times a day o Alkaline phosphatase
Formula for infusion rate = o/o Dextrose being infused x Rate o PTH
(ml/hr)/Body weight in kg x 6 o Urine catecholamine

SCHEDULE FOR BLOOD GTUCOSE CLINICAL FEATURES

MONITORING
1. Asymptomatic
2. Symptomatic: Neuromuscular irritabiliry myoclonic jerks,
exaggerated startle, jitteriness, seizures, apnea, cyanosis,
At risk neonates 2, 6, 12,24, 48,72 hr
tachypnea.
Sepsis, asphyxia, shock lvery 6-8 hr
ESSENCE OF PEDIATRICS

TREATMENT Table 2.23: Type of Fluid

< g
i000 5o'o DA
Dose:40 mglkgld of elemental calcium (4 mllkgld of l0o/o First 48 hr
>1000 g 10% DA
Ca gluconate)-IV infusion or orally (6 hourly) for 3 days
. <1000 g 5% DA in 0.25% NaCl
r IV preparation can be given orally After 48 hr
r Ca gluconate 1 ml = 10 mg >1 000 g 10% DA in 0.25% NaCl

Asymptomatic:
o 80 mg/kg/d (8 ml/Lgld of 70o/o Ca gluconate) for 48 hours
o Then 40 mglkgld (4 mVkgld of l0o/o Ca gluconate) for
24 hours, then stop
Symptomatic:
o Bolus 2 mllkg diluted with 1:1 wirh 5o/o DA given over 10
minutes under cardiac monitoring
o Then 80 mg/kg/d elemental Ca (8 ml/kgld of l0o/o Ca
gluconate) by continuous infusion for 48 hours
o Then 40 mglkgld (4 mllkgld of l0o/o Ca gluconate) for
24 hovs, then stop
SIDE EFFECTS AND PRECAUTIONS
a Bradycardia and arrythmia
a IV sites where Ca is infused should be checked dailv for
extravasation to avoid tissue necrosis.
Never give Ca in umbilical artery catheter.
After birth, there is eflux of water from intracellular fuid
(ICF) compartment to extracellular fluid (ECF) compartment
resulting in an increase in the ECF volume and loss of ICF
in the first week of life. There is also salt and water diuresis
Additional fluid for increased IWL in special situation:
o Radian warmer: Increase 20 mllkgld
o Phototherapy: 20 ml/kg/d
r Increased iosses from other route - volume for volume
(gastric aspiration)
Restrict fuid by 30o/o of maintenance in severe perinatal
asphyxia, RDS, meningitis, intraventricular hemorrhage (IVH),
PDA, SIADH.
If patient is in shock:
o 10-20 ml/kg NS immediately, then
r Assess the deficit by formula (wt in kg ' % of deficit x 10)
o Replace half by N/2 saline over B hours
r Remaining half by N/2 saline over 16 hours
Mechanical ventilation is defined as movement of gas in and
out of the lungs by an external source (resuscitation bag, CPAP
device, or ventilator).
Types of ventilator support:
o Continuous positiae airuay pressure (CPAP) can be deliv-
ered by Following devices
c Face mask

during 48-72 hours of life. Kidney of neonate has limited o Nasal or nasopharyngeal prongs
capacity to excrete dilute or concentrated urine. So the urine
o Endotracheal rube
osmolality is narrow, and the capacity to excrete or conserve o Mecbanical aentilator
sodium is limited. o Bag and mask or bag to endotracheal tube
Acceptable urine osmolaliry is 300-400 mosm/L with daily ,r Pressure control ventilators ;

urine output of 2-3 ml/kg/d. In addition to mandatory loss of r Synchronized and patient triggered ventilator
water by kidney and GIT (sensible water loss [SWL]), there is o Volume control ventilator
water loss from skin and lungs also (insensible water loss [IWL]). o High-frequencyventilator
I\(L is more in the preterm infant due to thinness of skin. As
the skin matures (cornification) in a preterm baby, the I\X/L CONTINUOUS POSITIVE AIRWAY PRESSURE
decreases and become similar to a term baby by the end of first
week. Refer ThbIe 2.22 for daily fluid requirements of a neonate CPAP is a modality of respiratory support in which increased
during the first week of life, and Table 2.23 for rype of fluid. pulmonary pressure is provided artificially during the expiratory

Table 2.22l. Daily Fluid Requirement during First Week of Life (mlikgld)

<l000g: BO 100 120 130 14Q 150 160

t
'10 120 140
1O0o-r5oo g 80 95 1 130 150
>1500 g ' 60 75 g0 105 120 135 150 1
t
l
t
I
L
!
:

NEONATOLOGY

phase of the respirationin a spontaneously breathing neonate. o Frequent apnea unresponsive to drugs
Itis distinct from IPPV or IMV in which breathing is taken o Impending or existing shock
over by the ventilator machine completely and the increase in o PaO, <50 mmHg in FiO, >1.0
pulmonary pressure occurs during both inspiratory as well as o PaCO, >60
expiratory phases. c pH <7.25
Indication of CPAP:
o General anesthesia
r Basic ventilator setting on IMV:
e Respiratory distress syndrome
r o Recurrent apnea
1. Intubate baby, fix endotrachial tube. Check ventilator.
, Air/oxygen should be warmed rc 37" C and humidified
l- o Meconium aspiration syndrome
v ro 70-100o/o.
o For extubation from IMV (prevent post extubation ate-
lactasis)
2. Initial settings:
o Fio,

]il
PaO, below 60 torr with FiO, over 0.6 (600/o) in oxygen hood 0.5
Rate 40-50 per minute
Guideline for initiation of CPAP: The following guidelines PIP 18-20 cm H.O
essentially apply to a baby with RDS due to mild to moderate
PEEP 4-5 cmHrO
HMD. For practical purpose, it is the nasal CPAP that is most Ti 0.4-0.25 sec
relevant to neonatal care:
3. Observe infant for cyanosis, absence of retractions, chest
o Start with nasal CPAP of 5-6 cm HrO and FiO, 0.4-0.5 wall movement and breath sounds.
o If oxygenation is inadequate, increase CPAP by 1 cm HrO, 4. If ventilation is inadequate, increase PiP by I cm HrO
as required every few breaths until air entry appears adequate.
o Reach a level of 8-9 cm HrO 5. If oxygenation is inadequate as indicated by cyanosis or
o Now increase FiO, in steps of 0.05 (5o/o) to a maximum poor saturation on pulse oximeter, increase FiO, by 0.05
of 0.8 every minute until cyanosis is abolished or the saturation
Obtain blood gas after initial stabilization and then as required. touches 90-95%.
The aim is to achieve satisfactory respiratory status clinically 6. Draw arterial blood gas.
and on blood gases. Changing ventilator settings on IMV: Refer Table 2.24.
'Weaning
from CPAP: Monitoring adequacy of ventilation therapy: The venti-
lator settings are always in a dynamic state, especially in the
o Reduce nasal CPAP to a level of 8 cm HrO acute stage making frequent alterations necessary. Judicious
o Reduce FiO, by 0.05 (5o/o) decrements to reach FiO, of 0.4 clinical monitoring along with pulse oximetry and periodic
o Now reduce CPAP by 1 cm HrO decrements blood gas analyses are critical to the success of ventilator
o Reach a level of CPAP of 4 cm HrO and FiO, of 0.4 therapy. The parameters indicating adequacy of ventilation
o Remove CPAP and place the baby in the oxygen hood are given below:
Adequacy ofCPAP: o Clinical parameters: Comfortable baby, absence of cyano-
o Comfortable baby sis, absence of retractions, Prompt capillary filling, normal
o Absence of retractions, grunt blood pressure, adequate chest expansion, adequate air entry.
o Absence of cyanosis o Pulse oxintetry: Saturation 90-93o/o.
r Capillary refill time of 3 seconds or less o Blood gases:
o Oxygen saturation 90*93o/o PaO, 60-80 torr
o Blood gases: PaO, 60-80 torr; PaCO, 40-55 torr; pH PaCO, Acute, 40-50 torr; chronic, up to 60 torr
7.30-7.40 pH 7.30-7.40

INTERMITTENT MAN DATO RY VENTI TATION Table 2.24: Changing Ventilator Settings on IMV
(rMV)
The decision to start mechanical ventilation in a neonate should
t ttr
be individualized and based on clinical as well as blood gas J r.t,t
parameters. Presence of two or more parameters given below, t ltt
in general, forms an indication for mechanical ventilation: J JJJ
o Retractions: Moderate to severe FiO,, fraction of inspired oxygen; PEEP, positive end expiratory pressure; PlP,
o Respiratory rate >70 peak inspiratory pressurei Ti, inspiratory time.
Hp.
Safery limit of MV: FiO, <0.6, PEEP <4 cm H"O, PIP <20 cm H,O. MAP <15 cm
o Cyanosis with FiO, >0.4
 _l

ESSENCE OF PEDIATRICS

t4. Tlxtbook of Neonatal Resuscitation. American Academy of Pediatrics,

1987.
15. fuhcraft I(N. Pediatric Surgeryt 3'd ed. \WB Saunders
Co., 2000.
1. ForfarJO, Arneil GC (ed). Textbook of PaediatricsT'6 ed. Edinburgh:
16. Sherlocks, Dooly J. Diseases of the Liuer (r Biliary System 17'h ed.
Churchill Livingstone, 2008.
Blackwell Scientific Publications, 2000.
2. Meherban Singh. Care of the Newborn 5'h ed. New Delhi: Sagar
17. Khan MR, Rahman ME, Chowdhury AM. Drugtherapy in Children
Publications, 1999.
1" ed. Dhaka, 2003.
3. Hutchison JH, Cockburn F. Practical Paediatric Problems 6th ed.
18. Parveen R, Nasreen R, Rahman ME. Study of Maternal Risk
Singapore: PG Publishing Pte Ltd., 1986.
Factors causing Early Onset Neonatal Sepsis - a case controi study.
4. Roberton N R C. Textbook of Neonatology 2nd ed. Churchili Liv-
M e di ca I Journ a I
My m ens i ngh 2000 ;9 (2) :97 -1 02.
ingstone,1992.
19. Muhuri BR, Rahman ME, Chowdhury PK. Outcome of neonatal
5. Clayden G, Hawkins R (ed). Paediatrics: Treatment and Prognosis
hyperbilirubinemia managed with ET. JCM C M 1998;9 (2) :22-28.
1" ed. New Delhi: Jaypee Brothers, 1989.
20. Ghai OP (ed). Essential Pediatrics 7'1' ed. New Delhi: CBS Publish-
6. Behrman RE,, Kliegman RM, Jenson HB. Nekon Textbooh of
ers. 2009.
Pediatrics 16'h ed. rVB Saunders Co, 2000.
21. Breast feeding management and promotion in a baby friendly
7. Cloherty JD, Stark AR. Manual of Neonatal Care 4'h ed. London:
hospital. UNICEF/WT{O, New York, 1993.
Lippincott Raven, 1998.
22. ParthasarathyA (ed). IAP Textbooh of Pediatrics 4"d ed. New Delhi:
8. Nahar N, et al (ed). Essential Newborn Care, Bangladesh Neonatai
Jaypee Brothers, 2009.
Forum, Dhaka, 2002.
23. Breast feeding counseling - A training course. Trainers guide.
9. Molla MR. Concise Textbooh of Pedianics 2"d ed. Dhaka, 2007. \XTIO/ 1. 93lD raft. 199 3.
10. Dutta DC. Textbooh of Obstetrics and Perinatologlt 4'h ed. Calcutta: 24. Gupte S. 7he Short Tixtbook of Pediatrics B'h ed. New Delhi: Jaypee
New Central Book Agenry, 1998.
Brothers,1998.
11. IMCI Manual, WT{O/UNICEF.
25 Gomella TL. Neonatologlt: Management, Procedures, On-call Pro blems,
12. Resuscitation at Birth. The Newborn Life Support Provider Course
Manual, Resuscitation Council, UK, 2001. Diseases, and Drugs 6'h ed. New York: Mc Graw Hill, 2009.
13. Kulkarni ML. Manual of Neonatology 1" ed. New Delhi: Jaypee
Brothers, 2000.

I
a
I
1

E l
1
a
 CHAPTER 3
lnfant and Young Child Feeding

Chopter Contenls
Breast-feeding.................................,1..................................,,.....49

Advantages of breast{eeding .. ..,...........................................49

Lactation and medicati0ns.........,..........................................50

Positioning and attachment ..........,....................................... 50
Milk production..... ,................................51

Table 3.1: Composition of Human Milk, Cow Milk and One

.l
lnfant Formula (Composition per 00 ml)

Appropriate feeding practices are essential for proper nutrition,

growth, development, and survival of infant and young children.
These feeding practices, which include both breast-feeding and l. Macronutrients
.1.6
Protein (g) 1.3 3.4
complementary feeding, are collectively known as infant and
Fat (g) 4.2 3.9 3.9
young child feeding (IYCF) practice. Saturated (7") 50.1 63.2
It includes (i) early initiation of breast feeding-infant Unsaturated 4A.5 36.6
should be breast-fed within half an hour of birth; (2) exclusive Carbohydrate as 7.O 4.6 7.2
lactose (g)
breast-feeding-breast-fed for the first 6 months of life;
(lll) complementary feeding-children aged 5-9 months who 2. Energy (kcl/l00 ml) 70 67 70

are breast-fed should receive complementary foods like, rice 3. Minerals

Sodium tmgt l5 1g
or starch plus foods from animal sources and one other item (mgl
Potassium 60 155 61
of fruits, pulses, or vegetables; and (iu) continued breast- Chloride tmgr 43 9B 43
feeding-breast-feeding should be continued up to 2 years Calcium rmgr 35 124 59
and beyond along with safe complementary foods. Magnesium img) 2.8 12 5.6
Phosphorus (mg) l5 9B i2
Infant and young children are defined as children aged
<3 years. Note: Calcium content o{ cow milk is 3-4 times more than that ol human milk.
Phosphate content of cow milk is 6-2 times more than that of human milk
Sodium content of cow milk is 4 times more than that of human milk.
Osmolality of cow milk is 3 times more than that of human milk.

The first leed should be colostrum and offered within half an ADVANTAGES OF BREAST.FEEDING
hour of birth. Baby should be provided feeding on demand.
Benefits to the baby:
Mothert miik is the best milk because of its nutritional, anti-
infective, anti-ailergic, contraceptive, and economic significance; o Great protection against infection
human milk must be considered as a resource priority in the r Complete food, species specific
national development, health, and family planning policies. o Easily digested and well-absorbed
Thble 3.1 lists composition of human milk, cow milk, and o Protects againsr inlections
formula. r Better intelligence, psychomotor, and social development
Exclusive breast-feeding means giving baby breast milk r Protecrs From allergy
only, not even a drop of water or other food till 6 months of r Lowers incidence of childhood cancer
Age. ORS or medicine can be given when indicated. o Decreases orthodontic and dental problems
 I

ESSENCE OF PEDIATRICS

Benefits to the mother: Specific and less commonly used drugs and breast-feeding:
a Helps in involution of urerus o Anticoagulants: Avoid phenindione. Heparin, warfarin, and
a Reduces postpartum hemorrhage others are safe.
a Reduces postpartum depression a Antihypertensives: Avoid acebutolol. Alternatives sa r.
e Leads to natural conrraception-delay in pfegnancy a Antimalarials: Avoid mefoquine. Other antimalarials are
o Lowers risk of breast and ovarian cancers safe.
r Decreases mother's work load Antimicrobials
o Develops emotional bonding-results less abuse ,r Metronidazole: Probably safe, use if drug of choice. High
and neglect
doses may make milk taste bitter. Some authorities sug-
o Effective way of shedding extra weight
gest stopping breast-feedingfor 12-24 hours after single
Benefits to the family and the society: dose treatment. Breast-feed normally if not practical to
inrerrupr breast-feed i ng
r Saves money
o Sulfonamides: Cotrimoxazole, sulfadoxine-pyrimeth-
r Promotes family planning
amine- avoid if the baby is jaundiced, or has G6PD
r Decreases need for hospitalization
deficiency.
o Decreases environmental pollution
o Saves foreign currency spent on import of milk powder
Antithyroid drugs: Avoid carbimazole. Use propylthiouracil,
monitor infant if dose is high.
Aspirin: Occasional use is safe. Repeated high doses may
EARTY INITIATION OF BREAST.FEEDING be dangerous.
Ergotamine: Single dose is safe. Repeated doses may be
It is estimated that about 1 million newborn deaths could be
dangerous.
prevented globally if breast-feeding would have been initiated
Iodine containing drugs: Iodine is concentrated in milk.
within an hour of birth. There is also published evidence that
Avoid therapeutic doses and expectorants that conrain
under-5 mortaliry rate could be reduced by l3o/o through
iodine. Nutritional supplements are safe.
exclusive breast-feeding up ro 6 months. Further 6% deaths
Laxatives: Senna may give baby loose stools. Use other
can be prevented by timely start of complementary feeding
laxatives.
with continuation of breast-feeding up ro 2 years of age.
Psychiatric drugs
r Antidepressants: Tricyclic drugs, e.g., amitripryline prob-
TACTATION AN D MEDICATIONS ably safe.
,r Antipsychotics: Chlorpromazine, haloperidol, probabiy
Breast-feeding is contraindicated if mother takes anricancer
safein moderate doscs.
drugs (antimetabolites), radioactive substances (stop breast-
,r Lithium: Toxicity possible. Minimal risk if levels well,
feeding temporarily).Breastfeeding should nor be discontin-
controlled. Monitor babv.
ued if mother takes psychiatric drugs and anticonvulsants.
Avoid repeated doses of barbiturates (including primidone)
and diazepam. There are some possible side effects for which POSITIONI NG AN D ATTACHMENT
the baby should be monitored, like abnormal sleepiness,
unwillingness to feed, and jaundice. Breast-feeding can be Signs of good positioning:
continued if mother takes sulfonamides (especially if baby o The whole body is fully supported rr

is jaundiced), chloramphenicol, and tetracyclines. Small o Body close to the mother

risk of side effects may occur; use alternative drug if pos- r Straight head and body
sible. Breast milk supply may be reduced if mother takes o Facing breast; nose opposite to nipple
estrogen (including estrogen-containing conrraceptives),
thiazide diuretics. Signs of good attachment:

Safe in usual dosage:

o The baby's chin is touching the breast
o The baby's mouth is open wide
Analgesics: Short courses of paracetamol, acerylsalicylic acid, o The babyt lower lip is curled ourward :
ibuprofen, occasional doses of morphine and pethidine. o The baby suckles, pauses, and sucks again in siow deep
Antibiotics: Penicillin, ampicillin, cloxacillin and related sucks. The mother may hear the babyt swaliowing
drugs, erythromycin. o More areola above than below the mouth \
Antihistamines, antacids, digoxin, insulin, bronchodilators
i
Signs of poor attachment:
(e.g., salbutamol), corticosteroids, antihelminthics, chloro- I
quine, antituberculars, antileprotics. o The nipple looks flattened or striped as it leaves the baby 1
a
Nutritional supplements of iron, iodine, and vitamins. mouth at the end of the feed
tl
T

. The mother feels pain in her nipples during and after
feeds
o The mother's breast may be engorged
o There will be inefficient removal of milk from the breast
MItK PRODUCTION
Signs that a newborn is receiving sufficient breast milk:
o The baby breast-feeds at least eight times in 24 hosrs.
v
o During a feed, babyt suckling rhythm will slow down as
I down.
I milk is released, and swallowing may be heard.
a Baby is contented between feeds.
a Baby has six or more wet diapers in 24 hours.
a Baby will have 3-8 bowel movements in 24 hours. As babies
grow older, stooling may be less frequent.
Baby shows a consistent weight gain, with an average of
18-30 g/d.
o The mothert breasts may feel full before a breast-feeding
and softer dfterward, though not all women experience a
dramatic change.
Principal causes of low milk production:
o The baby is not attached at the breast for effective suck-
ling.
o Breast-feeds are infrequent, short, and hurried.
r Other foods and drinks are being given.
o The baby has sucking confusion.
o Night breast-feeds were stopped too early.
How to increase milk production:
o Make sure the baby is attached for effective suckling at
breast-feeds.
o Offer both breasts at a feed, several times each, to increase
milk production.
o bowl of warm water. Do not heat it on the stove, oven a
Breast-feed more frequently and longer, day and night; at
least 10-20 times in 24 hours.
o Stop all use of feeding bottles and dummies.
r Increase the mother's food and fuids, if intake has been
1ow.
o Rest as much as possible, and relax during breast-feeds.
o Offer the breast for comfort if her baby is fussy.
o Use local galactogogues.
o Express breast miik between breast-feeds and feed the
expressed breast milk (EBM) to the baby with a cup or a
supplementer.
EXPRESSING BREAST MItK
Before expression, advise the mother to (l) wash her hands
with soap and water and (ii) massage her breast gently towards
the nipple and/or apply a warm moist cloth a few minutes
before expressing, to help milk flow. She can continue to
massage her breasts during milk expression to stimulate the
milk ejection reflex.
INFANT AND YOUNG CHILD FEEDING
a. Hand expression: Advise the mother to:
i. Put her thumb on her areola above the nipple and
her first finger on the areola below the nipple.
ii. Press her thumb and first finger inward towards the
chest wall a litde way (about 1-2 cm).
iii. Firmly press on the milk sinuses beneath the areola
between the finger and thumb.
iv. Press and release the thumb and forefinger several
times until milk starts to drip out. Milk may drip at
the beginning and the spray out after the milk lets
v. Rotate the thumb and forefinger around the areola
so that milk is removed from all the milk sinuses.
b. The "warm botde" method: tVhen the breast is heavily
engorged, and handling it would cause pain. Advise the
mother to:
i. Clean a large botde with a wide neck.
ii. Pour hot water slowly into the bottle until it is almost
full.
iii. Wrap the bottie in a cloth and pour out the water.
iv. Cool the neck of the bottle and place it over the
nipple so that it makes an airtight seal against the
breast. Hold it there patiently.
]il
v. As the bottle cools, it makes a gentle suction that pulls
the breast into the neck of the bottle and expresses
milk.
c. Breast pumps: Breast pumps are not always practical or
available. If available, show it to mothers how to use it
and sterilize it.
Feeding Expressed Breosl Milk to the Boby
o If the milk needs to be warmed, place the container in a
direct fire, or in a microwave oven.
o \(/arm only the amount of milk that wiil be used at one
feeding. Milk cannot be saved once it has been warmed.
o The fat may separate out in small globules. Gently shake it
to re-combine the fat with the rest of the liquid.
o Feed the milk to the baby with a cup and spoon.
Storing Expressed Breosl Milk
Prepare a clean container that can be covered for storing the
milk. The container should be washed in hot soap watet and
rinsed in hot clean water.
When refrigeration is not available:
o In a temperate climate, milk is safe in a clean con-
breast
tainer at room temperature for 8-10 hours.
When refrigeration is available:
o Place the container of milk in the coldest part of the refrig-
erator or freezer.
ESSENCE OF PEDIATRICS
Chill the milk weli before adding it to previously refriger-
ated or frozen milk.
If the milk is to be frozen, leave space in the container for
expansion.
Use the milk before the safe storage time expires:
o Refrigerator-for 72 hours
o Two door freezer-for 3 months
o Deep freezer at -20o C-for 7 year
MITTENNIUM DEVELOPMENT GOALS
AND BREAST.FEEDING E T+ PR] G
Goal 1: Eradicate extreme poverty and hunger
Breast-feeding s ignzfcantly reduces early c hi ldh o od
cost.
feeding
Goal 2: Achieve universal primary education
Breast-feeding decreases fequenqt of pneumonia and
diat"rhea and bence school abstinence.
Goal 3: Promote gender equality and empower women
Breast-feeding is the great equalizer giuing euerl chiLl
a fair strtrt to life. Mother has the right to take decision
about breast-feeding.
Goal 4: Reduce child mortality
Breast-feeding can reduce child motality by about 13%,
along with complementary feeding reduces another 60/o.
Goal 5: Improve maternal health
Breast-feeding is associated with deueased mdternal
postpartum blood loss. Breast, ouarian, and endome-
trial cancers /t/e ltlso reduced in fequency.
Goal 6: Combat HIV/AIDS, malaria, TB
Exclusiue breast-feeding is associated with reduced
Pltrent to child nansmission of HIV and other diseases.
Goal 7: Ensure environmental sustainabiliry
Milk industry waste and aluminium and tin
not occur in breast-feeding situation.
Goal 8: Develop a global partnership for development
B re as t -fe e di ng s trat eg/ fo s te rs mu h i s e c to ra I
collaboration.
BREAST-FEEDTNG AND H|V/AIDS
It is recommended that only when replacement feeding
acceptable, feasible, affordable, sustainable, and safe (AFASS),
in that situation breast-feeding by HIV infected mothers can
be avoided, otherrvise exclusive breast-feeding is recommended
during the first month of llfe and should then be discontinued
as soon as it is feasible.
ADVICE TO MOTHERS WHO WORK AWAY
FROM HOME
If possible, baby should be taken with rhe morher to work,
or she may go home to feed her baby during breaks, or ask
someone to bring the baby to her at work for breast-feeding.
If the home is too far from work place, mother can give her
baby the benefit of breast-feeding in the following ways:
o Breast-feed exclusiveiy and frequently for the whole mater-
nity leave. The first 2 months are the most imporranr.
b o Continue to breast-feed at night, in the early morning, and
at any time that mother is at home.
o Learn to express breast milk soon afrer baby is born.
o Express breast milk before going to work and leave it for
the care-giver to give it to the baby.
o After the expression, baby should be breast-fed.
o Teach the care-giver properly and carefully to use cup and
spoon, and not to use a bottle, how to clean them and give
It also increases IQ demand feeding or 4 hourly feeding.
o \J(/hile at work, express breast milk 2-3 times to prevenr
engorgement of breast and to increase milk supply. Mother
can store the milk and take it home for the babv.
WET NURSING
\Vet nursing is the process in which the baby feeds from the
breast of another mother. Wet nurses are those lactating women \
who breast-feed a baby who (baby) is not their own. The wet
nurses should be in good health, free from diseases, and must
have a sulficient supply of milk to feed her child and the new
child (the child in need of wet nursing).
and other diseases
Factors contributing to lactation failure:
waste do
o Lack of 'will" on rhe part of morher.
o Lack of support by the family or healthcare providers.
o Poor emptying of the breast due to absence of frequent and
vigorous suckling or too early introduction of bottle.
o Cracked, retracred, short, or too large nipples.
e Wrong technique of breast-feeding.
Preventive measures for lactation failure:
is o Thorough antenatal check-up of the breast.
o Antenatal preparation of the mother for breast-feeding.
o Feeding as early as possible after delivery. Frequent suckling.
o Remedial measures for anatomical defects in the breasts.
o Complete emprying of the breast; if necessary, even manual
expression of milk following feeds may be done.
\
 INFANT AND YOUNG CHILD FEEDING

How to use a breast-feeding suppl€menter:

l. Find a fine nasogastric tube, or other fine plastic tubing,
Relactation is defined as re-establishing adequate milk produc- and a cup to contain milk.
tion in a mother who has greatly reduced milk production or 2. Cut a small hole on the side of the tube, near the end
has stopped breast-feeding. of the part that goes into the babyt mouth (this is in
Iv It also includes increasing an insufficient milk supply. It addition to the hole at the end).
is also possible to induce lactation in women who have not Prepare a cup of milk (expressed breast milk or artificial
I lactated for years or who have never been pregnant. The same milk) containing the amount of milk that the baby needs
f.
principle applies to relactation as to induce lactation-both for one feed.
, Put one end of the tube along her nipple, so that the
I are initiated and maintained by an infant suckling frequently 4.
at the breast. baby suckles the breast and the tube at the same time.
i.
t Tape the tube in place on the breast.
t 5 Put the other end of the tube into the cup containing
L CONDITIONS THAT HEIP A MOTHER
, milk.
RETACTATE
v 6. Tie a knot in the tube if it is wide or pinch it. This con-
I a She is motivated trols the fow of milk, so that the baby does not finish
L
a support and encouragement from her healthcare
She receives the feed too fast.
L
team and family. 7. Control the flow of milk so that the baby suckles for
t
I a The baby is put to the breast frequently for suckling. about 30 minutes at each feed, if possible (raising the
a She expresses milk berween breast-feeds. cup makes the milk flow faster, lowering the cup makes
t,
a She reduces supplementation slowly as her
tion increases.
milk produc-

The baby is fed only at the breast, with a breast-feeding

the milk flow slower).
8. Let the baby suckle at any time that he/she is willing,
not just when the mother is using the supplementer.
9. Clean and sterilize the tube of the supplementer and the
]il
supplementer.
cup, each time the mother uses them.

RETACTATION IN COMPTETE TACTATION

EVIDENCE OF SUCCESSFUT RETACTATION
FAITURE
a Appearance of first milk secretion in 2-10 days.
This is rather more dificult situation. In addition to motiva- a Partial restoration of breast-feeding with reduction of top
tion, encouragement, and support, the following actions are feed to half of initial.
also warranted:
Complete restoration of breast-feeding with total withdrawal
o Nipple stimulation exercises by nipple stroking, massaging of top feed.
the breast, and rolling the nipple benveen thumb and the Satisfactory weight gain by the infant.
index finger.
o Frequent suckling, at least 8-10 times a day, each session
lasting 10-15 minutes for each breast.
Drop and drip method may be employed if the infant fails Lactational amenorrhea method (lAM) of contraception is
to suckle for 8-10 minutes. The method consists of pouring 98% effective, only if:
some expressed breast milk or top milk gradually, as drops,
over the breast. As the drops slide over the nipple down o The mother is <6 months postpartum with no menstrual
into the infant's mouth, the infant is stimulated to suckle bleeding.
at the breast. o The mother breast-feeds exclusively day and night in response
Breast-feeding supplementer may be used to induce suckling to the baby.

in an infant. It is a device for giving a baby a supplement o There are at least six feeds in 24 hours, with no very long
while he/she is suckiing at a breast that is not producing interval.
enough milk. A hungry baby may suckle at an "emprf' breast
a few dmes, but he may become frustrated and refuse to
suckle. To stimulate the breast to produce milk, it is neces-
sary for the baby to suckle. A breast-feeding supplementer Getting the baby accustomed to other foods besides breast
helps to get him to continue suckling. milk is termed complementary feeding. Complementary foods

ESSENCE OF PEDIATRICS
will be given timely after completion of 6 months (180 days).
It should be:
o Adequate: Meaning that these provide sufficient energy,
protein, and micronutrients to meet a growing childt
nutritional needs;
o Safe: Meaning that these are hygienically prepared and
stored and fed with clean hands using clean utensils and
not boftles and teats;
o Responsively fed: Meaning that these are given in consistenry
with a child s signals of appetite and satiety and the meal
frequency and feeding method; and
o Locally available, affordable, and culturally acceptable.
As a general principle, a single weaning food is added at a
time in small quantities; the quantiry is increased gradually,
followed by the second weaning food after some time, say I
week. Weaning of infants can be started with mashed ripe
banana (as it is gruel) and pulses; shortly thereafter khichuri
(prepared from rice, pulses, and vegetables; oil is added after-
wards) can be given. Fish, egg, meat, etc. should be introduced
one after another later. In case of egg, begin with the yolk.
The whole process of weaning should be completed gradually
by 9 months to 1 year of age when the child should be taking
almost the adult diet. Commercially availatrle weaning foods
should be avoided; these offer no advantages over the home-
made weaning foods.
Frequency of giving complementary foods:
o 6-10 months: 3 times daily.
o By 10-12 months: Increasing to at least 5 times (3 meals
and 2 snacks) daily.
o By 3 years: Accustom to family foods and stop breast-
feeding.
Itis a condition when a child becomes apathetic, dull, anemic,
and susceptible to all sorts of diseases due to consumption of
only milk over a prolonged period of time (1 year or more)
without taking any other food. Though milk is a balanced ideal
food, but it contains less iron and vitamin C. So, continued
consumption of only milk after 6 months first develops iron
deficiencv anemia and then scur\v.
Baby friendly hospital initiative (BFHI) is a global effort with
hospitals to provide support to the mother before, during, and
after delivery so that she has a joyful breast-feeding experience.
These hospitals must fully practice "ten steps to successful
breast-feeding" given in a joint W'HO/UNICEF document
(1 989).
E Ii
!
Thanslation of the 10 Steps from Baby Friendly Hospital
Initiative to the community:
1. Arrange meetings with community leaders to discuss
how to promote baby friendly policies in local materniry
facilities.
2. Advocate for raining of peer counselors and primary care
workers to support breast-feeding.
3. Arrange promotional activities to raise community aware-
ness of the importance of breast-feeding and the support
that new mothers need.
4. Work to increase community awareness of the impor-
tance ofearly initiation ofbreast-feeding. Show the video
"breast-feeding crawl" so people see what newborns can
do.
5. Ensure that all mothers learn the techniques for breast-
feeding and hand expression and how to overcome
common difficulties.
\Work with the communiry to raise awareness about the
5.
importance of colostrum, and why a baby needs nothing
else in the first few days, and ofcontinuing to breast-feed
exclusively for 6 months.
7. Support traditions and environments that allow a mother
and child to be together throughout early infancy.
8. Make families aware of feeding cues and the importance
of responding to them. They should feed a baby when it
is hungry and not wait for it to cry, but not over-feed a
child and risk obesity.
9. Talk to families and community groups about the risks
of using teats and pacifiers while breast-feeding is being
established. \7ork with communities to develop a source
of skilled support for breast-feeding mothers.
10. \7omen who have successfully breast-fed can form groups
to support each othet or they can lobby the health authori-
ties or a local organization to train peer counselors.
Cow milk cannot be an entirely satisfactory substitute for
human milk. Introduction of cow milk may cause obesity,
neonatal hypocalcemia, hypomagnesemia, hypernatremia,
increased incidence of allergic disorder, cot death, hyperten-
sion, and atherosclerosis in later life. Goat milk may cause
megaloblastic anemia.
If artificial milk has to be given, dilute cow milk by adding
50 ml of water to 100 ml of milk then add 10 g of sugar to
it with an approved micronutrient supplement. If possible do
not use for premature babies (WHO, 2005).Infant formula
is recommended in rare situations.
Most full-term infants are fed at 4 hourly interval. Howevet
many pediatricians suggest demand feeding.
The infant can be fed by a cup and spoon; bode feeding
should be avoided because it is difficult to keep the bottle
\
t
1
t
t
 INFANT AND YOUNG CHILD FEEDING

clean. Baby should be held with his head and shoulders raised Fildes V Breast, bottles and babies. Edinbulgh: Edinburgh University
during feeding. In any case, the infant should be propped up Press, 1986.

for 10-15 minutes to let him break wind. Milk should be at 6. King FS. Helping Mothers to Breastfeed 2"d ed. Nairobi, Kenya:
African Medical Research Foundation, 1992.
body temperature when it is offered to the infant.
7. Ahmed FU, Rahman ME, Alam MS. Prelacteal feeding. Bangladesh
7
Med Res Counc Bull 1995;22(2):504.
B. Strengthening action to improve feeding of infants and young
children 6-23 month of age in nutrition and child health
programmes. UNICEF/\ITIO, Geneva, 6-9 October 2008.
) Breast feeding management and promotion in a baby friendly
hospital. UNICEF/\XTIO, New York, 1993. Indicators for assessing infant and young child feeding practices,
t Part-3, Country Profile, \fT{O, 2010.
ParthasarathyA (ed). IAP Textbook of Pediatrics 4'h ed. New Delhi:
10 National strategy for IYCF in Bangladesh, IPHN, DGHS,
iI Jaypee Brothers, 2009.
MoHFV 2007.
3. Breast feeding counseling - A training course. Tiainert guide. 'WHO, Geneva, 2006.
\(HO/1.93l Draft. 1993.
11 Pocket book of hospital care for children,
4. Gupte S. 7he Short Textbooh of Pediatria 8'h ed. New Delhi: Jaypee
Brothers, 1998.
 I

CHAPTER

Growth and Development

Chopler Conlenls
Crowth and deve10pmen1................................,,................... 56 Early childhood deve10pment......................,...............,........63 Scales used for assessment of devel0pment................,,. 68

Assessment of gr0wth............................,,..,,,,........................ 58 Assessmeni of development ................................................ 64 Early stimulation program for development ................... 68

Crowth from birth to puberty..............,...............................59 Assessment of development of different areas and Failure to thrive ........,...,...,..... .............69

Crowth chart warning signs at various a9es...........................................65

o Development depends on maturation and myelination of

nervous system.
Sequence of development is same for all children, but rate
Growth and development begin at conception and end at
maturity. Frequently, the terms growth and development are of development varies from child to child.
used together. In a normal child, they progress together, and
Certain primitive reflexes must be lost before voluntary
movements develop.
are interdependent.
Continuous mass activity is replaced by specific individual
Growth means physical maturation and signifies an increase
responses.
in the size of body and its various organs. It occurs as a result
The direction of development is cephalocaudal.
of hyperplasia, hypertrophy, and differentiation.
Development is maturation of function. Development
means attainment of functional maturity that implies acquisi-
tion of skills, emotional development, and social adaptation. KEY NEEDS OF A CHITD
A complete child is one who has both the optimum physical
growth and mental development. For optimum growth, we have to ensure adequate nutrition
and healthcare. For optimum development, we have to ensure
Principles of development: the presence of an environment that facilitates positive interac-
r Development is a continuous process from conception to tion of the child with his caregivers and surrounding animate
maturity. and inanimate objects, besides nutrition and heaithcare.

lnteractive loving care

& opportunities

lnteractive loving care

& opportunities

\
1
a
t
t
-t
FACTORS AFFECTING GROWTH AND
DEVETOPMENT
Genelic Foclors
o Phenotype: The parental traits are usually transmitted to the
offspring. Thus, tall parents have tall children, and children
ofshort stature parents tend to be short in height. The size
of the head is more closely related to that of parents than
are the size and shape of hands and feet.
o Characteristics of parents: Parents with high intelligence
quotient (IQ) are more likely to have children with higher
level of inherent intelligence. This is further enhanced
because of greater degree of environmental stimulation.
Children of mentally subnormal mothers may have lower
IQ than the average.
Race: Growth potential of children of different racial groups
is variable.
Se* The pubertal growth spurt occurs earlier in girls; their
mean height and weight are usually less than those in boys
of corresponding ages at the time of full maturiry.
Biorhlthm and maturation: Daughters often reach menar-
che at a similar age as their mother.
Genetic disorders: Growth and development are adversely
affected by certain genetic disorders. The disorders may be
of two types, viz.:
Chrom.osontal abnortnalities.' Several chromosomal de-
fects manifest in severe growth disturbances. These in-
clude Turner syndrome and Down syndrome.
Gene mutatioz.' Mutation of a single or multiple genes
may result in inherited disorders of growth. The meta-
bolic defects are known in some of these, e.g., mucopoly-
saccharidosis and galactosemia, etc.
o Children of multiple pregnancies: Ultimate growth of
these children is related to the difference in birth weight
of twins. Smaller newborn babies are more likely to attain
lower height and weight.
Prenqlol Period
o Maternal malnutrition is associated with IUGR and small
size of the fetus. Medical illnessesof mother and infections
also result in poor growth of the fetus. Average birth weight
of infants born to mothers receiving nutrition supplements
higher than that of babies of mothers who
during pregnancy is
did not receive nutritional support in the antenatal period.
r consumption of particular rype of foodstuffs. These affect
Maternal infections, e.g., rubella, syphilis, viral hepatitis,
cytomegalic inclusion disease, toxoplasmosis, etc. may be trals-
mitted to the fetus and thus may arrest the fetal development.
o Hormonal infuences on growth:
o Tbyrorine significantly retards the skeletal maturation of
the fetus. Maternal myxedema results in fetal hypothy-
roidism. Administration of antithyroid drugs and iodides
during the later part of pregn ancy may induce fetal goiter
and hypothyroidism.
GROWTH AND DEVELOPMENT
o Insulin stimulates fetal growth. In mothers with overt
or latent diabetes, the fetus is usually large with excessive
birth weight.
o Somatomedin (insulinJike growth factors I and II).
In Larson dwarfism, length of the baby is small at the
time of birth because of somatomedin deficiency; even
thought human growth hormone level is elevated.
Poslnolol Period
o Nutrition: Growth of children suffering from protein-energy
malnutrition and anemia is retarded. Overeating and obesity
accelerate somatic growth.
Chemical agents: Administration of androgen/hormones
initially accelerates the skeletal growth. Ultimately, epiphyses
of bones close prematurely and therefore the bone growth
ceases relatively early in these cases. Final height of these
children is only marginally affected.
Trauma: Head injury may cause brain damage and seriously
jeopardize the mental development of a child.
Infections and infestations: Systemic infections and para-
sitic infestations and chronic diseases usually decrease the
velocity of growth.
Social and environmental factors:
Socioeconomic leael: Children from families with high
socioeconomic level usually have a superior nutritional
state.
Natural resources: Improved nutrition of children in the
community is secured when there is an increase in gross
national product and per capita income is high.
o Enaironrnmulpollutioa.'Pollution of ail water, and sound
adversely affect growth and development of children.
o Climate: The velociry of growth may alter in different
seasons and is usually higher in spring and low in summer
months. Infections and infestations are common in hot
and humid climate. \Teather also has a pivotal effect on
the agricultural productivity, ready availabiliry of food.
o Emotional factors: Children from broken homes and
orphanages do not grow and develop at an optimal rate.
Anxiety, insecurity, lack of emotional support and love from
the family prejudice the neurochemical regulation of the
growth hormone. Parents who had happy childhood and
carry a cheerful personality are more likely to have children
with happy and cheerful behavior.
o Cultural factors: There may be religious taboos against
the nutritional state and growth performance of children.
Growlh Polenliols
The smaller the child at birth (especially in context of gesta-
tion), the smaller he/she is likely to be in subsequent years.
The larger the child at birth, the larger he/she is likely to be in
later years. Thus, the growth potendal is somewhat indicated
by childt size at birth.
 I

ESSENCE OF PEDIATRICS

Table 4.1: Rate of Crowth of Different Organs After birth, brain development depends on increase in
Brain growth 9o%by 3 years of age connections.

Gonadal growth 90"'" by l3 I 5 years o{ age Interaction: It is a continuous process ofgive and take bewveen:
Somatic growth 100% by 1B years of age

r.._--L^:r _..^-..4L At
LVmOnOTO growrn
7 years o{ age, it exceeds 100%; then
declines and comes to normal at 1B years

POSTNATAT GROWTH PATTERI.I

(Fig. a.1 ond Toble 4.1) Things in the
environment
1. General body growth: Body as a whole, respiratory and
digestive organs, aorta and pulmonary trunk, kidneys,
spleen, blood voiume and the whole of musculature and Repeated interactions with or stimulation from the environment
skeleton. There are two periods of rapid general growth- increases the connections.
infancy and adolescence.
2. Brain growth: Brain and its parts dura, optic apparatus,
spinal cord. The brain grows rapidly during the latter
months of .fetal life and early months of post-natal life.
At birth, the head size is about 70o/o of the anticipated ASSESSMENT OF GROWTH
head size in adults. It reaches 90% ofthe adult head size
Growth can be measured in terms of:
by the age of 2 year.
3. Growth of gonads: Testes, ovary, epididymis, uterine o Nutritional anthropometry (weight, length/height, MUAC,
tubes, prostate, prostatic urethra, seminal vesicle. The circumference of head [OFC], chest, abdomen and pelvis)
genital growth is most rapid during adolescence; in the o Tissue growth (skin-fold thickness and measurement of
preceding years, it is more or less a flat curve. muscle mass)
4. Lymphoid growth: Thymus, lymph glands, tonsils, intes- o Bone age (radiological by appearance and fusion of the
tinal lymphoid masses. The growth of lymphoid tissue various epiphyseal centers)
is most pronounced during mid-childhood. During this o Dental age
period, the lymphoid tissue is overgrown, and its mass is o Body proportion
often more than that in adult. Children between 4 and 8 Anthropometry is the simplest tool. Nutritional anthropometry
years of age often have hypertrophied tonsils, adenoids, measures somatic growth.
and large lymph nodes.

Stimulation and interaction is extremely helpful for develop- N ulrilionql Anlhropomelry

ment in early childhood (3-5 year of age), which acts by
increasing neuronal synapses.
BRAIN GROWTH AND INTERACTION
o At Birth: 30o/o of adult brain weight achieved
o At 3 yearc: 90o/o of adult brain weight achieved.
the brain weight is attained by 3-5 years.
Length
llntill 24 months of age, length in recumbency is measured
using infantometer in centimeters up to one decimal point.
Height
Most of After theage of 2 years, standing height is recorded by stadi-
ometer. Subject stands perfectly straight with arms rela-red on

12 yr B 12 yr 12 yr
rl
Brain Somatic Lymph node Gonadal t
t
Fig.4.1: Charts showing postnatal pattern of brain, somatic, lymph node, and gonadal growth t
I
I
 GROWTH AND DEVELOPMENT

side; ankles and knees together; Iooks directly forwards with Body Proporlion
the Frankfurt plane (the line joining floor ofexternal auditory
Body Mass lndex (BMl)
meatus to the lower margin of orbit).
Useful for defining obesity after 2 years of age.

Sitting Height \X/eight (kg)

BMI =
For recording sitting height, the subject is made to sit on a Height (m'z)
table or convenient hard surface so that his/her head lies in
Frankfurt plane. The back should be straight, thighs horizontal
Ponderal lndex (Pl)
and comfortably positioned. The feet should be supported
on the foot board, and hands should rest comfortably on the Used in defining newborn infant with IUGR. A ponderai index
subject's lap. below l0'h percentile helps to identify neonates with IUGR,
especially those with birth weight below 2500 gram.

Body Proportions Birth weight x 100

Ponderal index =
The rotal body length is divided in two segments. The upper Crown-heel lenght3
segment is from head to pubic symphysis and lower segment
from pubic symphysis to the toes. The U/L segment ratio is Arm Span
1:7 at birth. At 3 years I.3; at 5-7 years, it reaches 1:l; and
at 10 years 1. It is the distance between tips of middle fingers when the arms
are outstretched. At birth, it is l-2 cm less than length; equals
at 10 years; and a{ter 12years, it is l-2 cm more than height.
Weight
Done by weighing scale. Accuracy up to 0.1 kg is quite accept- GROWTH FROM BIRTH TO PUBERTY
able. For smaller babies, accuracy up to 0'01 kg is desirable.
It is measured by beam scale, bath room scale (spring/digital), Newborn infant loses about 10olo of body weight and regains
and hanging scale. the weight by the age of 10 days, subsequently gains 25-30 gld
during first 3 months of life; 20 gldbetween 3 and6 months;
(ircumferences 15 gld during 6-9 months; and about 12 gld during 9-12
months. Birth weight doubles at 5 months; triples at I year;
r Head circumference (OFC): The maximum circumference quadruples at 2 years and 5 times birth weight at 3 years'
of head from occipital protuberance to the forehead above
eyebrows in cm. At birth, it is approximately 35 cm.
o Chest circumference: At the level of nipples midway between
[ength
inspiration and expiration, while child is in recumbent posi- Average length at birth is 50 cm; 60 cm at 3 months; 70 cm at
tion. At birth 2.5 cm less than OFC; by 6-12 months, it 9 months; 75 cm at I year; 86-87 cn at 2 year; and 100 cm at
is equal; after 1- year, it is large by 2.5 cm; and at 5 years, 4 year.lhereafter gains 5 cm every year, till the age of 10 years'
it is 5 cm more than OFC.
o Mid upper arm circumference (MUAC): At mid point of
upper arm in flexed or extended position.
Heod Circumference
At birth, 33-35 cm; at 3 months, 40 cm; at 12 months,46
cm; at 2 yeat, 49 cm; at 3 years, 51 cm' If head growth exceeds
Tissue Growlh
I cm in 2 weeks during first 3 months, hydrocephalus should
Skin-Fold Thickness be suspected.

Measured by Langes or Herpendens skin-fold callipers at the

mid arm over triceps on the left side. Weight ond Height
Formula for approximate average weight and height of normal
Bone Age r infants and children:
Should be assessed by a radiologist. The following bones should 'weight: 3-12 month, Age (mo) + 9
-
be evaluated in different ages. 2

o Newborn: X-ray of foot and knee 1.-6 year = [Age (yr) x 2] + 8

o Infant: 3-9 months-X-ray shoulder
o 1-13 years: X-ray hand and wrist 7-72 year =
[Ag. (yt) " 7] - 5

o 12-14 years: X-ray elbow and hip

ESSENCE OF PEDIATRICS

Height: Chronologicol Order of Appeoronce of

At birth : 50 cm Osseous Cenlers (Fig. a.3)
At 1 year : 75 cm
2-12 years: [Age (yr) x 6] + 77 cm At birth : Knee (epiphysis of lower end of femur and
upper end of tibia)
c---r -- afea
Jurtace -,-- = [Vt in kgx 4l + 7 Foot (cuboid, talus, calcaneus)
90+wtinkg 3 months : Head of humerus
4 months : Hamate
Refer Figure 4.2 for surface area nomogram. 6 months : Capitate
1'10
195 240
.190 5
=
=-2^^ 105

- =
I
-2.3 aa 220 100
180 : 210 95
200 90
170 -2.1
- 2.0 190 B5

160
_,n
_1.8
180
BO
170
75
160
150 70
10" -t.t 150
8"
145 _,u 65
140 tu 140
6"
135 =
z ,, '130 60
4" :
130 120 55
2"
125 --1 .3
I 110 50
120 --1 .2
o
11s 6 C)
C)
i-_ 100
456
E
1.1 0 E
c) E
c) 110 .E
c
C)
(E - 5eo
o 40€Y
E
o)
c ^,, J o-
o
105
6)
() q
c a -1.0 .E .E
C') .E .E
: E80 E
=4" 1oo E,
(D

E
: ^^
.9')
a) 35r
(E
= :
-.9U
0)
o = =
95
E
(u
-85
a:, --.80
:
:
: _-
-.tu
:
--.65
- .60
-

:
t-.50
:

_45 15
:
14

-40 13

12

11

tr
10 1

tig, 4.2: Surface area nomogram. !

I
*
t
 GROWTH AND DEVELOPMENT

------\ v
(t,/. ,/,
(J
_-_-----., =,-^(/ __-_^,(l
-______/ \
->
vJ a c -_>
U- uu =( .\.9/t)'
-otilfi; tildr
\B[';
rov
,w!to, t.o,
$[dr
O^
(Jl ."
oo-' s,d
nfl nfr ..,11 n\t ni1 nfi
o Q
d[l
v
@il 0n
(/ >-?
.V rCV
Hp
av

frl \1n \At

IV \
u IU ru
s'51 C,r
-CU 9cs 8"S
Birth 1 year 2i3 4 5

Head of humerus Great Head of radius

Shoulder 0 tuberosity
(3 rnths)

Elbow 0 Capitulum

Hamate (4 m) Triquentrum
Trapezium
Hand 0 Capitate (6 m) Ep- metacarpals Lunatum
Scaphoid
Ep. radius Ep. phalanges

Head offemur Great

Hip 0 trochanter
(9 m)

Knee
Head of fibula Patella
Ep. femur
and tibia

Foot lnt. cuneiform Mid cuneiform

Ext. cuneiform Ep. tibia
cuboid Ep. metatarsals Navicular

Fig. 4.3: Chronological order of appearance of osseous centers.

9 months : Head of femur 4 years : Lunate, greater trochanter of femur, head of

I year : Lateral cuneiform, epiphysis at the iower end fibula, middle cuneiform, navicular'
of tibia 5 years : Head of radius, trapezium, scaphoid, patella'
2 years : Greater tuberosity of humerus, capitulum,
epiphysis at the lower end of fibula Generally rwo carpal centers are present in the first year' The
3 years : Tiiquetral, epiphysis of metacarpals, epiphysis third center appears in the second year, and then one center
of phalanges, medial cuneiform, epiphysis of appears each year, except the last center (pisiform) that appears
metatarsals. by l0'h to l2'h year.
ESSENCE OF PEDIATRICS

4
w&
o^o $p
#ff nm DN

f) 7 B 9 10 11 yr

Unino head
and tuberosity

lnt. epicondyle Trochlea Ext.

Olecranon epicondyle

Trapezoid
Ep. ulna Pisiform

Union
ischium Ep. lesser
& pubis
trochanter

Tibial
tubercle

Ep. os
calcis

Fig.4.4: Chronological order of appearance of union of epiphysis with diaphysis

So for rough estimarion of bone age: tapezoid; and

Bone age = Number of carpal ossific center - l. Epiphysis at lower end of ulna.
Chronological order of appearance of union of epiphysis 7 years : Union of ischium and pubis of hip bone
8 years : Epiphysis of calcaneus
i
with diaphysis (Fig. 4.4): i
9 years : Tiochlea, olecranon t
6 yearc : Union of head and tuberosity of humerus; 10 years : Pisiform, epiphysis of lesser trochanter t
Medial epicondyle; 1 1 years : Lateral epicondyle, tibial tubercle. I
I
I
t{
 GROWTH AND DEVELOPMENT

Table 4.2: Time of Eruption and Shedding of Primary Teeth normal value is between 3'd and 97'h percentile. +1SD is equal
to 84'r'percentile, and -1 SD is equal to 15'h percentile; +2SD
corresponds rc 97'h percentile, and -2SD corresponds to 3'd
percentile. In order to know the growth (i.e., height or weight
Central incisor 6 71/z 6 7Vz
of a child), it is necessary ro compare againsr norr-nal standard.
Lateral incisor 79 7
\fHO has accepted growth chart developed by National Center
Cuspid 16 18 01/- 111/z
for Health Statistics, USA (NCHS) as international standard
First molar 12 14 10 101/z for growth of children.
Second molar 20 24 'l 1 101/z Each NCHS chart is composed of seven percentile curves-
lncisors Range +2 mo Range *6 mo 3',i, I 0,h, 25,h , 50,h, 7 5.h , 90,h, and 97,h percentilCs; 50,h percentile
Molars Range +4 mo is the median (standard). If the measurement of children falls
outside the area between the 3'd and 97'h percentile, it should
be regarded as abnormal unless proved otherwise. There are
separate growth charts for male and female. National Nutrition
Table 4.3: Time of Eruotion of Permanent Teeth
Council, Bangladesh (NNC) has developed a growth chart; this
and the NCHS growth charts are attached at the end of this
Central incisors 6-7 7-8 book along with \XrHO prototype growth chart.
Lateral incisors B-g
Cuspid 9-1 0 1 1-12
First bicuspids 10*12 10*1 1

Second bicuspids 1 1-12 10*12

First molars 6-7 6-7 EARTY CHITDHOOD DEVELOPMENT
Second molars 11*1 3 12-13
Period from conception to 5 years of age is early childhood.
lhrrd molars 1 7-21 1 7-21
This period is the key to subsequent growth, development,
From Massler and Sehour; Atlas of the Mouth, Chicago. American Dental Association and ulrimare productiviry.
A Child:
o According to the UN convention all human beings aged
Denlilion 0-18 years are children.
See Thbles 4.2 for time of eruption and shedding of primary
teeth and Table 4.3 for time of eruption of permanent teeth. The life of a child can be divided into several stagesl
o Intra-uterine: Embryo and fetus
GROWTH CHART o Neonate: Birth to 28 days of age
Growth chart is the most important tool in assessment of
o Early infancy: The first 6 months
growth of an individual chlld. A standard chart contains
r Late infancy: 6-12 months
weight for age, height for age, and weight for height. The head
o Toddler: l-3 years
circumference is included for first 3 years of life. The chart
o Pre-school Child: 3-6 years
depicts tSD, or percentile values at each age.
r Late childhood: 6-9 years
If the height or weight measurements in a large population
o Adolescence: 10-19 years

of normal children are arranged in a regular order, starting

from the lowest, going to the highest, a bell-shaped curve or
Gaussian distribution curve is formed.
In a rypical Gaussian distribution, median is expected to be
equivalent to mean; 68.30/o of the observations lie within one
standard deviation (SD) above or below the mean. A range
of 2SD around the mean include 95.5o/o of all observations.
Ifall the observations are divided into 100 equal parts, each
part represents a percentile. In percentile curve, 3'd percentile
curve denotes that 97o/o of observations are above and 2o/o
are below it.
The child on l0'h percentile on the height chart means that
9o/o of children fall below and 90o/o of children fall above the
height of that child in that population. Accepted range of
I m porlo nce of Eorly Childhood Developmenl
Early childhood is one of the most important periods in life
of a human being when the foundations for future learning
and qualiry of life take place.
Between the 6'h week and 5'h month of pregnancy, 100
billion cells grow in babyt brain. Some of these brain cells
are connected ar birth, but most are not. During the first 5
years of life, maximum connections occur. After 5 years of age,
connection occurs at a slower rate in the babyt brain.
At birth, 100 billion neurons form >50 trillion connections
in the brain (Fig.4.5). Each neuron is capable of forming up
to 15,000 connections or synapses. By 3 years ofage, about
1000 trillion synapses will have been formed. These synapses
ESSENCE OF PEDIATRICS

Birth
100 billion cells 3mo 3-5 yr 14 yr

L( L\, J--
$.j-,.='
,*
\'.'\\ )<^
:l
r' ,-'1 .
s-"\-pr
i -'*
l-: i\'
>+L\'.-
J' rF=
J-':
-f='<'\ ': .-
50 trillion 1000 trillion 100 trillion

Fig. 4.5: Age-wise number of neurons and synapses (mentioned at the bottom).

constitute wiring that allows learning/development to take ASSESSMENT OF DEVETOPMENT

place. About 80-90o/o of neuronal connections are completed
within 5 years. Development history and physical findings should be compared
with the achievements listed for normal children. For preterm,
one must take their corrected age into account. Development
Feolures on Developmenl of Broin assessment in infancy is important. If developmental delay is
o Brain is not mature at birth. identified in early stage of growth, the intervention can reduce
o Brain is changed by experience. long-term sequelae. Developmental delay is said to exist if the
o Human development depends on the interaction between child does not reach developmental milestones at the expected
nature and nurture. age. One should take into account the broad variations among
o The human brain has a remarkable capacity to change, but normal children. The process of development should be viewed
first 5 years of life are crucial. asan interaction between the child and environment, in which
. Any insult adversely impairs brain development. each have profound effect on other. Although severe disorders
o Malnourished children have better outcome when nutrition, can be recognized in infancy, speech impairment, hyperactiviry
as well as, stimulation is given. emotional disorders are difficult to diagnose before 3 or 4years,
o Principles of brain development: and learning disabilities are rarely picked up before children
start their schooling. Table 4.4 depicts assessmenr of different
o Development is a continuous process from conception
developmental aspects and warning signs at various ages.
to maturity.
The Denver Development ScreeningTest (DDST) assesses the
o Development depends on maturation and myelination
childt development in four separare segmenrs viz., gross moto!
of nervous system.
fine motor and adaptive, personal, social, and language functions.
o Sequence of development (craniocaudal) is same for all,
but rate of development varies from child to child.
Gross Molor Developmenl
Gross motor development involves control of the child over
WHEN TO SUSPECT DEVETOPMENT DETAY
his body. Development of the child is observed in the follow-
If a chiid: ing positions.

o Has no social smile by 3 months of age

o Has no neck control by 5 months of age
o Does not sit without support by 12 months of age
o Does not stand without support by 18 months of age
o Does not walks well by 20 months of age
o Does not speak 2-3 words sentence by 36 months of
o Does not tell self name by 48 months of age
r Does not have toilet contol by 60 months of age
Ventral Suspension
The baby is held in the prone position and then lifted up from
the bed, with the examiner supporting the chest or the abdomen
of the baby with the paim of his hand. In the newborn child,
age the head flops down. From the age of 4 weeks to 12 weeks,
the infant learns to lift and control his head in the horizontal
plane and then above the horizontal plane.

I
 GROWTH AND DEVELOPMENT

Table 4.4: Assessment of Different Developmental Aspects and Warning Signs at Various Ages

6 weeks Smiles, coos Stills to mother's voice, Follows face 90 degree, Primitive reflexes + r None is elicited
responsively startles at sudden stares intently head in line with trunk . Abnormal Moro's
noise when lifted . Persistent squint
6-9 months 6*7 mo: Changes 6 rno: Bears some on
. Slow social responses
6 mo: Enjoys bath, 6 mo: Responds to own
name. Speaks ma, da grasp palmar to index. legs, Rollingover, in
. Absence of babble
playing; boos and
Transfers objects hand prone head up wt. of . Persistence of hand
chews on items 9 mo: Mama, dada
to mouth hands regard
9 rno: Shows objects {double syllable). r Abnormal voluntary
to mother, pats mirror Understands'No' 9 mo: Pincer grasp, toot 7 mo: Crawls and pulls
hand grasp
rmage regard. Fixes pellet of to stand.
' Persistent priiritive
paper, follows fallen
reflexes
object
12 months Comes when called; IJnderstands some Throws ob jects, watches Shuffling gait like a bear . No tunefull babble
finds hidden objects; words, uses mama dada them fall, picks up cruises round holding . Holds objects close
waves bye-bye; gives with meaning'No' crumbs from floor. on to furniture. Walks to eyes
toys on request Pincer grasp, shakes one hand held pivots . lmmature gait
head, bangs two bricks when sifting . No sitting
together
t"
lB months Cup: Lifts, drinks, and Points to 3 body Neat pincer picking Walks well, carries toys/ . Drools no words
puts down. Self spoon parts. Obeys single of threads and pins. climb stairs; climbs into r Absent pincer grasp
i,
feeding. Pulls at dirty commands. Says 6 Scribbles using fisted chai r . Does not walk
I'
nappy. Does dusting, words, jargons grasp. Turns 2 or more
t sweeping Echoes, speech pages at a time. Builds
lI tower o{ 3-4 (2.5 cm)
t cubes
I
2-21/z years Plays alone, tantrums, Phrases of 2-3 words, Turns one page at a Pushes tricycle with . No speech
r . Unsteadv on feet
demanding. Dry by day, gives name, 50 words+, time, imitates a straight feet, walks downsiairs
line in both vertical and 2 feet per tread/ runs,
I puts on shoes, socks, naming games/ has
kicks ball, jumps on
l and pants. Turns door inner language horizontal and a circle.
handles. Uses spoon Unscrews lids. Makes the spot
and fork tower of 6-8 cubes

3-3 % years Coes toilet unassisted. Cives full name, sex. Mature pen grasp/ Stands on one leg for a . No phrases
Dresses/undresses with Counts to 10, 3-5 word copies + and 0. few seconds. Paddles r Persistent day time
minimum assistance. sentences. Correctly matches two tricycle, stairs adult style wettinglsoiling
Knows some nursery or more colors. Threads for ascent, jumps of . Clumsv
rhymes, handles knife large beads. Makes bottom step
and fork, plays with tower of 9
peers

4-5 years Wipes own bottom. Eats Cives address/age/ Matches 4 colors, 4 yr: Climbs trees and . Socially isolated
using knife and fork, telephone no. Counts Copies cross square and ladder, enjoys ball
. Unintelligible or
dresses (except ior tie upto10by4yr,2AbY by 5 a triangle. Draws a games ungrammatic speech.
and laces), imaginative 5 yr, knows 3 coins, recognizable man. . Unable to tell name
5 yr: Hops, skips, jumps
play, plays in groups, grammatical speech, or address.
off 3 steps, catches a
shares toys, obeys rules asks meaning of abstract ha ll
words.

Supine Position
The infant is placed in the supine position and is gently pulled
up by the arms to a sitting position. Movements of his head and
cuffature of the spine are obsen'ed. In the newborn infant, the head
lags behind completely and the back appears rounded. The infant
gradually gains control of his head berween the ages of 12 and
20 weetr<s, and the curvature of his spine becomes less prominent.
Prone Position
The examiner observes the baby while it is lying in the
prone position. At 3 months, the infant lifts his head and
upper part of forearms. By 5 months, he can lift his head
and greater part of his chest. Between the age of 5 and 8
months, he learns to roll in bed at first from back to side
and then from back to stomach. By the age of 8 months,
he crawls in bed.
Sitting
The infant iearns to control his body in the sitting position
from the age of 5 months onward. By the age of 8 months,
he can maintain steady, sitting position with straight back. By
the age of 10 months, he can pull himself up from the supine
to sitting position.
ESSENCE OF PEDIATRICS
Standing and Walking
From the age of 9 months, he makes early stepping move-
ments. By 10 months or so, the infants start cruising around
the furniture. By the first birthday, the child can stand without
support for 10 seconds. Child walks alone between 13 and 15
months of age. At the age of 15 months, most toddlers can
take several steps sideways, while pulling a toy attached to a
string or even take a few steps backwards.
Climbing Stairs
The child can climb up the stairs by the age of 2 years.
Pedaling Tricycle
The child can pedal tricycle between 2 and 3 years.
Fine Molor ond Adoptive Developmenl
The abiliry of the child to perform fine motor activity and
maneuvers is tested with the aid of particular test objects
such as (l) a red ring of diameter 6.5 cm, tied to a red string,
(ii) a pen torch, (ii) red cubes, (iu) a pellet, (z) cup with handle,
(zz) a spoon, (uii) a book of thick pages, (uiii) a red pencil,
(ix) paper, (x) wooden blocks, (xl) a doll, and (xii) mirror.
Hand-Eye (o-ordination
Red ring: At the age of 4 months, the infant tries to grasp
the red ring. Initially, he may overshoot, but eventually gets
it to his mouth.
Red cube: By the age of 5 months, he can reach for a
red cube, which is held within reach. He holds the cube in
his palms in a crude manner (palmar grasp) by 7 months of
age. Pincer grasp with the index finger and thumb apposition
is acquired by 9-10 months; by 12 months, pincer grasp
matures. He can transfer objects from one hand to the other
by 6 months.
Pellet: He develops finer and more coordinated hand skills
by the age of 9 months when he can scoop on a pellet crudely
with his palm. By 10 months, he can pick it up neady using
ends of his thumb and index fingers.
Hand to Mouth (o-ordination
o At 6 months child can take a biscuit in his mouth and chew.
r Feeding: This is assessed by observing his feeding behavior
with the spoon and cup. By the age of 1 year, the baby tries
to feed himself with a spoon but in the process he often
rotates the spoon and spills the contents.
By 15 months, he learns to feed himselfwith spoon with-
out spilling its contents.
By 18 months, he can feed himself from a cup with only
slight spilling.
Hand Skills
a Finger thumb opposition: 10-12 months.
a Picture book: At 13 months, the child can turn two or
three pages of a book at a time. By 24 months, the child
can turn pages of book one at a time.
Scribbling on paper with a pencil: The child scribbles
spontaneously by 12-24 months, copies, and draws a
horizontal or vertical line at 2 years, a circle by 3 years, a
cross (plus sign) by 4 years, and tilted cross (multiplication
sign) by 5 years. He can draw a rectangle by the age of 4
years and a triangle by 5 years.
Dressing: The child tries to remove his coat and attempts to
wear his socks or shoes without success by the age of I year.
At the age of 2 years, he is able to wear socks or shoes. By
3 years, the child can dress or undress himself completely
and often successfully buckles his shoes.
Making a tower of cubes: He can make a tower of two
cubes by 12-20 months, and of 4 cubes by 16-24 months.
Personol ond Sociol Developmenl
This domain relates to interpersonal and social skills.
Social smile and recognition of mother: The infant intently
regards the face of the mother or the examiner by the age of
I month. He smiles back (social smile) when the examiner
tries to speak to him or smiles to him at 2 months. The
child recognizes the mother by the age of 3 months. At the
age of 5 months, the infant enjoys watching his own image
in the mirror. He shows anxiety on meeting strangers as he
become 7-8 months old, at this stage he inhibits to 'no'.
Toys: The child resists if a toy is pulled from his hand by
the age of 7 months.
Mimicry: At 1 year of age, the child repeats any performance
that evokes appreciative response from parents and mimics
the action carried out by the mother at home.
Other interaction: He waves bye-bye at 9 months and
plays a simple ball game at 1 year; at 1 yea\ comes when
called, and knows his gender at 3 years. By 1 year, he can
understand where is papa. By 15 months, he can point to
objects in which he is interested. By 18 months, he follows
simple orders. At 2 year, when asked, names 2-3 objects,
points to 3-4 body parts.
Longuoge Developmenl
Hearing. At 1 month, he turns his head towards the sound
of a bell.
Sounds. By 6 months, he produces monosyllable sounds
like da, ma, but without attaching any meaning to them
(this is not true speech). Bisyllables (baba, mama) are spoken
:
by 9 months of age.
Understanding. By 10 months, he can understand spoken
speech and respond in an appropriate manner to verbal
:
requests, e.g., 'tVhere is daddy?' \
i
t
t
I
a I

GROWTH AND DEVELOPMENT

r Tiue speech. Usually by the age of I year, a child can use postpone his bowel movement. The child should be encouraged
two words (of his own jargon) with meaning for objects. to go to toilet by the age of 1 year, but the attiude of parents
His vocabulary increases during the second year. He uses to toilet training should be relaxed without undue anxiety.
6-20 words by the age of 18 months. Between the age of
2I and 24 months, he makes simple sentences using two EVALUATION OF DEVETOPMENT
or three nouns without the use of verbs and has a fairly
Dwelopmental delay is estimated to be present in 10olo of children.
good vocabulary of about 250 words when he is 3 years
It is possible to diagnose severe developmental disorders in early
old. He can give a coherent account of recent experiences
infanry. From history physical examination, and development
and events by the age of 4 years. Consonants such as g, I
assessment one should arrive at a conclusion that the child may
I, and r are learnt later than the other sounds.
or may not have developmental delay. By assessment of devel-
opmental quotient (DC), one can assume developmental delay:
Vision ond Heoring
Vision: At around 1 month, baby can fixate on his mother, Average age of attainment
x
DQ= 100
on an object at about 3-4 months. He can follow a moving Observed age at attainment
object around 1 year.
Hearing: Newborns respond to sound by startle, blink, A DQ below 70% is taken as delay and warrants detailed
quitting or change in ongoing activity. By 34 months, the evaluation.
child turns his head toward the source of sound. If we check
hearing by producing sound one and a half feet away from the Developmenlql Screening
ear, at 5-6 months the child turns his head to one side and Screening is a brief assessment procedure designed to identi$'
then downwards if a sound is made below the level of ears; children who need more intensive assessment. Some of the
one month later, he is able to localize sounds made above the common screening tools include the fbllowing.
level of ears. By the age of 10 months, the child directly looks l. Phataks Baroda Screening Test and Tiivandrum Devel-
at the source of sound diagonally. opment Screening Chart: Test items are arranged accord-
ing to age. The test has been validated at various Indian
Toilel Troining centers. It assesses gross moto! fine motot personal, social,
In the early months of life, the gastrocolic reflex is active and language, and adaptive areas. Development is assessed in
the infant tends to defecate after each feed. This reflex weakens children aging I-24 months.
by the age of 4 months. The following objects are needed for assessment: pen,
The infant can be placed on the toilet seat by the age of bell, transferring objects, pellet, ball, and a doll.
10 months since he can sit with good control by this age' The The development chart is given in Figure 4.6. Make sure
toddler can walk to the toilet by the age of 15-18 months your child sees, hears, and listens.
and is usually ready for starting toilet training. By the age of 2. Denver Development Screening Test (DDST)
2 years, the child is trainable. At 3 years, he can withhold and 3. Goodenough Harris Drawing Test

Make sure your child sees, hears and listens

Points to parts of doll (3 part)

Walks upstairs with help
Walks backwards
Says two words
Walks alone
Throws ball
Walks with help
Pats a cake
Fine prehension pellet
standing up byfurniture
Raises self to sittino oosition
Transfers ooJecIS nanci Io nano
Turn head to sound of bell/raitel
Rolls from back to stomach
Holds head steady
-
Eves follow PenrPencil
- -
bocrar slue -
1 --2 3 4 5 6 7 8 I 10 11 1213 14 15 16 17 18 19 2021 2223 z4months
-
Note: To use this chart, keep a pencil vertically on the age of the child. All milestones falling to the left oi the pencil should have
-
been achieved by the child.

Fig. 4.6: Trivandrum Development Screening Chart.

-
 I

ESSENCE OF PEDIATRICS

Table 4.5: Commonly Used Developmental Screening Test and Areas of lmpairment

Complete profile I . Bayley s Scale of ln[ant Developmenl rBSID-llr 1-42 mo Mental, motor, behavior
2. Stanford-Binet Intelligence Scale (fourth edition) 2-18 yr Mental, motor, behavior
I WPPSI-lll-UK lrd edition rWechrler Prcsr hool and Primary St ale of lnlelligencer 41 mo-6 year Verbal and performance
4. WISC-lll (Wechsler lntelligence Scale for Children) 6-1 6 yr Verbal and performance
5. Cessel rrevised) 1 mo-5 yr Mental, motor, behavior
6. Denver ll 0-6 yr Mental, motor, behavior
Cognition 1. Bayley's Scale of ln{ant Development (BSID-ll) 1-42 mo
2. Stanford-Binet lntelligence Scale (fourth edition) 2-1 I yr
J. WPPSI-lll-UK lrd edition (Wethsler Presthool and Primary Scalc ol Intelligencer 41mo*6 yr
4. Battelle Developmental lnventory: The Riverside Publishing Company 0-B yr
I. Bavley's Scale oI lniant Development ,BSID-llr 1--42 mo
2. Peabody Developmental Motor Scales: The Riverside Publishing Company O 7yr
'i. Transdisr iplinary Play-Based As*essment ,TBA': Paul H. Brookes Publishing
Company 0-6 yr
Adaptive/self help l. Baltelle Derelopmenlal ln\entory: lhe Riverside Publishing Company 0-8 yr
2. Vineland Adaptive Behavior 5caie 'VABS) 0-1 9 yr
3. Hawaii Early Learning Profile (HELP) 0 3yr
Socia l/emotiona I l. Child Behavior Checklist ,CBCL' 2-18 yr
2. Vineland Adaptive Behavior Scale (VABS) 2 mo-1 B yr
3. ( alilornia Preschool Sor ial Competenr y Sr ale 3-6 yr
4. Burks' Behavior Rating Scales 3-6 yr
5. Transdisciplinary Play-Based Assessment (TBA) 0-6 yr
Language/ l. Sequent ed Invenlory for Communit ation Development - Revi5ed iSICD' 4 mo4 yr
Commu nicalion 2. Preschool Language Scale-3 {PLS-3): The Psychological Corporation 0-6 yr
3. Clinical Evaluation of Language Fundarr-rental-Preschool: Charles E. Merrill 0-5 yr
Publi.hing Companr
4. Peabody Picture Vocabulary Test (PPVT-R): American Cuidance Service 2.5-40 yr
5. Test of Farly Language Development ,TLLD' 3 7yr
6. Transdisciplinary Play-Based Assessment iTBA): Paul H. Brookes Publishing 0-6 yr
Company.

Definitive Tests 6. Raynell Zinl<rnScale (for O-4year child): Global devel-

opmental delay assessment.
These tests are required once screening tesr or clinical assess-
ment is abnormal. They are primarily aimed at accurately define
7. ADOS (Autism Diagnostic Observation Schedule)
the impairments in both degree and sphere. For example, by
8. Conners Rating Scale for ADHD
giving scores for verbal, performance abilities, personal and
9. Rapid Neuro-Development Assessment (RNDA)
social skilis, these can be differentially quantified. The common
EARTY STIMULATION PROGRAM FOR
scales used include the following (Table 4.5):
1. of Infant Development (BSID-II)
Bayley's Scale DEVETOPMENT
(for l-42 month child): This scale provides three
Infants who show eariy signs of developmental delay need to
complementary tools for assessing the developmental
be provided opporrunities rhat promote body control, acquisi-
status of children, i.e., the motor scale (PDI lpsychomotor
tion of motor skills, language development, and psychosocial
development index]-gross and fine motor), the mental
maturity. Early stimulation is most beneficial if started in first
scale (MDl-cognition), and infant behavior record
few months of lilb and is also beneficial in early childhood
(behavior rating scale).
(<5 year). Early stimulation increases the synapses among rhe
2. Stanford-Binet Intelligence Scale (fourth edition) (for
neur-ons and improves development. Following method can be
2-lSyear olds): Performance based IQ test. Areas mostlv
used for earlv stimulation of infants below 6 monrhs:
tested are mental, motor, and behavior.
1. Visual
3. N7PPSI-III-UK 3'd edition (Wechsler Preschool and
Primary Scale of Intelligence) (for 4l mo-6year child): a. Hang mobile roy on right and left side
IQ test on verbal and performance. b. Talk to baby keeping face 6-8 inches ar.vay
4. \7ISC-III (Wechsler Intelligence Scale for Children) c. Hold mirror 7-8 inches from his eyes
(for 6*16 year old): IQ test on verbal and performance. d. Show child his hands and feet and move rhem togerher
5. IBAS (Independent Behavior Assessment Scale) (for 2. Tactile
2-9 year old): Adaptive behavior skill screen. a. Touch and handle in a loving, gentle manner
 GROWTH AND DEVELOPMENT

b. Hold baby while feeding CAUSES

c. Puff powder using cotton ball
1. Reduced intake
d. Pad body lotion
d. Provide furrylplastic toys a. Breast feeding: Insufficiency of breast milk
b. futificial feeding: Inadequate milk quantity for preterm
3. Auditory
baby, faulty feeding, inappropriate food selection
a. Sing to baby some poems or rhymes 2. Psycho-social: Low birth weight, emotional depriva-
b. Thlk or sing while giving a wash or dressing up tion, non-accidental injury, neglect, poverty, familial
c. Tie a bell to babys bed dys-harmony, psychosocial deprivation, poor parental
4. Sensory education, parental mental health problem
a. Hold baby in arms and walk around room 3. Defective digestion or absorption: Giardiasis, allergy
b. Genrly rub on head to cow's milk, pancreatitis, small intestinal disease,
c. Pat while you talk celiac disease, biliary atresia, cirrhosis of liver' protein
5. Bubbling losing enteropathy, cystic fibrosis, recurrent viral
infections.
a. -When child bubbles Ma, Pa, Ba, repeat the sound 4. Increased loss: Diarrhea, persistent vomiting, e.g', infan-
b. Sing a song while playing tile, hypertrophic pyloric stenosis, poll'uria
c. Talk to him while feeding or bathing 5. Organic fiseases
d. Repeat sounds a number of times
a. Neurologic: Mental retardation, cerebral, rumor, sub-
i dural effusion, degenerative brain disorders
I
L b. Cardiopulmonary: Congenital heart diseases, asthma,
t- suppurative lung disease
I c. Gastrointestinal: Malabsorption, lactose intolerance,
Failure to thrive (FTT) is a descriptive term rather than a
mechanical problem in sucking and/or swallowing, i.e',
r
F diagnosis. FTT is characterized by sustained weight loss, failure
in cleft palate/lip, GERD, hiatus hernia, Hirschsprung
I to gain weight, or a persistent fall in weight from the normal
t percentile of child. In practice, the essential comPonent in FTT
disease, cirrhosis, coeliac disease
t d. CKD, tubular acidosis, UTI
is sluggish weight gain rather than length/height or develop-
! ment. Of course, the latter may accompany poor weight gain.
e. Endocrinopathies: Hypothyroidism, diabetes mel-
t litus, diabetes insipidus, growth hormone deficiency,
I parathyroid disorders, adrenal insufficiency
f. Infections: Persistent diarrhea, tuberculosis
DEFINITION
t g. Inborn errors of metabolism
i 1. Attained growth h. Miscellaneous: Malignancy, collagen vascular disease
a. '$Teight <3rd percentile on NCHS growth chart i. Genetic conditions
b. \Weight 20o/o or more below ideal weight for height
c. Tiiceps skin-fold thickness (5 mm
DIAGNOSIS
2. Rate of growth
a. Depressed rate of weight gain
History
i) <20 g/d from 0 to 3 months of age
o Both the parents should be present during the interview and
ii) <15 g/d from 3 to 6 months of age
parent-child and parent-parent interaction must be critically
b. Downward crossing of >2 malor percentiles on NCHS assessed. Antenatal, natal, perinatal history; whether the
growth chart in a short time pregnancy was planned or unplanned, delivery was Preterm
c. Documented weight loss or term, and history of IUGR and intrauterine STORCH
infection should be taken.
Important points to be remembered: o Growth data: Evaluation of growth pattern from a road to
1. Label of FTT should not be given based on a single healrh card is most important.
observation, i.e., failure to gain weight or weight loss o Dietary history: Both present and past to evaluate caloric
should be observed over a period of time. and protein intake.
2. Usually children <3 year or maximum up to 5 year are o Social and family history: Evaluation about the influenc-
included in this definition. ing factors, e.g., financial constraints, lack of relatives and
3. Small size alone is not an adequate criterion for confirm- friends, psychiatric problems or drug abuse in family, marital
ing FTTI as constitutional and genetic factors may resuit problems, serious illness or death in family.
in short stature. o Evaluation of milestones of development.
ESSENCE OF PEDIATRICS

Table 4.6: Approach to Failure to Thrive Based on Age
Birth to 3 mo Perinaral infection, tUCR. LBW, feeding difficulties,
psychosocial, gastroesophageal ref lux, i nborn errors
of merabolism, cysiic filtrosis
3-6 mo Psychosocial FTT, feeding difficulties, milk protein
intolerance, gasrroesophageal reflux, inborn errors
of metabolism, renal tubular acidosis, cystic fibrosis,
AIDS
7-12 mo Psychosocial FTT, delayed weaning, intestinal
parasites, gastroesophageal reflux, renal tubular
acidosis
12+ mo Psychosocial FTT, gastroesophageal reflux
Age-wise diagnostic considerations have been discussed in
Table 4.6, and Figure 4.7 deptcts algorithmic approach to the
evaluation of failure to thrive.
general and systemic examination is a must, neurodevelopmen-
tal assessment should be performed. Label of FTT ,hould ,,ot
be given, based on a single observation i.e. failure to gain weight
or weight loss should be observed over a period of time. Small
size alone is not an adequate criterion for confirming FTI a,
constiturional and genetic factors may result in short starure.
Look for specific behavioral patrern including unusual Look
for specific behavioral patrern including unusual watchfulness,
decreased vocalization, lack
ofcuddliness, head banging, rocking
movements and rumination; signs of abuse and neglect, signs
of vitamin and nutrient deficiencies.
lnvestigoiions
For initial evaluation, complere blood count with ESR, urine
and stool examination, urine culture and sensitiviry tuberculin
test, BCG test, blood urea and serum crearinine; x-ray if TB
or physical abuse is suspected.

Physicol Exominolion o Hematological: CBC, ESR, with comment on peripheral

blood film
Detailed anrhropomerry including length/height, weight, head o Biochemical: urea, crearinine, BUN, electrolytes, Ca, pO,,
circumference, upper/lower segmenr rario, skin fold thickness alkaline phosphatase, SGPT
and mid-arm circumference should be taken. A thorough o Urine and stool for RArfE and urine for culture

Trial oJ feeding (2 weeks)

Sucking/
swallowing
problems

ry
CNS disorder
Abnormal stools tr Caloric
&

insufficiency I
i
Feeding Neuromuscular g Feeding problem,
Malabsorption
. persistent
Poor utilization problems disorder H
lnfection,
Thyrotoxicosis Gl abnormalities @#
Hypothyroidism,
diarrhea (GER), CRF Respiratory
. Giardiasis CNS problem insufficiency,
. Celiac disease Congenital heart
' Cystic fibrosis disease.

Fig' 4.7: schematic diagram showing evaluation of failure to thrive (FTT).

a
 GROWTH AND DEVELOPMENT

o Tirberculin test
o X-ray chest
r Echocardiogram 1. ParthasarathyA (ed). IA? Textbooh of Pediatriu 4'h ed. New Delhi:
o lJltrasound abdomen, barium study and jejunal biopsy, Jaypee Brothers, 2009.
o Investigation for coeliac disease, 2. Illingworth F.S. The Normal Child 9'h ed. New York: Churchill
o Endocrine: T4, TSH, prolactin, GnRH, TRH tests, growth Livingstone, 1990.
hormone provocation test Ghai OP (ed). Essential Pediatrics 7'h ed. New Delhi: CBS Publish-
o Skeletal survey, bone age, x-ray of pituitary fossa
ers,2009.
Proceedings on 2nd SAARC group meeting on reduction on
r Karyotype (girls)
childhood neuromorbidity. 7-11 Aug 2002, NI India Institute of
Medical Sciences, New Delhi -110029, India.
Inseley J, 'Wood B (ed). A Pediatric Vade Mecum 10'h ed. Singapore:
TREATMENT PG Publishing Pte Ltd., 1993.
6. Gupte S. 7he ShortTextbook of Pediatrics l}'h ed. New Delhi: Jaypee
1. Dietary managemen* For planning and supervising, an Brothers Medical Publishers (P) Ltd., 2004.
experienced dietician should be involved. A 2-week Sundara Lingam, David R Harvey. Manual of Child Deuelopment.
trial feeding is given either orally or through tube Churchill Livingstone, 1988.
feeding and daily monitoring of weight and total 8. Roland S. Basic Deuelopmental Screening: 0-4 Years 3'd ed. Iliing-
calorie consumed. At the end of 2 weeks, the child is worth: Blackwell Scientific Publications, 1987.
9. Michael Swash, Michael Glynn. Hutchisoni Clinical Methods: An
reassessed.
Integrated Approach to Clinical Practice 22"d ed. Saunders, 2007.
2. Developmental stimulation should be done. Parents
10. Behrman RE,, Kliegman RM, Jensen HB, Stanton BF. Nelson's
should be actively involved in the management. Textbook of Pediatrics 18'h ed. \XlB Saunders Co,2007.
3. Psychiatric advice if behavioral problem is there. 11. Kalra V. Practical Pedianic Neurologlt 2"d ed. New Delhi: Arya
4. Treatment of specific cause(s). Publication, 2008.
 CHAPTER

Nutritional Problems

Chopter Conlents

lh jamine rVilamin B I detiriency........... ............78

Table 5.3: WHO Classification of Under-nutrition

Protein-energy malnutrition (PEM) is a range of clinical-

pathological condition arising from lack ofvarying proportions Svmmetrical edema No Yes"

of protein and calories, encountered most frequently in infants Weight for height SD scoreb -2 to -3 SD score < 3

and young children and is usually associated with infection.

(measure of wasting) (70-79"/' of expected') (.<70o/" ol expected)

Height for age SD score'' -2 to -l SD score < -3

(measure for stunting) (85-89% ofexpected') (<85% of expected)
CTASSIFICATION a. This includes Kwashiorkor and marasmic Kwashiorkor

A. W'eight for Age Classifications: measure the degree of b. SD score (Z) =

Observed value - Expectec value (median)

under-nutrition. I Standard deviation

c. Median (501. percentile of NCHS standards).
i. Gomez classification: Refer Thble 5.1.
ii. \Teicome Tlust classification: Refer Table 5.2.
B. Iil7HO Classification of Under-nutrition: Refer Thble 5.3. _ 10kg-8.5kg
2
Finding out Z-score of a PEM patient:
_ 0.75
Example: A boy of 75 cm height weighing 7 kg, find out his Now,
weight for height Z-score (WHZ).
- Observed wt - Expected wt (median wt at 75 cm)
Median Reference Veight at75cm - 3rd ISD
Centile Reference \X/eight at75cm
SD=
_ 7kg-10kg
0.75
Table 5.1: Comez Classification =-4
C. Classification Based on Z-Score: Refer Thble 5.4.
76-90 Crade L mild malnutrilion
61-75 Crade ll, moderate malnutrition
Table 5.4: Classification of PEM Based on Z-Sct-rre
<60 Grade lll, severe malnutrition

5.2: Welcome Trust Classification Weight for age Z-score (WAZ) -3 to < -2 Moderate under-weight
Table
<-3 Severe under-weight
Weighr ior heighr Z-score (WHZ) -, Moderate wasting
=
60-80 Kwashiorkor Under-nutrition .1j. Severe wasting

<60 Marasmic Marasmus Height for age Z-score rHAZt - t lo < -2 Moderate stunting
Kwashiorkor <-3 Severe stunting
 NUTRITIONAL PROBLEMS

Table 5.5: Classification of PEM Based on BMI r Wt for Ht median (V/FIM) <70olo
,r 'i(/t for Ht Z-score <-3 SD
,r Bipedal edema (kwashiorkor, marasmic kwashiorkor-
>20 Normal edematous malnutrition)
18.5-20 Marginal o <6 months: A child should be classified as severely malnour-
17-18.4 Mild nralnutrition ished if he/she has one or more of the followings:
.l
6-1 6.9 Moderate malnutrition ,r Visible wasting
<.1 6 Severe malnutrition r \XGIM <70o/o or <-3 SD
'r Bipedal edema
D. Etiological Classification
Edema in all children is graded using the classification below:
i. Primary malnutrition-due to primary/ lack of food.
ii. Secondary malnutrition-due to chronic disease or Crade + Mild: bof h ieetyankles
causes other than lack of food. Cracle t r Moderate: hoth feet plus lower legs, hands, or lowerarms

E. Classification Based on BMI: Refer Thble 5.5. Crade r++ Severe: generalizecl edema including leel, legs. hand..
. drnrs, and iace

PRINCIPAT DIFFERENTIATING FEATURES Presence of any of the conditions listed in Thble 5.7 requires
facility-based inpatient rreatmenr.
Refer Thble 5.6 for principal differentiating features of protein-
energy malnutrition.
GENERAL PRINCIPLES OF MANAGEMENT
ASSESSMENT OF SEVERE ACUTE Severe acute malnutrition cases without complication should
MATNUTRTTTON (SAM) have community-based management (using local alternatives,
e.g., pushti', halwa, khichuri, etc.), and cases with complica-
o 6-59 months: A child is classified as severely malnourished tion need facility-based managemenr (using formulae F-75 and
if he/she has one or more of the followings: F-100); after stabilization phase, patienr is transferred to have
> Mid-upper arm circumference <110 mm communiw-based treatmenr.

Table 5.6: Principal Differentiating Features of Protein-Energy Malnutrition

Usual age 0-3 rr 1-3 yr

Essential features

Edema None *Lower legs (symmetrical), sometimes face or generalized.

Wasting *Cross loss of subcutaneous fat, 'skin & bone' Sometimes hidden
appearance Baggy pant appearance at bultock
Muscle wasting Obvious Sometimes hidden
Crowth retardation Obvious Sometimes hidden
Mental changes Usually apathelic. quiet L sually irritable. moaning. also apathetic.
Variable features
Appetite Usua lly good Usually poor
Diarrhea Often lpacl or presenl' Often (past or present)
Skin changes Seldom Oft en-difiuse depigmentation, flaly pa i nr dermatosis.
r-rlcerations
Hair changes Seldom Often sparse, straight silky depigmentation grey or reddish
Biochemistrylpathology
Serum albumin Usually normal or lon, 'I r)w
Lrver Dropsy *Normal or atrophic
'FdnI r ndnges
Endocrine changes
Serum cortisol Markedly elevated Elevated
Serum growth hormone Normal Elevated
Serurn insulin Normal Lorv. norml I

Somatomedin Decreased Det reared

T., T, Decreased Dec reased
T.SH Decreased Det reased
-These are the most characteristic or useful distinguishing features.
ESSENCE OF PEDIATRICS

Table 5.7: Signs that Warrant Facility-Based lnpatient Treatment B. Keep the child warm
C. Antibiotics
Edema Crade +++
D. Two-hourly feeds, day and night
Marasmic kwashiorkor: a chilcl wirh severe
wasting (MUAC <1 10 mm or WHM <7O"k or
Step 2: Treat or Prevent Hypothermia
WHZ <-3) and edema
Appetite/anorexia Poor appetite or unable to eat Ifthe axillary temperature is <36.0" C or 96.8" F:
Vomiting Persistent vomiting (>3 per hour) A. Re-warm the child: Either clothe the child including head,
cover with a warmed blanket and increase the ambient
Temperature Fever (39" C or 1O2.2" F axillary) or
hypothermia (<35" C or 95" F axillary) temperature with available but safe heat sources or put
Respiratory rate Rapid breathing according to lMCl
the child on the mothert bare chest (skin-to-skin) and
>60imin for children <2 months cover them.
>50/min for children 2 12 months B. Feed the child as in step 7
>Aolmin for children 12-59 months
C. Give antibiotics
Anemia Severely pale {severe palmar pallor) with or
without difficult breathing
Step 3: Treat or Prevent Dehydration
lnfection Exlensive lnfection requiring parenteral
trealmenI It is difficult to estimatedehydration status in a severely mal-
Alertness Very weak, apathetic, unconscious, fitting/ nourished child using clinical signs alone, because the clinical
convu lsions signs of dehydration may already present in severely malnour-
Hydration status and
Dehydration based primarily on a recent ished children or are also signs of septic shock. Dehydration
dehydrating diarrhea history of diarrhea, vomiting, fever or may be over-estimated in a marasmic/wasted child and under-
.
sweating, not passing urine for last 1 2 hours
estimated in a kwashiorkor/edematous child. Therefore, assume
and on recent appearance of clinical signs of
dehydration as reported by the caregiver that children with watery diarrhea may have dehydration.
Other crileria ln[anls <6 mo with severe acute malnutrition
1. Give all children with watery diarrhea ReSoMal (Rehydra-
Caregiver requests inpatient care tion solution for malnutrition) 5 ml/kg every 30 minute
Physician's i mpression for first 2 hours orally or by NG tube, then
2. at alternate hours for 4-10 hours ReSoMal 5-10 ml/kg/hr
(the exact amount to be given should be determined by
how much the child wants, and stool loss and vomiting).
Principles of treatment:
Also give F-75 at alternate hours during this period until
o Ten steps of management the child is rehydrated.
r fteatment of associated conditions 3. After rehydration, continue feeding F-75.
o Emergency treatment
Step 4: (orrect Electrolyte lmbalance
Ten Sleps of Treolmenl
Until stabilization give:
Ten steps and time frame for the management of a child with
severe acute malnutrition are summarized in Figure 5.1 and 1. Extra potassism 34 mmol/kg/d
have been described below. 2. Extra magnesium 0.4-0.6 mmol/kg/d
3. \(hen rehydrating, give low-sodium rehydration fluid
Step 1: Treat/Prevent Hypoglycemia (ReSoMal)
4. Prepare food without salt
Assume all severely malnourished children as hypoglycemic and
treat accordingly. Hypoglycemia is considered if blood sugar Do not treat edema with a diuretic.
level is <3 mmol/L or 54 mgldl.
A. If the child is conscious give: Step 5: Treat or Prevent lnfection
1. 50 ml bolus of 100/o glucose or sucrose solution (5 g Give routinely broad-spectrum antibiotics on admission:
or I rounded teaspoon of sugar in 50 mi or 3.5 table 1. If the child appears to have no complication: Amoxicil-
spoons water), orally or by NG tube. lin oral 15 mg/kg 8-hourly for 5 days or Cotrimoxazole
2. Then feed starter dlet F-75 every 30 minutes for 2 oral (trimethoprim 5mg/kg and sulphamethoxazole 25
hours. mg/kg) l2-hourly for 5 days.
If the child is unconscious or convulsing give: 2. If the child is severely ill (apathetic, lethargic, or looking
10%o glucose (5 ml/kg) IV followed by 50 ml of 10olo sick) or has complications: Ampicillin IMiIV 50 mg/kg
glucose or sucrose by NG tube. Then give starter F-75 6-hourly for 2 days then Amoxicillin oral 15 mg/lg S-hourly
as above. for 5 days and Gentamicin IM/IV 7.5 mglkg once daily
a
t
:
I
i
t NUTRITIONAL PROBLEMS

for 7 days. Do not give second dose of Gentamicin until
the child is passing urine.
3. If the child fails to improve clinically by 48 hours or
deteriorates after 24 hours, or if the child presents
with septic shock or meningitis: Antibiotics with a
broader spectrum may be needed (e.g., Ceftriaxone
50-100 mglkgl d IV/IM once daily along with or without
Gentamicin).
Step 6: (orrect Micronutrient Deficiencies
Give Vitamin A orally on day 1 unless there is definite evidence
that a dose has been given in the last month.
In the rehabilitation phase (gradual transition is needed
to avoid heart failure): Replace starter formula F-75 with
the same amount of catch-up formula F-100 every 4 hours.
Then, increase each successive feed by 10 ml, some feed
remains unconsumed. The point when some remains uncon-
sumed after most feeds is likely to occur when intakes reach
about 30 ml/kg/feed (200 ml/kg/d).
After the transition phase: Give frequent feeds (at least
4-hourly) of unlimited amounts of catch-up formula F-100.
This will lead to energy and protein intakes of 150-220
kcal/kg/d and 4-6 g protein/kg/d, respectively. Formula
F-100 contains 100 kcal and 2,9 g protein per 100 ml.
F-100 should gradually be replaced by khichuri/halwa
containing equivalent amount of calories and protein.

>12 months 200/000 IU If weight gain is:

.100,000
6*12 months IU
o Poor (<5 g/kg/d): child requires full reassessment.
O-5 months 50,000 lu o Moderate (5-I0 glkgl d): check whether intake is appropri-
ate, or infection has been overlooked.
t Give daily for at least 2 weeks: r Good (>10 g/kg/d): praise the staffand mother.
t
' o Multivitamin supplement (without iron)
Formula for calculadng wt gain:
i o Folic acid 1 mg/d (give 5 mg on day 1)
ir o Zinc 2 mglkgld
'W't W2-\(1 x100
a Copper 0.3 mg/kg/d (if available) gain (in g/lig/d) =
I Vl x Number of days from rVl to \72
I a Elemental iron 3 mg/kg/d but only when gaining weight
i
r (start on rehabilitation phase when gaining weight) may be \(/here, V1 = initial or lowest wt in kg
t continued for about 3 months or more. \72 = wt in kg on the day of calculation
T

I
l; Step 7:Start Feeding Cautiously Step 9: Provide Sensory Stimulation and Emotional Support

The essential features offeeding during the stabilization phase are: Provide:
I
o Small, frequent feeds of low osmolarity and low lactose o Tender loving care (smiling, laughing, patting, touching,
I o Oral or nasogastric feeds (no parenteral preparation) etc.)
It
o Energy intake of -100 kcal/kg/d o A cheerful, stimulating environment
o Protein intake of 1-1.5 g protein/kg/d o Structured play therapy 15-30 min/d
o Total fluid intake through feeds should not be >130 nn/k/d o Physical activity as soon as the child is well enough
(100 ml/kg/d if the child has severe edema, which means edema o Parental/caregiver involvement when possible so that the
ofthe legs, hands, and face) special care is continued at home
o If the child is breast-fed, encourage to continue breast-
feeding but give the prescribed amounts of starter formula
(F-75) ro make sure the childs needs are met. Formula F-75 Step 10: Prepare for Discharge and Follow'up after Recovery
contains 75 kcal, and 0.9 g protein per 100 ml. Criteria for discharge:
child:
Feeding frequency:
o 'S7HM VHZ equals or more
equals or more than 80% or
than -2 SD
1*2 2-hourly a Edema is resolved
3-hourly a Gaining weight at a normal or increased rate
6+ 4-hourly a Child eating an adequate amount of nutritious food that
the mother can prepare at home
All infections and other medical complications have
Step 8: Achieve (atch-up Gtowth
treated
Rehabilitation phase is started with the return of appetite, loss a Child is provided with micronutrients
of edema, about I week after admission. o Immunization is updated
 I
ESSENCE OF PEDIATRICS

Mother/caregiue r:
o Knows how to prepare appropriate foods and to feed the
child
o Knows how to make appropriate toys and to play with
the child
o Knows how to give home rreatment for diarrhea, fever, and
acute respiratory infections and how to recognize the signs
that she/he must seek medical assistance
o Follow-up plan is completed.
Helminthiasis
Albendazole 200 mg for children aged 12-23 months, 400 mg
for aged >24 months; or Pyrantel pamoare 10 mg/kg, single
dose (any age).
Diarrhea
Improve with cautious feeding. If giardiasis, treat with Met-
ronidazole 7.5 mglkg TDS for 5 days.

Follow-up:
Dysentery
o At 1 week after discharge
o Regular check-ups should also be made at 1 week, 2 week, If stool contains visible blood, treat with Ciprofloxacin l0
I month, 3 month, and every 3 months thereafter until mg/kg/dose l2-hourly for 3 days. If lactose inrolerance, trear
\fHM >90o/o or WHZ >-1 SD, at which poinr the child withF-75, yoghurt, lactose-free infant formula (e.g., rice-suji),
is discharged. re-introduce milk feeds gradually.

Tuberculosis
Treolmenl of Associoted Condilions
Manage according to the national guidelines.
Vitamin A Deficiency

If child shows any eye signs of deficiency, give vitamin A orally Emergency Treolment of Shock ond Very
on days 1,2, and 14: Severe Anemio
o Children 0-5 months: 50,000 iU
o Shock in Severely Malnourished ftildren
Children 6-11 months: 100,000 IU
r Children >12 months: 200,000 IU Severe dehydration and septic shock are difficult to differ-
entiate on clinical signs alone. Signs of septic shock may
If corneal clouding or ulceration, instill Chloramphenicol eye include:
drops 2-3 hourly for 7_70 days, instill Atropine eye drops ( l o/o)
three times daily for 3_5 days, cover with eye pads soaked in o Signs of dehydration, but without a history of watery
saline solution and bandage. diarrhea
o Hypothermia or hypoglycemia
o Children with dehydration will respond to IV fuid, while
Dermatosis
those with septic shock and no dehydration may nor
Dermatosis is treated with zinc supplementation, applying a respond.
gauge soaked in potassium permanganare solution over affected
Diagnosis of shock is based on the following criteria:
areas (keep for 10 minutes rwice daily); candidiasis should be
treated with Clotrimazole, used twice daily for 2 weeks, oral o Lethargy or unconsciousness
candidiasis by Nystatin 100,000 IU four times daily. Affected o Cold exrremiries
area should be kept dry. o Plus either
o Slow capillary refill (>3 sec)
Phase r \feak or fast pulse (160/min for 2-12 months old; 1401
Stabilization Rehabilitation
min for 1-5 year olds.)
Steps: D 1-2 D 3-7 Wk 2-6
1. Hypoglycemia ____________> Treatment:
2. Hypothermia -------------> 1. Oxygen inhalation
3. Dehydration 2. 10%o glucose (5 ml/kg) by IV
4. Electrolytes 3. IV fuid at 15 ml/kg over t hour
5. lnfection
-> a. funger lactate with 5%o dextrose; or
6. Micronutrients No iron With iron
7. Cautious feeding b. Half-normal saline with 5olo dextrose, or
8. Catch-up growth c. Cholera saline, or
9. Sensory stimulation d. Any other fuid except dextrose in aqua
10. Prepare for follow-up
4. Measure and record pulse and respiration rates every 30
Fig. 5.1: Time frame with respect to the ten steps of the minutes
management of severe acute malnutrition. I
5. Antibiotics
a
I
1
 I

NUTRITIONAL PROBLEMS

5. Keep the baby warm Table 5.9: Electrolyte-Mineral Solution (Add 20 ml of this
solution to 1000 ml of milk feed)
e If condition is irnlrroaed (pulse and respiration rates
f"tt)
1)A
r Repeat IV at 15 ml/kg over t hour, then Potassium chloride

t Switch to Oral or NG rehydration with ReSoMal 10 I flpotassrum crtrate 8.1

ml/kg/hr at alternate hours with starter F-75 for up Magnesium chloride 76

to 10 hours, then Zinc acetate 8.2
,; Continue feeding with starter F-75 Copper sulphate 1.4

o If there is No improaeTnent (pulse and reryriration Water: make up to 2500 ml

rates remains high)

o Maintenance IV fluid 3 ml/kg/hr while waiting for
blood Table 5.10r F-75 and F-l00 when Cooking Facilities are
,r 'W.hen blood is available, transfuse fresh whole biood U navailable
at 10 ml/kg slowly over 3 hours

o If there are signs of oaer-dehydration or cardiac

failure during treatment (breathing inneases by 5 Dried skimmed Dried skimmed milk 259 B0g
breaths or more/min and pulse inuetses by 25 or more milk
beats/min): Stop infusion to prevent the child's condi- Sugar 1009 sOg

tion worsening. Vegetable oil J5 ml /U ml

Electrolyte-mi neral mix 20 ml 20 ml
1000 ml
.l
Water: make up to 000 ml
Very Severe Anemia in Malnourished ftildren
Dried whole Dried whole milk 35g 1108
If Hb < 5 g/dl or packed cell value 45o/o or breathlessness milk
and Hb 5-7 gldlt Sugar 100g 509
o Blood transfusion: 10 ml/kg over 3 hours Vegetable oil 20 ml 35 ml
r Frusemide 1 mg/kg IV at start of transfusion Electrolyte-mineral mix 20 ml 20 ml
1000 ml 1000 ml
If signs of cardiac failure present: tansfuse packed cells Water: make up to

5-7 mllkg. Full-cream Full-cream cow 300 ml B80 ml

cow milk milk (fresh)
Monitoring: Sugar 100g 75g
o Stop transfusion if any of the following signs develop: Fever, Vegetable oil 20 ml 20 ml
itchy rash, dark red urine, confusion, shock. Also monitor Electrolyte-mi neral mix 20 ml 20 ml
R/R and pulse. Water: make up to l0O0 ml 1000 ml
r Oral Iron 3 mg/kg/d for 3 months in all cases of anemia
(mild, moderate, and severe), but start only after the begin-
ning of weight gain in the child.
Recipes:
Refer Thbles 5.8, 5.9, and 5.10 for preparing ReSoMai solu- Vitamin A is important for the function of rod cells in the
tion, electrolyte-mineral solution, and formulas (F-75 and retina, and for the integrity of epithelial tissues and mucous
F-100), respectively. membranes. Its deficiency can be described under the follow-
ing sub-headings:
1. Ocular: Xerophthalmia
\Wt{O classification of xerophthalmia (1982)

Table 5.8: ReSoMal Solution (lt contains approx. 45 mmol Classification code Clinical description
Na, 40 mmol K) XN Night blindness
X1A Conjr-rnctival xerosis
X1B Bitot spot
\Vater rboiled and cooledt 850 ml
xL Corneal xerosis
WHO-ORS (new formu lation) One 500 ml packet
}L3A Corneal ulceration/keratomalacia
5Lr8Jr 20g involving <ll3 of the corneal
Electrolyte-mineral solution 16.5 ml surface
 I

ESSENCE OF PEDIATRICS

X3B Corneal ulceration/keratomalacia drowsiness, and bulging of the fontanel, diplopia, papilledema,
involving >Il3 of the corneal cranial nerve palsies, and other symptoms suggesrive of brain
surface tumor (pseudotumor cerebri).
XS Corneal scar Chronic hypervitaminosis A appears after ingestion of exces-
XF Xerophthalmic fundi (white sive doses for several weeks or monrhs. An affected child has
retinal lesion) anorexia, increased irritabiliry tender swelling of bones, alope-

2. Extraocular cia, seborrheic dermatitis, fissuring of the corners of mouth,

o Dry scaly skin, especially over rhe outer aspect of the
limbs, called follicular hyperkeratosis (phrynoderma).
Toad skin is now beiieved to be due to deficiencv of
essential fatty acids.
Increased susceptibility to infections due to squamous
metaplasia of respiratory, urinary tract and vaginal
epithelium; renal and vesicle calculi may also occur.
Growth failure.
TREATMENT
Vitamin A (VAC-HP 2 lacs, Retinol forte cap 50,000 IU):
supplementation should be done on day-l, day-2, and on
day-I4 (3 doses) according to the following dose:
r <6 months: 50,000 IU
o 5-<12 months: 100,000 IU
r >l year: 200,000 IU
PREVENTION
o Two doses of Vitamin A should be given in measles (i.e., on
D-1 & D-2); single dose should be given in persistent diar-
rhea (i.e., on D-1) and in severe malnutrition (i.e., on D-l).
o Distribution of Vitamin A capsules (VAC-HP) should be
done among the communities with low vitamin A sratus,
once in every 6 monrhs up to 6 years of age, being started
after 6 months of age.
o Education on local sources of the viramin or carotenoids.
o Programs to encourage breast-feeding, increased consump-
tion of dark green leaf, vegetables, 'lal-shak, colored fruits
(e.g., papaya, jackfruits, carrors, etc.). Egg, livea fat offish
and meat, cod liver oil, mola fish, etc. should be given to
their children when parents can afford.
Note:
1. The eye lesions are completely reversible at the stage of conjunctival
or corneal xerosis, but once keratomalacia develops (rhis can proceed
unsuspected behind the photophobic childt closed eyeJids), blindness
is inevitable.
2. Early conjunctival signs respond to supplementation of vitamin A
within 2 weeks. Impaired dark adaptation is a sensitive measure of
continued deficiency.
increased intracranial pressure, and hepatomegaly. Craniotabes
and desquamation of the palms and soles are common. X-ray
reveals hyperostosis of the middle of the shaft of the long bones.
Thiamine is warer and alcohol soluble, fat insoluble. Active
coenzyme form of thiamine is thiamine pyrophosphate (TPP),
which acts in oxidative decarboxylation (conversion of pyruvic
i
acid with eventual entry inro Krebs cycle via aceryi coenzyme A
and that of alpha-ketoglutaric acid with entry into Krebs cycle
via succinyl coenzyme A). In thiamine deficiency, utilization i
of pyruvic acid is decreased, and therefore pyruvic acid and
;
lactic acid accumulate in the tissues.
CtINICAt FEATURES
The classical deficiency syndrome of thiamine is beriberi. It is
of two rypes in older children:
o Dry beriberi affects rhe nervous sysrem. Its symptoms include
irritability, polyneuritis, calf muscle renderness, difficulty
in standing from sitting position, sluggish tendon reflexes,
constipation.
o \7et beriberi is characterized by palpitation, tachycardia,
dyspnea, and edema.
Infantile beriberi may be of three qpes:
1. Cardiovascular type: It is characterizedby tachycardia,
dyspnea, cyanosis, and vomiting (onset-very acute).
2. Aphonic type: It has a subacute onset, hoarse cry, which
is followed soon by aphonia. Terminal manifestations
include dyspnea, puffiness, and pitting edema.
3. Neurologic typ€: Fearures inciude vomiring, rremors,
convulsions, ptosis, nystagmus, and extraocular muscular
paralysis. It starts in infants of 6-12 monrhs age and runs
a chronic course.
SOURCES OF THIAMIN
Good sources of thiamine include unmilled cereals, pulses,
oil-seeds, groundnuts.

TREATMENT

As soon as the diagnosis of thiamine deficiency is made, the I

Acute hypervitaminosis A may occur in infants after ingesting child should receive 10 mg thiamine inrravenously. In the 1
100,000 mg or more. The symptoms are nausea, vomiting, next 5 days, he should be given l0 mg of vitamin rwice daily 1
t
t
I,
 NUTRITIONAL PROBLEMS

followed by 10 mg daily oral1y for next 6 weeks. A breast- Clinicol Feqtures

feeding mother should also receive thiamine therapy.
Table 5.11 depicts the daily requirements, sources, features in
deficiency, and daily therapeutic doses of Vitamin B-complex,
E, and K.
SCURVY
Vitamin C deficiency results in faulry collagen tissues, inciuding
those of bone, cartilage, and teeth; the intercellular substance
of the capillaries is also rendered defective.
The peak incidence is at 6*24 months. At first, there are
vague symptoms of irritability (baby cries on being handled)
and loss ofappetite. Then there occurs generalized tenderness,
especially in the legs, resulting in pseudoparalysis. The legs
assume the typical frog position (hips, knee semi-flexed and
feet rotated outward). Sometimes, subperiosteal bleeding can
be palpated at the end of femur. Vhen the teeth are erupted,
bluish purple, spongy swellings of the mucous membrane
usualiy over the upper incisors are seen. A'rosary' at the cos-
tochondral junction (scorbutic beads are sharper than rachitic
rosary) and a depression ofthe sternum is noted. Perifollicular
hemorrhages, commonly found in lower thigh, followed by
petechiai hemorrhages in the ankles and feet, then ecchymosis
(found commonly in lower extrerniry) producing woody legs.

Table 5.11: Daily Requiremenis, Sources, Features in Deficiency, and Daily Therapeutic Doses of Vitamin B-complex, E, and K

Riboflavin (8,) lnfant: 0.5 mg Milk, liver, kidney, meat, Angular stomatitis, cheilosis, 5-1 2 mg
Children: butter, eggs, green and yellow glossitis, magenta tongue,
1.1 mg vegcldbles seborrheic dermatitis, keratitis,
photophobia
Nicotinic ar id rNiat jn B r Infanl: o mg Meal. fish. wholc mill. cereals, Pellagra uharat terized b1 1 00-500 mg
Children: put'er. coflee. regetables and dermatitis, diarrhea, and
.l
2 mg irLr i ts dementia
Pyridoxine (B,,) lnfant: 0.4 mg Meat, liver, egg yolk, pulses, Dermaliti:. t heilosi., angular . Pvridoxine-dependent
Childrer.r: vegelahle:. wheal. nuls stomatitis, glossitis, peripheral seizures: 50-100 mg PO/IM/
0.9 mg ncuruprlhy, hypot hromic lV; mainlenance dose 50-i00
anemia. convulsion tgrd
. Dietary deficiency: 5-15 mg/d
tor J-4 wk. then 2.5-5 mgid
. Drug induced neuritis: 1 mg/
k9d PO, IM, lV qd
Folic acid lntant: 50 pg Fish, leafy vegetables, liver, Features of megaloblastic lO I5mq
Children: kidney. milk. eggs anemia, slomalilir. heef red
200 pg tongue, and pallor

Cyanocobalamin (8,.)
lnfant:0.3 prg Liver, kidney. medt. cheese. cgg, Meg.rloblaslit rnemia afLer ln neurological involvement,
Children: milk. Creen vegetables usually gdstrec lomy; malahsorption; 1 mg vil. B .hould be given
1.5 pg do not tontain B nutritional deficiency in strict lM dailv for 2 wk, lhen I mg
vegetarian diet. lM monthly life-long. Oral
Neurologit a I manileslal ions ther.lpy not recommended ior
include subacr-rle combined uncertainty of absorption.
degeneration of spinal cord
lniant:5 lU Vegetable oils, egg, butter, . Premature infant may have l5-25 lU,)4 hr till J7 \ k in
Children: whole green cereals, nuts, peas low serum level oftocopherol Preterm I BW in[ant.
9lu with the development of
hemoll,tic anemia at the age
of 6 10 \4,k
. Anemia of kr.r,ashiorkor
. Focal necrosis of striated
mr,rscle and muscle weakness
. Increased platelet
adhesiveness

1-2 mg Leaiv vegetables, cheese, egg . Hemorrlragic di:order oi the . Mild case: 1-2 mg/24 hr
yolk, liver, etc. nei,r born ora lly
. Unr onlrollable hemorrhagit . Severe case: 5 mg/24 hr
disorder md) occur parenterally
ESSENCE OF PEDIATRICS
Hematuria, melena, orbital or subdural hemorrhages may be
found. \(ound healing is delayed.
Diognosis
X-ray knee is commonly done and the changes include (l)
ground glass appearances of the shafts of the bones with
pencil-point thinness of the cortex, (ii) a dense white line of
calcification (Fraenkel line) appears at the epiphyseal ends of
the long bones, and (iii) ofrcn there is a subepiphyseal zone
of translucency (fracture zofie-area of rarefaction) due to
an incomplete transverse fracture immediately proximal and
parallel to Fraenkel line. The lateral spurring (projection) of
the white line is known as corner sign. The epiphyseal centers
also have a ground glass appearance and are surrounded by a
white ring-ringing of epiphyses (\Timberger sign). Periosteal
elevation from subperiosteal bleeds may later form callus.
Other tests include estimation of ascorbic acid concentration
in the buffy coat layer of centrifuged blood.
Differenliol Diognosis
Suppurative arthritis, osteomyelitis, ITP, Henoch-Schonlein
purpura, leukemia.
Treolmenl
o Rapid recovery can be obtained with oral Ascorbic acid,
200 mg per day for several weeks. There is no advantage
in parenteral administration. The administration of 3-4
ounces of tomato juice or orange juice daily will also
quickly produce healing; despite, ascorbic acid treatment is
preferable. Clinical response occurs rapidly within 24-48
hours. Improvement in radiological picture takes a week or
two. Subperiosteal hemorrhages are likely to take months
to disappear.
o After scuny has been cured (assessed clinically and radiologi-
cally), the normal requirement of vitamin C (35-50 mg per
day) should be given in the form of drug or diet.
Prevenlion
r Scuny is prevented by a diet rich in vitamin C; citreous
fruits (e.g., guava, amloki, tomato, orange, etc.) and green
vegetables are excellent sources.
o Formula-fed infants should be supplied with 35 mg of
ascorbic acid daily. Cow milk contains only about a quarter
of vitamin C conrent of human milk, and this is further
destroyed by boiling.
o Breast-feeding should be encouraged.
. Lactating mothers should have a daily intake of 100 mg
of vitamin C.
Note:
1. Humans are unable to synthesize vitamin C within their bodl'.
2. Sculi,y is rare in the breast-fed infants unless mother suffers lrom
subclinical avitaminosis C.
RICKETS
fuckets is characterized by bone lesions that are caused by
failure of osteoid, the growing cellular matrix of bone, to
become mineralized. The under-mineralized bone is less rigid,
and growing, remodeling bone bends and rwists abnormally.
Mineralization failure of mature bone is called osteomalacia.
Clinicol Feolures
Characteristic physical findings are bowing of the legs, thick-
ening of the costochondral junction (rachitic rosary), knobby
prominence of the wrists and knees, and growth failure. In
infants, craniotabes (thinning of the skull bones) and fractures
are common.
The radiographic findings include metaphyseal widening,
cupping, and fraying (irregular) with generalized osteopenia in
the shaft. There occurs increased distance between metaphysis
and epiphysis.
Vorionls of Rickets
Rickets may be caused by vitamin D deficiency secondary to
decreased intake, decreased absorption, or decreased metabolism
of vitamin D, or lack of adequate calcium and phosphorus for
normal bone mineralization caused by either deficient mineral
intake or increased losses by the kidneys.
Nutritional Rickets
Lack of vitamin D in the diet results in decreased calcium
absorption in the intestine. Hypocalcemia stimulates PTH
secretion, which then causes increased reabsorption ofcalcium
from bone and decreased renal reabsorption of phosphorus.
o Etiology: Despite fortification of milk and cerea.ls with
vitamin D to provide the minimum requirement of 400 IU
daily, nutritional rickets still occurs in children who have low
vitamin D intake. Often other factors are present, such as
decreased exposure to the sun due to dark skin pigmentation,
urban living conditions, and the winter season.
o Clinical features: Nutritional rickets may develop within 2
months in breast-fed infants whose mothers have osteomala-
cia. Florid rickets appear toward the end of first and during
second year oflife. In nutritional rickets, the serum calcium
level is low (or it may be normalized by secondary hyper-
parathyroidism), the phosphorus level is low and the alkaline
phosphatase level is high due ro acrive bone resorption from
secondary hyperparathyroidism.
o Treatment
o Vitamin D is given in a dose of 15,000 pg (or 600,000
IU) PO or IM. If there is no sign of healing line in skia-
gram taken 3-4 weeks after therapy, the same dose can be
repeated. If there is no response within 3-4 weeks of the I
second dose, investigations for refractory rickets should t
I
be initiated.
I
I
tt

o 0.5-2 of I,25 - dihydrorTcholecalciferol (dicaltrol) can
p,g
also produces healing demonstrable on roentgenograms
within 3-4 weeks except in cases of vitamin D refractory
rickets.
o Calcium should be supplemented' Adequate sunshine
rv exposure should be ensured.
t. o After healing is complete, dose of vitamin D, is lowered
r to l0 mg/d.
r ondary hyperparathyroidism (unllke of nutritional rickets).
,
r Rickets Associated with Abnormal Metabolism of Vitamin D
I,
t Vitamin D-Dependent Rickets Vitamin D-dependent rickets, an
k autosomal recessive disease, is caused by absence of the renal
enzyme 1 a-hydroxylase, which converts 25 -hydtoxyvitamin D.
to the active metabolite 1,25-dihydroxyvitamin Dr.
o Clinical features: Vitamin D-dependent rickets manifests
in the same way as nutritional rickets. The serum calcium
level is low (or normal), the phosphorus level is low, and
the PTH level is elevated. Usually 25-hydroryvitamin D,
levels are normal, while 1,25-dihydroxyvitamin D, levels
are low.
r Therapy with physiologic doses of 1,25-dihydroxyvitamin
D. (i.e., 10 mg/d) is curative.
Chtonic Renal Disease One of the factors leading to renal
osteodystrophy (the complex of osteopenia, osteitis fibrosis,
and rickets) associated with chronic renal failure is decreased
activity of 1cr,-hydroxylase in the kidneys' Therefore, treatment
of renal osteodystrophy includes 1,25 dlhydtoxyvitamin D..
Chronic livet Disease Chronic liver disease, such as biliary
atresia and other cholestatic diseases, may result in rickets
from decreased intestinal absorption of vitamin D, a fat-soluble
vitamin, or decreased Z5-hydroxylation in the liver itself' The
treatment of choice is 25-hydroryvitamin D, or 1,25-dihy-
droxyvitamin Dr.
ftronicAnticonvulsantTherapy Phenobarbital and phenytoin
cause increased metabolism of vitamin D and may be associ-
ated with rickets. Increased demand for vitamin D can be
satisfied by increasing the daily intake of 1000 to 2000 IU/d
for children on chronic anticonvulsant therapy whose exposure
to sunlight and nutritional intake is marginal.
Rickets due to Mineral DeficiencY
Familial Hypophosphatemic Rickets oI Vitamin D-Resistant
Rickets The most commonly encountered non-nutritionai
form of rickets, mode of inheritance is X-linked dominant'
AR and sporadic forms have also been reported. Defects occur
in the proximal tubular reabsorption of phosphate (causing
phosphaturia) and in conversion of 25 (OH)D to 1,25(OH)rD'
o Clinical features: They usually present with bowing of the
legs (their mothers may also have bowing or short stature)
at the age of walking. Tetany is not present. Profound
myopathy, rachitic rosary, and Harrison groove characteristic
NUTRITIONAL PROBLEMS
of calcium-deficient rickets are not evident. These children
develop a waddling gait, bowing of the lower extremities,
coxa vara, genu varum, genu valgum, and short stature.
Pulp deformities in teeth may be present.
Investigations: Normal or slightly decreased serum calcium
Ievel (9-9.5 mgldl; 2.25-2.35 mmol/L) and moderately
reduced serum phosphate level (I.5-3.0 mg/dl; 0.48-0.96
mmol/L), elevated alkaline phosphatase, no evidence of sec-
Urinary phosphate excretion is large.
Treatment:
o Oral phosphate should be given to replace renal losses.
Phosphate can be given as Phosphate capsule (Nutrafos)
or as Joulie solution (dibasic sodium phosphate 136 glL,
and phosphoric acid 58.8 glL-5 ml dose to be given 5
times daily).
o Providing a vitamin D analogue is important for com-
plete bone healing and prevention of secondary hyper-
parathyroidism.
o Vitamin D, 2000 IU/kg/d or 1-25(OH)rD 50 ng/kg/d
has been usid effectively. The latter has got short half-life.
Care must be taken to avoid vitamin D intoxication with
hypercalciuria, nephrocalcinosis, hypercalcemia, and re-
nal damage.
o If appropriate doses of vitamin D failed to heal rickets
and if serum phosphate is not reduced, metaphyseal dys-
plasia should be considered.
o Corrective osteotomies should always be deferred until
rickets appears healed radiologically and until the serum
alkaline phosphatase level is in the normal range.
Prematurity Also known as metabolic bone disease of
Rickets of
the premature infant. Infants born prematurely have decreased
bone mineralizationcompared to full-term infants, because they
do not benefit from the major skeletal accretion of calcium and
phosphorus occurring in utero during the last trimester.
o Clinical features: Human milk and standard infant formulas
that provide adequate calcium and phosphorus for the full-
term infant are not adequate for the needs of the premature
infant, as evidenced by the occurrence ofsevere osteopenia
and rickets, which results in fractures in some premature
infants. In these infants, serum calcium levels are normal,
and phosphorus levels are low, 1,25-dlhydroxlwitamin D,
levels are elevated, probably because of the hypophospha-
temic stimulus.
o Treatment Forti$'ing human milk or formulas with addi-
tionai calcium and phosphorus results in improvement in
bone mineralizarion and healing of fractures and rickets.
.}TYPERVITAMINOSIS D
Ingesting excessive amount of vitamin D results in signs and
symptoms similar to those of idiopathic hypercalcemia- SYmP-
toms develop after 1-3 months of large intake of vitamin D;

ESSENCE OF PEDIATRICS
these include hypotonia, constipation, polydipsia, polyuria.
Hypercalcemia and hypercalciuria are notable. Aortic valvular
stenosis, hypertension, rerinoparhy, and clouding of cornea
and conjunctiva may occur.
The urine may show proteinuria. \7ith continued excessive
intake, renal damage and metastatic calcification occur. X-ray of
long bones reveals merastatic calcification and osteopetrosis.
Hypervitaminosis D can be prevented by careful evaluarion
of doses ofvitamin D. TLeatment include discontinuingvitamin
D intake and decreasing intake ofcalcium for severely affected
infants, aluminium hydroxide by mouth, corrisone, or sodium
versenate may be used.
Zinc is a component of over 100 metalloenzymes and par-
ticipates in many biological processes. Zinc functions as an
intercellular hormone contributing to the regulation of the
cellular growth and also implies on nucleic acid metabolism
and protein synthesis. Poor physical growth is a documented
feature ofzincdepletion in preschool and school-aged children.
Delayed sexual maturation is also a prominent fearure.
Meat, liver, cheese, eggs, nuts, legumes are good sources.
Cereal diets decrease its absorption due to high phytates.
REQUIREMENT
Preterm,I.5 mglkgld; term neonare, 1 mg/kg/d; children, 10
mg/d; adolescent,12-15 mg/d; lactating morher, 20 mgld.
CTINICAL FEATURES
The typical syndrome of zinc deficiency in humans consists of
growth retardation, hypogonadism, and anemia. Other major
symptoms include diarrhea, hair loss, anorexia, dermatitis, and
impaired immune function. Pica as well as altered tasre sensa,
tion may also present. \found healing is delayed, eye lesions
include phorophobia, blepharitis, and corneal opacities.
In pregnanry: premarure delivery, IUGR, neural tube defects.
Zinc is relatively non-toxic, but acute ingestion of large
amount may cause liver and kidney failure, copper deficiency
syndrome.
INVESTIGATION
Plasma zinc will be low-<6 mmol in severe deficiency, 6-9
mmol in moderare deficiency.
TREATMENT
0.5-l mg eiemental zinclkgld for several weeks or months.
Note:
l. Ztnc supplementation during pregnancy and lactation results
in better motor development, and more playfulness in low
birth weight infants.
!
2. 20 mg elemental zinc per day reduces the number of watery
stools and duration in diarrhea. Zinc supplementation also
reduces the frequency of pneumonia and malaria.
3. Zinc supplementation improves immunocompetence,
growrh, and psychomotor development of child.
4. It can be used in persistent diarrhea and PEM as additional
therapy.
v
It is an autosomal recessive disorder caused by an inabiliq' to
absorb sufRcient zinc from gut. Symptoms occur after weaning
from breast milk to cow milk.
o Clinical features
o Skin: Vesiculobullous, dry, scaly, psoriasiform skin le-
sions symmetrically distributed in perioral, acral, peria-
nal areas and in cheeks, knees, and elbows.
o Hair: Reddish tint with alopecia.
o Eyes: Photophobia, conjunctivitis, corneal dystrophy.
o Others: Chronic diarrhea, stomatitis, growth retardation,
delayed wound healing, bacterial infection, candidal in-
fection.
a Diagnosis: Decreased plasma zinc level.
a Tireatment
30-50 mgZn per day in infants and may be increased up
to 150 mg/d. If left untreated, death may ensue within 3
years because ofsevere PEM or superinfection.
Local application as cream is used in ulcer.
Obesity means excess fat, not merely excess weight. The rerm mosr
commonly used to quantifz obesity is body mass index (BMI).
Body weight is nor a reliable criterion for defining obesiry.
\Teight in lg
BMI =
(Height in m)2
BMI >85 percentiles for age is considered as risk for obesiry
while those >95 percentiles for age are obese. \Teight for
height >i20% is diagnosed as obesiry.
ETIOLOGY
Constitutional: Environmenral factors (over 90olo of all cases).
There is always imbalance between energy expenditure and
energy intake. Genetic factors playvery srrong role. In general,
obesity runs in families, parental obesity bring a strong risk
factor. Other contributing factors are decreased physical activ-
iry difference in food choices.
Pathological:
r Endocrine: Hypothyroidism, growth hormone deficieno'
(GHD), Cushing syndrome :
\
\
1
a
 NUTRITIONAL PROBLEMS

!
, not more than 0.5 kg per week. Avoid very low calorie diet
, a HJpothalamic Head injury, infection, brain tumor
(400-800 calories) as they derange metabolic parameters.
> a Genetic: Laurence-Moon-Biedl syndrome, Prader-Willi
Reduction of fat intake by avoiding high calorie and fried
I syndrome, Beckwlth-Wiedemann syndrome
foods, reduction of cooking fat with increased fiber is gen-
Drugs: Corticosteroids, sodium valproate, estrogen
I eraliy sufficient. Soft drinks, sweets, ice cream, fast foods,
etc. should also be restricted. Liberal intake of vegetables
CtINICAt EVATUATION and fruits should be encouraged.
I Life-style modifications: Physical activities should be
t The most important clinical feature that distinguishes patho-
increased. Exercise helps in long-term weight loss by increas-
I logical from constitutional obesiry is the height (Thble 5.12).
ing energy expenditure. Active games with fun, briskwalking
Children with pathological conditions are short, usually have
>30 minutes daily, competitive sports, aerobic exercise, active
developmental delay, while those with constitutional obesity
involvement in household work should be encouraged.
r tend to be taller than expected for age and family background.
Behavior modification and social support is very impor-
! History should include details of dietary intake, activiry pat-
tant in achieving sustained weight loss through long-term
r terns, mental development, and school performance. Examina-
changes in eating and behavior. Entire family should be
tion should include accurate measurement of height, parent's
i heights (calculation of midparental height); genital examination,
convinced of the need for weight loss.
t Mefications should be avoided in children. Orlistat is tried.
; pubertal staging, dysmorphic features, facies, fundus. Stretch
Metformin in insulin resistance, leptin in leptin deficiency,
penile length (SPL) should be measured since penis is often
I and octreotide in hypothalamic obesity can be given.
buried in abdominal fat. Micropenis may be found in growth
I hormone deficiency or in some syndromes. If childt height is
Monitoring weight loss shouid be monitored by taking
monthly weight.
t much higher than midparental height, most probably obesity
Specific treatment of any pathological cause of obesity
is constitutional; investigation should be minimum.
should be undertaken if found.
tr Surgery may be needed in massive obesiry. Laparoscopic
t COMPLICATIONS gastric banding is the procedure of choice and is directed
i at reducing gastric capacity.
F
Cosmetic problem, hypertension, diabetes mellitus, osteoar-
> throsis, heart failure, respiratory failure.
PREVENTION
I
p
INVESTIGATIONS Focus the child with risk factor for obesity-obese mother,
I
I obese father, obese sibling(s), maternal age over 35 years at
i r Blood glucose birth, single child, single parent. Start dietary and behavioral
r Serum lipids intervention in that focus group.
o Thyroid hormones-T3, T1, TSH Encourage breast-feeding, avoid bottle feeding and early
o Other investigations may be done according to clinical weaning.
evaluation. Parental education: Educate parents to (l) avoid force
feeding, (z) keep fat intake moderate, (iii) encourage
TREATMENT increased physical activity, (lz) ensure good company, and
(z) express affection or approval through ways other than
o Dietary therapy: It forms the backbone of weight loss food.
attempts. Intake of 1200-1800 calories depending upon
the age of the individual with 30-40o/o restriction is recom-
mended. In child with moderate to severe obesity, calorie
restriction should be moderate, aiming for weight loss of Management of severe malnutrition: a manual lor physicians &
other senior health workers. \fHO, Geneva, 1999.
Physical status: the use & Interpretation ofAnthropometry Report
Table 5.12: Comparison of Features of Constitutional and of a 'WHO expert committee. WHO, Geneva, 1995.
Pathological Obesity 3. Manual on Nutrition Tiaining for Pediatricians, special part (Draft).
MHAF\# & [CMH.2OO2.
4. Molla MR. Pediatric Diagnosis d, Tieatment 2"d ed. Dhaka, 2007.
Obesity dlstribution Generalized Usually central
5. Bangladesh Nutritional Blindness study. Hellen Keller International
Crowth (Height) Accelerated Retarded & Institute of Public Health Nutrition, 1985.
Development Normal Delayed 6. Hay \flf (ed). Current Padiatlc Diagnosis 6 Tieatment 14'h ed.
Stamford: P-H International Inc., 1997.
Bone age Advanced Retarded
7. Gupte S. Tbe Shor Textbook of Pediatics 86 ed. New Delhi: Jaypee
D-vsmorphism Atrsent May be present Brothers,199B.
ESSENCE OF PEDIATRICS

Behrman RE, Kliegman RM, Jenson lHB. Nekon Textbooh of t3. Thlukder K, Thlukder M Q-K. Manual on managemenr of severe
Pediatrics 18'h ed. rW4B Saunders Co, 2008. malnutrition in children. ICMH, 2000.
9. Bhatnagar S, Taneja S. Zinc and cognitive development. British t4. Castilloduran C, Cassoria F. Tiace minerals in human growth and
f Nutr 2001:85 Suppl 2:SI139-45. development. J Pediatr Endouino I Maab 1999 ;12(5) :589-60 1.
10. Black RE. Therapeutic and preventive effects of zinc on serious 15. Parthasarathy A (ed) . IAP Textbooh of Pediatrics 4'h ed. New Delhi:
childhood infectious diseases in developing countries. Am J Clin Jaypee Brothers, 2009.
Nutr 1998;68 April 2:4765-95. 16. ForfarJO, Arneil GC (ed). Tixtbook of PaediatricsT& ed. Edinburgh:
i1 Chang, Gerson BS, Subramaniom S. The role of copper, molyb- Churchill Livingstone, 2008.
denum, selenium and zinc in nutrition and health. Clin Lab Med 17_ Ghai OP (ed). Essential Pediatrics 7'h ed. New Delhi: CBS
1998;18(4):573-85. Publishers, 2009.
12, Black RE, Sazawal S. Zinc and childhood infectious diseases; mor- t8. National Guidelines for the management of Severely Malnourished
bidiry and mortaliry. Bntish J Nutr 2001;85 Suppl 2:S125-9. Children in Bangladesh. IPHN, DGHS, MoHFW GOB, May2008.

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 CHAPTER 6
Community Pediatrics

Chopter Conlents
Prevention in community pediatrics....................................85 Assess and classify the sick child age 2 months Treatment of a sick child aged 2 months to

lnternational Agencies and Child HeaIth..................,.........85 i0 5 years.......,........ ............................88 5 years in outpaiient health facility ............................... 9B

Adoption and care of 0rphans..........................,...,..............86 Assess, classify, and treat the sick young infant Counsel the m01her......................,...,....................................100

Millennium development goals .......................,....................87 aged t day up io 2 m0nths..............................................93 Teach the mother to treat local infections at h0me............103

lntegrated management of childhood Summary of urgent pre+eferral treatmenis for

illnes (lMCl).......... ..............,..............81 the sick child aged 2 months up t0 5 years.................97

The term communiry pediatrics includes the earlier disciplines

known as public health, preventive pediatrics, social pediatrics, Vast differences exist in the child healthcare problems
and community medicine. It focuses on the health needs of and services among the developed and developing
the community as a whole. nations.
Primary prevention: It is directed at avoiding disorders before ll. Misdistribution of doctors and poor doctor-patient ratio:
they begin, e.g., vaccination, clean water supply, and proper Approximately, 80o/o population lives in rural and tribal
sewage disposal, etc. areas, while 20% population lives in urban and semi-urban
areas; thus, doctors alone cannot cope up with the child
Secondary prevention: It indicates the recognition and care demands from difficult rural/tribal areas.
elimination of the precursors of the diseases, e.g., screening 111. Inadequate training in pediatrics for the doctors as well
programs for thyroid disorders, anemia, blindness, etc. Screen- as nursing and paramedical stafr does not make them
ing is the search for an asymptomatic illness in a defined feel confident to offer child care services in a large com-
population, which is usually performed for the purpose of muniry.
treatment. lv. Meager allocation of.budget for child care services,
Tertiary prevention: It indicates the measures for halting of the improper prioritization and maldistribution of available
disabilities arising from established disease, e.g., physiotherapy funds.
to prevent contractures in patients with chronic neurological
r
I diseases.
i Modern preventive medicine has been defined as the art and
i-
T science of health promotion, disease prevention, disabllity
I

r limitation, and rehabilitation.

> Social medicins It is the study of humans as a social being woRrD HEALTH ORGANTZATION (WHO)
tI in their total environment. The focus is on the health of the
It is a specialized, non-political health agency of the United
community as a whole.
I Nations, with headquarters in Geneva.
l, Epidemiology: It is the scientific study of factors influencing a 7'h April is celebrated every year as "'Worid Health Day'.
the health, disease, and the control of disease in populations, a A world health day theme is chosen evety year to focus
II rather than in individuals. attention on an aspect of public health.

r
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a
ESSENCE OF PEDIATRICS
Objectives
The preamble of the constitution states, "Health is a
state
of complete physical, mental and social well-being, and not
merely the absence of disease or infirmiry. The enjoyment of the
highest attainable standard of health is one of the fundamental
rights of every human being without being discriminated on
the basis of race, religion, and poiitical beliei and economic
and social condition."
World Health Organization is unique among the unspecial-
ized agencies in that it has its own constitution, own governing
bodies, own membership, and own budget. It is part of but
not a subordinare to the United Nations.
UNICEF
United Nations International Children's Emergency Fund
(UNICEF) is one of the specialized agencies of the United
Nations, established in 1946.In the early years, UNICEF and
\(/HO worked togerher on urgenr problems such as malaria,
tuberculosis, and venereal diseases. Later, it covered such fields
as maternal and child health, nutrition, environmental sanita-
tion, health centers and health education, and programs that
would directly or indirectly benefit childt health.
Greater arrenrion is being paid to the concept of the 'whole
child".
Conlents of Services of UNICEF
o Child health
r Child nutririon
o Family and child welfare
o Education formal and non-formal
In short, UNICEF activities cover programs assisting in child
survival, protection and developmenr, intervenrions like immu-
nization, improved infant feeding pracrices, child growth
monitoring, home-based diarrhea managemenr, drinking water,
environment sanitation, birth spacing, education of girls, and
income generating activities for women.
Adoption is essentially a social process by which the recipro-
cal need of childless parenrs and an orphan (parent-deprived)
chlld is satisfied.
Adoption is also a means through which one can ensure rhe
following important children's rights accepted by the United
Nations:
1. Inherent right to life survival and development
2. fught to name and nationality
3. Child's interesr being always a primary consideration
4. Special protection from physical harm and neglect
5. No discrimination through universal brotherhood
6. Social security through education, employment, and
earning
7. Special arrenrion to disabled children
MAKING UP A DECISION TO ADOPT
Childless couples have a desire for parenrhood.
PREPARATION FOR ADOPTION
The ideal age difference between at least one parenr and the
baby to be adopted should be not more rhan 40 years so thar
they have enough time and physical strength on their side to
look after the baby till it becomes a young adult and legally
a major.
MEDICAT SCREENING
\X4rile doing the medical screening,
the doctor should remember
that this baby should be examined and certified fit.
Clinical examination consists of anthropomerry, sysrem
examination, detection of any gross deficiency disorders and
overt or convert anomalies, clinical clues to metabolic disorders,
and physical and mental milestones evaluation. Standard inves-
tigations that are expected to be done are hemogram, urinalysis,
tuberculin test, X-ray chest, stool examination for parasites,
tests fo-r VDRL, HIV Hepatitis B, TORCH titers, and any \
other indicated tests with special reference to hypothyroidism, i
hemolytic anemia, chromosomal anomalies, and metabolic i
screening tests for mental retardation like phenylketonuria,
galactosemia, aminoaciduria, etc.
\
MEDICAI. CARE
Vast majority of orphans are vulnerable to diarrhea, sepsis, FTJ
malnutrition, frequent attacks of acute respiratory, gastrointes-
tinal, and skin infections. They may be frankly marasmic, and
lack of immunity increases morbidity and mortality among
them. In the management of sick orphans, three acrions are
of utmost importance: (z) immunization against Hepatitis B
and administration of other vaccines available in addition to
standard schedule; (ii) urgency of action when even slighdy
sick; and (iii) early use of better antibiotics even if prima facie
it may appear an irrational and expensive proposition. By
introducing simple inputs such as IV fluids, oxygen therapy,
and higher antibiotic therapy in the orphanage itself prior to
transferring to a big hospital, a large number of such babies
can be saved.
In post adoption care, in addition to problems of general
health and standard immunization schedule, an exrra eye should
be kept on feeding and sleep parrerns, motor development
pattern, speech and language development, which may appear
delayed for some time initially.
 I

COMMUNITY PEDIATRICS

FOLLOW.UP AND POST ADOPTION
COUNSETING
Follow-up after adoption is necessary for advice and care in
the following situations, which are likely to arise in some of
the babies:
r Sudden change crises, where the baby (and parents) may
take long time to cope up with the suddenness of change
in environment, food sryles, and surrounding strange faces
resulting into different types ofreactions like anxiery rejec-
tion, or aggression, or any combinations of these.
r A behavior crisis is likely manifest after first few years and
may be as a result of pampering and overprotection (or
even a covert rejection) by one or both parents and other
family members.
o Communication and identity crisis may come up at any
age if the childt sense of security is not well-ensured by
the parents and immediate family members.
o Goal
Crisis is likely to occur in a small number of cases where
the young adults may face discrimination in getting jobs,
marriage, etc.
in the year 2000 and set to be achieved by 2015, these provide
a framework for the entire international community to work
together. If these goals are achieved, half will cut world poverry
tens of millions of lives will be saved, and billions more people
will have the opportunity to benefit from the global economy.
The eight MDGs are:
Goal l: Eradiate extreme poverty and hunger
Goal 2: Achieve universal primary education
Goal 3: Promote gender equality and empower women
Goal 4: Reduce child mortality: Reduce by mo-thirds between
1990 and 20\5, by reducing the under-5 mortaliry
rate and infant mortality rate, and by improving the
proportion of 1-year-old children immunized against
measles.
Goal 5: Improve maternal health
6: Combat HWiAIDS, malaria, TB and other diseases
Goal 7t Ensure environmental sustainability
Goal 8: Develop a global partnership for development

TELLING ABOUT ADOPTION At a Glance: Goal 4

Encouraging, insisting, and helping the parents to tell the child
about his or her adoption is most vital post-adoptive follow-up
.1
action. Every child who is adopted must be told that he or she Under-5 mortality rate (per 000 Iive births) 146 48
is adopted by the parents themselves howsoever it may appear lnfant mortality rate (per 1000 Iive birthsr 92 31

difficult for them.\fhen a child comes to know the fact from Proportion o[ l-year-old children immunized 54 100
sources other than his parents, it can cause such a severe trauma against measles (7o)
(of betrayal or breach of trust) that it might even ruin relations
between the parents and the child forever, completely defeat-
ing the very objective for which the adoption was undertaken.
Later the age of the child when the trauma happens, worse are
the consequences for the parents. It is therefore necessary and
appropriate to encourage all adopting parents to tell the child Assess and classify the sick child aged 2 months up to 5
as early in life as possible when the child can comprehend years (Annexure I).
the concept of biological and adoptive parenthood. If done 11. Assess, classifii and treat the sick young infants aged 1
between the ages of 6 and 10 years, most children take into day up to 2 months (Annexure iI).
their stride and future problems can be averted. 111. Urgent pre-referral treatments for the sick child aged 2
months up to 5 years (Annexure III).
1V. Tieatment in the outpatient health facility of the sick
child aged 2 months up to 5 years (Annexure IV).
The millennium development goals (MDGs) are the most Counsel the mother (Annexure V).
broadly supported, comprehensive, and specific development vi. Teach the mother ro trear the local infection at home
goals the world has ever agreed upon. Adopted by world leaders (Annexure \/I).
 I

ESSENCE OF PEDIATRICS

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 COMMUNITY PEDIATRICS

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ESSENCE OF PEDIATRICS

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 I

COMMUNITY PEDIATRICS

Annexure lll: Summary of Urgent Pre-Referral Treatments

for the Sick Child from Age 2 Months up to 5 Years

For all chilclren before referral:

Prevent low blood sLrgar by giving breast milk or 5uBar wdler

l{thechildisconvulsing,givediazepam {1Om{2ml solution) indose0.l ml/kgrectally;if convulsions

continue after 1 0 mlnuiei give a second dose of diazepam rectally.

Cive first dose of an appropriate antibiotic. Recommended choices are cotrjmoxazole (5 mg o{

rrimethoprim/kg) or amoxicillin (50 mg/kg). lf the child cannottake an oral antibiotic (children in shock
or those who vomits everything or are unconscious), give the first dose of intramuscular ampicillin
(100 mg/kg). Other options for an intramuscular antibiotic for pre-re{erral use include Amoxicillin (50
mg,kgr 61 ( e[lri,]\one '50 mgrkgr.

Cive one dose of paracetamol (15 mg/kg) for high fever ( 101 .3"F or above).
Cive first dose of intramuscular quinine (20 mg/kg) for severe malaria ( high malaria risk).
Cive first dose o[ an appropriale antibiolic.

Cive {irsl du'e ol appropriate anlibiolic.

Cire vilamin A rlor children from 9-l l month: I lac unil & >1 yr 2 lac Unit
lf there is clouding of the cornea or pus draining from the eye, apply tetracycline eye ointment'

WHO TREATMENT PLAN C

lf there ir no orher:evere clasri[ication, lV fluids should be given in the oulpatient clini< at cording to
WHO lrealmcnt Plan C ,Anner-lVr.
Cive 100 ml/kg lV fluids, Cholera saline/Ringer lactate solution is preferred. Normal saline does not
correct aciclosii or replace potassium loses, but can be used. Plain glucose or dextrose solutions are
not act eplable ior lhe trealment of severt' dehydr.rtion.
li lV iniu>ion i: not po::ible, urgent re{erral lo lhe hospital lor lV trp.rtment is recommended.
147h€'nypierrat take, rJ0 minule:, fluids >hould be given by nasogartric lube. lI none of lhese are
possible and the child can drink ORS solution must be given by mouth.
Note: ln areas where Cholera cannot be excluded for patients >2 years old with severe dehydration,
antibiotics are recommended. Two recommended choices are Tetracycline and Erythromycin.

li there is rro other severe classification, treat dehydration before referral using WHO Treatment Plan
B [or rome dehydration and Plan C for.evere dehydralion. Then refer to ho:pital.

Cive first dose of an appropriate antibiotic. Two recommended choices are cotrimoxazole and
amoxicillin. If the child cannoi tnk" nn oral antibiotic (children in shock or those who vomtts everything
or who are unconscious), give the first dose of intramuscular ampicillin (100 mg/kg). Another option
for an intramuscular antibiotic {or pre-referral use include benzyl-penicillin or ceftriaxone.
Cive iirsl do:e of prracetamol for pain.

Ciive first dose of vitamin A, as mentioned above

ESSENCE OF PEDIATRICS

Annexure lV: Treatment of a Sick Child Aged 2 Months

up to 5 Years in the Outpatient Health Facility

a) Cive antibiotic {or 5 days.

The choice of antibiotic is based on the fact that most childhood pneumonia of bacterial orrgin is due to
5lreplt:crtt(u5 pncumoniae or llaenrcphiluq intluent,te. Non-severe cases oI pneumonia can be"treated with
eilher oral amoxicillin or cotrimo\azole for 5 days. Thec* two oral ,rntibiotic> are usually eifective agailrst these
two bacteria, both are relatively inerpensive, widely available, and are on the essential drug list of mosl countrles.
b' \oolhe the throat and relieve the cough wilh a safe remedy.
t, Advise molher r.n hen lo return immediately.
d) Follow-up in 2 days.

NO PNIUMONIA - a) Soothe the throat and relieve the cough with a safe remedy.
COUCH OR.COTD c) Advise mother when to return immediately.
dr Follow-up in 5 day..
SEVERE DTHYDRATION WHO Treatmenl PIan C
Start lV fluid immediately. lf the child can drink, give ORS by rnouth till the drip is ready. Cive 100 ml/kg of cholera
saline or Ringer Lanctate Solution (or, if not available, normal saline), divided as follows:
ACI First give 30 mllkg in Then give 70 ml/kg in
infants (under 12 months) t hour 5 hours
Children (1 2 nronths up to 5 years) l0 minutes 21lu hours

. Repeat once if radial pulse is still very weak or notdetectable.

c Reassess the ch ild every 1 -2 hours. lf hydration status is not improv ing, give the lV drip more rapidly.
. Also give ORS (about 5 ml/kg/hr) as soon as the child can drink:
. Usually after 3-4 hours (infant) or 'l-2 hours (children).
. Reassess an infant after 6 hours and a child after I hours. Classify dehydration. Then choose the appropriate
plan ,A. B. ur Ct to ronlinue l!'e.rtmenl.
Note: lf possible, observe tlre chilr{ at le.rst 6 hours aitcr rehvdration to be sure tlre mother can maintain hydration giving the child
OR5 .olution br mouth.

WHO Treatment Plan B

SOME DEHYDRATION Cive initial treatment with ORS over a period of 4 hours. The approximate amount of ORS required is 75 ml/kg;
dr-rring these 4 hours, the mother slowly gives the recommencled amount oi ORS by spoonfuls or sips.
Note: i) lf the child is breast-fed, breast-feecling shoLrld continue. Children uncler 6 months who are not breastfed-
:ht,uld be given 100 100 ml plain waler dLrring thi' period to prevent hypernatrcmia.
iir lI the t hild vornits, wait [or ]0 minules and then give more slowly.
iiir l{ the t hild wants more. givr' nrore OR5 solution.
After4 hours, the child is reassessed and reclassified for dehydration, and feeding should begin. When there are no
signs of dehydration, the child is put on Plan A. lf there is still some dehydration, PIan B should be repeated. lf the
child now ha'.pvq1. dehydration. the child should be put on Plan C.

NO DEHYDRATION. WHO Treatment Plan A

PIan A focuses on the {our rules of home treatment: give extra fluids, continue feeding, and advice the caregiver when to
return (if there
is blood in the stool, the child drinks poorly, becomes sicker, or is not getting better in 2 days).

FIuids should be given as soon as diarrhea starts; the child should take as much as s/he wants. Correct honre iherapy
can prevent dehydration in many cases. ORS may be used at home to prevent dehydration. However, other fluids
that are commonly available in the home may be less costly, more convenient and effective, especially when given
with food, e-g., Chira-pani, Cooked rice \\,ater (Bhater Mar), 1,ogurt.
Note: Fluids which should he avoided
Very sweei tea, soti drinks, ancl srveetened fruit drrrrks shoulcl be ;woicled. They can cause osmotic diarrhea and
hvpernatremia. FIuids lvith purgative action and stinrulants ie.g., coifee and some medicinal teas or infusior.rs) also
to be avoided.
Feeding - Enaurage mother to continue breast-feeding and family food according to age. Recommended home
iluid should be:
. Easy to prepare.The recipe should be iarniliar and its preparation should not require much effort or time. The
required ingredients and tne,t:uring uten'ils should be readily arailable and inexpensive.
. Acceptable. The fluid shouid be one that are culturally acceptable and the mother is willing to give freely to a
child with diarrhea and that the child will readily accept.
. Effective. Fluids that are safe are also effective. Most etiective fluids are those containing carbohydrates, protein,
and some salt. However, rrearly the same result is obtained when fluids are given freely JIong with weaningfoods
tha I t onta rn :a lt.
 I

COMMUNITY PEDIATRICS

PERSISTTNT DIARRHEA Encourage the rnother to continue breastfeeding. lf the child is artificially fed with animal milk, limit it to % of the
;;"rl;";;.;;t, *harthe cl^,ilcl was taking. criater amounl may aggravate the diarrhea.
Other foods according to age should be given in frequent, small meals, at least six times a day. All children with
persistent diarrhea shiuld receive supplementary multivitamins and minerals (iron, magnesium, zinO each day for
I u eek:.
(Dose for Zn:2 mgelemental Zn/kgldJ
Diarrhea is a ,eriou. and often [atal event in rhildren with revere malnutrition. For management oi dehydralion
in severely malnourished children: Full-strength ORS solution should not be used for oral or NC rehydratiort.
tt provides too much sodium and too little potassium. A suitable oral solution can be prepared by:
. Dissolving a neu, ORS (containing 75 m1qlL of Sodir-rm) packet into 2 liters of clean water
. Add ing ai m I of potassium ch loride solution (from stock solution conta ining 1 00 g KCL/L) and
. Adding 50 g sucrose
Thismo(lified solution provide /ess sodlunr (37.5 nmol/L) more potassium ('4O nmol/L) and added sugar (25 g/L),
which is appropriate for severely malnourished children with diarrhea.

The {our key elements of dysentery treatment are:

. Anlibiotit s
. Fluidi
. Feeding
. zint
follow-up Selet Iion oI an antihioli, is hased on sen.ilivily pallern5 ol strains of Shig"lla i'olated in lhe area
(Ciprofloxacin is the drLrg of choice). Recommended duration of treatment is 3 days. lf after 2 days (during follorv-Lrp)'
there is no lmprovemenl the antibiotic should be stopped and a second Iine ofdrug should be used.

Cive an oral antimalarial drug.

Cive one dose o{ paracetarnol for high fever (1 01'F or above).
And advice mother to give paracetamol at home for fever.

Cive one dose of paracetamol for high fever (1 01'F or above).

FEVER. MATARIA
UNLIKTTY I reat olher obvious Ldu\c5 ol fe\ er.

MEASLES WITH
OR EYT Cive first dose of Vitamin A. lf clouding of cornea or pus draining from the eye is present, apply tetracycline eye
MOUTH COMPLICATIONS ointmenl. Ilmouth ulcer:. lrerl with genlian riolet

;r$aiAsn'i'ic iL bHiNiii,.' ., :
; Cive tirsl dose of Vitamin A

::..5;'. ":-,'
YqNrry?,:;: .".;

ACUTE EAR INFECTION Cive appropriale antibiotir lor 5 day:.

Cive one dose of paracetamo! for pain and advice mother to give paraLetamol at home for pain.
Drv the ear b1 wir king.

CHRONIC EAR INTTCTION Dry the ear bv wi, ling.

Treal r,r ith lopit al quinolone car drt-rps for l4 da1 t.

ANEMIA OR VTRY LOW rnd tcounsel

As.ers lhe t hild's {ecding attd lhe *othul,
ounsel th" mother at
accordingly
cr on feeding.
WEICHT lf pallor is present: give irorr; give oral antimalarial if high malaria risk.
Cive Albendazole/Mebendazole if the child is 2 years or older and has not had a dose in the previous 6 months .

lf the chlld is <2 years old, assess the child's feeding and counsel the mother accordingl,v on feeding.
 ESSENCE OF PEDIATRICS

Annexure V: Counsel the Mother

> Assess the Child's Feeding: Assess feeding in every child betow 2 years of age and in chitdrii iiiln iiiiy'iiii*
Weight or Anemia
Ask questions about the child's usual feeding and feeding during this illness. Compare the mother,s answers to the Feeding
Recommendations for the child's age in the box below.

ASK- > Do you breastfeed your child?

- How many times during the day?
- Do you aiso breastfeed during the night?
> Does the child take any other food or fluids?
What food or fluids?
- How many times per day?
- What do you use to feed the child?
- lf very low weight for age: How large are servings? Does the child receive his own serving? Who feeds the child
and how?
> During this illness, has the child,s feeding changed? lf yes, how?
l How do you play with your child?
> How do you communicate with your child?

Feeding Recommendations During Sickness and Health

First 6 After 12 Months 2 Years and

Months of 6 Months up to Older
Ag" up to 2 Years
12 Months

Breastfeed as often as the
child wants, day and night,
at least B times in 24 hours.
Do not give other foods or
fluids. Not even water.
Breastfeed as often as the
child wants
After breastfeeding, give
adequate servings of rice
with dal, halwa, khichuri,
egg, fish, green Ieafy
vegetable, and yellow fruits
such as papaya, mango,
banana, and jackfruit.+
- 3 times per day if
breastfed;
- 5 times per day if not
breastfed.
Breastfeed as often as the
child wants.
Before breastfeeding, give
adequate servings of rice
with dal, halwa, khicuhri,
egg, fish, green leafy
vegetable, and yellow fruits
such as papaya, mango,
banana and jackfruit; or
give family foods 5 times
per day.
. Cive family foods at least
3 meals each day. Also,
twice daily, give nutritious
food between meals, such
as puffed rice with oil,
roli. biscuil. ripe papal a.
ripe banana, ripe mango,
jackfruit.

Feeding Recommendations For a Child Who Has pERSTSTENT DIARRHEA

o lf still breastfeeding, give more frequent, Ionger breastfeeds, day and night.
. lf taking olher miJk
- replace with increased breastfeeding OR
- replace half the milk with nutrient-rich semisolid food.
. For other foods, foilow feeding recommendations for the child's age.

I@
 I

COMMUNITY PEDIATRICS

Advise the Mother to lncrease Fluid During lllness

FOR ANY SICK CHI[D:
> lfchildisbreastfed,breastfeedmorefrequentlyandforlongerateach{eed. Ifchildistakingbreast-milksubstitutes,increasetheamount
of milk given.
L lncrease other fluid. For example, give rice water, chira pani, dub water, yogurt drink or clean water.
FOR CHILD WITH DIARRHEA:
) Civing extra fluid can be lifesaving. Cive fluid according to PIan A or Plan B on TREAT THE CHILD chart.

t-

D Advise the Mother When to Return to Health Worker

FOLLOW-UP VISIT
Advise the mother to come for follow-up at the earliest time listed for
the child's problems.

lf the child has: Return for

follow-up in:
PNEUMONIA 2 days
DYSENTERY
MALARIA, if fever persists
FEVER-MALARIA UNLIKELY, if fever persists
MEASLES, if measles now

PERSISTENT DIARRHEA 5 davs WHEN TO RETURN IMMEDIATELY

ACUTE EAR INFECTION
CHRONIC EAR INFECTION Advise mother to return immedicately if the child has any of these signs:
FEEDINC PROBLEM
ANY OTHER ILLNESS, i{ not imPorving Anv sick child Not able to drink or
breastfeed
ANEMIA 1 4 days Becomes sicker
VERY LOW WEICHT FOR ACE 30 days Develops fever
lf child has NO PNEUMONIA: a Fast breathing
NEXT WELL-CHILD VISIT COUCH OR COLD, also return if: Difficult breathing

Advise mother when to return for next immunization lf child has Diarrhea, also return if: Blood in stool
according to immunization schedule. Drinking poorly
ESSENCE OF PEDIATRICS
 COMMUNITY PEDIATRICS

Annexure Vl: Teach the Mother to

Treat Local lnfections at Home

I Dry the Ear by Wicking

L Dry the ear at least 3 times daily.
. Roll clean absorbent cloth into a wick.
. Place the w,ick in the child's ear.
o Remove the wicl< when wet.
. Replace the wick with a clean one and repeat these
steps until the ear is dry.

Y Treat Mouth lJlcers with Gentian violet

L Treat the mouth ulcers twice daily:
. Wash hands.
. Wash the child's mouth with wet clean soft cloth
Y Treat Eye lnfection with Tetracycline Eye wrapped around the finger.
Ointment . Paint the mouth with half-strength gentian violet.
. W,r'h handr atrin.
)r. Clean both eyes 3 times daily:
. Wash hands.
. Ask child to close the eye.
. Use clean cloth and water to gently wipe away plrs.
) Soothe the Throat, R.elieve the Cough with a
Safe Remedy
l Then apply tetracycline eye treatment in both eyes 3

times daily: ) Safe remedies to recommend:

. Ask the child to look up.

. Breastmilk for exclr-rsivelv breastfed infant.
. Squirt a small amount of ointment on the inside of the
. Warm water
lower lid. . Tulsi leaf juice
. Wash hands .rgain. . Lemon juice

) Treat until redress is gone. L Harmful remakes to discourage:

> Do not use other eye ointments or drops, or put anything . Medicines containing codeine, anti-histaminics, and
else in the eye. alcohol.

q

CHAPTER
Respiratory Diseases
Chopter Conlents
DISEASES OF THE EAR
Otilis Medio
Anatomic feature that makes children younger than 5 years
susceptible to ear infections include shorter, more horizontally
placed complaint Eustachian tubes, which permits refux of
nasopharyngeal secretions into the middle ear. Other risk
factors include exposure to cigarette smoke, over-crowding,
bottle-feeding, cleft palate, and allergic rhinitis. The same
risk factors are operative in the pathophysiology of the two
common varieties of otitis media seen in children-acute sup-
purative otitis media (ASOM) and otitis media with effusion
(oME).
Acute Suppurative 0titis Media
Childhood acute otitis media tends to occur in a bimodal age
distribution, with children between ages 6 and 24 months
and between ages 5 and 6 years at greatest risk. Steptococcus
pneumoniae and Haemophilus influenzne are the two most
common causative organisms of ASOM, accounting for
approximately 650/o of all cases. About l5o/o of the cases are
due to Branhamella catanhalis, Streptococcus pyogenes, and
Staphllococcus aureus infection. Respiratory viruses may play an
important role in initiating otitis media and may be the only
pathogens involved in some cases, as up to 20o/o of middle ear
aspirates are sterile.
I
7
Clinical Features Acute oritis media presents with a history
of ear discomfort or pain lasting an).where from a few hours
up to several days. There is often a history of recent upper
respiratory tract infection. Infants may present with irritability,
otalgia, and incessant crying. A child with acute otitis media
may also pull or rub the affected ear(s). Older children may
report impaired hearing in the affected ear. Fever is frequently
present, particularly in younger children. Dramatic relief of
pain, irritabiliry and crying indicates perforation of ear drum;
pus discharge is seen at this stage.
Otoscopic examination generally reveals a red and bulging
tympanic membrane, decreased mobility with loss of normal
landmarks. Suppuration (rupture of the drum with ear dis-
charge) may be seen filling the ear canal. Cleaning of this
fluid usually reveals an intact drum, as the rupture is small
and closes promptly after spontaneous perforation.
Treatment Antimicrobial therapy is the mainstay of rreatment.
Adjuvant treatment with oral decongestant drugs has not been
shown to be of any significant value, but may be used selectively
to provide symptomatic reiief of nasal blockage from co-exisring
cold. Topical decongestants, though frequently prescribed,
are detrimental due to rebound congestion and nasal and
nasopharyngeal mucosal irritation. Another frequently misused
class of drugs is antihistamines, which contribute iittle to the
resolution of otitis media and may precipitate sinus infections
due to their drying efFect on mucosal secretions.
o Amoxicillin should be the first-line therapy for acute otitis
media. Higher doses (60-90 mg/kg/d) may be considered
I
 I

COMMUNITY PEDIATRICS

Annexure Vl: Teach the Mother to

Treat Local lnfections at Home

I Dry the Ear by Wicking

L Dry the ear at Ieast 3 times daily.
. Roll clean absorbent cloth into a wick.
. Place the wick in the child's ear.
. Rernove the wick when wet.
. Replace the wick with a clean one and repeat these
steps until the ear is dry.

D Treat Mouth IJIcers with Gentian violet

i;q*da:t;l!i*::t!r,i;!;:
rlc. 14 ::*!r i€:ill:Fjar:a!,:rtl L Treat the mouth ulcers twice daily:
1r,r:i?..:,.t1; j,;:i:rer:;.,:,:J:t!):
. Wash hands.
. Wash the child's mouth with wet clean soft cloth
I Treat Eye lnfection with Tetracycline Eye wrapped around the finger.
Qintment . Paint the mouth with half-strength gentian violet.
. Wa'h hands again.
F Clean both eyes 3 times daily:
. Wash hands.
. Ask child to close the eye.
. Use clean cloth and \ /ater to gently wipe away pus.
D Soothe the Throat, Relieve the Cough with a
Safe Remedy
) Then apply tetracycline eye treatment in both eyes 3
times daily: L Safe remedies to recommend:
. Ask the child to look up.
. Breastmilk for exclr-rsively breastfed infant.
. Squirt a smalt amount of ointment on the insicie of the
. Warm water
lower licl. . Tulsi leai juice
. Wash hand. again. . Lemon juice

l Treat until redress is gone. ) Harmful remakes to discourage:

> Do not use other eye ointments or drops, or put anything . Medicines containing codeine, anti-histaminics, and
a lcohol.
else in the eye.

q
 CHAPTER
Respiratory Diseases
Chopter Contents
DISEASES OF THE EAR
Otitis Medio
Anatomic feature that makes children younger than 5 years
susceptible to ear infections include shorter, more horizontally
placed complaint Eustachian tubes, which permits reflux of
nasopharyngeal secretions into the middle ear. Other risk
factors include exposure to cigarette smoke, over-crowding,
bottle-feeding, cleft palate, and allergic rhinitis. The same
risk factors are operative in the pathophysiology of the rwo
common varieties of otitis media seen in children-acute sup-
purative otitis media (ASOM) and otitis media with effusion
(oME).
Acute Suppurative 0titis Media
Childhood acute otitis media tends to occur in a bimodal age
distribution, with children between ages 6 and 24 months
and berween ages 5 and 6 years at grearesr risk. Streptococcus
pneumoniae and Haernophilus influenzAe arc the rwo most
common causative organisms of ASOM, accounting for
approximately 65% of all cases. Abour \5o/o of rhe cases are
due to Branhamella ctftarrhalis, Streptococcus pl,ogenes, and
Staphylococcus aureus infection. Respiratory viruses may play an
important role in initiating otitis media and may be the only
pathogens involved in some cases, as up to 20o/o of middle ear
aspirates are sterile.
7
Clinical Features Acute otitis media presents with a history
of ear discomfort or pain lasting anylvhere from a few hours
up to several days. There is often a history of recent upper
respiratory tract infection. Infants may present with irritability,
otalgia, and incessant crying. A child with acute otitis media
may also pull or rub the affected ear(s). Older children may
report impaired hearing in the affected ear. Fever is frequently
present, particularly in younger children. Dramatic relief of
pain, irritabiliry and crying indicates perforation of ear drum;
pus discharge is seen ar rhis stage.
Otoscopic examination generally reveals a red and bulging
tympanic membrane, decreased mobility with loss of normal
landmarks. Suppuration (rupture of the drum with ear dis-
charge) may be seen filling the ear canal. Cleaning of this
fluid usually reveals an inract drum, as the rupture is small
and closes promptly after spontaneous perforation.
Treatment Antimicrobial therapy is the mainstay of treatment.
Adjuvant treatment with oral decongestant drugs has not been
shown to be of any significant value, but may be used selectively
to provide sympromaric relief of nasal blockage from co-existing
cold. Topical decongestants, rhough frequently prescribed,
are detrimental due to rebound congestion and nasal and
nasopharyngeal mucosal irritation. Another frequently misused
class of drugs is anrihistamines, which contribute little to the
resolution of otitis media and may precipitate sinus infections
due to their drying efFect on mucosal secrerions.
a
o Amoxicillin should be the first-line therapy for acute otitis t
media. Higher doses (60-90 mg/kg/d) may be considered I
1
1
{

where streptococcal resistance is endemic. Drugs such as
co-trimoxazole, amoxicillin-clavulanic acid, cefaclor, cefu-
roxime, or other newer cephalosporins are useful secondline
agents for unresponsive infections.
Some dissent exists regarding the routine use of antibiotic
for uncomplicated ASOM, because at least 50%o of uncom-
plicated cases experience spontaneous resolution within
2448hours. Antibiodcs are reserved for those children who
show continued symptoms after 48 hours. Initial antibiotic
therapy should last at least 7 days in uncomplicated cases,
with re-examination indicated after 34 days and at 3 weeks.
Current evidence does not support use ofdecongestant and
antihistamine in ASOM. Myringotomy/tympanocentesis is
indicated in children with severe refractory pain, hyperpy-
rexia, and complications (i.e., mastoiditis, facial paralysis,
labyrinthitis, or CNS infection).
o Many children present with recurrent episodes of ASOM.
If particularly troublesome, this condition may be treated
either with p{olonged antibiotic prophylaxis (e.g., amoxicillin
20 mglkg for 3-6 months) or insertion of tympanostomy
tubes. There are preliminary data that adenoidectomy may
be effective as well.
(omplications Extracranial complications include acute mas-
toiditis, subperiosteal and neck abscesses, and facial palsy. Acute
mastoiditis should initially be treated with parenteral antibiot-
ics, e.g., chloramphenicol. Surgery in the form of a cortical
mastoidectomy is reserved for cases with poor response to par-
enteral antibiotic therapy, subperiosteal abscess, an intracranial
complication, or acute mastoiditis in a chronic ear. Intracranial
complications are meningitis or intracranial abscess.
Otitis Media with Effusion
Following an episode of ASOM, serous middle ear effusions
may be seen in a number of children. Effusion has been found
to persist in up to 40o/o of children 1 month after their first
episode of otitis media and usually subsides within 3 months.
Tieatment is warranted in cases lasting for 3 months or more.
However, many children with fluid effusion in the middle
ear do not have any history of acute middle ear infections in
the past. Pathogenesis of this condition has not been clearly
established, but infectious, allergic, or immunologic mecha-
nisms may be at play.
Diagnosis Mild to moderate hearing loss and sensation of
ear blockage are the chief complaints, although the condition
may also be asymptomatic. Otoscopy reveals a dull tympanic
membrane, Ioss or distortion of cone of light with or without
a fluid level. Diagnosis is established by demonstrating reduced
mobiliry of the rympanic membrane with insuffiation, or more
reliably, by finding a q?e B pattern on tympanometry. Audi-
ometry may reveal varying degree of hearing loss.
Treatment Since 50% of serous middle ear effi:sions resolve
spontaneously within 3 months, newly diagnosed effusions
RESPIRATORY DISEASES
should be observed for this period in nearly all cases. If effusion
persists beyond 3 months, tympanostomy tube insertion may be
considered for significant hearing loss (>25 dB). Improvement
in hearing as well as ear discomfort is immediate and quite
gratifying, but lasts only as long as the tubes are in place. Mean
time before extrusion is usually benveen 5 andg months. The
majority of the ears recover by the time the tubes extrude, but
some children require repeated tube placements. Insertion of
tubes (of T-tube design) for long term or adenoidectomy may
be considered in cases of persistent symptomatic effusion. It
is probably safe for children to avoid swimming altogether to
prevent contamination of the middle ear space.
CSOM: The Draining Ear
Persistent or recurrent ear discharge is generally due to chronic
infection of the middle ear space. Such infection invariably
presents with chronic perforation of the rympanic membrane,
which allows egress of the suppurative material. Chronic sup-
purative otitis media (CSOM) most often results from neglected
acute middle ear infections.
Pseudomonas aeruginosa, S. aureus, Proteus species, E. coli, and
anaerobic streptococcal organisms are the organisms identified
commonly in chronically draining ears.
Less frequendy, CSOM may be seen with cholesteatoma,
which is a sac of squamous epithelium extending from the
tympanic membrane into the middle ear. Most cholesteatoma is
acquired, rarely, congenital. Though not malignant, cholesteatoma
may cause serious complications by slow expansion and local
destruction. Such complications include mastoiditis, mastoid
and neck abscesses, inner ear infection resulting in sensory
hearing loss, facial palsy, meningitis, and intracranial abscess.
Treatment Medical therapy consists primarily of topical anti-
biotics, though oral or parenteral administration may be
necessary in selected cases. Ear drying by wicking is helpful.
Instructions to parents to avoid water entry in the affected ear
should also be given. Otolaryngology referral is mandatory for
ruling out cholesteatoma.
Surgical therapy is primarily directed towards creating a
"safe ear". If cholesteatoma is suspected, ear exploration and
cholesteatoma removal is mandatory. In simple tympanic
membrane perforations without cholesteatoma, surgical repair
is now considered appropriate treatment in children older than
8 years of age. Tympanic membrane repair (tympanoplasty)
protects the ear from further contamination and infection,
often improves hearing, and may have a positive effect on the
quality of the childt life.
Otitis Externo
Acute diffirse otitis externa ("swimmer's eart') presents with
itching, pain, and fullness in the affected ear. Pain is severe
and precludes otoscopic examination. Tympanic membrane is
usually normal, if it is visualized. Erythema and edema of the

ESSENCE OF PEDIATRICS
canal skin and tenderness on moving the pinna and pressing
over tragus are diagnostic features. Cerumen is soft and white
instead usual firm-yellow. Preauricular lymph node may be
tender and palpable. P aeruginosa and S. aureus are rhe mosr
common organisms isolated in diffuse otitis externa. teatment
consists of ear canal cleaning by experienced personnel and
topical antibiodc (neomycin based preferable). Oral or even
parenteral anribiodcs may be needed for severe cases.
Localized otitis externa or furuncanlosis presenrs as an
exquisitely painful, superficial abscess in the outer portion of
the ear canal. Such infbction is commonly staphylococcai in
origin. Oral anti-staphylococcal antibiotics and analgesics bring
about prompt reliel Occasionally, incision and drainage of a
pointing abscess may be necessary.
Eczematous or psoriatic otitis externa describes a group of
inflammatory conditions in which there is drainage, pruritus
and/or scaling of the ear canal skin. Underlying causes include
contact dermatitis, atopic dermatitis, and seborrheic dermatitis.
To treat this condition effectively, the primary dermatologic
disorder must be addressed.
Otomycosis or fungal otitis externa is most common in
humid weather and presents with pain and pruritus of the
affected ear. Examination reveals fungal spores and filaments
along with cloudy discharge. Aspergillus is the most common
pathogen, though other fungi may be implicated. Aural toilet
and application of clotrimazole are curative.
Heoring Loss
Conductive hearing loss: Any process that interferes with the
conductive mechanism of the ear canal, rympanic membrane, or
ossicles may cause a conductive hearing loss. The most common
cause of conductive deafness in chiidren is otitis media with
effusion. Several congenital syndromes may also be associated
with middie ear abnormalities, such as Aperr, Crouzon, and
Treacher-Collins syndromes.
Sensorineural hearing loss: Sensorineural hearing ioss
(SNHL) is caused by a lesion of the cochlea, auditory nelve, or
central auditory pathways. SNHL can be acquired or congenital.
The majoriry of pediatric SNHL falls into the acquired caregory.
The most common cause of acquired sensorineural hearing
loss is meningitis. Other causes include perinatal infections
(TORCH, mumps, measles), neonaral hyperbilirubinemia,
perinatal asphyxia, and ototoxic medications (aminoglycosides,
loop diuretics).
Congenital causes ofsensorineural hearing loss can be further
divided into hereditary (i.e., Alport, Pendred svndromes) and
non-hereditary rypes. Causes of non-hereditary congenital
hearing loss include neonatal sepsis, prematuriry/low birth
ri'eight, and congenital infections.
Screening
Neonatal screening: All neonates with risk factors for hearing
loss mr-rst be screened with an auditory brainstem response
I@
I
I
(ABR) to exclude hearing irnpairment. It must be recognized,
however, that use ofclinical indicators to focus hearing screens
will miss as rnarry as 50o/o of all cases of impairment, while
universal ABR testing would be ideal.
Screening in older children: It has been shown that parental
assessment of the childt hearing, while helpful, is not always
reliable. Unrecognized hearing loss of even a mild to moderate
severity can lead to speech and language delays and academic
under-achievement. Examination should include otoscopywith
attention to middle ear pathology, such as OME and CSOM.
Any doubtful cases must be referred for detailed audioiogic
evaluation at the earliest opportunity in order that timeiy
intervenrion for hearing rehabilitation may begin. Multiple
techniques exist to assess hearing sensitivity and are selected
based on the age and the abiiities of the child. For younger
children unable ro undersrand instructions, behavioral or
play audiometry is usually performed. Pure-rone audiometry
is usually possible in children >5 years of age. Tympanometry
may be performed in nearly all children ro assess ear drum
mobiliry.
Treatment
Once the diagnosis of hearing loss has been established, treat-
ment is based on the extent of deficit and on the underlying
pathology. For very mild or unilateral hearing loss, rrearmenr
includes preferential seating in school. For significant conduc-
tive hearing loss, treatmenr may consist simply of tympanos-
tomy tubes or tympanopiasty.
Sensorineural hearing loss, in contrast, is generally more
difficult to correcr than conductive hearing loss. Theatment
of significant SNHL may require the use of assistive hearing
devices such as hearing aids from as early as 3 months of age .
Cochlear implants are indicated only for profound (tlO del
sensorineural deafness that is unresponsive to a 3-6 month
triai with the most powerful hearing aid available.
Lip reading, sign language, and deaf education programs
should be considered fbr children who are not candidates for
or cannot afford cochlear implantation.
DISEASES OF THE NOSE AND SINUSES
Allergic Rhinilis
Allelgic rhinitis is an inflammatory disorder characterized by
sneezing, itching, nasal blockage, and clear rhinorrhea; symptoms
may be seasonal ("hay fever") or perennial. This occurs mainly in
children older than 5 years; but diagnosis may be made in infants.
Symptoms in younger ones are sniffing, snorring, and rubbing
of nose; while older children tries ro ciear nose with repeated
blowing. Repeated allergic salute (rubbing nose with open palm)
creates nasal crease over the bridgeofthe nose. Nasal congestion
and blockage are severe enough at night to cause sleep disturbance,
irritabiliry and snoring. Asthma, sinusitis, allergic conjunctivitis,
and otitis media are comorbid conditions often associated with
I
I

allergic rhinitis. Examination reveals an edematous nasal mucosa,
boggy-hypertrophied inferior terbinates, mucoid discharge, hyper-
trophic pharyngeal lyrnphoid follicles (cobblestone appearance),
and postnasal drainage. Conjunctival itching and redness are
sometimes present. Inhaled allergens (pollen, spores, dust mites)
are the most common cause; food allergy (e.g., to cowt-milk) is
rarely implicated.
r,
Though the above symptomatology is characteristic, accu-
l- rate diagnosis may require demonstration of raised eosinophil
t counts in blood and nasal mucus, and skin and serologic tests
t
I
t=
to show specific IgE response to a variety of allergens (allergy
t, tests). High nasal eosinophil indicates allergic diagnosis, while
t higher neutrophil count supports bacterial infection
Treatment
Avoidance of known allergens, use of topical cromolyn
sodium and intranasal steroid sprays for prevention, oral
montelukast, and antihistamines for relief of symptoms.
Topical d€congestants should generally be discouraged as
they cause rebound congestion (short-term) and chemical
rhinitis or rhinitis medicamentosa (long-term). However, it
can be used in severe nasal blockage interfering daily life.
Use should be restricted to 5 days and once in a month.
Immunotherapy may be beneficial for refractory cases, but
its application is now infrequent owing to the success of
steroid sprays.
Virol Rhinilis (Common Cold)
Viral infections of the nose are very common in the pediatric
age group. Parents often need reassurance that such frequent
colds are not abnormal, provided their child has an otherwise
normal growth pattern. Malaise, low to moderate grade fever,
nasal congestion, and rhinorrhea are the presenting symptoms.
Treatment
fieatment is symptomatic and requires paracetamol for fever.
For nasal blockage, normal saline can be instilled in nostrils
every 4-6 hours. Plenty of fluid should be advised. Home
remedies (honey, tulshi, lemon juice) are beneficial for cough
and cold. Antihistamines are contraindicated. Otitis media and
sinusitis are frequent complications.
Sinusitis
Ethmoid and maxillary sinuses are the earliest to develop and
are the ones most commonly involved in infancy and early
childhood. Maxillary sinusitis may be associated with ethmoidal
infection in children older than 1 year, and frontal sinuses
may become involved only afier 4-5 years of life. Ethmoidal
pathology is now recognized to be the primary site of origin
of infammation in most cases of sinusitis.
The most common isolates are S. pneurnoniae, H. influenzae,
B. catarrhalis, and S. pyogenes.
!
RESPIRATORY DISEASES
Clinical Features
Acute sinusitis typically follows an episode of viral rhinitis
and presents with nasal discharge, nasal congestion, moderate
fever, foul breath (halitosis), cough and postnasal discharge.
Fever of moderate severity may be present in younger children.
Tenderness over the involved sinuses is present in adolescent
and adults; a purulent nasal and postnasal discharge may be
entirely negative. Children seldom complains of headache and
facial pain.
Diagnosis
CT scanning, when available and affordable, is far superior
to plain x-ray PNS OM view in the diagnosis of sinusitis.
Opacification, mucosal thickening, and air-fluid level are the
imaging findings of sinusitis, but do not point towards the
etiology.
(omplications
Preseptal celluliris, orbital cellulitis, and abscess are more
common. Infections behind the orbital septum can occur in
infants as well as older children, and present with proptosis,
chemosis, and painful ocular movement. In addition, ophthal-
moplegia, loss of vision, and severe toxemia indicate a spread
of infection to the cavernous sinus. Intracranial complications
such as meningitis and abscesses may also occur.
Treatment
o Amoxicillin given for 10 days should be the firstline of
medical therapy for acute sinusitis. Second-line agents, such
as amoxicillin-clavulanic acid, clarithromycin, or cefuroxime,
should be reserved for unresponsive infections or suspected
antibiotic resistance. Longer courses are indicated for refrac-
tory infections. fleatment of frontal sinusitis should be
initiated with parenteral antibiotic, because it can rapidly
progress to intracranial infection.
Other adjuvant measures that may have a possible
benefit include oral decongestants, mucolytic agents, and
topical nasal saline. Antihistamines are detrimental due
to their drying effect on mucosal secretions and are best
avoided.
An additional consideration in treating chronic or recur-
rent acute sinus infections is the presence of underlying
inflammation, such as that caused by allergy, passive
smoke inhalation, or exposure to environmental pollut-
ants. Control of such processes may help to eliminate
the infection. In refractory cases, a CT scan must be
ordered to identify anatomical abnormalities requir-
ing surgical correction. Surgery in chronic or recurrent
sinusitis should be directed towards opening the natural
sinus ostia ("functional" sinus surgery); however, with
aggressive medical therapy, the need to resort to sinus
surgery in children should be rare.
 ESSENCE OF PEDIATRICS
Nqsql Obslruclion
Chronic mouth breathing in children is generally caused by
blockage of nasal air-flow. The site of nasal blockage is more
often in the nasopharyngeal area, i.e., adenoid hypertrophy'
Intranasal causes ofobstruction inciude allergic rhinitis, recur-
rent sinusitis, nasal septum deviation, turbinate hypertrophy,
and antrochoanal polyp. Certain tumors (such as sarcomas or
angiofibromas) and congenital lesions (such as choanal atresia
or meningoceles) also belong in the differential diagnosis'
Diagnosis
Adenoid enlargement should be suspected in children' usually
older than 2 years, who present with nasal blockage, mouth
breathing, sleep disturbance, and chronic nasal discharge'
Examination must rule out nasal pathology. X-ray of naso-
pharynx (lateral view) will confirm the presence of soft tissue
enlargement in the nasoPharYnx'
Treatment
for recurrent
Tonsillectomy is often recommended attacks
of sore throat. It does not prevent recurrence of pharyngeal
infections. Tonsillectomy should be advised only if there are
more than six significant attacks of tonsillitis in a year for two
consecutive yeafs or ifthere is tonsiilar or peritonsillar abscess.
It may reduce the incidence of group A beta-hemolytic strep-
tococcal infection (GABHS). Tonsillectomy is recommended
in diphtheria carriers. Tonsils may be removed only if these are
acting as foci of infection for suPpurative otitis media' Tonsil-
lectomy should not be done during epidemics of poliomyelitis'
There is no indication for tonsillectomy after rheumatic fever
or glomerulonephritis.
Other indications include obsrructive sleep apnea or suspi-
I cion of malignancy. History of previous peritonsillar abscess
*ry also be a relarive indication.
|
I f.nsillar hypertrophy alone is not an indication. Recurrent
otitis media with effusion and deafness may be an indication
I if they are obese.
I fot tonsillectomy'
I Oaenoidectomy should be recommended for symptomatic
younger children. In older children, it is useful to remember
I
I that pubertal growth of the mid-face and regression of adenoid
Lire tends to resuit in relief of adenoid-related nasal obstruction
I n"m around rhe age oF 9 years'
I Cenerally speaking, surgery on the nasal septum should be
I in prepubertal children, as it may lead to retardation in
"rroided
Turbinate
I -id-face growth and saddling of the nasal dorsum.
I nypertrophy usually responds to treatment of allergy, though
I i" refractory cases, electrocautery may be used for reduction
I "f
trrrbinate size.
I
I and bloody vomiting or melena frequently causes diagnostic
I Sleep Disordered Breolhing
Obstructive sleep apnea (OSA) has been increasingly recognized
I
Snoring is the hatmark of sleep apnea' Adenotonsillar
Fhildren.
hypertrophy is the main cause of OSA in children. During sleep,
muscle tone reduces and there is critical obstruction of already
compromised upper airway (by adenotonsillar hypertrophy),
obstruction causes awakening from sleep and the airway again
opens. This goes on cyclically throughout the night, and there
is repeated hypoxemia-sympathetic hyperactivity.
0inical Features
Childhood OSA is a disorder of breathing during sleep, char-
actefized by prolonged upper airway resistance and partial or
complete airway obstruction disrupting pulmonary ventila-
tion and oxygenation. Night-time manifestations are snoring,
snorting, mouth breathing, increased respiratory effort, upper
torso sweating, choking, restless sleep, sleeping with hyperex-
tended neck and frequent awakening. Some children present
with secondary nocturnal enuresis. Daltime symptoms are
sleepiness school hours, early morning headache' tiredness,
fatigue, hyperactiviry poor attention sPan' poor school per-
formance, aggressiveness, failure to thrive, below average IQ,
and pulmonary hypertension. Physical findings are mid-facial
hypoplasia, receding chin, high arched palate, hypertension,
FTT, adenotonsillar hypertrophy, large tongue' and nasal block.
OSA is associated with Down syndrome, hypothyroidism, and
mucopolysaccharidoses (MPS).
Diagnosis
o Gold standard: Overnight polysomnography (PSG)->1.5
apnea-hypopnea index is diagnostic of OSA.
o X-ray nasopharynx (lateral view): Enlarged adenoid with
compression of the air column.
Treatment
o Topical nasal steroid (Fluticasone) use for 6 weeks has been
found to demonstrate moderate improvement in a double-
blind study in children.
o Adenotonsillectomy is found to be curative in 8570 cases,
even
o CPAP is used as secondline treatment' especially where
adenotonsillectomy is contraindicated or failed.
Epistoxis
Most epistaxis occurs in the antero-inferior portion of the
nasal septum due to the rich capillary vasculature in this
region known as Little's area (also known as Kiesselbach's
plexus). Minor trauma, vigorous rubbing of the nose, use of
nasal steroid, and dry winter air are frequently the cause of
bleeding. Daytime bleeding occurs without warning and is
spontaneous, while night-time bleeding may be swallowed
confusion.
Examination reveals prominent capillaries in Little's area that
bleed promptly when touched with a cotton-tipped probe'

Treatment
o Digital pressure by pinching the nose invariably stops
bleeding.
o Avoidance of nose picking, application of an antibiotic
ointment for lubrication and antimicrobial protection, and
for refractory cases, cautery (with silver nitrate or electro-
cautery), packing (anterior or posterior nasal packing), or
surgery (ligation of anterior ethmoidal artery) can be done.
o Control of allergy may be important in atopic children.
Hematinics to improve hemoglobin status.
o Bleeding disorders must be suspected in children with a
suggestive family history, a history of frequent bleeding from
other sites, or any nasal bleeding that does not respond in
the usual fashion.
o Nasopharyngeal angiofibroma is a tumor occurring exclu-
sively in adolescent males that can cause brisk bleeding in
a young boy.
Choonol Alresio
Failure ofthe nasal cavities to open posteriorly into the naso-
pharynx (choanae) during fetal development is called choanal
atresia. This process may be unilateral or bilateral. Bilateral
choanal atresia usually presents in the neonate immediately
after birth with respiratory disrress, which is due to the fact
that most infants are obligate nose-breathers. The affected
baby cycles between spells of cyanosis and crying. Anempts
at suckling immediately precipitate cyanosis.
Bilateral choanal atresia requires urgent management. The
airway may be established immediately by inserting a finger in
the babyt mouth; this can be replaced with a plastic oropha-
ryngeal airway or a McGovern nipple. Failure of these measures
to secure a satisfactory airway may necessitate endotracheal
intubation or tracheostomy. Once the airway is established,
diagnostic evaluation to confirm the presence of this condi-
tion and delineate its severiry must be initiated. Passage of
an 8 French catheter or, preferably, a narrow gauge fibreoptic
endoscope will be diagnostic. A CT scan will demonstrate the
thickness of the atretic plate and reveal whether it is bony or'
membranous.
Unilateral choanal atresia is a more indolent process and
may present later in infancy or early childhood with unilateral
nasal discharge or blockage.
Treatment
teatment is surgical. A variery of approaches is available and
includes transpalatal, transnasal, and trans-septal techniques.
Drilling may be required to create a new passage for bony
atresia. Stents are placed in the nasal passages to prevent reste-
nosis. These are left in place for 3-5 weeks and require close
nursing care to prevent blockage.
I
RESPIRATORY DISEASES
DISEASES OF THROAT
Acule Phoryngilis
Acute pharyngitis includes infections of pharynx and tonsils.
Most of the times, it is associated with rhinitis, sinusitis, and
occasionally laryngitis.
Commonly caused by viruses such rhino, corona, infu-
as
;
enza, parainfl uenza and adenoviruses5-30%
I of sore throats
are caused by bacteria. The important bacterial pathogen is
group A beta-hemolytic Streptococcus and C. diphtheriae.
Clinical Features
Children with acute pharyngitis may have fever, sore throat,
pain during deglutition, nasal discharge, conjunctival conges-
tion, and discomfort in throat. There may be enlargement of
tonsils with congestion and exudates over pharynx, tonsils,
and palate. Enlarged tonsils and soreness in throat may cause
blockade of oropharynx, leading to poor intake by children
and occasionally may present with drooling of saliva. Cervical
lymph nodes may be enlarged and tender.
Viral pharyngitis is self-limiting and recovers in 5-7 days.
Pharyngitis caused by group A beta-hemolytic Streptococcus
may lead to suppurative complications such as retropharyngeal
and peritonsillar abscess. Presence of these complications may
be indicated by high-grade fever, severe dysphagia, and bulge
in the posterior wall of pharynx or around tonsils.
The non-suppurative complications due to streptococcal
pharyngitis include rheumatic fever and acute
glomerulonephritis. For prevention of these complications, it
is important to treat streptococcal pharyngitis with penicillin,
as early as possible.
It is very difficult to differentiate viral from bacterial phar-
yngitis. Presence of exudates on pharynx with enlarged cer-
vical nodes and absence of nasal discharge suggest bacterial
pharyngitis.
Diagnosis
Acute pharyngitis is a clinical diagnosis. At times, it is very
difficult to differentiate nasopharyngitis from pharyngitis. Pos-
sibiliry of streptococcal pharyngitis can be made with presence
of exudates, enlarged tonsils, and absence of nasal discharge.
The diagnosis can be confirmed by throat swab culture. Now,
a rapid diagnostic test based on latex agglutination is also
available for diagnosis of streptococcal pharyngitis.
Treatment
o The major consideration in the treatment of acute pharyn-
gitis is to prevent rheumatic fever. If a clinical diagnosis of
streptococcal pharyngitis is made, a throat swab should be
ESSENCE OF PEDIATRICS
taken to
demonstrate sreptococci, and penicillin should
be administered.
o Penicillin can be given orally or by intramuscular route.
The duration of oral penicillin is for 10 days. If compli-
ance is a problem, single injection of benzathine penicil-
lin can be given.
o The other alternative antibiotics are ampicillin, amoxicil-
lin, or oral cephalosporins. If an individual is sensitive to
penicillin, he or she may be treated with erythromycin. The
newer macrolide such as roxithromycin, clarithromycin, and
azithromycin may be alternative to erythromycin in future.
At present, they are not recommended as firstJine drugs
due to less experience of these drugs in children.
o If diphtheria is suspected, the child should be managed
accordingly.
Acule loryn golroc heitis
Is the most common of the clinical entities termed "croup".
Acute laryngotracheitis is caused primarily by respiratory
viruses, most commonly parainfluenza virus. Because of its viral
cause, this croup syndrome is often referred to as viral croup.
Clinical Features
Viral croup usuallyhas a gradual onset and course. Symptoms
are often worse at night and persist for several days.
o Patients are initially seen with symptoms of upper respiratory
trad infecdon, followed after several days by the characteristic
barking cough, inspiratory stridor, and respiratory distress.
r Fever is usually low grade, but temperature as high as 104'
F have been noted.
o Hoarseness and aphonia are common.
o Classic triad-hoarse voice, harsh barking cough, and
inspiratory stridor.
Diagnosis
Diagnosis is clinical but can be aided by radiography of the
larynx, which reveals subglottic narrowing. An anteroposte-
rior view of the neck shows the classic narrowing of trachea
("Church steeple" or "wine bottle" sign). X-ray is not manda-
tory can differentiate from epiglottitis.
Treatment
Therapy for viral croup is mainly directed at improving air
exchange.
o Humidification:
o Home: Patients who have mild illness may be treated at
home with humidified air from a hot shower or bath,
hot steam from a vaporizer, or "cold steam" from a nebu-
lizer. Respiratory distress may improve within minutes,
but humidification should be continued until the cough
subsides, which usually is after 2 or 3 days.
o Hospitalization: Patients who have moderate-to-severe
illness should be hospitalized if any one of the following
signs and symptoms is noted: cyanosis, decreased level
of consciousness, progressive stridor, or a toxic appear-
ance. Cold, humidified oxygen should be provided, and
the patient should be observed closely in case emergency
intubation is needed. But otherwise the patient should
be disturbed as little as possible. Pulse oximetry and ar-
terial blood gas analysis are important in assessing the
adequacy of air exchange.
Racemic epinephrine (2.5% solution diluted l:8 with water
in doses of 2-4 ml for 15 minutes delivered by nebulizer)
has been shown to improve air exchange in these patients.
This drug should be used in moderately ill, hospitalized
patients, and it may eliminate the need for intubation during
the 24-48 hours when the illness is most severe. Racemic
epinephrinet effects are transient and acts by reducing
vascular permeability of the airway epithelium; therefore,
diminishing airway edema and improving airway caliber.
Systemic corticosteroids are effective in reducing symp-
toms within 6 hours and for at least 12 hours after initial
treatment. Dexamethasone 0.6 mg/kg/dose IM, IY PO; or
prednisolone 1-2 mglkg can be used.
Contraindications: Sedatives, opiates, expectorants, bron-
chodilators, and antihistamines should not be given to
these patients.
Acule Sposmodic loryngilis or Sposmodic
Croup
Itrefers to brief repeated attacks of symptoms that are clinically
similar to those of viral croup but less severe. Spasmodic croup
occurs most often in children who are between 1 and 3 years old.
Spasmodic croup is believed to be caused by viruses, although
important allergic and psychological factors probably contribute
to the illness in some patients.
0inical Features
Spasmodic croup is characterized by the sudden onset (usually
at night) of croupy cough and respiratory stridor. The episodes
usually last less than 1 day, but may recur several times per year.
Treatment
Tieatment at home with humidified air is generally sufficient
for this illness.
Croup ([oryn gotroc heobronchitis)
Croup is characterized by a distinctively brassy cough combined
with one or more of the following: hoarseness, inspiratory stridor,
and signs of respiratory distress due to laryngeal obstruction.
Laryngotracheobronchitis is more severe, most commonly i
caused by viruses. Severe tracheal obstruction with copious,
thick secretion is common.
\
1
I
 RESPIRATORY DISEASES

Laryngotracheobronchitis is caused by parainfluenza ot
influenza viruses.
Laryngotracheobronchitis is usually similar in onset to
Iaryngotracheitis but results in more serious illness.
Treatment
Nebulized racemic epinephrine is recommended. Oral steroid
(as described under laryngotracheitis) should be given in viral
croup. A helium-oxygen mixture is effective. Intubation with
vigorous suctioning of the airway to remove secretions may
be necessary.
Acule Epigloltilis
It is rapidly progressive infection of the epiglottis and contigu-
ous structures that may cause life-threatening airway obstruc-
tion, which must be regarded as medical emergency. It may
affect any age, but peak age is 3-6 years.
Almost all cases of acute epiglottitis in children are caused
by H. influenzae rype b.
restlessness may cause complete obstruction of the airway
by the swollen epiglottis. The diagnosis is based on
finding a swollen, cherry-red epiglottis. It is essential to
visualize the epiglottis with a laryngoscope or
bronchoscope in an operating room with complete
cardiorespiratory support. Visualization of the epiglottis
in other settings by depressing the tongue is contraindicated
because of the possibility of inducing airway obstruction.
Support from otolaryngologist or anesthetist should
be taken.
Radiography: In patients with mild stridor who are
not acutely ill, radiographs (lateral neck views) of the
nasopharynx and upper airway are useful in determin-
ing whether epiglottitis is present. The presence of the
"thumb printing" sign is a common radiographic marker
for epiglottitis.
Culture of the epiglottic surface and blood should be
obtained for identification of the causative organism and
its antimicrobial susceptibility pattern.

Clinical Features Treatment

The abrupt onset of high fever, moderate-to-severe respiratory
distress, and stridor in a child who is sitting forward with
his or her mouth open and drooling (because of the inabil-
ity to swallow normally) are symptoms highly suggestive of
acute epiglottitis. Ciassic "tripod position'-forward leaning
posture with bracing arms and extension of neck that allows
for maximal air entry.
Diagnosis
Ventilatory support: After visual confirmation of epiglot-
titis, the patient should be intubated and given ventilatory
support until edema subsides, usually after several days'
O, inhalation.
Antibiotic therapy is given for 7_70 days and is directed
against H. inf.uenzae type b. Ceftriaxone, cefotaxime, or
chloramphenicol can be used, initially IV then orally.
Confiaindications: Racemic epinephrine and corticosteroids
should not be given to these patients.

o Physical examination should be done quickly and with Characteristics of three upper airway infections have been
care to minimize anxiety. Even a slight increase in summarized in Table 7.1.

Table 7.1: Characteristics of Three Upper Airway lnfections

Etiology Respi ratory vi ruses, including Respiratory viruses and bacterja, H ae moph iIu s i nfl uenzae
parainfluenza vi ruses and j nf luenza including S. aureus. S. plogenes, type b
viruses S. pneumoniae

Common age of occurrence i months I years 3 months-5years 2-7 years

Clinical features
Onset Variable (1 2-48 h0 Cradually progres:ive r I2 hr-7 days) Rapid r4- I2 hrt
Fever Variable ,1 00"- I 05"Ft Variable (100'-1 05'F, Highr>l0t'F)
Hoarseness and barking cough Yes Yes No

Dysphagia No No
Course of obstruction Variable progression Variable progression, usually severe Kdprd progressron

Leukocyte count Mildty eJevated band form count Variable, possibly increased Usually markedly elevated with
increased band forms

Roentgenogram Subglottrc ndrrowtng on l'A Subglottic narrowing on PA Swollen epiglottis on lateral

rad iograph radiograph; irregular sott-tissue density radiograph
within trachea on lateral radiograph
Treatment Humidifir ation. epi nephrine. Humidifir ation, anlibiotit, intubalion Antibiolic, inlubation
corticosteroid
ESSENCE OF PEDIATRICS
Bronchiolitis is an acure viral infection of the bronchioles
resulting in inflammatory obstruction.
Common in children younger than 2 years (due to their
small airways) with peak incidence at 6 months of age; male
child, children who have not been breast-fed, who live in
crowded conditions, whose mothers smoke cigarettes are at
greater risk. Highest incidence is in winter and early spring,
occurs sporadically and epidemically.
Respiratory syncytial virus (>5070), parain{luenza virus,
adenovirus, metapneumovirus, and mycoplasma are common
pathogens.
CTINICAL FEATURES
Onset of bronchiolitis is characterized by mild upper respiratory
tract symptoms (serous nasal discharge and sneezing), which
last for several days and may be accompanied by mild fever
(temperature of 101'-102' F), in a healthy child.
Lower respiratory tract involvement follows, with gradual
development of respiratory distress (i.e., paroxysmal cough, wheez-
ing, dyspnea) accompanied by iritability and decreased appetite.
Most affected infants have a history of exposure to older chil-
dren/adult with minor respiratory diseases within the week preced-
ing the onset of illness. Local endemiciry is usually presenr.
Thchlpnea, flaring of alae nasi, and use of accessory muscles
of respiration result in intercostal and subcostal retractions and
occasionally cyanosis. Widespread bilateral rales and expiratory
wheezes are characteristic. Breath sounds are barely audible in most
severe cases. Liver and spleen may be palpable due to depression of
diaphragms by hlperinflated lungs. Pulse oximetry is very helpful
in detecting SpO, and is a useful guide in treatmenr.
DIFFERENTIAL DIAGNOSIS
Differential diagnosis includes asthma, bronchopneumonia,
congestive cardiac failure, foreign body inhalation, cystic
Table 7.2 Differentiating Features of Bronchiolitis and Asthma
De{inition Acute viral infections of the bronchioles caused
by respiratory syncytial virus, parainfluenza and
influenza virus, adenovirus, mycoplasma
Age of onset 2 months to 2 yr with peak incidence between
3 and 6 months
Frequency of attack Usually single
Family history of allergy and asthma lnfrequent
Concomitant allergic manifestation (eczema Absent
or atopy)
Response to bronchodilators. corticosteroids \one
Serum lgE and eosinophil count Normal
fibrosis. Refer tbles 7.2 and 7.3 for differentiating features
of bronchiolitis, asthma, and bronchopneumonia.
INVESTIGATIONS
Diagnosis is clinical; investigations exclude differential diag-
nosis and detect complicarions.
Total \fi|BC count and differential count are usually within
normal range unless associated with secondary bacterial
infection.
X-ray chest is not mandatory, but reveals hyperinflation
of lungs and occasional scattered areas of consolidation
due to atelectasis (3Ooto), peribronchial thickening, diffuse
interstitial infiltrates, increased anteroposterior diameter
on lateral view.
o Blood gas analysis in severe cases.
e Virus isolation from nasopharyngeal secretions by antigen
detection using monoclonal antibodies against RSV or by
PCR or culture.
TREATMENT
Mild cases can be cared for at home in humidified atmosphere.
If respiratory distress increases or feeding problem appearr
child should be hospitalized. Indications for hospitalization
include apnea, resting respiratory rate >70 breaths/min,
SpO, <95%, atelactasis on CXR, not able to feed, and age
<2-3 months.
Treatment in hospital:
o General measures
Bed rest in propped up position with head and neck el-
evated at an angle of 30"-40" with humidified oxygen in-
halation (mainstay of treatment). Pulse oxymetry should
be performed regularly to keep O, saturation >950/0.
Correction of dehydration, if any, orally or by perenteral
fluid.
Asthma is a chronic inflammatory disorder
t aused hyper-responsiveness lo Lertdin stimuli
resulting in recurrent airflow obstruction,
presenting as wheezing, breathlessness, chest
tightness, and coughing
26% within {irst vear of life and 74% in
cnrldren <5 vears
Recurrent attacks
Frequent
Usually present
lmprovement occurs
U:uallv elevaled. eosinophil in spulum is
c haracterrsl i c
 RESPIRATORY DISEASES

Table 7.3 Differentiating Features of Bronchiolitis and Bronchopneumonia

Definition Acute viral infection of the bronchioles resuliing lnflammation of the lung parenchyma
in obslruclion
Age of onset 2 mo to 2 vr wilh pe.rk inciclence berween t Bolh inlancy and rhildhood
and b months

Fever and cough Usually mild High fever and severe cough are common
a few days aller onsel

Chest indrawing Sudden Cradual, iI present

Srgns Hyper-resonanl percussion nole. Wide:pread
[ine rales ;rnd rhonchi with prolonged
e\pirdlory bredlh round. tn severe cases, brealh
sound barely audible on auscultation
Total WBC count Normal
lmpalred percussion note. Vesicular with
prolonged expiration. Creps and rhonchi may
also be heard on auscultation
Usuallv increased rincreased PMN r

Chest X-ray Hyperinflated lung field along with scattered Widespread patchy opacities orr both lung
areas o[ con>o]idation fields, pneumatocele tslaphylococcal r

may be presenl

.r Maintenance of nutrition by oral or NG tube feeding.

:r Sedatives should be avoided.

It is very difficult to differentiate between first attack of
asthma and bronchiolitis. Salbutamol with ipratropium
inhalation may provide some benefit. Aerosolized epineph-
rine provides some benefit.
Corticosteroid: Its use is controversial. Patients having a
family history of atopy were found to be benefited with
Prednisolone given orally for 3 days.
o Antibiotics have no role.
r Antiviral agent. Ribavirin is indicated only in very sick
infants or in infants with congenital cyanotic heart disease,
signifi cant bronchopulmonary dysplasia, severe immunodefi-
ciency; fubavirin is delivered by small particle aerosol, along
with oxygen, for 20-24 hr/day for 3-5 days.
o About 2-7o/o of infants progress to respiratory failure and
need CPAP or assisted ventilation.
. disease is characterized by increasing dyspnea, cough, sputum
Extracorporeal membrane oxygenation (ECMO), when not
controlled by mechanical ventilation.
In bronchiolitis obliterans, the bronchioles and smaller airways
are injured, and the attempted repair produces a large amount
of granulation tissue that causes airway obstruction. Eventu-
ally, the airway lumens are obliterated with nodular masses of
granulation tissue and fibrosis.
ETIOLOGY
Idiopathic. Infections by measles, influenza, adenovirus, myco-
plasma; pertussis; connective tissue disease; and drugs.
CtINICAt FEATURES
Initially, cough, respiratory distress, and cyanosis may occur,
followed by a brief period of apparent improvement. Progressive
production. and wheezing.

PROGNOSIS INVESTIGATIONS

In most patients recover spontaneousiy after the first

cases,. X-ray chest findings ranging from normal to miliary mottling,
48-72 hours of illness and do not require ribavirin therapy tn 70o/o cases unilateral hypertranslucency with decreased
or hospitalization. Overall mortality rate is <1%0. However, pulmonary vascular markings (Swyer James syndrome).
30-50o/o of all patients with bronchiolitis develop asthma, and a Bronchography reveals obstruction of the bronchioles.
the incidence is higher if there is family history of asthma or a CT scan revealt bronchiecrasis.
other atopic disorders. a Pulmor-rary function test variable, severe obstruction is most
conlmon.
PREVENTION

\iosr in-rportant method of prevention is frequent hand-

.'...: ing. Pooled h1'perimmune sera (respiratory syncytial virus
':-.-i: elobulin intravenous [RespiGam]) given IV and
':: I r:. . 11. svno'tial virus monoclonal antibody (Palivizumab
r :: r' rir':n L\{ are administered monthly during the
: -r' : i--:ra'. tial lirus se ason.

Eil
ESSENCE OF PEDIATRICS

At least one course of corticosteroid treatment in an attempt Table 7.4: Clinical Features Differentiating Mild lntermittent,
to reverse the obstruction or present ongoing damage. Mild Persistent, Moderate Persistent, and Severe Persistent Asthma
Antibiotics may be used.

Days with <2Ay'y'eek

<2,Ay'y'eek 3-6ANeek Daily Continrral
PROGNOSIS symptoms

Some patients deteriorate rapidly within weeks of onset of Nights with <2/Month 3-4lMonth >5/Month Frequent
symptoms
inirial symptoms. but mosr survive with chronic disability.
PEFR or >80% >80% >60-<80% <60%
FEV l

PEFR <2O'K 20-30"k >30'k >30"k

va natl rl rty

Asthma is a chronic inflammatory disorder causing hyper-

responsiveness of airways to certain stimuli, resulting in recur-
Table 7.5: Symptoms, Signs, and Clinical Parameters
rent airflow obstruction, presenting as wheezing, breathlessness,
Differentiating Mild, Moderate, and Severe Acute Exacerbation
chest tightness, and coughing; completely or partially reversible
of Asthma
with bronchodilator or spontaneously resolving.

ctAssrFrcATroN
Symptoms
Pothophysiologicol Clqssificotion Phvsicalexhaustion l\o No Yes

o Extrinsic or atopic asthma: 90o/o of all asthma, common Talk in Sentence: Phrases Wordslcan't
ta lk
in children, 80% with documented allergy.
r Intrinsic or non-atopic asthma: l0o/o of all asthma, Feedi ng Able to
feed
Feed with
difficulty
Too breathless
to feed
common in women after 30, follows upper respiratory
Signs
infection, symptom persist, and difficult to treat.
o Special variants: Consciousness +Agitated Usually Agitated to
agitated drowsy
,r Exercise induced: Almost all asthma patients experience
At t essory muscle use: No Yes Usually
it. Some patients have it as a precipitant. Reduction of slernoc leidomastoid prominent
FEVi >10% is diagnostic. retraction
c Cough variant: Chronic cough and sputum eosinophilia. Pulse (/min) 00 <1 00,1 60 1 >1 60
Mostly in young at night. Cyanosis Absent Absent Likely to be
o Drug induced: Aspirin, propranolol, timolol may induce present
in some patients. Wheeze End Throughout the Expiration+
'r Occupational: Agents inhaled in occupational settings- expirarory expiration
farmer, cigarette manufacturer, bakery worker, etc. il",
ffil,'#
'r Seasonal.
PEFR/FEVr >60"/" 40 b0o/o < 409o
Pulsus paracloxus Normal May be present 20-40 mmHg
Clinicql Clqssificolion SaO, (pulse >95.k 95,9101, <91"k
. Intermittent: Two or less than two nocturnal symptoms in oxymetry)
amonth. Between the episodes, the patient is symptom-free
and PFT is normal.
o Persistent: Frequent attacks, >2/month. In between, the Differentiation: Refer Table 7.4.
patient may or may not be symptom-free and PFT is
abnormal except in mild variery.
. Acute exacerbation: Loss of control of anv class or variant
mav cause rnild to life-threatening condition:
o Severity of persistent asthma:
Mild: More than 2 times/month, and PEFR or
t Mild: Patient is dyspneic but can complete sentences.
- FEV, is usually <800/o-650/o.
> Moderate..Patient is dyspneic and cannot complete sen-
tence in one breath.
Moderate.'Almost dally attack, and PEFR/FEV, is
- <650/o*500/o.
> Seuere: Patient is severely dyspneic, talks in words and
may be restless, eve n unconscious.
Seaere: Dyspnea continuousiy for 6 months or
- more, and PEFR or FEV' <50%. Differentiation: Refer Table 7.5.
 t
RESPIRATORY DISEASES

Table 7.6: Levels of Asthma Control (6.00 PM) in asthmatic patients. PEFR measurements
on morning and afternoon (for - 1 week) before
treatment can establish diurnal variabiliry increases
in variability of >20-30o/o, on an average, indicate
increased bronchial responsiveness and worsening
Daytime None (twice or More than asthma.
symptom lesslwk) twice/wk
Laboratory criteria:
Limitation of None Any
activities Three 'r Sputum eosinophilia, increased eosinophil count in
or more blood
Nocturnal symp/ \one Any {eatures
awakening of partly
r Blood gas analysis , pH
controlled CXR: Shows bilateral symmetric air trapping. Patches of
Need for None rlrt ice or More than
asthma
reliever/rescue less/wk) twice/wk atelectasis of various sizes due mucous plaque is not unusual.
present
trealmenl Extensive areas ofcollapse, consolidation suggest an alterna-
Lung function Normal <80% predicted tive diagnosis. X-rai, is also done to exclude TB. CXR may
(PEFR/FEV,) or personal best
be normai in asthma.
Allergy test: Skin test and RAST (radioallergosorbent test)
have limited usefulness, since the role of desensitization
Clossificolion on lhe Bqsis of Conlrol therapy is not fully established.
It is important and relevant for management of asthma. On
the basis of control, asthma can be classed as (l) controlled,
(ii) pardy controlled, and (iii) uncontrolled (Table 7.6). DIFFERENTIAL DIAGNOSIS

Pulmonary tuberculosis, bronchiolitis, bronchiolacia or tracheo-

malcia, foreign body aspiration, hypersensitivity pneumonitis.
PRINCIPLE OF DIAGNOSIS
cystic fibrosis, recurrent pneumonia, GERD.
Clinical criteria: Cardinal feature of asthma-paroxysmal
respiratory distress, recurrent cough, wheeze, chest tightness,
recurrent attacks due to multiple stimuli.
MANAGEMENT PtAN
Pumonary function tests (PFT): The important parameters Management goal is to achieve clinical control. GINA revised
in spirometry include PEFR, FEVI, FVC and FEY 25-75. guideline-Rule of '2't
r In asthma, FEV'/FVC is <0.8 (normal, 0.8-1). In o Day time symptoms <2/wk
asthma, FEV, is commonly used for assessing the severiry o Nocturnal symptoms <2lmo
of asthma. r Number of reliever drug <2lyr (salbutamol canister)
r FEV 25-75 is effolt independent and is probably more o No exacerbation
sensitive indicator of airway obstruction. o Normal or near lung function
r PEFR can be measured with peak expiratory flow meter, o No limitation of daily activities
while for other parameters spirometer is required. It
may be used as a diagnostic tool as well as monitoring
Scoring system for step care management:
of treatment. Abnormaliry in PEFR suggestive of asthma
include:

A diurnal variation o{ >20o/o, <80o/o of predicted, 1- Do you have dyspnea everyday? Yes=l No=0
- and improvement of >-20o/o after bronchodilator 2. Do you have not turnal attacls of dyspnea Yes-1 No=0
>2 timeshonthl
therapy.
Bronchodilator reversibiliry test: It is done to differ- 3. Have you suffered from dyspneic attacks Yes=1 No=0
- entiate obstructive defect from restrictive defect and
severe enough to necessitate steroid tablets
or injection. nebulizalion, lni. aminophylline
to differentiate asthma. Reversibiliry can be found out or hospitalization?
by FEV, before and 30 minutes after administration 4, Do you have persistent dyspnea for the last Yes=3 No=
of Br-agonist aerosol. An increase of >l2o/o in PEFR 6 months or more or are you taking sleroid
or FEV, after aerosol therapy is strongiy suggestive of tablets iprednisolone elc.) for any I year or

ilil
morei
asthma. Failure to respond, however, does not exclude
asthma. 5. ls patient's baseline Iasymplomatit stage) PLI- Yes=1 No=
<bOo/" of predicted value?
\iriabiliw tests: The PEFR is usualiy lowest in the
- :-.-.:ninq (6.00 AM) and highest in the afternoon Total score

Eil
 !

ESSENCE OF PEDIATRICS

Step detection: 2. Give 5 puffs of reliever inhaler with spacer/direct through

mouthpiece
3. \Vait for 5 minutes
4. If no improvement, give another 5 puffs
0 Step I 0 Step I
5. Repeat the process for 5 times; if little/no improvement,
1 Step Il 1 Step tl transfer to hospital with puffs every 5 minutes
2 Step lll 2 Step lll
3-6 Step lV 3 Step IVA
Pqlient Educolion qnd Self Monogement
4 Step IVB
The traffic zone system of control:
Step V
Green zone: Indicated all is clear, PEF B0-100o/o, <75o/o
Long-lerm Monogemenl of Aslhmo: variability, minimal symptom-patient has to continue
treatment
Step Core Monogemenl
Yellow zone: Indicated caution 60-800/o, 5-25%o variabiliry
Step care plan for children <5 year: asthma symptom may occur-intensification/stepping up
of maintenance
Red zone: PEF <60% and symptom at rest-immediate
Step I As per need salbutamol inhaler Step I
Fr-agonist use, follow yellow zone if improve or report to
Step ll Full-dose cromolyn or nedocromil emergency department.
Step ll
Step lll Low-dose ICS

Step lV High-dose ICS Step lll TREATMENT

High-dose ICS . LABA,T
Step VA Step lV
theophylline Treotment of Mild Acute Aslhmo
Step VB Step V + Oral eorticosteroid Step V
Inhaled salbutamol 1 puffstat, another one after 5 minutes;
then 1-2 pufts 3-4 hourly for the next 72-24 hour. Spacer
Step care plan for >5 year and adult: is preferable. If inhaled salbutamol is not available, give oral
salbutamoi 0.2-0.4 mg/kg/d 6-8 hourly for the next 12-24
hour.
Asthma education and Environmental control
If no improvement a.fter 24host, advise for hospitalization
As need rapid at ling p -agonist
f:or fu rrher managemen r.
Controller Select one: Select one: Add one/more: Add oner
option lTtore:
. Low-do>e . Low-dose . Mediuml .
Treolment of Moderole Acule Asthmo
Oral
ICS ICS i LABA high-dose glucocorl- Refer Figure 7 .l for treatment of moderate acute asthma.
ICS + LABA icosteroid

. Leukotriene . Medium/ . Leukotriene r Anti-lgC Treqlment of Severe Acule Aslhmq

modifier high-dose modifier
(receptor ICS
Refer Figure 7.2 for treatment of severe acute asthma.
antagonist/ . Low-dose o Sustained-
synthesis General management:
ICS + release
inhibitor)
Leukolriene theophylline 1. Dehydration, if any, must be corrected by dextrose saline.
modiIier
Potassium may be given if hypokalemia develops. Usually,
Low-dose
* more than 1-1.5 times maintenance level of fluid should be
ICS
s usla i ned- given. Care should be taken not to over-hydrate the parient.
release 2. Routine administration of antibiotics is not needed, but if
t heophyl I ine
there is consolidation on chest X-ray, blood neutrophilia, or
lCS, inhaled corticosteroid; LABA, long-acting beta, agonist. presence ofcoarse crepitations or bronchial breath sounds,
Afterstartingappropriatetrcatment,thepatientshouldbeseenevery4 l2weeksfor
give antibiotics, such as erythroml'cin or amoxicillin.
step up or step down.
3. Cl-rest X-ray should be obtained in ail ser.ere cases or when

Monogemenl of Asthmo ql Home mediastinal emphvsema, Pneumothorax, or pneumonia

is suspected.
First aid for asthma-"Rule of 5" 4. Sedation is hazardous. tanquiliizers, morphine and other
1. Ensure the patient is sitting comfortably upright, be calm, opiates are contraindicated because of their depressant
and reassuring effect on respiratory center.
 RESPIRATORY DISEASES

Salbutamol inhaler with Nebutized Salbutamol

spacer 2 puffs every 20 min 0.15-{.3 mg/kg
for 3 times No improvement or
inhaler not available

No improvement
or deterioration
after 3 doses
Continue Salbutamol
inhaler 2 puffs 2-4 hourly
for 24-36 hr

lmprovement but Hospitalization

wheeze still pesists for further
after 24-36 hr. management

Add oral prednisolone

1-2 mg/kfld in
3 divided doses
for 3 days

lmproved
No Wheeze

lmproved
No Wheeze Discontinue inhaler (retievers).
Maintain other advice, e.g.,
preventer, avoid allergens.

Fig.7.1: Treatment of moderate acute asthma

Rescue steroid therapy: During step care management, PEFR Nomogrqm

patient may lose asthma control at any step suddenly, for
See Figure 7.3 for nomogram relating PEFR to height. The
example, due to viral respiratory tract infection. No stepping
nomogram is based on a study conducted among South Indian
up is required prior to it. Patient should follow the exist-
children.
ing step after ending the rescue course. Oral prednisolone
I-2 mglkgl d in single morning dose or two divided doses
for 3-14 days should be given. No tapering of this dose is
PREVENTION
needed. o Avoid triggering factors (ASTHMA), i.e., Allergens (pollen,
dander, dust, fungal spore), Sports (exercises, games, traveling),
Drugs Used in Aslhmo Temperature (co1d weather, wet, windy weather), Heredirv
Refer Table 7.7 for list drugs used in Asthma. (environmental factors), Mites, Anxiety (stress, worries).
ESSENCE OF PEDIATRICS

lmmediate Hospitalization

Propped-up position lnj Hydrocortisone 3-4 mg/kg

Oxygen 4-6 L/min 4-6 hourly OR Oral prednisolone
Nebulized salbutamol 0. 1 5-0.3 lf improved 2mglkg single dose (max 60mg),
mg/kg/dose every 20 min preferably morning dose
3 times or continuously. (lf patient can swallow)
lnj. Hydrocortisone 3--4 mg/kg
4-6 hourly Or oral Prednisolone
2 mglkg starting dose & then
1 mg/kg 6-12 hourly
Complete Reliel
No improvemenl
Or deterioration

Discontinue inhaler
(relievers). Discharge with
Add lpratropium bromide advice, e.g., preventer, avoid
6 hourly-<2 yr, 250 pg; aflergens, follow-up after 3-7
>2 yr, 250-500 pg lf improved days, etc.

No improvement
Or deterioration

Add lnjAminophylline 5 mg/kg bolus

over 20 min and then 0.5-{.7 mglkg/hr
Or, lnj. Salbutamol 15 pg/kg
bolus then -0.1 pg/kg/min

No improvemeni

Not available &

I
I

t
ICU care Other drugs*
ventilator support epinephrine/MgSO"

Fig, 7.2: Treatment of severe acute asthma.

r Desensitization is not very effective, may sometimes be CLASSIFICATION

harmful.
o Community-acquired pneumonia: Bacterial (Strep.
pneumoniae, H. infuenzae, Staph. aureus, Kl. pneumoniae),
Viral (respiratory syncytial virus, seen mainly in infancy;
influenza virus; parainfuenza virus; measles virus; and
Pneumonia is defined as an acute inflammation of lower
varicella virus), Chlamydia (Chlamydia pneumoniae),
respiratory tract (lung parenchyma) associated with recently
Mycoplasma pneumonide, fuckettsiae (Coxie/la burnetii) can
developed radiological pulmonary shadowing.
 RESPIRATORY DISEASES

Table7.7: Drugs Used in Asthma

1. Salbutamol
a) Oral o.2-o.4 mg/kg/d Syrup, tablet
I tsf = 2mB; I tab = 2,4 mg
b) lnhaler 2 puffs qds. ln ilB 1-2 puff MDI 100 prg/puff
before exercise
u) Respirator solution {solution & nebuler 0.15 0.3 mg/kgrdose 1 ml Solution = 5mg
.1
<5 vr = 0.5 ml/dose Nebule = 2.5 mg
>5 yr = 1ml/dose
d) lnjection 15 mg/kg bolus over 30 min 1 ml= tmg
Then 0.1 pglkgrmin.
2. Salmeterol 2 puffs 12 hourly MDI 25 pglpuff
3. Hydrocortisone 3-4 mgzkg/dose 4 -6 hourly I vial = 100 mg
4. Methylprednisolone Loading do.e: 2 mgkg I vial = 40, 125.50O,1 000, 2000 mg
Maintenance dose: 1-2 mg/kg/d qds
5. Predn isolone l-2 mg/kg/d tds ttab=5mg
6. Aminophylline Loacling dose 5 mg/kg followed by t ml =25mg
0.5-O.7 mgikgihr
7. Ipratropium bromide <2yr = 250 prgdose: >2yr = 250-500 pg/dose Respiralor solution I ml - 250 pg
6 hourly
B. Epinephrine r l:1000 dilutiont 0.01 ml/kg/dose (highest 0.3 mlr lnjeclion (1: 1000r lmlrampule
9. Cromones
a) Sodium cromoglycare I puff qds MDI 5 mg/puff
b) Nedocromil sodium I puff qds & after control 1 puff bd MDI 2 mgrpuff
10. Bet lomethasone 1 2 puff tds-qds MDI s0,100,250 pg/puff
11. Budesonide so-a00 prg bd MDI 50, 100,200 pg/puff
12. FI uti c asone 50-.100 pg bd MDI 2s. 50,125,25O pr{puf(
13. trramcrnotone 1 -2 puff qds MDI 100 pg/puff
14. Theophyl I i ne 7;a n{kfldbd Syrup 1 lsp-120 m8
15. MgSO, 25-50 mgikg 'max 2 B) ln jection 5 ml = 2.5 mg { lg = 4 mmol)
16. Leukotrienes a ntd gon ists: > l2 years: 20 mg twire daily, I hour before or Tab. Monas 4.5, 1O mg
Montelukast 2 hour after meal.
(Not recommend below 12 years of age).
17. Combinationinhalers: >4 years: One inhalation r50 prg salmeterol
Salmeterol + Fluticasone and 100 pg fluticasone propionate) twice daily.
Or one inhalation (50 pg salmeterol and :50 prg
fluticasone propionale) twice daily.

+95%
Mean
E
J -95% also give rise to primary pneumonia. See Table 7.8 {or
o clinical features differentiating bacterial pneumonia from
(s

viral pneumonia.
F= Hospital-acquired pneumonia (pneumonia occurring at
I
(E
0)
(L
least 2 days after admission to hospital). Common organ-
isms are E. coli, Pseudomonas, Klebsiella, Staph.
anaerobic organisms.

90 100 110 120 130 140 150 160 170

w#{{:gt#fi lil
Height (cm)

Fig.7.3: Nomogram relating PEFR (on the ordinate) to height

ron the abscissa). Mean and 95% confidence limits are shown.
tBased on a study among South lndian children.) anaerobic bacteria.

4
ESSENCE OF PEDIATRICS

Table 7.8: Clinical Differentiation of Bacterial Pneumonia o Right upper lobe pneumonia: Suspect aspiration, especially
from Viral Pneumonia in neonates and infants
o Upper lobe pneumonia with cavitation: Tirberculosis
Onset Acute (Pry) Cradual
o Recurrent right middle lobe pneumonitis: Consider parrial
Epidemic pattern +
bronchial obstruction due to glands
Course Progressive Sel!limiting
o Lower lobe pneumonitis: Chemical pneumonitis
r Multiple small abscesses: Staphylococcal/Klebsiella pneu-
Temperature +++ +/*
monia
Toxemia +++ o Severe bilateral interstitial pneumonia: Viral
Dyspnea ++ + (infants) o Bilateral interstitiai pneumonia with malignancy: ?neumo-
Associated URTI + c)tstis cltrinii
Auscuhation
Creps ++ +/* Note: The X-ray changes often lag behind clinical findings, both at the
Rhonchi/Wheeze ++ +1- onset of pneumonia and at the time of resolution.
Radiological Confluent infiltrales Diffuse in{iltrates in
perihilar areas
DIFFERENTIAT DIAGNOSIS
Hyperinflation +/- +(RSV Infection)
Pleural involvemenl + Bronchiolitis, congesrive heart failure (CHF), asthma, aspiration
Pneumatocele + of foreign body, pulmonary tuberculosis, pulmonary abscess.

TREATMENT
SOME DEFINITIONS OF PNEUMONIA
fleatment plan of pneumonia:
Bronchopneumonia: It is primarily a spreading inflammation
of the terminal bronchioles and their related aiveoli.
o Specific: Antimicrobial therapy
o Supportive
Lobar pneumonia: It is a pathological srare of the lung, o O, inhalation
where the alveolar air has been replaced by celiular exudate o Hydration (i1uid may be restricted, considering SIADH)
and transudate. o Nutrition
Pneumonitis: It is a localized inflammation of the lung paren- o Antipyretics
chyma due to non-inFectious causes. o Bronchodilators
,r Physiotherapy
Post-measles bronchopneumonia: It is a mixed pneumonia
involving the alveoli, supporting tissue and bronchioles, usually
manifest with or after the onset of measles. Radiologically, it VIRAT PNEUMONIA
is seen as peribronchial thickening, usually bilateral and often
In general, lower respiratory tract viral infections are much
extensive.
more common during winter monrhs. The rypes and severity
Interstitial pneumonia: It is characterized pathologically by ofillness are influenced by severai factors including ager season
massive proliferation and desquamation of alveoiar cells and of the year, immune status of the host and environmental
thickening of alveoiar walls. Chest skiagram may reveal a difruse, factor such as over-crowding. The peak attack rate for viral
hazy, ground glass appearance, usually at the lung bases with pneumonia is berween the age of 2 and 3 years and decreases
poorly defined hilar densities. slowly thereafter.

Persistent pneumonia: It is defined as persistence of symptoms

and roentgenographic abnormalities for >1 monrh.
Recurrent pneumonia: It is defined as rwo episodes of pneu-
monia in 1 year or >3 episodes at any time with radiographic
clearance berween two episodes of illness.
Clinicol Feolures
Rhinitis, cough, low-grade fever, cyanosis, respiratory distress,
tachvpnea accompanied bv chest indrawing, and nasal flaring,
and use of accessorv muscle is common, Hyper-resonant chest,
rvide-spread crackles, and wheezing may be present.

