1

Secrets of
PAEDIATRICS

By: Moses Kazevu

MOSES KAZEVU

FOREWORD
This review book is designed for use by medical students and the junior medical
doctor in the diagnosis, management as well as curative care of regional Pediatric
conditions.
The book serves as a good guide and an effective revision tool for
board/shelf/licentiate examinations.
Effort has been put in to simplify the literature and make it as practical as possible
while maintaining scientific accuracy.
Despite all efforts, it is possible that certain errors may have been overlooked in this
book. Please inform the authors of any errors detected.
NOTE: Information contained in this publication is copyrighted as such
photocopying of any part of this book without any written permission from the
author is prohibited by Law.
MOSES KAZEVU
(Tel: +260979161345)

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MOSES KAZEVU

CONTENTS
INTRODUCTION TO PAEDIATRICS AND CHILD HEALTH 4
NORMAL GROWTH AND DEVELOPMENT 5
PEDIATRIC HISTORY .................. 18
PHYSICAL EXAMINATION ........ 31
IMMUNIZATON, VACCINATION AND IMMUNIZABLE DISEASES 34
PEDIATRIC GENETIC DISORDERS 41
NEUROLOGICAL CONDITIONS 34
RESPIRATORY CONDITIONS ... 37
CARDIOVASCULAR CONDITIONS86
HEMATOLOGY ............................. 111
GASTROENTEROLOGY ............ 125
GENITOURINARY TRACT........ 216
DERMATOLOGY........................... 219
ENDOCRINOLOGY ...................... 225
INFECTIOUS DISEASES ............ 257
NEONATOLOGY ........................... 302
MISCELLANEOUS........................ 374
END OF BOOK REVISION.......... 393
INDEX ............................................... 479

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MOSES KAZEVU
CHAPTER 1: INTRODUCTION
INTRODUCTION TO PAEDIATRICS AND CHILD
HEALTH
• Paediatrics (from the Greek word
pedo pais; “a child” iatros;
“healer”) deals with the care of
children and adolescents.
• Paediatrics covers the age group
less than 18 years of age
(Benchmark used in Zambia is 14
years).
➢ Postnatal period
o 0-28 days= Neonate
o 0-1 year= Infant
o 1-3 years= Toddlers
o 3-6 years= Preschool child
o 6-12 years= School going
child
➢ Adolescence- 10-20 years
o 10-13 years= Early
o 14-16 years= Middle
o 17-20 years= Late
➢ Note: 13-19 years= teenager
• A paediatrician specializes in
health care of children and
adolescents.
• The main goal of the specialty is to
enable a child to survive, remain
healthy and attain the highest
potential of growth, development
and intellectual achievement.
• Childhood is the state when the
human being is growing and
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developing. It is time to acquire
habits, values and lifestyles that
would make children responsible
adults and citizens.
• It is important to remember that a
child is not a miniature adult.
• The principles of adult medicine
cannot be directly adapted to
children.
• Pediatric biology is unique and risk
factors of pediatric disease are
distinct.
• Clinical manifestations of
childhood disease may be different
from adults.
• Drug dosages in children are
specific and not a mathematical
derivation of the adult doses.
• Nutrition is a critical necessity for
children not only to sustain life, but
to ensure their growth and
development.
MOSES KAZEVU

CHAPTER 2: GROWTH & DEVELOPMENT

NORMAL GROWTH AND DEVELOPMENT

• Growth is an essential feature that distinguishes a child from an adult.
• The process of growth begins from the time of conception and continues until the
child grows into a fully mature adult.
• Growth denotes a net increase in the size or mass of tissue.
➢ It is largely attributed to multiplication of cells and increase in the intracellular
substance.
➢ Hypertrophy or expansion of cell size contributes to a lesser extent to the
process of growth.
• Development specifies maturation of functions.
➢ It is related to the maturation and myelination of the nervous system and
indicates acquisition of a variety of skills for optimal functioning of the
individual.
• Growth and development usually proceed concurrently. They are closely related
and so factors affecting one also tend to have an impact on the other.

GROWTH
• There are specific periods in a child’s life when the rate of growth is steady,
accelerates or decelerates.
• The fetus grows fast in the first half of gestation. Thereafter, the rate of growth is
slowed down until the baby is born.
• In the early postnatal period, the velocity of growth is high, especially in the first
few months. Thereafter, there is slower but steady rate of growth during mid-
childhood.
• A second phase of accelerated growth occurs at puberty.
• Growth decelerates thereafter for some time and then ceases altogether.

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 Table 2.1: Periods of growth (Adapted from Ghai Essential Pediatrics 8th Ed)

• The general body growth is rapid during fetal life, first one or two years of
postnatal life and also during puberty.
• In the intervening years of mid-childhood, the somatic growth velocity is
relatively slowed down.
• Order of growth is cephalocaudal and distal to proximal.
➢ During fetal life, growth of head occurs before that of neck, and arms grow
before legs.
➢ Distal parts of the body such as hands increase in size before upper arms.
• In the postnatal life, growth of head slows down but limbs continue to grow
rapidly.
ASSESSMENT OF PHYSICAL GROWTH
• Includes assessment of (Anthropometric measurements):
➢ Weight
➢ Height
➢ Head circumference/ Occipital frontal circumference (OFC)
➢ Chest circumference
➢ Mid upper arm circumference (MUAC)
• It is difficult to precisely define the normal pattern of growth.
• Generally, it implies an average of readings obtained in a group of healthy
individuals, along with a permissible range of variation.
• Most healthy children maintain their growth percentile on the growth charts as
the years pass by.

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• Significant deviation in a child’s plotted position on the growth chart can be due
to recent illness or over- or undernutrition.
• It is also important to take into account the gestation age of infants born
prematurely.
• The duration of prematurity is subtracted from the infant’s chronological age.
This correction, however is not required after 2 years of age.
WEIGHT
• The weight of a child in the nude or minimal light clothing is recorded accurately
on a lever or electronic type of weighing scale.
• Spring balances are less accurate.
• The weighing scale should have a minimum unit of 100g.
• It is important that the child is placed in the middle of the weighing pan.
• The weighing scale should be corrected for any zero error before measurement.
• Serial measurements should be done on the same weighing scale.

Figure 2.1: Beam scale for accurate measurement of weight. The child should be nude or in minimal light
clothing. (Image courtesy of Ghai Essential Pediatrics 8th Ed)

• The average birthweight of neonates is about 3kg (2.5 – 3.5 kg).

➢ During the first few days after birth, the newborn loses extracellular fluid
equivalent to about 10% of the body weight.
➢ Most infants regain their birthweight by the age of 10 days.
• Subsequently, they gain weight at a rate of approximately 30g per day for the
first 3 months of life.
• Thereafter, they gain 20g per day between 3 and 6 months.
• Between 6 and 12 months they gain 10g/day.
• The birthweight can be calculated using the following formulae:
𝑨𝒈𝒆 𝒊𝒏 𝒎𝒐𝒏𝒕𝒉𝒔+𝟗
➢ For infants <12 month=
𝟐
➢ For children >1 year= 8 + (2 x age in years)
➢ So, at 4 years:

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 o Weight= 8 + (2 x 4)
o Weight= 8 + 8
o Weight= 16 kg
HEIGHT/ LENGTH
• Length/height is recorded for children under 2 years of age.
• Hairpins are removed and braids undone.
• Bulky diapers should be removed.
• The child is placed supine on a rigid measuring table or an infantometer.
• The head is held firmly in position against a fixed upright head board by one
person.
• Legs are straightened, keeping feet at right angles to legs, with toes pointing
upward.
• The free foot board is brought into firm contact with the child’s heels.
• Length of the baby is measured from a scale which is set in the measuring table.
• Measurement of length of a child lying on a mattress and/or using cloth tapes is
inaccurate and not recommended.

Figure 2.2: Measurement of length on an infantometer. Note how the knees are gently straightened while
the head and feet are aligned (Image adapted from Ghai Essential Pediatrics 8th Ed)

• Standing height:
➢ For the standing height, the child stands upright.
➢ Heels are slightly separated and the weight is borne evenly on both feet.
➢ Heels, buttocks, shoulder blades and back of the head are brought in contact
with a vertical surface as wall, height measuring rod or a stadiometer.
➢ The head is so positioned that the child looks directly forwards with Frankfort
plane (the line joining floor of external auditory meatus to the lower margin
of orbit) and the biauricular plane being horizontal.
➢ The head piece is kept firmly over the head to compress their hair.

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 Figure 2.3: Method of recording height: Note the erect posture and the bare feet placed flat on the
ground. The back of heels, buttocks, shoulders and occiput are touching the wall. (Adapted from Ghai
Essential Pediatrics 8th Ed)

• The infant measures approximately 50 cm at birth.

➢ At 3month= 60cm
➢ At 6 months= 70cm
➢ At 1 year= 76cm
➢ At 2 years-10 years= (age x 6.5) + 76
➢ After 4 years add about 5cm for each year until they are 12 years.
• After this, increments in height vary according to the age at the onset of puberty.
There is a marked acceleration of the growth during puberty.
HEAD CIRCUMFERENCE
• Hair ornaments are removed and braids undone.
• Using a non-stretchable tape, the maximum circumference of the head from the
occipital protuberance to the supraorbital ridges on the forehead is recorded.
• The crossed tape method, using firm pressure to compress the hair is the preferred
way to measure head circumference (as shown below).

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Figure 2.4: Method of recording head circumference. Note the crossed tape method (Adapted from Ghai
Essential Pediatrics 8th Ed)

• Head growth is rapid, especially in the first half of infancy.

• It reflects the brain growth during this period.
• The head growth slows considerably thereafter.
• At birth the head circumference is 35cm (34- 37cm).
• At the first year it increases by 10cm to about 45cm (44-47cm).
➢ First 3 months it increases by 2cm per month
➢ 3-6 months it increases by 1cm
➢ 6-12 month it increases by 0.5cm per month
• At 12 years it reaches about 55cm (10cm increase)
• Note: the anterior fontanelle is diamond in shape while the posterior fontanelle is
deltoid.
• The anterior fontanelle closes by 18 months.
• The posterior fontanelle closes by 3 months.
CHEST CIRCUMFERENCE
• The chest circumference is measured at the level of the nipples, midway between
inspiration and expiration.
• The crossed tape method is recommended.

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 Figure 2.5: Method of measurement of chest circumference at the level of nipples (Adapted from Ghai
Essential Pediatrics 8th Ed)

• The circumference of chest is about 3cm less than the head circumference at birth.
(32 cm)
• The circumference of the head and chest are almost equal by the age of 1 year
(45cm).
• Thereafter the chest circumference exceeds the head circumference.
MID UPPER ARM CIRCUMFERENCE
• To measure this first mark a point midway between the tip of acromial process
of scapula and the olecranon of ulna while the child holds the left arm by his side.
• Thereafter, the crossed tape method is used for measuring the circumference.
• It should be ensured that the tape is just tight enough to avoid any gap as well as
avoid compression of soft tissue.

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Figure 2.6: Measurement of mid upper arm circumference. Note how the anatomical landmarks are first
located (Arrows) to accurately meaasure the circumference (Adapted from Ghai Essential Pediatrics 8th
Ed)

BODY MASS INDEX (BMI)

• The formula to calculate BMI is weight (kg)/ height (meter)2.
• BMI is primarily used to assess obesity.
• BMI at or above the 95th centile for age or more than 30kg/m2 is obesity.
TABLE 2.5 Approximate anthropometric
values by age
Age Weight Height OFC
(kg) (cm) (cm)
Birth 3 50 35
6 months 6 65 43
1 year 10 74 45
2 years 12 86 48
3 years 14 94 49
4 years 16 100 50

• Weight, height, head circumference (until the age of 5) and sexual maturity are
routinely monitored during under-5 clinic to assess for adequacy of growth and
development.

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• Standardized growth curves represent normal values for age for 95% of children
and are used to plot weight, height, body mass index and head circumference.
• Special growth curves exist for children with particular genetic conditions (e.g.
Down syndrome, achondroplasia).

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DEVELOPMENT
• Development refers to maturation of functions and acquisition of various skills
for optimal functioning of an individual.
• The maturation and myelination of the nervous system is reflected in the
sequential attainment of developmental milestones.
• Developmental milestones are important, easily identifiable events during the
continuous process of development e.g. turning over, sitting, reaching for object,
and pointing to objects.
• Increasingly complex skills are learnt, matching the formation of new synapses
in the brain.
• While development is a global process reflected in new motor abilities and
language, social and cognitive skills, intelligence pertains to the part of the
development dealing with cognitive or adaptive behavior.
• Intelligence is the ability to apply knowledge to manipulate one’s environment
or to think abstractly and refers to the aggregate or global capacity of the
individual to act purposefully, think rationally and to deal effectively with the
environment.

RULES OF DEVELOPMENT
• Development is a continuous process, starting in utero and progressing in an
orderly manner until maturity.
➢ The child has to go through many developmental stages before a milestone is
achieved.
• Development represents the functional maturation of the nervous system.
• The sequence of attainment of milestones is the same in all children. E.g. all
infants babble before they speak in words and sit before they stand. Variations
may exist in the time and manner of their attainment.
• The process of development processes in a cephalocaudal direction.
➢ Head control precedes trunk control, which precedes ability to use lower
limbs.
➢ The control of limbs proceeds in a proximal to distal manner such that hand
use is learnt before control over fingers.
• Certain primitive reflexes have to be lost before relevant milestones are attained.
For example, palmar grasp is lost before voluntary grasp is attained and the
asymmetric tonic neck reflex has to disappear to allow the child to turnover.

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• The initial disorganized mass activity is gradually replaced by specific willful
actions.
➢ When shown a bright toy, a 3-4 month old squeals loudly and excitedly moves
all limbs, whereas a 3-4 year old may just smile and ask for it.
• Development may be influenced by both intrinsic factors (e.g. child’s physical
characteristics, state of health, temperament and genetic attributes) and extrinsic
factors (e.g. personalities of family members, economic status, depression or
mental illness in caregivers, availability of learning experiences in the
environment, cultural setting into which the child is born).
DOMAINS OF DEVELOPMENT
• Developmental milestones provide a systematic way to assess an infant’s
progress.
• The domains of development include:
➢ Motor development
o Gross motor development
o Fine motor skill development
➢ Cognitive
➢ Social and emotional development
➢ Hearing, speech and language
• Attainment of a particular skill depends on the achievement of earlier skill.
• Delays in one developmental domain may impair development in another domain
e.g. it may be difficult to assess problem-solving skills in a child with cerebral
palsy who understands the concept of matching geometric forms but lacks the
physical ability to demonstrate that knowledge.
• Information about motor milestones should be obtained from the history as well
as from observation during the physical examination.
GROSS MOTOR FUNCTION
• Motor development progresses in a cephalocaudal direction with suppression of
primitive reflexes and development of postural tone and secondary protective
reflexes.
• The primitive reflexes include the Moro, grasp, stepping and asymmetric tonic
neck reflexes must have disappeared by 3-6 months of age before head control (4
months) and independent sitting at 6-8 months can occur.
➢ Each primitive reflex requires a specific sensory stimulus to generate the
stereotypical motor response.

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 ➢ Infants with CNS injuries show stronger and more-sustained primitive
reflexes.
➢ Postural reactions such as parachute reaction are not present at birth (i.e. they
are required). These reactions which help facilitate the orientation of the body
in space, require complex interplay of cerebral and cerebellar cortical
adjustments to proprioceptive, visual and vestibular input.
➢ Infants with CNS damage have delayed development of postural reactions.
• At birth the infant is able to turn the head side to side.
• By 2-3 months the infant:
➢ Lifts their head when lying prone
➢ Has head lag when pulled from supine position
• By 4 months the infant is able to roll over and there is no head lag when pulled
from supine position. The infant also pushes chest up with arms.
• By 6 months the infant is able to sit alone and leads with head when pulled from
supine position.
• By 9 months the infant pulls to stand and cruises.
➢ An infant stands holding on furniture.
• By 12 months the infant is able to walk.
• A delay in walking beyond 18 months of age is a warning sign in children who
have been crawling as the early locomotor pattern.
• At 18 months of age, a child can climb onto a chair and walk up and down stairs
two feet per step by 24 months of age.
• By two and half years of age a child should be able to stand on tip-toes, jump on
both feet and kick a ball.
• A 3-year-old child can walk backwards and can ride a tricycle.
• There is further development of gross motor skill and balance with age and most
children can participate in a variety of activities like swimming, skating,
gymnastics and ball games by 6-7 years of age.

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 Table 1.6: Primitive Reflexes and Postural Reaction (Adapted from BRS Pediatrics)

FINE MOTOR SKILLS

• Involve the use of the small muscles of the hands.
• An infant’s fine motor skills progress from control over proximal muscles to
control over distal muscles.
• During the first year of life, as balance in sitting and ambulatory positions
improves the hands become more available for thee manipulation of objects.
• As control over distal muscles improves reaching and manipulative skills are
enhanced.
• During the second year of life the infant learns to use objects as tools (e.g.
building blocks).
• At birth the infant keeps the hands tightly fisted.
• By 3-4 months the infant can bring the hands together to the midline and then to
the mouth.
• By 4-5 months they can reach for objects.
• By 6-7 months they can rake objects with the whole hand as well as transfer
object from hand to hand.
• By 9 months they can use the immature pincer (ability to hold small objects
between the thumb and index finger)
• By 12 months they can use the mature pincer.

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RED FLAGS IN MOTOR DEVELOPMENT
• Persistent fisting beyond 3 months of age is often the earliest sign of neuromotor
problems.
• Early rolling over, early pulling to stand instead of sitting and persistent toe
walking may all indicate spasticity.
• Spontaneous postures such as scissoring in a child with spasticity or a frog-leg
position in a hypotonic infant are important visual clues to motor abnormalities.
• Early hand dominance (before 18 months of age) may be a sign of weakness of
the opposite upper extremity associated with a hemiparesis.
• Differential diagnosis of motor delay:
➢ CNS injury
➢ Spinal cord dysfunction
➢ Peripheral nerve pathology
➢ Motor endplate dysfunction
➢ Muscular disorder
➢ Metabolic disorders
➢ Neurodegenerative conditions
LANGUAGE SKILLS
• Delays in language development are more common than delays in other domains.
• Receptive language is always more advanced than expressive language (i.e. a
child can usually understand 10 times as many words as he or she can speak).
• Language and speech are not synonymous. Language refers to the ability to
communicate with symbols i.e. sign language, gestures, writing and body
language. Speech is the vocal expression of language.
• A window of opportunity for optimal language acquisition occurs during the first
2 years of life.
• At birth the infant attunes to human voice and is able to differentially recognize
parents’ voices.
• By 2-3 months: cooing (runs of vowels), musical sounds (e.g. ooh-ooh, aah-aah)
• By 6 months: babbling (mixing vowels and consonant together) (e.g. ba-ba-ba,
da-da-da)
• By 9-12 months: Jargoning (e.g. babbling with mixed consonants, inflection and
cadence). They also begin using mama and dada (non-specific).
• By 12 months: 1-3 words, mama and dada (specific).
• By 18 months: 20-50 words, beginning to use 2 word phrases.

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• By 2 years: 2-word telegraphic sentences e.g. mommy come, 25-50% of child’s
speech should be intelligible.
• By 3 years: 3 word sentences, more than 75% of the child’s speech should be
intelligible.
• Differential diagnosis of speech or language delay:
➢ Global developmental delay or mental retardation
➢ Hearing impairment
➢ Environmental deprivation
➢ Pervasive developmental disorders including autism spectrum disorders.
COGNITIVE DEVELOPMENT
• Involves skills in thinking, memory, learning and problem solving.
• Intellectual development depends on attention, information processing and
memory.
• Infant intelligence can be estimated by evaluating problem solving and language
milestones.
• Language is the single best indicator of intellectual potential.
• Gross motor skills correlate poorly with cognitive potential.
• By 3 months the infant can move objects.
• By 6 months the infant can imitate facial expression and recognize strangers.
• By 9 months they can imitate certain gestures.
• By 12 months they can understand simple commands.
• By 24 months they can identify shapes, colors and count.
RED FLAGS IN COGNITIVE DEVELOPMENT
• Language development estimates verbal intelligence, whereas problem-solving
skills estimate nonverbal intelligence.
• If skills are delayed significantly in both language and problem solving domains,
mental retardation should be considered.
• If only language skills are delayed, hearing impairment or a communication
disorder should be considered.
• If only problem-solving skills are delayed, visual or fine motor problems that
interfere with manipulative tasks may be present.
• If there is a significant discrepancy between language and problem-solving skills,
the child is at high risk for learning disabilities.

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SOCIAL SKILLS
• Social skills are the ability to interact with other people and the environment.
• Development of social skills is dependent on cultural and environmental factors.
• Milestones, in order include:
➢ Attachment: Bonding with a primary caregiver begins at birth. Developing
empathy is critical during the first 3 years of life.
➢ A sense of self and independence: The process of separation and individuation
begins at about 15 months of age.
➢ Social play: Toddlers exhibit parallel play during the first 2 years of life. They
learn to play together and share at about 3 years of age

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Table 1.7: Domains of development

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 CHAPTER 3: PEDIATRIC HISTORY

PEDIATRIC HISTORY
• Good doctors will continue to be admired for their ability to distil the important
information from the history, for their clinical skills, for their attitude towards
patients and for their knowledge of disease, disorders and behavior problems.
• The biggest challenge you may face as you take a history in pediatrics is that most
of your clients will be too young to tell you what the problem is and most parents
are often not always 100% truthful so never take everything you hear at face value
use your best clinical judgement.
• Always greet the child and parents by name, irrespective of age as this will
convey an attitude of concern and interest.
• Introduce yourself, explain what you are about to do and gain consent.
• Considerable tact and discretion are required when taking the history especially
in the presence of the child: questioning on sensitive subjects should best be
reserved for a time when the parents can be interviewed alone.
• It may therefore be necessary to separate the parent and child-patient when taking
the history especially when the problems are related to behavior, school
difficulties and socioeconomic disturbances in the home environment.
• Information taken in the history is usually obtained from the mother however
useful information can be obtained by observing the infant and young child
during the history-taking.
• The older child should be given an opportunity to talk, to present their symptoms
and to tell how they interfere with school and play activities.
• The simple act of offering a toy, picture book or pen-torch is often an effective
step toward establishing rapport.

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• The format of a pediatric history is as follows:
➢ Demographics
➢ Presenting complaint
➢ History of presenting complaint
➢ Review of system
➢ Past medical history
➢ Drug history
➢ Birth history (pregnancy and delivery)
➢ Immunizations
➢ Nutrition and feeding history
➢ Growth and development history
o Neonatal period and infancy
o Subsequent developmental milestones
➢ Family history
➢ Social-economic history
➢ Summary
➢ Differential diagnosis

DEMOGRAPHIC DATA/IDENTITY
• This includes identifying data such as:
➢ Patient’s name,
➢ Age,
➢ Sex,
➢ Region of residence,
➢ Religion
➢ Informant (parent or patient),
➢ Date of history/examination and
➢ Indication of whether it is a hospital referral or self-referral
• Key point: observe the child at play in the waiting area and observe their
appearance, behavior and gait as they come into the clinic room. The continued
observation of the child during the whole interview may provide important clues
to the diagnosis and management.

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PRESENTING COMPLAINT
• Reason that the child is seeking medical care and duration of symptoms.
➢ What prompted referral to the doctor?
➢ Onset: the order of onset of symptoms is important. If there is doubt about the
date of onset of the disease, the patient should be asked when he/she last felt
quite well and why he/she first consulted the doctor.
➢ Duration of the symptoms- indicated as follows 1/7 (1 days), 2/52 (2 weeks),
1/12 (1 month).
➢ What do the parents think or fear is the problem?
• Presenting complaints must be recorded in the informant’s words and never
interpreted by the doctor into medical terminology.
• When the patient’s own phraseology is used, the words should be written in
inverted commas, e.g. “giddiness”, “wind”, “palpitation” and an attempt should
be made to find out precisely what they mean to the patient.
• Presenting complaints can be listed in point form e.g.:
➢ Diarrhea: 2/7
➢ Joint weakness: 1/7
➢ Vomiting: 1/7

HISTORY OF PRESENTING COMPLAINT

• Describe the course of the patient’s illness, including when and how it began,
character of symptoms, aggravating or alleviating factors, pertinent positives and
negatives, past diagnostic testing.
• Days of the week should not be written in history since they don’t indicate
duration of disease.
• Let the parent give the history in their own way and then ask specific questions.
• Ask how severe are the symptoms, have they changed during the past days, weeks
or months, has there been any change recently in the child’s appetite, energy or
activities, has the child been absent from school, has anyone who cares for the
child been ill, has the child been thriving or losing weight, what change in
behavior has there been, change in micturition or bowel habit.
• The history of presenting complaint expands on the presenting complaint and
focuses on the affected system i.e. if a child presents with diarrhea ask questions
concerning the GIT (for questions see Review of systems).

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REVIEW OF SYSTEMS
• This systematic enquiry runs from the “head to toes”. And these questions should
be asked in order to find out any other associated symptoms of the disease.
• Note: some questions can’t be asked to very young children as they can’t talk and
the mother cannot justify if at all the child has experienced the symptom e.g.
dizziness.
CENTRAL NERVOUS SYSTEM
• Loss of consciousness: sudden, warning, injuries, passage of urine, duration and
after effect. Precipitating factors and witnesses?
• Dizziness and vertigo
• Numbness
• Seizures, headaches and behavioral changes
• Visual disturbance: seeing double (diplopia), dimness, zigzag figures
(fortification spectra)
• Deafness or tinnitus: Discharge from ears, pain and hearing loss.
• Speech disturbance: duration, onset, nature, problems in reading or
understanding.
• Memory: independent opinion of relative or friend must be sought.
RESPIRATORY SYSTEM
• Cough: character, frequency, duration, causing pain and timing, productive
• Sputum: Quantity, color, nature (frothy, stringy and sticky), odor, timing (when
most profuse), hemoptysis (blood), is the blood red (frank) or brown (digested)?
Is it pure blood or ‘specks’?
• Dyspnea: on exertion or at rest. Expiratory difficulties, precipitating factors,
cough, fog, emotion, change in environment and wheezing.
• Pain in chest: location, character, affected by respiration, positions that increase
or decrease pain
• Hoarseness: with or without pain (involvement of recurrent laryngeal nerve)
• Throat: soreness, tonsillitis, ulcers, infection
• Nasal discharge or obstruction
• Epistaxis
• Night sweats
• Wheezing: asthma, chest infection, relieving factor, COPD

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CARDIOVASCULAR SYSTEM
• Dyspnea: on exertion (degree), at rest, time especially if wakes at night, position
(orthopnea), relieving factors, gradual or sudden onset, change, duration
(paroxysmal nocturnal dyspnea), precipitating factors, number of pillows used.
• Chest pain: site, on exertion or at rest, character, radiation, duration, relief by
drugs. Etc. accompanying sensations e.g. breathlessness, vomiting, cold sweat,
pallor, frequency, other relieving factors
• Palpitation
• Dizziness and faints
• Swelling of ankles: time of day
• Intermittent claudication: at rest or exertion
• Coldness of feet: Raynaud’s phenomenon
GASTROINTESTINAL
• Abdominal pain or discomfort: site, character (constant or colicky), radiation,
relationship to food and bowel actions.
• Nausea and vomiting: frequency and relationship to food, amount of vomitus,
contents, color, blood (hematemesis)
• Appetite and loss of weight
• Dysphagia: food hard or soft, fluids, level at which food “sticks”. Pain,
regurgitation. Progression from solid food to liquid.
• Diarrhea (frequency and looseness of motions) and constipation (recent or
longstanding and severity)
• Nature of stool: black (melena), clay/pale (obstructive jaundice), bulky and fatty
(steatorrhea), pile
• Tenesmus: painful sensation and urgent need to defecate (rectal pathology)
• Flatulence
GENITOURINARY
• Micturition: frequency, retention, dribbling and amount of urine passed,
incontinence.
• Urine: color, amount, smell, blood (hematuria), frothy.
• Dysuria and flank pain
• Menstruation-when applicable
➢ Age of onset (menarche)
➢ Periods:

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 o Last normal menstrual period (LMP)
o Regularity: regular or irregular
o Menstrual cycle: number of days or interval e.g. 4/28
o Amount of loss: Increase or decrease in flow
o Inter-menstrual discharge- character, blood or otherwise
➢ Vaginal discharge: quantity, color (normally clear), smell and irritation.
MUSCULOSKELETAL AND SKIN
• Joint swelling
• Joint stiffness
• Skin rash: type, duration, any treatment, painful and itching (psoriasis)

PAST MEDICAL HISTORY

• Medical problems, hospitalizations and admissions, history of similar illness,
operations, accidents or injuries.
• Diabetes, Epilepsy, Asthma, TB, HIV and Sickle cell should be enquired
appropriately (remember the mnemonic D.E.A.T.H.S).
• Any history of any pediatric deaths- enquire about the cause of death.
• Enquire about history of past blood transfusions

DRUG HISTORY
• Past and present medications- including name and dosage.
• Allergies.

BIRTH HISTORY
• Include: Prenatal, Perinatal and Postnatal history
• Prenatal history:
➢ Diagnosis of pregnancy- gravindex or scan
➢ Maternal obstetric problems, delivery, prolonged rupture of membranes,
maternal pyrexia.
➢ Visits to antenatal care (normally should have at least four visits and should
have receive, hematinics, Fansidar (malaria prophylaxis), if mother is exposed
and on septrin there is no need for fansidar.
➢ Gestational age at birth and length of gestation (preterm, term or post-term).
• Perinatal history:
➢ Method of delivery: Spontaneous vertex delivery (SVD), forceps, caesarean
➢ Birth weight

29
 ➢ Apgar scores
➢ Birth complications (e.g. infection, jaundice),
• Postnatal history:
➢ Length of hospital stay and method of feeding.

IMMUNIZATIONS
• Are they up to date? (check under 5 card)

NUTRITION
• Type of diet, amount taken each feed, change in feeding habits, breast feeding
(duration and weaning)

DEVELOPMENTAL AND GROWTH HISTORY

• Age at attainment of important milestones (Head support, sit, stand, walking,
talking, self-care, smile).
• Relationships with siblings, peers, adults.
• School grade and performance, behavioral problems.
• Monitoring growth curves (Look at under-five card).

FAMILY HISTORY
• Are parents alive?
• Siblings and their ages
• Medical problems in family including: Diabetes, Epilepsy, Asthma, TB,
Hypertension and Sickle cell disease (Remember D.E.A.T.H.S).

SOCIO-ECONOMIC HISTORY
• Family situation, Occupation of parents and parental level of education
• Relationship of parent
• Water source and toilet
• Alcohol, smoking.
• A history of recent travel abroad, particularly in tropical areas is important as the
child may have a disorder uncommon in his/her own country but having been
contracted in another country where disease may be endemic e.g. malaria.

SUMMARY
• Short and brief highlighting the important positives and negatives.

30
DIFFERENTIAL DIAGNOSIS
• Listed in order from most-likely to least likely in relation to the history.

CHAPTER 4: PHYSICAL EXAMINATION

PHYSICAL EXAMINATION
• Examination of the pediatric patient requires a careful and gentle approach.
• It should be carried out in an appropriate environment with a selection of books
or toys around which can be used to allay the apprehension and anxiety of the
child.
• The baby should be examined in a warm environment in good light.
• Nappies must be removed to examine the baby fully.
• The examiner must look first at the baby as a whole noting especially the color,
posture and movements and then proceed to a more detailed examination starting
at the head and working down to the feet.
• Infants and young children are often best examine on the mother’s lap where they
feel more secure.
• The examiner should ensure that his hands and instruments used to examine the
child are suitably warmed.
• It is not always mandatory to remove all the child’s clothes although it is often
essential in the examination of the acutely ill child.
• Procedures which may produce discomfort such as examination of the throat, ears
or rectal examination should be left until towards the end of the examination.
• Examination of the infant or child is often preceded by recording the patient’s
height, weight and head circumference on the growth chart. (this may have been
done by a nurse before the doctor sees the family)
➢ These measurements are plotted on graphs or charts, which indicate the
percentile or standard deviations at the various age throughout childhood.
➢ If the measurements are outwitting the 3rd to 97th centile for children for that
sex and age further study is indicated.
➢ Inquiry of parental height, weight or head size may also be important e.g.
familial macrocephaly or constitutional short stature.

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• The sequence of examination consists of:
➢ General examination
o General inspection-general condition, nutritional status, pallor, jaundice,
cyanosis
o Vital signs
o Anthropometric measurements- height, weight, and head circumference
➢ Skin
➢ Head
➢ Face
➢ Eyes
➢ Ears and nose
➢ Mouth and pharynx
➢ Neck
➢ Cardiovascular system
➢ Breasts
➢ Abdomen
➢ Genitalia and rectum
➢ Musculoskeletal system
➢ Nervous system
➢ Otoscopic examination of the ears

GENERAL EXAMINATION
GENERAL INSPECTION
• General condition of the child: Is the child unwell (stable), breathless or
distressed?
• Does the child look like the rest of the family?
➢ The face may be characteristic in Down’s syndrome and other chromosomal
disorders or in mucopolysaccharidoses.
• Level of consciousness: Alert, hyper-alert, lethargic, stupor or comatose?
• Is the child cyanosed, pale or jaundiced (in carotinaemia the sclerae are not
yellow)
➢ Infant should be pink with exception of the periphery, which may be slightly
blue.
➢ Congenital heart disease is only suggested if the baby has central cyanosis.

26
 o The best areas to look for central cyanosis are the tongue and buccal
mucosa not the limbs and the nails.
➢ A pale baby maybe anemic or ill and requires careful investigation to find the
cause.
➢ A blue baby may have either a cardiac anomaly or respiratory problem and
rarely methaemoglobinaemia.
➢ Normal physiological jaundice appears 2-3 days after birth of the infant
peaking about the 5th day and subsequently disappearing within one week.
Jaundice within the first 24 hours is pathological.
• Check for capillary refill time and skin turgor (skin pinch).
• Posture and movements
➢ A term baby lies supine for the first day or two and has vigorous, often
asymmetric movements of all limbs.
➢ In contrast a sick baby adopts the frog position with legs abducted, externally
rotated and is inactive.
➢ Older infants and children should be observed for abnormal movements,
posture and gait.
ANTHROPOMETRIC MEASUREMENTS
• Height, weight, head circumference (Occipito-frontal circumference: OFC) and
mid-upper arm circumference should be plotted on a percentile chart.
• For details of each refer to Chapter 2.
VITAL SIGNS
• Parameters measured are
➢ Pulse: Palpate the femoral artery or brachial artery (in cubital fossa) or
auscultate the heart.
➢ Respiratory rate: Normal: 30- 60 breaths per minute
➢ Blood pressure: measured routinely in children above the age of 2.
➢ Temperature: Rectal preferred.
o Average rectal temperature is higher in infancy than in adulthood usually
not falling below 37.2 degrees Celsius until after 3 years.

SKIN
• Examine for texture and appearance.
➢ In infants it is soft and smooth because it is thinner than in older children.
• The presence of any skin rash, its color and whether there are present macules,
papules, vesicles, bullae, petechiae or pustules should be recorded.

27
 ➢ The skin texture, elasticity, tone and subcutaneous thickness should be
assessed by picking up the skin between the fingers.
➢ Pigmented naevi, strawberry naevi, haemangiomata or lymphangiomata may
be present and may vary in size and number.
➢ They may be absent or small at birth and grow in subsequent days or weeks.

Figure 4.1: Dermatological terminology (Adapted from Hutchinson's Paediatrics)

• Palpate the skin and roll it to check for skin turgor (dehydration)

HEAD
• The head should be inspected for size, shape and symmetry.
• Measure the head circumference (occipitofrontal circumference) with a non-
elastic tape by placing it to encircle the head just above the eyebrows around
maximum protuberance of the occipital bone should be performed and charted.
➢ An abnormally “large head” (more than 97th centile or 2 SD above the mean)
is due to macrocephaly, which may be due to hydrocephalus, subdural
hematoma or inherited syndromes.
➢ Familial macrocephaly (autosomal dominant) is a benign familial condition
with normal brain growth.
• In the infant the skull should be palpated to determine the size and tension in the
fontanelles an assess the skull sutures.

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Figure 4.2: Top of head anterior fontanelle and cranial sutures (Adapted from Hutchinson's Pediatrics)

• Premature fusion of sutures suggests craniostenosis.

• In neonates the posterior fontanelle may be very small and subsequently closes
by 3 months but the anterior fontanelle is larger, only closing at around 18 months
(1 year 8 months).
• A tense and bulging fontanelle suggests raised intracranial pressure and a deeply
sunken one suggests dehydration.
• Large fontanelles, separation of sutures, delayed closure of the fontanelles may
be associated with raised intracranial pressure, or other systemic disorders such
as hypothyroidism and rickets.
• Hair: alopecia, seborrhea of the scalp.

FACE
• Abnormalities of facial development are usually obvious and an example is the
infant with cleft lip.
• Associated with this there may be a cleft palate, but full visual examination of
the palate including the uvula is necessary to ensure that the palate is intact and
there is not a sub-mucous and soft palate clefts cannot be felt on palpation.

EYES
• Inspect for:
➢ Subconjuctival hemorrhages
➢ Cataracts
➢ Papilloedema
➢ Congenital abnormalities e.g. colobomata.

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• ‘Rocking’ the baby from the supine to vertical position often results in the eyes
opening so they can be inspected.
• Squint is a condition in which early diagnosis and treatment is important.

EARS AND NOSE

MOUTH AND PHARYNX
• Any discharges: basal skull fracture (CSF)
• Nasal congestion
• Any foreign objects
• Check for tonsils, teething, oral thrush

NECK AND THROAT

• Short, webbed (Turner syndrome), torticollis
• Thyroid: enlarged, bruit
• Swellings
• Midline: thyroglossal cyst, goiter
• Lateral: lymph nodes, branchial cyst, cystic hygroma, sternomastoid tumor.

EXAMINATION OF THE RESPIRATORY SYSTEM

INSPECTION
• Scars of past surgery (look at the front and the back of the chest).
• Shape: normal and symmetrical, pectus carinatum (undue prominence of the
sternum-pigeon chest), Pectus excavatum (funnel chest), Harrison’s sulci,
hyperinflation (increased anteroposterior diameter).
• Use of accessory muscles of respiration.
• Flaring of the nares.
• Intercostal recession, any stridor, audible wheeze or grunt.
• Count the respiratory rate.
PALPATION
• Chest wall movements: is it symmetrical?
• Feel the trachea: central or deviated
• Palpate for any fractured ribs or clavicle.
• Tactile vocal fremitus (over 5 years of age- ask the child to say 99).

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PERCUSSION
• Percuss all areas: normal, resonant, hyper-resonant, dull (collapse,
consolidation), stony dull (pleural effusion)
• Do not percuss in young children.
AUSCULTATION
• Air entry, vesicular (normal), absent breath sounds (pleural effusion) and
bronchial (consolidation)
• Added sounds: Rhonchi, stridor, inspiratory or expiratory crackles (fine versus
coarse), pleural friction rub
• Vocal resonance.

EXAMINATION OF THE CARDIOVASCULAR SYSTEM

• The examination of the CVS of infants and children is carried out in a similar
manner to that of adults.
• The examiner should always feel for femoral pulses and ascertain whether there
is any radiofemoral or brachiofemoral delay as this would suggest the possibility
of coarctation of the aorta.
• The most important factor in recording the blood pressure of children is to use a
cuff of the correct size.
• The cuff should cover at least two-thirds of the upper arm.
➢ If the cuff size is less than this a falsely high blood pressure reading may be
obtained.
➢ In small infants relatively accurate systolic and diastolic pressures as well as
mean arterial pressure can be obtained by use of the Doppler method.
• The apex should be visible and palpable and the position noted.
• The precordial areas should be palpated for the presence of thrills.
• If the apex beat is not obvious look for it on the right side of the chest as there
could be dextrocardia or a left-sided congenital diaphragmatic hernia with the
heart pushed to the right or collapse of the right lung.
• All areas should be auscultated while the baby is quiet systolic murmurs may be
very harsh and can be confused with breath sounds.
INSPECTION
• Are there features of Down’s (Atria septal defect-ASD, Ventricular septal defect-
VSD), Turner’s (coarctation of the aorta) or Marfan’s (aortic incompetence).
• Cyanosis: peripheral and central

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• Hands: clubbing (Cystic fibrosis) and splinter hemorrhages (endocarditis)
• Edema: precordium, ankles and sacrum
• Precordium for scars of past surgery.
PALPATION
• Pulses: Radial/brachial/femoral- radiofemoral delay, rate.
• Character of pulse- collapsing, volume
• Heart rate- rhythm
• Apex beat- position (normal position in children 4th-5th left intercostal space in
the mid-clavicular line), beware of dextrocardia.
• Palpate for a parasternal heave and for precordial thrills.
• Note: Percussion of the heart is not normally undertaken in children.
AUSCULTATION
• Listen to all 4 valve area (apex, lower L sternal edge, upper L sternal edge and
upper R sternal edge)
• Quality of heart sounds.
• Additional sounds i.e. clicks, murmur (timing of the murmur).
• Blood pressure-use a cuff that covers at least 2/3 of the upper arm or use Doppler.

EXAMINATION OF THE ABDOMEN

• In the infant the abdomen and umbilicus are inspected and attention should be
paid to the presence of either a scaphoid abdomen (in neonates may indicate
diaphragmatic hernia or duodenal atresia) or distended abdomen which suggests
intestinal obstruction especially if visible peristalsis can be seen.
➢ Peristalsis from left to right suggests a high intestinal obstruction whereas one
from the right to left would be more in keeping with low intestinal obstruction.
• Any asymmetry of the abdomen may indicate the presence of an underlying mass.
• Abdominal movement should be assessed and abdominal palpation should be
performed with warm hands.
➢ The edge of the liver can normally be felt in the new born baby
➢ The spleen can only be felt if it is pathological
➢ The kidneys can be felt in the first 24 hours with the fingers and thumb
palpating in the renal angle and abdomen on each side.

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• The lower abdomen should be palpated for the bladder and an enlargement can
be confirmed by percussion from a resonant zone, progressing to a dull zone.
• In a baby with abdominal distention where there is suspicion of perforation and
free gas in the abdomen the loss of superficial liver dullness on percussion may
be the only physical sign present early on.
• Areas of tenderness can be elicited by watching the baby’s reaction to gentle
palpation of the abdomen.
• There may be areas of erythema, cellulitis and edema of the abdominal wall and
on deeper palpation crepitus can occasionally be felt from pneumatosis
intestinalis (intramural gas in the wall of the bowel).
• Auscultation of the abdomen in the younger patients gives rather different signs
than in the adults. The infant even in the presence of peritonitis may have some
bowel sounds present.
• However, in the presence of ileus or peritonitis breath sounds become conducted
down over the abdomen to the suprapubic area and in even more severe disorders
the heart sounds similarly can be heard extending down over the abdomen to the
suprapubic area.
INSPECTION
• General distention.
• Superficial veins- direction of flow, striae, umbilicus.
• Masses, scars, visible peristalsis.
PALPATION AND PERCUSION
• First lightly palpate the entire abdomen, keep looking at the child’s face all the
time.
• Localized tenderness, rebound tenderness and rigidity
• Masses
• Ascites-percuss for the shifting dullness
• Spleen, liver and kidneys
• Hernial orifices
• Genitalia (testes) and anus (site)
AUSCULTATION
• Bowel sounds: absence implies ileus

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EXAMINATION OF GENITALIA AND RECTUM
•

EXAMINATION OF THE NERVOUS SYSTEM

• The neurological examination of the young infant and child is different from that
routinely carried out in the adult.
• Muscle tone and strength are important parts of the examination.
• In infants muscle tone may be influenced by child’s state of relaxation.
• Assess child’s developmental levels relating to gross motor, finer motor, vision,
hearing, speech and social skills.
• All older children should be observed for gait to detect abnormal coordination
and balance.
• Older children may be tested for sense.

STOOL AND URINE EXAMINATION

• Examination of a stool which preferably fresh is often informative.
• The color, consistency and smell are noted as well as the presence of blood or
mucus.
• Urine examination is also important in children since symptoms related to the
urinary tract may be non-specicific (Urinalysis).
• After a complete examination:
➢ Document findings and summarize.
➢ Provide a diagnosis with differentials.
➢ List appropriate investigations along with management plan.

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 CHAPTER 5: IMMUNIZATION AND VACCINATION

IMMUNIZATON, VACCINATION AND

IMMUNIZABLE DISEASES

IMMUNIZATION AND VACCINATION

• Immunization and vaccination are the most important components of well child
care and are the cornerstones of pediatric preventive care.
TYPES OF IMMUNIZATIONS
• These include:
➢ Active immunization: with live vaccines and non-live vaccines.
➢ Passive immunization
ACTIVE IMMUNIZATION
• Involves induction of long-term immunity through exposure to live attenuated or
killed (inactivated) infectious agents.
• Live vaccines induce long-lasting immunity but carry the risk of vaccine-
associated disease in the recipient or secondary host.
➢ These should be avoided in patients with compromised immunity e.g. cancer,
congenital or drug induced immunodeficiency.
➢ Examples: Oral polio (OPV), varicella and Mumps, Measles and Rubella
(MMR) vaccines.
• Non-live vaccines are not infectious and tend to induce immunity for shorter
periods, thus requiring booster immunizations.
➢ Examples: diphtheria, tetanus and acellular pertussis (DTaP), hepatitis A and
B, inactivated polio (IPV), Haemophilus influenzae type b (HIB), influenza,
pneumococcal and meningococcal vaccines.
PASSIVE IMMUNIZATION
• Involves delivery of preformed antibodies to individuals who have no active
immunity against a particular disease but who have either been exposed to or are
at high risk for exposure to the infectious agent.
• Examples:

34
 ➢ Varicella zoster immune globulin (VZIG) for immunocompromised patients
who have been exposed to varicella and are at high risk for severe varicella
infection.
➢ Newborns born to hepatitis B-positive mothers receive hepatitis B immune
globulin at birth
➢ Visitors to high risk areas may receive hepatitis A Ig before travel.
SPECIFIC IMMUNIZATIONS
BCG
• Rationale for Vaccine: protection against severe forms of TB e.g. TB meningitis
• Timing of vaccination (in Zambia):
➢ At birth
➢ If no scar repeat dose at 12 weeks (3 months) unless in symptomatic HIV
• It is given intradermally usually on the left upper arm
• It is not given to immunosuppressed people and individuals with active disease.
ORAL AND INACTIVATED POLIO VACCINES (OPV/IPV)
• Rational for vaccine: Poliovirus is an enterovirus with propensity for the CNS
causing transient or permanent paresis of the extremities and
meningoencephalitis.
• There are 2 types of vaccines
➢ Live attenuated (OPV-SABIN), administered orally
o Advantages: induction of both host immunity and secondary immunity
because it is excreted in the stool of the recipient and may infect and thus
immunize, close contacts (i.e. herd immunity)
o Disadvantages: possibility of vaccine related polio in host as well as
unimmunized contacts.
➢ Non-live or inactivated (IPV-SALK), administered subcutaneously or
intramuscularly, has the advantage of no vaccine-related polio but the
disadvantage of not inducing secondary immunity.
• Timing of vaccination (in Zambia):
➢ At birth to 13 days: OPV0
➢ At 6 weeks: OPV 1
➢ After at least 4 weeks of OPV1: OPV2
➢ After at least 4 weeks of OPV 2: OPV3
➢ OPV 4 is given at 9 months only if OPV 0 was not given

34
PNEUMOCOCCAL VACCINES (PCV)
• Rational for vaccine: Pneumococcus (Streptococcus pneumoniae) is the most
common cause of acute otitis media and invasive bacterial infections in children
younger than 3 years of age.
• There are 2 types of vaccines:
➢ Pneumovax: composed of polysaccharide capsular antigens from 23
pneumococcal serotypes.
o Advantages: vaccine contains antigens from pneumococcal strains causing
almost all cases of bacteremia and meningitis during childhood.
o Disadvantages: little immunogenicity in children younger than 2 years.
o Indications: older children and adults at high risk for pneumococcal
disease (e.g. patients with sickle cell anemia who are functionally asplenic,
immunodeficiency, chronic liver disease and nephrotic syndrome, and
patients with anatomic asplenia)
➢ Prevnar: composed of 7 pneumococcal serotypes
o Advantages: immunogenicity and efficacy in preventing meningitis,
pneumonia, bacteremia and otitis media from the most common
pneumococcal strains in children younger than 2 years of age.
o Disadvantages: does not confer as broad coverage against pneumococcal
strains as Pneumovax.
o Indication: all children younger than 2 years of age and selected children
older than 2 years of age who are at high risk for pneumococcal disease.
• Timing of vaccination (in Zambia):
➢ At 6 weeks: PCV 1
➢ After 4 weeks of PCV 1: PCV 2
➢ After 4 weeks of PCV 2: PCV 3
• The preferred site for infants and young children is intramuscular in the vastus
lateralis muscle in the anterolateral thigh. The preferred injection site in older
children and adults is the deltoid muscle.
DIPTHERIA, PERTUSSIS, TETANUS, HEPATITIS B AND
HEMOPHILUS INFLUENZAE TYPE B (DPT-HepB-HiB)
• Rationale for vaccine:
➢ Diphtheria, tetanus and pertussis all may cause serious disease, especially in
young infants.
➢ Hepatitis B infects 300 million worldwide.

35
 ➢ H. influenza type B was a serious cause of invasive bacterial infection,
including meningitis, epiglottitis and sepsis before vaccine licensure in 1985.
Since licensure it has become a rare cause of such infection.
• Vaccine is inactivated (injected).
• The preferred site for infants and young children is intramuscular in the vastus
lateralis muscle in the anterolateral thigh. The preferred injection site in older
children and adults is the deltoid muscle.
• Timing of vaccination (in Zambia):
➢ At 6 weeks: DPT-HepB-HiB 1
➢ After 4 weeks of DPT-HepB-HiB 1: DPT-HepB-HiB 2
➢ After 4 weeks of DPT-HepB-HiB 2: DPT-HepB-HiB 3
MEASLES, MUMPS AND RUBELLA
• Rational for vaccine: immunizes against 3 viral diseases:
➢ Measles: a severe illness with complications that include pneumonia
associated with significant mortality.
➢ Mumps: most commonly associate with parotitis but may also cause
meningoencephalitis and orchitis
➢ Rubella: causes a mild viral syndrome in children but may cause severe birth
defects in offspring of susceptible women infected during pregnancy
• Type of vaccine: live attenuated vaccine
• Timing of vaccination (in Zambia):
➢ At 9 months or soon after unless if symptomatic HIV
➢ At 18 months or soon after unless if symptomatic HIV
ROTA VACCINE
• Rationale for vaccine: protection against viral diarrhea causes by Rota virus.
• Timing of vaccination (in Zambia):
➢ At 6 weeks: ROTA 1
➢ After 4 weeks of ROTA 1: ROTA 2

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ZAMBIA IMMUNIZATION SCHEDULE/RECORD
TABLE 5.1: IMMUNIZATION RECORD (ZAMBIA)

IMMUNISATION against Tuberculosis (TB)

BCG (at birth) Date ………………..
If no scar after 12 weeks
Repeat dose Unless symptomatic HIV Date .……………….
IMMUNISATION against Polio (OPV), Diphtheria, Whooping Cough,
Tetanus, Hib, Hepatitis B, Meningitis, Pneumonia, (DPT-HepB-Hib),
Measles, Diarrhea (Rota), & Streptococcal Pneumonia (PCV)
OPV 0 (at birth to 13 days) Date ………………….
OPV 1 (at 6 weeks) DPT-HepB-HiB 1 (at 6 weeks)
Date ………………….. Date …………………..
OPV 2 DPT-HepB-HiB 2
(at least 4 weeks after OPV1) (at least 4 weeks after DPT-HepB-HiB
Date ………………….. 1)
Date …………………..
OPV 3 and IPV DPT-HepB-HiB 3
(at least 4 weeks after OPV2) (at least 4 weeks after DPT-HepB-HiB
Date ………………….. 2)
Date …………………..
OPV 4 Measles
(at 9 months, only if OPV 0 Rubella
was not given) (at 9 months or soon after unless
Date ………………….. symptomatic HIV)
Date …………………..
PCV 1 Measles
(at 6 weeks) Rubella (at 18 months or soon after
Date ………………….. unless symptomatic HIV)
Date …………………..
PCV 2 ROTA VACCINE 1 (at 6 weeks)
(at least 4 weeks after PCV 1) Date …………………..
Date …………………..
PCV 3 ROTA VACCINE 2
(at least 4 weeks after PCV 2) (at least 4 weeks after ROTA 1)
Date ………………….. Date …………………..

37
ADVERSE EFFECTS OF IMMUNIZATION
• Most vaccine side effects are mild to moderate in severity and occur within the
first 24 hours after administration e.g. local inflammation and low grade fever.
• Because MMR and varicella vaccines are live attenuated vaccines fever and rash
may occur 1-2 weeks after immunization (i.e. after the incubation period of the
virus)
• Serious side effects that may result in permanent disability or be life-threatening
are rare (e.g. vaccine related polio after OPV)
CONTRAINDICATIONS AND PRECAUTIONS TO
IMMUNIZATION
CONTRAINDICATIONS
1. Anaphylaxis to a vaccine or its constituents
2. Encephalopathy within 7 days after DTaP vaccine
3. Patients with progressive neurologic disorders including uncontrolled epilepsy
should not receive the DTaP vaccine until neurologic status is stabilized.
4. Immunodeficient patients should not receive OPV, MMR and Varicella vaccine.
➢ Household contacts of immunodeficient patients should not receive OPV as it
is shed in stool
5. Pregnant patients should not receive live vaccines.
PRECAUTIONS
• For all vaccines, moderate to severe illness (with or without fever) are not
contraindication to immunization.
• DTaP vaccine:
➢ Temperature of 40.5OC within 48hours after prior vaccination
➢ Collapse or shock-like state within 48hours after prior vaccination
➢ Seizures within 3 days after prior vaccination
➢ Persistent, inconsolable crying lasting more than 3 hours occurring within 48
hours after prior vaccination
• MMR and Varicella vaccines
➢ Immunoglobulin (IVIG) administration within the preceding 3-11 months
which might interfere with the patient’s immune response to these vaccines.

34
TETANUS
• Tetanus/lockjaw is caused by the bacterium Clostridium tetani a spore forming,
anerobic, gram-positive motile bacillus found in human and animal feces.
• It is characterized by spasms.
• Endospores are found widespread in the environment.
• Tetanus is common in areas where soil is cultivated, in rural areas, in warm
climates and during summer months.
• Despite adequate supportive care, mortality rate may be as high as 50% in severe
generalized tetanus and 90% in neonatal form. The outcome depends on the
incubation period, site of injury, rate of progression of illness and presence of
autonomic instability.
• Survivors do not manifest any neurological sequelae expect when apneic
episodes are unduly prolonged and unattended.
• The bacteria generally enter through a break in the skin such as a cut or puncture
wound by a contaminated object. They produce toxins that interfere with normal
muscle contractions.
• Given that C. tetani is an anaerobic bacterium, it and its endospores thrive in
environments that lack oxygen such as a puncture wound.
• Note: the disease does not spread between people.
➢ Rarely tetanus can be contracted through surgical procedures, IM injections,
compound fractures and dental infection.
➢ Animal bites can transmit tetanus.
➢ Tetanus is often associated with rust, especially rusty nails. Although rust
itself does not case tetanus, objects that accumulate rust are often found
outdoors or in places that harbor anaerobic bacteria. Additionally, the rough
surface of rusty metals provides crevices for dirt containing C. tetani while a
nail affords a means to puncture skin and deliver endospores deep within the
body at the site of the wound. NOTE: RUST DOES NOT CAUSE TETANUS
AND A PUNCTURE FROM A RUST-FREE NAIL DOES NOT ELIMIATE
THE RISK OF CONTRACTING TETANUS.
PATHOGENESIS
• C. tetani is a non-invasive organism, the spores of the organism remain non-
pathogenic in soil or contaminated tissues till conditions are favorable for
transformation into vegetative form.

35
• Transformation occurs in the presence of locally decreased oxygen reduction
potential typically in devitalized tissue, in the presence of a foreign body, trauma
and crush injury and suppurative infections.
• 2 types of toxins are produced by the organism:
➢ Tetanospasmin: this is the main toxin responsible for manifestation of the
disease. It binds to the neuromuscular junction at the site of injury, and
undergoes retrograde axonal transport to reach the presynaptic nerve terminal
where it prevents the release of the inhibitory neurotransmitters glycine and
GABA leading to uncontrolled contraction of muscle.
➢ Tetanolysin
CLINICAL FEATURES
• Mainly affects unimmunized and partly immunized individuals.
• Disease may occur in various forms:
➢ Neonatal tetanus (Trismus nascentium)
➢ Generalized tetanus
➢ Localized tetanus
➢ Cephalic tetanus
• Generalized and neonatal tetanus are the most common forms.
GENERALIZED TETANUS
• Most common type (80%)
• Incubation period= 8 to 10 days (range 2-14 days), disease can also occur months
after initial injury. Incubation period depends on the distance of the site of injury
from the CNS.
• Faster onset of symptoms= poorer prognosis.
• Features:
➢ Descending paralysis with initial involving of the jaw muscles, facial spasms,
followed by stiffness of the neck, difficulty in swallowing and rigidity of
pectoral and calf muscles
o Spasms of the masseters leading to trismus or lock jaw.
o Spasms can affect facial muscles resulting in an appearance called Risus
sardonicus/rictus grin (sustained, abnormal spasms of the facial muscles
that appears to produce grinning).
▪ DDx of Risus sardonicus: Tetanus, Wilson’s disease, Strychnine
poisoning and after judicial hanging.

36
 ▪ The condition’s name, associated with the Mediterranean island of
Sardinia, derives from the appearance of raised eyebrows and an open
“grin”- which can appear sardonic or malevolent (with evil intent) to
the lay observer
o Subsequent involvement of Chest, neck, back, abdominal muscles and
buttocks occurs, soon involving the whole body.
o Back spasms often cause arching called opisthotonos.
o Spasms las for several minutes and continue for up to 4 weeks and
complete recovery may take months.
➢ Generalized spasm with minimal stimuli as disease progress (hallmark of
disease and contribution to serious complications and death). Prolonged
muscle contractions become painful and powerful (tetany) and these episodes
can cause fractures and muscles tears.
➢ Sensorium is preserved
➢ Other symptoms:
o Fever
o Headache
o Restlessness
o Irritability and feeding difficulties
o Dysphagia
o Burning sensation during urination
o Urinary retention
o Loss of stool control
o Autonomic instability with blood pressure fluctuations in the form of
hypertension or hypertension, episodic rapid heart rate, diaphoresis and
arrhythmias
• Recovery usually beings after 3 weeks and approximately takes 4 weeks.
• Recovery from tetanus occurs by sprouting new nerve terminal in the spinal cord
leading to relaxation of the contracted muscles.
NEONATAL TETANUS/ TRISMUS NASCENTIUM
• Pregnant women not immunized against tetanus do not pass on protective
antibodies to their children.
• Infection results from unhygienic birth practices most commonly when the
umbilical cord is contaminated at the time of cutting after delivery.
• Symptoms appear by day 3 after birth, never in the first 2 days of life and rarely
after age of 2 weeks.

37
• Features:
➢ Excessive unexplained crying followed by refusal of feeds and apathy
➢ Progressive feeding difficulty is later followed by rigidity, paralysis and the
child may develop opisthotonic posturing and experience painful spasms, the
mouth is kept slightly open due to pull and spasm of the neck. Reflex spasm
of the masseter makes feeding painful. Pharyngeal muscles go into spasm and
cause dysphagia and choking, lockjaw, or reflex trismus followed by spasms
of limbs
➢ Generalized rigidity and opisthotonos in extension
➢ Spasm of larynx and respiratory muscles induced by stimuli such as touch,
noise, bright light, resulting in episodes of apnea and cyanosis.
➢ Constipation persists until the spasms are relieved.
• Intercurrent infections, dehydration and acidosis may complicate the clinical
picture.
• Fatality rate=70 to 100%
• Prognosis is worse if:
➢ Onset of symptoms occurs within the first week of life
➢ Interval between lockjaw and onset of spasms is less than 48 hours
➢ High fever and tachycardia are present
➢ Spasms, especially of larynx resulting in apnea and severe and frequent
LOCALIZED TETANUS
• Less severe
• Characterized by rigidity and pain confined to the muscle adjacent to the wound.
The contraction of the muscle may persist for many weeks before gradually
subsiding however, it may lead to generalized tetanus later.
• In patients with isolated localized tetanus, the mortality is less than 1%.
CEPHALIC TETANUS
• Rarest form of disease (0.9-3%) and is limited to muscles and nerves in the head.
• It usually occurs after trauma to the head area, including skull fracture, laceration,
eye injury, dental extraction, and otitis media but it has been observed from
injuries to other parts of the body.
• Paralysis of the facial nerve is most frequently implicated, which may cause
lockjaw, facial palsy or ptosis but other cranial nerves can be also be affected.

