Medstar Pedi

MEDSTAR
CLINICAL GUIDE AND

SYNOPSIS
First Edition
PEDIATRICS
FOR
Prepared By REVISION
ONLY
Medical Students of 2008 E.C Batch
Jimma University, Ethiopia
MEDSTAR
CLINICAL GUIDE AND
SYNOPSIS
FirstEdition
PEDIATRICS
MEDSTAR CLINICAL GUIDE AND SYNOPSIS - PEDIATRICS
TABLE OF CONTENTS
PREFACE---------------------------------------------------------------------------------- IX
ACKNOWLEDGMENT----------------------------------------------------------------- XI
CONTRIBUTERS----------------------------------------------------------------------- XII
Chapter 1 - Introduction to Pediatrics-------------------------------------15
Pediatrics History taking------------------------------------------------------- 15
Physical Examination------------------------------------------------------------20
Chapter 2 - Immunization------------------------------------------------------- 22
Type Of Vaccination-------------------------------------------------------------- 24
Side effects of vaccines---------------------------------------------------------- 26
Chapter 3 - GROWTH AND DEVELOPMENT------------------------------28
Growth.-------------------------------------------------------------------------------- 28
Development-------------------------------------------------------------------------32
Causes of Abnormal Development------------------------------------------ 34
Tanner staging (Sexual Maturity Ratings)------------------------------- 40
Chapter 4 - Approach to Generalized Body Swelling---------------- 41
Conditions and differential diagnosis--------------------------------------41
Malnutrition------------------------------------------------------------------------- 44
SAM (Severe Acute Malnutrition)--------------------------------------------47
Approach To Malnutrition------------------------------------------------------48
Complications-----------------------------------------------------------------------56
Management Principles--------------------------------------------------------- 58
Prepared by Jimma University Medical Students of 2008 E.C. Batch

Chapter 5 - Approach to Renal diseases---------------------------------- 69
AKI (previously called acute renal failure)------------------------------- 69
Approach to patient with AKI------------------------------------------------- 71
Glomerular disease--------------------------------------------------------------- 77
ACUTE POST STREPTOCOCCAL GLOMERULONEPHRITIS

(APSGN)------------------------------------------------------------------------------- 79
HSP (Henoch-Schönlein purpura)------------------------------------------ 83
Hemolytic- Uremic Syndrome------------------------------------------- 75
Nephrotic syndrome-------------------------------------------------------------- 91
Chapter 6 - Approach to shortness of breath (dyspnea)--------- 100
HEART FAILURE-----------------------------------------------------------------103
RHEUMATIC HEART DISEASE---------------------------------------- 111
INFECTIVE ENDOCARDITIS-------------------------------------------- 127
Congenital Heart Diseases--------------------------------------------------- 139
ACYANOTIC CONGENITAL HEART LESIONS------------------ 145
CYANOTIC CONGENITAL HEART LESIONS-------------------- 147
Chapter 7 - Approach to cough---------------------------------------------- 171
Approach to Acute Cough---------------------------------------------------------173
PNEUMONIA-----------------------------------------------------------------------174
Foreign body aspiration------------------------------------------------------- 188
Approach to chronic Cough------------------------------------------------------ 188
Tuberculosis----------------------------------------------------------------------- 189
Pertussis---------------------------------------------------------------------------- 211

Chpter 8 - APPROACH TO WHEEZE--------------------------------------- 215
CHILDHOOD ASTHMA-------------------------------------------------------- 230
ACUTE BRONCHIOLITIS----------------------------------------------------- 246
CHapter 9 - APPROACH TO STRIDOR------------------------------------ 251
Differential diagnosis-----------------------------------------------------------252
Chapter 10 - Approach to altered mental status--------------------261
Level of consciousness---------------------------------------------------------261
Malaria-------------------------------------------------------------------------------281
Meningitis-------------------------------------------------------------------------- 293
Sample history for meningitis --------------------------------------------- 306
CHapter 11 - Approach To Poly Symptoms (Polyuria, Polydipsia,

and Polyphagia)--------------------------------------------------------------------- 308
Differential Diagnosis of Polyuria with Polydipsia------------------ 309
A. Diabetes Mellites (Type 1)------------------------------------------------ 310
B. Diabetes Mellites (Type 2)------------------------------------------------ 315
C. Central Diabetes Insipidus (Neurogenic)--------------------------- 322
D. Nephrogenic Diabetes Insipidus---------------------------------------322
Chapter 12 - APPROACH TO DIARHEA---------------------------------- 326
Classification---------------------------------------------------------------------- 328
Differential diagnosis of diarrhea------------------------------------------329
Acute Gastroenteritis-----------------------------------------------------------331
Sample history for AGE------------------------------------------------------- 341
CHapter 13 - APPROACH TO ABNORMAL BODY MOVEMENT---- 343

Seizure ------------------------------------------------------------------------------ 344
Sydenham chorea--------------------------------------------------------------- 351
Spasm-------------------------------------------------------------------------------- 351
Tremor------------------------------------------------------------------------------- 352
Sample history of Abnormal body movement-------------------------354
CHapter 14 - Approach to Body Weakness----------------------------- 356
Weakness--------------------------------------------------------------------------- 356
Poliomyelitis----------------------------------------------------------------------- 361
Guillain-Barre syndrome (GBS)------------------------------------------ 370
Brain Tumor----------------------------------------------------------------------- 382
Transverse myelitis------------------------------------------------------------- 385
Myasthenia gravis (MG)------------------------------------------------------- 386
Toxin Exposure-------------------------------------------------------------------389
Sample History------------------------------------------------------------------- 395
Chapter 15 - Approach to fever----------------------------------------------398
CHapter 16 - Approach to Jaundice---------------------------------------408
DDx for Jaundice---------------------------------------------------------------- 411
HEPATIC CONDITIONS------------------------------------------------------- 411
I. VIRAL HEPATITIS------------------------------------------------------------ 411
II. Chronic Liver Disease (CLD)---------------------------------------------415
III. Liver cirrhosis---------------------------------------------------------------- 415
IV. HEPATOTOXICITY--------------------------------------------------------- 418

V. Autoimmune Hepatitis---------------------------------------------------- 421
VI. Metabolic Disorders------------------------------------------------------- 423
Causes of isolated hyperbilirubinemia---------------------------------- 428
Chapter 17 - Essentials of Neonatology--------------------------------- 430
History and physical examination of neonates-----------------------430
Neonatal history taking------------------------------------------------------- 430
Physical examination of newborns----------------------------------------430
Classification of the Newborn-----------------------------------------------436
Approach on selected cases------------------------------------------------- 438
Respiratory distress------------------------------------------------------------ 438
A. Neonatal sepsis--------------------------------------------------------------- 439
Perinatal asphyxia---------------------------------------------------------------444
Respiratory distress syndrome(HMD)------------------------------------449
Neonatal jaundice(hyperbilirubinemia)--------------------------------- 454
Sample History 1---------------------------------------------------------------- 461
Sample history 2----------------------------------------------------------------- 462
Miscellaneous------------------------------------------------------------------------ 454
Chapter 18 - APPROACH TO ANEMIA------------------------------------ 464
Classification of Anemia------------------------------------------------------ 464
Approach to the Patient------------------------------------------------------- 466
Diagnostic Approach By Laboratory Test Abnormalities--------- 475
Chapter 19 - Hemorrhagic disorders-------------------------------------- 478

Hemostasis------------------------------------------------------------------------- 478
Dissorders Of Hemostasis----------------------------------------------------479
Approach to a patient---------------------------------------------------------- 481
Chapter 20 - Skin lesions in pediatrics----------------------------------487
Primary skin lesions------------------------------------------------------------487
Secondary skin lesions-------------------------------------------------------- 487
Some common skin lesions--------------------------------------------------489
Scabies----------------------------------------------------------------------------489
Tineacapitis--------------------------------------------------------------------- 491
Tungiasis-------------------------------------------------------------------------494
Chapter 21 Pediatrics HIV/AIDS--------------------------------------------497
Etiology------------------------------------------------------------------------------ 497
Lifecycle----------------------------------------------------------------------------- 497
Transmission---------------------------------------------------------------------- 498
HIV transmission---------------------------------------------------------------- 499
Natural history------------------------------------------------------------------- 499
Systemic Manifestations of HIV/AIDS-----------------------------------500
HIV Exposed Infants (HEI)--------------------------------------------------- 506
Diagnosis of HIV infection----------------------------------------------------510
Treatment principles----------------------------------------------------------- 512
CHapter 22 Approach to Dysmorphic feature------------------------ 514
1. DOWN SYNDROME--------------------------------------------------------- 514

2. Edward syndrome (Trisomy 18)---------------------------------------- 527
3. TURNER SYNDROME (45, X)------------------------------------------- 531
Appendix ------------------------------------------------------------------------------ 528
References----------------------------------------------------------------------------- 539

PREFACE
This first edition of MEDSTAR CLINICAL GUIDE AND SYNOPSIS of
PEDIATRICS is one of the three sister books, which are; MEDSTAR
CLINICAL GUIDE AND SYNOPSIS of INTERNAL MEDICINE and
GYNECOLOGY. It is presented as a gift for our fellow medical
students across Ethiopia to be a guide through their clinical year
attachments. As its name suggests, we are hoping it will be like a star
in the sky showing directions to “med-travelers” through their
journey into becoming a Good Doctor.
It is prepared in the way we thought is simple and also inclusive of

major cases. In order to do so, primarily we identified common chief
complaints in our wards which must be discussed and are also
frequently encountered during our ward stay. Even though this
approach could address majority of cases, there were some cases
which are most important but couldn’t be addressed via the chief
compliant approach. So we included them by giving them the name
Miscellaneous including RVI; as you know this disease entity affects
almost every system presenting with many chief complaints. The
other point is that we have also included the general format of
pediatric history and physical examination, and details on
immunization and development under introduction part in the
beginning of the book.
In this Book major chief complaints are written as topic and their
differential diagnosis are listed. Approach for each chef complaint
and the listed differential diagnoses is written in the way it could lead
to a diagnosis of the specific differential diagnosis. It is presented by
listing what could a patient have in History (symptoms, risk factors
and complication), Physical examination (positive finding in each
system), Investigation (positive findings in each modality) and finally
only the management principles. At the end of each topic we have
prepared sample histories for the selected cases under their chef
complaints. We have tried to use Nelson as a major reference and
other latest editions of guidelines, Web-pages, uptodate and other

Books. After preparing the document, senior students, medical
interns and senior doctors had commented on it.
Medicine is an ever-changing science. As new research and clinical

experience broaden our knowledge, changes in treatment and drug
therapy are required. That’s why we have mentioned on the cover
page that this Book is “FOR REVISION ONLY “. Additionally, this
book is like a guide and students need to broaden their knowledge via
reading standard books, guidelines and other reading materials.
In conclusion, we are privileged to have compiled this edition and are

enthusiastic about all that it offers to our readers. We have learned
much in the process of compiling MEDSTAR and hope that you will
find this edition valuable as a guiding resource. We have tried to
make this guide as readable as possible. This work is just a start and
without doubt there might be some flaws in the document, but we are
going to add details and include more cases in the coming editions.
We have prepared an Email address and Telegram bot link so that we

could get your comments on the Book, so that we could come up with
a better MEDSTAR Book of second edition.
Email address: MEDSTARcomment@gmail.com
Telegram bot link: @MedstarCommentsBot
The contributors

ACKNOWLEDGMENT
The contributors of Medstar clinical guide and synopsis of pediatrics
wish to acknowledge the help and support of Dr. Temam Kedir (MD,
pediatrician), whose comments and suggestions for some of the cases
became invaluable for this book. We also appreciate the hard work of
our contributing team for their sacrifice committed to this book. In
addition, we would like to extend our thanks to Nafiyad Fekadu (MD),
Oliyad Shagana (MD), Markos Mehertab (MI), Birhanu Eshetu (MI),
and Negussu Legesse (C2) for their constructive comments and also
by encouraging us as well.
Finally, we are ever grateful for the support and encouragement of

EMSA JIMMA and all JU medical students.

CONTRIBUTORS
The Whole Medstar Team
MEDSTAR - Pediatrics Team

MEDSTAR - Pediatrics Contributors Name List
1. Benti Shentema 12. Kirubel Misganaw

2. Betelhem Birhanu 13. Lemmi Shiferaw
3. Dereje Gondol 14. Martha Fisseha
4. Dinaol Dinagde 15. Mehfira Abrar
5. Elsabeth Sisay 16. Meron Bayable
6. Eyasu Girma 17. Minase Getachew
7. Fasika Solomon 18. Mulat Alemu
8. Fikradddis Cheru 19. Nahom Asnake
9. Jordan Ashenafi 20. Nardos Befekadu
10. Kassa Kebede 21. Oliyad Yadeta
11. Kinfegebreal Wagaye

Page left blank Intentionally

CHAPTER 1
INTRODUCTION TO PEDIATRICS
Pediatrics History taking
DATE AND TIME OF CLERKING: Helps to compare current condition of
the patient with his condition at your time of clerking. Some patients
may improve while others may deteriorate within minutes to hours of
your clerking
IDENTIFICATION
 Name: helps to identify patient’s records.

 Age: Epidemiology of diseases varies with age. Knowing the age
can narrow our differential diagnosis even etiologic agents vary
with as in case of Pneumonia. While managing the patient we
need to adjust doses of drugs based on age.
 Sex: some diseases exclusively occurs in males or females.
Example Hemophilia occurs in males.
 Pediatric age group: We can predict the developmental
milestones the child expected to meet.
Pediatric age group Age
Neonate Birth-1month
Infant Up-to 1yr
Toddler 1-3 yrs
Preschool aged 4-6 yrs
School aged 7-12 yrs
Adolescent 13-18 yrs

 Address: prevalence and distribution of disease varies

geographically and among various cultural group.
Eg. A person who came from Malaria endemic area we need to be
suspicious. The prevalence of HIV, TB and other medical
conditions varies from place to place and from culture to culture.
 Hospital, ward and bed no.
 Date of admission
 Mode of arrival: Usually they arrive being supported by their
parents because they are neonates, infants or may become
irritable. However on elder children it is suggestive of severity of
the illness.
 Referral: what was the diagnostic impression there; what was
the treatment given there.
 Source of History (historian): usually parents but on older
children you can take from the child himself.
 With /without translator: may affect the quality of information.
PREVIOUS ADMISSIONS:
 Specify when, where, why (what was the illness), treatment &
outcome.
 If the patient P/A has direct relation with the current complaint,
it should be included in HPI. So you can say P/A is included in
HPI.
Sample
Date and time of clerking: Tuesday 07/02/2012 at 2:00 PM
Identification
This is Rufael Endashaw a 4 years old preschool aged male child from
Jimma zone, Mana Woreda admitted to JUMC, Pediatrics level one ward,
bed no. 3, 4 days back after being referred from a local health center. The
source of history is his mother with no language barrier.
Previous Admission
No History of previous admission
CHIEF COMPLAINT
The main reason the patient seek medical attention.

N.B Patient may have more than one complain but don’t forget to take
the main complain he came to hospital so that you can build your
differential diagnosis around the chief complaint and reach on
diagnosis.
A child may come with
 Shortness of breath
 Cough
 Wheeze
 Diarrhea
 Generalized body swelling
 Abnormal Body Movement
 Altered mental status
 Yellowish discoloration of eye/skin
 Abdominal pain
 Fever
 Body weakness etc….
Characterization of the chief complaint and ruling in/out the

differential diagnosis is discussed under each chapter of the book
with sample history for each so you can refer them depending on
your patient chief complaint.
NUTRITIONAL HISTORY
 Current nutrition:
 Staple food, type of food and composition of food (Eg. Enjera
made of tef or bread made of wheat..)
 Amount and frequency of meals per day
 Does the child finish his dish and does he share dishes with
siblings.
 Previous nutrition:
 Breast feeding History: Initiation of breast feeding, duration
of exclusive breast feeding (EBF), frequency of breast feeding,
switching of breasts, and total duration of breast feeding.

 Complementary feeding: Time of initiation, food type, and

frequency.
N.B Previous nutritional history is usually
important in under 2 yrs children.
 Sunshine exposure:
 Normally the child must be exposed in the early morning sun
undressed, with no oil/petroleum application for
20-30min.
N.B Asses for children 2wks–1yr old
DEVELOPMENTAL HISTORY
 All of the four developmental milestones should be assessed.

 If there is any developmental delay or regress then try search at
which age of the child the development began to deviate from
normal by tracing back to earlier age.
For example if a 3 years old child has developmental delay
then assess his developmental milestone at the age of 2yr,
1yr, 9mo… go back to get when the development began to
delay.
Details of the milestone discussed under Growth and
Development
IMMUNIZATION HISTORY
 Has the child completed the EPI schedule? Does he have the
yellow certificate card?
 If not vaccinated, why?
Details of Vaccination and the EPI schedule is
discussed under Immunization chapter

N.B In most of pediatrics diseases Nutritional, Immunization,

and Developmental Histories are a pertinent History so
include them in HPI.
PAST MEDICAL & SURGICAL HISTORY
 Childhood illnesses like measles, chicken pox, mumps…

 Previous History of same illness
 History of medication and drug allergy
 History of surgery and trauma
PERSONAL, FAMILY & SOCIAL HISTORY
 Personal History: birth place, growth, parents name and

occupation, academic life, current achievement and how the
illness affects his personal life
 Family History: family size; birth order, siblings’ health
status; maternal health; mother & father living together or
separated, family income
 Social History:
 Housing and no. of people per room (WHO Crowding
Criteria)
 Animals, pet, poultry living together
 Kitchen in the same house -exposure to domestic smoke.
 Waste disposal system: diarrheal diseases are usually
associated with poor personal and environmental hygiene.
REVIEW OF SYSTEM
Helps you to pick up what u have missed to ask.

Physical Examination
G/A: ASL/CSL or well looking
Vital Signs: Normal range of vital signs for specific age group
Age Heart rate Respiratory rate Blood pressure
(beats/min) (breath/min) (mmhg)
Premature 120-170 40-70 55-75/35-45
0-3 month 100-150 35-55 65-85/45-55
3-6month 90-120 30-45 70-90/50-65
6-12month 80-120 25-40 80-100/55-65
1-3 years 70-110 20-30 90-105/55-70
3-6years 65-110 20-25 95-110/60-75
6-12years 60-95 14-22 100-120/60-75
12+ 55-85 12-18 110-135/65-85
Age Breath/min
0-2month >= 60 breath/min
2month- 1yr >= 50 breath/min
1yr - 5yr >= 40 breath/min
5yr- 8yr >= 30 breath/min
>8yr >= 24 breath/min

Systolic Blood Pressure Calculation
Systolic blood pressure calculation in children over 1 year old:
1. Median SBP = 90 mmHg + (2 x Age in years)
2. Minimum SBP = 70 mmHg + (2 x Age in years)
Anthropometry: You have to be able to calculate WFA, HFA, WFH,

MUAC, BMI and HC for age and interpret it by WHO growth curves.
Discussed under SAM and you can also use WHO chart from the
annex
N.B While you are attaching pediatrics make sure that you are able to
take anthropometric measurements and interpret them using the
growth curves.
DO HEAD TO TOE EXAMINATION

CHAPTER 2
IMMUNIZATION
VACCINATION
 is the administration of any vaccine or toxoid for prevention of

disease.
IMMUNIZATION
 Is The Process Of Inducing Immunity Artificially.
IT CAN BE ACTIVE OR PASSIVE
oActive immunization involves stimulating the immune

system to produce antibodies and a cellular immune
response against infectious agent through the use of a
urine vaccine or toxoid.
oPassive immunization provides temporary protection
through the administration of exogenous antibody or
maternal antibody transfer to the foetus.
 Determinants of the Immune response

The nature and magnitude of the response to a vaccine or toxoid
depend on the following factors:
1. Age – presence of high concentration of maternal antibody and
immature response to some vaccines in the first four months of
life impair immunization. The measles vaccine is given at 9
months of age to reduce this effect.
2. Route of administration.
Vaccines given orally induce mucosal secretary IgA
e.g. OPV vaccine
Using an improper route to administer the vaccine may
reduce the immune response e.g. where BCG is administered IM
rather than intradermal

3. Nature of vaccine
Live attenuated vaccines induce immunity with a single dose
which lasts longer than inactivated ones
4. Genetic
Individuals genetically vary in their ability to respond to the
same vaccine.
5. Potency
Ensuring the potency of a vaccine, especially live attenuated,
requires keeping the cold chain.
Table 1 The main antigenic preparations
Type of antigen Vaccine example

Living organism Natural Small pox vaccine.
Attenuated * Polio (sabin) OPV,
Measles,
Mumps, Rubella, BCG
Intact but non Virus Polio (Salk)
living organisms
Bacteria Pertussis
Sub cellular Capsular Meningococcal
Fragments disaccharid
Surface antigen Hepatitis B.
Toxoids Tetanus, diphtheria
 Live attenuated vaccines, particularly viral ones like measles,

confer life long protection after a single immunizing dose, since
they closely simulate natural infection and contain the greatest
number of microbial antigen. Their drawbacks are:
o Reversion to wild type can lead to disease
o They can cause severe disease in immunocompromised
children
o Some people exhibit hypersensitivity to viral antigens.

Table 2 - Administrations of Vaccines
VACCINE DOSE ROUTE OF SITE OF

ADMINISTRATI ADMINISTRATION
ON
BCG Infants: 0.05 Intradermal Right deltoid region of the
ml0.1ml for arm
children>1yr
DTP 0.5ml Intramuscular Upper, outer portion of the
thigh
Polio 2 drops, or Oral Mouth
Measles 0.5 ml Subcutaneous Outer ,upper part of left arm
Tetanus 0.5 ml Intramuscular Deltoid region of the
Toxoid upper arm
Type Of Vaccination
1. ROUTINE IMMUNIZATION
The Expanded Programme on Immunization in Ethiopia
 The Expanded Programme on Immunization (EPI) was established by the

World Health Organization in 1974 to control vaccine preventable diseases.
 EPI in Ethiopia covers
 BCG (Bacillus-Calmette-Guerin)
 Diphtheria
 Hib (Haemophilus influenza type B)
 Hepatitis B
 Measles
 Pertussis
 PCV (Pneumococcal Conjugated Vaccine)
 Oral polio virus (OPV)
 Rotavirus
 Tetanus

Table 3: Immunization Schedule
Vaccination for infants
Age Visit Antigen

Birth 1 BCG, OPV-O
6 weeks 2 Pentavalent (DPT + Hep B + Hib),
OPV1, PCV1, Rotavirus
10 weeks 3 Pentavalent2, OPV2, PCV2, Rotavirus
14 weeks 4 Pentavalent3, OPV3, PCV3
9 month 5 Measles
2. CATCH-UP VACCINATION
For children below 5yr but not vaccinated in the first one year (catch-up
vaccination) the following should be administered:
Table 4: catch up vaccination
visit Vaccination
1st visit DPT
OPV
Mantoux test,
BCG if test negative in 3 days
2nd visit (1 month after 1st DPT
visit OPV
3rd visit (1month after 2nd DPT
visit) OPV
Measles, if not exposed
4th visit (1 year later) DPT
OPV
5th visit (4 years later) (adult type)
1. Outreach (Mobile schedule for remote areas):

This service gives coverage for people living within 20 km radius of the
health facility.
2. Mass campaigns:
National immunization days (NIDs) for polio (currently active) and
measles.

Side effects of vaccines

BCG:
Kochs phenomenon – self limiting acute inflammatory reaction four days
after vaccination.
Indolent ulcer – ulcer persisting 12 wk after vaccination or ulcer more than
10mm, mainly resulting from deep injection or secondary infection.
Deep abscess – abscess at site of injection or draining lymph nodes due to
subcutaneous or deep injections.
 Disseminated disease with BCG – 1 per million vaccines results in BCG
causing active disease especially in immunosuppressed children.
OPV: Paralytic polio from vaccine strain polio-virus
DPT: The major side effect is from the pertussis component
 Superficial injection can cause injection site abscess a week later.
 Encephalopathy ( 0.3 – 3/100,000 vaccines)
 Convulsion (0.3 – 90/100,000)
 Shock like state or collapse (hypotonic – hypo responsiveness episode)
(0.5 –30/100,000)
 Permanent brain damage (0.2 – 0.6/100,000)
MEASLES: May have mild reaction likes low grade fever irritability and allergic
reactions.
 Major side effects include (per 100,000 vaccinations):
o Encephalopathy (0.1)
o Convulsions (0.02 – 100)
o Sub acute sclerosing panencephalitis (0.01 – 0.05)
Contraindication
 The general contraindications for all vaccines include:
o Anaphylactic reaction
o Moderate to severe illness
o Live-attenuated vaccines for severely immunosupressed patient (exception
is measles) Not contraindications
o Moderate fever after prior vaccine dose
o Moderate local reaction after inject able vaccine
o Mild acute illness
o Prematurity (same dose as for full-term infants)
o Severe malnutrition (rather strong indication)
o Penicillin allergy (personal or family)

 The contraindications for specific vaccines include:

BCG : Symptomatic AIDS
OPV:
 Severe immune suppression
 Diarrhea is not contraindication .Give OPV without registering it
and repeat OPV 4 weeks later.
DPT:
Encephalopathy within 7 days of Previous dose not attributable to
another cause
Seizures if poorly controlled or new onset, defer until control.
Cold chain
 “Cold Chain” refers to the process used to maintain optimal
conditions during the transport, storage, and handling of vaccines
starting at the manufacturer and ending with the administration to
the patient or client.
 As vaccines are sensitive, their potency and effectiveness may be
negatively impacted if they are exposed to freezing temperatures,
Heat or direct sunlight
Table 5: Recommended Storage Temperature of EPI
Types of vaccine Storage temperature

Most Oral polio -15oC to – 25oC
sensitive Measles (freeze dried) -15oC to – 25oC
to heat DPT 2oC to 8oC
Least BCG (freeze dried) 2oC to 8oC
sensitive Tetanus toxoid 2oC to 8oC
to heat
In addition, protection from light is a necessary condition for some vaccines.

CHAPTER 3
GROWTH AND DEVELOPMENT
Stages of Growth and Development

o Infancy
 Neonate - Birth to 1 month
 Infancy - 1 month to 1 year
o Early Childhood
 Toddler - 1-3 years
 Preschool - 3-6 years
o Middle Childhood
 School age - 6 to 12 years
o Late Childhood
 Adolescent - 13 years to approximately 18 years
1. Growth.
 Diseases tend to have more impairment when they occur
during period of rapid growth.
 Deviation of child's own pattern of growth and development is
more significant than deviation from standard growth chart.
 Rate of growth is more important than actual size. So serial
measurement of growth is best indicator of health.
A. Weight
 Best index of nutrition and growth (especially in acute illnesses)
 1 kg = 2.2 Ib (pound)

Age Weight
At birth 3.2 kg / 7 Ibs
Birth - 10th day 10% of BW lost Because of:
 At 10th day  BW is regained  Loss of meconium
 Loss of urine
 Loss of physiologic
edema
 less intake
1 - 3 months increases by 200 g/wk
3 - 12 months increases by 150 g/wk
 6 months  Doubles the BW
 1 year  Triples the BW
2 years 4 times the BW
2 yr - puberty Increases by 5 Ibs / year
 7 years  7 times the BW
 10 years  10 times the BW
Puberty Growth spurt - rapid
weight gain
Formulas for approximate average weight
Age Weight (kg)
3 – 12 ⴘᎯ t
months
1 - 6 years ⴘᎯ t t
7 – 12 years ⴘᎯ t t t
B. Height
 Affected by chronic illnesses
Age Height
At birth 50 cm
1 year 75 cm
2 years 85 cm = Half of the adult's
height
3 years 90 cm
4 years 100 cm
up to puberty increases by 5 cm / year
At puberty growth spurt - increases

by 9 - 10 cm /yr for 2-3

years
Formulas for approximate average height

Age Height (cm)
2 – 12 years ⴘᎯ t  tt
C. Head circumference
 Estimate of brain growth
 HC is larger than chest circumference at birth; and equals at 1
year.
 HC rapidly increases during infancy.
 Small brain indicates:
 Abnormal brain growth
 Craniosynostosis (premature closure of sutures)
Age HC
At birth 35 cm
3 mn 41 cm
6 mn 44 cm
9 mn 46 cm
1 year 47 cm
2 years 49 cm
3 years 50 cm
5 years 51 cm
Up to puberty (12 years) in Increases by 0.5 cm / year
 12 years  54/55 cm
D. Dentition
 First eruption - at 6 months of age
 Last eruption - at 2.5 year (by now has 20 teeth in total)
 First shedding of deciduous teeth - at 6 years
 Last shedding (completed) - at 12 year
 Delayed eruption - considered when there are no teeth by
approximately 13 mo of age.

 Common causes include:

 Hypothyroid
 Rickets
 Hypoparathyroidism
 Familial
 Idiopathic (the most common)
 Mechanical blockage (crowding, gum fibrosis)
Chronology of Human Dentition of Primary (Deciduous) and Secondary

(Permanent) Teeth
 For Primary teeth
o Age at eruption:
 5 – 7 months old – Mandibular central incisors
 6 – 8 months old – Maxillary central incisors
 7 – 10 months old – Mandibular lateral incisors
 8 – 11 months old – Maxillary lateral incisors
 10 – 16 months old – Maxillary and mandibular 1st molars
 16 – 20 months old - Maxillary and mandibular canines
 20 – 30 months old - Maxillary and mandibular 2nd molars
o Age at shedding:
 6 – 7 years old - Mandibular central incisors
 7 – 8 years old -
Maxillary central incisors and
Mandibular lateral incisors
 8 – 9 years old - Maxillary lateral incisors
 9 – 11 years old - mandibular canines
 10 – 12 years old –
maxillary and mandibular 1st molars
maxillary 2nd molars
 11 – 12 years old – maxillary canines
 11 – 13 years old - mandibular 2nd molars
 For permanent teeth:
 6 – 7 years old –
Mandibular central incisors

maxillary and mandibular 1st molars

 7 – 8 years old –
Maxillary central incisors and
Mandibular lateral incisors
 8 – 9 years old - Maxillary lateral incisors
 9 – 11 years old - mandibular canines
 10 – 11 years old – Maxillary 1st premolars
 10 – 12 years old –
Mandibular 1st premolars
Maxillary 2nd premolars
 11 – 12 years old – maxillary canines
 11 – 13 years old – Mandibular 2nd premolars
 12 – 13 years old - maxillary and mandibular 2nd molars
 17 – 22 years old – Maxillary and mandibular 3rd molars
B. Development
 A child’s development represents the interaction of heredity and the
environment on the developing brain.
Child’s development
Affected by interaction of heredity and

environment
Environment
Heredity
influences the extent to which that
determines the potential of the potential is achieved
child
Env’t has to meet 2 needs for
for by
Prepared
Needed school age
Jimma childrenMedical Students of 2008
University E.C.for
Needed infants from their parents
Batch
o There is variation in the pattern of development between

children.
o There are four fields of developmental skills:
 Gross motor
 Vision and fine motor
 Hearing, speech and language
 Social, emotional and behavioral.
 Developmental milestones - the age of acquisition of important
developmental skills.
 The median age - the age when half of a standard population of
children achieve that level; it does not tell us if the child’s skills are
outside the normal range.
 Limit ages - the age by which the developmental milestones
should have been achieved.
o Major evaluating steps when analyzing a young child’s
developmental progress.
1. Questioning - consider the child’s age and then focus your
questions on the areas of likely current developmental progress
(shortcut approach)
2. Equipment - offer the child suitable toys to find out about
skills through play
3. Observation - observe how the child uses the toys and interacts
with people.

Development is in a cephalo-caudal direction.

The age when there is the most rapid emergence of skills in each
developmental field:
 The first year of life - gross motor development

 1 year of age onwards - vision and fine motor development
 18 months of age onwards - hearing, speech and language
 2.5 years of age onwards - social, emotional and behavioral
development
Then make a focused questioning considering the age of the
child. Thus for a child aged:
 less than 18 months – ask first gross motor abilities ->

acquisition of vision and hearing skills ->
questions about fine motor
 18 months to 2.5 years – ask first acquisition of speech and
language -> fine motor skills -> only brief gross motor skills
 2.5 to 4 years – ask speech and language -> social,
emotional, and behaviour development.
When evaluating a child’s development, consider:
 Each skill field separately

 The sequence of developmental progress
 The stage the child has reached for each skill field
 If progress is similar in each skill field
 Only at the end, the child’s overall
developmental profile and how that relates to
the child’s age.
Causes of abnormal development

Prenatal
GENETIC –

 Chromosome/DNA disorders, e.g. Down syndrome, fragile X

syndrome, chromosome microdeletions or duplications.
 Cerebral dysgenesis, e.g. microcephaly, absent corpus
callosum, hydrocephalus, neuronal migration disorder
CEREBROVASCULAR - Stroke – haemorrhagic or ischaemic
METABOLIC - Hypothyroidism, phenylketonuria
TERATOGENIC - Alcohol and drug abuse
CONGENITAL INFECTION - Rubella, cytomegalovirus, toxoplasmosis,
HIV
NEUROCUTANEOUS SYNDROMES - Tuberous sclerosis,
neurofibromatosis, Sturge–Weber, Ito syndrome
Perinatal
 EXTREME PREMATURITY - Intraventricular haemorrhage
/periventricular leucomalacia
 BIRTH ASPHYXIA - Hypoxic-ischaemic encephalopathy
 METABOLIC - Symptomatic hypoglycaemia, hyperbilirubinemia
Postnatal
 INFECTION - Meningitis, encephalitis
 ANOXIA - Suffocation, near drowning, seizures
 TRAUMA - Head injury – accidental or non-accidental
 METABOLIC - Hypoglycaemia, inborn errors of metabolism.
 CEREBROVASCULAR - Stroke
 NUTRITIONAL DEFICIENCY - Maternal deficiency (breast fed), food
intolerance, restrictions
 OTHER
Unknown (about 25%): chronic illness, physical abuse,
emotional neglect

 Developmental problems often present at an age when a specific

area of development is most rapid and prominent. Therefore:
 Motor problems - during the first 18 months of age,
 Speech and language problems - between 18 months and 3
years,
 Social and communication disorders - between 2–4 years of
age
Adjusting for prematurity
If a child has been born preterm, the anticipated developmental

skills of a 9-month-old preterm baby (chronological age) born 3
months early (at 28 weeks’ gestation) are more like those of a
6-month-old baby (corrected age).

DEVELOPMENT (MEDIAN AGES)
Neonate 3 months 6 months 9 months 1 year
Gross  Head  Lifts  Lifts head and  Riches for  Walk with support
motor lag head chest objects  Stands
 Tonic  Sits  Sits without  Pulls to independently
neck supporte support stand
reflex d  Rolls over both  Cruises
 Walk  Roll from way  Crawls
reflex stomach
to back
Fine  Fisted  Grasps  Palmar grasp  Pincer  Turns pages of book

motor hands rattle  Follow objects grasp  stacks 2 blocks
 Pupillar  Follow all directions
y reflex objects  Transfers
180° object hand to
hand
Speech  cries  coos  Monosyllabic  Polysyllabic  Says “mama” or

and  startle  turns to babble babble “Dada” (10 months)
language to loud nearby  Inhibits to  Speaks first real
noise voice “no” word
Social  Mostly  Loughs  Everything to  Stranger  Waves bye bye

and sleeps  Anticipat mouth fear  Points to desired
behavior  Primitiv e food on  Friendly with objects
e site strangers 
reflexes
 Social
smile
(4-6 wk)

18 months 2 years 3 years 4 years 5 years
Gross  Walk  Runs well  stands  Hops on 1 foot  skips

motor alone  walks up momentaril  throws ball  walks backward
steady and down y on 1 foot overhand  run stairs
 walks stairs, 1  walk  uses scissors
upstairs step at a stairs to cut out
(1 step at time alternatin pictures
a time)  jumps g feet  climbs well
 Throws  climb on  Rides
ball furniture tricycle
Fine  stacks 3  stacks 6  stacks 9  copy cross  copy triangles

motor blocks blocks blocks and square
 imitates  scribbles  copy circle  draws a man
scribbling  copy line with 2-4 parts
 makes a besides head
line with
crayon
Speech  Speaks  speak 2-3  gives full  speech is  Names 4 colors

and 10-15 word name intelligible to  Counts up to 10
language words sentences  knows age strangers  Define nouns
 names and sex  tell a story
pictures
 identifies
1 or more
parts of
body
Social and  Feeds self  Handles  helps in  Plays with  Dresses and
behavior spoon well dressing several undresses
 helps to (unbuttons children alone
undress clothing  goes to toilet  asks questions
and puts on alone (toilet about meaning
shoes) trained) of words
 washes  engages in
hands domestic
role-playing

Red flags (limit ages)

Tanner staging (sexual maturity ratings)

For females:
SMR Pubic hair Breasts
stages
1 preadolescent preadolescent
2 Sparse, lightly pigmented, straight, Breast and papilla elevated as small

medial border of labia mound; areola diameter increased
3 Darker, beginning to curl, increased Breast and areola enlarged, no contour

amount separation
4 Coarse, curly, abundant (but less Areola and papilla form secondary
than in adult) mound
5 Adult feminine triangle, spread to Mature, nipple projects, areola part of

medial surface of thighs general breast contour
For males
SMR Pubic hair Penis Testes
stages
1 None preadolescent Preadolescent
2 Scanty, long, slightly Minimal enlargement Enlarged scrotum,

pigmented pink, texture altered
3 Darker, starting to curl, small Lengthens Larger

amount
4 Coarse, curly, resembles adult Larger; glans and Larger, scrotum dark
type (but less quantity) breadth increase in size
5 Adult distribution, spread to Adult size Adult size

medial surface of thighs

CHAPTER 4
APPROACH TO GENERALIZED BODY
SWELLING
Body swelling: is a compliant of a patient that may suggest the
presence of edema (accumulation of excess interstitial fluid).
Can be localized or generalized:
 Localized swelling: inflammation, lymph edema, DVT, mass.

 Generalized body swelling: SAM, CHF, CLD, Nephrotic
syndrome.
Characterize the edema as:
 Pitting and non-pitting by pressing for 3-5sec against a bony

prominence on the most dependent areas ( dorsum of the foot,
pretibial, on sacral prominence)
 Grade as:
o Grade 1→only involving the foot
o Grade 2→extends to pretibial
o Grade 3→all the way up to sacral prominence, ascites, and
face.
Patho-physiology: results from an
 Imbalance between intra-capillary hydrostatic pressure

 Imbalance between plasma oncotic pressure and interstitial
oncotic pressure.
 Excess salt and water retention.
Conditions and differential diagnosis

 Increased hydrostatic pressure

o Volume expansion
 Acute glomerulo-nephritis
 Acute tubular necrosis
 Acute and chronic renal failure
 Heart failure
 Venous insufficiency
 Cardiac failure (low output and high output)
 Constrictive pericarditis
o Reduced plasma oncotic pressure
 Nephrotic syndrome
 Chronic liver failure, fulminant hepatitis
 Protein losing entheropathy
 Severe acute malnutrition (edematous)
 Severe burns
 Increased interstitial oncotic pressure
 Hypothyroidism
 Damage to capillary endothelium
 Vasculitis (SLE, anaphyloctoid,….)

 Sepsis
Approach to the patient

History
 Characterize the swelling
o Onset: sudden (sepsis, burn…) or Insidious(Chronic disease of

the heart, liver, kidney)
o Duration
o How patient first recognize swelling eg. Child unable to wear
his shoes or his clothes become tight.
o Pattern of the swelling:
 If from face downward may suggest renal diseases,
because in renal cases normal CVP but increased

hydrostatic pressure and low plasma oncotic

pressure results in excess interstitial fluid in loose
connective tissue area; the eyelid.
 If from leg upwards may suggest cardiac diseases
or SAM, because in cardiac disease increased CVP
results resistance to venous return resulting
peripheral congestion in the lower limb venous
system which ascends against gravity.
 If from abdomen downward to legs may suggest
liver diseases, because Liver disease cause portal
hypertension resulting ascites.
 Weight gain: usually precedes overt GBS, patient
may complain of difficulty putting shoes and cloths
particularly in the evening (why?)
 Associated symptoms:
 SOB,
 fatigue,
 jaundice,
 abdominal distention,
 urinary complaints,
 diarrhea,
 symptoms of hypothyroidism; cold intolerance,
constipation,
 Detailed history on nutrition and development

 Previous history of renal, cardiac, liver diseases and their risk
factors
See the physical Findings in each Differentials
Lab investigations:
See the Lab Investigations in each Differentials

1. Malnutrition
General concepts
 Encompasses the full continuum of under-nutrition and
over-nutrition of both macro and micro-nutrients.
 WHO definition; imbalance between nutrient supply and demand
of the body
 The greatest risk of under-nutrition occurs in the first 1000 days.
 Many poor nutritional outcomes begin in uterus and are manifest
as low birth weight (LBW<2,500 g).
Prevalence of Malnutrition (under-nutrition)

 LBW are highest (26%) in southern Asia, and are twice those of
sub-Saharan Africa.
 In 2011; 16% of children <5 yr of age were underweight (weight
for-age <−2 SD).
 According to the 2014EDHS, the prevalence of GAM at national
level is estimated to be at around 9%.
 And prevalence of stunting was stated to be 40%.
 Fetal growth restriction, sub-optimal breastfeeding, stunting,
wasting and deficiency of vitamin A and zinc jointly contribute to
45% of global child deaths (3.1 million deaths/yr),
Causes of under-nutrition
Nutritional status is determined by three factors:
 Immediate cause: act on the individual, these are inadequate food

intake and infection with their vicious cycle.
 Underlying cause: influence the households and the community;
these are drought, flooding, household food insecurity.
 Basic cause: influence communities and society these include the
country’s economy and political status.
Consequences of Under-nutrition

 Premature death.
 Undernourished women give birth to LBW babies.
 LBW and low weight gain in the first 2 yr are associated with an
increased risk of hypertension, stroke, and type 2 diabetes in
adults.
 Stunted child< 3yrs poorer motor and cognitive development and
altered behavior in later years.
 Iodine and iron deficiencies also lead to loss of cognitive potential.
ASSESSMENT OF NUTRITION - ABCDS

Anthropometry
 Anthropometry is the study and technique of human body
measurement.
 Used to measure and monitor nutritional status of the individual
or population group.
Biochemistry
 Measure levels of chemical substances present in the blood Like:
Hb, albumin, CRP, HbA1c,Na, urea, Ca, Po4, Mg, and
micro-nutrients
 Functional tests measure the function of vital organs such as
the kidneys or liver.
Clinical
 Different clinical manifestations of malnutrition on different
organ systems and
 Diseases that result malnutrition by increasing nutritional
requirements or decreased appetite.
Dietary
 An estimation of the total daily calorie intake, as well as overall
quality of diet is assessed.
 Helpful to understand patterns of eating, portion sizes, cooking
methods and types of food and drink taken.

Classifications
 There are two ways of classifying malnutrition; these are
community survey and clinical.
1. COMMUNITY SURVEY
INDEX GRADING
Gomez (underweight) Weight for age

Combined wasting and 90-75% of median Grade 1 (mild)
stunting 75-60% Grade 2 (moderate)
<60% Grade 3 (severe)
Waterlow (wasting) Weight for height

Acute malnutrition 90-80% of median Mild
<70% Severe
Waterlow (stunting), Height for age
Chronic malnutrition 95-90% of median Mild
90-85% Moderate
<85% Severe
WHO (wasting) Weight for height
Acute(new onset) -2SD to -3 SD Moderate
malnutrition <-3SD Severe
WHO (stunting) Height for age
Chronic malnutrition <-2SD to -3 SD Moderate
<-3SD Severe
WHO (wasting) MUAC
(for age group 6-59 mo) ≥125mm Normal
115mm to 125 mm Moderate
<115 mm Severe
(for age group 5-9 yrs)
≥140mm Normal
≥130mm to < 140 mm Moderate
< 130 mm Severe
(for age group 10-14
yrs)
180mm≥ Normal
(for age group 15-18) ≥ 160 to < 180 mm Moderate
< 160 mm Severe
≥190mm Normal
≥17mm to <190mm Moderate
<170mm Severe

The WHO further classifies SAM as complicated and

non-complicated.
2. CLINICAL
WELCOMES CLASSIFICATION.
 Marasmus: severe wasting; severe weight loss and muscle mass

leaving ‘skin and bones’.
 Kwashiorkor: characterized by edema (nutritional edema) that is a
clinical indicator for SAM.
 Marasmic-kwash: severe wasting + edema; combination of
kwashiorkor and marasmus.
SAM (Severe Acute Malnutrition)

 Is severe wasting (MUAC<11.5cm for children aged 6-59monthes,
weight for height<-3SD) and/or bilateral pitting edema.
 For infants (0-6): recent weight loss or failure to gain weight or
ineffective feeding (attachment, position and suckling).
Clinical presentation
Two clinical syndromes of malnutrition (under-nutrition) are well
recognized with their own patho-physiology and presentations. These
are:
 Edematous SAM (kwashiorkor and marasmic- kwashiorkor)
 Non edematous SAM (marasmus)
Patho-physiology
 Orderly progressing physiologic and metabolic changes to conserve
energy and prolong life.
 This process is called reductive adaptation often in marasmus. i.e.
reduced body homeostasis in order to survive on limited macro and
micro-nutrients intake.

 Atrophied organs and less functional organ system. The functions

of organs is preserved more in non-edematous SAM than
edematous.
 Compensatory breakdown of muscle proteins in non-edematous
SAM makes the reduction in serum proteins to be less extent.
o Dysadaptation results: small bowel over growth, infection, free
radicals and toxin accumulation while low anti-oxidation and
disposal. These results tissue damage and development of
kwashiorkor.
o Failure of the compensatory mechanism in long standing non
edematous SAM will lead to the development of
edema(kwash-marasmus)
FOUR DIFFERENT THEORIES PROPOSED FOR PATHOGENESIS OF

KWASHIORKOR
Different proposed mechanisms

1. Protein energy deficiency
2. Aflatoxin
3. Free radical theory
4. Reductive adaptive theory
Summarized as:
Classic protein deficiency theory:
 Starchy meal upon weaning
Newer free radical damage theory:
 Imbalance between production and disposal of free radicals
 Impaired anti-oxidant defense because inadequate diet ( Vit E,
amino acid,..)
 Toxins from infections and aflatoxins
Approach To Malnutrition
HISTORY
 Age at presentations is 12-36 mo for kwashiorkor.

 It usually occurs at the time of weaning, initiation of adult

diet, which is high in CHO but low in protein.
 Severe protein and essential amino acid deprivation while
adequate caloric intake.
 For marasmus, age at presentation 6-12 mo (“infantile
marasmus”)
 In non breast fed or inadequately breast feed infants
 'Late marasmus' in older children living on inadequate
diets for prolonged period.
 Both protein and CHO are inadequate.
SYMPTOMS DIRECTLY RESULTING FROM UNDER-NUTRITION:
Kwashakor
 Generalized body swelling staring from the legs.
 Loss of appetite, vomiting, abdominal distention
 Diarrhea as a result of mal-absorption, bacterial overgrowth,
vit A deficiency
 Growth retardation and mental changes together with GBS
are the three essential features of kwashiorkor.
 Symptoms of anemia; vertigo, easy fatigability, listlessness,
tinnitus, pica (craving for non edible matters; iron
deficiency)
Marasmus
 Growth retardation; failure to gain weight earliest
manifestation.
 Irritability, continuously crying because of hunger
 Good appetite, unlike kwashiorkor.
 Diarrhea and symptoms of micro-nutrient deficiencies.
Marasmic-kwashiorkor
 Clinical features of both marasmus and kwashiorkor are
present.

 Acute or chronic protein deficiency in a patient with chronic

energy(CHO) deficiency
 Muscles of the upper limbs are wasted while the lower limbs
appear swollen.
SYMPTOMS RESULTING FROM THE COMPLICATIONS OF UNDER-NUTRITION
INFECTION:
 Fever, chills, malaise… may not be apparent b/c low

immune response.
 Respiratory System (cough, fast breathing,…) and
GIT(diarrhea, vomiting,…) being most common. GUT
(dysuria, frequency,urgency,..), ear and eye, skin, etc…
HYPOGLYCEMIA:
 In severe cases, twitching, convulsions, limpness and loss

of consciousness.
HYPOTHERMIA:
 Patient may Shiver and be drowsy.

CORNEAL ULCERATION
 Early symptom of Vit A deficiency; night blindness because

absence of rhodopsin
 Late stage complete blindness or refractive defect because of
corneal ulceration or scaring.
RISK FACTORS
 Inadequate food intake due to scarcity of food cause primary

malnutrition
o Primary malnutrition commonly seen in children< 5 yrs
 Factors that decrease absorption, increase metabolic requirements
and decrease appetite cause secondary malnutrition.
o Inadequate food intake: loss of appetite (infection or chronic
illnesses), inadequate feeding (scarcity of food; economic status)

o Decreased absorption of nutrients: AIDS entheropathy,

intestinal TB, parasites, cow’s milk intolerance, …
o In many of these cases the child may present with failure to
thrive and chronic or persistent diarrhea.
o Increased daily nutritional requirements: physiologically

children and adolescents have more nutritional requirements.
o Others: infection, Parasites, especially hookworms,
Schistosomes, malaria, cause blood loss, which increases
nutrient needs.
To asses those risk factors it is important to ask the following part of
patient history
 Nutritional intake habit of the patient, past/present medical
history and personal, family and social history.
 Also consider asking immunization history because infection is the
commonest complication in malnourished children and one of the
immediate causes.
PHYSICAL EXAMINATIONS (SIGNS)
G/A: moon face (sparing peri-orbit) and apathy (kwashiorkor),

simian face (marasmus), visible wasting, CRD
Moon face Simian/ old man’s face

Vital Signs:
 BP; hypotension b/c shock and CHF

 PR; bradycardia (electrolyte abnormality and decreased mass of
ventricles)
 RR; tachypnea b/c of pneumonia, CHF
 T◦; fever T◦≥37.5◦c (for axillary) due to infection or hypothermia
T◦<35◦c (for axillary) because low energy for heat production.
Anthropometry: wt, ht/length for <2yrs
 BMI:< -2SD (thinness), between +1SD and +2SD (overweight) and

>+2SD (obesity).
 WFH/WFL: is an indicator of acute malnutrition ;( see WHO
classification)
 WFA: easy to measure but doesn’t differentiate between stunting
and wasting. (See Gomez classification)
 HFA/LFA: is a measure of linear growth, deficit represents the
cumulative impact of adverse events, (See WHO classification)
 MUAC: for community level screening (see above)
HEENT
 Head: craniotapes, frontal bossing, delayed fontanel closure (vit D)
 Eyes: dry eyes, pale conjunctiva, icteric sclera, periorbital edema

(kwashiorkor), broom stick eyelashes, eye lid retraction
(hypoglycemia)
 Sunken eye balls (marasmus), due to atrophy of
retro-orbital tissue also seen in dehydration b/c
vasoconstrictions of orbital Venus plexus.
 Bitot spot, xeropthalmia or keratomalasia (vit A),
 Ear: signs of otitis media, tragus and ante-tragus tenderness.

 Nose: bleeding nasal mucosa,

 Mouth and throat: dry oral mucosa and oral trash, angular
cheilosis, glotitis (Vit B2), spongy bleeding gums (vitamin C),
parotid enlargement, enamel mottling, delayed eruption (Vit D).
LGS
 SLAP: infection
Respiratory System:
 visible wasting, costo-chondral bead ( vit C; sharp and vit D

dull), Harrison’s groove
 Crepitation, decreased breath sounds, dullness (pleural
effusion) because of pneumonia and CHF.
CVS:
 raised JVP (CHF), small vessel vasculopathy, week pulses

 Slow capillary refill>3sec: press the tip of the finger nail and see
how fast the pallor is lost, up on releasing the pressure.
Abdomen:
 Distended (port belly), hepatomegaly with fatty liver is only

seen in kwashiorkor. Fluid thrill and shifting dullness

Musculoskeletal:
 Edema characterizes as pitting or non-pitting, and grade 1-3

 Muscle wasting and thighs
 Baggy pant appearance (See
picture)
 Sagging of the skin
around the buttocks of
the patient.
 Sign of hypocalcaemia Chvostek
sign: twitching of the face while
striking on jaw angle and
 Trousseau sign: flexion of metacarpo-phallengeal joint and
extension of inter phallengeal joints while inflating the BP cuff.
 Skeletal deformities (vit D,vitC and calcium deficiencies)
Integumentary
 Skin: Cold and clammy skin; shock, loose and wrinkled

(marasmus), shiny and edematous (kwashakor),
Different skin manifestations of malnutrition:

ACRODERMATITIS ENTEROPATICA:
 Inability to absorb sufficient Zn from diet

 Symmetrical vesiculobullous,
psoriasiform skin lesion on
perioral, acral, and perineal
areas.
 A serum zinc<50 µg/dL is
suggestive,
 Chronic diarrhea, stomatitis,
glossitis, paronychia, nail
dystrophy
 Growth retardation,
irritability, delayed wound
healing and infection.
Essential fatty acid deficiency

 Generalized, scaly dermatitis with thickened scally plaque
 Failure to thrive, alopecia, poor wound healing and
thrombocytopenia.
Kwash-Dermatoses
 Diffuse fine reddish brown
scaling (enamel/flky paint
sign).
 It is clinically graded as;
 Grade 1 localized
hypo/hyper pigmentation
 Grade 2 localized patchy
desquamating lesion
 Grade 3 secondary changes with fissure, ulcer and
exudative lesions with bacterial infection.
Pellagra
 Inadequate intake or malabsoption of niacin and
tryptophan.
 Edema, erythema, and burning of sun-exposed skin.
 Provoked by burns, friction and inflammation.
 Butterfly distribution on face and ‘casal's necklece'.
 Blisters and scales; skin increasingly becomes dry, rough,
thickened, cracked, and hyper-pigmented.
Vitamin A deficiency
 Proliferation of basal cells; hyperkeratosis, and formation of
stratifid cornifid squamous epithelium
 Hyperplasia of the epidermis lining hair follicles and
sebaceous glands
 Dry, scaly, hyperkeratotic patches; on the arms, legs,
shoulders, and buttocks.
Vitamin C deficiency

 Follicular hyperkeratosis; coiling of the hair on the upper

and lower extrimities
 Vascular fragility because Vit C is essential for collagen
synthesis.
 perifollicular erythematic particularly on the legs and
hemorrhage of gums and sub-priosteum.
 Best diagnosed by Vit C supplementation test
 Hair:
 Dull, sparse, alopecia, brooms stick eyelashes (Vit A

deficiency)
 Brownish discoloration; flag sign, interchanging periods
of protein deficiency and adequacy.
 Brownish discoloration is due to; low melanin
production b/c low serum essential amino acids.
 Easily pluck-able: hair is plucked by the examiner without
inducing pain.
 Nails: kolynichia, thin and soft nail plates, fissure and ridges.
Neurologic:
 lethargic, apathy, irritable, weak reflexes, impaired memory.
Investigations
 Diagnosis of SAM is clinical but investigations to look for
complications and underlying causes depending on the evidences
we have from history and physical examination.
o CBC, RBS, organ function tests, serum electrolyte, CXR, stool
exam, blood film,…
 Video on how to do appetite test
 https://youtu.be/gSZL6cudKVY
Complications
Infection

 An immediate cause and can result from low immunity of

malnutrition (vicious cycle).
 Poor prognosis owning to young age of the patients, severity of
the infection and presence of complications.
 Bronchopneumonia and gram negative septicemia are
common infections.
Hypoglycemia
 When serum glucose is <54 mg/dl(<3mmol/L)
 Mostly seen in marasmus and is not common to be
symptomatic.
 It is often associated with septicemia.
 Early and adequate feeding usually avoids this complication
Shock
 Feared emergency complication seen in SAM
 Can be hypo-volumic or septic
 Immediate diagnosis and appropriate management is critical
 Patient may be lethargic or unconscious
Hypothermia
 Medical emergency in malnourished patients.
 Often in severely wasted children and is associated with high
mortality.
 Results from Impairment of thermoregulatory control and
decreased energy stores.
 In addition, the loss of thermal insulation b/c of emaciation.
Severe anemia
 Is when Hb<4g/dl or Hct< 12%)
 Has different causes; iron and folate deficiency, low serum
protein, blood loss (hook worm,…), anemia of underlying
chronic illness,…

Corneal ulceration
 Eye changes rarely occur before the age of 2
 The cornea keratinizes, becomes opaque, is susceptible to
infection
 Forms dry, scaly layers of cells (xerophthalmia).
 Infection develop and cornea degenerates irreversibly,
keratomalesia and corneal ulceration
 Keratinization of conjunctiva, Bitot spot
OTHERS:
 Dehydration and electrolyte imbalance,
 skin lesions,
 CHF (a complication of the Treatment.)
Management Principles
Two steps of treatment;
I. Stabilization
 Shock, hypoglycemia, severe dehydration, very severe

anemia (Hb< 4g/dl) and corneal ulceration needs
Emergency treatment before stabilization.
 The stabilization phase repair cellular function, correct
fluid and electrolyte imbalance, restore homeostasis.
 Prevent death from the interlinked triad of hypoglycemia,
hypothermia, and infection.
II. Rehabilitation.
 The rehabilitation phase restores wasted tissues (i.e.,
catch-up growth).
 It is essential that treatment proceeds in an ordered
progression and that the metabolic machinery is repaired
before any attempt is made to promote weight gain or
correction of deficiencies.

o E.g iron should not be given to severely malnourished

child who can’t make Hb because of reductive
adaptation, in order to prevent the accumulation of
iron in excess.
Management (SAM guideline of ETHIOPIA 2019)

1. Admission
ADMISSION CRITERIA FOR STABILIZATION CENTER
 Young infants (0-6mo): if any one of these is present.

 WFL<-3SD
 Any grade of bilateral pitting edema
 Recent weight loss or failure to gain weight.
 Ineffective feeding (attachment, positioning and suckling)
directly observed for 15-20, minutes,
 Presence of signs and symptoms of the medical complications
 Any medical or social issue needing more detailed assessment
 Age group 6-59mo: if any one of these is present.
 Bilateral pitting grade 3 edema
 Bilateral pitting grade 1or 2 edema
 Severe wasting( MUAC< 11.5cm or WFH< -3SD) plus bilateral
pitting edema of any grade
 WFL/H<-3SD or MUAC <11.5cm and any sign ofmedical
complications or failed appetite test
 Or referred from out patient care
 Age group 5-18: if any one of these is present.
 WFL/H < -3Z score
 MUAC in severe category
 BMI for age <-3SD
 Edema of both feet (+, ++), PLUS any one of the medical
complications , or Failed appetite test
 Grade 3 edema

 Marasmic Kwashiorkor (WFL/H < -3SD with edema, OR MUAC

in severe category with edema)
CRITERIA FOR OUTPATIENT THERAPEUTIC PROGRAM (OTP)
 Aged 6-59 months

 Bilateral pitting edema grade 1 or 2 or
 MUAC <11.5cm or
 WFH< -3SD and
 Appetite test passed
 No medical complication
 Clinically well and alert
 Or referred from SC for stabilization
2. Management of SAM in stabilization centre Phase-1

(stabilization phase)
Treatment is always given in an in-patient setting. During this phase,
 Life-threatening medical complications are treated
 Routine drugs(vitA, folic acid, antibiotics, de-worming,…) are

given to correct specific deficiencies
 Feeding with F-75 milk (low caloric and sodium) is begun
TRANSITION PHASE:

 Here the patients are going to be prepared for phase

2(rehabilitation) and F75 in phase 1 is changed to F100 or
RUTF
 Transition is done when patient improve appetite and
complications are treated.
Criteria to transfer patients between the phases of management is
as follows
Details of criteria
From Phase 1 to  Return of appetite (easily finishes the
Transition Phase F-75 feeds) and
 Subsiding bilateral edema or minimal
edema (++ or less)
 No serious medical problems; vomiting,
watery diarrhea, dehydration, respiratory
distress, that needs NGT or IV line
Back from Signs of fluid overload develop

Transition Phase
to Phase 1  Weight gain more rapidly than 10g/kg/
associated with increased RR,
 Rapid increase in the size of the liver
 Increasing edema appearance of new
edema
 If tense abdominal distension develops
If the patient gets significant re-feeding
diarrhea so that there is weight loss
If patient develops medical complications
If NG tube or IV line is needed

Recovering patients can progress to OTP. Only those who cannot >
75% of RUTF remain in SC.
Phase 2 (rehabilitation phase)

 Can be given both in inpatient and out patient

 Vigorous approach to feeding is required to achieve very high

energy intakes and rapid weight gain of > 10 g/kg/day.
Phase 2 inpatient: when treating the patient in outpatient is not
reasonable; no capable care giver, no enough supply of RUTF and
patient doesn’t tolerate RUTF.
Phase 2 outpatient: when there is capable care giver, enough RUTF,

OTP program is near to home,…
Patients in phase 2, who develop any signs of a complication or any

clinical deterioration, should be returned to Phase 1.
Criteria to transfer patients between the phases of management table 2
Details of criteria
From Transition phase to Phase Transition takes 2-3 days.
2 feeding  Good appetite, taking all of
the F100 or >75% of the
RRUTF, for OTP
 Definite reduction of edema
(grade 1,2)
 For wasted patients,
complete absence of edema.
 Medical complications are
resolving
 Clinically well and alert.
Back from Phase 2 to Phase 1  Any signs of a complication

 Increase/development of
edema
 Development of re-feeding
 Diarrhea sufficient to lead
to weight loss
 Weight loss for two
consecutive weighing
 Static weight for three

consecutive weighing
 Fulfilling any of the criteria
of “failure to respond to
treatment”
Every treatment options should be followed to assess response and

possible complications.
 Good responses are measured by good weight gain ≥10g/kg/day,

gain of appetite, resolving of edema, and are criteria to transfer
patients to the next phase of management.
 Failure to respond to treatment can be primary or secondary;
Criteria to label a patient failed to respond table 3
Criteria Approximate time after

admission
Child in Phase I (Primary Failure)
Failure to regain appetite Day 4
Failure to start to lose edema Day 4
Edema still present Day 10
Failure to enter Phase 2 and gain Day 10
more than 5g/Kg/d
Child in Phase 2 ( Secondary Failure) During phase 2

Failure to gain at least 5 g/kg/day
for three successive
days after feeding freely on F-100
 Causes to failure to respond can be patient related or health

facility related
 Patient related problems can be underlying diseases and most
common are TB and HIV
 Others; mal-absorption, Vitamin and mineral deficiency.
 Health center related problems are improper diagnosis,
treatment, follow-up and recording of SAM patients.

Follow up vital signs and recognizing danger signs

Summary of danger signs table 5
Vital signs Description Suggestions:

Pulse and  Confirmed increase in Infection or
Respirations pulse rate of Heart failure (possibly
≥25beat/min, from over hydration due
 with confirmed increase in to feeding or rehydrating
RR ≥5 breath/min too fast)
Respirations  Fast breathing: Pneumonia

only ≥50 breaths/minute in
child 2-12 months
 ≥40 breaths/ minute or
more in child 12 months
up to 5 years
Temperature  Temperature <35 Hypothermia (possibly
due to infection, a
missed feed, or child

Temperature ≥38.5 being uncovered)

Any sudden increase or
decrease of axillary temp Infection
within the normal range.
Other danger signs include:
 Anorexia, abdominal distention, increased vomiting,

jaundice, bruising, large weight changes
 Cyanosis, difficulty of breathing and new edema
 Mental changes, drowsiness
 For infants of age 0-6 mon management should focus on:

 Prioritize re-establishing effective exclusive breastfeeding
 Promote and support the mother or caregiver to breastfeed.
 Assess the physical and mental health status of mothers or
caregivers.

3. Discharge
Criteria for discharge and transferring to OTP
Stabilized and Transferred to OTP
 Passed a RUTF appetite test; the child eats more than 75% of the
daily RUTF prescription and start of weight gain.
 Medical complications are resolving.
 Bilateral pitting edema is decreasing (if severe wasting with
bilateral pitting edema admission bilateral pitting edema is
completely resolved).
 Clinically well and alert.
DISCHARGE CRITERIA FOR INFANTS AGE (0-6MON)
 Infant is feeding well with the replacement feed for non breast fed
infants
 Successful re-lactation and effective breast feeding(for breast fed
infants)
 Adequate weight gain on exclusive breast feeding(for breast fed
infants)
 Has adequate weight gain and has a WFL ≥ -2 z-score.
 No bilateral pitting edema.
 Clinically well and alert.
 Infant has been checked for immunization and other routine
interventions
 Mothers or caregivers have been linked with community-based
follow-up and support
DISCHARGED AFTER FULL RECOVERY (FOR 6-59MO OF AGE)
 The anthropometric indicator that is used to confirm SAM should

also be used to assess whether a child has reached nutritional
recovery.
 If admitted with bilateral pitting edema, discharge cured when:
 No bilateral pitting edema for 2 consecutive visit

 AND MUAC ≥ 12.5 cm or WFH/WFL ≥ -2 SD

 AND Clinically well and alert
 If admitted with MUAC, discharge cured when;

 MUAC ≥ 12.5 cm
 AND No bilateral pitting edema
 If admitted based on WFH/WFL, discharge cured when:

 WFH/WFL ≥ -2 z-score
 AND No bilateral pitting edema
 The discharge criteria of OTP admitted patients is the same

with SC admitted patients.
Check details of management on SAM guideline 2019.
Complications in managing SAM

 Re-feeding syndrome
 It may follow overly aggressive enteral or parenteral
alimentation.
 When excessive carbohydrates are administered, the resultant
increase in serum insulin levels may produce hypokalemia,
hypophosphatemia, and hypomagnesemia.
 The hallmark of this condition is hypophosphatemia (≤0.5
mmol/L ) and clinically weakness, rhabdomyolysis, neutrophil
dysfunction, cardio-respiratory failure, arrhythmias,
seizures,altered level of consciousness, or sudden death may
occur.

Sample history
This is a 2 years old toddler, who is presented with GBS of 10 days duration
which has gradual onset from the legs and progress upward and involves the
abdomen and the face. In association with this he has history of loose stool 4
times a day and loss of appetite of 1 month duration and cough and fast
breathing of 2 day duration. The mother also stated he is less cheerful than
before and cries a lot.
He was exclusively breast feed for 4 months and started on cow milk, which
was diluted with water, 50% and latter gruel, which he takes 2 times a day;
and wean from breast milk at the age of 1 yr.
Now he is on family diet; bread made off teff and sauce made of potato and
lentils. He usually fails to finish his portion.
The food is prepared by the mother once a day and the dishes used to serve
are washed.
He was exposed to sun starting from the age of 1 month
He started sitting at the age of 6 months and standing at the age of 1yr and 6
month and he cannot walk still.
He is fully vaccinated
He lives in a house of 2 rooms 1 door and 2 windows with his mother and 4
other siblings; 3 older and one younger.
The mother is seronegative for RVI
Otherwise no history of:
 Difficulty of breathing
 Yellowish discoloration, stool and
 Frequency, urgency, dysuria, urine color change
 Vomiting, nausea,
 Skin rash, joint swelling
 LOC, sleepiness, abnormal body movement
 No history of contact with TB patient,/chronically
coughing
Prepared by Jimma University person
Medical Students of 2008 E.C. Batch
 Family history of same illness


CHAPTER 5
APPROACH TO RENAL DISEASES
Classification of renal disease based on:
1. Duration
 Acute kidney injury
 chronic kidney disease(rare in pediatrics age goup)
2. Histological – Glomerular disease
AKI (previously called acute renal failure)

It is a clinical syndrome in which a sudden deterioration in renal
function results in inability of the kidneys to maintain fluid and
electrolyte homeostasis
 It is clinical diagnosis not structural
 In adults objectively diagnosed based on Serum creatinine level in
three ways (one of the three)
 rise of Scr from baseline of at least 0.3 mg/dl within 48 hrs.
 at least 50% higher than baseline within 1 week.
 a reduction in urine output to less than 0.5ml/kg/hr for longer
than 6hrs.
 A modified RIFLE criteria (pRIFLE) was developed to characterize the pattern
of AKI in critically ill children.