RADIOLOGICAL DIAGNOSIS
Diognosis
A guide to radiological diagnosis of pneumonla: r Total count of V/BC tends to be normal or slightly elevated
o Acute lobar pneumonia: Consider pneumococcal pneumonia (<20,000/mm3), with a predominance of lymphocpes.

o Acute phase reactants (ESR or CRP) usually are normal or
slighdy elevated.
o Chest x-ray reveals diffi.rse infiltrates. H;,perinfation is common.
r Isolation oforganism from nasopharynx or throat by culture
or polymerase chain reacrion.
o Rapid diagnostic test (fluorescent antibody tests) that use
labeled virus-specific antibodies to detect viral anrigens in
respiratory secretions.
Treolmenl
o Specific measure: Antiviral agenrs, like aerosolized ribavirin
(for RSD. Oral amantadine or rimanradine (influenza virus).
o Supportive mea$ures: As mentioned earlier.
PN EUMOCOCCAT PNEUMONIA
S. pneumoniaa is still the most common cause of bacterial
infection of the lungs. The classical four stages of conges-
tion, red hepatization, grey hepatization, and resolution
are well-known.
Clinicql Feqlures
The onset is sudden, and high fever, cough, pain in the chest
on the affected side, tachypnea, circumoral pallor, inspiratory
dilatation of the alae nasi, expiratory grunr, diminished chest
movement on the affected side, dullness on percussion, bron-
chial breath sounds, increased vocal resonance, crackles, and
pleural rub are found.
Diognosis
r \X/hite blood cell count is usually elevated to 15,000-40,0001
mm3, with preponderance of PMN cells.
o Arterial blood samples usually show hypoxemia without
hypercapnia.
o Chest x-ray reveals consolidation. Pleural reacrion wirh
fuid may be seen.
presence of
o Isolation of the bacteria from blood or lung aspirate is
diagnostic.
Treolmenl
o Speciftc: The drug of choice is crystalline penicillin G
(100,000 unitslkgl24 hr). A third-generation cephalo-
sporin (cefotaxime, 150 mglkgl24 hr, or ceftriaxone,
75 mglkgl24 hr) should be used if the isolate of
S. pneurnoniar is resisrant to penicillin but sensitive to
cephalosporin. Vancomycin (40 mglkgl24 hr) should
be used if the isolate is resistant to penicillin and
third-generation cephalosporin.
o Supportive: As mentioned earlier.
RESPIRATORY DISEASES
STAPHYLOCOCCAT PN EUMONIA
Pneumonia caused by S. aureus is a serious and rapidly
progressive infection. It occurs less frequently than viral
or pneumococcal pneumonia. Although it may occur at
any age, 30o/o of all patients ar€ younger than 3 months
and 70o/o are younger than I year. The pulmonary lesion
may be primary infection of the parenchyma; or may be
secondary to generalized staphylococcal septicemia or may
be a complication of measles, influenza, or cysric fibrosis
of lungs or may follow minor staphylococcal pyoderma.
Debilitating conditions including malnutrition predispose
the infants to infection with staphylococci.
Clinicol Feolures
The illness usually follows upper respirarory rract infection,
pyoderma, or other associated purulent disease. The infant
becomes acutely ill with high fever, cough, respiratory dis-
tress, tachypnea, grunring respirations, chest indrawing, nasal
flaring, cyanosis, reluctant to feed, toxic appearance, dullness
on percussion with bronchial or diminished breath sounds,
crepitations over the affected areas.
Diognosis
o Chest x-ray reveals bronchopneumonia early in the illness.
The infiltrate soon become patchy or may be dense and
homogenous and involve an enrire lobe or hemithorax.
The involvement may be right sided (65%o) or bilateral
(<20o/o). A pleural effusion or empyema may be found
and pyopneumothorax in 25o/o of patients. Pneumatocele
of various sizes are common.
r Rapid progression from bronchopneumonia to effusion or
pyopneumothorax with or without pneumatoceles is highly
suggestive of sraphylococcal pneumonia.
o Total count of \7BC: Leukocytosis usually occurs with
predominance of the polymorphonuclear cells.
r Gram stain of aspirated material (from trachea) or pleural
tap reveals gram-positive cocci in clusters.
o Blood culture may be positive.
o Pleural fluid reveals an exudate with PMN cell counts of
300-100,000/mm3; proteir' >2.5 g/dl, and a low glucose
concentration.
Treolmenl
r Speciftc: A suitable antibiotic combination is flucloxacil-
lin (50-100 mg/kg/d) and ampicillin (50-100 mg/kg/d)
in four divided doses. Cefuroxime (50 mg/kg/d) or Naf-
cillin (200 mglkgld) is indicated if a favorable response
is not obtained within 72 hours. Empyema should be
drained by chest tube.
o Supportive: As mentioned earlier.
ESSENCE OF PEDIATRICS
HAEMOPHITUS INFTUENZAE PNEUMONIA
H. inf.uenzae type b is an important cause of serious bacterial
infection in infants and children who have not received
H. influenzae rype b vaccine (Hiberix).
Clinicol Feolures
The onset of the illness is gradual with nasopharyngeal infec-
tion. The child has moderate fever, cough, dyspnea, grunting
respiration, and chest indrawing. The course is subacute and
prolonged. The localized dullness on percussion, crepitation,
and bronchial breath sound may be noted.
Diognosis
o The diagnosis is established by isolating the organism from
the blood, pleural fuid, or lung aspirate.
o Moderate leukocytosis is usually found.
o In the absence of a positive culture, a positive urine latex
agglutination'test result suppons the diagnosis of this infection.
Treolmenl
r Specifie Ceftriaxone (75 mglkgl24 hr) or cefotaxime (150
mglkgl24 hr) is included in the initial antibiotic therapy.
Chloramphenicol 50-100 mg/kg/d in four divided doses
may also be given if one cannot afford ceftriaxone. If
the strain is sensitive, ampicillin (100 mg/kg/d) may be
administered. If the initial response to antibiotic is good,
oral treatment can be instituted to complete a 10-14 days
course. Pleural effusion may require drainage.
o Supportive: As mentioned earlier.
PRIMARY ATYPICAT PNEUMONIA
Primary atypicai pneumonia is mycoplasma pneumonia. The
disease is transmitted by droplet infection. It occurs in epi-
demics, chiefy in winter months. The disease is common in
children at age over 5 years.
Clinicol Feolures
Fever, cough, headache, sore throat, and myalgia. Onset is
insidious or abrupt. Cough is usually dry at the onset and then
it becomes productive. Mild pharyngeal congestion, cervical
lymphadenopathy, dullness on percussion, rhonchi, rales, and
decreased breath sounds may be present.
Diognosis
o Total and differential \XtsC counts are usually normal.
o The rising titer of cold agglutinins (which agglutinate human
red cells in the cold) should be determined. A titer o{ 1:64
or higher supports the diagnosis. Acute and convalescent
tkers for M. pneumoniae demonstrating a four-fold or greater
rise in specific antibody confirm the diagnosis.
o Chest x-ray usually demonstrates interstitial or bronchopneu-
monic infiltrate, frequently in the middle or lower lobes.
Treotment
Specifie Spontaneous recovery usually takes place after an
illness of 1-2 weeks duration. Eri.thromycin (50 mg/kg/d),
clarithromycin (15 mg/kg/d given in two divided doses
for l0 days), or azithromycin (10 mg/kg on day l,
and 5 mglkgl day on days 2-5), oxytetracycline (for
children aged >8 years, 50 mg/kg/d in four divided doses)
are effective in shortening the course of mycoplasmal
illness.
Supportive: As mentioned earlier.
HOSPTTAT-ACQUT RED (NOSOCOMTAT)
PNEUMONIA
Hospital-acquired or nosocomial pneumonia refers to a
new episode of pneumonia occurring at least 2 days after
admission to hospital. The term include post-operative and
certain forms of aspiration pneumonia, and pneumonia or
bronchopneumonia developing in patients with chronic
lung disease, general debility or those receiving assisted
ventilation. The mortality of hospital-acquired pneumonia
is high (30%);
Etiology
The majoriry of infections are caused by gram-negative bacteria.
These include Escherichia, Pseudomonas, Klebsiella, Staph. aureus
(including multidrug-resistant MRSA forms), and anaerobic
organisms.
Factors predisposing to nosocomial pneumonia:
Reduced host defenses against bacteria:
'
c Reduced immune defenses (e.g., conicosteroid treatment, :
diabetes, malignanry)
o Reduced cough refex (e.g., post-operative)
o Disordered mucociliary clearance (e.g., anesthetic agents)
o Bulbar or vocal cord palsy
' Aspiration of nasopharyngeal or gastric secretions:
o Immobiliry or reduced conscious level
c Vomiting, dysphagia, achalasia, or severe refux
c Nasogastric intubation
Bacteria introduced into lower respiratory tract:
r Endotrachealintubation/tracheostomy
c Nebulizers/bronchoscopes
, Denral or sinus infection
r Bacteremia:
o Abdominal sepsis
o Intravenous cannula infection
o Infected emboli
I
\
I
a
 RESPIRATORY DISEASES

Clinicol Feqlures
Cough, fever, rigors, vomiting or a febrile convulsion,
loss of appetite, tachycardia, tachypnea, breathlessness,
central cyanosis, pleural pain (uncommon), impairment Refer Thble 7.9 for summary of physical signs in common
of percussion note. In the early stages, the physical signs respiratory diseases.
are those of acute bronchitis followed by development of
crepitations.

Diognosis A lung abscess is a suppurative process resulting in destruction

of pulmonary parenchyma and formation of a caviry contain-
CBC shows neutrophilic leukocytosis.
ing purulent material. The causes of lung abscess (APISOCE)
Chest x-ray shows mottled opacities in both lung fields,
are as follows:
chiefly in the lower zones.
o Aspiration: Aspiration of infected material when the local
defense mechanisms are overwhelmed by a large number of
Treqlrnenl
virulent microorganisms or are compromised by factors such
r Specific: Gram-negative coverage is usuaily obtained with as recent surgery (e.g., tonsillectomy or adenoidectomy) or
third-generation cephalosporin (e.g., cefotaxime) plus an systemic disease. Aspirated material containing bacteria that
aminoglycoside (e.g., gentamicin) or imipenem. Aspira- are normal inhabitants of the nasopharynx and oropharynx
tion pneumonia can be treated with co-amoxiclav pius reaches the most dependent portion of the lungs. The pos-
metronidazole. terior segment of the upper lobe and the superior segment
e Supportive: As mentioned earlier. of the lower lobes are most frequently involved in the

Table 7.9: Summary of Physical Signs in Common Respiratory Diseases

Consolidation as in lobar Reduced on None Dr;ll High-pitched lncreased; Fine crepitations

pneumonia affected side bronchial whispering early; coarse
pectoriloquy crepitations later.

Collapse due to Reduced on Towards Dull Diminished or Reduced or absent None

obstruction of a major affected side lesion absent
bronchus (absorption
col lapse)

Collapse due to peripheral Reduced on Towards Dull High-pitched lncreased; None early; coarse
bronch ial obstrr,rction affected side Iesion bronchial whispering crepitations later.
pectoriloquy

Local ized f ibrosis and/or Slightly reduced on Towards Impaired Low-pitched lncreased Coarse crepitation
bronchiectasis affected side lesion bronchial

Cavitation (usually Slightly reduced on None, or lmpaired broncn ral lncreased; Coarse crepitation
associated with affected side towards whispering
consolidation or fibrosis) lesion pectoriloquy

Pleural effusion Reducecl or absent Towards Stony dull Diminished or Reduced or absent Pleural rub in
Empyema (depenclrnB on srze) opposite absent (occasionally (occasionally some cases (above
on aftected side side bronchial) increased) effusion)

Pneumothorax Reduced or absent Towards Normal Diminished or Reduced or absent Tinkling crepitation
(depending on size) opposite or hyper- absent (occasionally when fluid present
on affected side side resonant faint bronchial)
Bronchitis: acute or Normal or None Normal Vesicular with Normal Rhonchi, usually
chronic s,vmmetrically prolonged with some coarse
diminished expiration crepitations

Asthma Symmetricall;, None Normal Vesicular with Normal or reduced Rhonchi, mainly
diminished prolonged expiratory and high-
expiration pitched

lil
B ronchopneumon i a Symmetrically None May be Usually harsh Normal Rhonchi and coarse
diminished impaired vesicular with crepitations
prolonged
expiration

d
ESSENCE OF PEDIATRICS
recumbent position, and basilar segments of the lower lobes
are the most likely to be affected when aspiration occurs
during dental procedure (i.e., erect position); anaerobic
bacteria including bacteroides, fusobacterium, and anaerobic
streptococci are commonly isolated.
o Pneumonia caused by aerobic pyogenic organisms (Staph.
aureus and Klebsiella)
o Infarction: Bacterial infection of a pulmonary infarct may
produce a lung abscess.
o Septicemia, pyemia: Metastatic lung abscess secondary to
septic emboli from bacterial endocarditis in right side of
the heart and septic thrombophlebitis.
o Obstruction of bronchi due to enlarged lymph node,
tumor, or foreign body may occasionally be complicated
by formation.
abscess
r Collapse: Bacterial infection of a collapsed lobe from
tubercular lymphadenopathy, foreign body, and adenoma
may produce abscess.
. Extension from subphrenic abscess (amebic or pyemic liver
abscess) and mediastinal sepsis.
CtINICAt FEATURES
a Onset: Acute or insidious
a Symptoms
o Cough, productive of large amounts of sputum, which is
sometimes fetid and blood-stained.
o Pleural pain common.
o Sudden expectoration of copious amount of foul sputum
occurs ifabscess ruptures into a bronchus.
o Signs
o High remittent pyrexia
o Profound systemic upset
o Digital clubbing may develop quickly (10-14 day$
o Chest examination usually reveals signs of consolidation;
signs ofcavitation rarely found
o Pleural rub common
o Rapid deterioration in general health with marked weight
lciss can occur ifthe disease is not treated adequately.
IABORATORY FINDINGS
a CBC-Anemia, raised ESR, PMN leukocytosis
o Gram staining, Z-N staining, and microscopy and culture
of sputum
X-ray chest (P/A and lateral view): Homogenous opaciry (if
abscess does not communicate with air passage) or a rhick
walled lung cavity with or without fluid level (if commu-
nication with bronchus present).
CT scan may provide better localization of the lesion and
may be helpful in doubtful cases.
MT and BCG tests.
DIFFERENTIAT DIAGNOSIS
Loculated pleural effusion, Echinococcus cysr, neoplasm,
infected congenital cyst.
TREATMENT
Antibiotics: Should be chosen according to culture and
sensitiviry (C/S); sample may be taken from spurum, blood,
or pleural fluid for C/S. If organisms could not be identi-
fied, antibiotics directed at Staph. Aureug H. influenzae,
Pneumococcus and anaerobes (combination of ampicillin,
cloxacillin, and metronidazole or a combination of ceftri-
axone, cloxacillin, and metronidazole) can be used for 4-6
weeks. Antibiotic should be given parenterally fo r atlext 2-3
weeks. Chloramphenicol can be used if a patient is allergic
to penicillin. In non-response, TB should be considered.
a Postural drainage.
a Bronchoscopy to identif' and to remove a foreign body.
a Surgical resection should be considered only in children
with recurrent hemoptysis, repeated episodes of infection
or suspicion of malignancy.
PROGNOSIS
Although radiological resolution may be very sloq resolution
occurs in most patients without risk factors.
Bronchiectasis is a chronic suppurarive disease, characterized by
destruction of the bronchial and peribronchial tissues, dilata-
tion of the bronchi, and accumulation of infected material in
the dependent bronchi.
ETIOTOGY
r Acquired:
o Infections: Recurrent lung infections, measles, pneumo-
nia, pertussis, post-primary TB
r.l Bronchial obsrruction (followed by infection).
Lymphadenopathy (e.g., tuberculosis), aspiration of
foreign body, tooth or tonsil fragment, neoplasm, gasrro-
esophageal reflux with repeated aspirations, congenital
heart disease with compression of airway by abnormal
vessels.
Predisposing factors are cysric fibrosis, lung abscess, sar-
coidosis, localized cysts, persistent lobar collapse, and
emphysema with compression of lung parenchyma.
Immune deficiency syndrome with repeated attacks of
pneumonia and bronchitis. i
\
t
I
r
t
;

RESPIRATORY DISEASES

r Congenital: Congenital bronchiectasis, tracheo-broncho- coMPUCATIONS

megaly, primary ciliary dyskinesia (Kartagener syndrome-
bronchiectasis, sinusitis, transposition of viscera). Recurrent pneumonia, lung abscess, empyema, bronchopleural
fistula, hemoprysis, metastatic cerebral abscess, cor pulmonale,
t'
and rarely amyloidosis.
CTINICAL FEATURES
Bronchiectasis may produce no symptoms. TREATMENT
I
Symptoms: Postural drainage The aim of this measure is to keep the
t
o Due to accumulation of pus in dilated bronchi: Chronic dilated bronchi emptied of secretions. It is of great value
, productive cough usually worse in mornings and often both in reducing the amount of cough and sputum and in
brought on by changes ofposture. Sputum is often copious preventing recurrent episodes of bronchopulmonary infec-
and persistently purulent in advanced disease. tion. Postural drainage consist of adopting a position in
a
o Due to inflammatory changes in lung and pleura surround- which the lobe to be drained is upper most, thereby allowing
, secretions in the dilated bronchi to gravitate towards the
ing dilated bronchi: Fever and increased cough and sputum
t volume when spread of infection causes pneumonia, which trachea from where they can readily be cleared by vigorous
a
is frequently associated with pleurisy. Recurrent pleurisy in coughing. Percussion on the affected side of the chest wall
I the same site often occurs in bronchiectasis. with cupped hands aid dislodgement of sputum, and a
v
t o Hemoprysis: Can be slight or massive and is often recurrent, number of mechanical devices are available that cause the
usually associated with purulent sputum or an increase in chest wall to oscillate, thus achieving the same effect as
t
sputum purulence. It can, however, be the only symptom postural percussion and chest wall compression.
V
in the so-called dry bronchiectasis. Antibiotic therapy: Appropriate antibiotics are indicated
'W'eight
o General health: loss, anorexia, sleep sweating, and only when there is acute exacerbation, which is evident by
failure to thrive in children. In these patients, digital club- increase in quantiry of sputum, sputum becoming yellow,
bing is common. purulent and foul smelling, and presence of systemic symp-
toms such as fever. Choice of antimicrobials are co-amoxiclav,
Signs: ampicillin-cloxacillin, second- or third-generation cepha-
losporins, ciprofoxacin (if pseudomonas) and metronidazole
o \X/hen the disease is sufficiently developed, the character-
(if anaerobes). Anti-TB treatment, when indicated.
istic finding is that of persistent early and mid-inspiratory
Relief of atelectasis can be achieved by bronchoscopic
crackles. These crackles are frequently described as "coarse"
aspiration of secretion or removal of foreign body.
and are not shifted by coughing.
e Surgical treatment Indications are progression of localized
Clubbing of the fingers and/or toes is a feature of gross
severe disease despite adequate medical management, intrinsic
disease with prolonged bronchial infection.
anatomic obstruction of bronchus, life-threatening hemorrhage
o Signs of collapse and fibrosis may be present in advanced
from a demonstrable source, and suppurative lesion from
cases. There may also be cyanosis, with signs of pulmonary
aspiration of fragmented foreign bodies that include bron-
hypertension and right heart failure.
choscopic removal (e.g., grass fiber or fragments of pea-nut).

INVESTIGATIONS
o X-ray chest (P/A) shows honey combing of the involved
area indicating multiple small abscess cavities or marked
Pleural effusion is the collection of fluid in the pleural space.
linear streaking ("rail road tracks") with loss of volume It may be unilateral or bilateral, transudative or exudative.
("crowding"), which is highly suggestive.
o Bronchoscopy is undertaken where there is a possibility of
surgical intervention. ETIOLOGY
CT scan: High-resolution CT scan is replacing bronchos-
copy and is safer. e Exudatq Result from inflammation, or other diseases of the
Sputum should be sent for staining (Gram staining, AFB), pleural surface; total protein is >3.0 g/dl, LDH > 200 IUIL'
culture and sensitivity. o Pneumonia
a MI BCG tests. o Tuberculosis
a Sweat chloride test (by pilocarpine iontophoresis) is useful o Malignant diseases-lymphoma, metastases
in the diagnosis of cystic fibrosis. o Collagen diseases-juvenile rheumatoid arthritis, SLE

ESSENCE OF PEDIATRICS
o Subphrenic abscess: amebic or pyemic liver abscess, pan-
creatitis
o Pulmonary infarction (rare)
o Tiansudate Hydrothorax, result from mechanical factors
influencing the rate of formation of pleural fluid passively;
total protein is <3.0 g/dl.
c Nephrotic syndrome
o Cardiac failure
o Decompensated cirrhosis of liver
o Kwashiorkor
CtINICAI FEATURES
Clinical features depend on arnount of efi-rsion. Onset is usually
subacute widr such manifestation as high fever, cough, chest pain
on affected side that worsen on deep breathing and coughing,
reflex abdominal pain in case of basal effusion, and weight loss.
On examination: reduced chest expansion, srony dull per-
cussion note, absent or decreased breath sound (occasionally
bronchial), with no added sound, and absent or decreased
vocal resonance.
INVESTIGATIONS
a Complete blood count.
a Sputum examination (Gram staining, Z-N staining).
a X-ray chest (P/A): Homogenous dense opaciry obscuring
the underlying lung with a curved fuid line above. Large
effusion may shift the mediastinum ro the contralateral
side. Small effusion may only blunt the costophrenic angle.
Occasionally, the fuid is localized below the lower lobe
(subpulmonary effusion), loculated.
Ultrasonography: It is valuable to differentiate berween a
loculated pleural effusion and tumor and also help to localize
an efFrsion prior to aspiration and pleural biopsy.
o Aspiration of fluid by a pleural tap confirms the diagnosis.
a Pleural fluid analysis and pleural biopsy.
a BCG, MT test(s), if TB is suspected.
TREATMENT
Specific Chemotherapy depends on rhe etiology of pleural
effusion (e.g., anti-tubercular drugs for PTB, antibiotics for
pneumonia).
Supportive: Therapeutic rhoracocenresis is indicated in case
of large pleural effusion causing respiratory distress.
Empyema is an accumulation of pus in the pleural spaces. It
is most often associated with pneumonia due to staphylococci
and less frequently with pneumococci and H. influenzae.
{
ETIOTOGY
Empyema is always secondary to infection in a neighboring
structure, usually the lung. The principal infections liable
to produce empyema are bacterial pneumonia, lung abscess,
pulmonary tuberculosis, bronchiectasis, septicemia, metastatic
spread of suppurative foci from distant lesion such as osteo-
myelitis and rupture of a subphrenic abscess. It may follow
traumatic or surgical wound.
PATHOPHYSIOTOGY
Pathophysiology of empyema has been described in three stages:
o Exudative stage (f-3 days): This is parapneumonic efFrsion
with minimal cellular response and is usually culture negarive.
o Fibrinopurulent stage (4-14 days): Effusion at rhis srage
contains PMN and fibrin. Gram stain and culture shows
organism.
o Organizing stage (afiter 14 days): An inelastic membrane
is formed on both pleural and visceral surface.
CTINICAL FEATURES
Empyema is more common in younger children. High-grade
fever, tachypnea, dyspnea, cough, chest pain, roxemia are usual
presenting features. Long-standing cases develop clubbing,
anemia, and malnutrition. Chest examination reveals clinical
signs of pleural effusion as mentioned earlier. Immunocom-
promised patienr may nor have fever.
coMPucATtoNs
Bronchopleural fistulas, pyopneumothorax, purulent pericar-
ditis, pulmonary abscess, osteomyelitis of ribs, meningitis,
arthritis, and septicemia.
INVESTIGATIONS
CXR: Indistinguishable from those of pleural effusion. CT
can detect loculated empyema and pleural thickening in non-
responding parienr.
Aspiration of pus: Confirms the presence of empyema.
USi is recommended to identify the optimal site to undertake
pieurocentesis.
Bacteriological examination: Gram staining and Z-N staining
of pus may help to determine the cause of empyema.
PMN leukocytosis and elevated ESR may be found.
TREATMENT
i
. Intercostal tube drainage should be done without any
undue delay, as this is the single most important step that I
\
1
f
.:
will bring relief.Tube with largest possible size should
be used and connected to under water seal drainage.
Intercostal drainage is kept inside till drainage is <30-50
ml per day.
Choice of antibiotic is based on suspected microorganisms.
Staphylococcus responds to cloxacillin, nafcillin, vancomycin;
Pneumococcus to penicillin, cefotaxime, or ceftriaxone;
I and H. influenzae responds to cefuroxime, ceftriaxone, or
I azithromycin or chloramphenicol.
'Wben
organism is not idcntif.able, ampicillin with clox-
7 acillin or cloxacillin with third-generation cephalosporin
is the right combination.
'When
anaerobic organisms are suspected, metronida-
zole should be added. Systemic antibiodc therapy is re-
quired for at least 3-4 weeks.
Anti-TB treatrnent, if indicated.
o Supportive: O, inhalation, nutrition, hydration, antipyretics.
o Fibrinolytic agene Streptokinase and urokinase instillation
into the pleural cavity has been found to reduce adhesion
in both uniloculated and multiloculated empyema thoracis
o Physiotherapy.
o Surgical management is indicated in pleural thickening,
loculated empyema, non-expansion of lung with drainage
tube, and bronchopleural fistula. Surgical modalities are
(l) decortication, (ii) lobectomy, and (iii) video-assisted
thoracoscopic surgery (VATS).
Complications: Bronchopleural fistula and empyema necessitans
(cutaneous fistula).
Pneumothorax (accumulation of air in the pleural caviry) is more
common in term and post-term infants than in premature ones.
The incidence is increased among infants with lung disease, such as
meconium aspiration and HMD, in those who have hadvigorous
resuscitation or are receiving assisted ventilation.
ETIOLOGY
Neonate (usually following alveolar rupture):
o Respiratory distress syndrome
o Positive pressure ventilation
o Vigorous resuscitation
o Meconium aspiration syndrome
r Spontaneous or idiopathic
o Rupture of a congenital cyst
o Tiaumatic
o Ball-valve rype of bronchial or bronchiolar obstruction
resulting from aspiration
Children:
e Idiopathic
RESPIRATORY DISEASES
r Spontaneous
r Secondary to lung abscess, empyema, infarct, rupture of a
cyst or an emphysematous bleb (e.g., in asthma)
e Foreign bodies in lung
o Tiaumatic
o Post-pneumonic, especially staphylococcal
o Iatrogenic-1hs1a6sn1esis, tracheotomy, transbronchial
biopsy
CLINICAT FEATURES
Clinical features depend on degree of lung collapse and on the
extent of pre-existing lung disease. It ranges from asymptomatic
to severe respiratory distress, chest pain, and cyanosis.
On examination, there is increased respiratory rate, reduced
chest expansion, hyper-resonant percussion note, absent or
decreased breath sound with no added sound, and decreased
vocal resonance.
DI FFERENTIAT DIAGNOSIS
Large pulmonary cavities, diaphragmatic hernia, gaseous dis-
tension of the stomach, and emphysema.
INVESTIGATIONS
X-ray chest (P/A) shows presence of air in the pleural space
with sharply defined edge of deflated lungs (better film when
taken in expiration). Complete translucency between this and
chest wall with no lung markings. If pneumothorax is large
and under tension, compressive atelectasis of the underlying
lungs and shift of the mediastinum and trachea to the opposite
side may be demonstrated.
TREATMENT
A small or even moderate sized pneumothorax in an
otherwise normal child may resolve without specific
treatment, usually within a week. A small (<5olo collapse)
pneumothorax complicating asthma may also resolve
spontaneously.
If collapse >5o/o or in recurrent pneumothorax or in tension
pneumothorax, a definitive treatment is necessary.
o Administering 100o/o O, may hasten resolution by increas-
ing the nitrogen pressure gradient between the pleural air
and the blood.
r Pleural pain deserves analgesic treatment, but codeine may
be justified.
r Closed thoracotomy (i.e., simple insertion of a chest
tube) and drainage ofthe trapped air through a catheter,
the external opening of which is kept in a dependent
position under water seal, is adequate to re-expand the
lung in most patients. Chest tube is usually removed
 ESSENCE OF PEDIATRICS

24 hours after the lung has fully re-inflated and bubbling
has stopped. If bubbling in the underwater bottle stops
prior to full re-inflation, the tube is either blocked,
kinked, or displaced.
Recurrent pneumothorax: Chemical pleurodesis by intro-
duction of talc powder, tetracycline, or silver nitrate into
the pleural space prevent recurrences. Open thoracotomy
through a limited incision with plication of blebs, closure of
fistula, stripping ofthe pleura, and basilar pleural abrasion
is also an effective treatmenr.
teatment of the underlying lung
disease (i.e., antibacterial
or antitubercular drugs) should be given.
Rarely, dangerous compression of the trachea by air in the
surrounding soft tissue requires surgical intervention.
Respiratory failure is defined as inability of the respiratorv
system to deliver adequate oxygen or to remove CO, from
the circulation, leading to arterial hypoxia (i.e., arterial PO,
<50 mmHg), hypercapnia (i.e., arterial PCO2 >50 mmHg),
or both. Respiratory failure accounts for approximately 50o/o
of deaths of children under 1 year of age.

Advise nor ro fly for 3 months in case of spontaneous

pneumothorax. ctAsstFtcATtoN
II relates to the absence
Its classification into type I and type
or presence oF hypercapnia (raised PCO,) (Table 7.10).

CTINICAL FEATURES
lt is defined as free air in subcuraneous tlssue.
Respiratory: tVheezing, expiratory grunting. Decreased or
ETIOTOGY absent breath sounds, flaring of alae nasi, rerraction of chest
wall, tachypnea, bradypnea or apnea, and cyanosis.
Air leaks from pneumomediastinum, fracture orbit (air leak Cerebral: Restlessness, irritability, headache, confusion, con-
from sinuses), tracheotomy, deep ulceration in the pharyn- vulsion, coma.
geal region, esophageal wounds, any perforating lesion of
Cardiaq Bradycardia or excessive tachycardia, hypotension or
the larynx, or trachea. It is occasionally a complication of
hypertension, cardiac arrest.
thoracocentesis, asthma, or abdominal surgery. Air rarely
may be formed in the subcuraneous rissues by gas produc- General: Fatigue and sweating.
ing bacteria.
Interstitial emphysema from ruptured alveolus dissect along TREATMENT
peribronchial perivascular connective tissue sheaths ofthe roots
of lungs. If volume of air is great, rhen there is mediastinal I. Acute type I respiratory failure:
emphysema, pneumomediastinum, pneumothorax, subcuta- a. High-concentrarion oxygen (>35o/r1 by oronasal mask
neous emphysema. It may press pulmonary veins at hilum, or by oxygen tents.
interfering venous return and cardiac output. Table 7.10: Causes of Respiratory Failure

CtINICAt FEATURES Type-l Acute

(POr<8.0 kPa Severe acute asthma
Usually asympromatic. On palpation, crackling under the skin. PCO.<6.5 kPa) LVF and olher causes o[ pulmonary edema.
If air press on trachea or on pulmonary veins at hilum, venous pH nbrmal or low Acute respiratory distress syndrome
return may be disturbed and cardiac output may be decreased, Pneumonia
Pneumolhorax
respiratory distress may develop.
Chronic
Right to len shunt:
INVESTIGATIONS Lung iibrosis
Type-ll ACUIe
X-ray shows air in subcutaneous soft tissue. (PO.<8.0 kPa Ser,ere acute aslhma when lifethreatening.
PCO.>6.6 kPa) A( ute epiglotlitis
pH low, Inhaled foreign body
TREATMENT Respiratory muscle paralysis (polio, CBS)
Brain >lem lesion
If the air leak from the respiratory sysrem, the problem
cause is Sleep apnea
Narcotic drugs
self-limited and requires no specific treatment. Resolu-
is usually
Chronic
tion occurs by resorption of subcutaneous air after elimination Kyphos( oltosis
of its source. Primary alveolar hypoventilation
l I

RESPIRATORY DISEASES

b. Immediate tracheal intubation and mechanical ventila- e. Controlled oxygen therapp start with 24o/o controlled-
tion (if patient is very ill) fow mask. Aim for aPOr>7 kPa (a PO2 <5 is very
c. Close monitoring: Arterial blood gas analysis (blood dangerous)
should be taken within 20 minutes) to establish that f, Antibiotics
treatment has achieved acceptable PaO, levels Diuretics
d. Prompt diagnosis and treatment of underlying causes. Progress
II. Chronic type I respiratory failure: a. If PCO, continues to rise or patient cannot achieve
a. Long-term oxygen a safe PO, without severe hypercapnia and acidemia,
b. Tieatment of underlying disorder respiratory stimulants (e.g., doxapram) or mechanical
III. Acute type II respiratory failure: ventilation may be required.
V Type II chronic respiratory failure:
a. Rapid reversal of precipitating event, e.g., dislodge- a. teatment of underlying disorder
ment of foreign body or tracheostomy, reversal of
b. Controlled long-term oxygen therapy
narcotic poisons
c. Mechanical ventilatory support, if necessary
b. Tieatment of severe acute asthma
c. Controlled low-concentration O,
d. Mechanical ventilation if the condition causing respi-
ratory failure cannot be reversed immediately.
Ghai OP (ed). Esential PediatricsT'h ed. New Delhi: CBS Publish-
IV Acute on chronic type II respiratory failure:
ers, 2009.
Initial aisessment Dworkin PH. NMS: Pediatrics 4"' ed. Philadelphia: Lippincott
a. All patients may not appear distressed despite being Williams & Wilkins, 2000.
critically ill ). Parthasarathy A (ed). IAP Tbxtbooh of Pedianics 4'h ed. New Delhi:

b. Conscious level (response to commands, abiliry to Jaypee Brothers, 2009.

4. Behrman RE, Kliegman RM, Jenson HB. Nelson Tbxtbooh of
cough)
Pediatrics 18'h ed. \XlB Saunders Co,2007.
c. CO, retention (warm periphery, bounding pulses,
5. National Guidelines: Asthma & COPD.4'h ed. Mohakhaii, Dhaka,
flapping tremor) Bangladesh: Asthma Association, 2010
d. Airway obstruction (wheeze, intercostal indrawing, Mollah AH, Ahmed S, Nahar N. Shisuder Asthna O Thr Chikit-
pursed lips, tracheal "tug") sha Babosthapona 2"d ed. Dhaka, Bangladesh: Shaisob Koishor
d. Right heart failure (edema, raised JVP, hepatomegaly, Prokashonee, 2007.
7. Haslett C, et al. (ed). Dauidson's Principles and Practice of Medicine
ascites)
20'h ed. London: Churchill Livingstone, 2008.
e. Background functional status and quality of life Asthma management handbook 1998. National Asthma Campaign,
Inoestigations Australia.
a. Arterial blood gases (severity of hypoxemia, hypercap- g RingJC, et al. Nobel therapies for acute respiratory faillre. Pediatr
nia, and acidemia) Clin North Am 1994;41:1325.
b. Chest radiograph 10. Isaacs D. Problems in determining the etiology of community
Tieatment acquired childhood pneumonia. Pedian Infect Dis J 1989;3:143.
a. Maintenance of airway 1l Kabra SK, Lodha R. Essential Pedia*ic Pulmonolgl 1" ed. New
Delhi: Noble Vision, 2006.
b. Tieatment of specific precipitating event
12 Li AM, Chan DFY, Fok TF, \fing YK. Childhood obstructive sleep
c. Frequent physiotherapyt pharyngeal suction
apnea: an tpdate. Hong Kong Medical Journal 2004;10(6):406-13.
d. Nebulized bronchodilators

f
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r
a
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r
r
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Il
I'

8 CHAPTER
Diseases of Gastrointestinal System
Chopter Conlenls
Abdominal pain......................,................,...,...........................130
Castrointestinal b1eeding......................................................
Persistent dianhea......,............
Chronic dianhea and malabsorption syndromes......,...l35
Celiac disease ........ ...............................137
Milk protein intolerance.............. ..........................................138
Abdominal pain may arise because of inflammatory lesions of
different intra-abdominal structures. It may also arise because
of distention of the hollow viscus, where the discomfort caused
by distention is often felt as pain. Increased peristaltic activiry
of hollow organs may be felt as acute or recurrent colicky pain
in abdomen. The pain is usually felt in the same dermatome
that shares the neurological supply with the underlying organ.
Thus, pain arising from the stomach is felt in the epigastric
region (T8), while that arising from the urinary bladder is felt
at the suprapubic region (S2). Intestinal pains are usually felt
at the umbilical region (T10).
Abdominal pain can also be referred from structures like
pleura, spine, etc., which are outside the abdomen, but whose
dermatomes are represented in the abdominal region. Thus,
basal pneumonia and caries of thoracic vertebrae are frequent
causes of abdominal pain, which may be missed, if the phe-
nomenon of referred pain is not kept in mind.
ACUTE ABDOMINAT PAIN
'When a child is brought in with pain in abdomen for the first
time, a carefi;l evaluation is required for an underlying surgical
cause. Age of the child and attendant circumstance are helpful
in making diagnosis. \(/henever pain is the first symptom in a
triad of pain, vomiting, and fever, underlying surgical causes
should be strongly considered. On the other hand, in similar
circumstances when fever and vomiting precede the onset of
pain, the underlying cause is more like to be medical in nature.
Surgical causes:
o
Acute intussusception: Ihis is caused by the sliding in of
the proximal part of intestine into a distal part, causing
5
131
.......,....132
acute obstruction. Ileo-colic intussusception is the most
.
common.
Acute appendicitis: Ir can occur ar any age, but is com-
paratively less common in childhood. Usually triad of
pain, vomiting, and fever is seen, but the site of pain may
be arypical in children as the cecum (and the appendix) is
quite often in right lumbar or right hypochondrial areas
due to incomplete descent. Abdominal examination shows
rebound tenderness and rigidiry. Hemogram shows neurro-
philic leukocytosis. Ultrasound of the abdomen could be
diagnostically helpful. Urgent surgery is required for relief.
Acute intestinal obsnuctioz.. It is ofien caused by congenital
stenosis and strictures of different parts of intestines. These
usually present in newborn period. In older infants and
children, acute obstruction may be due to volvulus, round
worms, or tuberculosis.
Others: Acute cholecystitis, acute renal colic, and acute
pancreatitis are other causes of acute abdomen in children.
Medical causes:
Acute infectiae colitis: This is clinically characterized by
passage of small frequent stools, with mucus and blood,
and a feeling of tenesmus. Fever is often present. The most
common cause is shigellosis and treatment is with suitable
antibiotics. Ascariasis, food allergy, and drugs can also give
rise to acute abdominal pain. :
\
Acute mesenteric lympbadenitis: This condition is also
characterized by occurrence of pain, vomiting, and fever,
\
and may closely mimic acute appendicitis. However, rebound
tenderness is almost never present. The usual cause of this
otherwise selfJimiting condition is a viral infection.
Pneumonia: This is very frequent cause of apparent abdomi-
\
nal pain. There may be a rapid breathing, and careful I
examination of chest would reveal the correcr diagnosis. t
i
I
!

Others: Acute hepatitis, congestive heart failure causing
sudden enlargement of liver, acute pyelonephritis and consti-
pation are other causes of acute abdominal pain in children.
RECURRENT ABDOMINAT PAIN
The syndrome of recurrent abdominal pain (RAP) is very
common in school-age children. Apley defined RAP as occur-
rence of more than two episodes over a period of 3 months.
To rule out organic causes, the following investigations
should be done: stool, urine, MT/BCG tests; chest x-ray,
Ba-meal x-ray, USG of abdomen. If negative, child may have
psychogenic pain.
Causes:
o Organic:
o Gastrointestinal: Gastroesophageal reflux, peptic ulcer dis-
ease, non-ulcer dyspepsia, intestinal worms, tuberculosis
o Renal: Urolithiasis, urinary tract infection
o Recurrentcholecystitis
o Pancreatic: Recurrent/chronic pancreatitis
o Extra-abdominal: Spinal caries, root pains
o Psychogenlc.' Attention seeking
Features suggesting organic causes of RAP include (i) age <3
years; (ii) pain well-localized, consistent, away from umbilicus;
(iii) tenderness/rigidiry on local examination; (iu) accompany-
ing symptoms like fever, rash, joint pain; (u) jaundice; and (ui)
raised blood pressure.
However, for a definitive diagnosis of
psychogenic pain
abdomen, it is very important to establish "secondary gain
pattern", i.e., what is that which the child is achieving by
having the pain abdomen. School absenteeism, attention seeking
from parents, etc. are important secondary gains that the child
may be achieving. Help of a specialist would be required to
evaluate such children.
Treotment of RAP
o Ifan organic cause, like dyspepsia, gastroesophageal reflux
disease, peptic ulcer disease, urinary tract infection, chronic
colitis, constipation, etc. is found, they would need appro-
priate management.
o For psychogenic pain in abdomen, it must be realized that
the pain is real as far as the child is concerned. It should
never be suggested that child is only feigning symptoms
or the pain is in the "head". This will only worsen the
situation. Brief period of observation and assurance that
there is nothing seriously wrong with the child helps to
manage the child. Most children usually overcome their
problem, if is given to the child himself'
less attention
The cause of psychogenic RAP should be found out and
desensitized.
DISEASES OF GASTROINTESTINAL SYSTEM
NG
UPPER GASTROINTESTINAL BTEEDI
Upper gastrointestinal bleeding denotes bleeding from any
site in the gastrointestinal tract. It can present as hematemesis
(coffee ground vomitus) and/or melena (black tarry stools).
Massive upper gastrointestinal bleeds are known as hemato-
chezia (frank bleeding per rectum).
Common Couses
Newborns:
o Swallowed blood syndrome
o Hemorrhagic disease of newborn
r Septicemia and disseminated intravascular coagulation (DIC)
o Stress ulcers
r Necrotizing enterocolitis
Infants and children:
o Variceal bleed
o Drug induced-erosive gastritis
o Viral hemorrhagic fevers (e.g., dengue)
o Reflux esophagitis with hiatus hernia
o Mallory \feiss tear due to severe vomiting
o Gastric/duodenal ulcer (rare)
o Others: bleeding diathesis, scur\y
Clinicol Feoiures
Acute gastrointestinal bleed leads to hypovolemia and shock,
while chronic gastrointestinal bleed usually produces severe
anemia. The important points to be evaluated are:
o History: H/O previous jaundice or chronic liver disease
such as portai hypertension, and drug ingestion, especially
NSAIDs. Recurrent bleeds are common in variceal bleeding.
Painful bleed is usually seen in acid peptic diseases (APD),
gastroesophageal reflux disease (GERD), while painless
bleed is seen in stress ulcers and variceal bleeds. Bleeding
from other sites suggest coagulopathy, bleeding diathesis,
septicemia and/or DIC.
o Signs:
c Vital signs
o Signs ofchronic liver disease and portal hypertension
o Epigastric pain/tenderness
Treolmenl
r Check vital signs, and look for signs of hypovolemic shock
including (z) tachycardia, (ii) hypotension, and (iii) posturd'
fall in blood pressure. If the patient is in shock, resuscitate
with IV funger lactate, normal saline, or blood transfusion.
Emergency investigations, including hemoglobin, hemato-
crit, blood grouping/typing, should be done.
ESSENCE OF PEDIATRICS
a Nasogastric intubation, aspiration, and normal saline lavage.
o Parenteral somatostatin or its analogue to arresr rhe bleed.
a Once the patient is stable, urgenr upper GI endoscopy (within
G12 hour) to diagnose the cause of bleed should be done.
Further management depends on the cause and is discussed
under three categories: (a) varceal bleed, (/) GERD, reflux
esophagitis, hiatus hernia, Mallory \X/eiss rear, and (c) APD,
stress ulcers, erosive gastritis.
(a) Variceal bleeding
o Emergency endoscopic sclerotherapy (EST) or variceal
ligation/banding.
o Continue somatostatin or its analogue for 3 more days to
prevent early rebleeds. If early rebleed persists, IV vaso-
pressin andlor esophageal balloon tamponade.
o Management of late rebleeds (recurrent variceal bleeds
that occur 2-3 weeks after therapy).
Periodic or chronic EST every 3 weeks
- TIPS (transjugular intrahepatic portosystemic shunt)
- in older children
Surgery (shunt and non-shunt procedures)
-
(b) Gastroesophageal refux diseases
o Head-up position and low fat diet
o Hr-receptor antagonists, proton-pump inhibitors, and
prokinetics constitute the medical line of managemenr.
o Surgery.
(c) Acid peptic diseases
,r Screen for H. pylori, and treat with metronidazole
amoxicillin + proron-pump inhibitors.
o If negative, treat with antacids, Hr-antagonists, proton-
pump inhibitors.
o Laser photocoagulation for immediate control of ulcer
bleed.
TOWER GASTROI NTESTI NAt BTEEDING
o Common causes of lower gastrointestinal bleeding:
o In newborns
Swallowed maternal blood
- Hemorrhagic disease of newborn (HDN)
- Necrotizing enterocoliris (NEC)
- Malrotation and mid-gut volvulus
o -In infants and older children
Anal fissure
- Intussusception, gangrenous bowel due to volvulus
- Polyps
- Qa511ssnteritis, colitis
- Meckelt diverticulitis
- Inflammatory bowel disease
- AV malFormations
-
r Swallowed maternal blood is confirmed by positive Apt test.
o Hemorrhagic disease of the newborn responds promptly to
vitamin K therapy.
o NEC presents as recral bleeding, abdominal distension in
preterm or LBV baby or a poor, sick baby not on breastfeeds.
Plain x-ray abdomen usually shows pneumatosis intestinalis.
o Malrotation with mid-gut voh'ulus presenrs as sudden bilious
vomiting and passage of dark blood in stool. An urgenr
exploratory surgery should be done.
r Anal fissure presents with constipation and painful passage
of streaks of blood in stool. Direct anorectal examination
confirms the diagnosis. fieatment consisrs of stool softeners
and sitz bath.
o Intussusception presents as episodic abdominal pain, vomit-
ing, a sausage-shaped upper abdominal mass (in 60%o cases),
and red currant jelly stools. Barium enema is diagnostic and
may prove rherapeutic.
o Bleeding due to polyps is generally painless, episodic, and
rarely, severe. It is mostly found in rectosigmoid region,
and is identified by digital recral examination. Colonoscopy
is usually diagnostic. The treatmenr consisrs of
snare
polypectomy.
o Meckel diverticulum presenrs as significant painless bleeding
per rectum, which terminates abruptly after 1 or 2 days.
Diagnosis is scintiscan (nn-T.). Early surgery is recom-
mended.
e Angiomatous malformations are diagnosed by selective
angiovenous phase study and/or DSA (digital substraction
angiography).
Remember, massive upper gastrointestinal bleed can also present
as rectal bleed. Upper gastrointestinal endoscopy may be
+
required.
Diarrhea with or without blood that begins acutely and lasts
for 74 days or longer is called persistent diarhea (PD). The
etiology and pathogenesis of PD is complex and multifactorial,
and infective, nutritional, and allergic factors are usually associ-
ated with its cause. Most pathogens that cause acure diarrhea
may also cause PD with notable exceprion of Vibrio cbolerae.
Classification:
r
Severe persistent diarrhea (persistent diarrhea with some or
severe dehydration)
o Non-severe persistent diarrhea (persistent diarrhea with no
sign of dehydration)
Possible risk factors:
o Host factors-young age, malnurrition, impaired cell-me-
diated immuniry and low birth weight
o Previous infection-recent acute diarrhea and previous
persistent diarrhea
r Feeding practices-recent introduction of animal milk
o Microbial isolates during acure phase-Enteroadherent \
-E
coli, ShigelIa, and more than one parhogen
o Inappropriate management of acute diarrhea \
t
t
ta

Dietary: Food withholding, lack of breast-feeding, feeding
of non-human milk as predominant source, and unmodi-
fied bovine milk
a Drugs: Inappropriate antibiotic use, antimotiliry agents
a Intestinal-Infective (common enteric bacterial infection),
small bowel overgrowth, continuing mucosal injury, reduced
production of disaccharidase enzyme
l o
Extraintestinal-Urinary tract infection, respiratory tract
I
I infection
L
r Food allergic enteropathies-Cowt milk protein intolerance,
IL soy protein intolerance, multiple food intolerance
Etiologic agents in persistent diarrhea:
i tolyticawithin red blood cells;
l o Isolatedwith equal frequencyfrom episodes ofacute diarrhea
v OR
and persistent diarrhea-shigella, non-typhoid salmonella,
enteropathogenic E. co li, Campy lo b acter j ejuni, Aeromonas
v sp., C. dfficile, E. histolytica, Giardia lamblia.
t o Isolated with greater frequency from PD: Enteroadherent
I E. coli, enteropathogenic -d. coli, enteroinvasive E. coli,
I cryptosporidium.
Investigations:
o Blood: CBC, electrolytes, blood glucose, serum total protein
and albumin
a Stool: R/M/E, culture, pH, reducing substance, neutral fat
a Urine: R/M/E, culture
o Duodenal intubation: Culture for anaerobic and aerobic
bacteria, M/E for Giardia
o Sudan III staining, electrolytes and osmolaliry, lactose hydrogen
breath test
SEVERE PERSISTENT DIARRHEA
Diognosis
Infants or children with diarrhea lasting 14 days or more with
signs ofdehydration have severe persistent diarrhea and require
hospital treatment.
Treotmenl
t" o Assess the child for signs of dehydration and give fuids
r according to teatment Plans B or C, as appropriate.
ORS solution is effective for most children with persistent
I diarrhea. In a few, howeve! glucose absorption is impaired,
f'
and ORS solution is not effective. 'W{hen given ORS, their
stool volume increases markedly, thirst increases, signs of
i dehydration develop or worsen, and the stool contains a
! large amount of unabsorbed glucose. These children require
t IV rehydration until ORS solution can be taken without
v worsening the diarrhea.
I Routine treatment of persistent diarrhea with antibiotics
t Feeding should be given as soon as the child can eat. Food
is not efFective and should not be given. Some children,
however, have non-intestinal or intestinal infections that
require specific antibiotic therapy.
DISEASES OF GASTROINTESTINAL SYSTEM
r Examine every child with persistent diarrhea for non-
intestinal infections such as pneumonia, sepsis, urinary
tract infection, oral thrush, and otitis media, and treat
appropriately.
o Give micronutrients and vitamins
o Tieat persistent diarrhea having blood in the stools with
an oral antibiotic effective for Shigella (e.g., ciprofloxacin,
pivmecillinam, and other fluoroquinolones.
o Give treatment for amebiasis (oral metronidazole 7.5
mg/kg, 3 times a day for 5 days) only if:
c microscopic examination of fresh feces, carried out in a
reliable laboratory, reveals trophozoites of Entarnoeba his'
o two different antibiotics, which are usually effective for
Sbigella locally, have been given without clinical im-
provement.
o Give treatment for giardiasis (metronidazole 5 mglkg, 3
times a day for 5 day$ if cysts or trophozoites of Giardia
larnblia are seen in the feces.
Feeding
Careful attention to feeding is essential for all children with
persistent diarrhea.
Breast-feeding should be continued for as often and as long
as the child wants.
o Other food should be withheld for 4-6 hours-only for
children with dehydration who are being rehydrated fol-
lowing fieatment Plans B or C.
HospitalDiets
Children treated in hospital require special diets until their
diarrhea improves and they start gaining weight. The goal is
to give a daily intake of at least 1i0 calories/kg.
lnfants Aged <6 Months
o Encourage exclusive breast-feeding; help mothers who are
not breast-feeding exclusively to do so.
r If the child is not accepting breast-feed, give a breast milk
substitute that is low in lactose (such as yoghurt) or is
lactose-free. Use a spoon or cup; do not use a feeding bottle.
Once the child improves, help the mother to re-establish
Iactation.
o If the mother is not breast-feeding because she is HIV-
positive, she should receive appropriate counseling about
the correct use of breast milk substitutes.
(hildren Aged >5 Months
should be given 6 times a day to achieve a total intake of at
least 110 calories/kgld. If patient eats poorly, he/she can be
given nasogastric feeding for initial 24-48 hours.
- .i

ESSENCE OF PEDIATRICS

Two Recommended Diets Supplementory Multivitomins qnd Minerols

Given below are rwo diers recommended for children aged >6 Give all children with persistent diarrhea daily supplementary
months with severe persistent diarrhea (Diet l-starch-based, multivitamins and minerals for 2 weeks. fhese should provide
reduced milk concentration [low lactose] diet and Diet 2-no as broad a range ofvitamins and minerals as possible, lnclr-rdlrrg
milk fiactose-free] diet with reduced cereal [starch]). If there at least two recommended daily allowances (RDAs) of folate,
are signs of dietary failure (see below) or if the child is not vitamin A, zinc, magnesium, and copper.
improving after 7 days of rreatment, the first diet should be
stopped and the second diet given for 7 days. Successfrrl As a guide, one RDA for a child aged I year is:
treatment with either diet is characterized by: o Folate: 50 pg
o Adequate food intake t Zinc: l0 mg
r \Weight gain o Vitamin A:400 pg
o Fewer diarrheal stools
r Iron: 10 mg
o Absence of fever o Copper: I mg
r Magnesium: 80 mg
The most important criterion is weight gain. There should be
at least 3 successive days of increasing weight before one can Moniioring
conclude that weight gain is occurring.
Nurses should check the following daily: bodyweighr, remperarure,
Give additional fresh fruit and well-cooked vegetables to
children who are responding well. After 7 days of t.."t-..rt food taken, and number of diarrhea stools.
with the effective diet, they should resume an appropriate
diet for their age, including milk, which provides at least 110 NON.SEVERE PERSISTENT DIARRHEA
calories/kg/d. Children may then rerurn home, but follow them
These children do not require hospital trearmenr, but need
up regularly ro ensure continued weight gain and compliance
special feeding and extra fluids at home.
with feeding advice. Dietary failure is shown by:
r An increase in stool frequency (usually >10 watery stools a Diognosis
day), often with a return of signs of dehydration (this usually
Children with diarrhea lasting 14 days or longer who have no
occurs shortly after a new diet is begun); OR
signs of dehydration and no severe malnutrition.
e A failure to establish daily weight gain within 7 days.

Diet l: Starch-Based, Reduced Milk(oncentration (low lactose) Diet The Treoimenl

diet should contain ar leasr 70 calories/100 g; provide milk or o Tieat the child as an outpatient.
yoghurt as a source of animal protein, but no more than 3.7 o Give micronutrients and vitamins according to supplemen-
g lactose/kg body weight per day, and should provide ar leasr tary multivitamins and minerals (described earlier).
70o/o of calories as protein. The following example provides o Prevent dehydration: Give fluids according to Tiearmenr
83 calories/l}} g,3.7 g lactose/kg body weight per day and Plan A. ORS solution is effective for most children with
llo/o of calories as protein: persistent diarrhea. In a few, however, glucose absorption is
r Full-fat dried milk (orwhole liquid milk,85 ml): 1l g
impaired and when given ORS solution their stool volume
o Rice: 15 g increases markedly, thirst increases, signs of dehydration
o Vegetable oil: 3.5 g develop or worsen, and the stool contains a large amount
o Cane sugar: 3 g of unabsorbed glucose. These children require admission
o Water to make: 200 ml to hospital for IV rehydration until ORS solution can be
taken without aggravating the diarrhea.
Diet 2: No milk (lactose-Free) Diet with Reduced (ereal
(Starch) The o Identifr and treat specific infections: Do not routineb
second diet should conrain ar leasr 70 calories/l00 g, and treat with anilbioilcs as thqt are not ef,ectiue. However, give
provide at least l0o/o of calories as protein (egg or chicken). antibiotic trearmenr to children with specific non-intestinal
The following example provides 75 calories/100 g: or intestinal infections. Until these infections are rreared
correctly, persistent diarrhea will not improve.
r \7hole egg:64 g
o fuce:3g ,r Non-intestinal infections: Examine every child with
o Vegetable oil 4 g persistent diarrhea for non-intestinal infections, such as a

o Glucose: 3 g pneumonia, sepsis, urinary tract infection, oral thrush,

r \7ater to make: 200 ml and otitis media, and treat with appropriate antibiotics.
r Intestinal infections: Theat persistent diarrhea with
1
Finely ground, cooked chicken (12 g) can be used in place of blood in the stool with an oral antibiotic that is effective t
egg to give a diet providingT0 calories/l00 g. for Shigella.
i
I
t
1

Feeding
Careful attention to feeding is essential for all children with
persistent diarrhea. These children may have difficulty in digest-
ing animal milk other than breast milk.
o Advise the mother to reduce the amount of animal milk in
the child's diet temporarily.
o Continue breast-feeding and give appropriate compiemen-
tary foods:
o If still breast-feeding, give more frequent, longer breast-
feeds, by day and night.
o If taking animal milk, explore the feasibiliry of replacing
animal milk with fermented milk products (e.g., yoghurt),
which contain less lactose and are better tolerated.
o If replacement of animal milk is not possible, limit animal
milk to 50 ml/kg/d. Mix the milkwith the childt cereal, but
do not dilute it.
o Give other foods appropriate for the childt age to ensure
an adequate caloric intake. Infants aged >4 months whose
only food has been animal milk should begin to take solid
foods.
o Give frequent small meals, at least 6 times a day.
Su pplemenlory Micron ulrienls
(lncluding Zinc)
Same as in severe persistent diarrhea, described under the
heading "Supplementary Multivitamins and Minerals".
Follow-up
Ask the mother to bring the child back for reassessment
after 5 days, or earlier if the diarrhea worsens or other
problems develop.
ll, Completely reassess children who have not gained
weight or whose diarrhea has not improved in order
to identify any problems, such as dehydration or
infection, which need immediate attention or admission
to hospital.
111. Those who have gained weight and who have less than
three loose stools per day may resume a normal diet for
their age.
Malabsorption syndromes are characterized by the association
of chronic diarrhea, abdominal distension, and failure to thrive.
Chronic diarrhea is the direct consequence of malabsorption,
which in turn results in malnutrition and failure to thrive. Chronic
diarrhea has to be differentiated from persistent diarrhea, which
DISEASES OF GASTROINTESTINAL SYSTEM
is very common. General therapeutic approach for management
ofchronic diarrhea has been depicted in Fig. 8.1.
Persistent diarrhea starts as an acute diarrhea that lasts for
more than 14 days and associated with rapid weight loss.
Occasionally, the purge rate is so high that dehydration also
occurs. Persistent diarrhea mostly occurs in children below 1-2
years. Chronic diarrhea here refers to non-infectious conditions
associatedwith "abnormal stools" that continue or recurrently
occur over several months. Dehydration is rare. Stools may
be loose and bulky in celiac disease, pasty and yellowish in
exocrine pancreatic insufEciency, passed noisily with flatus in
cases of sugar intolerance.
Diarrhea due to exocrine pancreatic insufficiency is remark-
able by the macroscopic appearance of the stools; these are
more frequently loose and pasry than liquid, often obviously
greasy with undigested fat oozing like oil from the stool when
it is passed in a pot or floating on the surface of water in the
toilet, pale, with an offensive cheese smell.
Diarrhea due to intestinal malabsorption is loose or l.iquid,
often with an acidic smell, rarely greasy. These patients
have modest steatorrhea, abnormal D-xylose test, and
abnormal intestinal histology. Intestinal biopsy is necessary to
differentiate chronic diarrhea due to different pathophysioiogic
reasons.
Diarrhea due to fermentation is liquid, acidic (pH < 5.5) and
often passed with flatus, and its volume is variable, roughly
proportionate to the amount of malabsorbed carbohydrate
that has been ingested.
CTASSIFICATION
According to pathophysiology, three major categories are:
1. Chronic diarrhea due to impaired intraluminal digestion
All nutrients Cystic fibrosis, other pancreatic exocrine
def iciencies.
Fat lsolated lipase deficiency or bile duct atresia,
interrupted enterohepatic circulation (e.g..
Crohn's).
Protei ns Congenital trypsinogen deficiency. congenital
enterokinase deficiency.
2. Chronic diarrhea due to intestinal malabsorption
Total villous atrophy Celiac disease
Partial vi Ilous atrophy Foocl prolein sensitivity,e.g., cow milk
protein, wheat), Ciardla /amblla infestation,
J
immunodefic iency, bacteria I overgroMh,
tropical enteropathy, malnutrition. l
ESSENCE OF PEDIATRICS

3. Chronic diarrhea due to fermentation CtINICAt FEATURES

Symptoms: Anorexia, diarrhea, vomiting, abdominal distension,

Normal biopsy Congenital deficiency of mucosal enzymes
increased flatus, stool may be warery, foul smelling, bulky
digest i ng mono/dlsaccharides.
or may be floating, and malnutrition & failure to thrive
Non-specific Allconditions associated with total and
(a direct consequence of malabsorption).
inflammatory partial villous atrophy have carbohydrate
malabsorplion and fermentation.

Weight and height normal, Weight loss,

nutritional status normal, stools show fat
stools show no fat

Diagnostic lests
to determine
etiology

Excessive Excessive intake Fat intake low Specific

intake oJ fruit of carbonated treatment
lurces fluids

Decrease fluids
Decrease fruit lncrease fat to
to no more than
Jurces 3540%
90 ml/kg/d

ry
Lactose intolerance

Decrease lactose
intake
Add lactase tablets
ff

Lactose-free diet Sucrose-free diet I

Fig. 8.1: Ceneral therapeutic approaches for management of chronic diarrhea

 I
DISEASES OF GASTROINTESTINAL SYSTEM

Signs: PRINCIPTES OF TREATMENT

o Carbohydrate Intolerance
Generalized
,r Offending carbohydrates to be removed from diet (e.g.,
Weight loss AII types
lactose etc.)
Anemia Rare in uhronic pant realitis
o Enzyme replacement therapy (e.g., lactase, sucrase, pan-
Edema Hypoproteinemia creatic enzymes, etc.)
Ascites Hypoproteinemia
Fat Malabsorption
Abdominat distension [xcess gas due to baclerial {ermentdtion
o Low fat diet
Skeletal abnormalities Osteomalacia, childhood rickets
,> Correction of fat-soluble enzyme deficiencies
Skin
o Fat soluble vitamin supplementation
Pigmentation Severe disease c MCT based formula supplementation
Clubbing of fingers Crohn disease, lymphoma. t eliac di'ease r Pancreatic enzyme replacement
lchthyosis Falty ac id deficiency , Caloriesupplementation
Purpura Vitamin K deficiency Protein Malabsorption
Mouth ,r Protein and calorie supplementation
Angular stomatitis lron deficiency ,r Treatment of underlying disease
Clossitis lron & vitamin B deficiency o Correction of any co-existing nutritional abnormalities
Ma8enta tongue vilamtn b oeltctencv
Patients with specific GI disorders require disease specific
Aphthous ulcer Celiac disease, Crohn disease therapy. Patients with ceiiac disease require gluten-free
Neurological and aiso for a short period lactose-free diet. Giardia infbc-
Night blindness Vitamin A deficiency tion is treated with metronidazole and other antiparasitic
Peripheral neuropathy Deliciency of Vit 8., 8,. or Vit E. medication. Acrodermatitis enteropathica requires zinc
supplementation. Patients with ileal resection and vitamin
INVESTIGATIONS 8,, malabsorption need parenteral vit B,, therapy. Patients
with IBD need specific drugs along with high protein and
1. Diet record: 3 days vitamin supplementation.
2. Stools: Pus cells, blood, ova & parasites Management depends on the cause. Some patients with
3. Blood: Complete blood count, serum electrolytes decreased calorie intake and increased calorie requirement may
4. Specific tests for malabsorption: need calorie supplementation either orally, through NG tube,
A. Carbolrydrate malabsotTttion: or by parenteral nutrition. Calorie intake of l20o/o to l50o/o of
i) Stool pH & reducing substance the recommended daily allowance may be required for catch up
ii) Stool electrolytes, bicarbonate, & osmolality growth. Specific nutrient abnormalities need to be corrected.
iii) Lactose & breath hydrogen test
iv) Xylose absorption test
v) Small bowel biopsy for pathology & enzyme
analysis
Ceiiac disease, a gluten-sensitive enteropathy, is a permanent
B, Fat malabsorT,tion:
intestinal intolerance to dietary wheat gliadin and related pro-
i) PT/PTT teins that produce lesions in genetically suscepdble individuals.
ii) S. carotene/25-hydrory vit D It is an immunologically mediated small intestinai enteropathy.
iii) Fecal smear for fat (qualitative) The mucosal lesions suggest both cell mediated and humoral
iv)
72-hours fecal fat estimation (quantitative)
immunological over-stimulation.
v)
Pancrearic srimularion test:
vi)
Small bowei biopsv
I C. Protein malabsoryttion: CLINICAL FEATURES
I i) S. total protein & albumin
I ii) Urinalysis The common features of celiac disease are intestinal symptoms:
! iii) Small bowel biopsy chronic diarrhea, abdominal distension, muscle wasting, failure
I
t 5. Miscellaneous tests: to thrive, anorexia, and irritabilitv. Ciinical history reveals that
A. Urinalysis after initial normal collrse, child starts having foul, greasy, and
B. Sweat test for cystic fibrosis bullgr stools weeks to months after introduction of wheat into
C. S. folate & vit B,, level the diet; the weight gain stops, the appetite decreases, and the
D. Bone age child appears miserable.

I'

ESSENCE OF PEDIATRICS
Extra-intestinal symptoms are more common in children
who present late. In these children, short stature is a promi-
nent sign.
DIAGNOSIS
Complete hemogram, serum chemistry, and tests measuring
intestinal absorption, such as D-xylose absorption, fecal fat
excretion.
The two requirements mandatory for the diagnosis of celiac
disease are (i) uillous atrophy with hyperplasia of the crytpts and
abnormal sulface e?ithelium while the patient is eating adequate
amounrs of gluten, and (ii) full clinical and histological remission
afer withdrawal of gluten from the diet.
The presence of circulating antibodies to gliadin, reticulin,
and endomysium at the time of diagnosis, and their disappear-
ance on a gluten-free diet add weight to the diagnosis.
TREATMENT.
A strict gluten-free diet has become the cornerstone of manage-
ment of celiac disease. Rice and maize are nontoxic and act as
wheat substitutes. Iron and vitamin supplementation is advis-
able. The clinical response to gluten withdrawal is dramatic.
The growth velocity improves rapidly. The major problem is
of noncompliance, especially in teenagers. Iron and vitamin
supplementation is advisable.
Data available about prognosis suggest that there is increased
risk of lymphoproliferative diseases in patients on a normal
gluten-containing diet. A lifeJong strict gluten-free diet is
mandatory for children with celiac disease.
Symptoms of milk protein intolerance (MPI) manifest within
the initial exposure to cow milk. Clinical manifestation of milk
protein allergy includes diarrhea, vomiting, abdominal colic,
irritability, failure to thrive, and gastrointestinal hemorrhage.
Clinical intolerance to milk protein usually subsides by the
second year of life.
Diagnosis: The diagnosis of milk protein allergy is difficult.
Lactose intolerance should be first excluded. Jejunal biopsy shows
a patchy villous atrophy. The gastrointestinal symptoms improve
on elimination of milk from the diet and recur usually within
34 days of challenge with milk. Positive response to at least three
challenges with milk protein establishes the diagnosis.
Treatment: See treatment of persistent diarrhea.
Cystic fibrosis is an inherited disorder with autosomal recessive
transmission. AII the exocrine glands in the body, especially
!
those in the respiratory and gastrointestinal system, are involved
and produce abnormal viscid mucus. The inspissated secretions
obstruct the pancieatic ducts, and cause distension of acini,
which appear like cysts. These are surrounded by fibrosis. Pan-
creatic secretions do not pass into the duodenum. This leads
to impaired digestion and absorption. It has been estimated
that l0olo of patients may not show evidence of pancreatic
dysfunction. These patients do not suffer from other gastro-
intestinal complications.
In the lungs, thick mucus plugs obstruct bronchioles leading
to collapse, stasis, and infection. Parenchymal and bronchial
damage cause chronic pulmonary disease.
In the newborn period, thick meconium plugs (which have
not been liquefied by the pancreatic juice in the intestinal tract of
fetus) may cause meconium ileus. Increased secretion of chlorides
in the sweat may be an independent associated defect.
CtINICAI FEATURES
Intestinal obstruction may be an initial manifestation in
the neonatal period (meconium ileus). This may be due to
the presence of abnormal protein and mucoprotein secreted
by the pancreatic enzymes. Later, these children tend to
retain the food residue in the ileum, cecum, and colon, and
these may form firm masses. Impaction of the feces causes
intestinal obstruction. Ileocecal intussusception may occur
in <1% of the patients.
Two cardinal symptoms of the disease are chronic diarrhea
with massive steatorrhea and recurrent respiratory tract
infections.
a Failure to thrive is also a prominent feature.
a Rectal prolapse occurs frequently.
a Biliary cirhosis of liver is also often observed.
DIAGNOSIS
The diagnosis is suspected from the onset of diarrhea early in
infancy, usually associated with recurrent respiratory infections.
o Absorption test is normal,as monosaccharide does not need
hydrolysis before absorption.
o typsin in the duodenal juice and stools is reduced.
o X-ray of the chest shows pulmonary involvement. X-ray
abdomen for meconium ileus.
o Analysis of the sweat for chlorides is a reliable diagnostic
test. Level of chlorides >60 mEq/L in sweat obtained by
pilocarpine iontophoresis is suggestive of the diagnosis.
TREATMENT
The diet is introduced gradually. In the first phase, simple
tolerable nutrients are given. The calorie supply is built up
gradually, proportionate to the tolerance ofthe patient. In the
!
maintenance phase, the diet should be adequate to maintain t
I
i
I

growth and prevent specific nutrient deficiencies. The diet
should be rich in protein and sugars. Vegetable fats rich in
polyunsaturated fatty acids are preferred over the animal fats.
o Pancreatic supplement is given in a dose of 5-10 tablets
daily depending on the patientt clinical response. If the
diarrhea persists in spite of adequate therapy, other causes
like secondary disaccharide intolerance should be considered
and managed accordingly. Thurine supplements should be
given to provide substrate for increased hepatic synthesis of
bile acids. In resistant cases, misoprostol (a prostaglandin
analogue) had been used to inhibit gastric acid secretion
and stimulate bicarbonate secretion in upper gut.
. Suitable antibiotics (e.g., ciprofoxacin in Pseudomonas
infection) are essential for preventing pulmonary compli-
cations.
o Humidification of the inspired air helps. Aerosol therapy
with mucolltic agents such as acetylcysteine may be useful as
adjunct prior to the postural drainage ofsecretions. Breathing
exercises with chest physiotherapy are encouraged.
o Meconium ileus: Gastrograffin enema may help in reliev-
ing the obstruction. Gastrograffin has high osmolaliry and
therefore, draws water into the gut. This helps in expulsion
of the meconium.
Crohn disease-an idiopathic, chronic infammatory disorder
of the bowel-involves any region of the alimentary tract
from the mouth to anus. Infammatory process is segmental,
transmural, often with skip areas. Refer Thble 8.1 for features
differentiating Crohn disease from ulcerative colitis.
CtINICAt FEATURES
Manifestation depends upon regions of bowel involved, the
degree of infammation, and the presence of complications
(such as stricture or fistula).
o Children with ileocolitis rypically have crampy abdominal
pain and diarrhea often with blood. Ileitis may present with
right lower quadrant pain alone; colitis may be associated
with bloody diarrhea, tenesmus, and urgenry. Fever, malaise,
and easy fatiguabiliry are common. Growth failure with
delayed bone maturation also occurs.
r Partial small bowel destruction due to inflammation or
stricture may cause crampy abdominal pain, borborygmi,
and intermittent abdominal distension.
o Enteroenteric or enterocolonic fistulas are often asymptom-
atic, but may contribute to malabsorpdon. Enterovesical
fistulas originate from ileum or sigmoid colon and present
with signs of urinary tract infection, hematuria, or fecaluria.
Enterovaginal fistula originates from rectum and presents
with feculent vaginal discharge.
t
DISEASES OF GASTROINTESTINAL SYSTEM
o Hepatic, splenic, anorectal, psoas abscess and perianal fistula
may also occur. Extra-intestinal manifestations include
peripheral arthritis, ery"thema nodosum, digital clubbing,
renal stones, and gall stones.
DIAGNOSIS
o A complete blood count demonstrates anemia, normal or
raised ESR, elevated platelet count (>600,000/mm3), normal
or raised leukocyte count. Serum albumin level may be low
and stool cr,-antitrypsin may be elevated.
o Plain x-ray abdomen may be normal or may demonstrate
findings of partial small bowel obstruction or thumb
printing of colon wall. In contrast study, linear ulcers
may give a cobblestone appearance to the mucosal
surface.
o Colonoscopy with biopsy can be more helpful. Findings
on colonoscopy include patchy nonspecific inflammatory
changes, aphthous ulcers, linear ulcers, nodulariry and
strictures.
o USG and CT scans are most useful to detect intra-abdom-
inal abscess. MRI can localize areas of active disease and
can be safely used in pregnancy.
TREATMENT
e There is no single medical approach, and the aim of treat-
ment is largely to alleviate symPtoms.
o If the diseaseis largely limited to small bowel, oral Pred-
nisolone \-2 mglkgld (max. 60 mg/d) as single morning
dose is often the first treatment. As the disease become
quiescent (often 3-4 week), alternate day prednisolone
is used.
o If the disease is largely limited to colon, Sulfasalazine 50-
75 mglkgldin2-4 divided doses (max. 2-3 gld) is more
effective. Prophylactic use of sulfasalazine may reduce the
risk ofrecurrence.
o In steroid-dependent or steroid-unresponsive cases, Aza-
thioprine (or its metabolite 6 MP) 1-2 mg/kg/d can be
used. Metronidazole is also used in intractable Crohn
disease.
o In perianal fistula, Ciprofloxacin and Clarithromycin
can be used rvith sitz baths. Monoclonal antibody
(infliximab) is effective in moderate to severe Crohn
disease.
Nutritional therapy is an efFective primary treatment. Total
parenteral nutrition is effective not only in nutritional reple-
tion but also is quieting the active disease.
Surgical approach is to remove as small a region of bowel
as possible up to the margin that are free of disease. Intra-
abdominal, liver, perianal abscess require surgical drainage.
Perianal fistula should be managed medically, if severe by
fistulectomy.
ESSENCE OF PEDIATRICS

of Crohn Disease and Ulcerative Colitis earliest changes are fine granulariry followed by a more
course granularity with severe disease. "Collar button" ulcers

Rectal bleeding Sometimes Common

with submucosal undermining may be seen. Mucosal folds
become thickened and may be completely obliterated to give
Abdominal mass Conrmon Not present
a smooth appearing surface-"lead pipe colon'.
Rectal disease Occasional Nearly universal
r Endoscopy is more sensitive and offers the opportunity for
lleal involvement Common Unusual simultaneous biopsy.
Perianal disease Common Unusual
Stricture Conrmon Unusual
TREATMENT
Fistuli Common Unusual
Skip lesions Common Not present A medical cure for ulcerative colitis is not available; rreatmenr
Transmural involvement Common Unusual is aimed at conrrolling symproms and reducing the risk of
crypirabscesses
fecurrence.
Less common Common
Cranulomas Common Unusual Nutritional support can be provided by either enteral or par-
Risk for colonic cancer Slightiy increased Creatly inCreased enteral routes; enteral route is preferable. To ensure adequate
Erythema nodosum Common Less common growth, provide nutrients 125-1500/o of RDA with literal
Cholangitis Less common
amounts of micronutrients.
Common
Stroke Less common Common Drugs: Sulfasalazine is the most widely used aminosalicylate
preparation. Starting dose is 50-75lkgld in rwo to four divided
doses (max. 2-3 gld)" In proctitis, aminosalicylate enema
may be given. Hydrocorrisone enema (100 mg) is also used
in proctitis.
Prednisolone is given to children with moderare ro severe
Ulcerative colitis, an idiopathic chronic inflammarory disorder,
pancolitis or colitis unresponsive to sulfasalazine trearmenr,
is localized to the colon and spares the upper GIT. Disease
usuai starting dose of prednisolone is 1-2 mg/kg/d (ma-x.
virtually always begins in the recrum and extends proximally
60 mg/d).
for a variable distance. \iZhen it is localized to the rectum, the
In steroid-unresponsive or steroid-dependenr colitis, azathio-
disease is ulcerative procriris, whereas disease involving the
prine, 6-mercaptopurine, metronidazole, and cyclosporine
entire colon is pancolitis.
may also be used.

Surgical treatment is indicated in intractable or fulminant

CTINICAL FEATURES
colitis, colonic dysplasia, or persistent disease unresponsive to
o Symptoms of mild dysentery are rhe typical presenrarion. medical managemenr. Optimal approach is total colectomy
Tenesmus, urgency, crampy abdominal pain, and nocturnal with an endorectal pull through.
bowel movements suggesr a more severe colitis.
The onset may be insidious with gradual progression of
symptoms but can be fulminant. Fever, severe anemia,
hypoalbuminemia, leukocytosis, and stool frequency of >6 Acute and chronic inflammation of the liver with varying
days suggest fulminant colitis. Anorexia, weight loss, and
degree of hepatocellular necrosis may be due to a wide range
growth failure may be present. of causes including viral infections, auroimmuniry drugs and
a Chroniciry is an imporiant parr oF rhe disease.
toxins, metabolic, and infiltrative conditions. Chronic low-
a Extra-intestinal manifestations include pyoderma gangreno-
grade hepatitides may progress to cirrhosis despite repeatedly
sum, sclerosing cholangitis, chronic active hepatitis, and
measured nolmal serum transaminases.
ankylosing spondylitis.

DIAGNOSIS
Examination of blood reveals evidence of anemia, hypoal-
buminemia, raised ESR, elevated \fi{BC count. Blood anti-
neutrophil qtoplasmic antibody are presenr tn 650/o of cases.
Plain X-ray abdomen demonstrares loss of haustral mark-
ings in an air-filled colon or marked dilatation with toxic
megacolon. Doubie contrasr barium enema is best. The
VIRAT HEPATITIS
Viral hepatitis is a svstemic viral infection nrarked by diffuse
hepatic ceil necrosis and inflammation. In about 9070 cases,
hepatitis is cased bv viruses namely A, B, C, D, E, E G,
(transfusion associated virus), and in remainder 10%o cases by
CMV HSV EBV VZV adeno, enrero, echo, rubella, mumps
viruses. Table 8.2 lists features of main types of acute hepatitis
with outcome.
 DISEASES OF GASTROINTESTINAL SYSTEM

Table 8.2: Features of Main Types of Acute Hepatitis with Outcome

Type RNA DNA RNA RNA RNA

lncubation period I /wk 6 wk-6 mo 2 wk-6 mo Same as HBV 3-9 wk

Spread:
Feco-oral Yes No No No Yes

Blood No Yes Yes (common) Yes No

7
Sexual Uncommon Yes Uncommon Yes
Vertical No Yes Uncommon Yes No

Chronicitv lrlo Yes: aduit 5-1 OYo Yes (>50%) Yes No

Children:40%;
neonate 9070
.10%
Fulminant hepatitis o.1-1v; Yes Yes if coinfection High in pregnancy
207. if superinfection

Clinicol Feolures Hepatitis A:

Clinical features are often similar. In children below 2 years,
8570 cases of HAV infection may be asymptomatic; 5070 cases
among children aged between 2 and 4 year; and 2070 cases in
children aged 5 years and above. More than 90% childhood
HBV infections are also asymptomatic.
r Pre-icteric phase Anorexia, nausea, malaise, fatigue, lack of
energy, vomiting, supra-orbital headache, diffuse aches and
pain, right upper quadrant pain, diarrhea (in 50o/o children)'
Pruritus and fever (50% cases), serum sickness like syndrome
characterized by low-grade fevet urticarial rash, arthritis.
o lcteric phase: Jaundice and dark urine. Some patients
may have jaundice without any prodromal symptoms, and
rypically once jaundice appears, some of the prodromal
symptoms improve. Jaundice typically persists for <14 days
in case of HAV infection, but it may persist for a longer
period. Two to 3 weeks prior to the onset and up to I week
after the appearance of jaundice, patient remains infectious
in case of HAV infection.
o Post-icteric phase: Nausea disappears, appetite and well-
being returns.
Signs
Jaundice, tender hepatomegaly, splenomegaly (5-1 0%), lymph-
adenopathy, Gianotti-Crosti syndrome. Papular acroderma-
titis is seen on extremities due to vascular immune complex
deposition.
lnvesligolions
a. CBC
b. Liver function tests (LFT): AST,,{LI, bilirubin, aikaline
phosphatase, prothrombin time.
c. Blood urea, serum crearinine.
d. PCR: For detection of viral DNA/RNA
Liver biopsy-confirmatory. It differentiates cirrhosis from
CH.
I Serology
o Anti-HAV IgM detection by radioimmunoassay indicates
acute infection; it is detectable when the svmptoms are
ciinically apparent and remains positive for 4-6 months
after the acute infection.
e Anti-HAV IgG detection alone indicates past infection,
appears within 8 weeks of symptom onset and persists
lifelong.
r of blood is done for
Polymerase chain reaction acute
infection.
Hepatitis B
o HBsAg: Appears 6 weeks after infection and disappears by
3-6 months. The persistence of this antigen for more than
6 months indicates chronicity. It is a marker in both acute
and chronic infection. Negative HbsAg dose not exclude
infection.
o Anti-HBs: Is a protective antibody and appears soon after
HBsAg disappears from serum, and persists perhaps perma-
nendy. It indicates that either a natural protection following
an infection has occurred (anti-HBc will also be +ve) or
the individual has been vaccinated (anti-HBc is -r'e). The
protective titer is i0 mIU/L. Anti-HBs is never produced
in 10-15% of patients despite complete recovery.
o HBeAg: A marker of active viral replication. It usually
for
disappears by 6 weeks; persistence of this antigen >
5 weeks indicates progression to chronicitv.
o Anti-HBe: This is not a protective antibody. It appears afier
the disappearance of HBeAg. In chronic hepatitis following
treatment with antiviral or inrerferon, the seroconversion of
HBeAg to anti-HBe is one of the desired results.
o HBcAg (core antigen) is detected only in the hepatocyte
and not in serum.
o Anti-HBc: This antibody is used in serodiagnosis. Anti-HBc
IgM indicates recent infection and anti-HBc IgG indicates
past infection.
o HBV DNA: The presence of HBV DNA indicates active
replication.
 ESSENCE OF PEDIATRICS
Acute hepatitis B is diagnosed by the presence of HBsAg and
anti-HBc IgM. In chronic hepatitis B, requiring rrearment,
both HBsAg & HBeAg and HBV DNA will be positive.
Serological response to hepatitis B virus infection has been
depicted in Fig. 8.2.
Hepatitis C
r Detection of anti-HCV (positive by 4-B
weeks):
c Enzyme immunoassay (ElA)-\fidely used serological
test, may have false-positive (50-60o/o) and false-negative
results.
o Recombinanr immunoblot assay-Less sensitive, but
more specific than EIA, nor recommended for initial
HCV screening.
. Detection of HCV RNA (positive by 2 weeks): By poly-
merase chain reaction (nucleic acid test) in serum or tissue
sample; become positive within days of infection.
Hepatitis D
o Co-infection is diagnosed by the presence of HBsAg, anti-
HBc IgM, and low titers of anti-HDV IgM. In superinfec-
tion, HBsAg and high titers of anti-HDV IgM are present,
but Anti-HBc IgM will nor be presenr.
Treolment of Virol Hepotitis
l. All children wirh acute viral hepatitis do not require
hospitalization. In majoriry it is a self-limiting disease
with complere recovery; full restoration of liver function
and clearance of virus occur. Certain warning signs and
symptoms necessirate more monitoring: (z) persistent fever,
(li) persistent anorexia, (iii) deepenine jaundice, (iu) bleed-
ing tendency, (z) altered sensorium, (eri) gastrointestinal
bleed, (uii) fuid retention-pedal edema/ascite s, (uiii)
decreasing liver size, (zr) prolonged prothrombin time,
and (x) increasing serum bilirubin, AlI, and BUN.
2. Supportive measures: The general principles of
management are rest, avoiding vigorous physical exercise
and hepatoroxic drugs, and ensuring regular bowel habits.
The dietin a child with uncomplicated acute hepatitis
should be near normal. Fluid and salt restriction is
Fig. 8.2: Serological response to hepatitis B virus infection.
re
necessary only when there is pedal edema or ascites.
Proteins are withheld in children with impending or full-
blown hepatic encephalopathy. IV fluid may be given in
frequent vomiting.
3. Antivirals:
r HAV-Altiviral agents have no role because the hepatic
injury appears to be immunopathologically mediated.
r HBV-There are rwo approved therapies for chronic hepa-
titis in children:
o Interferon-Interferons (IFN) are a group of naturally
occurring agents wirh antiviral, antineoplastic, and
immunomodulatory properties. IFN-cr2b has long-term
eradication rates of 25o/o. Therapy with interferon-cr
(5-10 mU/m'z BSA SC 3 times/week) for 4-5 monrhs
will induce seroconversio n in 25-40o/o of children.
o Lamivudine-Lamivudine monotherapy for I year
provides sarisfactory results. The development of
resisrant viral mutants (\a4DD) limits the long-term use
l
of monotherapy.
Combinations of INF with lamivudine have comparable
effects and slightly better results than monotherapy in
children affected by chronic hepatitis B. Other drugs in-
cluding peginterferon, adefovir, entecavir, and dipivoxil
have documented clinical activiry against wild and lami-
vudine-resistant HBV, but needs to be further eva.luated
in children.
o HCV-The goal of rreatmenr is to achieve a sustained
viral response (S\rR.), as defined by the absence of viremia
6 months after stopping the medication. Therapy includes
combinarion therapy with interferon-c (3 mU/m2 BSA SC
3 times weekly) and ribavirin (15 mg/kg divided, rwice
a day) for 6-12 months. Long-term ciearance of virus is
seen in 40-500/o of treated children, with SVR in 80-90%
of children with genotypes 2 and 3. The use of pegylated
interferon in children is under studv.
Prevention
o General: Improving personal, food, and environmental
hygiene. Avoiding unnecessary needle pricks; using dispos-
able syringes and screened blood for transfusion.
o Specific Immunization against hepatitis A & B has been
described under EPI.
Prognosis
HAV infection:
o Majority of children show clinical and biochemical resolu-
tion within 60 days.
o Relapsing/polyphasic hepatitis (6-120/o): Acute hepatitis
followed by remission in 4_15 week then again relapses
of hepatitis. t
o Cholestatic hepatitis/Prolonged cholestasis may persist for t
I
>12 weeks.
t
{
1
 DISEASES OF GASTROINTESTINAL SYSTEM

Cirrhosis
v
Hepatocellular
carcinoma

Fig. 8.3: Range of possible clinical effects of HBV iniection

o Immune complex disorders (cutaneous vasculitis, arthritis, o SLE

etc.) may be seen following HAV infection. o Celiac disease
o Fulminant hepatic failure (<0.1%). o Drug-inducedhepatitis: Isoniazid, methyldopa, pemoline,
o Post hepatitis syndrome may occur in anxious patient and
nitrofurantoin, sulfonamides.
lasts for 2-3 months (prolonged malaise, abnormai enzymes,
e Metabolic disorders associated with chronic liver disease:
persistent anti-HAV IgM).
\Tilson disease, c{.r-antitrypsin deficienqr, Niemann-Pick
HBV infection: disease, type IV cystic fibrosis, galactosemia.

o fusk of chroniciry depends on the age of acquisition of

o Bile and biosynthesis abnormalities
infection and immune status of the host. The chance of
o (Jnknown cause

chroniciry is about 90% when infected at birth, 25-50o/o Histological features help characterize chronic hepatitis. Subdi-
when infected between I and 5 years, and 5-10% when vision of chronic hepatitis into persistent vs. active form on the
infection occurs in older children and in adults. basis of histologic findings is not as useful as once thought. The
r Approximately l0-I5o/o of chronic carriers may spontane- finding of inflammation contained within the limiting plate of
ously become HBsAg-negative. the portal tract (chronic persistent hepatitis) and the absence
o See Fig. 8.3 for range of possible clinical effects of HBV of fibrosis/cirrhosis suggest a more benign course. The finding
infection. of activity on biopsy may be predictive of response to antiviral
therapy ifhepatitis B infection is present and is a criterion used
CHRONIC HEPATITIS in the diagnosis of autoimmune hepatitis. Histologic features
help identify the etiology; characteristic PAS-positive, diastase-
Disorders Producing Chronic Hepotitis resistant granules are seen in cr,-antitrypsin deficiency, while
macrovesicular and microvesicular neutral fat accumulation
o Chronic viral hepatitis
within hepatoqte is a feature of steatohepatitis. Bile duct injury
,r HBV (approx. 75-20o/o of chronic hepatitis)
may suggest an autoimmune cholangiopathy. Ultrastructural
o HCV analysis may suggest distinct rypes of storage disorders.
o HDV
o Autoimmune hepatitis
Feolures Suggesting Chronicity
r Anti-actin antibody positive
Chronic hepatitis should be suspected in the following situ-
c Anti-liver-kidney microsomal antibody positive
ations:
r Others (includes antibodies to liver specific lipoproteins
or asialoglycoprotein) o Relapse of an apparent acute hepatitis.
r Overlap syndrome with sclerosing cholangitis and o Clinical or biochemical features of hepatitis persisting
autoanribodies beyond 8 weeks.

il

ESSENCE OF PEDIATRICS
HBsAg positive
1. Look for markers of active
viral replication (HBeAg,
HBV, DNA)
2. Look for co-infection (anti-
HDV lsM)
AIH
Fig. B.4: Algorithmic approach to the etiological diagnosis.
o Hepatitis occurring after a history of neonatal cholestasis.
o Signs such as a small or hard liver, enlarged left or collapsed
right lobe of liver, or other clinical features of liver disease
(jaundice, pruritus, leukonychia, clubbing, fat soluble vit
deficiency, xanthomas, splenomegaly, cutaneous shunts,
other cutaneous stigmata, hypersplenism, esophageal varices,
ascites, encephalopathy, dependent edema, malnutrition).
lnvesligolions
r Liver function tests: Serum bilirubin (direct/indirect); total
proteins and A/G ratio; ALI, AST, alkaline phosphatase,
and prothrombin time to assess liver damage.
o Illtrasonography of abdomen and upper gastrointestinal
endoscopy to rule out cirrhosis and portal hypertension.
r Liver biopry is needed for confirmation. At times, it is very
difficult to differentiate cirrhosis from chronic hepatitis. The
liver biopsy is confirmatory.
An approach to the etiological diagnosis is depicted in Fig. 8.4.
Treolmenl
Treatment of Associated Complications
o Bleeding: Endoscopic sclerotherapy (EST) is the preferred
mode to control bleeding from esophageal varices. Bleed-
ing due to coaguiopathy requires fresh blood transfusion
and vitamin K.
o Ascites: Peritoneal fuid should be analyzed to rule out
infection. Spontaneous bacterial peritonitis (SBP) should
be treated with appropriate antimicrobiai agents. Intake of
salt should be restricted, and diuretics help in controlling
the ascites.
o Encephalopathy: Intake of proteins should be restricted.
Animal proteins should be avoided. Tieatment is written
later.
ru
I
Autoantibodies (-ve)
1. Anti-HCV and HCV
2. Rule out Wilson disease
3. o,-antitrypsin deficiency
4. Drug induced
Specific Treatment
There is no specific treatment of chronic hepatitis, but certain
potentially treatable conditions are as follows:
r Autoimmune CAH: Steroids are very helpful but in certain
situations, azathioprine can be used. Prednisolone in dose
of 2 mglkgld is given till clinical and biochemical improve-
ment occurs. This is followed by maintenance dose till the
histological reversal is seen.
e Wilson fisease: Avoid copper containing food items like
chocolate, nuts, mushrooms, shellfish, liver, etc. D-penicil-
lamine (10-12 mg/kg/d), a copp€r chelator, is very efFective
and zinc can be given to prevent copper absorption from
gastrointestinal tract.
o Hepatitis B and C: Discussed earlier. Strict criteria should
be followed for CHB, the criteria are (l) disease >6 months,
(z) raised liver enzymes, (liz) positive HBe, and (la) HBV
DNA and liver histology showing core antigen positiviry.
Relapse rate is very common, especially in HCV and treat-
ment is also very costly.
o Congenital syphilis: This is treated with penicillin.
o Galactosemia: lVithdrawal of milk (galactose) from diet
can stop the ongoing liver damage.
Prognosis
Severe CAH invariably progresses to cirrhosis. Certain situa-
tions like Wilson disease and AIH can become normal with
specific therapy.
The currently accepted definition of fulminant hepatic failure
(FHF) in children includes (z) biochemical evidence of acute
liver injury (usually <8 week duration), (ii) no evidence of
 DISEASES OF GASTROINTESTINAL SYSTEM

chronic liver disease, and (iii) hepatic-based coagulopathy LABORATORY FINDINGS

defined as a PT >15 second or international normalized ratio
(iNR) >1.5 not corrected by vitamin K in the presence of
clinical hepatic encephalopathy, or a PT >20 second or INR
>2 regardless ofthe presence ofclinical hepatic encephalopathy.
o S. bilirubin-increased
o S. aminotransferase-markedly elevated. It does not cor-
relate well with the severiry of the illness and may actually
decrease as a patienr deteriorates.

ETIOTOGY Blood ammonia level-increased

r Prothrombin time-often prolonged and does not improve
Viral hepatitis (isolated/mixed): Hepatitis A, B, C, D, E, after administration of parenteral vit K.
E-B virus, adenovirus, enterovirus, CMV varicella zoster o Hypoglycemia-particularly in infant
a Auroimmune hepatitis o Hypokalemia
a Idiopathic FHF: 40-50% o Hyponatremia
a Drugs and chemicals: Carbon tetrachloride, acetaminophen o Metabolic acidosis
overdose, sodium valproate o Respiratory alkalosis
Ischemia and hypoxia: Hepatic vascular occlusion, congenitai
cyanotic heart disease, circulatory shock TREATMENT
o Metabolic disorders: \filson disease, galactosemia
a Supportive treatment
CLINICAL FEATURES a Prophylactic antibiotics, Hr-biocker
a If hypovolemia-cautious infusion of fluids and blood
FHF can be the presenting feature of liver or it can disease, products
complicate previousiy known liver disease. A child with FHF If renal dysfunction (due to dehydration, acute tubular
is usually previously healthy and most often has no risk factors necrosis, hepatorenal syndrome)-Electrolytes and glucose
for liver disease (such as toxin or blood product exposure). The solution should be given
clinical features are (l) progressive jaundice, (ii) fetor hepaticus, a Hyponatremia should be corrected
(iii) fever, (iu) anorexia, (z) vomiting, (zz) abdomind' pain, (uii) a Parenteral supplementation with calcium, phosphorus, mag-
hemorrhagic diathesis, (uiii) xcites, (ix) rapid decrease in liver nesium
size (is an ominous sign), and (x) F/O hepatic encephalopathy. For coagulopathy-parenteral administration of vit K, infu-
Hepatic encephalopathy (HE) is a state of disordered CNS sion of FFP and platelets, plasmapheresis, recombinant factor
function associated with severe acute or chronic liver disease. VIIa for transient correcrion
It may be acute and reversible or chronic and progressive. a Tieatment of infections
F,EG characteristics include delta waves-bilateral symmetrical a Treatment of HE
spikes followed by flattening of waves. See Table 8.3 for stages
: Avoidance of precipitating factors,
of hepatic encephalopathy. o Protein intake should be initially restricted or eliminated,
Precipitating factors for HE: Gastrointestinal bleeding
depending on d1e degree of HE
(100 ml = 14-20 g protein), constipation, high animal-protein
o Lactulose 10-50 ml every 2-4 hourly sufficient to cause
meal, rapid abdominal paracentesis, hypoglycemia, sepsis, diarrhea. The dose is then adjusted to produce several
CNS depressant drugs (e.g., benzodiazepines, phenobarbitone),
acidic, loose bowel movements daily. Lactulase syrup
hypoxia, and hypokalemia are common precipitating factors.
diluted with 1-3 volumes of water can also be given as a
retention enema every 6 hourly.
Table 8.3: Stages of Hepatic Fncephalopathy ,r Neomycin, a non-absorbable antibiotic, oral or rectal
administration may reduce enteric bacteria responsible
For ammonia producrion.
Symptoms Periods of Drowsiness, Stupor but Coma
lethargy, inappropriate arousable. lVa responcl
,r Flumazenil, a benzodiazepine antagonist, may temporar-
euphoria, behavior, confused, to noxious ily reverse early HE.
reversal of agitation, incoherent stimuli. tVb
day-night wide mood speech no response. Tieatment of cerebral sdsrnx-6s11icosteroid, osmotic
sleeping; swings, diuresis
May be disorientation For fulminant autoimmune hepatitis-immunosuppressive
alert agents
Signs Trouble Asterixis, fetor Asterixrs, Areflexra, r For fuiminant enteroviral hepatitis (in neonate)-
drawing hepaticus, hyper- no asterixis,
pleconaril
figures, incontinence. reflexia, f laccidity.

performing extensor o Endotracheal intubation may be required to prevent aspira-

mental reflexes, tion, to reduce cerebral edema by hyperventilation and to
tasks. rigidity. facilitate pulmonary toilet
ESSENCE OF PEDIATRICS
Mechanical ventilation and supplemental oxygen in advanced
coma
a Temporary liver support
a Orthotopic liver transplantation for children who reach
advanced stages of hepatic coma.
Chronic liver disease (CLD) is not a single entity, but a clini-
cal and pathological syndrome, which has several causes and
is characterized by varying degree of hepatocellular necrosis,
inflammation, and fibrosis. It may be defined as a continuing
inflammatory lesion of the liver with the potential to either
progress to more severe disease, to continue unchanged, or to
subside spontaneously or with treatment.
ETIOTOGY
o Chronic hepatitis can cause chronic liver disease. HBV
contributes about 8-15% of patients with CLD.
o Up to one-fourth of CLD patients may have metabolic
etiologies, of which Wilson disease is rhe commonest.
o Autoimmune liver disease has been reported in about
with CLD.
2-4o/o of children
o In up to 30-65Vo of children with suspected liver disease,
no cause can be identified.
CLINICAT FEATURES
Insidious onset: The patienr may have clinical feamres of pro-
longed/repeated episodes of jaundice, features of portal hyperten-
sion, upper gastrointestinal bleed, abdominal distention, failure
to thrive, shrunken or enlarged liver, presence of splenomegaly,
ascites, and cutaneous portosysremic shunts. Laboratory inves-
tigations may show elevated ffansaminases, and elevated serum
bilirubin, with or without the reversal of A:G ratio.
Acute hepatitis: Occasionally, CLD is diagnosed when a
child presents with acute viral hepatitis like features. Some
features in history and examination may lead to suspicion of
presence of an underlying chronic liver disease. Metabolic and
genetic disorders like \Tilson disease, ar-anritrypsin deficiency,
and autoimmune hepatitis may present as acure viral hepatitis
for the first time.
Asymptomatic presentation: Occasionally, the condition is
discovered in patients with no currenr or past history of jaun-
dice. The only presenting feature might be hepatosplenomegaly
with or without failure to thrive. Elevation of transaminases is
detected incidentally. Almost 40-50o/o of children with CLD
may have such presentations, particularly those with MLD.
Patients with any of the following features in the history
should be suspected of having a CLD: History of conjugated
hyperbilirubinemia in infancy; family history of chronic liver
disease; inherited or autoimmune disorders; and relapse of
apparent acute hepatitis or persistence of clinical features of
acute hepatitis for >3 months. Children who present with hepa-
tosplenomegaly or isolated hepatomegaly with previous history of
hepatitis B, C, or non-A, non-B hepatitis should be evaluated in
detail. Clinical examination suggestive of CLD include shrunken
liver with enlarged left lobe; hard or nodular liver; ascites; edema;
cutaneous portosystemic shunts; gastrointestinal bleeding growth
failure; muscle wasting; cutaneous features (facial telangiecrasia,
palmar eq{hema, clubbing, papular acrodermatitis); extrahepatic
manifestations of autoimmune chronic hepatitis, and presence
of Kayser-Fleischer rings ('Sfilson disease).
DIAGNOSIS
r Evaluation of liver function: S. bilirubin level, S. ALT, S.
AST level, alkaline phosphatase level, S. protein, prothrom-
bin time, blood sugar
a Abdominal ultrasonography
a Endoscopy ofupper GIT
a Liver biopsy: In one-fifth of patients of suspected CLD
(marker negative acute hepatitis and suspected metabolic
liver disease)
Determination of etiolog;r:
c Viral markers-HBsAg, HBeAg, Anti-HCV
o Auto-antibodies-anti-SMA, ANA, anti-LKM-l, p-
ANCA
.J S. ceruloplasmin
a) 24 hr urinary copper-penicillamine challenge test
!) Slit lamp examination for KF ring
.) Liver biopsy
!) Urinary reducing sugar
o Urinary aminoacidogram
o GALT (for galactosemia)
() Fructose tolerance test (for hereditary fructose tolerance
test)
After initial investigations, the following may be needed: (i)
HBV DNA, (ii) F{CY RNA, (ll, MRCB (za) ERCB (2,)
sigmoidoscopy or colonoscopy, (ui) bone marrow aspiration
for abnormal storage materials, and (uii) enzyme activity in
leukocytes or fibroblasts for specific liver disease.
MANAGEMENT
Chronic hepatitis B: The aims of treatment are sustained cessa-
tion of viral replication and remission of liver disease. Currently,
only rwo drugs are approved for use in children beyond 2years
of age-interferon and lamivudine. The overall response rare
of a combination therapy of interferon and iamivudine varies
between 40Vo and 600/o. Favorable predictors of response at the
time of initiating therapy are higher age, elevated AII levels,
and high HBV DNA levels.
Chronic hepatitis C: Combination therapy with INF-cr and a
ribavirin is approved for treatmenr of children aged 3-17 years
i
who have chronic HCV infection. t
I
,
I
Autoimmune hepatitis: The goal of therapy is the control of
disease. Currently, immunosuppression is achieved by corti-
costeroids and azathioprine, either singly or in combination.
Prednisolone ar \-2 mg/kg/d (maximum 60 mg per day)
and azathioprine at I.5-2 mglkg/d are administered orally.
Remission is achieved with prednisolone given everyday for
8-12 weeks followed by gradual tapering over 6-8 weeks till
a maintenance dose of 0.1-0.2 mglkgld or 5 mg per day.
Wilson disease: Copper chelating agents are the first-line
therapy. Penicillamine is the drug of choice. The usual dose is
10 mg/kg/d (maximum 1 g/d) given in 3-4 divided doses
I hour before meals; the dose is reduced after a few months'
Pyridoxine should be supplemented at a dose of 50 mg/wk.
Tiientine is also a copper chelator, acting Primarily by enhancing
urinary copper excretion. The usual dose is 25 mglkgl d in 2-3
divided doses t hour before meals' Ammonium tetrathiomolybdate
acts by preventing the absorption of copper from GI tract and
preventing its availabiliry for cellular uptake. Zinc prevents
intestinal absorption ofcopper and excretion in the feces. The
usual dose is 25-50 mg of elemental zinc daily, taken t hour
before meals. Patients should avoid copper-rich food such
as chocolate, nuts, shellfish and liver, and abstain from
cooking or taking food from copper bowls and plates' In
patients with fulminant \Tilson disease and in those with
decompensated cirrhosis, liver transplantation is the treatment
of choice.
Portal hypertension is defined as an elevation of portal pres-
sure >10-12 mmHg. It is a major cause of morbidiry and
mortality in children with liver disease. The normal portal
venous pressure is =7 mmHg. Th. clinical features of the
various forms of portal hypertension may be similar, but the
associated complications, management, and prognosis can vary
significantly and depend on whether the process is complicated
by hepatic insufficiency.
CAUSES
Extrahepatic: Portal vein agenesis, atresia, stenosis; portal
vein thrombosis or cavernous transformation; splenic vein
thrombosis; increased portal flow; arteriovenous fistula
Intrahepatic:
o Hepatocellular disease: Acute and chronic viral hepati-
tis; cirrhosis; congenital hepatic fibrosis;'Wilson disease;
ar-antitrypsin deficiency; glycogen storage disease rype
IV; hepatotoxiciry; methotrexate; parenteral nutrition
o Biliary tract disease: Extrahepatic biliary atresia; cystic
fibrosis; choledochal cyst; sclerosing cholangitis; intrahe-
patic bile duct paucity
: Idiopathic portal hypertension
r Postsinusoidal obstruction: Budd-Chiari syndrome;
veno-occlusive disease
DISEASES OF GASTROINTESTINAL SYSTEM
CTINICAI FEATURES
Variceal upper GI bleeding: Often life-threatening, often
massive and recurrent. A febrile or uPper respiratory illness
or drug intake may predispose to upPer GI bleeding.
a Splenomegaly invariably presents.
a Prominent veins on anterior abdominal wall and caput
medusae.
Ascites: Often accompany portal hypertension and suggest
cirrhosis or post sinusoidal hypertension'
a Anemia: Due to recurrent GI hemorrhage or hypersplenism.
a Shrunken, nodular liver along with stigmata of chronic liver
disease suggests cirrhosis
INVESTIGATIONS
r Complete blood count for degree of anemia and evidence
of hypersplenism.
o lJltrasonography for SPV-axis (size, patency of splenic vein;
and size, patency, collaterals ofportal vein); echotexture of
liver, free fluid in abdomen. Splenoportography.
o The use of Doppler flow ultrasonography can demonstrate
the direction of flow within the portal system. The pattern
of flow correlates with the severity of cirrhosis and encepha-
lopathy. Reversal of portal vein blood flow (hepatofugal
flow) is more likely to be associated with variceal bleeding.
Ultrasonography is also efFective in detecting the presence
of esophageal varices.
o Liver function tests; prothrombin time; specific tests and
liver biopsy to identifi' the exact etiology'
Endoscopy of upper GI tract: it is the most reliable method
for detecting esophageal varices and for identifying the source
of gastrointestinal bleeding. There is a strong correlation
between variceal size as assessed endoscopically and the
probability of hemorrhage. Red spots apParent over varices
at the time of endoscopy are a strong predictor of imminent
hemorrhage. This also gives opportunity to do therapeutic
procedures (ligation or sclerotherapy) to stop bleeding.
Barium swallow: It is helpful in demonstrating varices, but
not done frequently.
Grading of esophageal varices:
Grade 1: Visible only during inspiration
Grade 2: Visible during both inspiration and expiration
Grade 3: Projecting into lumen <5070
Grade 4: Projecting into lumen >50olo
TREATMENT
The therapy of portal hypertension can be divided into
emergency treatment of potentially life-threatening hemorrhage
and prophylaxis directed at prevention of initial or subsequent
bleeding.
 ESSENCE OF PEDIATRICS
Treolmenl of Potients wifh
Voriceol Hemorrhoge
1. General measure:
a. Fluid resuscitation, initially in the form ofcrystalloid
infusion, followed by the replacement of red blood
cells.
b. Correction of coagulopathy by administration of
vitamin K or the infusion of platelets or fresh frozen
plasma, or both therapies.
c. A nasogastric tube should be placed to document the
presence of blood within the stomach and to monitor
for ongoing bleeding.
d. An Hr-receptor blocker such as ranitidine should be
given intravenously to reduce the risk of bleeding
from gastric erosions.
2. Pharmacological therapy may be considered in parients
with continued bleeding:
a. Vasopressin: Initially
with a bolus of 0.33 U/kg over
20 minutes, followed by a continued infusion of the
same dose on an hourly basis or a continuous infusion
of 0.2U11.73 m2lmin is thought ro act by increasing
splanchnic vascular tone and thus decreasi.rg po.r"-i
blood flow but side effects such as coronary vaso-
consrricrion, bowel ischemia may be life_threatening.
Nitroglycerin should be administered with,r".opr.r.in
to counteract side effects.
b. The somatostatin analogue octreotide d.ecreases
splanchnic blood flow with fewer side effects and. may
be administered by conrinuous intravenous infusion
of 1.0-5.0 pg/kg/hr.
3. Endoscopic therapy: The most common and probably
the most effective nonsurgical therapies
.rrdor.opi.
"..irritant solu_
variceal sclerotherapy and ligation. Highly
tions such as erhanolamine, polidocanol or even absolute
ethanol are injected through endoscopic direct vision into
and around the bleeding varix. The subsequent inflam-
mation leads to eventual thrombosis and fibrosis of the
varix lumen. Endoscopic ligation or banding of the varices
using rubber bands is a relatively safer method to occlude
the varices followed by fibrosis and is more effective and
associated with fewer complications than is sclerotherapy.
4. Mechanical therapy: In patients who continue to bleed
despite pharmacologic and endoscopic methods to control
hemorrhage, a Sengstaken-Blakemore tube can be placed
to stop hemorrhage by mechanically compressing..oph"_
geal and gastric varices.
5. Surgical methods: \,X4ren all the above measures fail, emer_
gency surgery is indicated. The surgical procedures include
devascularization or transaction of the esophagus, which
blocla the blood flow ro rhe varices. Another
rype of surgery
is the transjugular intrahepatic portosystemi. .h,rrrt (TIPS).
In this procedure, a remporary shunt is created berween the
branches of hepatic and ponal veins via a catheter under
fluoroscopy to decompress the portal pressure.
long-Term Monogemenl
o Propranolol: Decreases porral pressure by decreasing cardiac
outpur and producing splanchnic vasoconsrricrion. Dose:
I mg/kg/d, titrated ro reduce heart rare by 25o/o or blood
pressure by 15 mmHg.
o Nitrates: Isosorbide (decreased portal pressure)
o EST (endoscopic sclerotherapy)
o EVL (endoscopic venoligation)
r Surgery: Shunts recommended presently are (l) selective
end-to-side or side-to-side distal spleno-renal (Varren)
shunt sparing the spleen or with ,p1..,..to-y, (r) TIPS
(transjugular intrahepatic portosystemi. shunr) is
effective
in portal hypertension uf hepatic origin and is done when
bleeding cannor be controlled by endosc opy, and (ili)
liver rransplarrtation-if the cause of portal hypert.nsion
is cirrhosis and hepatic failure, liver transplantaiion is the
definite management.
Selective surgery is done in case of (l) recurrent bieeds
after 4-6 ESI (r) hypersplenism, and, (iii) extrahepatic
portal hypertension.
Associoled Problems
Hypersplenism: Massive splenomegaly may cause excessive
destruction of the pooled blood cell componenrs leading
to_ thrombocl.topenia, leukopenia, and anemia. It
splenectomy.
-"y ...J
PROGNOSIS
Portal hypertension secondary to intrahepatic disease has a
poor prognosis. Portal hypertension is usually progressive in
these patients and is often associated with deteiioiting liver
function.
In.patients with portal vein obstruction, episodes of bleeding
may become less frequent and severe with age, as a collateral
circulation develops. Most patients can be treai.d conservatively
with endoscopic sclerotherapy when necessary. Children may
continue to experience significant bleeding during adolescence,
howeveq and may eventually require po.roryr,."-ic shunting
procedure. "
Indian childhood cirrhosis (ICC) is a progressive fatal liver
disorder of young Indian children fi.rt Ji..J,o..ed by BC Sen
in Calcutta in 1887. Many theories of its causation, from virus
to aflatoxin and genetics to autoimmuniry were suggested.
But in 1978, a striking association of greatly increasedtpatic
copper and ICC was discovered and hypothesis ofcopper con-
taminated milk feeds as a cause of ICC were pur foiward. In
an epidemiological study, it was discovered that boiled animal
milk stored in brass utensils, which is a common pracrice in
India, has a higher copper concenrrarion.
\
I
 DISEASES OF GASTROINTESTINAL SYSTEM

The characteristic epidemiological and clinical features of duction of copper chelation therapy has recently changed
ICC are: the natural course of ICC, and also the change in storage
of boiled milk has Ied to a reduction in number of cases
o Age: 6 months to 5 years with a mean age of 18 months. in India.
o Sex: Boys outnumber gids by 3:I.
o Genetics: High rates of parental consanguinity and up to
20o/o affecrion of siblings'
r Geography: Restricted to Indian subcontinent and more
among rural than urban children.
r Religion: More among Hindu children. Ascites refers to accumulation of free fluid in peritoneal
cavity.

CLINICAL FEATURES
ETIOTOGY
Divided into three stages:

Eady stage Anorexia, irritabiliry low-grade fever, abdominal

A. With portal hypertension:
distention due to an enlarged, smooth liver with a sharp,
I . Extrahepatic-splenic/portal vein thrombosis, inferior
vena caval obstruction, Budd-Chiari syndrome.
firm edge.
Intermediate stage Jaundice and signs of portal hyperten-
2. Hepatic
sion such as splenomegaly and ascites, associated with an
a. Biliary-Extrahepatic biliary atresia, choledochal
cyst, cholangitis
increased susceptibiliry to infection. Progression to cirrhosis
(from early to intermediate stage) takes 1*8 months. b. Hepatocellular-Hepatitis B, C, cirrhosis,
autoimmune hepatitis, \Tilson disease.
Late stage: Decompensated cirrhosis with jaundice, a shrink-
ing liver, GI bleeding, repeated infections, edema, hepatic
B. Without portal hnrertension: Tirberculosis; nephrotic
syndrome; bacterial peritonitis; malignancy-peritoneal,
encephalopathy, and finally death.
hepatiq pancreatitis; JRA; SLE; heart failure, chlamydial
infection.
LABORATORY FINDINGS
Serum ALT/AST, bilirubin, albumin, and PT follow the
INVESTIGATIONS
expected changes for the various stages of ICC. Reducing
,trtrr".r... in urine and generalized aminoaciduria may be Complete blood counts, complete urine examination, liver
present, indicating renal tubular dysfunction. Other charac- function tests, Mantoux test, renal and cardiac evaluation,
ieristic findings include raised serum immunoglobulin level, blood for viral markers.
positive smooth muscle antibodies (45o/o cases), and elevated o Abdominal sonography-sensitive detector
i..,r.r, o-f.toprotein level. Serum ceruloplasmin copper levels a Plain abdominal radiography
are normal to raised, distinguishing this from \Tilson disease' a Upper gastrointestinal endoscopy-to confi rm esophageal/
Characteristic histological features are (z) hepatocellular fundal varices
necrosis with prominent Mallory hyaline body, (ii) marked r CT and MRI-for determining edology in certain cases
pericellular fibrosis throughout the hepatic lobule (micronodu- o Abdominal paracentesis
lar cirrhosis), (lil) widespread coarse dark brown staining
granules representing copper in hepatocytes, and (iu) lack of
fucitic fluid analysis:
regenerative nodules. a) Cell count: Cell count >500 and PMN count >2501mm3
suggest infection; may reveal malignant cells'

TREATMENT
b)
In early stage, D-penicillamine can be used along with other
supportive therapy to chelate hepatic copPer' and it shows a
remission in up to 607o cases of early ICC. In case of decom-
pensated cirrhosis or D-penicillamine unresponsive cases, liver
transplantation should be considered.
PROGNOSIS
In untreated decompensated stage, mortaliry is 45o/o within 4
" of liver
weeks of presentation and860/o within 6 months. The intro-
Serum Ascites Albumin Gradimt (SAAG) (difference
benveen serum and ascitic fluid albumin): High albumin
gradient and low albumin gradient should replace the
terms "transudate" and "exudate", respectively, in the
classification of ascites, as accuracy is not good in the
latter system. The SAAG is based on oncotic hydrostatic
balance. If SAAG is >1.1 g/dl, patient has portal hyper-
tension with approximately 97o/o accuracy. If SAAG is
<1.1 g/dl, patient does not have portal hypertension'
The test is accurate despite ascitic fluid infection, diuresis,
therapeutic paracentesis, albumin infusion, and etiology
disease.
 t
ESSENCE OF PEDIATRICS

Classification of ascites by SAAG: Low albumin gradient ascites: These patients usually
do not have portal hypertension and do nor respond to salt
restriction and diuretics. Patients with "tuberculous peritonitis"
Cirrhosis Tuberculous peritonitis
are cured by antituberculous therapy. Pancreatic ascites may
Hepatitis \ephrotic syndrome
resolve spontaneously, require endoscopic stenting, or need
Ful minant hepatic fa ilure Pancreatic ascites
somatostatin therapy. Lymph leak usually resolves spontane-
Cardiac ascites Bowel obstruction/infa rc I ion ously or may require surgical intervention or perironeovenous
Budd-Chiari syndrome Biliary ascites shunting: Chlamydial peritonitis requires tetracycline therapy.
Myxedema Serositis in connective tissue Nephrotic and lupus ascites may require steroids. Malignancy
diseases requires surgical debulking and chemotherapy. Dialysis ascites
Massive liver metastases may respond to aggressive dialysis.
High albumin gradient ascites: They require the following
c) Cubure: Done like blood culture and gives a sensiriviry management plan:
of 92o/o
d) LDH is helpfui in distinguishing spontaneous bacterial o Bed rest: Upright position increases renin-aldosterone acriv-
iry and rerention of sodium or water. Bed rest reduces this
peritonitis from gut perforarion. LDH >400 IU will be
activity.
present in bacterial perironiris.
e) Amylase: In pancreatitis or gut perforarion, it is markedly
o Diet: Sodium and l1uid restriction. It is the sodium restric-
tion not the fluid restriction that results in weight loss. Fluid
elevated, usually >2000 IU.
restriction is only indicated when there is hyponatremia
f) Other tests: Gram stain, smear and cufture for tubercu-
(serum sodium <120 mEq/L).
losis, and cytology are done.
c Diuretics: Goal of diuretic rherapy is to produce a negarive
'lable 8.4 lists characteristics of ascitic {luid fuid balance of 10 ml/kg/d.
in various disease
states. ,r Potassium-sparing aldosterone antagonist:
Spironolacrone: Dose infant and young children, 1
TREATMENT
- mg/kg/d, daily dose can be increased by 1 mgikgid
up to a maximum 6 mg/kg/d.
The SAAG is helpful diagnostically as well as in therapeutic Amiloride: In dose of 10 mg/d, can substitute for
decision making. - spironolactone.

Table 8.4: Ascitic FIuid Characteristics in Various Disease States

Cirrhosis Straw-colored or <25 (95%) <25A (90"/"\

bi le-sta ined predominantly
mesothelial
Neoplasm Straw-colored, >2s (75%) 2Ao/" >1000 (5096); Cytology, cell block
hemorrhagic, variable cell types peritoneal biopsy
muclnous/ or
chylous
Tuberculous Clear, turbid, >25 (s0%) <1 .1 >1000 (70"/"); Peritoneal biopsy
peritonitis nemorrnagtc/ usually >70% stain and culture
chylous lymphocytes for acidjast bacilli
Pyogen ic Turbid or purulenl lf purulent, >2.5 <1.1 U nusual Predominantly + Cram's stain,
penton rtls polymorphon uc lear culture
leukocytes
Congestive Straur-colored Variable, 15-53 >1.1 r0% <1000 (9046);
heart failure usually mesothelial,
mononuclear
Nephrosis Straw-colored or <2s {100%) <.1 .1 Unusual <250; mesotheliaJ, lf chylous, ether
chylous mononuclear extraction, Sudan
staining
Pancreatic ascites Turbid, hemor- Variable, <1.1 Variable, may be Variable I ncreased
(pancreatitis, rhagic, or often >25 blood-stained amylase in
pseudocyst) chylous ascitic fluid and
SETUM
 I
DISEASES OF GASTROINTESTINAL SYSTEM

Loop diuretics: Colloid replacement: Dextran appears to be as efFective as

Furosemide and bumetanide are indicated in patients albumin. Rate of albumin replacement is 6-8 g/L of ascitic
- who respond partially to spironolactone. Dose starting fuid removed.
ar l-2 mglkg, increased by 1 mg/kg/d, maximum 5 a Peritoneal venous shunts (Leveen shunt)
mg/kg/d. a Orthotopic liver transplantation
Combination: Spironolactone (100 mg) and furo-
- semide (40 mg) or amiloride with furosemide is said
to be very effective.
o Hydrochlorothiazide: Indicated when diuresis is inade-
2-3 mglkgld.
quate on high doses of two diuretics. Dose ParthasarathyA (ed) . IAP Textbook of Pediatrics 4"' ed. New Delhi:
Duration of diuretics therapy: Jaypee Brothers, 2009.

-
to treat- diuretic therapy is continued till ascites is Clauden GS, Hawkins R(ed). Pediatics: Treatment & Prognosis 7"'
present; and ed. New Delhi: Jaypee Brothers, 1989.
to prevent - in certain conditions like cirrhosis, effec- t. International \Torking Group on Persistent Diarrhea. Evaluation of
- tive dose diuretics have to be continued for months an algorithm lor the treatment of persistent diarrhea: a multicentre
study. Bull \YtHO 1995 ;7 4:47 9-89.
to years to prevent reaccumulation of fluid.
4. \Worid Health Organization. Persistent diarrhea- update 2007,
o B-Blocbns (propranolol); Lowers portal pressure and inhib- Geneva.
its renin secretion or combination of these effects, results Ghai OP (ed). Essentlal Pediatrics 7'h ed. New Delhi: CBS Publish-
in increased natriuresis. ers, 2009.
6. Mascarenhas MR. Failure to thrive and malabsorption. In: Atschuler

REFRACTORY ASCITES SM, Liacouras CA (eds.). Clinical Pediatric Gastroenterology 7" ed.
Philadelphia: Churchill Livingstone, 1 998:7 1-80.
It is defined as fuid
overload unresponsiveness to salt restric- 7. Schmitz J. Malabsorption. In: 'Walker 'WA, Durie PR, Hamilton
\Watkins
tion and high-dose diuretic. JR, Valker-Smith JA, JB (eds.). Pediatric Gastrointestinal
Disease: Pathophysiologt, Diagnosis, Management 2"d eds. St. Louis:
Tireatment Mosby-Year Book, Inc, 1996:830-95.
o Therapeutic paracentesis: 8. Hodgson HJF. Malabsorption: Investigation and diagnosis. Medicine
Internatio na I 199 8 /3 :26-39.
o Volume of fluid to be tapped: Up to 100 ml/kg safely at
9. Behrman RE, Kliegman RIvl, Jenson IHB. Nelson Tbxtbook of
any rime. Pediatrics 18'h ed., 'W-B Saunders Co, 2007.
o Large volume tap is indicated in one sitting, rather than 10. Haslett C, et al. (ed). Dauidson's Principles and Practice of Medicine
frequent taps. 20'h ed. London: Churchill Livingstone,2007.
o Mechanism of relief by paracentesis: Thking out fluid 11. Desmet VJ, et al. Classification of chronic hepatitis: diagnosis,
from peritoneal cavity decreases systemic venous conges- grading & staging. Hepatolog/ 1994;19:1513-20.
tion and increases GFR and renal plasma flow, which l2 Sherlock S, et al. Disease of the liuer 6 biliary systems 70'h ed.
helps in producing diuresis. London: Blackwell Scientific Publications, 1997 :303-35.

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 CHAPTER 9
Ca rd iovascu lar Diseases

Chopter Contents
Fetal and neonatal circulation..,....,......., ....,...... .,...,...,.. ...152

Etiology of congenital structural dis0rders........,,...,..,.....153 Electrocardiography .... ........179

Alria|septa1defect....................... . . .,. ...153

Ventricular septal defect.......................................................154
Patent ductus arteri0sus......................,...,.....,........,...,...,...,,.155
Ductus-dependent systemic flow 1esi0ns........................ 156
Hypoplastic left-heart syndr0me................................ 156
Coarctation of the a0rta,....,...,,..,.................................. 1 57

Critical aortic sten0sis,,..................................................157

Ductus-dependent pulmonary flow lesions .................. l5B
Pulmonary atresia with intact
ventricular septum.......,............,.......,.....,........,...,...,...,,158
Tricuspid atresia...., .....................,158

Pulmonary atresia with VSD....................................... 158

Tetralogy of Fa110t....,...,.......,.................................................159
Ebstein anomaly..,. .,..........................,.. l6l

because of high pulmonary vascular resistance and

iow systemic vascular resistance, crosses the ductus
arteriosus to the descending aorta.
Patency of three structures-the foramen ovale, ductus arteriosus
and ductus ysn6sus-ls a critical feature of the cardiovascular
2. Thansition to neonatal circulation
anatomy and physiolory of the ferus. a. At birth, the infant's first breaths cause an increase in
arterial oxygen tension (POr). This lowers pulmonary
vascular resistance, resulting in increased pulmonary
NORMAL PHYSIOLOGY
blood flow. The increased pulmonary venous return
1. Fetal circulation to the left atrium causes the pressure to rise, resuldng
a. Fetal blood is oxygenated in the placenta and then in functional closure of the foramen ovale.
enrers the umbilical vein. b. Systemic vascular resistance is increased by the elimi-
i) One portion of the oxygenated blood perfuses nation of the low-resistance vascular circuit of the
the liver and proceeds to the inferior vena cava placenta at birth.
via the hepatic veins. c. Closure of the ductus arteriosus occurs shortly after
ii)
Another portion enters the ductus venosus, which birth, first functionally and then anatomically.
empties directly into the inferior vena cava. d. The neonatal circulation, with the ventricles working
b. Together with venous return from the lower part of in series, is thus established.
the body, this blood fows into the right arrium.
-). Normal changes in pulmonary vascular resistance:
i) Approximately one-third is shunred, via the Pulmonary vascular resistance is inversely related to the
foramen ovale, to the left atrium, ieft ventricle, diameter of the small pulmonary arterioles.
and ascendingaorta. a. In the fetus, high pulmonary vascular resistance is
ii) The remainder joins the venous return from the maintained by constriction of the muscular tunica
upper part ofthe body and enters the rightventricle media of these arterioles.
and pulmonaty artery. A small portion (< l0%) b. The arterioles begin to dilate after birth, and the
. of this blood enters the lungs, and the remainder, tunica media atrophies gradually.

Refer Thble 9.1 for cardiovascular manifestations of selected
congenital disorders.
The cause of congenital heart disease is usually unknown
in individual cases; evidence points to a multifactorial
etiology, with the insult probably occurring in the first
B weeks of gestation.
Most congenital heart lesions are sporadic. However, their
incidence is slightly higher in families that include one
member with such an abnormaliry; there are families with
several affected members.
Congenital heart disease has been associated with several
teratogenic factors:
,r Medications that are known cardiovascular teratogens in-
clude thalidomide, folic acid antagonists, dextroamphet-
amine, anticonvulsants, lithium, and esrrogens.
o Excessive maternal alcohol ingestion.
o Maternal infection: Antenatal rubella has proven to be
teratogenic. There is also evidence that maternai cyto-
megalovirus and coxsackievirus infections may cause
congenital cardiovascular abnormalities.
Atrial septal defect (persistent patency of the interatrial septum)
cal occur high in the septum (sinus venosus defect, 10%), in the
mid portion (ostium secundum defect, 50-70o/o), and low in the
septum (ostium primum defect,3.0o/o; also known as panial endo-
cardial cushion defect). In ostium primum atrial septal defect, the
anterior leaflet of the mitral valve is often cieft and incompetent.
PATHOPHYSIOTOGY
High left atrial pressure and greater right ventricular compli-
ance cause left to right shunt at the atrial level, thus increasing
ilow across the tricuspid and pulmonary vaives. As a result, the
right ventricle and the pulmonary artery are usually enlarged.
Right ventricle gets blood from both systemic veins (e.g., supe-
rior and inferior vena cavae) and shunted blood from left atrium.
It has to pump large volume of blood during systole resuiting in
ionger ejection time, e.g., P, is delayed, hence S, has wide splitting.
Right ventricular diastolic volume is constantly increased
throughout all phases of respiration, ejection time is prolonged.
So, S, is fixed in its splitting in all phases of respiration. S, is
characteristically widely split and fixed in ASD.
NATURAT HISTORY
o ASD <3 mm mav have 100o/o spontaneous closure by
1 vear. ASD >8 mm rarely closes spontaneously.
!
CAR DIOVASC U LAR DIS EASES
Table 9.1: Cardiovascular Manifestations of Selected
Congenital Disorders
Marfan syndrome Aortic aneurysm, aortic valve
insufficiency, mitral valve prolapse, and
regurgitation
Clycogen storage disease Hypertrophic ca rdiomyopathy
Down syndrome Endocardial cushion defect
Turner syndrome . Aortic coarclation
Noonan syndrome Pulmonary valve stenosis, aortic valve
stenos is
Trisomy 1B syndror.ne Venlricular seplal defect
Rubella svndrome Palent ductus arleriosus
o Most children are active and asymptomatic, bur some may
rarely develop congestive cardiac failure (CCF) during
infancy. If untreated, pulmonary hypertension and subse-
quent CCF may develop during or after third decade, and
reversal of shunt may occur (rare); it may be progressive
with pregnancy.
r Infective endocarditis does not occur in secundum ASD.
o Mitral stenosis may occur as a result of rheumatic fever in
a case of ASD (Lutembacher syndrome).
CtINICAt FEATURES
Symptoms may include slow weight gain and frequent lower
respiratory infections, but most commonly children with ASD
(i.e., secundum ASD) are asymptomatic.
DIAGNOSIS
The precordium is hyperdynamic, and a right ventricular heave
is present. A systolic ejection murmur 2-316 in the pulmonic
area and a mid-diastolic rumble in the lower right sternal area
reflect the increased fow across the pulmonary and tricuspid
valves. S, is normal, may be accentuated. S, is widely and
constantiy split.
TABORATORY EVATUATION
o Chest x-ray: The heart and main pulmonary arrery segmenr
are enlarged; pulmonary vascularity is increased.
o ECG: Right axis deviation is often seen in secundum
defects. The hallmark of a primum defect is an extreme left
a-xis deviation. fught ventricular hypertrophy is represented
by an rsR' in the right precordial leads with right bundle
branch block (RBBB) with rsR' in V,.
r Echocardiogram: The right ventricle is enlarged, and the
septum often moves in a paradoxical fashion. The defect
usually can be visualized on a nvo-dimensional study. Color
{low mapping demonstrates the direcdon of flow as well as
mitral valve anatomy and comperence.
ESSENCE OF PEDIATRICS
o Cardiac catheterization is usually not necessary for diagno-
sis. If it is performed, the presence and size of a left-to-right
shunt are indicated by an increase in oxygen saturation at
the atrial level.
Abnormally wide splitting of the second heart sound (Sr):
lncreased right Pulmonary valve slenosis
ventricular pressure
lncreased right Atrial septal defect
ventricular volume Anomalous pulmonary venous return
Ventricular septal defect
Right ventricular Right bundle branch block
conduction delay
Premature left ventricular Mitral valve regurgitation, ventricular
emptying septal defect
TREATMENT
Medical management Chest infections and CCF should be
treated, ifpresent. Bacterial endocarditis prophylaxis is not
necessary in secundum defects, but is indicated in primum
defects if mitral valve regurgitation is present.
Surgical closure of both secundum and primum defects
can be accomplished with minimal risk.
o Surgery is advised in all symptomatic and asymptomatic
patients with QP/QS ratio at least 2:1.
o Timing: Usually after first year, before school entry. The
defect is repaired under cardiopulmonary bypass.
c Results ofoperation are excellent. Early surgery decreases
morbidiry (i.e., I arrhythmias) and mortality.
Ventricular septal defects (VSD) may occur in any Portion of
the ventricular septum, majority are membranous type. VSD in
muscular part may be single or multiple (Swiss cheese septum).
VSD (persistent patency of the interventricular septum) is the
most common congenital heart disorder, accounting for 25o/o
of all congenital cardiac lesions. Inflow VSDs, also called endo-
cardial cushion defects, often have associated abnormalities of
the tricuspid and mitral valves and are most commonly seen
in children with Down syndrome.
PATHOPHYSIOTOGY
o In smali defects (usually 0.05 cm2), the size of the shunt is
determined by resistance at the defecu small defects result
in small shunts. If the defects are large, both the size and
direcdon of the shunt are determined by the relative resis-
tances in the pulmonary and systemic circuits.
o As long as pulmonary vascular resistance is lower than
systemic vascular resistance, the shunt is left-to-right, if
pulmonary vascular resistance rises above systemic vascular
resistance, the shunt reverses, but when the ratio of pul-
monary to systemic resistance approaches l:1, the shunt
becomes bi-directional.
o Large defects tend to result in pulmonary hypertension,
while in small defects pulmonary vascular dynamics remain
normal.
o The size of the left atrium and left ventricle is directly
proportional to the size of the left-to-right shunt. Right
ventricular enlargement occurs only when pulmonary vas-
cular resistance increases.
o Pulmonary hypertension may lead to the development
of pulmonary vascular obstructive disease (Eisenmenger
syndrome) and reversal of the shunt.
CTINICAL FEATURES
Symptoms are related to the size of the shunt.
o If the defect is small, no symptoms are present. Many of
the small defects close spontaneously.
o If the defect is large and pulmonary vascular resistance is
not significantly elevated (large left-to-right shunt), growth
failure, congestive heart failure, and repeated lower respira-
tory infections usually occur, most commonly beginning at
1-2 months of age.
o If the defect is large and pulmonary vascular resistance is very
high (i.e., Eisenmenger spdrome), shortness ofbreath, dyspnea
on exertion, and ryanosis may occur. Irreversible pulmonary
vascular obstructive disease is uncommon before2 years of age.
DIAGNOSIS
Physicol Exqminolion
A left-to-right shunt produces turbulence during isovolumic
contraction, and the murmur therefore begins with S,, and
ends in mid-systole in small defects, and extends to S, in
large left-to-right shunts. The murmur is harsh and is best
heard at the mid sternal or lower left sternal border. In large
left-to-right shunts, a mid diastolic rumble is also heard.
As pulmonary vascular resistance increases, and the left-
to-right shunt decreases, the mid diastolic murmur dis-
appears, the systolic murmur becomes shorter, and the
pulmonary component of S, increases in intensiry.
o In the presence of pulmonary vascular obstructive disease,
a right ventricular heave, short systolic ejection murmut
diastolic murmur of pulmonary valve insufficiency, and a
loud S, are heard.
[oborolory Evoluolion
o Chest x-rayt In small defects, the chest x-ray may be normal
or show mild cardiomegaly and a slight increase in pulmo-
nary vasculariry.
\

In large lefi+o-right shunts, cardiomegaly, increased pul-
monary vascularity, and enlargement of the left atrium and
left ventricle are s€en. fu a rule, the size of the heart is di-
rectly proportional to the magnitude of the left-to-right
shunt. As pulmonary vascular resistance rises and the left-
to-right shunt decreases, the heart and the distal pulmonary
arteries become smaller but the proximal pulmonary arteries
enlarge.
ECGI In small defects, the ECG is normal.
In large lefi+o-right shunts,left atrial, left ventricular, or
biventricular hypertrophy is seen. fught venuicular hyper-
trophy predominates when pulmonary vascular resistance is
high. An extreme left axis deviation is characteristic ofVSDs
in the endocardial cushion defect.
Echocardiogram: Chamber size can be determined, and
moderate to large defects can be identified with a two-
dimensional study. Color flow mapping can localize defects.
Continuous-wave Doppler allows estimation of right ven-
tricular and pulmonary artery pressures.
Cardiac catheterization: Measurement of intracardiac and
intravascular oxygen content defines the magnitude and
direction of shunting.
NATURAT HISTORY
a Spontaneous closure occurs in about 50% ofcases by 1 year.
a Congestive heart failure (CHF) develops in large VSD after
8 weeks of age.
In a large VSD, the shunt may reverse as early as 6-72
months of age, but Eisenmenger syndrome does not get
established till the teenage years.
a Infective endocarditis develops rarely.
a In large VSDs, infundibular stenosis may develop, which
decreases the magnitude of L-R shunt (acyanotic tetralogy
of Fallot).
TREATMENT
Medicol
o No exercise restriction in the absence of pulmonary hyper-
tension.
o Maintenance of good dental hygiene, antibiotic prophylaxis
against infective endocarditis.
r Chest infections, CCF should be treated if present.
o Adequate nutrition should be maintained.
Surgicol lndicotions
o Patients at any age with large defects in whom clinical
symptoms and failure to thrive cannot be controlled medi-
cally. Infants between 6 and 12 months of age with large
defects, associated with pulmonary hypertension, even if
symptoms are controlled.
CARDIOVASC U LAR DISEASES
o Large VSDs: If CCF responds to decongestive therapy, then
surgery is delayed. If CCF does not respond, then the VSD
should be closed within the first 6 months of life.
r After I year of age, significant L-R shunt with QP/QS
of >2:1 indicates surgery.
r Older infants with large VSDs and increased pulmonary
resistance should be operated immediately.
Contraindication:
o Surgery is contraindicated in presence of predominant R-L
shunt with pulmonary atrial hypertension.
o SmallVSD with no CCF and QP/QS less than 1.5:1 should
lil
not be operated.
The ductus arteriosus connects the pulmonary artery and the
descending aorta (i.e., 5-10 mm distal to the origin of the left
subclavian artery) in the fetus and normally closes shortly after
birth. Patency of the ductus constitutes approximately l0o/o of
congenital heart defects; patency is especially common in very
low-birth weight babies with pulmonary disease.
PATHOPHYSIOLOGY
The direction of flow through a large PDA depends on the
relative resistances in the pulmonary and systemic circuits. As
long as the former is lower than the latter, a left-to-right shunt
is present, if pulmonary vascular resistance rises above systemic
vascular resistance, a right-toJeft shunt develops'
The size of the shunt depends on the size of the PDA and
the relative resistances in the pulmonary and systemic circuits.
The left atrium and left ventricle enlarge in direct proportion
to the magnitude of the left-to-right shunt. If the PDA is large,
pulmonary vascular obstructive disease (Eisenmenger syndrome)
can develop. The right ventricle enlarges with the development
of an increase in the pulmonary vascular resistance. If the PDA
is small, its size limits the left-to-right shunt, and pulmonary
vascular disease does not develop.
NATURAL HISTORY
o If the shunt is large, recurrent chest infections and CCF
develop.
o Reversal of the shunt take place if a large PDA remains
untreated, and puimonary hypertension develops.
o Bacterial endocarditis may supervene more frequent with
small PDA than large ones.
CLINICAL FEATURES
Symptoms are related to the size of the defect and the direction
of flow. A small PDA causes no symptoms. A large PDA with
a large left-to-right shunt may result in congestive heart failure,
ESSENCE OF PEDIATRICS
slowed growth, and repeated lower respiratory tracr infections.
Even small left-to-right shunts may cause severe compromise
in low birth-weight infants with pulmonary disease. Rever-
sal of fow as a result of high pulmonary vascular resisrance
causes shortness of breath, dyspnea on exertion; and cyanosis
is present in toes but not in fingers (especially in pulmonary
hypertension and R-L shunt).
DIAGNOSIS
Physicol Exominotion
o Pulse volume is related to the pulse pressure, whichin turn
is related to the volume of the left-to-right shunt. If the
fow is small, pulses are normal. In a large shunt, bound-
ing pulses, with wide pulse pressure representing an aortic
diastolic run off, are palpated.
o The murmur is conrinuous loud and harsh 416, associated
with a thrill. It begins after S, peaks with Sr, and trails off
in diastole. If pulmonary vascular resisrance rises, first the
diastolic murmur and subsequently the systolic murmur
becomes softer and shorter, and S, increases in intensity. It
may be localized to the second left intercostal space or radiate
down the left sternal border or to rhe left clavicle.
[oborolory Evoluotion
o Chest x-ray: Heart size, pulmonary vasculariry and left
atrial and left ventricular size are all directly related to the
magnitude of the left-to-right shunt. In a small PDA, x-ray
is normal. If the PDA and left-to-right shunt are large,
cardiomegaly and left heart enlargement are pronounced.
o ECG: The ECG is normal if the PDA is small. Left ventricu-
lar or biventricular hypertrophy is seen if the left-to-right
shunt is large. Right ventricular hypertrophy predominates in
the presence of increased pulmonary vascular resistance.
o Echocardiogram: The PDA sometimes can be visualized on
a two-dimensional study. Doppler ultrasonography shows
diastolic turbulence in the pulmo nary artety and diastolic
runoff in the aorta. Color flow mapping demonstrates the
direction of flow. If the shunt is large, the left atrium and
ventricle are enlarged.
o Cardiac catheterization is not usually necessary for diag-
nosis, but if performed, will show a step,up in pulmonary
arterial oxygen saturation and pulmonaty artery pressure.
TREATMENT
o Medical: Bacterial endocarditis prophylaxis
is necessary as
long as the ductus remains patenr. Indomethacin is often
effective in closing a PDA in the preterm newborn infant,
and is ineffective in term infant.
o Surgrcal: Anatomical exisrence of PDA, regardless of size, is
an indication for surgery; but before that reversal of shunt
has to be ruled out. Surgical procedure is performed any
time between 6 months and 2 year or any time in an older
chlld. Procedure-Ligation and division through posrero,
lateral thoracoromy is safe; death rate is <1%o.
Ductus-dependent lesions are those abnormalities in which
either the systemic or pulmonary blood fow depends on
patency of the ductus arreriosus.
In this group of ductus-dependent systemic fow lesions,
systemic blood flow depends on ductal patency. Symptoms
and clinical findings are remarkably similar, despite anatomic
differences, because they result from interruption of systemic
flow when the ductus begins to narrow.
HYPOPTASTIC tEFT. H EART SYN DROME
This syndrome is a continuum of anomalies characterized by
underdevelopment of the aortic root, aortic valve, left ventricle,
and mitral valve. The aortic valve is usually atreric; mitral valve
atresia is common. The ascending aorta often measures only a
few millimeters, and a coarcration is often present. The right
atrium and right ventricle are dilated.
Pothophysiology
Because there is no antegrade aortic fow, perfusion of the
ascending aorta and its branches (coronary, cerebral) depends
on ductal patency. Systemic and pulmonary venous rerurn mix
at the atrial level (i.e., there is an obligatory left-to-right atrial
shunt). Because pulmonary vascular resisrance is high, flow
is directed through the ductus ro rhe aorra, with retrograde
coronary artery perfusion. Closure of the ductus arreriosus
interrupts flow to the vital organs.
Clinicol Feqlures
Symptoms, usually evident in the first few days of life, of
severe CHF, shock, and progressive acidosis occur as the ductus
arteriosus closes.
Diognosis
Poor systemic perfusion (shock) is characterized by mottling of
the skin, weak or absent peripheral pulses, tachypnea, dyspnea, \
grunting, and agonal respirations. Hepatomegaly, diffi.rse rales,
a gallop rhythm with a loud Sr, and a non-specific systolic
murmur are additional findings.
Loborolory Evoluolion
o Chest x-ray shows cardiomegaly and pulmonary vascular
congesrion or edema.
\
1
o ECG is often normal for age and is not helpfirl in diagnosis. I
a
1
I
f. I
:
CARDIOVASCU LAR DISEAS ES

o Echocardiogram is diagnostic; it shows the configuration [oborolory Evoluotion

and size of the various structures. The left ventricle is either
r Chest x-ray in the symptomatic infant shows cardiomegaly
absent or is tiny, the ascending aorta measures 2-3 mm,
: and pulmonary congestion.
and the mitral valve is atretic. Doppler ultrasonography and
a ECG often shows right ventricular hypertrophy with strain.
color flow mapping define the left-to-right atrial flow and a Echocardiogram is critical in defining the anatomy, localiz-
r right-toJeft ductal fow
ing the coarctation, and demonstrating associated anomalies.
r Doppler ultrasonography allows estimation of a pressure
I Treolmenl
? gradient, and color flow mapping defines patency and
a o Medical is palliative and is directed at preserving systemic direction of fow through the ductus.
perfusion; the ductus is opened and patency maintained C.ardiac catheterization with angiography allows definition of
by an infusion of prostaglandin El (PGE'). In addition, the coarctation, associated anomalies, and size of the aorta.
ventilatory support is provided and acidosis is corrected.
r Surgical: Corrective surgery is not feasible
o The Norwood procedure has allowed some children to sur-
vive infanry. The first state involves anastomosis of the main
Treolmenl
Medical: In the newborn with shock, therapy is directed at
unloading the left ventricle and improving systemic flow by
]il
pulmonary artery to the hypoplastic aorta, ligation of the
infusing PGE,, to dilate the ductus arteriosus. In addition,
distal main pulmonary afiery creation of a shunt between a
inotropic agents may be given to improve left ventricular
systemic and pulmonary anery and creation of an ASD. The
function, diuretics are given to decrease preload, and aci-
second sthte is a modified Fontan operation in which sys-
dosis is treated.
temic venous return is directed to the pulmonary arteries'
Surgical: The obstruction is relieved surgically. Restenosis
o Another proposed alternative is neonatal cardiac trans-
at the surgical site is not uncommon.
plantation.

CRITICAT AORTIC STENOSIS

COARCTATION OF THE AORTA
The constriction of the aorta is almost invariably located at
the ;'unction of the ductus arteriosus with the aortic arch, just
distal to the subclavian artery. The constriction may be discrete
or diffuse and is usually associated with isthmic narrowing.
Coarctation may be associated with PDA (660/o), VSD (30%),
and aortic valve abnormalities (often a bicuspid valve).
Pothophysiology
As the ductus constricts in the neonatal period, obstruction
increases at the coarctation site, thus causing increased left
ventricular afterload. Ifthe coarctation is severe and there has
been insuficient time for the development of collateral vessels
or left ventricular hypertrophy to accommodate this increase
in afterload, left ventricular dysfunction with symptoms of
congestive heart failure present in the neonatal period.
Clinicql Feolures
na
Symptoms are those of low cardiac output and congestive heart
failure (e.g., irritabiliry, lethargy, poor feeding, growth failure).
Diognosis
.l Physical examination reveals signs of low cardiac output and
I poor peripheral perfusion. Findings include ashen color, skin
ia mottling, decreased or absent lower extremiry pulses, gallop
Isolated aortic valve stenosis does not usually cause symptoms
in infancy unless it is critical, in which case signs and symptoms
of congestive heart failure and shock due to poor systemic
perfusion occur.
Polhophysiology
The small opening causes severe left ventricular obstruction
and an increase in ventricular systolic as well as end diastolic
pressures. Decreased myocardial perfusion during diastole may
result in myocardial ischemia and dysfunction. Tissue perfusion
may remain adequate as long as the ductus remains Patent'
but symptoms of low output and tissue ischemia develop when
the ductus begins to close.
Clinicol Feqlures
Symptoms are those of low cardiac output' shock, and conges-
tive heart failure and include lethargy, irritabiliry poor feeding'
and varying degrees of respiratory failure.
Diognosis
Physical examination: Infants have poor skin perfusion char-
acterized by mottling and ashen color. Peripheral pulses are
weak or absent. Auscultation may reveal an ejection click and
a systolic ejection murmut but these may be absent if cardiac

f rhythm, single loud Sr, a non-specific and often low-pitched output is suficiently depressed. The liver is enlarged, and there
>- slstolic murmur, and hepatomegaly. Differential cyanosis in the are signs of pulmonary venous congestion (e.g.' tachypnea,
I presence of a patent ductus is usually difficult to recognize. dyspnea, rales).
t
t
I

ESSENCE OF PEDIATRICS
Laboratory evaluation:
o blood fow depends on patency ofthe ductus and in tricuspid
Chest x-ray findings include cardiomegaly and pulmonary
congestion.
o ventricular outflow obstruction.
ECG may show right ventricular hypertrophy, left ventricular
hypertrophy, or evidence of myocardial ischemia.
r Echocardiogram is diagnostic. The site of the lesion, the
degree of obstruction, and the size and function of the left
ventricle all can be determined.
o At cardiac catheterization, the pressure gradient can be
measured, but the low cardiac output may underestimate
the degree of obstruction.
Treolmenl
r MedicaL Hemodynamic stabilization is achieved by reopening
the ductus aneriosus with PGE, infirsion, using inotropic agents
and diuretics, and correcting acid-base imbalance. Balloon
angioplasty of the aortic valve can be lifesaving, but it may be
difficult to pass the dilating catheter across the narrow orifice.
o Surgical: Aortic
valvotomy under direct vision may be
achieved but is associated with a high mortality rate.
In this group of lesions, pulmonary blood flow depends on
ductal patency. Symptoms and clinical findings are remarkably
similar, despite anatomic differences, because they result from
diminution of pulmonary blood flow as the ductus closes. These
constitute the hypoplastic right heart syndromes (pulmonary
atresia with intact ventricular septum, tricuspid atresia) and
pulmonary atresia with VSD.
PUTMONARY ATRESIA WITH INTACT
VENTRICUTAR SEPTUM
In 80o/oof cases, the pulmonary valve alone is involved and
forms an imperforate membrane; in 20o/o of cases there is
associated infundibular hypoplasia or atresia.
fught ventricuiar development is variable, ranging from
normal (10%) to extremely hypoplastic (50o/o).
A patent foramen ovale or true VSD is present. teatment
is written below under tricuspid atresia.
TRICUSPID ATRESIA
Characterized by atresia of the tricuspid orifice (which is often
identifiable only as a dimple), accompanied by a patent foramen
ovale or true ASD.
Polhophysiology
In both pulmonary with intact ventricular septum and
atresia
tricuspid atresia, a right-to-left atrial shunt is obligatory, with
re
L
'l
mixing of systemic and pulmonary venous return. Pulmonary
atresia, on the size of the VSD (if present) and associated
Clinicql Feolures
Severe cyanosis not relieved by inhaled oxygen is present in
the neonatal period and is inversely related to the pulmonary
blood flow.
Diognosis
Physical examination: A variable degree of desaturation is
present.
o In pulmonary atresia with intact ventricular septum, a
single S, is present, and a continuous murmur of a PDA
or a regurgitant murmur of tricuspid insufficiency, or both,
may be heard.
o Findings in tricuspid atresia are more variable and depend
on the size of the VSD and on pulmonary blood fow. If the
VSD is large and pulmonary blood fow is increased, signs
of congestive heart failure and a harsh VSD murmur prevail.
Laboratory evaluation:
o Chest x-ray shows diminished pulmonary vascular markings;
heart size is variable.
o ECG in both pulmonary and tricuspid atresia shows right
atrial enlargement and decreased forces over the right ven-
tricle. Left axis deviation is seen in tricuspid atresia.
o Echocardiogram is diagnostic and demonstrates the anatomic
defect. Color fow mapping demonstrates the direction of
blood flow and presence of a PDA.
o Cardiac catheterization and angiography confirm the anatomy
and allow quantification of pulmonary blood flow.
Treqlmenl
o Medical: PGE, infusion maintains parency of the ductus,
thereby preserving pulmonary blood flow, and should be
urgently instituted. At cardiac catheterization, a balloon
atrial septostomy is performed if the atrial communication
is inadequate.
o Surgical: initially, the aim is to maintain adequate pulmo-
nary blood flow via systemic artery-to-pulmonary artery
shunt. The most commonly performed shunt is the modi-
fied Bialock-Taussig shunt, which involves the interposition
of a graft between the subclavian and pulmonary arteries.
In addition, in pulmonary atresia with intact ventricular
septum, a pulmonary valvotomy is performed.
PULMONARY ATRESIA WITH VSD
This defect is the extreme expression of tetralogy of Fallot with
atresia of the right ventricular outflow and a large ventricular
septal defect. The pulmonary arteries are often hypoplastic.
a
Pothophysiology
Pulmonary blood flow depends on a PDA or on collateral flow
to the pulmonary arteries. A ventricular right-toJeft shunt is
obligatory.
Clinicol Feotures
Severe ryanosis not relieved by inhaled oxygen is present in
the neonatal period and is inversely related to pulmonary
blood flow.
Diognosis
Physical examination: A right ventricular heave may be
present. S, is single, and a continuous murmur of the ductus
or collateral vessels may be heard.
Laboratory evaluation:
o Chest x-ray reveals diminished pulmonary vascular markings,
aconcavity in the area of the pulmo nary aftery, and normal
heart size. A right aortic arch is often present.
o ECG show right axis deviation and normal neonatal right
ventricular forces.
o Echocardiogram is diagnostic and demonstrates the ana-
tomic defects. Color fiow mapping shows no antegrade
fow across the pulmonary valve, the right-toJeft ventricular
shunt, and the left-to-right ductal flow. The right ventricular
pressure can be estimated using continuous-wave Doppler
evaluation.
o At cardiac catheterization, right and left ventricular pressures
are equal. Angiography demonstrates the anatomy including
the source and adequacy of pulmonary blood flow and size
of the pulmonary arteries.
Treolment
Mefical: PGE, infusion maintains patency of the ductus
and preserves pulmonary blood flow. It should be started
as soon as desaturation is detected.
Surgical: The initial aim is to ensure adequate pulmonary
blood fow by creating a systemic artery-to-pulmonary
artery shunt. At a somewhat older age, complete correction
is performed by closing the VSD and interposing a graft
between the right ventricle and the pulmonary artery.
Gtralogy of Fallot (TOF) is the most common cyanotic con-
genital heart defect, representing about 10% ofall congenital
heart defects. It is believed to be due to abnormalities in the
septation of the truncus arteriosus into the aorta and pulmonary
arteries that occur early in gestation (3-4 weeks).
a. TOF includes:
i) Obstruction to right ventricular outflow (pulmonary
stenosis)
ii) vsD
CAR DIOVASCU LAR DISEAS ES
iii) Dextroposition of the aorta with over-ride of the
ventricular septum
iv) fught ventricular hypertrophy
b. A right aortic arch is seen in about 20o/o of cases and
varying degree of hypoplasia of the pulmonary arteries
is often present. An associated PDA may be present, and
defects in the atrial septum are occasionally seen.
PATHOPHYSIOTOGY
Of the four components of tetralogy of Fallot, only the VSD
and the right ventricular outflow obstruction are important.
This combination equalizes right and left ventricular pressures;
the magnitude of the right-to-left shunt depends on the degree
of right ventricular obstrucdon.
CLINICAT FEATURES
Cyanosis: Not present at birth, but with increasing hyper-
trophy of right ventricular infundibulum and growth of
child, cyanosis occurs later in the first year of life.
Dyspnea: Usually occurs on exertion, older children gets
relief by assuming a squatting position. Infants and toddlers
play actively for a short time and then sit or lie down and
can resume physical activity within a few minutes.
Paroxysmal hlpercyanotic attacks (hypoxic or blue spells):
This is a particular problem during the first 2 years.It occurs
most frequently in the morning on first awakening or follow-
ing vigorous crying. Infant suddenly becomes hyperapneic,
restless; cyanosis increases, gasping respiration ensues, and
syncope may follow. Spells may last from a few minutes to
a few hours but are rarely fatal. Severe spells may progress
to unconsciousness, convulsions, or hemiparesis.
Growth, development, and puberty are delayed.
DIAGNOSIS
Physical examination:
o Cyanosis is variable and sometimes may be absent (acyanotic
tetralogy).
o Digital clubbing and hyperapnea at rest are directly related
to the degree oF cyanosis.
o Precordial bulge may be present. A right ventricular heave
is present. S, is normal, S, often is single, a harsh systolic
ejection murmur 3-516 is heard along the sternal border; its
length and loudness are inversely proportional to the degree
of outflow obstruction. The more severe is the pulmonary
stenosis, softer and shorter is the murmur.
Laboratory evaluation:
o Chest x-ray: The heart size is normal. The apex is uptilted,
and a concavity is noted in the pulmonary segment, giving
heart the appearance of a boot. The pulmonary vascular
markings are diminished according to the severity of outfow
obstruction. The aortic arch may be in the right side.

ESSENCE OF PEDIATRICS
1. Place in knee elbow position
2. GiveO,
morphine
3. lnject lV
mg/kg
dose: 0.1
lf this fails
Fig. 9.1: Treatment of hypercyanotic spell.
o ECG shows right-axis deviation and evidence of right
ventricular hypertrophy.
o The two-dimensional echocardiogram is diagnosdc; it demon-
strates the location and size of the VSD, the outflow obstruc-
tion, the size of the pulmonary annulus, and the degree of
aortic oyerride. Doppler analysis makes it possible ro esrimate
the right ventricular ourflow pressure gradient, and color flow
mapping demonstrates rhe direction of flow through the VSD.
o Cardiac catheterization is useful in measuring the degree
ofdesaturation and the pressures in the ventricles and aorta.
Angiography is used to evaluare the pulmonary and coronary
arteries, the ventricular septal defect, and the nature of the
right ventricular outflow obstruction.
TREATMENT
Medicol
All patients with tetralogy of Fallot need surgical correction.
\Tithout surgery, 85o/o of infants die before they reach their
teens. Medical management includes the following:
o Correction ofanemia: Relative iron-deficiency anemia should
be corrected with iron, as anemia may increase frequency of
paroxysmal cyanotic attacks in infanry and cerebrovascular
accident. Increased demand for red cell production may cause
iron deficiency (though these cases may have normal Hb and
PCV level), and as the iron deficient red cells are more rigid,
they increase the risk of cerebral thrombosis.
o Tireatment of polycythemia: The Hb and PCV should be
monitored. Once PCV is higher than 650/o, there is increased
risk of cerebral thrombosis; symptoms of hyperviscosiry may
require phlebotomy. Venesection of 100-500 ml is carried
out either daily or EAD until PCV is about 55ol0. Blood
may be replaced with plasma, dextran, or saline ro prevenr
hypovolemia after phlebotomy.
r Prevention of dehydration: Prevention or prompr rrearment
of dehydration is required to avoid hemoconcenrrarion,
which may lead to thrombotic attack.
o Prophylactic antibiotic for infective endocarditis is required
for minor or major surgical procedures.
Early and prompr rrearment should be employed for
intercurrent infections. \7hen infective endocarditis is
suspected, treatmenr should be started without delay.
I
4. lnject lV sodium bicarbonate
1
I dose: 1 mmol/kg
i
i 5. lnject lV propranolol
i
0.1 mg/kg given over 1 min
I
1' -------l
o Tireatment of parorysmal hypercyanotic attacks: One or
more of the following procedures should be instituted in
sequence (Fig. 9.1):
c Placement of the infant on the abdomen in knee-chest
position or knee-elbow position.
o Oxygen (100%) by mask.
o Morphine sulfate 0.1-0.2 mg/kg SC.
o Sodium bicarbonate 1 mmol/kg IV to correct acidosis,
if present. Recovery from spell is rapid once rhe pH has
returned to normal.
o Propranolol 0.1-0.2 mg/kg IV especially in severe spells
and when accompanied by tachycardia.
Phenylephrine IV may be used in very limited cases ro
relieve symptoms.
o Keep the child calm and unnecessary blood tests and ma-
neuvers should be avoided.
o Prophylaxis. To decrease the frequency and severiry of the
hypercyanotic spell, oral propranolol in a dose of 1 mg/kg
every 6 hour may be given, but it is preferable to refer for
surgical rrearmenr as soon as spell begins.
o Treatment of severe TOF in neonate: Neonate with
severe tetralogl (i.e., marked right ventricular outflow tract
IRVOTI obstruction) requires prompr medical treatmenr and
surgical intervention. The therapy is aimed at providing an
immediate increase in pulmonary blood flow. Prostaglandin
El (0.05-0.2 pg/kg/min) should be administered by iV
infusion in neonates to keep the ductus arteriosus open and
provides adequare pulmonary blood fow until a surgical
procedure can be performed.
Surgicol
Palliative Shunt Procedures
Palliative shunt procedures are performed ro increase pulmonary
blood flow. Infications are rhe following, especially in the
poorer narions where primary repair is difficult:
r Neonates with TOF and pulmonary atresia.
o Infants with hypoplasric pulmonary annulus and hypo-
plastic PAS.
o Severely cyanotic infants-younger than 3 months and those
who have medically unmanageable hypercyanotic spells.

The shunt procedures include:
Modified Blalock-Taussig (B-T) shunt: Anastomo-
sis between subclavian artery and ipsilateral pulmo-
r- nary artery is usually done for an infant older than
3 months of age. The original B-T shunt consisted of a
direct anastomosis of the subclavian artery to a branch
pulmonary artery.
Gore-Tex interposition shunt, placed between subclavian
artery and ipsilateral PA, is ideal for infants younger than
3 months,
\Taterston shunt and Pott operation have been abandoned
because of high percentage of complications.
Total (orrective Surgery
Timings vary, depending upon the patient, but early surgery
is always preferred.
Indications and timing:
r Symptomatic infants who have favorable anatomy of RV
outflow tract and PAS-an early repair is advised, any time
after 4 months of age.
o Mildly cyanotic children who have had shunt surgery-total
repair l-2 years after shunt operation.
Procedure: Brockt procedure. Total repair of the defect is
carried out under cardio-pulmonary bypass and circulatory
arrest. RVOT obstruction is relieved by removing muscle
bundles; patch closure of VSD is done. If pulmonary valve
is stenotic, a valvotomy, and if pulmonary valve annulus is
'When
small, a valvectomy is performed. there is a previously
established systemic to pulmonary shunt, it must be obliterated
prior to full repair. Risk of total correction is <5%o.
Complications:
Cerebral tbromboses: Usually occurs in cerebral veins and
dural sinuses in presence of extreme polycy'themia and dehy-
dration. Phlebotomy and volume replacement are indicated
in the extremely polycy'themic patient.
Brain abscess: >2 year of age. Patient may have headache,
vomiting, seizures, and localized neurological signs with
raised intracranial pressure.
Antibiotic treatment may help to keep the infection
localized, but surgical drainage of the abscess is usually
necessary.
Bacterial endocarditis: Occurs in right ventricular infun-
dibulum or on the puimonic, aortic, or rarely tricuspid
valves. Prolonged treatment with antibiotics should be
given. Antibiotic prophylaxis, however, is essential prior to
and after dental and cefiain surgical procedures.
Heart failure: Rare. May occur in young infant with pink
or acyanotic TOF or in severe iron-deficiency anemia. Tieat-
ment with diuretics and digoxin should be given.
Cerebrouascular accidcnt: Supportive treatment, physio-
therapy is advised.
I
CAR DIOVASCU LAR DISEASES
In Ebstein anomaly (EA), the abnormal tricuspid valve is dis-
placed downwards into the right ventricle. The anterior cusp of
the valve retains some attachment to the valve ring, but other
leaflets are adherent to the right ventricle. The right ventricle is
thus divided into two parts by the abnormal tricuspid valve-
the first, a thin walled atrialized portion, is continuous with
the right atrium; the second consists of normal ventricular
myocardium. The ri6hr atrium is hu6e, and the rricuspid valve
is usually regurgitant. The effective output from the right side
of the heart is decreased because of the poorly functioning
small right ventricle, tricuspid valve regurgitation, and variable
degrees of obstruction of the right ventricular outflow tract
produced by the large sail-like anterior tricuspid valve leaflet.
The increased volume of the right atrial blood shunts through
the foramen ovale to the left atrium producing cyanosis.
EA patients are known to have high potential for developing
arrhythmias. PDA or coarctation of aorta may remain associ-
ated. In many patients, symptoms are mild, although some
patients may be asymptomatic until well into adult life.
CTINICAL FEATURES
Newborn infants with EA often present with severe cyanosis
and massive cardiomegaly. In many patients, symptoms (fadgue,
exertional dyspnea) and signs (cyanosis, conjunctival conges-
tion, clubbing, growth retardation) are present.
A holosystolic murmur is audible over the lower left sternal
border due to tricuspid regurgitation; gallop rhythm is also
common.
INVESTIGATIONS
ECG usually shows RBBB, tall and broad P waves, and some-
times Wolff-Parkinson-'il4rite syndrome. On chest x-ray, heart
size varies from normal to massive, box-shaped cardiomegaly;
pulmonary vasculature may be normal or decreased. Color
Doppler echocardiography shows the displaced tricuspid valve,
dilated right atrium, variable degree of tricuspid regurgitation,
and other associated cardiacdefects. Cardiac catheterization and
selective angiocardiography confirms large right atrium, abnormal
tricuspid valve, and right-to-left shunt at the atrial level. Prenatal
diagnosis of EA can be done by fetal echocardiography.
TREATMENT
o ticuspid valvuloplasty associated with longitudinal right
ventricular plication is superior to valve replacement.
o Neonates with severe hypoxia who are prostaglandin
dependent have recently been treated by surgical patch
closure of the tricuspid valve, atrial septectomy, and
piacement of an aortopulmonary shunt (Starnes procedure).
 ESSENCE OF PEDIATRICS
This operation creates a functional tricuspid atresia, which
can then be further repaired with first a Glenn and then a
Fontan operation.
o Radiofrequency current (RFC) ablation can be used safely
and effectively for treatment of various types of tachycardias
in patients with EA.
Pulmonary stenosis accounrs for 5-8o/o of congenital heart
defects. The pulmonary commissures are fused, and the valve
is domed and has a small central or eccentric opening; there
is post-stenotic dilatation of the main pulmonary artery. The
pulmonary valve is occasionally bicuspid.
PATHOPHYSIOTOGY
To maintain cardiac output, right ventricular pressure rises.
In severe stenosis, right ventricular end-diastolic pressure may
also increase. A consequent increase in right atrial pressure may
open the foramen ovale and cause a right-toJeft shunt.
CLINICAL FEATURES
Most patients are asympromaric. Severe to critical pulmo-
nary stenosis may cause exertional dyspnea, fatiguabiliry and
exertional chest pain. Congestive heart failure is unusual, except
in infants with critical srenosis.
DIAGNOSIS
Physical examination:
r An ejection click-the loudness of which varies with respi-
."1ion-2nd a harsh systolic ejection murmur are present
at the upper left sternal border.
o In moderately severe srenosis, a thrill and right ventricular
heave are palpable, the pulmonary component of S, is
diminished, the ejection click merges with S,, and the
murmur becomes longer and louder.
o If the stenosis is critical, cyanosis and an S, gallop may
be found.
Laboratory evaluation:
o Chest x-ray: Heart size and pulmonary vasculariry usually
are normal, but the pulmonary arrery segment is promi-
nent because ofpost-stenotic dilatation. In critical stenosis,
cardiomegaly and diminished pulmonary blood flow mav
be seen.
o ECG: The degree of right axis deviation and right ventricular
hypertrophy correlates well with right ventricular pressure
and therefore, with severiry of the stenosis. An R wave of
20 mm or greater in lead V, or a positive T wave is indicative
of systemic right ventricular pressure.
Echocardiogram: Findings depend on rhe degree of steno-
sis. Right ventricular hypertrophy, dilatation, doming of
the pulmonary valve, and post,stenotic dilatation of the
pulmonary artery can be seen.
Cardiac catheterization: Right ventricular function, anaromy
of the pulmonary valve, and the rransvalve gradient can be
assessed accurately.
TREATMENT
o Medical: Bacterial endocarditis prophylaxis is necessary.
Percutaneous balloon angioplasty of the pulmonary valve
may be performed ar the rime of cardiac carhet€rizarion.
o Surgical: Surgical resection of the pulmonary valve is
reserved for those patienrs in whom balloon angioplasry had
failed, including patients with dysplastic valves.
D-transposition of the great arreries, also known as simple
transposition, accounts for 5o/o of congenital heart defects
and is more common in boys than in girls. The anatomy is as
follows: the aorta arises from the right ventricle anteriorly and
to the right of the pulmonary arrery, which arises posteriorly
from the left ventricle. Associated abnormalities may include
VSD, PDA, pulmonary srenosis, or a combination of these.
PATHOPHYSIOTOGY
Systemic (unoxygenated) blood is recirculated through the
body, and pulmonary venous (oxygenated) blood is recircu-
lated through the lungs. A lesion that allows mixing of the
systemic and pulmonary circulations (e.g.,ASD, VSD, PDA)
is necessary lor survival.
CtINICAI FEATURES
Cyanosis is present from birth, the degree varying with the
associated mixing lesion.
DIAGNOSIS
Physical examination: Intense cyanosis is noted in the absence of
mixing lesions. In addition, a right ventricular heave and a single
loud S, are usually found, and a soft flow murmur may be heard.
Laboratory evaluation:
r Chest x-rav: Pulmonary vasculariry is increased or may be
normal. Slight cardiomegaly and a narrow base produced
by the anterior-posterior arrangement of the great arteries
give the heart the shape of an egg on its side.
o Arterial blood gas analysis shows severe hypoxemia; increas-
ing the ambient FiO, to 100% does not significandy alter r
1
the arterial POr. a
)
 I
r
1.

r CARDIOVASCU LAR DISEAS ES

l
v
t"
v
a ECG is normal in the newborn.
I a Echocardiogram shows the anterior-posterior arrange-
t
ment of the great arteries and the chamber from which
l o
F they originate, the normal anatomy of the ventricles,
and the presence of associated abnormalities. Color flow
ts
mapping shows the direction of flow through associated
abnormalities.
Cardiac catheterization: fught ventricular pressure is sys-
temic, left ventricular pressure may be systemic in the
newborn, but decreases with a decline in pulmonary vas-
cular resistance. Angiography confirms the anatomy and
indicates the direction of blood flow. Balloon atrial septos-
tomy (Rashkind procedure) allows the creation of an ASD,
through which the mixing of oxygenated and deoxygenated
blood can occur.
TREATMENT
Medical: Creation of an ASD by balloon atrial septostomy
may be lifesaving. Correction of acidosis, hypoglycemia, and
hypocalcemia in the neonatal period improved myocardial
function.
Strrgical: The current procedure of choice is the arterial
switch procedure with coronary artery re-implantation,
rnhich must be performed in the neonatal period and
I involves moving the arteries, but not the valves' into their
"normal" position.
i Laboratory evaluation:
t
t flow is present in unobstructed anomalous pulmonary
In this defect, the pulmonary veins are not incorporated into
the left atriurn. Instead, they carry orygenated blood to the
i, right atrium either directly or indirectly via venous channels.
I There are four possible routes:
l o Supracardiae (50o/o of cases): The pulmonary veins enter
] from hyaline membrane
the innominate vein and thence to the superior vena cava
tv
or directly to the superior vena caYa.
r Cardiac (2Do/o of cases): The pulmonary veins enter into
the coronary sinus or directly into the right atrium.
Infracardiac, also known as infradiaphragmatic (2Uo/o of
cases): The pulmonary veins drain into the portal or hepatic
vein and thence into the inferior vena cava'
Mixed (10% of cases): Pulmonary venous blood returns to
the heart via a combination of the above routes.
PATHOPHYSIOTOGY
o There is an obligatory right-to-1eftshunt, usuaily via a
stretched foramen ovale or an ASD. The magnitude of
pulmonary blood flow is inversely related to the pulmonary
venous resistance.
o High resistance to pulmonary venous flow may exist in all
rypes but is most common in the infracardiac rype.
o If obstruction is severe, the pressure in the pulmonary veins
and arteries is high, pulmonary blood flow is low, and sys-
temic arterial blood is markedly desaturated.
If there is no obstruction, pulmonary blood flow is high;
pressures are only mildly elevated, and arterial blood is only
mildly desaturated.
CI.INICAI FEATURES
Symptoms depend on the degree of pulmonary venous
obstruction. In severe obstruction, the classic presentation
is that of an intensely cyanotic newborn with tachypnea and
dyspnea. If no obstruction is present, desaturation may be
subclinical, and symptoms are those of pulmonary hyperflow.
DIAGNOSIS
Physical examination:
o If pulmonary venous fow is obstructed, there is a right
ventricular heave and a loud, narrowly split Sr, a gallop
rhythm, but usually no murmur.
o If pulmonary venous flow is unobstructed, the findings are
similar to those of a high-flow ASD, including a prominent
and active precordium with a right ventricular heave, clinical
cardiomegaly, wide and fixed split Sr, with a loud pulmo-
nary component, systolic ejection murmur at the upper
left sternal border with wide radiation, and mid-diastolic
rumble at the lower left sternal border.
o Chest x-ray: Cardiomegaly with increased pulmonary blood
venous return. In the supracardiac type, the base of the
heart may appear widened by dilated veins, and the entire
silhouette may have "a snowman" or figure "8" appearance.
In obstructed venous return, the heart size is normal, and
diffuse pulmonary edema may be difficult to distinguish
disease.
e ECG indicates right axis deviation and right ventricular
hypertrophy.
Echocardiogram: The right side of the heart and the pulmo-
nary artery are enlarged; left sided structures may be relatively
small. The pulmonary veins do not enter the left atrium. The
coronary sinus may be enlarged if the veins drain into it; in the
infracardiac qpe, the common pulmonary vein may be seen
behind the left atrium with the communicating vein traversing
the diaphragm. Such anomalies can be subtle and easily missed.
Color flow mapping is helpful in hlghlighting the pulmonary
veins and evaluating the direction of flow.
Cardiac catheterization: Because of mixing, oxygen satura-
tion is similar in all chambers and arteries. Saturation is
highest if sampling is performed near the drainage site of the
pulmonary veins. Pulmonary artery pressure is proportional
to the degree of pulmonaryvenous obstruction. Pulmonary
arteriography defines the pulmonary venous drainage.
 !

ESSENCE OF PEDIATRICS

Table 9.2: Cyanotic Congenital Heart Disease in Newborn

Persistent fetal ci rculation 1:400 live births Normal or decreased Normal RADTRVH
I ransrent myoLardtdl tschemra 307o of nonstructural lncrease. Pulmonary Cardiomegaly T-i nversion
heart disease edema
D-transposition of great arteries 5"k lncrease Egg-shaped with narrow Normal or RAD, RVH
pedicle
Total anomaious pulmonary J'r. lncrease Normal or small RAD, RVH, RAH
venous return with obstrrrction
Severe Fal lot tetralogy 4"/" Decrease Boot-shaped RAD, RVH
Incusprd atresia 2% Decrease Square heart LAD, LVH, RAH
Hypoplastic Ieft heart syndrome I ncrease Enlarged RAD, RVH
RDA, right axis deviation; RVH, right ventricular lr;,pertrophy; RAH riglrt atrial hypertrophy

TREATMENT CLINICAL FEATURES

o Medical: Congestive heart failure is treated. If pulmonary
edema is present, diuretics should be given. Mechanical
ventilation may be necessary.
o Surgical: Surgical redirection of the pulmonary veins into
the left arrium can be accomplished in all four types.
o Dextrocardia without any other cardiac defect-no
symptom.
o Apical impulses and heart sounds are heard on right side.
ECG findings: P wave is inverted in lead I, and lead II
resembles normal lead III and vice versa. 2D echocardiography
is extremely useful in defining the complex anatomy.

CYANOTIC CONGENITAT HEART DISEASES

Refer Tables 9.2 and 9.3 for lesions and their different fearures
in cyanotic congenital heart disease in newborns, infants, and
children; Thble 9.4 for causes of increased or decreased pul-
monarv blood {low.
TREATMENT
Dextrocardia with situs inversus and with no heart defects
needs no treatment, and the prognosis is excellent.

In the pediatric age group, the sequelae of rheumatic fever

In dextrocardia, apex of heart poinrs ro the right. There may or
may not be reversal of position of other organs (situs inversus).
If there is no reversal of other organs, the heart usually have
severe defects with complete situs inversus where othel organs
such as stomach and iiver are in opposite side; the heart is
usually normal.
consist of mitral, aortic, and tricuspid valve diseases. The mitral
valve involvement manifesrs predominandy as mitral regurgi-
tation and less commonly as mitral stenosis. The aortic valve
and tricuspid valve (rare) involvement presenr exclusively as
aortic and tricuspid regurgitation, respectively. Rheumatic aortic
stenosis has never been described below the age of 15 years.

Table 9.3: Cyanotic Congenital Heart Disease in lnfancy and Childhood

Fallot tetralogy Decrease Boot-shaped RVH, RAD VSD with rightto-left shunt
lotal anomalorrs lncrease Small if infracardiac, RVH Step up O- s.lturation in supgyl61 \ena ca\ d.
pulmonary venous return snowman if supracardiac, inferior vena cava, or right atrium.
large ii cardiac.
Single ventricle lncrease Large heart B iventricu lar hypertrophy O, step up in RV, PA, & AO. Saturation similar.
Truncus arteriosus lncrease Large heart B iventricular hypertrophy VAS, AO, Entered from right ventricle.
Ebstein anomaly Decrease Large and square RAH, RBBB Right-left shunt at atrial level.
VSD, ventricular septal defect; RV, right ventricle; RAH, right atrial hypertrophy RAD, right axis deviation; RVH, right ventricular hypertrophv; RBBB, right bundle branch
block; AO, aorta.
 !

CARDIOVASC U LAR DISEAS ES

Table 9.4: Causes of lncreased or Decreased Pulmonary Surgical:

Blood Flow
Transposition of great arteries
Total anomalous pulmonary veins
Truncus drteriosus
Tricuspid atresia
Pulmonary valve atresia with
intdct ventricular septum
Pulmonary valve atresia with VSD
Truncus arteriosus
Mital valve surgery: Indications include intractable CCB
progressive cardiomegaly with symptoms, and pulmonary
hypertension.
Mitral replacement or repair: In children, valve repair is
preferred because the valve is pliable and has a low mortaliry
rate. If valve is thick, scarred, grossly deformed, then valve
has to be replaced.

MITRAT REGURGITATION
Mitral regurgitation (MR) is the most common RHD. Mitral
valve leaflets are shortened because of fibrosis, which leads to
dilatation of mitral valve ring.
L eventually pulmonary congestion and hypertension develops.
Clinicol Feqlures
)
t- May be asymptomatic. Dyspnea on exertion, fatigue, and
i, palpitation are the commoner presenting features. The pulse
pressure is increased (small water hammer pulse). Apex beat
I congestion may cause a cough, recurrent bronchitis, and pulmo-
I is hyperdynamic. The first sound may be soft; generally it is
v inaudible as it is masked by the systolic murmur. The second
I
sound is normally split, and diminished. There may be a third
t:
heart sound due to rapid ventricular filling. The classical diag-
nostic sign is the pansystolic murmur, best heard at the apex
and radiating with equal intensity to the axilla and back, and
best heard in left decubitus position.
lnvesiigolions
Chest skiagram may show enlargement of heart; left atrial
enlargement may be inferred from the elevation of left bron-
chus but is more clearly outlined with barium swallow in right
anterior oblique (RAO) position. Features of left ventricular
and left atrial hypertrophy may be present in ECG. 2D echo
shows dilated left atrium (LA) and left ventricle (LV). Color
Doppler echo shows regurgitant jet into lA and assesses the
severity of regurgitation.
Treqtmenl
Medical:
o Mild regurgitation with no symptom-no treatment is
required. Mild to moderate mitral regurgitation is well-
tolerated for long periods.
o Diuretics to reduce pulmonary congestion; digoxin to control
the ventricular rate in atrial fibrillation (atrial fibrillation is
common as a consequence of atrial dilatation).
o Infective endocarditis and CCF should be treated.
r Prophylaxis
c Secondary prophylaxis against rheumatic fever for pro-
longed period, even for life, has been recommended.
r Antibiotic prophylaxis against infective endocarditis'
r Maintenance of dental hygiene.
MITRAT STENOSIS
Mitral stenosis (MS) is rare in children (it takes 5-10 years from
the initial attack to develop). In MS, the leafets are thickened
with fusion of commissures and calcification sets in. LA and
right ventricle (R\0 become hypertrophied and dilated, and
Clinicol Feolures
Dyspnea is the commonest presenting symptom. Pulmonary
nary hypertension leading to hemoptysis; hemoptysis can also
be due to pulmonary infarction from embolus originating in leg
veins. Systemic embolism is sometimes the presenting feature.
Pulse may be small or irregular due to atrial fibrillation.
The first heart sound is loud, may be palpable. The pulmonary
second heart sound (P2) is usually loud. Opening snap is a
high-pitched metallic sound that occurs immediately following
second heart sound. Typical murmur is low-pitched rumbling
mid-diastolic with presystolic accentuation and localized to
the apex. Accompanying mitral regurgitation may cause a
pansystolic murmur. Tlicuspid regurgitation secondary to right
ventricular dilatation can cause a systolic murmur. Precordium
may be prominent with palpable RV impulse.
lnvestigolions
ECG may show left atrial hypertrophy, atrial fibrillation, or
right ventricular hypertrophy. Chest x-ray shows the enlarge-
ment of the left atrium and its appendage and of the main
pulmonary artery; lung fields are congested. In the lateral and
RAO position, an enlarged left atrium causes a characteristic
backward displacement of the barium-filled esophagus.
Echocardiogram shows thickened mitral valve leaflets with
doming and dilated lA, main pulmonary artery RV RA. Doppler
estimates pressure gradient across valve and PA pressure.
Cardiac catheterization has been extensively used to
confirm the severiry of mitral stenosis.
Treolmenl
Medical:
o For mild dyspnea- diuretics. Digoxin is given to control
the ventricular rate in atrial fibrillation. Anticoagulation is
done to reduce the risk of systemic embolism.
ESSENCE OF PEDIATRICS

a Infective endocarditis and CCF should be ileared, when present. Echocardiogram shows state of aortic valve and may reveal
o Prophylaxis vegetations in infective endocarditis. Color flow and Doppler
o Secondary prophylaxis against rheumatic fevershould be echo assess the severity of AR.
given for prolonged period, even for life.
o Antibiotic prophylaxis should be given against infective Treotmenl
endocarditis. Medical:
Surgery: Indications are exertional dyspnea with paroxysmal o Mild to moderate aortic regurgitation is well-tolerated for years.
nocturnal dyspnea or pulmonary edema; relative indications are o For angina-glyceryl trinitrate; palpitation may respond
recurrent atrial fibrillation, hemoptysis and thromboembolic to beta-blockers.
phenomenon. o Infective endocarditis and LVF should be treated, when present.
Closed mitral commissurotomy is done in patients with o Prophylaxis
significant symptoms having pure mitral stenosis (no associ- c Secondary prophylaxis against rheumatic fever should be
ated MR) and mitral valve non-calcified and pliable. Mitral done for prolonged period, even for life.
valve replacement is indicated if there is substantial mitral c Antibiotic prophylaxis should be done against infective
regurgitation, or if the valve is heavily calcified. endocarditis.
o Good oral hygiene
AORTIC REGURGITATION Surgical:
Aortic valve replacement under cardiopulmonary bypass. Indi-
Aortic regurgitation (AR) is less common than MR. The semilu-
cations for aortic valve replacement inclLlde the following:
nar cusps ofaortic valve are deformed and shortened, thereby, the
aortic valve ring gets dilated and the cusps fail to appose lighdy. o tillhen symptoms, such as angina or exertional dyspnea
have appeared.
Clinicol Feolures o In asymptomatic patient, when either cardiothoracic ratio
is >55o/o or LV ejection fraction is <40o/o.
There may be no symptoms for years till cardiac failure devel-
ops. Palpitation is frequently a distressing symptom. With the
onset of LVF, dyspnea appears and rapidly progresses. Angina
is less common than in aortic stenosis. \,){/hen the leak is
Infective endocarditis is an inflammatory disorder, mainiy of the
large, the stroke output of the left ventricle may be doubled
cardiac valves, that resuits from infection by any ofseveral types of
or trebled, the major arteries are then conspicuously pulsatile
(prominent carotid pulsations [Corrigan's sign], visible arte- microorganisrns, including bacteria, fungi, rickemsiae, and viruses.
Acute endocarditis is a hectically febrile illness that rapidly
rial pulsations over the extremity vessels [dancing peripheral
arteries]). Arteriolar pulsations may be seen over the nail beds,
damages cardiac structures, hematogenously seeds in exrracar-
lips, ear lobes, and in the eye grounds. If the stethoscope is diac sites, and if untreated, progresses to death within weeks.
put over the brachial or femoral artery, pistol shot sounds may
be heard. Pulse is collapsing rype (water hammer) with rapid ETIOLOGY
rise, rapid fall and no sustenance.
o StrEtococcus uiridans: More common after dental procedure.
The pulse pressure is wide, because of increased systolic and
c Staphlthcoccus aureus and Staph/ococcus epidermidis: Common
lowered diastolic pressure. Apex beat is displaced laterally. The
in patient with no underlying heart diseases.
S, and S, are diminished; { is delayed and accenruated. There
. Enterococczs: Common after lower bowel or genitourinary
is a high-pitched early diastolic murmur immediately following
manipulation.
the second heart sound, usually best heard along the left sternal
o Pseudornonas aeruginosa: More common in intravenous
border in the 3rd and 4th interspace and radiates down the left
drug abusers.
sternal border to the apex. The murmur is best heard when the
o Culture negarive (107o of cases).
patient sits up and leans forward holding breath in expiration.
The longer the murmur, the more severe is the AR. Vibration of
RISK FACTORS
the anterior mitral cusp due to regurgitant blood srream produces
a low-pitched apical diastolic murmur (Austin-Flint murmur). o Children with acquired valvular disease
\7ith large aortic leaLs, there is also an ejection systolic murmur o Children with congenital heart disease
at the second right interspace, conducted to the neck.
:
o Intravenous drug abuse
o Children with central venous line i
Invesligolions o Poor oral hygiene
ECG shows left ventricular hypertrophy. Chest x-ray shows o No antibiotic coverage during minor or major surgical t
cardiac enlargement of the left ventricular rype. intervention a
t
.l
1
 CARDIOVASCU LAR DISEASES

PATHOGENESIS Table 9.5: Organisms Causing Endocarditis, Choice of Antibi-

otics, and Duration of Treatment
Endocarditis develops when a jet of blood, turbulence, or trauma
leads to cardiac endothelial damage, which serves as the nidus for
Streptococcus Penicillin and Cefazolin and 4 wk
bacterial infection. In most cases oral bacteria, which intermit- viridans aminoglycoside aminoglycoside
tently invade the blood stream, infect the damaged endothelium.
Croup A Penicillin and Cefazolin and 4 wk
The prototypic lesion at the site of infection, the'vegetation', streptococci amrnoglycosrde aminoglycoside
is a mass of platelets, fibrin, microcolonies of microorganisms Ampicillin and Vancomycin ancl 6 wk
Strcptocaccus
and scanry inflammatory cells. They may be single or multiple faecalis aminoglycoside arni noglycoside
and range in size from a few millimeters to several centimeters. Cloxacillin and Vancomycin and 6 wk
Staphylococcus
Pieces of the vegetations may break off and cause embolization aureus aminoglycoside aminoglycoside
(e.g., Rotht spot, splinter hemorrhage).
Escherichia coli Ceftriaxon and Ampicillin and 6 wk
aminoglycoside aminoglycoside
CTINICAL FEATURES Pseudomonas llazrcrLlrn anal 6wk
aminoglycoside
. Symptoms: Fever, chills, night sweats, myalgia, arthralgia, Culture negative Ampicillih and Cloxacillln and 6wk
loin pain, neurological manifestation (stroke, seizure, head- aminoglycoside aminoglycoside
ache), dyspnea.
o Signs: Thchycardia, clubbing, hematuria, new or worsening Principles of management are (l) identification of the
regurgitant murmur, splenomegaly, petechial rash, peripheral organism; (li) determining its antibiotic sensitivity; (iii) srart-
manifestation due toembolism (Osler node, splinter ing treatment as early as possible; and (iu) using bactericidal
hemorrhage in nail bed, Janeway lesion, Roth spot), antimicrobial agent(s) for an appropriate duration to obtain
arrhythmia, heart failure. cure and prevent relapse.
If the blood culture is positive, the choice of antibiotics is
coMPUCATTONS dictated by the antibiotic sensitivity. Ifthe culture is negative,
empirical therapy covering a wide range of organisms is nec-
Cardiac: Valvular insufficiency or stenosis with cardiac essary. Organisms causing endocarditis, choice of antibiotics,
failure, myocardial ring abscess, suppurative pericarditis and duration of treatment are listed in Thble 9.5. Table 9.6
Embolic Lefi-sided lesion-brain (cerebral abscess, menin- depicts the prophylactic measures to be taken during dental
gitis), spleen (abscess), kidney (abscess). Right-sided lesion- procedures and genitourinary and gastrointestinal operations.
lungs (abscess, pneumonia)
Note: High serum bactericidal levels must be maintained long enough to
Renal: Infarction, focal glomerulonephritis leading to neph- eradicate organisms that are growing in relatively inaccessible avascular
rotic syndrome or renal failure, diffuse glomerulonephritis vegetations. From 5 to 20 times the minimum in vitro inhibiting
leading to renal failure concentration must be produced at the site of infection to destroy
Depression may occur due to prolonged hospitalization. bacteria growing at the core of these lesions. Several weeks are required
for a vegetation to organize completely thus therapy must be continued
through this period, so that recrudescence can be avoided. A total of
INVESTIGATIONS 4-6 weeks of treatment is recommended.

Blood culture: At least three blood cultures within 24hours

from three different venepunctures, with at least I hour gap
beween two punctures (Positive > 9070 cases).
Leukocytosis, low hemoglobin content, proteinuria, micro-
scopic hematuria, raised ESR and CRP, hypergammaglobu-
linemia, hypocomplementemia, rheumatoid factor (+ve),
high blood urea, and serum creatinine levels.
ECG may reveal conduction defect due to abscess formation
or infarction due to emboli.
a Chest x-ray shows evidence of cardiomegaiy or heart failure.
a Echocardiography for detection of valve vegetations.
TREATMENT
The treatment of endocarditis can be considered under the
rwo headings-(l) treatment of the current episode and
(li) prevention of endocarditis.
The heart is safely enclosed in a pericardial caviry, the sero-mem-
branous visceral pericardium forming the outer layer of the heart
and the fibrous perietal pericardium separating it from the lungs.
It contains about 15-50 ml of ultrafiltrate of plasma.
ACUTE PERICARDITIS AND PERICARDIAL
EFFUSION
Pericardium gets involved in acute infective, inflammatory, neo-
plastic or traumatic processes. Pericardial involvement is most
often seen as one of the clinical manifestations of a systemic
disorder or ageneralized illness. \Thenever pericardial involve-
ment is detected clinically, a thorough clinical examination ro
detect the presence of generalized disease is essential.

ESSENCE OF PEDIATRICS
Table 9.6: Infective Endocarditis Prophylaxis
Dental treatment Penicillin V 2 g by mouth on an empty stomach
I hour before dental Lreatment. followed by 0.5 g
every 6 hours for J days; or
Crystalline penicillin C 1 million units mixed
with 600.000 l.-r oi procaine penicillin given lM
J0 60 minutes before procedure, followed by
oral penir illin V as above; or
Single dose of amoxicillin 50 mg/kg by mouth
I hour earlier.
Alternalivety. a single injeclion oI
various
forms of penicillin (300,000 units of crystalline,
300,000 units of procaine, and 500,000 units
.1
of benzathine penicillin) hour before the
procedure.
Patients with prosthetic heart valve: lM penicillin
with streptomycin or gentamicin t hour before
the procedure.
Cenitourinary and Amoricillin 25 mg/kg by mouth I hour before
gastrointestinal and 2 mg/kg lM gentamicin 30 minutes before
operations the operation. Additional metronidazole for
the patients who are going in for Cl operations.
Amoxicillin and gentamicin are repeated at least
for two more doses after the procedure.
Pothophysiology
Pericardial involvement, often as a result of infective or infam-
matory process, results in variable accumulation of fluid within
the pericardial sac, the degree of which will determine the
clinical picture. Mild effusion occulring in cardiac failure
or nephrotic syndrome as a transudate may go unnoticed.
Pyogenic infection will cause pyopericardium in which even
a small amount may be significant clinically. The symptoms
and signs are due to severe cardiac compression, impairment of
ventricular diastolic filling, increased systemic and pulmonary
venous pressures and eventually due to severely compromised
cardiac output and shock.
Clossificqtion of Pericqrdiiis qnd
Pericqrdiol Effusion
Clinical:
o Acute (< 6 weelis): Fibrinous, serofibrinous, effusive' serous,
purulent, hemorrhagic, chylous
o Subacute (6 weeks to 6 months): Effusive, constrictive
o Chronic (> 6 months): Constrictive, effusive, adhesive
Eliology
I. Infectious
o Viral: Coxsackie B, ECHO, adenovirus, cvtomegalo-
virus, varicella, rubella, herpes, influenza
o Bacterial: Staphl,/6s6s6i, strept0c0ccl, prteuntococci'
Haemophilus infuenzae, menlngococci, Sdlmonel/a,
Myco b acterium tu b ercu /o s is, Myc op lasma
-
o Fungal: Histoplasmosis
o Protozoal: Amebiasis, toxoplasmosis
II. Non-infectious, inf ammatory (hypersensitivity, auto-
immuniry)
o Rheumatic fever
o Coilagen vascular disorders: SLE, Rheumatoid arthritis,
scleroderma
o Drug-induced: Hydralazine, phenytoin, isoniazid
III. Non-infectious, non-infammatory
o CHF, nephrotic syndrome, cirrhosis liver
o Neoplasia: Lymphoma, leukemia
IV. Miscellaneous: Uremia, m;.xedema, chylopericardium,
sarcoidosis, bleeding disorders, radiotherapy.
Clinicol Feqlures
Acute Pericarditis
The chest pain of acute pericarditis is dull, sharp and stabbing
precordial pain, radiating to the epigastrium, neck, shoulder
or left arm, increasing on lying down and on deep inspiration,
decreasing on sitting position with forward leaning. Pericardial
sac is devoid of nerve supply and hence, the pain is due to
adjacent diaphragmatic or pleural irritation.
Cough, fever and breathlessness of a mild degree may be
associated symptoms.
The symptoms of primary etiology may overshadow the
mild secondary symptoms.
In acute pericarditis without significant effusion, in the early
stages, the only clinical finding may be a pericardial friction
rub, heard best with the diaphragm of the stethoscope kept
firmly pressed on the left sternal border and over the base of
the heart. It resembles the sound produced by the friction of
sandpaper on wood, better heard during expiration. It has a
to-and-fro component.
The ECG shows generalized elevation of the ST segments
(current of injury of the underlying myocardium). After a week,
the ST segments return to normal, but there will be T wave
flattening and inr.ersion in the same leads, which may persists
for months after resolution of the acute phase.
Pericardial Effusion
Vithin short period of 48-72 hours, most cases rvill go on to
develop pericardial effusion. Pyogenic organisms, stil1 one of
the most important causes of pericardial involr'ement, cause
pyopericardium and there is often high fever, toxemia, and
other clinical evidences of pvogenic inlections like pneumonia,
einpvena, or pvoderma. Tuberculous effusion is the next most
comrrron cause of pericardial effusion in developing countries.
This is follou.ed bv r.iral infections.
Svmptoms olchest pain u'ith a feeling of compression over
the chest in a child rvhile lying down, elevation of jugular
\renous pulse, presence of pulsus paradoxus, enlarged area of
 CARDIOVASC U LAR DIS EAS ES

cardiac dullness and diffuse apical impulse, well within the The diagnosis of tamponade is mainly indicated by the
outer border of cardiac dullness, shifting dullness, muffied clinical signs of pulsus paradoxus, prominent X-descent and
heart sounds with friction pericardial rub and unexplained absent Kussmaul sign (paradoxical rise in jugular venous pulse).
hepatomegaly all suggest pericardial effusion with moderate
collection. Treotmenl
Radiograph of the chesr shows "waterbottle" type of cardio-
megaly with no increased lung shadows. Fluoroscopy shows
o Pus in the pericardial sac is surgically drained with the
administration of appropriate antibiotics for prolonged
diminished myocardial contracdlity.
period. This has resulted in improved survival. Constrictive
ECG often shows diminished QRS voltages with gener-
pericarditis may occur, and the child must be on regular
alized ST segment elevation. The diagnosis is easily made
follow-up protocol.
with echocardiography. Pericardiocentesis is done through
Tuberculous effusion is treated with a minimum of three
the subxiphoid approach, and the fluid content is analyzed
antituberculous drugs and initial course of steroids. Chronic
microscopically, biochemically, and microbiologically. Gram
constrictive pericarditis, a common complication, is treated
staining is done to identi$' the bacteria. In hemorrhagic effu-
surgically. It can be prevented by early pericardiectomy.
sions, cytoanalysis for malignant cells is also undertaken.
Pericardiocentesis is done for diagnostic purpose, but if
the collection is large resulting in tamponade, therapeutic
ACUTE CARDIAC TAMPONADE removal of significant amount of fluid becomes essential.
Viral effusion needs only symptomatic treatment, and it
The common causes of cardiac tamponade are tuberculosis, neo-
resolves spontaneously in 2-4 weeks.
plasms, t."n*", uremia, and idiopathic pericardial effirsion.
Other conditions like collagen vascular diseases are treated
This term is applied to the state of acute heart failure due
appropriately and the effilsion resolves slowly with steroids.
to compression of the heart by massive, rapidly accumulating
pericardial effusion. Significant rise in intrathoracic pressure
and ventricular and diastolic, atrial, systemic, and pulmonary CHRONIC CONSTRICTIVE PERICARDITIS
venous pressures occurs due to impaired ventricular relaxation
Though less common in children, constrictive pericarditis may
and filling, resulting in poor cardiac output.
follow tuberculous pericardial effusion or pyopericardium.
t The characteristic clinical symptoms are precordial compres-
Rarely, it may occur secondary to viral or traumatic (hemor-
L sion, breathlessness, exercise intolerance, mild facial puffiness,
) rhagic) pericarditis. It usually occurs months or years after the
L and minimal pedal edema. The characteristic clinical signs of
treatment of primary condition.
t raised jugular venous pulse with prominent X-descent, pulsus
paradoxus, low volume pulse, cold and clammy peripheral4
extremities, relatively silent precordium, muffed heart sounds
Clinicol Feolures
with hepatomegaly suggest cardiac tamponade. The thick, fibrous and sometimes calcified pericardial sac impairs
Low ECG QRS voltages and electrical alternans may be diastolic ventricular filling, myocardial contractiliry and effective
present in cardiac tamponade. Ultrasonogram shows significant cardiac function. These changes gradually result in the develop-
pericardial effusion, right atrial collapse, and right ventricular ment of the characteristic clinical signs and symptoms of systemic
diastolic collapse. venous congestion-elevated jugular venous pulse with prominent
'y' descent, pulsus paradoxus, low volume pulse, low blood pres-
Diognosis sure, quite pericardium, muffied or distant heart sounds, early
pericardial knock heard at the dme of prominent 'y'' descent.
A high index of suspicion of pericarditis and/or effusion is These signs must be carefi,rlly looked for in those children, with
essential in any child with fever, chest pain, and breathless-
gradually developing pedal edema, minimal puffiness of face and
ness, elevated jugular venous pressure, mild pedal edema,
swelling of eyelids, raised NrI] and unexplained hepatomegaly.
unexplained hepatomegaly, and a pulse of low volume or of
paradoxus, which then needs confirmation by ultrasonography. lnvesligolions
Radiology and electrocardiography may be only suggestive of
Roentgenographic evidence of moderate cardiomegaly is seen
its presence but are not absolutely diagnostic.
The diagnostic confirmation of pericarditis and/or effirsion
in only half of these cases. Calcification of pericardial surface
may also be noted in similar number of cases. Echocardiog-
invites a proper clinical and appropriate laboratory evalua-
raphy may be heipful.
tion to find out its cause, e.g., pyogenic, tuberculous, viral,
rheumatic, etc.
Myocarditis and cardiomyopathy are important differ-
Treolmenl
ential diagnosis that can be excluded by careful history, o Radical pericardiectomy with decortication including the
physical examination, radiology, electrocardiogram, and sheathing over the great veins gives great reiief in a signifi-
echocardiography. cant number of children.
ESSENCE OF PEDIATRICS
o Postoperative cardiac failure is managed by conventional
decongestive measures.
Myocardiris is inflammation, necrosis of the myocardium with
or without associated systemic manifestation or involvement
of the endocardium or pericardium. Most common cause is
viral infection.
ETIOTOGY
o Vinrs: Adenovirus, coxsackie, EB! mumps, Rubella, influenza.
r Bacteria: C. diphtheriae, salmonella, mycoplasma,
meningococcus.
o Parasites: Toxoplasma, echinococcus.
o Fungi: Actinomycosis, histoplasmosis, coccidiodomycosis,
Rickettsiae; coxiella, rocky mountain spotted fever.
o Radiation, chemicals, and drugs: Lead poisoning, emetine,
chloroquine.
CTINICAL FEATURES
Presentation depends upon age and acute or chronic nature
of infection.
In neonate, fever, tachycardia out of proportion to the fever,
weak pulses, gallop rhythm, distant heart sounds, severe heart
failure, respiratory distress, cyanosis, acidosis, and shock. There
may be associated viral hepatitis, aseptic meningitis, and rash'
furhphmias may be the first clinical manifestations, and presence
of fever and large heart strongly suggest acute myocarditis.
INVESTIGATIONS
Elevated ESR and cardiac enzymes (lactate dehydrogenase,
creatine phosphokinase); positive serum viral titers are helpful,
but negative titers do not eliminate the diagnosis. PCR is used
to detect specific virai DNA or RNA.
Chest x-ray reveals enlarged heart with pulmonary edema.
ECG shows sinus tachycardia, low voltage ECG and ST
segment and T wave abnormalities; diphtheretic toxic myo-
carditis is characterized by A-V block, bundle branch block,
or extrasystoles.
Echocardiography demonstrates poor ventricular function and
often a pericardial efF:sion, mitral valve regurgitation and the
absence ofcoronary artery and other congenital heart disease.
Cardiac catheterization for endomyocardial biopsy to
confirm myocarditis.
DI FFERENTIAT DIAGNOSIS
Acute myocarditis is more likely when the child is in good health
prior to the onset of the illness. Differential diagnoses include
pericarditis, endocardial fibroelastosis, dilated cardiomyopathy'
TREATMENT
Therapy is directed towards the management of cardiac failure
and arrhythmia.
Bed rest and other supportive measures (O, inhalation,
diuretics, digoxin, afterload reducing agents, occasional posi-
tive pressure ventilation) are as like as in sevete congestive
heart failure. Digoxin (start with half the normal dose), as
the patients with myocarditis are more susceptible to this
drug. Digoxin should be maintained for several weeks after
recovery from acute attack.
Pericardiocentesis, if cardiac tamponade, and culture of {luid
to detect viral etiology.
Arrhythmia should be treated aggressively, intravenous amio-
darone may be required.
a Extracorporeal membrane orygenation in cardiogenic shock.
a Role of corticosteroid is controversial, found effective in
a few study: prednisolone 2 mglkgld, then tapered to
0.3 mg/kg daily over 3 months. Relapse may occur when
discontinued.
o Cardiac transplantation in patients with refractory heart
failure.
PROGNOSIS
The outcome of the symptomatic neonate with acute viral
myocarditis remains very poor with about 75o/o monality.
Patients with lesser symptoms may have a somewhat better
prognosis, and complete resolution may occur.
The outcome of older patients with chronic dilated car-
diomyopathy associated with prior viral infection is poor
without therapy. These patients continue to have dilatation,
fibrosis, and deterioration of cardiac function.
5070 ofuntreated older patients die within 2 year ofpresenta-
tion and 80% within B year without heart transplantation.
CONGESTIVE CARDIOMYOPATHY
Congestive, or dilated, cardiomyopathy is characterized by
myocardial dysfunction and ventricular dilatation. Although it
usually is a primary disorder, it may be associated with neuro-
muscular disease (e.g., Duchenne muscular dystrophy), beriberi,
or result from drug toxicity (e.g., doxorubicin, daunorubicin).
It may occur following viral myocarditis.
Pothophysiology
Failure of the 1eft ventricle causes an increase in end diastolic
volume, which results in increase in left atrial pulmonary venous
and pulmonary capillary pressures. Mitral valve regurgitation
I
may resuit from papillary muscle dysfunction or from severe 't
dilatation of the valve annulus. \
ft
a
1
a

Clinicql Feqlures
Initially, dyspnea on exertion is present. As left ventricular
failure progresses, small increase in left ventricular volume
occurs, followed by a marked increase in pulmonary capil-
lary pressure. This results in orthopnea, paroxysmal nocturnal
dyspnea, and bronchospasm. Eventually, right heart failure,
characterized by dependent edema, occurs.
Diognosis
Physical examination findings depend on the stage of the disease
but may include tachypnea, tachycardia, a right ventricular
heave, prominent second pulmonary sound, gallop rhythm,
and murmurs of mitral or tricuspid valve regurgitation. In
advanced stages, blood pressure may be low and pulse pressure
narrow; pulsus alternans may be present.
Loborotory Evoluotion
o Chest x-ray rnay show cardiomegaly, an enlarged left atrium,
pulmonary venous congestion, and pleural effusion.
o ECG shows rhythm disturbances, left ventricular hyper-
trophy as well as non-specific ST-segment and T-wave
changes.
o Left ventricular function can be assessed by echocardiog-
raphy, radionuclide studies, and if necessary, cardiac cath-
eterization. Myocardial biopsy may be helpful in defining
the pathologic process.
Treolmenl
o Tleatment is directed at improving left ventricular function
with inotropic agents and at unloading the left ventricle
with vasodilators (digoxin and ACE inhibitors).
o Preload is decreased with diuretics (frusemide), and antiar-
rhythmic medications are used to control potentially fatal
rhythm disturbances.
In the event of clinical deterioration, cardiac transplantation
may be needed.
HYPERTROPHIC CARDIOMYOPATHY
This disorder, also known as idiopathic hypertrophic subaortic
stenosis and hlpertrophic obstructive cardiomyopathy, is an autoso-
mal dominant genetic disorder but can also be sporadic associated
with Freidrech ataxia. The seprum is thickened out of proportion
to the left free ventricular rn'all, which may aiso be thickened.
Pothophysiology
In the thickened, stiff left ventricle, systolic function is well-
preserved, but diastolic function is compromised. Thickening
of the septum may result in left ventricular outflow obstruc-
tion and abnormal motion of the mitral valve. This abnormal
motion may result in mitral regurgitation.
CAR DIOVASCU LAR DISEASES
Clinicql Feotures
Symptoms include dyspnea on exertion, because of an inabiliry
to significantly increase cardiac output with exercise; chest pain,
due to myocardial ischemia; and syncope. Death may result
from rhythm disturbances.
Diognosis
Physical examination: The pulse often is double peaked
because ejection is interrupted by septal obstruction. A forceful
left ventricuiar impulse may be present and an So or S, may
be audible at the apex. Murmurs of left ventricular outfow
obstruction or mitral regurgitation may be heard.
Laboratory evaluation:
o ECG shows left axis deviadon, L\&I, ST depression, Tlinversion.
Rhfh- disturbances are best defined by Holter monitoring.
o Echocardiogram is diagnostic. Measurement of the septum
and the free wall of the left ventricle demonstrates the asym-
metric hypertrophy of the septum. In addition, the small
diastolic left ventricular cavity size can be demonstrated.
Doppler ultrasound and color {low mapping allow evalua-
tion of mitral valve regurgitation and estimation of the left
ventricular outflow gradient.
Treolmenl
o Calcium channel blocking and B-adrenergic blocking agents
(negative inotropic drugs, e.g., propranolol) to prevent fatal
arrhlthmias and to decrease the stiffness of the left ventricle.
Digoxin should be avoided. Amiodarone is helpful in atrial
fibrillation.
o Avoidance of competitive sports because of risk of sudden
death with exertion.
o Ventricular pacing may prove helpful.
Systemic hypertension in children is defined as blood pres-
sure (BP) values >95'h percentile for the corresponding age
and sex. \7hen hypertension is the result of another disease
process, it is referred to as secondary hypertension. \(/hen no
identifiable cause can be found, it is referred to as primary or
essential hJpertension. The Fourth US Thsk Force Report on
Hypertension in children and adoiescents and Indian Society
of Pediatric Nephrology recommend that hypertension be
diagnosed and staged as follows:
o Prehypertension: Systolic or diastolic blood pressure
90th-95th percentile.
. Hy-pertension: Systolic or diastolic blood pressure >95th
percentile.
o Stage I
hypertension: Systolic or diastolic blood pressure
berween 95th percentile and 99th percentile + 5 mmHg'
o Stage II hypertension: Systolic or diastolic blood pres-
sure >99th percentile + 5 mmHg.
ESSENCE OF PEDIATRICS

Blood pressure measurem€nt (auscultatory method, mmHg):
'1-3 mcnths 75+5
4-12 months 84+5
1-B years 95*5
9*14 years 105+5
Blood pressure should be measured in lying or sitting position
comfortably, with the sphygmomanometer at heart level. The
cuffmust cover at least two-thirds of upper arm. Measurement
of blood pressure should be routine part of clinical examina-
tion of all children older than 3 years. See Figures 9.2 and
9.3 for blood pressure percentiles in seated females and males,
respectively.
ETIOTOGY
Conditions Associoled with Tronsienl or
lnlermillent Hyperlension in Children
o Renal: Acute postinfectious glomerulonephritis, ana-
phylactoid (Henoch-Schonlein) purpura with nephritis,
hemolytic-uremic syndrome, acute tubular necrosis,
leukemic infiltration of the kidney, pyelonephritis.
o Drugs and poisons: Syrnpathomimetic agents, amphet-
amines, phencyclidine, oral contraceptives, corticosteroids
and adrenocorticotropic hormone, lead and vitamin D
intoxication.
o Central and autonomic nervous system: Increased intra-
cranial pressure, Guillain-Barr6 syndrome, Stevens-Johnson
syndrome, burns, poliomyelitis, encephalitis.
50t5 o Miscellaneous: Fractures of long bones, hypercalcemia,
65*5 chronic upper airway obstruction.
65+5
65r5 Condilions Associoled with Chronic
Hyperlension in Children
o Renal: Chronic pyelonephritis, chronic glomerulonephritis,
hydronephrosis, congenital dysplastic kidney, multicystic
kidney, solitary renal cyst, vesicoureteral reflu-x nephropathy,
ureteral obstruction, renal tumors, renal trauma, systemic
lupus erythematosus (other connective tissue diseases).
e Vascular: Coarctation of thoracic or abdominal aorta, renal
artery lesions (stenosis, fibromuscular dysplasia, thrombosis,
aneurysm), umbilical artery catheterization with thrombus
formation neurofibromatosis, renal vein thrombosis, vasculitis.
o Endocrine: Hyperthyroidism, hyperparathyroidism, con-
genital adrenal hyperplasia, Cushing syndrome, primary
aldosteronism, pheochromocytoma.
o Central nervous system: Intracranial mass, hemorrhage,
residual following brain injury, quadriplegia.
o Essential hlpertension: Low renin, normal renin, high renin.
CtINICAL FEATURES
Children and adolescents with essential hypertension are
usually asymptomatic; the blood pressure elevation is usually

Females
150 150 95
I
Sy str rlic
95 90
140 90 140 -l
75
l50
I

130 75
I
130

120 I
50
120 l1F
25
o)
I
110 7 , I

10 o 110
I

10
E I
E
E
100 F
E
roo
o 0)
f
a
a
90 590
a
o 4 6
o-
BO
tttl H. 80
E Diastolic E
o ttit o
o 95 o
m BO
90 c0
95
L 75
-i ) =
80 90 90
50
) a I
75

!l4
70 25 90
10 50
5 tq
60 2 70
to
50 90 c'
50
10 12 14 16 18 B 10 1214 1618
Age Age
\
Fig. 9.2t Percentiles of blood pressure in seated females Fig. 9.3: Percentiles of blood pressure in seated males. I
I
a
t
a
 CARDIOVASCU LAR DISEASES

mild and is detected during a routine examination or evalu- INVESTIGATIONS

ation before. Children with secondary hypertension can have
blood pressure elevations ranging from mild to severe. Helpful Screening investigations for patients with hypertension:
features of the physical examination are noted in Table 9.7 . o Complete blood counts
\fith substantial hypertension, headache, dizziness, epistaxis, o Blood urea, creatinine, electrolytes, glucose, uric acid
anorexia, visual changes, and seizures may occur. Hypertensive r Lipid profiie
encephalopathy is suggested by the presence of vomiting, o Urinalysis, culture (if necessary)
temperature elevation, ataxia, stupor, and seizures. o 24-hr urinary protein or spot albumin to creatinine ratio
Chronic hJpertension is often asymptomatic and detected o Chest x-ray
on incidental examination. Headache is an important feature.
o Ultrasonography for kidneys, adrenals
Complications of hypertension include epistaxis, facial paresis,
Screening for target organ damage:
and ocular symptoms, i.e., momentary loss of vision. A rapid
rise of BP may iead to hypertensive encephalopathy (i.e., senso- o Rerinal fundus examination
rial disturbance, seizures), left ventricular failure, pulmonary o Urine spot protein to creatinin ratio
edema. o ECG, echocardiography

Table 9.7: Physical Examination

Ceneral
Pale mucous membranes, edema, growth retardation Chronic renal disease

Webbing of neck, low h airline, widespread nipples. wide t arrying angle Turner ,yndrcrme

Moon {ace, buffalo hump, hirsutism, truncal obesity, striae Cushing syndrome

Habitus
I htnnec( Pheochromocyloma. renal clisease, hvperthyroidism

Virilizalion Congenilal adrenal hyperplasia

Rickers Chronic renal disease

Skin

Caf6 au lait spots, neurofibromas Neurofibromatosis, pheochromoLytoma

Tubers, "ash-lea f" spots luherous s( lerosts

Ra >he: SLE, vasculitis (HSP), impetigo with acute nephritis

Pallor, evanescent flushing, sweatinB Pheoch romocytoma

Bruises, striae Cr.rshing syndrome

Eyes

Fundal changes Non-specific, chronic, severe

Proptosis Hyperlhyroidism

Head and Neck

Coiter Thyroid disease

Cardiovascular signs
t Absent of diminished femoral pulses, low leg pressure relative to arm pressure Aortic coarctation

l' Heart size, rate, rhythm; murmLrrs; respiratory diificulty, hepatomegaly Aortic coarctation, congestive heart failure
)
Pulmonary signs
I Pulmonary edema Congestive heart iailure, acute nephritis
Abdomen
Abdominal masses Wilms tumor, neuroblastoma, pheochromocytoma, polycystic
I'
kidneys. hvdronephrosis

Neurologic signs
I' Neurologic deficits Chronit or severe aLUle hyperlen:ion with stroke
:

Genitalia
Ambiguous, virilized Congenital adrena I hyperplasia
t>
t
II
ESSENCE OF PEDIATRICS

Table 9.81 Diagnostic Tests for Sustained Hypertension Table 9.9: Doses and Routes of Administration of Antihyper-
tensive Drugs

Clomerulonephritis Complement, autoantibodies, renal biopsy

Reflux nephropathy Micturating cystourethroBranr. DMSA scintigraphy Propranolol 0.01 0.1 mg/kg/ IV slow 6-8 hr
dose push
Renovascular Doppler{lowstudies,captoprilrenography,
hypertension angiographv Atenolol 1-2 mg/kg/2a hr PO 24 hr

Coarctation of aorta Er hotardiography. angiographv Captopril Neonates <2 mo: PO 8-24 hr

0.05 0.1 mg/kg/dose
Endocrine cause. Plasma renin ar lir ity. aldoslerone; plasma, and r-rp to 0.5 m/kg/dose
urinary cortisoli plasma, urine catecholamines;
MIBC scan; CT/MR imaging; thyroid functions Enalapri I Children:0.1-0.5 rng/ PO 12-24 hr
kg/24 hr
Nifedipirre Children: 0.25-0.5 mg/ PO, SL Repeat

See Thble 9.8 for list of diagnostic tests for sustained hyper-
5
kg/dose, max q4-6hr
Hydrochloroth iazide 1-2 mg/k!24hr PO 12-24hr
tension.
mal200 mg24hr

MANAGEMENT
The primary classes of useful antihypertensive drugs are
(l) diuretics (hydrochlorothiazides, frusemide), (ii) B-adrenergic
blockers (propranolol, atenolol, labetalol), (iii) ACE inhibi-
tors (captopril, lisinopril, enalapril), (iu) Ca. channel blockers
(nifedipine, verapamil), (zr) vasodilators (hydralazine, diazoxide,
nitroprusside, and minoxidil), and (zi) sympatholytic agents
(ct-methyldopa).
Stepwise Treolment of Hyperlension
Essential hypertension: Changing lifesryle, receiving diet low
in salt and animal proteins, reduction in weight (5-10 mmHg
reduction ofsystolic pressure), ensuring regular exercise as well
as periods of relaxation mai' be effective and do not require
medications. Children with essential hypertension whose blood
pressure recordings are persistently above the 99'h percentile
should be treated.
Therapywith antihypertensive agents is required for patients
with (z) stage II hypertension, (ii) stage I or II hypertension
with evidence of end organ damage, or (iii) sustained stage
I hypertension that is not controlled despite 6-months of
lifestyle modification, including exercise and weight reduction.
Principles of antihypertensive therapy include:
o Start withACE inhibitors (e.g., enalapril, lisinopril, ramipril).
o If b rod pressure is not controlled, add a thiazide
ic.
diurt
r high cardiac output physiology respond best to B-blockers.
If bl< ,d pressure is still not controlled, add a calcium channel
blocl :r (e.g., amlodipine)
o blocker may be added to the diuretic, and an ACE inhibitor
Fina y consider addition ofa beta-blocker (atenolol, carve-
dilol, labetalol)
e If ACE inhibitors are associated with cough, shift to angio-
tensin receptor blockers (losartan)
Table 9.9 lists doses and routes of administration of some
antihypertensive drugs.
In selecting a drug regimen for long-term use, an
understanding of the underlying pathophysiology is helpful.
Drugs with different sites and mechanisms of action are
available so that therapy can be tailored to the specific
pathologic condition. For example, excessive activity of the
renin-angiotensin-aldosterone system may be treated effectively
with a B-biocking drug (propranolol) for suppression of
lenin secretion, an ACE inhibitor (captopril or enalapril),
or, rarely, an aldosterone antagonist (e.g., spironolactone).
ACE inhibitors are useful, not only in patients with high-
renin hypertension that is secondary to renovascular or renal
parenchvmal disease but also in patients with high-renin
essential hypertension. Excess angiotensin production is the
probable cause of most hypertension in neonates after partial
occlusion of a renal vessel by thrombus. Captopril or enalapril
are efrective agents in most of these patients, but must be used
with careful attention to renal function. u-Adrenergic blocking
agents (phentolamine, phenoxybenzamine) are beneficial in
patients with neural crest tumors who have high circulating
ievels of catecholamines. In such patients, B-blocking drugs
are also needed to control the heart rate, or an agent with
dual blocking action (labetalol) may be used. Sympathetic
blockade with labetalol is likewise e{icacious in patients who
experience marked stimulation of the cardiovascular system
Flom high doses oFcocaine.
Young patients with essential hypertension who require drug
therapy may be treated initially with a diuretic or a B-blocking
agent. Patients with volume-dependent hypertension usually
have an adequate response to diuretics; those with high-renin,
If the pressure is not lowered adequately, a calcium channel
may replace the B-blocker. Chronic use of diuretics mav result
in elevation of serum lipids, which may increase the risk of
ischemic heart disease in adults witl"r hypertension. L,ong-term
investigations of this side effect in children are not available.
p-Blocking agents have also been associated rvith changes in
serum lipids, and son-re studies suggest a mild reduction in
exercise tolerance in patients treated rvith propranolol. Patients
with reactive airway disease are often not able to tolerate a
B-blocking agent.
 CARDIOVASC U LAR DISEASES

Becauseof these side effects, ACE inhibitors and calcium Congestive heart failure is a biventricular heart failure;
channel blockers may be considered for initial therapy in an physical manifestations are a combination of both right- and
adolescent with significant hypertension. Although captopril left-sided heart failure.
has been used more often in young patients, enalapril has
a longer duration of action and thus requires less frequent ETIOTOGY
administration.
Fetal
Treolment of Hypertensive Emergency r Severe anemia (hemolysis, fetal-maternal transfusion)
A hypertensive emergency exists when central nervous system r Supraventricular tachycardia
signs of hypertension appear, such as papilledema or encepha- o Ventricular tachycardia
lopathy. Retinal hemorrhages or exudates indicate a need for o Complete heart block
prompt and efFective control. Because too rapid a reduction in o Premature neonate
blood pressure may interfere with adequate organ perfusion, a r Fluid overload
stepwise reduction in pressure should be planned. In general, r Patent ductus arteriosus
the pressure should be reduced by about one-third ofthe total o Ventricular septal defects
planned reduction during the first 6 hours and the remaining Full-term neonate
over the following 48-72 hours.
o Arteriovenous malformation (vein of Galen, hepatic)
o Drugs of choice: Intravenous labetalol or sodium nitroprus- e Left-sided obstructive lesions (coarctation of aorta, hyp-
side or sublinguai nifedipine. oplastic left side of the heart)
o Other agents: Esmolol, nicardipine, furosemide o Large mixing cardiac defects (single ventricle, truncus arte-
Labetalol (0.2-1.0 mg/kg/dose, max 20 mg dose intravenously) riosus)
blocks both u- and B-adrenergic receptors; with a single dose o Viral myocarditis
followed by continuous infusion, controlied reduction of blood Infant-toddler
pressure can be achieved. Similar control is possibie with an
infusion of nitroprusside (0.3-0.5 pg/kg/min, intravenous
r Left-to-right cardiac shunts (ventricular septal defect)
route). Nifedipine (0.25-0.5 mg/kg/dose, max 5 mg/dose)
o Hemangioma (arteriovenous malformation)
has a rapid onset of action, but its short duration of action
o Acute hypertension (hemolltic uremic syndrome)
must be anticipated. Because nifedipine is available only as a
o Supraventricular tachycardia
iiquid within a capsule, administration to younger children
o Total anomalous pulmonary venous return
has presented some dificulty. Although the drug has often
e Kawasaki disease
been placed in the sublingual space to achieve rapid absorp-
o Metabolic cardiomyopathy
tion, gastrointestinal absorption is also sufficiently rapid to be Child-adolescent
effective in a hypertensive crisis. o Rheumatic fever
Most children with hypertensive crisis have chronic or acute o Acute hypertension (glomerulonephritis)
renal disease; in these patients, management of blood pressure o Viral myocarditis
also requires careful attention to fluid balance, as well as diure- o Endocarditis
sis. Intravenous furosemide (1 mg/kg/dose) is usually effective, o Severe anemia
even though glomerular filtration may be impaired. o Hemochromatosis-hemosiderosis
o Cor pulmonale (cystic fibrosis)
o Cardiomyopathy (hypertrophic, dilated)
See Figure .4 for usual age of presentation of disorders causing
9
Heart failure is defined as a state in which heart cannot deliver
heart failure.
on adequate cardiac output to meet the metabolic needs of the
body. In the early stage of heart failure, variable compensatory
mechanisms (activation of the sympathetic nervous system,
CTINICAL FEATURES
activation of renin-angiotensin-aldosterone system, release of
Cqrdinql Signs of CHF
antidiuretic hormone) are evoked to maintain normal meta-
bolic function. As these mechanisms become ineffective' severe o Tachypnea: A respiratory rate >60/min in infants and
clinical manifestations results. >40lmin in older children is a significant finding. It mostly
Cardiac output can be calculated as the product of heart indicates left heart failure. It may be present for a short
rate and stroke volume (HR x SV). Primary determinants time before hepatomegaly occurs, although pure left-sided
of stroke volume include afterload (pressure work), preload or pure right-sided heart failure does not commonly exist
(volume work), and myocardial contractility. independently for long.