38
• Cephalic tetanus may progress to a more generalized form of the disease. Due to
its rarity, clinicians may be unfamiliar with the clinical presentation and may not
suspect tetanus as the illness.
• Treatment can be complicated as symptoms may be concurrent with the initial
injury that caused the infection.
• It has a poor prognosis.
DIAGNOSIS
• Clinical diagnosis. No blood tests are available for diagnosis of tetanus.
• The “spatula test” is a clinical test for tetanus that involves touching the posterior
pharyngeal wall with a soft tipped instrument and observing the effect.
➢ Positive: involuntary contraction of the jaw (biting down on the “spatula”)
➢ Negative: gag reflex attempting to expel the foreign object.
MANAGEMENT
• Admit to intensive care unit
• Reduce stimulation by avoiding noxious stimuli including bright lights, pain and
loud noises.
➢ Keep patient in a dark, quiet and isolated room, which should be lighted well
to permit observation of the child,
➢ HANDLING SHOULD BE MINIMUM
➢ Avoid IM injection
• Airway management:
➢ Tracheotomy (recommended as intubation may cause spasms) or Intubation
and mechanical ventilation for 3 to 4 weeks especially in severe cases and if
the infant gets frequent episodes of laryngeal spasms, apneic attacks with
cyanosis or central respiratory failure.
➢ Periodic oropharyngeal secretions.
• Breathing: maintenance of oxygen
• Circulation: set up an IV line to provide adequate fluids, calories and electrolytes
and for administration of medication
• Neutralize free toxin by administering human tetanus immunoglobulin however,
antitoxin cannot dislodge the toxin already fixed to the nerve root. Route of
administration is IM or intrathecal.
➢ Dose: 500 to 1000 IU
• Antibiotics to abolish bacteria from wound. Crystalline penicillin or
metronidazole are commonly used for 10 days.

39
• Relief spasms using benzodiazepines e.g. diazepam either as an intermittent IV
bolus or as continuous infusion.
➢ Diazepam prevents further spasms by causing GABA-mediated central
inhibition. It also helps by reducing anxiety and promoting muscle relaxation.
➢ Other agents used for severe spasms: Pancuronium bromide.
• Supportive:
➢ Daily wound clean and removal of dead and devitalized tissue
➢ Temperature control
➢ Hydration, early detection of myoglobinuria and prevention of renal shutdown
➢ Stop oral feeds after 3 to 4 days of treatment, milk feeding through NG tube
may be started. High caloric protein diet should be instituted often given in
liquid form through a tube directly into the stomach (percutaneous endoscopic
gastrotomy) or through parenteral nutrition.
➢ Control autonomic instability with alpha- and beta-adrenergic blockers e.g.
propranolol and labetalol. IV magnesium sulfate is effective in decreasing
autonomic instability and treating muscle spasms.
• NOTE: all patients should receive a complete course of immunization with
tetanus toxoid once recovered as the disease does not induce protective
antibodies.
PREVENTION
• Recovery does not usually result in immunity to tetanus. This is due to extreme
potency of the tetanospasmin toxin. Tetanospasmin will likely be lethal before it
will provoke an immune response.
• Immunization with tetanus toxoid IM leads to induction of protective antibodies.
• Adults should get a booster vaccine every 10 years and every person with a
puncture wound uncertain when they were last vaccinated or has had fewer than
3 lifetime doses of the vaccine should receive the booster.
• The booster may not prevent a potentially fatal case of tetanus from the current
wound, however, as it can take up to 2 weeks for tetanus antibodies to form.
• Maternal and neonatal tetanus can be effectively prevented by immunizing the
mother during pregnancy and ensuring clean delivery and cord care.
➢ Children under seven are given DPT/DTaP (diphtheria pertussis and tetanus
or diphtheria, tetanus and acellular pertussis)
➢ Adults and children above seven are given Td vaccine (tetanus and diphtheria)
or Tdap (tetanus, diphtheria, and acellular pertussis)

40
POLIO

CHAPTER 6: PEDIATRIC GENETIC DISORDERS

PEDIATRIC GENETIC DISORDERS

DOWN SYNDROME and exist through female
reproductive life.
(TRISOMY 21) • The sperm has a short lifespan and
• This is the most common therefore has less chances of
autosomal trisomy and the most injurious exposure.
common genetic cause of severe
learning difficulties. CYTOGENETICS
• The incidence (without antenatal • The extra-chromosome 21 may
screening) in live-born infants is result from
about 1 in 1000. ➢ Meiotic non-disjunction (94%)
• The syndrome was named after ➢ Translocation (5%)
Langdon Down, who first coined ➢ Mosaicism (1%)
the term monoglism because of the • Karyotype of the parent is only
mongoloid facial appearance of required if the affected child has
patients with the syndrome. translocation underlying Down
Nowadays the term mongolism is syndrome.
obsolete.
MEIOTIC NON-DISJUNCTION
• Chromosome 21 is present in
• Accounts for about 94% of the
triplicate, the origin of the extra
cases.
chromosome being either paternal
• Most cases result from an error at
or maternal.
meiosis.
• Down’s syndrome occurs more
• The pair of chromosome 21s fails to
commonly in mothers conceiving at
separate, so that one gamete has 2
older age. This is attributed to the
chromosome 21s and one has none.
exposure of the maternal oocyte to
harmful environmental influences • Fertilization of the gamete with two
for a longer period since Graafian chromosome 21s gives rise to a
follicles are present in the fetal life zygote with trisomy 21.

41
• Parental chromosomes do not need
to be examined.
• The incidence of trisomy 21 due to
non-disjunction is related to
maternal age.
• Meiotic non-disjunction can occur
in spermatogenesis so that the extra
21 can be of paternal origin.
TRANSLOCATION
• Accounts for 5% of all the cases.
• Occurs when the extra chromosome
21 is jointed onto another
chromosome (usually chromosome
14 –Robertsonian translocation, but
occasionally chromosome 15, 22 or
21).
• This may be present in a
phenotypically normal carrier with
45 chromosomes (2 being ‘joined
together’) or in someone with
Down syndrome and a set of 46
34
chromosomes but 3 copies of
chromosome 21 material.
• Parental chromosomal analysis is
recommended since one of the
parents may well carry the
translocation in balanced form
(25% of cases)- rearranged genetic
material (in mother or father) but no
extra-chromosomes.
➢ Balanced carriers have no signs
or symptoms of Down
syndrome but can pass the
translocation to the children.
• In translocation Down syndrome:
➢ Risk of recurrence is 10-15% if
the mother is the translocation
carrier and about 2.5% if the
father is the carrier.
➢ If a parent carries the rare 21:21
translocation all the offspring
will have down syndrome.
 ➢ If neither parents carry a o Upslanting palpebral fissures
translocation (75% of cases) the (Mongolian slant to eyes)
risk of recurrence is <1%. o Brushfield spots in iris
(speckled irides)
MOSAICISM o Flat nasal bridge
• Accounts for 1%. o Folded or dysplastic ears
• Some cells are normal and some o Open mouth (macroglossia-
have trisomy 21. protruding tongue,
• This usually arises after the microstonia- small mouth)
formation of chromosomally o Short neck
normal zygote by non-disjunction o Excessive skin at the nape of
at mitosis but can arise by later the neck
mitotic disjunction in a trisomy 21 o Characteristic grimace when
conception. crying
• The phenotype is sometimes milder ➢ Extremities:
in Down syndrome mosaicism. o Hypotonia
RISK FACTORS o Short broad hands
o Single palmar creases
1. Advancing age
(simian crease)
o 35 years 1 in 350
o Short, Incurved fifth finger
o 40 years 1 in 100
due to Clinodactlyly
o 45 years 1 in 30
(hypoplasia of middle
2. Having had a child with down th
phalanx of 5 finger)
syndrome (translocation)
o Wide ‘sandal’ gap between
3. Carriers of genetic translocation for
the big and second toe.
down syndrome
o Hyperflexible joints
CLINICAL FEATURES ➢ Other: duodenal atresia,
• Most affected infants are hypotonic congenital heart defects (40%)
• Clinical signs include: and Hirschsprung disease
➢ Craniofacial features: ➢ Neonatal features:
o Brachycephaly (skull is o Flat facial profile
shorter than usual) o Round face
o Flat occiput and third o Fine soft and sparse hair
fontanelle o Small dysplastic ears that are
o Epicanthal folds (a fold of low set
skin running across the inner o Poor Moro reflex
edge of the palpebral fissure)

35
 o Excessive skin at the nape of o It ranges from mild to
the neck moderate
o Slanted palpebral fissures o Starts in first year of life
o Hypotonia o Average age:
o Hyperflexibility of joints ▪ Sitting: 11 months
o Dysplasia of pelvis ▪ Walking 26 months
o Anomalous ears (twice normal)
➢ 6 or more of the above features o IQ declines in first 10 years,
should be preset for making a reaches a plateau at
diagnosis clinically. adolescence and continues
➢ Mental retardation through out adulthood.
o Almost all have mental
retardation

36
DIAGNOSIS
• Diagnosis can be difficult to make
when relying on clinical signs alone
and a suspected diagnosis should be
confirmed by a senior
paediatrician.
➢ History (usually from a third
party):
o Determine patient’s age and
obtain presenting complaint
o In History of presenting
complaint include parental
age, previous eyeglass
prescription, occlusion
therapy, onset of strabismus
and/or nystagmus, previous
external infections and
treatment modalities, tearing
and photophobia.
o Review pulmonary, CVS
(including any previous
cardiovascular surgery),
gastrointestinal and
neurological systems.
o Ask for history of any other
siblings with down
syndrome.
➢ Examination: 8 or more
characteristic clinical findings
lead to a definite diagnosis. In
doubtful cases, chromosomal
analysis may be necessary.
• Before blood is sent for analysis,
parents should be informed that a
test for Down syndrome is being
performed.
34
• The results may take 1-2 days using
rapid FISH (fluorescent in situ
hydridisation) techniques.
• Diagnosis is confirmed by
karyotype.
• Parents need information about the
short and long term implications of
the diagnosis.
• It is difficult to give a precise long
term prognosis in the neonatal
period, as there is individual
variation in the degree of learning
difficulty and the development of
complications.
• Important steps after suspecting
down syndrome in a neonate:
➢ Establish diagnosis
➢ Thorough clinical examination
to observe for congenital heart
disease or gastrointestinal
malformation
➢ Inform the parents in a
supportive way
➢ Laboratory orders:
o Chromosomal karyotype
o Thyroxine
o TSH
o Full blood count with
differential
o Platelet count
o Echocardiography
o Hearing screen
o Fundocscopy
➢ Discuss diagnosis and
management plan with parents
 ➢ Refer parents to an infant defects (ASD, VSD) to total
intervention program and to a absence of septum.
support group ➢ Murmur on auscultation is due
➢ Conduct a follow-up meeting to turbulent flow of blood
with family through defect.
• Other defects: PDA, Tetralogy of
COMPLICATIONS AND
fallot, Mitral valve prolapse, Aortic
ASSOCIATED and mitral regurgitation.
ABNORMALITIES • Presence of heart disease is the
CENTRAL NERVOUS SYSTEM most significant factor in
• Delayed motor milestones determining survival.
• Moderate to severe learning • Alerting signs are poor feeding,
difficulties easy fatigability, dyspnea,
• Early onset Alzheimer’s disease diaphoresis, cyanosis and a cardiac
• Epilepsy murmur.

EYE PROBLEMS HEMATOLOGICAL

• Increased risk of cataract, • Patients with Down syndrome are
nystagmus, squint and at increased risk of development of
abnormalities of visual acuity. lymphoproliferative disorders,
• Routine evaluation is performed in including acute lymphoblastic
infancy and then yearly. leukemia, acute myeloid leukemia,
myelodysplasia and transient
HEARING DEFECT lymphoploriferative syndrome.
• May be uni-/bilateral, conductive • Other disorders include
or sensorineural or mixed. polycythemia, thrombocytopenia
• 40-60% have conductive hearing and erythroblastosis fetalis.
loss and are prone to serious otitis
media (most commonly during the GASTROINTESTINAL
first year). MALFORMATION
• Routine evaluation before 6 months • Duodenal atresia present in 5% of
of age and then every year is cases.
advisable • The infant may present with bile
stained vomiting, abdominal
CONGENITAL HEART DISEASE distension and a visible peristaltic
• 50% have endocardial cushion wave. A “double bubble sign”
defects ranging from isolated septal

35
 characterizes the abdominal participation in special games or
radiograph. earlier if signs and symptoms
• The obstruction may be due to suggest cord compression.
congenital atresia, intrinsic • Down syndrome is associated with
stenosis, or extrinsic stenosis low birth weight, small head
secondary to annular pancreas or circumference, decreased length
malrotation of the bowel with and growth rate.
bands. • The prevalence of obesity is greater
• There is an increased risk of (weight is less than expected for
esophageal atresia, annular length).
pancreas, pyloric stenosis, ➢ Obstructive sleep apnea can
malrotation of the bowel, result from obesity.
diverticulum of the stomach and
Hirschsprung disease
SKIN
• Imperforate anus • Palmoplantar hyperkeratosis
• Strong association with celiac • Seborreic dermatitis
disease and cystic fibrosis • Fissured tongue
• Geographical tongue
ENDOCRINE • Xerosis (Dry skin)
• Hypothyroidism.
• Thyroid function tests (T3, T4 and REPRODUCTIVE
TSH) are recommended once in the • Females are fertile and may
neonatal period or at first contact become pregnant.
and then every year. This should • Males: micropenis, cryptorchidism,
ideally include antithyroid hypospadias.
antibodies specially in older MANAGEMENT AND
children as etiology is more likely
PROGNOSIS
to be autoimmune.
• Growth- plot growth on appropriate
• Diabetes mellitus type 1 (3 times
charts used in down syndrome.
risk)
• Cardiac disease screening
MUSCULOSKELETAL SYSTEM • Hearing- every 3 months until 3
• There is variable incidence of years and then annually.
atlanto-occipital subluxation. • Eye disorder screening
Lateral neck radiograph is • Thyroid function test
recommended once between 3 and • Celiac disease screening
5 years before surgery for • Hematology- FBC

36
• Atlanto-axial X-ray as they reach 4 years. The
• Patients with Down syndrome have remainder of patients have reduced
a shortened life expectancy. About life expectancy as compared to the
1/3 die within the 1st year, and ½ die general population.

37
SEX CHROMOSOME SYNDROMES
TURNER SYNDROME
• This is a chromosomal disorder in
which a female is born with only
one X-chromosome (45XO).
• Incidence is 1: 2 000 live birth.
• Turner syndrome is the most
important cause of hyper-
gonadotropic hypogonadism in
girls.
• These girls present with short
stature, classical phenotypic
features and delayed puberty.
• Most cases of turner syndrome are
not inherited. When this condition
results from monosomy X, the
chromosomal abnormality occurs
as a random event during the
formation of reproductive cells in
the affected person’s parent. It is
due to nondisjunction resulting in
the loss of a sex chromosome.
➢ Most common karyotype is 45,
X.
• In mosaic turner syndrome there is
a random event during cell division
in early fetal development. As a
result, some of an affected person’s
cells have the usual 2 sex
chromosomes and others have only
one X copy.
➢ Mosaic forms like 45, X/46, XX
and 45, X/46, XY have also
been observed.
• Rarely, turner syndrome can be
caused by a partial deletion of the
34
X-chromosome this can be passed
on the next generation.
• Most girls and women with turner
syndrome have normal intelligence,
developmental delays, nonverbal
learning disabilities and behavioral
problems although these vary from
individual to individual.
CLINICAL FEATURES
• Short stature (most frequent
finding, becomes evident by age 5).
• Webbed neck and low posterior
hairline.
• Shield chest with broadly spaced
nipples and scoliosis or kyphosis
• Swelling of the dorsum of hands
and feet (congenital lymphedema)
may be present at birth.
• Cubitus valgus (wide carrying
angle),
• Ovarian dysgenesis causes delayed
puberty. Turner syndrome should
be considered in any female with
pubertal delay. Hormonal therapy
is typically needed to stimulate
puberty.
• Cardiac defects usually include
left-sided heart lesions especially
coarctation of the aorta, mitral
valve prolapse, aortic valve
stenosis, and hypoplastic left heart
are common.
• Hypothyroidism and diabetes
mellitus may occur.
• Renal malformations like the renal pelvis and agenesis may
horseshoe kidney, reduplication of also be present.

DIAGNOSIS exclude cardiac and renal

• This is based on clinical features malformation.
and chromosomal analysis (to ➢ Periodic hearing evaluation for
exclude the presence of a Y deafness is recommended.
chromosome, which is associated MANAGEMENT
with gonadoblastoma in 25-30%
• Growth hormone therapy (0.1-0.15
cases).
unit/kg/day) is indicated in Turner
• Other investigations:
syndrome for improving stature.
➢ Ultrasound of pelvis:
• Estrogen treatment should be
hypoplastic uterus and poorly
deferred till the age of 12 years to
developed ovaries
ensure adequate growth.
➢ FSH levels: elevated
• Gonadectomy is recommended in
➢ Thyroid profile and blood sugar
patients with a Y chromosome in
should be done at baseline and
view of high risk of
yearly.
gonadoblastoma.
➢ Echocardiography and
ultrasound for kidneys to

34
CLINCAL CASE: A case of short girl
HISTORY
Tanya is a 4-year-old girl brought to the GP by her mother, who is worried
about her daughter’s growth. She has noticed that her shoe size has not changed
for almost 12 months and she is still in clothes for a 2- to 3-year old. She was
born at 38 weeks by normal delivery and weighed 2.1 kg (<9 th centile). Her
mother tried breast-feeding but she was never easy to feed, even with a bottle.
She is generally healthy, apart from recurrent ear infections that have needed
grommet insertion. She wears glasses for long-sightedness. Her development
is normal, although nursery staff have reported that she seems to have poor
concentration.

EXAMINATION
On examination she is generally healthy and certainly well nourished. The GP
notices wide-spaced nipples and a low hairline but can find no other obvious
abnormalities.

QUESTIONS
1. What is your diagnosis?
2. What are the clinical signs that suggest a pathological cause for short
stature?
3. What other features are consistent with this condition?

34
 33

KLINEFELTER
• Klinefelter is the most common
cause of male hypogonadism and
infertility in males.
• Chromosomal analysis generally
reveals an XXY genotype in males.
➢ In females a genotype of 47,
XXX is called triple X-
syndrome.
• Individuals with XXY/XY
mosaicism have a better prognosis.
• As the number of X chromosomes
increases beyond two, the clinical
manifestations increase
correspondingly.
• Risk increases with advancing
maternal age.
• Incidence is 1:500 live male births.
CLINICAL FEATURES
• Tall stature with long extremities
• Hypogonadism including small
penis and testes, delayed puberty
owing to lack of testosterone and
infertility. DIAGNOSIS
• Gynaecomastia (40%) • Based on chromosomal analysis
• Variable intelligence • The diagnosis should be considered
• Behavioral findings include in all boys with mental retardation,
antisocial behavior and excessive as well as in children with
shyness or aggression. These psychosocial, learning disability or
findings may be noted before the school adjustment problems.
appearance of the physical
MANAGEMENT
findings.
• Behavioral and psychosocial
• Occasionally, hypospadias or
rehabilitation.
cryptorchidism is present.
• Testosterone therapy should be
started in middle to late
 adolescence with monitoring of
levels.

CLINCAL CASE: A case of a boy with breasts

HISTORY
Anthony is a 15-year-old boy who presents to an outpatient clinic with an 18-
month history of gynaecomastia. There is occasional breast tenderness. There
is no history of galactorrhea. He has stopped doing sports at school as he is too
embarrassed to undress in front of his classmates in the changing room. He is
a little behind at school and requires extra help. His aunt has breast cancer and
his grandmother died of breast cancer. He is on no medication and has no
allergies

EXAMINATION
On examination there is moderate symmetrical gynaecomastia. Pubertal
staging is as follows: pubic hair, Tanner stage 4, genitalia, Tanner stage. Testes
are both 15ml in volume (using the Prader orchidometer). There are no
abdominal masses and no other signs. His weight, is 78kg (between 91 st and
98th centile) and his height is 169cm (50th centile).
FSH, LH, Testosterone and Estradiol level were done and all were normal.
QUESTIONS
1. What is your diagnosis?
2. What other features are consistent with this condition?
3. What is the treatment?

34
 CHAPTER 7: NEUROLOGICAL CONDITIONS

NEUROLOGICAL CONDITIONS
APPROACH TO NERUROLOGICAL DISORDERS
• Clinical assessment involves:
➢ History
➢ Physical examination
➢ Investigations
HISTORY
• Onset of illness:
➢ Mode of onset gives clues about the etiology
o Head trauma, vascular causes, acute demyelinating encephalomyelitis
(ADEM) and acute infections are sudden in onset
o Subacute onset is characteristic of infections with organism of low
virulence and neurodegerative process
o Meningococcal meningitis has a galloping course, whereas tuberculous
meningitis may go on for weeks
o A relapsing and remitting course can occur in multiple sclerosis and Devic
disease.
o A progressive course indicates degenerative and neoplastic disorders.
• Developmental history
➢ Helps define the time of onset and other ailment
➢ All the developmental milestones are delayed if the disease begins at or near
the time of birth of the child
➢ Milestones may regress with acquired insults or degenerative disease of the
nervous system
➢ Always ask for consanguinity (hereditary) and family history of neurological
disorders.
PHYSICAL EXAMINATION
• Inspection is a crucial part of neurological examination
➢ Observe posture, quality and symmetry of spontaneous movement, behavior,
apathy, interest in surroundings, hyperkinesis, involuntary movements such
as tremors, athetosis, chorea, myoclonus and convulsions.
• Cranial nerves

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 ➢ Response to light stimuli and pupillary reflexes shows integrity of the second
and third cranial nerves
➢ Ophthalmoplegia and paralytic squint indicate involvement of third cranial
nerve
➢ Down and out movement of the affected eye indicate 4th cranial nerve
involvement
➢ Fifth nerve integrity can be checked by conjunctival or corneal reflex
➢ 6th nerve paralysis, diagnosed by convergent paralytic squint may be a false
localizing sign
➢ Facial asymmetry, loss of nasolabial fold on the ipsilateral side, pulling of the
angle of the mouth on contralateral side and drooling of saliva indicates
paralysis of 7th cranial nerve. The integrity of cochlea division of 8 th nerve is
check by auditory tracking.
➢ 9th and 10th nerve integrity is determined by gag reflex and palatal movement
➢ If the child can shrug his shoulder and turn his neck from side to side, 11 th
(accessory) is intact.
➢ In 12th nerve palsy, the tip of the tongue is deviated to the side of the lesion.
• Motor examination
➢ Best power in all limbs during spontaneous movement should be recorded in
infants and toddlers
➢ Detailed assessment of power should be attempted in older children.
➢ Assessment of tone helps in the localization of lesion
➢ Deep tendon reflexes
o Best elicited when the concerned muscle groups are relaxed.
o Exaggerated deep tendon reflexes imply upper motor neuron lesions and
diminished reflexes are observed in lower motor neuron disease
o Cerebellar lesions cause pendular knee jerks
• Developmental examination
➢ In infancy, tone, posture, neonatal reflexes, appearance of postural reactions
are to be assessed.
➢ In addition, gross and fine motor functions, socioadaptive and language
evaluation should be done using standard tests or charts of development.
➢ Hearing and visual evaluation is also mandatory.
INVESTIGATIONS
LUMBAR PUNCTURE
• Indicated in:

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 ➢ Inflammatory CNS disorders
➢ Neonatal sepsis
➢ Malignancies (to determine CNS spread and for therapy)
➢ Autoimmune diseases
➢ Demyelinating illnesses, slow virus infections especially subacute sclerosing
panencephalitis (SSPE) and for lactate, neurotransmitters and glycine in
neurometabolic and neurotransmitter disorders
IMAGING
• These include
➢ Ultrasonography
➢ CT scan
➢ MRI
➢ Electroencephalogram (EEG): indicated in epilepsy and epilepsy syndromes.

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HYDROCEPHALUS
• CSF is secreted by the choroid plexus within the
ventricles.
➢ A large amount of CSF is formed in the lateral
ventricle.
➢ The majority of CSF formed in the lateral ventricle
enters the third ventricle by passing the foramen of
Monro.
➢ CSF passes from the third ventricle to the fourth
ventricle through the cerebral aqueduct (aqueduct of
Sylvius).
➢ From the fourth ventricle CSF flows to the Cisterna
magna (through the foramen Magendie-central
opening) and the Cisterna lateralis (through the
foramen of Luschka-lateral opening).
➢ From the cisterna magna and cisterna lateralis, CSF
circulates through the subarachnoid space over
spinal cord and cerebral hemispheres. It also flows
into the central canal of the spinal cord.
• CSF is absorbed via the arachnoid vili (granulations) into the venous channels
and sinuses.
• About 20ml of CSF is secreted in an hour and its
turnover is 3-4 times in a day.

37
• Hydrocephalus is increased cerebrospinal fluid (CSF) under pressure within the
ventricles of the brain.
• It results from blockage of CSF flow, decreased CSF absorption or rarely
increased CSF production.
TYPES OF HYDROCEPHALUS
• They include:
➢ Non-communicating or obstructive hydrocephalus: enlarged ventricles caused
by obstruction of CSF flow through the ventricular system. The block is at
any level of the ventricular system commonly at the aqueduct or foramina of
Luschka and Magendie (e.g. stenosis of the aqueduct of sylvius). The
ventricles are dilated above the block.
o Periventricular ooze on CT or MRI helps to identify obstructive
hydrocephalus.
➢ Communicating hydrocephalus: enlarged ventricles as a result of increased
production of CSF (e.g. tumors- papilloma of choroid plexus) or decreased
absorption of CSF (e.g. bacterial meningitis)
➢ Normal pressure hydrocephalus
➢ Hydrocephalus ex vacuo: this is enlargement of ventricular system due to
compensatory response to severe cortical atrophy. It is not true hydrocephalus.
ETIOLOGY
CONGENITAL CAUSES
• Intrauterine infections: Toxoplasmosis, Cytomegalovirus, Rubella
• Intraventricular and intracranial bleeds
• Congenital malformations:
➢ Congenital aqueductal stenosis (Some cases of aqueductal stenosis are
inherited as an X-linked trait and these patients may have thumb abnormalities
and other CNS anomalies such as spina bifida)
➢ Dandy-Walker malformation: a combination of an absent or hypoplastic
cerebellar vermis and cystic enlargement of the fourth ventricle which blocks
the flow of CSF. There is a posterior fossa cyst continuous with fourth
ventricle.
➢ Arnold Chiari type II malformation: characterized by downward displacement
of the cerebellum and medulla through the foramen magnum into the cervical
spinal, blocking CSF flow to the posterior fossa. This malformation is often
associated with a lumbosacral myelomeningocele.

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• Midline tumors obstructing CSF flow
ACQUIRED CAUSES
• Infections: TB, chronic and pyogenic meningitis.
• Post intraventricular hemorrhage
• Posterior fossa tumors: medulloblastoma, astrocytoma, ependymoma.
• Arteriovenous malformation, intracranial hemorrhage (most common in preterm
infants), ruptured aneurysm
PATHOLOGY
• Ventricles are dilated at times unevenly.
• Ependymal lining of ventricles is disrupted resulting in periventricular ooze and
hence periventricular white matter is compressed.
• Cortex is generally preserved until late but cortical atrophy may occur. The
process may be reversible if the treatment is imitated early.
CLINICAL FEATURES
• History
➢ Did the child have any fever before the head started getting big?
➢ Any history of convulsions?
➢ What was the baby’s head circumference at birth and was the baby born at
term or preterm?
➢ Did the child cry immediately after birth?
➢ At what age did the caregiver notice the head getting bigger?
➢ Any abnormality of the spine?
➢ Any similar illness in the siblings?
➢ What treatment was given and for how long?
• Increasing head circumference that crosses percentile lines or head circumference
>97% for age.
• Infants with open cranial sutures have the following clinical signs:
➢ Large anterior and posterior fontanelles and split
sutures.
➢ Sunset sign (i.e. sclera above the cornea becomes
visible), a tonic downward deviation of both eyes
caused by pressure from the enlarged third ventricle on
the upward gaze center in the midbrain.

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 ➢ Abnormal skull contour and prominent forehead. Scalp veins are also
prominent and dilated.
➢ Crack-pot sound on percussion
• Older children with closed cranial sutures have the symptoms and signs of
increased intracranial pressure:
➢ Headache
➢ Nausea and projectile vomiting
➢ Personality and behavior disturbances such as irritability, head banging,
apathy and drowsiness.
➢ Unilateral sixth nerve palsy: convergent strabismus or esotropia (one or both
eyes turn inward) of which the primary symptom is diplopia (double vision)
➢ Papilledema
➢ Brisk deep tendon reflexes but with a usually downward plantar response.
• Limbs become spastic because of stretching of cortical fibers.
• Distortion of the brainstem may lead to bradycardia, systemic hypertension and
altered respiration rate (Cushing’s Triad).
• Congenital hydrocephalus starts in fetal life and may manifest or even develop
subsequently. The large head size at birth causes difficult in delivery of the head
during labor. There may be associated congenital malformations.
EVALIATION
• Increasing head circumference and signs or symptoms of increased intracranial
pressure mandate an urgent CT scan.
• Accurate serial recording of the head circumference is
essential for early diagnosis of hydrocephalus and should be
supported by serial ultrasound
➢ An increase in the head circumference in the first 3 months
of life >1cm every 2 weeks should arouse suspicion of
hydrocephalus.
➢ The brain grows very rapidly in the first week of life and
therefore sagittal and coronal suture may be separated up
to 0.5cm. The physiological separation disappears after
the first 2 weeks of life. Persistent widening of
squamoparietal sutures is not physiological and should
arouse suspicion of hydrocephalus. Figure 7: Hydrocephalus. Axial
CT scan shows marked
• CT gives information about cortical mantle, periventricular dilatation of both lateral
ooze and etiology of hydrocephalus. ventricles

37
• MRI may be needed to determine the site of obstruction and in congenital
hydrocephalus to identify associated malformations.
➢ Arnold-Chiari malformation has downward displacement of cerebellum and
medulla obstruction of CSF pathway or migration defects
➢ Dandy-walker malformation reveals a cystic malformation, atresia of outlet
foramina or any brain malformations.
MANAGEMENT
• Includes accurate diagnosis and identification of associated malformation,
clinical course and severity of hydrocephalus.
• If hydrocephalus is arrested spontaneously, surgical intervention may not be
needed.
• Medical management: done when surgical intervention is not indicated
➢ Acetazolamide 25-100mg/kg/day diminishes CSF production in mild, slowly
progressive hydrocephalus
➢ Oral glycerol has also been used for a similar purpose
• If head size enlarges rapidly or associated with progressive symptoms, where
vision or life is endangered it is treated surgically.
• In congenital obstructive hydrocephalus, acquired hydrocephalus, periventricular
ooze with hydrocephalus a ventriculoartial or preferably ventriculoperitoneal
shunt should be done to drain the CSF directly into the circulation or the
peritoneal cavity.
• Third ventriculotomy by endoscopic approach is another option in children with
obstructive hydrocephalus.
• In cases of bacterial meningitis, the acute hydrocephalus is self-limited. Patients
with TB meningitis and progressive hydrocephalus require a shunt specially if it
is obstructive.
• Shunt options present:
➢ Ventriculoperitoneal shunt
➢ Ventricular-lumbar shunt
➢ Ventricular-atrial shunt
➢ Ventricular-pleural shunt
➢ Ventricular-spinal shunt
➢ Ventricular-osseous shunt
• Complications of ventriculoperitoneal shunts include shunt infection and shunt
obstruction.
➢ Acute:

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 o Infection,
o Slit ventricular syndrome (small ventricles formed in shunt).
➢ Long term:
o Shunt becoming short as the child is growing,
o Blocked shunt,
o Dislodged shunt and
o Shunt nephritis
DIFFERENTIAL DIAGNOSIS
• Megalencephaly: this is increase in volume of brain parenchyma. There are no
signs of increased intracranial pressure. The ventricles are neither large, nor
under increased pressured. Causes:
➢ Hurler syndrome
➢ Metachromatic leukodystrophy
➢ Tay-Sachs disease
• Chronic subdural hematoma: cause large head, mostly located in the parietal
region without prominent scalp veins or sunset sign.
• Large head size is also observed in
➢ Hydranencephaly,
➢ Rickets,
➢ Achondroplasia,
➢ Hemolytic anemia and
➢ Familial macrocephaly
PROGNOSIS
• Outcome depends on the cause.
• Patients with aqueduct stenosis have the best cognitive outcome.
• Patients with Chiari type II malformation may have low-normal intelligence and
language disorders.
• Patients with X-linked hydrocephalus may have severe mental retardation.

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CEREBRAL PALSY
• Cerebral palsy is a termed used for static/ non-progressive disorders of the brain
affecting the development of movement, posture and coordination acquired early
in brain development (below the age of 2 years).
• It is the most common motor impairment in children affecting 2 per 1000 live
births.
• These motor disorders are often accompanied by disturbances of cognition,
communication, perception, sensation, behavior and seizure disorder and
secondary musculoskeletal problems.
• Although the lesion is non-progressive, the clinical picture changes as the child
grows and develops.
• Cerebral palsy is used for brain injuries occurring up to the age of 2 years. Beyond
this age it is more appropriate to use acquired brain injury as the diagnosis.
• Although the underlying cause is static the resulting motor disorder may evolve,
giving the impression of deterioration.
• The diagnosis for each child should formulate:
➢ The distribution of the motor disorder
➢ The movement type
➢ The cause
➢ Any associated impairment
• In neonates the diagnosis may be suspected if a baby has difficulty sucking,
irritability, convulsions or an abnormal neurological examination.
CAUSES
• Around 80% of cerebral palsy is antenatal (prenatal) in origin due to vascular
occlusion, cortical migration disorders or structural maldevelopment of the brain
during gestation.
• Maternal risk factors include: Multiple gestation, Preterm labor.
• The causes of Cerebral palsy can generally be classified as prenatal, perinatal and
postnatal.
PRENATAL
• Genetic factors
• Cerebral malformations

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• Intrauterine infection during pregnancy (congenital infection- TORCH
infections)
• Radiation
• Cerebral infarcation
• Metabolic defects
• Hypoxia
PERINATAL
• Prematurity <37 weeks (approximately half the cases of CP are associated with
preterm delivery & low birth weight).
➢ Periventricular leukomalacia
➢ Intracerebral hemorrhage
• Birth asphyxia
• HIE in full term: affects thalami, basal ganglia, cortex or sub-cortical white
matter
• Birth trauma
• Prolonged, precipitous delivery
POSTNATAL (BEFORE 2 YEARS)
• Hypoglycemia
• Hyperbilirubinemia
• Meningitis/encephalitis
• Subdural hematoma
• Acute infantile hemiplegia
• Head trauma
• Cardiopulmonary arrest
CLINICAL PRESENTATION
• Numerous children who develop CP will have been identified as being at risk in
the neonatal period.
• Diagnosis is usually made in the first year when the early features emerge.
• Early features of CP are:
➢ Abnormal limb and/or trunk posture and tone in infancy: initially the tone may
be reduced but eventually spasticity develops.
➢ Delayed motor milestones: delays in sitting and rolling over, accompanied by
slowing of head growth.
➢ Abnormal patterns

41
 ➢ Feeding difficulties, with oromotor incoordination, slow feeding, gagging and
vomiting.
➢ Abnormal gait once walking is achieved
➢ Asymmetric hand function before 12 months of age
➢ Persistence of primitive reflexes: such as the moro, grasp and asymmetric
tonic neck reflex.
• In CP primitive reflexes which facilitate the emergence of normal patterns of
movement and which need to disappear for motor development to progress may
persist and become obligatory.
• The diagnosis is made by findings of abnormalities of tone, delays in motor
development, abnormal movement patterns and persistent primitive reflexes.
Diagnosis may be suspected in neonates but can only be made months later.
• There are 4 main clinical subtypes:
➢ Spastic (90%)
➢ Dyskinetic (6%)
➢ Ataxic (4%)
➢ Mixed
SPASTIC CEREBRAL PALSY
• There is damage to the upper motor neuron (pyramidal or corticospinal tract)
pathway.
• Limb tone is persistently increased (spasticity) with associated brisk deep tendon
reflexes and extensor plantar responses.
• Tone in spasticity is velocity dependent, so the faster the muscle is stretched the
greater the resistance it will have. This elicits a dynamic catch which is the
hallmark of spasticity.
• The increased limb tone may suddenly yield under pressure in a ‘clasp knife’
fashion.
• Limb involvement is increasingly described as unilateral or bilateral to
acknowledge asymmetrical signs.
• Spasticity tends to present early and may even be seen in the neonatal period.
• Sometimes there is initial hypotonia, particularly of the head and trunk.
• There are 3 main types of spastic cerebral palsy:
➢ Hemiplegia: characterized by unilateral spastic motor weakness.
o Unilateral involvement of the arm and leg.
o The arm is usually affected more than the leg with the face spared.

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 o Affected children often present at 4-12 months of age with fisting of the
affected hand, a flexed arm, a pronated forearm, asymmetric reaching or
hand function (attempts at grasping always on the same side and fisting or
absent pincer on one side).
o Subsequently a tiptoe walk (toe-heel gait) on the affected side may become
evident.
o Past medical history may be normal, with an unremarkable birth history
with no evidence of HIE.
o In some, the condition is caused by neonatal stroke. Larger brain lesions
(Strokes) may cause hemianopia (loss of half of visual field) of the same
side as the affected limbs.
o Most implicated risk factors: perinatal vascular insults, postnatal trauma,
CNS malformations.

➢ Quadriplegia: characterized by motor involvement of head, neck and all 4

limbs
o All four limbs are affected severely.
o The trunk is involved with a tendency to opisthotonos (extensor posturing),
poor head control and low central tone.
o This more severe cerebral palsy is often associated with seizures,
microcephaly and moderate or severe intellectual impairment.
o There may have been a history of perinatal HIE, CNS infections, trauma,
malformations.

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 o This is also associated with seizures, scoliosis, weakness of face and
pharyngeal muscles, dysphagia, gastroesophageal reflux or aspiration
pneumonia, failure to thrive, speech problems and sensory impairments.

➢ Diplegia: involves the lower extremities more than the upper extremities or
face
o All 4 limbs but the legs are affected to a much greater degree than the arms
so that hand function may appear relatively normal.
o There is increased tone.
o Motor difficulties in the arms are most apparent with functional use of the
hands.
o Walking is abnormal.
o “Scissoring” (extension and crossing of the lower extremities with
standing or vertical suspension, a sign of spasticity)
o Most implicated risk factor: prematurity due to periventricular brain
damage.
o There may be history of early rolling over.

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DYSKINETIC CEREBRAL PALSY
• This refers to movements which are involuntary, uncontrolled, occasionally
stereotyped and often more evident with active movement or stress.
• Arms are usually more affected than legs.
• Muscle tone is variable and primitive motor reflex patterns predominate.
• May be described as:
➢ Chorea- irregular, sudden and brief non-repetitive movements.
➢ Athetosis- slow writhing/twisting movements occurring more distally such as
fanning of the fingers.
➢ Dystonia- simultaneous contraction of agonist and antagonist muscles of the
trunk and proximal muscles often giving a twisting appearance.
• Intellect may be relatively unimpaired.
• Affected children often present with floppiness, poor trunk control and delayed
motor development in infancy.
• Abnormal movements may only appear towards the end of the first year of life.
• The signs are due to damage or dysfunction in the basal ganglia or their associated
pathways (extrapyramidal).
• The commonest cause was hyperbilirubinemia (kernicterus) due to rhesus disease
of the newborn but it is now HIE at term.
ATAXIC (HYPOTONIC) CEREBRAL PALSY
• Most are genetically determined.
• When due to acquired brain injury (cerebellum or its connection), the signs occur
on the same side as the lesion but are usually relatively symmetrical.
• There is early trunk and limb hypotonia, poor balance and delayed motor
development.
• Incoordinated movements, intention tremor and an ataxic gait may be evident
later.
MIXED TYPE
• A proportion of the patients have features of diffuse neurological involvement of
the mixed type.

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DIAGNOSIS
• Diagnosis is made on clinical grounds, with repeated examinations often required
to establish the diagnosis.
• Diagnosis should be suspected in a child with low birthweight and perinatal
insult, clinically has an increased tone, feeding difficulties and global
development delay.
• Abnormalities of tone posture, involuntary movements and neurological deficits
should be recorded.
• CT or MRI scan may be useful in demonstrating cerebral malformations,
delineating their extent and ruling out very rare progressive or treatable causes
such as tumors.
• In born errors of metabolism should be excluded by screening of the plasma
amino acids and urine organic acid, reducing substance.

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DIFFERENTIAL DIAGNOSIS
• Neurodegenerative disorders: there is progressively increasing symptoms,
familial pattern, consanguinity (hereditary), specific constellation of signs and
symptoms are clues for neurometabolic disorders. Failure to thrive, vomiting,
seizures are significant symptoms. Lab investigations are necessary.
• Hydrocephalus and subdural effusion: head size is large, fontanel may bulge and
sutures may separate.
• Brain tumors or space occupying lesions: features of increased ICP.
• Muscle disorders: Congenital myopathies and muscular dystrophies can mimic
cerebral palsy. Distribution of muscle weakness and other features is
characteristic, hypotonia is associated with diminished reflexes.
• Ataxia-telangiectasia: ataxic may appear before the ocular telangiectasia are
evident
• Spinal cord injury
MANAGEMENT
• Parents should be given details of the diagnosis as early as possible, but prognosis
is difficult during infancy until the severity and pattern of evolving signs and the
child’s developmental progress have become clearer over several months or years
of life.
• Management plan should be holistic and involve the family.
• Children with cerebral palsy are likely to have a wide range of associated
medical, psychological and social problems, making it essential to adopt to a
multidisciplinary approach to assessment and management.
➢ Physiotherapy: to advise on handling and mobilization. To minimize effects
of spasticity and prevent contractures.
➢ Occupational therapy: to advise on equipment such as wheelchairs and seats
and on play materials and activities that best encourage the child’s hand
function
➢ Speech therapy
➢ Pediatric management: monitor fetal developmental progress, medical
problems, development of contractures or dislocations, behavioral difficulties
nutritional status.
➢ Nutrition: feeding methods, nutritional caloric assessment.
➢ Orthopedic surgery: management of dislocation of hips with spasticity in
thigh adductors and fixed equinus deformity of the ankle as a result of calf
muscle spasticity.

37
• Children with CP need a lot of help in everyday tasks such as dressing, bathing,
feeding. Children with milder forms of cerebral palsy can cope at mainstream
school provided minor learning difficulties and physical access are addressed
otherwise those with severe CP need special to be in special schools for a
disabled.
• Prognosis depends on the degree and type of cerebral palsy, level of learning
disability and presence of other associated problems.
• The degree of independent living relates to:
➢ Type and extent of cerebral palsy
➢ Degree of learning disability
➢ Presence of associated problems e.g. visual impairment, epilepsy

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 MENINGITIS

MENINGITIS
• This is inflammation of the leptomeninges (pia mater and arachnoid mater).
• It is classified etiologically as:
➢ Infectious:
o Bacterial
o Viral
o Fungal
➢ Non-infectious
• Patients with diminished host resistance (complement, immunoglobulin or
neutrophil function defects), malignancies, on immunosuppressive drugs are
more susceptible to develop meningitis, by fungi, Listeria and Mycoplasma.
BACTERIAL MENINGITIS
• The highest incidence of bacterial meningitis is during the first month of life.
• Acute bacterial meningitis is commoner in neonates and infants than in older
children because of poorer immunity and phagocytic functions.
ETIOLOGY
• Causes of infection are based on the age of the child:
➢ 0-1 month: Group B streptococcus, Streptococcus pneumoniae, Pseudomonas
aeruginosa, Escherichia coli, Listeria monocytogenes, Staphylococcus
aureus, Streptococcus fecalis and Salmonella species.
➢ 1-3 months: Group B streptococcus, Streptococcus pneumonia, Listeria
monocytogenes
➢ 3months- 3 years: Streptococcus pneumoniae, Haemophilus influenza type B,
Neisseria meningitidis (meningococci)
➢ 3 years- adult: Streptococcus pneumoniae, Neisseria meningitidis
• The main causes of community acquired meningitis are:
➢ Streptococcus pneumoniae
➢ Haemophilus influenza type b
➢ Neisseria meningitidis

37
RISK FACTORS
• In neonates:
➢ Prematurity
➢ Low birthweight
➢ Complicated labor
➢ Prolonged rupture of membranes
➢ Maternal sepsis
➢ Neonates on artificial respiration or intensive care
• Young age
• Immunodeficiency (e.g. asplenia- in sickle cell patients, humoral-mediated
immunodeficiency and terminal complement deficiency)
• Anatomic defects (e.g. basilar skull fracture, ventriculoperitoneal shunt)
PATHOPHYSIOLOGY
• The infection spreads hematogenously to meninges from distant foci e.g.
pneumonia, empyema, pyoderma and osteomyelitis. Purulent meningitis may
follow head injury and rarely the infection may extend from adjacent septic foci
e.g. infected paranasal sinuses, mastoiditis, osteomyelitis and fracture of the base
of the skull.
• The leptomeninges are infiltrated with inflammatory cells, the cortex of the brain
shows edema, exudate and proliferation of microglia.
• Ependymal cells are destroyed and purulent exudates collects at the base of the
brain, most marked in interpeduncular and chiasmatic cisterns. Exudates may
block the foramina of Luschka and Magendie resulting in internal hydrocephalus.
• Thrombophlebitis of the cerebral vessels may occur leading to infarction and
neurological sequela. In most cases of meningococcal meningitis the illness may
be fulminating and death may occur within a few hours because of endotoxic
shock
➢ Bacterial pathogens on destruction liberate cell wall and membrane active
components (teichoic acids, endotoxins and peptidogylcans).
➢ In response host cells and capillary endothelia produce tumor necrosis factor,
cytokines and platelet activating factors, their interaction with the blood brain
barrier and neurons results in extensive host damage
➢ Cerebral edema (vasogenic) results due to endothelial cell injury or
cytotoxins, leukocytes products and toxic radicals.

37
 ➢ The role of dexamethasone in reducing host damage due to blockage of the
above mechanisms has been demonstrated in both experimental and clinical
settings.
CLINICAL FEATURES
• Onset is acute and febrile.
• Infants and young children often have minimal and nonspecific signs and
symptoms
➢ Poor feeding, alternating irritability and drowsiness (high pitched cry or a
cat’s cry or even poor cry), lethargy, respiratory distress.
➢ Vacant stare
➢ Fever may be absent or minimal. Hypothermia may be seen.
➢ A bulging fontanelle may be present on physical examination
➢ Projectile vomiting
➢ Seizures
➢ Hypertonia
➢ Shock, circulatory collapse
➢ Neurological deficits of varying types
• Older children often present with fever and signs suggestive of meningeal
irritation
➢ Alteration in level of consciousness with irritability, somnolence (excessive
sleep) or obtundation
➢ Nuchal rigidity and positive Kernig’s and Brudzinski’s signs (these signs are
less reliably present in infants and young children)
o Kernig’s sign: extension of knee is limited to less than 135 degrees i.e.
With the child laying supine, flexion of the hip followed by extension of
the knee is limited.
o Brudzinski’s sign: the knees show flexion as the neck of the child is
passively flexed
➢ Hypertonia
➢ Seizures
➢ Photophobia
➢ Emesis (projectile)
➢ Bursting headache either diffuse or in the frontal region spreading to the neck
and eyeballs.
• The fundus is either normal or shows congestion and papilledema.

38
• If the skin of the abdomen is lightly scratched, flushing may be seen (tache
cerebrale)
• Reflexes may be normal, diminished or exaggerated.
• Neurological deficits like hemiparesis, cranial nerve palsies and hemianopsia
may develop. Respiration may become periodic or Cheyne-stokes types with
shock in the late stages of illness.
Etiology Special features
Meningococcal • Epidemics due to serotype A and less commonly type C.
meningitis • Type B causes sporadic disease
• Common in children living in crowded houses, carrier state is common
in children.
• Aside features of meningitis, children have petechial hemorrhages on
the skin or mucosa.
• Meningococcemia may be associated with acute fulminant illness with
adrenal insufficiency, hypotension, shock and coma (Waterhouse
Friderichsen syndrome) due to hemorrhage and necrosis in the adrenal
gland.
• Chronic meningococcemia may occur with intermittent fever, chills,
joint pains and maculopapular hemorrhagic rash lasting for several
days
Pneumococcal • Occurs in all ages but uncommon in first few months of life
meningitis • Usually follows otitis media, sinusitis, pneumonia or head injury
• Exudates are common on the cortex and subdural effusion is a usual
complication
Staphylococcal • Neonatal staphylococcal meningitis is often associated with umbilical
meningitis sepsis, pyoderma or septicemia.
• In older children it follows otitis media, mastoiditis, sinus thrombosis,
pneumonia, arthritis and septic lesions of the scalp or skin
Hemophilus • Common between 3 and 12 months
influenza type B • Subdural effusion should be suspected in infants whom focal
meningitis neurological signs and fever persist even after the CSF clears
biochemically and microbiologically
• Residual auditory deficit is a common complication
• Prevented by vaccination at 6 weeks, 10 weeks and 14 weeks.

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DIAGNOSIS
• Index of suspicion for bacterial meningitis should be especially high in febrile,
irritable infants.
• Evaluation should include:
➢ Lumbar puncture, for CSF microscopy, culture, sensitivity and biochemistry.
o CSF has an elevated opening pressure and it is turbid.
o Pleocytosis (WBCs in the CSF) with predominance of neutrophils. The
CSF WBC often exceeds 5, 000 cells/mm3
o Hypoglycorrhachia (low CSF glucose) ratio of CSF to serum glucose
<0.40 or CSF glucose <40mg/dl.
o Increased protein above 100mg/dl
o Positive Gram stain and culture may be noted.
o Bacterial antigens may be tested but have a low sensitivity and are not
recommended on a routine basis.
o Pretreatment with antibiotics may sterilize the CSF culture but should not
alter the CSF cellular and biochemical profile above.
➢ Blood investigations
o Blood culture: positive in the majority of cases of bacterial meningitis
o Full blood count and ESR: increased WBC (neutrophilia) and increased
ESR
o Liver function tests
o Urea, electrolytes and creatinine: for SIADH- hyponatremia
➢ CT scan with contrast to evaluate for brain abscess is often recommended,
especially for patients with focal neurologic findings. CT may exclude
subdural effusion, brain abscess, hydrocephalus, exudates and vascular
complication however it is not necessary for diagnosis of meningitis. It is also
useful in distinguishing partially treated pyogenic meningitis from
tuberculous meningitis.
➢ Rapid diagnostic test: may be used to distinguish viral, bacterial and
tuberculous meningitis based on antigen or antibody demonstration e.g.
ELISA, latex particle agglutination, countercurrent immunoelectrophoresis.
These are rapid and are unaltered by previous antibiotic therapy. Latex
agglutination and ELISA have sensitivity and specificity of about 80%.
➢ PCR is used for diagnosis of infection with herpes simplex, enteroviruses,
meningococci and TB (GeneXpert)

40
• Contraindications for an immediate lumbar puncture:
➢ Cardiopulmonary instability requiring prompt resuscitative measures for
shock or in patients in whom positioning for lumbar puncture would further
compromise cardiopulmonary function.
➢ Seizures
➢ Evidence of increased intracranial pressure (other than a bulging fontanelle):
o 3rd or 6th cranial nerve palsy with depressed level of consciousness
o Hypertension, Bradycardia and respiratory irregular respiration-Cushing
triad
o Papilledema
➢ Infection to the skin overlying the site of the lumbar puncture.
DIFFERENTIAL DIAGNOSIS
• Cerebral malaria
• Acute viral meningoencephalitis
• TB meningitis
• Cryptococcal meningitis
• Brain abscess
• Parameningeal abscess
• Malignancy
• Collagen vascular syndromes
• Exposure to toxins

41
42
MANAGEMENT
• ABCs
• Check blood glucose and treat hypoglycemia (5ml/kg of 10% dextrose for older
children, for Neonates 2-3ml/kg both of which should be followed by an infsion)
• Early empiric treatment of bacterial meningitis is critical.
➢ Antibiotics: third generation cephalosporin is recommended (ceftriaxone or
cefotaxime) combined with Crystalline penicillin
➢ Duration of treatment 10-14 days but may go up to 21 days depending on
organism isolated.
➢ Empirically: Crystalline penicillin 100, 000 IU/kg QID + IV cefotaxime
100mg/kg QID or Ceftriaxone 100mg/kg OD (avoid in neonates due to
potential risk of it displacing bilirubin from albumin)
➢ Review antibiotics when infected organism has been identified.
➢ Ages:
o <1 month (GBS and E. coli): C penicillin + Cefotaxime for 21 days
o 1-3 months (GBS, E. coli, H. influenzae, S. pneumoniae): C penicillin +
Cefotaxime for 10-21 days
o >3 months (H. influenzae 7-10 days, S. Pneumoniae 10-14 days, N.
meningitides 7 days): C penicillin + Cefotaxine or ceftriaxone
➢ Duration is extended if complications e.g. subdural empyema, brain abscess
are present. Staphylococcal meningitis and gram-negative infection are
treated for 21 days.
➢ Routing lumbar puncture at the end of therapy is not recommended. In delayed
or partial clinical response, a repeat CSF examination is indicated.
➢ Therapy is stopped if child is afebrile, CSF protein and sugar are normal and
cell count in CSF <30/mm3.
➢ Observe for 24 hours after stopping therapy and if there is no complication,
patient can be discharged.
• Corticosteroids should be given before or with the first dose of antibiotics. This
has shown to be effective at reducing the incidence of hearing loss with H.
infleunzae B.
➢ Dexamethasone 0.15mg/kg/dose QID or 0.4mg/kg BD for 2 days.
• Supportive care:
➢ Repeated medical and neurological assessment to identify early signs of CVS,
CNS and metabolic complications.

43
 ➢ Monitor vitals, pupillary reflexes, level of consciousness (patients with GCS
<9 should have an NG tube and a urinary catheter)-4 hourly
➢ Measure and note head circumference daily if fontanel still open. Consider
cranial ultrasound or CT scan if effusion or hydrocephalus is suspected
➢ Input output chart, monitor specific gravity
➢ Fit/seizure chart
➢ Keep nil per oral initially if unconscious and give 2/3 of fluid requirement
until there is no sign of SIADH
➢ Treat hypotension with IV fluids e.g. normal saline. Reduced blood pressure
may result in reduced cerebral perfusion and CNS ischemia. Treat shock
aggressively to prevent brain and organ dysfunction. Patients with septic
shock may require fluid resuscitation and therapy with vasoactive agents e.g.
dopamine.
➢ Treat seizures
o Diazepam 0.3 mg/kg/dose IV or 0.5 mg/kg rectal
o Phenobarbitone 20mg/kg IV stat then 5 mg/kg/day for intractable seizures
(start with phenobarbitone in neonates as opposed to diazepam)
▪ Note: phenytoin 18mg/kg stat is preferred as it causes less CNS
depression and permits a patient’s level of consciousness to be
assessed)
➢ Treat increased intracranial pressure
o Elevation of the head of the bed in the midline to 30 degrees
o Endotracheal intubation with mild hyperventilation
o Fluid restriction
o Osmotic diuresis with 0.5-1g/kg of 20% mannitol give 4-6 hourly for a
maximum of 6 doses.
o IV furosemide 1mg/kg (less effective than mannitol but safer)
• Follow up
➢ Note development of child at home and school
➢ Note head circumference
➢ Ask for any occurrence of fits or behavioral abnormalities
➢ Assess vision, hearing and speech
➢ Early formal hearing assessment for proven meningitis cases
➢ Follow up child until child shown to have normal development (usually until
4 years old).

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COMPLICATIONS
• Complications are highest with Gram-negative organisms followed by S.
pneumoniae, H. influenza B and finally N. meningitidis.
• CNS complications: (suspected when infant and children fail to respond to
treatment, or if fever, focal neurological signs and constitutional symptoms recur
after a lapse of few days)
➢ Subdural effusion or empyema
➢ Ventriculitis, arachnoiditis
➢ Brain abscess
➢ Hydrocephalus
➢ Long term neurological deficits
o Sensorineural auditory impairment (deafness): Hearing loss is the most
common complication occurring in up to 25%of patients
o Learn disability
o Ocular palsies
o Hemianopsia,
o Aphasia
o Hemiplegia
• Systemic complications:
➢ Shock
➢ Myocarditis
➢ Status epilepticus
➢ SIADH (Syndrome of inappropriate ADH secretion)
PROGNOSIS
• Depends on
➢ Age: worse in younger patients
➢ Duration of illness prior to effective antibiotic treatment
➢ Causative organism: more complications with H. influenzae, S. pneumoniae
➢ Presence of focal signs.
ASEPTIC MENINGITIS

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46
 SEIZURE DISORDERS OF CHILDHOOD

SEIZURES DISORDERS OF CHILDHOOD

• A seizure is an abnormal, paroxysmal, excessive firing (electrical discharges) of
the cerebral neurons that may involve transient, involuntary alteration of
consciousness, behavior, motor activity, sensation or autonomic function.
➢ Motor movements consists of tonic and clonic components are the most
commonly observed phenomenon, except in the newborn period.
• Epilepsy is the occurrence of 2 or more spontaneous seizures without an obvious
precipitating cause (unprovoked).
• Status epilepticus is a seizure that lasts more than 5 minutes or when seizures
occur close together i.e. 2 or more seizures within a 5-minute period without the
person returning to normal between them.
➢ Previous it was described as a seizure that lasts more than 30 minutes.
• The seizure discharge is caused by an imbalance between excitatory and
inhibitory input within the brain or abnormalities in the membrane properties of
individual neurons.
• In some children the cause of seizures is known however in 60-70% of cases the
cause is unknown.
• Conditions that may mimic or be misinterpreted as a seizure include:
➢ Convulsive syncope with or without cardiac dysrhythmia
➢ Decerebrate posturing
➢ Psychogenic events
➢ Dystonia
➢ Migraine
CLASSIFICATION OF SEUZIRES
• Criteria for classification are:
➢ Whether consciousness is impaired or not
➢ Nature of the movements
➢ Extent of brain involvement
➢ Presence or absence of fever
• Generally, they can be classified into 2 broad categories:
➢ Febrile seizures: common but benign seizures associated with fever. Include:
o Simple
o Complex

47
➢ Afebrile seizure: Either classified as generalized or partial depending on
whether both sides or one side of the brain are involved as well as whether
consciousness is impaired or not
o Generalized seizures: caused by discharge from a group of neurons in both
cerebral hemispheres. Include
▪ Tonic-clonic/ Grand-mal (common): this is the most common type of
generalized seizure. They are characterized by increased thoracic and
abdominal muscle tone. Followed by clonic movements of the arms
and legs, eyes, rolling upward, incontinence, decreased consciousness
and a postictal state of variable duration.
▪ Tonic
▪ Clonic
▪ Myoclonic
▪ Absence/ Petit-mal (common): are brief staring spells that occur
without loss of posture and with only minor motor manifestations e.g.
eye blinking or mouthing movements. The seizure lasts <15 seconds
and there is no postictal state.
▪ Atonic
o Partial (focal) seizures are caused by the discharge from a group of neurons
in one hemisphere. The seizure symptoms may have predominately motor,
sensory or psychomotor features. There are 2 types:
▪ Simple: consciousness is not impaired.
▪ Complex partial seizures: consciousness is decreased.
o Partial seizures with secondary generalization

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ETIOLOGY
• Early neonatal period (0-7 days)
➢ Birth asphyxia and difficult obstructed labor
➢ Intraventricular, intracerebral hemorrhage
➢ Pyridoxine dependency, hypoglycemia, hypocalcemia, hyponatremia,
hypernatremia
➢ In-born errors of metabolism
➢ Maternal withdrawal of medications
➢ Injection of local anesthetic into the fetal scalp during the paracervical block
given to the mother
• Neonatal period (7-30 days)
➢ Transient metabolic: hypocalcemia, hypomagnesemia, hypoglycemia,
hyponatremia, hypernatremia
➢ Developmental malformations
➢ Infections: Meningitis, septicemia, tetanus neonatorum, intrauterine
infections
➢ Metabolic errors: Phenylketonuria, maple syrup urine disease, galactosemia,
urea cycle disorders
• Beyond neonatal period
➢ Simple febrile convulsions
➢ Epilepsy syndromes
➢ Infections: Bacterial meningitis, intrauterine infections, tuberculous
meningitis, aseptic meningitis, encephalitis, cerebral malaria, Reye syndrome
➢ Metabolic: hyponatremia, hypernatremia, hypocalcemia, hypomagnesemia,
inborn errors of metabolism
➢ Space occupying lesions: neoplasm, brain abscess, tuberculoma, cysticercosis
➢ Vascular: AV malformations, intracranial thrombosis, hemorrhage
➢ Miscellaneous: hypertensive encephalopathy, sequelae of birth trauma and
birth asphyxia, gray matter degeneration, storage disorders
➢ Drugs, poisons: phenothiazines, salicylates, phenytoin, strychnine, carbon
monoxide, lead

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APPROACH TO A CHILD WITH CONVULSIONS
HISTORY AND PYHSICAL EXAMINATION
• An accurate seizure description is more informative than detailed neurological
examination or investigations.
• Careful history is warranted to ascertain potential family history of epilepsy, a
prior neurological disorder or history of seizure without a fever.
• History of seizure:
➢ Mode of onset
➢ Details of aura
➢ Type of seizure
➢ Automatism
➢ Associated behavioral abnormalities
➢ Postictal phase
• Perinatal, developmental and family history of seizures helps in determining
cause
• Examine the child for evidence of raised intracranial tension, degenerative,
metabolic or congenital disorders.
INVESTIGATIONS
• Based on history, a normal neurologic examination and the exclusion of any CNS
infection.
• Examination should focus on the cause of the fever, which is usually a viral
illness, but a bacterial infection including meningitis should always be
considered.
• A lumbar puncture is necessary only if meningitis is suspected.
➢ If the child is unconscious or has cardiovascular/respiratory instability,
lumbar puncture is contraindicated and antibiotics should be started
immediately.
• Estimation of serum glucose, serum electrolytes (calcium, sodium and
magnesium) and screening tests for neurometabolic causes usually suffice.
Detailed metabolic studies including screening of amino acids, blood ammonia,
blood and CSF lactate/pyruvate levels are indicated if inborn errors of
metabolism are suspected and in familial seizures.
• Neither neuroimaging (Cranial imaging-MRI preferred over CT scan) nor EEG
is needed unless the neurologic examination is abnormal.