Pediatric-Modified Rifle (pRIFLE) Criteria

Criteria Estimated CCL Urine output
Risk eCCL decrease by 25% <0.5 ml/Kg/hr for 8 hr
Injury eCCL decrease by 50% <0.5 ml/Kg/hr for 16 hr
Failure eCCL decrease by 75% or <0.3 ml/Kg/hr for 24 hr or
eCCL <35 ml/min/1.73m² anuric for 12 hr
Loss Persistent failure >4 wk
End-stage End-stage renal disease
(persistent failure >3 mn)
CCL: creatinine clearance; eCCL: estimated creatinine clearance
Epidemiology
 Occurs in 2-3% of children admitted to pediatrics tertiary care
centers
 As many as 8% of infants in neonatal ICU.
Causes (DDx)
Classified as Pre-Renal(Most common cause), Renal(Intrinsic),
Post-Renal. (This classification is for management purpose)
 Pre- renal causes - In general anything that causes decrease blood
flow to the kidney and there is no structural abnormality in the
kidney.
 If the underlying cause of the renal hypoperfusion is reversed
promptly, renal function returns to normal. If hypoperfusion is
sustained, intrinsic renal parenchymal damage can develop.
 Dehydration,
 Hemorrhage,
 Sepsis,
 Hypoalbuminemia,
 Cardiac failure
 Renal causes -
 Glomerulonephritis
o PSGN
o SLE
o HSP
o Anti-glomerular basemen membrane
 HUS
 Acute tubular necrosis
 Cortical necrosis
 Renal vein thrombosis
 Tumor infiltrations
 Tumor lysis syndrome
 Post-renal causes –
 Uretropelvic junction obstruction,
 Posterior urethral valve
 Urethrocele
 Tumor
 Hemorrhagic cystitis
 Urolithiasis,
 Neurogenic bladder
 Tumor.
Approach to patient with AKI

Although diagnosis of AKI is objectively, History and Physical
Examinations will give us some clue about the cause.
1. History
 PRE RENAL CAUSES
 Vomiting, diarrhea, abdominal pain, decrease urine output

likely pre renal due to volume loss secondary to AGE but HUS
(See later) should be ruled out.
 In patient with body swelling starting from leg, SOB, PND,
orthopenia – likley cardiac cause.

 History of significant blood loss.

 RENAL CAUSES
 In a patient with age between 5-15 ys with History of sore

throat or skin infection, body swelling which starts from
his face, hematuria leads to the likely diagnosis of
postinfectious glomerulonephritis (renal cause).
 history of drug intake –nephrotoxic drugs like
Antibiotics( aminoglycosides and amphotericin B)
contrast agents, chemotherapeutic agents (Cisplatin
and carboplatin )
 self or family history of epilepsy ,History of burn injury
resulting in rabdomyolysis which leads to intrinsic cause
of AKI.
 POSTRENAL CAUSE
 Back trauma resulting post-renal AKI secondary to

neurogenic bladder.
 A neonate with a history of hydronephrosis on prenatal
ultrasound and a palpable bladder most likely has
congenital urinary tract obstruction, probably related to
posterior urethral valves.
2. Complications
Complications are not common but on rare occasions the child may
develop
 uremia- chx by bleeding complication and mental status
change
 hypervolumia – weight gain
 hyponatremia –Rx associated complication resulting in
seizure and mental status change.
 hyperkalimia- weakness and palpitation(fatal arrhythmia)
 cardiac complications- arrthymia(palpitation),
pericarditis(chest pain radiating to the shoulder which is
aggravated by lying supine and relived by leaning forward).

3. Physical examination
 GENERAL APPEARANCE
oLethargic, ASL(respiratory distress)

 VITAL SIGNS-
 BP
 High (glomerulonephritis),
 Low (pre renal causes),
 Normal
 PR = tachycardia in pre-renal causes as a main defense
mechanism to increase the blood pressure (BP=CO*TPR
---- CO=HR*SV)
 RR = rise (in uremia kussmal breathing) or normal
 T = elevated (eg. AGE) or normal
 ANTROPOMETRY
oWait gain (fluid overload), Wait loss (fluid deficient)

 HEENT
 Sunken eyeball(recent onset) due to dehydration (pre renal

cause)
 periorbital edema
 Dry mucosa
 Icteric sclera
 LGS - LAP (probably metastatic tumor, tumor lysis syndrome)
 CHEST - finding consistent with cardiac failure(explained in
cardiac section)
 CVS –
 Arterial examination - PR,BP, absent palpable peripheral
artery(pre renal causes).
 venous examination - raised JVP(cardiac causes)
 precordial examination - finding which is consistent with
heart failure (which is discussed in cardiac case)
 ABDOMINAL EXAMINATION –

Inspection
 protuberant abdomen with bulged flank (ascites)
 Everted umbilicus
Auscultation - hyperactive bowel sound (AGE)
Palpation –
 may be tenderness (in AGE)
 mass if it is cause is tumor ( eg. Wilms tumor) or palpable
 Flank mass (renal vein thrombosis)
 Percussion - sign of fluid collection
 Genitourinary examination
o CVA tenderness(AGN)
o Palpable Bladder (Neurogenic Bladder)
 IS
o Skin pinch(dehydration)
o Rash
o Arthritis(SLE And HSP)- swelling, redness, hotness,
tender
o Petechiae
o Edema
o Check for tungiasis ( common In our setup)
4. Investigation
 CBC
 for Anemia due to dilutional (ass with management) or

hemolytic (commonly in HUS, but also in case of SLE ,
renal vein thrombosis)
 Leukopenia – sepsis or SLE
 Thrombocytopenia- commonly in HUS, but can occur in
case of SLE, sepsis, renal vein thrombosis)
 SERUM ELECTROLYTE - Hyponatremia (dilutional),
Hyperkalemia, hypocalcimia, hyperphosphatemia usually due
to failure of the tubule of nephrons to reabsorb or secrete
secondary to ATN.
 RFT - for diagnostic purpose of AKI (stated in the definition)

 C3 LEVEL- low in case of post-streptococcal

SERUM
glomerulonephritis due to immune complex deposition in the
glomerules and activation of complement cascade.
 SERUM ANTIBODY- post-streptococcal glomerulonephritis,
SLE(ANA), against glomerular basement membrane
(Goodpasture disease).
 U/A- hematuria (GN), Proteinuria (nephrotic or nephritic
syndrome), casts.
 Urinary indices - for differentiation of pre renal from renal

causes.
 In pre renal –
 elevated specific gravity,
 urinary osmolality >500mosm/kg,
 low Urine sodium which is ≤20meq/L
 Fractional Na excretion ≤1% (neonate 2.5%).
In Intrinsic Cause The Opposite Is True
Urinalysis, urine chemistries, and osmolality in acute kidney injury

Acute Acute
Hypovolemia tubular interstitial Glomerulonephritis Obstruction
necrosis nephritis
Broad, WBC,
brownish eosinophils,
Sediment Bland RBC, RBC casts Bland or bloody
granular cellular
casts casts
Minimal but
mat be
Protein None or low None or low Increased, >100 mg/dl Low
increased by
NSAIDs
Urine Na+ <20 (acute)
<20 >30 >30 <20
(meq/L) * >40 (few days)
Urine
osmolality >400 <350 <350 >400 <350
(mOsm/kg)
Fractional
<1 (acute)
excretion of <1 >1 Varies <1
>1 (few days)
Na+ (%) ¶
* the sensitivity and specificity of urine Na+ of <20 meq/L in differentiating prerenal azotemia from acute
tubular necrosis (ATN) are 90% and 82% respectively.
¶ fractional excretion of Na+ is the urine:plasma (U:P) ratio of Na+ divided by U:P of creatinine x100. The
sensitivity and specificity of fractional excretion of Na+ of <1% in differentiating prerenal azotemia from
ATN are 96% and 95%, respectively.

 CXR- cardiomegaly (cardiac failure)

 RENAL U/S- usually for post renal causes but also for intrinsic
causes
 RENAL BIOPSY – rarely done to determine precise cause of ARF in
patient who do not have clearly defined pre renal or post renal
ARF.
5. Management principle
Determined based on the cause
 But the general Mgt principle are
Maintenance of fluid and electrolyte
Avoidance and treatment of life threatening
complications such as ; Hyperkalaemia, Metabolic
acidosis, Hypocalcaemia, Hypernatremia, GI
bleeding (due to uremic platelet dysfunction)
Adequate nutritional support
 Treatment of the underlying causes
6. Prognosis
The mortality rate in children with AKI is variable and
depends entirely on the nature of the underlying disease
process rather than on the renal failure itself.
Eg. PSGN – MR is less than 1%
-AKI with MOF MR is greater than 90%
 The prognosis for recovery of renal function depends on the
disorder that precipitated AKI
 Recovery of renal function is likely after AKI
resulting from prerenal causes ATN, acute interstitial
nephritis, or
tumor lysis syndrome
 Recovery of renal function is unusual when AKI
results from most types of rapidly progressive
glomerulonephritis, bilateral renal vein thrombosis, or
bilateral cortical necrosis

Glomerular disease
ANATOMY
 Each kidney has approximately 1 million

nephrons.
 formation of nephron completed at birth
but functional maturation occurs within
first decade of life.
 glomeruli is a network of specialized
capillaries serving as the filtering
mechanism of kidney.
GLOMERULAR INJURY
 occurs under four mechanisms:

1. genetic -mutations in DNA exons encoding proteins located
within the glomerulus, interstitium, or tubular epithelium;
mutations in regulatory genes controlling DNA transcription;
abnormal posttranscriptional modification of RNA
transcripts; or abnormal posttranslational modification of
proteins.
2. immunologic-glomerular deposition of circulating
antigen–antibody immune complexes or interaction of
antibody with glomerular antigens in situ.
3. perfusion disorder
4. coagulation disorder
CLASSIFICATION
 Pathologically
 Generalized or focal (with respect to degree of the glomerules
(plural))
 Diffuse or Segmental (with respect to gromeruli (singular))
 Clinically
 Acute nephritic syndrome
 Nephrotic syndrome

 chronic GN (persistently abnormal urinary sediment with

insidiously progressive decline in GFR)
 Rapidly progressive glomerulonephritis
 Asymptomatic urinary sediment abnormality (persistence
hematuria, proteinuria, casts, pyuria)
Acute nephritic syndrome (frequently called acute GN)
 Refers to a set of renal disease in which an immunologic
mechanism triggers inflammation and proliferation of glomerular
tissue that can result in damage to the basement membrane,
mesangium, or capillary endothelium.
Acute Nephritic Syndrome is characterized by:
 hematuria
 oliguria
 fluid retention manifested by –edema and hypertension
 ARF, manifested by sudden decrease in the GFR
CAUSES (DDX) OF AGN
1. Infectious Disease
A. Post Streptococcal Glomerulonephritis (PSGN)
B. Non Streptococcal Post Infectious Glomerulonephritis
a. Bacterial
 infective endocarditis,
 Sepsis,
 Pneumococcal pneumonia,
 E.coli,
 Typhoid fever,
 Secondary syphilis,
 Meningococcemia
b. viral
 Hepatitis B,
 Infectious Mononucleosis,
 Mumps,
 Measles,
 Varicella,
 Echovirus And
 Coxsackie Virus.
c. Parasitic- Malaria And Toxoplasmosis.
2. Multisystemic Disease
 SLE,
 vasculitis,
 HSP,
 Good pasture’s syndrome
3. Primary Glomerular Disease
 Berger’s disease,
 pure mesengial proliferative GN
4. Miscellaneous
 GBS (Gillian Barre syndrome)
 DPT vaccine,
 serum sickness
ACUTE POST STREPTOCOCCAL

GLOMERULONEPHRITIS (APSGN)
 the most common cause of GN.
 Is one of the complication of Group A b-hemolytic streptococcal
infection.
 APSGN follows infection of the throat or skin by certain
“nephrogenic” strains of group A β-hemolytic streptococcus.
 Poststreptococcal GN commonly follows streptococcal pharyngitis
during cold-weather months and streptococcal skin infections or
pyoderma during warm-weather month.
 infection associated with throat is serotypes M1, M4, M25, and
some strains of M12 and skin is serotype M49.
 Molecular mimicry whereby circulating antibodies elicited by
streptococcal antigens react with normal glomerular antigens, in
situ immune complex formation of antistreptococcal antibodies
with glomerular deposited antigen, and complement activation by
directly deposited streptococcal antigens continue to be considered
as probable mechanisms of immunologic injury.

APPROACH TO A PATIENT WITH PSGN
1. History
 RISK FACTOR
 age 5-12
 rural area residence
 overcrowding (refuge)
 History of sore throat or skin lesion (tungiasis common in our
setup)
 recent family history of similar attack
 SYMPTOM
 1-2 wk after an antecedent streptococcal pharyngitis (sore

throat) (but most of the time it is not recalled by patient due to
it is either asymptomatic or mild) or
 3-6 wk after a streptococcal pyoderma (as for recent skin
lesion).
 Generalized body swelling which started from face (periorbital)
 Reddish discoloration of urine (tea color)
 decrease amount and frequency of urine.
 headache (2° to hypertension)
 nonspecific symptoms:
 malaise,
 lethargy,
 flank pain,
 fever.
 COMPLICATION
1. hypertensive encephalopathy occurs in10%pt with HTN

associated with GN
 altered mental status, blurred vision, severe headaches,
altered mental status, or new seizures,
2. CHF
 PND, SOB, orthopnea

3. pulmonary edema
 cough, SOB
4. nephrotic syndrome - develops in a minority (<5%) of
childhood cases.
2. PHYSICAL EXAMINATION
PERTINENT POSITIVE FINDINGS:
 General Appearance-
 ASL or well looking
 may have altered mental status (HTN encephalopathy)
 Vital Signs-
 BP- hypertensive range (60% pt)
 PR
 low (in response to high BP),
 high (heart failure)
 RR- tachypnea (CHF, pulmonary edema)
 Chest-
o Crepitation (pulmonary edema)
 CVS- precordium
o sign of heart failure discussed under CHF part
 Abdomen-
 Inspection-
 Protuberant abdomen with bulged flank
 Everted umbilicus if it is significant ascites
 Auscultation
 Palpation- CVT, palpable liver if CHF occurred
 Percussion- sign of fluid collection shifting dullness and
positive fluid thrill.
 IS-
o Search for skin lesion Tungiaisis is common in our setup.
3. INVESTIGATIONS
DIAGNOSTIC:

 U/A –
 Hematuria,
 RBC casts (pathognomonic for GN),
 proteinuria with nephritic range+1,2,
 WBC (PMN),
 SERUM C3 (MORE THAN 90%)LEVEL
 It is low which is important for diagnosis (due to

activation of complement cascade).
 SERUM C4 - is usually normal
 Confirmation of diagnosis requires evidence of
streptococcal infection.
 ASO titer - in pharyngitis case,
 antideoxyribonuclease B level - in pyoderma
 Renal biopsy - is indicated only in case of- acute renal failure,
nephrotic syndrome, absence of evidence of streptococcal
infection, or normal complement levels. In addition, renal
biopsy is considered when hematuria and proteinuria,
diminished renal function, and/or a low C3 level persist more
than 2 mo after onset.
BASELINE:
 CBC- mild normochromic anemia may be present from
hemodilution and low-grade hemolysis.
4. MANAGEMENT
 Management is directed at treating the acute effects of renal

insufficiency and hypertension.
 10 day course of systemic antibiotic therapy with penicillin is
recommended to limit
the spread of the nephritogenic organisms and recurrence.
(but doesn’t affect the natural history of APSGN.
 Sodium restriction, diuresis (usually with intravenous
furosemide), and pharmacotherapy with calcium channel
antagonists, vasodilators, or angiotensin-converting enzyme
inhibitors, are standard therapies used to treat

hypertension.
PROGNOSIS
 Complete recovery occurs in >95% of children with APSGN.

 Recurrences are extremely rare.
 Mortality in the acute stage can be avoided by appropriate
management of acute renal failure, cardiac failure, and
hypertension
 Infrequently, the acute phase is severe and leads to
glomerulosclerosis and chronic renal disease in <2% of affected
children
 Generally acute phase resolves within 6-8wks.
 Proteinuria and HTN usually resolves by 4-6wks.
 Microscopic hematuia may last until 2yrs.
HSP (Henoch-Schönlein purpura)

 Is the most common vasculitis of childhood
 Is characterized by leukocytoclastic vasculitis and
immunoglobulin (Ig) A deposition in the small vessels in the
skin, joints, gastrointestinal tract, and kidney.
 Usually occurs following upper respiratory tract infection
(infectious triggers are group A β-hemolytic streptococcus,
Staphylococcus aureus, mycoplasma, and adenovirus).
 is a disease mediated IgA and IgA complex deposition in the
skin, GI, kidney, joints.
APPROACH TO PATIENT WITH HSP
1. History
 RISK FACTORS
 Common in white and Asian populations

 affects males more than females, with M:F= 1.2-1.8 : 1.

 Ages of 3 and 10 yr.

 Recent upper respiratory infection (Cough, sore throat,
fever...).
 SYMPTOMS
 The hallmark symptom is rash

 Lesions are usually symmetric and occur in
gravity-dependent
areas (lower extremities) or on pressure points
(buttocks).
 The skin lesions often evolve in groups, typically lasting
3-10 days,
 May recur up to 4 mo after initial presentation.
 Arthritis
 The arthritis tends to be self-limited and oligo articular
 Predilection for the lower extremities
 Does not lead to deformities
 Usually resolves within 2 wk but can recur.
 Presents with:
o Joint swelling,
o Limitation of range of movement
o Arthralgia (pain in the joint), is common, occurring
in up to 75% of children with HSP.
 Abdominal pain, Vomiting, Diarrhea, Paralytic ileus and
Melena (dark stool) occurs in the 85% of patient.
 Intracerebral hemorrhage, seizures, headaches, and behavior
changes (Neurologic manifestations of HSP, caused by
hypertension or central nervous system (CNS) vasculitis)
 RENAL MANIFESTATION occurs in 50% of patients, occurs weeks
to month after the rash, these are:
 Generalized body swelling which started from
face(periorbital)
 Reddish discoloration of urine (tea color)
 decrease amount and frequency of urine.
 headache (hypertension)

 nonspecific symptoms –malsise, lethargy, flank pain,

fever (Frequently renal manifestation are mild.)
 COMPLICATIONS (RARE)
 HYPERTENSIVE ENCEPHALOPATHY (altered mental status,

blurred vision, severe headaches, altered mental
status, or new seizures) 10%pt with HTN,
 CHF (PND, SOB, orthopnea), pulmonary
edema(cough, SOB),
2. PHYSICAL EXAMINATIONS
 ASL Or well looking
 may have altered mental statu
 Vital Signs-
 BP- hypertensive range
 PR-low(in response to high BP),high( heart failure)
 RR- tachypenic( CHF ,pulmonary edema)
 Chest- creptation (pulmonary edema)
 CVS- precordium –sign of heart failure discussed under CHF part
 Abdomen-
 inspection-
 protuberant abdomen with bulged flank
 everted umbilicus if it is significant ascitis
 auscultation – absent bowel sound(paralytic ileus)
 palpation- CVT, palpable liver if CHF occurred (tender
hepatomegaly)
 percussion- sign of fluid collection shifting dullness and
positive fluid thrill.
 MSS-
 Inspection-joint swelling, and erythemat(uncommon).
 Palpation- tenderness , hotness(uncommon), and limited
range of movement.

 IS- palpable purpura starting as

pink macules or wheals and
developing into petechiae, raised
purpura, or larger ecchymoses.
Occasionally, bullae and
ulcerations develop.
 CNS –
 GSC- may be less than 15
 loss of orientation to person,
place and time
3. INVESTIGATION
 No laboratory finding is diagnostic of HSP

 The diagnosis of HSP is a clinical one and is often
straightforward when the typical rash is present.
 CBC- mild anemia, Thrombocytosis, leukocytosis-
 ESR- rise
 STOOL TEST(OBT)- Blood in the stool
 U/S- often used in the setting of gastrointestinal complaints
to look for bowel wall edema or the rare occurrence of an
associated intussusception.
 Barium enema can also be used to both diagnose and treat
intussusceptions (Complication of HSP).
 U/A- hematuria, proteinuria
 Most patients who develop nephritis have urinary
abnormalities by 1 mo, and nearly all have
abnormalities by 3 mo after onset of HSP.
 Therefore, a urinalysis should be performed weekly in
patients with HSP during the period of active clinical
disease.
 Thereafter, a urinalysis should be performed once a month
for up to 6 mo.
 If all urinalyses are normal during this follow-up interval,
nephritis is unlikely to develop.

 If proteinuria, renal insufficiency, or hypertension

develops along with hematuria, consultation with a
pediatric nephrologist is indicated.
 RFT- if AKI develops.
4. PROGNOSIS AND MANAGEMENT
 The prognosis of HSP nephritis for most patients is

excellent.
 Untreated, the risk of developing chronic kidney disease,
including renal failure, is 2-5% in all patients with HSP,
but almost 50% in those with the most severe early renal
clinical and histologic features.
 MANAGEMENT IS SUPPORTIVE.
HUS (Hemolytic-uremic syndrome)

 characterized by the triad of
1. microangiopathic hemolytic anemia
2. thrombocytopenia, and
3. renal insufficiency.
 Causes can be:
 Infection-Induced (diarrheal type),
 Genetic (non-diarrheal),
 Medication- Induced, and
 HUS associated with systemic diseases characterized by
microvascular injury.
 Infection-Induced
The most common form of HUS is caused by toxin-producing
Escherichia coli(O157:H7 is most common serotype).
In the subcontinent of Asia and in southern Africa, the toxin of
Shigella dysenteriae type 1 is causative, whereas in Western
countries, verotoxin or Shiga-like toxin producing E. coli
(STEC) is the usual cause.

A rare but distinct entity of infection-triggered HUS is related

to neuraminidase-producing Streptococcus pneumonia.
 Genetic forms of HUS (atypical, nondiarrheal)
Inherited deficiencies of either von Willebrand
factor–cleaving protease (ADAMTS13)
complement factor H, I, or B, and
defects in vitamin B12 metabolism can cause HUS.
A major feature characteristic of genetic forms of HUS is the
absence of a preceding diarrhea prodrome.
APPROACH TO A PATIENT WITH HUS
HISTORY
RISK FACTORS
 Eating undercooked meat or unpasteurized (raw) milk and apple

cider.
 drinking contaminated municipal water supplies; petting farms;
and swimming in contaminated ponds, lakes, or pools.
 preschool and school-age children.
 recent History of fever, vomiting, abdominal pain, and diarrhea
 recent History of cough, fever, chest pain
SYMPTOMS
 Following the prodromal illness, the sudden onset of pallor,

irritability, weakness, and lethargy heralds the onset of HUS.
 Oliguria can be present in early stages but may be masked by
ongoing diarrhea.
 Hematuria, fluid retention (manifested by edema and
hypertension), ARF (manifested by sudden decrease in the GFR).
 But if the GI symptom predominates, fluid retention will not occur
rather dehydration will occur (manifested by recent onset of
sunken eye ball, mental status change).
 irritability, lethargy, or nonspecific encephalopathic feature

 Severe CNS involvement occurs in ≤20% of cases(Seizures and

significant encephalopathy are the most common manifestations
in those with severe CNS involvement, resulting from focal
ischemia secondary to microvascular CNS thrombosis)
 Recent History of fever, vomiting, abdominal pain, and diarrhea
 Recent History of cough, fever ,chest pain
PHYSICAL EXAMINATION
 General appearance - ASL

 Vital Signs
 BP
 High (fluid retention),
 Low (if diarrhea predominates)
 PR
 low if HTN occurs
 high if dehydration occurs
 T= elevated (eg. AGE) or normal (in noninfectious causes)
 HEENT
 sunken eyeball(recent onset) due to dehydration (if diarrhea
predominates )
 dry mucosa
 CVS-
 Arterial examination-PR,BP, absent palpable peripheral
artery(pre renal causes).
 ]Abdominal examination-
 inspection
 protuberant abdomen with bulged flank (ascites)
 Everted umbilicus
 Auscultation- hyperactive bowel sound (AGE)
 palpation- may be tenderness in AGE
 percussion-sign of fluid collection
 Genitourinary examination- CVA tenderness
INVESTIGATION

 CBC- anemia (deu to hemolysis), Thrombocytopenia(consumption

of platlets), Leukocytosis (infection)
 U/A- microscopic hematuria and low-grade proteinuria( nephritic
range).
 RFT- Scr and BUN (due to AKI)
 The stool culture is often negative in patients who have
diarrhea-associated HUS(due to the causative agents are rapidly
cleared before HUS develops).
 If no history of diarrheal prodrome or pneumococcal infection is
obtained, then evaluation for genetic forms of HUS should be
considered.
PROGNOSIS
 With early recognition and intensive supportive care, the mortality

for diarrhea-associated HUS is <5% in most major medical
centers.
 Most recover renal function completely, but of surviving patients,
5% remain dependent on dialysis, and up to 30% are left with
some degree of chronic renal insufficiency.
 The prognosis for HUS not associated with diarrhea is more
severe.
MANAGEMENT PRINCIPLE
 early recognition of the disease, monitoring for potential
complications, and meticulous supportive care.
 Supportive care includes careful management of fluid and
electrolytes, including prompt correction of volume deficit, control
of hypertension, and early institution of dialysis if the patient
becomes significantly oliguric or anuric, particularly with
hyperkalemia.
 Early intravenous volume expansion before the onset of oligo
anuria may be nephroprotective in
diarrhea-associated HUS
 Red cell transfusions are usually required as hemolysis can be
brisk and recurrent until the active phase of the
disease has resolved.

 In pneumococci-associated HUS, it is critical that any

administered red cells be washed before transfusion to remove
residual plasma, because endogenous IgM directed against the
revealed T antigen can play a role in accelerating the pathogenesis
of the disease.
 Platelets should generally not be administered, regardless of
platelet count, to patients with HUS because they are rapidly
consumed by the active coagulation and theoretically can worsen
the clinical course.
 Antibiotic therapy to clear enteric toxigenic organisms (STEC) can
result in increased toxin release, potentially exacerbating the
disease, and therefore is not recommended. However, prompt
treatment of causative pneumococcal infection is important.
Nephrotic syndrome
 Nephrotic syndrome is the clinical manifestation of glomerular
diseases associated with heavy (nephrotic-range) proteinuria
 Nephrotic range proteinuria is defined as proteinuria >3.5 g/24 hr
or a urine protein: creatinine ratio >2.
Triad of clinical findings
 Hypoalbuminemia (≤2.5 g/dL) (secondary to increase

protein loss 2° to increase glomerular permeability)
 Edema (2°to hypoalbuminemia), and
 Hyperlipidemia (2°to the result of increased synthesis as
well as decreased catabolism of lipid (cholesterol >200
mg/dL)
 Nephrotic syndrome affects 1-3 per 100,000 children <16 yr of
age
ETIOLOGY
 Primary Or Idiopathic (Commonest One)90%

 Minimal Change Disease (The Most Common),
 Focal Segmental Glomerulosclerosis,
 Membranoproliferative Glomerulonephritis,
 C3 Glomerulopathy, and

 Membranous Nephropathy
 Secondary To Systemic Disease
 Systemic Lupus Erythematosus,
 Henoch-Schönlein Purpura,
 Malignancy (Lymphoma and Leukemia),
 Infections (Hepatitis, Hiv, and Malaria).
 Drugs (Isoniazaid)
 Congenital Nephritic
 Defect on Podocine
CLINICAL CONSEQUENCE OF NEPHROTIC SYNDROME
 Edema
 Hyperlipidemia (increase in cholesterol, triglycerides,
low-density lipoprotein, and very-low-density lipoproteins the
implication is not much serious as children)
 Increased Susceptibility to Infections 2°to:
 Urinary loss of Ig G
 Defects in the complementcascade from urinary loss of
complement factors (predominantly C3 and C5)
 Alternative pathway factors B and D, lead to impaired
opsonization of microorganisms)
o Commonly cellulitis, spontaneous bacterial
peritonitis, and bacteremia
o Susceptible for encapsulated bacteria.
 Hypercoagulability state 2°to:
 Vascular stasis from Hemoconcentration and
intravascular volume depletion,
 Increased platelet number and aggregability, and changes
in coagulation factor levels.
 Increase in hepatic production of fibrinogen along with
urinary losses of antithrombotic factors such as
antithrombin III and protein S.
 Deep venous thrombosis may occur in any venous bed,
including the cerebral venous sinus,renal vein, and
pulmonary veins

 The clinical risk compared to adults in pediatrics age

group is low (2-3%) but has potential serious consequence.
Idiopathic Nephrotic Syndrome

 Approximately 90% of children with nephrotic syndrome have
idiopathic nephrotic syndrome.
 Idiopathic nephrotic syndrome is associated with primary
glomerular disease without an identifiable
causative disease or drug.
 approximately 85% of total cases of nephrotic syndrome in
children is Minimal Change Nephrotic Syndrome (MCNS)
 More than 95% of children with minimal change disease
respond to corticosteroid therapy.
APPROACH TO A PATIENT WITH MCNS
1. History
 RISK FACTORS
 Male patient (M:F = 2:1)

 Age between 2 -6
 SYMPTOMS
 Edema
 First noticed on the face then progressed to whole body
 Non specific symptoms Anorexia, irritability, abdominal
pain, and diarrhea are common.
 Hallmark is absence of hematuria and hypertension.
 COMPLICATIONS
 Bacterial infections (most common cause of death)

 Spontaneous Bacterial Peritonitis (SBP)-
presentation fever, abdominal pain, anorexia in
ascetic patient.

 Cellulitis- fever, swelling, reddish discoloration of the

skin over the swelling, limping (if it occurs in the lower
extremity.)
 DVT if it occurs in the:
 Lower extremity –intermittent claudication (pain
upon walking and relieved by taking rest)
 Cerebral sinus –body weakness, altered mental
status, or other focal neurologic sign.
 Massive Pleural Effusion –presentation SOB

 Hyperlipidemia –not significant as adults (increase the risk
of IHD, STROKE...)
2. PHYSICAL EXAMINATIONS
 Can range from
Well looking to ASL
 may have
altered mental status
 Vital Signs-
 BP- normal or low(expansion of fluid to the third space)
 PR-normal or high(2°to hypotension)
 RR- tachypenic( massive pleural effusion,pulmonary
edema)
 T- fever(if infection occurs(complications)), normal
 Chest- when there is complications like massive pleural effusion
 Inspection-
 chest lag,
 central cyanosis(rare),
 nasal flaring ,
 use of accessory muscles
 Palpation-
 decrease tactile fremitus
 decrease chest expansion

 Percussion- dullness in the side of effusion

 Auscultation-
 decrease or absent air entry
 crackle if pulmonary edema occurs
 Abdomen-
 Inspection-
 protuberant abdomen with bulged flank
 everted umbilicus if it is significant ascites
 Palpation- CVT
 percussion- sign of fluid collection shifting dullness
and positive fluid thrill.
3. INVESTIGATION
Diagnostic
 U/A
 3+ or 4+ proteinuria
 protein : creatinine ratio should be >2.0
 Serum albumin level is <2.5 g/dL
 Serum Cholesterol and Triglyceride levels are elevated
 Serum Complement levels are normal
 Evaluation to rule out secondary forms of nephrotic syndrome
(children ≥10yr)
 Antinuclear Antibody, double-stranded DNA
 Hepatitides B and C,
 HIV in high-risk populations; and
 Kidney Biopsy (for children ≥12 yr, who are less likely to have
MCNS
4. TREATMENT
 Corticosteroids are the mainstay of therapy for MCNS.

 Approximately 80-90% of children respond to steroid
therapy.
 Response is defined as the attainment of remission within
the initial 4 wk of corticosteroid therapy.

 Remission consists of a urine protein : creatinine ratio of

<0.2 or <1+ protein on urine dipstick for 3 consecutive
days.
 Managing the clinical sequel
 Edema-sodium restriction (<1500 mg daily),
 water/fluid restriction may be necessary if the child
is hyponatremic.
 A swollen scrotum may be elevated with pillows to
enhance fluid removal by gravity.
 Diuresis may be augmented by the administration of loop
diuretics (furosemide), orally or intravenously, although
extreme caution should be exercised. Aggressive diuresis
can lead to intravascular volume depletion and an increased
risk for acute renal failure and intravascular thrombosis.
 Dyslipidemia-managed with a low-fat diet.
 Dietary fat intake should be limited to <30% of calories

with saturated fat intake <10% calories.
 Dietary cholesterol intake should be <300 mg/day
 Infections- Families of children with nephrotic syndrome

should be counseled regarding the signs and symptoms of
infections.
The antibiotic provided must be of broad enough
coverage to include Pneumococcus and Gram-negative
bacteria.
A 3rd-generation cephalosporin is a common choice of IV
antibiotic
PROGNOSIS
 Most children with steroid-responsive nephrotic syndrome

have repeated relapses, which generally decrease in frequency
as the child grows older.
 Children who respond rapidly to steroids and those who have
no relapses during the first 6 mo after diagnosis are likely to
follow an infrequently relapsing course.
 Children with steroid-resistant nephrotic syndrome, most often

caused by FSGS, generally have a much poorer prognosis.
 These children develop progressive renal insufficiency,
ultimately leading to end stage renal disease requiring dialysis
or kidney transplantation.

Sample history for Nephrotic syndrome

Date=19/1/2012
c/c: generalized body swelling of 1month duration

HPI
This is a 13 ys old male patient presented with generalized body
swelling of month duration. The swelling was gradual and started
first around the eyes then progressed downward to involve the whole
body within month. The swelling worsened in the morning up on
walking up from bed and relatively decreases during the night. In
association to this he also has loss of appetite and easy fatigability of
1-month duration. over the past two weeks he has dry intermittent
cough with no known aggravating and reliving factors, frontal area
throbbing type headache.
Otherwise;
 he has no History of sore throat, skin lesion, decrease
urinary output (AGN)
 no History of pain during urination, color change in the
urine, flank pain (other renal causes)
 He has no History of SOB,
 He has no History of PND, palpitation (cardiac)
 He has no History of yellowish discoloration of eye, RUQP
(liver causes)
 No history of easy pluckability of hair (malnutrition)
 No History of similar illness in the family
 No History of malarial attack, malar rash or
photosensitivity (2°causes of NS)
 No History of vertigo, tingling sensation (anemia)
 Parents are sero negative for RVI

Before his illness the patient had been eating 3* in a day. His staple
diet is injera made of teff and rice and shiro wott. He eats avocado
and egg 4 times in a week. He didn’t get milk. He shares his food with
his younger siblings. He uses stream water for drinking purpose
without boiling or applying chemical.
Currently, he completed grade 7 and promoted to grade 8. He is one
of the top scorer student.

CHAPTER 6
APPROACH TO SHORTNESS OF
BREATH (DYSPNEA)
Definition
 is a symptom not a disease by it’s self
 is not complained by young patients, so they experience it through
signs like labored breathing and fast breathing.
 patients complain it as:
 chest tightness
 increased effort of breathing
 heavy breathing and
 air hunger
 These are called qualitative descriptors of dyspnea. (Nelson

pediatric symptom based diagnosis).
Mechanism Of Dyspnea:
 Respiratory sensations are the consequence of interactions
between the efferent, or outgoing, motor output from the brain to
the ventilatory muscles (feed-forward) and the afferent, or
incoming, sensory input from receptors throughout the body
(feedback) as well as the integrative processing of this information
that we infer must be occurring in the brain.

Differential Diagnosis
 Depends on
 Onset
 Duration
 quality of the symptom and
 age of the patient.
 These includes: PULMONARY CAUSES AND NON-PULMONARY
CAUSES
PULMONARY CAUSES
1. Tuberculosis
2. Pneumonia
3. Asthma
4. Pertussis
5. Foreign body
6. Pleural effusion or Empyema
7. Pneumothorax
8. Croup
9. Bronchiolitis

NON-PULMONARY CAUSES
1. CARDIAC FAILURE SECONDARY TO ANY UNDERLYING

CAUSES:
 The commons in pediatrics are:
 Congenital heart disease
 Rheumatic valvular heart disease and
 Severe anemia
 Other causes are Myocarditis, Pericardial Disease,
Severe Hypertension, Cardiomyopathies and Renal
Failure.
2. SEVERE ANEMIA… discussed in other chapter again.
3. NEUROLOGIC CAUSES: -
 Primary neurologic disorders, like increased intracranial
pressure, neuromyopathic weakness, may present in
respiratory distress (Dyspnea).
 Common symptoms include irregular respirations,
hypoventilation, or hyperventilation.
 Common causes are
 Meningitis,
 Cerebritis or encephalitis
 Intracranial hemorrhage
 Mass lesion, or toxic ingestion.
4. OTHERS: -
 Metabolic derangement that results in acidosis can produce
tachypnea and possibly dyspnea.
 Common causes of acidosis include
 Diabetic ketoacidosis
 sepsis and
 Ingestions (such as aspirin).

HEART FAILURE
 Occurs when the heart cannot deliver adequate cardiac output to
meet the metabolic needs of the body.
 In the early stages of heart failure, various compensatory
mechanisms are evoked to maintain normal metabolic function.
When these mechanisms become ineffective, increasingly severe
clinical manifestations result.
 The characteristic signs and symptoms are:
 Poor growth
 Feeding difficulties,
 Respiratory distress
 Exercise intolerance and
 Fatigue.
ETIOLOGIES:
 Are age dependent and they are a consequence of cardiac and

non-cardiac disorders, either congenital or acquired (as
mentioned above).
CLINICAL MANIFESTATIONS
 Depend in part on the degree of the child's cardiac reserve.

Therefore:
 In critically ill patients having exhausted compensatory

mechanisms to the extent of insufficiency to meet basal
metabolic need of the body, >>>> they may present with
cardiogenic shock.
 Some patients are comfortable at rest (but they can’t tolerate

even, mild activities) and
 Other patients need vigorous activity to have compromised

cardiac function (be symptomatic).

APPROACH TO THE PATIENT

1. HISTORY
Differs between older children and those of younger children &

infants.
IN OLDER CHILDREN:
The usual chief complaint is Shortness of breath, and associated
symptoms are: -
 Orthopnea,
 Paroxysmal nocturnal dyspnea.
 Cough
 Body swelling (edema) which may not be noticed in young
children and infants, rather it’s considered as normal
weight gain.
 Fatigue
 Effort or exercise intolerance >> may be considered as lack
of interest in activity wrongly.
 loss of appetite (anorexia)
 Focal neurologic deficit (body weakness) as complication
of heart failure, called cardio-emboli stroke.
 Some patients may have histories giving some clue for the
underlying lesion.
For example: History of Recent Sore Throat, Migratory Joint Pain,
Abnormal Body Movement and Recent Chest Pain (carditis) -->
Rheumatic Heart Disease
 NB: some patients may surprisingly lack respiratory complaints

and primarily have abdominal symptoms (abdominal pain,
nausea and anorexia). So, attention to cardiovascular system come
after the abdominal radiograph unexpectedly catches the lower
end of the enlarged heart.

IN INFANTS:
 Distinguishing heart failure is difficult from other causes of

respiratory distress in young infants.
 Has feeding problems usually, can also have others.
 Less volume of breast feeding (expressed in terms of
duration of feeding) and feeding difficulty.
 Fast breathing and Profuse Sweeting (perspiration) while
feeding.
 Poor wait gain
 Irritability
 Weak cry and
 Noisy and labored respiration.
2. PHYSICAL EXAMINATION
 GENERAL APPEARANCE
 Acute sick looking (b/c of cardiorespiratory distress),

 Looks depressed
 Acute on chronic sick looking (distress with emaciated body,
seen by zygomatic prominence). The Emaciated body is b/c of
two main reasons:
 cardiac cathexia and

 loss of appetite resulting bowel wall edema.
 Vital signs: (Please refer to the main topic for the reference
ranges)
 Tachypnea is the main finding of heart failure in pediatrics.
(Don’t forget to see the age of the patients while deciding the
tachypnea).
 Tachycardia

 HEENT:
 Paleness on the dorsum of the tongue and loss of tongue
papillae w/c are the manifestations of severe anemia (as a
cause of heart failure).
 Central cyanosis
 Respiratory system:
 Inspection
 Nasal flaring
 Intercostal and subcostal retraction
 Percussion -Bilateral dullness (there may be pleural
effusion)>> more common in adults.
 Auscultation:
 Bilateral roles (more in older children) and
 Wheezing than bilateral rales (in young infants)>>>
common finding of BRONCHIOLITIS.
 Cardiovascular system:
 Venous examination- Raised JVP>> difficult to assess in
young children and infants b/c of short neck and difficultly of
observing relaxing state.
 Arterial Examination- weak and rapid pulse, collapsing,
sometimes blowing, irregularity (findings depends on types of
lesions)
 Precordial Examination
 Inspection:
 Active precordium (more than one impulses in
intercostal space)
 Shifted apical impulse (if visible) >> indicates
cardiomegaly
 Bulged precordium (if present)>> indicates chronicity
 Palpation:
 Palpable p2 >> shows the pulmonary hypertension(2ry to
the failed Lt side heart)

 Palpable A2 >> shows the systemic Hypertension(can be

considered as cause of Heart failure)
 Heave >> (two types, Apical heave--LV hypertrophy &
Parasternal heave --RV hypertrophy)
 Thrill >> is palpable murmur(used for grading murmur)
 Shifted Point of maximal impulse(PMI) >> Cardiomegaly
 NB: Don’t do percussion for cardiovascular system [b/c ,its

significance is very small. It’s done in case we lose apical impulse
and heart sound from the left side (in dextrocardia) and search for
the dullness of heart on both sides]
 Auscultation:
 Gallop rhythm (s3)
 Murmurs (it’s not must to find murmur while having
failed heart) and if you find murmur, don’t forget to
characterize, and if its grade 4(murmur with thrill),
check grade 5(heard with half of the diaphragm) and
6(heard without touching the auscultatory area).
 Abdominal examination:
 Ascites (protruded abdomen, shifting dullness and fluid thrill)
 Tender hepatomegaly >>crucial finding for HF.
 Musculoskeletal: Bilateral pitting edema (try to grade it).
 Integumentary system:
 Yellowish discoloration of skin (if the liver is congested and
become ischemic 2ry to the failed heart, this is called
congestive hepatophathy & ischemic hepatopathy
respectively.)
 Paleness on the palm >> for the anemia (try to assess the
degree of paleness. I.e.: some paleness > without palmar crease
and severe paleness > paleness involving palmar crease).
 Nervous system:

 Decreased power of the muscle >> if there is a developed

stroke (called cardio-emboli stroke)
Clinical triads of Heart failure in

pediatrics:
1.Tachypnea
2.Cardiomegaly
3.Tender hepatomegaly
3. DIAGNOSIS (INVESTIGATIONS)
 Are ordered as its cost effectivity and availability.

 While calling, start from the basics and go to the diagnostic
investigation modalities.
BASIC INVESTIGATIONS:
 Complete blood count(CBC) with differential >> [To r/o 1. anemia

as a cause of HF, 2.infections >> one of the precipitating factor of
HF]
 ESR- to see inflammation (IE)
 CRP- to see inflammation (IE)
 RFT and LFT (when required)

 RFT >>To r/o the complication of HF on the kidney and also

for drug usage without doubt.
 LFT>> to see status of liver(it may be affected by congestion
and ischemia) which is called congestive hepatopathy and
Ischemic hepatopathy, w/c are expected more in adults.
DIAGNOSTIC INVESTIGATIONS:
 Chest X-ray>> to see cardiomegaly, some radiographic features of

CHD( e.g. Boots shape in tetralogy of fallot) and vascular marking
of lung especially in left side heart failure.
 ECG>> to see chamber hypertrophy and very importantly to r/o
arrhythmia>>is the feared complication of valvular heart disease.
 Echocardiography (GOLD STANDARD)>> to assess:
 Ventricular function via ejection fraction (normal range is
55-65%),
 intra mural thrombus and resulting in cardio-emboli
stroke--- is common in mitral stenosis,
 Any valvular lesion,
 Pericardial effusion
 Ventricular contractility
 Pulmonary artery pressure(PAP)
 Ventricular dilation
4. MANAGEMENT PRINCIPLES
 Stabilize the patient

 O2 supplementation (when saturation is below 90%)
 Bed rest with elevation
 Diet-salt restriction
 Treating the congestive state-loop diuretics, if used for long
time combine with potassium sparing diuretics or other
diuretic agent

 Treating the precipitating factors(remember HEART FAILS)

 Treating the underlying cause- including surgical intervention
 Make sure that you have addressed the following points on CHF:
 common causes of CHF in pediatrics
 precipitating factors
 complications
 Common investigation modalities with their findings
 management principles

THE COMMON CAUSES OF HEART FAILURES

CONGENITAL HEART DISEASES
ACQUIRED HEART DISEASES
 RHEUMATIC HEART DISEASE

 INFECTIVE ENDOCARDITIS- is also acquired Heart Disease,
but need an established underlying lesion, can also rarely
happen without any lesion>> see below)
1. RHEUMATIC HEART DISEASE

 Before discussing RHD, it’s better to see the preceding events
developing this complication.
 This is the sequence how RHD is developed:
GAS pharyngitis ARF RHD
A. Group A Streptococcal (GAS) pharyngitis

 is the common upper respiratory tract infection
 Diagnosis is mainly clinical.
PATIENT APPROACH:
HISTORY
 Symptoms: acute onset of Fever, vomiting, difficulty of
swallowing
 Complications (expected 10-21 days after the symptoms):
joint pain, chest pain, abnormal body movement (ARF),
decreased urine output, urine color change
(glomerulonephritis).

 Non suppurative (ARF & acute post streptococcal

glomerulonephritis).
The serotypes M1,3,5,6,18, 29 causes ARF and
 M4, 12, 25 – APSGN (discussed in other chapter).
 Suppuratives (skin lesions)-Serotypte M49.
PHYSICAL EXAMINATION:
Hyperemic enlarged tonsils with

follicle or exudates
Tender enlarged anterior cervical
lymph nodes
TREATMENT
 Should be within 9 days (Golden time) after start of the

symptoms.
Benzathine penicillin 600,000IU IM stat for children <27

kgs of weight; 1.2 million IU for child > 27 kg OR
Amoxicillin 50 mg/Kg/day po in three divided doses for 10
days
For patients allergic to penicillin, Erythromycin 40
mg/kg/day in four divided doses for 10 days and
FOLLOW UP in two days (if there is no improvement).

B. Acute Rheumatic fever(ARF)

 Is inflammatory disease involving heart, skin, joint and brain.
 Develops 10-21 days after GAS pharyngitis.
 Age 5-15 yrs is the greatest risky age group.
 2/3 patients have history of upper respiratory tract infection.
 Most patients have serologic evidence of recent GAS infection.
 If not treated, cause permanent lesion of cardiac valves (called
RHD).
 Patients developing ARF from GAS pharyngitis have special
susceptibility by genetic back ground, socioeconomic status, and
special strain of the bacteria resulted pharyngitis.
 Overcrowding of a family or community remains the main risk
factor for the spread of GAS infection.
Pathogenesis:
 Cytotoxicity theory-states the enzyme streptolysin O is directly

toxic to cardiac cells, but, its unable to explain the substantial
period (10-21 days) to develop ARF from GAS pharyngitis.
 Immune-mediated pathogenesis- molecular mimicry resulting
immunologic cross-reactivity between cardiac antigenic epitopes
and GAS cellular and extra cellular epitopes
PATIENT APPROACH:
 Because no clinical or laboratory finding is pathognomic for ARF,

we use Jones criteria (proposed by T. DUCKET JONES in 1944
and revised by AHA in 2015) for diagnosis and limit over diagnosis
of ARF.
 The table below gives us the detail information, and under the
table we use the moderate/high risk criteria for our Ethiopian
setup (all developing countries).

Guidelines for the Diagnosis of Initial or Recurrent Attack of Rheumatic Fever (Jones
Criteria, Updated 2015)
MAJOR MINOR MANIFESTATIONS SUPPORTING EVIDENCE OF
MANIFESTATIONS ANTECEDENT GROUP A
STREPTOCOCCAL INFECTION
Carditis Clinical features: Positive throat culture or rapid
Polyarthritis o Arthralgia streptococcal antigen test
Erythema marginatum o Fever Elevated or increasing
Subcutaneous nodules Laboratory features: streptococcal antibody titer
Chorea  Elevated acute phase
reactants:
- Erythrocyte
sedimentation rate
- C-reactive protein
 Prolonged P-R interval
I. Initial attack: 2 major manifestations, or 1 major and 2 minor

manifestations, plus evidence of recent GAS infection. Recurrent attack: 2
major, or 1 major and 2 minor, or 3 minor manifestations (the latter only in the
Moderate/High-Risk population), plus evidence of recent GAS infection.
II. Low-Risk population is defined as acute rheumatic fever (ARF) incidence <2
per 100,000 school-age children per year, or all-age rheumatic heart disease
(RHD) prevalence of <1 per 1,000 populations. Moderate/High-Risk population
is defined as ARF incidence >2 per 100,000 school-age children per year, or
all-age RHD prevalence of >1 per 1,000 populations.
III. Carditis is now defined as clinical and/or subclinical (echocardiographic
valvulitis).
IV. Arthritis (major) refers only to polyarthritis in Low-Risk populations, but also
to monoarthritis or polyarthralgia in Moderate/High-Risk populations.
V. Minor criteria for Moderate/High-Risk populations only include
monoarthralgia (polyarthralgia for Low-Risk populations), fever of >38°C
(>38.5°C in Low-Risk populations), ESR >30 mm/hr (>60 mm/hr in Low-Risk
populations).

NB: We dont adhere to Jones criteria in the following three circumstances:

1.When Chorea is the only major manifestation of ARF.
2.when Indolent carditis is the only manifestation of ARF
3.In Recurrent ARF attack.
THE BRIEF DISCUSSIONS OF THE MAJOR CRITERIA:

I. MIGRATORY POLYARTHRITIS
 seen approximately in 75% patients with ARF and is the earliest

manifestation of ARF
HISTORY:
 painful, migratory joint pain exquisitely tender even with
bedclothes.
 Involves large joints (knees, ankles, wrist and elbows).
 hot, swollen, red and tender joints
 Mono articular involvement is unusual unless anti-inflammatory
drug is given (NB: so if a child with fever and arthritis is suspected
to have ARF, it’s useful to withhold salicylate and observe for
migratory progression).
 Is highly responsive to even low dose of salicylate and if not think,
of alternative diagnosis.
 Become normal in 1-3 days without treatment or it can persist for
several weeks.
II. CARDITIS
 In 50-60% of patients.
 Most serious manifestation of ARF with RHD causing mortality
and morbidity
 Recurrent attack increase severity of cardiac disease (the
permanent valvular lesion)

HISTORY:
fever, chest pain and shortness of breath (if the heart is failed)
Physical Examination:
fever on vital sign,
tachypnea (may not present),
tachycardia,
cardiac murmur (with/without evidence of myocarditis or/and
pericarditis when seen on Echocardiography),
cardiomegaly,
tender hepatomegaly (the two show HF) and edema.
 MR-high pitch apical holo-systolic murmur radiates to axilla
 AR-high pitch decrescendo diastolic murmur at LSB.
 Echocardiography can show decreased ventricular contraction,
mitral/aortic regurgitation.
III. CHOREA
 in 10-15% of patients
 Presents as isolated, frequently subtle, movement disorder.
 Has longer latent period than carditis and arthritis (can be
months)
HISTORY:
 Emotional liability,
 poor school performance,
 uncontrollable movements and
 facial grimacing (all exacerbated by stress and disappear with
sleep)
PHYSICAL EXAMINATION: incoordination

IV. ERYTHEMA MARGINATUM
Rare- 1%
HISTORY:
disappearing non pluritic skin lesion
 Erythematous, serpiginous, macular lesion with pale centre.
 Mainly on trunk and extremities
 Accentuated by warming the skin.
V. SUBCUTANEOUS NODULES
Rare ≤1%
 reveals, Firm nodules approximately 0.5-1cm in diameter along
extensor surfaces of tendons near bony prominences.
 There is a correlation between presence of this nodules and
significant rheumatic heart disease.
DIFFERENTIAL DIAGNOSIS OF RF
Differential diagnosis of Acute Rheumatic Fever

Arthritis Carditis Chorea
Juvenile idiopathic arthritis Viral myocarditis Huntington chorea
Reactive arthritis (eg. Shigella, Viral pericarditis Wilson disease
Salmonella, Yersinia)
Serum sickness Infective endocarditis SLE
Sickle cell disease Kawasaki disease Tic disorder
Malignancy CHD Hyperactivity
SLE Mitral valve prolapse Enchephalitis
Lyme disease (Borrelia burgdoferi) Innocent murmur
Pyogenic arthritis
Poststreptococcal reactive arthritis

Treatment
 All patients should be placed on bed rest and monitored for
evidence of carditis and allow ambulation after improvement of
acute inflammation. Patients with carditis require longer bed rest.
 Antibiotics
 Anti-inflammatory therapy and
 Sedatives (for chorea).
COMPLICATIONS - arthritis & chorea completely resolve without

sequelae.
 Infective endocarditis
 Rheumatic heart disease
Prognosis:
 depends on clinical manifestations of initial episode, severity
of the episode and presence of recurrence
 ~ 50-70% of patients with carditis initially recover without
residual heart disease (the more severe initial cardiac
involvement the greater risk of residual heart disease).
 Patients without carditis initially are less likely to have
carditis on recurrence (but increased attack enhance risk of
cardiac involvement) and risk of permanent heart damage
increase with each recurrence.
NB: patients having previous ARF have 50% risk of recurrent attack
from each GAS pharyngitis, that’s why they require long term
continuous chemoprophylaxis (2ry prophylaxis). Risk of recurrence is
high in the first 5 yrs of initial episode & decreases with time.
Patients with pure chorea even without other manifestations of ARF
require prophylaxis, b/c ~ 20% of them without prophylaxis develop
RHD in 20 yrs.

Prevention
 PRIMARY PROPHYLAXIS - treating the first attack of GAS pharyngitis
in the first 9 days of the symptoms. (mentioned above)
 SECONDARY PROPHYLAXIS
Directed at preventing acute GAS pharyngitis in

patient with substantial risk of recurrent ARF
Requires continuous antibiotics (see tables below)
Should be given as soon as the DX of ARF has been
made and immediately after the full course of
antibiotic therapy has been completed.
Duration depends on presence of carditis or not and
whether a residual heart disease is developed or not.
Because, carditis on initial episode is most likely also
present on the recurrent attack and deteriorates
cardiac structures).
Decision to discontinue depends on careful
consideration of potential risks and benefits and
observation of epidemiologic factor like risks for
exposure to GAS pharyngitis.
 TERTIARY PROPHYLAXIS-to prevent further cardiac deterioration.
(Sometimes it’s not mentioned).

Chemoprophylaxis for Recurrences of Acute Rheumatic

Fever (Secondary Prophylaxis)
Drug Dose Route
Penicillin 600,000 IU for children weighing <60Ib IM
G and 1.2 million IU for children >60Ib
benzathine every 4wk
OR
Penicillin 250mg twice daily Oral
V
OR
Sulfadiazi 0.5g once daily for patients weighing Oral
ne or <60Ib
Sulfisoxaz 1g once daily for patients weighing >60Ib
ole
For people who are allergic to penicillin and sulfonamides Drugs
Macrolides Variable Oral
or Azalids
Recommendation
Category Duration
Rheumatic fever without carditis 5yr or until 21 yrs of age
whichever is longer
Rheumatic fever with caritas but 10yr or until 21 yrs of age
no without residual heart disease whichever is longer
(no valvular lesion)
Rheumatic fever with caritas and 10yr or until 40yrs of age
residual heart disease (persistent whichever is longer sometimes
valvular lesion lifelong prophylaxis

C. Rheumatic heart disease

 Is the permanent damage of valves
 Usually results from repeated episode of ARF
 Is most important sequela of ARF and is 2nd next to arthritis.
 Mitral valve is the most affected and followed by aortic valve. But
isolated aortic valve is rare rather it concomitantly occurs with
mitral valve.
 Right side heart manifestations are rare, it only associated with
the left side valve disease.
.
NB: DX can be made only by subclinical carditis (SCC) w/c means:
echocardiographic evidence of mitral or aortic valvulitis in absence of
auscultatory finding and not consistent with physiologic mitral or
aortic insufficiency.
Echocardiographic Findings in Rheumatic valvulitis

Pathologic Mitral Regurgitation Pathologic Aortic Regurgitation
1. Seen in at least 2 view 1. Seen in at least 2 view
2. Jet length >= 2cm in at least 2. Jet length >= 2cm in at least
1view 1view
3. Peak velocity >3meters/sec 3. Peak velocity >3meters/sec
4. Pan-systolic jet at least 1 4. Pan-systolic jet at least 1
envelope envelope
Patient approach:
HISTORY:
 Symptoms-
 Patients are usually asymptomatic in mild lesion.
 In severe lesion (differ by the lesion pattern , see
below):

 Palpitation, shortness of breath, fatigue and weight

loss are commonly seen in most patterns of the
lesions.
 edema
 orthopnea & PND
 bloody sputum
 sweating
 Complication –
 Infective endocarditis (see below)
 Chills, Chest pain and abdominal pain
 Arthralgia and myalgia
 Dyspnea ,malaise ,weakness
 Night sweat
 Differs again by the lesion type and severity again (murmurs,
heave, thrill, accentuated heart sounds, S3 gallop, tender
hepatomegaly and edema and splenomegaly for IE.
Patterns of valvular Disease

I. Mitral insufficiency(MR)
 Resulted from structural changes such as, loss of valvular
substances and/or subvalvular apparatus like elongation of
chordae tendonea.
 Results HF by coupling with pancarditis during the severe carditis
of ARF.
 Involve dilation of LV and enlarging LA by increased pressure in it
and bring pulmonary congestion finally.
IN MILD LESION-
 History: asymptomatic
 Physical Examination: shows quite precordium, high pitched
HSM @apex radiating to axilla and
 Investigations: normal ECG & CXR findings.

IN SEVERE LESION-
 History: palpitation, Shortness of breath and can have orthopnea

& PND.
 Physical Examination:
 Palpation: PMI is shifted(cardiomegaly), apical heave (LV
hypertrophy) & apical systolic thrill
 Auscultation: accentuated s2, prominent s3, apical HSM
radiating to axilla and short mid-diastolic rumbling murmur
(called relative mitral stenosis or Carey-Coombs murmur)
resulted from increased blood flow across the insufficient
valve.[BUT,MS is the result of chronic lesion after several yrs]
 Investigations:
 ECG- prominent, longer duration and often bifid p waves, signs
of both LV & LA hypertrophy, RV hypertrophy if PHTN is
developed.
 CXR- congestion of perihilar vessels, prominent LA (More seen
on Lateral view) and LV, Signs pulmonary vein HRN.
 ECHO- enlarged LA & LV, impaired LV systolic
function,[chordal thickening, chordal fusion and restricted
leaflet motion>> in Chronic RHD], NB: Calcification is rare in
children.
Treatment:
 In mild: typical Rx of ARF plus prophylaxis against recurrence.
 In severe: corticosteroids, Rx of complicating HF, arrhythmia and
Infective endocarditis, ACE inhibitors & diuretics.
 Surgery is indicated for persistent HF, dyspnea on mild

activities & progressive cardiomegaly. Patients with prosthetic
valve need prophylaxis during dental procedures to prevent

II. Mitral stenosis(MS)

 Is chronic process (≥10 yrs), but it may be accelerated.
 Resulted from fibrosis of mitral ring, commissural adhesion &
contraction of the leaflets
MILD LESION: asymptomatic, normal heart size and normal finding of

ECG and CXR, only evident in ECHO.
SEVERE LESION:
 History: Exercise intolerance, dyspnea, orthopnea, PND,

hemoptysis (rupture of bronchial & perihilar vessels + pulmonary
infarction).
 Physical Examination: Atrial fibrillation, RHF (hepatomegaly,
ascites, edema),moderate cardiomegaly, accentuated s1(MS)&
s2(PHTN),long low high pitched rumbling diastolic murmur with
Presystolic accentuation at apex, HSM of TR at LLSB(if present)
 Investigation:
 ECG-prominent & notched p wave, varying degrees of RV
hypertrophy, AF.
 CXR-LA enlargement, Kerley B line(horizontal lines @ lower
lung periphery)
 ECHO-thickening of mitral valve, restricted mitral valve motion,
“elbow (dog leg)” appearance of anterior leaflet of mitral
valve.[is the distinguishing feature of rheumatic mitral
stenosis from congenital stenosis).
TREATMENT – Medical + Surgical
III. Aortic insufficiency (AR)

 is acute and show poor coaptation of leaflets or leaflet prolapse.
 Can occur in isolation 1-2 yrs after initially combined with mitral
 Hx:
 palpitation, sweating and heat intolerance (b/c of excessive
vasodilatation), dyspnea, orthopnea, angina with heavy

exercise, nocturnal attack with sweating, tachycardia, chest

pain.
 Physical Examination:
 Findings in children with AR is often subtle because most
have mild disease. In addition, some of the classic findings
in adults are seldom found in children, even in those with
moderate to severe AR.
 Both examination of the peripheral pulses and auscultation
of the heart are particularly important. The increased stroke
volume results in abrupt distension of the peripheral
arteries and an elevation in systolic pressure. Regurgitation
back into the left ventricle then leads to a rapid decline in
pressure with quick collapse of the arteries, and a low
diastolic pressure that can fall below 30 mmHg in severe
disease.
 wide pulse pressure (pp) with bounding peripheral
pulse(called water-hammer or Corrigan pulse) ,elevated
SBP and decreased DBP, shifted PMI (in severe), diastolic
thrill
 Murmurs:
 High pitched, blowing, Diastolic murmur easily audible in
full expiration & leaning forward by placing diaphragm
firmly and heard best @upper & mid-left sternal border
radiating to apex and upper Rt sternal border.
 Aortic systolic ejection murmur b/c of increased SV.
 Apical presystolic murmur (Austin Flint murmur)
resembling MS & caused by large regurgitant aortic flow
in diastole preventing the opening of MV fully.
 Other findings are associated with a hyper-dynamic pulse: The so
called peripheral stigmata of AR:
 De Musset's sign − A head bob occurring with each
heartbeat

 Traube's sign − A pistol shot pulse (systolic and diastolic

sounds) heard over the femoral arteries
 Duroziez's sign − A systolic and diastolic bruit heard
when the femoral artery is partially compressed
 Quincke's pulses − Capillary pulsations in the fingertips
or lips
 Mueller's sign − Systolic pulsations of the uvula
 Becker's sign − Visible pulsations of the retinal arteries
and pupils
 Hill's sign − Popliteal cuff systolic pressure exceeding
brachial pressure by more than 60 mmHg
 INVX:
CXR-enlarged LV & aorta
 Investigations
ECG-may be normal or LV hypertrophy with

strain pattern and prominent p wave.

ECHO-dilated LV & diastolic MV flutter or oscillation

caused by aortic regurgitant flowing hitting the valve
leaflet.
 PROGNOSIS
 mild & moderate lesions are tolerated
 Unlike MR, AR doesn’t regress.
 TREATMENT: medical + surgical
IV. Tricuspid valve disease
 Primary involvement is rare, but resulted from Left side HF
 Is usually TR and On Physical Examination: characterized by
prominent pulsation of Jugular vein, systolic pulsation of liver,
blowing HSM at LLSB increase with inspiration
 Concomitantly found with mitral or aortic valve disease with/out
AF.
 RX: medical + rarely surgical.
V. Pulmonary valve disease

 Rare & PR occur 2ry to pulmonary HTN.
 Is late finding with MS.
 Murmur called Graham steel murmur is similar with AR, but
lack the bounding pulse.
 Correct DX is made by two directional ECHO and Doppler studies.
2. INFECTIVE ENDOCARDITIS
 Is inflammation of endocardium usually valvular part.
 Is acute or sub acute or can be non-bacterial (viral,fungal or
others)

 Is the complication of CHD or RHD, but can happen without any

lesion.
 Main cause of mortality and morbidity despite of the advanced
prophylaxis today.
Etiologies
 viridian & staph aureus are the most common
 Streptococcus viridan- after dental procedure

 Staph aureus- common in patients with no underlying heart
disease.
 Group D enterococci- after lower bowel or genitourinary
manipulation.
 Pseudomonas-in IV drug users
 Fungal- after open heart surgery. (usually in infancy done for
CHD)
 CONS-common in presence of indwelling central venous catheter.
PATIENT APPROACH:
 Ranges from acute/severe to non-acute manifestations (between

the extremes).
HISTORY:
RISK FACTORS LIKE-
 Prior CHD or RHD

 Preceding dental
 urinary tract or intestinal procedure
 Intravenous drug use
 Central venous catheter
 Prosthetic heart valve.
SYMPTOMS:
 Fever (prolonged and with weight loss, in viridan & acute with
sweating in staph aureus)>> it increases afternoon.

 Chills, Chest pain and abdominal pain

 Arthralgia and myalgia
 Dyspnea, malaise, weakness
 Night sweat
COMPLICATIONS
 Glomerulonephritis (See in Renal Diseases)

 Symptoms of Anemia (See in Anemia)
 Symptoms Of Heart Failure
 Distant Infections like in CNS (Causing Stroke, seizure, headache
etc)
 Elevated TO, Tachycardia

 Embolic phenomena (Roth’s spot, petechiae, splinter hemorrhage,
Osler Nodes, CNS or ocular lesions)
 Janeway lesion (painless, small, Erythematous or hemorrhagic
lesion on palms or soles)
 New or changing murmur- is associated with HF
 Splenomegaly with petechiae(in <50%)
 Arthritis, Arrhythmia
 Clubbing
LABORATORY STUDIES:
 Positive blood culture (mainly required)

 High ESR (may be low in HF or RF),Elevated CRP
 Anemia, leukocytosis, immune complexes,
hypergammaglobinemia
 Cryoglobulinemia, Rheumatoid factor, hematuria
 Azotemia,high creatinine(RF by glomerulonephritis)
IMAGING
 CXR- bilateral infiltrates, nodules, pleural effusions

 ECHO- evidence of valve vegetation, prosthetic valve dysfunction

or leak, myocardial abscess, or new onset valve insufficiency.
Diagnosis
Use the modified Dukes criteria:
A. DEFINITIVE IE
 Clinical criteria: 2 major or 1 major + 3 minors or 5 minors

 Pathologic criteria: microorganisms picked from culture or
histologic examination of a vegetation or pathologic lesion;
vegetation or intra cardiac abscess by histologically showing
active endocarditis.
B. POSSIBLE IE – 1 major + 1 minor or only 3 minor Criterias
C. REJECTED DIAGNOSIS OF IE
 Firm alternative DX explaining evidence of suspected IE or

 Resolution of IE syndrome with antibiotic therapy for ≤4 days, or
 No evidence of IE at surgery or autopsy ,or on antibiotic Rx for ≤4
days, or
 Doesn’t meet criteria for possible IE.

The Modified Duke Criteria for the Clinical Diagnosis of Infective

Endocarditisa
Major Criteria
Major Criteris Minor Criteria
Minor Criteris
1. Positive blood culture 1. Predisposition: predisposing heart
Typical microorganism for infective endocarditis conditionsc or injection drug use
from two separate blood cultures
 Viridans streptococci, Streptococcus 2. Fever ≥38.0°C (≥100.4°F)
gallolyticus, HACEK group organisms,
Staphylococcus aureus, or 3. Vascular phenomena: major arterial emboli,
 Community-acquired enterococci in the septic pulmonary infarcts, mycotic aneurysm,
absence of a primary focus, intracranial hemorrhage, conjunctival
or hemorrhages, Janeway lesions
Persistently positive blood culture, defined as
recovery of a microorganism consistent with 4. Immunologic phenomena:
infective endocarditis from: glomerulonephritis, Osler’s nodes, Roth’s spots,
 Blood cultures drawn >12 h apart; or rheumatoid factor
 All of 3 or a majority of ≥4 separate blood
cultures, with first and last drawn at least 1 h 5. Microbiologic evidence: positive blood
apart culture but not meeting major criterion, as
or noted previously,d or serologic evidence of active
Single positive blood culture for Coxiella burnetii infection with an organism consistent with
or phase I IgG antibody titer of >1:800 infective endocarditis
2. Evidence of endocardial involvement
Positive echocardiogramb
 Oscillating intracardiac mass on valve or
supporting structures or in the path of
regurgitant jets or in implanted material, in
the absence of an alternative anatomic
explanation, or
 Abscess, or
 New partial dehiscence of prosthetic valve,
or
New valvular regurgitation (increase or change in
preexisting murmur not sufficient)
 Transesophageal echocardiography is required for optimal

assessment of possible prosthetic valve endocarditis or
complicated endocarditis.
 Valvular disease with stenosis or regurgitation, presence of a
prosthetic valve, congenital heart disease including corrected or
partially corrected conditions (except isolated atrial septal defect,

repaired ventricular septal defect, or closed patent ductus

arteriosus), prior endocarditis, or hypertrophic cardiomyopathy.
 Excluding single positive cultures for coagulase-negative
staphylococci and diphtheroids, which are common culture
contaminants, or for organisms that do not cause endocarditis
frequently, such as gram-negative bacilli.
PROGNOSIS- 20-25% will die despite of Rx and 50-60% has serious
morbidity.
COMPLICATIONS:
 Systemic Emboli like to CNS(Major threat)

 pulmonary Embolism in patients with VSD or TOF
 Mycotic aneurysm,
 Acquired VSD
 Heart block resulted from abscess on conduction system
 Osteomyelitis
 Arthritis
 Renal abscess
 Purulent pericarditis and
 Immune complex mediated glomerulonephritis.
TREATMENT
 Bactericidal antibiotics(Parenteral) immediately after

definitive DX is made
 Amphotericin B and 5-fluorocytosine for Fungal
Endocarditis.
 Surgery
 Treat any complications like heart failure (diuretics, digitalis
and ACE inhibitors), stroke and RF immediately.