ESSENCE OF PEDIATRICS
I Hypoplastic left heart
I Severe aortic stenosis
Coarctation of aorta
Truncusarteriosus
Total anomalous pulmonary venous drainage
- Supraventricular tachycardia
- Endocardialfibroelastosis
- Myocarditis
- Cardiomyopathy
Birth 3mo 6mo
Fig. 9.4: Usual age of presentation of disorders causing heart failure.
o Tachycardia: A
heart rate >180/min in infant and
>150/min in older children is- a valuable sign.
Hepatomegaly: It is the cardinal sign of right-sided heart
failure. The liver is capable of trapping relatively large
arnount of edema fluid in infant that would be more evident
as peripheral edema in older child and adult. Liver size
provides an indication of the severiry of the heart failure.
Cardiomegaly: Cardiomegaly is invariably present in con-
gestive heart failure. One should be cautious about the
diagnosis of congestive heart failure in the absence of an
enlarged heart. Cardiomegaly without orher signs of conges-
tive failure may well be taken as early or homeostatically
compensated congesrive heart failure.
Though the above menrioned four cardinal signs are the main-
stay of heart failure, the overall presenrarion differs in infants
and older chiidren.
ln lnfonts
Heart failure may be more difficult to identify. Main features
include tachypnea, feeding difficulties (takes less volume per
feeding, becomes dyspneic while sucking, and may perspire).
Poor weight gain, labored respirations with chest indrawing
as well as flaring of the alae nasi. These may be indistinguish-
able from those of bronchiolitis; wheezing is mosr prominent
finding. Pneumonitis with or without atelactasis is common,
especially of the right, middle, and lower lobes; it is due to
bronchial compression by the enlarged heart. Hepatomegaly
usually occurs and cardiomegaly is invariably present; galiop
rhythm can be recognized. Other auscultatory signs are those
produced by underlying cardiac lesion. Assessment of JVP is
dificult because of the shortness of the neck. Edema may be
generalized, usually involving the eyelids as well as the sacrum
and less often the legs and feet.
ln Children
Signs and symproms are similar ro rhose in adulrs. Patient may
have cough and dyspnea. Dyspnea is a reflection of pulmonary
congestion. Orthopnea and basilar rales may be present. Depen-
dent edema or anasarca, increased JVP, hepatomegaly may be
I
Patent ductus arteriosus
Ventricular septal defect
Atrioventricular canal defect
lyr 5yr 10yr
-
present. Cardiomegaly is invariably noted. A gallop rhythm is
common. Other auscultatory findings are rhose produced by
the underlying cardiac lesion.
INVESTIGATIONS
A single diagnosis of heart failure is an incomplete diagnosis,
and a cause should be determined.
X-ray chest: A chest x-ray almosr shows cardiomegaly. Fluffy
peripheral pulmonary markings suggesrive of venous conges-
tion and acute pulmonary edema are seen in severe degree of
heart failure.
ECG: It is helpful in assessing the chamber hypertrophy as
the cause of heart failure but does not establish the diagnosis.
ECG is the best tool for evaluating dysrhythmias as a porenrial
cause of heart failure.
Echocardiography: This is done to evaluare vah'ular disease and
ventricular function and also to diagnose congenital heart diseases.
Doppler studies can be used to calculate cardiac ourpur.
Blood gas analysis: Arterial oxygen level may be decreased
when ventilarion/perfusion inequalities occur secondary to
pulmonary edema. \7hen hearr failure is severe, respiratory
and/or metabolic acidosis may occur.
TREATMENT
Generol Meosures: Decreqse Physicol Activity
Strict bed rest is rarely necessary except in extreme cases, but
:
it is importanr rhat the child take rest and sleep adequately.
Patient may be propped up; infant may be on infant chair. :
After patient begins to respond ro rrearmenr, acrivities can be
modi6ed. For patients with pulmonary edema, 40o/o oxygen
should be given; positive pressure ventilarion may be required l
along with other drug therapy.
Diet 1
Increasing the number of calories per ounce of infant formula \
(or supplementing breast-feeding) may be beneficial. The use of l,
 CARDIOVASCU LAR DISEASES

low sodium formuias in the routine management of infants with
heart failure is not recommended, because these preparations are
often poorly tolerated and may exacerbate diuretic-induced hypo-
natremia. Most older children can be managed with "no added
salt" diets and abstinence from foods containing large amounts of
sodium. Oral feeding can be allowed if the patient can take orally.
Severely ill infant (in face of extreme fatigue, rapid respirations,
and generalized weakness) need NG tube feeding.
Digitolis
Digoxin is the digitalis glycoside used most often in pediatric
patients. In order of onset of effect, it may be used IV IM, or
PO (Table 9.10). Digoxin is eliminated by the kidney.
Digitalization
A. Routine digitalization PO within 24 hr: Half of total
digitalizing dose (TDD) should be given initially, followed
by one-fourth of TDD every 8-12 hour for 2 doses, then
Maintenance-one-fourth of TDD (or 5-10 prg/kg/d)
divided 12 hourly (n.rorning and evening), starting
12 hour after full digitalization. See Table 9.11 for
calculation of total digitalizing dose.
B. Rapid digitalization: Rapid digitalization of infants and
children in heart failure may be carried out intravenously.
The dose depends on the patient's age (see Table 9.12).
Steps of digitalization:
1. Base-line ECG (rhythm and P-R interval), S. electro-
lyte, and S. calcium 1evel.
2. Calculation of TDD:
o 50o/o TDD immediately (1st dose),
o After 12 hr,25o/o TDD (2nd dose),
o After 12hr,25o/o TDD (3rd dose).
3. Maintenance dose: 12 hr after finalTDD; ECG before
For patients who are initially given digitalis IV mainte-
nance digoxin can be given orally once oral feeding is
tolerated. Because absorption fi'om the GI tract is less
certain, the oral maintenance dose is usually 20-25o/o
higher than when digoxin is used IV \7hen the child is
in acute congestive heart failure (criticallv i' )' IV digoxin
is given.
C. Slow digitalization: In a patient with cL rnic conges-
tive heart failure who does not to be hor ritalized, full
digitalization can be achieved within 7-1 days on an
outpatient basis. Give the maintenance do : of digoxin,
i.e., one-fourth of TDD div. in morning and evening
doses witirout prior loading dose (slow dig talization).
If an infant improves significantly when rec :iving digoxin
over a period of few months and the need for * : drug appears
to be lessening (i.e., a VSD becoming smaller the dosage is
not ir-rcreased as the child gains weight. If thc clinical status
permits, the drug is discontinued.
Hypokalemia, hypomagnesemia, hyperc icemia, myo-
carditis, and prematurity may potentiate digitalis toxiciry.
Any form of arrhythmia occurring after institution cf
digitalis therapy must be considered to be drug related
until proved other wise . Succeeding doses should be with-
held until the issue is resolved (digoxin toxicity mav be
assessed clinicaily, by doing ECG, or by measuring serum
digoxin level).
Digoxin Toxicity
. Extracardiac manifestations:
'l Anorexia, nausea, vomiting, diarrhea
r Altered color vision
o Cardiac manifestations:
,r Bradycardia: The following rates are often taken as a

starting maintenance dose. guide to digoxirr toxicity:

-
Infants - below 100/min.
Young children - below 80/min.
Table 9.10: Pharmacokinetic features of Digoxtn - Older children - below 60/min.
Half life 36 hours -
> Dysrhythmias: Multiple ventricular ectopics, ventricu-
Onset of aclion
lar bigeminy, paroxysmal atrial tachycardia, ventricular
lntravenous 15-30 min tachycardia, ventricular fi brillation.
Orallv 30 nrin
o ECG changes with digoxin toxicity:
Peak action

lntravenous 1-4 hours

r Prolongation of PR interval
r Profound sinus bradycardia or sinoatrial block
Orally 2-6 hor-rrs

Table 9.12: Digoxin Dosage (Note: These doses are PO; IV

Table 9.11: Calculation of Total Digitalizing Dose (TDD) dose is 75"1' oi PO dose)
Digitalization Prenrature:20prg/kg
I Premature 0.02 mg/kg
(1/zinitially, Full-term neonate (up to 1 mo): 0.02-0.03 mg/kg
fotlowed by lnfant or child: 0.025-0.04 mg/kg
t Neonate 0.02-0.0 t mg/kg 1/n q12hr x 2) Adolescent or adult: 0.5*1 mg in divided doses
l' lnfant or child 0.04-0.00 mg/kg Mainlenance 5-t0 p{k{d, divided q12hr
tt Adolescent
(Roughly <2 yr 0.o4
0.5
m{kg; >2 yr 0.04-0.06
1.0 mg in divided doses
mg/kg) .1
Trough serum level: .5-3'0 ng/ml <6 mo old;
1-2 n{mL >6 mo old

t
a
?
I'
I
 ESSENCE OF PEDIATRICS
o Supraventriculararrhythmias
c Ventriculararrhythmias
Tieatment of digoxin toxicity:
r Stop digoxin; srarr continuous ECG monitoring.
r Check urea, elecrrolltes, and plasma digoxin level.
o Correct hypokalemia and/or dehydration.
r Correcr bradycardia by using atropine and/or pacing.
o Tieat atrial tachycardia with beta-blocker.
e Tieat ventricular tachycardia with lignocaine.
e Use antidote digoxin immune fab.
Diurelics
These agents interfere with reabsorption of water and sodium
by kidneys that results in reduction of circulating blood volume
and thereby reduces pulmonary fluid overload and ventricular
filling pressure. Used in conjunction with digitalis therapy in
severe CHF.
Furosemide: It inhibits reabsorption of sodium and chloride
in the distal tubule and loop of Henle:
a) For acute diuresis: 1_2 mglkgldose IV or IM. This
dose may be repeated 2-3 times daily while monitored
in hospital, especially serum electrolltes.
b) Slow or chronic diuresis or maintenance diuretic therapy:
l-4 mglkgld given orally berween I and 4 times a day.
Careful monitoring of electrolytes is necessary. \fhen
given daily, potassium chloride supplementation is nec-
essary,but when administered every other day dietary
potassium supplementarion may be adequate (fruit,
coconut water, etc.) to maintain normal serum potassium
level. V4ren a potassium sparing diuretic (spironolactone)
is given concomitantly with furosemide, hypokalemia
does not occur and potassium supplementation is not
needed.
Spironolactone: It is an inhibitor of aldosterone that
enhances potassium retention. It is commonly used in
combination with chlorothiazide ro eliminate need
for potassium supplement. It is given orally in a dose of
2-3 mglkgld in 2-3 divided doses.
Aflerlood Reducing Agenls ond
ACE lnhibitors
Afterload reducing agents reduce ventricular afterload by
decreasing peripheral vascular resistance (vasodilatation) thereby
improving myocardial performance. Some of these agents aiso
decreasepreload by decreasing systemic venous tone. Afterload
reducers are especially useful in children with heart failure sec-
ondary to cardiomyopathy and in patients with severe mitral
or aortic insufficiency. They may also be effective in patients
with heart failure caused by left-to-right shunts. They are not
generally used in the presence of stenotic lesions of the left
ventricular outflow tract.
ACE inhibitors may have additional beneficial effects on
cardiac remodeling independent of their influence on afterload.
In adult parienrs with dilated cardiomyopathy, the addition
of an ACE inhibitor to standard medical therapy reduces
both morbidiry and morraliry. They are most often used in
conjunction with other anticongestive drugs such as digoxin
and diuretics.
Nitroprusside This peripheral arterial, and to some extent
venous, vasodilator should be administered only in intensive
care setting and in critically ill patients. It is used IV in a dose
of 0.5-8 pg/kg/min while continuous monitoring of blood
pressure is done during and after administration.
Hydralazine: This direct arteriolar smoorh muscle relaxanr
is used usually rogether with a venodilating agent like nitrite
derivative. The IV or IM dose is 0.1-0.5 mg/kg/dose and
the oral dose is 0.5-7.5 mg/kg/d in 3 divided doses. Adverse
reactions are vomiting, headache, palpitation, and SLE after
prolonged use in a large dose. These are reversible when the
drug is stopped.
Captopril: This ACE inhibitor produces marked arterial
dilatation by blocking the production of angiotensin II. It
may cause venodilatation and reduce preload. It also helps
control of salt and warer rerenrion by interfering aldosterone
production. The oral dose is 0.3-G mglkgl24 hr in 2 or 3
divided doses. Hypotension, maculopapular pruritic rash,
neutropenia, chronic cough, and renal toxicity may occur
as adverse reaction.
Enalapril: It
is a longer actingACE inhibitor. Doses: 0.08-0.5
mg/kg/dose daily or rwice a day.
lnotropic Agenls (Olher thon Digitolis)
The positive inotropic agenrs orher than digitalis are B-adrener-
gic agonists and phosphodiesterase inhibitors.
q- and
$-Adrenergic Agonists
These drugs are usually administered in an intensive care serring,
where the dose can be carefully titrated to hemodynamic
response. Though extremely efficacious in the acute intensive
care setting, some evidence indicates that long-term admin-
istration of adrenergic agonists may in certain cases increase
morbidity and mortality in patients with heart failure.
Dopamine It is a predominantly B-adrenergic receptor agonist,
but it has cr-adrenergic effects at higher doses. It is usefui par-
ticularly in patients with compromised renal function. At a dose
of 2-10 pg/kg/min I{ it increases cardiac contractiliry with :
little peripheral vasoconsrrictive effect. If the dose is increased
beyond 15 pg/kg/min, however, its peripheral cr-adrenergic :
effects may result in vasoconstriction and cause an increase in I
pulmonan' vascular resistance. 1
Doburqmine: It is used to treat low cardiac output and may t
be used as an adjunct to dopamine therapy to avoid the t
t
I
 CARDIOVASCU LAR DISEASES

I
F vasoconstrictive effects of high-dose dopamine. The usual IV
I dose is 2-20 p,glkglnin.
t
Isoproterenol: It is a pure B-adrenergic agonist. It enhances Electrocardiography (ECG) is a recording of the electricai
I) changes that occur within the heart during the cardiac cycle
myocardial contractility by both central and peripheral
r B-adrenergic effect and also reduces cardiac afterload. Admin-
from the body surface.
I
istered intravenously in intensive care setting; the IV dose is
a
titrated between 0.01 and 1.5 pg/kg/min. l. Limb leads:
]'
Epinephrine: It is a mixed a- and B-adrenergic receptor
, agonist that is usually reserved for patients with cardiogenic
t- Bipolar or standard limb leads
shock and low arterial blood pressure. Although epinephrine Lead Right arm*Left arm
, Leacl ll
I

Right arrn-Left leg

l' can raise blood pressure effectively, it
also increases systemic
I Lead lll Left arm-Left leg
vascular resistance and therefore increases the afterload against
Unipolar or augmented limb leads
which the heart has to work. aVR Right arm
aVl Left arm
aVF I eft foot
Phosphodiesterase lnhibitors
Milrinone It is used in patients with low cardiac output who
are refractory to standard therapy. It has both positive inotro- 2. Chest leads:
pic effects on the heart and significant peripheral vasodilatory
effects and has generally been used as an adjunct to dopamine
4'h intercostal space, right sternal border
or dobutamine therapy in the intensive care unit. It is given
4' inlerroslal spate. left sternal border
by intravenous infusion at 0.25-I pg/kg/min, sometimes with
Midway between V and V
an initial loading dose of 50 pg/kg.
Midclavicular line in the leit 5h intercostal space
Amrinone: Another phosphodiesterase inhibitot can cause Anterior axillary line, horizontal lo V,
thrombocytopenia; the dose is 3-10 pg/kg/min.
Midaxillary line horizontal to V,-V
The extra two chest leads used in pediatric ECC are:
Chronic Treatment with p-Blockers V,R Same as Vr but on the right side
Studies in
adults with dilated cardiomyopathy show that VuR Same as V, but on the riBht side
B-adrenergic blocking agents, introduced gradually as part
of a comprehensive heart failure treatment program, improve
exercise tolerance, decrease hospitalizations, and reduce overall NORMAT PQRST WAVES
mortality. The agents most often used are metoprolol (a
F,-adrenergic receptor selective antagonist) and carvedilol (an One cardiac cycle is represented by successive wave forms on
agent with both a- and B-adrenergic receptor blocking as well an electrocardiographic tracing-the P wave, QRS complex,
as free radical scavenging effects). B-blockers are used for the and the T wave. These waves produce cwo important intervals
chronic treatment of patients with heart failure and should (PR and QT) and rwo segments (PQ and ST) (Fig. 9.5a).
not be administered when patients are still in the acute phase In normal sinus rhlthm, the sinoatrial (SA) node is the pace-
of heart failure (i.e., receiving intravenous adrenergic agonist maker for the entire heart; the SA node impulse depolarizes the
infusions). right and left atria by contiguous spread, producing the P wave.
\X4ren the atrial impulse arrives at the atrioventricular (AV] node,
Discover ond Treol the Underlying Cquse it passes through the node at a much slower velociry than any
other part of the heart, producing the PQ interval. Once the
o If the cause is a congenital cardiac anomaly or rheumatic electrical impulse reaches the bundle of His, the conduction veloc-
heart disease, prepare the patient for surgical treatment after
ity becomes very fast and spreads simultaneously down the left
control of heart faiiure.
and right bundie branches to the ventricular muscle, through the
o If it is secondary to sepsis, anemia, asphlxia, these should
Purkinje fibers, producing the QRS complex. The repolarization
be treated along with the treatment of heart failure.
of the ventricle produces the T wave, but the repolarization of
o If it is following rheumatic carditis or viral myocarditis,
the atria is not usually visible on the ECG tracing (Fig. 9.5b).
prednisolone should be added.
o If it is a cardiomyopathy, medical treatment will provide Systemic approach
temporary relief of symptoms and should have continuous The following sequence can be followed in reading ECG:
treatment to allow time to wait for cardiac transplantation 1. Rhythm by considering the P axis
when indicated and a heart donor is available. 2. Heart rate
ESSENCE OF PEDIATRICS

SA node

AV node
Bundle of His

Left bundle branch

Right bundle branch

Purkinje fibers

Fig. 9.5: Definition of electrocardiographic configuration (a), and diagrammatic representation of conduction system of the heart (b)

3. The QRS axis

4. Intervals: PR, QRS and QT
5. The P wave amplitude and duration
6. amplitude and the R/S ratio and abnormal Q
*;]*
7. ST segment and T wave abnormalities

EVALUATION OF WAVES-DU RATIONS

AND INTERVATS
P duration is measured from the onset of the P wave and up
to the end. The mean P amplitude in lead II or any other lead
is maximum of 3.0 mm. Thli P waves (>3 mm) are an indica-
tion of RAH or P-pulmonale (since it is associated with cor
pulmonale). The maximum P duration is 0.10 sec. Prolongation
of P wave duration (wide and often notched) is seen in LAH
or P-mitrale (since it is associated with mitral stenosis).
Deflections of lhe Electrocordiogrom
Depolarization
of ventricles
Fig9.7: Electrophysiologic significance of a cardiac cycle.
The P Axis
The same methods used to determine the QRS axis can be used
for the P axis, the only difrerence being that one looks for a
lead with flat P if equiphasic waves are not found.
The P axis represents the direction ofthe atrial depoiarization
vector and therefore gives information about the pacemaker site
and the location of the SA node. Accordingly, it is important
in the interpretation of abnormal rhythm and chamber local-
ization. Two examples of P-axis determination are as follows.

lsoelectric line
Example 1
il "'

lt
tt
tt
II
r<-)
QRS
duration
ffiffiffi aVL aVF

Fig 9.6: Measurement from the electrocardiogram. Note that the

P-R interval is measured from the start of the P wave to the start
of the QRS complex, the Q-T interval from the start of QRS to
the end of T. ffiffiffi {
1
!
t
1
I
ta
r
*.

l"
t,

r CARDIOVASC U LAR DIS EASES

t
r
I) Since the P waves are positive in leads I and aVF, the It is preferable to measure the Q wave duration in lead II or in
v
P axis is in the left lower quadrant (0 to +90 degrees). In most leads with clearlyvisible Qwaves. The average QRS durations are:
t instances, that is enough evidence to say there is sinus rh1'thm, Premature infants 0.04 sec
II i.e., the SA node is the pacemaker. (In sinus rhlthm, the P axis Full-term infants 0.05 sec
t" is directed anteriorly as well. This is shown as a positive P wave Children I-3 yr 0.06 sec
I in V, and Vr).T. be more precise, we find a flat P wave in a\{L; Children >3 yr 0.07 sec
),
therefore, it is perpendicular to a\{L, and the P axis is + 60 degrees. Adults 0.08 sec
v
Example 2 il Abnormally wide QRS duration occurs in Bundle Branch
Block (right or left), 'Wolff-Parkinson-\7hite syndrome,
t

, arrhythmia of ventricular origin, hyperkalemia, ventricular

ffiffiffi
hypertrophy.
)-
The most important diagnostic criteria of ventricular hyper-
I trophy are abnormally large QRS voltages in the leads repre-
I senting the respective ventricles.
I
t Low-voltage QRS complexes (deflections <5 mm) occur in
I aVR myocarditis, pericardial effusion, chronic constrictive pericardi-
V tis, hypothyroidism, thick chest wall, normal newborn infants.
f,

ffiffiffi
I
I') QRS Axis (Fig.9.8)
The electrical axis of the heart is the direction of the maximum
v electrical force during depolarization. Its approximate value can
, be derived from the QRS complexes in tr,vo or more leads. The
L The negative P in lead I and the Positive P in aVF place usual method is to employ leads 1 and 2, but an easier method
I the P a-xis in the right lower quadrant (+90 to +180 degrees). uses lead 1 and aVF (see below). The range of QRS axis is
I The P is almost flat (although slightly positive) in aVR. There- wide. The mean QRS axis according to the age is as follows:
fore, the P axis is +120 degrees or slightly greater. This is an Newborn + 125 degrees
abnormal P axis. This patient has mirror-image dextrocardia. lmo + 90 degrees
3yt + 60 degrees
PR lnterval Adults + 50 degrees

PR interval is measured from the onset of the P wave to the
beginning of the QRS complex and therefore is sometimes
called PQ interval. The PR interval is ordinarily measured in
lead iI or other leads with visible Q waves. In certain leads
that are perpendicular to the direction ofseptal depolarization
(which produces a Q wave), the Q wave may be isoelectric
or absent.
The normal PR interval varies with age and heart rate.
Upper limit of normal PR interval is 0.16-0.18 sec and lower
limit is 0.08-0.1 sec.
Left axis deviation occurs in ostium primum defect, LVH
particularly with volume overload, left bundle branch block
(LBBB).
Right axis deviation occurs in ostium secundum defect,
RVH, RBBB.
Superiorly oriented axis (superior axis) is present when the
S wave is greater than R wave in aVF. It occurs in left ante-
rior hemiblock particularly with endocardial cushion defect,
tricuspid atresia, RBBB.

PR prolongation occurs in: R and S Waves

o First-degree A-V block. The sizes of the R and S waves in the various leads of the elec-
o Myocarditis: Rheumatic, viral, or diphtheritic. trocardiogram are determined by the thickness of the ventricular
o Congenital heart defects (endocardial cushion defect, ASD, wall. Detection of early right or 1eft ventricular hypertrophy
Ebstein anomaly) is accomplished by recognizing that these voltages lie outside
o Digitalis toxicity. the normal range for the patientt age (Table 9.13).
o Hyperkalemia.
r Ischemia or profound hypoxia. QWave
Th. Q wave is produced primarily by depolarization of the
QRS Duration ventricular septum. A Q wave is commonly present in leads
QRS duration is measured from the onset of the Q wave (or 1,2, 3 and a\G and almost always present in V, and V.. The
R wave if no Q wave is visible) to the termination of S wave. Q wave is absent in V,.
ESSENCE OF PEDIATRICS

Table 9.13: Normal Voltages (mm = 1/1 0 mv) in Precordial Abnormal R/S ratios:
Leads
fuS ratio >ULN in RPLs suggests RVH
<LLN in RPLs suggests LVH
R/S ratio >ULN in LPLs suggests LVH
Birth 12 $ 2A) 1 (0*20)
0 3)
s (1-1 6 (0-1 5) <LLN in LPLs suggests RVH
6 monlhs 11 (3-17) 10 {l-25r 14 t5-25t 1r0-l0r
Abnormal R/S ratios may also be seen in ventricular conduc-
I year 9 Q-16) I0rl-l2r 1415 25r 2rO-7t
tion disturbances and myocardial infarction. See Table 9.14
10 years 5 (1-12) 1o (1-2s) 16 (5-30) 2 (0-s) for R/S ratios with respecr ro age.
Figures in parentheses indicate the normal range.

The maximal Qwave ampliude in leads aVE V5, and Vu is
usually <5 mm in children of any age. The maximal Q wave
amplitude in lead 3 may be as large as 5-8 mm in children.
The average Q wave duration is 0.02 sec and normally does
not exceed 0.03 sec.
Deep (but not wide) Q wave occurs in LVH of volume
overload (VSD, single ventricle), combined ventricular hyper-
trophy (VSD, pulmonary hypertension), cardiomyopathy.
Deep and wide Q wave occurs in myocardial infarction,
myocardial fibrosis.
ST Segment
The ST segment occurs after ventricular depolarization (the
QRS complex), and before ventricular repolarization (the
T wave). The normal ST segment is horizontal and isoelectric
(at the same level as the PQ and TP segments). In the limb
leads, elevation or depression of the ST segment up to I mm is
not necessarily abnormal. A shift of up to 2 mm is considered
normal in the left precordial leads. No data are available on the
normal ST segment duration, but an abnormally prolonged ST
segment will result in prolongation of QT interval, for which
normal values have been established.

ffi
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Axis of lead
i1 80"

a\
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30'
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-L- >,t .O-r
64; +120' +60"
+90' $o(s
+90"

(b) (c)

Fig.9.B: (a) Calculation of the QRS axis (in the frontal plane). (b) Using leads 1 and 2. Measure the height of R in lead 1 (2 mm) and
subtract the depth of S (B mm). Plot the answer (-6) along the axis of lead 1, which is horizontal (O degrees) in arbitrary units. In lead
2,4-2-9-3=+6,whichisplottedontheaxisoflead2,whichisat60degrees.Dropperpendicularsfromtheseaxes,andthe
line from the origin through the intersection is the electrical axis. (c) The same calculation, using aVF instead of lead 2. This has the
advantagethatitisatright-anglestoleadl,buttheresultofR-5(10-3=7) hastobemultipliedbyafactor, l.3,toallowforthe
fact that aVF is an augmented unipolar lead and not, like lead 1, a bipolar lead. A rough method is to look for a lead were R and S
are nearly equal, in this case aVR. The axis will be at right-angles to the axis of that lead, and it will be clear from examining one
other lead in which direction it points.
 I

CARDIOVASCU LAR DISEASES

Table 9.14: R/S Ratio According to Age: Mean, Lower, and both ventricular depolarization (QRS duration) and ventricular
Upper Limits of Normal repolarization (to the end of the T wave). Th. QT interval
varies with heart rate but not with age, except in infancy.
Therefore, the QT interval must be interpreted in relation to
LLN 0.s 0.3 0.3 0.5 0.1 0.1s 0.1 0.0 the heart rate (corrected QT interval, QT.). One may use the
Mean 1.5 1.5 L2 0.8 0.65 0.5 0.1 0.J Bazett formula:
ULN 19 S=0 6 4 2 1 1 1
: Qt/u**
Bazett formula: QTc
tLN 0.3 0.3 0.3 0.3 0.05 0.1 0..1 0.1
Mean 1 1.2 1 0.8 0.5 0.5 0.5 O.2 According to Bazett formula, the QTc should not exceed
1.5 1.5 1.2 1.2 2.5 0.44 second, except in infants. The QTc of up to 0.49 second
ULN 3 4 4
may be normal for the first 6 months of life.
LLN 0.1 1.5 2 3 2.5 4 2.5 2.s
U tuaues are small, positive waves that occur toward the end
Mean 2 4 6 20 20 20 10 I of the T wave and should not be included in the QT measure-
UtN S=0 .5=0 S=0 S=0 S=0 S=0 S=0 S=0 ment. The lJ waves are usualiy prominent in hypokalemia and
LLN, lower limits of normal; ULN, upper limit o{ normal. may produce the appearance of a prolonged QTc, whereas the
true QTc (without the U wave) is not prolonged.
Abnormal ST segment (elevated or depressed): Pericar-
Abnormal QT intervals:
ditis, myocarditis, acute myocardial infarction or ischemia,
hyperkalemia or hypokalemia, severe ventricular hypertrophy l. Prolonged QT interval may be seen in:
('strain'), ventricular aneurysm, drug effect (digitalis), intra- a. Hypocalcemia
cranial pathology. b. Myocarditis: Rheumatic or viral
c. Long QT syndrome: Jervell and Lange-Nielsen syn-
TWave drome and Romano-Ward syndrome
The normal T wave should measure about one quarter to one-
d. Head injury or cerebrovascular accidents

third of the magnitude of R wave in leads with predominant e. Diffuse myocardial disease
R waves. After the first 3 days of life, the T wave is inverted
f. Quinidine, procainamide, etc.
in V, and usually inverted in V, and V, in early childhood. 2. Short QT interval may be seen in:
The age at which the T wave becomes upright in V, and V, a. Hypercalcemia (due to short ST segment)
varies from 5 to 15 years.
b. Digitalis effect
The T wave represents the ventricular repolarization process.
The amplitude of T wave is best measured in the left precor-
dial leads, although the T amplitude is influenced by many Pathologic ST-T Changes
physiologic processes, the normal T amplitude is usualiy less ST & T changes are relatively infrequent, because of a low
than the following: incidence of myocardial disorders. Not all ST segment shifts
In Vr: <1 year 11 mm are abnormal; slight shift is common in normal children.
>I year 74 mm Elevation and depression up to I mm in the limb leads and
In Vo: <1 year 7 mm up ro 2 mm in the precordial leads are within normal limits.
>1 year 9 mm Abnormal shifts of the ST segment are often accompanied
After adolescence, the amplitude is generally less than before. by T wave changes. An abnormal ST segment shift assumes
one of the following two forms:
Abnormal T wave:
1. Tall, peaked T waves may be seen in hyperkalemia, LVH
Downward slanting followed by diphasic or inverted
("volume overload"), cerebrovascular accident, particularly
T wave

hemorrhage, posterior myocardial infarction (tall T in

Straight or horizontal ST segment sustained for over
0.08 second
precordial leads)
2. Flat or low T waves may be seen in normal newborn Conditions that produce abnormal ST segment and T wave
infants, hypothyroidism, hypokalemia, hyper- or hypo- changes in children are:
glycemia, pericarditis, myocarditis, myocardial ischemia
(hypoxemia, anemia, shock, etc.), digitalis effect.
o L\GI or RVH with "strain"
o Digitaiis effect
o Pericarditis
QT lnterval o Myocarditis
QT interval is measured from the onset of Q wave to the end r Myocardial infarction
of the T wave. The QT interval represents the time required for r Electrolyte disturbances: hypokalemia and hyperkalemia
 !

ESSENCE OF PEDIATRICS

J-o*. + * -nll'*
il
lLqR -4- 't ..,{_ *o,"n"o*

T
o' V

,/L **, -,f, 0,.*,

Fig. 9.9: Examples of common QRS morphologies.

IVH or RVH with "Strain" Fig. 9.10: Measurement of amplitude.

The ST shift is seen in severe ventricular hypertrophy with

strain. There occurs ST segment depression and sharply inverted If a positive deflection is measured from the lower margin of
T wave in leads that represent the left or right ventricle. the baseline to the top of the positive defection, a Factitiously
large amplitude can result (Fig. 9.10).

Morphology of ECG Defleclions

USE OF ECG
The QRS (omplex

The initial negative (downward) deflection is called a Q wave ECG is usually employed in:
and the initial positive (upward) defection an R wave. A a Determination of heart rate and rhythm.
negative defection after the R wave is called an S wave. A a Determination of hypertrophy (atrial, ventricular).
secondary positive deflection after the S wave is labeled R', and a Atrioventricular conduction disturbances.
secondary negative wave after the R is called S'. 'When there is a Arrhythmias.
only a negative de{lection (without discernible R) in the QRS a Inflammations (pericarditis, myocarditis).
complex, it is calied a QS complex. Capital letters are used a Ischemia (myocardial infarction).
to describe the major deflection or a deflection that is at least a Digitalis toxicities.
one-half the amplitude of the major deflection. Lower-case a Electrolyte disrurbances.
letters are used to describe minor deflections with less than a Specific electrocardiographic diagnosis in certain heart
one-half the amplitude of the major deflection. diseases.
Examples of common QRS wave forms are shown in Fig. 9.9 .

HEART RATE AND RHYTHM

The P and T Waves and ST Segments
In routine electrocardiographic practice, the recording speed
P waves T waves ST seqments
of the paper is 25 mm per second. Therefore,
1 mm = 0.04 second
^ Upright P Uprighi T ]^ Elevated ST
5 mm = 0.20 second (1 large division between the
....- lnverted P lnverted T
j t\-/- Depressed ST
heary lines)
25 mm = 1.0 second (5 large divisions)
-\- Diphasic P Diphasic T (+/-) 30 mm = 1.2 seconds (6 large divisions), and
vr Notched P Diphasic T (-/+)
l^ Short ST
300 large divisions = 1 minute.
ECG paper: Time is measured on the horizontal axis. Each 1
JL Peat<eo P
JL Peaked P
1_,.- Long ST
millimeter equals 0.04 sec, and each 5 mm (a large division)
equals 0.20 second (Fig. 9.11). Thirty millimeters (or 6 large
Flat T
divisions) equals 1.2 seconds or ll50 minute. Every 7.5 cm
marked on the top margin of the paper equals 3.0 seconds or
Delerminolion of Amplitude 1/20 minute.
The amplitude of any positive deflections, such as B R, and T These features are used to determine the heart rate in the
waves, is measured from the upper margin of the baseline to following ways:
1. \fhen the heart rate is fast, count the RR cycles in
the very top of the positive deflection. The amplitude of any
negative defections (Q and S waves) is measured from the 6 large divisions (1.2 seconds) and multiply them by I\
lower margin of the baseline to the lowest point of the wave. 50 (one can count the RR cycles in 5 large divisions,
1
1
't
I
 CARDIOVASC U LAR DIS EAS ES

RAH
I
il

'3'r_./UZ\_
LAH >0.1 0

flr---
Iu,
CAH
l.-l 0.20 sec
5mm
Fig. 9.13: Criteria for atrial hypertrophy.
Fig. 9.11

2.
1.0 second, and multiply them by 60, but multiplying
by 50 is easier than by 60).
\W4ren the heart rate is slow, count the number of large
divisions between two R waves and divide into 300 (300
divisions = I minute).
Approximation of heart rate can be achieved by memorizing
heart rates for selected RR intervals. 'il{hen RR intewals are
5, I0, 75, 20, 25 mm, the heart rates are 300, 150, I00,75,
60 beats per minutes, respectively.
greater may satisft the criterion. A broad and notched P wave
in the limb leads is characteristic of LAH. Often the P wave is
diphasic in V, with a negative, prolonged, terminal segmenr.
Even in the presence ofnotched or diphasic P waves, prolonga-
]il
tion of the P duration is a requirement for LAH.
Combined atrial hypertrophy (CAH): Combined atrial hyper-
trophy produces a combination of increases in amplitude and
duration of the P waves.

Rhylhm Crilerio for Right Venlriculor Hypertrophy

The normal rhythm for any age is sinus rhlthm, in which the 1. Right axis deviation for the patientt age.
SA rode is the pacemaker of the entire heart. There must be a
2. Increased rightward and anterior QRS vector.

P wave (only one) in front of each QRS complex, and the P axis a. R in V,,% or aVR greater than the ULN for the
must be in the range of 0 to + 90 degrees. Therefore, P waves are patient's age.
always upright in lead II and usually upright in leads I and aW. b. S in I or Vn grearer than the ULN for the patient's
Abnormal or nonsinus rhythm is suggested by the pres- age.
ence of either abnormal number or shape of the P waves or 3. Abnormal R/S ratio in favor of the right ventricle (in the
abnormal P axis. See Fig. 9.12 for P wave changes for right absence ofbundle branch block).
atrial hypertrophy (RAH), left atrial hypertrophy (IAH), and
a. R/S ratio in V, and V, greater than the ULN for age.
combined atrial hypertrophy (CAH).
b. R/S ratio in Vu less than 1 after 1 month of age.
HYPERTROPHY 4. Upright T in V, in patients more than 3 days of age,
provided that the T is upright in the left precordial leads
Crileriq for Alriql Hypertrophy (Fig. ?.13) (Vr,Vu).

Right atrial hypertrophy (RAH) produces tall P waves (3 mm

5. A q wave in V, (qR or qRs patterns) is suggestive of
R\GI. (Make sure that here is not a small r in an rsR'
or greater) in any lead. This pattern is present most often in
configuration).
Iead II and occasionally in V, and Vr.
In general, the greater the number of positive, independent cri-
kft atrial hypertrophy (I-AH): LAH produces prolongation of
teria, the greater the probability of an abnormal degree of RVH.
the P duration, 0.10 second or greate! in any lead. In infants
less than 12 months of age, a P duration of 0.08 second or
RVH in the Newborn
Normal RAH LAH CAH The diagnosis of RVF{ in newborn infants is particularly dif-
RA RA ficult because of the normal dominance of the RV during that
il RA LA RA LA Arn period of llfe. The following clues, however, are helpful in the
)-t- -rtto _x_ diagnosis of RVH in newborn infants.
RA
*h* RA RA 1. Pure R wave (with no S) in V, greater than 10 mm.
VI
LA LA
LA "LA 2. R in V, greater than 25 mm, or R in a\{R. greater than
8 mm.
Fig. 9.12: P wave changes are presented for right atrial hypertrophy, 3. A qR pattern in V, (also seen in l0olo of healthy newborn
lett atrial hypertrophy, and combined atrial hypertrophy. infants).

ESSENCE OF PEDIATRICS
4. Upright T in V, in neonates more than 3 days of
(with upright T wave in Vu) is suggestive of R\&{.
5. Right a-xis deviation greater than +180 degrees.
Crileriq for Left Ventriculqr Hyperlrophy
Left a-ris deviation for the patientt age.
QRS voltages in favor of the left ventricle (abnormal QRS
voltages inferiorly, leftward and/or posteriorly).
a. R in I, II, III, aVL, aVF, Vr, or Vu greater than the
ULN.
b. S in V, or V, greater than the ULN.
Pressure overload (e.g., aortic stenosis) is often reflected
in tall R waves in II, III, and aVB whereas volume
overload (e.g., patent ductus arteriosus IPDA]) is
refected in V, and Vu.
3. Abnormal R/S ratio in favor of the LV:
a. R/S ratio in V, and V, less than the lower of
normal (LLN) for the patient's age.
4. Q in V, and Vu, 5 mm or more, coupled with tall sym-
metrical T waves in the same leads (suggestive of LVH),
so-called LV diastolic overload.
The greater the number of positive, independent criteria, the
greater the probabiliry of abnormal degree of LVH.
Crilerio for Combined Ventriculor
Hyperlrophy
l. Positive voltage criteria for right and left ventricular
hypertrophy (in the absence of bundle branch block or
pre-excitation).
2. Positive voltage criteria for RVFI or LVH and relatively
large voltages for the other ventricle.
3. Large equiphasic QRS complexes in two or more of the limb
leads and in the midprecordial leads (V, through Vr).
ARRHYTHMIAS
Clossificolion
1. Arrhythmias with sinus pacemaker: Sinus arrhythmia,
sinus bradycardia, sinus tachycardia.
2. Arrhythmias with ectopic pacemaker: Ectopic
(supraventricular and ventricular), atrial flutter and fibril-
lation, ventricular fibrillation, ventricular tachycardia.
3. Arrhythmias caused by conduction defects: Atrioventricu-
lar block (first degree, second degree, and third degree);
bundle branch block (right and left); sinoartrial block.
4. Others: Wolff-Parkinson-\7hite (\K"\7) syndrome.
Rhythms Originoling in the SA Node
All rhythms that originate in the sinoatrial node (sinus rhythm,
the normal rhlthm at any age) have the following characteristics:
1. P waves preceding each QRS complex, with a regular
PR interval (the PR interval may be prolonged in first-
I
age
Regular sinus rhythm
Sinus tachycardia
Sinus bradycardia
Sinus arrhythmia
Fig. 9.14: Normal and abnormal rhythms originating in the SA
node. All these rhythms have a P wave in front of each QRS
complex with a regular PR interval and a P axis in the 0 to
+90 degree quadrant.
degree AV block, as discussed later, but it is still a sinus
mechanism.)
2. Normal P axis (0 to +90 degree). This produces an upright
P in lead II and an inverted P in aVR.
Regular sinus rhythm: The rhythm is regular and the rate is
normal for the patient's age. The characteristics of sinus rhy'thm
(normal or abnormal) are shown inFig. 9.I4.
Sinus Tachycardia
The rate is faster than normal for the padent's age, and the
P-QRS complexes are perfectiy normal. In adults, a rare in
excess of 100 beats per minute is considered tachycardia. In
general, a rate in excess of 140 per minute in children and a
rate of 160 or more in infants may be significant.
In children, sinus tachycardia is usually due to anxiety created
by undergoing an ECG and therefore is not as significant as it
is in adults, unless it occurs during sleep. Tachycardia during
sleep or at rest can be caused by congestive heart failure second-
ary to congenital or acquired heart disease, myocardial disease,
fever, thyrotoxicosis, or shock. fteatment is required not for
the tachycardia but for the underlying disorder.
Sinus Bradycardia
beats
The rate is slower than normal for the patient's age, and the
ECG complexes are completely normal. In adults, a rare under
60 beats per minute is defined as bradycardia. The definition
is not ciear-cut for pediatric patients, but a rate under 80 per
minute in newborn infants and under 70 per minure in older .
children may be significant. During sinus bradycardia, the AV
:
node may capture the pacing roie by virtue of a higher rate
of automaticiry.
Sinus bradycardia is rare in healthy children but is seen in
trained athletes. Increased intracranial pressure, hypothyroid-
ism, hypothermia, profound hypoxia, "sick sinus" syndrome, '1
tl
hyperkalemia, and drugs such as digitalis and beta-adrenergic
blockers may cause sinus bradycardia. Sinus bradycardia per t
'f
I
1r
+
 !

CAR DIOVASCU LAR DISEASES

se rarely requires treatment, but treatment should be directed Premature artial .- RR* 2XRR '
to correct an underlying cause. beats
-
High
Sinus Arrhythmia
Low
Sinus arrhythmia is a phasic irregularity of the heart rate,
increasing during inspiration and slowing during expiration
but maintaining the normal PQRS configuration and relation. Atrial tachycardia
Sinus arrhl'thmia is pronounced in adolescents. This rhythm Pl P1 pt pt p1 p1 p1
P1 P1
indicates that the cardiovascular system is under vagal control
and not under sympathetic control and therefore is regarded Atrial flutter
as a sign of good cardiac reserve.
Atrial fibrillation
Sick Sinus Syndrome Rapid ventricular
response
Sick sinus syndrome (SSS), a well-known entiry in adults, is
Slow ventricular
now increasingly recognized in children who undergo extensive response
cardiac surgery.
The sinus node may fail to function as the dominant pace- Fig. 9.15: Arrhythmias originating in the atrium.
maker of the heart, resulting in a variety of arrhythmias, with
or without symptoms. The arrhythmias include profound sinus
bradycardia, sinus arrest with junctional escape, paroxysmal may also be associated with structural heart disease. PAB per
se does not require treatment.
atrial tachycardia, slow or fast ectopic atrial or nodal rhythm,
and bradytachyarrhythmia. The rhlthm m^y Yary from one
rype to another; the abrupt slowing after tachycardia is the Atrial Tachycardia
most worrisome. Very rapid tachycardia (usually 240 x 40 beats per minute),
Patients who suffer from SSS in the immediate postopera- with normal appearing QRS complexes, was formerly thought
tive period may have frequent episodes of tachycardia, requir- to be produced by rapid firing of a single focus in the atrium.
ing antiarrhlthmic drugs such as propranolol. Frequency of At very rapid rates, the P wave is buried in the T of the preced-
tachycardia tends to decrease over the years. Children with ing beat so that atrial tachycardia is difficult to separate from
periods of extreme bradycardia following tachycardia may the more rare nodal tachycardia. This led to use of the term
require demand pacemaker therapy. supraventricular tachycardia (SW) to include both of these
arrhythmias. Actually, the great majoriry of occurrences of SVT
are due to AV reentry or reciprocating tachycardia rather than
Rhythms Originoting in the Alrium (Olher rapid firing of a single focus.
thqn SA node), Eclopic Airiql Rhythm This is the most common abnormal tachycardia during the
The term ectopic beat is used to signify the nonsinus rhythm infancy and childhood. It is characterized by abrupt onset and
in which other parts ofthe heart, rather than the SA node, are cessation, may be precipitated by an infection. Rate usually
the pacemakers. Ectopic beats are usually premature but may exceeds 180/min, may be as rapid as 300/min. CHF is more
come after a longer than normal pause (escape beat). Ectopic common in infants than children. Vagotonic maneuvers (ice
beats may be atrial, AV junctional, or ventricular in origin. bag placing on the nasal bridges, drinking ice water, breath
Atrial arrhythmias are characterized by the following holding, valsalva) are efFective. IV digoxin is effective if above
(Fig.9.l5): measures fail. Propranolol can be given, which decreases sinus
heart rate. Ca-channel antagonists are also effective. DC car-
P waves of unusual contour and/or an abnormal number
dioversion is recommended in critically ill patients, when CHF
per QRs complex,
has already. developed.
QRS complexes of normal duration but with occasional
bizarre, wide QRS complexes due to aberrancy.
Atrial Flutter
Atrial activity is seen flutter or F waves with a "sawtooth"
as
Premature Atrial Beats (PAB) configuration, best seen in leads V,, II, and iII. The atrial rate
Characterized by an abnormal shaped P wave that occurs prema- is about 300 per minute (240-360 per minute). The AV node
tureiy, in a normal QRS complex and no compensatory pause. cannot respond that rapidly, fortunately, so there is a degree
Premature atrial beats are common in healthy children, of AV block (2:,1, 3:1, 4:1, etc.). The QRS configuration is
even in newborn infants, and may have no significance. They usually normal.

ESSENCE OF PEDIATRICS
Atrial flutter is rare in infants and children. It may be associ-
ated with structural heart disease with dilated atria, myocarditis,
or other acute infectious diseases. teatment consists of digoxin
(to increase the AV block and slow the ventricular rate) with or
without propranolol. Electrical cardioversion may be required.
Quinidine may prevent recurrences.
Atrial Fibrillation
The atrial waves are totally irregular and vary in size and shape
from heat to beat. They are usually most prominent in V,. The
atrial rate ranges from 350 to 600 per minute. The ventricular
response is irregularly irregular and may be fast or slow. The
QRS complexes are usually normal.
Atrial fibrillation, like flutter, is rare in children. When
present, it is usually associated with structural heart defect.
Treatment with digoxin is used initially ro decrease ventricular
rate. Propranolol may be added if necessary. Conversion to
sinus rhythm is worth trying in an acute situation, either by
counter shock, with quinidine, or both.
Rhylhms Originoting in the AV Node
Rhythms originating in the AV nodal or junctional area
characterized by the following:
r The P waves may be absent, or if present they occur after
the QRs complex and are inverted.
o The qRS complexes are usually normal in duration and
configuration.
Rhylhms Originoling in the Venlricle
Ventricular arrhythmias are characrerized by the following
(Fig.9.15):
o QRS complexes are bizarre in configuration and long in
duration.
. QRS complexes and T waves olten point in opposite direcdons.
r QRS complexes are randomly related to P waves, and fusion
beats are common.
Premature Ventricular Contraction
A premature ventricular conrraction (PVC) is characterized
by a wide QRS complex occurring before the next expected
QRS complex in a regular rhythm. The T wave points in the
Premature ventricular
contraction (PVC)
Ventricular iachycardia
Ventricular fi brillation
Fig. 9.16: Ventricular arrhythmias.
I
direction opposite to the QRS complex. There is no premature
P wave preceding the premature QRS complex. The retrograde
impulse is usuaily blocked in the atrioventricular node, and
the SA node is not depolarized by the retrograde conduction;
the SA node "clock' keeps its original pace. Therefore, there is
a full compensarory pause. This means that the length of two
cycles, including one premarure beat, is rhe same length as
two normal cycles (2 x RR). If the PVC arises from a single
focus (unifocal), the QRS complexes will be of the same
configuration in the same lead. If they arise from different
foci (multifocal), the QRS complexes will be of different
configurations in the same lead. If each PVC alternates with
normal ventricular complexes regularly, the rhythm is called
ventricular bigeminy or coupling. If each PVC regularly
follows two normal QRS complexes, rhe rhythm is called
ventricular trigeminy.
Occasional PVCs are benign in children, particularly if they
disappear or decrease in number with exercise. Antiarrhythmic
drugs such as lidocaine, quinidine, propranolol, diphenylhy-
dantoin, or procainamide may be indicated.
Ventricular Tachycardia
are Ventricular tachycardia (W) is a series of three or more PVCs
occurring ar a rate of 120-180 per minute. It is diflicult to
differentiate VT from supravenrricular tachycardia with aber-
rant (intraventricular) conduction.
Ventricular tachycardia is rare in children but is a serious
arrh;.thmia and may signi$' myocardial damage or dysfunction.
It can deteriorate ro ventricular fibrillation, although this is
not as likely as in the adult with coronary artery disease. Faster
rates should be treated prompdy with anti-arrhlthmic drugs
such as IV lidocaine. Cardioversion is rarely of more than
transienr efrectiveness. Complete abolition of the arrhythmia
is less importanr than keeping the rate bellow 150 for infants,
and 130 for older children.
Ventricular Fibrillation
Ventricular fibrillation (VF) is characterized by a bizarre ven-
tricular QRS pattern of varying size and configuration. The
rate is rapid and irregular. This is usually a terminal arrhythmia
as it cannor provide effective perfusion of the myocardium.
Successful resuscirarion depends on prompt recognition and
cardiac defi brillation.
\
1
1
'l
!
 CARDIOVASC U LAR DISEAS ES

ATRIOVENTRICU LAR CON DUCTION First degree

DISTURBANCES AV block

Second degree
Criteria for RBBB: AV block
1. Right axis deviation, at least for terminal portion of QRS (Wenckebach
Phenomenon)
(QRSI); initial part of QRS (QRSi) is unchanged.
2. QRS duration longer than the ULN for the patient's age. 2:1 AV block
3. Terminal slurring of the QRS complex directed to the
right and usually, but not always, anteriorly. Complete
a. Wide and slurred S in I, Vr, and \ (third degree)
AV block
b. Terminal, slurred R' in aVR and in V,, and V,
4. ST depression and flwave inversion are common in adults Fig. 9.17: Disturbances of atrioventricular conduction.
with RBBB, but not in children.
First-Degree AV Block
Criteria for LBBB:
In first-degree AV block, there is a disturbance in conduction
1. Left axis deviation for the patient's age.
between the sinus node and the ventricles, produced by an
2. QRS duration longer than the ULN for the patientt abnormal delay in conduction through the AV node. This
age'
results in prolongation of the PR interval beyond the upper
3. Loss of Q waves in I, Vr, and Vu.
limit of normal for the patientt age and rate. Sinus rhphm is
4. The slurred QRS complex is directed to the left and
maintained and no dropped beats occur. The QRS complex
posteriorly.
is normal in configuration.
a. Slurred and wide R waves in I, aVL, Vr, and Vu, It is sometimes seen in healthy children and in children with
b. '$7'ide S waves in V, and Vr. infectious disease. It is sometirnes associated with a wide variety
5. ST depression and T wave inversion in V, through Vn of cardiac conditions such as rheumatic fever, cardiomyopathies,
are common. atrial septal defect, and Ebstein anomaly. It is also a sign of
6. QRS voltages may be greater than normal because of digitalis toxicity. First-degree AV block (not caused by digitalis)
the asynchrony of depolarization of each ventricle. One does not produce symptoms or require treatment.
should not make a diagnosis of ventricular hypertrophy
when LBBB is present. Second-Degree AV Block
Second-degree AV block is characterized by some, but not all,
Criteria for \il7PW syndrome:
dropped beats in which some P waves are not followed by QRS
1. Short PR interval, less than the lower limits of normal
complexes. There are several types:
(LLN) for the patient's age. The LLN according to age
is as follows: MobitzType I (W'enckebach Block or Phenomenon): There
Less than 3 years 0.08 second is a progressive lengthening long diastolic pause results and
3-16 years 0.10 second the cycle is resumed. The number of beats in each cycle is
More than 16 years 0.12 second not necessarily constant. The QRS complexes are normal
in configuration. Type I block with a normal QRS complex
2. Delta wave (initial slurring of the QRS complex).
almost always takes place at the level of the AV node. It may
3. Vide QRS duration (beyond the ULN).
be a sign of digitalis toxiciry and can occur in any condition
that causes first-degree AV block.

Alriovenlriculor Bloc k
Atrioventricular block is a disturbance in conduction between
the normal sinus impulse and the eventual ventricular response
(Fig. 9.17). Depending on the severity of the conduction
disturbance, AV block is classified into three classes: (i) A
simple prolongation of the PR interval is called first-degree AV
block; (2) second-degree AV block is an intermediate grade of
conduction disturbance in which some atrial impulses are not
conducred into the ventricle; (iii) third-degree AV block (or
complete heart block) is the most extreme form of AV block
in rvhich none of the atrial impulses are conducted into the
r.entricle.
Mobitz Tlpe II: The AV conduction is "all or none." There is
either normal AV conduction with normal PR interval, or the
conduction is completely blocked. The atrial rate is normal, but
the ventricular rate depends strictly on the number of success{irl
conducted auial impulses. The failure is at the His bundle level.
This rype of second-degree AV block is more serious than
type I, since it may progress to complete heart block. Prophy-
lactic pacemaker therapy may be indicated in older adults who
could not survive sudden, complete heart block.
Two-to-One (or Higher) AV Block A ventricular complex
follows every second (third or fourth) atrial complex, resuiting
in 2:I (3:l or 4:l) AV block.
ESSENCE OF PEDIATRICS

Fig. 9.18: AV dissociation owing to either marked slowing of the sinus node or acceleration of the AV node.

Complete (Third-Degree) AV Block

I
In complete AV block, the atria and ventricles beat entirely jt-\ Elevated ST segment
independently of one another. The atrial rhythm is regular
(regular PP interval), and the rate is that of the average sinus
rhythm for the patientt age. The ventricular rate is also quite
regular (regular RR interval) but of much slower rare. The QRS
It^ Return of ST segment
toward normal
complex is normal if the pacemaker is in the AV node or a level
il
higher than the bifurcation of the His bundle. Most children
with congenital complete heart block belong in this category.
-tv- T wave inversion

The QRS complexes will have the appearance of ventricular

premature beats if the pacemaker is in either ventricle with a
slow rate (about 40lmin), called idioventricular rhythm.
Asymptomatic children with congenital heart block do not
require pecemaker therapy until they become symptomatic.
Surgically induced complete heart block may require pacemaker
therapy, at least during the immediate postoperarive period.
Alrioventriculor Dissociolion (Fig. 9.1 8)
Confusion has existed and still exists regarding the definition
of atrioventricular dissociation. A widely accepted use of AV
dissociation is any condition in which the atria and ventricles
be at independently, so that the P waves and QRS complexes
do not have any relationship.
In AV dissociation, the atrial rate is slower than the ven-
tricular rate, whereas in complete heart block the ventricular
rate is usually slower than the atrial rate. In AV dissociation,
an atrial impulse may conduct to the AV node if it comes ar
the right time. The conducted beats can be recognized by their
relative prematurity. In complete AV block, no atrial impulse
goes through the AV node. Therefore, the RR interval maintains
clockJike regulariry and the ventricular rate is relatively slow.
PERICARDITIS
The ECG changes seen in pericarditis are rhe result of subepi-
cardial myocardial damage and or pericardial effusion.
1. Subepicardial myocardial damage produces the following
time-dependent changes in the ST segmenr and T wave
(Fig.9.i9):
a. Elevation of ST segment in many limb and precordial
leads, particularly those representing the LV
b. lil/ithin 2-3 days, the ST segmenr rerurns close to
normal. The T wave is small but still upright. It is
difficult to detect the abnormaliry at this stage.
c. Two to four weeks after the onset, T waves become
sharply inverted, and there is an isoelectric ST segment.
This change may persist 1-2 months.
Fig. 9.19: Time-dependant changes of ST segment and T wave
in pericarditis.
2. Pericardial effusion may result in low QRS voltages in
many leads. Low voltages are said to be present when
the amplitude of the QRS complexes in every one of the
limb leads is 5 mm or less.
Acute myocardial infarction produces ECG findings similar
to those of pericarditis. The main differences are that in
acute myocardial infarction, rhe changes are more localized,
ST segment and T wave changes occur simultaneousll., and
pathologic Q waves appear.
MYOCARDITIS
Electrocardiographic findings of myocarditis (rheumatic or
viral) are relatively nonspecific and may include changes in
all phases of cardiac cycle, even arrhythmias or ectopic beats.
One or more of the following changes are seen in myocarditis:
o Delayed AV conduction (first-degree AV biock).
r Prolongation of QTc.
o Decreased amplitude of the T wave.
o Low QRS amplitude, i.e., 5 mm or less in all six limb leads.
o Arrhlthmias or ectopic beats.
MYOCARDIAT INFARCTION
Myocardial infarction is rare in pediatric cases. In the early
phase of acute myocardial infarction, three electrically distinct
zones are present: the zone of necrosis (center) is surrounded
by a zone of injury, rvhich in turn is surrounded by a zone of .
ischemia. No depolarizarion occurs in the necrotic area; leads
facing the necroric area will record depolarization from live
mvocardium away from the electrode, producing the pathologic
Q wave. A iead facing the area of injury will record the ST 1
vector shift toward the lead, producing ST segment elevation. 1
(The injured cells are partially depolarized and actually create 1
t
1
1
a
 !

CAR DIOVASC U LAR DISEAS ES

A Toxicity:
Hyperacute phase /-\ Elevated STsegment
(a few hours) L Deep and wide e wave o Prolongation of PR interval is a more reliable early sign of
1
toxicity than arrhythmias. It may progress to second-degree
phase
days) -Vi\- \z=
Early evolving Deep and wide e wave AV block (some normal children may have a prolonged
(a few Elevated ST segment
Diphasic T wave PR interval).
/t o Profound sinus bradycardia or SA block.
Late evolvino onase /L .- Deep and wide Q wave o Supraventricular arrhlthmias, such as atrial or nodal ectopic
(z-a weJ<il - V V Sharply inverted T wave
beats, and tachycardia, particularly if accompanied by AV
block, are more common than ventricular arrhythmias in
Resolving phase -l//\* ,.- Deep and wide Q wave
Almost normalr wave
(foryears) Y children.
r Ventricular bigeminy or trigeminy is extremely rare in
Fig. 9.20: Sequential changes of ST segment and T wave in children with digitalis toxiciry although common in adults.
myocardial infarction. Premature ventricular contractions are not uncommon in
children; howevet death may follow ventricular tachycardia.
a depression of the entire baseline ITP and PQ segments] A sound rule is to assume that any arrhythmia occurring
with the exception of the ST segment; owing to the record- with digitalis is caused by digitalis until proved otherwise.
ing characteristics of ECG machines, this appears as an ST
elevation). The leads facing the ischemic area record the T
ELECTROTYTE DISTU RBANCES
vector going away from the area. Thus, a lead facing the area
of infarct will record all three patterns, including a pathologic Two important serum electrolytes that produce ECG changes
Q wave (wide and deep), ST segment elevation, and T wave are calcium (Ca.) and potassium (K.).
inversion. The pathologic Q waves of myocardial infarction
1. Hypocalcemia (Fig. 9.21): The calcium ion is mainly
are of longer duration (at least 0.03 sec and usually 0.04 sec)
concerned with phase 2 of the action potential and affects
and of greater amplitude than normal.
only the duration of the ST segment. Hypocalcemia
The ECG findings of myocardial infarction are time-
prolongs the ST segment with resulting prolongation of
dependent (Fig. 9.20). The short-lived hyperacute phase,
characterized by ST segment elevation and pathologic Qwave
QTc; however, it does not alter the duration or vector
of the T wave. The ST segment remains isoelectric. The
is seen during the first few hours after the infarction. More
T wave is simply delayed, not actually widened.
common ECG findings of myocardial infarction are abnormal
2. Hlpercalcemia (Fig. 9.21): This shortens the ST segment
Q waves, ST segment elevations, and T wave inversions. without affecting the duration of the T wave, with result-
These changes are seen from several hours to days after the
ing shortening of QTc.
onset of the infarct (early evolving phase). During the first
few weeks after the infarction, there is a gradual return of
3. Hypokalemi a (Fig. 9 .22); This produces one of the least
specific ECG changes. \7hen serum K- is below 2.5
the elevated ST segment toward the baseline (late evolving
mEq/L, ECG changes consist of the following.
phase). The abnormal T waves gradually return to a normal
or near-normal configuration (resolving phase). Therefore, a. Prominent IJ wave: There is an apparent prolongation
in a stabilized old infarction the only evidence of a previous of QTc, but actually a "long" QU interval is present.
myocardial infarction may be a pathologic Q wave in leads b. Flat or diphasic T waves.
oriented to the infracted scar. c. ST segment depression.
\With further lowering of serum K., the PR interval may
become prolonged and sinoatrial block may occur.
DIGITATIS TOXICITIES AII of the ECG features of hypokalemia may be
found in LVH with "strain", including prominent U
Digitalization in children needs to be monitored closely by
waves. In L\rH with "strain", the ST segment shift
frequent rhythm strips, primarily to detect digitalis toxicity.
The ECG does not answer the question of whether the patient

+rl^+^
is "fully' digitalized; it is a clinical decision. Findings sugges-
tive ofdigitalis effect and those suggestive oftoxiciry are listed
below. An ECG before starting dlgitalls therapy is mandatory.

Effect:
Hypercalcemia Normal Hypocalcemia
a Shortening of QTc-the earliest sign of digitalis effect.
a Sagging ST segment, i.e., depression of terminal portion of Fig. 9.21: ECC findings of hypercalcemia and hypocalcemia.
St segment and decreased T amplitude. Hypercalcemia shortens and hypocalcemia lengthens the ST
Slou'ing of the heart rate. segment. The T wave is not altered.
ESSENCE OF PEDIATRICS

Serum K* left axis deviation, in addition, tricuspid atresia is almost cerrain.

Depressed ST segment If the a-xis lies benveen +30 and +90 degrees, pulmonary atresia
<2.5 mEq/L
---/Lr"- Diphasic T wave

Normal _J- Prominent U wave with intact ventricular septum is likely. If the a-ris is beyond
degrees, tricuspid atresia with transposition is likely.

>6.0 mEq/L
-^lt^- /\
Tall T wave
/ \ /\ Lons PR interval
>7.5 mEq/L ---,.\) W \- Wide QRS duration
Tall T wave
Fig. 9.22: ECC iindings of hypokalemia and hyperkalemia.
is usually toward the right and anteriorly, whereas
inhypokalemia the ST segment shift is toward the
left and posteriorly. Both hypokalemia and digitalis
produce ST segment depression. Digitalis, however,
produces a short QT interval and ordinarily no promi-
nence of the U wave.
4. Hyperkalemia (Fig. 9.22): A tall, peaked, symmetric
T wave with a narrow base, the so-called "tented" T wave
is the earliest ECG abnormality. The following ECG
sequence is associated with a progressive increase in the
serum K* level. These changes are usually best seen in
leads II, III, and left precordiai leads.
a. Tall "tented" T wave, usually best seen in precordial
leads.
b. Prolongation of QRS duration (intraventricular block).
c. Prolongation of PR interval.
d. Disappearance of P wave.
e. \Wide, bizarre, diphasic QRS complex ("sine wave").
f. Ventricular fibrillation or cardiac arrest.
SPECIFIC ETECTROCARDIOGRAPH IC
DIAGNOSIS IN CERTAIN HEART DISEASES
A few ECG patterns are suggestive of certain lesions. Prolonga-
tion of the P-R interval suggests an endocardial cushion defect,
or Ebstein anomaly. Right bundle branch block pattern sug-
gests atrial septal defect. Vith right axis deviation, an ostium
secundum defect is likely; with left axis deviation, an osrium
primum. Right bundle branch block pattern with right atrial
hypertrophy suggesrs Ebstein anomaly.
Left axis deviation suggesrs an endocardial cushion defect
or ventricular septal defect. Exrreme left a-ris deviation (-90
to -150 degree) suggests a large ventricular septal defect, or
origin of both great arteries from the right ventricle.
fught atrial hypertrophy and absence of right ventricular
hypertrophy in cyanotic lesions suggest either tricuspid atresia
or pulmonary atresia with intact ventricular septum. If there is
Shock is a state of widespread reduction in effective tissue
perfusion resulting in insufficient distribution of oxygen and
nutrients, which leads to anaerobic metabolism, accumula-
tion of lactic acid and consequently cellular damage, multiple
organ dysfunction and finally cardiovascular collapse. The
pathophysiological event in shock, regardless of etiology, is
tissue hypoperfusion, which leads ro rissue hypoxia, acidosis,
and end-organ dyiluncrion.
Maintenance of an adequate mean arterial pressure (MAP) is
fundamental to ensure adequate perfusion and organ function.
\When MAP falls, blood fow decreases, resulting in tissue isch-
emia and organ failure. The kidney receives the second highest
blood fow of any organ; measurement of urine output and
creatinine clearance can be used as an indicator of adequate
perfusion pressure.
ETTOLOGTC CTASSt FtCAT|ON
The patient with shock may have abnormalities in the blood
volume, pumping function of the heart, and blood fow dis-
tribution:
1. Hypovolemic (decreased circulating blood volume): It
is usuaily caused by vomiting, diarrhea, or hemorrhage.
Shock may appear abruptly or gradually over several stages.
\(hen cardiac output (CO) is unable ro meet the demands
of the tissues, compensarory sympathetic activity produces
selective vasoconstriction ofthe skin and splanchnic vessels
to divert blood flow to vital organs, namely brain, heart
and kidney. Once the volume deficit exceeds 25o/o of
the total volume, the compensatory mechanism fails and
profound reduction of CO and fall of BP occur.
2. Cardiogenic shock: It is known as pump failure. It will
have low CO, hypotension, and clinical signs of inad-
equate tissue perfusion. Typically intravascular volume is
adequate, but cardiac dysfunction limits CO.
J. Distibutive shoclc The common denominator of this shock
is leakage of intravascular fluid through capillary bed into
interstitial space known as third spacing of fluid because of
endothelial damage. Early sepdc shock is known as warm
shock or hyperdynamic phase as it is characterized by warm
extremities, high or normal CO, normal BII increased pulse
pressure, and low systemic vascular resistance. Despite high
CO, shock and metabolic acidosis develop; blood flow is
inappropriately distributed. Early trearmenr ar rhis stage
may prevent progression. The later phase of cold shock or
hypodynamic phase is characterized by cold extremities, high
 CAR DIOVASCU LAR DIS EAS ES

systemic vascular resistance, iow CO, narrow pulse pressure, o Use of vasoactive drug: Inotrops increase myocardial con-
and hypotension leading to hypoxia, acidosis, and death. tractiliry and often increase heart rate (e.g., dobutamine,
mid-dose dopamine [5-10 prg/kg/min], low-dose epineph-
Table 9.15 summarizes a list of causes of shock.
rine [<1 pg/kg/min]).
Vesopressors increase systemic and pulmonary vascular
CLINICAL FEATURES AND STAGES resistance and typically will increase systemic and pulmonary
arterial pressure (e.g., norepinephrine, high-dose dopamine
Shock can progress over a span of few minutes to hours. [>10 pg/kg/min], high-dose epinephrine [>1 pg/kgimin]).
The progression can be arbitrarily divided in three stages Vasodilators are designed to reduce systemic and pulmo-
(Thble 9.16): (i) early compensated shock, (il) decompensated nary vascular resistance. They reduces ventricular afterload
shock, and (liz) irreversible shock. and often improve stroke voiume and CO (e.g., nitroglyc-
erine and nitroprusside).
TREATMENT Dopamine increases CO with doses 5-10 pg/kg/min'
Vasoconstrictor effect of dopamine is evident at doses >15
o Start with ABCs pg/kg/min due to release of norepinephrine from sym-
o O, inhalation pathetic system. Dopamine refractory shock responses to
o Fluid therapy: First choice of fluid should be 0.9% NS or norepinephrine or high-dose of epinephrine.
funger lactate. Crystalloids are the first choice in acute phase, o Acid-base normalization: NaHCO. therapy to improve
but when the fluid requirement is high colloids (Dextran, myocardial function should be given when pH < 7, ar'd
5% albumin) may be given. Packed RBC can also be given IV infusion of 1-2 mli kg of 10% Ca-gluconate shouid be
at 10 mlikg to maintain PCV 30% or Hb 10 g/dl. given when ionized calcium level falls below 2-4 mg/dl.
Fluid infusion is best started with boluses of 20 ml/kg- Hypoglycemia, when present, should be corrected.
titrated with heart rate, capillary refill time, sensorium- o Antibiotics: Appropriate antibiotic (third-generation cepha-
to be infused rapidiy over 5-10 minutes. If no improve- losporin with vancomycin) should be given, especially in
ment, repeat-boluses of Z0 mi/kg shor-rld be given. Large septic shock.
volume fuid deficit may require 40-50 ml/kg and o Adjunctive therapy: Done with steroids and immuno-
maximum up to 200 mlikg over first hour for make up therapv, especially in septic shock, without much success.
the deficit. The patient who do not respond to rapid Extracorporeal membrane oxygenation (ECMO) is useful
boluse s of 40-60 ml/kg in first hour of therapy are labeled therapy for refractory shock in neonates'
as fluid-refractory shock and should be given inotropic . teatment of cause(s).
suppoft.
See Figure 9.23 for guidelines to manage septic shock.
Table 9.15: Causes of Shock Theropeutic End - Poinls
Hypovolemia
The therapeutic end-points of shock resuscitations are:
. Fluid and electrolyte loss: vomiting, and pathologic
renal Ioss a Capillary refill time <2 sec
. Blood loss: lntracranial bleeding in neonate and Cl bleeding
a \7arm extremities
. o Urine output >1 ml/kg/hr'
Plasma loss: Leaky capillaries in sepsis, dengue shock syndrome,
third space loss in intestinal obstruction and peritonitis, and a Normal mental status
hrrrn. a Normal pulses and respiration
. Endocrine: Diabetes insipidus, adrenal insufficiency
Cardiogenic Table 9.16: Stages of Shock
. Myocardial insufficiency: CCF, cardiomyopathies, arrhythmias,
n-ryocardial depression due to hypoglycemia
. Outflow obstruction; Cardiac tamponade, tension pneumothorax Heart rate Tachvcardia Marked Severe tachycardia,
tach,vcardia bradycardia
Distributive
Respiratory rate Normal Tachypnea Tachypnea/apnea
' Septic shock*
Hypotension Severe hypotension
Normal
. Anaphylaxis
BP

Pulse pressure Normal Low Markedly low

. Den8ue shocl< svndrome
Cool Mottled Cold and cyanotic
. Prolong hypoxia of ischemia
Skin
Mental status Anxious Obtunded Coma
-septic shock actually has components of several groups including distributive,
Urine Normal (Jlrguna Anuria
card ogenic, and hl,povolemia.
ESSENCE OF PEDIATRICS

Recognize $eptie, shock: rti4aintain

airway and lV rt"*r
qg,pei:pedidn'c
advanced life €upp$rt (PAIS), :,' ,

2O ml/ft9 NS botus _up to'And:,over'

60 ml/kg. Correct hypoglycemia,

Fluid refractory shock

I

I
I Y

+ Start dopamine or dobutamine at

10 pg/kg/min.
Observe in PICU I
ffi lnserl.CVPrlin€ g€aft arteiial l

pressure monitoring

Dopamine/dobutamine-resistant shock

Cold
-**
Startnorepinephrine Startepinephrine
**
lf shock persists, cathecholamine
resistant shock
,

i
I

Give hlldrocortisone if adrenal

insulf iejenqp,$u$peeied.

Cotd shock + ' Q6lfl sftsek 1': ' r' Ifrlaffn'Shoek +

Normal BP' l lsw 8P lowBP'-: r ,

Add Vasodilato,r. Titratevolufie, Titrate' ,,

or type lll .. and epinephrihe volume alld:'ri i

Phogphodiesle(ase 11o!:epi{eBhrtne
inhibitor

Fig. 9.23: Cuidelines to manage septic shock.

Hay V W (ed). Current Padianic Diagnosis & Tieatrnent 14.h ed. :

Stamford: P-H Internatio nal lnc., 7997.
Behrman RE, Kliegman RM, Jenson HB. Nelson Tbxtbook of 5. Molla MR. Heart Diseases in Children 2"d ed. Dhaka: Daisy QMB,
2007. t
Pediatrics 18'h ed. Singapore: Harcourt Asia Pte Ltd., 2009.
2. Parthasarathy A (ed). IAP Tbxtbook of Pedianics 4,h ed. New Delhi:
6. Park MK, Gunther Oth !?G. Hout to Read Pediatic eCGs 3d ed,. \
Jaypee Brothers, 2009.
New Delhi: Jaypee Brothers, 1992. I
3. HohnAR Diagnosis & management of hypertension in childhood. 7. Dworkin PH. NMS: Pediatrics 4.h ed. Philadelphia: Lippincott 1
'$Tllliams 1
Pediatr Ann 1997 ;26:705. & \fitkins, 2000.

I'
 CHAPTER 10
Renal Diseases

Chopter Conlents
Clomerular dis0rders................. . .. ,.....195 Distal renal tubular acidosis..... ..198 Vesicoureieric ref|ux...................,...,,.................................205

Acute posistreptococcal glomerulonephritis................195 Proximal renal tubular acid0sis.................,.....................198 Acute renal failure ..................... .....,..206

Focal segmental g10meru10sc1er0sis.............................. 197 Nephrotic syndrome................ ...........198 Chronic kidney disease .......,................................................ 207

lgA nephropathy (Berger disease)................................. 197 ldiopathic nephrotic syndr0me....,...,.......,...,...,............,.....198 Peritoneal dialysis ............,........ ..........209

Hemolytir uremic syndrome ........................................... 197 Urinary tract infecti0n...........................................................202

Tubular disorders.. ...............................,l98 Recurrent UT|......................................................................204

Clinical presentation of glomerulonephritis:

Disorders of glomeruli may manifest either as leakage of blood
constituents normally retained by the glomerular basement
membrane (e.g., proteins, red blood cells) or by impaired
filtration causing retention of metabolites that are normally
excreted, or by a combination of both.
Classifi cation of glomerular disorders:
A. Primaryglomerulonephritis
1. Immune complex glomerulonephritis
a. Postinfectious acute glomerulonephritis
b. Mesangial IgA nephropathy (Berger disease)
c. Membranoproliferative glomerulonephritis
including crescentic glomerulonephritis
d. Membranous glomerulonephritis (idiopathic)
2. Anti-GBM antibody mediated glomerulonephritis
3. Uncertain etiology, e.g., minimal change glomerulo-
nephritis, focal segmental glomerulosclerosis.
B. GlomerulonEthritis associated uitb systemic disorders
1. Immunologically mediated
a. Henoch-Schonlein purpura
b. Systemic lupus erythematosus and other
disorders, e.g., scleroderma
c. Polyarteritis nodosa, \Tegener granulomatosis.
d. Systemic infections (bacterial endocarditis, syphilis,
malaria, hepatitis B or C)
2. Hereditary disorders
a. Familial nephritis, e.g.,
b. Sickle cell anemia
3. Other conditions: Diabetes mellitus, amyloidosis
o Acute glomerulonephritis (acute nephritic syndrome)
r Rapidly progressive glomerulonephritis
o Nephrotic syndrome
o Chronic renal failure
r Asymptomatic hematuria and/or proteinuria
Causes of acute glomerulonephritis (AGN)
o Post-infectious: Bacteria-GroupA Streptococcus, Staphy-
lococcus, Pneumococcus, Salmonella; Virus-Hepatitis B,
C, HIV EBV.
o GN of undetermined etiology: Membranoproliferative GN,
rapidly progressive GN
e Collagen vascular diseases: SLE, Henoch-Schonlein
purpura
. IgA nephropathy
o Hemolytic uremic syndrome (HUS)
ACUTE POSTSTREPTOCOCCAT
GLOMERUTONEPHRITIS
This acute, self-limiting glomerulonephritis is the classic example
collagen of the acute nephritic syndrome-the sudden onset of gross
hematuria, edema, hypertension, and renal insufficiency.
Acute poststreptococcal glomerulonephritis follows pha-
ryngeal or cutaneous infection with a nephritogenic strain of
Group A beta hemolytic streptococci (throat: serotype 12, skin:
serorype 49). During cold weather, it follows streptococcal
pharyngitis; during warm weather, it follows streptococcal skin
Alport syndrome infection or pyoderma. The latent period between streptococcal
infection and onset of nephritis is 2-3 weeks or longer. Attack
rate is 10-1570, second attacks are rare.
ESSENCE OF PEDIATRICS
Clinicol Feolures
Common in children, but rare before 3 years of age. Non-specific
symptoms such as fever, lethargy, and abdominal or flank pain
are common. May also present with asymptomatic microscopic
hematuria, nephrotic syndrome, or acure renal failure. The rypical
patient develops an acure nephritic syndrome.
The severiry of renal involvement may yary from asymptom-
atic microscopic hematuria with normal renal function to acute
renal failure. Depending on the severity, patients may develop
edema, hypertension, oliguria, heart failure, and encephalopa-
thy. The acute phase generally resolves within 2 months after
onset, but urinary abnormaliry may persist for > I year.
Diognosis
o Urinalysis: RBC, RBC casts, proteinuria
e Complete blood count: Polymorphonuclear leukocytosis,
normocytic normochromic anemia
o Serum Co level is reduced
o Evidence of streptococcal infection: Positive throat swab
culture, raised ASO titer, positive srreprozyme resr.
o Renal biopsy: Usuaily not required, may be done in atypi-
cal presentation, e.g. nephrotic syndrome, ARF, absence
of evidence of streptococcal infection, absence of hypoco-
mplementemia, or the persistence of marked hematuria or
proteinuria or both, or a low C, level for >3 months after
onset.
Treolmenl
No specific treatment, treatment is symptomatic and support-
ive. Mild cases (mild oliguria, normal blood pressure) require
only careful monitoring of blood pressure and fluid intake
and restriction of potassium intake. Diuretics are not routinely
indicated, since edema is rarely massive. A lO-day course of
penicillin is recommended to limit the spread of nephritogenic
Streptococcus; however, it does not affect the natural course
of glomerulonephritis.
The major life-threatening problems encountered during
the initial 1-2 weeks are due ro:
o Acute renal insufficiency: Fluid restriction ro an amounr
equal to insensible fluid loss (about 400 mllm2l d) plus previ-
ous day's urinary output, loss in stool, vomitus, and suction.
At least 400kcallm2ld in the form of carbohydrate and fat
are needed to minimize endogenous tissue catabolism. Total
60-100 kcal/kg/d or more can be given. Protein should be
restricted ro up to 0.5-1 glkgld.
r Electrolyte and acid-base abnormalities:
o Metabolic acidosis: It should be corrected by 8.4o/o
NaHCO, solution by using the foliowing formula: 0.3 x
Vt (kg) x (12 - sodium bicarbonate mEq/L) = amounr
of bicarbonate in mmol. In urgent situation where aci-
dosis is evident clinically, sodium bicarbonate can safely
be given in a dose of 3 mllkgldose; half of the amount
diluting with equal volume of fluid can be given through
slow IV, the remaining half can be put into the infusion
bag to be infused within 6-8 hours (1 ml of 8.4olo NaH-
CO, solution = 1 mEq of NaHCOo).
o Hyperhalemia: Rapid development of hyperkalemia
(serum level >6 mEq/L) may lead to cardiac arrhythmia,
death; patient should receive no potassium-containing
food, fluid or medications. If serum potassium ualue > 5.5
mEq/L, all solutions given to parient should contain high
concentrations of glucose. Sodium polysryrene sulfonate
resin (kayexalare) I g/kg may be given orally or by reten-
tion enema. For best result, the resin should be given
orally, suspended in 2 mllkg of 70o/o sorbitol. Resin
therapy may be repeated every 2 hour. If the serum
potassium rises to >7 mEq/L, in addition to kayexalate, the
following trearment should be given:
Calcium gluconate 10%o solution, 0.5 ml/kg IV slowly:
- The heart rate musr be closely monitored during the
infusion; a fall in the rate of 20 beats/min requires
stopping the infusion until the pulse rerurns ro pre-
infusion rare.
Sodium bicarbonate 7.5o/o solution, 2-3 ml/kg IV:
- Possible complications include volume expansion,
hypertension, terany.
Glucose 25o/o solution, 2 mllkgwith regular insuiin
- 1 unit/5 g of glucose, given IV over I hour: Patient
should be closely monitored for hypoglycemia.
,> Persistent lryperhalemia, especially in patienrs requiring
emergency measures, should be managed by dialysis (he-
modialysis or peritoneal dialysis-preferably peritoneal
dialysis).
'.; Hyponatrernia: Restriction of fluid alone is usually
enough, 3% sodium chloride solution can be used IV
over 15*60 minutes in an amount calculated to achieve
a halfcorrection ofserum sodium concentration (correc-
tion is started when Na. concenrrarion is <120 mEq/L).
Normal saline can also be used.
The formula used for calculation of total amount of
sodium (in mmol) required is: 0.6 x Wt in kg (125 , serum
sodium mEq/L). If serum sodium level falls acutely be-
low 120 mEq/L, the patient is at risk of cerebral edema
and CNS hemorrhage.
Acute hypertension: Diuretics and angiotensin converting
enzyme inhibitor (captopril 0.2-2 mglkgld) are used to
treat hyperrension.
r Hjpertensiae encepltalopatlty: Hypertensivc emergen-
cies shouid be treated with sublingual nifedipine (0.25-
0.5 mg/kg) or IV diazoxide (i-3 mg/kg, maximum 150
mg) or IV labetalol. \X{hen severe h;,pertension is associ-
ated with circulatory overload, frusemide 24 mglkg
may also be given. Seizure is controlled by diazepam
given IV or rectally. Phenobarbitone or phenytoin can be
used as maintenance therapy.
I
r
!
:
a RENAL DISEASES
L

L
, Intravenous administration is preferred in order to care- r A further one-third continues with proteinuria and hema-
l. Because too rapid fall in BP may
fully titrate the fall in BP turia with normal renal function.
Y
l' interfere with adequate organ perfusion, a stepwise reduc- o One-third will have a persistent severe nephrotic syndrome
ts tion of pressure should be planned. In general, pressure and progress to end-stage renal failure.
v should be reduced by about one-third ofthe total planned
l. reduction during the first 6 hour and the remaining over lgA NEPHROPATHY (BERGER DISEASE)
L
48J2 hour.
the following
v
,
o Left uennicular failure: The child should be propped Berger disease is one of the principal causes of the syndrome
up. Allow oxygen inhalation. Frusemide 5 mg/kg IV is of benign recurrent hematuria. It presents with recurrent bouts
I
I' efFective. Digitalization may be needed. Hypertension of hematuria, often precipitated by intercurrent viral infec-
, should be controlled by antihypertensive drugs, tions, with completely normal urine between attacks. Renal
I
biopsy shows focal proliferative glomerulonephritis or only very
, o Circulatory congestion: Restriction of sodium and fluid
intake, control of hypertension by antihypertensive drug, trivial changes, but immunofluorescence reveals mesangial IgA
v
I IV in immunoglobulin.
relief of congestion with frusemide, and refractory
v
cases, dialysis is needed.
. Tjreatment of scabies, if present: Activity need not be Treolment
r
restricted, except during the acute phase of the disease when o No specific treatment is available.
l the complications of acute renal failure may be present. o Corticosteroid may be of some value in patients with neph-
Family members of patients should be cultured for Group A rotic syndrome or in a rapidly progressive course.

I B-hemolytic streptococci and treated, if culture positive.

I Prognosis
I Prognosis
,
Complete recovery occurs in >95o/o of cases. Few percentage
may die due to complications like hypertensive encephalopathy,
CCF or ARF; few percentage may progress to CRF. Recur-
rences are extremely rare.
FOCAT SEGMENTAL GLOMERU TOSCLEROSIS
Focal segmental glomerulosclerosis (FSGS) is the most common
histological pattern found in steroid-resistant nephrotic syn-
drome in childhood. It is frequently associated with hema-
turia, renal impairment, hypertension, and poorly selective
proteinuria.
Treolmenl
o Tieatment is generally unrewarding.
o Prednisolone (60 mglm2ld until remission + 3
A minoriry of patients with FSGS will respond
nisolone at least initially.
o Cyclophosphamide (3 mglkgld for B weeks) will induce
remission in a small proportion of children with FSGS.
o Other therapies:
r Cyclosporine A is presently being evaluated.
; severe anemia, dehydration, edema, petechiae, hepatospleno-
Combination chemotherapy with vincristine, cyclophos-
phamide, and prednisolone is currently under trial.
Good. Onset in later life may be associated with progressive
renal impairment in a minoriry of cases.
HEMOTYTIC UREMIC SYNDROME
It is a heterogenous group of disorders characterized by the
triad of microangiopathic hemolltic anemia, thrombocltopenia,
and acute renal insufficiency-one of the common causes of
acute renal failure in young children.
Etiology
Gastroenteritis caused by an enterohemorrhagic E. coli, Shigella
dysenteriae rype 1, Salmonella, Campylobactor.
Less frequent causes: Other bacterial infections, viral infec-
tions, i.e., coxsackie, Echo, influenza, vaicella.
days).
Clinicol Feqlures
to pred-
Most common in children <4 years of age. Onset usually pre-
ceded by gastroenteritis (fever, vomiting, abdominal pain and
diarrhea, usually bloody) or less often by an upper respiratory
tract infection. After 5-10 days, sudden onset of severe pallor,
weakness, and oliguria occurs. Physical examination may reveal
megaly, and hypertension.

e Slmptomatic treatment for hypertension, edema, and pro-

lnvesligolions
qressive renal failure is needed.
Hematological examination:
Prognosis o Hemolytic anemia (Hb 5-8 g/dl)
r -{borlt one-third of children with FSGS will remit spontane- r Increased reticulocyte count, Coombs test negative
tr-u-rir-. although the time course may be prolonged. r Thrombocl'topenia (20,000-100,000/mm3)
 ESSENCE OF PEDIATRICS
o Blood film reveals helmet cells, blurr cells, and fragmented
RBCs.
r Partial thromboplastin time and prothrombin time are
usually normal, fibrin degradation products (FDP) increased,
\7BC count increased with left shifting (leukamoid
reaction).
Serum chemistry: BUN and crearinine levels are increased
(electrolyte imbalance, metabolic acidosis).
Urinalysis:
r Low-grade microscopic hematuria
o Proteinuria
r Cellular, granular, hyaline casts.
Complicolions
Anemia, acidosis, hyperkalemia, fluid overload, hypertension,
heart failure, uremia; CNS manifestarions include seizures,
thrombosis, coma; colitis (melena, perforations).
Treqlmenl
o Acute renal insufficiency:
o Fluid restrictions: Daily intake of fuid should include
insensible water loss 400 mllm2ld plus losses by vomit-
ing, gastric suction, stool, and urine if voided.
o Maintain calorie intake enterally or parenterally, 60-100
kcallkgld. Protein should be restricted to 0.5-1 glkgld.
o For management of complications, also see treatment
under acute renal failure.
Management of hematologic abnormalities: Fresh blood
transfusion (20 ml/kg) to mainrain hemoglobin concenrra-
tion around 8-9 gldl. For symptomatic bleeding, platelet
transfusion may be needed.
Antimicrobial agents: In Shigella infection, specific anti-
biotic nalidixic acid 55 mglkgld or pivmecillinam 40-60
mg/kg/d tds or qds for 5 days should be given.
o Nephrotic syndrome is characterized by massive proteinuria (> 1
Hemodialysis or peritoneal dialysis, if indicated.
Prognosis
90% of patients recover with normal renal function. Long-term
follow-up is necessary for late development of hypertension or
chronic renal diseases. Recurrence is rare.
Disorders of tubular function result in abnormal or inap,
propriate composition or volume of the final urine. In the
normal kidney, approximately 98o/o of the glomerular filtrate
is reabsorbed except for reduced protein levels; the ultrafiltrate
of blood that enters the proximal tubule is similar to plasma.
Body homeostasis is maintained by tubular reabsorption of
salts and water.
DISTAL RENAI. TUBULAR ACIDOSIS
Hydrogen ion homeostasis requires bicarbonate reabsorption in
the proximal tubule and the excretion of net hydrogen ion in the
distal renal tubule. Failure of either or both of these mechanisms
results in acidosis. The most common form is distal renal tubuiar
acidosis (typ. 1, classical). Mosr cases are sporadic, although
dominant and recessive forms have been described. Children
present between 2 and 4 years of age with failure to thrive,
rickets, and nephrocalcinosis. Hypokalemia and hyperchloremic
metabolic acidosis are found with urine pH always >6.
Treotment
Treatment consisrs of alkali 1-3 mmol/kg/d in divided doses.
Prognosis
Prognosis is dependent on the extent of nephrocalcinosis ar
diagnosis. If this is nor severe, the long-term prognosis can
be favourable.
PROXIMAT RENAL TUBUI.AR ACIDOSIS
Pure primary proximal renal tubular acidosis (rypr' 2) is very
rare and may be permanent or transient. It presents in infancy
with failure to rhrive, vomiting, and a hyperchloremic acidosis.
Urine acidificarion is normal when the plasma bicarbonate is
below threshold.
Treotmenl
Sodium bicarbonate (>10 mmol/kg/d) is required ro resrore
the plasma bicarbonate to normal. Prognosis is good.
glm2l24 hr), hypoalbuminemia (<25 glL), generalized edema,
hyperlipidem ia (>220 mg/dl).
Etiolog;r:
l Idiopathic nephrotic syndrome (90%-minimal change
nephrotic syndrome 85ol0, mesangial proliferation 5o/o,
focal sclerosis 10olo).
2. Remaining 10% include membranous nephropathy and
membranoproliferative glomerulonephritis
See Thble 10.1 for summary of primary renai diseases that
present as idiopathic nephrotic syndrome.
IDIOPATHIC NEPHROTIC SYNDROME
Among the three idiopathic nephrotic syndromes, minimal
change nephrotic syndrome (MCNS) is the commonest. It has
been reported rhat one of the three histological q.pes has been
{
lr
t
 RENAL DISEASES

Table 10.1: Summary of Primary Renal Diseases that Present as ldiopathic Nephrotic Syndrome

Frequency 75'/,, 10% <s'.h 10% 10%

Clinical mani{estation 2-6 210 40,50 .5-1 5 515
age group (yr)

Sex 2:1 male 1.3:1 male 2:1 male Male-female Male-{emale

Nephrotic syndrome 100% 90%o B0% 60"k 60%

Asymptomatic 0 10% 20"k 40% 40%

Protei nuria/Hematuria 10-20% 60*80,,/o 60"/" B0% B0%

Hypertension 10% 20'k early I nfrequent 35%

Rate of progression to Does not progress 10 yr 50% in 10-20 yr 1O-20 yr 5-.1 5 yr

renal failure
Associated conditions Al lergy, Hodgkin disease, None Renal vein thrombosis, None Partial
usually none cancer, SLE, hepatitis B lipodystrophy
Laboratory findings Normal C,, C* Normal Cr, C., Normal C,, C, Low C,, Cr, C., C,, Normal C,, Co,
low C,-C,
Renal pathology Light normal Focal sclerotic Thickned CBM, spikes Thickned CBM, Lobulation
microscopy lesions proliferation
lmmunofluorescence Negative lgM, C, in lesions Fine granular lgC, C, Cranular lgC, C.,

Electron microscopy Foot process fusion Foot process Subepithelial deposits Mesangial and C. only. Dense
fusion subendothel ial deposits
deposits
Response to steroids 90'a 15-200/" May be slow progression Not established Not established

transformed into another qrpe, which suggest that this syndrome o Serum albumin level <2.5 gldl
rnay be a single disorder with various histologicai features. o Serum C. level: Normal. Total serum calcium level is
decreased; total serum globulin level is normal or increased,
Clinicol Feqlures but albumin globuiin ratio is altred.
o Age and sex: more common o Indications of renal biopsy:
between 2 and 6 yr of age,
boys: girls = 2:1 r At onset
o Edema: Pirting edema, at first periorbital, then becomes Ag. of onset <1 year.
- Gross hematuria, persistent microscopic hematuria,
generalized, urinary output decreases. Fluid may accumulate
- or low serum C3.
in any serous sac (pleural effusion, ascites).
o Anorexia, abdominal pain, diarrhea are common, hyperten-
-
Sustained hyperrension.
rion and gross hemaruria are uncommon. Renal failure not attributable to hypovolen-ria.
-
o Prone to infection, e.g., peritonitis, UTI, cellulitis, sepsis
-
Suspected secondary causes of nephrotic syndrome .

& others. r After initial treatment

Plsteinuria persisting despite 4-rveeks of daily corti-
lnvesligolions - costeroid therapy.
Before treatrnent with cyclosporine A or tacroli-
For Diagnosis - mus.
o Urinalr.sis
, l\lassive proteinuria (+3 or +4) For Infection Screening and Others
,\licroscopic hematuria (20% cases)
Complete blood count, urine, blood and ascitic fluid culture
o Slot urine sample for protein to crearinine ratio >2 and sensitivity, S. electrolyte and S. calcium, chest x-ray,
o --:i:ran' roral protein (> I glm')l24 hr or 40 mg/m2/hr) ultrasonography of KUB region, HBsAg, VDRL, M1-, MB
r >-:-::r cholesrerol and triglyceride levels >250 mgldl ANA.
 ESSENCE OF PEDIATRICS
Definitions ond Terminology
Remission: Urine albumin nil or trace (or proreinuria
<4 mglm2lhr) for three consecutive early morning specimens.
Relapse Urine albumin 3+ or 4+ (or proteinuria >40 mglm2.lhr)
for three consecutive early morning specimens, having been in
remission previously.
Frequent relapses: Two or more relapses in initial 6 months,
or more than three relapses in any 12 months.
Steroid dependent Tivo consecutive relapses when on alternate
day steroids or within 14 days of its discontinuarion.
Steroid resistant: Absence of remission despite therapy with
4 weeks of daily prednisolone in a dose of 2 mglkgld.
Treolmenl
Supportive (are
Diet A balanced diet, adequate in protein (1.5-2 g/kg) and
calories is recommended. Patients with persistent proteinuria
should receive 2-2.5 glkgof protein daily. Not more than 30%o
calories should be derived from fat, and saturated fats should
be avoided. Salt restriction is not necessary in most patients
unless there is marked edema or ascites.
Fluid lntake Fluid intakeis neither restricted nor encouraged
unless the edema is severe.
Edema Diuretics are avoided unless edema is signifr,cant. Oral
frusemide (1-3 mg/kg daily), if persistent edema and weight
gain of 7-70o/o. Potassium-sparing diuretics, e.g., spironolactone
(2-4 mglkg daily), if higher doses and prolonged duration of
treatment with frusemide is required. Blood pressure should
be monitored frequently. Albumin (20%) 0.5-l g/kg over
24 hour foliowed by administration of frusemide (1-2 mg/kg
intravenously) for patients with refractory edema.
0ther Medications Antacids or histamine receptor antagonists
(e.g., ranitidine), if upper gastrointestinal discomfort. Long-
term calcium supplementation if the patient receives more than
3 months rrearment with prednisolone. Daily oral penicillin is
recommended for prophylaxis against pneumococcal infection.
Physical Activities As tolerated; may attend school.
SpecificTreatment
Prednisolone is the drug of choice (Fig. 10.1).
Treatment of lnitialAttack Prednisolone at a dose of 2 mglkgld
(maximum 60 mg in single or divided doses) for 6 weeks, fol-
lowed by 1.5 mg/kg (maximum 40 mg) as a single morning
dose on alternate days for the next 6 weeks; therapy is then
discontinued.
Treatment of Relapse Correction of infection with appropri-
ate therapy might rarely result in spontaneous remission,
E l
thereby avoiding rhe need for treatment with corticosteroids.
Prednisolone is administered at a dose of 2 mglkgld (single
or divided doses) undl urine protein is trace or nil for three
consecutive days. Subsequently, prednisolone is given in a
single morning dose of 1.5 mg/kg on alternate days for
4 weeks, and then discontinued. Patients showing no remission
despite 4 weeks' treatment with daily prednisolone should be
referred for evaluation.
Frequent Relapses (FRNS) and Steroid Dependence (SDNS) Follow-
ing treatment of a relapse, prednisolone is gradually tapered
to maintain the patient in remission on alternate day dose of
0.5-0.7 mg/kg, which is administered for 9-18 months. A
close monitoring of growth and blood pressure, and evaluation
for features of steroid toxicity is essential. If the prednisolone
threshold, to maintain remission, is higher or if features of
corticosteroid toxiciry are seen, additional use of the following
immunomodulators are suggested.
Levamisole: 2-2.5 mglkg on alternate days for 12-24
months. Co-treatment with prednisolone at a dose of 1.5
mg/kg on alternate days for 2-4 weeks; its dose is gradually
reduced by 0.15-0.25 mg/kg every 4 weeks to a maintenance
dose of 0.25-0.5 mg/kg that is continued for 6 or more
months. The chief side effect of levamisole is leukopenia;
fu-like symptoms, liver toxiciry convuisions, and skin rash
are rare. The leukocyte count should be monitored every
12-16 weeks.
Cyclophosphamide: 2-2.5 mgl kgl d for 12 weeks. Predniso-
lone is co-administered at a dose of 1.5 mg/kg on alternate
days for 4 weeks, followed by I mg/kg for the next 8 weeks;
steroid therapy is tapered and stopped over the next 2-3
months. Total leukocpe counts should be monitored every
2 weeks; treatment with cyclophosphamide is temporarily
discontinued if the count falls below 4000/mm3. Complica-
tions are hemorrhagic cystitis, alopecia, nausea and vomit-
ing, gonadal toxicity.
Cyclosporine (CsA): 4-5 mglkg daily for 12-24 monrhs.
Prednisolone is co-administered at a dose of 1.5 mg/kg on
alternate days for 2-4 weeks; its dose is gradually reduced
by 0.15* 0.25 mglkgevery 4 weeks to a maintenance dose
of 0.25-0.5 mg/kg that is continued for 6 or more months.
Side effects are hypertension, cosmetic symptoms (gum
hypertrophy, hirsutism) and nephrotoxicity; hypercholes-
terolemia and elevated transaminases may occur.
Thcrolimus: Is an alternative agent, 0.1-0.2 mg/kg daily
for 12-24 months. Side effects are hyperglycemia, diar-
rhea, and rarely neurotoxiciry (headache, seizures). Use of
tacrolimus is preferred especially in adolescents, because of
lack of cosmetic side effects. Blood levels of creatinine and
glucose should be estimated every 2-3 months.
Mycophenolate mofetil (MMD: 800-1200 mg/m2 along
with tapering doses of prednisolone for 12-24 months. Side
effects are gastrointestinal discomfort, diarrhea, and leukopenia.
Leukocyte counts should be monitored every l-2 months;
treatment is withheld if count falls below 4000/mm3.
I
1
 RENAL DISEASES

First Episode of Nephrotic Syndrome

Absence of hypertension, hematuria, azotemia

Prednisolone 2 mglkg daily for 6 weeks, followed by 1.5 mg/kg

on alternate days for 6 weeks.

Frequenl relapses
Steroid dependence

Prednisolone 2 mg/kg daily untit Refer for evaluation Refer for evaluation
remission, then 1.5 mg/kg on alternate Alternate day prednisolone to maintain Therapy based on renal
days for 4 weeks remission; assess steroid threshold biopsy findings

Threshold <0.5 mg/kg on Threshold >0.5 mg/kg on

alternate days alternate days or steroid toxicity

Alternate day prednisolone Levamisole, cyclophosphamide,

for 9-1 8 months tacrolimus mycophenolate
mofetil, cyclosporine

Fig. 10.1: Management of patients with steroid-sensitive nephrotic syndrome

IV methylprednisolone, cyclosporine A, chlorambucil, ACE immobilization) and decreased fibrinolytic factors (urinary
inhibitors may be tried in steroid-resistant cases. losses of antithrombin III, protein C & S).

Management of Edema Patients requiring high,dose frusemide

or addition of other diuretics should be under close supervi-
sion, preferably in a hospital (Fig. 10.2). Monitoring of serum
eiectrolytes is necessary in all patients receiving diuretics.
Patients showing hypokalemia require potassium supplements
or co-administration of spironolactone. The medications are
reduced stepwise once diuresis ensues.
Complicolions
o Infections: Major complication, e.g., SBP (by S. pneumoniae,
E. coli), sepsis, cellulitis, pneumonia, urinary tract infec-
tion, etc. occur due to urinary losses of immunoglobulins
and properdin factor B, defective ceil mediated immunity,
immunosuppressive therapy, malnutrition, edema/ascites
(potential culture media).
o Thromboembolic events: 2-5o/o; renal vein thrombosis,
pulmonary embolism, sagittal sinus thrombosis, thrombo-
sis of indrvelling arterial and venous carherers. The risk of
lhron-rbosis is related to increased prothrombotic factors
hbrinogen. thrombocltosis, hemoconcentration, relative
o Cardiovascular disease: Due to hyperlipidemia. MI is a
rare complication in children.
o Hlpovolemic shock: Due to unsupervised use of diuret-
ics, especiaily if accompanied by septicemia, diarrhea, or
vomiting.
Follow-up
o Urine examination for protein at home using dipstick or
heat coagulation test. The examination should be done every
morning during a relapse, during inrercurrenr infections or
if there is even mild periorbital puffiness. The frequency
of urine examination is reduced, to once or twice a week,
during remission.
Maintain a diary showing results of urine protein examina-
tion, medications received, and intercurrent infections.
Ensure normal activity and school attendance; the child
should continue to participate in all activities and sports.
Immunization: All children with nephrotic syndrome should
receive immunization against pneumococcal and variceila
infections. The vaccine should be given during remission, pref-
erably when the child is not receiving daily prednisolone.
ESSENCE OF PEDIATRICS

CTINICAL FEATURES
Yes*
Pyelonephritis (infection involving renal tissue) is character-
ized by any or all of the following: abdominal or flank pain
and tenderness, fever, chills, vomiting, jaundice (in neonate),
Frusemide 1-3 mg/kg/d
May add spironolactone 24 mglkg/d and occasionally diarrhea. Some newborns and infants may
show non-specific symptoms such as poor feeding, irritability,
No response (no weight loss or
and weight loss. If there is no parenchymal involvement, the
diuresis in 48 hr)
condition may be termed as pyelitis. Acute pyelonephritis may
Double dose of frusemide until diuresis
result in renal injury which is termed as pyelonephritic scarring.
or maximum daily dose of frusemide
(4-6 mg/kg/d) is reached Cystitis includes dysuria, urgency, frequency, suprapubic
pain, incontinence, and malodorous urine. Cystitis does not
No response cause fever and does not result in renal injury. Ifcystitis is not
treated, it may result in pyelonephritis.
Add hydrochlorothiazide 1 -2 mg/kg/d
or metolazone 0.1-0.3 mg/kg/d
Asymptomatic bacteriuria refers to children who have
positive urine culture without any manifestation of infection
and occurs almost exclusively in girls. This condition is benign
and does not cause renal injury.
Frusemide lV bolus 1-3 mg/kg/dose or
In recurrent UTI, day-time incontinence and often mani-
infusion 0.1 -1 mg/kg/hr
festations of bladder instabiliry may present.

20% Albumin 1 g/kg lV followed INVESTIGATIONS

by lV frusemide
o Urine culture and microscopy: Urine is collected by mid-
* Management o{ hypovolemia consists of rapid infusion of normal saline at a dose stream urine sample (first morning sample is better), sealed
ol 15 20 mL/kg over 20-30 minutes; this may be repeated if clinical leatures of sterile collection bag, catheterized specimen, suprapubic aspi-
hypovolemia persist. lnfusion of 5Yo albumin (10-1 5 mUkg) or 20% albumin
(0.5-1 g/kg) may be used in subjects who do not respond despite two boluses radon (in < I year children). Refer Thble I0.2 for diagnostic
of saline. significance of colony counts in urine specimens.

Fig. 10.2: Management of edema in patients with nephrotic

r Imaging of the urinary tract: Imaging of the urinary tract
is recommended for all children with UTI. The aim of these
syndrome.
investigations is to identi$, patients at risk of developing
renai damage, mainly those below 5 years of age, with VUR
Prognosis or urinary tract obstruction (Fig. 10.3).
Detailed evaluation with ultrasound, MCU, and renal
Prognosis of minimal change disease is satisfactory. Most of
scan are recommended for all children with recurrent UTI.
the steroid-responsive nephrotic syndrome cases have repeated
relapses until the disease resolves spontaneously towards the end ,r USG of KUB: Is usually done to exclude hydroneph-
of second decade of life (t4-20 year) with no residual renal rosis, structural urinary abnormalities, nephronia, and
dysfunction. Prognosis is guarded in steroid-resistant cases. pyonephrosis. It also may suggest acute pyelonephritis

Table 10.2: Diagnostic Significance of Colony Counts in

Urine Specimens

Urinary tract infection (UTI) is defined as the presence of

organisms within the urinary tract, growth of which is at least Clean voided 1 specimen >105 80
>105 colony forming units per ml. Infection usually occurs via
Suprapubic 3 specimen >1 0i 95
hematogenous or ascending route. Cram-negativebacilli: 99
There are three basic forms of UTl-pyelonephritis, cystitis, any number
and asymptomatic bacreriuria. Cram-positive cocci:
> a few thousand
UTIs are caused mainly by colonic bacteria. In females,
Catheterization >10,, 95
75-90o/o of all infections are caused by E. coli, followed by
10r 10; lnfection likely
Klebsiella and Proteus. In males older than 1 year of age' 10r 10r Repeat
Proteus is as common as E coli. Staphylococcus saPrzphyticus <1 0l lnfection unlikely
is a pathogen in both sexes. Adenovirus may also cause UTI, Modified irom UTI in Children, S Hellerstein (ed.) Chicago: Year Book Publishers,
especially cystitis. r 982.

First urinary
traet infection,
<2'years 2:-5 years >5 ){ears IVU, MCU
MCU and DMSAscan; no further and DMSA
evaluation scaR
DMSA scan MCU il scar
on DMSA
Fig. 10.3: lnvestigations to identify patients (mainly those below
5 years of age) at risk of developing renal damage.
by demonstrating an enlarge kidney and many but not
all renal scars.
o Voiding cystourethrogram (VCUG): The most com-
mon finding is vesicoureteral reflux (identified in 40o/o of
patients). Indications of VCUG are febrile UTI,2 ot 3
UTIs within 6 months in girls and more than one UTI in
boys; if renal sonogram shows any significant abnormal-
ity such as hydronephrosis, disparity in renal length or
bladder wall thickening should be suspected.
o DMSA (dimercaptosuccinic acid) scan: It is done in
VUR to assess the degree of scarring and suspicion of
acute pyelonephritis. In approximately 50o/o of children
with a febrile UTI, irrespective of age, the DMSA scan
demonstrate parenchymal involvement. Among children
with grade III, IV orV reflux and a febrile UTI' 80-90%
show acute pylonephritis.
o DTPA. It helps to differentiate obstruction from dilata-
tion. It is an excellent agent for visualizing the pelvica-
lyceal collecting system and ureters; it can confirm ob-
struction at pelviureteric junction and can assess GFR.
r CT scanning: It is used to evaluate the upper urinary
tract in demonstrating renal scarring.
RISK FACTORS FOR URINARY TRACT
INFECTION
Femaie gender, uncircumcised male, vesicoureteral reflux,
voiding dysfunction, obstructive uropathy, urethral instrumen-
rarion, rviping from back to front in females, tight clothing
tundenvear), pinworm infestation, constipation, anatomic
abnormaliry (lablal adhesion), neuropathic bladder, sexual
actir-in', and pregnancy.
RENAL DISEASES
TREATMENT
Increasing bacterial resistance has limited the usefulness of
some antibiotics, such as amoxicillin. TMP-SMZ is used
frequently, although resistance to this drug also is increasing.
Oral third-generation cephalosporins (cefixime, cefpodoxime)
are effective but expensive. Children with high fever or other
manifestations of acute pyelonephritis often are hospitalized
for initial treatment with parenteral antibiotics, such as cefo-
taxime. Patients with systemic toxicity (chills and high fever)
should be hospitalized and treated with IV cefotaxime and
tX4ren the patient has
gentamicin (or another aminoglycoside).
improved and is afebrile, oral therapy with an agent to which
the cultured organism is sensitive is administered to complete
7-I4 days of total therapy.
The degree of toxiciry dehydration, and abiliry to retain
oral intake of fluids should be assessed carefully. Restoring or
maintaining adequate hydration, including correction of elec-
trolyte abnormalities that are often associated with vomiting
or poor ora.l inrake, is important'
Infants and children who do not show the expected clinical
response within 2 days of starting antimicrobial therapy should
be re-evaluated, have another urine specimen obtained for
culture, and undergo ultrasound promptly and either VCUG
or radionuclide cystography, which should be performed at
the eariiest convenient time.
See Fig. 10.4 for the management of the first urinary tract
infection and Table 10.3 for the list of drugs commonly used
in the treatment of UTIs.
Note:
r Empirical therapy: For symptomatic children and for all
children with a urine culture confirming UTI.
o Parenteral or oral antibiotic: For a child who does not
appear ill but with a posirive urine culture.
o Hospitaliz tionand parenteral antibiotic: For a child with
,,rrp..t.d UTI who appears toxic, appears dehydrated, or
is unable to retain oral fluids.
o Neonates: Parenteral antibiotics for 10-14 days.
o Older children with acute cystitis: Oral antibiotic for
7-14 days.
COMPLICATIONS
Bacteremia occurs in 2-5o/o of episodes of pyelonephritis and
is more likely in infants than in older children. Focal renal
abscesses are an uncommon complication.
PROGNOSIS
The relapse rate of UTI is approximately 2540o/o, with most
relapses occurring within 2-3 weeks of treatment' Follow-up
,rrine cultures should be obtained 1-2 weeks after completing
therapy.
 ESSENCE OF PEDIATRICS

Prophylactic antibiotics should be administered until the 'llable 10.3: Drugs Commonly Used for Treatment of UTI
VCUG has been completed, and the presence of refux is
known. TMP-SMZ (2 mglkg TMB tO mg/kg SMZ) and
Co-trimoxazole 6-8 mg of trimethoprim
nitrofurantoin (1*2 mg/kg) given once daily at bedtime are 2

recommended as prophylactic agents. Clinical follow-up for Amoxicillin, 30-50 mg 3

at least 2-3 years, with repeat urine cuhure as indicated, is Cefaclor )O-4'l mo 3

prudent. Nalidixic acidr 50 mg 3

Follow-up urine cultures should be obtained afrer recurrenr Ciprofloxacinr 10-l5 mg 2
cystitis or pyelonephritis, monthly for 3 months, at 3-month Centamicina 5-7.5 me 2-3
intervals for 6 months, rhen yearly for 2-3 years. Grade I to
Cephalexin 50-70 mg 3
3 reflux resolves at a rare of about 73o/o per year for the first
Cephradine 50 mg 2-4
5 years, then at a rate of 3.5o/o per year. Grade 4 to 5 reflix
Cefixime B-10 mg 2
resolves at a rate ofabout 5Vo per year. Bilateral reflux resolves
Amikacina 15 mg
more slowly than unilateral refux. 2

Cefotaxime4 100 mg 2-3

PREVENTION Ceftriaxonea /J mg 1

1. Dose adjustment required in renal insufficiency.

2. Amoxicillin may be used alone or in conrbination wjth clavulanic acid.
Primary preventionis achieved by promoting good perineal
3. Not to be used as first-line drugs.
hygiene and managing underlying risk factors for UTI, such 4. Parenteral use for pyelonephritis.

Sick in{ant or child (e.9., fever, vomiting, weight loss, or
urinary symptoms)
Collect urine Jor culture and microscopy (leukocytes
>S/HPF in a centrifuged sample & >10/HpF in
uncentrifuged sample is significant).
Start treatment with broad-spectrum antibiotic (see Table
10.3) lor 7-10 days (oral or intravenous route, depending
on age and clinical condition).
as chronic consripation, encopresis, and day-time and night-
time urinary inconrinence. Secondary prevention of UTI with
antibiotic prophyla-ris given once daily is directed toward pre-
venting recLlrrenr infections, although the impact of secondary
prophyla-ris ro prevenr renal scarring is unknown.
RECURRENT UTI
Organisms causing recurrenr UTI are E. coli, proteus, Kiebsiella,
Pseudomonas, Staphylococcus.
Risk Foclors
o Organic
o Vesicoureteral reflux

Change route of administration Jrom intravenous to oral

o Obstruction: Congenital bladder neck obstrucrion, pos-
when fever settled {or 24 hours, change drug according terior urethral valve, phimosis
to sensitivity al24-48 hours if necessary. lf fever persists r Hydronephrosis
for over 36 hours, review route of administration, review c Megacystis megaureter
sensitivity of organism, and arrange urgent ultrasound.
o Associated disorders that impair defense mechanism:
Juve-
nile diabetes mellitus, consripation, inadequate voiding.

Start prophylaxis (i.e., trimethoprim 1-2 mg/kg, nitrofurantoin Treqlmenl

1-2 mg/kg) as soon as treatment completed.
o The above mentioned
lnvestigate according to age of child
Diecontinue prophylaxis if all tests are normal and
i Vesicoureteric reflux has been excluded.
Fig. 10.4: Managernent of the first urinary tract infection
risk factors should be looked for
and treated.
Medical trearmenr: Treatment should be started empirically
as described earlier or changed according to culture and
sensitivity or clinical response.
Prophylactic trearmenr against re-infection should be
started rvith cotrimoxazole, nitrofurantoin or cephalexin for
6 months to 2 years (also described under VUR) at
one-third to one-forth of the normal therapeutic dose nec-
essarv ro treat an acute infection once a day at bed time.

@
 RENAL DISEASES

o Counseling of theparents and patient about frequent Clinicql Feotures

complete voiding (3-4 hourly), double micturition at bed
The child may have recurrent UTI with or without a structural
time, more fluid intake, avoidance of constipation, regular
anomaly of the urinary tract. Those with reflux nephropathy
treatment of enterobiasis, circumcision.
and marked renal parenchymal scarring may present with
acidosis, hypertension, or renai failure.
VESICOURETERIC REFTUX
lnvesligolions
Vesicoureteric reflux (VUR) is the retrograde flow of urine
from the bladder to the ureter and renal pelvis. \rUR is the o Urine culture and microscopy
commonest abnormality of the urinary tract found in children o USG of KUB
investigated for UTI and seen in 20-35o/o of cases. In up to o Micturating cystourethrogram (MCUG) with or without
80% of children, it disappears spontaneouslywith time. More IVU
severe grades are associated with significant ureteral and renal o DMSA, DTPA
pelvic dilatation, and high grades of VUR have significant risk
of renal scarring. Treqlmenl
\rUR may be familial and evaluation of siblings younger Medical: Continuous antibiotic prophylaxis is recommended
than 5 years is recommended. as the initial treatment in almost all children with \rUR (see
Thble 10.4 for drugs and dosages). Infected VUR can almost
Clqssificqtion entirely be prevented; normal renal growth without scarring
WR may be primary or secondary. can be anticipated. Chemoprophylaxis is continued as long
as VUR is present. In most cases, reflux stops naturally at
sometime during childhood.
Primary Congenital incompetence of
the vulvular met hanism of lhe Surgical: Operative correction of \rUR is indicated in patients
vesicoureleral iunt tion with (;) persistent severe (grade IV or V) reflux, (ii) noncom-
Primary associated with Ureteral dupl ication. Utreterocele pliance or intolerance to medication, (iii) aPpearance of new
other malformations of the with duplication. Ureteral ectopia renal scars or deterioration of renal function during medical
ureterovesical junction therapy, and (iu) multiple recurrent UTI despite prophylaxis.
Secondary to increased NeLrropathit bladder. Bladder Standard surgical procedures of ureteric re-implantation have
intravesical pressure outlet obstruction
cure rate of 95-97%.
Secondary to inflammatory Severe bacterial cystitis. Foreign General measures have been written earlier under recur-
process bodies. Vesical calculi.
renr uTI.
Secondary to surgical procedures
involving the urelerovesica I
iu ncl ion
Follow-up
Foilow-up up to adulthood is an essential component in the
Refux severity is graded using the international study clas- management of all children with VUR, with or without renal
sification of I to V (given below) and is based on appearance scarring. It includes assessment of somatic and renal growth,
of the urinary tract on a contrast voiding cystourethrogram. and regular urine culture, testing ofrenal function, and regular
Grading recording of blood Pressure. Investigations and imaging should
be done rwice a year.
Grade I : Refux into a non-dilated ureter
Grade II : Reflux into the upper collecting system without
Table 10.4: Antibiotics for Long-Term Prophylaxis against
dilation
Vesicoureteral Reflux
Grade III : Reflux into dilated ureter and for blunting of the
calyceai fornices
Grade IV : Reflux into a grossly dilated ureter Co-trimoxazole 1-2 mg of Mai ntain adequate fluid
trimethoPrim intake; avoid in infants under
Grade V : Massive reflux, with significant ureteral dilatation
6 weeks
and tortuosity and loss of papillary impressions
Nitrofurantoin 1 2 Considerable Cl uPset.
\r-R is important it
encourages infection of the
because Contraindicated in
C6PD deficiencY, infants
u:inan. tract rvhen refluxed urine returns to and remains in <3 months, and renal
::. bladder after micturition. It also provides a direct route insufficiency. Resistance rare
:c: rhe transmission of both organisms and bladder pressure Cephalexin l0 May be used in Young infants.
:: :he renal papillae and could give rise to pyelonephritis and Trimethoprim 1-2 Reasonably safe.
.,,-:.equent renal scarring.
 ESSENCE OF PEDIATRICS

o Clinical features may suggesr cause. History of fluid or

blood loss with severe dehydration (acute tubular necrosis);
Acute renai failure (ARF) denotes an acute redu.ction of renal edema, hematuria and hypertension (AGN); dysentery,
function leading to retention of nitrogenous waste products pallor and petechiae (HUS); a history of sudden pallor,
(urea, creatinine) and a disturbance of warer, electrolyte, and passage of dark red urine and .jaundice (acute intravascular
acid-base balance (notably hyperkalemia, rnetabolic acido- hemolysis); a history of interrupted urinary stream and a
sis). Oliguria (urine volume <400 mi/m,/d) or anuria is the palpable bladder or kidney suggesr an obsrructive uroparhy,
prominent feature. Causes of AR.F are classified as prerenal, and that of abdominal colic, hematuria and dysuria suggesr
renal, and postrenal, which are listed in Table 10.5. Facrors a urinary tract calculus or infection.
suggesrive of ARF are iisted in'lable 10.6, which lists factors
differentiating ARF from chronic renal failure. INVESTIGATIONS
a Urine R/E, CBC, and PBF
CtINICAt FEATURES
a Blood urea, S. creatinine, creatinine clearance test
Pallor, diminished urine ourpur (oliguria) or anuria, edema a S. electroiytes, blood pH and bicarbonate, calcium, and
(salt and water overload). Hypertension, vomiting, lethargy phosphate levels
(uremic encephalopathy). a ECG (for evidence of K- roxicity)
Features of complication of ARF include volume overload a Chest x-ray (P/A): For pulmonary plethora and cardiac
with heart failure and pulmonary edema, arrhythmia, gasrro- enlargement
intestinal bleeding due to stress ulcers or gastritis, seizures, a X-ray abdomen
coma, and behavioral changes. a USG: Allows visualization of pelvicalyceal system, and assess-
ment of the renal size, structural anomalies, and calculi.
ASO and throat culture for Streptococcus, streptococcal
antibodies
Table 10.5; Causes of Acute Renal Failure a Serum complement (C.)
a MCUG, IVU, DTPA, or DMSA renai scan
Hypovolemia C lomeru lonephritis Obstructive uropathy a Renal biopsy
Hemorrhage Poststreptococcal Obstruction at
Castrointestina I Lupus erythematosus ureteropelvic junction TREATMENT
losses U reterocele
Membra noprol iferative
Hypoproteinemia ldiopathic rapidly Urethral valves l. General therapeutic measures: If the patient is dehy-
lebflte rntecttons progressive Vesicoureteral reflux drated, he/she should be rehydrated immediately with
with inadequate 57o dextrose in normal saline.
-Vhen
Anaphylactoid Acquired: stones ARF is due to hypo-
fluid intake purpura volemia, an increase in urine output may be anticipated
Hypotension following rehydration. If an increase in urine ourpur does
Localized i ntravascu lar
Seoticemia coagulation not follow correcrion of volume depletion, frusemide
Disseminated Renal vein thrombosis 2 mglkgslowly IV may be given. If no response occurs, a
intravascular Hemolytic uremic second dose (10 mg/kg IV) should be given. If circulatory
coagulation syndrome congesrion is nor present, 1 g/kg of 20o/o mannitol IV
Heart failure
Acute tubular necrosis can be given for the same purpose. Mannitol should not
Hypoxia
Heavv metais
Pneumonia
Drugs
Respiratory
Hemoglobin,
Table 10.6: Factors Suggestive of Acute Versus Chronic Renal
distress Fai I ure
myoglobin
syn0rome
Shock
Cystic disease
Ischemia History: related to Poor grorvth {height and weight should be
Acute interstitial CAUSE measured)
nepnntrs Previouslv healthy Family history of renal disease
lniection Oliguria or anuria Chronicalll ill appearance, polyuria. noLturia,
Tumors ac ido: i>

Renal parenchymal Hematuria Previous abnormal urine or hypertension

infiltration Edema Edema rif terminalr
Developmental
Large kidneys Hypertensive reti nopathy
abnormalities
Bone disease, featu res of hyperparathyroidi sm
Hypoplasia dysplasia Smallkidneys

3
I

be given to a patient in cardiac failure, nor frusemide to
one who is hypovolemic.
a. Fluid: Daily requirement of fluid should include
insensible water loss (400 mllmzld) plus losses by
vomiting, gastric suction, stool and urine (if voided).
Fluid balance should be maintained strictly. Urea,
creatinine, electroiytes should be monitored daily.
b. Calorie: 60-100 kcal/kg/d (one should try to give
maximum tolerable amount of calorie to the patient).
Allocation of calorie should come 60-700/o from car-
bohydrate, l0-l5o/o from protein, and25-30o/o from
fat. Protein should be restricted to 0.5-1.0 glkgl24
hour (1 tsp of milk powder contains 1 gm protein);
carbohydrates and fats can be given liberally, low-
protein diet should be allowed.
c. Mode of feeding: Total amount of daily fuid and
calorie requirements should be divided by the total
number of feeds (4-10 feeds/24 hour) to be given.
Required calorie for each feed can be calculated from
milk (1 tsp = 20 kcal), sugar (1 tsp = 20 kcal), and
soyabean oil (1 tsp = 45 kcal), and added to the
amount of fluid given in each feed. Feeding can be
given orally or through nasogastric tube. In case of
frequent vomiting, 10olo dextrose in normal saline can
be given IV initially for a few days.
", Electrolytes, acid-base balance:
a. Metabolic acidosis: Can be corrected by treating
shock, infection, hypoxia, and by provision of
adequate calorie intake. it should be treated by IV
sodibicarb (dose is mentioned eisewhere) carefully,
otherwise the latter might expand the extracellular
fluid volume, and might precipitate heart failure.
Severe acidosis can be corrected by dialysis.
b. Hyperkalemia: Schedule of treatment is described
elsewhere (see treatment of acute post-streptococcal
glomerulonephritis). Infection, acidosis, hemolysis,
etc. may aggravate the condition, so these should be
treated adequately.
c. Hypocalcemia: teated by aphosphate binding
calcium carbonate or gluconate 30-50 mglkgld.
3. Hypertension, cardiac failure and fluid overload:
a. BP should be reduced to a safe level (45o/o ofexpected
normal), without causing it to fall precipitously and
produce cerebral ischemia. teatment of hypertension
has been described earlier under GN.
b. For cardiac failure, see Chapter 9, "Cardiovascular
Diseases".
c. Fluid ovedoad: Very common, characterized by
increased J\?, peripheral edema, hepatomegaly, pul-
monary edema, tachycardia; heart failure may also
develop. Both of these conditions can be managed
rvell by fluid restriction and IV frusemide and aiso by
dialysis (in refractory cases) using hypertonic dialysate
solution (3.8o/o or more).
RENAL DISEASES
4. Anemia: Severe anemia can be corrected by packed cells
3-5 ml/kg.
6. lnfection: Prophylactic antibiotic is not advised. Various
infections (ARI, UTI, septicemia, peritonitis, etc.) should
be treated with antibiotics; nephrotoxic antibiotics should
be avoided.
7. GI Bleeding: May be prevented with calcium carbonate
antacid, which also lowers the serum phosphorus level.
IV ranitidine may be administered at a dose of 5-10
mg/kg/12 hour.
8. Seizure: Diazepam and treatment of underlying cause.
9. Dialysis: Peritoneal or hemodialysis when indicated. Indi-
cations commonly inciude severe acidosis, hyperkalemia,
fluid overload, CCB CNS disturbances.
10. Treatment of cause, when identified.
PROGNOSIS
In general, recovery is likely in cases of prerenal causes, hemo-
lytic uremic syndrome, acute tubular necrosis, acute interstitial
nephritis, or uric acid nephropathy. Recovery is unlikely in
rapidly progressive GN, bilateral renal vein thrombosis, or
bilareral cortical necrosis.
Chronic kidney disease (CKD) implies persistent renal damage
an or decreased GFR with a risk of developing progressive loss
of renal function. It is usually of gradual onset, and there
may be no symptoms in the early stages (see Table L0.7 for
stages of CKD). eKD signifies structural or functional abnor-
malities of the kidneys for >3 months, as manifested by either
(a) kidney damage with or without decreased GFR or (r) GFR
<60 ml/min/1.73 m2 with or without kidney damage.
CAUSES
Congenital nephropathies and glomerulonephritis are the
commonest causes.
Congenital disorders:
o Malformations: Renal hypoplasia, dysplasia, obstructive
uropathy (posterior urethral valve), neurogenic bladder.
Table 10.7: Stages of Chronic Kidney Disease
'l
Kidney damage with normal CFR >90
2 Kidney damage with mildly 60-89
decreased CFR
3 Moderately decreased CFR 30-59
4 Severely decreased CFR 15-29
5 Kidney iailure (end-stage renal disease) <15
ESSENCE OF PEDIATRICS

o Hereditary disorders: Polycystic kldney disease, Alport syn, Table 10.9: Pathophysiology of CKD
drome, congenital nephrotic syndrome.

Acquired disorders: Accumulation of Decline in glomerular filtration rate

nitrogenous waste
o Glomerulonephritis: Focal segmental glomerulosclerosis, products (azotemia)
crescentic GN, membranoproliferative GN. Acrdosrs Urinary bicarbonate wasting
o Vascular nephropathy: Hemolytic uremic syndrome. Decreased ammonia excretion
o Non-obstructive pyelonephritis with or without vesi- Det reased acid excretion
coureteric reflux. Sodium wasting Solute diuresis
o Miscellaneous: Intersritial nephritis, reflux nephropathy. lubular damage
Functional tubular adaptation for sodium
excretion
CtINICAI. FEATURES Sodium retention Nephrotic syndrome
Congestive heart failure
Patient may remain asympromaric until GFR is 20 ml/min/m2 Anuria
or less (normal 80-120 ml/min). Excessive salt intake
Frequent passage ofurine, nocruria and increased thirst (due Urinary concentrating Nephron loss
to loss of urinary concenrraring abiliry), pallor (normocltic defect Solute diuresis
lncreased medullary blood flow
normochromic), hypertension, anorexia, malnutrition, skeletal
Hyperkalemia Decline in glomerular filtration rate
deformities (rickets and genu valgum), growth retardation,
Aciclosis
tiredness or breathlessness, deep respiration (Kussmaul res-
Excessive potassium intake
piration), hiccough, pruritus, vomiting, muscular twitching, Hypoaldosteron ism
fits, drowsiness, and coma ensue. See Thble 10.8 for clinical Renal osteodystrophy Decreased intestinal absorption o{ Vit D
assessment of the etiology of CKD and Thble 10.9 for the
Crowth retardation Prolei n-energy defic iency
pathophysiology of CKD. Renal osteodystrophy
Acidosis
Anemia
INVESTIGATIONS
Bleeding tendency Thrombocytopen ia
Defective platelet function
Important investigations for CKD include crearinine clearance
test to assess glomeruiar function, and specific gravity and/or Infection Defective granulocyte function
lmpaired cellular immune {unction
urinary osmolality ro assess tubular functions. For assessment
Pericarditis and Unknown
of both glomerular and tubular functions, IVU can be done.
cardiomyopathy
Other investigations include:
o Urine zuE & C/S; CBC
o Blood urea, S. crearinine, blood pH Reduced GFR
r Serum electrolytes, alkaline phosphatase, serum calcium, Or
phosphate, PTH Proteinuria, Hematuria
a S. albumin, total protein, C3, C4, ANA
a ECG
a X-ray of hands, knee pelvis and spines
a IVU, DTPA
Figure 10.5 depicts the algorithmic approach to the evalua-

ry
tion of CKD.
llll:i'"". $ ;gn:ffi"r'i';,. $ No''"rr<ionevs $

Table 10.8: Clinical Assessment of the Etiology of CKD

Recurrent fever, dysuria, pyuria Reflux nephropathy

Poor urinary stream, urinary Obstructive uropathy
retention
lncontinence Neurogenic bladder Reflux ! Neurogenic Chronic GN g
*
nephropathyI bladder,
Polyuria, polydipsia, acidosis Tubulointerstitial disease # Oostructtve
Positive fami ly history Alport syndrome, polycystic
kidney disease
Edema, hematuria, hypertension Clomeru lonephritis
Fig. 10.5: Algorithmic approach to the evaluation of CKD.

TREATMENT
l. Nutrition: The goal of dietary managemenr is to maximize
calorie intake, reduce excretory solute load, and preserve
residual renal function. The diet should be high in car-
bohydrates and fat, with limited protein (of high biologic
value) (1-2 glkgld) and phosphate intake. Protein resrric-
tion may produce protein energy malnutrition (PEM),
hence is not recommended. CKD patients are able to
maintain water and sodium balance until end-stage renal
failure.
", Growth: Most children who have CKD grow poorly due to
a number of factors, including inadequate calories, acidosis,
anemia, and renal osteodystrophy. Recent studies involving
the use of recombinant human growth hormone (GH) have
been very encouraging and demonstrate increased growrh
rates without significant side effects. The dose of GH is
0.05 ng/kg/d 6-7 days a week.
3. Renal osteodystrophy results from several factors, includ-
ing impaired vitamin D metabolism, decreased intesti-
nal calcium absorption, phosphate rerenrion leading to
hyperphosphatemia, secondary hyperparathyroidism, and
metabolic acidosis. The combination of skeletal demin-
eralization and hyperparathyroidism retards growth and
leads to rickets.
a. Therapy with vitamin D (1,25 dihydroxy vitamin Dr)
prevents the skeletal abnormalities.
b. Calcium carbonate is given to provide extra calcium
for absorption and act as a binder ofdietary phosphate
and to suppress hyperparathyroidism.
c. To prevent hyperphosphatemia, it is often necessary to
limit phosphorus intake and give phosphate binders;
calcium carbonate can be given with meals.
4. Hyperkalemia: Dietary potassium limitation is usually
not needed until the GFR falls to very low levels (below
5 mllminll.T3 m2). Potassium balance is maintained in
chronic renal failure by increased excretion from remaining
nephrons and significant intestinal potassium loss. A potas-
sium binding resin (e.g., sodium polystyrene sulphonate)
may be given orally or rectally to remove potassium.
5. Hypertension: Hypertension in CKD often results from
fluid overload. Some patients remain hypertensive despite
salt restriction and diuretics and may require treatment
with ACE inhibitors (enalapril), beta-blocker (atenolol),
calcium channel blocker (nifedipine, amlodipine). ACE
inhibitors may help in retarding the progression of CKD;
however, they should be cautiously used as they may cause
reduction in GFR and hyperkalemia.
a. Nifedipine 0.25-0.50 mg/kg orally may be given
carefully in hypertensive emergency.
b. Hrpertension with circulatory overload is treated by
frusemide (1-4 mglkgl24 hours).
6. Metabolic acidosis: It should be corrected with sodium
bicarbonate (2-3 mEglkS. The dose should be titrated to
maintah a serurn bicarbonate level ofaround 1G18 mEq/L.
RENAL DISEASES
7. Anemie Normochromic, normocytic anemia is common
in chronic renal failure for several reasons:
a. Decreased erythropoiesis results from decreased
erythropoietin levels and uremic inhibition of bone
marfow.
b. Shortened er;.throclte survival results from the uremic
state and damage caused by hemodialysis.
c. Gastrointestinal blood loss results from the uremic
bleeding diathesis.
d. Nutritional anemia may result from iron or folate
deficiency or both.
Despite the availability, efFectiveness, and widespread use
of human recombinant erythropoietin (50-150 U/kg/dose
thrice a week SC), anemia remains common, generally the
result of iron deficiency. Thus, oral iron supplementarion
2-6 mglkgld is indicated. Blood transfusion (10 ml/kg
of packed cells, when Hb level is <6 g/dl) is reserved for
severe anemia.
8. UTI, ARI, or other infections are common in CRF.
Non-nephrotoxic antibiotics should be used in such
infections.
9, End-stage renal disease (ESRD), when the progression
of chronic renal failure is no ionger adequately managed
by medica.l means, replacement therapy is required, using
hemodialysis, a peritoneal dialysis regimen, or transplan-
tation (living related donor, cadaveric donor). An adult
kidney can be transplanted to a child.
Peritoneol Diolysis vs. Hemodiolysis
Peritoneal dialysis:
o More easily carried out in children
o May be done entirely at home; proximity to dialysis center
not required
o Less expensive
o Peritoneal cavity access easier to achieve and maintain
o Less restriction of dietary intake and physical activity
o Less risk of disequilibrium (i.e., headache, nausea, cramps
and seizures resuldng from osmolar shifts)
Hemodialysis:
r More efficient form of dialysis
o Carried out by nursing staff, does not require participation
of family
r Less chance of fatigue (burn out) for child and family
The peritoneum is a semi-permeable membrane that allows
difirsion of water and solutes along concentration gradient.
If the concentration of a substance is greater in the plasma
than in the dialyzing fluid, there will be a net transfer of the
substance from plasma into the dialying fluid. This transfer
depends on:
ESSENCE OF PEDIATRICS

o Difference berween the concentrations of substances on rwo
sides of membrane (i.e., peritoneal membrane).
r Molecular size: Smaller molecules diffusing more rapidly
than larger one.
o The length of time that the blood and the fluid remain in
contact with the membrane.
Few points to be noted:
ARF. For peritoneal dialysis, currenrly three clinical techniques,
which utiiize the peritoneal membrane, are in use:
1. Manual PD: Requires manual closing and opening of
flow controls and needs close and consrant supervision by
the trained staff. Acute peritoneal dialysis or conrinuous
equilibration peritoneal dialysis (CEPD) can be done in
the management of acute renal failure .

r In the uremic plasma, K* concenrration 2. Continued ambulatory peritoneal dialysis (CAPD), inter-
is higher. So this
mittent peritoneai dialysis (IPD), and conrinuous cyclic
ion diffuses rapidiy through the peritoneal membrane from
peritoneal dialysis (CCPD) are commonly done.
plasma, so rapidly that its concenrration falis to equai to
the dialyzing fluid within 3-4 hours of exposure.
o On the other hand, there is no urea, creatinine, urare, PROCEDURE
PO4, SO1 in the dialyzing fluid, but they are presenr in
1. It should be done under all asepric precaurions. First warm'
high concentration in the uremic plasma. Therefore, when
the dialysis fluid (1.5%) to body temperature (37"C) by
uremic patient is dialyzed, these substances are lost in large
immersing the bag in warm water bowl. Now, bag should
quantities into the dialyzing fluid in the peritoneal cavity,
be prepared by adding to it Inj. heparin 500-1000 unitsl',
from there to outside the body via drainage tube, thereby
inj gentamicin 5 mg' or inj ampicillin 25 mgil of {iuid.
removing major proportion of them from plasma.
o Inj. potassium chloride 2 mEqlL is r,rsually added from
Thus, constiruenrs of dialyzing fluid are chosen, so rhat
6th bag onwards, or when serum potassium is <4 mEq/L.
these substances which are excess in extracellular fuid
Suspend the prepared bag in the saline stand. Then attach
(ECF) can be removed at rapid rates, while the normal
the administration ser and suspend the drainage bag by
electrolytes remain essenrially normal. See Table 10.10 for
the side of the bed. Fill rhe administration set with fluid
comparison of constituents in normal plasma, dialyzing
and check the flow controls.
fluid, and uremic plasma.
2. Preparation of the patient: It should be ensured before-
Both peritoneal dialysis (simple, safe, and effective) and hemodi- hand that bladder is empty. Then clean the abdomen r.vith
alysis (not simple, but more effective than PD) can be done in iodine or betadine ancl place the stelile abdominal clraw
sheet. Choose a point in the midline over linea alba one-
third the way from umbilicus (or over the left llank just
Table 10.10: Comparison of Constituents in Normal PIasma, beyond the margin of the rectus abdominis in the line of
Dialyzing Fluid, and Uremic Plasma umbilicus). Infiltrate the skin with local anesrhetic and
then infiltrate the peritoneum. Now, a small rransverse
stab incision is made in the skin by a surgical blade just
Electrolytes (mEq/L) to pass the catheter. If incision is big, then stitches may
Na* 142 141 142 become necessary.
Kr5 0 7
J. The Y connector should then be taken out from the sterile
cl- 107
PD set. Attach the long arm of the elbow bent with the
101 107
administration set, and keep short arm ready to attach
cHrcooHcor 27 45 14
to the catheter. Another arm of the connector is then
Ca*t 3 3.5 2
attached through tubing to the drainage bag.
HPO- 3 0 9 4. Now insert the stylet into the catheter ro rhe ma-rimum
Mg-- 1.5 1.5 1.5 and grasp the handle of the srylet with palm of the
Lactate 1.2 1.2 1.2 hand, and carheter u'ith thumb and forefinger. Hold the
Urate 0.3 0 2 catheter vertically and introduce through the incision by
Sulphate 0.5 0 3
continuous nvisting morion and pressure. Now the stylet
Non-electrolytes (mg%) pierces first the linea alba, then peritoneal membrane.
As soon as the catherer enters into the abdominal caviry
Clucose 100 I 15 or more 100
an immediate sense of pressure release will be felt and a
Urea 26 0 200
gush of peritoneai {luiJ comes our as soon as rhe sryler
Creatinine l 0 6 is withdrawn. Push the carherer to the iliac fossa, then

a. Advantages of warming of bag: it decreases heat loss from body, increases clearance of urea
b. Heparin should be used for initial few cycles, or until the peritoneal e{lluent becomes clear.
c. Use of prophylactic antibiotic in the bag (gentamicin or ampicillin) is not advocated now-a-days.
 RENAL DISEASES

srylet should be withdrawn gradually. Connect the cath-
eter with the short arm of the Y-connector and thus the
circuit becomes complete.
5. Open the flow control of the administration set while
keeping drainage set closed, and allow the dialysis fluid
to flow into the peritoneal cavity. The flow should be in
a continuous stream. If continuous stream is not there,
wirhdraw a little of the catheter.
During each exchange of the cycle, 25-30 ml/kg of
PD fluid is taken into the abdominal cavity, then stop
the control of giving set and open the control of the
drainage set, and thus the cycles are continued. This
acute peritoneal dialysis is usually continued for 1B-20
hour; longer duration is likely to have higher risk of
infection.
In CAPD, required volume of fluid (25-30 mllkgl
cycle) entered into the peritoneal cavity is kept there for
2 hours, and then withdrawn. The procedure is continued
for 4-5 days; may be repeated, if needed.
6. Finally clean the wound and dress the site around the
catheter with gauze and leukoplast. Catheter should be
made anchored.
o Difficulry in drainage: This may occur due to kinking of
the catheter, catheter blockage; occurs by omental plugging
or by fibrin clots.
r Loss ofultrafiltration due to repeated episodes ofperitonitis
and is associated with gradual ineffective dialysis, hypergly-
cemia, and hyperlipidemia.
r Protein loss (0.5-1 g/L).
INDICATIONS OF PERITONEAL DIATYSIS IN ARF
a In uremic encephalopathy.
a Blood urea >25 mmollL or BUN >100 mg/dl.
a Creatinine >500 mmol/L
o Uncontrollable hyperkalemia>7 mmollL (or ECG changes)
a Intractable severe metabolic acidosis (<13 mmol/L)
o If anuria is for more than 3 days.
a Fluid overload with circulatory congestion, pulmonary
edema, or heart failure.

PD is usually stopped when spontaneous improvement of Behrman RE, Kliegman RM, Jenson HB. Nelson Tbxtbook of
blood chemistry has started. In fluid overloading situation, if Pedia*ics 18'h ed. \MB Saunders Co, 2009.
Parthasarathy A (ed). IAP Textbooh of Pediatrics 4'h ed. New Delhi:
body fluid removal is needed, a hypertonic dialysate solution
(3.860/o) can be used. Jaypee Brothers, 2009.
3. Dworkin PH. NMS: Pediatrics 4"' ed. Philadelphia: Lippincott
rX/illiams & \filkins, 2000.
4. Hoque M. Peritoneal dialysis in children. Bangladesh Paediatrics
COMPLICATIONS 1984;8(112):15-7 .

Clayden G. Hawkins R(ed). Paediatrics: Ti'eatment and Prognosis.

Infection: Peritonitis is common, organisms responsible are 1" ed. New Delhi: Jaypee Brothers, 1989.
Staphylococcus epidermidis from skin (50o/o), E. coli, Staph. 6. Bagga A. Consensus statement on management of urinary tract
aureus, Klebsiella, Proteus, Pseudomonas, fungi. infections. Indian J Pediatr 200 1 ;38: 1 1 06-1 5.
a Intraperitoneal hemorrhage, especially in HUS. 7. Bagga A. Revised guidelines for management of steroid-sensitive
a Perforation of bowel or bladder. nephrotic syndrome. Indian J Nephrol 2008;78:3I-9.

J
 -
-

CHAPTER 11
Fluid Electrolytes and Acid-Base Disturbances

Chopter Contents
Hypokalemia...,...... ...............,...........215 Respiratory a1ka10sis..,........................................................217
Hyperkalemia ......... .......,...............,...215 Mixed acid-base disturbances..,....,.... .......................,.....21i
Disturbances in acid-base staius.......................................2i6 Electrolyte disturbance in 1 H PS.,...,............... ......,,..........211

l\4etabolic acidosis .........,.......,............................................216 Anion gap............... ...............,...............219

Metabolic a1ka10sis.............,..... .................,................,........216
Respiratory acid0sis.................... ........................................2i1

o Angiotensin II has got two principal effects that can elevate

arterial pressure:
Total body water (TB\X) as a percentage of body weight changes Causes vasoconstriction of arterioles, increases peripheral
with in early life. At birth, TBW is
age, decreasing rapidly resistance and thereby raise the arterial pressure. Mild
78o/o of total body weight. In first few months of life, TBV venoconstriction also occurs, causing increased venous
drops dramatically to approximate the adult level of 600/o of return ofblood to heart.
body weight at I year of age. Acts directly on the kidneys ro decrease the excretion of
both salt and water and eventually causes salt and water
TB\7 (L) = 0.61 x \Weight in kg + 0.251
retention. It also causes secretion of aldosterone, which
TBW break-ups: increases salt and water reabsorption (i.e., absorption of
sodium and simultaneous excretion of potassium) by the
o Intracellular (ICF):
40-70o/o of TB\7
kidney tubules (distal tubule and collecting ducts).
o Extracellular (ECF): 20-25o/o of TBV
Net effect is increased arterial pressure.
o Inrravascular = Plasma (5%)
o Extravascular = Interstitial (ISF) 1570; transcellular Atrial natriuretic factor (ANF): This hormone is secreted
(TCF) 2o/o. from atria. Net effect of it is as follows:
Net effect : J blood volume, J BB natriuresis and diuresis.
REGUTATION OF ECF r Loss: Insensible loss from lungs and skin + urinary and
fecal loss.
Tonicity
Tonicity (effective osmolaliry) is maintained by: Electrolyles
o ADH: Synthesized at hypothalamus Can conduct electric current in solution. Ions are charged
r Thirst: stimulate hypothalamus at >290 mOsm/kg atoms, radicals, or molecules.
o Renin: Renin-angiotensin sysrem is stimulated by decreased + charged ions: Cation
circulatory volume in kidneys. - charged ions: Anions

Renin-An g iote nsin-Aldoslerone Syslem Dehydrotion

o Renin, an enzyme, is released from JG cells of afferent arre- Loss of fluid and electroll'tes: J pCp volume leads to J tissue :
perfusion, compensarory tachycardia and lactic acidosis occur.
rioles of kidneys, when arterial pressure falls. Renin acrs on
a globulin called angiotensinogen ro release angiotensin I. Sodium homeostasis: Maintained by two mechanisms:
o Angiotensin I is converted into angiotensin II by a convert- 1. Glomerular filtration: 80% of sodium is reabsorbed by 1
ing enzyme while blood flows through the small vessels of PCT. So GFR is important. If GFR is decreased, Na* 1
the lungs. excretion is decreased. So Na* concentrarion is retained. 't
1
1
at
 I

FLUID ELECTROLYTES AND ACID-BASE DISTURBANCES

Table 11.11 Major Osmolar Substances in ECF, lSF, and ICF Normol Acid-Bose Regulolion
The number of potential hydrogen in the body is huge. Most
are buffered and therefore are not in free form. At the usual pH
of 7 .4, the concentration of free hydrogen ions in the blood is
Na* 142 139 14
only 3.8 x 10 8 Eq/L (often expressed as 40 nEq/L):
K- 4.2 4.O 140
Cat* 1.3 11 0 lSaltl
Mgll 0.8 Q.7 20
rnH:PK*lop.-o lAcid]
cl- 108 108 4 pH :- log (H.) -- log (3.8 x 10{)
HC03- 24 28.3 10 : -(0.60-8.0) : z.q
'1
HPOI-' H?PO, 2 2
Acid-base balance is maintained by the interaction of the
1

5On-- 0.5 0.5 1

lungs, kidneys, and systemic buffering system (i.e., carbonic
Protein 1-2 0.2 4 acid and bicarbonate system >50o/o, Hb 30o/o, remainder by
Clucose 5.6 5.6 phosphates, ammonium, and protein).
Total m0sm/L 301 .B 300.8 3A1.2
The carbonic acid-bicarbonate system is as follows:
CO, +HrO <+ H2CO. <+ H* + HCOr-

2. Reabsorption of Na*: Occurs by DCT and renin-angio-

Regulotion of Body pH
tensin-aldosterone mechanism. o Buffers represent the first line of defense against changes
, in pH, and hydrogen ion concentrations are maintained
See Table 11.1 for major osmolar substances in ECF, ISE in relatively narrow ranges. Chemical buffer system is in
t
t'.
and ICF. the form of bicarbonate-carbonic acid system (ECF com-
F partment), protein, organic phosphate, hemoglobin (ECF
r compartment), and phosphate in monohydrogen and dihy-
I REGUTATION OF ACID-BASE BATANCE
drogen forms (urine).
An acid is a proton (i.e., hydrogen ion) donor. Hydrochloric, o Pulmonary mechanism that lends support to the bicarbon-
sulfuric, phosphoric, and carbonic acids are conventional acids, ate-carbonic acid buffer system by eliminating excess CO,
each dissociating to liberate protons. through rapid breathing.
A stong acid is one that is highly dissociated and therefore o Renal mechanism by excreting hydrogen ions as phosphate
produces a high concentration ofhydrogen ions; a weak acid buffer salts and ammonia ions and by reabsorption of bicar-
L
is one that is poorly dissociated. bonates in the proximal tubules.
A base is a hydrogen ion acceptor. Bases bind free hydro-
gen ions, reducing their concentration, e.g., hydroxyl ions,
ammonia, and the anions of weak acids.
A buffer is defined as a substance that reduces the change HYPONATREMIA
in free hydrogen ion concentration ofa solution on the addi-
tion of an acid or base. The presence of a buffer in a solution Serum sodium concentration <135 mmol/L is called hypona-
increases the amount of acid or alkali that must be added to tremia.
cause a change in pH.
Aprotes are cations such as sodium, potassium, calcium, and Eliology
magnesium that carry one or more positive charges, depending -Vater
o Hypovolemic hyponatremia: and sodium levels are
on valency, or anions such as chloride and sulfate that carry both low. This may occur, for exampie, when exercising
negative charges. Because aprotes can neither donate nor accept in the heat without replenishing fluid electrolytes, or with
protons, these are not acids, bases, or buffers. marked blood loss.
The term pH is employed to denote acidiry, alkalinity'
r Diarrhea
or neutrality. A neutral solution has a pH of 7; blood pH is
c Vomiting
7.4 x 0.05 means that it is slightly alkaline. Blood pH beyond
o Use of loop diuretics
7 and 7.7 is not compatible with life. Hydrogen ion concen- o Sweating
tration is dependent on the ratio of PCO, and bicarbonate.
o Burn
pH so is given by this ratio rather than the individual values
of the components. If PCO, rises by 1 mmHg, pH is lowered r Euvolemic hyponatremia: Normal water levels are combined
bv 0.01. if HCO3 falls by I mEq/L, pH is lowered by 0'02. with low sodium levels. This condition is commonly
ESSENCE OF PEDIATRICS
due to chronic health conditions, cancer or certain
medications.
o SIADH
o Hypothyroidism
o Psychogenicpolydipsia
r Glucocorticoid deficiency
Hypervolemic hypona*emia: Excess water dilutes the
sodium concenrrarion, causing low sodium levels. Hyper-
volemic hyponatremia is commonly the result of kidney
failure, heart failure, or liver failure.
O Congestive heart failure
() Cirrhosis
o Nephrotic syndrome
t) Renal failure
t) Protein-losing enteropathy
Clinicol Feotures
It depends upon rapidity ofsodium depletion, degree ofsodium
depletion (symptomatic in <120 mEq/L serum Na*), and sex
of the patient (females are affected more).
In acute loss, symptoms may be hyperexcitabiliry agitation,
muscle twitching, decreased reflexes irritability, convulsions,
and apnea.
In chronicloss, symptoms maybe lethargy, headache, anorexia,
nausea, emesis, malaise, confusion, stupor, and coma.
Treolmenl
1. teatment of asymptomatic hyponatremia depends
on its cause. \X/ith fluid overload, fluid restriction is the
appropriate measure: the serum sodium level may return
rapidly to normal if there is good renal function, but this
may take several days or weeks for patients with SIADH.
Adding extra salt to the diet or increasing the sodium
concentration of IV administered fluid often correcrs a
sodium deficit.
Urinary sodium concentration is often >20 mmol/L
in renal salt losing conditions and <10 mmol/L in other
situations. Correction requires administration of isotonic
saline-
\Mhen hyponarremia is caused by an excess
of total
body water (e.g., SIADH, hypothyroidism, erc.), urinary
sodium usually exceeds 20 mmol/L and therapy consists
of water restriction.
V/hen hlponatremia is caused by excess sodium and water
(e.g., nephrotic syndrome, cirrhosis of liver, etc.), urinary
sodium level is usually <10 mmol/L and therapy consisrs
of water and salt restriction. However, in acute on chronic
renal failure, urinary sodium may be >20 mmol/L.
2. Treatment of symptomatic hyponatremia consists of
administering a hypertonic saline solurion, calculated
according to the following formula:
(Cd-Ca) x K x wt in kg = mEq required
Here, Cd: serum sodium concenrrarion desired; Ca =
serum sodium concentration actually presenu K : 0.7-0.8
for newborn or premarure infant and 0.6-0.7 for child
and adolescent.
A dose of 12 mllkg of body weight of 3o/o NaCl solution
(6
,mEq Na./kg) usually raises rhe serum sodium level by
10'mEq/L. Rapid correction of hyponatremia may be associated
with myelinolysis of CNS, which present with disturbed spastic
quadriparesis and pseudobulbar palsy. Initial rapid therapeutic
increase in serum Na* concenrration should be only up to
125 mEqlL, no more than 10 mEq/L should be raised in any
24hour period. Subsequent elevation ofserum sodium concen-
tration should be done in small incremenrs over several hours.
HYPERNATREMIA
Serum sodium concenrrarion >145 mBqlL is called hyper-
natremia.
Etiology
e Primary sodium excess: Improperly mixed formula or
rehydration solurion, accidental substitution of NaCl for
glucose in infant formulas, excessive administration of
sodium bicarbonate during resuscitation, hypertonic saline
intravenous administration, intentional salt poisoning, inges-
tion of sea water, hyperaldosteronism.
o Primary water deficit: Diabetes insipidus, diabetes mel-
litus, diarrhea (i.e., warer loss greater than solute loss),
animal milk feeding, erraric ORS or formula preparation,
intentional withholding of water intake, increased insensible
water loss (e.g., premature infant, sweating, fever, radiant
warmer, phototherapy).
r 'W'ater
and sodium deficit: Gastrointestinal losses (diarrhea,
emesis), curaneous losses (burns, excessive sweating), renal
losses (diabetes mellitus, osmoric diuretics).
Clinicol Feolures
Irritabiliry
resdess, weakness, lethargy, high-pirched cry, hyper-
apnea, fever, confusion, convulsion, coma, brain damage may
occur due to sudden transfer ofwater from brain cells, causing
distension ofcerebral vessels leading to subdural, subarachnoid
and intracerebral hemorrhage. Skin and subcutaneous rissue is
firm and doughy; metabolic acidosis may occur.
Treolmenl
Hypernatremia is associated with a high mortality rate if serum
Na* concenrrarion is >158 mEq/L.
o Intravenous fluids should consisr of glucose in water, potas-
sium acerare and calcium as needed.
o Hypertoniciry should be correcred slowly, 10-15 mEq/L/d,
using 5% dextrose in 0.2o/o saline to replace the calculated ''.l
fluid deficit over 48 hours. If corrected too rapidly, there 1
may be cerebral edema, seizure, and CNS injury. I
'l
I
1
 FLUID ELECTROLYTES AND ACID-BASE DISTURBANCES

In patients with salt poisoning, peritoneal dialysis with r The following formula is also used in some cenrers for
glucose solution 30-45 mllkg containing 4.25o/o glucose correction of hypokalemia:
can be injected intraperitoneally and withdrawn t hour (Cd-Ca) x K x \7t in kg: mEq/L (required).
later. As serum Na* concentration falls, subsequent dialysis
Here, Cd: serum concentration of K* desired; Ca = Serum
can be carried out with I.25o/o glucose solution.
concentration of K*, which is actually present, K : 0.3.
To prevent or control seizures, phenobarbital should be
r Tieatment of cause.
administered. ,
o To treat heart failure, inoropic support may be necessary.
o Metabolic acidosis, if present, be corrected by NaHCO. HYPERKATEMIA
or THAM. Serum potassium concentration 5.5 mEqlL or more is called
o Tieatmenr of underlying cause. hyperkalemia.

HYPOKATEMIA Etiology
Serum potassium concentration <3.5 mEqlL is called hypo- a AGN, acute or chronic renal failure.
kalemia. O Adrenal insuffi ciency, hypoaldosteronism, insulin defi ciency
a Release of K* from tissues into ECF: Metabolic acidosis,
Etiology hemorrhage, hemolysis, burn.
r a Drugs: Potassium-sparing diuretics, digitalis overdose.
tanscellular shift:
o Excessive intake: Excessive parenteral administration of
o Alkalosis potassium.
,r Insulin
, B-adrenergic agonist Clinicql Feqlures
o Drugs (theophylline)
c Hypokalemic periodic paralysis o Cardiac: Arrhythmias, e.g., AV block, ventricular tachy-
cardia, fibrillation. In ECG, widening of QRS complex,
o Renal loss: tented peak T wave.
r Diarrhea, vomiting, sweating Skeletal muscles: \Teakness, ascending paralysis, flaccid
o DKA, PEM quadriplegia; respiratory paralysis may occur.
r c Renal tubular acidosis Other: Tetany.
t o Drugs: Amphotericin B, cisplatin, aminoglycosides
o Bartter and Gitelman syndrome Treolmenl
t
o Cushing syndrome Tireatment goals:
t o Primary aldosteronism. e To stabilize the heart to prevent life-threatening arrhlthmia.
l-
o Extrarenal loss: diarrhea, sweating o To remove potassium from the body.
tI Clinicql Feolures Treatment:

t o Heart: furhy'thmias, e.g., ectopic o Rapid IV administration of NaHCO, (1-3 mEq/kg) or

beats, atrial and ventricular
tachycardia, fibrillation. In ECG, ST depression, flattened T
waves, prolonged Q-T interval, and abnormal U wave.
o Skeletal muscles: \Teakness, hypotonia, areflexia, respiratory
muscle paralysis may occur.
r Smooth muscles: Paralytic ileus and gastric dilatation.
o Kidney: Inability to concentrate urine, pol1'uria, polydipsia.
o Prolonged hypokalemia results in pathologic changes in
kidney (nephrosclerosis and interstitial fibrosis).
Treotmenl
o Administration of adequate amounts of potassium (up to
3 mEqlkgl24 hr).
r Corrected gradually over 24 hr period by IV infusion,
infusate should contain K' 20-40 mEq/L.
o Oral K* supplements for weeks to replenish depleted body
stores.
glucose and insulin (0.5-1 g of glucose/kg with I U crys-
talline insulinl3 g of glucose) result in the movement of
potassium into cells and lower serum potassium level.
o Salbutamol, given IV at 5 pg/kg over 15 minutes or by
nebulizer at 2.5*5.0 pg is very eflective in lowering potas-
sium level for 24 hour; nebulized Albuterol is best.
o Intravenous calcium gluconate (up to 0.5 ml of a 10%
solution/kg given slowly over several minutes) counters rhe
cardiotoxicity of potassium, but ECG monitoring is required
during irs adminisrrarion.
All
measures described above are temporary measures, but
negative potassium balance can be established by using
ion exchange resins, e.g., kayexalate (1 g/kg/dose given by
oral or rectal routes every 6-12 hr) or by hemodialysis or
peritoneal dialysis.
o If patient i5 n61 2nu1i6-loop diuretics.
a Tieatment of cause: If Addisons disease-fludrocortisone.
ESSENCE OF PEDIATRICS

2. fleatment of underlying cause, e.g., using glucose and

METABOTIC ACIDOSIS
Metabolic acidosis occurs either due to decreased extracellular
HCO3 or addition of strong acids.
Etiology
o Hyperchloremic metabolic acidosis (normal anion gap,
8-16 mEq/L): Diarrhea (loss of bicarbonate occurs), renal
tubular acidosis, mineralocorticoid deficiency, urinary
tract diversions, posthypocapnia, ammonium chloride
intake.
o Normochloremic metabolic acidosis (increased anion
gap >16 mEq/L):
o Ketoacidosis: Diabetes mellitus, starvation
o Lactic acidosis: Tissue hypoxia (shock, hypoxemia, severe
anemia), liver failure, malignancy, sepsis, inborn errors
of metabolism
c Exogenous toxins: Salicylates (acids), paraldehyde, ethyl-
ene glycol, methanol, toluene
o Renal failure
Clinicol Feolures
insulin in diabetic ketoacidosis; improving circulation in
shock, eliminating saiicylate, methanol and other toxins
and trearing underlying sepsis.
). Calcium gluconate is indicated if patient develops hlpocal-
cemic tetany following correction of metabolic acidosis.
4. In case of severe acidosis with hypernatremia, it is advisable
to substitute sodium bicarbonate by tris-hydroxymethyl-
aminomethane (THAM).
5. Dialysis, especially in lactic acidosis.
METABOTIC AtKAtOSIS
Occurs either due to decreased concentration of extracellular
H* or due to increased concentration of HCO. .
Eliology
o Chloride-responsive (urinary chloride <15 mEq/L)
o Gastric losses (emesis or nasogastric suction)
o Diuretics (loop or thiazide)
o Chloride-losingdiarrhea
o Chloride-deficient formula
o Cystic fibrosis
o Post-hypercapnia

The normal respiratory response to metabolic acidosis-com- o Chloride-resistant (urinary chloride >20 mEq/L)
pensatory hyperventilation-may be subtle with mild metabolic o High blood pressure
acidosis, but it causes discernible increased respiratory eflort Adrenal adenoma or hyperplasia
with worsening acidemia. - Glucocorticoid-remedial aldosteronism
In mild metabolic acidosis, there is nausea, vomiting, - f{sn6yx5sular disease
headache, and abdominal pain. In chronic acidosis, fatigue, - Renin-secreting tumor
anorexia, and failure to thrive occur. - 17 u-hydroxylase deficiency
ln significant acidosis (pH . 7.2), respirations become - 11B-hydroxylase deficiency
deep and rapid (Kussmaul breathing), and the child becomes - Cushing syndrome
drowsy, confused, and stuporous. - I 1B-hydroxysteroid dehydrogenase deficiency
In severe acidosis, peripheral vasodilatation, vascular -
o Normal blood pressure
collapse, and shock may follow. Hyperkalemia may accompany.
Gitelman syndrome
- Sx111s1 syndrome
Treolment - {s1656rn21 dominant hypoparathyroidism
1. Severe metabolic acidosis (pH <7.2) is corrected by - Base administration
administration of NaHCO. 1-2 mEq/kg, or preferably
-
Three basic mechanisms may produce metabolic alkalosis:
calculated from the following formula:
Excessiae loss of lrydrogen ion:Persistent vomiting associated
(Cd-Ca) x K x \(/t in kg = mEq required
with pyloric stenosis, prolonged gastric aspiration.
Here, Cd = serum HCO3 concentration desired; Ca = Increased addition of bicarbonate to the ECF:
serum HCO, concentration actually presenu K = 0.5-0.6,
means 0.5 ml/kg of a molar solution of NaHCO, would
,r Excessive parenteral administration

raise the serum HCO, level about 1 mEq/L.

c Excessive oral administration, e.g., milk-alkali syndrome

Half of the calculated sodium bicarbonate should be

given immediately and the remaining half as an infusion
in 12-24 hour period. The usually available 7.5o/o solu'
tion providing 0.9 mEq/ml of bicarbonate needs to be
diluted with equal volume of distilled water or double
volume of 5%o dextrose.
o Increased renal reabsorption of bicarbonate: Primary
hyperaldosteronism, Cushing syndrome (by shifting H*
into the cells)
o ECF aolume contraction, which increases bicarbonate con-
centration in ECF and increases bicarbonate reabsorption
in proximal tubule.

\
:
I
1.

!
I FLUID ELECTROLYTES AND ACID-BASE DISTURBANCES
I
I
i Clinicol Feolures Treqlmenl
r Hypoventilation, apathy, confusion, drowsiness, stupor, and o Improvement of alveolar gas exchange by ventilation or
I coma. Alkalotic tetany due to low ionic calcium and cardiac assisted ventilation (PCO? >75-usually require mechani-
arrhl'thmia due to hypokalemia may occur. cal ventilation).
F
P
o Avoid inrravenous sodium bicarbonate, which may cause
, Treolmenl hyperosmolality and cardiac failure.
1
1. In mild to moderate alkalosis, no treatment is requirtd.
o Tieatmenr of underlying cause.

Treatment needed, if pH is >7.5.

I 2. If alkalosis is due to persistent vomiting or conrinuous
l
t stomach aspiration, replacement should be done by iso-
tonic or half-isotonic normal saline infusion with added
, ventilation) leading to blood pH greater than7.5, respiratory
potassium.
l Ammonium chloride infusion is indicated in
t case of
] severe alkalosis resistant
to saline. Dose may be calculated
I by the following formula:
r
I (Cd-Ca) x K x \Wt in kg = mEq required
; Here, K = 0.2-0.3.
.'). If alkalosis is due to volume conrraction, rrearment is
I
l directed at volume expansion and replacement of chloride
r and potassium. Inj. KCI (1 rrrl:2 mEq) is added to infusate
II to increase concentration of K* up to 2040 mEq/L.
4. Treatment of cause.
I
t of the extremities. Less common manifestations include tetany,
t RESPIRATORY ACIDOSIS
It means marked reduction in blood pH (pH <7.30), pri-
marily as a result of retention of carbon dioxide from poor
ventilation.
Patients usually have tachypnea, cyanosis, and features of
underlying disease.
Clinicql Feolures
Patients with a respiratory acidosis are often tachypneic in an
effort to correct the inadequate venrilation. The potential central
nervous system manifestations of respiratory acidosis include
anxiery dizziness, headache, confusion, asterixis, myoclonic
jerks, hallucinations, psychosis, coma, and seizures. An arte-
rial pH <7.20 impairs cardiac contractiliry, increases the risk
of cardiac arrhlthmias, especially in a child with underlying
cardiac disease.
Etiology
o Acute and chronic lung fiseases: Acute airway obsrruction,
croup, RDS, bronchospasm, asrhma, pneumonia, pulmonary
edema, bronchiolitis.
o Impaired lung motion: Pleural efirsion, pneumothorax,
thoracic cage abnormalities (kyphoscoliosis).
o Neuromuscular disorders: Encephalitis, stroke, poiiomy-
elitis, Guillain-Barre syndrome, brainstem or spinal cord
injury or tumor, myasthenia gravis, myopath,v, muscular
dvstrophy, hypothyroidism, malnurrition, hypokalemia,
CNS depressants.
RESPIRATORY ALKAIOSIS
\X{henever there is a primary decrease in PCO, (e.g., in hyper-
alkalosis occurs. Compensation is provided by kidneys by
decreasing blood HCO, level.
Etiology
Salicylate intoxication, hysterical hyperventilation, mechanical
ventilation, CNS irritation from meningitis or encephalitis,
liver failure.
Clinicol Feolures
Acute respiratory alkalosis may cause chest tightness, palpita-
tions, light headedness, circumoral numbness, and paresthesias
seizures, muscle cramps, and syncope. lJnconsciousness may
result from vasospasm of cerebral vessels due to hypercapnia.
Seizures may be due to decreased ionized Ca**.
Treotment
o fleatment of underlying cause.
o Re-breathing in a paper bag.
o Injection calcium gluconate IV in tetany, seizure.
r Acidifying agent, such as ammonium chloride is not indicated.
MIXED ACID-BASE DISTU RBANCES
The following three types of mixed acid-base disturbances
may be encountered:
1. Respiratoryacidosis-metabolic acidosis, e.g., respiratory
distress syndrome.
2. Respiratory acidosis-metabolic alkalosis, e.g., following
use of excessive diuretic therapy in chronic respiratory
acidosis in patient with CCF.
3. Metabolic acidosis-respiratory acidosis, e.g., hepatic
failure.
ETECTROTYTE DISTURBANCES IN INFANTITE
HYPERTROPHTC pyLORtC STENOSTS (tHpS)
There is no single parrern of electrol;,te disturbance in pyloric
stenosis. There may be oliguria or polyuria, and the urine may
be alkaline or acidic. A fall in plasma chloride level is inevitable,
and with it there is a rise in the level of plasma bicarbonate. The
ESSENCE OF PEDIATRICS

plasma K* Ievel may be deceptively high due to its mobilization Normal up to 16 mEq/L (average range of normal anion gap
from the cells prior to its loss in the vomitus and the urine. is 4-11).
Despite Na* depletion, plasma Na* level is sometimes raised
Increased anion gap occurs in:
in the early stages, possibly due to continuous loss of water by
insensible routes and through urine. NaHCO, administration 1. Increased unmeasured anions: Lactic acidosis, ketoacidosis,

aggravates the alkalosis and all the effects occurring from it.
The following sequence of events follows the persistent
vomiting of pyloric stenosis:
1. There is primary depletion of Na*, Cl-, and HrO, which
leads to dehydration and oliguria.
2. Soon the loss of Hn (because ofvomiting) leads to a metabolic
alkalosis. The kidney responds at first by secreting alkaline
urine rich in HCO.. The urinary excretion of K* is also
increased due to loss of Na* and water in the vomitus, by
decreasing the ECB powerfully stimulate the secretion of
aldosterone, which preserve Na* in exchange of K*.
3. There is a progressive depletion of K* as a result of its loss
both in vomitus and in urine. The urinary loss is due to
hyperaldosteronism. Cl- depletion is also due to vomiting.
Cl depletion leads to secretion of considerable amount
of H* and Kt in the urine (mechanism is not clear).
Ifthe K. depletion persists for a long time, it may
impair renal function by interfering with the normal con-
centration of urine. Under these circumstances, polyuria
may develop and increase the dehydration.
4. In due course, the Nat loss may become very severe as a
result ofvomiting. If both the Na* and K* depletion become
marked, the condition of 'paradoxical aciduria' may occur.
This remarkable state of afrairs is brought about by the almost
complete reabsorption of Na* in the distal tubule. As there
is virtuaily no K* to be excreted in exchange, the place of
this cation is taken by H*, which are then secreted into the
urine. The high concentration of H+ in the cells secondary
to K* depletion may assist this process, but Cl depledon is
the important factor in "Paradoxical aciduria'.
Treolmenl
Therapy differs little from that for other causes of dehydra-
tion, except that potassium replacement should begin early, as
soon as the child has urinated. These patients have a chloride-
responsive metabolic alkalosis, and only volume repletion, via
administration of sodium chloride and potassium chloride,
will allow correction of the metabolic alkalosis.
Administration of isotonic saline corrects dehydration,
restores the circulation to normal, and also raises the Ci-
concentration of plasma, and therefore corrects the effects of
Cl depletion on the kidne,v.
renal failure, drugs (salicylate, penicillin).
2. Decreased unmeasured cations: Hypocalcemia, hypomag-
nesemia.
Decreased anion gap occurs in: Hypoalbuminemia, hypercal-
cemia, hypermagnesemia.
FTUID RESUSCITATION
o D5 0.225o/o (baby saline) can be given up to I year of age.
o D5 0.45o/o saline can be given up to 10 years, and 5% DNS
for >10 year (Rule of thumb).
FIUID THERAPY IN BURN
Parkland formula:
o <10%o of BSA can be managed as outpatient basis.
o Burns >1570 requires resuscitation:
o Initial resuscitation: funger lactate/Hartman solution 4
mllkglo/o of burn in the 1" 24hours.
Half of total fluid in the first 8 hour and
- Remaining half in the next 16 hour.
-
o In addition, maintenance fuid must be given.
The same protocol can be adopted during management of
patient with Stevens Johnson syndrome.
Indications of packed cell transfusion:
o If hematocrlt <24o/c: (Hb 8 g/dl)
o If hemato crit <30o/o (Hb <10 g/dl) with systemic infection
and ongoing loss.
Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of
Pediatrics 18d' ed. 1ilrB Saunders Co, 2007.
2. Ghai OP (ed). Essential Pediatrics 7'h ed. New Delhi: CBS Publish-
ers, 2009.
3. Parthasarathy A (ed). IAP Textbook of Pediatrics 4'h ed. New Delhi:
Brothers, 2009.
Jaypee
4. Guyton AC, Hall lE. Ti*book of Medical Physiolog 10'h ed. Harcourt
Asia Pte Ltd. 2000.
5. Ganong WF. Reuiew of Medical Ph4siology 20'h ed. McGraw Hill
Co., 2001.
'Walter
JB, Thlbot IC. \Yaber & Israel General PathologltT6 ed. New
l
York: Churchill Livingstone, 1996.

Difference between sum-total of cations, sodium, and potassium

and sum-total of anions, chloride, and bicarbonate. I
I
Anion gap: (Na* + K.) - (Cl + HCO. )
a
i
)t
I
 CHAPTER
Endocrine and Metabolic Diseases
Chopter Conlents
HYPOTHATAMUS
At least five hypothalamic releasing factors for secretion of GH,
ACTH, TSH, FSH, and LH have been recognized. The factors
control secretion of hormones from anterior pituitary.
Arginine vasopressin (A\?) and oxytocin are secreted by
separate cells of the supraoptic and paraventricular nuclei of
hypothalamus. These hormones are transported to the nerve
terminals in the posterior pituitary, where these are secreted
in the free form.
PITUITARY GTAND
Two functional parts:
r Anterior pituitary gland (adenohypophysis)
o Posterior pituitary gland (neurohypophysis)
Anlerior Pituitory Glond
The hormones produced are:
. Somatotropin, growth hormone (GH): Its deficienry causes
pituitary dwarfism (short stature); excess causes gigantism
(before puberty) and acromegaly (after puberry).
o Marnmalotropin/lactotropin, prolactin (PRL): Concerned
with initiation and maintenance of lactation. Its secretion
is regulated by baby's suckling of the nipple.
o Thyrotropin, thyroid-stimulating hormone (TSH):
Deficiency of TSH causes pituitary hypothyroidism and
exc€ss causes hyperthyroidism.
L2
Female pseud0hermaphr0ditism...............,....1,............. 245
Disorders of puberty.,,...,...,.... ...........246
Delayed puberty ... ............,.............247
Precocious puberty..........................................................,.248
Parathyroid g1and.......................,........................................,,.249
Primary hyperparathyroidism ........,,..,. ..,....,...................749
Secondary hyperparathyr0idism......,...,........,,............... 249
Hypoparathyroidism .......................250
Syndrome of inappropriate secretion of ADH...............250
Corticotropin, adenocorticotroPic hormone (ACTH):
Its deficiency, if absolute, kills the patient in short time.
Relative deficiency causes Addison disease and excess leads
to Cushing syndrome.
Gonadotropins
o Follicle-stimulating hormone (FSH): FSH causes fol-
licular growth of the ovary in females, and in males, it
stimulates gametogenesis.
o Luteinizing hormone (LH): LH in females causes lu-
teinization, rupture of the follicle and transformation of
follicle into corpora luteum. LH stimulates secretion of
testosterone. Deficiency of LH causes sexual infantilism
and excess leads to precocious puberty'
Poslerior Pituitory Glqnd
The hormones produced are:
o Arginine vasopressin (AVP; antidiuretic hormone): The
stimuli for AVP (or ADH) release are increased plasma
osmolality (perceived by osmoreceptors in the hlpothalamus)
and decreased blood volume (perceived by baroreceptors in
the carotid sinus ofthe aortic arch).
It has blood pressor and antidiuretic activities; it can
also affect platelet aggregation, release of factor VIII and
hepatic glycogenolysis. A synthetic analogue, desmopres-
sin, has selectiviry for antidiuretic hormone receptors and
resistance to degradation by proteases.
o Oxytocin: Stimulates milk secretion and uterine
contfaction.
ESSENCE OF PEDIATRICS

DIFFERENTIAT DIAGNOSIS

a GH deficiency due to emotional deprivation

Hypopituitarism is a recognizable feature (either clinically or
a Hypothyroidism
by laboratory) of secretory functions of one or more hormones
a Nutritional dwarfing
of anterior pituitary (isolated/panhypopituitarism).

TREATMENT
ETIOTOGY

Idiopathic GH deficiency (most common): Defect in

o It is important ro observe child with growth records for
^ 6-12 months before doing expensive investigations. A child
hypothalamus, resulting in deficient GRF stimulation of
'who gains >4 cmlyr is ,rot lilely to have hlpopituitarism.
pituitary growth hormone.
Secondary GH deficiency:
o If GH level low: Recombinant GH 0.07-0.1 IU/kg/d or
every alternate day subcuraneously at night till adequate
o CNS tumors: Craniopharyngioma, glioma, chromophobe growth. Child gains 10-12 cm height in first year.
adenoma. o Supplementation of other pituitary hormones if needed.
O Surgery, injury: Hypothalamus, pituitary gland o Correction of intracranial lesion if any.
o Infiltration: Histiocytosis, congenital toxoplasmosis
o Irradiation
a) Tiauma: Battering, shaken baby syndrome, motor vehicle

crush
Short stature is said to be present when height is below 3'd
centile for age or more than 2 SD below the mean height for
CLINICAT FEATURES that age with height velocity <25'h centile for age.

o Normal weight and length at birth (GH not needed for

fetal growth). ctAsstFtcATtoN
Growth slows by 6-12 months, height <3'd centile by
2 year, in prepubertal growth velocity < 4 cmlyn 1. Normal variants
Male infants with microphallus (J intrauterine gonado- a. Familial generic shorr starure
tropins). b. Constitutional delay in growth and puberry
Newborn: Symptomatic hypoglycemia, rremor, convulsion, 2. Disproportionate short stature
sweating, apnea, cyanosis, prolonged neonatal jaundice.
a. Skeletai dysplasia, e.g., achondroplasia
Children:
b. Rickets
o Tiuncal obesity with small hands and feet c. Untreated congenital hypothyroidism
o Face-immature facial appearance, frontal bossing, flat
3. Proportionate short stature of prenatal onset (primor-
nasal bridge, prominent philtrum, single incisor teeth,
dial short stature)
delayed dentition, cleft palate
o Delayed sexual maturation, high-pitched voice a. Intrauterine growth retardation (F/H negative, H/O
o If CNS cause-bursting headache, visual IUGR, bone age normal, puberty normal)
field
defects, polyuria, polydipsia, abnormal neurological i) Intraurerine infections
signs ii) Placentalinsufficiency
iii) Teratogens-al'cohol, nicotine, drugs

INVESTIGATIONS
b. Chromosomal defects-Turner syndrome, Down syn-
drome
r X-ray Lt hand and wrist: bone age delayed c. Dysmorphic syndrome-Silver-Russell syndrome,
r X-ray skull, CT scan, MRI-if CNS lesion suspected Seckel syndrome (bird-headed dwarfism).
. GH level after provocative rest-by exercise, insulin- 4. Proportionate short stature ofpostnatal onset .
induced hypoglycemia, arginine infusion, L-dopa, glucagon, a. GiT: Chronic diarrhea, liver disease, celiac disease,
clonidine regional iieitis, glycogen srorage disease
o Level 7-10 nglml indicates partiai GH deficiency (must b. Malnutrition (hypocaloric)
be interprered in clinical context) c. Endocrine: Hypothyroidism, hypopituitarism, Cushing
o Level <7 nglml by rwo separare provocarive tests indicates syndrome, diabetes mellitus, diabetes insipidus, con-
GH deficiency genital growth hormone deficiency t
t
I
t
I
t
 !

ENDOCRINE AND METABOLIC DISEASES

d. Chronic anemia: Hemolytic anemia (Father's height + mother's height)

e. Chronic infection: Tuberculosis MPH (for girls) = - 6.5 cm

f. Cardiac: Congenital heart disease

\When child's height falls within target height, it
g. Respiratory: Chronic severe asthma, cystic fibrosis,
bronchiectasis indicates familial genetic short stature.
h. Renal: Chronic renal failure, tubular dysfunction e. General appearance and activity: Dysmorphic features
i. Psychosocial short stature (deprivation dwarfism) suggestive of specific syndrome, obesity (Fig. 12.1).

5. Miscellaneous
f. Skin, abnormal pigmentation or cyanosis. Coarse skin
or hair texture for clue to hypothyroidism.
Hurler, Hunter, progeria, Laurence-Moon-Biedl
syndrome, Noonan syndrome, mucopolysaccharidoses,
g. Head, ears, eyes. Midline defects (e.g., clefts), ocular
t or dental abnormalities. Visual field examination and
Prader-\Willi syndrome.
fundoscopy to look for underlying CNS disease or
GH deficiency.
APPROACH TO A CHILD WITH SHORT STATURE
h. Neck: Presence of goiter or nodules.
1. Growth chart: Accurate and serial measurements are i. Chest and heart: Cardiopulmonary disease or heart
plotted on growth chart. mufmur.
2. History: Information that may have effect on child's j. Abdomen: Tenderness or bloating indicating celiac
growth is gathered from talking with the child or his/ disease, intestinal TB, or inflammatory bowel
her caregivers, usually parents. disease.

a. Birth weight, length, gestational age, congenital anom- k. Genitalia: Undescended testes, hypospadias in boys
alies, duration of pregnancy. and clitoromegaly, labial fusion in girls. Stretched
b. Nutritional history penile length and testicular size noted. Pubertal staging
c. Social history: Home and social situation, stressors, done.
social habit such as tobacco use. l. Extremities: Abnormalities of digits, joints, body
d. Behavioral problems and school performance. proPortions.
e. Growth records kept by the parents, schools, doctors m. Neurological examination: To rule out CNS disease,
are helpful. especially any tumor that may cause GH deficiency.
Information about height of parents, siblings, and other n. Specific signs:
relatives. Family history of other medical problems. i) Turner syndrome: \Tebbed neck, shieldlike chest,
Review of systems: Sleep patterns, headache, visual widely spaced nipples, increased carrying angle'
changes, vomiting, constipation, abdominal pain, ii) Hypothyroidism: Coarse facies, mp<edema,
diarrhea, constipation, status and progress of sexual dry rough skin, macroglossia, developmental
maturation, polyurea, polydipsia, etc. retardation, infantile body proportion.

). Physical examination:
iii) Hypopituitarism: Cleft lip, cleft palate, single
incisor teeth, microPhallus.
a. Pallor, blood pressure, respiratory rate, cyanosis, club-
bing, frontal bossing.
iv) Bone age: By x-ray wrist and elbow. Bone age
is appropriate for chronological age in familial
b. Height: Supine length by infantometer from birth to
genetic short stature, IUGR, dysmorphic
24 months of age. Standing height by stadiometer for
syndromes. Bone age delayed but appropriate
children 2-I8 years.
for height age in constitutional growth delay.
c. Body proportions: Upper to lower segment ratio
Bone age retarded for both chronological age and
(US:LS ratio) and arm span to height ratio. US:LS
height age in endocrine disease.
ratio is approx 1.7 at birth, 7.3 at 3 years, 1.1 by 6
years, and 1 at 10 years. Arm span 2.5 cm less than Investigations: see Fig. I2.2 for investigations involved in
length at birth, equal at 1 1 years, and then greater establishing the cause and diagnosis of short stature.
than height. An abnormal US:LS ratio or arm span to
height ratio indicates disproportionate short stature.
Conslilulionql Delqy of Growlh ond
d. Mid parental height (MPH): Mid parental height is
a crude way to assess genetic component and gives
Developmenl
target height (MPH t 1SD, where 1SD is about 3 Growth Assessment
cm) for a child.
o These children grow at or below the 3'd percentile (NCHS
(Father's height + mother's height) standard) at normal growth velocities, which results in a
\{PH ifbr boys) = + 6.5 cm
curve that is parallel to the 3'd Percentile.
 't
ESSENCE OF PEDIATRICS

Child suspected of having short stalure

Looks normal $

Growih velocity
normal for age
and pubertal slage

Syndromes
Short trunk

Achondroplasia $ MPS
w"d
Familial
short rhin ffi

stature
IUGR &
v

Chronic systemic disease Hypopituitarism

Cong. heart disease Hypolhyroidism
Diabetes mellitus Hypoparathyroidism
Malnutrition Glycogen storage
Psychosocial disease
Deprivation
Anorexia nervosa

Fig. 12.1: Assessment of general appearance and activity in a suspected case of short stature

o Puberty is delayed and usually refects significantly delayed
skeletal maturation.
r Family members usually are of average height, but there
often is a family history of short stature in childhood and
delayed puberty in other family members.
lnvestigations
Minimal diagnostic tests are indicated, including thyroid
studies, complete blood count, erythrocyte sedimentation rate,
electrolytes, BUN, and bone age assessment. These children
have no findings srrggestive of other endocrine or chronic
systemic disease, and growth veiocity is normal.
Treatment
In many instances, reassurance that no significant endocrine
disease exists andthat normal growth and puberry with rea-
sonable adult stature are expected is all that is required. For
boys and girls with no signs of puberry by 14 years of age
and with a diagnosis of constitutional delay of growth and
development, a short course (4*6 months) of the appropriate
sex steroid may be helpful.
Counseling: These children and their families should be coun-
seled about this pattern of growth and development as a variant
of normal conditions and reassured about their potential for
normal height, usuaily in the range expected for their families.
Fomiliol (Genetic) Shorl Slolure
Growth Assessment
o These children establish growth curves at or below the 3'd
percentile by 2-3 years of age. They are otherwise com-
pletely healthy, with a normal physical examination. Bone
age in these children is normal. Therefore, puberty occurs
at the usual age, and thus limits potential for growth late
into adolescence.
r Short stature usually is found in at least one parent. However,
occasionally short stature may be present only in more
distant relatives.
Investigations
The same minimai diagnostic evaluation may be performed in
these children as for those with apparent constitutional delay of
growth to rule out subtle thyroid dysfunction or chronic disease.
 ENDOCRINE AND METABOLIC DISEASES

Short child

History + Examination
Mid parental height, anthropometry,
growth velocity, bone age

Clue from history examination

ffi gI

Specifie tests to diagnose Screening tests

Specifie treatment (lf delayed bone age or height < 3SD
Features suggestive of FF or decreased growth velocity)
(usually height <2SD)

Hb/CBC/ESR
Monitor growth velocity S. calcium/S. phosphorus, S. alk
Counsel parenls phosphatase
Blood gases (for acidosis), S. Na./K-lCL
S. creatine, SGOT, SGPT, Proteins
a Urine routine
a Stool routine
X-ray skull lateral

Abnormd Normal
ry*
$
ffi
I
I
f
Specific and tr
Karyotype in all females wi th significant
management ffi short stature
Consider tests Jor GHD & '
l

hypothyroidism (GH stimulation tests/T3,

T4, TSH)

Normal

*"ffi*ffij
Fig. 12.2: Algorithmic approach to the investigation of a child with suspected short stature.

Counseling Growth Assessment

Because puberty occurs at the expected time, these children Children with classic GH deficiency grow at subnormal growth
seem to be at less of a disadvantage socially and emotionally velocities (<5 cm/yr) and have significant retardation of skeletal
to those with constitutionai delay, despite the fact that their maturation. Therefore, evaluation with GH testing should be
potential for adult height in the normal range is less. reserved for children who satisfir those criteria.

e A history of birth asphyxia or neonatal hypoglycemia, or

GH Deficiency physical findings of microphallus or midline defects, is
F..,','er rhan 5olo of children have GH deficiency. strongly suggestive of idiopathic GH deficiency.

ESSENCE OF PEDIATRICS
. GH deficiency secondary to a hypothalamic or pituitary
tumor usually is associated with other neurologic or visual
complaints and findings. In an older child with more recenr
onset of subnormal growth, the index of suspicion for tumor
should be high.
lnvestigations
After establishing that currenr growth velocity is <5 cm/yr and
that thyroid function is normal and other systemic disease is
unlikely, GH testing should be carried out. Evaluarion for
other pituitary hormone deficiencies also should be performed.
Treatment
o For children deemed GH-deficient, recombinant human GH
(0.03-0.05 mg/kg) is given by subcutaneous injection every
day. Accelerated growth velocir;" on GH rreatment results
in some catch-up growth in mosr children.
o If puberry is .delayed beyond 14 years of age, the addition
of sex steroid may be considered, both to augm€nr rhe
growth response to GH and to stimulate secondary sexual
development. In children who have permanent gonadotropin
deficiency, sex steroids may need to be replaced indefinitely,
as physiologically as possible.
o fieatment with cortisol,thyroid hormone, and vasopres-
sin (ADH) may be needed, depending on the degree of
hypopiruirarism.
Primory Hypothyroidism
Itcauses marked growrh failure, with growth velociry <5 cmlyr,
and marked retardation of skeletal maturation. Because primary
hypothyroidism is easily treatable, almost all children with short
stature should have Tr, Tr, and TSH levels measured, even in
the absence of obvious symproms, to rule out any degree of
hypothyroidism. teatment is with sodium-L-thyroxine.
Cushing Diseqse
It is a very rare cause of short srarure in children. However,
hypercortisolism (from either exogenous treatment with phar,
macologic doses of steroids or endogenous over-secretion)
may have a profound growth-suppressing effect. Usually orher
features of Cushing syndrome are evident.
Primordiol Growth Foilure
Ithas been used to describe a large, diverse group of children
who have normal endocrine function but who have inherent
limitations on skeletal growth.
Etiology
The cause of short starure in these children usually is easily
identified on the basis of abnormal body proportions (skeletal
dysplasias), dysmorphic features (chromosome abnormalities),
3 I
t
and other characteristics of the history or physical examination
(e.g., Prader-Willi syndrome, Noonan syndrome, intraurerine
growth retardation).
lnvestigations
Special attention should be given to the evaluation of girls
with short stature. Although a short girl with all of the physi-
cal stigmata of Ti-rrner syndrome may be easily identified, the
features may be quite subtle in some girls. Therefore, girls, with
short stature and delayed puberty should have gonadotropins
and chromosomes measured. Elevated gonadotropins indicat-
ing primary ovarian failure and chromosome abnormalities are
diagnostic of Turner syndrome.
Treatment
Although children with primordial growth failure are not
thought to have classic GH deficiency, the response to GH
treatment in these children is being explored. In Turner syn-
drome, treatment with GH + low-dose estrogen + anabolic
steroid (oxandrolone) is helpful. Limb lengthening surgery
may be done for skeletal dysplasia.
Chronic Syslemic Diseqses
The impact of chronic systemic diseases on growth is well-
known. Types of diseases:
r Cyanotic congenital heart disease, poorly controlled diabetes
mellitus and tuberculosis, severe rheumatoid arthritis have
a deleterious effect on growth.
o Some chronic diseases may have minimal symptoms and
yet have a significant effect on growth. The two best known
categories of chronic disease that may presenr first as short
stature are gastrointestinal diseases, specifi cally inf ammatory
bowel disease (Crohn disease) and celiac disease, and renal
disease associated with renal tubular acidosis or uremia.
lnvestigations
Screening studies that may be helpful are complete blood count,
erythrocyte sedimentation rate, serum electrolytes, and BUN.
In some children where there is no explanation for growth
failure, additional diagnostic tests for gastrointestinal disease
are performed.
Psychosociol Deprivolion .
In some children, a hostile or neglectful environment appears
to result in functional GH deficiencv.
Clinical Features
Children with psychosocial deprivarion characteristically
show bizarre behavior, including hoarding food, gorging, !
drinking from puddles and toilet bowls, immature speech, 1
disturbed sleep-wake cycles, and diminished perception of 1
1
rt
 ENDOCRINE AND METABOLIC DISEASES

pain. Clinically, they resemble children with GH deficiency, Bechutitb-Wiedemann syndroze (B\7S) (Beckwith
with marked retardation of bone age and delayed puberty. syndrome): Large newborn with umbilical hernia
(omphalocele), large tongue (macroglossia), renal and
lnvestigations pancreatic hyperplasia, hypoglycemia due to hyperinsu-
linemia, prominent facial nevus flamus, increased growth
If GH testing is done while the children remain in the hostile
in childhood, increased proneness to \7ilm tumor.
environment, it usually shows a blunted response (switch ofl). Homocjtstinuria:
til{hen taken out of that environment, the children show catch-
ffu1e56rnal recessive disorder, tall stature since child-
up growth, and testing reverts to normal (switch on)' - hood, eunuchoid body proportion
Arachnodactyly, lens subluxation may lead to glau-
Mqlnulrilion - coma, myopia, and retinal detachment
See nutritional disorders. $svs16 osteoporosis begins in adolescence and kypho-
- sis. Mental retardation common. Marked tendency
of arterial thrombosis, resulting in MI leading to
premature morbidity and mortality of the patient'
Urine contains excess amount of homocysteine.
An individual is of tall stature when growing above the 97'h
percentile.
i-) Ex*a Y syndrome; Normal birth length, excess growth
rate, tall final height, no evidence of growth hormone
excess, karyorype 47 YW or 48 XXYY
ETIOIOGY.
Endocrine disorders with tall stature
Non-endocrine causes: Constitutional tall stature, familial
o Pituitary gigantism:
genetic tall stature, cerebral gigantism, Marfan syndrome,
Growth hormone secreting pituitary adenoma before
: Beckwith-\Tiedemann syndrome, homocystinuria, extra Y - epiphyseal fusion
syndrome, Klinefelter syndrome.
! Growth velocity very high
Endocrine disordets: Pituitary gigantism, thyrotoxicosis, sexual
- Pubertal maturation may be delayed
l -
precociry hypogonadism. Serum growth hormone and somatomedin levels are
- high
r Non-endocrine causes oftall stature
c Constitutional tall stature:
o Thyrotoxicosls.' Increased growth velociry, advance in
bone maturation, hyperthyroidism and over treatment
Taller than peers
- Growth velociry within normal range per bone age
with thyroid hormone in early life.
- o Sexual precociQt: Inappropriate exposure to gonadal
Bone age is usually more than the chronological
- age
hormone (estrogen/testosterone) brings abnormally rap-
of tall id growth velocity and advance bone age leading to tall
Absence of any signs relating to other causes
- stature
child. But because ofearly epiphyseal closure, their final
adult height is short.
There may be history of a close relative being taller
- than peers during childhood who eventually reaches
o Hypogonadism: There are varieties of hypogonadism
that lead to tall stature with eunuchoid body proportion.
a normal adult height
Example: Kallmann syndrome, Klinefelter syndrome,
Final heights is usually within normal adult range
- and castration before puberty.
Familial genetic tall stature: Genetic height potential is
are tall). Growth is at high normal rate.
high (i.e., parents
Bone age is close to chronological age, and final height TREATMENT
is tall.
Tieatment should be started according to cause. In Familial
a) Cerebralgigantism: Rapid growth in infancy, prominent
genetic short stature, final height can be reduced by promoting
forehead, high-arched palate, hypertelorism, growth rate
early epiphyseal closure with estrogen (for girls) and testoster-
decreases to normal in late childhood and no evidence of
one (for boys).
growth hormone excess.
Marfan syndrome:
Thll and thin build, eunuchoid body proportion,
- lower segment of the body is prominent
Long thin spiderJike fingers (arachnodacryly), pectus
- Thyroid function:
excavatum, joint laxiry high-arched palate, lens sub-
luxation, aneurysm of the ascending aorta, and pro- o Thyroid hormone is essential for normal maturation
lapse mitrai valve the CNS in children. Deficiency of thyroid hormone
ESSENCE OF PEDIATRICS

the first 2 years of life may result in severe psychomotor Table 12.2: lodine Deficiency Disorder (lDD) Prevalence
retardation. lndicator (WHO 1994)
r Thyroid hormone is also necessary for normal skeletal growrh
and maturation in growing children.

Thyroid metabolism: Prevalence of goiter <5 5 1 9.9 20-29.9 >10

in:chool-age children
o Synthesis: Iodide absorbed from the intestine is trapped in the (SAC) r'bt

thyroid gland and is organically bound to tyrosine residues of Frequency of thyroid <5 5-1 9.9 20-29.9 >30
thyroglobulin. Iodination of tyrosine forms monoiodoryrosine volume in SAC >q7l
centile by ultrasound r"/o'
(MIT) and diiodoryrosine (DIT), which condense to form
thyroxine (T,) and riiodothyronine (Tr). $edian urinary iodine in 100 200 50 99 20 49 <20
SAC and adult: rprg/Lr
r Feedback control: Thyroid hormone synthesis is controlled
q.9
Frequency of neonatal <3 3-1 20-19.9 >40
by a negative feedback loop invoiving the CNS at the level TSH -5 prUrml whole
of the hypothalamus, and pituitary gland. blood tozo)

c Low levels of circulating thyroid hormones stimulate

hypothalamic release of thyrotropin-releasing hormone
(TRH), which then stimulates production of thyroid-
stimulating hormone (TSH) in the pituitary gland. TSH
congenital hypothyroidism. Maternal hypothyroxinemia during
stimulates increased production of T, and T. by the thy-
early pregnancy is a key factor in the developmenr of the
roid gland.
neurological damage in the cretin. Iodine deficiency in early
r Circulation: T, and T. in
plasma bound to
circulate
life results in irreversible brain damage if not treated within
thyroid-binding proteins-thyroxine-binding globu-
14 days of birth. Iodine deficiency results in loss of 10-15
lin (TBG) and thyroxine-binding prealbumin. Protein
intellectual quotient (IQ) points at a population level, and
bound T, and T. account for over 99o/o of circuiating thy-
constitutes the world's greatest single cause of preventable brain
roid hormones. The free (i.e., not protein bound) Tn and
damage and mental retardation.
T. are the metabolically active forms of the hormone.
Multiple epidemiological criteria are used to monitor world-
wide iodine deficiency problem (Table 12.2).

INVESTIGATIONS
Iodine is an element required in rrace quanrity by the body, t. Thyroid function evaluation:
deficiency of which resuits in a specrrum of physical and
mental disorders that altogether are known as iodine deficiency
a. Thyroid uptake study of radioiodine (Ir31): Normal
thyroid uptake in %o is:
disorders (IDD) (Thble 12.1). Most familiar effect of IDD is
goiter and brain damage. 1) At2 hours after ingestion of radioiodine-3*I0%
Maternal iodine deficiency-induced hypothyroxinemia ii) 24 hours after ingestion of radioiodine-70-35o/o
is responsible for still birth, abortion, neonatal goiter, and Uptake increase by the thyroid gland is associated
with iodine deficiency or hyperthyroidism. The rwo
conditions may be separated by thyroid hormone
Table 12.1: lodine Deficiency Disorders (Hetzel, 983)
.1
assay.

Abortion
b. Radionuclide thyroid scan: Scan of thyroid gland
may be done by radioactive subsrance (radionuclide)
Still birrhs
99m-Technitium or I'3r. Scan assesses thyroid size,
Brain damage (cretinism)
nodularity, presence of cold nodule and also thy-
Neonatal goiler roiditis, which can be sequei of iodine-deficiency
Brain damage goiter. In IDD, gland shorvs diffuse and avid uptake
Coiter of radiotracer.
Thyroid deficiency (loss of energy) c. Thyroid hormone assay: In presence of hypothyroid-
Impaired school performance ism, both T- and T, are below the normal range
(<0.8 nmol/L and <52 nmol/L) and serum TSH is
Retarded physical development
above normal value (>5 mlU/L). Subclinical IDD
Coiter with its complications
shows elevated serum TSH, lower To, and somerimes
Thyroid deficiency (loss of energy)
elevated T, level in the serum.
lmpaired mental function
2. Urinary iodine excretion: Not routinely used.
 ENDOCRINE AND METABOLIC DISEASES

TREATMENT o Endemic goiter

o Congenital goiter
r Tieatment of individual disease: o Hashimoto thyroiditis goiter
o Congenital hypothyroidism: Life-long thyroxine Hlpothyroid goiter
10-15 pglkgl24 hr in neonate and 4 pglkgl24 hr in
older children. a Dyshormonogenesis
c Endcmic goiter of school-age children (SAC) can be o Drug-induced goiter, e.g., iodine, lithium, amiodarone
treated with iodine supplementation (Lugol iodine a Congenital goiter
2 drops nvice daily mixed with a glass of water or milk) a Hashimoto thyroiditis
and thyroxine (50-100 pgl24 hr) to give rest to the
Hyperthyroid goiter
hyperactive thyroid gland.
o Graves disease
o Surgical treatment is required when gland is large and
o Toxic nodule
nodular.
o Hashimoto thyroiditis
IDD in mother if detected during pregnancy can be corrected
with Lugol iodine (2 drops twice daily mixed with a glass of Goiter grading
water or milk). Maternal hlpothyroxinemia associated with
IDD should be corrected by thy'roxine supplementation. Gr- 0 : No palpable or visible goiter
Prevention: Iodine deficiency disorder can be completely Gr- I : Palpablegoirer
eradicated if,iodine supply is ensured. Fetal complications,
Io : Only palpable but not visible even when neck
is fully extended
still birth, and congenital hypothyroidism can be prevented
if mother is supplied iodine during pregnancy. Salt iodiza- IB : Both palpable and visible with neck extended
tion is the most popular and currently advocated method Gr - II : Goiter visible with neck in normal position
of iodi ne supplementation. Gr - III : goiter visible from a distance
Gr - IV : Very big goiter

CONGENITAT GOITER
Enlargement of thyroid gland is called goiter. Goiter may May be due to maternal ingestion of antithyroid drugs, or iodine,
result from: or other goitrogens during pregnanry or due to inborn biosynthesis
o Iodine deficiency defects. Congenital goiter may also be caused by neonatal Graves
r IncreaseTSH secretion in response to low circulating level disease. Infant is clinically euthyroid or may show evidence
oF thyroid hormones of hypothyroidism. Occasionally, congenital goiter may cause
o Neoplasm or inflammation difficulty during child birth or respiratory distress in neonates.
t-
o Presence of thyrotropin receptor stimulating antibodies
(TRSAb) Treolmenl
t
I r Thyroid hormone replacement to hasten disappearance of
I' ETIOTOGY goiter or to treat clinical hypothyroidism.
I o Partial thyroidectomy when respiratory obstruction is
t" a Physiological: Puberty goiter severe,
t o Autoimmune: Hashimoto thyroiditis, Graves disease
a Simple or colloid goiter
a Iodine deficiency: Endemic goiter srMPrE GOTTER (COttorD GOITER)
a Dyshormonogenesis
Also called diffuse nontoxic goiter, more common in girls with
a Goitrogens in diet
a peak incidence around puberty. Euthyroid goiter and often
a Infiltrative thyroid disease or neoplasia
no cause is evident goiter, may be small or large and firm in
consistency, easily confused with goiter of chronic lymphocytic
ctAsstFtcATtoN thyroiditis.

Congenital or acquired; endemic or sporadic; diffuse or nodular.

lnvestigolions
Functionally may be euthyroid, hypothyroid, or hyperthyroid.
r TSH level is normal or low with normal Tr,Tr.
Euthyroid goiter o Thyroid antibodies (antiperoxidase, antithyroglobulin)
r Puberw goiter absent.
r Simple or colloid goiter r Thyroid scintiscans are normal.
ESSENCE OF PEDIATRICS

o Radio-iodine uptake test (usually normal).

o Biopsy-normal or reveals visible follicular-size dense
and flattened epithelium.
Treolmenl
Best treated by thyroxine. fteatment usually is lifeJong and
mly help to avoid progression to a large multinodular goiter.
Sometimes, spontaneous regression may occur. Untreated
patient should be re-evaiuated periodically.
ENDEMIC GOITER
Caused by dietary iodine deficiency and is more prevalent
in areas where iodine deficiency is severe. Goiter occurs
when iodine intake or excretion in urine is <20 pg/d.
Endemic goiter is more common in girls than in boys and
almost clinically euthyroid. Maternal iodine deficiency
causes endemic cretinism in about 5_15% of neonates who
develop endemic goiter.
lnvesligolions
e Serum Tr, To, and TSH estimation (low T, and T.
increased TSH; T" may sometimes be elevated)
r Radio-iodine uptake test (increased uptake)
o Thyroid scan
Treolmenl
TSH suppression by thyroxine ro
regress goiter size, followed
by iodine supplementarion to prevenr development of endemic
goiter. Only iodine supplementarion without regression has
no vaiue, further, it may increase risk of autoimmune thyroid
dysfunction.
SPORADIC GOITER
colloid
May occur at birth (congenital hypothyroidism) or ar any time
during childhood or adolescence (juvenile hypothyroidism).
Because of the importance of thyroid hormone for normal
brain growth and development in the first 2 years of llfe, rhe
clinical considerations are different for infants than for older
children and adolescents.
CONGENITAT HYPOTHYROIDISM
Unlike acquired thyroid diseases, affects males and females
equally.
Eliology
o Developmental thyroid defect (thyroid agenesis or dysgen-
esis) is the primary cause of congenital hypothyroidism.
o Defective biosynthesis of thyroid hormone (frequently
resulting in goiter) also may cause this disorder.
o Transient congenital hypothyroidism may occur as a result of
transplacental passage of maternally ingested goitrogens
(e.g., iodine expecrorants, radioiodine, goitrogenic diet,
with
antithyroid drugs, or maternal antithyroid antibodies).
o Maternal iodine deficiency.
Clinicol Feotures
Because thyroid hormone does not appear to be necessary
for fetal growth, infants with congenital hypothyroidism are
normal in size.
r Symptoms: Although a newborn rarely may have some
physical features of hypothyroidism in the first week of
life, usually it is not apparenr. Often the first symprom is
prolonged neonaral jaundice (7Soto). Orher symptoms rhar
develop in the first 1-2 months of life are feeding problems
(40o/o), lethargy, and constipa tion (45o/o).

Patients are usually euthyroid, but may be hypothyroid. Most

o Signs: Signs are coarse facies with large open fontanelles
(35Vo),large protruding tongue (45o/o), hoarse cry (40o/o),
common cause of sporadic goiter is lymphocytic thyroiditis.
Dyshormonogenesis, ingestion of goitrogens or drugs (e.g., umbilical hernia (600lo), cool, dr;., mottled sltn (45o/o),
lithium) may also cause sporadic goiter. The occurrence of hypotonia, and delayed development.
this disorder in sibling, onset in early life, association with o Severe manifestations: Severe congenital hypothyroid-
hypothyroidism provide important clue for diagnosis of dys- ism is characterized by short limbs, epiphyseal dysgenesis,
hormonogenesis sporadic goiter. impaired physical growth and development, and menral
retardation.
Treolmenl
Differenliol Diognosis
Dyshormonogenesis goiter: Replacemenr therapy with thy-
roxine. Down syndrome, Hurler syndrome (see Table 12.3 for fea-
tures differentiating hypothyroidism from Down and Hurler
syndromes).
HASHIMOTO GOITER
1. Small goiter in euthyroid state: Regular follow-up. lnvesligolions a
!
2. Large goiterwith hypothyroifism: Replacement rherapy o The diagnosis of congenital hypothyroidism is made by 1
with thvroxine. documenting decreased serum concentrations of total Tr, 1
t
1
I
a
 ENDOCRINE AND METABOLIC DISEASES

Table 12.3: Differential Diagnosis of Hypothyroidism

1. Family history Consangu inity rarely present Consanguinity may be present High maternal age is usually present

2, Physical activity Letha rgi t Diminished Active, playful

3. Physical characteristics
i) Face Coarse (wrinkled forehead, low Crotesque Iike (large nose wrth FIat facies with small nose and depressed
hair line and large protruding depressed nasal bridge, heavy nasal bridge
tongue, depressed broad nose) supraorbital ridge and eye brows,
and thick lips)

ii) Eyes Swollen eye lids with narrow Hypertelorism with clouding of Upward slanting of eyes with epicanthic
palpebral fissures and eve\ dppear cornea folds. Brushfield spots are present
1ar apad.

iii) Head Normal head size with widely Large scaphocephalic head with Small head with flattening occiput
open dnterior and posterior frontal bossing (brachycepha ly)
fontanel les. Scanty hai rs

iv) Skin Dry coarse t old skin Dry coarse skin Nornral skin

v) Hancl Broad hands with short fingers Broad hands with characteristic Broad hands with clinodactyly little
limitation of extension of joints of fingers. Simian crease may be present.
fingers

vi) Hepatosplenomegaly Absent Present Absent

vii) Physical deformity Lumbar lordosis may be present Lumboclorsal kyphosis, genu Absent
valgum, coxa valga are common.

viii) Associated Absenr Absent ASD, VSD, TOF, duodenal atresia,

congenital anomalies imperforate anus or Hirschsprung disease
may present

4. lnvestigations
i) X-ray ot wrist Bone age markedly delayed Delayed bone age with Bone age delayed
characteristic taperi ng of proximal
ends and widening of distal ends
of metacarpal bones
ii) X-ray oi Anterior beaking of vertebral Ovoid shape vertebral bodies with Normal finding
thoracolumbar bodies may present beaking oi Iower Jnterior mdrgin
spr nes of vertebral bodies may present
iii) Confirmatory tests T,, Tr, and TSH assay (low T, and Detection of L-iduronidase enzyme in
To and high TSH is diagnostic) leukocytes or cultured fibroblast (absent)

5. I reatment lhyroxrne replacement Bone marrow transplantation No specific treatment

decreased T. resin uptake, and elevated serum concentra- Treqtment

tions of TSH:
Thyroid hormone replacement, using synthetic thyroxine as
.Lr ,trsFl = Primary hyporhyroidism a single daily oral dose, should be given in empty stomach,
'
, JT . J rsu = Secondary hypothyroidism at a convenient fixed time regularly. The dosage for infants is
approximately 1 .5-50 p,gl 24 hr), children
0- 1 5 pg/kg/d (37
Assessment of skeletal age: Knee radiography-absent lower 4 Vglkgld. As the child grows, the dosage is adjusted to
femoral and upper tibial epiphyses suggest intrauterine maintain the serum Tr, TSH in the normal range. Treat-
hypothyroidism. X-ray hip joint for femoral epiphyseal ment is usually life-long.
dvsgenesis. Follor.v-up: Grorvth and neurologic development and level
A ee"'Tc thyroid scan before initiating therapy mav be helpful of T, or free T. and TSH are evaluated at regular follow-up
in ascertaining the etiology of congenital hypothyroidism, visits every month ir.r the 6rst 6 months of life, then every
u'hich may have prognostic implications. 2 3 monthly in the first 2 years, with somewhat less frequent
; Absence of ee"Tc uptake indicates thyroid agenesis follow-up visits after 2 years of age.
' Increased eenTc uptake in a normally positioned
Prognosis
riand implies an enzymatic defect in thyroid hormone
r:oduction \fhen the diagnosis of congenital hypothyroidisrn is delayed
.-rr: rcropic gland is demonstrated by abnormal localiza- beyond 3 months of age, a high proportion of children suffer
:- -.: of """Tc uprake permanenI neurologic impairment.
ESSENCE OF PEDIATRICS
Screening
Screening is done between 5 and 74 days of life. May give
false-positive result due to physiological high surge of TSH
during birth if done before 5 days after birth.
Done by measuring TSH or T4. 1TSH o, .1,T,
"rorrr.
suspicion for congenital hypothyroidism. Screening results
dre not cenr percenr diagnostic.
Suspected case should be further evaluated by T, and TSH
measurement, radiography, and thyroid scanning.
JUVENTLE (ACQUtRED) HyPOTHyROtDtSM
\7hen symptoms appear after the first year of life, hypothy-
roidism is presumed to be acquired. Juvenile hypothyroidism
is more common in girls than in boys.
Etiology
The most common cause of juvenile hypothyroidism
autoimmune destruction of the thyroid secondary to chronic
lymphocytic thyroiditis (Hashimoto thyroiditis).
Other causes include ecropic gland, thyroid dysgenesis,
goitrogens (e.g., iodide cough syrup, antithyroid drugs).
e Iodine deficiency.
o Surgical or radioactive ablation for treatment of
hyperthyroidism.
Clinicol Feotures
Symptoms: Slow linear growth is the hallmark of hypothy-
roidism. Puberty usually is delayed although occasionally
may be paradoxically precocious. Other symptoms include
cold intolerance, gradual weight gain, lethargy, and consti,
pation. School performance may or may not be impaired.
Signs: Affected children may have coarse, puffy facies with
a flattened nasal bridge; immature body proportions (short
stature, upper segment and lower segment ratio is infantile),
pauciry of speech and movement, bradycardia, non-pitting
mlxedema, thin hair, and dry skin. Deep tendon reflexes
show delayed relaxation time. Goiter may or may nor be
present.
lnvesligolions
1. The diagnosis is made on rhe basis of documentation
decreased serum concentrations oftotal To, decreased T,
resin uptake, and elevated serum concenrrarions of TSH.
2. Skeletal maturation may be markedly delayed:
(a) X-ray of wrist joint for bone age (can be done for
1-13 year of age)
(b) X-ray of Ll, T12 vertebrae (for anterior beaking)
(c) X-ray of skull (for intersutural wormian bones, elon-
gated sella)
3. The presence of circulating thyroid autoantibodies (anti-
peroxidase, antithyroglobulin) implies an auroimmune
basis for the disease.
A eemTc thyroid scan is not indicated unless there are irregu-
larities in thyroid consisrency on palpation. In that case, a
scan looking for a thyroid nodule would be appropriate. Scan
should also be done in thyroid ectopia.
Treotment
Thyroid hormone replacement therapy is begun with synthetic
thyroxine 3-5 p.glkg as a single daily oral dose (to be given
in empty stomach regularly). Adults require 2 mglkgl24 hr.
The adequacy of replacemenr can be judged by measurement
of serum Tn and TSH, which should be in the normal range.
Follow-up
o Clinical:
is o Overdose manifestations (restlessness, sleeplessness, diar-
rhea, tremor, arrhythmia, weight loss, craniosynostosis)
should be looked for.
c Growth and neurological developmenr are evaluated
once on every 2 months in the first 2 year with somewhat
Iessfrequent follow-up visits after 2 year of age. Growth
rate (i.e., weight, height, US:LS rario, etc.) provides an
excellent index of adequacy of therapy.
r Biochemical: T, and TSH are done usually 3 monthly.
T, and TSH should be maintained within normal range.
Raised TSH is a sensirive indicator for increasing dose of
L-thyroxine.
o Radiological: Done annually. Adequate maintenance dose
permits linear growth, does not leave bone age retarded.
However, it is undesirable to permit bone age to far beyond
chronological age (i.e., over 2 year) or premarure fusion of
the epiphyses.
It results from excessive secretions of thyroid hormones. Girls
are more commonly affected than bovs (in a ratio of 4:l), and
there often is a family history of Graves disease or Hashimoto
thyroiditis. The usual age at presenrarion is adolescence; it is
unusual before 5 vear of age.
of
ETIOLOGY
o Graves disease (thvrotoxicosis) is the mosr common cause
of hyperthyroidism. It is an auroimmune thyroid disorder
in which enlargement and hyperfunction of the thyroid
I
gland may be stimulated by circulating immunoglobulins, \
a thyroid-stimulating immunoglobulin (IgG) that binds to 1
thyrotropin receprors on thyroid cells. The increased levels 1
I
t
a

of free Tn suppress TSH to undetectable levels. Thus, thyroid
hyperfunction is not TSH dependent. Thyroid receptor
stimulating antibody (TRSAb) causes uncontrolled synthesis
and release of thyroid hormones.
o Neonatal Graves disease: Neonatal hyperthyroidism is
thought to be caused by transplacental passage of thyroid-
stimulating immunoglobulins.
o 'Other etiologies for hyperthyroidism in children are rare
but include hyperfunctioning "hot" thyroid nodule and
acute suppurative thyroiditis.
CtINICAt FEATURES
Symptoms: The onset of symptoms is insidious, and emotional
labiliry increased appetite, heat intolerance, palpitation, weight
loss, frequent loose stools, deterioration of behavior and short
attention span, school performance, and poor sleeping are the
most common symptoms.
Signs: The child appears fidgety, flushed, and warm. Marked
tachycardia (sleeping), fever, diaphoresis, nausea, and vomit-
ing indicate thyroid storm, which is a sudden exacerbation
of symptoms.
r Proptosis and widened palpebral fissures may be present. Lid lag
in looking downward, improvement of convergence, retraction
of upper eye lids with infrequent blinking may be presenr.
o The thyroid gland usually is diffusely enlarged, smooth,
firm, and nontender.
o The precordium is hyperactive, and resting tachycardia,
widened pulse pressure and hypertension are present.
o The skin is smooth, warm, fushed, and moist.
o A fine tremor of outstretched fingers may be seen.
Neonatal Graves disease: Some infants who are born to
women with Graves disease exhibit jitteriness, stare, hyperac-
I tiviry increased appetite, and poor weight gain. tchycardia is
present, and thyromegaly may be detectable. Thyroid hormone
I levels are elevated above the normal range for the newborn,
t CtINICAt
and TSH is suppressed.
i o
INVESTIGATIONS
t fusely enlarged, and the surface may feel pebbly. \With long
I o Hyperthyroidism is diagnosed by documentation of increased
i serum concentrations of total T, and total Tr, increased To
resin uptake, and low or suppressed levels of TSH. If T1
levels are borderline, absence of the TSH response to TRH
injection indicates autonomous thyroid hyperfunctions.
r Presence of thyroid antibody (TRSAb)-confirmatory.
o Radioiodine uptake-rapid uptake and rapid turnover.
TREATMENT
a. Medications:
1. Initial treatment consists of antithyroid medication,
eirher propvlthiouracil (PTU) 5-10 mg/kg/d 8 hourly
!
ENDOCRINE AND METABOLIC DISEASES
or methimazole (0.25-1.0 mg/kg/d) in three divided
doses. Clinical response is obtained within 3-6 week,
and adequate control is evident in 34 months. The
dose is then decreased to minimal level required to
maintain euthyroid state. Tieatment may be necessary
5 years or longer. PTU offers the advantage ofblocking
the peripheral conversion of Tn to T.. The addition of
propranolol (0.52 mg/kg/d) four times daily may give
symptomatic relief.
About 5o/o of patients experience side effects (e.g.,
skin rash, arthralgias, drug-induced hepatitis) whiie
on antithyroid medication. Less common, but more
serious, is the occasional occurrence of agranulocytosis.
2. About 40-50o/o of children with Graves disease go into
a natural remission and may be taken off antithyroid
medication after 12-24 months of treatment.
b. Surgery: Subtotal thyroidectomy usually is selected for recur-
rent hlperthyroidism after a course of medical trearmenr or
if the patient is noncompliant with medical therapy.
c. Radioactive iodine: Although ablation of thyroid tissue
by radioactive iodine has traditionally been reserved for
adults, it has been used regularly in some centers as the
preferred treatment for children with no untoward efFects
on subsequent fertility or fetal wastage.
d. Treatment for neonatal Graves disease: Therapy with
PTU (5-10 mg/kg every 5 hours) is given. Propranolol
and iodide solution may be added in very symptomaric
infants. Neonatal Graves disease usually resolves over the
first several months of life.
Itis commonly referred to as Hashimoto thyroiditis and is the
most common thyroid condition in childhood and adolescence.
Girls are affected more than twice as often as boys.
FEATURES
Asymptomatic thyroid enlargement (goiter) is the most
common presenting complaint. The thyroid gland is dif-
duration, the gland becomes hard and nodular.
o Although most children are euthyroid, a few may be hypo-
thyroid and very rarely some may have symptoms of thy-
rotoxicosis (Hashitoxicosis). Occasionally, distinguishing
Hashimoto thyroiditis from Graves disease may be difficult,
because elements of both diseases mav coexisr.
INVESTIGATIONS
r The most significant laboratory test supporting the diagnosis
is the presence of high titers of thyroid autoantibodies in
the serum. Antithyroglobulin and antimicrosomal antibodies
are most commonly found.
ESSENCE OF PEDIATRICS

Measurement of serum concentration of T, and TSH may o Gestational diabetes mellitus

be normal, or Tn levels may be normal with elevated TSH r Neonatal diabetes mellitus
("Compensated" hyperthyroidism), or T, levels may be ,r Jixn5isnl-without recurrence
decreased with elevated TSH (hypothyroidism). o Jian5is11-recurrence 7-20 year later
Thyroid scanning is not indicated unless a nodule is suspected. o Permanent from onset

TREATMENT CtINICAI FEATURES

L-thyroxine is reserved for those children with evidence of May present at any age, most common in early adolescence.
hypothyroidism, either decreased serum concentrations of T, Classical symptoms are:
or normal T, with elevated TSH. The disease may resolve
completely with or without treatment in up to 50% of children
o Polyuria (nocturia may occur)
with Hashimoto thyroiditis. In some children, thyroid function
o Polydipsia
continues to deteriorate, and permanent hypothyroidism results.
o Polyphagia
o \Teight loss
o \(/eakness

Diabetes mellitus (DM) is a syndrome characterized by hyper- INVESTIGATIONS

glycemia caused'by absolute or relative deficiency of insulin
New diagnostic criteria for diabetes mellitus:
secretion, and resulting in abnormal metabolism of carbohy-
drate, protein and fat as well as electrolyte disturbances. o Symptoms (polJ'uria, polydipsia, and unexplained weight loss
with glucosuria and ketonuria) of diabetes plus a random
plasma glucose >200 mg/dl (1 1.1 mmol/L) on two separate
ETTOTOGtC CTASSTFTCATIONS
occasions (if symptoms not typical)
Typ" 1 diabetes (B-cell destruction, usually leading to of
absolute insulin deficiency): Immune-mediated idiopathic. o Fasting plasma glucose >126 mgldl (7.0 mmol/L) on rwo
Type 2 diabetes (may range from predominantly insulin occasions
resistance with relative insulin deficiency to a predominantly or
secretory defect with insulin resistance): Dominant type 2 o 2-hr plasma glucose during the oral glucose tolerance test
due to sulfonylurea receptor 1 mutation. (glucose load 1.75 glkg) >200 mg/dl on two occasions.
Other specific q'pes:
Note:
t Genetic defects ofB-cellfunction
Screening procedures, such as postprandial determinations of blood
c Genetic defects in insulin action: Typ. A insulin resis- glucose or screening oral glucose tolerance tests have yielded low detec-
tance tion rates in children, hence such screening procedures are not routinely
Diseases of the exocrine pancreas: Pancreatitis, trauma, recommended in children.
pancreatectomy, neoplasia, cystic fibrosis, hemochroma-
tosis, fibrocalculous pancreatopathy, pancreatic resec-
DI FFERENTIAT DIAGNOSIS
tion, others
t) En do crin op ath ie s : Acr o megaly, Cush in g disease, gluca- o Diabetes insipidus
gonoma, pheochromocytoma, hyperthyroidism, soma- o Chronic renal failure
tostatinoma, aldosteronoma, others r Hypercalcemia
Drug- or chemical-induced: Pentamidine, nicotinic o Hysterical polydipsia
acid, glucocorticoids, thyroid hormone, diazoxide,
B-adrenergic agonists, thiazides, B-interferon, 61hs15-
cyclosporine, tacrolimus.
COMPLICATIONS
Infections: Congenital rubella, Cytomegalovirus, Acute (metabolic decompensations)
others-hemoly"tic uremic syndrome. :
i) (Jncommon forncs of immune-mediated diabetes: o Diabeticketoacidosis
"Stiflman' syndrome, cytomegalovirus, others o Hypoglycemia

Other genetic syndromes sometirnes associated uith

o Lactic acidosis
i)
,> Nonketotic hyperglycemic hyperosmolar coma (rare) \
diabetes: Down syndrome, Klinefelter syndrome, Turner 1
Chronic (vascular affection)
syndrome, \Tolfram syndrome, Friedreich ataxia, I
Huntington chorea, Laurence-Moon-Biedl syndrome, o Macrovascular t
myotonic dystrophy, porphyria, Prader-\7illi, others
-
Cerebrovascular disease tfl

I
 !

ENDOCRINE AND METABOLIC DISEASES

Coronary artery disease o The serum sodium level usually is low or normal. The
- Peripheral vascular disease serum potassium level usually is normal or high and does
-
o Microvascular not reflect total body porassium depletion. A low serum
Diabetic retinopathy potassium level (<3.5 mEq/L) indicates profound depletion
- Diabetic nephropathy and potential for life-threatening hypokalemia during the
- Diabetic neuroparhy early course of treatment of DKA.
- a BUN is elevated, creatinine normal.
o Others: Pubertal delays, growth retardation (short stature).
a PO, is >95 mmHg (normal), PCO, is <30 mmHg (low),
and pH is 69-7.3.
TREATMENT a ECG for potassium status.
a Blood or urine culture.
Diabetes treatment includes (l) diabetic ketoacidosis rrearment,
a HbAlc.
(ii) post acidosis treatment in hospital, and (iiD home rreatmenr. a Chest x-ray.
a Urine for ketone body.
Diqbetic Keloocidosis (DKA)
Definition Management
DKA is likely to be present when: l. Emergency assessment: Confirm the diagnosis:
o Heary glycosuria (>55 mmol/L) and ketonuria, r Characteristics history-polyuria, polydipsia.
o Hyperglycemia (BG >11 mmol/L), o Biochemical confirmation-glycosuria, ketonuria, BG, pH.
o pH <7.3, o Clinical assessment-full examination paying special atten,
r Bicarbonate <15 mmol/L, tion to:
o 5o/o or more dehydration. o Severity ofdehydration
v
, o t Vomiting, and 3o/o just detectable
o + Drowsiness. - 5o/o dry mucous membranes, reduced skin turgor
- l0o/o capiilary return 3 seconds or more, sunken eyes
Severity of DKA - 10o/o+ shock, poor peripheral pulses
Severiry of DKA is categorized by degree of acidosis: - Clinical assessmenr of dehydration may be difficult,
t - especially in young children. Severity ofdehydration
o Mild: Venous pH <7.3 or bicarbonate <15 mmol/L
o Moderate: pH <7.2, bicarbonate <10 mmol/L is often overestimared.
o Severe: pH <7.1, bicarbonate <5 mmol/L o Evidence of acidosis-hyperventilation
t-
o Assessment of conscious level (and examine pupil and
(linical Features fundus ofredna)
It
, Symptoms are polyuria, polydipsia, fatigue, nausea, vomiting, 2. Resuscitation: In shock with poor peripheral pulses, or
and abdominal pain. Lethargy may progress to obtundation coma:
and coma.
t . Oxygen 100% by face mask
Signs include tachycardia, deep and rapid respiration (Kuss-
o Normal saline 0.9% 10 ml/kg over 10-30 minutes (should
maul), fruity odor of acetone breath; hypotension indicates
be repeated if peripheral pulses remain poor)
severe dehydration and intravascular volume depletion. The
o Nasogastric tube to drain stomach if there is vomidng t
child appears acutely ill, and dehydrated. Abdomen is mildly
impaired consciousness
tender. Must rule out infections.
3. Clinical observation and monitoring:
Differential Diagnosis
o Hourly
Hypoglycemic coma, uremia, diarrhea with metabolic acidosis, o Pulse rate, respiratory rate, blood pressure.
meningitis, encephalitis, septicemia, salicylate intoxication. o Accurate fluid input and output (when level ofconscious-
ness is impaired, a urinary catheter may be necessary).
lnvestigations Test each urine specimen for glucose and ketones.
o Hyperglycemia (glucose >300 mg/dl), ketonemia (>1:2 a Hourfu or more frequent: Neurological observations
dilirtion of serum), acidosis (pH <7.3 and bicarbonate <15 a ECG monitoring in severe DKA to assess T-waves
mEq/L), glucosuria, and ketonuria.
o 4. Rehydration and insulin management:
The hemoglobin and hematocrit usually are increased
because of hemoconcentrations. TLC may show marked a Fluids:The cause of cerebral edema during rrearmenr remains
Ie ukocrtosis. unclear. However, too rapid reduction in intravascular
ESSENCE OF PEDIATRICS

Table 12.4t Maintenance Fluid Volumes for Different Ages If serum potassium is not available before the comple-
tion of resuscitation, ECG monitoring is recommended
before potassium is added to the infusion fluid
<,1 3-9 BO Starr potassium replacement as soon as resuscitation is
1-5 't
0-1 9 7A
completed and the ECG does not show elevated T-waves
(or ifserum potassium is not elevated)
6-9 2A-29 60
r) Potassium chloride 40 mmol is usuailv added to each
1 0-14 30-50 50
liter of saline infusion.
>15 >50 35
c Insulin:
i) Insulin should not be started until shock has been suc-
osmolality may aggravate the process. It seems prudent cessfully reversed by emergency resuscitation and a sa-
therefore that rehydration should occur more slowly in line/potassium rehydration regimen begun (this avoids
children with DKA than in other causes of dehydration. sudden influx of potassium from plasma into cells, with
Proceed with urgency, but with caution. Use either Model danger of cardiac arrhythmia).
I ot 2 for Fluid calculation. During the first 60-90 minutes of initial rehydration,
the BG may fall substantially even without insulin treat-
Model 1: Requirements = Deficit + Maintenance
ment.
Deficit = Estimated 0/o dehydration
" body weight (kg and Insulin by continuous low-dose fV infusion is the
equivalent in ml) optimal method [An initial bolus of insuiin is not
Maintenance (ml)-see Table 12.4. recommendedl.
Then add deficit to 48 hr maintenance and replace this volume r) A solution of soluble insulin 1 unit/ml made up in nor-
evenly over 48 hr as normal saline 0.97o initially. mal saline is best by electronic pump.
Recommended initial insulin dose = 0.1 units/kg/hr (for
Model 2: Maintenance + 10% Deficit (given evenly over 48 hr example, dilute 50 unit regularfsoluble] insulin in 50 ml
in chiidren of all sizes) normalsaline. 1unir= I ml)
o 6 ml/kg/hr for chiidren weighing 3-9 kg tVhen syringe pumps are not available, a separate low-
,r 5 ml/kg/hr for children weighing 10-19 kg dose infusion may be given, e.g., soluble insulin 50 units
,> 4 mllkglhr for children weighing >20 kg (up to maximum of in 500 ml normal saline (i.e., I unit insulin per 10 ml
250 ml/hr) saline), the bag being changed every 24hr to avoid inac-
These calculationswill usually cover ongoing losses, which in tivation of insulin.
most cases do not need additional replacement, but excessive \7hen insulin infusion methods are not available , the use
continuing fluid losses might need replacing if the severity of hourly IM/SC injections of soluble or rapid-acting in-
of dehydration is not improving sulin 0.3 units/kg SC initial dose followed I hour iater by
When the BG falls to 14-17 mmol/L, the infusion 0.1 unit/kg every hour has been shown to be effective.
should be changed to a fluid containing glucose, the most After resuscitation, the typical rate of fall of BG is
commonly recommended being saline 0.45o/o (or 0.9olo) 2-5 mmol/hr.
'When
with glucose 4-5o/o (or glucose 5o/o with added sodium IJ BG falls to 14-17 mmol/L, change to the glucose
chloride 80 mmol/L or more). saline infusion (as above) to maintain BG in the desired
It may be necessary to use 107o or even 1'2.5o/o dextrose range of 8-i2 mmol/L.
to prevent hypoglycemia while continuing to infuse insulin If blood glucose falls very rapidly (>5 mmol/Lihr) after
to correct the metabolic acidosis. initial fluid expansion, consider adding glucose even be-
fore plasma glucose has decreased to 17 mmol/L.
o Oral fluids:
If BG rises again above 15 mmol/L, increase the insulin
o In severe dehydration and acidosis, only allow sips of infusion by 25o/o.
cold water or ice to suck r) If BG A[s to <8 mmol/L, or falls too rapidly >5 mmol/L/
,r Oral fluids (e.g., fruit juice/oral rehydration solution) hr, increase the concentration of glucose to 10% (or
should only be offered after substantial clinical improve- more) with added saline.
ment and no vomiring r) The insulin infusion rate should only be decreased if the
o Oral fluid volume should be subtracted from the IV cal- BG level remains below the target range despite glucose
culations supplementation.
o Potassium: Do not stop insulin infusion or decrease below 0.05
o Total body potassium is always substantially depleted in units/kg/hr because a continuous supply of both insulin
DKA and giucose substrate is needed to promote anabolism
o Serum/plasma potassium may be low normal, or high and reduce ketosis.

t
 ENDOCRINE AND METABOLIC DISEASES

o Bicarbonate:Therc is no evidence that bicarbonate is either
necessary or safe in DKA. Bicarbonate should not be used
in the initial resuscitation. Potential hazards ofbicarbonate
therapy:
r Exacerbation of CNS acidosis
; Hypokalemia and altered calcium ionization
r Excessive osmolar load
r cTissue hypoxia
r Persistent acidosis is likely to be caused by inadequate
resuscitation, inadequate insulin effect, or sepsis
r Bicarbonate may be considered for treatment of im-
paired cardiac contractility in persistent severe shock (If
bicarbonate is considered, proceed with caution giving
1-2 mmol/kg bicarbonate over 50 minutes).
include (i) younger age, (ii) new onset diabetes, and (iii) Ionger
duration of symptoms. Epidemioiogically potential risk factors
at diagnosis or during treatment of DKA include:
o Greater hypocapnia at presentation after adjusting for degree
of acidosis.
o Increased serum urea nitrogen at presentation.
o More severe acidosis at presenrarion.
o Bicarbonate treatment for correction of acidosis.
o An attenuated rise in measured serum sodium concentra-
tions during therapy.
o Greater volume of fluid given in the first 4 hours.
o Administration of insulin in the first hour of fluid treatment.
'Warning
signs/symptoms of cerebral edema:

5. Monitoring progress:
o Headache and slowing of heart rate
o Change in neurological status (restlessness, irritability'
o Capillary ,BGr Monitor hourly (cross-check every 2 or increased drowsiness, incontinence)
4 hr against laboratory venous glucose). o Specific neurological signs (e.g., cranial nerve palsies)
c Laboratory /ests.' Electrolytes, urea, BG, and blood gases r fusing blood pressure
should be repeated every 2-4 hr until acidosis is reversed. o Decreased O, saturation
o Sodium and osmolality: Despite the depletion of total body
sodium in DKA, the elevated osmolality in the hypergly- Diagnostic criteria:
cemic state results in a dilutional effect on the measured o Abnormal motor or verbal response to pain
sodium. a Decorticate or decerebrate posture
r Serum sodium often rises as the BG fails. Theoretically, a Cranial nerve palsies
sodium should rise by 2 mmol for every 5.5 mmol fall in a Abnormal neurogenic respiratory pattern (e.g., gruntinS,
BG, resulting in a slower fall in osmolality. tachypnea, Cheyne-Stokes respiration)
r Serum osmolaliry can be measured directly or calculated Major criteria:
from: Serum osmolality (mOsm) = 2 x (Na + K) + BG
(mmol) a Altered mentation/fluctuating level of consciousness.
Sustained heart rate deceleration (decreased more than 20
I
a
i r ,{n initial serum sodium >150 mmol/L might prompt an
I eten slol-er rehvdration rate than 48 hr beats per minute) not attributable to improved intravascular
volume or sleep state.
i o Potassium.' The potassium inhrsion should be titrated to Age appropriate i nconrinence.
I ;:i:inrain s-rum potassium u-ithin rhe laboraton' normal
I
fang-. Minot'aiteria:
t
:
t L'ine output: If this is inadequate (<1.5 mli kglhri. the cause o Vomiting
r must be souqht ie.q.. acute renal fhilure. continuing shock' o Headache
I
) urinan' obstruction. bladder retention). If fluid retention is o Lethargy or not easily arousable
I occurring, there is some evidence that a single dose of a loop o Diastolic blood pressure >90 mmHg
I
diuretic might be helpful in promoting water diuresis. o Age <5 years
r
I
6. Transition to SC insulin injections: One diagnostic criterion, two major criteria, or one major and
!
I o Insulin by SC injection may be started when oral intake two minor criteria have a sensitivity of 92o/o and a false-pr':;irive
) is tolerated rate of only 4o/o.
!
The dose and rype of SC insulin given will depend on local
t circumstances
Treatment of cerebral edema:
I
r To prevent rebound hyperglycemia, do not stop the IV a Initiate treatment as soon as the condition is suspected.
t insulin infusion until 60 minutes after the first SC iniection a Reduce the rate of fluid administration by one-third.
i
of short- or rapid-acting insulin. a Give mannitol 0.5-1 g/kg IV over 20 minutes, and repeat
i if there is no initial response in 30 minutes to 2 hours'
I
Hypertonic saline (30lo), 5-10 ml/kg over 30 minutes mav
,
)
Complications be an alternative to mannitol or a second-line therapv if
! (erebral Edema Incidence is 0.5-0.9%; mortality rate2|-24o/o. there is no initial resPonse to mannitol'
7
Factors associated with an increased risk of cerebral edema Elevate the head of the bed.
;

t
F

ESSENCE OF PEDIATRICS
o Intubation may be necessary for the parient with impending
respiratory failure.
o After treatmenr for cerebral edema has been started, a cranial
CT scan should be obtained to rule our orher possible
intracerebral causes of neurologic deterioration, especially
thrombosis or hemorrhage.
o Mannitol should be immediately available during the treat-
m.ent of DKA.
Hypoglycemia and Hypokalemia Avoid by careful monitoring
and adjustment of infusion rates.
Aspiration Pneumonia Avoid by nasogastric tube in vomiting
child with impaired consciousness.
0thetAssociationswithDKA These require specific managemenr,
e.g., continuing abdominal pain (due to liver swelling/gastritis/
bladder retention, but beware of appendicitis), pulmonary
edema, unusual infections (e.g., TB), hyperosmolar hypergly-
cemic nonketotic coma.
Note: To prevent rebound hyperglycemia, do not srop rhe IV
insulin infusion unril 60 minute after the first SC injection
of short- or rapid-acting insulin.
Post Acidosis Treotmenl
Subcutaneous insulin therapy should be started when acidosis
subsides. A mixture of regular and intermediate-acting (lente/
NPH) insulin is given rwice a day, half an hour before breakfast
and dinner. The total daily dose (0.5-1.0 U/kg) is roughly
divided into rwo-thirds to be given before breakfast and one-
third before dinner. Two-thirds of each dose should consist of
lente insulin and one-third of regular insulin (approximately).
These arejust initial guidelines. From the next day, doses should
be titrated according to blood glucose responses obtained the
previous day.
Home Treolmenl
lnsulins
At the onset of diabetes or after recovery from ketoacidosis,
the total daily dose of insulin is about 0.5-1.0 U/kg. The
actual total daily requirement of insulin is estimated from
the representative 24-hr period when only regular insulin was
administered during the transition phase after resolution of
ketoacidosis.
a) Two daily injections: Recommended routinely. In this
plan, nvo-thirds of the daily total dose is given before
breakfast and one-third before dinner; each injection con-
sists of intermediate- and short-acting insulin in propor-
tions of 2-3:1. For example, assuming a total daily dose of
1 U/kg for a 30-kg chlld, t4 U of NPH combined with 6
U of regular insulin would be given before breakfast and
6 U of NPH with 4 U of regular insulin wouid be given
before the evening meal. These are just initial guidelines.
From the nexr day, doses should be titrated according to
\
blood glucose responses obtained the previous day, i.e.,
as with single daily dose regimen (see beiow).
b) Single daily injection: Many children can be managed with
single daily injection. One intermediate insulin (NpH),
plus (because ofits delayed action) a fast-acting (regular)
insulin is usually combined with it-approximately two-
thirds of the total dose is an intermediate-acing insulin
(i.e., NPH or lente) and the remainder is regular insulin; the
injection is given 30 minutes before breakfast. Ad.jusrments
in the dose of insulin are made in relation ro the partern
of blood glucose values. If hyperglycemia or glucosuria
occurs during late morning, rhen the regular insulin is
increased by 10-l5o/o.If the predominant hyperglycemia
or glucosuria occurs during late afternoon or evening, then
the intermediate-acting insulin is increased by l0-l5o/o.
Should hypoglycemia occur berween mid morning and
noon, the regular insulin is reduced by l0-l5o/o; if
hypoglycemia occurs in late afternoon, the intermediate-
acting insulin is decreased by 10-l5o/o.
The two insulins, regular and NPH, can be drawn
into the same syringe-first regular, followed by lente-
to be given as a singie injection. Injections are given
SC, rotating sites on back of arm, thighs, bunocks, and
abdomen in a regular sequence; rotation helps to ensure
adequate absorption of insulin, prevent fibrosis, and mini-
mize lipodystrophy. Children over 10-72 year should be
encouraged to administer their own insulin. Long-acting
insulins (ultralente) are nor often used in children.
Nutrition
1000 calories at 1 year, and additional 100 calories per year of
age up to puberry. Calorie mixture should consist approximately
of 50o/o carbohydrate, 30o/o fat, and l5o/o protein. Complex
carbohydrate such as starch is better. Sucrose and highly refined
sugar, carbonated beverages should be avoided. Aspartame is
used in a variery of products. Sorbitol should be avoided.
Diets with high fiber (legumes, whole-meal bread, fruits,
vegetables) are useful.
Dietary fat from animal sources should be reduced and
are replaced by polyunsaturated fats from vegetable sources.
Substituting margarine for butter, vegetable oil for animal oil
in cooking, lean cuts of meat, poultry and fish for fatry meats.
Egg yolk consumption should be reduced.
The dayt meal plan should be divided into three meals
(breakfast, lunch, dinner) and 2-3 snacks (mid-morning,
evening, and bed time). Occasional excesses for birthdays and
other parties are permissible and are tolerated in order not to
foster rebellion.
Monitoring
Home monitoring: With the advent of glucose oxidase strips,
blood glucose can be measured at home by a visual reading of
I
1
color change on the strips. Urine glucose (positive only when 1
t
rl
 I

ENDOCRINE AND METABOLIC DISEASES

blood glucose rises above 180-200 mg/dl) is less informative Clinical Features
than blood glucose testing. A combination of blood and urine
a
Adrenergic symptoms (sweating, pallor, trembiing, tachycar-
glucose testing schedules can be used.
r
dia) and cerebral glycopenic symptoms (personality changes,
ideally capillary blood glucose should be tested daily fasting,
drowsiness, confusion, coma, convulsion).
r pre-lunch, pre-dinner, and at bed time.
Patients are taught to recognize these symptoms. Hypoglyce-
a
Goals of conventional and intensive therapy: mia occurs suddenly or over a span of minutes, in contrast to
diabetic ketoacidosis, which develops over hours or days. Thble
o Conventional therapy:
12.5 lists the features differentiating coma due to hypoglycemia
with that due to ketoacidosis.
o Premeai blood glucose 120-160 mg/dl
,> Absence of polyuria anll ketonuria
Treatment
o Intensive therapy:
,; HbA,. 6.0-7.0o/o o lJnderstanding of the symptoms and signs of hypoglycemic
,r Premeal blood glucose 80-120 mg/dl reaction by the patients and the family members.
o Post-meal blood glucose <180 mg/dl o Confirmation of hypoglycemia by a blood glucose test'
o For immediate rise in blood sugar, simple sugar (5-10 g= 1-2
Laboratory monitoring: Long-term glycemic control is pro- 5-ml spoonfuls) in the form of sugar, glucose, fruit juice,
duced by measurement of glycosylated hemoglobin (HbA,.), or as carbonated drink (- I glass) must be taken.
which represents the fraction (o/o) of hemoglobin to which 0.5 mg glucagon IM should be given when patient is
glucose has been non-enzymatically attached in the blood strealn. losing consciousness or is vomiting (if giucagon is not avail-
The formation of HbA,. is a slow reaction that is dependent able, the child should receive 25o/o dextrose 2-4 mllkglY).
on the prevailing concentration ofblood glucose; it continues
irreversibly throughout the red cells lifespan of approximately
o After an episode of severe hypoglycemia is treated, the family
must assess the reasons for its happening.
120 days. The higher the blood glucose concentration and the
longer the red blood cells exposure to it, the higher the frac-
r Ifexercise has been the precipitating factor, take additional
calories prior to exercise.
tion of HbAr., which is expressed as 7o of total hemoglobin.
Because a blood sample at any given time contains a mixture
o Reductions of 10-15% of insulin dosages shouid be
of red blood cells of varying ages, exposed for varying times instituted, when hypoglycemia shows a recurring
pattern.
to varying blood glucose concentrations' an HbA,. measure-
ment refects the average blood glucose concentration during
o Missed meals should be avoided.
2-3 months.
the preceding
HbAr. measurement can be done once on every 3
months.
HbA,. fraction is usually <7o/o in normal individuals; in
diabetics 6-970 represents very good metabolic control, 9-72o/o
fair control, and values above I2o/o poor control. Table 12.5: Differences in Coma due to Hypoglycemia and
Ketoacidosis in IDDM
Exercise

Exercise should be encouraged. A major complication of History No foocl; too much insulin; Too little or no insulin;
exercise in diabetic patients is the presence of a hypoglycemic Unaccustomed exercise an infection; digestive
disturbance
reaction during or within hours after exercise. If hypoglycemia
Onset In good previous health; lll-health {or several clats
does not occur with exercise, adjustments in diet or insulin are
retated to last insulin
not necessary, and glucose regulation is likely to be improved injection
through the increased utilization of glucose by muscles. (Jlycosurra an0
Symptoms Hypoglycemia; occasional
vomiting from depot dehydration, abdominai
Patient Education insulins pain, and vomiting.
5r8ns Moist skin and tongue; full Dry skin and tongue; week
Education of the patient and family plays an integral role in pulse; low blood pressure;
pulse, normal or raised
the management diabetes. BP. shallow or normal air hunger; diminished -
breathing; brisk re{lexes reflexes
Hypoglycemic Reoction (lnsulin Shock) Urine No ketonuria; no Ketonuria: glucosuria
gl ucosuna
\lismatch berween insulin dose on the one hand, and meal
Blood Hypoglycemia; normal Hyperglycemia; reduced
and exercise on the other hand results in hypoglycemia (with plasma bicarbonate
plasma bicarbonate
bLood elucose of <60 mg/dl).
 I
ESSENCE OF PEDIATRICS

TREATMENT IN SPECIAT SITUATIONS Two types:

Treolment of Diobeles Mellitus during Surgery

o Pancrearic fibrosis
o Pancreatic calcification
'When
surgery is elective:
r The patient should be admitted 24 hour prior to surgery; lnvestigations
during this time, the usual nutritional requirements and o Pancreatic calculous is demonstrable by x-ray, ultrasonogram,
insulin doses are provided. CT scan, ERCP
o On the morning of surgery, an infusion of 57o glucose in o Insulin and glucagon secretary capacity is low
0.45o/o saline plus 20 mEq/L of potassium chloride is begun; o Pancreatic exocrine function is normal
initially iU of regular insuhn is added to the infusate for
each 4 g of administered glucose.
Treatment
r The rate of infusion should provide maintenance fluid
requirements, plus estimated losses during surgery. Insulin can be used in the rreatment of FCPD.
o The blood glucose concenrration should be monitored at
periodic intervals before, during, and after surgery; con-
centrations of approximately 120-150 mg/dl should be rhe
goal; this can be achieved by varying the rate of infusion DI may result from either a lack of arginine vasopressin (AW;
of the glucose and electrol;,te mixture, or the amount of antidiuretic hormone IADH]), or failure of AVP-sensitive epi-
insulin added. thelial cells of the kidney collecting duct to respond normally
o This regimen may be discontinued when the parienr is awake to the hormone.
and capable of taking food and fluid orally. Quantitatively, urine volume is >2 lirers per day (>30 ml/
r Prior to reinstitution of the parient's usual diet, regular kg/d) with a specific gravity <1010 or osmolaliry <300 mOsm/
insulin may be administered at a dose of 0.2 lJlkg at 6-hr kg water. \
intervals; approximate adjustments in the dose are based on
1. Neurogenic diabetes insipidus (central DI): Patients are
blood or urinary concenrrations of glucose.
unable to produce appropriate levels ofvasopressin.
For emergency surgery:
Causes in children:
r An infusion is initiated that provides 5-10olo glucose in
0.45o/o saline, 20 mEq of potassium chloride, and 1 U of
o Genetic: Congenital malformation of hypothaiamus and
pituitary.
regular insulin for each 2-4 g of glucose. Blood glucose con-
centration should be maintained at approximately 120_150
r Infections: Viral encephalitis, bacterial meningitis, tuber-
culosis, tuberculoma.
mg/dl.
r \When possible, rehydration and metabolic o TLmors: Germinoma, pinealoma, craniopharyngioma, optic
balance should
glioma, histiocytosis, AML.
precede the surgery.
o After surgery, the regimen described earlier can be instituted.
o flauma including surgery.
For minor surgery under LA, usual insulin and dietary
o Drugs: Phenytoin, halothane, opiare antagonist.
regimens can be maintained. Causes in newborn infants: Birth asphp<ia, intracranial bleed-
ing, and meningitis.
Treqlment of Diobetes Mellilus during Pathophysiology: See Fig. 12.3.
lnfeclions or Troumq
2. Nephrogenic diabetes insipidus: Occurs due to unrespon-
o Requires additional insulin.
siveness of the kidney to vasopressin (vasopressin recepror
o In face ofexcessive glucosuria, added l0-20%o ofthe total defects). It is a sexlinked recessive disorder.
daily dose as regular insulin prior to each meal.
r Subsequent increase or decrease should then be based on Pathophysiology: See Fig. 12.4.
careful monitoring of urinary or blood glucose values.
3. Compulsive water drinking: Polyuria due to compulsive
water drinking occurred usually as a result ofpsychogenic cause. :
Fibrocqlculous Poncreqlic Diqbeles (FCPD)
Pathophysiology: See Fig. 12.5.
Occurred due to calcific chronic pancreatitis, which may clini-
call;' be insulin-dependent or non-insulin dependent. History
a
of recurrent abdominal pain may be obtained. CtINICAI FEATURES t
Protein deficient pancrearic diabetes (PDPD) as described 1
bv \MHO has largely been discarded now, as no direc role of Polydipsia, irritabiliry failure to rhrive, intermittent fever, and 1
protein deficiency in the etiology of diabetes has been found. visual disturbance may occur. ttl
E
li I
 ENDCCRINE AND METABOLIC DISEASES

o If plasma osmolality reaches >300 mOsm/kg and urine

osmolality <660 mOsm/kg, then administer DDAVP
Interpretation:
.LVasopressin secretion
Diabetes insipidus is confirmed by a plasma osmolality
>300 mOsm/kg with a urine osmolality <600 mOsm/kg.
Central diabetes insipidus is confirmed if urine osmolalitv
rises to >660 mOsm/kg after DDAVP
Nephrogenic diabetes insipidus is confirmed if DDAW
JFeabsorption of water from DT & CD does nor corrcenlrare rhe urine.

tDilute urine flow TREATMENT

Adequate fluid intake should be allowed.