39
 ➢ EEG: it is the best supplementary test for classification and diagnosis of
epilepsy. It should be used to support the diagnosis of seizure, diagnosis of
certain epilepsy syndromes, localize an epileptic focus and determine its
anatomical basis. EEG does not always help in determining the duration of
therapy. Focal EEG abnormalities justify the need for imaging.
Cranial imaging: X-ray films of the skull are not helpful, except in microcephaly,
scattered calcification, suture evaluation and thickening of the calvarium. MR
imaging, CT scans and functional imaging are indicated in partial seizures, seizures
with focal neurological deficits, dysmorphic features, or skin lesions suggesting
neuroectodermatoses and in the presence of raised intracranial pressure.
STATUS EPILEPTICUS
• This is a prolonged single seizure or multiple episodes of seizures lasting more
than 5 minutes without regaining consciousness in between (20 to 30 minutes
used to be used as the threshold)
• Status epilepticus can be classified as:
➢ Convulsive (common): associated with significant morbidity and mortality
o Tonic-clonic
o Clonic
o Myoclonic
➢ Nonconvulsive
o Absence
o Non convulsive
o Speech sensorial alteration
• The neurological sequelae following Status epilepticus depends upon etiology,
age and duration of status epilepticus.
• The risk of complications increases substantially with duration (>60 minutes).
• Neurological residua include:
➢ Retardation
➢ Focal neurological deficits
➢ Behavioral disorders
➢ Chronic epilepsy
ETIOLOGY
• Infectious
➢ Encephalitis
➢ Meningitis

40
 ➢ Intercurrent infections in known epileptics
• Non-infectious
➢ Known epileptic who is non-compliant to treatment, is on erratic supply of
drugs, is sleep deprived
➢ Initial presentation of epilepsy
➢ Congenital malformations of the brain (lissencephaly, shizencephaly)
➢ Electrolyte abnormalities- hypocalcemia, hypoglycemia
➢ Drug intoxication- amphetamines, cocaine, phenothiazines, theophylline,
tricyclic antidepressants
➢ Idiopathic- e.g. following sudden withdrawal of anticonvulsants
➢ Others- Reye’s syndrome, lead intoxication, extreme hyperpyrexia, brain
tumor
PATHOPHYSIOLOGY
• Status epilepticus results from excessive and persistent excitation, or ineffective
recruitment of inhibition.
• Excitatory neurotransmitters include glutamate, aspartate and acetylcholine and
the dominant inhibitory neurotransmitter gamma-aminobutyric acid.
• The blockage of N-methyl-D-aspartate (NMDA) channels by magnesium ions
seems to be important in the pathogenesis of neuronal damage in status
epilepticus.
• Associated hypoxia, hypotension, acidosis and hyperpyrexia exacerbate the
neuronal.
EVALUATION OF THE PATIENT
• History is taken for description of the event, associated symptoms, duration and
the post-ictal period, prior history of seizures, non-compliance with antiepileptic
drugs or change of AED and history of prior neurological development.
• If postictal confusion does not resolve search for other causes e.g. hypoglycemia,
dyselectrolytemia, CNS infection, CNS vascular event, drug toxicity, psychiatric
disorders and nonconvulsive status epilepticus.
• Investigations:
➢ Blood:
o Full blood count with differential count
o Random blood sugar
o RDT and Malarial parasite slide (if fever is present)
o Urea, electrolytes (calcium, magnesium, phosphate) and creatinine

41
 o Liver function tests
o Serum lactate
o Arterial blood gasses if present
➢ CSF studies: CNS infection
➢ Neuroimaging: can reveal neuroinfections, neurocysticercosis, acute head
trauma, malignancy, meningoencephalitis, neurometabolic disorders,
persistent headache
➢ EEG: an urgent EEG is recommended for patients with status epilepticus if
non-convulsive status epilepticus is suspected
MANAGEMENT
• Goals of therapy
➢ Ensure adequate vital signs, systemic and cerebral oxygenation
➢ Terminate seizure activity
➢ Prevent seizure recurrence
➢ Establish the diagnosis and treat the underlying cause if possible
• ABCDE
➢ Establish and secure airway, ensure patient is breathing, and place patient in
left lateral decubitus position. Suction airway if necessary
➢ Give oxygen by nasal cannula or mask, assess for need for endotracheal
intubation
➢ Gain venous access (2 IV access), send bloods to lab, administer dextrose
emperically and administer drugs to abort seizure
• Give 10% dextrose- 5ml/kg IV stat, then 10% dextrose infusion over 4-6 hours.
Systolic blood pressure should be maintained at normal levels, temperature
should be monitored and hyperthermia treated accordingly.
• Diazepam IV 0.3mg/kg stat or 0.5mg/kg rectal (Lorazepam 0.1mg/kg is better as
it does not depress respiration as compared to diazepam and has a longer duration
of action.) If seizure continues repeat after 5-10 minutes up to a maximum of 10
mg.
• If seizure continues, phenytoin IV loading dose of 20mg/kg to run in 20 minutes
(max 50 mg/min) in saline. A therapeutic effect can be seen in 20 minutes.
NOTE: Saline should be used for dilution and not dextrose, dextrose solutions
precipitate phenytoin and so should not be used.
➢ Side effects of phenytoin: hypotension, cardiac dysarrhythmia, phlebitis and
tissue necrosis from extravasation, movement disorder and cerebellar ataxia.

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• In case of poor response to benzodiazepines and phenytoin, phenobarbitone IV
is administered at loading dose of 20mg/kg at rate of 2mg/kg/min, maintenance
is 5mg/kg OD or in 2 divided doses. Caution must be exercised in patients who
have already received a benzodiazepine because respiratory depression may be
exacerbated. Phenobarbitone is the drug of choice in neonatal seizures, cardiac
conduction abnormalities and in hypersensitivity to phenytoin.
• Paraldehyde can be administered rectally (0.3 ml/kg diluted 1:3) or
intramuscularly. It should be given in a glass syringe as it dissolves plastic or
rubber.
• For refractory status epilepticus:
➢ Modalities include barbiturate coma, diazepam or midazolam infusion, IV
sodium valproate, thiopental and inhalation anesthesia. This is done in PICU
where facilities for artificial ventilation exist.
• Treat the underlying cause if possible

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44
FEBRILE SEIZURES
• A febrile seizure is any seizure that is accompanied by a fever (Temp> 38oC) and
is not caused by any CNS infection such as meningitis in neurologically normal
patients from 6months to 6 years of age.
• The pathophysiologic mechanism is unknown.
• Febrile seizures can be inherited and several gene mutations have been
implicated.
• Febrile seizures occur in about 3% of children.
• The convulsions are not related to the degree of temperature but are frequent if
temperature rises abruptly.
CLASSIFICATION
• Simple/benign febrile seizure: Occurs within 24hours of the onset of fever, lasts
less than 15 minutes and is generalized tonic-clonic.
o There is no postictal neurological deficit.
o Simple febrile seizures do not cause brain damage.
o There is a 1-2% risk of developing epilepsy
• Complex/ atypical febrile seizure: lasts longer than 15 minutes, has focal features
or recurs within 24 hours.
o These are associated with post-ictal focal deficit.
o Presence of family history of epilepsy, neurodevelopmental retardation
and atypical episodes increase recurrence risk of febrile seizures and
subsequent epilepsy.
o Convulsions in developmentally challenged children may be precipitated
by fever, as the cerebral threshold for seizures is reduced with the elevation
of temperature. These are distinct from febrile convulsions, which occur in
a neurodevelopmentally normal child
o Complex febrile seizures which are focal, prolonged or repeated in the
same illness have an increased risk of 4-12% of subsequent epilepsy.
CAUSES
• Viral upper respiratory tract infections
• Malaria
• Acute otitis media
• Genetic predisposition e.g. autosomal inheritance pattern in some families.

45
DIAGNOSIS
• Blood: to find cause of fever and rule out other causes of seizures
o Full blood count with differential count
o Random blood sugar
o RDT and Malarial parasite slide
o Urea, electrolytes (calcium, magnesium, phosphate) and creatinine
o Liver function tests
o Serum lactate
o Arterial blood gasses if present
• Nasopharyngeal swab
• Urine microscopy/culture/sensitivity
• Lumbar puncture: infections of the CNS such as meningitis or encephalitis, are
important causes of convulsions associated with fever and can be easily confused
with simple febrile convulsions. Lumbar puncture should be performed in the
first episode a febrile seizure in infants below 1 year who are not immunized
against HiB and pneumococcal vaccine or if immunization status is not known
and where meningitis is suspected. Though this is not required routinely for every
febrile convulsion.
• EEG and neuroimaging have no role in febrile seizures.
MANAGEMENT
• Maintenance of airway, breathing and circulation.
• First time or occasional febrile seizures are not treated with anticonvulsants.
• Aggressive antipyretic treatment of subsequent febrile illnesses may help prevent
febrile seizures.
➢ Paracetamol at 10 to 15mg/kg/dose QID for 24 hours
• Tepid sponging is no longer recommended.
• Nurse in semi-prone position
• Frequent recurrent febrile seizures do pose a risk and may require additional
treatment including:
➢ Daily anticonvulsant prophylaxis with valproic acid or phenobarbital
➢ Abortive treatment with rectal diazepam
➢ Injection of midazolam or diazepam (0.3 mg/kg/dose IV) is given for control
of seizures or rectal (0.5mg/kg)
• If duration >10 minutes treat as status epilepticus.
• Treat underlying cause- malaria, upper respiratory tract infection etc.

46
• The family should be taught the first aid management of seizures. If there is a
history of prolonged seizures (>5 min), rescue therapy with rectal diazepam or
buccal midazolam can be supplied.
• Prognosis: approximately 30% of patients with 1 febrile seizure will have a
recurrence. Recurrence risk decreases with increasing patient age. The risk of
epilepsy is low (2%).
• Prophylaxis: may be continuous or intermittent
➢ Intermittent prophylaxis (with clobazam-0.75-1mg/kg/day, antipyretics,
hydrotherapy and meticulous temperature recording) indicated for
o 3 or more febrile seizures in 6 months
o 6 or more febrile seizures in 1 year
o Febrile seizures lasting more than 15 minutes or requiring pharmacological
therapy to control
➢ Continuous prophylaxis (with sodium valproate 20mg/kg/day or
phenobarbitone 3-5mg/kg/day for 1-2 years or until 5 years of age)
o Failure of intermittent therapy
o Recurrent atypical seizures
o Parents are unable to promptly recognize seizures

EPILEPSY
• Epilepsy is the occurrence of 2 or more spontaneous seizures without an obvious
precipitating cause (unprovoked).
• Classification similar to that listed under seizures.
• Partial seizures accounts for 60% of seizures in childhood. Common causes
include inflammatory, granulomas, atrophic lesions, vascular insults, birth
asphyxia, head trauma and neoplasms. Neurocysticercosis has emerged as a
common cause. Neurocutaneous syndrome, arteriovenous malformations and
infarcts are less frequent.

47
CLINICAL FEATURES
TYPE OF SEIZURE FEATURES
GENERALIZED SEIZURES
Tonic-clonic seizures • Most frequent
(Grand Mal type) • Has four phases
➢ Aura: a transitory premonitory symptom or aura heralds the
onset of a seizure. Aura may be sensory, visceral, motor or
autonomic. It is seen in 1/3 of patients.
➢ Tonic phase (ictal): skeletal muscles go into sustained spasm.
The muscular rigidity is most marked in antigravity muscles
e.g. flexors of arms and extensors of lower extremities. The
child becomes unconscious, falls, face appears pale, pupils are
dilated and eyes are rolled and there is frothing from the
mouth. Urine or stool may be passed involuntarily. This phase
lasts for about 30 seconds.
➢ Clonic phase (ictal): rhythmic alternating contractions of
muscle group. In many patients, epileptic phases overlap each
other.
➢ Postictal phase: child may complain of headache, confusion,
and has little recollection later. Rarely, the child develops a
transient paresis, may lose bladder/bowel control or injure
himself. EEG shows generalized burst of spikes and irregular
4-6 Hz spike wave complex
Absence seizures • Starts abruptly in childhood, peak prevalence is between 6-8
years.
• Absence seizures are not preceded by aura.
• The patients have a brief abrupt lapse of awareness or
consciousness, sudden discontinuation of the activity being
performed with starring spell, eye fluttering or rhythmic
movements. The seizure lasts less than 30s. There is no loss of
posture, incontinence of urine/stools or breathing difficulty.
• Other neurological manifestations and postictal phenomena are
absent and development is normal. Unaware of the nature of their
illness, school teachers may consider them inattentive pupils.
• Hyperventilation for 3 min often precipitates the attacks. Absence
seizures may occur in multiples, every day. Attacks following in
close succession indicate petit mal status or pyknolepsy.
• About half of patients become seizure free and the rest develop
tonic-clonic fits.

48
 • Learning disabilities and behavioral disorders when present are
probably related to associated condition.
• EEG shows a characteristic 3 per second spike and slow wave
pattern. Absence fits are distinguished from complex partial
seizures by shorter duration (10s), absence of aura and abrupt
return of full consciousness.
PARTIAL SEIZURES
Partial seizures • Simple partial without loss of consciousness, with motor,
sensory, autonomic or mixed symptoms.
• Begin with a focal epileptiform discharge howsoever brief.
• Symptoms include tingling, pain, sensation of cold, burning.
Sometimes visual, olfactory, auditory or taste hallucinations.
• When simple seizure spreads unilaterally as per the motor cortex
it is called Jacksonian march.
• Complex partial with impairment of consciousness and
automatisms, psychomotor or limbic system symptoms
• Originates from parietal or temporal lobe
Partial with secondary generalization

MANAGEMENT
• Management includes drugs, psychosocial and lifestyle management.
• Epilepsy requires management for a period of 1 to 4 year.
• Drugs used
➢ Carbamazepine:
o 10-30mg/kg/day, start with low doses
o Half-life: 13-18 hours
o Side effects: GI symptoms, hepatitis, rash, bone marrow depression
o Uses: partial, tonic clonic, atonic and akinetic
➢ Sodium valproate
o 10-60mg/kg/day in 2-3 doses
o Half-life 7-11 hours
o Side effects: idiosyncratic fatal hepatic necrosis (especially in infants),
nausea, sedation, weight gain, hair loss
o Used for all types of seizures
➢ Phenobarbitone
o 5-10 mg/kg single dose
o Half-life: 20-80 hours

49
 o Side effects: not a preferred agent, drowsiness, hyperkinesia, drug
dependency
o Uses: tonic clonic, akinetic, febrile seizures
➢ Phenytoin
o 5-10mg/kg/day in 1-2 doses
o Half-life 2-20 hours
o Side effects: hirsutism, gingival hyperplasia, toxicity if blood levels
>20microgram/ml. Ataxia, nystagmus, diplopia, drowsiness, seizures
(phenytoin encephalopathy), rash. Interferes with calcium metabolism, can
cause megaloblastic anemia.
o Uses: tonic-clonic, atonic and akinetic
➢ Lamotrigine
o 5-10 mg/kg/day; 1-5 mg/kg/day single dose with valproate 5-15mg/kg/day
with enzyme inducers
o Half-life: 14-50 hours
o Side effects: dizziness, drowsiness, blurred vision, rash, Stevens-Johnson
syndrome
o Uses: broad spectrum
SEIZURE TREATMENT
Tonic-clonic Carbamazepine is an effective drug for partial and generalized
seizures tonic-clonic seizures. Phenobarbitone is the drug of choice of the
first year of life. Phenytoin can be used as a cheaper option.

The drug should be initiated with lowest dose, the drug dose
should be increased gradually. If seizure control is inadequate or
toxicity appears an alternate antiepileptic drug should be tried
and the initial drug tapered. Polytherapy should be discouraged
unless monotherapy fails.
Complex Drug of choice is carbamazepine 10-30 mg/kg/day in 2-3
partial seizures divided doses. It has a positive psychotropic effect.
Absence Sodium valproate, lamotrigine, ethosuximide and
seizures benzodiazepines can be used
Myoclonic and Sodium valproate, benzodiazepines (clonazepam, or clobazam
atonic seizures may be used)

• Drug withdrawal should be attempted after a seizure free interval of 2 years,

gradually over 3 months.

50
• About 10-15% of patient relapse after withdrawal.
• Treatment is lifelong in patients with juvenile myoclonic epilepsy.
• Seizures are refractory in patients with underlying structural pathology in brain.
• Surgical choices:
➢ Lesional resection of epileptic areas, resection of corpus callosum
➢ Focal resection of parts of cerebral cortex such as temporal lobe and
extratemporal regions involved in epileptogenesis
• Social aspects: encourage full participation in educational and extracurricular
activities except certain sports/games like unsupervised swimming without
buddy, playing kite on roof and riding bicycle in traffic, which may endanger the
child’s life. Patients are advised regarding the necessity of regular treatment and
continuous follow up.

51
 CHAPTER 8: RESPIRATORY CONDITIONS
RESPIRATORY CONDITIONS
RELEVANT ANATOMY
AND PHYSIOLOGY OF
THE RESPIRATORY
SYSTEM
DEVELOPMENT
• By 16 weeks gestation, the
bronchial tree has developed.
• By 26-28 weeks gestation sufficient
air sacs and pulmonary vasculature
have developed so that the fetus is
able to survive.
• 90% of alveolar development
occurs after birth and alveoli
increase in number until 8 years of
age.
• During the nascent stage of
development, the lungs develop
and gradually become more
efficient and adapt to respiration.
• Fetal lung development has 5
distinct stages:
➢ Embryonic phase
➢ Pseudoglandular phase
➢ Canalicular phase
➢ Terminal saccular phase
➢ Alveolar phase
• To remember these stages, recall
the mnemonic “Every Premature
Child Takes Air”.
• The first and last stages i.e.
embryonic and alveolar stages are
37
almost 5 weeks in duration (but the
alveolar stage continues after birth
to childhood) and the middle 3
stages i.e. pseudoglandular,
canalicular and terminal saccular
stages are almost about 10 weeks in
duration. This is an easy way to
remember the stages of lung
development in fetus.
EMBRYONIC PHASE
• This phase is around 3-8 weeks of
the gestational age.
• Two lung buds branch off forming
the right and left lung.
➢ There is formation of respiratory
diverticulum (from foregut
endoderm in 4th week) to
formation of major
bronchopulmonary segments.
• The larynx and trachea develop
from the foregut.
PSEUDO-GLANDULAR PHASE
• The pseudo-gandular phase is
between the 5th-16th week of
gestation.
• There is formation of all the
conducting airways (up to terminal
bronchioles-acinus).
• 16 airway generations in humans
are completed by 16 weeks
• No respiratory bronchioles or
alveoli are present so respiration is
not possible.
CANALICULAR PHASE
• Occurs between the 16-26 week of
th
gestation.
• Respiratory bronchioles and
alveolar ducts form.
• A few terminal sacs form towards
the end of the stage- may rarely
survive with intensive care.
• A barrier between air and blood
forms which enables oxygen to
supply blood to respiratory
capillary and carbon dioxide to
depart from the respiratory
capillaries in the lungs.
TERMINAL SACCULAR PHASE
• This occurs between the 26 to 37th
week of development (birth).
• Terminal sacs/ primitive saccule
(alveoli) form. They are separated
from each other by primary septa.
• The production of surfactant starts.
• Surfactant is crucial during delivery
since it allows the amniotic fluid in
the lungs to drain away and fills the
lungs with air appropriately.
ALVEOLAR PHASE
• Lasts until early childhood (8 years
of age).
• Growth of bronchioles and alveoli.
• Gas carrying tissue in the lungs
expands and becomes more
37
efficient for carrying air during the
alveolar phase.
ANATOMY
• The right lung has 3 lobes while the
left has 2 with a lingula.
• Infants are at a higher risk for
respiratory insufficiency than older
children and adults because infants
have anatomically smaller air
passages, less compliant (Stiffer)
lungs with more compliant chest
wall, and less efficient pulmonary
mechanics.
• Congenital malformation of the
respiratory tract may be associated
with other congenital anomalies
especially of the cardiovascular
system.
PHYSIOLOGY
• Pulmonary vascular resistance
decreases after birth when the fetal
pulmonary and systemic
circulations separate and the lungs
ventilate for the first time.
• The primary function of the lungs is
gas exchange.
• Lung pathology may be classified
as obstructive or restrictive:
➢ Obstructive defects: secondary
to decreased airflow through
narrowed airways. Examples
include asthma, bronchiolitis
and foreign body aspiration
➢ Restrictive defects: secondary to
pulmonary processes that
 decrease lung volume (the • Environmental history: is
amount of air filling the alveoli). extremely important because
Examples include pulmonary fumes, strong odors, tobacco
edema, scoliosis, pulmonary smoke, allergens, animals and day
fibrosis and respiratory muscle care attendance may cause or
weakness. exacerbate pulmonary disease.
CLINICAL ASSESSMENT PHYSICAL EXAMINTION
OF PULMONARY • Should emphasize the chest and
respiratory system
DISEASE
• In general assessment, assess for
HISTORY evidence of increased work of
• History is most important in the breathing such as tachypnea
diagnosis. (Normal respiration rate in new
• Antenatal, prenatal and neonatal borns is 30-50 and infants + the
histories are very important rest=20-30 B/min), nasal flaring,
because complications of expiratory grunting and chest wall
pregnancy, fetal or postnatal retractions.
tobacco exposure, prematurity and • Evaluate ears, nose, and throat for
airway instrumentation can cause signs of obstruction, atopy or
pulmonary problems. infection.
• Review of systems: should include • Perform a chest examination:
documentation of atopy (Asthma), ➢ Inspiratory stridor suggests
failure to thrive or steatorrhea extrathoracic obstruction e.g.
(cystic fibrosis), choking croup and laryngomalacia
(aspiration) or recurrent infections (softening and weakness of
(immunodeficiency). laryngeal cartilage that
• Past medical history: should collapses into the airway,
include previous respiratory especially when in the supine
problems including frequent position).
respiratory tract infections, cough, ➢ Expiratory wheezing suggests
wheeze, stridor, snoring and intrathoracic obstruction, as in
exercise intolerance. asthma and bronchiolitis.
• Family history: should include ➢ Crackles/rales/crepitations
assessment for genetic diseases e.g. suggests parenchymal disease
cystic fibrosis, asthma. such as pneumonia and
pulmonary edema.

38
• Assess for related findings in other
organs: e.g. heart murmurs,
increased second heart sound
(elevated pulmonary pressure),
eczema, and digital clubbing.

INVESTIGATIONS
• Pulse oximetry: measures oxygen
saturation.
• Arterial blood gases (ABG): gold
standard to measure oxygenation
(PO2) and ventilation (PCO2).
• Imaging studies:
➢ Chest X-ray (CXR)
➢ Computed tomography (CT)
scan
➢ Magnetic resonance imaging
(MRI)
➢ Nuclear studies e.g. ventilation-
perfusion scans
• Pulmonary function tests: measure
airflow as a function of time
(spirometry) and lung volumes.
• Laryngoscopy and bronchoscopy
are performed in some conditions
to visualize the upper or lower
airways or to obtain
bronchoalveolar lavage specimens
for laboratory analysis. Common
indications include persistent
pneumonia, cough, stridor or
wheezing.

39
 UPPER RESPIRATORY TRACT
INFECTIONS

UPPER RESPIRATORY TRACT INFECTIONS

• The term upper respiratory tract infection embraces a number of different
conditions including:
➢ Common cold (coryza)
➢ Sore throat (pharyngitis, including tonsillitis)
➢ Acute otitis media
➢ Sinusitis (relatively uncommon)
• The commonest presentation is a child with a combination of nasal discharge and
blockage, fever, painful throat and earache. Cough may be troublesome.
• Upper respiratory tract infections may cause:
➢ Difficulty in feeding in infants as their noses are blocked and this obstructs
breathing.
➢ Febrile convulsions
➢ Acute exacerbations of asthma.
• Admission may be required in order to exclude a more serious infection, if
feeding is inadequate or for parental reassurance.

CORYZA (COMMON COLD)

CORYZA (COMMON COLD/ NASOPHARYNGITIS)

• This is the commonest infection of childhood.
• The commonest pathogens are viruses- rhinovirus (over 100 different serotypes),
coronavirus, RSV, influenza, parainfluenza and adenovirus.
• Transmission is by droplet infection.
• Predisposing factors:
➢ Chilling, sudden exposure to cold air
➢ Overcrowding,
➢ Rhinitis could also be due to allergy.

40
CLINICAL FEATURES
• Classical features:
➢ Fever (low grade), irritability
➢ Clear or mucopurulent nasal discharge (rhinorrhea)
o Mucopurulent discharge does not always indicate secondary infection it
can sometimes result from shedding of epithelial and inflammatory cells
resulting from the viral infection.
➢ Nasopharyngeal and nasal blockage (which can cause respiratory distress in
young infants because they are obligate nose breathers)
➢ Eustachian tube openings may be blocked leading to serous otitis media and
congestion of tympanic membrane.
➢ Narrowing of the airway and pharyngeal irritation causes dry hacking cough.
➢ Sore throat.
➢ Cervical lymph node enlargement.
• Excessive lacrimation is due to blocked lacrimal ducts in the nose.
• Health education to advise parents that colds are self-limiting (usually lasting 3-
4 days) and have no specific curative treatment may reduce anxiety and save
unnecessary visits to doctors.
• Persistent symptoms for more than 10 days or fever should prompt the clinician
to evaluate for bacterial superinfection (e.g. sinusitis, acute otitis media)
DIAGNOSIS
• Clinical.
• Viral agent is rarely identified.
MANAGEMENT
• Principles of management:
➢ Relieve nasal congestion
➢ Control fever
➢ Manage pain
➢ Antibiotics
• Relieve nasal congestion:
➢ Nasal saline drops PRN (when necessary)
➢ Nasal decongestants (ephedrine, xylometozoline) may cause rebound
congestion and should not be used routinely and only used in refractory cases
for limited duration.
➢ Antihistamines are also not recommended in children below 6 months.

41
 ➢ Non-sedating angents e.g. cetirizine and loratadine may be used in allergic
rhinitits. Terfenadine should not be used in children because of its potential
cardiotoxicity
• Fever and pain:
➢ Best treated with paracetamol (calpol).
➢ Avoid giving cough syrups: if cough is suppressed in infants and young
children mucoid secretions may be retained in the bronchi and this may
predispose to spasmodic cough, wheezing, atelectasis and suppuration.
• Antibiotics are of no benefit as the common cold is viral in origin. They are used
if the secretions become purulent, the fever continues to rise and if the child
develops bronchopneumonia.
• Secondary bacterial infection is very uncommon but should be treated with
antibiotics when suspected.
• There is no evidence supporting that large doses of vitamin C are helpful.
DIFFERENTIAL DIAGNOSIS
• Differential diagnosis:
➢ Foreign body in nose (presents with unilateral serosanguineous or purulent
discharge from a nostril)
➢ Snuffles of congenital syphilis: this is severe rhinitis with bilateral
serosanguineous discharge commonly excoriating the upper lip and leaving
fine scars. Nasal strictures may ulcerate leaving a flat nasal bridge.
COMPLICATIONS
• Complications:
➢ Otitis media
➢ Sinusitis
➢ Laryngitis
➢ Bronchiolitis
➢ Exacerbation of asthma
➢ Bronchopneumonia

42
 LOWER RESPIRATORY TRACT INFECTIONS

LOWER RESPIRATORY TRACT INFECTIONS

• These are the leading cause of death in children below 5 years of age.
• These include:
➢ Croup syndromes
➢ Bronchitis
➢ Bronchiolitis
➢ Pneumonia

CROUP

CROUP
• This is a term used for a variety of conditions characterized by a peculiar brassy
cough as the main presenting feature.
• Inspiratory stridor, hoarseness or respiratory distress may not always be
associated with croup.
• Diseases include:
➢ Acute epiglottitis
➢ Laryngitis
➢ Laryngotracheobronchitis
➢ Spasmodic laryngitis
• Differential diagnosis
➢ The syndromes of croup should be distinguished from each other and also
from the croup associated with diphtheria in which a membrane is seen on
laryngoscopy or occasionally with measles.
➢ Rarely the croup may result from angioneurotic edema.
➢ A retropharyngeal abscess may cause respiratory obstruction
➢ Aspiration of a foreign body is an important cause of obstruction. It may be
rarely confused with wheezing in asthma.

43
EPIGLOTTITIS
• This is acute inflammation and edema of the epiglottis, arytenoids and
aryepiglottic folds.
• There is intense swelling of epiglottis, aryepiglottic folds and arytenoids
associated with septicemia.
• Supraglottitis includes both epiglottitis and inflammatory edema of the
hypopharynx.
• The disorder is common in children 2-7 years of age, with equal incidence in
males and females. Though all age groups can be affected.
• Infection with Hemophilus influenzae type B (HIB) was the most common cause
before HIB immunization.
• This is now rare because of the success of the HIB immunization program.
• Beta-hemolytic streptococcus (Streptococcus pyogenes), Streptococcus
pneumoniae, and Staphylococcus species e.g. S. aureus may also cause
epiglottitis.
CLINICAL FEATURES
• Abrupt onset of rapidly progressive upper airway obstruction without prodrome.
• It sometimes starts with a minor upper respiratory tract illness which progresses
rapidly within the course of a few hours.
• The following signs and symptoms may occur:
➢ High fever (can be above 40oC) and toxic appearance
➢ Muffled speech (sore throat with difficulty in speaking) and quiet stridor
(softer than laryngotracheobronchitis).
➢ Ds:
o Dysphonia (muffled speech- sore throat with difficult in speaking)
o Dysphagia
o Drooling of saliva.
o Dyspnea
➢ Sitting forward in tripod position: Hyperextended neck, leaning forward and
mouth open
➢ Use of accessory muscles of respiration
➢ Marked suprasternal and subcostal retraction of the chest.
• It may progress to restlessness, pallor/cyanosis, coma and death

44
• Severe respiratory distress develops within minutes to hours and complete airway
obstruction with respiratory arrest may occur suddenly.
DIAGNOSIS
• Diagnosis is made by cautious direct laryngoscopy where there is visualization
of a cherry red swollen epiglottis when airway is established.
• Attempts to lie the child down or examine the throat with a spatula or perform a
lateral neck X-ray must not be undertaken as they can precipitate total airway
obstruction and death
• Investigations: Lab studies demonstrate
➢ Leukocytosis with left shift.
➢ 90% of patients have a positive blood culture if the epiglottitis is secondary to
HIB.
➢ Epiglottis appears like a “thumbprint” on a lateral radiograph of the neck.

Figure 8: Thumb Sign of epiglottitis on lateral soft tissue neck demonstrating epiglottitis.

DIFFERENATIAL DIAGNOSIS
• Croup
• Bacterial tracheitis
• Retropharyngeal abscess

45
DIFFERENTIATING BETWEEN SUPRAGLOTTIC AND SUBGLOTTIC
DISORDERS
Feature Supraglottic Subglottic
(Epiglottitis) (e.g. Viral croup)
Stridor Quiet Loud
Cough None Barky
Voice Muffled Hoarse
Dysphagia/ Present Absent
drooling
Fever High Low to moderate (croup)
High (tracheitis)
Toxicity Present Absent unless tracheitis is
present
Posture Neck Normal
hyperextended,
tripod position

MANAGEMENT
• Epiglottitis is a medical emergency.
• Hospital admission. A senior anesthetist, pediatrician and ENT surgeon should
be summoned and treatment initiated without delay.
• The child should be transferred directly to the intensive care unit or an anesthetic
room and must be accompanied by senior medical staff in case respiratory
obstruction occurs.
• Do not sedate the patient
• Airway and breathing management:
➢ Controlled nasotracheal intubation should be performed by experience
personnel.
➢ Before intubation, minimize stimulation by administering humidified oxygen
by hood (or next to a bowl of water or a wet towel). Face masks are not well
tolerated by these children.
➢ Keep the child calm. As oxygen therapy masks cyanosis, a careful watch
should be kept for impending respiratory failure.
➢ Do not lay the child down, keep him in a sitting position
➢ Avoid causing distress or examining the throat with a tongue depressor as this
may cause fatal laryngospasms and respiratory arrest.

46
 ➢ An urgent tracheostomy can be done also if intubation cannot be performed.
➢ The tracheal tube can usually be removed after 24 hours.
• Steroids are also given to reduce inflammation.
➢ Dexamethasone 0.3-0.5mg/kg, repeat 8-hourly for about 2-3 days max
• Circulation: cannulate, send blood for labs and start IV fluids
• Antibiotic therapy typically includes a second or third generation intravenous
cephalosporin for 5-7 days.
➢ Ceftriaxone 80-100 mg/kg/day IM in 2 injections for 5 days.
➢ Cefotaxime 100-150 mg/kg/day in 2-4 divided doses increased up to
200mg/kg daily in very severe infections.
➢ Ampicillin IV 200mg/kg/day in 3 or 4 injections, change as soon as possible
to oral treatment with amoxicillin (PO 100mg/kg/day in 2 or 3 divided doses)
to complete the 5 days of treatment
➢ Chloramphenicol 50-100mg/kg/day IV in 4 divided doses for 5 days, change
as soon as possible to oral treatment, at same doses for remaining days of
treatment
➢ Ampicillin can also be used.
• If epiglottitis is secondary to HIB, rifampin prophylaxis is indicated for
unimmunized household contacts younger than 4 years of age and close contacts.
• Prognosis is generally good with appropriate treatment and a secure airway
however, when not treated it is almost invariably fatal.
COMPLICATIONS
• Cellulitis
• Cervical adenitis
• Empyema
• Epiglottic abscess
• Meningitis
• Pneumonia
• Pulmonary edema
• Respiratory failure
• Septic shock
• Hypoxia
• Prolonged ventilation
• Death

47
LARYNGOTRACHEOBRONCHITIS (INFECTIOUS CROUP)
• Croup is inflammation and edema of the subglottic larynx, trachea and bronchi.
• There are 2 forms of croup:
➢ Viral croup
➢ Spasmodic croup
VIRAL CROUP
• This is the most common cause of stridor. It is characterized by varying degrees
of inspiratory stridor, barking cough and hoarseness due to inflammation and
edema in the laryngeal region.
• It typically occurs in children of age 1-3 years of age in the late fall and winter.
• The male to female ratio is 2:1
• Parainfluenza viruses (especially type 1) are the most common cause.
• Other organisms: respiratory syncytial virus (RSV), parainfluenza (type 2 and 3)
rhinovirus, adenovirus, influenza A and B and Mycoplasma pneumoniae.
• Bacterial etiology or bacterial superinfection are unusual.
• Clinical features:
➢ Onset is gradual, it begins with low grade fever and upper respiratory infection
(Coryzal symptoms) prodrome for 2-3 days followed by stridor and a harsh
barking cough.
o Stridor is most evident when the child is upset or agitated
➢ Symptoms:
o Mild Inspiratory stridor but as obstruction increases the stridor becomes
more marked and the suprasternal and sternal recession with respiration
become manifest.
o Fever
o Barky cough
o Hoarse voice which typically lasts 3-7 days.
o Respiratory distress may occur: Child becomes restless and anxious with
fast breathing due to increasing hypoxemia. Eventually cyanosis appears.
➢ Stridor and cough worsen at night and with agitation.
➢ As obstruction worsens, breath sounds may become inaudible and stridor may
apparently decrease. This may unfortunately be misinterpreted as clinical
improvement by an unwary physician.

48
• Usually resolves over a 3-4 day period
SIGN MILD MODERATE SEVERE
Stridor Only when At rest Severe, biphasic
agitated
Recession Mild subcostal Moderate tracheal Use of accessory
tug muscles
Level of Restless when Anxious, agitated Lethargic, drowsy
consciousness disturbed

• Anterior-posterior radiograph of the neck demonstrates the “steeple sign” (also

known as wine bottle sign) of subglottic narrowing.
➢ It is tapering of the upper trachea on a frontal chest radiograph reminiscent of
a church steeple.

Figure 9: Steeple sign/ Wine Bottle sign (Croup)

• Diagnosis should be suspected on the basis of clinical features. Neck X-rays

unnecessary unless the diagnosis is in doubt
• DDx:
➢ Acute epiglottitis
➢ Bacterial tracheitis
➢ Foreign body inhalation
➢ Anaphylaxis
➢ Retropharyngeal abscess

49
• Management:
➢ Keep the child on mother’s lap and handle gently.
➢ Do not attempt to examine the oropharynx.
➢ Assess level of severity, management is dependent on it
o Mild croup (Stridor on agitation, mild subcostal recession and restless
when disturbed):
▪ Dexamethasone 0.6 mg/kg PO/IM stat (max 12 mg) or Prednisolone
2mg/kg PO stat or Nebulized budesonide 2mg stat
▪ Observe for 4 hours
▪ Discharge after 4 hours if child stable and well
o Moderate croup (stridor at rest, moderate tracheal tug, anxious and
agitated)
▪ Admit to acute bay
▪ Dexamethasone 0.6 mg/kg PO/IM stat (max 12 mg) or Prednisolone
2mg/kg PO stat
▪ Nebulized adrenaline 1:1000 (1-5 mls) diluted in 0.9% normal saline
can be repeated every 30 minutes (2 to 3 times)
o Severe croup (Severe & biphasic stridor, use of accessory muscles,
lethargic and drowsy)
▪ Admit to pediatric intensive care unit
▪ Consult ENT surgeon/anaesthetist for possible tracheostomy
▪ Dexamethasone 0.6 mg/kg PO/IM stat (max 12 mg)
▪ Nebulized adrenaline 1:1000 (1-5 mls) diluted in 0.9% normal saline
can be repeated every 30 minutes (2 to 3 times)
▪ Give oxygen 1-3 L/min
➢ Supportive care involving Cool mist and fluids. Improvement is also noted
when patients are exposed to cool night air.

SPASMODIC CROUP
• This occurs year-round in preschool age children.
• It occurs in children between the age of 1 and 3 years.
• There is sometimes no preceding coryza.
• The cause is unknown however it is likely secondary to a hypersensitivity
reaction.
• It characteristically presents with acute onset of stridor usually occurring at night.

50
• The child wakes up suddenly with brassy cough and noisy breathing. The
symptoms improve within a few hours. The illness may recur on subsequent days.
The course is generally benign and patients recover completely.
• Spasmodic croup typically recurs and resolves without treatment.
• Humidification of the room in which the child is nursed is all that is necessary.

BACTERIAL TRACHEITIS
• This is an uncommon but reemerging cause of stridor.
• It is acute inflammation of the trachea.
• It is caused by Staphylococcus aureus (60%), Streptococcus, and nontypeable
Hemophilus influenza.
• Clinical features:
➢ Abrupt onset
➢ Toxicity, high fever and mucous and pus in the trachea
• Management: Appropriate antistaphylococcal antibiotics and airway support are
indicated.

BRONCHIOLITIS
• It is a viral infection of upper and lower respiratory tract (medium and small
airways).
• It denotes inflammation of the bronchioles.
• Bronchiolitis is the most common lower respiratory tract infection in the first 2
years of life (most commonly between the ages of 1 and 6 months).
• This disorder predominantly affects children younger than 2 years of age and is
rare after the age of 2.
• The male to female ratio is 2:1.
• Risk of infection is increased with
➢ Day care attendance,
➢ Multiple siblings,
➢ Exposure to tobacco smoke and

51
 ➢ Lack of breastfeeding (High quantities of secretory IgA antibodies to RSV are
present in the colostrum and breast feeding reduces the likelihood of an infant
being hospitalized with acute bronchiolitis).
• More significant disease occurs in patients with chronic lung disease, congenital
heart disease, history of prematurity, immunodeficiency diseases and in infants
younger than 3 months.
• Etiology:
➢ Respiratory syncytial virus is the most common (80%).
o Protection against RSV is mediated by antibiotics of IgG3 subclass. These
antibodies have shorter half life and do not cross the placenta in substantial
amount so as to offer protection to the infant.
➢ Less common causes include parainfluenza, influenza, adenovirus, human
metapneumovirus, rhinovirus, and Mycoplasma pneumoniae (Rare).
• Dual infection with RSV and human metapneumovirus is associated with severe
bronchiolitis.
PATHOGENESIS
• The inflammation caused by viral infection of bronchiolar mucosa leads to
edema, thickening, formation of mucus plugs and cellular debris.
• Bronchiolar spasm occurs in some cases.
• The bronchial lumen which is already narrow in the infants is further reduced.
➢ Recall: airway resistance is inversely related to the 4 th power of the radius so
even slight narrowing of the bronchiolar lumen causes marked increase in the
airway resistance both during inspiration and expiration.
• During expiration, the bronchioles are partially collapsed and egress of air from
the lungs is severely restricted resulting in trapping of air inside the alveoli
causing emphysematous changes.
• When obstruction becomes complete the trapped air in the lungs may be absorbed
causing atelectasis.
• Due to diminished ventilation and diffusion, hypoxemia is produced in almost all
of these infants. (ventilation perfusion mismatch)
• Retention of carbon dioxide leads to respiratory acidosis.
• The presence of eosinophils in the blood and respiratory secretions suggest that
the virus infection initiates the wheezing attack in a child who is already
sensitized.

52
CLINICAL FEATURES
• Onset is gradual with upper respiratory symptoms such as rhinorrhea, nasal
congestion, fever and cough occurring initially.
• A few days following an upper respiratory tract infection breathing becomes fast
and respiratory distress develops.
• Paroxysmal wheezing- common but may be absent, cough and dyspnea are
present.
• On auscultation fine crackles can be heard
• The spleen and liver may appear enlarged as a result of lung hyperinflation. There
is also increase in anteroposterior diameter of the chest and resonance on
percussion due to hyperinflation.
• Apneic spells may be present and these should be monitored especially in young
infants and in children with a history of apnea of prematurity.
• Majority of infants have mild symptoms and recover in 3-7 days.
• Those with severe disease may develop retraction of lower intercostal spaces and
suprasternal notch by 3-5 days.
• In severe infection infant is dyspneic and may appear cyanosed. The fever is
moderately high. Accessory muscles of respiration are working. Expiration is
prolonged, fine crepitations and rhonchi are auscultated.
• Breath sounds may be faint or inaudible in severe cases.
• Hypoxemia may occur.

INVESTIGATIONS
• CXR reveals:
➢ Hyperinflation with air trapping
➢ Patchy infiltrates
➢ Focal Atelectasis

53
 ➢ Diaphragm is pushed down
➢ Lung fields appear abnormally translucent.

Figure 10: Chest X-ray. Bronchiolitis showing hyperinflation (Image adapted from Essential Pediatrics 8th
Ed)

Figure 11: Bronchiolitis with air trapping and right apical pneumothorax (arrow). Additional findings
include bilateral parahilar infiltrates and atelectasis (Image adapted from Hutchinsons Pediatrics)

• Full blood count: Leukocyte count is normal or slightly elevated.

• Viral antigen/ antibody testing.
• PCR on nasopharyngeal aspirates.

54
DIAGNOSIS
• Made on the basis of clinical features (Clinical diagnosis) and may be confirmed
with viral antigen/ antibody testing or PCR on nasopharyngeal aspirate.
• Bronchiolitis is generally a self-limiting illness.
• Symptoms subside in 3-7 days.
• Death may occur in 1% of the severely ill patients due to respiratory failure.
• The relationship between acute bronchiolitis to bronchial asthma later in life is
observed in about 25%.
➢ Symptoms of asthma can be identical to bronchiolitis. Suspect asthma if:
o Family history of asthma
o Prior episodes
o Responds to bronchodilators
MANAGEMENT
• Treat is essentially symptomatic.
➢ Admission, Oxygenation and position (30-400)
➢ Nasal bulb suction
➢ Hydration
• Mild disease can be managed at home in a humidified atmosphere.
• Admission (low threshold for hospitalization for high-risk infant)
➢ Hospitalization is indicated for respiratory distress, hypoxemia, apnea,
dehydration or underlying cardiopulmonary disease.
• Primarily supportive with nasal bulb suctioning, hydration and oxygen as needed.
• Careful handwashing to prevent spread of infection is necessary.
• Sitting position at angle 30 to 40 degrees with head and neck elevated.
• Moist oxygen is given continuously even in the absence of cyanosis. (keep
oxygen saturation more than 92%).
➢ Very sick infants may need a concentration of 60% oxygen given though
hood.
➢ Continuous positive airway pressure (CPAP) or assisted ventilation may be
required to control respiratory failure.
➢ Extracorporeal membrane oxygenation is effective, if respiratory failure is not
controlled by mechanical ventilation.
• Fluids and electrolyte balance should be maintained.
• Nebulized bronchodilators (beta-2 adrenergic drugs and ipratropium) are
controversial and may only be effective in up to 50%.

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 ➢ Trial of nebulized albuterol.
➢ They are not recommended for infants less than 6 months.
➢ A recent review shows beneficial effect of inhaled salbutamol with
ipratropium and epinephrine
➢ If a patient shows improvement, the bronchodilators may be given every 4-6
hourly.
• Steroids are controversial and may be most effective in patients with a prior
history of wheezing.
• Nebulized racemic epinephrine may be effective in reducing airway constriction.
• Aerosolized ribavirin a nucleoside analog with in vitro activity against RSV may
be considered for very ill infants. Evidence of definitive benefit is lacking.
• Antibiotics have no role. They may shorten the course of illness in infants with
underlying congenital heart disease, chronic lung disease and immunodeficiency.
• RSV monoclonal antibody (palivizumab) may be given prophylactically by
monthly intramuscular injection during RSV season to prevent severe disease in
infants with a history of prematurity, chronic lung disease or cyanotic or
hemodynamically significant congenital heart disease.
DIFFERENTIAL DIAGNOSIS
• Bacterial pneumonia
➢ Signs of obstruction in pneumonia are less pronounced, fever is high and
adventitious sounds in lungs are prominent.
• Foreign bodies in trachea
➢ Diagnosed by the history of aspiration of foreign body, localized wheeze and
signs of collapse or localized obstructive emphysema.
• Bronchial asthma
➢ Bronchiolitis is often confused with bronchial asthma.
➢ Bronchial asthma is unusual below the age of 1 year, a family history of
asthma is usually present.
• Congestive heart failure
➢ Suggested if there is cardiomegaly on X-ray, tachycardia, large tender liver,
raised JVP, edema and rales on auscultation of the lungs.

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COMPLICATIONS
• Superadded bacterial pneumonia
• Bacteremia
• Dehydration
• Respiratory failure
• Pericarditis
• Cellulitis
• Empyema
• Meningitis
• Suppurative arthritis

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 PNEUMONIA

PNEUMONIA
• Pneumonia is an acute inflammation of the lung parenchyma distal to the terminal
bronchioles i.e. respiratory bronchioles, alveolar ducts, alveolar spaces and
interstitial tissue.
• Pneumonia is infection and inflammation of lung parenchyma.
• Pathologically, there is a consolidation of alveoli or infiltration of the interstitial
tissue with inflammatory cells or both.
CLASSIFICATION OF PNEUMONIA
• Pneumonia can be classified according to:
➢ Etiology
➢ Setting in which it is contracted
➢ Age group affected
➢ Morphologically/anatomically/radiologically
ETIOLOGICAL CLASSIFICATION
• These are divided into:
➢ Infectious:
o Viral (most common cause of pneumonia in all age groups)
▪ Respiratory syncytial virus
▪ Influenza virus (A and B)
▪ Para-influenza
▪ Adenovirus
▪ Astrovirus
▪ Calicivirus
▪ Coxsackie virus
▪ Coronavirus
o Bacterial (less common but severe)
▪ Streptococcus pneumoniae (often causes lobar pneumonia)
▪ Haemophilus influenza type b
▪ Mycoplasma pneumoniae
▪ Group B Streptococcus (in newborns)
▪ Staphylococcus aureus
▪ Klebsiella pneumoniae
▪ Legionella pneumophilia

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 ▪ Chlamydophila pneumonia
▪ Coxiella burnetti (Q fever)
▪ Listeria monocytogenes
▪ E. coli
o Fungal
▪ Pneumocystis jirovercii (PCP)
▪ Histoplasmosis
▪ Coccidiodomycosis
➢ Non-infection:
o Aspiration of food
o Oily nose drops
o Liquid paraffin
o Kerosene poisoning
SETTING IN WHICH PNEUMONIA IS ACQUIRED
• Pneumonia can either be
➢ Community acquired pneumonia: with no underlying immunosuppression
o Acquired outside the hospital setting
o In younger children, viral organisms are the common causative agents.
o Streptococcal pneumoniae is the most likely cause in older children.
o Mycoplasma pneumoniae should be considered in school going children
and adolescents.
➢ Hospital acquired pneumonia (nosocomial infection).
➢ Pneumonia in the immunocompromised e.g. in HIV infected children bacteria
pneumonias are most common. Fungal pneumonias (PCP/PJP, histoplasmosis
and coccidioidomycosis) may be seen in immunocompromised children.
CLASSIFICATION ACCORDING TO AGE GROUP
• 0-3 months:
➢ Congenital infections: Toxoplasma, syphilis, rubella, CMV, herpes simplex
virus and tuberculosis
➢ Intrapartum acquired infections: Group B streptococcus (most common
cause), Gam negative rods (Klebsiella, E. coli), gram positive organism
(pneumococci and staphylococci) and Listeria monocytogenes
➢ Postpartum infections: RSV and other respiratory viruses
➢ Afebrile pneumonitis: Chlamydia trachomatis, Ureaplasma urealyticum,
Mycoplasma hominis, CMV and PCP
• 3 months-5 years:

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 ➢ Viruses: adenovirus, influenza A and B, parainfluenza and RSV (note: RSV
pneumonia is uncommon beyond 2-3 years)
➢ Bacteria: Streptococcus pneumonia (most common), Staphylococcus aureus
and Hemophilus influenzae type B
• Age 6 and older:
➢ Bacteria: Streptococcus pneumoniae (most common), Staphylococcus,
Mycoplasma pneumonia and Chlamydia pneumoniae are becoming common
➢ Viruses: adenovirus, influenza A and B, and parainfluenza
• Gram negative organism cause pneumonia in early infancy, severe malnutrition
and immunocompromised children.
• The most common causes of pneumonia in children under 5 years are:
➢ Viruses
➢ Pneumococci
➢ Haemophilus influenza
• The most common causes of pneumonia in children over 5 years and adults are:
➢ Viruses
➢ Pneumococci
➢ Mycoplasma pnemoniae (especially in school going children and adolescents)
RADIOLOGICAL/MORPHOLOGICAL/ANATOMICAL
CLASSIFICATION
• Pneumonia can be classified as:
➢ Lobar pneumonia: involves the entire lobe
➢ Segmental/lobular pneumonia: involves part of the lobe
➢ Bronchopneumonia: involving the alveoli adjacent to the bronchi
➢ Interstitial pneumonia: involving the interstitial tissue
RISK FACTORS
• Immunosuppression: malnutrition and HIV infection
• Prematurity, low birth weight, lack of breast feeding, young age, Vitamin A
deficiency
• Social-economic factors:
➢ Poverty,
➢ Crowding
➢ Exposure to tobacco smoke (passive smoking)
➢ Urban residence, indoor air pollution
➢ Multiple siblings (large family size)

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• Respiratory pathologies:
➢ Family history of bronchitis
➢ Congenital anomaly of the bronchi
➢ Inhaled foreign body
➢ Recurrent aspiration (e.g. with a trachea-esophageal fistula)
➢ Cystic fibrosis
CLINICAL FEATURES
• Onset of pneumonia may be insidious starting with upper respiratory tract
infection or may be acute with high fever, dyspnea and grunting respiration.
➢ Rarely pneumonia may present with symptoms of acute abdominal emergency
this is attributed to referred pain from the pleura.
➢ Apical pneumonia may be associated with meningismus and convulsions. In
these patients CSF is always clear.
• Acute pneumonia usually presents with a short history of fever (>38.5oC), cough,
tachycardia, crackle and signs of respiratory distress including tachypnea (most
reliable sign), dyspnea and subcostal and intercostal recession.
➢ Grunting, fever and feeding difficulties are common findings in infants.
➢ Productive cough, and chest pain are seen in older children.
➢ Pneumonia should be suspected in all children who present with cough or
difficulty breathing (shortness of breath)
• Signs include dullness to percussion, bronchial breathing (Expiration and
inspiration of the same duration and intensity) and crackles reflecting the
underlying consolidation.
• The cut-off points for fast breathing vary with age:
➢ <2 months: 60 breaths/min or more
➢ 2 months-1 year: 50 breaths/min or more
➢ 1 year-5 years: 40 breaths/min or more
• Clinical signs are often not reliable in infants and the diagnosis should always be
confirmed by Chest X-ray (which may either indicate lobar pneumonia or a more
widespread bronchopneumonia)
• Signs of serious illness (in a child under 5 who is either resting/ sleeping) are:
➢ Chest in-drawings
➢ RR> 60b/m in children under 2 months, >50b/min in children between 2-
12months and >40b/min in children between 1-5 years
➢ Cyanosis (examine the lips, buccal membranes and fingernails)
➢ Nasal flaring

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 ➢ The child refuses to drink to breastfeed.
➢ The child is abnormally sleepy or difficult to wake
➢ Stridor (hoarse noise on inspiration)
➢ Grunting (a short repetitive noise produced by a partial closure of the vocal
cords) on expiration
➢ Severe malnutrition
INDICATIONS FOR ADMISSION
• Infants:
➢ Cyanosis, Oxygen saturation <92%
➢ Increased respiratory rate
➢ Subcostal recession
➢ Intermittent apnea, grunting
➢ Poor feeding
• Older children:
➢ Cyanosis, Oxygen saturation <92%
➢ Increased respiratory rate
➢ Subcostal recession
➢ Restlessness or agitation
➢ Signs of dehydration
INVESTIGATIONS
• Bloods:
➢ FBC, ESR
➢ Urea, electrolytes and creatinine
➢ Blood culture (positive in 10-30% of bacterial cases)
➢ Arterial blood gases (if available)
• Imaging:
➢ Chest X-ray
o Viral (hyperinflation, perihilar infiltration, peribronchial infiltration, hilar
adenopathy and atelectasis)
o Bacterial (alveolar consolidation)
o Mycoplasma (interstitial infiltrates)
o Tuberculosis (hilar adenopathy)
o Pneumocystis (Reticulonodular infiltrates)

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MANAGEMENT
• General measures:
➢ Oxygen by nasal cannula or mask
➢ IV fluids if required should be <2/3 of requirements (risk of SIADH in
hypoxic children)
➢ Antipyretic and analgesia as indicated
➢ Monitor vital signs
• Antibiotics therapy:
➢ 0-3 months: Benzyl penicillin 50, 000IU/Kg/dose QID and gentamicin 7.5mg
OD or in divide 2 doses.
➢ >3 months: Benzyl penicillin 50, 000IU/kg/dose QID
➢ Ceftriaxone (50mg/kg/dose OD) or cefotaxime (50mg/kg QID) should be
considered if no improvement within 48 hours
➢ In infants with HIV infection or exposure, PCP therapy with high dose IV/PO
cotrimoxazole (20mg/kg/day of trimethoprim) should be included
➢ Macrolides should be considered in school going children and adolescents
who do not improve on 1st line
o Erythromycin
▪ 1 month-2 years: 125mg QID
▪ 2-8 years: 250mg QID
▪ 8-18 years: 250-500mg QID
o Azithromycin
▪ 6 months-2 years: 10mg/kg OD
▪ 3-7 years: 200mg
▪ 8-11 years: 300mg
▪ 12-14 years: 400mg
▪ >14 years: 500mg
COMPLICATIONS
• Pleural effusion
• Septicemia
• Bronchiectasis
• Empyema thoracis
• Lung abscess (following staphylococcal pneumonia)

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VIRAL PNEUMONIA
• RSV is the most important cause in infants under 6 months. At other ages,
influenza, parainfluenza and adenoviruses are common.
• The bronchial tree or alveoli are involved resulting in extensive interstitial
pneumonia
• Symptoms often begin with upper respiratory complaints e.g. nasal congestion
and rhinorrhea. Fever, cough, tachypnea and dyspnea typically follow.
• Physical exam may demonstrate tachypnea, wheezing, rales or respiratory
distress.
• A child tachypnea if
➢ RR> 60b/m in children under 2 months.
➢ RR>50b/m in children from 2 to 1 year
➢ RR> 40b/m in children from 1 year to 5 years
• Diagnosis is suggested by interstitial infiltrates, hyperinflation, perihilar &
peribronchial infiltration, hilar adenopathy and atelectasis on CXR and a WBC
count < 20, 000 cells/mm3 with lymphocyte predominance. Features of
consolidation are usually not present.
• Management is supportive.
BACTERIAL PNEUMONIA
PNEUMOCOCCAL PNEUMONIA
• It is due to S. pneumoniae. It transmitted by droplets and is more common in
winter months.
• Overcrowding and diminished host resistance predisposes the child to infection.
• Bacteria multiple in the alveoli and an inflammatory exudate is formed. Scattered
areas of consolidation occur, which coalesce around bronchi and later become
lobular or lobar in distribution. There is no tissue necrosis.
• Pathological stages:
➢ Congestion: due to congested vessels and edema.
➢ Red hepatization: due to exudate, neutrophils and hemorrhage filling the
alveolar air spaces giving the normally spongy lung a solid consistency. There
is a firm, ‘meaty’ and airless appearance of the lung (it looks like a liver)
➢ Gray hepatization: due to macrophage mediated degradation of red cells and
fibrin within the exudate. There is less hyperemia.
➢ Resolution: Lysis and removal of fibrin via lymphatics and sputum.