PREVENTION: is by antibiotics by using d/t criteria and having good

oral hygiene.
 Make sure that you have grasped the following points under RHD
 GAS pharyngitis and golden time for Rx(9 days) + RX options
 DX of ARF (Modified Jones criteria )
 The prophylaxis (10,20,some times 30)
 Common investigations with their findings
 Maneuvers for Murmurs.
 IE: common causes with their predisposing factors, modified
Dukes criteria and complications of IE.

SUMMARY OF APPROACH TO CHF 2ry to RHD.
 History
 ID: age (commonly 5-15 yrs) and being female is pertinent
 PREVIOUS ADMISSION: Essential to know any recurrence, write
the details in HPI
 C/C: SOB commonly, OR GBS
 HPI (on SOB)
 Elaboration of the symptom: when it started, gradual or
acute onset (HF is usually gradual), the progression
(initially with heavy exercise followed by moderate activities
like fast walking, next with mild activities and eventually @
rest)
 ASSOCIATED SYMPTOMS:
 Cough (characterize it)
 Orthopnea (ask number of pillows),
 PND,
 weight loss,
 appetite loss,
 fatigue,
 body swelling and
 any related recent sore throat + migratory joint pain +
abnormal body movement (ARF).
 RF: large family size, being in developing country by its self
 ANY PREVIOUS TREATMENT? - is important to know:
 the recurrence of the previous episode
 misdiagnosis made and given inappropriate
medication
 any side effect of drugs>>if present;
 Ask when it was? Complaint of the patient by then,
Dx made, drug given [name or color and shape), dose

in number or gram, frequency] and outcome after Rx

and time of Discharge.
 COMPLICATIONS:
 body weakness (cardio-emboli stroke)
 prolonged fever, weakness, night sweat (Infective
endocarditis),
 right quadrant pain, yellowish discoloration of eye and
skin (congestive hepatopathy),
 Decreased urine output (Cardio renal syndrome) or
urine color change (glomerulonephritis.)
 DDX:-
 History of chronic cough and history of contact with chronic
coughing (R/o pulmonary TB)
 History of episodic cough worsening by cold air, history of Asthma
in family (r/o Asthma)
 History of pleuritic chest pain (r/o Pleural effusion)
 Other Differential DIagnosislike pertussis, croup and bronchiolitis
can be ruled out by age and while characterizing the cough.
 Self and family history of DM, HTN, and whether he/she has
been tested for RVI and the result (+ve/-ve). If not evaluated for
DM (ask the poly Symptoms).
 Nutritional hx, Developmental hx, Immunization hx should be
assessed carefully
 Physical examination: (the pertinent systems)
 GA: acute sick looking, acute on CSL, may look depressed,
conscious or unconscious (if so assess GCS).
 Vital Signs: tachypnea, tachycardia, normotensive or
hypotensive, fever (as precipitating factor, evidence of IE).
 ANTHROPOMETRY: may show SAM (as a cause for HF),
 HEENT:
EYE- Pallor (anemia), jaundice
MOUTH- Central cyanosis

 Chest: Chest lag, dullness on percussion, bilateral rales and

decreased or absent breath sound-pleural effusion
 CVS: -
 Venous examination
 Arterial Examination
 Precordial Examination
 Inspection
 Palpation
 Auscultation –heart sounds, gallops and Murmurs.
 Abdomen- Ascites, tender hepatomegaly, splenomegaly

 MSS- Edema, arthritis.
 Integumentary system– Pallor (anemia), jaundice, peripheral
cyanosis, clubbing
 NS: motor examination may reveal weakness or paralysis
(cardio-emboli stroke).
 INVESTIGATIONS
 CBC (anemia,infection)
 Blood culture (for IE, if suspected)
 ESR (IE)
 CRP (IE)
 RFT (complication, if suspected)
 LFT (complication again)
 CXR (Cardiomegaly, pulmonary vessel prominence, kerley B
line)
 ECG (r/o arrhythmia, hypertrophy)
 ECHO (Vascular lesion, Ejection fraction, Intra mural thrombus,
chamber dilation, PAP) …. GOLD STANDARD

Sample history for RHD

 ID- Female patient and Age -13 yrs
 C/C: Shortness of breath of 2 weeks’ duration
 HPI:
 She was relatively healthy 6 weeks back at which the time she
started to experience shortness of breath with vigorous
activities like running with her friends. 4 weeks back she
couldn’t walk up the stairs comfortably and 2 weeks later she
even was uncomfortable at rest.
 Associated with this she has hx of dry and hacking cough of 1
weeks duration w/c exacerbates on lying on her back and
relieves by sitting. She also has hx of orthopnea of 3 pillows,
PND and loss of appetite of the same duration.
 For the above complaints she visited the nearby health center
and referred immediately to our hospital.
 2 months back, she was having migratory type of joint pain on
her knee, ankle and wrists joints, but relieved without
treatment, but she has no hx of abnormal body movement and
she denied any recent sore throat. (ARF for RHD)
 Otherwise,
 She has no hx of fever,chills, chest pain, abdominal pain, night
sweat. (Complication>>IE)
 She has no hx of body weakness (Complication>>cardio
embolic stroke)
 Again, she has no hx of chronic cough and contact with chronic
coughing person. (DDX>> r/o Pulmonary TB) [other symptoms
like night sweats, weight loss can be for IE too).
 She has no hx of pleuritic chest pain (r/o Pleural effusion)

 She has no hx of episodic coughing which worsen by cold air

and has no family hx of Asthma. (R/o Asthma)
 She has no hx of unusual thirst, hunger and excessive
urination (to ask DM historically, which is one of the
chronic diseases. In patients who have never had blood
test for DM, ask poly symptoms) and no family hx of DM.
 She has no self and of family hx of HTN.
 She was screened for RVI 6 months back and told to be
sero-negative.
 (Chronic diseases: DM, HTN, RVI and Asthma…. Should be
asked usually).

2. Congenital Heart Diseases

 Congenital heart disease (CHD) occurs in approximately 0.8% of
live births.
 The incidence is higher in still borns (3–4%), spontaneous

abortuses (10–25%), and premature infants (approximately 2%
excluding [PDA]).
 Congenital cardiac defects have a wide spectrum of severity in

infants:
 CHD remains the leading cause of death in children with

congenital malformations.
 Most congenital defects are well tolerated in the fetus because of

the parallel nature of the fetal circulation
 One notable exception is the case of severe regurgitant lesions,

most frequently of the tricuspid valve , In utero heart failure may
occur --> non immune hydrops fetalis
 Associated non cardiac malformations noted in identifiable

syndromes may be seen in as many as 20-45% of patients with
congenital heart disease.
 Gender differences in the occurrence of specific cardiac lesions

have been identified.
 Example: TGA more common in boys whereas ASD, VSD, PDA

and PS are slightly common in girls

Approach to A patient with Congenital Heart Diease

History
RISK FACTORS
 The cause for most is unknown.

 Many cases are multifactorial.
 Chromosomal abnormalities, in particular, trisomies 21,13 &
18 and Turner syndrome
 Maternal infection during pregnancy
 Congenital rubella syndrome(PDA,
 Maternal history of teratogenic medications like lithium,
warfarin anticonvulsants…
 Maternal alcohol consumption
 Fetal alcohol syndrome (craniofacial abnormality, IUGR, and
cardiac defect-->VSD,ASD)
 Maternal history of smoking
 Maternal history of DM commonly associated with dTGA, VSD,
and AS
 Maternal obesity
 Family history CHD, neonatal death--->risk of recurrence is
higher if first degree relative are affected
 Prematurity and high altitude are risk factor for (PDA)
SYMPTOMS: VARY BASED ON,
 Type of the defect
 Size of the defect
 Whether its compensated or decompensated
 Age of the patient
 Presence or absence of complications
 Infants

 bluish lips, skin, fingers, and toes worsen while crying

 breathlessness or fast breathing
 feeding difficulties and sweating ask frequency of feeding
and time spent on each breast
 low birth weight
 Older children
 Difficulty of walking in equal pace with peers
 Orthopnea or shortness of breath in supine position
 delayed growth
 dizziness
 Cough and trouble breathing
 Fainting(syncope)
 Body swelling usually start from lower extremity
 Fatigue and exercise intolerance
 Squatting after exercise to relief shortness of breath is
the feature of child with TOF
 Low urine output due to decreased renal perfusion in a
patient with (COA)
 Weakness or pain in legs after exercise(claudication) in a
patient with(COA)
 History of cough and fever(=>pulmonary infection as
complication)
 Chest pain is unusual manifestation in pediatric
patients
PAST MEDICAL AND SURGICAL HISTORY: ask for previous history of
admission with symptoms of heart failure or any medication the
patient is taking or any cardiac surgery done

COMPLICATIONS
 Cerebral thrombosis secondary to polycythemia on long

standing cyanosis ask any history of body weakness
 Brain abscess ask for fever, weakness and headache in older

children.
 Infective endocarditis (fever, malaise, weight loss)
 Recurrent pulmonary infection ask history of fever, cough and

fast breathing
 Infective endarteritis(PDA)
 Reversal of shunt (Eisemenger syndrome)=>leads to cyanosis
 Recurrent heart failure (large VSD, large PDA...)
Physical examination
GENERAL APPEARANCE
 Visible wasting,
 Signs cardio-respiratory distress
 Look for any dysmorphic features
 Color(cyanosis)
VITAL SIGNS
 PR:
 Careful evaluation of the character of the pulses is
important (characterize rate, rhythm, and volume)
 palpate all accessible arteries

 The radial and femoral pulses should always be palpated

simultaneously
 tachycardia(CHF
 wide pulse pressure(PDA,A-V shunt
 Radio-femoral delay(COA
 RR => should be counted before touching because agitation

increase the rate
 T => fever => IE, lung infection

 BP => in all four extremity =>COA [upper limb=> hypertension,
and lower limb => hypotension]
ANTHROPOMETRY:
Accurate measurement of height and weight and plotting on a
standard growth chart are important
HEENT:
pale conjunctiva, central cyanosis (see lips, tongue, and mucous
membrane)
RESPIRATORY SYSTEM
 Inspection
 any chest deformity for associated malformation (Marfan
syndrome)
 Signs of respiratory distress nasal flaring, chest

retraction and tachypnea
 Palpation: increased tactile fremitus

 Percussion: relative dullness

 Auscultation: Basal crepitation, wheezing (cardiac asthma)

CVS
 General:
 cyanosis
 Clubbing
 JVP: not reliable in infants, but measure for older children

 Precordium examination
 Inspection
 Precordial bulging (cardiac enlargement => chronic) best
appreciated by having the child lay supine with the
examiner looking up from the child’s feet.
 Active or quite(>2 impulse=>active)
 Apical impulse normally in the left fifth ICS at MCL
 Palpation:
 Parasternal heave => Right ventricular hypertrophy
 Apical heave => Left ventricular hypertrophy
 Thrill systolic or diastolic (characterize by palpating

carotid pulse)
 PMI normally at left fifth ICS at MCL (laterally

shifted=>cardiomegaly)
 Auscultation
 S1&S2

 Murmur characterize by grading, timing, site, quality,

radiation,
ABDOMEN:
 tender hepatomegaly => CHF

 Shifting dullness and fluid thrill=>Ascites
I/S:
Palmar pallor grades it as severe if there is involvement of palmer
crease
MSS:
peripheral edema usually starting from lower extremity
 Congenital cardiac defects can be divided into 2 major

groups based on the presence or absence of cyanosis, which
can be determined by physical examination aided by
Pulseoximetry.
1. ACYANOTIC CONGENITAL HEART LESIONS

 Can be classified according to the predominant physiologic load
that they place on the heart.
A. ACYNOTIC LESIONS RESULTING IN INCREASED VOLUME LOAD
 The most common lesions in this group are those that cause
left-to right shunting
 Atrial septal defect(ASD)
 Ventricular septal defect (VSD),
 AV septal defects (AV canal), and
 Patent ductus arteriosus. (PDA)
COMMON FEATURES:

i. Degree of left to right shunt, and consequently

clinical manifestations is dependent on:
a- Size of the defect.
b- Pressure gradient across the defect.
c- Pulmonary vascular resistance.
ii. Manifestation of increase pulmonary blood flow:

a- Poor feeding & tachypnea.
b- Recurrent chest infections & chest wheezes.
c- Recurrent heart failure.
d- Growth failure.
iii. Heart failure doesn't occur in neonates but can occur in

infancy as pulmonary vascular pressure declines
iv. Prolonged pulmonary blood overflow  pulmonary

hypertension develops  . with time right heart pressures
exceed that of the left  reversal ofthe shunt (Eisenmenger
syndrome)  central cyanosis,
 This occur: -
 Early in large VSD & ECD.
 Late in ASD & other small defects.
B. ACYANOTIC LESIONS RESULTING IN INCREASED PRESSURE LOAD
 The pathophysiologic common denominator of these lesions is an

obstruction to normal blood flow.
 The most frequent are obstructions to ventricular outflow:
 valvular pulmonic stenosis,
 valvular aortic stenosis, and

 coarctation of the aorta
 Less common are obstruction to ventricular inflow:
 tricuspid or mitral stenosis,
 cortriatriatum and obstruction of the pulmonary veins.
 Ventricular outflow obstruction can occur: at the valve, below the

valve, or above it.
2. CYANOTIC CONGENITAL HEART LESIONS

 These can also be further divided into 2 according to
pathophysiology:
I. PULMONARY BLOOD FLOW IS DECREASED (OLIGAEMIC)
 Tetralogy of Fallot (TOF)
 Pulmonary atresia with an intact septum(PA)
 Tricuspid atresia(TA) and
 Total anomalous pulmonary venous return with

obstruction(TAPVR)
II. PULMONARY BLOOD FLOW INCREASED (PLETHORIC)
 Transposition of the great vessels(TGA)
 Single ventricle,
 Truncus arteriosus,
 Total anomalous pulmonary venous return without

obstruction).
 The chest radiograph is a valuable tool for initial differentiation

between these two categories.

Lesion % of all lesions
Ventricular Septal Defect 25-30
ASD (Secundum) 6-8
PDA (Patent ductus arteriosus) 6-8
COA (Coarctation of aorta) 5-7
TOF (Tetralogy of Fallot) 5-7
Pulmonary valve stenosis 5-7
Aortic valve stenosis 4-7
D-TGA 3-5
Hypoplastic left ventricle 1-3
Truncus arteriosus 1-2
TAPVR 1-2
Tricuspid atresia 1-2

Congenital malformation syndromes associated with CHD
SYNDROMES COMMON FEATURES
Down syndrome Endocardial cushion defect, VSD,

ASD
Turner syndrome Bicuspid aortic valve, COA
Trisomy 18(EDWARD’S) VSD, ASD, PDA, COA
Trisomy 13(PATAU) VSD, ASD, PDA, COA
Marfan syndrome Aortic and mitral insufficiency,

MVP
Congenital rubella syndrome PDA, Peripheral PS
Fetal alcohol syndrome ASD, VSD
VATER* association VSD, TOF, ASD, and PDA
*VATER (vertebral, anal, tracheoesophageal, radial and renal

anomalies

Discussion of some common CHD

1. Tetralogy of fallot (TOF)
 Is one of the conotruncal family of heart lesions in which the
primary defect is an anterior deviation of the infundibular septum
(the muscular septum that separates the aortic and pulmonary
outflows).
 The consequences of this deviation are the 4 components:
1. Obstruction to right ventricular (RV)outflow (pulmonary
stenosis),
2. Non-restrictive type of ventricular septal defect (VSD),
3. Dextroposition of the aorta so that it overrides the ventricular
septum, and
4. Right ventricular hypertrophy
 The degree of pulmonary outflow obstruction and whether the
ductus arteriosus is open or closed determine the degree of the
patient's cyanosis and the age at first presentation.
 Degree of pulmonary stenosis (P.S.) determine the degree of
right to left shunt
 When pulmonary stenosis is mild to moderate and balanced
shunt across VSD present, the patient may not be visibly
cyanotic (pink TOF)
 CHF is not usual manifestation of TOF
APPROACH
HISTORY
RISK FACTORS
 Viral illness during pregnancy, rubella (Germans measles) ask for

fever, rash.
 Alcoholism during pregnancy
 Poor maternal nutrition
 Advanced maternal age >40yrs

 Presence of Down syndrome
SYMPTOM
 Difficulty breathing on exertion in older children
 Squatting positioning after short time of activity to relief

dyspnea
 Difficulty in feeding
 Failure to gain weight
 Loss of consciousness(fainting)
 Irritability and prolonged crying
 General Appearance:
 CSL (wasting, Zygomatic prominence)
 Dysmorphic features
 Vital sign
 PR: usually normal
 BP: usually normal
 RR: maybe tachypnea
 Anthropometry: growth retardation(stunted)~in untreated cases
 HEENT:
 gray sclerae on longstanding cyanosis
 pale conjunctiva

 CVS
 General
 Central cyanosis:-Usually appear later in the first year of
life (may beat birth)- Cases with mild to moderate
pulmonary stenosis may not be initially visibly cyanotic
(acyanotic m: pink Fallot).
 Clubbing: related to the degree & duration of cyanosis
 Paroxysmal hypercyanotic (hypoxic) spells: Due to
infundibular spasm~ decrease of already reduced
pulmonary blood flow.
 Precordial examination
 Inspection: bulged pericordium in older children
 Palpation:
 Parasternal heave
 Systolic thrill may be felt along the left SB in the 3rd and
4th spaces.
Auscultation:
 systolic murmur is usually loud and harsh
 most intense at the left sternal border.
 generally ejection in quality at the upper sternal

border
 more holosystolic toward the lower sternal border
 S2 is single, or the pulmonic component(P2) is soft

DIAGNOSIS
CXR
 Decreased pulmonary blood flow (oligaemic lung field)
 Boot shaped heart
 Aorta is usually big (right arch of aorta in 20%of cases)
ECG : RVH (right axis deviation)
ECHOCARDIOGRAPHY
 Diagnostic tool
 Degree of overriding
 Degree of pulmonary stenosis
 RV wall thickness
CARDIAC CATHETERIZATION: preoperative
Treatment
 Medical: Treatment of hypoxic spells
 Avoid cerebral thrombosis
 In Fallot with severe cyanosis at birth --+ keep PDA by PGE 1

infusion.
 Prophylaxis & treatment of infective endocarditis.
 Surgical
 Palliative shunts
 Modified Blalock - Taussig operation: anastomosis between

subclavian artery & ipsilateral pulmonary artery
 Waterston operation: anastomosis between ascending aorta

& right pulmonary artery (obsolete)
 Potts operation: anastomosis between descending aorta &

left pulmonary artery (obsolete).
 Total correction: can be done between 4 months to 2 years

according to severity.
PROGNOSIS
 After successful total correction, patients are generally

asymptomatic and are able to lead unrestricted lives.
 Asymptomatic patients nonetheless have lower-than-normal
exercise capacity, maximal heart rate, and cardiac output.
2. Dextro Transposition of Great Arteries(dTGA)

 Common cyanotic congenital anomaly, accounts for
approximately 5% of all congenital heart disease
 The aorta arises from the right ventricle and The pulmonary artery
from the left ventricle
 The systemic and pulmonary circulations exist as 2 parallel

circuits.
 Survival in the immediate newborn period is provided by the

foramen ovale and the ductus arteriosus
 Approximately 50% of patients with dTGA also have a ventricular

septal defect (VSD), which usually provides for better mixing.
 The clinical findings and hemodynamics vary in relation to the

presence or absence of associated defects (e.g., VSD or pulmonary

stenosis).
 DTGA is more common in infants of diabetic mothers and in males

than female (3 : 1)
 Before the modern era of corrective or palliative surgery, mortality

was >90% in the 1st yr of life
APPROACH
HISTORY
 RISK FACTORS
 History of maternal viral infection during pregnancy
 Drinking alcohol during pregnancy
 Smoking during pregnancy
 A mother who has poorly controlled diabetes (ask for

polysymptoms)
 SYMPTOMS
 Bluish lips, skin, fingers and toes

 Fast breathing is most often recognized within the first hours
or days of life.
 The infant will be easily fatigued
 Interruption to breast feeding
 Untreated, most of these infants would not survive the
neonatal period
 Physical findings, other than cyanosis, maybe remarkably

nonspecific
 Pericordial examination

 Palpation:
 The precordial impulse may be normal,
 Parasternal heave may be present.
 Auscultation:
 (S2) is usually single and loud, although it may be split.
 Murmurs may be absent, or a soft systolic ejection murmur
may be noted at the mid-(LSB)
DIAGNOSIS
 CXR
 mild cardiomegaly
 a narrow mediastinum (the classic “egg-shaped heart”
 normal to increased pulmonary blood flow.

 ECG: usually normal
 Echocardiography: diagnostic
 Catheterization
TREATMENT
 Medical: Infusion of prostaglandin E1
 Palliative operation: Rashkind balloon atrial septostomy
 Total correction: Arterial switch operation or Atrial switch

operation

3. Atrial Septal Defect (ASD)
 Can occur in any portion of the atrial septum

 Ostium secundum (commonest): middle part;
 Ostium primum: (lower part) or
 sinus venosus (upper part of the septum)
 The majority of cases are sporadic
 An isolated valve-incompetent patent foramen ovale (PFO) is
usually of no hemodynamic significance and is not considered an
ASD
OSTIUM SECUNDUM DEFECT
 Females > Males(2:1)
 Peak age usually third decade or later
 In the region of the fossa ovalis
 the most common form of ASD
 may be single or multiple (fenestrated atrial septum)
 The degree of left-to-right shunting depends on:
 the size of the defect,
 the relative compliance of the right and left ventricles,

and
 the relative vascular resistance inthe pulmonary and

systemic circulations
APPROACH
HISTORY
 RISK FACTORS

 Rubella infection
 Cigarette smoking
 Drinking alcohol
 Maternal diabetes
 Symptoms
 A child with an ostium secundum ASD is most often

asymptomatic
 Mild fatigue or difficulty breathing, especially with exercise
 Even an extremely large secundum ASD rarely produces

clinically evident heart failure in childhood
 Younger children may show subtle failure to thrive
 Older children may have varying degrees of exercise

intolerance.
PHYSICAL FINDINGS
 Precordial examination
 Inspection: mid precordial bulge
 Palpation: parasternal heave
 Auscultation:
 second heart sound (S2 ) is widely split and fixed in its
splitting during all phases of respiration
 A systolic ejection murmur is heard at left middle and upper
SB
DIAGNOSIS
 CXR
 Cardiomegally
 The pulmonary artery is enlarged,

 Pulmonary vascularity is increased (plethoric lungs)
 ECG: shows RV volume overload
 Echo: diagnostic
TREATMENT
 Medical:
 Control heart failure (diuretics, digoxin, vasodilators)
 Prophylaxis against infective endocarditis usually not needed.
 Follow up with ECG & Echo to confirm to spontaneous closure
 Surgical: Transcatheter or open-heart surgical closure at 3-5

years.
PROGNOSIS
 Small to moderate-sized ASDs detected in term infants may

grow smaller or close spontaneously.
 Secundum ASDs are well tolerated during childhood, and

significant symptoms do not usually appear until the 3rd
decade or later
4. Ventricular septal defect (VSD)

 The most common cardiac malformation and accounts for 25% of
congenital heart disease.
 Defects may occur in any portion of the ventricular septum, but

the most common are of the membranous type
TYPES OF VSDS:

 Based on site:
1. Perimembraneous defect: The commonst type (70%)
2. Outlet defect; (also called infundibular or subarterialor

supracristal or subpulmonary).
3. Inlet defect.
4. Muscular defects: Either single or multiple (Swiss cheese)
 The physical size of the VSD is a major (but not the only)
determinant of the size of the left-to-right shunt.
 Based on the size of defect
 Restrictive VSD(<5mm)
 ModerateVSD(5mm_10mm)or
 NonrestrictiveVSD(>10mm
 In nonrestrictive defects the direction of shunting and the
shunt magnitude are determined by the PVRespiratory
SystemVR ratio
 The clinical findings of patients with a VSD vary according to
the size of the defect and pulmonary blood flow and pressure.
APPROACH
A. SMALL VSD
HISTORY
 These patients are usually asymptomatic
 Usually found during routine physical examination.
 precordial examination
 Palpation: Thrill at LLSB

 Auscultation:
 a loud, harsh, or blowing holosystolic murmur
 heard best over the lower left sternal border,
 it is frequently accompanied by a thrill
B. LARGE VSD
 Large VSDs with excessive pulmonary blood flow and pulmonary

hypertension are responsible for signs of congestive heart failure:
HISTORY
 RISK FACTORS
 Down syndrome
 Family history of VSD
 Maternal diabetes
 Maternal alcohol consumption during pregnancy
 SYMPTOMS
 Breathlessness (particularly on feeding or exertion)
 feeding difficulties (interruption while breast feeding)
 Cough and difficulty breathing
 Fatigue and weakness
 Body swelling usually starting from lower extremity
 poor weight gain
 profuse perspiration(sweating) and

 recurrent pulmonary infections in early infancy=>ask for

fever, cough and fast breathing
 General Appearance
 Look for any dysmorphic featurēs
 Cardiorespiratory distress
 Vital signs
 PR: tachycardia
 RR: tachypnea
 Anthropometry: measure weight and height, plot on appropriate

growth curve and interpret
 HEENT: pale conjunctiva

 Respiratory System: relative dullness (pulmonary edema) or
stony dullness(effusion), Bibasilar rales, Wheezing (cardiac
asthma)
 CVS:
 JVP: not reliable in infants
 precordial examination
 Inspection: active pericardium
 Palpation: Apical heave

 Auscultation:
 holosystolic murmur at LLSB less harsh than that of a

small VSD
 Accentuated P2
 mid-diastolic, low-pitched rumbling murmur at the

apex
 Abdomen: Palpation
 tender hepatomegaly
 Percussion: fluid thrill and shifting dullness --->ascites may

present
 I/S: palmar pallor
 MSS: pitting edema

DIAGNOSIS
 SMALL VSD
 CXR:
 usually normal,
 minimal cardiomegaly
 ECG:
 is generally normal but may suggest LV hypertrophy.
 The presence of RV hypertrophy on ECG is a warning that

the defect is not small
 LARGE VSD

 CXR:
 gross cardiomegaly
 Pulmonary vascular markings are increased,
 frank pulmonary edema, including pleural effusions, may

be present
 ECG:
 biventricular hypertrophy
 the P waves may be notched
 ECHO: visualize the defect
TREATMENT
 Medical:
 Control heart failure (diuretrics, digoxin, vasodilaters).
 Prophylaxis against infective endocarditis.
 Antibiotics for chest infections.
 Follow up with ECG & Echo to confirm spontaneous closure.
 Surgical: Types:
a- Palliative: Pulmonary artery banding (less favored).
b- Direct closure of the defect.
PROGNOSIS
 A significant number (30-50%) of small defect close

spontaneously, most frequently during first 2 years of life
 It is less common for moderate or large VSD to close

spontaneously
5. Patent Ductus Arteriosus(PDA)

 Functional closure of the ductus normally occurs soon after birth,
usually within the 1st wk of life
 But if the ductus remains patent when PVR falls, aortic blood then
is shunted left to right into the pulmonary artery.
 Because of the higher aortic pressure postnatally, blood shunts
left to right through the ductus, from the aorta to the pulmonary
artery.
 The extent of the shunt depends on the size of the ductus and on
the PVRespiratory SystemVR ratio
 Patients with a small PDA may live a normal span with few or no
cardiac symptoms, but late manifestations may occur.
 In patients with a large PDA, cardiac failure most often occurs in
early infancy but may occur later in life
 Spontaneous closure of ductus after infancy is extremely rare
 Female patients with (PDA) outnumber males 2 : 1.
APPROACH
HISTORY
 RISK FACTORS
 Maternal rubella infection during pregnancy
 Prematurity
 High altitude
 SYMPTOMS
 Small PDA:

 is usually asymptomatic
 is usually diagnosed by the presence of a heart murmur.
 is associated with normal peripheral pulses
 Large PDA:
 will result in heart failure
 Retardation of physical growth
 Vital signs
 PR: bounding pulses
 BP: wide pulse pressure
 PRECORDIAL EXAMINATION:
 Inspection: pericardial bulging
 Palpation:
 apical impulse is prominent and shifted
 Apical heave
 Systolic thrill second left ICS
 Auscultation: The classic continuous murmur
 “Machinery-like” in quality.
 It may be localized to the 2nd left intercostal space
 Radiate down the left sternal border or to the left clavicle.

Differential Diagnosis of machinery murmurs: e.g.:

1- Aortico pulmonary window.
2- Arterior venous fistula (systemic or pulmonary).
3- Ruptured sinus of valsalva..
INVESTIGATIONS:
 Chest x-Ray:
 Cardiomegaly (LVH & LAD), or BVH Plethora (increased

pulmonary vascular marking)
 aortic knob may be normal or prominent
 ECG: - LVH & LAD
 Echo: diagnostic.
 Cardiac catheter: pre-operative.
TREATMENT
 Medical:
 Control heart failure & prophylaxis against infective
endocarditis
 medical closure in preterm by I.V. indomethacin in the 1st
week of life
 Surgical: surgical ligation or trans catheter closure
6. Coarctation of Aorta(COA)
 Constrictions of the aorta of varying degrees may occur at any
point from the transverse arch to the iliac bifurcation,
 But 98% are juxta ductal coarctation (adult type)

 Post & preductal (infantile-type) are less common
 The anomaly occurs twice as often in males as in females.
 Coarctation of the aorta may be a feature of Turner syndrome
 Associated with a bicuspid aortic valve in >70% of patients
HISTORY
 RISK FACTORS
 Turner syndrome
 Edward’s syndrome
 Patau syndrome
 Anticonvulsants (valproate)
 SYMPTOMS
 Coarctation of the aorta recognized after infancy is not usually
associated with significant symptoms.
 Shortness of breath

 Poor feeding
 Sweating
 Headache
 Nasal bleeding
 Low urine out put
 Some children or adolescents complain about weakness or

pain/claudication (or both) in the legs after exercise
 VITAL SIGNS
 PR: tachycardia
 The femoral, popliteal, posterior tibial, and dorsalis

pedis pulses are weak (or absent in up to 40% of
patients), in contrast to the bounding pulses of the arms
and carotid vessels.
 Radio femoral delay (the femoral pulse is felt after the

radial pulse.)
 RR: tachypnea
 BP: BP in the legs is lower than that in the arms
 In normal persons (except neonates), systolic BP in the

legs obtained by the cuff method is 10-20 mm Hg higher
than that in the arms.
 CVS
 General: differential cyanosis in preductal type with patent

ductus.
 precordial examination
 Inspection: bulged pericardium
 Palpation: Apical heave
 Auscultation: systolic murmur is heard at the left sternal

border with a loudS2 radiate to left infra scapular area
and occasionally to neck
DIAGNOSIS
 CXR: findings depend on age of the patient

 Infants - cardiomegaly and increased pulmonary
vascularity
 Childhood - mild cardiomegaly and notching (Rosler sign)

of ribs(4th-8th)due to pressure from enlarged collateral
vessels
 ECG
 Normal in young children
 LVH in older patients
 Echo: visualize segment of coarctation

 associated anomalies of mitral and aortic valve

CHAPTER 7
APPROACH TO COUGH
COUGH is an explosive expiration that provides a normal protective
mechanism for clearing the tracheobronchial tree of secretions and
foreign material
 It Is an important defense mechanism of the lungs and is a
common symptom.
 The duration of the symptoms
 Determines the level of concern and
 Degree of workup that is warranted.
 An acute cough, lasting < 2 weeks, is frequently related to an
infectious illness and is often self-limited.
 A chronic cough, a cough that persists for ≥ 2 weeks and
suggests a potentially more serious underlying cause.
Physiologic Mechanics of cough

 THREE PHASES
1. Inspiratory phase
 air inhalation lengthens the expiratory muscles (favorable
length-tension relation)
2. Compressive phase
 Contraction of expiratory muscles against a closed glottis
leads to an increase in intrathoracic pressure.
3. Expiratory phase
 Opening of the glottis results in high expiratory flow and
audible coughs

APPROACH
HISTORY
 QUESTIONS TO ASK
1. Duration of cough
 Acute(less than 2 weeks)
 Chronic cough(more than two weeks)
2. Nature of cough
 Watch "Different types of cough sounds" on YouTube

https://youtu.be/YizKqqN8SWQ
 Barking/brassy cough (Laryngotracheobronchitis)

o Watch "What croup Cough sounds like and how to treat it -
Home Remedies" on YouTube
 https://youtu.be/O7fZDEBJ7TM
 Staccato cough ( Chlamydia in infant)

o Watch "Staccato Baby" on YouTube
 https://youtu.be/qdexsh6Ejmk
 Honking or Goose-like( Psychogenic )

 Whooping cough (Pertussis)
o What Whooping Cough Sounds Like - Toddler with the
"whoop" sound
 https://youtu.be/zuK4honWVsE
3. Time of the day the cough worsens
 Asthma (worsen during night and early morning)

4. Type of exposure that triggers the cough

 Seasonal aero allergens
 Noxious odor (perfumes)
5. The cough aggravator and reliever
 Asthma aggravated by exposure to allergens
6. Any shortness of breath (dyspnea)
 pericarditis
7. Associated vomiting (post-tussive emesis)
 Pertussis
8. Evidence of fevers, failure to thrive or weight loss
 Tuberculosis
9. Child exposure to smoke (tobacco, wood burn)
 Triggers asthma
10. History of choking
 foreign objects in airway
11. Pets or animals contact
 Triggers asthma
12. Prenatal and neonatal history.
 pneumonia
13. Family history of atopy (eczema, allergies, asthma)
Approach to acute cough

DIFFERENTIAL DIAGNOSIS FOR ACUTE COUGH
 Upper respiratory infection
 Rhinitis
 Gastroesophageal reflux
 Bacterial and other infections

 Croup (details under stridor)

 Bronchiolitis (details under wheezing disorders)
 Pneumonia
 Sinusitis, especially maxillary
 Bordetella pertussis
 Asthma (details under wheezing disorders)
 Inhalation of irritants (environmental, occupational)
 Smoke/smog
 Noxious fumes
 Extremely hot or cold air
 Pulmonary embolism
 Aspiration pneumonia
 Foreign body aspiration
 Laryngeal inflammation
 External or middle ear disease
 Acute pleural, pericardial, mediastinal, or diaphragmatic
inflammation
1. PNEUMONIA
Pneumonia is an inflammation of the parenchyma of the lungs.
EPIDEMIOLOGY
 Is a substantial cause of morbidity and mortality in childhood

(particularly among children <5 yr of age) throughout the world.
 Developed countries…….incidence of 0.026 /child-year

 Developing countries…...incidence of 0.28 /child-year
 Viral pneumonia…….peaks b/n 2 & 3 yrs, decreasing slowly thereafter.
ETIOLOGIES
 Although most cases of pneumonia are caused by microorganisms,

noninfectious causes include:-
 aspiration of food or gastric acid,
 foreign bodies,

 hydrocarbons, and lipoid substances,

 hypersensitivity reactions, and
 drug- or radiation-induced pneumonitis.
 Viral pathogens are a prominent cause of lower respiratory tract
infections in infants and children <5 yr of age, influenza virus and
respiratory syncytial virus are the major pathogens, especially in
children <3 yr of age.
 S. pneumoniae, H. influenzae, and S. aureus are the major causes
of hospitalization and death from pneumonia among children in
developing countries,

The age of the patient may help identify possible pathogens.

AGE GROUP COMMON PATHOGENS* (IN
APPROXIMATE ORDER OF
FREQUENCY
Neonates (up to 1 Group B streptococcus, Escherichia coli, other gram
month of age negative bacilli, Streptococcus pneumonia
1 to 3 months
Respiratory syncytial virus, other viruses (parainflenza
Febrile viruses, influenza viruses, adenoviruses),
pneumonia S.pneumoniae, H. inflenzae (type b,& non typable)
Afebrile Chlamydia trachomatis , Mycoplasma hominis,

pneumonia Ureaplasma urealyticum, CMV, Bordetella pertussis
3 months to 5 RSV, other respiratory

years viruses (parainfluenza viruses, Influenza viruses,
Human metapneumovirus, adenoviruses),S.
pneumoniae, H. inflenzae (type b,& nontypable)
5 to 18 years M. pneumoniae, S.
pneumoniae, C. pneumoniae,
≥18 years M. pneumoniae, S.pneumoniae, C. pneumoniae,
H. inflenzae (type b, nontypable), inflenza viruses,
adenoviruses,
PATTERNS OF PNEUMONIA
 There are five patterns of bacterial pneumonia:

1. Lobar pneumonia: involvement of a single lobe or segment of a
lobe; this is the classic pattern of S. pneumoniae pneumonia
2. Bronchopneumonia: primary involvement of airways and
surrounding interstitium; this pattern is sometimes seen in
Streptococcus pyogenes and Staphylococcus aureus
pneumonia
3. Necrotizing pneumonia:- associated with aspiration pneumonia
and pneumonia resulting from S. pneumoniae and S. aureus.
4. Caseating granuloma (as in tuberculosis pneumonia)

5. Interstitial and peribronchiolar with secondary parenchymal

infiltration: this pattern typically occurs when a severe viral
pneumonia is complicated by bacterial pneumonia
 There are two patterns of viral pneumonia:

1. Interstitial pneumonitis
2. Parenchymal infection with viral inclusions
Patient Approach
HISTORY
 Symptoms can be helpful in determining: -

 the etiologic agent,
 the likelihood of infection with an organism that is resistant to
antibiotics, and
 The severity of illness.
 Important parts of pneumonia hx;-
 Characterization of cough
 Age of the child
 Recent upper respiratory tract infection
 Fever and associated symptoms (eg, headache, conjunctivitis,
malaise, lethargy, pharyngitis, nausea, vomiting, diarrhea,
abdominal pain, rash)
 chest pain,
 shortness of breath,
 Increased work of breathing
 Choking episodes (may indicate foreign body aspiration)

 Duration of symptoms (chronic cough [ie, more than four

weeks])
 Previous episodes (recurrent episodes may indicate:-
 aspiration,
 congenital or acquired anatomic abnormality,
 cystic fibrosis,
 Immunodeficiency (HIV, immune suppressant
medications…),
 Asthma
 Immunization status (completion of the primary series of
immunizations for Haemophilus influenzae type b, Streptococcus
pneumoniae, and Bordetella pertussis decreases, but does not
eliminate, the risk of infection with these organisms)
 Previous antibiotic therapy (increases likelihood of antibiotic
resistant bacteria)
 Maternal history of chlamydia during pregnancy
 Exposure to tuberculosis
 Ill contacts (more common in viral illnesses)
 Travel or residence in certain areas that suggest endemic
pathogens (eg, Coccidioides immitis, Histoplasma capsulatum,
hantavirus)
 Animal exposure (may indicate histoplasmosis, psittacosis, Q
fever)
 Daycare center attendance (exposure to viruses and antibiotic
resistant bacteria)
 The child's fluid and nutrition intake

 Difficulty or inability to feed (in young infants)
Risk factors
 Low socioeconomic status ( overcrowding )
 Unimmunization
 Malnutrition, including Vitamin A and D deficiencies
 Congenital heart disease
 Bronchopulmonary dysplasia
 Asthma
 Male sex
 Immunodeficiency disorders(Congenital and acquired)
 Sickle cell disease
 Neuromuscular disorders, especially those associated with a
depressed consciousness
 Some gastrointestinal disorders (eg, gastroesophageal reflux,
tracheoesophagealfistula)
 Cigarette smoking (the adolescent or mother of the infant)
 Alcohol and other substances of abuse
 Cystic fibrosis
 General appearance
 In the young infants,

 ASL /respiratory distress and presence of cyanosis/
 Respiratory distress
 tachypnea,
 hypoxemia (usually oxygen saturation <94 to 96 percent in
room air), and

 increased work of breathing:
 Intercostal, subcostal, or suprasternal retractions;

 Nasal flaring;
 Grunting;
 Use of accessory muscles
 Infants and children with hypoxemia may not appear

cyanotic.
 Oxygen saturation should be measured in any child with
increased work of breathing, particularly if he or she has a
decreased level of activity or agitation
 More specific than fever or cough for lower respiratory tract
infection (LRTI).
 Unlike tachypnea, the absence of these findings does not
exclude a diagnosis of pneumonia.
 Vital signs
 Fever
 Is nonspecific and invariably present.
 Young infants may have afebrile pneumonia related to C.
trachomatis or other pathogens.
 On the other hand, fever may be the only sign of occult
pneumonia in highly febrile young children.
 Tachypnea
 Is the most sensitive and specific sign

WHO age-related definitions of tachypnea:

 Younger than two months: >60 breaths/min
 Two to 12 months: >50 breaths/min
 One to 5 years: >40 breaths/min
 ≥ 5 years: >20 breaths/min [ >30 @JMC and ≥ 24 for
≥8 yrs. @JMC]
 In infants with pneumonia (RR >70 breaths/min) also has

been associated with hypoxemia.
 Less useful early in the course of illness (eg, less than
three days).
 RR varies with activity in infants and young children, and in

these patients is best assessed by counting for a full 60
seconds by observing the chest wall movements.
 May increase by as many as 10 breaths per minute per
degree (Celsius) of fever in children with pneumonia.
 RESPIRATORY EXAMINATION
Provide clues to the diagnosis of pneumonia and/or potential

complications.
 Inspection: wheezing (in pneumonia caused by atypical
bacteria and viruses than bacteria.)
 Palpation: Decreased tactile fremitus (pleural effusion)
Tracheal deviation (pleural effusion)
 Percussion: Dullness (pleural effusion)
 Auscultation: of all lung fields.
 Crackles
 Decreased breath sounds (Consolidated lung parenchyma)
 Bronchial breath sounds
 Distant breath sounds and pleural friction rub (pleural
effusion)
Sign or symptom Classification
Cough or difficulty in breathing with: Severe

• Oxygen saturation < 90% or central Pneumonia
cyanosis
• Severe respiratory distress e.g.
grunting,
severe chest indrawing
• Signs of pneumonia with
• a general danger sign (inability to
breastfeed or drink, lethargy or
reduced
level of consciousness, convulsions)
Fast breathing Pneumonia
≥ 60 breaths/minute in a Child aged
<2 months;
≥ 50 breaths/ minute in a child aged
2–11 months
≥ 40 breaths/minute in a child aged
1–5 years
OR
• Chest indrawing
• Definite chest findings on
auscultation
DIAGNOSIS
 The diagnosis of pneumonia is clinical.
 Baseline investigations
 CBC (Leukocytosis)
 ESR and CRP (raise)

 Diagnostic investigation
 CXR
 Confirms the diagnosis of pneumonia and may indicate a
complication such as a pleural effusion or empyema.
 Viral pneumonia is usually characterized by:
 hyperinflation with bilateral interstitial infiltrates
and
 peribronchial cuffing.
 Confluent lobar consolidation is typically seen with
pneumococcal pneumonia.
 INDICATIONS:
 Severe disease, as defined above.

 Confirmation of the diagnosis when clinical findings are
inconclusive.
 Exclusion of alternate explanations for respiratory distress (eg,
foreign body aspiration, heart failure), particularly patients with
underlying cardiopulmonary or medical conditions.
 Assessment of the presence of complications, particularly in
children whose pneumonia is prolonged and unresponsive to
antimicrobial therapy
 Exclusion of pneumonia in:
 young children (3 to 36 months) with fever >39ºC and
leukocytosis (≥ 20,000 WBC/microL) and

 older children (<10 years) with fever >38ºC, cough, and
leukocytosis (≥ 15,000 WBC/microL)

 Pleural fluid analysis

Differentiation of pleural fluid
Transudate Exudate Complicated empyema
Appearance Clear Cloudy Purulent
Cell count <1000 >1000 >5000
Cell type Lymphocytes, PMNs PMNs
monocytes
LDH <200 U/L >200 U/L >1000 U/L
Pleural/serum <0.6 >0.6 >0.6
LDH ratio
Protein>3 g Unusual Common Common
Pleural/serum <0.5 >0.5 >0.5
protein ratio
Glucose* Normal Low Very low* (<40 mg/dL)
pH* Normal(7.40–7.60) 7.20–7.40 <7.20
Gram stain Usually >85% positive unless
Negative positive patient received prior
antibiotics
COMPLICATIONS OF PNEUMONIA
 Local complications
 Pleural effusion,
 Empyema and
 Lung abscess
 Pneumatoceles.
 Septic emboli in pulmonary veins
These complications are more common with staph and klebseila

pneumonia
 Systemic complications
 Septicaemia is the most common pneumonia complication
and occurs when the bacteria causing pneumonia spreads
into the blood stream
The spread of bacteria can lead to septic shock or

metastatic secondary infections like meningitis especially

in infants, peritonitis, and endocarditis especially in
patients with valvulvar heart disease or septic arthritis.
 Metastatic abscesses
MANAGEMENT PRINCIPLES
1, SUPPORTIVE CARE
 Bed rest.
 With severe respiratory distress- humidified O2 inhalation &
restricted I.V. fluids
 Symptomatic treatment e.g. antipyretics for fever.
 Treatment of complications e.g. Heart failure.
 Chest tube drainage for massive effusion or empyema.
2, ANTIBIOTIC THERAPY
 Bacterial pneumonia => Antibiotics Choice:

As suggested by clinical picture & chest X-ray.
According to culture & sensitivity if available.
Antibiotic combination if the cause cannot be detected.
Empirical therapy: For
 Mild cases: amoxicillin or cefuroxime or amoxicillin
clavulanate
 Hospitalized cases:
 Parenteral cefuroxime 75-150 mg/ kg/day
 Add vancomycin or clindamycin if staphylococci is
suspected
 Mycoplasma pneumonia: Erythromycin or
azithromycin or clarithromycin
 Viral pneumonia:
 Antibiotics may be used as coexisting bacterial infection
exists in 30% of cases
 Antiviral (for immunodeficient):
 Ribavirin for RSV.
 Amantidine for influenza A virus

ADMISSION CRITERIA:
 Age <6 mo
 Sickle cell anemia with acute chest syndrome
 Multiple lobe involvement
 Immuno compromised state
 Toxic appearance
 Severe respiratory distress
 Requirement for supplemental oxygen
 Dehydration
 Vomiting
 No response to appropriate oral antibiotic therapy
 Noncompliant parents

Sample history for pneumonia

C/C: cough of 4 days duration
HPI:
This is a 5 y.o male preschool who was relatively healthy 7 days
back at which time he started to develop sneezing and runny nose,
three days later he stated experiencing intermittent non barking
cough(to r/o croup) and fast breathing that begun insidiously. He
also has high grade intermittent fever. The following day shortness
of breath and grunting started to occur.
 He was fully vaccinated (being not vaccinated is a risk factor)
 He lives with his parent in a family 7 in a house with 2 rooms
having one door and 2 windows. (Overcrowding is a risk)
 Otherwise,
 He has no hx of chocking and gagging before the onset of the
cough; to r/o foreign body aspiration
 No hx of wheezing and family hx of asthma; to r/o asthma
 No hx of body swelling or sudden awakening from sleep; to r/o
congestive heart failure
 No hx of hoarseness of voice during cough; to r/o
tracheobronchitis
 No hx of ear discharge; to r/o otitis media
 No hx of red throat and difficulty of swallowing: to r/o
tonsillopharyngitis
 No hx of headache, loss of consciousness; to r/o complication –
meningitis
 No hx vomiting and diarrhea (systemic infections)

2. Foreign body aspiration

PATIENT APPROACH
HISTORY:
 Commonly in children 6 months to 3 years of age
 History of sudden chocking or frank history of foreign body
aspiration
 Triphasic history may be obtained:
 Initial phase: cough ,chocking, stridor or gagging
 Silent phase: if foreign body pass and impact in smaller
airways
 Phase of complications: recurrent pneumonia , abscess,
bronchiectasis
 SIGNS
 Fixed localized (unilateral) wheeze.

 Diminished breath sound over one lung, one lobe or one segment.
 Air trapping with hyper-resonance and mediastinal shift.
 Same site recurrent pneumonia, abscess, bronchiactasis.
 CHEST X-RAY
 Positive only in about 50°/o of cases

 There may be obstructive collapse or obstructive emphysema in
expiratory x ray film.
 Detect an area of hyper-inflation or collapse, mediastinal shift
(away from the affected side), or a foreign body if it is
radio-opaque.
 Treatment
 Emergency first aid for the choking child is mandatory. Attempt

to dislodge and expel the foreign body. The maneuver depends
on the age of the child. Approach to chronic Cough

Differential diagnosis for CHRONIC COUGH

 Respiratory system
 Tuberculosis
 Asthma
 Pertussis
 Foreign body aspiration
 Tracheobronchomalacia
 Dyskinetic cilia
 Cardio vascular system
 Congenital heart disease
 Gastrointestinal system
 Gastoesophageal reflux disease
 Systemic
 Measles
 Human immunodeficiency virus (PCP)
1. Tuberculosis
Definition
Tuberculosis is a chronic infectious disease caused by
Mycobacterium tuberculosis.
Etiology
 M. tuberculosis complex:- M. tuberculosis, (the most important
cause in humans), M. bovis, M. africanum, M. microti and M.
Canetti
 Mycobacterium tuberculosis
 Non-spore-forming, non-motile, Acid fast ,obligate aerobes
 Mycobacteria grow slowly, their generation time being 12–24 h
Epidemiology
 Currently, 95% of tuberculosis cases occur in developing
countries.
 Almost 1.3 million cases occur in children each year.
 More than ⅓ of the world's population is infected

 (WHO) estimates that >8 million new cases of tuberculosis occur

and approximately 3 million people die of the disease worldwide
each year
MODE OF TRANSMISSION
 Inhalation:- pulmonary tuberculosis

 Ingestion (raw milk):- intestinal Tuberculosis
 Wound contamination: - cutaneous tuberculosis
 Hematological spread form primary Tuberculosis focus
Pathogenesis
PRIMARY PULMONARY DISEASE
 Primary Focus (Ghon focus) at the site of first implantation

 Primary Complex: primary focus , hilar lymph nodes and
draining lymphatic’s
 Nonproductive cough and mild dyspnea are the most common
symptoms in infants
PROGRESSIVE PRIMARY PULMONARY DISEASE
 Primary focus enlarges steadily and develops a large caseous

center
 Significant signs or symptoms are frequent in locally
progressive disease in children.
REACTIVATION TUBERCULOSIS
 More common in those who acquire the initial infection above 7

year of age
 Usually remains localized to the lungs, because the established
immune response
 radiographic presentations are extensive infiltrates or

thick-walled cavities in the upper lobes
 It is highly contagious if there is significant sputum production
and cough
Risk factors for Tuberculosis Infection
Children exposed to high-risk adults:-
 HIV-infected adults with tuberculosis,

 homeless persons,
 persons who inject drugs,
 health care workers caring for high-risk patients
Risk factors for progression of latent tuberculosis infection to

tuberculosis diseases
 Infants and children less than 4 years of age,especially those less than 2
years of age
 Adolescents and young adults
 Persons co-infected with HIV
 Persons with skin test conversion in the past 1–2 year
 Persons who are immunocompromised

Time between initial infection and clinically apparent disease:

 Disseminated and meningeal tuberculosis are early
manifestations (2–6 months)
 Significant lymph node or endobronchial tuberculosis (within
3–9 months)
 Lesions of the bones and joints take several years to develop,
 Renal lesions (decades after infection)
 From 25 to 35% of children with tuberculosis develop extra
pulmonary manifestations compared with about 10% of
immunocompetent adults.
Clinical Manifestations
1. PULMONARY TUBERCULOSIS
 It involves:- cavities, upper lobe infiltrates, fibrosis
 Pulmonary TB is uncommon in childhood
 Most common radiographic presentation is extensive infiltrates
or thick walled cavities in the upper lobes
 Systemic manifestations (Night sweating, fever, loss of weight
& appetite than those with primary Pulmonary tuberculosis)
 Manifestations of hilar lymphadenopathy may include:

o Nonproductive brassy cough, face edema, dyspnea &
cyanosis
o Emphysema
o Lung collapse
 Manifestations of extension
o Bronchopneumonia.
o Pleural effusion.

o Miliary tuberculosis
2. EXTRA PULMONARY TUBERCULOSIS

 It involves: - pleural effusion, lymphadenopathy, meningitis,
pericarditis, intestinal tuberculosis
 MILIARY TUBERCULOSIS
 Common in infants, malnourished & immune compromised

persons
 Hematogenuous spread of Tuberculosis bacilli
 Multiple organ involvement (lung, kidneys, liver, spleen,
meninges).
 Insidious onset with early systemic manifestations (anorexia,
weight loss, and low-grade fever
 Choroid tubercles are highly specific for the diagnosis of
miliary tuberculosis.
 small miliary shadows (snow flake opacities) are seen on x ray
 TB LYMPHADENITIS
 TB of Superficial LNs (Scrofula) is most common form of EPTB

 Common sites are cervical lymph node , occasionally axillary
and groin LN may be involved
 Firm, discrete, non-tender and matted nodes
 Systemic symptoms (except fever) are rare
 Capsule will be ruptured & spread to adjacent nodes results in
draining sinus tract
 PERICARDIAL TB

 Hematogenous spread or due to rupture of a mediastinal

lymph node into the pericardium space.
 Dry pericarditis: Acute pain behind the sternum, pericardial
friction rubs
 Pericardial effusion: Breathlessness, fever, distant heart
sound, pulsus paradox us, raised JVP
 Constrictive pericarditis: The pericardium is thickened,
sometimes with calcifications preventing cardiac dilatation
 TUBERCULOSIS OF THE CENTRAL NERVOUS SYSTEM
Two types Tuberculosis: TB meningitis and Tuberculoma
A. TUBERCULOSIS MENINGITIS
 Lymphohematogenous dissemination of the primary infection.

 The brainstem is often the site of greatest involvement
CLINICAL FEATURE
 Rapid or slowly progressing

 Mostly slow progression having 3 stages;
Stage I (1-2wks)
 Non-specific symptoms (fever, headache, irritability,
malaise)
 Focal neurologic deficits are rare
 Stagnation or loss of developmental milestones
Stage II:
 Lethargy ,Nuchal rigidity ,Seizures
 Hypertonia ,Vomiting ,other signs of raised ICP

 Cranial nerve palsies(CN3 , 6 , 7)

 focal neurologic signs
Stage III:
 Coma
 Hemi-or paraplegia
 Hypertension
 Decerebratation /decortication
 Deterioration of vital signs
B.TUBERCULOMA
 Tumour like mass resulting from aggregation of caseous

tubercles
 In children most common infratentorially (base of the brain
near the cerebellum)
 The most common symptoms are: - headache, fever, and
convulsions
 BONE AND JOINT DISEASE
 The classic manifestation of tuberculous spondylitis is

progression to Potts disease, in which destruction of the
vertebral bodies leads to gibbus deformity and kyphosis.
 Involved bones :
 Vertebrae, Most common in lower thoracic (gibbus) and

lumbar spine
 knee,
 hip,

 elbow
 ABDOMINAL AND GASTROINTESTINAL DISEASE
 Oral cavity or pharynx is rare

 Esophageal Tb is rare (may be associated with traceesophageal
fistula)
 Intestinal tuberculosis is due to:-
o Ingested tuberculosis bacilli in milk
o Swallowed sputum from T.B lesions in the lungs
 Primary intestinal tuberculosis: commonly ileocaecal region
 Secondary intestinal tuberculosis: commonly in the terminal
ileum
 Tuberculosis enteritis:-
 Caused by hematogenous route or swallowing of bacilli
from their own lungs or ingestion of raw milk (M.bovis)
 Most common sites of involvement; ileum, jejunum &
appendix
 C/m: Pain, diarrhea/constipation, weight loss, fever due
to shallow ulcer
 Mesentric adenitis is common
 Enlarged nodes may cause intestinal obstruction or erode
through the omentum to cause generalized peritonitis
 Tuberculosis peritonitis:- due to Spread from intestinal or
genitourinary tuberculosis
 Diagnosis:

o Ultrasound: enlarged mesentric or

retroperitoneal lymph nodes
o Paracentesis: lymphocyte predominance
 GENITOURINARY DISEASES
 Renal tuberculosis is rare

 Renal tuberculosis is often clinically silent in its early stages,
marked only by sterile pyuria and microscopic hematuria
 As the disease progress:- dysuria, flank or abdominal pain,
and gross hematuria develops
HIV INFECTION AND RISK OF TB
 HIV increases susceptibility to infection with M. tuberculosis

 HIV increases the risk of progression of M. tuberculosis
infection to TB disease
 Tuberculosis in HIV-infected children is often more severe,
progressive, and likely to occur in extra pulmonary sites.
HIV status Life time risk of developing

TB
Negative 5-10%
Positive 50%
Approach to Patient
HISTORY
 Duration of the cough

 CHARACTERIZE THE COUGH

o Dry or productive cough? if productive (color, amount,

odor (offensive smell), position dependence)
o Associated with: whoop, post-tussive vomiting, inspiratory
stridor, expiratory wheeze
o Hacking, brassy, barking, paroxysmal or on-and- off type
 ASSOCIATED SYMPTOMS
o Weight loss, night sweats, fever, anorexia and failure to

thrive
 Risk factors
o Children exposed to high-risk adults (close contact with a
known TB patient)
o Homeless persons
o Immunocompromised persons(HIV)
o Steroids
o Housing condition
 Personal and family history of diabetes, hypertension, and
asthma
 IF EXTRA PULMONARY TB ASK
o Fever, headache, vomiting ,irritability (TB meningitis)

o Cough, Chest pain, hemoptysis (pleural TB)
o Shortness of breath, pain behind sternum (TB pericarditis)
o Abdominal pain, diarrhea and tenesmus (TB enteritis)
o Dysuria, hematuria and flank pain (genitourinary TB)
 ASK PREVIOUS HISTORY OF TB AND TB TREATMENT

 ASK COMPLICATIONS
Massive hemoptysis,
foul smelling sputum (bronchiectasis, lung abscess)
 GENERAL APPEARANCE
 Acutely sick looking /chronically sick looking

 Nutritional status (visible wasting)
 VITAL SIGN
 Tachycardia (secondary to fever, TB pericarditis)

 tachypnea
 febrile
 HEENT: Look for signs of anemia (Pale conjunctiva, Pale
buccal mucosa)
 LGS: firm, discrete, non-tender and matted lymph nodes
 CHEST EXAMINATION
 Inspection
 Chest lag on the affected site, kyphosis
 Palpation
 Tracheal deviation away from the affected site
 Asymmetrical chest expansion
 Decreased tactile fremitus on the affected site
 Percussion
 Stony dullness on the affected site
 Auscultation
 Absent/decreased air entry on the affected site

 Bronchial breath sound above the effusion
 CARDIOVASCULAR EXAMINATION
 Raised JVP
 Pericardium friction rub
 Distant heart sound
 ABDOMINAL EXAMINATION
 Abdominal tenderness
 Hepatomegaly, splenomegaly
 Hypoactive bowel sound (TB peritonitis)
 MUSCULOSKELETAL EXAMINATION
 Gibbus deformity
 CNS EXAMINATION
 Altered mental status (lethargy)

 Cranial nerve palsies (CN 3 , 6 , 7)
 Hypotonia
 Hemi or paraplegia
 Positive Meningeal signs

Tuberculin test
It detects delayed hypersensitivity reaction to tuberculosis protein
MANTOUX TEST- intradermal injection of 0.1 ml containing 5

tuberculin units of purified protein derivatives (PPD)
Interpretation: measure induration after 48-72 hours
Criteria for positive Tuberculin skin test

Reaction Population
1.Greater than 5-mm 1. close contact with active
induration tuberculosis patient
2. child having clinical or chest x ray
compatible with tuberculosis
3. immunodeficiency
2. Greater than 10 –mm 1. Children less than 4 year
induration 2.Birth or recent immigration from
endemic area
3.Exposure to people from endemic
area
4.Medical conditions with increased
risk(diabetes, renal diseases
3. Greater than 15-mm 1. person with no risk factor for
induration tuberculosis
2. children older than 4 years

False positive PPD

 Hypersensitivity to constituents
 Technical error
 Cross-sensitization to Antigens of NTM (usually below 10mm)
 Previous BCG vaccine causes reaction:
 If 2 BCG vaccinations given
 50% never develop the reaction
 Reactivity usually wanes in 2-3yrs
 If > 10mm , it is taken as positive
 Prior vaccination with BCG is never a contraindication to tuberculin
test
False negative PPD

 Very young age (less than 3 month)
 Severe PEM
 Infections (measles, mumps, varicella)
 Vaccination with live-viruse vaccines (within 6wks)
 Overwhelming TB
 Wrong technique
 HIV
 Steroids

Investigations
1. BACTERIOLOGICAL METHODS
 Microscopic examination
 The most specific confirmation is isolation of
M.tuberculosis
 collect sputum on Spot-spot with an interval of 30-60
minutes
 Early morning gastric aspirate (young age)
 Culture
 It is the gold standard
 Negative culture never exclude pulmonary TB
2. MOLECULAR TEST
 Gene Xpert
 It is a new and rapid DNA test for TB
 Indicated for diagnosis of TB in high MDR-TB and TB/HIV
 For diagnosis of TB in children and Extra pulmonary TB
 Line Probe Assay (LPA)
 Identify specific mutations on the genes of TB bacilli
responsible for Isoniazid and Rifampicin resistance
 It is rapid and accurate test to identify MDR-TB
3. HISTO-PATHOLOGICAL EXAMINATION
 PLEURAL Fluid Analysis

 Color: Yellow with blood tinged
 Chemical: Protein (2-4gm/dl, glucose (20-40 mg/dl)

 Cells: lymphocyte predominance

 CSF Analysis (TB meningitis)
 White Blood Cell(10-500mg/dl)
 Glucose less than 40mg/dl
 AFB and culture yield depends on volume (if 5-10ml:AFB is
positive in 30%, culture positive in 50-70%)
 Fine needle aspiration (TB lymphadenitis)
 Tissue biopsy (bronchial, pleural, pericardial, peritoneal,
liver tissue)
4. RADIOLOGIC EXAMINATION
 Chest X-ray
 Upper lobe infiltration
 Enlarged hilar and mediastinial lymph nodes and
opacification in lung tissue
 Cavitation (common with older children),pleural
effusion, emphysema, collapse
 Miliary mottling in lung tissue
 X-ray of bones
o Narrowing of disc space
o Collapse of vertebral body, Extensive destruction with
kyphosis (pott disease)
 CT/MRI
o Diffuse brain edema and lepto-meningeal inflammation,
meningeal enhancement (TB meningitis)

o Discrete lesions with significant surrounding edema and

ring enhancement (Tuberculoma)
DIAGNOSIS OF TUBERCULOSIS
 SUSPECTED TUBERCULOSIS
Any ill child with a history of contact with a confirmed case of

pulmonary tuberculosis or
Any child
o Not regaining normal health after measles or whooping cough

o With Loss of weight, cough and wheeze not responding to
antibiotic therapy
o With Painless swelling of the superficial lymph nodes
 PROBABLE TUBERCULOSIS
A suspected case and any of the following
o Positive more than 10mm induration

o Suggestive appearance on chest X-ray
o Suggestive histologic appearance of biopsy material
 CONFIRMED TUBERCULOSIS
o Detection by microscopy
or culture of tubercle bacilli
o Identification of tubercle bacilli mycobacterium tuberculosis by
culture

STANDARDIZED TB CASE DEFINITIONS
I. CASE DEFINITION BY SITE AND RESULT OF SPUTUM SMEAR FOR PTB
Smear positive case:
 At least 2 sputum smears positive for AFBs or

 1 sputum smear positive and X-ray abnormality consistent with
TB
Smear negative case
 At least 2 (preferably3) sputum smear negative for AFB and

 Chest X-ray consistent with TB
If AFB is negative, diagnosis is based on:

 Contact with patient(adult) with pulmonary TB
 Sign and symptom suggestive of TB
 X-ray finding consistent with TB
 Positive TST
If 3 are fulfilled, TB is likely diagnosis
If severe malnutrition or immunosuppression, 2 criteria are enough
II. CASE DEFINITION BY PREVIOUS TREATMENT
 New case (N): a patient who has never taken treatment for TB or
has been on anti-TB treatment for less than one month.
 Relapse(R): a patient who has been declared cured or treatment
completed of any form of TB in the past, but who reports back to
the health service and is found to be AFB smear-positive or
culture positive.

 Treatment failure(F): a patient who, while still on treatment

remains smear-positive or comes again sputum smear-positive 5
months or more after starting treatment
 Return after default (D): a patient who has previously been
recorded as defaulted from treatment (completed at least one
month of treatment and interrupted for at least 2 months) and
returns to the health service with smear-positive sputum.
 Transfer in (T): a patient registered for treatment in one district
and is transferred to another.
 Chronic case (C): a patient who remains smear-positive after
completing a supervised re-treatment regimen. This is usually
seen in adults and is rare in childhood but may occur in
adolescents.
 Other (O): a patient who does not easily fit into one of the above
case definition (e.g. a smear-negative PTB who returns after

default)
Principle of management
 Chemotherapy
 Nutritional rehabilitation
 Screening of the family
 follow up (Adherence, response, drug side effect)
PHASES OF CHEMOTHERAPY
 Intensive phase
 Reduces bacterial load( makes patient noninfectious)
 Prevent emergence of drug resistant strains

 At least 3 bactericidal drugs used for the first 8 weeks

 Continuation phase
 To ensure the patient is permanently cured and no relapse
after completion of treatment
 To clear dormant bacilli
 At least 2 bactericidal drugs used for 4 month (exceptions
are disseminated TB diseases and TB meningitis for which
the recommended duration is 9 to 12 months)
Anti tuberculosis drugs and their adverse reaction
Antituberculosis drugs Adverse reaction
Isoniazide Hepatitis
Peripheral neuropathy
Rifampicin Body secretion discoloration
Interference with oral contraception
Pyrazinamide Hepatitis
Hyperuricemia
Ethambutol Optic neuritis
decreased red-green color discrimination
Streptomycin Nephrotoxicity
Auditory and vestibular toxicity

Steroids are given for:

 TB Meningitis/ tuberculoma
 TB Pericarditis
 Adrenal TB
 Airway obstruction (LAP, laryngeal TB)
 Endobronchial TB with localized emphysema
 Spinal TB with neurologic deficit
FOLLOW UP
 Clinical improvement
 Radiologic improvement
 ESR
PREVENTION
 Case finding and treatment

 Searching for index case and treatment
 Isoniazid prophylaxis
 TB screening in infants and children who have contact with
adult case
 BCG vaccine

Sample history of TB
C/C: cough of two weeks duration
This is a two years old toddler boy who was relatively healthy two
weeks back at which time he started to develop cough. The cough is
nonproductive, persistent at any time of day and night, and it has no
any aggravating or relieving factor. Associated with the cough he has
history of low grade intermittent fever, loss of appetite and night
sweat. His mother claimed he has significant but unquantified weight
loss
His staple food is injera made of teff and wot made of lentils. He
sometimes eats fruit and vegetable
He lives in a house having 2 rooms, 1door, and 2windows with his

parents and 3 other siblings;2 older and one younger all of them are
healthier .The kitchen is separate from the house
Otherwise:-
 He has no history of contact with chronically coughing person

or previous TB treatment (risk factor)
 He has no history of shortness of breath, body swelling (CHF)
 He has no history of post tussive vomiting (pertussis)
 He has no history of food regurgitation or vomiting(GERD)
 He has no history of chocking (foreign body aspiration)
 He has no history of vomiting, diarrhea, abdominal pain
(intestinal TB )
 He has no history of pain during urination,
hematuria(Genitourinary TB)
 He has no history of headache, loss of consciousness
(meningitis)
 He has no personal and family history of diabetes,
hypertension, asthma (chronic disease)
 His mother does not know his serostatus (HIV)

Pertussis
Etiology
 Borditella pertussis and Borditella Para pertussis
 Route of infection- Droplet infection (mainly in child less than 5
year)
Clinical picture
1. CATARRHAL STAGE (1-2 WEEKS)
 Coryza (mucoid rhinorrhea),conjunctivitis, cough, mild fever

 The most infectious stage
2. PAROXYSMAL STAGE (4-6 weeks up to 10 weeks)
 Paroxysms of cough
 Series of more than 5 cough in single expiration followed by
a whoop
 During the attack; face redness, bulging eyes, tongue
protrusion, distended neck veins
 Posttussive vomiting is very common
 No abnormal signs on chest examination
 Usual paroxysm is usually absent in infants less than 2
months
 Triggers- attacks may be triggered by eating, drinking, exertion
3. CONVALESCENCE STAGE (1-2 weeks)
 Gradual decline in severity of paroxysms but cough may last for

months

Diagnosis
 CLINICAL
o Absent fever, exanthemes, sore throat, tachypnea, wheezes

or rales
o Cough more than 14 days with at least 1 paroxysm, whoop
or posttussive vomiting
o Apnea or cyanosis in infants less than 3 months
 Nasopharyngeal swab for
o Microscopic examination
o Culture on Regan- Lowe charcoal agar
o PCR
 CBC- leukocytosis with absolute lymphocytosis
 Chest x-rays show perihelia infiltration and atelectasis
 Confirmed by positive culture of B. pertussis
DIFFERENTIAL DIAGNOSIS
 Adenovirus infections- fever, sore throat and staccato cough,

conjunctivitis
 Chlamydia trachomatis infection- staccato cough, purulent
conjunctivitis .wheezes and rales
 Bronchial asthma- recurrent wheezy chest- related to allergens or
exercise-responds to bronchodilators
 Foreign body inhalation
 Pulmonary tuberculosis
 Mycoplasma pneumonia
 Suppurative lung diseases
Management principle
 Limit number of paroxysms
 Clearance of air ways and respiratory support if paroxysms are
life threatening

 Maximizing nutrition
 Adequate rest
 SUPPORTIVE CARE
 Small volume frequent feeds between cough episodes

 Suction air ways
 Oxygen if in distress
 ANTIBIOTICS
 Indicated for suspected or confirmed cases for potential

clinical benefit and to limit spread of infection
 Standard treatment is erythromycin
Admission criteria
 Infants below 3 months of age

 Witnessed severe paroxysms
 Children with significant underlying cardio
pulmonary or neuromuscular disorders
PROGNOSIS
 Young age(less than 2 months) and severe malnutrition are poor

prognostic factors
COMPLICATIONS
 RESPIRATORY
oOtitis media
oBronchopneumonia and pneumonia
oApnea and cyanotic attacks in infants less than 6 months
oAtelectasis and Bronchiectasis

 CONVULSION DUE TO-
o Brain damage (due to hypoxemia)

o Intra cranial hemorrhage
o Tetany
 MECHANICAL
oSubconjuctival hemorrhage
oEpistaxis and intracranial hemorrhage
oUlcer of tongue frenulum
oPneumothorax
 MALNUTRITION due to anorexia, vomiting, and faulty food
restriction
PREVENTION
 Primary prevention involves 3 doses of

pertussis(DPT)vaccine(EPI)
 Secondary prevention involves isolating patient and treating
them, vaccination and giving erythromycin for contacts

CHPTER 8
APPROACH TO WHEEZE
Epidemiology
 One in three children experience at least one acute wheezing
illness before the age of three years.
 A large worldwide study looking at older children showed a
global prevalence of wheezing of 11.6 percent in children
aged 6 to 7 years and 13.7 percent in children between 13 to
14 years of age.
DEFINITION
Wheeze is a musical continuous sound that originates in narrowed

airways heard on expiration as a result of airway obstruction.
MECHANISM
- Wheeze is produced by the oscillation of opposing walls of an

airway narrowed almost to the point of closure.
- It requires sufficient airflow to generate airway oscillation
and produce sound in addition to narrowing or compression of
the airway.
- It can occur during inspiration or expiration.
- It can originate from airways of any size throughout the
proximal conducting airways.