Neurogenic diabetes insipidus:

o Neonates and infants ale best treated solely with fluid therapy
Slight rise in plasma osmolality (3 Llm'1l24hr) with high nutritive value.
r In children, drug of choice is desmopressin (DDAW [desa-
mino-D-arginine vasopressin]), a long acting analogue of
lncreased thirst vasopressin. Given as a nasal drops or spray, usual dose is
5-10 pg daily either as a single or 2 divided doses. Children
under 2 year require lesser doses (0.15-0.50 mg/kg).
l-lurci rntaKe
o Oral chlorpropamide 20 mglkgl24 hr in 2 divided doses'
It increases the sensitivity of the renal tubule to vasopres-
Replenish decreased body water sin; it reduces polydipsia and polyuria in partial deficiency.
ffi
@ Potential side effects are hypoglycemia, SIADH.
Fig. 12.3: Pathophysiology of neurogenic diabetes insipidr-rs Nephrogenic diabetes insipidus:
Thiazide diuretics: Hydrochiorothiazide 1 J mgl kgl d improves
polyuria by reduction of extracellular volume and increase
INVESTIGATIONS in proximal tubular reabsorption with or without amiloride
o Polyuria (>2 Llm2l24hr) 20 mgll.73 m)124 hr: it also reduces urine outPut.
o Serum osmolality >300 mOsm/kg a I-ow solute diet.
a Indomethacin: Patients who fail to respond to a low solute diet,
o lJrine osmolality <300 mOsm/kg
diuretics may be candidates for treatment along with inhibitors
r \(/ater deprivation test
of prostaglandin synthetase (i.e., indomethacin).
teatment of cause, if any.
Woler Deprivolion Tesl
If serum osmolality <300 mOsm/kg but >270 mOsm/kg with
polyuria and polydipsia presents, water deprivation test is nec-
essary for diagnosis of diabetes insipidus and to differentiate Vasopressin secretion capacity - normal
cenrral lrom nephrogenic car.tse.

Procedure: Raised plasma vasopressin in face oJ high plasma osmolality

o No coffee or tea on the test day.
o Free iluids until 07.30 a.m. on the morning of the test, but
Renal insensitivity for vasopressin (complete/partial)
discourage patients from extra fluid.
r Attend at 08.30 a.m. and record body weight, and plasma
and urine osmoialiry.
Decreased renal reabsorption of water
r Record body weight, urine volume, urine and plasma
osmolaliry and thirst score every 2 hours for up to B hours.
r Stop the test if the patient loses 5%o of body weight or tOitute urine flow
uhen urine osmolaliry does not change significantly
130 mOsm/kg) in three consecutive determinations. Fig. 12.4: Pathophysiology of nephrogenic diabetes insipid
ESSENCE OF PEDIATRICS

Normal vasopressin secretory capacity (Addison disease) or a lack of ACTH stimulation (secondary
adrenal insufficiency).

CONGENITAI. ADRENAL HYPERPTASIA

The clinical characteristics of congenital adrenal hyperplasia
(CAH) depend on which enzyme in the pathway of cortisol
synthesis is deficient. Even for a specific enzyme, variabiliry
exists in the severity of disease expression and timing of onset
of symptoms. It is helpful to review the pathways of adrenal
steroidogenesis (Fig. 12.6) to appreciate better the consequences
toilute urine flow ofan enzyme defect on decreased synthesis ofcortisol or aldos-
terone, increased ACTH production (low cortisol production
Fig. 12.5: Pathophysiology of compulsive water drinking. results in increased ACTH production and adrenocortical
hyperplasia), and over-production ofprecursors rhar are shunted
to androgens. The wo mosr common defects are 2l-hydroxylase
deficiency and 1 1-hydroxylase deficiency.

21 -Hy dr oxylose Deficiency

The products of adrenocortical steroidogenesis are glucocorti-
2l-Hydroxylase deficiency accounrs for 90o/o of cases of con-
coids, mineralocorticoids, and sex steroids. Cortisol, the major
genital adrenal hyperplasia and occurs in several forms (classic
glucocorticoid, is stimulated by pituitary ACTH. Aldosterone,
salt-wasting, simple virilizing, and nonclassic). These disorders
the principal mineralocorticoid, is controlled by the renin*
are inherited as autosomal recessive traits.
angiotensin system. The major sex steroids are androgens.
Inherited deficienry ofvarious enzymes involved in cortisol and
(lassic Salt-Wasting 21 -Hydroxylase
aldosterone synthesis leads to congenital adrenal hyperplasia.
Autonomous hyperfunctioning of the adrenal cortex leads to Classic salt-wasting 27-hydroxylase deficiency is a severe
hypercortisolism or Cushing disease, and decreased adrenocorti- deficiency resulting in decreased cortisol and aldosterone
cal secretion may be caused by primary adrenal insufficiency secretion, increased ACTH, and increased precursor

Testis
:t:t

44 Pathway : Progesterone 1 7a-Hyd roxyprogesterone Androstenedione :e Testosterone

(1) = 21o-Hydroxylase

(2) = 1 1B-Hydroxylase

Fig. 12.6: Summary of steroidogenesis in the adrenal cortex, ovary, and testes. (1) and (2) indicate site of block.
 ENDOCRINE AND METABOLIC DISEASES

of the 21-hydroxylase step, 17 hydroxyprogesterone. Investigation: Based on the measurement of elevated 17-hy'
l7-hydroxyprogesterone is metabolized to adrenal androgens, droxyprogesterone in serum.
namely dihydroepiandrosterone (DHEA) and androstenedione.
Treatment: Principles of treatment with cortisol and follow-up
There is a one in four recurrence rate in siblings of children
are the same as for salt-wasters. Some children, even without
with classic salt-wasting 21 -hy dr oxylase defi ciency. clinical symptoms of salt wasting, have elevated levels of
t renin. The addition of a mineralocorticoid (fludrocortisone)
Clinical features:
facilitates suppression of adrenal androgens with smaller doses
o Female infants are born with ambiguous genitalia. Clitoro-
of cortisol.
megaly and labioscrotal fusion may lead to erroneous male
I sex assignment. Because there is normal ovarian develop-
L Non-classic 21 -Hydroxylase Defi ciency (Acquited or
, ment, internal genital stru0tures are female. Male infants
v have no genital abnormalities; excess pigmentation of penis, late-0nset)
t scrotum, and nipples may be present. This variant is most commonly diagnosed in female adolescents
v o Symptoms of salt wasting, vomiting, dehydration, and shock or adults.
v develop in the first 2-4 weeks of life. Infants are hypona-
L tremic, hyperkalemic, acidotic, and often hypoglycemic. Clinical features: Patients manifest signs and symptoms of
androgen excess (i.e., menstrual irregularities, hirsutism, acne,
Investigations: advanced bone age). Male and female may Present with preco-
o 17-hydroxyprogesterone-markedly increased. cious puberche.
r Serum cortisol-decreased. Investigations: Basal levels of 17-hydroxyprogesterone
v
r Serum electroll'tes: Hyponatremia, hyperkalemia, metabolic may be only modestly elevated. However, the excessive
acidosis. rise of 17-hydroxyprogesterone after ACTH stimulation is
o Blood sugar-hypoglycemia. diagnostic.
I
l Treatment: Treatment: A glucocorticoid suppresses adrenal androgens and
Infant with sah loss and shoeh: ameliorates symptoms.
o Give normal saline 150 ml/kg/d + 70o/o dextrose as neces-
sary t plasma infusion. 11 -Hydroxylqse Deficiency
o Hydrocortisone 50 mg IV IM stat and 8 hourly and oral
11-Hydroxylase deficiency accounts for about 5o/o of cases of
fludrocortisone 0. 1-0.3 mg/d.
congenital adrenal hyperplasia. Lack of 1 1-hydroxylase results
o Tieat hyperkalemia.
in decreased conversion of 11-deoxycortisol to cortisol, with
o Adjust dosage by monitoring daily Na., K. levels and fre-
precursors shunted toward overproduction of androgens' as
quent 17-OHP level.
in 21-hydroxylase deficiency. I l-Hydroxylase is also necessary
Infant without sah loss: for conversion of deoxycorticosterone to corticosterone in the
aldosterone pathway.
o Cortisol replacement (10-20 mglnlldof oral hydrocortisone
divided and given every 8 hours) to suppress ACTH and Clinical features: Overproduction of deoxycorticosterone'
overproduction of androgens. which itself has mineralocorticoid activiry results in hyperten-
r Mineralocorticoid (fudrocortisone 0.1 mg/d) adjusted to sion and hypokalemia in most of these patients.
suppress the plasma renin level.
Investigation is based on the measurement of increased
o Surgical correction of female genital abnormalities to make
11-deoxycortisol and deoxycorticosterone in serum or their
fertile.
tetrahydrometabolites in the urine. Serum androstenedione
Follow-up:lhese children must be monitored closely for linear and testosterone also elevated, and renin and aldosterone are
growth and sexual development. suppressed. In the milder nonclassic form, the biochemical

Counseling: Counseling about nature of recurrence and pre- abnormalities are expressed after ACTH stimulation.
r-ention of CAH, about life-long treatment with steriod, and Tireatment Tieatment strategies are the salne as for 21-hydroxy-
need for increase in steroid dose during stress. lase deficiency.

Simple Virilizing 21 -Hydroxylase Defi ciency
Clinical features are caused solely by overproduction ofadrenal
androqens. Therefore, only female infants with ambiguous
genitalia are diagnosed during the neonatal period. Boys and
slrls have ercessive grofih and premature appearance of pubic
h;:: precocious puberw).
Prenatal diagnosis: Prenatal diagnosis of CAH is done by
CVS (chorionic villous sampling) around 9-1 1 week, or by
17-OHP in amniotic fluid around 15-18 week.
Neonatal screening: Dried capillary blood 17-OHP is esti-
mated on 3'd_5'h day of life (obtained by heel pick and spotted
on filter paper).
 ESSENCE OF PEDIATRICS

ADRENOCORTICAT I NSU FFICI ENCY lnvesligotions

Adrenocortical insufficiency include conditions in which pro- r Estimation of serum cortisol before and after administra,
duction of one or more adrenal corrical hormones is impaired tion of ACTH (250 mg IV)
due to congenital or acquired lesions of the hypothalamus, o Low resting level and no increase afrer ACTH injec-
pituitary, or adrenal correx. tion-Addison disease
,r Low resting level and significantly increase after ACTH-
Etiology secondary insu{fi ciency

1. Primary o Plasma ACTH measuremenrs:

a. In the newborns: ,r Increase level-primary adrenal insufliciency
i) Adrenal hypopla'sia or aplasia o Low or normal-secondary adrenal insufficiency
ii) Salt-losing form of congenital adrenal hyperplasia
iii) Familial unresponsiveness ro ACTH r CRH stimulation test: IV bolus I mg/kg of CRH is
administered. Plasma ACTH and cortisol is measured ar
iv) Adrenal hemorrhage or overwhelming sepsis
(\Taterhouse Friderichsen syndrome) 0, 30, 60, 120 minutes intervals.
v) Adrenal leukodystrophy o In primary insufficiency-marked ACTH response, bur
b. In childhood-Addison disease: no cortisol response
i) Autoimmune o In secondary insufficienry-no ACTH or corrisol response
ii) TB o Other tests which are suggesrive of adrenocortical insufficienry:
iii) Fungal infection of adrenal gland o Serum electrolytes (Na- and Cl-levels decreased, Kr level
iv) Intra-adrenal hemorrhage (.Waterhouse
increased, and metabolic acidosis)
Friderichsen syndrome)
r) Blood glucose (hypoglycemia mainly in primary disorder)
v) Metastating rumor, e.g., lymphoma, r) X-ray chest (microcardia)
hemochromatosis
a.) ECG (features of hyperkalemia)
vt) Drugs: Ketoconazole, rifampicin, phenytoin,
a) USG, CI or MRI: To detect the size of adrenal gland.
phenobarbitone

2. Secondary Treolmenl
a. Hypothalamopituitary disease:
Adrenal(risis
i) Panhypopituitarism
ii) ACTH deficiency o Fluid and electrolyt es; 5o/o dextrose in normal saline 1 0-20
iii) Craniopharyngioma ml/kg given over first hour. Repeat if necessary. Subsequently
iv) Pituitary infiltration: TB, actinomycosis, sarcoidosis maintained by normal saline (volume 2 times of normal
b. Adrenal insufficiency may occur after withdrawal of maintenance fluid requirement). Intravenous glucose ( 1 0olo
steroid therapy as a result ofsuppression ofpituitary glucose2 mVkg) every 6 hourly for hypoglycemia.
ACTH. o Glucocorticoid: Inj. hydrocorrisone 15 mg for infants,
25 mg for toddlers, 50 mg for children, tOO mg for
".rJ
adolescent 6 hourly for the fitst 24 hours;
elinicql Feotures when patient
become stable reduce steroid dose in the next 24 hours.
Depending on pathologic lesions, symproms may appear Equivalent dose of dexamethasone instead of hydrocorti-
abmptly (adrenal crisis) or insidiously, beginning in infancy sone can also be used. After 48 hour, when oral intake is
or iater. satisftcrory, omit IV fluid. Give corricosrerone orally (cor-
Adrenal crisis (acute form) is characterized by vomiting, tisone 5-20 mg B hourly) and subsequently switch over to
dehydration, cold clammy skin, weak rapid pulse, labored maintenance doses.
respiration, and cyanosis. The crisis precipitated by infection, o Mineralocorticoid: Fludrocortisone 0.1 mg/d, when oral
trauma, excessive fatigue, or drugs (such as morphine, barbi- intake is rolerared.
turates, insulin, and thyroxine). o Tireatment of hyperkalemia accordingly.
In older children: o Identifr the precipitating causes(s) and treat accordingly
(anti-TB or anti-fungal trearment).
r Symptoms: Weakness, lethargy, anorexia, weight loss, and
salt craving. Chronic Adrenal Insuffi <iency
r Signs: Hypotension, increased pigmentation over face, hands,
genitalia, axilla, nipple, joints, umbilicus, and recenr scars. Maintenance therapy:
Oral mucosa is often bluish brown. Vitiligo may be inter- o Glucocorticoid: Oral hydrocortisone ar 10 mglm2ld in I
spersed with dark area. three divided doses. \
a
t
.:

o Mineralocorticoid: Fludrocortisone 0.05-0.3 mg/d.
o During period of stress, infection, and minor surgery, dose
of glucocorticoid should be doubie of the maintenance dose.
o Advice to parents:
o Offer frequent meals to children.
c Ready access to table salt.
o Keep injection hydrocortisone, which should be given
when oral medication is not possible.
o Maintain a steroid card where diagnosis, dose of steroid,
and name and address of the doctor is written.
CUSHING SYNDROME
of
Signs and symptoms of Cushing syndrome develop as a result
excessive cortisol, due to either endogenous overproduction of
cortisol or exogenous treatment with cortisol for other illnesses'
Etiology
o Bilateral adrenal hyperplasia (Cushing disease) is
most common etiology in children older than 7 years of
age. This is now generally believed to be caused by chronic
over-secretion of ACTH by a pituitary tumor. In many
instances, the tumor is a microadenoma.
o Adrenal tumors also may cause Cushing syndrome. Most
adrenal tumors are adenomas; in younger children and
infants, the possibility of malignant adrenal carcinoma is
greater. Although most adrenal tumors are virilizing, rare
feminizing adrenal tumors have been reported.
o Steroid therapy.
Clinicol Feolures
The classic manifestations of Cushing syndrome in children
are slow growth (100o/o), truncal obesity with rounded "moon"
face (95o/o), buffalo hump, purple sviae (20o/o), hirsutism and
acne (55Vo), emotional lability (40%o), easy bruising (1'5o/o),
hyperpigmentation (10%), Hypertension (25o/o) and muscle
weakness (65Vo).
lnvesligolions
Initial laboratory studies document the presence of increased
cortisol secretion. Elevated serum cortisol levels and absence
of the normal diurnal variation are difficult to interpret in the
stressed or hospitalized child.
o A24-hoar urine test for free cortisol is the most discriminat-
ing test. Failure to suppress the morning serum cortisol ievel
to <5 mg/dl after receiving 0.3 mglm2 of dexamethasone
at 11 p.m. the night before (the overnight dexamethasone
suppression test) is supportive of possible Cushing syndrome.
o A prolonged dexamethasone supPression test is needed
differentiate Cushing disease (bilateral adrenal hyperplasia
due to pituitary adenoma) from Cushing syndrome due to
adrenal tumor, if both the 24-hour urine free cortisol test
and the overnight suppression test are positive. Low-dose
ENDOCRINE AND METABOLIC DISEASES
oral dexamethasone (1.2 mglmzld divided and given every
6 hours for 2 day$ is followed by a high dose (4.8 mglmzld
for 2 day$, with monitoring of serum cortisol and 24-hov
urine free cortisol levels.
,r Ifserum and urine cortisol levels are suppressed to <50o/o
of the baseline on the first 2 days, Cushing syndrome is
not Present.
c Failure to suppress levels on the first 2 days (low-dose dex-
amethasone) but suppression on the last 2 days indicates
bilateral adrenal hyperplasia due to pituitary adenoma.
o Failure to suppress levels on high-dose dexamethasone
indicates adrenal tumor.
o Adrenal scintigraphy to detect micronodular adrenal hyper-
plasia (where CT fails).
o CT/MRI of the pituitary and adrenal areas is also warranted.
MRI is useful for pituitary microadenoma.
o Serum electrolytes.
the
Treolmenl
Bilateral adrenal hyperplasia is usually treated by surgical
excision of the pituitary adneoma. tans-sphenoidal micro-
surgery is the treatment of choice for microadenomas. Some
children have been treated with pituitary irradiation.
Adrenal tumors are treated by unilateral or bilateral adre-
nalectomy. Chemotherapy for malignant metastatic disease
may be palliative (with mitotone and cisplatin).
In all cases when surgery is performed, perioperative steroid
coverage must be provided to prevent possible adrenal
insuf[ciency.
Iatrogenic Cushing syndrome is managed by gradual transfer
to alternate day steroid therapy.
ADRENAL MEDUTLA
The adrenal medulla is composed of chromaffin cells derived
from neural crest tissue. The adrenal medulla produces
catecholamines (epinephrine and norepinephrine) in response to
sympathetic nervous system stimulation. Catecholamines exert
widespread metabolic eflects on glycogenolysis, lipolysis, and
gluconeogenesis as well as effects on the cardiovascular system.
Pheochromocylomo
It is a rare tumor of chromaffin tissue. The most common site
of occurrence is the adrenal medulla. Most tumors in childhood
are benign. Morbidity and monality result from the effects of
overproduction of catecholamines.
Clinical Features
to
Hypertension is usually sustained but may be paroxysmai. Head-
ache, palpitation, abdominal pain, vomiting, pallor, and sweating
are prominent. Hypertensive encephalopathy may be life- threat-
ening. Good appetite, but does not gain weight; growth failure.
 !

ESSENCE OF PEDIATRICS

lnvestigations b. Testosterone is converted to dihydrotestost€rone

(DHT) by the action of 5a-reductase. DHT is nec-
Investigation is based on finding elevated levels of epineph-
essary for differentiation ofthe external genitalia into
rine, norepinephrine, or their metabolites (i.e., metanephrine,
the scrotum, phallus, and phallic urethra, which is
normetanephrine) and 3-methoxy-4 hydroxymandelic acid in a
completed by l2*I4 weeks gestation.
24-how urine sample. It is significant if urinary catecholamine
c. The fetal testes also contain Sertoli cells, which secrere
is >300 ygl24h.
antimullerian factor (AMF), causing regression of mul-
Imaging studies: An attempt to localize the tumor by
lerian ducts in the male fetus by B weeks gestarion.
noninvasive measures first should be made using abdomi-
nal ultrasonography and CT or magnetic resonance imaging
(MRI) scanning. Tirmors in the thorax may be identified on
FEMAI.E DIFFERENTIATION
chest radiography or chest CT. 123l-metaiodobenzylguanidine
scintigraphy is helpful in imaging the adrenal medulla as well The female genorype is 46, )O( Female difrerentiation
as extra-adrenal chromaffin tissue. occurs in the absence of testicular determining factors (i.e.,
Y chromosome, TDF, testosterone, DHI and AMF).
Treatment 1. In the absence of a Y chromosome and TDF, the
Treatment for pheochromocyroma is surgical excision. undifferentiated gonad develops as an ovary.

r Careful attention must be paid to perioperative control of In the absence of AMF, mullerian ducts develop into the
hypertension and other symptoms. Preoperative medication fallopian tubes, uterus, and upper one-rhird ofthe vagina.
consists ofan cr-blocker, either the long-acting phenoxyben- In the absence of
J, testosterone and DHT, the wolffian
zamine or prazosin (a shorter-acting u-blocker), sometimes ducts degenerate and the external genitalia differentiate as
combined with a B-blocker. the clitoris, labia majora and minora, and separate urerhral
. Hypertension due to reduced vascular volume after the and vaginal openings.
removal of tumor is managed with aggressive fluid replace-
ment.
o Glucocorticoids must be given if bilateral adrenaiectomy
is done.

Abnormal sexual differentiation results in a newborn who

appears sexually ambiguous (i.e., discrepancy berween external
genitalia and internal gonads).
The human embryonic gonad is undifferentiated before 45-50
days of gestation. The internal sexual ducts consist of both
male (wolffian) and female (mullerian) strucrures ar rhis early MALE PSEU DOH ERMAPH RODITISM
state. Thereafter, sexual differentiation proceeds along distinctly
It refers to infants who are 45,YY males (with testes) but who
different paths dictated by the genetic and hormonal factors
appear to have signs of incomplete masculinization, including
in the male and female fetus. Fetus has innate tendency to
hypospadias, a small phallus, and a poorly developed scrotum
develop into female.
with or without descended tesres. Male pseudohermaphroditism
can be caused by a variety of endocrine disorders involving
testosterone synthesis, metabolism, or action at the cellular level.
MAIE DIFFERENTIATION
1. Defects in testosterone synthesis and metabolism
The male genorype is 46, XY are very rare and may be caused by one of five enzyme
1. Determinants on the Y chromosome (SRY gene, the sex deficiencies inherited as aurosomal recessive traits. The
determining region on shorr arm ofY) direct the synthesis first three result in defects in cortisol synthesis as well,
of testis determining factor (TDF). In the presence of and therefore are classified as forms of congenital adrenal
TDF, the undifferentiated gonad differentiates as resris; hyperplasia.
in the absence of TDA, the undifferentiated gonad dif-
ferentiates as an ovary. Cholesterol desmolase deficiency results in severe salt
2. The fetal testes secrete testosterone from Leydig cells under wasting. Profound deficiency in mineralocorticoid,
the direction of human chorionic gonadotropin (HCG) glucocorticoid, and androgen results in death in
and fetal pituitary gonadotropin. infancy in spite of adrenal steroid replacement.
\
a. High local concentrations of fetal testosterone stabi- b. 3B-Hydroxysteroid dehydrogenase deficiency: 1
lize wolffian structures, which develop into the vas Boys are incompletely virilized because of deficient I
deferens, epididymis, and seminal vesicles. testosterone synthesis. Girls may be mildly virilized. \
1
I
,

Diagnosis rests on measurement of elevated serum
DHEA and 1 7-hydroxypregnenolone.
c. 17-Hydroxylase deficiency: Because this defect
results in increased deoxycorticosterone, a weak
mineralocorticoid, hypokalemia, and hypertension
may be present. Boys have ambiguous genitalia
because of the inabiliry to produce sex steroids. Girls
have normal sexual differentiation, but secondary sex
characteristics fail to develop at puberry.
l siblings would be expected to be female carriers
I d. l7 Oxidoreductase deficiency prevents conversion
) of androstenedione'to t.stosterone. Diagnosis may
] infertile or cryptorchid relatives is suggestive.
be made in infancy by finding an increased ratio of
l
: androstenedione to testosterone after stimulation
with HCG.
l
F
e. 17, 20-Desmolase deficiency is an extremely rare cause
of male pseudohermaphroditism that results from the
I inability to convert progestogens to androgens.
>
2. Defects in androgen actioni
t degree of labioscrotal fusion, formation of a urogenital sinus,
a. 5ct-Reductase deficiency impairs conversion of testos-
terone to DHT.
(i) Boys are born with ambiguous genitalia because
l DHT is necessary for masculinization of the
! male external genitalia. Some of
these infants
are assigned a female sex because of the minimal
virilization apparent at birth.
(ii) At puberry testosterone-dependent pubertal
changes take place, such as clitoral enlargement
and descent of inguinal testes into the regulated
labioscrotal folds. Muscle mass increases, and the
voice deepens.
(iii) The diagnosis of a 5cr-reductase deficiency may be
made in childhood by finding an increased ratio
of testosterone to DHT after HCG stimulation.
b. Androgen resistance syndromes (testicular feminiza-
tion syndrome): In the normal male newborn, tes-
tostetone levels are significantly elevated for the first
several months. Inappropriately elevated neonatal
restosrerone with high LH might indicate androgen
resistance due to a receptor defect for failure of
normal negative-feedback suppression. The pattern
of normal to high testosterone with hlgh LH is
well-documented in postpubertal individuals with
androgen resistance:
(i) In complete androgen resistance, an XY male
infant with testes appears unambiguously female
because of complete resistance to androgen action
at the cellular level. The first clue to this disorder
may be the discovery of testes in inguinal hernia
sacs in early childhood. Some children may Present
as female adolescents with primary amenorrhea'
Because the undescended testes produce AMF
in utero, the vagina is a shallorv, blind-ending
pouch. If the testes are not removed before the
ENDOCRINE AND METABOLIC DISEASES
time of puberty, normal female breasts develop
from the increased conversion of testosterone to
estrogen by the testes.
(ii) In partial androgen resistance, the afFected XY
individual has ambiguous genitalia. The diagnosis
of partial androgen resistance is difficult to make
in the newborn with an XY karyotype. Because
it is inherited as an Xlinked recessive trait, the
infantt mother must be a carcier and half of her
or affected males. Therefore, a family history of
FEMALE PSEU DOH ERMAPH RODITISM
Female pseudohermaphroditism refers to infants who are 46,W
females (with ovaries) but who appear masculinized at birth.
Exposure of the female infant to increased androgen during
the critical period of 8-12 weeks gestation causes a variable
and clitoral enlargement. Exposure after the 12'h week cannot
cause labioscrotal fusion, but it can induce clitoral enlargement.
Some infants appear to be cryptorchid boys at birth.
e Congenital adrenal hyperplasia: Defects that cause female
pseudohermaphroditism are 2l-hydroxylase deficiency,
I l-hydroxylase deficiency, and 3B-hydroxysteroid dehy-
drogenase deficiency.
o Maternal androgen or progestin exposure: \7ith increas-
ing awareness of the effects of medication and other drugs
on the developing fetus, exogenous ingestion ofandrogenic
substances is a rare cause of female pseudohermaphroditism.
Occasionally, virilizing tumor or disease during pregnancy
a
in the mother may cause this syndrome. A detailed history
of the pregnancy, including drugs taken and medical illness,
should be obtained.
Abnormol Gonodol Ditferenliolion
True Hermaphroditism
Tiue hermaphroditism occurs when there is both ovarian and
testicular tissue present either in the same or opposite gonads.
In approximately 80o/o of cases, the karyotype is 46, )C(, and
in the remainder it is 46, XY or mosaicism. The exact etiol-
ogy is unknown.
0inical Featutes Usually there is significant masculinization, and
consequendy most true hermaphrodites are reared as boys. Gyneco-
mastia and ryclic hematuria from uterine bleeding may occur.
lnvestigations Tiue hermaphroditism may be strongly sus-
pected in an infant with ambiguous genitalia, an )O( karyotype,
and normal serum l7-hydroxyprogesterone levels, ruling out
2l-hydroxylase deficiency. The final diagnosis rests with surgi-
cal expioration and demonstration of gonads containing both
ovarian and resricular tissue.
ESSENCE OF PEDIATRICS
Mixed Gonadal Dysgenesis
Mixed gonadal dysgenesis involves a karyorype of 45X|46XY.
(linical Features There is a spectrum ofappearance ofthe exter-
nal genitalia from completely male to completely female.
o The gonads may appear as streak ovaries to dysgenetic testes
and are often asymmetric. The more testicular tissue that
is present, the greater is the likelihood of wolffian duct
development on the side of that gonad.
o stimulation identifies rare forms of congenital adrenal
Because of the 45X cell line, some somatic features ofTi.rrner
syndrome may be present'(e.g., short stature, webbed neck,
congenital heart disease).
lnvestigation Diagnosis is made by karyotyping.
0ther Defects in External Genital Development
Other defects in external genital development include hypo-
spadias and microphallus.
o Hypospadias of varying degrees of severiry may occur alone or
with other birth defects, especially of the genitourinary sysrem.
o Microphallus describes boys with abnormally small but well-
differentiated genitalia. Standards are available for assessing
stretched penile length from infancy through adulthood.
Male genital growth depends on fetal pituitary gonadotropin
stimulation of the fetal testis.
Etiology Microphallus mayindicatepostnatal hypogonadotro-
pic hypogonadism, as in Kallmann syndrome, or may present
as part of congenital hypopituitarism.
Treatment Therapywith testosterone (25 mgevery 3 weeks for
3 months) may demonstrate response and has a positive cos-
metic effect without significantly advancing skeletal maturation.
MANAGEMENT OF THE CHILD WITH
AMBIGUOUS GENITALIA
Diognostic Evqluotion
Complete diagnostic evaluation should be undertaken as soon
as possible after the birth of a child with ambiguous genitalia.
Parents should be encouraged to delay naming and announcing
the child's sex until diagnostic workup is complete. A careful
family and pregnancy history and physical examination are the
basis for further testing.
1. Physical examination: The size of the phallus, position
ofthe urethra, palpable gonads (usually testes), and other
dysmorphic or asymmetric features should be noted.
2. Laboratory studies include chromosome analysis and
evaluation of electrolytes, testosterone, LH, FSH, and
l7-hydroxyprogesterone levels. Radiographic dye study
of the urogenital sinus is often helpful to delineate the
presence of a vagina and cervix, and occasionally fal-
lopian tubes may be seen. Pelvic ultrasonography may
demonstrate the presence of ovaries and a uterus.
a) If the karyorype is 46,)0( and the 17-hydroxyproges-
terone level is elevated, the most likely diagnosis is
2l-hydroxy'ase deficiency. If the 17-hydrox)?roges-
terone level is normal, true hermaphroditism is likely.
Measurement of l1-deoxycortisol and DHEA levels
rules out the remote possibility of 11-hydroxylase defi-
cienry or 3B-hydroxysteroid dehydrogenase deficiency.
b) If the karyotype is 46,XY, measurement of gonadal
and adrenal steroids before and afterACTH and HCG
hyperplasia and defects in testosterone synrhesis and
metabolism.
(i) Infants must be monitored closely for evidence of
salt wasting and glucocorticoid deficiency while
awaiting diagnostic test results.
(ii) Diagnosis of partial androgen resistance depends
on a family history compatible with Xlinked
recessive inheritance. High neonatal testosterone
levels with elevated LH levels are suggestive of
androgen resistance.
Gender Assignmenl
It is important to arrive at the most specific diagnosis possible
for a variety of therapeutic and management considerations.
1. Even markedly masculinized girls with 2l-hydrorylase
deficiency should be reared as female, because they have
reproductive potential with adequate medical management
of their disease and cosmetic repair of the external genitalia.
2. For 46,XY boys with ambiguous genitalia, gender assign-
ment should be based on consideration of the possibiliry of
normal adult male sexual function. This generally depends
on the size of the phallus and the surgeon's estimation of
surgical correctability of the hypospadias.
a) Dysgenetic testes and ovotestis should be
removed because of their potendal for malignant
transformation.
b) Gonads that do not agree with gender assignment
should be removed to avoid the possibiliry of
undesirable hormonal influences at puberry.
Treolmenl
o Appropriate hormonal replacement at the usual age of
puberry should be provided.
o Genetic and sexual counseling for families and for children
is an important aspect of medical care.
o In general, full but sensitive disclosure of all test results
leads to successful psychosexual adjustment in adulthood.
t
Normal pubertal development occurs as the period of transi- \
tion from sexual immaturity to the sexually mature adult state. I
During this time, secondary sex characteristics are acquired and I
t
!
i
 ENDOCRINE AND METABOLIC DISEASES

reproductive capaciry is attained as a result of the secretion of d. Anorexia nervosa may cause delayed puberty or,
gonadal steroids under the direction of gonadotropin-releasing in older adolescents, secondary amenorrhea due to
hormone (GnRH) and pituitary gonadotropins (i.e., FSH and gonadotropin defi ciency.
LH). Deficiencies or defects in the functioning of these secre-
Prader-til7illi syndrome is characterized by short stature, obesity,
tions may result in abnormal pubertal development.
mental retardation, and hypogonadotropic hypogonadism.

Mole Deloyed Puberly

DETAYED PUBERTY
The earliest sign of puberty in boys is testicular enlargement.
Femole Deloyed Puberty Absence of any evidence of puberty by 14 years of age is
considered delayed.
By definition, the absence of secondary sex characteristics at
13 years of age is considered delayed. Normal secondary sex 1. Constitutional delay of puberty is the most common
characteristics but absence ofmenarche by l6 years ofage also cause of delayed puberry in boys. Frequently these boys
is considered delayed (primary amenorrhea). are short (at or below the 5'h percentile) but growing at
a low to normal prepubertal growth velocity. The bone
1. Constitutional delay of puberry is less commonly diag-
age is significantly delayed. The physical examination is
nosed in girls than in boys.
negative except for sexual immaturity. There usually is a
a. Etiology: Constitutional delay of puberry is a designa-
family history of delayed pubertal development.
tion reserved for otherwise healthy children. Delayed
puberty may also be secondary to a variery of endo-
2. Primary testicular failure: In boys with primary testicular
failure, gonadotropin levels are elevated in the castrate
crine and systemic diseases, such as hypothyroidism,
range by the usual age of puberty because of the lack of
rheumatoid arthritis, inflammatory bowel disease,
testosterone feedback on the pituitary gland.
chronic renal failure.
a. Congenital bilateral anorchia ("Vanishing testes" syn-
b. Clinical Features: Girls with constitutional delay of
drome): Boys with this syndrome have normal male
puberry frequently are short but growing at normal
sexual differentiation with apparent cryptorchidism.
prepubertal growth velocities. Bone age is often signifi-
However, no testis is found on surgical exploration,
cantly delayed; however, there is no evidence ofother
and there is no testosterone response to HCG stimula-
endocrine or systemic disease. There is frequently a
tion. Because there are normal male external genitaiia
strong history of delayed puberry or menarche in
and there are no mullerian remnants internally, it is
adult family members. Late but otherwise normal
presumed that testes must have been present in early
puberty occurs.
fetal life and subsequently "vanished."
2. Primary ovarian failure: Girls with primary ovarian b. Chemotherapy, irradiation, surgical excision, trauma,
failure have castrate levels ofgonadotropins by the usual and infection in the prepubertal boy may result in
age ofpuberty (10-12 years) because oflack offeedback testicular failure.
of gonadal steroids on the pituitary gland. c. Primary testicular failure also may be associated with
a. Tirrner syndrome is the most common cause of the normal onset of puberty. in Klinefelter syndrome,
primary ovarian failure. a common cause of testicular failure, puberty begins
b. Prepubertal surgical removal or irradiation of the at the usual age and secondary sex characteristics are
ovaries for treatment of cancer also may cause primary acquired. However, these boys have small, firm testes
ovarian failure. and ofren have gynecomastia.
c. Autoimmune ovarian failure may occur in association 3. Hn>ogonadotropic hypogonadism: As in girls, isolated
,, with other autoimmune diseases, adrenal insufficiency, hlpogonadotropic hlpogonadism in boys may occur alone
thyroiditis, hypoparathyroidism, and IDDM. or as pa-rt of arecognizable disease (e.g., Kallmann syndrome,
3. Hypogonadotropic hypogonadism may be difficult to hypopituitarism, CNS tumors, Prader-\Willi syndrome).
distinguish from delayed puberty because, in each situ-
ation, gonadotropin levels are low and the response to Diognosis
GnRH stimulation is also minimal. This disorder often A careful history and physical examination should be taken,
a
is part of other recognizable syndromes. including height and weight, Tanner staging of pubertal
7
l. a. Kallmann syndrome involves anosmia with hypogo- development, and presence of dysmorphic features of other
f- nadotropic hypogonadism. endocrine or systemic disease.
r b. Hlpopituitarism may include gonadotropin deficiency Laboratory studies include an evaluation of skeletal matura-
t'
?
as one of several deficiency pituitary hormones. tion (bone age) as well as FSH, LH, prolactin, T' TSH, and
t c. Hrpothalamic and pituitary tumors such as adenoma, testosterone or estrogen levels. To rule out systemic disease,
) microadenoma. complete blood count, electrolytes, and BUN levels may be
I
I

ESSENCE OF PEDIATRICS
helpful. Depending on rhe clinical siruarion, CT or MRI
scan of the head and testing of other pituitary hormones
may be indicated.
Treotmenl
o fleatment with appropriate sex steroid replacement for ado-
lescents with a permanent cause of delayed puberry (either
primary gonadal failure or hypogonadotropic hypogonadism)
should be instituted at the usual age ofpuberty. Because sex
steroids promote epiphyseal fusion while stimulating linear
growth, this effect must be taken into consideration when
treating children with short stature, especially that caused
by GH deficiency.
o For adolescents with constitutional delay of puberry smaller
doses of the appropriate sex steroid may be used for a
short course of treatment (3-6 months). This will initiate
some development of secondary sex characteristics and be
psychologically beneficial while not promoting premature
epiphyseal fusion and loss of adult height.
PRECOCIOUS PUBERTY
Sexual development is considered to be precocious if there are
any secondary sex characteristics present in girls before 7 years
of age and in boys before 9 years of age.
Menarche before 10 years is also considered precocious.
Onset of puberry is marked by appearance of breastbud and
enlargement of areola (B2) in girls and testicular enlargement
(volume >4 ml) (G2) in boys.
Clqssificqlion
r Cental or true precocious puberty (CPP), gonadotropin
dependent: Is due to premature activation of hypothalamo-
pituitary-gonadal axis and is therefore isosexual. It is more
common in girls than in boys. In girls, there is rarely
underlying CNS disease, but in boys significant numbers
have CNS pathology.
o Peripheral or pseudoprecocious puberty (PPP): Is due to
production of sex steroids independent of hypothalamo-
pituitary axis and may be isosexual or heterosexual.
o Incomplete forms or pubertal variants:
o Premature thelarche: Isolated breast development
o Premature adrenarche: Early appearance of axillary andl
or pubic hair
o Premature menarche: Early initiation of menstruation
Etiology
e Central isosexual precocious puberty: 5 times more
common than peripheral
o Idiopathic in girls (90olo)
o Neurogenic
Intracranial tumors: Hypothalamic hamartomas,
- gliomas, pineal tumors, astrocytoma
3 t
!
Post infectious: Meningitis, encephalitis, tuberculous
- Congenital malformation: Hydrocephalus, micro-
- cephaly, myelomeningocele
Thaumatic: Perinatal, accidental
- Neurofibromatosis, tuberous sclerosis
- Hypothyroidism
-
r Peripheral precocious puberty:
o Isosexual
Girls: Ouaryt-granulosa cell tumor, theca cell tumor,
- teratoma, autonomous functional cyst; adrenal-
feminizing adenoma; exogenous estrogens.
Boys: Testes-Leydrg cell tumor; adrenal-congen-
- ital adrenal hyperplasia, adenoma, carcinoma; exog-
enous androgens.
o Heterosexual
Feminization in boys-adrenal cortex tumo! estrogens
- Virilization in girls-congenital adrenal hyperplasia;
- virilizing ovarian and adrenal neoplasm, administra-
tion of androgens.
History
Sex: CPP in girls mostly constitutional. CPP in boys almost
always with intracranial pathology.
Age of onser: Idiopathic CPP between 5 andT years. Hypo-
thalamic hamartoma in first 3-4 vears of life.
o Pubertal progression: Very slow in idiopathic CPP in girls,
very rapid in androgen producing tumors, ovarian cysts,
hypothalamic hamartomas.
o Accelerated growth: Absent in pubertal variants and in
hypothyroidism.
o Irregular vaginal bleeding: In functioning ovarian rumor.
o H/O past CNS infection including TB. Headache, visual
disturbances, personaliry changes, developmental delay, and
seizures suggest neurologic disorder.
o H/O drug exposure.
o Symptoms and signs of hypothyroidism.
o Family history: Positive in constitutional precocious puberry.
H/O precocious puberty in boys and ambiguous genitalia
in girls of same family suggest CAH.
Physicol Exqminolion
o Look for androgen gf[e615-26ns, hirsutism, increased muscle
mass, clitoromegaly. Hyperrension suggesrs adrenal cause.
o Pubertal Tanner's sraging.
o Abdominal and rectal examination for uterine size, ovarian
mass, adrenal tumors.
o Testicular palpation
,r Testes volume > 4 ml: Tiue precocity-CPP
.r Symmetrically enlarged testes: CPP
c Unilateral enlarged testes: Testicular rumor
r Inappropriate small testes: PPP (probably adrenal cause) \
,> Scrotal mass t
o fum61-lesticular or adrenal \
I
l
 ENDOCRINE AND METABOLIC DISEASES

o Skin lesions: Neurofibromatosis, tuberous sclerosis, McCune o Surgery not indicated in hypothalamic hamartomas.
Albright syndrome (MAS)
a Radiotherapy: Germ cell tumor, pineal tumor.
r Irregular skin pigmentation, bone pain, pathological frac-
o Psychological support.
tufe
o Neurological examination including fundoscopy, perim-
etry
o Signs of hypothyroidism

Calcium and phosphorus homeostasis is maintained by having

lnvesligolions adequate nutritional intake of calcium, phosphorus, and vitamin
o Bone age: Advanced in all except hypothyroidism D and a normally mineralized skeleton, the major reservoir
o X-ray skull: For sella, caldification, signs of increased intrac- of these minerals. The serum calcium and phosphorus are
ranial pressure finely regulated by the action of parathyroid hormone (PTH),
a CT/MN: To determine etiology of CPP in boys which acts on rhe bone and kidneys ro raise the serum levels
o USG of pelvis and abdomen: To evaluate uterus, ovaries, of calcium and lower the levels of phosphorus.
adrenals
o Skeletai survey in suspected MAS
PRIMARY HYPERPARATHYROIDISM
o Testicular biopsy: If tumor is suspected
a Vaginal cytology: To see estrogen effect It is rare in childhood. Increased PTH levels cause increased
a Hormonal evaluation: mobilization of calcium and phosphorus from bone. The effect of
, T,, T.. TSH: Abnormal in hypothyroidism increased PTH on the kidneys is decreased tubular reabsorption
r FSH, LH: tin CPP; J in PPP of phosphorus. Thus, the serum levels of calcium are elevated
,> GnRH stimulation test: LH response higher than FSH (hypercalcemia) and the phosphorus levels are low.
in CPP
Pubertal LH response: LH >10 IlllL30 minutes Clinicol Feoiures
after GnRH indicate CPP
o Serum estradiol: ttt 1.".1, i.e., >100 ng/ml in ovarian o Symptoms are related to hypercalcemia and include nausea,
vomiting, constipation, lethargy, and confusion.
tumor, cyst
o Serum testosterone: Very high in primary gonadotropin
o Hypertension, hematuria, and renal colic secondary to
kidney stones are common.
excess
o Serum DHEAS: fin virilizing adrenal problem in pre-
o Osseous changes may produce pain in the back or extremi-
ties.
mature adrenarche
o 17-OH progesterone, 11-deoxycortisol: t in CAH
o HCG: Increased in HCG secreting tumor Invesligolions
o Urinary 17-ketosteroid and pregnanetriol: 'l i" CRH r Laboratory studies show elevated serum levels of calcium
(total and ionized), low levels ofphosphorus, increased alka-
Treolment line phosphatase, and low tubular reabsorption ofphospho-
rus (<800/o). PTH levels are elevated relative to the elevated
Treatment of CPP
serum calcium. PTH secretion is autonomous.
Should be considered when: o Radiographs of bone show subperiosteal bone resorption
that is especially evident in the clavicles.
r evidence that adult height may be compromised,
o menarche before 6 years,
o puberry psychologically disturbing, and Treqlmenl
o rapid pubertal development. Therapy involves excision of the tumor if an adenoma is found
and subtotal parathyroidectomy for hyperplasia.
Drugs: GnRH agonist-leuproiide acetate (depot) 0.25-0.3
mg/kg IM 4 weekly (minimum 7.5 mg), Or intranasal nafarelin
(synarel) 800 pg twice daily. SECON DARY HYPERPARATHYROIDISM
Monitoring: Every 6 week until suppression, then every 3
Diseases that cause hypocalcemia stimulare PTH to be released.
months.
The elevated PTH then restores the serum calcium to normal,
but not elevated levels, but at the expense of having low
Treatment of PPP phosphorus levels.
o Ketoconazole 600 mg/d in 3 divided doses. Condidons that are associated with secondary hyperpara-
o Surgery: Tumor of ovary, adrenal gland, and testes. thyroidism are chronic renai disease, liver disease, and lack of
 ESSENCE OF PEDIATRICS

vitamin D. In each of these situations, the initiating event is

related to lack of intake, absorption, or metabolism of vitamin
D, which leads to the hypocalcemic stimulus for PTH secretion.
Diagnosis and teatment: The radiographic manifestation is In a number of conditions, described below, plasma level ofADH
of rickets, and treatment is provision of adequate vitamin D. (vasopressin) is inappropriately high. SIADH causes expansion
of vascular volume and hyponatremia; clinical features are due
HYPOPARATHYROIDISM to hyponatremia and are those of water intoxication.
EtiologS,':
ldiopolhic Hypoporolhyroidism
o Pulmonary: Pneumonia, bronchiolitis
Itmay present in the neonatal period or at any time during o CNS diseases: Meningitis, encephalitis, brain abscess, head
childhood. The etiology may be autoimmune.
trauma, Guillain-Barre syndrome
Clinical features: Symptoms are caused by low serum levels of o Drugs: Vincristine, cyclophosphamide, chlorpropamide
calcium and include seizures, tetany, numbness of the face and
Clinical features: Lethargy, confusion, convulsion (when serum
extremities, and carpopedal spasm. Associated diseases may be
sodium falls below 110 mEq/L).
thyroiditis, diabetes, adrenal insufficiency, and mucocutaneous
candidiasis. Investigations: Serum sodium and chloride levels are low.
Urinary osmolaliry is greater.
Investigation: At the time when serum calcium levels are
low and phosphorus levels are high, the PTH levels are Diagnostic criteria:
inappropriately low, indicating lack of PTH response to the
o Absence of renal, adrenal, or thyroid insufficiency; congestive
hypocalcemia signal.
heart failure; nephritic syndrome; or cirrhosis
o Diuretic ingestion
Pseudo hypopqrqlhyroidism o Dehydration
Itpresents as symptomatic hypocalcemia, but PTH levels are r Urine osmolality >100
very high, indicating PTH unresponsiveness due to a receptor o Serum osmolaliry <280 mOsm/kg
or post-receptor defect. Patients with pseudohypoparathyroid- o Serum sodium <135 mmol/L
ism may have distinctive skeletal and facial characteristics, r Urine sodium >25
inciuding short stature, round facies, a short, thick neck, and
short metacarpals. Mental retardation is common. Treatment:
o 20o/o fluid restriction.
Treqlmenl o Symptomatic hyponatremia is treated with normal saline
or sodium chloride solutions (150 ml/m'? over t hour)
1. Vitamin D is the treatment for idiopathic hypoparathy- 3%o
+ Frusemide (1 mg/kg).
roidism and pseudohypoparathyroidism.
o Successful treatment of underlying disorder (meningitis,
a. Because low levels of PTH inhibit 1,25 hydroxyla' pneumonia) is followed by spontaneous remission.
tion of vitamin D in the kidneys, PTH deficiency is
associated with a deficit of 1,25-dihydroxyvitamin D.
(calcitriol), the most active metabolite of vitamin D.
fleatment w ith I,25 dihydroxyvitamin D, overcomes
the deficit and also stimulates increased calcium
absorption in the intestine.
1. Behrman RL, Kliegman RM, Jenson HB. Nelson Textbook of
Pediatrics 16'h ed. India: \(B Saunders Co., 2000.
b. Other forms ofvitamin D, such as 25-hydroq,vitamin 2. ParthasarathyA (ed). IAP Textbook of Paediatrics 4'h ed. New Delhi:
D. (calcidiol) and vitamin Dr, (cholecalciferol), are Jaypee Brothers,2009.
'!il'iikins,
also efFective but are required in larger doses. Because 3. Dworkin PH (ed). Pediatrics 3'd ed. PA: 'sfilliams and
of the longer half-life of vitamin Do, however, toxiciry 1996.
(hypercalcemia) is more serious. 4. Khan MR, Ahmed T. Essence of Endotrinologlt 1" ed. Dbaka, 1997.
5. Gotlin R.\f' (ed). Current Pediatric Diagnosis and T\eatment l9'h
2. Oral calcium supplementation may speed the restoration of ed.., 1999.
normal calcium levels. These children should have serum 6. Hutchison JH, Cockburn F. Practical Paediatric Problems 6't' ed.
calcium and phosphorus levels monitored frequently to Singapore: PG Publishing Pte Ltd, 1985.
avoid hypercalcemia and potential nephrocalcinosis and 7. Hetzel BS, Pandav CS. 7he Conquest of lodine Dficiency Disorders
renal damage. Oxford University Press, 1994. \
I

E .\
t
a
 I
ENDOCRINE AND METABOLIC DISEASES

8. Hetzel BS. Iodine deficiency disorders (IDD) and their eradication. 13. Delange F. Neonatal thyroid screening as a monitoring tool
Lancet 7983:2:1126-9. for control of iodine deficiency. Acta Paediatr 1999;
9. \X'HO, UNICEF, ICCIDDD. Indicators for assessing iodine defi- 5ryp1432:21-4.
ciency disorders and their control through salt iodization, !fHO/ 14. Ghai OP Essential Paediatrics 7'\ ed. New Delhi: CBS Pubiishers,
NUT/94-6. Geneva: \fHO, 1994. 2009.
l0 Yusuf HK, Quazi S, Kahn MR, et al. Iodine deficiency disorders 15. Dworkin PH. NMS: Pediatrics 4'h ed. Philadelphia: Lippincott
in Bangladesh. Indian J Pediatr 1996;63(1):105-10. \X/illiams and Vilkins, 2000.
ll Delange F. The role of iodine in brain development. Proc Nutr Soc 16. Haslett C, et al. (ed). Dauidson\ Principles and Practice of Mtdicine
2000;59( 1):75-80. 18'r' ed. London: Churchill Livingstone, 1999.
12. Hetzel BS. Iodine and neuropsychological developmenr. J Nutr
2000: 1 30(2S Suppl):49J-5.

J
 !

CHAPTER L3
Neurology

Chopler Contents
Spinal cord compression ........... ..,...,,........,..,......................213

Cuillain-Barre syndr0me............. .................... 21 3

Neurometabolic dis0rders.................

ctAsstFtcATroN

A seizure (convulsion) is defined as a paroxysmal involuntary lnlernqlionol Clqssificqlion of Epileplic

disturbance of brain function that may be manifested as an Seizures: ltAE l98l
impairment or loss of consciousness, abnormal motor activiry 1. Partial seizures
behavioral abnormalities, sensory disturbance, or autonomic
A. Simple pafiial seizure (consciousness not impaired)
dysfunction. Some of the seizures are characterized by abnormal
with:
movements without loss or impairment of consciousness.
Epilepsy is defined as recurrent (two or more) unprovoked a. Motor signs (focal motor -+ Jacksonian march,
seizures occurring 24 hours apart in a child > I month old. versive, postural, phonatory)
Ail epilepsies are seizures, but all seizures arenot epilepsies. b. Sensory: General and special sense, like delusion,
Seizures are a common neurologic disorder in the pediatric age hallucination.
group and occur in 3-5olo of children. Epilepsy occurs in 0.5-1.00/o c. Autonomic symptoms and signs: flushing of face,
of the population and begins in childhood in 600/o of cases. piloerection etc.
Refractory epilepsy is defined as when seizure control is d. Psychic symptoms (dysphasia, deja vu, dreamy
not obtained with consecutive trials of two well-tolerated anti- state, anger, fear)
epileptic drugs for adequate time (6 months). B. Complex partial seizure (consciousness impaired)
Table 13.1 lists features differentiating epilepsy from a. Simple partial, then impairment of consciousness
syncope, and Thbie 13.2 differentiates epileptic seizures from b. Impairment of consciousness from onset
pseudoseizure.
C. Partial seizures -) secondarily generalized
a. Simple partial -+ generalized seizure
ETIOTOGY
b. Complex partial -+ generalized seizure
o Idiopathic: Genetic in origin, intraurerine infection c. Simple partial -) complex partial -+ generalized
(TORCH, HIV) seizure.
o Abnormal brain development: Neuronal migration defect, 2. Generalized seizures
p*rinatal insults A. Absence: rypicaliarypical
o CNS infections B. Myoclonic
o Brain injury C. Atonic I
r Brain tumor D. Tonic \
e Neurometabolic and neurodegenerative diseases E. Clonic I
o Chromosomal disorders: Fragile X, Thisomies F. Tonic clonic II
t
tt
 NEUROLOGY

3. Unclassified 3. Undetermined:
A. Neonatal seizure (e.g., subtle seizure) A. Neonatal seizures (subtle seizure)
B. Infantile spasm B. Severe myoclonic epilepsy of infancy (Dravet syn-
drome)
lnlernolionol Clossificotion of Epilepsies C. Landau-Kleffner syndrome
ond Epileptic Syndrome (lCE): ILAE 1989 4. Special syndromes:
Four broad groups are (l) localization related epilepsies and A. Febrile convulsions
syndromes, (ii) generalized epilepsies and syndromes, (iii) B. Hemiplegia-hemiatrophy epilepsy (HHE)
undetermined-whether focal or generalized, and (iu) special C. Isolated status epilepticus,
syndromes. D. Seizures accompanying acute toxic/metabolic events.
,
1. Localization related epilepsy: PartialSeizure
A. Idiopathic: Partial seizures are those in which, the first clinical and
Benign childhood epilepsy with centrotemporal electroencephalographic changes indicate initial activation of
- spikes (BECTS)
a system of neurons limited to part of one cerebral hemisphere.
Childhood epilepsy with occipital paroxysm When consciousness is not impaired, the seizure is classified as a
- Primary reading epilepsy
-
B. Symptomatic:
Epilepsia partialis continua Table 13.2: Difference between Epileptic Seizures and
- Frontal/temporal/parietal/occipital Pseudoseizure
-
C. Cryptogenic: Probably symptomatic
Often an emotional
2. Generalized epilepsies and syndromes: Precipitant May be precipitated by
fever, emotron, irregular precipitant.
A. Idiopathic: rnedication.
Benign neonatal familial convulsion (BNFC) Circnmstances
- Benign neonatal convulsion (5'h day fit) in sleep Common Rare
- Benign myoclonic epilepsy of infancy when alone Common Occurs, especially
- Childhood absence when an onlooker is
present (there occurs a
- Juvenile absence subconscious desire for
- Juvenile myoclonic epilepsy secondary gain).
- Generalized tonic-cionic seizure on awakening Onset Usually abrupt. May May be gradual with
-
B. Symptomatic: have short aura. increasing emotional
symptoms.
Early myoclonic encephalopathy
- Early infantile epileptic encephalopathy Cry at onset Common U nusual
-
C. Cryptogenic or symptomatic: Motor Stereotyped, usually Variable, often tonic
phenomenon both tonic and clonic or clonic only; clonic
\(/651 syndrome
- lsnnoy*Gastautsyndrome
phase. components vary in
amplitude and frequency
- Myoclonic-astatic epilepsy (Doose syndrome) during the attack,
- Myoclonic absence epilepsy sometimes bizarre, wi ld
- movements occur.
lnjury with Common Rare
Table 13.1: Difference between Epilepsy and Syncope tongue biting
I nconti nence Common U nusual

Ppt. factor Rare Common Consciousness Usually totally Iost in Variable, often possible
convulsive seizures to communicate during
Occurrence Awake, sleep Awake
an attack.
Onset Abrupt Cradual
Restraint No ettect May resist, sometimes
Duration 60-90 sec 1 0-1 5 sec terminates an attack.
Jerking limbs Yes Occasional Duration of Usually short (>1-5 min) May be prolonged
Facial color Flushed Pale convu lsion

Perspiralion Hot, sweaty Cold, calmy Postictal phase Vomrting, headache, Confusion unusual.
confusion cofflmon. Drowsiness or sleeP
Postictal recovery Slow Rapicl
Drowsiness, sleep, and un trsual
Postictal confusion Common Uncommon automatism may occLlr.
EEC and prolactin Positive Negative Diagnostic Normal
ESSENCE OF PEDIATRICS
simple partial seizure; with impaired consciousness, the seizure
is classified as complex partial seizure (CPS). Impairment of
consciousness may be the first clinical sign of CPS, or simple
partial seizures may evolve into CPS.
Simple Partial Seizures These may present with (l) motor signs,
e.g., focal motor or Jacksonian march, versive (head turning
to one side, usually contraversive to the discharge), phona-
tory (vocalization or arrest of speech) or (ll) with somatosen-
sory (pins-and-needles or a feeling of numbness, tingling) or
special-sensory symptoms like simple hallucinations, visual
(light fashe$, auditory (buzzirtg), olfactory (unpleasant odors),
gustatory (pleasant or odious taste hallucinations), vertiginous
(sensations of falling in space, foating, as well as rotatory
vertigo) symptoms (iii) with autonomic symptoms or signs
(including vomiting, epigastric sensation, pallor, sweating,
flushing, piloerection and pupillary dilatation, and incontinence
may occur as simple partial seizures). A partial seizure may not
terminate, but instead progresses to a generalized motor seizure,
i.e., partial seizure evolving to secondary generalized seizure.
(omplex Paftial Seizute The central feature of CPS is impair-
ment of consciousness. During the period of impaired con-
sciousness, aberrations of behavior (automatisms) may occur.
So, three cardinal features of CPS are impaired consciousness,
aura, and automatism. Complex partial seizure may also be
associated with psychic symptoms like dysphasic, dysmnesic
(e.g., deja vu), cognitive (e.g., dreamy states, distortions of time
sense), affective (fear, anger, etc.), illusions (e.g., macropsia),
structured hallucinations (e.g., music, scenes).
Aura: The aura is the portion ofseizure that occurs before con-
sciousness is lost and for which memory is retained afterwards.
Auras are special sensory or psychic phenomenon that can be
described only by the patients. They occur in a variery of forms
depending on their origin from temporal (psychic symptoms),
frontal (automatic behavior), parieto-occipital area (sensory symp-
toms like tingling, numbness, burning or seeing lighrs, colors,
halos, tinnitus, hallucination) and thus have a iocalizing value.
Automatism: An automatism is a coordinated involuntary motor
activity occurring during the state ofclouding ofconsciousness
either in the course of or after an epileptic seizure, and usually
followed by amnesia for the event. The automatism may be
simply a continuation of an activity that was going on when
the seizure occurred, or conversely, a new activity developed
in association with the ictal impairment of consciousness.
Automatisms usually represent the release of automatic behavior
uncier the influence of clouding of consciousness. Five rypes of
phenomena may occur during an automatism:
o Alimentary automatism-chewing movements, swallowing,
increased salivation, borborygmi.
o Mimetic phenomena (automatism of mimicry expressing
emotional state, fear, anxiery anger(and joy during seizure),
like movements of face resulting in expressions of fear,
discomfort, vacant look, laughing, crying
r Gestural phenomena, like repetitive movemenrs of hands
and fingers (clapping, scratching), sexual gestures, fumbling
of the clothes, scratching, etc.
r Ambulatory phenomena, like wandering, running, riding a
bicycle even violating trafEc rules
o Verbal phenomena, like short phrases, explosives, or swearing
are commonly repeated in an auromatic fashion.
Generalized Seizure
Generalized seizures are those in which the first clinical changes
indicate initial involvement of both hemispheres.
Absence Seizures The hallmark of the absence attack is a sudden
onset, interruption of ongoing activities, blank stare, possibly
a brief upward rotation of the eyes. If the patient is speaking,
speech is slowed or interrupted; ifwalking, he stands transfixed;
if eating, the food will stop on his way to the mouth. Usually,
the patient will be unresponsive when spoken to. In some,
attacks are aborted when the patient is spoken to. The attack
lasts from a few seconds to half a minute. Absence seizure
may be induced through voluntary hyperventilation in >9070
cases. Normally, these are brought about by asking the patient
to over breathe for 3 minutes while standing, counting breaths
and with hands extended in front.
Simple (typical) absence (petit mal) seizures are more
prevalent in girls, uncommon before age of 5 year, never
associated with an aura; rarely persist longer than 30 seconds;
not associated with postictal state. Children may experience
countless seizures daily and do not lose body tone. Immediately
after the seizures, patients resume pre-seizure activity with no
indication of postictal impairment. Classical EEG finding is
regular, symmetrical, generalized spike and wave discharges
of 3 HZ. Four differenr rypes of absence seizures have been
described by ILAE: childhood absence, juvenile absence, juve-
nile myoclonic epilepsy, and myoclonic absence epilepsy.
Table 13.3 list features differentiating absence seizures from
complex partial seizures.
Myoclonic Epilepsies Myoclonic jerks (single or multiple) are
sudden, brief, shock-like contractions, which may be gener-
alized or confined to the face and trunk or to one or more
extremities or even to individual muscles or groups of muscles.
Myoclonic jerks may be rapidly repetitive or relatively isolated.
They may occur predominantly around the hours of going to
sleep or awakening from sleep. Common myoclonic epilepsies
in infancy and childhood include the following:
o Early myoclonic encephalopathy (Ohtahara Aicardi
syndrome)
o Benign myoclonic epilepsy of infancy
o Severe myoclonic epiiepsy of infancy (Dravet syndrome)
o Myoclonic astatic epilepsy
o Progressive myoclonic epilepsy
\
Atonic Seizure A
sudden diminution in muscle rone occurs, I
which may be fragmentary, leading to a head drop with slack- \
I
\
1
a
 NEUROLOGY

Table 13.3: Difference between Absence and Complex Partial theophylline and methylphenidate) are known to precipitate
Seizures GTCS.
EEG shows generalized burst of spikes and ftregular 4-6
Hz spike wave complex.
Frequency/day Multiple Rarely >1 to 2

<lOseconds >l minute West Syndrome

Duration
Aura Never Frequent May be sympromatic, cryptogenic, and idiopathic. Characterized
Clonic component Common, eye blink Rare by'
Postictal impairmenl Never Frequent
o Onset 3-12 months.
Hyperventilation Frequenlly induce attack Rarely
o Seizure characteristics include infantile spasm (flexor, exten-
't sot or mixed), singly or in clusters, more on awakening'
Photic stimulation Frequent Rarely
Seizures are sudden, brief, bilateral symmetrical contraction
lnterictal EEG Ceneralizedspike-wave Focalspike-wave of neck, trunk, and extremities.
o Hypsarrhythmia.
ening of the jaw, the dropping of a limb or a loss of all muscle o Mental retardation.
tone leading to a slumping to the ground. \X{hen these attacks o Developmental retardation.
are extremely brief, they are known as "drop attacks." So-called o May evolve into severe epilepsy syndromes, e.g., Lennox-
drop attacks may be seen in conditions other than epiiepsy, Gastaut syndrome (LGS).
such as brainstem ischemia and narcolepsy cataplery syndrome.
Often follows perinatal hypoxia, hypoglycemia, metabolic
Tonic Seizures A tonic seizure is a rigid, violent muscular errors, and cerebral malformations.
contraction, fixing the iimbs in some strained position. There
Types:
is usualiy deviation of the eyes and of the head toward one
side, and this may amount to l'otation involving the whole o Symptomatic (70-80o/o): HIE, other perinatal insults, cere-
body. Tonic axial seizures with extension of head, neck, and bral malformations, tuberous sclerosis, infections (pre- and
trunk may also occur. postnatal, neurometabolic disorders).
. Crptogenic (20-30oh): Without any obvious cause; cause
ClonicSeizures Generalized convulsive seizures occasionally
may be there; normal development till onset of spasm'
lack a tonic component and are characterized by repetitive o ldiopathic: <10%.
clonic jerks. As the frequency diminishes, the amplitude of
the jerks does not. lnvestigations EEG shows hypsarrhythmia or modified hyp-
sarrhythmia, burst suppression, CT scan shows lissencephaly,
Genetalized Tonic-Clonic Seizures (GT(S) Classic forrn has the
calcified tubers.
following phases:
Treatment Both ACTH and prednisolone can be used.
o Aura: A transitory premonitory symptom or aura heralds
ACTH acts by inhibiting corticotropin-releasing hormone,
the onset of a seizure. Aura may be sensory, visceral, motor'
which has possible spasmogenic influence. Dose is variable
or autonomic.
ranging from 0.02 to 100 units/sq meter. Tireatment schedule
o Tonic phase: During this phase, skeletal muscles undergo
(Fig.13.r):
into a sustained spasm. Spasm of the laryngeal muscles
forces the air in the lungs out through a partially closed r Steroids: Prednisolone 2 mglkgld for 4 weeks, then half
glottis resulting in a shrill cry. The muscular rigidity is dose for 4 weeks and one-fourth dose for another 4 weeks.
most marked in the antigravity muscles. The face appears o Increase ACTH till response to up to 60 unit/m2 daily, then
paie, pupils are dilated, and eyes are rolled either upward alternate day for 4-6 weeks, then weekly for 4 weeks and
or to the side. There is biting of tongue and frothing from then stop (monitor hypertension).
the mouth. Loss of sphincter control (bowel or bladder) is o Other anti-epileptic (AEDs): Sodium valproate, Benzodi-

o
common. This phase lasts for about 30 seconds.
Clonic phase: The clonic phase is characterized by rhvthmic
alternating contractions of muscle grouPs, which persist for
o
azepines, or newer AEDs.
Vigabatrin (50-100 mg/kg/d): It is the first choice, especially
in tuberous sclerosis.
H
a few minutes to even a few hours.
r Postictal phase Children initially are semicomatose and [ennox-Gastaut Syndrome
typically remain in deep sieep for 30 minutes to 2 hour. This
o 2-6 years of age. No family history of childhood epilepsy.
phase is associated with vomiting and an intense bifrontal
o Multiple seizure rypes: GTCS, tonic seizures, myoclonic
headache. Automatic behavior or violence may occur'
seizure, arypical absence or atonic seizure.
GTCS may last for 1-5 minutes. Fever associated with infection, o Often evolve from \fest syndrome.
or emotional stress, and various drugs (e.g.,
excessive fatigue o Mentai retardation >90o/o: status epilepticus common.
ESSENCE OF PEDIATRICS

Oral prednisolone (2 mg/kg/d in 2 doses) EVATUATION OF SEIZURE DISORDER

Or ACTH 40 uniVm, single dose lM for wk
Figure 13.2 depicts algorithmic approach to the evaluation of
seizure disorder.

INVESTIGATIONS
o Jhe minimum work-up for the first afebrile seizure ilrclude
fasting glucose, calcium, magnesium, and serum electrolyte
levels. CSF study is indicated if infection process is suspected
t*. or in subarachnoid hemorrhage.
Recurrence No recurrence
@ffid
tr
M ffi Important investigations of epilepsy include EEG (routine,
!
I !
.L video EEG): normal EEG does not exclude epilepsy.
o Skull x-ray: Not very informative.
Repeat ACTH No AED ffi

TrialVGB ffi
a Cranial ultrasound in infant while the ultrasonic window
K
@ (anterior fontanel) is patent.
CT/MRI: Should be reserved for patients in whom an
Continue ACTH (Dose may be increased)/Steroids and add AED. intracranial lesion is suspected.
ff
a Functional scans: Posirron emission ton-rography (PET),
.l
Fig. 3.1: Treatment schedule for West syndrome. single photon emission computcei rornography (SPECT)
can be done.

TREATMENT

Epilepsy Monogemenf
Step I : Confirm diagnosis of true seizures.
Non-epileptic seizure Step 2 : Establish Seizure rype and Epilepsy syndromes.
Step 3 :
ffi
lW
Evaluate need lor treatmenr initiation: Fir-st vs.
second seizure, widely apart seizure, benign vs.
malignant epileptic syndromes.
Provoked seizure Step 4 : Select AED based on seizure type and epilepsy
(symptomatic)
syndromes: considerations are spectrum, efficacy,
adverse reacrion, drug interaction, tolerabiliry
Febrile seizure, compliance, age, sex, weight, lifestyle, psychiatric
CNS infections, and other comorbidides.
Two or more
trauma,
seizures
Step 5: Start monotherapy with chosen first-line drug in low
metabolic
(24 hr aparl) dose, titrate f slowly ("Start low go slou" policy)
disturbances, till seizure control/ma-ximum pharmacological dose/
hypoxia,
intoxication
maximum tolerated dose appears. (t Slowly over
Epilepsy weeks, depending on narure ofAED and urgencv of
situation) (Fig. 13.3).
Fig" 13.2: Algorithm for evaluation of seizure disorder.
Step 6 : Ifseizure persists (Fig. 13.3)
o Switch ro another monotherapy (alternative first-
line or second-line) if first drug is ineffective or
a E,EG shows bilateral slow spike waves, slow background.
poorly tolerated
a Investigate for r-rnderlying causes (as in \7est syndrome).
o Add-on therapy (combination with different
Treatment Seizure response to AEDs is poor. Polytherapy is mechanism of action) with a second drug if the
required. Control of seizure with broad AED that is Sodium val- first drug is partly effective and well-tolerated.
proate, build rapidly to 50 mg/kg/d, add Benzodiazepines.
Newer AEDs such as lamotrigine and toperamate are now
Srst-line add-on drugs. ACTH/Prednisolone may be tried. Properties of an ldealAED
t
Ketogenic diet may be given. Corpus callosotomy in intractable High oral efficacy without seizure aggravation, good tolerabiliry t
epilepsies can be done. and no teratogeniciry no or minimal drug interaction, once
I
I
t
 NEUROLOGY

or twice daily dosing, range of formulation available, low cost Table 13.4: Choice of AED in different Epileptic Seizures
and high cost effectiveness.
Anti-epileptic drugs are divided into older and newer anti- Partial CBZ,PHT, SVA, PB OXC, LTC, TPM, other
epileptic drugs. Thble 13.4 suggests AEDs according to the new AEDs

rype of epileptic seizure, and Table 13.5 suggests AEDs for 2"CTCS SVA, ESM, CZP,CLB LTC, TPM, LEV
different epiieptic syndromes. CTCS SVA, CBZ, PHT, PB TPM. LTC, OXC, LEV

0lder AEDs Effectiveness against many seizure types and epi- Absence SVA. CZP, CLB LTC, TPM, LEV
leptic syndrome. The adverse efFects contribute to >40o/o of Myoclonic SVA LTC, TPM, LEV
initial Rx failure, variable pharmacokinetics, hepatic enzyme Atonic/Tonic SVA CZP, CLB, NTZ, LTC, TPM
induction lead to troublesome drug-drug interactions. Old anti- Mixed SVA
epileptic drugs are Phenobarbitone (PB), Phenytoin (PHT)'
CBZ-carbamazepine; PHT phenytoin; PB-phenobarbitone; OXC oxcarbazepine; LTC-
Carbamazepine (CBZ), Sodium valproate (SVP) or SVA, lamotrigine; TPM{opiramate; ESM-ethosuximide; CZP-clonazepam; CLB-clobazam;
Ethosuimide (ESM), and Benzodiazepines (BDZ)-Clobazam LEv-evetiracetam; CTCS-generalized tonic clonic seizure.

(CLB), Clonazepam (CZP), Nitrazepam (NZP).

NewerAEDs Effectiveness same, less adverse effects, tolerability

high, reduced drug-drug interaction, efficacy against refractory o Suspected toxicity.
epilepsy. Usually non-inducer of liver enzyme except OXC,
o After each AED change.
TPM and FBM can do so in high doses. Reserved for refrac- o In special clinical conditions like status epilepticus and
tory cases not responding to older AEDs/intolerant' These seizures not controlled despite adequate dose.

are used either as first-line or second-line adjunctive therapy.

Duration of Treatment
New anti-epileptic drugs are Vigabatrin (VGB), Oxcarbazepine
(OXC), Lamotrigine (LTG), Topiramate (TPM), Levetiracetam r Withdrawal: If remain seizure free for 2 years
(LEV), Zonisamide (ZNS), Gabapentine (GBP), Tiagabine o Vithdraw one drug at a time
(TGB), Pregabalin (PGB), Felbamate (FBM). o Gradual withdrawal over 5-12 weeks
o Longer withdrawal for BDZ (6 months or more): As with-
Goal ofAED Treatment drawal symptoms are more common.
e Complete seizure control with no or minimal side-effects.
o Maintenance of normal life style.
Monogemenl of First Seizure
o Reduce morbidity and mortaliry. o First afebrile seizure (GTCS and partial seizure) needs to
be addressed.
ILAE Guideline for A[D Drug Level Monitoring i> Chance for recurrence ofsecond seizure after first episode:
o Base-line level of AED after initiation of therapy. 50o/o '

r To check compliance: once or twice yearly. o Chance for recurrence of third seizure after second sei-
zure: B0o/o.
,r Most (80%) of the recurrences occur within first year
and 90% within first 2 years.
Overall recurrence rate for second seizure is 50%.
- R66Ll11snce rat€ for first partial seizure is 80%.
-
a Neurological deficit.
1't monotherapy H---* Seizure free a Underlying cerebral lesion/epileptogenic focal lesion.
a \il4ro have
high risk of epilepsy syndrome: JME vs. BECTS.
a
* a Abnormal EEG done within 24 hour of first seizure.
Seizure free a Seizure rype: Atonic, tonic, as t morbidiry/mortaliry.
a Parrial seizure as recurrences is more.
a Status epilepticus.

Principles of Combination Therapy

Ketogenic diet
Vagal nerve
o 30-40o/o need combination therapy to control seizure'
stimulation o Combinations are prescribed to the patients who remain
Surgery unresponsive to monotherapy.
o Combine either two appropriate firstline or one firstline
Fig. 13.3: Treatment of a newly diagnosed case of epilepsy' and a second alternate iine/newer AED.
 I
ESSENCE OF PEDIATRICS

Table 13.5: Choice of AED in difierent Epileptic Syndromes Myoclonic and Absence 5eizure Aggravating Agents
Carbamazepine, oxcarbamazepine, phenobarbitone,
tnfantile spasms Steroids, vigabatrin SVA, TPM, CZP, CLB gabapentine, vigabatrin, tiagabine, pregabalin, lamotrigine
Lenox-Castaut LTC, SVA, TPM CLB, CZP, LEV (myoclonic seizure only).
Landau-Kleffner SVA, steroids, LTC TPM, LEV
BECTS CBZ, OXC, SVA, LTC TPM, LEV
PROGNOSIS
Benign myoclonic
SVA
epilepsy of infancy
Almost 70%o become seizure {ree, 5-10Vo responders subse-
Severe myoclonic
SVA, TPM, CZP, CLB LEV, Stiripentol quently relapse and remain uncontrolled, 30o/o are "diffjcult to
epilepsy of infancy
treat/control" from outset. Relapse rate is low in generalized
Myoclonic -astatic SVA, TPM, CZP, CLB LTC, LEV
tonic-clonic and absence seizures. Treatment has to be continued
lifeJong in juvenile myoclonic epilepsy. Lack of neurologic and
AEDs with different mechanism of action: Sodium channel psychologic residue indicate better prognosis. It is difficult to
blocker + GABAergic drugs, e.g., PB,BDZ,VGB, andTGB. control seizure in children with neuro-developmental handicaps
Similar spectrum of activity but different adverse evenr and post-traumatic epilepsy. Partial complex seizures are more
profiles + TPM. difficuk to control.
Drug interactions are more common in hepatic enzyme
inducing AEDs like PB, PHT, CBZ.
a SVA is an enzyme inhibitor. , EPILEPTIC ENCEPHALOPATHY
a Enzyme inducing AEDs: J Cotr.. of TPM, LIG, TGB,
ZNS, FBM. It is a condition where medicaily intractable seizures and/or
Drug-drug interactions are unlikely for non-heparic enzyme epileptiform discharges are associated with a progressive decline
inducing AEDs: GBB LEV PGB. in cognitive and behavioral function. Differences between
Better combinations: \?A + II G I BDZ I CBZ. If G + TPM. early myoclonic encephalopathy and early infantile epileptic
CBZ + TGB, PB + PHT. encephalopathy have been listed in Thble 13.6.
o Bad combinations: PHT + CBZ, CBZ + LIG.
CONTROL OF NEONATAL SEIZURE
Strategy for Monotherapy Switchover
See Figure 13.4 for algorithmic approach to conrrol the neonatal
o No conclusive evidence for choosing between alternative
seizure.
monotherapy and switching to combination therapy when
firstline monotherapy fails. Recommendation is to J dose
of first drug and adding second drug Or Unexploined Neonqtql Seizure
o Start second drug -+ build up to an adequare or maximum
Pyridoxine 100 mg bolus IV with EEG, then 10 mg/kg q8hr
tolerated dose and only then taper off the first drug slowly.
PO x 24 hours. If no definite response (EEG normalizarion),
o If second drug is unhelpful, taper either first or second Folinic acid 5 mg/kg q24hr PO x 3 days. If no definite response ,
depending on relative efficacy, side-effects, or tolerabiliry.
Pyridoxal 5'-phosphate (PLP) 10 mg/kg q8hr PO x 3 days.
c Consider combination therapy if seizure conrinues afrer
attempts with monotherapy. If first combination is not
eiTective, a sequence of combinations with potenrial comple-
Table 13.6: Difference between Early Myoclonic Encephal-
inentary mode of action can be tried (dual/triple). opathy and Early lnfantile Epileptic Encephalopathy
If trials of combination not beneficial, reverr ro regimen
inono or combination) that provided,best balance berween
tolerability and reducing seizure frequency.
Onset Neonatal 1-3 months
Filst/second monotherapy improves control but does not
Seizure type M,voclonic Tonic spasm
ploduce seizule freedom: An AED with different but mul-
tiple mode of action should be added. Etiology IEM Anoxia, cerebral dysgenesis
!{ost (60-70%) respond to monotherapy either old or Suppression burst Onlv in sleep Both in awake and sleep
nerver AED, combination therapy I lO-t5Vo more chance on FEC

r',i control. Evolution West svndrome West syndrome, LCS

a 30-{0% rvill need combination therapy. Treatment Benzodiazepine ACTH, Benzodiazepine,
a Oulcorne is better when a second drug is added immediately Bu, Vigabatrin

arte: rhe first drug fails, rather than waiting to see whether Outcome Refractory to Refractory to treatment
s;-o::d druq rvorks. treatment
i

t
 NEUROLOGY

STATUS EPITEPTICUS iii) Put the child in the recovery position.

Status epilepticus (SE) is defined as a seizure lasting longer than
30 minutes or two or more seizures in 30 minutes without
a return to the baseline level of consciousness between the
seizures. Nowadays, the duration is shortened to 5 minutes
for practical purposes.
The Status Epilepticus W'orking Party definition: Status
epilepticus refers to )5 min of (a) continuous seizures or (b)
two or more discrete seizures between which there is incomplete
recovery of consciousness.
SE has three phases: (l) early SE (5-AO minutes), (ll) estab-
lished SE (>30 minutes), or (iii) refractory SE (seizures persist
despite treatment with adequate doses of two or three initial
anticonvulsant medications).
Common acute causes of SE in children include prolonged
febriie convulsion, meningitis (bacterial and viral), traumatic
brain injury, stroke (ischemic and hemorrhagic), inflammatory
conditions, metabolic abnormalities, and sudden withdrawal
of anti-epileptic drugs.
. iii)
Principles of Monogemenl
I
1. ABC
I
I
r
A-Airway (oral cavity, nostrils):
i) Opening up the airway by HeadTilt-Chin Lift maneu-
ver (head and neck slightly extended and line from
chin to jaw angle perpendicular to iloor) or by putting
airway tube.
ii) Clearing up the airway of secretions' vomitus by suction
with sucker machine or by manual rubber sucker.
iv) Sometimes, endotracheal intubation may be necessary
to maintain the airway.
B-Breathing:
i) Assess breathing-signs of respiratory distress
(respiratory rate >70 breaths/min, oxygen saturations
(SpO, <90%, flaring alae nasi), clinical examination
of the chest (symmetry, expansibiliry intercostals and
subcostal recession).
ii) If the patient have dyspnea or cyanosis, give oxygen
through face mask (>5 L/min) or nasal catheter (1-2
L/min).
iii) If impen ding respiratory failure, provide assisted ven-
tilation by bag and mask ventiiation or intubation and
mechanical ventilation.
C-Circulation:
i) Assess circulation-pulse rate, blood pressure, capil-
lary refill time, cardiovascular examination.
ii) Secure an IV line and infuse crystalloid fluid if
required.
If patient is in shock (low systolic blood pressure
and capillary refill time >3 seconds), then manage
the patient with:
IV Normal saline (20 ml/kg) bolus rapidly, then
- reassess and if no improvement repeat.
Injection Dopamine (10-20 prg/kg/min), if
- necessary.
Current protocols all begin with administration of abenzodiaz-
epine, followed by the use of phenytoin and then phenobarbital.
If SE persists at this point, it can be considered refractory SE,

Correci hypoglycemia or
hypocalcemia if abnormal and
simultaneously load with PB

Wean AED slowly when

seizure stops
lnvestigation for
lV PB 20 mg/kg slowly over
specific cause
20 min @ <1 mg/kg/min
depending upon
clinical feature Consider other anti-epileptic drug $
@
A
I
I

Repeat PB 10 mg/kg/dose. lf Consider Midazolam/

seizure continues, repeat PHB Lidocaine, Pyridoxine, Folinic
10 mg/kg/dose after 10 min acid, consider ventilation
Start Phenytoin 20 mg/kg/dose
(lV only) or Fosphenytoin 30 mg/
kg/dose (lv/lM) diluted in normal
Seizure continues saline @ <1 mg/kg/min ..........''.............- Seizure continues

.l
Fig. 3.4: Algorithmic approach to control neonatal seizure
 -
ESSENCE OF PEDIATRICS

Advantages of phenytoin over phenobarbitone as second-

line treatment:
PR Diazepam 0.5 mg/kg
max 10 mg
o Traditionally, based on long-term clinical experience and
case-control studies, IV phenytoin has been used as the
Seizure continue C second-line drug for SE.
c)
E
o
o Phenytoin nters the brain more rapidly than
e

Repeat dose of PR o, phenobarbitone.

c(s
Diazepam after 10 min
c)
o SIow onset of action of phenobarbirone compared to phenytoin.
E o Lack of significant CNS depression compared to
.9
Seizure continue
phenobarbitone.
-o
lV PHT drip 20 mg/kg
(U
c)
o If phenobarbitone is to be used as a back-up to a benzodi-
E azepine, the child mLrsr be intubated.
(Max 1500 mgl24hr)
c)
@rate <1 mg/kg/min _c
Monitoring of the patient:
(max 50 mg/min) or
od
over 20 mins r Monitor RBS, electrolytes, calcium, magnesium, HR, BB
E and SpOr. Tieat acidosis. For children less than 3 years of
Seizure continue c)
age, consider vitamin 86 IV 100 mg in seiected cases with a
O)
PB 20 mg/kg (Max 1g) o
o_
history of neonatal seizures, infantile spasms or early onset
@ rate <1mg/kg/min -c seizures and EEG.
(max 100 mg/min) c(U
or over 20 min =E FEBRILE CONVUTSION
Seizure continue- o
c Febrile convulsions are the commonest type of neurological
o
c) problem in children. It is estimated that about 3o/o of all chil-
Midazolam; 0.2mglkg of
dren suffer fi'om febrile seizures and <3o/o may develop epilepsy.
boluses max. 2 mgkg, EX
then 0.05-2 mg/kg/hr LU It is believed that febrile convulsion occurs due ro remporary
increase every impairment of the balance between the convulsant and anti-
15 minuies up to 2 convulsant systems of the brain. Probably the threshold level
mglkglhr of the anticonvulsant system in these genetically predisposed
Seizure continue chiidren is comparatively lower and therefore, they react rvith
convulsion due to rapid rise of temperature.

Propofol 1-2mglkg boluses, max. 10 mg/kg, Criteriq for Diognosis

then 2-10 mg/kg/hrllntubation under GA
-Give lV glucose, sodibicarb, anti-edema measures (e.9.,
Corticosteroid
and mannitol).
Fig. 13.5: Treatment algorithm ior status epilepticus.
and the current standard of care is to place the patient into
drug-induced coma (Lorazepam/Propofol) (Fig. t3.5).
r Intravenous diazepam can be given in a dose of 0.3 mg/kg
to a maximum of 10 mg. As an initial agent, lorazepam
is better than diazepam because of equal e{icacy, but IV
lorazepam has a longer half-life and less respiratory depres-
sion. It is given in a dose of 0.1 mg/kg (ma-x.4 mg) over
2-3 minutes or at the rate of 1-2 mg/min. Intravenous
lorazepam was significantly more iikely to terminate SE
than per rectal (PR) diazepam.
r Intravenous fosphenytoin may be administered more rapidly
than phenytoin (in children, ma-r. 3 mg/kgimin fospl-re-
nytoin vs. 1 mg/kg/min phenytoin).
Age and sex: Usually 6 months-5 years, peak age 18 monrhs.
Males are affected slighdy more commonly than females.
Family history: There may be positive family history (50ok).
Mode of inherirance is autosomal dominant, demonstrated
in some families.
Infections: 907o of cases are due to viral infectior-r. Common
infections are pharyngitis, otitis media pneumonia, UTI,
roseola.
a
a Seizure occurs with a rapid rise of temperature.
a Onset is within 24 hour of illness, mostly within 12 hour
of developmenr of fever. Type is generalized tonic-clonic,
duration is <i5 rnin. Usually, there is one seizure attack at
each episode and rarely r,,vo. There is no residual weakness
of limbs or disability excepr a brief period of drowsiness.
Recurrence: 50%o under I year and 30o/o in other situation.
Frequency decreases after 5 years of age.
EEG: EEG done within a rveek after a febrile convul-
sion may be abnorrnal, but aftel a u'eek it usually shows
no abnormaliqr EEG should not be advised routinely, as
EEG change does not alter the course of FS or subsequent
development of epileps,v.
I
t
t

Invesligotions
Lumbar puncture (LP) is not indicated in all cases of febrile
convulsion. It depends on the experience and judgment of the
doctor concerned. However, if a child is below 1 year of age and
coming with first episode or if the age is >5 years, LP should
always be considered. In addition, if there is even slightest
doubt of CNS infection as evidenced by undue prolongation
of postictal state, it is always better to do an LP.
Treolmenl
r Explanation and reassurance of parents.
o Removal of excess clothing.
o Reduction of body temperature by tepid sponging. It is
better to use normal tap-water and use a fan, rather than
using ice-cold water.
o Antipyretic:
o Paracetamol 10-15 mg/kg/dose can be given orally or
rectally 4-5 hourly, when the temperature is >100oF.
r In rare cases, when the temperature does not subside
with paracetamol, ibuprofen 5 mg/kg/dose three times
t
daily can be given orally.
o The source of infection should be looked for and treated.
o To control convulsion: Diazepam 0.3 mglkgldose can be
given very slowly IV or 0.5 mg/kg PR. Required amount
of diazepam is taken in 3 cc disposable syringe. The nozzle
is attached to a 6 FG nasogastric tube (or to a tube of a
butterfy needle), which is introduced into the rectum.
Diazepam is now pushed into the rectum, and then 2 cc of
normal saline is pushed through the tube. The tube is then
withdrawn and the buttocks are approximated by hands or
leucoplast for sometime. Nowadays, diazepam is available
in suppositories, which can be used easily.
Prophyloxis
o The risk of recurence can be reduced by diazepam 1 mg/kg/d
(max. 10 mg) PO 8 hourly for 2*3 days to be started at
the onset of each febrile illness. Alternatively, oral Clobazam
I mg/kg/d (max. 20 mg) can be used considering same
efficacy, singie daily dose, and less ataxia and drowsiness
compared to diazepam. (
o Short-term anticonvulsant prophylaxis is not indicated.
o Prolonged anticonlulsant prophylaxis for preventing recurrent
febrile convulsion is no longer recommended. Phenytoin and
carbamazepine have no efFect on febrile seizures. Phenobarbi-
tone is ineffective, may decrease cognitive function. Sodium
r-alproate is effective in the management of febrile seizures,
bur the potential risks of the drug do not justifz its use in a
disorder.,vith an excellent prognosis irrespective of treatment.
Prognosis
\o aC','crse efrect is seen on academic, or behavioral activity
.r'-:: i: r;izures are recurrent. Seizure type, tfeatment modali-
:-;.. FiG ,hanges do not alter eventual outcome as epilepsy.
NEUROLOGY
ATYPICAI FEBRITE CONVUTSIONS
If the conr'ulsion with fever persists for >15 minutes,
associated
occurs more than once in 24 hours, focal or unilateral in nature
and/or is followed by Toddt paraiysis, but no significant other
reason or CNS infection cannot be found for the fit, it may be
called atypical febrile con'ulsion. In these children, the EEG may
remain abnormal for 2 weela or more following the attack. In
these cases, continuous prophylaxis therapy may be given.
Crileriq for Prophyloctic Anliconvulsonl
Theropy
o Patients under 18 months of age with previous abnormal
development or abnormal neurological signs.
o Atypical febrile seizures.
r Recurrent febrile seizures.
o High level of parental anxiety.
Continuous daily prophylaxis can be given with sodium val-
proate 30-60 mg/kg/d in 2 div. doses, to be continued for at
least 2 year fit-free or unril the child is 6 year old, whichever
comes earlier.
BREATH HOTDING ATTACKS
There are two types of breath holding attacks or spells:
(l) cyanotic and (;z) pallid.
Cyonolic Breolh Holding Allqcks
Age of occurrence is 3 months to 5 years. Following a trauma
or an emotional event, the child cries for a few seconds and
then holds the breath in expiration leading to cyanosis. The
child then loses consciousness and becomes limpy. Usually the
attacks end here. But if the hypoxia continues for a longer
time, the child may develop convulsion. However, usually the
recovery is rapid. Such attacks may be very frequent. occurring
several times a day.
Treatment
No specific treatment is necessary or effective. Parents should
be reassured that the attacks are harmless and they will stop
in course of time. Parents should try to avoid precipitating
H
factors, such as emotional upset of the child.
Pqllid Breolh Holding Atlqcks
This is a misnomer, as these are not true breath holding attacks.
They are, in fact, a type of reflex asystole and called reflex
anoxic seizures caused by vagal responsiveness. The episodes
start in a similar way as breath holding attacks. Following a
minor trauma or a bump to the back of the head, the child
becomes suddenly pale and limp and loses consciousness. Brief
convulsion may occur. The whoie episode lasts from 30 seconds
to 1 minute and the recovery is rapid.
ESSENCE OF PEDIATRICS
Treatment
Usually not necessary. If very frequent, atropine may be used.
Correction of associated iron deficiency reduces frequency.
Zinc can also be given.
PEDIATRIC EEG
Electroencephalogram (EEG) is the most useful tool to assess
cerebral function and identi$. dysfunction. It provides the high
temporal resolution of neuronal interactions at the network
level. The membrane currenrs generated by the neurons pass
through the extracellular space. The electrical field potential
recorded at any given site are the current from the source
(intracellular space to the extracellular space) and sinks (current
from the extracellular space to the intracellular space). These
electrical field potential measurements provide the best experi-
mentai and clinical tool for assessing cooperative neuronal
activity at high temporal resolution.
The advanced equipment provides lots of flexible methods
and technique for the data recorder and the reviewers. Vith
the present computerized machinepthe dara can be displayed in
vary many ways changing the montages ar rhe same recording
point. The time-locked VIDEO recording helps the interpreta-
tion a lot nowadays.
Adult vs. Pediolric EEG
Although the basic principle being the same, rhe pediatric EEG
varies in some ways such as limited parienr cooperation thar
takes extra time and work out with the family and the parienr,
particularly in infants and children with behavioral problems,
with poor cognition and multiple disability. Because of vari-
able size and shape of head and scalp of newborn and young
infants, the electrode placement technique needs extra care
and training. The evolving functional pattern of the growing
brain, their wide range of normal variation in physiology
and pathophysiology are the main reason why and how the
pediatric EEG differs from that of the adult. The plus point
in pediatric EEG is that the pathology is readily identifiable
probably because of the thin skull.
'
Recording Techniques
Electrode: Preferably the lighter electrode cup with hole in
the middle and the wire longer and lighter works better for
the children, which allows the eiectrodes remain attached
with the scalp better even when the child moves the head
while playing.
Electrode placement: The Committee of the International
Federation of Societies for Electroencephalography and Clinical
Neurophysiology (IFSECN) recommended a specific system of
electrode placement under standard conditions for use in all
laboratories: 1 0-20 system. American Clinical Neurophysiology
Society has recommended using a minimum of 21 eiectrodes
in the international 10-20 system (some use the modified
Moudsley system). Odd-numbered electrodes are placed on the
left side of the head and the even-numbered electrodes on rhe
right side of the head. Specific letters designate rhe anatomical
area; for example "F" means frontal.
Machine setting: Certain important points need to be men-
tioned at this point. The recording band pass should not be
restricted just to make it arftifact-free, because that may change
the morphology of actual wave or discharges. As, for example,
restricring high frequencies with filters may make it difficult to
differentiate fast activities or even spikes from filtered muscle
action potentials.
Activation process: Will provoke the potential epileptogenic
area in the brain. Activation process musr be performed care-
fully, because that would make a rouring test complete and
reduce the chance for the second call to complere the report.
The activation process must include photic stimulation, eye
closure, drowsy and early sleep state and hyperventilation
in older children in particular. Sedation should not be used
merely to obtain an aftifact-free EEG recording, because
recording in waking state even through lots of movement
artifacts are on the whole more useful, although EEGs taken
during drowsiness and sleep (natural) can add further infor-
mation in certain instances. In case when recording started
in sleep state, the parient needs to be awakened to obtain
data in waking state. In certain cases, sleep deprivation test
is advised to obtain enough data available for reviewing,
particularly children with suspected complex partial epilepsy,
idiopathic generalized epilepsy, children with behavioral
disorder. speech delay. or regression.
The technicians should preferably be equipped with toys
and other needful to get cooperation from the children.
Reporting and interpretation of the report: Description
part of the EEG report should explain the whole cerebral
background activiry change of the rhlthmic activities of specific
areas with change of the childt vigilance, reacrivity to exrernal
stimuli, including the dysfunction.
In the comment part, an experienced neurophysiologist
should be able to specif, whether the wave bands are within
or out of the normal limit for the age and state of the child.
Identi$' the specific abnormal pattern and correlate them with
the child's clinical condition ro suggesr a probable diagnosis.
Terms Commonly Used in EEG
EEG montage: It is the general arrangemenr of the EEG
recording channels. This refers to the reiationship berween the
electrode connections and the channels displayed. \7ith present
advanced digital machine, this can be reformatted after record-
ing, i.e., while reviewing according to the reviewer's choice.
-
Polygraphy: Describes the simultaneous recording and display \
of multiple physiologicai measurements such as EEG, ECG, t
EMG, respiration, eye movements, etc.
I
I
i
I
rt

Frequency: Is the number of repetitive waves in 1 second,
written as cycles/sec (or c/s). The EEG activiry frequencies are
divided into frequency bands-deba: 'slow' activity below 4
cls; theta:'intermediate slow' activity, 4 to <8 cls; aQha: 8-13
c/s, 'alpha rhythm' specifically refers to the normal rhythmic
activity of this frequency seen over the posterior region of the
brain on eye closure; beta'.'cast' activiry faster that 13 cls.
Amplitude (voltage): Refers to size of signals and is usually
measured (peak to peak) in microvolts (pV).
Mu-rhJthm: Short runs or rhythmic activities, 7-11 cls,
having an archlike or comb tooth like shape seen over the
frontocentral region that appear during wake and alert state.
t to describe the EEG at certain levels of anesthesia. It should
This indicates the state of vigilance of the person. First appear
> not be confused with "trace alternant", which is seen in quiet
during the age of 2-3 year, increased during the age of 8 and
I 16 years. It is more commonly seen in girls than the boys.
l
I Lambda waves: They are normal sharp, triangular-shaped tran-
I sients, seen over the occipital region during visual exploration,
may be seen in'young children.
Background activity: Ongoing EEG activity, excluding the
I o
specific activities, representing tBe setting in which a given
normal or abnormal pattern appears or stands out.
Sleep rhyhmic pattern: Particularly in children, the drowsy
states are weli-noticed in the EEG even when the child looks
play4ul and waking. The appearance of rhythmic theta waves
over the frontocentral area followed by apparent paroxysms of
theta waves of higher amplitude compared to the background
activities indicates the drowsy state (hypnogogic hypersyn-
chrony).
Vertex sharp transients: Normal"sharp transients that usually
appear at the first stage of sleep,
K-complexes: Large amplitude usually biphasic slow waves
having a slope with fast activities, frequently associated with
sleep spindles around the vertex. They are first seen at 4 months
of age appears in first to second stage of sleep that starts to
disappear at third stage of sleep.
Sleep spindle: Runs of rhythmic activities appearing in spindle,
l2-I4 cls most prominent over the vertex (central region)
during second to third stage of sleep.
Spikes/sharp waves: tansients that are clearly distinguishable
from the background/rhythmic activities and are usually surface
negative. A spike is of shorter duration (<70 ms, approximately
1l\4'h of a second) compared to a sharp wave (70-200 ms).
Polyspikes are multiple spikes grouped together.
i Comple* A sequence of two or more waves recurring with
F
I similar relationship to each other, e.g., spike-wave complexes,
I complex of multiple sharp and polymorphic slow waves, etc'
I Discharge: This is an interpretative term, which usually
a
I refers to sharp waves/spikes with or without associated slow
\\'a\-es. Some authors mention this as hallmark of the epilepsy
I
t
F
NEUROLOGY
diagnosis. Epileptiform activity has similar connotation.
Dysrhythmia is a non-specific term, which has been used
to describe a wide range of cerebral dysfunction including
epileptiform discharges.
Parorysms: Are clearly defined episodes with abrupt onset
and termination that may include slow waves. Usually, when
a paroxysm contains definite spike/sharp wave is termed as
bursts with such range of discharges.
Burst-suppression is a pattern characterized by burst of
theta and/or delta waves sometimes mixed with faster activ-
ity and/or spiky components separated by periods of relative
quiescence. Theta are widely misused and should be restricted
sleep state of normal neonates.
Role of lnlerictol EEG
EEG recorded during interictal period will help in:
o Confirmation of diagnosis.
Defining type of epilepsy-partial vs. generalized.
o Identification of epileptic syndromes: LKS, \7est syndrome,
Rolandic, LGS, CAE where EEG is always abnormal.
o Planning drug management.
o Epilepsy surgery.
o Evaluation of first seizure: Risk of seizure recurrence can
be predicted.
o MonitoringAED withdrawal: Guide decision but presence of
occasional brief epileptiform discharges should not preclude
withdrawal in seizure-free patient.
o Sensitivity: First EEG 30-55o/o; serial EEG 80-90%.
o Interictal recording is normal in -40o/o of patients. Activation
procedures including hyperventilation (absence 80%, focal
10%), eye closure, photic stimulation, and sleep deprivation
increase the positive yield.
o Up to 3.5o/o of normal children: EEG may be abnormal.
EEG Wqves
An EEG may have different forms of waves:
Alpha waves: Usually 8-13 cycles/sec with high amplitudes.
Found in the posterior region of the brain, highest in the
dominant side. They may be brought about by closing eyes,
while disappear during eye opening.
Beta waves: Usually > i 4 cycles/sec, but with iower amplitude.
These waves are accentuated by sedation. These rhythms are
dominant in an alert and awake patient. They are absent or
diminished in cortical damage.
Theta wavest 4-7 cycleslsec. These are high-voltage waves.
Presence of these waves in awake adults or older children may
be abnormal. However, up to the age of 72 years, presence of
these waves is considered as normal. If present in an uncon-
scious child, it suggests encephalopathy.
 ESSENCE OF PEDIATRICS

Delta waves: <3 cycles/sec. Normal in infants, especially in thereafter crosses Blood-CSF barrier at choroid plexus oflateral
sleep. They may be focal in subcortical lesion. Found as gen- ventricle/cerebral capillaries. This way, organisms ultimately get
eralized waves in diffuse brain disease. entery into CSF spaces (ventricle and subarachnoid space).
Presence of the follolving waves is aiways considered as
abnormal: Clinicol Feolures
o Spikes Fever, alteration of mental starus (assessed by GCS), convulsion
o Monospikes (found in benign rolandic epilepsies) (30% cases), headache, vomiting, photophobia. Bulged Gnianel
. Spikes and waves (3 cycles/sec in absence seizures, 2.5 cyclesl (<2 years) and signs of meningeal irritation: neck rigidiry (>1
sec in Lennox-Gastaut syndrome) year of age) and Kernig sign.
r Polyspikes
r Sharp waves (normal in children posteriorly) Diognosis
o Burst suppression o CBC, CSF study (Table 13.7).pSF cell counr ro be done
within half hour, otherwise cells will disintegrate.
o Agglutinarion test. ,.
o Blood sugar should be estimated half hour before lumbar
it may be defined as infammation of the leptomeninges (pia punciuie for accurare calculation of CSF sugar.
and arachnoid mater) covering the brain and spinal cord. o CT scan/MRI of brain: lllese are done when complications
are suspected.
Classification:
A. According to etiology: Treolmenl
1. Pyogenic or bacterial m€ningitis
Resuscitation
2. Yiral meningitis
3. Tubercular meningitis Airway, Breathing, and Circulation should be maintained, if
4. Parasitic, fungal meningitis (malaria, amebic, fungal) required.

B. According to duration: Specific Treatment

1. Acute meningitis (<4 wks): Bacterial and virai
meningitis
2.
Chronic meningitis (>4 wks): Partially treated pyogenic,
tuberculaq and fungal meningitis
BACTERIAL MENINGITIS
Eliopothogenesis
Haemophilus influenzae rype b, Streptoc\ccus pneumoniae, Neis-
seria meningitides are common in older infants and children.
Post neurosurgery, posr skull trauma, or in CSF shunt, the
common organisms are staphylococci including coagulase-
negative species, Pseudomonas, and enterococci.
_ Bicterial colonization of nasopharynx proceeds towards
epithelial invasion and endocytosis. This is followed by inva-
sion of organism into blood stream. This hematogenoqs spread
leads to bacteremia and entry into CNS. There may also be
direct spread into CNS from contagious sites (sinus, middle
ear infection, fracture cranium). Once into CNS, the organism
Initial antibiotic therapy should be started with vancomycin
(60 mg/kg/d given in infusion every 6 hour). As part of initial
, empirical therapy, cefotaxime (200 mg/kg/d given every 6 hour)
or ceftriaxone (100 mg/kg/d as a single or 12 hourly doses)
should also be used. f;atients allergiclo BJactam antibiorics
and>1 month of ."n be treaied with chloramphenicol
(100 mg/kg/d given"$
every 6 hour).
In .S. pneumznide meningitis, rhe trearment should be
completed in 10-14 days, in lVeisseria meningitides rn 5-7
days, and in H. influenz/te type B meningitis (uncomplicated)
in 7-10 days.
, Antibiotics should be chosen according to culture and
sensitivity or clinical response. In case of suspected organism,
antibiotic may be chosen according to Thble 13.8.
Dexamethasone Treatment
,In pyogenic meningitis, dexamethasone acrs as an anri-in{lam-
matory agent, reduces vasogenic cerebral edema, reduces hearing

Table 13.7: CSF Picture in Various Condirions

0-4 mononuclear 20-40 40-80

Acute bacterial meningitis (ABM) 10:] polymorphs lncreased Markedly * Cram staining 90% +ve
.l
ABM partially treated 0r-1 03 mononuclear or polymorphs Nll NiJ
Aseptic meningitis 1 O-1 02 mononuclears N/f N Virus isolation
Tubercular meningitis 1 0-l 02 mononuclear t + AFB +ve 30%

t
I
 NEUROLOGY

Ioss relatedto H. infl.uenzae and pneumococci, and reduces
the incidence of long-term neurological sequelae and overall
mortality rates. .Dexamethasone 0.15 mg/kg/dose is given
every 6 hour for ) d"y.. In children older than 6 weeks having
H. influenzae type B and pneumococcal meningitis, it should
be given before starting antibiodcs.
Symptomatic Treatment
Patient may present either with history of seizure or ongoing
s9!zu1e (convulsing state). The ongoing seizure can be termi-
nated by diazepam (PR or IV route) as per usual dose. $not
controlled, then IV phenytoin 20 mg/kg diluted with 50 ml
of normal saline over half hour or at the rate 1 mg/kg/min
through syringe infusion pump should be given, In its absence,
phenobarbitone IV 20 mg/kg at the rate 1 mg/kg/min can be
used. The maximum dose of phenytoin or phenobarbitone
is 1000 mg and 300 mg, respectively, irrespective of age
and weight. Thereafter, acute symptomatic seizure should be
managed with any one of the oral fonnulations like phenytoin,
carbamazepine, or phenobarbitone as per usual dose so long
the acute state ofthe disease persists (1 week).
Treatment of Complications
Raised lntrarranial Pressure (l(P)
Almost all patients with menin-
gitis have high intracranial pressure, but this does not preclude
Iumbar puncture. Cerebral edema, whether cytotoxic, vasogenic,
or interstitial in origin, is the major element contributing to the
increased ICP. The signs of raised intracranial pressure include
altered level of consciousness, bradycardia, hypertension, pap-
illoedema, headache, vomiting, and cranial nerve palsy (IIi and
VI) or its attended complication such as cerebral herniation.
Symptoms and signs of cerebral herniation:
r Glasgow coma score <8
o Abnormal pupil-unequal size and reaction, uni or bilateral.
o Abnormal 161s-ds6s11icate/decelebrate posturing, flaccidity

Table 13.8: Recommended Antimicrobial Therapy

Croup B streptococci Penicillin/Ampicill in + Centamicin Ceftriaxone or Cefotaxime

Li s te r i a m o n o cytogeres Ampicillin + Centamicin Co-lrimoxazole

H aemop h iI us i nfl ue n zae Ampicillin + t hloramphenicol or Cefolaxime or Cefepime or fluoroquinolones l ike gatiflorac in.
Cettriaxone moxifloxacin
Nelsserra men r ngitides Penicillin or Ce{triaxonc or Cefotaxime Chloramphenicol

Stte ptococcu s pneu mon i ae i. Penicillin or Ceftriaxone or Cefotaxime Chloramphenicol. Meropenem,

ii. Ceftriaxone or Cefotaxime + vancomycin
iii. Cefotaxime or Ceftriaxone + Rifampicin
iv. Vancomycin + rifampicin (B-lactam allergy)
Staphylococci Nalcillin or Oracillin Vancomycin + Rifampicin
Pseudomonas i. Ceftazidime + aminoglycoside Meropenem + aminoglycoside
ii. Cefepime + aminoglycoside

F. coll and other Enterococcus Ampicillin + genlamicin Vancornycin - gentamir in

Arnpicillin 150 mg/kg (B) 200 mg/kg (6-8) 200-300 nrlkg (5)

Centamicin s mg/kg (12) 7.s mg/kg (8) 7.s mg/kg (B)

Tobramycin s mg/kg (12) 7.s mg/kg (B) 7.5 mg/kg (B)

Amikacin 1s-20 mg/kg (12) 30 mg/kg (B) 20-30 nrg/kg (B)

Ceiotaxime 100-ls0 mg/kg (B-1 2) 1 s0 200 mg/kg (6-8) 200-300 mg/kg (6-S)
Cettriaxone 80-1 00 mg/kg(12-2a)
Chloramphenicol 2s mglkg {2a) 50 nlg/kg (,12-24\ 75-1 00 mg/kg (6)

120 mglkg (B)

"',e:openem
nte 1s0 mg/kg (s)
-:ler:
.::-C':a\Cin
-
20-30 mg/kg (B-1 2) 30-a5 mg/kg (5-8) 60 mg/kg (6)
--.- i ::e 100 1s0 mg/kg (B 12) 150 mg/kg (B) 1s0 mglkg (B)
 ESSENCE OF PEDIATRICS
o Respiratory abnormalities-hyperventilation, Cheyns-g1sks.
breathing. apnea, respirarory arresr
r Abnormal doll's eye movemenrs
Management of increased ICP:
r Elevate the head of the bed to 30" to maximize venous rerurn.
o fV Frusemide (1 mg/kg/dose) and Mannitol (0.5-1 g/kg/dose)
may reduce increased ICP. Oral glycerol (glycerin) also
decreases ICP by reducing cerebral edema.
Subdural Effusion It is present in one-third of cases. It results
from altered permeabiliry ofvessels traversing subdural space and
rupture of veins in subdural space, which results from shift of
intracranial contents follorving raised ICP They are asymptomatic
and resolve spontaneously without permanent neurologic sequelae
in most cases. The clinical features of both subdural effusion
and empyema are same, which includes persistent or recurrent
fevers, seizures, tense anterior fontanel, declining neurological
status despite adequate antibiotic rherapy. tansillumination test,
,cranial ultrasound, and CT scan ofbrain are different diagnostic
techniques for its detection. Subdurai paracenresis should be
performed if it does nor resolve spontaneously.
Subdural Empyema This is an uncommon complication occur-
ring in 2o/o cases. Management includes surgical drainage via
subdural taps or burr holes and appropriate antibiotic.
Ventdculitis Ventriculitis results from the extension ofmeningitis
into the ventricles, rupture ofa brain abscess or a neurosurgical
procedure or device. Ventriculitis as a complication of meningitis
is more common in children than adults. Among children, it
occurs predominantly in neonatal and infantile period. It is
particularly associated with gram-negative bacillary and staphy-
lococcal (aureus and epidermidis) meningitis, which usually
develop resistance ro conventiohal andbiotics. Non-resolving
feve; progressive hydrocephalus are clues for its suspicion. It
is confirmed by ventricular tap. Other non-invasive diagnostic
methods are cranial ultrasonogram and CT scan-MR[ of brain.
The ultrasonographic features of bacferial ventriculitis are (l)
increased echogenicity of the ventricular fluid, either in a fine
homogenous pattern, or with strandlike material and coarse par-
ticles, echogenic materials within ventricles (ventricular sludge);
(li) intraventricular septa formation/ventricular compartmen-
talization or loculation (sonography is superior to CT scans in
identifizing intraventricular septae) ; (lll) increased echogeniciry of
the ependymal lining of the ventricles; (iu) ventricular dilatation
with irregularity of the ventricular inargins; (a) loss of definition
ofthe surface ofthe choroids plexus, i.e., poorly defined choroids
plexus margins with loss of the normal smooth contour; and
(erl) increased periventricular echogenicity.
Supportive Treatment
Fluid management:
o Parient should initiallv receive nothing by mouth. If
a patient jusr ro be normovoiemic x'ith normal blood
pressure, IV fuid should be restricted to one-half to
two-thirds of maintenance, or 800-1000 ml/m2/d, until
it can be established that increased ICP or SIADH is
not present. Fluid administration may return to normal
(1500-1700 ml/m'zld) when serum sodium level is normal.
British Infectious Society working party, however, showed
that liberal fluid administration did not aggravate brain
edema in meningitis.
A significant proporrion of meningitis cases present with
dehydration and/or hypovolemia for other reasons (e.g.,
vomiting, dimlnished intake, diarrhea), which also require
fluid resuscitation.
Syndrome of inappropriate secretion ofADH (SIADH)
occurs in 5-80o/o cases of meningitis. The cardinal features
of SIADH described by Bartter and Schwartz are as
follows:
o Hyponatremia with corresponding hypo-osmolality of
the serum and extracellular fuid.
,r Osmolality of urine grearer rhan rhat appropriate for
the concomitant tonicity of th<i plasma (i.e., less than
maximally diluted).
o Absence of chemical evidence of volume depletion (i.e.,
normal skin turgor and blood pressure).
o Normal renal function.
o Continued renal excretion of sodium.
c In addition, all the physiologic srimuli that contribute
to control of ADH secrerion, such as hypertoniciry low
blood volume, temperarure, body position, and pain,
must be absent.
SIADH treatment: If SIADH develops, about 800*1000
mllm2ld should be given inltially that is fluid restriction
should be done abou Il2 of the daily maintenance. Once
serum Na level is >135 mEq/L, the volume of fuids can
be gradually liberalized by increments of 200 mllm2ld at
intervals of 6-12 hours. In most cases, normal mainte,
nance rate of 1500-1700 ml/m2ld could be reached over
48 hours. 5%o dextrose in0.9o/o saline or a muiti-eiectrolyte
solution containing approximately 40 mEq sodium/L,
20-40 mEq potassium/L, and 20 mEq acetate/L can be
given.
Treatment of unconscious patient: Adequate eye, skin,
bladder, and bowel care, and oral hygiene should be main-
tained.
Prevention
1) Vaccination
a) Hib vaccine for H. influenzae rype-b
b) Pneumococcal polysaccharide vaccine heptavalent
(PCV7-pneumococcal conjugare vaccine-7 valenr).
c) Quadrivalent meningococcal vaccin e: Al CIY l\Y135
vaccine
2) Effective chemoprophylaxis of carriers of Hib and
meningococcal meningitis.

VIRAT MENINGITIS
Common etiological agents are (l) enteroviruses, such as
coxsackie viruses, echoviruses and polioviruses (80%);
(il) arboviruses; (iii) mumps and measles viruses; (iz) herpes
simplex and varicella zoster viruses; and (z) cytomegaloviruses
in immunocompromised children.
Clinicol Feolures
Clinical features have a wide range of variability, even if the
infection waS by same agent. The onset is generally acute'
followed by a short introductory non-specific febrile illness.
Older children may complain of headache and hyperesthesia;
in more younger ones, irritability and lethargy may be found.
Fever, nausea and vomiting, photophobia, and pain in the
neck, back, and legs are common; high rise of temperature
may lead to stupor with bizarre movements and convulsions.
Stationary, progressive or fuctuating focal neurological signs
may be found. R.arely, some chiidren may come with sudden
deterioration lapsing into coma. Exanthemas are common just
prior or during the illness.
Diognosis
The diagnosis is made on the clinical presentation, supported
by CSF findings. In the CSF, there will be moderate increase
in lymphocytes. The glucose level is usually normal (in mumps
hypoglycorrhachia may be found). The protein content is mildly
increased, rarely exceeding 100 mg/dl.
The EEG shows diffuse slow wave activities, without focal
changes. CT scan and MRI of brain shows generalized swelling
of the brain parenchyma.
Treolmenl
The course is usually self-limited with variable sequelae.
Until a bacterial cause is excluded, parenteral antibiotic
therapy (chloramphenicol + ampicillin or'cefotaxime +
ampicillin) should be given. Once the diagnosis is confirmed,
no specific treatment is needed (except HSV encephalitis).
In patients with HSV acyclovir should be given. The dosage
is-for neonates, 30-45 mglkg/d IV in divided doses
8 hourly; for children, 15 mg/kg/d IV in divided doses
8-12 hourly to be given for 10 days.
a For headache and fever, analgesics should be given.
a IV f uid may be needed, with 20o/o restriction if oral intake
is poor.
However, severe patients may develop convulsions, cerebral
edema, hyperpyrexia, respiratory distress, fuid and electro-
lr'te imbalance, even cardiac and respiratory arrest. These
patients should be treated accordingly.
Treatment rvith steroid, interferon has not yielded satisfac-
:on' result.
I
NEUROLOGY
TUBERCUTOUS MENINGITIS
Tirberculous meningitis is commonest in children under
5 years of age. Tuberculous meningitis usually arises from the
formation of metastatic caseous lesion in the cerebral cortex
or meninges that develops during the lymphohematogenous
dissemination of primary infection. This initial lesion increases
in size and discharges small numbers of tubercle bacilli into
the subarachnoid space. Occasionally, tuberculous meningitis
occurs many years after the infection, when ruPture of one or
more of the subependymal tubercles discharges tubercle bacilli
into the subarachnoid space.
Polhogenesis
Most tuberculous'infections of the CNS are caused by Mycobac'
terium tuberculosis.TB bacil[ reach the CNS by hematogenous
route secondary to disease elsewhere in the body. The CNS
tuberculosis develops in Lwo stages. Initially small tuberculous
lesion (fuchs foci) develop in the CNS, either during the
stage of bacteremia of primary tuberculous infection or shordy
afterwards. These initial tuberculous lesions may be in the
meninges, the subpial or subependymal surface of the brain or
the spinal cord, and may remain dormant for years after initial
infections. Later, rupture or growth of one or more of these
small tuberculous lesions produces development ofvarious types
of CNS tuberculosis. Rupture into the subarachnoid space or
into the ventricular system results in meningitis. Infrequently'
infection spreads to the CNS from a site of tuberculous otitis
or calvarial osteitis. The pathogenesis oflocalized brain lesions
is also thought to involve hematogenous spread from a primary
focus in the lung.
Clinicol Feolures
The signs and symptoms progress slowly over several weeks
and can be divided into three stages:
lst stage: week. Characterized by
It typically lasts first 1-2
non-specific symptoms such as fever, headache, irritability,
drowsiness, and malaise. Focal neurological signs are
absent.
2nd stage: Usually begin more abruptly. The most common
features are lethargy, nuchal rigidiry seizures, positive Kernig
H
or Brudzinski sign, hypertonia, vomiting, cranial nerve palsies,
and other focal neurological signs. The accelerating clinical
illness usually correlates with the development of hydrocephalus,
increased intracraniai pressure, and vasculitis. Some children
have no evidence of meningeal irritation but may have signs
of encephalitis-disorientation, movement disorders, or speech
impairment.
3rd stage: Marked by coma, hemiplegia, paraplegia, hvper-
tension, decortication, decerebrate posturing, disorientation,
deterioration ofvital signs, and eventually death.

ESSENCE OF PEDIATRICS
Diognoslic Feqlures
o Clinical
r Fever and headache for >14 days
o Vomiting
.r Altered sensorium or focal neurological deficit
r CSF
o Pleocytosis (>20 cells, >50o/o lymphocytes)
,r Increased proteins (>100 mg/dl)
> Low sugar (<600lo ofcorresponding blood sugar)
,r India ink stain (for Cryptococcus) and microscopy for
malignant cells should be negative
o Imaging
c Exudates in basal cisrerns or in sylvian fissure
o Hydrocephalus
c Infarcts (basal ganglia)
'r Gyral enhancements
, diabetes insipidus, or mental retardation.
Tuberculoma formarion
o Evidence of tuberculosis elsewhere
Complicolions
Hydrocephalus, cranial nerve palsies, blindness, deafness, hemi-
plegia or paraplegia, speech impairment, mental retardation,
diabetes insipidus. ruberculoma.
lnvesligolions
o CSF analysis:
r Color-hazy I clear lhemorrhagic (rarely).
r Cell i6un1-u5uxlly ranges from 10 to 500 cell/mm3
r Polymorphonuclear leukocyte may be presenr initially,
but lymphocyres predominare in majority of
.) CSE glucose-qpically <46 mg/dl, but rarely <20 mg/dl.
o CSF proteins-elevated and may be markedly high,
400-500 mg/dl.
r AFB staining and culture. Using conventional
enstein Jensent medium requires 2-8 weeks to detect
growth of Mycobacterium, while use of BACTEC 460
TB system requires much less time (l-2 weeks) to isolate
TB. CSF ADA (Adenosine deaminase) has a sensitivity
of 44-100o/o and specificity ofTl-100o/0.
c CBC: Hbo/0, ESR may be high.
a CXR
e -luberculin test:20-50o/o cases positive. BCG test: 90-100%
rases posirive
* {,,astric aspirates for AFB and culture
a iil-/MRI: Thickening and intense enlargement of basilar
meninges. Basilar exudate appears as intensely enhanc-
ing areas in to spider leg appearance
basal cistern leading
(Pathognomonic for TBM). Hydrocephalus, infarct, ven-
tricular enlargement.
o PCR: To identi$, mycobacterial RNA or DNA sequence
in CSF; has a sensitivrty of 760/o and specificity
\
Treolment
o 2HRZ|l0 HR plus Prednisolone l-2 mglkgld in 2 div. doses
for 4-6 weeks and then gradual tapering over 2-4 weeks.
r Fits should be controlled by IV or per rectal diazepam.
o In the acutely ill patient, IV fluid should be restricted to
50 ml/kg/d.
Tieatment of cerebral edema, subdural effusion, hydrocephalus
and other complications should also be done.
Prognosis
Prognosis is related to age of child (young children have worse
prognosis) and the stage of disease at which therapy is com-
menced. Complete recovery occurs as follows: Stage I 960/o,
stage II 78o/o, stage IIL 2lo/o. l7o/o of chiid in stage III died
as opposed to 170 in stage II.Most patients in stage III have
permanenr disabiliries including blindness, deafness, paraplegia,
Encephalitis is an acure inflammatory process that afrects brain
tissue and is almost always accompanied by inflammation of the
adjacent meninges, cerebellum; cord involvemenr may accom-
pany. Involvement of the brain without a direct inflammatory
invasion is called encephalopathy and occurs in enreric fever,
shigellosis, following vaccines, high fevers, Reye syndrome,
exanthematous illness, roxins, and drug-induced states.
Types of encephalitis:
o Based on pathological process
cases. r Infectious encephalitis
r Post-infectious/para-infectious/acute disseminated en-
cephalomyelitis (ADEM)
o Autoimmune
Low- o Based on host resistance
o Immunocompetent: HSV-I, YZY, enterovirus, measles,
mumps
i> Immunocompromised: CMV EBV HHV-6, HSV-2
o Based on duration: Acute, Subacute, & Chronic
Encephalopathy: Disruption of brain function that is not
because of a direct structural or inflammatory process or non-
inflammatory diffuse brain dysfunction is termed encepha-
lopathy. Hypoxic, metabolic, toxic, and septic encephalopathy
are importanr causes. Thble 13.9 lists feat'.rres differentiating
encephalitis and encephalopathv.
Acute disseminated encephalomyelitis (ADEM) is an
immune mediated disease of the brain. It usuallv occurs fol-
lowing a viral infection but ma\/ appear follorving vaccination,
bacterial or parasitic infection, or even appear spontaneously.
As it inr.'olves auroimmune demyelination, it is similar to
of 89o/o. multiple sclerosis.
L
 NEUROLOGY

Table13.9: Difference between Encephalitis and Table 13.10: Difference between Encephalitis and Acute
Enceph;ilopathy Disseminated Encephalomyelitis

':.':::,,,,,,r,:',{1;;:ft
ii.:glti*e$,.:ll,
ii,::;.ilr llltl,lCliFi{tll.fg4ltf€
'..,t-r .:,::i.'1.,:ii. lri',,'::'1:,1 ,,. ;'.1.-11:,'1:1'
li,:::r.ii.]:l:lrl:l:l:i'r,. .::::].r:.:'i,i: i r,.:.r'',:l:]rrr.l:ll
Most common age Any age Children
Fever Common Absent/U ncommon
Recent Uncommon Common
Headache and Common Uncommon vaccination
meningism Prodromal illness Occasionally Usually
Depressed mental May fluctuate Steady deterioration FEVCT Common May occur
sl.itus
Visual loss Uncommon Ma1r 66,,ut
Focal neurologic sign Common Uncommon
Spinal cord sign Rare May occur
Types of seizure Ceneralized or focal Ceneralized : :
_i,:. .. .!',
;'::l:r,;:.rtr.i:li:r:l::tl
':::r,:il.:tl l:.1',,,:i l: l:-'.:l:it,i,r t.tr:;t::t'..t:i ,t:'t.tALirrq! . ;:fi ndirt$1 ,':,rr :.-.... .. r:..ii
..l.,
Laboratory findings
Blood Leukocytosis common Leukocytosis
Blood Leukocytosis Leukocytosis occasional
common uncommon
CSF Lyrnphocytic Lymphocytic
CSF Pleor ytosis r onrmon Pleot vto'i.
pleocytosis, elevated pleocytosis, elcvatr:d
un( ommon protein, normal protein, normal
FFC Diffuse slowing and Diffuse slowing glucose, and negative glucose, and negative
tbcal abnormalities cultures. Red blood cultures. Red
ceils seen in herpes blood cells seen !n
MRI Focal abnormalities Often normal
acute hemorrhagic
simplex encephalitis
leukoencepha litis

MRI One or more diffuse Multiple focal areas of

AlthoLrgh it occurs in ali ages, most reported cases are in (T2 image) areas of hyperintensity hyperintensity that are
involves the grey the same and involve
children and adolescents, with the average age around 5-8 white matter of both
matter of both cerebral
years. ,The mortaiity rate may be as high as 570; fuli recovery hemispheres and its hemispheres, basal
is seen in 50-75o/o of cases, while up to 70-90o/o recover with underlying white nralier ganglia, brain stem
some minor residual disability. The average time to recover is and, to a lesser extent cerebellum, and spinal
brain stem cerebellum cord
l-6 nronths.
and spinal cord
ADEM produces multiple in{larnmatory lesions in the
blain and spinal cord, particularly in the white matter.
Usuaily, these are found in the subcortical ar.rd central PATHOGENESIS
white matter and corticai gray-white junction of both
cerebral hemispheres, cerebellum, btainstem, and spinal Encephalitic process begins with entrv of agents with the
cold but periventricular white matter and gray matter host. Entry may occur in various ways, e.g., ,lhrough bite of
of the cortex, thalami anci basal ganglia may also be infected mosquitoes, ticks, animals, or,..contact of infected
involved. secretions with the mLlcolts membrane or,-swings in polluted
When the patient suffers more than one demyeiinating water or treavelling in endemic zone, etc. After gaining entry,
episode, it is called recurrent disseminated encephalomyelitis replication occurs at extraneural site and then reach the CNS
or multiphasic disseminated encephalomyelitis (MDEM). either through hematogenous via choroid plexus or through
Table I 3. 1 0 lists features differentiating encephalitis and acute retrograde axonal transport. After reaching CNS' some agents
dissen.rinated encephalomyelitis. have a predisposition to involve specific areas of brain.

ETIOLOGY CLINICAT FEATURES

o Enterovirus, ccrxsackie virus, echovirus, polior.irus Prodromal stage (up to 1 q'eek) ranges from mild febrile illness
o Herpes viluses associated with headache and n-ralaise to aseptic meningitis
Herpes simplex virus 1 and 2, varicella zoster virus, Epstein- like picture, e.g., fever, nausea, vomiting, neck pain, and
Barre virus, cytomegalo virus photophobia.
r - ---1'-,-1111n61,irus: Henipal-r (e.g., nipah) virus, measles virus, Encephalitic stage (lasts for 7_70 days) reveals alteration of
:- . :r'-r! r-irus, parainlluenza virus level of consciousness ranges from mild iethargy and confusion
. ' :-:.-.: fapanese B encephaiitis virus, equine encepha- to coma. Generalized or focal neurological deficits (e'g., cranial
' - .:. \i. Louis encephalitis virus, West Nile virus nerve palsy) malz [3 stationary, progressive or fluctuating; sei-
. -. :.us-influenzavirus zures are common and unprovoked emotional burst and loss
r -'::. i.rbeila viruses, dengue virus, and HIV. of bowel and bladder control may occur.
 ESSENCE OF PEDIATRICS

Features of raised intracranial pressure include tense parenterally (because of vomiting). In prolonged stares of
bulging fontanels, distended scalp veins, and papilledema with coma, parenteral alimentation with 1070 dextrose saline
evidence of brainstem dysfunction; unchecked brain swelling is indicated; with improvement, feeding with full energy
may lead to herniation ar rentorial hiatus, compression of the milk formula can be started through N-G tube first, then
brain-stem causing deterioration in consciousness, pupillary by mouth. initially, fluid should be restricted (r.e.,20o/o).
abnormalities, ptosis, M nerve palsy, ophthalmoplegia, paralysis o Conr,'ulsion: If status epilepticus, Inj. diazepam 0.3-0.5 mg/kg
of upward gaze, Chyne stoke breathing, hyperventilation, and slow IV repeat at 5 minutes, 10 minutes up to 3 doses
bradycardia. Herniation of cerebellum through the foramen (max. 10-20 mg); rectal route may be used 0.5-0.75 mglkgl
magnum causes distortion and compression of medulla dose. If convulsion not controlled, then Inj. pheny'toin
oblongata with severe disturbance of vital cenrers leading 15-20 mglkg IV 1 ml/kg/min in 50 ml normal saline. If
to respiratory or cardiac arresr. Exrrapyramidal symproms seizure continues, then Inj. phenobarbitone 75-20 mg/kg
are ommon in Japanese B encephalitis, and lateralization to IV at the rate of 1.5 mg/kg/min should be given. In non-srarus
one side with temporal or frontal involvement is common epilepticus cases, oral forms of phenobarbitone, phenytoin, or
in herpes encephalitis. In convalescenr srage, there may be valproate may be used.
recovery with or without sequelae. r Cerebral edema: A number of methods are proposed to
minimize cerebral edema and to diminish the consequences
of cerebraI anoxia:
TABORATORY STUDIES
o IV mannitol given as a 20Vo solution in a dose of 0.25-l
1. Cerebrospinal fluid srudy, cryptococcal antigen, HIV g/kg over a period of 30 minutes. This may be repeated
antibody, toxicology screen, measurement of electrolyte, every 4-6 hourly.
glucose, ammonia, and pH levels o Glycerol 0.5-1 ml/kg diluted with orange juice can be
2. Testing to consider: given by nasogastric tube. This is nontoxic, may be re-
peated every 6 hours.
a) Antibody to arbovirus
b) Polymerase chain reacrion for herpes simplex virus
o IV frusemide l-2 mg/kg or oral acetazolamide 8-30
and enterovirus
mglkgld in l-4 divided doses (max. I g/d) may be used.
c) Antibody to specific pathogens o To prevent gastric hemorrhage, Inj. ranitidin e l-2 mglkgl d
J. Culture: Viral cuitures of nasopharynx, recrum, urine, in 34 divided doses may be used.
and blood (bufl, coar) o To reduce increased body temperature, tepid sponging of
4. Blood culture for bacteria the body and antipyretics (paracetamol 15 mg/kg/dose)
5. Serum testing may be used.

6. Neurodiagnostic resting: Specific treatment:

a) Magnetic resonance imaging o Until a bacterial cause of CNS inflammation is excluded,
b) Electroencephalography parenteral antibiotics (as used in meningitis) should be given.
7. Brain biopsy (when indicated) o \7hen HSV encephalitis is suspected, IV acyclovir should
be given at 10 mg/kg/dose in IV B hourly for 14-21 days,
each dose should be infused over t hour.
coMPUCAT|ONS r In varicella encephalitis, IV acyclovir may be used.
Specific complicarions inciude encephalomyelitis, Guillain-
o In cytomegalovirus encephalitis, gancyclovir l0 mg/kg/d
Barre syndrome, acute transverse myelitis, acute hemiplegia,
IV (over t hour) divided 12 hourly for 14-21 days may
be given.
acute cerebellar ataxia.
o In Rickettsial encephalitis, tetracycline may be used.

TREATMENT
PROGNOSIS
Supportive tfeatm€nt:
HSV: Vithour Rx, 70% mortality; 2.5o/o returns to normal
e Airways should be cleared, oxygen may be needed. Measures
function. Relapse 5o/o, if given for 10 days.
shouid be taken to prevent aspiration pneumonia. Equip-
Better prognosis for HSE:
ments and personnel for handling emergencies, such as
cardiac and respiratory arresr, musr be consrandy at hand. r Young age
Care of eyes, mourh, bowel, bladder, and skin should be , <4 days disease durarion
taken.
,r GCS 6 or less at the time of acyclor.ir administration
o Maintenance of fluid, electrolyte, and acid-base balance. jBE: Mortality 30o/o; 50o/o of sunir.ors have severe neuro- a
I
All fluids, electrolytes, and medications are initially given logical deficits.
I
t
I
,
 t
NEUROLOGY

PREVENTION Clinicql Feqtures

Typical symptoms include fever, headache, psychiatric symp-
A. Environmental control:
toms, seizures, vomiting, focal weakness, memory loss, photo-
1. Personal protection: Avoid exposure to mosquitoes by
phobia, altered mental status, and movement disorders.
using long-sleeved shirt/pant, screened homes, impregnated
Typical signs include alteration of consciousness, fever,
mosquito nets, using insect repellents, instructing children
dysphasia, ataxia, seizures, hemiparesis, cranial nerve palsies,
to play in open, areas away from forest, fringe etc.
visual field loss, and papilledema.
B. By using vaccines: MMR, polio, Japanese encephalitis,
varicella, rabies vaccines, etc. are used effectively nowadays. Diognosis
The Japanese encephalitis vaccine is given in a three dose r CSF analysis: Mononuclear pleocytosis with mildly elevated
series (0.5 ml for 1-3 years of age, 1 ml for >3 years of age) protein and normal or mildly reduced glucose. Because of
subcutaneously; the first two doses are given 1 week apart, and the hemorrhagic nature of the process within the brain
the third dose 30 days later, with booster doses every 2 year' parenchyma, RBC count is usually elevated. HSV is rarely
cultured. PCR analysis of CSF is 94o/o sensitive and 97o/o
Y
, specific for HSV and has replaced brain biopsy. Antigen
HERPES STMPLEX ENCEPHAHTIS (HSE)
I detection by ELISA is 84o/o specific.
I In children beyond neonatal period and in adults, HSE usually o After 5-6 days of illness, a CSF to serum ratio of anti-HSV
t IgG >20 is suggestive of HSE.
is localized to the temporal and frontal lobes and is caused
, e MRI shows hypodensities, usually bilateral in the medial
I by herpes simplex virus rype l. In neonates, however, brain
invoivement is diffuse, and the usual cause is herpes simplex temporal and inferior frontal areas. CT scan also shows
I
similar changes, but is less sensitive.
rype2, which is acquired at the time of delivery.
L

t"
Brain infection is thought to occur primarily by direct neuro-
v
nal transmission of the virus from a peripheral site to the brain
V
via the trigeminal or olfactory nerve. Primary infection occurs in
one-third of the cases, remaining are due to recurrent infections.
t Peripheral lesions (e.g., herpes lablalis) have no relationship
t-
with HSE.
r HSE occurs due to primary infection, reactivation of latent
HSV or re-infection.
C/F: No specific S/S for HSV. Abrupt onset, frontotem-
poral features like acute anemia, recurrent memory loss,
aphasia, personality change, focal seizure are predominant
presentations.
CSF HSV Ab: Helpful diagnostically after 1 week, but
sequential estimation is required.
Serum and CSF Ab against HSV poses interpretation
problem due to polyclonal activation following persistent
previous viral inFection or reactivarion.
a HSV CSF PCR: Sensitivity >90o/o, specificity 100o/o.
a EEG: Abnormal in all cases of HSV encephalitis. The
classical finding is background slowing and focal 2-3 Hz
spikes and waves discharges from temporal lobe, called
PLED (periodic lateralized epileptiform discharges, which
is pathognomonic), occurs mostly between 5-10 days'
PLED may also occur in stroke, subarachnoid hemorrhage,
migraine, and multiple sclerosis.
MRI: Medial temporal lobe involvement, orbital surface of
frontal lobe, insular cortex, and angular gyrus demonstrate
edema and inflammation, which is hyperintense on T,
u'eighted and FLAIR sequence of MRI. Gadolinium (GAD)
contrast enhancement may occur. Diffusion weighted MRI
rD\Xl) sequence is very sensitive in early phase of illness
shorving increased signal intensity.
o EEG shows periodic high-voltage spike and slow-wave activi-
ties in temporal lobe, which is usually suggestive.
o Brain biopsy for isolation of virus.
Treolmenl
o Supportive treatment (discussed under encephalitis).
o Specific treatment: Drug of choice is acyclovir, has inhibi-
tory activity against both HSV-I and HSV-2. Dose is 10
mg/kg/dose (or 500 mg/m'z) IV 8 hourly for 14-21 days,
each dose should be infused over I hour.
Prognosis
The mortality rate in untreated patients is70o/o. Among treated
patients, the mortaliry rute is 20o/o and >50o/o of serious are
left with moderate or of
severe neurological deficits. 5-l0o/o
surviving patients relapse days to weeks after completion of
treatment may be due to reactivation of viral infection or
post-infectious encephalitis.
Preveniion
No effective measures for prevention; person-to-person trans-
mission does not occur. Prophylactic treatment of close contacts
and special isolation precautions are unnecessary.
Acute disseminated encephalomyelitis is a monophasic autoim-
mune demyelinating disease of the central nelvous system that
typically follows a febrile illness (bacterial or viral infection,
 !

ESSENCE OF PEDIATRICS

especially exanrhematous viral illness),*serum administration,
or vgccination., ln'about 50-75o/o of all cases, the clinical onset
of disease is pr6ceded by bacterial or viral infections. Typically,
there is a latency of 7-14 days berween antecedent events (febrile
illness/vaccination) and the onset of neurological symptoms.
CLINICAT FEATURES
The subacutely developing polysymptomaric onset of neuro-
logical deficits arrriburable ro mulriFocal CNS lesioni usually
occurs in ADEM. The common clinical presentations include
: fever, headache, altered level of consciousness, convulsion,
meningeal signs, multifocal neurological deficit like cranial
l nerve palsy, hemiparesis, paraparesis, and ataxia.
flaccid and a sensory level is present, usually in the mid-thoracic
region. Pain, remperarure, and light touch sensarion are affected,
but joint position and vibration sense may be preserved.
o Sphincter disturbances are common.
o Fever and nuchal rigidity are present early in mosr cases.
o The neurologic deficit evolve s for 2-3 days and then plateaus
with flaccidiry gradually changing ro spasricity; examination
reveals weak and flaccid lower limbs with impaired pain,
temperature, and touch sensations. Sensory level is impaired
up to mid thoracic region. The limbs remain faccid for a
few days and gradually become spastic with development
of upper motor neuron signs.
DIAGNOSIS

DIAGNOSIS Diagnostic criteria for idiopathic acute transverse rnyelitis

(ATM):
o The diagnosis ofADEM should be readily considered when- o Development of sensoryJ motor, or autcnornic dysfunction
ever there is a close temporal relation between antecedent
attributable to spinal cord
events and neurological presentations. o Bilateral symproms and signs
o CSF may show pleocytosis and increased albumin. r Inflammation within spinal :r;rd: f CSp ceil, t IgG index,
g Typical MRI leJions: Multiple diffuse asymmetrical subcortical t ltru signal with galoli.ril,- contrasr
white matter lesions exhibiting indistinct borders and enhance- o Progression to nadir: 4 hour-2l days.
ment following gadolinium contrast adminisrrarions. The whire
matter lesions are hypo-intense on T,-weighted and exhibit Investigations:
hyperintensity on Tr,weighted, F[A]R and D\X4 images of
. CSF examination shows moderate lymphocyte pleocytosis
MRI. Sometimes lesions in gray marrers like basal ganglia
and a normal or slightly elevated protein level.
and thalamus at rhe whire to gray mafier junctions have been
reported. The typical MRI lesions usually resolve by 6 months
o CT scanning/MRl reveals mild fusiform swelling in the
afrected region (e.g., Tr).
or may remain unchanged despite clinical improvement
o Virus isolation or seroiogy.

TREATMENT Investigations need to be repeated after 1 week, if negative

initially.
Inj. Methylprednisolone 30 mglkgld (max. 1 g/d) as pulse
therapy diluted with 100 ml of DNS over 1-2 hours daily for TREATMENT
5 days. This pulse therapy is followed by oral prednisolone 1-2
mglkgld for 2-4 weeks followed by tapering over 2 weeks. r Posture, physiotherapy, rnineralizing agenr like calcium
and vit D
Progrosis: Favorable in most (70-90%) cases with minor o Bladder dysfunction: Anti-cholinergic Rr
disabiiity, if any.
o Bowel dysfunction: high fiber diet
o Pain: Tiamadol/Carbamazepine/Nortriptyline/Gabapentin/
Pregabalin
o Spasticity: Beclofen/Tizanadin/Diazepam/Botox
o Inj. Methylprednisolone 30 mg/kg as single daily dose diluted
Tiansverse myelitis (TM) is characterized by abrupt onset of
with 100-200 ml of fluid for 5 days t piasma exchange. Oral
progressive weakness and sensory disturbances in the lower
prednisolone to be started from 6.r'day at a dose of l-1.5
extremities. A history of a preceding viral infection accompanied
mg/kg for another 3-6 weela depending on response.
by fever and malaise is present in most cases.
Agents responsible are Epstein-Barre virus, herpes, influenza, Other treatment oprions include:
rubelia, mumps, and varicella viruses. o Inj. Dexamerhasone 200 mg/d, 3_5 days or 5 mg/kg as
single daily dose for 5 days
CL;NICAL FEATURES o Pulse IV Cyciophosphamide 500-1000 mglm2
o Azathioprine 150-200 mg/d
o Low back or abdominal pain and paresthesias of the legs are o MTX l5-20 mg/wk
prominent in the early stages. The leg muscles are weak and o Mycophenolate2-3 gld
\
I

PROGNOSIS
Recovery begins within 6 months, continue till 2 years, but
vast majority have some neurodeficit within 8 weeks.
a One-third recover with little or no sequelae.
a One-third with moderate disability.
a One-third with severe disabilitv.
Acute spinal compression is a common neurological emergency.
a The early stage of damage is reversible, but severely damaged
r neurons do not recover; hence, early diagnosis and treatment
r
r are important.
r
CAUSES
r Vertebral (80o/o): Tiauma (extradural), TB, intervertebral
disc prolapsed.
Meninges (intradural extrameddllary) (l5o/o)z Tumor
(meningioma, neurofibroma, lymphoma, leukemia)
Spinal cord (intradural intramedullary) (5olo): Tirmor
(glioma, ependymoma).
CtINICAI FEATURES
o Pain: Localized over the spine or a narrow distribution, which
may be aggravated by coughing, sneezing, or straining.
o Sensory: Paresthesia, numbness, or cold sensation, espe-
cially in the lower limbs that spreads proximaliy to a level
on the trunk.
. Motor: \Teakness, heaviness, or stiffness of the limbs, most
commonly in the legs.
o Sphincters: Urgency or hesitancy of micturition,'leading
eventually to urinary retention.
Signs of spinal cord compression:
Ceruical, aboae C5:
o Upper motor neuron sign and sensory loss in all four limbs
o Diaphragm weakness (Phrenic nerve)
Ceruical, C5 to T1:
o Lower motor neuron signs and segmental sensory loss in
the arms; upper motor neuron signs in legs
o Respiratory (intercostal) muscle weakness
Thoracic cord:
o Spastic paraplegia with a sensory levei on the trunk
Contu medull.aris:
o Loss of sacral sensation and extensor plantar responses
C-cda equina:
r - : -:: motof neUfOn Sign
NEUROLOGY
INVESTIGATIONS
Plain x-ray of spine, chest x-ray, MRI of spine, CSF study,
Serum B,r.
TREATMENT
Tieatment depends on underlying lesions:
o Benign tumors should be excised.
e Surgical decompression or radiotherapy for extradural tumor.
o Anti-TB chemotherapy for tuberculous lesion t surgical
treatment.
o Specialized neurosurgical treatment for traumatic lesions.
Guillain-Barre syndrome (GBS) is a post infectious polyneuropa-
thy that causes demyelination in mainly motor but sometimes
sensory n€rves also. This syndrome usually develops 1-4 weeks after
viral infection and rarely follows surgery and immunization.
ETIOLOGY
Occurs commonly with gastrointestinal infection (especially
Campylobactor jejuni) or respiratory tract infection (Mycoplasma
pneumoniae).
CTINICAL FEATURES
In case of acute onset, leg pain and stiffness are common,
transiently bladder may be involved.
The characteristic clinical features are symmetrical muscle
weakness; weakness begins usually in the lower extremities
and progressively involves the trunk, upper limbs, and
finally the bulbar muscles (50%o), a pattern formerly known
as Laundry ascending paralysis. The onset is gradual and
progresses over days or weeks.
Respiratory insu{ficiency may result. Dyspnea and facial
weakness are impending signs of respiratory failure.
Autonomic manifestations are cardiac arrhythmia, fluctua-
tion ofblood pressure and heart rate, postural hypotension,
profound bradycardia.
On examination, profound muscle weakness and loss of
tendon reflexes occur. The Miller-Fisher syndrome-consists
of acute external ophthalmoplegia, ataxia, and areflexia-
may also be observed.
coMPHCATTONS
a Respiratory paraiysis
o Urinary incontinence or urinary retention
a Aspiration pneumonia
a Chronic relapsing polyradiculoneuropathy and chronic unre-
mitting polyradiculoneuropathy.
Relapse
 q

ESSENCE OF PEDIATRICS

INVESTIGATIONS Table 13.1 1 lists the differentiating fearures of poliomyelitis,

CSF study: Usually shows albumino-cytological dissocia-
tion. The protein concentration becomes elevated in 75o/o
cases;this finding may nor appear unril 1-2 week after the
onset of clinical manifestations. Protein may be as high as
400-500 mg/dl, although the cell count remains normal
or only slightly raised.
a Motor nerve conduction velocity-reduced.
a Electromyogram (EMG): Shows evidence of acute dener-
vation of muscie.
a Serum creatine phophokinase level mildly elevated or normal.
a Sural nerve biopsy shows segmental demyelination, focal
-Wallerian
inflammation, and degeneration, which is con-
firmatory.
o Stool culture and serological test for Campylobaaor jejuni.
GBS, traumatic neuritis, and transverse myelitis.
TREATMENT
1. Mainly supportive with physiotherapy to assist chest
drainage and prevention of contractures (to prevent
foot drop) with good sLrpportive treatment; >90o/o of
children will recover. Respiratory insufficiency secondary
to paralysis of intercostal muscles is the most serous
complication. Involvement of bulbar muscles and
respiratory insuliciency may need tracheal intubation
and assisted ventilation until recovery. Recovery has been
teported to occur after >8 months of complete vcntilatory
suppoft.

Table 13.11: Features Differentiating CBS, traumatic and transverse mvelitis

Etiology Polio virus type l, ll Likely delayed Trauma Usually unknown;

and lll and other
enterovir,uses
Onset of paralysis 24 to 48 hours
Fever at onset High. Always present at
onset of flaccid paralysis,
disappears later
Flaccid paralysis Acute, asymmetrical,
principally proximal
Muscle tone Reduced or absent in
the affected limb
Deep tendon reflexes Decreased or absent
Sensation Severe myalgia, backache
Cranial nerve Only when bulbar/
involvement bulbospinal involvement
is present
Respiratory Only when bulbar/
insufficiency bulbospinal involvement
is present
Autonomic signs Dysautonomia
and symptoms
Bladder dysfunction Absent
CSF: WBCs High WBCs
Protein Normal or slightly elevated
Nerve conduction Anterior horn cell
values at 3rd week disease (normal during
the first 2 weeks)
EMG at 3 weeks Abnormal
Sequelae at 3 months Severe, asymmetrical
and up to a year atrophy, skeletal
deformities developing later
hypersensitivity
immunologic
Few hours to 1 0 days
Not common
Cenerally acute,
symmetrical and distal
Clobal hypotonia
Clobally absent
Cramps, tingling
hyperesthesia of palms
and soles
Often present
Miller-Fisher
syndrome
ln severe cases
Frequent blood pressure
alterations, sweati ng,
blushing and body
temperature f luctuations
Transient
<10 WBCs
High
Abnormal demyel ination
Normal, depending
upon recovery
Symmetrical atrophy of
distal muscles
multiple viruses
Few hours to four da1,s Few hours to 4 days
Con-rmonly present Rarely present
before, during, and
after f laccid paralysis
Asymmetrical, acute, and AcLrte symmetrical usually
affecting only one linrb involving lower limbs
Reduced or absent in Hypotonia in lower Iimbs
the afiected Iimb
Decreased to absent Absent in lower limbs
early, hyper-ref lexia late
Pain in gluteus, Anesthesia of lower limbs
hypothermia with sensory level
Absent Absent
Absent Absent
Rare Hypothermia in afiected
limb
Never Present
Normal Normal
Normal Normal or sllghtly elevated
Abnormal, suggestive Normal or abnormal
of axonal damage No diagnostic value
Normal, depending Normal
upon recovery
Moderate atrophy, only in Flacc:C c : =. :.. atrophy
affected lower limb aier r =.--.-

t
 NEUROLOGY

2. Specific treatment: o Inflammatorydisorders

a) I\4G (Humaglobin): A total of 2 glkg to be given r Meningitis: Viral, bacterial, tuberculosis
through IV infusion for 3_5 days as a single daily o Systemic infection: Local head and neck infections
dose. o Autoimmune diseases: Systemic lupus erythematosus,
b) Plasmapheresis juvenile rheumatoid arthritis

3. Rehabilitation o Metabolic disease associated with stroke: Homocysti-

nuria
o Intracerebral vascular processes: Ruptured aneurysm,
PROGNOSIS arteriovenous malformation, moyamoya disease, migraine
(within 6-12 months). headache, Sturge-\7eber syndrome
Full recovery: Approximately 85o/o
Permanent neurological deficit: Approximately 15%' The clini-
o Thauma and other external causes: Child abuse, head
cal course is benign, and spontaneous recovery begins within trauma/neck trauma, oral trauma
2-3 weeks. Most patients regain firll muscular strength, although
some are left with residual weakness. A small proportion Arleriol Thrombosis/Embolism
I

I
(>10% cases) also relapse or recur. Less than 5o/o of cases die
Arterial thrombosis and embolism may involve major cerebral
either from autonomic involvement with cardiac arrhythmias,
arteries (internal carotid or anterior, middle, and posterior
arterial pressure instability, respiratory insufficiency, or from
t cerebral artery occlusion) or smaller cerebrai arteries.
complication of assisted ventilation.
I Thrombosis of the internal carotid artery may result from
blunt trauma to the posterior pharynx due to fall on a pencil
, in the child's mollth. The iniury produces a tear in the intima
L

,
of the vessel wall; this may lead to formation of a dissecting
a aneurysm. Cerebral symptoms result from shedding of emboli
,-
Neurological deficit ofcerebrovascular cause that persists beyond from the thrombus. The onset of symptoms may be delayed
F 24 hours or is interrupted by death within 24 hours. The for up to 24 hour after the accident, but progressive flaccid
t pediatric causes of r,rok. digtinctive and are established
".. hemipiegia, lethargy, and aphasia may occur, if the dominant
r-
in approximately 75o/o of cases. Types of stroke include arte- hemisphere is involved. Focal motor seizures are a common
rial and.venous thrombosis,.intracranial hemorrhage, arterial complicarion.
embolism, and miscellaneous conditions. In retropharyngeal abscess, arterial thrombosis results from
inflammation of the intima and clinical pictures are same. A
CAUSES cerebral angiogram or MRl/magnetic resonance angiography
(MRA) typically demonstrates occlusion of the internal carotid
o Cardiac disease artery and CT/MRI scan shows a hypodense lesion outlining
,r Congenital the area of infarction.
Aortic stenosis Embolization of cerebral vessels may also produce acute
- Mitral stenosis hemiparesis but is rare in children. Cardiac abnormalities are
- Ventricular septal defect the most common cause of thromboembolic stroke in chil-
- Patent ductus arteriosus dren. Cardiac causes include arrhythmias (particularly atrial
- Cyanotic congenital heart disease involving right to fibrillation), mle<oma, paradoxical emboli through a patent
- left shunt foramen ovale, and bacterial endocarditis that results in mycotic
aneurysm. Air emboli may complicate surgery, and fat emboli
,; Acquired
occur with fracture of long bones. Septic emboli may seed the
Endocarditis (bacterial)
- Kawasaki diseasc
cerebral vessels and evolve into an area of cerebritis leading to
-
H
a cerebral abscess.
Cardiomyopathy
- Atrial mlxoma
Cyanotic congenital heart disease in children y6unger than
- 2 year may cause thrombosis, particularly of the middle cere-
Arrhythmia
- bral artery. These patients are particularly vulnerable when the
o Hematologic abnormalities oxygen saturation is significantiy decreased together with a viral
, Hemoglobinopathies illness or dehydration. Cardiac catheterization and complex
o Sickle cell (SS) disease cardiac surgery may also cause thromboemboiism. Echocardio-
r Polycythemia gram must be done if cardiac cause is suspected.
r Leukemia/lymphoma Basal arterial occlusion with telangiectasia or moyamoya
r Thrombocytopenia (puff of smoke) disease has a characteristic angiography. It
Disorders of coagulation is common in girls and often presents with headache and
 !

ESSENCE OF PEDIATRICS

biiateral upper moror neuron signs. It may also present with aneurysm and coarctarion of aorta and bilateral polycystic
chorea. The prognosis for recovery is poor, with intermittent kidney disease. Most ruptured aneurysms bleed into the sub-
episodes of TIA coupled with progressive neurological signs arachnoid space and causes inrense headache, nuchal rigidiry
and severe disability. and coma; intracerebral hemorrhage and progressive hemiparesis
may also occur.
Venous Thrombosis Hematologic disorders, particularly thrombocytopenic
The symptoms and signs due ro venous thrombosis may evolve
purpura and hemophilia, and rrauma can also produce intra-
cranial hemorrhage.
over days and in neonares are characterrzedby diffuse neurologic
signs and seizures, whereas focal neurologic signs are more
A contrast CT scan orMRI is useful for identifying arte-
riovenous malformations and cerebral aneurysm. However,
prominent in children. Dilated scalp veins, a bulging anterior
four-vessel cerebral angiography is the study of choice.
fontanel, and symptoms and signs of raised intracranial pressure
may be present. Venous sinus thrombosis may be subdivided
into septic and aseptic causes. cllNtcAt tocAUzATtoN
Septic causes ofvenous sinus thrombosis include encephalitis
and bacterial meningitis. Hemiplegia is a relatively common 1. Cortical
complication of bacterial meningitis due to thrombosis of r Convulsion
the superficial cortical and deep penetraring veins. Additional r Unconsciousness
causes include otiris media and mastoiditis with involvement o Contralateral hemiplegia-flaccid, nor dense
of dural vessels, and retrograde orbital infections producing r Aphasia (dominant hemisphere)
cavernous sinus thrombosis. o Loss ofcortical sensation
Aseptic causes include severe dehydration in infancy, which o Face and hemiplegia-same side
may cause thrombosis of superior sagittal sinus and the superfi-
cial cortical veins due to hyperviscosity and sludging of blood.
2. Corona radiara
Conditions resulting in hypercoagulability, cyanotic congenital o Convulsion-uncommon
heart diseases and leukemic infiltrates of cerebral veins are r Contralateral hemiplegia
additional causes. o Other features similar to cortical involvement
3. Internal capsule
I ntrqcro niol Hemorrho ge o Contralateral hemiplegia often with sensory loss
Intracranial hemorrhage may occur in the subarachnoid space, o Lower facial weakness on opposite site
or bleeding may be primarily located in the parenchyma of 4. Mid-brain
the brain. Subarachnoid bleeding is characterized by severe
headache, nuchal rigidity, and progressive loss ofconsciousness; e Weber syndrome
intracerebral bleeding is characterized by focal neurological Ipsilateral III nerve palsy
signs and seizures.
- Contralateralhemiplegia
-
Arteriovenous malformations produce abnormal shunting of e Benedicts syndrome
blood, causing an expansion ofvessels and a space-occupying Ipsilateral III nerve palsy
effect or ruprure of a vein and intracerebral bleeding. - Contralateralhemiplegia
Arteriovenous malformations are rypically located in the cerebral - Tiemors (red nucleus affection)
hemisphere, but they may be situated in the cerebellum, 5. Pons-
brain stem, or spiral cord. Usually, the malformation remains
asymptomatic throughout the life, but rupture and bleeding
o Millard-Gubler syndrome
can occur at any age. Patients give history of seizures and Ipsilateral VI and \4I nerve palsy
- Crossed hemiplegia
headaches. On auscultation of the skull, a high pitch bruit
is heard in 50o/o of cases. Rupture causes a severe headache, 6. Medulia
vomiting, nuchal rigidiry progressive hemiparesis, and a focal Contralareralhemiplegia
- Ipsilateral involvement several cranial nerves
or generalized seizure. An arteriovenous malformation of the -
vein of Galen during infancy can cause congestive cardiac 7. Spinal cord (medulla up to 5'h spinal segment)
failure and is associated with poor prognosis. Ipsilateralhemiplegia
- No cranial nerve involvement
\
Cerebrai aneurysms in children tend to be iarge and are -
located at the aortic bifurcation or on the anterior and poste- Fig. 73.5 depicts localization of lesion site br- inspection. and a
rior cerebral arteries. There is an association between cerebral a
Table 13.72 lists clinical features rvith respecr to lesion site.
t
I
I
i
lr
 NEUROLOGY

RADTOTOGTCAT TOCAHZATTON ,r Coagulation profile

,r Total homocysteine
.r Fasting cholesterol, triglycerides, and lipoprotein levels
Infection screen, including Mycoplasma, Chlamydia and
Helicobacter,
a X-ray, ECG, echocardiogram
a CSF study
a Neuroimaging of brain and vessels:
o Magnetic resonance imaging (MRI) and MR angiogra-
phy (MRA) to:
, Exclude hemorrhage
- Define extent and territory of infarct
- Define vascular anatomy of circle of \Willis and neck
- vessels

o Computed tomography (CT) scan to exclude hemor-

rhage
INVESTIGATIONS ,r EEG
o o Isotope brain scan
Hematological tests: Full blood count, diiferential white cell
count, and erythrocyte sedimentation rate
TREATMENT
o Iron, folate, and hemoglobin electrophoresis
o Protein S and protein C Acute management
o Keep temperarure between 36.5"C and 37'C
Look at lace & extremity involvement ffi
Lr Tieat acute seizures
For hemorrhagic stroke
,r Immediate referral to a center with neurosurgical facili-
Hemiplegia +
ties (?for drainage)
ipsilateralUMN
facial palsy For cerebellar stroke presenting in coma
o Referral to a center with neurosurgical facilities (for
drainage hydrocephalus or decompression)

For large middle cerebral artery territory lesions present-

ing in coma
o Referral to a center with neurosurgical facilities (for de-
compression)

For stroke in sickle cell disease

Contralateral Contralateral
Upper pons &
cortico-subcortical paracentral
$
ffi
o Exchange transfusion
above
#ew#
lobule ffi For ischemic stroke occurring in hospital and imaged
within 3 hours
Fig. 13.6: Localization of site of lesion by inspection.
r Consider intravenous tissue plasminogen activator

Table 13.12: Clinical Features and Site of Lesion

lpsilateral to lesion lpsilateral UMN facial palsy Upper pons and above

Contralateral to lesion lpsilateral to lesion Brain slem lesion

Hemiplegia Proportionate lnternal capsule

Hemiplegia Disproporlionate; I Facio-brat hial Cortico-subcortical: Contralateral

Hemiplegia Disproportionate: t Crural Paracentral lobule; Contralateral

Paraplegia Parasagittal infarct/meningioma/cervicothoracic myelopathy,rbilateral lumbar plexopathies

Sirgle limb weakness ACA infarcVparacentral lobule (PCL) mass lesion/Ertramedullary spinal cord lesion (Brown Sequard syndrome)
ESSENCE OF PEDIATRICS

For venous sinus thrombosis, extracranial arterial dis-

section, and known prothrombotic disorder ASSESSMENT

'r Heparin acutely t. History: A detailed history of trauma, toxic ingestion,

,; Warfarin for3-5 monrhs
For strokes secondary to other mechanisms
r Early prophylaxis with low-dose aspirin (1 mg/kg)
For all
o Eariy rehabilitation by team comprising nursing staff,
physiotherapist, occupational therapist, speech therapist,
and psychologist.
PREVENTION OF RECURRENCE
o For sickle cell disease: Regular transfusion (4-6 weekly)
to keep hemoglobin S <20o/o.
o For moyamoya: Consider revascularization, particularly if
transient ischemic attacks or cognitive decline.
For homozygotes for the thermolabile methylene
tetrahydrofolate reductase gene: B complex vitamin
supplementation.
For those with an important prothrombotic disorder or
extracranial arterial dissection: Consider warfarin (discuss
with hematologist in individual case).
For others with stroke in a vascular distribution and/or
cerebrovascular disease: Low-dose aspirin 5 mg/kg (max.
300 mg).
Coma is the result of
inadequate interaction between the
cerebial hemispheres and the reticular activaring system of the
diencephalon, mid-brain, and pons.
infection, fasting, .drug use, diabetes, seizure, or other
neurological disorders should be taken.
2. Examination: Heart rate, BP, temperarure, respiratory
pattern, level ofconsciousness, pupillary size and refexes,
motor responses, fundoscopy and focal neurological signs
should be assessed. Clinical presenrarion depends on rhe
part of the brain involved.
, ") If cerebral hemispheres and basal ganglia are
inaolaed: Presents with confusion or delirium into
a normal breathing partern, normal or small-sized
feactive pupils, roving eye movements, and sponrane-
ous movements of the extremities.
b) If Diencephalon is inaolaed: Prese nrs with stupor
and unresponsiveness, periodic and Cheyne-
Stokes breathing, small reactive pupils, doll's eye
movements, oculovestibular reflexes, and decorticate
posturing.
c) If mid-brain and upper pons are inaolaed:
Presentswith hyperventilation, moderate pupillary
dilatation, decon jugate gaze with loss of
oculocephalic and oculovestibular reflexes and
decerebrate posturing.
d) Iflouter pons and medullary leael are inaolued:
Presents into a shallow and irregular breathing pattern,
fixed and diiated pupils, absent eye movemenrs and
flaccid paralysis.
For determining the severity of coma, Glasgow coma scale
along with modified coma scale for infants and children are
widely used (Thble 13.13).
Besides specific diagnostic investigations such as CT scan,
MRI, LB EEG, toxicology, metabolic screening, x,ray, erc., rhe

Table 13.13: Clasgow Coma Scale

Eye opening
Spontaneous Spontd neotrs Spontaneous 4
lo verbal commanri To speech To speech 3
To pain To pain ro parn 2
No response No response No response 1

Motor
Follows commands
Localizes pain
Withdraws to pain
Abnormal flexion
Abnormal extension
No response
Normal spontaneous movements Obeys verbal commands 6
Withdraws to touch localizes pain 5
Withdraws to pain Withdraws to pain 4
Abnormal flexion to pain r6lsc6rtl.r1e poslure) Abnormal flexion (decorticate posture) 3
Abnormal erlension to pain'decerebrate posturer Abnormal extension to pain (decerebrate posture) 2
No response No response 1

Verbal
Oriented and converses Coos, babbles Smiles, oriented to sound, follorvs objects, interacts 5
Confused lrritable Consolahle 4
I
Inappropriate words Cries to pain lnconsistently consolable Moaning
Nonspecific sounds Moans to pain lnconsolated/irritable and restless
3
2
\
No response No response No response .l
t
I
t
,

following investigations and follow-up management should
be considered:
o 4-hourly blood gases
o Continuous monitoring of O, saturation with pulse oximetry
r Bedside measurement of blood glucose level by Dextrostix/
BM stix 4 hourly
o Blood osmolality (preferably B hourly)
o Calcium and phosphate levels (preferable 12 hourly)
o ECG and if possible, EEG
r Blood pressure, preferably 2 hourly
o Coagulation profile, at least once, then whenever needed
o Temperature 2 hourly
o Strict intake and output chart
o Blood count and hemoglobin levels
o Chest x-ray
o Urea and electrolltes (rwice daily)
o Liver function tests
For monitoring coma state, followings'should be monitored:
o Glasgow (modified) coma scale
o Ocular responses (pupils, external ocular movements, etc.)
o Bulbar refexes
o Temperature, pulse rate, respiratory rate, and blood pressure.
TREATMENT
Regardless of the etiology of coma, the management should
be done as follows:
o teatment of infection if any
o Control of seizures
r Detection and treatment of raised ICP
o Maintenance of cerebral blood fow
o Maintenance of cerebral metabolism
o Maintenance of homeostasis
o Removal of circulating toxin
o Adequate fluid, electroly'tes, and calorie should be ensured.
Eyes, mouth, bladder, bowel and skin care should be taken.
Hydrocephalus results from an increased volume of CSF due to
either increased production, obstruction, or impaired absorp-
tion resulting in pathological increase in ventricular size. The
ventricles not only become dilated, but the pressure is usually
increased. All large ventricles are not always hydrocephalus.
In hydrocephalus ex vacuo, the frontal horn is not rounded,
no periventricular ooze, there is features of cortical atrophy.
ctAsstFtcATtoN
e Non-communicating or obstructive hydrocephalus:
Hvdrocephalus resulting from obstruction within the
NEUROLOGY
ventricular system. The common sites of obstruction are
before the outlet offourth ventricle, the aqueduct ofSylvius
or foramen of Monro. There is excess of CSF in ventricular
system.
Aqueductal stenosis (70Vo) (XL R, IVH, meningitis),
midline brain tumors, vein of Galen malformation, cerebel-
lar tumors, Dandy-Walker malformation, Arnold Chiary
malformations.
Communicating or non-obsttuctive hydrocephalus:
Hydrocephalus resulting from obstruction at the arachnoid
yilli or in the basal cisterns. There is an increase in the
ventricular volume and the subarachnoid spaces of cranium
and spine. There is defective absorption.
Occurs in meningitis-tubercular (commonest), pyo-
genic, congenital infections. Secondary to subarachnoid
hemorrhage.
Acquired: Overproduction of CSF-choroid plexus papil-
loma, IVH, craniosynostosis, achondroplasia, neoplasms.
PATHOPHYSIOLOGY
CSF is secreted by the choroid plexus in the lateral ventricles by
plasma uitrafiltration and active secretion. The total volume of
CSF is about 50 ml in an infant to about 100 ml in children.
After secretion, CSF circulates through 4'l'ventricle and reaches
the basal cistern via the foramina Luschka and Magendie and
subarachnoid spaces at the base and cortex. The CSF is absorbed
mainly through the arachnoid villi.
The dilatation of the ventricles in response to high CSF
pressure is termed as active or progressive hydrocephalus.
Finally, when the pressure returns to normai levels with severely
dilated ventricles, a state of arrest, is termed as compensated
or arrested hydrocephalus.
CtINICAt FEATURES
Eorly Onsel (0-2 Yeor)
o Congenital hydrocephalus due to aqueductal stenosis is the
commonest cause, and Chiary malformation type II is the
next in frequency.
The clinical presentation of hydrocephalus depends on the
age ofonset, nature ofobstructive lesion, the duration and
the rate of rise of intracranial pressure.
Progressive infantile hydrocephalus (about 5070 cases may
remain asymptomatic): Headache or irritability, vomiting,
anorexia, drowsiness, or lethargy.
Inappropriately increasing occipito-frontal circumference
(OFC) (approximately 75o/o); wide open, bulging and often
tense anterior fontanel; there is often characteristic scalp
vein distension.
"Setting sun" eye sign (loss of upward gaze due to IV nerve
paralysis caused by the pressure effect) and broad forehead
are characteristic signs with ptosis and nystagmus. Neck
retraction or rigidiry brisk tendon reflexes, spasticiry ankle
ESSENCE OF PEDIATRICS
clonus, and Babinsky signs may be present. Splayed sutures
may be palpated or indication by "cracked pot" or Macewen
sign on percussion. Neurogenic stridor and decerebration
are late signs.
r Progressive increase in OFC > I crn/rvk an infant.
oFC (cm)r
o Head index -
BVing
(Normal: 10 t 1, consranr from birth to 18 mo).
o Chiari malformation type I: Hydrocephaius insignificant,
occurs beyond adolescence, signs of brain stem dispiace-
ment, long tract signs, neck stiffness.
o Chiari type II: Caudal displacement of cerebellar tonsils,
progressive hydrocephalus, myelomeningocele, medulla/
pons displaced.
o Dandy*Walher malformation: Cyxic enlargement of ventricle
Il hydrocephalus (90%).
Lqle Onsel (2-10 yeor)
Papilledema, headache, vomiting, increasing head circumference
occurs only in 600/o cases. McEwen sign (crack por resonance on
percussion of the skull), pyramidal signs (40%), psychomotor
retardation, gait anomaly (3Ooto\, epilepsy (20Vo).
Arresled Hydrocephqlus (Long Sionding
Coses)
A large head with wide fontanel may be the only presenra,
tion. Features of ataxic and spastic cerebral palsy, precocious
puberty, mental retardation, and specific learning problem may
be present according to the prior cerebral insult. Differential
diagnoses include macrocephaly, megalencephaly associated
with CNS diseases, subdural effusion, achondroplasia, familial
large heads.
INVESTIGATIONS
r Serial measurements of OFC to assess ventricular size .
r Transillumination of the skull: Positive with massive ven-
tricular dilatation.
o Ophthalmoscopy: Papilledema may be seen in oider children
due to raised intracranial pressure. It is rare in infants and
young children.
X-ray skull may show separation of sutures, erosion of the
posterior clinoid processes in older child, and an increase in
convolutional markings known as "beaten silver" appearance
with long-standing increased intracranial pressure.
Cranial ultrasound in infant: USG is the screening investiga-
tion with open AF. CT is the most important to identify
the specific cause. MRI not indicated usually, except for
associated maiformation in the posterior fossa, or white
matter lesions.
Psychomotor assessment; To evaluate performance in motor,
adaptive, language, personai social domains.
coMPHCAT|ONS
Raised intracranial pressure, cerebral palsir, hemiplegia, epilepsy,
mental retardation.
TREATMENT
Goal of therapy includes decreasing ICP to safe limits preserv-
ing cortical tissue.
Congenital: Mild/arrested hydrocephalus does nor need
surgery, may be managed medically. Moderate hydrocephalus
should be operated early to improve neuro motor olrtcome.
Severe hydrocephalus with corrical mantle size <1 cm,
or hydrocephalus with associated malformations, surgely
generally not indicared because of pool ourcome . Medical
management may be su{licient. Aqueductal srenosis should
be operated early, as result is good. Third ventriculostomy
is the preferred surgical inrervention, iffeasible, as it avoids
shunt complications.
Post TBM hydrocephalus: In acure srage, surgerv is nor
generally indicated because in many cases it may resolve with
anti-TB chemotherapy and steroids. Raised ICP shoud be
managed medically. Indications for surgery are papilledema,
no improvement in sensorium after 7-i0 days of anti-TB
drugs and steroids, persistent decerebration, imminant coning.
Hydrocephalus with pyogenic meningitis: It is associated
with ventriculomegaly in 30o/o cases, usually reversible, does
not require shunt. Manage raised ICP medicall,v.
o Medical:
.r Mannitol 20o/o: 5 ml/kg initially then 2 rnlikg (6 hourly)
for 48 hours (for urgent reduction of ICP).
'r Carbolic anhydrase inhibitor: Acetazolamide 50-100
mg/kg/d in 3 doses can reduce CSF production by 50%.
Adverse effect, sucl.r as metabolic acidosis, shoLrld be
monitored.
r Oral glycerol: 1 ml/kg/dose 8 hourly.
r Furosemide: Redr.rces CSF production r-rp to 50o/o.
o Surgical:
'> Procedure of choice is ventriculoperitoneal shunt. Ven-
triculope ritoneal shunt is associated with lcsser con-r-
plications as compared to ventriculoatrial shunt. Many
types ofvalves are used for shunt operarion, e.g., Silastic
shunt with one way low pressure valve, Indian valve (Up-
adhya).
Indications for shunt: Papilledema or periventricular
- ooze on fundoscopy and CT scan, respectivell'; cortical
mantle <2-2.5 cm on initial neuroimaging (especially
in infancy); progressive thinning ol cortical mantie
despite medical trearment.
r Endoscopic third ventriculostomy: Ir needs evaluation in
childlen.
 NEUROLOGY

-Note head size, associated

malformation, syndromes
-Etiology

To assess:
-Ventriculomegaly
-Cortical mantle
-Periventricular
ooze
Clinical evaluation
development, neurological

Moderate Massive
Periventricular ooze, Papilledema Optic atrophy,
Delayed milestones Cortical mantle < 1cm

>

!
F

I
_l*sii
I

I
I
MedicalTT
poor outcome
lmproved Follow-up ffi

Continue medical
therapy

Fig. 13.7: Algorithmic approach to the management o{ hydrocephalus.

Fig. 13.7 depicts algorithmic approach to the management of
hydrocephalus.
FROGNOSIS
Hydrocephalus often gets arrested spontaneously; surgery is
not needed in all. For untreated hydrocephalus, approximately
50%o survive and of survivors,20-30o/o have normal intelligence
and 20-30o/o have motor or other handicap.
Virtually any organ can be involved, but four basic involve-
ments are neurological, hepatic, cardiac, and storage disor-
ders. Genelally, these are progressive in nature. On substrate
basis, amino acids, organic acids, fatty acids, or carbohydrate
metabolisms are affected. The common mode of presentation
includes acute, chronic, or acute on chronic encephalopathy.
The manifestations mimic many common pediatric illnesses iike
,lrypoxic ischemic encephalopathy, neonatal sepsisrxnexplained
metabolic derangements, etc. So a high index of suspicion is
necessary to diagnose NMD in a specific situation.

'When to suspect NMD:

Neurometabolic disorders (NMD) are inborn errors of metabo- a Parental consanguinity

lism with neurological manifestations. Mostly these are auto- a Suggestive family history (sibs/family member affected,
somal recessive and less commonly X linked/mitochondrial/ sibs death)
autosomal dominant. NMDs difrer tremendously in pathology, H/O intermittent vomiting, lethargy, convulsion: Encepha-
age at onset, clinical presentation, and diagnostic approaches. lopathy like presentation
 ESSENCE OF PEDIATRICS

o Failure to thrive, unusuai body odor TREATMENT

o lJnusual reaction to minor infection: Acute encephalopathy
o Recurrent/chronic encephalopathy For acurely ill child:
o Plateau in milestone acquisition or regression of milestone o Stop oral feeds, process/store blood and r-rrine samples before
of developmenr stopping feeds
Investigations: o Start parenteral fluids (appropriate for age)
o Correct dehydration, acidosis, and dyselectrolytemias
r Blood glucose, calcium, LF l, and blood counts o Start specific therapy if any (e.g., oral sodium benzoate for
o ljrine metabolic scleen hyperammonemic states)
o Four simple tests: o Monitor and maintain vitals, BS, pH, electrolytes
o Arterial blood gases (ABG) o Tiansler to a tertiary center
,r Arterial lactate (4 hour fasting sample)
r Blood ammonia (analyzed immediately)
PREVENTION
r Urine ketones
r Plasma and urinary aminoacidogram o Genetic counseling
o Amino acids in CSF r Prenatal diagnosis
o Urine for organic acids
o Urine for orotic acid
r Neuroimaging and neurophysiological studies
o Enzyme estimation, tissue diagnosis, molecular/genetic Progressive ioss (regression) of acquired milestone of develop-
testlng ment after a period of normal development.
o Specific tests: HPLC, TMS, GCMS
When to suspect degenerative brain disorders (DBD):
Classification: 1. Grajt matter DBD: Cognit'ion, seizure, behavior, per-
Refer Tables 13.14 and 13.15 and Fig. [or classification sonality change, dementia, speech and language disorder.
of NMDs.

Table 13.14: Urine {or Metabolic Screen to Classify NMD

Phenylketonuria
Galactosemia
Organic aciduria
Amino aciduria
Homocystinuria
Mucopolysact haridoses
DN PH, Din itrophenl,lhl,drazine; tt1PS, rrucopolysaccharidosis

Table '13.15: Six Tests for Classification of NMD

++ N/J Mapple syrup urine disease

+++ ++ Ji. Organic acidurias
+ + N/J Lactic acidosis
N Urea cvcle disorders

N NKH

=
t JJ FAOD
++ ++ N rvliio. ETC disorder
I

3 \
 NEUROLOGY

Suspected neurometabolic disorder

Blood pH & CO, Blood pH & CO,

Normal
Ketosis -ve

Urea cycle defect Ketosis -ve $ Ketosis with or without

I lactic acidosis
tI

Estimate plasma citruline Fatty acid oxidation defect

Hish Low
$ ffi

\..
Citrulinemia ffi
orc,
CPS, ASA

Normal ammonia and normal blood pH

Repeated vomitings Refractory myoclonic Dysmorphism, hypotonia,

Mental retardation seizures, spasticity, seizures, blindness,
opisthotonus rhizomelia, deafness, lbng
tract signs

PKU $ ruk***$ Galactosemia $ neroxisomal

lll-*r ry _
----q|*:9:':*J
I

+r+i
ttPlasma phenyl alanine tPlasma +ve urinary and Reducing substances
&
tvlcre in blood
K $
^Urinary metabolites of CSF glycine GALT assay $
phenyl alanine

Fig. 13.8: Algorithmforclassificationof neurometabolicdisorders.OTC,ornithinetranscarbamylasedeficiency; CPA,carbamylphosphate

;
J
sr nthetase; ASA, arginosuccinate aciduria.
F

II
t
 -
ESSENCE OF PEDIATRICS

Destruction of
Huntington chorea previously normal
Hallervorden-
Spatz disease
e.g., Schilder disease ffi

Macrocephaly ffi
Fffiffi#W4@M
!

With organomegaly *
lnfantile Gaucher Adrenoleuko MLD
Alexander ffi
Niemann*Pick **un*nn*u***-
Canavan
M ucopolysaccharidoses
Megalencephalic
leuko.

Fig. 13.10: Algorithmic approach to the diagnosis of white matter

Genetic $ Acquired # d isease.
Leish I SSPE
#M
ffi

#X*-#
Fig. 13.9: Algorithrnic approach to the diagnosis oi gray matter Clinical features:
d iseases.
o May be uni-system/multi-system, affects different systems
in different age'.
2. lYhite matter DBD: Spasticity, long tract sign, ataxia' gait, o Age of onset: During school vear, but may be varia'itle.
visual and hearing problem.
o Llepatic and hen-rolytic manifestations appc: r' i.l!'iy, neu-
rologic later.
Mimickers of DBD:
o Untreated diseate is plogressive.
o Postencephalitic sequelae e There may be a positive farnily history
o HIV encephalopathy I Nervous system: D1'stonia, chroreo-athetosis' psvchiatric
c Progressive rubella panencephalitis manifestations, speech disturbances, poor school perfor-
r Chronic subdural hematoma mance, and slow deme rrtia.
e Neoplasia with raised ICT r Eyes: Cataract and I(F ring (Greenish brown ring usually
c Toxins: lead encephalopathy seen at the limbic region, present at the time when, neuro-
r Fpileptic encephalopathies logic or psychologic symptoms deveioped, best detected by
Figures 13.9 and i3.10 depict algorithmic approaches for grav slir lamp exrminlrion).
matter DBD and white matter DBD, r:espectively.
c Hepatic: Acute hepatitis, fulminate hepatic failure, CLD.
c Others: Hemolytic anemia, rickets, renal tubular acidosis'
Treatment:
a Svmptomatic treatment
INVESTIGATIONS
r Fhr.siotherapy
s iiounseiing r Serum cerulopiasn-rin <20 mg/dl; 24-hr Urinary coPper
r i : . Jrment of underlving catr.es >100 prg/24 hr.
D-penicillamine cl.rallenge test: 500 mg of penicillamine
is givei-r orally immediately before and 12 hour after urine
collection-24-hr Urinary copper >1575 p'g|24 hr.
\X/ilson disease is an autosomal recessive cor.rdition inr-olr'ing Others: Liver function tests, liver biopsy for copper esti-
copper metabolism resulting in abnormal deposition in tl-re maticn.
basal ganglia, iiver, cornea, and other svstems. c Serum copper is not very usefr-rl for diagnosis.
 NEUROLOGY

r MRI: of basal ganglia and helps in

Reveals the changes and practical value of different diagnostic categories. Epilepsia
1 996;36(1 1):105 1-9.
distinguishing mild cortical atrophy from mitochondrial
Chokrovorry S (ed). Management of epilepsy Boston: Butterworth-
disorders.
Heinemann, 1999.
o The gold standard for diagnosis remains liver biopsy with Rogers M (ed). Tbxtbook ofPediatric Intensiue Care3'd ed. Baltimore:
quantitative copper assay->200 pg/g dry weight of liver. \Williams and Hilkins, 1996.
Appleton RE. The new antiepileptic drugs. Arch Dis Childhlzd
TREATMENT 199675:256-52.
Pomeranz A, et al (ed). Pediatric Decision making strategies- To
D-penicillamine 10*30 mg/kg/d orally 3-4 div. doses 30 accompany. Nelson Textbook of Pediatrics 16'r' ed. Philadelphia: VaB
minutes before meals. Begin Penicillamine therapy with Saunders Co,2002.
25-50o/o of the desired doses for first 1-2 week then t0. Parthasarathy A (ed) . MP Textbook of Pediatrics 4'h ed. New Delhi:
Jaypee Brothers, 2009.
increased over l-2 week. Dose may be reduced.after control
11. Ghai OP (ed). Esential PediatricsT'h ed. New Delhi: CBS Publishers,
is achieved. Tieatment is lifelong, supplement Pyrodoxine 2009.
25 mg daiiy. Common side effects of D-penicillamine are 12. Molla MR. Concise Tixtbook of Pediatics 2"d ed. Dhaka: Daisy
fever, rash, pancytopenia, and nephritic syndrome. QNB, 2007.
a Tiientine may also be used. 13. Kalra V. Practical Paediatric Neurologt. 2"r ed. New Delhi: Arya
a Ztnc:lt reduces copper absorption from gut. A daily 75-100 Publication. 2008.
mg elemental zinc in 3-4 div. doses with meals for whole r4" Abend I'JS, Marsh E. Convulsive and nonconvillsive rr:tus cpi-
lepticus in children. Current Tieatment Options in Neurology
life.
2009:17:262-72.
Currently recommended treatment includes Tetrathiomo-
15. The Status Epilepticus tVorking Party. The treatment of convulsive
lybdate with zinc. status epilepticus in children. Arch Dis Child2000;83:415-9.
Tieatment will be continued for lifelong with low copper 'Willams
t6. Menkes JH. Child Neurohgy 7'r' ed. Lippincott: & \X/ilkins,
diet. 2006.
-WD. Status epilepticus in c.hildren" Current
r Family screening: Families of all index cases should be 17. Singh RK, Giallard
scgeened by clinical evaluation, slit lamp examination for Neuro loglt and Neuros cience Reports 2009 ;9 :137 -44.
18. Bueno SC, et al. Bacterial meningitis in children. Pediatr Clin N
KF ring, serum ceruloplasmin, and 24-hr urinary copper.
Am 2005;52:795-810.
Asymptomatic affected sibs should be put on low copper
19. Oates-whitehead RM, et al. Fluid therapy for acute bacterial
diet and zinc therapy lifelong. meningitis. Cochrane Database 2005 ;(3) :CD0047 86.
20. Duke T, Mokela D, Frank D, et al. Management of meningi-
tis in children with oral lluid restriction or intravenous fuid
of maintenance volume: a randomized tiil.. Ann I/op Paediatr
1. Campbell AGM, Mclntosh N (eds). Fotfar and Arneils Textbooh 2002;22:145-57.
' ofPediatrics 5"'ed. Edinburgh: Churchill Livingstone, 1998. 2t. Chaudhuri A. Adjunctive dexamethasone treatment in acute bacte-
2. Behrman RE, Keigman Rlvl, Jenson HB (ed). Nelson Tixtbook rial meningitis. Lancet Neurol 2004;3(l):54-62.
of Pediatrics 18'h ed. Philadelphia: \7B Saunders Co, 2007. 22. Mclntyre P Should dexamethasone be part of routine therapy of
3. Siberry GK, Iannone R (ed). The Harriet Lane Handbook. 15'r' bacterial meningitis in industrialized countries? Adu Exp Med B;ol
ed. Mosby Inc, 2000. 2005;568: I 89-9-.
4. Committee on Classification and Terminology of the International 23. Parhan N, Faust 5N, l.evin &4. Bacterial mcningiris and brain
League Against Epilepsy. Proposal for revised clinical and ab'scess. hf edidile 200I t29 :49-5 /+ "

electroencephalographic classification of epileptic seizures. Epelepsia 24 Shorvon SD. International Child Neurology Education Conference,
19B1;22(4):490 50t. Delhi 2000. J Neurol 2005;252:125-30.
5. Committee on Classificaiion and Terminology of the International 25. Rahman MM, Saha NC, Mannan MA, Neonatal Seizure: An
League Against Epilepsy Classification of epilepsia: its applicability Update. Bangladesh J Child Health 2008;32(1):21-8.
 CHAPTER 14
Muscle Disorders

Chopter Contents
Muscle disorders... ..............................286 Limb-girdle muscular dystr0phies...................,..,..............288
Duchenne muscular dystrophy ....286 Facioscapulohumeral dy$r0phy........... ... ...289
Myasthenia gravis .....................,. ...........................................281 Inflammatory myopathies............. ,..., ...289

o Repeated respiratory tract infections due to weakness of

muscles with resultant inefrective cough.
Classification Pharyngeal muscle weakness leads to aspiration, nasal,_regur-
o Muscular dystrophy gitation, and nasal voice.
c Duchenne muscular dystrophy Contractures most commonly involve the ankle, hip, knee,
o Becker muscular dystrophy and elbow. Scoliosis is common.
o Hypertrophy involving the calf muscles, tongue, and forearm
Myoionic muscular disorder
in the descending order of frequency.
o Myotonic muscular dystrophy a Cardiomyopathy (a constant feature).
o Congenital myotonic muscular dystrophy a Intellectual impairment'in all patienrs, although only
o Myotonia congenita (Thomsen disease)
20-30o/o have an IQ <70% (majority of them have learn-
o Metabolic myopathies ing disabiliries).
o Glycogen storage diseases Type II, V Ml Death (usually by 18 years of age) is due to respiratory
'r Lipid storage myopathies failure, intractable heart failure, pneumonia, aspiration, and
,r- Familial periodic paralysis airway obstruction.
Infammatory myopathies Most of the patients confined to. wheel chair by the age

c Polymyositis of 12 years. ,

,r Dermatomyositis
Congenital myopathies
o Central core disease
o Nemaline myopathy
This is the most common hereditary neuromuscular disorder,
inherited as X-linked reces-sive trait (30o/o fresh mutation), the
abnormal gene being located on X p21.
CLINICAT FEATURES
a .Poor neck control (earliest manifestation).
a Patient climbr up on his own leg wh-eq. asked to _staLnd fto-
supine position (Gower sign) dueto weakness of pelvic-girdle
muscle. tendelenburg gait or waddling gair to compensate
for the pelvic-girdle muscle
Unable to support body weight when suspended by the a-xilla.
LABORATORY DIAGNOSIS
Creatine phosphokinase (normal <160 IU/L; in DMD
15,000-35,000 IU/L) is consistently elevated in DMD even
in presymptomatic stage inciuding at birth. A normal serum
creatine kinase (CK) Ievel is incompadble with the diagnosis
of Duchenne dystrophy, although in terminal stages of the
disease, the serum CK value may be considerably lower
than it was a few years earlier because there is less muscle
to degenerate. Other lysosomal enzvmes presenr in muscle,
such as aldolase and aspartate aminotransferase, are also
increased but are less specific.
Blood- polyrnerase chain reaction (PCR) for tlfe*dygrophin
if the clinical features and
gene mutation is rhe primary test
serum CK are consistent with the diagnosis.
Dystrophin immunocytochemistry: It is more specific and
weakness. is performed on muscle biopsy sections; it detects one-third
of cases that do not show a PCR abnormality.
I
 MUSCLE DISORDERS

o Immunohistochemistry for dystrophin I, II, and III
abqent dystrophin in DMD and reduced, patchy dystrophin
st4ining in Becker muscular dystrophy (BMD). It may be
prognostic of the clinical course as Duchenne or Becker disease.
o Muscle biopsy from vastus lateralis (quadriceps femoris)
and the gastrocnemius shows connective tissue proliferation,
scattered degenerating and regenerating myofibers, foci of
mononuclear inflammatory cell infiltrates as a reaction to
muscle fiber necrosis, mild architectural changes in still
functional muscle fibers, and many dense fibers. Confirma-
tion of the diagnosis by either blood PCR or muscle biopsy
should be done in every case.
o Electromyography: Characteristic myoPathic changes are
present but not specific for DMD.
o Cardiac assessment by echocardiography, electrocardiogra-
phy (ECG), and radiography of the chest is essential and
should be repeated periodically.
o plications of chronic steroid therapy, including considerable
Carrier detection: Asymptomatic carrier has increased serum
epf i" 80% of cases. In prepubertal carrier girl, CPK is
highest at8-l2year of age. In2\o/o cases, there is normal CPK
level. Muscle biopsy of suspected female carrier may detect
an additional 10% in whom serum CPK is normal. Specific
genetic diagnosis by PCR on peripheral blood is definitive.
Antenatal diagnosis: Can be diagnosed as early as 12'h week
of gestation from chorionic villi DNA analysis by southern
blot-br PCR.
Differential diagnosis:Becker muscular dystrophy, limb-girdle
muscular dystrophy, cerebral palsy, myelopathies, polyneuritis.
Thble 14.1 lists features differentiating DMD from
muscular dystrophy.
reveals patients usually are already using their entire reserve for daily
function, and exercise cannot further strengthen involved
muscles. Excessive exercise may actually accelerate the process
of muscle fiber degeneration.
r Cardiac decompensation often responds initially well to
digoxin.
a Pulmonary infections shouid be promptly treated.
a Patients should avoid contact with children who have obvious
respiratory or other contagious illnesses. Immunizations for
infuenza virus and other routine vaccinations are indicated.
Drug therapy: Prednisone (0.75 mglkgld) for the first 10
days of each month (for about 6 months) to avoid chronic
complications. Glucocorticoids decrease the rate of apoptosis
or programmed cell death of myotubes during ontogenesis and
may decelerate the myofiber necrosis in muscular dystrophy.
Strength usually improves initially, but the long-term com-
weight gain and osteoporosis, may offset this advantage or
even result in greater weakness than might have occurred in
the natural course of the disease. Nevertheiess, some cases
of Duchenne dystrophy treated early with steroids appear to
have an improved long-term prognosis as r4veli as the short-
term improvement and may help keep patients ambl]atory
for more years than expected if untreated.
Follow-up: At 6-monthly intervai for observing progression
of weakness in skeletal muscles, drug side-effects, cardiac
status, development of deformities' muscle charting, timed
functional activities.
Becker

TR.EATMENT
Myasthenia gravis, an autoimmune disease, is usualiy sporadic
o No specific treatment, treatment is mainly suPportive. but may be of familial origin. It is characterizedby develop-
Nutritional support with adequate calcium and fluoride ment of autoantibody against acetylcholine receptor protein
suppiementation. at motor end plate.
o oPhysiotherapy delays, but does not always prevent contrac-
tures. It contributes little to muscle strengthening because
CtINICAt FEATURES

Table 14.1: Difference between Duchenne Muscular Dystro- o Clinical form: The disease may occur at different ages:
phy (DMD) and Becker Muscular Dystrophy (BMD)
o Neonatalmlasthenia: One in seven mothers having this
disease transmits antibody to the fetus transplacentally.
Age of presentation Usually between 1 and Later The infant develops transient myasthenia, starting dur-
4 years ing the first week of life and lasting <2 months'
Molecular defect Absent dystrophin' Qualitatively/ Congenital myltsthenia: Antiacetylcholine antibodies
protein Quantitatively are not detectable in the patientt serum in this form.
abnormal dystrophin
protein Ptosis, usually the first symptom, is noted by 2 years of
age; swallowing difficulties and truncal weakness may
Severity Clinically severe Clinically milder
fo11ow.
Ambulation Patient is in wheel Ambulatory even at 12
.l
chair by the age o{ 2 years of age Juuenile myasthenia: This form is similar to aduit form,
years except it starts late in the first decade or in second decade

Learning disability More frequent Less trequent of life.

Death Usually by the end of Much later fusociated disorders: Rheumatoid arthritis, thyroiditis,
2*rdecade thymoma, and diabetes'mellitus occasionally may occur.
ESSENCE OF PEDIATRICS
DIAGNOSIS
o Repetitive stimulation: Electromyography (EMG) reveals
a decremental response of the compound muscle action
potential in response ro repetirive stimulation of motor
nerve. Singie fiber electromyography is more sensirive
measure of neuromuscular transmission than repetitive
stimulation. If myasthenia is limited ro rhe exrraocular
muscles, levator palpebrae, and pharyngeal muscles,
evoked-potential EMG of the muscles of the exrremi-
ties and spine (diagnostic in the generalized disease) is
usually normal.
o Antibody testing: Anti-ACh antibodies should be assayed
in the plasma but are inconsistently demonstrated. About
one-rhird of affected adolescents show elevations, bur
anti-ACh receptor antibodies are only occasionally dem-
onstrated in the plasma of prepubertal children. Many
juvenile myasrhenics who show no anti-ACh antibodies in
serum have instead antibodies against the receptor tyrosine
kinase (MuSK), which is also localized at the neuromus-
cular junction and appears essenrial to fetal development
of this junction.
r Other serologic tests of autoimmune disease, such as anti-
nucfear antibodies and abnormal immune complexes, are
positive in more extensive autoimmune disease involving
vasculitis or tissues other than muscle. A thyroid profile
should always be examined. The serum CK level is normal
in myasthenia gravis.
o The role of conventional muscle biopsy in myasthenia gravis
is limited. It is not required in most cases, bur about l7o/o
of patients show inflammatory changes. Muscle biopsy tissue
in myasthenia gravis shows nonspecific rype II muscle fiber
airophy, similar to rhar seen with disuse atrophy, steroid
effects on muscle, polymyalgia rheumatica, and many other
conditions.
o Edrophonium (Tensilon) test: It is a clinical test. Edro-
phonium chloride, a short-acting cholinesterase inhibitor,
is administered intravenously (0.15_'0.2 mg/kg; max.
10 mg). Ptosis and ophthalmoplegia improve within a
few seconds, and fatigability of other mqgcles decreases.
r$(/here should test be performed? The setting may
be
the emergency department, hospital ward, or, at times, a
physician's office; the important issue is preparation for
potential complications such as cardiac arrhythmia or cho-
linergic crisis.
o The hearr is not involved, and electrocardiographic findings
remain normal. Radiographs of the chest often reveal an
enlarged thymus, but the hypertrophy is not a thymoma. It
may be further defined by tomography or by CT scanning
of the anterior mediastinum.
TREATMENT
Mild myasthenia gravis requires no rrearment.
1. Cholinesterase inhibiting drugs:
a. Neostigmine methyl sulfate (0.04 mg/kg) may be
'
given IM 4-6 hourly, but most patients tolerate oral
neostigmine bromide (0.4 mglkg 4-5 howly).
If dysphagia is a major problem, drug should be given
about 30 minutes before meal to improve swallowing.
b. Pyridostigmine, an alternative, but dose required is
about four times greater than that of neostigmine.
2. Long-term steroid therapy with prednisolone may be
effective due to auroimmune basis of the disease.
). Thymectomy may provide cure. Thymectomy is most
effective in patients with high titers of anti-ACh receptor
antibodies in the plasma and who are sympromatic for
<2 years.
4. Plasmapheresis is efFective, especiallv among who do not
respond to steroid, but provide onl-7 rsmpolxry remission.
5. IWG (intravenous immunoglobulin) is some times ben-
eficial and it is less invasi',-r than plasmapheresis.
6. Refractory patients ma| rerfoilci to rituximab, a mono,
clonal antibody to rhe B-ceil CD20 antigen.
7. Neonates with transient matgrnallv transmitted myasthenia
gravis require cholinesterase inhibitors for onlj'a few
days or occasionally for a few weeks, especially to allow
feeding. No other trearment is usuaily necessary.
coMPilcATlONS
Neuromuscular blocking drugs, such as succinylcholine and
pancuronium may cause paralysis for weeks even after a single
dose. Also, cerrain anribiotics may porenriate myasrhenia; rhese
include the aminoglycosides (gentamicin and others), eryrhro-
mycin, penicillin, sulfonam(es, fluoroquinolones.
PROGNOSIS
Some patients undergo spontaneous remission afrcr a period
of months or years. Others have a permanent
disease extend-
ing into adult life.
This term encompasses a group of progressive hereditary myopa-
thies that mainly affect muscles of the hip and shoulder-girdles.
Distal muscles also eventually become atrophic and weak.
Hypertrophy of the calves and ankle contractures develop in
some forms, causing potential confusion with Becker muscular
dystrophy.
I
I

The initial clinical manifestations rarely appear before
middle or late childhood or may be delayed until early adult
life. Low back pain may be a presenting complaint because
of the lordotic posture, resulting from gluteal muscle weak-
ness. Confinement to a wheelchair usually becomes obliga-
tory at about 30 years of age. The rate of progression varies
from one pedigree to anothet but is uniform within kindred'
Although weakness of neck flexors and extensors is universal,
facial, lingual, and other bulbar-innervated muscles are rarely
involved. As weakness and muscle wasting progress, tendon
stretch reflexes become diminished. Cardiac involvement is
unusual. Intellectual function is generally normal. The clini-
cal differential diagnosis of hmb-glrdle muscular dystrophy
includes juvenile spinal muscular atrophy (Kugelberg-\(elander
disease), myasthenia gravis, and metabolic myopathies.
Most cases of limb-girdle muscular dystrophy are of auto-
somal recessive inheritance, but some families express an
autosomal dominant trait.
The EMG and muscle biopsy show confirmatory evidence
of muscular dystrophy, but none of the findings is specific
enough to make the definitive diagnosis without additional
clinical criteria. In some cases, adhaiin (a dystrophin-related
glycoprotein of the sarcolemma) is deficient; this specific
defect may be demonstrated in the muscle biopsy by immu-
nocytochemistry. Increased serum CK level is usual, but the
magnitude of elevation varies among families. The ECG is
usually unaltered.
This relatively benign muscular dystrophy manifests around
puberry. Facial, shoulder-girdle, and proximal arm muscles are
involved. Patients cannot close the eyes forcefully, whisde or
hold air in the oral caviry. Smiling or grimacing produces faint
muscle movements. Winging and elevation of scapulae occur
due to weakness of the scapular muscles. Foot drop results from
weak peroneal and anterior tibial muscle . Progress of weakness is
relatively slow. Pelvic-girdle involvement is late and less marked'
Hlpertrophy does not occur. Neurological involvement occurs in
form of sensorineural hearing loss. Cardiac muscles and mental
functions are notably spared.CPK levels may be slightly elevated.
Muscle biopsy and EMG frequently show changes suggestive of
a neurogenic origin, along with myopathic changes.
These myopathies are characterized by inflammation in skeletal
muscle resulting in muscle fiber damage and subsequent clinical
muscle weakness. There are two major categories-idiopathic
and infectious.
MUSCLE DISORDERS
Idiopathic Juvenile dermatomyositis, poll'rnyositis, inclusion body
myositis, myositis as overlap syndrome, and others-(z) eosino-
philic myositis, (ii) focd,nodular myositis, (lii) sarcoid myopathy.
Infectious: Viral myositis (inf uenza, human immunodefi ciency
virus, and others); parasitic myositis (trichinosis, toxoplasmosis,
cysticeraosis); bacterial myositis; and fungal myositis.
Floppy infant is an infant with marked hypotonia of the
muscles and variable associated weakness. This may be associ-
ated with frequent respiratory infections, feeding difficulties,
facial weakness, ptosis, ophthalmoplegia, and dislocated hips'
Contractures may develop in later stages.
Common causes of foppy infant are:
o Central nervous system: Perinatal asphl'xia' neonatal
encephalopathy, kernicterus, cerebral palsy (atonic type),
intracranial hemorrhage, chromosomal anomalies including
Down syndromer inborn errors of metabolism.
o Spinal cord: Anterior horn cell disease, \Terdnig Hoffman
(spinal muscular atrophy), poliomyelitis.
o Peripheral nerves: Familial dysautonomia, hereditary motor
sensory neuroPathy.
o Myoneural junction: Neonatal myasthenia gravis' infantile
botulism, following antibiotic therapy.
o Muscles: Muscular dystrophies, congenital myotonic dystro-
phies, congenital myopathies, polymyositis, glycogen storage
disease, arthrogryposis multiplex congenita.
o Miscellaneous: Protein-energy malnutrition, hypothyroid-
ism, rickets, Prader-\X1lli syndrome, Ehler-Danlos syn-
drome, cutis laxa.
Common conditions resulting in a limp:
o Neuromuscular disorders: Paralysis of muscles, (e.g., acute
poliomyelitis, traumatic neuritis), hemiplegia, acute hemiple-
gia of childhood, myopathies and muscular dystrophies,
hemi-hypertrophy of muscles.
o Disorders of bones and joints:
,> Hip: Congenital dislocation of hip, pyogenic arthritis,
tuberculosis, transient synovitis, trauma' rickets, slipped
epiphysis, osteochondritis
o Knee and anhle: Ti-rberculosis, rheumatoid arthritis,
transient synovitis, trauma, osteochondritis
Foot Painful lesions of the nails, toes, and soles, e.g', warts,
corns, blisters, paronychia, ingrowing toe nail, fracture III-
fitting shoes, osteochondritis.
Spine Tuberculosis, scoliosis, congenitai defects.
ESSENCE OF PEDIATRICS

3. ForfurJO, funeil GC (ed). Tbxtbook of PaediatricsT& ed. Edinburgh:

4,
Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of
Pediatrics 18'h ed. \VB Saunders Co., 2008.
2. Dworkin PH. NMS: Pediatrics 4.h ed. philadelphia: Lippincott
\X/illiams & \filkins, 2000.
Churchill Livingstone, 2008.
Rahman MM, et al. Prednisolone in Duchenne muscular dystrophy.
Bangladesh Med Res Counc Bull 2001:2711):3842.
Ghai OP, Paul VIi Bagga A (eds.). Essential Pediatriu 7s ed. CBS
Publishers & Distributors Pvt Ltd, 2009.
 CHAPTER T5
Hematology

Chopter Contents
Diseminated intravascular c0agu1ati0n........................... 500
Adverse events following blood transfusion .301

iv) Sequestration in mononuclear phagocytic system,

e.g., hypersplenism.

Anemia may be defined hemoglobin (Hb) content per

as decreased 3. Impaired red cell production:
unit volume of blood, in respect with age and sex. Hemoglobin a) Deficiency of substances needed for RBC produc-
level <11 g/dl in the children between 6 months and 6 years and tion: Iron, folate, vitamin B,r, vitamin Bu, vitamin
below 12 g/dl in the older children are recognized as anemia. C, protein, and thyroxine.
b) Bone marrow faihJrer Bone marrow aplasia or bone
marrow infiltration.
CLASSIFICATION
c) Anemia associated with chronic disorders, such as:
According to etiology, anemia can be classified as: i) Infection
1. Due to blood loss
ii) Connective tissue disorders 0lA, SLE, PAN, etc.)
iii) Chronic renal failure
a) Acute: Trauma, hematemesis from drug-induced iv) Disseminatedmalignancy
. gastric erosion, bleeding from varices or vascular
malFormations.
4. Others: Anemia of Brematurity, physiological anemia of
early infancy, PFM.
b) Chronic: Hookworm infestation, polyposis, pro-
lapse rectum, portal hypertension, ulcerative colitis,
shigellosis.
2. Hemolytic anemia: Increased rate of destruction
CAUSES
a) Intrinsic (intracorpuscular) abnormalities of red cells
i) Disorders of hemoglobin synthesis: Decreased iron stores:
(1) Deficient globin synthesis: thalassemia ,.r Preterm, small for dates, twins
(2) Hemoglobinopathies: Hb E diseases, sickle r> Feto-maternal hemorrhage
cell anemia o Repeated venous sampling
ii) Red cell enzyme deficiencies: G5PD deficiency
iii) Red cell membrane disorders: Hereditary Decreased intake:
spherocytosis (HS) 'r Delayed weaning
b) Extrinsic abnormalities , lron poor dier
i) Antibody mediated: Decreased absorption:
(a) Isohemagglutinins, e.g., transfusion reaction, o PEM
erythroblastosis fetalis o Malabsorption syndromes
(b) Autoantibodies, e.g., idiopathic, drug o Chronic diarrhea
associated, SLE
[) Mechanical trauma to red cells: Microangiopathic Increased losses:
hemolytic anemia, DIC o GI bleeding
iii) Infections: Malaria o Hookworm infestation
 -

ESSENCE OF PEDIATRICS

r Peptic ulcer contains 0.5 mg/dl), iron fortified cereals, pulses, beans,
,> Bleeding diathesis ripe banana, green leafi, vegetables, meat, fish, eggs, liver
r should be introduced gradually.
Increased demand
Preterm baby and those with diminished iron srores should
o Prematurity, LBW receive 1*2 mg of elemental iron/kg/d from the age of 6
r Recovery from PEM weeks for about 3 months.
r Adolescence
' a Periodic deworming.
a Iron containing foods such as meat, fish, and poultry and
CTINICAL FEATURES facilitators of iron absorption such as vitamin C rich foods
(citrus, tomatoes, potatoes) should be included in the diet.
Symptoms:
o Pallor; anorexia, palpitation, shortness of breath; breathless- Orol lron Theropy
ness on exertion
o Increased desire to ingest unusual substance, i.e., rubber,
Daily dose: 6 mg elemental ironikg/d in 3 divided doses.
Thble 15.1 lists elemental iron content of some commercially
brick, dirt, mud (pica)
available iron preparation.
Signs:
Duration of therapy: 6-8 weeks beyond hemoglobin and red
o Pallor of the skin, mucous membrane, palms, and conjunctiva cell indices has returned to normal. It takes about 8 weeks to
o Arrophic glossitis. sromaritis increase hemoglobin level to normal level. So in total, nearly 4
o Koilonychia months treatment is needed.
o Irritability, "poor" mental and motor developmental test
scores on Bayley scale Response to iron therapy in iron-deficienry anemia:
o Shorter attention span and decreased school performance. 72-24 hours : Replacement of intracellular iron enzymes,
o Splenomegaly is present in l5o/o of cases. The triad of dyspha-
subjective improvement, decreased irritabiliry
gia due to esophageal web, koilonychia, and splenomegaly in
and increased a_ppetite.
a patient with IDA is known as Plummer Mnson syndrorire.
36-48 hours : Initial bone marrow response, erythroid
hyperpiasia.
INVESTIGATIONS 48-72 hours: Reticulocytosis, peaking at5-7 days.
4-30 days : Increase in hemoglobin level.
o Hemoglobin: Beiow the acceptable level for the age.
1-3 months : Repletion of stores.
r Redcell indices: Lowel than normal MCV MCH, MCHC for
age; widened red celi distribution width (RDV| (>l4.5ok). Hemoglobin level should rise 1 g/dl every 10 days after a lag
r Blood smear: Red ceils are hypochromic and microcytic period of 7-10 days. If this qise does not happen, the possibili-
with anisocytosis and poikilocytosis. ties ofblood loss, infection, ?enal failure, folic acid deficiency,
o Reticulocyte count: Usually normal. or rhalassemia should be considered.
o Serum ferritin: Always reduced; <72 nglml is considered
diagnostic of iron deficiency. Porenterol lron Theropy
o Serum iron reduced, but its estimation has serious limita-
Indications:
tions and is not a recommended test for iron deficiency.
r Newer tests: Decreased serum transferrin receptor level o Intolerance to oral iron
(STfR), high free erythrocyte protoporphyrin (FEP) level. o Poor patient compliance
o Bone marrow: Decreased or absent stainable iron. o Chronic diarrhea
o Bleeding (from gastrointestinal tract when agglavated by
DIFFERENTIAL DIAGNOSIS oral therapy)
Dosage of iron dextran: Total dose is 100 mg for infants
Thalassemia, anemia of chronic disease, iead poisoning, sidero-
under 6 months, 200 mg between 6 and 12 montl-rs, 300 mg
blastic anemia.

TREATMENT Table 15.1: Elemental lron Content of Commerciallv Avail-

able lron Preparations
N ulrilionql Counselin g
o Exclusive breast-feeding for at least 6 months. Ferrous sulfate 200 40
o Supplementary foods rich in iron should be administered
Ferrous gluconate 300 35
from the age of 5 months. As milk is the poor source of
Ferrous fumarate 200 65 !
iron (cow milk contains 0.02-0.3 mg/di, and human milk
t
I
l
 HEMATOLOGY

ftom 12 months to 24 months, and 400 mg for children over Severe aplastic anemia is defined as bone marrow cellularity
24 months of age. of <25o/o, and at ieast two of the following:
An alternative formula is: mg of iron to be injected (TDD = o Cranulocyte count <500/mm'(<200/mm' in very severe
Desired rise of hemoglobin (g/dl) , wt in kg x 4 aplastic anemia)
TheZ-tract injections (deep IM, intragluteal) are also given r Platelet count <20,000/mm3
50 mg (1 ml) dose daily. o Reticulocyte count <40,000/mm3

Blood Tronsfusion
Blood transfusion is not recommended for simple correction
of iron deficiency. It may be given in severe anemia requiring
correction more rapidly than is possible with oral or parenteral
iron or because of presence of some complicating factors, such
as, prior to surgery or debilitated children with infection,
especially when signs of cardiac dysfunction are present and
the hemoglobin level is 4 gldl or less.
In such low hemoglobin level, packed blood cells should be
given only 2-3 ml/kg at any time. If there is evidence of congestive
cardiac failure, modified exchange transfusion can be considered;
frusemide can be administered; digitalis may be given.
Aplastic anemia is defined as hypoplastic or aplastic bone
marrow coupled with peripheral biood pancytopenia. Initially,
patient may have only mono- or bicytopenia.
It can be a consequence of stem cell failure (seed theory)
that is either related to a drug, toxin, or a virus; or that has
resulted from eithel cell-mediated or antibody-dependent cyto-
toxicity; or abnormality of the supporting microenvironment
(soil theory) resulting from drugs, toxin, viruses, or immune-
mediated mechanism.
CtINICAI FEATURES
Thrombocytopenia will lead to bleeding manifestation, espe-
cially skin bleeds, mucosal bleeds, hematuria, and rarely intrac-
ranial hemorrhage. Neutropenia may present as fever with or
without localization of infection. Anemia appears last and if
severe will lead to fatigue, breathlessness, puffiness, and features
of congestive cardiac failure. One should look for history of
hepatitis and history of drugs, etc.
INVESTIGATIONS
. Pancytopenia is common; bicytopenia or monocytopenia
may also occur.
Peripheral blood film: There wili be anemia in which the
red cells are normochromic and normocytic, occasionally
macrocytic. There is ptesence of leukopenia with decreased
absolute neutrophil count, as also a decreased p'latelet
count.
Bone marrow study: Shows hypocellular bone marrow with
empry spicules, increased f3t spaces, and increased lympho-
cyte/plasma cells, etc.
Chromosomal study to rule out Fanconi anemia, which will
show chromosomal breaks.
Tests to find out etiological factors like viral markers, etc.

CTASSIFICATION TREATMENT
1. Inherited: Supporlive
a) Fanconi anemia
b) Familial aplasric anemia Blood transfusion/packed cell transfusion should be given to
c) Shwachman Diamond syndrome (predominantly neu- maintain Hb level >8-10 g%. Avoid blood from relatives if
tropenia) bone marrow transplantation (BMT) is contempiated. Iron
d) Diamond-Biackfan anemia chelation may be necessary afrcr 40-50 transfusions, so as to
keep serum ferritin level <1000 ng/ml.
2. Acquired:
a) Primary or idiopathic Bleeding: Platelet concentrates are given if patient has acute
b) Secondary - mucosal bleeds or severe internal bleeding, especialiy with
i. Drugs piateiet count <5000-10,000/mm3. General measures like
ii. Chemical agents local pressure, dental care, avoiding use of NSAIDs also help
iii. Radiation prevent bleeding.
iv. Infection-EB virus, HBV HIV parvovirus B19 Infection: Treatment should be commenced immediately
(especially in children with hemolltic anemia) with an antibiotic regimen (e.g., penicillin and gentamicin/
v. Malnutrition cefotaxime/ceftazidime, etc.) before collecting sample from
Aplastic anemia is staged as mild, moderate, and severe. The blood, urine and obvious site of infection for culture and
definition of mild and moderate aplastic anemia varies among sensitivity (C/S). Modi& the antibiotics after culture report,
researchers and institutions. or considering the clinical response in 48-72 hours.

ESSENCE OF PEDIATRICS
One may consider use of antifungal (fluconazole/ampho-
tericin B) if culture shows fungal growth or when patient does
not improve despite changing antibiotics in 7-10 days time.
Growlh Foclors
Hemopoietic growth factors like G-CSF and GM-CSF have
been used to counteract neutropenia in aplastic anemia, espe-
cially neutropenia that occurs following BMT. Neupogen
(Filgrastim = G-CSF) is given by continuous subcuraneous
infusion (20 mglkgld) or continuous intravenous infusion (30
mg/kg/d) after dilution with 5o/o glucose solution.
lmmunolheropy
Cyclosporine-A, antithymocyte globulin (ATG), or
antilymphocyte globulin (ALG) is used either alone or
in combination. Cyclosporine-A is given orally in a dose
of 12 mglkgld for up to 6 months. Side effects like
electrolyte disturbances, renal toxicity, hirsutism, gingival
hypertrophy cln occur. ATG/ALG are animal sera against
human lymphocyte or T-cells, which can be used in a dose
of 10-40 mglkgld in IV infusion for 5*10 days after test
dose. The success rate is 60-700/o.
Sleroids
Steroids stimulate erythropoiesis. These also stabilize capillary
membrane and decrease bleeding. They are useful to counteract
side effects of androgenic steroid on growing epiphysis and the
serum sickness of immunotherapy. Oral prednisolone of 0.5-1
mglkgld and then tapered to a minimum effective dose. IV
methylprednisolone has been used in dose of 15*20 mglkgld
and tapered gradually over 15 days. (It should be given along
with androgen or AIG). It has been found to be effective in
30-40%o cases.
Bone Morrow Tronsplo nlolion
The treatment of choice is bone marrow transplantation using
HLA identical sibling donor. Cure rate is 70-80o/o in severe
aplastic anemia. Problems are high cost, GVHD-x6111s 8a
chronic, infection, graft failure, etc.
Olhers
Other therapies that have been used in the past with incon-
sistent results include androgen, cyclophosphamide, and plas-
mapheresis.
PROGNOSIS
Aplastic anemia is a serious disorder that frequently terminates
in death within 6 months. Mortality rate vary in different
series from somewhat <50o/o to as high as 80% to the first
year after presentation. Death is usually due to bleeding and/
or infection.
q
Thalassemia and hemogiobin-E disease are rhe commonesr
forms of hereditary hemoglobin disorders (HHD). Hemoglobin
E disease occurs as a result of substitution of glutamic acid by
lysine at the2Sth position of the B-globin chain. Thalassemia
syndrome result from reduced or absent production of B-chain
in globin. Thalassemia and hemoglobin E disease are almost
all inherited as recessive disorders so that heterozygote subjects
are generally healthy.
Normal hemoglobin structure: Hemoglobin is composed
of heme + four globin polypeptide chains. In older children
and in adults, there are three different types of hemoglobin
present normally.
r HbA = 2 alpha and 2 beta chains (>96.5%)
o HbA, = 2 alpha and 2 delta chains (<3.5%)
o HbF = 2 alpha and 2 gamma chains (<2%)
Common HHD:
o B-Thalassemia:
,r B-thalassemiamay'or
o B-thalassemia intermedia
o B-thalassemia minor (i.e., thalassemia trait or carrier) '
r Hemoglobin E disorder
c Hemoglobin E disease
o Hemoglobin E trait (i.e., carrier)
o Hemoglobin E-B-thalassemia
,r Mild hemoglobin E-B-thalassemia
c Moderate hemoglobin E-B-thalassemia
,r Severe hemoglobin E-B-thalassemia
Two types of E-B-thalassemia have been described that depend
on the presence or absence of HbA.
First is the combination of hemoglobin E with B-thalassemia
that produces no B-globin chains. This genorype results in
E-B-thalassemia, characterized by the presence of HbE, and
HbF without detectable HbA, I{bE constitute about 50% and
the rest is HbF. Second is E-B-thaiassemia in which certain
amounts of HbA are also detected in addition of HbE and
HbF. In a study conducted in Bangladesh, out of 136 HHD
cases, 99 (72.7o/o) cases were E-B-thalassemia and 37 (27.2o/o)
cases were B-rhalassemia major.
Pedigree: Parenrs to offspring rransmission:
Ifa B-thalassemia carrier individual (father or mother) marries
another healrhy individual (father or mother), 50% of the oF
springs will be normal and another 50% will be B-thalassemia
carrier (they are reasonably normal, can lead normal life).
If a B-thalassemia carrier individual (father or mother)
marries another B-thalassemia carrier (father or mother),
50o/o of the offsprings will be B-thalassemia carrier (can
)
I
$

lead normal l[e),25o/o will be absolutely normai, and rest
25o/o will be B-thalassemia major (diseased).
o If a hemoglobin E carrier individual (father or mother)
marries another healthy individual (father or mother), 507o
of the offsprings will be healthy and50oh will be hemoglobin
E carrier (can lead normal iife).
o If a hemoglobin E carrier individual (father or mother)
marries another hemoglobin E carrier individual (father or
mother), 50% of the offsprings will be hemoglobin E carrier
(can lead normal hfe), 25o/o will be absolutely healthy, and
r
?
the rest 25o/o u,ill have hemoglobin E disease (diseased).
a
r If a thalassemia carrier individual (father or mother) marries
a hemoglobin E carrier individual (father or morher),25o/o
of the offsprings will be hemoglobin E carrier (can lead
normal hfe), 25o/o of the ofrsprings will be p-thalassemia
carrier (can lead normal life), and 25o/o will be healthy child,
resr 25o/o will have E-B-thalassemia (diseased).
CtINICAL FEATURES
B-Tholossemiq
The spectrum of clinical features of p-thalassemia varies. One
end of the spectrum is the serious homozygous form (thalas-
semia major) that presents in early infancy (6-18 months) with
(l) progressive pallor, (ii) splenohepatomegaly, (iii) bony changes
(rhalassemic facies), and (iu) growth retardation; if left untreated
is invariably fatal during the first few years of life. At the other
end of the spectnrm is a heterozygous form (thalassemia minor
or trait) in which the patient can lead practically a normal
life. In between these two extremes are thalassemia intermedia
(they are also homozygous), characterized by varying degrees
of pallor, splenohepatomegaly, and bony changes.
Hemoglobin E Disorders
Hemoglobin E disease (homozygous) usually does not exhibit
any clinical symptom; however, these patients may be mildly
anemic. Individual with hemoglobin E trait (hemoglobin E
heterozygote) is clinically normal with minimal changes in the
hematologic parameters.
E-B-Tholossemiq
E-B-thalassemia is an important of chronic childhood
cause
disease in South-East Asia. Hemoglobin levels in E-p-thalas-
semia studied recently ranged from 3 to 13 g/dl with a remark-
able variability in severity; some (about 50%) present with
features of thalassemia major, some (about 5070) present with
features of thalassemia intermedia, and some (few percentage)
present with mild symptoms.
Screening for carrier detection (for genetic counseling):
o Low hemoglobin, MCV and MCH.
o Osmotic fragility test, a cheap procedure, has been developed
for detection of b-thalassemia trait.
I
HEMATOLOGY
o Hemoglobin electrophoresis: Thehemoglobin electrophoresis
will show the following findings in a case of thalassemia
minor (carrier): (l) decreased HbA, (ii) increased Hb{
(, 3.5o/o), and (lll) normal or slightly increased HbF.
If there is associated iron deficiency, then the elevated
Hb{ usually falls to normal, but return to supranormal
level when iron stores are replenished by iron therapy.
DIAGNOSIS
CBC: Morphology of RBC shows anisopoikilocytosis, micro-
cytosis, hypochromia, target cells, basophilic stippling, and
nucleated cells. Reticulocyte count is increased.
a Osmotic fragiliry (reveals reduced fragility).
a Iron studies (treru- iron level, J iro.r binding capaciry
t transferrin saturation, and t ferritin level).
Radiological study (widening of medulla, thinning of cortex
and prominent trabeculation-hair on end appearance).
Test of organ dysfunctions (LFT, ECG, endocrinal
studies).
Hemoglobin electrophoresis: Findings are as follows:
o B-thalassemia major: HbA-absent or decreased;
HbAr-variable; HbF-increased
o Hemoglobin E trait: HbA-decreased; HbAr-variable;
HbE-20-35o/o.
,> Hemoglobin E disease: HbA-decreased; Hb{-vari-
able; HbF-normal or increased slightly; HbE-majori-
ty of hemoglobin are HbE.
o E-B-thalassemia: HbA-decreased; HbAr-increased;
HbE-present.
TREATMENT
-
Trqnsf usion-Chelotion Theropy
Regular blood transfusion (hypertransfusion): This aims to
maintain hemoglobin level between 10 and 12 go/o by trans-
fusing 10-15 ml of packed blood cells/kg/transfusion once
in every 2-4 weeks (raises hemoglobin levei 6y abott 3.5 gl
dl). On an average, annual blood requirement is 180-200 ml
packed cells/kg. Neocyte (mean age being 12 days) transfusion
prolongs the intervai between the two transfusions.
Chelation: Chelation therapy should be started when serum
ferritin level is >1000 ng/ml.
o Desferrioxamine (DFO, Desferol 500 mg) 30-70 mglkgld
should be given SC 5-6 times/wk over 6-8 hours usuaily
by an infusion pump. Recent development of light weight
disposable elastomeric (balloon) pumps for continuous IV or
SC chelation has improved the tolerability and effectiveness
of DFO. It is preferred by all patients to the conventionai
bartery-operated pumps. A single gram of DFO is able to
bind 84 mg of iron. In general, rhe goal is to reduce serum
ferritin level <1000 ng/ml. 600/o of DFO chelated iron is
excreted in urine and 40o/o in stool. Addition of Vitamin C
ESSENCE OF PEDIATRICS
100 mg daily prior to DFO therapy increases iron excretion.
Vitamin C helps in conversion of hemosiderin into ferritin
from which iron can be chelated.
o Defewiprone, L,(Kelfer,500 mgcap): Dose is 50-100 mg/kg/d
given orally. Result shows that it is 70*80o/o as effective as
DFO. It mobilizes iron from hemosiderin, transferrin, and
ferritin. Common adverse ellects of L, are severe neutropenia
and arthropathy and GI symptoms. Its safety and efficacy
is still under evaluation in severai countries.
c Deferasirox (ICL 670): Given once daily at 20 mglkg
seems to be an effective orally active iron chelator and is
reasonably well-tolerated.
o Combination therapy: Combined therapy with the DFO at
time of transfusion and deferriprone in between transfusion
has been proved to be effective, safe, and acceptable to patient.
Spleneclomy
If the child has already developed splenomegaly and signs of
hypersplenism (requirement of packed celis 200 ml/kg/yr or
more, decreased \WBC and platelet counts) and is older than
5 years, splenectomy is indicated. All children needing sple-
nectomy should receive pneumococcal vaccine (Pneumovax),
H. influenzae vaccine (Hiberix), and meningococcal vaccine 4
weeks prior to surgery. Prophylactic penicillin therapy must
be continued lifeJong. Hepatitis B and chicken pox vaccine
(varilrix) should aiso be given.
Bone Mqrrow Tro nsplonlolion
Allogenic bone marrow transplantation from HLA identical
donors represents the only definitive cure for thalassemia
major. The three most important adverse prognostic factors
for survival are (l) hepatomegaly (>2 cm), (ii) poral fibrosis,
and (iii) iron overioad.
Three prognostically useful categories of patients were
identified: Class I, with neither of these variables; Class II,
with one or two variables; Class III with all of the afore-
mentioned variables. The event-free survival rate is 90%o for
patients in Class l, 82o/o for Class II, and 53% for Class
IIL Cord blood stem cell transplantation has also been
tried successfully.
lnduclion of Gommq Choin Synthesis
Augmenting the production of gamma chain reduces globin
chain imbalance and increases synthesis of HBF and lead to an
improvement in effective erythropoiesis with consequent better
survival of RBC and correction of anemia; commonly used
drugs are hydroxyurea, recombinant human erythropoietin,
and butyric acid analog. The efficacy of these drugs is variable.
Gen-e Theropy
Gene therapy aims at a stable transfer of globin genes into
hemopoietic stem cells of the recipients. This remains to be
the major challenging goal of future curative therapy.
PREVENTION
L Genetic counseling: Counseling in the preventive program
is based on accurate diagnosis and confidentiality. Empha-
sis should be given to the following pointsi
i) Natural history of the disease and range of treatment
available and outcome. Genetic basis of the disease
should be stated.
ii) Risk estimation: Approximate risk of recurrence
should be told to the concerned.
iii) Support group: A combined approach is necessary
for continuing sLrpport to the patient and his family.
Support group should include physicians, surgeons,
blood bank personnel, nurse, social worker, NGO
workers, journaiists. A 10-20 member support group
can be organized for a single thalassemia parient;
the members will donate blood life-long to this
patienr.
i r) Carrier identification and marriage counseling: This
issue has been discussed earlier. Once a carrier is
detected, he should be informed weli about his carrier
state, and marriage counseling should be done. Mar-
riage between rwo carriers as well as cousin marriage
should be discouraged.
v) Reproductive choice: In counseling session, the repro-
ductive choices, named the birth control or adoptibn
should be discussed in detail.
vi) Prenatal diagnosis: Prenatal diagnosis can be done
around 8-9 weeks of gestation by analysis of
amniotic fluid cells or chorionic villi by means
of DNA analysis or by polymerase chain reaction
(PCR) based procedure. Selective termination of
affected fetus can,prevent birth of a thalassemic
ch ild.
2. Marriage between two carriers should be prohibited by
passing law in the pariiament. Marriage registrar should
not register marriage between two carriers. Each suspected
couple before their marriage should submit a certificate
of testing before they can be married.
General awareness: Public education should be accom-
plished by mass media, posrers, and informative booklets,
lectures and discussion with the community leaders, and
the population at large.
It occurs commonly in African, Medirerranean, Chinese, and
South-East Aian ancestry. Bart's hemoglobin is usually detected
by neonatal screening. Most of the cr-thalassemia syndromes
are the result of deletion of one or more of the u-globin genes
on chromosome 16. Normal diploid cells have lour cr-globin
genes; thus, the variable severity of this disease is related to
the gene relation.
 HEMATOLOGY

CtINICAL FINDINGS o Anemia when exacerbated during infection, transfusion

Persons with three cr-globin genes (one gene deletion)
asymptomatic and have no hematologic abnormalities; Hb
electrophoresis in the neonatal period shows 0-3olo Bart's hemo-
globin. Persons with two ct-globin genes (two gene deletion)
are typically asymptomatic; Hb electrophoresis shows 2-10%o
Bartt hemoglobin in the neonatal period, but is normal in
older children and aduits. Persons with one u-globin gene
(three gene deletion) have a mild to moderate severe microcltic
hemolytic anemia (Hb 7-10 g/dl), which may be accompa-
nied by hepatosplenomegaly and some bony abnormalities;
Hb electrophoresis shows l5-30o/o Bart's hemoglobin in the
neonatal period; later in life Hb H (composed of 4 B-globin
chain) is present.
The deletion of all four u-globin genes causes severe
intrauterine anemia, asphyxia and results in hydrops fetalis'
and fetus dies shortly after delivery. Extreme pallor and massive
hepatosplenomegaly ar€ present. Hb electrophoresis reveals
>75o/o Bart's hemoglobin with a complete absence of normai
Fetal or adult hemoglobin.
Table 15.2 summarizes the o-thalassemia syndrome.
coMPUCATTONS
o Iron loading: 'When microcltic anemia is treated with iron.
o Person with Hb H disease may have exacerbation of anemia,
: requires blood transfusion.
l.
o Hypersplenism requires splenectomy.
F
o \(/omen pregnant with hydropic cr-thalassemia are subject
L
to roxemia and PPH.
TREATMENT
t
o o-Thalassemia trait requires no treatment.
o Those with Hb H disease should receive folic acid and
avoid oxidant drugs that could cause hemolysis in G6PD
deficiency.
Table 15.2: cr-Thalassemia Syndrome: Cenotype, Number of
Cenes, and Clinical Features
may be required.
are o Hypersplenism may develop later in childhood; it requires
splenectomy.
o Genetic counseling and prenatal diagnosis should be offered
to families at risk.
Purpura is the spontaneous bleeding or extravasation ofblood
in the skin that does not blanch on pressure.
o Petechiae: Pin-head or pin-point sized macule of blood in
the skin.
o Ecchymosis: Large extravasation of blood in the skin.
o Hematoma: Fluctuant swelling due to gross bleeding.
Bleeding is usually absent when platelet count is 40,000-80,000/
mm3. Bleeding is common when count is <30,000-40,000/
mm3, and severe spontaneous bieeding occurs when platelet
count is <10,000/mm3.
Tourniquet test: Is performed by inilating a blood pres.sure
cuff to a point midway between the systolic and diastolic
pressure for 5 minutes. 3 minute after the cuff has been
defated, the test is considered positive when 10 or more
petechiae per 2.5 cm2 area, about 1 cm below the cubital
fossa are observed.
TMMUNE THROMBOCYTOPENIC PURPURA (lTP)
Clqssificqlion
Acute ITP: It is associated with severe thrombocltopenia and is
preceded by viral infection in 50oio of cases; majority (80-90%)
of children have a natural remission within 2 months.
Chronic ITP: Patients have an insidious onset of symptoms
with moderate thrombocytopenia persisting for at least 6
months. Children with chronic ITP have only l0-20o/o chance
of naturai remission.
Relapsing ITP: Onset is similar to acute ITB but children have
relapses and remission during the course of the disease.

aalart 4 Normal N N
Clinicol Feqiures
-clcro 3 Silent carrier O-3"/" N Acute ITP
Hb Bart's
Perfectlv healthy 3-5 years old child suddenly develops asym-
-/ua or 2 a-thal trait 2-10o/. N
metrical petechiae and purpura. Bleeding from gum and
-al'.rr Hb Bart's
mucous membrane occuts when thrombocytopenia is severe'
-/-a 1 HbHdisease 15-30% Hb H present
There is a history of preceding viral infection 1-4 weeks before
Hb Bart's
onset of thrombocytopenia. 17o of patients develop intracranial
-/- 0 Fetal hYdroPs >75o/"
hemorrhage.
Hb Bart's

1
Physical examination is normal except signs of hemorrh age.
a indicates presence of u-globin gene; indicates deletion of c-globin gene
N = Normal results, Hb Bart's -'y,, Hb H - F, Mucous membrane lesion is the hall mark.
ESSENCE OF PEDIATRICS

Chronic ITP Treqlment

70-20o/o of children with acute ITP go on to develop chronic
ITP It is characterized by recurrent bleeding persisting for
>6 months. Spleen may be palpable rn 5-l5o/o of children
with chronic ITP ReferThble 15.3 for featurres
acute and chronic ITPs. Thble 15.4 lists features distinguish-
ing coagulation disorders and disorders of platelet or vessels.
Differenliol Diognosis
Aplastic anemia, acute leukemia, hemophilia, Henoch-
Schonlein purpura, drug-induced thrombocytopenia, scurry,
SLE.
lnvesligolions
o CBC-thrombocytopenia.
o Bleeding time-prolonged; CT and APTT-normal.
r Bone marrow examination reveals normal granulocytic
erythrocytic series with normal or increased number of
megakaryocytes. It is essential to exclude leukemia, aplastic
anemia, and tumor cell infiltration.
o Anti-nuclear antibody (ANA) to exclude SLE.
o In 600/o of patients, evidence of circulating anti-platelet
antibodies can be demonstrated by immunofluorescence.
Acute ITP
IV immunoglobulin and corticosteroid have been used most
commonly lor the management of severe bleeding.
differentiating
e IV immunoglobulin IWG (Sandoglobulin):When resistant
to corticosteroids
o i g/kg/d for 1-2 days through infusion to be given in
4-6 hours causes rapid rise ofplatelet count. It blocks the
Fc receptors and thus prevents the piatelet phagocytosis.
r IV anti-D 50-75 mglkg to only Rh-positive children.
o Prednisolone 2 mglkgl d for 2-3 weeks or until a rise in
platelet count to >20,000/mm3. Then taper the dose to
another 2 weeks to avoid side effects of steroid.
r Methyiprednisolone 30 mg/kg/d IV for 3 days shows
quick response.
and o Supportive care:
,r If there is massive bleeding and volume replacement is
needed, blood transfusion may be given and in case with
life-threatening intracranial bleeding; platelet transfu-
sion needs to be avoided (may be given when indicated
under cover of steroids). Blood transfusion and platelet
transfusions are not routinely needed.