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• Clinical features:
➢ Incubation period: 1-3 days
➢ Abrupt onset of headache, chills, cough and high fever.
➢ Cough is initially dry but may be associated with rusty sputum.
➢ Child may develop pleuritic chest pain.
➢ Respiration is rapid
➢ In severe cases: grunting, chest indrawing, difficulty feeding and cyanosis
may be seen.
➢ Dullness to percussion, reduced air entry, crepitation and bronchial breathing
may be heard over consolidation. Bronchophony and whispering pectoriloquy
may also be observes
➢ Meningismus may be present with apical pneumonia
• Diagnosis is suggested by a
➢ History and physical examination findings
➢ Chest X-ray findings of lobar consolidation
➢ WBC count> 20, 000 cells/mm3 with a neutrophil.
• Bacteriological confirmation is difficult, sputum is examined by gram staining
and blood cultures are positive only in about 5-10% of cases.
• Management includes appropriate antibiotics and supportive care.
➢ Drug of choice is penicillin (penicillin V 250mg BD-TDS orally, penicillin G
0.5MU/Kg/day IV or procaine penicillin 0.6MU IM daily for 7 days),
amoxycillin (30-40mg/kg/day for 7 days with or without clavulanic acid).
➢ 1 to 3 months old: erythromycin or cefuroxime
➢ 3 months to 5 years old: ampicillin or cefuroxime
➢ 5 years: erythromycin or cefuroxime
➢ The need for oxygen is guided by signs of respiratory distress (Rapid
breathing, chest retractions, nasal flare), presence of cyanosis or hypoxemia
or pulse oximetry.
➢ Patients may be dehydrated and require IV fluid. Fever should be managed
with paracetamol
STAPHYLOCOCCAL PNEUMONIA
•
CHLAMYDIA TRACHOMATIS
• Chlamydia trachomatis is a common cause of afebrile pneumonia at 1-3 months
of age (pneumonitis syndrome).

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• Symptoms: staccato-type cough, dyspnea and absence of fever. A history of
conjunctivitis after birth may be identified in 50% of patients.
• Physical examination may demonstrate tachypnea and wheezing.
• Diagnosis is suggested by eosinophilia and CXR with interstitial infiltrates.
• Definitive diagnosis is by positive culture or direct fluorescent antibody (DFA)
staining of cells from conjunctiva or nasopharynx.
MYCOPLASMA PNEUMONIA
• Mycoplasma pneumonia: common cause of pneumonia in older children and
adolescents.
• Symptoms: low-grade fever, chills, nonproductive cough, headache, pharyngitis
and malaise. The cough may last 3-4 weeks.
• Lung examination demonstrates widespread rales. Examination findings are
often worse than expected by history.
• Diagnosis:
➢ Positive cold agglutinins are suggestive but nonspecific.
➢ CXR findings vary but may show bilateral diffuse infiltrates
➢ Definitive diagnosis is by elevation of serum IgM titers for Mycoplasma.
• Management includes oral erythromycin or azithromycin.

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 PERTUSIS

PERTUSIS
• Pertussis (Latin= “Intense cough”) is an acute respiratory infection also known
as “Whooping cough”.
• It is caused by the bacterium Bordetella pertussis.
➢ It is a tiny, gram-negative cocco-bacilli
• B. parapertussis causes illness that appears clinically very similar to pertussis.
• Transmission is by direct inhalation of microdroplets spread by infected patients.
• Routine immunization beginning at 2 months of age has been effective in
reducing the overall incidence of pertussis infection.
• Universal vaccination is recommended at 2, 3 and 4 months of age.
➢ Combined diphtheria-tetanus-pertussis vaccine are given from the age of 6
weeks, 3 doses of 0.5 ml at 4 weeks intervals; booster dose 1 year after the 3 rd
dose.
• Infants younger than 6 months of age are most at risk for severe disease.
• Adolescents and adults whose immunity has waned are the major source for
pertussis infection of unimmunized or underimmunized children.
• Infection is highly contagious.
• Placental transfer of antibody does not protect young infants passively.
PATHOGENESIS
• B. pertussis produces a pertussis toxin as well as few other biological active
substances.
• These are responsible for various inflammatory changes with pertussis toxin
playing a central role.
• The mucosal lining of the respiratory tract is inflamed with necrosis and
desquamation of epithelial cells leading to obstruction, atelectasis and
accumulation of secretion.
• The resultant hypoxia can affect liver and brain also.
CLINICAL FEATURES
• Incubation period is typically 7-10 days.
• Pertussis is characterized by 3 stages:

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 ➢ Catarrhal stage (lasts 1-2 weeks): characterized by upper respiratory
symptoms such as rhinorrhea, nasal congestion, conjunctival redness and low-
grade fever
➢ Paroxysmal stage (lasts 3-4 weeks)
o Characterized by fits of forceful coughing (“paroxysms”) that are the
hallmark of pertussis.
o A whoop is an inspiratory gasp against a partially closed glottis heard at
the very end of a coughing fit (the whoop is heard rarely in young infants
because they cannot generate enough pressures in their respiratory
passage).
o The coughing fits are exhausting and post-tussive vomiting is common.
o Young infants may have cyanosis, apnea and choking during the
paroxysms of cough.
o Between the fits, children appear well and are afebrile
➢ Convalescent phase (lasts weeks to months): recovery stage in which
paroxysmal cough continues but becomes less frequent and less severe over
time.
o The cough can persist for some time and hence the disease has also been
called ‘Cough of 100 days’.
DIAGNOSIS
• Diagnosis is suspected based on clinical features.
• WBC are elevated with a lymphocytosis.
• Low ESR
• Diagnosis is confirmed by identification of organisms on culture (gold standard)
of nasopharyngeal secretions plated on Regan-Lowe or Bordet-Gengou media,
or by positive direct fluorescent antibody tests of nasopharyngeal secretions or
PCR on nasopharyngeal swab.
COMPLICATIONS
• Respiratory system is the site for most complication.
➢ Secondary bacterial infections
➢ Otitis media
➢ Emphysema
➢ Air leaks- pneumothorax or pneumomediastinum
➢ Subcutaneous emphysema
• During paroxysmal stage serious CNS complications:

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 ➢ Seizures
➢ Encephalopathy
• Complications associated with severe cough:
➢ Subconjunctival hemorrhage (raised pressure in various blood vessels)
➢ Retinal hemorrhage
➢ Epistaxis
➢ Intracranial hemorrhage
➢ Inguinal hernia (increased intra-abdominal pressure)
➢ Rectal prolapse
➢ Diaphragmatic rupture (rare)
• Due to protracted course:
➢ Vomiting
➢ Poor feeding
➢ Malnutrition
➢ Flaring of underlying tuberculosis can also occur
DIFFERENTIAL DIAGNOSIS
• Viral infection e.g. Adenovirus, influenza, RSV
• Mycoplasma infection
• Foreign body aspiration
• Endobronchial TB
MANAGEMENT
• Hospitalization of young infants often occurs during the paroxysmal phase
because of choking, apnea, or cyanosis.
• Supportive care and oxygen (if needed) are important therapies.
• A nebulized mist and/or salbutamol can be helpful in some patients.
• Antibiotics are given to all patients to prevent the spread of infection
(azithromycin or erythromycin is used).
➢ Antibiotics do not alter the patient’s clinical course unless they are
administered during the catarrhal phase or very early in the paroxysmal phase.
• All patients need to be isolated till they have received at least 5 days of
antibiotics.
• Maintain nutrition: small, frequent, easily swallowable and caloric dense
foodstuffs should be given. Forced feeding should be avoided. Feeds are better
given soon after a bout of coughing.

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• All contacts irrespective of symptoms, age and immunization status should be
given antibiotics for 2 weeks.
• For unimmunizaed or incompletely immunized contacts, the schedule should be
completed.
• Those whose have received a vaccine dose >6 months back should receive a
booster.

NON-INFECTIOUS DISEASES
BRONCHIAL ASTHMA (REACTIVE AIRWAY DISEASE)
• This is a chronic inflammatory disorder of the airways associated with airway
hyperresponsiveness that leads to recurrent persistent episodes of wheezing,
breathlessness, cough, dyspnea and chest tightness.
• Symptoms are typically associated with widespread, variable airflow obstruction
that is at least partially reversible either spontaneously or with therapy.
• Inflammation causes airway hyper-responsiveness to many stimuli.
• Asthma is the most common chronic pediatric disease.
• 50% of children have symptoms by 1 year and 90% by 5 years of age.
• 30-50% have remission by puberty.

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ETIOLOGY
• The 2 main causes of asthma symptoms are:
➢ Hyperresponsiveness: increased tendency of the airway to react to stimuli or
triggers to cause an asthma attack
➢ Bronchoconstriction: narrowing of the airways that causes airflow obstruction
• Predisposing factors:
➢ Atopy, family history of asthma and exposure to tobacco smoke.
➢ Infection, diet and pollution increase susceptibility in predisposed patients.
• Trigger factors:
➢ Respiratory infection e.g. especially viral infections: they interfere with the
integrity of mucosal surfaces by opening up tight intraepithelial cell junctions
and induce shedding of epithelium
➢ Exercise (in older children): in genetically susceptible individuals, lose of
water from the respiratory tract induces mucosal hyperosmolarity which
stimulates mediator release from mast cells
➢ Weather: Cold air, loss of water and heat from the lower airways and sudden
release of airborne allergens in atmosphere
➢ Emotions: increased vagus output causing bronchial smooth muscle spasms.
➢ Allergens: seasonal allergens e.g. pollen, green and non-seasonal animal
feathers, dust mites and molds as well as food proteins or additives; peanuts
are a good example
➢ Gastroesophageal reflux
➢ Exposure to pollutants
➢ Endocrine factors: some endocrine changes including puberty may increase
symptoms of asthma
• Asthma may accompany other acute or chronic lung diseases such as cystic
fibrosis.
CLASSIFICATION
• Asthma has can be classified as:
➢ Extrinsic asthma:
o Occurs in atopic individuals (i.e. individuals with positive skin-prick
reaction to common inhaled allergens e.g. dust mite, animal danders,
pollens and fungi)
o 90% of children with persistent asthma have positive skin prick test.
o It may be associated with eczema (atopic dermatitis) in childhood.
➢ Intrinsic asthma

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 o Common at an older age (middle age)
o Causes include:
▪ Intolerance to NSAIDs e.g. aspirin or because of beta blockers
▪ Viral infections that cause epithelial damage and mucosal
inflammation
▪ Emotional upset: mediates excess parasympathetic input
▪ Exercise: water and heat are lost from airways
▪ Cold weather
o These causes should be avoided in asthmatic patients
• Previously it was classified as:
➢ Atopic/Allergic
o IgE mediated
o Triggered by allergens
➢ Non-atopic
o Non IgE mediated
o Triggered by infection
➢ Mixed

PATHOPHYSIOLOGY
• The hallmark of asthma (extrinsic) is an IgE mediated type 1 hypersensitivity
reaction.
• Exposure to an antigen or allergen leads to sensitization and production of IgM.
With subsequent exposure to the same antigen or allergen IgE is produced.
• IgE via its Fc portion can bind to the surface of mast cells and basophils via Fc
receptors (FcR1).
• Cross-linkage of adjacent bound antibodies provides the signal to cause mast cell
degranulation (histamine and heparin are released) and release of chemical
mediators of inflammation resulting in vasodilation, increased pulmonary
capillary permeability, viscous mucus production and severe constriction of
bronchioles.
• In an acute attack there are 2 phases:
➢ Early phase:
o Characterized by bronchospasm

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 o Starts withing 10 minutes of exposure to allergen and lasts 2 to 3 hours
o It is due to interaction of mast cells and trigger leading to release of
preformed mediators of inflammation, mucosal edema, bronchospasm and
increased mucus production all which manifest as airway obstruction.
o Mediators include: histamine, heparin, leukotrienes, prostaglandins,
platelet activating factor and bradykinin.
o This phase is inhibited by beta-2 agonists.
➢ Late phase:
o Associated with inflammation
o Develops 3-4 hours later and lasts 8 to 12 hours
o It is due to infiltration of T lymphocytes, monocytes and eosinophils. There
is airway hyperactivity
o This phase presents as clinical asthma.
o The release of mast cell mediators is inhibited by premedication with
steroids suggesting that airway narrowing is mainly due to an
inflammatory reaction and mucosal edema.
• Airway obstruction in asthma is caused by:
➢ Vascular congestion, edema and inflammation of mucous membrane lining
airways
➢ Excessive secretion of mucus, inflammatory cells and cellular debris
➢ Spasms of the smooth muscle of the bronchi (bronchoconstriction)
➢ Injury and desquamation of the airway epithelium and impaired muco-ciliary
transport
• The obstruction is diffuse but not uniform.
• Airway resistance is increased more during exhalation because airways close
prematurely.
• Lungs are hyperinflated; elasticity and frequency dependent compliance of the
lungs is reduced, breathing involves more work resulting in dyspnea
• Perfusion of inadequately ventilated lungs causes hypoxemia.
• In the early stages of the partial pressure of carbon dioxide falls because of
hyperventilation however, when obstruction becomes more severe, alveolar
hypoventilation supervenes leading to hypercapnia and respiratory acidosis.
• In chronic asthma there is airway remodeling.

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 74

CLINICAL FEATURES
• Clinical features are variable
• Acute asthma may usually begin with a cold, or bouts of spasmodic coughing. In
early phase the cough is non-productive. The patient becomes dyspneic, with
prolonged expiration and wheezing. Use of accessory muscles of respiration,
profuse sweating, cyanosis, apprehension, restlessness and fatigue may be seen.
• In severe episodes the child may show air hunger.
• Typical features during an exacerbation:
➢ Tachypnea
➢ Dyspnea
➢ Nasal flaring
➢ Retractions
➢ Multiphonic (multiple pitch) wheezing with a prolonged expiratory phase.
 o This is believed to be due to many airways of different dimensions
vibrating from abnormal narrowing.
o Asthma should be suspected in any child with wheezing on more than one
occasion.
o Although it may be clear to most clinicians what ‘wheezing’ is, patients
and parents do not always mean the same thing. It is best to describe the
sound to a parent (e.g. ‘a whistling in the chest when your child breaths
out’) and ask if that fits with the child’s symptoms.
o The presence of wheeze is confirmed on auscultation by a health
professional to distinguish it from transmitted upper respiratory noises.
• Some patients have only chronic or recurrent cough.
• Symptoms usually worsen at night and in the early morning.
• They may have triggers e.g. exercise, pets, dust, cold air, emotions, laughter.
• There may be a personal or family history of an atopic disease and a positive
response to asthma therapy.
• On examination
➢ Onset of the disease in early childhood may result
in Harrison sulci as well as a barrel shaped chest.
➢ Chest is hyper-resonant because of excessive air
trapping
➢ Generalized polyphonic expiratory wheezing is
present and this is an ominous sign. Absence of
wheezing in the presence of cyanosis and
respiratory distress should not be considered as an
evidence of clinical improvement. As child improves the airflow increases and
wheezing may reappear, with remission wheezing disappears.
➢ There is a prolonged expiratory phase.
➢ Breath sound may become feeble due to severe obstruction and airflow
decrease.
• Severe hypoxemia in asthma results in cyanosis and cardiac arrhythmias. Pulsus
paradoxus indicates severe illness. Finger clubbing is unusual.
• Growth should be plotted but is normal unless the asthma is extremely severe.
• The presence of a wet cough or sputum production, finger clubbing or poor
growth may suggest chronic conditions such as cystic fibrosis or bronchiectasis.

75
APPROACH TO THE PATIENT
• Always treat the asthmatic symptoms first when patient is received as asthma is
a life-threatening condition then once patient has stabilized take a history,
perform physical examination and formulate a definitive management plan.
HISTORY
• Once suspected, the pattern or phenotype should be further explored by asking:
➢ How frequent are the symptoms?
➢ What triggers the symptoms? Specifically, are sport and general activities
affected by the asthma?
➢ How often is sleep disturbed by asthma?
➢ How severe are the interval symptoms between exacerbations?
➢ How much school has been missed due to asthma?
PHYSICAL EXAMINTION
• As discussed above
DIAGNOSIS AND INVESTIGATIONS
• Diagnosis is mostly clinical.
• Recurrent attacks of wheezing indicate bronchial asthma.
• Note: “all that wheezes is not asthma”.
• Although intermittent attacks of coughing may be due to recurrent viral
infections, the diagnosis of asthma should be considered. The cough associated
with asthma tends to worsen after exercise.
• Sputum is generally clear and mucoid but expectoration of yellowish sputum
(Attributed to large number of eosinophils) does not exclude the diagnosis of
asthma.
• Chronic spasmodic cough may suggest occult asthma
• Investigations (usually have an important role in diagnosis of doubtful cases and
monitoring response to treatment)
➢ Pulmonary function tests (spirometry)
o PFTs reveal increased lung volumes and decreased expiratory flow rates.
o Peak expiratory flow rate (PEFR): reduced, this can be measured with peak
expiratory flow meter unlike parameters below that are measured by
spirometry. Abnormality in PEFR suggestive of asthma include:
▪ Diurnal variation of more than 20%
▪ ≤80% of predicted and improvement of ≥20% after bronchodilator
therapy.

76
 o Forced expiratory volume in 1 second (FEV1): reduced, this is used to
document severity of asthma.
o Forced vital capacity (FVC): reduced
o FEV 25-75: reduced, this is effort independent and is probably more
sensitive indicator of airway obstruction.
o FEV1/FVC ratio <0.7 confirms obstructive airway disease.
➢ CXR shows:
o Hyperinflation (bilateral and symmetric)
o Peribronchial thickening, Peribronchial cuffing may occur due to the
presence of edema fluid in perivascular and peribronchial interstitial space.
o Main pulmonary artery is prominent due to pulmonary hypertension.
o Patchy atelectasis due to unusual mucus plugs
o Note: chest X-ray film may often be normal. Extensive areas of collapse
or consolidation should suggest an alternative diagnosis.
➢ Allergy test: Skin test and radioallergosorbent allergen specific IgE (RAST)
have limited usefulness. Few children need skin test to identify sensitivity to
different antigens since the role of desensitization therapy is not established.

77
 78

DIFFERENTIAL DIAGNOSIS
DIFFERENTIAL DIAGNOSIS DETAILS
BRONCHIOLITIS •
CONGENITAL MALFORMATION
ASPIRATION OF FOREIGN BODY
HYPERSENSITIVITY
PNEUMONITIS
CYSTIC FIBROSIS

MANAGEMENT
• Treatment depends on the severity of illness and trigger factors.
• Self-management with a normal lifestyle is the primary goal.
• Control of asthma
➢ Assessment and monitoring of asthma symptoms.
➢ Control of trigger factors: avoidance of causal allergens, dust mites, molds,
animal dander, cockroaches, pollens, smoke, pollution and irritants.
➢ Patient and family education
➢ Pharmacologic therapy added in a stepwise fashion based on disease severity
o Sympathomimetics
▪ Beta 2 adrenergic agonists may be given by inhaler or by nebulization
 ▪ hort-acting bronchodilators e.g. albuterol are first line therapies for
exacerbation. They can also be used for prevention of exercised
induced symptoms.
▪ Long-acting preparations may be used for chronic control
▪ If asthma is persistent i.e. more severe than intermittent, it is more
effectively controlled by the addition of anti-inflammatory
medications.
o Cromolyn sodium and nedocromil sodium
▪ Anti-inflammatory prophylaxis is induced by inhibition of activation
and release of inflammatory mediators.
▪ Prevent degranulation of mast cells.
▪ These drugs have no effect on acute symptoms but may help prevent
exacerbations.
o Corticosteroids
▪ Most effective anti-inflammatory agents,
▪ Systemic steroids are given 5-10 days for moderate to severe
exacerbations.
▪ Inhaled steroids are very effective in preventing exacerbations.
o Anticholinergic agents (e.g. atropine or ipratropium bromide= Atrovent)
▪ Second-line bronchodilators
▪ Decrease airway vagal tone and block reflex bronchoconstriction and
they may be useful in severe exacerbation
o Leukotriene modifiers (motelukast, zafirlukast) are oral anti-inflammatory
agents for long-term control of mild, persistent asthma
o Methylxanthines (theophylline) play a controversial role in asthma
management. They are bronchodilators with possible anti-inflammatory
effects but have a narrow toxic-therapeutic ratio and their use has therefore
decreased.

79

CYSTIC FIBROSIS
• Cystic fibrosis is a multisystem
autosomal recessive disorder that
results in altered content of
exocrine gland secretions.
• The condition has equal incidence
in males and females.
• Median age of survival is 31-32
years.
PATHOPHYSIOLOGY
• The genetic defect (Long arm of
Chromosome 7) produces an
abnormal ion-channel regulator
protein known as cystic fibrosis
transmembrane conductance
regulator (CFTR) that causes
sodium and chloride transport
dysfunction in epithelial cells.
➢ CFTR is a cyclic AMP-
dependent chloride channel
found in the membrane of cells.
• The failure of the chloride
conductance by epithelial cells
leads to dehydration of secretions
that are too viscid and difficult to
clear.
• Abnormal mucus produced in
airways helps create airway
obstruction (through reduction in
the airway surface liquid layer and
consequent impaired ciliary
function), inflammation and
infection (especially with
Staphylococcus aureus and
Pseudomonas aeruginosa).
80
• In the intestine, thick viscid
meconium is produced leading to
meconium ileus in 10-20% of
infants.
• The pancreatic ducts also become
blocked by thick secretions leading
to pancreatic enzyme deficiency
and malabsorption.
• Abnormal function of the sweat
glands results in excessive
concentrations of sodium and
chloride in sweat.
CLINICAL FEATURES
• Classic hallmarks of CF:
➢ Chronic progressive pulmonary
insufficiency
➢ Pancreatic insufficiency
➢ High sweat electrolytes
• The features depend on age of
diagnosis and treatment received.
The common clinical presentation
includes meconium ileus in
neonatal period, recurrent
bronchiolitis in infancy and early
childhood, recurrent lower
respiratory tract infections, chronic
lung disease, bronchiectasis,
steatorrhea and with increasing age
pancreatitis and azoospermia.
Pancreatic insufficiency is present
in >85% of CF patients.
• Clinical expression is variable.
➢ Meconium ileus at birth is
present in 20%
o Inspissated meconium
causes intestinal obstruction
 with vomiting, abdominal
distension and failure to pass
meconium in the first few
days of life.
o Initial treatment is with
Gastrografin enemas but
most cases require surgery.
➢ Recurrent or chronic respiratory
symptoms, steatorrhea and
failure to thrive (FTT) are
typical presenting features.
➢ Pneumonia develops as lungs
become colonized first with
Staphylococcus aureus and later
with Pseudomonas aeruginosa.
➢ Common pulmonary
complications: hemoptysis,
pneumothorax, asthma, chronic
sinusitis and nasal polyps.
Recurrent pneumonia,
bronchiectasis, pulmonary
fibrosis, cor pulmonale and
respiratory failure eventually
occur.
➢ Pancreatic insufficiency occurs
in 90% of patients with
malabsorption predisposing to
malnutrition and FTT.
CHRONIC PROGRESSIVE
PULMONARY INSUFFICIENCY
• Persistent colonization or infection
with CF pathogens e.g.
Staphylococcus aureus,
Haemophilus influenzae,
Pseudomonas aeruginosa or
Burkholderia cepacia.
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• The child has a persistent loose
chronic cough, wheeze, purulent
sputum production
• On examination: hyperinflation of
the chest due to air trapping, coarse
inspiratory crepitations and/or
expiratory wheeze.
• Mucus plugging
• Nasal polyps and chronic sinusitis
• Chest radiography:
➢ Bronchiectasis
➢ Atelectasis
➢ Pulmonary infiltrates
➢ Hyperinflation
GASTROINTESTINAL
ABNORMALITIES
• Meconium ileus and distal
intestinal obstruction syndrome
• Rectal prolapse
• Pancreatic insufficiency (lipase,
amylase and proteases) and
recurrent pancreatitis
➢ Pancreatic insufficiency can be
diagnosed by demonstrating low
elastase in feces.
➢ Maldigestion and malabsorption
(passing frequent large, pale,
very offensive and greasy
stools-steatorrhea)
• Chronic hepatic disease
NUTRITIONAL ABNORMALITIES
• Failure to thrive
• Hypoproteinemia
• Edema
• Fat-soluble vitamin deficiencies
(A, D, E, K)
METABOLIC ABNORMALITIES
• Salt depletion
• Classic electrolytes: hyponatremic,
hypochloremic, hypokalemic
metabolic alkalosis
OTHER
• Finger clubbing
• Obstructive azoospermia

DIAGNOSIS
• Needs:
➢ One or more phenotypic
features or
➢ Positive family history or
➢ Increased immunoreactive
trypsinogen on newborn screen.
• Laboratory evidence of abnormal
CFTR:
➢ Sweat test: chloride >60mmol/L
on at least 2 occasions (normal
10-40mmol/L in normal
children)
o Sweating is stimulated by
pilocarpine iontroporesis
➢ Two CF mutations (confirmed
diagnosis)

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 ➢ Characteristic ion transport ➢ Good nutrition, pancreatic
abnormality across the nasal enzyme replacement and fat
epithelium soluble vitamin (Vitamins A, D,
E, and K)
MANAGEMENT ➢ Oxygen as needed for
• Treatment requires a team approach hypoxemia
and includes: ➢ Anti-inflammatory and
➢ Antibiotics for pulmonary immunosuppressive therapy
exacerbations ➢ Lung transplantation and
➢ Pulmonary toilet with chest ultimately future gene therapy
percussion and antimucous ➢ Psychological support for
therapy patients and families
➢ Bronchodilators for wheezing

83
84
85
CHAPTER 9: CARDIOVASCULAR CONDITIONS

CARDIOVASCULAR CONDITIONS
CONGENITAL HEART DISEASES
• These are a group of diseases involving malformations of the heart and major
blood vessels.
• In common usage, CHD refers to structural heart defects that are present at birth.
• They account for about 1% of all births but incidence is higher in premature
babies.
• There are 3 main categories of congenital heart diseases:
➢ Left to right shunts
➢ Right to left shunts
➢ Obstructive congenital heart disease
• History, physical examination, chest X-ray, ECG and echocardiography help in
identifying the presence of CHD, except perhaps in the early newborn period
where the diagnosis can be challenging.
• Palliative or corrective surgery is feasible for most patients with CHD, provided
if under-taken in a timely fashion.
• A substantial proportion of patients with CHD have significant problems
involving other organs system, or specific chromosomal and single gene
disorders.
• Many children need lifelong followup.
FETAL CIRCULATION
• In utero the fetal lungs are non-functional.
• Pulmonary vasculature and systemic vasculature respond differently to hypoxia
and oxygen
➢ Pulmonary vasculature vasoconstrict in response to hypoxia whilst systemic
blood vessels vasodilate in response to hypoxia.
➢ Pulmonary vasculature vasodilates in response to oxygen whilst systemic
blood vessels vasoconstrict in response to oxygen
• Vasoconstriction results in increased resistance
• Vasodilation results in decreased resistance

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• In utero pulmonary resistance is greater than systemic resistance because the
lungs are non-functional. When the child is born and the lung become functional
pulmonary resistance drops and it becomes less than systemic resistance.
• The fetal circulation is specially designed to bypass the non-functional lungs in
the fetus
• By the 3rd month of development all major blood vessels are present and
functioning.
• The heart assumes its normal four chambered shaped by the end of 6 weeks of
intrauterine life.
• The fetus must have blood flow to the placenta. (recall: the placental facilitates
gas and nutrient exchange between the maternal and fetal blood as they do not
mix)
• Resistance to blood flow is high in the lung. In utero pulmonary resistance is
greater than systemic resistance.
• Pair of umbilical arteries carry deoxygenated blood and waste to the placenta
• The umbilical vein carries oxygenated blood and nutrients from the placenta
towards the fetus.
• 2/3 of the blood from the umbilical vein enters the ductus venous (a shunt which
bypasses the liver and puts blood into the hepatic veins which drain into the
inferior vena cava, it joins the portal vein with the inferior vena cava) (the inferior
vena cava has oxygenated blood in utero).
• Some blood from the umbilical vein enters the portal circulation allowing the
liver to process the nutrients. This blood as well is drained into the inferior vena
cava.
• Blood from the inferior vena cava drains into the right atrium and the foramen
ovale shunts some this blood from the right to left atrium, skipping the lung).
More than 1/3 of the blood takes this route.
• Some of the blood passes through the tricuspid valve into the right ventricle and
it is pumped out of the ventricle passed the pulmonary valve through the
pulmonary trunk. Most of this blood is shunted into the aortic arch through the
ductus arteriosus (which connects the pulmonary trunk and the aortic arch).
• Blood then flows to the rest of the body and eventually reaching the umbilical
arteries completing the cycle.
• The shunting of blood passed the lung is made possible because in utero
pulmonary resistance is greater than systemic resistance.

87
• When the child is born and takes the first breath systemic resistance becomes
greater than pulmonary resistance and the shunts close.
• The foramen ovale closes shortly after birth, it fuses completely in the first year.
• The ductus arteriosus closes soon after birth (within 7 days) and becomes the
ligamentum arteriosum in about 3 months
• The ductus venosus closes and becomes the ligamentum venosum
• The umbilical arteries become the medial umbilical ligaments
• The umbilical vein becomes the ligamentum teres

Figure 12: Fetal circulation

• Defects in the fetal circulation or development of the heart lead to congenital

heart defects.
• These are the most common causes of heart disease in children (8 in every 1000
births).
• They range from asymptomatic murmurs to severe CHF immediately after birth.
• They arise during embryogenesis (usually between 3-8 weeks of gestation).
CHDs result in shunting between the left (systemic) and right (pulmonary)
circulations.

88
• Recall:
➢ The right-side flow: low pressure, low oxygen
➢ The left side flow: high pressure, high oxygen
➢ Mixture of the two results in changes in the oxygen content of blood and
causes symptoms
➢ Defects with left to right shunting may be relatively asymptomatic at birth but
the shunt can eventually reverse (Eisenmenger syndrome)
➢ Increased flow through the pulmonary circulation results in hypertrophy of
pulmonary vessels and pulmonary hypertension
➢ Increased pulmonary resistance eventually results in reversal of shunt, leading
to late cyanosis with right ventricular hypertrophy, polycythemia, and nail
clubbing.
➢ Defects with right to left shunting usually present as cyanosis shortly after
birth
ETIOLOGY
• Genetic and environmental factors are implicated.
• Only 10% can be linked to definite etiology.
➢ They may be associated with some chromosomal abnormalities e.g. Down
syndrome (trisomy 21), Turner syndrome (Monosomy X).
o Endocardial cushion defects, which result in atrial and ventricular septal
defects and atrioventricular valve deformities are frequently associated
with Down syndrome.
➢ They may also be associated with maternal infection e.g. rubella (German
measles) infection and Mumps infection
• Majority of congenital heart diseases (CHD) are idiopathic
CLASSIFICATION
• There are 3 main categories of congenital heart diseases:
➢ Acayanotic congenital heart disease (left-to-right shunt)
o Increases the amount of blood delivered to the right side of the heart and
will result in hypertrophy and dilation of the right atrium or right ventricle
(or both) depending upon the type of shunt.
o Eventually, the pressure in the right side of the heart increase and surpasses
that in the left side of the heart, resulting in a reversal of the shunt from
left-to-right to a right-to-left shunt. This change is called Eisenmenger
syndrome. In time, acyanotic defects become cyanotic.

89
 o Defects: atrial septal defects, ventricular septal defects, patent ductus
arteriosus.
➢ Cyanotic congenital heart disease (right-to-left shunt)
o Deoxygenated blood from the right side of the heart goes to the left side,
thus deoxygenated blood is delivered to the body.
o Defects (Remember they all start with “T”): Tetralogy of Fallot,
Transposition of the great arteries, Truncus arteriosus and Tricuspid
atresia.
o This type of defect results in cyanosis at the time birth.
➢ Conditions causing obstruction
o An abnormally formed valve or vessel leads to pressure overload of the
involved atrium or ventricle.
o Defects: coarctation of the aorta, pulmonary stenosis & atresia, Aortic
stenosis & atresia.

ACYANOTIC CONGENITAL HEART DISEASE

• Left to right shunts:
➢ Atrial septal defects
➢ Ventricular septal defects
➢ Patent ductus arteriosus
ATRIAL SEPTAL DEFECTS
• Initially ASD are left to right shunts because the pressure in left atrium is greater
than the pressure in the right atrium but the shunt can reverse after occurrence of
pulmonary hypertension.
NORMAL DEVELOPMENT OF THE ATRIAL SEPTUM
• To understand Atrial septal defects it is essential to understand normal interatrial
septum development:
➢ In early development of the heart, the heart is not a 4 chambered organ but
rather a 1 chambered organ around 5th week of intrauterine development.
➢ The single chamber of the heart develops an atrial septum, a ventricular
septum and a structure between the atrium and ventricle which is derived from
endocardial cushions (Atrioventicular cushions) making it into 4 chambers.
➢ Throughout prenatal life, blood is allowed to physiologically shunt from the
right atrium to the left atrium.
➢ The atrial septum starts to develop from the top of the primitive atrium, this
crescent-shaped septum is known as the septum primum. While the septum

90
 primum is descending another septum develops from the apex towards the
base (in the ventricle, which fuses with the endocardial cushion forming the
interventricular septum).
➢ In the initial part of development of the atrial septum as the septum primum
descends, it does not fuse with the endocardial cushion and so a foramen
formed between the free edge of the septum primum and AV cushions called
the foramen primum.
➢ The foramen helps shunt blood from the right to left atrium.
➢ This foramen is closed when the primum fuses with the AV cushion. When
the foramen primum closes the center of the septum primum develops several
openings which coalesce with each other to form the foramen secundum.
➢ Once the foramen secundum has been formed another septum develops on the
right side. This is known as the septum secundum.
➢ The crescent shaped septum secundum descends and covers the formen
secundum (on the septum primum) however the septum secundum has a
foremen known as the foramen ovale.
➢ In early development of the heart blood is shunted through the foramen
primum and then the foramen secundum and lastly the foramen ovale &
foramen secundum.
➢ At birth the septum primum fuses with the margins of the foramen ovale
forming the fossa ovale.
➢ Later in life the septum primum and septum secundum anatomically fuse to
complete the formation of the atrial septum.
➢ Note also that in the initial stages of development, a part of the inferior vena
cava fuses with the interatrial septum to form the sinus venosus.
CLASSIFICATION OF ATRIAL SEPTAL DEFECTS
• There are 3 main variants of ASD. They include:
➢ Ostium primum
o This type of ASD is a defect in the lower portion of the atrial septum
(Septum primum).
o There is failure of the septum primum to grow down in order to reach the
endocardial cushions leaving the foramen primum open.
o It accounts for about 5% of the ASDs.
o It is associated with abnormalities in development of AV valves (mitral
anterior leaflet defect- Mitral regurgitation or tricuspid septal leaflet). This

91
 is because there is failure of the fusions of the septum primum with the
endocardial cushions.
o Ostium primum ASD is a common congenital heart lesion in Down
syndrome.
➢ Ostium secundum
o This type of ASD is a defect in the middle portion of the atrial septum.
o The septum primum develops completely with the foramen secundum but
the septum secundum does not develop enough to cover the foramen
secundum and due to this under-development of the septum secundun the
foramen ovale becomes large and the foramen secundum is uncovered.
o Ostium secundum is the most common type of ASD (90%)
o It is not associated with mitral or tricuspid defects.
➢ Sinus venosus
o This type of ASD is a defect high in the septum near the junction of the
right atrium and superior vena cava.
o In sinus venosus, the right pulmonary veins usually drain anomalougly into
the right atrium or superior vena cava instead of into the left atrium.
PATHOPHYSIOLOGY
• Blood flows across the septal defect from the left atrium to the right atrium.
• The direction of the blood flow is determined by the compliance of the right and
left ventricles (compliance is determined by systemic and pulmonary vascular
resistance).
• Blood therefore flows from areas of higher resistance to areas of lower resistance.
• Increased blood flow across the ASD leads to an increase in size of the right
atrium and right ventricle and to increased pulmonary blood flow.
CLINICAL FEATURES
• Symptoms are minimal, if any except in patients with an ostium primum defect
who develop mitral regurgitation that results in CHF.
• Physical examination:
➢ Increased right ventricular impulse as a result of right ventricular overload
➢ Systolic ejection murmur (from excessive pulmonary blood flow) best heard
at the mid and upper left sternal borders. A mid-diastolic filling rumble
representing excessive blood flow through the tricuspid valve may also be
heard.

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 ➢ Fixed split second heart sound because of the excessive pulmonary blood
flow, the normal physiologic variation in timing of the aortic and pulmonic
valve closure with respiration is absent.
DIAGNOSIS
• Usually diagnosed incidentally.
• Echocardiography is diagnostic.
• ECG shows:
➢ Right axis deviation
➢ Right ventricular hypertrophy
➢ Right atrial enlargement
• Chest x-ray shows:
➢ Right atrial and ventricular enlargement
➢ Increase pulmonary vascular markings
MANAGEMENT
• Treatment is closure by open heart surgery to prevent right sided hear failure,
pulmonary hypertension, atrial dysrhythmias and paradoxical embolism.
• Some centers close ASDs using interventional catheterization procedures.
VENTRICULAR SEPTAL DEFECTS
• Ventricular septal defects are classified by location as:
➢ Inlet
➢ Trabecular (muscular)
➢ Membranous
➢ Outlet (supracristal)
PATHOPHYSIOLOGY
• After birth as pulmonary vascular pressure decreases, blood flow across the VSD
from the left ventricle to the right ventricle owing to the lower resistance within
the pulmonary circulation compared with the resistance within the systemic
circulation.
• However, with time the pulmonary vessels hypertrophy in response to this
increased pulmonary flow.
• This hypertrophy may lead to increased pulmonary vascular resistance
(pulmonary hypertension).

93
CLINICAL FEATURES
• Clinical features and course vary greatly depending on the magnitude of the left
to right shunt across the VSD.
• The amount of blood flow directed from one side of the heart to the other side is
determined by both the size of the VSD and the degree of pulmonary vascular
resistance.
• For example, the larger the VSD and the lower the pulmonary vascular resistance
(PVR) the greater the blood flow across the VSD into the pulmonary vessels. The
greater the pulmonary blood flow, the more symptomatic the patient.
• Typical clinical presentations include:
➢ Small VSDs
o Little to no shunt across VSD
o May close spontaneously
o On examination, a thrill at the lower left sternal border and a grade 4 high-
pitched holosystolic murmur may be present, indicative of a very
restrictive defect with a high flow velocity across the VSD.
o As the size of the VSD decreases the intensity of the murmur increases.
➢ Moderate VSDs
o May have a larger shunt across the VSD that may result in signs and
symptoms of CHF.
o A holosystolic murmur is usually present and its intensity depends on the
size of the shunt.
o If excessive blood flows across the VSD (2:1 pulmonary to systemic flow)
then a diastolic murmur of mitral turbulence (mitral filling rumble
representing the excess blood from the lungs not passing through the mitral
valve) may be heard at the apex.
➢ Large VSDs
o Often cause signs and symptoms of CHF.
o Have less turbulence across the VSD, so the systolic murmur is shorter and
lower in pitch.
o A mitral filling rumble may be heard at the apex.
• Pulmonary vascular resistance may eventually become elevated in moderate or
larger VSDs in response to chronically high pulmonary flow.
• When PVR becomes elevated the right ventricular impulse is noticeably
increased and the second heart sound may be single and loud. The mitral filling

94
 rumble disappears because of diminished pulmonary blood flow as a result of
decreased left to right shunting.
• If PVR remains elevated, pulmonary hypertension becomes irreversible even if
the VSD is surgically closed. In the extreme situation in which PVR exceeds
systemic vascular resistance shunting changes from left-to-right to right-to-left a
condition termed Eisenmenger syndrome.
DIAGNOSIS
• Confirmed by ECHO
• ECG shows:
➢ Normal or mild left ventricular hypertrophy with small VSDs
➢ Left ventricular hypertrophy and right ventricular hypertrophy if pulmonary
hypertension is present in moderate VSDs
• Chest X-ray findings
➢ Normal in small VSDs
➢ Cardiomegaly and increased pulmonary vascular markings in moderate or
large VSDs
MANAGEMENT
• Medical:
➢ Medical management of CHF in a symptomatic child. Large shunts are also
associated with high incidence of pulmonary infections from excessive blood
flow.
➢ Surgical closure, indicated in:
o Heart failure refractory to medical interventions
o Large VSDs with pulmonary hypertension are usually surgically closed at
3-6 months of age.
o Small to moderate VSDs are usually closed between 2 to 6 years of age.
PATENT DUCTUS ARTERIOSUS
• In the fetus, the ductus arteriosus connects the pulmonary artery to the aorta. It
serves as a by-pass because the lungs are not function hence pulmonary vascular
resistance is high.
• After birth as the PaO2 rises the ductus normally fibroses. If it remains open it is
termed a PDA.
• Incidence of PDA is especially high in preterm infants.
• Note: intrauterine the PDA is kept open by production of PGE2

95
PATHOPHYSIOLOGY
• In a PDA, blood flow through the ductus from the aorta to the pulmonary artery
(left to right shunt) leading to increased pulmonary blood flow.
CLINICAL FEATURES
• Signs and symptoms depend on size of PDA and the relationship between
systemic vascular resistance and pulmonary vascular resistance.
• Small PDAs: usually produce no symptoms
• Moderate or Large PDAs generally result in signs and symptoms of CHF due to
increased pulmonary blood flow
• Physical findings:
➢ Classical murmur which is described as a continuous “machinery-like”
murmur heard best at the upper left sternal border.
➢ If the left to right shunt is large, there may also be a diastolic rumble of blood
flow across the mitral valve at the apex, a widened pulse pressure (>30mmHg)
and brisk pulses.
• Risk of pulmonary hypertension caused by excessive pulmonary blood flow is
significant in children older than several years of age.
MANAGEMENT
• Indomethacin is used in premature infants to close a PDA medically.
➢ Indomethacin an NSAID inhibits production of PGE2 which is required to
keep the PDA.
• PDAs may also be closed surgically by coil embolzation, video-assisted
thoracoscopic surgery, and ligation in a thoracotomy.

CONGENITAL CYANOTIC HEART DISEASES

• Cyanosis may be peripheral, central or both.
• Peripheral cyanosis is usually caused by vasomotor instability or vasoconstriction
as a result of cold temperature.
• Central cyanosis especially in the tongue and inner mucus membranes may be
attributed to both cardiac and non-cardiac causes.
➢ Non-cardiac causes: pulmonary disease, sepsis, hypoglycemia, polycythemia
and neuromuscular disease that impair chest wall movement
➢ Cardiac: Remember them by the 5Ts:
o Tetralogy of Fallot (TOF)
o Transposition of great arteries

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 o Tricuspid atresia
o Truncus arteriosus
o Total anomalous pulmonary venous connection

TETRALOGY OF FALLOT
• This is the most common cyanotic congenital cardiac disease in children.
CLINICAL FEATURES
• As the name suggests TOF has 4 anatomical features:
➢ Subpulmonary stenosis (Right ventricular outflow tract obstruction)
➢ Right ventricular hypertrophy
➢ Ove-riding of the aorta with respect to the ventricular septum (aorta overlies
a portion of the ventricular septum this is due to a defect of the development
of the aorticopulmonary septum)
➢ Large ventricular septa defect (VSD)
• The severity of right ventricular outflow obstruction determines the amount of
right to left shunting across the VSD.
• Because of the right ventricular outflow obstruction blood flows right to left
across the VSD and into the overriding aorta, as a result there is diminished blood
flow and cyanosis results.
• Most are diagnosed:
➢ Antenatally or
➢ Following the identification of a murmur in first 2 months of life. Cyanosis at
this stage may not be obvious although a few present with severe cyanosis in
the first few days of life.
• Hypercyanotic spells (tet spell), may lead to myocardial infarction,
cerebrovascular accidents and even death if left untreated.
• Symptoms depend on the severity of right ventricular obstruction
• They are characterized by a rapid increase in cyanosis, usually associated with
irritability or inconsolable crying because of severe hypoxia, and breathlessness
and pallor because of tissue acidosis.
• To compensate, a child with TOF learns to squat. This position (knee-chest
position in an infant) increases venous return to the heart and increases systemic
vascular resistance thereby decreasing the right to left shunt.
• On auscultation there is a very short murmur during a tet spell.
• Signs:

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 ➢ Clubbing of the fingers and toes in older children
➢ A loud harsh ejection systolic murmur at the left sternal edge from day 1 of
life with increasing right ventricular outflow tract obstruction, which is
predominantly muscular and below the pulmonary valve, the murmur will
shorten and cyanosis will increase.
• Note:
➢ Actions like increase systemic vascular resistance (exercise, vasodilation,
volume depletion) or increase resistance through the right ventricular outflow
tract (e.g. crying, tachycardia) increase right to left shunting resulting in
cyanosis.
➢ Actions that increase systemic vascular resistance or reduce resistance
through the right ventricular outflow obstruction (e.g. volume infusion,
systemic hypertension, Valsalva maneuver, bradycardia) reduce the right to
left shunt through the VSD and increase systemic arterial saturation.
➢ TOF is condition that is PDA dependent.
INVESTIGATIONS
• Chest X-ray shows:
➢ Mild to moderate cardiomegaly
➢ Uplifted apex secondary to right ventricular enlargement and concavity in the
region of pulmonary artery segment giving a boot shaped heart.
➢ A right sided aortic arch occurs in 25% of patients with TOF.
➢ Decreased pulmonary vascular markings reflecting reduced pulmonary blood
flow

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• ECG
➢ Normal at birth
➢ Right ventricular hypertrophy when older
• Echocardiography
➢ This will demonstrate the cardinal features, but cardiac catheterization may
be required to show the detailed anatomy of the coronary arteries.
MANAGEMENT
• Initial management is medical with definitive surgery at around 6 months of age.
It involves closing the VSD and relieving the right ventricular outflow tract
obstruction sometimes with an artificial patch, which extends across the
pulmonary valve.
• Infants who are very cyanosed in the neonatal period require a shunt to increase
pulmonary blood flow. This is usually done by surgical placement of an artificial
tube between the subclavian and the pulmonary artery (a modified Blalock-
Taussig shunt) or sometimes by balloon dilatation of the right ventricular outflow
tract.
• Hypercyanotic spells are usually self-limiting and followed by a period of sleep.
➢ Placement in knee-chest position (mimics squatting position): this traps
systemic venous blood in the legs thereby decreases the systemic venous
return and helping to calm the baby. This position may also increase SVR by
reducing arterial blood flow through the femoral arteries.
➢ Sedation and pain relief (morphine 0.2 mg/kg sc, IM or IV is excellent):
morphine suppresses the respiratory center and has a sedative effect.
➢ Sodium bicarbonate 1mmol/l IV: corrects acidosis and eliminates respiratory
center-stimulating effect of acidosis. The same dose can be repeated in 10 to
15 minutes.
➢ Administration of oxygen may improve arterial oxygen saturation a little.
➢ Intravenous propranolol 0.01 to 0.25mg (or an alpha adrenoceptor agonist e.g.
phenylephrine at 0.02mg/kg IV) which probably works both as a peripheral
vasoconstrictor and by relieving the subpulmonary muscle obstruction that is
the cause of reduced pulmonary blood flow as well as reduce heart rate,
contractility and ventricular outflow obstruction.
➢ Ketamine at 1 to 3 mg/kg given over 1 minute is a good drug to use since it
increases SVR and sedates the patient both of which are known to terminate
the hypercyanotic spell.
➢ Intravenous fluid administration

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 ➢ Muscle paralysis and artificial ventilation in order to reduce metabolic oxygen
demand.
➢ Correction of significant anemia with transfusion
• Good dental hygiene and antibiotic prophylaxis against subacute bacterial
endocarditis should be instituted.

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RHEUMATIC FEVER
• Rheumatic fever is a delayed non-suppurative autoimmune complication of upper
respiratory infection with Lancefield group A beta-hemolytic streptococcus
(Streptococcus pyogenes) characterized by inflammation of the connective
tissues.
• There also seems to be a familial tendency to develop the disease and the
expression of certain HLA groups on a host’s B-cells may make them more
susceptible to rheumatic fever.
• Rheumatic fever predominantly affects the heart, blood vessels, joints, CNS and
skin.
• It is most common in children 5-15 years of age, reflecting the age group most
susceptible to streptococcal throat infections.
• It has no gender predilection but mitral valve disease and chorea is more common
in girls whereas aortic valve involvement is more often seen in boys.
• The major risk factor is pharyngitis caused by certain strains of group A beta-
hemolytic streptococcus. Especially the M serotypes.
• The streptococcal strains that cause streptococcal skin infection (i.e. impetigo)
do not cause rheumatic fever.
• The cause of rheumatic fever is unknown but the disease appears to be
autoimmune in nature.
PATHOGENESIS
• Antibodies produced in response to a streptococcal throat infection in a
susceptible host react to streptococcal M protein as well as cross react with host
connective tissues of the body.
• This phenomenon is attributed to molecular mimicry.
• Rheumatic fever seems to be the result of the host’s unusual response at both the
cellular and humoral level to Streptococcus.

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CLINICAL FEATURES
• Major features:
➢ Sydenham’s chorea
➢ Polymigratory arthritis
➢ Erythema marginatum
➢ Subcutaneous nodules
➢ Carditis
• Minor features:
➢ Fever (>39.5OC), arthralgias, leukocytosis, increased ESR (≥30mm/hr),
increased C-reactive protein (≥30 mg/l), previous rheumatic fever, and
prolonged PR interval on ECG.
SYDEHAM CHOREA
• Syndenham chorea (St. Vitus’ dance/ Rheumatic chorea) is a self-limited
autoimmune disorder associated with rheumatic fever that presents with chorea
(uncontrolled, restless proximal limb movements) and emotional lability.
• It is seen in approximately 25% of patients with rheumatic fever and usually
presents as a late manifestation occurring about 3 months after the onset of acute
rheumatic fever.
• Onset is most common between 5 and 15 years of age.
• It is more common in girls.

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• Syndeham chorea occurs secondary to antibodies that cross-react with membrane
antigens on both group A beta hemolytic streptococcus and basal ganglia.
• Children appear restless.
• The face, hands and arms are mainly affected and the movements appear
continuous, quick and random. The chorea may begin as clumsiness of the hands.
➢ Incoordination may be marked or only obvious when the child is asked to pick
a coin off the floor.
• Involuntary purposeless, non-repetitive movements of the limbs, face and trunk
e.g. grimacing, wriggling and writhing. The movements can be brought under
voluntary control temporarily.
• The movements are aggravated by excitement/stress, attention and they disappear
during sleep.
• The first indication may be that the child begins to drop things or her handwriting
suddenly deteriorates or she gets in trouble with her elders for making faces.
Sometimes the movements are confined to one side of the body (hemichorea)
• Hypotonia may result in muscular weakness. It also causes the characteristic
posture of the outstretched hand in which the wrist is slightly flexed, whereas
there is hyperextension of the metacarpophalangeal joints (choreic hand).
• Occasionally the child is unable to stand or even to sit up (chorea paralytica)
• Speech is also affected and can be jerky or indistinct.
• Patients are unable to sustain protrusion of the tongue (chameleon tongue).
• On gripping the examiner’s fingers, patients are unable to maintain the grip
(milkmaid’s grip)
• Gait and cognition are not affect but emotional lability is common.
• Infrequently the child becomes confused or even maniacal (chorea insaniens).
• The following clinical maneuvers are helpful in arriving at the diagnosis:
➢ When the hand is outstretched above the head, forearms tend to pronate
(pronator sign)
➢ When hands are stretched forwards, wrist flexes and fingers hyperextend
➢ The child relaxes hand grip on and off as if he is milking a cow (Milkmaid
grip)
➢ The child cannot maintain tongue protrusion (darting tongue/ chameleon
tongue)
➢ During speech an audible click is heard.
➢ The knee reflex may show a sustained contraction resulting in a hung up
reflex.

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• There is no single confirmatory test for syndenham chorea.
➢ The diagnosis rest on presumptive evidence of rheumatic fever and exclusion
of other likely causes of chorea
➢ Elevated antistreptolysin O (ASO) or anti-DNase B (ADB) may indicate a
recent streptococcal infection.
➢ Neuroimaging such as head MRI may show increased signal intensity in the
caudate and putamen on T2-weighted sequence.
➢ Single-photon emission computed tomography (SPECT) may demonstrate
increased perfusion to the thalamus and striatum.
• Symptoms may last from several months to 2 years. Generally, all patients
recover. Relapses or recurrences can occur.
• Differential diagnosis:
➢ Encephalitis
➢ Kernicterus
➢ Systemic lupus erythematosus
➢ Huntington’s disease
➢ Wilson’s disease
• Management:
➢ Reduce physical and emotional stress and use protective measures as
indicated.
➢ Eradicate Group A streptococcus and then secondary prophylaxis with a
penicillin.
o Secondary prophylaxis
▪ Benzathine penicillin every 4 week: 1, 200, 000 IU in all people above
20kg and 600, 000 IU in those less than 20 kg
▪ Penicillin V 250mg BD
▪ Erythromycin 250mg BD in those with penicillin allergy
➢ Anti-inflammatory agent is not indicated
➢ For severe chorea any of the following drugs may be used:
o Phenobarbitone 15 to 30mg every 6-8 hours PO
o Haloperidol 0.5-2.0 mg every 8 hours PO
o Chlorpromazine 0.5-2mg/kg 6-8 hourly PO or PRN
o Diazepam 0.3 mg/kg PRN PO
o Steroids

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POLYMIGRATORY ARTHRITIS
• This is classically migratory, asymmetric and exquisitely painful.
• It occurs in 70% of patients and most commonly involves the elbows, knees,
ankles and wrists.
• Uncommonly smaller joints may also be involved.
• The affected joints show redness, warmth, swelling, pain and limitation of
movement.
• The pain and swelling appear rather quickly last 3 to 7 days and subside
spontaneously to appear in some other joint.
• It does not result in chronic disease.
• Arthritis tends to be commoner in older patients.
• Management:
Arthritis alone Minimal carditis Moderate carditis Severe carditis
Prednisolone Nil Nil 2-4 weeks 2-6 weeks
Aspirin 1-2 weeks 2-4 weeks 2-8 weeks 2-4 months

➢ Aspirin dose= 100mg/kg/day in 4 to 6 divided doses. Aspirin may be reduced

to 60mg/kg/day after 2 weeks of therapy
➢ Prednisolone dose= 2mg/kg/day in 4 divided doses. The dose of prednisolone
should be tapered off the prednisolone should be tapered and aspirin started
during the final week
ERYTHEMA MARGINATUM
• This is a non-pruritic/itching rash that starts as pink to red macules with a pale
center which may coalesce and spread centripetally with central clearing over the
trunk and proximal limbs.
• It is believed that the inability to recognize erythema marginatum is not because
it does not occur but because of the dark complexion of the skin.
SUBCUTANEOUS NODULES
• Although rarely seen are associated with severe cardiac involvement.
• These small, mobile and painless nodules occur on the bony prominences (shins,
elbows, occiput & spine) of the extensor surfaces of the extremities.
• They are non-tender and last from a few days to weeks but have been known to
last for almost a year.

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CARDIAC INVOLVEMENT
• This is found in 50% of patients.
• It is the hallmark and most important complication of rheumatic fever.
• Rheumatic carditis is designated as a pancarditis involving the pericardium,
myocardium and endocardium
• Endocarditis is the most common cardiac findings and typically causes
insufficiency of the left-side valves (mitral and aortic). It rarely affects the
pulmonic or tricuspid valves.
➢ Endocarditis is represented by a pansystolic murmur of mitral regurgitation
with or without an associated aortic regurgitation murmur.
• Myocarditis is usually manifested by tachycardia out of proportion to the extent
of the fever. Other severe manifestations include cardiac dilatation and heart
failure.
• Pericarditis and pericardial effusions are less common.
➢ Pericarditis results in precordial pain that may be quite severe.
➢ On auscultation a friction rub is present.
➢ ECG shows ST and T changes consistent with pericarditis.
➢ Rheumatic pericarditis is associated with only small effusions and generally
does not result either in tamponade or constrictive pericarditis.
➢ A patient of rheumatic pericarditis always has additional mitral or mitral and
aortic regurgitation murmurs.
• Carditis is an early manifestation of rheumatic fever. In 60-70% it is clinically
obvious whereas in the remaining the diagnosis is based on echocardiographic
findings labeled as subclinical carditis.
• Other features of carditis are:
➢ Cardiac enlargement
➢ Soft first sound
➢ Protodiastolic (S3) gallop
➢ Congestive cardiac failure
➢ Cary Coombs’ murmur: a soft delayed diastolic mitral murmur heard
transiently during the course of acute rheumatic fever possibly as a result of
flow across the inflamed and thickened mitral valve.
• Management of carditis involves:
➢ Bed rest and ambulation
➢ Uses aspirin and prednisolone as described earlier. Congestive heart failure
should be managed with conventional management.

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 Arthritis alone Minimal carditis Moderate carditis Severe carditis
Prednisolone Nil Nil 2-4 weeks 2-6 weeks
Aspirin 1-2 weeks 2-4 weeks 2-8 weeks 2-4 months

➢ Aspirin dose= 100mg/kg/day in 4 to 6 divided doses. Aspirin may be reduced

to 60mg/kg/day after 2 weeks of therapy
➢ Prednisolone dose= 2mg/kg/day in 4 divided doses. The dose of prednisolone
should be tapered off the prednisolone should be tapered and aspirin started
during the final week
➢ Note:
o Minimal carditis: questionable cardiomegaly
o Moderate carditis: definite but mild cardiomegaly
o Severe carditis: marked cardiomegaly or CCF
Arthritis alone Minimal Moderate Severe carditis
carditis carditis
Bed rest 1-2 weeks 2-3 weeks 4-6 weeks 2-4 months
Indoor 1-2 weeks 2-3 weeks 4-6 weeks 2-3 months
ambulation
Outdoor 2 weeks 2-4 weeks 1-3 months 2-3 months
activity
(school)
Full activity After 4-6 After 6-10 After 3-6 Variable
weeks weeks months

DIAGNOSIS
• The JONES criteria for rheumatic fever is used in diagnosis of rheumatic fever.
• Diagnosis requires evidence of recent streptococcal infection and either 2 major
criteria or 1 major plus 2 minor criteria.
• Lab findings:
➢ Nonspecific inflammatory markers:
o Elevated ESR
o Elevated C reactive protein
o Elevated WBC
➢ Serologic markers
o Antistreptolysin-O titers are abnormally elevated in 70-80% of patients
with rheumatic fever.

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 o Anti-DNase antibodies
o Anti-hyaluronidase antibodies
• Echocardiography typically shows evidence of carditis, such as decreased
ventricular function, valvular insufficiency or pericardial effusion.

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MANAGEMENT
• Eradication of GABHS infection
➢ Benzathine penicillin intramuscular infection (one dose)
➢ Penicillin orally for 10 days
• Bed rest:
Arthritis alone Minimal Moderate Severe carditis
carditis carditis
Bed rest 1-2 weeks 2-3 weeks 4-6 weeks 2-4 months
Indoor 1-2 weeks 2-3 weeks 4-6 weeks 2-3 months
ambulation
Outdoor 2 weeks 2-4 weeks 1-3 months 2-3 months
activity
(school)
Full activity After 4-6 After 6-10 After 3-6 Variable
weeks weeks months

• Control of inflammation
➢ NSAIDs are useful for control of joint pain and swelling, but if given before
definitive diagnosis, they may obscure the diagnosis by halting the
development of migratory arthritis. Therefore, their use is recommended only
after the diagnosis of rheumatic fever is certain.
➢ Corticosteroids are often used in patients with severe cardiac involvement
such as CHF and severe valvuar dysfunction.
Arthritis alone Minimal carditis Moderate carditis Severe carditis
Prednisolone Nil Nil 2-4 weeks 2-6 weeks
Aspirin 1-2 weeks 2-4 weeks 2-8 weeks 2-4 months

➢ Aspirin dose= 100mg/kg/day in 4 to 6 divided doses. Aspirin may be reduced

to 60mg/kg/day after 2 weeks of therapy
➢ Prednisolone dose= 2mg/kg/day in 4 divided doses. The dose of prednisolone
should be tapered off the prednisolone should be tapered and aspirin started
during the final week
• Supportive therapy:
➢ CHF: diuretics, dietary salt restriction, digoxin and bed rest
➢ Syndeham’s chorea, if severe may be treated with haloperidol, phenobarbital
or valproic acid.