NB: Infants are more likely to wheeze than are older children, as a
result of:
- Airway size (small-caliber peripheral airways)

- Chest wall compliance is also quite high (Differences in tracheal
cartilage and airway smooth muscle tone, and inward pressure
produced in normal expiration) subjects the intrathoracic
airways to collapse
- Marginal additional narrowing, such as that caused by
inflammation, is then more likely to result in wheezing.
Differential diagnosis
 Wheezing can be divided clinically according to the acuity of its
onset, the mechanism of airway narrowing and age of onset of
wheezing.
 According to the mechanism of airway narrowing:
1. INFECTIONS: - viral: Respiratory syncytial virus, Human

metapneumovirus, Parainfluenza, Adenovirus, Influenza,
Rhinovirus, Bocavirus, Coronavirus, Enterovirus
Others: Chlamydia trachomatis, Tuberculosis, Histoplasmosis,
Papillomatosis, pneumonia
2. ASTHMA
3. ANATOMIC ABNORMALITIES:
- Central Airway Abnormalities

Malacia of the larynx, trachea, and/or bronchi, Laryngeal or
tracheal web, Tracheoesophageal fistula (specifically H-type
fistula), Laryngeal cleft (resulting in aspiration)

- Extrinsic Airway Anomalies Resulting in Airway Compression

Vascular ring or sling, Mediastinal lymphadenopathy from
infection or tumor, Mediastinal mass or tumor, Esophageal
foreign body
- Intrinsic Airway Anomalies
Airway hemangioma, other tumor, Congenital pulmonary airway
malformation (cystic adenomatoid malformation), Bronchial or
lung cyst, Congenital lobar emphysema, Aberrant tracheal
bronchus, Sequestration, Congenital heart disease with
left-to-right shunt (increased pulmonary edema), Foreign body
- Immunodeficiency States
Immunoglobulin A deficiency, B-cell deficiencies, AIDS,
Bronchiectasis
4. MUCOCILIARY CLEARANCE DISORDERS
Cystic fibrosis, Primary ciliary dyskinesia, Bronchiectasis
5. ASPIRATION SYNDROMES
Gastroesophageal reflux disease, Pharyngeal/swallow
dysfunction
6. OTHERS
Bronchopulmonary dysplasia, Eosinophilic granulomatosis
with polyangiitis, Interstitial lung disease, including
bronchiolitis obliterans, Heart failure, Anaphylaxis, Inhalation
injury—burns

 According to acuity:
Acute (hours to days) FUNCTIONAL ABNORMALITIES

Chronic or recurrent
 Asthma
 Structural abnormalities  Asthma
 Bronchiolitis*
 Tracheo-bronchomalacia*  Gastroesophageal reflux
 Bronchitis
 Vascular  Recurrent aspiration
 Laryngotracheobronc
compression/rings*  Cystic fibrosis
hitis¶
 Tracheal stenosis/webs*  Immunodeficiency
 Bacterial tracheitis
 Tumors/lymphadenopath  Primary ciliary dyskinesia
 Foreign body
y  Bronchopulmonary dysplasia
aspiration¶
 Cardiomegaly  Retained foreign body (trachea
 Esophageal foreign
 Vocal cord dysfunction Δ or esophagus)
body
 Bronchiolitis obliterans
Pulmonary edema
 The age of onset of wheezing can also help determine the

etiology: …… (*up-to-date)
* - These disorders tend to present in infancy.
¶ - These disorders are more commonly seen in young children
(toddlers and preschoolers).
Δ - These disorders are more commonly seen in teenagers.
Approach to Wheezing child

1. TAKE A HISTORY
1st characterize the chief complaint (if wheeze)
2nd think of your differential diagnoses
3rd ask the associated symptoms, risk factors, complications of your

top differentials
4th write your HPI

2. DO PHYSICAL EXAMINATION
3. ORDER AN INVESTIGATION FOR YOUR SUSPECTED DIAGNOSIS
4. TREAT THE PATIENT
Clinical history
1. CHARACTERIZE WHEEZE
 https://www.youtube.com/watch?v=T4qNgi4Vrvo …
hear the sound
 IS IT WHEEZING? — What they actually are experiencing or
hearing
(The word "wheezing" is used as a general term to describe noisy
breathing that is primarily due to upper airway noises, including
snoring, congestion, rattling, gurgling noises, or stridor)
 AGE AT ONSET —
 since birth - a congenital abnormality
 During infancy and early childhood - Structural
abnormalities, virus-induced wheezing and foreign body
aspiration (FBA) are seen.
 in later childhood to adolescence - Other disorders,
such as vocal cord dysfunction
 SPEED OF ONSET AND PERSISTENCE — whether the onset of

wheezing was acute or gradual and whether the wheezing is
intermittent or persistent.
 Acute onset or Persistent wheezing with sudden onset -
FBA (particularly if there is a history of choking)
 Slowly progressive onset - extra luminal bronchial
compression by a growing mass or lymph node.
 Persistent wheezing - a congenital or structural
abnormality (particularly if presenting very early in life)

 Paroxysmal or Intermittent wheezing – Asthma

(particularly if it has triggering history like; upper
respiratory infections, weather changes, exercise, or
allergens and has seasonal variation)
 GOOD RESPONSE TO BRONCHODILATORS - Asthma. It can also be

seen with other conditions that may lead to inflammation and
bronchoconstriction, such as bronchopulmonary dysplasia
[BPD], cystic fibrosis [CF], and aspiration.
2. ASSOCIATED SYMPTOMS
 COUGH – characterize it:

 Wet cough - mostly due to infection or inflammation
 Dry cough – due to pure bronchoconstriction or structural
causes (eg, airway malacia or compression, foreign body,
vascular ring).
NB: Asthma - either a dry or wet cough depending upon the degree of
airway obstruction and the amount of mucus produced (mucus
production can vary from one patient to another and can vary at
different times in the same patient).
 Shortness of breath and chest tightness (if older children) -

Asthma
 Sneezing and clear rhinorrhea (nasal discharge) -
bronchiolitis
 Fever – infections (bronchiolitis, pneumonia…)
 Regurgitation or vomiting – GERD
 Choking – FBA
 Difficulty of swallowing – swallowing disorder, esophageal
foreign body

 Dysphonia (unable to make a sound), throat tightness,

choking sensation – vocal cord dysfunction
 Poor weight gain – immunodeficiency, ciliary diseases, cystic
fibrosis
 Stridor – malacia, vascular ring, VCD
Symptoms vary with position change – malacia or vascular rings
Symptoms (shortness of breath, cough or wheeze) worsen at

night-time or early morning. – Asthma
3. PAST MEDICAL AND SURGICAL HISTORY
 Allergy history of:

 Eczema
 Persistent rhinitis (apart from acute URTI)
 To allergens like food, dusts, pollen.
 Medical history:
 Recurrent ear infection (ear discharge), upper and lower
respiratory tract infections (nasal discharge, sneezing) – CF,
immunodeficiency, ciliary dyskinetic ds
 Neurologic disease – swallowing disorders
 Any comorbid conditions like Congenital heart disease _
(cardiac wheeze)
 Asthma diagnosis or long-term treatment for asthma
 Surgical history – lung transplantation and hematopoietic cell

transplantation – Bronchiolitis obliterans (rare disease)
4. PERSONAL, FAMILY AND SOCIAL HISTORY
 Family history of:

 Asthma and atopy/allergy in 1st degree relatives:

o Paternal asthma is a risk factor for persistent wheeze in

boys
o Maternal asthma was a risk factor for persistent wheeze in
girls and boys
 Cystic fibrosis
 Immunodeficiency
 Social history:
 Second hand tobacco and other smokes exposure – RF for
bronchiolitis + asthma
 Living room not separated from kitchen (coal smoke
exposure) – RF for bronchiolitis + asthma
 Day care exposure – RF for bronchiolitis
 Number of siblings or older family members (siblings) with
even minor URTI (cold) – RF for bronchiolitis
 Crowded living conditions – RF for bronchiolitis +
pneumonia
 Pets in the house – trigger for Asthma
 Home environment – dust mite, construction dust, heating
and cooling technique, mold, cockroaches – trigger for
Asthma
 Any lapse in supervision of the child – RF for FBA
5. IMMUNIZATION HISTORY – if unvaccinated – RF for pneumonia and
bronchiolitis
6. NUTRITIONAL HISTORY
 bottle feed in bed or crib especially in propped position – RF

for GER
 not breastfed – RF for bronchiolitis
7. DEVELOPMENTAL HISTORY – if failure to thrive - immunodeficiency,
ciliary diseases, cystic fibrosis
8. BIRTH HISTORY
 Gestational age at delivery – if preterm (BW <1250g) –

bronchopulmonary dysplasia
 Neonatal complications: respiratory problems since birth or

intubated, hospitalized, admitted to ICU for respiratory
distress – congenital abnormalities or bronchopulmonary
dysplasia
 Mother smoked during pregnancy – bronchiolitis, Asthma
 Maternal complications
 It should include head to toe:
 General Appearance - acute sick looking (RD)
 vital signs including oxygen saturation
 measurement of weight and height,
 complete lung and chest examination, and
 digital inspection for the presence of cyanosis or
clubbing.
1. CHEST EXAMINATION
 INSPECTION
- respiratory distress: retractions, nasal flaring, Lower chest

indrawing (if severe)
- tachypnea
- wheezing on expiration
- prolonged expiration
- structural abnormalities:
 increased anteroposterior (AP) diameter - chronic
hyperinflation
 pectus excavatum - chronic airway obstruction and
exaggerated swings in intrathoracic pressure
 scoliosis complicated by airway compression.
 PALPATION - tracheal deviation – mediastinal mass, supratracheal

lymphadenopathy

 PERCUSSION
- Define the position of the diaphragm – if hyperinflation

- Hyper resonance
 AUSCULTATION
- Identify location of wheezing: if focal wheezing - a localized and

mostly structural airway abnormality and FBA
- Decreased breath sounds - nearly complete bronchiolar
obstruction
- prolonged expiratory phase
- Crackles in conjunction with wheezing - in asthma, pneumonia
and in those leading to bronchiectasis (eg, CF, primary ciliary
dyskinesia, immune deficiency).
 Early inspiratory crackles - asthma (due to air flowing
through secretions or slightly closed airways during
inspiration)
 Late inspiratory crackles - ILD and early congestive
heart failure.
NB: Thus, the presence of crackles does not exclude the

diagnosis of asthma
NB: The lack of audible wheezing is not reassuring if the infant shows
other signs of respiratory distress, since complete obstruction to
airflow can eliminate the turbulence that causes wheezing.
2. HEENT: Nasal examination - for signs of allergic rhinitis,

sinusitis, or nasal polyps (indicates cystic fibrosis)
3. The cardiac examination: auscultation for murmurs and
evaluation for signs of heart failure - CHD
4. Skin Examination - for eczema (common in atopic patients) or
other cutaneous lesions

Investigation
In most cases, the probable diagnosis is suspected on the basis of

the clinical history and physical examination.
TO CONFIRM THE DIAGNOSIS:
1. RESPONSE TO TREATMENT
For patients suspected of having asthma, a trial of inhaled

bronchodilators with or without glucocorticoids can be used to
confirm the diagnosis prior to initiating a more extensive work-up.
NB: A partial or negative response to bronchodilators only may

not rule out asthma, because Inflammation and airway swelling
may contribute to wheezing, in addition to bronchoconstriction,
especially in infants and young children. Thus, the combination of
inhaled glucocorticoids and bronchodilators for at least two
weeks (or five to seven days of oral glucocorticoids if the patient has
more severe symptoms) may result in significant improvement in
symptoms and help in making the diagnosis of asthma.
Further work-up is indicated if the response to therapy is inadequate:
2. RADIOGRAPHY —
 A chest radiograph (AP and lateral films)

Indications:
 new-onset wheezing of undetermined etiology or

 chronic, persistent wheezing not responding to therapies
NB: It is not necessary to obtain a chest radiograph with every
exacerbation in children with asthma, unless there is a specific
indication.
CXR is important to:

 To rule out pneumonia, atelectasis, and air leak,

o if there are focal examination findings (eg, crackles or decreased
breath sounds), fever (>39ºC), severe disease, uncertainty
about the diagnosis, or tachypnea, hypoxemia, or chest pain
that are present after initial therapy has been given
 To differentiate between diffuse and focal disease:

o Diffuse - asthma, CF, primary ciliary dyskinesia, and
aspiration.
o Focal - structural abnormalities or FBA.
 To reveal cardiomegaly, enlarged pulmonary vessels, pulmonary

edema, or other signs of cardiac failure.
 To detect mediastinal masses or enlarged lymph nodes and may

suggest the presence of vascular rings (eg, right aortic arch)
Other radiologic studies:
 CT scan - provide detailed anatomy of the mediastinum, large

airways, and lung parenchyma.
 MRI with contrast - the study of choice when a vascular ring or
sling is suspected.
 Barium swallow - done when vascular rings, swallowing
dysfunction, aspiration syndromes including GER, and some cases
of tracheoesophageal fistula suspected.Pulmonary function tests
o Infant PFT, if available, can help quantify airway
obstruction and the response to bronchodilators
o In older children (who are cooperative), PFT with inspiratory
and expiratory flow-volume loops is helpful in determining
the presence, degree, and location of airway obstruction,
and response to bronchodilators
Schematic flow-volume loop configuration in a spectrum of airway
lesions

(A) Normal.
(B) Variable extrathoracic upper airway obstruction.
(C) Variable intrathoracic upper airway lesions.
(D) Fixed upper airway obstruction.
(E) Lower airways obstruction.
NB: Any studies obtained beyond chest radiographs and PFTs

should be based upon the suspected diagnosis.

Summary to Approach to evaluation of wheezing in children

based upon suspected diagnosis
Suspected Signs and symptoms Diagnostic evaluation

diagnosis
Acute
Asthma History of recurrent History, PFT with
wheeze, cough, at least bronchodilators, empiric trial of
partial response to bronchodilators, exercise or
bronchodilator methacholine challenge testing,
chest radiography only if atypical,
skin (or in vitro) testing for
aeroallergen sensitization if
history suggests inhalant allergen
triggers
Viral Prodrome with rhinitis, History, age, season, rapid
bronchiolitis occurs in infancy and antigen testing (RSV, influenza),
early childhood, seasonal viral cultures, chest radiography
pattern
Foreign body Sudden onset of coughing History, physical examination,
and wheezing chest radiography, bronchoscopy
Chronic
Asthma As above As above
Tracheomalaci Persistent wheeze, starts History, fluoroscopy, flexible
a early in life, poor response bronchoscopy
to bronchodilators, varies
with position and activity
Cystic fibrosis Chronic productive Sweat chloride test, genetic
cough, crackles, with or testing
without clubbing, failure
to thrive, recurrent
respiratory infections
Swallowing Neurologic abnormality Videofluoroscopic swallowing
dysfunction (nonuniversal), choking study (modified barium swallow)
with eating, symptoms
exaggerated by feeding
Gastroesophag Symptoms sometimes 24-hour esophageal pH

eal reflux related to eating, monitoring, multichannel

vomiting, refusal to eat, intraluminal impedance
failure to thrive monitoring
Vascular ring or Persistent symptoms, Barium swallow, MRI
sling starts early in infancy,
may be exaggerated by
position, homophonous
wheeze
Tracheal Persistent symptoms, Chest radiograph, CT scan,
stenosis with or without stridor, bronchoscopy
homophonous wheeze
Mediastinal Persistent symptoms, Chest radiograph, CT scan
nodes or mass localized wheezing, no
response to
bronchodilator, systemic
symptoms of underlying
disease
Immunodeficie Recurrent sinopulmonary Immunoglobulins, vaccine
ncy infections, crackles, FTT, responses
clubbing
Primary ciliary Persistent sinusitis and Ciliary biopsy, with or without
dyskinesia otitis media with draining genetic testing
ears, recurrent
respiratory infection, wet
cough with sputum
production, crackles,
clubbing, FTT
Vocal cord Inspiratory stridor, poor Exercise testing, pulmonary
dysfunction response to function tests, laryngoscopy
bronchodilators, absent while symptomatic
symptoms during sleep,
teenage, exercise related
Bronchiolitis History of predisposing Chest CT scan, lung biopsy
obliterans disease, ie, viral infection
or transplantation,
dyspnea, persistent
wheezing

CHILDHOOD ASTHMA
Definition
ASTHMA is a chronic inflammatory condition of the lung airways
resulting in episodic airflow obstruction. (Which is reversible
obstruction
ETIOLOGY AND PATHOGENESIS

ASTHMA TRIGGERS
1. COMMON VIRAL • Dust

INFECTIONS OF 4. STRONG OR NOXIOUS
RESPIRATORY TRACT ODORS OR FUMES
2. AEROALLERGENS IN • Perfumes, hairsprays
SENSITIZED ASTHMATIC • Cleaning agents
PATIENTS 5. OCCUPATIONAL
Indoor Allergens EXPOSURES
• Animal dander • Farm and barn exposures
• Dust mites • Formaldehydes, cedar, paint
• Cockroaches fumes
• Molds 6. COLD DRY AIR
Seasonal Aeroallergens 7. EXERCISE
• Pollens (trees, grasses, weeds) 8. CRYING, LAUGHTER,
• Seasonal molds HYPERVENTILATION
3. AIR POLLUTANTS 9. COMORBID CONDITIONS
• Environmental tobacco smoke • Rhinitis
• Ozone • Sinusitis
• Nitrogen dioxide • Gastroesophageal reflux
• Sulfur dioxide 10. DRUGS
• Particulate matter • Aspirin and other
• Wood- or coal-burning smoke nonsteroidal antiinflammatory
• Mycotoxins drugs
• Endotoxin • β-Blocking agent
Epidemiology
 Childhood asthma is among the most common causes of
childhood emergency department visits, hospitalizations, and
missed school days.
 Worldwide, childhood asthma appears to be increasing in
prevalence.

 Asthma is more prevalent in

Asthma prevalence is
modern metropolitan locales
correlated well with:
and more affluent nations.
 allergic
 In contrast, children living in
rhinoconjunctiviti
rural areas of developing
s and
countries and farming
 atopic eczema
communities with domestic
prevalence
animals are less likely to
experience asthma and allergy.
Types and patterns of Asthma

Four patterns of infant wheezing:
1. NEVER WHEEZERS (51%) – Healthy children who never wheezed

2. EARLY, TRANSIENT WHEEZERS (20%) – began wheezing before 3
years of age and resolved by 6 years of age
 characterized by lower lung function at birth which
improves with growth resulting in resolution of wheezing
by age 3
 primarily triggered by common respiratory viral
infections
3. PERSISTENT WHEEZERS (14%) – began wheezing before 3 years of
age and was still present at 6 years of age
 characterized by declining lung function and wheezing
before and after age 3
 Associated with atopy
 Clinical (e.g., atopic dermatitis in infancy, allergic
rhinitis, food allergy)
 Biologic (e.g., early inhalant allergen sensitization,
increased serum IgE, increased blood eosinophils)

4. LATE-ONSET WHEEZERS (15%) – develop wheezing between 3 and

6 years of age
 characterized by relatively stable lung function and
wheezing that does not begin until after age 3.
NB: Children in the persistent wheezing and late-onset wheezing

groups are at increased risk for persistent asthma-like symptoms into
adolescence and adulthood.
Asthma prediction
 Early Childhood Risk Factors for Persistent Asthma include:
Parental asthma* Wheezing apart from colds

Allergy: Male gender
• Atopic dermatitis (eczema)* Low birthweight
• Allergic rhinitis Environmental tobacco
• Food allergy smoke exposure
• Inhalant allergen Reduced lung function at
sensitization* birth
• Food allergen sensitization Formula feeding rather than
Severe lower respiratory tract breastfeeding
infection:
Asthma at age of 7-10 yr
• Pneumonia
• Bronchiolitis requiring Moderate to severe asthma
hospitalization
*indicates – Major risk factors
Asthma Predictive Index (API)

 It identifies those children who will continue wheezing into

older childhood.
 It was derived from children who had wheezed at least once
during the first three years of life.
The major criteria The minor criteria
 Clinician-diagnosed  Clinician-diagnosed
eczema allergic rhinitis,
 Parental asthma  Wheezing apart from
 Inhalant allergen colds,
sensitization (this criteria  ≥4% peripheral blood
is added on nelson) eosinophil
 Food allergen
sensitization
A positive loose index: any parental report of wheezing at two or

three years of age and either one major criteria or two minor
criteria.
 four times more likely to have active asthma at 6, 8, 11, or 13

years of age
A positive stringent index: frequent wheezing (score of ≥3, scale: 1
to 5, from "very rarely" to "on most days") plus the same combination
of major or minor criteria.
 seven times more likely to have active asthma at 6, 8, 11, or 13

years of age
NB: The sensitivity of the API index is low (15 to 57 percent). However,
the API has a high negative predictive value

Approach
HISTORY
1. SYMPTOMS
 Intermittent dry coughing

 expiratory wheezing

 shortness of breath
in older children and adults
 Chest congestion and tightne
 intermittent, nonfocal chest pain – in younger children
 self-imposed limitation of physical activities,
 general fatigue (possibly resulting from sleep disturbance)
 Difficulty keeping up with peers in physical activities.
Worse at night or sleep - especially during prolonged exacerbations

triggered by respiratory infections or inhalant allergens. Daytime
symptoms - linked with physical activities or play
2. Ask the common triggers and risk factors of persistent asthma
 Expiratory wheezing
 Prolonged exhalation phase
 Decreased breath sounds (commonly the right lower
posterior lung field - regional hypoventilation caused by
airways obstruction)
 Rhonchi and crackles (excess mucus production and
inflammatory exudate)
 Fast breathing
 Hypoxia (oxygen saturation < 90%)
 absence of fever
 hyperinflation of the chest
 In severe exacerbations (the greater extent of airways

obstruction):
 inspiratory and expiratory wheezing
 increased prolongation of exhalation
 poor air entry
 suprasternal and intercostal retractions
 nasal flaring
 Accessory respiratory muscle use
 silent chest (no wheezing heard) – no air entry by

complete obstruction
If the diagnosis is uncertain, quick resolution (within 10 min) or

convincing improvement in symptoms and signs of asthma with
administration of an inhaled short-acting β-agonist (SABA -
albuterol) is supportive of the diagnosis of asthma.
Investigations
1. Lung function tests – can help to confirm the diagnosis of
asthma and to determine disease severity.
2. Exhaled nitric oxide – helps diagnose asthma, assess asthma
control and adherence with ICS
therapy, predict response to ICS therapy, and predict future
asthma exacerbations.
3. Radiology - hyperinflation and peribronchial thickening and
identify asthma mimics and complications of asthma.
4. Allergy testing to assess sensitization to inhalant allergens

Lung Function Abnormalities in Asthma:
 Spirometry
Airflow limitation:
• Low FEV1 (relative to percentage of predicted norms)
• FEV1 /FVC ratio <0.80
 Bronchodilator response assesses reversibility of airflow
limitation.
Reversibile – if an increase in either FEV1 >12% or predicted
FEV1 >10% after inhalation SABA*
 Exercise challenge:
• Worsening in FEV1 ≥15%*
 Daily peak expiratory flow (PEF) or FEV1 monitoring:
day-to-day and/or AM -to-PM variation ≥20%*
 Exhaled nitric oxide (FeNO)
• A value of >20 ppb – diagnose asthma
FeNO can be used to predict response to ICS therapy:
• <20 ppb: Unlikely to respond to ICS because eosinophilic
inflammation unlikely
• 20-35 ppb: Intermediate, may respond to ICS
• >35 ppb: Likely to respond to ICS because eosinophilic
inflammation is likely
NB: * - means Main criteria consistent with asthma.

Asthma masqueraders (common DDx of Childhood Asthma)
 Upper Respiratory Tract Conditions

Allergic rhinitis
Chronic rhinitis
Sinusitis
 Middle Respiratory Tract Conditions
Laryngotracheobronchomalacia
Laryngotracheobronchitis (e.g., pertussis)
Vocal cord dysfunction
Foreign body aspiration
Chronic bronchitis from environmental tobacco smoke
exposure
 Lower Respiratory Tract Conditions
Viral bronchiolitis
Gastroesophageal reflux
Treatment
The key components to optimal asthma management are:
(1)Assessment and monitoring of disease activity;

 Assess asthma severity
 Monitor control
(2)Education to enhance patient and family knowledge and skills
for self-management
(3)Identification and management of precipitating factors and
comorbid conditions
that worsen asthma; and
(4)Appropriate selection of medications to address the patient's
needs.
(5)Management of acute exacerbations
The long-term goal of asthma management is optimal asthma
control.

Asthma severity classification

Mild Asthma Moderate Asthma Severe Asthma
 Normal mental  Normal to  Agitated or

state agitated confused
 Talks in  Talks in phrases  Talk in single
sentences  Mild to word or unable
 Little or no moderate to talk
accessory accessory  Significant
muscle use muscle accessory
 SPO2>95% use muscle use
 Wheeze +  SPO2 90-95%  SPO2<90%
normal  Wheeze +  Wheeze
breath sounds reduced +significantly
 PR <100 breath sounds reduce
 PR 100-120 breath sounds
PR >120

Classification of Asthma severity (for children aged 0 – 4 yrs)

Components of
Intermittent Persistent
severity
Mild Moderate Severe
>2 days / wk
Symptoms ≤ 2 days / wk Daily Throughout the day
(but not daily)
Night time 1 – 2 times/ 3 – 4 times/

0 >1 times / week
awakening month month
SABA use for >2 days / wk

≤ 2 days / wk Daily Several times / day
symptom control (but not daily)
Interference with Some

None Minor limitation Extremely limited
normal activity limitation
Exacerbations  ≥ 2 in 6 months, or
requiring oral 0 – 1 / year  ≥ 4 wheezing episode lasting > 1 day PLUS risk
systemic steriod factor for persistent asthma
Classification of Asthma severity (for children aged 5 - 11

yrs)
Components of
severity Intermittent Persistent
Mild Moderate Severe
>2 days / wk Throughout the

Symptoms ≤ 2 days / wk Daily
(but not daily) day
> 1 times
Night time ≤ 2 days/ 3 – 4 times/
/week (but 7 times /week
awakening month month
not nightly)
SABA use for >2 days / wk Several times /

≤ 2 days / wk Daily
symptom control (but not daily) day
Interference with Minor Some

None Extremely limited
normal activity limitation limitation

FEV1 = 60 - 80%
FEV1 > 80% FEV1 ≥ 80% FEV1 < 60%
Lung function FEV1/FVC = 75
FEV1/FVC >85% FEV1/FVC >80% FEV1/FVC < 75%
-80%
Exacerbations
requiring oral 0 – 1 / year ≥ 2 in 1 year
systemic steriod
Asthma management types
Control Classification
Clinical assessment while asthma being managed and treated
 Well controlled
 Not well controlled
 Very poorly controlled
Management Patterns
 Easy-to-control: well controlled with low levels of daily

controller therapy
 Difficult-to-control: well controlled with multiple and/or high
levels of controller therapies
 Exacerbators: despite being well controlled, continue to have
severe exacerbations
 Refractory: continue to have poorly controlled asthma despite
multiple and high levels of controller therapies
ASTHMA EXACERBATIONS AND THEIR MANAGEMENT
 Asthma exacerbations are acute or sub-acute episodes of

progressively worsening symptoms and airflow obstruction.

 It often worsen during sleep (between midnight and 8 AM )

 Its complications include:
oatelectasis (common)
opneumomediastinum and pneumothorax (rare)
Status asthmaticus is a severe exacerbation of asthma that does not

improve with standard therapy.
TREATMENT OF EXACERBATIONS:
 Assessment of severity and risk factor of asthma morbidity

and mortality
 Oxygen
 Systemic medical treatment
PROGNOSIS
 Recurrent coughing and wheezing occurs in 35% of

preschool-age children.
 One third of them continue to have persistent asthma into
later childhood,
 and two thirds improve on their own through their teen years.
PREVENTION
 Avoidance of environmental tobacco smoke (beginning

prenatally),
 prolonged breastfeeding (>4 mo),
 an active lifestyle, and
 a healthy diet
 A hygiene hypothesis purports that naturally occurring
microbial exposures (in rural areas) in early life might drive
early immune development away from allergic sensitization.

Sample History of Asthma

ID: This is _________ a 5 years old boy from jimma town (asthma
more common in urban town) currently attending lower KG at
school.
C/C: cough and wheeze of 3 days duration
HPI:
He was relatively healthy 3 days back at which time he started to

experience an intermittent dry cough which worsens at night time
and sometimes early in the morning but it is not associated with
feeding (r/o: Gastro esophageal reflux). His mother also noticed a
noisy breathing like a wheeze which is not associated with runny
nose, sneezing and nasal stiffness (r/o: common cold). She denies
that he has any prior wheezing episodes.
Associated with this, he also has history of difficulty of breathing and

difficulty of keeping up with his friends at school during different
physical activities and playing. For these reasons, he discontinued to
attend school for 2 days.
Yesterday, his symptoms worsened during day time while playing

and jumping around the house with his brother and had a difficulty
of catching his breath and continuous cough. Then his mother
rushed him to this hospital emergency department and he was given
a drug which is inhaled through his mouth. After few minutes he
became well.
He has a past history of food allergy for peanut butter and a skin
allergy.
He is fully vaccinated. (RF for pneumonia, some etiology of acute

bronchiolitis).

He was first exclusively breastfed for 6 months and had been

breastfeeding for 2 years. (RF for acute bronchiolitis).
He lives in a total family size of 5. He has one big brother aged 8 years,
one younger sister aged 3 years, and his both parent. All of them are
healthy. They live in a house with 3 rooms, 2 of them have a single
window in each while the other has 2 Windows and 2 doors which is
separated from the kitchen and toilet. They have no any animal in the
house. (RF for acute bronchiolitis, pneumonia).
He started to sit unsupported at age of 8 months and walk steadily

unsupported at age of 1 and half years. At age of 4, he started to run
up and downstairs, to dress and undress himself without help and to
speak fully understandable sentences. (if failure to thrive- CF,
immunodeficiency).
He was born by spontaneous vaginal delivery as his other siblings at

gestational age of 39 weeks with a birth weight of 2.5 kg with no
neonatal and maternal complications. (congenital abnormalities
or bronchopulmonary dysplasia)
Otherwise,
He has no history of previous asthma diagnosis and treatment
He has no history of second hand tobacco and other smoke exposure.

(Bronchiolitis, asthma)
He has no history of clear nasal discharge, sneezing (Bronchiolitis,

transient asthma, pneumonia)
He has no history of fever. (Bronchiolitis, pneumonia)
He has no history of choking (foreign body aspiration)
He has no history of difficulty of swallowing and any neurogenic

disease. (Swallowing disorders)

He has no history of recurrent upper respiratory tract infection and

poor weight gain. (Cystic fibrosis, immunodeficiency, ciliary
kinetic disorders)
He has no history of food regurgitation or vomiting

(Gastroesophageal reflux)
He has no history of difficulty of making a sound and throat

thightness. (Vocal cod dysfunction)
He has no history of prior surgery like lung transplantation.

(Bronchiolitis obliterans)
He has no personal and family history of cardiac disease (r/o

congenital heart disease), asthma, cystic fibrosis,
immunodeficiency, TB, renal disease and RVI

2. ACUTE BRONCHIOLITIS
Definition:
is defined as a clinical syndrome that occurs in children <2 years of
age and is characterized by upper respiratory symptoms (eg,
rhinorrhea) followed by lower respiratory infection with inflammation,
which results in wheezing and/or crackles (rales).
Etiology
 Respiratory syncytial virus (RSV) - 50% of cases
 human metapneumovirus,
 rhinovirus,
 parainfluenza,
 influenza,
 corona virus
 bocavirus, and
 adenovirus.
Epidemiology
 It is a leading cause of hospitalization in infants and young
children.
 In tropical and semitropical climates, seasonal outbreaks of
RSV usually are associated with the rainy season.
Risk factors
 Prematurity (gestational age ≤36 weeks) and Low birth weight
 Age less than 12 or 24 months (more severe disease if <12
weeks)
 Not breastfeed
 passive smoking,
 mother smoking during pregnancy
 crowded household,

 daycare attendance,
 being born approximately two months before or after the start of
the epidemic,
 concurrent birth siblings or older siblings
 high altitude (>2500 meters)
 Males
Clinical course
Respiratory distress ensues,

paroxysmal dry cough,
dyspnea, and irritability.
Exposure/contacts
Sneezing and Diminished Tachypnea, (which can
with a minor
respiratory illness clear appetite
(within the Rhinorrhea and fever
previous week)
Apnea (particularly if: very
young infants, Term infants at
a postconceptual age of <44
wk and preterm
infants at postconceptual age
<48 wk)
 respiratory rate and oxygen saturation (is an important initial
step)
 wheezing and crackles (dominant finding)
(If no wheeze - Complete obstruction to airflow) it doesn’t R/O the
Dx
 prolonged expiration
 Increased Work of breathing - nasal flaring and retractions.
 Poorly audible breath sounds – if severe disease

Diagnosing bronchiolitis
The diagnosis of acute bronchiolitis is clinical:
 A previously healthy infant presenting with a first episode of

wheezing following a
period of upper respiratory symptoms.
Investigations
Are not routinely recommended
 Chest radiography is not routinely indicated.

- Reveal if there is: associated atelectasis
 Viral testing (PCR or rapid immunofluorescence) is not
routinely recommended
Management
Most children can be treated at home.
 Hospital admission is indicated if any of the following are

present:
o Apnea (observed or reported)
o Persistent oxygen saturation of < 90% when breathing air
o Inadequate oral fluid intake (50–75% of usual volume)
o Severe respiratory distress – grunting, marked chest
retraction or a respiratory rate >70
breaths/minute.
o inability to breastfeed or drink, or vomiting everything
o convulsions, lethargy or unconsciousness

The treatment of children with viral bronchiolitis is SUPPORTIVE

Management
 Supplemental Oxygen: - if spo2 <90%
 Target oxygen saturations: a threshold of 90%
(national guidelines in the United States)
 supplemental hydration
 Nutrition: Encourage the child to eat as soon as food can be
taken or Nasogastric feeding
 Frequent suctioning of nasal and oral secretions
 Antipyretic: if fever (≥ 39 ° C)
Risk factors for severe or complicated bronchiolitis include:
 Prematurity (gestational age ≤36 weeks)

 Low birth weight
 Age less than 12 weeks
 Chronic pulmonary disease, particularly bronchopulmonary
dysplasia (also known as chronic lung disease)
 Anatomic defects of the airways
 Hemodynamically significant congenital heart disease
 Immunodeficiency
 Neurologic disease
Prognosis:
 Bronchiolitis is a
self-limited illness and often resolves without complications in
most previously healthy infants. Severely affected infants are at
increased risk for complications and recurrent wheezing.
 The overall
mortality rate in children hospitalized bronchiolitis in developed
countries is less than 0.1%.

Complications
 Atelectasis (if complete obstruction)
 Pneumothorax
 Hypoxemia
 Respiratory failure
 Apnea
 Secondary bacterial infection
DISCHARGE CRITERIA — Minimal clinical criteria for discharge from

the hospital include:
 Respiratory rate: <60 bpm for age <6 months, <55 bpm for age
6 to 11 months, and <45 bpm for age ≥12 months
 Caretaker knows how to clear the infant's airway using bulb
suctioning
 Patient is stable while breathing ambient air: the patient
remain stable for at least 12 hours prior to discharge
 Patient has adequate oral intake to prevent dehydration
 Caretakers are confident they can provide care at home
 Education of the family is complete: no smoking, proper
breastfeeding

CHAPTER 9
APPROACH TO STRIDOR
Definition
Stridor is a harsh, musical sound during inspiration due to partial
obstruction of the lower portion of the upper airway (including the
upper trachea, larynx, oropharynx and subglottis).
Mechanism
 During inspiration, the pressure inside the extrathoracic
airway falls below atmospheric pressure, causing airway
collapse.
 In contrast, during expiration intrathoracic pressure rises on
expiration and causes airway collapse.
 Stridor is caused by the oscillation of a narrowed airway.
 It is explained by Bernoulli's Principle, which states that as
the speed of a moving fluid increases, the pressure within the
fluid decreases.
Airflow Local area of Vacuum effect This airway walls Squeak

through a low pressure formed distal to collapse and characteristic of
narrowed tube created the narrowing vibrate stridor generated

DDX Unique features
Viral croup Barking cough

Respiratory distress
Hoarse voice
If due to measles, signs of measles
Retropharyngeal Soft tissue swelling

abscess Difficulty in swallowing
Fever
Foreign body Sudden history of choking

Diphtheria Bull neck appearance due to enlarged cervical nodes

and oedema
Red throat
Grey pharyngeal membrane
Blood-stained nasal discharge
No evidence of DTP vaccination
Congenital Stridor present since birth

anomaly (laryngotracheomalacia)
Epiglottitis Soft stridor

‘Septic’ child
Little or no cough
Drooling of saliva

Inability to drink
Anaphylaxis History of allergen exposure

Wheeze
Shock
Urticaria and oedema of lips and face
Burns Swollen lips

Smoke inhalation
NB: Croup accounts for more than 90% of all cases of stridor in
children. It most commonly occurs in children 6 to 36 months of
age and in preschool children, but is rare beyond age six years.
Croup Epiglottitis
Onset Over days Over hours
Preceding Yes No
coryza
Cough Severe, barking Absent or slight
Able to drink Yes No
Drooling saliva No Yes
Appearance Unwell Toxic, very ill
Fever <38.5° C >38.5° C
Stridor Harsh, rasping Soft, whispering
Voice, cry Hoarse Muffled, reluctant to

speak

Diagnosis of croup is clinical, based on the presence of a

barking cough and stridor, especially during a typical
community epidemic of one of the causative viruses.
Clinical assessment of a child with a stridor

Diagnostic examinations may need to be delayed to provide
appropriate initial supportive care.
1. SECURE ABC OF LIFE
A rapid assessment of Airway patency, Breathing effort and

Circulation collapse needed to identify patients who need immediate
intervention in conditions like:
 Epiglottitis
 Anaphylaxis
 Airway burn
 Foreign body aspiration
Once the child is stable, the next step is a detailed history and
physical examination to identify the cause of the stridor.
2. HISTORY
 Characterize the stridor

https://www.youtube.com/watch?v=Hd7A28T0TOE ……
… hear the sound
oAge of onset:
 Neonates and infants – Congenital disorders presenting
in the first few weeks of life.
 Infants and toddlers – Croup, Foreign body aspiration
and Epiglottitis (uncommon but potentially
life-threatening)

 School-aged children and adolescents – peritonsillar

abscess and vocal cord dysfunction (presenting with
recurrent episodes of stridor and dyspnea)
 All ages – Anaphylaxis and bacterial tracheitis
oAcuity of onset:
 Acute onset - within minutes or a few hours
 With fever – epiglottitis or bacterial tracheitis
 Without fever – Foreign body aspiration or
anaphylaxis
 Subacute onset – over the course of a few days
 Laryngotracheitis (croup), Peritonsillar and
retropharyngeal abscesses
 Chronic or recurrent – subglottic stenosis or exogenous
compression (vascular ring or a tumor), vocal cord
dysfunction.
oOnset during:
 feeding- aspiration 2° to swallowing dysfunction,
tracheoesophageal fistula, GER
 Sleep – pharyngeal origin, Spasmodic croup
 Activity (exercise, agitated) – mild croup, vocal cord
dysfunction
 Quiet/Rest - severe croup
ASSOCIATED SYMPTOMS
oFever –
 High grade fever - serious bacterial infection (epiglottitis,
peritonsillar or retropharyngeal abscesses or
bacterial tracheitis)
 Mild fever - croup
oAltered mental status (lethargy, anxiety) - an impending airway
obstruction
oDrooling of saliva – epiglottis, a foreign body in the trachea, or a
mass compressing the anterior esophageal wall.
oBarking cough – croup
oHoarseness of voice – vocal cord injury due to inflammation
(croup) or paralysis

oMuffled voice – supraglottic obstruction, (such as retropharyngeal

or peritonsillar abscess or epiglottitis)
oDysphagia - epiglottis, a foreign body in the trachea, or a mass
compressing the anterior esophageal wall.
oChoking – foreign body aspiration
oHives – (Presence of rash, hypotension, and wheezing with acute
onset of stridor) - an allergic reaction with angioedema.
 PMSHx
oPrenatal and perinatal period (infections, prematurity,
complicated delivery, necessity of intubation, and
mechanical ventilation) – congenital disease
oPrevious admissions secondary to respiratory diseases –
chronic stridor causes
oSurgical history of the upper chest or neck - might have
contributed to vocal cord paralysis, or tracheal or
subglottic stenosis
opotential exposure to food or environmental allergens –
anaphylaxis
 Immunization history
oDPT vaccination - Diphtheria
oHib (H. influenza type b) vaccination – Epiglottitis
oMeasles vaccination – croup caused by measles virus
 General Appearance –
 Vital signs
oThe height and weight –
 Failure to thrive - a chronic process
 recent weight loss - a subacute process (infection)
 HEENT
o craniofacial malformation
o swollen neck (bull neck) – Diphtheria

o grey oropharyngeal membrane (which couldn’t be

scrapped) – Diphtheria
 Lymphoglandular system
oLymphadenopathy - intrathoracic process (infection or
tumor compressing the airway)
 Respiratory –
oInspection: both during rest and after activity
 Audible stridor
 Cyanosis, nasal flaring, and retractions.
 Children - sit up in a "tripod" or "sniffing" position
(trunk leaning forward, neck hyperextended, chin
thrust forward) – epiglottitis (also in severe asthma)
 Infants - extend their neck backwards in an effort to
maximize upper airway patency – epiglottitis
oAuscultation
 Stridor - heard best over the anterior neck.
- Inspiratory stridor – extrathoracic obstruction
- Expiratory stridor - intrathoracic obstruction
- Biphasic (inspiratory and expiratory) stridor -
critical or fixed obstruction (at any level), or
obstruction between the glottis and subglottis
- Snoring (when asleep) or stertor (when awake) -
Nasal, nasopharyngeal, or oropharyngeal
obstruction
 Decreased or absent air entry – severe case
 Cardiovascular findings – if complicated Diphtheria
 Skin and extremities –
oSkin hemangiomas - Airway hemangioma possibility

oClubbing - congenital heart disease or bronchiectasis
oMaculopapular rash - measles
4. Investigations
 CBC with differential

oIncreased lymphocytes – viral infections (croup)
oIncreased PMN cells – bacterial infections (epiglottitis)
 Viral culture – etiologic diagnosis
 Radiography
oNeck x-ray –
 soft tissue air-fluid levels, increased retropharyngeal
space – Retropharyngeal abscess
 "steeple" sign (the subglottic arch becomes
edematous and shows an inverted "V" in AP view) –
croup
 edematous epiglottis with the thumb sign and
enlarged aryepiglottic folds in lateral view –
epiglottitis
oChest x-ray — if intrathoracic problem suspected
 mediastinal lymphadenopathy or masses
 vascular rings (a right aortic arch)
 foreign body aspiration (mediastinal shift, unilateral
hyperinflation, atelectasis, or actual foreign body, if
radiopaque)
 CT scan of the chest and neck with contrast - diagnosis of
retropharyngeal cellulitis. also used to look for enlarged
lymph nodes, tumors, aberrant arteries, and vascular rings.
 MRI - if a vascular ring is suspected after plain radiography
 Spirometry —
oIt is difficult to perform in infants and young children.
oIt can be performed in children over six years of age.
oIt is useful for those with chronic stridor in whom the
location or nature of the obstruction cannot be localized by
the radiographic.
 Nasopharyngoscopy, laryngoscopy, and bronchoscopy -
allows definitive diagnosis of the cause of stridor.
oconfirm laryngomalacia
odiagnose foreign body aspiration and remove the foreign
body
ofor patients with chronic or intermittent stridor

ogold standards for evaluation and diagnosis of subglottic,

tracheal, and central airway lesions
Treatment of croup
Is based on the croup score
Clinical feature Assigned score

Level of consciousness Normal, including sleep = 0
Disoriented = 5
Cyanosis None = 0
With agitation = 4
At rest = 5
Stridor None = 0
With agitation = 1
At rest = 2
Air entry Normal = 0
Decreased = 1
Markedly decreased = 2
Retractions None = 0
Mild = 1
Moderate = 2
Severe = 3
Score Severity Description Management
≤2 Mild Occasional barky Home treatment: Symptomatic

cough, no stridor at care including antipyretics, mist,
rest, mild or no and oral fluids
retractions
Outpatient treatment: Single dose
of oral dexamethasone 0.15 to 0.6
mg/kg (maximum 16 mg)*
3 to 7 Moderate Frequent barky cough, Single dose of oral dexamethasone

stridor at rest, and 0.6 mg/kg (maximum 16 mg)*
mild to moderate
Nebulized epinephrine¶
retractions, but no or
little distress or Hospitalization is generally not
agitation needed, but may be warranted for
persistent or worsening symptoms

after treatment with glucocorticoid

and nebulized epinephrine
8 to 11 Severe Frequent barky cough, Single dose of oral/IM/IV

stridor at rest, marked dexamethasone 0.6 mg/kg
retractions, significant (maximum 16 mg)*
distress and agitation
Repeated doses of nebulized
epinephrine¶ may be needed
Inpatient admission is generally

required unless marked
improvement occurs after
treatment with glucocorticoid and
nebulized epinephrine
≥12 Impending Depressed level of Single dose of IM/IV

respiratory consciousness, stridor dexamethasone 0.6 mg/kg
failure at rest, severe (maximum 16 mg)
retractions, poor air
Repeated doses of nebulized
entry, cyanosis or
epinephrine¶ may be needed
pallor
Intensive care unit admission is
generally required
Consultation with anesthesiologist

or ENT surgeon may be warranted
to arrange for intubation in a
controlled setting

CHAPTER 10
APPROACH TO ALTERED MENTAL
STATUS
 Consciousness = Arousal + Awareness
 Arousal-determined in the brainstem’s ascending RAS
 Awareness- determined in cortex
Level of consciousness
can be described-
1.Qualitative description
2.Quantitative description
1. Qualitative description
a) Delirium
 “clouding of consciousness”
 acute or sub-acute reduction in level of consciousness
 usually fluctuating accompanied by abnormal
sleep/wake patterns & psychomotor disturbance
b) Lethargy-
 state of weariness
 diminished energy mental capacity
c) Obtundation-
 hypersomnia, pathologic drowsiness
 Increase above the patient’s normal sleep/
wake ratio
 Response to pain & other stimuli
d) Stupor –
 spontaneous arousability
 Responsive only to pain
e) Coma
 state of unarousable, unresponsiveness
 Unresponsive to pain

f) Syncope-
 brief loss of consciousness caused by global
failure of cerebrovascular perfusion
g) Dementia-
 sustained or permanent multidimensional or global
decline in cognitive function.
H) Vegetative state-
 sustained, complete loss of cognition
 Caused by-
 acute, severe, bilateral cerebral damage
 End stage of progressive dementia
I) Locked- in-state-
 severe or total incapacity to express voluntary
response
 Due to damage to descending motor pathway and
peripheral motor nerves
 Most patients can use vertical eye movements to
signal by code
2. Quantitative description
A. GLASGOW COMA SCALE (GCS)
 15 point scale, evaluates three areas of CNS function

 15= full function 3= no function
I. EYE OPENING
 evaluates arousability and alertness
II. VERBAL RESPONSE
 evaluates awareness
III. MOTOR FUNCTIONING-
 evaluates mentation and integrity of major CNS

pathway
 The best motor response of any limb is taken as
score

 Eye opening-
1- Does not open eyes
2- Opens eyes in response to noxious stimuli
3- Opens eyes in response to voice
4- Opens eyes spontaneously
 Verbal response-
1- No verbal response
2- Incomprehensible sounds
3- Inappropriate words
4- Confused and disoriented fluid speech
5- Oriented with normal speech
 Motor response-
1- No movements
2- Extension to noxious stimuli
3- Flexion to noxious stimuli
4- Withdrawal to pain
5- Localizes to pain
6- Obeys commands
DISADVANTAGE OF GCS
 doesn’t fully assess brainstem function
 fails to discriminate between low score & intubated patients
B. FULL OUTLINE OF UNRESPONSIVENESS (FOUR)
 17 point scale, with potential scores ranging from 0-16.

evaluates four variables
i. Eye response
ii. Motor response
iii. Brainstem reflex- pupillary&corneal reflex
iv. Respiratory effort

 Has advantage for intubated patients
 Eye response-
o4- Eyelids open and comply with verbal stimuli
o3- Eyelids open but not tracking
o2- Eyelids closed but open to loud noise
o1- Eyelids closed but open to noxious stimuli
o0- Eyelids remain closed
 Motor response-
o4- Thumbs up, fist or peace sign
o3- Localize to pain
o2-Flexion to pain
o1-Extension to pain
o0-No response to pain or generalized myoclonus status
 Brainstem reflex-
o4- Pupil and corneal reflexes present
o3- One pupil wide and fixed
o2-Pupil or corneal reflex absent
o1-Pupil and corneal reflexes absent
o0- Absent pupil, corneal, and cough reflex
 Respiration-
o4- Not intubated Regular breathing pattern
o3- Not intubated, Cheyne–Stokes respiration
o2- Not intubated, irregular breathing
o1- Intubated but breathing above the ventilator y rate
o0- Breathing at vent rate or apnea

C. THE REED CLASSIFICATION
 In the setting of poisonings or intoxication to evaluate

increasing depth of coma encountered with antidepressant
drugs
 CVS is included because ingestions may depress myocardial
contractility or cause vasodilation.
Grade 0- Asleep, can be aroused, will answer questions

Grade 1- Comatose, withdraws from painful stimuli, intact
reflexes
Grade 2- Comatose, does not withdraw from painful stimuli,
no respiratory, circulatory depression, intact reflexes
Grade 3- Comatose, reflexes absent, no respiratory,
circulatory depression
Grade 4- Comatose, reflexes absent, respiratory or
circulatory problems
Grade 0, 1&2- good prognosis
Grade 3&4- very serious
D. PEDIATRIC GCS (MODIFIED GLASGOW COMA SCALE)
 for under 5 children

 In intubated patients, verbal response is not assessed; the suffix
‘T’ is added

 Eye opening
o4- Spontaneously
o3- To speech
o2- To pain
o1- None
 Verbal
o5- Appropriate words; smiles; fixes and follows
o4 -Consolable crying
o3-Persistently irritable
o2-Restless agitated
o1-None
 Motor-
o5-Obeys commands
o4-Localizes pain
o3-Flexion to pain
o2-Extension to pain
o1-None
 Normal Total Score Based on Age
 Birth–6 mo- 9
 7-12 mo- 11
 1-2 yr -12
 2-5 yr- 13
 >5 yr- 14
E. Blantyre Score
 Modification of pediatrics GCS

 Designed to assess malaria/coma in children
 Minimum score= 0, maximum score=5, abnormal less than or
equal to 4

Best motor response

2- localizes pain
1-withdraws limb from pain
0-non-specific or absent response
Verbal response
2-appropriate cry
1-moan or inappropriate cry
0-none
Eye movement
1- directed (e.g. follows mother’s face)
2- not directed
F. AVPU (ALERT, VOICE, PAIN, UNRESPONSIVENESS)
 system used by first responders& emergency medical professionals

Advantages:
 Unlike GCS not developmentally dependent (a child does not have
to understand spoken language or follow commands.)
 A= Alert- can answer questions
 V=Voice- respond to verbal commands
 P=Pain- reponds to pain
 U=Unresponsiveness
Coma is caused by-
1. structural brain disease

2. diffuse neuronal injury
3. psychogenic causes
Etiologic classification

1. Infectious
 Viral-Aseptic meningitis
 Bacterial- meningitis, brain abscess, empyema, systemic
infection with shock
 Fungal-fungal meningitis, fungal brain abscess
 Protozoan-meningitis, abscess, cerebral malaria
2. Metabolic/Systemic
 Hypoglycemia, DKA
 inborn errors of metabolism
 Encephalopathy-hepatic, uremic
3. Toxic
4. Traumatic- concussion, contusion, DAI, hematoma
5. Anatomic- tumor, hydrocephalus, hematoma, brain abscess
6. Hypoxic-ischemic- neonatal asphyxia, near drowning, CO
poisoning
7. Epileptic- post-ictal state, status epilepticus
8. Vascular- acute confusional migraine
9. Psychological- conversion disorder
Common causes of altered mental status by age:
1. Neonate-hypoglycemia, birth asphyxia, congenital anomalies of

CNS, systemic infectionwith shock, congenial infection, bacterial
meningitis, birth trauma
2. Infant- meningitis (viral, bacterial)
- Trauma (abuse, asphyxia)
- Septic shock
- Post-immunization
3. Child- meningitis (viral, bacterial), encephalitis, trauma, seizure,
DKA, septic shock, toxic shock syndrome, intentional ingestion

Management approach
Have four parts

1. Stabilization
2. Rapid clinical assessment
3. Reversal of immediately treatable toxic or metabolic
causes
4. Determination of level of CNS function and causes of
coma
NB:
 Any alteration in consciousness must be managed as a life

threatening emergency until proven otherwise
 Suspect abuse in a child if:
oThe history and physical finding does not match
oBruising in other site
oAbnormal behavior
1. Stabilization
 Includes ABC of life
A. Evaluating a patient’s Airway& secure a patent airway may
involve- positioning (jaw thrust and chin uplift)
- suctioning
- intubating
B. Assessing patient’s Breathing- obtunded, stuporous or
comatose patients -usually require intubation unless their mental
status is improving or can be readily reversed
C. Assessment of Circulation- evaluation of:
 vital signs
 presence& volume of peripheral pulses
 adequacy of end-organ perfusion
 best evaluated in the:

 skin= temperature, color and capillary refill

 kidneys=urine output
 brain=mental status
2. Rapid clinical assessment

 Focused history+physical examination with a targeted
neurological examination
A.Pertinent history
 recent history preceding the change in mental status
 The patient’s medical history (hx of DM)
 family history (e.g. seizures, encephalopathy)
 traumatic injuries in the previous few days
 recent fevers
 signs or symptoms of infection or systemic disease
 endemic malaria
 vaccination history
 dietary history
 Infants
 hypoglycemia (fasting or emesis)
 hyponatremia (ingestion of free water)
 Exposure to drugs or toxins especially:
 patient with a sudden onset of unexplained symptoms (coma,
seizures)
 Gradual onset of symptoms preceded by a period of confusion
or delirium.
 Caregivers should be asked about possible access to medications,
illicit drugs, and environmental toxins.
B. Physical Examination
GENERAL APPEARANCE-
 Qualitative description of level of consciousness

 Posturing

VITAL SIGNS-
 BP- hypotensive in case of shock

 PR-
 tachycardia----shock (early phase)
 Bradycardia
 RR-bradypnea, tachypnea
 Temperature : - increased- systemic illness, infection
HEENT
 Head
NB:
 In Infants bulging fontanel =raised intracranial pressure
 In the absence of a febrile illness, trauma should be suspected in
any infant with a bulging fontanel.
NB
 Absence of history of trauma or physical finding does not exclude
a traumatic or anatomic cause of coma.
- Trauma in the head
Signs of Head Trauma
General: laceration, hematoma, ecchymosis, swelling, palpable

crepitation, step-off of skull
Signs of basilar skull fracture:
 hemotympanium
 CSF rhinorrhea (anterior basal fracture)
 CSF otorrhea (middle basal fracture)
 Racoon eyes(anterior basal fracture)
 Battle sign(middle basal fracture)
 Fundoscopic examination

 Retinal hemorrhage in children with:

 Abusive head trauma
 Subarachnoid hemorrhage (ruptured aneurysm AV
malformation)
 Hydrocephalus
 Papilledema- increased ICP
Signs and symptoms of increased intracranial pressure (ICP)
Early sign and symptoms Late sign and symptoms
 Headache  Further decrease in level of

consciousness
 Emesis  Bulging fontanels
 Change in level of  Decreased spontaneous

consciousness movement
 Decrease in GCS score  Posturing
 Irritability  Papilledema
 Sunsetting  Pupil dilation with decreased

or no response to light
 Decreased eye contact  Increased blood pressure

(infants)
 Pupil dysfunction  Irregular respirations
 Cranial nerve  Cushing’s triad (late, ominous

dysfunction sign)
RESPIRATORY EXAMINATION
Respiratory pattern assesses brainstem function
 Chyne- Stokes Respiration-

o periods of hyperpnea alternate with shorter apneic phase

o in the presence of-

 bilateral hemispheric dysfunction
 diencephalic dysfuntion
 may also precede transtentorial herniation
 CENTRAL NEUROGENIC HYPERVENTILATION-
o Tachypnea and hyperpnea

o in the presence of mid-brain dysfunction
 APNEUSTIC-
o prolonged pause at full inspiration
o in the presence of damage to lower pontine region
 Cluster breathing-
o cluster of breaths separated by periods of apnea
o low pontine to upper medullary lesion
 Ataxia
o irregular breathing
o medullary lesion
 Agonal respiration
o slow regular breathing
o medullary lesion
 Kussmaul breathing
o Rapid and deep breathing
NEUROLOGIC EXAMINATON
It should focus on:
1. identifying lateralizing or Focal finding
2. Recognizing brainstem dysfunction
3. Defining severity of illness
1. FOCAL FINDINGS
 Can be determined by:pupils asymmetry in size or reactivity
 asymmetric motor examination- should be tested
1st - spontaneous purposeful movement

2nd - movement in response to command
3rd movement in response to noxious stimuli
2. BRAINSTEM FUNCTION
 Evaluated by
 Respiratory pattern
 Assessing corneal reflex
 Testing oculocephalic or oculovestibular reflex
3. Reversal of Immediately Treatable Toxic or Metabolic Causes
 If the etiology of altered consciousness is not known yet,

emphasis should shift to a systemic review of reversible cause:
A. Hypoglycemia
B. Narcotic intoxication
C. Benzodiazepine ingestion
A. Hypoglycemia
 Medical emergency
 All unresponsive children should receive dextrose (bolus), unless
a diagnosis other than hypoglycemia is apparent
 The dextrose bolus should be followed by continuous infusion of
glucose & electrolyte if the child’s mental status improves (there is
lab confirmation of hypoglycemia) to prevent rebound
hypoglycemia.
B. Narcotic Intoxication
 Naloxone is administered- competitive antagonist of narcotics
 Finding- marked depression of consciousness without an
obvious cause
 Hypoventilation
 miosis (not always)
C. Benzodiazepine ingestion

 Flumanezil is given if only benzodiazepine ingestion is suspected as

administration of flumazenil to a patient who has ingested multiple
agents may precipitate seizure (flumazenil is a competitive antagonist
of seizure).
4. Level of CNS function and causes of Coma

 If coma is stable, detailed physical examination & expanded
laboratory evaluation can be done to determine level of CNS
function & causes of coma.
 Coma can be considered stable

1) No focal neurologic finding
2) No evidence of significant brainstem dysfunction
3) ICP is not raised
4) No evidence of head trauma or CNS infection
5) No rapidly reversible toxic or metabolic cause
 Causes of coma can be

 Structural
 Metabolic
 Origin of coma can be

 Hemispheric
 brainstem
 Origin of coma can be elucidated by evaluation

I. Pupillary size & reactivity
II. Eye movements
III. Motor response
IV.Respiratory pattern
I. PUPILLARY SIZE & REACTIVITY
Generally
 preserved in metabolic encephalopathy except in drug-induced esp.
potent anticholinergic component

 absent in structural lesion
NORMAL FINDING
 Pupillary light reflex involves adjustment in pupil size with

changes in light levels
 Normal finding
 Direct response- constriction of the pupil in the eye
to which the light is directed
 Consensual response- constriction of the pupil in
the eye opposite to the eye which the light is directed
 PUPILLARY LIGHT REFLEX
 Unilaterally dilated & fixed pupil

 Uncal herniation (with entrapment of CNIII)
 small, reactive pupil (Hypothalamic dysfunction=
 ipsilateral miosis with Horner syndrome)
Horner syndrome
Miosis, Ptosis, Anhidrosis
 Midbrain damage
 in the nuclei= mid-sized fixed pupil
 tectal region= mid-position or slightly large, fixed pupil
 (Pupillary size fluctuates spontaneously)= intact
accommodation
 Disrupts both sympathetic fibers & parasympathetic
stimulation
 Pontine lesion
 Affect descending sympathetic fibers
 Pin-point pupil- symmetrically small pupil
 Medullary lesion
 laterally= Horner syndrome
 centrally= fixed dilated pupils

II. Eye movement

 Degree of eye deviation is more dramatic with hemispheric damage
 Bilateral hemispheric depression=Roving eye movement (if
brainstem function is intact)
 Seizure emanating from one hemisphere=tonic lateral deviation to
the contralateral side
If patients eye is not moving, reflex eye movement are tested by:
 REFLEX EYE MOVEMENT
 Positive eye reflex- absence of cortical input on an intact

brainstem
 involves nuclei of CN VII, VI, IV
A. Oculocephalic Reflex (Doll’s eye)
Elicited by-
 rotating the child’s head side to side
 If brainstem function is intact, the eye deviates opposite to the
head
 moving the child’s head in vertical plane
 Positive reflex-upward gaze when head is flexed and downward
gaze when head is extended
 Contraindicated if cervical spine injury is suspected
B. Oculovestibular reflex (cold caloric)

 Tested by instilling upto 120ml of icewater into the ear canal.
1st ear canal must be visualized to ensure that

-there is no obstruction
-tympanic membrane is intact
2 Head is placed at 300 angle from the horizontal (semicircular
nd
canal=vertical)
3rd After a minimum of 5 minutes, the other ear must be tested.
 POSITIVE RESPONSE in awake patient=nystagmus with the slow

component toward the irrigated ear & fast component away from
the stimulus.

 BILATERAL HEMISPHERIC DEPRESSION
-fast phase of nystagmus dissipates

-eyes tonically deviated toward the stimulus
 LOW BRAINSTEM LESION-
 both eye reflexes are absent
 DAMAGE TO MEDIAL LONGITUDINAL FASCICULUS (MIF)
 ipsilateral eye fails to adduct on irrigation of contralateral

ear canal
 opposite eye abducts normally
E.g. damage to left MIF- on irrigation of right ear canal
 Right ear abducts
 Left eye doesn’t adduct
Corneal reflex
 Normal effector response (in response to loud noise or bright

light)-upward deviation of the eye (CN III) + closure of the eyelid
(CN VII).
 Absence of blink-dysfunction of pontine reticular formation
 Unilateral absence of blink-facial nerve lesion
III. Motor response

 includes:
A. Body position
B. Spontaneous movement
C. Response to noxious stimuli
 sternal rub or
 increasing subungual pressure to the fingernails or
toenails
A. Body position
 Intact brain stem

 Normal position

 Spontaneous non posturing movement

 Hemiparesis or hemiplegia
 Structural lesion
 Contralateral hemisphere
 Subcortical region
 Ipsilateral spinal cord injury
 Hypertonia or hyperreflexia
 Previous corticospinal tract disease
 Acute brainstem injury at mid brain pontine level
 Severe metabolic deraingement
 Hypotonia
 Bilateral hemispheric dysfunction
 Medullary or spinal cord lesion
 Falcid paralysis to noxious stimuli
 Pontomedullary damage
 Spinal cord damage
POSTURING
 Decorticate posturing(flexion)- Hemispheric dysfunction with

intact brainstem
 Decerebrate posturing
oMore ominous
oOpisthotonus+clenched teeth
 Severe form of decerebrate posturing
 Suggests brain stem compression
 Severe structural injury to mid brain pontine region
 Severe metabolic disease
IV. BREATHING PATTERN
Based on the above finding
1. Anatomic cause
Patients should undergo emergency head CT-scan
2. Metabolic disease
LABORATORY EVALUATION

 Glucose level
o Hypoglycemia, hyperosmolar coma
 Lumbar puncture
o Infection, hemorrhage, meningeal carcinomatosis
 Electrolytes
o Sodium= osmolar abnormalities
o Calcium=hypo or hyper calcemia
 BUN= uremia
 Arterial blood gas, PH, PCO2, PO2, Sao2
o Acidosis, alaklosis, hypoxia, CO, methhemoglobinemia
 LFT, drug level, blood and CSF culture, EEG
 Toxicology screen
NB: negative result does not rule out on undetermined ingestion
NB
- If physical finding and laboratory studies do not yield a

diagnosis, head CT-scan should be done to rule out anatomic or
vascular cause
- If head CT is normal, DO MRI
If MRI is normal
Most likely cause of coma is:
Intoxication
Psychological factor
Seizure disorder
Red flags of coma

 Personal history of toxic ingestion, trauma, seizure, infections,
metabolic disturbance
 Signs of increased ICP
 Rapidly progressive rostral-caudal deterioration

Malaria
Definition
MALARIA
 Is an acute illness characterized by paroxysms of fever, chills,

sweats, fatigue, anemia and splenomegaly
 One of the leading cause of morbidity and mortality
ETIOLOGY
MALARIA
 Caused by intracellular plasmodium protozoa
 ransmitted to humans by
 By female anopheles’ mosquito

 Blood transfusion
 Transplacentally
 Use of contaminated needles
 Organ transplantation
 Causative agent
 P.malaria, P.vivax, P.ovale, P.falciparum, P.knowlesi(newly

discovered)
Epidemiology
 Occur in 95 countries (half of the world population)
 Principle area of transmission
 Africa, Asia and South America
 P.falciparum and P.malaria are found in most malarious areas
 P.ovale is the least common and is transmitted primarily in

Africa.