Table 15.3: Acute and Chronic ITP

r Restriction ofphysical activities, sports, and avoidance of
aspirin and other NSAIDs. All immunization should be
avoided during the acute phase.
Peak age incidence Young children (4 yi1 Adolescence
Serpredrlection None F:M = 3:1 (hronic ITP
Antecedent Common; on:el-d( ute U nusuai
infeclion insidious
The principle of therapy in patient with chronic ITP is to
decrease the risk of serious hemorrhage rather than to achieve
Hemorrhagic bullae Common in severe cases Rare
tin oral mur osa) normal platelet count. Treatment of chronic ITP should be
Platelet count <20,000/mmr 30-B0,00Olmmj
individualized.

Duration 4-6 wk >6 mo o Prednisolone is started as in acute ITP but after 4-6 weeks
Spontaneous B0% Uncommon of therapy, steroids are used in smaller doses either on
remission alternate days or daily for 6*9 months.
o Imm.unoglobulins (iVIG) have been used 1 g/kg/dose every
2-6 weeks.
Table 15.4: Distinguishing Clinical Features of Disorders o1 o Other drugs, e.g., vincristine (0.02-2 mg/kg) or vinblastine
Coagulation and Disorders of Platelet or Vessels
(0.4 mg/kg) or cyciophosphamide I-2 mglkgldose, have
been used weekly.

Onset of bleeding Insidious onset

r Other immunosuppressive drugs like azathioprine,
Acute onset
cyclosporin have been used with varying success.
Petechiae Rare Characteristic r Danazole, a semisynthetic androgen, has been used in dose
Mucosal bleeding Less common Common of 300-400 rng/m2/d.
Superficial ecchymosis Characteristic Rare o Splenectomy: Majority of patients (65-850/o) achieve remis-
Hemarthrosis Common Rare sion immediatelv after splenectomv.
Hematoma (deep Common Rare
muscle) Prognosis
Sex Hemophilia Chronic ITP 10-20% olchildren with acute ITP go on to develop chronic
common in male common in female
ITP, and about 8070 of children with acute ITP have sponrane-
Positive family history Common Rare
ous resolution within 6 months.
 HEMATOLOGY

a. Dosage scheduleforfactor WII: Factor VIII is given

IV 12 hourly to maintain the required blood level, as
its half-life is 8 hours. I unit of factor VIII/kg raises
Hemophilia A and B are the most common inherited bleeding
factor VIII level by 2o/o.
disorders and account for 80-90% of coagulation disorders.
They are inherited as sexlinked recessive disorders (sporadic
mutation is 20-30o/o). \7omen are the carriers but are asymp-
tomatic, while their male ofrsprings suffer from the disease.
Hemarthrosis 20 Ulkg 12 hourly x 3 days Rest to affected part.
.l
5 U/kg 12 hourly x 2 days lce packs. Elastic
CLASSIFICATION 10 Ulkg'12 hourly until bandage, non-weight
the joint normalizes bearing, analgesics,
rarely joint
r Hemophilia A: Due to deficiency of factor \4iI. Normai
aspirations.
level of factor \4II is >30-40 U/dl.
Intramuscular 20U/k3,12 hourly
r Severe hemophiiia: Factor viii level < I U/dl. hematoma until hematoma is
r Moderate hemophilia: Factor viii level >1-5 U/dl. well-resolved
,r Mild hemophilia: Factor viii level >5-30 U/dl. Maior surgery, 50 U/kg 12 hourly x
life+hreatening 7 days, then initiate
o Hemophilia B: Due to deficiency of factor IX. hemorrhage continuous in{usion of
r Hemophilia C: Due to deficiency of factor XI. (e.g., CNS, 2-3 U/k{hr to maintain
Cl bleeds, airway factor Vlll >100 U/dl
obstruction) far 24 hour, then give
CTINICAL FEATURES 2-3 U/kg/hr continuously
for 5-7 daYs to mainiain
Mild hemophiliac patients are usually asymPtomatic and may the level >50 uldl
develop prolonged bleeding following tooth extraction, severe
trauma, or sufgery. b. Fresb frozen.plasna (FFP) useful for mild bieeding;
Severe hemophiliac patient develops spontaneous bleeding c. Cryoprecipitates arc used in both hemophilia and
in the skin, subcutaneous tissue, and in the musculoskeletal von'\?illebrand disease.
system. Common manifestations are easy bruising, hemarthro- d. Fresh blood nansfiision can be used where factor
sis, and intramuscular hematoma. The hail mark of hemophilia VIII, FFB or cryoprecipitates are unavailable.
is the hemarthrosis of knee or ankle. Nearly one-third of 2. Desmopressin acetate (DDAVP): An analogue of ADH
patients develop bleeding following circumcision, l-2o/o of
0.3 pg/kg in mild to moderate hemophilia, which induces
neonates may develop intracranial bleeding. Excessive bleeding
the release of factor VIII and plasminogen level.
may result from the umbilical cord, since maternal factor VIII
3. Epsilon amino caproic acid (EACA) and tranexamic
cannot pass through placenta.
acid: These antifibrinolytic agents act by inhibiting the
plasminogen activiry and are useful for the management
DI FFERENTIAT DIAGNOSIS of mucosal bleeding.
4., Primary prophylaxis is a better mode of management
Idiopathic thrombocytopenic purpura, aplastic anemia, acute in patients with severe hemophilia, they should be given
leukemia, von \Tillebrand disease. factor VIII replacement 2-3 times a week.

INVESTIGATIONS Hemophilio B

CBC: Platelet count is normal. Clotting time is prolonged, Hemophilia B should be treated with factor IX. One unit
but sometimes it may be normal. Bleeding time is normal. of factor IX/kg raises factor IX level by lo/o. In emergencies,
APTT (activated partial thromboplastin time) prolonged, when factor IX is unavailabie, fresh frozen plasma can be given.
2-3 times the upper iimit of normal. Cryoprecipitate does not contain factor IX.
o Mixing tests of APTT: Studies with normal plasma or with
absorbed plasma.
o Factor assay: Factor viii level is decreased in hemophilia. PROPHYTAXIS
o Patient should avoid trauma, aspirin, other NSAIDs, and
TREATMENT intramuscular injections.
o Prophylactic immunization against hepatitis B should be
Hemophilio A done.
I . Replacement therapy: Sources of factor VIII are fresh frozen e Careful dental hygiene maintenance and regular dental
plasma, cryoprecipitate, and factor VIII concentrate. examination.
 ESSENCE OF PEDIATRICS
PROGNOSIS
Great majority of patients with severe hemophilia and moder-
ate hemophilia may now expect ro live well into adult life, to
earn their own living. The most serious hazards are intracranial
bleeding and development of inhibitors (IgG antibodf) fo1-
lowing factor VIII concentrate.
In disseminared intravascular coagulation (DIC), intravascular
activation of the coagulation cascade leads to (i) fibrin
deposition in the small blood vessels; (ll) tissue ischemia,
resulting in release if tissue thromboplastin and consumprion
of labile clotting factors (i.e. platelets, factor II,
V VIII, and
fibrinogen); and (iii) acrivarion of the fibrinolytic system.
ETIOTOGY
Infections:
o Bacterial (septic sboch): Meningococcus, Staph. Aureus,
S. pneumoniae, Pseudomonas, E. coli, H. influenzae.
: Viral: Measles, dengue hemorrhagic fever, varicella
'> Fungal: Candida, Histoplasma, Aspergillus
o Protozoa:Malaria
Malignancies: Acute leukemia (promyelocytic), neuroblas-
toma, histiocytosis X.
Neonatal causes: Neonatal sepsis, birth asphyxia, hypo-
thermia, respiratory distress syndrome, se,r.re Rh-irrcom-
patibiliry necrorizing enterocoiitis.
Miscellaneous: Purpura fulminans, giant hemangioma,
hemolytic transfusion reactions, major surgery.
CLINICAT FEATURE
Usually presents with:
o Bleeding tendency manifested as purpura, ecchymosis, easy
bruising, mucous membrane bleeding, localized hemorrhagic
gangrenous skin lesions, oozing from puncture sites.
r Thromboembolic phenomenon with hematuria, oliguria
leading ro acure renal failure, diarrhea, abdominal distension,
ileus, respiratory distress, deterioration of consciousness,
coma, and convulsions.
o Pallor or jaundice secondary to hemolytic anemia.
. Hypotension leading to vascular collapse and shock. Sudden
onset of bleeding from multiple sires associated with throm-
bocytopenia should suggesr DIC. Hypotension is a feature of
DIC associated with factor XII acrivarion. Clinical manifes-
tations of the underlying conditions and sudden worsening
of the child are evident.
TABORATORY DIAGNOSIS
Peripheral blood film in DIC shows fragmented, distorted, and
contracted red cells along with spherocytes, thrombyctopenia.
Hemolysis of red cells is indicated by increased levels of plasma
hemoglobin and reduction of hepatoglobin levels. Consumption
of fibrinogen and various clotting facrors (II, d 1111; ,..r.rtm
in prolongation of thrombin time, p! and pTT. Fibrinogen
levels are reduced, and levels <150 mg/dl are seen in70-80o/o
of patients. Partial thromboplastin time (pTT) is prolonged,
and FDP level is increased.
TREATMENT
Successful managemenr of a bleeding child with DIC is
dependent primarily on rhe efrective control of underlying
disease and elimination of the aggravaring factors. Mar"gel
ment of aggravating factors like acidosis, hypoxia, ,hoik,
etc. should be corrected. Effective and prompt rrearmenr
of underlying condition is the key for successful manage-
ment of DIC.
General measures should include (l) compulsory inrravenous
administration of all medications and (ii) administration of
vitamin K ro correcr vitamin K dependent factors.
Specific rrearmenr includes (z) drug therapy to interrupr
clotting process, (ii) replacement of coagulation f".tor, ,rrd
platelets, and (iii) exchange transfusion ro remove the potential
triggers and to replace the coagulation facrors.
Heporin
Heparin inhibits thrombin formarion, and thus it is more
effective in cases with prominenr thrombotic manifestations.
It is administered in loading dose of 50 U/kg followed by
50*100 U/kg every 4 hourly. Coagulation factors should
become normal if heparin therapy is effective. Heparin therapy
should be continued until fibrinogen levels are >100 mgful
and platelet counr increases to >100,000/pL. CT shoulJ be
checked during rrearment.
Replocement Theropy
Platelet concenrrare along with coagulation factors have
been used to replenish the platelets, coagulation factors, and
antithrombin III levels. Infants may be given 10-15 ml/kg
of fresh frozen plasma along wtth ll2 to 1 unit of platelei
concentrares every 12-24 hourly. Platelet counrs shouid be
maintained above 50,000/prl, while fibrinogen levels should
be maintained >75 mgldL Blood should be administered to
corfect anemia.
Exchonge Tronsfusion
Exchange transfusion not only provides the factors bur has
the added advantage of removing the (z) circulating FDp,
(ii) actlated procoagulanrs, and (iii) roxins. Ali these factors

aggravate bleeding. Two volume exchange transfusion should be
performed with fresh blood or with packed red cells suspended
in fresh blood or with packed red cells suspended in fresh
frozen plasma. Plateiet concentrates should be administered
Table 15.5: Adverse Events Following Blood Transfusion
Acute hemolytic Preformed al Ioantibodies (most
trans{usion rommonly to ABOI and occasionally
reaction autoantibodies cause rapid intravarcular
hemolysis of transfused cells with
activation of clotting (DlC), activation of
inflammatory mediators and acute renal
fa i lu re.
Delayed hemolytic l-ormation o[ al loant ihodie: after
transfusion reaction lran<fusion and re.ullant destrurtion
oI transfused red cells. usually by
extravascular hemolysis.
Bacterial Conlaminalion of units result: in growth
contamination of bacteria or produt tion oi clinically
significant levels of endotoxin. Hepalitis
B, C, AIDS, cytomegalovirus infection,
malaria, syphilis can be lransmitted.
C raft-versus-hosi Lymphocyles from donor lransfused in
disease an immunocompelenl host.
Febrile reactions Usually caused by leukoagglutinins in
recipient cytokines or other biologically
acl ive compounds.
Allergic reactions Most causes not identified. In lgA-
deficient individuals, reaclion occurq as
a resull o[ anlibodies to lgA.
lron overload There is no physiologic mechanism
to e\crete excess iron. Targ,et organs
include liver, heart, and endocrine
organs. ln patients reteiving red cell
transfusions over long periods of time,
there is an increase in iron burden.
Dilutional Massive blood lo.s and transiusion
coagulopathy with replacement with fluids or blood
componenls and defir ient clotting far tors.
Trans{usion-related Ar ule lung injury orcurring wilhin
acute lung injury 4 hours a{ter trans[usion. Two sets
of factors interact to produce the
syndrome. Patient factors: infection,
surgery, cytokine therapy. Blood
component factors: lipids, antibodies,
cytokines. Two groups of factors interact
during trans{usion to result in lung injury
indistinguishable from ARDS.
Circulatory Clinical feature is that of
overloading pulmonary edema, heart {ailure,
somelimes complicated by lerminal
bronchopneu mon a. i
Air embolism Small amount of air may not cduse dny
problem. 10-40 ml may cause alarming
symptoms, even death in a sick patient.
after the exchange. Thble 15.5 iists adverse events following
blood transfusion.
Mortaliry in cases of DIC is very high, and it varies between
50o/o and B0o/o.
Fever, chills, nausea, chest pain,
back pain, pain at transfusion site,
hypotension, dyspnea. oliguria, dark
uri ne.
Fever, jaundice, anemia. A small
percentage may develop chronic
hemolysis.
Chills. high iever, hypotension, other
symptoms of .ep.is or endoloxemia or
endotoxic shoc k.
Syndrome can present with a variety
of organs involved, usually skin, liver,
gaslrointestinal trar t, and bone marrow
Fever. May also have chills.
Itching, hives, occasionally chills and
fever. ln severe reactions, cigns of
anaphylaxis may be seen: dyspnea,
pulmonary edema.
Signs and symptoms of dysfunctional
organs affected by the iron deposition
Bleeding
Tachypnea, dyspnea, hypoxia. Diffuse
interstitial markings. Cardiac evaluation
normal.
Progressive dyspnea, cyanosis, basal
creps over 12-24hr.
Sudden severe dyspnea, cyanosis, pulse
may be rapid, thready, JBP. Syn.ope
from cerebral ischemia.
!
HEMATOLOGY
The risk of this type of reaction is low
(1: t0000r, but the mortality rate is high
tup to 40"'or. Stop the transfusion; maintain
renal output with intravenous fluids and
diuretics (frusemide or mannitol); treat
DIC with heparin; and institute other
appropriate supportive measures.
Detection, definition and documenlation
(for future lransfusions). Supponive care.
Risk, I:2500.
Stop transfusion; make aggressive attempts
to identilv organism; plasma volume
expander, pressor agents. hydrocortisone,
antibiotic regimen (covering both Cram
+ve and -ve organismst.
Preventive management: lrradiation
'- I 500 cCyr of cellular blood
components transfused to individuals.
Supporlive; slowing of drip, antipyretic;
consider leukocyte poor products for
future. Risk per transfusion, 1:200.
Mild to moderale rea(tions:
cliphenhydramine. More severe
reactions: epinephrine SC and steroids
lV. Risk for mild to moderale allergic
reactions 1: I 000: severe anaphylactic
reactions, 1 :1 50,000. Washed red cells,
antihistamine arrd steroids may be given
before transfusion.
Chronic administralion of iron chelator,
such as desferoxamine.
Replacement of clotting factors or
plalelers with appropriate blood
( omponents.
May consider packed red blood cells
<2 weeks, platelets <3 days, washing
components to prevent syndrome.
Management: supportive care.
Stop transfusion, propped-up position,
O, inhalation, Digoxin & Diuretjcs +
morphine IV.
Place the patienl on his left side in a
head down position.
 -

ESSENCE OF PEDIATRICS

5. Dworkin PH (ed). Pediatrics 4'h ed. Philadelphia: Lippincott \Vil-

liams & Vilkins, 2000.
6. Behrman RE et al. Tbxtbook of Pediatriu 18'h ed. Singapore: Har-
Firkin F (ed). de Gruchy's Clinical Hematologlt in Medical Practice court Asia Pte. Ltd., 2007.
5'h ed. Delhi: Oxford University Press, 1989. Oski FA. Iron deficiency in infancy and childhood. N Eng J Med
7.
Clayden GS, Howkins R (ed). Pediatrics: Tieatment (r Prognosis 1."' 1993;329:190-3.
ed., New Delhi: Jaypee, 1989. B. Hoque MS, Alam MA. Thalassemia - Situation in DSH. In: Fight
Hay V\7, et al (ed.). Current Pediatric Diagnosis y'r Iieatment l8'h Thalassemia 1999; 4-11.
ed. Stamford, Connecticut: Appleton k Lange,2007. 9. Rahman ME, Perveen R, Choudhury AM, et al. Updating of
4. Parthasarthy A (ed). IAP Textbooh of Pediatrics 4'h ed. New Delhi: hereditary hemoglobin disorder. MMJ 2002;11 (1):49-53.
Jaypee, 2009.

re
 CHAPTER T6
r
0ncology

Chopler Contents
Childhood 1eukemia..............................................................303
Acuie lymphoblasiic Ieukemia................."......................303
Acute myeloid leukemia ..................................................304
Chronic myelocrrtic leukemia .........................................306
Non-Hodgkin 1ymph0ma.....................................................306 Retinoblasioma..........

Clinicol Feolures
a General systemic effects: Fever, malaise, headache.
Acute leukemias are defined as malignant clonal proliferation of a Features of bone marrow failure J Rgc-*.akness,
hemopoietic precursor cells Ieading to replacement of normal pallor; J \MBC-frequent infection, I platelet-bleeding.
bone marrow by immature cells and infiltration to lymph Features due to lymph node and other organ
nodes, spleen, liver or other organs. involvement:
The common childhood leukemias are: o Lymphadenopathy

r Acute lymphoblastic leukemia (ALL)

o Splenomegaly

o Acute myeloid leukemia (AML)

o Hepatomegaly

o o CNS manifestations-headache, vomiting, convulsion,

Chronic myeloid leukemia (CML)
cranial nerve palsy
o Congenital leukemia.
o Bone pain, arthralgia
Chronic lymphoid leukemia rarely affects children. o Painless testicular swelling
Of the childhood leukemias, 97o/o are acute leukemias, of o ALL is disseminated from the very beginning, so there is
which ALL is75o/o, AML20o/o, and remaining are less frequent
no scope for staging.
types. Chronic myeloid leukemia constitutes only 3o/o.
Ditferentiol Dio g nosis
Aplastic anemia, infectious mononucleosis, lymphoma, ITB
ACUTE TYMPHOBTASTIC TEUKEMIA
rheumatic fever.
It comprises 75o/o of all leukemias in children.
lnvestigotions
Morphological (FAB) classification:
o Complete blood count:
r L,: Lymphoblasts are small with scant cytoplasm and absent
o Hemoghbin: Moderate to marked reduction with nor-
or inconspicuous nucleoli.
mocytic normochromic red cell.
e Lr: Lymphoblasts are larger with abundant cytoplasm and
o Wbite cell count: Low normal, or increased.
one or more prominent nucleoli.
o c Blood vnear: Blast is usually abundant when count is
Lr: Cells are large with deeply basophilic and vacuolated
>10,000/mm3. Blast may be few or absent when leuko-
cytoplasm and prominent nucleoli.
penia is present.
Immunologically, AI-L may be pre-B cell AlL, B-cell ALL, o Bone maftota: Usually hypercellular, may be hypocellu-
T:cell AlL. Following features dictate the possibility of T:cell lar. Though >25o/o lymphoblast is diagnostic, in most of
AIL: (z) occurrence in older children and teenagers, (ii) pre' the cases, the marrow is completely (80-100%) replaced
dilection for males, (iii) high white blood cell count, often by blast. Specific bone marrow studies like cytochemis-
>100,000/mm3, (la) presence of anterior mediastinal mass, try immunophenoryping, and cytogenetic studies help
and (z) early dissemination to meninges and testes. in detail cell classification.
ESSENCE OF PEDIATRICS

o Chest radiograph: Mediastinal mass may be present. Intensification:

o Cerebrospinal fluid: CNS involvement in leukemia o TIT-MTX (<2 yr: B mg; 2 yr: 10 mg; >3 years: 12 mg)
classified as: + Hydrocortisone (25 mg) + Inj. Cytarabine (30 mg).
o CNS 1: <5 WBC + No blast r Vincristine 1.5 mglm2 IV flash weekly for 4 weeks.
o CNS 2: <5 \7BC + Blast o Daunorubicin 45 mglm2 IV in 100 ml of saline on days
o CNS 3: >5 \XrBC + Blast 1 and 2.
o Inj. Etoposide 100 in 100 ml of saline on days
o Bichemical assays:
r_5
^gl^'IV
o Serum LDH is usually raised, sometimes very high o Inj. Cytarabine 100 mg/m2 IV in 100 ml of saline, 12
o Serum uric acid is normal to raised hourly, on days 1-5.
c Serum electrolyte (hyperkalemia, hyperphosphatemia) r Thb Thioguanine 80 mg/m2 PO on days 1-5.
o Coagulation profile (if needed) o Tab. Prednisolone 40 mglm2 PO on days 1-5.
o Serum creatinine, SGPT baseline cardiac function should High-dose methotrexate: 2.5-5 g/m2 IV in drip. 10% of total
be done for management purpose, prior to initiation of MTX in 1" hour and rest of MTX in 23 hours in 6 divided
therapy. doses followed by Inj. Folinic acid 15 mglm2IV 6 hourly on
days 2-4.
Treolmenl Maintenance:
General Supportive Care r TIT-MTX (<2 yr: 8 mg; 2 yr: 10 mg; >3 yr 12 mg) +
Hydrocortisone (25 mg) + Inj. Cytarabine (30 mg) once
At presentation, patient may be dehydrated, infected, having
bleeding and anemia, and there may be impaired hepatic and
in every 3 months.
renal function due to leukemic infiltration. So, at first, the
r Vincristine 1.5 mg/m2 IV flash monthly.
child needs to be prepared for chemotherapy.
o Thb. Methotrexate 10-20 mg/m2lwk PO on days B, 15,
'r1 'rc:,
Hydration (3 Llm'124 hr), alkalinization, allopurinol or
a/.
-Lt

recombinant urate oxidase are used in the preparatory regimen.

Platelet transfusion and packed red cell transfusion are needed
fairly frequently when aggressive multi-agent chemotherapy is
employed.
e Tab. Prednisolone 40 mglm2 PO on Days l*5.
o Tab. Cotrimoxazole 10 mg/m2 PO 12 hourly every alterna-
tive day.
Total duration of treatment 2.5-3 years.

Prognosis
5pecifi c Treatment: Chemotherapy
Remission induction rate with these rypes of regimen is about
Intensive chemotherapy remains the mainstay of treatment of
95olo. Despite current intensive treatment, 25-30o/o of children
ALL. Many successful treatment regimens are available. All
with ALL show bone marrow relapse . This may be an isolated
modern ALL regimens include certain treatment slsmsnl5-
event, or may be combined with relapse at other sites.
induction of remission for 28 days, intensification for 5 days,
Patients with initial high VBC counr, age older than i0
CNS directed therapy, and maintenance therapy. The treatment
years and those younger than 12 months and patients who have
is tailored according to the risk to rhe parienr.
chromosomal rearrangement, like t(9 ;22) -Phlladelphia chromo-
Here, one of the chemotherapy schedules for standard risk
some or t(4;11)-MLL, have poor prognosis. Hyperdiploidywith
(age 1-10 yr,'WBC count <50,000/mm3, no CNS involvement,
>50 chromosomes is associated with a favorable ourcome.
no bad cytogenetic abnormaliry) ALL is given:

Treatment of ALL (standard risk): MRC XI ACUTE MYETOID LEUKEMIA

In ducti o n of remissi o n :
It comprises 15-20o/o of all leukemias in children.
o Vincristine 1.5 mg/m2 IV flash weekly for 4 weeks.
o L-asparaginase 6000 IU/m'zIM on alternate days starting Clossificolion
from day 2 =Total 9 doses.
o Tiiple intrathecal therapy-Methotrexate (TIT:MTX) (<2 FAB Classification
yr: 8 mg; 2 yr: l0 mg; >3 years: 12 mg) + Hydrocortisone M0: Minimally differentiated
(25 mg) + Inj. Cytarabine (30 mg), mixing together to be M1: Myeloblastic leukemia without maturarion
given weekly for 4 weeks. M2t Myeloblastic leukemia with maturation
o Daunorubicin 45 mglmz IV in 100 ml of saline on days M3: Hypergranularpromyeiocyticleukemia
1 and 2. M4: Myelomonocytic leukemia
o Tab. prednisolone 40 mglmz for 28 days. M4Eo: Variant, increase in marrow eosinophils
 \

ONCOLOGY

M5: Monocytic leukemia

Diagnosis *
M6: Erythroleukemia(DiGuglielmodisease)
M7z Megakaryoblasticleukemia I
ADE t
WH0 0assifi(ation
o AML with recurrent cytogenetic translocations
o AML with t(8 ;2 I ) q22;q22) AML /CBF-alpha/ETO
( 1
ADE g

o Acute promyelocytic leukemia: AML with t(15;17)

(q22;ql2) and variants PML/RAR-alpha
o AML MACE E
with abnormal bone marrow eosinophils inv(16)
(p | 3 ; q22) v agy t(I 6 ; I 6) (p | 3 ; q22) B F - b e tal MYH 1

c AML with 11q23 MLL abnormalities

o AML with multilineage dysplasia
o Y/ith prior MDS
o \Tithout prior MDS
o AML with myelodysplastic syndrome, therapy related
Allogenic BMT No further therapy I
o Alkyladng agent related Fig. 16.1: Algorithmic approach to the treatment of acute
o Epipodophyllotoxinrelated myeloid leukemia.
o Other types
o AML not otherwise categorized & cytosine):
Course 1 ADE (daunorubicin, eroposide,-i*ld
c AML minimally differentiated r Inj. Daunorubicin 50 mglm2 slowly IV push on days 1,
r AML withour maturation
r AML wirh maturation J, and 5.
o Acute myelomonocytic leukemia o Inj. Etoposide 100 mg/m2 IV in 100 ml over I hr on days

o Acute monocltic leukemia L5.

o Acute erythroid leukemia o Inj. Cytosine (Ara-C) 100 mg/m2 bd IVpush on days 1-10.
o Acute megakaryocytic leukemia Course 2 ADE (daunorubicin, etoposide, & cy.tosine):
o Acute basophilic leukemia o Inj. Daunorubicin 50 mglm2 slowly IV push on days 1,
o Acute panmyelosis with myelofibrosis J, and 5.
o Inj. Etoposide: 100 mglm2ld IV in 100 ml over t hr on
Clinicol Feqlures days 1-5.
o Sign and symptoms are related to anemia, thrombocytope- o Inj. Cy"tosine (Ara-C) 100 mg/m2 BD IVpush on days 1-10.
nia, and neutropenia, like those in ALL.
Course 3 MACE (M-amsa, cytosine, & etoposide):
o Organomegaly more prominent than AlL. Chloroma (a
localized mass of leukemic cells)-orbital or epidural-may o Inj. M-amsa 100 mglmzld IV (1 hr infusion) on days 1-5.
occur. Orbital chloroma causes proptosis. o Inj. Cytosine 200 mglm2ld IV continuous infusion on
r Leukostasis-resulting in hemorrhagic infarction in brain, days 1-5.
stroke, and other organs. o Inj. Etoposide 100 mglm2ld IV in 100 ml over t hr on
o Bleeding due to DIC is a common feature of acute promy- days 1-5.
elocltic leukemia (AML-M3). Course 4 Mid AC:
o Gum hypertrophy or leukemia cutis are characteristic of
acute myelomonoc).tic leukemia (AML-M4/M5).
o Inj. Mitoxantrone 10 mg/m2 slowly IV push on days 1-5.
r Inj. Cy.tosine (Ara-C): I glm2 bd IV over 2 hr on days 1-3.
Treolment Interval between courses = 2I days. All doses willbe 25o/o
for children <1 yr. M-amsa = Amsacrine.
General supportive measures: Same as ALL. Intensive induc-
tion regimen of AML is associated with an increased risk of
Prognosis
sepsis and fungal infection. Rapid initiation of broad-spectrum
antibiotic and possibly antifungal therapy is necessary for any Intensive chemotherapy foilowed by bone marrow transplanta-
lebrile episode while neutropenic. tion (like DCTER regimen) has shown to achieve cure rate of
Treatment of AML (except AML-M3 and AML of aboutT0o/o. Only chemotherapy based protocol, without BMT
trisomy 21): MRC X. Tieatment approach has been depicted like MRC-10 regimen can achieve a cure rate of 50o/o. Children
in Fig. i6.1 and has been described below: with AML who relapse have an extremely poor prognosis.

ESSENCE OF PEDIATRICS
cHRONTC MYELOID LEUKEMIA (CMt)
CML is insidious in onset. Diagnosis is generally made when
a blood count is performed for some other reason. Two rypes
of CML occur during childhood. Adult qpe CML, which is
twice as common as juvenile CML, is a clonal myeloproliferative
disorder arising from a neoplastically transformed stem cell. The
neoplastic cells almost invariably contain the Philadelphia chro-
mosome t(9;22). Juvenile CML is a form of myelomonocltic
leukemia, which is characterized by a proliferation of cells of
monoc)'tic and granulocltic origin. It is not a variant of adult
CML, and its cells do not contain the Philadelphia chromosome.
Clinicol Feolures
Adult type CML
1. Adult rype CML occurs in older children and teenagers
who are initially seen with:
(a) Lassitude and weight loss from hypermetabolism
(b) Bone pain
(c) Increasing abdominal girth from massive splenomegaly
2. Characteristic laboratory findings include:
(a) Extreme hyperleukocytosis, which is characterized by
a white cell count >100,000/mm3, a predominance
of more mature granulocytes on peripheral smear,
eosinophilia and basophilia
(b) Normal to increased platelet count
(c) Mild anemia
(d) Extreme myeloid hyperplasia in the bone marrow
(e) The Philadelphia chromosome
Juvenile (ML
1. Juvenile CML occurs predominantly in children younger
than 5 years of age and is more common in male patients
presenting with:
(a) Suppurative lymphadenopathy
(b) Moderate hepatosplenomegaly
(c) Desquamative, erythematous rash
(d) Purpura
(e) Pulmonary infiltrates
2. Characteristic laboratory findings include:
(a) Anemia with characteristics of fetal erythropoiesis
(b) Thrombocy'topenia
(c) Moderate hyperleukocy'tosis, which is characterized by a
mean white cell count of 60,000/mm3 and an increase
in monoc''tes and granulocl'tes in the peripheral blood
(d) An increase in
monocytes and granulocytes and
decrease in megakaryocltes in the bone marrow
I
o Bone marrow transplantation has been curative for both
adult and juvenile CML. For patients who have adult CML,
bone marrow transplantation is much more ef[cacious if it
is done during the chronic phase.
e Interferon has been shown to decrease the proliferation of
the Philadelphia chromosome- positive cells in adult CML
causing remission in 20olo cases.
Prognosis
Adult type CML has a median survival of >2 years and can
be subdivided into the following phases.
1. During chronic CML, the disease manifestations can be
well-controlled by chemotherapy.
2. During accelerated CML, clinical and laboratory findings
show marked deterioration, and patient responsiveness to
therapy diminishes.
3. During blastic CML, which is of short duration, the
disease acquires the features of a fatd. acute leukemia.
Blast crisis can be:
(a) Myeloid, which is more common than lymphoid and
usually is unresponsive to further therapy
(b) Lymphoid, which usually is brielly responsive to therapy
Juvenile CML is more rapidly fatal, with a median survival
of 9 months.
These are a heterogeneous group of diseases characterized by
neoplastic proliferations of immature lymphoid cells that, unlike
the malignant lymphoid cells of AlL, accumulate primarily
outside the bone marrow.
Non-Hodgkin lymphomas represent approximateiy 60/o of aJl
childhood cancers. They occur predominantly in older children
and teenagers and have a strong predilection for males.
Childhood non-Hodgkin lymphomas differ from many
adult cases in that they are:
o Predominantly extranodal in presentation
o As likely to be T:cell lymphomas as B-cell lymphomas
o Highly aggressive, high grade
o Rarely of nodular histology-usually diffuse
Burkitt lymphoma occurs in an endemic form in Africa, with a
presentation as a mass in the jaw or abdomen. Induction of the
B-cell lymphoma in Africa has been linked to a prior Epstein-
Barr virus infection occurring in a young child who has been
immunosuppressed by malaria or some other infection.
a
CTASSIFICATION
1. Histological:
a) Lymphoblastic non-Hodgkin lymphoma: It is usually
of T:cell origin; the cells resemble the L, and L, cells
seen in the usual cases of childhood AIL.
 ONCOLOGY

b) NonJymphoblastic non-Hodgkin lymphoma: The cells DIAGNOSIS

resemble transformed cells of the germinal center and
may be: a Histopathological study, biopsy, or FNAC for specific diagnosis
i. Small non-cleaved cell lymphoma (SNCCL, a Complete blood count (without marrow involvement,
Burkitt and non-Burkitt subrypes, B-cell origin, usually normal)
resembling L, cells). a Chest x-ray
ii. Large cell lymphoma (LCL)-T or B or a Bilateral bone marrow study
a CSF study
indeterminate cell origin.
2. Immunological: a CT scan of chest

a) T:cell origin is demonstrated in almost half of the cases. o Ultrasonography of abdomen

The cells generally have a lymphoblastic morphology a CT scan of abdomen
and contain TdT.
a Biochemical studies:

t b) B-cell origin is demonstrated in most other cases. The o Serum LDH (usually raised)
L cells are non-lymphoblastic and lack TdT activity. o Serum electro\te: calcium, phosphorus, magnesium
c) Non-T, non-B-cell origin is uncommon. r SGPT, serum creatinine
o Viral studies: HAV HBV HCV HIV CMV varicella and
CTINICAL FEATURES HSV screening.
All childhood non-Hodgkin lymphomas are I .O
ranidlv srowins,
" TREATMENT
and thus, symptom duration is short.
L Anterior mediastinal masses, sometimes associated with Generol Supportive Core
pleural effusions, are the most common presentation of
T:cell or iymphoblastic lymphomas. They can produce: Two potentially life-threatening complications are seen in
patients with NHl-superior vena caval syndrome (SVCS)
a. Respiratory distress from airway compromise
and tumor lysis syndrome (TLS).
b. Superior vena cava syndrome In a patient with SVCS, it may be necessary to withheld all
2. Abdominal masses are the most common presentation of the diagnostic procedure and corticosteroid with or without
B-cell or non-lymphoblastic lymphomas. These lympho- radiotherapy is started. It brings about rapid resolution of
mas arise from either abdominal lymph nodes or from SVCS if it is due to NHL. \W/hen patientt condition permits,
intestinal Peyer patches. They can cause: guided biopsy is done.
a. Abdominal distension from a rapidly growing tumor' Otherwise, the patient is first made hemodynamically and
which sometimes produces pain, ascites, and urinary metabolically stable prior to initiation of chemotherapy.
tract obstruction
b. Intestinal obstruction, by serving as the lead point Chemotheropy
[or an intussusception
Consists of various regimens of combination chemotherapy,
). Jaw masses are a common Presentation for endemic
depending on stage and histologic type of disease' Here one
Burkitt lymphoma.
treatment protocol is given:
4. Peripheral lymph node enlargement can be seen with
any Lype of childhood non-Hodgkin lymphoma. teatment groups can be divided into the following categories:
5. Less common presentations include: o Localized (stage I and II) lymphoblastic lymphoma (LL)
a. Obstructing nasopharyngeal tumor o Advanced (stage III and IV) lymphoblastic lymphoma
b. Bone tumor o Localized B-lineage NHL BL, BLL, and DLCL (diffuse
c. Skin tumor large cell lymphoma)
o Intermediate risk
STAGING o BL (B-lineage), BLL (lymphoma), DLCLwith CNS involve-
ment or bone marrow wirh >25o/o lymphoblasts
The St. Jude staging system is easy and important for treatment. o Large-cell anaplastic NHL

Stage I Localized disease of limited bulk, often confined to A. Tlreatment of advanced-stage BL, BLL, DLCL: Lymphome
one side of diaphragm and carrying a good prognosis Malins de Burkitt (LMB)-96
Stage II Regional (except for mediastinal tumor, designated Reduction phase 7 days
stage III)
Stage III Extensive (within the mediastinum or abdomen) COP:
Stage IV Hematogenous dissemination (bone marrow and/ o Inj. Cyclophosphamide 300 mglm2ld IV (over l5 minutes)
or CNS) on day 1.
 ESSENCE OF PEDIATRICS

o Inj. Vincristine I mglm2ld IV flash on day 1. B. Tireatment of NHL (non-B cell lymphoma): BFM-90
. Thb. Prednisolone 60 mglm2ld PO (in divided doses) on fleatment plan of NHL according ro srage of the disease has
days 1-7. been depicted in Fig. 16.2 and has been described below.
tiple IT (age adjusted MTX, HC and Cytarabine) on days t, Induction protocol I
3, and 5 . Tab. Folinic acid I 5 mglm2ld PO bd on days 2 and 4.
o Tab. Prednisolone 60 mglm2ld PO (in divided dose) daily
Induction: Two courses of COPADM started on day 8 for 28 days.
COPADM I (21 days interval):
o Inj. Vincristine 1.5 mglm2ld IV flash on days 8, 15,22,
and 29.
o Inj. Cyclophosphamide 250 mglm2ld IV bd on days 2-4. o Inj. Daunorubicin IV over t hr, 30 mglm)ld on days 8,
r Inj. Vincristine 2 mglmzld IV flash on day 1. 15, 22, and 29.
o Thb. Prednisolone 60 mglm2ld PO (in divided dose) on o Inj. L-asparaginase IV over I hr 10,000 IU/m'? on days 12,
days 1-5. 15, 19, 21,24,27,30, and 33.
o Inj. Doxorubicin (Adriamycin) IV over t hr, 60 mg/mr/d r Inj. Cyclophosphamide 250 mglm')ldlV bd on days 36, 64.
over 6 hr, on day 2. o Inj. CytarabineT5 mglm2ld IV in 100 ml saline on days
o Triple IT (age adjusted MTX, HC and Cytarabine) on 38-41, 45-48, 52-55, 59-62.
days 2, 4, and 6. r Mercaptopurine 60 mglm2ld PO on days 35-63.
o Tab. Folinic acid 15 mglrn')ld PO q6hr on days 2-4. o Methotrexate (IT) 12 mg on days l, 15,29,45, and 59.
COPADM 2 (21 days after completion of COPADM 1): Consolidation (protocol M)
(Begin when absolute neutrophil counr [ANC] > 1000/mm3,
platelet >1 laclmmi) o Mercaptopurine 25 mglm2ld PO on days 1-56.
o Methotrexate (24-hr infusion) 5 glm2 on days 8, 22, 36,
CWE I and.2 (21 days after completion of COPADM 2): and 50.
(Begin when ANC > 1000/mm3, platelet >1 laclmm3) o Methotrexate (IT) 12 mg on days 8,22,36, and 50.
o Cytarabine 50 mg/m'z/d as 12-hr infusion on days 1-5. Reinduction protocol II
o Etoposide 200 mglm2ld over 2-hr on days 2-5.
o Cytarabine high dose 3000 mglm2ld over 3-hr infusion
o Dexamethasone PO 10 mglm2 on day l-27.
on days 1-5.
o Inj.\4ncristine 1.5 mglm2ldlVflashondays 8, 15, 22,and29.
o Granulocytes colony stimulating factor (G-CSF) 5 pg/kg/d
o Inj. Doxorubicin IV over t hr, 30 mglm2ld on days B, 15,
SC, from day 7 until ANC > 3000/mm3.
22, and 29.
o Inj. L-asparaginase IV over t hr 10,000 IU/m, on day 8,
Maintenance therapy: Total courses are 4 at 27 days interval 11, 15, and 18.
Drugs used in each course: e Inj. Cyclophosphamide 1000 mg/m2ld IV bd on day 35.
o High-dose MTX 8 glm2ld over 4 hr IV on day 1. r Inj. CytarabineT5 mglm2ld IV in 100 ml saline on days
r Folinic acid 15 mglm2ld PO 6 hourly, total 12 doses. 38-41,4548.
o Tiiple IT (age adjusted MTX, HC, and Cytarabine) o Thb. Thioguanine PO 60 mg/m2 on days 38 and 45.
o Inj. Cyclophosphamide 500 mgm'zld IV over 30 min on o Methotrexate (IT) 12 mg on days 38 and 45.
days 2-3. Maintenance therapy
r Inj. Doxorubicin (Adriamycin) IV over I hr, 6O mglm2ld
over 6 hr on day 2.
o Mercaptopurine (PO) 50 mglm2ld PO daily.
r o Methotrexate (PO) 20 mglm2ld PO weekly.
Thb. Prednisolone 60 mglm2ld PO (in divided dose) on
days l-5. Total duration 2 years.

I r*rr @
lnduction *.+ Protocol M
ff--+ Maintenance

I ttt*rv
#J- n:ilguction l+ ggllT%fi
Fig. 16.2: Treatment of NHL according to stage of the disease, see text for explanation

rc

Rodiolheropy
Generally not indicated except for life-threatening emergencies,
such as SVCS not responding to initial chemotherapy.
Surgery
Almost no role. It should be employed only in whom there is
good reason that total resection can be achieved (e.g., localized
bowel disease).
PROGNOSIS
Depends on appropriateness of histological and immune-
phenotypical diagnosis and proper protocol. In 1970s, survival
rate was fewer than 20%. Vith the progress in therapy, >75o/o
of children can now be cured (Link and \Teinstein, 2005).
Hodgkin for 5o/o of all childhood cancer. It
disease accounts
appears to arise in lymphoid tissue and spreads to adjacent
lymph nodes area in an orderly fashion. Hematogenous spread
also occurs, Ieading to involvement of liver, spleen, bone marrow,
brain, and usually associated with systemic symptoms.
CLINICAT FEATURES
1. Localized adenopathy, especially in the cervical region, is
the most common presenting symptom. Supraclavicular
or mediastinal lymph nodes are also involved oftenly.
2. Systemic symptoms occur in up to 30o/o of children and
consist of:
(a) Temperature exceeding 100.5"F (38"C)
(b) Drenching night sweats
L
(c) \Weight loss in of l0o/o of body weight in 6 months
excess
3. Depending on the extent and location of nodal and
extranodal diseases, patient may present with features of
airway obstruction, pleural or pericardial effusion, hepato-
cellular dysfunction, bone marrow infiltration, nephrotic
syndrome (rare).
4. Concomitant TB, varicella zoster, fungal infecdon may occur.
crAssrFrcATroN
Classification based on histopathology divides Hodgkin disease
into:
Lymphocyte predominance (most favorable prognosis)
with many lymphocytes and a few Reed-Sternberg cells, a
large (1545 mm in diameter) cell with multiple or mul-
tilobulated neuclei.
Mixed cellularity, in which there are more Reed-Sternberg
with a heterogeneous population of reactive cells.
cells admixed
L;'mphocyte depletion (least favorable prognosis) with
many Reed-Sternberg cells and a few reactive cells.
ONCOLOGY
o Nodular sclerosis, in which dense fibrotic bands separate
islands of reactive cells from Reed-Sternberg cell variants
(called lacunar cells).
STAGING
Approximate stage can be assigned by a combination of imaging
and laboratory tests, but definitive staging often requires explor-
atoiy laparotomy with splenectomy. This surgery is often not
necessary and is only indicated if precise staging is needed to
determine the rype of therapy to be employed. Four stages
are defined; however, for any given stage, patients are further
subdivided into 'A" or "B" depending on the absence (A) or
presence (B) of systemic symptoms.
o Stage I: Disease is confined to one group of nodes.
r Stage II: Disease is present in more than one group of nodes
but is limited to one side of the diaphragm. Approximately
600/o of children have localized (stage I or II) disease.
o Stage III: Disease involves nodes on both sides of the dia-
phragm, with the spleen considered a node.
r Stage fV: There is hematogenous spread to the liver, bone
marrow, lungs, or other non-nodal sites.
DIAGNOSIS
e Biopsy of lymph node: Specific histopathological features
of HD
o Complete blood count
a Biochemical studies:
o Liver function tests
o Renal function test
c Serum copper
o Serum ferritin and transferrin
a Chest x-ray
a CT of neck, chest, and abdomen
a Ultrasonography of abdomen
a Bone scan
a Bone marrow study
a Immunologic evaluation:
o Absolute lymphocyte count
o T:-cell and B-cell count
c T-cell function studies
Thble 16.1 lists features differentiatingNHl from Hodgkin disease.
TREATMENT
Tieatment usually includes combined modality treatment,
which consists of chemotherapy and low-dose (20-25 Gy)
involved field radiotherapy. Standard chemotherapy in HD is
6 cycles of ABVD or 6 cycles of alternating COPP and ABVD
with cure rate up to 90o/o.
Drugs used in ABVD:
ui
o Doxorubicin (Adriamycin):25 mg/m2 IV, Day 1, 15.
ESSENCE OF PEDIATRICS

Differentiation of NHL from Hodgkin's Disease (HD)

At any age, <14 yr (childhood form) Metastatic brain tumors are relatively rare in children as against
predominantly in older 15-30 yr (young adult form) the adults. About rwo-rhirds of the brain rumors of childhood
children and teenager 50-70 yr (older adult forml
between 2 and 12 years ofage are infratentorial. In adolescents
Sex Male preponderance (2:-l ) Male preponderance (2: 1 ) and children below 2 years, tumors occur with equal frequency
Frequency More common (607o) Less common (40%) in the posterior and supratentorial region.
At the time ursease rs generalrzed Local ized
o{ diagnosis (disseminated)
CLASSIFICATION
Common Mediastinal mass + Axial predisposition
presentation cervical or axillary
lymphadenopathy (80%)
resulting in cervical
(80%), mediastinal (50%)
r Infratentorial: Cerebellar astrocytoma (most common pos-
or periaortic lymphnode terior fossa tumor), medulioblastoma (the next common),
Abdominal tumor in
SNCCL t inguinal involvement brain stem glioma, ependymomas.
lymphadenopathy + jaw Less common . Supratentorial: Craniopharyngioma (most common), optic
involvement nerve giioma, astrocytoma, choroid plexus papilloma, leu-
Hepato- Common Rare kemia, metastatic tumor.
splenomegaly
Dissemination Commoner and qriicker Less common
in bone
CLINICAL FEATURES
marrow & CNS
Clinicai features depend on the iocation, type, rare of growth
Svsf emic Iealures Not used [or staging Used {or ctaging
fever, wl lors.
of tumor, and age of the child.
'
\weal i ngr
Symptoms:
Alterations in personality are often the initial symptom,
irrespective of location. The child becomes lethargic, irritable,
o Bleomycin:10 units/m2 IV, Day 1, 15. hyperactive, or forgetful.
o Vinblastine (Velban): 6 mg/m2 IV Day 1, 15. In infratentorial tumors, common symptoms are features of
o Dacarbazine (DTIC): 375 mglmz IV, Day i, 15. raised intracranial pressure, like headache, vomiting, visual
symptoms. Headache is dull, generalized, initially tends to
Drugs used in COPP:
occur at morning. Vomiting is usually in morning without
o Cyclophosphamide: 500-600 mg/m2lV, Day 1, 8. associated nausea and often projectile. Visual symptoms include
o Vincristine: 1.5 mg/m2 IV, Day 1, 8. blurring of vision, squint, fieldloss or complete loss of vision.
o Procarbazine: 100 mg/m2 PO, Day 1, 14. Cerebellar symptoms vary depending on locations; midline
r Prednisone: 40 mg/m2 PO, Day 1, 14. tumors produce truncal ataxia, whereas hemispheric lesions
Repeat once on every 28 da,vs. Total 6-8 cycles are given. cause dysmetria, incoordination, ipsilateral limb weakness.
Supratentorial tumors present with seizures and focal neuro-
Prerequisite to start chemotherapy: Total WBC count logical deficits like hemiparesis. Raised intracranial pressure
>4000/mm3, platelet count >1 laclmm3, and Hb level >B g/dl. is relatively late to present.
Patient who never achieve remission or relapses < 12 months Hydrocephaius (occurs early and is usually obstructive),
after chemotherapy are candidates for myeloablation and bone squint, head tilt, nystagmus, visuai 1oss, bradycardia, irregular
marrow transplantation. pulse, systemic hypertension, ataxia, dysmetria, long tract
Note: signs. Secondary optic atrophy occurs in advanced stage.
1. Radiotherapy to involved nodes plus the nexr node group to Papilledema is common and is preceded by loss of venous
which spread could occur is sometimes used lor localized disease. pulsations, blurring of disc margin.
For patients treated rvith radiotherapy alone, chemotherapy can
sometimes be used as successful salvage therapy for relapse.
DIFFERENTIAL DIAGNOSIS
2. Combination chemotherapy is usually indicated for all stage IV and
most stage III patients as rvell as lor parients u'ho have localized Brain abscess, granuloma, subdural hematoma.
but bulky disease, such as a large mediastinal mass. Chemotherapy
often is given in conjunction with radiotherapy. INVESTIGATIONS
3. Prognosis is good and varies lrom >900/o cure ofstage t disease to
an approximately 60-70o/o cure of stage IV disease. Most brain tumors are diagnosed by CT scan or MRI with or
4. Patient who relapses after 12 months usually respond to additional without contrast. Skull radiograph and conventional angiograph
chemotherapy or irradiation or both. are rarely necessary.
 I

ONCOLOGY

TREATMENT Table 16.2: Classification and Treatment of Childhood Histio,

cytosis
. Surgery is indicated for the tumors whose location permits
resection:
Langerhan cell Familial Malignant
o Resectable tumors include cerebellar and cerebral tumors. h istiocvtosis erythrophagocytic histiocytosis
o Brain stem gliomas usually are not resectable, their loca- lymphohistiocytosis Acute monoc).tic
(FEL) lnfection- leukemia
tions makes biopsy risky.
associated
o Radiotherapy plays major role for management of tumors hemophagocytic
syndrome (IAHS)
of all locations.
r Chemotherapy is a recent addition. It prolongs survival in Langerhan cells with
Birbeck granules
Morphologically Neoplastic
normal reactive proliferation of cells
high-grade astrocytoma and increases cure rate in medullo- macrophages with characteristics
blastoma. with prominent of monocyte
erythrophagocytosis macrophage or their
precursors
PROGNOSIS
o
i!#iffi i:il:fi liirl
jlillii;
:il':*$lffi ffi i ji;;:iiiiitiilitiili
Prognosis depends upon rype of tumor, stage, location, and Local therapy for Chemotherapy, Antineoplastic
treatment modalities available. isolated lesions allogenic chemotherapy-
o The cerebellar astrocytoma has the best prognosis; 5-year
(intralesional bone marrow anthracycl ine,
corticosteroid), transplantation vinblastin, etoposide
survival rate is >900/o. (experimental)
chemotherapy for
Prognosis is good for medulloblastoma occurring in children disseminated disease
older than 4 yeag smaller in size, and that have not spread;
5-year survival rate is 30-40o/o.
Prognosis is poor for brain stem gliomas (5-year survival
CTINICAL FEATURES
<5o/o) and for medulloblastoma, which occurs in young Class I histiocytosis: - This includes the clinical entities of pre-
children and are larger in size and spread into the CSF. vious (i) eosinophilic granuloma, (il) Hand-Schulier-Christian
Due to cranial irradiation, there may be impaired cognitive disease, (iii) Litterer-Siwe disease.
behavior, verbal performance, and academic achievements; i. Eosinophilic granuloma is a benign and self-limited
there may also be abnormalities in linear growth and endo- disease. It is uncommon in infants. Eosinophilic granu-
crine disturbances, such as hypothyroidism. loma is usually limited to a single bone lesion that is
painful and located in the femur or skull.
ii. Hand-Schuller-Christian disease is a more chronic and
extensive disease. It is also uncommon in infants. The
Histiocytosis syndrome refers to a heterogeneous group of disease commonly involves the bone and skin. Occasion-
disorders characterized by a prominent proliferation/accumu- ally, its presentation involves a classic triad of:
lation of cells of the monocyte-macrophage system of bone a. Skeletal lesions
marrow origin. b. Diabetes insipidus
Three classes ofchildhood histiocytosis are recognized based c. Exophthalmos
on histopathologic findings (Thble 16.2). The most well-known
iii. Litterer-Siwe disease, an acute disease that often terminates
childhood histocytosis, previously known as histiocl.tosis X,
in fatal condition, occurs in infants.
constitutes class I histiocytosis. The name Langerhans cell
histiocytosis (LCH) has also been applied to the class I histio- a. This disease involves the skin, liver, spleen, lymph
nodes, bone marrow and lungs.
cytosis. The Birbeck granule, a tennis racket shaped bilamillar
granule seen in cytoplasm of cells by electron microscope are b. Bone lesions are less striking than in the other forms
diagnostic of LCH. of histioc)'tosis X.
Class l\' hisfrissytsses ane ghan'agterized b7 acctrsrrtrlatiorr c. A poor outcome is heralded by dysfunction of one or
of antigen presenting cells (i.e., macrophages). The two major mot. olth" follo*itrg' l,rngr, li r.a bo.,. marrow.
"nd
diseases in this class are familial erythrophagocytic lympho- Class II and class III histiocytosis: The major forms of class
histiocytosis (FEL) and infection-associated hemophagocytic II histiocytosis, FEL and IAHS, have a remarkably similar
svndrome (IAHS). The mixed cellular lesions of both class I presentation. Distinction can be made only by a positive
and class II histiocytosis suggest that these may be disorders family history, and affected children are always younger than
of immune regulation. 4 years of age in FEL. IAHS may present at an older age.
In contrast, class III histiocytoses are unequivocal malignan- General signs and symptoms are fever, weight loss, irritabiliry
cies of cells of monocyte-macrophage lineage. Acute monocytic hepatosplenomegaly, severe immunodeficiency, and symptoms
ieuliemia and true ma,lignant histioc).tosis are included in this class. of CNS involvement (with an aseptic meningitis).
 q

ESSENCE OF PEDIATRICS

The features of class III histiocytosis are those of acute

d. Epidural tumors arise from the posterior growth
monocytic leukemia.
in dumb-bell fashion of abdominal or thoracic
tumors. They grow through the neural foramina
INVESTIGATIONS into the epidural space, where they compress the
spinal cord, producing back pain and symptoms of
Tissue biopsy of skin and bone lesions is diagnostic. cord compression. Patients who have this presenta-
CBC, liver function tests, coagulation studies, skeletal tion require rapid evaluation with imaging studies
surveys, chest x-ray, urine osmolality should be carried out. to define the epidural component of the tumor
and then therapy to prevent cord ischemia and its
neurologic sequelae.
2. Metastases are common at diagnosis and often cause the
symptoms that lead to the diagnosis of neuroblastoma.
Itis a malignancy of neural crest cells, which in the course of a. Nonspecificsymptoms:
their normal development, give rise to the paraspinai sympa- i) lVeight loss
thetic ganglia and the adrenal medulla. ii) Fever
Neuroblastoma is the second most common solid tumor of
b. Specific symptoms:
childhood. Only brain tumors are more common. Neuroblas-
toma occurs predominantly in infants and preschool children, i) Bone marrow failure
with more than half of the patients younger than 2 years of ii) Cortical bone pain, resulting in a limp if present
age and one-third younger than I year. There is a slight male in the lower extremity
predominance. Occasionally it is congenital. iii) Proptosis and periorbital ecchymoses from
retrobulbar and orbital infiltration
iv) Liver infiltration, causing hepatomegaly
CTINICAL FEATURES v) Distant lymph node enlargement
vi) Skin infiltration, causing palpable subcutaneous
Clinical features are extremely variable and refect the widespread
nodules
distribution of neural crest tissue. Approximately one-half of
newly diagnosed patients with neuroblastoma have distant 3. Remote effects (paraneoplastic syndrome) are occasion-
metastases at diagnosis and as consequence, they appear ill. ally seen.

1. Primary sites a. \Tatery diarrhea may occur in patients who have dif-
ferentiated tumors that secrete vasoactive intestinal
a. Abdominal tumors are the most common presenta-
peptide (VIP).
tion, accounting for 70o/o of the cases; half arise from
extra-adrenal tissue and half from the adrenal medulla.
b. Flushing, excessive sweating, hypertension due to
catecholamine release.
Presenting features are:
c. Acute myoclonic encephalopathy is a rare manifes-
i) Abdominal mass, which often displaces the
tation associated with an excellent prognosis. They
kidneys anterolaterally and inferiorly
present with:
ii) Abdominal pain
iii) Systemic hypertension, if there is compression of
i) Opsoclonus (rapid eye movements)
the renal vasculature
ii) Myoclonus
iii) Cerebellar ataxia.
b. Thoracic tumors are the next most common presenta-
tion and are located in the posterior mediastinum,
presenting features are: STAGING
i) Respiratorydistress
r Stage I and II tumors must not be large enough ro cross
ii) Incidental finding on a chest radiograph
the midline.
c. The presentation of head and neck tumors involves a Stage III tumors cross the midline.
palpable tumors that sometimes produce Horner a Stage IV: Any primary tumor with dissemination to distant
syndrome (usuaily in cervical neuroblastoma), which ofgans.
consists of: Stage WS: This is a special type of metastatic tumor that
i) Miosis occurs in infants and carries a much better prognosis than
ii) Ptosis the usual stage IV tumors. Stage IVS tumors are small
iii) Enophthalmos primary tumors that occur in young infants who have
iv) Anhidrosis metastases limited ro the skin, liver, and bone marrow but
v) Heterochromia of the iris on the affected side not cortical bone.

3Z
 ONCOLOGY

DIAGNOSIS . Stage:
,r Stages I and II parients and stage IVS infants have a good
Diagnosis of neuroblastoma is established under one of the
prognosis.
following circumsrances:
o Most stages III and IV patienrs have a poor prognosis.
o Histopathological diagnosis made from rumor rissue by
I light microscopy with or without immunohistochemistry,
l
or increased urine/serum catecholamines or metabolites.
o Bone marrow showing metastatic cells and urine/serum
catecholamines or metabolites are increased. RB is a rare malignanr rumor of the embryonic neural tissue
The following investigations are also necessary (to see the extent within the retina. Involvement may be unilateral and non-
of disease and distant metastasis): hereditary (60Vo), unilateral and hereditary (AD l5o/o), and
bilateral and hereditary (AD 25%). Familial cases are generally
I o X-ray chest or CT scan of chest multifocal and bilateral, whereas non-familial
I cases tend ro
I o CT scan/MRI of primary area have unifocal and unilateral involvement.
t a Bone marrow study from two sites; bone scan.
l a Urinary/serum catecholamine metabolites (VMA/HVA)
, a Radio-isotope scanning (MIBG scan) CtINICAI FEATURES
v a Tumor markers are extremely useful in evaluating children
l The most common sign of RB is the white or catt eye reflex
who have neuroblastoma:
) (leukokoria), the appearance of the yellow-white rumor mass
l o Urinary markers: Catecholamines are elaborated by most
l behind the lens. Visual impairment, srrabismus, pseudohy-
tumors and are useful for diagnosis, following up on re-
I pophyon, vitreous hemorrhage, proptosis, signs of increased
t sponse to therapy, and detection of recurrence. Particu-
larly useful markers whose urinary excretion levels can be
intracranial pressure and ocular pain-if secondary glaucoma.
Bone pain, hyphema, orbital infammation and pupillary
measured include:
irregularity are present in very advancing disease.
l Vanillylmandelic acid (VMA)
I - At diagnosis, mosr rumors are localized to the globe.
Homovanillic acid (HVA).
I - Metastasis occurs late and spreads to the:
I o Serum markers: Elevation of the following serum mark-
i. Meninges via the opric nerve
ers is often associated with a poor prognosis.
Ferritin
ii. Bone marrow and cortical bone via blood.
- la61x1s dehydrogenase Fundoscopic examination under general anesthesia, orbital
- USG and CT scan to evaluate intraocular extent and exrraocu-
lar spread; bone scan, CSF for rumor cell and bone marrow
TREATMENT sampling are necessary if indicated.

a Surgery alone often suffices for stages I and II patients.

a Spontaneous regression without any therapy is common in
stage IVS infants.
Aggressive multi-agent chemotherapy regimens can often
produce dramatic tumor regression in stages III and IV
disease. The following drugs are used: cyclophosphamide,
doxorubicin, cisplatin, etoposide.
a Radiotherapy for progressive hepatomegaly.
a Autologous bone marrow transplantation has been used for
stages III and IV patienrs who have a poor prognosis. Up
to 50o/o of these patients are cured. Their chances of cure
appear to be increased if they are offered post-transplant
cis-retinoic acid, an agenr rhar is used for terminal differ-
entiation and death of any neuroblastoma cell that survived
the transplant procedure.
PROGNOSIS
Prognosis depends on the following factors:
o Age: Infants younger rhan I year ofage have rhe best prognosis.
TREATMENT
Initial therapy for retinoblastoma is dependent on the extenr
of the disease. Trearmenr should be carried out in specialized
center. The goal is to maximize preservation of usefulness.
Therapeutic modalities include:
o Systemic chemotherapy
o External beam radiotherapy
o Local ophthalmic therapy
o Enucleation
Chemotherapy is used in a preoperative (neoadjuvanr) serring.
Three drug combination regimen (Vincristine, Carboplatin,
and Etoposide) given every 28 days, for 6 cycles.
Radiotherapy is indicated to rreat any residual orbital disease
and manage the remaining eye in lateral cases along with other
physical modalities (e.g., laser therapy, cryotherapy).
Enucleation of the eye is recommended when there is no
chance for usefulness even if the entire rumor is destroyed. It
is also indicated if chance of metastasis is high.
 ESSENCE OF PEDIATRICS

PROGNOSIS INVESTIGATIONS

I. The prognosis is excellent for those patients whose disease a CBC

is limited ro rhe eyes. a Biochemical-LFTs, RFTs
2. Dissemination carries a poor prognosis. a Urinalysis
O USG abdomen/CT abdomen
a X-ray chest/CT thorax
a FNAC of abdominal mass
a Bone scan-in clear cell sarcoma
a CT brain, if metastasis is suspected
Neoplastic embryonal renal cells of the metanephros give rise to
this kidney tumor. The classic triphasic Vilm tumor, considered
to be favorable histology (FH), is made up of varying proportion TREATMENT
of three cell types-blastemal, stromal, and epithelial; unfavorable
Surgery, chemotherapy, and radiotherapy all are employed.
histology (UH) is characterized by presence of focal or diffuse
There is controversy about initial surgery or neoadjuvant che-
anaplasia, clear cell sarcoma, and rhabdoid tumor.
motherapy followed by surgery, then adjuvant chemotherapy.
\7ilm tumor accounts for 60/o of all childhood cancers. It
There are points in favor of both groups. 'Whatever the timing,
is predominantly a tumor that occurs during the first 5 years
it is always expected to do total nephrectomy, if possible.
of life. Occurrence is equal in males and females.
Chemotherapy: Favorable histology (FH)
CtINICAt FEATURES Stage I and II: Surgery + Chemotherapy (AMD, VCR) =
18 weeks.
Most children with newly diagnosed Vilm tumor have local-
ized disease and appear well. Stage III andl% Surgery + Chemotherapy (AMD, VCR, and
1. Abdominal mass is by far the most common presenta- ADR) + Radiotherapy = 24 weeks.
tion. The mass is usually asymptomatic and detected on Note: AMD, Actinomycin D (45 pg/kg IV); VCR, Vincristine (0.05
routine physical examination or by a caretaker bathing the mg/kg IV); ADR, Adriamycin (1.0 mg/kg IV). Radiotherapy and drug
patient. On imaging studies, the mass characteristically doses are different in different schedules.
occurs within the kidney and displaces and distorts the
renal collecting system. Radiotherapy is timed shortly after surgery, with the aim of
2. Hypertension (25o/o) may be related to elaboration of eradicating tumor cell that may have spilled during surgery.
renin by tumor cells or, less frequently, to compression
of the renal vasculature by the tumor. PROGNOSIS
3. Hematuria is not common but, when present, ls more
often microscopic than gross. Patientswith localized disease and a favorable histology have
4. Abdominal pain, especially with hemorrhage into the a >90o/o chance of survival. Bilateral advanced stage disease
tumot is less commonly present. There may be associated has guarded prognosis.
fever and anemia.
5. Associated abnormalities that occur in a few patients
include:
a. Aniridia: Children who have both sporadic aniridia
and the chromosome deletion have an almost 507o Ewing sarcoma is a highly malignant tumor occurring usually
chance for development of \7ilm tumor. in children and adolescents and is associated with both sys-
b. Hemihypertrophy: Some children with hemihypertro- temic and localized symptoms and onion peel radiological
phy have the Beckwith-\(idemann syndrome, which shadow.
also includes visceromegaly, omphalocele, macroglos-
sia, neonatal hypoglycemia, and increased risk of
developing hepatoblastoma, adrenal cortical carcinoma
CTINICAL FEATURES
as well as
tVilm tumor.
o Children, adolescents, and young adults are affected (usually
c. Ambiguous genitalia. 5-25 years).
r Diaphysis of long bones particularly tibia, femur, fibula, and
ulna are affected. Among flat bones, pelvic bone involve-
DIFFERENTIAT DIAGNOSIS
ment is common.
Neuroblastoma, hydronephrosis, renal ryst, renal cell carcinoma, o Pain and tenderness with progressive severity is the com-
mesoblastic nephroma (common in neonate). monest symptom.

E
 ONCOLOGY

o Diffuse swelling over bone with local rise of temPerature

and shiny skin over midshaft region.
. Systemic manifestations are very common' particularly fever Itis the commonest and highly malignant primary bone tumor
and anemia, and may mimic acute osteomyelitis' characterized by the invariable formation of neoplastic osteoid
o Recurrence is very common. and tumor tissue.
o Metastasis occurs in other bones like skull, vertebrae, and
Tlpes: On the basis of bone formation and destruction:
ribs in addition to lungs through blood stream'
o Osteoblasric/osteoscleroric I

o Osteolytic
DIFFERENTIAL DIAGNOSIS o Mixed osteoblastic and lytic
o Osteosarcoma: Occurring in same age group, metaphy-
o Thlangiectatic
seal involvement, radiologically have sunray appearance'

.
Codmann triangle is more typical, alkaline phosphatase
levei may be high, bioPsY helPful'
Osteomyelitis: Systemic symptoms like fever and toxemia
CtINICAt
o
FEATURES

Age and sex: Common within 10-20 years' common in

Hl
more common, pain more severe from very beginning; male.
metaphyseal involvement' periosteal bony reaction and o Common sites: Metaphysis of growing long bones, mostly
sequestration may be seen. around knee, upper humerus, lower tibia, and lower
radius.
o Pain: Earliest and dominating symptom' Initially mild'
INVESTIGATIONS intermittent, then gradually increasing in intensiry which
becomes constant and severe.
a CBC (t leukocytosis, .L glY., f,Sn)'
a CXR to exclude any metastatic lesion'
o Swelling: Diffuse metaphyseal swelling may look fusiform
with raied local temperature and shiny skin with overly-
a X-ray of bone shows marked destruction of bone cortex
ing engorged veins. The tumor is moderately tender' May
associated with reactive new bone formation in layers under
present with fracture (pathological).
the raised periosteum producing onion peel appearance in
diaphyseal area. The bone may look osteopenic and moth
o In 50% cases, history of trauma is present'
eaten.
Bone scan: Increased radio uptake of isotopes may be seen' INVESTIGATIONS
Easy to localize the metastatic lesion.
Biopsy: Mandatory before starting treatment' Round cells o CBC: J UUy., very high ESR, leukocltosis' Alkaline phos-
of malignant characterwith clouding of cells with minimum phatase level is high.
'CXR,
intercellular substance may be seen on microscopy' . To ,." 1rr.tg metastasis (i'e', cannon ball
appearance).
X-ray appearance of bone: Metaphyseal regions of
TREATMENT growing^ Lo.t., involved' Combination of bone
i"..rtr.iio.t and "r.
formation' areas of osteoporosis, ill-
As the tumor is aggressive, multidisciplinary treatment by
defined clouds ofincreased density and perforated cortex
oncologist, radiotherapist and orthopedic surgeon is essential'
twith zurgery alone, prognosis is poor' It is highly radio-sen- may be present.
Periosteal reactions: There is reactive new bone forma-
sitive; radiotherapy gives the initial dramatic results' It melts
tion subperiosteally at the junction of the lifted periostium
like wax, but recurs very quicklY'
and the normal bone (triangular in shape)-this is called
o Pre-operative radiotherapy and multidrug chemotherapy (VCR, Codman triangle.
adriamycin, and cyclophosphamide given concurrently'' Sunray .pp""r.n." due to deposition of osteoid along
o Operative-wide excision or amPutation' new vessels.
o Postoperative chemotheraPy. Soft tissue shadow and pathological ftacture may be

seen.
i Bone scan helps in localizing the lesion' showing the extent
PROGNOSIS
of metastasis.
The overall 5-year survival for the localized Ewing sarcoma Biopsy: Spindle-shaped cells with mitotic character with
I treated rvith surgery, radiation, and multi-agent chemotherapy malignant osteoid in between cells'
is approximat ely 55-70o/o. Patients who present with metastasis MRI and CT scan: Helpful in definitive operative plan'
at diagnosis have a 5-year survival of 20-30o/o' particularly in limb salvaging surgery.
J

d I
/:
ESSENCE OF PEDIATRICS

DIFFERENTIAT DIAGNOSIS Two types of tumors occur:

Subacute or chronic osteomyelitis, Ewing sarcoma, scur\y, 1. Hepatoblastoma, the most common type, occurs within
cellulitis. 18 months of age.
a) It is usually unifocal.
b) It often presents with large asympromatic mass (liver),
TREATMENT
weight loss, abdominal pain associated with throm-
Surgery: Early definitive ampuration is to be done to remove bocytosis, and merastasizes primarily to the lungs and
primary tumor. Resect large pulmonary metastasis. Palliative lymph nodes.
amputation may also be done. c) Hepatoblastomat ourcome depends on whether it can
Chemotherapy: Various protocols are available. All employs be completely resected (can be resected in 50-G0o/o
very high dose MTX. POG protocol utilizes 6 drug regimen cases).

(methotrexate, doxorubicin, cispiatin, cyclophosphamide,
bleomycin, and actinomycin D) in a total 42 weeks regimen
given pre- and or/postoperatively.
a Postoperative chemotherapy
a Immunotherapy by BCG/allogenic sarcoma tumor cells.
a Ideal treatment: Neoadjuvant multi-agent chemotherapy
with limb preservation surgery.
PROGNOSIS
r Age <10 years, size of the tumor >1/3 of limb, axial skeleton
involvement, proximal location in a limb, merasrasis ar
diagnosis, incomplete resectability and finally poor response
to therapy are poor prognostic indicators.
r Non-metastatic tumor treated with adjuvant chemotherapy
has a survival rate of 60-800/o.
o Metastasis at diagnosis lowers the value to 10-40o/o.
d) Hepatoblasroma that is not resectable may sometimes
be sufficiently reduced in size by chemotherapeutic
agents (e.g., doxorubicin and cisplatin) to permit
curative resection.
2. Hepatocellular carcinoma, which is less common rhan
hepatoblastoma, is seen in older children and teenagers
(about 12 year at diagnosis).
a. It is often multifocal and is less curable than hepato-
blastoma; presenrs with abdominal mass, abdominal
pain, and weight loss.
b. Hepatocellular carcinoma may be related ro a pre-
ceding hepatitis B or C infection, just as is the case
in adults. Because of multicentric origin, complete
resection of this tumor is accomplished in only
30-40% cases. Chemotherapy including cisplatin,
doxorubicin, etoposide, 5-fuorouracil causes some
improvement.

1. Behrman RE et al. Textbook of Pediatria 18,', ed. Singapore: Har-

Liver tumors accoullt for only lo/o of childhood cancers.
However, they are the most common epithelial malignancy
of chlldhood
CtINICAI. FEATURES
'lle presentation of liver tumor is that of a mass, occurring
primarily in the right upper quadranr. These rumors often
elaborate cr-fetoprotein (AFP), which is a rumor marker useful
for diagnosis, (AIP is increased in70Vo of children with hepato-
blastoma and 50o/o of children with hepatocellular carcinoma).
CT/MRI can detect extent of tumor.
court Asia Pte Ltd., 2007.
Arya LS. Lymphoma in children. Paper presented in 1st interna-
tional seminar and CME programme on Pediatric Hematology and
Oncology in Dhaka in 2001.
). Firkin F (ed). de Gruchy's Clinical Hematologlt in Medical Practice
5'r' ed. Delhi: Oxford Universiry Press, 1989.
4. Parthasarathy A (ed.) . IAP Textbook of Pediatrics 4,h ed. New Delhi:
Jaypee Brothers, 2009.
Dworkin PH (ed.). Pediatrics 4'r'ed. Philadelphia: Lippincon Vil-
liams and \Wilkins, 2000.
Custleberry RP Biology and treatmenr of neuroblastoma. Pediatr
Clin North Am 1997;44:919-37.
7 Lanzkowsky P. Manual of Pediatric Hematologt and Oncology 4,h
ed. London: Elsevier Academic Press, 2005.
 CHAPTER 17
Connective Tissue Diseases

Chopter Contents
Rheumatic fever...........,,...,...,,........,...................................,,..317 Mlixed connective tissue disease.... .326 Vasculitis syndrome ,............................. .....,...,.....................327

.luvenile idiopathic arthritis .................... ....... Miscellaneous conditions -,,f,r .nf,lri i, tU*Oatt .326 Differential diagnosis of rheumatic diseases....,.............327

Systemic lupus erythematosus....... ....................................323 Lyme disease......... .326

Henoch-Schonlein purpura......,..........................................325 5r leroderrna........... .321

o Chorea (Sydenhamt) occurs in 70-30o/o of the patients.

Often insidious in onset, characterized by random, jerky
movements and may be associated with emotional lability,
Rheumatic fever is a multisystem, nonsuppurative inflammatory
hypotonia, incoordination, poor school performances, facial
disease involving the joints, heart, CNS, skin or subcutane-
grimacing, personality changes-increases in stress, disap-
ous tissue triggered by a group A B-hemolytic streptococcal
pears during sleep.
(GABHS) infection of the upper respiratory tract.
Patients in whom rheumatic fever develops have a marked r Clinical maneuvers to elicit features of chorea include
tendency to sufler recurrent attacks after subsequent GABHS (l) demonstration of milk-maidt grip, (lz) spooning and
infections of the upper respiratory tract. Recurrence could pronation of the hands when the arms are extended, (iii)
be prevented by continuous antistreptococcal prophylaxis. wormian darting movements of the tongue upon Protru-
Rheumatic fever is a potentiaily serious disease, because it may sion, and (iu) exam of handwriting to evaluate the fi;te
cause permanent damage to heart muscie and valves (rheumatic motof movements.
heart disease). ,r Chorea runs a self-limited course of 6-13 weeks dura-
Bacteria are responsible for 10% ofcases ofpharyngitis, and tion, and recovery is complete.
3o/oof l:acrerial pharyngitis could give rise to rheumatic fever. Less common findings include subcutaneous nodules
The peak incidence of initial and recurrent attacks of rheumatic (small, painless swellings found overlying bony prominences)
fever is between 5 and 15 years of age, which coincides with and erythema marginatum (a pink, evanescent rash over
the high frequency of GABHS infections in this age group. the trunk [bathing suit area]).

CtINICAt FEATURES
DIAGNOSIS
The revised,/ones criteria for diagnosis of an initial attack of
Polyarthritis, usually with fever, is the presenting finding
rheumatic fever:
in about 75o/o of patients. The arthritis chiefly affects large
joints (commonly eibow knee, ankle, and wrist), is char-
acteristically migratory, and is painful out of proportion
Carditis Fever
to objective findings of redness and swelling. There are no
Polyarthritis, migratory Arthralgia
sequelae.
Chorea Llevated ESR, C-reactive protein
Carditis is pancarditis, invoiving pericardium (rub, pericardial
Erythema marginatum Prolonged P-R interval on an ECC
effirsion), myocardium (tachycardia, conduction defects, cardi-
omegaly), and endocardium (murmurs); occurs in about 50olo Subcutaneous nodules
of the patients and may be as)..rnptomatic, unless pericarditis or
PLUS
heart failure is present. A pansystolic, blowing mitral murmur
is the hallmark. Less cofirmon is a diastolic aortic murmur Eaidence of preceding GABHS infection: Positive throat
heard along the left sternal border. Murmurs may remain but culture or rapid streptococcal antigen test or elevated or rising i

often disappear if rheumatic fever does not recur. streptococcal antibody titer.
ESSENCE OF PEDIATRICS
Principle of diagnosis of initial attack of rheumatic fever:
o 2 mqor criteria, plus evidence of preceding GABHS infec-
tion, or
o I major and 2 minor criteria, plus evidence of preceding
GABHS infection.
Exceptions to Jones criteria:
In the two special circumstances, listed below, the diagnosis
of rheumatic fever is acceptable without 2 major or 1 major
& 2 minor criteria; requirement for evidence of a preceding
GABHS infection can be ignored:
1. Chorea, if other cltases of cborea are excluded.
2. Insidious or late-onset carditis ttitb no otber explana-
tion. These patients come to medical attention months
after the onset of rheumatic fever (Echocardiogram may
be done).
Recurrent rheumatic fever: Recurrence of rheumatic fever
should be considered in patients with prior rheumatic fever or
rheumatic heart disease and with evidence of a recent GABHS
infection with I major or 2 minor criteria.
TREATMENT
l Bed rest: Long periods of bed rest are not necessary for
most patients. Bed rest is indicated for the therapy of
patients with carditis and CCE but prolonged bed rest is
usually unnecessary. Patient should be kept in bed until
ESR becomes normal and CCF has been controlled.
2. Streptococcal pharyngitis: Following treatment should
be started: Penicillin V 250 mg 6 hourly for 10 days,
or single IM Inj. Benzathine penicillin G (6 lakhs for
children <60 ib, and 12lakhs for children >60 lb).
Erythromycin 40 mglkgld may be used for 10 days
in patients who are allergic to penicillin.
.). Symptomatic:
(a) In arthritis only andlor carditis uithout cardio-
megaly:
i. Salicylates 100 mg/kg/d for 2 week, rhen 75
mg/kg/d for another 4-6 weeks.
ii. Hr-blocker may be given.
(b) Carditis utitb cardiomega$r or CCF:
i. Prednisolone 2.5 mglkgld in 2 divided doses
for 2-3 weeks (depending on clinical and lab
response), then taper over 2 weeks. Salicylates 75
mg/kg/d should be added at the time of tapering
(i.e., at 2-3 weeks) and continued for about 6
weeks.
ii. CCF should be treated by conventional regimen-
Or, diuretics, and small-dose digoxin should be
given. -fi4ren frusemide and digoxin are given,
or only frusemide is given for >5 days, potassium
should be added orally 3-5 mmol/kg/d.
Table 17.1: Dose and Duration of Antibiotics in Secondarv
Prophylaxis Against CABHS
Benzathine lM
12 lakh units Duration is 5 yr after the most
penicillin Or every j-4 wk recent dttd(k, or when they reach
birthdaY' whichever is
ffii].
Penicjllin V Or 250 mg PO. Rheumatic fever with carditis-
twice daily duration 10 yr or till 30 yr of age.
whichever is longer
Erythromycin 250 mg PO Rheumatic hearl disease (valvular
twice daily disease) duration lifelong.
(c) Chorea:\hen occurs as isolated manifestation, no and-
inflammatory drug is given. In early course, sedative is
helpful; phenobarbital 15*30 mg every 6-8 hour PO
is the drug of choice, if ineffective, then haloperidol
(0.01-0.03 mg/kg/d bid) or chlorpromazine (0.5 mg/kg
every 4-5 hour PO) should be given.
(d) Erytbema rnarginaturu and subcutaneous nodules:
No specific treatment.
PREVENTION
Secondary prophylaxis: Refers to the prevention of coloniza-
tion or infection of upper respiratory tract with GABHS in
individuals who have already had a previous attack of acute
rheumatic fever (see Table 17 .l for dose and duration of anti-
biotics used in secondary prophylaxis).
Primary prophylaxis: Refers to antibiotic treatment of the
streptococcal URTI to prevent an initial attack of rheumatic
fever. PenicillinY 250 mg 6 hourly for 10 days or single IM Inj.
Benzathine penicillin G (dose has been described earlier).
Juvenile idiopathic arthritis (JIA) is the most common rheu-
matic disease in childhood.
ctAssrFrcATtoN
The term juvenile rheumatoid arthritis (lRA) had been used
extensively in the United States and Canada as the preferred
term. The European League Against Rheumatism (EULAR)
used the term juveniie chronic arthritis (JCA). In order to
unif, the disease in a universally accepted manner, a task force
was established by the International League Against Rheuma-
tism (ILAR). This group proposed new set of criteria for JIA
in 1995. In this classification, each subset is ciearly defined
with description of the required characteristics, exclusion and
inclusion criteria. \fHO endorsed the proposed classification
in 1999. Table 17.2 lists characteristics of the ACR, EULAR,
and ILAR classifications of childhood arthritis.
 t
CON NECTIVE TISSUE DISEASES

Table 17.2: Characteristics of the ACR, EULAR, and ILAR Types of JIA (ILAR Clqssificotion)
Classifications of Childhood Arthritis
o Rheumatoid factor (RF)-positive polyarthritis
o RF-negative polyarthritis
Onset types Three Six Seven o Oligoarthlili5-s116nded and persistent
Age at onset of arthritis <1 6 Year Year
<1 6 <i 6 Year o Systemic onset JIA
Duration of arthritis >6 wk >3mo >6 wk o Enthesitis-related arthritis (ERA)
lncludes juvenile ankylosing No Yes Yes r Psoriatic arrhritis
spondylitis e Llndifferentiated
No Yes
lncludes psoriatic arthritis Yes
Oligoarthritis: Four or fewer joints are affected in the oli-
lncludes "lBD" No Yes Yes
goarthritis type. It is the most common form of JIA; about
I ncludes reactive arthritis No No No half of ali children with JIA have this type. Knees, ankles, and
Fxclusion of other diseases Yes Yes Yes elbows are large joints of lower extremities and other joint such
as wrist, spine, and even offingers or toes can also be affected.
Arthritis that remains confined to four joints is designated as
Diogt'ostic Criterio for lhe Clqssificolion persistent oligoarticular JIA. A child who develops active
of :tRA arthritis of five or more joints after the first 6 months of disease
is considered to have extended oligoarticular JIA.
1. Age of onset <16 year.
2. Arthritis (swelling or effusion or presence of two or more Polyarthritis: The common of onset of polyarticular disease
age

of the followings signs: limitation of range of motion,
tendelness or pain on motion, and increased heat) in
one or more joints.
3. Duration of disease 6 weeks or longer.
4. Onset type defined by type of disease in fir'st 6 months:
Polyarthritis: 5 or more inflamed joints.
Oligoarthritis: <5 in{lamed joints.
Systemic onset: Arthritis with char-acreristic fever'
5. Exclusion of other forms of juvenile arthritis.
For subgroups of juvenile rheumatoid arthritis,
Tal:le 17.3.
Differential diagnosis: Acute rheumatic fever, SLE, acute leu-
kemia, reactive arthritis, ankylosing spondylitis. See Thble 17'4
for features differentiating JRA from rheumatic fever.
(30-35o/o) is 1-5 years in the first 6 months of disease. This
subrype includes children with RF-negative disease (10-30% of
JIA patients) and RF-positive disease (5-tOo/o of JIA patients).
In poiyarticuiar disease, both large and small joints are sym-
metrically involved with time . Older teenage girls with polyar-
ticular disease often have a positive rheumatoid factor'
Systemic onset JIA: Boys and girls equally have systemic
onset JIA (20o/o). These children present lr'ith daily high
spiking fever that may persist for weeks or even months. Pale
refer pink rash may appear on the chest, thigh, and some times,
on other parts of the body. Both large and small joints are
affected. Almost all children with this type of JIA are negative
for both RF and ANA. This group of children has the worst
long-term prognosis.

Table 17.3: of Juvenile Rheumatoid Arthritis

Percentage of 20 30 5,1 0 30-40 t0-15 1 0-20

IRA patients
Sex 90% girls 80"1' girls B0% girls 90% boys 60% boys

Age at onset Any Late chi ldhood Early childhood Late chi ldhood Any

An,v, multiple Any, multiple Few l-arge joints, knee, Few large joints; hip girdle Any multiple
Joints
ankle, elbow

S.:croilrrtis No Rare No Common No

-': ,,_:: Rare No 30% chronic iridocyclitis 1 O-2O"h acute iridocycl itis No

\ eqatiIe 1 00% Negative Negative Negative

lr , 9On/o Negative Negative

,severe arthritis Ocular damage 107o Subsequent Severe arthrttls Z5Y"

:;,):-. Polyarthritis 20% spondyloarth ropathy
ESSENCE OF PEDIATRICS

Table 17.4= Differentiating Features between Rheumatic Fever and JRA

Onset Sudden lnsidious

Age at onset 5-1 5 years (both sexes) 1 year or older (both sexes), during childhood
and adolescence (mostly girls)
Type: ol loinls involred Joints (knee, ankle, elbow, wrist), Small, also large joints. Both symmetrical and
usually asymmetrir al asymmetrical involvement (depends on
sub-groups)
Chronicity Recurrent attack may occur Present
Deformil ies, musr le atrophy Absent Present
l-l itting polyarthritis Common Rare
Cardiac involvement Pantarditis is r ommon Myocarditis or pericarditis occur rarely
Chorea Not uncommon Uncommon
Other extra-articular manife:lat ions: adenopalhy, Not occur May occur
hepatosplenomegaly, polyserositis, iridocyclitis
Lab investigations Raised ASO titer, positive throat culture RF or ANA may be positive
Response to aspirin Dramatic. Abolishes pain and fever Fr-rll therapeutic response may requrre about 4
within I2-24 hr. rvk. Prednisolone mav relieve symptoms
within 72 hr.
Cause lmmune re5ponse 1o CABH5. Unknown.

Enthesitis-related arthritis (ERA) is the most common in Table 17.5: Frequency, Age at Onset, and Sex Distribution of
boys older than 8 years ofage. Juvenile ankylosing spondyljris ILAR Categories of JIA
and arthritis associated with inflammatory bowel disease are
included in ERA. Any child with chronic arrhritis of the axial Systemicarthritis 1-17"h Throughoutchildhood F =M
and peripheral skeleton and enthesitis should be suspected
Oligoarihritis 27-56"/" Early childhood; peak F>>>M
for ERA at 2-4 years

Psoriatic arthritis: The diagnosis of juvenile psoriatic arthritis Rheumatoid 2-7o/n Late childhood or F>>M
factor-positive
by IIAR criteria needs simultaneous presence of anhritis and adolescence
polyarthritis
a typical psoriatic rash or, if a rash is absent, the presence of
arthritis and any two of the following: lamily history of pso-
Rheumatoid 11-28% Biphasicdistribution; F>>M
factor-negative early peak at 2-4
riasis in a first-degree relative; dacryiitis that extends beyond polyarthritis years and later peak at
the joint margins; and nail pifting. 6-1 2 years

Undifferentiated arthritis does not represent a separare subset, Enthesitis-related 3-11"/' Late chilclhood or M>>F
aI1hritis adolescence
but includes patients who do nor satisfi/ inclusion criteria for
Psoriaticarthritis 2-11o/. Biphasicdistribution; F>M
any category, or who meet the criteria for more than one.
early peak at 2-4
Table 17.5 lists frequency, age at onset, and sex distribution years and later peak at
9-1 1 years
of ILAR categories of JIA.
Undifferentiated 11-21"/"
arthritis
TABORATORY FINDINGS
Although the laboratory may provide supporr for a diagnosis
of JIA, the diagnosis is essentially clinical.
Liver function test (LFT): ALT to exclude the possibiiiry
r Blood count including Hbo/o, TC, DC, PBR ES& and of hepatitis prior to initiate the treatment s.irh NSAIDs,
C-RP: Shows elevated white biood cell and platelet counts and is a baseline investigation before giving DMARDs.
and decreased hemoglobin concenrrarion. ESR and CRP are Antinuclear antibody (ANA): Should be done in all JIA
always elevated in children with SOJIA, and polyarticular patients because 40-8io,to of all children with oligoarticular
disease, but are often normal in oligoarthritis and ERA. or polyarticrrlar _JIA may have elevated ANA titer. But this
o Urine R/E: To exclude the possibility of infection; rhis may is alu'als negarive in systemic onset disease. ANA positivitr.
triggerJIA. Sometimes to exclude systemic lupus erythema- rs usuallr' associated with an increased risk of er-e invoh e -
tosus and reactive arthritis. menr (uveiris).
 CONNECTIVE TISSUE DISEASES

Rheumatoid factor: Should be done in all polyarticular and
systemic onset JIA.'RF positiviry is usually associated with
poor overall prognosis and eventual functional disabllity.
Anti-CCP antibodies: Anti-cyclic citrullinated peptide
(ACCP) antibodies is a good serological marker for early
diagnosis of JIA and is highly specific gAVol .It distinguishes
JIA from other arthritis that mimic JIA. Antibodies could
be detecred in 68Vo of RA sera.
Imaging studies: Radiography of affected joint: Gives infor-
mation about soft tissue swelling, decreased bone density,
joint erosion, joint space narrowing, deformiry fracture.
Ultrasonography: Is often the best way of identifying
intra-articular fluid, particularly in joints such as hip and
shoulder.
Magnetic resonance imaging: MRI provides detailed and
sensitive information on both stiucture and physiology of
cartilage, bone and other loco-motor tissues.
MANAGEMENT
Management of JIA is based on a combination
cological interventions, physical and occupational therapy,
and psychosocial support (Fig. 17.1). The aims of treatment
of pharma-
pyramid, with gradual addition of more active treatments, has
been reversed. Tiearment regimens are individualized depending
on the subtype ofJIA and according to individual response to
achieve maximum regression of the disease.
Thero peutic Modolilies
During the past few years, remarkable advances in the treat-
ment of JIA have been made with the advent of new disease-
modifying anti-rheumatic agents (DMARDs) and biologic
therapy. Physical and occupational therapy are aids to medica-
tion because it helps to maintain and improve range of motion,
muscle strength, and skills for activities of daily living. Splints
may be used to prevent contractures or work to improve range
of motion.
Non-Steriodal Anti-lnfl ammatory Medications
Intra-articular long-acdng corticosteroid injections and NSAIDs
are initial treatment for most patients with JIA. Naproxen is
widely used in different types of JIA. Indomethacin is also
used, particularly in systemic onset JIA patients. Ibuprofen

are, therefore, to control pain and preserve range of motion,

can be used.

DMARDS
H
muscle strength and function, to manage systemic complica- DMARDs are shown to be effective in JlA, these include
tions, and to facilitate normal nutrition, growth, and physical methotrexate (MTX), sulfasalazine, hydroxychloroquine (Jen-
and psychological development. The concept of a therapeutic nifer,2005).

All sub-types of JIA

NSAIDs & intra-articular steroids

4-8 weeks active disease 4-8 weeks N >5 active joints

lntra-articular steroids Methotrexate (MTX)

j:mm"$ Active severe

systemic disease
4-6 months > 5 active joints
max MTX
I
Consider
sulfasalazine or
I
:9T3:t|Ti*1T3
$
methotrexate
Methotrexate (MTX) Glucocorticoids &
MTX as sieroid-
sparing agent
: 3-6 months Active systemic
features
!
Consider anakinra or enbrel or infliximab or thalidomide or lL-6 MRA ff
I
Eig,. 17.1: Suggested treatment algorithnr for JIA (Jennifer, 2005)

7
t

ESSENCE OF PEDIATRICS
Methotrexate ln po\yarticu\ar and sysrenaic onset JlA, IVITX
is the mainstay of treatment and is used as a firsr-line agenr,
either alone or with initial pulses of methylprednisolone and/
or multiple intra-articular steroid injections to achieve rapid
disease control. MTX therapy is given at a dose of 10-15
mglm2 and is administered weekly, either orally or parenter-
ally (subcutaneously or intramuscularly). 60-70o/o of patients
with JIA benefit significantly from MTX therapy, with the
maximum therapeutic effect usually becoming apparent 4-6
months after the beginning of treatment (Ravelli and Martini,
2007). GI toxicity (nausea, anorexia, stomatitis) and transient
elevation of serum aminotransferase levels are the reported
common side effects of MTX therapy. There are occasional
reports of alopecia, hematologic toxiciry headache, and mood
changes in children with JIA. Because folate deficiency has
been thought to play an important role in the development
of MTX side effects, folic or folinic acid supplementation
has been proposed to reduce toxicity associated with MTX
therapy.
Baseline information to be obtained before commencing
MT)G
o Full blood count, ESR +i- C-reactive protein, transaminases,
renal function tests, urinalysis
o Varicella titet even if there is a history of chickenpox*
o MMR titers, if none or primary dose only has been
givenx
*l{ the child proves to be negative to any of the above, ideally vaccination should
be offered prior to commencing MTX .
Sulfasalazine In a double-blind study of patient with JIA,
sulfasalazine was found significantly more effecdve than placebo
in suppressing disease activiry. However, drug toxicity is a
problem: headache, rash, gastrointestinal toxicity, elevated liver
transminases, leukopenia, and gastrointestinal problems con-
tributed rc a 30o/o.
Glucocorticoids
Glucocorticoids are potent anti-infammatory medications
that should be used judiciously in patients with arthritis,
because the side-effect profile includes cushingoid appearance,
hyperglycemia, immunosuppression, cararacts and glaucoma,
adrenal suppression, peptic ulcer, dyslipoproteinemia, hyper-
tension, avascular necrosis ofbone, and central nervous system
disturbance. Although glucocorticoids are the mainstay of
treatment for controlling serious systemic manifestations of
SO]IA, use among polyarticular patients should be limited
to patients with extreme pain and functional limitation while
waiting for a second-line agent to show some effect. Pulse
methylprednisolone (30 mg/kg, maximum I g) has been used
to treat SOJIA patients with systemic manifestations. Once
disease improvement is noted, steroids should be tapered as
quickly as possible or used at the lowest dose that controls
ru
-
syrnptorns. Treatment of a few joints with intra-articular cor-
ticosteroid injections is an effective merhod ro rreat arrhriris
while minimizing systemic side efFects from oral medicarions
flennifer,2005).
Cyclophosphamide
Pulse ryclophosphamide therapy is used in refractory JIA cases.
Biological Agents: Newer Drugs
Etanercept It is administered subcutaneously rwice weekly
for an indefinite period and may be used with or without
methotrexate. It is licensed for use in children aged 4-17 years
in accordance of specific guidelines. Etanercept appears well-
tolerated by children. Although a higher incidence ofheadache,
nausea, abdominal pain, and vomiting have been reported in
children than in adults treated with etanercept for RA.
lnfliximab Infliximab is a human murine monoclonal antibody
that binds both soluble and cell-bound TNF. It is licensed for
use in RA, but not for use in children. In'a recent small, non-
randomized study, etanercept and infliximab were found to be
equally efficacious in treating patients with juvenile psoriatic
arthritis, polyarticular arthritis, and SOJIA.
Anakinra It is a recombinant IL-l antagonist. Response was
documented in 58o/o of subjects after 4 months of therapy, but
was highest (79o/o) in the systemic-onset patients. This agent
might prove to be very effective in this subrype of JIA.
Autologous Stem (ell Transplantation
It has been considered in recalcitrant cases of SOJIA. Stem cell
transplantation should be performed only at experienced centers
after all other treatment options have failed (Jennifea 2005).
Monogemenl of Uveitis
teatment of chronic uveitis includes local steroids and mydri-
atics. Slit-lamp exam should be done once in every 6 months
in oligoarticular cases and annually in other forrff.-*"-
Monogemenl of Osteoporosis
Patients with JIA are at risk of osteoporosis for a number of
reasons. Most pediatric rheumatologists give.-vitamin D and
calcium supplements in addition to early use of DMARDs,
minimal use of steroids, and encouragemenr for daily physi-
cal therapy.
PROGNOSIS
The course ofJIA in an individual child is unpredictable; some
general statements can be made concerning onset rype and
outcome (Thble 17.6).
 CONNECTIVE TISSUE DISEASES

Table 17.6: Prognosis of ilA by Type of Or-rset

Polvarthritis RF-seropositive Female

Old age
AN A-seropos it ive Fen'rale Cood
Young age

Seronegative Variable

Oligoarthritis A N A-seropos itive Female Excellent (except eyes)

Young age
Chronic anterior uveitis

RF-seropositive Polyarthritis Poor

H LA-2 7-positive Male Cood

Older age
serone!aiive Cood

Systemic disease Oligoarthritis Cood

Polyarthritis Erosions Poor

person shal1 be said to have systemic lupus if any four or more

Systemic lupus erythematosus (SLE) is a systemic disease
that characteristically affects many organ systems; it is often
progressive, terminating in death if untreated, but may remit
spontaneously or smolder for many years. A variety of immune
phenomena occur in SLE: antibodies ale found which react
with various nuclear constituents (ANA)' gamlna globulin
(rheumatoid factors), red blood cells (positive Coombs test)'
white blood ce1ls, antigens used in serological tests
plgle-lets,
for syphilis (false-positive serology).
The onset is gradual with grolonged irregular fever with
remission of variable duration, arthritis, weight loss, and a char-
acteristic erythematous rash resembling the wings of a butterflv
(butterfly rash) over the bridge of the nose and cheeks' Rash
may also occur elseu4rere. Alopecia may also be found'
The disease does not sPare any organ' Renal involvement,
neurologic manifestations, polyarthritis, pericarditis, pleural
effusion, hepatosplenomegaly, panq,'topenia," generalized lymph-
adenopathy, vomiting, diarrhea may occul.
DIAGNOSIS
The diagnosis is confirmed by the presence of antinuclear anti-
body (ANA). The other antibodies to one or more components
of cell nucleus include antibodies against double-stranded DNA,
which are associated with active disease, especially nephritis.
Antibodies to Sm are lelatively specific for SLE. LE prepara-
rion is less sensitive.
Revised criteria for diagnosis of SLE have been depicted in
Tal:le 77 .7. The proposed classification is based on 11 criteria'
of rlre I I criteria dre present. scrially or simultaneously during
arry interval oF obselvation.
MANAGEMENT
o General:
r Counseling, edr-rcation, team approach
r Adequate rest, appropriate nutrition
r Use of sunscreen
r Immunizations, especiallv anti-pneumococcal vaccine
.
r Prompt management of infection
. Non-steroidal anti-infammatory drugs: For muscuiosk-
eletal signs and symptoms.
r Hydrorychloroquine: For cutaneous disease and as an
adjunct to glucocorticoids for systemic disease.
o Glucocorticoids:
r Oral prednisolone 1-2 mg/kg/d.
r IV methylplednisolone indicated for severe disease 30
mg/kg/d on 1 to 3 consecutive days.
Anticoagulation: if anticardiolipin antibodies are present
in significant titers:
r Low-dose aspirin unless thrombosis has occurred.
r Heparin followed by warfarin if thrombosis has occurred'
Others:
,r Mycophenolate rnofetil (MMF): In recent years, few
drugs have raised as many hopes in the lupus commlLniry
as MMF. It is prescribed at a dose varying from 1 to 3 g/d

For the purpose of identif ing patients in clinical studies' a according to GIT at-rd hematological toxicit,v.
 I

ESSENCE OF PEDIATRICS

Table 17.7: Revised Criteria for Diagnosis of Systemic Lupus Erythenratosus

Malar rash Fired ervlhema. flal or raised over the malar eminent es.

Discoid rash Frythematous raised p.rtches witir adherent keratolic .t aling ancl follrcular plugging; atrophi( scarring may oLCUr in older
lesions.
Photosens itivity Ra:h at a re.ult oi unusual reaclion to \unlight.
Oral ulcers Oral or nasopharyngeal ulceration, urually painless. observed by a phyrician.
Arthritis Nonerosive arthritis involving two or more peripheral joints characterized by tenclerness swelling or effusion.
Serositis Pleuritis (convincing history of pleuritic pain or rub hearcl by a physician or evidence of pleural effusion) or pericarditis
(documented by ECC or rub or evidence of pericardial effusion).
Renal disorder Persistent proteinuria >0.5 g/d or >3+ if quantitation not performed or Cellular casts may be red biood cell, hemoglobin,
granular, tubular, or mixed.
Neurologic disorder Seizures in the absence of offending drugs or known metabolic derangements (e.g., uremia. ketoacidosis, or electrolyte
imbalanr et or
Psychosis in the absenee of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte
imba la nce'
Hematologic disorder Hemolvtic arremia wilh reticuloL),losis or
Leukopenia <4000,mm lota] on lwo or more occdsions or
Lymphopenia - l 500lmm on lwo or more oc( asions or
Thrombor ytopenia < 100,000,mm .

lmnrunologic disorder Posiiire I I r ell prepar.rtion or

Antinuclear antibodv
Anli-D\A antibody to native DNA in ahnormal titer or
Anli-Sm-presenr e o[ antibody to Sm nuc]ear anligen or
False posilive.erologit lest resulf for syphili> linown to be positive {or at least b mo and uonfirmed by lreponema pallidum
immobilizatron or fluorescent treponemal antibody absorption test.
Att abnottnal liter of anlinu( lear anlihody at any poinl in lime anci in the absence o{ drugs known to be associated with
drug-indur ed lupus syndrome.

Biologics: futuximab (anti-CD20) is "one of the most thy with characteristic curaneous findings and focal areas of
promising biologics based on some unconrrolled series of myositis resulting in progressive proximal muscle weakness
lupus patients including lupus nephritis patients (Cassi- that is responsive ro rhe prompr insrirution of immunosup-
dy and Petry, 2005). pressive therapy.
JDM is characterized by vasculitis affecting small vessels of
Lupus Nephritis skeletal muscle, with immune complex deposition and subse-
quent inflammarion of blood vesseis and muscle. It has been
Management of lupus nephritis has been summarized in
Thble 17.8.
documented to follow infections, allergic reacrions, or sun
exposure, but no causal relationship has been shown.

,luvenile Dermotomyositis
-Juvenile dermatomyositis (JDM), the most common of rhe
pediatric inflammatory myopathies, is a systemic vasculopa-
Table 17.8: Management of Lupus Nephritis
Symplomal ic mdndgement
Prednisolone 0.5 mg/kg for 2-4 months. then
ldper 1o maintenanr e lerei.
Prednisolone 1 mg/kg for 3-6 months, then
faper lo mainlenance level.
Predni:olone 1 mg/kg with possible addition of
Cvt lophosphrmide r5O0-1000 nrgzm monthll
for 6 monrhs arrd then qudrterly for 2-J yeors,
or Azathioprine rl 2 mgkg/d orailyr
Diagnosis
To make definitive diagnosis of dermatomyosiris, four of the
following five criteria are required.
i. Rash typical of dermatomyositis consisting of heliotrope
discoloration of the eyelids with periorbital edema and an
erythematous, scaly rash over dor-sal aspects of metacarpo-
phalangeal and proximal interphalangeal joints (Gottron
papules).
ii. Symmetric proximal muscle weakness.
iii. Elevated one or more muscle enzymes (LDH, CPK, and
aldolase).
iv. EMG abnormalities rypical of dermatomyositis (fascicu-
lations, needle insertion irritabiliry and high-frequency
discharges).

v. Muscle biopsy documenting histologic evidence of necrosis
and inflammation.
Complications
Dystrophic calcification is one of the hallmark sequelae of
JDM. Calcification is reported in30*70o/o of patients in various
series. Calcinosis may involve skin or sometimes muscles. Cal-
cinosis may occur as superficial lump, deep tumorous deposits
around joints or plates along fascial planes. Muscle calcification
results in contractures or severe muscular pain.
Treatment
:
Corticosteroids are the mainstay of therapy for children with
JDM. Early and adequate treatment,with glucocorticoids
is probably the single most important factor in improved
prognosis of JDM. Children with mild cutaneous finding
may take hydroxychloroquine with low daily dose of steroid.
IV methylprednisolone may be used in case of incomplete or
absent response. A number of secondline agents are used in the
treatment of JDM including methotrexate, cyclophosphamide,
cyclosporin, and azathioprine.
Prognosis
The course of JDM in children usually has several phase-s. The
early prodromal phase is followed by a period of progressive
muscle weakness and rash that then stabilizes for 1-2 yeara
before recovery. Acute exacerbations and remission without
any stabilization of the initial course of diqease may occur in
about 20o/o of children. Late progression of JDM is reported
with a recurrence of active disease after a prolonged remission.
Henoch-Schonlein purpura (HSP), a vasculitis of small vessels,
occurs as a result of deposition of immune complexes. The
antigen provoking the hypersensitiviry reaction is not usually
apparent. GABHS infection of upper respiratory tract may be
responsible in 50o/o of cases. It is the most common cause of
non-thrombocytopenic purpura.
CL!NICAt FEATURES
Onset may be acute with the appearance of several manifesta-
: tions (involving skin, GI tract, joints, and kidney) simultane-
ously, or insidious with sequential occurrence of symptoms
; over a period of weeks or months.
1. Skin lesions (100%): Begin as red macule, become
i urticarial and purpuric and mainly involves the extensor
surfaces of limbs, buttocks, and back. Angioedema_may
I
t proceed to purpura, edema occurs primarily in the depen-
It dent areas. evelids, lips, scrotum (with or without testicular
torsioni. or the dorsum of the hands and feet.
I
t
CON NECTIVE TISSU E DISEASES
The hallmark of the disease is the maculopapular
purpuric rashes that become hemorrhagic and palpable.
These rashes tend to occur in crops, last from 3 to l0
days and may appear at intervals that vary from a few
days to as long as 3-4 months. Recurrence of the rash
may not end until as late as a year.
2. Arthritis (75o/o): One or two joints (involving knees and
ankles) may be involved. They may recur.
J. GI tract (5OVo1 . Intermittent abdominal pain (often
colicky); patient may have diarrhea, melena, or hemate-
mesis. Intussusception (ileoileal) with bowel perforation
may occur.
4. Renal (25-50%). Patient may present with features
of glomerulonephritis, nephrotic syndrome, or renal
failure.
5. Others: CNS involvement (seizures, paresis, or coma);
hepatosplenomegaly and lymphadenopathy may
occur.
DIAGNOSIS
The hemorrhagic appearance and distribution of rashes are
characteristic of the syndrome. The piatelet count and coagula-
tion studies differentiate it from other hemorrhagic disorders.
Ba-enema may be used for both identification of intussuscep-
tion and non-surgical reduction. Urinalysis and renal function
test should be done when indicated. Throat culture and ASO
titer can be estimated.
Definitive diagnosis of vasculitis can be confirmed by biopsy
of an involved cutaneous site. Renal biopsy when indicated.
TREATMENT
The disease has a self-limited course of 4-6 weeks. No specific
treatment. teatment is symptomatic. P,enicillin therapy is
indicated if a throat culture is positive for GABHS or ASO
titer is high.
o Paracetamol can be given for joint pain.
o For abdominai pain, oral prednisolone 1-2 mg/kg/d pro-
duces dramatic improvement, may be continued for 2-3
weeks depending on ciinical response. In severe acute
abdomen, patient should be kept on nothing per oral,
suction, IV fluid, and IV corticosteroid. After improvement,
IV corticosteroid may be replaced by oral prednisolone. In
intussusception, Ba-enema should be done. Antispasmodic
may be used for abdominal pain.
o HSP nephritis is usually mild and self-limited. Prednisolone,
azathioprine can resuit prolonged remission.
o Corticosteriods can also be used in CNS complications.
Adequate hydration and plain diet should be allowed. If edema
involves the scrotum, elevation of the scrotum and local pulling
(as tolerated) may decrease discomfort.

ESSENCE OF PEDIATRICS
Mixed connective tissue disease is a connective tissue disorder
that combines rhe clinical and laboratory features of JRA,
SLE, dermatomyositis, and scleroderma. Therapy is the same
as that for SI-8.
REACTIVE ARTHRITIS
Reactive arthritis is the occurrence of an aseptic arthritis after
an infection elsewhere in the body, eqpecially after gastro-
intestinal infections. The bacteria most frequently involved
include Yersinia, Salmonella, Shigella, and Campylobacter sp.
The arthritis occurs 1-2 weeks after onset of gastrointestinal
symptoms. The course is self-limited, and treatment of the
joint maniFesrations is sympromatic.
REITER SYNDROME
Reiter syndrome is a form of reactive arthritis that, in addi-
tion to joint manifestations, is characterized by urethritis and
conjunctivitis or iritis. It is usually associated with Chlam-vdia
nachomatis infections. The arthritis is mild, and chronic arthritis,
rarely occurs. NSAIDs can be used. Corticosteroid injection into
non-responsive joint and areas of enthesopathy is very helpful.
SERUM SICKNESS
Serum sickness may be associated.with painful swelling around
the joints without warmth or redness. teatment is symptomatic
and there are no sequelae.
KAWASAKI DISEASE
It
is an acute febrile mucocutaneous lymph node syndrome,
mainly affecting infants and young children <5 year (80%).
Like rheumatic fever, it is also a cause of acquired heart disease;
this disease involves coronary vessels with a constellation of
other systemic manifestations. The diagnosis is clinical, and
criteria are given below. The laboratory investigations may
show polymorphonuclear leukorytosis, thrombocltosis, elevated
C-reactive protein, and raised ESR.
Diognostic Criterio
o Fever lasting for at least 5 days.
o Presence of four of the following five conditions:
o Bilateral non-purulent conjunctival injection.
-
'r Changes of the mucosa of the oropharynx, including in-
jected pharynx, injected and/or dry fissured lips, straw-
berry tongue.
o Changes of the peripheral extremities, such as edema
and/or erythema of the hands or feet, desquamation,
usually beginning periungually.
o Rash, primarily ffuncal: polymorphous but nonvesicular.
'r Cervicallymphadenopathy.
o Illness not explained by other known disease process.
Treolmenl
Tieatment consists of administration of aspirin in doses of
75-80 mglkgld in 3-4 divided doses till the child becomes
afebrile. Later on, aspirin is continued in doses of 5 mglkgld
single dose daily as an antithrombotic agent for 6-8 weeks.
Intravenous gammaglobulin administered early in doses of
2 glkgas single dose has shown to decrease incidence of coronary
artery complications.
ft is zoonosis caused by the transmission of Borrelia
burgdorferi to humans through the bite of an infected tick
(Ixodes species).
CtINICAt FEATURES
Early localized feature is the typical annular rash, named
erythema migrans, usually present in axilla, periumbilical
area, thigh, and groin. ,It may be associated with recurrent
fever, myalgia, or headache. \fhen disseminated, second-
ary erythema migrans develops, which may be associated
with conjunctivitis, lymphadenopathy, aseptic meningitis,
and cranial nerve palsy (VII nerve palsy) . Asymmetric
oligoarthritis or arthralgia (involving the knees) occurs as
a late manifestation. Other late manifestations are features
of CNS (rare).
DIAGNOSIS
Diagnosis is based on the demonstration of antibody to B.
burgdorferi in the serum.
Isolation of the bacteria in culture media is expensive and
time consuming (>4 weeks). PCR can be done.
TREATMENT
Amoxicillin 90 mglkgld for earlv cases, and ceftriaxone 100
mglkgld for disseminated cases.
\I
I
 !

CON N ECTIVE TISSU E DISEASES

TREATMENT

Although there is no specific treatment, immunosuppressive agents,

Scleroderma, a chronic disease of unknown cause, is character- including methotrexate and corticosteroids, in the early stages of
izedby fibrosis affecting the dermis causing hardening of skin the disease may be helpfi.rl in curbing inflammation. Howeve!
and arteries of the lungs, kidneys, and gastrointestinal (GI) corticosteroids later in the course ofthe disease do not appear to
tract. Antinuclear antibodies (ANAs) specific for topoisomerase be effective and may exacerbate hl,penension. Additional treatment
I (SCL70) and centromere are found in many patients, which includes physical and occupational therapy to improve fexion
suggests that autoimmune processes play a role in pathogenesis. contractures and maintain muscle strength, and springJoaded
splints in selected patients (Michael LM,2007).
DIAGNOSIS
The diagnosis of scleroderma requires the presence of the major
criterion or two of the three minor criteria.
Major criterion Vasculitis is defined as inflammation of blood vessels. Although
o Proximal scleroderma: Typicai sclerodermatous skin cha-nges many vasculitides affect both adults and children, such as
(tighmess, thickening, and non-pitting induration, excluding Kawasaki disease, occur aimost exclusively in childhood.
localized forms of scleroderma) involving areas proximal to Vasculitis is also a component of many auto-immune diseases.
the metacarpophalangeal or metatarsophalangeal .ioints. The extent of vessel damage can range from moderate, as in
most children with Henoch-Sch6nlein purpura, to severe, as
Minor criteria in children with polyarteritis nodosa. Most classifications of
o Sclerodactyly: Sclerodermatous skin changes limited to digirs. the vasculitic syndromes are based on the size and location of
o Digital pitting scars resulting from digital ischemia. the blood vessels that are primarily involved, as well as the
o Bibasilar pulmonary fibrosis not attributable to primary rype of inflammatory infiltrate.
L lung disease.
!
I
I' CLASSIFICATION CTASSIFICATION OF CHITDHOOD
I VASCUIITIS (Ozen el q1.,2006)
i. Systemic sclerosis:
l- I Predominantly large vessel vasculitis
r o Diffuse: Systemic widespread skin fibrosis, including proximal
Thkayasu arteritis
limbs, trunk, and face; early internal organ involvement.
I
II Predominantly medium sized vessel vasculitis
I o Limited: Systemic distal skin involvement, often face, with
Childhood polyarteritis nodosa
late, if any, internal organ involvement.
Cutaneous polyarteritis
o Overlap: Sclerodermal skin changes with features of other
Kawasaki disease
connective tissue disorders.
III Predominantly small vessel vascuiitis
o On face, forehead, or scalp. (A) Granulomatous: \Tegener granulomatosis
o On extremity. (B) Non-granulomatous: Henoch-Schonlein purpura
Localized scleroderma: IV Other vasculitides:
o Morphea Behcet disease
o Generalized morphea Vasculitis secondary to infection (including hepatitis B
o Linear scleroderma associated polyarteritis nodosa), hypersensitivity vasculitis

LABORATORY FINDINGS DIAGNOSIS

Inflammation early in systemic disease may be rellected by See Figr.ire L7.2 for diagnosis
anemia and sometimes eosinophilia. ANAs are often Present.
Anti-SCL70, which is specific for topoisomerase 1, and anticen-
tromere auto-antibodies are strongly suggestive of a diagnosis of
scleroderma. In Iocalized scleroderma, laboratory abnormalities
are usually restricted to positive ANA (with anti-SCL70 and
anticentromere antibodies much less common than in systemic See Table IT.9 for differential diagnosis of some common
sclerosis) and, on occasion, eosinophilia. rheumatic diseases.
 !
-

ESSENCE OF PEDIATRICS

lndications for testing

nexplained systemic illn-ess
U
Multiple system involvement (renal, pulmonary, dermatologic)

The following have been ruled out: lnfection

nT,;;r,

Determine systems involved

. Urinalysis (UA). Complete OozOgsO (hematuria)
. Sedimentation rate, Westergren (ESR) 0040325 (elevated)

2 of the

UA+ 1. Skin involvement (livedo

ESR+ reticularis, tender
subcutaneous nodules)
2. Myalgias or muscle
tenderness
J. Systemic hypertension
4. Mono or polyneuropathy
Fever of > 4 days and 4 of the 5. Abnormal UA
following: 6. Testicular pain or tenderness
1. Erythema of palms and sole 7. Signs and symptoms
with possible desqL/amation suggesting organ Polyafteritis ff
2. Erythematous rash within 5 involvement vaculitis -----> nodosa
Kawasaki ffi daYS of fever onset
AND
(PAN) $
ffi

disease me3. Bilateral conjuctival injection @*!@ffi#

4. Oral cavity changes including Biopsy showing small and

erythema, dryness and midsize afiery vasculitis
cracking of lips, strawberry
tongue and diffuse erylhema OR
5. Cervical lymphadenoplhy Angiographic abnormalities
demonstrating vasculitis
Palpable purpura

AND
At least 't of the following:
1. Diffuse abdominal pain -+ Henoch-Schonlein
purpura (HSP)
2. Arthritis or arthialgia
OR

Any biopsy with lgA deposition

Any 3 of the following:
At least 1 of the following: 1 . Granulomatous inflammation
1. Decreased peripheral on biopsy
arterial pulses 2. Abnormal UA
2. Blood pressure difference J. Nasal-sinus inflammation
>10 mmHg between 4. Subglottic, tracheal or
Takayasu
extremities endobronchial stenosis
X-ray r wegener
arteritis
(aortic arch
3. Bruits over aorta or its trAbnormal chest tr
major braches or CT of the chest granulomatosis
syndrome)
4. Hypeftension Elevated c-ANCA as
@ $

AND determined from anti-

neutrophil cytoplasmic
Angiographic abnormalities of antibody with reflex to titer
the aorta or its main branches & MPO/PR3 antibodies
0050523
FigL. lZ.Z: Tests involved in the diagnosis and classification of vasculitis in children
 I

CONNECTIVE TISSUE DISEASES

Table 17.9: Differential Diagnosis of Rheumatic Diseases

Sex No predilection Dependent on Cirls > boys No predilection Cirls 3:2

subgroup
Age at onset 5-1 5 yr 1 yr or older Usually over age 8 yr Usually = 4 y1 2 ),r or older
Joint manifestation Transient migratory Pauciarticular or Arthralgia, transient Pain and snelling Joint contractures;
arthritis-large joints polyarticular Chronic arthritis, chronic of hands and feet. arthritis occasional ly
(6 wk or more) arthritis Arthritis occasional Iy
Extra-articular Fever Dependent on Occasionally Fever, conjunctivitis, Rash, muscle
manifestations Cardiac disease subgroup. S),stemic multisystem disease mucocutane()us rveakness, pain.
Chorea juvenile rheumatoid including nephritis, les ions, Castro intestina l,
Rash, nodules arthritis, fever, rash, rash, hernatologic and lymphadenopathy, respiratory
etc. Pauciariicular: CNS involvement vasculitis of coronary
iridocyclitis and large vessels
Laboratory Prior streptococcal May have antinuclear Antinuclear ar-rtibodies Abnormal coronary Abnormal
infection, antibodies, Auto-antibodies Low vessels on muscle enzynres,
Echocard iograph i c rheurnatoid factor complement Antibody echocard iogram electronryogranr,
or FCC evidence of to DNA muscle biopsy
card r tr s

Pathogenesis Poststreptococca I Unknown lmmune cornplex Unl<nown Unknown

event disease
Diagnosis Clinical (ones criteria) ( lrnrcal (JUVenrle Clinical plus Clinical (Kawasaki Clinical, rash plr-rs
rheumatoid arthritis laboratory (systemic criteria) myositis. Muscle
criteria) lupus erythematosus biopsy
criteria)
Natural history Arthritis-trans ient Chronic arthritis rnay Chronic or recurrent Self.limited Coronary Chronic
carditis may car,rse be destructive may be fatal. vasculitis. May be May be fatal.
permanent darnage fataL
Therapy Anti-inf lammatory Anti-inllammatory Anti-in{lammatory lntravenous globulin. Corticosteroid
group A Streptococcus Physical therapy. Corticosteroid Aspirin. Cytotoxic.
prophylaxis to prevent Cytotoxic.
rec u frence

10. Yatnamoto KS. Juvenile Rheurnatoid Arthriti,r 2003, Case baserl

PaediatritsJbr Medica/ students nnd ksidentt, Depattrnent of paediatrics,
University of Hawaii John A. Burns School of Medicine, X\4.2, 1 7.
l. Behrman RE, Kliegman RM, Jenson lHB. Ne/son Ti:xtbook of
Peditttrics 16"' ed. Philadelphia: WB Saunders Co., 2000.
ll Cassidy JT, Petty RF.. Tixrbook of Pedianic Rheumatolog1 5'\ et1.
USA: Elsevier Saunders Company, 2005:214-95.
2. Ghai OP. Essential Pediarrics 7'r' ed. Nevr Delhi: CBS Publishers.
2049.
t2. Hofer M, South \7oocl TR. Classificatior-r of childhood arrhriris. Bd.r/
Practice antl Research Clinical Rhettmdtologt 2002;16:374 96.
Clayden G, Hawkins R (ed). Pediatrzt's: Trerttment dnd. Prognosis
13. Swijayaiatrre L. Juvenile idioparh.ic arrhritis. Sri Lanka Journal of
1" ed. New Delhi: Jaypee Brotl.rers, 1989.
Cl,ild Hrnlth 2001;.t0:q.r 5.
Parthasarathy A (ed). IAP Tixtbook of Pediarrits 4'r' ed. Nlelv Dell'ri:
t4. lennifer E\X/, Norman '1'1. Juvenile idiopathic arrhritis. Pediarrir.
Jaypee Brotl-rers, 2009.
Clinic of North Americtt 2005;52:413 Q.
Drvorkin PH (ed): Pediatrics: NMS 3'l ed. Baltimore: Villiams
15. Sa\\'hennv S, V'/oo P Diasnosis and managemenr of jur-enile idiopathic
and \7ilkins, 1996.
erthritis: currenr srarus. Indhn I'edi,ttir:i 2001138:1083-90.
Bisnoal. GroupA streptococcal inflctions ancl acure rhcumatic
I (r. N{ichael Ltrf . EvalLration of susp.-cted of rheunatic disearse. In:
N Engl J Med 1991;325733 93.
fever.
fuchard E. Behramrn. Robert N4. Kliegn.ran, Hal B. Jenson (eds.)
Guidelines for the diagnosis of N-reumatic fe:'er. .1,4)[A
7'elso;t Ti'ttbool of'?edidtric: 18'i'ed., Chapter 143. India: Saunders.
1992;268:73.
Tan EM, Cohen AS, Fries JF, et al. The 1982 rer.ised crireria fbr'
l00r:-9i 5.
L
-. RavelliA. i\{artini A. Jr,rvenile idiopathic Lancet
the classification of SLE. Artbrttis Rheum I9B2;25:I271. ^rthti:is.
200 7 r.i69:767-78.
Br-rkulmez H, Murray H, Passo, Villiam S, RoN'e. Classification of
18 ()zen S, Ruperto N, Dillon MJ, Bagga A, Barron K, et al. EULAR/
juvenile chronic idiopathic arthropathies: is it time to change yet!
Arthritis Foundation, Bulletin on the rhelrmatic disease 2003;51.
PReS endorsed consensus criter.ia tor the classification ofchildhood
r.asculitides. Art tt Rh eum Dis 2006:65 :936-41.
i:1-5.
 CHAPTER 18
Child Psychiatry and Developmental Disorders

Chopler Conlents
-rOr-

Psychopharmacology

Mental retardation. .. ..............336

o Space occupying lesion (SOL)

,r Infection
Headache (HA) is a common problem in pediatric practice; 'r Cranial neuralgia
40% of children snder 7 years and 75o/o undet 15 years of
.r Substance abuse/withdrawal
age experience HA at some time. The corresponding figures
r Facial or cranial structures related HA
for migraine headache are 7o/o and 5o/o, respectively.
,r Psychiatricdisorders

The pattern of HA could be: DIAGNOSIS

o Acute: HA persisting From minures to 72 hours.
Diognostic Criteriq ond Types of Migroine
o Acute recuffent
o Chronic: HA persists for >72 hours. The chronic HA could o Migraine without aura:
be primary (dwration <3 months) or secondary (duration >3 A. At least 5 attacks fulfilling criteria B-D
months). B. Attack lasting 1-72 hours
C. At least two of the following:
ctAsslFlcATloN i. Bilateral or unilateral-frontal, temporal, not
occipital
The International Classification of Headache Disorder (ICHD
11. Pulsating
2004) classification system divides HA into: iii. Moderate-severe intensity
o Primary headache disorder: Underlying causes are intrinsic iv. Aggravated by routine physical activity
to nervous system: D. At least one of the following:
. o Migraine l. Nausea,vomiting, or both
,r Tension-rype headache ii. Photophobia and phonophobia (inferred from
i> Cluster headache behavior)

o Secondaryheadache disorders: HA attributed to a second- Migraine with aura:

ary cause. The secondary causes of HA includes: A. At least two attacks
,r Tiauma B. Migraine aura fulfilling criteria i-ii for one of the
o Vascular disorder subforms.
 CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS

Aura consisting of at least one of the following, but Table '18.1: Difference between Migraine and Tension-Type
no motor weakness: Headache

a) Fully reversible visual symptoms including positive

features (e.g., flickering lights, spots, or lines) and/or Appearance lll looking, pale Normal
negative features (i.e., loss ofvision). Sex Female Female
b) Fully reversible sensory symptoms including Family history l' relatives + l' relatives +
positive features (i.e., pins and needles) and/or Location Usually biJrontal, Diffuse
negative features (i.e., numbness). unilateral
c) Fully reversible dysphasic speech disturbance. Character Pulsating quality or Band-like, pressing,
ii. At least two of the following: throbbing, pounding sustai ned contractions
of scalp, squeezing or
a) Homonymous visual symptoms and/or unilateral tightening quality
sensory symptoms.
Severity Moderate/severe Less severe
b) At least one aura symptom develops gradually
Duration Brief; 30-'1 20 min Lasts all day
over >5 minutes and/or different aura symptoms
occur in succession over >5 minutes. Frequency Weekly/less often Daily/several times per v',k

c) Each symptom lasts )5 and <60 minutes. Aura Seeing light, spots, No aura
blurred vision,
C. Not attributed to another disorder. somatosensory/ speech
Associated Nausea, vomiting, No autonomic elernents
Diognoslic Crileriq of Tension-Type feature photophobia, like migraine
Heqdqche (TTH) phonophobia,
osmophobia
A. At least 10 episodes fulfilling criteria B-D Aggravation Activity, Iight, noise, smell Stress
B. Headache lasting 30 minutes to 7 da1's Relieving Sleep, analgesic
C. At least nvo of the following: faclor
i. Pressing/tightening quality Relation to Awaken from sleep Starts after child ar,vakes
ii. Mild-moderare inrensiry sleep at morning/occur on
awakening
iii. Bilateral location
iv. Not aggravated by routine activity Activities lnterrupts, rest in quiet,
dark room
Continue

D. Both of the following: Disability Creater Less

i. No nausea or vomiting Pericranial Abserrt Present
ii. No photophobia or phonophobia tenderness

Table 18.1 lists features differentiating migraine and tension-

tvpe headache.
r Acetaminophen (15 rng/kg/dose), ibuprofen (10 mg/kg/
Indicqlions for Neuroimoging dose), naproxen (5 mg/kg/dose)
o Abnormal neurological sign/seizure :, Nasal Sumatriptan (5 or 20 mg) during very early (aura
o S/o t rcp phase) part of mieraine only

o Nocrurnal HA awaking parient o Prophylaxis:

o Chronic progressive headache
o Headache in VP shunt t Migraine: >3 episodes/month + interference with
c Occipital HA activities-Flunarizine 5-10 mg at bed time (class I
o HA <1 month duration/recent onset HA evidence) for at least 6 months. Other drugs that can be
e Change in headache type/new onset HA used include propranoloi (2-4 mglkgld), amitriptyline
o Occurrence wirh straining (1 nig/kg/d at bed time. max. 25 mg), Topiramate or
o Change in headache pattern 5odiunr r alproare.
t Tbnsion-type beadacbe: Frequent episodic TTH, ami-
triptviine onlv
MANAGEMENT

Monogement of Heodoche Monogemenl of Slqlus Migroine: Severe

o Acute symptomatic management: To abort ongoing episode
Heodsche >72 Hours
of HA o Hydration: NS l0 ml/kg -+ 100/o Dextrose in baby sali
 F

ESSENCE OF PEDIATRICS

Antiemetic The pediatrician should consult school personnel for infor-

o Ondansetron 0.15 mg/kg IV
o Me toclopramide 0. 1 5 mg/kg (max. 1 0) IV over 1 5 min
c Prochlorperazine 0.15 mg/kg (max. 10) IVover 15 min
o Promethazine0.25-l mg/kg/dose (max. l0 mg)
a Analgesic: Ketorolac 0.5-1 mg/kg IV/IM (max. 30 mg)
a Tryptans: Sumatriptan 5-20 mg, Zolmitriptan 5 mg intra-
nasally at onset in cases refractory to analgesics
a Sedation: Lorazepamldiphenhydramine 0.1 mg/kg each
a Infusions:
,r Valproic acid 15 mg/kg bolus -+ 5 mglkg 8 hourly till
HA freedom/undl 10 doses administered
c Magnesium 50-75 mglkgldose IV over 30 min (max. 2 g)
Steroids: Dexa 0.6 mg/lg (max. 20), MPS 2
^elk1(max.
mation about the child's pattern of behavior at school and
should ask the parents' opinion about what could be wrong
with their child. It is helpful to meet with the parents and
child separately, so that there is less denial of the problem
on either of their parts.
If possible, arrange for the mother or father to accompany
the child to school. Once the child is actually at school, he
or she will generally stay.
The school personnel should be alerted prior to the child's
return to school to make arrangements to reduce further
anxiety the child may experience while in the class.
Psychotherapy may be indicated for the parents and child.
Behavioral management techniques, such as desgnsitization,
125) may be helpful.
Short-term use of anti-anxiery medications may be necessary,
if the child's anxiery is overwhelming.

School phobia or school refusal in a child is manifested by poor

school attendance caused by unwarranted fear or inappropriate
anxiery about leaving home, or in particular, the child! mother. Persistence in ingesting non-foodsubstances constitutes pica.
Children with school phobia usually dread one aspect of school, It is most common between 1 and 6 years of age and affects
e.g., a teacher. The school-phobic child prefers to remain at home both sexes equally. Age of onset is between 12 and 24 months,
and avoid school. Typically, the phobic child has problems return- and it declines with age.
ing to school after vacations. The phobia is often accompanied
by a vague abdominal pain or a headache alleviated by school
absence. Sometimes, it has been seen that his parents push him ETIOTOGY
too hard in relation to his capabilities.
Exact cause is unknown. Howeve! nutritional deficiencies
particularly iron and zinc deficiencies, parental neglect and
ASSESSMENT deprivation, mental retardation, and low socio-economic status
are found associated.
1. School phobia has been described as "the great imitator".
Typical findings are:
a. Vague physical symptoms. DIAGNOSIS
b. Negative physical examination and laboratory findings.
c. Poor school attendance attributed to the somatic DSM-IV (Diagnostic and Statistical Manual of Mental
symptoms. Disorders, IV ed.) diagnostic criteria for pica:
2. A thorough history and physical examination, in addition o Persistent eating of non-nutritive substances for a period
to selected laboratory tests (e.g., complete blood count, of at least I month.
ESR, urinalysis), are often necessary to assure parents that o The eating of non-nutritive substances is inappropriate to
the childt symptoms are not secondary to organic disease. developmental level.
Also a symptom diary is often helpful in this regard. o The eating behavior is not part of a culturaliy sanctioned
practice.
TREATMENT o If the eating behavior occurs exclusively during the course of
another mental disorder (e.g., mental retardation, pervasive
After the parenrs are assured that their child is well, the developmental disorder), it is sufficiently severe to warrant
physician should insist on the immediate return of the child independent clinical attention.
to school. The physician shouid then review with the parents
rvl-rat approach to use if the child reports that he or she is Laboratory examination:
sick the next day. No single test confirms or rules out a diagnosis of pica. it
If the anxiety about going to school occurs in a child 3-5 However, serum levels of iron and zinc should always be t
I
years old following a medical illness, it usually responds to obtained. Serum levels oflead should be obtained to exclude
I
support from the parents. lead toxicity.
t
tI
 CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS
coMPUCATTONS
The most serious complications are lead poisoning from lead-
based paint, intestinal parasites after ingestion of soil or feces,
anemia and zinc deficiencies afier the ingestion of clay, severe
iron deficiency afier ingestion of large amount of starch, intestinal
obstruction from ingestion of hair balls, stones, or gravel.
TREATMENT
Environments that neither stimulate nor furnish adequate
care contribute to the general boredom of many children
with pica. The childt day should be evaluated and restruc-
tured, so that supervision, play opportunities, and involve-
ment with others are provided.
There is a relation berween psychological deprivation and
pica as a compensatory mechanism to satisi/ oral needs. So
if there is parental negligence, counseling should be done
with the parents.
a Iron and zinc supplementation, if there is deficiency.
a Antihelminthics.
a Psychiatric referral if associated psychologicai problems.
Enuresis is the involuntary emptying of the bladder beyond the
age when bladder control should have been established. Onset is
usually between 3 and 8 years of age. Children are not generaily
labeled enuretic unless the symptoms persist beyond the age of 5
year. It usually occurs at night; daltime enuresis is rare and usually
implies a more serious problem. Night-time enuresis usually stops
by 34 year of age and daltime enuresis by 2Vz year. After a child
has been able to remain dry enuresis may occur transiently in
response to an overwhelming stressful situation. Boys:girls is 2:1.
There is often a family history of enuresis.
ETIOLOGY
Physiological factors play a major role. Normal bladder control,
which is acquired gradually, is influenced by neuromuscular and
cognitive development, socio-emotional factors, toilet training, and
genetic factor. So difficulties in any areas lead to urinary incon-
rinence. Urinary tract infection, CRF, diabetes mellitus, diabetes
insipidus, and psychosocial stress sometimes precipitate enuresis.
*
I
DIAGNOSIS
DSM-IV diagnostic criteria for enuresis:
o Repeated voiding of urine into bed or clothes (whether
involuntary or intentionai).
o The behavior is clinically significant as manifested by either
2
I a frequency of twice a week for at least 3 consecutive
I low frustration tolerance, hyperactivity, and poor incoordina-
I
months or the presence of clinically significant distress or
I impairment in social, academic, or other important areas
i of functioning.
o Chronological age is at least 5 years.
o The behavior is not due to the direct physiological effect of
a substance (e.g., a diuretic) or a general medical condition
(e.g., diabetes, spina bifida, a seizure disorder).
Types of enuresis are:
o Nocturnal only.
o Diurnal only.
o Nocturnal and diurnal.
TREATMENT
Reward the child for dry night. Punishment and humiliation
by parents or siblings should be discouraged.
Urinary tract infection, CRB diabetes mellitus, diabetes
insipidus, etc. must be ruled out. Fluid restriction before
bed and night lifting to toilet will produce a temporary
improvement in most, but the majority will relapse.
Parents should be told that enuresis is not a willful symptom
and should be advised to be as evenhanded as possible.
Patience and tact are needed in explaining this symptom
to the child's family.
Conditioning with a buzzer or alarm to wake the child at
night may be helpful and generally works within weeks or
months, if there already has been resolution of the underlying
conflict. A brief course of imipramine may help children
(the dose is 25 mg to the start, and then increased at 2
weekly interval to up to a maximum of 75 mg given at
night for 2-3 months).
The use of a star chart will often be effective not only in
providing a baseline record, but in providing method for
stopping the wetdng.
Encopresis is the involuntary passage of feces at any age by
which bowel control should have been established. Bowel
control is established in >95o/o of children by the fourth
birthday and in 99o/o by the fifth birthday' After the age of
four, encopresis is 3-4 times more common in boys than
in girls.
ETIOTOGY
Encopresis involves a complicated interplay berween physi-
ological and psychological factors' Inadequate training or
lack of appropriate toilet training, emotional disturbances
(including anger, anxiety, fear) or some combination of these
may cause encopresis. It may be associated with neurodevel-
opmental problems as easy distractibility, short attention sPan'
tion. It may also be precipitated by move to a new home or
start of school.
ESSENCE OF PEDIATRICS
DIAGNOSIS
DSM-IV diagnostic criteria for encopresis:
o Repeated passage of feces into inappropriate places (e.g.,
clothing or floor), whether involuntary or intentional.
o At least one such event a month for at least 3 months.
r Chronological age of at least 4 years (or equivalent devel-
opmental level).
o The behavior is not due exclusively to the direct physi-
ological effect of a substance (e.g., laxatives) or a general
medical condition except through a mechanism involving
constiparion.
Two types:
o \fith constipation and overflow incontinence.
e Without constipation and overflow incontinence.
Laboratory examination:
No specific test indicates diagnosis. However, Hirschsprung
disease should be ruled out clinically in which rectum is empty
and there is no desire to defecate but still have an overflow
of feces.
TREATMENT
Family tensions about the symptom must be reduced, and a
non-punitive atmosphere established. Similar effects should
be made to reduce the child's embarrassment at school.
Many changes of underwear with a minimum fuss should
be arranged.
A combination of daily laxatives or mineral oil along with
sitting on the toilet for timed intervals daily is regarded for
successful defecation. Children who are not constipated and
have good bowel control, la{atives are not necessary.
Supportive psychotherapy and relaxation techniques may
be useful in a child with anxieties.
Family intervention can be helpful in children with good
bowel control.
Intermittent dificulty in producing a smoorh flow of speech
mav begin I or 2 years after child learns to speak. Between 2
and 5 years of age, many children experience normal disfluency,
which is characterized by repetition ofwhole words and phrases.
In contrast, stuttering is characrerized by partial word repeti-
tions, multiple rather than single repetitions; irregular, rapid,
or abrupt repetitions, and a high frequency'ofnon-fluency. It
is more common in boys than in girls and is often familial;
40o/o of stutterers ourgrow their symptoms.
TREATMENT
o First, evaluate for family discord; the symptom may be
secondary to it.
In families where the parents become angry and punish
the child for stuttering, i, ."r, be helpful to e*plain to the
parents that the symprom is secondary to the child's anxiery.
Such children actually have aggravated symptoms of stut-
tering, even when the parents show only anxiety about the
symptom. Therefore, explanation of the natural evoiution
of speech patterns from the non-fluent srate ro the point
where the infant begins stuttering is helpful in gaining the
parents' understanding and cooperation.
Positive reinforcement of fluent periods of speech is impor-
tant and consists of maintenance of eye contact with the
child while speaking, encouraging speech during fuent
periods, and being a patient listener. Negative reinforcement
comes from admonitions to inhibiting remarks, which make
the child more aware-of the stuttering. The entire family
should be told not to give signals that indicate to the child
that the stuttering is being noticed.
If it is found that the foregoing -.ulrrr., do not help within
3-4 weeks, both parent counseling and speech therapy
should be undertaken.
It is one type of maladjustment of children with siblings.
Children could be resentful of success of achievements of their
own kith and liins. The childt emorional need of affection
and security may appear to be threatened with the birth of
another child. The older sibling may feel deprived, and this
may initiate hostility in his behavior.
TREATMENT
r Parents have to give a part of their time and attention to
the new baby.
r Conscious efforts should be made to involve the older child
in the care of the younger sibling under parental eye, so
that he can relate to the sibling with love, afl[ection, and
feeling of belonging.
Conduct disorder is defined as persistent antisocial behavior
of children and adolescents that significantly impairs their
ability to function in the social, academic, or occupational
area. Youngsters with the disorder repeatedly violate the age-
appropriate rules ofsociety, displaying a lack ofconcern for the
rights and feelings of others. The rate is much higher among
boys than the girls.
ETIOLOGY
Conduct disorder is strongly associated with abusive chaotic I
and neglectful family environments. Several specific risk factors {
have been identified, including exposure to marital conflict and I
 !

CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS

physical aggression, maternal depression, large family size combined
with lower socio-economic status, and early loss of father due to
divorce. Parental psychopathology, such as antisocial personality
may contribute both genetically and environmentally towards
manifestation of conduct disorder in the offspring.
Other postulated biological factors include high testosterone
levels. This theory was put forward due to the high male to
female ratio for the disorder, but there is no conclusive evidence
in favor of this theory.
CLINICAT FEATURES
There is evidence for two clusters of symptoms in conduct
disorder: aggressiveness and delinquency. Aggression may be
directed towards people (e.g., peers) or animals (crueity towards
animal) or objects (destruction of property). Delinquencies,
on the other hand, include antisocial behaviors, such as lying,
stealing, running away and truancy that do not primarily
involve physical attack on others.
\7hile evaluating a child for conduct disorder, one must
consider whether the reported behavior is appropriate, devel-
opmentally, for the age of presentation. Defiance, and temper
tantrums are often, occur among children between 7t/z and
3 years of age when frustrated. This often resolves with time.
Similarly, lying is often used by2-4 year olds as a method of
playing with language. Almost ali children steal something at
some point in their lives. It becomes a problem when it happens
more than once or twice. Thus, certain behaviors become a
symptom only when they occur at a greater frequency or persist
beyond a developmentally appropriate age.
Unlike the previous behaviors, however, truancy, run-away
behavior, destruction of property (e.g., through fire-setting),
and repeated aggression against animals or others are never
developmentally appropriate.
TREATMENT
Types of developmental delay:
o fsolated: Restricted to a particular domain of development,
e.g., isolated motor, hearing, or visual delay, etc.
o Ghbal: Significant delay in two or more developmental
domains.
Etiology of developmental delay can be grouped into prena-
tal, natal, post-natal, and idiopathic categories like that of
cerebral palsy. Etiology remains unknown tn 20o/o cases even
after extensive investigations. Developmental delay should be
suspected if a child does not achieve:
o Social smile: by 3 months
o Neck control: 5 months
o Sits without support: 12 months
o Stands without suppori: lB months
o \7alks well: 20 months
o 2-3 words sentence: 36 months
o Tells self name: 48 months
o Toilet control: 60 months
The suspected developmental delay must be assessed accurately
by applying different deveiopmental scales as appropriate for
the child.
Most of the etiologies of developmental delay can be ascer-
tained from morphological appearance and physical examina-
tion of the child. As for example by looking at abnormalities in
hair, head size, facial dysmorphism, eye, ear, skin, organomegaly,
etc. that may give a clue for underlying disorders, e.g., hypo-
thyroidism, Down syndrome, etc. Sometimes developmental
delay could be a manifestation of other diseases also, like
cerebral palsy, neurometabolic and neurodegenerative disorders.
By looking at developmental velociry the algorithm depicted
in Figure 18.1 will help to classi$' them.
The investigation plan depicted in Figure iB.2 can be
helpful in identifying etiologies in selected cases of develop-
mental delay.

There are not much data on the pharmacotherapy of conduct

disorder. Stimulants (like methylphenidate and d-amphet-
amine), lithium carbonate, carbamazepine, and antipsychot-
ics have all been tried without any proven efficacy.
Many different nonpharmacological treatments have been
for developmental velocity
used in the management of conduct disorder. These include
individual therapy based on alliance building and behavioral
principles. Family therapy designed to improve communica-
tion among family members and to elicit underlying conflict
is somewhat effective.
o Correctional schools can address the educational needs of
*# I
Static

ffi
Progressive
juvenile delinquents. ff

#
+
Neurometabolic$ Neurodegenerative$

Definition: Failure to achieve or slow progression towards age Fig. 18.1 : Algorithm for diagnosing cerebral palsy (CP), neuro-
appropriate development. degenerative and neurometabolic disorders. i
ESSENCE OF PEDIATRICS

lf history and clinical exam does not give any clue
First line
CBC, TORCH, Thyroid function, urine R/M/E, S. feritin,
S. Ca, P, Alk Phos, CPK, Fragile X chromosome, Lead,
biotinidase, Uric acid
MENTAT RETARDATION
The American Association of Mental Deficiency (AAMD)
defines mental retardation as a significantly subaverage general
intellectual ftrnctioning resulting in or associated with concur-
rent impairments in adaptive behavior and manifested during
the developmental period, before the age of 18. The child
has diminished learning capacity and does not adjust well
socially.

Eliology
A. Prenatal
1. Genetic: Galactosemia, phenylketonuria, gargoylism,
Gaucher disease, microcephaly, craniosynostosis, con-
Blood Urine MRI > CT genital hydrocephalus.
Ammonia, Orotic acid, 2. Chromosomil: Down syndrome, fragile X syndrome.
Lactate, Organic ). Abnormal pregnancy: Ivlainutrition, anemia, hyperten-
Transferrin, acid, GAGs, sion, infection with rubella, toxoplasma, cltomegalovirus,
Amino acides, Oligosaccharides syphilis, smoking, alcohol intake.
VLCFA,
Homocysteine, B. Perinatal
Carnitine
1. .Birth trauma, cerebral anoxia

Fig. 18.2: lnvestigation plan for identifying etiology in selected

cases of developmental delay.
Developmental disorders are a group of disorders resulting
fiom injury to the developing brain (prenatal, perinatal, or
postnatal).
Developmental disorders can be described as a severe chronic
disability that is attributed to a mental or physical impairment
or a combination of physical and mental impairment, which
is likely to continue indefinitely.
2. Plematuriry small for dates infants.
C. Post-natal
l. Infections: Meningitis, encephalitis
2. tauma: Head injury, subdural hematoma
3. Cerebrovascular episodes: Thrombosis of cerebral vessels
4. Endocrine andmetabolic: Hypothyroidism, hypogl,vcemia,
profound dvselectrolytemia, hyperbilirubinemia.
5. Others: Gross PEM in earlyinfancy, CNS malformation, lack
of stimulating environment, schoois with low standard.
Preventable mental retardation embraces such conditions as
hypothyroidism, galactosemia, phynylketonuria, birth trauma,
cerebral anoxia, prematurity, and PEM.

Included in this broad definition of developmental disor-

ders are: Clqssificolion
o Mental retardation Mental age
o Cerebral palsy IQ (intelligence Quotient) =
Chronological age
r Learning disability Mild mental retardation IQ level 55-70
o Attention deficit hyperactive disorder :

o Autistic spectrum disorders Moderate mental retardation : IQlevel 40-55

Severe mental retardation : IQlevel 25-40
These can occur in isolation or in combination (multiple Profound mental re taldarion : IQ level below 25
handicaps). Borderline mental retardation : IQ level 7l-84, is not within
Together developmental disabilities accounr for 15% of the the category of mental retar-
population of children. In modern day practice of medicine dation but may be a focus of
with improved obstetrical and neonatal care, substantial number psychiatric attention.
of infants born are premature, or suffer from severe perinatal Current pracrical classification of MR is: Mild-IQ 50-70;
adversities (hypoxic ischemic encephalopathies, meningiris, Severe-<50.
seizures) and will survive. At least a third of this population Developmental characteristics of mentally retarded children
will suffer from the developmental disabilities. are listed in Table 18.2.
 !

CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS

Table 18.2: Developmental Characteristics of Mentally Problems of Menlolly Retorded Children

Retarded Children
Behavioral problem, self injury ar.rd accidents, pica, autism,
CB epilepsy, toilet ploblerns, sleep disorders, eating problems,
social and sexual abuse, delinquency.

Profound Crossretardation:Minimal Some motor development

capacity for functioning in present, may respond to Diognosis
sensorimotor areas, needs minimal or Iimited training
nursing care, constant aid in self-help. History
and supervision required.
The history, most often taken from the parents with particular
Severe Poor motor develoPment, Can talk or learn to
attention to the mother! pregnancy, labor, and delivery; fam-
speech minimal, generally communicate, can be
unable to profit irom trained in elemental ily's pedigree (consanguinity of the parents); and hereditary
traininB in self-help, Iittle health habits, profits from disorders. Also, assessment of socio-cultural background, homet
or no communimtion systematic habit trai ni ng, emotional climate and the parent's intellectual functioning'
ski I Is. unable to profit from
vocational training.
Physical Examination
Moderale Can rall or learn lcr Can profit from training in
communicate, poor social social and occupational Configuration and size of the head offer clues to a variety
awareness, fair motor skills, unlikely to progress of conditions, e.g., microcephaly, hydrocephalus, and Down
development, profits beyond second grade level syndrome. Face may have some signs, e.g., hypertelorism, flat
from training in self-helP, in academic subjects, maY
nasal bridge, prominent eye brows, epicanthic folds, corneal
can be managed with learn to travel alone in
moderale superi i.ion. familiar places. opacities, retinal changes, low set and small ears, protruding
Mild Can develop social and Can Iearn academic skills tongue, and disturbance in dentition. The color and texture
communication skills, up to approximately sixth of the skin and hair, a high arched palate, size of the thyroid
minimal retardation in grade Ievel by late teens, gland, and the size of the child and his/her trunk at-rd extremi-
sens0rimolor areas, olten can be guided toward
ties should also be noticed.
not distinguished from social conformitv.
normal until later age. Neurological Examination Incidence and severity of neuro-
logical abnormalities rise in dilect proportion to the degree of
retardation. Sensory disturbances may include hearing difficul-
ties, ranging from cortical deafness to disturbances of spatiai
concepts, design recognition, and concepts of body image.
Colegories of Menlol Retqrdqlion Disturbances in motor areas are manifested in abnormalities in
muscle tone (spasticiry or hypotonia)' reflexes (hyper-reflexia),
Physiological MR In 40-60% cases, no causes are identifi-
and involuntary movements (choreoathetosis), clumsiness; poor
able; it includes patients with uqdifferentiated mental deficiency
co-ordination reveals smaller degree of disabii;ty.
without neurological deficit. There are no metabolic, infectious,
or structural anomalies of the brain. Maternal education level Psychological Assessment
and economic status are low. Parents are often of lower intel-
Psycl"rological testing performed bv an experienced psycholo-
ligence. Etiology may be postnatal trauma, neglect' iron defi-
gist is a standard part of evaluation for mental retardation. The
ciency, nutritional deficiency, poor environmental stimr-rlation,
Gessell and Bayley scales are most commonly used with infants.
marefnal infections.
\Techsler Preschool and Primary Scale of Intelligence (W"PSD
Pathological MR: Causes are usually identifiable. Children and Vechsler Intelligence Scale for the Children (\MSC) are
usually present with neurological deficit. most widely used. Stanford-Binet intelligence scalcs are also done.
o Chromosomal abnormalities (Down's and fragile-X consti-
Investigations
tute about 300/o)
o Teratogens and postnatal injuries (20-i0%) Exarnination of blood and urine for metabolic disotders, enzymatic
o CNS malformations (10-l5%) studies, homronal studies (T3,1'4, TSH), STORCH antibod-
o Hypothyroidism (3-5%o) ies, lurr-rbar plrncture for ir-rfections and antibodies. Karyoqping
for chromosomal abnornralities, x-rav skull for craniosvnostosis,
Treatable MR: Hypothyroidism and iodine deficiency, hvdrocephalus and othcr disorders that result in intracranial
early iron or trace element deficiency, LBW, perinatal calcificatior-r, e.g., toxoplasmosis, tuberor-rs sclerosis' etc. CT
insults, kernicterus (early), IEM (galactosemia, PKU), and scan and MRI for ir.rternal ht'drocephaius, col'tical atr-ophy, or
malnutrition. porencephaly. EEG can also be done. Amniocentesis and cho-
Preventable MR: Antenatal and perinatal hypoxia, neonatal rionic villi sampling are two screening techniques for detecting
sepsis, LBV, IEM (PKU), sociocultural causes. fetal abnorrnalities, and termination of pregnancl' can be done '
 I

ESSENCE OF PEDIATRICS

DSM-IV: Diagnostic criteria for mental retardation Table 18.3: Cerebral Palsy Types: Etiological
Significantly sub-average intellectual functioning: An IQ of
approximately 70 or below on an individually administered Spastic Perjventricular Prematurity
IQ test. diplegia leukomalacia
Concurrent deficits or impairments in present adaptive func- Periventricular
nemorrnagtc rnrarcilon
tioning, i.e., the child's effectiveness in meering the standards
expected for his/her age by his/her cultural groups in at Spastic Multicystic Peri natal/i ntrauteri ne
qudriplegia encephalopathy with hypoxic ischemic events
Ieast two of the following areas: communication, self-care, cortical atrophy.
home living, social/inter personal skills, use of community Selelctive neuronal
resources, self-direction, functionai academic skills, work, necrosis. Parasaggital
cerebral i njury. Cerebral
leisure, health, and safery.
malformations
r The onset is before age 18 years.
Spastic Cerebral injury MCA Cenetic, vasculopathy,
hemiplegia territory (infarction prenatai events
Treotmenl necrosis). Cerebral like hypoperfusion
teatment is based on each individual case, and a multidis- malformations hemorrhage

ciplinary approach may be required in many of the cases. Dyskinetic Basal ganglia Kern icterus
Status mermoratus Perinatal aspshyxia
Multidisciplinary approach includes the following:
urIrrutlrn deposrtron
o Specialized educational faciiities including a comprehensive Ataxic, Cerebellar lesions Perinatal aspshyxia,
program that addresses adaptive skills training, social skills hypotonic Enlarged ventricles Cenetic
training, and vocational training have often been a successful
format. Appropriate schooling is of utmost importance.
c Behavior, cognitive, and psychodynamic therapies: Positive Clossificolion of Cerebrql polsy
reinforcement for desired behavior and benign punishment 1. Majority are mixed type
(such as loss of privileges) for objectionable behavior may be
2. Spastic type
helpful. Cognitive therapy, such as dispelling false beliefs and
relaxation exercises with self instruction are recommended
a) Hemipiegic
to those who are able to follow insrrucrions.
b) Diplegic (Lower limb more severely affected )
r Physiotherapy, speech therapy, and psychiatric rreatment.
c) Quadreplegic
r Aggression and self injurious behavior: Lithium is useful.
d) Monoplagic (Rare)
Naltrexone is used in patients associated with autism. Car- 3. Dyskinetic (Chorioathetosis,tremor ,rigidiry & dystonia )
bamazepine and valproic acid are also useful in some cases 4. Ataxic
having seizure.
o Stereorypical motor movemenrs: Haloperidol and chlorpro- Etiology (Toble 18.3)
mazine decrease repetirive self-stimulatory behavior. Perinatal asphp<ia, birth trauma, intraventricular hemorrhage,
e Propranolol and bispirone are used to decrease explosive hypoglycemia, infection, kernicte rus, congenital brain
rage behavior. malformations.
o Other treatments include maintenance of adequate nurrition,
treatment of hearing and vision problems.
c Co-morbidities
Tieatable etiology, if found, should be treated early.
o Mental retardation: 30o%
Preventable mental retardation embraces conditions like birrh o Epilepsy: 30%
trauma, perinatal asphJxia, prematurity, congenital rubella o Visual problem: Squirrt. J visual acquiry
-.yncirome, hypoglycemia, hypothyroidism, hyperbilirubinemia, o l Hearing
PEI-4, galactosemia, and phenylketonuria.
r Speech dilijculrv
o Cortical sensory deficirs: Abnormalities of proprioception
CERTBRAT PATSY and tactile sensory deficits
tlel:l;r:.rl palsy (CP) is a disorder of posture and movement
o Feeding di{hculry

resuiting from permanent non-progressive insult to

r Behaviour problem

developing brain. The motor disorders of cerebral palsy are

o Sleep problems

often accompanied by disturbances of sensarion, perceprion,

cognition, communicarion, and behavior, by epileps,v, and by
Diognosis
secondary musculoskeletal problems. The diagnosis is clinical:

CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS
. H/O probiem related to posture and movement.
e Signs related to centre's controlling posture and movement
should elicit neurological signs related to pyramidal, extra-
pyramidal, or cerebellar involvement depending on type
of cerebral palsy.
o Delayed milesmnes of development.
In addition, there may be:
o Developmental delay, which is improving over time indi-
cating static encephalopathy. This can be assessed from
developmental velocity.
o H/O brain insuit (t).
. Persistance of primitive reflexes.
Differenliql Diognosis
o Developmental delay (idiopathic and maturational)
o Neurodegenerative disorders:
o Tay-Sach disease
o Krabbe disease
o Metachromaticleukodystrophy
o Neurometabolic disorders: Organic aciduria like glutaric
t) aciduria (dystonic)
t more of the basic psychological processes involved in understand-
I ing as using language, written or spoken, which may manifest
t Monogemenl
)
t- Multidisciplinary:
I o Early stimulation: Visual, auditory, tactile, speech, emotion
I o Management of spasticity:
I The term specific learning disability does not include children
F o PhysiotherapyJ occupational therapy: Better training for
activities of daily living (ADL) like feeding, bathing,
dressing, toilet training, antispasticiry agents, intrathe-
cal baciofen, botulinum toxin, phenol block, splints and
braces, orthotics.
r Surgery: After gait maturation at 6-10 years. Tenotomy,
tendon lengtheningitransfer, selective dorsal rhizotomy
and femoral osteotomy can be done.
facilitates postsynaptic action of GABA; dose 0.2-
0.8 mg/kg, 3-4 div. doses; Clonazepam 0.1-0.2 mglkg,
2-3 div. doses. Tizanidine, central cr-2 noradrenergic
agonist, 6 mgld. Gabaergic-Baclofen 2.5 mgld -+
titrate up till 20-60 mg/d. Injection Botox can be given
in a planned way, Baclofen can also be given.
Theatment of associated conditions:
r Drooling: Atropine, Benztropine, Botox
r Behaviour problem: Haloperidol, Buspirone
r Sleep problem: Melatonin 2 mgVz hr before sleep
Management of dyskinetic CP:
r Thihexiphenidyl hydrochloride (Artane/Pacitane)
0.03 mg/kg/d l-2 div. dose or
!
,; 0.2mglkgld-0.5 > 1.0> 1.5=>2.0=2.5mglkgld
in 3 div.
doses on week 1 to week 6, respectively.
o Levo-dopa trial can be given if no definite cause is found.
o Necessary counseling should be done.
Prevenlion
Prevention of maternal infection, perinatal asphyxia, birth
trauma, LBW baby, neonatal sepsis, dyselectrolytemia, convul-
sion, jaundice, etc.
TEARNING DISABITITIES
Learning disability, scholastic bachvardness, school failure are
common terms applied to children who experience dificul-
ties in coping with academic skills. It is found in 1-10% of
school-aged children.
Clqssificotion
Global: It occurs when the child has di{ficulties in all the facul-
ties, which is usually the result of subnormal intelligence.
Specific Specific learning disabilities means a disorder in one or
itself in an imperfect abiiity to listen, think, speak, read, write,
spell or do mathematical calculation. The term includes such
conditions as perceptual handicaps, brain injury, minimal brain
dysfunction, dyslexia, and developmental aphasia.
who have learning problems that are primarily the result of
visual, hearing or motor handicaps, or mental retardation, or
emotional disturbance, or environmental, cultural or economic
disadvantages.
Etiology
No specific cause for learning disabilities is commonly accepted.
Most likely, a number of subgroups of children who have spe-
cific learning disabilities will be identified. Etiologic hypotheses
include central nervous system damage, individual human
variation, toxins, diet, and environmental factors.
Assessment
History: Elements of the history should include:
r Review of the perinatal course
o Evidence of medical problems (e.g., persistent otitis media,
seizure disorders)
o Early developmental history with an emphasis on language
acquisition (there is often an uneven profile in the develop-
ment of children who have learning disabilities)
a History of other family members who have learning problems
a Review oF school lunctioning
ESSENCE OF PEDIATRICS
Physical examination findings are usually negarive.
o An emphasis is often placed on a search for minor neurologic
indicators, or "soft signs," which have been reported more
frequently in learning disabled children than in controis
(e.g., synkinesis fmirror movements]; dysdiadochokinesia
[difficulty with rapid, alternating movements], choreoform
movements of the fingers). The implications of these signs
remain controversial.
o Hearing and vision should be screened.
Laboratory investigation is not called for unless it is suggested
by the history or physical examination. Computed tomography
(CT) scan and electroencephaiography are not helpful.
Psychoeducational assessment includes a battery of tests of
intellectual functioning (IQ tests) as well as specific academic
tests to profile a childt strengths and weaknesses.
Treolment
o Educational intervention is the mainstay of treatment. Typi-
cally, this occurs through a modification of the child's regular
classroom experience or byvarying degrees ofspecial education,
ranging from resource room support to a separate classroom.
The educational pendulum has swung strongly toward inclu-
sion of most children, even those who have significant deficits.
Educational intervention should identifz specific goals (i.e.,
should be individualized) and should be monitored carefully.
r Psychological counseling is indicated for children with learn-
ing disabilities who suffer from diminished self-esteem that is
not improved by a special education program. School phobia
can develop in children who have learning disabilities, and
this problem can be addressed in counseling.
r Various support organizations are helpful in providing
parents and teachers with a forum to address these complex
issues associated with these disabilities.
ATTENTION DEFICIT HYPERACTIVITY DISORDER
Diognosis
Diagnostic criteria DSM-IV TR:
A. Either (1) or (2)
(1) Six or more of the following symptoms of inattention have
persisted for at least 6 months to a degree that is maladap-
tive and inconsistent with the developmental level:
Inattention
o Often fails to give close attention to details or makes care-
Iess mistakes in schoolwork, work, or other activities
o Often has difficulq' sustaining attention in tasks or play
activities
o Often does not seem to listen when spoken to directly
r Often does not follow through on instructions and
fails to finish schoolwork, chores, or duties in the
workplace (not due to oppositional behavior or failure
to understand instructions)
o Often has difficulty organizing tasks and acriviries
e Often avoids, dislikes, or is reluctant ro engage in tasks
that require sustained mental effort (such as schoolwork
or homework)
o Often loses things necessary for tasks or activities (e.g.,
toys, school assignments, pencils, books, or tools)
o Is often easily distracted by extraneolls stimuli
o Is often forgetful in daily activities
(2) Six (or more) of the following symptoms of hyperactiv-
ity-impulsivity have persisted for at least 6 months to
a degree that is maladaptive and inconsistent with the
developmental level:
H4leractivity
r Often fidgets with hands or feet or squirms in seat
o Often leaves seat in classroom or in other situations
in which remaining seated is expected
o Often runs about or climbs excessively in situations in
which it is inappropriate (in adolescenrs or adults, may
be limited to subjective feelings of restlessness)
o Often has dilficulry playrng or engaging in leisure
activities quietly
o Is often "on the go" or often acts as if "driven by a
motor"
r Often talks excessively
Impulsivity
. Often blurts out answers before questions have been
completed
o Often has difficulty awaiting turn
e Often interrupts or intrudes on others, e.g., butts into
conversations or games
B. Some hyperactivity-impulsive or inattentive symptoms that
cause impairment were present before the age of 7 years.
C. Some impairment from the symptoms is present in more than
two or more settings (e.g., at school [or work] or at home).
D. There must be clear evidence of clinically significant impair-
ment in social, academic, or occupational functioning.
E. The symptoms do not occur exclusively during the course
of a pervasive developmental disorder, schizophrenia, or orher
psychotic disorder, and are not betrer accounted for by another
mental disorder (e.g., mood disorder, anxiery disorder, dissocia-
tive disorder, or a personality disorder).
Monogemenl
1. Behavior modification
2. Pharmacological treatment
a. Methylphenidate: l-2 mglkgld in 2 divided doses.
Start rvith 0.3 mg/kg in the morning (rounded to 5 I
mg), titrate this morning dose to 0.6 mg/kg after 2 t
t
weeks if inadequate response. An extra dose of 0.3
mg/kg can be added in the afternoon if indicated. ?
I
 CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS

b. Clonidine: 0.003-0.01 mg/kg in 2 divided doses' (z) language as used in social communication, or (iii) s).nnbolic
c. Atomoxetine: 0.5-1.5 mg/kg/d in 2 divided doses. or imaginative play.
3. Follow-up: Pretreatment assessment and follow-up with C. The disturbance is not better accounted for by Rett disorder
Conner rating scale. or childhood disintegrative disorder.

PERVASIVE DEVELOPMENTAI DISORDERS Differential Diagnosis

Speech and language regression without seizures:
Autistic Disorder
o Autism spectrum disorders
Diagnosis r Landau-Kleffner dysphasia
Diagnostic criteria DSM-IV TR:
o Language and cognitive deterioration with continuous spike
and wave in slow wave sleep (CS\7S)
A. A total of six (or more) items from (1), (2), and (3), rvith
at least nvo from (1), and one each from (2) and (3).
o Speech deterioration in the epileptic syndrome of congenital
bilateral perisylvian syndrome and opercular syndromes
(1) Qualitative impairment in social interaction, as manifested
o Late-onset sensorineural hearing loss
by at least two of the following: o Elective mutism
a. Marked impairment in the use of multiple nonverbal
behaviors such as eye-to-eye gaze, faciaJ'expression, body
Management
postures, and gestures to regulate social interaction
I b. Failure to develop peer relationships appropriate to o Early intervention to improve speech and communica-
; developmental level tion: Most interventions in ASD are evaiuated against
I c. A lack of spontaneous seeking to share enjoyment, outcomes such as cognition, language, or amelioration of
r interests, or achievements with other people (..g', by maladaptive behaviors rather than by qualiry of life.
I a lack of showing, bringing, or pointing out objects o Pharmacological treatment They are generally considered for
I of interest) the treatment of comorbid psychiatric or neurodevelopmental
I d. Lack of social or emotional reciprocity conditions in ASD and are usually a short to medium
) term intervention. Pharmacological treatments include
F (2) Qualitative impairments in communication as manifested
Risperidone 0.5-3.5 mg/d for 3-4 months for the short-
t by at least one of the following: term treatme m of signifcant aggression. Methylphenidate:
I a. Delay in, or total lack of, the development of spoken start with low dose (5 mg/d) in the morning and gradually
language (not accompanied by an attempt to com- titrate up to 50 mg/d in exceptional cases, for the treatment
t pensate through alternative modes of communication of attention dfficubies and hyperactiuity. Melttonin 3 mg
: such as gesture or mime) at bed time for 2 weeks for the treatment of innactable
!.
b. In individuals with adequate speech, marked impair- sleep problems.
I' ment in the ability to initiate or sustain a conversation
with others
t c. Stereoryped and repetitive use oflanguage or idiosyn- Rett Syndrome
lI cratic language Diagnostic criteria DSM-IV TR:
d. Lack of varied, spontaneous make-believe play or social
A. All of the following:
I imitative play approoriate to developmental level
I (3) Restricted repetitive and stereoryped patterns of behavior, (1) Apparently normal prenatal and perinatal development.
interests, and activities, as manifested by at least two of (2) Apparently normal psychomotor development through
the following: the first 5 months after birth.
t a. Encompassing preoccupation with one or more ste-
(3) Normal head circumference at birth.
v
reotyped and restricted patterns of interest that is B. Onset of all of the following after the period of normal
abnormal either in intensiry or focus development:
b. Apparently inflexible adherence to specific, nonfunc- (1) Deceleration of head growth berween ages 5 and 48
tional routines or rituals months.
c. Stereotyped and repetitive motor mannerisms (e.g., (2) Loss of previously acquired purposefui hand skills benveen
hand or finger flapping or rwisting, or complex whole- ages 5 and 30 months with the subsequent development

t; d.
body movements)
Persistent preoccupation with parts of objects
of stereoryped hand movements (i.e., hand-wringing or
hand washing).
(3)
I B. Delays or abnormal functioning in at least one of the following
areas, with onset prior to age 3 years: (z) social interaction,
Loss of social engagement early in the course (although
often social interaction develops later).

h
ESSENCE OF PEDIATRICS

(4) Appearance of poorly coordinated gait or trunk move, B. Restricted, repetitive, and stereotyped patterns of behavior,
ments. interests, and activities, as manifested by at least one of the
(5) Severely impaired expressive and receptive language devel, following:
opment with severe psychomotor retardation.
Encompassing preoccupation with one or more srereo-
typed and restricted parrerns of interest that is abnormal
Childhood Disintegrolive Disorder either in intensity or focus
(2) Apparently inflexible adherence to specific, nonfunctional
Diagnostic citeria DSM-IV TR:
routines or rituais
A. Apparently normal development for at least first 2 years (3) Stereotyped and repetitive motor mannerisms (e.g., hand
after birth as manifested by the presence of age-appropriate
or finger fapping or rwisting, or complex whole-body
verbai and nonverbal communication, social relationships, play,
movements)
and adaptive behavior.
(4) Persistent preoccupation with parts of objects
B. Clinically significant loss of previously acquired skills (before
C. The disturbance causes clinically significant impairment in
age l0 years) in at least two of the following areas:
social, occupational, or other important areas of functioning.
(1) Expressive or receptive language
(2) Social skills or adaptive behavior D. There is no clinicaliy significant general delay in language
(3) (e.g., single words used by age 2 years, communicative phrases
Bowel or bladder control
(4) Play used by age 3 years).
(5) Motor skills E. There is no clinically significant delay in cognitive devel-
C. Abnormalities of functioning in at least rwo of the fol- opment or in the development of age-appropriate self-help
lowing areas:
skills, adaptive behavior (other than in social interaction), and
curiosity about the environmenr in childhood.
(1) Qualitative impairment in social interacrion
(e.g., impair-..ri i.r nonverbal behaviors, failure to E Criteriaare not met for another specific pervasive develop-
develop peer relationships, lack of social or emotional mental disorder or schizophrenia.
rcciprocity)
(2) Qualitative impairments in communication (e.g., delay or
lack of spoken language, inability to initiate or sustain a
Atypicol Aulism
conversation, stereotyped and repetitive use of language, Pervasive developmental disorder, not otherwise specified is
lack of varied make-believe play) stated as atypical aurism. This category should be used when
(3) Restricted, repetitive, and stereotyped patterns ofbehavior, there is a severe and pervasive impairment in the developmenr
interests, and activities, inciuding motor srereotypies and of reciprocal social interaction or verbal and nonverbal com-
mannerisms munication skills, or when stereoryped behavior, interests, and
activities are present, but the criteria are not met for a specific
D. The disturbance is not better accounted for by another spe-
pervasive deveiopmental disorder, schizophrenia, schizotypal
cific pervasive developmental disorder or by schizophrenia.
personality disorder, or avoidant personality disorder. For
example, this category includes "atypical
"n,irt-1i'-presenta-
Asperger Syndrome tions that do not meet the criteria for autistic disorder because
of late age of onset, atypical symptomatology, or subthreshold
Diagnostic criteria DSM-IV TR:
symptomatology, or all of these.
A. Qualitative impairment in social interaction, as manifested
by at leasr rwo oF rhe lollowing:
(1) Marked impairment in the use of multiple nonverbal
behaviors, such as eye-to-eye gaze, facial expression, body
postures, and gestures to regulate social interaction A colicky infant is one who is healthy and well-fed but cries
(2) Faiiure to develop peer relationships appropriate to devel- >3 hours a day for >3 days a week and for >3 weeks-the rule
opmental level of three's ('\Tessel).
(3) Lack of spontaneous seeking to share enjoyment, inter- The problem usually starts within the first week after birth,
ests, or achievements with other people (e.g., by a lack reaches a peak at the age of 4-6 weeks and improves after 4_5
of showing, bringing, or pointing out objects of interesr months of age in 80% of cases. It is observed in up to 15%
to other people) of otherwise healthy newborns. It does nor have any adverse
(4) Lack of social or emotional reciprocity efrect on health.

A
I
 CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS
ETIOTOGY
The cause of infantile colic remains unknown. Postulated impor-
tant factors have included abnormal mother infant interaction,
protein allergy, hormonal imbalances, and increased sensitiviry
to colonic distension.
DIAGNOSIS
o A clinical diagnosis is based on a characteristic history (e.g.,
pulling up of legs during paroxysms, often with a change of
facial color to bright red; dilficulry with defecation despite
soft stools; inconsolability) and negative findings from
physical examination.
o Other causes of irritability (e.g., protein allergy, hernia,
gastroesophageal reflux) must be excluded.
TREATMENT
Parents should be counseled and reassured about the tran-
sient nature of the illness.
The baby should be fed in upright position and burped to
promote egress of swallowed wind. He/she should be put
in bed on right side for 10-15 minutes.
Crib attachments simulating car travel and rocking may
be helpFul.
Phenobarbitone, dicyclomine, and simethicone (i.e., flacol)
have been found to be somehow helpful, but their use is to
be discouraged because of risk of adverse reactions.
A trial of changing the feedings, eliminating cow milk from
the formula or from the mother's diet are sometimes beneficial.
It presents with somatic symptom and/or dysfunctions, which
the patient actually experiences but for which physical findings
are absent to explain the symptoms. The conditions causing
somatoform disorder are described below.
Body dysmorphic disorder: It is characterized by extreme
preoccupation with perceived abnormdities in appearance when
perception is not reflective ofthe actual physical features. The
anxious obsession is associated with social limitations.
Conversion disorder: It of physical functioning without
is loss
demonstrable organic pathology. Such reaction may take the
form of blindness, paralysis, diplopia, pseudoseizure, and
hyperventilation. Physical symptoms starts suddenly, do not
show objective abnormalities. The history may show those
relationships with someone who exhibited similar symptoms.
Atasia-abasia is a conversion disorder manifests as an inabiliry
to stand or work. Post ictal serum prolactin level (eievated in
true seizure) is useful in diagnosing pseudoseizure.
Hypochondriasis: Presenting complaint is a physical sign or
symptoms. Physical sign and symptoms are normal, may be
related to depression or anxiery. Patients interpret the physical
symptoms to indicate disease. Symptom does not respond to
reassuarance; medication directed at underlying psychologic
problems often helps.
Somatization disorder: Each of the following criteria must
have been met with the individual symptom occurring at any
dme during the course of disturbance: Four pain symptoms,
Two gastrointestinal symptoms, One sexual symptom, One
pseudoneurologic symptom.
Presenting complaints are usually >13 physical symptoms in
girls and >11 in boys. Physical symptoms are caused by a
known physiologic or pathologic mechanism; it is related to
the need to maintain the sick role. Symptoms tend either to
persist or to change character despite treatment.
Somatoform pain disorder: Pain at >1 anatomic sites is
predominant focus clinical presentation and is of su{ficient
severiry to warrant attention. The pain causes significant dis-
tress or impairment in social life. Psychological factors are just
to have an important role. The symptom is not intentionally
produced as in factitious disorder or malingering. If duradon
of pain is <6 months, pain is acute; when duration is >6
months, it is chronic.
Malingering: Presenting complain is physical symptom and is
under the voluntary control. Symptom is used to gain reward
(that is money, extra parental attention, avoidance of service
and school).
Factitious disorder: Presenting complaint is a symptom
complex mimicking a known syndrome, which is under vol-
untary control. Symptom complex is used to attain medical
treatment (including surgery). Symptom complex often results
in multiple diagnosis and multiple operations.
TREATMENT
a Psychotherapy especially to desensitize the stress factors.
a It is essential to explore whether there is a gain that may
result from the sick role.
Sometimes, parental treatment is necessary to ensure the
favorable outcome.
Interventions that address the psychologic factor of both
the child and family by educational approaches.
Emphasis should be given to early return to the normal
activities including school, recreation, and socialization
with peers.
Ideally, parents and children should be made to understand
the psychologic origin of the symptoms.
Medication may be useful in anxiery or in depression.
ESSENCE OF PEDIATRICS

TEETH GRINDING OR BRUXISM

This is characterized by the periodicity ofattacks and absence

It results from tei;sion originating in unexpressed anger or
resentment. It may create problem in dental occlusion. It is
of signs of physical disease between attacks. The best known
usually not associated with helminthiasis.
form of the syndrome is cyclical vomiting in which there
are recurrent attacks of severe vomiting that last for 12-72
hours. Attacks seem sometimes to be precipitated by upper
Treolmenl
respiratory infections or emotional upsets. Headache may Assurance. Helping the child to find ways to express resenr-
be a constant feature. Recovery always cccurs spontaneously, ment may relieve the problem.
and after the attack, the child may seem to be exceptionally
well and vigorous.
THUMB SUCKING AND NAIT BITING
Attacks of para-umbilical abdominal pain (button pain) are
frequently associated with headache, while vomiting is slight Thumb sucking or nail biting indicates that a pleasurable sensa-
or absent. Although cyclical vomiting or attacks of periodic tion is derived by the child from this self-stimulation. Thumb
abdominal pain usuaily disappear at puberty, a considerable sucking may interfere with dental alignment, may increase the
proportion of these patients develop migraine during adoles- incidence ol helminthiasis.
cence or early adult life.

Treoimenl
TREATMENT A bitter solution may be applied on the thumb to discour-
age the child from thumb sucking. Antihelminthic may be
o An appropriate start is to explain the nature of the condition
needed.
to the parents. They should be assured that the attacks will
not harm childb future health.
o In cyciical vomiting, sips of giucose drinks should be MASTU RBATION
given frequently. Vomiting can often be stopped with IM
promethazine Hcl, given 8-hourly, to be followed by oral The child may obtain pleasure by genital stimulation, rubbing
administration for a day or two after the vomiting has of thighs against each other, or by rhphmic swaying movement.
stopped. Dehydration should be corrected by IV saline,
when present.
Treolmenl
o Undue excitement and overprotection should be avoided.
o Parental anxiety should be allayed, as this is generally
Note: The periodic nature ofthe attacks and the finding ofEEG abnor-
harmless.
malities in some patients have led some pediatricians to suggest that the
periodic syndrome is a variety of epilepsy. o In severe cases, psychiatric treatment may be necessary.

Trcs
Tics are sudden, rapid, repetitive involuntary purposeless
movement of circumscribed muscle groups. Examples of tics
Habit disorders include tension discharging phenomenon such
include lip smacking, grimacing, tongue thrusting, eye blink-
as head banging and rocking, teeth grinding, thumb sucking
ing, throat clearing, not accompanied by loss of consciousness.
and nail biting, masturbation, and tics.
Tics are not seen during sleeps, can be controlled voluntarily
for short period.
HEAD BANGING OR ROCKING IN BED
A toddler who is fatigued or is under stress may bang his head Treolmenl
against the bed, or rock it in rhythmic movements often seen o Behavior therapy using massed practice of the tics in which
in mentally retarded child or in emotional deprivation. Prob- the child is taught consciously to carry out and then stop
ably this gives them a pleasurable sensation. the abnormal movement, or relaxation produces definite
improvement in 30Vo.
o Haloperidol 0.05-0.1 mglkgld is justified if tics are severe
Treolment and handicapping. Anti-parkinsonian drug, benztropine
o Assurance: Treat mental retardation, if remains associated. mesylate 0.5-2 mgld should be added. l
o The bed should be padded to prevent injury. o Psychotherapy does not cause improvement.
t'
t
lr
4
 CHILD PSYCHIATRY AND DEVELOPMENTAL DISORDERS

Table 18.4: Psychopharmacology: Drugs, lndications, Dosage, Side Effects

Antipsychotics
Low potency: Thioridazine,
Chlorpromazine
Mid-potency Mescridazine
High potency: Trifluoperazine,
Haloperidol
AII classes: Severe rgitation
schizophrenia; mania, autism,
extreme aggressiveness,
High-potency class: Cills de
Tourette syndrome; other tic
disorders (haloperidol)
Low potency: 30-1 50 mg/24 hr
in divided doses; avarlable in
concentrated forn-r
Mid-potency: 10-75 mg/24 hr in
la
divided doses
High Potency: l-5 mg24 hr in
divided doses
All classes: Sedation, \ ,eight gain,
anticholinergic effects (dry mouth,
blr.rrred vision, constipation);
hypersensitive reaction (hepatic,
skin); blood dyscrasias,
parkinsonism
Longterm effects: risk of tardive
dyskinesia

Stimulants lnsomnia, decreased appetite,

(6 yr and older) possible weight loss, irritability
Methylphenidate Attention deficit disorder 0.3-1 .0 mg/kg/24 hr and lenrtulners. ahdonrin.rl pain.
Dextroamphetamine Attention deficit disorder 0.2-0.5 mg/kg/24 hr headache; elevated systol ic
Pemoline Attention deficit disorder 37.s-112.s m{k{2a hr blood pressure; development and
worsening of tics. A long{erm
effect may be height and weight
reduction. Pernoline is associated
with h),persensitivity reaction,
especially hepatic-

Antidepressants
Desipramine Major depressive disorder,
attention deficit disorder
unresponsive to stimulants
(12 yr and older)
Flrroxetirre Mild depression and anxiety
For major depressive disorder and
separation anxiety, 2-3 m{kn2a
hr in divided doses (blood levels;
therapeutic, 1 00-250 ng/ml)
10-30 mg/24 hr
ECC and blood pressure shorlld
be monitored for hypertension,
orthostatic hypotension, cardiac
arrl'rythmia, or lengthening of PR
or QRS irrterval, Monitor plasma
level. lor therapctrlic ranqe.
Agitation, headaches, anxiety,
rnsomnia, weight loss; binds
tenaciously to proteins and has a
long half-life.

Mood stabilizers Mania, some cases of unipolar 600-1 200 m{24 hr (blood levels; Castroi ntestina I distr-rrbance,
Lithium carbonate i lness extreme aggression
I therapeutic 0.6-1 .2 mEq/L) tremor, atax'a, confusion, coma,
death, hypothyroidism
Carbamazepine Mania, aggression. .el[-iniuriou' 400-1 000 mg/24 hr (blood levels; Fever, sore throat, hematological
I therapeutic B-1 2 pg/rrl) problems, drowsiness, neuro-
behavior in organically impaired
patients muscular disturbance
Anti hypertensives ADHD not responding to 0.1 -0.25 m{24 hr Bradycardia, hypotension.
It Clonidine stimulants
ADHD with aggression

>

GILLES DE tA TOURETTE SYNDROME The disorder usually persists throughout life, but studies
have shown a diminution in s1'mp1611r in half to t\,vo-thirds
Itis a special type of tics disorder characterized by multiple of cases 10-15 years after the initial evaluation.
motor tics, compulsive barking, grunting or shouting obscene
words. Children with Gilles de la Tourette svndrome often
I
suffer from secondary behavioral, emotional, and academic
iI problems. Many environmental precipitants have been noted
I See Table 18.4
to serve as emotional stfessors, which also precipitate or worsen
tics and Gilles de la Tourette syndrome.

Treotmenl l. Behrman RE, Kleigman RN, Jensen HB. Nelson Tixtbook of

Mainstay of pharmacotherapy of this condition is dopamine Pedirttrics 18'1' ecl. Singapole: Harcourt Asia Pte Ltd., 2007.
antagonists, e.g., haloperidol or pimozide. Clonidine, carba- ). Hutchir^on JH, Cockburn F. Practirdl Paediatric Problnts 6'\' ed.
mazepine, and clonazepam have aiso been tried. Singapore: PG Publishing Pte Ltd., 1986.
 !

ESSENCE OF PEDIATRICS

3. Ghai OB Gupta P, Paul VK. Essential Pediatrio 7'r'ed. New Delhi: 8. Courchesne E. Neuroanatomic imeging in aurism. Pediatrics
CBS Publishers, 2009. 7991;87:781-90.
4. Clayden G, Hawkins R (ed.). Paedianics, Ileatment and Prognosis 9. Rosenbloorn I-. Diagnosis and management of cerebral palsy. Arch
1" ed. New Delhi: laypee Brothers, 1989. Dis Child 1995;72:350-4.
5. Dworkin PlH. NMS: Pediatrics 4'r' ed. Philadelphia: Lippincott 10. SwaimanKF.PediatricNeurologl:Principles&Practice4'r'ed.Mosby
\Williams and \Wilkins, 2000. Elsevieq 2006:1098.
6. AmericanPsychiatricAssociation.DiagnosticandStatistical Manua/ tr1" -{benc{ NS, Marsh E. Convrrlsive anci loncor.lvLllsi\E si;rrris
of Mental Disorders 4'r'ed. Washington f)C: APA, 1994. cpilepricr-rs ir ch:ildren. Current T^eatvnent Options in Neurologt
7 . Parthasarathy A (ed). IAP Texrbooh of Pediatriu.4'r' ed. New Delhi: 2009;11:262-72.
Jaypee Brothers, 2009.
 L9
CHAPTER

Dermatology

Chopler Conlents
Co^tan dermat,tis. .......,. .....351
Scabies....... 5:perlicial {unga' in{eorons.. .... . ... . .. .351

Pedrculosrs.............. ..............................348

Urticaria, angioedema, and anaphylactic sh0ck............ 349 Piryriasis a1ba...... ... 154
lnfantile seborrheic eczema................................................349 Ph otosensitivity.. 454

.,....,................350

Table 19.1: Hemangioma Complications and Their Treatment

Primarily composed of capillaries and is characterized by Severe u Iceration/maceration Encourage twice daily cleansing
endothelial ceii proliferation (proliferative hamartomas of regimen

vascular endothelium). Hemangiomas should be classified as Dilute sodium bicarbonate soaks

superficial, deep, or rnixed. - Flashlamp pulsed dye laser
The immunohistochemical marker GLUT-I separates t Oral corlicosleroids
hemangiomas from the other vascular tumors of infancy.
t Metronidazole creAm
Superficial hemangiomas are bright red, protubet:ant,
Bleeding (not KMP) Celfoam or Surgifoam
compressible, sharply demarcated lesions that may occur on
Compression therapy + embol ization
any area of the body. Sometimes present at birth, they more
Hemangioma with Patching therapy as directed by
often appear in the first rwo montirs and are heralded by an
ophthalmologic sequelae ophthalmologist
erythematous or blue mark or an area of pallor. The presenting
lopical vs. inlrale>ional vs. oral
sign may occasionally be an ulceration of the perineum or lip.
corl ico5teroids
Giris are affected more often than boys. Face, scalp, back, and
Subglottic hemangiona f)ral r ortit osleroicls r KIP laser
anterior chest are the favored sites; lesions may be solitary or
Traclreolomy if required
multiple. Patterns of facial involvement include frontotemporal,
rnaxillary, mandibular, and frontonasal regions. Kasabach-Merritt Corlicosteroids, aminot aproic acid.
phenomenon vincristine, interferon-o t embolization
Deep hemangiomas: Hemangiomas that are mole deeply
situated are more diffuse and are less defined than superficial HighJlow lrepatic Corli(o)leroids or intederorr t
hemangioma embolization
hemangiomas. The lesions are cystic, firm, or compressible,
and the overlying skin may appear normal ir-r colol or have
a bluish hue. TREATMENT
Most hemangiomas are mixed hemangiomas and have both
superficial and deep components. Rapid expansion, stationarv Management must be conservative, and surgical interven-
period, and spontaneous involution ale the phases. Blancl-red or tion should be avoided. Because almost all lesions regress
pale gray areas indicate fibrosis and regressio n. 600/o involution spontaneously, therapy is rarely indicated and may cause
l by 5 year and 90-95o/o by 9 year of age, but 1ip lesions may further harm. Residual telangiectasias may be treated with
persist longer. Complications include ulceration, secondary pulsed dye laser (PDL).
infection, and rarely, hemorrhage. Thble 19. i lists hemangioma In the rare case in which intervention is required, if the
t complications and their treatment. lesion is very superficial, early therapy with PDL may be

I
ESSENCE OF PEDIATRICS

beneficial in decreasing growth of the hemangioma. PDL is
also useful for the treatment of small (<4-5 cm) ulcerated
hemangiomas.
o Indications for active treatment are those lesions that, by
virtue of their size and site, compromise vital structures, such
as the airway or the eyes. in this emergency situation, the
treatment of choice is a short course of oral prednisolone
(2 mglkgld), single morning dose given for 4-6 weeks,
and then tapered.
o Periorbital hemangioma can be treated by intralesional
steroids.
r Interferon 2-cr inhibits angiogenesis. It should be used only
for life-threatening hemangiomas refractory to corticosteroid
therapy.
o Gamma benzene hexachloride (Lorexane): Single 24 hour
application.
o Monosulphiram (Tetrasol): Applied as benzyl benzoate.
May be repeated after I week. Should be diluted before
use. Application may inciude scalp and face, if lesions are
present.
o Pruritus ot eczematization may be alleviated by an anti-
histamine and a topical corticosteroid preparation (i.e.,
dermasol ointment).
Note:
1. Clothing, bed linens should be cleansed by boiling, sunned, and
subject to ironing.
2. All members of the lamiiy must be treated whether or not they
exhibit symptoms (asymptomatic carrier), otherwise treatment failure
or relapses will result.

Scabies is caused by the arthropod (has four sets of legs), by

burrowing and release of toxic or antigenic substances by the Caused by lice-body or clothing lice (Pediculus humanus
female mite Sarcoptes scabiei and transmitted by direct contact. corporis), head lice (Pediculus humanus cdpith), and pubic or
crab lice (Phthirus pubi). Both nymphs and adult lice feed on
Diagnostic triad: human blood, injecting their salivary juices into the host and
l. Nocturnal itch depositing their fecal marter on rhe skin. The hallmark of all
2. Particular distributions of lesions types of pediculosis is pruritus.
3. Presence of disease in other family members (e.g.,
mother)
TREATMENT
Lesions:
Intensely pruritic rash (with papules, vesicles, or pustules) o Pediculosis capitis: Permethrin 1o/o cream rinse applied for
serpiginous and burrows, distributed in the interdigital spaces 10 minutes with a repeat application in7-10 days, natural
of the fingers, front of the wrist, the axillary folds, around the pyrethrin shampoos, and lo/o lindane shampoo for 10
umbilicus, and on the genitalia. Palms, soles, face, and head minutes with a repeat application in 7-10 days. Malathion
are also affected in infants. 0.5o/o in isopropanol is FDA approved for the treatment of
head iice and should be applied to dry hair until hair and
DIAGNOSIS scalp are wet, and left on for 12 hours.
o All household members should be treated at the same
Scrapings of the burrows with mineral oil can reveal the mite. time.
o Nits can be removed with a fine-toothed comb after ap-
coMPucATroNs plication of a damp towel to the scalp for 30 minutes.
c Clothing and bed linens should be laundered in very hot
Secondary bacterial infections, eczematization, post-strepto- water or dry-cleaned; brushes and combs should be dis-
coccal glomerulonephritis. carded or coated with a pediculicide for 15 minutes and
then thoroughly cleaned in boiling water.
TREATMENT Pediculosis corporis: Permethrin cream applied for 8-12
hour can be used to eradicate any eggs and lice that happen
Begin with hot bath with soap and water. Initial course of
to be on body hair. Clothing should be boiled or laundered.
oral antibiotic (e.g., flucloxacillin), if superadded bacterial
Examine clothing and bedding for nits and lice.
infection, can be given.
Pediculosis pubis: 10 minute application of a pyrethrin
o Permethrin topical cream (treatment of choice), must be preparation is usually effective. Re-treatment mav be required
applied to enrire skin below neck. Applied locally overnight in 7-10 days. Infestation of eyelashes is eradicated by
and allowed to remain on the lesions for about B-12 hour. petrolatum applied 3-5 times per 24 hour for 8-10 days.
May be repeated I or 2 week later. Clothing, towels, and bed linens may be contaminated 1
o i
Benrylbenzoate: Two-three applications on consecutive with nit-bearing hairs and should be thoroughly laundered
I
2-3 d. or dry-cleaned. a

Urticaria and angioedema affect 20o/o of individuals at some
point in their lives. Episodes of hives that continue for <6
weeks are considered acute, and those that persist for >6 weeks
are designated chronic. The distinction is important, because
the causes and mechanisms of urticaria formation and the
therapeutic approaches are different in each instance.
Ciassical urticaria mainly affects the dermis. The hallmarks
are transient erythema (lasting <24 hours), transient edema,
transient itch. Acute urticaria is a self-limited condition in
most cases.
The subcutaneous variant, of which edema is the main
feature, is called angioedema. The older term angioneurotic
edema is synonymous with angioedema.
The features of anaphylactic shock are bronchospasm, laryn-
geal edema with extreme dyspnea and cyanosis, and marked
fall of blood pressure.
Urticaria can also be classified according to the temporal
relationship to a stimulus and the duration of a typical hive.
Lesions that last 1-2 hour are typically encountered with physi-
cally induced hives and an inciting stimulus that is present only
briefly. There is prompt mast cell degranulation, and biopsy of
such lesions reveals little or no cellular infiltrate. A second form
ofurticaria can occur spontaneously and last 6-36 hours. These
lesions rypicaliy have a prominent cellular infiltrate and can be
found with food or drug reactions, chronic idiopathic urticaria,
chronic autoimmune urticaria, and delayed pressure urticaria.
Serum sickness reactions can be seen as a manifestation of drug
reactions, and biopsy reveals a small-vessel cutaneous vascuiitis.
Urticaria in association with systemic lupus erythematosus or
other vasculitides appears similar.
TREATMENT OF URTICARIA
Most cases respond to treatment with an oral antihistamine
(to be given for 7-10 days after control of symptoms),
hydroxyzine and diphenhydramine are sedating, but they
are e{fective and commonly used. Newer non-sedative drug
(e. g., cetirizine/loratidine/fexofenadine) is usually preferred,
and the dose should be increased if the response is poor.
The effect of adding an H, antagonist is unpredictable, but
a minoriry of difficult cases improve.
Short courses of systemic corticosteroid may be required in
severe attacks, but long-term use is seldom indicated.
Etiological factors should be looked for and eliminated
/ (drugs, diet, physical stimuli, worm and protozoai infesta-
r tions, bacterial and viral infections).
; CHRONIC URTICARIA
? unknown cause. It starts soon after birth as cradle cap and/
\ o Urticaria that lasts >6 weeks is termed chronic, usually
l occurs in about one-third of cases.
DERMATOLOGY
. Tty to find out the cause (food, inhalants, NSAIDs, infec-
tions and infestations, physical stimuli) and avoid.
Treolmenl
Long-term antihistamine treatment till the urticaria remits
spontaneously. No role of skin test and desensitization. If
hives persist after maximal H,- and/or Hr-receptor blockade
has been achieved, alternate-day therapy with corticosteroids
is the most efFective treatment. In general, prednisone 20 mg
orally as a single morning dose on alternate days is used, with
the dosage decreased by 2.5-5.0 mg every 1-3 week depending
on the clinical response. Theatment of autoimmune chronic
urticaria refractory to medical therapy includes intravenous
immunoglobulin, plasmapheresis, or both.
URTICARIAT VASCULITIS
Urticarial vasculitis is differentiated from ordinary urticaria by:
o Longer duration (>24 hours) of individual lesions.
r A minimal response to antihistamines.
o A burning or tingling sensation, rather than itching.
e There may be associated arthralgia and abdominal pain.
Treqlmenl
o Antihistamine (e.g., chlorpheniramine maleate).
o The main treatment is systemic corticosteroid, the dose of
which can be reduced to acceptably low levels.
PHYSICAT URTICARIA
Physical urdcarias are induced by physical stimuli, particularly
stroking or scratching, sweating (cholinergic unicaria), cold or lighr
Lesions usually appear within minutes and clear within 1-2 hour.
TREATMENT OFANGIOEDEMA AND
ANAPHYLACTIC SHOCK
Adrenaline 0.2-0.3 ml of the standard 1:1000 solution SC or
iM, may be repeated every 20 or 30 minutes in severe cases.
Hydrocortisone 50-100 mg IV or IM, may be repeated 6
hourly if required.
Antihistamine, for example, chlorpheniramine 10 mg IM.
Subsequent doses may be given orally.
a Fluid: 5% DNS (dextrose normal saline), dextran'
a Oxygen inhalation.
Itis a chronic infammatory erythematous scaly eruption of
or diaper/nappy rash that spreads rapidly and may become
extensive in an otherwise healthy baby.
ESSENCE OF PEDIATRICS
TREATMENT
Often no treatment at all is necessary, apart from a bland
emollient, such as aqueous cream.
For the more severely affected, use 0.5%o or 1%o hydrocor-
tisone alone or in combination with clotrimazole applied
sparingly once or twice daily.
The thick scaling of cradle cap can be removed by arachis
oil, massaged on to the scalp, prior to washing with a mild
baby shampoo (selenium sulphide shampoo).
Topical immunomodulatory agents (tacrolimus, pimecroli-
mus) approved for the treatment of atopic dermatitis in
children 22 year of age may have a role in the treatment of
other eczematous disorders such as seborrheic dermatitis.
It is the prototype of irritant contact dermatitis. Rash in the
diaper region is common during early infancy occurring in
half of all infants. It is more common in artificially fed infants,
especially in those, in whom diaper is made of toweling and is
changed infrequently. It may involve convex surfaces such as
buttocks, scrotal sac, mons pubis or inner side of thigh. Only
flexures may be affected. Lesions may be sharply demarcated
or there may be satellite lesions.
o Convex surfaces: The skin appears red, like a parchment-
like scalded area, which soon becomes infected giving rise
to pustular erosions. Most often these lesions are contact
dermatitis secondary to detergents used in laundering the
diaper and antiseptic medications. Bacterial action on rhe
urine soiled diaper produces ammonia (from urea) and
nonalkaline smelly putrefactive enzymes, which produce
diaper rash.
o Involvement of foids of skin: Retention of sweat makes the
area moist and macerated. Constant rubbing of skin causes
erosion and denudation of the skin. Bacteria grow easily in
this environment and cause secondary infection. The lesions
are generally sharply demarcated.
r Band like erythematous lesions are attributed to contact
dermatitis with elastic band at the diaper edges.
c Prickly heat or miliaria may appear as diaper rash. Generally
there is evidence of miliaria on the rest of the body.
o Bullous impetigo is not uncommon in the diaper area.
r Rarely, psoriasis and acrodermariris enteroparhica may
present as diaper rashes.
TREATMENT
o Diapers used should be made of single layer of porous soft
cloth. Excess layers of clothing should be avoided. Use of
impervious plastic diaper covers for the nappies should be
avoided, except during important social events and that also
only for very brief periods.
Diapers should be washed with mild soap and rinsed
thoroughly. Synthetic detergents should be avoided while
laundering diapers. In case of ammoniacal dermatitis, rhe
diaper should be dipped in vinegar and then dried.
The acute rash should be managed with cool wet compresses
(using 1 teaspoonful of salt in a pint of water) intermittently
for 2 or 3 days. Ointments should not be used. A cream or
lotion may be used to control infection. Contact dermatitis
and seborrheic dermatitis respond to 0.5-1o/o hydrocortisone
cream. \Xlhen candida infection is suspected, nystatin dusting
powder or clotrimazole may be used.
The term eczema and dermatitis are nowadays used synony-
mously. To some dermatologists, however, the term eczema
implies a constitutional eczema, whereas dermatitis is a reac-
tion to an external agent. Eczema is characterizedby confluent
edema in the epidermis with an irritable papulovesicular rash.
ATOPTC DERMATTTTS (ATOptC ECZEMA)
Atopic dermatitis is a chronic skin lesion occurring in early
childhood. Disease is rarely present at bifth but may srarr by
the age of 3 months. The onset may be deiayed in some cases.
The classical features are erythema, exudation, lichenification,
and intense itching. Infants with atopic dermatitis are predis-
posed to developing allergic rhinitis and/or asrhma later in
childhood, the so-cailed "atopic march."
Pothogenesis
There is a strong epidemiological association between atopic
dermatitis, allergic rhinitis, asthma, and immune deficiency
disorders, such as Wiskott-Aldrich syndrome. It is now widely
believed to be a late-phase IgE-mediated reacrion due to a
constitutional anomaly in the immune system. The disorder
may be triggered by an extrinsic factor that could be a food
allergen or rarely an inhaled allergen. A chronic late-phase
allergic inflammatory reaction of the skin sets in with resui-
tant itching. Scratching of skin to relieve itching encourages
entry of potential allergens, such as resident fora of the skin,
viz., staphylococci, streptococci and even pneumococci. These
further exacerbate the inflammation of the skin, seming up a
vicious cycle. Factors released from inflamed skin perpetuate
further changes in the dermal and circulating immune com-
petent cells and set up a second vicious cycle.
Clinicql Feofures
o The earliest presenration before the age of 3 monrhs may be
like that of seborrheic dermatitis. Erythematous squamous I
lesion first appears on the scalp, behind the ears, around \
the nose, buttock or genital region. At this stage, itching
I
I
 DERMATOLOGY

may not be as pronounced. Most cases resolve in 4-6 weeks Treqlmenl

without leaving any residual sequelae, but some parients go
The treatment includes skin hydration, topical anti-inflam-
on to full-fledged infantile eczema by the age of 34 months.
matory therapy, identification and elimination of flare factors,
Infantile eczemat It manifests as rosy erythema over the
and, if necessary, systemic therapy.
cheeks. There is brawny desquamation, small papule for-
mation, and some crusring. The skin folds behind the ear Mild eczema:
become fissured and neck creases appear sodden. These may
Daily bathing with small quantiry of liquid antiseptic soap,
be secondarily infected with candida. Extensor surfaces of
( then use of toilet cream.
arms, legs, and wrist may show dryness and scaling. Gener- a Soap substitutes (emulsi$'ing ointment).
ally perioral, periorbital, and nasal regions are spared. Itching
I
a Coal tar creams.
is marked. Buttocks generally escape because of protective
r clothing. Most children show a resolution by the age of I Moderate eczem .

or 2 years; but the illness may continue with remissions o As for mild eczema.
and exacerbations in few cascs. o Topical corticosteroids (1% hydrocortisone) to face, and
i
Flexural eczema: As the age of the child advances or in moderately porent corricosteroid to trunk and limbs, twice
V
those with onset after the age of 1 year, lesions are more
I daily.
r pronounced over the flexures of the elbows, knees, neck o Antibiotics, if infected (cloxacillin or erythromycin).
and front of ankle. There is redness, scaling and licheni-
fication, Severe eczemai
o Other variants: Atopic dermatitis may present with r As for mild and moderate eczema.
disseminated lesions as scattered round patches of scaling, r Potent topical corticosteroids applied to worst areas for
lichenification and miid itching or as nummular 1 week.
eczema, with coin-shaped vesicular lesions with intense o Systemic corticosteroids (e.g., ACTH gel or prednisolone
itching. for 1 month).
Pityriasis alba with siightly hypopigmented patches on
Note:
face, previously atributed to infection with Staphylococcus 1. Allergens (e.g., diet, drug, dust, etc.) should be avoided.
epidermitidis is now believed to be a mild variant of atopic 2. Tl-re natural tendency for the disease is to improve with age. About
dermatitis. 600/o of patients have comfortable skin by the age of 6 years, and
90o/o by puberty.
Diognostic Crilerio
Hanifin and Rajka defined ma.ior and minor criteria for diag- CONTACT DERMATITIS
nostic accuracy of atopic dermatitis. Three major and three
Acute contact dermatitis is characterized by itchy inflamed
minor criteria should be present.
skin that is red, swollen, and papular with vesicles. Two rypes
Major criteria: may be distinguished:

o a. Irritant dermatitis is caused by substances, such as, acids

Pruritus.
and alkalis, which directly damage the skin. The common
o Distribution on the face and convexities in children under
offenders are alkaline cleansers, degreasing agents, and oils.
the age of 2 years and over flexures in older chiidren.
o b. Allergic dermatitis follows contact with an agent to which
Tendency to chroniciry.
the skin has been sensitized by a previous exposure. The
o Personal or family history of atopy such as asthma, allergic
commonest allergens are rubbel resins, and medicaments.
rhinitis, or atopic dermatitis.

Minor criteria: Treolmenl

o Immediate skin tesr reacrion. r Remove the cause.
r Delayed blanching to cholinergics. o Same as the treatment of eczematous diseases (mentioned
o Anterior subcapsular cataract. above).
o Xerosis. o Education ofpatients and parents about the causes is crucial
o Ichthyosis vulgaris with accentuation over palmar creases. for successful therapy.
r Facial pallor/suborbital shadowing.
o Infraorbital folds.
; o Keratoconus.
7 o Recurrenr skin infections.
t o Tendency to nonspecific dermatosis of hand. Superficial mycoses include dermatophyte infection,
2
t o Raised serum total IgE. versicolor, and candidiasis.
,
 I
ESSENCE OF PEDIATRICS

DERMATOPHYTOSES

The dermatophytoses (ring worm) are caused by a group of

Candidiasis may present as oral thrush, diaper rash, vulvovag-
filamentous fungi with a propensity for invading the stratum
initis, or paronychia.
corneum, hair, and nails. The keratinophilic fungi responsible
Oral thrush is treated with local nystatin drop or clotrimazole
for superficial fungal infections (teniasis) are members of three
oral cream. Therapeutic agents in decreasing order of efficacy
genera: Microsp orum, Tiic h op hyton, and Epiderrnop hyton. Host
include miconazole gel, amphotericin B suspension, genrian
defense has an important influence on the severity of the
violet. and nystarin suspension.
infection. Disease tends to be more severe in individuals with
diabetes mellitus, lymphoid malignancies, immunosuppression,
and states with high plasma cortisol levels, such as Cushing
syndrome. For diagnosis, scrapings should be taken for micros-
copy and fungal growth on Sabouraudt culture medium. Infection of the skin by pyogenic bacteria, Staph. aureus and
Streptococcal pyogenes. May be primary (caused by direct inva-
Treqlmenl sion) or secondary (following damage to skin as in scabies,
pediculosis, wounds, eczema, etc.).
Localized lesions can be cleared by topical therapy (i.e., by
clotrimazole lo/o cream), used rwice daily for about a month.
Griseofulvin 10 mg/kg/d is specific for dermatophyte infec- TREATMENT
tions (e.g., tinea capitis, tinea corporis, tinea cruris, tinea
Cloxacillin, cephaiosporin, or erythromycin oraily for 7-10
pedis) given for 4-6 weeks. Vgorous shampooing with a
days.
2.5o/o selenium sulfide, zinc pyrithione, or ketoconazole
Mupirocin and sodium fusidate are efrective topical therapy,
shampoo is helpful in tinea capitis. Therapy of tinea unguium
when lesion.s are few.
(infection of nail) is frequently disappointing; prolonged
teatment of underlying cause (i.e., scabies, pediculosis, etc.).
therapy with griseofulvin (1 year or more) and the appli-
cation of topical fungistatic agents to the nail bed may
be effective. 'Iotal removal of nail followed by long-term
antifungal agent is also effective.
Ketoconazole 3-5 mglkgld or fluconazole can also be used. \(/arts are verrucolls papules caused by the human papilloma
virus (HPV). There are different clinical types of warts: plantar,
plane, filiform, and genital warts. \(/arts on the anogenital area
TINEA VERSICOTOR
may indicate sexual abuse.
Tinea versicolor is a chronic fungal infection caused by the Spontaneous resolution is the usual outcome, takes several
dimorphic yeast Malassezia furfur. These lesions vary in hue months.
(white, tan, brown).
TREATMENT
Diognosis
Tieatment is by daily application of a wart paint (keratolytic
A potassium hydroxide (KOH) preparation of scrapings is
agents), such as 16.50/o salicylic acid with 15.5o/o lactic acid.
diagnostic.
Cryotherapy using liquid nitrogen. Curettage, cautery, and
laser can also be done.
Treolmenl Genital warts respond to treatment with podophyilum (l5o/o,
o Most cases will respond to 2.5o/o selenium sulphide shampoo 20o/o, or 25o/o in benzoin compound tincture) applied once
over affected skin 15-20 minute daily for 1-2 weeks. weekly for up to 6 weeks.
o Topical imidazole cream (e.g., clotrimazole, miconazole,
econazole) are effective,but require twice daily treatment
lor 2-4 weeks.
Orai ketoconazole 3-5 mglkgld is effective in 7-10 days,
but this drug should be reserved for cases not responding Mollusca contagiosa are smooth translucent papules with a
to topical therapy as there is a risk of hepatotoxiciw. Flu- characteristic central punctum, caused by a pox virus. These
conazole is also effective. tend to occur in crops. Spontaneous resolution usually occurs
25o/o sodium thiosulfate lotion is applied twice dailv for in 6-9 months in most children.
2-4 weeks. 1. If lesion persist >6-9 months, mollusca can be removed
Many patients relapse, and for these a prophylactic treatment by curettage or application of 10% trichloroacetic acid.
with selenium sulphide overnight once a week is useful. 2. Electrocautery and cryotherapy can also be done. t
rr
t
I
I
 DERMATOLOGY

genetically predisposed individuals; frequently associated with

systemic illnesses.

Treqlment
This serious complication occurs as a result of certain phage
types ofstaphylococci that produce an exotoxin causing wide- o Most patients with veiling require no treatment; their cos-
spread toxic epidermic necrolysis. metic disability can be disguised with cosmetics.
Some patients who have a marked cosmetic disability can
be treated with systemic psoralens with ultraviolet llght
TREATMENT
GIVA;. This form of photochemotherapy is known as PUVA
IV flucloxacillin or cloxacillin or fusidic acid for 2-3 days, treatment and is quite effective. 8-methoxypsoralen 0.6
mg/kg should be taken 2 hour before exposure to sunlight
then orally for a further 7 days.
or ultraviolet light. Tieatment should be carried out 2-3
times a week for at least 6 months and in some patients
for several years.
It is important to note that treatment with topical psoralens
Characterized by blisters within the epidermis, and deposition can be hazardous and may lead to untoward blistering of skin.
of immunoglobulins and complements within the intercellular Some patients with localized vitiligo may be treated with
space. more potent topical corticosteroid preparations and a few
with a skin bleaching cream, e.g., 20o/o monobenzyl ether
Pemphigus vulgaris: Patients present with mouth ulceration
of hydroquinone.
but rapidly become ill and develop widespread blisters that
No dietary restriction.
ulcerate. Nikolsky sign is present. The lesions rupture and
enlarge peripherally, producing painful raw, denuded areas that
have little tendency to heal. If untreated, the disease is fatal. TEPROSY
Pemphigus foliaceus (rare): There are transient blisters. Patients Leprosy is a chronic disease resulting from infectionwith Myco-
present with red, scaly and crusted areas that may become bacterium leprae and moderated by the ensuing host response.
generalized. The illness is less acute and more benign than The respiratory mucosa, skin, and peripheral nervous system
pemphigus vulgaris, and carries a better prognosis. are most prominently afrected, with occasional testicular and
l ocular involvement. It presents classically with hypopigmented,
1
DIAGNOSIS anesthetic macules. Howeve! anesthesia may be difficult to
k
I
ascertain in younger children.
Y
o Direct and indirect immunofluorescence studies (IgG and In tuberculoid leprosy, the macule will, in addition, show
I C3 can be demonstrated). atrophy and hair loss. In lepromatous leprosy, there is a
I o Tzanck smear. diffuse thickening of the skin and slit skin smears for acid-
I fast bacilli (AIB) are positive. The nerves may be thickened
I TREATMENT and/or tender.
F Typ. I reaction in leprosy manifests with erythema in the
I o Pemphigus vulgaris: The disease is best treated with high- skin lesions, nerve pains, and swelling of the hands and feet.
! dose systemic corticosteroid therapy. Azathioprine, cyclo- Type II reactions manifest with fever, tender erythematous
t
phosphamide, or methotrexate have been used as mainte- nodules (erythema nodosum leprosum), nerve pains, and
nance therapy. lymphadenopathy.
t
t
Pemphigus foliaceus: Systemic corticosteroid produces
t long-term remission. Treqlmenl
t Dapsone, topical corticosteroid are occasionally sufficient.
o The drugs used in the treatment of leprosy are dapsone
t o Supportive treatment.
*
I
100 mg daily and rifampicin 600 mg once a month for
t 6 months in smear-negative patients, and dapsone 100 mg
daily, rifampicin 600 mg once a month and clofazimine
300 mg once a month and 50 mg daily for 2 years in smear-
positive patient. For children <45 kg in weight, the dose
VITILIGO
should be suitably ad.iusted. It is recommended that 25o/o of
The lesions are circumscribed, milky white in colour (syn. leu- these doses be given if the child is <15 kg, 50o/o of the dose
koderma) and appear to invade the normally pigmented skin. be given if the weight is between 15 and 30 1g and 75o/o of
It develops because of autoimmune damage to melanocytes in the dose be given if the weight is between 30 and 45 kg.
It

l
h

ESSENCE OF PEDIATRICS
Mild reactions should be treated with non-steroidal anti-
inflammatory drugs. Systemic corricosreroids are required
for severe reacrions. Thalidomide is highly effective in type
II reactions but is not available. AntiJeprosy medication
should not be discontinued during reacions.
One dose of BCG gives 50% prorecrion and the second
dose increases the protective benefit.
PITYRIASIS AIBA
Pityriasis alba is a common disorder that affects many normal
children. Hypopigmented, ill-defined, finely scaly macules
develop on the face, neck, upper trunk, and proximal portions
of the arms. Lesions come and go, often for many years. The
condition is often ascribed (mistakenly) to viriligo, rinea versi- 2. Antibiotic therapy: Can be given. Initiated with retracy-
color, tinea corporis, worm infestation, calcium deficiency, liver
disease, etc. Spontaneous improvement by puberry is the rule.
Polymorphous (several different forms) skin eruptions are
common; occur at the start of the summer season. Pruritic
papules' macules' plaques' or erythema develop 1-2 days after
sun exposure'
Actinic prurigo: A combination ofvesicles and papules develop
several hours after sun exposure.
solar urticaria:'riansient, usually lasts for <24hour.
-l
TREATMENT
o Avoid sun exposure
I Physical or chemical sunscreens may give some protection.
o Topical corticosteroid
o Prophylactic photother apy, e.g.,uvA or puvA.
Acne is a polymorphous skin eruprion, characterized by inflam-
mation of the sebaceous glands occurring in areas of the skin
that are rich in these glands (i.e., face, back, and chest).
Tire condition may be mild clearing up with time, but in
a few cases disfigures the face (acne vulgaris). Acne vulgaris
has 4 basic: open and closed comedones, papules, pustules,
and nodulocystic lesions. One or more rypes of lesions may
predominate.
TREATMENT
Treatment is aimed at modifying the pilosebaceous duct
obstruction, the microfloral population, and the seborrhea.
Mild acne requires no therapy. Severe acne requires topical
!
agents plus sysremic antibiodcs. Very severe acne requires
therapy with 13-cis retinoic acid.
1. Topical agenr is used for removing the keratin plugs using
a chemical peeling agenr, such as:
a) Benzoyl peroxide, available as a gel, cream, or lotion
in 2.5o/o, 5o/o, and l0olo concenrration (e.g., Acetoxyl,
Benoxyl, Quinoderm). This is applied once daily after
washing, preferably at night. Retinoic acid is also
effective.
b) Cleansing with soap and water. Repetitive cleansing
is not needed.
c) Tretinoin, a derivative of retinoic acid, can be applied, -
once a day after washing {or 3-6 months.
d) Topicat antibiotics, e.g., erythromycin.
cline 1 g/d, div.^BD for -6 iveeks, followed by a gradual
d.crease to the minimal eflective dose. Better to gi re in
combination with benzoyl peroxide or rrerinoin. Alter-
native to tetracycline include erythromycin, doxycycline
clindamycin.
3. Alternative therapies: Patients who fail to respond, develop
side effects, relapse rapidly require ar, :
".rd7o,
therapy to reduce th. ,.b.r- .*cretion rate.
"lt.rn"ti.,e
a) Isotretinoin 13-cis retinoic acid 0.5-1.0 pg/kg/d
procluces a 90o/o reduction in the sebum excretion
rate. It will improve the most severe forms of acne .
within 4-6 months and produces a remission period
2 yeat in 50o/o of cases' It is however '
:^?:]::::
teratogen lc.
b) Hormone therapy: The hormone preparation contain-
ing cyprotero.r. 2 mg plus ethinylestradiol 50
"..,".. .
ffi:'fl:1T'j.:'3;'.1,;l',;:"",:.,;ff1"."?:.oil _
needs to be given for 6_12 months i, .#..ti.,. .
contraceptrve preParatlon'
rrarion.
"rrd "r-,
Erythema multiforme (EM) has numerous morphologic mani-
festations on the skin, varying from ery'themarous macules,
papules, vesicles, bullae, or urticaria appearing patches of
confluent erythema. It is usually asympromaric, although a
burning sensation or pruritus may be presenr. The diagnosis
of EM is established by finding the classic lesion: targetJike
(iris, or bullt eye) papules with an erythematous outer border,
an inner pale ring, and a dusky purple to necrotic cenrer.
EM is characterized by an abrupt, symmetric curaneous
:
eruption, most commonlv on the extensor upper extremities;
lesions are relativelv sparse on the face, trunk, and legs. The -1
eruption often appears initially as red macules or urticarial t
plaques then expand centrifugally ro form lesions up to 2 cm tt
in diameter with a dusky to necroric cenrer. Oral lesions may
occur, but other mucosai surfaces are spared. Approximately
4l
ri
I
 DERMATOLOGY

25o/oof cases of EM appear to be confined to the oral mucosa,
generally sparing the gingivae. Prodromal symptoms are
generally absent. Lesions typically resolve without sequelae in
about 2 week, and progression to Stevens-Johnson syndrome
does not occur.
The differential diagnosis of EM also includes bullous
pemphigoid, pemphigus, bullous drug eruption, urticaria,
viral infections such as herpes simplex, Kawasaki disease, and
allergic vasculitis. EM that primarily involves the oral mucosa
may be confused with a handful of other conditions, including
bullous pemphigoid, pemphigus vulgaris, recurrent aphthous
stomatitis, and primary herpetic gingivostomatitis.
Among the numerous factors implicated in the etiology of
EM, infection with herpes simplex virus (HSV) is the most
common. HSV labialis and, less commonly, HSV genitalis have
been implicated in 60% of episodes of EM and are believed
to trigger nearly all episodes of recurrent EM, frequently in
association with sun exposure. Most patients experience a single
self-limited episode of EM. Lesions of HSV-induced recurrent
EM typically develop 10-14 days after onset of recurrent HSV
eruptions, have a similar appearance from episode to episode
but may vary in frequency and duration in a given patient.
The pathogenesis of EM is unclear, but it may be a host-
specific cell-mediated immune response to an antigenic stimu-
lus, resulting in damage to keratinocytes. Cytokines released by
activated mononuclear cells and keratinocytes may contribute
to epidermal cell death and constitutional symptoms.
TREATMENT
o Supportive.
o Topical emollients, antihistamines, and nonsteroidal anti-
infammatory agents do not alter the course of the disease
but may provide symptomatic relief.
No controlled prospective studies support the use of corti-
t-
I
costeroids in the management of EM. Rather, glucocorticoid
t" therapy may be permissive of HSV replication and make
EM episodes more frequent or continuous.
t Prophylactic oral acyciovir given for 6 months may be effec-
tive in controlling recurrent episodes of HSV-associated EM.
I On discontinuation of acyclovir, both HSV and EM may
recur, although episodes may be less frequent and milder.
t
trointestinal tract, or anogenital mucosa. A burning sensation,
edema, and erythema of the lips and buccal mucosa are often
the presenting signs, followed by development of bullae, ulcer-
ation, and hemorrhagic crusting. Lesions may be preceded by a
fu-like upper respiratory illness. Pain from mucosal ulceration
is often severe, but skin tenderness is minimal to absent, in
contrast to toxic epidermal necrolysis.
Corneal ulceration, anterior uveitis, panophthalmitis, bron-
chitis, pneumonitis, myocarditis, hepatitis, enterocolitis, pol-
yarthritis, hematuria, and acute tubular necrosis leading to
renal failure may occur.
Disseminated cutaneous bullae and erosions may result in
significant blood loss, increased insensible fluid loss, and a
high risk of bacterial superinfection and sepsis. New lesions
occur in crops, and complete healing may take 4-6 wk; ocular
scarring and visual impairment and strictures of the esophagus,
bronchi, vagina, urethra, or anus may remain. Toxic epidermal
necrolysis is the most severe disorder in the clinical spectrum
of the disease, involving considerable constitutional toxicity
and extensive necrolysis of the mucous membranes and >30o/o
of the body surface area.
Mycoplasma pneumoniae is the most convincingly demon-
strated infectious cause of Stevens*Johnson syndrome. Drugs,
particularly sulfonamides, nonsteroidal anti-inflammatory
agents (butazones, ibuprofen, piroxicam, and salicylates), and
anticonvulsants (phenytoin) are the agents most commonly
precipitating Stevens-Johnson syndrome and toxic epider-
mal necrolysis. Table 19.2 lists potential causes of erythema
multiformc, Stevens-Johnson syndrome, and toxic epidermal
necrolysis.
Table 19.2: Potential Causes o{ Erythema Multiforme,
Stevens Johnson Syndrome, and Toxic Epidermal Necrolysis
Phenytoin
Herpes simplex 1, 2*
M ycoplas ma p ne unto niae Phenobarbital
M ty cob acter i u m tu be r c u Io s is Carbamazepine
Croup A streptococci Vatproic acid
Hepatitis B
Radiation therapy, sr-rnl ght
i

Remove the offender.

I Leukemia Captopril
rF Lymphoma Aspirin, allopurinol
I

NSAIDs

Penicillin
Sulfonamides
l. Cutaneous lesions in Stevens-Johnson syndrome generally
I consist initially of erythematous macules that rapidly and lsoniazid

variably develop central necrosis to form vesicles, bullae, and Tetracycl ines
t areas of denudation on the face, trunk, and extremities. The Cephalosporins
skin lesions typically are mor€ widespread than in EM and are Quinolones
accompanied by involvement of rwo or more mucosal surfaces, *Recurrent erythema multiforme
namely the eyes, orai cavity, upper airway or esophagus, gas- Drug reactions occur 1 -l weeks a{ter exposure
ESSENCE OF PEDIATRICS

TREATMENT Although corticosteroids are somerimes advocated in early

Management of Stevens-Johnson syndrome is supportive
and sympromatic.
Ophthalmologic consultation is mandatory because ocular
sequelae such as corneal scarring can lead to vision ioss.
Oral lesions should be managed with mouth washes and
glycerin swabs. Vaginal lesions should be observed closely
and treated to prevent vaginal stricture or fusion.
Topical anesthetics (diphenhydramine, dyclonine, and
viscous lidocaine) may provide relief from pain, particularly
when applied before eating. Denuded skin lesions can be
cleansed with saline.
Treatment may require admission to an intensive care
unit; intravenous fluids; nutritional supporr; daily saline
compresses; paraffin gauze dressing of denuded areas; saline
compresses on the eyelids, lips, or nose; analgesics; and
urinary catheterization (when needed).
Systemic antibiotics are indicated for urinary or curaneous
infections and for suspected bacteremia because infection is
the ieading cause of death. Prophylactic sysremic anribior-
ics, however, are not necessary. Erythromycin is indicated
in mycoplasma infection.
severe cases of Stevens-Johnson syndrome, no prospec-
tive double-blind study evaluating their efficacy has been
reported. Most authors discourage their use because of
reporrs of increased morbidity and mortaliry (sepsis) with
their administration.
Remove the offender.
Highet AS. Pruritus and urticaria. Medicine International: Bangla-
desh edition, 19BB:3(1).
Clayden GS, Hawkins RL (ed). Paediatrics: 7leatment & Prognosis
1" ed., New Delhi: Jaypee Brothers, 1989.
Jones H. Acne vulgaris. Medicine International: Bangladesh edition,
198B:3(1).
4. Jordon \XrE, et al. Diaper dermatitis. Pedia* Dermatol7986;3:198-
207.
Dworkin PH (ed.). Pediatrics: NMS for independent study. 4,r,ed.
Philadelphia: \X/illiams & \Wilkins, 2000.
Parthasarathy A (ed) . IAP Textbook of Pediatics 4'r' ed. New Delhi:
Jaypee Brothers, 2009.
Behrman RE, Kliegman RM, Jenson HB. Nelson Tbxtbooh of
Pediatrics 1B'h ed. USA: \X/B Saunders Co.,2007.