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 o Phenobarbitone 15 to 30mg every 6-8 hours PO
o Haloperidol 0.5-2.0 mg every 8 hours PO
o Chlorpromazine 0.5-2mg/kg 6-8 hourly PO or PRN
o Diazepam 0.3 mg/kg PRN PO
o Steroids
• Long term management
➢ Continuous antimicrobial prophylaxis to prevent recurrent episodes of
rheumatic fever. Monthly injections of benzathine penicillin is the most
effective prophylaxis. Alternatively, the penicillin can be given orally every
day but compliance may be a problem. Oral erythromycin can be substituted
in those sensitive to penicillin.
o Benzathine penicillin every 4 week: 1, 200, 000 IU in all people above
20kg and 600, 000 IU in those less than 20 kg
o Penicillin V 250mg BD
o Erythromycin 250mg BD in those with penicillin allergy
➢ The length of treatment is controversial. Most recommend treatment of the
age of 18 or 21 years but more recently lifelong prophylaxis has been
advocated.
➢ Aspirin is very effective at suppressing the inflammatory response of the
joints and heart. It needs to be given in high dosage and serum levels
monitored.
PROGNOSIS
• There are no chronic sequelae of the join, skin and CNS.
• Cardiac inflammation often leads to severe valvular dysfunction which may
require intervention, immediately or many years after the event.
• Valvular insufficiency or stenosis is usually delayed (usually more than 3 years
after rheumatic fever) and if severe may require valve replacement or
valvuloplasty.
• The severity of eventual rheumatic valvular disease relates to the number of
childhood episodes of rheumatic fever.

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 CHAPTER 10: HEMATOLOGY

HEMATOLOGY
SICKLE CELL DISEASE
• This is an autosomal recessive disease that results from the substitution of
glutamic acid (a negatively charged hydrophilic amino acid) for valine (a neutral
hydrophobic amino acid) at position 6 of the beta-globin chain.
• The disorder results from a point mutation (GAG changing to GTG) on
chromosome 11p15.5 (chromosome 11 position short arm on position 15.5).
• Deoxygenation of the heme moiety of sickle hemoglobin leads to hydrophobic
interactions between adjacent sickle hemoglobin (HbS) molecules that aggregate
into larger polymers.
• Sickle red blood cells are less deformable and obstruct the microcirculation,
resulting in tissue hypoxia, which further promotes sickling.
• These red blood cells are rapidly hemolyzed and have a life span of only 10-20
days.
• The usual onset of the disease is 5-6 months of age as HbF (which has 2 alpha
chains and 2 gamma chains) concentration reduces.
• Depending on the type of hemoglobin chain combinations, 3 clinical syndromes
occur:
➢ Sickle cell trait
➢ Sickle cell disease
➢ Sickle cell anemia
• Sickle cell trait (Heterozygous HbA HbS): these are carriers of the sickle cell
gene and a normal adult hemoglobin gene. They are asymptomatic but can be
symptomatic on certain occasions e.g. hypoxia/at high altitudes.
➢ These individuals have both HbA (50-60%), HbS (35-45%) and a small
percentage of HbF.
➢ Patients are usually asymptomatic without anemia unless exposed to severe
hypoxemia.
➢ Some patients have an inability to concentrate urine (isosthenuria) or
hematuria (5%) during adolescence.
➢ Carriers are protected against Plasmodium falciparum.

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• Sickle cell disease (Combined heterozygosity): these are individuals with an
abnormal sickle cell gene i.e. HbS and another abnormal hemoglobin gene e.g.
HbC. They often suffer intermediate symptoms.
• Sickle cell anemia (Homozygous HbS HbS) have the most severe disease. They
have 2 abnormal sickle cell genes. These are not protected from malaia.
PATHOGENESIS
• Hemoglobin S maintains normal function in oxygenated state.
• Deoxygenated HbS molecules are insoluble and polymerize. This increases
viscosity.
➢ Polymer formation at concentrations exceeding 30g/dl.
➢ Polymers form a gel-like substance containing Hb crystals called tactoids.
• Flexibility of the cells is decreased and they become rigid and take up their
characteristic sickle appearance.
• This process is initially reversible but with repeated sickling the cells eventually
lose their membrane flexibility and become irreversibly sickled.
➢ This is due to dehydration, partly caused by potassium leaving the red cells
via calcium activated potassium channels called the Gados channel.
• These irreversibly sickled cells are dehydrated and dense and will not return to
normal when oxygenated.
• Sickling produces:
➢ Shortened red cell survival
➢ Impaired passage of cells through the microcirculation, leading to
obstruction of small vessels and tissue infarction
• Precipitating factors for sickling:
➢ Hypoxia
➢ Dehydration
➢ Cold
➢ Acidosis
➢ Infection and fever
➢ Living at high altitude
• A crisis may occur without the presence of these factors.
• Adhesion proteins on activated endothelial cells (VCAM-1) may play a role
particularly in vaso-occlusion when rigid cells are trapped, facilitating
polymerization.

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• HbS releases its oxygen to the tissues more easily than does normal Hb and
patients therefore feel well despite being anemic (except of course during crises
or complications).
CLINICAL FEATURES
• Clinical features are often termed ‘crises’ because they occur suddenly.
• They include:
➢ Sequestration crisis
➢ Hyper-hemolytic crisis
➢ Aplastic crisis
➢ Vaso-occlusive crisis
➢ Mixed
• Although infection, dehydration, acidosis, cold temperature, exercise, extreme
emotions (all of which favor sickling) can act as triggers, in most cases no
predisposing cause is identifiable.
VASO-OCCLUSIVE CRISIS
• This is the most common crisis.
• This occurs when the microcirculation is obstructed by sickled red blood cells
resulting in ischemic injury.
• The major complaint is pain usually affecting bones such as femur, tibia and
lower vertebrae.
➢ Typically presents as deep, gnawing or throbbing pain lasting 3-7 days.
➢ In young children it affects the extremities however in older patients it affects
the head, chest, abdomen or back.
➢ The pain is recurrent
• Triggers of vaso-occlusive crisis include:
➢ Hypoxemia: may be due to acute chest syndrome or respiratory
complications
➢ Dehydration: acidosis results in a shift of the oxygen dissociation curve

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 ➢ Changes in body temperature (Fever)
• Vaso-occlusion may present as:
➢ Acute Dactylitis (Hand and foot syndrome): painful
symmetric swelling of digits of the hands and feet due
to ischemic necrosis of small bones of hands and feet.
It is also thought to be due to rapidly expanding bone
marrow resulting in choking of blood supply
o DDx: osteomyelitis
➢ Acute abdomen: abdominal pain and distention often
caused by sickling within mesenteric artery. Figure 13: Dactylitis in SCA
o DDx: cholecystitis, appendicitis, splenic
sequestration
➢ Infarctions:
o Cerebrovascular accidents (Stroke): Dysathria, hemiplegia, may be
asymptomatic.
o Retinal hemorrhages
o Pulmonary infarcts and acute chest syndrome: Pneumonitis and fat emboli
o Auto-infarction of spleen and autosplenectomy (by 6 years it is usually not
palpable) leading to increased susceptibility to encapsulated organisms
especially pneumococcus and H. influenzae. This because of a reduction
in serum opsonins.
o Kidney: Involvement of the kidney results in papillary necrosis leading to
inability to concentrate urine (isosthenuria).
o Bone/bone marrow: osteomyelitis (commonly due to salmonella infection)
o Avascular necrosis of the femoral head
o Priapism: sustained painful, purposeless erection. Always consider SCD in
any patient presenting with priapism.
ACUTE CHEST SYNDROME
• This is a type of vaso-occlusive crisis that affects the lungs and presents with
chest pain, cough, tachypnea, dyspnea, hypoxemia, hypotension, fever or X-ray
findings e.g. a new pulmonary infiltrate.
• Causes include infection (e.g. viral, Mycoplasma pneumoniae, Chlamydia
pneumoniae, Streptococcus pneumoniae), sickling, atelectasis, fat embolism,
painful bone crises involving the ribs and pulmonary edema from fluid overload.

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• Management:
➢ Admission
➢ Hydration: IV fluids (1.5 x maintenance)
➢ Pain management: analgesics- NSAIDs (diclofenac/ naproxen)
➢ Oxygen support- ventilation (CPAP may be necessary)
➢ Antibiotics (also should cover for mycoplasma and chlamydia, usually
cefuroxime and azithromycin)
➢ Incentive spirometry
➢ Bronchodilators
➢ Steroids
➢ Early use of partial exchange transfusion in a patient who does not improve
rapidly.

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SEQUESTRATION CRISIS
• This is due to sickled cells that block splenic outflow, leading to the pooling of
peripheral blood in the engorged spleen resulting in splenic sequestration.
• Less common also occurs in the liver.
• This occurs in patients <6 years of age.
• Presents with abdominal distension, abdominal pain, shortness of breath,
tachycardia, pallor, fatigue, and shock/circulatory collapse.
• The exact reason as to why this happens is largely unknown though it may follow
a febrile illness.
• Mortality can be high.
• Labs reveal reticulocytosis.
• There will be severe hemolysis, rapid splenomegaly and a high reticulocyte
count.
• Splenectomy is recommended by some practitioners because recurrence occurs
in up to 50%.
• Children have increased susceptibility to encapsulated organisms (e.g.
Haemophilus influenza, Streptococcus pneumoniae, Neisseria meningitidis)-
thus vaccine should be given
• They are also at risk of other common infectious organisms such as Salmonella,
Mycoplasma pneumoniae, staphylococcus aureus and Escherichia coli).
APLASTIC CRISIS
• This most commonly occurs following infection with erythrovirus B19
(previously called Parvovirus B19) which invades proliferating erythroid
progenitors.
• There is a rapid fall in hemoglobin with no reticulocytes in the peripheral blood
because of failure of erythropoiesis in the marrow.
• Presents with pallor, fatigue, tachycardia.
• Children with aplastic crisis may present with congestive heart failure due to
severe anemia.
• Usually only supportive care and occasionally packed red blood cells
transfusions are required.

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HYPERHEMOLYTIC CRISIS
• Rapid hemolysis.
• Often occurs in patients with other hemolytic diseases (e.g. G6PD deficiency)
• Presents with pallor, fatigue, tachycardia and jaundice.
➢ Remember jaundice also results from turn-over of RBCs with life span of 10-
20 days.
• Usually only supportive care and occasionally packed red blood cells
transfusions are required.
• The hyperhemolytic paradigm proposes that hemolysis in sickle cell disease leads
to increased cell-free plasma Hb which consumes Nitric oxide (NO) leading to a
state of deficiency, endothelial dysfunction and a high prevalence of pulmonary
hypertension.
DIAGNOSIS
• Requires:
➢ Full history: recurrence of symptoms (usually pain- joints, back, abdominal),
recognition of specific processes (acute chest syndrome, cholecystitis, splenic
sequestration and priapism)
➢ Full examination noting neurological changes
o General examination: Fussiness, irritability, poor feeding
o Icterus, pallor, maxillary hyperplasia
o Vital signs
o Neurological examination
o Cardiac: murmurs
o Respiratory: asymmetry of breath sounds
o Abdomen: assess for spleen, Murphy’s sign
o Genitourinary: priapism
o Extremities: edema, inflammation
➢ Investigations
INVESTIGATIONS
• Hb electrophoresis (diagnostic gold standard): can differentiate between
homozygous or heterozygous. (this needs to be checked prior to blood
transfusion)
• Blood investigations:
➢ FBC: findings include
o Hb: 6-9g/dl (normocytic anemia)

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 o Hematocrit: 18-27%
o Reticulocyte count: 5-15% (this may be seen as a raised WBC count)
o WBC count: 12 000-20 000 cell/mm3 with neutrophil predominance
o Platelet count: increased, often >500, 000 platelet/micro litre
o Increased RDW. (Normal 11-15%)
➢ Peripheral smear
o Shows: Sickle shaped cells, Target cells, Poikilocytes and Howell-Jolley
bodies

Figure 14: Sickle Shaped RBCs with normal shaped RBCs

o Howell-Jolley bodies indicate that the patient is functionally asplenic.

o Howell-Jolley bodies are basophilic nuclear remnant (clusters of DNA) in
circulating erythrocytes, during maturation in the bone marrow, late
erythroblasts normally expel their nuclei, but in some cases a small portion
of DNA remains.

Figure 15: Howell-Jolly Bodies

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 o Target cells: abnormal form of red blood cell which appears as a dark ring
surrounding a dark central spot. Also known as codoctytes.

Figure 16: Target cells

Figure 17: Peripheral smear showing: Target cells, sickle shaped red blood cells and Howell Jolley bodies

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 ➢ Liver function tests
➢ Urea and electrolytes
➢ Hemoglobin solubility test
➢ Sickling test (Sodium metabisulfite hemoglobin solubility test): if the
diagnosis has not been made this is done to establish the presence of sickle
hemoglobin.
➢ Blood culture if febrile
➢ Arterial blood gases

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• Imaging:
➢ Chest X-ray
➢ X-ray of skull
o Shows hair on end appearance.
o This is caused by marrow hyperplasia due to chronic
hemolysis so the vertical trabeculae found between the
inner and outer tables of the diploe bones becomes
accentuated thus having the hair on end appearance.
o This is also seen on CT scan and can be also illustrated in
thalassemia and other forms of hemolytic anemias.
➢ MRI: for early bone marrow changes Figure 18: Hair on end
appearance
➢ CT
➢ Technetium 99 scan to detect early osteonecrosis
➢ Transcranial Doppler U/S: to detect risk of stroke in children
➢ Abdominal U/S: cholecystitis, cholelithiasis, or ectopic
pregnancy and to measure splenomegaly and hepatomegaly
➢ ECHO for pulmonary hypertension
• Spirometry
COMPLICATIONS
CENTRAL NERVOUS SYSTEM
• Cerebral infarction: most common finding is obstruction of a distal
intracranial internal carotid artery or a proximal
middle cerebral artery.
➢ Transient ischemic attacks
➢ Stroke
➢ Cerebral hemorrhage
• Seizures
• Diminished cognition
• Coma
• Eyes:
➢ Ptosis
➢ Proliferative retinopathy
➢ Retinal hemorrhages
➢ Retinal detachment

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RESPIRATORY
• Pulmonary emboli
• Fat emboli
• Respiratory distress
• Respiratory failure
• Pulmonary hypertension: NO deficiency by increased free plasma Hb that binds
NO and causes a deficiency.
CARDIOVASCULAR
• Cardiomegaly
• Arrhythmias
• Dilatation of both ventricles and left atrium
• Hemochromatosis and Iron overload cardiomyopathy
• Myocardial infarctions
• Congestive heart failure
• Cor-pulmonale- from pulmonary hypertension
GASTROINTESTINAL
• Cholelithiasis- pigment stones occur as a result of chronic hemolysis
• Chronic hepatomegaly
• Liver dysfunction
• Acute Abdomen-Mesenteric artery blockage
• Autosplenectomy
GENITOURINARY
• Chronic tubulointerstitial nephritis
• Isosthenurea: kidneys lose concentration capacity.
• Priapism
• Impotence
GYNAE
• Menstrual irregularities
• Spontaneous abortion: impaired placental blood flow
• Intrauterine growth retardation
• Pre-eclampsia
• Fetal death

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DERMATOLOGY
• Leg ulcers over the medial or lateral malleoli.
• Poor wound healing
MUSCULOSKELETAL
• Growth and developmental delay: young children are short but regain their height
by adulthood. They however remain normal weight.
• There is often delayed sexual maturation (delayed puberty) which may require
hormone therapy.
• Bones:
➢ Avascular necrosis of head of femur
➢ Compression of vertebrae
➢ Shortening of bones of hands and feet.
➢ Osteomyelitis due mostly to Staphylococcus aureus and Salmonella.
MANAGEMENT AND PROGNOSIS
• Avoid and treat precipitating factors quickly.
• ABCs
• Rehydration- 1.5 x maintenance.
• Pain control: NSAIDs e.g. ibuprofen, acetaminophen, opioids (morphine)
• Oxygen and antibiotics are only given if specifically indicated:
➢ Antibiotics: cefuroxime, amoxicillin/calvulanate, penicillin V, Ceftriaxone,
azithromycin, cefaclor
➢ For acute chest syndrome use macrolides (azithromycin or erythromycin)
• For respiratory distress
➢ Antibiotic coverage
➢ Supplemental oxygen
➢ Partial exchange transfusion
• For splenic sequestration
➢ Repletion of intravascular volume
➢ Severe anemia- transfuse
• For priapism
➢ Analgesia
➢ Hydration
➢ Partial exchange transfusion

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• Transfusion (15-20ml/kg maximum of Packed red cells): for sudden, severe
anemia due to acute splenic sequestration, aplastic crisis or hyper-hemolytic
crisis.
• Outpatient:
➢ Review regularly with FBC
➢ Prophylaxis with penicillin 500mg daily and vaccination with polyvalent
pneumococcal, meningococcal and Haemophilus influenza type b vaccine.
➢ Folic acid is given in all patients with hemolysis. 1mg/day
➢ Hydroxyurea has be given to patients with SCD (with CVA for example), it
increases HbF but has carcinogenic effect (leukemia) aside this HbF has
higher affinity to oxygen so there is decreased oxygen delivery to tissue (tissue
hypoxia).
o 15mg/kg/day increased by 2.5mg over 12 weeks depending on response.
• Consider bone marrow transplant for severe cases. Bone marrow transplantation
is the only cure however not all patients are eligible for transplant.
• Median life expectancy is in the mid-40s.

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 CHAPTER 11: GASTROENTEROLOGY

GASTROENTEROLOGY
MALNUTRITION

MALNUTRITION
• Nutrition is the provision of adequate energy and nutrients to meet the metabolic
demands of the body.
• Essential nutrients cannot be synthesized by the body and must be derived from
the diet. They include certain vitamins, minerals, amino acids, fatty acids and
carbohydrates.
• Non-essential nutrients can be synthesized from other compounds or may be
derived from the diet.
• Macronutrients supply energy and essential nutrients needed for growth,
development, disease prevention and activity.
• Malnutrition is defined as the cellular imbalance between supply of nutrients and
energy and the body’s demand for them to ensure growth, maintenance and
specific function. (WHO)
• Malnutrition is the result of a lack or an excess in the provision of energy and/or
nutrients to the body (undernutrition or overweight/obesity).
• The term malnutrition thus refers to both undernutrition and overnutrition.
TYPES OF MALNUTRITION
• These include:
➢ Undernutrition:
o Protein-energy malnutrition (PEM)
o Micronutrient deficiencies
➢ Over-nutrition:
o Overweight and obesity
➢ Co-existence of under and over-nutrition “double burden of malnutrition”
o Obesity and PEM
o Obesity and micronutrient deficiencies in the same individual
➢ Acute and chronic malnutrition

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UNDERNUTRITION
• There is inadequate consumption, poor absorption or excessive loss of nutrients.
• The terms protein energy malnutrition (PEM) and malnutrition are sometimes
used interchangeably with undernutrition.
• Undernutrition is the lack of macronutrients (calories) or a lack of micronutrients
(vitamins, minerals).
• Malnourished children may suffer from numerous associated complications.
• They are susceptible to infections, especially sepsis, pneumonia and
gastroenteritis.
• Vitamin deficiencies and deficiencies of minerals and trace elements can also be
seen.
• The most immediate consequence of undernutrition is premature death.
• By 1 year of age, undernutrition is likely to have caused significant damage that
can affect future health, cognition, welfare and well-being.
• Malnutrition in young children is conventionally determined through
measurement of:
➢ Height,
➢ Weight,
➢ Skinfold thickness (or subcutaneous fat)
➢ Age.
CLASSIFICATION OF UNDER-NUTRITION
• Malnutrition is classified in different ways including:
➢ According to anthropometric measurements:
o Basic groupings:
▪ Wasted (Low weight for height)
▪ Stunted (Low height for age)
▪ Undernourished/Underweight (Low weight for age)
o WHO classification
ANTHROPOMETRIC MEASUREMENTS
BASIC GROUPINGS OF MALNUTRITION
• Weight-for-age (WA), height-for-age (HA) and weight-for-height (WH) are the
3 anthropometric indices used in assessing nutritional status.
• Wasting: child is thin, he/she has lost fat and muscle mass so they have a low
weight for height (below -2SD the median weight for height).

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 ➢ Usually due to a recent lack of food or illness (infections) that prevents the
child from eating or absorbing nutrients of foods.
➢ Wasting is an indicator of acute undernutrition.
• Stunting: child is short in stature, She/he did not grow in length/height as he/she
should have (Low height for age below 2SD the median weight)
➢ This is a long-term process, often starting in utero which is due to mother’s
malnutrition, food intake lacking quality (insufficient intake of essential
micronutrients) and the repetition of episodes of common infections.
➢ Stunting is an indicator of chronic malnutrition
• Underweight/undernourished: child weighs less than they should (low weight for
age)

Figure 19: Basic groupings of malnutrition

• Note: A child can be both wasted and stunted.

• Worldwide- 20% of children below 5 years are wasted and 32% are stunted.
WATERLOW CLASSIFICATION
• Classification by weight for height or by Height for age.
➢ 80-90% below median WH is considered mild malnutrition
➢ 70-80% below median WH is considered moderate malnutrition
➢ <70% below median WH is considered severe malnutrition

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GOMEZ CLASSIFICATION
• Uses weight for age.
➢ Over 90% is normal compared to mean WA.
➢ 76-90% is mild malnutrition (First degree)
➢ 61-75% is moderate malnutrition (Second degree)
➢ Less than 60% is severe malnutrition (Third degree)

WELCOME CLASSIFICATION
• The welcome classification is a clinical classification for children admitted to a
hospital or a nutrition unit.
➢ It is based upon Weight for age (WA).
➢ A child with a WA greater than 60% with edema has kwashiorkor.
➢ A child with a WA less than 60% and no edema is said to have marasmus.
Weight/Age With edema Without
edema
60-80% of Kwashiorkor Undernutrition
expected
weight
<60% of Marasmic Marasmus
expected kwashiorkor
weight

WHO CLASSIFICATION
• It uses weight-for-height Z scores, Mid-upper arm circumference (MUAC) and
the presence of edema.
• The WHO recommends the use of Z scores or standard deviation scores (SDS)
for evaluating anthropometric data.
• SD score is defined as the deviation of the value for an individual from the median
value of the reference population divided by the standard deviation of the
reference population.
𝑂𝑏𝑠𝑒𝑟𝑣𝑒𝑑 𝑣𝑎𝑙𝑢𝑒−𝑀𝑒𝑑𝑖𝑎𝑛 𝑟𝑒𝑓𝑒𝑟𝑒𝑛𝑐𝑒 𝑣𝑎𝑙𝑢𝑒
• SD score=
𝑆𝑡𝑎𝑛𝑑𝑎𝑟𝑑 𝑑𝑒𝑣𝑖𝑎𝑡𝑖𝑜𝑛 𝑜𝑓 𝑟𝑒𝑓𝑒𝑟𝑒𝑛𝑐𝑒 𝑝𝑜𝑝𝑢𝑙𝑎𝑡𝑖𝑜𝑛
• The calculation gives a numerical score indicating how far away from the 50 th
centile for the child’s measurements fall.
➢ -2 to -3 SDS indicates moderate malnutrition

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 ➢ <-3 SDS indicates severe malnutrition
➢ +2 to +3 SDS indicates overweight
➢ >+3 indicates obesity
AGE INDEPENDENT INDICES OF MALNUTRITION
• These include:
➢ Mid-upper arm circumference (MUAC)
➢ Skinfold thickness
MID-UPPER ARM CIRCUMFERENCE (MUAC)
• MUAC increases rapidly in the first year (11-16cm) and then is relatively stable
between ages 1 and 5 years at a value of between 16 and 17cm.
• Any value below 13.5cm is abnormal and suggestive of malnutrition.
• A value below 11.5cm is suggestive of severe malnutrition.
• Methods of assessing MUAC:
➢ Measuring tape method
➢ Shakir tape method: this is a special tape with colored zones i.e. red, yellow
and green corresponding to <12.5cm (wasted), 12.5 to 13.5cm (borderline)
and over 13.5cm (normal) MUAC respectively
➢ Bangle test: a bangle with internal diameter of 4 cm is passed above the elbow.
In severe malnutrition it can be passed above the elbow, in normal children it
cannot.
SKINFOLD THICKNESS
• It is an indication of subcutaneous fat. Triceps skin fold is the most representative
of the total subcutaneous fat up to 16 years of age.
• It is usually above 10mm in normal children whereas in severely malnourished it
may fall below 6mm.
WHO CLASSIFICATION
Acute malnutrition MUAC (cm) Weight/height Z score
None >13.5 >-1 SD
Mild (at risk) 12.5-13.4 -2 to -1 SD
Moderate 11.5-12.4 -3 to -2 SD
Severe <11.5 < -3SD
Presence of bipedal edema

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SEVERE ACUTE MALNUTRITION
• In a child 6-59 months it is defined by:
➢ Severe visible wasting
➢ Presence of bipedal edema
➢ Weight-for height below-3 standard deviation of median WHO growth
reference
➢ Mid upper arm circumference < 11.5cm (in children below 6 months of age,
the MUAC cannot be used and SAM should be diagnosed in the presence of
the other parameters).
• In child <6 months:
➢ Severe visible wasting
➢ Presence of bipedal edema
➢ Weight-for height below-3 standard deviation of median WHO growth
reference
➢ Infant too weak or feeble to suckle effectively.
• No distinction is made between the clinical conditions of kwashiorkor or severe
wasting because their treatment is similar.
• Children who are <-3SD weight-for-age may be stunted (short stature) but not
severely wasted.
• Stunted children who are not severely wasted do not require hospital admission
unless they have a serious illness.
• The causes of malnutrition can be viewed as immediate, underlying and basic:
➢ Immediate determinants: these are immediate determinants of a child’s
nutritional status, they include:
o low birthweight (infants born small, remain small)
o Infection (e.g. diarrhea and pneumonia and other infections that consume
energy and hamper growth, nutrition loss in stool) and
o Inadequate dietary intake: delayed complementary feeding and inadequate
intake of food.
➢ Underlying determinants: include food (insufficient quality, quantity and
variety), health (Access to curative and preventive health services) and care
(Availability of food)
➢ Basic determinants: social economic status, education level of the family,
woman’s empowerment, cultural taboos regarding food and health, access to
water and sanitation etc.

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• Children with SAM should be assessed with a full clinical examination to confirm
whether they have any general danger sign, medical complications and an
appetite.
• Uncomplicated SAM
➢ Child >6 months
➢ Child is alert
➢ Preserved appetite
➢ Clinically assessed to be well
➢ Child is living in a conducive home environment
➢ Can be managed as outpatient
• Complicated SAM
➢ Child <6 months
➢ Not alert
➢ Having loss of appetite
➢ >6 months but not clinically well
➢ Institutional care is considered mandatory
CLINICAL FEATURES OF MALNUTRITION
• Clinical features include
➢ Hair changes
➢ Eye signs
➢ Apathy
➢ Reduced cardiac function
➢ Shock
➢ Anemia
➢ Diarrhea and GIT features
➢ Skin changes
➢ Muscle wasting
➢ Edema
➢ Recurrent infections
➢ Micronutrient deficiencies
HAIR CHANGES
• Hair is Dull, lusterless and hypopigmented. There is dyspigmentation, loss of
characteristics curls and sparseness over temple and occipital regions
• Keratin synthesis impaired due to cysteine and methionine deficiency, thus brittle
hair easily pulled off/breaks.

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• Pigment melanin formed from tyrosine so its deficiency leads to hair color
changes i.e. reddish or grey.
• Periods of good nutrition alternating with poor nutrition results in a flag sign
(alternating bands of hypopigmented and normally pigmented hair).
• Dullness and lack of luster due to weathering of hair cuticle.
EYE SIGNS
• Due to vitamin A deficiency
• Presents as
➢ Photophobic (keep the eyes closed)
➢ Dry conjunctiva or cornea
➢ Bitot spots
➢ Corneal ulceration or keratomalacia
APATHY
• There is unhappiness, apathy or irritability with sad, intermittent cry. They show
no signs of hunger and it is difficult to feed them
• Seen in kwashiorkor, it is due to
➢ Hypokalemia- muscle weakness and easy fatigability of muscles
➢ Lack of stimulation and deprivation causes reduced growth of brain and
nerves thus there is mental slowing
➢ Zinc deficiency
➢ Reduce basal metabolic rate
REDUCED CARDIAC FUNCTION
• Cold, pale extremities due to circulatory insufficiency and are associated with
prolonged circulation time, bradycardia, diminished cardiac output and
hypotension.
• Selenium deficiency may be associated with reduced cardiac muscle function.
• Arrhythmias are due to potassium and magnesium deficiency.
SHOCK
• Child is lethargic or unconscious with cold peripheries, delayed capillary refill
time (>3s) or weak rapid pulse or low blood pressure.
• Signs of dehydration indicate hypovolemia or septic shock
• If severe pallor is present this is due to anemia
• If signs of Congestive cardiac failure are present suspect cardiogenic shock.

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ANEMIA
• Due to:
➢ Lack of iron, folic acid and vitamin B12 (hyperpigmented skin)
➢ Parasitic infestations e.g. hookworm.
➢ Malabsorption due to recurrent diarrhea.
➢ Inadequate copper intake.
GASTROINTESTINAL FEATURES
• Mucous membrane lesions: Smooth tongue, cheilosis, and angular stomatitis are
common. Herpes simplex stomatitis may also be seen
• Anorexia, sometimes with vomiting is the rule. Abdominal distension is
characteristic. Stools may be watery or semisolid, bulky with a low pH and
contain unabsorbed sugars.
• Diarrhea:
➢ Recurrent infection due to decreased immunity- decreased secretory IgA
levels and decreased secretion of stomach acid
➢ Malabsorption- pancreatic enzyme deficiency resulting from pancreatic
atrophy/protein deficiency
➢ Due to zinc deficiency, reduced gastrointestinal function leads to chronic
diarrhea
• Potbelly:
➢ Hypotonic muscles of abdominal wall resulting from muscle wasting
➢ Paralytic ileus due to hypokalemia
➢ Overgrowth of bacteria in the gut due to decreased immunity
➢ Hepatomegaly because of fatty liver
o Free radicals damage reticuloendothelial enzymes in the live causing
decreased synthesis of proteins.
o Beta lipoprotein deficiency results in accumulation of triglycerides in the
liver (fatty liver hepatomegaly)
SKIN CHANGES
• Flaky paint dermatosis- hyperpigmentation, desquamation area (flake) over raw
skin (due to zinc and niacin deficiency).
• Crazy pavement dermatosis: dry, hyperkeratotic, fissured skin with alternate
areas of hyper and hypopigmentation.
• Mosaic dermatosis: mixed lesions in mosaic form
• Atrophy of sweat and sebaceous glands leads to excessive dryness of skin

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• Acrodermatitis enteropathica due to zinc deficiency
• Skin changes are usually seen on the perineum, groin, limbs, behind the ears and
in the armpits.
• Grading dermatosis
➢ Mild: discoloration or a few rough patches of skin
➢ Moderate: multiple patches on arms and/or legs
➢ Severe: flaking skin, raw skin, fissures (opening in the skin)
MUSCLE WASTING
• Severe wasting is due to:
➢ Calorie deficiency- fats and tissue proteins mobilized to supply energy for
metabolic processes
➢ Recurrent infection coupled with hypoglycemia cause acute stress response-
cortisol released-wasting
➢ Effects of associated infections e.g. HIV wasting syndrome, TB
• It is important to remove the child’s cloths in order to examine for severe wasting.
A severely wasted child will have the following signs:
➢ Outline of the child’s ribs easily seen
➢ The skin of the upper arms look loose
➢ The ribs and shoulder bones easily seen
➢ Flesh missing from the buttocks
➢ Baggy pants appearance.
➢ Loss of subcutaneous tissue on cheeks and face (previously referred to as
‘monkey facies’.
EDEMA
• Usually seen in marasmus as bilateral nutritional pitting edema. If swelling is
only in one foot it may just be an infected foot.
• It is due to:
➢ Protein deficiency: hypoalbuminemia, reduced plasma oncotic pressure and
fluid shift the interstitium
➢ Free radical damage of cell membrane- Na/K ATPase malfunction- fluid leaks
➢ Hypovolemia (due to vomiting and diarrhea)- reduces Glomerular filtration
rate (GFR) and activates the renin-angiotensin-aldosterone system causing
sodium and water retention
➢ Increased levels of leukotrienes causing uncontrolled vasodilation,
hypovolemia and increased tubular reabsorption of salt and water
• Grading edema

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 ➢ Grade 1 (mild): both feet and ankle
➢ Grade 2 (moderate): both feet plus lower legs, hands with arms
➢ Grade 3 (severe): limbs and trunk
➢ Grade 4 (very severe): anasarca (whole body) from limbs to face
RECURRENT INFECTION
• Decreased immune response due to inability to synthesize IL-1, IL-6, TNF-alpha
due to lack of essential amino acids.
• C3, C5 and factor b levels reduced- opsonization and phagocytosis are reduced
• Decreased phagocytic and bactericidal activity of leucocytes- NADPH oxidase
and lysozyme deficiency.
• Atrophy of thymo-lymphatic glands causes depletion of T-lymphocytes and
depressed cell mediated immunity thus infections like herpes, candidiasis are
common.
• Immunosuppression is seen in malnutrition and changes correlate with poor
outcomes and mimic the changes observed in children with AIDS. (Loss of
delayed hypersensitivity, few T lymphocytes, impaired lymphocyte response,
impaired phagocytosis secondary to decreased complement and certain cytokines
and decreased secretory IgA)
• This predispose the child to severe and chronic infections such as infectious
diarrhea which further compromises nutrition causing anorexia, decreased
nutrient absorption, increased metabolic needs and direct nutrient losses.
MICRONUTRIENT DEFICIENCIES
• Iron (microcytic anemia): fatigue, anemia, decreased cognitive function,
headache, glossitis and koilonychias
• Folate- glossitis, anemia (megaloblastic), neural tube defects (in fetuses of
women without folate supplementation)
• Iodine- goiter, developmental delay and cognitive impairment
• Zinc- anemia, dwarfism, hepatosplenomegaly, hyperpigmentation and
hypogonadism, acrodermatitis enteropathica, diminished immune response, poor
wound healing. Zinc is important during rehydration and refeeding process.
Urinary zinc is proportional to overall zinc status.
• Vitamin A- night blindness, xerophthalmia, keratinous changes of cornea and
conjunctiva, skin keratinization, poor growth and hair changes.
• Vitamin D- poor growth, rickets and hypocalcemia

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MARASMUS AND KWASHIORKOR
• Usually associated with one of classical syndromes, namely marasmus,
kwashiorkor or with manifestations of both.
• Kwashiorkor and marasmus are 2 forms of PEM described.
• The distinction between the 2 forms of PEM is based on the presence
(Kwashiokor) or absence (marasmus) of edema.
• Marasmus involves inadequate intake of protein and calories (total caloric
deficiency)
• Kwashiorkor has inadequate protein intake.
• Although significant clinical differences between kwashiorkor and marasmus
exist, some studies suggest that marasmus represents an adaptation to starvation
whereas kwashiorkor represents a dysadaptation to starvation.
• In addition to PEM, children may be affected by micronutrient deficiencies that
can have detrimental effects on growth and development.
• The most common and clinically significant micronutrient deficiencies in
children and childbearing women worldwide are iron, iodine, zinc and vitamin
A.
• Marasmus (total caloric deficiency):
➢ Results from rapid deterioration in nutritional status.
➢ Acute starvation or acute illness over a borderline nutritional status could
precipitate this form of undernutrition
➢ It is marked by wasting of fat and muscle as tissues are consumed to make
energy.
➢ The main sign is severe wasting. The child appears very thin (skin and bones)
and has no fat. There is severe wasting of the shoulders, arms, buttocks and
thighs.
➢ The loss of buccal pad of fat creates the aged or wrinkled appearance that has
been referred to once as monkey facies.
➢ Baggy pants appearance refers to loose skin of the buttocks hanging down.
Axillary pad of fat may also be diminished.
➢ Affected children may appear to be alert in spite of their condition.
➢ There is no edema.

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Figure 20: An 8 year old child with SAM. Note in (A) dull, lustreless, sparse hair, temporal hollowing, loss
of buccal pad of fat, and in (B) loose folds of skin in the gluteal region giving a 'baggy pant' appearance

• Kwashiorkor
➢ Usually affects children aged 1-4 years.
➢ Main sign is pitting edema, usually starting in the legs and feet and spreading
in more advanced cases to the hands and face. Because of the edema, children
with kwashiorkor may look healthy so that their parents view them as well
fed.
➢ General appearance: child may have a fat sugar baby appearance
➢ Edema ranges from mild to gross and may represent 5-20% of the body weight
➢ Muscle wasting: always present. Child is often weak, hypotonic and unable to
stand or walk
Clinical finding Marasmus Kwashiorkor
Deficiency Total caloric deficiency Protein deficiency
Occurrence More common Less common
Appearance Weight loss and muscle Weight loss, lethargy,
wasting irritability
Simian facies- Moon facies
cachectic/old man face
Patient appears Patient appears volume
dehydrated overloaded
Edema Absent, loss of skin Present with flabby skin tissue
turgor/wrinking
Activity Active Apathetic
Appetite Good Poor
Liver enlargement Absent Present,
hepatomegaly/hepatosteatosis-

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 fatty liver due to decreased
apolipoprotein synthesis and
accumulation of VLDL in the
liver (fatty liver). Vacuoles are
seen on microscopy
GIT Constipation or Diarrhea, vomiting
starvation diarrhea
Muscle tone Low muscle Loss of muscle tone
tone/hypotonic
Other features Hypothermia Dermatitis-
hyperpigmentation,
desquamation (decrease in the
enzymes necessary for skin
function)
Anemia- decreased globin
Mortality Less than kwashiorkor High in early stage
Recovery Recover early Slow recovery
Infections Less prone More prone
• Marasmic kwashiorkor: it is a mixed form of PEM and manifests as edema
occurring in children who may or may not have other signs of kwashiorkor and
have varied manifestations of marasmus.
• Other symptoms of malnutrition may be present: hypocalcemia (positive
chvostek/trousseau sign), skeletal deformities consistent with rickets, scurvy
etc.
ASSESSMENT AND DIAGNOSIS OF MALNUTRITION
• Malnutrition must be recognized and accurately defined for rational decisions to
be made about refeeding.
• Evaluation is divided into:
➢ Assessment of past and present dietary intake (History)
➢ Anthropometry (Examination)
➢ Laboratory assessments
HISTORY
• Clinical signs and symptoms of malnutrition:
➢ Poor weight gain
➢ Slowing of linear growth
➢ Behavioral changes- irritability, apathy, decrease social responsiveness,
anxiety and attention deficits

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• Clinical signs and symptoms of micronutrient deficiency:
➢ Iron: fatigue, anemia, decreased cognitive function, headache, glossitis and
koilonychias
➢ Iodine- goiter, developmental delay and mental retardation
➢ Vitamin D- poor growth, rickets and hypocalcemia
➢ Vitamin A- night blindness, xerophthalmia, poor growth and hair changes.
➢ Folate- glossitis, anemia (megaloblastic), neural tube defects (in fetuses of
women without folate supplementation)
➢ Zinc- anemia, dwarfism, hepatosplenomegaly, hyperpigmentation and
hypogonadism, acrodermatitis enteropathica, diminished immune response,
poor wound healing.
• History of presenting complaint
➢ Usual diet before current episode of illness
➢ Breastfeeding history
➢ Food and fluid intake taken in past few days
➢ Recent sinking of eyes
➢ Time when urine was last passed
➢ History of body hotness or febrile illness
➢ Presence of diarrhea (duration, watery/bloody)
➢ Information on vomiting, loss of appetite, cough
• Review of systems:
➢ CNS: irritability, apathy, decreased social responsiveness, anxiety and
attention deficits.
➢ CVS: easy fatigability, palpitation, ankle or leg swelling, dyspnea and
palpitations
➢ Respiratory system: Cough >12 weeks
➢ GIT:
o Vomiting & diarrhea
▪ Duration and frequency
▪ Type of diarrhea (watery/ bloody)
▪ Loss of appetite
➢ GUT: Time when urine was last passed
➢ Musculoskeletal and skin: poor growth, pathological fractures, rickets, dry
peeling skin with raw exposed areas, and hyperpigmented plaques over areas
of trauma
• Past medical history:
➢ Contact with TB

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 ➢ Recent contact with measles
➢ Known or suspected HIV infection/exposure
• Birth history: birth weight
• Immunization history
• Nutritional history:
➢ Parents are asked to record the food the child eats during several days.
➢ This gives a guide to food intake.
➢ Breast feeding
• Developmental history: milestones reached
• Family history: any recent death of sibling, history of TB and measles.
• Social economic history
➢ Employment status of parents
➢ Education level of parents
➢ Situation at home (who takes care and feeds child)
EXAMINATION
• On examination look for:
➢ Shock: lethargic or unconscious, with cold hands, slow capillary refill (>3s)
or weak (low volume), rapid pulse and low blood pressure
➢ Signs of dehydration
➢ Severe palmar pallor
➢ Bilateral pitting edema
➢ Eye signs of vitamin A deficiency:
o Dry conjunctiva or cornea, Bitot spots (these are greyish foamy patches on
the exposed bulbar conjunctiva they are due to build-up of keratin in
Vitamin A deficiency)
o Corneal ulceration
o Keratomalacia: softening of the cornea, followed by perforation of the
eyeball and permanent blindness (extreme care must be taken during
ophthalmic examination at this stage, due to risk of rupturing cornea)
➢ Localizing signs of infection, including ear and throat infections, skin
infection or pneumonia.
➢ Signs of HIV infection
➢ Fever (temperature >37.5oC) or hypothermia (rectal temperature <35.5oC,
Axillary temperature <35oC)
➢ Mouth: angular stomatitis, cheilosis, smooth tongue
➢ Skin changes of kwashiorkor

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 o Hypo-or hyperpigmentation
o Desquamation
o Ulceration (spreading over limbs, thighs, genitalia, groin and behind the
ears)
o Exudative lesions (resembling severe burn) often with secondary infection
(including Candida)
➢ Conduct an appetite test:
o Check if the child has appetite by providing ready to use therapeutic food.
ANTHROPOMETRY
• In addition to weight and height, skinfold thickness of the triceps reflects (mid-
arm circumference) subcutaneous fat stores and can be assessed by measuring it.
➢ Weight for length/height <-3SD (wasted)
➢ Mid upper arm circumference <11.5cm
➢ Edema of both feet (kwashiorkor with or without severe wasting)
PHYSICAL FINDINGS
• Hair changes: hair is thin, sparse, brittle, easily pulled out, flag-post hair, and
turns a dull brown or reddish color.
• Decreased subcutaneous tissue: legs, arms, buttocks and face
• Oral changes: cheilosis, angular stomatitis, papilla atrophy
• Edema: distal extremities and anasaraca (generalized edema)
• Nail changes: nails become fissured or ridged.
• Abdominal findings: abdominal distension secondary to poor abdominal
musculature, hepatomegaly secondary to fatty infiltration
• Skin: dry peeling skin with raw exposed areas, hyperpigmented plaques over
areas of trauma
LABORATORY INVESTIGATIONS
• Essential for detection of early physiological adaptation to malnutrition.
• Clinical history, examination and anthropometry are of greater value than any
single biochemical or immunological measurement.

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• Lab investigations:
➢ Blood investigations
o Full blood count: check for anemia, and
infections. There may be low lymphocyte
count indicating an impaired cell-mediated
immunity
o ESR: may be raised if there is an
underlying inflammatory process
o Malaria slide (thick and thin smear)
o Blood culture
o Urea and electrolytes (calcium,
magnesium and phosphate levels are low),
creatinine
o HIV test
o X-match blood
Figure 21: Nutritional assessment in malnutrition
o Liver enzymes
o Total protein and Serum albumin
o Clotting profile
o Assay of specific minerals and vitamins
o Thyroid function tests (low T3 and 4, High TSH)
➢ Urine microscopy/culture/sensitivity
➢ Gastric lavage for Acid fast bacilli (TB)
➢ Stool studies for GI infection
• Other labs are indicated by history and physical examination findings e.g. lumbar
puncture, chest X-ray
• Children with severe acute malnutrition should first be assessed with a full
clinical examination to confirm whether they have any general danger sign,
medical complications and an appetite.
• Children with severe acute malnutrition with loss of appetite or any medical
complication have complicated severe acute malnutrition and should be admitted
for inpatient care.
• Children who have a good appetite and no medical complications can be managed
as outpatients.

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APPETITE TEST
• This is conducted on children with SAM to determine the phase of treatment.
Children who pass and have no other medical complications may be started on
phase 2 treatment while those that fail are stated on phase 1.
• The appetite feed (energy protein diet-EPD) contains:
➢ Roasted ground nuts 1000g
➢ Milk powder 1200g
➢ Sugar 1120g
➢ Coconut oil 600g
• How to prepare the EPD
➢ Take roasted ground nuts and grind them in a mixer
➢ Grind sugar separately or with roasted groundnuts
➢ Mix ground nuts, sugar, milk powder and coconut oil
➢ Store them in air tight container and in a refrigerator
➢ Prepare only for 1 week to ensure the quality of feeds
• How to perform an appetite test
➢ Do the test in a separate quiet room
➢ Explain to the mother/caregiver how the test will be done
➢ Mother/caregiver should wash their hands
➢ The mother then sits comfortably and with the child on her laps offers the
therapeutic food.
➢ Child should not have taken any food for last 2 hours
➢ The test usually takes a short time but may take up to an hour.
➢ The child must not be forced to take the offered food
➢ When the child has finished the EPD, the amount taken is judged/measured.
• The test is failed if the child does not eat the amount of EPD according to weight
at the end of an hour:
BODY WEIGHT (Kg) EPD (grams)
3-3.9 <15
4-6.9 <20
7-7.9 <25
8-8.9 <30
10-11.9 <35
12-14.9 <40
• Note:
➢ Those that pass without any other medical complication are started on F100
and locally made special feed (phase 2).

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 ➢ Those that fail (with one or more danger sign or medical complication) are
treated with F-75 and 10 steps of management of SAM (Phase 1)
GENERAL MANAGEMENT SEVERE ACUTE MALNUTRITION
• The general treatment involves 10 steps in 3 phases:
➢ Stabilization phase
➢ Transition phase
➢ Rehabilitation phase
STABILIZATION PHASE
• Focuses on restoring homeostasis and treating medical complications and usually
takes 1-2 days of inpatient treatment.
• Children with SAM and a failed appetite test and/or a major medical complication
are stabilized first by 10 steps of management.
• F-75 (starter diet) is used which promotes recovery of normal metabolic function
and nutrition- electrolyte balance.
• During this phase the child should be monitored for signs of overfeeding and
dehydration.
TRANSITION PHASE
• Subsequent part of stabilization phase
• Lasts 3-7 days
• It is intended to ensure the child is clinically stable and can tolerate an increased
energy and protein intake.
• The only difference is change in the type of diet
➢ Transition gradually from starter F75 to catch up F100
➢ Quantity of F100 given is equal to the quantity of F75 in the stabilization
phase
• The child moves to the transition phase from stabilization when:
➢ At least the beginning of loss of edema
➢ Return of appetite
➢ No nasogastric tube feeds
➢ No severe medical problems
➢ Child is alert and active
REHABILITATION
• The rehabilitation phase focuses on rebuilding wasted tissues and may take
several weeks.

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• It can take from 2 to 6 weeks.
• The child progresses from transition phase to rehabilitation phase when:
➢ Child has reasonable appetite
➢ Finishes >90% of the feed given without significant pause
➢ Major reduction or loss of edema
➢ No other medical complications
• The aim is to promote rapid weight gain, stimulate emotion and physical
development and prepare the child for normal feeding at home
• Principles of management involves correction and prevention of:
1. Hypoglycemia
2. Hypothermia
3. Dehydration
4. Electrolytes
5. Infections
6. Micronutrients
7. Initiate feeding
8. Catch-up feeding
9. Sensory stimulation
10.Prepare for follow up

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HYPOGLYCEMIA
• All severely malnourished children are at risk of hypoglycemia and immediately
on admission, should be given a feed or 10% glucose or sucrose. Frequent 2
hourly feeding is important.
• Diagnosis:
➢ Blood glucose <3mmol/litre (<54mg/dl)
➢ If blood glucose cannot be measured it should be assumed that all children
with SAM are hypoglycemia and given treatment.
➢ Hypoglycemia may be asymptomatic or symptomatic. Symptomatic
hypoglycemia manifesting as lethargy, unconsciousness, seizure, peripheral
circulatory failure or hypothermia is more common in marasmus, where
energy stores are depleted or when feeding is infrequent. Sweating and pallor
may not occur in malnourished children with hypoglycemia

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• Treatment:
➢ For asymptomatic hypoglycemia: give 50ml of 10% dextrose or sucrose
solution (1 rounded teaspoon of sugar in 3½ tablespoons of water) orally or
by NGT followed by the first feed as soon as possible.
➢ For symptomatic hypoglycemia: 5ml/kg of 10% dextrose should be given
intravenously. This should be followed with 50ml of 10% dextrose or sucrose
solution by nasogastric tube.
➢ Give the first feed of F-75 therapeutic milk, if it is quickly available and then
continue with feeds every 2h for 24h, then continue feeds every 2h or 3h, day
and night.
o F-75 (Formula 75 contains 75 Kcal/100ml of feed) as quickly as possible
and then continued 2-3 hourly day and night.
o Initially a quarter of the 2 hourly feed should be given every 30 minutes
till the blood glucose stabilizes
➢ If IV glucose is not available, give one teaspoon of sugar moistened with 1 or
2 drops of water sublingually and repeat every 20 min to prevent relapse.
Children should be monitored for early swallowing which leads to delayed
absorption, in this case another dose of sugar should be given. Continue with
2h oral or NGT feeds to prevent recurrence
➢ Hypoglycemia, hypothermia and infection generally occur as a triad.
➢ Start on appropriate IV or IM antibiotics.
• Monitoring
➢ Repeat glucose measurement every 30min until glucose level is normal and
stabilizes.
➢ If blood glucose <3mmol/l (<54 mg/dl) repeat the 10% glucose or oral sugar
solution.
➢ If the rectal temperature falls <35.5oC or if level of consciousness deteriorates
repeat glucose measurement and treat accordingly.
➢ Once glucose level is stable the 2 hourly feeding regimens should be started
• Prevention
➢ Frequent feeding every 2h starting immediately or when dehydrated,
rehydrate first. Continue feeding throughout the night.
➢ Encourage mothers to watch for any deterioration, help feed and keep the
child warm.
➢ Check on abdominal distention.

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 HYPOGLYCEMIA IN MALNUTRITION MANAGEMENT BOX
• Blood glucose level <3mmol/L (<54 mg/dl)
• If glucose can’t be done, assume child has hypoglycemia
• Hypoglycemia, hypothermia and infection generally occur as a triad
• Treatment:
➢ Asymptomatic hypoglycemia:
o Give 50ml of 10% glucose or sucrose solution orally or by NGT
followed by first feed
o Feed with starter F-75 every 2 hourly day and night
➢ Symptomatic hypoglycemia
o Give 10% dextrose IV 5ml/kg
o Follow with 50ml of 10% dextrose or sucrose solution by
nasogastric tube
o Feed with starter F-75 every 2 hourly day and night
➢ Start appropriate antibiotics
• Prevention
➢ Feed 2 hourly starting immediately
➢ Prevent hypothermia

HYPOTHERMIA
• This is very common in malnourished children and often indicates coexisting
hypoglycemia or serious infection.
• It is due to impairment of thermoregulatory insulation from body fat.
• Children with marasmus, concurrent infections, denuded skin and infants are at
greater risk.
• Diagnosis
➢ Axillary <35oC or does not register on a normal thermometer, assume
hypothermia.
➢ When a low-reading thermometer is available take rectal temperature
(<35.5oC) to confirm hypothermia
• Treatment:
➢ All children with hypothermia should be treated routinely for hypoglycemia
and infection.
➢ Feed the child immediately and then every 2h unless they have abdominal
distention, if dehydrated, rehydrate first.
➢ Re-warm child:

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 o Provide heat using radiation (overhead warmer) or conduction (skin
contact) or convection (heat convector).
o Rapid rewarming may lead to disequilibrium and should be avoided.
o Ensure child is clothed (especially the head), cover with a warmed blanket
and place a heater (not pointing directly at the child) or lamp nearby, or
put the child on the mother’s bare chest or abdomen (skin-to-skin) and
cover them with a warmed blanket and/or warm clothing.
➢ Keep the child away from draughts.
➢ In cases of severe hypothermia (rectal temperature <32OC):
o Give warm humidified oxygen
o Give 5ml/kg of 10% dextrose IV or 50ml of 10% dextrose by NGT (if IV
access is difficult).
o If clinical condition allows the child to take orally, warm feeds should be
given immediately or else the feeds should be given by NGT.
o Avoid rapid rewarming. Provide heat using radiation (overhead warmer)
or conduction (skin contact) or convection (heat convector)
o Monitor temperature every 30 minutes.
o If there is feed intolerance or another contraindication for NGT feeding,
maintenance IV fluids (prewarmed) should be started.
➢ Give appropriate IV or IM antibiotics
➢ Note: hypothermia and hypoglycemia may be a feature of underlying
infection
• Monitoring
➢ Rectal temperature 2 hourly until it rises to >36.5oC. Take it every 30 min if
a heater is being used.
➢ Ensure child is covered at all times, especially at night. Keep the head covered,
preferably with a warm bonnet, to reduce heat loss.
➢ Check for hypoglycemia whenever hypothermia is found
• Prevention
➢ Feed immediately and then every 2-3h, day and night.
➢ Place the bed in a warm, draught-free part of the ward and keep the child
covered.
➢ Use the kangaroo care/technique for infants cover with a blanket and let the
mother sleep with child to keep the child warm.
➢ Avoid exposing the child to cold (e.g. after bathing or during medical
examination)

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 ➢ Change wet nappies, clothes and bedding to keep the child and the bed dry.
Dry carefully after bathing but do not bathe if very ill.
➢ Use a heater or incandescent lamp with caution.
➢ Do not use a hot water bottle or fluorescent lamp.

HYPOTHERMIA IN MALNUTRITION MANAGEMENT BOX

• Rectal temperature less than <35.5oC or axillary temperature less than 35oC
• Always measure blood glucose and screen for infections in the presence of
hypothermia
• Treatment:
➢ Clothe the child with warm clothes, ensure that the head is also covered
with a scarf or cap
➢ Provide heat using overhead warmer, skin contact or heat convector
➢ Avoid rapid rewarming as this may lead to disequilibrium
➢ Feed the child immediately
➢ Give appropriate antibiotics
• For severe hypothermia (rectal temperature <32oC)
➢ Give warm humidified oxygen
➢ Give 5ml/kg of 10% dextrose IV or 50ml of 10% dextrose by NGT (if IV
access is difficult).
➢ If clinical condition allows the child to take orally, warm feeds should be
given immediately or else the feeds should be given by NGT
➢ Avoid rapid rewarming. Provide heat using radiation (overhead warmer) or
conduction (skin contact) or convection (heat convector)
➢ Monitor temperature every 30 minutes.
➢ If there is feed intolerance or another contraindication for NGT feeding,
maintenance IV fluids (prewarmed) should be started.
• Prevention
➢ Place the child’s bed in a draught free area
➢ Always keep the child well covered, ensure that head is also covered well
➢ May place the child in contact with the mother’s bare chest or abdomen
(skin to skin)
➢ Feed the child 2 hourly starting immediately after admission

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DEHYDRATION
• Dehydration tends to be over-diagnosed and its severity overestimated in children
with SAM because it is difficult to determine accurately from clinical signs alone.
➢ Loss of elasticity of skin may either be due to loss of subcutaneous fat in
marasmus or loss of extracellular fluid in dehydration
• It is important to note that poor circulatory volume (hypovolemia) or perfusion
can co-exist with edema.
• In dehydration, the oral mucosa feels dry to the palpating finger gently rolled on
the inner side of the cheek.
• Presence of thirst, hypothermia, weak pulses and oliguria are other signs of
dehydration in severely malnourished children.
• NOTE: all star signs of dehydration are unreliable in SAM therefore, assume that
all children with watery diarrhea or reduced urine output have some dehydration.
• Only history of fluid loss and very recent change in appearance can be used
• Dehydration should be corrected slowly over a period of 12 hours.
• Do not use the IV route for rehydration, except in cases of shock.
• Rehydrate slowly either orally or by nasogastric tube using oral rehydration
solution for malnourished children (ReSoMal) (5-10ml/kg per h up to a
maximum of 12 hours)
➢ Give the ReSoMal orally of by NG tube, more slowly than you would when
rehydrating a well-nourished child
➢ Give 5ml/kg every 30 minutes for the first 2hours
➢ Then given 5-10 ml/kg for the next 4-10h on alternate hours with F-75 formula
i.e. Feeds at 2, 4, 6 hours and ReSoMal at 1,3 and 5 hours.
➢ The exact amount depends on how much the child wants, the volume of stool
loss and whether the child is vomiting.
➢ Ongoing stool losses should be replaced with approximately 5-10ml/kg
ReSoMaL per losses stool. The frequent passage of small unformed stools
should not be confused with profuse watery diarrhea as it does not require
fluid replacement.
• The standard WHO ORS solution for general use has a high sodium and low
potassium therefore it is not suitable for severely malnourished children.
• If not available then give half-strength standard WHO oral rehydration solution
with added potassium and glucose as per the ReSoMal recipe unless the child has
cholera or profuse watery diarrhea.

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• If ReSoMal not available use low osmolarity ORS add 15ml of KCL (15ml
contains 20 mEq of potassium) to 1L of ORS.

WHO ORS WHO low ReSoMal

osmolarity ORS
Sodium 90 75 45
Potassium 20 20 40
Glucose 111 75 125

• If rehydration is still required at 10hours give starter F-75 instead of ReSoMal at

the same times. Use the same volume of starter F-75 as of ReSoMal
• If in shock or severe dehydration but cannot be rehydrated orally or by NG tube
give IV fluids either Ringer’s lactate solution with 5% dextrose or half-strength
Darrow’s solution with 5% dextrose. If neither is available, 0.45% saline with
5% dextrose should be used.
➢ Give it as a slow infusion of 15ml/kg over the 1st hour with continuous
monitoring of pulse rate, volume, respiratory rate, capillary refill time and
urine output.
➢ If there is improvement (pulse slows, faster capillary refill) at the end of the
1st hour of IV fluid infusion then a diagnosis of severe dehydration with shock
should be considered and rehydrating fluid repeated at the same rate of
15ml/kg over the next hour. This should follow ReSoMal at 5-10ml/kg/hr
either orally or by NG tube. Patients should be monitored for features of
overhydration and cardiac decompensation.
• If at the end of the 1st hour of IV rehydration there is no improvement or
worsening, septic shock must be considered and appropriate treatment started.
➢ Give maintenance IV 4ml/kg/hr
➢ Review antibiotic treatment
➢ Start dopamine 10g/kg/min
• Monitoring:
➢ Monitor respiration and pulse, they should fall. Urine should also be passed.
The return of tears, a moist mouth, less sunken eyes and fontanelle and
improved skin turgor are also signs that rehydration is proceeding but many
severely malnourished children will not show these changes even when fully
rehydrated.

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 ➢ Monitor rehydration every 30 minutes for 2 hours, then every hour for the next
4-10 hours. Be alert for signs of overhydration, which is very dangerous and
may lead to heart failure. Check for
o Weight gain to ensure that it is not quick and excessive
o Increasing edema and increasing periorbital puffiness
o Increase in respiratory rate (5/min)
o Increase in pulse rate (15/min)
o Urine frequency (has the child urinated since last checked?)
o Enlarging liver size on palpation
o Frequency of stools and vomit
➢ If you find signs of overhydration (Early signs are respiratory rate increasing
by 5/min and pulse rate by 25/min), stop ReSoMal immediately and reassess
after 1 hour.
• Prevention
➢ Measures similar to those used in continuing watery diarrhea in well
nourished children except the ReSoMal fluid is used instead of standard ORS.
➢ Continue breast feeding if child is breastfed.
➢ Initiate re-feeding with starter F-75
➢ Give ReSoMal between feeds to replace stool losses. 5-10ml/kg after each
watery stool.