 NB: Malarial death in areas of high malaria transmission occur

primarily in children <5yrs of age
Pathogenesis
Plasmodium species have two reproductive cycle
1. Asexual phase
 In human host
 Marked amplification(100 to 1014) occurs here
 Is a two-step process
 Exo erythrocytic phase
 Erythrocytic phase
2. Sexual phase
 In the mosquito
 The pathogenesis results from combination of
1. PARASITE SPECIFIC FACTOR
 Sequestration - Interaction between infected RBC and

vascular endothelium
 Adhesion Of IRBC and vascular endothelium
 Roseting Adherence between of NIRBC to IRBC
 Agglutination IRBC to IRBC
2. HOST IMMUNE RESPONSE
 TNF-alpha
 Poly clonal activation
 Leads to hypergammaglobulinemia and formation of
immune complex
 Immune suppression
 Symptoms are due to

1. ERYTHROCYTE RUPTURE and release of merozoites leads to
Paroxysmal symptoms

 Every other day for p.ovale and p.vivax(72 hrs)

 Every 3 day for p.malaria
 24 hrs for p.falciparum and p.knowlesi
2. CYTOADHERANCE
 Leads to obstruction of blood flow and capillary damage

that cause vascular leakage of blood, protein, fluid and
tissue anoxia
 prevent Infected RBC from circulating through spleen
3. Parasite ANAEROBIC METABOLISM
 Leads to hypoglycemia and metabolic acidosis
SEVERE MALARIA IS MORE COMMON IN P.FALCIPARUM DUE TO:
 Higher density of parasitemia(60%) this is because p.falciparum

infects both mature and immature ertythrocytes(leads to excessive
production of pro-inflammatory cytokines)
 Cythoadherance is only for p.falciparum
 Polyclonal activation
 p.ovale and p.vivax infects immature erythrocytes

 p.malaria infects mature erythrocytes
Immunity
 Is incomplete (prevent severe infection but allow future infection)
This is due to:
 Intracellular replication
 Cytoadherance
 Rapid antigenic variation
 Alteration of the host immune system
HOST PROTECTIVE FACTORS AGAINST PLASMODIUM INFECTION:
 Hemoglobin S (sickle erythrocyte)

 Resist malaria parasite growth

 Lacking of duffy blood group antigen

 Resistance to P.Vivax
 Hemoglobin F (fetal hemoglobin) and ovalocyte
 Resistance to P.Falciparum
NB
 In hyper endemic areas

oNewborns rarely become malaria ill due to:
 Passive maternal antibody
 High levels of fetal hemoglobin
 Severe disease occur
oChildren 3 months up to 2-5 yrs (have little specific
immunity to malaria)
oElderly
oPregnancy (particularly 1st)
oAfter extended residency outside the endemic region
Clinical manifestation
 Children are asymptomatic during Incubation period
 9-14 d for P.falciparum
 12-17 d for P.vivax
 16-18 d for P.ovale
 18-40 d for P.malaria
 Incubation period can be prolonged

 6-12 months for p.vivax
 Patients with partial immunity
 Patients with incomplete chemoprophylaxis
Approach
History
1. RISK FACTOR ASSESSMENT

 Environmental risk assessment

o Presence of bushes
o Presence of stagnant water
o Rain fall season (major transmission season)
 Sept to December
 April to may
o Low altitude area (<2000m)
o High temperature area
o Agricultural work (may increase night time exposure to
mosquito bites)
 Use of protective factor
o ITN (insecticide treated net)
o IRS (indoor residual spraying)
 Living in malaria endemic area
o Endemic area
 stable malaria- occur for many months in a year
over many years
 people have high level of immunity
 in Ethiopia- Gambella, butajara, mizan, bonga,
kaffa
o Epidemic area
 unstable malaria
 seasonal type of transmission in area of low
endemicity or
 outbreaks in areas previously without malaria or
among non-immune persons
 History of previous malarial attack

o Recrudescence
 Occur from the survival of erythrocyte forms in the
blood stream
o Long term relapse
 Release of merozoites from an exoerythrocytic
source in the liver(hepatocyte schizont)
 P.ovale and P.vivax

 Persistent with in the erythrocyte

 P.malaria
o Re-infection
 History of travel to endemic area
 Maternal history of malaria in neonates to asses for
CONGENITAL MALARIA
 Acquired from the mother prenatally or perinatally

 Occur in offspring of non-immune mother with p.vivax or
p.malaria infection
 First symptom occur between 10-30 d of age
 Sign and symptom:
 Fever, restlessness, drowsiness, pallor, jaundice, poor feeding,
vomiting, diarrhea, cyanosis, HSM
 IUGR and LBW can occur even without transmission of malaria
2. SYMPTOMS
 Children often lack the typical paroxysms in adults

 High fever followed by
 Shaking chills and then
 Diaphoresis
Prodromal symptoms
 Headache, fatigue, anorexia, myalgia, slight fever, pain in the
chest abdomen and joint
 Due to release of inflammatory contents
 Symptoms of anemia
 Easy fatigability, lethargy, shortness of breath, dizziness,
tinnitus
 Due to
 Hemolysis
 Bone marrow suppression

 Sequestration of RBCs
 Yellowish discoloration of the eye

 due to increased bilirubin secondary to excessive RBC
destruction, hepatocyte destruction
3. SYMPTOMS OF COMPLICATION
 WHO has identified 10 complication of P.falciparum that define

severe malaria
I. SEVERE MALARIAL ANEMIA
 Hemoglobin <5 g/dl

 Most common severe complication of malaria in children
 Leading cause of anemia leading to hospital admission in
African children
II. Cerebral malaria

 Presence of coma in a child with p.Falciparum parasitemia and
an absence of other reasons for coma or
 Unarousable coma >30 min with GCS<7 with evidence of acute
falciparum infection
 Common In areas of midlevel transmission
 Fatality rate of 15-40%
 9-26% of children will have neurologic squeal
 Symptoms are repeated seizure, altered mental status
 Cerebral edema with increased ICP is the leading cause of death
in children with cerebral malaria
III. RESPIRATORY DISTRESS
 poor prognostic indicator in severe malaria

 caused by metabolic acidosis
IV. Seizure
 Common complication of cerebral malaria
V. Hypoglycemia

 More common in:

 Children
 Pregnant women
 Patients receiving quinine therapy
 May be confused with cerebral cerebral malaria
NB
 Any child with impaired consciousness and malaria should have

glucose level checked, if glucometers are not available, empirical
bolus of dextrose should be given
 Children with cerebral malaria fail to arouse from coma after
infusing a dextrose infusion
VI. Circulatory collapse (algid malaria)

 Rare complication
 Symptoms are hypotension, rapid weak pulse, shallow breathing
and vascular collapse
 Caused bacterial super infection
 15% of children in endemic area may have concurrent bacteremia
 Death may occur with few hours
VII. Hyperactive malarial splenomegaly(HMS)

 Chronic complication in which massive splenomegaly persists
after treatment of acute infection
 Occur exclusively in endemic areas with repeated exposure
 Due to impaired immune response to p.falciparum antigen
MAJOR COMPLICATION
 Splenomegaly(>10cm)
 IGM >2SD above local mean
 High levels of antibody to blood-stage p.falciparum
 Clinical response to antimalarial drug
VIII. Acute kidney injury

 Poor prognostic factor

 Common complication
IX. Jaundice
X. Prostration
 In ability to
 Sit (>1yr)
 Eat, drink, or breast feed(<1yr) in the absence of impaired
consciousness
 OTHER COMPLICATIONS INCLUDE
 PULMONARY EDEMA
 HEMOGLOBINURIA
 ABNORMAL BLEEDING
 Nephrotic syndrome
 Rare complication of P.malaria
 Poorly responsive to corticosteroid
 Lowlevel, undetected P.malaria infection may be present for
years and is sometimes unmasked by immunosuppression or
physiologic stress
Physical finding
 GENERAL APPEARANCE
 Qualitative description of altered mental status Hypoglycemia,

cerebral malaria
 ASL= pain, cardiorespiratory distress
 Seizure
VITAL STATUS
 BP=hypotension
 PR=rapid weak pulse(algid malaria)
 RR=tachypnea(respiratory acidosis)
 Temp=fever usually >40c may be due to cerebral malaria

HEAD AND NECK
 Pale conjunctiva(anemia)
 Icteric sclera(jaundice)
 Gum bleeding
FUNDOSCOPIC EXAMINATION
 Malaria retinopathy
 Retinal hemorrhage
 Peripheral whitening
 Macular whitening
 Vessel changes
NB
 Children with cerebral malaria who do not have malarial

retinopathy should be carefully assessed for other causes of coma
RESPIRATORY SYSTEM
 Kussmaul breathing (rapid and deep breathing)

 Metabolic acidosis
 Wheeze(pulmonary edema)
 Ronchi, bi-basal end-inspiratory crackle
 Pulmonary edema
ABDOMINAL EXAMINATION
 Hepatosplenomegaly
 Sign of peritonitis in case of ruptured spleen
 Guarding, generalized tenderness, rigidity
GENITOURINARY SYSTEM
 Costovertebral angle tenderness

 Oliguria/anuria
ISS

 Yellowish skin Discoloration (jaundice)

 Excessive bleeding from puncture site
MSS
 Muscular twitching, rhythmic movement of the head or the

extermities(cerebral malaria)
NERVOUS SYSTEM
 Quantitative description of altered mental status

 Cerebral malaria
 Contracted or un equal pupil
 Hemiplegia
 Absent or exaggerated DTR
 Positive babiniski sign
Investigation
1. DIAGNOSIS
NB
 Any child who presents with fever or unexplained systemic
illness and has traveled or resident in a malarial endemic area
with in the previous year should be evaluated for malaria
(regardless of the use of chemoprophylaxis)
Diagnosis is established by identification of organisms on:

A. Giemsa-stained smear of peripheral blood
 Superior to wright or leishman stain
 Both thick and thin smear should be obtained
 Thick smear
 Concentration of RBC 20-40 times than thin smear
 Used to quickly scan large number of erythrocyte
 Thin smear
 Allow positive identification of malarial species and
determination of infected erythrocyte percentage
 Useful for follow up

NB
Most guideline recoomends atleast 3 negative blood smear to rule out
malaria in children
 Histologically
 Band form trophozoite= P.malaria
 Banana shaped gametocyte= P.Falciparum
 Schufner dots in infected RBC= P.Vivax & P.Ovale
 Ameboid trophozoite= P.Vivax
B. Rapid immune chromatographic assay

 Rapid stick test
 100 times less sensitive than blood film
2. INVESTIGATION FOR COMPLICATION
 Full blood count

 Anemia(HGB, HCT)
 Algid malaria(WBC count)
 Platelet assessment(bleeding tendency)
 RBS
 Blood group and cross matching
 Incase blood transfusion is needed for severe anemia
 Coagulation profile
 Lumbar puncture (cerebral malaria)
 Increased pressure
 Mildly increased CSF protein
 No pleocytosis
 Normal glucose
 Blood culture
 Algid malaria
 LFT (ALT, AST, indirect bilirubin)
 Chest x-ray
 Arterial blood gas analyasis
 Metabolic acidosis
 Abdominal ultrasound
 Hepatosplenomegaly

 CT or MRI
 Cerebral malaria
Management principle
1. General management
 Health Education
 Anti-Pyretics (e.g. Paracetamol)
 Treatment Of Complication
 Close monitoring
2. specific management
 Use of anti-malarial drug
 the first line of drug is Arthemesinin based combination
therapy
 For uncomplicated malaria
 P.vivax –Chloroquine
 P.falciparum –Artemether/lumefantrine (AL) also called
Coartum
 Mixed infections-AL
 For severe malaria

 Artisunate IV or IM-first line
 Artemeter IM- 2nd line
 IV Quinine -3rd line followed by po AL
Prevention
 Avoid mosquito bites(wearing long sleeves, using ITN)
 Control mosquito
 Hemoprophylaxis
Meningitis
Definition:

 inflammation of the membranes covering the brain & spinal cord.
Types
1. Bacterial
2. Aspetic e.g. viral, fungal
a) Tuberculous
Bacterial (septic) meningitis

Etiology
For neonates: For infants and children:

 E. coli  Pneumococci
 Heamophilus influenza  Staphylococci
 Listeria monocytogens  N. meningitides
Transmission:
 Droplet infection mostly
 Blood borne - in neonatal sepsis
Epidemiology:
 Meningococci (19.4%) and pneumococci (12.9%) were the major
disease-causing organisms in Ethiopia (from Mihret W, Lema T,
Merid Y, Kassu A, Abebe W, Moges B, et al. Surveillance of bacterial
meningitis, Ethiopia, 2012–2013. Emerg Infect Dis. 2016 Jan)
 Ethiopia has the second-largest population (≈94 million in 2013)
among the meningitis belt countries of sub-Saharan Africa.
 The largest meningitis epidemics occurred in 1981 and 1989,
resulting in ≈45,000 and ≈50,000 cases, respectively.
Approach
History
SYMPTOMS

 The classic triad of bacterial meningitis are

 Fever,
 Headache, and
 Neck stiffness
 Other symptoms:
 nausea,
 vomiting,
 photalgia (photophobia),
 sleepiness,
 confusion,
 irritability,
 delirium, and coma.
 rash – if meningococcemia
 preceding systemic symptoms like: (if viral meningitis)
 myalgias,
 fatigue,
 poor feeding or anorexia

 onset of acute meningitis has two predominant patterns:

 More often:
 less common presentation: sudden onset with rapidly

progressive manifestations of shock, purpura, disseminated
intravascular coagulation (DIC), and reduced levels of
consciousness often resulting in progression to coma or death
within 24 hr.
Risk factors
 Immunization history
 Additional risk factors include:
 History of close contact, crowding/poor living conditions
(household, daycare centers, college dormitories, military
barracks) -N. meningitidis or H. influenzae type
 History of head injury, otitis media (ear ache, discharge),
mastoid tenderness, sinusitis
 poverty/low income
 Male sex
 Age: <5 yrs aged children - meningococcal infection.
 Geographic location and travel history: Epidemics in ‘African
meningitis belt’ in a cyclical pattern in 8-15 yrs.
NB: Northern and western parts of Ethiopia are parts of this belt)
 Previous medical treatment and existing conditions

-Defects of the complement system (C5-C8)
 recurrent meningococcal infection

 Splenic dysfunction (in sickle cell anemia) or asplenia –

presents with easy fatiguability, dizziness, bone or joint
pain - pneumococcal, H. influenza type b, and meningococcal
sepsis and meningitis
 T-lymphocyte defects (congenital or acquired by chemotherapy,
AIDS, or malignancy)- Listeria monocytogenes.
 Congenital or acquired CSF leak in:
 lumbar dual sinus,
 cranial or midline facial defects (cribriform plate),
 fistulas of the middle ear or inner ear
 basilar or other skull fracture (head trauma)
 Lumbosacral dermal sinus and myelomeningocele -
staphylococcal, anaerobic, and Gram-negative enteric bacterial
meningitis
 CSF shunt infections -Pseudomonas aeruginosa, Staphylococcus
spp., Propionibacterium spp
Complications
ACUTE CNS COMPLICATIONS
 Seizures - (persistent seizure after the 4th day of illness is

taken as complication)
 cranial nerve palsies - history of photophobia
 stroke - history of body weakness, slurred speech
 cerebral or cerebellar herniation - history of loss of
consciousness, difficulty in breathing
 thrombosis of the Dural venous sinuses
 Subdural effusions
 SIADH - history of nausea or vomiting, depressed mood, memory
impairment, irritability, Abnormal Body Movement, Loss of
Consiousness
 Thrombocytosis
 Eosinophilia
 anemia - history of easy fatigability, dizziness
 DIC – history of easy bruising, red dots on skin (petechiae), vaginal
or rectal bleeding
 Pericarditis - history of chest pain

 Arthritis - history of joint pain, joint swelling

LONG TERM SEQUALAES
 Sensorineural hearing loss – history of hearing loss

 mental retardation
 recurrent seizures
 delay in acquisition of language
 visual impairment – history of visual loss
 behavioral problems – history of abnormal behaviour
 Infants may have the following sign and symptoms:
 Bulging fontanelle (if euvolemic)

 Paradoxic irritability (remaining quiet when stationary
and crying when held)
 High-pitched cry
 Hypotonia
 The examination should evaluate the following:
 Focal neurologic signs
 Signs of meningeal irritation
 Systemic and extracranial findings
 Level of consciousness
 looking especially for the following (if chronic meningitis):
 Lymphadenopathy
 Papilledema
 Meningismus
 Cranial nerve palsies
 Other focal neurological signs

 Enteroviral infection is suggested by the following:
 Exanthemas
 Contact with small children with febrile illnesses
 Symptoms of pericarditis, myocarditis, or conjunctivitis
 Syndromes of pleurodynia, herpangina, and
hand-foot-and-mouth disease
GENERAL APPEARANCE
ASL (pain or loss of consciousness)
VITAL SIGNS:
Tachycardia or Bradycardia
Hypotension or Hypertension
High or Low Temprature
H.E.E.N.T:
 bulging fontanel or diastasis (widening) of the sutures

 Papilledema - Shows Increased Intracranial Pressure In
complicated meningitis.
 Signs suggestive of existing infection may indicate the source of
infection.
o Sinusitis - Nasal congestion, Mucosa Erythema, edema,
tenderness over the sinuses..
o Ottitis Media - Pain, Discharge from the ear, otorrhea
Hearing Loss,
 On Otoscopic Examination
 Hyperemic Bulging TM
 Loss of Landmarks on the TM
CVS
 The Precence Of Murmur May Suggest Infective Endocadritis

With Secondary Bacterial Seeding Of The Menenges.
INTEGUMENTARY SYSTEM: petechiae, purpura, or an erythematous

macular rash.
 tache cérébrale - elicited by stroking the skin with a blunt object

and observing a raised red streak within 30-60 sec.
NB: Meningococcemia rash typified by - an initial petechial rash that

evolves into ecchymotic and purpuric lesions.
CNS:
 General: Altered mental status - irritability, lethargy, stupor,

obtundation, and coma.
 Cranial nerves neuropathies of II, III,VII and VIII.
 Meningeal irritation –
 nuchal rigidity
 back pain
 Kernig sign (flexion of the hip 90 degrees with subsequent pain
with extension of the leg)
 Brudzinski sign (involuntary flexion of the knees and hips
after passive flexion of the neck while supine)
NB: In children, particularly in those younger than 12-18 mo, the

Kernig and Brudzinski signs are not consistently present
Investigations
 CBC: leukocytosis/leukopenia, thrombocytopenia, anemia
 ESR/CRP: elevated
 Blood cultures: reveal the responsible bacteria in up to 80–90% of
cases of meningitis

 Lumbar puncture (LP): most important step in the diagnosis of

meningitis.
 For CSF analysis (see the table below)
Contraindications to an immediate LP:
 evidence of increased ICP (other than a bulging fontanel) - 3rd

or 6th cranial nerve palsy with a depressed level of
consciousness, or the Cushing reflex (hypertension,
bradycardia and respiratory abnormalities)
 severe cardiopulmonary compromise
 because requires resuscitative measures for shock or
 because positioning for the LP would further compromise
cardiopulmonary function
 Infection of the skin overlying the site of the LP.
 Thrombocytopenia (but it is relative contraindication for
LP)
NB: Normal CSF contains no RBCs.

 The presence of RBCs indicates a traumatic tap or a SA
hemorrhage (SAH)
 To differentiate them:
 Traumatic tap:
 The supernatant in immediately centrifuged Bloody CSF is
clear
 Progressive clearing of bloody CSF is noted during collection
of the fluid in the case of a traumatic tap.
 SAH:
 The supernatant is xanthochromic
 Xanthochromia may also result from hyperbilirubinemia,
carotenemia, and a markedly elevated CSF protein.
 CT scan: not recommended prior to LP unless the patient has
clinical signs or is at risk for elevated ICP, like:
 papilledema,
 focal neurologic findings,
 coma,

 history of hydrocephalus, or
 history of a previous neurosurgical procedure including
CSF shunt placement.
CSF Values of common CNS disorders
CONDITION PRESSUR LEUKOCYT PROTEIN GLUCOSE (mg COMMENTS

E ES (mm3 ) (mg/dL) /dL )
(cm H2 O)
Normal <28 <5, ≥75% 20-45 >50 (or

Lymphocyte 75% serum
s In glucose)
neonates:
<20
Acute Usually 100-10,000 Usually Decreased, Organisms usually
bacterial elevated or more; 100-500 usually <40 seen on Gram stain
meningitis usually (or <50% of and isolated by
300-2,000; serum culture
PMNs glucose)
predominate
Viral Normal or Rarely > Usually Generally HSV encephalitis
meningitis slightly 1,000 50-200 normal; may by focal findings on
or elevated PMNs early be decreased MRI or CT scans or
meningoen but to < 40 in EEG.
cephalitis mononuclea some Most arboviruses
r cells viraldiseases, detected by serology.
predominate particularly Most other viruses
through mumps detected by PCR of
most (15–20% of CSF
of the course cases)
1. Acute viral meningoencephalitis: is the most likely infection to
be confused with bacterial meningitis
 A number of noninfectious CNS disorders can also mimic bacterial
meningitis, like:
2. Subarachnoid hemorrhage (SAH) is generally the major
consideration.

3. Other possibilities include:

a. Chemical Meningitis due to rupture of tumor contents
into the CSF (from a cystic glioma or craniopharyngioma
epidermoid or dermoid cyst)
b. Drug Induced Hypersensitivity Meningitis;
c. Carcinomatous Or Lymphomatous Meningitis;
d. Meningitis Associated with Inflammatory Disorders such
as sarcoidosis, systemic lupus erythematosus (SLE)
 On occasion, Subacutely Evolving Meningitis may be considered
in the differential diagnosis of acute meningitis. The principal
causes:
 Mycobacterium tuberculosis,
 Cryptococcus neoformans,
 Histoplasma capsulatum,
 Coccidioides immitis, and
 Treponema palladium
See Table 621.2 on nelson 21st edition for other less common
differentials
Treatment
Essential to improving clinical outcomes in patients with bacterial
meningitis is prompt recognition, diagnostic testing, and initiation of
appropriate antimicrobial therapy.
1. Supportive:
 Antipyretics if fever >38.5ºC

 Maintenance fluid (if only indicated)– one half to 2/3 of
normally given
2. Manage if there are signs of increased ICP or focal neurologic

findings
 Antibiotics should be given without performing an LP
 Intubation and Ventilation
 Positioning - head midline and elevated 30 degrees and Deep
sedation is helpful to minimize movement.

 Ventricular Drainage, Diuretics, Steroids
3.Anti-epileptic-
 For seizures that last >10 minutes and also ≥2 seizures (after
rulling out acute metabolic causes) must be treated aggressively.
4. Antibiotic therapy
 First line:
 Crystalline Pencillin PLUS Chloramphenicol
 If Haemophilus Influenza B: Chloramphenicol
 If Pneumococcus and Meningococcus: penicillin G
 Second line:
 Ceftriaxone
5. Need for Corticosteroids:

 Because rapid killing of bacteria releases toxic cell products after
cell lysis (e.g. endotoxin) that precipitate inflammation.
 The resultant edema formation and neutrophilic infiltration
may produce additional neurologic injury with worsening of
CNS signs and symptoms
NB: Do not use steroids in:

 Newborns
 suspected cerebral malaria
 suspected viral encephalitis
 areas with a high prevalence of penicillin-resistant
pneumococcal invasive disease
Prognosis
 The highest mortality rates is in pneumococcal meningitis
 Severe neurodevelopmental sequelae - in 10–20% of patients
 Neurologic sequelae - in 50% of patients
 Poor prognostic factors/associated with long term sequels

 infants younger than 6 months’ old

 those with high concentrations of bacteria/bacterial
products in their CSF.
 seizures occurring more than 4 days into therapy or with
coma or focal neurologic signs on presentation.

Sample history for meningitis

Identification
This is _______ a 7 years old male patient, born to father __________ a

50years old farmer and ________ a 40 years old housewife, who are
currently residents of limugenet, Jimma zone. He was admitted to
JUMC level 2 ward room #16 bed #1 on 21/11/2007 after he arrived
carried by his teachers with a referral paper from a private clinic. The
source of history is his father with a help of translator.
Previous admission: Mentioned in HPI
Chief compliant
Headache and neck stiffness of 1 week duration
History of present illness
He presented with a headache of 1 week duration. His headache is
global and worse in the evening. His headache is dull, steady and
aching type. His pain has been progressively getting worse and there
has not been any remission. He describes the magnitude of the pain
as very severe but it does not wake him from sleep. He started to
experience neck stiffness for the last 2 days before admission.
Associated with this he also has history of high grade persistent fever
of the same duration and vomiting of two episodes which was
projectile and of ingested matter.
He has a history of recent facial pain, cough, sore throat and nasal
discharge.
Otherwise,
he has no history of head injury. He has no history of loss of
consciousness.
He has no night sweats, or recent weight loss. He had no contact with
a chronic cougher. He has not suffered from previous pulmonary TB.
He has finished vaccination according to the EPI schedule.

He has no history of fatigue, irritability,

He has had no recent change in personality, mentation or speech.
He has no history of loss of balance, loss in coordinating his
movements or trouble walking.
He does not complain of blurred vision or double vision. He has no
aversion towards bright light.
He has no history of weakness, no numbness, no history of bladder or
bowel incontinence, no seizure or defects in speech.
His urine color has not changed recently. He has no body swellings.
He has no history of rash
He is one of seven children and lives with his mother and father, all of
whom are alive and haven’t experienced similar complaint. They live
in a house with 2 windows n 1 door, they hv no pets and the cattle
have a separate room. The kitchen is also separated from TB house.
He has a history of malarial attack 7 months ago and he had finished
his treatment regimen

CHAPTER 11
APPROACH TO POLY SYMPTOMS
(POLYURIA, POLYDIPSIA, AND
POLYPHAGIA)
1. Definitions
Polyuria
 Is excessive or abnormally large production or passage of urine that
is > 40 ml/kg/24h or > 2000 ml/m2/24h
 It should not be confused with Frequency which is frequent
passage of small amount of urine which occurs in cases like UTI,
Urolithiasis and Urinary incontinence.
 There are four mechanisms, which can cause polyuria. One or more
of these will be operating.
I. Increased intake of fluids as in psychogenic causes, stress
and anxiety
II. Increased GFR as in hyperthyroidism, fever, hypermetabolic
states
III. Increased output of solutes (urine osmolality > plasma
osmolality) as occurs in DM, hyperthyroidism,
hyperparathyroidism, use of diuretics (which present
more solute at the DCT)
IV.Inability of the kidney to reabsorb water in DCT as in Central
Diabetes Insipidus (CDI), Nephrogenic Diabetes Insipidus
(NDI), Drugs and chronic renal failure (CRF).
 Dehydration with Polyuria indicates osmotic Diauresis.
Polydipsia (>2000 ml/m2/24h)

 means excessive intake of water which is usually associated with
Polyuria as the body tries to compensate for the water loss.
 Types of Polydipsia

o Psychogenic (primary) polydipsia: This type of polydipsia

is caused by anxiety, boredom, stress, or underlying
mental health issues, rather than something biological.
o Drug-induced polydipsia: This is caused by certain drugs
or vitamins that cause polyuria, such as diuretics, vitamin
K, salt intake, and corticosteroids.
o Compensatory polydipsia: Compensatory polydipsia is
caused by lowered levels of antidiuretic hormones in your
body. This can lead to excessive urination.
POLYPHAGIA
 means excessive hunger or abnormally large intake of solid by

mouth.
NOCTURIA
 the complaint that the individual has to wake at night more than
one times for voiding.
Differential Diagnosis of Polyuria with Polydipsia

 Diabetes mellites (Type 1, Type 2 and Other types)
o is a common, chronic, metabolic disease characterized by
hyperglycemia as a cardinal biochemical feature.
 Diabetes Insipidus
o Diabetes insipidus is a condition characterized by excessive
thirst and excretion of large amounts of severely dilute urine
caused by absence of ADH (Central DI) or lack of ADH receptors
(Nephrogenic DI).
 Central Diabetes Insipidus
o Genetic (Autosomal Dominant)
o Acquired
o Trauma (Accidental or Iatrogenic)
o Congenital malformations (holoprosencephaly,
septoopticdysplasia, encephalocele)
o Neoplasms (craniopharyngioma, germinoma, metastasis)

o Infiltrative (Langerhans cell histiocytosis), autoimmune

(lymphocytic infundibuloneurohypophysitis), and infectious
diseases
o Drugs (chemotherapy)
o Idiopathic
 Nephrogenic Diabetes insipidus
o Genetic (X-linked, autosomal recessive, autosomal dominant)
o Acquired
o Hypercalcemia,
o Hypokalemia
o Drugs (lithium, demeclocycline)
o Kidney disease
o Primary polydipsia
o Sickle cell anemia
 Psychogenic Polydipsia
A. Diabetes Mellites (Type 1)

Is an autoimmune disorder that causes Pancreatic B-cells destruction.
The destruction leads to insulin deficiency that result in
hyperglycemia and disrupt energy storage and metabolism.
 THE NATURAL HISTORY HAS FOUR STAGES:
(Read Pathogenesis and Natural History on Nelson Textbook Pediatrics

for detail).
o Preclinical autoimmune destruction of pancreatic beta cells

o Onset of clinical symptoms
o Transient remission
o Established diabetes with acute and chronic complications
Approach
 Epidemiology and Risk Factors
o Common type in pediatrics age group with almost the same M:F
ratio. Thought there is a female predominance in low risk
countries
o Peak age 2-5 and at Puberty

oFamily history & Genetic Predisposition increase the risk

oEnvironment factors like
oViral Infections
oCongenital Rubella Syndrome
oPrenatal infection with rubella is associated with β-cell
autoimmunity in up to 70%, with development of T1DM in up to
40% of infected children.
o Enteroviruses
o Mumps Virus (Refer Childhood Infections)
o The Hygiene Hypothesis: Possible Protective Role of Infections
o Diet
 Cow milk increase the risk of T1DM.
 Vit D, Vit E, Vit C, Zink deficiencies have also been linked
with increased risk.
 Psychogenic stress
 Symptoms of T1DM
o Insulin Deficiency first causes Postprandial Hyperglycemia
o Glycosuria occur when serum glucose level exceeds the renal
threshold for glucose reabsorption (180mg/dl)
o Glycosuria will cause will cause osmotic diuresis leading to
Dehydration.
o Polydipsia occurs as the patient tries to compensate for the fluid
loss.
o Weight loss result from the catabolic state and loss of calories
through glycosuria and Ketonuria.
→ Due to these T1DM can present in the following ways
o Classic New Onset (Hyperglycemia Without Acidosis)

 Polyuria: Re-emergence of bedwetting, nocturia, and a need to
leave classes in school to use the bathroom are complaints
that suggest polyuria.
 Polydipsia
 Weight loss
o Diabetic ketoacidosis (See later)
o Silent Presentation

 Diagnosis before onset of clinical symptoms which typically

occurs in children with family history of T1DM due to close
monitoring.
 COMPLICATIONS OF T1DM
 The complications of Diabetes can be an Acute or Chronic
Acute Complications include
o Diabetic ketoacidosis
o Hypoglycemia
o Hyperosmolar Hyperglycemic state (More common in T2DM
patients)
Chronic Complications:
o Since these complications take longer time to manifest, they are

not common in pediatrics age group.
The chronic Complications can be classified as
o Vascular complications
 Macrovascular Complications
 Coronary Heart Disease
 Cerebrovascular Diseases
 Peripheral arterial Diseases
 Microvascular Diseases
 Diabetic Retinopathy
 Diabetic Nephropathy
 Diabetic Neuropathy
o Non-Vascular Complications
 Cataract
 Infections
 Skin changes
 Hearing loss etc..
1. Diabetic Ketoacidosis
 May be the first manifestation of T1DM.Resulting from a severe
deficiency of insulin or insulin effectiveness which in turn causes
burning of fatty acids and formation of acidic Ketone Bodies.

 Characterized by ketonemia, ketonuria and Metabolic acidosis.

 The entire process evolves in short period of time (form few weeks in
infants to few months in Adolescents).
 usually follows some kind of precipitants like
o Infections (Most common) The most common infections being
 Pneumonia (History of Caught, Fever and Fast breathing) and
 Urinary tract infections (History of fever, Urgency, Frequency,
Burning sensation during urination, discharge from urine.)
o Stress
o Trauma
o Drug Discontinuation or
o Inadequate therapy (common In adolescent age due to
physiologic changes)
o Myocardial Infarction (Rare in pediatrics age group) and
Cerebrovascular accident (also Rare in pediatrics age group)
 Symptoms Include
o Poly symptoms Plus
 Abdominal Pain or Discomfort
 Nausea and Vomiting – which exacerbates the dehydration
by preventing fluid intake.
 If severe, they may present with altered mental status or
even comma.
 DKA Can be classified as
Normal Mild Moderate Severe

Clinically No change Oriented, Kussmaul Kussmaul or depressed
alert but respirations; respirations;
fatigued oriented sleepy to depressed
but sleepy; sensorium to coma
arousable
CO2 20-28 16-20 10-15 <10

(mEq/L,
venous)
pH 7.35-7.45 7.25-7.35 7.15-7.25 <7.15

(venous)

o We commonly use the clinical staging in our setup.

o Severe hypernatremia (corrected Na >150 mEq/L) would also be
classified as severe diabetic ketoacidosis.
2. Hypoglycemia (See Loss of consciousness for more)

 Clinical manifestations of Hypoglycemia can be classified into two
1. Neurogenic Symptoms Due 2. Neuroglycopenic Symptoms Due To
To Activation Of Decreased Cerebral Glucose Use
Autonomic Nervous System
Sweating Headache
Shakiness, trembling Visual disturbances
Tachycardia Lethargy, lassitude
Anxiety, nervousness Restlessness, irritability
Weakness Difficulty with speech and thinking,
Hunger inability to concentrate
Nausea, vomiting Mental confusion
Somnolence, stupor, prolonged sleep
Loss of consciousness, coma
Hypothermia
Twitching, convulsions, “epilepsy”
Bizarre neurologic signs
Motor disturbances
Sensory disturbances
Loss of intellectual ability
Personality changes
Bizarre behavior
Outburst of temper
Psychologic disintegration
Manic behavior
Depression
Psychoses
Permanent mental or neurologic damage

B. Diabetes Mellites (Type 2)

Type 2 diabetes mellitus is a metabolic disorder characterized by
peripheral insulin resistance and a failure of beta cells to compensate,
leading to hyperglycemia.
Approach
 Epidemiology and Risk Factors
o More common in adult age group, but Its prevalence is
increasing in pediatrics age group Most being diagnosed in
adolescence and incidence increases with increasing age.
 Risk factors
o Ethnicity (African American, Hispanic, Native Americans, Pacific
Islanders, Asian Americans),
o Family history,
o Obesity, and sedentary lifestyle,
o A positive first-degree relative with the disorder,
o Low birth weight,
o Mother with gestational diabetes, and
o Female sex.
 Presentation
Childhood type 2 DM can present in the following ways:
o Symptomatic
 Due to hyperglycemia and include: Polyuria, Polydipsia, And
Nocturia
 Recent weight loss is less frequent
 Adolescent girls: vaginal discharge due to candida infection
may be initial presentation
o Diabetic ketoacidosis (see above)
 Hyperglycemia, ketonuria, acidosis
 10% will present with DKA
o Hyperosmolar Hyperglycemic State
 characterized by hypertonicity, extreme hyperglycemia (> 600
mg/dl), and severe dehydration. The profound hyperglycemia
results in continued osmotic diuresis and intravascular
depletion.
 Less common than DKA in pediatrics age group.

o Asymptomatic
 Identified based on screening (for type 2 DM or urinalysis as
part of a regular physical exam)
Physical Findings Of DIABETES

GENERAL APPEARANCE of the patient may show altered mental status if
the patient has DKA or HHS
VITAL SIGNS
 T0 may be elevated if the patient has Precipitant Infections
 BP may be low, due to the dehydration
 If high it may be secondary to Diabetic Nephropathy

(Chronic complication)
 Check for Postural Hypotension which is a decrease in

systolic BP by 20 or decrease in Diastolic BP by 10 when
you stand up from sitting or supine position.
 RR – May be tachypneic and May have abnormal pattern
 Kussmaul Breathing is a deep rapid non labored breathing

commonly in patients with DKA but may also occur in other
causes of metabolic acidosis.
 PR may be weak and tachycardic due to dehydration.
ANTHROPOMETRY
 They may have signs of Acute or Chronic Weigh loss
 The patient may be Overweight or Obese In case of type 2

DM
HEENT
 Eyes may appear sunken (It have to be recent onset to consider

it as a sign of Dehydration.)
 Look for cataract

 Mouth for Dry buccal mucosa, Oral Candidiasis, tooth Decay.
 Ear for signs of Infection.
 Vertigo, earache, Ear discharge, feeling fullness in ear…
LGS
 Inspect for Goiter, Palpate thyroid glands. (For Concomitant

autoimmune thyroid problems)
RESPIRATORY SYSTEM
 Fruity odor (DKA)
 Chest examination is usually normal, but in some cases lung

examination may reveal underlying triggering causes of
diabetes ketoacidosis. ex Pneumonia
 So Look for Retractions, Crackles or Wheeze.
CVS
 Precordial findings are usually normal unless there is

additional
ABDOMEN
 Inspect for Injection site (See

Figure), fat atrophy, hypertrophy
or any distension.
 Check for Hepatomegaly (Fatty

liver in DM patients)
GUS FIGURE - INJECTION

SITES FOR INSULIN
 Tanner Staging (See on Growth and

Development)
 Look for CVA tenderness, Suprapubic tenderness

(Pyelonephritis)
 For Female asses for Genital Candidiasis

MSK AND IS
 Look for acanthosis nigricans in case

of type 2 DM(See Figure 2) (which is
a dark, velvety rash present in the
axillae and neck)
 Striae and an increased waist : hip
ratio. (in case of type 2 DM)
 Inspect for any skin infections
 Look for poor skin turgor
(Dehydration)
 Inspect the extremities for Ulcers FIGURE ACANTHOSIS
NIGRICANS IN TYPE 2 DM
(diabetic foot ulcers) and trophic changes.
NS
 GCS
 Cranial nerve examination
 Focus on CN II (Optic nerve) And asses for Visual acuity

and Visual field - Diabetic Retinopathy (Chronic
Complication)
 Sensory
 some cases may elicit finding of decreased sensation in

the extremities so Check for Sense of touch, pain and
vibration in all extremities progressing form distal to
proximal.
 Motor Examination
 Meningeal Signs
Investigations
DIAGNOSTIC
 Fasting Blood Sugar

o Is a test that determine how much glucose is in the blood after

overnight (8 Hours) of fasting
 If greater or equal to 126mg/dl or
 Random Blood Sugar
o A random blood sugar test checks blood glucose at a random
time of day.
 If greater or equal to 200mg/dl with Poly Symptoms or
 HgA1c
o evaluates the average amount of glucose in the blood over the
last 2 to 3 months.
 If greater or equal to 6.5 or
 2-hour Glucose Tolerance Test –
 If greater or equal to 200mg/dl.
 If the diagnosis between type 1 and type 2 diabetes mellitus is not
clear, helpful labs include Fasting Insulin or C Peptide (both
usually high or normal in type 2 diabetes mellitus, low in type 1
diabetes mellitus ), and autoantibodies for type 1 diabetes mellitus.
INVESTIGATION FOR DKA
o (In already Confirmed Diabetic Patient) (See table above)
 Arterial Blood Gas (ABG) will demonstrate Acidosis.
 Serum bicarbonate levels will be low.
 Urine Ketone (Acetoacetate) or Blood Ketone
(β-hydroxybutyrate) will be elevated
Basic
 CBC – May show Leukocytosis

 Serum Electrolytes – Will show
o Hyponatremia - due to efflux of fluid to the extracellular space in
the presence of hyperglycemia. An increased or even normal
serum sodium concentration in the presence of hyperglycemia
indicates a rather profound degree of free water loss.
o Hyperkalemia - because of an extracellular shift of potassium
caused by insulin deficiency, hypertonicity, and acidemia.
Patients with low normal or low serum potassium concentration
on admission have severe total-body potassium deficiency and
require careful cardiac monitoring and more vigorous potassium

replacement because treatment lowers potassium further and

can provoke cardiac dysrhythmia.
o Hyperphosphatemia- due to shifts of intracellular phosphate to
the extracellular space.
o High anion gap (Indicating Acidosis)
 The anion gap is a measurement of the difference-or
gap-between the negatively charged and positively charged
electrolytes.
 Anion gap = (Na+) - [(Cl- + HCO3- (mEq/l)]
 Normal Range is between 3-11 mEq/L
 EKG – Prolonged Corrected Q-T interval due to the hypokalemia
 Urea and Creatinine are can be elevated in Prerenal Azotemia due
to the dehydration.
 Chest Radiograph – to look for chest infection
 Lipid Profile will show Hyperlipidemia characterized by elevated
triglycerides and lowdensity lipoprotein cholesterol levels is
commonly seen in patients with T2DM at diagnosis.
Screening Guideline for Complications of diabetes mellitus in

childhood

WHEN TO FREQUENCY PREFERRED METHOD POTENTIAL

COMMENCE OF SCREENING INTERVENTION
SCREENING
Retinopathy After 5 yr duration 1-2 yearly Fundal photography Improved glycemic

in control, laser
prepubertal therapy
children,
after 2 yr in
pubertal
children
Nephropathy After 5 yr duration Annually Spot urine sample for Improved glycemic
in albumin:creatinine control, blood
prepubertal ratio pressure control,
children, ACE inhibitors
after 2 yr in
pubertal
children
Neuropathy Unclear in Unclear Physical examination Improved glycemic

children; control
adults at diagnosis
in
T2DM and 5 yr
after
diagnosis in T1DM
Macrovascular After age 2 yr Unclear Lipids Statins for

Disease hyperlipidemia
Blood pressure
control
Thyroid Disease At diagnosis Every 2-3 yr or Lipids Thyroxine

more
frequently
based
on symptoms
or
the presence of
antithyroid
antibodies
Celiac Dieses At diagnosis Every 2-3 yr Tissue transglutaminase, Gluten-free diet

endomysial antibody

CRITERIA TESTING FOR TYPE 2 DIABETES IN CHILDREN
 Overweight (body mass index >85th percentile for age and sex,
weight for height >85th percentile, or weight >120% of ideal for
height)
Plus Any 2 of the following risk factors:
o Family history of type 2 diabetes in 1st- or 2nd-degree relative
o Race/ethnicity (Native American, African-American, Hispanic,
Asian/Pacific Islander)
o Signs of insulin resistance or conditions associated with insulin
resistance (acanthosis nigricans, hypertension, dyslipidemia,
polycystic ovary syndrome)
 Age of initiation: age 10 yr or at onset of puberty if puberty occurs at
a younger age
o Frequency: every 2 yr
o Test: fasting plasma glucose is preferred
C. Central Diabetes Insipidus (Neurogenic)

 polyuria and polydipsia and resulting from vasopressin (ADH)
deficiency.
 Etiology and Presentation
oCauses may be acquired as
 brain tumors,
 head trauma,
 granulomatous diseases and
 autoimmunity.
oIt may also be inherited as Autosomal Dominant.
oDrugs like ethanol, phenytoin, opiate antagonists, halothane,
and α-adrenergic agents can inhibit the rerelease of ADH
oUsually the urine volume is very high (> 8–10 L/day). Polydipsia
is usually a feature and
is very troublesome.
D. Nephrogenic Diabetes Insipidus

Nephrogenic diabetes insipidus occurs due to nonresponse of kidneys
to ADH which may be Congenital or Acquired.

 Presentation
Congenital
 Usually affects males, though women can transfer the gene to
their child.
 The polyuria and polydipsia associated with genetic NDI usually
occur within the 1st several weeks of life, but may only become
apparent after weaning or with longer periods of nighttime sleep.
 Many infants initially present with fever, vomiting, and
dehydration.
 Failure to thrive may be secondary to the ingestion of large
amounts of water, resulting in caloric malnutrition.
 Longstanding ingestion and excretion of large volumes of water
can lead to nonobstructive hydronephrosis, hydroureter, and
megabladder
Acquired
 Acquired NDI can result from hypercalcemia or hypokalemia and

is associated with lithium, demeclocycline, foscarnet, clozapine,
amphotericin, methicillin, and rifampin.
 Impaired renal concentrating ability can also be seen with
ureteral obstruction, chronic renal failure, polycystic kidney
disease, medullary cystic disease, Sjögren syndrome, and sickle
cell disease.
 Decreased protein or sodium intake or excessive water intake, as
in primary polydipsia, can lead to diminished tonicity of the
renal medullary interstitium and NDI.
 They will usually complain of extreme thirst, Frequent need to
get up to urinate during the night and preference to cold water.
 Complications
oDehydration which manifest as
 Dry mouth
 Sunken Eyeball
 changes in skin elasticity
 Thirst
 Fatigue
 Electrolyte Imbalance which can manifest as

 Weakness
 Nausea
 Vomiting
 Loss of appetite
 Muscle cramps
 Confusion
Other Types of Diabetes Insipidus

 Gestational diabetes insipidus. Gestational diabetes insipidus
is rare. It occurs only during pregnancy when an enzyme made
by the placenta destroys ADH in the mother.
 Primary polydipsia. Also known as dipsogenic diabetes
insipidus, this condition can cause production of large amounts
of diluted urine. The underlying cause is drinking an excessive
amount of fluids. Primary polydipsia can be caused by damage
to the thirst-regulating mechanism in the hypothalamus. The
condition has also been linked to mental illness, such as
schizophrenia.
Investigations
 Water Deprivation Test
oThis test measures changes in body weight, urine output, and
urine composition when fluids are withheld. Also measuring
blood levels of ADH during this test is necessary.
oDecreased ADH to serum osmolality ratio indicates CDI and
increased ratio means it is NDI.
 Magnetic resonance imaging (MRI).
oAn MRI can look for abnormalities in or near the pituitary gland.
This test is noninvasive. It uses a powerful magnetic field and
radio waves to construct detailed pictures of brain tissues.
 Genetic screening.
oIf others in your family have had problems with excess
urination, your doctor may suggest genetic screening.

Sample History
Identification
This is a 14-year old male student
PA – None
CC – Excessive urination Of 2 weeks Duration
HPI – He was relatively healthy till two weeks back where he started to
experience excessive urination which was out of his usual frequency. He
estimates the amount to be around 3L and frequency of 8-10 times a day.
At first he was having this symptom only during the day time but in the
past week he was waking up from sleep 2 or more times in a single night.
Associated with this, he have was feeling thirsty all the time even if he
have been drinking his usual amount of water and he had also
unspecified but significant weight loss despite his good appetite.
His mother is a known diabetic patient and have been on follow up for
the past 20 years.
Otherwise
He has no history of Trauma or surgery to the head.
He has no history of fever, headache or abnormal body movement.
He has no history of any drug intake with in the past weeks.
He has no history of loss of consciousness.
He has no history of body swelling
He has no history of urine color change, pain during urination or lower

abdominal pain,
He has no personal habits of drinking alcohol.
He has no history of nausea or vomiting.

CHAPTER 12
APPROACH TO DIARHEA
Definition
Diarrhea is best defined as excessive loss of fluid and electrolyte in the
Stool.
Subjectively, Diarrhea is the passage of loose or watery stools at
least three times in a 24-hour period.
Objectively, diarrhea is defined as stool volume of more than 20
grams/kg/day in infants and toddlers (<10 kg), or more than 200
grams/day in older children or teenagers
Diarrhea is an increased volume, fluidity, or frequency of stool
relative to the usual pattern of individual. (WHO)
Pathophysiology
 The basis of all diarrheas is disturbed intestinal solute transport
and water absorption.
 Disorders that interfere with absorption in the small bowel tend to
produce voluminous diarrhea,
 whereas disorders compromising colonic absorption produce
lower-volume diarrhea.
The explanations for the pathogenesis are:
1.SECRETORY DIARRHEA
 Occurs when solute transport system is in an active state of

secretion by:
o a secretagogue - gastrointestinal peptides (such as
vasoactive intestinal peptide and gastrin),
o cholera toxin
 Decreased absorption
 Persists during fasting
 on stool examination
o no stool leukocytes

o Watery, normal osmolality with ion gap <100 mOsm/k

 Example
o Cholera, toxigenic Escherichia coli; carcinoid, VIP,
neuroblastoma, congenital chloride diarrhea, Clostridium
difficile, cryptosporidiosis (AIDS)
2.OSMOTIC DIARRHEA
 occurs after ingestion of a poorly absorbed solute. The solute can

be:
o not well absorbed (magnesium, phosphate, lactulose, or
sorbitol)
o not well absorbed due to the disorder of the small bowel
(lactose with lactase deficiency, glucose with rotavirus
diarrhea)
o lesser volume than a secretory diarrhea and stops with
fasting.
o On stool examination
 no stool leukocytes
 Watery, acidic, reducing substance and increased
osmolality with ion gap >100 mOsm/kg.
3.MOTILITY DISORDER
 Can be increased or decreased motility

 Increased: the diarrhea is due to decreased transit time.
o Loose to normal appearing stool, stimulated by gastrocolic
reflex
o Ex: Irritable bowel syndrome, thyrotoxicosis,
postvagotomy dumping syndrome.
 Decreased: the diarrhea is due to Defect in neuromuscular unit(s)

stasis (bacterial overgrowth)
o Loose to normal appearing stool
o Pseudo obstruction, blind loop
4.DECREASED SURFACE AREA
 The diarrhea is due to Decreased functional capacity

 The diarrhea is watery in consistence

o Ex: Short bowel syndrome, celiac disease, rotavirus
enteritis
5.MUCOSAL INVASION
 The diarrhea is due to Inflammation, decreased colonic

reabsorption, increased motility
 Blood and increased WBCs in stool
o Example: Salmonella, Shigella infection; amebiasis;
Yersinia, Campylobacter infection.
Classification
Based on onset and duration diarrhea is classified as:
1. ACUTE DIARRHEA: is defined as sudden onset of excessively loose
stools of >10 mL/kg/day in infants and >200 g/24 hr in older
children, which lasts <14 days.
 Normally in young infant ~ 5 mL/kg/day of stool and ~ 200
g/24 hr in an adult.
DYSENTERY is a term for small volume, frequent bloody stools
with mucus, tenesmus, and urgency. It is the predominant
symptom of colitis.
2. CHRONIC DIARRHEA: last >14 days with gradual onset.
 typically associated with serial enteric infections and
malnutrition;
 comorbid conditions, such as HIV/AIDS, malaria, or tuberculosis,
result in malnutrition that impairs the child’s immune response,
thereby potentiating the likelihood of prolonging diarrhea or
acquiring another enteric infection.
Example:
o Escherichia coli and Giardia lamblia
o Clostridium difficile or cytomegalovirus (opportunistic
agents in oncologic patients and in inflammatory bowel
diseases)
o Cryptosporidium (in AIDS patients)

3. PERSISTENT DIARRHEA: lasts > 14days but starts as acute diarrhea.

Further classified as:
 severe persistent diarrhea (with any kind of dehydration)
and
 persistent diarrhea (without dehydration).
Dehydration: is majorly a complication of acute diarrhea. Is classified

as no dehydration, some dehydration, and severe dehydration.
Differential diagnosis of diarrhea

Cause Infants and young children Older children and
adolescents
Gastrointestinal Viruses Viruses
infections
Bacteria Bacteria
Parasites Parasites
Non-gastrointestional Otitis media Systemic infections
infections (parenteral
diarrhea) Urinary tract infections
Other systemic infections
Anatomic Intussusception Appendicitis

abnormalities
Hirschsprung disease (± Partial obstruction
toxic megacolon)
Blind loop syndrome
Partial bowel obstruction
Blind loop syndrome
Intestinal lymphangiectasis
Short gut syndrome
Inflammatory bowel Ulcerative colitis (± toxic

disease megacolon)
Crohn's disease (± toxic

megacolon)
Malabsorption or Cystic fibrosis Celiac disease

increased secretion
Celiac disease Disaccharidase deficiency
Disaccharidase deficiency Acrodermatitis enteropathica
Acrodermatitis enteropathica Secretory neoplasms

Immunodeficiency Severe combined Human immunodeficiency
immunodeficiencies and virus infection (HIV)
other genetic disorders
Human immunodeficiency
virus infection (HIV)
Endocrinopathy Congenital adrenal Hyperthyroidism

hyperplasia
Hypoparathyroidism
Miscellaneous Antibiotic-associated Antibiotic-associated diarrhea
diarrhea
Pseudomembranous colitis
Pseudomembranous colitis
Toxins
Toxins
Irritable bowel syndrome
Hemolytic uremic syndrome
Psychogenic disturbances
Neonatal drug withdrawal

Acute Gastroenteritis
Definition
 It is an infection of the gastrointestinal tract caused by bacterial,
viral, or parasitic pathogens.
Diarrheal disorders are more commonly used to denote infectious
diarrhea, although several noninfectious causes with vomiting and/or
diarrhea are well recognized.
Epidemiology
 Accounts 9% of childhood deaths, with an estimated 0.71 million
deaths per year globally, making it the second most common
cause of child deaths worldwide.
 Preventive rotavirus vaccination, improved case management of
diarrhea, and improved nutrition of infants and children have
decreased diarrheal mortality (However, no significant changes in
incidence)
Etiology
Transmision is:
 By person-to-person contact (those that are infectious by small
inoculum): Shigella, enterohemorrhagic Escherichia coli,
Campylobacter jejuni, noroviruses, rotavirus, Giardia lamblia,
Cryptosporidium parvum, Entamoeba histolytica
 By contaminated food or water supply: cholera
 VIRAL CAUSES
 rotavirus The most common

 noroviruses
 sapoviruses,
 enteric adenoviruses
 astroviruses.
 BACTERIAL CAUSES

 Salmonella,
 Clostridium perfringens, The most common
 Campylobacter
 Staphylococcus aureus,
 E. coli, Clostridium botulinum, Shigella, Cryptosporidium,
Yersinia, Listeria, Vibrio, and Cyclosporaspecies, (in that
order)
Approach
History
SYMPTOMS:
 Characterize the diarrhea: its duration (acute or chronic),

frequency, and character (if bloody or not)
 The most common are:
 diarrhea,
 abdominal cramps, and
 vomiting
 nausea and vomiting (nonspecific symptoms) - upper intestine
infection
 Fever - inflammatory diarrhea and also dehydration or coinfection
(e.g. urinary tract infection, otitis media)
 Severe abdominal pain and tenesmus - the large intestine and
rectum.
Nausea and vomiting and absent or low-grade fever with mild to

moderate periumbilical pain and watery diarrhea - small intestine
involvement and reduce the likelihood of a serious bacterial infection.
RISK FACTORS
 poverty (ask family income)

 poor personal and environmental hygiene (ask personal
experience of washing hand after toilet and washing of food
preparing materials and hands properly before preparing food)
 Malnutrition –

NB: There is a vicious cycle relation between diarrhea and

malnutrion. (so ask properly to know which one is the cause for
the other, meaning whether the diarrhea (infection) or the edema
(malnutrition) comes first?)
 Bottle feeding
 Lack of immunization (rota virus)
Physical examination — Assessment of a child with acute diarrhea
should include evaluation of the following:
G/A acute sick looking (severe abdominal pain)

V/S
 Temperature —
 Fever is common in diarrheal illness.

 fever or hypothermia with watery diarrhea should raise
clinical suspicion of a comorbid illness. (Example,
malaria in endemic area)
 Pulse rate — weak pulse, tachycardia (due to dehydration and

hypovolemia)
 Respiratory rate — tachypnea
 Blood pressure — decreased (sign of dehydration and then
hypovolemic shock)
HEENT - sunken eyeball (sign of dehydration)
 R/S — Tachypnea can be a sign of pneumonia in the setting of
cough or difficulty breathing. Children with dehydration should
be reassessed for pneumonia following initial rehydration.
 ABDOMEN — cylindrical abdominal mass is palpable (in the case
of Intusseception)
 IS - check for skin pinch (sign of dehydration)
 CNS —
 Irritability (in moderate dehydration)

 lethargy and coma (in severe dehydration)
 Encephalopathy or seizures - in Shigella and Salmonella

The differential diagnosis of seizures in a child with diarrhea includes

hypoglycemia, hyponatremia, hypernatremia, encephalopathy,
meningitis and febrile seizures.
CLINICAL ASSESSMENT
The assessment of the child with diarrhea can be divided into four
components to guide clinical management:
 Classification of the type of diarrheal illness
Classification Signs or symptoms Treatment

No dehydration Not enough signs to  Give fluid and food to
classify as some or treat diarrhea at home
severe dehydration (see diarrhea treatment
plan A)
 Advise mother on when
to return immediately)
 Follow up in 5 days if not
improving.
Some Two or more of the  Give fluid and food for
dehydration following signs: some dehydration
■ restlessness, (treatment plan B)
 5 to 10 percent irritability  After rehydration, advise
dehydration ■ sunken eyes mother on home
■ drinks eagerly, thirsty treatment and when to
■ skin pinch goes back return immediately
Slowly  Follow up in 5 days if not
improving
Two or more of the  Give fluids for
Severe following signs: severe
dehydration ■ lethargy or Dehydration (treatment
unconsciousness plan C)
 >10 percent ■ sunken eyes
dehydration ■ unable to drink or
drinks poorly
■ skin pinch goes back
very slowly
Prepared by Jimma University (≥2 s) of 2008 E.C. Batch
Medical Students
 Assessment of hydration status

 Assessment of nutritional status
 Assessment of co-morbid condition
1. CLASSIFICATION OF DIARRHEA — as acute, chronic, persistent or

dysentery.
2. HYDRATION STATUS — The degree of dehydration should be
assessed at presentation based on physical signs and
symptoms.
The World Health Organization (WHO) has issued recommendations

for assessing dehydration based on four clinical signs.
3. NUTRITIONAL STATUS — Because, recurrent diarrhea in childhood

is associated with malnutrition, which contributes to delays or
irreversible deficits in physical and cognitive development.
NB: Children with acute diarrhea and malnutrition are at

increased risk for developing fluid overload and heart failure
during rehydration.
4. DIAGNOSTIC STUDIES — Most children with acute diarrhea do not

require laboratory testing, although in complex cases some
laboratory studies may be useful:
 GLUCOSE AND ELECTROLYTE ASSESSMENT – if with seizures or
altered consciousness
 RELEVANT INVESTIGATIONS - for suspected pneumonia, sepsis,
meningitis, urinary tract infection or HIV infection
 IMAGING STUDIES - for patients with acute abdominal findings
on physical examination.
 MICROSCOPY - for presumptive diagnosis of two important
causes of gastroenteritis:
 Cholera (using dark field microscopy to detect motile
Vibrios, which appear as "shooting stars")
 Amoebic dysentery (using direct microscopy to
detect Entamoeba trophozoites containing red blood
cells)

 Microbiology laboratory evaluation - for invasive diarrhea

(dysentery) who do not respond to empiric antibiotic
therapy.
COMPLICATION
 Dehydration
 Hypovolemic shock
 Malnutrition
 Secondary infection
 Micronutrient deficiency (iron, zinc, vitamin A)
 Based on the pathogen different extraintestinal manifestations.
Treatment
PRINCIPLE OF MANAGEMENT
oral rehydration therapy

enteral feeding and diet selection
zinc supplementation
and additional therapies such as probiotics.
1. REHYDRATION THERAPY: FLUID AND ELECTROLYTES — Fluid

management consists of two phases: replacement and
maintenance.
 Replacement therapy
 is to replenish deficits in water and electrolytes lost.
continued until all signs and symptoms of diarrhea are absent

and the patient has urinated; ideally this is achieved during
the first four hours of therapy.
 Maintenance therapy counters ongoing losses of water and

electrolytes; this phase is continued until all symptoms resolve.
 The approach to fluid and electrolyte management depends on
the degree of dehydration:

 NO SIGNS OF DEHYDRATION;
 do not require a replacement phase and can begin

maintenance therapy.
 usually do not require hospital admission
 Ideally ORS is administered for maintenance fluids to
counter ongoing fluid and electrolyte losses.
 SOME DEHYDRATION
 Replacement therapy with ORS in a supervised

setting. If ongoing stool losses (Measured) are
profound, these losses can be added to the initial
amount of fluids given over the first four-hour period.
Ideally stool output is measured by collecting stool using a cholera cot.

Alternatively, stool output can have estimated as 10 to 20 mL/kg of
body weight for each diarrheal stool.
 Maintenance fluids begins once dehydration has

been corrected,
 SEVERE DEHYDRATION
 Manage urgently with IV fluids in a hospital setting.

 The goal of rehydration with intravenous fluids is to
stabilize the circulation immediately.
 For developing settings:
 the WHO recommends that a bolus of isotonic
crystalloid fluid of 30 mL/kg given over 30
minutes (or one hour in infants <12 months)
 followed by additional isotonic fluids to
correct the bulk of the remaining fluid deficit, by
giving 70 mL/kg of isotonic crystalloid over 2.5
hours (or 5 hours for infants).

NB: Crystalloid fluids such as Ringers’ Lactate solution or

normal saline are used. Colloids, blood products, or hypotonic
fluids can be harmful and should NOT be administered since these
may cause fluid shifts which exacerbate fluid loss in the cellular
compartment.
Rehydration for Malnourished children - refer on SAM

management
2. NUTRITION — The goal of nutritional management for patients

without malnutrition is to encourage sufficient feeding both during
and after the diarrheal illness episode to prevent development of
malnutrition and chronic enteropathy.
 FOR INFANTS WITH DIARRHEA, once rehydration is completed:
oBreastfeed or,
oIf they are not breastfed, encourage them to continue to take
undiluted formula at least every three hours, in addition to
ORS.
 FOR CHILDREN WITH DIARRHEA, encourage them to take solid
foods immediately after initial dehydration is corrected.
o AS LONG AS DIARRHEA PERSISTS, foods high in energy content
and micronutrients should be offered at frequent intervals (at
least six meals a day).
o AFTER DIARRHEA RESOLVES, at least one extra meal per day
should be continued for a minimum of two weeks, or until the
patient regains normal weight-for-height.
 In children with severe malnutrition, refer on SAM management.
VITAMINS AND MINERALS:
3. ZINC SUPPLEMENTATION

 Several studies have demonstrated that zinc supplementation

reduces the severity and duration of diarrhea and reduces
the incidence of subsequent episodes of diarrhea for several
months.
 Based on these studies, the WHO recommends zinc for children
under 5 years of age with diarrhea (10 mg/day for under 6
months and 20 mg/day for 10 days for 6 months to 5 years).
4. OTHERS
 VITAMIN A SUPPLEMENT —
 Because children with diarrhea in developing countries are

at high risk of vitamin A deficiency.
 If patients with signs of xerophthalmia, severe malnutrition,
or a history of measles (a three dose series of repeated
treatments)
 Antibiotics — are not indicated for most children with acute

watery diarrhea; suspected cholera is an important exception in
which antibiotic therapy is useful.
Dysentery treatment
 Includes correction of fluid and electrolyte losses, appropriate

nutritional care, and treatment of the underlying cause of illness.
 The management of fluids and nutrition is as described in the

preceding sections.
 Empiric antibiotic therapy for acute bloody diarrhea should
be targeted against Shigella species. It reduces the duration of
fever and diarrhea, decreases the duration of bacterial
shedding, and the risk of life threatening complications of
infection (bacteremia).

NB
Sodium loss
o Fluid loss in acute watery diarrhea can be isonatremic,

hyponatremic, or hypernatremic.
o Corrected by ORS gradually (has advantage of reducing the risk
of the neurologic complications due to rapid shifts in
osmolarity that may occur with IV fluids)
Potassium loss
o Stool potassium losses commonly result in hypokalemia.
o manifests with muscle weakness, paralytic ileus or arrhythmia.
o replaced using ORS
PREVENTION — WHO recommendations to prevent diarrhea include:
 Exclusive breastfeeding until age six months, and continued

breastfeeding with complementary foods until 2 years of age.
 The consumption of safe food and water. Boiling water.
 Handwashing after defecating, disposing of a child's stool, and
before preparing meals.
 The use of latrines; these should be located more than 10 meters
and downhill from drinking water sources.
 Immunizations — WHO strongly recommended rotavirus
vaccine in countries where diarrheal deaths account for ≥10
percent of mortality among children aged <5.

Sample history for AGE
C/c: diarrhea and vomiting of 4 days duration
HPI:
This a 9 and ½ month male infant. He was relatively healthy 4 days

back until he developed vomiting and diarrhea of 4 days duration. The
vomiting is non-projectile, non-blood tingled, non-foul smelling, and
bilious with 3-4 episodes per day. The diarrhea is watery, non- blood
tingled and yellowish to brown in color with 4-5 episodes per day.
Associated to this he has lost his appetite. In addition, the pt has hx
of sunken eye ball(dehydration) which is first noticed by his mother 3
days back. His mother also complains that he has hx of fever 0f 2 days
duration. (sign of infection)
For the above complaints he was taken to yabu local health center
where he was given ORS and referred to JMC for better management.
Otherwise
- he has no hx of change in urine output dehydration

- has no hx of use drugs (antibiotics clostridium deficil)
- has no hx of generalized body swelling (SAM hygiene RF for
AGE)
- there is no cholera outbreak in the society
He has been on exclusively breast fed for 6 months. He was fed 6-8 x
per day. At 6th month he started a complementary feeding with a
diluted cow milk, gruel made of barley, potato and fruits like banana,
mango and orange. While he started the complimentary food the
feeding was with bottle. (SAM and BOTTLE feeding hygiene RF for
AGE)
His mother claims that she washes her hand and food preparing
materials before she cooks food. (personal hygiene hygiene RF for
AGE)

He has been exposed to sun light starting from the age of 1 month,
necked and without lubricating agent.
His father is a farmer and his mother is a house wife. They produce
about 85 Kesha of coffee per year.
They live in a house of 3 room and 3 windows with a kitchen and

latrine separated from the main house. The latrine is 10 m away from
the main house. (poverty and environmental hygiene RF for AGE)

CHAPTER 13
APPROACH TO ABNORMAL BODY
MOVEMENT
Approach
History
1. CHARACTERIZATION OF THE ABNORMAL BODY MOVEMENT
 Duration
 Mode of onset and its progression
 Frequency
 Time of occurrence
 State of consciousness
 Generalized or focal onset
 If it is focal from which part does it starts from face or
extremity.
 Aura: - sensory experiences reported by the patient not
observed externally.
 It could be visual, olfactory, auditory, déjà vu, tingling,
chest tightness, epigastric pain, fear.
 Child behavior before the event like cyanosis, urine on cloth.
 Vocalization
 Posture of the patient
 Automatism: -automatic semipurposefull movements.
 Example: chewing, salivation, dilation of pupils,
flushing, lip smacking, picking at clothes.
 Postictal state
 Sleep, headache, hemi paresis, aphasia.
2. DETERMINIG THE TYPE OF ABNORMAL BODY MOVEMENT (see below)

3. IDENTIFYING THE UNDERLYING ENTITY

 History of personality change or symptoms of increased

intracranial pressure can suggest an intracranial tumor.
 History of cognitive regression can suggest a degenerative
or metabolic disease.
 Certain medications such as stimulants or antihistamines,
can precipitate seizures.
 Any evidence of active CNS infection.
 Any history of trauma.
4. RISK FACTOR FOR THE UNDERLYING CAUSE
5. PREVIOUS SIMILAR ATTACK
6. FAMILY HISTORY
7. IMMUNIZATION HISTORY
8. DETAILED PRENATAL HISTORY (ASPHYXIA, JAUNDICE, MENINGITIS)
9. DETAILED DEVELOPMENTAL HISTORY
 developmental delay can suggest etiologic congenital or

perinatal brain dysfunction.
TYPES OF ABNORMAL BODY MOVEMENT (DDX)
1. Seizure
2. Sydenham chorea
3. Spasm
4. Tremor
5. Paroxysmal vertigo
6. Syncope Seizure mimicking events
7. Psychogenic seizures
1. Seizure -
is a transient occurrence of signs and/or symptoms resulting from
abnormal excessive or synchronous neuronal activity in the brain.
 Focal (previously called partial) seizures - 40% of seizures in

children

 focal seizures with preserved awareness (previously

called simple partial seizures) - consciousness is not
impaired
 focal seizures with impaired awareness (previously
called complex partial seizures) - consciousness is
affected.
 Epilepsy - is present when two or more unprovoked seizures

occur in a time frame of longer than 24 hours.
 Epidemiology
 4-10% of children experience at least 1 seizure (febrile
or afebrile) in the 1st 16 yr of life
 life time incidence of epilepsy is 3%.
 Annual prevalence is 0.5-1.0%.
 Status epilepticus (SE):
 For generalized tonic-clonic seizures (called convulsive

SE), SE is defined as continuous convulsive activity or
recurrent generalized convulsive seizure activity without
regaining of consciousness (t1 = 5min, t2 ≥ 30 min)
 Focal seizures with impaired awareness (t1 = 10 min, t2 =
30 min) and absence SE (t1 = 10-15 min, t2 = unknown)
NB: The ILAE has refined the definition of SE to reflect:

 the time at which treatment should be initiated (t1) and
 the time at which continuous seizure activity leads to
long-term sequelae (t2) such as neuronal injury, depending
on the type of SE.
 Epilepsy Syndrome - is a disorder that manifest as one or more

of specific seizure type:
 epileptic encephalopathy is an epilepsy syndrome in
which there is a severe EEG abnormality which is thought to
result in cognitive and other impairments in the patient.