:
,l
i
e
tl
I
 CHAPTER 20
Poisoning

Chopier Conlenls

Hydrocarbon poisoning...,...

assisted ventilation through mouth-to-mouth breath-

ing, ambu bagging, or do intubation, give mechanical
ventilation if PaO, < 8.0 Kpa or PaCO, > 6.6 Kpa or
rising. C (circulation)-cardiac massage. Prompt treat-
It has been observed that 80o/o of the cases having acute ment is essential because irreversible brain damage will
poisoning require nothing more than observation while they occur unless some circuiation of oxygenated blood can
metabolize and eliminate the drugs they have taken, 5%o need be achieved within 2 or 3 minutes.
intensive supportive care, and 15%o require antidotes. 2. Prevention of further absorption and increasing elimi-
Two peak ages for poisoning: l-5 year (accidental) and nation of poisons:
adolescence (suicidal).
a) if the poison has been inhaled or absorbed through
the skin, the patient should be removed from the toxic
Features ofpoisoning:
atmosphere, soiled clothing should be removed, and
contaminated skin should be cleansed thoroughly.
Sympathomimelic b) Removal of gastric contents:
trcitability, tachycardia, Amphetami nes, ephedri ne i. Gastric lavage: Poor recovery is likely if lavage is
hyperlension, dilaled pupils,
>- performed >2 hour after the overdose. The largest
sweating
bore tube that can be passed should be used because
t Sympatholytic
L
it will allow the removal of larger pill fragments.
Depressed consciousness, Opiates, barbiturates,
t" Lavage with normal saline should be contained
bradycardia, hypotension, narrow phenothiazine, alcohol
pupils until the return fuid is clear for 2 or 3 passes.
t Cholinergic
ii. Emesis: Stimulation of the pharynx with the
I
fingers is safe and is a reasonable first aid measure.
t Agitation, anxiety, narrow pupi ls, Organophosphates,
Syrup of ipecacuanha is a safe and effective
t bradycardia, increased sweating n r cotr ne
emetic, contains the active aikaloids, emetine
Il Anticholinegric
causes vomiting within 30 minutes of intake to
Tachycardia. dilated pupils, hot Atropine, dhatura,
f.
dry s[in, decreased peristalsis, antihistaminics,
be given 10-15 ml each time. It induces vomiting
t urinary relenlion antidepressant drugs causing gastric irritation plus stimulatingCTZ of
the floor of 4th ventricle of the brain.
l-
iii. Adsorbents (activated charcoal): Charcoal is
TREATMENT
able to adsorb a wide variery of drugs and toxic
l. Resuscitation: ABC of cardiopulmonary resuscitation agents and prevents further absorption (but iron
includes the following: A (airway)-pull the tongue salts, acids, and bases are not). Dose: 15-30 g
forward, clear the airway of vomitus or debris, extend for children 42 year and 50-100 g for children
the neck and raise the chin, turn the child semiprone; >1.2 year. The most convenient method is via a
insert an airway tube. B (breathing)-give oxygen and nasogastric tube, particularly if repeat doses of

ESSENCE OF PEDIATRICS
adsorbents are to be given. Alternatively patienrs
may be asked to drink the prepararion from a
'Coke' can, so rhar they cannot see it. Charcoal
will prevent significant absorption of drug if
given within I hour of drug intake.
iv. Purgation: Purgatives, e.g., magnesium sulfate
(250 mglkg) have been used alone and in
conjunction with activated charcoai; their efficacy
has not been established.
c) Increasing elimination by
i. Alkaline diuresis: Increases the elimination
of salicylates and phenobarbitone. Sodium
bicarbonate administered as an B.4o/o solution,
which contains I mmol bicarbonate in 1 ml fluid,
is infused to ensure that pH of the urine is >7.5
and preferably closer to 8.5. Forced diuresis is no
longer used.
ii. Acid diuresis: Used for eiimination of quinine.
iii. Dialysis (peritoneal and hemodialysis): Increases
the elimination of methanol, ethanol, salicylate,
lithium.
iv. Hemoperfusion: Involves the passage of blood
through an absorbent material and is used for
barbiturates.
3. Antidotes:
a) Anticoagulants (coumarins) : Vitamin K (phltomenadi-
one) i-10 mg IV slowly if continued anticoagulation
is not intended; if it is, give fresh frozen plasma.
b) Arsenic/Mercury/Gold/C opper I Zinc
c) Dimercaprol (BAL) 2.5-5 mg/kg IM (deep) 4hourly
for 2 days;2.5 mglkg IM BD for l-2 weeks.
d) Beta-blockers: Atropine 0.6-2 mgIV for bradycardia,
glucagon 50-150 pg/kg IV followed by l-2 mglhr
until recovery.
e) Benzodiazepines: Flumazenil IV 200 pg over 15
then 100 pg at 50 second intervals SOS,
seconds
maximum 300-600 pg.
f) Digoxin: Digoxin specific Fab antibody fragments.
s) Iron: Desferrioxamine 15 mg/kg/hr (total dose 80 mg/
kg in 24 hr IV or 1 g IM 12 hourly until recovery).
Gastric lavage should be performed with a solution
of desferrioxamine (2 g in I liter of warm water).
h) Lead: Sodium calcium edetare 50J5 mglkgby slow IV
inflrsion daily for 5 days (2 g in 250 ml normal saline).
Opioids: Naloxone 0.01 mg/kg IV or IM, repear as
necessary every 2-3 minures (400 pg/ml).
Organophosphorus insecicides: Atropine 0.5-2 mg
IV, repeat as necessary to maintain full atropinization
(dry mouth, pulse >70lmin).
k) Pralidoxime mesylate (PrS) :O mg/kg IV in 10-15 ml
water over 5-10 minutes and repeat even'30 minutes
for l-2 doses or obidoxime 3a mglfu IV 4 hourly
for 24 how.
I
4. Hypotension: Foot end of the bed is elevated by about l5
cm to increase venous r€turn to the heart. Use intravascu-
lar volume expander (normal saline, dextran, or plasma).
Dopamine 2-5 ptglkglmin infusion. Check BP
5. Convulsions: Anticonvulsant.
6. Cardiac conduction defects and dysarrhythmias: Antiar-
rhythmic drugs.
7. Supportive measures:
a) Hypothermia: Patient should be covered with clothes
or blankets, nursed in a warm room.
b) Acid-base abnormalities and dyselectrolytemia should
be corrected.
c) Specific organ damage (pneumonia, acute renal or hepatic
failure, skin bullae) should be treated, when present.
d) Bladder and bowel care.
e) Adequate calorie and fluid intake.
Aspirin is widely consumed and is an important cause of drug
poisoning in children. Aspirin is a weak acid that is absorbed
rapidly from the stomach and small bowel into the circula-
tion and is both freely ionized and protein bound. The drug
is metabolized by the liver and excreted through the kidneys.
PATHOGENESIS
There is increased sensitiviry of the respiratory centers of the
brain to changes in carbon dioxide and oxygen concenrrarion,
leading to increased rate and deprh ofrespiration, and resultant
respiratory alkalosis. To compensate, hydrogen ions move from
cells to the extracellular space.
Oxidative phosphorylation is uncoupled, increasing the
metabolic rate and causing increased metabolism of glucose
and oxygen, resulting in excess heat production. This causes
tachycardia, tachypnea, fever, and hypoglycemia. Aspirin also
inhibits the Krebs cycle, causing metabolic acidosis.
Aspirin damages hepatocytes, causing liver toxicity and pro-
longed prothrombin time. It also inhibits platelet organization,
causing prolonged bleeding time.
CtINICAt FEATURES
Clinical features include tinnitus and vomiting (the vomitus
may be heme-positive); hyperpnea, fever, lethargy, and confu-
sion; conr,rrlsions, coma, and respiratory or cardiac failure.
;
TREATMENT
I
r
I
Ipecac-induced emesis, followed by administration of acti- ']
vated charcoal and cathartics. 1
o Alkalization with sodium bicarbonate given intravenously
.|
(a urinary pH of 8 is desired). t}t

Adequate fluids to correct loss; colloid (as dextran, albumin,
or plasma) given to correct shock.
Dialysis or hemofiitration, which should be considered
in severe cases or when there is renal, hepatic, or cardiac
failure.
Paracetamol has replaced aspirin as an antipyretic analgesic for
use in children. In general, children younger than 5 years ofage
seem relatively resistant to severe toxic sequelae compared to
adults, but still should be evaluated and managed aggressively.
CLINICAT FEATURES
There are three main phases of acetaminophen poisoning:
Phase I: Usually begins 30-50 minutes after ingestion and
may last for 12*24 hours.
o Most patients with mild poisoning never progress beyond
this stage and are asymptomatic.
o In moderate to severe poisoning, gastrointestinal signs
(anorexia, nausea, and vomiting) as well as pallor and dia-
phoresis predominate.
o Changes in level ofconsciousness do not occur at this stage
and if present suggest the ingestion of a different agent,
perhaps in addition to acetaminophen.
Phase II: Occurs 24-48 hours after ingestion and may persist
up to 4 days.
o During this phase, the patient is usually clinically asymp-
tomatic, although mild right upper quadrant tenderness
relative to hepatic enlargement may occur.
o Hepatic enzymes, serum bilirubin, and prothrombin time
rise as hepatic necrosis progresses.
o Patients who are moderately poisoned do not progress
beyond this point and gradually recover.
Phase III: Occurs 3-5 days after ingestion, and symptoms are
related to hepatotoxicity.
o Symptoms may be limited to anorexia, nausea) malaise,
and abdominal pain.
o More severe cases may progress to confusion and stupor as
well as sequelae related to hepatic toxicity, including jaun-
dice, coagulation defects, hypoglycemia, and encephalopathy.
Renal failure and myocardiopathy may also occur.
o Death occurs from irreversible hepatotoxiciry.
ASSESSMENT
r Maximum number of tablets or liquid missing from the
container should be presumed ingested.
o If the presumed ingested dose is < I 00 mg/kg of body weight,
the ingestion is mild and need not be treated.
POISONING
o If the presumed dose is unknown or >100 mg/kg, the child
requires clinical evaluation and intervention.
TREATMENT
Syrup of ipecac should be given to empty the stomach; if
there is a change in the level of consciousness because of
ingestion of another substance, gastric lavage shouid be
performed.
Activated charcoal usually has not been advised because
it binds and inactivates N-acetylcysteine, the antidote for
acetaminophen. Recent data suggest that a higher dose of
N-acetylcysteine might overcome residual activated char-
coal.
A loading dose of N-acetylcysteine (140 mg/kg bodyweight)
should be given followed by a maintenance dose (70 mglkg)
every 4 hours (may be given up to 17 doses). A serum
sample for acetaminophen assay should be obtained 4 hours
or more after ingestion.
If the serum acetaminophen level is in the toxic range, the
patient should be hospitalized, liver function tests (bili-
rubin, ALII PT) performed, and electrolyte, glucose, and
creatinine concentration obtained. Laboratory tests should
be repeated daily while treatment is underway until 4 days
after ingestion.
In hepato-renal failure, give 10% dextrose to prevent hypo-
glycemia and FFP to maintain PT <60 seconds. Give IV
mannitol 1 g/kg in hepatic encephalopathy.
Supportive care should be provided, depending on clinical
observation and laboratory data.
Iron-containing products, such as ferrous saits alone or iron
as part of multivitamin tablets, are a significant toxicologic
hazard. Abdominal radiographs may be helpful because iron
pills are radio opaque.
CTINICAL FEATURES
There are four main phases of iron poisoning:
1. Gastrointestinal symptoms: \Within 30-60 minutes, vomit-
ing, colicky abdominai pain, gastrointestinal hemorrhage,
and diarrhea occur. Iron acutely and directly damages
the gastrointestinal tract, especially the gastric and small
intestinal mucosa.
2. There is a period of relative stability from 3 to 4 hours
until 48 hours. It is marked by subtle changes and failure
to recognize them. There is no evidence of change in the
central nervous system (CNS).
3. Circuiatory shock occurs after 48 hours. It is caused by
a combination of gastrointestinal fluid and blood loss,
increased capillary permeabiliry and loss of vascular tone,
 ESSENCE OF PEDIATRICS
all of which are direct effects of
excess iron. A secondary
coagulopathy, thrombocltopenia, and hypoprothrombine-
mia may result. Shock results from absolute hypovolemia
and the inability of the body to respond to it.
4. Late manifestarions: Gastric scarring occurs within 2-6
weeks. Because iron acrs directly on rhe gastrointestinal
tract as a corrosive, the healing process may result in areas
of scarring and stenosis of both pre-pyloric region and
duodenum.
Rarely, hepatic necrosis or hepatic failure may result from direct
liver damage by iron.
TREATMENT
The toxic dose ingested (mg/kg) should be calculated, based
on the childt weight. In general, <20 mg/kg of elemental
iron poses little risk; 20-60 mg/kg requires ipecac syrup for
ipecac-induced emesis within 1-2 hour and reevaluation if
gastrointestinal pain or bleeding occurs.
Gastric lavages if >10 mg/kg of elemental iron is ingested,
100 ml of milk of magnesia or 100 ml of 5o/o NaHCO.
solution can be ieft in the stomach ro protect the denuded
gastric mucosa and ionize the residual iron to prevent its
absorption.
Serum iron level should be measured 4-6 hours after inges-
tion. By that time, emeric and cathartics will have been
given (activated charcoal has no effect).
o If the concenrrarion is <300 prg/dl, it is not significant.
,r If the iron concentrarion is 300-500 pLg/dl, if it is greater
than the total iron binding capacity or if the patient is
symptomatic, the serum iron levels are considered to be
significant.
c If the concenrration is >500 pg/dl, it is toxic.
Desferrioxamine, a chelating agenr specific for iron, is rec-
ommended for intravenous use in parienrs with significant
or toxic levels noted earlier. It is given by slow continuous
infusion at 15 mg/kg/hr. Its positive effect is noted when
the urine turns pink, because the iron-chelate complex is
excreted in the urine. Continuous infusion is continued for
a period of 24 hour after the urine becomes clear, or dll
serum iron levels are <300 pgldl.
Accidental ingestion of kerosine, petrol, diesel, and lamp oil is
quite common among children. These substances are commonly
stored in unmarked containers. Their taste prevenrs a large
amount to be consumed and rhe ingestion is rarelr. >30 ml.
TOXIC EFFECTS
o Aspiration of the substance into the respiratory rracr is rhe
major danger. The more highly volatile (because of low
re
I I
viscosiry and low surface tension) the hydrocarbon, the
more likeiy is aspiration. The major effect of aspirarion is
chemical irriiation, which damages the alveolar lining and
the capillaries, causing pneumonitis, atelecrasis, or pulmo-
nary edema. The degree of lung involvement is parallel
to the degree of clinical compromise. Results are severe
hypoxemia and ventilation-perfusion abnormalities, causing
respiratory acidosis. Because of this risk, emesis should not
be induced in management of volatile hydrocarbon inges-
tion. Secondary bacterial infection may occur in a very
small percentage of cases.
CNS depression: AII hydrocarbons are absorbed to some
degree. The amounr absorbed determines the degree of
CNS depression.
Pathological changes occur in other organs, which are mosdy
due to hypoxia.
CTINICAL FEATURES
Symptoms usually have their onset within 6 hour of aspira-
tion, though occasionally can be delayed up to 24 hours.
Early on, there is burning in the mouth and throat choking
and gagging causing nausea, vomiting, and hemoptysis.
There may cyanosis, tachycardia, tachypnea, chest indrawing,
rhonchi and creps. A chest radiograph shows changes before
the onset of significant clinical findings. The radiograph may
reveal punctate motded densities of pneumonitis, atelectasis
or both, with findings tending ro be more prominent in
the dependenr porrion of the lung. Radiographic findings
peak at 72 hours and then begin to clear.
One hour after ingestion, symptoms of CNS depression
occur (e.g., general weakness, hypotonia, mental confusion,
irritabiliry agitation, or convulsions).
There is a poor correlation among clinical symptoms, physi-
cal findings, and radiographic abnormalities. Fever and
Ieukocytosis (may be misleading) usually result from the
pyrogenic effect of the agent.
TREATMENT
o Treatment is essentially supporrive. Emesis and lavage are
contraindicated for fear of aspiration.
o If the amounr of ingestion is small (1-2 mouthfulls) and
the child is asymptomatic for a period of 6 hours post
ingestion, the child can be observed ar home.
Children with symptoms need to be managed with oxygen, :
positive pressure venrilation (PP$ and if required IPPV
Bronchodilators or anriconvulsants mav be given, if needed.
Prophvlactic antibiotics are useful onlv in the presence of :
preexisting respiraton. illness, malnourished children or rhose t
u'ith severe pneumonia. Corticosteroid is contraindicated. 1
a Fiuid, nutrition should be maintained.
a Majoriw of the children usuallv recover fully within 3-8 {
a
davs. Death may occur due to respiratory failure.
i

Lead poisoning (plumbism) is a chronic disorder, which may
be punctuated by acute episode. Most are asymptomatic, but
many children may have decreased school performance associ-
ated with chronic lead exposure.
Sources of increased lead absorption among children include:
1. Ingestion of paint and plaster chips in old dilapidated
buildings
(a) The eating of nonfood items (pica) is common among
infants and toddlers
(b) The combination of pica and living in old housing
accounts for most cases of symptomatic poisoning.
2. Living in close proximity to a lead smelter
3. From practice of employing kajal or surma containing
black oxide of lead in eyes
4. Lead-contaminated soil, exposure to dust
5. Other less common sources (e.g., contamination of acidic
beverages and foods in lead-lined containers, lead-painted
furniture and toys, the burning of battery casings in
fireplaces).
CtINICAI FEATURES
The clinical diagnosis tends not to be suspected until the onset
of CNS symptoms. Onset is insidious. Here lies the importance
ofscreening for increased lead absorption during child health
maintenance visits.
General symptoms include anemia, apathy, decreased play,
abdominal pain, clumsiness and intermittent vomiting. A
lead line in the gums is characteristic.
Acute lead encephalopathy is manifested by vomiting, stupot
and ataxia followed by coma and seizures.
Chronic lead poisoning may present as nonspecific devel-
opmental delay, behavioral and attention disorders, seizures
or a peripheral neuropathy. Follow-up studies suggest that
developmental abnormalities persist over a 10-year period
and may manifest as poor schooi performance.
LABORATORY FINDINGS
Biochemical evidence of lead toxicity includes elevations of
blood lead and signs of interference with hemoglobin synthesis
such as an increase in free erythroc).te protoporphyrin as well
as increased lead excretion in the urine after administration
of a chelating agent, such as calcium ethylenediaminetetra
acetic acid (CaEDTA).
Urine lead level >80 ytgldll24 hr is diagnostic. Blood
lead level is usually >80 pgldl.
a There may be evidence of a sideroblastic anemia.
a Radiographic findings may include lead lines at the meta-
physes of the long bones and radio opaque foreign material
within the small bowel.
!
POISONING
TREATMENT
For children with symptomatic lead poisoning, the source of
lead must be identified and the child's intake immediately
interrupted.
For such children, chelation therapy with agents such as
CaEDTA, dimercaprol, or D-penicillamine is indicated.
o Specific treatment as recommended is a combination of
dimercaprol (BAL 4 mg/kg/dose every 4 hour IM and
CaEDTA I2.5 Wglkgldose every 4 hour IM or IV for 2 days
then add oral D-penicillamine (Byanodine) 25 mglkgldfor
,
5 days. After an initial course of parenteral CaEDTA
or CaEDTA-dimercaprol, oral D-penicillamine can be
given for 2-6 months to prevent rebound increases in
blood lead.
o Supportive treatment may include control of increased
intracranial pressure (by mannitol) and seizure control.
o Careful, long-term follow-up is essential because learn-
ing and behavioral problems are late sequelae.
The approach to asymptomatic children with evidence of
increased lead absorption is still controversial.
a) The Center for Disease Control and Prevention, Atlanta
recommends aggressive screening for all children every
6 months in the first 5 years of life with more frequent
screening of children with mildly elevated blood lead lev-
els (>15 pgldl) that do not require chelation.
r) Prompt identification of environmental hazards is critical.
a) Parents should be counseled routinely to encourage fre-
quent handwashing in children and to damp-mop house
dust.
o CaEDTA may be administered to children with persis-
tent elevations ofblood lead.
Prevention: Is the key in eliminating this imporrant source
of morbidity.
o Replacing or renovating old, substandard housing is the
ultimate solution.
a Avoidance of paints with lead additives is critical.
a Screening of children decreases the incidence of frank lead
poisoning.
Decreased lead in gasoline will help reduce environmental
exposure.
Adequate prevention and treatment of pica.
The phenothiazines (chlorpromazine, fluphenazine,
prochlorpera zine, mazine, promethazine, thiorid azine) ar e
pro
neuroleptic drugs that bring about tranquiliry i.e., peace of
mind and relieve of tension, anxiety, and apprehension. They
primarily act by blocking type-2 dopamine receptors in the
CNS. They also have variable inhibitory activiry at L-adrenergic,
histaminergic, muscarinic, serotonergic, and other dopamine
receptor subrypes.
ESSENCE OF PEDIATRICS

CLINICAL FEATURES CTINICAL FEATURES

Toxic effects begin within 30-60 minutes of ingestion and e The clinical featrr res are characterized by neuroiogic depression.

include: The respiration may be shallow and occasionally progress to

o apnea. Hypotension occurs with severe poisoning.
CNS depression, respiratory depression, hypotcnsion, slur-
ring of speech, hypothermia. Pupils are constricted and the
o Pupils may be dilated or constricred. There is loss of light
and corneal reflex.
skin becomes warm and dry.
o Cardiac effects include tachycardia, atrioventricular block,
o The bullous lesions that are noticed in a few patients are
more due to pressure rather than specific skin pathology.
atrial and ventricular arrhythmia.
o Acute dystonic reactions are characterized by sustained
o Cause of death in barbiturate poisoning is respiratory
failure.
muscle contractions resulting in abnormai posturing of eyes,
face, tongue, jaw, neck, back, abdomen, and pelvis. Dry
mouth, diplopia, incoordination, akinesia, rigidity. DIAGNOSIS
o Cholestatic jaundice associated with cholangitis and agranu-
locytosis is sometimes seen. Patients may be anxious but Serum levels of >10 pg/dl for long-acting,7 Vgldl for inter-
remain alert and oriented during these symptoms. Rarely, mediate-acting, and >3 pgldl for short-acting are considered
neuroleptic malignant syndrome (rigidiry hyperthermia, to be in the toxic range.
altered mental status resembling catatonia, labile blood EEG changes depend on the severity of poisoning. In mild
pressure, autonomic dysfunction) may develop in acute poisoning, there may be fast activity, which progresses into
overdose. siowing of activity or a flat EEG in severe poisoning.

TREATMENT
INVESTIGATIONS
Induction of with syrup of ipecac is useful. Gastric
emesis
Urinary levels of these drugs are diagnostic. ECG findings
lavage using salineor tap water is effective if performed
may show prolonged P-R, QRS ad QT intervals, UandT
within 4-6 hottr of ingestion. Activated charcoal absorbs
wave abnormalities.
barbiturates effi ciently.
Symptomatic treatment for respiratory depression, hypoten-
TREATMENT sion, and hypothermia is provided.
r Forced alkaline diuresis is effective in enhancing excre-
o Stomach wash is given. Activated charcoal is the preferred
tion.
method of gastrointestinal decontamination.
Extrapyramidal symptoms respond rapidly with intravenous
diphenhydramine (1 mgikg over 2 minutes) or benztropine
(0.02 mg/kg, max. 1 mg IV or PO). Doses may be repeated
in 20 minutes if the response is incomplete. Tieatment Caustic injuries to the esophagus and stomach can result due
should be continued with an oral formulation for 2-3 days,
to ingestion ofvarious cleansing solutions like detergents, and
since these reactions can recur in the absence of additional
toilet bowl cleaners, acids or alkalies.
exposure.
Supportive care includes airway protection and mechanical
ventilation for CNS and respiratory depression. Fluid resus- CtINICAI FEATURES
citation followed by pressors for hypotension and anticon\all-
sants for seizures. Diuresis and dialysis are ineffective. Seizures
. dysphagia, drooling, and abdominal pain are
should be treated with benzodiazepines, and hypotension I;Jjln
should be managed with volume expanders and pressor agents.
o Involvement of the glottis leads to stridor and shock.
Physostigmine is used for anticholinergic toxiciry.
o The oral cavity may show edema, ulceration, and pseudomem-
brane over the palate, uvula, and pharynx.
o The child mav have fever and leukocytosis. Some children :
may not have any immediate complications, but on follow-
up are found to have a stricture. t
Barbiturate poisoning is likely to occur among children suf- 1

fering from epilepsy or in homes in which a family member ,}

TREATMENT
is suffering from the same. Ingestion of 1 g of any barbiturate 1
should be viewed with concern, and 3 g should be considered r General measures: Pain from ulceration may be severe, and t
{;
potentially lethal. opiates are often necessary.
t
I

Vomiting should not be induced. Child may be given a
small amount of water or milk. The chlld with mild calrstic
ingestion and no symptoms can be observed at home'
Tlrose with ingestion of concentrated solutions of acids or
alkalies need hospitalization even if asymptomatic' All such
children are kept nil orally for 24 hours' The inabiliry to
predict esophageal injury by oral cavity examination makes
it ,.r".rd"tory for evaluation by flexible endoscopy within
the first 24-48 hours. Patients with first degree injuries to
the esophagus need no specific therapy. The child is advised
oral fluidr, a.td subsequently oral diet. Children with second
or third degree injuries require intensive therapy'
Steroids are administered to reduce edema, inflammation,
and subsequent stricture formation.
a Antibiotics are given to prevent bacterial infection'
a Antacids and Hr-antagonists are given to suppress acid
secretion, which should be continued for 6-8 weeks' Child
should be allowed to drink fluids as soon as possible'
o Endoscopic evaluation is done again at 2-3 weeks for any
stricture, which if found are managed by reverse dilatation
through a gastrostomy. If strictures are severe, they may
not respond to dilatation and these children would require
reconstnrctive surgery with a jejunum or colonic
".ophageal
,.g-.r-r,. A recent method is to place a stent in the area
of ,trictur. to Prevent further narrowing and allowing the
wound to heal over the stent so that a functional esophagus
is avaiiable.
PROGNOSIS
Mild of corrosive injury will have full recoYery' Moder-
cases
ate and severe cases will result in stricture formation requiring
regular dilatation and possibly surgery.
r Organophosphorus compounds and carbamates are used as
insecticides in the household and agriculture' Some of the
commonly used compounds are:
,r Organophosphates: Tetraethylpyrophosphate (TEPP)'
ethylparathion, fenthion (Baltex), malathion, temephos
(Abate).
r Carbamates: Methocarb (Nudrin).
o Minimum lethal dose is 0.02-1 g.
PATHOPHYSIOLOGY
Organophosphorus compounds and carbamates inactivate cho-
lin.rte.as., so that the acetylcholine released at nerve endings
does not get inactivated. Organophosphorus compounds inhibit
this enzyme irreversibly so that the normal activiry can be
resumed only on generation of new enzyme or if the efizyme
I
POISONING
is reactivated by drugs like pralidoxime. Carbamates inhibit
cholinesterases temporarily.
These substances mediate their effects by accumulation of
acerylcholine at various recePtor sites. These substances are well-
absorbed through skin, gastrointestinal, and respiratory routes'
CLINICAT FEATURES
Appears within hours of exPosure:
e Presence of smell of organophosphorus compounds'
o Muscarinic eflects (SLUGE): This is characterized by excess
salivation, lacrimation, urination, and gastroenteritis (nausea,
vomiting, pain abdomen, diarrhea). The pupils are pinpoint
and there is marked bradycardia. Bronchospasm, wheezing,
and pulmonary edema may occur.
r Nicotinic effects: These are seen in severe poisoning, chiefly
muscle twitching, fasciculations and cramps followed by
muscle weakness and paralysis particularly of the respira-
tory muscles.
o Central efFects: These are seen only in severe poisoning and
include restlessness, confusion, headache, slurred speech,
ataxia, seizures, and coma.
The signs and symptoms due to carbamate poisoning are similar
except milder and of shorter duration.
DIAGNOSIS
The diagnosis is based on the history and clinical features' The
RBC cholinesterase level is <20o/o in symptomatic patients'
TREATMENT
o Further exposure to the insecticides must be avoided' The
clothes shouid be removed and skin washed with soap and
water.
If ingested, gastric decontamination is done by emesis and
lavage.
Symptomatic treatment of complications is provided' Start
IV infusion of dextrose-saline for 12-24 houl
Atropine antagonizes the central and muscarinic actions
of insecticides but not the nicotinic actions' Hence'
atropinization will not be abie to prevent the muscle
weakness and respiratory failure. Children >12 years
may be given the adult dose of 1'0-2'0 mg of atropine
(0.6 .ngl"-p) intravenously every 10-30 minutes until
cholineigic ,ign, reversed. Younger children can be
"..
given intravenous atropine in the dose of 0'05 mg/kg'
follo*.d by maintenance of 0.02-0.05 mg/kg every 10-30
minutes to a maximu m of 2-5 mg till the patient is fully
atropinized (pupil widely dilated' dry mouth, tachycardia/
pulse >70lminutes). Then reduce the dose slowly' but
L..p th. patient atropinized for about 2-3 days till the
enzyme regenerates or is reactivated.

ESSENCE OF PEDIATRICS
Pralidoxime, a cholinesterase enzyme acrivaror, is the specific
antidote for organophosphorus compounds. Enzyme activa-
tion occurs most rapidly at rhe neuromuscular junction
with rapid r€srorarion of skeletal muscle response. The
muscarinic or the central acdons are nor significandy reversed
by this drug. Hence for this reversal, atropine has to be used in
conjuncdon. Pralidoxime is used in the dose of 25-50 mg/kg;
for a child >72 years, the adult dose of l-2 gcan be used. The
drug is given over a period of 15-30 minures and repeated
after lJ hour. Inj. Obidoxime 3 mg/kg/hr seems more
effective (can cross blood-brain barrier).
Following recovery, the child should be observed closely
for at least 24-48 hours to ensure that the cholinergic
signs do not occur as atropine and pralidoxime effects
wear off.
Enzyme reactivation has no role in the treatment of carba-
mate poisoning and may be harmful.
Antibiotics for infections.
Crushed or powdered seeds or exrracr is used by criminals for
stupefying a victim prior to robbery, rape, or kidnapping. It is
usually given in food or drink, e.g., chapatis, curry, sweers, rea,
liquor, etc. ro rravelers in railway or bus starions. Accidental
cases occur usually in children by eating rhe fruits.
CLINICAT FEATURES
o Morton has described the symptoms as, "Hor as a hare,
blind as a bat, dry as a bone, red as beet and mad as wet
hen'. If the seeds are eaten, symproms appear within half
an hour; if a decoction of the seeds is given, within a few
minutes; and if alkaloids are used, almost immediately.
o A bitter tasre, dryness of mouth and throat with difficulty
in talking, dysphagia, burning pain in the stomach and
vomiting are first noriced.
r The voice becomes hoarse, face become fushed, conjunctiva
congested. Pupils are widely dilared with loss of accommoda-
tion for near vision and temporary blindness, photophobia
and diplopia develops. Light reflex is ar first sluggish and
later becomes absent.
o Mental changes include restlessness and agitation. patient
cannot recognize relatives or friends. Urinary retention
occurs. The patient becomes confused, giddy, staggers as if
drunk. The skin is dry and hot.
o The pulse is rapid 120-140/ minutes, full and bounding
but later becomes weak and irregular; the respiratory rate is
increased. The temperature may be raised by 2-3"F.
o Muscle tone and deep reflexes are increased, and there may
be muscular spasm.
o The patienr is restless and purposeless, delirious and is indi-
cated by exciremenr talkariveness, incoherence. The patient
-.!
may be silent but usually he is noisy, rries to run away
from his bed, picks at the bed clothes, tries to pull imagi-
nary threads from the tip of the fingers, threads imaginary
needles. Hallucinations of sight and hearing and delusions
may occur. This stage of excirement usually lasts for 2-3
hours, after then the patienr goes into deep sleep or coma
for 2-3 days but usually significant improvemenr occurs
after 24 hours. The toxic dose is 100-120 seed (0.6-1 g)
and fatal period is 24 hours.
DIAGNOSIS
Diagnosis is supported by detecting those substances in the
urine. It can be confirmed by demonstrating resolution of
anticholinergic toxiciry in respond to physostigmine.
TREATMENT
Emetics can be used.
o \7ash out the stomach repeatedly with a weak solution of
tannic acid or normal saline.
o Activated charcoa.l adsorbs these agents effectively and is the
preferred method of gastrointestina.l decontamination.
aCathartics are used frequently.
aPhysostigmine 0.5_2 mg IV given over 2-5 minutes may
be repeated at hourly interval if incomplete response or
recurrent toxicity.
a Delirium can be controlled by phenobarbitone.
a Light diet and free purgation should be carried on for 34
days to remove the seeds.
o Comatose parienr may require intubation and mechanical
ventilation.
Death within 24 hours of submersion is termed drowning,
which may be immediate or may follow resuscitation. Death
usually occurs due to asphyxia.
Survival of >24 hour is termed "near drowning" regardless
of whether the victim later dies or recovers.
After submersion in a liquid media, suffocation and asphlxia
may occur with or without pulmonary aspiration. Irrevers-
ible multisystem injury occurs very rapidly, often leading to
death.
Drowning occurs more often in fresh water than in sea
water. Though the pathology is almost same in both types,
the increased salinity of sea water can lead to hypovolemia,
hypernatremia, myocardial dysfunction, and acute tubular
necrosis in kidneys.
PATHOPHYSIOTOGY
Hypoxemia is the principal problem in submersion injuries.
Aspiration of water occurs in 90o/o of cases. The three effects ,
I
1
+

of aspiration are aspiration pneumonia, hypoxemia, and hypo-
thermia. Hypoxemia for >5 minutes results in neurological
damage. The brain is, to a certain extent' protected from
neurological damage by extreme hypothermia. Aspiration
pneumonia is characterized by alveolar collapse, hypoxemia
and adult respiratory distress syndrome (ARDS). The changes
that occur in lungs are almost similar in both fresh water and
sea water aspiration.
Major organ system involve:
Brain-hypoxic brain injuries are most serious and life-threat-
ening as well as are likely to cause long-term damage'
Lungs-pulmonary edema results from decreased surfactant
and results in atelactasis.
Kidneys-AM occurs in most lengthy drowning, but 80%
of the patients recover in 10 daYs.
TREATMENT
1. Resuscitation (ABC). Cardio-pulmonary resuscitation
(CPR) is the major determinant of out come.
a) Airway should be cleared of water, vomitus.
b) Breathing: Give oxygen and assist ventilation by
mouth-to-mouth breathing, ambu bagging' Chil-
dren who are comatose or those with apnea require
intubation or IPPV to keep the blood gases at an
acceptable limit (PaO, 100 mmHg and PaCO, 20-30
mmHg).
c) Circulation: Cardiac massage if needed.
2. Rewarming measures: Should be rewarmed rapidly'
Passive rewarming is done by warm water and dry blan-
kets. Active rewarming can be done by warm blankets,
radiant warmer, warm IV fluids. Temperature should
be monitored.
3. Pulrnonary edema should be treated by oxygen inhala-
tion and diuretics. Pneumonia, pneumothorax, or ARDS
should be treated, if present.
4. Mannitol can be used in cerebral edema. Seizure should
be controlled by anticonvulsants.
5. IV fuids and inotropic drugs (dopamine) are indicated
in shock and myocardial dysfunction.
6. ARF when present should be treated.
7. Metabolic: Hypoglycemia or hyperglycemia when present
should be given due attention.
8. Dyselectrolytemia: Sodibicarb may be administered for
metabolic acidosis. Hyponatremia (occurs when SIADH
is present), hypernatremia (occurs in saline water
aspiration), and hyperkalemia (when RBC breakdown
occurs in water intoxication) should be treated when
present.
9. bleeding due to thrombocytopenia or DiC should be
reated, when present.
10. Antibiotics should be given if infection is suspected'
11. A child should have aquatic skills (swimming).
POISONING
Source: \7ell water contaminated with arsenic Pesticides,
herbicides, dyes, homeopathic folk remedies. Industrial expo-
sure: Glass manufacturing pottery' electronic, semiconductors,
mining, smelting, refining.
Pathogenesis: Trivalent arsenic binds to sulfhydryl group
of mitochondrial enzymes such as pyruvate dehydrogenase,
u-ketoglutarate and interferes with glycolysis, hydrolyses AIP
Pentavalent arsenic uncouples oxidative phosphorylation'
.
CtINICAI FEATURES
Acute poisoning:
GIT Nausea, vomiting, diarrhea, stomatitis, abdominal
pain, salivation, epistaxis, hemorrhagic gastroenteritis with
fuid loss and shock.
Carfiac: Prolonged QT, ventricular tachycardia, congestive
cardiomyopathy, pulmonary edema, cardiogenic shock'
Neurologic: Delirium, seizures, cerebral edema,
encephalopathy, coma, peripheral neuropathy in severe
case, ascending paralysis resembling Guillain-Barre
syndrome.
Others: Fever, hepatitis, hemolytic anemia, pancytopenia,
alopecia.
Chronic intoxication:
o General: Fatigue, malaise, headache, chronic cough, weight
loss, painless perforation at nasal sePtum.
. Skini Finely mottled brown pigmentation in skin flexures,
temples, eyelids, neck, Ieg (rain drop pigmentation)'
o Hyperkeratosis of palms and soles.
o Thickening of nails and transverse white striae in fingernails
(Aldrich-Mee lines).
o CNS: Symmetrical sensorimotor peripheral neuropathy
(sensory predominate), anesthesia, paresthesia, encephalopa-
thy, wrisi drop, cramps, muscular tenderness, foot-drop'
r Eye Congestion, watering' photophobia.
. ill Diarihea, salivation, cralnPs' cirhosis of liver, jaundice'
r Kidney and CVS: Peripheral edema, chronic nephritis, CCF'
o Hematologic: Bone marrow suppression, anemia, throm-
bocytopenia.
INVESTIGATIONS
Plain x-ray abdomen: Radio opaque (in acute arsenic poi-
soning).
24-hour urinary arsenic level >50 pg/L (provided sea food
is not taken recently).
a Elevated arsenic in hairs/nails.
o Blood: Anemia, thrombocytopenia, leukoqtosis/leukopenia'
basophilic stippling of RBC.
Urine: Polyuria, proteinuria, hematuria.

ESSENCE OF PEDIATRICS
TREATMENT
Removal from the source.
Acute:
o Induction of vomiting/gasrric lavage.
o Administration of freshly prepared ferric chloride in
water.
o Fluid and electroly'te replacemenr.
o Dimercaprol3-5 mg/kg IM 4 hourly for 2 days;6 hourly
on 3rd day, then 12 hourly for l0 days (oral chelating agenr
may be substituted).
o Blood transfusion sos.
o Management of renal failure, if present.
Chronic:
o Removal from the source: Offer drinking warer from ponds/
rain warer if contaminated well warer is responsible.
o BAI (Dimercaprol)
o DMSA
o Penicillamine
o Spirulina-on trial.
Scalds are due to wer heat, most often following a spill of hot
milk, water, or oil being handled by the morher. Intentional
scalding as seen in child abuse can lead to a sharply demarcated
scald injury of the limbs or the glureal region.
Flame burns result from exposure to heat of high intensity as
in building fires and clothes catching fire. These produce full
thickness burns distributed over a larger area and may have
associated pulmonary complications.
Chemical burns result from conract with corrosives. These
involve limited area, and severiry of injury is dependent on
the strength of the corrosive.
Electrical injury is usually localized. High,voltage electricity
is however potentially lethal.
The severiry of the burn is assessed and graded from I to
3. Grade 3 burns are the severest and usually due to electrical
and chemical burns. Standard charrs are available for infants,
children, and adults, which grade the extent of burns. patients
with >30% burn area, inhalation burns, or third-degree burns
need to be referred to a special care burn unit. Intravenous
fluids may be started and oxygen administered
in case of
inhalation burn injury.
TREATMENT
Aid ql Home
First
r In case of flame burns, the fire is
put off by the parienr
rolling on the ground ("drop and roll") or covering the
I
patient with noninflammable material like blanket. In cases
of chemical burns, the burnr area is washed with cool water
to minimize tissue damage.
o For electrocution, the victim should be removed from
contact by use of a nonconducting material (wood or plastic).
CPR should be initiated as needed.
r The burn area should be covered with clean dry sheet and if
burn area is <15-20o/o, cold wet compresses may be applied.
Colored anriseprics like gentian violet and mercurochrome
should not be applied, blisters should not be punctured.
o Cardiovascular and pulmonary status should be examined
carefully. In case ofburns >10-l5o/o, inrravenous access'and
nasogastric tube are placed.
r In case of perineal or genital burns, an indwelling urinary
catherer is placed.
r Iftransportation ofthe parienr is going to be delayed by >30
minutes, small quantity of oral fluids can be permitted.
r Burns otier than those needing hospitalization can be managed
in the outpatienr. The burnr area should be dressed with baci-
tracin, nitrofurznne, or silver sulfadiazine cream twice a day.
Hospilol Treolmenl
As soon as patient arrives, an intravenous access is secured,
and assessment of severity and depth of burn is done. An
indwelling urinary carherer is placed if burn area is >l5o/o
or patient is in shock. The respiratory srarus is monitored.
100% oxygen should be administered where carbon mon-
oxide poisoning is suspected.
Fluid resuscitation for moderare and severe burns is impor-
tant. The 24-hour fuids to be infused are calculated using
the formula:
(4 mllkg body weight x o/o body surface area burnt) +
maintenance requirement of 1500 ml/m,
Of the total requiremem, 50o/o is given within the first
B hours, and the resr over the next 16 hours. funger lactate
is the preferred fluid for the first day; subsequently fluids
may be given as N/2 in 5olo dextrose. The fluids should be
reduced to 50o/o after the first day. Colloid can be admin-
istered if the serum albumin levels are <2 mgldl, or fuid
requirement is in excess of 300 mllm2lhr.
The burn wounds are ideal site for bacterial growth due
to the presence of necrotic tissue. Barrier nursing, early
excision of necrotic tissue, and provision of adequate skin
cover are useful in preventing infections. Antibiotics need
to be given in the presence of active infection (erythema,
purulent discharge, black or green discoloration). Surface
swabs from the wound will indicate the flora. A combination
of an antistaphylococcal penicillin and an aminoglycoside
can be started pending sensitiviry results.
:
Follow-Up
Following burn injuries, in spite of active physiotherapy, scar i
tissue may form over critical areas iike joints, neck, axilla, and "l
fingers. The scars in children are hypertrophic and may take !
1
1
til
rl

2-3 years to mature. Surgery for contracture release may need
to be postponed till the scar matures.
Snake bite is an important emergency public health problem.
The outcome of snake bite depends on the size of child, the
site of bite, type of snake, degree of envenoming, and effec-
tiveness of treatment. ,;
Of the 3000 known species of snake, only 200 are poisonous,
of these 90o/o are members of one of the three families:
o Hydrophiidae (poisonous sea snakes)
r Elapidae (cobra)
o Viperidae (vipers)
50% bites by Russell vipers and 30% bites by cobras do not
result in any symptoms or signs of envenoming.
o 80% of snake bites are non-poisonous.
o Cobras, kraits, and vipers are the common poisonous snakes
in Asia.
Poisonous snakes are of following types:
o Neurotoxic (cobra): Impair nerve conduction and causes
paralysis of muscles.
o Hemorrhagic-hemotoxic (viper): Causes tissue necrosis and
coagulopathy resulting in local and systemic bleeding.
o Neuro-hemotoxic (krait): Causes both neurotoxiciry and
hemotoxiciry.
r Sea snakes are both myotoxic and nephrotoxic.
CtINICAL FEATURES OF POISONOUS
SNAKE BITE
Viper bites can cause symptoms within minutes, always within
6 hours. Manifestations in cobra bites start within 15 minute
to 5 hour.
o Non-specific Apprehension, generalized weakness, nausea,
vomiting, abdominal pain, fainting.
o Local: Pain, swelling at bite site, rapidly spreading edema
and large blisters, bruise, swelling of limbs, necrosis, gan-
grene, fang marks, painful lymphadenopathy.
. Systemic:
c Neurological symptoms: Muscle paralysis, ptosis, diplo-
pia, ophthalmoplegia, broken neck sign, dysphagia,
drooling, nasal regurgitation, ioss of gag reflex, palatal
palsy, bulbar palsy, dysphonia, faciai palsy.
r Drowsiness, coma, flapping tremor, muscle rwitching,
convulsions.
r Respiratory: Respiratory distress, paralysis of chest mus-
cles and diaphragm, respiratory failure.
r Hematological: Spontaneous bleeding from various sites
(puncture sites, gut, gum, urinary tract, bloody saliva-
tion, intracranial).
POISONING
t) Coagulopathy
.J Circulatory collapse
o Renal: Scanty urine, myoglobulinuria, renal tubular ne-
crosis, acute renal failure.
o Myotoxicity: Muscle weakness, tenderness, black urine.
GRADING OF ENVENOMATION
o Grade 0: No envenomation
o Grade 1: Minimal envenomation (local swelling and pain)
r Grade 2: Moderate envenomation (local swelling, pain,
ecchymoses, mild systemic and lab manifestations).
o Grade 3: Severe envenomation (remarkable local response,
severe systemic findings, and significant alteration in lab
findings).
TABORATORY I NVESTIGATION
o CBC
r Coagulation test: 20 minutes WtsCT
o ECG
o Blood urea, serum creatinine, CPK
r Urine RME, myoglobinuria
o Immunodiagnosis (by ELISA)
MANAGEMENT
Antivenom is effective if given within 4 hours of bite, little
value if delayed beyond 12 hours. Despite the potentid for
mortality and severe morbidity, both can be minimized by
early use ol appropriate antivenom.
First Aid Treolment
r Reassurance
o Immobilizarion of bitten limb by a splint or a sling.
o If the bite is in lower limb, do not walk.
o If the bite is in upper limb, do not move the limb.
o Ideal is to provide pressure immobilization, especially helpful
for cobra and krait bite.
Pressure immobilization method:
o Pressure immobilization can be done by a crepe bandage
or by any long strips of clothe (like gamchha). The bandage
is wrapped firmly around the entire bitten limb, starting
H
distally around the fingers or toes and moving proximally
to include a rigid splint.
r Bandage should not be so tight to occlude the peripheral pulses
or a finger cannot easily be introduced beneath the bandage.
r Ideally compression bandages should not be released until
patient is under medical care.
Tronsfer lo Hospitol
o Quick transfer. Try to avoid oral feeding if the victim has
difficulty in swallowing, nasal voice.
o During transfer, bitten part shouid remain immobilized.
 Treotment in Heolth Focilities B) Antibiotic in contamination and multiple incisions
C) In case of local necrosis and gangrene, antibiotics,
l. Rapid clinical assessment: Look for envenomation
surgical debridement, and skin grafting.
2. Resuscitation:
A) Airutay (maintain clear airway) 6. Treatment of complications :

B) Breathing (mouth-to-mouth breathing and ambu A) Treatment of shock-fluid, dopamine

bagging; if facilities are available-intubation and B) Fresh blood 112n5fss16n-for bleeding
mechanical ventilation) C) Respiratory support in ICU (in bulbar/respiratory
C) Circul.atioz (assess pulse, blood pressure-support by paralysis)
IV fluid, preferably normal saline) D) Theatment of acute renal faiiure

3. Specific treatment Antivenom

E) Surgery when there is compartmental syndrome

A) Pollvalent antivenom is available in lyophilized powder 7. Drugs/Theatment not recommended:

form. Each vial contains 10 mg of antivenom effec- A) Antihistamine except for antivenom reaction
tive against cobra, krait, Russell viper and saw scaled B) Corticosteroid except for antivenom reaction
viper.
C) Sedative
A) Routine skin sensitiviry test for antivenom is not D) Aspirin and NSAID
recommended.
E) Heparin
B) Dose of polyvalent antivenom: Each dose consists of F) Tieatment by ozha
l0 vials irrespective ofage and sex. Each vial is diluted
with 10 ml D'W. Ten such vials (100 ml) are further 8. Monitoring:
diluted with 100 ml of fluid (saline), then administered A) Monitor viral signs
through IV infusion within 40-60 minutes. B) Assess signs and symptoms of (z) early anaphylaxis,
D) In case of H/O asthma, adrenaline prophylaxis is (ii) pyrogenic reaction, and (lll) late reaction.
recommended before starting therapy. i. Early anaphylaxis: Develops within 10-180
Infications for using polyvalent antivenom: minutes of starting antivenom. Common features
are urticaria, fever, angio-edema, dyspnea due to
o Neurotoxic signs: Ptosis, external ophthalmoplegia,
bronchospasm, hypotension, nausea, vomiting.
broken neck sign, nasal voice, respiratory difficulty.
Tieatment includes the following:
o Rapid extension of local swelling
. Acute renal failure a) Temporary suspension of antivenom
o Acute circulatory failure administration.
o Bleeding abnormalities b) Adrenaline{ I : 1 0000 solution) 0. I -0.3 mllkgl
o Hemoglobinurea/myoglobinurea dose IM, can be repeated every 5-10 minutes
interval.
Indication of monovalent antivenom: c) Antihistamine (chlorpheniramine maleate)
If the snake is definitely identified with signs and symp- 0.2 mglkgslow IV after dilution.
toms of envenoming. d) Hydrocorrisone24 mg/kg IV.
Criteria for repeating antivenom: e) Ranitidine I mg/kg IV.
r Persisting or deteriorating s/s of systemic envenoming. ii. Pyrogen reaction: Usually develops 1-2 hour
o No improvement after l-2 hours of completion of after treatment. Clinical features include fever
antivenom therapy. with chills and rigors, fall of BB febrile convulsion
o Persistence or recurrence ofblood incoagulability after may occur. Tieatment includes the following:
6 hours of antivenorn therapy. a) Paracetamol
b) Tepid sponging, fanning, plenry of fluid, t IV
4. Additional treatment:
fuid.
A) Combination of NeostigminelAnopine: Indicated for
neurotoxic features only-Inj. Atropine 15 pglkg IV
iii. Late reaction (serum sichness Upe)t M"y
develop l-12 days (mean7 days) after treatment.
followed by Inj. Neostigmine 50-100 pglkg SC, every
Clinical features include fever, itching, recurrent
4 hours until neurotoxic features are overcomed.
urticaria, arthralgia, myalgia, lymphadenopathy,
B) Antibiotic when indicated
proteinuria. Tieatment includes:
C) Tbtanrc prophylaxis if appropriate. a

a) Antihistamine
5. Tireatment of bitten part: b) Prednisolone, when no response to antihista-
A) \Washing with antiseptic solution mine t
{

rc r!
1
{
/
t
I

I
t.
POISONING
I
i
I In nonvenomous snake bite, patient should be kept on o Ingestion of oil, ghee, pepper, etc.
: observation for 24 hour. Tiaditional treatment of snake bite a Application of stones, seeds, saliva over bite sites.
I commonly practiced in Indian Subcontinent:
t:
v Tladitional healers (ozha) treat the victim in a variety of
t. traditional methods, most of them are harmful (can cause
l
infection, burn, and gangrene). 1. Faiz MA (ed.). Tiaining manual on manag€ment of poisoning
, guideline. \7HO, DGHS, 2008.
a Application of multiple tight arterial tourniquets.
I 2. Behrman RE, Kliegman RM, Jenson HB. Nelson Textbooh of
,
a Shedding of blood by multiple incisions. Pediatrics i8'h ed. Phiiadelphia; Saunders Elsevier, 2007.
I o Incision and suction of bite sites by mouth. 3. Khan MR, Rahman ME. Essence of Pediatrics 3'a ed., 2004.
F a Chemical cauterization by carbolic acid. 4. Park JE, Park K. Parki Tbxtbooh of Preuentiue dnd Social Medicine
t a Application herbal paste. 15'h ed. Jabai Pur, India: Banarasidas Bhanot Publisher,2005.

t"
t

t
t

:
:
f
?
r
f'p
 5

CHAPTER 21
lmmunization and lnfectious Diseases

Chopter Contents

Fevers in childhood.. ... .... .... .... . .. . ...............411 .

Short duration fever................................... ,''..,,..'.....417

Scarlet fever..........,.

14wk Pentavalent-3, OPV-3

9 mo Measles vaccine, OPV-4-"
'psnlxyalsnl-DPT, Hepatitis-8, Hib vaccine.
'Vtamin A (100,000 IU) is given along with measles vaccine.
Target populations:
EPI schedule of TT vaccine for 15-49 year old women:
0-1 I month old children and women at childbearing age (i.e.,
15-49 year). TT-1 At 15 years of age
Preventable diseases:
TT-2 At least 28 days after TT:1
1. Tuberculosis
TT-3 At least 6 months after TT-2
2. Poliomyelitis
TT-4 At least I year after TT-3
3. Diphtheria
TT-5 At least I year after TT-4
4. Pertussis o Though any woman comes late for the next scheduled dose,
5. Tetanus TT should be given to her according to the sequence of
6. Hepatiris-B order.
7. Haemophilus infuenza b o No need to take more than 5 doses of TT vaccine.
8. Measles r If 5 doses ofTT vaccine are raken according to the schedule,
no extra TT vaccine is needed during pregnancy.

EPI SCHEDUTES Dose, route of administration and adverse effects of vaccines

of EPI schedules have been listed in Table 21.1. ^
EPI schedule for 0-11 month old children:
At birth BCG, OPV-0 Note:
6 wk BCG (if not given at birth), Pentavalent.-1, OPV-I o All the vaccines under EPI schedule should be completed i
l0 wk Pentavalent-2, OPV-2 within I year of age. 1
a
I
e
fl
 IMMUNIZATION AND INFECTIOUS DISEASES

Table 21.1: Dose, Route of Administration and Adverse o If papule/scar formation does not occur with the BCG
Efiects of Vaccines of EPI Schedule vaccine within 3 months, it should be readministered along
with Pentavalent-3.
BCC 0.05 ml Ulceration, regional lymph node
o OPV vaccine should be given despite the fact that the
(intradermal) enlargement, Lupus vulgaris baby have acute watery diarrhea, it rn'ould be considered
rl0-15'/o) as additional dose; another dose to be given after 28 days
Pentavalent t,.5 ml Low-grade fever, redness. swelling. - should be registered.
(intramuscular) and pain at the site of vaccination' o The measles vaccine should be given at 9 completed months
Pollo 2 dropsldose Vacci ne-as:ociated pa ra lytic although the baby suffered from measles previously. Second
(oPV) poliomyeliris (VAPP' (l 5/10 dose of measles vaccine may be given after 4 weeks.
million doses)
r If administration of three doses of DPT/Pentavalent vaccine
0.5 ml Fever (>39.5'C), rashes (5-1 5%),
Measles is documented, these will be considered as two doses of
(SC) seizures, encephalitis r 1 /3
million dosest. TT at 15 years of age and the third dose of TT will have
to be scheduled.
TT iJ.5 mt Systemic reactions very unusual,
ilM) local reactions may oLcur
after repeated doses. When vaccine should not be given:
The complication of DPT/Pentavatent vaccine is convulsion or unconsciousness due o Very sick child admissible to the hospital.
lo lhe p|rtus.is ( omponcnl o[ lhe rar, ine. a H/O convulsion or unconsciousness with DPT/Pentavalent
vaccine previously. In this case, one dose ofTT/DT vaccine
If any dose is given before completion of the minimal inter-
should be given in place of DPT/Pentavalent.
val of the schedule, that dose wili be considered as invalid.
Parents do not consent.
In case of OPV Pentavalent, and TT vaccine, the highest
interval is unlimited. V{hatever may be the interval between
two doses, the first dose should not be started again. NEWER VACCINES
Though there develops abscess at the injection site, the next
dose should be administered timelv. Newer vaccines have been listed in Table 2I.2.

Table 21.2:. Newer Vaccines

Bacterial:
1. Typhoid 0.5 ml single dose lM or SC at 2 years of age Every 3 years, beginning
Vi capsular polysaccharide by the age 2 yr till age of
1B yr

Adverse effect,-mild local pain and swelling

Live attenuated Ty21a (suitable for use Oral capsule 3 doses on alternate days on empty stomach Every 3-5 years
onll in children above 6 years of age)

Antibiotics are contraindicated between 3 days before to 7 days after the vaccine
2. Haemophilus influenza b (conjugate 6 wk to 6 mo: 0.5 ml of lM, 3 doses, 4 wk interval. At 1B months
vacci ne) 6-12 mo: 2 doses, 4 wk interval.
>12-15 mo: One dose
(Beyond 1 5 months, one dose is sufficient)

Adverse effects - Iocal reaction (25'k), fever, headache, myalgia (<5%)

3. Pneumococcal
Unconjugated potysaccharide 23 valent 0.5 ml single dose lM, at >2 yr of age After 3-5 yr in high-risk
rat t ine {PPV 23) ch ildren-
Pneumococcal conjugate vaccine (PCV 0.5 ml, lM 3 doses at 6, 10, and 14 wk
At 15-1I mo
l;1i #; i,' 3li:,:;'#?:il'
2-5 Yr: Single dose
\drcr.e e//ects pain and redness al iniection site. fever. myalgia r<toL'. anaphylaxis rare.
4. Meningococcal
\ol ior rouline use
L nconiugated polysaccharide rBivalenl- 0.5 rnl IM or 5C single dose After 3-5 years (if required)
A,C; tetravalent-A,C,Y,W1 35)

( Conti n uecl on re\: :i:r H

 I

ESSENCE OF PEDIATRICS

Table 21.2: Continued

{dverse effec{s -:' lacal,iajn,::fiver;.and *ry$efu;iil;icaon:site,,,

Viral:
1. Hepatitis 0.5 ml (10 trrg) in children up 1o,,r l:y-rif 1r,0,,l11i.tio.rbrib ave,.rr,..:,
. . :: ::,,-:,,.ti.ii.,r,:l:g::yf;l.M.. t. . I,.i..
. - : ..i::r:,,; :, ::
r1The.dose,i9:dirub,l,gdjnpdlien19l,a,*hemodialysis, immunocomprOrtrised

N,lt.;aqnired
Schedule: ldeal at birth, 1 and 6 mo

lf birth dose has been missed, at 6 wk. l0 wk, and l4 wk

Adverseeffects-minorpainatiniectionsite,fever(1-6%).
2,r;,tlepatifisA '''i 'i ' l/M, 2 doses
+a betgiven Not,e411ir,d
6:mo:apirt after 18.:mo of iaee.,., .,,,,, . ..,'.,....,

Redness. swelling, pain at injection site

'3.
MMn.., :.. .. . . .
0.5 ml SC or lM
(eombinEd live attenudted vacc'ine),,' l.n doie.at 1i-15 mo of ige. Z:drdos€ after e, *n;g.,,,::r,,,r

4. Rotavirus vaccine
Two live oral vaccine
t'
.: IRotarix ' Orally 2 doses, at 2 mo and 4 rno of age.
RotaTeqr' ,' , ,
Orally 3 doses; 4t2t 4i and 6 ma:l i.:'": i- , r:ri I ::r'r'::r. :: r''ir': :

. t.: t.' ' : Shou|d,ll-o('begiven'<{yiofebe,. :. : ,. .1,,, ,,,

l.

Macilopapular:or.vAricelJiform ,ya5!1ISli);,toiai riinan |ZSV"), fever. ,, , ,, ,

6, Rabies: .:. -
.:.]:rjar:.i'...r:.:::.ll:i,.'.: r.,r:i:,,rr::::,r.i: -,,r::.i:-,.,
'.::Modiin,tissu€iuti*reyia'iil',ei$io,..':,$a,ii*$-;ryr*,
. Purified chick embryo cell (PCEC) 1 0 mr rM lelqspt v.r;rifb,'i;ut;.'.. ' -o; .., 14.ft er. ltl'.y,.eai rand en91y. 5.
28 t go
.r,.:r.:,.v€c.i p-ui:.1,,i.,- :i lyeairlferea€er,,,,:,:,:r.
:,' )!:':iPlrlrified:veIct,-eell
vaCcine.(FVRV1.-,.' . ,,.,.. . . .. l . .l
'1:r,,:,,"1.,'|,':.:,:::.lt',:
Verorab

Rabivax

:7.,ParidemicinflueirzaA (lllNl),vaciiner, , tO,s

ml fMslngledu-se , :, ,,,.,.,,-
'Sickle cell anemia, nephrotic
syndrome, renal faijure, organ trdnspldnts, or dsplenia.

VACCINATION IN SPECIAI. CIRCUMSTANCES o \VHO also recommends administering measles vaccine ro

all hospitalized severely malnourished children. A second
Prelerm Low Birth Weight Boby dose ofvaccine should be given before discharge.
o Same as EPI schedule
o Hepatitis B and BCG vaccinarion can be delayed at >2 kg
Immunodeficiency Stole
or at >2 months of age.
o But in case of bab;' of HBsAg-positive mother, both active o Children getting immunosuppressive
roid, cyclophosphamide. Children with malignant diseases
therapy like corticoste- :
and passive vaccination should be started ar birth.
like leukemia, lymphoma. Children with AIDS.
-
1
Protein-Energy Moln ulrition o Live attenuated vaccines (viral or bacterial) should nor be i
given in immunodeficiency state. 1
o OPViDPT-same as EPI schedule. r
t
Children getdng corticosreroid <2 mglkglL4 hr can be
o \ilZHO recommends administering BCG to malnourished. immunized during trearmenr. If the dose of the steroid is 2
children in endemic areas. mglkgl24 hr for <14 days, vaccination delayed until end of
 I

IMMUNIZATION AND INFECTIOUS DISEASES

therapy. If the duration is >14 days, immunization should heterotypic non-neutralizing antibodies act as a virus specific
be delayed at least 1 month. receptor, promoting the entry of the virus into mononuclear
phagocytes and thus convert mild infection into a severe
Boby of HBsAg-Posilive Molher infection. This is known as antibody dependent enhancement.
Interaction berween lymphocytes and dengue virus infected
o The risk of transmission is greatest (70-90oto) if the mother monocytes activates complement system and generates a lot of
is HBeAg positive. The child should be immunized within cytokines and other chemical mediators that act on capillary
few hours after birth: endothelium, making them permeable.
o HBIG (Hepabig) 100 IU in 0.5 ml, IM within 24hour Extravasation of plasma through these porous capillaries
of birth, Plus manifests as hemoconcentration (rise in PCV) in a mild case
o HB vaccine (Engerix B 10 pg in 0.5 ml vial) 3 doses (0, (DHF), but can produce a fatal shock in a severe case (DHF/
1, and 6 months) IM at separate sites in the anterolaterai DSS). This vasculopathy of increased capillary permeabiliry is
thigh. If HBIG is not administered, the baby should be the central pathogenic mechanism in DHF/DSS. Along with
,r".iirr"t.d at 0, 1 and 2 months along with an additional vasculopathy, DHF/DSS is accompanied by certain changes in
dose at 9-12 months. hemostatic mechanism, i.e., thrombocl'topenia, PT & APTT'
o At 12 months, blood should be tested for HBsAg and anti- and increased antiplasmin activity.
DSS sets in with the defervescence; hence, any child dete-
HBs. If anti-HBs is present, patient is protected; if HBsAg
riorating or failing to improve with subsidence of fever should
is positive, the patient is a carrier. If none is positive, the
be carefully assessed for signs of shock.
baby should be immunized afresh at 0, 1, and 6 months
schedule.
CLASSIFICATION OF DENGUE INFECTION
Dropout Coses ([ole Comers): Common
Voccines l. Asymptomatic
2. Symptomatic:
Ifa child misses a dose, complete the series of vaccination, no A) Undifferentiated
need for starting the vaccination afresh' B) Dengue syndrome
Immunization schedule first time for 1-5 year of age: i. Dengue fever-without hemorrhage
with hemorrhage
o Immunization with 3 DPT and 3 OPY Hep B, Hib' ii. Dengue hemorrhagic fever-without shock
o BCG at first visit. / with shock
o Measles vaccine can be given up to 2 yeat of age' MMR
can be given at 15-18 months of age. A second dose of
MMR should be given at 4-6 year or Il-12 yeat of age' CASE DEFINITIONS FOR CtINICAI
Immunization schedule first time after 5 year of age: MANAGEMENT
r 2 doses of OPV Tl Hep B. Dengue Fever
o BCG, if MT negative.
Dengue fever is an acute febrile illness of 2-7 days duration,
r MMR should be given.
sometimes with two peaks having the following manifestations:
1. Sudden onset continuous fever
and
2. Two or more of the following features:
Tiansmitted by infected female mosquitoes, mainly by Aedes a. Severe headache
aegypti (and also by Aedes albopictus)' Dengue illnesses are b. Retro-orbital pain
caused by four strains of dengue viruses (Den- I , Den-2, Den-3 c. Severe myalgia/arthralgia/back pain
and Den-4). Though it is a self-limited disease, but it has got d. Hemorrhagic manifestations
rwo potentially fatal symPtoms-dengue hemorrhagic fever e. Nausea/vomiting/abdominal pain
(DHF) and dengue shock syndrome (DSS). f. Leukopenia
First infection with anv of the dengue virus results in self- g. Rash
limiting febrile illness; recol'en'from first infection is accompa- and
nied bv generation of immunological responses. These resPonses ). High index of suspicion based on Period, Population and
F are characterized bv development of homon'pic neutralizing Place
l' antibodies and heteronpic non-neutraiizing antibodies' Homo-
r qpic antibofies confer a lifelong immuniw against causative
and
ft Absence of convincing evidence of any other febrile illness'
F strain. During second infection by another strain, pre-existing 4.
 ESSENCE OF PEDIATRICS

Dengue Hemorrhogic Fever Table 21.3: Crading of Dengue Syndrome

Dengue hemorrhagic fever is a probable manifestation of
dengue syndrome with hemorrhagic manifestarions having Features of DF asper Leukopenia
the following fearures: t a>e definition * Thrombocytopenia
1. Features of dengue fever at stage No change in
hematocrit
and
Featureslhistory of Thrombocy.topen ia
2. Hemorrhagic manifestations evidenced through one or features of DF (<100,000/mm3)
more of the following: + Hematocrit rise >2O"h
Positive Tourniquet
Positive tourniquet test Test
b. Petechiae/ecchymosis/purpura Features/history o{ Thrombocytopen ia
c. Mucosal bleeding: Epistaxis, gum bleeding features of DF (<100,000/mmr)
d. Bleeding from injection or other site Hematocrit rise>20"/o
Spontaneous bleeding
Hematemesis, melena, hematuria, PV bleeding
f. Thrombocytopenia with piatelets 100,000/mm3 or DHF (DSS) III l-eatures/historV ot Thrombocytopenia
less
teatures ot DF (<100,000/mmr)
and + Hematocrit rise >207o
3. Any evidence of plasma leakage due to increased capillary Features of circulatory
failure
permeability manifested by one or more of the following:
DHF (DSS) IV Features/history of Thrombocytopen ia
a. > 20o/o rise in hematocrit for age or sex features of DF (<100.000/mm')
b. 2 20o/o drop in hematocrit following treatment with + Hematocrit rise >207"
fluids as compared to baseline. Profound shock
c. Pleural effusion/ascites/hypoproteinemia
With or without Table 21.4: DHF Crades I and lt Therapy Chart
4. Hepatomegaly and circulatory disturbances

The cut offpoint berween Dengue Fever and Dengue Hemor- Febrile - Temp 39-40" C (102- At home
rhagic Fever is the evidence of plasma leakage, which will not
Duration 2-3 days 104" F) ' Bed rest
- Headache - Keep the body
be present in the former but invariably in the latter. - Retro-orbital pain temperature
- Muscle pain below 39'C
Dengue Shock Syndrome - Joint/bone pain - Paracetamol
- Flushed face - No aspirin/
Dengue shock syndrome is a presentarion of dengue syndrome - Rash NSAIDs
when a case of DHF manifests circulatory failure with one or - Skin hemorrhage, - Oral fluids (fresh
more of rhe Following fearures: bleeding from nose, fruit juices) and
gums electrolyte therapy
o Hyporension lor age - Positive Tourniquet test
o Cold clammy skin, restlessness, rapid weak pulse - Liver often enlarged - !O,TS)
roilow-up tor any
- Leukopenia change in plateleV
o Narrow pulse pressure (<20 mmHg) - PlateleyHct normal Hct
o Profound shock Afebrile (Critical) - Same as during febrile - Bed rest
Duration 2*3 days phase - Check platelet/Hct
GRADING OF DENGUE SYNDROME After febrile stage ' lmprovement in - Oral fluids and
general condition electrolyte therapy
Grading of dengue syndrome has been described in Thble 21.3. - Platelet/Hct normal
o At the initiai phase, one cannot differentiate DF and DHF. - Appetite rap)dly
regained
o The course is a continuum, passing from one grade to another.
o The transition period is at the afebrile phase. Convalescence - Further improvement - No special advice
Duration in general condition - No restriction
o If appropriate rrearment is not institured in proper time, ./- I U c.lavq and return of appetite - Normal diet
there is a risk of death in DHF-II, DHF-III, and DHF-IV after critical stage - Bradycardia
- Confluent petechial
rash with white center/
TREATMENT itching
' Weakness for
DHF Grodes I ond ll .1
or 2 weeks

Therapy chart for DHF grades I and II has been depicted

in Table 27.4, and voiume replacement flowchart has been lmprovement: Hematocrit fa1ls, pulse rate and blood pressure
depicted in Fig. 21.1. stable, urine output rises.
a
 IMMUNIZATION AND INFECTIOUS DISEASES

Hemorrhage (bleeding) tendencies,

Thrombocytopenia, Hematocrit rise,
Pulse pressure is low

lnitiate lV Therapy 6 mVkg/hr

Crystalloid solution for 1-2hr

Reduce lV 3 ml/kg/hr lncrease lV 10 ml/kg/hr

Crystalloid, duration Crystalloid, duration 2 hours

Further tr No improvement
improvement tr Unstable vital signs

*
Reduce lV to 6 ml/kg/
Discontinue lV hr crystalloid with further
alter 2,4hr reduction to 3 ml/kg/hr
discontinue after 24*48 hr

lV colloid (Dextran Blood transfusion

40) 10 ml/kg/hr 10 ml/kg/hr
duration t hr duration t hr

lV therapy by crystalloid. Successively reduce the flow from

$
10 to 6, 6 to 3 ml kflhr. Discontinue after 24-48 nr $

Fig.21 .1: DHF grades I and Il: volume replacement flowchart.

No improvement: Hematocrit/pulse rate rises. Pulse pressure Fluids recommended:

falls below 20 mmHg, urine output falls.
Unstable vital signs: Signs of shock; urine output falls.
When hematocrit cannot be done or is not available, the
following clinical tips may help:
o Crystalloids: (i) 5% dextrose in isotonic normal saline solu-
tion (5o/o DNS), (li) 5o/o dextrose in half-strength normal
saline solution (5o/oDlIl2lNS), (ir) 5%o dextrose in lactated
funger solution (5% D/RL), (ir) 5o/o dextrose in acetated
Ringer solution (5% D/RA), (u) 5o/o dextrose in aqua (5%

.jJi*
o If the patient has/had deep/massive bleeding from gut or
DA), and (erz) cholera saline.
other sites, the possibility is that the patient may have lower ' fri!;t {i""?"rffi #;:l"tfai
hematocrit because of blood loss.
o If the patient has/had surface/mild bleeding, the possibility
is rhat the patient may have higher hematocrit.
DHF Grodes lll qnd lV
o Sudden unexplained deterioration of hemodynamic status
and or refractory to adequate fluid therapy, the possibil-
ir"' is more of blood loss and hence low hematocrit
i:', el.
Therapy chart for DHF grades III and IV has
in Thble 21.5, and volume replacement flowc
depicted in Fig. 27.2. In case of acidosis, hv
funger lactate solution should not be used. il
 -l
ESSENCE OF PEDIATRICS

Table 21.5: DHF Crades and IV Therapy Chart

Afebrile {Critical) In addition to the manifestations of DHF Crade ll:
Duration 2-3 days - Circulatory failure manifested by rapid and weak
after febrile phase pulse, narrowing of pulse pressure r<20 mmHg) or
hypotension with the presence of r old clammy skin
- Capillary refill time >2 set
Profound shock with unstable pulse and blood pressure
- Check H( t and TPL
- lnitiate lV therapy r5% DNSr l0 ml/kg/hr
- Check vital signs, Hct. limed urine output
- lf improves. lV fluids should be reduced every hour from 1O to
6 and then from 6 to 3 ml/kg4rr, which can be maintained up to
24-48 hours.
- lf patient has already received one hour treatmenl of 20 mlzkg/hr
of lV fluids and vital signs are not stable, check Hct again anJ
- lf Hcl is increasing change lV fluid to colloidal solution preferably
Dextran or Plasma at 10 ml/kg/hr
- lf Hct is decreasing from the i"nitial value, give fresh whole blood
lransfusion. t0 mlzkglhr and corrlinue fluid therapy at l0 mUkg/hr
and reducing it stepwise lo 3 mlikglhr and mainlain it up to
24-48 hours
- lnitiale lV therapy r57o D\S) 20 ml/kgihr as a bolus one or rwo
times
- Orygen therapy
- lf t ontinued shock, colloidal fluids lDextran or Plasma.l at 1O-20
ml/kg/hr to be instituted
.l
- Persisting shock with declining Hct fresh whole blood 0 ml/kg as
a bolus
- Vital signs monitoring half hourly
- lf severe bleeding, fresh whole blood 20 ml/kg as bolus
- lf TPL < 500- 1000 mm , platelet-rich plasma transfusion
- After blood transfusion, conlinue fluid therapy al l0 mlikglhr and
stepwise reduce it lo 3 ml/kg/hr lo be maintained up to 24 48
hou rs

Convalescence 6-1 2 hours after critical/shock stage, some symptoms - Rest for 1 -2 days r

Duration 2-3 days of respiratory distress ipleural effusion, ascites) - Normal diet and no medicalion
after recovery from 2 '3 days after critical stage, stronS pulse and normal - Continued observation
critical stage blood pressure
lmproved general condition with return of appetite
Cood urine output
Slable Hcl
TPL >50,000 mm'
Bradyt ardia/arrhythmia
Eligible for discharge lrom hospital
Asthenia and depression continue for tbw weeks in adult

1. Capillary refill time. It can be measured by pressing the nail ofthe thumb of left hand in right handed person or vice versa till blanching, then suddenly release the pressure
The time taken for flushing is the capillary re{ill time.
2. Oxygen is obligatory until shock has been overcome. Pulse BP, temperature should be recorded half hourly.

Some lmporlonl lnsiruclions If improvement occurs, reduce the speed from 20 mi to 10

Check list:
r Cases of DHF should be observed every hour.
o Serial platelet and hematocrit determinations for drop in
platelets and rise in hematocrit are essential for early diag-
nosis of DHF.
o Timely intravenous therapy with isotonic crystalloid solu-
tion may prevent shock and or lessen the severity. Be careful
about the temperature of fuid to avoid chills and rigors.
o If patient's condition becomes worse despite giving 20 mi/
kg/hr, replace crystalloid solution with colloid solution such
as Dextran or plasma. As soon as improvement occurs,
replace with crystailoid.
o Preferred dose of colloid is 10 ml/kg (maximum dose 30
ml/kg/d).
ml, then 6 ml, and finally to 3 mllkg.
If hematocrit falls, give blood transfusion 10 ml/kg and
then give crystalloid IV fluids at rhe rare of 10 ml/kg/hr.
In case ofsevere bleeding, give blood rransfusion about 20
ml/kg over 1-2 hours. Then give crystalioid at 10 mI/kg/hr
for a short time (30-50 minutes) and later reduce the speed.
a In case of shock, give oxygen.
a For correction ofacidosis (sign: deep rapid brearhing), use
sodium bicarbonate. Available Sodibicarb solurion is of the
srrength 7 .5o/o, i.e., it contains 2 mmol/ml. So, 50-i 00 ml
of Sodibicarbonate is to be added to make up to one liter
of IV fuid of glucose containing crystalloid.
Check for any concomitant other medical or surgical condi-
tion and or any rnaintenance therapy.
I

.t
{
 IMMUNIZATION AND INFECTIOUS DISEASES

Unstable vital signs

Urine output lalls
Signs of shock

lmmediate, rapid volume replacement, initiate

lV therapy 10-20 ml/kg/hr crystalloid solution 1*2 hour

No improvement
$
@

t
I

lV therapy by crystalloid
successively reducing from 20 to
10, 10 to 6, and 6 to 3 ml/kg/hr

lV colloid (Dextran 40) or plasma Blood transfusion (10 ml/kg/hr) i{

Discontinue intravenous therapy 10 ml/kg/hr as intravenous bolus Hematocrit is still >35%
atler2448hr (repeat if necessary)

ln case of acidosis, hyperosmolar or

Ringer lactate solution should not be used. flow
lV therapy by crystalloid, successively reducing the H

*9T 9-19 L9 !'""gl1!e/Jj*i :9gll' e"*".J319*[

"1
J
tig.2"l.2: DHF grades lll and lV: volume replacement flowchart.

Donts: o No evidence of external or internal bleeding

o Do not give aspirin or NSAID for the treatment of fever.
r Return of appetite
o Avoid giving intravenous therapy before there is evidence
o Good urinary output
of hemorrhage or bleeding.
o Stable hematocrit
o Avoid giving blood transfusion in DHF unless indicated,
o Convalescent stable petechial rash
fall in Hct or severe bleeding.
o Do not use antibiotics per se for dengue syndromes. Dischorge Crilerio
r Do not change the speed of fluid rapidly, i.e., avoid rapidly o Absence of fever for at least 24 hours
increasing or rapidly slowing the speed of fluids. o Presence of signs of recovery
o Introduction of nasogastric tube to determine concealed
bleeding or to stop bleeding (by cold lavage) is nor recom-
mended, since it is hazardous.
o Avoid IM injections.
e Avoid tooth brushing in presence of gum bleeding. Measles is caused by an RNA paramp<ovirus and is characrer-
o Avoid steroids. ized by coryz4 fever, conjunctivitis, cough, Koplik spot, and
a distinctive maculopapular rash. It occurs usually before 3
Please note that DF and DHF are self-limiting conditions, so
one has to provide just therapeutic and other supporrive care

il
to prevent complications.

Signs of Recovery
o Srable pulse, blood pressure, and breathing
**r
r.t
rate
o Normal temperature
 I
ESSENCE OF PEDIATRICS

characterized by cough, cotyza, conjunctivitis, fever. At the TREATMENT

end of prodromal stage, Koplik spot, which is pathognomonic
of measles, appears on buccal mucosa opposite to lower molar No specific trearment. Bed rest, antipyretics and adequate
tooth as greyish white grain ofsand surrounded by red areola. fluid and calorie intake are indicated. Oral hygiene should
It may occur in other parts of buccal mucosa. A transverse line be maintained. Isolate from non-immune children until 5
of conjunctival inflammation demarcated by eyelid margin days after the rash has appeared.
is also important. Rash appears on 4-6th day of fever, and Bacterial superinfection ofthe ears or lungs should be treated
with the peak of fever. The erythematous maculopapular rash promptly with suitable antibiodcs; co-trimoxazole is appro-
starts from behind the ear, spreads in cephalocuadal direction priate for both conditions, although it may be necessary to
to the extremities in 3-4 days and fades in 3-4 days leaving use Inj. penicillin and gentamicin for severe pneumonia.
desquamation. Measles is highly infbctious 2-4 days before In non-responsive cases, pulmonary tuberculosis should be
and 4 days after appearance of rash. looked for and treated if found.
A single oral dose of vitamin A H-P (200,000 IU if >1 year,
and 100,000 unirs to <1. year of age) should be given on
crAssrFtcATroN admission to prevent ocular complications.
Inosiplex (isoprinosine) has been used in the long-term
L Severe complicated measles: \Vhen children with measles
treatment of subacute sclerosing panencephalitis.
having any general danger sign (convulsion, lethargy/
unconsciousness, inability to drink/breasdeed, vomits
everything) or deep and extensive mouth ulcers or clouding PREVENTION
ofthe cornea.
Active immunization: Measles vaccine at 9 month to 2 years.
2. Measles with or mouth complications: Children with
eye
Active immunization of measles vaccine can be given after 9
less severe measles complications' such as pus draining
months of age and again at 15-18 months of age along with
from the eyes (a sign of conjunctivitis) or non-deep and
MMR vaccine.
non-extensive mouth ulcers.
3. Measles: If children come with measles or history of
measles within last 3 months, but having no complica- PROGNOSIS
tions of measles.
o It may be associated with 1-3olo mortality (5-l5oh during
epidemic).
coMPHCATTONS o Death is usually from fulminant toxic illness, second-
ary pulmonary or intestinal complication, myocarditis or
Respiratory: Otitis media, laryngitis, post-measles broncho- encephalitis.
pneumonia, giant cell pneumonia, emPyema, mediastinal
emphysema, faring of TB
a GIT: diarrhea, cancrum oris
a Myocarditis
Mumps is highly contagious acute viral (Paramlxovirus) disease
a Malnutrition, vit. A deficiency, septicemia especially with
characterized by painful enlargement of salivary glands (mainly
Staph. aureus, DIC
parotid) and spread by direct contact, droplet infection, and
a CNS: Encephalitis, Guillain-Barre syndrome
by fomites. Disease is rare before I year of age, and incidence
a Eye: Keratitis, corneal ulceration
is highest among school-going children.
a Subacute sclerosing panencephalitis

CTINICAL FEATURES
DIAGNOSIS
After an incubation period of 2-4 weeks, prodromal symptoms
Suspect measles if
(z) maculopapular rash after 3 days of
of anorexia, fever, sore throat, and ear ache on chewing and
fever, (ii) fever >39"C, and (iii) any one of cough, cotyza,
swallowing may occur. Tender edematous swelling of parotid
con junctivitis.
gland (unilateral or bilateral) occurs within 1-3 days obliterating
mandibular angle. Enlarged parotid gland displaces the ear lobe
DIFFERENTIAT DIAGNOSIS upwards medially and associated with homolateral pharyngeal .
and soft palate edema with displacement of tonsil medially.
Scarlet fever, enteric fever, dengue fever, exanthem subitum, Stensen duct opening is red. Tenderness and pain subsides \
I
drug rash, infecdous mononucleosis. in 1-3 days, but for complete regression of swelling it needs a
1

ll
I

6-7 days. Submandibular and sublingual glands involvement
may accompany parotitis but rarely is the only manifestation
of the disease. The testes, ovaries, pancreas, and CNS may be
affected by mumps.
DIFFERENTIAL DIAGNOSIS
Cervical (pre-auricular or anterior cervical) lymphadenitis
(mandibular angle may be obliterated but earlobe is not
displaced, Stensen duct orifice normal, and neutrophilic
leukocytosis in blood).
Acute suppurative parotitis (tenderness and toxicity of
patient. Pus comes out through Stensen duct).
Recurrent parotitis (idiopathic or associated with calculus
in duct).
DIAGNOSIS
o H/O exposure to mumps patient.
o Characteristic clinical features and elevated serum amylase
(salivary level) in blood.
o Culture of virus from saliva, blood, urine, CSF or significant
increase in mumps antibody from acute to convalescent
stage.
TREATMENT
Treatment is entirely supportive and symptomatic with treat-
ment of complications if any.
I
I o Paracetamol for pain and fever
I o Local appiications of heat
I o
I o \7arm saline mouth wash
o teatment of orchitis: Bed rest, local support, prednisolone
(40 mg daily for 4 days to relieve pain and swelling, but
I
t does not reduce the risk oftesticular atrophy and the dura-
L tion of orchitis).
t autism, diabetes or thyroid dysfunction, progressive rubella
i'
Ir coMPUCATTONS
Usually uncommon and not severe.
o Meningoencephalitis (10%).
e Orchitis: Rare in prepubescent boys, but develops in20-30o/o
of postpubertal man; it usually is unilateral. Approximately
50o/o of infected testes show some degree of atrophy, but
infertiliry is rare.
o Pancreatitis: Usually present as epigastric pain and tender-
ness combined with fever, chills, and vomiting.
Unilateral deafness in 1:20,000 mumps Patient, but deafness
is transient or permanent. Bilateral severe deafness is rare.
Other complications: Oophoritis, nephritis, thyroiditis,
mvocarditis, mastitis, arthritis, thrombocytopenic purpura.
IMMUNIZATION AND INFECTIOUS DISEASES
PREVENTION
Isolation of patient until period of infectivity (2 days
before and 7 days after the appearance of salivary gland
swelling).
MMR should be given as described below under rubella.
Rubella (German measles or 3 days measles) is a viral exan-
thema caused by an RNA rubella virus and characterized by
mild constitutional symptoms, lymphadenoPathy (suboccipital,
postauricular, and posterior cervical) and maculopapular rash
of 3-5 days duration. Arthralgia may be Present.
CONGENITAL RUBELTA
Rubella virus is teratogenic, causes congenital rubella syndrome
(CRS), which is a tragic but preventable disease. During
maternal infection, rubella virus can cross the placenta, infect
the fetus and result in death of the concePtus or birth of a
newborn infant with congenital rubella. The gestational age
of the conceptus at the time of infection is a critical factor in
determining the outcome.
Detectable defects due to infection: 907o before 11th week'
diminishing to 10-l2o/o by the end of lst trimester; maternal
infection after 16th week poses a low risk ofcongenital defect.
Clinically, CRS includes a triad of malformations-cataract,
sensorineural deafness, and PDA'
The most common manifestations of rubella embryopathy are:
50-85o/o growth retardation (<2500 g at birth), microcephaly.
o 35o/o congenital cataract, 90olo hearing loss, 5%o congenital
glaucoma.
o l0-20o/o active meningoencephalitis.
o (PDA, pulmonary artery stenosis, VSD)'
30o/o cardiac defects
o Other features are pneumonitis, hepatitis, Purpura, infantile
encephalitis.
DIAGNOSIS
o Clinical sus?icion
o Virus isolation: May be isolated from throat, urine' or CSF'
o Serological test: Detection of rubella antibody: four-fold
rise, presence of IgM antibody to rubella virus in neonate
indicates rubella infection.
TREATMENT
1. Postnatal infection
a) No specific treatment: Tleatment is symptomatic'
b) Arthritis usually responds to analgesic.

ESSENCE OF PEDIATRICS
c) Steroid therapy is of no proven benefit for complica-
tions such as arthriris, encephalitis, or thrombocyto-
Penia.
2. Congenital infection
a) Immune serum globulin (20 ml) may be effective
in preventing the disease, if administered to a non-
immune pregnant woman within T2hours of exposure
to rubella.
b) Termination of pregnancy should be considered fc;r
serologically proven rubella in early pregnancy.
c) Most babies with congenital rubella are contagious,
and should be isolated. Some may excrere virus for
>1 year.
d) MMR (priorix) is recommended at 12-15 months'of
age (95o/o efficacy); second MMR is recommended at
4-5 year, or children who have not previously received
the second dose should be immunizedby 17-12 year
of age. Protective efficacy is 97o/o.
Roseola infantum (exanthema subitum) is caused by herpes
virus 6. It is common in children between 3 months and 4
years of age with a peak incidence in the first year.
Clinical features include abrupt fever lasting 1-5 days, rapid
defervescence on day 3 or day 4 and subsequent appearance
of any erlthemarous maculopapular rash largely confined to
the chest and abdomen. This rash usually disappears within
24 hours. The following features may also occur: cervical and
post-occipital lymphadenoparhy, mlld coryza, upper eyelid
edema, seizures (commonly febrile convulsion) during the
early stages of illness.
Diagnosis is made clinically, as viral studies are often
negative.
Treatment: Mild, often self-limited. There is no specific
treatmenr orher rhan symptomatic measures.
Note:
Other viruses (e.g., adenovirus, parainfluenza virus, RSV, and mycoplasma
infections) have occasionally been associated with maculopapular rashes,
may mimic rubella or roseola.
Er1'thema infectiosum (fifth disease) is a contagious disease of
infancy and childhood, caused by parvovirus Bl9 infection.
It is characterized by rash, but is not usually associated with
constitrltional symptoms, such as fever, headache, anorexia,
or arthralgia. The rash appears in three phases: it begins with
a livid erythema of the cheek described as slapped cheeks; a
maculopapular rash then appears on rhe exrremities and trunk
and the rash may recur periodically over a period of 24 week
especially if the skin is irritated. Epidemics rnay occur. Sequelae
I
are uncommon, but may be associated with an aplastic crisis.
Diagnosis is made clinicaily.
T]leatment:
Supportive; no specific rreatment. IV immunoglobulin in
im munocomprom ised patienrs.
Coxsackievirus A and B and echovirus have been associated
with many rash syndromes. Hand-foot-and-mouth disease is the
most specific syndrome following a short prodrome characrer-
rzedby malaise, anorexia, and fever. The patient becomes aware
of a sore mourh and throat. A vesicular exanthem subsequently
develops, involving the buccal mucosa and less commonly, the
tongue, palate, and gums. A vesicular exanrhem also appears
on the hands in about 55Vo of patients, and the feet are often
affected. Enteroviral infection may also produce morbilliform
roseola-like or rubella-like exanrhems. Pleurodynia or asepric
meningitis may occur. There may be epidemic. Diagnosis is
by virus isolation.
No specific trearment.
Caused by polio virus, an RNA enterovirus, having three dis-
tinct serotypes: Type I, Typ. II Type III polioviruses.
Humans are rhe only reseruoir of poliomyelitis, and rransmis-
sion occurs mainly by feco-oral route. Peak age group around
2-3 year. Incubation period is about 2-3 weeks.
Polio virus can involve whole CNS but commonly anterior
horn of spinal cord, cranial nerve nuclei, and motor area of
cerebral cortex. It may presenr with acute flaccid paralysis (i.e.,
acute-rapid progression from weakness to paralysis (<14 day$;
flaccid-floppy, neither stiff nor spastic; and paralysis-r.rnabie
to move the affected parr and the paralysis is not from birrh,
not from injury).
Commoner causes of acute flaccid paralysis (AFP) are para,
l1'tic poliomyelitis, GBS, transverse myelitis, rraumaric neuritis.
CTINICAI FEATURES
Depend on clinical types of polio and involvement of neurons.
TYPES
1. Asymptomatic (90-95o/o).
2. Abortive (4-8o/o)z Presents with infuenza-like manifesta-
tions (e.g., fever, nausea, vomiting, sore throat, headache,
and loss of appetite) with no neurological manifestations.
3. Non-para$tic polio (I--2o/o): Polio virus invades the CNS \
without destroying neurons. Headache, vomiting, neck 1
t
and back pain are complained. Neck rigidiry is usually
t

present. Tiipod sign, kiss the knee test, and head drop
signs can be elicited. Patient remains entirely conscious
despite signs and symptoms simulating meningitis.
4. Paralytic polio (0.5-lVo): Four types depending on
involved sites:
A) Spinal form: Extremities are commonly involved
(lower > upper, and proximal group of muscle > distal).
Flaccid rype of paralysis develops suddenly. Paralyses
are asymmetricai and sensation is intact. Involvement
of diaphragm or intercostal muscles causes respiratory
distress.
B) Bulbar form: Involves cranial nerve nuclei and med-
ullary vital centers. Paralysis of vagus nerve results in
weakness of soft palate, pharynx; nasal regurgitation of
food and nasal voice. Breathing and swallowing becorne
difficult. Pulse, BB and respiration become unstable
and irregular due to involvement of vital center.
C) Bulbo-spinalform: Features of both spinal and bulbar
polio.
D) Encepbalitic form: Rare and presents with features
of encephalitis-like irritabiliry, drowsiness, and tremor
or convulsion.
INVESTIGATIONS
r Isoiation of polio virus from the stools and throat swab are
diagnostic (two samples of stools 24-48hours apart within
14 days ofonset ofparalysis is taken for isolation ofvirus).
Virus is found in the feces from 72 hour prior to paralysis
to 6 weeks after infection, especially during 2 weeks after
onset of paralysis.
o Demonstration of increase in polio antibody titer in paired
sera taken 2 weeks apart is diagnostic.
o CSF study: Paralpic poliomyelitis shows clear CSE increased
cells (1 0-500/mm3, predominantly lymphocytes).
TREATMENT
Bed rest: Complete bed rest is always indicated. Exertion in
acute phase is correlated with more severe paralysis. Change of
posture every 2-3 hours to prevent bed sore and pneumonia.
Relief of muscle pain, spasm: Fomentation using hot packs
with soaked towel for 10 minutes 2-3 times daily, analgesic
given for pain and fever.
Correct positioning of limbs: Parulyzed limbs should be
placed in the optimum position for relaxation (e.g., hip
slight flexion, knee 15o flexion, etc.).
Passive movement of limbs: Joints and paralyzed muscles
should be moved passively gently through fuli range of
motion to prevent contracture. Massage and intramuscular
injections are contraindicated in acute stage. As acute phase
subsides, active movement is initiated. Maximum recovery
of the affected muscles takes place in the first 6 months,
and slorv recovery continues up to 2 years.
IMMUNIZATION AND INFECTIOUS DISEASES
o Monitor for respiratory failure and treat appropriately with
physiotherapy and ventilation, if necessary. tacheostomy,
in respiratory difficulty.
o Bladder paralysis may respond to bethanechol 5-10 mg
orally, or catheterization may be necessary' Transient urine
retention responds to alternate hot and cold compression.
o Long-term management of paralysis, muscle wasting, and
skeletal deformiry may require specific ofthopedic procedures
and physiotherapy.
PROGNOSIS
o in non-paralytic poliomyelitis, with
Recovery is complete
or without muscle weakness.
Some improvement of paralysis is expected with time
(depending on the extent and localization of the nerve cell
damage), e.g.,60% of ultimate recovery by 3 months, 80%o
of ultimate recovery by 6 months.
Mortality is overall 4o/o with 10% mortaliry in bulbar form
despite full supportive therapy. Mortality is usually from
respiratory failure (both spinal and bulbar polio).
Vasomotor center involvement may result in cardiovascuiar
complications (hypertension, arrhythmias, shock).
STRATEGIES FOR POTIO ERADICATION
Conduct "Pulse Polio Immunization days" every year for
3-4 years or until poliomyelitis is eradicated.
Oral polio vaccine (OPU is excellent, if administered as per
standard schedule (see immunization schedule)'
Improved surveillance capable of detecting all cases of acute
faccid paralysis due to polio and non-polio etiology.
Rapid case investigation, including the collection of stool
samples for virus isolation.
Follow-up of all cases of acute flaccid paralysis at 60 days
to check for residual paralysis.
Outbreak control for cases confirmed or suspected to be
poliomyelitis ro stop transmission'
Human rabies is viral infection of central nervous system
caused by neurotropic RNA virus. Disease is 100% fatal' once
symptoms develop. Disease is transmitted by contamination
of wound by saliva of rabid animal viz., dogs in 90olo cases;
wolf, fox, monkey, vampire bat, domestic animals such as
cat, goat, sheep, horse in the remainder. The transmission is
through (l) animal bke; (ii) lick on abraded skin, or abraded
and unabraded mucosa; (iii) aerosol (respiratory) spread by bats;
and (iu) rarely, by human bite, corneal and organ transplant'
The incubation period is variable, usually 20-180 days (may be
as long as 7 year).
 -
ESSENCE OF PEDIATRICS

CTINICAL FEATURES
Rabies could manifest as "furious" or "paralytic" type. Begins
with prodromal symptoms like headache, malaise, sore throat,
and light fever lasting for 3-4 days. About B0% of the parienrs
may complain of pain or tingling at the sire of the bite.
The prodromal stage is followed by widespread excitation
and stimulation of all parts of nervous system, as evidenced
by intolerance to noise, bright light, or a cold draught of air.
(sensory); aerophobia (fear of air) may be present. It is elicited
by fanning a currenr or air in front of face, which in rurn
induces violent spasms of the pharyngeal and neck muscles.
Increased reflexes and muscle spasms (motor), dilatation of
pupiis, increased perspiration, salivation and lacrimation (sym-
pathetic) are elicitable during the course of the disease. Mental
changes iike fear ofdeath, anger, irritabiliry and depression are
noticeable. As the disease progresses, the mere sight or sound
of water may provoke spasm of the muscles of degliitition
(hydrophobia), which is most pathognomonic in humans and
NOT characteristic in animals. The duration of whole illness
is 2*3 days, but may be prolonged to 5-5 days. The parient
may die abruptly due to convulsions or pass on ro a stage of
paralysis and coma. In 20%o cases, ascending symmetric paralysis
with flaccidity and decreased tendon reflex occurs.
Cleaning and flushing the wound thoroughly with plenty of
soap water to remove as much saliva as possible, and thereby
the virus from the wound. The mechanical removal of virus
should be foilowed by irrigation of wound by virucidal, such
as 1%o povidone iodine for at least 10 minutes. Tire wound
should nor be surured ar least within 24-48 hours of injury,
because it wili help the virus to gain access inro deeper rissues.
Measure should be raken againsr reranus.
PREVENTION
Pre -exposure Prophyloxis
Preventive vaccination is recommended for people exposed to
repeated risk of infection through close contacr with reservoirs
or animal vectors e.g. laboratory staffs, veterinary slrrgeons,
doctors. \fHOrecommended the foliowing vaccine schedule:
Thus preventive rabies vaccination comprises human diploid
cell vaccine (HDCV each dose = 1 ml), purified vero-rabies
vaccine (e.g. Verorab, each dose = 0.5 ml) or purified chick
embryo cell (PCEC e.g. Rabipur, each dose = 1ml). Vaccine is
given on DO, D7 and D28 IM over deltoid. A booster dose
is given after 1-3 years.

DIAGNOSIS
Posl-exposure Prophyloxis (Toble 21 .6)

The presence of hydrophobia (50o/o of cases), history of dog

Table 21.6: Recommendations on Post-exposure Prophylaxis
against Rabies
bite in a rabies endemic counrry, absence of muscle rigidity
between spaslxs, lymphocytic CSF with normalislightly raised
protein shouid caution the diagnosis of rabies, rvhich should be
subsequently confirmed by (z) demonsrration of rabies antigen
Dogs, cats Healthy and Should not begin
by antibody techniques in corneal smear, skin biopsy from available for 10-day prophylaxis unless
the back of the neck at the hairline or fi'om the brain biopsy, observation signs of rabies
(ll) isolation of rabies virus from saliva by mouse inoculation develop in animal*
or tissue culture (takes 7 days), (iii) demonstration of rising Rabid of suspected lmrnediately
antibody titer in serum and CSF, (izr) demonstration of Negri rabid (unknown or vaccinate
bodies on brain biopsy, or (zr) culture of rabies virus. In animals, escaped)

rabies can be confirmed by demonstration of rabies antigen, Skunks, raccoons, Regarded as rabid Consider immediate
foxes, most other unless animal vacci nation
Negri bodies on brain biopsy, or cultule of rabies virus.
carnivores, bats proven negative by
laboratory tests
DI FFERENTIAL DIAGNOSIS Livestock, small L onsrder rndrvrcju.t ly
I Consult public health
rodents, lagomorphs officials; bites of
Encephalitis (both primary and following rabies vaccine), (rabbits and hares) squirrels, hamsters,
tetanus, bulbar poliomyelitis, and Guillain-Barre syndrome, large rodents guinea pigs, gerbils,
(woodchucks and chipmunks, rats,
hysteria.
beavers), other mice, other small
mammals rodents, rabbits, and
TREATMENT hares almost never
require antirabies
prophvlaxis
No effective treatment is available. The only oprion is support-
-During the 1O-clar holcling periocl. beg:n posi-e\posure prcphrlaris \vlth hunran
ive management. Adequate opiate analgesic will relieve terror
rabies inrnrune g obulir HRIC :ncl raitjes raccine HUC\', PCEC. or PVRV) at first
and pain. Intensive care for prolonging, or possiblr- saving, life siqn ot rabies in a cloe or c.r: th.rt has bitten someone. li the aninral exhibits clinical
and to prevent compiications. The patient should be strictlv sign: oi rabies. jt shoulcl lte euthanizecl inrnrecli.rtelr ancl testecl. :,
isolated, and the staff should wear goggles, masks, and gloves \lodified from Centers ror Disease Control: Human rabies prevention - United States, 1
in addition to being immunized.
1999, Reconrmend.rtions of the Aclvison' Conrmittee on lmmunization practices
{ACIP)
t.t

t

Active lmmunization
Injection HDCV PVRV (i.e., verorab), and PCEC are com-
monly used in active immunization' These are risk-free' well-
tolerated, and more antigenic. NTV-ARV are often associated
with severe neuroparalytic complications (polyneuropathy,
encephalomyelitis).
Dose schedule:
(*) Subject unaaccinated against rabies: Inj. HDCV (1
ml) or Verorab (0.5 ml) or Rabipur (1 ml) is given on
day 0,3,7, 14,30,1 90 IM over deltoid' Individuals at
continued risk of rabies exposure should have antibody
titer determined every 2 years; if inadequate, should be
given a booster dose.
(b) In subject fully aaccinated:
i. If bite within I year: One injection should be given'
ii. If bite within 5 years: 2 injections on days 0, 3 should
be given.
iii. If bite after 5 years: Revaccination done.
(.) NTV-ARV (Nerae tissue uaccine-anti rabies aaccine
or lrhenolized sheep brain uaccine): 14 injections at a
dor. of 2.5 ml (in case of children) or 5 ml (in case of
adult) by SC route in the anterior abdominal wall around
umbilicus with three booster doses on days 10, 20, 90
after completion of 14 injections may be given'
Passive lmmunization
Human rabies immunoglobulin (HRIG; dose' 20 IU/kg) or
equine anti-rabies serum (ARS; dose, 40 IU/kg) infiltrated in
the area around and into the wounds. Passive immunization
should not be given once B days has elapsed.
Acquired immunodeficiency syndrome (AIDS) is caused by
human immunodeficiency virus type 1 and 2 (HIV)' Th'
predominant cells infected are CD4+T lymphocytes (i'e', T -
i,elper cells, CD - cluster differentiation), depletion of which
causes immunodeficiency. HIY-2 infection is rare in children'
TRANSMISSION
The pediatric population at risk for HIV-1 infection are infants
born to infected mothers, children given HIV-1 contaminated
t ,.rrrl, i., severe chronic diarrhea and malnutrition' Atypical
blood or blood products, and adolescents who acquire infection
I mycobacterial infection wirh Myco bacterium auium intracel-
sexualiy or bv use of intravenous drugs. Most large studies in
a United States and Europe have documented mother-to-child
c transmission rates in untreated women between l2o/o and30o/o'
In contrast, transmission rates in Africa and Asia are higher,
t with herpes simplex, varicella zoster' cytomegalovirus, and
up to 50%. Postnatal transmission via breast feeding has been
> measles virus are often seen.
r found to be 15o/o.
IMMUNIZATION AND IN FECTIOUS DISEASES
Vertical transmission of HIV-I: HIV-1 may be transmitted to
the infant during gestation (in utero), during delivery (intrapar-
tum), or postpartum, through breast feeding. Potential routes of
infection include admixture of maternal fetal blood or infection
across the placenta antenatally or extensive mucocutaneous
exposure to maternal blood and vaginal secretions intranatally'
Breast milk acquired HIV-f infection: Tiansmission through
breast feeding seems to be related to the virus load in breast
milk as well as to the duration of time the child is fed. HIV-I
has been detected by culture and by the PCR method in cel-
lular and acellular components of breast milk. The colostral
viral load appears to be particularly high.
The risk of transmission to a recipient of HIV-I infected
blood is about95o/o.
CLINICAL FEATURES
Clinical manifestations are the result of multisystem involve-
ment associated with chronic persistent viral infection and
secondary immunodeficiency' Failure to thrive, unexplained
persistent fever, parotitis, persistent oral thrush, recurrent
gastroenteritis, otitis media, lymphadenopathy, hepatospleno-
megaly are the common early and mild signs and symptoms'
Lymphocytic interstitial pneumonia (LIP), organ specific
infections, and dysfunctions are dyspnea' interstitial pneu-
monia, repeated severe bacterial infection, loss of develop-
mental milestones, meningitis, encephalopathy, idiopathic
thrombocytopenia are commonly seen.
a Embryopathy, microcephaly, facial dysmorphism may occur'
a Recurrent bacterial infections are the result of defect in
humoral immunity. Any prolonged unexplained illness
should raise a suspicion of HIV-1 infection.
Neoplasms are relatively uncommon in pediatric HIV-I
infection. Non-Hodgkin lymphoma and primary CNS
lymphoma are most commonly reported malignant disease'
. Opfortunistic infections: Occur as CD4+ count declines
(see Thble 2I.7 for classification of severity of immunosup-
pression in relation to CD4 cell count). Pneumocystis jiroueci
ipreviously caIled Pneumuqtstit cdrinii pneumonia IPCPI)
pneumonia is the most common and iethal opportunistic
infection. Clinically, there is respiratory distress with cough
and hypoxemia. Chest x-ray reveals interstitial infiltrates or
diffuse alveolar disease. Diagnosis is made by demonstra-
tion of P carinii in bronchoalveolar fluid or lung biopsy'
Oral candidiasis is the most common fungal infection' It
may involve the esophagus' resulting in vomiting, fevet
dysphagia, and anorexia. Interstitial cryptosporidiosis may
lu.lare complex (MMC) has been recognized increasingly'
Opportunistic pathogens include Tbxoplasma gondii Myco-
bic|rrirm tuberculosis and malarial parasites. Viral infection
 ESSENCE OF PEDIATRICS

Table 21.7: Classification of Severity of lmnrunosuppression Table 21.8: WHO Clinical Sraging of HIV/AIDS for Chilclren
in Relation to CD4 Levels with Conl'irmed HIV lnfet rion

Asymptomatic
Persistent general ized lymphadenopathy

Not significant >35 >30 >25 >500

U nexplai ned persistent hepatosplenomegaly
Mild 30-35 25-30 20-25 350-499 Papular pruritic eruptions
Advanced 25-30 20-25 1 5-20 200-349 I-ungal nail iniection
Severe <25"k or <20nk or <15'k or <1 5% or
Arrgular cheilitis
<i 500/mmr <7So/mm) <350/mmr Lineal gingival erytherna
<200/mm-r
Extensive wart virus itrfection
lrtensive mollusr um t ontagiosum
Recurrent ora I ulceration
CLINICAL STAGING U nexplained persistent parotid enla:-gernent
Herpes zoster
Thble 21.8 summarizes WHOI clinical staging of HIV/AIDS
Recu.rrent or chronic upper respiratorl, ii-:ict infections (otitis
media,
for pediatric parienrs with confirmed HIV iniection. otorrhea, sinusitis, tonsillitis)

Unexplained moderate malnutrition or v,/asting not adequately

DIAGNOSIS
All infants born to HlV-infected morhers rest anribody-posiri\€
at birth_ because of passive rransfer of maternal HIV antibody
across rhe placenta during gesration. Most uninfeced infanis
lose marernal antibody berween 6 and 12 monrhs of age and
are known as seroreverters. Because a small propo.ttn of
uninfected infants conrinue ro tesr HIV antiboJy
fositive for
Lrp to 18.months of age, posirive IgG antibody rests, including
the rapid resrs, cannor be used to make a definitive diagnosil
of HIV infection in infants younger than this ag.. Th. pr.r_
ence of IgA or IgM anti-HIV in the infant's circulation
indicate HIV infection, because these immunoglobulin classes
clo not cross rhe placenta; howeve4 IgA and IgM anti_HIV
assays have been both insensitive and nonspecificand
therefore
are nor valuable for clinical use. In any child >1g rnonths of
age, demonsrrarion of IgG antibody r; HIV by a repeatedly
reactive enzyme immunoassay (EIA) and confirmatory resr
(immunoblot or immunofluorescence assay) establishes the
diagnosis of HIV inFecrion.
Viral diagnostic assays, such as HIV DNA- or RNA-pCR,
HIV culture, or HIV p24 antigen immune-diss ociated. p24
(ICD-p24), are considerably more useful in young infants,
allowing a definitive diagnosis in mosr infected infants by 1_6
months of age. By 4-6 monrhs of age, the HIV cuiture and/
or PCR identif, all infected infants. HIV DNA-pCR is the
preferred virologic assay in developed counrries (Fig. 21.3).
Aln'iost 40o/o of tnfected newborns have positive rest results in
the first 2 days of life, with >900/o testing positive by 2 rveeks
of age . Piasma HIV RNA assays, which detect viral replicatiolr,
nra;" i:rs more sensitive rhan DNA-PCR for early jiagnosis,
but data are limited. HIV cr_rlture has similar sensitir.irr. to
HIV DNA-PCR; hows1,6r, it is technicallv more cornol.x
responding to standard therapy
Unexplained persistent diarrhea (14 days or more)
Unexplained persistent fever (above 37.5. C intermittent or consrant
lor I morrthr
r Persistent oral candidiasis (aiterthe first 6_8 weeks of life)
. Oral hairy leukoplakia
. Acute necrotizing ulcerativc ging!vitis or periodontitis
. Lymph node or pulmonary tuberculosis
.
Sc\ ere rcL ulent bacleri,rl pneumonia
. Symptomatic lymphoid interstitial pneumonitis
. Chronic lung disease including bronchiectasis
. Unexplained anemia (<B g/dl), neutropenia (<0.5 x .l 0,r/L) or
chronic thrombocytopenia (<50 x l0ell)
can
Unexplained severe wasting, stunting, or severe malnutrition not
responding to,tand.rrd therapy
Pneumocystis pneumonia
Recurrent severe bacterial infections (e.g., empyema, pyornyositis,
' Ir
bone or joint infet tion. meningitisr
r Chronic herpes simplex infection (orolabial, cutaneous >l month
duration or visr eral dt any siiel
. Esophageal candidiasis (or candidiasis of trachea, bronchi, lungs)
. Extrapulmonary or disseminated TB
o Kaposi sarcoma
o Cytomegalovirus infection: Retinitis or CMV infection affecting
another organ, with on5et at age > I monlh
r Central nervous system toxoplasmosis (after 1 month of life)
o Extrapulmonary cryptococcosis (including meningitis)
. HIV enrephaloparhy
. Disseminated endemic mycosis (extrapulmonary htstoplasmosis,
cocc id io idomycosis)
. Disseminated non-ti:berculous rrvcobacterial infection
. Chronic cryptosporidiosis twith cliarrhea)
. Clrronic isosporiasis
. Cerebral or B-cell non-Hodgkin lymphoma
. Progressii.,e multifocal ieukoencephalopathy
. Svmptomatic HIV-associated nephropathy or l-llV-associated
t ard,omr opalhl

and expensive, and results are often not available fo, ,.uer"I L nexplained ref-^rs to rvhere the concljtion is not explained by
otherr causes.
weeks compared with 2_3 days for pCR. The p2l antigen ln cljnical stage.+, some addjtion.rl conditjons can also be inclLrdecl
in regional
classifjcations (e.g., in Asia-penicilliosis; in Anrericas region reactivation
assay is also highly specific and easy to perform, but it is less ot
Amcrican trypanosomiasis; and in Africa HlV,associ.rtecj rect"ovaginal iistulat.
 IMMUNIZATION AND INFECTIOUS DISEASES

* Delivery HIV-exposed infant

@
HIV+pregnantwoman $'-J-_.+
& (breastfed and non-breastfed)

Symptomatic
HIV-exposed in{ant
<18 months age
(not previously
diagnosed)

1"t HIV DNA PCR

Repeat HIV DNA

PCR to confirm

2"dPcR at
6 months to
confirm status

Repeat test and Report HIV Repeat test and

refer ior follow-uP negative refer for follow-uP

Fig.2l.3: HIV diagnosis in children below the age of 1B months with DNA-PCR.

It is not recommended
sensitive than the other virologic tests.
for diagnosis of infection in infants <1 month of age' In
developlng countries, theICD-p24 test may be considered for
older infants; however, if results are negative, it does not rule
out infection (Table 21.9).
\(orld Health Organization (WHO) criteria for diagnosis
of pediatric AIDS in developing countries:
Major criteria
o Weight loss or abnormally slow growth
o Chronic diarrhea for over 1 month
o Prolonged or intermittent pyrexia for over 1 rnonths
r
r Minor miteria
o Generalized lymphadenoPathY
r Oropharyngeal candidiasis
t r Recurrent common bacterial infections
II r Persistent cough for over 1 month
Table 2'1.9: Laboratory Diagnosis of HIV lnfection
HIV DNA-PCR Preferred test to diagnose HIV-1 subtype B infection
in infants and children younger than 1 8 months
of age; highly sensitive and specific by 2 wk of
age and available; performed on peripheral blood
mononuclear cells. False negatives can occur in
non-B subtype HIV-l infections
HIV p24 Ag Less sensitive, false-positive results during 1 mo of
life, variabie results; not recommended
ICD p24 Ag Negative test result does not rule out infection; not
rer ommended
HIV culture Expensive, not easily availabte, requires up to 4 lvk
to do tesL not recommended
HIV RNA.PCR Not recommended for routine testing of infants
and children younger than 1 B mo of age, because
a negative result cannot be used to exclude HIV
infection definitively. Preferred test to identify non-B
subtype HIV-1 infecLions.
Ag, antigen; lCD, immune complex dissociated; PCR, polvmerase chain reaction
 ESSENCE OF PEDIATRICS
o Generalized dermatitis
o Confirmed HIV infection in the mother.
Note:
The existence of two maior and nvo minor criteria in the
absence of other
known causes of immunodeficiency is diagnostic of AIDS.
TREATMENT
The management of HlV-infected child includes antirerrovirai
therapy, prophylaxis and rreatment of opportunistic infections
and common infections, adequate nutrition, and immunization.
Antiretroviral therapy: Decisions about antiretroviral therapy
for pediatric HiV-infected parients are based on the magnituie
of viral replication (i.e., viral load), CD4 lymphocyte .1.,r,, o,
percentage, and clinical condition. A child who has \X4IO
stage
3 or 4 clinical disease should receive ART irrespective of tf,e
immunologic stage. Children who are asympromatic or have
stage 1 or 2 disease may receive ART if they have evidence
of
advanced or severe immunosuppression.
The availability of antiretrovirai therapy has transformecl
HIV infection from a uniformly fatai condition to a chronic
infection, where children can iead a near normal life. The
currently available therapy does not eradicate the virus and
cure the child; itrarher suppresses rhe virus replication for
extended periods. The three main groups of drugs aie nucleoside
reverse rranscriptase inhibitors (NRTI), non-nucleoside reverse
transcriptase inhibitors (NNRTI), and protease inhibitors (pI).
Highly acrive anrirerroviral therapy (HAART) is a combination
of rwo NRTIs with a pI or an NNRTI.
Cotrimoxazole prophylaxis: In resource-limited serrings, corri_
moxazole prophylaxis is recommended for ail HlV-exposed
infants srarring at 4-6 weeks of age (or at first encounter wirh
the healthcare system) and continued until HIV infection can
be exciuded. Corrimoxazole is also recommended for HIV_
exposed breastfeeding children of any age, and corrimoxazole
prophyla-xis should be continued until HIV infection can
be
excluded by HiV antibody resring (beyond 1g months of age)
or virological testing (before l8 months of age) at leasr 6
after complere cessarion of breastfeeding.
.be All children younger than 1 year of age documented to
living with HIV should receive cotrimolxazole prophylaxis
regardless of symptoms or CD4 percentage. After t-year
of
age, initiation of cotrimoxazole prophyla_xis is recommended
for symptomatic children (VTIO .linic"l srages 2, 3, or 4
for HIV disease) or children with CD4 <25o/o. All children
who begin cotrimoxazole prophylaxis (irrespective of whether
cotrimoxazole was initiated in the first year of life or
that) should continue until the age of 5 years, when they can
be reassessed.
Nutrition: It is important to provide adequate nutrition to
HlV-infected children. Many of these children have failure to
thrive. These children will need nutritional rehabilitation. In
addition, micronurrients like zinc may be useful.
Immunization: The vaccines that are recommended in the
national schedule can be administered to HlV_infectecl children
except rhar sympromaric HlV-infected chilclren should
nor be
given OPV and BCG.
Counseling and support: l)ue to the stigma attached to the
diagnosis of HIV families often feel socialiy and culturally iso_
lated. Illness and unemploymenr can creat; financi.l probl.-r.
It is important to establish links with support agencies in the
communiry, whiie respecting the familyt need for confidendaliry.
A multidisciplinary team of health professionals, who can deliver
co-ordinated care in a family oriented and child centered manne!
is necessary for the supporr of the family afliicted with AIDS.
HW vaccine: Still on trial.
PREVENTION
Prevenlion of Molher lo Child Tronsmission
(MTcT)
The risk of MTCT can be reduced rc <2o/o by interventions
that include antiretroviral (ARV) prophylaxis given ro women
during pregnancy and labor and to the infant in the first weeks
of life, obsterrical interventions includ.ing elective cesarean
delivery (prior to the onset of labor
.upt-.rr. of membranes),
"rrd
and complete avoidance of breastfeecling.
Breastfeeding: Exclusive breastfeeding has been reported to
catry a lower risk of HIV rransmission than mixej feeding.
According ro currenr UN recommendations (\(.HO, 200i)
when replacement feeding is acceptable,
feasibh, ffirtlable, sus,_
tainable and safe, avoidance of all breastfeeding by HlV-infected
mothers is recommended. otherwise, excrusive breasfeecling
is
recommended during the first monrhs of life. WT{O recom_
mends that the transition between exclusive breastfeeding
and
early cessation of breastfeeding should be kept as short as'pos_
sible, 'early and abrupt cessarion' bearing in mincl that mixed
feeding during this period carries a 70o/ofreater risk of MTCT.
*"ek,
Other Woys of prevenlion
o Avoid unsafe sex (condom should be used during coitus),
avoid pregnancy by the mother suffering from AIDS.
o Inform the uninformed about the nature, transmission, trear,
ment, and outcome of the disease. Immunization_vaccine
is expected to be developed within a couple of years.
o Drug abuse should be stopped. Blood and bloocl products
should be screened for HIV
after
o Syringes should be disposable. Contaminated syringes,
needles, khur or/and blade (in saloon) mlrst be avoideJ.
Severe acure respiratory syndrome (SARS) is a clinical illness
characterized by features of atypical pneumonia with rapid
1
t
I

deterioration of pulmonary function, rapid nosocomial spread,
and have a pandemic potential.
ETIOTOGY
SARS is caused by an agent linked to corona virus. First noticed
in Guangdong province, China on November 16, 2002. Then
SARS spread to Hong Kong through a visitor, then worldwide'
\fHO official Dr. Carto Urbani reported an unusual pneu-
monia in Hanoi on February 26' 2003.
SARS has become a global health hazard. SARS is very
alarming, because (l) infectivity is very high' (ii) health care
personnel are vulnerable, (iii) management is not optimized,
and (iu) mortaiiry rate is around 5%o'
MODE OF TRANSMISSION
o Tiansmission occurs either directly by contact between
persons or indirectly via contaminated surfaces (fomites).
o Tiansmission by droplets containing microorganisms (gen-
erated by sneezing, coughing, talking and suctioning) are
propelled to a short distance (1-2 meters) through air and
infect via eyes, nose, or mouth.
o Airborne transmission (rare), though airborne dropiet nuclei
or dust particles remain suspended in the air.
CtINICAt FEATURES
Incubation period is 2_16 days. Symptoms are fever (100%)'
chills and rigor (73o/o), myalgia (610/o), non-productive cough,
headache (50o/o), dizziness (43o/o), dyspnea, sputum produc-
tion (29o/o), sore throat (23o/o), nausea and vomiting (20o/o).
Physical examination reveals dullness on percussion and crackles
on auscultation.
DIAGNOSTIC CRITERIA AND INVESTIGATIONS
Diagnostic criteria (CDC, Atlanta, 2OO3):
o High fever >38' C (100.4' F)
o Cough and shortness of breath
o CXR or CT Scan of lung: Consolidation
o H/O exposure to a suspected SARS patient
Investigations:
CBC shows leukopenia (3 4o/o), lymphopenia (7 0o/o) and throm-
bocytopenia (45Vo). Elevated lactate dehydrogenase (71o/o),
elevated creatine kinase (56%), mildly elevated aminotrans-
ferases (78olo), and hyponatremia are common.
Plain chest x-ray: Consolidation with predominant periph-
eral involvement. CT scan of lung (may be positive when plain
x-ray chest is normal) may show ground glass opacification in
peripheral lung parenchyma.
TREATMENT
o No specific treatment. Patient should be isolated.
I
IMMUNIZATION AND INFECTIOUS DISEASES
o O, inhalation, when needed.
r Antimicrobial agents: \&'HO has suggested that antimi-
crobial agents should be given in line with treatment of
community acquired pneumonia, plus antiviral treatment
(i.e., Ribavirin). Though efficacy of antibiotics and ribavirin
had not been proven.
o ICU care (23o/o).
o Avoid: Nebulization, chest physiotherapy, bronchoscopy,
gastroscopy.
PROGNOSIS
907o improve by day 5 or 6. About 100/o develop severe respira-
tory illness; mortality is -50lo. Dearh occurs due to progressive
respiratory failure.
PREVENTION
a High index of suspicion.
a
.
Isolation and barrier nursing.
a Use of personal protective equipment (PPE); wearing sci-
entific masks, gloves, and sun glass'
a Masking of patients to reduce respiratory spread.
a \Tashing hands, mouth with antiseptics. Mucous membranes
should not be touched with unwashed hands.
a Keep patient quarantined.
a Strict vigilance on airport and land of entry of SARS cases.
It is an acute infection characterized by fever, sore throat,
lymphadenopathy, splenomegaly, atypical lymphocytosis, and
the presence of heterophile antibody. Infectious mononucieosis
most often affects adolescents and young adults.
Infectious mononucleosis caused by Epstein-Barr virus A,
herpes virus, cytomegalovirus and T gondii cause illnesses that
are virtually indistinguishable from Epstein-Barr virus induced
mononucleosis. Transmitted by droplet infection, sexual inter-
course, or blood transfusion.
CTINICAT FEATURES
Clinical features of infectious mononucleosis are highly variable.
Symptoms are usually less severe in younger children than in
oider children, adolescents, or adults. A prodrome of maiaise,
fever, and headache may prevail for 3-7 days before the onset
of more profound symptoms.
o Fever is invariably present and may last as long as 21 days.
Temperature may reach as high as 104" F (40' C).
r Pharyngitis occurs in approximately B0% of patients and
is exudative.
o Lymphadenopathy is usually generalized and most often
involves the cervical nodes. Other anatomic sites may also
be afrected.

ESSENCE OF PEDIATRICS
r Splenomegaly is noted in most patients who have infectious
mononucleosis; rupture is rare, occurs following significant
trauma.
o Rash occurs in approximately 5o/o of parients and may be
macular, petechial, urticarial, or erythema multiforme-like.
Administration of ampicillin ro patienrs who have infectious
mononucleosis produces a rash in 90-100% patients, which
is pruritic and maculopapular and may appear afrer cessa-
tion of antibiotic trearment.
r Other clinical findings include fatigue, eyelid edema, abdom-
inal pain, and rarely, jaundice.
DIAGNOSIS
Suggestive laboratory tests include a predominance of mono-
nuclear cells on CBC (>50% mononuclear cells), >10% atypi-
cal lymphocytes, and elevated levels of serum rransaminases.
Laboratory confirmation consisrs of positive serologic findings.
Several serologic tesrs have been developed:
a. The Paul-Bunnell-Davidsohn test is an extension of
the classic Paul Bunnell resr for the heterophil antibody
characteristic of infectious mononucleosis. Heterophil
antibodies (non-specific antibodies) can reacr wirh antigens
that are different from the antigens that induced their
production.
b. Commercial heterophil antibody kits (e.g., Monospot
test) are simple, rapid, and fairly sensitive. The test resuits
are usually positive within the first week of infection and
remain positive for several months. However, only B0%
of patients with Epstein Barr virus infection have positive
test results.
c. Antibodies to Epstein-Barr virus: Patients who have
infectious mononucleosis produce antibodies to various
specific antigens, including Epstein-Barr viral capsid
antigen (VCA), Epstein-Barr nuclear antigen (EBNA),
and Epstein*Barr virus induced early antigen (EA).
i. Antibodies to VCA initially immunoglobulin M
(IgM), followed by IgG peak in the second or rhird
week of illness and persist for life.
ii. Antibodies to EA appear early in rhe course of illness
and disappear 2-6 months later.
iii. Antibodies to EBNA appear 3-4 weeks after the onset
of infection and probably persist for life.
TREATMENT
In most cases, rest is the only treatment that is necessary;
there is no specific drug therapy for infectious mononucleosis
except paracetamol for fever. Convalescence may take weeks to
months and is relatively shorter in younger p",i..rr, compared
with older patienrs.
Corticosteroids are generaliy used in parienrs wirh
impending airway obstruction, (ii) severe thrombocytopenia,
(z)
and (iii) hemolytic anemia.
I
coMPUCAT|ONS
Splenic rupture, due either ro trauma or spontaneous occur-
rence, is a rare complication.
Airway obstruction due to tonsillar or pharyngeal hyper-
trophy is also rare. Jieatment with corticosteroids is often
e{fective.
Neurologic complications are usually self-limited and revers-
ible and include aseptic meningitis, encephalids, myelitis,
peripheral neuropathies, and Guillain-Barre syndrome.
Corticosteroids may be effecdve.
Icteric hepatitis occurs in approximady 5o/o of patients,
whereas subclinical heparitis occurs in approximately
20-40o/o.
Other rare complications include autoimmune hemolytic
anemia, thrombocytopenia, acute renal failure, complete
heart block, pericarditis, pneumonia.
Varicella and zosrer are two different infectious diseases caused
by varicella-zosrer virus.
Varicella (chickenpox) is a highly contagious disease, occurring
primarily in children younger than l0 years of age. Usually, it
is a mild, self-limited disease in otherwise healthy children, but
it may be a severe or even fatal illness in immunocompromised
children. A varicella vaccine has been shown to decrease the
risk of disease.
Zoster (shingles) represents a reacrivation ofvaricella infection,
occurring predominantly in adults who previously had varicella
and who have circulating antibodies. Although zoster occurs
in children, it is uncommon in those younger than l0 years
of age. Zoster is an acute infection characterized by crops of
vesicles confined ro a dermatome and often accompanied by
pain in the affected dermatome.
CtINICAI FEATURES
Vqricello
1 After an incubation period ranging from 10 to 2l days,
a prodrome begins that consisrs of mild feveq malaise,
anorexia, and occasionally, morbilliform rash.
2. The characteristic pruritic rash begins the day following
the start of the prodrome, appearing first on the trunk
and spreading peripherally.
(a) The rash begins as red papuies and develops rapidly
into clear "teardrop" vesicles that are approximately
1-2 mm in diameter on an erythemarous base. The
vesicles become cloudy breaking down into thin ulcer-
ative lesions that crust before healing.
(b) The lesions occur in widely scattered "crops" so that
several stages of the lesions are usually presenr at the
I
same time. Vesicles may occur on mucous membranes.
1
tt

). The severiry of the illness ranges from a few lesions
associated with a low-grade fever to hundreds of lesions
associated with temperatures up to 105' F to fatal dis-
seminated disease in immunocompromised children. In
most children, varicella manifests as a generalized rash
with mild fever and mild systemic symptoms.
4. Infectious period. Patients are infectious beginning approx-
imately 24 hours before the appearance of the rash until
all lesions are crusted, which usually occurs 5-7 days after
the onset of the rash.
Zosler
1. Attacks of zoster may begin with pain along the affected
t
sensory nerve that is accompanied by fever and malaise.
2. A vesicular eruption simiiar to the vesicular form of vari-
cella then appears in a dermatome area; in most cases,
this eruption clears in 7-I4 days. However, the rash may
last as long as 4 weeks.
1. The lesions are infectious if there is either direct contact
or a susceptible individual inhales aerosolized infected
epithelial cells.
4. Post-zoster neuralgia is common in the elderly, but rarely
occurs in children.
DIAGNOSIS
Diagnosis of both variceila and zoster is usually obvious from
the clinical presentation.
o If the diagnosis is unclear, aTzanck test or varicella fuores-
cent antibody test should be performed early in the course
ofillness on scrapings taken from the base ofa vesicle' The
demonstration of multinucleated giant cells with intranuclear
inciusions indicates varicella zoster, or herpes simpiex infec-
tion. On the other hand, the varicella fluorescent antibody
test specifically identifies the varicella virus.
r The definitive diagnosis is made by positive culture result
from a vesicular scraping or demonstration of a four-fold
rise in antibody titer between acute and convalescent sera.
TREATMENT
Uncomplicated cases of varicella or zoster are treated with
an antipruritic medication and daily bathing to reduce
secondary bacterial infection.
Immunocompromised children (e.g., those with AIDS or
leukemia and those receiving immunosuppressive drugs)
who have not had varicella and have been exposed to
someone with the disease should receive prophylaxis
with varicella zoster immunoglobulin within 96 hours
of exposure. Immunocompromised patients who develop
varicella or disseminated zoster should be treated with
intravenous acyclovir (250 mglm2 IV 8 hourly over 1
hour for 10 days).
IMMUNIZATION AND INFECTIOUS DISEASES
o Oral acyclovir (20 mg/kg 6 hourly for 5 day$ is given to
adolescents and adults with varicella, because they have
increased risk of serious disease.
Prevention is effectively achieved through the use ofvaricella
vaccine, i.e., varilrix.
Post-exposure prophylaxis by YZ immunoglobulin is given
in high-risk suscepdble individuals, i.e., immunocompromised
children, newborn whose mother deveiops varicella 5 days prior
to delivery and within 48 hour after delivery. VZ immuno-
globulin should be given within 96 hour of exposure. Dore.'
Newborn infants, 125 units IM. Children, 125 units/10 kg
body weight IM.
coMPUCATTONS
o The most common complications of a varicella-zoster infec-
tion include encephalopathy, cerebellitis, Guillain-Barre
syndrome, aseptic meningitis, pneumonia, Reye syndrome,
hepatitis, thrombocytopenic purpura, purpura fulminans,
cellulitis, formation, and arthritis. Varicella is an
abscess
important risk factor for severe, invasive group A B-hemolytic
streptococcal disease.
Progressive varicella (with meningoencephalitis, pneumonia,
and hepatitis) occurs in immunocompromised children and
is associated with a mortality rate of approximately 20o/o.
Maternal varicella during the first trimester may be associ-
ated with congenital malformation (limb anomaly, cortical
atrophy) in 7-2o/o of patients.
Scarlet fever is an acute illness characterized by fever, pharyn-
gitis, and an er)'thematous rash. Scarlet fever is rare in infancy.
It can occur more than once in a single patient.
Scarlet fever results from infection with group A strepto-
coccal strains that produce erythrogenic toxin. The disease is
usually associated with pharyngeal infections but, in rare cases,
follows streptococcal infections at other sites (e.g., wound
infections, impetigo).
CLINICAT FEATURES
The characteristic rash is erythematous, and blanches with
pressure. It appears initially on the trunk and becomes
generalized within a few hours to several days. The face
is flushed with circumoral pallor, and there is increased
erythema in the skin folds (Pastias lines). The skin may
feel rough, similar to sandpaper. The skin rash fades over
1 week and is followed by desquamation, which may last
for several weeks.
A strawberry tongue (rough, erlthematous, swollen tongue)
and pharyngeal erythema with exudate may be present'
 ESSENCE OF PEDIATRICS
Diagnosis is made on rhe basis of the clinical presenrarion
and the isolation of group A streptococci on throat culture.
TREATMENT
Therapy for scarler fever is the same as rhat for streptococcal
pharyngitis consisting of 10 days of orally administered peni,
cillin or single dose of benzathine penicillin G.
coMPHCAT|ONS
Both suppurative (e.g., cellulitis) and nonsuppurative (e.g.,
glomerulonephritis, rheumatic fever) complications can occur
with scarlet fever just as with streptococcal pharyngitis.
Gingivitis and stomatitis refer to inflammatory disease of the
gingivae (gums) and oral mucosa, respecrively. Combined
inflammation of the gingivae and oral mucosa is termed gin,
givostomatitis.
N ECROTIZING UTCERATIVE GI NGIVITIS
(vtNcENT'S DTSEASE)
It consists of necrosis and ulceration of the interdental papil-
lae. This infection is most common in adults but may also
occur in children.
Etiologl,,:
o Necrotizing ulcerative gingivitis results from a decreased
resistance of the gingivae to infection by normal oral flora.
Subgingival plaque is present in large amounrs and consists
of a mixture of fusiform bacilli and spirochetes.
o The infection begins in an area of the gum that is in contacr
with plaque (the interdental papillae) and results in the
punched out, eroded papillae and purulent, gray membrane
characteristically seen in these patients.
Clinical features include gingival pain, fever, malaise, and
foul-smelling breath.
Treatment:
o Oral irrigation with oxidizing agenrs relieves the pain associ-
ated with the infection.
o Antimicrobial therapy (usually penicillin G or amoxicillin)
is effective against the infection and along with oral irriga-
tion by oralon or 1%o povidone mourh wash usually brings
prompt relief.
APHTHOUS STOMATITIS
It is a common
and often recurrent oral mucosal iesion; it
consists of circular shallow ulcers that are painful and may
occur any'where on rhe oral mucosa, lips, particularly the freely
movable (buccal) mucosa. The lesions may occur single or in
clusters, are covered by a gray membrane and are surrounded
by a raised border of infammation. No fever, no lymphade-
nopathy is remained associated.
Etiology: The exact cause is nor known, may be ailergic or
autoimmune.
Tieatment: Aphthous sromariris is a selflimited infection that
heals in 1-2 weeks without rrearment, but tends to recur in
susceptible individuals.
Symptomatic therapy with saline mouthwash may be helpful
in patients who have mild symptoms.
Coating the lesion with topical antacid or sucralfate
4 times daily.
Topical corticosteroid either in a dental paste, i.e., triamcino-
lone (kenalog in orobase) or in a mourhwash 4 times daily.
Paracetamol for pain.
H ERPETIC GI NGIVOSTOMATITIS
This condition is the mosr common rype of gingivosromariris in
children. The first infection (primary infection) usually occurs
within the first 5 years of life. Most cases are caused byherpes
simplex virus type 1 rather than type 2.
Pathogenesis:
o Primary infection aflects the mouth and gums, whereas
recurrent disease usually affects the junction of the lip and
skin (herpes labialis) and is less severe rhan the primary
infection.
o Recurrent herpes labialis may be precipitated by emotional
stress, exposure ro the sun, or febrile illness (e.g., viral upper
respiratory rract infecrion, pneumonia, meningitis). There-
fore, these lesions are also called sun blisters or fever blisters.
Clinical features: Primary herpetic gingivostomatitis causes
painful, erythematous, edematous, and ulcerative lesions (>10
ulcers 1-3 mm) on the buccal mucosa, gums, and sometimes,
hard palate, pillars, and tongue. Fever is usually present often
to a temperature of 105" F. Lymph nodes are enlarged. The
infection occurs afrer a 3_9 day incubation period, improves
after 3-5 days, and usually resolves within 2 weeks.
teatment: Cold foods (e.g., ice cream) and oral fluids should
be given. Viscous xylocaine (2o/o) canprovide some pain relief.
The condition may be severe enough that a child refuses to eat
or drink and requires inrravenous rehydration in the hospital.
Orally administered acyclovir (10 mg/kg PO three times
daily for 7 days) may decrease the severity of illness if started
within 2448 hours after onset.
HERPANGINA :
Although herpangina has been considered a specific febrile \
disease, the term is more appropriateiy used to refer to the
t,

characteristic oropharyngeal lesions that are one ofthe protean
manifestations of enteroviral infections.
Coxsackieviruses (types A and B) and echoviruses are the
causative agents. Herpangina occurs almost exclusively in the
summer and fall, when enteroviruses are prevalent.
Clinical features:
o Fever, sore throat, and pain on swallowing are the hallmarks
of herpangina. The fever is of sudden onset and may rise to
106' F. Headache, myalgia, and vomiting may also occur
at the onset of the illness.
o The characteristic lesions are 1_2 mm vesicles and ulcers
surrounded by an erythematous ring measuring up to 10
mm in diameter. The lesions occur in the posterior pharynx,
including the anterior tonsillar pillars, soft palate, uvula'
tonsils, and pharyngeal wall.
r The fever subsides in 2-4 days, but the ulcers may persist
for a period of up to 1 week.
Tireatment: No treatment is necessary other than preven-
tion of dehydration and observation for signs of more severe
enteroviral illness.
CAN DI DAt GI NGIVOSTOMATITIS
Thrush is the term used to describe gingivostomatitis due to
infection by Candida albicans.
Candidal gingivostomatitis is common in newborns. The
condition usually clears by 3 months of age except in severely
debilitated infants. Oral antibiotic therapy may predispose an
individual to thrush. \,X4ren candidal gingivostomatitis occurs
after the first year of life, a defect of cell-mediated immunity
should be considered.
Clinical features: Grayish white lesions occur on the buccal
mucosa and dorsum of the tongue. Occasionally, the gingival
mucosa and posterior pharynx may be involved. If a scrap-
ing from the affected area is Gram-stained, both yeast forms
and pseudohyphae are seen. Culture on blood agar will yield
Candida sp organisms.
teatment It consists of administering 1 ml of nystatin suspen-
sion orally four times daily for 1 week. Miconazole (or gentian
vioiet) gel can be used. Large plaques should be removed by
a piece of gauze.
It refers to inflammation and enlargement of the lymph nodes
of the neck. Swollen and tender cervical lymph nodes are
common in children. In many cases, the illness is self-limited
(as in cervical adenitis associated with a viral infection);
howeveq in other cases the illness requires prompt and specific
treatment, such as cervical adenitis due to S. aureus or group
A streptococci.
IMMUNIZATION AND INFECTIOUS DISEASES
ETIOTOGY
Most cases are related either to bacterial infection of the oral
cavity or other areas of the head and neck (e.g., streptococcal
pharyngitis) or to viral upper respiratory tract infections.
a. Common agents: The most frequently identified bacterial
agents of childhood cervical adenitis are S. aureus and
group A streptococci, anaerobic bacteria, either alone or
in combination.
b. Less common agents:
i) Cat-scratch disease primarily affects children and is
an important cause of cervical adenitis. The causative
organism has been identified as Bartonella henselae.
tansmission is usually by a car scratch; occasionally,
the skin injury results from the scratch of a dog or
other animal, a splinter, or a thorn.
ii) Several species of atypical mycobacteria cause cervi-
cal adenitis in infants and young children. The most
common of these arc Mlcobacterium scrofulaceum and
Myc o b acterium au ium i nnac e l lu lare.
iii) Other agents of childhood cervical adenitis include:
Epstein*Barr virus, measles, rubella, cytomegalovirus;
M. tuberculosis, T. gondii.
CtINICAI FEATURES
o Typically, a child who has cervical adenitis is initially seen
with swollen, tender nodes in a single location of the
neck. Bilateral involvement suggests a non-specific or viral
infection, which usually resolves spontaneously. Unilateral
involvement with nodes that are more severely swollen (3-6
cm in diameter), tender, and warm suggests a pyogenic
infection. Low-grade fever is an inconsistent finding.
o In cat-scratch disease, which is unilateral, the involved nodes
may be quite large; in |0-25o/o of cases, these nodes are
suppurative. Low-grade fever and a transient maculopapular
rash may also be noted.
o In atypical mycobacterial infection, the cervical adenitis
usually involves the submandibular or submaxillary nodes,
is unilateral, and runs an indolent course. Fever and other
systemic signs are usually absent.
DIAGNOSIS
A. Medical history and physical examination: The medical
history of a child who has cervicai adenitis should include
information concerning the duration of the lymphade-
nopathy, recent upper respiratory tract infections, contact
with pets (especially cats), and exposure to individuals
who have tuberculosis. All node sites should be examined,
with dimensions noted. Liver and spleen size should also
be noted.
Specific diagnosis ofthe cause ofcervical adenitis is usually
not arrempted if the child has only slightly enlarged and
 ESSENCE OF PEDIATRICS
minimally tender lymph nodes. A diagnostic work-up is
performed if (l) the child has moderate fever and sysremic
symproms when first examined, (ii) a Iarge (>3 cm) or
fluctuant node is found, (iii) frndings suggest an unusual
cause, or (izr) empiric antibiotic therapy has failed.
1. Needle aspiration, incision and drainage, or excision
and biopsy are the most direct nierhods for idendfring
the cause of cervical adenitis. The aspirated material
should be prepared fcr Gramt stain, acid-fasr stain,
and culture.
2. BCG or MT test
3. Serologic tests may help to identif' viruses (e.g.,
Epstein-Barr virus); bacteria and protozoa (T gondii).
4. Other diagnosric rests include:
a) Gramt stain and culture of any primary focus of
infection
b) Blood culture
c) Complete blood count
d) Chest radiograph
TREATMENT
a. Cervical adenitis that is characterized by only slight
enlargement (<3 cm) and minimal tenderness of the
lymph nodes is closely observed but otherwise untreated.
b. Cervical adenitis that is characterized by more severe
enlargement and tenderness is usually treated first with
empiric antibiodc therapy for 10-14 days. The preferred
agents are penicillinase-resistant penicillins (e.g., cloxacil-
lin, amoxicillin-clavulanate or cephalexin).
c. If there is poor response to empiric therapy, a diagnostic
work-up is performed that may include needle aspiration.
If a specific etiologic agenr is determined, appropriate
therapy is as follows:
i) Cervical adenitis due to .9. Aurex{; or group A strepro-
cocci is treated with an oral antistaphylococcal agent
or oral penicillin, respectively. Excision and drainage
may be necessary in severe cases.
ii) Cervicai adenitis associated with cat-scratch disease
is usually selfiimited and requires only analgesics.
Antibiotic therapy may be considered in more severe
cases and might include trimethoprim-sulfamethoxazole
given orally or gentamicin given parenteraliy, although
efficacy of antibiotic therapy has not been demonstrated.
iii) Cervical adenitis due to atypical mycobacteria is treated
with excision of infected nodes. Antituberculous drug
therapy alone is usually unsuccessful, although it may
be used in addition to surgical excision.
Pertussis is an acute infection ofthe respirarory rracr caused by
Bordetella pertussis or Bordetella parapertusis. Similar symproms
are sometimes produced by advanced virus infection. It is
highly communicable.
CTINICAT FEATURES
Incubation period is 7_15 days.
o Catarhal phase: Lasts for 7-I0 day.It is the mosr infecrious
period. The initial manifestations are indistinguishable from
UMIs. Here cough does not improve in a few days, but
becomes more severe and frequent with the passage of time.
o Parorysmal phase Lasts for 2-4 weeks. There is a rapid
succession of cough coming in an explosive manner, occurs
during expiration, the bout of cough rerminates during
inspiration with a "whoop". The "whoop" is produced by
the air rushing in during inspiration through the half-open
glottis. The 'whoop" may not always be presenr in infants.
During bout, child may appear choked, is unable to breathe,
looks anxious, and has a suffused face. Subconjunctival
hemorrhage and ulceration of frenulum of the tongue may
be present.
o Convalescent phase: Lasts for 2-3 weeks. Interval between
paroxysm of cough increases and severity decreases. Vomit-
ing becomes less frequent and severity decreases. Appetite
and general condition gradually improve.
DIAGNOSIS
Suspect pertussis if
intense cough with paroxysms >2 weeks
with posttussive if there is no Fever, sore
emesis, especially
throat, exanthem, enanthem, tachypnea, rales.
INVESTIGATIONS
r CBC: Leukocytosis (15,000-100,000 ceils/mmr) with abso,
lute lymphocytosis is characteristic.
r X-ray chest: Atelactasis with perihilar infiltrate.
o DFA (direct fluorescent antibody) resring for B. pertussis of
nasopharyngeal secretion.
DIFFERENTIAT DIAGNOSIS
Adenovirus infection: Like pertussis but with fever, sore
throat. and conjuncriviris.
Mycoplasma: Occurs in school age with fever, headache,
systemic symproms, cfeps.
Bacterial pneumonia: High fever, toxicity with consolida-
tion on chest x-ray.
TREATMENT
o Infants younger than 3 monrhs, infants between 3 and 6
;
months with severe paroxysms, and children with compli- I
carions should be hospitalized for nursing, feeding, and
t
observation. \
I

Erythromycin 40-50 mglkgld in four divided doses for 14
days, or clarithromycin 10 mg/kg/d in two divided doses
for 7 days, or Azithromycin 10 mg/kg/d once a day for 5
days should be given. Co-trimoxazole is modestly effective,
but first- and second-generation cephalosporins are not.
Antispasmodics, antitussives, sedatives are of no proven
value.
a Steroids and salbutamol may help.
O Household and close contacts should be treated with
erythromycin for 14 days. Children should be isolated for
5 days after initiation of erythromycin therapy.
PREVENTION
80% of exposed are protected by three doses of DPT.
Acellular vaccine (DTaP) is recommended in USA, Japan.
Pertussis immune globulin has been used in children younger
than2year (7.2 ml for 3-5 doses).
coMPHCATTONS
Pneumonia (25o/o), seizure (4o/o), encephalopathy (10lo). The
principal complications of pertussis are apnea, secondary infec-
tions, pulmonary hemorrhage, and conjunctival hemorrhages.
t Petechiae, epistaxis, hemorrhage in the central nervous system
(CNS) and retina, pneumothorax and subcutaneous emphy-
sema, and umbilical and inguinal hernias, laceration of the
lingual frenulum, rectal prolapse, dehydration and malnutrition
IY. following posttussive vomiting. Tetany has been associated with
I
L
profound posttussive alkalosis.
I 100,000 U should be given ll2IM and ll2IV as a single
v
V dose. If sensitivity to AIS is demonstrated, desensitization
Tetanus is an acute toxemic illness due to soluble exotoxin
t liberated by vegetative forms of Cl. tetani and is characterized
f"
by painful spasm and stiffness of muscles.
t,
Spores of Cl. tetani germinate in wounds or cut surface
t under anaerobic environment and liberates exotoxin, which is
transported from peripheral nerves to motor neurons of spinal
I cord by retrograde axonal transport.
Toxin acts on spinal cord, brain stem, peripheral nerves,
neuromuscular junction, autonomic nervous system, but prob-
ably spares cerebral cortex.
CTINICAL FEATURES
o Localized tetanus: Painful spasm of muscles adjacent to
wound site and may precede generalized tetanus.
o Generalized tetanus follows 2-74 days (even months)
after injury (in 30o/o cases no portal of entry apparent).
Tlismus (lock jaw, i.e., the inability to open mouth fully) is
often the first symptom. Pain and rigidity at site of injury,
headache, stiffness, generalized rigidity, and opisthotonos
ma\- occlrr. Painful spasms occur in conscious state in
IMMUNIZATION AND INFECTIOUS DISEASES
response to minor stimuli such as noise, touch, medical
nursing procedure, and may lead to laryngeal obstruction.
Autonomic disturbances such as urinary retention, forced
defecation, tachycardia, arrhythmias, labile hypertension,
and diaphoresis may occur. Spasms are prominent in first
2 weeks followed by autonomic disturbance, which reaches
peak during second week. Rigidiry lasts longer.
Neonatal tetanus: Typically manifests in neonate within
3-12 days of birth as progressive difficulty in feeding, stiff
abdomen, body/limbs; lockjaw (masseter spasm), spasms
with or without opisthotonos.
Cephalic tetanus follows wound of face, nostrils, head,
and chronic otitis media. Characterized by retracted eyelids,
deviated gaze, trismus, risus sardonicus, spastic paralysis of
tongue and pharynx. Cephalic tetanus is associated with
higher mortaliry. Death occurs from aspiration pneumonia,
exhaustion, laryngeal obstruction, respiratory arrest, cardiac
arrhythmia.
DI FFERENTIAL DIAGNOSIS
Meningitis encephalitis, rabies, tetany, drug-induced dystonia,
hysteria.
TREATMENT
e Tetanus immune globuiin (TIG) 500 U IM as soon as
possible (better to give before tetanospasmin gets fixed to
the neural tissue).
IfTIG is not available and skin testing shows no hypersen-
sitivity reaction, injection tetanus antitoxin (ATS), 50,000-
should be done.
Antibiotics: Injection C-penicillin 100,000 units/kg/d in
four divided doses for 10-14 davs. Metronidazole 500 mg
IV or PR given 8 hourly is effective. Erythromycin and
tetracycline (>8 years old child) are alternatives for penicil-
lin allergic patients.
Seizure control: Diazepam provides both relaxation and
seizure control; the initial dose of 0.1-0.2 mg/kg every
3-6 hours given IV (or >0.5 mg/kg PR) is titrated to
control the tetanic spasms. After which it is sustained for
2-6 weeks before it is tapered. Inj. MgSO' midazolam,
and chlorpromazine are also used. Neuromuscular blocking
agent such as vecuronium and pancuronium are used when
facilities of mechanical ventilation are availabie.
Pyridoxine 100 mg/d (increase GABA concentration) can
also be used.
Supportive treatment: Meticulous nursing care is imperative.
The patient should be placed isoiated in a quiet environ-
ment and every effort made to control or eliminate auditory
and visuai stimuli. A respirator, oxygen, suction apparatus,
and equipment for tracheostomy (in laryngospasm) should

ESSENCE OF PEDIATRICS
be available. An adequate intake of fluid, electrolytes and
calories should be maintained. For this, nasogastric feeding
or intravenous alimentation is required. \7ound should be
cleansed and debrided, if necessary. Foreign bodies musr be
removed and the wound left open.
o Umbilical stump or wound shouid be taken care of.
coMPUCAT|ONS
The following complications should be looked for and rreated:
Aspiration pneumonia, atelactasis, mediastinal emphysema,
pneumothorax, respiratory muscle spasmJ laryngospasm,
respiratory obstruction, cyanosis and asphp<ia, cardiac
arrhythmia, unstable BP, venous thrombosis, pulmonary
embolism.
Lacerations of the tongue, buccal
mucosa, gastric ulcer,
intramuscular hematomas, myoglobinuria, renal failure,
and vertebral fracture.
a Hyperhidrosis, paraly'tic ulcer, decubitus ulcer.
a If the course is prolonged, dehydration and PEM may
develop.
PREVENTION
Active immunization againsr retanus has been mentioned earlier
in the immunization schedule.
Diphtheria is an acute toxic infection caused 6y Corynebacte-
rium diphtheriae.
CtINICAI FEATURES
After incubation period of 1-6 days, signs and symptoms
develop according to the site of infection:
Nasal: Serosanguinous, purulent, erosive rhinitis with white
membrane formation. Shallow ulceration of external nares
and upper lip is characteristic.
Tonsillar/pharyngeal: Sore throat in all but low-grade
fever, dysphagia, hoarseness of voice in some cases.
Bilateral or unilateral tonsillar membrane extends to
uvula, soft palate, posrerior oropharynx, hypopharynx,
larynx; membrane is grayish white and is difficult to
remove. tWhen membrane is removed, raw bleeding spots
are seen. Soft tissue edema and lymphadenopathy can
cause bull neck appearance.
o Laryngeal: Noisy breathing, stridor, hoarseness; may be fatal
due to laryngeal obstruction caused by necrotic membrane,
o
edema oF soft tissues.
Other forms are curaneous, vaginal. \found diphtheria
is characterized by ulcerative lesions with membrane
formation.
I
DIFFERENTIAT DIAGNOSIS
a Nasal snuffles
a Streptococcal tonsillitis: Membrane limited to tonsils, easily
removable; high fever.
a I n Fectious mononucleosis
a Candidiasis
o Acute laryngotracheobronchitis
a Acute epiglottitis
o Foreign body
DIAGNOSIS
o Microscopic examination afrer Gram staining or A]bert
stain of material
o Culture: Positive for bacillus
COMPLICATIONS
a Laryngeal obstruction.
a Myocarditis: Occur from 2 ro 40 days after pharyngitis
and characterrzed by rapid thready pulse, indistincr hearr
sounds. ST-T wave changes, conduction abnormalities,
dysarrhythmias or cardiac failure; hepatomegaly and {luid
retention. Damage is reversible. Death in 50-607o cases.
Polyneuritis: Neuritis of nerves of palate and pharynx
resulting in nasal speech, nasal regurgitation offood occurs
during first or second week. Diplopia, strabismus, difficulty
in accommodation due to paralysis of ocular and ciliary
muscles occur during rhird week. Neuritis of peripherai
motor nerves supplying inrercostal muscles, diaphragm
(phrenic nerve) also occurs. Generalized paresis occurs afrer
fourth week.
r Bronchopneumonia: Secondary pneumonia common in
fatal cases.
TREATMENT
o Neutralization of free circulating diphtheria toxin: Inj.
antidiphtheritic serum (ADS in 10,000-20,000 U per
amp) is given early IV orIM after skin sensitiviry tesr-O.1
ml of a 1:1000 dilution of antitoxin in saline can be
given intracutaneously or placed in the conjunctival sac. A
positive reaction (>10 mm of erythema within 20 minutes,
or development of conjunctivitis or rearing) necessitares
desensitization done by slowiy increasing doses (bv SC, IM,
and then IV route) given at 20 minutes intervals. Reactions
should be treated with aqueous epinephrine (1:1,000) IV
Dose of Inj. ADS:
,
r 20,000-40,000 U in nasal diphtheria
r 40,000-80,000 U in tonsillar and pharyngeal diphtheria
r 80,000-i20,000 U in laryngeal diphtheria
l
Diphtheria immunoglobulin (DIG) can be used in place \
of ADS in a dose of 0.6 ml/kg.
lr
I

Antibiotic(s) to halt toxin production, treat localized infec-
tion, and prevent transmission of the organism to con-
tacts: Inj. c-penicillin 100,000-150,000 Ulkglz4 ht in
four divided doses IV or IM, or Inj. procaine penicillin
25,000-50,000 Ulkglz4 hr in two divided doses IM or
Erythromycin 40-50 mglkgl24 hr in four divided doses.
Therapy is given for 14 days. Antibiotic for 7-I0 days is
given for cutaneous diphtheria.
Supportive therapy: Bed rest for 2 weeks to decrease
hazards of cardiac complications; maintenance of hydration
and nutrition.
Tireatment of complications:
o Congestiae cardiacfailure (CCF): Bed rest, oxygen, di-
uretics, digoxin. In arrhythmia, quinidine. In myocardi-
tis, prednisolone 1_2 mg/kg/d is useful.
,.> Respiratory obstructioz; Tlacheostomy is life-saving, es-
pecially in laryngeal obstruction.
o In case of palatal paraljtsis: Frequent aspiration of se-
cretions and feeding through NG tube and IV fuids are
recommended.
o Prevention: Can be done by DPT (described earlier in
this chapter).
PROGNOSIS
Mortality ranges from 3o/o to 25o/o and high if myocarditis
occurs early.
The term enteric fever includes typhoid fever caused by
Salmonella enterica uar ryphi and paratyphoid fever caused
by S. enterica uar para1rphl A, B, or C. The ratio of disease
caused by S. typhi to that caused by S. paratyphi is about
10:1
.
ETIOPATHOGENESIS
S. enterica serotype 4tphi/para4tphi is a gram-negative, non-lactose
fermenting, fagellate bacterium. The somatic or O antigen is
shared among various salmonellae; the fagellar or H antigen
is specific to the serovar. S. enterica uar ryphi also possesses a
Vi polysaccharide capsule.
The disease occurs by the ingestion of the organism, and a
variery of sources of fecal contamination have been reported,
,:
including street foods and contamination of water reseryoirs.
The infecting dose is about 105-10e organisms with an
incubation period ranging from 4 to l4 days, depending on
i the inoculating dose of viable bacteria. After ingestion, S.
?
4'phi organisms are thought to invade the body through the
qut mucosa in the terminal ileum, possibly through special-
r ized antigen-sampling cells, known as M cells that overlie
gut-associated lymphoid tissues through enterocytes or via
I
IMMUNIZATION AND INFECTIOUS DISEASES
a paracellular route. S. typhi crosses the intestinal mucosal
barrier after attachment to the microvilli by an intricate
mechanism involving membrane ruffiing, actin rearrange-
ment, and internalization in an intracellular vacuole. After
passing through the intestinal mucosa, S. ryphi organisms
enter the mesenteric lymphoid system, and then pass into
the bloodstream via the lymphatics. This primary bacteremia
is usually symptom-less, and blood cultures are frequently
negative at this stage ofthe disease. The blood-borne bacteria
are disseminated throughout the body and are thought to
colonize the organs of the reticuloendothelial system, where
they may replicate within macrophages' After a period of
bacterial replication, S. typhl organisms are shed back into
the blood, causing a secondary bacteremia, which coincides
with the onset of clinical symptoms and marks the end of
the incubation period.
CtINICAI FEATURES
There is no appreciable difference between the manifestations
of ryphoid and paratyphoid fever. The hallmark of enteric fever
is fever that starts as low-grade fever and then shows stepwise
increase, peaking to as high as 103-104"F by the end of the
first week. This pattern differentiates it from viral fever, where
the peak is usually at the ons€t of fever.
With fever there is associated malaise, dull headache,
anorexia, nausea, poorly localized abdominal discomfort,
and mild cough. There may be diarrhea; constipation in
children is rare. Physical findings are unremarkable with the
exception of a coated tongue, tumid abdomen, and some-
'Western
times hepatosplenomegaly. The rash described in
textbooks is seldom or never seen in Indian subcontinent'
Infants and young children with enteric fever may have
diarrhea as a predominant manifestation or a short-lasting
undifferentiated febrile illness.
In the absence of treatment, fever may continue for 3-4
weeks followed by natural remission or by development of
complications.
coMPHCATIONS
Intestinal hemorrhage (<1%) and perforation (0.5-1%) is
infrequent among children, seen in the second or third week
of illness. Intestinal perforation may be preceded by a marked
increase in abdominal pain (usually in the right lower quadrant),
tenderness, vomiting, and features of peritonitis. Intestinal per-
foration and peritonitis may be accompanied by a sudden rise
in pulse rate, hypotension, marked abdominal tenderness and
guarding, and subsequent abdominal rigidiry. A rising white
6lood .itt count wirh a lefi shift and free air on abdominal
radiographs may be seen in such cases.
E*ir"-intesti.tal infectious complications of typhoid fever
caused by Salmonella enterica serltYpe typhi have been listed
in Table 21.10.
ESSENCE OF PFDIATRICS
Table 21.10: Extra-intestinal Infectious Complications of
Typhoid Fever Caused by Salmonella Enterica Serotype Typhi
Central nervous system Encephalopathy, cerebral edema, subdural
empyema. cerpbra j abscess, meningitis.
ventricul itis, transient parkinsonisrn, motor
neuron disorders, ataxia, seizures, Cu jllain-
Barre syndrome. psychosi.
Cardiovascular system
Endoclrditis. myor arditrs, perir ardilis.
arleritis. congest ive heart [ailure
Pulmonary system Pneumonia. empyemd. bront hopleural [isrula
Bone and joint Osteomyel itis, septic arthritis
Hepatobiliary system Choleclitilis, hepatitis. hepatit absces5es.
splenic abscess, perltonitis, paralytic ileus
Cenitourinary system Urindry tract infection. renal abscess. peh ic
inlet t ions, lesticular abscess, prostatili5,
epididymil is
Soft tissue infections Prod\ absce.s, gluteal abst e)s, ( uldneous
vascu I iti.
Hematologic Hemophagocytosis syndrome
DIAGNOSIS
l. Leukopenia, but there is a wide range in counrs; in younger
children, leukocytosis is a common association and may
reach 20,000_25,000/mm.. Thrombocytopenia may be
a marker of severe illness and accompany DIC.
2. Bacteriologic cultures are diagnostic [bone marrow culture
is mosr sensitive (positive 85-90o/o) and blood culture is
positive in 40-600/o of casesl.
The gold standard for diagnosis is blood culture. Salmo_
nella is an easy organism to culture and use ofbile broth
media and automated culture system such as BACTEC
improve recovery. Stool and urine cultures become posi_
tive after the first week.
3. 'Widai test is done after the first week for the detection
of salmonella anribodies in patientt serum. Interpretation
of Widal test:
a. No singie titer is diagnostic, it may be presumptive.
b. In the non-immunized child, O aggiutinin titers of
> 1:1 60 are suggestive of infection. A rising tirer over
a period of 7-10 days is, however, of greater value.
c. Elevation of antibody titer against O (somatic) anrigen
has a better diagnostic value. If a child had a previ_
ous infection with typhoid, paratyphoid fever or had
immunization with TAB vaccine , the H antibody titer
rises rapidlv r.vith non-typhoid fevers, but the titer of
O antibodies does not rise. This is called anarnnesric
(do nor forge r r reacrion.
d. High anri-O and ior.r. anti-H suggesr active infection.
e. High anti-H and lorv anri-O suggesr anamnesric
reaction.
f High anti-Vi suggesrs carrier srate (usuallv >Il2i).
tearment with antibiotics may depress an anribody
tVidal test may
response. be fllse positive and filse negarive.
4. Newer diagnostic rests using monoclonal antibodies have
been developed that directly detect S. typhi-spectfrc antr_
gens in the serum or S. typhi Vi antigen in the urine.
However, few have proved sulllciently robusr in large_scale
evaiuations. A nested PCR using HI-d prtmers has been
used to amplif, specific genes of S. tlphi in the blood of
patienrs, and given the low level of bacter.emia in enreric
fever is a promising means of making a rapid diagnosis.
5. Liver function resr resuks may be de ranged; significant
heparic dysFrrncrion is rare.
TREATMENT
o Antibiotics: Tiadirional therapy with either chloramphenicol
or amoxicillin is associated with relapse rates of 5_I5o/o
and 4-8o/o, respecriyely, whereas the quinolones and third_
generarion cephalosporins are associared with higher cure
rares. Over the past two decades, emergence of multidrug-
resistant srrains of S. typhi (i.e., isolates fully resistant to
amoxicillin, trimethoprim-sulfamethoxazole, and chloram_
phenicol) has necessitared treatmenr with fluoroquinolones
or cephalosporins. Relapse with all anribiorics may occur in
15o/o of previously treared patienrs (Thble 21.11).
r In addition to antibiotic therapy, dexamethasone 3 mg/kg
for the initial dose, followed by 1 mg/kg every 6 t ouifoi
48 hour, impr-oves the survival rate of parienrs with shock,
obtundation, stupor, or coma.
o Supportive: Adequate resr, hydrarion, and maintenance of
appropriate fluid and electrolyte balance musr be under_
scored. Antipyretic therapy (acetaminophen 120-750 mg
every 4-6 hour PO) should be provided as required. i
soft, easily digestible diet shoLrld be continued unless the
patient has abdominal distention or ileus. \When intestinal
hemorrhage is severe, blood transfusion is needed.
o Surgery: Surgical interference with broad-specrrum antibi_
otic is recommended for intestinal perforation. In presence
of cholelithiasis or cholecystitis, cholecystecromy within I4
days of antibioric ,..",rn"n, i, ,..o--.nd.d. '
r Caruier: Individuais who excrete S. typhi for >3 months
after infection are regarded as chronic carriers. However, the
risk for becoming a carrier is low in children and increases
with age, but in general is <2o/u for all infected children.
Children with schistosomiasis can de'elop a chronic urinary
carrier srare. Tleatme nr rvith amoxicillin (1 00 mg/kg/d) with
probenecid (30 mg/ke/d) or cotrimorazole (10 mg/kg/d)
for 6-12 u'eeks is recommendecl. If the strain is nalidixic
acid sensirive. quinolones fbr 2.S dar.s is a better option.
THERAPY OF RETAPSES
Relapse rares vary r,r'ith the npe of drug and are most common
rvith beta-lactams (ceftriaxone, celixime) especiallv if shorter dura-
 I
IMMUNIZATION AND INFECTIOUS DISEASES

Table 21 .11: Treatment of Typhoid Fever in Children

Uncomplicated typhoid fever:

Fully sensilive Chloramphenitol 50-75 1 4-21 Fluoroquinolone, e.g., ofloxaci rr 15 5-7'
Amoxicillin 75-1 00 14 or ciprofloxacin

Multidrugresisldnt Fluoroquinoloneor 15 Azithromycin B-1 0 7

cefixime 15-24 7*1 4 Cefixime 1 5-20 7-1 4

Azilhromycinor 8-1 0 7 Cefixime 20 7-14

Quinolonere>ist.lnl
ceftriaxone 75 10-1 4

Severe typhoid fever:

Ampicillin or 100 14 I Iuoroquinolone. e.g.. o[lorac in 15 10-.14
Fully sensitive
ceftriaxone 60-75 10*1 4 or ciprofloracin

Multidrug resistant F luoroqu rnolone 15 10-14 Ce[triarone or 60 10-14

cefotaxime BO

resistant Ceftriaxone 60-75 10-14 Fluoroquinolone 20-30 14

Quinolone
A 3 day course is also etfective, particularly for epidemic containment.

10 14 davs are effective.

tion of therapy is employed. Relapses may be satisfactorily treated
rvith the same drug as used for primary therapy but at appropri-
ate dose and for appropriate duration. Howeve! if the isolate is
nalidlxic acid sensitive and fluoroquinolones were not used for
primary therapy, they should be used for ffeatment of relapse.
Azithromycin is likely to be a promising agent for this purpose.
PREVENTION
General personal hygienic measures (hand washing and atten-
tion to food preparation practices) are necessary. Sanitation
should be improved. Safe water should be taken. Carriers must
be treated (because humans are the only reservoir of S. typhi).
Globally, three vaccines are currently available for potential
use in children. An oral, live-attenuated preparation of the
Tv2ia strain of S. typhi (efficacy, 65-B2oh) for up to 5 years.
The Vi capsular polysaccharide (efficacy of 70-80%) can be
used in people 22 year ofage. The recent Vi-conjugate vaccine
has been shown to have a protective efficacy exceeding 9070
in younger children.
Meningococcal disease is caused by Neisseria meningitidis. N.
ttteningitidis is thought to be acquired by respiratory route.
and hypotension. Purpura, petechiae, and occasionaliy bright
pink tender macules or papules over the extremities and
trunk are seen. The rash usually progresses rapidly. Occa-
sional cases lack rashes.
Fu lm in an t m e n i ngo c 0 c c e m i a (Warcrho use-Frideri chs en
syndrome) progresses rapidly and is characterized by dissemi-
nated intravascular coagulation, massive skin and mucosal
hemorrhages, and shock.
Chronic meningococcemia is characterized by periodic
bouts offever, arthraigia or arthritis, and recurrent petechiae.
Splenomegaly is often present. The patient may be free of
s)'mptoms beween bouts. Chronic meningococcemia occurs
primarily in adults and mimics Henoch-Schonlein purpura.
r Meningitis: In many children meningococcemia is followed
within a few hours to several days by symptoms and signs of
acute purulent meningitis with severe headache, stiff neck,
nausea) vomiting, and stuPor.
DIAGNOSIS
Definitive diagnosis of meningococcal disease is made by the
isolation of the organism from a usually sterile body fluid such
as blood or CSF.
DIFFERENTIAL DIAGNOSIS

This includes acute bacterial or viral meningitis, encephalitis,

CLINICAT FEATURES
\lanv children with clinical meningococcemia also have men-
insitis. All children with suspected meningococcemia should
lave a lun-rbar puncture.
r \Ieningococcemia: A prodrome of upper respiratory infec-
:i-.: is fbllori'ed bv high fever, headache, marked toxicity,
serum sickness, collagen vascular diseases, Henoch-Schonlein
purpura, hemolytic uremic svndrome.
COMPLICATIONS
These include adrenal hemorrhage, arthritis, myocarditis, pneu-
monia, lung abscess, peritonitis, and renal infarcts. Deafness,

ESSENCE OF PEDIATRICS
ataxia, seizures, blindness, cranial nerve palsy, hemiparesis,
quadriparesis, or hydrocephalus may also occur.
TREATMENT
Inj. penicillin Gis 250,000-300,000 Ulkgl24hr, administered
intravenously in six divided doses. Cefotaxime (200 mglkgl24
hr) or ceftriaxone (100 mglkgl24 hr) and chloramphenicol are
effective empirical therapy for meningococcal disease. Therapy
is continued for about 7 days.
PROGNOSIS
Despite the use of appropriate antibiotics, the mortaliry rare
for disseminated meningococcal disease remains at" 8-l2o/o.
PREVENTION
Close contacts: Prophylaxis is indicated as soon as possible for
household contacts. Rifampicin 10 mg/kg/dose orally every
12 hour for 2 days (total offour doses). The dose is reduced to
5 mglkg for very young infants. Patients with meningococcal
disease should receive rifampicin before discharge.
Meningococcal vaccine is present for the prevention of
meningococcal disease.
Syphilis is a systemic communicable infection caused by
Ti'eponema pallldum.
Congenital syphilis results from transplacental transmission
of spirochetes. Syphilis during pregnancy has a transmission
rate approaching 100%. Fetal or perinatal death occurs in
40o/o of affected infants.
CtINICAL FEATURES
Among survivors, manifesrarions have traditionally been divided
into early and late stages. The former appears during the first
2 year of life, while the latter appears gradually during the
first 2 decades.
Eorly Sloge
Clinical features are analogous to the secondary stage of
acquired syphilis. Two-thirds are asymptomatic at birth.
Hepatosplenomegaly, jaundice, lymphadenopathy, Coombs-
negative hemolytic anemia, and thrombocyropenia may occur.
Characteristic osteochondritis, periostitis, mucocutaneous
rash, mucous patches, rhinitis (snu{fles), condylomata, and
desquamation of hands and feet occur. Bone involvement
occurs. Roentgenographic abnormalities include multiple sites
of osteochondritis at the wrisrs, elbows, ankles and knees,
and periostitis of the long bones, pseudoparalysis of Parrot.
I
Central nervous sysrem (CNS) abnormalities, failure to thrive,
chorioretinitis, nephritis, and nephrotic syndrome may also
be seen. Clinical manifestations of renal involvement include
hypertension, hematuria, proteinuria, hypoproteinemia,
hypercholesterolemia, and hypocomplemenremia. Less
common clinical manifestation of early congenital syphilis
includes gastroenteritis, peritonitis, pancreariris, pneumonia,
eye involvement (glaucoma and chorioretinitis), nonimmune
hydrops, and testicular masses.
Lote Sloge
The late manifestations result primarily from chronic inflam-
mation of bone, teeth, and the CNS. Frontal bossing, a bony
prominence of the forehead (olympian brow); unilateral or
bilateral thickening of the sternoclavicular porrion of the
clavicle (Higoumenaki sign); an anrerior bowing of the mid-
portion of the tibia (saber shins), and scaphoid scapula. Dental
abnormalities are common and include Hutchinson teeth,
abnormal enamel, Mulberry moiars, abnormal first lower (6
year) molars. Defects in enamel formation lead to repeated
caries and eventual tooth destruction. A saddie nose, a perfo-
rated nasal septum, rhagades, juvenile paresis, juvenile tabes
with spinal cord involvement, and cardiovascular involvement
with aortitis are extremely rare. Other late manifestations of
congenital syphilis may represent a hypersensitiviry phenom-
enon. These include interstitial keratitis with symptoms such
as intense photophobia and lacrimation, corneal opacification
and complete blindness. Less common features are choroiditis,
retinitis, vascular occlusion, and optic atrophy, VIII nerve deaf-
ness. The Clutton joint, soft tissue gumma (identical ro rhose
of acquired disease), and paroxysmal cold hemoglobinuria are
rare hypersensitivity phenomena.
DIAGNOSIS
The serological tests in the mother are usualiy associated with
positive tesr in the newborn infant. If both tests (VDRL and
FTA ABS igM) are positive, congenital syphilis should be
suspected strongly, and the baby should be treated. Dark field
microscoplz, TPI, treponemal antibody titers, PCR can also be
done if facilities are available. Passively acquired VDRl-positive
test from the mother usually becomes negative within the first
3 months, but passively transferred FTA ABS IgM remains
positive for over 8 months.
Imaging: Radiographic abnormalities are presenr in 90o/o
of infants with symptoms of congenital syphilis and 20o/o of
asymptomatic infants. Metaphysial lucent bands, periostitis,
and a widened zone of provisional calcification may be presenr.
Bilateral symmetric osteomvelitis with pathologic fractures
of the medial tibial metaphysis (Wimberger sign) is almost
I
pathognomonic. 1
Placentai examination is informative. It is dispropordonarely I
iarge; histology shows focal proliferative villitis, arteritis, and 't
immaturity of placental villi.
\
t
t
rl
 IMMUNIZATION AND INFECTIOUS DISFASES

TREATMENT

o IV Peniciilin G (100,000-i50,000 Ulkgl24 hr) and IM

STDs are acquired by sexual contact and intercourse (including
procaine penicillin 50,000 lJlkgl24 hr given for 10-14 days.
genital, rectal, and oral penetration). These diseases are most
o Mother should also be treated.
common in the adolescent and young adult population (15-24
years of age) (Thble 21.12).

PREVENTION Agents of specific STDs:

Routine prenatal screening. Any woman who is delivered of
a stillborn infant >20 weeks of gestation should be tested for
syphilis.
PROGNOSIS
Severe disease, undiagnosed, may be fatal in the newborn.
if
Complete cure can be expected if the young infant is treated
rvith peniciilin. Serologic reversal usuaill' occurs within
1 year.Tieatment of primary syphilis with penicillin is curative.
Permanent neurologic sequelae may be seen with meningovas-
cular syphilis.
I
Chlamydia trachomatis is one of the most common non-
^.
viral STDs. It is an intracellular bacterium (know as an
inclusion body), found in columnar lining cells of the
cerix, uterus, llllopian tubes, liver capsule, urethra, rectum,
pharynx, and skin. C. trachomatis is the most common
cause of non-gonococcal urethritis in men. It also causes
lymphogranuloma venereum and inclusion conjunctivitis.
It is one of the leading causes of infertiliry in females. This
microorganism can also cause epididymitis in men.
b. l{eisseria gonorrhoeae is an intracellular, gram-negative
diplococcus found in a distribution similar to that of C.
trachomatis.In addition to infection in the genitourinary
area, joint involvement can lead to arthritis.

i.
I Table 21.12: Sexuallv Transmitted Diseases in Adolescents
I

Ch I amvd i a t racho ntati s Cervical ectopy and friability. Mucoid cervical discharge with Chlamydia inclusion bodies identified
I
leukocyte count. Dysuria (in men may not be accompinied through cell culture or rapid test such as
by discharge). Pelvic tenderness in women. Pharyngitis, rectal fluorescenl antibody staining or spectromelric
i rrit.ltion/f enderness evaluation
Similar tlinical features as witlr chlamydial infetlion Positive culture for gonorrhea
I

Nersserla gonorrhoeae
Infection in men more likely to include purulent urethral Cram-negative diplococci in male urethral
di:r harge discha rge

Human immunodeficiency Different from adult prerentdtion: lower male 10 femdle rdlio. Spet ifir blood assay for HIV r irus
virus (HlV) More prevalent in blacVHispanic urban youth.
Higher percentage of heterosexual transmission
Mycoplasma Similar r haracterislics to chlamydial infection Culture

Treponema pallidum syphilis' Slage ol disease: Dark field examination or direct fluorescent
Primary (1 0-40 days): chancre, regional lymphadenopathy anlihody tesls of lesion
Secondary (2-5 months): generalized malaise, lymphadenopathy, Serologic tests: Nontreponemal
skin change:/aloper ia. lreponemal
I ate ,2- I 0 y"orri, , enlral nervous sy:tem t hanges, t ardiovascular
t hanges. musculo:kelelal involvement

Haemophilus ducreyi Painful genital ulcer Culture

(chancroid) Tender inguinal lymphadenopathy Clinical picture

Herpes simplex virus Vesictes/ulcers on external genitalia, in vagina, on cervix, and Viral culture
around rectal area. Tender inguinal lymphadenopathy lzlncL tesl
Dysuria
Dyspareunia

Conclyloma acuminatun Single or group of painless warts Clinical appeardn( e

i\,lol luscum contagiosum Small papule with umbilical centers B iopsy

CIinir al dppearan( e
Fotassium hvdroxide smear of contents.
Biopsy

Trichomonas vaginalis Presence of flagellate protozoan. Malodorous yellow-green Microscopir idenrifit aLion o[ organism

H
vaginal di>charge
, i^ i'i.. cubr-. i pedicu losis Pruritus in public hair Crab lice or egg cases in pubic hair
:1..

4
: -: . cr.:c l:ce' Tan egg cases in pubic hair
ESSENCE OF PEDIATRICS

c. Human immunodeficiency virus (HIV) is acquired

through sexual transmission or intravenous spread from
blood products or illicit drug use.
Human brucellosis is caused by one of four biovars: Brucella
d. Mycoplasma is the second most common cause of non-
abortus, B. melitensis, B. suis, and B. canis.
gonococcal urethritis in men. It is a mycoplasma
Brucellosis is prevalent in the Mediterranean basin, Arabian
with genital and pelvic distribution similar ro thar of
gulf, and the Indian subcontinent.
C. trachomatis.
Infected through cuts or abrasions in the skin, inoculation
ne?onema pallidurn, the cause of syphilis, is a less common
of conjunctival sac, inhalation of aerosols, or ingestion of
cause of infection in adolescents compared to adults. It
contaminated mear or diary products.
is seen more regularly in association with other STDs
and in adolescents who use illicit drugs. T, pallidurn is a
motile spiral microorganism 5-20 mm long. The infec- CLINICAI. FEATURES
tion can start in the genital area with a skin lesion called
Symptoms can be acute or insidious in nature and are usually
a chancre, but can also occur without such a lesion and
non-specific, beginning 2-4weel<s after inoculation. The classic
in other parts of the body.
Haemophilus ducreyi, rhe cause of chancroid, should
triad is fever, arthralgia/arthritis, and hepatosplenomegaly.
Other symptoms include abdominal pain, headache, diarrhea,
be considered in the differential diagnosis of a painful
rash, night swears, weakness/fatigue, vomiting, cough, and
genital ulcer often accompanied by tender inguinal
pharyngitis. The bones and joints are involved frequently;
lymphadenopathy.
commonly sacroiliac joint, knee, and ankles.
o
b' Herpes simplex virus (HSV): The most common form
causing genital involvement is HSV type 2.
h. Condyloma acuminatum (venereal warts) is a form of INVESTIGATIONS
papillomavirus infection and the mosr common STD
found in adolescents. Blood and bone marrow culture. Complement fixation and
Molluscum contagiosum is a poxvirus infection found in anti-human globulin tests are more useful in chronic infections.
any part of the body, but it is usually presenr in greater
concentration in the genital area when sexual ransmission DIFFERENTIAT DIAGNOSIS
is involved.
j. most
Thichomonas uaginalis is a flagellate prorozoan presenr Tirberculosis, enteric fever, lymphoma, infectious mononucleo-
commonly in the vagina but also found near the urethra sis, leukemia.
of both sexes.
k. Phthirus pubis (crab lice) is a parasite that is <4 mm long. TREATMENT
l. Scabies caused by S. scabiei.
Uncomplicated:
r Co-trimoxazole 10 mg/kg/dose plus Rifampicin 10-20
TREATMENT
mg/kg/d for 6 weeks.
Therapy for STDs should include all exposed individuals,
o More than 8 years: Doxycycline 200 mgld for 6 weeks plus
whenever possible. The specific treatmenr depends on accurate
Streptomycin 1 g IM for l-2 weeks or Gentamicin 3-5
identification of the causative organism; the choice of antibiotic mglkgld IM/IV for l-2 weeks.
must take into consideration the organisms' sensitiviry and Complicated with meningitis, osteomyelitis, endocarditis:
patientt age and history of allergies. Two general rrearmenr
recommendations should be taken into considerarion.
r StreptomycinIM for 4-6 months plus Rifampicin 10-20
mg/kg/d PO for 4-6 months.
a. \Xrhenever possible, trear the STD with a single dose of
medication right on the spot to ensure compliance. This
is possible for gonorrhea, chlamydial, and trichomonal PREVENTION
infections.
Eradication of organism from cartle, goats. Pasteurization of
b. If either gonorrhea or chlamydial infection is suspected,
treatment should be given for both diseases.
milk and diary products. :
\
1
PREVENTION 1
.t
Proper use of condoms can reduce the risk of transmitting Tirberculosis is a chronic infectious disease caused in vast t
most STDs. majority of cases by M. tuberculosis.

I

Primary complex: The primary focus in lungs, lymphangitis
and regional lymphadenopathy are together known as primary
complex of Ranke. Outcome of disease depends on fate of
primary complex. Fate of primary complex may be divided
into (a) occult primary complex, (/) progression of lung focus,
(r) progression of lymph node, and (/ lymphohematogenous
dissemination.
a. Occult or silent primary complex: In well-nourished
children, primary complex heals completely by fibrosis
or calcification and shows no symptoms. CXR normal.
If child immunity is reduced as in malnutrition, measles,
and whooping cough, bacilli within the complex may
become active and start to multiply.
b. Progression of lung focus: Subpleural lung focus may
enlarge and rupture into pleura causing tuberculous
pleural effusion or tuberculous empyema. Lung focus
may enlarge and liquefr. Caseation material may empry
into bronchus forming thin walled caviry or may spread
through bronchus causing unilateral bronchopneumonia
or bronchial obstruction.
c. Progression of lymph node lesion: Mediastinal, hilar or
paratracheal lymph nodes may enlarge and press upon
narrow air passages causing Partial obsffuction, unilateral
wheezing, emphysema or complete obstruction, collapse/
consolidation and bronchiectasis. Soft contents of lymph
nodes may erode bronchus causing bronchopneumonia, may
spread to pericardium (pericardial effiusion) and esophagus.
d. Lymphohematogenous dissemination: Bacilli may escape
from lymph node and lung focus through blood stream
or lymphatics to distant organs such as liver, spleen, bone
marrow kidneys. If immunity is not well-developed as
in very young children or is weakened, acute miliary TB
and/or tuberculous meningitis develops soon. In pres-
ence of good defense, tubercles in distant organs may get
healed or may cause tuberculosis of lymph nodes, bone,
joints, kidneys months to years after primary infection.
In adolescent, the hematogenous foci in lungs known
as Simon foci at apices of lungs may get activat€d to
'Ashman focus", which progresses locally to form cavity.
Neonate gets infected from mother through placentitis,
aspiration of infected amniotic fluid or postnatal airborne
transmission.
r for another MT. Palpate induration (thickenning of the skin)
Clinical types of tuberculosis include (i) primary puimonary
disease, (zz) pleural efftrsion, (liz) progressive primary pulmonary
disease, (iu) reactivation TB (post-primary), (z) lymphohema-
togenous dissemination (miliary TB), (ui) Iymph nodeTB' (uii)
tuberculous meningitis, (u i i i) tuberculoma, (zr) gastrointestinal
I TB, and (x) TB bones and joints.
f'
r in severly malnourished children, HIV infected children.
r PRIMARY PUTMONARY DISEASE
r
Subpleural lung foci occur in 70o/o cases with localized pleurisy.
t Halimark is relatively large size of regional lymphadenopathy.
IMMU NIZATION AN D INFECTIOUS DISEASES
Clinicql Feotures
50%o cases are asymptomatic. Symptoms: non-productive
cough, mild dyspnea, fever, night sweats, anorexia, decreased
activity, failure to gain weight.
Signs: localized wheezing, dullness, diminished breath sound
due ro bronchial obsrruction.
Diognosis ond lnvestigotions
o History of contact with a TB patient.
e CXR: Perihilar lymphadenopathy, collapse with consolida-
tion; focal hyperinflation, atelactasis.
r Finding AFB in three consecutive morning gastric aspirates
(50% sensitive).
o Culture: Lowenstein-Jensen (LJ) medium, 7-10 week of
incubation is needed. Microscopic examination of thin layer
culture plate may lead to detection of micro colonies of M.
tuberculosis as early as after 7 days. Less time consuming
other techniques for culture are BACTEC radiometric assay,
Septichek AFB system, and mycobacterial growth indicator
tube system.
o Polymerase chain reaction (PCR): It may be used to
(z) diagnose tuberculosis rapidly by identifying DNA from
M. tuberculosls in clinical samples that are negative by micro-
scopic examination; (ll) determine rapidly whether acid-fast
organisms identified by microscopic examination in clinical
specimens are M. tuberculosis or atypical mycobacteria; and
(iii) identify the presence of genetic modifications known
to be associated with resistance of some antimycobacterial
agents. The sensitivity ranges from 4o/o to 80o/o and the
specificiry 80-1000/0.
o Serodiagnosis: ELISA has been used in children to detect
antibodies to various purified or complex antigens
of M. tuberculosis. At present, serodiagnosis does not
have any role in the diagnosis of childhood pulmonary
tuberculosis.
o Positive MT or BCG test.
Tuberculin Test: Mantoux Test (MT)
PPD-S (5 TU per 0.1 ml) is used. The result should be read
between 48 andT2hour of administration. A patient who does
not return within 72hour will probably need to be rescheduled
not erythema (reddening of the skin). Measure diameter of
induration using a clear ruler:
o Place 0 of ruler line on the inside left edge of the induration.
r Read ruler line on the right edge of the induration
Interpretation:
Diameter of induration of >5mm is considered positive
Diameter of induration of >10mm is considered positive
in all other children (whether or not they have received
BCG vaccination).
ESSENCE OF PEDIATRICS

Causes offalse positiae and fabe negatiae MT PROGRESSIVE PRIMARY PULMONARY

False positive results: DISEASE
i. Infections due to atypical mycobacreria \X/hen primary focus enlarges, there develops caseous center
ii. BCG vaccination
iii. Infection at the site of test
with cavity formation, and large number of tubercle bacilli
extend into neighboring bronchus and lung tissue.
False negative results:
Symptoms: High fever, severe cough with sputum production,
i. Infections:
weight loss, night sweats.
a) Viral (measles, mumps, chicken pox, HIV)
b) Bacterial (ryphoid fever, brucellosis, ryphus, leprosy, Signs: Restricted movement of chest, dull percussion nore,
pertussis, ovem'helming tuberculosis) diminished brearh sound, may be bronchial, creps + egophony.
c) Live virus vaccinations (measles, mumps, polio, varicella) Investigations:
ii. Metabolic derangements: Chronic renal failure, liver
o CXR may present cavity or consolidation.
failure, severe malnutrition
r Sputum may be positive for AFB; BCGT or MT-positive.
iii. Diseases affecting lymphoid organs: Hodgkin disease,
lymphoma, chronic leukemia, sarcoidosis
iv. Drugs: Corticosteroids, other immunosuppressive agents REACTTVATTON TB (POST- PRTMARY)
v. Age: Newborns, elderly parients Endogenous reactivation of tuberculous focus may occur in
vi. Stress: Surgery, burns, mental illness, graft-versus-host adolescence. Common in those who acquire primary infec-
reactions
tion after 7 years of age. Disease localized to lungs. Highly
vii. to the tuberculin used:
Factors related
contagious.
a) Improper storage (exposure to light and heat)
Symptoms: Fever, weight loss, night sweats, productive cough,
b,) Improper dilutions
hemoprysis, chest pain.
c) Chemical denaturation
d) Contamination Signs: t Percussion note dull, craps, vocal resonance.
viii. Factors related to the method of adminisrrarion: Investigations: Exrensive infiltrates, thick walled cavities in
a) Injection of too little antigen upper lobe.
b) Subcutaneous injection
c) Delayed administration after drawing into syringe TYMPHOH EMATOGENOUS DISSEMI NATION
d) Injection to close ro orher skin tests (MTUARY TB)
ix. Factors related to reading the test and recording results:
Most common form of lymphohematogenous dissemination
a) Inexperienced reader
is miliary TB, when massive number of TB bacilli are reieased
b) Conscious or unconscious bias
c) Error in recording into blood and then into lungs, liveq spleen, brain. More
common in infants and young children; usually occurs within
BCG Test (BCGT) 2-6 months of primary infection.
Not universally accepted as a diagnostic tool. (Read on third
S;'rnptoms: Onset explosive or insidious; weight loss, anorexia,
day, but can be read from 24 or 48 hours to 10 days).
1. Negative: normal reaction and evolution in nontubercu, low-grade fever, PUO. It may be pulmonary rype (severe
toxemia with dyspnea), septicemic type (resembles septicemia
lous children:
or severe typhoid), and meningitic rype (convulsion and neu-
a) Papule with induration 2 week, pustule 4-6 weeks,
rological features).
b) Healing with scab formation 7-10 weeks.
2. Positive (accelerated reaction) Signs: Hepatosplenomegaly (in 50%), generalized lymphade-
a) 24-72 hours induration nopathy, cough, dyspnea, wheezing, pneumothorax, peritonitis,
meningitis (2040ok). Skin lesion-necroric, nodular, purpuric.
i. 5-9 mm-Mild (l+)
ii. Choroid tubercle (found in 15-85o/o cases) is highly specific
10-20 mm-Moderare (2+)
for miliary TB see n b,v ophthalmoscope . Choroid tubercles are
iii. 2l-30 mm-Severe (3+)
1-3 mm vellowish, rounded, slightly raised spots; the edges of
b) l-S days Pustule formation. which fade with general pinkness of retina. They are most likely .\
c) 10-15 days healing with scab formation. to be found within 2 disc diameters of the center of optic disc. rf
As tubercles get older, edge become definite and center white. 1
PtEURAt EFFUSION 1
Investigations: High degree of suspicion, choroid tubercle
Pleural effusion has been described under Respiratory Diseases. on ophthalmoscopy. CXR: normal at first, miliary mottling
\,
I

(2-3 mm) appear at later stage. Liver biopsy, bone marrow-
histology. AFB and culture and sensitivity may be positive'
H/O recent exposure to adult TB.
LYMPH NODE TB
Superficial lymph nodes, especially cervical, are commonly
affected either by drinking unpasteurized cow milk or by
Iymphatic dissemination from mediastinal lymph nodes. Nodes
are firm discrete then become fluctuant, matted, and get fixed
to skin. Then nodes soften forming cold abscess that burst
through skin forming sinuses. Nodes may or may not be tender.
{ Healing occur by fibrosis with scarring. Lymph node biopsy
t will show TB granuloma.
V
l.
t
TU BERCULOUS MENI NGITIS
a See Neurology chapter.
TUBERCUIOMA
Tirberculoma is a tuberculous foci that enlarges within the
brain tissue without rupturing, presenting clinically as brain
tumor, and may account for 40o/o of brain tumors in some
areas of world. May present with headache, fever, convulsions,
signs of raised intracranial pressure or hemiplegia, cranial nerve
palsies. X-ray skull may show calcification. CT scan shows a
hypodense mass and ring enhancement with contrast' MT,
BCG is positive. CXR is normal. Surgery usually not necessary.
GASTROINTESTINAL TB
GIT TB includes TB of esophagus, stomach, small bowel, large
intestine. TB bacilli may be human type, bovine, or atypical
mycobacteria. Children may be infected by (l) ingestion of
unboiled milk containing bacilli, (ll) by foods, liquids' sPoons
that carry bacilli from aduk, (iii) by lymphatic or hematogenous
spread from infected lymph nodes or primary focus, and (lz)
by direct extension from adjacent organs.
Site: Ileocecal region is the commonest site because of physi-
ological stasis, abundance of lymphoid tissue, increased rate
of absorption.
Types
o Enteric ulcerative, usually secondary to pulmonary TB.
Undermined ulcers occur in terminal ileum. Ulcer heals
forming stricture. Hemorrhage is rare due to associated
endarteritis.
r o Hyperplastic €nteric: Palpable mass is formed due to
? hyperplasia. Cecum, transverse colon, ascending colon are
I involved in descending order of frequenry'
r r Tirbercular mesenteric: Infection occurs at submucosa.
Bacilli are carried to lymph nodes. So, there is hyperplasia
7
I
I
IMMUNIZATION AND INFECTIOUS DISEASES
and caseation necrosis of lymph nodes. Mesentery and bowel
may adhere forming a mass.
r TB peritonitis: TB peritonitis are of two rypes-moist or
dry. In moist type, exudative ascites is formed and may be
encysted. In dry rype, plastic exudate glues intestine.
Clinicol Feotures
Symptoms: Duration is usually 1-3 years.
Pain abdomen: Usually right lower quadrant, colicky in
nature but may be dull aching aggravated by meal or relieved
by vomiting and passage of flatus. Pain may be around
umbilicus, left iliac fossa, or epigastrium.
a tVeakness, anorexia, loss of weight
o Constipation, vomiting
a Chronic diarrhea
a Feeling of a gas/lump
Signs:
o Distension of abdomen
o Diffuse tenderness, especially in TB peritonitis
o Mass in right iliac fossa: May be well-defined, firm, and
mobile in hyperplasric rype
o Palpable lymph nodes: Peripheral, abdominal (mobile
mesenteric, fixed if paraaortic)
Visible peristalsis and ill-defined tender lump, especially in
TB mesenterica, TB peritonitis
a Doughy feeling of abdomen, especially in TB peritonitis
a Hepatosplenomegaly t chest signs
In ascitic form of peritoneal TB, abdomen is distended
with diffuse pain, flatulence, constipation, doughy feeling of
abdomen, guarding of abdominal wall, and diffuse tenderness
with or without feeling of a mass.
In plastic form of peritoneal TB, child may present with dull
aching pain, alteration of bowel habit, marked emaciation,
ill-defined tender lump.
In any child with gastrointestinal compliant, e.g.' recurrent
abdominal pain with malabsorption, suspect abdominal TB.
Diognosis
1. Suspicion
2. H/O contact with tuberculous patients
3. Raised ESR
4. Positive MT/BCG rest
5. Ascitic fluid-protein >25 glL, cells >25Oldl, ADA raised
(sensitiviry 9 5-L 00o/o, specifi city 9 6-9 8Vo)
6. Radiology:
a) Plain x-ray abdomen-calcification, fuid level
b) CXR-may have features of TB
c) Barium follow through/contrast x-rayldouble contrast
x-ray: (i) irregularity in cecum, (li) sterling sign, lack
of retention of barium, (iii) string sing-string like
appearance ofintestine due to stricture, (la) Fleischner
 I

ESSENCE OF PEDIATRICS

sign-triangular appearance of terminal ileum, and TB Hip Joint

funnel-shaped cecum.
(zr)
7. Demonstration of AFB in lesion/culture or tuberculous
granuloma with caseation necrosis (gives definitive diag-
nosis) in the following materials: (z) sur.gical specimen, (iz)
FNAC from palpable mass, (iiz) endoscopic biopsy, (lz)
peritoneoscopic biopsy (peritoneal biopsy by Vim Silver
Mann needle), (z) ascitic fluid, and (ui) lymph node/liver
biopsy.
8. CT scan for nodal involvement.
Patient may present with limping with pain (pain may be
referred to knee). Shortening of leg and wasring of thigh
muscles may also be present. It should be excluded from
septic arrhriris or Perthes disease (where acetabulum remains
intact).
X-ray shows narrowing of joint space berween acetabulum
and head of femur; changes in femur and destruction of
acetabulum.

TB BONES AND JOINTS TREATMENT OF TUBERCUIOSTS (Tobtes 21.19

TB bones and joints results from hematogenous spread usually
affecting a single or few joints within 6-36 months of primary
infection. The spine is affected in over half the cases followed
by knee, hip, and ankle. In TB spine, lower half of one verte-
brae and upper half of vertebrae below with intervening disc
is affected, followed by narrowing of disc space and formation
of cold abscess. Cold abscess may presenr as retropharyngeal
abscess from cervical vertebrae or from lumbar vertebrae as a
psoas abscess in groin or may compress the spinal cord. Com-
monest site is Tl0.
Joints: Swelling, pain, abscess, sinus formation of one or
more joints.
Spine
Pain: Locally in back or along nerve routes. Pain is increased
by pressure on spine or by rotation; referred to occiput and
arm when cervical, to intercostal when thoracic, to girdles and
epigastrium when thoracolumbar. Patient may have night cries;
pain relieved by rest.
Gait Disturbed, walk with legs semlfexed. Bends knee or
hip but not spine in picking up objects from floor. Keep
neck straight without turning if cervical. If thoracic spine is
involved, keeps back stiff, turn, by moving feeu if lumbar spine
is affected, walks with waddling gait with legs apart.
Iruegularities of spine: Visible swelling or gibbus in back:
kyphosis, scoliosis, lordosis, obliteration of anterior-posrerior
curve in lumbar region.
Abscess: Intercostal abscess in chest wall; psoas abscess in groin
or thigh, retropharyngeal abscess in neck.
Paralysis: Spastic paraplegia if thoracic.
to 2l .15)
Dispersible, fixed-dose combination (FDC) tablets are now
available with the National TB Control Program. During the
initial phase of 2 months, rrearmenr is with 3 FDC tablets
each containing rifampicin 60 mg, isoniazid 30 mg, and
pyrazinamide 150 mg. In addition, ethambutol should be
given according to body weight. During the continuation
phase of 4 monrhs, 2 FDC tablets each containing rifampicin
60 mg and isoniazid 30 mg are given. This is writren as
2(HRZ)E/4(HR).
Steroids in TB: Steroids are useful in tuberculous meningitis,
tuberculous pleural effusion, acure pericardial effusion, ruber-
culous peritonitis. Prednisolone is given 2 mglkgld, with a
maximum dose of 60 mg/d for 4-6 weeks, in addition to the
usual anti-TB drugs. The dose should be rapered gradually
over 2 weeks before stopping.
Preventive therapy: Preventive therapv comprises of INH
monotherapy for 6 months. Following documented close con-
tacts/infection, groups of children are particularly vulnerable
and should receive prevenrive therapy:
o Very young (immune immarure) children <3 year of age.
r Immune compromised children (e.g., severely malnourished
or HIV infected or on steroids, immunosuppressive drugs),
irrespective of their age.
o Baby born to TB infected morher. BCG can be given at
the end of INH therapy.
Chemoprophylaxis for children: Children aged <1 year
whose household contacts are under rrearmenr for TB should
Table 21.13: Recommended Doses of Firstline Anti-TB Drugs

fusociated: Fever, night swears, weight loss, hepatospleno-

megaly, lymphadenopathy.
lsoniazid (H) 10 {5-1 5) [maximum 300 mgl
Investigations:
Rifampicin (R) 15 (1 0-20) lmaximum 600 mgl
o TB arthritis: X-ray joint AP/lat view, synovial fluid study, Pyrazinamide (Z) 35 (30-40) [maximum 2000 mgi
and synovial biopsy. Ethambutol (E) 20 5-25) [maximum 1200 mg]
(1
o TB spine: X-ray spine AP/lat view-loss of anterior or Streptomycin (S) 15 (12-18) [maximum 1000 mg)
1
1
inferior angle of body with narrowing of disc space. Abscess
Ethambutol is now considered to be sat-e in children at a dose ol 20 m{kg/d.
may also be seen. Streptomycin is mainly reserved for the first 2 months of treatment of TB meningitis. \
il
 IMMUNIZATION AND INFECTIOUS DISEASES

Table 21.14: Recommended Treatment Regimens for Children Treqlmenl of Tuberculosis in Speciol
in Each TB Diagnostic Category Situolions
Drug-lnduced Hepatitis
The anti-TB drugs should be stopped until the jaundice or
I . All intrathoracic TB 2(HRZ)E 4(HR) hepatic symptoms have resolved and liver function tests have
in absence oi lung returned to normal. If LFT cannot be done' wait 2 weeks after
cavities or extensive
the jaundice has disappeared before recommencing anti-TB
alveolar consoliation,
TB lymph node treatment. Then restart the same anti-TB drugs either gradually
(one by one) or all at once (if the hepatitis was mild).
o All extrathoracic TB 2(HRZ)E 7(HR)
excePt TB meningitis. If the hepatitis ploduced severe jaundice, pyrazinamide
lntrathoracic TB should be avoided; suggested regimen in such patient is
in lung cavities or 2SHE/10 HE. A severely ill TB patient with drug-induced
extensive alveolar
hepatitis who may die without anti-Tts drugs should be treated
consolidation
o TB Meningitis 2(HRZ)S 7(HR)
with streptomycin and ethambutol. After the hepatitis has
resolved, usual TB treatment should be restarted.
ll . Previously treated 2(HRZ)ESll 6(HR)E
smear-positive Pul TB (HRZ)E
In case of extensive TB, ofloxacin can be considered in
(relapse, treatment conjunction with streptomycin and ethambutol as an interim
after interruPtion, non-hepatotoxic regimen.
treatment failure)
I\ . MDR-TB Specially designed
. Acute Viral Hepatitis
XDR TB standardized regimens
(second-li ne anti-TB drugs,
If it is necessary to treat during acute hepatitis, combination
e.g., Fluoroqui nolones,
Kanamycin, Amikacin,
of streptomycin and ethambutol for 3 months is the safest
Capreomyci n, Cycloserine, option. If the hepatitis has resolved, the patient can receive a
PAS) continuation phase of 5 months isoniazid and rifampicin. If the
Should be discussed r'vith an hepatitis has not fully lesolved, streptomycin and ethambutol
expert
should be continued for a total of 12 months.
E, ethambutol; H, isoniazid; R, rifampicin; S, streptomycin; Z, pyrazinamide

(hronic Liver Disease

Table 21.15: Drugs and Age-speciiic Doses for the lnitial and The regimen should be 2 SHRL/6 HR. Total 8 months.
Continuation Phase of Treatment for Children
RenalFailure
Isoniazid, rifampicin, and pyrazinamide can be given in normai
No. of 3 FDC (R/H/Z: No. of 2 FDC (R/H:60/30
mg) mg) daily during next 4
doses as these are either eliminated almost entirely by biliary
60/30/1 50) + E (100
daily dr-rring {irst 2 months months excretion or metabolized into non-toxic products. Streptomycin
2) 0.5 0.5 and ethambutol are excreted by the kidney and can be given
in reduced doses or intermittently where facilities for close
47 tl
,) monitoring of renal function are available. The safest regimen
B-1 4
is 2 HRZ/4 HR.
15 19 Jl
20-29 44 Pregnancy
Most anti-TB drugs are safe fol use in pregnancy with the
exception of streptornycin, which is ototoxic to the fetus'
be given chemoprophyla-ris with isoniazid 5 rng/kg/d foL 6
months irrespective of BCG status, and the child is free of
Breast-feeding Women
active TB. Follow-up should be carried out at least every 2
months until completion of treatment. An infant born to a A woman with TB who is breast-feeding should receive a full
mother with infectious pulmonary TB can be safely breastfed course of anti-TB drugs. Breast-feeding should be continued.
if given isoniazid prophylaxis. If a child receiving isoniazid Prophylactic treatment with isoniazid should be given for at
develops symptoms, assessment for TB should be done. If the least 3 months ahead of the time the mother is considered
child has not been BCG vaccinated, BCG should be given non-infectious. BCG vaccination of the newborn should be
F alier completion of isoniazid treatment. postponed until the end of isoniazid prophylaxis.
lF
F
F
 -{

ESSENCE OF PEDIATRICS

Treolmenl Oulcome Descriplions

Cured: A smear-positive patient will be declared cured if the
following conditions are fulfilled: Leprosy, also known as Hansen disease, is a chronic granu-
o The entire course of 6(7) and 8(9) months lomatous disease caused by M. leprae affecting almost all the
of treatment has
organs, particularly the skin and nerves.
been completed and
o The sputum smears are negative on at least two occasions:
o At the end or during the last month of the treatm ent and cr.AsstFtcATtoN
o On at least one previous follow-up occasion, at least The Ridley-Jopling classification, which is based on clinico-
I month apart.
pathological, immunological, and bacrereological parameters,
Tieatment failure A patient who, while on ffearment, remained classifies leprosy inro:
smear-positive or became smear-positive again at 5 months or o Indeterminate
later after the start of rreatmenr. Or A patient who was initially o Determinate
smear-negative and was found smear-positive ar rhe end of the
second month of treatment.
,r Polar (stable) leprosy:
leprosy (TT)
Default A patient whose treatmenr was interrupted for -Tirberculoid
Lepromatous leprosy (LL)
2 consecutive monrhs or more after completion of trearment o -Borderline (unstable) leprosy
for I month or more. Borderline tuberculoid leprosy (BT)
- Borderline leprosy (BB)
- Borderline lepromatous leprosy (BL)
DRUG. RESISTANT TU BERCU TOSIS -
Types of drug resistance:
CIINICAI FEATURES .

o Depending on the number of drugs having resistance
against them:
o Monoresistance: Resisrance to one type of drug (e.g., iso-
niazid).
o Multidrug-resistanr TB (MDR-TB): This is a subcat-
egory ofpoly-resisrance. TB resistant to ar ieast isoniazid
and rifampin.
,r Extensive drug-resistant TB (XDR-TB): This is a sub-
category of MDR-TB. XDR-TB is defined as MDR-TB
plus resistance to quinoione and an injectable second,
line drug (kanamycin, capreomycin, etc.).
Skin lesions: Macules, plaques, and nodules that can be
hypopigmented, anesthetic/hypoesthetic. Skin appendages (hair,
sweating) on the lesions are reduced, and there is epidermal
atrophy.
Nerve involvement: Nerves can be thickened and tender, and
there may be associated sensory and motor impairmenr.
Acid-fast bacilli (AFB): AFB can be demonstrated in
some
forms (usually LL, BL, and less frequently BB).
Lepra reactions: Two rypes of acute episodes are seen in
course oF leprosy.

T[berculosis that is sensitive to all drugs is called . Type I lepra reacrion:

pansusceptible TB. o Is due to alteration in host's CML
o Depending on the way resisrance is acquired: c Occurs in borderline specrrum (BI BB, BL).
o Acquired or secondary resisrance: Resistance to one or o It can be an upgrading (reversal reacrion) with
improvement of CMI or a downgrading reaction (as seen
more anti-TB drugs, which arises during the course of
in natural course of disease) when CMI decreases.
treatment, usually due to non-adherence to the recom-
mended regimen or due ro incorrecr drug prescription
o Characterizedby edema and erythema of preexisting le-
sions and neuritis, which may result in sensory and mo,
and inrake.
tor impairment.
o Primary resistance: Presence of resistant strains of M. tu-
berculosis in parients who have been infected with resis- . Type 2lepra reacrion (erythema nodosum leprosum):
tant bacilli by another parient and subsequently develop o Is an immune complex reaction.
the disease. o Occurs in BL and LL.
o Depending on the rrearmenr history: o Characterized by several tender erythematous, transient
nodules on face, flexures, and legs. It is also associated
\
o Resistance among new patients, i.e., patients who were 1
never treated before or were treated for maximum
with neuritis, orchitis, iridocyciitis, arthralgia, and fever. \
1
l month. The profile of different clinical rypes of leprosy is discussed
!
r Resisrance among re-rreatment patients. in Thble 21.16.
\
til
il I
 IMMUNIZATION AND INFECTIOUS DISEASES

Table 2'l .16: Clinical Profile of Spectrum of Leprosy

Skin lesions
Number Single Single/few Few Several Numerous lnnumerable
Size Variable Variable large
May be Variable Small Small
Sensations Variable Anesthetic Hypoeslhelic Hypoeslhelic Hypoesthetic Normoesthetic
Symmetry Asymmetrical Asymmetrical Asymmetrical Bilateral.but, Tendency to Symmetrical
asymmetrical symmetry
Morphology AIways Matule/plaque; Pl.rques; well- Macules/plaque; Macules/papules; Macu Ies/papu Ies,
macule on face oi well-defined delined with wilh sloping edge nodules, plaques; nodules, plaques,
ch ild salellile lesions tinverted saurer ill-defined ill-defined
appearance) Diffuse infiltration
o{ face (leonine
iacies)

Singletrunk/ieeder Asymmetrical.Few Asymrnetrical. Almost Symmetriial.

nerve related to nerves thickened. Several nerves asymmetrical. Several nerves
lesion thir kened' Anestheria in thickened Several nervec thickened. Clove
nodular distribution of thickened. Clove ancl stocking
nerve and stocking anesthes i a
anesthesi a

Systemic Lyrnphadenopathy,
involvemenl hepatosplenomegaly, ocular and
tesl icular involvement

Reactions Stable Type-l Type-l Type-1Aype-ll Type-ll

Lepromin +/- + +/-
AFB +/- +l- +/++ +++

\FB, acid-fast bacilli; TT tuberculoid; BT, borderline tuberculoid; BB, borderline; BL, borderline lepromatous; LL, lepromatous.

coMPucATroNs pathognomonic of leprosy. A full-thickness skin biopsy from an

active lesion (stained with both a standard histologic stain and
a Tiophic ulcers. an acid-fast stain) is the optimal procedure for confirmation of
a Deformities: like claw hand, clawing of toes, foot drop and the diagnosis and accurate disease classification. Acid-fast bacilli
saddle nose of deformity. are rarely found in patients with indeterminate or tuberculoid
Ophthalmologic complications: Dirninished corneal sensa- disease, but the presence of granulomas and lymphocl'tic infiltra-
tion, lagophthalmos, and recurrent iridocyclitis. tion of nerves in anesthetic skin lesions confirms the diagnosis.
Renal involvement. For purpose of assigning patients to the appropriate V&{O
multidrug regimen, slit skin smears are assessed to determine
whether patients have paucibaciliary infection (<5 skin lesions
INVESTIGATIONS
and no bacilli on skin smears) or multibacillary infection (>6
o Slit skin smears: Slit skin smears are taken from the skin lesions and bacilli on skin smears). The bacterial index
lesions, and ear lobules. flese are stained with modified can range from 0 (no bacilli in 100 oil-immersion fields) to
Ziehl-Neelsen method. 6+ (>1000 bacilli per field). PCR detects M. leprae oniy wher-r
o Lepromin test: Lepromin test is of prognostic value. biopsy specimens are also positive fbr acid-fast bacilli.
o Histopathology: Granulomas and neural involvement are Note:
seen. Bacillarv index: lt is a semiquantitativc e'timltion of the density of
r PCR for M. leprae. bacilli present in the skin smears and biopsies :rnd is measured on nvo
scales, namelv tl-re Dharmendra scale and Ridely scale. It measures the
total acid-fast bacilli in microscopic field.
DIAGNOSIS
The critical factor in the diagnosis of leprosy is to consider this TREATMENT
in the differential diagnosis of a skin disorder in anyone who has
:esided in an endemic leprosy region. Anesthetic skin lesions r The patients (and parents) need reassurance, counseling
"'.'rrh
or u,ithout thickened peripheral nerves are virtually regarding treatment and care of hands, feet, and eyes.
ESSENCE OF PEDIATRICS

Table 2'1.17:, WHO Recommendation for Treatment of Leprosy in CASE DEFINITIONS OF MATARIA
.l
Children Aged 0-1 5 Years
I. Falciparum malaria
Definition 5 or < Iesions ? >5 lesions ?
a. Uncomplicated malaria (UM)
Diagnostic criteria:
Duration of therapy 6 months o[ treatment I 2 months to be
to be completed in q completed in 18 o Fever or H/O fever within last 48 hours, and
monlhs monlhs
o Absence of convincing feature of any other febrile illness,
Drugs
and
Supervised (monthly) Rifampicin 450 mg Rifampicin 450 mg o High index of suspicion: Based on dme, place and person.
+ Clofazimine 150 mg
Diagnosis is confirmed by presence of asexual form of
Unsupervised (daily) Dapsone 50 mg Dapsone 50 mg
Plasmodiurnfalciparum in blood slide examination (BSE)
+ Clofazimine 25 mg
'ln children younger than 10 years, dose or rapid diagnostic test (RDT).
is given according to weight. Rifampicin:
l0 mg/kg; clofazimine: 1 mg/kg daily, 6 mg/kg monthly; dapsone: 2 mg/kg body
b. Seaere malaria (SM)
weight.
Diagnostic criteria:
Table 21.18: Treatment of Reactions
o Fever or H/O fever within last 48 hours and
o One or more of the following ciinical or laboratory
Mild NSAIDs NSAIDS features oF severiry:
Moderate \SAlDs NSAIDS o Clinical features:
Oral corticosteroids Thalidomide*
A change of behavior, confusion, or drowsiness
Chloroquine
Clofazimine
- Altered consciousness or coma (cerebral malaria)
- Generalized conr.rrlsions (>2 episodes in 24 hours)
Severe NSAIDs Thalidomide'
- Acidosis
Oral t orlicosleroicls Corticosteroids
Antimony - Difficulty in breathing or acute pulmonary
(parenteral) - edema and adult respiratory distress syndrome
'Not to be used in the reproductive age group.
Oliguria or acute renal failure (<17 ml/hr or
- <400 mll24 hr and 0.3 ml/kg/hr in children)
r For the purpose of treatment, ieprosy is classified into pauci- Severe anemia
bacillary and multibacillary leprosy. Multidrug therapy (MDT) - Circulatory collapse or shock (algid malaria)
is instituted based on number of lesions (Table 21.17). -
Jaundice (clinical)
r Newer drugs: Newer drugs are required in case of resis- - Bleeding tendency or abnormal bleeding
tance or intolerance to conventional therapy. These include - Severe prostration, i.e., exrreme weakness for which
ofloxacin, sparfloxacin, minocycline, and clarithromycin. - the patient cannot walk, stand, or sit without
o Treatment of reactions: Acute lepra reactions may be ofvari- assistance and in case of small child unable to eat
able severity and are managed as depicted in Table 21.18. Severe vomiting leading to non per os
-
,r Laboratory findings:
PREVENTION Hypoglycenia (<2.2 mmol/L or <40 mg/dl)
- Severe normogztic anemia (Hb <5 g/dl, PCV < 15%)
o No effective vaccine. -
r BCG booster vaccination (2 dose BCG regimen) provides Hemoglobinuria
_ Fluid and electrolyte disturbance (hyponatremia)
50-7 5o/o protection against leprosy.
Hyperparasitemia (>5%)
- Metabolic acidosis (bicarbonate <15 mmol/L)
PROGNOSIS - Renal impairment (serum crearinine >3 mg/dl)
Excellent Progression of tissue and nerve
with treatment. and
damage does not occur, but recovery of lost sensory and motor Presence of asexual form of P falciparum in BSE
- or positive RDT for P falciparum
function is incomplete.
II. Vivax Malaria (VM)
Diagnostic criteria:
Maiaria is a paroxysmal febrile illness caused by a protozoa o Fever or H/O fever within last 48 hours, and
(P falciparum, P. uiuax, less commonly by P ouale and P o Absence of convincing features of any other febrile illness,
t
ntalariae).It occurs usually following the bite of an infected and
female anopheline mosquiro. o High index of suspicion: Based on time, place and person.
\
a
t
t
 !

IMMUNIZATION AND INFECTIOUS DISEASES

Diagnosis is confirmed by presence of asexual form of P. uiuax Table 21.19: Drug and Doses
in BSE or positive RDT.

MODIFIED GTASGOW COMA SCATE 1st Ohr 1 2 3 4

.l
(usuArrY FoR ADUTTS) Day
2nd Bhr I 2 \ 4
Artemether+
For assessment o{ grade of coma. Lumefantrine Jrd 24hr t ) 3 4
. I)av I
Eyes open Score
combrnatron 4lh J6hr I ) i 4
(ALC)
Spontaneously 4 5th 48hr 1 2 3 4
Day 3
To speec h 3 6th 60hr 1 2 3 4
To pain 2
Artemether + Lumefantrine combination is well-absorbed rvith f.rtty meals, so patient
No response 1 should be encouraged to have it with rnilk or fatty meals.
Best verbal response
Non-intubatecl o First-line treatment Artemether + Lumefantrine com-
Orienred and talks 5
bination (ALC)-6 doses over 3 days (Table 21.19).
Disoriented and talks 4
o If for any reason Artemether + Lumefantrine combina-
lnappropriate words t
tion cannot be given, alternative treatment may be:
Incomprehenriblesounds 2

No response 1
,r Quinine 7 days + Tetracycline 7 days (Q/ *T7), Or
I ntubated r Quinine 7 days + Doxycycline 7 days (Q7 * D7), Or
Seems able to talk 5 r Quinine 7 days + Clindamycin 7 days, Or
Questionable ability ro talk 3 ,r Other WHO recommended treatment (Artesuna-
Cenerally unrespon\ive I te-mefloquine) when available.
Best motor response Note:
Verbal commands 6
- Tetracycline and dorTcycline are contraindicated in children
Localizes pain 5 <8 year old and pregnant and lactating woman.
Withdrawal to pain 4 - Quinine is given at a dose of 10 mgikg B hourly for 7 days.
Decorticate 3 - Clindamycin is given at a dose of 10 mgikg mice daily for 7 days.
Decerebrate 2
o Use of gametocytocidal drug to reduce transmission:
None
r
1
A single orge should not be given in pregnancy and
Total 3 15
in children <4 year old.
Total score = eye opening score + verbal score + motor score.
Total score ranges from 3 to 1 5, Unarousable coma reilected in a score of <9 b. Severe malaria (SM)

Blantyre coma scale for children: o Pre-referral treatment: IM Quinine/rectal Artesunate:

Eye movements: Directed (e.g., follows mother's face) I : Quinine dihydrochloride:20 mglkg stat IM should
Not directed 0 be given with half dose in each thigh.

Verbal response: Appropriate cry 2 t Artesunate rectal ca?sule: 10 mg/kg.

Moan or inappropriate cry I o Immediate referral should be made to the nearest
health facilities.
Nonc 0
Best motor o Hospital treatment: Perenteral treatment is either:
response: Localizes painfulstimulus" 2
t M Artesunate: 2.4 mg/kg stat followed by 2.4 mglkg
Withdraws limb from painb 1
daily untii patient can tolerate oral medication, Or
Non-specific or absent response 0 > IM Ammaher:3.2 mglkgstat followed by 1.6 mg/kg
Total 0-5 daily until patient can tolerate oral medication, Or
These scales can be used repeatedlv to assess improvement or > Quinine dihydrochloride: 20 mg salt/kg of body
deterioration. weight (loading dose) by infusion over 4 hours in
a. Rub knuckles on patient's sternum. 5%o dextrose saline (5-10 ml/kg of body weight
b. Firm pressure on thumbnail bed with horizontal pencil. depending on the patient's overall fluid balance).
Maintenance dose: Eight hours after the start of
Total score can range from 0 to 5;2 or less indicates unarous-
the loading dose, maintenance dose of quinine
able coma.
10 mg salt/kg of body weight in dextrose saline
diluted as above should be given over 4 hours. This
f TREATMENT
r maintenance dose should be repeated every 8 hours
2 I. Falciparum malaria (FM) till the patient can take quinine orally (Total dose of
F a. Uncomplicated malaria (UM) Quinine will be given for 7 days).
?
t
It
ESSENCE OF PEDIATRICS

; Follow on tretttment: Following initial perenteral
treatment, once the patient can tolerate oral ther-
apy, it is essentiai to continue and complete treat-
ment with an effective oral anti-maiarial drug:
Artemether plus Lumefantrine, Or
- Artesunate plus clindamycin or doxycycline, Or
- bacterial meningitis, encephalitis). Cive
Quinine plus clindamycin or doxycycline or
- tetracycline.
r Use of gametocytocidal drug to reduce transmission:
,r Asingle oral dose of 0.75 mg base/kg ofprimaquine
is to be added at the end of treatment with Arte-
mether + Lumefantrine combination or Q + T7 I
D7lChn7.
r Primaquine should not be given pregnancy and
in children <4 year oId.
II. Vivax malaria (VM): If BSE and/or RDT is positive for
P uiuax:
o Chloroquine 3 days + Primaquine 14 days (CQ3 + PQ14)
ll Chloroquine (CQ):
lst day: 10 mg/kg (4 tab for adult)
- 2"r day: 10 mg/kg (4 tab for adult)
- 3"r day: 5 mglkg (2 tab for adult)
- Correct arterial pH.
r Primaquine (PQ):
0.3 mg/kg daily for 14 days for children
- I tab daily for 14 days for adult (1 tab = 15 mg)
- stop intravenous fluids, give diuretic
Management of severe complicated falciparum malaria has
been summarized in Table 21.20.
Kala-azar or visceral leishmaniasis is an infection commonly
caused by Leishmania donouani, transmitted by an infected
female sand fly. Phlebotomus ltryentipes.
EPIDEMIOTOGY
Incubation period usually 3-6 months, which may range from
10 days to 2 years.
CLINICAT PRESENTATION OF KALA-AZAR
A. Classical presentation
1. Fever: Usually insidious and may be associated with
Table 21.2Ot Summary of the Management of Severe and
Complicated Falciparum Malaria
Coma (cerebral Maintain airway, nurse on side, exclude other
malaria) treatable Laurps of t ofird re.g., hypoglycemia.
prophylaclit anticonvulsant r Phenobarbital
sodium l0 mg/kg of body weight,
intramuscularly). Avoid harmful adjuvanl
lredtmenls such as corticosteroid, heparin.
and epinephrine.
Convulsions Prevent with phenobarbital sodium.
Maintain airway; treat with diazepam
given intravenously or per rectum or lM
paraldehyde injection (0.1 ml/kg from a glass
syringe).
Severe anemia Transluse tresh whole blood or packed cells.
Acute renal failure Exclude dehydration, maintain strict fluid
balance, carry out peritoneal dialysis (or
hemodiall sis if availabler.
l-lvDoslvcemra Measure blood glucose, give 25% glucose
injection 50 mg (2 ml/kg for children)
followed by 5'k or 107o glucose infr-rsion.
Metabolic acidosis Exclucle or treat hypoglycemia, hypovolenria
and gram-negative septicemia. Cive oxygen.
Acute pulmonary Prevent by avoiding excessive rehydration.
edema Prop patient up, give oxygen. lf the
pulmonary edema is due to over hydration,
(frusemide 40 mg intravenously) and
withdraw 250 ml of blood by venesection
into a donor bag.
Shock, algid malaria Suspect gram-negative septicemia, take
blood samples for culture. Cive parerrteral
antimicrobials, correct hemodynamic
disturbances by normal saline.
Spontaneous bleeding Transfuse fresh whote blood or clotting
and coagulopathy factors. give r itamin K iniecLion.
Hyperpyrexia U.e lepid spongrnB and fanning: gir e
antipyretic (paracetamol 1 5 mg/kg of body
weight).
Hyperparasitem ia Cive initial dose of parenteral antimalarial
therapy if parasitemia in a severely ill patient
.1
exceeds 07o carry out exchange or partial
exchange transfusion.
Malarial Conti nue antimalarial treatment; trans{use
hemoglobinuria fresh blood to maintain hematocrit above
2O'k; give frusemide 20 mg intravenously.
Aspiration pneumonia Cive parenteral antinricrobials, change
position of patients, give physiotherapy; give
oxrvgen_

chills and rigor. Fever intensiry decreases over time,

and patient may become afebrile for weeks to months 1. Bleeding manifesrarion, e.g., episraxis, GIT bleed-
foliowed by relapse of fever ing. This occurs as a result of thrombocytopenia and
2. \Teight loss heparic dvsfuncrion.
3. Swelling of upper abdomen, especially left side 2. Features of secondary infection.
4. Increased pigmentation-2 fs21u1s of advanced disease C. Atypical presentation
B. Features of complication 1. Subclinical-No fever
 IMMUNIZATION AND INFECTIOUS DISEASES

Signs: CtINICAI CASE DEFINITION OF KAIA.AZAR

A. General examination: AND PKDL (WHO: 2008)
1. Raised temperature There are three case definitions:
2. Anemia: Moderate to severe; may result from bone
marrow infiltration, hypersplenism, autoimmune o Kala-azar (KA):
hemolysis, and bleeding Fever or history of fever for 2 weeks or more:
3. Gum bleeding and epistaxis
1. Irregular pattern of fever
4. Lymphadenopathy: More common in Africa, less in
And
India
Splenomegaly
B. Abdominal examination And
1. Splenomegaly in 10070 cases. Develops quickly in 2. High index of suspicion based on residing/traveling in
the first few weeks and become massive as the disease endemic area
progresses. And
2. Hepatomegaly 3. Absence of convincing evidence of any other febrile illness
3. Ascites, edema, anasarca in progressive disease, cause
And
by hypoalbuminemia. 4. rk39 test positive
o Kala-azar treatment failure (KATF):
Clinicol Presenlolion of PKDL 1. Earlier diagnosed as kala-azar
And
PKDL usually develops 6 months to 5 years following an
attack of untreated or incompletely treated visceral leishmani-
2. Took complete treatment
And
asis. Howeve r, I5o/o cases of PKDL occur without the preced-
ing history of kala-azar. They have only skin lesions that are 3. Again features of kala-azar
varied. The lesions may be macular, papular, nodular, or mixed. And
Sometimes, the lesions of PKDL are extensive. Overlapping 4. Any positive lab evidence: Bone marrow/Splenic aspiration
of three stages (macular, papular, and nodular) can occur in And
an individual simultaneously. In PKDL cases, sensation over 5. Period: \Tithin 1 year
the lesions is preserved in contrast to leprosy where similar o Post Kala-azar Dermal Leishmaniasis (PKDL):
lesions have no sensations or less sensation. Though PKDL is
1. Multiple hypopigmented areas on skin without loss of
a rare condition (1 in 50-100 cases of kala-azat), it is epide-
sensatl0n
miologically important because they act as the main reservoir
V(/ith or without
of infection. In PKDL cases, the parasite concentrates in the
skin lesions and is readily available to the insect (Phlebotomus)
2. Any one or combination or all
a) Macule
vecror when it bites the patient.
b) Papule
I
The clinical manifestadons of these dermal lesions may be
c) Nodule
I of three types:
d) History of kala-azar
I

t o Macular lesion And

I o Papular lesion ^). High index of suspicion based on residing/traveling 1n
I o Nodular lesion endemic area
I
L. And
I
4. rk39 positive
I COMPTICATIONS OF KAIA.AZAR
I
t Secondary infections: Pneumonia, pulmonary tuberculo-
Cqse Definilions for RePorting
I sis, amebic or bacillary dysentery, gastroenteritis, herpes
t zoster, chicken pox, skin infection (boils, cellulites, scabies), Suspected kala-azat: Patients with fever (of >2 weeks) from
an endemic area who has one or more of the following
I
cancrum oris, septicemia, otitis media.
features: (l) splenomeg aby, (ii) anemia, and (iii) weight loss'
I
L
l a Bleeding manifestation: From nose, GIT, retina, etc.
t a Post kala-azar dermal leismaniasis (PKDL). Confirmed kal the suspected case is confirmed
^-atLtr,\'ilZhen
; a Post kala-azar laryngitis and colitis. by positive rk39 or demonstration of parasite in the tissue
t"
a Post kala-azar splenomegaly. (BM/SP) or by PCR.
I o
a Renal: Glomerulonephritis, nephrotic syndrome. Kala-azar treatment failure (KATF)
a Cirrhosis of liver. o PKDL

I
 .r

ESSENCE OF PEDIATRICS

DI FFERENTIAT DIAGNOSIS In case of missed doses, the scheduled 28 doses may be

taken within a peliod of 35 days. The daily dose should never
Malaria, leukemia, lymphoma, CLD, portai hypertension, exceed the recommended amount.
congenital hemolytic anemia, tuberculosis, srorage disease.
The doses should be calculated as follows:
o >12 years and weighing >25 kg: 100 mg (Cap. 50 mg in
INVESTIGATIONS
morning and in evening)
1. Indirect evidences: o >12 years but weighing <25 kg: 50 mg (Cap. 50 mg in
a) Hematological changes: Anemia, raised ESR, leuko- the morning)
penia (neutropenia with relative lymphocytosis and o 2-12 years: 2.J mg/kg body weight (in two divided dose
monocytosis), thrombocytopenia. not exceeding 15 mg/d)
b) Biochemical inaestigations (according to need): If an exact dose cannor be administered, the closest 10 mg
Serum bilirubin, SGOT, SGPT serum albumin, serum increment will be chosen at the dose.
globulin, urea, crearinine.
c) Serological:
i. Rapid dipstick 'rk39' test (lCT for kala- Treolment B
azar) having about 100o/o sensitiviry and 95o/o If Miltefosine is nor available or in KAIF, sodium antimony
specificity. This test may become positive for gluconate (SAG).
2 years after completion of kala-azar rrearmenr. Ir
is a realable tesr for diagnosis ofkala-azar in field
Dose and duration: 20 mg/kg IM/IV for 30 days
situatio n.
ii. Direct agglutination rest (DAT)
iii. Indirect fluorescenr antibody test (IFAI), CFT Upto3 0.5
iv. ELISA 4,5 1

v. Napier aldehyde test 6-B 1.5

2. Direct evidences: 9-1 0 2
a) Demonstration of parasites is the most conclusive
11-1 3 2.5
evidence in the diagnosis of kala-azar and pKDL.
14-15 3
Parasites are detected by microscopic examination
of smear prepared from (z) bone marroq (ll) splenic 16-18 l-)

aspirares, (iii) butry coat, (iu) liver biopsy, (z) lymph 1 9-20 4
node biopsy, (zz) skin lesions. 21-2i 4.5
b) Culture of the biopsy material in different media like 24-25 5
NNN media, Eagle media, etc. for isoiation of parasite. 26*28 5.5
c) PCR for DNA detection of LD from the peripheral 29-30 6
blood and tissue.
31-35 7
Results of smears (amastigote form) or cultures done in NNN 3 6-40
blood agar media (promasrigore form of LD body) of materi- >41 8,5
als from spleen, bone marrow, or lymph node aspirations are
usuaily diagnostic:
Site Sensitivity Treqlmenl C: Second-Line Treqlment for
Spienic puncrure 95-9To/o Kolq-Azor
Bone marrow 55-85o/o Indication: Kala-azar rrearmenr lailure (KATF) and in case
Buffy coat prepararion 70o/o
of pregnancy.
Lymph node 70o/o a) Amphotericin-B and Deoxycholate (non-liposomal):
1 rng/kg bodv r.r.t in
solution W daily for 20 days.
5%o dextrose

TREATMENT
b) Amphotericin-B (liposomal): 3 mg/kg/d IV bodywt daily
tor ) days.

Ereatmenl A
Treotmenl D: Treolmenl of PKDt
Miltefosine is the first-line rreatment for kala-azar.
a) Sodium antimony gluconate: 20 mglkg body wt IM/
Dose: 2.5 mg/kg body weight, w,'ice daily by mouth in the IV daily for 20 days per cycle. Six cycies with 10 days
morning and evening after meal for 28 days. interval between cycles.

\
\
 IMMUNIZATION AND INFECTIOUS DISEASES

:
H/O fever for >2 wks
I

I
I
t Hypopigmented patches Splenomegaly
ls the patient pregnant?
I (macule) without loss of Weight loss
I sensation with or without Anemia
I
. Erythematous patch
t (papule)
I . Sub-cutaneous nodule
I . Fever or H/O KA
t.

I
I
6
I Yes *
&

l.
****#
I
v
s
Record as Kala-azar and
rk3e positive use Treatment A or B
ffi
s##ffie@Fs@
according to availability
I
v
Record as PKDL and u
Treatment D

Record as KATF and use $ _ .,^^

ooF Was complete treatment
Treatment c Fq**- ' g*4:*** with Miltefosine or sAG
*S -****J within last year?
done within vear?

tig.21.4: Diagnosis and management chart for kala-azar and PKDL

b) Amphotericin-B and Deorycholate (non-liposomal):
1 mg/kg in 5olo dextrose solution IV daily for 15 days.
Six cycles with 10 days interval benveen cycles.
.) Amphotericin B (liposomal): 3 mg/kg in 570 dextrose
solution IV daily for 5 days. Six cycles with 10 days
interval between cycles.
Algorithmic approach to the diagnosis and management of
kala-azar and PKDL has been depicted inFig.2l.4.
Inflammation: Patient may have acute illness with fever'
lymphangitis, lymphadenitis, orchitis, epididymitis, Iymph
edema, and delirium (filarial septicemia).
Obstructive phase: Usually follows repeated attacks' Ele-
phantiasis of the affected part (lower limbs and genitalia)
is the most remarkable manifestation. Chyious ascites,
chyluria, or collection of milky fuid in other body cavities
may also occur.