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DEHYDRATION IN MALNUTRITION MANAGEMENT BOX
• Difficult to diagnose in a severely malnourished child
• Assume all severely malnourished children with watery diarrhea have some
dehydration
• Low blood volume (hypovolemia) can coexist with edema
• Treatment
➢ Rehydrate slowly over 12 hours
➢ Give ReSoMal orally or by NG tube
o Give 5ml/kg every 30 minutes for the first 2hours
o Then given 5-10 ml/kg for the next 4-10h on alternate hours with F-75 formula
i.e. Feeds at 2, 4, 6 hours and ReSoMal at 1,3 and 5 hours.
➢ The exact amount depends on how much the child wants, the volume of stool loss
and whether the child is vomiting.
➢ Ongoing stool losses should be replaced with approximately 5-10ml/kg
ReSoMaL per losses stool. The frequent passage of small unformed stools should
not be confused with profuse watery diarrhea as it does not require fluid
replacement.
➢ Alternative fluids if ReSoMal is absent: half-strength standard WHO oral
rehydration solution with added potassium and glucose as per the ReSoMal recipe
➢ If rehydration is still required at 10hours give starter F-75 instead of ReSoMal at
the same times. Use the same volume of starter F-75 as of ReSoMal
➢ If in shock or severe dehydration but cannot be rehydrated orally or by NG tube
give IV fluids either Ringer’s lactate solution with 5% dextrose or half-strength
Darrow’s solution with 5% dextrose. If neither is available, 0.45% saline with 5%
dextrose should be used
o Give it as a slow infusion of 15ml/kg over the 1st hour with continuous
monitoring of pulse rate, volume, respiratory rate, capillary refill time and
urine output.
o If child improves then diagnosis was likely severe dehydration with shock
so rehydrating fluid repeated at the same rate of 15ml/kg over the next hour.
This should follow ReSoMal at 5-10ml/kg/hr either orally or by NG tube.
• If at the end of the 1st hour of IV rehydration there is no improvement or worsening,
septic shock must be considered and appropriate treatment started.
➢ Give maintenance IV 4ml/kg/hr
➢ Review antibiotic treatment
➢ Start dopamine 10g/kg/min

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 • Monitor
➢ Monitor
o Respiration and pulse, they should fall.
o Urine Output should also be passed.
o The return of tears, a moist mouth, less sunken eyes and fontanelle and
improved skin turgor.
➢ Monitor rehydration every 30 minutes for 2 hours, then every hour for the next
4-10 hours. Be alert for signs of overhydration, which is very dangerous and
may lead to heart failure. Check for
o Weight gain to ensure that it is not quick and excessive
o Increasing edema and increasing periorbital puffiness
o Increase in respiratory rate (5/min)
o Increase in pulse rate (15/min)
o Urine frequency (has the child urinated since last checked?)
o Enlarging liver size on palpation
o Frequency of stools and vomit
➢ If you find signs of overhydration (Early signs are respiratory rate increasing by
5/min and pulse rate by 15/min), stop ReSoMal immediately and reassess after
1 hour.
• Prevention
➢ Continue breast feeding if child is breastfed.
➢ Initiate re-feeding with starter F-75
➢ Give ReSoMal between feeds to replace stool losses. 5-10ml/kg after each
watery stool.

ELECTROLYTE IMBALANCE
• All severely malnourished children have deficiencies of potassium and
magnesium which may take around 2 weeks to correct.
➢ Manifestations of severe hypokalemia:
o Weakness of abdominal, skeletal and even respiratory muscles that may
mimic flaccid paralysis.
o ST depression, T wave inversion and presence of U waves.
o If serum potassium is <2 mEq/L or <3.5mEq/L with ECG changes,
correction should be started at 0.3-0.5mEq/kg/hr infusion of potassium
chloride in intravenous fluids, preferably with continuous ECG
monitoring.

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• Edema is partly as a result of potassium deficiency and sodium retention. Do not
treat edema with a diuretic. Excess body sodium exists even though the plasma
sodium may be low. Giving high sodium loads could kill the child.
• Treatment:
➢ Give extra potassium (3-4 mmol/kg/day) orally for 2 weeks.
➢ Give extra magnesium (0.4-0.6mmol/kg/day) or 0.2-0.3 ml/kg/day of 50%
magnesium sulfate (4mEq/ml) IM on day 1 then orally
• Extra potassium and magnesium should be added to the feed during its
preparation if not pre-mixed.
➢ Add 20ml of this solution to 1 liter of feed to supply the extra potassium and
magnesium required
➢ Alternatively use commercially available pre-mixed sachets (especially
formulated for malnourished children)
• Prepare food without added salt.

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 ELECTROLYTE IMABALANCE IN MALNUTRITION MANAGEMENT
BOX
• All severely malnourished children have deficiencies of potassium and
magnesium which may take around 2 weeks to correct.
➢ Manifestations of severe hypokalemia:
o Weakness of abdominal, skeletal and even respiratory muscles that may
mimic flaccid paralysis.
o ST depression, T wave inversion and presence of U waves.
o If serum potassium is <2 mEq/L or <3.5mEq/L with ECG changes,
correction should be started at 0.3-0.5mEq/kg/hr infusion of potassium
chloride in intravenous fluids, preferably with continuous ECG
monitoring.
• Do not treat edema with a diuretic.
• Treatment:
➢ Give extra potassium (3-4 mmol/kg/day) orally for 2 weeks.
➢ Give extra magnesium (0.4-0.6mmol/kg/day) or 0.2-0.3 ml/kg/day of 50%
magnesium sulfate (4mEq/ml) IM on day 1 then orally
➢ Extra potassium and magnesium should be added to the feed during its
preparation if not pre-mixed.
➢ Add 20ml of this solution to 1 liter of feed to supply the extra potassium
and magnesium required
➢ Alternatively use commercially available pre-mixed sachets (especially
formulated for malnourished children)
• Prepare food without added salt.

INFECTION
• In SAM, the usual signs of bacterial infection such as fever and tachycardia are
often absent yet multiple infections are common.
• Assume that all children with SAM have an infection on their arrival in hospital
and treat with antibiotics immediately
• Hypoglycemia and hypothermia are signs of severe infection.
• The most common sites for infection are the skin, the GIT, the respiratory tract
(including the ears, nose and throat) and the urinary tract.
• Majority of infections and septicemia are caused by gram-negative organisms.
• Investigations:

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 ➢ Full blood count with differential count
➢ Peripheral smear
➢ Blood culture
➢ Urinalysis, Urine microscopy, sensitivity and culture
➢ Chest X-ray
➢ Gastric aspirate for AFB
➢ RDT and Peripheral smear for malaria
➢ CSF examination for meningitis
• Treatment with broad spectrum antibiotics.
• Measles vaccine if the child is older than 6 months and not vaccinated or was
vaccinated before 9 months age. Delay vaccination if the child is in shock.
• Treatment:
➢ Uncomplicated SAM: Oral amoxicillin (25-40mg/kg) for 5 days
➢ Complications (hypoglycemia, hypothermia or child looks lethargic or sickly)
or any other medical condition:
o Benzylpenicillin (50 000 U/Kg IM or IV QID) or ampicillin (50mg/kg IM
or IV QID) for 2 days, then oral amoxicillin (25-40mg/kg TDS for 5 days)
plus
o Gentamicin (7.5mg/kg IM or IV OD) or amikacin 15-20mg/kg IM or IV
OD once a day for 7 days.
o Adapt regimen to local resistance patterns
o If no improvement occurs within 48 hours change to IV cefotaxime (100-
150mg/kg/day TDS to QID) or ceftriaxone (50-75mg/kg/day BD)
o Metronidazole 7.5mg/kg TDS for 7 days may be given in addition however
the efficacy of the treatment has not been established by clinical trials.
➢ Treat other infections appropriately:
o Meningitis: do a lumbar puncture for confirmation, where possible and
treat with antibiotic regime
o Specific infections (such as pneumonia, dysentery, skin or soft tissue
infections) give antibiotics as appropriate
o Add antimalarial treatment if the child has a positive blood film for malaria
parasites or positive malaria RDT.
o TB is common but anti-TB treatment should be given only if TB is
diagnosed or strongly suspected
o HIV: test for HIV. If infected start ART as soon as possible after
stabilization of metabolic complications and sepsis. They should be
monitoring (inpatient and outpatient) in the first 6-8 weeks following

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 initiation of ART to identify early metabolic complications and
opportunistic infections.
o Parasitic worms: treatment should be delayed until the rehabilitation phase.
Give albendazole as a single dose (for <2 years 200mg Stat and those about
2 years 400mg stat) or mebendazole 100mg PO BD for 3 days. In places
prevalent also give mebendazole to children with no evidence of
infestation 7 days after admission.
• Monitoring: if the child is still anorexic after 7 days of antibiotic treatment,
continue for a full 10-day course. If anorexia persists reassess the child fully.
• Response to treatment will be indicated by resolution of initial symptoms and
signs of infection, if any. The child’s activity, interaction with parents and
appetite should improve. If there is no improvement or deterioration of the
symptoms/signs of infection, the child should be screened for infection with
resistant bacterial pathogens, TB, HIV and unusual pathogens.
• Prevention:
➢ Follow standard precautions like hand hygiene
➢ Give Measles vaccine if the child is older than 6 months and not vaccinated
or was vaccinated before 9 months age. Delay vaccination if the child is in
shock
➢ Screen for HIV and TB

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 INFECTIONS IN MALNUTRITION MANAGEMENT BOX
• Multiple infections are common, assume all children presenting to hospital with
SAM have an infection and treat.
• Usual signs of infection such as fever are often absent
• Majority of bloodstream infections are due to gram-negative bacteria
• Hypoglycemia and hypothermia are markers of severe infection
• Treatment with broad spectrum antibiotics.
• Treatment:
➢ Uncomplicated SAM: Oral amoxicillin (25-40mg/kg) for 5 days
➢ Complications (hypoglycemia, hypothermia or child looks lethargic or sickly) or
any other medical condition:
o Benzylpenicillin (50 000 U/Kg IM or IV QID) or ampicillin (50mg/kg IM or
IV QID) for 2 days, then oral amoxicillin (25-40mg/kg TDS for 5 days) plus
o Gentamicin (7.5mg/kg IM or IV OD) or amikacin 15-20mg/kg IM or IV OD
once a day for 7 days.
o Adapt regimen to local resistance patterns
o If no improvement occurs within 48 hours change to IV cefotaxime (100-
150mg/kg/day TDS to QID) or ceftriaxone (50-75mg/kg/day BD)
o Metronidazole 7.5mg/kg TDS for 7 days may be given in addition however
the efficacy of the treatment has not been established by clinical trials.
➢ Treat other infection adequately with their specific antibiotics.
• Monitoring: if the child is still anorexic after 7 days of antibiotic treatment, continue
for a full 10-day course. If anorexia persists reassess the child fully
• Prevention:
➢ Follow standard precautions like hand hygiene
➢ Give Measles vaccine if the child is older than 6 months and not vaccinated or
was vaccinated before 9 months age. Delay vaccination if the child is in shock.

MICRONUTRIENTS
• All severely malnourished children have vitamin and mineral deficiencies.
• Micronutrients should be used as an adjunct to treatment in safe and effective
doses.
• Up to twice the recommended daily allowance of various vitamins and minerals
should be used.

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• Although anemia is common iron should not be given to prevent the danger of
generating free radicals and bacterial proliferation. It should be added only after
a week of therapy when the child has a good appetite.
• Vitamin A:
➢ Give to all severely malnourished children on day 1 unless there is evidence
that a dose has been given in the last month.
o Infants 0-5 months: 50 000 IU
o 6-12 months: 100, 000 IU
o Children >1 year 200, 000 IU
➢ In the presence of xerophthalmia, the same dose should be repeated on day 2
and day 14.
➢ Children >1 year but weighing <8kg should receive 100, 000IU. In the
presence of clinical evidence of xerophthalmia the administration of vitamin
A should be considered an emergency as the changes may progress to
keratomalacia within hours
• Vitamin K: Single dose of 2.5 mg IM at time of admission.
• Daily multivitamin supplements should be given:
➢ Thiamine 0.5mg/1000kcal
➢ Riboflavin 0.6mg/1000kcal
➢ Nicotinic acid (niacin equivalents) 6.6.mg/1000kcal
• It is better to give formulations that are truly multivitamin (one that has vitamin
A, C, D, E and B12)
• Folic acid 1mg/day (5mg on day 1), Zinc 2mg/kg and copper 0.2-0.3 mg/kg/day
should be given daily.
• Iron 3mg/kg/day should be added once child starts gaining weight after
stabilization.
• Emergency treatment of severe anemia:
➢ If a severely malnourished child has severe anemia with a hemoglobin < 4g/dl
or between 4-6g/dl but with respiratory distress, a blood transfusion should be
given with whole blood 5-10ml/kg bodyweight slowly over 3 hours.
➢ Furosemide (1mg/kg stat dose) should be given at the start of the transfusion.
If the severely anemic child has signs of cardiac failure, packed cells rather
than whole blood should be transfused.
➢ Hemoglobin concentration may fall during the first week of treatment. This is
normal and no transfusion should be given. In mild to moderate anemia, iron

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 should be given for 2 months to replenish iron stores but this should not be
started until after the initial stabilization phase has been completed.

MICRONUTRIENTS IN MALNUTRITION MANAGEMENT BOX

• Vitamin A:
➢ Give to all severely malnourished children on day 1 unless there is evidence
that a dose has been given in the last month.
o Infants 0-5 months: 50 000 IU
o 6-12 months: 100, 000 IU
o Children >1 year 200, 000 IU
➢ In the presence of xerophthalmia, the same dose should be repeated on the
next day and 2 weeks later.
➢ Children >1 year but weighing <8kg should receive half the age-related
dose. In the presence of clinical evidence of xerophthalmia the
administration of vitamin A should be considered an emergency as the
changes may progress to keratomalacia within hours
• Vitamin K:
➢ Single dose of 2.5 mg IM at time of admission.
• Daily multivitamin supplements should be given.
• Folic acid 1mg/day (5mg on day 1), Zinc 2mg/kg and copper 0.2-0.3
mg/kg/day should be given daily.
• Iron 3mg/kg/day should be added once child starts gaining weight after
stabilization.
•
ANEMIA IN MALNUTRITION MANAGEMENT BOX
• If a severely malnourished child has severe anemia with a hemoglobin < 4g/dl
or between 4-6g/dl but with respiratory distress, a blood transfusion should be
given with whole blood 10ml/kg bodyweight slowly over 3 hours.
• Furosemide should be given at the start of the transfusion. If the severely
anemic child has signs of cardiac failure, packed cells rather than whole blood
should be transfused.
• Hemoglobin concentration may fall during the first week of treatment. This is
normal and no transfusion should be given. In mild to moderate anemia, iron
should be given for 2 months to replenish iron stores but this should not be
started until after the initial stabilization phase has been completed.

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INITIATION OF FEEDS
• Feeding should be started as soon as possible with a diet with:
➢ Osmolarity <350 mOsm/L
➢ Lactose not more than 2-3 g/kg/day
➢ Appropriate renal solute load (urinary osmolarity <600mOsm/L)
➢ Initial percentage of calories from protein of 5%
➢ Adequate bioavailability of micronutrients and low viscosity
• The preparation should be easy to prepared and socially acceptable and there
should be facilities for adequate storage, cooking and refrigeration.
• Feeding starts as soon as possible as frequent small feeds. If child is unable to
take orally with cup and spoon or takes <80% of the target intake offered at two
consecutive feeds, nasogastric feeds should be initiated.
➢ Breastfeeding should be continued ad libitum
• Initiation feed is F-75 (with 75Kcal/100ml and 0.9g of protein/ 100ml). Older
children could be started on cereal based diets.
➢ Begin with 2 hourly feeds of 11ml/kg/feed F75 orally or by NGT tube. This
provides
o Calories-100 Kcal/kg/day.
o Protein at 1-1.5g/kg per day.
o Liquid- 130ml/kg/day (100 if with severe edema, i.e. 8-9ml/kg/feed)
➢ Night feeds are essential and the volume of feeds are increased gradually
while decreasing the frequency of administration.
➢ The calories are increased only after the child can accept the increased volume
of feeds.
DAY FREQUENCY Volume/Kg/feed Volume/kg/day
1-2 2 hourly 11ml 130ml
3-5 3 hourly 16ml 130ml
6-7 4 hourly 22ml 130ml

• Volume of feed given per day remains the same in stabilization and
transition phase, it is the frequency that reduces.

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 INITIATION FEEDS IN MALNUTRITION
• Start small frequent feeds as soon as possible orally or by nasogastric tube if
patient is unable to take orally
• Total fluid recommended is 130ml/kg/day, reduce to 100ml/kg/day if there is
severe edema (8-9ml/kg/day)
• Continue breastfeeding ad libitum
• Start with F-75 (with 75Kcal/100ml and 0.9g of protein/ 100ml) feeds every 2
hourly
➢ Begin with 2 hourly feeds of 11ml/kg/feed. Increase to 16ml/kg/feed 3
hourly on day 3-5 and then 22ml/kg/feed 4 hourly on day 6-7.
➢ Night feeds are essential and the volume of feeds are increased gradually
while decreasing the frequency of administration.
➢ The calories are increased only after the child can accept the increased
volume of feeds.
• If persistent diarrhea give cereal based low lactose F-75 diet as starter diet
• If diarrhea continues on low lactose diets give, F-75 lactose free diets (rarely
needed)

CATCH-UP GROWTH
• Once appetite returns, higher intakes should be encouraged
• F-75 is gradually replaced with F-100 (100kcal/100ml and 2.5-3.0g
protein/100ml).
• It is recommended that each successive feed is increased by 10ml until some is
left uneaten.
• The frequency of feeds gradually decreased to 6 feeds/day and the volume
increased till the child is being offered 200ml/kg/day and 4-6g/kg/day of protein
• Breastfeeding should be continued ad libitum.
• For children with persistent diarrhea, who do not tolerate low lactose diets,
lactose free diet can be started. In these diets, carbohydrates (rice, sugar and
glucose) can be given in varying proportions according to the patients’ individual
tolerance to achieve optimal balance between osmolarity and digestibility.
• Monitoring:
➢ If there is a good weight gain of >10g/kg/day, the same treatment should be
continued till recovery.

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 ➢ If there is a moderate weight gain of 5-10kg/day, food intake should be
checked and the children should be screened for systemic infection
➢ If there is poor weight gain <5g/kg/day possible causes like inadequate
feeding, untreated infection, psychological problems and co-existing
infections like tuberculosis and HIV should be looked for and managed
appropriately.

READY TO USE THERAPEUTIC FOOD (RUFT)

• This is an energy dense, mineral and vitamin enriched ready to use therapeutic
food with a similar nutrient profile but greater energy and nutrient density than
F-100.
• F-100 even though extremely effective during rehabilitation phase in
inpatients centers, is very vulnerable to bacterial contamination and must be
used within a couple of hours being made. This restricts its use to inpatient
facilities.
• RUTF is an oil-based paste and such can be stored at home unrefrigerated with
little risk of microbial contamination for several months.
• The daily amount of RUTF to be consumed varies according to body weight:
➢ 3-4.9 kg: 105-130g
➢ 5-6.9kg: 200-260
➢ 7-7.9kg: 260-400g
➢ 10-14.9kg: 400-600g
• The amount is to be given along with plenty of water in 2-3 hourly feeds. The
child should continue to receive other foods and breastfeeding during medical
nutritional therapy with RUTF.
• Complementary foods should be added as soon as possible to prepare the child
for home foods at discharge. They should have comparable energy and protein
concentrations once the catchup diets are well tolerated.

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 CATCH UP FEEDS IN MALNUTRITION MANAGEMENT BOX
• Once appetite returns in 2-3 days, encourage higher intakes
• Increase volume offered at each feed by 10ml (maximum 200ml/kg/day) and
decrease the frequency of feeds
• Continue breastfeeding ad libitum
• Make a gradual transition from F-75 to F-100 diet
• Increase calories to 150-200kcal/kg/day and proteins to 4-6 g/kg/day
• Add complementary foods as soon as possible to prepare the child for home
foods at discharge
• RUTF can also be used.

SENSORY STIMULATION
• Delayed mental and behavioral development often occur in SAM.
• Encourage a cheerful, stimulating environment, structured play therapy for at
least 15-30 min/day
• Provide suitable toys and play activities for the child.
• Physical activity as soon as the child is well enough and tender loving care.

SENSORY STIMULATION IN MALNUTRITION MANAGEMENT BOX

• A cheerful stimulation environment
• Age appropriate structured play therapy for at least 15-30 min/day
• Age appropriate physical activity as soon as the child is well enough
• Tender loving care

PREPARE FOR FOLLOW-UP

• The child is said to have recovered when the weight for height is 90% of the
median, or has 15% weight gain and has no edema.
• The child is still likely to have low weight for age because of stunting.
• Ideally 6-8 weeks of hospitalization is required for complete recovery.
• Follow up visit at 2 weeks, 1 month, 3 months (minimum of 3 visits). After 6
months do yearly visits till weight/height reaches >-1SD. Child should be
followed up for at least 3 years.
• Criteria for discharge:
➢ Alert and active, eating at least 120-130kcal/kg/day with a consistent weight
gain (of at least 5g/kg/day for 3 consecutive days) on exclusive oral feeding

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 ➢ Receiving adequate micronutrients
➢ Free from infection
➢ Has completed immunization appropriate for age
➢ The caretaker has been sensitized to home care
• The caregiver should be advised to bring the child back for regular follow-up
checks, ensure booster immunizations, make sure that vitamin A is given every
6 months, feed frequently with energy and nutrient dense foods and give
structured play therapy.
• Early discharge can be considered if:
➢ Age >12 months
➢ Has good appetite with satisfactory weight gain
➢ Completed antibiotic treatment
➢ Has taken 2 weeks of potassium/magnesium/mineral/vitamin supplement (or
continuing supplementation at home is possible).
➢ The mother/caretaker has the financial resource to feed the child, specifically
trained to give appropriate feeding (types, amounts, frequency), lives within
easy reach of the hospital, is trained to give structured play therapy and is
motivated to follow advice given
• Home care:
➢ Give frequent meals with a high energy and protein content. Aim at
achieving at least 150kCal/day and adequate protein (at least 4g/kg/day).
This would require feeding the child at least 5 times per day with foods that
contain approximately 100kCal and 2-3 protein per 100g of food.
➢ Vitamin, iron and electrolyte/mineral supplements can be continued at home.
High energy snacks should be given between meals (e.g. milk, banana,
bread, biscuits)
➢ The child should be assisted and encouraged to complete each meal.
• Primary failure to respond is indicated by:
➢ Failure to regain appetite by day 4
➢ Failure to start losing edema by day 4
➢ Presence of edema on day 10
➢ Failure to gain at least 5g/kg/day by day 10
• Secondary failure to respond is indicated by
➢ Failure to gain at least 5g/kg/day for consecutive days during the rehabilitation
phase

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PREPARE FOR FOLLOW UP IN MALNUTRITION MANAGEMENT BOX
• Primary failure to respond is indicated by:
➢ Failure to regain appetite by day 4
➢ Failure to start losing edema by day 4
➢ Presence of edema on day 10
➢ Failure to gain at least 5g/kg/day by day 10
• Secondary failure to respond is indicated by
➢ Failure to gain at least 5g/kg/day for consecutive days during the
rehabilitation phase

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MANAGEMENT SUMMARY BOX FOR MALNUTRITION
HYPOGLYCEMIA IN MALNUTRITION
• Blood glucose level <3mmol/L (<54 mg/dl)
• If glucose can’t be done, assume child has hypoglycemia
• Hypoglycemia, hypothermia and infection generally occur as a triad
• Treatment:
➢ Asymptomatic hypoglycemia:
o Give 50ml of 10% glucose or sucrose solution orally or by NGT followed by first feed
o Feed with starter F-75 every 2 hourly day and night
➢ Symptomatic hypoglycemia
o Give 10% dextrose IV 5ml/kg
o Follow with 50ml of 10% dextrose or sucrose solution by nasogastric tube
o Feed with starter F-75 every 2 hourly day and night
➢ Start appropriate antibiotics
• Prevention
➢ Feed 2 hourly starting immediately
➢ Prevent hypothermia
HYPOTHERMIA IN MALNUTRITION
• Rectal temperature less than <35.5oC or axillary temperature less than 35oC
• Always measure blood glucose and screen for infections in the presence of hypothermia
• Treatment:
➢ Clothe the child with warm clothes, ensure that the head is also covered with a scarf or cap
➢ Provide heat using overhead warmer, skin contact or heat convector
➢ Avoid rapid rewarming as this may lead to disequilibrium
➢ Feed the child immediately
➢ Give appropriate antibiotics
• For severe hypothermia (rectal temperature <32oC)
➢ Give warm humidified oxygen
➢ Give 5ml/kg of 10% dextrose IV or 50ml of 10% dextrose by NGT (if IV access is difficult).
➢ If clinical condition allows the child to take orally, warm feeds should be given immediately or
else the feeds should be given by NGT
➢ Avoid rapid rewarming. Provide heat using radiation (overhead warmer) or conduction (skin
contact) or convection (heat convector)
➢ Monitor temperature every 30 minutes.
➢ If there is feed intolerance or another contraindication for NGT feeding, maintenance IV fluids
(prewarmed) should be started.
• Prevention
➢ Place the child’s bed in a draught free area
➢ Always keep the child well covered, ensure that head is also covered well
➢ May place the child in contact with the mother’s bare chest or abdomen (skin to skin)
➢ Feed the child 2 hourly starting immediately after admission

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DEHYDRATION IN MALNUTRITION MANAGEMENT
• Difficult to diagnose in a severely malnourished child
• Assume all severely malnourished children with watery diarrhea have some dehydration
• Low blood volume (hypovolemia) can coexist with edema
• Treatment
➢ Rehydrate slowly over 12 hours
➢ Give ReSoMal orally or by NG tube
o Give 5ml/kg every 30 minutes for the first 2hours
o Then given 5-10 ml/kg for the next 4-10h on alternate hours with F-75 formula i.e. Feeds at 2,
4, 6 hours and ReSoMal at 1,3 and 5 hours.
➢ The exact amount depends on how much the child wants, the volume of stool loss and whether the
child is vomiting.
➢ Ongoing stool losses should be replaced with approximately 5-10ml/kg ReSoMaL per losses stool.
The frequent passage of small unformed stools should not be confused with profuse watery diarrhea
as it does not require fluid replacement.
➢ Alternative fluids if ReSoMal is absent: half-strength standard WHO oral rehydration solution with
added potassium and glucose as per the ReSoMal recipe
➢ If rehydration is still required at 10hours give starter F-75 instead of ReSoMal at the same times.
Use the same volume of starter F-75 as of ReSoMal
➢ If in shock or severe dehydration but cannot be rehydrated orally or by NG tube give IV fluids
either Ringer’s lactate solution with 5% dextrose or half-strength Darrow’s solution with 5%
dextrose. If neither is available, 0.45% saline with 5% dextrose should be used
o Give it as a slow infusion of 15ml/kg over the 1st hour with continuous monitoring of pulse
rate, volume, respiratory rate, capillary refill time and urine output.
o If child improves then diagnosis was likely severe dehydration with shock so rehydrating
fluid repeated at the same rate of 15ml/kg over the next hour. This should follow ReSoMal
at 5-10ml/kg/hr either orally or by NG tube.
• Monitor
➢ Respiration and pulse, they should fall.
➢ Urine Output should also be passed.
➢ The return of tears, a moist mouth, less sunken eyes and fontanelle and improved skin turgor.
➢ Monitor rehydration every 30 minutes for 2 hours, then every hour for the next 4-10 hours. Be alert
for signs of overhydration, which is very dangerous and may lead to heart failure. Check for
o Weight gain to ensure that it is not quick and excessive
o Increasing edema and increasing periorbital puffiness
o Increase in respiratory rate (5/min)
o Increase in pulse rate (15/min)
o Urine frequency (has the child urinated since last checked?)
o Enlarging liver size on palpation
o Frequency of stools and vomit
➢ If you find signs of overhydration (Early signs are respiratory rate increasing by 5/min and pulse
rate by 25/min), stop ReSoMal immediately and reassess after 1 hour.

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• If at the end of the 1st hour of IV rehydration there is no improvement or worsening, septic shock
must be considered and appropriate treatment started.
➢ Give maintenance IV 4ml/kg/hr
➢ Review antibiotic treatment
➢ Start dopamine 10g/kg/min
• Prevention
➢ Continue breast feeding if child is breastfed.
➢ Initiate re-feeding with starter F-75
➢ Give ReSoMal between feeds to replace stool losses. 5-10ml/kg after each watery stool.

ELECTROLYTE IMABALANCE IN MALNUTRITION

• All severely malnourished children have deficiencies of potassium and magnesium which may
take around 2 weeks to correct.
➢ Manifestations of severe hypokalemia:
o Weakness of abdominal, skeletal and even respiratory muscles that may mimic flaccid
paralysis.
o ST depression, T wave inversion and presence of U waves.
o If serum potassium is <2 mEq/L or <3.5mEq/L with ECG changes, correction should be
started at 0.3-0.5mEq/kg/hr infusion of potassium chloride in intravenous fluids,
preferably with continuous ECG monitoring.
• Do not treat edema with a diuretic.
• Treatment:
➢ Give extra potassium (3-4 mmol/kg/day) orally for 2 weeks.
➢ Give extra magnesium (0.4-0.6mmol/kg/day) or 0.2-0.3 ml/kg/day of 50% magnesium
sulfate (4mEq/ml) IM on day 1 then orally
• Extra potassium and magnesium should be added to the feed during its preparation if not pre-
mixed.
➢ Add 20ml of this solution to 1 liter of feed to supply the extra potassium and magnesium
required
➢ Alternatively use commercially available pre-mixed sachets (especially formulated for
malnourished children)
• Prepare food without added salt.
INFECTIONS IN MALNUTRITION
• Multiple infections are common, assume all children presenting to hospital with SAM have an
infection and treat.
• Usual signs of infection such as fever are often absent
• Majority of bloodstream infections are due to gram-negative bacteria
• Hypoglycemia and hypothermia are markers of severe infection
• Treatment with broad spectrum antibiotics.

171
• Treatment:
➢ Uncomplicated SAM: Oral amoxicillin (25-40mg/kg) for 5 days
➢ Complications (hypoglycemia, hypothermia or child looks lethargic or sickly) or any other
medical condition:
o Benzylpenicillin (50 000 U/Kg IM or IV QID) or ampicillin (50mg/kg IM or IV QID)
for 2 days, then oral amoxicillin (25-40mg/kg TDS for 5 days) plus
o Gentamicin (7.5mg/kg IM or IV OD) or amikacin 15-20mg/kg IM or IV OD once a day
for 7 days.
o Adapt regimen to local resistance patterns
o If no improvement occurs within 48 hours change to IV cefotaxime (100-150mg/kg/day
TDS to QID) or ceftriaxone (50-75mg/kg/day BD)
o Metronidazole 7.5mg/kg TDS for 7 days may be given in addition however the efficacy
of the treatment has not been established by clinical trials.
➢ Treat other infection adequately with their specific antibiotics.
• Monitoring: if the child is still anorexic after 7 days of antibiotic treatment, continue for a full
10-day course. If anorexia persists reassess the child fully
• Prevention:
➢ Follow standard precautions like hand hygiene
➢ Give Measles vaccine if the child is older than 6 months and not vaccinated or was vaccinated
before 9 months age. Delay vaccination if the child is in shock.

MICRONUTRIENTS IN MALNUTRITION
• Vitamin A:
➢ Give to all severely malnourished children on day 1 unless there is evidence that a dose has
been given in the last month.
o Infants 0-5 months: 50 000 IU
o 6-12 months: 100, 000 IU
o Children >1 year 200, 000 IU
➢ In the presence of xerophthalmia, the same dose should be repeated on the next day and 2
weeks later.
➢ Children >1 year but weighing <8kg should receive half the age-related dose. In the presence
of clinical evidence of xerophthalmia the administration of vitamin A should be considered
an emergency as the changes may progress to keratomalacia within hours
• Vitamin K:
➢ Single dose of 2.5 mg IM at time of admission.
• Daily multivitamin supplements should be given.
• Folic acid 1mg/day (5mg on day 1), Zinc 2mg/kg and copper 0.2-0.3 mg/kg/day should be given
daily.
• Iron 3mg/kg/day should be added once child starts gaining weight after stabilization.

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ANEMIA IN MALNUTRITION
• If a severely malnourished child has severe anemia with a hemoglobin < 4g/dl or between
4-6g/dl but with respiratory distress, a blood transfusion should be given with whole blood
10ml/kg bodyweight slowly over 3 hours.
• Furosemide should be given at the start of the transfusion. If the severely anemic child has
signs of cardiac failure, packed cells rather than whole blood should be transfused.
• Hemoglobin concentration may fall during the first week of treatment. This is normal and
no transfusion should be given. In mild to moderate anemia, iron should be given for 2
months to replenish iron stores but this should not be started until after the initial
stabilization phase has been completed.

INITIATION FEEDS IN MALNUTRITION

• Start small frequent feeds as soon as possible orally or by nasogastric tube if patient is
unable to take orally
• Total fluid recommended is 130ml/kg/day, reduce to 100ml/kg/day if there is severe edema
(8-9ml/kg/feed).
• Continue breastfeeding ad libitum
• Start with F-75 (with 75Kcal/100ml and 0.9g of protein/ 100ml) feeds every 2 hourly
➢ Begin with 2 hourly feeds of 11ml/kg/feed. Increase to 16ml/kg/feed 3 hourly on day 3-
5 and then 22ml/kg/feed 4 hourly on day 6-7.
➢ Night feeds are essential and the volume of feeds are increased gradually while
decreasing the frequency of administration.
➢ The calories are increased only after the child can accept the increased volume of feeds.
• If persistent diarrhea give cereal based low lactose F-75 diet as starter diet
• If diarrhea continues on low lactose diets give, F-75 lactose free diets (rarely needed)

CATCH UP FEEDS IN MALNUTRITION

• Once appetite returns in 2-3 days, encourage higher intakes
• Increase volume offered at each feed by 10ml (maximum 200ml/kg/day) and decrease the
frequency of feeds
• Continue breastfeeding ad libitum
• Make a gradual transition from F-75 to F-100 diet
• Increase calories to 150-200kcal/kg/day and proteins to 4-6 g/kg/day
• Add complementary foods as soon as possible to prepare the child for home foods at
discharge
• RUTF can also be used.

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READY TO USE THERAPEUTIC FOOD (RUFT)
• This is an energy dense, mineral and vitamin enriched ready to use therapeutic food with a
similar nutrient profile but greater energy and nutrient density than F-100.
• F-100 even though extremely effective during rehabilitation phase in inpatients centers, is
very vulnerable to bacterial contamination and must be used within a couple of hours being
made. This restricts its use to inpatient facilities.
• RUTF is an oil-based paste and such can be stored at home unrefrigerated with little risk of
microbial contamination for several months.
• The daily amount of RUTF to be consumed varies according to body weight:
➢ 3-4.9 kg: 105-130g
➢ 5-6.9kg: 200-260
➢ 7-7.9kg: 260-400g
➢ 10-14.9kg: 400-600g
• The amount is to be given along with plenty of water in 2-3 hourly feeds. The child should
continue to receive other foods and breastfeeding during medical nutritional therapy with
RUTF.
• Complementary foods should be added as soon as possible to prepare the child for home
foods at discharge. They should have comparable energy and protein concentrations once
the catchup diets are well tolerated.

SENSORY STIMULATION IN MALNUTRITION MANAGEMENT

• A cheerful stimulation environment
• Age appropriate structured play therapy for at least 15-30 min/day
• Age appropriate physical activity as soon as the child is well enough
• Tender loving care

PREPARE FOR FOLLOW UP IN MALNUTRITION

• Primary failure to respond is indicated by:
➢ Failure to regain appetite by day 4
➢ Failure to start losing edema by day 4
➢ Presence of edema on day 10
➢ Failure to gain at least 5g/kg/day by day 10
• Secondary failure to respond is indicated by
➢ Failure to gain at least 5g/kg/day for consecutive days during the rehabilitation phase

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MANAGEMENT OF SAM IN CHILDREN <6 MONTHS
• Initial steps of management i.e. hypoglycemia, hypothermia, dehydration and
septic shock are the same as older children.
• Feed with appropriate milk feeds for initial recovery and metabolic stabilization
• Breastfeeding or expressed milk is preferred
• If breast milk is insufficient initially combine expressed breast milk and
supplementary milk feeds
➢ Supplementary suckling techniques: this is a strategy to initiate relactation in
mothers who have developed lactation failure
➢ The cup of milk should be 5-10cm below the nipple to prevent chances of
aspiration
• Give supplementary milk feeds for non-breastfed babies or mothers who are HIV
positive and opt for replacement feed.
• In supplementary milk feeds, dilute F100 milk by 1/3 extra water to make volume
135ml in place of 100ml.
• On discharge the non-breastfed infants should be given locally available animal
milk with cut and spoon.
COMPLICATIONS OF MALNUTRITION AND NUTRITIONAL
REHABILITATION
REFEEDING SYNDOME
• This is a complication of nutritional rehabilitation, severe hypophosphatemia.
• Symptoms: weakness, neutrophil dysfunction, rhabdomyolysis, arrhythmias,
seizures, altered mental status, cardiorespiratory failure.
• Diagnosis: low serum phosphate in setting of symptoms.
• Treatment: administer B1, replace phosphate and monitor phosphate levels
NOMA (CANCRUM ORIS)
• Rapidly progressive necrotizing, ulcerating gingival and perioral inflammation
seen in PEM patients, typically it is preceded by an infection or debilitating
illness.
• Symptoms: Gingivitis, halitosis, anemia, fever
• Treatment-
➢ Local wound care
➢ Penicillin and metronidazole
➢ Treat underlying malnutrition

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PSEUDOTUMOR CEREBRI
• Overenthusiastic nutritional correction in malnourished infants may be
accompanied by transient rise of intracranial tension.
• It is benign and self-limiting
NUTRITIONAL RECOVERY SYNDROME
• It is a sequence of events seen in children being treated with very high quantity
of proteins during the course of rehabilitation.
• It presents as:
➢ Abdominal distension
➢ Increasing hepatomegaly
➢ Ascites
➢ Prominent thoracoabdominal venous network
➢ Hypertrichosis
➢ Parotid swelling
➢ Gynecomastia
➢ Eosinophilia
➢ Splenomegaly
• Its development may be related to endocrinal disturbances possibly by an
increase in the estrogen level and by a variety of trophic hormones produced by
the pituitary gland
ENCEPHALITIS LIKE SYNDROMES
• Seen in 1/5 of patients with kwashiorkor they may become drowsy within 3-4
days after initiation of dietary therapy.
• Most often the condition is self-limiting.
• Occasionally, it may be accompanied by progressive unconsciousness with fatal
outcome. Even more rarely a transient phenomenon marked by coarse tremors,
parkinsonian rigidity, bradykinesia and myoclonus may appear several days after
starting the dietary rehabilitation. These encephalitis states are considered to be
the result of too much proteins in the diet.
PREVENTION OF MALNUTRITION
• At national level
➢ Nutritional surveillance to define the character and magnitude of nutritional
problems in order to formulate strategies to counter these problems.

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 ➢ Nutritional planning: involves political commitment by the government,
formulation of a nutrition policy and planning to improve production and
supplies of food and ensure its distribution
➢ Nutrition supplementation by fortification of staple food e.g. Vitamin A to
mealie meal, sugar and iodination of common salt and food supplementation
• At community level
➢ Health and nutritional education on common foods, irrational beliefs about
certain foods and cultural taboos about feeding.
➢ Promote education and literacy in the community
➢ Growth monitoring using growth cards.
➢ Vigorous promotion of family planning programs to limit family size.
➢ Integrated health package: preventive immunization, oral hydration, periodic
deworming, health checkup, and early diagnosis and treatment of common
illness to all sectors of the population
• At family level
➢ Promote exclusive breastfeeding for first 6 months of life
➢ Complementary foods should be introduced in the diet of infant at age 6
months
➢ Vaccination
➢ Iatrogenic restriction of feeding in fevers and diarrhea should be discouraged
(continue breastfeeding)
➢ Adequate spacing between two pregnancies to ensure proper infant feeding
and attention to the child before the next conception

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CALCULATION OF CALORIES
• Recall:
➢ Carbohydrates are the main source of energy and contribute 55-60% of total
energy intake
o The contribute taste, texture and bulk to the diet.
o Deficiency (<30%) may produce ketosis, loss of weight and proteolysis.
o They are divided into:
▪ Simple carbohydrates: monosaccharides and disaccharides e.g.
glucose and fructose in fruits vegetable, and honey, sucrose in sugar
and lactose in milk
▪ Complex carbohydrates: oligosaccharides and polysaccharides such as
starch in cereals, millet, pulses and root vegetables.
o Carbohydrates provide 4Kcal of energy per gram.
o Glucose is used as a fuel by the cells and is converted to glycogen by liver
and muscle. Excess carbohydrates are converted to fat.
➢ Proteins are the second most abundant substance in the body after water.
o About 8-12% of the total energy should be provided from protein sources.
o They are required for growth, synthesis of tissues in the body, formation
of digestive juices, hormones, plasma proteins, enzymes and hemoglobin,
as buffers to maintain acid-base equilibrium in the body and an alternative
source of energy.
o They are made of amino acids which may either be
▪ Non-essential: can be synthesized by the body
▪ Essential: cannot be synthesized by the body and are required in diet
e.g. leucine, isoleucine, lysine, methionine, phenylalanine, threonine,
tryptophan and valine.
o Histidine and arginine are essential during infancy because the rate of their
synthesis is inadequate for sustaining growth
o Proteins provide 4 Kcal energy per gram.
➢ Fats
o About 25-30% of energy intake should be from fat.
o Are composed of saponifiable esters of long chain fatty acids
o They are structural elements of the cell membrane, are a major source of
energy, carry fat soluble vitamins (A, D, E, and K) and are precursors or
prostaglandins and hormones.

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 o Are present in diet in the form of fatty acids (triglycerides), phospholipids
and cholesterol.
o Fats provide 9Kcal of energy per gram.
• There are 3 critical periods in early life of a young child with regards to energy
requirements:
➢ Around 6 months when complementary feeding is initiated
➢ 1 and 2 years when physical activity is increased
➢ 10 and 12 years for girls and 15 to 18 years for boys when puberty is attained.
• Calculation of energy requirement should account for the level of physical
activity and the energy required to allow for optimal growth.
• For children with normal body weights, the energy requirements are calculating
roughly:
➢ 100kCal for the first 10Kg
➢ 50Kcal/kg for next 10kg
➢ 220Kcal/kg for remaining kg above 20
➢ Example a child weighing 30kg would require (10 x 100) + (50 x 10) + (10 x
20) = 1700kcal

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 DEHYDRATION

DEHYDRATION
• For all children with diarrhea, their hydration status should be classified.
• The degree of dehydration is graded according to symptoms and signs that reflect
the amount of fluid lost, they include:
➢ No dehydration (usually <5% loss of body weight)
➢ Some dehydration (usually 5-10%)
➢ Severe dehydration (usually >10%)
• This classification makes management of acute diarrhea easy.
• Signs of dehydration include:
➢ Sunken anterior fontanelle
➢ Listlessness or irritability
➢ Sunken eyes
➢ No tears when crying
➢ Dry mucous membranes
➢ No wet diapers for 3 hours or more
➢ Rapid thready pulse, low blood pressure, capillary refill >2 secs
➢ Reduced skin turgor
➢ High/low temperature
NO DEHYDRATION
• There are not enough signs to classify as some or severe dehydration.
• Child is well alert; eyes are normal (not sunken), tears are present when crying,
mouth and tongue are moist.
• In some infants and children, the eyes normally appear somewhat sunken. It is
helpful to ask the mother if the child’s eyes are normal or more sunken than usual.
• Child drinks normally and is not thirsty.
• Skin pinch goes back quickly.
• Use treatment plan A.
MANAGEMENT FOR NO DEHYDRATION
• The 3 essential elements in the management of all children with diarrhea are:
➢ Rehydration therapy (Plan A)
➢ Zinc supplementation
➢ Counselling for continued feeding (increase frequency and duration), educate
mother on prevention

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• Patients usually do not need admission if there are no other co-morbidities.
• Educate the mother/caregivers on the danger signs of dehydration.
➢ Continuing diarrhea beyond 3 days
➢ Increased volume/frequency of stools
➢ Repeated vomiting
➢ Increasing thirst, refusal to feed, fever or blood in stool
• Encourage the mother to continue breast feeding (longer and more frequently).
• Give ORS (10ml/kg per loose stool)
➢ <2 years: 50-100 ml per loose stool
➢ 2-5 years: 100-200 ml per loose stool
➢ For older children encourage to take as much as they want
➢ Tell the mother to give frequent small sips from a cup, if the child vomits wait
10 minutes. Then continue but more slowly. Continue giving extra fluid until
the diarrhea stops.
• Note:
➢ If the child is exclusively breastfed, give ORS or clean water in addition.
➢ If child is not exclusively breastfed, give one or more of either ORS, food-
based fluids (such as soup, rice water, yoghurt) or clean water.
➢ Teach mother how to mix and give ORS. Give mother 2 packets of ORS to
use at home. Also educate mother on how to make ORS
➢ If ORS packs are not present oral rehydration salts can be made using:
o 1 level teaspoon (5ml) of salt (do not use too much salt. If solution has too
much salt the child may refuse to drink it).
o 8 level teaspoons of sugar (preferably brown sugar which contains more
potassium than white sugar)
o 1 liter of drinking water
➢ Avoid juices and carbonated drinks
• Zinc sulphate
➢ For <6 months: half tablet (10mg) per day for 10-14 days.
➢ For >6 months: one tablet (20mg) per day for 10-14 days.
➢ For infants dissolve the tablet in a small amount of clean water, expressed
milk or ORS in a small cup or spoon.
➢ Older children can chew the tablet or drink it dissolved in a small amount of
clean water in a cup of spoon
• Advise mother to return to clinic/hospital if condition worsens or does not
improve.

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• Give antibiotics: ciprobid or cefotaxime, add metronidazole if there is bloody
diarrhea
• Deworm older children with mebendazole if not dewormed in past 6 months.
SOME DEHYDRATION
• There are 2 or more of the following signs:
➢ Restlessness, irritability
➢ Sunken anterior fontanelle and eyes
➢ Tears are absent when crying
➢ Mouth and tongue are dry
➢ Eager drinking, thirsty
➢ Skin pinch goes back slowly (1-2 sec)
• Weigh the patient, if possible and use treatment plan B.
MANAGEMENT
• The 3 essential elements in the management of all children with diarrhea are:
➢ Rehydration therapy (Plan B)
➢ Zinc supplementation
➢ Counselling for continued feeding and prevention
• All cases with obvious signs of dehydration need to be treated in health centers
or hospitals.
• Give 75ml/kg in 4 hours of ORS (if fluids can be tolerated) and reassess after 4
hours. If there is no dehydration transition to plan A (10ml/kg per loose stool).
➢ Tell the mother to give frequent small sips from a cup, if the child vomits wait
10 minutes. Then continue but more slowly. Continue giving extra fluid until
the diarrhea stops.
• If fluids are not tolerated orally give IV ringers lactate.
• Zinc sulphate
➢ For <6 months: half tablet (10mg) per day for 10-14 days.
➢ For >6 months: one tablet (20mg) per day for 10-14 days.
➢ For infants dissolve the tablet in a small amount of clean water, expressed
milk or ORS in a small cup or spoon.
➢ Older children can chew the tablet or drink it dissolved in a small amount of
clean water in a cup of spoon
• Give antibiotics: ciprobid or cefotaxime, add metronidazole if there is bloody
diarrhea
• Deworm older children with mebendazole if not dewormed in past 6 months.

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SEVERE DEHYDRATION
• There are 2 or more of the following signs:
➢ Lethargy or unconsciousness
➢ Depressed (severely sunken) anterior fontanelle and eyes
➢ When child cries there are no tears
➢ Mouth and tongue are dry
➢ Unable to drink or drinks poorly
➢ Skin pinch goes back very slowly (>2s)
• Weigh the patient and use treatment plan C urgently.
MANAGEMENT
• The 3 essential elements in the management of all children with diarrhea are:
➢ Rehydration therapy (Plan C)
➢ Zinc supplementation
➢ Counselling for continued feeding and prevention
• Start IV fluid immediately. If the child can drink, give ORS by mouth while the
drip is being set up.
• Give 100ml/kg Ringer’s lactate solution/ ½ strength Darrows (or if not available
use normal saline)
• For children less than 1 year (give fluids over 6 hours)
➢ First 30ml/kg: give in 1 hour
➢ Last 70ml/kg: give in 5 hours
• For children above 1year to 5 years (give 100ml/kg in 3 hours)
➢ First 30ml/kg: give in 30 minutes
➢ Last 70ml/kg: give in 2hours 30 minutes
• Reassess the child every 10-15 min. If hydration status is not improving give IV
drip more rapidly. Also watch for over-hydration.
• If IV therapy is not available then ORS by nasogastric tube or orally at
20ml/kg/hour for 6 hours (total of 120ml/kg) should be given. If abdomen
becomes distended or the child vomits repeatedly then ORS should be given more
slowly.
• Also give ORS (about 5ml/kg per h) as soon as the child can drink: usually after
3-4 h (infants) and 1-2 h (children).
• Reassess an infant after 6 hours and a child after 3 hours. Classify dehydration.
Then choose the appropriate plan (A, B or C) to continue treatment.
• Zinc sulphate

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 ➢ For <6 months: half tablet (10mg) per day for 10-14 days.
➢ For >6 months: one tablet (20mg) per day for 10-14 days.
➢ For infants dissolve the tablet in a small amount of clean water, expressed
milk or ORS in a small cup or spoon.
➢ Older children can chew the tablet or drink it dissolved in a small amount of
clean water in a cup of spoon
• Give antibiotics: ciprobid or cefotaxime, add metronidazole if there is bloody
diarrhea
• Deworm older children with mebendazole if not dewormed in past 6 months.

No dehydration Some dehydration Severe dehydration

General condition Well, alert Restless, irritable Lethargic or
unconscious
Eyes/anterior Normal Sunken Very sunken
fontanelle
Thirst Drinks normally, Thirst, drinks Drinks poorly, or not
not thirsty eagerly able to drink at all
Skin pinch Goes back quickly Goes back slowly (1- Goes back very
(<1sec) 2 secs) slowly >2sec
Management plan WHO Plan A WHO Plan B WHO Plan C

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 DIARRHEAL DISEASE

DIARRHEAL DISEASE
• Diarrhea is defined as change in consistency and frequency of stools i.e. liquid or
watery stools, that occurs >3 times a day.
• The increased water content in stool (above the normal value of approximately
10ml/kg/d in the infant and young child or 200g/d in the teenager and adult) is
due to an imbalance in the physiology of the small and large intestines involved
in the absorption of ions, organic substrates and thus water.
• If there is associated blood in stool it is termed dysentery.
• Although the term “acute gastroenteritis” is commonly used synonymously with
“acute diarrhea”, acute gastroenteritis is actually a misnomer. The term
gastroenteritis implies inflammation of both the stomach and the small intestine,
whereas, in reality, gastric involvement is rare if ever seen in acute diarrhea.
Furthermore, Cholera and shigella are examples of infectious acute diarrheal
syndromes that do not cause enteritis. Therefore, it is more accurate to use the
term acute diarrhea as opposed to acute gastroenteritis.
• The most important consequences of diarrhea in children are malnutrition and
dehydration.
➢ Malnutrition and diarrhea form a vicious cycle since malnutrition increases
the risk and severity of diarrhea.
➢ Impaired absorption, loss of nutrients, increased catabolism and improper
feeding in diarrhea aggravate the severity of malnutrition.
• The peak incidence of diarrhea as well as mortality is among 6-11 months old
infants.
• Diarrhea if prolonged may significantly impair psychomotor and cognitive
development in young children.
CLASSIFICATION OF DIARRHEA
• Diarrhea can be classified according to duration as:
➢ Acute: occurring within 14 days with sudden onset.
➢ Persistent: starts off as acute and lasts more than 14 days.
➢ Chronic: has an insidious onset and lasts longer than 14 days.
• Etiologically it can be classified as:
➢ Infectious (organic)- common
➢ Non-infectious (inorganic)

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ACUTE DIARRHEAL DISEASE
• Acute diarrheal disease (ADD) is the abrupt onset of 3 or more loose stools per
day.
ETIOLOGY
• Causes are divided into:
➢ Infectious (organic)- common
➢ Non-infectious (inorganic)
INFECTIOUS CAUSES
• These can be classified as:
➢ Viral: Rotavirus (common in children), Norovirus spp (Norwalk virus),
cytomegalovirus, Enteric adenovirus, Astrovirus, Coronaviruses,
Picornavirus, human calicivirus, HIV
➢ Bacterial
o Bacterial producing inflammatory diarrhea
▪ Campylobacter jejuni*
▪ Clostridium difficile (common in antibiotic induced diarrhea)
▪ Clostridium perfringens
▪ Enteroinvasive E. coli*
▪ E. coli O157:H7*
▪ Salmonella*
▪ Shigella*
▪ Yersinia enterocolitica
▪ Vibrio parahaemolyticus
o Bacteria producing non-inflammatory diarrhea
▪ Enterotoxigenic E. coli
▪ Vibrio cholerae 01 and 0139
▪ Enteropathogenic E.coli
▪ V. parahaermolyticus
▪ S. aureus
➢ Parasitic
o Giardia lamblia*,
o Cryptosporidium parvum,
o Entamoeba histolytica*,
o Cyclspora cayetanensis,
o Isospora belli,

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 o Balantidium coli,
o Blastocystis hominis,
o Trichuris trichiura
o Strongyloides stercoralis
• Note ‘*’ denotes causative agents that can cause dysentery
• Globally, E. coli is the most common organism causing diarrhea followed by
Rota virus, Shigella species and non-typhoidal Salmonella species.
• Enteropathogens that are infectious in a small inoculum e.g. Shigella, E. coli,
enteric viruses, G. lamblia, C. parvum and E. histolytica may be transmitted
by person-to-person contact whereas others e.g. cholera usually required
ingestion of contaminated food or water.
NONINFECTIOUS CAUSES
• Include:
➢ Drugs: antibiotics (erythromycin), NSAIDS
➢ Certain foods/Malabsorption syndromes: lactose (lactose intolerance), Gluten
(celiac disease), food allergy (Cow’s milk, soya)
➢ Inflammatory bowel disease: Crohn’s disease
➢ Toxins: Tetrodotoxin from consumption of puffer fish and Botulism due to
improper preservation of food.
➢ Surgical conditions: Appendicitis or Hirschsprung disease
➢ Endocrine: Hyperthyroidism
• Some of the above causes may lead to persistent/chronic diarrhea
• The majority of cases of acute diarrhea in children are of infectious origin.
RISK FACTORS
• Young age
➢ Viral diarrhea is most common in young children. Rotavirus and adenovirus
are common in children <2 years. Astrovirus and norovirus usually infect
children younger than 5years.
➢ Yersinia enterocolitis typically infects children younger than 1 year and
Aeromonas organism is a significant cause of diarrhea in young children.
• Immune deficiency- malnutrition, HIV, drugs
• Measles
• Lack of exclusive breastfeeding
• Vitamin deficiencies and mineral e.g. Vitamin A and zinc

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PATHOPHYSIOLOGY
• The changes in the small bowel are typically non-inflammatory while the ones in
the large bowel are inflammatory.
• The number of pathogens required to cause infection varies from as few as one
(for cryptosporidium) to as many as 108 (for V. cholera).
• Diarrhea is the reversal of the normal net absorptive status of water and
electrolyte absorption to secretion.
• This can be as a result of either: an osmotic force that acts in the lumen to drive
water into the gut (osmotic diarrhea) or the result of an active secretory state
induced in the enterocytes (Secretory diarrhea).
• In osmotic diarrhea, diarrhea is osmolar in nature, as is observed after the
ingestion of nonabsorbable sugars e.g. lactulose or lactose in lactose
malabsorbers.
➢ The stool output is proportional to the intake of the unabsorbable substrate
and is usually not massive, diarrhea stools promptly regress with
discontinuation of the offending nutrient.
➢ Stool anion gap= 290mOsm/kg – [(Na + K) x 2]
➢ Stool anion gap is high (exceeding 100 mOsm/kg), this indicates impaired
nutrient absorption capacity (accumulation of electrolytes, unabsorbed
nutrients and their degradation product)
• In secretory diarrhea, there enhanced anion secretion (mostly by the crypt cell
compartment) which may be as a result of enterotoxin-induced diarrhea e.g. in
cholera.
➢ After colonization, enteric pathogens may adhere to or invade the epithelium,
they may produce enterotoxins (Exotoxins that elicit secretion by increasing
intracellular second messenger systems) or cytokines which trigger an
inflammatory response, increase secretion and the release of prostaglandins
or platelet-activating factor. This type of diarrhea includes a high purging rate,
and a lack of response to fasting.
➢ There is a normal stool anion gap i.e. 100mOsm/kg or less indicating that
nutrient absorption is intact.

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CLINICAL PRESENTATION
• Clinical manifestations and clinical syndromes of diarrhea are related to the
infecting pathogen and the dose/inoculum.
• Other manifestations depend on the presence of complications such as
dehydration and electrolyte imbalance and the nature of the pathogen.
• Knowledge of the characteristics of consistency, color, volume and frequency
can be helpful in determining whether the source is from the large or small bowel.

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Stool characteristics Small bowel Large bowel
Appearance Watery Mucoid and/or bloody
Volume Large Small
Frequency Increased Highly increased
Blood Possibly positive but never Commonly gross blood
gross blood
pH Possibly <5.5 >5.5
Reducing substances Possibly positive Negative
WBCs <5 per high power field Common >10 per high
power field
Serum WBCs Normal Possible leukocytosis,
bacteremia
Organisms • Viral: • Invasive bacteria:
➢ Rotavirus ➢ E. coli
➢ Adenovirus (enteroinvasive,
➢ Calicivirus enterohemorrhagic)
➢ Astrovirus ➢ Shigella species
➢ Norovirus ➢ Campylobacter
• Enterotoxigenic bacteria species
➢ E. coli ➢ Yersinia species
➢ Klebsiella ➢ Aeromonoas
➢ Clostridium species
perfringens ➢ Plesiomonas
➢ Cholera species species
➢ Vibrio species • Toxic bacteria
• Parasites ➢ Clostridium
➢ Giardia species difficile
➢ Cryptosporidium • Parasites
species ➢ Entamoeba
organisms

• Rotavirus
➢ RNA virus usually seen in the winter months and spreads via fecal-oral route.
➢ Incubation period of 1-3 days and patients may be asymptomatic or may have
vomiting, diarrhea, and dehydration. Diarrhea is usually self-limited and lasts
4-7 days. Symptoms of upper respiratory tract infection may sometimes be
present.
➢ Diagnosis: positive stool enzyme-linked

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• E. coli
➢ Enteropathogenic E. coli (EPEC): has an incubation period of 6-48 hours.
Presents as self-limiting diarrhea occasional fever and vomiting.
➢ Enteroinvasive E. coli (EIEC): has an incubation period of 1-3 days. Presents
as watery diarrhea, occasionally bloody diarrhea.
➢ Enteroaggregative E. coli (EAEC): has an incubation of 8-18 hours. Presents
as watery, mucoid diarrhea. Bloody diarrhea is seen in a third of cases.
➢ Enterohemorrhagic E. coli (EHEC)/Shiga-toxin producing E.coli
(STEC)/Verocytotoxin producing E. coli (VTEC): has an incubation period
of 3-9 days. Presents as abdominal pain, vomiting, bloody diarrhea, hemolytic
uremic syndrome is seen in 10% of cases.
➢ Enterotoxigenic E. coli (ETEC): has an incubation period of 14-30 hours.
Presents as watery diarrhea, fever, abdominal pain and vomiting
➢ Diffusely adherent E. coli: has an incubation of 6-48 hours. Presents as watery
diarrhea.
• S. aureus: Ingestion of pre-formed toxins is associated with the rapid onset of
nausea and vomiting within 6 hours with possible fever abdominal cramps and
diarrhea within 8-72 hours.
• Shigella has an incubation period of 16-72 hours and presents as mucoid and
bloody diarrhea (may be watery initially), fever, toxicity, tenesmus
• Enterotoxin-producing bacteria such C. perfringens and B. cereus are associated
with watery diarrhea and abdominal cramps after 8-16 hour incubation period
• Campylobacter has an incubation period of 2-4 days and presents with abdominal
pain (Frequently right sided), watery diarrhea (occasionally mucoid and bloody),
with fever.
• Abdominal cramps and watery diarrhea after a 16-48 hour incubation period can
be associated with calicivirus, several enterotoxin-producing bacteria,
Cryptosporidium and Cyclospora.
• Several organisms such as Salmonella, Shigella, C. jejuni, Yersinia
enterocolitica, enteroinvasive E. coli and Vibrio parahaemolyticus are associated
with diarrhea that may contain fecal leukocytes, abdominal cramps and fever,
although these organisms can cause watery diarrhea without fever.
• Bloody diarrhea and abdominal cramps after a 72-120 hour incubation period are
associated with infections due to Shigella and also Shiga-toxin producing E. coli
such as E. coli O157:H7.