 Idiopathic epilepsy is epilepsy syndrome that is genetic or

presumed genetic; there is no underlying disorder affecting
development or other neurologic function
e.g. petit mal epilepsy
 Symptomatic epilepsy refers to an epilepsy syndrome caused
by an underlying brain disorder that may or may not be genetic
e.g. epilepsy secondary to tuberous sclerosis or to an old
stroke.
 Unknown epilepsy - designating that the underlying cause of
the epilepsy is as yet unknown
 Mechanism
1. Decreased excitability in inhibitory GABAergic interneurons,
leading to increased excitability and epilepsy.
2. Activation of metabotropic and ionotropic glutamate receptors,
as well as the tropomyosin-related kinase B receptor.
 Causes of seizure:
1. Idiopathic
2. CNS infections
4. Structural
 Meningitis  Head trauma
 Encephalitis  Brain tumor
 Brain abscess  Stroke
 Syphilis(tertiary) 5. Others
3. Metabolic
 Sepsis
 Electrolyte imbalance
(calcium, sodium,
 Drug abuse
magnesium, phosphorus)  Ingestion of toxins
 Hypoglycemia (accidental and
non-accidental)
 THERE ARE 4 TYPES OF SEIZURE based on presumed mode of
seizure onset:
1. FOCAL SEIZURE - initial activation of a system of nervous
limited to one part of hemisphere.
FOCAL AWARE SEIZURE
FOCAL SEIZURE WITH IMPAIRED AWERENESS

FOCAL TO BILATERAL TONIC CLONIC
2. GENERALIZED SEIZURE - first clinical and EEG changes

indicate synchronous involvement of all both
hemispheres.
3. Unknown onset seizure – when not enough clinical
information available to determine if the seizure is focal
or generalized.
4. Unclassified seizure
 TYPES OF FOCAL AND GENERALIZED SEIZURES:-
 Tonic:-increased tone or rigidity.

 Atonic:-flaccidity or lack of movement during convulsion.
 Clonic :-rhythmic fast muscle contraction and slightly
longer relaxation.
 Myoclonic:-shock like contraction of a muscle < 50msec
often repetitive.
 Other type of seizure: -

 ABSENCE SEIZURE: - are generalized seizures consisting of
staring, unresponsiveness, and eye flutter lasting usually
for few seconds.
 FEBRILE SEIZURE: -a seizure that occur at 38oc or higher,
that are not the result of CNS infection or any metabolic
imbalance, and in the absence of prior afebrile seizures.
(occurs b/n 6-60 months)
 SIMPLE FS: - primary generalized, usually tonic-clonic,
lasting for a maximum of 15 min, and not recurrent
with in 24hrs.
 COMPLEX FS: - is focal, more prolonged (>15 min),
and/or reoccurs within 24 hr.
 FEBRILE STATUS EPILEPTICUS: - lasting longer than 30
min.
-It is the most common type of SE in children.
 CAUSES FOR FEBRILE SEIZURE:

 Otitis media, roseola and human herpesvirus (HHV) 6

infection, shigella, or similar infections.
 In patients with febrile status epilepticus, HHV-6B
(more frequently) and HHV-7 infections - account for
one-third of the cases.
Risk factors for recurrence of febrile seizure
MAJOR
MINOR
 Age <1 yr
 Family history of febrilw
 Duration of fever <24 seizure
hr
 Family history of epilepsy
 Fever 38 - 39⁰c
 Complex febrile seizure
 Daycare
 Male gender
 Lower serum sodium at
time of presentation
 Apply ABC rule

 an assessment of the adequacy of the airway, ventilation,
and circulation.
 Checking vital sign: -temperature, blood pressure (part of ABC)
and others.
 ANTHROPOMETRY: -focus on head circumference.
 HEENT
 Eyes: - papilledema, optic neuritis, retinal hemorrhages,

uveitis, cho- rioretinitis, coloboma, or macular changes, as
well as retinal phakoma.
 LGS: -LN enlargement

 CHEST AND CVS
 symptoms of heart failure: for cardiogenic stroke

 ABDOMEN: -check for organomegally

HSM: - to know underlying metabolic or storage disease as
the cause of the neurologic disorder.
 INTEGUMENTARY
 Neurocutaneous disorder may be indicated by the

presence of vitiliginous ash leaf–type lesions using an
ultraviolet light (Wood lamp),
 of adenoma sebaceum, shagreen patches, or of retinal
phako- mas (tuberous sclerosis),
 of multiple café-au-lait spots (neurofibromatosis),
 of V1 or V2 distribution nevus flammeus (Sturge-Weber
syndrome).
 CNS
 Level of conscious: using modified GCS or Blantyre coma

score
 Posture: decorticate or decelerbrate
 Focal neurological deficit:
Hemi paresis with hyperreflexia, an equivocal Babinski sign, and a

downward-drifting of an extended arm with eyes closed, might
suggest a contralateral hemispheric structural lesion, such as a
slow-growing glioma, as the cause of the seizure disorder.
 Meningeal signs: - if meningitis

 Neck stiffness, Brudzinski and Kerning sign
INVESTIGATIONS
 Blood studies
 RBS: -hypoglycemia

 Serum electrolyte: -electrolyte imbalance as the

underlying cause.

BC: -for infectious causes.
 LP: -to rule out meningitis.
 EEG: - help predict the risk of seizure recurrence and to assess
for focal abnormalities.
 CT or MRI: -for structural underlying causes.
 ECG: - to rule out long QT or other cardiac dysrhythmias.
TREATMENT
 FEBRILE SEIZURE
 Counseling
 anti epileptic: Only acute treatment of seizure is
necessary if seizure lasts >5minutes. Otherwise long
term antiepileptic treatment is not recommended.
 anti pyretic
 treat the underlying cause of fever
 screen and treat Iron deficiency: since it is associated
with an increased risk of febrile seizures.
 Epilepsy
 Deciding on long term therapy
 Counseling
 Antiepleptics
 Status epilepticus:
 Stabilization phase (0 – 5 min) – start with ABC of life
 Initial phase (5 – 20 min) – if seizure continues give first
line antiepileptic in this timeline (after 5 min)
 Second phase (20 – 40 min)
 Third phase (40 – 60 min)

2. Sydenham chorea
 occurs in approximately 10-15% of patients with Acute
rheumatic fever
 usually an isolated, frequently subtle, movement disorder.
 Emotional labiality, incordination, poor school
performance, uncontrollable movements, and facial
grimacing,
 All exacerbated by stress and disappearing with sleep, are

characteristic.
 Clinical maneuvers to elicit features of chorea include:

1. Demonstration of milkmaid’s grip (irregular
contractions and relaxations of the muscles of the fingers
while squeezing the examiner’s fingers)
2. Spooning and pronation of the hands - when the
patient’s arms are extended.
3. Wormian darting movements of the tongue upon
protrusion
4. Examination of handwriting to evaluate fine motor
movements.
DIAGNOSIS
 is based on clinical findings with\without supportive

evidence of GAS antibodies.
3. Spasm
 stiffness and rigidity of muscle.
 usually occur in patients with tetanus.
NB: Neonatal spasms are type of neonatal seizure that are sudden
generalized jerks lasting 1-2 sec.

 distinguished from generalized tonic spells by their shorter

duration and by association with a single, very brief,
generalized discharge.
4. Tremor
 a rhythmic and oscillatory movement of a body part with a
relatively constant frequency and variable amplitude.
 caused by alternative or synchronous contractions of
antagonist muscles.
 most common of all movement disorders, occurring time
to time in normal individuals.
 Classification:
I. Static tremor
 Resting tremor: occurring at rest. most common
cause Parkinson disease and other parkinsonian
syndrome.
 Postural tremor: occurring with head and limb held
in fixed position.
II. Action tremor; remains unchanged during the course of
voluntary movement.
III. Intension tremor: increase during the course of goal
directed movement.
 Causes of tremor:
 parkinson disease  stoke

 parkinsonian syndrome  wilson's disease
 hyperthyroidism  MS
 hypoglycemia  heavy metal poisoning
 INVESTIGATION
 Blood test
 RBS
 TFT
 Screening for heavy metals

 Screening for Wilsons disease

 Brain imaging
 TREATMENT
 depending on the underlying cause.

Sample history of Abnormal body movement

C\C: Abnormal body movement of 1day duration.
HPI:
This is an eight years old female patient who was relatively
healthy 1day back; at which time she started to experience a
sudden onset of generalized flexion and extension type of
movement of both extremities. The abnormal body movement
lasted for about 1 minutes with episodes of 3-4 times a day and it
wasn’t accompanied by any kind of vocalization, up rolling of the
eye, drooling of saliva, lip smacking, chewing or picking on cloth.
There was no loss of consciousness or lose of bladder control
during the event. She also doesn't recall experiencing any strange
feeling before the occurrence of the event. After every episode the
patient was tired and slept for about 30 minutes.
Three days back she had a global type of head ache and a high
grade fever. She also had slight pain while moving her neck side to
side. For the above complaints she visited a local health center and
was referred to here for better management.
Otherwise: -
 She has no family history of similar illness. (epilepsy)

 She has no history of previous attack. (epilepsy)
 She has no history of trauma to the head. (head injury)
 She has no history of deliberate or non-intentional toxic
ingestion. (toxic exposure)
 She has no history of behavior change like sleep
disturbance. (encephalitis)
 She has no history of vomiting and diarrhea. (metabolic
abnormality)
 She has no history of body weakness. (brain tumor)
 She has no history of extremity swelling, Orthopnea and
PND. (Cardiogenic stroke)
 She has no history of drug intake. (drug intoxication)
 She has no history of URTI, skin rashes. (meningitis)

 She has no history of ear pain, discharge or implant.

(meningitis)
 She has no history of contact with chronically coughing
person. (tuberculoma)
 The mother doesn't know the sero-status of her daughter.
 She has no history of immunization. (meningitis)
- Before illness; she was active and played with her siblings. She
was enrolled to a school at the age of 4 and learned up to 2th
grade. She has a good academic performance. She socializes well
with her class mates.
- Her staple diet is injera made of teff and wot made of beans and
lentils with occasional meat consumption. She sometime eats
fruits and vegetables. She eats 3 times a day.

CHAPTER 14
APPROACH TO BODY WEAKNESS
Weakness
is a reduction in the power that can be exerted by one or more
muscles.
 Due to disorders of UMNs or LMNs
 UMNs originate in the cerebral motor cortex:
o Their axons form the CST ending in the spinal cord
o Control voluntary motor activity
 LMNs and muscle units:
o Anterior horn cells
o Their motor roots
o Peripheral motor nerves
o Neuromuscular junctions
o Muscles
 Maintenance of normal strength, tone, and coordination requires
integrated communication of:
o Cerebral Cortex
o Basal Ganglia
o Cerebellum
o Spinal Cord
o Brainstem
o thalamus
 These motor System Dysfunction leads to:
o Weakness
o Paralysis
o Ataxia
o Abnormal movements
o Loss of full control of bodily movements.
 The mode of onset, distribution, and accompaniments of weakness
help suggest its cause
 TERMINOLOGIES:
o Paralysis - severe weakness in which a muscle cannot be

contracted at all
o Paresis - mild or moderate weakness

o hemi- one-half of the body

o Mono - single of a pair of limb
o Para - both legs
o Quadri-“-all four limbs
o Plegia- -signifies severe weakness or paralysis.
 The distribution of weakness helps to indicate the site of the
underlying lesion
 UMNs Weakness occurs:
 Extensors and abductors of the upper limb
 Flexors of the lower limb
o Dysfunction of UMNs causes:
 Loss of voluntary control, but not total loss of movement
 because motor nuclei of the basal ganglia, thalamus, and
brainstem have tracts that produce simple or complex
stereotyped patterns of movement
 The corticospinal tract permits fine motor activity and is
best tested by rapid alternating movements of the distal
extremities
o In the acute phase
 Hypotonia
 Decreased deep tendon reflexes
 Spasticity
 Hyperreflexia
o Mild dysfunction
 slowed, stiff motions
o Severe dysfunction
 Stiff
 Abnormal involuntary postures
 Spasticity
o A central lesion
 results in more pronounced weakness in7 the flexors of the
lower extremities
 patients tend to be spastic with legs extended and adducted
o In the upper extremities
The extensors are weaker than flexors
Elbows and wrists are flexed
o Damage to the spinal cord
 Leaves residual simple
 stereotyped reflex movements below the level of the lesion
 LMNs Weakness
 Does not have any selectivity
 Depends on level involvement at

o Anterior horn cells

o Nerve root
o Limb plexus
o Peripheral nerve
 Destruction LMNs the final common pathway
o Hypotonia
o Total absence of movement
 Function is best tested by
o Measuring the strength of individual muscle groups
o In a young child by observing ability to perform tasks
requires _muscle groups activity
 Walk up or down stairs
 Arise from the ground
 Walk on toes or heels
 Raise the hands above the head
 Squeeze a ball
o Myopathic Weakness
 most marked in proximal muscles
Clinical signs Upper motor neuron Lower motor neuron
(corticospinal tract) (neuromuscular)
Tone Increased (spastic) Decreased
Reflexes Increased Decreased
Babinski Present Absent
reflex
Atrophy Possible Possible
Fasciculation Absent Possible

Differential Diagnosis
Anatomical regions Corresponding disorders
CNS – brain Brain tumor

Trauma (accidental, non-accidental)
Infections:
 Meningitis
 Encephalitis
 Abscess
 TORCH
Ischemia (arterial or venous)
Hemorrhage
Demyelinating disease
Metabolic disease
 leukodystophy
 inborn error of metabolism:
 lactic acidosis
 mitochondrial encecephalopathy
degenerative disease
CNS – spinal cord Transverse myelitis
Tumor
Abscess
Trauma
Infarction
Anterior horn cells Poliomyelitis
Spinal muscular atrophy
Peripheral nerve Guillain-barre syndrome
Hereditary motor sensory neuropathy
Tick paralysis
Bell palsy
Neuromuscular junction Myasthenia gravis (juvenile, transient neonatal,
congenital)
Botulism
Muscle Muscular dystrophy

Myotonic dystrophy
Congenital myopathy
Metabolic myopathy
Dermatomyositis
Polymyositis
TORCH: Toxoplasmosis, Other [syphilis, varicella-zoster, parvovirus
B19], Rubella, Cytomegalovirus, and Herpes)

A. DDX of UMN weakness in children

o Tumors
 Neuroblastoma
 Lymphoma
 sarcoma
o Traumatic brain injury
o Infections
 Meningitis
o Stroke
o Demyelinating syndromes
o Spinal cord trauma or mass
o Metabolic diseases
o Neurodegenerative diseases
B. DDX of UMN weakness in neonates
o TORCH infections
o Developmental brain anomalies
o Hemorrhage
o Stroke
o Hypoxia
o Genetic syndromes
o Metabolic/electrolyte disturbances
o Toxic exposure
C. DDX of LMN weakness
oThey are generally a Neuromuscular diseases.
Some of selected DDX of body weakness for discussion

1. Poliomyelitis
2. Guillain-Barre syndrome
3. Pediatric stroke
4. Brain Tumor in childhood
5. Transverse myelitis
6. Myasthenia gravis
7. Heavy metals, biologic toxins, Organophosphate
pesticides, Snake venom or drug intoxication
8. Brain abscess

Discussion of common differential diagnosis
1. Poliomyelitis
Definition:
The infectious disease caused by polioviruses which are nonenveloped,
positive-stranded RNA viruses belonging to the Picornaviridae family,
in the genus Enterovirus.
3 antigenically distinct serotypes:
 Types 1
 Type 2
 Type 3
Approach
History
ABORTIVE POLIOMYELITIS
 Occurs 5% of patients
 A nonspecific influenza-like syndrome occurs 1-2 wk after infection
(termed as abortive poliomyelitis)
 Main symptoms:
 Fever,
 malaise,
 anorexia, and
 headache sore throat
 Other symptoms
 abdominal or muscular pain
 irregular Vomiting
 weakness
NON-PARALYTIC POLIOMYELITIS
 In 1% of patients infected with wild-type poliovirus, signs of

abortive poliomyelitis are present
 more intense headache,

 nausea, and vomiting,
 soreness and stiffness of the posterior muscles of the neck,
trunk, and limbs.
 Fleeting paralysis of the bladder and constipation
PARALYTIC POLIOMYELITIS
 Paralysis occurs if >50% of the neurons supplying the muscles are

destroyed
 The extent of involvement is usually obvious within 2-3 days
 Paralytic poliomyelitis develops in approximately 0.1% of persons
infected with poliovirus
SYMPTOMS:
o Severe headache and fever occur with exacerbation of the

previous systemic symptoms
 Preceded by the patient recovers and feels better for 2-5
days
o Severe muscle pain,
o sensory and motor phenomena
 Paresthesia
 Hyperesthesia

POLIO ENCEPHALITIS:
 SYMPTOMS
oVery rare
oIrritability
oDisorientation
oDrowsiness
ocoarse tremors
 RISK FACTORS:
o Lack of universal vaccination

o poor sanitation
o Increasing overcrowding
o hx of IM injections (provocation paralysis) – bulbar
poliomyelitis
o Increased physical activity, exercise, and fatigue in early
phase of illness a higher risk for paralytic disease
o Surgery e.g. tonsillectomy
o Puberty
o Pregnancy
o Increased physical activity
o exercise & fatigue
 COMPLICATIONS:
oParalysis
oSkeletal deformities
oAcute gastric dilation
oMelena
operforation is rare
oMild hypertension (in acute stages)
ohypertension (in later stages) due to immobilization:
 Hypercalcemia (from skeletal decalcification)
 Nephrocalcinosis
 Vascular lesions
 Dimness of vision, headache, and a lightheaded feeling
associated with hypertension
 Hypercalciuria (result in urinary calculi)
Clinical Findings:
NON-PARALYTIC POLIOMYELITIS
 CNS:
o Sensory system
 Sensory defects do not occur in poliomyelitis
o Deep tendon reflexes
 changes in superficial and deep reflexes
 In the early stages the reflexes are normally active and remain
so unless paralysis supervenes
 Changes in reflexes, either increased or decreased, may
precede weakness by 12-24 hr
 The superficial reflexes, the cremasteric and abdominal
reflexes, and the reflexes of the spinal and gluteal muscles are
usually the first to diminish
 The spinal and gluteal reflexes may disappear before the
abdominal and cremasteric reflexes.
 Changes in the deep tendon reflexes generally occur 8-24 hr
after the superficial reflexes are depressed and indicate
impending paresis of the extremities
 Tendon reflexes are absent with paralysis
o Meningeal signs:
 Nuchal rigidity and spinal rigidity (during the second phase)
 nuchal-spinal signs
 Nuchal rigidity or true nuchal rigidity (persists with
maneuver)
ABORTIVE POLIOMYELITIS
 Normal or may reveal nonspecific:

 Pharyngitis,
 Abdominal or muscular tenderness
 Recovery is complete, and no neurologic signs or sequelae develop

PARALYTIC POLIOMYELITIS
Causing 3 clinically recognizable syndromes that represent a

continuum of infection differentiated only by the portions of the CNS
most severely affected
These are:
oSpinal Paralytic Poliomyelitis
oBulbar Poliomyelitis
oPolio Encephalitis
SPINAL PARALYTIC POLIOMYELITIS:
 The most common

 GUS
o Bowel and bladder dysfunction (from transient incontinence to

paralysis with constipation and urinary retention often
accompany paralysis of the lower limbs)
 MSS
o Spotty paralysis
o Single muscles, multiple muscles, or groups of muscles
involved in any pattern
o Asymmetric flaccid paralysis or paresis occurs (within 1-2 days)
o Involvement of one leg is most common, followed by
involvement of one arm
o The proximal areas of the extremities tend to be involved to a
greater extent than the distal area
o Muscle tenderness
o In the spinal form, there is weakness of some of the muscles of
the neck, abdomen, trunk, diaphragm, thorax, or extremities
o Lack of improvement from paralysis (permanent paralysis)
o Atrophy of the limb, failure of growth, and deformity (in the
growing child
o In most patients, flaccid paralysis occurs abruptly
o The return of strength is slow
 CNS:
O MOTOR SYSTEM
 Spasm and increased muscle tone

O SENSORY SYSTEM
 Sensation is intact; sensory disturbances, if present,

suggest a disease other than poliomyelitis
o Deep tendon Reflexes
 Initially hyperactive deep tendon reflexes (for a short
period)
 Followed by absence or diminution of reflexes, and
paresis or flaccid paralysis
 Return of reflexes is slow
o Meningeal signs
 Nuchal stiffness or rigidity
BULBAR POLIOMYELITIS:
 Clinical entity with no apparent involvement of the spinal cord

 Infection is a continuum
 The clinical findings:
 VITAL SIGNS
 BP – changed - usually increased

 Hypertension
 hypotension and shock
 PULSE RATE- irregularities- involvement of vital centers in the
medulla
 RESPIRATORY RATE -depth, and rhythm of respiration-
involvement of vital centers in the medulla
 TEMPERATURE- rapid changes in body
 HEENT:
 Dysphagia- resulting in accumulation of saliva in the pharynx,

indicating partial immobility
 Deviation of the palate, uvula, or tongue
 Paralysis of 1 or both vocal cords
 Paralysis of extraocular, facial, and masticatory
 Palatal paralysis
 the rope sign, an acute angulation between the chin and larynx
weakness of the hyoid muscles
 R/S:
 Respiratory difficulty- nasal twang to the voice or cry caused by

palatal and pharyngeal weakness

hard-consonant words such as “cookie” and “candy” bring

this feature out best
 Accumulated pharyngeal secretions - cause irregular
respirations
 irregular or failed respiratory effort
 CVS:
 cardiac arrhythmias
 GUS:
 Hypercalcemia
 Hypercalciuria
 I/S:
 Alternate flushing and mottling of the skin
 MSS:
 Atrophy of muscles
 CNS:
 Delirium
 Coma
Polio encephalitis:
 Increased reflexes
 Hypoxia and hypercapnia
 Seizures, coma, and spastic paralysis
Investigation
 Stool culture
o Two stool specimens (8-10 gm each)
o Collected 24-48 hrs. apart
o Confirm by isolation and identification of poliovirus
 PCR technique- Is a sensitive, specific, and rapid technique for the

diagnosis of enteroviral infections
o It can be performed on:

Stool
 Blood
CSF
Throat specimens
 CSF analysis
o normal during the minor illness
o Pleocytosis with 20-300 cells/μL with CNS involvement
o polymorphonuclear cells early course disease but shift to
mononuclear cells soon afterward.
o In 2nd phase:
CSF cell count falls to near-normal values
the CSF protein content is normal or only slightly
elevated
 Serologic testing:
o demonstrates seroconversion or a 4-fold or
o greater increase in antibody titers from the acute phase of
illness to 3-6 wk later.
 Nerve conduction studies and electromyogram
 Muscle biopsies
Management
 No specific antiviral treatment for poliomyelitis
 The management is supportive aimed at:
 Limiting progression of disease
 Preventing ensuing skeletal deformities
 Preparing the child and family for the prolonged treatment
required and for permanent disability
Abortive Poliomyelitis and Non-paralytic poliomyelitis
 Supportive treatment
oAnalgesics
oSedatives
oAn attractive diet
oBed rest until child’s temperature is normal
oFirm bed & footboard or splint
oHot packs & gentle physical therapy
oRe-examine 2 months later
Paralytic poliomyelitis
• Most patients require admission
• Complete physical rest in a calm room for 1-2 weeks

• Suitable body alignment - use of boards, sandbags, light splint

shells
• Change position every 3-6 hours
• Moist hot packs
• Active & passive movements
• Prevention of fecal impaction and Constipation
• Opiates & Sedatives- If there is no ventilation impairment
• Rx of bladder paralysis
o Parasympathetic stimulant
e.g. bethanechol
o Manual compression of the bladder
o Catheterization
• Adequate dietary & fluid intake
• Early orthopedist & physiatrist evaluation
Pure Bulbar Poliomyelitis

 Maintaining the airway
oAvoid risk of aspiration of saliva, food, or vomitus by:
 Using aspirators
 Gravity drainage
 Nursing on lateral or semi-prone position
 IV infusion for fluid & electrolyte balance
 Measure BP at least 2 x per day
 Tracheostomy
 Mechanical respirators
NB: All intramuscular injections and surgical procedures are contraindicated

during the acute phase of the illness, especially in the 1st wk of illness, because
they might result in progression of disease.
Prognosis
 Outcome of inapparent, abortive poliomyelitis and aseptic
meningitis syndromes is uniformly good, rare death and no
Long-term sequelae
 Outcome of paralytic disease is determined primarily by degree
and severity of CNS involvement
Prevention
 Vaccination is the only effective method of preventing
poliomyelitis.
 Hygienic measures
 Decreasing overcrowding
2. Guillain-Barre syndrome (GBS)

Definition:
Guillain-Barre syndrome (GBS) is an autoimmune disorder that is
thought to be a post infectious polyneuropathy, involving mainly
motor but also sensory and sometimes autonomic nerves.
History
 EPIDEMIOLOGY
 Affects people of all ages, commonly from 3-12 years old

 Bulbar involvement of GBS occurs in about 50% of cases
 GBS is not hereditary
 INITIAL SYMPTOMS:
 Numbness and paresthesia, followed by weakness

 Onset of weakness is preceded by approx. 10 days non-specific
GIT, respiratory tract and systemic infections
 Radicular back pain and myalgia
o Common
 Irritability
 The weakness is characterized by:
o Usually beginning in the lower extremities
o Progressively involves the trunk, the upper limbs, and finally
the bulbar muscles
o But weakness is sometimes proximally prominent
o Many patients develop facial weakness, but rarely involve
extra ocular muscles
o Weakness is essentially symmetric
 In most cases
o Weakness progresses over days or weeks, the clinical nadir
 Less than 4 weeks
o Children lose the ability to walk
 Approximately 60%
o A small proportion progress to flaccid tetraplegia
o The maximal severity of weakness is reached by 4 wk. after
onset
BULBAR INVOLVEMENT:

 Result in respiratory insufficiency

 Dysphagia and facial weakness can be
o signs of impending respiratory failure
o interfered with saliva control and swallowing,
o increased the risk of aspiration
 Vocal cord paralysis
o dyspnea or a hoarse voice
 Severe bulbar and respiratory muscle involvement can lead to
death if GBS is not recognized and treated
RISK FACTORS
 Consumption of undercooked poultry,

 unpasteurized milk
 contaminated water
 administration of vaccines against rabies, influenza, and
conjugated meningococcal vaccine
e.g. serogroup C
 infectious precursors of GBS include mononucleosis, Lyme
disease, cytomegalovirus
Guillain-Barre syndrome
o paralysis is characteristically symmetric
o sensory changes and pyramidal tract signs
o Fever, headache, and meningeal signs are less
Prepared byo Jimma
the CSFUniversity
has few cells but an
Medical elevatedofprotein
Students content
2008 E.C. Batch
The autonomic nervous system involvement will cause
 VITAL SIGNS
 BP:
 Lability of blood pressure
 Postural hypotension
 Asystole-due to severe weakness- common in young pt
 Pulse rate:
 Episodes of profound bradycardia or tachycardia
NB: Cardiovascular monitoring is important because especially early in

the disease course, when rapid progression of weakness, respiratory
insufficiency, and autonomic instability can be life-threatening.
 CNS:
 Deep Tendon reflexes
 The tendon reflexes are lost in GBS, usually early but

are sometimes preserved until later
 Areflexia is more common but hyporeflexia may be seen
 Of affected children, 10% retain their reflexes
throughout
 Motor --> acute ascending flaccid paralysis:

Criteria:
 Bilateral & symmetric usually
 Associated hypotonia & hyporeflexia even in uninvolved muscles.
Progress:
 Lower Limb --> trunk --> upper limb.
Bulbar palsy --> dysphonia, dysphagia & lost bulbar reflexes.
Respiratory muscles --> respiratory failure.
 Sensory --> mild; tender calf.
 Autonomic --> labile blood pressure & heart rate

Guillain-Barre Syndrome Subtypes:

 Acute inflammatory demyelinating polyradiculoneuropathy
(AIDP)
o Common (more in Western world)
 Acute motor axonal neuropathy (AMAN)
o Common (more in developing countries
 Acute motor and sensory axonal neuropathy (AMSAN)
 Miller Fisher syndrome (MFS/FS)
o Uncommon
Chronic inflammatory demyelinating polyradiculoneuropathy
(chronic inflammatory relapsing polyneuritis)
 Is a more chronic
 Slowly progressive, acquired inflammatory neuropathy
 With some clinical overlap with GBS
 Symptoms:
oweakness
osensory paresthesia in 64%
odevelop over more than 4-6 wk
orecur intermittently (relapsing)
oprogress slowly over periods of months to years
 Weakness is characterized by:

o generally, both proximal and distal, and variably severe
 Hyporeflexia or areflexia (almost universal)
 Motor deficits occur in 94% of cases and %,
 cranial nerve and autonomic involvement is uncommon
 Investigation for CIDP

 The cerebrospinal fluid (CSF -no pleocytosis
o CSF protein is almost always elevated
 Nerve conduction studies -slowing of nerve conduction
 Sural nerve biopsy -patchy myelin loss and focal inflammatory
changes
NB:
- Acute-onset CIDP may be difficult to distinguish from GBS
- CIDP may be difficult to distinguish from GBS with treatment-related
symptom fluctuations.
Congenital GBS (very rare)

Clinical features:

o generalized hypotonia
o weakness
o areflexia in an affected neonate
Rx: - Not always required
INVESTIGATION FOR GBS
 CSF studies: -
o CSF protein is usually elevated to more than twice the upper
limit of normal
o glucose level is normal
o no pleocytosis
o fewer than 10 white blood cells/mm3
 Bacterial cultures – negative
o High CSF protein and a lack of cellular response in a patient
with an acute or subacute polyneuropathy
 Diagnostic of GBS
o These findings not be apparent in the first week after the
onset of symptoms
 On MRI of the spinal cord in GBS
o Thickening of the cauda equina and intrathecal nerve roots
with gadolinium enhancement
o Imaging in CIDP is similar but demonstrates greater
enhancement of spinal nerve roots
 Nerve conduction studies and electromyography
o Early signs of peripheral nerve inflammation in GBS
 Electromyography
o Acute denervation of muscles
 Serum creatine kinase
o mildly elevated or normal
 Serum antiganglioside antibodies against GM1 and GD1
o Elevated in GBS
 Sural nerve biopsy
o Segmental demyelination, focal inflammation, and Wallerian
degeneration
 Serologic testing for Campylobacter and Helicobacter infections
o To establish causation
TREATMENT (RX)
 Supportive care: -
o Hospitalization

o Cardiac monitoring
o Nasogastric feeding
o Care of bladder
 Catheterization & neostigmine
o Physiotherapy
o respiratory support
o prevention of pressure sores
o nutritional support
o pain management -example:
 Neuropathic pain in GBS should be treated
aggressively
 by narcotic analgesics
o prevention of deep vein thrombosis
o treatment of secondary bacterial infections
Specific treatment
o IVIG: 0.4gmlkg/day for 5 days or 1g/kg/day for 2 days
o Alternatives: - Plasmapheresis / immunosuppressive drugs is
equally effective as IVIG
o Combined IVIG and interferon is effective in some patients
o Steroids are not effective for weakness but may help with pain
o CIDP can be treated with either oral or pulsed steroids or IVIG

Prognosis
 GBS is usually a monophasic illness
 Spontaneous recovery begins within 2-3 wk but can take months
 Therapy with IVIG hastens recovery but not does alter the
long-term outcome
 As many as 60% become nonambulant during their illness, but
most eventually regain full strength
 A minority has some residual weakness, most often of the ankle
dorsiflexors
 Children with demyelinating forms of GBS generally recover more
quickly than those with axonal forms
 The tendon reflexes are usually the last function to recover

 Improvement usually follows a gradient opposite the direction of

involvement, with bulbar function recovering first and
lower extremity weakness resolving last
 Bulbar and respiratory muscle involvement can lead to death if
the syndrome is not recognized and treated.
 Fatigue is the most common long-term residuum of GBS
 Relapses occur in about 4% of children with GBS and are
generally responsive to immunomodulatory treatment.
Pediatric Stroke
 An important cause of acquired brain injury in:
o Newborns
o Children
o adolescents
 Arterial ischemic stroke (AIS) and cerebral sinovenous
thrombosis (CSVT) are, together, more common than brain
malignancy
o Incidence ~ 5 in 100,000 children per year
 Is more common (1 in 2,500-4,000 live births)
 Is the leading cause of hemiparetic cerebral palsy
 A similar number of children have hemorrhagic stroke (HS)
Note: Acute stroke is a neurologic emergency
 The incidence is 2.5-10 per 100,000 children and is higher

among neonates
 CSVT may be more common in children than in adults, and
risk is greatest in the neonatal period
APPROACH
HISTORY
 SYMPTOMS OF AIS:
o Acute onset of focal neurological deficits in a child is

stroke(AIS)
o Visual, speech, sensory, or balance deficits
o Focal seizures are quite common (AIS)
o Neonatal AIS most often presents with acute
symptomatic focal seizures
o Typically, in the first day of life or encephalopathy.

O SYMPTOMS OF CSVT:
o Progress more gradually

o Be more variable
o Nonspecific than AIS
o Acute focal deficits may be present or
o The child may have progressive signs of elevated
intracranial pressure:
 Headache
 Diplopia -most often from CN VI palsy
 Seizures
 Confusion
O SYMPTOMS OF HS:
o Present acutely, with a sudden “thunderclap” headache

o Loss of consciousness
o Focal deficits
o Seizures
RISK FACTORS:
o Catheterization-AIS
o Surgical repair-AIS
e.g. AIS complicates approximately 0.5% of pediatric
cardiac surgeries
o Sentricular assist device use-AIS
o Reoperation increases the risk-AIS
o Migraine, acute childhood illnesses, chronic systemic
illnesses-AIS
o Illicit drugs and toxins-AIS/CVST
o Drugs/toxins (cocaine, amphetamine)-HS
o Trauma -HS e.g
Subarachnoid hemorrhage
Hemorrhagic contusions (coup and contrecoup)
Nonaccidental trauma (subdural hematomas of
different ages)
o Hereditary prothrombotic states and prothrombotic
medications -AIS/CVST
 e.g. factor V Leiden

o Acquired prothrombotic states and prothrombotic

medicationa
e.g., antiphospholipid antibodies, lipoprotein-a
elevation, asparaginase, oral contraceptive
o Maternal, prenatal, perinatal, obstetric, and neonatal
factors - Perinatal stroke
e.g. infertility, primiparity, multiple gestation
o Pregnancy/puerperium -CVST
o Dehydration -CVST
e.g., gastroenteritis, neonatal failure to thrive
o Otitis media, mastoiditis, bacterial meningitis, sinusitis,
dental abscess, pharyngitis-CVST
o Sepsis-CVST
o Trauma: skull fractures, closed head trauma-CVST
o Compression: birth, occipital bone compression in
neonates in supine lying-CVST
COMPLICATIONS
o Brain shift
o Increased intracranial pressure
o Herniation
o Duret brainstem hemorrhages
o Death
o Cardiac and pulmonary complication - Systemic effects of
subarachnoid hemorrhage
o Delayed neurologic complications
 PHYSICAL EXAMINATION
 Lethargy
 Nuchal rigidity
 Papilledema
 Hemiparesis is most common
ETIOLOGY
ARTERIAL ISCHEMIC STROKE [AIS]
 Due to ischemia
 Focal brain infarction
 Results from occlusion of the arteries in the brain

 Causes:
o Arteriopathy
 Idiopathic arterial stenosis
 Vasculitis (autoimmune or infectious; may be focal
or diffuse) *
 Arterial dissection (traumatic or spontaneous)
o Cardiac*
 Cyanotic congenital heart disease
 Valvular disease
 Patent foramen ovale
 Arrhythmias
 Cardiomyopathy
 Infective endocarditis
o Hematological
 Sickle cell anemia*
 Iron-deficiency anemia
 Hypercoagulable state
 Hereditary prothrombotic states (factor V Leiden)
 Acquired prothrombotic states (antiphospholipid
antibodies)
 Prothrombotic medications (oral contraception)
 CEREBRAL SINOVENOUS THROMBOSIS [CSVT])
 Hematological
o Hypercoagulable states
o Iron-deficiency anemia
o Severe dehydration
 Infections
o Meningitis
o Otitis media
o Mastoiditis
 Systemic disease
 Leukemia
 Inflammatory bowel disease
 Nephrotic syndrome
 Trauma
 HEMORRHAGE-
 Intraparenchymal
 Primary or secondary bleeding after AIS
 Associated with intraventricular, subarachnoid, subdural,or
epidural bleeds

 Causes:
o Head trauma (accidental or abusive)
o Vascular malformations
o Arteriovenous malformations
o Cerebral aneurysms
o Brain tumor
o Vasculitis
Note:
o Hemorrhage stroke can be rapidly fatal.
o Some early-life strokes are not recognized until later in life, when an infant
or child presents with hemiplegia. Such congenital hemiplegia becomes
increasingly apparent as infants develop.
Diagnostic testing and imaging
 CONTRAST CT VENOGRAPHY OR MR VENOGRAPHY
o Demonstrate filling defects in CSVT

 A NON -CONTRAST HEAD CT SCAN:
o Is highly sensitive to acute HS, but lumbar

puncture to exclude subarachnoid hemorrhage
o Can reveal larger, mature AIS and exclude
hemorrhage
o Acute, non-hemorrhagic stroke may not be seen on
routine CT
 MRI:
o MRI offers superior parenchymal imaging

compared with CT
o MRI is highly sensitive to even small amounts of
both acute and chronic hemorrhage
 With diffusion-weighted imaging
 Identify early small infarcts
 Acute, non-hemorrhagic stroke
 CSVT
 Magnetic resonance angiography (MRA), conventional
catheterization angiography or CT angiography

o Confirm arterial occlusion in AIS

o Can identify underlying arteriopathy, vascular
malformations, and aneurysms
 Cranial ultrasound
o Neonatal HS
Note: The diagnosis of HS relies on imaging
Treatment
 Treatment must focus on limiting secondary neuronal injury

and prevention of future strokes.
 Neuroprotection by maintaining control of:
 Temperature
 Blood pressure
 Glucose
 Seizures
 Anticoagulants (IV heparin)
oSecondary stroke prevention
 Long-term rehabilitation
Brain Tumor
Primary central nervous system (CNS) tumors are a heterogeneous
group of diseases that collectively are the most common malignancy
in childhood and adolescence
History
Epidemiology
 A male predominance is noted in the incidence of

medulloblastoma and ependymoma
 Familial syndromes associated account for
approximately 5% of cases
 The incidence of CNS tumors is highest in infants and
children ≤5 yr old
 approximately 52 cases/1 million children
 In children 0-14 yr old, the most common tumors are:
 Pilocytic astrocytoma’s (PAs)
 medulloblastoma/primitive neuroectodermal tumors
(PNETs)

 In adolescents (15-19 yr), the most common tumors are:

 pituitary/craniopharyngeal tumors and PAs
 congenital (neonatal) tumors
 During the 1st yr of life:
o supratentorial tumors predominate
 choroid plexus complex tumors
 Teratomas
 In children 1-10 yr old:
 infratentorial tumors predominate
 juvenile PA
 medulloblastoma
 After 10 yr of age
 Supratentorialtumors Again Predominate
 Diffuse Astrocytomas The Most Common
 Tumors Of The Optic Pathway
 Hypothalamus Region
 The Brainstem
 The Pineal-Midbrain Region
SYMPTOMS
 Symptoms of increased ICP:

 Vomiting
 Lethargy
 Irritability
 The classic triad of infratentorial tumors
 Headache
 Nausea
 Vomiting
 Headaches
 New onset
 Persistent (but usually <6 mo.
 Associated with either of papilledema, cognitive-behavioral
changes, seizures, focal motor deficits
 Associated with emesis
 Occur on awakening or wake the patient from sleep
 Blurred vision
 Diplopia
 Nystagmus
 Supratentorial tumors are more frequently associated with:

o Focal motor weakness

o Focal sensory changes

o Language disorders
 Suprasellar region tumors and 3rd ventricular region tumors
o Manifest initially as neuroendocrine deficits
Subacute Development of Obesity
Abnormal Linear Growth Velocity
Diabetes Insipidus
Galactorrhea
Precocious Puberty
Delayed Puberty
Hypothyroidism
 Pineal region tumors
o Nystagmus to convergence or retraction
 Spinal cord tumors and spinal cord dissemination of brain
tumors
o Bowel and bladder deficits
o Back or radicular pain
RISK FACTORS
 Cranial exposure to ionizing radiation
COMPLICATION
 Chronic neurologic deficits

e.g., focal motor/sensory abnormalities
 Seizure disorders
 Neurocognitive deficits
 e.g. developmental delays, learning disabilities
 Neuroendocrine deficiencies
e.g. hypothyroidism, growth failure, delay/absence of puberty
 Risk for secondary malignancie
 Papilledema
 Cognitive-Behavioral Changes, Seizures, Focal Motor
Deficits
 Associated With Emesis
 Cerebellar Tonsil Herniation
 Disorders Of Equilibrium
 Gait
 Coordination
 Gaze Palsy

 Multiple Cranial Nerve Palsies

 Upper Motor Neuron Deficits
E.G. Hemiparesis, Hyperreflexia, Clonus
 Focal Seizures
 Reflex Asymmetry
 Visual And/Or Afferent Oculomotor Disturbances
 Decreased Visual Acuity
 Marcus Gunn Pupil (Afferent Pupillary Defect)
 Nystagmus
 Visual Field Defects
 Paresis of Upward Gaze
 Pupillary Caliber Reactive to Accommodation but
Not to Light (Pseudo–Argyll Robertson Pupil)
 Eyelid Retraction
INVESTIGATION
 Neuroimaging
 CT scan
 MRI with and without gadolinium
 For primary brain tumors,
 Both serum and CSF measurements of
 β–human chorionic gonadotropin (β-hCG)
 α-fetoprotein (AFP)and
 placental alkaline phosphatase are used in diagnosis of
germ cell tumor
 LP is contraindicated in patients with newly diagnosed
hydrocephalus secondary to CSF flow obstruction
TREATMENT
 The treatment approach for these tumors is multimodal:

 Neurosurgery
 Radiation therapy
 Chemotherapy
 Immune therapy
Transverse myelitis
Transverse myelitis is a neurological condition in which the inflamed
spinal cord leads to lose of myelin coating from damage to nerve
fibers so that result in decreased electrical conductivity in the CNS
History

 SYMPTOMS
 Weakness and numbness of the limbs

 Deficits in sensation and motor skills
 Dysfunctional sphincter activities,
 Impaired function of the bladder and bowel
 Progresses rapidly over hours to days
 RISK FACTORS
 Bacterial Infections -mycoplasma pneumonia Lyme disease,

 Viral Infections -herpes simplex herpes zoster,
cytomegalovirus, Epstein-Barr virus, HIV/AIDS
 Vaccinations rarely e.g. against hepatitis B,
measles-mumps-rubella, diphtheria-tetanus-pertussis
 Multiple sclerosis
 Vascular etiology
 PHYSICAL EXAMINATION
o Acute symmetric paralysis of the lower limbs

o Autonomic signs of hypothermia in the affected limbs are
common
o Bladder dysfunction
o CSF is usually normal
 INVESTIGATION
 Neuroimaging
 MRI
 CT scan
 Lumbar puncture after imaging
 TREATMENT
 Treatment options also vary according to the underlying

cause
 In some cases, plasmapheresis
Myasthenia gravis (MG)

Definition

 Myasthenia gravis (MG) is a chronic autoimmune disease of the

postsynaptic endplate leading to abnormal neuromuscular
transmission or blockade
 Characterized clinically by rapid fatigability of striated muscle,
particularly extraocular and palpebral muscles and those of
swallowing
History
 Epidemiology
o The age of onset for immune-mediated MG ranges anywhere
from 11 mo to 17 yr of age
o In the prepubertal age-groups, the female: male ratio is about
1.5: 1,
o in the post pubertal age-groups, the female: male ratio is about 1:
1
 Symptoms
o In juvenile autoimmune MG
 unilateral or bilateral but usually asymmetric ptosis
 extraocular muscle weakness are the earliest and most
constant signs
 it is progressive(duration)
o Older children
 double vision
o Young children
 Hold open their eyes with their fingers or thumbs if the ptosis
is severe enough to obstruct vision
 Pupillary responses to light are preserved
 Dysphagia and facial weakness (common)
 In early infancy, feeding difficulties
 The cardinal sign of myasthenia
 Hypotonia
 External ophthalmoplegia
 Ptosis
 Weak cry
 Easy muscle fatigue generally
o In severe cases, aspiration and airway obstruction
o Poor head control because of weakness of the neck flexors
o Involvement initially may appear to be limited to
bulbar-innervated muscles, but the disease can be systemic and
progressive weakness eventually involves limb–girdle muscles
and distal muscles of the hands in many cases

o Tendon stretch reflexes may be diminished but rarely are lost

Ocular myasthenia gravis
In prepubertal patients are more likely to have ocular only myasthenia

A majority of post pubertal patients with myasthenia will have generalized
symptoms
Rapid fatigue of muscles distinguishes it from most other neuromuscular
diseases
Holding the head up from the surface of the examining table while lying supine
is very difficult and gravity cannot be overcome for more than a few seconds
Dysphagia can interfere with eating
 Risk factors
 Intercurrent infection
 Surgery
 Organophosphate chemicals
 Emotional stress
 Familial hx
 Hypothyroidism, usually Hashimoto thyroiditis
 Antibiotics:
 aminoglycosides,
 beta blocking agents
 procainamide
 Chloroquine
 Fluoroquinolones
 Complication
 Respiratory failure
 Risk of aspiration
 Transient neonatal myasthenia
 Do not tolerate neuromuscular-blocking drugs
 Fatigability, delayed motor milestones
Investigation
 Electromyography
 Diminished muscles potentials from repetitive
stimulation
 Nerve biopsy
Treatment
 mild MG require no treatment

 Cholinesterase-inhibiting drugs
 Intravenous immunoglobulin
 Plasmapheresis
Toxin Exposure
 Toxin can be:
 Heavy metals intoxication
 biologic toxins
 Organophosphate pesticides
 Snake venom or drug intoxication
 four of the World Health Organization's (WHO) “Ten chemicals of
greatest public health concern,”
 Lead
 Mercury
 Arsenic
 Cadmium,
 The most prevalent of these exposures is lead
HISTORY
 ARSINE GAS
 history of exposure
 Inhalation causes no immediate symptoms
 After a latent period of 2-24 hr exposed individuals experience
o massive hemolysis
o malaise
o Headache
o Weakness
o Dyspnea
o Nausea & vomiting
o abdominal pain
o Diarrhea
 MERCURY
o rapid onset of cough,

o dyspnea
o chest pain,
o fever, chills,
o headaches, and visual disturbance
o metallic taste, salivation, nausea, vomiting, and diarrhea

o necrotizing bronchiolitis,interstitialpneumonitis, pulmonary

edema, and death from respiratory failure-pulmonary
toxicity
o Renal dysfunction and neurologic disturbances
o Ataxia persistent weakness emotional liability
o metallic taste
o oropharyngeal burns
o nausea, hematemesis
o severe abdominal pain
o Hematochezia
o acute tubular necrosis
o cardiovascular collapse and death- from ingestion
 Chronic inorganic mercury intoxication
Classic Triad
tremor
neuropsychiatric disturbances
Gingivostomatitis
 Symptoms
o generalized pain
o paresthesia’s
o An acral (hands, feet) rash
red-pink
popular
pruritic
painful
o Anorexia
o Apathy
o Photophobia
o Irritability
o tremors
o Diaphoresis
o Insomnia
 ARSENIC GAS
o Hepatomegaly
o Pallor

o Jaundice
o extensive fluid loss and third spacing- hypovolemic shock-
Hemorrhagic gastroenteritis
o hemoglobinuria, and renal failure
Cardiovascular toxicity
o QT interval prolongation
o Polymorphous
o ventricular tachycardia
o congestive cardiomyopathy
o pulmonary edema
o cardiogenic shock
Acute neurologic toxicity
o Delirium
o Seizures
o Edema
o Encephalopathy
o coma
 MERCURY
o Hypotonia
o Hypertension
o Tachycardia
INVESTIGATIONS
 Urinary markers of early nephrotoxicity:

o Microalbuminuria
o retinol binding protein
o β2 -microglobulin
 severe central nervous system toxicity is apparent on CT or MRI
 Abdominal radiographs may demonstrate ingested radiopaque
arsenic syndromes describe the clinical presentation of mercury
poisoning
TREATMENT
 The principles of management for arsenic and mercury

intoxication
o prompt removal from the source of poisoning
o aggressive stabilization

o supportive care
o decontamination
o chelation therapy
Brain Abscess Or Cerebral Abscess

Brain abscess is an abscess caused by inflammation and collection of
infected material, coming from local or systemic infectious sources,
within the brain tissue
History
 Epidemiology
 The incidence of brain abscess is between 0.3 and 1.3 cases per
100,000 people per year
 Nearly 80% of abscesses occur in the frontal, parietal, and
temporal lobes.
 abscesses in the occipital lobe, cerebellum, and brainstem
account for the remainder of cases
 In 18% of cases, multiple brain abscesses are present
 in nearly 20% of cases, no predisposing risk factor can be
identified
 Symptoms
 In the early stages of cerebritis and abscess formation
o Asymptomatic
o sometimes associated with nonspecific symptoms
 L ow-grade fever
 headache
 lethargy
 As the inflammatory process proceeds
oDrowsiness
oConfusion
oHemiparesis
oSpeech difficulties together with fever with a rapidly progressive
course
o Vomiting
o Severe headache
o Seizures
o Papilledema
o Focal neurologic signs (hemiparesis)
o Coma
 A cerebellar abscess
o Nystagmus

o Ipsilateral ataxia
o Dysmetria
o Vomiting
o Headache
 Complication
o hemiparesis,
o seizures
o hydrocephalus
o cranial nerve abnormalities
o behavioral and learning difficulties
o shock and death
 Risk Factors
 Contiguous spread from an associated infection
 meningitis
 otitis media
 Mastoiditis
 Sinusitis
 soft tissue infection of the face
 scalp, orbital cellulitis, or dental infections
 Direct compromise of the blood–brain barrier due to
penetrating head injuries or surgical procedures
 Embolic phenomena
 Endocarditis
 right-to-left shunts
 congenital heart disease or pulmonary arteriovenous
malformation
 Immunodeficiency
 infection of foreign material inserted into the central
nervous system (CNS)
 Ventriculoperitoneal shunts.
 Sinus dental infections—Aerobic and anaerobic
streptococci, anaerobic gram-negative bacilli (e.g,
Bacteroides) S. aureus, and Enterobacteriaceae
 Penetrating trauma—S. aureus, aerobic streptococci,
Enterobacteriaceae, and Clostridium spp.
 Pulmonary infections—Aerobic and anaerobic
streptococci, anaerobic gram-negative bacilli (e.g.
Prevotella, Porphyromonas,), Fusobacterium,
Actinomyces
 Congenital heart disease—Aerobic and
microaerophilic streptococci, and S. aureus

 HIV infection—T. gondii, Mycobacterium,

Cryptococcus, and Listeria monocytogenes
 Transplantation—Aspergillus, Candida,
Cryptococcus, Mucorales, Nocardia, and T. gondii
 Neutropenia—Aerobic gram-negative bacilli,
Aspergillus, Candida, and Mucorales
Investigation
o CBC peripheral
 white blood cell count5
o blood cultures
 identifying bacterial pathogens
o Lumbar puncture is not routinely recommended in cases of brain
abscesses, because the procedure could cause brain herniation from
elevated intracranial pressure
o EEG -identify corresponding focal slowing.
 Diagnostic is imaging of the CNS
o Brain MRI with contrast
 to differentiates abscesses from cysts and necrotic tumor
 increased signal intensity
o cranial CT can
 provide more rapid imaging results but cannot provide the
fine tissue detail offered by MRI
 parenchymal low-density lesion,
o Both MRI and CT scans with contrast can demonstrate a

ring-enhancing abscess cavity
Treatment
 Initiation of an antibiotic regimen
 Empiric therapy
 3rd-generation cephalosporin
 Metronidazole
 Ampicillin with gentamicin in neonates
 Broad -spectrum antibiotic coverage -in
immunocompromised patients
 Administration of glucocorticoids
 Parenteral antibiotic therapy – usually for week
 Surgical excision of an abscess

Sample History
C/C: weakness of extremities of 6 days duration
HPI
This is 12 years old female patient who was last relatively healthy 6
days back at which time she started to have difficulty in getting up
from a sitting position, followed by complete failure to move her lower
extremities. After one day she couldn’t also move her upper
extremities. Associated with these symptoms she developed difficulty
in speech and swallowing, hoarseness of voice. After the onset of
the weakness of her extremities her father took her to Shanan Gibe
Hospital and they referred to our JUMC where she was admitted to
Pediatric Dept. Level-I Ward two days back.
Two weeks before her admission to JUMC she had a non-radiating
severe crampy type of abdominal pain which felt all over her abdomen
and the pain was associated with mild amount of watery diarrhea 3-4
times per day. The diarrhea had a foul smelling with mucus but no
blood. She also had low grade intermittent fever. Because of the
diarrhea she developed, her father took her to a private clinic in jimma
town and they were told that she had unspecified intestinal infection
for which she was given unspecified drug which didn’t remember its
color, but She took it for about a week, 3 times a day. The diarrhea
was subsided within four days of treatment
She is vaccinated for her age. Before her illness she used to eat three
times a day usually bread of made wheat, “injera made of teff”, “shiro
made of beans”, vegetables and sometimes meat. She used to finish
up to one injera in one sit and she didn’t share a plate with her
siblings. Now she only takes milk and different juices through a tube
because of difficulty of swallowing. She is a grade five student who is
top three from her class in academic performance. She had
comparable growth with children of her age group and has a good
interaction with her friends.

She lives with her family seven members in their own 3 room house
each with one window. They have their own supply of clean water and
a separate kitchen and latrine. Her father is a driver and her mother a
housewife. They earn about 3000 ETB monthly. She is the 5th child to
the family. She has three sisters and three brothers who are all
healthy.
Otherwise:
 she had no history of falling down, accidents or trauma to
the back or to the head…… Traumatic Brain Injury
 No history of loss of consciousness or abnormal body
movements……… Hemorrhagic Stroke
 No Similar illness previously and there is no similar
illness in her family …….. Brain Tumour
 There is no inability to control urine and stool…….
Poliomyelitis
 No upper respiratory tract infection, skin lesions, or
rashes ……. Brain abscess
 No difficulty of breathing, orthopnea or paroxysmal
nocturnal dyspnea……. Complication of GBS
 No Recent vaccination for rabies or any other diseases….
Risk Factor of GBS
 No fear of water or behavioral change…….. Rabies As risk
factor
 No history of contact with a chronic cougher or a known
TB patient…… TB meningitis
 Has no history of poisoning exposure……. Toxin exposure
Investigation
 Stool culture……… poliomyelitis
o Two stool specimens (8-10 gm each)

o collected 24-48 hrs. apart

o confirm by isolation and identification of poliovirus
 PCR technique- Is a sensitive, specific, and rapid technique for the

diagnosis of enteroviral infections……. poliomyelitis
o It can be performed on:
Stool
 Blood
CSF
Throat specimens
 CSF analysis ………. Poliomyelitis and GBS
o normal during the minor illness
o pleocytosis with 20-300 cells/μL with CNS involvement
o polymorphonuclear cells early course disease but shift to
mononuclear cells soon afterward
o Two stool specimens (8-10 gm each) collected 24-48 hrs. apart
o confirm by isolation and identification of poliovirus
 MRI/CT scan............Stroke and childhood brain tumor
 Electro myelography……. Myasthenia gravis

CHAPTER 15
APPROACH TO FEVER
 FEVER is an elevation of body temperature that exceeds the normal
daily variation and occurs in conjunction with an increase in the
hypothlamic set point. (Axillary temperature greater than 37.5)
ETIOLOGY OF FEVER
4 main categories:
1. Infectious,
2. Inflammatory,
3. Neoplastic, and
4. Miscellaneous
Most common causes of acute fever (fever lasting 7 days or less)
are viral infections (like common cold, gastroenteritis) and
uncomplicated bacterial infections (otitis media, pharyngitis,
sinusitis)
PATHOGENESIS OF FEVER
Three different mechanisms:
1. PYROGENS: raise the hypothalamic temperature set point.
 ENDOGENOUS PYROGENS include:

 cytokines interleukins 1 and 6, tumor necrosis
factor α, and interferons β and γ
 lipid mediators, like PG-E2
 Infectious diseases and drugs, malignancy and
inflammatory diseases can cause fever through the
production of endogenous pyrogens.

 EXOGENOUS PYROGENS or substances that come from

outside the body include mainly infectious pathogens and
drugs.
 Drugs that are known to cause fever include
vancomycin, amphotericin B, and allopurinol.
2. HEAT PRODUCTION EXCEEDING LOSS
e.g.
 salicylate poisoning
 malignant hyperthermia
3. DEFECTIVE HEAT LOSS
e.g.
 in children with ectodermal dysplasia or
 victims of severe heat exposure.
Pattern of Fever
Can provide clues to the underlying etiology
 Intermittent fever: an exaggerated circadian rhythm that includes
a period of normal temperatures on most day.
 Septic or hectic fever: extremely wide fluctuations.
 Sustained fever: persistent and does not vary by more than
0.5°C/day.
 Remittent fever: persistent and varies by more than 0.5°C/day.
 Relapsing fever: is characterized by febrile periods that are
separated by intervals of normal temperature. Ex
oTertian fever occurs on the 1st and 3rd days (malaria caused
by Plasmodium vivax),
oQuartan fever occurs on the 1st and 4th days (malaria caused
by Plasmodium malariae).
 There are many diseases that are characterized by relapsing fever:
Relapsing fever (Borrelia recurrentis), Trench fever (Bartonella
quintana), Q fever (Coxiella burnetii), Typhoid fever (Salmonella
typhi), Syphilis (Treponema pallidum), Tuberculosis,

Histoplasmosis, Dengue fever, Yellow fever, and many others.
 Biphasic fever (a.k.a camelback fever pattern): indicates a single

illness with 2 distinct periods.
e.g. Poliomyelitis
 A brief recall of poliomyelitis:
oIt may follow one of several courses:
 Inapparent infection: 90-95% of cases.
 causes NO disease and NO sequalae.
 Abortive Poliomyelitis
 Nonparalytic Poliomyelitis
 Paralytic Poliomyelitis: causes three distinctly recognizable
syndromes
I. Spinal paralytic poliomyelitis
II. Bulbar Poliomyelitis
III. Polioencephalitis
 May occur as the second phase of a biphasic illness.
 1st phase = corresponds to abortive poliomyelitis which is
characterized by:
Occurs 1 to 2 weeks after infection
FEVER, malaise, anorexia, and headache are its
prominent featues.
lasting up to 2 to 3 days.
 2 phase = After the patient appears to recover and feels better
nd
for 2-5 days, then severe headache and FEVER occur with
exacerbation of previous systemic symptoms.
 A biphasic course is also a characteristic of other enteroviral
infections, leptospirosis, dengue fever, yellow fever, Colorado
tick fever, spirillary rat bite fever (Spirillum minus), and the
African hemorrhagic fevers (Marburg, Ebola, and Lassa fevers).
 Periodic fever:
 describes fever syndromes with a regular periodicity (cyclic
neutropenia and periodic fever, aphthous stomatitis, pharyngitis,
and adenopathy)

 it include disorders characterized by recurrent episodes of fever

that do not follow a strictly periodic pattern (familial
Mediterranean fever, hyperimmunoglobulin D syndrome, the
Muckle-Wells syndrome).
 Factitious fever (or self-induced fever): may be caused by

intentional manipulation of the thermometer or injection of
pyrogenic material.
 The double quotidian fever:
 fever that peaks twice in 24 hr
 is classically associated with inflammatory arthritis.
 Generally, a single isolated fever spike is not associated
with an infectious disease.
 Similarly, temperatures in excess of 41°C are most often
associated with a noninfectious cause.
 Causes include:
 central fever (resulting from central nervous system
dysfunction involving the hypothalamus), malignant
hyperthermia,
 malignant neuroleptic syndrome,
 drug fever, or
 Heatstroke

Classification of fever
 Fever lasting less than  Fever lasting more than 7

7days days
Meningitis Abscess
Otitis media Salmonella infection
Mastoiditis (non-typhoidal)
Osteomyelitis Infective endocarditis
Septic arthritis Rheumatic fever
Acute rheumatic fever Miliary TB
Skin and soft tissue infection Brucellosis
Pneumonia
Fever with Rash

 Bacterial and viral infections are frequently associated with a
rash and fever in children.
 Most exanthems are self-limited and resolve in 7 to 10 days and
only symptomatic treatment is needed.
 Vaccinations have significantly decreased the incidence of
measles, rubella, varicella, and their congenital complications.

Diagnosis of fever In favour

Measles Typical rash ,Cough, runny nose, red
eyes ,Mouth ulcers ,Corneal clouding
Recent exposure to a measles case
No documented measles
immunization
Viral infections Mild systemic upset
Transient non-specific rash
Meningococcal infection Petechial or purpuric rash Bruising
Shock Stiff neck (if meningitis)
Relapsing fever Petechial rash / skin haemorrhages
Jaundice
Tender enlarged liver and spleen
History of relapsing fever
Positive blood smear for Borrelia
Typhus Epidemic of typhus in region
Characteristic macular rash
Clinical Features associated with Fever

 can range from no symptoms at all to extreme malaise.
 Some clinical features are:
 Feeling hot or cold
 Facial flushing
 Shivering
 Fatigue and irritability
 Looking ill/pale
 Decreased appetite
 The underlying etiology can also produce accompanying
symptoms; for instance, fever with petechiae in an ill-appearing
patient indicates:
oMenegiococcemia,
oRocket Mountain Spotted fever, or

oAcute bacterial endocarditis
 Association of Fever and Heart Rate:

oNormally, HR increases by 10bpm/1°C rise in T° for children
>2 months of age.
Relative Tachycardia:
o is when PR increases disproportionately to the
temperature.
o is usually caused by noninfectious diseases or infectious
diseases in which a toxin is responsible for the clinical
manifestations.
Relative Bradycardia: is when PR remains low in the
presence of fever.
 It can accompany the following diseases:

 Typhoid fever
 Brucellosis
 Leptospirosis
 Drug fever
 Bradycardia in the presence of fever also may be a result of a
conduction defect resulting from cardiac involvement with acute
rheumatic fever, Lyme disease, viral myocarditis, or
infective endocarditis.
Evaluation of Fever
 Detail history and complete P/E (a complete head-to-toe screen)
should be obtained.
 Immunization historyand any major illness should be asked.
 Laboratory studies, if required, should be based on case-by-case
basis.
 For instance, CXR if pneumonia is suspected; Stool if rotavirus
is suspected; and the like.

Dx History P/E & Lab. Ix. Remark

MALARIA  paroxysms of fever, chills,  splenomegaly  A prodrome (headache,
sweats, fatigue. (common), fatigue, anorexia, myalgia,
 may lack typical paroxysms  hepatomegaly, slight fever, and pain in the
and have nonspecific and chest, abdomen, and joints)
symptoms: fever (often  pallor as a lasting 2-3 days.
>40°C), consequence of  Patients with primary
headache, drowsiness, anemia. infection (travelers) also may
anorexia, nausea, vomiting,  Typical have irregular symptomatic
and diarrhea. laboratory episodes for 2-3 days before
 fever or unexplained findings include regular paroxysms begin, so
systemic illness + hx of travel anemia, most travelers presenting
or residence in a thrombocytopen with malaria lack a classic
malaria-endemic area within ia, and a normal malaria fever pattern.
the previous year = have or low leukocyte
life-threatening malaria until count.
proven otherwise regardless  ESR is often
of the use of elevated.
chemoprophylaxis.
MENEGITIS  Classic triad:  Signs of  See more under

Fever menengial meningitis.
Headache irritation
Neck stiffness (Kernig’s and
 Additional symptoms: Brudzinski
Nausea signs)
Vomiting  Altered
Photophobia mentation
Confusion  In infants:
Irritability Bulged
Sleepiness fontanelle
Delirium High
pitched
Coma
cry
 Hx of preceding systemic
symptoms= Viral menengitis  Blood cultures
 LP
 ESR/CRP
 CBC
ENTERIC  C/M varies from a mild  Coated tongue  macular or

FEVER illness with low-grade fever, (common in maculopapular rash (“rose
(THYPHOID malaise, and slight, dry 76%) spots”) may be visible
FEVER) cough to a severe clinical  Hepatomegaly around the 7th-10th day
picture with abdominal (37%) of illness, on the lower
discomfort and multiple  Pallor (20%) chest and abdomen and
complications.  Splenomegaly last 2-3 days. (25%)
 high grade fever with a wide (17%)  more dramatic in children
variety of associated  Jaundice (2%) <5 yr old, with
features, such as generalized  Obtundation comparatively higher rates
myalgia, abdominal pain, (2%) of complications and
and anorexia.  culture from hospitalization.
 In children, diarrhea may the blood or  Diarrhea, toxicity, and
occur in the earlier stages of another complications such as DIC

the illness and may be anatomic are also more common in

followed by constipation. site (like infancy, resulting in
stool, urine, higher case fatality rates.
BM culture).
 Widal test
(however,
many
false-positive
and
false-negativ
e results
occur.)
ACUTE  Nasal congestion  Erythema Certain patterns suggestive of
BACTERIAL  purulent nasal discharge and swelling sinusitis:
SINUSITIS (unilateral or bilateral) of the nasal  persistence of nasal
 fever mucosa with congestion, rhinorrhea (of any
 cough. purulent quality) and daytime cough
 Less common symptoms: nasal ≥10 days without
 bad breath (halitosis) discharge. improvement;
 a decreased sense of  Sinus  severe symptoms of
smell (hyposmia) tenderness temperature ≥39°C with
 periorbital edema (adolescents purulent nasal discharge for 3
 Additional symptoms: and adults) days or longer; and
 maxillary tooth  Transillumin  worsening symptoms either
discomfort ation reveals by recurrence of symptoms
 pain or pressure an opaque after an initial improvement
exacerbated by bending sinus that or new symptoms of fever,
forward. transmits nasal discharge and daytime
light poorly. cough.
CA-ACQUIRE  typically begin with:  Tachypnea  Auscultation may be
D  high fever  Increased work misleading, particularly in
PNEUMONIA  cough, and of breathing young infants, with poor
 pleuritic chest pain.  intercostal, findings disproportionate to
 preceded by several days of subcostal, and the degree of tachypnea.
symptoms of an upper suprasternal  Some infants with bacterial
respiratory tract infection, retractions pneumonia may have
typically rhinitis and cough  nasal flaring associated gastrointestinal
 Abdominal pain: in  use of accessory disturbances characterized
lower-lobe pneumonia. muscles. by vomiting, anorexia,
 In infants:  cyanosis and diarrhea, and abdominal
 prodrome of upper lethargy, (in distention secondary to a
respiratory tract infection severe infection, paralytic ileus.
 poor feeding especially in  Rapid progression of
 abrupt onset of fever, infants.) symptoms is characteristic in
 restlessness,  crackles the most severe cases of
 apprehension,  wheezing bacterial pneumonia
 ill-appearance, and  complications
 respiratory distress (pleural effusion
or empyema)
 dullness
 ↓breath
sounds.
 A lag in

respiratory
excursion
 The liver may
seem
enlarged
(because of
downward
displacement
of the
diaphragm
UTI  abdominal, back, or flank  Costovertebr  Pyelonephritis is the most
 Clinical pain; fever; malaise; al angle or common serious bacterial
Pyeloneph nausea; vomiting; and, suprapubic infection in infants
ritis occasionally, diarrhea. tenderness younger than 24 mo of age
 Fever may be the only  Crying while who have fever without an
manifestation. passing obvious focus.
urine
 UTI may be
 dysuria, urgency, suspected
 Cystitis frequency, suprapubic based on
pain, incontinence, and symptoms or
malodorous urine. fndings on
 Cystitis does not cause urinalysis, or
fever and does not result both
in renal injury.  A urine
culture is
necessary for
confirmation
 Asympto and
matic appropriate
Bacteriuri therapy.
a

CHAPTER 16
APPROACH TO JAUNDICE
Definition
 Jaundice is a yellowish discoloration of body tissues resulting from the
deposition of bilirubin.
 Tissue deposition of bilirubin occurs only in the presence of serum
hyperbilirubinemia
 It is a sign of
 Liver disease
 Less often, a hemolytic disorder or disorder of bilirubin metabolism.
BILIRUBIN METABOLISM
HEMOLYTIC
DISORDERS
(Increased Production)
LIVER
DISORDERS
(Decreased
Excretion)
Two types of Hyperbilirubinemia

A. Unconjugated (Indirect} Hyperbilirubinemia

This is suggestive of overproduction of bilirubin
 Hemolytic disorders and ineffective erythropoiesis
 Impaired hepatic uptake/conjugation of bilirubin (drug
effect or genetic
disorders)
B. Conjugated (Direct) Hyperbilirubinemia.
This is suggestive of
 Hepatocellular Conditions
 Cholestatic Conditions
 SO when we approach a child presenting with yellowish

discoloration of the body Our Primary target is to know whether it
is liver disease, hemolytic disorders or disorders of bilirubin
metabolism.
 Once we know the underlying problem (liver or hemolytic disorders)
we go to specific etiologies
History
CHARACTERIZATION OF JAUNDICE
 Is it really a Jaundice?z
 If child presents with yellowish discoloration of the skin, one
must first determine whether jaundice actually exists.
 Children who eat a large amount of yellow, orange, or red
carotene-containing vegetables or fruits like carrot can
develop keratodermia or
 lycopenemia (owing to ingestion of certain red foods,
such as tomatoes).