DI FFERENTIAL DIAGNOSIS
Filariasis is caused by W bancrofii and B. malayi transmitted by Congenital lymphedema, venous thrombosis, angioedema,
biting of Culex mosquitoes through which filarial larva enters disease causing generalized edema.
into the body. Larva develops into adult worms and resides in
lymphatics. 'Worms become sexually mature and release large
number of microfilaria in blood. INVESTIGATIONS

CLINICAT FEATURES
o Initial or phase of invasion: Characterized by the pres-
ence of transient urticaria, fever, myalgia, lymphadenitis,
and eosinophilia.
o Leukoqtosis and high ESR. Total eosinophil count varying
berween 4ooo and 50,000/mm3.
o Peripheral blood film for microfilaria (blood taken at mid-
night).
o Detection of microfilarial antigen or antibody.

ESSENCE OF PEDIATRICS
TREATMENT
1. Symptomaticmeasures include antipyretic, anrihisramine.
2. Diethylcarbamazine (DEC)
o 1 mg/kg single dose-Dl
r 3 mg/kg in 3 divided doses-D2
. 3-6 mg/kg in 3 divided doses-D3
o 6 mg/kg in 3 divided doses-D4-l4
Repeated courses may be needed for complete parasitic
cure. Practiced on mass basis; a single dose of DEC of
3-6 mglkggiven once or twice a year reduces microfilaria
density by 80-90%.
3. Single-dose ivermecrin, a macrolide anribiodc, 20-200 mglkg
for children >15 kg is also effective in iowering microfilaria,
but it is associated with high recurrence rate (540o/o).
Both beef tape worm (Taenia saginata) and rhe pork (Taenia
solium) cause taeniasis. Infection acquired by ingestion of raw
or inadequately cooked infected beef or pork meat. Man is
definitive host of taenia and adult worms live in intestine.
CtINICAt FEATURES
Infection may be asymptomaric or may present with epigastric
discomfort, abdominal pain, perianal pruritus, abdominal dis-
tension, loss of weight, and growth retardation. Most parents
bring their child for passing l-2 cm long segments (proglottis)
in stool or crawling over perianai area.
DIAGNOSIS
Naked eye examination of stool for gravid segment (proglot-
tides). Rectal swab microscopy for detecting eggs, as eggs are
often absent in stool.
TREATMENT
Praziquantel 5-10 mg/kg PO single dose is trearmenr
of choice. Symptomatic neurocysticercosis is treated with
albendazole 15 mglkgld for a month.
Niclosamide I g PO for children berween 1l and 35 lbs
and 1.5 gfor >35 lbs is effecdve parricularly for T saginata.
Hydatid disease develops when hydadd cyst is formed following
infection with larval stage of canine rape worm, Echinococcus
granulosus. Human are infected when they ingest food or warer
contaminated with eggs or from direct contact with infected
eggs. Hydatid cysts are usually formed in liver and infrequently
in lungs, brain, bones, and spleen.
t
CtINICAI FEATURES
Liver cyst may remain asymptomatic or may present with
hepatomegaly, palpable mass, vomiting, or abdominal pain.
Jaundice is rare. Lung cyst may cause chest pain, cough, or
hemoprysis. Rupture of cyst may lead to anaphylaxis.
DIFFERENTIAL DIAGNOSIS
Other hepatic cysts, liver abscess, neoplasm.
INVESTIGATIONS
USG and CT scanning, Casoni test,Antibodydetection byELISA.
TREATMENT
Albendazole 15*30 mg/kgld for 2 weeks, repeated for 4
courses with 2 weeks drug-free interval berween rwo courses.
Surgical removal can be contemplated for a large solitary
cyst following albendazole therapy.
Giardiasis is caused by Giardia lamblia, a protozoan parasire
that colonizes the human small intestinal lumen. The parasite's
lifecycle comprises rwo srages-morile trophozoites and thick-
walied ellipsoidal cysrs thar are excrered in the feces. New hosts
acquire the infection by ingesting G. lamblia cysts; exposure of
cysts to gastric acid leads ro emergence of trophozoites from
the cysts. Tiophozoites encysts in the small intestinal lumen;
and the resulting cysts are excreted from the host.
G. lamblia infection is usually acquired by drinking water
that contains cysts.
CTINICAT FEATURES
G. lamblia infection can be asymptomatic and can also cause
a variety of clinical problems. These include warery diarrhea,
steatorrhea, and weight loss. Other clinical fearures include
nausea, abdominal cramps, diminished apperite, abdominal
distension, and passage of foul smelling flatus per recrum.
Giardiasis does not lead to gastrointestinal bleeding. Untreated
giardiasis typically lasts for several weeks. Occasionally, mega-
loblastic anemia may occur following impaired absorption of
vitamin B,, or folic acid.
:
DIAGNOSIS
The organism is pear shaped, with rwo oval nuclei and four
pairs of flagella. Cysts have a thick wall around four small
1
nuclei. The cysts may be found in the stools, but more often in
1
duodenal aspirates. Duodenal or jejunal biopsy will sometimes t
reveal the organism when aspirates are negative. t
1
I
ar

Table 21.21:. Drugs Commonly Used in the Treatment o{
Ciardiasis
Metronidazole 15 mg/kg 7 days Divided in 3 doses/d
Tinidazole 50 mg/kg )rngle dose
l-urazolrdone 5 mg/Kg 7-1 0 days Divided in 3 doses/d
/
Qulnacnne 5 mgiKS oays Divided in 3 doses/d
TREATMENT
Commonly used drugs include metronidazole, tinidazole, and
furazolidone. Recently, albendazole has been used with some
success (Table 2I.21). The most effective drug quinacrine is
generally not tolerated weil by children.
The question whether to treat or not to treat asymptom-
atic G. lamblia infection has generated some controversy. The
current consensus is that asymptomatic patients or cyst excre-
tors should be treated.
PREVENTION
\7ater can be filtered through sand or earth. The cysts can
also be removed from water by membrane filters that have
a pore diameter of <5 mm. Giardia cysts in water are killed
by boiling.
Amebiasis is caused by Entamoeba histolytica, an intestinal
protozoa. The infection is spread by infective cysts in stools
that can contaminate food and drinking water. Cockroaches
and house flies can spread the cysts. Tiophozoites penetrate
the mucous membrane of the bowel in regions of relative
stasis such as cecum, appendix, and colon. A small abscess
forms and may ulcerate. Amebae can enter the portal vein
and lodge in one or more areas of the liver, or travel to
lungs and pleura.
DIAGNOSIS
Most infected individuals are asymptomatic (90o/o). Symptoms
occur when tissue is invaded and a regional ulcerative colitis
results. Patients usually present with acute dysentery; diarrhea
with blood and mucus, pain, tenderness, chronic nondysenteric
diarrheas, chronic amebic colitis is clinically indistinguishable
from infammatory bowel disease, hepatic abscess, lung abscess,
and ameboma.
Diagnosis is confirmed by trophozoites (with RBC), cysts
in stool or trophozoites in abscesses; serologic evidence of
amebic infection. Three separate stool examination have 90o/o
sensitiviw for the diagnosis of amebic dysentery.
I
IMMUNIZATION AND INFECTIOUS DISEASES
TREATMENT
1. Asymptomatic: Diloxanide furoate 20 mglkgld in three
divided doses for 10 days.
2. Intestinal amebiasis or hepatic abscess:
o Metronidazole 30-50 mglkgld in three divided doses
for 10 days.
o Tinidazole 50-60 mg/kg, single dose for 3 days.
This treatment should be followed by intraluminal cysticidal
agent, i.e., iodoquinol or diloxanide furoate.
This common infection is caused by Ancylostoma duodenale or
Necator americAnus, nematodes that live in the human intestine
and produce chronic blood loss giving rise to impaired growth,
PEM, anemia, hypoalbuminemia, and heart failure. Some
patients may present with ground itch, Loeffier syndrome.
NATURAT HISTORY
The adult worms reside in the jejunum and feed on blood
(0.05-0.5 ml/worm/d). They expel ova into the feces. The
eggs hatch in damp shady soil and become infective larvae in
about 2 weeks. They can then penetrate the skin of bare feet,
enter the bloodstream, go to the lungs, exit through alveolar
walls, migrate up the tracheobronchial tree, and be swallowed.
With hear,y infection, pulmonary lesions may be evident, ancl
cough and dyspnea may occur. Itching of the soles of feet and
between toes may indicate entrance of the larvae.
DIAGNOSIS
Ova can be seen in fecal smears. Occult blood in stools
common, as are hypoalbuminemia and eosinophilia.
TREATMENT
Mebendazole (100 mg bid for 3 days for children over
2 years) is effective not only for hookworms, but also for
ascariasis, enterobiasis, and trichuriasis. It is contraindicated
in pregnancy. Pyrantel pamoate is given in a single oral
daily dose of 11 mg/kg for 3 days. Albendazole and thia-
bendazole are useful for creeping eruptions.
Iron therapy with t blood transfusion.
Infection with the nematode Ascaris lumbricoides gives rise
a constellation of symptoms:
a) \forms: Malabsorption, malnutrition, vit. A deficiency,
may obstruct or perforate the small bowel, biliary system
(biliary ascariasis), or appendix.
 5
ESSENCE OF PEDIATRICS

b) Larvae: Acute transient eosinophilic pneumonia (Loeffier gravid female migrates ro the anus to deposit eggs, which are
syndrome). infective and may survive several weeks outside the body. Re-
c) Nonspecific: Anorexia, vomiting, abdominal pain, rarely infection by hand contamination is common.
diarrhea.
d) Usually remain as asymptomatic.
CtINICAI FEATURES

NATURAT HISTORY
The worm is normally located in the small intestine. Its life rycle
depends on the gravid female depositing 200,000 eggs daily,
which require 3-4 weela to become infective. On ingestion, the
larva hatches in the small intestine, penetrates the intestinal wall,
and enters the portal circulation. It is carried to the heart, then
the lungs, where it breaks through the capillaries and becomes a
fourth-stage larva. It then moves up to tracheobronchial tree and
enters the esophagus. \X4ren it reaches the intestine, it becomes
sexually mature, and the rycle is repeated.
Most infected individuals are asympromatic. The major symp-
toms are pruritus ani, vaginitis, which can interfere with sleep.
Occasionally is associated with enuresis. A cellophane tape
should be pressed against the perianal skin (adhesive side down)
when the child awakens. Eggs fasten to the underside of tape
can be seen under a microscope.
DIAGNOSIS
Observing the worms or eggs in stool or perianal skin.

TREATMENT
DIAGNOSIS
o Therapeutic trial is justified without a confirmed diagnosis.
Diagnosis by observing the worms (passed in feces, vomitus o Specific measures: Tieat all household members at the same
or through nose), or ova in stool. time to prevent re-infection. Since the drugs are not effective
against eggs, therapy should be repeated afrcr 2 weeks to
TREATMENT kill the recently hatched adults. Pyrantel pamoate 11 mg/
kg single dose, a single oral dose of 100 mg mebendazole
Mebendazole 100 mg BD for 3 days, pyrantel pamoate or 400 mg of albendazole is used for all ages.
11 mg/kg, single dose, albendazole 400 mg in a single Intractable family infections can be controlled by treatment
dose are highly and equally efFective. Berter to give on of all family members with 100 mg mebendazole a week
empty stomach. for l2 weeks.
In intestinal or biliary obstruction, piperazine 150 mg/kg
initially, followed by 6 doses of 65 mglkg every 12 hour by
NG tube is recommended, because it narcotizes the worms PREVENTION
and helps relief of obstrucrion. tJ7e
treated a number parienrs
Change bed sheet, night cloth daily; cut nail short; careful
of intestinal obstruction with nothing per oral (for 2-4 hand washing.
d); NG suction, IV fuid, antispasmodics, antihelminthics
(pyrantel pamoate, albendazole or levamisole) with excellent
result. Surgery is occasionally required.

PREVENTION Natural habitat of Trichurk trichiura is the cecum and ascend-

Improved sanitation is the essential method of control. 'When ing colon. Infection is transmitted directly by ingestion of
human feces are used as fertilizer, avoidance of green vegetables embryonated eggs in contaminated food, drinks and hands
and salads is advised. and indirectly through fies. Larvae escape from the eggs in
small intestine moving down to lodge in cecum.

CTINICAT FEATURES
May be asymptomatic. Massive worm load may cause growth
It is the most common nematode infection caused by Entero-
retardation, epigastric pain, abdominal distension, hypopro-
bius uermicularis.
teinemia, and uncommoniy anemia (sucks 0.005 ml blood/
worm/d).
NATURAT HISTORY
DIAGNOSIS
These light yellow worms (5-10 mm long) with a pointed tail
inhabit the cecum and adjacent parts of large intestine. The Demonstrating lemon-shaped eggs with mucus plugs in feces.
\
\
1
1
tl

TREATMENT
Mebendazole 100 mg BD for 3 days is associated with70-90o/o
cure rate.
Strongtloida stercoralis infects the skin with infective filariform
larvae or human by autoinfection. 30o/o are asymptomatic. The
rest may present with cutaneous, abdominal or pulmonary
symptoms. Diagnosis is confirmed by seeing larva in stool or
duodenal fluid.
TREATMENT
o For creeping eruption: Thiabendazole (Mintezol cream
I5o/o). 15o/o cream in a water soluble base may be applied
locally4timesaday.
r For adult worms: Thiabendazole (Mintezol 500 mg tab.,
500 mg/5 ml susp.) 50 mg/kg/d orally BD for 2 days, or
mebendazole 100 mg rwice daily for 4 days.
TPE, is a syndrome probably due to filarial infecdon.
Clinical features: There is paroxysmal nocturnal cough,
dyspnea, wheeze, fever, easy fatigability.
Diagnosis: Total circulating eosinophil count > 2000 per
mm3; serum IgE is elevated. CFT for filaria positive. CXR
shows increased bronchovascular markings, discrete opacities,
difruse miliary mottlings.
Tireatment: Diethylcarbamazine 7-I0 mglkg/d q8hr for 2-3
weeks.
American College of Emergency Physicians (ACEP) chooses
a rectal temperature of >38"C (100.4'F) as the most widely
used definition of fever.
Fevers are induced by pyrogens. Bacteria, fungi, viruses,
malignancies, connective tissue disorders, certain drugs and
trauma may endogenously stimulate production of pyrogens.
Common endogenous pyrogens include Interleukin-1 ([-1),
tumor necrosis factor (TNF), and interferon. Exogenous pyro-
gens include lipopolysaccharides, enterotoxins, and exotoxins.
Fever may be beneficial in the sense that it increases host
defense mechanisms, augments immunological functions, and
suppresses growth of some microbes. Howeve! documented
: evidence against use of antipyretic therapy for fever is not
I forthcoming. Fever in children increases the risk of febrile
/ seizure and should therefore be cautiously handled. Fever
r above 41.5'C is called hyperpyrexia and warrants aggressive
7 artipr.retic therapy because of risk of irreversible organ damage.
I
IMMUNIZATION AND INFECTIOUS DISEASES
Acetaminophen (paracetamol), non-steroidal anti-inflammatory
drugs (NSAID), and steroids are effective antipyretics.
SHORT DURATION FEVERS
Most cases of short duration fevers (<2 weeks) are attributed
to infections due to viruses, bacteria, or protozoa.
Respiratory system: Common cold, pharyngitis, acute otitis
media, mastoiditis, acute bronchitis, and pneumonia are the
usual acute respiratory illnesses. These are often viral in etiology.
Ordinarily, in viral infections a number of mucosal surfaces
are involved simultaneously or in quick succession. In bacte-
rial infections, the inflammatory process is usually localized.
Regional lymph nodes are involved. The rate of spread of infec-
tion is slower in bacterial infections. The throat should always
be examined to exclude follicular tonsillitis, pharyngitis, and
diphtheria (suspected if the fever is moderate and the patient
appears toxic).
Flaring of the alae nasi and working of accessory muscles
of respiration and tachypnea indicate lower respiratory tract
involvement.
Meningitis: Marked irritability and photophobia with head-
ache and persistent vomiting should arouse the suspicion of
meningoencephalitis. The patient may show neck rigidity,
positive Kernig sign, neurological deficits, convulsions, stupor,
or coma. The diagnosis should be confirmed by lumbar punc-
ture and examination of the cerebrospinal fluid. Meningitis
and septicemia in the first 4-6 months of life usually have an
atypical presentation.
Urinary tract infections: Infections of urinary tract are
equally common in infancy among both sexes. After infancy,
girls are more vulnerable to urinary tract infections. Symp-
toms and signs referable to urinary tract may be minimal
or absent. The fever may be hectic and intermittent, at
times associated with chills. Some patients may present
with fever, vomiting, diarrhea, and abdominal pain. This
may mislead the physician to focus attention on the gas-
trointestinal system. Urinalysis with culture colony count
must be carried out for the diagnosis of unexplained short
fevers, especially in girls.
Exanthematous disorders: Careful search should be made for
exanthem or enanthem in all cases of fever.
Pyogenic infections: A meticulous search should be made
for the foci of infections in the liver, perinephric and subdia-
phragmatic regions, bones and joints. Long bones should be
palpated for tenderness over metaphyses.
Malaria: til7ith the resurgence of malaria in most developing
countries of Asia, it should always be considered in differential
diagnosis of short term fever.
Typhoid fever: Typhoid fever is endemic in most tropical and
subtropical countries with poor standards of sanitation and per-
 I

ESSENCE OF PEDIATRICS

sonal hygiene. Exanthem ofryphoid fever (rose spots) is usually o Primary influenza virus pneumonia
evanescenr and may be missed in dark skinned individuals. o Secondary bacterial infection
Classical step ladder partern onset, with sustained character of ) StrcptocLtccas pneumonia(
temperature is not always present. Most patients have distended o Staph. aureus
tympanitic abdomen and mild hepatosplenomegaly. o H. influenzae
Other causes: Heat hyperpyrexia, dehydration fever, allergy Non-pulmonary complications:
to drug (drug fever).
o Myositis
o Cardiac complications
o Encephalopathy
r Liver and CNS-Reye syndrome
It is caused by an enveloped influenza virus, virion of which o Peripheral nervous system-GBS
contains an RNA polymerase and rwo types of membrane
transport spikes-H protein (hemagglutinin) and N protein Mortality: Occurs commonly due to bacterial pneumonia and
(neuraminidase). cardiac failure; 90o/o of deaths in those over 65 years of age.
Tiransmission:
o Virus is spread by inhalation of small aerosol droplets TABORATORY DIAGNOSIS
expelled during talking, breathing, coughing, and sneezing.
Case definition: A confirm case is defined as a person who
o Virus is extensively spread by school children.
has an acute febrile respiratory illness with one or more of
Infectiviry: the following:
o Incubation period 2-7 days. o RIPCR
o Period of suffering 5-7 days;10+ days in children. o Vral culture
r Period of communicabiliry: 7-10 days, more on first 3-4
days and from 1 day before onset of symptoms.
PATTENT MANAGEMENT (WHO 200?)
Clinical features:
(Fig. 21 .5)
Abdominal pain, diarrhea, vomiting, fast breathing, lower chest
indrawing, grunting, stridor. Category A: Uncomplicated influenzalinfluenza-like illness
(ILI) not in'risb goup'.
coMPUCATTONS
Category B : Uncomplicated infl uen za I inflienza-like illness in
'rish group'. fusk groups include:
Pulmonary complications:
o Croup (you.rg children) o Children (<5 year)

Patient with pandemic influenza A H1N'1

:*fYtI
$

Y
Home management Home management Hospitalization
No antiviral Antiviral recommended Antiviral is recommended
.ook for danger signr Look for danger signs Critical care service if needed

1
Presence ol danger signs I
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Fig. 21.5: H1N1 patient management (WHO 2009).
t
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 IMMUNIZATION AND INFECTIOUS DISEASES

o Elderly (>65 year) -

Dose of Oseltamivir:
r Pregnant woman - Children age <1 year
o Patients with chronic co-morbid conditions, such as cardio- . <3 months: 12 mg orally bid for 5 days
) vascular, renal, respiratory or liver disease, diabetes mellitus
. 3-5 months: 20 mg orally bid for 5 days
t
I o Those with immunosuppression related to malignancy, . 6-11 months: 25 mg orally bid for 5 days
) chemotherapy, HIV infection, etc. - Children age >1 year
t
r
T
r Obesity defined as a BMI >30. ' < 15 kg: 30 mg orally bid for 5 daYs
I Category C: Complicated or severe pandemic infuenza/severe
' 75-23 kg 45 mg orallY bid for 5 daYs
F Side effects of Oseltamivir
I
acute respiratory illness (SAzu) -
r - Usually well-tolerated.
t o Symptoms of Ill-category A/B - Occasionally:
I o Presenting with shortness of breath, dyspnea, tachypnea, ' Nausea, vomiting, headache
I hypoxia, and/or ' Abdominal pain, dyspepsia, diarrhea
i o Radiological signs of pneumonia ' Conjunctivitis, epistaxis
I r CNS findings (e.g., encephalopathy) . Rash
o Severe dehydration - Rarely hypersensitivity reaction
I o Renal failure - Neuro-psychiatric disorders

F
o Multi-organ dys[unction - Acute hepatitis, and
r Sepsis syndrome - V.ty rarely Stevent Johnson syndrome.
I o Rhabdomyolysis, myocarditis
I In patients who develop AU/ARDS (Acute Lung Injury)-
I o Any of the signs of disease progression (danger signs):
fluid restriction.
r
t o S/S of cardiopulmonary insu{ficiency
,r S/S suggesting CNS complications Chemoprophylaxis
r Evidence ofsecondary bacterial infection o Routine chemoprophylaxis for contacts with cases is not
.r Severe dehydration
recommended.
Very high risk group population with H/O contact with
Treatment
ILI/SAzu may receive prophylaxis.
Category A:
Healthcare providers shouid wear surgical masks during
o Home isolation and social distancing till symptoms resolve patient care.
(about 7 days). Should use N95 mask and PPE, if uses aerosol generating
e Supportive care (rest, adequate nutrition, and more fuid) procedures.
o Paracetamol, if needed (do not use Aspirin/NSAID) Healthcare providers who get ILI etc. should be treated
e Antihistamine if needed with Oseltamivir.
r Follow leaflet instructions
o Respiratoryetiquette
o No antiviral (Oseltamivir).
Category B:
Clinically compatible case that is laboratory confirmed by:
o Home isolation and social distancing till symptoms resolve
(about 7 days) r Demonstration of B. anthracis (Gram stain)
r Supportive care (rest, adequate nutrition, and more fluid) o Positive nucleic acid test for B. anthrarzs (PCR)
o Paracetamol if needed (do not use Aspirin/NSAID) o lsolation of B. anrhracis
o Antihistamine if needed Types:
r Follow the instructions (watch for danger signs)
o Respiratory etiquette o Cutaneous anthrax: It has rwo stages-vesicular & eschar .

r Antiviral (Oseltamivir) is recommended. o Gastrointestinal anthrax

o Inhalation anthrax (pulmonary)
Category C: o Althrax meningitis
a The patients shouid be managed in a hospital.
a Supportive management: Antibiotic(s).
TREATMENT
a Some patients may require critical care:
r Antiviral drug (Oseltamivir): Oseltamivir is available as Refer Tables 2l .22, 2l .23, for treatment of cutaneous
and 27 .24

75 mgcapsules and oral powder for suspension that con- anthrax, inhalational and GI anthrax, and anthrax meningitis,
tains 12 mg/ml after reconstitution. respectiYely.
 I
ESSENCE OF PEDIATRICS

Table 21.22: Cutaneous Anthrax

Children Ciprofloxacin: 10-15 mg/kg ql2hr Oral 10 Days The term fever of unknown origin (FUO) is best reserved
or for children with a fever documented by a healthcare provider
Doxycycline
and for which the cause could not be identified after 3 weeks
>8 years and >45 kg - t OO mg q I 2hr
or of evaluation as an outparient or after 1 week of evaluation
<8 year: and <45 kg= 2.2 mg/kg q1 2hr in hospital. Patients with fever nor meering these criteria, and
specifically those admitted to hospital with neither an appar-
Table 2'1.23:. Inhalational and Castrointestinal Anthrax ent site of infection nor a noninfectious diagnosis, may be
considered to have fever without localizing signs.
Children Ciprofloxacin: 10-1 5 mg/kg q12hr tnitial lV 60 Days
or then
CAUSES
Doxycycline: Oral
>8 years and >45 kg = I 00 mg I2
hrly
Infections: Enteric fever, malaria, UTI, tuberculosis, chronic
OT hepatitis, HIV infection, hidden abscesses (liver, pelvic),
<B years and <45 kg = 2.2 mg/kg mastoiditis, sinusitis, osteomyelitis, meningitis, infectious
l2 hrlv mononucleosis, infective endocarditis, brucellosis, cpomega-
lovirus, toxoplasmosis, kala-azar.
Table 21.24:. Anthrax Meningitis
Autoimmune: Systemic onser juvenile rheumatoid arrhritis,
SLE, polyarteritis nodosa, Kawasaki disease, inflammatory
Children Cryslalline penicillin lnitial tV 60 Days bowel disease.
2 million units IV qJhr initially then o Malignancies: Leukemia, lymphoma, Langerhan cell his-
for 14 days or as individualized Oral
tiocytosis.
Chloramphenicol
I g lV q4hr Most fevers of unknown or unrecognized origin result from
100 mg/kgld, max. 1 g/d atypical presentations of common diseases. In some cases, the
Supporrive 20%Mannitol presentation as an FUO is characteristic of the disease, such as
Hydrocortisone juvenile rheumatoid arrhritis (JRA), but the definitive diagnosis
can be established only after prolonged observation, because
PROGNOSIS initially there are no associared or specific findings on physical
examination and all laboratory results are negative or normal.
r Cutaneous anthrax: < 1olo mortality with treatment; wirhout Drugs fever is nor unusual in children. Infections accounr for
treatment, more chance of dissemination. most of the cases of FUO in children (60-700/o). Even with
o Inhalational Mortaliry rate of B0%o with treatment; without extensive investigations, the cause of FUO remains undiagnosed
treatment, no chance for survival (100% mortality). in l0-2006 of the cases.
. Gastrointestinal: Mortality rate of 50o/o without tr-earment.
o Meningeal: 100o/o mortality in absence of rrearmenr.
DIAGNOSTIC APPROACH
PREVENTION The first step is to identi$' sick patients who need stabilization
and urgent referral ro a rertiary care cenrer. Subsequently, all
o No need to isolate the patient. attempts should be made to reach an eriologic diagnosis. A
a Control measures: Break the cycle- detaiied history is of paramount importance. History should
r Disposal of carcasses (buriai incineration) include the following:
o Disinfection, deconramination, and disposal of contami-
o \(/here and how fever was documented.
nants (Autoclave 721" t 1"C 30 min, OR 10% formalin
for >12 hour)
o Duration and parrern of fever (distinguish from recurrenr
fever).
o Vaccination of animals
o Symptoms referable to all organ sysremsJ r,veight loss.
Protection for healthcare provider by using gloves, face r History of recurrenr inlections, oral rhrush (HIV infection).
mask, and gown. o History ol joint pains, rash, photosensitiviry (autoimmune
disease).

PROPHYLAXIS o History of contact with tuberculosis and animals (brucel-

losis).
r Ciprofoxacin: 10-15 mg/kg PO ql2hr for 60 days, Or o Tiavel to endemic zones (kala-azar). I

o Dorycycline:2.2 mg/kg PO q12hr for 60 days. o Drug history particularly anticholinergics (drug fever).
\
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 IMMUNIZATION AND INFECTIOUS DISEASES

. History of pica, ingestion of dirt could be an important 9. Repetitive chills and temperature spikes-septicemia
clue towards the diagnosis of Toxocara or Toxoplasma (regardless of cause), particularly when associated with
gondii. renal disease, liver or biliary disease, infective endocarditis,
malaria, brucellosis, rat-bite fever, or a loculated collection
Physicol Exominolion of pus.
10. The general acdyity of the patient and the presence or
Careful and meticulous physical examination is mandatory in
absence of rashes should be noted.
all children who have undiagnosed fever. Repetitive examina-
11. Hyperactive deep tendon reflexes may suggest thyrotoxi-
tion, preferably darly, is also important to pick up subtle or
cosis as the cause of FUO.
new signs, which may appear during the course of the illness.
t2. Careful palpation for all groups of lymph nodes and
1. Sweating: The continuing absence of sweat in the pres-
detection of hepatic and splenic enlargement may be vital
ence of an elevated or changing body temperature sug-
to follow the appropriate line of investigation.
gests dehydration due to vomiting, diarrhea, or central
or nephrogenic diabetes insipidus. However, a physical examination is not always conclusive in
2. A careful ophthalmic examination: defining the cause of a prolonged fever so that investigations
a) Red, weeping eyes-connective tissue disease, particu- are always necessary to determine the diagnosis and to confirm
larly polyarteritis nodosa. the etiology.
b) Palpebral conjunctivitis-measles, coxsackievirus
infection, tuberculosis, infectious mononucleosis,
lymphogranuloma venereum, and cat-scratch disease.
lnvesligolions
c) Bulbar conjunctivitis-Kawasaki disease or CBC, ESR, PBE MB M! gastric washings for AIB; serol-
Ieptospirosis. ogy: widal, aldehyde test, rk39 test (ICT for kala-azar).
d) Petechial conjunctival hemorrhages-infective endo- Tests for brucella, salmonella, rickettsiae (test for febrile
carditis. triple antigen), RA, ANA.
e) Uveitis-sarcoidosis, JRA, systemic lupus o Chest X ray, urine analysis, blood and urine cultures, stool
erythematosus, Kawasaki disease, Behget disease, and examination, LFT, and CSF analysis.
vasculitis. o Liver biopsy.
f) Chorioretinitis-CMV toxoplasmosis, and syphilis. o lJltrasound examination, IVIJ, bone marrow aspiration,
g) Proptosis-orbital tumor, thyrotoxicosis, merasrasis and biopsy; FNAC/biopsy of lymph node.
(neuroblastoma), orbital infection, \Tegener granu-
lomatosis, or pseudotumor.
3. tnderness to tapping over the sinuses or the upper teeth TREATMENT
suggests sinusitis.
As far as possible, any sort of treatment for FUO should be
4. Recurrent oral candidiasis may be a clue to various dis-
started only when sufficient grounds for the diagnosis are not
orders of the immune system.
available; mild antipyretics (paracetamol + tepid sponging)
5. Fever blisters-common findings in patients with pneu-
may be given. Hydration should be maintained. Empirical
mococcal, streptococcal, malarial, and rickettsial infection,
trial with antibiotics may mask diagnosis of subacute bacte-
meningococcal meningitis (which usually does not present
rial endocarditis, meningitis, or osteomyelitis, In general,
as FUO), rarely are seen in children with meningococ-
observation of the temperature pattern, repeated clinical
cemia, Salmonella or staphylococcal infections.
examination, and careful laboratory evaluation and inter-
6. Hyperemia of the pharynx, with or without svud216-
pretation of the results might throw light on the diagnosis.
infectious mononucleosis, CMV infection, toxoplasmosis,
a Specific treatment depends on the diagnosis.
salmoneliosis, tularemia, Kawasaki disease, or leptospirosis.
a Empirical treatment is generally avoided, except in criti-
7. Point tenderness over x !6ns-666ult osteomyelitis or
cally ill patients where anti-TB treatment can be given in
bone marrow invasion from neoplastic disease.
suspicion of disseminated TB.
a) Gnderness over the trapezius muscle-may be a clue
to subdiaphragmatic abscess.
b) Generalized muscle tenderness-dermatomyositis,
polyarteritis, Kawasaki disease, or mycoplasmal or
arboviral infecrion.
8. Rectal examination-perirectal lymphadenopathy or ten-
l. Behrman RE, Kliegman Rivf, Jenson HB. Nelson Tbxtbook of
Pediatrics 1B'h ed. India: \7B Saunders Company, 2009.
derness, which suggests a deep pelvic abscess, iliac adenitis,
Parthasarathy A, et al (ed). IAP Textbook of Pediatrics 4'r' ed. New
or pelvic osteomyelitis. Delhi, India: Jaypee Brothers Medical Publishers (P) Ltd., 2009.
a) Occult blood loss may suggest granulomatous colitis 3. ForfarJO, Arneil GC (ed.). Textbook of PaediatricsT't'ed. Edinburgh:
or ulcerative colitis as the cause of FUO. Churchill Livingstone, 2008.
ESSENCE OF PEDIATRICS

4. \William \WHO, Anthony RH, Myron JL, Judirh MS. Current
Pediatric Diagnosis dnd Tieatment 14'r' ed. Connecticut: Appleton
& Lange, 1999.
5. Dwarkin PH. NMS: Pediatrics 4'h ed. Maryland, Baltimor-e: Lip-
pincot rVilliams and \Wilkins, 2000.
6. Ahmedsyah I, Selim A. Tieatment of tetanus: an open study to
compare efficacy of procaine penicillin and metronidazole. BMJ
1985;291:648-50.
7. Godei JC. Tiial of pyridoxine therapy for teranus neonarorum.
Infe ct D is 19 B2;1 45 :5 47 -9.
8. Ciaglia f; Firsching R" Syniec C. Effective percutaneous diiational
tracheostomy: a new simple bed side procedure. Chest 1985;87:715-9.
9 . Ghai OB Gupta P, Paui VK. Essential Pediatrics 7'h ed. New Delhi,
India: CBS Publishers, 2009.
10. American Academy of Pediatrics Committee on Pediatric AIDS.
Evaluation and medical treatment of the HIV exposed infant.
Pediatrics 1997 99.
11. Scarlatti G. Pediatric HIV infection. 7he Lancet 1996;348:863 B.
12. Gupta S. Short Textbooh of Pediatrics 8'h ed. New Delhi: Jaypee
Brothers,1998.
13. Gilles HM. Management of severe and complicated malaria - a
practical handbook. 2"d ed. Geneva, 1991.
14. Diagnosis, managemenr chart of kala-azar, \X&IO, 1994.
15. Rahman ME, Nath PK, Aimed FU, et al. Falciparum malaria of
childhood; complications and outcome. JCMCTA 1996;7 (53):347 .
16. Rahman MR, Faiz MA, Yunus EM, et al. Malaria: new clinical case
definition and ffeatment guidelines. JCMCTA 1996;7 (53) :7 5-82.
17. Rahman ME, AIam B, Ahmed FU, et al. Elficacy of ciprofloxacin
in the treatment of childhood ryphoid fever. Bangladesh J Child
Heahh 199 5 :19 l37 6-80.
t8. National guidelines for Ciinical Management of Dengue Syndrome
DGHS, MOH & F\W and \XIHO, Bangladesh 2000.
19. Clayden GS, Howkins RL (ed.). Paediatrics: TVeatment dt Prognosis
1" ed. New Delhi: Jaypee Brothers, 1989.
20. Haslett C, et al. (ed). Dauidsonls Princip/es and Practice of Medicine
1B'h ed. London: Churchill Livingstone, 1999.
2t. Rahman ME, Chowdhury M, Mollah AM. Study of Childhood
leprosy. Bangladesh I 0f Child Helath i.991;15(314):52-7.
22. Rahman ME, Samad R, Rahman MR, et a1. Prognostic factors
relating to outcome of severe malaria. Bangladah Med Res Counc
Bull 2001.27(I):l-8.
23. Faiz MA, Rashid R, Palit R, et al. Parasight TM F test in cerebral
malaria patients before & after treatment in CMCH, Bangladesh.
Tians R Soc 7/op Med 2000;94:56,7.
24. Guidelines for the Surveillance and Control ofAnthrax in Human
and Animals. 3'd ed. l7HO. Emerging and other Communicable
Diseases, Surveillance and Control. Department of Communicable
Diseases Surveiliance and Response, 2005.
25. European Guidelines for the Clinical Management ofAnthrax and
Bioterrorism-Related Anthrax. CDC, Atlanta, 2005.
26. National guidelines and training module lor Kala-azar elimination
in Bangladesh, January 2008.
27 National guideline for clinical management of pandemic (H1N1)
2009 influenza. Bureau of Health Education, DGHS, MoHF\7,
2009.
la \X/HO Guidelines lor Pharmacological Management of Pandemic
(H1Ni) 2009 Influenza and other Infuenza Viruses, 20 August
2009.

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 CHAPTER
Genetic Disorders
Chopter Conlenls
Basic genetics......... ..............................423
Classification of genetic dis0rders................. ................424
Mendelian disorders: unifactorial dis0rders.................... 424
Autosomal dominant inheritance.................................. 424
Autosomal recessive inheritance................................... 425
Xlinked dominant inheritance ......................................425
X-linked recessive inheritance.........................................426
Y-linked disorder... ..............................426
Mitochondrial inherilance....................................................426
Microdeletion syndr0me................ ......................................427
Multifactorial disorders................. ........................................427
Cenetic counseling .............................427
Of all neonates, 2-3Vo have at least one major congenital
abnormality, at least 50o/o of which are caused exclusively
or partially by genetic factors. The incidences of chromo-
some abnormalities and single-gene disorders in neonates are
approximately 7:200 and 1:100, respectively. Genetic disorders
account for 50o/o ofall childhood blindness, childhood deafness,
and severe learning difficulty. In developed counties, genetic
disorders and congenital malformations together also account
for 300/o of all childhood hospital admissions and 40-50o/o of
all childhood deaths. The basic unit of inheritance is present
in chromosomes of all cells. The genetic material in nucleus
of cell can be grouped in two-three pairs of chromosomes, of
which 22 pairs are apparently alike and are called autosomes.
The remaining23rd pair is called sex chromosome, which is
being represented in female by xx and in male by xy.
The portion of chromosome, or precisely portion of DNA,
which codes for a character is called a gene. Genes are arranged
in a chromosome like beads on a string. The position of gene
in chromosome is called locus. Alleles are the genes present in
corresponding loci on two members of a pair on chromosomes.
If alleles code for same trait, they are in homozygous state; if
they code for different traits, they are in heterozygous state.
If an allele clinically manifests itself even in the heterozygous
state, it is called a dominant gene. If an allele does not manifest
22
in heterozygous state (i.e., when other allele from other parent
is normal), it is called recessive gene.
The genetic make up of a person is called genotype. The
clinically manifest characters are known as phenotype.
CHEMISTRY OF GENES
Genes are made up of deoxyribonucleic acid (DNA). Most
of the DNA resides in nucleus. The DNA molecule has
the form of a continuous double strand twisted in a helical
fashion (nvisted rope ladder). The basic unit of DNA strand
is a nucleotide, which is composed of sugar (deoxyribose),
phosphate backbone, and one of the four nitrogenous bases.
Nitrogenous bases are two purines (adenine [A] and guanine
[G]) and two pyrimidines (thymine [T] and cytosine [C]). The
bases of two strands are linked together by hydrogen bonds
occurring between purines on one strand and pyrimidines on
other strand. Adenine always pairs with thymine and guanine
always with cytosine.
Genetic diseases are ubiquitou s. 2.5o/o of ail live born infants
have genetic disorders or congenital malformations. In general,
human diseases may be seen lying on a continuum, ar rhe
one end are those that are entirely genetic in origin and at
other are those that are entirely environmental. Between these
extremes are various common multifactorial diseases in which
both genetic and environmental factors play role.
 ESSENCE OF PEDIATRICS

CTASSIFICATION OF GENETIC DISORDERS individual can be tra,:ed from one generation ro rhe nexr
(Fig.22.U\).
A) Genetics disorders due to traditional modes of a Both sexes may be affected equally.
inheritance: a Ifone of the parents is affected, the risk of recurrence is
1) Mendelian disorders (single gene) 50o/o in each subsequent pregnancy (Fig. 22.18).
a. Autosomal dominant The disease usually appears in every generation of the family.
b. Autosomal recessive Occasional instances of poor penetration (e.g., retinoblas-
c. X-linked recessive toma, etc.) are exceptions to this rule, where a generation
d. X-linked dominant may be skipped (Fig.22.lC). The traits aiso have variable
2) Chromosomal disorders expressivity and sex-limitation. Marfan syndrome may have
a. Numerical abnormalities full blown picture in one person and partial picture in
b. Structural abnormalities another person of same family.
3) Multifactorial disorders If a heterozygote (affected) parent mates with a homozygote
4) Somatic cell mutations normal parent (which commonly occurs), on rhe average,
B) Genetic fisorders due to non-traditional modes of one-half of the progeny 600/o) will be affected heterozy-
inheritance: gotes and one-half (50%) will be normal. If rwo affected
1) Mosaicism heterozygotes mate, their alleles will segregare during gamate
2) Genomic imprinting formation and recombine randomly, and it could be pre-
3) Uniparental disomy dicted that one-fourth of their offsprings would be affected
4) Inheritance of unstable murarions homozygotes, two-fourth affected heterozygores, and one-
5) Cytoplasmic/mitochondrial inheritance fourth normal homozygotes. 75o/o (314) of the offsprings
from this mating would be affected by the dominant rrair.
Nu mericol Abnormolities Homozygocity is rare but dangerous (Fig. 22.1D).
Euploid: A cell with the exact multiple of the haploid number, Because of the smaller size of the modern families, by
e.9.,46,69,92. chance, all the children of an afrected individual may be
normal, or all his children may also be affected. It is on an
Polyploid: Euploid cells with more than normal diploid number average that half of the offsprings of an affected individual
of 46 chromosomes is called polyploid-69 chromosomes. will be affected.
Aneupliod: Cell deviating from one of the euploid numbers
is called aneuploid.

tisomy: Presence of one chromosome additional to the normal

homologous pair, e.g., tisomy 21.

Monosomy: A chromosomal number is one less than the (A)

diploid number-Turner syndrome.
Mosaicism: An individual with two or more cell lines derived
from a single zygote.
Aa

(B)

AUTOSOMAL DOMINANT IN H ERITANCE

Autosomal dominant disorders are those conditions that can

be expressed in the heterozygous state (i.e., a single muranr (c)
gene from either of the parents is needed to manifest, in other a

words, he or she possesses both the mutant gene and the normal I Hetero affected
gene). This is the common mode of inheritance in humans. The I Homo afiected \
mutant gene is contributed by the affected parent present on 50% 25% 15% I
one of the autosomes. The characteristic features of autosomal (D) \
dominant inheritance are as follows (Fig.22.1): I
o There is parent
Fi9.22.1: Autosomal dom i nant disorders-(A) vertical transm ission, t
to offspring transmission of character, i.e., (B) 50% risk at each pregnancy, (C) lack of penetrance in "A", Ii)r
the transmission is of vertical pattern and the affected and (D) homozygosity.

ru
I
 !

GENETIC DISORDERS

New cases may be found in unaffected family due to new I]---r___<}

mutation, where parents are not found to be affected. Achon-
droplasia in 80%, Marfan in 30o/o, Apert syndro me in 960/o, la (A)
c
and neurofibromatosis in 40o/o are due to fresh mutations.
Unaffected members of the family generally cannor transmir Aa
the disease.
Homozygosity (getting double dose) is rare, usually dies in
the neonatal periods.
(B)

Aulosomol Dominont Disorders

o Neryous: Huntington disease, neurofibromatosis, myotonic
dystrophy, tuberous sclerosis.
. GIT and hepatic Familial polyposis coli, Gilbert syndrome.
o Urinary: Polycystic disease of kidney.
o Musculoskeletal: Marfan syndrome, Ehlers-Danlos syn- (c)
drome, osteogenesis imperfecta, achondroplasia, facioscapu-
lohumeral muscular dystrophy, myotonia congenita. tig. 22.2: Autosomal recessive (AR) disorders-(A) horizontal
o transmission, (B\ 25% risk, and (C) consanguinily in AR.
Hematopoietic: Hereditary spherocytosis, von \Tillebrand
disease.
o Metabolic: Familial hypercholesterolemia, acute intermit-
tent porphyria. o The heterozygous carrier can be detected, because they
o Other: Noonan syndrome. exhibit minor stigmata of the condition (though healthy
phenotypically); detectable either by clinical examinarion
or by biochemical investigarion.
AUTOSOMAT RECESSIVE IN H ERITANCE

Uniike dominant traits, recessive rrairs manifest only in the

homozygous state, that is, in those individuals who possess
double dose of the mutant gene. Heterozygotes who possess
only one mutant gene are usually perfectly healthy.
The characteristic features of autosomal recessive inheritance
are as follows (Fig. 22.2):
o If a heterozygote individual marries another heterozygote
person (common), one-quarter of the offsprings will be
normal, one-half will be healthy heterozygotes (i.e., 50o/o
carrier) and one-quarter will be affected (1:2:1) (shown below).
At conception, there is a I in 4 chance that any child of two
heterozygous parents will be aliected. These are also average
figures; by chance all the offspring of such a couple may be
affected, or similarly all of them may be normal.
If two persons homozygous for the same recessive rrait
marry (most unlikeiy), all their children would be affected.
The pattern of transmission is called horizontal, because the
siblings are affected in a horizontal pattern and the parenrs
are phenotypically normal. However, they are genotypically
abnormal heterozygotes carriers.
a
Since the recessive traits are iocated on autosome, male and
I female are affected equally.
a Since recessive traits are rare in the population, consan-
I guineous matings are more likely to produce the affected
t homozygote. The chance that first cousins will carry the
7 same recessive gene is 1 in 8.
t exhibiting such condition.
)
'
F
Aulosomql Recessive Disorders
a Metabolic Cysticfibrosis,phenylketonuria,galactosemia,
homocystinuria
Lysosomal storage disease, antitrypsin
deficiency, \Tilson disease
Hemochromatosis, glycogen storage disease,
Gaucher disease
Hurler syndrome
Hematopoietic Thalassemia, sickle cell anemia
Endocrine Congenital adrenal hvperplasia
Skeletal Ehlers-Danlos (some), alkaptonuria
Nervous SMA, Friedreich ataxia
Other Color blindness
X-tlNKED DOMINANT INHERITANCE (Fig. 22.3)
The characteristic features are as follows:
X-linked dominant conditions manifest both in )O( females
who are heterozygous for the mutanr gene, and in males who
carry the mutant gene on their single X chromosome.
An affected female will transmit the trait to half of the
offsprings of either sex, but an affected male will transmit
the trait to all of his daughters but to none of his sons.
There will, therefore, be an excess of females in the families
 I

ESSENCE OF PEDIATRICS

o She may be a homozygous for the mutant gene (rare);

situation arises if a hemizygous affected father marries a
heterozygous carrier female.
o She may have an abnormal chromosomal constitution
resulting in her having only one X chromosome, such as
XLD disorders in Turner syndrome (XO).
Fig. 22.3: X-linked dominant disorder.
o If the X chromosome containing the normal allele is inac-
tivated leaving the cells with only the mutant gene of X
chromosome (Lyon hypothesis).
X-linked Dominonl Disorders
o Familial hypophosphatemic rickets X-linked Recessive Disorders
o Oro-facio-genital syndrome
o Incontinentia pigmenti Musculoskeletal DMD (Duchenne muscular dystrophy)
Becker muscular dystrophy
r Rett syndrome
Blood Hemophilia A and B, G6PD deficiency
Metabolic Diabetes insipidus, Hunter syndrome
X-tlNKED RECESSIVE INHERITANCE (Fig. 22.4) Nervous Fragile X syndrome
Renal Alport syndrome
The disorders have the following features:
o In X-linked recessive conditions, only males are affected as
there is no corresponding allele. All his daughters will be
carriers as they receive abnormal X from father.
Ylinked disorders are rare and cause minor phenorypic features.
An Xlinked recessive trait will not manifest in females as
Only males are affected who get their abnormal genes from
the other X contains normal partner gene. 50o/o sons will
their father. Examples: Porcupine skin, hairy pinna, webbed
be affected and 50o/o daughters will be carriers when the
toes, and frontal baldness. Like surname, the trait is transmit-
mother is a carrier.
ted only to sons.
a Normal sons cannot transmit the disease.
a The pattern of inheritance here is called "oblique" as only
males on the maternal side are affected, i.e., maternal IYON HYPOTHESIS
uncles, patientt mothert maternal uncles, and mothert
In 1961, Dr. Mary Lyon proposed that one of the two X
sistert sons.
chromosomes in each of the somatic cells of normal female is
o Female may be affected, if an affected male marries a carrier
genetically inactivated and any additional X chromosome also
female or in Turner syndrome where there is only one X
becomes inactive, and appears as, Barr body. Lyon hypothesis
(45, x).
provides an explanation for occurrence of hemophilia in females.
o Fresh mutations are known.
Normally, one X chromosome is inactivated, but in hemophilic
o Carriers may have biochemical abnormalities, e.g., increased
female X chromosomes containing the normal allele for factor
CPK levels in Duchenne muscular dystrophy.
MII production are inactivated leaving only the X chromosome
Yery rarely, a female may be a victim; may manifest an Xlinked containing the mutant allele.
recessive trait in the following situations:

Mitochondria are intracellular organelles. Mutations in mito-

chondrial DNA can cause various disease mainly involving
organs highly dependant on aerobic metabolism such as brain,
skeletal muscle, heart, renal and endocrine system. Their pre-
sentation is non-specific with encephalomyopathy, failure to
thrive, seizure, ophthalmoplegia, and sensorineural hearing
loss. Diagnosis requires a battery of clinicai investigations, :
muscle biopsv (structure, histochemistry and enzyme rtudy),
and DNA analysis. ;
Mutations in mitochondrial DNA are of maternal origin. \
(B) t
Examples:
I
tig,. 22.4: X- I i n ked recess ive d i sorders-(A) ob I i q ue transm rssron; 1. MEI-AS syndrome: One of the commonest mitochondrial
(B) boys 50% risk affected, girls 50% risk carrier. disorders. Mitochondrial myopathy, encephalopathy, lactic \
l
ll n

acidosis, and stroke (MEIAS) syndrome is a progressive
neurodegenerative disorder. Patients may present sporadi-
cally or as members of maternal pedigrees with a wide
variery of clinical presentations. The rypical presentation
of patients with MEIAS syndrome includes features that
comprise the name of the disorder, such as mitochondrial
encephalomyopathy, lactic acidosis, and stroke-like epi-
sodes. Other features, such as seizures, diabetes mellitus,
hearing loss, cardiac disease, short stature, endocrinopa-
thies, exercise intolerance, and neuropsychiatric dysfunc-
tion are clearly part of the disorder.
MELAS syndrome onset may occur early in infancy
with a history of developmental delay and learning disabili-
ties. Developmental delay, learning disability, is primarily
found in patients prior to the development of first stroke.
An encephalopathic picture that is progressive and leads
k
I to dementia may be present.
a Stroke-like episodes are the hallmark feature of this
t-
disorder. Initially, episodes may manifest with vomiting
and headache that may last several days. These patients
may also experience episodes of seizures and visual abnor-
malities followed by hemiplegia. Seizure types may be
tonic-clonic or myoclonic. Patients may have visual
complaints due to ophthalmoplegia, and they may experi-
ence blindness because of optic atrophy and difficulties
with night vision due to pigmentary retinopathy' Some
patients may experience hearing loss, which may accom-
pany diabetes. It may be observed in association with
the classic disorder of MELAS syndrome. Some patients
may develop apnea and an ataxic gait in association with
neuroradiologic features of MELAS syndrome.
Tieatment with coenzyme CoQl0 has been helpful in
some patients with MELAS syndrome. Phytomenadione
(K-1) and Riboflavin have some function. Dichloroacetate
is another compound used with these agents, since levels
of lactate are lowered in plasma and cerebrospinal fluid
(CSF). Sodium succinate has been used, and a patient
with MEIAS syndrome reportedly had fewer stroke-like
episodeswith its use.
) MERRF (myoclonic epilepsy and ragged red fibers):
The classic picture is of progressive myoclonic epilepsy,
myopathy, and slowly progressive dementia, oPtic atrophy.
3. Leber hereditary optic neuropathy (I,HON).
4. Leigh syndrome: Subacute necrotizing encephaiopathy,
failure to thrive, developmental delay, hypotonia, exrernal
ophthalmoplegia, optic atrophy.
5. Kearns-Sayre syndrome: Chronic progressive external
ophthalmoplegia, maternally inherited diabetes mellitus,
and deafness.
6. Pearson syndrome: Pancytopenia, failure to thrive,
short stature, malabsorption, due to pancreatic enzyme
deficiency.
7. Barth syndrome: Congenital dilated cardiomyopathy,
myopathy, growth retardation.
I
GENETIC DISORDERS
The term microdeletion refers to a chromosome deletion,
which is too small to see on standard karyotyping. They can
be detected using specific fluorescent probes (Fluorescent in
situ hybridization [FISH]). Examples:
o DiGeorge syndrome: Parathyroid gland hypoplasia with
hypocalcemia, thymus hypopiasia with T-lymphocyte defi-
ciency, congenital cardiac malformations, cleft palate, due
to micro deletions at chromosome 22)
o \Tilliam syndrome (micro deletion on chromosomeT).
Nearly 600/o of birth defects are due to this kind of inheri-
tance. Recurrence risks are much less compared to single gene
disorders.
Multifactorial disorders are anencephaly (asthma, cerebral
palsy, cleft lip t cleft palate, cleft palate only, clubfoot' con-
genital heart diseases, congenital pyloric stenosis, diabetes mel-
litus (IDDM), dislocation of hip, exomphalos (omphalocele),
epilepsy, Hirschsprung disease, hydrocephalus, hypospadias (in
males), mental retardation, renal agenesis, schizophrenia, spina
bifida, tracheo-esophageal fistula.
Genetic counseling has been defined as an educational process
that seeks to assist affected and/or at risk individuals to under-
stand the nature of a genetic disordeq its transmission, and
the options available to them in management and family plan-
ning. The counselor ofFers investigations, options and support
whereas the consult (the person who seeks the advice) makes
his own decision, which is known as non-directive counseling.
Indications:
o Known or suspected hereditary disease in a patient or
family.
r Birth defects in previous children.
o Unexplained mental retardation/dysmorphism/rnultiple
malformations in a child.
o Consanguinity.
o Exposure to teratogen during pregnancy'
o Identification of malformation by ultrasonography during
Pfegnancy.
STEPS IN GENETIC COUNSELING
Diognosis qnd Construclion of Pedigree
An accurate diagnosis is the first essential requirement for
genetic counseling. The first and most important step in
diagnosis of genetic disorders is construction of a family tree'
Th. p"tt..n olinheritance can be shown from the pedigree, for
 ESSENCE OF PEDIATRICS

M Table 22.1: Mendelian Disorders Amenable to Carrier

Detection
tr o Unaffected

I a Affected Familial
hypercholestero lemia
Thalassemia Ocular albinism
Tay Sachs disease Hemophilia A
I Propositus (index cases)
Adu lt polycystic kidney
disease
Sickle cell disease
Family based screening
Hemophilia B

\ Duchenne muscular

tro Heterozygous canier (AR)

N eu rofibromatos is
Tuberous sclerosis
n-1-Antitrypsin
deficiency
dystrophy
Hunter syndrome
Myotonic dystrophy Congenital adrenal
o Heterozygous carrier (XLR) Huntington chorea hyperplasia
Cystic fibrosis
d Deceased Calactosemia
M ucopo lys acc ha ridos is

c Sex Unknown
Phenylketonuria

E Subject without offspring

Table 22.2= Approaches to Carrier Detection
I r-:----1_) Marriage (Consanguinous)

o'\ Twins (Dizygotic) DNA analysis Thalassemias, Duchenne and Becker

A Twins (Monozygotic)
Specific gene probe:

Biochemical: enzyme
Fig. 22.5: Pedigree symbols. deficiency, protein defect
example, vertical transmission in autosomal dominant disorders, Physiological
horizontal transmission in autosomal recessive disorders, and Electroreti nogriphy
oblique transmission in Xlinked recessive disorders. Electromyography
muscular dystrophy, hemophilia A and
B, n-1-antitrypsin deficiency, sickle cell
disease
Hexosaminidase A (Tay-Sachs) disease;
Factor Vlll assays (hemophilia A)
Serum creatine kinase (Duchenne
muscular dystrophy), phenylalanine load
(phenyl keton u ri a)
X-linked retinitis pigmentosa, muscular
dystrophies (myotonic and Duchenne)

Standard pedigree symbols in pedigree are given in Cytogenetic studies Chromosome translocation, fragi le X

Fig.22.5. syndrome

All affected individuals must be examined; asympromaric Microscopy:

individuais should also be examined to exclude mild or early Blood Sickle cel l di.e.rse, Thal,rssemia>
disease. Home visits should be made; distant relatives and tt ropsy Duchenne muscular dystrophy
older members of the family should be interviewed and special Radiology fuberou. sclerosis rt erebral r alcilit aliorr)
investigations like radiologv cytology, DNA studies, and histol- Clinical Skin (Fabry disease)
ogy may have to be done. Muscle (Dr-rchenne dystrophy)

Risk (ond Recurrence) Estimotion

The mathematical risk calculated from data on a pedigree may
often be modified by additional information from specific tests
to detect carriers.
Detection of carriers: A carrier is an individual who possesses
in heterozygous stare the gene determining an inherited disorder
and who is essentially healthy ar the rime of the study. Iden-
tifying carriers of genetic disorders in families or populations
at risk play an important parr in preventing genetic disease.
Mendelian disorders amenable ro carrier detection are listed ir.r
Table 22.1 . Ta6le 22.2 lists approaches ro detecr carrier.
*bligate carriers: Families in rvhich there is a generic disorcler,
some members musr be carriers because of the wav in rvhich
the condition is inherited, these are called as obligate carriers.
Example: (l) autosomal dominant-person with affected parent
and child; (ii) autosomal recessive-parents and child (children)
of affected person; (lzz) X-linked recessive-woman with nvo
affected sons or one allected son and another affected male
maternal relative and ali daughters of an allected man.
The carrier srate can be determined by clinical signs, analysis
of genes, analysis of gene products and secondary biochemical
abnormalities such as raised serum crearine kinase activity in
Duchenne and Becker, muscular dvstrophies.
Communicqtion ond Supporf
Counselir.rg should be r-ron-directive. A good supporr should be
established. Both rhe parenrs should be present for discussion.
Genetic basis for- the problen.r should be described using simple
language. The counselor should interpret the anticipated risk of
recLlrrence of the inire rited disor-de r in a meaningful manner and
I
I
\
4
 GENETIC DISORDERS

treatment options available for prevention of genetic disorders, so
that family can arrive at a rational decision. The counselor should
reassur€ the parents that risk of recurrence is low in multifacto-
rial disorders. \X{hile conveying infolmation to the parents, the
counselor should take special care not to infuse sense ofguilt in
the parents. Counselor can also show the way to prevent genetic
disorders by carrier screening and proper selection of mates as in
thalassemia. Counselor should also give information regarding
ways of prenatal diagnosis of disease and options of treatment
available if fetus is carrying a disease.
A combined approach is necessary for continuing support
to the patient and his familv. Involvement of the genetic
counselor, family practitioner, specialist genetic nurse, social
worker, should be ensured. Support groups provide psycho-
logical support for families by bringing them into contact witl-r
others with a similar problem. All families with an affected
member, and carriers of specific genetic disorder, should be
pr.lt il1 touch with relevant support groups where those exist.
Such groups identify the concerns of families and allow the
community to participate in developing service.
Prenqiql Diognosis
The technical advances, especially in molecular genetics, and
availabiiiry ofvarious prenatal diagnostic techniques have added
t
a new dimension in the process of genetic counseling. Prenatal
diagnosis is possible in following ways:
I
Cordocentesis: Withdrawing blood from the umbilical vein
under ultrasound guidance, cordocentesis helps in prenatal
diagnosis of genetic disorders and understanding of fetal
physiology, development, and metabolism.
Fetoscopy: A fine caliber endoscope is inserted into the uterus,
the direct visualization of the fetus is possible and fetai blood
sampling can be done.
FISH: Molecular cytogenetics has given rise to the powerful
new technology or fuorescent in situ hybridization (FISH). A
fluorescently labeled DNA probe is hybridized to a standard
chromosome preparation on a microscopic slide. This method is
useful in detecting chromosomal abnormalities, both numerical
and microdeletion syndrome.
The various techniques used in prenatal diagnosis, their timing,
method and complications are summarized tnTable 22.3.
Prevenlion of Genelic Disorders
Carrier screening: Screening of carriers of disease help in
prevention. Those who are suffering from B-thalassemia trait
or HbE disease can be detected by population screening. Mass
motivation to restrict marriage berween carriers will limit
the spread of thalassemia in a community. The incidence of
thalassemia has been brought down by mandatory carrier
screening of population and proper genetic counseling.
Genetic metabolic screening: Newborn infant can be
routinely screened for inborn errors of metabolism, e.g.,

Chorionic villus sampling (CVS): A technique for obtaining

fetal celis for prenatal diagnosis by biopsy ofthe placenta, now
usually done transabdominally after 10 weeks of pregnancy.
Amniocentesis: It is the withdrawal of amniotic fluid from
the amniotic sac surrounding the fetus. Amniocentesis is done
ar I 5-l(r weeks of gestation.
PKU, galactosemia, familial hypercholesterolemia, congenital
hypothyroidism, and the handicap can be prevented by
early treatment.
Neural tube defects (NTD): Recurrence of NTD can be
prevented by taking folic acid 4 mgevery day 1 month before
to 3 months after conception. It has also been recommended

Table 22.3t Prenatal Diagnostic Techniques, their Timing, Methods, and Complications

Amniotic fluid a. Conventional amnlocentesis 15-16 AFP/ACHE Abortion, need puncture

b. Early amniocentesis 11-14 hcc i njuries, placental abruption,

chorioamnion itis, preterm labor,

dmniori( fluid leakage
Amniocytes .r. ConvenLional antniot enlerl' 15 16 Cell culture for karyotypes, enzyme
b. Early amniocentesis 11-14 assay, DNA studies, FISH

Chorionic Villi a. Transvaginal 9-1 1 Biochemical, chromosomal, T%fetal loss, limb defects,
b. Transabdomirral 12-24 DNA mosaicism and maternal bleeding.
.1
Fetal blood Fetoscopic 1 B-20 Coagr-rlation factor immunoglobuIin % fetal loss, Rhesus sensitization,
aspiration antibodies estimation; DNA and fetal infection, PROM
Cordocentesis 16-20 enzyme study, karyotype, FISH
Maternal serum Maternal blood flow 12 14 AFP/UE/hCC, FISH, fetal Nil
Fetal cells in cytometry, PCR/monoclonal 1st sexing DNA tests
maternal antibodies trimester
circu lation IVF
Preirnp la ntation Biopsy of blastocysts 4 B cells stages DNA PCR, enzyme assay Nil
ii:ir.r,, o biopsi
t t::'t-' . : PCR, polr rrt-rasc ch.rin rear:tion; ACHE, arcet)'lcholinesterase; PROM, prenrature rupture ot menrbranes lrCC, human chor ontc si-r'::1::-:: - ,

- -- : ir:rar,r!lug.rlerl eslrlo
.
 ESSENCE OF PEDIATRICS
that all expecrant mothers should consume about 0.4 mg of
folic acid daiiy to prevenr firsr occurrence of NTD.
o Prenatal diagnosis: Prenatal diagnosis is possible in a large
number of single gene disorders, in chromosomal disorders,
in neural tube defects, so that selective termination of
pregnancy can be done.
TREATMENT
The successful treatment of genetic disorders requires accurate
diagnosis, knowledge of biochemical basis of the disorder and
early intervention.
Major principles of treatment include (l) restriction of poten-
tially toxic environmenral agents; (ii) replacement of missing
gene product, deranged organ, or even gene itself; (iii) removal
of toxic subsrances of organs; (lz) metabolic manipularion; and
(er) surgical managemenr.
Reslriclion
Examples for restriction are as follows: (a) Phenylkglonulia-
phenylalanine should be restricted in the diet. (6) Galactosemia-
galactose should be eliminated from the diet early in life. If
prenatal diagnosis is made, the mother should not drink milk
in pregnancy. (r) G6PD deficiency-primaquine, sulfa drugs,
and fava beans should be restricted. (fi Acate intermittent
porphyria-phenobarbitone, sulfa drugs, estrogen, erc. should
be restricted. (r) Alpha-l-antitrypsin deficiency-resrriction
of cigarette, smoking prolongs or prevents the development of
pulmonary emphysema. (l Familial hypercholesterolemia-
cholesterol and saturated fats should be restricted.
Replocement
o Hemophilia A and B: The trearment aims at replacement
of factor \4II and factor IX by transfusion.
o Diabetes mellitus: Insulin is the agent replaced.
o Alpha-l-antitrypsin deficiency: Can be successfully treated
by replacement with recombinant alpha-l-antitrypsin.
o Congenital adrenal hyperplasia: The agents repiaced are
cortisone and aldosterone.
o Cystinosis: Kidney transplantation.
e Types ofGene Therapy
Adenosine deaminase deficiency: Bone marrow transplan-
tation or somaric cell gene therapy.
r Familial hypercholesterolemia: Liver transplantation.
Removol
The examples are as follows: (a) Wilson disease-ir is an
autosomal recessive disorder characterized by accumulation
of copper in various organs like rhe liver, brain, and kidnevs.
Copper can be e€icectively chelated by oral administration of
penicillamine. (&) Hemochl6malssi5-rhe excess iron srores
are removed by repeated phlebotomy. (r) Familial polyposis
coli-colectomy is advised to prevent carcinoma of colon,
which develops during the fourth decade of life.
Melo bolic Monipulotion
Metabolic manipulation can correcr certain types of disor-
ders, e.g.: (a) Neural tube defects-periconceptionai folic
acid therapy has shown to prevent neural tube defects. (/)
Homocystinuria-Classic homocystinuria is due to deficiency
of cystathionine synthetase. Among these, 40% of patients
respond to high doses of vitamin Bo. (r) Criggler-Najjar syn-
drome type Il-these patients benefit by oral phenobarbitone
administration, which induces heparic glucoronyl transferase
activity. (/ Methylmalonic acidemia-administration of
vitamin B,r. (e) Familial hypercholesterolemia-lovastatin
to block endogenous synthesis of cholesterol.
Surgicol Monogemenl
Surgical management can be considered in certain cases: (a)
Congenital adrenal hyperplasia-clitoroplasry and vaginal
reconstrucrion surgery. (b) Marfan syndrome-surgery for
aortic dilatation. (c) Hydrocephalus-shunr su,rgery. (fi
Obstructive uropathy-inrraurerine shunt or correction. (r)
Diaphragmatic hernia-repai r.
Gene Theropy
It is the
inserrion of normal gene into the appropriare tissues
of an individual affected with a generic disorder in order to
precisely and permanenrly correcr the disorder.
Potential candidates for gene replacement are patients with
(z) hemophilia A and B, (ii) alpha-t-antitrypsin deficiency, (iii)
phenylketonuria, and (iz) hemoglobinopathies.
Approaches to Gene Therapy
Two generai strategies are used to deliver genes to specific
tissues and correct genetic defects.
i. Ex vivo approach: The target cells are removed from the
body, and a normal gene is then introduced in vitro by
one of the gene d elivery methods.
ii. In vivo approach (direct gene transfer): The therapeutic
gene is introduced directly into the affected tissue without
removing cells from the body.
Somatic cell therapy: The insertion of a therapeutic gene into
somatic cells, which include fibroblasts, myoblasts, epithelial
cells, endothelial cells, nervous, glial cells, etc.
Germline therapy: It is the introduction of a foreign gene
into cells, i.e., sperm, ovum, or fertilized egg, resulting in their
expression in both somatic as well as germ cells. This is not
advocated in humans.
I
Down syndrome is the most common chromosomal disorder \
and a leading cause of mental retardation. Incidence is
\
t
I
!
 GENETIC DISORDERS

approximately 1 in 700 live births. Trisomy 21 (three copies of Down syndrome are summarized in Table 22.5 (Figures in
of chromosome 21) is present in95o/o of cases, translocation parenthesis indicate appropriate time for the test).
of long arm of chromosome 21 to another chromosome
(e.g.,74,21, or 22) is present in 4%o cases, and mosaicism COUNSETING
in 1olo cases. The incidence of trisomic cases increases with
advancing maternal age with 170 recurlence risk' In trisomic Parents of a child with Down syndrome should be counseled
casesr extra 21 results from meiotic non-dysjunction of ovum with tact, compassion, and truthfulness. The parents should
or sperm (extra 21 is maternai in 75a/o cases and paternal be told about the associated problems, and the importance
in remainder). Tianslocation is not related to maternal age of early stimulation should be highlighted. The risk of recur-
but is inherited. Mosaics have less malked physical stigmata rence should be clearly told to the parents. The risk of Down
and higher inteiligence. Mongoloid facies is always present syndrome increases with maternal age; the risk at 20 years of
or pathognomonic. Mental retardation is constant but non- age is 1:1526, at 30 years is 1:909, 35 years is i:384, and at
specific. Clinical featules of Down syndrome are given in 45 years is 1:28.
'fable 22.4. The risk of Down syndrome in relation to maternal age is
During adolescence, Down syndrome patients develop found at amniocentesis is 3.9 per 100 at 35 years of age and
normal primary and secondary sex characteristics, obesity, skin 44.2 per 100 at 45 years of age. The risk of tlanslocarion Down
inf-ections. Adults with Down svndrome may have cataract' syndrome in subsequent live binh is given below:
hypothyroidism, mitral valve prolapse, hearing loss, Alzheimer
disease. Infants with Down syndrome may have loose skin
Types of Parents carrying Risk to
folds on the nape of the neck, cutis marmorata' acrocyanosis translocation balancedtranslocation offspring%
of extremities. The dentition is alwavs delayed, irregular with 14/21 Mother 10
smali, malformed teeth, and aplasia of enarnel.
Father 2.5

None <1
DIAGNOSIS )1/)1 100

Diagnosis depends mainiy on the characteristic clinical features.

Cytogenetic studies have important etiological implications and
help in generic counseling.
Prenatal diagnosis of Down syndrome is offered to women
at high risk, i.e., advanced maternal age, previous history of Incidence is approximately 1 in 2500 female live births, and
a child with Dowr.r syndrome, and translocation carriers or parental age has no effect although 600/o of patients with Tirrner
partners of translocation carriers. Methods of prenatal diagnosis syndrorne have a 45 XO karyotype.

Table 22.4: Clinical Features of Down Synclrome

Mongoloid facies Central nervous system (CNS) Endocrine

* Brachycephaly with flat occiPut " MentJl retJrddlion * Congenital hypothYroidism
* Small head. flat round face * HypotoniJ ^ Cirls fertile. bor -. .t"riie.
* Morrgoloid slartt of eves * Alzhein-rer disease after Orthopedic problems
* Depressed nasal bridge 25 t0 years oi age. *
Joint laxity, dislocaiion oi hiP,
+ lnner epicanthal folds alldntoaridl dislot ation
Psychiatric
* Protruding tongue * Reactive depression in adolescents Hematological system
Ocular system and young adults * Transient leukemoid reaction
* Brush{ield spots * Autistic behavior * Acute leukemia k2 yr, megakaryocytic
* Congenital cataract AML, M7; >2 yr, ALL)
Cardiovascular system (CVS)
* Nystagmus * Congenital heart disease. mainly septal clefects, Bv I 5 vi'. r'isk ot letrl.emia diminishes
* Strabismus * Congenital thrombocytopenla
especially of the endocardial cushion type,
' Refractive errors TOF, PDA. Castrointestinal system (ClT) ' Neondldi jaundice
* Cataract after 25 years of age. * Atresia of gut (duodenal, esophageal, 'Polycythemi,r.
Ears ,tnd analr Respiratory
' smrll. mi:shapen. low set * HirschsprunB disease t lnciderrce of pneumonia due to
' Dealne.s,'cencorineural/( ottduclir e'. decreased in-rmun ity, muscu lar
Cenitalia
Hands and feel hypotonia and associated congenital
'Simian crease
' Small penis
'Cryptorchidism heart disease
' :hofl broad hand: General
- Hvpoplasia oi middle phalanx of 5th finger lmmune syslem
* Frequent infection, autoimmune diseases, Short statu re
* ^ Cop betr.r,een 1st and 2nd toes
 ESSENCE OF PEDIATRICS

Table 22.5: Methods of Prenatal Diagnosis of Down Childhood: Short stature, short webbed neck with low posterior
Syndrome
hairline, shield-shaped chest and wideiy spaced nipples, cubitus
Maternal serum markers: valgus, pigmentary nevi, cardiac anomalies like coarctation of
Alpha fetoprotein Decreased (.14-1 6 weeks) the aorta; mental development is normal.
Unconjugated estriol Decreased (14 16 weeks), Triple Puberty: Turner syndrome may presenr for the first time
test
with primary amenorrhea and failure of secondary sexual
Human t horionic gonadolropins Increased ('14-1 6 weeks)
development. Streak gonads ar-e found with ultrasour-rd and at
(detection rate 60%)
laparotomy. The patients are almosr invariably sterile, bur men-
Pregnancy associated placental lncreased 1 st trimester
protein
strlration and secondary sexual development may be induced
by estrogen replacement. Girls with Tirr-ner syndrome have
Amniocentesis:
a normal lifespan. Hypertension and osreoporosis may be a
Farly Karyotype on cultured amniocytes
complicarion in adult life. Wilm tumor and colon cancer had
Conventional FISH on uncultured cells also been reported.
Cordocentesis (1 8-20 weeks) Fetal lymphocytes; culture for
karyotype; FISH on non-dividing
fetal cells
DIAGNOSIS
CVS rq-ll weeks' Direct preparation for karyotype, In the past, Tirrner syndrome was diagnosed solely by buccal
short-term culture
smear analysis. This technique is not always reliable. Therefore
Ultrasound rl I 20weeksr Nuchal fold > 4 mm, double chromosome analysis with full banding should be done.
bubble in abdomen, short femur,
cl inodactyly, macroglossia
Any girl presenring with short srarure with no clue, chro-
mosome analysis should be done.
Fetal echocardiography Atrioventricular canal defects
Fetal cells in maternal circulation FISH
DIFFERENTIAL DIAGNOSIS

Although Noonan syndrome is phenotypically similar to Turner

Chromosome abnormalities in Tirrner syndromc:
in having webbing of neck, shieldlike chest, low posrerior hair
45 XO 60o/o line, ptosis, antimongoloid slant of eyes; it has many dillbren-
Mosaic )OUXO l5o/o tiating points as shown in Thble 22.7. Noonan syndrome is an
Isochromosome Xq or Xp 70o/o autosomal dominant condition. Around 50% have murations
46 x del (X) 5o/o on chromosome 12. The karyorype is usually normal.
46 x ring (X) 5o/o
\fith Y chromosome 5o/o TREATMENT
It usuallv from mitotic rarher than meiotic error. It is not
arises
o Recombinanr growth hormone I anabolic steroid (oxandro-
associated with increase of recurrence in further pregnancies. lone 1.25 mg daily) increases height velocity.
The X chromosome is maternal in origin in 7 60/o and paternal
rn 24o/o. The presence of XY cell line in mosaic Tirrner syn-
Table 22.6: Clinical Features of Turner Syndrome
drome may be associated with virilization at birth and again
Typical features Cardiovascular system (1/3 cases)
at puberry and 20o/o risk of gonadoblastoma.
Short stature Bicuspid aortic valve (507")
Webbed neck Coarctation of aorta (20-50%)
Lymphedema in infancy Mitral valve prolapse
CtlNlCAt FEATURES (Toble 22.6) Low posterior hair line Dissecting aortic aneurysm
Cr-rbitus valgus ldiopathic hypertension
The clinical features depend on chromosome constitution of Conadal dysgenesis
the individuals. The parienrs with 45x Turner syndrome (clas-
sical Tirrner syndrome) show many characteristics of somatic Craniofacial anomalies Renal (40% cases)
Triangular face Horseshoe kidney,
abnormalities, whereas those with mosaicism and structural
Antimongoloid eye slant duplex ureter,
abnormalities of X chromosome (variant Thlner syndrome) Ptosis urinarv tract iniection
show minimal somatic abnormalities. However, short stature High arched palate
and streak ovaries are consranr findings irrespecrir.,e of chro- Multiple nevi Others
Convex nails Variable pst,chomotor developrnent
mosomai constitution.
Broad chest, w,idely spaced Learning disabilities
rripples
At birth: Lymphedema, especially of rl-re in the dorsum Autoirrmune thvroiditis
Scoliosis
hand, and redundant skin over the back of the neck. Deafness
Hydrops and cystic hygroma in urero and occasionaliy in rhe Clucose intolerance
neonate, Skeletal dysgenesis iKnee, wrist;

I
\
t
 GENETIC DISORDERS

Table 22.7: Features Differentiating Turner and Noonan The defect is caused by deletion of material from terminal
Syndromes end of short arm of chromosome 5 (5p). The affected chil-
dren have characteristic cat-like cry. This cry is related to the
Sex Only female Both sexes hypopiastic larynx and tends to lessen with advancing age and
growth of the larynx.
Chromosome analysis X-chromosome Normal 46xx
abnormal usually
45x0
Face l flangular lace Broad forehead,
CtINICAt FEATURES
Hypertelorism,
Epicanthic fold,
e General: Low birth weight, profound mental retardation,
Micrognathia cat-like cry.
Ear Normal Abnormal low set
r Craniofacies: Microcephaly, round face, hypertelorism, epi-
canthus, antimongoloid palpebral fissure, microphthalmia,
Heighi Marked short stature Lower limit of normal
low set malformed ears, preauricular tags.
lntelligence Normal Often mental
retardation
o Thorax Congenital heart disease (occasional).
Cardiac and renal Pulmonary stenosis
o Pelvis and abdomen: Inguinal hernia, diastasis recti, small
Coarctation of aorta
Bicuspid aortic valve ASD
iliac wings.
Horseshoe kidney, No renal anomaly o Hands and feet Short metacarpals or metatarsals, partial
duplex ureter syndactyly, pes planus, simian crease.
Sexual maturation Streak gonad, girls ln girls, sexual
invariably sterile maturation may be
Prognosis: Many patients can survive up to adulthood.
delayed by 2 yr.
ln boys, It may
be normal or
hypogonadism.
Cryptorhidism in 2/3
boys. This syndrome was first described by Klinefelter in 1942 with
characteristic features that become evident at adolescence.
Incidence is 1:1000 ar bifih.
o Replacement therapy: Approximately I in 1000 newborn males has a 47 xxy
karyotype and an additional 1.2 per 10,000 show 46 Wl47
) Estrogen 0.3-0.625 mg daily for 3-6 months to induce
)O(Y mosaicism. The extra X is maternal in origin in 670/o and
puberty. The estrogen then is cycled (taken on days
paternal in 33o/o. Advanced maternal age results in increased
1-23), pius
meiotic non-dysjunction resulting in Klinefelter syndrome.
o Progestin 5-10 mg daily is then added (taken on days
The classical Klinefelter syndrome has 47XXY chromosomal
10-23).In the remainders of the calendar month during
constitution. In the so-called variant Klinefelter syndrome,
which no treatment is given, withdrawal bleeding usually
there are multiple X chromosomes or mosaicism. The greater
occurs.
the number of chromosomes from )O(Y to )OOOry, the more
o Surgery for Co Ao. severe the mental retardation and other clinical features.
o Psychological support.
Clinical features of Klinefelter syndrome (47 )C{Y):
o Successful pregnancies have been carried out using ovum
donation and in vitro fertilization. o Thll (+10 cm on the average)
o Eunuchoid long lower limbs
PROGNOSIS o Small resris, gynecomastia
a Breast cancer (4olo)
45X or 45X mosaicism have a low fertility rate and those who a Small phallus, cryptorchidism, hypospadiasis in some
become pregnant have a high risk of fetal wastage (i.e ., abor- a High FSH, LH, and low testosterone
tion), furthermore their live born offsprings have an increased a Behavioral and psychiatric disorder, antisocial immature,
frequency of chromosomai abnormalities. aggressive
JIq ro-. problems with learning, speech and social adjust-
ment: criminal propensiry.

DIAGNOSIS
Cri-du-chat syndrome is one of the most common chro-
mosomal deletion syndromes, with an estimated incidence The diagnosis should be suspected in prepubertal children
of 1 in 50,000 births. About 70o/o of the affected are who have long legs, smaller than normal testes, small phallus,
temales. learning disorders, delay in language development, mental
ESSENCE OF PEDIATRICS

retardation, or psychosocial behavior problems. Diagnosis of CHARACTERISTICS

the syndrome in patients after puberty is not difficult by the
findings of the typical phenotype of tall stature, incomplete o Growth Overgrowth postnatally
virilization, smali firm testes, and elevated serum gonadorropin o Craniofacial - Large ears
concentration. The diagnosis can be confirmed by the finding Prominent forehead
of 47, WY karyotype. Testicular biopsy after puberty reveals Long face
hyalinization of seminiferous tubules, adenomarous clumping Prominent jaw
of Leydig cells and azoospermia. Macrocephaly (mild)
High arched palate
Long chin
TREATMENT o Neurologic Mental retardation
It has been suggested that treatment with testosterone o Behavioral Temper tantrum
should be started by l1-I2 years ofage to initiate puberty
Hyperactivity
and prevent physical and psychological complications Autism
Extreme shyness
of hypogonadism. At the same time, this would prevenr
excessive adult height. tearmenr could be started with
e Others ]oint looseness
Pes planus
50 mg of testosterone enanrhare IM monthly when the
bone age is 11 or 12, increasing to 100 mg IM monthly Soft skin
when the bone age is 14, and to 200-250 mg, every 3 Mitral valve prolapse
or 4 weeks, when full virilization is desired or when r Genitalia Macroorchidism (males) after puberty
growth is ending.
o Inheritance X-linked with premutations and full
mutations
Psychologically disturbing or persisting gynecomastia should
be corrected by reduction mammoplasty.
Eariy intervention for learning and behavioral disorders DIAGNOSIS
may be beneficial.
Suspect fragile X in male or female with mental retardation
and phenotypic change. Diagnosis is confirmed by DNA
PROGNOSIS testing. Cyrogeneric srudies, which are time consuming and
less sensitive, can be done when DNA testing is not feasible.
o Infertiliry is common, but mosaics may be fertile.
o There is language delay in 50olo of cases.
o 30o/o have delayed emorional maturity, learning, and
behavioral problem. TREATMENT
o There is increased incidence of diabetes.
No treatment excepr for supportive measures for
psychological and behavioral disorders. No rreatment needed
for overgrowth.
Feature of some less common chromosomal disorders
(trisomy 18, trisomy 13, and XIY syndrome) have been
Fragile X syndrome or Martin*Bell syndrome is the second summarized ]n able 22.8.
most common genetic cause of mental retardation after Down
syndrome, with a frequenry of I in every 7250 males and 1
in every 2500 females.

ETIOTOGY Phenylketonuria (PKU) is an aurosomal recessive metabolic

The rerm fragile X syndrome derives from the presence of a for the hepatic enzyme phenylalanine hydroxylase (PAH), ren-
fragile site expressed as an isochromarid gap in the metaphase
chromosome at map position Xq 27.3 on rhe distal portion
of the long arm of X chromosome in a proportion of ceiis of
affected individuals.
In normal person, trinucleotide CGG repeats range from 6
to 45, but in premutatio n 50-230 copies, and in full murarion
trinucleotide repears are >230 copies in FMR,I gene in long
arm of chromosome.
genetic disorder characterized by an error in the genetic code
dering it nonfunctional. This enzyme is necessary to metabolize
the amino acid phenylalanine (Phe) ro rhe amino acid ryro-
sine. \[hen PAH enzymatic activity is reduced, phenylalanine
accumulates and is converted into phenylpyruvate (also known t
as phenylketone), which is detected in the urine. The PAH
gene is located on chromosome 12 in the bands 12q22-q24.1. a
I
More than 400 disease-causing murarions have been found in
the PAH gene. PAH deficiency causes a specrrum of disorders, \
i
rl
r!
 !

GENETIC DISORDERS

Table 22.8t Features of Some Less Common Chromosomal day research now has shown diet alone may not be enough to
Disorders prevent the negative efFects of phenylaianine levels. Optimal
treatment involves iowering blood Phe levels to a safe range
and monitoring diet and cognitive development. Lowering of
Trisomy 1in Mental retardation, hypertonia,
18 (Edward 8000 births failure to thrive, low birth weight, Phe levels to a safe range may be achieved by combining a
syndrome) prominent occiput, micrognathia, low-Phe diet with protein supplements. There is currently no
low set malformed ears, congenital cure for this disease; however, some treatments are available
heart disease (mainly VSD and
PDAr, short sternum, diaPhragmal ic
with varying success rates. In general, PKU is detected through
hernia, renal anomalies, overlapping newborn screening and diagnosed by a geneticist. PKU clinics
of fingers, rocker bottom feet, death around the world provide care for PKU patients to optimize
in infancy Phe levels, dietary intake, and cognitive outcomes.
Trisomy 1in Mental retardation, microcePhalY,
1 3 (Patau 20,000 births cleft lip + palate, midclle scalp
defect., microphthalmia,
syndrome)
SCREENING AND PRESENTATION
colobomata, low set malformed
ears, congenital heart disease,
polycystic kidneys, cryptorchidism,
Blood is taken from a 2-week-old infant to test for phenylke-
polydactyly, simian crease, earlY tonuria. PKU is normally detected using the HPLC test, but
death some clinics still use the Guthrie test. Most babies in developed
XYY 1.5 per nhhough more prevalent among countries are screened for PKU soon after birth' For HPLC, a
:yndrome 1000 inmates of high security institutions, cutoff of 30.0 mg/L may be used, with higher values defining
newborn male the syndrome is Iess strongly
phenylketonuria.
infanls associated with aggressive behavior
than previously thought. Mild menlal If a child is not screened during the routine newborn
retardal ion, behavioral problems screening test (typically performed 6_14 days after birth,
and tall staLure are usual features using samples drawn by neonatal heel prick), the disease may
present clinically with seizures, aibinism (excessively fair hair
and skin), and a "musty odor" to the babyt sweat and urine
(due to phenylacetate, one of the ketones produced). In most
cases, a repeat test should be done at approximately 2 weeks of
including classic phenylketonuria (PKU) and hyperphenylal-
age to verift the initial test and uncover any phenylketonuria
aninemia (a less severe accumulation of phenyialanine)'
Tetrahydrobiopterin-deficient hyperphenylalaninemia, a that was initially missed.
rarer form of hyperphenylalaninemia, occurs when PAH is Untreated children are normal at birth, but fail to attain
normal, but there is a defect in the biosynthesis or recycling early developmentai milestones, develop microcephaly, and
of the cofactor tetrahydrobiopterin (BH4) by the patient. This demonstrate progressive impairment of cerebral function.
cofactor is necessary for proper activiry of the enzyme. The Hyperactivity, EEG abnormalities and seizures, and severe
coenzyme (called biopterin) can be supplemented as treatment.
learning disabilities are major clinical problems later in life. A
"musty or mousy'' odor of skin, hair, sweat and urine (due to
Levels of dopamine can be used to distinguish between
phenylacetate accumulation); and a tendency to hypopigmenta-
these two types. Tetrahydrobiopterin is required to convert
phenylalanine to tyrosine, but it is also required to convert tion and eczema are also observed.
tyrosine to L-DOPA (via the enzyme tyrosine hydroxylase), In contrast, affected children who are detected and treated
which in turn is converted to dopamine. Low levels of dopa- are less likely to develop neurological problems or have sei-
mine lead to high levels of prolactin. By contrast, in classical zures and mental retardation, though such clinical disorders
PKU, prolactin levels would be relatively normal. are still possible.
Phenylketonuria can exist in mice, which have been exten-
sively used in experiments into an effective treatment for it. PATHOPHYSIOTOGY
The macaque monkefs genome was recently sequenced, and
the gene encoding phenylalanine hydroxylase was found to Classical PKU is caused by a mutated gene for the enzyme
have the same sequence that, in humans, would be considered phenylalanine hydroxylase (PAH), which converts the amino
the PKU mutation. acid phenylalanine to other essential compounds in the body.
Since its discovery, there have been many advances in its Other non-PAH mutations can also cause PKU' This is an
treatment. It can now be successfully managed by keeping the example of generic heterogeneity.
patient under ongoing medical supervision to avoid the more
+
serious side effects. If, however, the condition is left untreated, METABOLIC PATHWAYS
a it can cause problems with brain development, Ieading to Pro-
gressive mental retardation, brain damage, and seizures. In the The enzyme phenylalanine hydroxylase normally converts the
V past, PKU was treated with a low-phenylalanine diet. Latter- amino acid phenylalanine into the amino acid tyrosine. If this
It
I
lt

ESSENCE OF PEDIATRICS
reaction does not take place, phenylalanine accumulates and tyro-
sine is deficient. Excessive phenylalanine can be metabolized into
phenylketones through the minor route, a transaminase pathway
with glutamate. Metabolites include phenylacetate, phenylpyru-
vate, and phenethylamine. Elevated levels of phenylalanine in the
blood and detection of phenylketones in the urine is diagnostic.
Phenylalanine is a large, neutral amino acid (LNAA). LNAAs
compete for transport across the blood-brain barrier via the large
neutral amino acid transporter (LNAAI). If phenylalanine is
in excess in the blood, it will saturate the transporrer. Excessive
levels ofphenylalanine tend to decrease the levels of other LNAAS
in the brain. However, as these amino acids are necessary for
protein and neurotransmitter synthesis, Phe buildup hinders the
development of the brain, causing mental retardation.
TREATMENT
Prevention of mental retardation can be achieved by early
identification and restriction of dietary intake of phenylalanine.
Dietary restriction (i.e., milk with low phenylalanine content)
should be started along with breast milk very early in infancy.
Fruits, vegetables (except peas, beans) can be given without
restriction. Case management should be individualized.
Phenylalanine resrricrion in diet should be adjusted accord-
ing to blood phenylalanine level (3-8 mg/dl), which should be
tested weekly for the first 2-3 monrhs, biweekly during 3-6
months and thereafter monthly. Most of the children can be
given normal diet by 10 years of age. In some children, mod-
erate dietary restriction may have to be continued life long.
PROGNOSIS
Individuals with PKU who carefi.rlly comply with the dietary
management are able to live normal lives. However, women with
PKU who have discontinued dietary restriction are at substantially
increased risk for having children with birth defects, especially
microcephaly, congenital heart disease, and mental retardation.
Decarboxylation of leucine, isoleucine, and valine is carried
out by a complex enzyme sysrem. Deficiency of this enzyme
system causes MSUD, named after the sweer odor of maple
syrup found in body fluids, especially urine. Newborn infants
with this autosomal recessive disorder present in the first week
of life with vomiting, then alternating decreased and increased
tone, proceeding to death within a few weeks if left untreated.
Diagnosis is suggested by increased excretion ofthe branched-
chain amino acids-valine, leucine, and isoleucine in the
urine, that is confirmed by the presence of the three essential
branched-chain amino acids in blood.
Management in acute stage consists of provision of high calories
intravenously and doing peritoneal dialysis ro remove excess
!
amounts of branched-chain amino acids from circulation. The
diet should be low in these amino acids and synthetic formulas
are available. The long-term outcome is guarded; some forms
of MSUD respond ro rhiamine.
These are groups of disorders inherited as autosomal recessive
traits due to deficiency of enzymes involved in the various steps
of conversion of galactose to glucose. There are three forms of
this disorder-galactosemia I (also called classic galactosemia),
galactosemia II, and galactosemia IIL
CTASSIC GATACTOSEMIA
Etiology
Results from deficiency of galactose-1-phosphate uridyl trans-
ferase. The absence of this efizyme results in accumulation of
galactose-l-phosphate. This accumulation causes injury to the
kidney, liver, and brain.
The clinical manifestations in the newborn include jaundice,
hepatomegaly, vomiting, hypoglycemia, convulsions, lethargy,
irritability, feeding difficulties, poor weight gain, aminoac-
iduria, cataract, hepatic cirrhosis, ascites, splenomegaly, and
mental retardation. Gram-negative sepsis, especially with E
coli, is common.
In less severe cases, presentation occurs in childhood with
mental retardation, bilateral cataract, and cirrhosis of liver.
PATHOGENESIS
Administration of galactose resuks in increased galactose,l-
phosphate, which inhibits phosphoglucomutase resulting in
impairment of conversion of glycogen to glucose and thus
produces hypoglycemia. Galactose-l-phosphate is responsible
for liver and brain damage, whereas galactilol (a product of
alternative pathway) produces cataract.
DIAGNOSIS
The diagnosis is made by demonstrating a reducing substance
in several urine samples collected while the parienr is receiving
milk. So, Benedicts tesr is posirive, but glucose oxidase test
for glucose is negative. The reducing substance found in the
urine can be identified as galactose by chromatography or by
enzymatic test specific for galactose. Confirmation of diagnosis
is made by estimating transferase enzymes in erythrocytes.
TREATMENT
t
o teatment consists of total elimination of galactose from 1
I
diet (milk and milk products) right from birth to avoid liver L
injury mental retardarion, cataract, and recurrent hypogly- t
cemia. Soya based milk such as pregestimil can be used. t
t
Ii
 t
GENETIC DISORDERS

o Supplementary vitamin and mineral must be given.

o Milk elimination from the diet of pregnant women carrying
fetus with galactosemia prevents minor cognitive function
disorders and ovarian failure noticed in treated children.
PROGNOSIS
Marfan syndrome is an autosomal dominant (sporadic in
l5-30o/o) disorder with complete penetrance but variable
expressiviry with an incidence of 1 in 10,000 people and high
degree of mutation.

Tleated individuals often have normal intelligence if the diag- ETIOTOGY

nosis is made and treatment is initiated early. However, there
is an increase in the incidence of learning disorders even in
treated individuals. Affected women have a high incidence of
ovarian hypofunction and premature ovarian failure.
ljntreated infants often die, either from inanition or Esch-
erichia coli sepsis. Untreated survivors suffer from growth
retardation, mental retardation, and cataract.
The basic defect in Marfan syndrome has now been traced to
a defective fibrillin gene mapped to chromosome 15. Fibril-
lin is a recently discovered connective tissue protein found
in microfibrils, a constituent of elastic tissue and abundant
in tissues affected in Marfan syndrome, including the aorta,
suspensory ligament of the lens, and periosteum.

CLINICAT FEATURES
Skeletal Long thin face, limbs, and digits
The second most common inborn error of metabolism. Mode Disproportionate tall stature
of inheritance is autosomal recessive (AR). Occurs due to defi- (reduced US/LS ratio,
ciency of cystathionine B-synthetase or due to defect in the arm span to height ratio >1.05)
biosynthesis of methyl 8,, resulting in increased concentration Disproportionate tall stature
t- of homocysteine and its dietary precursor methionine in blood. High arched palate
I deformity (asymmetric pectus excavatum/
p
CtINICAI carinatum)
I FEATURES
I Hypermobile joints (wrist and thumb signs)
i- o Asymptomatic in infancy. Scoliosis
o Ectopia lentis (dislocated lenses), mental retardation, Cardiac Aortic root dilation
thromboembolic disorders of arteries and veins may lead Mitral valve prolapse
to myocardial infarction and CVD; skeletal abnormalities fusk for aortic aneurysm rupture
(pectus excavatum, arachnodactyly, scoliosis' biconcave Ophthalmologic Lens subluxation
vertebrae, osteoporosis). Flat corneas
o Microcephaly, seizure, myopia, cataract, glaucoma, retinal Severe myopia
detachment, optic atrophy may also occur. Pulmonary Spontaneous pneumothorax
Emphysema
CNS Dural ectasia
DIAGNOSIS
Homocystinuria in freshly passed urine. High serum methio- DIAGNOSIS
nine and homocysteine level. Megaloblastic anemia when
Vit-B,, deficiency is there. Cultured fibroblast study for specific Only70-B5o/o of affected people have an affected parent, owing
diagnosis. to the high incidence of new dominant mutations in Marfan
syndrome. Variability in severiry and in manifestations aiso can
make it difficult to prove a positive family history. Usually, it
TREATMENT
is necessary to examine the parents and siblings of a possibly
. 1. Vitamin Bn responsive rype (50olo): Vit. Bu 100-150
affected individual and to obtain and review medical records
mg/d. of family members who died suddenly or of unknown causes.
7 2. Vitamin Bn non-responsive tyPe: Low methionine diet Diagnostic criteria: To make the diagnosis at least rwo of the
may prevent mental retardation and delay lens dislocation. following major manifestations must be present:
2 Betaine 250 mglkgld increases methylation of homocys-
I i) Typical skeletal findings
teine to methionin€.
3 ii) Typical ocular findings
I \titamin B,, I mg IM every alternate day is indicated in some iii) Typical cardiovascular findings
V patients. iv) Positive family history

I
I
 ESSENCE OF PEDIATRICS
Once diagnosis is made, the other manifestations should be
sought by skeletal measuremenrs, ophthalmologic evaluarion,
and echocardiography.
TREATMENT
Therapy of Marfan syndrome focuses on prevenrion of compli-
cations and genetic counseling. Individuals with or surpect.d
of having Marfan syndrome should be followed with annual
physical examinarions, ophthalmologic evaluations, and echo-
cardiography.teatment with B-blockers has been efGctive in
reducing the progression of aortic root dilatation, and some
cardiologists suggesr srarring them as soon as the diagnosis is
made.
It of the neurocuraneous disorders occurring in l:4000 of
is one
population and transmitted by autosomal dominant inheritance
with variable expression. 50o/o of cases of NF are due to new
mutations. There are two distinct forms of neurofibromato"i.-
NF-l and NF-2.
CLINICAL FEATURES AND DIAGNOSIS
A. Neurofibromatosis -l (NF-l; von Recklinghausen
disease): The diagnosis of NF-l is establisheJ by the
presence of two or more of the following:
1. Six or more cafe-au-lait spots >5 mm in greatest
diameter in prepubertal individuals and >15 mm in
postpubertal individuals (in l00o/o).
2. Two or more neurofibromas of any type or one plexi,
form neurofibroma (usually in temporal or orbital
region of face with hyperpigmentation).
3. Two or more Lisch nodules (pigmented hemarromas
of iris) (r90o/o of cases) detected by slit lamp.
Axillary or inguinal freckling (hypopigmented
2-3 mm in diameter).
5. Optic glioma (can be detected by CT scan as thicken-
ing or mass originating from nerve head).
6. A distinctive osseous lesion (e.g., sphenoid dysplasia
or thinning of long bone correx with or without
pseudoarthrosis).
7. A first degree relative with NF-l according to
above criteria.
B. Neurofibromatosis-2 (NF-2): Diagnosis of NF-2 is estab-
lished by the presence of:
1. Bilateral WII nerve masses consisrent with acoustic
neuroma as detected by CT scan or MR[.
2. A first degree relative with NF-2 and either unilateral
VIII nerve mass or two of the foilowing: neurofibroma,
meningioma, glioma, schwannoma, or juvenile pos-
terior subcapsular lenticular opaciry.
INVESTIGATIONS
Screening of visual change, hearing loss, learning disabilities,
skeletal survey should be routine. All asymptomatic children
sho_uld undergo annual neurological examination including
auditory and visual tests. A CT scan should be done if there
is clinical suspicion of tumor.
coMPHCAT|ONS
.:
There is high incidence of CNS rumors such as opric gliomas,
meningiomas of brain and spinal cord, neurofibroma, asrrocy_
toma in NF- I . Neurofibroma may turn into neurofibrosarcoma \
or malignant schwannoma. The incidence of pheochromocy_
toma, rhabdomyosarcoma, leukemia, \Wilms rumor is higher in
child with NF than in general population. Children with NF_t
are susceprible to neurologic compiications such as learning \
i
disabllities, seizures, hydrocephalus, macrocephaly, hemiparesii
i
intellectual defi cit, psychological disturbances.
i
TREATMENT
No specific rrearmenr. Management includes genetic counseling
and early detection of treatable conditions or complicationsl
Prenata-l diagnosis is possible in some cases.
PROGNOSIS
Depends on complications. If tumors are confined to peripheral
nerves only, a normal life without deficit is likelv.
It is most likely a sporadic disease, although familial cases have
been described.
area
CTINICAL FEATURES
o Portwine stain (capillary hernangioma) occurs unilaterally
over forehead and maxillary area (usually in the distribution
of first branch of trigeminal nerve), occasionally bilateral.
o Contralateral focal seizures, focal, tonic-clonic seizures +
hemiparesis. :
the o Calcification of correx and sub-cortical strucrures.
o Glaucoma on the same side as the skin lesion in 50%o cases.
o Mental retardation and learning disabiliry may occur in
50olo cases. :
INVESTIGATIONS \
I
o i
Skull x-ray shows intracranial calcification in the occipito_
parietal region. Most patienrs assume rail road ,r"& o,
I
serpentine appearance.
 GENETIC DISCRDERS

CT scan shows extent of calcification, usually associated h. Others: Rhabdomyosarcoma of heart (in 50olo), hem-
with unilateral cortical atrophy and ipsilateral dilatation of artoma or polycystic disease of kidney, cysric changes
lateral ventricle. in lungs.

DIAGNOSIS
TREATMENT
High index of suspicion in a child with infantile spasm or a
Anticonvulsant therapy. careful search for typical skin and retinal lesion in all patients
For patients with well-controlledseizures and near normal
with seizure disorder gives a clue. CT scan or MRI of head
development, treatment is conservative. confirms diagnosis.
Hemispherectomy or lobectomy in recalcitrant seizures, and
to prevent development of mental retardation.
TREATMENT
a Laser therapy for clearance of porrwine stain.
a Special education facility. a Control of seizures.
a teatment of glaucoma, if any. a Baseiine USG of kidneys, echocardiogram and chesr x-ray
for associated kidney, heart or lung lesions, and appropri-
ate treatment.
If raised intracranial pressure (suspect obstruction of foramen
of Monro by tuber), immediate investigation and surgical
It is a neurocutaneous disorder inherited as autosomal intervention.
i dominant trait. 50o/o of cases are sporadic due to new
(
mutations. The TS gene is located on chromosome 9q34.
Characteristic brain lesions are tubers, which are usually
located in the subependymal region of cerebral hemisphere
and undergo calcification and may project into ventricles It is a disorder of endochondral bone formation with resultant
producing "candle dripping" appearance. T[ber may press poor development of cartilage column of growth and base of
foramen of Monro causing obstruction of CSF flow and the skull. Membranous bone formation and arricular carrilage
hydrocephalus. are normal. It is inherited as autosomal dominant, but over
90olo cases are due to fresh mutations.
CtINICAI FEATURES
CtINICAt FEATURES
1. Infancy:
Infancy: Infants are dwarfed, limbs are short proximally and
a. Infantile spasm with hypsarrhythmic EEG.
transverse skin creases. Limbs are bowed and bone ends are
b. Ashleaf hypopigmented macules better visualized by
bulbous. The feet and hands are broad and short. Fingers
\7oods ultraviolet lamp (in >9070 cases)
are almost equal in length with limitation in full adduction
c. Calcified tubers in periventricular region detected by
giving trident hand deformity. Cartilaginous base of skull
CT scan (better detected at3-4 year of age).
is poorly formed but vault of skull grows normaily giving
d. Myoclonic epilepsy
rise to iarge cranium, frontal bossing, small face, depressed
e. Mental retardation
nasal bridge. Infant shows delayed motor development with
2. Childhood: hypotonia.
a. Generalized seizures.
Older child: Lumbar lordosis, thoracolumbar kyphosis, pro-
b. Ashleaf hypopigmented macuies.
tuberant abdomen, waddling gait, bowing of legs. Mid point
c. Sebaceous adenoma (develop by 4-6 year)-tiny red
of body iies above umbilicus. Fingers do not reach greater
nodules over nose and cheeks. Later, they enlarge, trochanter. Intelligence normal. Neurological complications
coalesce, and assume {leshy appearance.
such as hydrocephaius, cord compression leading to paraplegia
d. Shagreen patch: Characteristic of TS. Roughened, are frequent and serious.
raised lesion with orange peel consistency in the
: lumbosacral region.
INVESTIGATIONS
t e. Subungual fibromas, periungual fibromas.
a
|,
I Retinal lesions: Mulberry tumors of optic nerve head or X-ray changes: typical; long bones are short and thick,
round, flat grey colored (phakoma) in region of disk. proximal bones are more affected than distal; metaphyses
I g. Brain tumors-malignant astrocltoma. are splayed and have central v-shaped notch, which forms
r
I
I
 I
ESSENCE OF PEDIATRICS

as socket into which epiphyses protrudes (Ball and socket
deformity). Metacarpals, metatarsals, phalanges are short
and stubby. Mid face is hypoplastic. fubs are short. X-ray
spine shows that interpeduncular space narrows from above
downwards (opposite normal) with tapering of pedicles
(more in lumbar region). Vertebral bodies are small cuboidal
with beaking of L7,L2. Posterior surface of vertebral bodies
are concave. Pelvis shows narrow sacnlm, small iliac wings,
horizontal acetabular roof, narrow sciatic notch.
DI FFERENTIAT DIAGNOSIS
Hypochondroplasia: Craniofacies normal; hands short but
not trident, short limbs (more proximal).
Morquio disease: Craniofacies normal; short neck; deformed
chest, short trunk, long limbs, genu valgum.
TREATMENT
No specific treatment; hydrocephalus require shunting; ieg
lengthening surgery may be tried.
Prognosis: Achondroplasts have good general health, is physi-
cally active, fertile. Final adult height 437 cm. Increased
tendency to disc degeneration and herniation.
Glycogen storage diseases are a group of autosomal recessive
disorders caused by lack of enzymes involved in glycogen
synthesis or breakdown with resultant buildup of glycogen in
tissues. The most common one is von Gierke disease.
voN GTERKE DTSEASE (GSD TYPE t)
so severe as to precipitate convulsions and even lead to mental
impairment. Trere is tendency to lactic acidosis, hyperlipidemia,
acidemia, gout, bleeding. There may be glycosuria, aminoac-
iduria due to renal damage.
Diognosis
In child with gross hepatomegaly with rounded facies and
repeated hypoglycemic attacks, GSD type I should be suspected
and following resr may be done:
o Flat blood giucose curve is obtained when adrenaline (0.3-
0.5 ml subcutaneously) or glucagon (1 mg intravenousiy)
and blood glucose are measured for t hour (normally blood
glucose level rises 40-600/o above fasting).
o Glucose tolerance test shows abnormal high rise and delayed
fall.
o Conclusive proof by study of specimen of liver removed ar
laparotomy, showing (i) liver glycogen contenr over 5Vo of
wet weight, (ii) glycogen of normal srrucrure, (lll) absent
or very low glucose-6-phosphatase acriviry.
Differential diagnosis: Pompe disease and Mc Ardle syndrome
(Table 22.9).
Treolment
o Frequent feeds by day time and night time; glucose infusion
or intragastric feeding help a lot. Catch-up growth occurs
and liver regresses.
o In those who develop hyperuricemia, allopurinol prevents
uric acid nephropathy.
o Episodes of severe metabolic acidosis, often precipitated
by infection should be treated by NaHCO., IV giucose,
and antibiotic.

The absence of glucose-6-phosphatase in liver results ln Prognosis

accumulation of glucose-6-phosphate in liver.
Clinicol Feqlures
Gross hepatomegaly, renomegaly, rounded "doll face", stunted
growth, normal mental development. Hypoglycemia may be
If untreated, hypoglycemia, lactic acidosis lead to death in
infancy.
Those who survive infancy tend to improve spontaneously
after adolescence though some ultimately develop gour, uric
acid nephropathy, and hepatic adenomas.

Table 22.9t Features Differentiating CSD Type I , Pompe Disease, and Mc Ardle Syndrome

von Cierke disease Clucose-6-phosphatase lnfancy Hepatomegaly, Prevention of

renomegaly, hypoglycemia, complex
hypoglycenria, acidosis carbohydrate {corn
starch), continuous feeds
Pompe disease Acid maltase lnfancy Cardiomegalv, None available, lethal by
.l
hepatomegafy, hypotonia year of age
Mc Ardle syndrome Skeletal phosphorylase Late childhood, Fatigue, crampsi Avoidance of exercise \
adolescence, adulthood myoglobinuria stress
\
t
t
a
 GENETIC DISORDERS

long term, although there is a risk of spinal cord compression

due to progression of the skeletal involvement.
The mucopolysaccharidoses are a group of inherited disor- The diagnosis is confirmed by the presence of keratin sulfate
ders caused by incomplete degradation and storage of acid in urine and deficiency of either galactosamine-6-sulphatase or
beta-galactosidase.

H
mucopolysaccharides (glycosaminoglycans). Accumulation of
mucopolysaccharides in various organs results in the clinical
manifestations. Dermatan sulfate, heparan sulfate, and keratan CtINICAt FEATURES
sulfate are the major mucopolysaccharides involved in the
Infants with Hurler syndrome appear normal at birth, the
pathogenesis of the mucopolysaccharidoses. Specific degrada-
features start appearing during the first year of life. The
tive lysosomal enzyme deficiencies have been identified for all
features include large dolicocephalic head with frontal
the mucopolysaccharidoses.
bossing, depressed nasal bridge, flat and broad nose,
Mucopolysaccharides are major components of the inter-
facial features become progressively coarser, clouding of
cellular substance of connective tissue, hence bony changes
the cornea, developmental regression, mental retardation,
are characteristic of the MPS. The skeletal deformities seen
hepatosplenomegaly, exaggerated kyphosis, and umbilical
on roentgenograms are referred to as dysostosis multiplex,
and inguinal hernias. The downhill course continues rapidly
which includes large dolicocephalic head, thickened calvarium,
after the second or third year of life, they usually die by
hyperostosis of the cranium, boot- or J-shaped sella turcica,
their early teens.
thickened medial third of ciavicle, ovoid shaped vertebral
bodies in the lower thoracic and upper lumbar regions, while
their upper margins are hypoplastic with beaklike projections
DIAGNOSIS
on their lower anterior margins, resulting in gibbus deformity, The diagnosis is suggested by clinical and roentgenographic
spatulated ribs. X-ray hip shows faring of iliac bones with finding of dysostosis multiplex and urinary excretion of derma-
shallow acetabulae, progressive coxa valga deformity. X-ray tan and heparan sulfates. Definitive diagnosis requires detection
hand shows tapering of the terminal phalanges and widening of a-L-iduronidase dcfciency in white blood cells, serum, or
at the distal ends and tapering at the proximal ends of the cultured skin fibroblasts. Diagnosis of Hunter syndrome is
metacarpals. Irregular widening and cortical thinning in the
confirmed by enzyme studies showing iduronosufate sulfatase
long bones and the articular surfaces of the radius and ulna defciency in serum, \MBCs, or cultured fibroblasts.
face one another forming a V Humerus may be angulated and
glenoid fossa like acetabulam may be shallow.
TREATMENT
The central nervous system, cardiovascular system, liver,
spleen, tendons, joints, and skin may be involved. Bone marrow transplantation is the rrearment. Hurler disease
The MPS follows an autosomal recessive mode of inheri- benefit much by improvement of intellectual abilities, clear-
tance, with the exception of Hunter syndrome, which is ance of cornea, regression of liver and spleen, disappearance
inherited as an Xlinked recessive trait. of mucopolysacchariduria. Skeletal changes do not improve
unless bone marrow transplantation is done early in life. In
MPS types: Sanfillippo patients, intellect does not improve. Experience
with other forms of MPS is limited.
Genetic counseling: Prenatal diagnosis and carrier detection
Hurler ++ + ++ + is available in all forms of MPS.
Hunter ++ r r
Sanfillippo + + ++ PROGNOSIS
Morquio ++ + +
Children with Hurler die by early teens and Hunter by late
teens due to CCF or repeated respiratory infections. Children
Hurler syndrome (MPS IH): This is the most severe form of with Sanfillippo die by middle reens due to rapid neurological
the MPS. The basic defect is a deficiency of a-L-iduronidase, deterioration. Children with Morquio syndrome die in third
which leads to accumulation of dermatan and heparan sulfates or fourth decade from cor pulmonale.
in tissues and their urinary excretion. Almost every tissue in
the body is affected.

Morquio syndrome (MPS IV): Children with Morquio syn-

drome present in the second or third year of life with skeletal Lipidoses are a group of inherited merabolic disorders in which
abnormalities that include short stature, thoracic deformiry lipid content of tissue and plasma is elevated. Accumulation
and curvature of spine. Intelligence is normal and survival is of lipid in cells (histiocytes) gives them appearance of large
ESSENCE OF PEDIATRICS
foamy cells. As a result, liver, spleen, lungs, and marrow are
infiltrated with foamy cells. In some lipidoses, neurological
function is impaired either due to deposition of lipids in
brain or due to metabolic defects. Lipidoses with deficient
enzymes are Gaucher disease (glucocerebrosidase), Niemann-
Pick disease (sphingomyelinase), Krabbe disease (galactocer-
ebrosidase), Metachromatic leukodystrophy (arylsulfatase A),
Fabry disease (alpha-d-galactosidase). Ali ofthese except Fabry
disease (X-linked) have autosomal recessive inheritance.
GAUCHER DISEASE
In Gaucher disease, glucosylceramide (glucocerebroside) accu-
mulates in the cell of reticuloendothelial system. The cerebroside
laden cells are large with eccentric nuclei. Their cytoplasm
appears like crumpled silk (Gaucher cells). The disease occurs
in three forms:
Type l-chronic non-neuropathic is the most common.
Present at any age with gross splenomegaly, moderate hepato-
megaly. There are signs of hypersplenism such as progressive
anemia, leukopenia, thrombocytopenia. X-ray shows expansion
of long bones, especially flaring of metaphyseal ends of femur
and humerus with thinning of the cortex. The eyes show a
yellow brown wedge shaped elevation with base towards cornea
(pinguecula). Characteristic brownish pigmentation may be
presentin skin of face, neck, hands, and legs.
Type 2-infantile, acute neuropathic: Onset in infancy. In
addition to hepatosplenomegaly, infant develops hypotonia,
catatonia, opisthotonos, dysphagia, respiratory difficulty. They
show characteristic triad of retroflexed head, trismus, and squint.
Death occurs before 3 years of age.
Type 3-juvenile subacute neuropathic: Onset is in the
second year; course is subacute; child shows spasticity, ataxia,
ocular palsies, progressive dementia and seizures in addition
to hepatosplenomegaly. Death is inevitable.
Diognosis
o Suggested by demonstration of Gaucher cell (large foam
cell) in materials from bone marrow spleen, lymph nodes.
o Confirmed by glucocerebrosidase level in leukocytes and
skin fibroblast.
Treolment
r Enzyme replacement with glucocerebrosidase (naturai or
recombinant) is effective but expensive. No treatment avail-
with neurologic involvement.
able for cases
o Genetic counseling after prenatal diagnosis is possible.
NIEMANN-PICK DISEASE
Infants with Niemann-Pick disease present with faiiure to
thrive and hepatomegaly and can be found to have cherry-
red spot on their macula. Developmental regression rapidly
progresses by the end of the first year, with death by the age
of 4 years. A char:-cteristic finding of foam cell in the bone
marrow due to sphingomyelin accumulation. Confirmation of
the diagnosis is by demonstrarion of deficiency of the enzyme
sphingomyelinase
Newborn screening is the process of testing newborn babies
for treatable genetic, endocrinologic, metabolic, and hemato-
logic diseases.
Common considerations in determining whether to screen
for disorders:
o A disease that can be missed clinically ar birth
o A high enough frequency in the population
r A delay in diagnosis will induce irreversible damages to
the baby
o A simple and reasonably reliable resr exists
o A treatment or intervention that makes a difference if the
disease is detected early
The first test to be universally mandated across the US was the
Guthrie test for phenylketonuria, others are congenital adrenal
hyperplasia, congenital hypothyroidism, sickle-cell disease,
galactosemia, maple syrup urine disease , homocystinuria,
biotinidase deficiency.
A drop of blood is usually obtained by pricking the heel of
a newborn infant in a hospital nursery on rhe sixth or sevenrh
day of life (end of the first week). The blood is collected on a
piece of filter paper and sent to a central laboratory.
The term Guthrie testis sometimes used in a broader sense
to describe dried blood spor testing, which can be used to test
for several other conditions. The filter cards are sometimes
called "Guthrie cards". The sample for the Guthrie rest can be
analyzed for the following seven conditions: phenylkeronuria,
maple syrup urine disease, galactosemia, biotinidase deficiency,
congenital adrenal hyperplasia, cystic fibrosis, and congenital
hypothyroidism.
GENETIC TECHNOTOGY AND ITS
APPLICATION
Polymerose Choin Reoclion
The polymerase chain reaction (PCR) is a scientific technique
in molecular biology to amplif| a single or a few copies of a
piece of DNA across several orders of magnitude, generaring
thousands to millions of copies of a particular DNA sequence.
The method relies on thermal cycling, consisting of cycles of
repeated headng and cooling of the reaction for DNA melting
and enzymatic replication of the DNA.
PCR is now a common and often indispensable technique i
used in medicai and biological research laboratories for a variery
I
t
I
t
 GENETIC DISORDERS

of applications. These inciude DNA cloning for sequencing,
DNA-based phylogeny, or functional analysis of genes; the
diagnosis of hereditary diseases; the identificadon of genedc
fingerprints (used in forensic sciences and paternity testing);
and the detection and diagnosis of infectious diseases.
PCR permits early diagnosis of malignant diseases such as
leukemia and lymphomas. PCR aiso permits identification
of non-cultivatable or slow-growing microorganisms such as
mycobacteria, anaerobic bacteria, or viruses from tissue culture
assays and animal models. The basis for PCR diagnostic appli-
cations in microbiology is the detection of infectious agents
and the discrimination of non-pathogenic from pathogenic
strains by virtue of specific genes. Viral DNA can likewise be
detected by PCR.
and cloned. A vector is a piece of DNA that is capable of
independent growth; commonly used vectors are bacterial
plasmids and viral phages. The gene of interest (foreign
DNA) is integrated into the plasmid or phage, and this is
referred to as recombinant DNA.
Uses: Isolation of large quantities of protein, follicle-stimu-
ladng hormone (FSH), insulin, gror,tth hormone, which are now
available as recombinant products. Identification of mutations.
Diagnosis of affected and carrier states for hereditary diseases.
Tiansferring of genes from one organism to another. Mapping
of human genes on chromosomes.

Koryotyping Elsas JL, et al. Medical genetics. In Sodman and Sodman: Pathologic
Physiology 6'h ed. Igaku - Shoin/Saunders, 1981.
Karyotyping is a test to examine chromosomes in a sample of
Clayden GS, Hawkins RL (ed.). Paediatrics:Ileatment and Prognosis
cells, which can help identify genetic problems as the cause of 1" ed. New Delhi: Jaypee Brothers, 1989.
a disorder or disease. This test can count the number of chro- 3. Parthasarathy A (ed.). IA" Tbxtbook of Pediatrics 4'h ed. New Delhi:
mosomes and can look for structural changes in chromosomes. Jaypee Brothers, 2009.
The test can be performed on almost any tissue, including 4. Ghai OP (ed.). Essential PediatricsT'h ed. New Delhi: CBS Publish-
amniotic fluid and blood. ers, 2009.

Abnormal results may be due to a genetic syndrome or 5.
condition, such as Down syndrome, Klinefelter syndrome,
6.
Philadelphia chromosome, trisomy 18, Tirrner syndrome,
ambiguous genitalia, chronic myelogenous leukemia (CML) or 7.
other leukemias, developmental deiays, and mu.ltiple birth defects.
Recombinont DNA Technology
Recombinant technology begins with the isolation of a 9.
gene of interest. The gene is then inserted into a vector
H"y (J.) \X M (ed). Current Pediatric Diagnosis and Tieatment 74'h
ed. Connecticut: Appleton k. Lange, 1999.
Lees MM, 'Winter RM. Advances in genetics. Arch Dis Chlld
1996;75:346-50.
Dworkin PH (ed). NMS: Pediatrirs 4d' ed. Maryland: Lippincot,
lvilliams & Vilkin, 2000.
Hali JG. Chromosomal abnormalities. In: Behrman RE, ed. Nelson
Textbook of Pediatrics 16"' ed. Singapore: Harcourt Asia Pte Ltd',
2000.
Peter DT, Sian E. Emery's Elements of Medlcal Genetics 12'h ed.,
Elsevier, 2005.

:
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)

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CHAPTER 23
Common Surgical Problems

Chopter Contents

o It may involve soft palate and hard palate incompletely

Cleft lip occurs as a result of deficient or defective fusion of
frontonasal and maxillary processes during facial development.
Incidence is of about 1 in 600 live births. Cleft lip alone shows a
male:female ratio of 3:1. There is evidence of an increase in cleft
lip with older maternal age and lower socioeconomic groups.
r Commonly involves upper lips, may be unilateral (common),
bilateral, or central (rare). Cleft lip is called complete when
cleft extends to nostril.
o May be associated with cleft alveolus and cleft palate.
o Rarely lower lip cleft occurs, where the defect is in man-
dibular process, and it is in the midline.
PROBLEMS
Isolated cleft lip occurs without any functional disability except
cosmetic disfigurement. However, bilateral cleft lip causes some
difficulty in feeding as it is usually associated with cleft alveolus
and cleft palate also.
TREATMENT
Rule of 10: 10 weeks of age, 10 kg of body weight, and
l0 g% of hemoglobin are the usual three parameters for cleft
lip reconstruction. But it may be done at earlier age.
Millard repair is commonly practiced.
Failure of fusion of palatine processes of maxilla results in com-
plete or incompiete clefts of the primary or secondary palate
or both. Cleft palate is more common than cleft lip.
o It may involve only soft palate.
(behind the incisive foramen).
o It may be unilateral or bilateral, i.e., involving both nasal
cavities.
PROBTEMS
l
o Difficult feeding with nasal regurgitation and the babv is
unable to suck.
o Recurrent respiratory tract infections.
r Recurrent otiris media.
o Nasal intonation.
o Speech difficulty.
TREATMENT
Feeding by long handled spoon in upright position. Palatal
shield may be of help in preventing nasal regurgitation.
Feeds may be divided into frequent small feeds.
Respiratory tract infection and middle ear infection should
be treated promptly.
For surgical coffecrion, rule of 1 0 may be applied, i.e., 1 0 months
of age, 10 kg of body weight, 10 g% of hemoglobin.
In the treatment of cleft palate, timing is very important.
If operation is not done before the onset of clear voice and
speech, nasal intonation and speech defect may not be cor-
rected even after corrective surgery, because if the child is
accustomed once to nasal voice, it wiil continue. So, the
operation should be done befii'een 10 and 12 months by
a pediatric surgeon/plastic surgeon.
:
rt
I
Congenital diaphragmatic hernia occurs as a congenital weak-
I
ness at certain points of diaphragm, which permits entry I

of abdominal contents to thorax. Congenital diaphragmatic
hernia occurs in approximately I in 4000 live births. The
hernia occurs on the left side in 85-90o/o of most series.
Female:male = 2:1 .
ETIOLOGY
o Persistence of pleuroperitoneal canal (foramen of Bochdalek).
r Absence or weakness of diaphragmatic musculature or
paralysis of phrenic nerve leads to eventration of diaphragm.
o Hernia through foramen of Morgagni.
CLINICAT FEATURES
o In severe cases, symptoms appear just after birth with respira-
tory distress. Breath sound is absent in the ipsilateral chest.
Cyanosis is proportionate to the dyspnea. Intestinal gurgle
may be heard over the chest. Abdomen remains scaphoid.
o In mild to moderate cases, symptoms appear weeks, months,
or even years later. The later is the symptom, the better is
the prognosis.
INVESTIGATIONS
o confirmed by trying to pass a firm catheter (e.g., rubber
X-ray chest: To see bowel loops in the chest. Usually only a
small portion of lung is visible in the upper portion of chest
with displacement of the heart to the opposite side.
r Increasing use of prenatal ultrasound has led to the dis-
covery of diaphragmatic hernia in fetus. Polyhydramnios is
found in 70-75o/o cases.
o Estimation of POr, PCO2, HCO. is important.
DI FFERENTIAT DIAGNOSIS
Congenital cysdc disease of the lung, staphylococcal pneumonia
with pneumatocele.
TREATMENT
In severe case of respiratory distress with marked cyanosis,
t ECMO (extracorporeal membrane oxygenation) can save
I life and surgical correction can be done later.
l a Immediate nasogastric suction to empty the stomach.
L a Oxygen inhalation. if cyanosis.
I a Patient should be prepared for immediate surgical correction.
Basic principle of surgery includes reposition of the herniated
viscera with closure of the gap in the diaphragm.
t
I
t
F
I with end-to-end repair of
I
Tiacheoesophageal fistula occurs in about I in 3000-4500
live births. The trachea and esophagus are formed from the
v
t primitive foregut around the fourth week of intrauterine life.
I The foregut tube develops lateral indentations forming ridges,
iI
I
lb"
COMMON SURGICAL PROBLEMS
which deepens and fuses to form two separate tubes. Any
abnormality in this process causes this anomaly.
Types:
o Esophageal atresia with distal segment tracheoesophageal
fistula (87olo).
o Esophageal atresia without fistula (8%).
r Esophageal atresia with both proximal and distal segment
tracheoesophageal fistula (4%).
r Esophageal atresia with proximal segment tracheoesophageal
fistula (<1%).
o Tracheoesophageal fistula without atresia <1%o (H-fistula).
CIINICAT FEATURES
of esophageal atresia is regurgitation
Earliest clinical sign
of saliva-continuous saliva outpouring through angle of
the mouth.
The first feeding is followed by choking, coughing, and
regurgitation. Aspiration may occur resulting in pneumonia
or collapse.
Respiratory distress is progressive and more severe in those
infants with a distal tracheoesophageal fistula.
o If esophageal atresia is suspected, the diagnosis can be
catheter) through the mouth into the esophagus.
INVESTIGATIONS
r Plain X-ray abdomen and chest in erect posture with a Ryle
tube having radio-opaque bid at the tip in the esophagus.
The tube is coiled in the esophageal pouch, and abdomen
contains gas if a communicating fistula is present.
o Gastrograffin/water soluble dye x'ray through the Ryle
tube will delineate the fistula and the esophageai pouch.
TREATMENT
The approach differs according to risk groups:
Group A: Birth rn'eight 2.5 kg or more and general condition
is reasonably well. Immediate operative repair is indicated. The
line of treatment is:
r The neonate is kept in incubator with humid oxygen.
r Repeated or continued gentle suction of upper esophageal
pouch to prevent aspiration.
o IV fuid and electrolyte therapy.
o Antibiotic when needed.
o Corrective surgery as early as possible.
Transthoracic extrapleural tracheoesophageal disconnection
esophagus is preferable. Gastrostomy
may be needed.
Group B: Birth weight is 1.8-2.5 kg with moderate pneumo-
nia or other anomalies. Delayed primary repair is indicated.
ESSENCE OF PEDIATRICS
Group C: Birth weight is <1.8 kg with severe pneumonia or
other anomalies. Staged repair is advised.
Group B and group C are high-risk groups with poor
prognosis. Their line of rreatment includes:
a Kept in incubator with humid oxygen.
a Intensive antibiotic therapy.
O Constant upper pouch sucrion.
a Intravenous fluid and electrolyte therapy.
a Gastrostomy.
a If possible, primary repair or cervical esophagostomy
Gastroesophageal reflux (GER) is regular reflux of the gastric
contents into the esophagus usually after a major meal. 24-hour
pH monitoring has showed that acid gastric content is regurgitated
into the esophagus in normal people. Reflw that produces no
s)tnptoms can be considered as physiological refltx. 50-650/o
of children with GERD will undergo sponraneous symprom
resolution by 2 years of life. Pathologic re{lx produces symptoms.
ETIOTOGY
After 6 months of age, all chronic GER should be considered
pathologic and its etiology designated as failure or absence of
mechanical factor or lack of balance berween intra-esophageal
pressure (closing pressure or seal pressure) and pathologic
intragastric pressure (opening pressure).
Pathologic increase in intragastric pressure is due to
following:
r Gastric rerenrion leading to gasrric dilatation as in pyloric
stenosis.
o Increased intra-abdominal pressure as in tumors, ascites.
o Altered intrinsic gastric muscle tone from neurologic con-
dition.
CTINICAL FEATURES
o Related to vomiting: Intractable vomiting, nutritional
failure, neurological impairment.
o Related to esophagitis: Esophageal pain, bleeding anemia,
stricture.
o Related to aspiration: Apnea, croup-cough-choking, recur-
rent pneumonia.
NORMAT ANTI.REFIUX MECHANISM
i'inch-cock action of right crus of diaphragm through which
the esophagus passes.
Length of intra-abdominal esophagus (3-7 cm) under
relatively high intra-abdominal pressure comparing to
intrathoracic pressure.
Angle of His is an acute angulation of esophagus just prior
to joining stomach, and it acts as a barrier against reflux.
o Mucosal roserre forrned by convoluted folds of mucosa
gastroesophageal junction acts as weak anti-reflux valve.
INVESTIGATIONS
o Barium esophagogram: Reveals reflux of barium in the
esophagus with dilated esophagus.
Esophageal pH monitoring: pH dropped to 4 or below
in the distal esophagus is significant.
a Esophageal manometry to detect the closing pressure.
a Endoscopy with possible biopsy from lower esophagus to
derecr grade of inf ammarion.
coMPUCAT|ONS
Esophagitis, esophageal stricrure, Barrett esophagus.
TREATMENT
o Medical: Once instituted, this regimen should be continued
for at least 6 months.
o Chiid should be fed in seated or in 60-70" semi-upright
position.
o Thick frequent small feeds.
,r Domperidone 0.2-0.4 mg/kg/dose, 3-4 times daily may
be added. Occasional sedation gives good result.
o Antacids or H, antagonist may be of help.
r Surgical: \X/hen adequate medical rrearment failed to control
the
the reflux and cannot ensure adequate weight gain, surgery
should be considered. The major objectives of operarive
procedure are to increase the high pressure zone in the lower
esophagus, to accentuare the angle of His, and to increase
the length of the abdominal esophagus.
In this condition, the muscie of the pyloric sphincter hyper-
trophies, causing the pylorus to become thick and long. The
hypertrophied muscle impinges on the lumen of pyloric canal
causing gastric ourler obstruction.
More common in maies than females with a ratio of
approximately 2:1, first born child is affected more frequently.
The triad of IHPS includes visible peristalsis, palpable pyloric
tumor. and projecrile vomiting.
CLINICAT FEATURES
o Rarely present at birth. Symptoms start usually around
third week.
o Non-bilious projectile vomiting after each feed. Contents of
vomitus may be curded. Blood may be presenr late! indicating
gastritis. The baby is typically hungry after vomiring and
tI
demands another feed oniy to bring ir out as before.
I

t COMMON SURGICAL PROBLEMS

Visible peristalsis is seen passing from left to right, com-
monly after a feed. This reveals distended stomach, which
is easily visible.
Dehydration, under nutrition, and constipation are common.
Prolonged jaundice may be seen.
A small firm grape-sized movable mass may be felt below the
right costal margin, under the lateral margin of the right rectus
muscle and is best felt from the left side with the left hand.
CLINICAL FEATURES
The cardinal signs of biliary atresia are jaundice, clay-colored
stool, and hepatomegaly.
Jaundice is usually progressive for certain period with
more of conjugated type. Stool is acholic with highly
colored urine. Hepatomegaly (firm liver) develops first,
then splenomegaiy.

INVESTIGATIONS DIAGNOSIS

USG: Pyloric muscle thickness >4 mm, diameter of pylorus
>11 mm, and pyloric channel length >17 mm are diag-
nostic.
Gastrograffin or thin barium meal x-ray to show dilated
stomach and rat-tail pylorus (string sign).
Estimation of serum electrolJte is important as electrolyte
imbalance is frequently present. Hypochloremic, hypokalemic
alkalosis are the usual findings.
o USG of hepatobiliary system: To locate and delineate the
common bile duct with its branch is almost diagnostic.
Intrahepatic biliary channel may be involved.
o HIDA scan will show that the radiotracer is taken up by
the hepatocytes but is not excreted into the intestine.
o Duodenal fluid aspiratiell-xf56p6e of bile'
o Liver biopsy will show the changes of biliary atresia.

DIFFERENTIAT DIAGNOSIS
DIFFERENTIAT DIAGNOSIS
Idiopathic neonatal hepatitis (Thble 23.1), STORCH infection,
Gastroesophageal refltx, pylorospasm, congenital adrenal hyper- hypothyroidism, biliary hypoplasia.
plasia, antral web.

Table 23.1: Differentiation of ldiopathic Neonatal Hepatitis

TREATMENT from Biliary Atresia
o Nothing per oral. Nasogastric tube decompression and
saline lavage.
o Dehydration should be corrected by normal saline. Once Cause ldentified (i nfection, Not identified
metabolic, {amilial)
a good urine output is achieved, potassium can be added
Ketauon wrtn Preterm, IUCR Full term
to the fluid.
o Surgery as early as possible after correction of deficit. Organ involved Hepatosplenomegaly Hepatomegaly

Ramsted pyloromyotomy of hypertrophied muscle is the Abnormal size Less common Usual
t
\ surgery of choice. Stool lncomplete cholestasis Complete cholestasis
o Oral iluid and feeding can be started after anesthetic recovery (stool with some color) (acholic white stool)

of the infant, usually after 4-6 hours. The hepatic ducts, Not atretic
the common bile May be atretic
duct and the gall
PROGNOSIS bladder
HIDA scan Uptake sluggish. Hepatocyte function
t
After surgery, mortality is rare. Complete recovery is the rule. Fxcretion into the is intact, uptake of the
biliary tract and agent is unimpaired,
intestine eventually excretion into the
i occurs. intestine is absent.

t. HIDA scan after Enhances biliary Not improved

L A whole or a part of the extrahepatic bile ducts is absolutely phenobarbitone excreiion of the isotope
atretic in biliary atresia (though the affection is variable and Liver biopsy Severe diffuse Bile ductular
t completely obstructs bile flow). Hepatic ducts, the common hepatocel lular disease, proliferation, the
distortion of lobular presence o{ bile plugs,
bile duct along with the gall biadder is atretic. The incidence portal or perilobular
architecture, marked
of biliary atresia is about I in 10,000 live births. infiltration with edema and fibrosis, the
inflammatory cells, basic hepatic lobular
Classification: Three rypes: focal hepatocellular architecture is intact
1. Atresia at porta hepatis-88%. necrosis. the bile
ductules show little
2. Atresia at common bile duct-10o/0. alteration.
3. Atresia at common hepatic duct-2o/o.
 ESSENCE OF PEDIATRICS

TREATMENT
Surgical trearmenr is the treatment of choice for the establish-
ment of conrinuity of biliary sysrem with intestine. Kasai por-
toenterostomy is done, i.e., a loop of intestine is anastomosed
to the porra to replace the absent hepatic and bile ducts.
The operation should be done as early as possible within
60 days of age for a favorable outcome.
In failed Kasai procedure or in a late case with established
cirrhosis, living related liver transplantation is the answer.
\(ithout surgical correcrion, most patients die in the first
2 year of life because of complications.
Congenital cystic dilatation of biliary sysrem is known as cho-
ledochal cyst. It usually involves rhe common bile duct, but
it may involve intrahepatic biliary channels when it is usualiy
multiple in nature.
ETIOTOGY
Actual cause is not known, but probably gradual destruction of
the epithelial lining of the bile duct either from a viral infection
or reflux of the pancreatic juice from abnormal pancreatobiliary
ductal junction anomalies are likely causes.
CtINICAI FEATURES
Two distinct clinical parrerns:
1. Infantile form: Age from 1 to 3 months. Present with
obstructive jaundice, acholic stools, and hepatomegaly
with a clinical picture indistinguishable from that of
biliary arresia.
2. Adult form: Clinical manifestations
do not generaily
become evident until after the patient is 2 years
age. In this group, the classic triad of abdominai
pain, palpable abdominal mass, and jaundice may be
present.
DIAGNOSIS
TREATMENT
The treatment is the total surgical excision of the cysts with
a Roux-en-Y reconsrrucrion with jejunal loop and remaining
bile duct.
Cystojejunostomy is condemned as there is late incidence
of cholangiocarcinoma from cyst epithelium.
Megacolon refers to an enlargement of the colon that may be
functional, organic, or truly congenital in origin. Tiue congeni-
tal megacolon is a congenital anomaly characterized by partial
to complete colonic obstruction associated with the absence
of intramural ganglion cells in the distal alimentary rract. This
disorder is known as "congenital aganglionosis", "aganglionic
megacolon', or Hirschsprung disease (HPD).
The fundamental lesion in HPD is the absence of intramu-
ral ganglion cells in the distal intestine. This absence involves
both the submucosal and intermuscular nerve plexuses and
is associated with an increase i:i the size and prominence of
the nerve fibers. The disease starts below from the anorectal
junction for variable length upwards.
The incidence of HPD is around 1 in every 5000 live births.
Eighty percenr parienrs with Hirschsprung disease are boys.
ETIOLOGY
Enteric ganglion cells mature from neuroblasts. HPD results
from arrested caudal migration of neuroblasts in the alimen-
tary tract.
CLASSIFICATION
Short segment or classical (7lo/o)t Involves the anus,
rectum, and part of sigmoid colon.
of
Ultra short segment: Involves the anus and the part of
rectum below the pelvic floor.
Long segment: Aganglionosis up to colon proximal to
sigmoid colon.
Total colonic aganglionosis: Ganglion cell absent through-
our rhe whole large gur.

o Abdominal ultrasound can easily detect the biliary channel

dilatation, extrahepatic or intrahepatic or both. CT gives the
CtINICAI
anatomical details. HIDA scan is also diagnostic.
o Endoscopic retrograde cholangiopancreatography (ERCP)
can demonstrare an anomalous choledochopancreatic ducr
junction.
r Liver function test: Conjugated hyperbilirubinemia.
DIFFERENTIAT DIAGNOSIS
Carol i disease, hepatic fibrosis, biliary atresia, neonaral hepatitis.
Eil
FEATURES
Delayed passage of meconium: Majoriry of neonares defecate
within 12 hours of birth and certainly within 24 hows. lf a
neonare does not pass meconium within 48 hours of birth,
Hirschsprung disease should be suspected strongly.
Constipation, gradual distension of abdomen (due to col-
lection of feces and flatus), and vomiting.
There may be occasional attacks of severe diarrhea (entero-
colitis). If the patient becomes toxic, necrotizing enterocolitis \
I
must be suspected and treated promptly. \
It

o There is anorexia, anemia, and undernutrition.
a Per rectal digital examination shows empty rectum and
gripping of the examining finger with evacuation of gush
of flatus and stool during withdrawal of examining finger.
INVESTIGATIONS
o Barium enema in unaided gut: Thin barium enema film
shows a conical transition zone (i.e., a cone-shaped colon
at the junction of the ganglionic and aganglionic segments)
L from distal non-dilated colon or rectum to proximal dilated
colon. In neonates, a delayed film taken after 24 hours
is important; presence of retained barium in that film is
suggestive.
Rectal biopsy: Suction rectal biopsy or conventional full
thickness rectal biopsy reveals absence of ganglion cells with
thickened nerve fibers.
Plain x-ray of abdomen shows features of intestinal obstruc-
tion. Large dilated colon loops are seen. In newborns' the
pelvic region is frequently gasless due to the lower colon
being empry.
Anorectal manometry.
DIFFERENTIAT DIAGNOSIS
Meconium ileus or meconium plug syndrome, small left colon
syndrome, hypothyroidism, sepsis, functional constipation.
TREATMENT
Once proved, the treatment is surgical. The principle of
surgery is the removal of the aganglionic segment of colon,
and ensuring that normal ganglionic bowel is brought down
to the anus.
o Acute enterocolitis requires broad-spectrum antibiotic, fluid
and electrolyte replacement, and if does not imProve or
deteriorates, relief of obstruction by an emergency colostomy
with leveling biopsies.
r Definitive surgical treatment of HPD involves bringing
normal bowel as low as in the rectum as is technically pos-
sible by resecting or bypassing the aganglionic bowel.
Preliminary decompression by laparotomy, leveling biopsy,
and an appropriately placed colostomy is the "1sr stage" of
surgical treatment.
"2nd stage" on definitive surgical procedure is deferred until
the infant is approximately 1 year of age or until the older
; patient is returned to good nutritional status.
I
"jrd stage": The definitive procedure is followed in 3-4 weeks
I by colostomy closure.
Recently single stage procedure with primary definitive
It operation without colostomy is practiced in selected group of
I patients with good results.
r
I
l|
COMMON SURGICAL PROBLEMS
Atresias are congenital blocks in the intestine due to a complete
or partial occlusion of the lumen.
The incidence of duodenal atresia is more frequent than
ileal and jejunal atresias. Colonic atresias are the least common.
Duodenal atresia is especially common in babies with Down
syndrome, and there is a high association with heart anoma-
lies.
With the increasing use of routine antenatal ultrasound,
the atresia may be picked up earl;., and the classical symp-
toms and signs may not be seen. The classic presentation
is that of bilious vomiting within the first hour of life in
an otherwise stable neonate, abdominal distension may or
may not be present.
A plain x-ray of the abdomen is usually diagnostic. In the
case of duodenal atresia, the stomach and first part of the
duodenum are the only parts of the intestine filled with gas,
thus exhibiting the "Double Bubble" sign. Contrast x-ray
of upper GIT with water-soluble dye is often diagnostic in
confusing cases.
Lower atresias show more bowel loops filled with gas, with
the appearance of air fluid level, and a gasless lower abdomen,
representing the distal unfilled intestine.
TREATMENT
o Surgery should be done as soon as possible to restore the
intestinal lumen and aliow early feeding.
o After the abdomen is entered, the atresia is identified, and a
resection of the atretic part of the intestine followed by anas-
tomosis of the proximal and distal ends is carried out'
The anorectal malformations are a group of anomalies that
affect the perineum in either the male or the female. A common
defect in all the anomalies is that the anus does not open nor-
mally at the usual site. The opening may be severely stenotic
or may be entirely absent, in which case the rectum or anus
end as a blind pouch. A blind ending rectum frequently has
a fistulous communication to the urinary tract, which lies just
anterior to the rectum. A further variant of the malformation
is the anal opening being present anterior to the normal site,
especialiy in the female, where it may open in the vestibule
or in the vagina.
crAssrFrcATroN
The basic classification centers on the main muscle of conti-
nence, namely the levator ani, which is present as a sling in
the pelvis through which the rectum passes. On contraction
of the levator, the flow of feces is stopped. This is aided by
the external sphincter.

-
ESSENCE OF PEDIATRICS
The shape of the levator sling is somewhat like that of a
funnel. If the rectum is blind ending above the levator funnel,
it is termed a "high" variety of malformation. Here, if a fistula
is present, it opens in the urinary bladder or the vagina. If
the recrum passes rhrough the levator funnel entirely, this is
termed a "low" variery of malformation. Fistulae through the
anorectal pouches open out on the skin either at the site of
the anus or anteriorly on the perineal or scroral raphe in the
male or in the vestibule or at the vulva in the female. Rectum
may end blindly without any fistula.
In the intermediate variery the rectum enters the levator
funnel but does not pass through it entirely. There are frequently
fistulae to the posterior urethra in the male, or the vestibule
in the female.
INVESTIGATIONS
An invertogram is performed. This is an x-ray of the baby
taken when the baby is held upside down, suspended by the
legs. The air that passes down to the blind rectum will rise up
in this position and show the level of the rectal pouch.
More recently, x,ray cross table lateral view is performed to
avoid some of the hazard of invertogram. Here, rhe neonare
is placed on table in prone position with hip slightly raised,
and x-ray is taken from lateral side.
TREATMENT
If the anomaly is a low anomaly, a surgical exploration through
the perineum is performed and the new anus is created by
suturing the pouch ro rhe skin after opening it (anoplasty).
If the anomaly is intermediate or high, a colosromy musr
be made to relieve the intestinal obstruction. At a later stage,
a formal operation can be performed. This rectum is then
brought down through the levator muscles to try and preserve
as much of the continence mechanism as possible (pSARp).
At the third stage, the colostomy can be closed.
Nowadays single stage PSARP without colostomy is being
practiced in many cenrers with good results.
In both exomphalos and gastroschisis, there is herniation of the
contents of the abdominal cavity through a defect in the anterior
abdominal wall. In exomphalos, the umbilicus itself is widely
open aliowing herniation of the abdominal viscera, whereas
in gastroschisis there is a full thickness defect in the anterior
abdominal wall next to a normally formed umbilicus.
In exomphalos, the herniation may be of variable size. In
some cases, only a few loops of small bowel are presenr within a
sac in the umbilical cord (exomphalos minor). In more severe
cases, there may be a large gaping defect in the umbilical region,
I
with almost the entire intra-abdominai viscera including most of
the intestine, stomach, and liver presenr in the sac (exomphalos
major). The sac irself is formed by a layer of Wharton jelly
lined by peritoneum inside and amniotic membrane outside,
and appears at birth to be a thick translucenr membrane, which
after some hours becomes white and opaque.
DIAGNOSIS
The diagnosis of both conditions does not require any special
investigation as they are obvious clinically. Antenatal ultrasound
can diagnose both conditions in urero.
TREATMENT
The aim in both conditions is ro secure closure of the
abdominal wall after reduction of the contents within the
abdominal cavity. This is usually simple in exomphalos
minor, but in exomphalos major, there may be dilficulty
in reducing the conrents of the sac in the abdominal
cavity, since the abdominal cavity may not have the
capaciry to include the herniated contents. In this situ-
ation, conservarive therapy may have to be used. The
skin from the abdominal wall eventually grows over rhe
sac forming a large ventral hernia, which will need to
be closed at a later stage.
There is however a high incidence of infection in this case,
causing morbidity and mortality.
Intussusception refers to the invagination of one part of the
intestine into another. It is a well-recognized event in pediatric
patients, especially around B*10 months of age.
The most common site of intussusceprion is the ileocolic
region, just proximal to the ileocecal valve. In a few cases,
there is a definite lesion in the intestine, which is recognized
as the cause ofthe lead point ofthe intussusception, such as
a polyp. In >95o/o ofcases, it is thought that a hypertrophied
Peyer patch may cause a loop of intesrine ro invaginate within
the distal loop during the propagation of a peristaltic wave.
The hypertrophy in the Peyer patch is thought to be due
to a change in the bacrerial flora that accompanies weaning
from breast feeds and introduction of other feeds, which ii
a feature in babies of this age group. In other instances, it
is postulated that an upper respiratory tract infection may
cause a generalized lymphoid hyperplasia, leading to peyer
patch hypertrophy.
The ileum, being the mosr commonly involved, invaginates
into the cecum and through it into the colon carrying with
it the lead point at the apex. The part of the intestine that
lies within is cailed the intussusceprum, while the part that
lies externally rhat receives the proximal intestine within it is
called the intussuscipiens.
t
I
't
I
I

As the mass advances, there is venous congestion within the
innermost layer, causing the secretion of bloody mucus, which
is characteristically passed as stool. Finally, there is further
vascular compromise especially at the apex, and in cases that
are not reduced early enough, gangrene may set in.
CLINICAT FEATURES
Intussusception classically affects a healthy, well-nourished
child <l year of age. Males are afFected often more than
females. There may be a history of an upper respiratory
tract infection just prior to the attack.
The main complaints are vomiting, passage of blood in
the stool, and abnormal crying due to abdominal pain. The
pain is colicky, and occurs in short bursts of severe pain
causing an otherwise well child to shriek with pain and
draw the legs up towards the abdomen. As soon as the colic
settles, the baby quiets down. The relief is temporary, since
the attack repeats itself.
After a few episodes of such attacks, the child vomits and
passes a blood stained mucoid stool per rectum, traditionally
known as the red currant jelly stool. Significantly, there is
no fecal matter mixed with this stool, a differentiating factor
from dysentery.
On palpation of the abdomen, a typical banana-shaped
mass representing the intussusception can be felt in the
abdomen or per rectum; the concavity of the mass being
oriented towards the umbilicus.
In cases that are diagnosed late, there are general signs of
septicemia. The abdomen may be distended and may show
evidence of peritonitis.
INVESTIGATIONS
a Ultrasound of the abdomen can diagnose intussusception.
a A plain x-ray is non-specific, but in
cases where the diag-
nosis has been delayed, there will be signs of intestinal
obstruction.
Barium enema is diagnostic (demonstrates a coiled - spring
appearance to the bowel) as well as therapeutic in the early
stages of intussusception.
TREATMENT
1. \X4ren intussusception is diagnosed early (i.e., within 24
hours of the onset of the attack) and when frank signs
of intestinal obstruction have not set in, reduction of the
intussusception using barium, or saline instilled through
t the rectum may be successful. It must be stressed that this
procedure should be considered as significant as surgery is,
and should only be done by a surgeon with facilities for
operation being kept ready in case of failure. Peritoneal
I signs or the presence ofperitoneal free air are an absolute
contraindication to this procedure.
t
COMMON SURGICAL PROBLEMS
On instilling the barium, the intussusception is seen
as the barium oudines the rounded head of the leading
end known as the coiled spring sign or the claw sign.
The pressure of the column of barium may be able
to push back the invaginated mass of intestine till it
completely reduces. If barium is ultimately seen freely
filling the coils of small intestine, the reduction has been
complete and successful. There is usually passage of fatus
and, stools soon after, the mass felt earlier disappears and
there are no more episodes of colic. To further prove
the completeness of reduction, some charcoal powder
is usually given to the child through the stomach tube.
Recovery of the charcoal in the stool the next day is
proof that the patency of the intestine has been restored.
2. If reduction of the intussusception is not successful by
barium, then operative reduction should be performed.
At surgery, if there is no evidence of bowel gangrene,
manual reduction is done. If there is gangrene, then
resection of the entire intussuscepted mass, with
anastomosis of the proximal and distal intestine must
be performed.
Juvenile polyps of the rectum are common in children. The
history is of passing a few drops of blood after the passage of
a stool. Unlike a fissure, the blood is distinct from the stool
and not smeared on it. Here per rectal bleeding is painless.
TREATMENT
Pol;.pectomy under general anesthesia is the treatment of choice.
If the polyp is readily felt on rectal examination, it should
be removed per rectum using routine instruments (if it can be
approached from below). For polyps that are slighdy higher
up, endoscopic removal using wire snares and electrocautery
is effective.
Obstruction to the flow of urine promotes urinary stasis,
which leads to infection. Common sites where obstruction is
encountered are:
The pelviureteric junction: This causes dilatation of the
pelvis and calyces of the kidneys causing hydronephrosis.
The vesicoureteric junction: This causes ureteric dilata-
tion, which if severe can also affect the kidneys and result
in hydroureter and hydronephrosis.
The posterior urethra: Posterior urethral valves are curtain-
like membranes in the posterior urethra that cause an
obstruction to the flow of urine from the bladder.
ESSENCE OF PEDIATRICS
PETVIU RETERIC JUNCTION OBSTRUCTION
The obstruction is a result of a congenital srenosis at the pel-
viureteric junction (PUJ), or due to external compression of
the PUJ most commonly due to an abnormal artery to the
Iower pole of the kidney that causes a kink in the ureter at
its origin.
The urine formed in the kidney thus cannot be transported
into the ureter and collects in the pelvis. The pelvis dilates to
accommodate the increasing amount of urine being formed.
The pressure in the pelvis rises and is transmitted to the
calyces, which also distend. The increasing size of the pelvis
and calyces causes pressure upon the kidney parenchyma, which
is compressed and is eventually thinned out. The function of
the kidney begins to get affected as the parenchyma becomes
thinner. In severe cases, the kidney may be represented by
a thin membranous sac containing urine. If this urine gets
infected, pyonephrosis (collection of pus or infected urine in
a dilated pelvicalyceal system) results.
Clinicol Feqlures
Common presenting symptoms are UTI, pain in abdomen, and
lesscommonly, hematuria. Sometimes, a lump in the abdomen
is noted by the parents or the examining physician.
Gastrointestinal symptoms like nausea, vomiting, and
anorexia may be present, and at other times the symptoms are
non-specific such as failure to thrive, weakness, and lethargy.
Hypertension in children is an indication to ruleout kidney
disorders.
The term "Dietel crisis" is used to describe a rypical feature
of hydronephrosis. There is an abdominal lump with pain that
may be severe and that is suddenly relieved and accompanied
by diuresis. This happens when the pressure in the renal pelvis
builds up to a great degree and is able to overcome a srenosis at
the PUJ. The collected urine is passed down with symptomatic
relief, A lump in the abdomen due to hydronephrosis is noted
to have disappeared.
lnvesligolions
1. A hemogram and tests for renal function are necessary.
2. A urine routine test and urine culture, if infection is
suspected.
3. Imaging: The test of first choice is an ultrasonogram, which
will confirm the diagnosis. An intravenous urogram will
show dilatation of the calyces and the pelvis, and non-
visualization of the ureter on the affected side. In more
seyere cases, where the renal parenchyma is compressed
towards the periphery of the hydronephrosis, the contrast
is seen as thin crescents around a non-opacifiring central
mass. This is called the crescent sign. There may be delayed
uptake of the contrast, and in severe cases, there may be
no uptake seen on the affected side. A micturating cys-
tourethrogram is useful to see if concomitant vesicoureteric
reflux is present.
4. Radionuclide scanning: A scan using DTPA with furo-
semide is extremely useful in quantifting the degree of
obstruction, and the function of the affected kidnev.
Treolmenl
On the basis of the renal scan, we now know that some cases
of hydronephrosis still have excellent kidney function, and will
continue to retain this excellent function in spite of the hydro-
nephrosis. Some cases of hydronephrosis resolve spontaneously
over a few years. Thus, ifkidney function on rhe affected side
is >40o/o, and if there is no complication, or symptoms, rhe
child can be observed. In case of function <40o/o, or if there
are compiications like recurrenr infection, or symptoms like
pain, or if the renal function is seen to be deteriorating on
observation, then pyeloplasty is performed.
Pyeloplasty means reconstructing the pelviureteric junction
to eliminate the obstruction and allow easy passage of urine
through it.
POSTERIOR URETHRAL VALVES
Posterior urethral valves are thin curtain-like membranes that
are seen in the posterior urethra of male child. These valves
cause a partial obstruction to the flow of urine, resulting in
high pressures being generated in the urinary bladder, as it
attempts to overcome the obstruction.
Diognosis
The diagnosis is usually at birth due to antenatal ultrasound,
which shows bilateral hydroureteronephrosis. After birth, con-
firmation of the diagnosis is done by repeating ultrasound and
performing MCU.
Treolmenl
Ifposterior urethral valves are present, they need to be burnt
(cystoscopic fulguration) to restore the caliber ofthe bladder
outflow. However, frequently, the kidneys have already suf-
fered significant damage and the prognosis for renai function
is not good. These children in spite of having had a good
urine flow restored do not grow well and may suffer renal
failure in childhood or adolescence. Kidney transplant is
the only alternative in this group.
Other children who are fortunare to have a lesser degree of
obstruction may present in later childhood due to infection
or voiding difficulty. Here the kidney function is relatively
better preserved and the long-term prognosis should be
better.
Hernia and hydrocele result from a patent processus vaginalis,
which is a tongue like extension of the peritoneum rhar develops
along the inguinal canal in utero.
\

In most children, the processus closes down or fuses, and
there is no connection between the peritoneum and the
inguinoscrotal region. However in some children, the processus
remains patent and can allow contents from the abdominal
cavity to enter the scrotum.
If the opening of the processus is large, the abdominal
viscera like intestines can herniate into the scrotum forming a
t hernia. If the opening is very small, only peritoneal fluid can
t enter, forming a hydrocele.
I,
CtINICAI FEATURES
The main complaint is swelling in groin or scrotum. In case
of hernia, there is a history of increase in the size of swelling
on crying or after activity. In hydrocele, there is increase in
the size of swelling in the evening after an active day, and a
slight decrease on waking up in the morning.
On examination, the hernial swelling is reducible, whereas
the hydrocele is irreducible. This is because a hydrocele, though
in communication with the peritoneum, has a very small orifice,
and on application of pressure on it, the inverted ink bottle
effect is produced, causing irreducibility. After a nightt rest,
however, there is a decrease in size due to slow spontaneous
reduction.
COURSE AND COMPLICATIONS
A hydrocele has a very high chance ofresolution by the age of
18 months, since the patent processus will often close sPontane-
ously. There is essentially no complication that a hydrocele can
undergo, hence in these cases surgery is not performed till the
age of 18 months. After this age, there is a very small chance
of spontaneous cure; hence, an operation should be done.
TREATMENT
Herniotomy (for hydrocele persisting after 18 months):
The communication with the peritoneum is exposed and
ligated. This operation is essentially the same as that done
for a hernia.
For an inguinal hernia, surgery should be advised as soon as
the hernia is diagnosed. This is because a hernia has good
chance of complication in the form of irreducibiliry obstruc-
tion and strangulation of its contents. In fact, premature
babies and infants have a higher chance of complications;
hence, one should not wait till the baby has grown to
advise surgery.
If the hernia does become irreducible, the child is kept under
observation and sedated. Reduction is tried under sedation
and after keeping the child in the head low position for
a few hours. In most cases, the hernia can be reduced, in
which case surgery should be planned within a few days. If
J the reduction is not successful, an emergency surgery should
N
be performed. The chances of complication in emergency
I
COMMON SURGICAL PROBLEMS
hernia surgery are much more than during routine surgery,
hence, as far as possible, hernias should be treated on an
elective basis as soon as diagnosed.
Undescended testis is best defined as a testis that cannot be
manipulated to the bottom of the scrotum without undue
tension on the spermatic cord.
During the third trimester, the undifferent gonads begin to
descend through the inguinal canal to reach the scrotum by
birth. After the testis descends, the patent processus vaginalis
usually closes.
Descent ofthe testes is essential for adequate germ cell devel-
opment, since the scrotum maintains the testes at a temperature
a few degrees lower than the body core temperature.
The causes ofnon-descent ofthe testes have been postulated
to be genetic, hormonal, or mechanical. The exact cause is not
always known.
CtINICAt FEATURES
The scrotum on the side of non-descent will be seen to be
empty and not as well-developed as the contralateral normal
side. The testes may be palpable in the inguinal region.
A true undescended testis lies in the abdomen along the
line of testicular descent or in the inguinal region or in the
groin outside the external ring. These varieties are called,
respectively, the abdominal testis, the inguinal testis, and
the emergent testis. Clinically, they may also be broadly
classified into the palpable and the impalpable testes.
An ectopic testis is one that has successfully descended, but
has not found its way into the scrotum after emerging from
the external inguinal ring.
A retractile testis is one where the normal cremasteric reflex
is hyperactive. The testis gets pulled up due to the action of
the cremaster and ties above the scrotum. In such a case, the
scrotum is well-formed. On examining the child in the cross
legged squatting position, the testis can be seen to descend
in the scrotum, and following manipulation the testis can
be brought down into the scrotum and be maintained in
that position.
coMPHCAT|ONS
Al undescended testis can undergo complications:
Fertility problems: Poor germ cell maturation if the testes
are not brought down by the age of I year. This affects
fertility.
Trauma: In the inguinal region, the testis can get damaged
due to blunt trauma.
Torsion: The undescended testis is prone to torsion and
can present as an acute abdomen or as an enlarged tender
mass in the groin.
ESSENCE OF PEDIATRICS
o Tumor: Undescended testes are prone to malignant change.
This propensiry may be decreased by bringing the testis
down to the scrotum by the age of I year.
TREATMENT
An undescended testis at birth has an excellent chance of
spontaneous descent up to the age of 3 months. It has also
been noted that if a testis remains undescended beyond the
age of 1 year, irreversible changes develop in its structure,
that are damaging to the capaciry of the testis to form mature
germ cells. For this reason, surgery should be performed
around I year of age, if the testis has not yet descended.
In some cases, giving hormones llke hCG has proved successfirl
in bringing down an undescended testis, since it has been felt
that a poor secretion of hormones is responsible for nondescent.
Howeve! the use of these hormones is controversial and not
everybody is convinced that they are really useful.
Phimosis is the inability to retract the prepuce fully and freely
to expose the glans penis. It is one of the most common prob-
Iems referred to the pediatric surgeon.
Phimosis is physiological in infancy and early childhood,
since there are adhesions between the glans and the foreskin
that separate spontaneously by the age of 2-3 years, sometimes
taking up to 5 years or so.
Phimosis is pathological when it causes symptoms such
as straining at micturition, burning micturition, or recurrent
attacks of balanoposthitis.
Paraphimosis implies that the foreskin that has been retracted
back to expose the glans and the coronal sulcus cannot be
repositioned to cover the glans. The foreskin gets entrapped
behind the coronal sulcus due to a small preputial opening.
TREATMENT
Physiological phimosis should be left alone. Unless there are
clear symptoms associated with phimosis, no specific treatment
is required in early childhood.
If symptoms occur, a circumcision can be done to relieve
the symptoms. It was the practice in some rvestern countries
to routinely circumcise all newborn boys. This has now been
found to be unnecessary since there is no clear medical benefit
to performing this operation. In certain communities, neonatal
circumcision may be performed for religious reasons.
Behrman RE, Kliegman RM, Jenson HB. Nelson Tixtbook of
Pediatrics 18'h ed. tX/B Saunders Co, 2008.
Parthasarathy A (ed). IAP Textbook of Pediatrics 4'r' ed. New Delhi:
Jaypee Brothers, 2009.
Molla MR. Pediatric Diagnosis & Ileatment 2"d ed. Dhaka: Daisy
QNB, 2007.
4. Dworkin PH. NMS: Pediatrics 4'r' ed., Philadelphia: Lippincott
Villiams & \fikins, 2000.
Teitelbaum DH, Coran AG. Hirschsprung's disease. In: Spitz L
Coran AG, ed. Pediatric Surgerl 5't' ed. London: Chapnan and
Itall Medical. 1998.
6. GrosfeldJL, O'NeillJA, Coran AG, Fonkalsrud EY/ (ed.). Paediatric
Surgery 6'h ed. Philadelphia: Mosby, 2006.
\
I
1
I
iI
 CHAPTER
Procedures in Practice
Chopler Conlenls
tr
t"
Y
a
Blood is collected before institution of any antibiotic therapy.
A dry sterilized all-glass syringe is used, and about 5 ml venous
blood is collected with all asepric precautions. Using a separare
sterile needle, blood is introduced aseptically into a blood
culture bottle, which conrains appropriate media. Blood culrure
bottle is a screw-capped bottle of 4 oz capacity; the cap has a
hole in the center and is fitted with a rubber diaphragm. The
screw-cap is covered with viscap, and the bottle contains about
50 ml of media. Before introducing blood, viscap is ripped
off and blood is injected in through the hole in the cap. The
bottle is gently agitated and then incubated. Subcultures are
made on appropriate solid media at regular intervals, and a
negative report is given in the absence of growth after 10
days incubation (but 3 weeks for Brucella abortus). For isola-
tion of salmonellae, gentian violet-bile-salt broth is used; for
cocci, glucose broth; and for brucelia, liver infusion broths are
employed. Occasionally, infective endocarditis is caused by an
anaerobic Streptococcus or bacteroides; it is therefore advis-
able to inoculate some of the blood anaerobically in a suitable
medium, e.g., cooked-meat broth.
i Possible pathogens are Streptococcus uiridans, Salmonella,
t Stap hy lococcus aureus, Strep toco ccus
?logenes, B ruce lla, Gram-
t negative organisms.
L
I,
A catheter specimen may nor be required. A mid-stream
specimen of urine in a sterile container is to be collected from
male or female. Urine sample could be collected by suprapubic
t
I
24
Exchange transfusion.............. ...........461
Endotracheal intubation........................................ ... . 4El
Transfusion of small-volume blood for infants.............. 461
Suprapubic bladder aspiration
aspiration in infants. For a female patient, a wide-mourh con-
tainer is preferable. Anogenital region should be cleansed with
soap-water; the patient then passes urine with labia separated,
and mid-stream urine is collected. Specimens from infants
may be collected with a sterilized self-adhesive collecting bag.
Specimens are to be forwarded to the laboratory within Zz-1
hour after collection. Possible pathogens are E coli, Enterobacter,
Klebsiella, Pseudomonas, Proteus.
The specimen of feces should be collected in sterile container
free from antiseptics, and should be unmixed with urine. The
stool may also be taken by a recral swab, and then transferred ro
a sterile tube. The sample should be forwarded to the laboratory
as early as possible. If a delay of >18 hours is expected, feces
should be preserved in a buffered alkaline medium. Specimen
may also be taken by a swab, and then forwarded into a sterile
test tube. In case of cholera, the sample may either be taken
in alkaline peptone warer or in Monsur's transporr medium.
Possible pathogens are Salmoneila, Shigella, V cholerae, E.
ca#, Polio virus.
Throat swabs are taken with a properly sterilized cotron-wool-
tipped swab under direct vision using a rongue depressor
and a good source of light. Swab should be taken before any
antiseptic gargling or taking any antibiotic. The specimen in
a sterile test tube should be forwarded to the laborarory as
early as possible. If any deiay (>12 hours) is anticipated,

ESSENCE OF PEDIATRICS
serum-coated swabs (to ensure survival of Streptococcus pyogenes)
are to be used preferably.
Possible pathogens are S*eptoczccus plogenes, C. diphtheriae,
Vincentt organism, Candida species.
Pus and exudate should be collected in a sterile container or
in swabs, before application of local antiseptics. It should be
forwarded immediately. At least three swabs from the appro-
priate site should be taken. In case of deep-seated lesion with
a sinus, entire inner dressing may be sent.
Possible pathogens arc Staphylococcus pllgenet Streptococ-
cus and other pyogenic organisms, Mycobacterium tuberculosis,
anaerobic organisms.
Coughed out material should be collected; it should not be
saliva or post-nasal discharge. The material should be forwarded
to the laboratory as early as possible. Bacterial flora in the upper
respiratory tract may normally consist of pathogens. Cultural
findings should therefore be evaluated with clinical features.
Possible pathogens are Staphylococcus pyogenes, Streptococcus
pJrogenes, Streptococcus pneumoniae, H. influenzae, K. pneu-
moniae, Pseudomonas, Tubercle bacilli.
In most cases of failure to complete a procedure successfully,
the fault lies in undue haste in preparing a struggling or crying
patient. The physician should become acquainted with various
methods of restraining pediatric patients. In using total body
restraint, be certain that cardiorespiratory functions are not
impaired. After a procedure has been completed, the physician
should personally observe the child long enough to be certain
that no untoward reaction has developed.
1. Sites: For small infants, a scalp, wrist, hand, foor or arm
vein will usually be most convenient. In an emergency if
the vein can not be entered, fluids may be administered
intraperitoneally.
2. Equipments: (z) Infusion set preferably with a burette,
(ii) scalp vein set (butterfly needle 23G, 25G), (iii) an
intravenous carheter, and (iu) leukoplast.
3. Technique:
a) Insertion of tbe bunnfly.' First flush the tube with
an isotonic solution. Insert the needle under the skin,
' a
when the needle tip enters the vein, advance the needle
until blood return is noted in the tube. 'Watch care-
fully for evidence of extravasation. Secure the needle
re
.t
and wings of the butterfly in place with leukoplast.
Booster the wings of the butterfy with cotton to hold
the needle at a proper angle. Coil the butter{ly tubing
and the leukoplast away from the needle entry site.
b) Insertion of catbeter: The catheter/stylet apparatus
consists of an intravenous catheter with removable
stylet. Insert the apparatus under the skin, and advance
it until blood drips from the carherer hub. Holding
the srylet stationary, advance the catheter over rhe
stylet into the vessel. \Tithdraw the srylet and attach
intravenous tubing to the catheter hub. Secure the
catheter in place carefully. Padded board may be used
for immobilizing rhe extremiries.
4. Precautions: (4 lh. rate of flow should be checked
frequently, (ii) an accurate record must be kept of the
amount and type of fluid added, (iii) it is best to remove
the needle and change its location every 48-72 hours, and
(lz) inspect the limb at regular intervals for evidence of
undue pressure and circulatory embarrassment.
ANTECUBITAT VEIN PUNCTURE
Antecubital vein is the besr vein for the purpose of venipuncture
in children, even in neonates.
FEMORAT VENIPUNCTURE
1. The leg is abducted, and is held tightly at the lower end
of the femur.
2. The femoral artery is palpable below the inguinal ligament
in the middle of the femoral triangle; the vein runs on
its medial side. A-fter the usual anriseptic cleaning with
spirit and iodine, the position of the femoral artery is
determined by checking the pulse.
3. One butterfy needle (21G or 23G butterfly needle being
attached with a 5 cc syringe) is then introduced perpen-
dicular to the skin on rhe medial aspect of the artery below
the ligament. Slowly and gradually withdraw the needle
(until there is spontaneous blood flow in the tubing) using
sustained negative pressure by the syringe. The blood will
{low back as soon as the needle enters inro the lumen of
the vein. The blood fows in powerful jets and is bright
in color, if artery is punctured. After the maneuver, the
needle is withdrawn; sustained pressure is applied over
the site of puncture by sterile cotton for 8-10 minutes.
Gangrene of the toes or feet, infection, hematoma, or septic
arthritis, etc. are occasional complications.
t
EXTERNAL JUGUTAR VEIN PUNCTURE
External .iugular vein puncrure is safe and mosr frequently used.

Preparation: Hold the child firmly so that the arms and legs
are adequately restrained. Place the child on a fat, firm table so
that both shoulders are touching the table; the head is rotated
fully to one side and extended partly over the end ofthe table
so as to align the vein. Adequate immobilization is essential.
Technique Use a 21 G or 23G butterfly needle for withdrawing
blood. Thrust the needle under the skin and create constant
negative pressure within the syringe as the vein is entered. This
will prevent air embolism resulting from air being drawn into
the vein when the child inspires. After removing the needle,
exert firm pressure over the vein for 8-10 minutes while the
child is in sitting position.
INTERNAT JUGUTAR VEIN CANNUTATION
Internal jugular vein cannulation provides an excellent approach
ro rhe central circulation with a high success rate and minimal
complications. With left sided cannuladon, there is potential for
injury to the thoracic duct, and there is a higher risk for pneumo-
thora-r because the apex of the left lung is higher than on the right.
SUBCLAVIAN VEIN
The subclavian vein is the preferred site in patients with long-
term carheter requirements because of its relatively high level of
patient comfort and ease of catheter maintenance. In patients
with hypovolemia, the subclavian vein does not coliapse as
readily as some of the other major vessels because of its fibrous
attachments directly below the clavicle. The major compli-
cations include pneumothorax, subclavian artery puncture,
hemothorax, and risk of subclavian vein stenosis.
Indications: To obtain arterialized capillary blood for blood gas
analysis, bilirubin, glucose, hematocrit, and other biochemical
Parameters.
Technique: Pre-warming the infant's heel (cotton pledget
soaked in sterile warm water at 40"C or hot towel) is impor-
tant to obtain capillary blood gas samples, and warming also
increases the fow of blood greatly for collection of other
specimens. After ensuring asepsis, a sterile blood lancet or a
needle is punctured at the side ofthe heel. The central portion
of the heel should be avoided as it might injure the underlying
bone, which is close to the skin surface. The blood is obtained
by alternate squeezing and releasing of calf muscles.
INDICATIONS
Y Therapeutic: (l) Nasogastric feeding in premature low birth
weight baby, protein-energy malnutrition, postoperative
I
PROCEDURES IN PRACTICE
patients, very sick or unconscious patients; (ll) nasogastric medi-
carion; (iii) gastric lavage in poisoning; (iz) decompression of
abdomen in acute abdomen; and (z) umbilical catheterization.
Diagnostic Isolation ofAFB, analysis of gastric juice, diagnosis
of choanal, esophageal and anal atresia.
PROCEDURE
One should choose the largest size tube feasible, without causing
undue discomfort to the child; an 8-Fr tube in newborns, 10-Fr
for 1 -year-old , and 14-16 Fr for teenagers are appropriate. The
length of the tubing to be passed is estimated by adding 8-10
cm to the distance from the nares to the xiphoid process. One
should prepare the child by explaining the procedure as fully
as possible; sedation is rarely needed. Infants and obtunded
children require the supine position with their head turned
to the side.
The curved tube is straightened out and its patency is
checked with a syringe. Lubricant is applied to facilitate atrau-
matic nasal passage. The tube is grasped 5-6 cm from the distal
end and advanced posteriorly along the floor ofthe nose. It is
inserted with the natural curve of the tube pointing downward
in order to pass the bend the posterior pharynx makes. If the
child coughs or gags persistently or the tube emerges from the
mouth, one should temporarily discontinue the procedure.
\7hen the tube is successfully passed to the measured length,
its position is checked by attaching a 5 ml syringe filled with air
to the proximal end and, while depressing the plunger rapidly,
Iistening with a stethoscope for gurgling over the stomach. The
tube is taped securely to the nose.
Bone marrow puncture is commonly employed to examine the
marrow in anemias (e.g., aplastic or hypoplastic), leukemia,
lymphomas, ITB and to detect certain parasites, e.g., LD
bodies in kala-azar, in storage diseases.
PROCEDURE
t. For children above 2 years of age, the iliac crest (manu-
brium sterni may also be used; in the sternum, the site
is just above or below the manubrio-sternal angle) is the
preferred site for bone marrow aspirationl it is done on a
point 1 cm below the iliac crest about l-2 cm posterior
to the mid-a-xillarv line. The child may be in prone posi-
tion or on his side. The apparatus used for aspiration is
a trochar and cannula fitted with an adjustable stop and
so made that syringe fits into the top of the cannula.
2. After aseptic preparation of the skin, the areais infiltrated
with l-2o/o lignocaine up to the periosteum of sternum.
The stop ofthe trochar is unscrewed and adjusted accord-
ing to the build of the patient; the adjustment may well
ESSENCE OF PEDIATRICS
be 0.5-1 cm at first. The tip of the trochar and cannula
is introduced through the skin perpendiculariy by boring
motion; pressure should be applied from palm of the
hand. Similarly, iliac crest may also be punctured and
aspirated. Incision over skin is not necded.
3. The entry into the marrow cavity is indicated by a
sudden lack of resistance. After the cavity is penetrated,
the stilette is withdrawn and a tightly fitting syringe is
attached. Strong but brief suction yields about 0.2 ml of
bone marrow tissue and contaminating peripheral blood.
Without disconnecting the syringe, the cannula is removed
and firm pressure with sterile dry gauze is applied over
the site of puncture.
4. In children below 2 years, the upper third of the mediai
aspect of the shaft of the tibia is a good site for marrow
aspiration.
5. Fiims are prepared immediately by placing the aspirated
materiai on a glass slide, sucking off most of the blood,
and preparing a film where the particles are drawn along
by a spreader to leave trails of dislodged bone marrow
cells. Particles may also be added to fixatives for prepara-
tion oF fixed-tissue sections.
Indications include obtaining CSF for the diagnosis of men-
ingitis, meningoencephalitis, subarachnoid hemorrhage, and
other neurologic syndromes.
PROCEDURE
1. Preparation of a patient is important in order to complete
the procedure successfully.
2. An experienced assistant has a vital role in positioning,
restraining, and comforting the patient.
3. The patient should be lying on his side on a firm table
with the knees and chin as nearly approximated as possible;
his back should be right at the edge of the table and its
transverse axis, i.e., a line passing through the posterior
superior iliac spines should be vertical.
4. The skin is thoroughly prepared with a cleansing
agent.
5 The physician should wear a mask and be gowned and
gloved; the patient should be draped.
6. The neck and legs ofthe patient are fexed by an assistant
to eniarge the intervertebral spaces. The ideal interspace for
LP isL3-L4 or L4-L5, which is determined by drawing
an imaginary horizontai line from one anterior superior
spine of the ilium to the other.
7. A disposable needle of 23G for younger children and22G
or 19G for older children, l-2 inches, sharp, beveled with
a properly fitting sry1et is introduced firmly through the
skin in the midline betq'een the spines, and is pressed
steadily forward and slightly toward the head.
The stylet is removed frequently as the needle is slowly
advanced to determine whether CSF is presenr. A pop
is felt as the needle penetrares the dura and the pressure
gives way, this sensation is commonly felt and needle
enters the subarachnoid space.
c)
CSF will come out and required amounr (-0.5-3 cc
may be sufficient) should be collected in sterile test tubes
(syringe is not used for collection). If the needle seems ro
be entered far beyond the space and CSF has not drop
out, the needle should be withdrawn gradually and care-
fully.
10. For small infants, lumbar puncrure may be performed
at the level of the superior iliac crest, with the patient
in sitting position and leaning forward. CSF may Ilow
very slowly and the "give" may not be felt in small
infants. Gentle aspiration with a small syringe may be
After the needle is withdrawn, sterile dressing should
be applied. The patient should lie fat for 8-24 hours
afterwards (without pillow) and should be given drink
immediately after the maneuver.
CONTRAINDICATIONS
Elevated ICP owing to a suspected mass lesion of the brain
or spinal cord.
Symptoms and signs of pending cerebral herniation in a
child with probable meningitis.
Critical iliness (on rare occasions).
Skin infection at the site of the LP
Thrombocytopenia.
INDICATIONS
Diagnostic: Pleural effusion or empyema.
Therapeutic: \X{hen large collections of pleural f uid compro-
mise ventilatory function.
PROCEDURE
1. X-ray of the chest should be routinely done before thora-
centesis to know the exact location of effusion. Normally,
the pleural space contains <15 ml of pleural fluid. Tho-
racentesis should be done at the dependent part, which
can be determined from the area of maximum opaciry
on x-ray or of maximum dullness on percussion.
2. The procedure is usually done with the patient sitting and
leaning forward, and the needle is most often inserted
through the inferior aspect of the 7'h or 8'h inrercosral
space aiong the upper border of lower rib to avoid injury
to the neurovascular bundle in the mid or posterior axil-
\t
lary line. The landmark for evacuation of the fuid is the !
t
f
-

angle of the scapula that corresponds approximately to
the Sth rib interspace
The area is cleansed using spirit and iodine and draped.
Local anesthesia with 2o/o lignocaine is given at the
expected site of aspiration.
4. A wide gauze aspiration needle (blood transfusion needle
can be used) having tubing attached with it is introduced
perpendicular ro rhe skin. Fluid may be withdrawn by a
sterile 50 ml syringe after removing the clamp (clamping
can be made by a stopcock or by an artery forceps). It
is safe to use a three-way stopcock between the needle
and the syringe to minimize the risk of pneumothorax.
In general, as much fluid as possible can be withdrawn
gradually, and an upright chest x-ray should be obtained
after the procedure is over.
5. A sterile dressing is applied after the maneuver.
Complications: Infection, pneumothorax, and bleeding (on
the right by puncture or laceration of the capsule of liver; and
on the left the spleen).
Specimens obtained should always be cultured, examined
microscopically for evidence of bacteriai infection, and evalu-
ated for total protein and total differential cell counts. Lactic
acid dehydrogenase, glucose, cholesterol, triglyceride (chylous),
and amylase determinations may also be useful. If malignancy
is suspected, cytologic examination is imperative.
Tiansudates result from mechanical factors influencing the
rate of formation or reabsorption of pleural fluid and gener-
aily require no further diagnostic evaluation. Exudates result
from in{lammation or other disease of the pleurai surface
and underlying lung and require a more complete diagnostic
evaluation. In general, transudates have a total protein of <3
g/dl or a ratio ofpleural protein to serLlm protein <0.5, a total
leukocyte count of fewer than 2,000/mm3 with a predominance
of mononuclear cells, and low lactate dehydrogenase leveis.
Exudates have high protein levels and a predominance of
polymorphonuclear cells (although malignant or tuberculous
effusions may have a higher percentage of mononuclear cells).
Complicated exudates often require continuous chest tube
drainage and have a pH <7.2. Ti-rberculous effusions may have
low glucose and high cholesterol content.
This is the most direct method of obtaining bacteriologic
specimen from the pulmonary parenchyma, and the only
technique other than open lung biopsy not associated with
I contamination by oral flora. Indications for lung tap include
I
roentgenographic infi ltrates of undetermined etiology, especially
, if unresponsive to therapy.
t
PROCEDURE
l. The technique is very similar to that for thoracentesis.
A 19G or 22G disposable needle, attached to a 10 ml
F
I
l
PROCEDURES IN PRACTICE
syringe containing approximateiy 1 ml of sterile saline,
is inserted through the inferior aspect of an intercostal
space in the area of interest.
2. The needle is rapidly advanced into the lung, the saline
injected and re-aspirated, and the needle withdrawn; all
as quickly as possible.
3. This procedure usually yields a few drops of lung juice,
which should be cultured and examined microscopicail,v.
Complications are the same as those for thoracentesis,
although the incidence of pneumothorax is higher.
INDICATIONS
Diagnostic Determine etiology of ascites; diagnose peritonitis.
Therapeutic: Remove large volume of ascitic fluid (if causing
respiratory compromise).
PROCEDURE
1. The patient voids urine and lies in the supine position.
2. Paracentesis is usually performed at the lower quadrants
on either side of abdomen or at a site in the mid-line
halfrvay beween umbilicus and symphysis pubis, avoid-
ing inferior epigastric vessels, visible venous collaterals,
and scars of previous operations. Usually, the left lower
quadrant is preferred to the right in critically ill children
because they may have cecal distention.
3. Aseptic technique is used throughout the procedure.
4. The skin is cleansed and local anesthetic (2o/o lignocarne)
is infiltrated through to the peritoneum.
5. The aspiration needle (19-22G disposable needle) is
inserted through the skin and pulled sideways for a while
and is then inserted through the abdominal wall muscle
and peritoneum. Approximately 10-15 ml of fuid is
aspirated for studies, more to relieve respiratory distress.
6. The fluid obtained is examined macroscopicaliy, and
then sent to the biochemistry, bacteriology, and cytol-
ogy laboratories for analysis (culture, levels of amylase,
lactate dehydrogenase, bilirubin, albumin and protein are
estimated other than Gram stain and cytology).
Complications: Hemorrhage, fluid leak, intestinai or bladder
perforation and hypotension, if large volumes are removed.
This procedure is helpful to rule out subdural effusion or
empyema during infancy. This procedure is done in children,
with an open anterior fontanel usually up to the age of
18 months. Indications include unexplained excessive head
growth, bulging anterior fontanel, positive transillumination
of the skull.
 ESSENCE OF PEDIATRICS
PROCEDURE
1. The scalp hair must be shaved, and strict asepric precau-
tion should be taken.
2. A disposable needle 19G or 2lG is used for the procedure.
3. The patient is placed in the supine position and is firmly
held by an attendanr.
4. The subdural space is approached at the lateral border
of the anterior fontanel or along the upper margin of
the coronal suture ar least 2-3 cm from the midline to
prevent injury to the underlying sagittal sinus. The needle
is introduced at 90" degree to the scalp.
5. After a local anesthetic, the needle and stylet are slowly
advanced through the skin and underlying rissue with a
z-like movement until the dura is entered with a sudden
popping sensarion.
5. The needle should nor be advanced >1.5 cm from the
scalp surface ro prevenr advancement of the needle into
the cerebral correx. A hemostat amached -5-7 mm from
the beveled end ofthe needle should provide an adequate
safeguard.
7. The subdurai fluid, which may squirt our under pressure,
is collected and sent for protein analysis, cell count, and
culture. Bilateral subdural taps may be indicated, because
subdural collections are bilateral in mosr cases.
8. The amounr of fluid removed with each tap should be
limited to a total of 15-20 ml from each side in order ro
prevenr rebleeding from a sudden shift ofthe intracranial
contents.
9. At the termination of rhe procedure, a sterile dressing is
applied, and the child is placed in a sitting position
that tends to prevenr leakage offluid from the puncture
site.
Complications:
o Rapid removal of large quantities of fluid may cause shock
or intracranial hemorrhage from sudden shift of the intra-
cranial structures.
c Laceration of meninges or cerebral cortex due ro frequent
Complications: Infection, portal vein thrombosis, air embo-
to and fro movements of the needle.
c lism, overhydrarion, cardiac arrhythmias (caused by a catheter
Infection.
that is inserted too far and is irritating the heart), necrorizing
enterocolitis, hepatic necrosis, portal hypertension.
Note:
INDICATIONS
s Exchange transfusion or partial exchange transfusion.
* I;rtravenous infusion or medication when other veins are
n$t immediately availabie.
o Paienteral alimentation.
o Blood sampling.
o Cenrral venous pressure monitoring.
o Long-term central venous access in extremely low birth
weight infants.
PROCEDURE
1. The whole procedure should be done with utmost asepric
measure. The person doing it should wear gown, mask,
cap, and gloves.
2. Umbilical cord stump and surrounding skin is to be
cleansed with spirit/povidone-iodine solution.
3. Baby is to be draped with sterile roweis or drawsheet
keeping only the cord and umbilicus exposed. Excess
umbilical cord is cur rransversely with a surgical blade/
scaipel or scissors leaving a stump of 0.5-1.0 cm.
4. The umbilical vein is identified. It has the widest lumen
among the three vessels of umbilical cord and wall is
thin and remains in collapsed state, in contrast, arteries
are two in number (AVA), their wall is thick and lumen
is smaller.
5. The cord is kept upright and steady by the grip of
thumb and fingers of the left hand of the operator or
by the curved hemostat. The umbilical vein is opened
and dilated with the forceps or dilators. An umbilical
catheter (No. 5 French catheter for infants weighing
<3.5 kg and a No. 8 French carheter for those weighing
>3.5 kg or 6 or 8 FG size NG tube) is introduced by
the right hand through the umbilical vein for 5 cm;
being directed backward first, then upward, and slightly
backward. Another method is ro measure the length from
the xiphoid to the umbilicus and add 0.5-1.0 cm, which
indicate how far rhe venous catheter should be inserted.
When free flow of blood is obtained, the catheter is
usually in a large hepatic vein or the inferior vena cava.
Gentle aspiration may be done with the syringe to see
whether the blood {lows easily or not. If not, catheter is
further introduced 1 cm at a time and aspirating until
free fow of blood is observed. Usually, catheter needs
not to be passed beyond 7 cm.
Catheter is transfixed now to the stump of the cord with
silk, and is connected with an infusion ser, and drip started
to maintain the patency. A dry dressing is applied.
V4ren the cord becomes dried up (5-7 days' old), umbili
cal catheterization is done by (z) cutting the cord at its
insertion where it is still moist; vein can be identified with
care; and (ii) the cord may be kept moist by covering the
umbilicus and stump with a wer gauze for a few hours,
then identificarion of the vein will be easier after curting
the stump. But if the shedding of the cord had occurred
2-3 days previously, it is often difficult to identif, the vein.
When the umbilical cord has fallen off or umbilical vessel
cannot be cannulated, cut-down should be performed by
 PROCEDURES IN PRACTICE

making an incision on the anterior abdominal wall in the
mid-line above the umbilicus.
o The polyvinyl feeding tube of optimum size, sterilized with
gamma irradiation, can be used as a substitute of umbilical
catheter.
Exchange transfusion is done commonly in hemolytic disease
of the newborn due to Rh incompatibility. The aims of
exchange transfusion in Rh incompatibility are to correct
anemia, to remove damaged and antibody-coated RBCs from
the circulation, to remove unfixed antibodies, and to reduce
hyperbilirubinemia.
The procedure has been detailed under Neonatal Jaundice
in the Chapter "Neonatology".
Indications: To provide mechanical respiratory support, obtain
aspirates for culture, assist in bronchopulmonary hygiene (pul-
monary toilet), alleviate subglottic stenosis, clear the trachea of
meconeum, endotracheal medication (e.g., lidocaine, atropine,
naloxone, epinephrine).
7. Advance the blade a few millimeters, passing it beneath
the epiglottis.
8. Lift the blade verdcally to elevate the epiglottis and visual-
ize the glottis.
c)
To better visualize the vocal cords, an assistant may place
gende external pressure on the thyroid cartilage.
10. Pass the ET tube along the right side of the mouth and
down past the vocal cords during inspiration. It may be
helpful to tape the tube at the lip when the tube has
been advanced the measured length. The stylet sllould
be removed gently.
11. Confirm the position of the tube. The resuscitation bag is
attached to the tube, and an assistant provides mechanical
breath while the physician listens for equal breath sounds
on both sides of the chest. Auscultate the stomach to be
certain that the esophagus was not inadvertently entered.
12. Paint the skin with tincture of benzoin. Tape the tube
securely in place.
13. Obtain a chest x-ray film to confirm proper placement
of the tube.
Complications: tacheal perforation, esophageai perforation'
laryngeal edema, improper tube positioning, tube obstruction
or kinking, palatal grooves, subglottic stenosis.

PROCEDURE

1. The endotracheal (ET) tube should be precut to eliminate

dead space (cut to 15 cm). Refer Thble 24.1 for ET size INDICATIONS
and depth of insertion.
2. Be certain that the light source on the laryngoscope is
a Septicemia with or without sclerema
a Protein-energy malnutrition with anemia
working. A bag-and-mask apparatus with 100% oxygen
a Gastroenteritis with anemia
should be available at the bedside. Place the stylet (if
a Shigellosis
used) in the ET tube in such a way that the srylet does
a Hemorrhagic disorders
not protrude out of the end of the ET tube.
a Hemolytic-uremic syndrome
3. Place the infant in the "sniffing position' with the neck
a Preterm infants
slighdy extended.
a Severe anemia due to any cause
4. Cautiously suction the oropharynx as needed to make
the landmarks clearly visible.
5. Monitor the infantt heart rate and color. PROCEDURE
5. Hold the laryngoscope with left hand. Insert the scope
into the right side of the mouth, and sweep the tongue I. The whole procedure is to be performed with strict
to the left side. aseptic precautions. The operator shouid wash his hands
thoroughly and wear gloves. One assistant is necessary.
Blood grouping of the donor and recipient patient is done
earlier and cross-matched.
Table 24,1= Cuideline for Endotracheal (Et) Tube Size and
Depth of lnsertion 3 ml of anticoagulant (ACD facid citrate dextrose]) is
drawn in a 20 ml disposable plastic syringe from the
anticoagulant containing bag.
<1000 2.5 6.5-7 ). Then an easily-approachable medium-sized vein, e.g.,
antecubital vein of the donor is selected. After proper
1000-2000 3 7B
local antiseptic care, venipuncture is done by a 21G but-
2000-3000 3.5 8-9
terfy needle, and donor's blood is drawn into the syringe
>3000 3.5-4 >9
containing 3 ml of anticoagulant up to the mark 20 (i.e.,

ESSENCE OF PEDIATRICS
up to 20 ml). The syringe is disconnected and the blood
is transferred immediately into an empty blood-collection
bag (which can be procured from blood bank or market).
o The assistant should twist and press rhe tube of the
butterfly needle by his index and thumb obstructing
the blood flowing our. In this way, blood collection
from the donor and then transferring into the blood-
collection bag is continued dll the required amounr
of blood (20 ml/kg) is collecred.
4. Bag containing the required amounr of blood is then
suspended from a stand, and transfusion started at a rare
of 6-8 drops per minute. Complication of this procedure
is practically nil, except those of transfusion reacrions.
Note:
o Small-volume blood transfusion from mobile donors (parents
and relatives, doctors and social worker - available readily)
is found to be safe, effective, time saving, life saving, and
economic.
Itis undesirable that blood from professional donors are used
having the risk of lowhemoglobin and transmissible diseases.
Infants require only small amounr of blood. Therefore, the
traditional collection of blood from a donor (amounring ro
about 400 ml) for transfusing to small children is a wastage.
Moreover, obtaining blood from blood transfusion service
requires more time, and often fresh blood is not available.
Other advantages are donors are available at hand, blood
is not wasted, and the same donor may be used frequently.
Blood from the healthy donor is drawn at bed side.
The only reliable way ro obtain a reliable urine specimen in
neonates and young infants is by suprapubic aspiration. In
children <2years ofage, urine obtained by suprapubic aspiration
is likely to be contaminated and is the preferred specimen for
diagnosing UTI; isolation of any organism from this specimen
indicates bacteriuria. This method may also be used whenever
the results of mid-stream urine examination are not clear.
PROCEDURE
1. Strict aseptic precaurions should be taken. The newborn
infantt diaper should be dry for at least I hour ro ensure
that the bladder is distended with urine. Fluid may be
allowed to drink beforehand. In older infants, it may be
possible to percuss or to palpate the distended bladder. An
experienced assistant is needed to immobilize the infant.
2. The operatort hands should be washed thoroughly. The
suprapubic area is cleansed with spirit-savlon.
3. A disposable needle (21-23G) attached to a syringe (5
or 10 cc disposable syringe) is inserted I-2 cm above the
symphysis pubis in the midline (there is olten a skin crease
'l
in this location) with the syringe held in approximately
perpendicular ro the skin angled slighdy (10 degrees)
towards the head; the needle is inserted for an inch or
more. Slight decrease in resisrance may be felt when the
bladder is entered.
4. Minimal sucrion is applied and urine is aspirated. If no
urine is obtained, the needle should be withdrawn. Aimless
probing or repeated atremprs are nor desirable.
5. The syringe having urine is sealed with a sterile cap, or a
sterile second needle is attached to the syringe and made
bent, and is then sent ro the laboratory within half an hour.
Complications: Overall the procedure carries a complication
rate of approximately 0.2o/o.
r Microscopic hematuria (frequent)
o Gross hematuria
o Suprapubic hematoma
o Perforation of abdominal organ (e.g., bowel etc.)
Note:
o The common cause of failure in performing a suprapubic
tap is failing to wait until the bladder is fuli.
o If the needie is inserted roo close to the pubis or is angled
towards the feet, the bladder may frequently be missed
(since the bladder is an abdominal organ in the newborn).
Liver biopsy is used to determine rhe cause of acute or chronic liver
disease-neonatal cholestasis, chronic acrive hepatiris, metabolic
liver disease, suspected Reye syndrome, intrahepatic cholestasis
(paucity of bile ducts), congenital hepatic fibrosis, or undefined
portal hypertension; enzyme analysis to detect inborn errors of
metabolism; analysis of stored material such as iron, copper, or
specific metabolites; assess prognosis, determine response to *rerapy
and detect complications of treatment with potentially hepatotoxic
agents, such as aspirin, anti-infectives (ery.thromycin, minorycline,
ketoconazole, isoniazid), antimetabolites, antineoplastics, or anti-
conlr-rlsant agents. Analysis of the biopsy specimen can include
histology, metal conrenr, biochemical or enzyme assay, culture for
viral, bacterial or fungal pathogens, and electron microscopy. It
should be performed in hospital by someone experienced in the
technique. Prior to biopsy, it is necessary ro ensure thar:
o Any history of abnormal bleeding has been investigated
o The patient's blood group is known
o Tiansfusion facilities are available
o Hemoglobin is above 10 g/dl
e Platelet counr is at least 80 x l0eil
r Prothrombin dme is prolonged for no more than 3 seconds
(than that of control)
o Bleeding time has been checked if drugs affecting platelet
function have been taken
o HbsAg is negative \
o \Tritten consenr ofthe parents should be taken beforehand I
ll

PROCEDURE
Liver biopsy can be performed percutaneously at the bedside
or with ultrasound guidance. It can be performed by either
an anterior, subdiaphragmatic, or right lateral approach; the
last being the most commonly used.
1. The patient is placed supine with his right side close to
the edge of the bed, his arms behind his head. Careful
explanation and instruction should be given, particularly
in holding the breath at full expiration.
2. The point of maximum dullness to percussion at full
expiration in the right mid-axillary line in the 8th to
10th intercostal space is marked. After skin antisepsis,
1%o lignocaine is used to anesthetize down to the liver
capsule while the patient is at full expiration.
3. The Vim-silverman needle is usually employed (it consists
of a hollow needle, solid stylet, and inner split needle);
a tru-cut disposable needle can be used. It is safer to
Menghini biopsy needle for obtaining small sample.
The liver is palpated in the mid-axillary line. The
upper border of the liver dullness is also percussed and
marked. The skin (usually 10'h space) is cleaned with
spirit, draped and locally anesthetized. The skin is nicked
with a scalpel blade and the needle with stylet is inserted
medially through the ICS till it is felt to enter the liver,
the stylet is withdrawn and the split needle is introduced
and advanced fully into the liver. The outer hollow needle
is advanced completely and then whole needle is rotated
through 350" to break the core attaching to liver.
4.
Occasionally, small and fragmented specimens are obtained
from cirrhotic livers; a second pass usually yields sufficient
tissue, but no further passes should be attempted.
Contraindications: Include prolonged PT or INR; thrombo-
cytopenia; suspicion of a vascular, cystic, or infectious lesion
in the path of the needle; and severe ascites.
Complications: The risk of development of a complication
such as hemorrhage, hematoma, creation of an arteriovenous
fistuia, pneumothorax, or bile peritonitis is small.
If administration of fresh frozen plasma or of platelet
transfusions fails to correct a prolonged PT, INR, or throm-
bocytopenia, a tissue specimen can be obtained via alternative
techniques. Considerations include either the open laparotomy
(wedge) approach by a general surgeon, or the transjugular
approach under ultrasound and fuoroscopic guidance by an
experienced pediatric interventional radiologist in an appro-
priately equipped fluoroscopy suite.
INDICATIONS
Significant value: Steroid-resistant nephrotic syndrome, acute
renai failure of unknown cause, rapidly progressive renal failure,
I
PROCEDURES IN PRACTICE
systemic renal disease, inherited nephropathies, renal allograft
dysfunction.
Less value: Chronic renal failure, non-nephrotic proteinuria,
microscopic hematuria
PROCEDURE
I. Localization of the kidney: The lateral border of the lower
pole ofthe kidney is the safest part for biopsy. It is neces-
sary to locate the kidney by a plain x-ray of abdomen,
USG, or I\/U (if required). Ultrasound is particularly
useful in patients with impaired renal function, as it avoids
the use of large doses of contrast media. "Blind biopsy''
using plain abdominal radiography (KUB) or intravenous
urography to mark the position of the kidney has been
superseded by modern imaging techniques.
2. Choice of biopsy needle: Disposable needle, such as the
use
tru-cut needle has become more popular.
3. Biopsy of the kidney: The biopsy can be made consid-
erably less frightening by adequate premedication and
intravenous sedation with diazepam during the procedure.
It is important to fix the kidney so that it does not move
significantly with the patient's respiration, thus avoiding
laceration by the biopsy needle. The patient should be kept
in prone position. With his head turned to one side, arm
abducted; a rolled up towel is placed under the abdomen.
The position of the lateral border of the lower pole of
the patient's kidney is then marked on the patientt skin
on the back over the lumbar region. The bony landmarks
for selection of biopsy are the dorsal process of lumbar
spine and the lower border of 12'h rib. A point about 2
cm below the lower border of the rib is usually chosen as
the site for biopsy. The depth of the kidney is measured
using ultrasound. The biopsy can then be performed using
a tru-cut disposable needle (or Vim-Silverman needle),
which is inserted on the lateral border of the lower pole
of the kidney.
5. It is preferable to biopsy the kidney that can be more
easily visualized; a previously biopsied kidney should
be avoided if possible, as some of the histopathological
changes may represent damage caused by the first biopsy.
Enough tissue must be taken for light microscopy and
immunofl uorescence techniques.
COMPLICATIONS
Bleeding:
a Microscopic hematuria occurs in almost all patients.
a Macroscopic hematuria occurs in 5-10o/o of patients.
a Bleeding requiring transfusion occurs in approximately 17o
of patients.
Arteriovenous fistulas causing bleeding or significant hemo-
dynamic patterns are most uncommon.
ESSENCE OF PEDIATRICS
o Asymptomatic arteriovenous fistulas may be quite common;
their incidence is difficult ro assess.
e Perirenal hematomas, as demonstrated by CT scan, occur 4.
in up to 50o/o of parients, but are seldom troublesome.
5.
Other reported complications (rare):
o Pneumothorax
o Ileus
o Laceration of the liver, spleen, mesenteric artery, and bowel
o Death-none of the most recent reviews of renal biopsies 8.
reported death as a result of biopsy.
9.
10.
Behrman RE, Kliegman I)M, Jenson HB. Nelson Tixtbook of 11
Pediatrics 18'h ed. USA: \XrB Saunders Co.,2007.
Absar MN, Kawser CA. Umbilical catheterization in neonates. t2.
Bangladah J of Child Health 1985;9(2):1L4-5.
Hutchison JI{.. Practical Paediatic Problems 6,h ed. London: Loyd
Luke, 1986.
Ghai OP Essentials of Pediatrics 7'h ed. New Delhi: CBS Publishers,
2009.
Talukder M QK, et al. Small blood transfusion for infants from
mobile donors. Bangladesh J of Child Health 1985;9(4):215-7.
Kabir ARiVIL, Manajjir Ali. Suprapubic aspirarion of urine in
infants. Bangladesh J of Child Health 1.985;9(4):2\4-20.
Absar MN, Kawser CA. Exchange transfusion. Bangladah J of
Child Health 1 985;9(3) : 1 80-4.
Taube D. Percutaneous renal biopsy. In: Medicine Internationa/,
Bangladesh edition, l986;2(II):1307 -8.
Silver HK, Kemple CH. Handbook of Paediatics 15'h ed. Singapore:
Appleton & Lange, 1987.
Singh M. Care of the Newborn.3'd ed. New Delhi: Sagar Publica-
tions, 1985.
Parthasarathy A (ed.). IAP Textbooh of Pediatrics. 4'h ed. New Delhi:
Jaypee Brothers, 2009.
Gomella TL. Neonatokgy: ManagemeTtt, Procedures, On-call Prob lems,
Diseases, and Drugs 6'h ed. New York: Mc Graw Hill, 2009.
I
 CHAPTER
Some Selected Syndromes
Acrodermatitis Enteropathica (Brandt Syndrome) : Chronic
diarrhea (frequently steatorrheic), dermatosis usually around
body openings, alopecia, paronychia, frequent conjunctivitis
and blepharitis, zinc deficiency, J akaline phosphatase, famil-
ial, autosomal recessive.
Albright Syndrome (Fibrous Dysplasia of Bone): Skin pig-
mentation, precocious puberry areas of osseous rarefaction
resembling cysts, advanced bone age, fractures.
Alphar-Antitrypsin Deficiency: Persistent jaundice (obstruc-
tive) in a newborn, cirrhosis, adult emphysema, autosomal
recessive.
Alport Syndrome: Hereditary nephritis (more severe in male)
and perceptive nerve deafness.
Apert Syndrome: Consists of deformity of the skull second-
ary to closure of the coronal sutures' symmetric syndactyiy of
hands and feet, autosomal dominant.
Ataxia Telangiectasia: Progressive ataxia, choreoathetosis'
telangiectasia of conjunctiva, face, elbows and knees, sinopul-
rnon"ry infections, bronchiectasir, J ,.rrrrn IgA, autosomal
recessive.
Beckwith Syndrome (Beckwith-rViedemann Syndrome):
Macroglossia, macrosomia, omphaloceie, hyperplasia of
kidneys and pancreas, proneness to \7ilm tumor' hypoglyce-
mia, prominent facial nevus flamus.
Berger Disease: Gross hematuria (intermittent), benign focal
glomerular lesion.
Blackfan-Diamond Anemia: Congenital pure red cell hypo-
plastic anemia.
Bloom Syndrome: Dwarfism; congenital telangiectatic
erythema over malar area of face, nose, and lips;
photosensitivity; small narrow face; high-pitched voice;
protruding ears; diminished immunoglobulins; high
fre quency of chromosomal breakage.
25
Banti Syndrome: Splenomegaly from portal hypertension,
thrombocytopenia, leukopenia from hypersplenism.
Blue Diaper Syndrome Failure to thrive, blue discoloration
of diaper right from early infancy, irritabiliry constipation,
infections, recurrent fever of unknown etiology, high blood
urea, hypercalcemia, extensive nephrocalcinosis.
Caffey Disease (Infantile Cortical Hlperostosis): Non-
suppurative, tender, painful swellings over the flat and tubuiar
bones (subcutaneous tissue and joints are spared), irritabiliry,
fever, anemia, Ieukocytosis, high ESR and alkaline phospha-
tase, x-ray of bones reveals cortical hyperostosis; self-limited;
corticosteroid indicated in advanced cases.
Chediak-Higashi Syndrome: Oculocutaneous albinism, fre-
quent bacterial infection, lymphadenopathy, hepatospleno-
megaly, impaired platelet function and hemorrhage, abnormal
chemotaxis and lysosomal neutrophils contain greenish gray
granules, autosomal recessive.
Cleidocranial Dysostosis: Absent clavicles, delayed closure of
fontanels; au rosomal dominant.
Congenital Chloridorrhea: Neonatal diarrhea, low serum
chioride and potassium, metabolic alkalosis; autosomal reces-
sive.
Conn Syndrome (Hyperaldosteronism): May be associ-
ated with congenital adrenal hyperplasia in boys or adrenal
adenoma in females, hypertension, polydipsia, polyuria, hypo-
kalemia, alkalosis, tetany, growth retardation, increased excre-
tion of aldosterone in urine with normal 17-oxosteroids and
1 7-hydroxysteroid.
Cri-du-chat Syndrome: Chromosomal disorder, deletion
C5p), mewingJike cry, LBW, mental retardation, antimon-
goloid slanting of eyes, epicanthic folds, hypertelorism, micro-
cephaly, chromosomal disorder.
Crigler-Najj ar Syndrome : Typ eI neonatal j aundice,
-severe
autosomal recessive. Type 2-,mild neonatal jaundice, autoso-
mal dominant, responds to phenobarbital. Cause: Glucuronyl
transferase defi ciency.

ESSENCE OF PEDIATRICS
Crouzon Syndrome Acrocephaly, hypertelorism, shallow
orbits, exophthalmos, hypoplastic maxilla (fat facies), beak-
shaped nose, shorr upper lip and protruded lower lip; autoso-
mal dominant.
Cystinosis: Failure to thrive, cystine crystal deposits in eyes,
marrow and reticuloendothelial system; autosomal recessive.
Dandy-W'alker Syndrome: Hydrocephalus following atresi a
of the foramina of Luschka and Magendie when theZ,h ven,
tricle distends into a huge cyst; autosomai recessive.
Dermatitis Herpetiformis: Skin eruprions (vesicular and
itching), malabsorption consistent with celiac disease. Skin
lesions show slow response to elimination of gluten from d.iet.
DiGeorge Syndrome: Defects of heart and face, repeated
infections, neonaral retany, absent thymus and parathyroids,
normal immunoglobulins.
Dubin-Johnson Syndrome: Intermittent obstructive jaun-
dice, black pigment in liver biopsy; aurosomal recessive.
Iil.
Ebstein Anomaly: Tlicuspid valve arise from right ventricle,
tricuspid insufficiency, poor growth, distended neck vein with
CV waves, systolic murmur, large square cardiac shadow with
huge right arrium, notched P with RBBB on ECG.
Edwards Syndrome: Tlisomy 18. The skull is long and narrow
ears malformed, the hallux tends to be short and dorsiflexed,
there may be talipes calcaneovalgus. The calcaneus may be
prominent posteriorly, rocker-bottom feet; webbing of neck,
hypoplastic nipples, cleft lip or palate; cardiovascular anoma-
lies present. Dermatoglyphic findings are characeristic.
Ehlers-Danlos Syndrome Hyperelastic and easily scarred
skin, easy bruising; hypermobility and recurrent dislocation of
joints; autosomal dominant.
Evan Syndrome: Hemolyric anemia, thrombocyropenia.
Fetal Alcohol Syndrome: Excess consumption of alcohol
during pregnancyr pre- and post-naral growrtrfailure in weight,
height, OFC, short palpebral fissure, epicanthic folds; Jhin
upper lip, broad nasal bridge, upturned nose, maxillary hypo-
plasia, absent philtrum, nail hypoplasia, mild ro severe mental
retardation, incoordination, hypotonia; congenital anomalies
of heart, genitourinary tract, eye, ear, mouth, skeleton.
Familial Dysautonomia (Riley-Day Syndrome): Absence of
tears, poor perception of painful stimuli, excessive drooling,
..1
.l
l
-l
-l
.,1
sweating, skin blotching, paroxysmal hypertension; aurosomal -l
recessive. l
Fanconi Anemia: Congenital malformation of bones of
forearm, dwarfism, mental retardation, aplastic anemia devel-
oping in toddler; autosomal recessive.
Fragile-X Syndrome: 1:1000 males, accounts for 30-50o/o of
mental rerardation; more in males, fragile site on long arm ofX
chromosome, mental retardation, oblong face with .arr, larg.
testicles especially after puberry hyperextensible joints, ,p"..h
delay, hyperactiviry, infantile aurism; Xlinked recessive.
Freeman-Sheldon Syndrome (whistling-face syndrome,
craniocarpotarsal dysplasia): Stifl mask-like facies with
flattened facial bones, ptosis, blepharophimosis, small nose,
high-arched palate, microstomia with small rongue and thin
protruding lips.
Friedreich Ataxia: Cerebellar ataxia due to spinocerebeliar
degeneration, pes cavus, myocarditis, followeJ by scoliosis
later, at times diabetes insipidus; autosomal recessive.
Frohlich Syndrome: Obesiry hypogenitalism, growth retarda-
don, diabetes insipidus; Cause: IJsually inrracranial tumor.
G.
Gilbert Syndrome: Fluctuating unconjugated hyperbilirubi_
nemia (mild), which is aggravated by administration of nico-
tinic acid; aurosomai dominant.
Gray Baby Syndrome: Following 2-4 days' administration
of chloramphenicol may occur in the newborn as vomiting
or regurgitation, refusal to suck and abdominal distension,
peripheral circulatory failure. In another day or so, the baby
develops ashen-gray color and becomes limp and severely dys-
pneic. He may die within 1-2 days of onset of manifestations.
Hand-Schuller-Christian Disease: Histiocytic infiltration
of bone lesions, exophthalmos, and diabetes
causing triad
insipidus.
Hartnup Disease: Intermittent ata,xia, photodermatitis, psy_
chosis, generalized neutral aminoaciduria; autosomal rec.ssirr..
Holt-Oram Syndrome: Atrial septal defect, triphalangeal
thumb.
Hunter Syndrome: Deafness, cornea not affected. Re.sem_
ble those with Hurler syndrome but with less severe somaric
changes; X,linked.
Hurler Syndrome (Gargoylism): Groresque features, large
head, short neck, bony abnormalities, enlargement of liver aid
spleen. Menta-l retardation, corneal opaciry cardiomegaly.
)
t

I3.
Kartagener Syndrome: Dextrocardia (usually situs inversus
totalis), chronic sinusitis, chronic bronchitis, bronchiectasis.
Kasabach-Merritt Syndrome: Giant hemangioma, platelet
trapping and consumption, DIC.
Kawasaki Disease (Mucocutaneous Lymph Node Syndrome):
Prolonged high pyrexia, skin and mucous membrane lesions,
bilateral conjunctival congestion, erlthema palms & soles with
desquamation, cervical adenopathy; arthralgia/arthritis, pyuria,
proteinuria, mild hepatitis, aseptic meningitis, cardiovascular
involvement infrequent with coronary aneurysm.
II.
Larsen Syndrome: Multiple congenitai dislocations, includ-
ing anterior dislocation of the tibia, ilat facies, frontal bossing,
hypertelorism, depressed nasal bridge, talipes equinovarus.
Laurence-Moon-Biedl Syndrome: Obesity, hypogenitalism,
short stature, retinitis pigmentosa, polydactyly, mental retarda-
tion; autosomal recessive.
Lazy Leukocyte Syndrome Gingival stomatitis, recurrent
upper respiratory infections, otitis media, skin infection, intrac-
tabie persistent pyrexia, leukopenia; a neutrophil defect leading
to absence of polymorphonuclear motility from bone marrow.
Leigh Syndrome: Subacute necrotizing encephalopathy with
progressive neurologic deterioration. Early manifestations-
feeding difficulties, feebie or absent cry, floppiness. Late mani-
festations-optic atrophy, seizures. Autosomal recessive'
Lesch-Nyhan Syndrome: Psychomotor deterioration pro-
gressing to choreoathetosis and self-mutilation by 2 to 3 years,
abdominal pain, uric acid crystaluria, renal failure, elevated
plasma uric acid; X-linked recessive.
Letterer-Siwe Disease: Histiocytic infiltration leading to hep-
atosplenomegaly, purpuric seborrheic eczema'
Lowe Syndrome: Cataract, buphthalmos, mental retardation,
aminoaciduria; X-linked recessive.
Lutembacher Syndrome: Atrial septal defect, mitral stenosis.
Maroteatrx-Lamy Syndrome (pycnodysostosis) : Dwarfi sm,
delayed closure of fontanels, dysplasia of skull, cortical den-
sities of the bones, short digits with wrinkled skin and nail,
parrotJike nose, partial anodontia.
Mc-Cune-Albright Syndrome: Precocious puberry fibrous
dysplasia of bones, feathery-edged pigmentation.
Megacystis Mega-ureter Syndrome: Bilateral dilatation of rhe
ureters is associated with greatly distended bladder, recurrent
pr-uria, no apparent organic obstruction; more in girls.
SOME SELECTED SYNDROMES
Mikity-\(zilson Syndrome (BubblyJung Syndrome) : Resp i-
ratory distress, expiratory grunting, chest retraction, cyanosis,
occurs in premature infants shortly after birth; X-ray chest
shows combined segmental collapse and over inflation.
Morquio Syndrome: Neck is short, marked dorsal kyphosis,
sternum protrudes, arms are long extend up to the knees, wad-
dling gait, epiphyses are misshapen and fragmented. Urine
contains keratan sulfate.
MURC Association: An association of Mullerian duct aplasia/
hypoplasia (MU) manifesting as genital anomalies, renal agen-
esis/ectopy (R), and fusion of cervicothoracic vertebrae (C),
probable teratogenic origi n.
Myotonic Dysrophy: Infantile hypotonia, feeding difficul-
ties, mentai retardation, cataracts, myocarditis, frontal baldness
(later); autosomal dominant.
o.
Osteopetrosis (Marble Bone Disease): Thickened fragile
bones, pancytopenia, splenomegaly; autosomal recessive.
Oropalatodigital Syndrome: Small nose, hypertelorism,
broad nasal root, frontal and occipital bossing, cleft palate,
growth and mental retardation, irregular fingers and toes,
limited elbow extension, and wrist supination.
II.
Patau Syndrome (trisomy 13-15 or D): Head is small and
abnormal, low set ear, eyes tend to be small with colobomata
of iris, cleft lip and/or palate, deafness and convulsion, poiy-
dactyly, cardiac malformations such as PDA and polycystic
kidneys, characteristic dermato glyphic fi ndings.
Peutz-Jeghers Syndrome: A rare type of polyposis involv-
ing the jejunum, duodenum, and sometimes the stomach.
Small pigmented areas, attacks of incomplete intestinal
obstruction due to recurrent intussusceptions, hemateme-
sis, and melena.
Pierre-Robin Syndrome: Micrognathia-the primary abnor-
mality is hypoplasia of the mandible, a secondary effect is glos-
soptosis due to backward displacement of the attachments of
the genioglossi to the mandible; this allows the tongue, which
falls backward and downward, to obstruct the oropharynx.
There is inspiratory stridor, cyanotic attacks, sternal recession,
and feeding dilficulties. Death commonly occurs from pneu-
monia.
Prune Belly Syndrome: Bladder neck obstruction with gross
deficiency of the abdominal musculature, patent urachus may
be present, allows urine to drain from the umbilicus.
Prader-Villi Syndrome: Short stature, hypotonia, obesity,
mental retardation, hypogonadism, cryptorchidism, almond-
shaped eyes, sma1l hands and feet.

ESSENCE OF PEDIATRICS
Progeria: Normal birth weight, early growth failure, prema-
ture senility, remarkable loss of subcuraneous fat, bald head,
absence ofeyebrows, atrophic nails, osteoarthritis, arterio scle-
rosis.
Rett Syndrome: Occurs in females; a previously normal child
begins slowing at 7-B monrhs of age, deceleration of head/
brain growth, early communication dysfunction with autistic
features, loss of purposeful hand skiil, severe impairment of
expressive and receptive language, stereoryped hand move-
ments, gait apraxia, truncal ataxia, dementia, seizures; by the
age of 10-12 years, child becomes wheel chair bound due
to hypertonia, rigidity. Defect in zona compacta of substan-
tia nigra, disturbance in neocortical maturation with reduced
microtubule associated protein (MAP).
Reye Syndrome: Acute encephalopathy, hypoglycemia,
hyperammonemia, greatly elevated rransaminases, prolonged
prothrombin time, metabolic acidosis, respiratory alkalosis,
microvesicular fatty change in liver, mitochondriai change
in hepatocytes and neurons, associated with aspirin and viral
infection.
Riley-Day Syndrome: S ee familial dysautonomia.
Ritter Disease: Bright eryrhemarous eruprion over face, neck,
axilla and groin, changing into a wrinkled appearance with ill-
defined flaccid bullae filled with clear fluid, areas of epidermis
separate when gently stroked (Nikolsky sign); within 2*3 days,
postinflammatory desquamation; conjunctivitis, pharyngitis,
stomatitis caused by Stapbylococcus Aureus.
Rothmund Syndrome Poikilodermia, cararacs, small saddle
nose, microdontia, hyperkeratosis of pa"lms and soles, mental
retardation.
Rotor Syndrome: Neonatal jaundice, which persists; auroso-
mal dominant.
Rubinstein-Taybi Syndrome (Broad Thumb-Hallux
Syndrome): Growth and mental retardation, characteristic
facies with beak-like nose, narrow high palate, prominent
forehead, low-set and slightly anomalous ears, palpebral
fissures showing antimongoloid slant, abnormally wide
thumbs and first toe.
Russell-Silver Syndrome: Short srarure, asymmerry of the
body, triangular face, clinodacryly, early sexual development.
Schmidt Syndrome Idiopathic adrenal insufficiency, hypo-
thyroidism, insulin-dependent diabetes mellitus, hypoparathy-
roidism, gonadal failure, and autoimmune endocrinopathy.
Sjogren-Larsen Syndrome: Mental retardation, spastic paral-
vsis. congeniral ichrhl osis.
-l
Sotos Syndrome (Cerebral gigantism): Excessive growrh
(height and weight are significantly large in first 4 years), mild
mental retardation, acromegalic facies, large hands and feet,
large head with broad forehead, receding hair line, anrimon-
goloid slant, bone age advanced.
Spasmus Nutans: Abnormal posrure and movements of head,
nystagmus.
Sturge-W'eber Syndrome: Extensive curaneous porrwine
hemangioma of upper face and scalp and a similar vascular
anomaly of the underlying meninges of the same side, convul-
sion, hemiparesis, menral deficiency, glaucoma, tram-line cal-
cification on X-ray skull.
Subacute Sclerosing Panencephalitis (SSPE): Progressive
dementia, spasticity, seizures (especially myoclonic). EEG
showing burst suppression pattern, measles antibodies in CSF;
supposed to be secondary ro an old attack of measles.
Ir.
teacher-Collins Syndrome: Hypoplasia of malar bones,
antimongoloid slant of palpebral fissures, colobomas, pre-
auricular pits, deafness, micrognathia, cleft palate, malocclu-
sion of teeth, absent eye iashes, CHD t; aurosomal dominant.
W.
'Waterhouse-Friderichsen
Syndrome: Acute bilateral adrenal
apoplexy is most commonly seen in fulminating cases of
meningococcal septicemia. Child suddenly becomes ill, there
is vomiting, diarrhea, patienr becomes semicomatose, there are
patchy purple rashes in the skin. Features of meningitis.
.Weber-Christian
Syndrome: recurrent episodes of fever, non-
suppurative nodules in the subcutaneous tissues.
$/olf Syndrome: Many fearures similar to rhe Cri-du-chat
syndrome, although the cat-like cry often absent. Differentiat-
ing features include broad root of nose, prominent glabella,
marked hypertelorism, coloboma of iris, pre-auricular dimples,
mid-line defects of the scalp, hypospadias, and low-set ears.
'Wolman
Disease: Failure to thrive, vomiting, diarrhea,
organomegaly, adrenal calcification, leukocyte acid lipase
absenr; au tosomal recessive.
Z.
Z,ellweger Syndrome (Cerebrohepatorenal Syndrome):
Hypotonia, flat facies with high forehead, low birth weight,
1'aundice developing in first few days or weeks, delayed psycho-
motor developmenr, death usuaily occurs by slxth month of age.
Zollinger-Ellison Syndrome: Peptic ulceration, hypertrophy
of gastric mucosa and excessive acid secretion due to non beta-
islet-cell adenoma.
\t
It
a
I
 I

CHAPTER 26
Laboratory Medicine

Chopter Contenls

Complete blood count:

Hematocrit (HCT, Hct) % of packed red {V red cellsl/ Volume fraction
whole blood cells x I00t (V red cellsA/ whole blood)
Calculated from MCV
and RBC count 1 day 48-69"/" x O-01 0.48-0.69
2 days 48-75"/" 0.48,0.75
3 days 44-72.k o.44-0.72
2mo 0.28-0.42
6-12 yr 35-45"/" 0.3 5-0.45
12-"18 yr, M 37*49"k 0.37-O.49
F 36,45% 0.36-0.45

Hemoglobin (Hb) sldl mmol/L

l-t days 14.5 )2.\ x 0.155 2.25-3.49

2 mo q.0- 14.0 1.40-2.17
b-12 yr I 1.5 15.5 1.78-2.40
l2-lByr,M li.0-tb.0 2.Q2-2.48
F t2.0-16.0 .1
.86-2.48

Erythrocyte indices:
MCH oercel I
fmol/cell

Birth 3l-37 x 0.01 55 0.48-0.57

1 3 days 3l-37 0.48-0.57
I wk-l mo 28-40 o.41-O.62
2 mo 26-34 0.40-0.53
3-b mo 25-t5 0.39-0.54
0.5 )yr )3 31 0.36-0.48
2 oyr 24 30 o.37-O.47
6-12 yr 25-33 0.3 9-0.5 1
12-18 yr 25-35 0.39-0.54

MCHC ToHb/cell or mmol

I Hb,dl RBC Hb/L RBC
Birth 30-35 x 0.155 4.65-5.s8
1-3 days 29-37 4.50 5.74
1-2 wk 28 38 4.34-5.89
1-2 mo 29-37 4.50-5.74
3 mo*2 yr30-36 4.65-5.58
2-18 yr 31-37 4.81-5.74

um'
l-3 days 95-1 2 I 95-121
0.5 yr 2 7O-86 70-86
6 12 yr 77-q5 77-95
l2 lByr,M 7B-qB 7B*98
F 78- lO) 78*102
 a

ESSENCE OF PEDIATRICS

ESR
Westergren. modified chitd 0-1 0 mm/hr x'l 0-10 mm/hr
Wintrobe child 0-13 mm/hr x1 0-13 mm,4rr
Leukocyte courrt x 1GOO cells/mm3 {pl) x 1 0' cells/L
Birth 9.0-30.0 .x1 9.0-30.0
24 hr 9.4*34.0 9.4-34.O
l mo 5.O-1q.5 5.0-19.5
1-3 yr 6.0-1 2.5 6"0*17.5
4-7 yr 5.5-1 5.5 5.5*15.5
B-1 3 yr 4.5-1 3.5 4.5*1 3.5
Adult 4.5-11.o 4-5-11.O

Leukocyte differential Number fraction

Myelocyles 0o/" x 0.01 0
Neutrophils tbandr J-5"h 0.03-{.os
Neutrophils lsegsr 54*62y, 0.544.62
Lymphocytes 25-33;oA, 0.25-0.33
Monocytes 3,70/" 0:03-0.07 :
Eosinoph ils 1-3% 0.0'l-0.03
Basophils O*O:7SYi'. 0.0.0075
Platelet count x 10r1mm3 (pL) x I Oe/L
rThrombocyte count) Newborn 84478 {a{ter 1 wk, x lOb 84478
same as adult)
Adult t5o-4oo 150-400
Alanine aminotransferase (ALT, 0*5 day 6'.s0 U/L x1 6-50 UiL
SCPT) 1-1 9 yr 5-45 U/L 545 UlL
Albumin Premature I day 1.8-J.0 gidl x 10 1 B-30 gil
Full-term <5 day 2.5-3.4 g/dl 2s-34 gL
<5 yt 3.q-5.0 B/dl 39-s0 g/L
5-19 yr 4.0-5.j g/dl 40-53 gL
S. protein tgldl1 lnfant 6.1 -7.9; thereafter 6.4-8.2.
S. bilirubin 1mg/dlr Cord blood: Pieterm and term
baby: <2
>7d: Preterm <2.0; Term 0 .2-1
Ammonia 18-90 pmol/L xl 1B-90 pmol/L
Amylase J0.1 00 u/L x-l 30-1 00 ull
Anion gap {Na tCl.HCOr}) 7-16 nEQL x 7-16 mFq/L
Aspartate ami notransferase (AST, 0-5 day 35-140UlL x 35-140 U/L
SCOT) t-9 yr l5-55 U/l x 1 5-55 U1L

10-1 9 yr 545 U/L X s-4s u/L

Bicarbonate 22-29 mmal/L xl 22-29 mmol/L
C-reactive protein <6 mgldl x1 <60 mgll
Calcium, ionized a.3-s.0 mg/dl x 0.25 1.07-1 .25 mmol/L
Calcium, lolal 9.0-1 1 mgidl x 0.25 2.3-2.75 mmol/L
Creati.nine 0.3-1 .2 mg/dl x BB.4 27-1O6 pmol/L
Creatine kinase Newborn 85:1 ?00 UIL
Adulr 5-1J0 U/L
Ceruloplasmin 0*5 day 5-25 mgidl
1-19 yr 22-45 mgldl t
Ferritin 7-14O ng/ml x1 7-1ao p{L
Clucose Cord blood
45-96 mgJdl x 0.0555 2.5-5.3 mmol/L
i
Premature 20-60 mg/dl 1.1-3.3 mmol/L :
Newborn
day
1 a0-60 mg/dl 2.2-3.3 mmol/L
day
>1 50*90 mgidl 2.8-5.0 mmol/L I
Child 60-100 mg/dl 3.3-5.5 mmol/L
 LABORATORY MEDICINE

LDH 1 9 yr 1 50-500 U/L

10-19 yr 120-330 U/L

lron All ages 22-1Ba Vg/dl x 0.1791 4-33 pmol/L
lron binding r apacity, tolal rTlBCr lnfanl 100 400 pgrdl x 0.179 17.9O-71 .60 ltmol/L
Thereafter250 400 pg/dl 44.75-71.60 pmol/L
Chloride 96- I 0b mmol/L xl 96-1 06 mmcl/L
Cortisol Newborn 1-)a pg/dl x 27.59 28-66.2 nmollL
Adult, B:00 AM \-) l 138-635 nmol/L
4:00 PM t- I 5 B2-4 13 nmol/L
8:00 PM <50% of B:00 AM

pH Birth: 7. I 7.Jb:
Older , hildren: 7.l5-7.4\
Osmolality <lyr: 275-295 mOsm/kg
Potassi u m <2 mo 3.0-2.0 mmol/L x1 3.0-7.0 mmolll
2 12 mo 3.5-6.0 mmol/L 3.5-6.0 mmol/L
> l2 mo i.5-5.0 mmoli L 3.5 5.0 mmol/L
Sodium l.]4 l4b mmol/l x1 134 146mmol/L
Thyroid-sti mu lating hormone Cord blood 2.3-1 3.2 mlU/L x1 2.3-1 3.2 mlUil
I 2 days i.2 J4.b mlL/L 3.2-34.6 mltJ/L
3-4 days 0.7-15.4 mlU/l O.7-15.4 mlU/L
2-20 wk 1.7-9.1 mlU/L 1 .7-9.1 mll-)/L
2l wk-20 y( 0.7-6.4 mlu/l O.7-6.4 mlu/L
Thyroxine, total 1 3 days 8.2 19.9 pg/dl x 12.9 106-256 nmol/L
1 wk 6.0-1 5.9 prg/dl 77-205 nmol/L
1- l 2 mo 6.1-1+.9 1t{c1l 79-192 nmol/L
I-J yr b.B- 1 3.5 pgidl BB-1 74 nmolll
t- l0 yr 5 5-12.8 pgdl 71-1 65 nmol/L
'10 yr 4.2-13.0 pg,dl 54-167 nmol/L
Thyroxine, free 3 day 2.0 4.e ng/dl x 12.9 26-631 pmol/L
lnlanl 0.9-2.0 ngzdl 12-33 pmol/L
Prepubertal children 0.8-2.2 nf,dl 10 28 pmol/L
Puberty & adult
0.8-2.3 ng/dl 10-30 pmol/L
Tri-iodothyron ine, free Cord blood 20 2a0 pg/dl x 0.01536 0.3-3.7 pmol/L
1 3day 200-610 pg/dl 3..1-9.4 pmol/L
6 wk & thereafter 2a0-560 pg/dl 3.7-8.6 pmol/L
Tri-iodothyronine, total Cord blood 30-70 n{dl x 0.0.1 54 0.46-l.08 nmol/L
Newborn 75 260 ng,/dl 1.16-4.00 nmol/L
1-5 yr 100-260 ng/dl 1.54-4.00 nmol/L
5-1 0 yr 90*240 n{dl 1.39-3.70 nmol/L
10*15yr B0-21 0 ng/dl 1.23-3.23 nmol/L
I nereafter 1 1 5-1 90 ng/dl 1.77 2.93 nmol/L
Uric acid 1-5 yr 1.7-5.8 mg/dl x .59-48 100-350 pmol/L
6-11 yr 2.2-6.6 mg/dl 130-390 pmolll
12-19 yr, M 3.O-7.7 mg/dl 180-460 pmol/L
12-19 yr, F 2.7 5.7 mg/dl 160 340 pmol/L
Urea <'lyr 5 lBmg/dl
>1 yr 7-18 mg/dl
O, saturation (Arterial SaO,) New,born: 85-90%
Thereaiter: 95 999L

Cell count/cmm:
Cell type Preterm Term Neonate Thereafter
Volume: Lymphocytes 0-25 0-20 0-5 0-5
Child: 60-100 ml
Polymorphs 0-20 0-.1 0 0-1 0 NiI
Adult: 100-160 ml
"a RBCs O
.lOO0
o-Bo0 0-50 Nil
I Pressure: 70-80 mm of H,O

I
It
 I

ESSENCE OF PEDIATRICS

Protein (mg/dl): Preterm-40-300; Term-45_190;

Neonate-40- I 20 ; Thereafter- 1 0-20
Glucose (mg/dl): 50-70o/o of corresponding blood glucose level 1. ParthasarathyA (ed). IAP Tbxtbooh of Pediatrics 4,h ed. New Delhi:
Jaypee Brothers, 2009.
Chloride: I 15*130 mmol/L 2. Behrman RE, Kliegman Rlvl, Jenson IHB. Nelson Textbook of
\7B Saunders Co.,2007.
Pediatrics l8'h ed.

Chloride 40-50 mmol/L (Border line); Normal <40 mmol/L.

In Cystic fibrosis values are >60 mmol/L

:
\
ilil
1l
 CHAPTEF-2T

Drug Therapy in Children

Chopter Contents
Ceneral medicati0ns...........,.......... 473 Anlilungal meditalior .503 Antiparasitic medications ..,..................................................505
Antibacterial medications (antibiotics) ............ .................497 Antiviral medications............ .... .504
Antimycobacterial medica1i0ns........................................... 503 Antiretroviral HIV medications 504

Acetaminopherr (Paracetamol) Mild to moderate pain (inhibits prostaglandin synthesis in Overdose can cause fatal hepatic necrosis,
Tamen, Ace120 mgl5 ml syp, CNS and peripheral pain impulse generation). Treat acute overdoses with acetylcysteine.
500 mg lab; Apa. Napa, I 15, Fever (inhibits hypothalanric heat regulation center).
250 mg suppository, B0 mg/ml drop 10-1 5 mg/kg/dose q 4-6 hr. PO, PR; max 5 doses/24 hr.