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• Note:
➢ Although nausea and vomiting are non-specific, they are indicative of
infection in the upper intestine.
➢ Features such as nausea and vomiting, absent or low-grade fever with mild to
moderate periumbilical pain and watery diarrhea are indicative of upper
intestinal tract involvement.
➢ Fever is suggestive of an inflammatory process and also occurs as a result of
dehydration. Fever is common in patients with inflammatory diarrhea, severe
abdominal pain and tenesmus are indicative of involvement of the large
intestines.

FEATURE No Dehydration Some dehydration Severe dehydration

(<5% loss of body (5-10% loss of (>10% loss of body
weight) body weight) weight)
Mental status Well, alert Normal, irritable, Lethargic, apathetic,
lethargic unconscious
Eyes/fontanelle Normal Sunken Deeply sunken
Tears Present Deceased Absent
Mucous Moist Dry Parched
membranes
Skin pinch Normal Goes back slowly Goes back very
(1-2 sec) slowly (>2 sec)
Extremities Perfused +/- delayed Delayed capillary
capillary refill refill >2 sec, cold,
mottled
Pulse Normal Rapid Thread, weak,
volume/heart rate impalpable
Blood pressure Normal Decreased Hypotensive/
unrecordable (in
shock)
Breathing Normal Fast Rapid/deep
Urine output Normal Decreased Absent for >8 hours

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ASSESSMENT OF A CHILD WITH ACUTE DIARRHEA
HISTORY
• The history of presenting complaint should contain information on:
➢ Onset of diarrhea, association with meals
➢ Duration and number of stools per day
➢ Nature: Blood or mucus in stools
➢ Number of episodes of vomiting, amount, color of vomitus, is the vomiting
projectile (pyloric stenosis)?
➢ Is the child passing less urine than normal? (Ask when was the last wet nappy)
➢ Presence of fever, cough, rash, abdominal pain or other significant symptoms
(e.g. convulsions, recent measles, attacks of crying with pallor in an infant)
➢ Is the child able to drink or feed?
➢ If so, is he/she thirstier than normal, does he/she drink eagerly?
• Past medical history:
➢ History of recent antibiotic use (e.g. may be as a result of C. difficile infection)
➢ Does the child have cystic fibrosis or diabetes?
• Drug and immunization history
➢ Drugs or other local remedies taken (including opioids or anti-motility drugs
e.g. loperamide that may cause abdominal distention)
➢ Immunization history
• Nutrition and feeding history:
➢ Type and amount of fluids (including breast milk) and food taken during the
illness and just before the illness
➢ Unusual foods or recent restaurant meal
• Social economic: day care attendance or travel
PHYSICAL EXAMINATION
• A detailed physical examination should focus on assessment of hydration,
particularly in young children.
• Look for:
➢ Signs of dehydration
o General condition: Lethargy/unconsciousness, Restlessness or
irritability
o Depressed anterior fontanelle (in infants below 18 months)
o Sunken eyes
o Lack of tears when crying

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 o Dry mucous membranes
o Unable to drink or drinks poorly/ drinks eagerly, thirsty
o Skin pinch (turgor):
▪ Flatten immediately
▪ Goes back slowly (1-2s)
▪ Goes back very slowly (>2s)
o Tachycardia or hypotension (shock)
➢ Blood in stool
➢ Signs of severe malnutrition
o Anthropometric measurements
o Wasting
o Edema
o Signs of vitamin deficiency
➢ Signs of infection:
o Cough
o High grade fever
o Tachypnea and/or chest in-drawing suggesting pneumonia
o High grade fever with splenomegaly suggests malaria
o Fungal infections (oral thrush or perianal satellite lesions)
➢ Abdominal mass
➢ Abdominal distention
• Note: there is no need for routine stool microscopy or culture in children with
non-bloody diarrhea.
INVESTIGATIONS
• Patients can usually be managed even without investigations.
• Stool microscopy, culture and sensitivity (MCS)
➢ Stool microscopy is not helpful in management except in selected cases e.g.
cholera and giardiasis (for identification of the parasite).
➢ Stool culture is of little value in routine management of acute diarrhea.
➢ Stool specimen should be examined for mucus, occult blood, Parasites & ova
and leukocytes (use Wright stain)
o Fecal leucocytes (>10 cells per HPF) are indicative of bacterial invasion
of colonic mucosa although some patients with shigellosis may have
minimal leukocytes at an early stage of infection, as do patients infected
with Shiga toxin-producing E. coli and E. histolytica.

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 ➢ Sensitivity is important in deciding on antibiotic therapy e.g. in patients with
Shigella dysentery and do not respond to initial empiric antibiotics.
• Full blood count: ruling out any underlying anemias, systemic infections,
coagulopathies
• Urea, Creatinine and electrolytes: for electrolyte imbalance and renal function
➢ There is a non-anion gap hyperchloremic metabolic acidosis as a result of
bicarbonate loss in stool.
• Other investigations that may be indicated:
➢ ESR
➢ Abdominal X-ray
➢ Barium enema/meal
TREATMENT
• Principles of management:
➢ Diagnosis of condition (history, physical examination and investigations)
➢ Oral Rehydration therapy
o Correct dehydration in 4-6 hours.
o Those in shock and those unable to tolerate oral fluid may require
intravenous rehydration however, oral rehydration is the preferred mode
of rehydration and replacement of on-going losses.
o Low osmolality oral rehydration fluids are effective in reducing stool
output as compared to standard ORS.
o For children at risk of dehydration from vomiting taking a single dose of
the anti-emetics such as metoclopramide or ondansetron may be helpful.
o No dehydration:
▪ Encourage the mother to continue breast feeding (longer and more
frequently).
▪ Give ORS (10ml/kg per loose stool)
- <2 years 50-100ml per loose stool
- >2 years 100-200ml per loose stool
o Some dehydration:
▪ Give 75ml/kg in 4 hours of ORS (if fluids can be tolerated, small sips)
and reassess after 4 hours. If there is no dehydration transition to plan
A (10ml/kg per loose stool).
o Severe dehydration:
▪ Start IV fluid immediately. If the child can drink, give ORS by
mouth while the drip is being set up.

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 ▪ Give 100ml/kg Ringer’s lactate solution (or if not available normal
saline)
▪ For children less than 1 year (give fluids over 6 hours)
- First 30ml/kg: give in 1 hour
- Last 70ml/kg: give in 5 hours
▪ For children above 1year to 5 years (give 100ml/kg in 3 hours)
- First 30ml/kg: give in 30 minutes
- Last 70ml/kg: give in 2hours 30 minutes
▪ Reassess the child every 10-15 min. If hydration status is not
improving give IV drip more rapidly. Also watch for over-
hydration.
▪ Also give ORS (about 5ml/kg per h) as soon as the child can drink:
usually after 3-4 h (infants) and 1-2 h (children).
▪ Reassess an infant after 6 hours and a child after 3 hours. Classify
dehydration. Then choose the appropriate plan (A, B or C) to
continue treatment.
➢ Treatment of underlying cause Antimicrobial therapy
o Reduces duration and severity of diarrhea and prevents complications.
o G. lamblia, E. histolytica, Blastocystis hominis: Metronidazole 30-40
mg/kg per day divided TID for 7-10 days.
o Shigella (Severe dysentery and EIEC dysentery): Ciprofloxacin= 20-
30mg/kg per day divided BID for 7-10 days.
o EPEC, ETEC, EIEC: Trimethoprim sulfamethoxazole: 10mg/kg per day
of TMP and 50mg/kg per day of SMX divided BID for 5 days or
ciprofloxacin 20-30mg/kg per day QID for 5-10 days.
o Cryptosporidium species: Paromomycin or azithromycin 25-35mg/kg per
day divided TID for 5-10 days
o Note: while these agents are important to use in specific cases, their
widespread and indiscriminate use may lead to development of
antimicrobial resistance.
➢ Nutrition and diet
o Continue appropriate feeding. Once rehydration is complete, food should
be reintroduced while oral rehydration can be continued to replace ongoing
losses from stools and for maintenance.
o Breastfeeding should resume as soon as possible.
o Provide a minimum of 100kcal/kg per day and a protein intake between 2-
3g/kg per day.

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 o With exception of acute lactose intolerance with diarrhea most children are
able to tolerate milk and lactose containing diets.
o Zinc supplementation (zinc sulphate)
▪ Zinc supplementation reduces the duration and severity of diarrhea.
- It is recommended that all children (>6 months of age) with acute
diarrhea should receive oral zinc in some form for 10-14 days
during and after diarrhea (10-20mg per day).
- For <6 months: half tablet (10mg) per day for 10-14 days.
- For >6 months: one tablet (20mg) per day for 10-14 days.
- For infants dissolve the tablet in a small amount of clean water,
expressed milk or ORS in a small cup or spoon.
- Older children can chew the tablet or drink it dissolved in a small
amount of clean water in a cup of spoon
➢ Pain management: Analgesics for the abdominal pain.
• There is no role for antisecretory or antimotility agents in treatment of diarrhea
in children.

ZINC SULPHATE AND DIARRHEA

• There are several mechanisms of action of zinc in diarrhea, these include:
➢ Re-epitheliazation: zinc restores mucosal barrier integrity and
enterocyte brush boarder enzyme activity
➢ Promotes the production of antibodies and circulating lymphocytes
against intestinal pathogens
➢ Has a direct effect on ion channels acting as a potassium channel
blocker of cAMP mediated chloride secretion
• Zinc therefore reduces the duration, morbidity and mortality of diarrhea
both for the current episode and next episodes in the next 3 months.

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198
PREVENTION OF DIARRHEA
• Improve water and sanitary facilities
• Promote good personal and domestic hygiene e.g. handwashing with soap
• Promote exclusive breastfeeding: exclusive breastfeeding promotes very young
infant from diarrhea through passive immunity and through reduction in intake
of potentially contaminated food and water. Evidence even shows that in HIV
endemic populations in developing countries, exclusive breastfeeding may
reduce risk of mortality without enhancing the rates of mother to child
transmission of the virus through breast milk.
• Safe complementary feeding practices.

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• Rotavirus immunization. Other vaccines that could potentially reduce the burden
of severe diarrhea and mortality in young children are vaccines against Shigella
and enterotoxigenic E. coli (ETEC).
CHRONIC DIARRHEA
• This is diarrhea of insidious onset lasting more than 14 days. It is a complex
condition with multitude of etiologies.
MECAHNISMS OF DIARRHEA
• Osmotic e.g. lactose intolerance
• Secretory e.g. cholera
• Mixed secretory-osmotic e.g. Rotavirus
• Mucosal inflammation e.g. invasive bacteria, inflammatory bowel disease
• Motility disturbance
CAUSES
• Infections: giardia, cryptosporidium, Isospora belli and microsporidia in AIDS
patients.
• Non-infectious:
➢ Inflammatory bowel disease
➢ Celiac disease
➢ Tropical sprue
➢ Pseudomembranous colitis
➢ Thyrotoxicosis
➢ Diabetes
CLINICAL FEATURES
• Diarrhea, bloody diarrhea or steatorrhea
• Abdominal pain and vomiting
• Weight loss
• Anemia
INVESTIGATIONS
• Stool microscopy, culture and sensitivity- look for ova and parasites
• Full blood count and ESR
• Rectal/jejunal biopsy and barium enema may be considered in some patients

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MANAGEMENT
• Investigate child adequately and treat underlying cause.
• Fluid therapy- oral fluid use should be stressed except for patients presenting with
severe dehydration in whom intravenous fluids should be used. However, even
with severe dehydration, oral fluids should be given concurrently.

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 ENTERIC FEVER

ENTERIC FEVER
• Enteric fever is an acute systemic illness characterized by fever, headache and
abdominal discomfort caused by Salmonella enterica variant typhi (and less
commonly Salmonella paratyphi A, B, or C).
• Man is the only known host for S. typhi.
• Enteric fever is a misnomer, in that the hallmark features of this disease- fever
and abdominal pain are variable.
• The organism enters the body via the gastrointestinal tract and gains access to the
bloodstream via the lymphatics.
• The disease was initially called typhoid fever because of its clinical similarity to
typhus.
• Enteric fever is a common cause of fever lasting for more than 7 days in clinical
practice.
STRUCTURE OF SALMONELLA
• Salmonella is a member of the family Enterobacteriaecae.
• It is a flagellated, encapsulated, facultative anaerobic non-spore forming gram
negative bacilli.
• Like other organisms in the family the bacterium has:
➢ Somatic antigen (O antigen)
➢ Flagella antigen (H antigen)
➢ Capsular antigen: Vi (K)
PATHOGENESIS
• Transmission is via fecal oral route.
• The infective dose of typhoid/paratyphoid bacillus varies from 103 to 106
organism.
• Incubation period is 3 to 21 days.
• After ingestion, the organism should survive the gastric barrier to reach the small
intestine hence conditions which reduce gastric acidity such as use of antacids,
H2 receptor blockers and proton pump inhibitors reduce the infective dose
(increase chance of infection).
➢ Gastric acid is protective so for infection to happen its either there is low
gastric acid secretion, immunocompromise or a large inoculum.

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• Infection with salmonellae is characterized by attachment of the bacteria by
fimbriae/pili to cells lining the intestinal lumen.
• The organism specifically infects the M cells of the Peyers patches (in the
terminal part of the ileum).
• The bacteria are internalized by receptor mediated endocytosis and transported
with phagosomes to lamina propria where they are released.
• Once in the lamina propria to salmonella induces an influx of macrophages
(typhoidal strains) or neutrophils (non-typhoidal strains),
➢ The Vi antigen is important in preventing antibody mediated opsonization and
complement- mediated lysis.
• The S. typhi organisms through induction of cytokine release spread through the
reticuloendothelial system to the regional lymph nodes (Especially the
mesenteric lymph nodes).
• After of about 7 to 14 days, bacteremia results and there is Did you know?
spread to the phagocytes of the liver, gallbladder, spleen, Mary Mallon aka Typhoid
bone marrow, and Peyers patches of the ileum. This marks Mary was an Irish-born
the onset of clinical manifestations of enteric fever. Infection cook believed to have
leads to both local and systemic immune response which are infected 53 people, 3 whom
however, inadequate to prevent relapse or reinfection. died, with typhoid fever,
• The usual site of carriage is the gallbladder (chronic carrier is and the first person in the
associated with gall stones). US to be identified as an
➢ In some areas endemic for urinary schistosomiasis chronic asymptomatic carrier of the
carriage is in the urinary bladder. disease
• The pathogen can be excreted into bile and feces further
transmitting the disease.
CLINICAL FEATURES
• A pro-drome of nonspecific symptoms often precedes fever and include:
➢ Chills
➢ Dull Headache
➢ Anorexia
➢ Nausea
➢ Cough
➢ Weakness/malaise,
➢ Sore throat
➢ Dizziness
➢ Muscle pains

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• Gastrointestinal symptoms are quite variable.
• The onset of illness is insidious and non-specific with persistent step ladder fever
(lasting more than one week, caused by endotoxins), headache, asthenia
(someone is weak and does not have any strength to walk), insomnia, anorexia,
epistaxis and abdominal pain.
➢ The fever starts as low-grade fever and then shows stepwise increase peaking
to as high as 39.4oC to 40oC by the end of the 1st week.
➢ Recall in viral infections, fever the peak is usually at the onset of the fever
• Patients can present with either diarrhea or constipation (due to hypertrophy of
Peyers patches)
➢ Diarrhea is more common among patients with AIDS and among children <1
year of age.
➢ Diarrhea occurs early but usually disappears by the time fever and bacteremia
occur.
• After 1 week as bacteremia becomes sustained, high fever, delirium, tender
abdomen and enlarged spleen occur.
• Rose spots (rose colored macules) on the abdomen may be seen but rarely occur.
• Leukopenia and anemia are often seen.
• Physical findings:
➢ Coated tongue
➢ Rose spots (rarely seen)
➢ Abdominal tenderness
➢ Lymphadenopathy
➢ Hepatosplenomegaly
• Within the 3 weeks without treatment serious complications can arise such as:
➢ Meningitis
➢ Encephalitis
➢ Coma
➢ Lobar pneumonia
➢ Myocarditis
➢ Splenic abscess
➢ Hepatitis
➢ Cholecystitis
➢ Osteomyelitis
➢ Disseminated intravascular coagulation
➢ Intestinal perforation and acute abdomen

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 ➢ Peritonitis
➢ Intestinal hemorrhage: due to erosion of a necrotic Peyers patch through the
wall of a vessel. It is usually mild but can be life-threatening.
• The term severe or complicated enteric fever is used for patients presenting with
neurological complications (delirium, coma, obtundation, stupor) and/or shock
• The fourth week of illness is characterized by gradual improvement but 30% of
those infected will die and 10% of untreated will relapse.
• After clinical recovery 5-10% of patients will continue to excrete the pathogen
several months (convalescent carriers)
• 1-4% will carry organisms for more than 1 year (Chronic carriage)
➢ Carriage in the gall bladder and urinary bladder.
DIAGNOSIS
• Definitive diagnosis: culture of S. typhi or S. paratyphi from patient
➢ Blood culture is positive in most cases in first 2 weeks.
➢ Culture from intestinal secretions and urine is also used.
➢ Bone marrow culture is more sensitive (gold standard) than blood culture but
is rarely required in patients on antibiotics.
➢ Culture media- bile broth media.
➢ Collect 10ml in adults and 5ml in children and a blood: media ratio of 1: 5
should be maintained.
• Remember “BUS”, You find the parasite in blood in first week, urine in the
second week and Stool in third week
• FBC:
➢ Leukopenia with absolute eosinopenia and neutrophilic predominance is
common but non-specific.
➢ Anemia and thrombocytopenia in advanced stages
• CRP: high (this helps distinguish enteric from viral fevers especially dengue)
• There may be mild elevation of transaminases to 2-3 times normal (AST>ALT)
• Serological test as Widal antigen test are of little practical value.
➢ It is easily misinterpreted
➢ Sensitivity and specificity are poor.
➢ It detects presence of IgG and IgM antibodies to:
o H (flagellar antigen) common to typhi and paratyphi A and B.
o O (somatic antigen) common to typhi and paratyphi A and B.
➢ Anti O titers are both IgG and IgM that rise and decline early, while anti-H
are primarily IgG that rise and decline late in course of the disease.

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 ➢ Conventionally, demonstration of 4-fold rise in antibody titers in two samples
is considered positive.
➢ A single titer of at least 1:160 for both O and H is also considered positive.
DIFFERENATIAL DIAGNOSIS
• Malaria
• Urinary infections
• Lower respiratory tract infection
• Dengue fever in endemic areas.
MANAGEMENT AND PREVENTION
• Isolate the patient.
• Children with persistent vomiting, inability to take orally, severe diarrhea or
abdominal distension usually require intravenous antibiotics therapy and
intravenous fluids necessitating admission to hospital.
• Keep under close surveillance, hydrate, treat fever.
• Resistance has been seen with drugs that were used as first line such as
chloramphenicol (50mg/kg/day), trimethoprim, sulfamethoxazole and
ampicillin.
• Resistance to nalidixic acid has been suggested as a marker to fluoroquinolone
failure.
• Third generation cephalosporins e.g. ceftriaxone and cefixime are the drug of
choice.
• Fluoroquinolones may still be considered the drug of choice in areas where
quinolone resistance is infrequent.
• Oral route is more effective than parenteral, if patient cannot take oral treatment
start by injectable route and change to oral route as soon as possible.
• Uncomplicated enteric fever:
➢ Ciprofloxacin PO for 5 to 7 days
➢ Children: 30mg/kg/day in 2 divided doses (usually not recommended in
children under 15 years however, the life-threatening risks of typhoid
outweighs the risk of adverse effects)
➢ Alternative:
o Cefixime PO for 7 days in children under 15.
▪ For those over 6 months: 15 to 20mg/kg/day in 2 divided doses.
▪ Adults: 1g/day in 2 divided doses
o Azithromycin 10-20 mg/kg/day

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• In patients presenting severe typhoid with toxic confusional state (hallucinations,
altered consciousness) or complications:
➢ IV ceftriaxone and cefotaxime can be used at a dose of 100mg/kg/day
o Parenteral treatment is continued until defervescence has occurred, oral
intake has improved and complications resolved. Thereafter, oral cefixime
can be given to complete a total duration of 14 days.
o Other drugs that can be used for switch therapy: Azithromycin,
cotrimoxazole and amoxicillin
o In patients with penicillin or cephalosporin allergy, azetreonam,
chloramphenicol (in higher than usual dose) or cotrimoxazole (in higher
than usual dose) can be used as second line.
➢ Dexamethasone: IV loading dose 3mg/kg and then 1mg/kg every 6 hours for
2 days.
➢ Second line drug in resistant cases-Imipenem
• If cultures are positive and show quinolone sensitive, change to ciprofloxacin
20mg/kg/day. (quinolones are associated with faster defervescence and lower
relapse rate as compared to ceftriaxone).
• If culture is positive and shows quinolone resistance as well as resistance to
ampicillin, chloramphenicol and cotrimoxazole switch to ceftriaxone.
• If cultures are negatives and defervescence has not occurred by day 7, a thorough
search for alternative etiology for fever should be made and ceftriaxone
continued. There is no role for changing the antimicrobial agent or adding another
drug, since ceftriaxone resistance is still anecdotal.
• Relapses:
➢ Commonly seen with beta lactams (ceftriaxone, cefixime) especially if shorter
duration of therapy is used.
➢ Relapse can be satisfactorily treated with the same drug as used for primary
therapy but at appropriate dose and duration.
➢ If the isolate is quinolone sensitive and fluoroquinolones were not used for
primary therapy, they should be used for treatment of the relapse.
• Carriers: this is uncommon in children and testing for chronic carriage 3 months
after episode of enteric fever is not recommended however, if demonstrated:
➢ Amoxicillin (100mg/kg/day) with probenecid (30mg/kg/day) or
➢ Cotrimoxazole (10mg/kg/day) for 6-12 weeks
➢ If stain is nalidixic acid sensitive quinolones for 28 days is a better option.
• Prevention:

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➢ Disinfection of feces with 2% chlorine solution
➢ Individual (hand washing) and collective hygiene (safe water supply,
sanitation)
➢ The possibility of vaccination must be considered: it can be useful in some
situations (high-risk-age group, hyper-endemic zone), but its effectiveness
remains controversial as both injectable inactivated or oral live attenuated
vaccines only give partial protection.

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 CHOLERA

CHOLERA
• Cholera is caused by Vibrio cholerae. It is an illness characterized by excessive
diarrhea and vomiting.
• Vibrio cholerae is a gram negative, motile, comma shaped organism with a
flagellum.
• Two pathogenic strains V. cholerae 01 and 0139 are known.
• The route of transmission is feco-oral.
• Contaminated water serves as a reservoir and frequently the source of infection.
• Other sources of infection include contaminated foodstuffs, utensils and
houseflies.
PATHOGENESIS
• Cholera has one of the shortest incubation period of 6 hours to 5 days.
• After ingestion, the organisms have to pass through the acid barrier of the
stomach.
➢ In the stomach they inactivate almost all their biological processes
• Once they survive the stomach acid they colonize the upper small intestines.
• For colonization, a relatively large inoculum of V. cholera is required.
• The organisms produce an enterotoxin, cholera toxin which causes symptoms.
➢ The cholera toxin has 2 subunits i.e. an A and B subunit.
➢ The B unit has affinity for intestinal epithelial mucosa
➢ The A unit enters the enterocytes and activates adenylyl cyclase which
increases the intracellular concentration of cAMP.
➢ This results in the excretion of chloride into the lumen leading to decreased
absorption of sodium from villous cells.
➢ As sodium absorption is impaired, water is poured out from intestinal
epithelium.
➢ The outpouring of fluid and electrolytes produces the watery diarrhea and
related changes.
CLINICAL FEATURES
• Infection can be mild self-limiting or even asymptomatic.
• Severe infection leads to profuse watery diarrhea accompanied by vomiting.
• In young children there can be significant fever.

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• The stools are watery with a fishy odour and the mucus flakes give it the typical
rice water appearance.
• The fluid and electrolyte loss can be massive leading to symptoms and signs of
severe dehydration and even circulatory collapse and acute renal failure.
• The outpouring of watery diarrhea can continue for 5-7 days.
• Classically, it has 3 phases:
➢ Evacuation phase: abrupt onset of painless, profuse watery diarrhea associated
with vomiting in severe forms. Stools may be rice-water.
➢ Collapse phase: if appropriate treatment is not given. It is characterized by
features of circulatory shock (cold clammy skin, tachycardia, hypotension and
peripheral cyanosis) and dehydration (sunken eyes, hollow cheeks and
diminished urine output). There may also be muscle cramps. Children may
also have convulsion due to hypoglycemia. Complications such as renal
failure and aspiration of vomitus may occur.
➢ Recovery Phase occurs if the patient survives the collapse phase
DIAGNOSIS
• History of massive watery diarrhea (rice water) in a patient returning from or
residing in an endemic area.
• Examination of fresh stool sample as a hanging drop preparation under the
microscope can show the darting motile V. cholerae.
• There are generally no fecal leucocytes.
• Stool culture confirms the diagnosis as well as helps in identifying the type V.
cholerae.
• It is best cultured on thiosulphate citrate bile sucrose media (TCBS)
• Estimation of serum electrolytes and blood sugar levels is useful for appropriate
management of sick children.
TREAMENT
• Fluid and electrolyte replacement (See management dehydration).
• If the child can take orally then ORS should be given. Continue ORS till diarrhea
stops. Continue breastfeeding normally and encourage the patient to eat.
• In case oral intake is not possible or inadequate, intravenous rehydration is
essential.
• Hyponatraemia, hypokalaemia and acidosis need appropriate attention.
• Antibiotics can help in shortening the duration of illness and possibly the carrier
rate but are generally not used.

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 ➢ Drug of choice is oral tetracycline for 3 days.
o For children >12 years 12.5mg/kg 4 times daily for 3 days
o Adults 500mg 4 times daily for 3 days
➢ Erythromycin can also be used
o Children 10mg/kg 3 times daily for 3 days
o Adults 250mg
➢ In younger children, trimethroprim-sulphamethoxazole (septrin) combination
can be used.
• Prevention is by practicing good hygiene and health measures.
• There are 2 oral vaccines, 1 killed subunit and another live attenuated present but
protection only lasts for 6 months and they do not protect against 0139 strain.
COMPLICATIONS
• Electrolyte imbalance
• Shock and dehydration

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 DYSENTERY

DYSENTERY
• This is the passage of bloody diarrhea or mucus or both in stool.
• There are 2 types:
➢ Bacillary
➢ Amoebic
BACILLARY DYSENTRY (SHIGELLOSIS)
• Shigellosis is caused by shigella group of organisms.
• They are four etiological species:
➢ Shigella dysenteriae
➢ Shigella flexneri
➢ Shigella boydii
➢ Shigella sonnei
• Shigellla species are gram negative facultative anaerobic, non-spore forming
non-motile rod-shaped bacteria closely related to E. coli.
• The infection is more common during warm season and source is contaminated
water and food.
• Shigella can survive in milk for up to 30 days. Flies also excrete shigella. A small
inoculum of 10 to 100 bacteria is adequate to cause disease (unlike cholera which
requires a larger inoculum).
• Asymptomatic individuals can carry Shigella organisms and be a source of
infection.
• Person to person transmission due to poor hand washing also occurs.
PATHOGENESIS
• Shigellas are invasive organisms and affect the colon.
• There is colitis with mucosal edema, ulceration and bleeding.
• The deeper layer of colonic wall, i.e. muscularis mucosa and submucosa can also
be affected by the inflammatory process.
• S. dysenteriae also produces an exotoxin, shiga toxin which can cause watery
diarrhea.
CLINICAL FEATURES
• All four types cause a similar clinical picture though severity may vary.

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• After a short incubation period of 12 hours to 2 days, the clinical presentation
may start with loose stools, abdominal pain, fever, nausea and vomiting.
• Soon the fever becomes higher, there are severe abdominal cramps, tenesmus
with blood in stools.
• Other features: malaise, fecal urgency
• Abdominal examination may reveal distension and tenderness.
• There can be accompanying features of fluid and electrolyte loss.
• In some children, neurological manifestations like convulsions, headache and
lethargy may occur
DIAGNOSIS
• Stool microscopy, culture and sensitivity
➢ Numerous leucocytes seen
➢ Culture proves diagnosis
• Full blood count:
➢ Leucocytosis
➢ Leukemoid reaction can also occur in some children
• Blood culture may be indicated in sick and toxic looking children
MANAGEMENT
• Fluid and electrolyte balance with either oral or intravenous rehydration (see
dehydration)
• Antibiotic therapy is recommended as it shortens the episode and improves the
outcome and also decreases carrier state.
• Antibiotics used should be guided by sensitivity however effective antibiotics
include oral ampicillin, trimethoprim-sulphamethoxazole or Nalidixic acid. In
older children quinolones may also be used.
➢ Nalidixic acid- 50mg/kg orally in 4 divided doses for 7 days or
➢ Ciprofloxacillin- 15mg/kg twice daily for 3 days (Ciprofloxacillin use in
children is contraindicated except if benefits outweigh risks)
COMPLICATIONS
• Dehydration and electrolyte imbalance
• Sepsis and bacteremia can occur with S. dysenteriae and organisms may be
isolated from blood culture.
• Arthritis, conjunctivitis
• Encephalopathy

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• Hemolytic uremic syndrome is mediated by shiga toxin
➢ Hemolytic uremic syndrome is a condition characterized by acute renal failure
in the presence of microangiopathic hemolytic anemia and thrombocytopenia.
➢ Vascular endothelial injury by the shiga toxin is the key to the pathogeneiss
of the injury
➢ The toxin binds to endothelial cells, causing endothelial cell injury, most
especially in the renal vasculature leading to platelet thrombi formation and
renal ischemia.
➢ Recall: antibiotics are not indicated for HUS. In addition, antibiotic treatment
of E. coli hemorrhagic colitis may increase the likelihood that the patient will
eventually develop HUS.
• Toxic megacolon
• Persistent diarrhea and malnutrition
• Rectal prolapse- in malnourished children there can be rectal prolapse due to
tenesmus
• Seizures due to neurotoxin release with S. sonnei.
PREVENTION
• Drink clean, boiled/chlorinated water
• Good sanitation
• Good personal hygiene
AMOEBIC DYSENTERY
• This is caused by the parasite Entamoeba histolytica
CLINICAL FEATURES
• Bloody diarrhea with mucus
• Low grade fever
• Dehydration is unusual
DIAGNOSIS
• Stool microscopy
• Sero diagnosis
TREATMENT
• Fluid replacement (See dehydration)
• Analgesics
• Antibiotics

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 ➢ Metronidazole
o 1-3 years, 200mg orally TDS for 5 days
o 3-7 years, 200mg orally QID for 5 days
o 7-10 years, 400mg orally TDS for 5 days
➢ Tinidazole 50-60mg/kg orally for 3 days
• Avoid use of anti-diarrhea agents
COMPLICATIONS
• Fulminant colitis
• Colon perforation
• Peritonitis
• Chronic infection
• Stricture formation
• Severe hemorrhage
• Amoebic liver abscess
• Amoeboma
PREVENTION
• Good disposal of excreta- good pit latrines, flush toilets
• Provision of clean water
• Boiling water. This kills amoeba cysts if water is boiled for at least 10 minutes
• Chlorination of water- effects variable on amoeba
• Personal hygiene- washing of hands after use of toilet, when preparing food and
before eating.

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 CHAPTER 12: GENITOURINARY TRACT o IgA nephropathy,
o Membranoproleferative
glomerulonephritic and
GENITOURINARY o PSGN
TRACT ➢ Nephrotic syndrome that results
from systemic disease:
NEPHROTIC o Systemic lupus
SYNDROME erythematous
• This is a condition characterized by o HSP
heavy proteinuria (> 50mg/kg/24
hours), hypoalbuminemia,
PATHOPHYSIOLOGY
hypercholesterolemia and edema. • The basic physiologic defect is a
• 2/3 of cases present before 5 years loss of the normal charge and size
selective glomerular barrier to the
of age.
filtration of plasma proteins.
• In young children the ratio of boys
• Excessive urinary protein losses
to girls is 2:1.
lead to hypoproteinemia of
• By late adolescence, both sexes are
nephrotic syndrome.
equally affected.
• Due to hypoproteinemia,
CLASSIFICATION hypercholesterolemia results
• There are 3 categories of nephrotic ➢ Reduced plasma oncotic
syndrome: pressure induces increased
➢ Primary nephrotic syndrome hepatic production of plasma
o These are not a consequence proteins, including lipoproteins.
of systemic disease. ➢ Plasma lipid clearance is
o Account for 90% of all reduced because of reduced
childhood cases of nephrotic activity of lipoprotein lipase in
syndrome. adipose tissue.
o The most common cause is
CLINICAL FEATURES
minimal change disease
• Edema: ranges from mild
(MCD)-90% in young
children and 50% in older periorbital edema (particularly on
children and adolescents waking) to scrotal or labial edema
➢ Nephrotic syndrome from other to widespread edema (leg, ankle
edema and ascites).
primary glomerular disease
including:

216
• The edema often follows an upper
respiratory infection (URI). Pleural
effusions (causing breathlessness)
and hypotension may also occur.
• Patients may rarely be
asymptomatic at time of diagnosis.
• Patients are predisposed to
thrombosis secondary to
hypercoagulability (loss of anti-
thrombin III). Patients may present
with stroke or other thrombotic
events such as renal vein
thrombosis and sagittal sinus
thrombosis.
• Patients are also at an increased risk
for infection with encapsulated
organisms, such as S. pneumoniae
and therefore may present with
spontaneous bacterial peritonitis,
pneumonia or overwhelming
sepsis.
➢ Urea and Electrolytes,
Creatinine and lipid studies:
o Metabolic acidosis which
may be caused by renal
tubular acidosis
o Hypoalbuminemia and
elevated serum cholesterol
o BUN and creatinine should
be measure to assess for
renal impairment
➢ C3, ANA and antistreptococcal
antibodies are indicated to rule
out causes other than MCD.
➢ Renal ultrasound: may show
enlarged kidneys
➢ Renal biopsy: rarely indicated in
children with typical nephrotic
syndrome unless the creatinine
clearance is impaired or initial
management with
corticosteroids is ineffective.

DIAGNOSIS MANAGEMENT
• Based on clinical features and the • Treatment is dictated by the
following studies: underlying cause.
➢ Urinalysis • Supportive therapy is universally
o 3+ to 4+ proteins indicated.
o Microscopic hematuria ➢ Hospitalization
o Elevated TP/CR ➢ IV infusion of 25% albumin in
o Presence of RBC casts children with widespread
indicate a cause other than edema, scrotal or labial edema,
MCD hypotension or symptomatic
➢ Full blood count may show pleural effusions.
o Elevated hematocrit (due to ➢ Salt restriction
hemoconcentration resulting • Most patients with MCD respond to
from hypoproteinemia) therapy with corticosteroids:
o Thrombocytosis steroid-dependent or steroid

217
 resistant patients may respond to
cyclophosphamide or cyclosporine.
• Because of the risk of
pneumococcal infection, if the
child is febrile, evaluation should
include blood culture, urine culture
and chest radiography
➢ If peritonitis is suspected,
paracentesis for Gram stain,
culture and cell count of the
ascites fluid is indicated.
➢ Empiric broad spectrum IV
antibiotics coverage should be
initiated.
PROGNOSIS
• 5% mortality in steroid resistant
patients is due overwhelming
infection or thrombosis.
• In steroid sensitive patient relapses
typically occur with varying
frequency but often disappear by
completion of puberty.
➢ Less than 10% who are initially
steroid sensitive develop end-
stage renal disease (ESRD)
resulting in focal sclerosing
glomeruloscleross (ESGS).
➢ Majority of patients with steroid
resistant or dependent nephrotic
syndrome eventually develop
end-stage renal disease.

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 DERMATOLOGY
CHILDHOOD
EXANTHEMS
• An exanthem/ exathema (Greek for
“a breaking out”) is a widespread
rash usually occurring in children.
• It can be caused by toxins, drugs, or
micro-organisms or can result from
autoimmune disease.
• It can be contrasted with an
enanthem.
➢ An enanthem/enanthema is a
rash (small spots) on the mucous
membranes.
➢ It is characteristic of patients
with smallpox, measles, chicken
pox and roseola infantum.
➢ It can also indicate
hypersensitivity.
➢ Enanthema can also present
with viral exanthema.
• Historically 5 classical infectious
childhood exanthems have been
recognized.
• 4 of them are viral.
• They include:
➢ Measles (Rubeola)/English
measles/ ‘First disease’
CHAPTER 13: DERMATOLOGY o Caused by measles virus
➢ Scarlet fever/ ‘Second disease’
o Caused by Streptococcus
pyogenes
➢ Rubella/German measles/ ‘third
disease’
o Caused by rubella virus

219
 ➢ Erythema infectiosum/’Fifth
disease’
o Caused by Parvovirus B19
➢ Roseola infantum: caused by
HHV-6 and HHV-7
• Note “fourth disease” a condition
whose existence is not widely
accepted today was described in
1900 and is postulated to be related
to staphylococcus aureus.
• Many other common viruses apart
from the above mentioned can also
present with an exanthema as part
of their presentation although not
classically considered as part of the
classic numbered list, these
include:
➢ Varicella zoster (chicken pox or
shingles)
➢ Mumps
➢ Rhinovirus (common cold)
➢ Unilateral laterothoracic
exanthema of childhood
➢ Some types of hemorrhagic
fevers
➢ Tick borne diseases e.g. Rocky
Mountain spotted fever
MEASLES
• Measles/morbilli/ rubeola/ red
measles/ English measles is a
highly contagious infectious
disease caused by the measles virus
(a single stranded negative sense
enveloped virus of the genus
Morbillivirus within the family
paramyxoviridae).
220
• The virus is highly contagious and
is transmitted through droplets
from the secretions of the nose and
throat.
• The virus can live up to 2 hours in
the airspace or nearby surface.
• Humans are the only known hosts
of the virus.
• Risk factors for infection:
➢ Immunodeficiency (HIV/AIDs)
➢ Immunosuppression after organ
transplant
➢ Drugs: Alkylating agents/
corticosteroid therapy
➢ Travel to areas with measles
➢ Loss of passive inherited
antibodies before age of routine
immunization
PATHOPHYSIOLOGY
• Once the measles virus gets onto
the mucosa it infects the epithelial
cells of trachea/bronchi.
• It attaches to its target receptor with
the surface protein hemagglutinin
(H protein).
• Once bound the fusion/ F protein
helps the virus ultimately get inside
the cell.
• The virus is a single stranded RNA
virus and is negative sense so it has
to be transcribed by RNA
polymerase into a positive sense
mRNA strand.
• After that it is ready to be translated
into viral proteins, wrapped in the
 cell’s lipid envelope and sent out of
the cell as newly made virus.
• Primary viremia occurs resulting in
infection of the reticuloendothelial
system
➢ Within days the measles virus
spreads through local tissue and
is picked up by dendritic cells
and alveolar macrophages that
carry it from the local tissue in
the lungs through blood to the
local lymph nodes.
• Secondary viremia results in
systemic symptoms
➢ From the local lymph nodes it
continues to spread and reaches
more lung tissue as well as
organs like the intestine and the
brain.
• The incubation period is about 10
days.
• The disease is most common in
preschool children, infants are
protected by transplacental
antibodies which generally decay
by 9 months (hence the rationale for
vaccination at this age).
CLINICAL FEATURES
• Symptoms usually develop 10-12
days post exposure and last 7-10
days.
• Clinical features include a classic
prodrome, followed by a transient
enanthem (rash on mucous
membranes) and a characteristic
exanthema (rash on the skin)
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• Initial symptoms include:
➢ Fever (>40 degress)
➢ Cough: dry hacking
➢ Runny nose
➢ Inflamed eyes
• The classic symptoms include a 4-
day fever (the 4D’s) and the 3 C’s:
➢ Cough
➢ Coryza (head cold, fever, and
sneezing)
➢ Conjunctivitis (red eyes)
• Small white spots known ‘Koplip’
spots may form inside the mouth 2
or 3 days after the start of
symptoms.
➢ They are commonly seen inside
of the cheeks opposite the lower
second molars.
➢ They are diagnostic of measles.
• The rash usually starts on the face
and then spreads to the rest of the
body typically begins 3 to 5 days
after the start of symptoms.
• The rash coincides with the rise in
fever.
• The rash is described as generalized
red maculopapular rash.
• It starts on the back of the ears and
after a few hours, spreads to the
head and neck before spreading to
cover most of the body.
• It covers the lower extremities by
the second day, becomes confluent
by the third day and lasts 4-7 days.
• The rash is said to “stain” changing
color from red to dark brown before
disappearing.
DIAGNOSIS
• The basis of diagnosis is clinical
features (the 3C’s, koplik’s spots).
• Confirmation of measles infection
is by serologic testing:
➢ Measles IgM antibodies
(present 3 days after rash and
persist 1 month) or
➢ Isolation of Measles virus RNA
from respiratory specimens
➢ If blood can’t be drawn salivary
IgA specific to measles can be
tested.
• Measles need to be differentiated
from other childhood
exanthematous illnesses.
➢ The rash is milder and fever less
prominent in rubella, enteroviral
and adenoviral infections
➢ In roseola infantum, the rash
appears once fever disappears
while in measles the fever
increases with rash.
➢ In rickettsial infection, the face
is spared which is always
involved in measles.
➢ In meningococcemia, the upper
respiratory symptoms are absent
and the rash rapidly becomes
petechial.
➢ Drug rashes have history of
antecedent drug intake.
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➢ In Kawasaki disease, glossitis,
cervical adenopathy, fissuring
of lips, extreme irritability,
edema of hands and scaling are
distinguishing clinical features.
MANAGEMENT
• Supportive care is most important.
➢ Antipyretics
➢ Maintenance of hygiene
➢ Ensuring adequate fluid
➢ Caloric intake and
humidification
• Vitamin A has shown to improve
outcome.
• Immunoglobulin can be used for
postexposure prophylaxis in high-
risk individuals (e.g. children with
HIV and other immunodeficiency
states) who are exposed to measles.
• Complications should be managed
appropriately.
• Measles is a preventable and
potentially eradicable disease
through universal immunization
(MMR vaccine)
COMPLICATIONS
• Complications are more frequent in
the very young, malnourished and
the immunocompromised.
• Bacterial pneumonia is the most
common complication and the most
common cause of mortality.
➢ Usual bacterial pathogens:
pneumococcus, Staphylococcus
 aureus and sometimes gram- streptococcus (GABHS) that
negative bacteria produce erythrogenic toxin.
• Otitis media is also common • The peak incidence is in winter and
• Laryngotracheitis. spring.
• Giant cell pneumonia • Transmission is by large respiratory
• Bronchiectasis droplets or by infected nasal
• Flaring up of latent M. tuberculosis secretions.
infection CLINICAL FEATURES
• Encephalomyelitis (inflammation
• The exanthema may develop during
of both brain and spinal cord)
any GABHS infection (e.g.
• Subacute sclerosing impetigo, cellulitis, pharyngitis).
panencephalitis is a rare late
• Before or during the exanthema,
complication.
fever, chills, malaise and often an
• GIT complications: exudative pharyngitis may occur.
➢ Diarrhea
• The exanthema has the following
➢ Appendicitis
characteristic features:
➢ Hepatitis
➢ Begins on the trunk and mover
➢ Ileocolitis
peripherally
➢ Precipitated malnutrition and
➢ Skin is erythematous with tiny
cause noma or gangrene of the
skin-colored papules
cheeks
(scarlatiniform appearance) and
has the texture of sandpaper
(sandpaper rash). The rash
blanches with pressure.
➢ Petechiae are often localized
within skin creases in a linear
distribution (“Pastia’s lines”).
➢ Desquamation of dry skin
occurs as the infection resolves.

SCARLET FEVER DIAGNOSIS

• Scarlet fever is a toxin-mediated
bacterial illness that results in a
characteristic skin rash.
• The cause of infection is strains of
group A beta hemolytic
• The basis of diagnosis is clinical
features and a positive throat
culture for S. pyogenes (gold
standard) or positive rapid

223
 streptococcal tests that detect
GABHS antigen.
RUBELLA
MANAGEMENT
• The goal is to prevent development
of rheumatic fever.
• Appropriate antibiotics include oral
penicillin VK, intramuscular
benzathine penicillin or for
penicillin-allergic patients
erythromycin or macrolides.
COMPLICATIONS OF GABHS
INFECTION
• Post-streptococcal
glomerulonephritis: may occur
several weeks after streptococcal
pharyngitis. Patients present with
hypertension and cola-colored
urine. Antibiotic therapy does not
prevent this complication.
• Rheumatic fever
• Post-streptococcal arthritis:
characterized by joint symptoms
(without other features of
rheumatic fever) that may last for
weeks. Antibiotic therapy does not
prevent this complication.
• Pediatric autoimmune
neuropsychiatric disorders
associated with streptococcal
infection is a phenomenon in which
patients develop the acute onset
obsessive compulsive symptoms or
a tic disorder after streptococcal
infection. Antibiotic therapy prevents
this complication.

224
ENDOCRINOLOGY
DIABETES MELLITUS
• Diabetes mellitus is a metabolic disorder of carbohydrate metabolism
characterized by persistent hyperglycemia and glycosuria due to either insulin
deficiency or resistance.
➢ Normal blood glucose in health individuals should be regulated between 3.5-
8.0 mmol/L (63-144 mg/dl) despite varying demands of food, fasting and
exercise.
➢ Normal fasting blood sugar (after at least 8h fast) = 3.5-5.5 mmol/L (63-
100mg/dl).
➢ Normal random blood sugar= 4.4-7.8 (70-144mg/dl).
➢ Pre-diabetic=
o Impaired fasting blood glucose= 5.6-6.9 mmol/L
o Impaired oral glucose tolerance= 7.8-11.1 mmol/L.
➢ Note: to change from mg/dl to mmol/L divide by the factor 18.
• This is the second most common chronic disease of childhood, affecting 1 of 500
children. Diabetes mellitus is the leading cause of end-stage renal disease, non-
traumatic leg amputation and adult blindness.
• It affects boys more than it does girls (2:1).
• The factors that contribute to hyperglycemia include decreased insulin secretion,
decreased insulin action and increased glucose production.
• Based on etiology the types of diabetes include:
➢ Primary diabetes mellitus (Idiopathic):
o Type 1: formerly known as insulin dependent (idiopathic or autoimmune
B-cell destruction): absolute insulin deficiency result from beta-cell
destruction.
o Type 2: formerly known as non-insulin dependent (Defects in insulin
secretion and action): there is peripheral resistance to insulin and a relative
insulin deficiency.
➢ Secondary diabetes:
o Infectious: Congenital rubella, cytomegalovirus, Coxsackie & other
enteroviruses, Mumps, Reoviruses
o Diseases of the exocrine pancreas: Trauma, cystic fibrosis, Pancreatitis,
hemochromatosis

225
 o Drugs: Corticosteroids & immunosuppressives (ciclosporin, Tacrolimus),
HIV drugs (protease inhibitors), phenytoin and antipsychotics (clozapine,
olanzapine)
o Genetic disorders: Down’s syndrome, Turner’s syndrome, Myotonic
dystrophy and Huntington’s chorea
o Endocrinopathies: acromegaly, Cushing’s syndrome, Pheochromocytoma,
somatostatinoma and aldosteronoma
➢ Gestational diabetes: Occurs when diabetes onsets during pregnancy.
➢ Other specific types: maturity onset diabetes of the young (MODY)
o MODY comprises a group of dominantly inherited forms of relatively mild
diabetes. Insulin resistance does not occur in these patients, instead the
primary abnormality is an insufficient insulin secretory response to
glycemic stimulation. Treatment depends on the type and can include the
use of sulfonylureas.

Figure 22: Classification of DM in adolescents and children

226
• Almost all children have type 1 diabetes requiring insulin from the outset.
• Type 2 diabetes due to insulin resistance is starting to occur in childhood, as
severe obesity becomes more common.
• Type 1 DM is uncommon in infants. The incidence of diabetes mellitus increases
in children with advancing age all the way to adolescence, with peaks at 5 and 12
year of age.
• Occasionally, an adolescent with type 2 diabetes may present with ketoacidosis
and patients with type 1 Diabetes mellitus may present late and progress slowly.
• For diagnosis of diabetes mellitus:
➢ Symptoms of diabetes plus one of the following
o Fasting blood sugar >7mmol/L (126 mg/dl)
o Random blood sugar ≥11.1 mmol/L (200mg/dl)
o 2-hour postprandial plasma glucose >11.1 mmol/L (200mg/dl) after a
glucose load of 75g in 300ml of water (during an oral glucose tolerance
test)
o HBA1C >6.5%
➢ If there are no symptoms an abnormal fasting blood sugar, random blood
sugar and glucose tolerance test must be present to make a diagnosis.
➢ Note: these criteria should be confirmed by repeat tests on a different day.
• Impaired glucose tolerance (IGT) indicates blood glucose levels between normal
and diabetic cut off points during glucose tolerance test. (between 7.8-
11.0mmol/l 2 hours after glucose load of 75g).
TYPE 1 DIABETES MELLITUS
PATHOGENESIS
• Type 1 DM develops due to immune-mediated destruction of pancreatic cells,
resulting in severe impairment of insulin secretion in genetically susceptible
children.
• In type 1 DM etiology is multifactorial with genetic, environmental and
autoimmune factors:
➢ Genetic factors:
o There are strong genetic influences but inheritance has not been found to
fit into the Mendelian patterns (autosomal or X-linked).
o About 95% of patients have HLA-DR3 or DR4.
o Monozygotic twins have a 50% concordance rate, whereas dizygotic twins
have only have a 30% concordance rate.

227
 o 7% of children whose fathers have type 1 DM develop type 1 DM. Mothers
with type 1 DM do not confer a similar risk
o Siblings are not at higher risk of developing type 1 DM.
➢ Environmental triggers:
o Viral infections have been implicated including enteroviruses (coxsackie),
mumps, reoviruses and rubella.
o Whether the early introduction of cow’s milk (protein) might trigger DM
is controversial.
o Nitrosourea containing compounds have also been implicated
➢ Autoimmune factors: the autoimmune process begins with lymphocytic
infiltration of the pancreas (Lymphocytic insulinitis). Once the islet cells are
completely destroyed inflammation abates and the islets atrophy
o Clinical diabetes occurs when the pancreas loses 80% or more of its insulin
secretary ability
o Islet cells antibodies (ICA) present in 85% of patients such as IA-2/ICA-
512 and GAD-65
o These antibodies may be detected in asymptomatic patients 10 years before
the clinical symptoms. They decline after clinical disease has manifested.
o Other markers include: antibodies against insulin and against glutamic acid
decarboxylase (GAD)
• Molecular mimicry probably occurs between an environmental trigger and an
antigen on the surface of the B-cells of the pancreas resulting in an autoimmune
process (especially in genetically predisposed individuals) which damages the
pancreatic B-cells and leading to insulin deficiency.
• Note: up to 10% of the general population may have islet antibodies however to
develop type I DM children must have a combination of these antibodies,
environmental factors and a genetic predisposition.
• Note: counter –regulatory hormones to insulin include: glucagon, cortisol,
catecholamines, thyroxine, GH & somatostatin and Sex hormones

228
 229

Figure 23: Factors causes diabetes type I

CLINICAL FEATURES
• There are 2 peaks of presentation of type 1 DM: preschool and teenagers.
• Usually patients have present with symptoms of diabetes that have been going on
for a month or two prior to seeking physician contact, with an acute increase in
symptoms over the last week.
• Classical presentation: several weeks of:
➢ Polyuria and Nocturia and occasional enuresis (bedwetting)
➢ Polydipsia,
➢ Polyphagia
• As symptoms progress, weight loss (from persistent catabolic state and loss of
calories through glycosuria and ketonuria), fatigue, vomiting and dehydration
(from glycosuria and osmotic diuresis) occur.
• Diabetic ketoacidosis (DKA) may be the initial presentation in 50% of patients.
The younger the patient, the shorter the course of symptoms before DKA occurs.
• DKA may be misdiagnosed if the hyperventilation is mistaken for pneumonia or
the abdominal pain for appendicitis or constipation.
• Adolescents may present with type 1 DM during their pubertal growth spurt with
hormones that are antagonistic to insulin action (specifically growth hormone and
sex steroids)
• Remember: acute infection in young non-diabetic children can cause
hyperglycemia without ketoacidosis.
• Girls who have protected cases of monilial vulvovaginitis (i.e. vaginal
candidiasis) may have early type I DM.

Figure 24: Clinical features of DM

DIAGNOSIS
• Symptoms (polyuria, polydipsia, weight loss or nocturia) plus:
➢ A random blood sugar above 11.l mmol/L (>200mg/dl).
➢ A fasting blood glucose above 7mmol/L (126mg/dl)
➢ 2-hour postprandial plasma glucose >11.1 mmol/L (200mg/dl) after a glucose
load of 75g in 300ml of water (during an oral glucose tolerance test)
➢ Raised glycosylated hemoglobin (HbA1C) >6.5% though this may not be
reliable when dealing with mild elevations of blood sugars.

230
 Figure 25: Criteria for diagnosis of diabetes mellitus

COURSE OF ILLNESS
• Most patients respond to insulin therapy. Once insulin is initiated blood sugars
gradually decline. Often, after around a week of insulin therapy, the need for
exogenous insulin declines due to a transient recovery of insulin secretion
(Honeymoon phase).
• Some children can go completely insulin free during this time. The honeymoon
phase lasts from a few days to a month.
• It can rarely extend as long as one year. Insulin needs increase over time still such
time as when the pancreas can no longer secrete insulin. At this point the daily
insulin requirement plateaus at 0.8-1 unit/kg/day.
MANAGEMENT
• The goals of therapy include:
➢ Eliminate symptoms related to hyperglycemia.
➢ Reduce and delay the complications.
➢ Achieve normal lifestyle and normal emotional and social development.
➢ Achieve normal physical growth and development
➢ Detect associated disease early.
• Symptoms of diabetes abate with blood sugars <11.1mmol/L

231
INSULIN THERAPY
• Principles of insulin therapy:
➢ Daily insulin dosage varies between individuals and changes over time
➢ The correct dose of insulin for any individual is the dose that achieves the best
glycemic control without causing obvious hypoglycemia problems and
achieving normal growth (height and weight).
➢ Dosage depends on many factors
o Age, weight, stage of puberty
o Duration and phase of diabetes, state of injection sites
o Nutritional intake and distribution
o Exercise patterns, daily routine
o Results of blood glucose monitoring, glycated hemoglobin A1c and
intercurrent illnesses
• Types include: short-acting, intermediate-acting, long-acting and very long-
acting.

Figure 26: Types of insulin

• Insulin can be given subcutaneously into the thighs and lower abdominal wall by
fine needle syringes, pen injectors and jet injectors or by infusion pump.
• Current insulin preparations are generated using recombinant DNA technology
and animal insulin (Bovine or Porcine) should be avoided.
• Insulin pumps are now being used in children to achieve better glucose control
and to improve lifestyles.
• Daily insulin requirements 0.5-1 unit/kg/day for prepubertal and 2 IU/kg/day for
adolescents.

232
 ➢ During partial remission phase, total daily insulin is usually 0.5 IU/Kg/day
➢ Prepubertal children (outside the partial remission phase) usually require
insulin of 0.8-1.0 IU/kg/day
➢ During puberty requirement may rise to 1-2 IU/Kg/day
• In most traditional regimens, intermediate or long-acting insulin is utilized to
provide background insulin to maintain glycemic control during the fasting state.
Short-acting insulin is used to provide glycemic control in the postprandial state.
• Current regimen uses soluble insulin (appears clear) and lente (Appears cloudy
like milk)
➢ 2 subcutaneous injections are given before breakfast and at dinner.
➢ Calculate total daily requirement of insulin
➢ 2/3 of total insulin requirement is given in the morning
o 2/3 of this being lente
o 1/3 of this being soluble insulin
➢ 1/3 of total insulin requirement is given at dinner
o 2/3 of this being lente
o 1/3 of this being soluble insulin
• Example if daily requirements is 30IU/day
➢ Morning= 2/3 x 30IU= 20IU (2/3 is lente- 13 IU and 1/3 is soluble insulin-
7IU)
➢ Evening= 1/3 x 30IU= 10IU (2/3 is lente -7IU and 1/3 is soluble insulin-3IU)
• Note:
➢ If using regular insulin, inject 20-30 minutes before each main meal (breakfast
and main evening meal)
➢ If using rapid-acting insulin analogue inject immediately before or after each
main meal (breakfast and main evening meal)
➢ When drawing up a mixed dose of insulin, short acting insulin (clear solution)
should be drawn before the long acting insulin (cloudy solution), as accidental
introduction of longer-acting insulin in short-acting insulin can result in
increasing the duration of effect of short acting insulin.
• A meal is planned incorporating 3 meals and 2 or 3 snacks
• If only soluble insulin is present, then the total daily insulin requirement can be
divided into 4 doses.
• Adjust insulin by reviewing blood sugars (which should be monitored at least 4
times a day prior to meals and bedtime)

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 Figure 27: Target indicators of glycemic control

MONITORING
• Daily blood glucose: before all meals and at bedtime.
➢ Self-monitoring should be regular before and 2 hours after meals and
occasionally at 3am for adjustment of insulin dosage.
• Glycosylated hemoglobin level reflecting diabetic control for the past 2-3
months, should be check every 3 months.
➢ Glycosylated fructosamine is an even accurate indicator to use.
• Watch for hypoglycemia: all patients should have parenteral glucagon available
in case of seizure or coma secondary to low blood sugar.
• Watch for “honeymoon” period. Within a few weeks after initial diagnosis, 75%
of patients exhibit a temporary progressive reduction in their daily insulin
requirements.
➢ This is because of a transient recovery of residual islet cell function, resulting
in endogenous release of insulin in response to carbohydrate exposure.
➢ This honeymoon period may last anywhere from months to 1-2 years.
➢ It can confuse patients and parents if not educated about it early.
• Watch for somogyi phenomenon (rebound effect): A low blood glucose in the
late evening causes a rebound effect in the body leading to hyperglycemia in the
early morning.

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 ➢ This occur when the evening dose of insulin is too high, causing
hypoglycemia in the early morning hours, resulting in the release of counter-
regulatory hormones (epinephrine and glucagon) to counteract this insulin-
induced hypoglycemia.
➢ The patient then has high blood glucose and ketones in the morning.
➢ The treatment is to actually lower the bedtime insulin dose and not to raise it.
• Dawn effect:
➢ It is similar to the somogyi effect in that people experience hyperglycemia in
the morning (3am to 8am) but for reasons that differ.
➢ It results from a rise in early morning blood sugar levels which are triggered
by declining levels of insulin and an increase in growth hormone.
➢ Testing blood sugar levels at 3am and again in the morning can help
distinguish between the somogyi and dawn phenomenon.
➢ Blood sugar that is low at 3am indicates somogyi effect while high or normal
blood sugar at that time suggests the dawn phenomenon.
• Diet: follow the American diabetic association (ADA) diet.
➢ It is important to follow the diet outlined in the plan and to adhere to meal
timings as variation in meal amounts and timings can result in wide
fluctuations in blood sugar with high blood sugars from eating excessively
and low blood sugars with insufficient food intake and delated meals.
➢ Foods with low glycemic index (diet high in vegetables, fruits) and fiber is
encouraged.
➢ The intake of saturated fats should be limited and the intake of trans fats
should be minimized.
➢ 5 Sweeteners (acesulfame, aspartame, neotam, saccharin, sucralose) are
approved for use in children. Excessive use should be avoided.
➢ Total energy intake:
o 55% complex unrefined carbohydrates
o 15% proteins
o 30% fats: <10% should be saturated and polyunsaturated fat.
➢ In patients on twice daily injections of short-acting and intermediate acting
insulin, the carbohydrate intake should be distributed into 3 main meals and 3
snacks in order to avoid hypoglycemia in between meals. Snacks may not be
needed in patients on basal-bolus regimen using peak less long-acting and
short-acting insulin analogue before meals.