 ONSET: tells us if it is long lasting pathology or recent liver injury

or Hemolytic disorders.
If it persists for more than 6mo we may suspect chronic
complications.
 IN WHICH BODY PART FIRST RECOGNIZED: if it starts from the eye
most probably it is pathologic. If it is recognized only on the skin
it could be from consumption of carotene containing fruit and
vegetables
 HOW IT PROGRESS: Jaundice usually becomes apparent in a
cephalocaudal progression, starting on the face and progressing
to the abdomen and then the feet, as serum levels increase.
 Face ≈ 5 mg/dL;
 Mid-abdomen≈ 15 mg/dL;
 Soles≈ 20 mg/dL
 HOW IT LOOKS:
 Jaundice from deposition of indirect bilirubin in the skin

tends to appear bright yellow or orange.
 Jaundice of the obstructive type (direct bilirubin) has a
greenish or muddy yellow cast.

DDx for Jaundice

HEPATIC CONDITIONS
 Viral Hepatitis
 Drug Hepatotoxicity
 Autoimmune Hepatitis
 Metabolic Disorders: Wilson Disease, AAT deficiency
HEMOLYTIC CAUSES
 Hemoglobinopathies
 RBC defects
 Autoimmune hemolytic anemia
 Massive blood transfusion
CHOLESTATIC CONDITIONS
 Cholelithiasis (gallstones)
 Choledochal cyst
 Parasitic disease (ascariasis)
 Periampulary carcinoma
 Postoperative biliary strictures
 Primary sclerosing cholangitis
HEPATIC CONDITIONS
I. VIRAL HEPATITIS
Acute viral hepatitis is the most common cause of conjugated
hyperbilirubinemia in older infants and children.
ETIOLOGIES
 5 hepatotropic viruses are: HAV, HBV, HCV, HDV, and

HEV viruses

 Additionally, there are HSV, CMV, EBV, VZV, HIV, rubella,

adenoviruses, enteroviruses, parvovirus B19, and arboviruses
TRANSMISSION
 Feco oral: HAV and HEV

 Parentral and sexual: HBV HCV and HDV
 Perinatal: HBV and HDV
RISK FACTORS
 Poor hygiene for HAV and HEV

 Unimmunized child
 Contact with a person having viral hepatitis
 blood transfusions
 Sexual activity ( for adolescence age children )
 Maternal infection for
 HBV (If the mother is HBeAg-positive; up to90% of these
infants become chronically infected if untreated.)
 HCV and HDV: Perinatal transmission occurs in up to 5% of
infants born to viremic mothers.
 Tattooing and parenteral drug use: for HCV

SYMPTOMS
 Preicteric phase: fever, headache, malaise, and abdominal

complaints (e.g.,
abdominal pain, nausea, vomiting, anorexia) may last up to 5
days.
 This phase is followed by the appearance of dark urine and
frank icterus, usually as the fever begins to resolve
 rash, arthralgia (in HBV infection)

 Stms Pt present with features of fulminant hepatitis, hepatic
necrosis, cirrhosis, and hepatocellular carcinoma
P/E
 G/A:
 ASL because of abdominal pain
 CSL because of development of CLD eg. Wasting
 Vital Signs
 Anthropometry: Malnutrition of chronic illness
 HEENT:
 Ichteric sclera, pale conjunctiva
 LGS:
 SLAP: If the child develops HCC metastasis
 Gynecomastia on adolescents If there is cirrhosis
 Testicular atrophy
 R/S: Clubbing in case of CLD

 Plueral effusion Because of ascites
 CVS:
 Cardiac Failure my lead to cardiac cirrhosis so search for
any finding suggestive of HF
 ABDOMEN:
 Abdomenal distention because of Ascites
 Check Hernia sites if there is ascites
 +ve fluid thrill and SD
 Hepatomegaly, RUQ tenderness
 MSS:
 Pitting edema in case of GBS due to cirrhosis
 GUS:
 I/S:
 Bruising, rush, spider telangiectasia CLD resulting
decreased synthesis of coagulation factors.
With the exception of factor VIII, which is produced by

vascular endothelial cells, the blood clotting factors are
made exclusively in hepatocytes.
 N/S:
 Altered mentation when child develop Hepatic
encephalopathy
Complications
I. ALF
 Acute liver failure is suspected when the patient develops

 Coagulopathy with generalized rash and bruising,
 Encephalopathy with altered mentation from being
lethargic to comatose stage
 cerebral edema: Pt will show signs of increased ICP like
Head ache, Cushing triads(Hypertension, Bradycardia and
irregular breathing)
 Occurs more commonly with HBV.
 Risk factors:

 Coinfection or superinfection with HDV

 Immunosuppressed child.
 Prognosis→ MR is >30%
 Management→ liver transplantation is the only effective
intervention.
II. Chronic Liver Disease (CLD)

 Defnition
When the duration of liver disease is >3-6 mo OR when there is
evidence of chronic hepatic decompensation like physical stigmata
of CLD (clubbing, spider telangiectasia, splenomegaly, ascites) or
(hypoalbuminemia, thrombocytopenia).
 Etiologies
The most common causes of CLD are:
 Chronic viral hepatitis: HBV, HCV, and HDV
 Autoimmune hepatitis
 Drug-induced hepatitis: Commonly used drugs in
children that can cause CLD includes isoniazid, methyldopa,
pemoline, nitrofurantoin, dantrolene, minocycline, pemoline, and the
sulfonamides.
 Metabolic disorders like: Wilson disease, Nonalcoholic
steatohepatitis, α1-Antitrypsin deficiency, Tyrosinemia,
Niemann-Pick disease type 2, Glycogen storage disease type iv,
Cystic fibrosis, Galactosemia, Bile acid biosynthetic abnormalities
III. Liver cirrhosis

 In 25% of patients infected by HCV ultimately progress to cirrhosis
 Patient develop the clinical symptoms when the cirrhosis is
decompensated
A. PORTAL HYPERTENSION:

 Portal hypertension is defined as the elevation of the hepatic

venous pressure gradient (HVPG) to >5 mmHg.
 The three primary complications of portal hypertension are
gastroesophageal varices with hemorrhage, ascites, and
hypersplenism.
B. MALNUTRITION IN CIRRHOSIS
C. ABNORMALITIES IN COAGULATION
 More commonly, the synthesis of vitamin K–dependent clotting

factors is diminished because of a decrease in hepatic mass.
D. HEPATIC ENCEPHALOPATHY
 Portosystemic encephalopathy is a serious complication of chronic

liver disease and is broadly defined as an alteration in mental
status and cognitive function occurring in the presence of liver
failure.
Investigations
A. Liver Enzymes:
 ALT and AST: in viral hepatitis ALT is more elevated than AST
B. LFT
a) Synthetic function:
 Coagulation profile: Prolonged PT and PTT
b) Serum Albumin level falls
 Excretory function: TSB level will be elevated
C. PICHT: Because transmission of HIV and Hepatitis Virus is similar

and it also affects our management choice.
D. Liver biopsy is the only means to assess the presence and extent
of hepatic fibrosis.
 A liver biopsy is indicated only before starting any treatment
and to rule out other causes of overt liver disease.
E. Viral markers

Diagnostic Blood Tests: Serology and Viral PCR
HAV HBV HCV HDV HEV
ACUTE/ACTIV Anti-HAV Anti-HBc Anti-HCV(+) Anti-HDV IgM(+) HEV IgM(+)

E INFECTION IgM(+) IgM(+) HCV RNA(+) Blood PCR Blood PCR
Blood HBsAg(+) (PCR) positive* positive*
PCR Anti-HBs(−) HBsAg(+)
positive HBV DNA(+)
(PCR)
PAST Anti-HAV Anti-HBs(+) Anti-HCV(−) Anti-HDV IgG(+) Anti-HEV IgG(+)

INFECTION IgG(+) Anti-HBc Blood Blood PCR(−) Blood PCR(−)
(RECOVERED) IgG(+) PCR(−)
CHRONIC No Anti-HBc Anti-HCV(+) Anti-HDV IgG(+) No

INFECTION IgG(+) Blood PCR Blood PCR (−)
HBsAg(+) (−) HBsAg(+)
Anti-HBs(−) PCR (+)or (−)
PCR (+)or (−)

Management Principles
 HAV
 Supportive treatment
 consists of intravenous hydration as needed and antipruritic
agents and
 fat-soluble vitamins for the prolonged cholestatic form of
disease.
 HBV
 Acute HBV infection is largely supportive. Close monitoring for
liver failure and extrahepatic morbidities is key.
 Chronic HBV infection is in evolution; no one drug currently
achieves consistent, complete eradication of the virus.
Prognosis
 HAV has excellent prognosis, with no long-term sequelae. The only
feared
complication is ALF
 HBV (See Diagram Below)
IV. HEPATOTOXICITY
 Drug and toxins (Environmental toxins like fungal toxins,
pesticides, or heavy metals) induced hepatitis
Etiologies

 In a recent series, acetaminophen, antimicrobial (50%) and

central nervous system agents (40%) were the most commonly
implicated drug classes causing liver injury in children.
Patterns of Hepatic Drug Injury

Disease Drugs
Centrilobular necrosis Acetaminophen
Halothane
Microvesicular steatosis Va Valproic acid
Acute hepatitis Isoniazid

General hypersensitivity Sulfonamides
Phenytoin
Fibrosis Methotrexate
Cholestasis Chlorpromazine
Erythromycin
Estrogens
Sinusoidal obstruction Irradiation plus busulfan
syndrome Cyclophosphamide
(venoocclusive disease)
Portal and hepatic vein Estrogens
thrombosis Androgens
Biliary sludge Ceftriaxone
Hepatic adenoma or Oral contraceptives
hepatocellular Anabolic steroids
carcinoma
Nelson 20th Edition Etiologies of drug induced liver injury

MECHANISM OF DRUG OR TOXIN INDUCED LIVER INJURY
 Clinical manifestations can be mild and nonspecific, such as fever,
malaise and features of liver injury (which is the same as viral
hepatitis).
 On History we ask if there is any medication that the child taking

currently. Then try to assess the specific type of medication.
Investigations
1. LIVER ENZYMES: Hepatocyte damage can lead to elevations of serum
aminotransferase
2. LFT

a) Synthetic function:
i. Coagulation profile: Pronlonged PT and PTT
ii. Serum Albumin level falls
b) Excretory function: TSB level will be elevated
3. Toxicologic screening: of blood and urine specimens can aid in
the detecting drug or toxin exposure
4. Percutaneous liver biopsy
a) In hepatotoxicity it may reveals proliferation of smooth
endoplasmic reticulum
b) Development of complications like cirrhosis and liver failure
Treatment
 Mainly supportive
 Avoiding the offending agent
V. Autoimmune Hepatitis
DEFNITION
 Autoimmune hepatitis is a chronic hepatic inflammatory process

manifested by elevated serum aminotransaminase concentrations,
liverassociated serum autoantibodies, and/or
hypergammaglobulinemia.
ETIOLOGIES
 Autoantibodies against once own hepatocytes.

 Triggering factors can include:
 Molecular mimicry
 Infections
 Drugs
 Environment (toxins) in a genetically susceptible host.
RISK FACTORS
 Female Gender
 Predominantly childhood and young adulthood (2-14yrs) in T2AH
(Type 2 Autoimmune Hepatitis)
CLINICAL FEATURES

Symptoms include:
 fever, fatigue, weakness, abdominal pain, and joint pain.
 Features of liver injury
 Some Pt may develop the features of cirrhosis (ascites, bleeding
esophageal varices, or hepatic encephalopathy).
 Symptoms of other autoimmune manifestations may also be
present
(e.g.. thyroiditis, autoimmune hemolytic anemia).
Investigations
1. LFT
 Enzymes: Serum aminotransferase can be in

excess of 1,000 IU/L in symptomatic young patients.
 Synthetic function:
 Serum γ-globulin levels can show marked
polyclonal elevations.
 Hypoalbuminemia is common.
 Excretory function: TSB level will be elevated
2. CBC
 Anemia, leukopenia, and thrombocytopenia are present in the
development of portal hypertension and hypersplenism
3. Serum Autoantibodies level:
ANA, SMA, LKM-1 &3

Treatment
 Immunosuppressive therapy
VI. Metabolic Disorders

A. Α1-ANTITRYPSIN DEFICIENCY
 Older children can present with asymptomatic

hepatomegaly or manifestations of chronic liver disease or
cirrhosis, with evidence of portal hypertension.
B. WILSON DISEASE
Wilsonian hepatic disease may manifest as:

 Asymptomatic hepatomegaly (with or without splenomegaly),
 Subacute or chronic hepatitis
 Acute hepatic failure (with or without hemolytic anemia)
 Cryptogenic cirrhosis, portal hypertension, ascites, edema,
variceal bleeding other effects of hepatic dysfunction (delayed
puberty, amenorrhea,coagulation defects)
 Neurologic disorders can develop insidiously or precipitously,
with intention tremor, dysarthria, rigid dystonia, Parkinsonism,

choreiform movements, lack of motor coordination,

deterioration in school performance, or behavioral changes.
 Psychiatric manifestations include depression, personality
changes, anxiety, or psychosis.
Investigations
 Serum ceruloplasmin levels (<20 mg/dL)
 Urine copper excretion >1.6 µmol/24 hr
C. FAMILIAL NONHEMOLYTIC UNCONJUGATED HYPERBILIRUBINEMIA
 UDPGT activity is deficient or altered in 3 genetically and

functionally distinct disorders
 Crigler-Najjar [CN] syndromes type I (Complete absence of
UDPGT activity) and type II (Partial absence of the UDPGT
activity)
 Mostly ocure during the neonatal period.
 Gilbert syndrome: Gilbert syndrome is diagnosed much more
often in older children and adults than it is in infants.
 Patients with Gilbert syndrome present with a benign
increase in unconjugated bilirubin, caused by a mild
decrease in UDPGT activity.
Hepatobiliary Tract Disorders Causing Jaundice

 rare in most pediatric patients
 cholelithiasis (gallstones).
 C/M
 acute onset of right upper quadrant pain
 A palpable right upper quadrant mass may be felt,
jaundice.
 If fever is present, acute cholecystitis may be
complicating the picture.
 Choledochal cyst
 C/M
 RUQ pain or diffuse abdominal pain and vomiting
 Periampulary carcinoma:
 includes Cholangiocarcinoma, Pancreatic cancer,
Gallbladder cancer and Ampullary cancer
Hemolytic Causes of Jaundice (discussed under anemia )

a) Hemoglobinopathies
b) RBC defects
c) Autoimmune hemolytic anemia

Sample History
C/C: Yellowish Discoloration of the eye of 1 month duration
HPI:
This is a 10 yrs old school age child who was relatively healthy 1mo
back @ which time he started to experience yellowish discoloration
of the eye. Prior to yellowish discoloration he has experienced
LGIF, malaise, and mild global type of headache in which the
malaise and headache persists to now. Since the past 1mo he also
has experienced right upper quadrant burning types of pain
which is aggravated when he sleeps on right recumbent position
and alleviated by lying on left recumbent position but with no
radiation characteristics. In addition to this he also has loss of
appetite, significant but unquantified weight loss and darkening of
urine. Since 2 wks he has hx of easy fatigability while playing with
his friends which makes him currently not to attend school. He also
has abdominal swelling that makes him unable to wear his clothes
progressing to legs after a week. For the past 3 days he has
experienced non bilous non projectile non blood tinged vomiting
and early satiety.
His staple food is injera made of tef and bread made of maize and
wheat with shiro made of bean and pea consumed 3* per day. He
rarely consumes vegetables like cabbage, tomatoes or carrot. The
house hold use stream water for drinking and cooking.
He has no hx of immunization.
He had frequent contact with his neighbor who had yellowish
discoloration of the eye.
Otherwise he has no hx of
 Tattooing and parenteral drug use: for Hcv
 Sharing of sharp materials
 Family hx of same illness or liver disease: for AIH
 Altered mentation, disorganized speech or behavioral
change like restlessness: Hepatic Encephalopathy

 Bleeding from mouth or anus: Portal Hypertension

 Orthopenea, PND, or cough: to r/o cardiac cirrhosis
 Recent drug use like Anti TB drugs, immunosuppressant drugs: r/o
Drug induced Hepatotoxicity
 Heat or cold intolerance, personal or familial hx of DM: thyroiditis
and DM can occurs on pts with AIH
 Tremor, difficulty of articulating words, poor school performance:
r/o Wilson disease
 No hx of contact with chronically coughing or TB patient: to r/o
Disseminated TB
 Itching sensation: to r/o Obstructive Jaundice
 He was screened for RVI @ local health center and found to be NR:
b/c affects HBV management and Progression of disease
Investigations
Lab studies
 TSB
 Van den Bergh method is used.
 How direct (conjugated) and indirect (unconjugated) bilirubin
level is determined??
 Bilirubin is exposed to diazotized sulfanilic acid
 The direct fraction is that which reacts with diazotized sulfanilic
acid in the absence of an accelerator substance such as alcohol
that absorb maximally at 540 nm, allowing photometric
analysis.
N.B The direct fraction provides an approximation of the

conjugated bilirubin level in serum.
 The total serum bilirubin is the amount that reacts after the
addition of alcohol.
 unconjugated bilirubin in serum
 Indirect fraction= total serum bilirubin level – direct bilirubin

levels

N.B
 The normal serum bilirubin concentration usually is between 1

and 1.5 mg/dL.
 Unconjugated hyperbilirubinemia is present when the direct
fraction is <15% of the TSB.
 Conjugated hyperbilirubinemia is always associated with
bilirubinuria
 Detection of bilirubin in urine via dipstick test is extremely helpful
to confirm the presence of conjugated hyperbilirubinemia in a
patient with mildly elevated direct fraction.
Causes of isolated hyperbilirubinemia

I. Indirect hyperbilirubinemia
A. Hemolytic disorders
B. Ineffective erythropoiesis
C. Increased bilirubin production: due to Massive blood
transfusion or Resorption of hematoma
D. Drugs like Rifampin, Probenecid, Ribavirin, Protease
inhibitors (Atazanavir, Indinavir)
E. Inherited conditions like Crigler-Najjar types I and II, or
Gilbert’s syndrome
II. Direct hyperbilirubinemia (inherited conditions)

A. Dubin-Johnson syndrome
B. Rotor syndrome
 LFT
 Viral markers:
 CBC: Anemia, leukopenia, and thrombocytopenia are present in
the development of portal hypertension and hypersplenism
 PIHCT: risk factor for RVI and HBV is same and affects our
management choice.
 DM screening
 Serum Autoantibodies level: ANA, SMA, LKM-1 &3

 Serum ceruloplasmin levels and Urine copper excretion: if we

suspect Wilson disease.
 Peripheral morphology of RBC: For searching Hemolytic causes
of jaundice.
IMAGING
 Abdominal U/S: for documenting gallstone disease

 CT
 MRI: for searching causes of obstructive jaundice.
 ERCP: for diagnosis of CBD stone used only if there is indications
LIVER BIOPSY
 In hepatotoxicity it may reveals proliferation of smooth

endoplasmic
reticulum
 The only means to assess the presence and extent of hepatic
fibrosis.
Indications for liver Bx
1. Hepatocellular disease of uncertain cause
2. Prolonged hepatitis with the possibility of AIH
3. Unexplained hepatomegaly
4. Unexplained splenomegaly,
5. Hepatic lesions uncharacterized by radiologic imaging,
6. Fever of unknown origin
7. Staging of malignant lymphoma

CHAPTER 17
ESSENTIALS OF NEONATOLOGY
History and physical examination of neonates
1. NEONATAL HISTORY TAKING
a. Maternal profile: age of the mother, occupation, parity, blood

group and Rh, chronic maternal illnesses, history of sexually
transmitted diseases, Hepatitis B infection
b. Current pregnancy: LNMP (last normal menstrual period),
gestational age, ANC, bleeding, hypertension, diabetes, thyroid
diseases, eclampsia, acute or chronic infection.
c. Previous pregnancy:history of abortion, fetal death, early
neonatal death, premature birth, history of early neonatal
jaundice, history of birth defect.
d. Drug history: history of alcohol ingestion, cigarette smoking ,
any medications during pregnancy (anticonvulsants, anti TB,
warfarin, HAART, thyroid treatment drugs , antenatal steroid
use, contraceptives)
e. Family history: the health worker needs to know the family
history to see if there are any inherited diseases like diabetes
mellitus, hypertension, bronchial asthma, thyroid disease and
others.
f. Labor and delivery: presentation, onset of labor, duration of
rapture of membranes, duration of labor, mode of delivery,
presence of meconium, breathing condition of at birth,
resuscitation, birth weight, place of delivery.
Mostly Presenting compliant: like failure to suck, fever, breathing

difficulty (fast breathing), abnormal bodymovement, yellowish
discoloration of skin and eye.
2. PHYSICAL EXAMINATION OF NEWBORNS
 QUICK EXAMINATION

 The initial examination of a newborn infant should be

performed
as soon as possible after delivery.
 Value: To detect life threatening insults.
I. Apgar scoring - (done at I, 5 minutes).

II. Level of consciousness
 Normal newborn is conscious, active, alert.
 Disturbed consciousness as lethargy, coma or weak
suckling may indicatecerebral lesion or severe infection.
III. Color:
 Normal newborn is pinkish in color.
 Abnormal appearance of the newborn may be:
o Pallor (anemia or shock)
o Plethora (Polycythemia)
o Cyanosis
o Jaundice.
IV. Vital signs:
 Heart rate
 (120- 150 beat/minute)… normal
 < I00 beat/minute … Bradycardia.
 > 160 ...tachycardia.
 Respiratory rate
 (30 - 50 /minute) … normal
 > 60 …tachypnea (RD)
 <30…..bradypnea
 Temperature
 (36- 37.2°C) - normal
 < 35.5 - hypothermia
o 36 - 36.4 mild hypothermia
o 32 - 35.9 moderate hypothermia
o <32 severe hypothermia
 > 37.5…. fever.
 >41.5 ……hyperthermia
 Blood pressure - standardize Bp based on age,sex and
length

 After the end of quick examination the newborn will be considered

as:
 Normal --+ proceed to other lines of examination.
 Abnormal --+ resuscitative measures.
 Temperature, pulse, respiratory rate, color, signs of respiratory

distress, tone, activity, and level of consciousness of infants
should be monitored frequently until stabilization.
 In other word APGAR SCORE
Components and scoring as follows: APGAR score
Sign Score 0 Score 1 Score 2

Activity Flaccid Some flexion Well flexed
Pulse Absent <100 per minute >100 per
minute
Grimace No response Grimace Cough or sneeze
Appearance Pale/Blue Blue extremities Completely pink
Respiration Absent Weak Good cry
 Three levels of score:

 Low APGAR score 0-3
 Moderate APGAR score 4-6
 Normal APGAR score 7-10
Note: A newborn with an APGAR score of less than 7 needs

special attention.
 DETAILED EXAMINATION
Anthropometric measurement (normal values for term neoate):

 Weight: 2500g -3999gm
 Length: 48-53 cm (average: 50cm)
 Head circumference: 33-38cm
Systemic examination
 HEENT

ocaput succedaneum(scalp swelling that extends across the

midline and over suture lines and is associated with head
molding)
osubgaleal hemorrhage(a fluctuant boggy mass developing

over the scalp (especially over the occiput) with superficial
skin bruising),
oCephalohematoma(),
osutures (craniosynostosis),
ofontanel,
oface, nose, ears, mouth, neck,clavicles, eye discharge,

icterus, cataract
 Neck
oShort neck or webbing {Turner)
oGoiter (enlarged thyroid).
 Cardiovascular system
o Apex beat: Normally in Left 4th space just outside mid
clavicular line.
oMurmurs: Most of murmurs in early neonatal period are
transient
oThe 2nd heart sound may not be splitted in the 1st day of life
oFemoral pulsations: If absent Aortic coarctation is
suspected.
 Chest examination
oCheck for signs of respiratory distress, breathing pattern,
respiratory rate, air entry
to the lungs
oAP diameter andsymmetry of the chest,
oAuscultation for wheezes, crepitations,
 Abdominal examination
oLiver may be palpable 2 cm in neonates
oBoth kidneys should be palpable in the 1st day of life
oCheck for organomegaly, ascites, umbilicus, ..…
oCauses of neonatal abdominal masses e.g.:
 Hydronephrosis.
 Multicystic- dysplastic kidney

 Ovarian cyst.
 Intestinal duplication
 Neuroblastoma
 Wilm's tumor
 External genitalia:
oAmbiguous genitalia
oUndescended testis
oopening of urethral meatus
ovaginal bleeding or discharge.
 Musculoskeletal:
olimb defects (clubfoot, syndactyly, polydactyly)
ofractures and birth injuries,
ospinal bifida, joints
oErb's palsy
oMalformations.
oHip dislocation detected by:
 Gluteal fold asymmetry
 Limited hip abduction.
 Unequal leg length
 Hip X-ray
 Skin
oMeconium staining
oEdema (Hydrops fetalis).
orash,
ojaundice,
opallor,
oplethora,
ocyanosis,
obirthmarks, etc.
Note: Acral (extremity) cyanosis is a normal finding in

newborns

 Neurological examination
oLevel consciousness.
oMuscle tone (normally flexed all limbs).
NEONATAL REFLEXES.
 Moro reflex:
 The infant is placed in a semi-upright position. The head is
momentarily allowed to fall backwards, with immediate
re-support by the examiners hand. A complete response consists
of abduction of the upper limbs at the shoulder, extension of the
forearms at the elbow and extension of the fingers followed by
adduction and flexion.
 check for completeness and symmetry
 Absent or weak Moro response indicate serious CNS
disturbance.
 Asymmetries occur with Erb palsy and clavicular fractures.
 This reflexdisappears by 4 to 6 months of age.
 Rooting reflex
 Stimulating one corner of the mouth or the lower lip causes the
infant to turn to wards the touch and open his/her mouth.
 This reflex disappears by 4 to 7 months of age.
 Grasp reflex (arm and plantar )
 is elicited by placing a finger in the open palm of each
hand;normal infants grasp and hold the object.
 This reflex diminishes by 3 to 4 months of age.
 Sucking reflex:
 When an object is placed in the infant’s mouth or touches the lips,
a sucking reflex is elicited.
 reflex disappears by 12 months of age
 Check for its absence or presence
SPECIAL EXAMINATION
 Check for congenital anomalies

 Cleft lip - Tracheo-esophageal fistula
 Limb anomalies - Imperforate anus.

Classification of the Newborn

1. Based on the gestational age, a newborn could be classified
in to:
 Preterm: less than 37 Completed weeks
o extremely preterm(extremely low GA)(<28wks)
o Very preterm (28-32wks)
o Moderate preterm (32-34wks)
o Late preterm(34-37wks)
 Term: 37- 42 weeks
 Post term : more than 42 weeks
Gestational age could be estimated by one of the following

methods:
 On the bases of the first day of the last menstrual period
 Ultrasound estimation: gestational age estimate during the first
trimester isultrasonography can be accurate within +/- 5-7
days.
 Based on Ballard score
o The new Ballard has two components, neuromuscular
and physical maturity scoring and theaccuracy is
within a range of +/- two weeks
Neuromuscular maturity
Physical maturity

2. Classifications of the newborn based on the birth weight:

 Macrosomia : birth weight of 4000 gram and above
 Normal weight : 2500 – 3999 grams
 Low birth weight : 1500 – 2499 grams
 Very low birth weight : 1000 – 1499 grams
 Extremely low birth weight : less than 1000 grams
3. A newborn can also be classified with respect to birth weight
and gestational age as follows:
 Appropriate for gestational age (AGA) if the birth weight is
between 10-90%
 Large for gestational age (LGA) if birth weight is greater
than 90%

 Small for gestational age (SGA) if birth weight is less than

10%
 Basic Definitions:
 Neonatal period: a period from birth to 28 completed days
of life.
 Early neonatal period: this is a period from birth to 7
completed days of life
 Late neonatal period: A period from 8 to 28 completed days
of life
 Perinatal period: This period includes from 28 completed
weeks of gestation to 7 days after birth
 Gestational age: this is the time counted (in weeks) from the
first day of the woman’s last menstrual period to the day of
delivery (or current date if baby not yet born).
 Chronologic age: this is the age of the baby counted from
the time of birth
 Corrected age: this is the age of the baby which is counted
by reducing the
Chronological age from the number of weeks born before 40
weeks of gestation
Approach on selected cases

Respiratory distress in neonate is fast breathing and any sign of
respiratory distress
DDx:-
 Neonatal sepsis
 Perinatal asphyxia
 Respiratory distress syndrome(HMD)
 Meconium aspiration syndrome
 Transient tachypnea of new born
 Critical CHD
 Tracheo-esophageal fistula
 Diaphragmatic hernia

 Hypothermia
 Hypoglycemia
Let us see few of them as follows:
A. Neonatal sepsis
 is defined as a clinical syndrome manifested with systemic
signs and symptoms of infection in the first 4 weeks of life.
 Classified as: -
I. Early-onset neonatal sepsis(EONS) (Birth to 7 days)
II. Late-onset neonatal sepsis(LONS) (8 to 30 days)
III. Very-late-onset infections (onset after 1 month of life)
I. EARLY ONSET NEONATAL SEPSIS
 occur from birth to 7 days, usually less than 72 hrs.

 is usually due to vertical transmission by ascending
contaminated amniotic fluid or during vaginal delivery from
bacteria colonizing or infecting the mother's lower genital tract.
 the risk for sepsis increases from 1 to 4 percent in neonates born
to mothers with chorioamnionitis.
 Meningitis present with Early-onset sepsis in 30% of cases.
ETIOLOGY:-
The commonest organisms in EONS are (in order of decreasing

frequency):
 Group B Streptococcus
 Escherichia coli (E. coli),
 Streptococci other than Group B
 Staphylococcus aureus
 Haemophilus influenza
 Listeria monocytogenes
 Gram negative anaerobes
 Fungi
 Chlamydia trachomatis
Some viral causes are:- CMV, HSV, enteroviruses, and HIV.
 Maternal risk factors for early onset sepsis
oMaternal fever (temp >38ºC) during labor or within 24 hours
afterdelivery(choriamnionitis), meconium stained liquor
oMaternal urinary tract infection in current pregnancy or

bacteriuria
oDuration of membrane rupture > 18 hours before delivery
oProlonged labor(>24hr)
oUnclean PV
oFoul smell vaginal discharge
oBirth weight <2kg or prematurity
oHome delivery
opoor cord care
oCongenital anomaly (gastrocshisis, omphalocele,
myelomeningocele)
oPoor aseptic technique in handling baby
II. LATE-ONSET NEONATAL SEPSIS
 occur b/n 8- 30 days or it may extend up to 60 day even up to 90

day but at least delay for more than 4 day after birth.
 can be acquired by the two following mechanisms: -
 Maternal vertical transmission, resulting in initial neonatal
colonization that evolves into later infection.
 Horizontal transmission from direct contact with care providers or
environmental sources. Disruption of the intact skin or mucosa ,
which can be due to invasive procedures (eg, intravascular
catheter),increases the risk of late-onset infection.
 In late onset sepsis meningitis is present in 75% of
case.
ETIOLOGY:-
the commonest organisms in LONS are (in order of decreasing

frequency):
 Coagulase negative Staphylococcus
 Staphylococcus aureus
 Klebsiella
 Group B streptococcus
 Enterobacter
 Escherichia coli,
 Enterococcus / group D Streptococcus
 Pseudomonas,

 Other Gram negatives

 Candida albicans
RISK FACTORS
 use of forceps during delivery

 Invasive intrauterine monitoring which penetrate the neonatal
defensive epithelial barriers of the skin and mucosa
 Neonatal resuscitation
 Catheterization
 Iv Cannulation
 Intubation
 Bottle feeding
Clinical features:-
 The common clinical manifestation(triad) is
ochange in behavior,
ochange in feeding pattern (failure to suck) and
orespiratory distress which is manifested by gradual or
sudden onset symptoms and signs.
A. EARLY MANIFESTATIONS- Non specific - not doing well baby
 Respiratory distress and apneic attacks
 Lethargy
 Poor feeding and vomiting
 Unstable temperature (mainly hypothermia)
 Staring
 Poor Moro and suckling reflexes
 pale with circumoral cyanosis. Sometimes apnea, cyanosis
with hypothermia may be the manifestation.
B. LATE MANIFESTATIONS - focal infections
 Respiratory- Pneumonia with respiratory distress
(tachypnea, retractions...)
 Neurologic-
 Meningitis: -
 Seizures
 Tense bulging fontanelle
 High pitched cry

 Irregular respiration
 Hypotonia & hyporeflexia
 Cardiac: -
o Shock pallor, cold skin, hypotension, oliguria
o Heart failure
 tachycardia,
 tachypnea,
 tender liver,
 cardiomegaly
 Gastrointestinal: -
o Vomiting, diarrhea.
o Direct hyperbilirubinemia due to hepatitis.
o Hepatosplenomegaly
o Necrotizing enterocolitis.
 Heamtologic: -
o Pallor
o Purpura I DIC
o Bleeding tendency
 Skin:
o Sclerema - hardening of the skin
 poor prognosis.
 In general Signs and Symptoms neonatal sepsis is non-specific

and the neonate can present with nonspecific s/s rarely with
focal signs of infection.
 Neonate presenting with onset of the following s/s with r/f
should be suspectedand screened for neonatal sepsis:
I. Abnormal vital signs
II. Fever (temp >38 ºC), hypothermia (temp <36 ºC) or
temperature instability
III. Tachycardia (HR > 180) or bradycardia (HR <80)
IV. Tachypnea (RR > 60) or bradypnea (RR < 30) including apne
V. Poor perfusion: capillary refill time > 3 seconds, hypotension
VI. Abnormal breathing: gasping, grunting, severe chest in drawing,
nasal flaring or apnea
VII. Abnormal color: cyanotic, pale, grey, mottled, jaundiced,
erythematous including umbilical flare

VIII. Abnormal activity: tremors, irritability, seizures, floppiness,

stiffness or
minimal response to stimulation, lethargy
IX. Abnormal feeding: poor feeding, abdominal distention,
recurrent vomiting, diarrhea, otherwise unexplained hypo- or
hyperglycemia
X. Severe Jaundice
XI.Bulging fontanel
DIAGNOSTIC WORKUP
 CBC (Complete Blood Count with differential):Concern for sepsis if:

 Total WBC is abnormal (<5,000 or >20,000)
 Differential with granulocytes >70%.
 Anemia
 Thrombocytopenia
 Micro-ESR or CRP
 Culture (blood,CSF and urine)
 U/A:-for >1wk
 Lp:-
Diagnosis of meningitis
Clinical evidence + CSF analysis
CSF analysis suggestive of meningitis:

 Identification of organism on gram stain or culture (70-80
 WBC count greater than or equal to 15 cells/mm3 in term
and 30
cell in preterm
 Low glucose (less than two third of serum value-70%) or
30mg/dl
 Protein greater than 150 mg/dl in term and 175mg/dl in
preterm
infant
 CXR: -Consider chest x-ray if respiratory distress or oxygen
desaturation

Treatment
 TREATMENT OF NEONATAL SEPSIS INCLUDE:
1. GENERAL SUPPORTIVE MEASURES:
 Assess airway,breathing,saturation and provide oxygen if

needed .
 Provide maintenance iv fluid for the first 12hr, give blood if
hemoglobin is <12g/dl.
 Treat convulsion if present.
 Treat hypoglycemia and hypothermia if present.
2. ANTIBIOTIC TREATMENT: -
 The choice of antibiotic is ampicillin andGentamycin in early
onset sepsis but cloxacillin is added in late onsetsepsis
specially if staphylococcus is suspected.
B. Perinatal asphyxia
Important terminologies
 Anoxia is a term used to indicate the consequences of complete
lack of oxygen as a result of a number of primary causes.
 Hypoxemia refers to decreased arterial concentration of oxygen.
 Hypoxia refers to a decreased oxygenation to cells or organs.
 Ischemia refers to blood flow to cells or organs that is
insufficient to maintain their normal function
Perinatal Asphyxia: -
 is defines as failure to initiate and sustain breathing at birth or it
is 5th minute Apgar score of 0-3
 Or defined as low Apgar score at one minute of birth(0-6) with
hypotonia or seizure.
 It can also be defined as placentalor pulmonary gas exchange
impairment leading to hypoxemia and hypercarbia.
Note: Perinatal asphyxia results from compromised placental or
pulmonary gas exchange.
This lead to
Hypoxia and hypercarbia

Anaerobic glycolysis and lactic acid production
Hypoxia and acidosis
depress myocardial function, leading tohypotension and ischemia
further compromise, specifically to cns and kidney
Note: Perinatal asphyxia is the second commonest cause of neonatal

mortality only preceded by infection and the commonest cause of
disability in surviving newborns.
RISK FACTORS
 Antepartal cause(20%)
 Maternal hypotension of any cause usually APH
 Severe anemia
 Cardiopulmonary diseases
 Maternal hypertension
 Preeclampsia/eclampsia
 Maternal diabetes
 Intrapartum cause (70%)
 Cord compression (E.g. Cord prolapse)
 Meconium aspiration
 Prolonged labor (maternal/ fetal causes)
 Postpartal cause (10%)
 Prematurity
 Cardiovascular abnormalities
 Pulmonary malformations
 Neurologic abnormalities
 Severe infections
 Bleeding, shock
 Congenital heart disease

CLINICAL PICTURE
Depends on duration & severity of asphyxia

a. In the fetus : Fetal monitoring shows:
Slow, weak, irregular heart beats(type II
deceleration)
Scalp pH less than 7.2
 Action: give mother high oxygen concentration
and prepare for immediate delivery.
b. After delivery :
Meconium staining of the newborn, amniotic
fluid and vernix caseosa
Decreased consciousness with failure of
spontaneous breathing.
Low Apgar score with cyanosis and flaccidity .
c. Later manifestations of perinatal asphyxia :

1. Hypoxic-ischemic encephalopathy (HIE)
2. Cardiac :- Heart failure, hypotension
3. Respiratory :- Meconium aspiration , persistent
pulmonary hypertension of newborn, respiratory
distress.
4. Renal:- Acute tubular necrosis, hematuria,
oliguria (< 0.5ml/kg/hr
5. GIT:-Necrotizing enterocolitis and intestinal
perforation
6. Metabolic:-Hypoglycemia,
hypocalcemia ,hypomagnesemia,
hyponatremialactic acidosis and syndrome of
inappropriate secretion of ADH
ORGAN INVOLVEMENT
The target organ involved in PNA are :
 Kidney (50%)
 CNS (28%)
 CVS (25%)
 Pulmonary (23%)
 GIT

Hypoxic-ischemic encephalopathy (HIE)

 Hypoxia from PNA impair cerebral perfusion that progressively
result inNa-K ATPase impairment, cell swelling and cell
death.HIE result in focal or diffused cerebral injury. The
commonly affected areainclude periventricular area, thalamus
and parasagittal supriomedial cortex.
 At birth, depressed and fail toinitiate and sustain breathing

spontaneously.
 Later, theymay remain hypotonic or change from a hypotonic to
a hypertonic state, ortheir tone may appear normal.
 Pallor, cyanosis, apnea, a slow heart rate, andunresponsiveness
to stimulation are also signs of HIE.
Note: Cerebral edema maydevelop during the next 24 hr and result in

profound brainstem depression.
 During this time, seizure activity may occur; it may be severe
and refractoryto the usual doses of anticonvulsants.
Note: Though most often a result of the HIE,seizures in asphyxiated

newborns may also be a result of
Clinical spectrums of HIE includes mild, moderate or severe according

to Saranat stages of HIE
Signs STAGE 1 STAGE 2 STAGE 3

Level of Hyper alert Lethargic Stuporous, coma
consciousness
Muscle tone Normal Hypotonic Flaccid
Posture Normal Flexion Decerebrate
Tendon Hyperactive Hyperactive Absent
reflexes/clonus
Myoclonus Present Present Absent

Moro reflex Strong Weak Absent
Pupils Mydriasis Miosis Unequal, poor
light
reflex
Seizures None Common Decerebration
Electroencephalog Normal Low voltage Burst
raphic changing to suppression to
findings seizure isoelectric
activity
Duration <24 hr if 24 hr-14 days Days to weeks
progresses;
otherwise,
may
remain
normal
Outcome Good Variable Death, severe
deficits
CLINICAL MANIFESTATIONS OTHER THAN CNS

- Renal failure: Oliguria
- Respiratory distress,
- Tachycardia
- Cardiomegaly
- Hepatomegaly
- Shock
- Necrotizing enterocolitis /Bloody stool
- Hypoglycemia
- Hypocalcemia
LABORATORY EVALUATION
 CBC
 RBS
 Urine analysis
 Stool for blood
 Renal function
 Liver function test
 Echocardiography as needed
 Serum electrolytes
 EEG
 CXR
 Brain imaging
MANAGEMENT OF ASPHYXIA
 Perinatal management of high risk pregnancies and early

detection offetal hypoxia
 Neonatal resuscitation
 Postnatal management of asphyxia
 Cardiorespiratory support
 Fluid Management
 Oxygenation
Cooling therapy (standard Rx for HIE). But not feasible in our country
 Correction of Metabolic States (hypoglycemia,
hypocalcemia…)
 Seizure Treatment
 Parent counseling has to be the integral part of
management
PROGNOSIS OF HIE
 Stage I(mild HIE) 98- 100% of newborns will have a normal

neurologicaloutcome and < 1% mortality
 Stage II(moderate HIE) 20-37% of them die or have
abnormalneurodevelopmental outcome.
 Stage III(Severe HIE) death is more likely survivors would
have one ormore major neurodevelopmental disability such
as Cerebral palsy,intellectual disability, visual impairment
or epilepsy.
Respiratory distress syndrome(HMD)
RDS
due to
Deficiency of pulmonary surfactant

primarily in premature infants
Note: inversely related to gestational age and birthweight.

 It occurs in 60-80% of infants <28 wk of gestational age, in
15-30% of those between 32 and 36 wk of gestational age, and
rarely in those>37 wk of gestational age.
THE RISK FOR DEVELOPMENT OF RDS ARE:
Prematurity (about 60%)

maternal diabetes: -
Maternal fetal reduced fetal

hyperglycemia hyperinsulinemia cortisone
cesarean delivery:-
 Due to lack of stressful delivery leads to reduced
fetal cortisone.
Precipitous delivery:- for the same reason
Perinatal asphyxia:-
 Due to hypoxemia ofalveolar cells typeII.
Hypothermia
maternal history of previously affected infants.
male sex
 The risk of RDS is reduced in pregnancies with chronic

orpregnancy-associated hypertension, maternal heroin use,
prolonged ruptureof membranes, and antenatal
corticosteroid prophylaxis.
PATHOPHYSIOLOGY
deficiency of pulmonary
surfactant,

higher surface tension
Lowlung volume, and
decreased compliance.
ventilation andperfusion
mismatch
Hypoxemia
Note: Metabolic acidosis also may be present due to lactic acid

production fromanaerobic metabolism, in response to hypoxemia and
compromised tissue
 Signs of respiratory distress:
 Tachypnea, retractions, grunting even cyanosis in

severe cases.
 Develops within hours after birth (4-12 hours).
 Progressively increases to reach the peak at the 3rd
day oflife.
 Chest Auscultation:- In severe cases
 diminished air entry
 bilateral fine basal crepitations

 Course:
o In mild cases: -gradual improvement occurs after the 3rd
day.
o In severe cases: - end in death or complications.
 COMPLICATIONS
Complication of the disease Complication of

treatment
- Hypoxia & acidosis .J, myocardial - Pneumothorax
contractility - Pulmonary hemorrhage
cardiogenic shock - Chronic lung disease
- Patent ductus arteriosus due to hypoxia - Retinopathy of
- Intra ventricular hemorrhage. prematurity
INVESTIGATIONS
Suspected clinically in cases with respiratory distress in presence of

risk factors
 Chest X-ray:
 Mild RDS:
 Diffuse reticula-nodular infiltrates (ground glass
appearance)
 Air bronchogram: outline of air filled large airways against
opaque lungs
 Small lungs volumes
 Severe RDS:- White lungs (opacification of both lungs).

 Shake test:
 Done on gastric aspirate before baby is one-hour age
 Absence of bubbles ………. indicate absent surfactant
 Incomplete circle of bubbles ……. intermediate risk of
RDS
 Double rows of bubbles or more ……no risk of RDS
(mature lung)
 Arterial blood gases:

 In severe RDS: hypoxemia+ hypercapnia+ respiratory
acidosis
 Sepsis workup: Blood picture &blood culture to rule out early

onset sepsis.
Prevention of RDS
TREATMENT
I. Supportive measures
 Incubator care: with frequent monitoring of vital signs &
arterial blood gases.
 Respiratory support
-Oxygenation
 Support circulation
- IV fluids.
- Correct hypotension (plasma, albumin, dopamine).
 Support nutrition
 Symptomatic treatment: - correct metabolic acidosis and
anemia.
II. Specific treatment

 Antibiotics
Why indicated :- because it is difficult to differentiate RDS
from congenital pneumonia
 Surfactant
Indications:
 Immediate after birth for very low birth weight
(prophylactic treatment).
 Cases of established RDS (rescue treatment)..

C. Neonatal jaundice(hyperbilirubinemia)
 Neonatal jaundice :-is a yellowish discoloration of the
skin and or sclera due
to bilirubin deposition.
 Normal cord bilirubin is less than 3 mg/dl.
 Jaundice is obvious clinically in neonate when serum
bilirubin exceeds 5 mg/dl ;versus 3 mg /dl in adults.
BILIRUBIN METABOLISM
Old
RBC's
Iron Heme Globin Amino acid

pool
Heme oxygenase
Bilirubin (lipid soluble,

Biliverdin
water insoluble)
PHYSIOLOGIC VS PATHOLOGIC JAUNDICE
No Features Physiologic Pathological

Jaundice Jaundice
1 Clinical onset of >24 hrs <24 hrs
jaundice (after
birth)
2 Jaundice still Term < 8 days Term ≥8 days
clinically visible Preterm < 14 days Preterm > 14 days
(day after
birth)

3 Peak Total Serum Term < 12 mg/dl Term > 12 mg/dl

Bilirubin ( TSB) Preterm < 15 mg/dl Preterm > 15
mg/dl
4 Rise in TSB < 5mg/dl/24 hrs > 5mg/dl/24 hrs
5 Conjugated serum <2mg/dl >2mg/dl or 15 % of
bilirubin level TB
ETIOLOGY
In general the common cause of jaundice are:-

 Physiologic jaundice
 Hemolytic
 Sepsis
 Internal hemorrhage
 TORCHS infection
 Breast milk
 Breast feeding jaundice
 Cigler najar
 Gilbert syndrome
 Biliary atresia
 The cause of jaundice categorized based on metabolism of

bilirubin
A. Increased production:- most common cause of

pathologic indirecthyperbilirubinemia:
 Isoimmune-mediated hemolysis (eg, ABO or Rh(D)
incompatibility).
 Inherited red blood cell membrane defects (eg,
hereditary
spherocytosis and elliptocytosis).
 Sepsis (hemolysis)
 polycythemia or sequestration of blood within
aclosed space
 ineffective erythropoiesis (macrosomic infant)
B. Decreased clearance:-Inherited defects in the UGT1A1

gene,
 Crigler-Najjar syndrome: it can be relative or

absolute UGTdeficiency.

 Gilbert's syndrome. mutation in the promoter

regionof the UGT1A1 gene (unconjugated
hyperbilirubinemia.)
C. Breast milk jaundice:-
Physiologic jaundice beyond the first week of age.

2% of breast feed term baby. ( typicallyfirstthree to
five days of life).
If nursing is discontinued, the serum bilirubin level
fallsrapidly.
caused by a factor, thatpromotes an increase in
intestinal absorption of bilirubin.
Beta-glucuronidase is one proposed substance as it
deconjugatesintestinal bilirubin, increasing its
ability to be absorbed (ie, increasingenterohepatic
circulation).
D. Breast feeding jaundice:- caused by decrease intake

resulting inexcessive weight and fluid loss. (1st seven days of
life)
 lactation failureleads to inadequate intake with

significant weight and fluid lossresulting in
hypovolemia.
 This causes hyperbilirubinemia (jaundice) and
in some cases, hypernatremia defined as a serum
sodium >150 mEq/L.
E. Intestinal obstruction:-Ileus or anatomic causes of

intestinalobstruction increase the enterohepatic circulation
of bilirubin and resultin jaundice.
 CAUSE OF JAUNDICE BASED ON DURATION
1. In the 1st 24hr

Hemolysis secondarily to Rh and ABO incompateblity
and heridatoryhemolysis
Sepsis
Con-sealed hemorrhage
TORCH infection
Crigler najar syndrome

Drug(oxitosin,diazepam and sulphonamide)
2. Between 24-72hr
 It is time for physiologic jaundice but can be aggravated

byprematurity, hypothermia, hypoglycemia, hematoma,
drug, breast feeding andPNA
3. Between 3-14 day
Sepsis
Breast milk and breast feed jaundice
Torch infection
Bile ducte abnormality
Metabolic disease(galactosemia,CF)
HPS
Hypothyroidism
 Apparent in a cephalocaudalprogression, starting on
face≈ 5 mg/dL abdomen≈ 15 feet, ≈ 20 mg/dL),

mg/dL
(as serum level increases)
Kernicterus
Kernicterus, or bilirubin encephalopathy:-
 neurologic syndromeresulting from the deposition of
unconjugated (indirect) bilirubin inthe basal ganglia and brain
stem nuclei.
 Disruption of the blood–brain barrier by disease, asphyxia, and
otherfactors and maturational changes in blood–brain barrier
permeabilityaffect risk.
 occurs only in infants with a bilirubin>20 mg/dL.
 The more immature theinfant is, the greater the susceptibility to
kernicterus

 appear 2-5 days after birthin term infants and as late as

the 7th day in preterm infants.
 The early signs may be subtle and indistinguishable
fromother acute systemic illnesses
 The common initial signs are:-
o Lethargy
o poor feeding, and
o loss of the Moro reflex
 Subsequently, theinfant may appear

o gravely ill and prostrate,
o with diminished tendonreflexes and respiratory
distress.
o Opisthotonos with a bulging fontanel,
o twitching of the face or limbs, and
o a shrill high-pitched cry may follow.
NOTE: In advanced cases, convulsions and spasm occur, with
affected infantsstiffly extending their arms in an inward rotation with
the fists clenched
 Later in the 1st yr, opisthotonos,muscle rigidity, irregular
movements, and convulsions tend to recur.
 In the 2nd yr, the opisthotonos and seizures abate, but
irregular, involuntary
movements, muscle rigidity, or, in some infants, hypotonia
increase steadily.
 By 3 yr of age, the complete neurologic syndrome is often
apparent.
ACUTE BILIRUBIN ENCEPHALOPATHY HAS THREE PHASES:
 Phase -1 ( 1st – 2 Days Of Age):

oPoor motor reflex
ohigh pitched cry,
odecreased tone,
olethargy,
opoor feeding
 Phase- 2 (middle of 1st week of age):
oHypertonia,
oseizure and depressed sensorium,
ofever,
oopisthotonos posturing,

oparalysis of upward gazing.

 Phase -3 (after 1week of age):
oHypertonia decreases,
oHearing and visualabnormality,
opoor feeding,
oAthetosis and seizure may also occur
Chronic form of bilirubin encephalopathy (Kernicterus):

 It is seen after 1 year of age and manifests with:-
oChoreoathetoid cerebral palsy
oUpward gaze palsy
oSensorineural hearing loss
oThe intellect may be spared with severe physical handicup
DIAGNOSTIC APPROACH
 Assess important risk facter for pathologic jaundice and do the

following
investigation:
 Serum total and direct bilirubin

 HCT,hgb or CBC
 RPI or reticulocyte count
 Maternal and neonate BG and Rh
 Peripheral morphology
 Liver fuction test and abdomenal ultrasound if
conjugated bilirubin is
elevated
 Coomp test
 indirect Coombs test (ie, determination of antibodies in the

plasma)
it is a technique used to now if maser is sensetized for Rh
antigen and prodused anti Rh antibody

MANAGEMENT
 Principles of treatment include

oAny factor that make the CNS more affected should
becorrected(hypoglycemia,hypothermia and hypoxia)
oAvoid drug interfere with metabolism of bilirubin
oDrug therapy like phenobarbe to increase conjugation
oImproving the frequency and efficacy of breastfeeding
orsupplementing inadequate breastfeeding with formula
oLowering serum bilirubin by
oExchange transfusion
oPhototherapy

Sample History 1
C/C: fast breathing of 1day duration
HPI:
This is a 23 days old male neonate from 35yrs old Para v (4 alive,1
dead) mother who doesn’t remember her LNMP but claims to be
amenorrheic for the past 9months. She is housewife who lives with
her husband. She had ANC follow up at local health center 4times and
there was no abnormality detected. She has no history of smoking,
drinking alcohol and medications intake other than ordered by
physician. She doesn’t know her blood group and had no history of
fever, headache, foul smelling vaginal discharge, DM, HTN, and TB
during current pregnancy. The baby was delivered at this hospital by
SVD and the duration of labor was 8hr and rupture of membrane was
immediately before delivery. The baby cried immediately after the
birth and cord care was given. The breast feeding was initiated 1hr
after birth and there was no prelacteal feeding. The baby was well
looking for the past 22 days at which time he started to develop fast
breathing and grunting. Associated with this he has history of high
grade intermittent fever, failure to suck and high pitched cry. There
was no history of catheterization and bottle feeding. They live in house
with 2 rooms and 3 windows and kitchen is separated from main
house. There is no infected family member but the baby has contact
with coughing individual from their neighbor. Otherwise he has
-no history of abnormal body movement
-no history of vomiting and bloody stool
-no history of excessive sweating
-yellowish discoloration of skin

Sample history 2
C/C: yellowish discoloration of skin of 3days duration
HPI:
This is a 21days old male neonate from 31yrs old Para 2 (both alive)
mother who doesn’t remember her LNMP but claims to be
amenorrheic for the past 9months. She is merchant who lives with
her husband. She had ANC follow up at local health center 4times and
there was no detected abnormality during follow up. Her blood group
is A- and received anti D antibody at the time of her first delivery. She
has no history of smoking, drinking alcohol and medications intake
other than ordered by physician. She had no history of fever,
headache, foul smelling vaginal discharge, DM, HTN, and TB during
current pregnancy. The neonate was delivered at this hospital by SVD
and the duration of labor was 6hr and rupture of membrane was
immediately before delivery. The baby cried immediately after birth
and cord care was given and breast feeding was initiated 30 minutes
after birth. There was no prelacteal and bottle feeding and baby is
vaccinated for his age. The baby was well looking for the past 9days at
which time he started to develop yellowish discoloration of skin which
started from the face and later involved extremities. Associated with
this he has history of low grade intermittent fever. There was no
infected family member and contact with infected individuals. They
live in house with 2 rooms and 3 windows and the kitchen is
separated from main house. Otherwise he has
- No history of fast breathing and grunting
- No history of abnormal body movement
- No history of excessive sweating and feeding interruption
- No history of bloody stool and abdominal distention
- No history of failure to suck


CHAPTER 18
APPROACH TO ANEMIA
Definition
 Anaemia is defined as an Hb level below the normal range. The
normal range varies with age and sex. (See table below)
Table. Normal Mean and Lower Limits of Normal for Hemoglobin,
Hematocrit, and Mean Corpuscular Volume.
 51% of under 5yr children in developing country are anemic

(different degrees)-(WHO
Classification of Anemia
There are many methods of classification of anemia but the popular
ones are
1. Morphologic classification
Based on the MCV value and microscopic appearance

Normocytic Microcytic Macrocytic Anemia

Anemia Anemia
Aplastic anemia Iron deficiency Normal newborn

Leukemia Thalassemias Vitamin B12 or olate
deficiency
Drug induced Lead toxicity
Down syndrome
Hemophagocytic Anemia of chronic
syndromes disease Hypothyroidism
(occasionally)
Acute infection Copper deficiency
Drugs (zidovudine,
Transient Sideroblastic
chemotherapy)
erythroblastopenia anemia
Chronic liver disease
Anemia of chronic
disease (Early Stage)
Renal disease
Hypothyroidism
Drug induced
2. Physiologic classification
There are 2 major categories:
A. Increased destruction of RBCs and Blood loss
 Hemolytic anemia
Cellular-
 membrane defects - Hereditary spherocytosis
 Enzyme defects - G6PD deficiency, pyruvate kinase
def.
 Hemoglobinopathies - Sickle cell anemia,
thalassemia
Extra cellular –
 autoimmune hemolytic anemia
 Hemolytic Dx of the new born
 Drug induced

 Fragment hemolysis – DIC, hemolytic uremic

syndrome, TTP, prosthetic heart valve
 hypersplenism
 Plasma factors -liver Dx ,abetalipoprotienemia
 Infections, toxins &venoms
 Blood loss
Occult Bleeding - Blood in thr feces that is not visibly
apparent ie. Hidden. They can be seen in different
causes of Upper GI or Lower GI bleeding.
Overt Bleeding - as in leech infestation and bleeding
disorders
B. Decreased production
Mainly caused by nutritional deficiency, infections and chronic
illnesses
 IDA
 Folate & VB12 def.
 Anemia of chronic illness
 Physiologic anemia of infancy
 Aplastic /hypo plastic anemia-secondary to infections ex.
parvovirus B19
 Drugs, radiation, immune mediated
 Congenital hypo plastic anemia (diamond-Blackfan anemia)
Approach to the Patient

History and physical examination
1. General approach
Usually careful history and physical examination (Hx and P/E)
suggest the type of Anemia the patient have. Clinical findings
generally do not become apparent until the hemoglobin level falls to
<7-8 g/dL.
History
The symptoms may varry on the degree of the Anemia and the
etiologic agents.

 AGE: The age of the patient is important to consider because

normal values of hematocrit (HCT) and hemoglobin (HGB) vary
greatly with age, because different causes of anemia present at
different ages.
oIf Age at onset is at birth usually- Hemolytic Anemia of the
newborn
 ask maternal & Neonate’s blood group.
 Ask the Gestational Age
 Presence of Jaundice.
In Anemia of Prematurity, the anemia will be due to

oInadequate Erythropoietin production.
oReduced red cell lifespan.
oFrequent blood sampling whilst in hospital.
oIron and folic acid deficiency (LBW infants are at
risk of IDA & folate def. early
oAnemia at 2-3 months of age- usually physiologic anemia

oPeak age for folate def. 4-7 months
oPeak age for IDA 9-12 months. Screening for iron deficiency
anemia is recommended in all children at 9 to 12 months of
age.
oIron deficiency rare in the absence of blood loss before 6 mo
in term or before doubling birth weight in preterm infants
oNeonatal anemia with reticulocytosis suggests hemolysis
or blood loss: with reticulocytopenia it suggests bone
marrow failure or suppression (as in Congenital infection
with parvovirus B19 and Congenital red cell aplasia or
Diamond–Blackfan anaemia)
oSickle cell anemia and β-thalassemia appear as fetal
hemoglobin disappears (4–8 mo of age)
oCommon causes of Pathologic anemia in newborns include:
blood loss, immune hemolytic disease (ie, Rh or ABO
incompatibility), congenital infection, twin-twin
transfusion, and congenital hemolytic anemia (eg,
hereditary spherocytosis, glucose-6-phosphate
dehydrogenase [G6PD] deficiency)

Physiologic Vs Pathologic Anemia

 At birth, normal full-term infants have higher hemoglobin (Hb)
levels and larger red blood cells (RBCs) than do older children
and adults.However, within the 1st wk of life, a progressive
decline in Hb level
begins and then persists for 6-8 wk. The resulting anemia is
known as the Physiologic Anemia Of Infancy.
 Pathologic anemia in newborns and young infants is
distinguished from physiologic anemia by any of the following:
 Anemia (HGB <13.5 g/dL) within the first month of life
 Anemia with lower HGB level than is typically seen with
physiologic anemia (ie, <9 g/dL)
 Signs of hemolysis (eg, jaundice, scleral icterus, or dark urine)
or symptoms of anemia (eg, irritability or poor feeding)
 SEX
oSome inherited causes of anemia are X-linked (eg, G6PD

deficiency and X-linked sideroblastic anemia) and occur
most commonly in males.
oIn postmenarchal girls, excessive menstrual bleeding is an
important cause of anemia.
 FAMILY HISTORY AND GENETIC CONSIDERATION
oX linked: G6PD deficiency
oAutosomal dominant: spherocytosis
oAutosomal recessive: sickle cell, Fanconi anemia
oFamily member with early age of cholecystectomy (bilirubin
stones) or splenectomy: hemolysis
 DIETARY HABIT
oCow’s milk diet predisposes to iron deficiency

oStrict vegetarian predisposes to vitamin B12 or iron
deficiency
oGoat’s milk is a poor source of folate (Afar &Somali region)
oKwashiorkor- Folate and other Micronutrient deficiency
oPica: plumbism and iron deficiency
oCholestasis: malabsorption and vitamin E deficiency
 DRUG INTAKE

oG6PD -susceptible agents

oImmune-mediated hemolysis (e.g., penicillin)
oBone marrow suppression
oPhenytoin: increases folate requirements
 Symptoms of MALABSORPTION SYNDROMES (Like, Diarrhea, weight
loss, flatulence, abdominal bloating, abdominal cramps, and
pain)
oMalabsorption of vitamins B12 and E and iron
oInflammatory bowel disease predisposes to Anemia Of
Chronic Disease or Blood Loss
oMilk protein allergy-induced blood loss
oIntestinal resection and vitamin B12 deficiency
 BLOOD LOSS or History of bleeding disorder.
oHx of blood loss (occult or Overt)
 GI - Hookworm infection, severe dysentery, IBD, PUD
diverticular diseases, hemorrhoids
 GU - Hematuria, schistosomiasis
 History of BAREFOOT walking (hookworm infestation rate is
25%in children under 5yr of age)
 Symptoms of CHRONIC ILLNESSES AND INFECTIONS.
oGiardia causes iron malabsorption
oIntestinal bacterial overgrowth (blind loop) is a risk for
developing vitamin B12 deficiency
oFish tapeworm is also a risk factor for vitamin B12 deficiency
oEpstein–Barr virus, cytomegalovirus cause bone marrow
suppression
oMycoplasma and hemolysis
oParvovirus is strongly associated with bone marrow
suppression
oChronic infection like TB and RVI
oEndocarditis
oMalarial attacks
oHepatitis and aplastic anemia
 GROWTH AND DEVELOPMENT (Assessing developmental Milestones)
 Hx of JAUNDICE and Gallbladder disease,
Symptoms
Early symptoms

 Malaise
 Fatigability
 Anorexia
 Poor concentration
 Pallor (When Hg <7-8 g/dL.)
 Palpitation
 Shortness of breath on Exertion
Eventual symptoms
 Mental and physical retardation
 Symptoms of congestive heart failure:
o General body swelling,
o Inability to carry out the usual childhood activities like
playing and keeping up with their peers.
 General appearance
 Acutely sick looking with anemia and fever - infectious causes
like malaria ,sepsis,
o Other diseases like Leukemia, neuroblastoma usually
present with infection.
 Chronically Sick Looking as in anemia of chronic
Illness(Disease)
 Vital signs
 Increased RR, PR & hypotension – case could be acute blood
loss or severe infection
 In chronic casesl,
o Bounding pulse, Wide pulse pressure - because of High
output state.
 Anthropometric assessment
◦ presence of Acute or Chronic nutritional Deficiency.
◦ In Neonates
▪ Measure Head Circumference to assess Subgalial
hemorrhage, Cephalhematoma
 HEENT
 Frontal bossing (Due to peripheral hematopoesis) – can be due
to Thalassemia major, severe iron deficiency.
 Eye –
o Pale Conjunctiva (Indicative of anemia)

o Icterus – indicate hemolysis, severe malaria or some liver

disease
 Tongue & buccal mucosa-
o Pallor (Indicate anemia)
o Angular stomatitis in Iron Deficiency Anemia
o Glossitis - VitaminB12 deficiency and IDA
o papillary atrophy in tongue - IDA
 LGS
 Thyroid Examination
 LN enlargement - TB, Leukemia, HIV, IMN, lymphoma
 Parotid enlargement- HIV
 CHEST
 In Anemia due to Acute blood loss, the patients may be in
Respiratory Distress.
 Basal crepitation (In care of heart failure)
 CVS
 Signs of heart failure
o Ex Gallop Rhythm, Cardiac Enlargement
 Flow Murmur (Functional Murmur) can be heard.
o Flow Murmur is a heart murmur that is primarily due to
physiologic conditions outside the heart, as opposed to
structural defects in the heart itself.
 ABDOMEN
 Organomegally - Hepatosplenomegaly (HSM)
in infants suggest- congenital infections (usually
associated with jaundice, anemia & thrombocytopenia)
o Causes - toxoplasmosis, syphilis, CMV, Rubella &
parvovirus B19
 Splenomegaly -common in malaria
infants & children
o Malignancies, TB, HIV, lymphoma, Epstein–Barr
virus, portal hypertension,
 Integumentary system:
 Skin
o Petechiae, purpura & bruising

 suggest cause of BM failure, autoimmune hemolysis

with autoimmune thrombocytopenia, hemolytic uremic
syndrome or any cause of bleeding Disorder.
o Palmar pallor
 Hair
o Silky or easily pluck able hair- HIV, Malnutrition
 Nails
o Koilonychia (Spoon nails) - Seen In IDA
 MSS:
 Fracture- Chronic hemolysis, malignancy etc
 bone tenderness- leukemia also ass. with joint
swelling
 NS
o Peripheral neuropathy - Seen in Vit B12 deficiency
Laboratory studies
The initial laboratory evaluation of the Anemic child generally
consists of
 CBC with differential,
 Platelet count,
 Peripheral morphology,
 Stool exam,
 Blood film and
 Reticulocyte count.
1. Complete blood count — The CBC provides information about
the RBCs and other cell lines (ie, white blood cells [WBCs] and
platelets). All three cell lines should be evaluated for
abnormalities.
 Hemoglobin and hematocrit
 Red blood cell indices — The RBC indices are an integral
part of the evaluation of the anemic child. These include:
o Mean corpuscular volume –
 Mean corpuscular volume (MCV) is measured directly by
automated blood cell counters and represents the mean
value (in femtoliters [fL]) of the volume of individual RBCs
in the blood sample.

 Normal values for MCV vary based upon age (infants have
increased MCV compared with older children). In
preterm infants, MCV values increase with decreasing
gestational age.
 MCV is the most useful RBC parameter when evaluating
a patient with anemia and is used to classify the anemia
as Microcytic, Normocytic And Macrocytic.
 Red cell distribution width –
 The red cell distribution width is a quantitative measure
of the variability of RBC sizes in the sample
(Anisocytosis).
 Normal values vary little with age and are generally
between 12 and 14 percent.
 Mean corpuscular hemoglobin concentration –
 The mean corpuscular hemoglobin concentration (MCHC)
is a calculated index (MCHC = HGB/HCT), yielding a
value of grams of HGB per 100 mL of RBC.
 MCHC values vary depending upon the age (infants have
higher values than older children) and sex (males have
slightly higher values than females) of the child.
 MCHC also increases with decreasing gestational age.
Anemia can also be classified on the basis of MCHC as

Hypochromic, Normochromic and Hyperchromic
2. Blood smear — A review of the peripheral smear is an essential
part of any anemia evaluation. Even if the patient's RBC indices
are normal, review of the blood smear may reveal abnormal cells
that can help identify the cause of anemia.
The following features should be noted:
 RBC size – A normal RBC should have the same diameter as the
nucleus of a small lymphocyte. This comparison will help the
investigator identify the patient with microcytosis or
macrocytosis.
 Central pallor – Increased central pallor indicates hypochromic
cells, which most often are seen in iron deficiency and
thalassemia. On the other hand, spherocytes and reticulocytes
do not display central pallor because they are not biconcave
discs.