Acetazolamide Hydrocephalus due to communicating intraventricular Metabolir acidosis, h;pothloremia.

Acemox, Remox, 250 rng tab. hemorrhage (carbonic anhydrase inhibition decreases hypokalemia, anorexia, drowsiness, muscle
CSF prodr-rction). weakness, renal calculi.
Neonates: 25 nrg/kg q day to start and increase to bid, tid,
and qid over 4-Z da1,s.
Claur onra t arbonic anhydrase inhibirion delrea\es
formation of aqueous hr-rmor) B-30 mg/kg/d PO
dividedqo Bhr.
Epilepsy. a. adiunt I lo olher drugr in refrartory seizurps
(uncertain mechanism) B-30 mg/kg/d in 1-4 divided
doses ,m.rrimum I Brdr.
Albumin Human Plasma volume expansion and treatment of hypovolemia Precipitation of heart failure,
20%, albutein 25% inj in 50 ml & (increase intravascular oncotic pressure and mobilize flurd pulmonary edema, hypertension lar hyr ardia
100 ml bottle. from interstitium to intravascular space). due to volume overload. lmmune reactions
.l
0.5 g/kg/dose (max 6 glkg/d). re.B.. fever. t hills, ra'hr

Allopurinol Prevents attacks of gouty arthritis ancl nephropathy. Skin rashes including erylhemd multiforme.
Esloric, AIoric 100 mg tab Prerenl: t ancer chemolherapy indur ed hyperuricenria renal impairmenl, hepatilis. peripheral
'inhihitr ranthine oxidase thur preventing ronversion neuropalhv, r asculitis.
of hypoxathine lo uric at idr.
lO mg/kgrd in 2- | divided dose*.
Cout, chemotherapy-induced hyperuricemia 500 800
mgd in I I dir ided doqe5 startinq I 2 davs prior 1o
r hemolherapy anrl t onlinue for I day5.

Aminocaproic Acid Treatment of excessive bleeding resulting from systemic Hypotension, bradycardia, arrhythmias,
Caproli.in 2 g in l0 nrl amp. hyperfibrinolysis (inhibits activation of plasrninogerr), headache, nasal congestion.
loading dose PO, IV 100-200 rng/kg; maintenance '100
mg/kg eve11, 6 hr, continuous infusior.r.
Aminophylline' Apnea of prematuritv. Bronchocl lator,',veak pu lnronarv
i Feeding intolerance in neonates or Cl
Cardophylin 100 mg tab, anti-i nf lammatorr, efiects. discomfort, vomiting. CNS irritability, agitation;
125 m{5 ml amp. lncreases contractilitv ancl decreases fatigLrabilitv of tachvcardia, and tachyarrhythmias.
Aminomal R 600 mg tab; diaphragm and respiratorl' muscles, CNS stimulation.
350 mg/2 ml and 240 mg/10 ml amp; Exact nrechanisms for these effects renrain control,ersial.
J50 mg suppository. Neonates (for apnea of prematurity or bronchospasm):
Loading dose, 5 mg/kg lV or PO; maintenance dose, 2.5-3
mg/kg/dose q 12 hr lV or PO.
ln severe acute asthma, 5 mg/kg/dose lV with eq vol of 5'lo
rF D/saline as a bolus over 20 min, then 0.5-0.7 mg/kg/hr.
I

l,
ESSENCE OF PEDIATRICS
Amitriptyline Hydrochloride
Tryptin, Saroten 10 mg 25 mg tab;
Saroten retard 25 mg. 50 mg cap.
Antihemophilic Factor, Vlll lluman
Emoclot Dl 250 lUlvial
Antivenin Polyvalent
lohn Hopkins) Antivenom
Lyophilized serum, diluent (10 ml);
one vacuum vial to yield l0 ml of
antivenom.
Ascorbic Acid
Ascoson 250 mgtab, 500 mg in 5
amp; Celin, Ceevit 250 mg nb:' : '
Asparaginase
Asparaginase, Kidrolase I 0.000 and
50,000 IU/vial.
Aspirin
Ecosporin, Cawa 75 mg, 300 mg tab.
Disprin 300 mg CV 100 mg tab.
Atenolol
Betanol, cardipro 50 mg, 100 mg tab.
Atorvaslatin
Lipitor, Anzitor 10, 20 mg tab.
Alropine Sulfate
Atropin, Mydripine 0.6 mg/l ml amp
Azalhioprine
lmuran 50 mg tab.
Baclofen
Baclofen, Lioresal 10 mg tab, 5 mg/5
ml liquid.
Beclomethasone
Decomit, Beclomin inhaler 50 pg,
100 pglpuff,
Beclo{ortq 250 mglpuff
Beeospray, Beconase nasal spray
50 pg/spray
Depression (increases. CNS concentrations of serotonin Dry mouth, constipation, weight gain, postural
and norepiitephrine by inhibiting reuptakb). hypotension, drowsiness, headache, visual
1-1 .5 mg/kg 24 hr divided tid. disturbance.
Migraine prophylaxis:
Children-0.1 mg,4<g at bedtime and adVance over 2-3 wk
to effect. Max 2 mg/kg at bedtime,
Adolescents-25 mg divided bid and increase dose to
effect or maximum dose 200 m/d
Factor Vltl deficiency in hemophilia (provides factor Vllt). Tachycardia, allergy, blood-borne viral
Units required = weight (kg) x 0.5 x desired increase infections.
factor Vlll (7o of normalr
Antivenom for snake bite (Cobra tCokhral, Krait lKeoreyl, Sensitivity reactions, including anaphylaxis
Russels viper lChandraboral, carpet viper.). (treat with epinephrine. hydrocortisone and
Dosing based on severity of bite: Mild, 5 vials; moderate, antihistamine and brief holding of dose).
1 0 vials; severe, >15 vials (to be given with 5% DNS over
30-60 min or lV push or deep lM).
Scurvy: 1 00-j00 mg/d. Cl upset, renal stones
ml Urrinary'acidiiication; 500 mg eyery 6 hr. .
Prior to DF therapy in Thalassemia: 100 mg daily,
Cancer chemotherapy (inhibits protein synthesis to deprive Myelosuppression: onset Z days, nadir 14 days,
cancer cells of asparaginei. recovery 21 days, Hepatotoxicity, pancreatitis,
Doses may vary depending on specific pratocol being CI upset, hyperglycemia, coagulopathy.
used; 10,000 ulmz/d 3 times/wk lM for 4,6 wk in ALL.
Pain, inflammation, tever {prostaglandin synthesis Bleeding from gums or Cl tract, gastric ulcers,
inhibitiont. bronchospasm in asthmatics, hearing loss,
l0-l 5 mg/kg/dose q 4 6 hr. tinn itus. hyperventi lation.
Rheumatic fever, jRA 60-100 mg/kg/d divided every 6 hr. Contriindicated in children < '1 6 yr with
Kawasaki disease (acute phase) 80-100 mg/k$d divided chicken pox or flue due to risk of Reye
every 6 hr. syndrome, and in hemophilia. Discontinue if
hearing loss or tinnitus.
Hypertension, arrhythmias (competitiverbeta I -blocke0. B.radycardia, headache, constipation, wheezi ng,
0.8-1 .s mg/kg/d (max 2 mg/kg/d). dyspnea.
Hypercholesterolemia {- HMC-CoA reductase & J Dyspepsia, pancreatitis, hepatitis arthralgia.
.10-80
cholesterol level.) rng/d.
Preoperative medication to inhibit secretions and Tachycardia, palpitations, delirium, ataxia, dry
salivation (blocks action of acetylcholine and antagonizes hot skin, tremor, impaired vision.
histamine and serotonin).
Neonates and children: 0.1-0.2 mg/kg 30 min,precip then
every 4-6 hr.
Treatment of sinus bradycarclia: Neonates and children.
0.02 mg/kg (min dose 0..1 mg) lV (max 0.5 mg); may
repeat 5 min later, one time.
Antidote to organophosphate poisoning: lnitially a bolus
o{ 0.05 mglkg/dose lV stat then 0.02-0,05 mg/kg every
10-20 min until atropine effect (tachycardia, rnydriasis,
fever), then every 1-4 hr for at least24 hr. ''
TreaLment of autoimmune diseases, e.g., lupus, arthritis, Fever, chil ls, vomiting, diarrhea,
nephrotic syndrome, (inhibit synthesis of DNA, RNA, and thrombocytopenia, leukopenia, hepatotoxicity,
proteins. Antagonize purine metakrol ism.): skin rash.
1 m{kg/d x 6-8 wk.
Spasticity associ.ted with CP/MS or spinal Cdrd lesions Drowsiness, vertigo, psychiatric reactions,
(inhibits transmission of monosynaptic and polysynaptic ataxia, hypotonia.
reflexes at the spinal cord level).
10-1 5 mgid divided q B hr and titrate up every 3 days
(max 40 mg/d orally).
Asthma (oral inhalation), rhinitis (nasal aerosol), Candida in mouth. irritation of nasal mucosa,
anti-inflammatory, immune modulator.
.l-2
cough, hoarseness, headache. .r
lnhaler: inhalations 2-4 times daily
(max dose 10 puffs daily).
'l
Nasal sprays: 1 spray in each nostril 2 4 times daily.
1
f,
I
 '1

DRUG THERAPY IN CHILDREN

Benzoyl Peroxide Acne treatment (keratolytic and comedolytic effects and Conla( I dermatitis local irritalion or erylhema
Caress cream 2-5ol" kill anaerobic bacteriar.
Apply sparingly 1-3 times daily for I 5 min. May increase
strength and duration of exposure as tolerated.

Benzlropine Mesylate
Cogentin,
lnjection: 1 mg/ml (2 ml).
Tablet: 0.5 mg, 1 mg, 2 mg.
Benzylpenicilloyl-polylysine
Diagnostic agent, penicillin allergy
ski n test.
Pre-Pen.
lnjection: 0.25 mL.
Parkinsonism. anlirhotinergit agenl. clrug indured
extrapyramidal reaction (blocks striatal cholinergic
re( eplL)rsr.
Children > 3 yr: 0.02-0.05 mg/l<g/dose 1-2 times daily
Adjunct to assessing the risk of penicillin hypersensitivity
(elicits type-1 urticarial reactions by lgE-mediated
rcactionl.
Children and a,lulls: Scralch ter hnique uses a 2O-gauge
needles to make a 3-5 mm scratch on dermis, apply a
small drop of solr-ttion to scratch and rub it gently with
applicator. Intradermal injection of 0.1-0.2 ml of Pre-Pen
.rnd 0.q1,, saline in I sile' al leasl I int h apar1.
Tachycardia, drowsiness, nervousness,
hallucinations, dry mouth, blurred vision,
mydriasis.
Monitoring: Scratch test is positive if a pale
wheal of 5-l5 mm or more occurs within
l0 minutec. lntradermal tesl is posilive in
5-1 5 min. Discontinue antihistamines before
performing tesls.

Beractant Prophylaxis and treatrnent of respiratory distress syndrome Bradycardia, hypotension, oxygen desaturation,
Lung surfactant in premature infants (replace deficiency of endogenous putmonary air leaks, airway obstruction,
Survanta. surfactant). pr-rlmonary hemorrhage, hypocarbia.
Suspension: 200 mg (B mL). Neonates: 4 mVkg via endotracheal tube. May repeat every
6 hr up lo a lolal of 4 do.es. Rotale baby to right, then left
and rdmini:ler l/2 dose on eat h side over 2-l sec.

Betamethasone
Betnesol 0.5 mg tab, 4 mg/1 ml amp
Betnelan, Betamesan 0.5 mg tab.
Neocort, Betnovate cream/oint.
Betason-N eye drop and ointment.
Bethanechol
Duvoicl, Myotonachol, Urecholine,
lnjection: 5 mg/mL (1 mL)
Tablet: 5 mg, 10 mg, 25 rng, 50 mg.
Bleomycin
Bleocin, Blenoxane 15 units/amp.
Systemic use to stimulate fetal lung maturation in preterm
labor. Topical use to treat inflammatory dermatoses.
Topical application of thin filrn to affected area
2-4 times daily.
Pregnant female: 12 mg IM q24 hr {or 2 doses.
Cholinergic agent.
Treatment of non-obstructive urinary retention or
gastroesophageal reflux (stimulate cholinergic receptors in
smooth muscle in urinary and gastrointestinal tracts).
0.3-0.6 mg/kg/d divided into 3-4 doses.
Palliative treatment for several cancers and sclerosing
agent for malignant efiusions (inhibits synthesis of DNA).
10-20 units/mr/dose lV, lM, SC (0.25-0.5 units/kg)
I 2 limes per wk in combination regimens.
Maternal pulmonary edema and hypertension.
headache.
Hypotension, abdominal cramps, diarrhea,
vomiting, salivation, urinary frequency,
bronchial constriction, sweating.
lnterstitial pneumonitis, pulmonary fibrosis,
phlebitis, leukopenia, thrombocytopenia,
:tomalilis. vomilinE, alopet ia, hyperkeratosir
of hand and nails, desquamation, Raynaud
phenomenon.

Budesonide Treatment of chronic rhinitis or asthma (suppresses Oral thrush, dysphonia (minimize by rinsing
Rhinocort. inflarnmation). mouth aher doset.
Aerosol: 50 mg released per Children >o yr: \asal 'pray 2 pufk in eath noslril lwit e
actuation to deliver (200 metered daily or 4 puffs in each nostril once daily
doses). Children >6 yr:1-2 inhalations bid.
Pulmicort turbuhaler
lnhalation powder 200 pg/inhalation
Busulfan Treatment of chronic myelocytic leukemia or as pan Severe pancytopenia, leukoPenia,
M),leran, 2 mg tab. of marrow ablation conditioning prior to bone melrrow thrombocytopenia, and bone marrow
transplant (interferes with DNA alkylation). suppression (onset 7-10 days, nadir 14-21
For CML remission: 0.06-0.12 mg/kg once daily, titrate days, recovery 2B days).
dose to keep Ieukocyte count >40,000/mmr.

Caffeine Treatnrent of apnea of prematr-rrity (stimulate central Tachycardia, agitation, i rritabi lity, gastric
Caffeine citrate inspiratory drive and sensitivity to carbon dioxide). irritation
Tablet 65 mg (anhydrous Neonates: Oral lcitrate or benzoate), lV (benzoate), Dose
caffeine 32.5 mg). as caffeine base: Ioading dose I0 mg/kg. Maintenance
dose: 5-1 0 mg/kgcl as I or 2 doses/d.
 a

ESSENCE OF PEDIATRICS

Calcitriol Treatment of hypocalcemia and metabolic bohe.disease, Hypercalcemia, vit D toxicity,

Vitamin D analog; reduce elevated parathyroid horinone levels (regulate
1,25 dihydroxy-choleca Ici ferol seru m cal c'ium. homeostai s and inerease ca lci u nr
i

Rocaltrol, absorption).
Dicaltrol O.25 pg,0.50 pgicap. ,Plemature infants (hypocalcemia):' 0,05 p#k#d lV of
I prg/d orally.
Vitamin D deficieniy rickets: 0.5-2 pg 1, 2S-dihydroxy
cholecalciferol (si ngle dose).
Vitam in D resistani riakets (Fam ilial hypophosphatemic) :
:5V65 ngl.kgl24 hr along with oral phosphate (oulie l
sol ulion).

G cium'la{lE loral:and lV} Hypocalcemic tetany, cardiac distrubances of Constipation, hypercalcemia, mi lk alkali
Calcium Carbonate-Calsan 250 mg hyperkalemia. syndrome.
.t$b;,C6194.oq,u.al I 2s0 mg tab i
Hypocalcemic tetany: Neonafes**l a mEq{k{c} in divjded
(500 mg elemenlal calcium). mg of
doses (if, due to citrated.blood transfusion give 0.45 mEq
Calcium lactate-Calcital, .l
per 00 mL lransfused lrloqd)^ lnfants and chiJdren,-I1 Salt ca'.risalt - T!
La'"/gsalt,,
Caltate 300 mg tab. l0olo mglkg over 5:10 min (may repeat in 6-8 hr) followed by telem"entalr
C-calcium gluconate, infuiion withrmax dose of 200 mg/kg&: . : Ca carbonate mg400 20 mEq
107o calcium f ayson 5 ml, Cardidc arrest: lnfants and ihildren, 20 mglkg lV and rnay
10 ml amp. repeat in l0 min.
Ca gluconatemg 90 4.5 mEq
Ca lectate t:O mg 6.5 mEq
Captopril Management oi hypertension and treatment of heart Cough, angioedema, oliguria, hyperkalemia.
Cardopril, Acetor 25 mg, 50 mg tab. failure (ACE inhibitorr.
Antihyperlensive agents: Neo-ndle5: hitial 0.05-0.1 mglkg/dose every 8.24 hr and
1. Diuretics titraG upward to response (max dose 0'5 mglkg/dose every
i.r.j:,, r,4),:riThlazides.hlrdr:ochlor thiazide. 6 -24 hr\.
,:,...,',:.b},,.LOop diuretics.Frusemi.de . lnfants: Initial 0.'15-0.3 mg/kg/dose; and ritrate upward
2. Sympatholytic drugs (max 6 mglkg/d in 1-4 divided doses),- '
a). B-idrenergic blocking- Children: lnitial 0-3-{.5 mglkg/dose and titrate upward
propranolol (max 6 mg/kg/d divided into 2*4 doses).
b). c-adrenergic blocking-
prazosi n
3. Vasodilators
a). Arteria l-hydralazine
b). Arteriovenous-nitroprusside
4. Ca channel blot ker: niiedipine,
diltiazem.
5. ACE inhibitors: captopril,
enalapri I
6. Angiotensin receptor antagonist:
Losa rtan

Carbamazepine tr*u,*6n1. gf .g€neralized tonic-clonic a-nd partial seizures,
Car.bazine;,.Tegrelol 200 rng 14[, pain relief in trigeminal neuralgia.
100 mg/5 ml susp. Children: < 6yr initial 5 mlkld in 2*4 divided doses;
may ir.rerease eVery 5-7 days by 5 mg/kg, based on effect
or loxicity and serum concentralion.
6-1 ? yr:: Initial 1 0 m#kgid in 2:*4 divided-doses;
Sedation, ataxia, blurred vision; bone
marrow depression. leukopenia, neutropenia,
throixbocytopenla, pancytopenia, aplastic
anemia, hepatitis, hy.persensitivjty reactions,
erythema multiforme
Monitoring: Serum concentrations correlate .

incr.eqse by 100,n'lg or 5 mglkg/d at week,ly ihtervals until with clinical response (6-12 pglml), and
therapeutic le-vels are achieved (usual dose800-1200 , neurologic and visual toxicity
mg/d). t>8 pg/mL bul particularly > l2 prg/mL).
Cetirizine Child over 6 yf: 10 m/d bd; Same as.chlorpheniramine but sedation and
Cetrin, Alatrol 10 mg tab; 2-6 yrsr5 mg/d anl imascarinic effects low.
5 mg/5 ml syp.
Chloral hydrate Shortterm sedative/hypnotic {mechanism unkriown)^ Cl disturbances. drowsiness, dizziness, ataxia,
Chtoral hydrate 500 mg/5 ml and Neonates: 25 mg/kgldose. headache, delirium.
200 mg/5 ml susp, 500 mg cap. lnfants and children: 25-100 mg/kg/dose.
Doses may be repeated every b B hr. 1
Lower-end doses cause sedation, higher-
end doses cause hypnosis. I
t
1

Chlorambucil
Antineoplastic alkylating agent,
Leukeran
2 mg lablel.
Chlorpheniramine Maleate
Sinamin, Histal 4 m8 tab;
2 mg/5 ml susp.
Antihistamines: H,-receptor
antagonisls
Sedative: promelhazine,
diphenhydrami ne-chlorpheniram ine
Less sedalive: Cetirizine, loratadine
Hr-receptor antagonists: Ranitidine,
Famotidine.
Chlorpromaziire (Phenothiazine)
Largactil, Opsonil 25 mg. 50 m&
I00 mg tab; 5O m{2 ml amp;
25 mg/5 ml syp.
Chorionic gonadotropin
Conadotropin, ovulation slimulator.
Pregnyl 5000 lUiamp.
Cisplatin
Platosin 10 mg powder in 10 ml vial.
Clonazepam
Rivoril, Lonazep 0.5 mg, 2 mg tab.
Clonidine
Alpha-adrenergic agonist
Catares Tablet 0.1, 0.2, 0.3 mg.
Codeiire
Narcotic analgesic
Codeine phosphate 1 5, 30, b0 mg
I
tab; 3 mg/5 ml susp.
DRUG THERAPY IN CHILDREN
Management of various cancers including Hodgkin and Bone.marrow suppression (onset I days, ,
non-Hodgkin lymphoma and CLL; & nephrotic syndrome nadir 10*14 days, recovery 28 days); skin
ialkylation interferes wilh DNA replication and RNA ras hes,. hyperu r cern ia, vom iti n g, d i d-rrhea, ora I
j
transcriptionl. ulceration. pulmona ry fibrosis, hepatic necrosis,
0. l-0.2 mg/kg/d for 3-6 wk. periphera I neuropathy.
Treat allergic symptoms (competes with histamine for Drowsiness, excitation or hyperactivity, dry
H,-receptor sites) mouth, blurred vision.
Children; 1-2 mg every 4-6 hr or sustained-telease B mg
al bedtime.
Treatrnent of psychosis, mania, Toureite syndrome,. Hypotension, tachycardia, anhythmias,
behavioral problems, nausea and vomiting{blocks pseudoparkinsonism, tardive dyskines a, i
poslsynapti( mesolimbic dopaminergic receplors in dystonias, nasal conBestion, dry mouth,
the brain, strong alpha-adrenergic blocking effect). malignant hyperpyrexia.
Children > 6 mo:
Oral, 0.5-l mg/kg/dose every 4-6 hr;
lM or IV, 0.5*1 mglkg/do5e every 6-8 hi
Treatment o{ hypogonadotropic hypogonadism; Mental depression, precocious puberty,
cryptorchidism, induce ovulation {stimulates produclion premature closure of the epiphyses.,
of gonadal steroid hormones, substitute for LH to stimulate
ovlrlalronl.
Prepubertal cryptorchidism:
1000 2000 units/m /dose 3 times/wk for 3 wk or
500 units 3 times/wk for 4-6 wk.
Hypogonadotropic hypogonadism:
500- 000 unitsldose 3 times per wk for 3 wk; or
1
4000 units 3 times/wk for 6-9 mo, then taper'to
2000 unils 3 limes weekly for 3 mo.
Treatment of multiple tumor types (inhibit DNA synthesis). Vomiting (lasts up to 1 wk postdose),
37-75 m{m2/once every 2*3 wk or 50-120 mglm2 once myelosuppression (onset 10 days, nadir 14=23.
every 21 -28 days (admin ister over 4-6 hr) days, recovery 21-39 days), acute renal {ailurd,
chronie nephropathy, peripheral nei;ropathy , :
(irr.everr.ible), ototoxicjty, extravasation injqry,,
elevated liver enzymes, alopecia, optic neuritis,
arrhythmias.
Prophylaxis of seizure types: Absence, Lennox-Castaut, Tachycardia, drowsiness, impaired memory and
akinelic. myoclonic tdepresses nerve transmission in the co-ordination, blurred vision, hypersalivation,'.
motor corlexl. bronchial hypersecretion, respiratory
0.01*0.03 mg/kg/d in 2-3 divided doses depression, physical and psychological
tmax 0.05 mg/kg/dr. dependence.
Treatment of hypertension; attention deficit clisorder Drowsiness, dry mouth, constipation,
(ADD); aid in diagnosis of pheochromocytoma and growth hypotension.
hormone def iciency (stimu Iates alpha-2 adrenoreceptors
in the brain slemr.
ADD: lnilial 0.05 mg/d, increase every 3-Z days by
0.05 mgld given in J-4 divided doses to response
(max 0.4 mdd).
Hypertension: 5-10 pg/kg/d in 2-4 divided doses
(max 0.9 p{kg/d).
Treatment of mild to moderate pain and cough (inhibition Drowsiness, constipation, anorexia, vomitingt
of ascending pain pathways; central action in medulla to sedalion.
suppress cough;.
Pain: 0.5-1 mglkgldose every 4-6 hr (max 60 mg/dose)
Cough: 1-1 .5 mg/kgid divided every 4-6 hr.

ESSENCE OF PEDIATRICS
Colfosceril Palmitate
Lung surfactant
Exosu rf.
lntratracheal suspension,
108 mg/1 0 mL.
Corticotropin, ACTH
Adrenal corticosteroid.
Acthar.
lnjection, repository: 40, B0 units/mL.
Tablet: 5, 10, 25 mg.
Cortisone
Acetate adrena I corticosteroid.
Corlone.
lnjection: 50 mg/mL.
Tablet: 5, 10, 25 hg.
Cromolyn Sodium
Mast cell stabilizer
Sodium chromoglycate: lntal
Nacromin 5, 5 mg/Puff.
Nedocromil sodium: Tilade
2 mg/puff .
Cusicrom eyedrops,
Nacromin nasal soln.
Crotamiton
Scabicidal
Eurax, Cordex
Cream: 107o
Lotion: l0o/o
Cyanocobalamin
Vitamin B,,
Cynomin,VitaminBrlmg
{1000 pg)/l ml amp.
eyilophoryhamide
Endoxan-Asta 50 mg tab;
200 mg/vial
Cyclosporine
Sandimmune, Neoral, 25 mg,
100 mg cap,
i!+*+€!]ilE!rF-!
i
Neoriatal respiiatory distress syndrome (replaces deficient Pulmonary hemorrhage, overventilation
surfactani, Iowers surface tension at air-fluid interface in lcausing hyperoxia and hypocarbiar, PDA
alveolir.
Neonates: 5 ml/kg/dose as prophylaxis or rescue therapy
for:RDS (max 4 doses although no proven beriefit foi >2
doses).
lnfantile spasnrs; diagnostic agent in adrenocortical lnsomnia, increased appbtite, diabetes mellitus,
i nsuf f ic iency. (3ti rn u afes ad renal cortex to rel ease adrena I
I epistaxis, pancreatitis, muscle wasting, bone
steroidS/ an.drogenic substances and a small amount of growth suppression, opportunistic infections.
a ldosterone).
Children:
tnflammation or immunosuppression: lV, lM, SC
(aqueous): 1.6 units/kg/d or 50 units,lm: divlded q 6*8 hr;
lM {gel): 0.8 units/kg/d divided every 12*24 hr.
tnfantile spasms: 5-1 60 units/kg/d has been used for
1 wk*12 mo as lM gel (prednisone 2 n{kg/d has equal
efficacy).
Management oi adienocortiial insufficiency lnsomnia, pseudotumor cerebri, increased
treplacement). appetite, peptic ulcer, diabetes mellitus, edema,
Children: Oral-0.5*0.7 ng/k{d divided every B hr. hypertension, cataract/ glaucoma, hypokalemia.
lM-0.25-0.35 mg/kg once daily.
Prevention of persistent symptoms of asthrna, rhinitis, Hoarseness and coughing (mainly with powder
conjunctivilis, food allergy (prevents mast cell release of for inhalationr. burning at administralion site.
5, histamine and leukolrienest.
Asthma: I-2 puffs {MDl) or 2 ml (nebulizer solulionl
3-4 times daily.
Rhinitis: 1 spray each nostril 3-4 times daily.
Conjunctivitis: 1-2 drops 4-6 times daily
Food allergy: 100 mg/dose 4 rimes daily (max 40 mg/kg/d).
Treatment of scabies (mechanism unknown). Wash area Local irritation
thoroughly, towel dry, apply a thin layer and massage
drug into skin. Repeat application in 24 hr; take a
cleansing bath 48 hr after final application. May repeat in
:",:[ IT:::'a, vitamin B,, dericiency (coenzyme ror Neuropsychiatric problems (rare).
various metabolic functionsi
Pernicious anemia: 30-50 pgld ,, i^
to +^+-r r^.^ 1ft^n
total dose tr^^^
1000,5000
619 then follow with 100 pg mo.
Vitamin 8,, de{iciency: 0O pgid for 1 0-.1 5 days then once
I
or twice a wk for several mo.
Managemenl of various cancers including Hodgkin Cautions: Maintain high fluid intake to
disease, malignant lymphomas, nephrotic syndrome; avoid hemorrhagic cystitis and consider
Sl-F, rheumatoid arthritis (interferes with normal function administration of mesna.
of DNA by alkylationr. Cardioloxicity with high doses, pericardial
For treatment oi Iymphoma effusion, CCF, alopecia. vomiting, stomatitis,
SLE: 500-700 mg/m: every mo. JRA: lV 10 mglkg every hemorrhagic c)'slilis, leukopenia ronset 7
2 wk. days, nadir B- I5 days, recovery 21 days),
Nephrotic syndrome; Oral 2-3 mC/kC/d (when steroids thrombocytopenia, hepatotoxicity, renal
fail, use for up 1o l2 wki. toxicity. secondary malignancy.
lmmunosuppressant used in Aplastic anemia and to
Hypertension, hirsutism, tremor, nephrotoxicity, E
prevent graft versus host disease in organ transplantation gingival hypertrophy, leg cramps, CI
rinhibits production and release of interleukin ll and
distomforl, seizure. :
activation of resting T-lymphocytes by interleukin ll). ?
1O mg/kg/d in aplastic anemia for - 6 mo.
\
\
\
1
 DRUG THERAPY IN CHILDREN

Cytarabine HCl, Ara-C Used in combination therapy to treat leukemias and Fever; rash, oiallanal rilceration, Cl'up$elr
Cytosar-U tpowder for injection: 0.1, lymphomas {inhibits DNA polyrnerase to inhibit DNA mucositis, Iiver dysftinction, Lrleeding, , r" '

O.5, 1, 2 g). Tarabine PFS. sy nthes i s). myelosuppregsion (anset,4-7 daysr,nadir i

lnjection: 20 mg/mL. Typical dose: 1 4*1 B days, rqcoyely 2 -28 da;,s); alopeeia;
1

lnduction: lV 100-200 mg/m:/d for 5-1 0 days or until con junctivitis, dizziness, headache;.neur,itis,
remission.
Maintenance: lV 70-200 mg/mld for 2-5 days at monthly
intervals; lM, 5C 1-1 .5 mg/kg single dose at
1 to 4 wk inlervals.
lT: 5*75 mg/m': every 2*7 days until CNS findings
'
normalize.
Pain and burning at infusion site, vomitin&
l

Dacarbazine Treatment of iarious tilmors (alkylating agent and possiblyi

Antineoplastic agent some antimetabolite activitY). leukopenia (onset T days, nadir I 0*1 4 days, '

DTIC-Dome.
Injection: 100, 200, 500 mg
Dactinomycin, Actinomycin D
Anlineoplastic agent.
Cosmegen.
Powder {or lnjection 0.5 mg vial
Solid tumorsr 2OV47O mf,m2ld civer'S days every
21*28 days, neuroblastoma; 800*900 mg1m': cin day 1 of ':
combination therapy every 3*4 wk.
Hodgkin disease: 375 mg/m: on days 1 and 15 of
combination treatmenu repeat every 28.days.
Treatment of various tumor types ibinds to guanine portion
of DNA blocking replication and trariscription of the DNA
lemplate).
Children >6 mo: 15 Uflkg/d or 400-600 pg/m:/d for
5 days; may repeatevery 3*6 wk.
recovery 21*28 days).
Polyr"ieuropathy, .elevated liver enzymes, ..
alopec'ia.
Myelosuppression {onset 7 days, nadit 14-21
i days, fdcovery 21-28 days), fgver, aleipecia;
skin eruptions, acne, GI upset, muaositisr ..
stomatitis, hypocalcemia, hyperuricemia.

Dantrolene Treatment of spastici{ associated with upper motor Drowsiness, blurred vision, seizures, Cl upset,
Sodium neuron disorders, such as spindl cord injury, stroke, pleural effusion with pericarditis, hepatitis. r

Skeletal must le relaxanl
f)antrirtm.
Capsule: 25. 50, I 00 mg
Powder for lnjection: 20 mg vial
cerebral palsy (interferes with release of calcium ion from
the sarcoplasmic reticulum).
Spasticity: 0.5 mg/kg/dose twice daily, increase frequency
every 4-7 days to 3-4 times daily, then increase dose by 0.5
mgikg to max 3 mg/kg/dose 2-4 times daily'

Daunorubicin Hydrochloride Treatment oi ANLL and myeloblastic leukemia {inhibition Alopecia, red discoloration of urine,'Gl upset,
Antineoplastic o{ DNA and RNA synthesis). stomatitis, myelosuppression (onset ': i

Daunoblastin 20 mg, vial. Remission induction for AI-L (combination therapy): 7 days, nadir 14 days, recovery 21-.2.8.
25-45 mg'm'zon day 1 every wk for 4 cycles days), extiavasation, related tiisueL uleerdtion. ,r'

(max total 300 mg/m'Z). and netiosis, congeitive heart.failute;.' ' l:.
f
hyperuricemid,hepatotoxicity.' -' ii

Deferriprone Mobilizes iron from lransferrin, ferritin, hemosiderin, C I symptoms, arthropathy (25%), neiltiopenia,

Kelfer 500 mB cdp. 50- I 00 mg/kg/d thrombocytopenia

Deferoxamine Mesylate Treatment of acute iron intoxication Qr.secondary chronic Loca,l pain and induration, flushing; : '.. .,,,, .,'

Chelating agenl iron overload (i.e., thalassemia); (forms complex with iron hypotension, hearing loss, blurred yiq!.o1, .,;'..

Desferal to form ferrioxamine, which is removed by kidneys). cataracts.

Powder for lnjection: 500 mg/vial. Acute iron intoxication: lM 90 mg/k$dose every 8 hr. lV
l5 mg/k{hr rmax 6 g/d).
Chronic iron overload (i.e., thalassemia): lV 15 mg/kg/hr
(max 12 g/d). 5C 20-40 mg/kg/d ovef B-12 hr via portable
infusion device.
Desmopressin Acetate Treatment of diabetes insipidus, control of bleeding in Faci al flushing, headache, dizzi ness, increased
Vasopressin analog. certain types of hemophilia, primary nocturnal enuresis blood pressure, water intoxication.
DDAVP, Stimate (enhances reabsorption of water in the kidneys, dose
lnjection: 4 m{mL. dependent increase in factor Vlll and plasminogen
Nasal solution: 0.1 mlml dctivator).
Diabetes insipidus:
3 mo-12 yr: Oral 0.05 pg initially then titrate to response.
lV: 5 pgld in 1-2 doses
Hemophilia:
>3 mo, lV 0.3 p{kg, may repeat dose if needed,
use 30 minutes before procedure.

( Nocturnal enuresis:
>6 yr, 2A pg at bedtime.
r
r'

ESSENCE OF PEDIATRICS
Dexameihasone
Oradexon, Decason
0.5 mg tab. 5 mg/1 ml amp
Ophthalmic ointment: 0.05%
Ophthalmic suspension: 0. 1, 0.5olo.
Dextran 40
(low mol wl),
70 thigh mol wt)
Macrodex 70: Dextran 70 in
DA, Saline
Rheumacrodex 40: Dertran 40 in 5ol.
DA, Saline
Dextroamphetamine
CNS stimulanl
Dexedrine.
Tablet: 5, l0 mg
Sustained-release capsule:
5, 10, 15 mg.
Dextromethorphan
Antitussive
Dephar, D-cough 10 mg/5 ml susp.
Diazepam
Sedil, seduxen 5 mg tab;
10 mg/2 ml amp,
Easium l0 mg suppository.
Diazoxide
Ant ihypertensive
Eudemine, Hyperstat,
Proglycem 50 mg/cap;
15 mg/ml amp; 50 mglml susp.
b"
'l
Systemically and locally for acute and chronic Insomnia, increased appetite, hypertension,
inflamniation; allergic, neoplastic, and autoimmune hyperglycemia, Cl hyperacidity, cataraiq
diseases, ceretrral edema, septic shock, H. in{luenzae adrenal suppression, poor growth,
meningitis, diagnostic agent (decreases inflammation and
suppresses normal immu ne responsei.
Anti-inflammatory: oral, tM, lV 0.0S-0.3 rng/kg/d divided
every 6-1 2 hr.
Bacterial meningitis: lV 0.a mg/kgidose dvery 12 hr
for 4B hr. or 0.1 5 nrglkg/dose every 6 hr for 48 hr.
Cerebral edema: Oral. lM, lV: loading dose 1'-2 mg/kg,
then 1*1 .5 mg/kgid divided every 4-6 hr.
Ophthalmic: Ointment, apply every 3-4 hr to conjunctival
sacas.thin coating; suspension: instill 2 drops into
ccnjunctival sac every hour during day and every other
,
hour at night..Cradually taper doses when inflammation
resolves.
Topical: Apply 1-a times daily.
Blood Volirme expander in shock or impending shock Pulmonary edema, bleeding due to impaired
(similar to albumin). Max = 20 mUkg on clay .l then platelet function.
10 mUkg/d for not >5 days.
5"/o,
Treatment of attention de{icit d.isorder and exogenous Hypertension, palpitations, arrhythm ias,
obesity (blocks reuptake ol dopamine and norepinephrine insomnia, agitation, irritability, depression,
from the syndpsel tremor, exacerbation of tics and movemenl
Children 6-12 yr: disorders, mydriasis, physical and psychological
Attention deficit disorder: lnitial 5 m{tJ, may increase by dependence, Cl upset, growth suppression.
5 mld at weekly intervals to response {rnax 60 mgld).
>i2yrchildren:
lnitial 2O mgld, may increase at 10 mg increments weekly
(max 60 mg/di.
:
\
Symptomatic relief of coughs; best when cough is Drowsiness, respiraiory depression, blurred
nonproductive (depresses the medullaiy cough centre), vision, Cl upsel, conslipation.
Children 24 yr: 2.5-7.5 mg every 4,8 hr.
Children >6 yr: 10-30 mg every 4-B hr.
Treatment of anxiety, panic disorders. status epilepticus, Hypotension, bradycardia, cardiac arrest
provide gedation and skeletal muscle relaxation ithought rwith lV dosei, drowsiness, ataxia, confusion.
to increase neuroinhibitory action of CABA). Prevent impaired co-ordination, paradoxical
febrile seizure. excitement, amnes ia, bl urred vision, diplopia,
Stdtus epilepticus: sweating, dry mouth, increased or decreased
lV-0.05-0.3 mflkg/dose given over 2-3 min, may repear appetite. physical and psychological
every 30 min to max total dose of 5-10 mg. dependence.
Rectal-0.5 mg&g. then 0.25 mlkg in 10 min if needed.
Sedation: Oral-0.2*0.3 mg/kg (max t 0 mg); tM/tV-
0.04*0.3 mglkg {max 0.6 mg/kg/B hr).
Tetanus: 0.1*0.2 mg/kgidose lV every 3*6 hr (then titrate).
Emergency lowering of BP, treatment of hyperinsulinemic Hypotension, dizziness, weakness.
hypoglycemia related to islet cell tumors (smooth muscle
relaxation, inhibits insulin release from the pantreasl.
Hypertension:
Children: 1-3 m{kg, may repeat in 5-1 5 min, dose every
4-24 hr.
Hyperinsul inem ic hypoglycemia:
Newborns and infants: Oral 8-t 5 n{k{d divided every I
& 12 hr (start on low endt.
Children: Oral 3-8 n{k{ddivided every 8-1 2 hr
{start on low end). \
I
tr
l
r
I
DRUG THERAPY IN CHILDREN

F
Dibucaine Temporary relief of pain and itching due to hernonhoids Local, irritation;. eontact dermatitis;
Local anesthetic, and minor skin irritalion (block initiation and conduction
N uperca ina l, of nerve impulsest.
Cream: 0.57o Topical: Apply gently to affected area 17:5 #d),
''
Ointment: lolo. Rectal: lnsert wilh rectal applicator morning, evening, and
after each howel movemenl.

Diclofenac sodium Tieatrrent of mild to moderate acute.or'chronic pain , Fl,uid retentiot;'abdorninal pain, peplic uleer; '

Gl bleedingi renai impa'irment' ' : '

'
Clofenac, Ficlon, Vollaren 25 mg, (inhibits proslaglandin synlhesis).
50 mg tab; 75 m{3 ml amp; 2*3 mgikgld in 2*4 divided doses.
i
12.5 & 50 mg suppository.
Dicyclomine Treatment of {unctionai disturbances of Cl moti}ity, I
'
Tachycardia, palpitations, irrirability, muscle
Anticholinergic agent
:ir
e.g., irritable bowel syndro-rne (block the actionsiof 'r"' hy,potonia; bluiied lvhion; photophobia; ur|nai
Cyclomin l0 mg tab, 20 mg cap, acetylcholinel. retcfiion; constipation; dry mouth, urticarla;
10 mgl5 ml susp, 10 mg/0.5 ml lnfants >5 ms:.'Ordl 5 mgldose 3'4'ti'mes'dailry;. :
',i
drops. Children: 1 0 mg 3-4 times/d, i, . , .

Digoxin Treatment of congestive heart failure and supraventricular Caulions: Contraindicated in A-V block or
Cardiac glycoside. tachyanhythmias (increaies intracell'ular calEium thrsqgh conslrictive pericarditis.
Digoxin, Lanoxin 0.25 mg tab; inhibition of sodium/potassiuln' ATPase pump; suppressi on Anorexia, nausea/ vomitin.g; diarrhea;. 1.,',:. 1,.r.,r. .1.

0.5 mflz ml amp. of A-V node conduction). bradycardia, arrhythmias, blurred visiori,',r:','. .
Digitalization PO (l/2 of TDD initially, diplopia, photophobia, yellow or green vision.
followed by % of TDD every B-12 hr x 2 doses.) Check FCG; selum electr,olytes, ealciuriland
Dose: magnesium. Check hea( rate: . . .. , ' I
Premature: 0;02 mglkg {TDD)
Neonate: 0.02-0.03 mg/kg tTDDt
Infant & Childr 0.03-0.0a m#kg{TDD)
lV dose is 75% of PO dose
Maintenance: 0.005-0.01 mg/kg/d div q 12 hr.
lV dose is 75% o{ PO dose
Dihydrotachysterol Treatment of hypocalcemia associated with Hypercalcemia, hyperearliiuria, elevated:.serum
Vitamin D analog hypoparathyroidism and renal osteodystrophy (stimulates creatini'ne,. ...

Hytakerol calciurrr and phosphate intestinal absorptioir): '

Capsule: 0.125 mg Neonates: 0.05 0. I mg/d.
Tablet:0.125 mg lnfants and young children: 1-5 mg/d fdr 4 days then
Solution: 0.2 mg/ml. 0.2 mg/5 mL. 0.5-l .5 mg/d.
Otder children; 0-75-2.5 mg/d for 4 days,
then 0.2 I mg/d (max 1.5 mg/dr.
Diltiazem Renal osteodystrophy: 0. l-0.6 mg/d. Hypotensi on, b,tadycardi a, edema; A=V Lrlock,
Calcium channel blocker Treatment of hypertension and atrial tachyarr:hythrnias dizziheis-' . .

Cardizem, Diltizem 30 mg, (inhibits calcium ions from entering the slow channels
60 mg lab. during depolarization).
Children: Oral 1.5-2 mglkg/d in 3-4 divided doses
Adolescents: Oral 90-480 mgid in 3-4 divided doses.
Dimercaprol Antidote to gold; arsenic, and mercury po'issninS,rand Hypeitension, tachycardia, convulsions; fe$4
BAL adjunct to edetate calcium disodium in'lead poisoninf heaelache, nephrotoxicity,'
lnjection: 100 mg/mL. (chelates with heavy metals to form nonioiic stable'
compounds). ... ':l

Children:
Mild arsenic and gold poisoning: 2.5 mg/kg/dose lM every
6 hr for 2 days, then every 1 2 hr on day 3; then eveiy
24 hr {or 1 0 days.
Severe arsenic or gold poisoning: 3 mg/kg/dose every
4 hr for ) days, then every 6 hr on day 3, then every
I2hrforl0days.
Lead poisoning:
Mild: a mg/kg load, then 3 mg/kfdose every 4 hr for
2-7 days.
Severe: 4 mg/kfldose every 4 hr for 2-7 days.
r Diphenhydramine Antihistamine {competitive inh ibitor of H,-receptor). Hypotension; taehycardia, diowsineqq;''1..' .,;...

Pedeamin, Phenadryl l0 mg/5 ml 5 m$kg/d divided every 6 hr as needed paradoxical excitement; th ckehed bion.ch i al
i

syp. lmax 300 mg/d). secretions,dry.mo!th.'.'

ESSENCE OF PEDIATRICS

Dobutamine Treatment of hypotension (stimulates beta-l adrenergic Tachycardia, ectopic heaft beats, angina,
Dobutrex: 12.5 mg/ml vial. receptors). palpitations, tachyarrhythmias, paresthesias, leg
Neona tes: 2-2O 1tg/k{ min cramps.
Children: 2.5.4.0 yglkg/min constant infusion.
Domperidone A prokinetic agent with anti-errietic property, used in Hyperprolactinemia lmay result in galactorrhea,
Motigut, Cosy, 5 mg/5 ml susp. vomiting ef various origin, in gastroesophageal reflux. breast enlargement, reduced libido), dry mouth,
5 mg/ml drop. l0 mg tab. 0.2-0.4 nrg/kg every 4-B hr daily 30 minutes before meal. thirst, headache, nervousness, drowsiness,
diarrhea, skin rash. Extrapyram i dal reactions
rdre t0.0570).
Dopamine iprecursor of Treatrnent of hypotension and shock (stimulates Tachycardia, ectopic beats, ventricular
noradrena I ine; arrhythmias, tissue necrosis with extravasation,
Depamin 40 m{ml in 5 ml amp. vasoconstriction, gangrene of extremities,
i::,ilr-it:i'ffi f-;il::fr sf ffi *i,ri:*" excess urine output (doses <5 pgikginrin),
For ionotropic & vasodilatory actlcns, 1-5 pg/kg/min is .. oliguria rdoses >10 y.t{k{min.
used, higher doses. may cause vasoconstiiction.
Doxapram Treatment of apnea of prernaturity refractory to Hypertension/ anhythmias, CNS stimulation,
Dopram. methylxanthines irespiratory and CNS stim ulant). irritability, seizures, hyperpyrexia.
Injection: 20 mg/ml. Neonates: lnitial 2.5-3 mg/kg {ollowed by
infusion of 1 mg/kg/hr {maximum 2.5 mglkg/hr).
Doxorubicin Hydrochloride Antineoplastic used for various tumor types (inhibits DNA Cardiotoxicity, alopecia, hyperpigmentation of
Adriamycin, 1 0 nrg/vial and RNA synthesis). nail bed, hyperuricemia, stomdtitis, esophagitis,
35-75 m{m'/dose, repeat every 21 days; mucositis, vomiting, thrombocytopenia (onset 7
or 20*30 mg/m2, repeat every wk. days, nadir 1 0-1 4 days, recovery 2 I -28 days).
extravasation, tissue necrosis, phlebitis.
D.Xylose Diagnostic agent used to evaluate intestinal disorders due Nausea. vomiting crampi ng, i ntestinal bloati ng.
Xylo-pfan to d'isease orlnjr-lry (mechanism not understood).
Powder for oral solution. 500 mg/kg as 5-1 0% sofution, max 25 g.
Edetate Calcium Disodium Antidote for acute and chronic lead poisoning Arrhythmias, hypotension, seizures, skin
Calcium Disodium tchelating agentr. eruptions, hypomagnesemia, hypokalemia,
Versenate- 500 mglm'?ldose once daily. hypocalcemia, hyperuricemia. Cl upset, muscle
lnjection: 200 mg/ml. cramps, paresthesia, Letany, nephrotoxicity,
respiratory arrest.
fdiophonium Chloride Diagnosis of myasthenia gravis, differentiation of Arrhythmias, hypotension, nausea, Cl upset,
Reversol, Tensilon. cholinergic crisis from myasthenia crisis, iinhibits excess sweating, uri nary frequency, diplopia,
lnjection; 10 mg/ml. destruction of acetylcholi ne by acetylcholinesterase). miosis, laryngospasm. bronchospasm,
lnfants: lM 0.5-1 mg, lV 0.1 mg followed by 0.4 mg respiratory para lysis.
tif no rcsponset.
Children: diagnosis (initial):
lM: <34 kg: 1 mg, >34 kg: 5 mg.
lV: 0.04 mglkg over 1 min followed by 0.16 mg/kg given
within 45 sec (if no response), max dose 10 mg total.
Enalapril Treatrnent of hypertension and congestive heart failure Hypotension, tachycardia, syncope, headache,
Anapril, Minipril 5 mg l0 mg tab. (angiotensin-converting enzyme inhibition). cough, hyperkalemia, hypoglycemia.
Neonate:
Oral: 0.1 mglk$d in 1-2 doses {may increase to
O.A m{kgld for congestive heart failure or adequate
hypertension response).
lnfanls and children:
Oral: 0.1-0.5 m{k{d in 1-2 doses.
Adolescent:
Oral: 2.5-5 mgld and titrate to max 40 mg/d in
2 doses.
Epinephrine Trealment of cardia< arrest, bronchospasm, anaphylaclic
:
Tachycardia, hypertension, nervousness, rf
Adrdiralin, Adrin, 1 mg/ml amp. reactions, (stimulates alpha, beta-1, and beta-2 receptors). restlessness, irritability, headache, tremor,
il :1000). Neonates: lV 0.01-0.03 mgikg (0.1-0,3 mVkg of vomiting, acute urinary retention.
!
1;10,000 solution) every 3-5 min.
lnfants and children: SC 0.01 mg/kg (0.01 mUkg/dose o{ \
1 :'l 000 solution, or 0.005 mUkg/dose of suspension).

lV 0.01 mg/kg (0.1 mVkg of 1:10,000 solution)

(max.l mg).
Continuous infusion: 0.1-1 pg/kfmin per response.
I
t"

DRUG THERAPY IN CHILDREN

Erythropoietin Anemia associated with prematurity, end-stage renal Hypertension, edema, headache, fever, rash,
Lpoetin Alfa, disease (induces erythropoiesis). arthralgia, hypersensit ivity.
Epogen. Administer lV, SC.
.l
ln jection: lnj Recormon. 1 00 500 units/kg/dose every l -2 days for 0-21 days in
neonale>.

Ergocalciferol (calciferol, vit D,) Treatmenl of relraclory rickets. hypophosphatemia. Hypercalcemia, hypertension, arrhythmias,
Calciferol, Tablet, capsule: hypoparathyroidism (sti mulates calci um and phosphate vomiti ng, constipation, nephrocalcinosis,
50,000 units. absorplion t. photophobia.
lnjection: 500,000 units/mL Ricket:: 75 125 pgid
r I lrg - 40 unitc) Renal failure: 100-1000 pg/d
Hypoparathyroidism: I .25-5 mg/d.
Ergotamine Prevent or aborr vascular headaches, e.g., migraine or Tachypnea, vasospasmi bradycardia, vomitinS,
Cafergot. Migrin cluster heaclache (ergot alkaloid alpha adrenergic blocker)' diarrhea, Ieg cramps, muscle weakness,
Tablet: I mg. Older children: 1 mg SL or oral at onset of attack and paresl hesi a s.

every 30 min to relief (max 3 mg per attack).

Ethosuximide For rredtment o[ ab.encc-, myocloni, . and akinetic Seclation, vomiting, anorexia, abdominal
larontin. 250 mg rap; epilepsy. pain, leukopenia, thrombocytopenia, aplastic
250 mg/5 ml su:p. -6 ) r: Start | 5 mg/k8/d in 2 doses ; ine rease every a nemia.
4-7 days to therapeutic level, usually 15-40 mg/l<g/d in 2

doses. Mar 1.5 9d.

>6 yr: Start 250 mg twice daily; increase by 250 mg/d
every 4-7 days up to therapeutic level or 1.5 pg/d.

Etoposide For treatment of various cancers (inhibits mitotic activity).
Eposin 20 mg/ml vial IV 150 m/m']ld for 3 days for 2-3 cycles for AML
remission; 150 mglm'/d for 4 days for BMT conditioning.
Hypotension, tachycardia, headache,
alopecia, rash, vomiting, diarrhea, mucositis,
myelosuppression, anemia (nadir 7-1 4 days),
thrombocytopenia (nadir 9-.1 6 days), peripheral
neuropathy, bronchospasm.

Famotidine Tredlmenl ol gdslric and duodenal ult er.
Famotack, Servipep 20 mg, (blocks histamine-2 receptors).
40 mg tab. Oral, lV 1-2 n{k{d in 1-2 doses, max 40 mg/d.
Fat emulsion \utritional supplement with parenteral nutrition.
Liposyn ll 1O"k,20%. Premature infants: Start 0.5 gkg/d and increase by
0.5 g/kg/d as tolerated to 3 g/kgld.
lnfants and children: Starl 0.5 1 gkg/d and increase at 0.5
g/kg/d increments as tolerated to max 3-4 g/kg/d.
Filgrastim. C-CSF Cranulocl te colonv stimulating fat tor rslimulate lhe
Neupogen, production, maturation, and activation of neutrophils).
lnjection: 300 mg/mL. Neonates: 5 pg/kg/dose daily for 3-6 doses.
Children: 5- 10 prg/lgldo.e daily tor up to I4 ddys
Cl discomfort, thrombocvtopenia, increased
liver enzymes.
Hyperlipidemia, hepatomegaly, dyspnea and
hypoxemia may occur if infused too quickly or
excessive dose.
Hypotension, vasculilis, {ever. exat erbalion of
pre-existing skin disorders, increased uric acid,
thrombocytopenia, medullary pain, hematuria,
proleinurid.

Fludrocortisone Acetate Partial replacement therapy for adrenal insu{ficiency. Hypertension, congestive heart failure,
Florine{. lnfants and children: 0.05-0.1 mg/d. convulsions, headache, acne, rash, bruising,
Tablet: 0.1 mg. hypokalemia. HPA-axic (adrenalr suppression,
peptic ulcer, muscle weakness.

Flumazenil Benzodiazepine antagonist to reverse sedative effects
Romazicon. (antagonize benzodiazepine effects on CABAI
lnjection. benzodiazepine receptor conrplex).
0.U05-0.0 I mglg load, lhen as continuous inlusion
0.005-0.01 mg/kg/ hr (max cumulative dose 1 mg).
Fluorouracil Antineoplastic antimetabolite that inhibits thymidylate
Adricil, Efudex, synthase leading to thymidine depletion.
lnjection, topical solution, cream lV 12 mg/kfld (max 800 mg/d) for 4-5 days then
6 mlkg every other da,v for 4 doses. Repeat in 4 r'vk.
Cream or solution 5%: Appl,v to entire altected area
trvice daily.
Arrhythmias, hypo- or hypertension, seizures,
acute withdrawal syn-rptoms.
Arrhythmias, hypotension, heari failure,
cerebellar ataxia, alopecia, skin pigmentation,
photosensitivitl,, loss of naiis, Cl upset,
stomatitis, hepatotoxicity, myelosuppression
IWBC and platelets: onset 7-1 0 days,
nadir 9-1 4 days, recovery 2 l days).

r
F

Y
h
 'l
ESSENCE OF PEDIATRICS

Fluticasone
l-lonase, Flovent
Nasal solution: 50 mg/spray.
Metered dose inhaler: 44, I lO,
22O mg/spray
Rotadisk: 50, 100, 250 mg/dose
ravailable with salmeterol as
seretide).
Treatffient of allergic rhinitis and persistent asthma. Dysphonia, oral thrush, adrenal suppieision,
Nasal spray: 1 -2 spray in each nostril once daily, gfol|/th suppression, cataracts.
MDI BB-480 mg twice daily (depending on asthma
severity).
Rotadisk 50*1 000 mg twice daily
(depending on asthma severity).

Fluoxetine Hydrochloride Treatment of depression and obsessive tompulsive Headache, nervousness, insomnia, anxiety, .

Prolerl, Modipran; 20 mg cap. d sorders (antielepressang inhibits CN S, ierotonin uptake).

i mania, suicidal ideation, tremor, anoreria,
Children 5-18 yr: Initial 5-1 0 mg/d then titrate slowly to diarrhea; constipation, dry mouth, weight loss.
effect (max 20 m$d).
Folic Acid Treatment of folate deficiency anemias, .i.e., megaloblastic, Nontoxic in man.
Folison, Folac 5 mg tab. macrocyrtic (cofacto,r for normal erythropoiesis)
0.2 mg/k{d PO, single dose.
Furosemide- Diurelic f inhibits sodium and chloride reabsorption at the Dehydrat ion, eleclrolyte loss. hyperuricemia,
Lasix, Fusid ascending loop of Henle and disial tubule). photosensitivity, hepatitis, hypercalciuria, renal
.l
lnjection: 0 mg/ml, in 2 ml amp Premature infants: O.5-2 mglkg lV or 1-4 mg/kg oral every slones, otoloxicity, Cl intolerance.
Tablets: 40 mg. 12*48 hr (dose to response).
Infants and children: 1-2 mg/kg lV or 1-4 mg/kg oral
ever\/ 6-24 hr or continuous infusion start
at 0.05 mgikgihr (dose to response).
Cabapentin ,{djunct to treatment of partial and seccndarily generalized Somno lence, depression, hyperactivity,
Neurontin. sei zu res. aggression, weight gain, diplopia, CI upset,
Capsule: 100, 300, 400 mg Children 2*12 yr: 5,35 mg/kgld in 3 divided doses constipalion.
(mdx s0 n{k{d1
'Children >1 2 yr: Start 300 mg daily, then daily increase
by
300 mg to 900-3600 m{d in 3 divided doses.
Gintian Violet Treatment oJ cutaneous and mucocutaneous infections Burning, local irritation or sensitivity reactions.
Viola lotion (kills candida, staphylococcal gpecies, and some Caution: do not swallow.
Topical sol utio n: 1
o/o, 2a/o: .vegetative gram-positive bacteria).
lnfants: Apply 3-4 drops of a 0.5% solution under tongue
or on lesion after feedings.
Children: Apply 0.5-2% with cotton to
lesion 2-3 times/d for 3 days.
Glucagon Treatment o{ hypcgJycem ia isti mulates hepatic glycolysis Nausea, vomiting, hypersensitivity reactions.
Powder for iniection. and gluconeogenesis).
Neonates: 0.3 mg/kg/dose (max 1 mg) lV, lM, SC.
Children: 0.025-0.1 mglkg/dose imax 1 mg), may repeat
in 20 min.
Cold Sodium Thiomalate Treatment of rheumatoid arthrilis (mechanism unknown). Headache, seizures, exfoliative dermatitis,
Myoihrysine, Children: Test dose 10 mg llvt followed by 1 mg/kg lM erythema nodosum, hives, alopecia, Ioss of
lnjection: 25 mg/mL, ergry wk for 20 wk; then 1 mg/kfldose every 2-4 wk nails, stomatitis, leukopenia, thrombocytopen.ia,
(max 50 mg/doser. hematuria, proteinuria, nephrotic syndrome;
interstitial pneumonitis, hepatotoxicity, :
periphera I neuropathy.
Haloperidol Treatment.of severe behavioral problems including Drowsi nets, restlessness, extrapyramidal
Peridol, Serenace 5 mg tab, 5 mg/ml psychose! (competitive blocker of dopamine reqeptorii, symptoms/ dystonia, tardive dyskinesia,
in 5 ml amp. Children 3*12 yr: Oral, start 0.25-{.5 mgld in 2-3 divided seizures, constipation, swelling of breasls,
doses, then iricrease weekly by 0.25-0.5 mg daily based hypotension, tachycardia, arrhythmias, urinary
on response to max 0,1 5 mg/kg/d. retention, blurred vision, cholestatic liver
6^12 yr: IM, 1-3 i.ng/dose every 4*B hr disease, agranulocytosis, leukopenia.
(max 0.15 mgikgldi.

Heparin, 5000 lU/ml in 5 ml vial
Prophylaxis and treatment of thromboembol ism (potentiate Bleeding from various sites, e.g., urine, gums,
aclions of anlithrombin lll). nose; bruising, thrombocytopenia, thrombosis.
Neonates, infants, and children: -
Thrombosis and FCMO: Ioad 50 units/kg tV bolus, and 1
15-35 unifVklhr continuous lV infusion maintenance dose, ?
Catheter patency: 0.5-.1 unit/ml. 1
J,
a
I
 DRUG THERAPY IN CHILDREN

Homatropine Hydrobromide
l\opto
Homatropine
Ophthalmic solution 2'k, 5%
Human Crowlh Hormone
Norditropin 12 lU/vial
lnjection.
Producing cycloplegia and mydriasis for refraction, Blurred vision, photophobia, local stinging,
treatment of uveitis (antichol i nergic). respi ra tory congestion.
Children: For mydriasis: 1 drop of 27o solution before
procedure. may repeal every l0 min as needed.
Uveitis: '1 ckop2oh solution 2-3 timesld.
lrealmenl oi growth failure due to inadequate Erowth Local lipatrophy, hypothyroidism, pain in hip
hormone secretion (replacement therapy). or knee-
Humatrope: 0.06 mg/kg (0.15 lu/kg) 3 times/wk.
Nulropin: 0.0a t mgrkg/d.
Protropin: 0.1 mg/kg (0.26 lU/kg) 3 times/wk.

Hydralazine
Apresoline 25. 50 mg tab;
20 mgJl ml amp.
Hydrochlorothiazide
Combination Hydrochloroth iazide
50 mg - Amiloride 5 mR rAmizider.
Hydrochlorothiazide 25 mg -
t rirmterine 50 mg rDezider.
Tre.rlment o[ hypertension, aditrnct trealment of congeslive
heart failure (vasodilation of arterioles).
Neonates: lV, 0.1-0.5 mg/k3/close every b B hr.
Oral 0.25-1 mg/kg/dose every 6-8 hr'
lnfants and chiidren: IM, lV start 0. l- 0.2 mgrkgrdose
every 4-b hr and tilrare lo effect {mar 3.5 mglkg/dr.
Oral 0.75-1 mg/kg/d in 2-4 divided doses
tn..." i.s ,.rgrkfi.ri
Trealment o[ hyperlension and fluid overload redemat
states. e.g.. CHI ,diurelic inhibits sodium reabsorplion in
distal lubule'.
2 4 mg/kgzcl in I 2 divided doses.
Palpitation. flush ing, tachycardia, Cl upset,
lupus-like syndrome, arthralgia, peripheral
neuropathy (related to pyridoxine deficiency)
Hypokalemia. hypochloremia, hvperglycemia,
hyperuricemia, hyperlipidemia, pancrealilis.
leukopenia, thrombocytopen ia, aplastic
anemia, hepatitis, intrahepatic cholestasis,
prerenal azolemia.

Hvdrocorlisone Treatment of adrenal insufficiency, congenital adrenal Hypertension. hvperglycemia. hypokalemia,

Cotson, Solucorlel 100 mg/2 ml vials. hvperplasia. shot l, a:lhma anti-intlammatorl , euphoria, headache, Cushing syndrome, peptic
glucot orticoidr. ulcer, cataracts, immunosuppression, skin and
Neonates, infants, chi ldren: muscle atrophy. Acne, edema.
Adrenal insufficiency; 1-2 mg/kg lV bolus, Caution: Abrupt withdrawal may cause acute
then 25-1 50 mg/d clivided every 6 hr. adrenal insuf{icienc y.
Congenital adrenal hyperplasia: lV. 0.5-0.7 mg/kg/d start, Anti- Sodium
then U.3- 0.4 mg/kgrd mdinlenance lherapy, give dose: as Drug inllammatorY retaining
% in AM % at noon, and % at night. effect (mg) eflect (mg)
Shock: lV, 35-50 mg/kg, then 50-1 50 mg/kg/d divided Hydrorortisone 100 100
erery b hr for +A lihr. Corliqone BO B0
Anti-inflammatory: lV, lM t-5 mg/kgid in 1-2 doses, Prednisolone 20 100
oral 2.5 lO mgikgd divided every 6 B hr. Prcdnisonc 20 I 0O
Shock: lV 50 mg/kg, dose everY 4 hr. Melhylprednisolone ltr 0
Severe acute utihn-ru' lV 3-a mg/kg/dose every 4-6 hr. I riamr inolone lb O

Deramelhasone 2 0

Hydroxycobalamin, Vitamin B,r, Treatment of pernicious anemia, vitamin 8,, deficiency. Comment: May require coadmini<tration of
Cvanomin-H 1000 pg/l ml amP. 100 prg/d lM iotal I prg over 2 wk. then l0 50 pg each mo. folate.

lbuprofen
lnflam, Rheumafen 200, 400 mg tab
Suspension: 100 mg/5 mL
Brulen 5R jUO mg cap
Lsrufen 5R 600 mg cap.
lmipramine
Treatmenl of pain. fever, rheumatoicl arlhritis (nonsleroidal
anti-inflammatory, inh ibit prostaglandin synthesis).
Pain, fever: 5- I 0 nrg/kg/dose every b B hr.
Juvenite rheumatoid arthritis: l0-50 mg/kg/d in 4 divided
dose..
lrealmenl of depression, enuresis, pain rtrit yclic
Abdominal cramps, heartburn, Cl bleeding,
Cl perforalion, [luid reLenlion. edema.
hypertension. dcute renal [ailure.
At ropine-l i ke side effet 1s: arrhythm ias, poslura I

Melipramin, Tofranil 25 mg tab.
anlidepressdnl, increase. synaptiL concentrations of hypolension. drowsiness, sedation. dry mouth,
norepinephrine and serotonin). constipation, urinary retention, increased liver
Children: enz) me\. seizttrec.
Depression: Start 1.5 mg/kg/d, may increase by 1 mg/kg1d
every 3 4 days (max 5 mg/kg/d).
Enuresis: >6 yr,lO-25 mg at bedtime.

a
a
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 a-

ESSENCE OF PEDIATRICS

lmmune globulin, intravenous
(rvtc).
Humaglobin.
Sandoglobulin 2.5 g/vial.
lndomethacin
lmet, lndomet, 25 mg cap,
100 mg suppository
Reumacid supposilory I00 mg/stick
lmmunodeficiency syndrome, lTP, acute bacterial or Flurhing tachycardia, chi I ls, nausea, dyspnea,
viral infections in immunocompromisecl or neutropenic fever, hypersensitivity reactions, headache,
patients/ Cuil lai n Barre syndrome, demyelinati ng aseptic meninBitis.
polyneuropathy (replacement therapy or interference
with Fc receptors in the reticulo-endothelial system {or
autoimmune dir_"1:"t)
.
Neonates: 500-750 mg/kg once.
Children:
Immunodeficiency syndrome: 1 00-400 m$kg/dose
every 2-4 wk.
ITP: 1000 mfkg/dose for 2*5 consecutive days
then every 3- 6 wk.
Kiwasaki disease: 2 g/kg single dose.
CMV infection: 500 mg&g/dose every irther day for
7 doses.
Severe systemic infection: 500-1000 mglkg once wk.
Polyneuropathy:1 g/k$dfor2 consecutive days each mo.
Closure of the patent ductus arlerious (PDAr in neonates, Dizziness, vomiting, abelominal pain, CI
treatment of rheumatoid disorder, (NSAID, prostaglandin bleeding, ulcers, Cl perforation, bone
inhibitionr. marrow suppression, impaired platelet
Neonates: lV, 0.10-0.25 mg/kgldose every 12 hr aggregation, renal failu re, hypertension, edema,
for I
6 doses. hyperkalemia.
lnflammatory rheumatoid disorders:
Children: 1-2 m:flkg/d in 2-4 doses (max 4 mgikgld). .

lnsulin Treatment of insulin dependent diabetes mellitus Hypoglycemia (and associated symptoms of
Short acling: Ireplacement therapyt. dizziness, weakness, paresthesia. numbness
Insulin actrapid, insulin actrapid HM, Neonates: Regular insulin 0.01-0.1 units/kfl hr continuous of mouth, fatigue, mental confusion, hunger,
40 lU, 100 lU/ml in l0 ml vial. infusion, or 5C 0.1-0.2 unitVkg every 6-12 hr. nausea), visual problems. hypokalemia.
Medium acting: Children: 0.5-1 uniVkg/d. Adjust doies io blood glucore
lnsulin mixlard 30 HM; 40 IU, and hemoglobin A,. results. Adolescents (during growth
100 lU/ml in 10 ml vial. spurt): 0.8 1.2 units/kg/d.
Diabetic ketoacidosis: Continuous infusion lV 0.1 unitsikg/hr
Long acting:
adjusted to serum glucose.
lnsulin lente 40 lU, i00 IUiml in
Hyperkilemia: Try calcium gluconate and NaHCO. first,
10 ml vial.
then dextrose 50% 0.5*1 mUkg and regular insulin 1 unit
per 4-5 g dextrose.
Interferon Alpha-2a, In hemangiomas of infancy and pulmonary hemangiomas Tachycardia, arrhythmias, hypotension, edema,
Ro[eron-A, (inhibits cellular growth, alters cellular differentiation). CNS depression, dizziness, flu-like symptoms. \
lntron-A 3 million units/ml, 5C 1-3 million units/m2 once daily.
5 million units/ml vial.
lpecac lnduces vomiting to treat certain toxic ingestions Lethargy, persistent vomiting, diarrhea.
Syrup Ceneric (stimulates medul lary chemoreceptor tri gger zone).
Syrup: 70 mg/ml. 10-30 ml followed by 20 ml/kg of water.
lpratropiuin Bronchodi lator, treatment of rhinitis {anticholinergic). Dry mouth, nervousness, dizziness, headache, _
Atrovent, lpravent, Neonates: Nebulized 100 mldose or MDI blurred vision, urinary retention.
Nebulization solulion 0.02%, l-2puffsl4times/d.
Metered dose inhaler: 1B mg/puff. lnfants and children: Nebulized 125-250 pg or
MDI l-2 puffs 3-6 times/d
.1-2
Nasal spray: Q "3ak, 0.60/". Nasal spray for rhinitis: sprays in each
nostril 2 3timesdaily.

;
!
t

j
 DRUG THERAPY IN CHILDREN

lron Treatment of iron deficiency (replacement therapy). Oral: Cl irritation, nausea, constipation, dark
lron lnfant: 2 mg elemental ironlkg/d PO in 1*3 divided doses slools.
Folfetab, Ferocit (Ferrous fumarate Children: 6 mg elemental iron/kg/d PO in lV, lM: Hypotension, {lushing, dizziness, fever,
200 mg + Folic acid 200 mg/tab.) l--| divided doses. metall ic taste, arthralgia, anaphylaxis.
Aristoferon, Fe-plus (Ferrous sulfate
200 mg/5 ml syp.)
Feridex, Fesyrup {Ferrous glucondte
300 mg/5 ml syp)

lron Total Flemental

salt confent lron
Ferrous 200 mg 65 mg
fumarate
Ferrous 200 mg 40 mg
v su lfate
Ferrous 300 mg 35 mg
gluconate

lron (lll) hydroxidr Polymaltose 5 mg of elemental iron/kgid; preterm-3 mglkg/d Cl i rritation, vomiting, diarrhea.
complex after meal
Polyron 50 mg/5 ml syp.
lsoproterenol Asthma, ventricular arrhythmias due to AV node block, Tachycardia, palpitations, chest pain,
Ceneric, low-output shock states (stimulates beta-l and beta,2 nervousnessi restlessness, insomnia, tremor,
V
lnjet tion, sublingual tablets, receptors). Cl distress, paradoxical bronchospasm.
t- nebulizer solution. Neonates, infants. and children:
metered dose inhaler- lV infusion 0.05-2 pglkglmin.
v C.hildren: MDI 1-2 puffs every 4 hr as needed;
t SL tab 5-10 rng every 3-4 hr

Kelamine Anesthesia for short procedures (direct action on cortex Hypertension, tachycardia, hypotension,
Calypsol, 50 mg/ml in 10 ml vial. ancl limbic system to produce dissociative anesthesia). bradycardia, increased cerebral blood flow and
Cive 30 min prior to procedure. intracranial pressure/ hallucinations. delirium,
fM 3-Z mglkg; lV 0.5-2 pg/kg. ton ic-clonic movements, hypersal ivation,

t vomiting, respiratory depression, cough.

Ketorolac Treatment of pain (NSAlD, inhibits prostaglandin). Edema. dizziness, headache, Cl upset,
Toradol, Emodol 10 mg, Children 2-16 yr. bleeding, peptic ulcer, impaired platelet
30 mglml amp, 10 mg tab lM, IV 0.4-l mg/kg/dose. aggregation, acute renal failure, clyspnea,
Oral 1 mg/kg/dose every 6 hr if needed. wheezing.
Labetalol Treatment of mild to severe hypertension iblocks alpha Orthoslatic hypotension, CHI-, conduction
t Normodyne, Trandate, and beta adrenergic receptorsi. disturbance, bradycardia, drowsiness, dry
lnlection: 5 mg/ml. Oral: Start 4 rng/kg/d in 2 doses, then gradually increase mouth, nasal congestion, bronchospasm.
Tablet: 100, 200, 300 mg. (max 40 mg/kg/d).
r lV: Start 0.2-l mg/kg/dose (max 20 mg/dose), continuous
lV infusion 0.a-1 mg/kg/hr (max 3 mglkg/hr).
L
Lactulose Treatment of constipation. hepatic encephalopathy Flatulence, abdominal discomfort, diarrhea,
IF Avolac, Lactu, Osmolax (osmotic effect on stool in colon, acidification of stool vomiting.
3.4 g/5 ml susp. prOmotes NHr" elimi nation).
infants 2.5-1d ml/d in 3-4 doses
L
Children: 40-90 ml/d in 3-4 doses.
Lamotrigine Treatment of partial seizures (blocks sodium channels and Sedation, headache, agitation, exacerbation of
Lamictal 50 mg tab inhibits presynaptic release of glutamate and aspartate). seizures, rashes, angioedema, photosensilivity,
I 2-16yr: Z n{kgld in 2 doses for 2 wk, then 5 mgikgld nystagmus, amblyopia.
in 2 doses for 2 wk, then l0 mg/kg/d in 2 doses if needed
i (usual 5-1 5 m{k{d, max 400 mg/d).
lf patient is on Valproate:0.2 m{kg/d in 2 doses for 2 wk,
t
f,
then 0.5 mgkgd in 2 doses for 2 wk, then 1 mg/kg/d in 2
doses (max 5 mgikgld).
Levothyroxine Thyroid replacement therapy Tachycardia, arrhythmias, hypertension,
F Oroxine 50 pgitab Oral: O-6 mo: 8-10 V{kS/d; 6-12 mo 6-8 yglk{d; insomnia, headache, hair loss, increased
p{k{d; >12 yr:2-3 Vgkgld.
1*12 yr'. a-6 appetite, weight loss, tremor, sweating.
I Myxedema coma: 200-500 pg for one dose:
i
I)
t
L
F {

ESSENCE OF PEDIATRICS

Lidocaine Anhyth:mias, heart block, lethargy, coma,

lasocaine 1 ",6, 2\%, 4n/o, seizure, vomiling, paresthesias/ b lurred vision,
in 2 ml amp. 50.mI vial. diplopia, local skin irritation or rash.
Iasocaine 29,6 gelly.
Lidocaine spray.

lithium Polydipsia, diarrhea, bloated feel ing, weight

Camcolit, Lithosun SR 400 mg tab. gain. tremor, muscle twitching, weakness,
diabeles insipidus, renal tubular acidosis,
leukocytosis, vision problems, hypothyroidism,
goiter, skin eruptions, acne.

Loratadine Somnolence, fatigug anxiety, depression,

Oradin, Lorfast, Eladin 10 mg tab, headache.
5 mP/5 mt syp.

Lorazepam Several cases of myoclonus have been

Alivan reported in neonates, tachycardia, drowsiness,
Injection depression, paradoxical excitemenl, blurred
Tablet: 1, 2 mg. vision, diplopia.
Oral solution: 2 m{mL.

Magnesium Hydroxide, Milk of Hypermagnesemia, hypotension, abdominal

Magnesia. cramps, muscle weakness, CNS depression.
400 mg/5 ml susp.

Magnesium Sulfate Hypermagnesemia, hypotension, abdominal

C-Magnesium sulfate 50% cramps, muscle weakness, CNS depression.

Mannitol Circulatory overload, congestive heart failure,

Mannitol 20%, Osmosol 20% in headache. chills, seizures, fluid and eleclrolyte
50o ml bottle. imbalance.

Mechlorethamine Vomiting, diarrhea, myelosuppression (onset

Nitrogen Mustard 4-7 days, nadir 14 days, recovery 21 days),
Mustargen Hydrochloride ototoxicity, precipitation o{ herpes zoster,
lnjection alopecia, hyperuricemia.
Caution: Extravasation should be treated
promptly with sterile sodium thiosulfate and :
apply cold compress for 6-12 hr.
Mercaptopurine Tieatment of leukemias and non-Hodgkin lymphoma Hepaloloxicity, Cl upset, stomatitis, mucositis,
\
Purinethol, 50 mg tab. (antimetabolite; blocks purine synthesis), skin rash, hyperpigmentation, myelosuppression 1
Oral: lnduction 2.5-S m{kg once daily; (onset 7-1 0 days, nadir 1 4 days. recovery 2 1
1
maintenanc€ 1.5-2"5 mglkg once daily. daysr, renal loxicity, hyperuricemia, drug fever.

I
 DRUG THERAPY IN CHILDREN

Methimazole Treatment of hyperthyroidism (blocks iodine synthesis in Fever, skin rash, leukopenia, agranulocytosis,
Tapazole. the thyroid gland, inhibits synthesis of thyroid hormonel. SLE iike syndrome, vomiting, stomach pain,
Tablets 5, 10 mg. 5tart 0.4 mg/lg/d. lhen maintenanr e 0.2 mgkgrd loss of taste, cholestatic jaundice, constipation,
weight gain.
Methotrexate Treatment of neoplasms, psoriasis, rheumatoid arthritis Hepatolor.it ity, nephropathv. vascuiilis.
Methotrexate 2.5 rng/tab (antimetabolite, inhibition of DNA and purine synthesis). encephaloprthy, headache, seizures. Iever,
Emthexate 50 mg/vial Juvenile rheumatoid arthritis: Oral, lM 5-15 mg/m'/wk as cystitis, Cl upset, alopecia, increase or decrease
a single dose. in skin pigmentation, urticaria, arthralgia,
Antineoplastic: Oral, lM 7.5-30 mgm every 1-2 wk, IV h1 peruricemia, myelo5uppre\sion roniet 7 davs.
.10-33
g/mr bolus dose or infused over 6-42 hr. nadir 1 0 days, recovery 21 days).
Methyldopa lreatmenf of hypertension ,false alpha neurotransmitter Drowsiness. depre<'ion, rertigo. lluid retenlion.
Fidopa, Dopegyt 250 mg tab. metabol ite stimu lates i nhibitory alpha-adrenergic choleslalic I iver diseare, cirrhosi:, pan( reatiti5,
re( eplorsi. hemolytic anemia, positive Coombs test,
Oral: Start l0 mgrkg in 2-4 doses, ma1 increase every leukopenia. lhrombocylupenia, hypotension.
2 day. 111.11 65 m8/l8/d t-r1 I g'dr. bradyt ardia.
Melhylphenidate Aflenlion deiir it disorder. narr oleprv, adjunct ior pain \ervousness. in*omnia, agitation, anoreria,
Krtalrn management (CNS stimulant). weight loss, tachycardia, hypertension,
Tablet: 5, 10. 20 mg. Children >5 y r: 0. t 0.b rng'kg,dose rma\ 2 mg/kgdr. movement disorders, tics, growth retardation,
Tablel, sustained release: 20 mg. addir tion ,nol a LonLern with typit al ADD
dos i ng r.

Methy lpredn isolone Anti-inflammatory and immunosuppressant used in Hypertension, edema. p>yt ho>ir, pseudomolor
Solu-Medrol, Depo-Medrol. allergic. inllammalory rnd neopla,lic disorder.. and at ute cerebri, headache, euphoria, hyperglycemia,
40 mg/1 ml vial. spinal cord injury. HPA-axis (adrenal) suppression, Cushing
Anli-inflammalory and immunosuppressanl: lM, lV 0.5-2 syndrome. .kin atrophy. brui.ing,
m{k{d divide every 6-12 hr. hyperpigmentation, peptic ulcer disease,
Lupus nephritis: IV 30 mg/kg every other day for 6 doses. muscle weakness, bone loss, joint pain,
.1
Acute spinal cord injury: 30 mg/kg over 5 min, followed growlh relard,rtion. c.rtdracl.. glaur oma,
in 45 min by tontinuous in[u:ion of 5.4 mglkq,hr for 2 t immuno:upprer:ion.
hr.
Metoclopramide Treatment of gastroparesis, gastroesophageal reflux, and Drowsi ness, diarrhea, prolacti n sti mu lation,
Metocol, Motilon, 10 mg tab; nausea associated with chemotherapy and surgery (blocks breast tenderness, extrapyramidal reactions,
5 mg/5 ml syp; I mg'l ml drop; dopanrine re( eptor. in chemore, epior trigger 7one. IV administration is associated with an intense
i0 mg/2 ml amp. enhances Cl motility and gastroduodenal sphincter tone). feeling ol anriely and restlessness followed by
Neondtes infants, and t hildren: dror,r riness.
Castroesophageal reflur: lV. Oral 0.0J-0.i mg/kg/dose Comment: Administer oral doses ]0 min beiore
every B hr. meals and Jt bedtime.
Children:
Chemotherapy anti-emetic: Oral, lV l-l mgkgdo.e
elerv 2-4 hr pretreal wirh diphenhydramine ro aroid
extrapyramidal reactions).
Metolazone lreatment of iiuid overloacl states rdiuresis. inhibits l-luid and electrolyte imbalanre. hyperglycemia,
Zaroxolyn. Mykrox. sodium reabsorption at distal tubules). hypocalcemia, hypomagnesemia, nausea,
Tabler. tJ.2 0.4 mg/kg/d in I 2 doses. vomiting. blood dyscrasias.
Metoprolol Trealrnenl of hyperlenrion. tachyarrhythmias, migraine Depression, bradl r arclia, redur ed periplreral
Betalor , 50 rnB tab. prophylaris r:eleclire blot kers oi beta- I receptor\r. circulation, worsen diabetes, worsen asthma,
Oral I-5 mg kgrd. insomnia, nightmares.
Midazolam Sedation. antit onvulsanl'benzodiazepine, increase Several cases of myoclonus and prolonged
Dormicum, 7-5 mg, 15 mg tab; CABA effer tr. movement disorders have been noted in
5 mg/5 ml, 15 m{3 ml amp, 2 mg/ Neonates: lV continuous infr-rsion 0.15-0.5 mg/kg/min for neonates treated with midazolam, withdrawal
ml, 5 ml amp. sedation; lV bolus 0.05 0.15 prg/kg every 2 4 hr. reactions may occur if abrupt discontinuation,
lnfants and chrldren: sedation, paradoxical excitalion, blurred vision,
Status epilepticus: lV load 0.1.5 mg/kg followed by diplopia, apnea, respiratory depression.
Lontinuous iniu.ion I pg kglmin.
Sedation: lV 0.05 0.2 pglkg Ioad, then either same dose
every.l-2 hr or continuous infusion 1-2 pg/kg/min.
Mitomycin Cancer chemotherapy (antibiotic type alkylating agent Vomiting, myelosr.rppression (onset 2.1 days,
Mitomy( in-C I mg tab; 2 mg. inhibits DNA and RNA svnthesis). nadir t6 days. rer overy 42.50 days). rineling
7 l0 mgivial Depends on proto( ol; typicall) lV 3 mgm /d Ior s days of extremities, paresthesias, alopecia, mouth
every 4-6 wk; up to 40 50 mg/mr single dose for BMT. ulrer\, cardid( failure rdoses 2l0 mg),
l interstitial pneumon itis, pul monary fibrosis.

F
 i
ESSENCE OF PEDIATRICS

Montelukast Headache, dizziness, dyspepsia, fatigue,

Singulair, Monas elevated liver enzymes.
Tablet: 4, 5, 1o mg
Morphine Hypotension, bradycardia, vomiting,
Morphine, 15 mg/1 ml amp. conitipation, sedation, decreased urination,
respiralory depression.

Mupirocin Stinging and irritation at application site.

Bactroban
Ointment: 2olo.

Naloxone May precipitate acute opiate withdrawal.

Narca n. Duration of effect of many opiates may be
lnjection: 0.4 mg/ml. longer than naloxone requiring individualized
lnjection neonate: 0.02 mg/mL. naloxone dosing.

Naproxen Dizziness, Cl irritation, rash, age related

Napro Anaflex, 250 mg. 500 mg tab; decreased renal function.
12\ mg/5 ml susp; 10o/o as gel.

Nedocromil Dysphonia, chest irritation/pain.

Tilade, 2 mg/puff.

Neostigmine Bradycardia, abdominal cramps, urinary

Prostigmin. 0.5 mg/l ml amp. frequency.

Niacin Flushing, tachycardia, dizziness, hyperuricemia.

Nicoson 50 mg, 500 mg lab.

Nifedipine Profound, acute hypotension, flushing,

Adalat, Nificap, 10 mg cap dizziness. Preferred route is oral, not SL.
Nifin, Nidipine, 10 mg tab
Nidipine SR, 20 mg tab.

Nitroprusside Profound hypotension, tachycardia, thyroid

Nipride.
lnjection: l0 mg/mL, 25 mg/mL.
Nortriptyline
Nortin, 10 mg, 2 5 mg cap.
Nortrilen, 10 mg, 25 mg lab.
suppression, acidosis, seizures. Cyanide
toxicity-metabolic acidosis, pink skin
methemoglobinem ia.
Ant ichol inergic effects tdry moulh, tdchycdrclia,
blurred vision, urinary retention), sedation.

Octreotide Flushing dizziness, hypolhyper-glycemia.

Sandostatin. lnfuse IV over 20-30 min. lV push over 3 min.
lnjeclion: 0.05, 0.1, 0.2,
0.5, I mg/ml.
Omeprazole Rashes, urticaria, diarrhea, headache, insomnia,
:
Seclo, Losectil, 20, 40 mg cap. paresthesia.

\
Pancreatin Rash, abdominal complaints, constipation,
Zymet, Suzyme. hyperuricemia, a I lergy.
325 mg tab.
 DRUG THERAPY IN CHILDREN

Pancurortiuin Anesthetidskeletal muscle relaxarlt. .. Tachycardia, hypertension, prolonged muscle

Pavulon. Nqndqpolarizing neuromuscular ailtagonist' weakness.
2 myJmL,2 ml amp. 0.04'0,1 m$kg:lV q 20-30 min' Dose titrated to
desired effects.

Paraldehyde Anticonvulsant, sedative, generalized CNS depressanq Sedal.ion, gastric irrilation, toxic hepatitis,
Paral. 2,5 ml amp. used as adjunct treatment for refractoiy status epilepticus,l thrombophleliitis,i azeter-nia, oliguria,
0.1 5 ml/kg/dose PO, PR. May repealonce in 4-6 hr' albuminuria.

Pemolide Central nervous system stimulant used'in the treitment Centia'l.nervqus syslem stimulation, seizures,
Cylert. of attentioh deiicit disorder. Structural'ly unique from . hypertension; increased liver function studies,
Tablet: 18.75; 37.5, 75 mg. methylphenidate. hepatitis;'.n'rovernent disorclers.
Tablef chewable: 37.5 mg. I mglkg/24 hr PO ar single dose each morn'ing.
Titrate to effect 0.5 m{kglz+ hours at eVery l -2 wk. ,

Usual max dose 3 mg/kg/d (- 1'12.5 mgld). ,

Penicillamirie Metal chelating agent with affinity for copper (Wilson Ragh; pruritus, vomiting anemia, bone marrow
Byanodine, 150 mg cap' disease) and leail. Also used as an adjunctfor the ' suppre5sion, nephrotic syndrori're, SLE like :

treatment of Severe rheunlatoid arthritis. svndrome.

Pentazocine
Stopain, 25 mg tab; 30 mglml inj
Pentoxifylline
Trental, Agapurin.
Tablet, timed-release: 400 mg.
Wilson disease: Dose titrated to maintain >1 mfd urinary
copper excretion. 20 mglkgld PO q 6-12 hr {mai t g/d).
Lead intoxication: 30-40 mg/kg/d PO q S-12 hi
rmax 1.5 g/dr.
Rheunratoid arthritis: 3 mgikgld PO q 1 2 hr, increasing by
3 mglkg/d every 2-3 mo to max 10 mglkgld'
Opiate analgesic of the benzomorphan type for the CNS depression, vomiting, respiratory
treatment of moderate to severe pain. depression, histamine release.
Children >1 4 yr of age: 50 mg PO q 3-4 hr, titiate io effect
to 100 mg dose not to exceed 600 mg/d- May give
lM or lV r:educing oral dose by one-third.
Used in the treatment of peripheral vascu'lar disease' . Hypotension; tachycardia, nausea, vomiting,
(Raynaucl syndronre) and investigationally reducing
tumor necrosis factor, neutrophil adhesion and platelet
aggregation.
Antiplul"l*t in Kawasaki disease:
"ff*ct
20 m{kg/2a hr PO q B hr.

Phenobarbital
Cardinal, Berdinal, 30 mg, 60 mg
tab; 200 mg/1 ml amp.
Phenytoin
Diphedan.
100 mg tab, 125 mg/5 ml susP.
Phentin, 50 mg cap.
Bartlitqrate central nervous system depressant used as a Hypotension, drowsiness, respiratory
sedative, hypnotic anticoilvLilsent. depression, paradoxiial hyperactivity
Anticonvulsant: Loading dose, 15-20 mglkg PO, lV'
Maintenance dose-
Neonates: 3-am{kg/2ahr PO, lV, q12*24hr.
Childrenr 5*6 n{kgl2a hr PO, U, q 12-24hr.
Sedation: 2 mg&g per dose.
Anticonvulsant.and antianhythrnic. H irsutism, gingival hyperplasia; rash, Stevens-
Statusepilepticus:loadingdose- l lohnson syndrome, hepatitis, thrombophlebitir,
Neonate: 15-20 mg/kg lV; do not exceed 0.5 mg/kg/min. ataxia, nystagmus,
Child: 1 5*1 B mg/kg lV; do not exceed 1-3 mg/kg/min.
Maintenance dose-
Neonate: 5 mg/k{2a hr PO, lV q 12-24 hr.
Children: S-10 mglkg/Z4 hr.
Arrhythmias: Loading dose-
1.25 mg/kg lV q 5 min until desired effect or total dose
15 mg/kg.

I'hysostigminc
Antilirium
lniection
Maintenance dose-S-l 0 m{kS2 hr q 8-12h.
Competitive antagonist o{ acetylcholine. Unlike
neostigmine, crosses the blood-brain barrier with central
effects. Used with extreme caution in the reversal of
Palpitations, restlessness, excessive
sal ivation, secretions, muscl e fasciculations,
bronchospasm.
H
antichol i nergic effects.
0.001-0,03 mg/kg/dose lM, lV, SC, repeated q 15-20 min
to desired effect (max total dose 2 mg)'

J
 J

ESSENCE OF PEDIATRICS

Vitarnin K1 for nutritional supplementation and treatment FIush ing, hypotension.

l(ona'kion MM, 2 mg/O.2 ml, of hemorrhagic disease of the newborn.
10 mg/J ml amp; 10 mgrab. Children: 1-2 mg/dose lM, lV, PO.
NewLrorn prophylactic: I mg postpartum, repeated in 24
hr,
Active bleeding:10 ml/kg fresh frozen plasma + 1 mg lV
Pifa€€tam. Acute or chronic cerebral ischemia, MR, behavioral or Agitation, sleep disturbances, Cl upset, sexual
:NeuroleB &00 mg tab. psychotic problems. slimulation. rCl: ARF. hepatic impairmenil.
50 mg/k$d in 3 divided doses {il2 dose once desired
outcome is observed;.
Piioxicari. , Nonsteroidal anii-inflammatory agent used as an analgesic Dizziness, Cl upsel, ulcer, hepatilis, decreased
Flexisar4l0rngcqp. and in the treatment o{ rheumatoid disorders. renal function.
40nSZ ml amp. 0.2-0.3 mdkg q 24 hr PO {max dose t 5 mg/kg/24 hg.
rPizotiferr .'.
Vascular headache, migraine upto ,l.5 mg daily in div Anti-mascarinic elfecls, drowsiness, increased
'Pizofen,O.5 mg tab. doses (max single dose at night is 1 mg). appetite, wt gain, CI upset, CNS stimulation.
Pralidoxime Acetylcholinesterase reactivator used in the treatment of Hypertension, clizziness, nausea, muscle
PAM-A' 500 mg/vial, organophosphate poisoning. wea kness/rigidity.
20-50 mg/kg/dose lM, lV repeated in l-2 hr if riruscle
weakness. has not been rel ieved.
Prazosin Competitive antagonist of postsynaptic alpha-adrenergic Syncope, palpitations, dizziness, fluid retention.
Alphapress; 1 mg,:2 mg tah. receptors used in the treatment of hypedension/heart
failure.
A.1 mSk{2a hr PO q 5 hr titraring dose to desired blood
pressure. Usual max dose 0.4 mg/kgl2a hr or 15 mg total
dose.
Prednisolone Clucocorticosteroid used in the treatment of inflammatory Edema, hypertension, psychosis, Cushing
Prednisolone, Precodil 5 mg tab. disorders including a llergic, respiratory (asthma), syndrome, HPA-axis ladrenal; suppression,
iheurnatic, and neoplastic disorders. peplic ulcer.
1*2 m/kg/2a hr PO q 6-12hr.
P.reilni5one Clucocorticosteroid used in the treatment of inflammatory Edema, hypertension, psychosis, Cushing
Dellasone disorders ncluding a llergic, respiratory rheumatic,
i
syndrome, HPA-axis (adrenal) suppression,
Tablet: 1,2.5, 5, endocrine, and neoplastic disorders. peplic ulcer.
1O,,,2Q 50,mg- Aslhma:
Children: 0.54 mglk{2a hr PO q 6-12 hr.
Anti-inflammatory:
Children: O.1-2 milk1,/24 hr PO q 6-8 d.
:Priimidone
AnticonVulsant used in the treatment of generalized Sedation, ataxia. raslr
Myloline tonic-clonic, complex partial and focal seizures.
Ta,b!e!;,59,,s9, 250 mg Neonate: 12-2O mglk$2a hr PO q B-12 hr.
Suipensian:250 mg/S mL Children: 10-25 mg/k{2a hr PO q 8-1 2 hr,
?r.ocirinamide Anti-arrhythmic, ventri cular tachycardia, pVCs, pAT, atri a I Hypotension, arrhythmias, A-V block,
Proriestlrlr,'Procari, . fibrillation. confusion, agranulocytosis, SLE-like syndrome,
fablet and capsule 250, Loading dose: 3-6 mgikgldose lV over 5 rnin not to fever, rash.
375;r500 mg. exceed 1 00 mg/dose; repeat q 5-l 0 min as needed to max
15 mglkg total dose. Do nor exceed 500 mg in
J0 min.
Maintenance dose: 15-50 m{kg24 hr PO q 3-6 hr;
20-30 mg/kg 24 hr llA, IV; nor to exceed 4 g/24 hr;
continuous IV infusion 20-S0 ;iglkg/min,
usual max 2 {24hr.
Fiiicaibazine Antineoplastic used in the treatment of Hodgkin CNS depression, confusion, ataxiar marrow
Captffe:rr50.mg lymphoma. suppression, alopecia, flu-like syndrome.
Hodgkin disease: 1 .5-3 mglk{2a hr (50-1 00 mg/m,) pO
qd foi 1 0*1 4 days per 28 day cycle.
Neuroblastoma/medulloblastoma: I 00-200 mg/m'/dose
per protocol.
!
Prochlorperazine Phenolhiazine anti-emetic, use should be avoided in Sedation, extrapyramidal reactions,
Stemetil, Vergon;
5 rng tab; 12.5 m{1 ml amp.
children. photosensitivity. cholestati< jaundir e. \
Child: 0.4 m{kg/z+ hr PO, 0.1-0.15 mg/kg/24 hr tM q +
B-12 hr. t

I
t DRUG THERAPY IN CHILDREN
I
I
i Promethazine Phenothiazine with primary antihistaminic activity used in Sedation, hypotension, extrapyramidal
Phenerex, Phenergan I0 mg, the treatment of vomiting, motion sickness, allergy. reactions, blurred vision.
25 mg tab; 25 mg/ml in 2 ml amp; Motion sickness: 0.5 mg/kg PO, 30-60 min before
Otosil, Phenerelx syp 5 mg/5 ml. departure; then q B-1 2 hr as needed.
Sedation anti-emetic: 0.25-1 n{kg'dose lM, lV.
:
Propantheline Bromide Synthetic anti cho inergic antispasmodic used as adju ncti ve
I Sedation, tachycardia, dry mouth. blurred
Propanthene 15 mgtab. therapy of Gl or bladder spasm, irritable bowel. vision, mydriasis.
:
1.5-3 mg/k/24 hr PO, q 't-8 hr. Dose lo desired effect.
) Propranolol Nonselective beta-adrenergic receptor antagonist f)ecreaced cardiac contractiliiy, hypotension,
i Adlock, Indevar. 10, 40. (beta-l and beta-2). trradycardia, hypoglycemia, bronchospasm.
t B0 mg tab. Arrhythmia/hypertension: O.5-1 mg/k!2a hr PO q 6.'8 hr
lnjection: I mgiml rlnderal) titrated upward to 2.5 mglkg/24 hr, over 3-5 days.
lV dose-0.01 -0.1 mflkg/dose: infused over 1 0-.1 5 min as
: needed tmax dose 1 mg infants; 3 mg children;
Migraine prophylaxis: 0.6-2 m{k{2a hr PO q 6-8 hr;
I usual max a m{kg/2ahr.
I Thyrotoxicosis: 2a m{kgl2a hr PO q 6-8 hr; titrate to
t response.

Pyridoxine Pyridoxine-dependent seizures: 50-1 00 m$d Nausea, decreased folic acid.

I Pyrovit 20 mg lab. Dietary deficiency: 5- 15 m{dlor3-4 wk then 2.5-5 m{d
Drug-induced neuritis (lNH, Hydralazinei: 1 mg/k{2ahr
PO.

I Ranitidine H stam ne-2 (Hr) receptor anta8oh ist competitively inhibits

i i

gastric acid secretion in gastric/peptic ulcer diseaselstress

Headache, mental confusion.
Zanlac, Neotack, 1 50,
I 300 mg tab ulcer prophylaxis, CE reflux disease.
Cepin syrup. 75 mg/5 ml Neonate: 1 .5-2 m{k$2a hr PO, lV q 12 hr;
t) Ranidin, Ranison 50 mg/2 ml amp. Children: 1-5 mf,kg/24 hr PO, tM, lV q 6-8 hr;
conlinuous 24 hr lV infusion 2-5 mg/kg/z4 hr.
Riboflavin Vitamin used in supplementation and deficiency states. Extremely rare.
Riboflavin, Riboson. Deficiency: 2.5*1Q m{24 hr PO, q 8*12 hr.
I 5 mg/tab.
Salmeterol Long-acting beta-2 adrenergic agonist 1*B-1 2+ hr); Tachycardia. palpitations. headache,
Bexitrol, Salmate inhaler 25 mg/puff. bronchodilator used in the treatment of reversible airways nervousnessr muscle tremor, cough, airway
Used with Fluticasone as seretide. disease. Excellent in patierits with nocturnal asthma. irritation.
I Caution: Not for use in acute asthma attack.
I 1 -2 puffs, q 1 2 hr titrate to desired effect-
Senna Stimulant calharlic for shorl-term trealment of Abdominal cramping, diarrhea, fluid and
I Laxenna 600 mg tab. conslipalion, bowel preparation prior to radiology. eleclrolyte imbalance
I 10-20 mg/kg/dose P0, q 12-24hr. Caution: Avoid prolonged use {>1 wk).
! Antiflatulent for symptomatic relief of infantile colic, Safe, without clinically significant side effects.
Simelhicone
Flacol, Simecon 40 mgi0.6 ml. excessive gas.
Children i2 yr: 2O mg/dose PO q 4-6 hr.
Children 2-12 yr:40 mgldose q 6 hr.
Sodium Polysterene Sulfonate lon-exchange resin that removes potassium for sodium for Abdominal cramping, l:loating, hypokalemia
Kayeralate Powder for suspension. treatment of hyperka lemia. Cautions: Follow serum potasiium closely. Do
I Children: 4 gkg24 hr PO q 4-8 hr; not mix with potassium containing liquids (e.g.,
I Rectal: 4-1 2 gkg24 hr PR q 2-6 hr. orange juice).
I Spironolactone Compel.itive aldosterone antagonist used as a mild, Lethargy, hyperkalemia, Synecomastia, rash.
\ Aldactone, Pilactone, potassium-sparing di uretic, antihypertensive, i n chron ic Caution: Careful monitoring of serum potassiurn
t- Tablet: 25 mg. liver disease.
Neonates:1-3 rng/kg/d PO divided q12-24hr.
t Children: 1.5-3.0 mg/kg/d PO divided q 8-24 hr.
Streptokinase Thrombolytic agent used in the treatment of deep vein Bleeding, bronchospasm, flushing, rash.

t; Streptase, Kabi Kinase,

'l
.5 million units/vial
thrombosis. stroke, catheter patency.
Thrombosis: 3500-4000 units infused lV over 30 min
iollowed by 1000 1500 units lV continuous infusion.
Clofted catlreter: i 0,000-25,000 units in normal saline,
the volume of the catheter instilled into catheter for t hr
I then removed (aspirated).

I
i
]
I

t
t
 I
ESSENCE OF PEDIATRICS

Sulfadoxine Malaria, in low MP density. Skin rdshes, bone marrnw suppression, .

(Combined with pyrimethaminer
Malacide, Sulphamin 525 mgl
tab (sulfadoxine 500 mg +
pyrimethamine 25 mg).
Sulfasalazine
Silazine,
Salazopyrin 500 mg tab.
25 mg/kg PO, single doie.
Anti-,inflamniatoryr 5 aminosalicylic acid derivative
combined with sulfonamide used in the treatment of
in{lammatory bowel disease, JRA;
lnirial 4Q-75 m{k{d PO divided q 4-6 hr not to exceed 6
g/d, maintenance 30*50 mglkgld PO divided q 6-B hr.
thrombocytopenia, crystallu ria, meg)aloblastic
anemia.
Rash, diziness, headache, bone marrow
suppre3sion.
Caution: Hypersensitvity to sulfa drugs.

leihutaline Sulfate Bronchodilator (beta-2 r6ceptoi agonist). .

BricanJ 0.5 mg/ml ampule.
Metered-dose inhaler (MDl):
0.25 mg/dose.
Tachycardia. arrhythmias, flushing, headache,
Children <12 yr: Oral 0.05 mg/kgldosd (max 5 mg) every Rerveusness, tremor, hypokalernia, muscle
I hr. crainps; paradoxical bronchospasm.
SC 0.005-0.01 mg/kfldose (max 0.4 mg), may iepeat in
l5 20 min.
Children >12 yr: Oral 2.5-5 mg/dose every 6-8 hr; SC
0.25 mg/dose, inay rbpeat in 15 min. :

MDI 1 *2 puffs every 6*8 hr as needed.

Testoslerone Androgen replacement in male hypogonadism and Acne, bladder i rritabil ity, aggressive behavior,
Andriol (40 mg/cap), delayed puberty (replacement therapy); depression, sleeplessness, headache, hirsutism,
Sustanon {250 mg/l ml amp' Male hypogonadism: lriitiation of prepubertal growth and hepatic dysfunction.
delayed puberty, 40-50 mglmYdose monthly; terminal
gronith phase, -l 00 mg/m:/dose twice.riro.
Teiinus Antitoxin (ATS) Prevention or treatment of letanus when tetanus immune Serum sickness, urticaria, skin eruptions,
1O,O00,lUlvial globulin unavailable. allergic reactions.
SC, IM
Prophylaxis: <30 kg, 1500 units; >30 kg,3000*5000 units.
Treatment: 40,000-1 00,000 units IV.
T€fhnris. lmmune Clobulin Prophylaxis and treatment of tetanus. Allergic reactions.
TIG 25Olu/l ml injdction Prophylaxis: 4 units/kg/lM.
Treatmentl 500=3000 units IM. i

Theoplrylline
lhenglaie;r 120 mg/5 ml syp.
Iheonate; 150 mg/5 ml syp,
30C mgAab.
Treatment of apnea of prematurity; symptoms of reversible
airway disease {affects iritracellular transport of calcium,
phosphodiesterase in hib itoi. weak anti-i nf lammatory).
Neonates:
Apnea, bronchodilation: Loading dose 6*10 mg/kg,
maintenance dose 2-4 m6/kg/dose every 12 hr. .
Tachycardia, nervousness, hyperact ivity,
difficu lty concentrating, irritabi I ity,
Cl upset, agitation, frequent urination, 5etzures
and arrhythmias at toxic level\.

lnfants and children:

6 wk-5 mo: 1O mg/kg/d.
6 mo 1 yr: t2- IB mg/kg/d.
1-12 yr:20aa m{kgd.
Thiamle Nutritional supplement, treatment of beriberi and. Cardiovascular collapse with repeated lV doses,
Beovit, Thiason Wernicke encephalopathy (essential conenzyme in angioedema, rash.
lnjectiOn: 100 mg/l ml amp. carbohydrate metabolismr.
Tabl*,100 mg. Beriberi: Children: lM, lV 1O-25 mg/d or
. .1
oral 10-50 mg/d for 2 wk, then 5-10 mg/d for 1 mo.
..
Werniike: lM, lV I00 nrld until consuming a balanced
diet.
Thioridazine Trealment o{ psychosis, neurosis. and severe behavior Pseudoparki nSon sm, tardiVe dyskinesia,
i

Melleril,
Tablet: 10, 25, 100 mg.
Tissue Plasminogen Activator, TPA
nitep!ase, Retevase
lnjection.
problems in children (phenothiazine, block dopamine dystonias, irnpaired temperature regulation,
reLeplors in the brainr. orthostatic hypotenSion, pigmentary
Children >2 yr: 0.5-3 mg/kgid in 2-3 doses. retinopathy, cholestatic jaundice, leukopenia,
Children >12 yr:25-800 mg/d in 2-4 doses. agranulocytosis. urinary retention, Cl upset.
hyperpigmentation.
Thrombolytic therapy (enhances conversion of
plasminogen to plasmin).
Bleeding, arrhythmias
(related post l st
Ml reperfusion).
\
Neonates: 0.1-0.5 mg/kg/hr for 3-1 0 hr Cl upset,
t
t
Children:0.1-0.6 mglkg/ hr for 6 hr
t
 \

DRUG THERAPY IN CHILDREN

Tolmetin Sodium Treatment of rheumatoid arthritis including.lRA Peptic urlcer disease, hypertension,.edeme; ,r,', '..
Tolectin (NSAlD, prortaglandin inhibition). dizziness; headache, relnal failure, tinnitus',' ': l',:

Tabler: 200, b00 mg Children >2 yr: 15-30 ng/k{d in 3 4 doses.

Capsule: 400 mg. Ana lg,esra: 5-l mB/k8/dose.

Tranexamic Acid Use in hemophilia patients during and following : Hypotension, thromboembot ic compl ications
Traxyl 250 mg Cap, tooth exlractions to reduce or prevent hemorrhage iincluding CNS), thrombocylopenia.
250 mg/5 ml amp (competitively inhibits activation of plasminogen).
.l
Tranex, 500 mg Cap, IV 0 mg/kg immediately before surgery;
500 mg/5 ml amp. then oral 25 mg/kg/dose 3-4 times/d for 2.B days.
Tretinoin Treatment of acne vulgaris. photo-damaged:skin (inhibits Excessive skin dryness, erythema, scating local
Retin-A, Relino-A, microcomedone formation and eliminates lesions). . ng ancl bu rn i ng photosensitivity (u se rsun.'::
sti Rgi

cream: 0.025olo, 0.05%, 0. l?o
Triamcinolone
Kenatort, 4 mg tab.
Kenalog. 4O mg/l ml amp,
0.1 7o cream. oinl.
MDI (Azmacorlr Nasal spray
(Nasacort)
Children >12 yr: Apply weaker formulation once daily at block), initial acne flare-up.
bedtime. lncrea5e as needed.
Treatment of inflammatory and allergic conditions Atrophy o[ tissue at local application site,
(corticosteroid r. fatigug'cataracts, osteoporosis, oral candidiasis
Children 6-12 yrz (tith MDI), poor growth:
lM 0.03-0.2 mg/kg every 1*7 days. MDl 2 puffs 2*4
-l
times/d: lntranasal: spray in each nostril l-2 times/d.
Injection: intra-articular, intrahursal .or tendon. sheath
2.5-.1 5 mg (repeat as needed). .

Children >12 yr:

MDI 24 puffs 2-4 times/d; Intranasal: 2 sprays in each
nostril once daily (max 4 sprays/d).
lntra-articular, intrasynovial: 2.54O m8;
Oral: 40-1 00 mgld in 1-4 doses;
Topical: Apply as thin film 2-3 timeld.
Triamterene Diuretic to treat edema or hypertension (competes with Constipatiorl, headache, fatigue; hyperkalemia;
Dezide icomhination of aldosterone for receptor sites in distal renal tubules). hyponatremia, hyperchloremic metabolic'
hydrochlorothiazide 25 mg + Oral 2*4 mglk$d in 1-2 doses (max 6 mg/kgld) acidosis.
triamlerene 50 mg)
Trientine Treatment of Wilson disease in palients intolerant to lron deficiency anernia, epigastric pain,
Syprine thickening and {issurlng of skin, i

Capsule: 250 mg muscle cramps, SLE.

Triflupeiazine
Telazine, Stelazine 1 mg, 6 mg tab
:nn:ir :r ;:. ;tffill ry: ir i-i t:::::
Treatment of psychosis (phenothiazine, btocks dopamine Hypotension, tachycardia, arrhythmias,
in the CNSr. pseudopaiki nscnism, tardive dyskinesia,
Children 6-12 yr: Oral 1 mg l-2 times/d, gradually dyston ias, constipation, dry mouth. mal ignaol.
increase to effecl {ma\ l5 m8/dr. hypertension.

Tromethamine Corrertion of metabolic acidosis (combines wilh hydrogen Apnea, hyrpoglycemia, hyperkaldmia, tissue '

Tham. ions to form tricarbonate and buffer). Correction of irritation, or hecrosis il direct coniacl
lnjection: melabolic acidosis:
0.3 M (1 mEq THAM = 3.J ml r Neonates, infants, children:
Dose (mL of 0.3 M solution) = Weight (kg) x base deficit;
or 1-2 mEq/kg/dose.
Tropicamide Shoft-acting mydriatic agent (blocks sphincter muscle Tachyqqrdia, drows.iness, headache, dry rnoqth,
Mydriacil of iris and ciliary bodyfrom respondingto cholinergic blurred vision, photophobia.
Ophthalmic solulion O.5"/o, 11o- stimulation).
Children and adults:
Cycloplegia: lnstill 1-2 drops l% solution, may repeat in
5 mrn.
Mydriasis: lnstill 1-2 drops of 0.5% solution 15-20 min
before exam.
Ursodiol, Ureodeoxycholic Acid Callbladder stone dissolution, reversal of TPN-induced Diarrhea, dyspepsia, biliary pain, rhinitis,
Actigal cholestasis in neonates (decreases cholestercl pruritus, headache.
Capsule 300 mg. content of bile).
Neonates: 10 15 mg/kg/d PO qd.
Infants: 30 ngkgd q 8-1 2 hr
t- Adults: 300 mg at bedtime {or 6-12 mo.
ESSENCE OF PEDIATRICS

Valproic acid Treatment of simple and complex generalized and partial Drowsiness, tremor, sensorineural hearing loss,
Valex, Epilex 200 mg tab. seizuies (blocks sodium and slow T channels). hyperammonemia, hepatotoxicity, Cl upse!
200 mg/5 ml syp. Neonates: pancreatitis, thrombocytopen ia, increased
Refractory seizures: Load 20 m$kg orally, appetite, weight gain.
then 1 0 mg/kgldose every/ 12 hr. Caution: Hepatic Iailure with fatalities have
Children' been reported, especially if patient <2yr or
Seizures: 10-'l 5 mg/kg/d in 3 doses, then increaseweekly receiving other anticonvulsants.
by 5-1 0 mg/kgld to effect or therapeutic levels.
Vasopressin Treatment of diabetes insipidus, acute CI hemorrha.ge. lncreased blood pressure, bradycardia,
Pitressin Diabetes insipidus: lM, SC 2.5-10 units/dose 2-4 times/d. arrhythmias, fever. Cl upset, tremor, swealing.
lnjection: 20 pressor units/mL. Cl hemoruhage: lV continuous infusion 0.002*0.01 circumoral pallor, water intoxication.
unirs/kg/min.
Vinblastine Sulfate Treatment of several cancers ibinds to mitotic spindle to Alopecia, abdominal cramps, stomdtitis,
Vinblastin 5 mg, 10 mgivial inhibit metaphase). myelosuppression ionset 4-7 days, nadir
2.5 mg1m'? [V increased by 1.25 mg/m, weekly 4-1 0 days, recovery 17 days), orthostatic
to a maximum ol 7.5 mg/m2. hypotension, dermatitis, photosensitivity,
Hodgkin disease: lV 2.5*6 mg/m2/d (max 12,5 mgim:/wk). muscle pain, urinary retention, hyperuricemia,
periphera I neuropathy.
Vihcristine Treatment of various cancers (binds to mitotic spindle to Constipation, paralytic ileus. optic atrophy,
Vincristin (0.5 mg, 1 mg/vialr inhibit metaphaser. blindness. peripheral neuropathy, SIADH,
Vincristine {1 mglvial). 1.5-2 m{mr lV weekly. photophobia, hyperuricemia, stomatitis,
Anti-cancer drugs: phlebitis, myelosuppression (onset 5 days, nadir
1. Alkylating agents: 10 days. recovery 2 I daysr.
Busulphan, chlorambucil.
ryclophosphamide. melphalan.
mustin HCl.
2. Antimetabolites:
a; Folic acid Antagonisl:
Methotrexale
br purine antagonist:
Mercdptopuri ne. azathioprine,
th ioguanine
c,) Pyramidine analogue:
5 flurouracil, cltarabine
J. Natural, semisynthetic agents:
a; Alkaloids: vincrisline,
vi nblasti ne
b) Antibiotics: actinomycin,
bleomycin, mitomycin,
daunorubicin, doxorubicin
ci Asparaginase: L-asparaginase
dr Clucoside: Etoposide
e) Platinum derivatives: cisplatin
fl lnter{erons
g) Miscel laneous-Procarbazi ne,
hydroxyurea
Vitamin A Treatment or prevention of deficiency. lrritabi ity, verti go, lethargy, tev er, headache,
I

Retinol Forte {50,000 lU/cap) Dose hypercalcemia.

Vitavit 1200,000 lU/capt.,
50,000 lU/lab. Age Vitamin A
9 mo upto 12 mo 1 lakh units
12 mo up to 5 Yr 2 lakh units
Cive 3 doses (on d-1 , d-2, & d-l4) for corneal opacity;
2 doses (on d-l & d-2) for measles & 1 dose for each of
persistent diarrhea or severe malnutrition.
Vitamin E

E-Cap ('l 00 mg/Cap)

Nutritional supplement {antioxidant), Rare \
Neonates, premature infants: 25-50 units/d:
Evit (200 mg/tab). I
Children: 1 uniVkg/d; sickle cell anemia: 450 units/d;
cystic fibrosis: 100-400 units/d.
\
,l

I
 I

DRUG THERAPY IN CHILDREN

Warfarin Anticoagulant that antagonizes hepatic vitamin K synthesis Bleeding, skin necrosis, hemoptysis
Farevan (5 mg/tab) depleting vit K-dependent clotting factors: ll, VII, IX,
Marevan (1 mg, 3 mg & 5 mg/tab). and X. lnitial dose 0.2 mg/kg once PO then usual dose
approximates 0.1 mg/kg/d PO. Dose titrated to desired PT
and INR target..
Xylometazoline Symptomatic retief of nasal congestion (stimulates alpha- Palpitations, headache, dizziness, drowsiness,
Antazol, Rhinozol nasal drop adrenergic receptors to produce vasoconstriction). sweal ing, blurred vision.
Nasal solution: 0.05"/,', 0.1 "/. Children 2-12 yr: lnstill 2-3 drops 0.05'/o solution in each Cdulion: Do not use lor more lhan 4
Antazol nasal spray. no\lril evcry B-10 hr. conseculive days or il may cause rebound
Children >12 yr: lnstill 2-3 drops 0.17o solution in each congestion.
nostril every B-'l 0 hr.
Zafirlukast Leukotriene D4 and F4 dnl.rgonirl inhibiting the efler t Headache, nausea, dyspepsia, elevated liver
Freesy, Zafir 10, 20 mg tab oi slow rea, tive subslJnLerst ol anaphylaxi'SRS-Ar on lunclion Ierts.
bronchial smooth nruscle. Not effective in reversing acute
bronchoconstriction, though therapy can be continr-led in
.rcr.rte atlacks.
Children >7 yr:20-40 mg/d PO divided q 12 hr.
Administration of zalirlucast with food increases
hi,,ar ailabilitl b; r. mu, h ar 40o6.

Zinc lrevenlit-rn and treatmenl ot zinc delir ienr ). Rare, but if excessive doses are used may cause
Xinc (10 mg/5 ml syp) lnfants and children: 0.5 1 rrg/kg/d in 1-3 doses. cupper deficiency.
Pep2 (10 mg/5 ml syp)

Amikacin Sulfate
Kacin, 'l 0O mg/2 ml &
500 mg/2 nrl injections.
Aminoglycosides:
Centam ici n, streptomvci n,
kanamycin, neomycin, tobramycin,
nelilmicin, .rmika, in. spc( linom\ ( in.
framycetin.
Amoxicillin
Fimoxyl, Moxacil.
250 mg, 500 mB cap;
125 mg/5 ml susp;
125 m{1.25 ml drop;
250 mg, 500 mg/vial.
Amoxicillin-Clavulanate
Moxaclav, Fimoxyclav.
375 mg (Amox. 250 mg + CIav.
.l
25 mg) tab; 625 mg (Amox. 500 mg
.l
+ Ciav. 25 mg) tab; 156.25 rng/5 ml
(Amox. 125 mg + Clav 31 .25 mg)
Fimoxiclav injection: 600 mg/vial
(Amox. 500 nrg + clav. 100 mg),
1 .2 {vial (Amox. 1g + clav. 200 n'rg).
Ami noglycoside antibiotic effective aga inst gram-negative See under gentamicin.
bacilli, esp. Pseudomonas, Proteus, E. c-di. Klebsiella,
EnLerobacteria.
Neonates: lM, lV (over 30 60 min) Postnatal age =7
days-1 200 2000 9,7.5 mg/kg q 12-1 B hr; >2000 g,
10 mskg q 12 hr. Postnatal age >7 days-1 200 2000 g,
7.5 mg/kg q 8*1 2 hr; >2000 g, 10 mg/kg q B hr.
Children: I5-25 rng/kgd divided q 8-12 hr.
Strcplocurt aI phrrl neilis. pneumoni.-t See under penicillin C, diarrhea, abdominal
20-50 mg/kg/d PO in 3 clivided doses; 50-1 00 mg/kg/d .ra mps.
lM, IV in three divided doses in serious infections. Higher
dose 80 90 mg/kg/cl for otitis media.
Enteric fever: 1 00 rng/kg/d PO in three divided dose.
Endocarditis prophylaris: 50 mg/kg t hr beiore dental,
oral, or respiratory surgical procedure.
Bela-lar lam ramorir i Il irrt bet.r-l.rL rdm,r)e inhibitor See under ampicillin, hepatitis, cholestatic
,t lar. ulanale, enhan, e: amoricillin rr livilv againrl jaundice, diarrhea.
penici I I i nase-producing bacteria: S. au reu s, Stre ptococcus, Drug dose on amoricillin Lomponent.
H. influenzae, M. catarrhalis, E. coli, Klebsiella, B. fragilis.
Neonates: 30 mg/kg/d PO divided q 12 hr.
Children: 20-a5 mg/kg/d PO divided q B-l2 hr. Higher
dose 80-90 mg/kg/d for otitis media.

Ampicillin Sanre spectrr:m oi antibacterial activitv as amoxicillin. Diarrhea, rashes (discontinue treatment),
Ampicin, Ficillin. Neonaies: antibiotic associated colitis (see also under
.1
250 nrg cap; 25 mg/5 ml susp; Po:'tt.'tal .5- - rl,rr,- - jl)00 S lor 'ep-i' bcnz; I penrt illin'.
125 mg/1.25 ml drop, 250 mg, nreningitis 100 nrgrkgicl clivided q 12 hr;
500 mg/vial >20{J0 g rrreningitis 150 mgkgrd divided q B hr.
Postnatal age >7 da1,5 <2000 g ior sepsis,
meningitis 100 1 50 mgr'kg/d divided q B hr; >2000 g for
sepsis, meningitis 200 mg/kg/d divided q 6 hr.
Children: 100-200 mg/kg/d lM, lV divided q 6 hr;
meningitis 200-400 nrg/kg/d divided q 4-6 hr.
ESSENCE OF PEDIATRICS

Azithromycin Azilide antitriotic with activiqr agailits. aureus, Same under erythromycin, inteistitial nephritis,
Aa1h, Zimax 250 mg Cap; Str.eptococcus, H - i n fI u e n z ae, M y.cop I as m a, C. ARF, hepaiitis.
500 mg tab; 200 mg/5 ml susp. tracho m atis., Legionel I a.
Macrolides: 10 mglkg PO on day 'l (max 500 mg) fr:llowed by
1. Erythromycin (against gram- 5 mglkg PO qd for 4 days. Suspected sffep infection I 2
positive bacteria, mycoplasma, mg/kg/d (max 500 mg) for 5 days.
chlamydiae. Legionellar
2. Chlarithromycin (against gram-
positive organisms).
3. Azithromycin (against gram-
positive, H. influenzae,
N. gonorrhoeae. S. typhi,
Chlamydiaet.
4. Spiramycin
Carbenicillin Extended-spectrum penicillin susceptible to penicillinase Painful .by lM, rash, interferes with platelet
Pyopen lg powder/vial destruction/ active against Pseudomonas, Enterobacter, aggregatiori/ increases in liver function tests.
Proteus. See also under Benzyl penicillin.
Neonate: Postnatal age <7 days*<2000 g, 200 mgkg/d
lM, IV divided q B hr; >2000 g, 300 mg&gid divided q 6 hr;
>7 days,300-400 mgkg/d divided q 6 h1.
Children: 400-600 mg/kfd lM, lV divided q 4-6 hr.
Cefaclor Second.generation cephaloiporin, active against 5. aureus. Dif{erent types of rashes, anaphylaxis, Cl
Biocef, Loracef 250 mg, 500 mg cap. Stteptococcus, Pneumococci, H. iifluenzae, E. coli, upset, diarrher. t:"ansient liepatitis, cholestatic
125 m{5 ml susp. Proteus. Klebsiella. jaundice, !i:. . r!ioallopenia, aplastic anemia,
Classification of the cephalosporins: 20aO mg/k{d PO,divided q 8*.1 2: hr (max dose 2 g). interstitial nepiri ;ti:, hyperactivily, sleep
First generation: disturbances.
Cephalexin. Cephradin, Cefadroxil,
Cephalothin.
Second generation:
Oral: Cefaclor. Cefuroxime axetil,
Ce[uroxime
Parenteral: Cefamandole, Cefoxitin.
Third generation:
Oral: Cefixime, Cefpodorime,
Cefetamet pivoril
Parenteral : Cefotaxime, Ceftriaxone,
Ceftazidime.
Fourth generalion:
Parenteral: Cefepime iniection.

Cefadroxil First-generation cephalosporin, active agai nst .5 . du re u s i See under Cefaclor.

Adora. Arocef 250 mg, 500 mg cap;
125 mgi5 ml susp.
Cefepime
Maxipime
ln jec lion.
Cefixime
Cef-J, Roxim. 200 mg cap
100 mg/5 ml susp.
Cefotaxime sodium
Maxcel Taxim. 250 mg,
500 mg 1 g/vial
Streptocaccus, E. coli, Proteus, Klebsiella.
3Q mgikgld PO divided q12hr.
Expanded-spectrum, fourth-generation cephalosporin, Rash, eosinophilia.
active against many gram-positive and -negative
pathogens including many multidrug,re$istant parhogens.
100-1 50 mgikg/d rV, lM, q 8,12 hr.
Third-generation cephalosporin, active against See under Cdfaclor
; H. i n fI u e n z ae, M. catarr h al is,
Str e pto coicc u s,
N. gonarrhoeae, Salmonella, P vulgarls. No
antistaphylococcal or antipseudomonal activity.
B mg/kg/d PO divided q12-24 hr.
2A mglk{d PO divided q12*24 hr in enteric fever
Third-generation cephalosporin, ac[ive against gram- See under cefaclor, rarely arrhythmias following
positive and -negative pathogens. No antipseudomonal rapid injection.
activity.
Neonates: <7 days, 1 00 rnglkg/d lM, lV divided q 12 hr;
>7 days,1 50 mg/kg/d divided q 8 hr.
Children: 150 m{kSd lM, IV divided q 6-8 hr \
(sepsis, meningitis 200 mlkgld tV divided q ffi
hr). t

t
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Cefpodoxime proxetil
Taxetil, Ximeprox.
Suspension: 40 mg/5 ml
Capsule: 100 mg.
F
t-
b Ceftazidime
). Tazid
Fortum 250, 50o mg, 1 g/vial-
t IV divided q12 hr; >7 days, 150 mglkgld divided q
I Children: 150 mglkgid IM, lV divided q B hr.
L
F
Ceflriaxone Sodium
Traxon, Ceftron 250 mg,
500 mg, 1 fvial.
Ce{uroxime
Zinacef 750 mg/vial
Zinnat, Axim 125'm& 250 mg
125 mp/5 ml susp.
Furex 125 mg15 ml susp.
Cephalexin
Ceporex, Acelex,250 mg 500 mg
cap; 125 mg/5 ml susp;
125 m{1.25 ml drop.
Cephradine
Sefrad, Lebac
250 mg, 500 mg cap;
125 mg/5 ml susp;
125 m{l.25 ml drop,
250 mg, 500 mg/vial
Chloramphenicol
Ramex, Fionicol 250 mg, caP,
125 mg5 ml susp;
Medophenicol t givial
A-phenicol,
SQ mycetin 0.5% eYe droP, lolo oint.
Ciprofloxacin HCI
Neofloxin, Ciprocin 250 mg,
500 mg, 750 mg tab,
200 mg/100 ml infusion.
Cipror, ciprocin eye drop, oint.
4-fluoroquinolones:
Ciprofloxacin, ofloxacin,
norfloxacin, sparfloxacin, pefloxacin,
Ievof loxacin, lamefloxaci n.
Third-generation cephalosporin, active' against 5. aureus.
Streptacaccus, H. influenzae, A coli; N. gonor\hoeae,
Klebsiella, Proteus. No antipseudomonal activity'
Children:10 mglkgd PO divided q!2 h1.
Decrease dose in renal insufficiency.
Third-generation cephalospori n, active against graqr:
positive and -negative patho$ens including
P seudomon as aerugi nosa.
Neonate: Postnatal age <7 days, 100 mg/kg/d lM,
(Sepsis, mdningitis 150 mg/k/d lV divided q B h$.
Third-generation cephalospori n, active against gram-
positive and -negative pathogens. No antipseudomonal
activity. Very potent and beta-lactamase stable.
Neonate: 50-75 mgkg lM, lV g 24 hr
Children: 50-75 mglkg lM, lV q 24 hr
(meningitis 80*1 00 mlkgid divided 912*2a hr!-
Second-generation cephalosporin, aative against 5. aureus,
Streptococcus/ H. influenzae, E. cali, M. catarihalis,
tab-; ProLeus. Klebsiella.
Neoriates: 40-1 00 mlkgld IM, lV clivided q 12 hi'
Children: 2O014o mg/kg/d IM, lV divided q 8 hr;
oral administration 20-30 mg/kgid divided q I hr.
First-generalion cephalosporin; active against 5. aureus,
Streptococcus, E. cali, Proteus, Klebsiella.
2s-l00 n{k{d
PO divided q 6-8 hr.
f irst-generation cephalospori n, active aga i nst S.
Streptococct!s, E. cr:!i, P roteus, Klebsiella.
50-100 mg/kgld PO divided q 6*12 hr.
Broad-spectrum protein synthesis inhibiror, active against
many gram"positive and -negative tracteria.
Rickettsi a, Cht amyd i a, M ycopl asm a, S al monell a,
Bacteroides; anaerobes, Pseudornonas' usually'resistant:
Neonates: lnitial loading dose 20 rnglkg{ollowed 12 hr
later by:
Postnatal age <7 days, 25 mg/k6/d lV q 24 hr;
>7 days and 2000 9 25 mglkgld lV q24 hr;
<2000 g, 50 m51kg,rd lV divided q 12 hr.
'
Children: 50-75 mgikgld lV, PO divided q 6-8 hr
(meningitis 7s-1o0 m$k/d lV divided q o hO-
Quinolone antibiotic active against P aeruglnosa,
E nter obacte r spp., S h i ge I I a, S al m o n e I Ia, C am py I ab acter,
N. gonorrhoeae, H. influenzae, some Staphy/ococcus and
streptococcus spp.
1s mflkgid.
DRUG THERAPY IN CHILDREN
CI upset, colitis
rash,,eosirioph ilia
. lncreasbd bioavailability when iaken with food.
See under Ceiaclor.
I hr.
See under Cefaelor; calcium-ceft riaxone
precipitates in urine or'irr gallbladder (Sludge)-
consider discontinuation if symptomaiic.
Prolongation of prothrombin time, pancJeatitis.
See under C€faclor.
.See under Cefac.lor.
au r e u s, See under Cefailor
Blood disorders including reversible and
irreVersible aplastic anemia (1 in 4Q0O0) with
reports of leukemia, Crey baby syndrome
(abdomen distension, pallid cyanosis,
circulatory tollapse); in 1 959. Sutherland ,
described 3 infants who died of Crey'baby
syndrome after reqeiving 200 mgikg of l
chloramphenicol daily. Peripheral neuritis, skin
rashes, Cl.upse! stomatitis.
Quinolones cause arlhropalhy in the weight
bearing joints of immature animals and are
therefore not recommended in children.
However, the significance of this effect in
humans is uncertain and in some speciiic
circumstances, short{erm use oI quinolone
in children may be jusrified. Nalidixic acid
is used for UTI in children over 3 mo of age
and ciprofloxacin is licensed for pseudomonal
infections in cystic fibrosis for children >5 yr
of age.
Arthropathy, hepatitis, nephritis, ARF. Ct upset
rashes, erythema nodosurn, tenosynovitis, blood
disorders.
ESSENCE OF PEDIATRICS

Clarithromycin Newer macrolide antibiotic with activity against See under erythromycin.
Claricin, Rolacin. S. aureus, Streptococcus, H. influenzae, Mycoplasma,
250 mg, 500 mg tab C. trachomatis. t egionella-
Klaricid 125 mg/s ml susp, 15 mg4€/d PO divided q12hr.
500 mSvial.
Clindamycin Protein synthesis inhibitor, active against most gram- Antibiotic associated colitis (stop treatment),
Dalacin-C 150 mg Cap, positive aerobic and anaerobic cocci (not Enterococcus). Cl upsel, jaundicg agranulocytosis,
75 m$5 ml susp. Neonates: Postnatal age <7 days, t 0-l 5 mg/k$d lM, neutropenial thrombocytopen ia, rashes.
IV divided q t2 hr.
>7 days, 10,1 5 mglkgid lM, tV divided 12 hr.
Q
Children: 10-40 mg/kg/d IM, tV, PO divided q 6-8 hr.
Cloxacillin sodium Penicillinase-resistant penicillin effective against S. aureus See under Penicillin C.
Ficlox, Cloxin. 250 mg, 500 mg, and other gram-positive cocci except Entercoccus and Hepalitis, cholestatic jaundice.
125 mg15 ml, 125 mg4 .25 ml drop; coagu lase-negative staphylococr i.
250 mg, 500 mg/vial. 50-100 mg/kg/d PO divided g 6 hr.
Co-trimoxazole Sequential antagonism oi bacterial {olate synthesis Rashes (StevensJohnson syndrome, toxic
{trimethoprim-sul{a methorazole} with broad-spectrum antibacterial activity: S h iget t a, epidermal necrolysis, erythema multiforme),
Cotrim, Fisat, 480 mg lab, and DS Pne u moclr stis c ari n i i, Legione a, Ch I amyd i a.
11 blood dyscrasias {myelosuppression,
960 mg tab, susp: sulpha 200 mg. 6-20 mgTMPlkg/d PO, divided q12hr. agranulocytosisr, hepatitis, colitis, dseptic
TMP 40 mg/5 ml. Pneumocystis: 15-20 mg TMP/kg/d PO divided q 12 hr. meningitis, megaloblastic anemia due to
Pneumocystis prophylaxis: 5 mg TMP/kg/d or 3 times/wk. trimethoprim, crystalluria and renal failure.
Dapsone Leprosy Peripheral neuropathy, dermatitis, hepatitis,
Dapsone. Avlosulphone 50 mg, 1-2 mg,/kg/d. psychosis, agranulocytosis, dapsone syndrome
100 mg tab. (rash, fever, eosinoph ilia), hemolltic anemia.
Doxycycline Tetracycline active against most gram-positive cocci The tetracyclines are deposited in growing
Doxatil, Doxicap. 100 mg cap. (except fnterococcus), Vibrio cholerae, many gram- bone & teeth (being bound to calcium) causing
negative bacilli, Mycoplasma, Barrelia burgdorferi (Lyme staining & occasionally dental hypoplasia &
disease), C hlamydia, anaerobes. should not be given to children <12 yt or to
Children: 2*5 m{k{d PO, divided q 12-24 hr pregnant or breast-feeding women.
(max dose 200 mg/d).
Cl upset, diarrhea, erythema, benign
intracrania I hypertension, hepatotoxicity,
pancrealilis.
Avoid in porphyria.
Erythromycin Bacteriostatic macrolide most active against gram-positive Cl upset, urticaria, reversible hearing loss,
Eromycin, Etrocin 250 mg, organisms, M. pneumoniae, C. diphtheriae, B" pertussis. cholestatic jaundice, arrhythmia.
500 mg tab. Neonates: Postnatal age <7 days, ZO mg/k9/d pO divided
125 mg/5 ml syp. q12 hr; >7 days,20-30 mglkg/d divided q B-i 2 hr.
Children: 30-50 mgikgld PO divided q 6*8 hr.
Flucloxacillin Cram-positive infections including infections caused by Mild Cl uFiset, skin rashes. See under penicillin.
Phylopen, p-lactamase produci ng staphylococci 1 2.5-2 S mgkg
Staphen 125 milS ml syp, 250, 4 times dailv.
500 mg Cap.
Gentamicin Aminoglycoside anribioti c effective against gram- Vestibular & auditory damage, nephrotoxicity,
Cenacyn, Centin 20 mg, negative bacilli, especially Pseudomonas., prateus, E. coli, CI upse! colitis.
80 m!2 ml amp. Klebsiel I a. En Leroba c t ers.
Neonates: lM, lV (over 30*60 min) Postnatal age <Z days-
1 200-2000 g, 2,5 mg/kg q 12*18 hr;

>2000 g, 2.5 m{kg q 12 hr. Postnatal age >7 days-

1200 -2000 g, 2.5 mg/kg q B-l 2 hr; >2000 g, 2.5 mg/kg
qBhr.
Children: 2.5 m{kg 8-12 hourly.
Metronidazole Highly effective in the treatment of infections due to Cl upset, unpleasant (metallic) taste, rashes
Filmet, Flagyl 400 mg, 200 mg lab; anaerobes- (urticaria, anaphylaxis), ataxia, peripheral
500 mg/l 00 ml bottle, Postnatal age <7 days: 1200-2000 g,7.5 mg/kgld pA, neuropathy (on prolonged therapy), convulsion.
20O mg5 ml susp, lV q 24 hr; 2000 g, I 5 mg/kg/dPO, lV divided q 12 hr. F
Flagyl suppository 500 mg. Poslnatal age >7 days: 15-30 mg/kg/d PO, lV divided
q 12 hr. I
Children: 30 mg/k{C, PO, lV divided q 6-8 hr. i

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 DRUG THERAPY IN CHILDREN

Mupirocin Topical antibiotic effective against staphylococci and Minimal systemic absorption as drug
Bat troban. streptococci. metabt-rlized within the skin.
2ozo ointment. Topical application: Nasal (eliminates nasal carriage) and
to the skin 2*4 times per day.
a
r Nalidixic Acid First-generation qu i nolone effective for short-term See under ciprofloxacin.
Nalid, Naligram 500 mg tab; treatment of Shigellosis & lower urinary tract infections Psychosis, increased intracranial pressure,
f 300 mg/5 ml susp. caused by E. coli, Enterobacter, Klebsiella, Proteus. cranial nerve palsy.
Children: 50-55 mglkg/d PO divided q 6 hr; suppressive
therapy-25-33 mg/kg/d P0 divided q 6-8 hr.
t
Neomycin Sulfate Aminoglycoside antib iotic used for topical appliiation or Primarily relaled to topical application,
II Neomycin j50 mg cap. orally in FHF & prior to surgery to decrease gdstrointestinal abdominal cramps, diarrhea, rash.
F
ilora (nonabsorbabler and hyperammonemia. Aminoglycoside toxicilies if absorbed.
lnfants: 50 mglkg/d PO divided q 6 hr.
Children: 50-1 00 mgikgld PO divided q 6-8 hr.
Netilmicin Septicem ia, gra m-negative i nfecl ion resistant to See under genlamicin.
Netromycin 300 mg/1.5 ml, gentamicin.
4O0 m{2 ml, 500 m{2 ml. Neonate (up lo I wk): 3 mg/kgldose l2 hrly.
Infant (over 1 wk): 2.5-3 mg/k$dose 8 hrly.
Children: 2-2.5 m{kSdose B hrly'
Nilrofurantoin Effective in the treatment of lower urinary tract infections Vertigo, jaundice, interstitial pneumonitis, Cl
Furadantin, FurAnamine 50 mg, caused by gram-positive and -negative pathogens, upset, didrrhea, peripheral neuropathy, rashes,
100 mg tab. 5-7 m{kgld PO divided q 6 hr (max dose 400 mg/d); hepatitis, cholestatic jaundice, Lr lood dyscrasia,
suppressive therapy.l-2.5 m{k{d PO divided q1)-24hr i ntracra nia I hypertension.

(max dose 100 mg/dt.

Ofloxacin 15-2O m{k{d 12 hourly PO. See under ciprofloxacin.

Rutix, Oflacin 200 m9,400 mgtab. Tachycardia, transient hypolension, tremor,
unsteady gait, neuropathy, extrapyramidal
symptoms, psychotic reactions (discontinue
treatment).

Penicillin G, Benzyl penicillin
Penicillin C sodium, Pen-C 5 lac.
l0 lat:/vial.
Penicillin G, Benzathine
Benzapen, Diamine Penicillin 6 lac,
12 lac per vial.
Penicillin C, Procaine
Seclopen, Pronapen 4 lac units/vial
Penicillin V
Pen-V, Penvik 250 mgtab;
125 mgl5 ml susp.
Active against most gram-positive cocci; pneumococci Rash, eosinophilia, hypersensitivity reaclions
tresistance escalatingt, group A S. viridans and some (urticaria, anaphylaxis, serum sit kness-like
gram-negative bacteria tN. gonorrhoeae. N. meningitidisr reactions), hemolytic anemia/ interstitial
Neonatei: lM, IV Postnaral age <7 days- I 200-2000 g for nephritis, diarrhea, bleeding diathesis,
sepsis, meningitis 100,000 unitslkg/d divided q 12 hr; convulsion (with high dose).
>2000 g for sepsis, meningitis 150,000 units/kg/d divided
q B hr. Postnatal age >7 days -<1200 g for sepsis,
meningitis 100,000 unitsikg/d divided q l2 hr;
1200-2000 g {or sepsis, meningitis 225,000 unitvkg/d
divided q B hr], >Z-OpO g for sepsis. meningitis 200,000
units kg/d lV divided q 6 hr.
Children: 100,000-250,000 units/kg/d lV, lM divided q
4 6 hr (up to 400.000 unitslkgidi.
Long-acting penicillin effective in the treatment of See under penicillin C.
infections responsive to persistent, Iow penicillin Traumalic neuritis, if faulty technique is
concentralions {1-4 wk), e.g., strep pharyngitis, adopted in giving lM inj.
rheumatic fever prophylaxis.
300,000-1 .2 million units/kg lM once every 3-4 wk,
mar. I .2 2.4 million unils/dose.
Repository form of penicillin providing tow penicillin See under penicillin C.
concentrations for -1 2 hr. Not for lV administration.
Neonates: > l 200 g, 50,000 units/kg lM qd.
Children: 25,000 50,000 unitslkg/lM qd for 10 days,
max. 4.8 million units/dose.
Active against most gram-positive cocci; pneumococci See under Penicillin G.
(resisiance escalating), other streptococci and some
gram-negative bacteria, N. meningitidis),
25-50 mglkgld PO divided q 4-B hr.

I
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ESSENCE OF PEDIATRICS

Pentamidine lsethionate Antiprotozoal agent effective in the prevention and Hypotension, hypoglycemia, cardiac
Pentam, Penlacarinate 300 mg -treatment af Pneumocystis carinl infections, VL arrhythmias, nephrotoxicity; Ieukopenia,
powder vial. Children: P calinll treatment 4 mg/kg/d lM, IV {preferred) thrombocytopenia, cardiovascular collapse
qdfor l4days. when used lV.
Prophylaxis: a m{kg lM, lV every 24 wk.
Visceral leishmaniasis, a mg/k{d deep lM 3 times a wk for
s wk (1 s inj).
Piperacillin Sodium lxtended-spectrum penicil Iin active against See under penicillin C.
Pipracil Enterobacter, E. coli, Bactercides spp., P. aeyugjlosg Renal impairment, severe drug rashes.
lnjection. Neonates: Pestnatal age <7 days, 150 mg/k/d lV divided
q B-12 hr; >7 days, 200 mg&g/d divided q 6-B hr.
Children: 200-300 mg/kgld divided q 4-6 6y
Cystic fibrosis 350-500 mg/kg/d lV.
Pivmecillinam In shigellosis 40-50 mg/k/d in every 6-8 hr PO tM, IV. See under Penicillin C.
Selexid, Alexid 200 mg tab, Vomiting.
400 mg/vial
Roxitfuromycin Against atypical & typical pathogens of URTI & LRTI, Low. Cl upsel, rashes, tinnitus, vertigo.
Pedilid 150, 30O mgtab, skin infections.
50 rtrl susp. 2.5-5 mg/kg twice a day for 5-1 0 days.
Sulfadiazine lndicated for the treatment of Toxoplasmosis. ABranulocytosis {0. 1 %), aplastic anemia,
Sulphadiazine ' Neonates: t 00 mglkgid PO divided q 12 hr with thrombocytopenia; crystalluria, skin rashes
500 mg talr. pyrirnethamine 1 mglkg/d PO qd (with folinic acid). ( 1 .5%), urticarial, petechia I rashes,
erythema
Children: 120*200 mg/kg/d PO divided q 6 hr wirh nodosum; Stevens-Johnson syndrome,
pyrimethamine 2 mglkg/d PO divided q 12 hr x 3 days exfoliative dermatitis, hepatic necrosis (0.I %),
then 1 mg/kg1d (max dose 25 mgld) with folic acid. hemolytic anemia (0.05%).
Rheumatic fever prophylaxis: <30 kg, 500 mg/d;
.l
>30 kg, g/d PO qd.
Spiramycin Used in URTI and LRTI. Cl upset, skin rashes (rare).
Rovamycine, 1.5 MIU tab, >7 years (>20 kg): 1.5 MIU BD
3 MIU tab. <7 years (1 0-20 kg): 112 o'i 1.5 MIU BD
.1.5
lnf6nr (<10 kg): ltath of Mtu BD.
Tobramycin Am inoglycoside a ntibiotic effective aga inst gram-negative Vestibu I ar damagen reversitrle nephrctoxicity,
Brulamycin BO m{2 ml amp. bacilli, e.9., Pseudomonas, Proteus, E. coli, Klebsiella, rnood disorder, vonriting, altered liver function
Tobracin eye drop and oint. Interobacter. tesl.
Neonates: lM, lV (over 30-60 min)*Poitnatal age < Z
days, 2.5 mdkg q 12 hr; postnatal age >Z days, 2.5 mg/kg
qB l2hr.
Children: 2.5 m{kgld divided q B-12 hr,
Trimethoprim Folic acid antagonist effective in the prophylaxis and Megaloblastic anemia, bone marrow
Prolopiim, Trimpex, treatment of E- coli, Klebsiella, Proteus and Enterobacter suppression, naurea, epigastric distress, rash.
Tablet 100 mg, 200 mg. spp. urinary lract infeclions.
P ne u moc y sti s carlnll pneumon ia:

Children: Fqr UTI 4-6 m{kg/d PO divided q 12 hr

P neu.mocystis carinri pneumonia (with dapsone):
15*20 rng/kgid PO q 6 hr for 21 days.
Vancomycin Clycopeptide antibiotic etfective dgainst mosi gram-
Vancocin, Luphocin. positive pathogens including staphylococci (methicillin-
Iniection. resistant 5. aureus and coagu lase-negative staphylococci
)
Capsule: 125 m9,250 mg. and enterococci (resistance developi ng), c lostri dia,
pneumococci i ncluding pen ici li n-resistant strains.
I Cautions: Ototoxicity, nephrotoxicity
Neonate: Postnatal age <7 days, 15-30 mg/kg/d
lV divided q 24*12 hr; postnaral age >t days,
1 5-45 mg/kg/d lV divided q24-B hr;
Children: as-60 mflkg/d lV divided q B-12 hr;
oral dosi ng for antib iotic-associated enterocolitis,
a0-50 mg/kg/d PO divided q 6-8 hr.
Nephrotoxicity. i ncluding renal failure,
ototoxicity, blood disorders (neutropenia,
thrombocytopenia, drug rashes, shock, red-man
syndrome (flushing of upper body).
particularly when coadminislered with other
oto- and nephro-toxins, in{use lV over 45-60
min., flushing (red-man syndrome) associated
with rapid lV infusions. fever, chills, phlebitis
(prefer central linet.
:,

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DRUG THERAPY IN CHILDREN

Ethambutol HCI
Fiambutol, Sural 400 rng tab.
lsoniazid
INH, lsotab 100 mg, 300 mg tab.
For use in combirration with other agents. Rerrally
elrrninated.
15 rng/kg/d PO single dose. At 25 mg/kg/d shows
bactericidal effect.
For use in combination with other agents.
10-20 mg/kg/d PO, max dose 300 mg/d;
prophylaxis dose 10 mg/kg/d.
Optic neuritis, red/gree n color blindness.
decreased visual acuity, headache, dizziness,
ra.h. peripheral nerrronrlhv.
Caution: Check visual acr.rity every 1-2 month
Avoid in yor-rng children as ophthalmological
examination is diificult.
Dizziness, seizures, rash, Cl upset, peripheral
neuropathy (may give 8,, 1-2 m/kg/d without
effect on anti-TB activity), hepatotoxicity.

Kanamycin Aminoglycoside antibiotic used in combination with See under gentamicin.

Kanacyn, Kantrex 500 nrg
1glvial.
Pyrazinamide
PZA-ciba, Firazin 500 mg tab.
Rifampicin
Firifam, C-Rifampicin 150 mg, 450
mB cap; Rircin lO0 mgl5 rnl .Lrsp.
Streptomycin
Streptomycin 1 g/vial.
other agents.
.1
15 mg/kg day lN4, IV divicleci q 2 hr.
For use in combination with othe r agents. Photusensitir il1 . Cl up.et, h1 peruritemia.
15 a0 mg/kg/d PO divided 1112-24 hr arlhralgia. Irepatoloxicity
'm,rr dose I g/dr. (especially doses >30 mg/kg/d).
l or rrre in t onrbinuli,,n r,r ilh ollrer lgenl'. Hepatolorit i1y, intluenza-likc svndronre, ra:lr,
I0-20 mgzkgrd PO. prLrrituc leukopenia. arthralgia. md) cause
orange-red dis, uloration of Lrrine. lears, )$eal.
Aminoglycoside antibic'rtic used in combination with See under gentamicin.
other agents.
\eonatc: 1U-20 rng lgd lrul.
Childrerr:20-40 mg/kg/d lM clivided q 12 hror
once daily not to exceed 1 g/cl.

Amphotericin B Polyene effective agai;:s1 a brciad spectrum of fungi: Nephrotoxicity, Cl upset, hepatotoxicity
Fu ngizone Carrdida, Aspergi I i us, Corciciicicies, H istoplasma, dyselectrolytemia, cardiotoxicity, blood
ln.jection. Blastornyces. disorders, neurotoxicity, rashes.
Ch!ldren: 0.'1-0.25 mg/kg initial dose.
Maintenance dose: 0.5-1 rng/kg/d
infused lV over 4-6 hr q day.
Liposomal ampholericin B 2 my,/k{d 3 injections for drug-resistarrt kala-azar
Ambisome ln.j.
Clotrimazo!e Topical imidazole active against cryptococci, Aspergillus, Nausea, skin irritation.
Canesten, Clotrinr creanr Candida, and Coccidiodes {or the treatment of skin and Topical: ine{fective for systemic infections
Neo\len r aqrnal 2{)0 rng. tab. vaginal infections.
Vaginal cream/tahlcl l00-200 mg qhs.
Topical cream: Appl1, tr,r,ice daily continuing 14 days after
lesions have healed.

Econazole nitrate Topical agent effective in the treatnrent of tinea corporis, See under clotrimazole.
Fungidal, Pevaryl cream. cruris, pedis, and cutaneous candidiasis.
I -2 times daily continuing for l4 d.rys after Iesions have
healed.

Fluconazole
Flugal, Flucoder 50 mg,
15t) mg cap. 50 mgi5 ml susp.
Griseofulvin
Crisovin-FP, Fisovin 500 mg tab
lmidazole effective aBarnst cr),ptococci and Candida CI upset, elevated LFTs, skin rash, angioedema,
infections oi tlre orophary'nx, vagina, meningitis. anaphvlaxrs, SJ syndrome.
Neorrate: Thrush 5 nrglkg, lV, PO qd first da,v tlren
I rrg/kg d qrl tur I-1 I I dar'.
Systemic infectior-rs: Poslnatal age <.1,1 days,
6 12 mg/kgld PO, lV c1 72 hr; >14 da;,s, en." 6u,'t.
.1
Children: cryptococcal meningitis, 2 mg/kg/d first clay,
then 6-1 2 mg/kg/d lV, PO q day.
Treatment of tir-rea infections of the hair, nails, and skin Cl upset, rashes, photosensitivity,
due to Microsporum, Epidermophyton, agranulocytosis, leukopenia, lupus
Trichophyton. erythematosus, polyneuropathy. Admin istration
10-1 2 mg/kgid PO divided c112-24 hr. with a fatty meal irrcrease oral absorptiorr.

tt
l.
 1
ESSENCE OF PEDIATRICS

.14
Ketoconazole 3 mg/kg/d PO as a single dose (7 d). Cl upset, urticaria, pruritus, liver damage
Keloral 200 mg lab (if taken >2 wk).
.l
00 mg/5 ml susp.
Miconazole lmidazole active against cryptococci, Candida, See under clotrimazole.
Micoral oral gel, Coccidioides. Use in superficial infections. Topical cream
C-miconazole cream 2"k, apply twice daily, continr-re 10 days after lesions have
Fungidal cream 2"k. healed.

Nystatin Oral candidiasis. Mirrimal side effects with topical application

Nystat, Candex 1 lac units/ml susp,
Canstat 5lakh unit tah.
Ornidazole
Ornid 500 mg tab
Neonate: 100,000 units 4 times per day (topical). nausea, skin irritation.
lnfants: 200,000 units 4 times daily (topical).
Child: 400,000-600,000 units 4 times daily (topical)
continue for 7 days after lesions have healed.
-l
Vaginal trichornonrasis 5 mg/kg sirrgle dose. Dizziness, metallic taste

Acyclovir
Zovirax, Virux 200 mg tab
Virux cream 50 mg/g.
Clovir eye oint 3%
Cyclovex eye drop 3ol'.
Herpes simplex (HSV) encephalitis; mucosal, cutaneous, Cl upset, hepatotoxicity, bone marrow
gental inietlion'; herpes zoster, raritella-zosler, suppression, neurological reactions.
cytomegalovi rus (CMV) prophylaxis.
Neonate: HSV encephalitis: 30-,15 mg/kg/d
lVdividedqBhr.
Children: I s mg,kgd lV divided q B l2 hr.
HSV infection in imnrunoconrpromised host:
15-30 mg/kgld lV divided q B-1 2 hr.
HSV encephalitis/varicetla infection/CMV prophylaxis in
i mmunoconrprom ised host: 30 mg/kg/d

lVdividedqBhr.
Oral dosing ior HSV,zocter inlettions: 100 mg 5 times
daily PO for 10d. (maximum pediatric dose B0 mg/kg/d).
Canciclovir Treatment of CMV infections including retinitis. Cl upset, hepatotoxicity, bone marrow
Cytovene. CMV retinitis: suppression, nephrotoxicrty, neurological
Injection lnduction therapy: 10 mg/kg/d lV (over 2 hr) l reactions.
Capsule: 250 mg. divided q 12 hrfor 14-21 days; maintenancetherapy:
5-6 m{kgld lV once daily.
ldoxuridine (lDU) Topical therapy for herpes simplex keratitis. Apply Local irritation, pruritus, ocular edema.
Eye oint 2.5 mg/g. ointment 5 times daily and ophthalmic solution (1 drop) to
.1
Herpidu eye drop mg/ml. a{fected eye(s) 7 1 0 time s daily and at bedtime.

Ribavirin Aerosol therapy for RSV infections, particularly for patients Rash, irritation, hypotension.
Virazole with BPD and/or congenital heart diseases.
Powder for aerosol- Use SPAC-2 small particle generator at 20 mg/mL
concentration for conti nuous aerosol izatlon
1 2-1 B hr per day.

Didanosine Purine analog - intracellular metabolite inhibits viral RNA Headache (30"/o), diarrhea, pancreatitis,
Videx, ddi, directecl DNA polvnrerase. peripheral neuropathy, optic neuritis, liver
Chewable buffered tab let 25 mg, lniants <90 davs: 100 mg'rr'/d PO divided q 12 hr. dysfunction. Renallv eliminatecl. Food
50 mg, 100 mg, 150 mg. Children: I B0 -100 tngrnr'.'cl PO clivided q 12 hr. decreases bioavailabilitv Llp to 50',;.
Buffered powder packet: i00 mg Administer.Jn .rn emptv stor-nach t hr before or I hr afier
167 mg,250 mg. a nreal to decrease iood eiiect.

Lamivudine Reverse transcriptase inhiiritor used in combination * ith Heaclache. psvchomotor disorders, ieeding
Zeffix 100 mg tab. zidovudine and./or other anti-Hl\1 clrLrg-r. prohlem.. ab,lorrinal p"in parcrealili..
Epivir 150 mg tab. a mg/kg/d divided q 12 hr ineonate, nr.rx 100 mg alone or neutropenia, muscu loskeletal pain.
with interferon alpha in chronic hepatitis B infection.
I
I
I
 DRUG THERAPY IN CHILDREN

Zaicitabine Nucleoside analog reverse transcriptase inhibitor. Cun'ru lative dose-related peripheral neuropathy,
Hivid: ddc. 0.015-0.03 mg/kg/d PO divided q B hr. pancreatitis. Cardiac dysfunction, lactic
Tablet: 0.375 mg, 0.75 mg
acidosis, marrow suppression, hepatitis,
jaundice, rash.
Zidovudine Nucleoside analog reverse transcriptase inhibitor. Seizure, Iactic acidosis, diarrhea, bone marrow
Retrovir: 100 mg cap. Neonates: 8 mg/kg/d PO divided q 6 hr. suppression, cholestatic lrepatitis, rash.
Infanr/child: 27o 540 mg/mr/d pO divided q 6 B hr

Helminth iasis. CI upset, headache, changes in liver enzymes.

Alben, Ben A 200 mg tab >2 yr: 400 mg single dose before meal.
Alben DS, Ben A 400 mg tab. 1-2 yr: 2O0 mg single dose before meal.
Artemether Severe malrria, MDR nraiaria. Neuropsychiatric disorders, slight rise in
Paluther B0 mg/ml/amp. 3.2 mg/kg lM on D1, then 1.6 mg/kg lM for 3 5 days. transaminases.
Benzyl Benzoate Apply to whole body except head & face daily for 2-3 Avoid conlar t wilh eyer & u:e irr pregnan().
Ascabiol, Scablsol emulsion 25,k w/w days, may be repeated. To be used after infancy. Convu lsion.
Cloroquine Phosphate Effective in the suppression and treatment of malaria and Hypotension, psychotic episode, rash,
Jasochlor, Nrvaquine P 250 mg tab. extra-intestinal amebiasis. Dose drug on base equivalent. peripheral neuropatlry, blood dyscrasias,
(150 rng mg base) B0 mg Malaria prophylaxis: 5 mg/kg/wk PO (Max dose retinopathy, tinnitus. Administer with
(50 mg base)/5 ml. 300 mg/dose). meals to decrease Cl upset.
Acute malaria treatment: l0 mg/kg PO initial dose
(max dose 600 mg); 5 mg/kg 6 hr larer rhen 5 mg/kg
PO once daily for 2 days.
Extra-intestinal amebiasis: 10 mg/kg PO daily for 2,3 wk
(max daily dose 300 mg).
Diethylcarbamazepine ln filariasis: Headache, malaise, loint pain, vomiting
Notezine, Benocide 50 mg tab 1 mg/kg/Po a single dose on Di. resulting from filariacidal action, edenra,
Z ng/kgld in three clivided dose on D2. itching, skin rash, hyperpyrexia, lymphadeniris.
3-6 mg/kg/d in three divided dose on D3.
6 mg/kg/d in three divided doses on D4-D14.
Furazolidone Effective in the treatment of protozoal diarrhea, May cause hernolytic anemia in infants <i mcr
Furoxone. enteritis.5 l0 mg/kg/d PO divided q 6 hr oi age; caution in C6PD, hypotension, nausea,
Tablet: 100 mg (max daily dose 400 mg). vomiting, hypoglycernia, hypersensitivity
Suspension: 50 mg/5 mL. reactions, pulmonary infiltration.
Lindane Topical treatnrent for scabies, head lice (pediculus capitis), Dermai ab'orplion may r du\e seizLlreq.
Kwell, Scabene crab lice (Pediculus pubis). clizziness, hepatitis, blood dyscrasias. Do not
Lotion: 1"/" Scabies: Apply thin layer to affected area, remove (shower) apply to denuded/inflamed skin; avoid contact
Shampoo:17o in 6-8 hr irr children and after iB-24 hr in adults. to eye5/rnu( ous rnemhranes.
Pediculosis: Shampoo with adequate amount (15-30 mL)
and lather for 5 min then rinse thoroughly and comb.
Mebendazole Ireatment of ascariasis (ror,rndworm), hookworm, Very well tolerated; dizziness, nausea, rash,
Ermox 100 mg, enterobiasis (pinworm), trichuriasis (whipworm). Ieukopenia, transient elevation in liver function
500 mg tab 100 mg/5 ml syp Children and adults: tests. Enhanced absorption when adrninistered
.l
Meben 00 mg tab, Pinworm: 100 mg PO once; may repeat in 2 wk. with food.
100 mg/5 ml susp. Hookworm/roundworrn/whipworm: l O0 mg pO q 12 hr
for 3 consecutive day5; ln6J course, if needed in 3-:l wk.
Mefloquine Treatnrent & prophr,laris of malaria Cl upset, neuropsvchiatric disorders, tinnitus,
Lariam 250 mg tab. (ch loroqr-r i n e- res sta nt ia ci pa ru nt nra
i I I arja t. vestibular disorders, arrhythnrias, rashes, bone
1 5 mg/kg single dose rr.lar 750 nrgr, marro\ / depression.
follow,ed b1, 10 mgrkg 6 hr later.
Prophvlaxis 5 nrgikg rveeklv once.
Metronidazole Efiective in the treatnrent o1'amebiasis, giardiasis, CI upset, metallic taste, rashes, seizures, ataxia.
Filmet, Flagyl 200 mg, 400 mg rab trichomoniasis, anaerobic bacterial in1'ections.
; 200 mg/5 ml, susp; Amebiasis: 35-50 mg/kg/d PO div q B hr.
\rr0 mg in t00 ml borlle iniusion.
I Other parasitic infections: I5-30 mg/kg/d PO div q B hr.
ESSENCE OF PEDIATRICS

Monosulfiram Flammable iavoid contact with flame)., l

25ok w/w Do not store in cool place.

Tetrasol, Monosol soln J0 ml, o0 ml Rash, idiosyncrasy.
Niclosamide Dizziness, rash, alopecia, abdominal
Yomesan, Nicloside 500 mg tab. pain, nausea. Palients should chew tablets
completely.

Pentamidinelsethionate Hypotension, taehycdrdla, arrhythmia,

Pentam, Nebupent cardiovascul.ar collapse, hypoglycemia, nausea,
lnjection: 300 mg powder/vial marrow suppression, neph rctoxicity, cough,
blood dyscrasias.

Permethrin Pruritus, erythema, stinging, rarely rash.

Lotrix. Scabex cream' 5Y;. Do not apply to denuded/inflamed skin; avoid
contact to eyes/mucous membranes.

Praziquantel Central nervous system depression, dizziness,

Biltricide fever, rash, abdominal pain, eosinophilia.
Tablet: 600 mg.

Primaquine Phosphate Pruritus, abdominal pain, methemoglobinemia,

Jasoprim, Kanaprim 15 mg tab hemolyic anemia in C6PD deficiency.

Py.rantel pamoate Rash, elevation in liver function tests,

Delentin, Melphin. abdominal cramps, dizziness, headache
125 mg tab; 50 mg/ml syp.

Pyrimethamine Seizures, headache, photosensitivi{, rash, fol ic

Malomin 10 mg tab. acid deficiency, mdrrow suppression.
Adminisler folinic acidi5 l0 mgikg J tirrres wkt
lo prevent hematologic toxicity.

Sodium Antimony Cluconate (SAG) Dysrrhythmias (when use lV) myalgia,

lnj Stibatin, Stiboson arthralgia, thrombocytopenia, hepatitis
100 mgiml in a l0 ml bottle. 'i

t
5
 DRUG THERAPY IN CHILDREN

Quinine Used usually in falciparum malaria. Arrhythmias (atrial fibrillation, conduction

lacoquin, Quins
|00 mg lab; .100 mg/5 ml amp
Treatment ol malaria in endemit areas: delects. heart blockr. thrombo( ytopenia,
Uncomplir ated malaria: Chloroquine l0 mgrkg PO on day hypog,lycemia. cinr honism including, tinnilus.
1 then 7.5 mg/kg on day 2,3 + primaquine on day 4. r isuai disturbance5 {tenrpordry blindness),
Treatment failure malaria: Quinine 10 mg/kg on days 1, 2, C6PD hemolisis, rashes.
i + Faniidar on day 3 + Primaquine on da1 4.
Severe malaria: Qtrinine 20 mg//kg in 5o,o DNS in 4 hour
lV stat (or 20 mg/kg in 2 divided doses lM in two thighs
clat'. lhen 10 mg/kg lV in dertrose saline B hourly over
3-4 hr until child can take drug orally, then quinine
should be taken PO for 3 days more + Fansidar on 3rd day
- Primaquine on 4th crlay, orQuinine lO mg/kg/dose lV
B hrly in 5'% dextrose saline over 3-4 hr; then 10 mg/kg/
dose B hrly PO (when can take orally) for a total of 7 days
(lV + oral). Primaquine on 8th day.

1. Behrman RE, Kiiegman R.NI, Jehson BH. Nelson 'fextbook of British National Formulary. British Medical Association: 43, March
Pediatics 16'h ed. Philadelphia:VB Saunders Co., 2000. 2002.
2. Parthasarathy A led). IAP Tixtbooh of Pediatrics 4'h ed. New Delhi, 5. Laurence DR, Ber-rnett PN, Brown MJ. C/inical Pharmacologt 8't'
Jaypee Brothers, 2009. ed. Edinburgh: Churchill Livingstone, 1997.
3. MIMS Asia, 135 Cecil Street 13-00, LKV Building. Singapore, 6. Goodman & Gilmans The Pharmacological Basis of Therapeutics.
069 536. 10'h ed. New York: McGraw-Hill, 2001.
 I

CHAPTER 28
Growth Charts

Chopler Conlenls
Weightf or-age percentiles: Boys,
binh to 36 m0nths.................,..........................................508
Weighlfor-age percentiles: Cirls,
birth to 36 m0nths......,..,.,...,.......................................... .509
Weight-for'age percentiles: Boys, 2 to 20 years.............510
Weight-for'age percentiles: Cirls, 2 to 20 years.............511
Weight'for-length percentiles: Boys,
birth to 36 m0nths.......,...,...,.............................................512
Weight{or-length percentiles: Cirls,
Weight-for-stature percentiles: Boys...,......,,...,,.,,,.............514
Weight-tor-stature percentiles; Cir1s............,..,...,,..,,..........515
LengthJor-age percentiles: Boys, birth t0 36 months..516
Length{or-age percentiles: Cirls, birth to 36 months..517
Staturejorage percentiles. Boys, 2 to 20 year5.............518
5ralure f0r-age percenriles: C'rls, 2 lo 20 yeari...... .:19
Head circumference{or age percentiles:
Boys, birth to 36 m0nths................................................520
Head circumference{or-age percentiles:

birth t0 36 m0nlhs.........,...................................................513 Cirls, birrh to 36 m0nths..................................................521

Weight-for-Age Percentiles: Boys, Birth to 36 Months

tt-
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Birth 3 6 I 12 15 18 21 24 27 30 33 36
I
Age (months) I
a
I
 GROWTH CHARTS

Weight-for-Age Perceniiles: Girls, Birth lo 36 Monlhs

15 18 21
Age (months)

'

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I
ESSENCE OF PEDIATRICS

Weight-for-Age Percentiles: Boys, 2 lo 20 Yeors

10 11 12 13
Age (years)

bl
t
 GROWTH CHARTS

Weight-for-Age Percentiles: Girls, 2la 20 Yeqrs

ESSENCE OF PEDIATRICS

Weight-for-lenglh Percenliles: Boys, Birth lo 36 Months

Kg- tb
tb

23-
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22- A"R.
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AA 60 65 70 75 B0 85 90 95
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 !

GROWTH CHARTS

Weight-for-Length Percentiles: Girls, Birth lo 36 Months

lh) tb

6rl

48

AA

'o 5ft
_l /\ 42

. 90th 40

aa- 5th 38-

?6
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5th ?4

t Ottr l-- 32-

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in le ts l0
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cm is 50 55 60 ob 70 75 Bo 85 90 95 1oo
Length

t,
I
 I
ESSENCE OF PEDIATRICS

Weight-for-Slolure Percenliles: Boys

kg

34

JJ

32

31

30

29

28

27

26

25

24

23

22

21

20

19

1B

17

16

15

14

13

12

11

10

in31 32 33 34 35 36 37 38 39 40 41 42 43 44 4s 46 47
cm B0 85 90 95 100 105 110 115 120

Stature

il
 GROWTH CHARTS

Weight-for-Slqlure Percenliles: Girls

kg tb tb
76
34

33
72
32

31 -68
30

64 fh
29

28
60
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23 T
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in 31 32 33 34 35 36 37 38 39 4 3 4 4t "7
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cm B0 85 90 95 100 105 110 15 20

Stature
 I
ESSENCE OF PEDIATRICS

Length-for-Age Percenliles: Boys, Birlh lo 36 Monlhs

15 18 21
Age (months)
 GROWTH CHARTS

Lenglh-for-Age Percentiles: Girls, Birth to 36 Monlhs

15 18 21
Age (months)
 .I

ESSENCE OF PEDIATRICS

Stoture-for-Age Percenliles: Boys, 2 lo 20 Yeors

I
i
 t
GROWTH CHARTS

Stoture-for-Age Perceniiles: Girls, 2 lo 20 Yeors

9 10 1112
Age (years)
 t

ESSENCE OF PEDIATRICS

Heod Circumference-for-Age percenliles: Boys, Birth to 36 Months

cm- tn
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 GROWTH CHARTS

Heod Circumference-for-Age Percentiles: Girls, Birth to 36 Monihs

cm tn
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 l
ESSENCE OF PEDIATRICS

Body Moss Index-for-Age Percentiles: Boys, 2lo 20 yeqrs

23456789 13 14 15 16 17 18 19 20
Age (years)
 GROWTH CHARTS

Body Moss lndex-for-Age Percenliles: Girls, 2lo 2O Yeors

5 6 7 I 9 10 11 12 13 14 15 '16 17 18 1

Age (Years)
 t

ESSENCE OF PEDIATRICS

WEIGHT FOR AGE

1.63 2.04 2.45 2.86 1.1 / 0 2.74 2.24 1.75 1.26

1.55 2.24 2.92 3.61 4.29 1 3.98 3.39 2.79 2.19 1.59
1.76 2.62 .).+ / 5.1 9 2 4.71 4.43 .). )) 2.67 1.99
2.18 3.13 4.08 5.03 5.98 J s.40 4.65 3.91 3.16 a /1

1,/-) 4.70 5.69 6.68 4 6.05 5.25 4.46 3.66 z.o /

7.30

u
a.)f ).)1 6.31 5 6.65 5.82 4.98 4.15 ). ) I

3.94 4.92 5.89 6.87 / -o) 6 7.21 6.34 5.47 4.60 -)./ 1

4.47 5.44 6.41 o- 1+ 7.71 6.80 5.90 5.00 4.OC)

4.g2 5.89 o. o) 7.82 o./o o 8..1 6 7.22 6.29 5.35 4.42

5.30 6.27 8.21 9. 1B 9 8.56 7.59 6.63 5.66 4.70
5.62 6.60 7.58 8.56 9.54 10 8.92 7.92 6.93 5.93 4.94

I
5.88 6.BB o.6/ 9.86 "t1 o )/ B).2 7.20 6.17 5.1 5
6.09 7.11 8.12 9.14 10.15 12 9.53 o. +o 7.43 5.39 5.34
6.26 7.30 8.34 9.38 10.41 13 9.79 8.72 7.65 6.57 5.50
6.40 7.46 8.53 9.59 10.65 14 10.03 8.93 7.84 6.74 5.64
6.51 7.60 8.69 9.78 10.87 15 10.25 9.1 3 8.01 6.89
6.60 B.84 9.96 1 1.08 16 10.45 9.31 8.17 7.04 5.90
6.68 8.98 10.13 11 .28 "17 10.64 9.49 6.O2
6.76 7.93 9.1 1 10.29 11 .47 18 10.83 9.65 o.+o / 1l 6.1 4
6.83 8.04 9.25 10.45 1 1.66 19 1 1.01 9.82 8.64 7.46 6.27
.10.6.1
6.91 o. l) 9.38 1 1.85 20 11.19 9.99 8.80 7.50 6.11
7.00 8.26 9.52 10.78 12.O4 21 11.37 10.16 8.96 7.7\ h54
7.OB 9.6.5 10.94 12.22 ia I 1.55 10.33 9.12 7,90 6.68
.1
7.17 9.79 1.1 0 12.41 23 11.73
.1
0.50 9.28 o.trJ 6.82
7.84 8.97 I0.09 11.22 12.34 24 i 1.80 10-62 9.45 8.28 7.10
/ -o) 9.03 10.20 11 .37 12.54 25 12.01 10.81 9.61 8.40 7.20
7.87 9.09 10.30 11.52 12.74 26 12.23 10.99 9.76 8.53 7.29
7.89 9.15 I0.41 1.1 .68 12.94 27 12.43 11.17 9.91 o.o) 7.39
7.91 9.22 10.52 1 1.83 1 i.1i 28 12.53 1 1.35 10.06 8.77
7.94 9.28 10.63 1 I.98 I )- I l 29 11.52 10.21 B.89 7.58
7.97 9.3 6 10.7 4 12.13 13.52 30 13.03
.1
1.69 10.35 9.01 7.67
8.00 9.43 10.85 12.28 13 .71 31 13.22 1 1.85 10.49 9.1 3 7.76
8.04 9.51 10.97 13.89 32 13.40 12.01 10.63 o )/ 7.85
8.09 9.58 11.08 12.58 14.08 JJ 13.58 12.17 10.76 9.3 5 7.94
8.1 3 9.66 11 .20 12.73 14.26 34 13.76 10.90 9.16 8.03
B.'1 B 9.75 11.31 12.88 14.44 35 r 3.93 12.48 I 1.03 9.57 8.r 2
9.83 11.43 I3.03 14.62 36 14.10 12.63 11.1-5 q.68 8.21
8.29 9.92 11.55 14.80 37 I )1 12.7I 11.28 9.;9 8.29
^
o.Jl r 0.01 11.67 14.98 1 4.41 1).92 1t.+t 9.89 o-to
8.12 10.10 11.79 13.47 15.16 39 r-1.6t) I l.Otr 11.;l 9.99 o.+o
8.48 r0.19 11.91 13.62 15.33 40 11.76 r 3.20 1r.61 10.10 8.54
8.55 10.29 12 Ai 13.77 15.51 41 14.91 I )_ )+ 11 ,77 r0.20 8.62
 GROWTH CHARTS

8.62 10:39 12.1 5 13:91 15.68 42 15.07 13.48 11.89 10.29., '..:B:70,
8.70 10.48 12.27 14.06 15.85 43 15.22 13.61 12.04 10.39 ':8.7B l

10.58 12.40 14.21 16-O2 44 15.37 13.74 12.12 10,49 ''8:86

8.85
.10.68
12.52 14;3:5 16.19 45 15.52 13-88 12.23 10.,58 8.9.4
8.93 10.79 12.64 14.50 16.36 46 15.67 14.00 12.34 10.68 '9.01r'
9.01 10.89 12.77 14.65 16.53 4:7 15.81 14.13 12.45 1O,77 , 9.09
9.10 11.00 12,90 14.79 16,69 4S 15.96 14;26 12:56 1 0_86 '.9.1 6
9.18 1.1 .10 13.02 14.94 16.S6 49 r6.10 '1.4.39 1,2.6.7 1O.95 :'9.23
o'r7 11-21 13.1 5 15.O9 17.03 50 16,25 14.51 12.77 11:04 ' S.30 '

15.23 .19 5I 16 39 14.53 ,12.88 1 .13 ':.9.37

9.36 11,32 13.28 17 '1

9.45 11.43 13,40 15.38 17.36 52 1 6.53 14.76 12.98 11,21 .9,44
13.53 15.53 17.52 53 16.67 i 4-BB 13.O9 1'.l -30 r 9.51
9.54 11.34
9.64 't 'l .65 13.66 15.67 17,69 54 16.81 15;00 13.19 11.:38 '9.57 r'
9.73 11 .76 13.79 15.82 17.85 55 16.95 15.i2 13.2g 11.46 ':.:9:64 '

9.82 11.87 13.92 15.97 18.02 56 17.O9 15.2 5 13.40 11.55 9.74

9.92 11.99 14.05 't6.12 1B.1 B 5l 17.24 15.37 13.50 11.63 9.76

10.02 12^10 14.18 16:26 18.34 5B 17.38 15.49 13.60 11 .71 9.82

10.1 1 12.21 14.31 16,41 18.51 59 17.52 15.61 13.70 ,11.79 '9.88
10.21 1 2.33 1,4.44 16.56 18.67 60 17.66 'l 5.73 13.80 11.87 ,.9;94
10.31 12.44 14.57 16.71 1 8.84 61 17.81 15.85 13.90 11.95 9:99 :

'l 0.41 12;56 14.70 ,1

6,85 19.00 62 17.96 15.98 14.aO 12.0:2 1'0.04
10.50 12.67 14.84 17.00 19.17 63 1,8.10 16.10 14;10 12,10 10:1O
10.60 12.78 14.97 17.15 19^33 64 18.25 16.23 14:20 12.17 ,:1
0:1 5

10.70 12.90 15:10 17.30 19.50 b5 18.40 16.35 14.30 12.25 '10.2D
10.79 13.01 15.23 17,45 15.67 66 18.56 16.4& 14.40 12,32 :t 0.25
10,89 13.13 15.36 ,17.60 19.84 67 18.71 16.61 14.50 12,40 1,D.29
10.99 13.24 15,49 17.75 20,00 68 18.87 16.74 14.64 12.47 i '1'034
11.08
.13;35
J s.63 17.59 20.17 69 19.03 16.87 14.70 12:54 r 0.38
':1

1 1..1 8 13.47 15.76 18.0s 20.34 78 19"19 17.OO 14.81 12.62 .l1O:42
1-l.27 13,58 15.89 18.20 20.51 7l 19.36 17.13 14.91 12.69 10.46'
11.36 13.69 16.02 I8"35 20.59 72 19.52 17.27 :l 5.01 12.76 :1O.50
11.45 13.80 16.1 5 l B.sl 20.86 73 19.78 17.41 15.12 12.83 1:A.54
11 .54 13.91 16.29 18.66 21 .O3 74 19.87 17.55 15.22 12.90 10.57
11.63 14,A2 16.42 18,81 21 .21 75 20.05 17.69 15.33 12.97 10.61
't1.71 14.13 16.55 18.97 21 "38 76 12 a2 | / -o.a 15.43 13.04 10.64
11.80 14,24 16.68 19.12 21 .56 20.42 17.98 15.54 13.11 10.67
1',I .86 14.35 16.81 19.28 21 .74 78 20.61 to. lJ 15.65 'I
3.1B 10,70

11 .96 14.45 16.94 19-43 21 .92 79 20.80 18.28 15.76 13.24 10.72
12.O4 14.56 17.D7 19.59 22.10 80 21.00 18.44 15.87 13.31 10.75
12.12 14.66 17.20 19.7s 22.29 81 21 .20 18.59 15.99 13.38 1A.77
12-19 14.76 17.33 19.90 22.47 82 21 .41 18.76 16.10 13.45 10.79
12.26 14.86 17.46 20.46 22.66 B3 21 .62 18.92 16.22 13.52 10,81
12.33 14.96 17.59 20,22 22.85 84 21 .84 19.09 16.34 13.58 10.83
12-39 15.06 17.72 20.38 23.O4 B5 22"06 19.26 16.46 13.65 10,85
12.46 15.15 17.85 2A.54 23.24 86 22.29 19.43 16.58 13.72 10.86
12.52 15_25 17.97 20.70 23.43 87 22.53 19.61 16.70 17.79 14.87
 I

ESSENCE OF PEDIATRICS

:l'2157. '1 ,24.87 '.22:76

5.34 1r8.10 23.63 8B 19-79 16,82 1 3:85 10.88
1,2,:,63
15.43 18.23 21.03 23.83 .89 23.O1 19.98 16:95 13.92 10.89
,12,,68 ,l'5.52 18,35 21:19 24.O3 90 23-26 '2:fr",17 17 SE 13:99 I0.90
:12:72 ,1,5.60
1B.4B 2-t.36 24.24 '9I 23.51 20.36 17:21 14.06 10.91
1,2:7,.7, 1,5169 1'8.61 21_52 24.44 92 23:77 20,55 1,7,34 '14:13 10.9?
1?;8'1, ,15.77 1B:73 21.69 24.65 93 24.A3 20;75 17 '48 14.20 10.92
L2':M, ,15:85 1.8;85 21.86 24.86 94 24.30 20,95 17,61 14.27 1 0,93
1,2,.87 15,92 18.98 22,A3 25.08 ,95 74,57 21,16 1.7,75 14,34 10.93
1:2.9fi;. 16.00 :19J 0 22:28 2s.30 .96 24::84 21,:37 17:a9 '14-41
10.94
1:2tlg2, 1'6;07 19:22 22:37 25.52 97 25:12 2,1 .58 18,03 14.49 10.94
J'2:94 1,6.14 I9'34 22.54 25.74 9B 25.41 21 :79 1 8.i18 14:56 10.94
''12,96 1'6t1 1g.46 22,71 25.97 99 2570 22;01 18.32 14:63 10:95
12,: 7 16.28 19.s8 22.89 26.19 r00 25:99 22.23 1B-47 14.7'1 10-95
T7,.q8 1.6.34 1'9.70 23.06 26.43 10-l 26.29 22.:45 18.62 14.79
-.t' 10.96
1?.e8i J6-44 19.A2 23.24 26.66 102 26,59 22,68 14.77 1,4.87 1O:96
l:l:99 16;46 1,,9:94 23:42 26.90 1q3 26.89 22.91 18.93 14.95 10.97
1:2,.99 16.52 20.06 23.60 27.14 104 27.24 23,14 19.08 15.03 10.97
1,2:98 i 6.ts 20.1 B 23.78 27.38 105 27.51 2r3.38 :19.24 15.11 10.98
12:98 16.64 20.30 23.97 27.63 106 27.82 23.61 19,44 15.20 10.99
T2]:::9:7: 16.7A 2A.43 2415 1/.OO 107 28.14 23,85 19.57 15:25 1 l:00
:1:2'i197 16:76 20.55 24.34 28.13 108 26:.46 24.10 19,.73 15.:37 1 l.o:l
12,96 16.8-2 2rJ.67 24.53 28.39 :109 28.79 24.34 19-9O 15.46 11.O2
:l?'95 I 6-87 20.80 24.72 2,8.65 lto 29;11 24.59 20.97 15.5.5 11,03
12:.94,,; 16.93 20.93 24.92 28.91 111 29:44 24.84 20-24 15.65 11.05
1,2,.::93;.'. 1 6:99 21 .Q5 25.12 29.18 l12 29.78 25.:10 2O:42 1.5,74 11.05
:ltii9tI 17;05 21 .18 25.32 29.45 113 30,1 2 25"36 20"60 15.84 11;OB
.li?:90 17,11 21 .31 25.52 29.72 114 30;45 25:62 20;78 1,5.94 i1::iO
1,7.1,7 21 .45 25.72 30.oo 115 30.80 25.8S 20.96 16,O4 11 .12
12'.eei
1,2:'.,88' 17.23 21 .58 25.93 30.28 116 3,1.14 26,14 21.15 'l
6:1 5 1 1.15
t:I:8;r 17;3'0. 21.72 26.,14 30.57 117 31,49 26.41 Lt-.)). 1r6.'25 t 1.18
12.&6 17,36 21 ,86 26:36 30.86 118 31.84 26.68 21 ,52 16.36 11.21
12.46 17..43 22.OO 26,57 31.',t 5 119 32.19 25.95 21.72 16.48 11 .24

)
ll
I
 GROWTH CHARTS

CATCUI.ATING CHILD'S WEIGHT FOR AGE

c)
o)
(E
c
.g')
Qo
50 =€
OP
>o
=(E sg

Nc)o.)@1.-@rorfcDNO<'r@
NNN

I(6
an 0)
=>
tll +
)s

cDNoo)coN(oro<-ca

c
'q)
o
c =
o) o
'c) =
3
o
j
o
=
 q

ESSENCE OF PEDIATRICS

WEIGHT FOR TENGHT

\flHO/NCHS Normalized Reference'Weight-for-Length (49-84 cm) and \Teight-for-Height (85-110 cm), by Sex

1.8 2.1 2.5 2.8 3.1 49 2"9 2.6 2.2 '1.8

.1.8
2.2 2.5 2.9 J.J 50 3.4 .).. 2.3 1.9
,2.6
.,1:a
2.2 2.6 3.1 3.5 5t 3.5 3.1 2'.7 z.a 1.9
1.9 2.3 2.8 3.2 '3.7 52 3,7 J,J 2,8 2.4 2

1,9 2.4 2.9 3.4 3.9 53 .3;9

'3'.4 2.5 2.1
2 2.6 3.1 3.6 4.1 :54 4.1 ,u ,3'1
1a 2.2
2.7 1- t 3.8 4.3 JJ 4.3 J.O. l --) 2.8 2.3
2:3 2.9 11 4 4.6 '5,6 4.5 4 3.5 J 2.4
2.5 3.'t i4.8 J1 4:8 .:4'2 3.1 2.6
4-/ J.J 3.9 4.5 5.1 5B 5 4.4 3.9' 3.3 aa
2.9 3.5 4.1 4.8 5.4 59 5.3 4.7 4.1 3.5 2.9
3.1 3,7 4.4 5 5.7 60 5.5 4.9 4.3 J. t

-). ) 4 4.6 5:3 5.9 61 5.8 5.2 4.6 3.9 3.3

3.5 4.2 4.9 5.6 6.2 62 6.1 5.4 4.8 4.1 J.f
I

3.8 4.5 5.2 )-6 6.5 .53 6.4 5.7 5 4.4 .J./

4 4.7 5.4 6.1 6.8 64 6,7 .6 5.3 4.6 3-9

4.3 5 5.7 6.4 7.1 65 7 6.3 5.5 4.8 4,1
+.J 5.3 6 6:7 7.4 7.3 6.5 f.o 5..1
96
4.8 5.5 6.2 7 7.7 67- n_o 6 5.3 +. -)

5.1 5.8 6.5 o 6B 7.8 7.1 o_ 1 5.5 4.8

6 6.8 /.3 o.J 69 8.1 / .a 6.5 f-o 5
5.5 6.3 7 7'B 8.5 70 8.4 7.6 6.8 6 5.2
J.O 6.5 / .J B..t o.o 7l 8.6 7.8 7 6.2 5.4
n 6.8 7"5 8.3 9.1 72 8.9 8..1 7.2 6.4 5.6
6.2 /.o 8,6 9.3 73 9.1 8.3 7.5 6,6 5-S
6.4 7.2 B o.o 9.6 74 9.4 8.5 7.7 6.8 h

6.6 7.4 B^2 I LB /J 9.6 o./ 7.9 7 6,2

6,8 7.6 8"4 9.2 10 76 9;B 8.9 8.1 7.2 6.4
7 7.8: 8.6 9,4 10.3 77 10 9.1 o.J 7.4 6.6
7.1 8 8.8 9.7 10.5 7B 10.2 9.3 8.5 7.6 6.7
7.3 8.2 o 9.9 10.7 79 10.4 9.5 8,7 /,o 6.9
7,5 9.2 10.1 10.9 80 10.6 9.7 B.B 8 7.1
7.6 8.5 9.4 14.2 11.1 81 10.8 9.9 o 8.1 7.2
7.8 8.7 9.6 10.4 11.3 82 11 10.1 9.2 d. t
7.9 8.8 9,7 10.6 1 1.5 B3 1"t.2 10.3 9.4 8.5 7.6
8.1 I s.9 10.8 11.7 84 11 .4 10.5 9.6 o-/ 7,7
7.8 8.9 9.9 11 12.1 8s 1 1.8 10.8 9.7 8.6 7.6
7.9 9 10.1 11.2 12.3 B6 12 11 9.9 7.7
8.1 9.2 i 0.3 1.5 12.6 87 12.3 't1.2 10.1 9
\
1
1 7.9

I
8.3 9.4 10.5 11 .7 12.8 B8 12.5 11.4 10.3 9.2 8-1

8.4 9.6 to.7 1 1.9 13 89 1 2.7 11.6 10.5 9.3

 GROWTH CHARTS

o.o 9.8 r 0.9 12-1 13.3 90 12.9 1 1.8 10.7 9.5 8.4

o.o 9.9 1 1.1 12.3 13.5 91 13.2 12 10.8 9.7 8.5

13.7 92 13.4 12.2 11 g.g 8.7

8.9 10.1 1 1.3 12.5
.1.5 11.2 10 8.8
9.1 10.3 1 12.8 14 93 13.6 12.4

9.2 10.5 11 .7 14.2 94 13.9 12.6 11 .4 10.2 I

9.4 10.7 1 1.9 13.2 14.5 95 14.1 12.9 11 .6 10.4 9.1

9.6 10.9 12.1 13.4 1 4.7 96 1 4.3 13.1 11.8 10.6 9.3

9.7 11 12.4 t)./ 15 97 14.6 12 10.7 9.5

'l 0.9 9.6
9.9 11.2 12.6 13.9 15.2 98 14.9 1 3.5 12.2

r 0.1 11 .4 12.8 14.1 r 5.5 99 15.1 tl.o 12.4 1i.1 9.8

10.3 11.6 13 14.4 15.7 100 1 5.4 14 12.7 1 1.3 9.9

10.4 11.8 13.2 14.6 16 101 15.6 14.3 12.9 11.5 1 0.1

10.6 12 13 .4 14.9 16.3 102 15.9 1 4.5 13.1 11.7 10.3

10.8 12.2 13 .7 15.1 16.6 103 16.2 14.7 13.3 I 1.9 10.5

11 12.4 13.9 1 5.4 16.9 104 16.5 15 13.5 12.1 10.6

.t .1

11.2 12.7 14.2 15.6 17 .1 105 16.7 5.3 3.8 12.3 10.8

11.4 12.9 1 4.4 15.9 17 .4 106 17 15.5 14 12.5 11

10.5 12 13.4 14.9 102 15.9 14,5 13.1 11 .7 10.3

10.8 12.2 13.7 15.1 16.6 103 16.2 14.7 1 3.3 11.9 10.5

16.9 104
.1
6.5 15 13.5 1) t
10.6
11 12.4 13.9 15.4
11.2 12.7 14.2 15.6 17 .1 105 16.7 15.3 lJ.o 12.3 10.8

11.4 1)q 14.4 15.9 17 .4 106 17 15.5 14 12.5 11

1 1.6 13.1 1 4.7 16.2 17 .7 107 17.3 t)-o I +_ ) 12.7 11.2

I l.o 13.4 14.9 16.5 lo 108 17.6 16.1 14,5 13 11 .4

12 1 3.6 15.2 16.8 18.3 109 17.9 16.4 14.8 13.2 .1 1.6

12.2 13.8 1 5.4 17 .1 18.7 110 r 8.2 r6.6 15 13 .4 11.9

72:27i B.l\.
Length is measured below g5 cm; height is measured 85 cm and .rbove. Recumbent lerrgth is on aver.rge 0.5 cm greater than standing height, although the difference is of no
imp"ortance to the individual child. A ionection rray be made b,v deducting 0.5 cm fronr all lengths above 84.9 cm ii slanding height cannot be measured

I
l'
h
 lndex

Amphotericin B, 503 Atrial septal defect, 153

A Atrioventricular biock, 1 B9
Ampicillin, 497
Abdominal pain, 130 Anaphylactic shock, 349 Atropine sulfate,474
Abdominal paracentesis, 45 9 Ancylostomiasis, 415 Attention deficit hyperactiviry disorder, 340
Anemia, 291 Atypical lebrile convulsions, 261
ABO hemolytic disease of the newborn, 40
Anencephalus, 11 Aura,254
Absence seizures, 254
Aneupliod, 424 Auscultatory areas, 3
ACE inhibitors, 178
Anion gap, 218 Autistic disorder, 341
Acetaminophen, 473
Acetazolamtde. 473 Anorectal anomalies, 449 Automatism, 254
Anthrax, 419 Autosomal dominant inheritance, 424
Achondroplasia, 439
Acid-base regulation, 213 Antihemophili c factor, 47 4 Autosomal recessive inheritance, 425
Antihypertensive drugs, 1 74 Autosomes, 423
Acne,354
Antimicrobial therapy, 29 Azathioprine, 47 4
Acquired immunodeficiency syndrome, 383
Anti-TB drugs,404 Azithromycin, 498
Acrodermatitis enteropathica, 82, 465
Actinomycin D,479 Antitoxin serum, 33
Acute bilirubin encephalopathy, 40 Antiven in p olyvalent, 47 4
Aortic regurgitation, 166 B
Acute cardiac tamponade, 169
Acute disseminated encephalomyelitis, 271 Aortic stenosis, 157
Baby friendly hospital initiative, 54
Acute lymphoblastic leukemia, 303 Apert syndrome, 465
Apgar scoring system, 14 Baclofen,474
Acute myeloid leukemia, 304
Aphthous stomatitis, 390 Bacterial meningitis, 264
Acute pericarditis and pericardial effusion, 168
Apnea,22 Banti syndrome, 465
Acute pharyngitis, 109
Approach to failure to thrive based on age, 70 Barbiturate poisoning, 362
Acute poststreptococcal
Ara-C,479 Barlow test, 12
glomerulonephritis' I 95
Areas of impairment, 68 Barth syndrome, 427
Acute renal failure, 206
Arginine vasopressin, 219 Basic genetics, 423
Acute srroke syndrome, 2-5
Arrhythmias, 186 Bayleyt scale of infant development, 68
Acyclovir, 504
Arsenic poisoning, 365 BCG Test, 402
Adoption and care oforphans, 86
Artemether, 505 Becker muscular dystrophy, 287
Adrenal crisis,242
Artificial feeding, 54 Beckwith syndrome,465
Adrenal gland,240
Ascariasis,4l5 Beclomethasone, 474
Adrenocortical insuffi ciency, 242
Ascites, 149 Benzathine,50l
Advantages of breast-leeding, 49
Ascitic fluid characteristics, 150 Benzoyl peroxide,475
AIDS,383
Ascorbic a'cid,474 Benztropine mesylate, 475
Albendazole, 505
Asparaginase, 474
Benzyl benzoate, 505
Albright syndrome, 465
Asperger syndrome, 342 Benzyl penicillin, 501
Albumin hwan,473 475
Aspirin,474 Benzylpenicilloyl-polylysine,
Aigorithm for treating apnea,23
Aspirin poisoning, 358 Betactam,475
Alleles. 423
Assessing nutritional status (SD), 524 Berger disease, 465
Allergic rhinitis, 106
Assessment and classification of the sick child Betamethasone, 475
Allopurinol, 473
aged 2 mo to 5 yr, 88 Bethanechol, 475
Alphal -antitrypsin defi ciency, 465
Assessment, classification, and treatment of Biliary atresia, 447
Alport syndrome, 465
sick young infant aged 1 d to 2 mo, 93 Blackfan-Diamond anemia, 465
Ambiguous geni:alia, 246
Assessment of development, 64 Blanryre coma scale, 409
Amebiasis,415
Assessment of different developmental
Bleomycin, 475
Amikacin sulfate,497
aspects, 61 Blood biochemistry, 469
Aminocaproic acid,473
Asthma, 114 Blood culture, 455
Aminophylline, 473
Ata-ria telangie 455 Bloom syndrome, 461
Aminophylline IV 22 ctasia-,
Atenolol,474 Blue diaper syndrome, 465
.\mitrip wl ine hy dr ochlo ride, 47 4
cr-Thalassemia, 295
BMI,82
,{mniocenresis, 429
Atopic dermatitis, 350 Body mass index-lor-age percentiles :
-r.noricillin. .197 r

Atorvasratin. 47 4 boys, 2 to 20 yearc,122

F. ---::-. :r.i;illin-clavulanate, 497
f'
t
F
 INDEX

Body mass index-for-age percenriles: Childhood leukemia, 303 Congenital infections, 26

girls, 2 to 20 years,523 Chloral hydrate,475 Congenital Rubella syndrome, 31
Body water, 212 Chlorambucil 477 Congenital syphilis, 29, 398
Bone marrow aspiration, 457 Chloramphenicol, 499 Congenital reeth, 11
Bone marrow transplantation, 296 Chlorpheniramine maleare, 47 7 Congestive cardiomyoparhy, 1 70
Brain growth and interaction, 58 Lhlorpromazlne, 4 /,/ Conjunctivitis, 26
Brain tumors,3l0 Choanal atresia, 12, 109 Conn syndrome (hyperaldosteronism), 465
Breast-feeding, 49 Choice of blood group in exchange Contact dermatitis, 35 1
Breast-feeding and HIV/AIDS, 52 transfusion, 3B Continuous positive airway pressure, 46
Breast milk jaundice, 40 Choledochal cyst,44B Control of tetanic spasm, 33
Breath holding attacks, 261 Chorionic gonadotropin, 47 7 Cordocentesis, 429
Bronchiectasis, 124 Chorionic villus sampling, 429 Coring system to evaluate severity of
Bronchiolitis, 1 I 2 Chronic bilirubin encephalopathy, 40 respiratory distress, 24
Bronchiolitis obliterans, 1 13 Chronic constrictive pericarditis, 169 Corticotropin, 478
Brucellosis, 400 Chronic diarrhea and malabsorption Cortisone, 478
Bruxism, 344 syndromes, 135 Co-trimoxazole, 500
B-thalassemia, 295 Chronic hepatitis, 143 Counseling, 222
Budesonide, 475 Chronic hypertension in children, 172 Cranial nerves, 5
Burns, 366 Chronic kidney disease, 207 Creatine phosphokinase, 286
Busulfan, 475 Chronic liver disease, 146 Cri-du-chat syndrome, 433, 465
Chronic lymphocJ'tic thyroiditis, 231 Crigler-Najjar syndrome, 465
Chronic myeloid leukemia, 306 Crohn disease, 139
c Chronological order of appearance of Cromolyn sodium, 478
osseous centers, 61 Crotamiton, 478
Caffeine, 22,475 Ciprofoxacin HCl,499 Croup, 110
Caffey disease (infantile cortical Cisplatin,477 Crouzon syndrome, 465
hyperostosis), 465 Clarithromycin, 500 CSF values, 471
Calciferol, vitDr,483 Classification of PEM based on BMl,73 CSOM, 1O'
Calcitriol,4T6 Classification of PEM based on Z-score, 72 Cushing disease,224
Calcium saks,476 Clean delivery and neonatal kit, B Cushing syndrome, 243
Calculating childt weighr for age,527 Clefrlip,444 Cyanocobalamin, 478
Candida infection,2T CIeft palate, 444 Cyanocobalam in (.B 7 9
rr),
Candidiasis, 352 Cleidocranial dysostosis, 465 Cyanotic congenital heart diseases, 164
Captopril,476 Clindamycin,500 Cyclophosphamide, 478
Carbamate poisoning, 363 Clinical presentations of SEH-IVH, 21 Cyclosporine, 478
Carbamazepine, 476 Clonazepam,4TT Cystic fibrosis, ) 38
Carbenicillin, 498 Clonic seizures, 34 Cystinosis, 466
Cardiomyopathy, 170 Llontdtne. 4 / / Cystitis, 202
Cardiovascular system, 2 Cloroquine pho'phare, 505 Cytarabine HCl,479
Care at birth, 8 Clotrimazole, 503 Cytomegalic inclusion disease, 31
Carrier detection, 428 Cloxacillin sodium, 500
Case record of a newborn infant, B Coarctation olthe aorta, 157
Causes ofintrauterine growth retardation, 17 Codeine,477
D
Causes ofprererm birth, 17 Colfosceril pelmitate, 47 B
Caustic injuries, 362 Colloid goirer,227 Dacarbazine,4T9
Cefaclor, 498 Coma, 278 Dactinomycin, 479
Cefadroxil, 498 Common congenital malformations, 11 Daily fluid requirement, 10
Cefepime, 498 Commonly used developmental Daily fluid requirement during first
Cefixime, 498 screening test, 6B week of life, 46
Celota-rime sodium, 498 Commonly used developmental screening Dandy-\Walker syndrome, 466
Cefpodoxime proxeril, 499 test and areas of impairment, 68 Dantrolene, 479
Ceftazidime, 499 Complementary feeding, 53 Dapsone, 500
Ceftriaxone sodium, 499 Complete blood count, 469 Daunorubicin hydrochloride, 429
Cefuroxime, 499 Composition of human milk, 49 DBD.282
Celiac disease, 137 Conduct disorder, 334 Deferriprone, 479
Cephalexin, 499 Congenital adrenal hyperplasia, 240 Uela)'ed puberr)', l4 '
Cephradine, 499 Conger-rital and perinatal infections, 29 Deliven- room management, 16
Cerebral palsy, 338 Congenital anemia of the nervborn. 37 D.liren room rrearmcnr. l5
Cerebrospinal fuid examination in Congenital chloridorrhea, 46i Dengue fever, 313
neonates, 28 Congenital diaphragmaric hernia, 444 Dengue hemorrhagic fever, 374
Cervical adenitis, 391 Congenital dislocarion of Dengue shock svndrome, 374
Cetiizine,476 the hips, 12 Dermatitis herpeti[ormis, 466 n
Chediak-Higashi syndrome, 465 Congenital goiter,227 Dermatology,34T
1
t
-d
 INDEX

Dermatophytoses, 352 Ductus-dependent systemic fow lesions, 156 Expressing breast milk, 51
Desferoxamine, 479 D-rylose,4B2 External jugular vein puncture, 456
Desmopressin, 479 Dyselectrolytemias, 273 Extrahepatic disorders, 41
Development, 63 Exudate, l26
Developmental delay, 335 E-B-thalassemia, 295
Developmentai disorders, 330, 336 E
Developmental screening, 67
Development delay,64 Early breast-feeding jaundice, 40 F
Dexamethasone, 480 Early jaundice (within 10 days of age), 35
Dextran 40,480 Early-onset hypocalcemia, 45 F-75,77
Dextroamphetamine, 480 Early-onset sepsis, 27 F-100,77
Dextrocardia, 164 Early stimulation program lor Facioscapulohumeral dystrophy, 289
Dextromethorphan, 480 development, 6B Failure to thrive, 69
Dhatura, 364 Ebstein anomaly, 161, 466 Familial dysautonomia (ri1ey-day
Diabetes insipidus, 238 Econazole nitrate, 503 syndrome), 466
Diabetes mellitus, 232 Eczematous disease, 350 Familial (genetic) short stature, 222
Diabetic ketoacidosis, 233 Edetate calcium disodium, 482 Famotidine, 483
Diaper rash, 350 Edrophonium chloride, 482 Fanconi anemia,466
Diazepam, 480 Edwards syndrome, 466 Fat emulsion, 483
Diazoxide, 480 Effects ofasphlxia, 14 Features on development ofbrain, 64
Dibucaine,48l EFR nomogram, 118 Febrile convulsion, 260
Diclofenac sodium, 481 Ehlers-Danlos syndrome, 466 Feeding expressed breast milk, 51
Dicyclomine, 48J Electrocardiogr aphy, 17 9 Feeding recommendations during sickness
Didanosine, 504 Electrollte disturbances, I 91 andhealth, l00
Diethylcarbamazepine, 505 Electrolyte-mineral solution, 77 Female pseudohermaphroditism, 245
Differenrial diagnosis of rheumaric Electrolytes, 2 1 2 Femoral venipuncture, 456
diseases, 329 Empirical choice of antibiotics, 29 Fetal affection by the Rh-antibody, 37
Differentiating features of physiological and Empyema thoracis, 126 Fetal alcohol syndrome, 466
pathological jaundice, 35 Enalapril,482 Fetal and neonatal circulation, 152
Differentiation of idiopathic neonatal hepatitis Encephalitis, 268 Fetal hypoxia, 13
from biliary atresia,, 42 Encopresis, 333 Fetoscopy,429
DiGeorge syndrome, 427, 466 Endemic goiter,228 Fever of unknown origin (FUO), 420
Digitalis toxicities, 191 Endotracheal intubation, I 5 Fevers in childhood, 417
Digitalization, 177 Enteric fever, 395 Filgastrim, G-CSE 483
Digitalizing dose, 177 Enterobiasis, 4 1 6 Fine motor and adaptive
Digoxin, 177,481 Enteroviral infections, 380 development, 66
Dihydrotachysterol, 48 i Enuresis, 333 First aid f61 251162-"1ule of 5", I l6
Diltiazem,48l Epiglottitis, 1 1 1 FISH, 429
Dimercaprol,4Sl Epileptic encephalopathy, 25 8 Floppy infant, 289
Diphenhydramine, 481 Epinephrine, 482 Flucloxacillin, 500
Diphtheria, 394 Epistaxis, 108 Fluconazole, 503
Disorders of puberty, 246 Epstein pearls, I 1 Fludrocortisone acetate, 48 3
Disseminated intravascular coagulation, 300 Ergocalcilerol, 483 Fluid and electrolyte management, 46
Distinguishing preterm and SFD, 1B Ergotamine, 483 Fluid & nutrition requirements, 19
Disturbances in acid-base status, 216 Erlthema infectiosum, 380 Fluid resuscitation, 218
Diuretics, 178 Ery'thema multiforme, 354 Fluid thrill,4
DMARDs,32i Ery'throcy'te indices, 469 Flumazenil, 483
Dobutamine, 482 Erythromycin, 500 Fluorouracil, 483
Domperidone, 482 Ery'thropoietin, 483 Fluoxetine hydrochloride, 484
Dopamine, 482 Essential newborn care, B Fluticasone, 484
Down syndrome, 430 Ethambutol HCl, 503 Focal segmental glomerulosclerosis, 197
Doxapram, 482 Ethosuximide. 483 Folic acid, 79,484
Doxorubicin hydrochloride, 482 Etoposide, 483 Formula for infusion rarc,45
Doxycycline, 500 Euploid,424 Fragile X syndrome, 434, 466
D-penicillamine, 285 Evaluation of development, 67 Freeman Sheldon syndrome, 466
Drowning,364 Evaluarion oflailure to thrive, 70 Frequenq. of giving complementary
Drug-resistant tuberculosis, 406 Evan syndrome, 466 foods, 54
Drugs used in asthma, 119 Ewing sarcoma, 314 Friedreich ataxia., 466
Drug therapy in children,473 Exchange transfusion, 38, 300 Frohlich syndrome, 466
Dubin-Johnson syndrome, 466 Exclusive breast-feeding, 49 Fulminant hepatic failure, 144
Duchenne muscular d,vstrophy (DMD), 286 Exomphalos, 450 Fungal infections,35l
Ductus-dependent pulmonary {1ow Expanded program on immunization Furazolidone, 505
lesions, lSB (EPI), 370 Furosemide, 484
 INDEX
G Head circumference-lor-age percenriles: girls,
Gabapentin, 484
Galactosemia, 436
Ganciclovir, 504
Gastroesophage al reilux, 446
Gastrointestinal bleeding, 131
Gastrointestinal TB, 403
Gastroschisis, 450
Gaucher disease,442
General examination, I
Generalized seizure, 254
Gene therapy, 430
\Jenetrc counseltng, 42 /
Genetic disorders, 423
Gentamicin, 500
Gentian violec 484
GH deficiency, 223
Giardiasis, 414
Gilbert syndrome, 466
Gilles de la Tourette syndrome, 345
Gingivitis, 390
Glomerular disorders, I 95
Glucagon, 484
Glycogen storage diseases, 440
Goitel227
Gold sodium thiomalate, 484
Gomez classifi cation of malnu
Gonadotropins, 219
Grading of a murmur, 3
Grading ofdengue syndrome, 374
Grading of envenomation, 367
Grading ofesophageal varices, I47
Grading of reflexes, 5
Grading of subependymal-intraventricular
hemorrhage, 22
Graves disease, 230
Gray baby syndrome, 466
Griseofulvin, 503
Gross motor development, 64
Growth,58
Growth assessment, 221
Growth charts, 63, 508
Growth from birth to puberty, 59
Guillain-Barre syndrome, 273
H Hydrops fetalis, 37
Habit disorders, 344
Haloperidol, 484
Hand expression, 51
Hand-eye co-ordination, 66
Hand-Schuller-Christian disease. 466
Hand skills, 66
Hand to mourh co-ordination. 66
Hartnup disease, 466
Hashimoto goiter, 228
Hazards of exchange rransfusion, 39
Headache, 330
Headbanging,344
Head circumference-for-age percentiles: bols
birth to 36 months, 520
birth to 36 monrhs, 521
Hearing loss, 106
Heart failure, 175
Heart rate and rhythm, 184
Heel prick,457
Hemangioma,34T
Hemoglobin E disorders, 294,295
Hemolytic uremic syndrome, 197
Hemophilia, 299
Hemophilia A, 299
Hemophilia B, 299
Hemorrhagic disease of the newborn, 43
Henoch-Schonlein purpura, 325
Heparin, 300,484
Hepatitis, 140
Hepatitis B, 141
Hepatitis C, 142
I-Iepatitis D, 142
Hepatoblastoma, 316
Hepatocellular carcinoma, 316
Hernia and hydrocele, 452
Herpangina, 390
Herpes simplex encephalitis, 271
Herpetic gingivostomatitis, 390
High-risk babies, 45
Hirschsprung disease, 448
tition, 7 2 Histiocytosis syndrome, 31 1 Infl ammatory myopathies, 289
HIV 383
Hodgkin disease, 309
Holt-Oram syndrome, 466
Homatropine hydrobromide, 48 5
Hospital-acquired (nosocomial)
pneumonia, 122
How to write progress note, 6
Human growth hormone, 485
Hunter, 441
Hunter syndrome, 466
Hurlet 441
Hurler syndrome (gargoylism), 466
Hydatid disease (echinococcosis), 414
Hydralazine,4B5
Hydrocarbon poisoning, J60
Hydrocephalus, 279
Hydrochloroth iazide, 485
Hydrocortisone, 485
Hydroxycobalamin, 485
Hymenal tags, 11
Hyperkalemia, 21 5
Hypernatremia, 214
Hyperthyroidism, 230
Hypertrophic cardiomyopathy,
Hypervltamlnosrs A, z8
Hypervitaminosis D, B1
Hypocalcemia, 44, 45
Hypochondroplasia, 440
H1'pogi1'cemia. 44
Hvpokalemia,215
Hrponatremia. 2 13
Hvpoparathvroidism, I j0
Hvpopigmentan- disorders,
3
Hypopituirarism, 220
Hypoplastic left-heart syndrome, I 56
Hypothalamus, 219
Hypothyroidism, 228, 230
I
Ibuprofen, 485
Icterus gravis neonatorum, 37
Idiopathic neonatal hepatitis, 41
Idoxuridine (IDU), 504
IgA nephropathy, 1 97
Imipramine, 485
Immune globulin, 486
Immunization, 370
Incubator care, 19
Indian childhood cirrhosis, 148
Indication of CPAP,47
Indomethacin, 486
Infant and young child feeding,4g
Inlantile colic,342
Infantile hypertrophic pyloric stenosis
(rHPS), 446
Infant of diabetic mother, 44
Infectious mononucleosis, 387
Infective endocarditis, 1 66
Infective endocarditis prophyla-xis, i 67
Insulin, 4B6
Insulin shock, 237
Integrated management of childhood
illness (IMCI),87
Interferon alpha,2a, 48 6
Intermittent mandatory ven tilation, 25, 47
Internal jugular vein cannulation, 457
International agencies and child health, 85
Intestinal atresia, 449
Tntracranial hemorrhage. 2 I
Intrahepatic disorder, 4 1
IVIG,486
Intravenous therapy, 456
Intussusception, 4j0
Invertogram of neonatal resuscitation, l7
Iodine deficiency disorders, 226
Ipecac, 486
Ipratropium, 486
hon,487
Iron-deficiency anemia, 291
Iron (III) hydroxide polymaltose complex, 487
Iron poisoning, 359
Iron therapy, 292
Isoniazid, 503
1 7 1 Isoproterenol, 487
J
Jaundice due to rhesus incompatibiliry,, 37
Joint tenderness, 4
Jur.enile (acquired) hvporhyroidism, 230
Juvenile dermatoml.ositis, 324
Juvenile idiopathic arthritis, 3 1 B
j3
Juvenile poIyp,451
II
 INDEX

K Lupus nephritis, 324 Miltefosine,4l2

Lutembacher sy ndrome, 467 Mitochondrial inheritance, 426
Kala-azar 410 Lyme disease, 326 Mitomycin, 489
Kanamycin,503 Lymph node TB, 403 Mitral regurgitation, 165
Kangaroo mother care, 10 Lyon hypothesis, 426 Mitral stenosis, 165
Kartagener sy ndrome, 467 Mixed acid-base disturbances, 217
Karyotyping,443 Moderate acute asthma, 117
Kasabach-Merritt syndrome, 467
M Modified glasgow coma scale, 409
KATF, 41 1 Mollusca contagiosa, 352
Magnesium hydroxide, 48B
Kawasaki disel.se, 326, 457 Mongolian blue spots, 10
Magnesium sulfate, 488
Kearns-Sayre sy ndr ome, 427 Monosomy,424
Malaria, 408
Kernicterus, 40 Monosulfiram, 506
Male pseudohermaphroditism, 244
Ketamine, 487 Monteleukast, 490
Management of neonatal seizure, 35
Ketoconazole, 504 Morquio disease, 440
Mannitol,4BB
Ketorolac, 487 Morquio syndrome,467
Maple syrup urine disease, 436
Klinefelter syndrome, 433 Mosaicism,424
Marfan syndrome,437
Motor functions, 5
Maroteaux-Lamy syndrome, 467
Mucoid vaginal secretions, 11
L Mastitis neonatorum, 1 1
Mucopolysaccharidosis, 44 1
Masturbation, 344
Multifactorial disorders, 427
Labetalol, 487 Maternal corticosteroid therapy, 24
Mumps,378
Laboratory medicine, 469 Mc Ardle syndrome, 440
Mupirocin, 490,501
Lactational amenorrhea, 53 Mc-Cune-Albright syndrome, 467
MURC association,46T
Lactation and medications, 50 Measles, 377
Murmurs, 3
Lactation failve,52 Mebendazole, 505
Muscle disorders, 286
Lactuiose, 487 Mechlorethamine, 488
Muscle power (MRC scale), 4
Lamivudine,504 Meconium aspiration syndrome, 25
Myasthenia gravis,287
Lamotrigine, 487 Mefoquine, 505
Mycophenolate mofetil, 200
Language development, 66 Megacystis mega-ureter syndrome, 467
Myocardial infarction, 1 90
Larsen syndrome, 467 MEIAS syndrome, 426
Myocarditis, i70, 190
Laryngomalacia, 12 Mendelian disorders: unifactorial
Myoclonic epilepsies, 254
Laryngotracheitis, I 10 disorders,424
Myoclonic seizures, 34
Late-onset sepsis, 27 Meningitis, 264,397
Myotonic dy str ophy, 467
Laurence-moon-biedl syndtome, 467 Meningocele, 11
Lazy Leukoryte syndrome, 467 Meningococcal infection, 397
Lead poisoning, 361 Mental functions, 5 N
Learning disabilities, 339 Mental retardation, 336
Leber hereditary opdc neuroparhy, 427 Mercaptopurine, 4BB Nail biting, 344
Leigh syndrome, 427, 467 MERRF,427 Nalidixic acid, 501
Length-for-age percentiles: boys, birth Metabolic acidosis, 216 Naloxone, 490
to 36 months, 516 Metabolic alkalosis, 216 Naproxen, 490
Length-for-age percentiles: girls, birth Methimazole,4B9 Nasal obstruction, 108
to 36 months, 517 Methotrexate, 489 Nasogastric f eeding, 457
Lennox-Gastaut syndrome, 255 Methyldopa,489 Necrotizing enterocolitis, 42
Leprosy, 353,406 Methylphenidate, 489 Nedocromil, 490
Lesch-Nyhan syndrome. 46- Methylprednisolone, 489 Neomycin sulfate, 501
Letterer-Siwe disease, 467 Metoclopramide, 489 Neonatal cholestasis, 41
Levothyroxine, 487 Metolazone, 489 Neonatal hepatitis syndrome, 41
Lidocaine, 488 Metronidazole, 500, 505 Neonatal infections, 26
Limb-girdle muscular dystrophies, 288 Miconazole, 504 Neonatal jaundice, 35
Limping, 289 Microcephaly, 11 Neonatal seizure, 3 3, 25 8
Lindane, 505 Microdeletion syndrome, 427 Neonatal sepsis, 27
Lipidoses, 442 Micronutrient deficiencies, 75 Neonatal ventilation, 46
Liposomal amphotericin B, 503 Midazolam, 489 Neostigmine,490
Lithium,48B Mid parental height, 221 Nephrotic syndrome, 198
Liver tumors, 316 Migraine, 330 Nervous system, 5

Locomotor system, 4 Mikiry-!ililson syndrome, 467 Netilmicin,50l

Loratadine, 48B Milia, 10 Neuroblastoma, 312
Lorazepam, 488 Miliary TB, 402 Neurodegenerative brain disorders, 282
Low birth-weight neonates, 17 Milk injury, 54 Neurofibromatosis, 438
Lowe syndrome, 467 Milk production, 51 Neuromerabolic disorders, 2B 1
Lumbar puncture, 458 Milk protein intolerance, 138 New Ballard scoring system, 18
Lung abscess, 124 Millennium development goals, 52, 87 Newborn screening,442
INDEX

Newer vaccines, 371 Pentamidine isethionate, 502, 506 Prazosin,492

Niacin, 490 Pentazocine,4!1 Precocious puberty, 248
Niclosamide,506 Pentoxif'lline, 491 Prednisolone, 492
Nicotinic acid (niacin B.), 79 Percutaneous lung tap, 459 Prednisone, 492
Niemann-Pick disease, 442 Pericardial diseases, l6B Prenatal diagnosis, 429
Nifedipine, 490 Pericarditis, 190 Presenting complaints, I
Nitrofurantoin, 501 Perinatal asphla<ia, 13 Prevention in community pediatrics, 85
Nitroprusside, 490 Perinatal herpes simplex virus Prevention of Rh isoimmu nization, 39
Non-Hodgkin lymphoma (NHL), 306 infection, 32 Pri maquine pho.phare. 506
Non-retracrable prepuce, I I Perinatal infections, 29 Primary apnea, 13
Normal sexual differentiation, 244 Periodic syndrome, 344 Primary atypical pneumonia, 122
Normal-term newborn, 7 Peritoneal dialysis (PD), 209 Primary hyperparathyro idism, 249
Nortriptyline, 490 Permethrin, 506 Primary hypothyroidism, 224
Nutritional anrhropomerry, 5B Persistent diarrhea, 132 Primidone, 492
Nutritional rickets, B0 Persistent hy p o gly cemia,, 44, 45 Primordial growth failure, 224
Nystatin,504 Persistent jaundice, 36 Principles of development, l6
Persistent or late-onset PR interval, 181
hypocalcemia, 45 Problems of preterm and term small-for-date
o Personal and social development, 66 babies,19
Pertussis, 392 Procainamide, 492
Obesity, 82
Pervasive developmental disorders, 34 1 Procaine,50l
Obstructive uropathies, 45 1
Peurz Jeghers
'1'ndrome.
46- Procarbazine, 492
Octreotide, 490
Phenobarbital, 491 Procedures in practice, 455
Ocuiar xerophthalmia, 7 7
Phenothiazine, 477 Prochlorperazine, 492
Ofloxacin,50l
Phenothiazine poisoning, 36 1 Progeria, 468
Omeprazole, 490
Phenylketonuria, 435 Prolonged jaundice, 36
Ophthalmia neonatorum, 2/
Phenytoin,49l Promethazine, 493
Opportunistic infections, 3 83
Pheochromocltoma, 243 Prominent xiphisternum, 1 I
Organophosphorus and carbamate
Phimosis,454 Propantheline bromide, 493
poisoning, 363
Photosensitiviry 354 Propranolol, 493
Ornidazole, 504
Phototherapy, 36 Protein-energy malnutrition, 72
Oropaiatodigit al sy ndr o me, 467
Physiological hyperbilirubinemia in term and Prune belly syndrome, 467
Ortolani test, 12
preterm baby,36 Pseudohypoparathyroidism, 250
Osteopetrosis, 467
Physostigmine, 491 Psychopharmac ology, 3 45
Osteosarcoma, 31 5
Phytomenadione, 492 Psychosocial deprivation, 224
OraTeq,372
Pica,332 Pulmonary atresia with intact ventricular
Otitis externa, 105
Pierre-Robin syndrome, 467 septum, 158
Otitis media, 104
Piperacillin sodium, 502 Pulmonary atresia with VSD, 158
Piracetam,492 Pulmonary stenosis, 162
P Piroxicam,492 Purpura,297
Pituitary gland, 219 Purulent sticlry eyes, 27
Pancreatin, 490 Pityriasis alba,354 Pus and exudate culture,456
Pancuronium,4!1 Pivmecillinam, 502 Pyelonephritis, 202
Pandemic infuenza A (HlNl), 418 Pizotifen,492 Pyodermas, 352
Paracetamol,4T3 PKDL, 411 Pyrantel pamoate, 506
Paracetamol poisoning, 359 Pleural effusion, 126, 402 Pyrazinamide, 503
Paraldehyde, 491 Pneumococcal pneumonia, 121 Pyridoxine, 493
Paralysis offacial nerve, 5 Pneumonia, 119 Pyridoxine (86),79
Parathyroid gland,249 Pneumothorax, 127 Pyrimethamine, 506
Paronychia,2T Poisoning, 357 , 364
Partial seizure, 253 Poliomyelitis, 380
Patau syndrom, 467 Polymerase chain reaction, 442 a
Patent ductus arteriosus, 1 55 Polyploid,424
Pearson syndrome,427 Pompe disease, 440 QRS, 181
Pediatric EEG,262 Portal hypertension, 147 QRS complex, 184
Pediculosis, 348 Positioning and attachment, 50 Quinine,507
Pedigree,427 Posterior urethral valves, 452
Pelviureteric junction obstruction, 452 Post-term inlants, 43 R
Pemoline,491 Postural drainage, 125
Pemphigus, 353 PQRST rvaves, 179 Rabies,381 1

Penicillamine, 491 Prader-\{'illi svndrome, 46l Racemic epinephrine. 1 10

I
l.
Penicillin G,50i Pralidoxime, 492 Range of possible clinical eilecrs of HBV j
i

Penicillin V 501 Praziquantel, i06 infection. 143 I

'f
.i
 INDEX

Ranitidine, 493 Seizure disorders, 252 Subclavlan veln. 4) /

Rate ofgrowth ofdifferent organs, 58
Reactivation TB, 402
Reactive arthritis, 326
Recombinant DNA technology, 443
Recurrent abdominal pain, 131
Recurrent apnea,22
Recurrent VTL204
Reflexes, 5
Reiter syndrome, 326
Relactation, 53
Renal tubular acidosis, 198
Renin-angiotensin-aldosterone system,
Rescue steroid therapy, 1 17
ReSoMal solution, 77
Respiratory acidosis, 2i7
Respiratory alkalosis, 217
Respiratory distress syndrome
(hyaline membrane disease), 23
Respiratory failure, 12B
Respiratory system, I
Restraint, 456
Retinoblastoma (RB), 313
Rett syndrome, 341,468
Reye syndrome, 468
Rheumatic fever,377
Rheumatic heart diseases, 164
rubavlnn. )()4
Ribofavin, 493
Ribofavin (Br),79
Rickets, B0
Rickets due to mineral deficiency, 81
fufampicin,503
Riley-Day syndrome, 468
Rocking inbed,344
Roseola infantum, 380
Rotarix, 372
Rothmund syndrome, 468
Rotor syndrome, 458
Rough guide to serum bilirubin levels with
respect to dermal staining, 35
Roxithromycin, 502
Rubella, 379
Rubinstein-Taybi syndrome, 468
Russell-Silver syndrome, 468
s Stature-for-age percentiles:
Salmeterol, 493
SAM,73
Sarnat & Sarnat stages ofhypoxic ischemic
encephalopathy, 15
SARS, 386
Scabies, 348
Scarlet fever, 389
Schedule for blood glucose monito ring, 45
I Schmidt syndrome, 468
I School phobia, 332
Scoring system for step care managemenr,
'; Scurry, 79
Seborrheic eczema,349
Secondary apnea, 13
S:condarv hyperparathyroidism, 249
Ii
Senna, 493 Subconjunctival hemorrhage, I 0
Sensory functions, 6 Subcutaneous emphysema, 128
Septic screening, 2B Subdural tap,459
Serological response to hepatitis B virus Subependymal hemorrhage-intraventricular
infection, 142 hemorrhage, 21
Serum sickness, 326 Subtle seizures, 34
Severe acute asthma, 116 Sulfadiazine, 502
Severe acute respiratory syndrome, 386 Sulfadoxine, 494
Severe malaria (SM), 408 Sulfasalazine, 494
Severity ofpersistent asthma, 1 l4 Supine position, 65
Sexually transmitted diseases (STDt, 399 Surfactant replacement therapy, 24
2i2 Shifting dullness, 3 Surgical problems,444
Shock,192 Sweat,472
Short stature, 220 Symptomatic hypo gly cemia, 44
stADH,250,266 Syndromes, 465
Sibling rivalry, 334 Syphilis, 398
Sick sinus syndrome, 187 Systemic hypertension, 171
Signs of meningeal irritation, 6 Systemic lupus erythematosus, 323
Simethicone, 493
Sinus arrhy'thmia, 187
Sinus bradycardia, 186 T
Sinus tachycardia, 186
Sjogren-Larsen syndrome, 468 Theniasis, 414
Skin sepsis/pyo derma, 26 Thlepes equinovarus, 12
Sleep disordered breathing, 10B Tall stature, 225
Snake bite, 367 TB bones and joints, 404
Sodium antimony gluconate (SAG), 506 Teach the mother to treat local infections at
Sodium polysterene sulfonate, 493 home, 103
Somatoform disorder, 343 Teeth grinding, 344
Sotos syndrome, 468 Ten steps of treatment, T4
Spasmodic croup, 1 10 Tension-type headache, 33 1
Spasmus nutans, 468 Terbutaline sulf ate, 49 4
Specifi c electrocardiographic diagnosis in Test for surfactant function,24
certain heart diseases, 192 Testosterone, 494
Spinal cord compression, 273 Tests for CDH, 12
Spiramycin, 502 Tetanus, 393
Spironolactone, 493 Tetanus antitoxin (ATS), 494
Splenectomy, 296 Tetanus immune globulin, 494
Sporadic goiter,228 ttanus neonatorum, 33
Sputum cukure,456 Tetralogy of Fallot, 159
Stages of hepatic encephalopathy, 145 Thalassemia, 294
Stages ofshock, 193 The abdomen, 3
Stanford-Binet intelligence scale, 6B The history, I
Staphylococcal pneumonia, 121 Theophylline, 494
Staphylococcal scalded skin syndrome, 353 The physical examination, 1
The precordium, 2
boys, 2 to 20 years, 518 Thermal protection in newborn, 9
girls, 2 to 20 years, 519 thiamine,494
Status epilepticus, 259 Thiamine (vitamin B,) deficiency, 78
Step care plan for >5 year and adult, 1 16 Thioridazine, 494
Step care plan lor children <5 year,716 Thoracentesis, 458
Stevens-Johnson syndrome, 355 Throat swab culture, 455
Stool culture, 455 Thumb sucking, 344
Storing expressed breast milk, 51 Thyroid gland,225
Stork bites, 10 Tics,344
Streptokinase, 493 Time of eruption and shedding of primary
Streptomycin, 503 teerh. 63
1 15 Strongyloidiasis, 4 1 7 Time of eruption of permanent reerh, 63
Sturge tVeber syndrome, 468 Tinea versicolor, 352
Stuttering, 334 Tissue plasmin ogen activ ator, 49 4
Subacute sclerosing panencephalitis Tobramycin, 502
(SSPE), 468 Toilet training, 67

I
t
 .,fl

INDEX

f.
Tolmetin sodium, 495 Undescended testes, 453 \Taterhouse-Friderichsen syndrome, 468
Tongue-tie, 1 1 UNICEF,86 \7eber-Christian syndrome, 468
Tonic seizures, 34 Urinary tract infection, 202 \Weight for age,524
Total anomalous pulmonary venous return, 163 Urine culture, 455 'Weight-for-age percentiles :
Tourette syndrome, 345 Ursodeoxycholic acid, 49 5 boys, 2 to 20 years, 510
Toxic erythema, 10 Ursodio1,495 boys, birth to 36 months, 508
Toxoplasmosis, 30 Urticaria, angioedema, 349 gir1s, 2 to 20 years, 51 1
TPA,494 Urticarial vasculitis, 349 girls, birth to 36 months, 509
Tiacheoesophageal fi stula, 445 Use of ECG, 184 \Weight lor lenght, 528
Tiacheomalacia, 13 Veight-forJength percentiles :
Tianexamic acid,495 boys, birth to 36 months, 512
Tiansfusion-chelation therapy, 295 V girls, birth to 36 months, 513
'Weight-for-stature
Tiansient tachypnea ofthe newborn, 26 percentiles :
Vaccines, 371
Tlansposition ofthe great arteries, 162 boys,5l4
Vaginal bleeding, 11
Ti'ansudate, 126 girls, 5 1 5
Valproic actd,496 tVelcome trust classification, 72
Transverse my ehtis, 27 2
Vancomycin,502 til/est syndrome, 255
teacher*Collins syndrome, 468
Varicella, 388
Tieatment modalities of hyperbilirubinemia in \7et nursing, 52
Varicella and zoster, 388
LB\(l babies, 37 V{HO classification of under-nutrition, 72
Varicella zoster virus infection, 33
Tieatment of hypertension, 174 \7ilm tumor (nephroblastoma), 314
Vasculitis; 328
teatment of persistent cholestasis, 42 Wilson disease, 284
Vasculitis syndrome, 327
Ti'etinoin, 495 \(olfsyndrome,468
Vasopressin, 496 -Wolman
tiamcinolone, 495 disease:, 468
Venipunctures, 456 \X/orld Health Organization, 85
Tiiamterene, 495
Ventral suspension, 64
tichuriasis, 416 \{?PSI-III-UK 3rd edition, 68
Ventricular septal defect, 154
Tiicuspid atresia, 1 58
Vesicoureteric ref ux, 205
Tiientine, 495
YIIIhuman,474 X
tifuperazine, 495
Vinblastine sulfate, 496
Tiimethoprim, 502
Vincristine, 496 X-linked dominant disorders, 426
Trisomy,424
Viral hepatitis, 140 Xlinked dominant inheritance, 425
Tiivandrum development screening chan, 67
Viral meningitis, 267 X-linked recessive disorders, 426
Tiomerhamine. 495
Viral pneumonia, 121 Xlinked recessive inheritance, 426
Tiopical pulmonary eosinophilia (TPE), 417
Viral rhinitis, 107 Xylomerazoline, 497
Tropicamide, 495
Vrslon ancl heafing, b/
Tiue hermaphr oditism, 245
Vitamin A, 496
Tirberculin test: Mantoux test (MT), 401 ,.
Vitamin A defr,ciency, 77 Y
Tuberculoma. 40J
Vitamin B,r,4B5
Tuberculosis, 400
Vitamin B-complex, E and K deficiencies, 79 Ylinked disorders, 426
Tuberculous meningitis, 2tr-. 403
Vitamin E, 496
Tuberous sclerosis, 439
Vtiligo, 353
Tubular disorders, I 98
Von Gierke disease, 440
z
Turge-tWeber syndrome, 438
Ti,rrner syndrome, 432 Zafirhkast,49-
Typhoid lever. J95 W Zaicitabine,505
Zellw eger syndrome, 46 8
Warfatin,497 Zidovudine, 505
"1X/arm bottle" method, 51 Zinc,497
U
Warm chain, 9 Zinc defr,ciency,82
Ulcerative colitis, 140 \(arning signs at various ages, 65 Zollinger-Ellison syndrome, 468
'S/ans, J52
Umbilical hernia, 11 Zoster, 38B
Umbilical sepsis, 27 'Warer deprivation iesr. 2J9 Z-score of a PEM p^tient, 72

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