 Fragmented cells – Although the patient's overall RBC indices

may be normal, review of the blood smear may reveal the
presence of small numbers of fragmented cells, indicating a
Microangiopathic process.
 Other features – Other anemias may be characterized by typical
morphologic abnormalities, which may go undetected without
inspection of the peripheral smear; these include:
oSickle cells, as seen in sickle cell disease
oElliptocytes, as seen in congenital
oStomatocytes, as seen in hereditary or acquired
stomatocytosis
oPencil poikilocytes, which can be seen in iron deficiency
anemia or thalassemia
oTarget cells, as seen in the various hemoglobinopathies,
including thalassemia, as well as in liver disease and
postsplenectomy
oBite cells and Heinz bodies are seen in hemolytic anemia
due to oxidant sensitivity, such as G6PD deficiency
oHypersegmented neutrophils suggest vitamin B12 or
folate deficiency.
oThe presence of early WBC forms (eg, blasts) along with
anemia should raise the suspicion of leukemia or
lymphoma.
3. Reticulocyte count —
 Identified by the presence of residual RNA.
 The reticulocyte is reported as a percentage of the RBC
population.
 After the first few months of life, the normal reticulocyte
percentage is the same as that of the adult, approximately
1.5 percent
 The simplest approach is to calculate the absolute reticulocyte
count (ARC) as follows:
 ARC = Percent Reticulocytes x RBC count/L
The ARC is an indication of bone marrow erythropoietic activity and is

used to classify the bone marrow response to anemia
 Anemia with a high ARC reflects an increased
erythropoietic response to hemolysis or blood loss.

 Anemia with a low or normal ARC reflects deficient

production of RBCs (ie, a reduced marrow response to
the anemia).
Diagnostic approach by laboratory test abnormalities

The history, physical examination, and initial laboratory tests are
used to narrow the diagnostic possibilities and guide further testing.
See fig below.
Corrected Reticulocyte Count
<2% (anemia of ≥2%

underproduction)
Mean corpuscular volume Hemolysis Blood

Intrinsic loss
Membrane defect*
Enzyme defect†
Hemoglobinopathy
(Sickle cell anemia)
Low Normal High Extrinsic
Iron deficiency Vitamin B12/folate Immune-autoimmune
Thalassemia Diamond-Blackfan Infection
Lead Fanconi anemia Microangiopathy
Chronic disease
Sideroblastic Disseminated
anemia intravascular
coagulation
Hemolytic uremic
syndrome
Thrombotic
thrombocytopenic
Isolated anemia Pancytopenia purpura
Acute infection Aplastic anemia Liver disease
Transient erythroblastopenia Leukemia Paroxysmal nocturnal
Anemia of chronic disease Drug induced hemoglobinuria
Renal disease Hemophagocytic Hypersplenism
Hypothyroidism syndromes
Drug induced
Abnormalities in other cell lines — the first step in narrowing the

diagnostic possibilities is determining whether the patient has an
isolated anemia or if other cell lines (ie, white blood cells [WBCs] and
platelets [PLT]) are also abnormal

 Pancytopenia − Causes of pancytopenia in children include

leukemia, infection, myelosuppressive medications, aplastic
anemia, and hypersplenism
 Anemia with thrombocytopenia − Causes of anemia

associated with low PLT count include hemolytic uremic
syndrome (HUS), thrombotic thrombocytopenic purpura (TTP),
and Evans syndrome.
 Anemia with thrombocytosis − Iron deficiency anemia is

commonly associated with thrombocytosis but can also be
associated with thrombocytopenia. Other causes of anemia
associated with elevated PLT count include postsplenectomy
anemia and infection or inflammation.
 Anemia with leukocytosis − Causes of anemia associated with

elevated WBC count include leukemia and infection.
Prognosis
 Varies with the type of anemia and age of the patient
 Death may result because of cardiac failure
General management of anemia
 vitamins and minerals supplements ( iron tablets, folate and
vitamin b12)
 changing child's diet
 medication and/or discontinuing causative medications
 treatment of the underlying disorder ( like malaria and
hook-worm)
 surgery to remove the spleen (if related to certain hemolytic
anemias)
 blood transfusions, if necessary (to replace significant
loss)Blood transfusions in those without symptoms is not
recommended until the hemoglobin is below 6 to 8 g/
 antibiotics (as appropriate if infection is the cause)
 stem cell transplant (for bone marrow failure, such as aplastic
anemia, Fanconi anemia or Diamond-Blackfan anemia)

 Erythropoiesis-stimulating agents
 Hyperbaric oxygen

CHAPTER 19
HEMORRHAGIC DISORDERS
Hemostasis
Definition
 Hemostasis is the active process that clots blood in areas of blood
vessel injury yet simultaneously limits the clot size only to the
areas of injury.
▪Over time, the clot is lysed by the fibrinolytic system, and normal
blood flow is restored.
▪ If clotting is impaired, hemorrhage occurs. If clotting is excessive,
thrombotic complications ensue.
▪ The hemostatic response needs to be rapid and regulated such
that trauma does not trigger a systemic reaction but must
initiate a rapid, localized response.
 The main components of the hemostatic process are the
1. Vessel Wall, Platelets,
2. Coagulation Proteins,
3. Anticoagulant Proteins, and
4. Fibrinolytic System
 The classic hemostasis has 5 stages:
 Vascular response- vessel spasm- constricts the blood
vessels and reduces blood flow in a matter of seconds
 Formation of platelet plug-Platelet adhesion and
aggregation
 Formation of fibrin plug- Clot stabilization by interaction of
thrombin and fibrinogen to form fibrin
 Limitation of clot to the site of injury-
 Reestablishing vascular patency( fibrinolysis and
vascular healing)- by the help of Plasminogen is a proenzyme
for fibrinolysis is activated to plasmin

In the normal conditions (without vessel injury or any pathology) the

procoagulants and anticoagulants are in constant balance.
Dissorders Of Hemostasis
Generally bleeding disorders may occur as a result of:
1. Qualitative or quantitative abnormalities of platelets
2. Disorders of coagulation
3. Vascular abnormalities
1. Platelet disorders (qualitative and quantitative)

A. QUANTITATIVE (THROMBOCYTOPENIA)- Platelet counts less than
150,000/mm3.
 the hallmark of platelet disorder is Muco-cutaneous
bleeding

 THE RISK OF BLEEDING CORRELATES IMPERFECTLY WITH THE

PLATELET COUNT.
 Children with platelet counts less than 20,000/mm3 are at

risk for spontaneous bleeding.
 The etiology of thrombocytopenia (quantitative platelet
disorders) may be organized into disorders of:
 Decreased platelet production,
 Increased destruction, and
 Sequestration
Decreased Production Increased Destruction Sequestration
Hereditary -ITP (Idiopathic - Hypersplenism

Thrombocytopenic Purpura)
- CAMT (Congenital - Hemangioma
amegakaryocytic - Drug-Induced
thrombocytopenia) - Hypothermia
- NATP (Neonatal Alloimmune
- TAR Thrombocytopenic Purpura)
(Thrombocytopenia with
absent radii) - Infection-Induced
- WAS (Wiskott-Aldrich - Microangiopathic hemolytic

Syndrome) anemias (TTP, HUS)
Acquired - DIC (Disseminated

Intravascular Coagulation)
- Aplastic anemia
- Kassabach-merit syndrome
B. QUALITATIVE PLATELET DISORDERS
 ACQUIRED
 Liver diseases- decreased production of proteins that are

important for platelet function
 Renal diseases- any disease that causes uremia
 Drugs- aspirin, valporic acid and penicillin
 Disorders that trigger increased amounts of fibrin
degradation products.
 Inherited
 Bernard-Soulier syndrome (GPIb complex deficiency)

 Glanzmann thrombasthenia (αIIb-β3 complex deficiency)
2. Disorders of Coagulation
• Hereditary disorders
 Hemophilia[A,B]
 Von Willebrand’s diseases (vWD)
• Acquired disorders
 Disseminated intravascular coagulopathy
 Vitamin K def.
 Liver diseases
3. Vascular abnormalities
o Henoch-Schonlein Purpura(HSP)
o Ehlers-Danlose Syndrome(EDS)
o Scurvy
o Severe malnutrition
o Vasculitis
Approach to a patient
History
 GENERAL QUESTIONS
- Determine the site or sites of bleeding,

- The severity and duration of hemorrhage, and
- The age at onset.
- Was the bleeding spontaneous, or did it occur after
trauma?
- Previous personal or family history of similar problems?
- Did the symptoms correlate with the degree of injury or
trauma?

- Does bruising occur spontaneously?

- Are there lumps with bruises with minimal trauma?
- If the patient had previous surgery or significant dental
procedures, was there any increased bleeding?
 Specific questions
 After surgery of the mucosal surfaces, like tonsillectomy or
dental extractions, the absence of bleeding usually rules
out a hereditary bleeding disorder.
 Delayed or slow healing of superficial injuries- hereditary
bleeding disorder.
 Careful menstrual history- common bleeding disorders,
such as von Willebrand disease (VWD)
 Women with mild VWD who have a moderate history of
bruising frequently have a reduction of symptoms during
pregnancy or after administration of OCP.
 History of drug intake- Aspirin and other NSAIDs, may
inhibit platelet function and increase bleeding symptoms
in patients with a low platelet count or abnormal
hemostasis.
 Outside the neonatal period, thrombotic disorders are
relatively rare until adulthood.
 In the neonate, physiologic deficiencies of procoagulants
and anticoagulants cause the hemostatic mechanism to be
dysregulated, and clinical events can lead to either
hemorrhage or thrombosis.
 If a child or teenager presents with DVT or pulmonary
emboli,
 Detailed family history must be obtained to evaluate
for DVT, pulmonary emboli, MI, or stroke in other
family members.
 Even with no family hx- consider hereditary dx

 Mucous membranes or skin (muco-cutaneous bleeding) or the

muscles and joints (deep bleeding).
 Check for the presence of petechiae, ecchymoses, hematomas,
hemarthroses, or mucous membrane bleeding.
 Patients with defects in platelet–blood vessel wall interaction
(VWD or platelet function defects) usually have mucocutaneous
bleeding-epistaxis, menorrhagia, petechiae,ecchymoses,
occasional hematomas, and less commonly, hematuria and
gastrointestinal bleeding.
 Individuals with a clotting factor deficiency, such as hemophilia
(factor VIII or factor IX deficiency), have symptoms of deep
bleeding into muscles and joints, with extensive ecchymoses
and hematoma formation.
 Patients with mild VWD or other mild bleeding disorders may
have no abnormal findings on physical examination.
 Individuals with disorders of the collagen matrix and vessel wall
may have loose joints and lax skin associated with easy bruising
(Ehlers-Danlos syndrome).
 for thrombotic disorders-asked about swollen, warm, tender
extremities or internal organs (venous thrombosis), unexplained
dyspnea or persistent “pneumonia,” especially in the absence of
fever (pulmonary emboli).
 Arterial thrombi usually cause an acute illness such as stroke,
MI, or a painful, white, cold extremity
Investigations
• Investigation done for bleeding patient are the following:
 Platelet count
 Prothrombin time (PT)
 Active partial thromboplastin time (aPTT)
 Thrombin time
 Bleeding time
 Reptilase time
 Mixing study

 Clotting factor analysis

 Platelet aggregation testing
 Platelet function analyzer
 D-Dimer
 Test for Thrombotic predisposition
 Elevated homocysteine
 Tests of the fibrinolytic system
• First do a platelet count, PT, and partial thromboplastin time
(PTT).
• If the results are normal, a thrombin time to evaluate fibrinogen
function and VWF testing should be considered.
• In individuals with abnormal screening test results, further
specific factor work-up should be undertaken
To see some of them…

 PLATELET COUNT (N= 150,000-450000/microlitre)
oPatients with a platelet count of >50,000/mm3rarely have
significant clinical bleeding.
oThrombocytosis in children is usually reactive-not associated
with bleeding or thrombotic complications.
 BLEEDING TIME.
o Bleeding time assesses the function of platelets and their

interaction with the vascular wall.
o Bleeding usually stops within 4–8 min
o Has inter-laboratory & inter-observant variations.
o If abnormal with platelet count of >100,000/mm3 consider
platelet dysfunction
 PROTHROMBIN TIME
oPT measures the activation of clotting by tissue factor

(thromboplastin) in the presence of calcium.
o An isolated abnormal prothrombin time (PT) suggests F VII
deficiency.

onormal -10-13sec
 INTERNATIONAL NORMALIZED RATIO (INR)
o PT has been standardized using the International Normalized

Ratio (INR) so that values can be compared from 1 laboratory
or instrument to another.
 PARTIAL THROMBOPLASTIN TIME.
oEvaluates the intrinsic pathway -It does not measure factor VII,
factor XIII, or anticoagulants.
oProlonged activated partial thromboplastin time (aPTT)
indicates most commonly hemophilia or FXI deficiency.
oThe prolongation of both PT and aPTT suggests deficiency of FV,
FX, FII, or fibrinogen abnormalities.
 REPTILASE TIME.
oReptilase time uses snake venom to clot fibrinogen.

oReptilase time is not sensitive to heparin and is prolonged only
by reduced or dysfunctional fibrinogen and fibrin split
products
 MIXING STUDIES.
oIf there is unexplained prolongation of PT, PTT, or thrombin

time, a mixing study is usually performed.
oNormal plasma is added to the patient's plasma, and PT or PTT
is repeated. Correction of PT or PTT by 1:1 mixing with normal
plasma suggests deficiency of a clotting factor, because a 50%
level of individual clotting proteins is sufficient to produce
normal PT or PTT.
o If the clotting time is not corrected or only partially corrected,
an inhibitor is usually present.
oAn inhibitor of clotting is-like heparin or an antibody directed
against a specific clotting factor or the phospholipid used in
clotting tests.
General management

There are three general and mainstay treatments for bleeding

disorders but specification to each disorder is required.
 RISK REDUCTION
oPatients with bleeding disorders should avoid medications

that thin the blood.
oThey may also need to make some changes to lifestyle or
activities to reduce their risk of bleeding.
oFor patients who require surgery, physicians can take steps
to reduce the risk of operative and post-operative bleeding.
 MEDICATIONS
Several drugs are available that improve blood coagulation or help

prevent clots from dissolving
 REPLACEMENT THERAPY
oPatients with moderate to severe bleeding disorders may

require transfusion of blood platelets or clotting factors.
Clotting factors may be donated human blood products or
lab-synthesized proteins. Patients with severe bleeding disorders may
receive clotting factor transfusions as a preventive measure.

CHAPTER 20
SKIN LESIONS IN PEDIATRICS
Primary skin lesions
1. Macules – alteration in skin color which cannot be felt and that is
<1 cm in diameter.
2. Patches - macuels which is>1 cm in diameter
3. Papuels - palpable solid elevated lesion<1 cm in diameter
4. Plaque - papules>1 cm in diameter
5. Nodules - palpable lesion>1cm with a round surface
6. Tumors- larger nodule that is suspected to be neoplastic in origin
7. Vesicles - raised fluid filled lesion <1cm in diameter
8. Bullae - raised fluid filled lesion >1 cm in diameter
9. Pustules - a small elevation of skin containing purulent
material(pus)
10. Wheals - flat topped, palpable lesion of variable size, duration and
configuration that represent dermal collection of edema fluid
11. Cysts- circumscribed thick walled lesions which are covered by a
normal epidermis and contain fluid or semisolid material
 Primary lesions may change to secondary lesions or secondary
lesions may develop overtime where no primary lesion excited.
Secondary skin lesions

1. Scales - dry or greasy laminated mass or compressed layer of
keratin that represent thickened stratum corneum
2. Purpura - bleeding into the skin that has red-purple
color(flat/palpable)
3. Petechiae -small purpura<2-3mm

4. Erosion - focal loss of epidermis and they heal without scarring

5. Ulcer - extend to dermis and tend to heal with scarring
6. Excoriations - ulcerated lesion inflicted by scratching
7. Fissures - caused by splitting or cracking
8. Crust - matted, retained accumulation of blood, pus or serum
usually mixed with epithelial debris on the surface of weeping
lesion
9. Scar - end stage lesion that can be thin, depressed and atrophic,
raised and hypertrophic or flat and pliable
10. Lichenification - visible and palpable epidermal thickening
with accentuated skin markings-caused by chronic irritation
(rubbing, scratching) or inflammation

Some common skin lesions

Scabies
 Causative agent-female mite called sarcoptesscabieivarhominus
 Transmitted by
ophysical contact with an affected individual I.e is affected by the
extent and duration of contact
oRarely byfomites b/c the isolated mite dies within 2-3 days
 intensive pruritus particularly at night
 Red papules (1-2mm) -----first sign
 Thread like burrow------classic lesion
i.e. may not be seen in infants (bullae and pustules are common)
 Common site
 Covered areas like:
 interdigital space
 wrist flexors
 belt line
 anterior axillary folds, umbilicus groin in males &areola in
females
i.e. face, palms and soles are spared
 in infants, scalp, neck and face are often affected

 other clue-poor response to topical antibiotic &transient
response to topical steroids
 complications (If left untreated)
 secondary infection
 eczematous dermatitis
 impetigo
 folliculitis
 cellulitis
 Glomerulonephritis…
 Latent period of 1 month follows initial infestation


 Treatment;
 Permertin>treatment of choice
 Additional therapies; lindane,crotamiton,oralivermectin(for
immune compromised)
 Clothing, bed lines&towels should be washed with hot water
 Entire family should be treated
 i.e. pruritus (hypersensitivity to mite antigens may persist for

anumber of daysto weeks
 Nodules may take several months to resolve
 Transmission is unlikely more than 24 hours after treatment
Tineacapitis
 Is dermatophytic infection of the scalp most often caused by
Trichophytontonsurans, occasionally by Microspordium
Epidimology
Common in black children age 4-14 yr.

If the patient is above age of 14 with the same compliant, We should consider
Seborric Dermatitis.)
Clinical manifestation- varies with the infecting organism
 Endothrix infection
o Caused by T.tonsurans
o An infection within the hair shaft characterized by
“black-dot ringworm”
o Initially many small circular patches of alopecia in which
hairs are broken off close to hair follicle
o diffuse scaring with minimal hair loss;resemble seborrhic
dermatitis,psoriasis, atopic dermatitis
o Kerions(elevated boggy granulomatous mass caused by
sever inflammation) which are often studded with pustules
o fever ,pain,lymphadenopathy & permanent scarring
&alopecia
o chronic alopecia

 Ectothrix infection
o Caused by some other types of Trichophyton infections
o Spores are are distributed in sheath like fashion around the
hair shaft
o M.audoini
 Initially a small papule at the base of hair
follicle,spreads peripherially,forming an erythematous
&scaly circular plaque(ring worm)
 Numerous patches of alopecia
 Severe pruritus
 Favus
 Chronic form of tineacapitis which is rare
 Caused by T.schoenleinii
 Starts as yellowish red papule at the opening of hair follicles,
the papules expand and coalesce to form cup shaped
yellowish, crusted papule that fluoresce dull green under
wood lump
Method of transmission
 mostly by contact with infected hair, combs, hats &seats

 may also be air born with in the immediate environment (school
mate &house hold members)
 zoophilic; cats and dogs<<<<< M.canis
oSeborrhic dermatitis
oPsoriasis
oAlopecia areata
oDystrophic hair disorders
Diagnosis
Wood lamp
Microscopic examination with KOH preparation
Culture
Treatment
 Oral grisofulvin

 Terbinafine
 Adjuvant therapies  vigorous shampooing with 2.5%
selenium sulfate, zinc, pyrithione or ketoconazole for patient
and potential carrier

Tungiasis
 Tungiasis is a skin infection caused by a burrowing flea
called tunga penetrans/chigao flea/jigga.
 he skin is infested by the adult through a bite on the
surface.then it burrows through the skin to the epidermis to be fed
from the blood vessels in the upper dermis.
Epidimology
 It is more common in Caribbean region, Africa, india,

Pakistan and latin America.
 Its very common in our setup.
 People who walk on bare foot in dry soil, sandy beaches,
stables and farm are at higher risk.
 Most common site is feet.

 Tungiasis by itself only caused morbidity, though
secondary infection may lead to mortality
 The clinical presentation depend on the stage of the disease
Stage 1 - Stage of Penetration (Asymptomatic)
Stage 2 (1-2 days) - Complete Penetration of the body except the

Anus part and the flea begins to feed on the host’s blood.
SYMPTOMS
 Erythema, boring pain and the curious sensation of

pleasant itching occur.
Stage 3 - maximum hypertrophy is achieved and the flea’s midsection

swells to the size of a pea
 Due to the expanding flea, the outer layer of the skin is

stretched thin, resulting in the appearance of a white halo
around the black dot (rear end of the flea) at the center of
the lesion
 Egg release is common

SYMPTOMS
 pain can be severe, especially at night or, if the nodule is

on the foot, while walking.
 Eggs will also begin to be released and a watery secretion

can be observed.
Stage 4-the flea loses its signs of vitality and appears near death
 As a result, the lesion shrinks in size, turns brown,and

appears wrinkled
 the lesion is seen as brown or black.
 Extremely itchy, erythematous skin in the parts of feet
 After this stage they may develop papules/nodules which
has apunctum(sharp point or tip with a black dot )
 The lesion can range from 4-10 mm
 Some may develop ulceration afterwards
 Multiple lesions on the feet can lead to difficulty in walking
 Complication
 Predispose to PSGN
 Secondary bacterial infection
 Tetanus
 Gangrene
Investigation
 Parasitologic diagnosis
 Extraction of the gravid flea and/ or the eggs in the lesion
is bothe diagnostic and therapeutic.
 Dermoscopy
 Typical lesion with central irregular brown discoloration
with middle plugged opening or a gray-blue discoloration can be
identified.

 Cercarial dermatitis
 Insect bites
 Sea bather’s eruption
MANAGEMENT
 General
 Tetanus toxoid vaccine
o As prophylaxis
o For patients whose TT vaccination is unknown or not
up to date
 Specific
 Observation
o Most resolve by themselves with in 2 weeks
o The flea sloughs off naturally as the skin sheds
 physical removal of the flea
 Topical ivermectin
 Cryotheraphy
 Antibiotic
o Topical---if secondary infection is suspected Systemic
----if superinfection is severe

CHAPTER 21
PEDIATRICS HIV/AIDS
Etiology
There are two types of HIV:
 HIV-1, which is found worldwide and is responsible for the

worldwide pandemic.
 HIV-1 has many subtypes, often varying in transmissibility and
virulence, as well as other characteristics.
 HIV-2 which is found mainly in West Africa, Mozambique and
Angola. HIV-2 is less pathogenic and makes little or no contribution
to pediatric AIDS.
Lifecycle
The HIV life cycle in the host cell can be divided into several steps;
1. Binding; HIV binds to cells via interaction between the HIV

envelope glycoprotein (gp120) and the host cell receptors (CD4
molecule) and co-receptors.
2. Fusion; binding results in the insertion of the trans-membrane
glycoprotein (gp41) into the cell membrane of the host cell, with
fusion of the two membranes.
3. Entry; The virus particle leaves its membrane behind (uncoating)
and the core of the virus is released into the cytoplasm of the
host cell.
4. Reverse transcription; For the virus to multiply, the viral
(single strand) RNA must first be converted into (double-strand)
DNA by reverse transcriptase.
5. Integration and replication; the viral DNA is then able to enter
the host nucleus and the viral enzyme integrase is used to insert
the viral DNA into the host cell’s DNA.
6. Budding; newly formed immature viral particles gather at the
membrane of the CD4 cells and push through the cell membrane
by budding.
7. Maturation; The gp160, embedded in the cell membrane, is

cleaved by the enzyme protease to produce functional gp41 and
gp120 to form a mature virus, which is then ready to infect a new
cell.
Transmission
 sexual contact
 Parenteral exposure to blood
 Vertical transmission from mother to child. It is primary route of
infection in the pediatric population.

Maternal and neonatal factors that may increase the risk of
HIV transmission
Pregnancy Labor and delivery Breastfeeding
• Viral, bacterial, or • Rupture of • Any exposure to
parasitic placental membranes for more breast milk
infections, such as than 4 hours • Duration of
malaria • Invasive delivery breastfeeding
• Sexually procedures that • Mixed feeding
transmitted increase contact with (giving water, other
infections (STIs) mother’s infected liquids, or solid foods
• History of past and blood or body fluids in addition to
current multiple (episiotomy, artificial breastfeeding)
sexual partners rupture of • Breast abscesses,
membranes) nipple fissures,
• Chorioamnionitis mastitis
(from untreated STI • Oral disease in
or other infection) • the baby (thrush or
Preterm delivery sores)
• Low birth weight
Natural history
 Children prenatally infected with HIV fit into one of three categories:
 Category 1 (25–30%): Rapid progressors, who die by the age of
one and who are thought to have acquired the infection in utero
or during the early perinatal period.

 Category 2 (50–60%): slow progressors Children who develop

symptoms early in life, followed by a downhill course and death
by the age of three to five.
 Category 3 (5–25%): Long-term survivors, who live beyond the
age of eight.
 Children with HIV/AIDS can present with different complaints,

Common conditions experienced by HIV-infected children are:
 diarrhea,
 acute lower respiratory tract infections,
 septicemia,
 acute suppurative otitis media,
 sinusitis, and
 failure to thrive.
 In young infants the earliest clinical signs and symptoms may be
nonspecific, such as:
 failure to thrive,
 acute respiratory infections, and
 diarrhea.
 Clinical manifestations found more commonly in children than
adults with HIV infection include recurrent bacterial infections,
chronic parotid swelling, lymphocytic interstitial pneumonitis
(LIP), and early onset of progressive neurologic deterioration.
Systemic Manifestations of HIV/AIDS

1. Diarrhea
 Acute diarrhea is one of the most common causes of morbidity and
the leading cause of death in HIV-infected children during the first
year of life.
 Diarrhea in HIV-infected children tends to be prolonged and is
usually complicated by dehydration and malnutrition.
 The infectious causes of diarrhea in HIV-infected children are
similar to the common causes in non-infected children. The leading
cause of diarrhoea is rotavirus (RV), followed by bacterial causes
that include Enterobacter, Escherischia coli, Shigella species,

Salmonella species, Campylobacter jejuni, Giardia lamblia,

Entamoeba histolytica, and Candida albicans.
 In HIV-infected children, other infectious causes of diarrhoea
include AIDS-defining illnesses such as cryptosporidiosis,
isosporiasis, cytomegalovirus (CMV) infection, atypical
Mycobacteria species, HIV enteropathy, and parasitic
infections, including Strongyloides stercoralis and Tricuris
tricuria.
2. Pulmonary conditions
The most common include:
A. BACTERIAL PNEUMONIA;
 Pneumonia is the leading cause of hospital admissions and death in

HIV-infected children. Although the typical pathogens (S.
pneumoniae, H. influenzae, Moraxella catarrhalis) are most
common, unusual pathogens such as P. aeruginosa, yeast, and
anaerobes may be present in chronic infections and result in
complications such as invasive sinusitis and mastoiditis.
CLINICAL PRESENTATION
 History of fever, cough, and fast breathing (tachypnoea) with or

without chest in-drawing (retractions), cyanosis, and lethargy
 On auscultation, crepitations, decreased breath sounds or
bronchial breathing (lobar pneumonia) may be present
 When pulse oximetry is available, persistent hypoxia is
demonstrated (oxygen [O2] saturation less than 90%).
Diagnosis – clinical
B. PNEUMOCYSTIS PNEUMONIA (PCP);
 PCP is a major cause of severe pneumonia (15–30%) and death

(30–50%) in HIV-infected infants.

 CLINICAL FEATURES
Clinical features of PCP in children include:

 Low-grade fever or afebrile
 Marked respiratory distress (chest in-drawing, rapid
progression, cyanosis, inability to drink)
 Auscultation: clear chest or diffuse fine crepitations
 Poor response to standard antibiotic treatment
 Pulse oximetry: severe persistent hypoxia.
Diagnosis - Sputum induction with nasophyaryngeal aspirates or

bronchoalveolar lavage may help in diagnosing PCP.
C. TUBERCULOSIS
 The burden of TB in children depends on the burden of the disease

in the adult population.
 These children are at increased risk of developing primary
progressive TB because of the associated severe immune
suppression resulting from their young age and HIV.
 Extrapulmonary TB is seen more often in HIV-infected children.
CLINICAL FEATURE
HISTORY
 Unexplained weight loss or failure to grow normally

 Unexplained fever, especially if more than 14 days
 Chronic cough (more than 14 days)
 Failure to respond to appropriate antibiotic treatment of
presumed bacterial pneumonia or meningitis
 Exposure to an adult with probable or definite pulmonary
infectious TB
PHYSICAL EXAMINATION;

 Fluid on one side of chest (dullness to percussion, reduced air

entry)
 Enlarged, non-tender lymph nodes or abscess, especially in the
neck
 Signs of meningitis, especially if subacute and developing over
several days
 Abdominal swelling, with or without palpable lumps
 Progressive swelling or deformity of a bone or joint, including
the spine
LABORATORY INVESTIGATIONS;
 Microscopic examination for acid-fast bacilli (ZiehlNielsen stain)

and culture of specimens, such as early morning gastric
aspirates for three consecutive days and pleural, ascitic and
cerebrospinal fluid as relevant.
 Chest radiograph for lobar opacity, pleural effusion, miliary
pattern.
 PPD tuberculin skin test (>5 mm is positive).
DIAGNOSIS OF EXTRAPULMONARY TB
 When there are superficial enlarged lymph nodes, biopsy or fine

needle lymph node aspirate microscopy and culture may be
diagnostic.
 Body fluids: ascitic, pleural, or cerebrospinal can be subjected
to microscopy, biochemical analysis, Ziehl-Nielsen (ZN)
staining and culture. The yield from ZN staining and culture is
usually poor.
 Cerebrospinal fluid contains predominantly lymphocytes and
elevated protein
 Bone marrow aspirate and culture may be diagnostic in
disseminated TB with persistent fever and wasting.

 Ultrasound can help differentiate loculated fluid and

consolidation, document ascites and intra-abdominal lymph
nodes, and identify features of pericardial TB
D. LYMPHOID INTERSTITIAL PNEUMONITIS (LIP):
 Lymphoid interstitial pneumonitis (LIP) is common in HIV-infected

children.
 It occurs in at least 40% of children with perinatal HIV, and usually
occurs in children more than two years of age.
 LIP is often mistaken for pulmonary TB (miliary) because of the
chronic cough and the miliary-like pattern on chest X-ray.
CLINICAL SYMPTOMS:
Diagnosis of LIP is usually by exclusion. However, the following may

be helpful:
 The patient is usually in good general condition despite

respiratory distress
 Recurrent cough and dyspnoea are invariably present
 Typical radiological features are usually associated with parotid
enlargement, generalized lymphadenopathy, and
hepatosplenomegaly
 Finger clubbing may be present
 Terminally chronic lung disease with hypoxia
 The child may present with cor pulmonale and/or right heart
failure.
E. OTHER PULMONARY MANIFESTATIONS;
 Bronchiectasis
 Viral pneumonitis

 Fungal chest infections (e.g. aspergillosis, nocardia, cryptococcosis,

and candida
 Kaposi’s sarcoma (KS) is the most common HIV-associated
malignancy associated with the
3. Central Nervous System

Manifestations include;
 developmental delay
 progressive encephalopathy with loss or plateau of
developmental milestones
 cognitive deterioration,
 impaired brain growth resulting in acquired microcephaly, and
 symmetric motor dysfunction.
A. HIV ENCEPHALOPATHY
 HIV encephalopathy is an encephalopathy caused by HIV infection

of the brain.
 It manifests clinically with various neurodevelopmental, cognitive,
motor, and behavioural abnormalities.
 Diagnosis is mainly clinical and depends on the presence of

least two of the following for at least two months:
 Failure to attain or loss of developmental milestones or loss of
intellectual ability
 Impaired brain growth or acquired microcephaly
 Acquired symmetrical motor deficit manifested by two or more
of the following: paresis, pathological reflexes, ataxia, or gait
disturbances
 Cerebrospinal fluid is normal or has non-specific findings and
CT scan shows diffuse brain atrophy.

B. NEUROPATHY
 It presents with dysaethesias and numbness in a ‘glove and

stocking’ distribution.
C. SEIZURES
 may result from:
 Space-occupying lesions (most often cerebral toxoplasmosis or

tuberculoma)
 Meningitis (most often cryptococcal)
 Metabolic disturbances
 No identified cause other than HIV infection
D. CNS OPPORTUNISTIC INFECTIONS:
 The most common OI in children is reportedly CMV infection.

Other viruses, especially herpes simplex and varicella-zoster
virus, can also cause acute encephalitis.
 Fungal infections, particularly candida and aspergillus
meningitis, are reported to be the second most common infection
in children. Cryptococcal meningitis is rarely seen in young
children
HIV Exposed Infants (HEI)

INTRODUCTION
 HEI: is infants born to HIV positive pregnant women.

 These infants can be infected with HIV during pregnancy, labor or
after birth through breast feeding.
 All HEI (infected and non-infected) will test antibody positive
during the first few months of life.
 HIV infected infants are susceptible to many opportunistic
infections including PCP, TB and other bacterial infections that are
associated with high rates of mortality.

 In the provision of care for these children, we use the national HIV
exposed follow-up card.
Components of clinical care for the HEI
1. History
3. Growth assessment
Children with HIV infection are at high risk for poor growth so,
growth should be monitored closely for all HIV exposed and
infected infants.
4. Developmental assessment: Use developmental check list to
assess growth & development.
5. Infant feeding: Nutrition and feeding history should be assessed
regularly.
6. Immunization: All HEI should be immunized according to EPI.
7. ARV infant prophylaxis
 For infants born to HIV infected mothers and on breastfeeding

 Initiate ART for the mother.
 Infant prophylaxis with daily NVP for 6 weeks.
 Collect specimen for DNA PCR testing at 6 weeks of age.
 For infants born to HIV infected mothers but not breast
feeding:
 Initiate ART for the mother.
 If the infant is brought within 72 hours of birth
provide NVP prophylaxis for 6 weeks; otherwise there
is no need to provide NVP syrup for the infant.
 Collect specimen for DNA PCR testing at 6 weeks of
age. Refer algorism for testing of HEI less than 18
months.
 High-risk infants are defined as those infants:

 Born to women with established HIV infection who

have received less than four weeks of ART at the time
of delivery; OR
 Born to women with established HIV infection with
viral load >1000 copies/mL in the four weeks before
delivery, if viral load measurement available; OR
 Born to women with incident HIV infection during
pregnancy or breastfeeding (incident HIV infection is
new HIV diagnosis in pregnancy or breastfeeding
woman with a prior negative HIV test during
pregnancy); OR
 Identified for the first time during the postpartum
period, with or without a negative HIV test prenatally.
8. Co-trimoxazole preventive therapy (CPT): Using pediatric
co-trimoxazole in ALL HIV EXPOSED INFANTS significantly
reduces the rate of PCP and other bacterial infections.
9. TB risk assessment: At each visit the infant should be
evaluated for Tuberculosis.
10. Determination and evaluation of infection status
 One of the goals of follow-up of HEI is to identify and treat
the HIV infected ones early. All HEI should have virologic
testing at 6 weeks of age or at earliest opportunity
thereafter.
11. Current assessment and plan Based on above assessment
 Follow-up visits and schedule of HEI
Monthly for the first six months of life then every 3 months until
infection status is determined.

WHO paediatric clinical staging of infants and children with

established HIV infection (WHO 2010)
STAGE1
 Asymptomatic
 Persistent generalised lymphadenopathy (PGL)
STAGE 2
 Unexplained persistent hepatosplenomegaly

 Extensive wart virus infection; facial, more than 5% of body area
or disfiguring
 Papular pruritic eruptions
 Fungal nail infections
 Lineal gingival erythema
 Extensive human papilloma virus (HPV) or molluscum
contagiosum (>5% of body area/face)
 Recurrent oral ulcerations (>2 episodes/6 months)
 Unexplained persistent parotid enlargement
 Herpes zoster
 Recurrent or chronic upper respiratory tract infection (URTI):
otitis media, otorrhoea, sinusitis, tonsillitis (with at least 1
episode in the last 6 months)
STAGE 3
 Unexplained moderate malnutrition (-2 SD or Z score) not

adequately responding to standard therapy • Unexplained
persistent diarrhoea (≥14 days)
 Unexplained persistent fever above 37.5 °C (intermittent or
constant) for longer than 1 month
 Persistent oral candidasis (after first 6 weeks of life)
 Oral hairy leukoplakia
 Lymph node TB
 Pulmonary tuberculosis
 Severe recurrent presumed bacterial pneumonia (current
episode plus 1 or more episodes in previous 6 months)
 Acute necrotizing ulcerative gingivitis/periodontitis

 Symptomatic lymphoid interstitial pneumonistis (LIP)

 Chronic HIV-associated lung disease including bronchiectasis
 Unexplained anaemia
STAGE 4
 Extrapulmonary tuberculosis
 Kaposi’s sarcoma
 Oesophageal candidiasis (or candida of trachea, bronchi or
lungs)
 CNS toxoplasmosis (after the neonatal period)
 HIV encephalopathy
 Cytomegalovirus (CMV) infection; retinitis or CMV affecting
another organ with onset at age over 1 month
 Extrapulmonary cryptococcosis, including meningitis
 Any disseminated endemic mycosis (extrapulmonary
histoplasmosis, coccidiomycosis)
 Chronic cryptosporidiosis with diarrhoea
 Chronic isosporiasis
 Disseminated non-tuberculous mycobacterial infection
 Acquired HIV-associated rectal fistula
 Cerebral or B cell non-Hodgkins lymphoma
 Progressive multifocal leukoencephalopathy (PML)
 HIV-related cardiomyopathy or nephropathy
Diagnosis of HIV infection

Laboratory assays: There are two types of laboratory tests:
1. Antibody tests
 Antibody tests are the most widely used HIV diagnostic tests and
provide reliable evidence of HIV infection in adults and children who
are older than 18 months.
 The HIV antibody test is less reliable in infants aged less than 18
months because they may still be carrying HIV-specific antibodies
acquired from the mother in utero.
Interpretation of test results

 In children more than 18 months of age:

 HIV infection can be confirmed in those with positive
antibody results.
 HIV infection can be excluded in those with negative
antibody results.
 HIV-exposed children who continue to breast feed should be
provided with cotrimoxazole prophylaxis and re tested a
minimum of six weeks after complete cessation of
breastfeeding before HIV infection can be excluded. In
addition, the child should be retested at any stage during
breastfeeding should features of HIV infection occur.
 In children less than 18 months of age:
 A positive antibody test (mother’s or of a child less than 18
months old) should be a trigger for virologic testing.
 DNA or RNA PCR is considered best for infant diagnosis.
 All infants born to HIV-infected women should have DNA
PCR at six weeks of age. If the test is positive, the infant is
presumed to be HIVinfected and should be referred for care
and treatment.
2. Virology tests
 In order to make a definitive diagnosis of HIV in infants less than
18 months, assays that detect the virus or its components
(virological tests) are required.
Interpretation of test results
 A negative test in a non-breastfed infant, ≥4–6 weeks old excludes

HIV infection.

 A positive test confirms HIV infection.

 HIV-exposed infants who continue to breast feed should be
provided with cotrimoxazole prophylaxis and should be re tested a
minimum of six weeks after complete cessation of breastfeeding
before HIV infection can be excluded. In addition, the infant should
be re tested at any stage during breastfeeding if features of HIV
infection occur
Treatment principles
 Treatment involves: Supportive care:
 Counselling of care-giver on the options for infant feeding (see
Infant feeding section)
 Proper feeding
 Immunization (standard)
 Growth surveillance and social support
 Early access to medical care
 Treatment of opportunistic infections
 Antiretroviral drugs (according to the national guide)

Sample history
Chief complaint: diarrhea of 2 weeks duration
HPI
This is a known RVI patient for the past 2 years on HAART and has
a good adherence. He was relatively healthy until a month back by
which time he started to experience non bloody, non-mucoid
diarrhea of about 3x per day which was about one cup per episode.
Associated with this he has crampy, diffuse abdominal pain with
no radiation of the same time duration. His mother also noticed
significant but unquantified weight loss which she noticed it
because his clothes were becoming loose. Other wise
He has no history of:
 nausea or vomiting ….. (associated symptoms)

 difficulty of swallowing, ulceration in mouth ……. (HIV
associated fungal infections)
 cough or shortness of breath….. (pcp)
 fever, night sweat ………. (MAC,TB)
 headache abnormal body movement, loss of consciousness,
body weakness …. (CNS lymphoma, toxoplasmosis, PML..)
 body swelling …….. (heart failure)
 urinary compliants …….. (HIV nephropathy)
 skin rash, ulceration …… (hsv, hzv, molluscum
contagiosum, drug reactions)
 tinnitus, vertigo, blurring of vision……….. (anemia)
 bleeding tendencies………… (thrombocytopenia)

CHAPTER 22
APPROACH TO DYSMORPHIC FEATURE
Definition
A dysmorphic feature is a difference of body structure.
 It can be an isolated finding in an otherwise normal individual,

or it can be related to a congenital disorder, genetic syndrome, or
birth defect.
 One of the key challenges in identifying and describing
dysmorphic features is the use and understanding of specific
terms between different individuals.
 Clinical geneticists and pediatricians are usually those most
closely involved with the identification and description of
dysmorphic features, as most are apparent during childhood.
 Dysmorphic features are invariably present from birth, although
some are not immediately apparent upon visual inspection.
Common Dissorders associated with Dysmorphic Features.
1. Down Syndrome
2. Edward Syndrome
3. Turner Syndrome
1. DOWN SYNDROME
 Trisomy 21 is the most common and best known chromosomal
disorder in humans and the most common cause of intellectual
disability.

Epidemiology and risk factor

INCIDENCE (1:733) prevalence are increasing b/c of increase in life
span in the last decade.
AGE-RELATED DEMOGRAPHICS occurrence is strongly dependent on

maternal age.
 15-29 years-1case in 1500 live births

 30-34 years-1 case in 800 live births
 >45 years-1 case in 50 live births
SEX-RELATED DEMOGRAPHICS; M>F (1.15:1)
 NB: 50% of female patients with trisomy 21 are fertile and these
females have up to 50% chance of having a live child who also has
trisomy 21. On the other hand, men with down syndrome are
usually infertile, except for those with mosaicism.
RACE-RELATED DEMOGRAPHICS; found in all race (African American
patients live shorter life spans white patients)
Types of down syndrome
 Down syndrome is caused by 3 the following 3 cytogenic variants;

1. Three full copies of chromosome 21 (trisomy 21)
 Prevalence; 95 %
 Origin; maternal non disjunction, with meiosis I in 97% and
the rest are paternal
2. Chromosomal translocation that results in 3 copies of the
critical region for down syndrome
 Prevalence ;4%
NB Carries high recurrence rate relative to other form which is 100% for
translocation 21;21, other t (15;21) have a 5-7% recurrence.
 The majority of translocations in Down syndrome are

fusions at the centromere between chromosomes 13, 14,
15, 21, and 22 known as Robertsonian translocations.
 The translocations can be de novo or inherited.
 It is not possible to distinguish the phenotypes of persons
with full trisomy 21 and those with a translocation.
3. Mosaicism (with some cells having 46 chromosomes)
 Prevalence;1%
 Patients who are mosaic tend to have a milder phenotype.
History
When recording the history from the parents of a child with Down
syndrome, the clinician should include the following;
 Parental concern about hearing, vision, developmental delay,

respiratory infections, and other problems
 Feeding history to ensure adequate caloric intake
 Prenatal diagnosis of Down syndrome
 Vomiting secondary to GI tract blockage by duodenal web or
atresia
 Absence of stools secondary to Hirsch sprung disease
 Delay in cognitive abilities, motor development, language
development (specifically expressive skills), and social
competence

 Diaphoresis while feeding, arrhythmia, fainting episodes,

palpitations, or chest pain secondary to heart lesion
 Symptoms of sleep apnea, including snoring, restlessness during
sleep, difficulty awaking, daytime somnolence, behavioral
changes, and school problems (b/c of macroglossia)
 Maternal and paternal age(RF)
 Family history of similar illness b/c of recurrence
 Easy fatigability, neck pain, limited neck mobility or head tilt,
torticollis, difficulty walking, change in gait pattern, loss of motor
skills, incoordination, clumsiness, sensory deficits, spasticity,
hyperreflexia, clonus, extensor-plantar reflex, loss of upper-body
strength, abnormal neurologic reflexes, change in bowel and
bladder function, increased muscle tone in the legs, and changes
in sensation in the hands and feet (symptoms of atlantoaxial
instability)
 In rare cases, the symptoms progress to paraplegia, hemiplegia,
quadriplegia, or death
 DYSMORPHIC FEATURES
oUp slanting palpebral fissures,
oepicanthic folds, and
obrachycephaly are nearly universal features of DS.
 The other dysmorphic features of DS are each present in (47 to
82 %)of cases.
 These features predominantly affect the head and neck and the
extremities.
 HEAD AND NECK;

oUp slanting palpebral fissure

oEpicanthic folds
oFlat facial profile/flat nasal bridge
oFolded or dysplastic ears
oLow-set small ears
oBrachycephaly
oBrushfield spots
oOpen mouth
oProtruding tongue
oFurrowed tongue
oShort neck
oExcessive skin at nape of the neck
oNarrow palate
oAbnormal teeth
 CVS
oEndocardial Cushing defect

oVentricular septal defect
oAtrial septal defect
oPatent ductus arteriosus
oAberrant subclavian artery
oPulmonary hypertension
 GIT
oDuodenal atresia
oAnnular pancreas
oTracheoesophageal fistula
oHirschsprung disease
oImperforate anus

oNeonatal cholestasis
 Extremities
oShort broad hands
oIncurved fifth finger with hypoplastic mid phalanx
oTransverse palmar crease
oSpace between the first and second toes (sandal gap)
oHyper flexibility of joints
 SKIN
oCutis marmorata
 NEONATAL FEATURES
 Ten of the characteristic dysmorphic features are common in

newborns with DS and are usually recognized soon after
birth.
 These 10 criteria are called HILL’S CRITERIA used to
diagnosis down syndrome in the absence of prenatal
diagnosis.
 In the presence of 8 criteria we can diagnosis a full-blown
down syndrome
 Flat facial profile

 Slanted palpebral fissures
 small, dysplastic ears
 Hypotonia
 Poor Moro reflex
 Dysplasia of midphalanx of fifth finger
 Transverse palmar (Simian) crease
 Excessive skin at nape of the neck

 Hyperflexibility of joints
 Dysplasia of pelvis
Complications
Complications of Down syndrome involve almost every organ system of

the body.
 HEENT
 Congenital or acquired hearing loss

 Serous otitis media
 Refractive errors (myopia)
 Congenital or acquired cataracts
 Nystagmus
 Strabismus
 Glaucoma
 Blocked tear duct
 Sinusitis and nasopharyngitis may occur secondary to
narrow nasal passages and sinuses.
 CARDIOPULMONARY Cardiovascular complications are
important in Down syndrome.
oAcquired mitral, tricuspid, or aortic valve regurgitation
oEndocarditis
opulmonary arterial hypertension
omitral valve prolapses
oObstructive sleep apnea secondary to enlarged tonsils or to
other causes of upper airway obstruction.
 GASTROINTESTINAL

oCeliac disease
oGastroesophageal reflux
oInfants with oral-motor difficulties may present with choking
and gagging on feedings,
oDysphagia may affect children as well as adults.
oChronic constipation.
oDelayed tooth eruption
 MUSCULOSKELETAL
oAtlantoaxial instability defined as increased mobility of the

cervical spine at the level of the first and second vertebrae,
can lead to subluxation of the cervical spine
oHip dysplasia
oSlipped capital femoral epiphyses
oAvascular hip necrosis
oRecurrent joint dislocations (shoulder, knee, elbow, thumb)
 ENDOCRINE
 Congenital or acquired hypothyroidism

 Diabetes mellitus
 Infertility
 Obesity
 Hyperthyroidism
 HEMATOLOGIC
 Transient myeloproliferative syndrome

 Acute lymphocytic leukemia
 Acute myelogenous leukemia

 CUTANEOUS
 Hyperkeratosis
 Seborrhea
 Xerosis
 Perigenital folliculitis
 NEUROPSYCHIATRIC
oDevelopmental delay
oSeizures
oAutism spectrum disorders
oBehavioral disorders (disruptive)
oDepression
oAlzheimer disease (Alzheimer disease are present in almost
all individuals with Down syndrome by age 40 years)
 Abuse
 Individuals with Down syndrome are at high risk for

physical and sexual abuse
Investigation
 The diagnosis of Down syndrome is most commonly made by
prenatal screening followed by definitive diagnostic testing.
 When prenatal diagnosis has not been made, down syndrome is
usually apparent from the clinical examination of the newborn.
 Diagnosis should be confirmed through chromosomal analysis.

 Since Down syndrome is associated with multisystem

involvement, additional diagnostic studies are performed as
appropriate.
Prenatal Screening and Diagnosis

 Prenatal screening using a combination of maternal serum
biomarkers and ultrasonography can detect up to 95% of
pregnancies affected by Down syndrome. The false positive rate is
5%.
Recently updated guidelines from the American College of
Obstetricians and Gynaecologists state the following:
 all women should be offered screening for aneuploidy before 20

weeks' gestation
 All pregnant women, regardless of their age, should have the
option of diagnostic testing.
FIRST-TRIMESTER SCREENING
 For pregnant women, for whom an early diagnosis is important,

a first-trimester "combined test" performed at 11-14 weeks
involving sonographic testing for NT together with testing for
PAPP-A and hCG provides a detection rate of 82-87% for Down
syndrome.
SECOND-TRIMESTER SCREENING
 Tests used for second-trimester screening include the triple and

quadruple screens. The triple screen measures serum hCG, AFP
and unconjugated estriol to calculate the risk of Down syndrome
and can detect up to 69% of Down syndrome pregnancies.

 Currently, the quadruple test, usually performed at 15-18

weeks' gestation, is the most common screening test performed
in the second trimester.
 This screen measures inhibin A in addition to the biochemical
markers measured in the triple screen and provides an 81%
detection rate for Down syndrome.
 In addition, the quadruple test serves as a screening test for
open neural tube defects (since it involves measurement of AFP)
and can also detect trisomy 18.
INTEGRATED SCREENING
 With integrated screening, the pregnant woman undergoes a

first-trimester screening (involving NT testing, PAPP-A, hCG)
followed by the quadruple screen in the second trimester. This
combined screening approach increases the detection rate of
Down syndrome to 95%, with a false positive rate of only 5%.
ULTRASONOGRAPHY
 Prenatal ultrasonography may reveal the following in a fetus

with Down syndrome:
 Ultrasonography soft markers for Down syndrome observed in
the second trimester include absent or hypoplastic nasal bone,
thickened nuchal fold, echogenic bowel, shortened long bones,
and pyelectasis
Commonly performed studies in individuals with Down syndrome
include the following.
CYTOGENETIC STUDIES

 The clinical diagnosis of trisomy 21 should be confirmed with

cytogenetic studies.
 Karyotyping is essential to determine the risk of recurrence. In
translocation Down syndrome, karyotyping of the parents and
other relatives is required for proper genetic counselling.
INTERPHASE FLUORESCENCE IN SITU HYBRIDIZATION
 Fluorescence in situ hybridization (FISH) may be used for

rapid diagnosis of trisomy 21. It can be successful in both
prenatal diagnosis and diagnosis in the neonatal period
MEASUREMENT OF IMMUNOGLOBULIN G
 Measurement of immunoglobulin (Ig) G levels focuses on

identifying deficiencies of subclasses 2 and 4.
RADIOGRAPHY AND ULTRASONOGRAPHY
 Current evidence does not support performing routine screening

radiographs for assessment of potential atlantoaxial instability
in asymptomatic children.
 Cervical radiography with lateral flexion and extension views)
is required to measure the atlantodens distance and to rule out
atlantoaxial instability at the age of 3 years.
 MRI is also recommended regularly for evaluation.
 Echocardiography should be performed on all infants
suspected of having trisomy 21 to identify congenital heart
disease, regardless of findings on physical examination.
INVASIVE DIAGNOSTIC TESTS
AMNIOCENTESIS,

 Routinely performed at 14-16 weeks’ gestation, remains the

criterion standard of invasive diagnostic tests.
 Testing for chromosomal disorders is 99.5% accurate.
CHORIONIC VILLUS SAMPLING (CVS) is performed at 10-13 weeks’
gestation
 The accuracy of CVS (96-98%) is less than that of midtrimester

amniocentesis, because of confined placental mosaicism and
maternal-cell contamination.
PERCUTANEOUS UMBILICAL BLOOD SAMPLING (PUBS) is approximately
95% successful in obtaining a blood sample for cytogenetic testing.
LABORATORY STUDIES
 A complete blood count (CBC) with differential and bone marrow

examination to rule out leukaemia is indicated.
 Thyroid-stimulating hormone (TSH) and thyroxine (T4) levels
should be obtained at birth, at 6 and 12 months, and annually
thereafter, to rule out hypothyroidism.
NB Guidelines for health supervision of down syndrome individuals

from birth to early adulthood have been published by the American
academy of paediatrics (read more from nelson text book of
pediatriatrics 20th edition page 614 or you can get from Medscape)
Principle of treatment

 Management of down syndrome requires an organized approach

to ongoing evaluation and monitoring for associated
abnormalities and prevention of common disorder. (for more
information check on up-to-date)
2. Edward syndrome (Trisomy 18)

Definition
Among live born children, trisomy 18 is the second most common
autosomal trisomy after trisomy 21.
 The disorder is characterized by;
• severe psychomotor and growth retardation,
• microcephaly,
• microphthalmia,
• malformed ears,
• micrognathia or retrognathia,
• microstomia,
• distinctively clenched fingers, and other congenital
malformations.
 Trisomy 18 severely affects all organ systems.
 In translocations that result in partial trisomy or in cases of
mosaic trisomy 18, clinical expression is less severe, and
survival is usually longer.

Prevalence: 1;6000-8000 live births
Mortality/Morbidity

• Approximately 95% of conceptuses with trisomy 18 die as

embryos or foetuses; 5-10% of affected children survive
beyond the first year of life.
Race
• Trisomy 18 has no racial predilection.

Sex
• Approximately 80% of trisomy 18 cases occur in females.

Age
• Trisomy 18 is detectable during the prenatal and new-born

periods.
RISK FACTORS
• The incidence rate increases with advanced maternal age.

• In approximately 90% of cases, the extra chromosome is
maternal in origin, with meiosis II errors occurring twice as
frequently as meiosis I errors.
• This is in contrast to other human trisomies, which exhibit
a higher frequency of nondisjunction in maternal meiosis I.
TYPE OF TRISOMY 18
- Full trisomy 18 is responsible for 95% of Edwards

syndrome cases.
- Mosaicism and
- translocations cause few cases.
NB; An extra chromosome 18 is responsible for the phenotype.

COMMON PHYSICAL FINDING
 Small and premature appearance

 Tight palpebral fissures
 Narrow nose and hypoplastic nasal alae
 Narrow bifrontal diameter
 Prominent occiput
 Micrognathia
 Cleft lip or palate
 Microcephaly
 Congenital heart disease (e.g., VSD, PDA, ASD)
 Short sternum, small nipples
 Limited hip abduction
 Clinodactyly and overlapping fingers; index over 3rd, 5th over
4th;
closed fist
 Rocker-bottom feet and Hypoplastic nails
 Severe developmental delays and prenatal and postnatal growth
restriction
 Premature birth, polyhydramnios
 Inguinal or abdominal hernias

NB; Only 5% live >1 year
Investigation
 First trimester non-invasive screening based on maternal age,
serum markers, and sonographic “soft markers” have
demonstrated a high sensitivity for the diagnosis of trisomy
18.
 Low levels of human chorionic gonadotrophin (hCG) and
low unconjugated estriol (uE3) in maternal serum during
mid trimester are useful predictors for an increased risk for
trisomy 18.
Laboratory Studies
Prenatal diagnosis
 Amniocentesis
 Chorionic villus sampling (CVS)
 Percutaneous umbilical blood sampling (PUBS)
Postnatal diagnosis
1.Haematological studies in patients with trisomy 18 during the

first week of life
 Thrombocytopenia
 Neutropenia:
 Abnormal erythrocyte values
 anaemia was detected in 40%, and
 polycythaemia was detected in 17%.

2.Conventional cytogenetic and fluorescence in situ

hybridization (FISH) studies
 FISH for rapid diagnosis (most laboratories, ≤24 hours) is more

sensitive for mosaicism in the neonatal period (if unknown
prenatally), followed by karyotyping, which is necessary even if
FISH confirms the diagnosis for the rare translocation;
karyotyping is also necessary if the diagnosis is made prenatally
to confirm the type of trisomy 18.
Principle of treatment
 Management of Edward syndrome requires an organized

approach to ongoing evaluation and monitoring for associated
abnormalities and prevention of common disorder.
3. TURNER SYNDROME (45, X)

 Turner syndrome is a condition characterized by
complete or partial monosomy of the X chromosome and defined
by a combination of phenotypic features.
 Half of the patients with Turner syndrome have a 45, X
chromosome complement.
 The other half exhibits mosaicism and varied structural
abnormalities of the X or Y chromosome.
Prevalence; 1 in 5,000 female live births.
Sex; occur only in female.
Race; no racial or ethnic predilections are known.

NB; Maternal age is not a predisposing factor for children with

45, X.
 In 75% of patients, the lost sex chromosome is of paternal origin
whether an X or a Y).
 It has been estimated that 95-99% of 45, X conceptions are
miscarried
COMMON FINDING ASSOICIATED WITH TURNER SYNDROME
Clinical findings in the newborns can include
 small size for gestational age

 webbing of the neck,
 protruding ears
 lymphedema of the hands and feet
Older children and adults have short stature and exhibit variable
dysmorphic features.
 Congenital heart defects (40%) and structural renal anomalies

(60%) are common.
 The most common heart defects are bicuspid aortic valves,
coarctation of the aorta, aortic
stenosis, and mitral valve prolapse.
 The gonads are generally streaks of fibrous tissue (gonadal
dysgenesis).
 There is primary amenorrhea and lack of secondary sex
characters.
 These children should receive regular endocrinologic testing.
 Most patients tend to be of normal intelligence, but intellectual
disability is seen in up to 6% of affected children.

 They are also at increased risk for behavioral problems and

deficiencies in spatial and motor perception.
 Patients with 45,X/46,XY mosaicism, can have Turner
syndrome, although this form of mosaicism can also be
associated with male pseudohermaphroditism, male or female
genitalia in association with mixed gonadal dysgenesis, or a
normal male phenotype.
 This variant is estimated to represent approximately 6% of
patients with mosaic
Turner syndrome.
Signs Associated with Turner Syndrome
 Short stature
 Congenital lymphedema
 Horseshoe kidneys
 Patella dislocation
 Increased carrying angle of elbow (cubitus valgus)
 Madelung deformity (chondrodysplasia of distal radial epiphysis)
 Congenital hip dislocation
 Scoliosis
 Widespread nipples
 Shield chest
 Redundant nuchal skin (in utero cystic hygroma)
 Low posterior hairline
 Coarctation of aorta
 Bicuspid aortic valve
 Cardiac conduction abnormalities
 Hypo plastic left-heart syndrome and other left-heart
abnormalities

 Gonadal dysgenesis (infertility, primary amenorrhea)

 Gonadoblastoma (increased risk if Y chromosome material is
present)
 Learning disabilities (nonverbal perceptual motor and
visuospatial skills) (in 70%)
 Developmental delay (in 10%)
 Social awkwardness
 Hypothyroidism (acquired in 15-30%)
 Type 2 diabetes mellitus (insulin resistance)
 Strabismus
 Cataracts
 Red-green color blindness (as in males)
 Recurrent otitis media
 Sensorineural hearing loss
 Inflammatory bowel disease
 Celiac disease (increased incidence)


FIGURE above PUFFINESS OF THE HAND AND FEET
Investigation
DIAGNOSIS
 Turner syndrome is occasionally diagnosed incidentally during

prenatal testing.
 More commonly, it is suspected based upon characteristic
clinical features.
 The diagnosis is confirmed by karyotype analysis.
 Prompt diagnosis is important to permit management of
comorbidities, including effective treatment of short stature.
Indications for testing — A karyotype analysis for Turner syndrome

should be performed in any female with characteristic features, which
vary by age group.
● Prenatal
 Cases of Turner syndrome are sometimes discovered

incidentally during chorionic-villus sampling or amniocentesis
that was performed for unrelated reasons, such as advanced
maternal age.
● Newborn period
 Turner syndrome may be apparent at birth, presenting with

congenital lymphedema of the hands and feet, webbed neck, nail
dysplasia, narrow and high-arched palate, and short fourth
metacarpal.

● Infants and children
 In infants and children, Turner syndrome should be suspected

in any female with unexplained growth failure, defined as a
growth velocity less than the 10th percentile for age or stature
that is substantially less than predicted from parental heights
● Adolescence – Turner syndrome should be suspected in adolescent

girls who fail to start or complete breast development, or those with
secondary amenorrhea, especially if short stature and/or other
features suspicious of Turner syndrome are present.
Diagnostic test
 Standard karyotype analysis

 The karyotype analysis is sufficient to establish the diagnosis
in most cases. However, in certain cases, the karyotype should
be repeated to confirm the initial result:
 If the initial karyotype is normal in a patient with a strong
clinical suspicion of Turner syndrome, a second karyotype
should be performed using a different tissue such as the skin
(fibroblasts), bladder epithelial cells in a urine sample, or
buccal mucosa cells.
 Real-time PCR
 High-throughput pyrosequencing
 Whole-exome sequencing

ADDITIONAL TESTING
Y chromosome mosaicism
Other tests
 Once the diagnosis of Turner syndrome is established by

karyotype analysis, management includes evaluation and
monitoring for associated abnormalities, including cardiac
pathology, thyroid disease, hearing and eye abnormalities, and
learning disabilities.
Key screening steps at diagnosis are:
 Renal ultrasonography.
 Comprehensive cardiovascular evaluation by a cardiology
specialist, consisting of echocardiography in infants and
children and magnetic resonance imaging (MRI) in older girls
and women.
● Laboratory tests:
Age four years and older – Serum thyrotropin (thyroid-stimulating

hormone [TSH]), to screen for chronic autoimmune thyroiditis, and
tissue transglutaminase (tTG) with total immunoglobulin A (IgA), to
screen for celiac disease.
Age 10 years and older – Fasting blood glucose, glycated hemoglobin

(A1C), alanine aminotransferase (ALT) and aspartate
aminotransferase (AST), serum creatinine, and urinalysis, because of
the risks of diabetes mellitus, fatty liver disease, and kidney
dysfunction.

Principle of management
 Management of turner syndrome requires an organized

approach to ongoing evaluation and monitoring for associated
abnormalities and prevention of common disorder.

APPENDIX

NEW WHO GROWTH STANDARDS
INTERPRETATIONS
WEIGHT FOR AGE: Weight-for-age reference data are not available beyond age 10 because this
indicator does not distinguish between height and body mass in an age period where many children are
experiencing the pubertal growth spurt and may appear as having excess weight (by weight-for-age)
when in fact they are just tall.
BMI
Overweight: >+1SD (equivalent to BMI 25 kg/m2 at 19 years)
Obesity: >+2SD (equivalent to BMI 30 kg/m2 at 19 years)
Thinness: <-2sd…… Wasted
Severe thinness: <-3 sd ……severely wasted
Prepared by Gezahegn D.
MID UPPER ARM CIRCUMFERANCE
COMA SCALES IN PEDIATRICS
REFERENCES
1. Kliegman,Stanton, St. Geme, Schor, NELSON TEXT BOOK OF PEDIATRICS,20th edition, 2016
2. Tom Lissauer, Will Carroll, Illustrated textbook of Pediatrics, 5th edition, 2018
3. KAREN J. MARCDANTE, ROBERT M. KLIEGMAN, NELSON ESSENTIALS OF PEDIATRICS, 8th edition,

2019
4. Jameson, Fauci, Kasper , Hauser, Loscalzo; HARRISON’S PRINCIPLES OF INTERNAL MEDICINE ,

20th edition : 2018
5. NATIONAL GUIDELINES FOR THE MANAGEMENT OF ACUTE MALNUTRITION, 2019
6. National guideline for comprehensive HIV PREVENTION, CARE AND TREATMENT, February 2018
7. ROBBIN’S TEXTBOOK OF PATHOLOGY,10th edition
8. Dr. Mohamed A. EL Komy, BABY NELSON PEDIATRICS, 1st Edition
9. FMOH, Pediatric Hospital Care: ETHIOPIA POCKET BOOK, Second Edition, 2016
10. POCKET BOOK OF Hospital care for children, GUIDELINES FOR THE MANAGEMENT OF COMMON
CHILDHOOD ILLNESSES, Second edition, 2013
11. EMSA AA SCOME 2012/13
12. Up-to-date 2018

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MEDSTAR

CLINICAL GUIDE AND

Chapter 1 - Introduction to Pediatrics-------------------------------------15

Pediatrics History taking------------------------------------------------------- 15

Side effects of vaccines---------------------------------------------------------- 26

Chapter 3 - GROWTH AND DEVELOPMENT------------------------------28

Causes of Abnormal Development------------------------------------------ 34

Tanner staging (Sexual Maturity Ratings)------------------------------- 40

Chapter 4 - Approach to Generalized Body Swelling---------------- 41

Conditions and differential diagnosis--------------------------------------41

SAM (Severe Acute Malnutrition)--------------------------------------------47

Prepared by Jimma University Medical Students of 2008 E.C. Batch

Chapter 5 - Approach to Renal diseases---------------------------------- 69

AKI (previously called acute renal failure)------------------------------- 69

Approach to patient with AKI------------------------------------------------- 71

ACUTE POST STREPTOCOCCAL GLOMERULONEPHRITIS

HSP (Henoch-Schönlein purpura)------------------------------------------ 83

Hemolytic- Uremic Syndrome------------------------------------------- 75

Chapter 6 - Approach to shortness of breath (dyspnea)--------- 100

RHEUMATIC HEART DISEASE---------------------------------------- 111

INFECTIVE ENDOCARDITIS-------------------------------------------- 127

Congenital Heart Diseases--------------------------------------------------- 139

ACYANOTIC CONGENITAL HEART LESIONS------------------ 145

CYANOTIC CONGENITAL HEART LESIONS-------------------- 147

Chapter 7 - Approach to cough---------------------------------------------- 171

Approach to Acute Cough---------------------------------------------------------173

Foreign body aspiration------------------------------------------------------- 188

Approach to chronic Cough------------------------------------------------------ 188

Prepared by Jimma University Medical Students of 2008 E.C. Batch

Chpter 8 - APPROACH TO WHEEZE--------------------------------------- 215

CHILDHOOD ASTHMA-------------------------------------------------------- 230

ACUTE BRONCHIOLITIS----------------------------------------------------- 246

CHapter 9 - APPROACH TO STRIDOR------------------------------------ 251

Chapter 10 - Approach to altered mental status--------------------261

Sample history for meningitis --------------------------------------------- 306

CHapter 11 - Approach To Poly Symptoms (Polyuria, Polydipsia,

Differential Diagnosis of Polyuria with Polydipsia------------------ 309

A. Diabetes Mellites (Type 1)------------------------------------------------ 310

B. Diabetes Mellites (Type 2)------------------------------------------------ 315

C. Central Diabetes Insipidus (Neurogenic)--------------------------- 322

D. Nephrogenic Diabetes Insipidus---------------------------------------322

Chapter 12 - APPROACH TO DIARHEA---------------------------------- 326

Differential diagnosis of diarrhea------------------------------------------329

Sample history for AGE------------------------------------------------------- 341

CHapter 13 - APPROACH TO ABNORMAL BODY MOVEMENT---- 343

Prepared by Jimma University Medical Students of 2008 E.C. Batch

Seizure ------------------------------------------------------------------------------ 344

Sydenham chorea--------------------------------------------------------------- 351

Sample history of Abnormal body movement-------------------------354

CHapter 14 - Approach to Body Weakness----------------------------- 356

Guillain-Barre syndrome (GBS)------------------------------------------ 370

Brain Tumor----------------------------------------------------------------------- 382

Transverse myelitis------------------------------------------------------------- 385

Myasthenia gravis (MG)------------------------------------------------------- 386

Sample History------------------------------------------------------------------- 395

Chapter 15 - Approach to fever----------------------------------------------398

CHapter 16 - Approach to Jaundice---------------------------------------408

DDx for Jaundice---------------------------------------------------------------- 411