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• Regular Exercises: Physical activity is important for children with diabetes. It
increases glucose utilization and insulin sensitivity, improving metabolic control.
It also builds self-esteem. Recommended activities include walking, jogging,
swimming and organized sports
• Education and close follow-up every 3 months. Parents are advised to bring the
child to hospital if the child has altered sensorium, rapid breathing, fruity odor,
signs of dehydration, persistent vomiting or abdominal pains

Figure 28: Exercise and diabetic education

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• At the 3 month visits the following should be done:
➢ Physical examination with specific focus on:
o Assessment of growth, weight and puberty
o Thyroid, injection sites, fundus, foot and neurological examination
o Eye examination if initial eye exam was normal
➢ Assessment of blood sugar records- check the pre-meals and at bedtime.
Periodic measurement should be advised at 2AM and postprandially if blood
sugar range is inconsistent with glycated hemoglobin. Insulin adjustments are
performed, if needed.
➢ Ongoing DM education (prevention and management of hypoglycemia and
sick day principles)
➢ Psychological assessment and referral if needed.
➢ Investigations
o Glycated hemoglobin checked at 3 month intervals
o Thyroid function tests (yearly) to assess hypothyroidism.
o Urinalysis to assess urine microalbumin (normal 30-399mg/ 24 hr)
o Fasting serum lipids (annually)
➢ At each visit always assess nutrition, revisit nutritional plan and advise
regarding physical activity.
COMPLICATIONS
EARLY COMPLICATIONS
• These include:
➢ Hypoglycemia: mostly due to insulin therapy
➢ Diabetic ketoacidosis (discussed later): due to insulin deficiency
➢ Hyperglycemic hyperosmolar state (discussed later)
HYPOGLYCEMIA
• This is a blood sugar less than 3 mmol/L (<2.6 mmol in kids and <4 mmol/L in
hospital) with or without symptoms.
• Triggers:
➢ Insulin therapy (increased insulin): excess dosage or wrong injection
technique
➢ Increased activity/exercise
➢ Decreased food intake
➢ Infection

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• Remember: counter-regulatory hormones (adrenaline, glucagon and cortisol are
secreted to correct hypoglycemia)
• Clinical features:
➢ Onset is usually rapid i.e. a few minutes
➢ Autonomic response:
o Shakes/tremors
o Pallor
o Palpitations and tachycardia
o Sweating
o Nervous
o Hunger
➢ Neuroglycopenia (decreased availability of glucose to the brain)
o Tingling
o Numbness
o Fatigue, drowsiness, confusion, fainting
o Coma, seizures
• Prevention of hypoglycemia should be discussed with the patient and family
during diabetes education sessions.
• Management (Rule of 15):
➢ If child can take orally
o 15g of free sugar are given in form of sugar, honey juice, or carbonated
drink followed by recheck of blood sugar in 15 minutes.
➢ If child cannot take orally
o If child is unconscious glucagon is administered intramuscularly. The dose
is dependent on the age and weight of the child:
▪ Infants: 0.3 mg
▪ Child <25kg: 0.5mg
▪ Child >25kg: 1.0 mg
o If glucagon is unavailable intravenous dextrose is given
▪ 5ml/kg of 10% dextrose
▪ Adolescents: 20-50ml 50% solution or 40-100ml of 25%
INTERMEDIATE COMPLICATIONS
• Lipoatrophy: this is fat atrophy at the injection site. It can be prevented by
rotation of injection site.
• Limited joint mobility: typically noted in the hands. This occurs due to flexion
contractures of the metacarpophalangeal and proximal interphalangeal joints.

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• Growth failure: occurs in children whose diabetes is not well controlled. Mauriac
syndrome occurs with poor control. These children have hepatomegaly, pale skin
and extreme short stature.
• Delay in sexual maturation: is associated with inadequate control of diabetes and
delayed bone age
• Hypolygemic unawareness: this is caused by frequent hypoglycemia associated
with tight control of diabetes. It is due to impaired counter regulatory response to
hypoglycemia. Raising blood sugar targets and prevention of hypoglycemia
usually cause reversal of hypoglycemic unawareness.
CHRONIC COMPLICATIONS
• Microvascular complications:
➢ Diabetic retinopathy:
o This is characterized by microaneurysms and proliferative diseases.
o Previously 80-90% of individuals developed eye disease by 15 years of
diabetes.
o With intensive management of diabetes this complication is delayed to
beyond childhood.
o Ophthalmologic examination should be conducted once the child is older
than 10 years and has had diabetes for 3-5 years. Annual follow-up is
suggested.
➢ Diabetic nephropathy
o This is defined by albuminuria in the urine and is preceded by
microalbuminuria.
o It causes significant morbidity and mortality.
o Annual screening of microalbuminuria is initiated once the child is 10
years or has had DM for 5 years.
o If screening shows an elevated ratio of spot urine microalbumin to
creatinine, 24hr urine microalbumin is estimated
o Patients with elevated mciroalbumin to creatinine ratio should receive
ACE inhibitors to delay the progression of nephropathy
➢ Peripheral neuropathy: This is unusual in children and adolescents. It results
in decreased nerve conduction velocity and sensory changes. An abnormality
in vibration perception may be the first finding.
• Macrovascular complications: usually seen in adulthood and include
atherosclerotic disease, hypertension, heart disease and stroke.
• Dyslipidemia:

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 ➢ Fasting lipid profile is performed on all pre-pubertal children >2 years of age
at the time of diagnosis (After glucose control is achieved) If there is a family
history of elevated cholesterol (>240mg/dl) and/or cardiovascular event
before age 55 years in the family.
➢ If there are no concerns of hyperlipidemia in the family, screening is
performed after onset of puberty (>12 years).
➢ If LDL is <100mg/dl a lipid profile is repeated every 5 year.
➢ For pubertal children (>12 years), a fasting lipid profile is performed at
diagnosis after glucose control is achieved.
➢ If LDL is <100mg/dl, lipid profile is checked in 5 years.
➢ If lipids are abnormal, annual monitoring is recommended in both age groups.
➢ Intervention is needed if fasting LDL is >100mg/dl once glucose control is
established.
➢ Initial therapy is nutritional modification with decrease in saturated fat in diet.
➢ A pharmacological agent is added for LDL >160mg/dl and in patients at risk
for cardiovascular disease and LDL values 130-159mg/dl after initiation of
dietary changes and lifestyle intervention.
➢ The goal of therapy is LDL value of <100mg/dl
• Celiac disease: evaluation of celiac disease involves testing for serum IgA,
antigliadin antibodies and transglutaminase antibodies. Further evaluation is
suggested if these antibodies are elevated.

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TYPE 2 DIABETES
MELLITUS
• This occurs in 2-3% of all children
with diabetes.
• In the last decade there has been a
10-fold increase in the incidence of
type 2 DM in children due to an
increase in obesity (change in
dietary habits and lifestyle-
increase in TV watching, time spent
playing video games rather than
outdoor play i.e. a sedentary
lifestyle).
• Etiology:
➢ Very strong hereditary Figure 29: Acanthosis nigricans
component (stronger for type 2
DIAGNOSIS
than type 1)
• Type 2 diabetes should be
➢ The cause is likely a
suspected if there is a family
combination of peripheral tissue
history, and in severely obese
resistance to insulin and
children with signs of insulin
progressive decline in insulin
resistance (Acanthosis nigricans)
secretion, both of which result in
a hyperglycemic state. • Sometimes autoantibodies are also
present in children with type 2DM
CLINICAL FEATURES
MANAGEMENT
• Clinical presentation is variable.
• Oral hypoglycemic agents may be
• Asymptomatic (50%) to mild
used if blood sugar levels are not
DKA.
very high.
• Serious DKA is uncommon
• Insulin therapy may be required for
because type 2 DM is more of an
those patients who have high blood
insulin resistance rather than a
sugar or who fail oral agents.
deficiency.
• Obesity (some children may have
weight loss)
• Acanthosis nigricans (velvety and
hyperpigmented skin of the neck
and axillary folds) is common.

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 Figure 30: Distinguishing between type 1 and 2 DM

DIABETIC KETOACIDOSIS
• This is hyperglycemia usually greater than 16 mmol/l (>300mg/dl) with
ketonuria and a serum bicarbonate level <15mmol/l or serum pH <7.30.
• Moderate DKA is characterized by:
➢ Serum pH <7.2 and bicarbonate <10 mEq/L
• Severe DK is characterized by:
➢ Serum pH<7.1 and bicarbonate <5 mEq/L
• It is a state of hyperglycemic dehydration and ketotic academia.
• It may be triggered by infection or poor compliance (common trigger).
• DKA can occur as the initial presentation of type 1 DM, 15-70% of newly
diagnosed children present in DKA.
• DKA at diagnosis is more common in children under 5 years of age.

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PATHOPHYSIOLOGY
• Insulin deficiency creates a state of diminished glucose substrate at the cellular
level, despite the high serum levels of glucose.
• The body’s need for substrate to make energy therefore results in
gluconeogenesis (synthesis of glucose from non-carbohydrate precursors) and
glycogenolysis (break down of glycogen to glucose).
• Hyperglycemia resulting from insulin deficiency leads to an osmotic diuresis
with polyuria and eventual dehydration which can lead to hypovolemia, severe
dehydration and shock.
• Dehydration also causes lactic acidosis which increases acidosis.
• Counter-regulatory stress hormones (i.e. glucagon, epinephrine, cortisol and
growth hormone) are released and contribute to fat breakdown (lipolysis) into
glycerol and free fatty acids.
• Glucagon stimulates conversion of free fatty acids into ketone bodies (acetone,
acetoacetate and beta-hydroxybutyric acid) through a process of beta-oxidation
which takes place in the liver.
• Ketosis and acidosis results in electrolyte imbalance and other most diagnostic
features of DKA including fruity odor and rapid respirations/Air hunger
(Kussmaul breathing)
• Acidosis causes shift of intracellular ions most commonly potassium and
phosphate to the ECF. These are lost in urine in excess amounts resulting in total
body potassium and phosphate depletion.
• Potassium levels may vary depending on the stage of DKA.
• Initially serum potassium levels are high and once treatment with insulin is
initiated the child becomes hypokalemic.
• Phosphate is a major component of 2,3 DPG and its depletion results in decrease
in 2,3 DPG and reduced oxygen delivery to the tissues.

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 Figure 31: Pathophysiology of DKA

CLINICAL FEATURES
• Features may develop in 24 hours while metabolic derangements take longer to
develop.
• Patients with mild DKA may present with vomiting, thirst, polyuria, polydipsia
and mild to moderate dehydration.
• Patients with severe DKA may present with severe dehydration (sunken eyes, dry
tongue, lethargy/comatose and poor skin turgor), hypotension, shock, severe
abdominal pain that may mimic appendicitis, rapid and deep (kussmaul)
respirations and coma.
• In the presence of ketones that gives the patient with DKA “fruity breath”.
Ketones also contribute to coma in severe DKA.
• Lethargy and cerebral depression may evolve into coma.
• Children may also have signs of infection including fever which precipitate DKA.
• Cerebral edema is a serious complication of DKA and is frequently seen in
children.

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 Figure 32: Features of DKA

• Suspect DKA if
➢ Dehydration
➢ Abdominal pain
➢ Ketone smell on breath
➢ Acidosis with acidotic breathing
➢ Unexplained coma
• Death due to DKA is due to hypokalemia or cerebral edema.
LABORATORY FINDINGS
• Investigations:
➢ Blood investigations:
o Serum Blood glucose (> 16 mmol/L)
o Serum ketone (>3.0 mmol/L): beta hydroxybutyrate is higher than
acetoacetate.
o Urea and electrolytes (dehydration)
▪ Increased anion gap (metabolic acidosis)
▪ Hyperkalaemia caused by metabolic acidosis (potassium moves out of
the cells in the face of acidosis as hydrogen is taken into the cells) or
normokalemia
▪ Serum sodium is low
o Serum Creatinine is elevated reflecting dehydration
o Blood culture (for evidence of precipitating cause) as well as FBC
o Liver function tests
o Malaria parasite slide
o Blood gases analysis (Severe metabolic acidosis)
o Hemoglobin A1C
o Serology:

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 ▪ Anti-islet cell antibodies (ICA) and Anti-insulin antibody (IAA) may
be present in 85-90% of newly diagnosed children and adolescents
with the autoimmune form of DM
o Thyroid function tests, thyroid antibodies and anti-gliadin, anti-
endomysial and tissue transglutaminase antibodies for coeliac disease
should be done to rule out concomitant thyroid and coeliac disease.
➢ Urine investigations:
o Urinalysis: glucose, ketones
o Urine microscopy, culture and sensitivity (to Rule out infections)
➢ Imaging:
o Cardiac monitor for T-wave changes of hypokalemia
▪ Flat T waves- hypokalemia
▪ Peaked T waves- hyperkalemia
o Chest X-ray (as indicated to rule out infections)

MANAGEMENT
• Principles:
➢ Confirm diagnosis
o History: Polydipsia, polyuria
o Clinical: Acidotic respiration, dehydration, drowsiness, abdominal
pain/vomiting
o Biochemical: high blood glucose on finger prick, ketones or glucose in
urine
o Perform investigations (as above)
➢ General resuscitation: ABCD
o A- secure Airway

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 o B- ensure patient is breathing and give 100% oxygen. An NG tube can be
inserted
o C- gain venous circulation with 2 large bore cannulae and send blood for
labs
o D-disability (neurological)
▪ Assess conscious level: Assess AVPU (Alert, responds to voice,
Responds to Pain, unresponsive)
▪ Institute hourly neurological observations. If less than alert on
admission or deterioration, record Glasgow coma score and transfer to
ICU. Consider cerebral edema management
▪ Cerebral edema-irritability, headache (late signs- slow pulse,
hypertension and papilloedema)
o E-exposure and environment (admit to ICU)
o F-Fluids- catheterize the child if unconscious or unable to void on demand
(e.g. in infants and very ill young children)
o G- glucose control
➢ Management of DKA
o Fluid and electrolyte replacement
o Insulin therapy
o Potassium replacement
o Antibiotics
➢ Monitor
o Input output chart
o Glucose hourly
o Daily weighing
o GCS or BCS
o ECG
FLUID AND ELECTROLYTE REPLACEMENT
• Assess clinically for dehydration.
➢ <5% (no dehydration): dry mucous membranes
➢ 5-10% (some dehydration): Dry mucous membranes, sunken eyes, reduced
skin turgor, restless and irritable
➢ >10% (Severe dehydration): Everything above plus shock (severely ill with
poor perfusion, capillary return >2sec, thready rapid pulse, reduced blood
pressure, rapid breathing, altered consciousness or coma)

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• Correct fluid over 48-72 hours. Rapid rehydration should be avoided as it may
lead to cerebral edema.
• Strict fluid balance essential (input output chart)
• Insert central venous line (CVP) and urinary catheter if shocked. A nasogastric
tube is passed for acute gastric dilation if there is vomiting or depressed
consciousness. Gastric contents should be emptied as there is gastric paralysis in
DKA.
• Do not stop the intravenous insulin infusion until 1h after subcutaneous insulin
has been given (reestablish oral fluids, subcutaneous insulin and diet)
• If in shock initial resuscitation is with normal saline.
➢ Begin with a bolus of 10-20ml/kg of NS over 1 hour. Repeat as necessary up
to 60ml/kg (max 4 boluses). if still in shock contemplate vasopressors after a
reevaluation to assess the cause.
• Fluid requirements= Deficit + Maintenance
➢ Deficit for moderate DKA uses 5-7% dehydration
➢ Deficit for severe DKA is calculated based on 10% dehydration (severe)
therefore 100ml/kg body weight is used
➢ Maintenance fluid:
o 100ml for first 10kg
o 50 ml for next 10kg
o 20ml for remaining kg
➢ Do not exceeding 4000ml/m2/day (normal saline)
➢ Calculate fluid requirement for 48 hours then divide it and give it over 6 hours.
➢ When glucose <12mmol/L change from normal saline to DNS to prevent
hypoglycemia. In the presence of hypernatremia use half normal saline (please
consult)
➢ Example: Suppose a child weighing 28kg presented in severe DKA, the fluid
calculation would be as follows
o Fluid requirement in 24 hours = deficit (10% dehydration) + maintenance
o Fluid requirement in 24 hours= (100 x 28) + (100 x 10) + (50 x 10) + (20
x 8)
o Fluid requirement in 24 hours=2800+ 1660
o Fluid requirement in 24 hours= 4460 ml
o Fluid requirement in 48 hours= 4460ml + 1660 (maintenance for 24 hours)
o Fluid requirement in 48 hours= 6, 120ml
o Therefore 3, 060ml should be given in each 24 hours (6120ml/ 2), and this
can further be given as 765 ml given 6 hourly (3060ml/4).

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• Ignore fluids used during resuscitation
• Monitor:
➢ Fluid input and output
➢ Electrolytes, creatinine and acid-base status regularly
➢ Neurological state
INSULIN THERAPY
• Continuous low dose IV is the preferred method. Do not give a bolus as this may
increase the risk of cerebral edema.
• Insulin infusion (0.05-0.1 IU/kg per h) is stated after 1-2 hours after fluid therapy,
titrating the dose according to the blood glucose. This is maintained until the
ketoacidosis is controlled.
• Make 1 unit per ml of human soluble insulin by adding 50 units (0.5 ml) insulin
to 50ml normal saline (dilute 50IU/0.5ml of soluble insulin in 50ml NS,
1unit=1ml). Attach using to Y-connector to IV fluids already running. Do not add
insulin directly to fluid bag. Solution should run at 0.1 units/kg/hr (0.1 ml/kg/h).
➢ If no syringe pump, give SC boluses of actrapid (soluble insulin) 6 hourly at
0.6 units/kg/dose (that is 0.1 units/kg/h) give half dose if the blood sugar falls
too fast. (Do not use this in patients with impaired peripheral circulation)
➢ Initial dose: SC: 0.3 units/kg, followed 1h later at SC 0.1 unit/kg every hour
or 0.15-0.20 units/kg every 2 hours.
• Monitor blood glucose regularly (1 hourly). Aim for gradual reduction of blood
glucose of about 2-5mmol/h, as a rapid reduction is dangerous.
• If rate of blood glucose fall exceeds 5mmol/L per hr reduce insulin infusion rate
to 0.05 units/kg/hr.
• Change to 5-10% dextrose/0.18% saline (half normal saline) or 5% glucose in
0.45% saline after 24h when the blood glucose has fallen to 12mmol/L to avoid
hypoglycemia or when it is greater than 5 mmol/L after insulin infusion.
• If blood glucose <7 mmol/L consider adding extra glucose to infusion.
• If blood glucose rises re-evaluate (consider sepsis or other condition) and
consider re-starting protocol. In the presence of severe infection when there is no
change in plasma glucose in 2-3 hr increase insulin infusion to 0.15U/kg/hr.
• Treatment can be switched to subcutaneous insulin once the patient is fully
conscious, out of ketoacidosis and able to tolerate oral feeding.
POTASSIUM
• Potassium repletion (20-40mmol/l of KCL to each 1 L of infused fluid)

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 ➢ Although the initial potassium may be high, it will fall following insulin and
rehydration.
➢ Potassium replacement must be instituted as soon as urine is passed and
potassium <5.5 mEq/L.
➢ If there is anuria, peaked T waves on ECG or serum potassium is >7mmol/L
do not start potassium
➢ Continuous cardiac monitoring and regular plasma potassium measurements
are indicated until the plasma potassium is stable.
ANTIBIOTICS
• Antibiotics: For proven infection.
• Consider them if sepsis is suspected (rising blood sugar)
BICARBONATE
• Rarely, if ever necessary. Only given if profoundly acidotic (pH <7.0) and
shocked with circulator failure to improve cardiac contractility
• Half-correct acidosis over 60 minutes
• Volume (ml 4.2 sodium bicarbonate) = 1/3 x body weight (kg) x base deficit
(mmol/L) = half-correction
• Base deficit= (Expected bicarbonate- actual bicarbonate)

• Note: Blood sugar, neurological status, vitals, fluid balance should be done
hourly and serum electrolytes, acid-base status should be done 2 hourly.

250
251
252
COMPLICATIONS OF DKA
• Include:
➢ Cerebral edema
➢ Pulmonary edema
➢ Aspiration pneumonia
➢ Severe hypokalemia, Arrhythmias
➢ Venous thrombosis
➢ Rhadomyolysis and acute pancreatitis
➢ Hypocalcemia due to either excessive use of potassium phosphate or osmotic
losses.

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CEREBRAL EDEMA
• Usually occurs 4-12 hours into therapy and rarely after 24 hours. It can also be
present at presentation before starting treatment.
• Risk factors include:
➢ Younger than 5 years of age,
➢ Severe metabolic acidosis and hypercapnia.
➢ An attenuated rise in serum sodium level during treatment, initial drops in
serum glucose levels faster than 5mmol/L per hour
➢ Fluid administration greater than 4L/m2 per 24hours.
➢ Elevated serum urea
➢ Administration of insulin in the first hour of fluid replacement
➢ Treatment of acidosis with bicarbonate
• Mortality as high as 70%
• Clinical features:
➢ Headache, Confusion, Irritability, Reduced conscious level, seizures
➢ Development of neurological signs (cranial nerve palsy, incontinence)
➢ Small pupils
➢ Vomiting
➢ Bradycardia
➢ Increasing blood pressure and slow pulse (these are late signs)
➢ Possibly respiratory impairment (desaturation in an otherwise improving
child)

254
 Figure 33: Diagnostic criteria for cerebral edema

• Management:
➢ Exclude hypoglycemia
➢ Initiate treatment as soon as condition is suspected
➢ Give mannitol 250-500 mg/kg (1.25-2.5 ml/kg) mannitol 20% over 15
minutes as soon as suspected. Repeat if there is no initial response in 30
minutes to 2 hours.
o Alternative: hypertonic saline (3%) 5-10 ml/kg over 30 minutes can be
given as an alternative to mannitol, especially if there is no initial response
to mannitol
➢ Restrict IV fluids to 2/3 maintenance
➢ Intubate and ventilate: keep PaCO2 to 3.5-5.0 kPa
➢ Keep sodium >135 mmol/L
➢ Keep head in midline and 30 degrees elevated
➢ Avoid fever (>38.0oC)
➢ Repeat mannitol every 4-6 hours to control ICP
➢ After treatment for cerebral edema has started. A cranial CT scan should be
done to rule out other possible intracerebral causes of neurologic
deterioration.

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NON-KETOTIC HYPEROSMOLAR STATE
• This is condition characterized by severe hyperglycemia (>30 mmol/L),
hyperosmolality (>350 mOsm/Kg), low plasma ketones (negative or positive at
<1:2 dilution) and dehydration.
• Although usually seen as a complication of Type 2 DM it can occur in Type 1
DM in children if insulin is present to prevent ketoacidosis but is insufficient to
control the blood sugar.
• The principles of treatment include judicious fluid replacement, regular insulin
and fluid therapy.

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 INFECTIOUS DISEASES

NEONATAL SEPSIS
• Neonatal sepsis is a type of neonatal infection and specifically refers to the
presence of a bacterial blood stream infection during the neonatal period (<28
days).
• It was previously referred to as “sepsis neonatorum”.
• Neonatal sepsis also denotes systemic bacterial infections incorporating
septicemia, pneumonia and meningitis.
➢ Septicemia: is a condition in which micro-organisms and their toxins are
present in blood, multiplying rapidly and destroying tissues.
➢ Sepsis: focus of infection + SIRS.
• The implicated organisms include:
➢ Group B streptococci
➢ Escherichia coli
➢ Listeria monocytogenes
➢ Klebsiella spp
➢ Coagulase negative staphylococci
➢ Staphylococcus aureus
➢ Acinetobacter
➢ Pseudomonas
• Neonatal sepsis is divided into 2 categories: Early-onset sepsis and Late-onset
sepsis.
• Early onset sepsis presents in the first 72 hours.
➢ Infections are caused by organisms prevalent in the maternal genital tract e.g.
GBS and E. coli or in the delivery area
➢ Predisposing factors include:
o Intrapartum maternal pyrexia (>38oC)
o Chorioamnionitis, maternal peripartum infection
o Maternal urinary tract infection
o Positive maternal high vaginal swab for GBS, previous pregnancy of baby
with GBS sepsis
o Multiple per vaginal examinations,
o Prolonged rupture of membranes (>18 hours) and aspiration of meconium

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 o Foul smelling liquor,
o Septic or traumatic delivery, fetal hypoxia
o Prematurity
o Low birth weight,
o Male gender
o Neutropenia due to other causes
o Infants with galactosemia (increased susceptibility to E. coli)
➢ Early onset sepsis manifests as pneumonia and less commonly as septicemia
or meningitis.
• Late-onset sepsis presents after 72 hours.
➢ The infections are caused by organisms thriving in the external environment
of the home or the hospital.
➢ Infection is often transmitted through the hands of the care-providers.
➢ Presentation is that of septicemia, pneumonia or meningitis.
➢ Predisposing factors include:
o Prematurity
o Low birth weight,
o Male gender
o Neutropenia due to other causes
o Overcrowded nursery
o Lack of breastfeeding,
o Poor cord care,
o Central lines, peripheral venous catheters, umbilical catheters, mechanical
ventilators
o Superficial infections (pyoderma, umbilical sepsis/omphalitis)
o Aspiration of feeds and disruption of skin integrity with needle pricks and
use of IV fluids.
o Association with indomethacin for closure of PDA, IV lipid administration
with coagulase-negative Staphylococcal bacteremia
• Neonatal sepsis is the single most important cause of death in hospital as well as
community in developing countries.
CLINICAL FEATURES
• Signs and symptoms are non-specific/vague and require high index of suspicion
for early diagnosis.
• Features include:
➢ Temperature instability: hypo or hyperthermia

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 ➢ Change in behavior: lethargy, irritability or change in tone (‘baby just doesn’t
seem right or doesn’t look well)
➢ Skin: poor perfusion, mottling, pallor, jaundice, petechiae
➢ Feeding problems: poor feeding, vomiting, diarrhea (uncommon), abdominal
distension
➢ Cardiovascular: tachycardia, hypotension
➢ Respiratory: apnea, tachypnea, cyanosis, respiratory distress
➢ Metabolic: hypo or hyperglycemia, metabolic acidosis
• An early but non-specific manifestation is alteration in the established feeding
behavior.
➢ The baby who had been active and sucking normally refuses to suck and
becomes lethargic, or unresponsive.
• A heart rate above 160 can also be an indicator of sepsis, this tachycardia can
present up to 24 hours before the onset of other signs.
• Fast breathing, chest retractions and grunt indicate pneumonia.
• Most cases of meningitis do not have any distinct clinical picture per se, making
it mandatory to suspect meningitis in all cases suspected of sepsis.
➢ The presence of excessive or high-pitched crying, fever, seizures, blank look,
neck retraction or bulging anterior fontanel are suggestive of meningitis.
• Shock, bleeding, and renal failure are indicators of overwhelming sepsis.
• Evaluate neonate (late onset sepsis) carefully for primary or secondary foci e.g.
meningitis, pneumonia, urinary tract infection, septic arthritis, osteomyelitis,
peritonitis, omphalitis or soft tissue infection.

Figure 34: Approach to neonate suspected of sepsis

259
DIAGNOSIS AND INVESTIGATIONS
• Diagnosis is difficult as a host of conditions such as hypothermia, hyperthermia,
hypoglycemia, hypoxia, late metabolic acidosis, congestive heart failure and
even simple conditions like nasal block may mimic sepsis.
• A careful clinical examination and relevant investigations are necessary to
differentiate these conditions from neonatal sepsis and avoid unnecessary use of
antibiotics.
• No investigation is required to start treatment in a sick baby who has high
probability of sepsis.
• Although culturing for microorganisms from a sample of CSF, blood or urine
may yield false negatives due to the low sensitivity of the culture methods and
concomitant treatment with antibiotics. It still remains the gold standard in the
definitive diagnosis of neonatal sepsis.
• The current practice in newborns less than 30 days is to perform a complete work-
up includes:
➢ Urine: Urinalysis and urine culture
➢ Blood
o Full blood count with differential,
o Blood culture: should be taken before starting antimicrobial therapy, after
cleaning skin (alcohol, povidone-iodine and again alcohol), a specimen of
0.5 to 1 ml of blood is taken in a small culture media bottle containing 5 to
10ml of the liquid broth
o CRP (perform serial 24 hours apart)
➢ CSF (Lumbar puncture)
o CSF culture, CSF studies
➢ Imaging
o CXR, AXR
➢ Other samples
o Culture of endotracheal tube aspirate (culture of the trachea do not predict
the causative organism in the blood of the neonate with clinical sepsis)
➢ Admit the newborn to the hospital and treat empirically for serious bacterial
infection for at least 48 hours until culture demonstrates no growth.
• A sepsis screen should be performed in equivocal (confusing) cases.
• The screen consists of:
➢ Leukocyte count (TLC <5000/mm3)
➢ Absolute neutrophil count (ANC <1800/ mm3)

260
 o Differential leukocyte count showing increased number of polymorphs
o Differential leukocyte count (band cells> 20%)
➢ Immature to total neutrophil ratio (I/T ratio, more than 20%)
➢ Increased haptoglobin
➢ Gastric aspirate showing >5 polymorphs per high power field
➢ Newborn CSF screen showing increased cells and proteins
➢ CRP (more than 1mg/dl)
➢ Micro ESR (15mm or more in the first hour)
➢ Suggestive history of chorioamnionitis, PROM (premature rupture of
membrane) etc.
• Sepsis screen is considered positive if 2 of these parameters are positive.
• Value of sepsis screen is more for exclusion of diagnosis of neonatal sepsis.
• Lumbar puncture should be performed in all cases suspected of neonatal sepsis
except in asymptomatic babies being investigated for maternal risk factors.
TREATMENT AND PREVENTION
• Good supportive care should be provided to the infant:
➢ Provide warmth, ensure normal temperature (36.5 to 37.5 degrees)
➢ Start oxygen by hood or mask, if the baby is cyanosed or grunting. Provide
bag and mask ventilation if breathing is inadequate. Instilling normal saline
drops in nostrils may help clear the nasal block.
➢ Assess peripheral perfusion by palpating peripheral pulses, capillary refill
time (normally <2-3 seconds) and skin color.
➢ Infuse normal saline or Ringer lactate 10ml/kg over 5-10 minutes, if perfusion
is poor. Repeat the same 1-2 times over the next 30-45 minutes, if perfusion
continues to be poor. Dopamine and dobutamine may be required to maintain
normal perfusion.
➢ Insert IV line. If hypoglycemia is suspected infuse glucose (10%) 2ml/kg stat.
Do not use glucose boluses routinely (to avoid rebound hypoglycemia).
➢ Provide a maintenance fluid and electrolytes and glucose (4-6 mg/kg/min) add
potassium to IV fluids once normal flow of urine has been documented.
➢ Enteral feeds should be initiated early if there is no abdominal distension and
baby is hemodynamically stable. Feed mother’s milk. Consider parenteral
nutrition if baby is not expected to receive enteral feeds for prolonged period.
➢ Administer vitamin K 1mg IM.
➢ Transfuse packed cells, if baby has low hematocrit (<35-45%). Do not use
blood/plasma transfusion on routing basis for ‘boosting’ immunity.

261
• Note that diagnosis of neonatal sepsis is difficult and so if there is any remote
suspicion of sepsis treat with antibiotics empirically until cultures are sufficiently
prove to be negative.
• In infants suspected with sepsis is beta-lactam antibiotic (usually ampicillin) in
combination with an aminoglycoside (usually gentamicin) or a third-generation
cephalosporin (usually cefotaxime… Ceftriaxone is generally avoided in
neonates due to the theoretical risk of kernicterus.)
• Organisms targeted are those found in the female GUT to which neonates are
especially vulnerable especially Group B streptococcus, Escherichia coli and
Listeria monocytogenes (the main rationale behind the use of ampicillin versus
other beta lactams).
• Neonates are also vulnerable to other common pathogens that cause meningitis
and bacteremia such as streptococcus pneumoniae and Neisseria meningitidis.
• Although uncommon, if anaerobic species are suspected (such as in cases where
necrotizing enterocolitis or intestinal perforation is a concern) Clindamycin is
often added.
• Antimicrobial therapy can be made specific once a positive culture and sensitivity
report is available.
• Early onset sepsis antibiotics:
➢ IV crystalline penicillin (benzyl penicillin)/ampicillin and Gentamicin
➢ Specific choice when specific organisms suspected/confirmed
➢ Change antibiotics according to culture and sensitivity results.
• Late onset sepsis antibiotics:
➢ Community acquired infection:
o Cloxacillin/ampicillin and gentamicin for non-CNS infection
o Crystalline penicillin and Cefotaxime for CNS infection
➢ Hospital acquired (nosocomial sepsis)
o Choice depends on prevalent organisms in the nursery and its sensitivity
o MRSA present may need vancomycin
o Anaerobic infection e.g. intraabdominal sepsis consider metronidazole
o Consider fungal sepsis if patient no responding to antibiotics especially if
preterm/very low birth weight or with indwelling long lines
• Duration of antibiotics:
➢ 7-10 days for pneumonia or proven neonatal sepsis
➢ 14 days for GBS meningitis
➢ At least 21 days for Gram negative meningitis

262
• Intensive care and monitoring is a key determinant of improved survival of
neonates.
• Outcome depends upon weight and maturity of the infant, type of etiologic agent,
its antibiotic sensitivity pattern and adequacy of specific and supportive therapy.
➢ Early onset sepsis carries a higher risk of adverse outcomes.

263
COMPLICATIONS
• CNS
➢ Seizures
➢ SIADH
• Cardiovascular
➢ Hypotension and shock
• Respiratory: respiratory failure

264
• Hematological: DIC
• Metabolic:
➢ Hypoglycemia
➢ Hyperglycemia
➢ Electrolyte imbalance
➢ Acid-base disorder
DIFFERENTIAL DIAGNOSIS
• Meconium aspiration syndrome
• Necrotizing enterocolitis
• Pericarditis (bacterial)
• Pulmonary hypoplasia
• Respiratory distress syndrome
• Bowel obstruction in the newborn
• Congenital diaphragmatic hernia
• Congenital pneumonia
• Heart failure (congestive)
• Hemolytic disease of newborn

EARLY ONSET GROUP B STREPTOCOCCAL (GBS)

INFECTION
• Infection in newborns occurring withing the first week of life are designated early
onset disease. Late onset infections occur in infants aged >1 week, with most
infections evident during the first 3 months of life.
• The information below is for early onset disease only
• Maternal prophylaxis:
➢ Prevention is provided by intrapartum antibiotic prophylaxis given to mother
at the onset of labor. The intrapartum antibiotic prophylaxis is given at least 4
hours before delivery reduces the risk of neonatal infection by 90% but may
be less effective in preterm labor.
➢ Drug: Pen G/ampicillin/cefazolin
➢ Indications:
o Previous infant with invasive GBS disease
o GBS bacteriuria during any trimester of the current pregnancy
o Positive GBS vaginal-rectal screening culture in late gestation (35-37
weeks) during current pregnancy

265
 o Delivery at <37-week gestation when the mother has been in labor
o Amniotic membrane rupture >18 hours
o Intrapartum temperature >38oC
• Neonatal prophylaxis
➢ Indication: intrapartum antibiotic prophylaxis for mother is partially (given
<4 hours prior to delivery) or not given
➢ Drug: Benzyl penicillin (Pen G) and gentamicin
➢ Stop after 36-48 hours if blood culture negative and clinically normal
• Neonatal treatment:
➢ Indications: signs of maternal or neonatal sepsis
➢ Drug: Benzyl penicillin IV (Pen G)
➢ Meningitis: 100 000 U/kg/dose 8 hourly 2-3 weeks
➢ CSF normal: 100 000 U/kg/dose 12 hourly for 10 days
➢ Add gentamicin if GBS not the only risk factor

266
267
 TUBERCULOSIS
TUBERCULOSIS
• This is a chronic disease caused by
Mycobacterium tuberculosis.
• Other species of Mycobacterium
include: M. bovis, M. avium
complex and M. leprae.
• All cases of pulmonary tuberculosis
and most cases of extrapulmonary
disease are caused by human type
strain M. tuberculosis.
• A few cases of extrapulmonary
illness particularly the tubercular
lymphadenitis may be due to the
bovine strain.
• The bacterium has the following
characteristics:
➢ Pleomorphic
➢ Weakly gram-positive
➢ Non motile and non-sporing
➢ Resistant to acid discoloration
after staining due to the
presence of mycolic acid in the
cell wall.
➢ Slow growing (generation time
is 12-24 hours and culture
require at least 3-6 weeks).
• The infection is spread by the
tuberculous patient who discharges
tubercle bacilli in his sputum or
nasopharyngeal secretion during
bouts of coughing or sneezing etc.
This is the usual mode of infection.
268
• Transmission is usually from the so
called “sputum positive” adults,
children are rarely contagious as
they rarely expectorate infected
sputum.
• In the pediatric group few
infections may also occur by
transplacental route (congenital
tuberculosis)
• Other possible route of infection is
through gastrointestinal system if
the bovine strain M. bovis is
ingested.
• Exposure is the term used to
describe an individual who has
been in recent contact with an
individual with contagious
pulmonary TB.
➢ Physical examination,
tuberculin skin test and chest
radiograph are all normal.
• Latent tuberculosis infection is the
term used to describe an
asymptomatic individual with
positive tuberculin skin test, normal
physical examination and a chest
radiograph that either is negative or
shows only pulmonary granulomas
or calcifications with or without
regional lymph nodes.
• Those at high risk include:
immigrants from highly endemic
regions of the world, health care
personnel, homeless individuals,
residents of institutions or
 correctional facilities (prison) and
individuals with immunodeficiency
conditions (e.g. HIV, chronic
disease, immunosuppressive
medications).
PATHOGENESIS
• Infection is by respiratory droplets.
• The damage caused to the lungs is
due to the delayed hypersensitivity
reaction (Type IV)-T-cell mediated
• Once inhaled into the lung, alveolar
macrophages ingest the bacteria.
• The bacilli then proliferate inside
the macrophages and cause the
release of neutrophil chromo-
attractants and cytokines resulting
in an inflammatory cell infiltrate
reaching the lung and draining hilar
lymph nodes.
• Macrophages present the antigen to
the T lymphocytes with the
development of a cellular immune
response.
• A delated hypersensitivity type
reaction occurs resulting in tissue
necrosis (caseous necrosis) and
formation of the granuloma.
• The granuloma consists of
➢ A central area of necrotic
material called caseation
➢ Surrounding epithelioid cells
➢ Langhans’ giant cells with
multiple nuclei
➢ Lymphocytes
➢ Varying degrees of fibrosis
269
• Note: epithelioid cells and
Langhans’ giant cells are both cell
being derived from the
macrophage.
• In primary tuberculosis the
caseated area may heal completely
and may become calcified.
• Some of these calcified nodules
contain bacteria which are
contained by the immune system
and are capable of lying dormant
for years. This initial focus is
termed the ‘Ghon focus’
➢ On X-ray the Ghon focus is
evident as a small calcified
nodule often within the upper
parts of the lower lobes or the
lower parts of the upper lobes,
seen in the mid zone.
➢ When there is associated hilar
lymphadenopathy the lesion is
termed a ‘Ghon complex’
(Primary complex of Ranke)
• In children especially those with
risks e.g. immunocompromised or
other co-morbidity or <2 years
primary infection often progresses
to an active disease.
• If the immune system contains the
infection and the patient develops
cell-mediated immune memory the
to the bacteria this is termed latent
tuberculosis.
• Later in life after initial exposure
TB, the latent infection can reactive
and cause active disease when
immunity decreases.
• Sometimes primary infection can
end in hematogenous spread
causing miliary tuberculosis.
• The TB bacilli seed various organs
through blood borne or lymphatic
spread. If the number of bacilli are
more and the host immunity
inadequate disseminated
occurs.
CLINICAL FEATURES
• Can manifest as:
➢ Pulmonary TB
➢ Extrapulmonary TB
➢ Mixed
• In children approximately 25-30%
cases are of extrapulmonary TB.
PULMONARY TUBERCULOSIS
• The symptomatology of pulmonary
TB is almost uniform across
various types of involvement in the
lungs.
• Children tend to have more non-
specific symptoms:
➢ Cough (inconsistent and may be
absent in advanced disease)
➢ Low grade fever
➢ Loss of appetite
➢ Lethargy
➢ Weight loss
➢ Night sweats
➢ Failure to thrive (commonest
presentations)
• The additional clinical signs of the
disease depend upon the type and
extent of the involvement.
• There may be no clinical signs or
range from findings of pleural
effusion, consolidation and
bronchopneumonia.
• In miliary type of pulmonary TB
there may be high grade fever, toxic
look and splenomegaly.
TB ➢ The disease is most common in
infants and young children.
➢ The onset of illness is often
sudden.
EXTRAPULMONARY
TUBERCULOSIS
• TB can affect any of the body
system and different organs.
• They include:
➢ CNS
o TB-meningitis
o TB-encephalitis
o TB-spine
➢ TB-abdomen (ileitis)
➢ TB of the skin, joints and
skeletal disease (Pott’s disease)
➢ Disseminated or miliary disease
• In children CNS TB is a frequent
occurrence.
CENTRAL NERVOUS SYSTEM
DISEASE
• This is the most serious
complication of TB in children.
• It arises from the formation of a
caseous lesion in the cerebral
cortex or meninges that results
from occult lymphohematogenous
spread.
270
• These lesions can be single or
multiple and enlarge presenting as
intracranial tumors.
• TB may also be confined to the
spinal cord.
• TB meningitis is commonest in
children between 6 months to 5
years of age.
• In infants and young children, the
disease runs a rapid course, it may
present with:
➢ Rapid progression to
hydrocephalus, bulging anterior
fontanelle
➢ Lethargy
➢ Anorexia, headache, vomiting
➢ Impaired level of consciousness
➢ Seizures
➢ Raised intracranial pressure
➢ Irritability
➢ High pitched cry, drowsiness,
coma
• In older children signs and
symptoms progress over the course
of several weeks beginning with
fever headache, irritability and
drowsiness.
• The disease advances with
symptoms of lethargy, vomiting,
nuchal rigidity, seizures,
hypertonia and focal signs.
• The final stage of disease is marked
by coma, hypertension, decerebrate
and decorticate posturing and
death.
271
• The diagnosis should be considered
for any patient with basilar
meningitis, hydrocephalus or
cranial nerve involvement that has
no other apparent cause.
• A lumbar puncture is needed to
demonstrate AFB in CSF.
• CSF findings are as follows:
➢ Color: clear or opaque
➢ Opening pressure: increased
➢ WBC= 50 – 500 cells/mm3
(initially polymorphonuclear
neutrophils then lymphocytes
predominate)
➢ Proteins: very high
➢ Glucose: low to very low
➢ Culture: AFB smear and culture
rarely positive, PCR may be
positive
TUBERCULOUS PERITONITIS
• Uncommon in children but can
occur due to either hematogenous
spread or local extension from
abdominal lymph nodes or
intestines.
• Presents with: low grade fever,
pain, ascites, weight loss.
TB ABDOMEN
• There are 2 major clinical
presentations:
➢ Tuberculous peritonitis
➢ Tuberculous enteritis
• Tuberculous peritonitis is
uncommon in children but can
occur due to either hematogenous
 spread or local extensions from
abdominal lymph nodes or
intestines.
➢ Low grade fever and pain,
ascites, weight loss are the
typical features.
• Tuberculous infection of the
intestines occur either secondary to
hematogeous spread or from
ingestion of TB bacilli from
sputum. Small intestines and
appendix are the usual sites of the
involvement.
• Tuberculous ulcers or later
strictures can cause the clinical
features:
➢ Low grade fever, weight loss,
diarrhea or constipation or
features of subacute intestinal
obstruction can be the
presenting features.
GENITOURINARY TB
• Renal TB has a long incubation
period and hence is generally seen
in older children or adolescents.
• Kidneys as well as other parts of the
urinary system can be involved.
• Renal involvement is unilateral and
initially present as sterile pyuria
and microscopic hematuria.
• Later abdominal pain and mass,
dysuria and flank hematuria with
progression to hydronephrosis or
urethral strictures may be seen.
272
TB OF BONES/JOINTS
• Skeletal TB is a late complication.
• The commonest involvement is
vertebra leading to classical Pott’s
spine with formation of gibbus and
kyphosis.
• Dactylitis of metacarpals is seen in
children.
• Tuberculous arthritis of any of the
joints can occur.
TB ADENITIS
• Cervical lymphadenitis (Scrofula),
is the most common form of
extrapulmonary TB in children.
• The nodes draining the lungs fields
are usually involved.
• The common group of nodes
involved are cervical and axillary
but other groups can also be
involved especially secondary to
drainage form an infected organ.
• The node involvement usually
occurs 6-9 months after primary
infection.
• The affected nodes are firm, non-
tender, fixed and often matted due
to periadenitis.
• There may be accompanying low
grade fever. In case a node breaks
down it leads to sinus formation.
• On biopsy they show caseating
granuolmas
DISSEMINATED/ MILIARY TB
• Two forms of disseminated spread
are seen:
➢ Hematogenous spread: there is
usally fever,
hepatospenomegaly and
lymphadenopathy
➢ In the other more serious
disseminated form there is large
hematogenous spread and in a
patient with inadequate
response there is miliary TB.
o Miliary TB may start with an
insidious fever, malaise and
loss of appetite
o The fever rises and there is
lymphadenopathy with
hepatosplenomegaly.
CONGENITAL TB
• Perinatal transmission of TB can
occur as a hematogenous spread
through the placenta in mother with
active disease.
• Inhalation/ingestion of infected
amniotic fluid or exposure after
birth to a positive contact can also
cause infection in a neonate.
• The transplacental infection
presents with a primary complex
like manifestation in the abdomen.
• The liver has the focus with nodes
in porta hepatis also involved.
• If the infection is through
inhalation or the hematogenous
spread occurs further to lungs then
respiratory signs are predominantly
present.
273
• The neonate can have acute onset
respiratory distress, fever, poor
weight gain, lymphadenopathy and
hepatosplenomegaly.
• Occasionally meningitis can also
occur.
• The clinical signs and symptoms
can be similar to other infections.
• A maternal history or contact with
an AFB positive person should
raise the suspicion.
DIAGNOSIS
• Diagnosis of TB in children is not
simple.
• A high index of suspicion in the
endemic area is important.
• The modalities most frequently
used for diagnosis are
➢ Mantoux test
➢ Radiological examination
➢ Gastric aspirate/sputum AFB
smear and culture
➢ Polymerase chain reaction
(PCR)
• Diagnosis in children is usually
based on clinical signs and
symptoms, an individual’s risk
factor for infection (chronic
disease, contact), tuberculin skin
test findings, chest radiographic
findings and culture.
➢ A contact is defined as any child
who lives in a household with an
adult taking ATT or has taken
such therapy in past 2 years.
• Demonstration of mycobacterium
in various clinical specimens
remains gold standard.
CULTURE, MICRSCOPE AND
SENSITIVITY
• Sputum samples are generally
unobtainable from children under
about 8 years, unless specialist
induction techniques are used.
➢ For example, following an
overnight fast, the patient
receives salbutamol by
nebulized followed by
hypertonic (3% or 5%)
inhalation by nebulizer.
➢ Older children may provide
expectoration at end of
procedure. In young children
including infants a effusion, or cavitary disease
nasopharyngeal aspirate is
collected and processed like
sputum for smear and culture.
• Children usually swallow sputum
so gastric washing (lavage) on 3
consecutive mornings are required
to visualize or culture acid-fast
bacilli (AFM) originating from the
lungs.
➢ To obtain these, a nasogastric
tube is passed and secretions are
rinsed out of the stomach with
saline before food.
➢ Microscopic stain used is
modified Ziehl-neelson stain
(ZN stain)
274
• Urine, lymph node excision
(showing caseating granulomas),
CSF should be performed where
appropriate.
• Although it is difficult to culture
TB from children the presence of
multidrug resistance strains makes
it important to try to grow the
organism so that antibiotic
sensitivity can be assessed.
RADIOLOGICAL
• Findings include:
➢ Hilar or mediastinal
lymphadenopathy
➢ Ghon complex- small
parenchymal infiltrate with
enlarged hilar lymph nodes
➢ Lobar involvement, pleural
which typically affects the upper
lung segments.
➢ Miliary picture
POLYMERASE CHAIN
REACTION
• PCR is used to detect DNA from
clinical samples that are negative
by microscopy.
• It has high sensitivity and
specificity when compared with
traditional microbiological
methods.
• It is suitable in diagnosis of TB in
children especially when diagnosis
is difficult or needed urgently.
• The possibility of false positive
results must be considered
especially when the clinical
symptoms and history of exposure
of the child make the diagnosis
improbable.
• GenXpert has been developed for
identification of M. tuberculosis in
sputum of adult patients.
➢ This method gives results within
2 hours, in addition it provides
results about sensitivity to
rifampicin.
➢ The test is under evaluation for
diagnosis of TB in children.
MANTOUX TEST
• If TB is suspected a mantoux test is
performed.
• 2 units of purified protein
derivative (PPD) of tuberculin (2
TU SSI, 0.1ml intradermal
injection read after 48-72 hours as
induration not erythema in mm
across the forearm).
• Because PPD is a mixture of
proteins some of which are
common to TB and BCG, the test
may be positive because of past
vaccination rather than TB
infection.
• A history of BCG immunization
therefore needs to be taken into
account when interpreting the test.
• Patients who are
immunocompromised,
malnourished or received live
275
vaccines may have false negative
reactions.
• The test is positive when:
➢ Induration >5mm in children
with close contact who has
clinical or radiological findings
consistent with TB disease or
immunocompromise.
➢ > 10mm if the child is younger
than 4 years, has a chronic
medical condition, lives in an
area endemic for TB or has not
received BCG
➢ >15 mm where BCG has been
given, there are no other risk
factors or in children older than
4 years
• Heaf tests are no longer used for
screening TB.
• A new generation of diagnostic
tests is the interferon-gamma
release assays (IGRA)
➢ These are blood tests that
assess the response of T cells to
stimulation in vitro with a small
number of antigen found in TB
but not in BCG
➢ Positive results therefore
indicate TB infection rather
than BCG vaccination.
➢ Sensitivity and specificity of
these tests in different settings
is being evaluated but its
routine use in clinical practice
is increasing.
➢ Co-infection with HIV makes
the diagnosis even more
 difficult because with advances
immune suppression both skin
tests and IGRA are unreactive.
• The IGRA or Mantoux test cannot
distinguish between TB infection
and TB disease so correlation with
clinical signs and symptoms is
required.
• Various scoring systems have been
developed for diagnosis of TB. In
these scoring systems more weight
is given to Lab tests i.e.
demonstration of AFB, tubercules
on histology, suggestive radiology
and tuberculin test >10mm
induration. These scoring system
may be used as screening tools but
not for starting treatment.
TREATMENT OF
TUBERCULOSIS
• The principles of therapy in
children with TB are similar to
those of adults.
• Treatment for a duration of 6
months has become the standard
practice.
• Extrapulmonary TB
(Osteoarticular TB, neurological
TB) and disseminated TB are
treated for longer durations of 12
months or more.
• Patients with latent tuberculosis are
treated with isoniazid for 9 months.
Older adolescents, pregnant
adolescents, and adults are also
given daily pyridoxine (Vitamin
276
B6) to prevent neurologic
complications of isoniazide
treatment. (peripheral neuropathy)
• Patients with TB disease are treated
on the basis of the location of the
TB disease and the susceptibility
pattern of the organism.
• Treatment includes
➢ 2 months (intensive phase) of
Rifampicin, isoniazide,
pyrazinamide and ethambutol
(now given to children
according to newer guidelines
despite its side effect of optic
neuritis)
➢ 4 months (continuation phase)
of rifampicin and isoniazide
• Close follow-up is needed and
frequently children are put on
Direct Observed therapy (DOTS)
where nursing staff observe the
therapy being given 3 times a week.
➢ During the intensive phase the
health worker or trained person
watches as the patient swallows
the drugs in his presence
➢ During the continuation phase
the patient is issued medicines
for one week in a multi-blister
combi-pack of which the first
dose is swallowed by the
patient in the presence of the
health worker or trained
person.
➢ The consumption of medicines
in the continuation phase is also
checked by return of the empty
 multi-blister combi-pack when o Ciprofloxacin
the patient comes to collect o Levofloxacin
medicine for the next week. o Movifloxacin
• Side effects of the drugs: o Gatifloxacin
➢ Rifampicin (bactericidal): ➢ Injectables
Discoloration of body fluids (to o Amikacin
orange-pink), hepatotoxicity o Kanamycin
➢ Isoniazid (bactericidal): o Capreomycin
peripheral neuropathy (give o Streptomycin
vitamin B6), hepatotoxicity
COMPLICATIONS OF
➢ Streptomycin (bactericidal):
ototoxicity and nephrotoxicity TUBERCULOSIS
➢ Pyrizinamide (bacteriostatic): • Pulmonary complications:
hepatotoxicity, hyperuricemia ➢ Pleurisy
(gout) ➢ Pleural effusion
➢ Ethambutol (bacteriostatic): ➢ Empyema
Optic neuritis and color ➢ Pneumothorax
blindness ➢ Aspergillosis
• Drug resistant TB accounts for ➢ Endobronchitis
possibly only 2% of the million ➢ Bronchiectasis
cases of TB in the world. ➢ Laryngitis
• Drug resistant TB denotes ➢ Cor pulmonale
resistance to one of the first line TB ➢ Ca bronchus
drugs (being Rifampicin or ➢ Miliary TB
isoniazid)
• Multidrug resistant (MDR) TB
denotes resistance to at least 2 main
first line TB drugs i.e. rifampicin
and isoniazide.
• Extreme drug resistant (XDR) TB
denotes MDR-TB + resistance to
any fluoroquinolone and at least
one of the 3 injectable second-line
drugs (amikacin, kanamycin or
capreomycin)
• The second line drugs include:
➢ Oral: fluoroquinolones

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 MALARIA

MALARIA
• Human malaria is a protozoal infection caused by one of five species of the
genus Plasmodium: P. falciparum, P. vivax, P. ovale, P. malariae, and P.
knowlesi.
• Majority of cases seen in Zambia are due to P. falciparum.
• Malaria is transmitted by the bite of a female anopheles mosquitoes.
➢ The parasite develops in the vector’s body and makes it infectious if the
mosquito is susceptible, feeds on human blood and lives for at least 10-12
days after an infective blood meal.
• Malaria can also be transmitted in contaminated blood transfusions, or
transplacentally from mother to the fetus (congenital malaria).
➢ P. falciparum and P. vivax are more commonly seen in sub-Saharan Africa.
➢ P. ovale is rare and seen mainly in Africa.
➢ P. malariae is the rarest.
• Patients with sickle cell trait, thalassemia and glucose-6-phosphate
dehydrogenase deficiency are relatively immune to malaria.
• Homozygotes of sickle cell disease are not protected from malaria but
heterozygotes are immune.
LIFE CYCLE
• The female mosquito becomes infected after taking a blood meal containing
gametocytes, the sexual form of the malarial parasite.
• The sexual developmental cycle in the mid-gut of the mosquito usually takes 7-
20 days (Depending on temperature, from zygote, to ookinete to oocyst
containing sporozoites), culminating in infective sporozoites migrating to the
insect’s salivary glands.
• The sporozoites are inoculated into a new human host and those which are not
destroyed by the immune response are rapidly taken up by the liver (hepatic
stage/exoerythrocytic schizogony).

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• In the liver they multiply inside hepatocytes as merozoites; this is pre-
erythrocytic (or hepatic) sporogeny.
➢ In the hepatocytes, each parasite replicates to form 2,000 to 15,000
merozoites in the case of P. vivax infection and 40,000 merozoites for P.
falciparum.
• The hepatic phase is asymptomatic and constitutes the incubation period lasting
about 10 days.
• After a few days, the infected hepatocytes rupture, releasing merozoites into the
blood from where they are rapidly taken up by erythrocytes (Erythrocytic
schizogony).
➢ In the case of P. vivax and P. ovale, a few parasites remain dormant in the
liver as hypnozoites. These may reactivate at any time subsequently
causing relapsing infection (malaria relapse).
➢ P. vivax and P. ovale may last for 2-3 years and P. malariae for 10-20
years.
• Inside the red cells, some parasites again multiply changing from merozoite to
early (Ring form) and late trophozoite to schizont and finally appearing as 8-24
new merozoites.
• The erythrocyte ruptures releasing the merozoites to infect further cells.
➢ Each cycle takes about 48hours in P. falciparum, P. vivax and P. ovale and
about 72hours in P. malariae.
➢ P. vivax and P. ovale mainly attack reticulocytes and young erythrocytes,
while P. malariae tends to attack older cells.
➢ P. falciparum will parasitize any stage of erythrocyte.
➢ Repeated cycles of erythrocyte invasion and rupture leads to chills, fever,
headache, fatigue and non-specific symptoms and with severe malaria,
signs of organ dysfunction.
➢ Manifestations of severe malaria, including cerebral malaria, non-
cardiogenic pulmonary edema and renal failure are caused by high
concentrations of P. falciparum infected erythrocytes in the
microvasculature.
➢ Since mature P. falciparum organisms in the erythrocytes adhere to
endothelial cells, only ring forms circulate (except in severe infections)
and levels of peripheral parasitemia may be low despite substantial
infection.

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• A few merozoites do not develop into trophozoites but into gametocytes
(microgametes and macrogametes). These are not released from the red cells
until taken up by a feeding mosquito to complete the life cycle.

• In the midgut of the mosquito the gametocytes fuse to form a zygote which
develops into an ookinete and then an oocyst which ruptures to give sporozoites
which migrate to the salivary glands awaiting to be injected into a person in
which it bites for a blood meal.

CLINICAL FEATURES
• The incubation period of malaria varies between 9 and 30 days, the least for P.
falciparum and longest for P. malariae.

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• The onset of the disease is marked by a sudden rise of fever which may be
periodic.
➢ Erythrocyte ruptures releasing the merozoites to infect further cells.
➢ Each cycle takes about 48 hours in P. falciparum, P. vivax and P. ovale and
about 72 hours in P. malariae.
➢ P. vivax and P. ovale mainly attack reticulocytes and young erythrocytes,
while P. malariae tends to attack older cells.
➢ P. falciparum will parasitize any stage of erythrocyte.
• The initial findings include vague flu-like symptoms that typically include
headache, malaise, lassitude (lack of energy), anorexia and pain in the limbs.
• The fever may be continuous or remittent for several days before it becomes
classically intermittent. The classical intermittent fever is not usually seen in
children.
• Cyclic fevers follow the flu-like prodrome and occur 48-72 hours in correlation
with RBC rupture and parasitemia.
• Rigors, chills, sweating, headache, abdominal pain, nausea and vomiting may
also accompany the fever.
• The illness is characterized by:
➢ Cold stage: chills and rigors with headache, nausea, malaise and anorexia.
➢ Hot stage: dry flushed skin, rapid respiration and marked thirst
➢ Sweating stage: temperature falls by crises
• Other features include hemolytic anemia, splenomegaly, jaundice and
hypoglycemia. Cerebral malaria, renal failure, shock and respiratory failure all
may occur.
• The gastrointestinal and respiratory symptoms are seen more often in young
children and infants.
• After a few days of fever, the patient begins to appear pale and may even have
jaundice.
• In adults there is a definite periodicity of fever, which is generally not seen in
children.
• The clinical presentation in some children may be different with low-grade
fever, hepatosplenomegaly, anemia and thrombocytopenia.
• Congenital malaria is considered in a neonate whose mother was symptomatic
during later pregnancy. The neonate may or may not have any fever along with
poor, feeding, lethargy and vomiting.

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• Unexplained anemia and severe jaundice (indirect hyper-bilirubinaemia) are
additional features.

FEATURES OF SEVERE MALARIA/COMPLICATED MALARIA

(1) Cerebral malaria
➢ Multiple convulsions- more than 2 episodes in 24 hours not attributed to any
other cause aside malaria.
➢ Impaired consciousness or unrousable coma not attributed to any other cause
aside malaria.
(2) Prostration i.e. generalized weakness so that the patient is unable to walk or sit
up without assistance
(3) Acute non-cardiogenic pulmonary edema (radiological) and acute respiratory
distress (acidotic breathing), deep breathing
(4) Shock (‘Algid malaria’)
➢ Algid malaria is a severe infection with P. falciparum. There is hypotension,
shock, shallow respiration and pallor with a rapid fatality. Gram negative
sepsis is often associated.
➢ Circulatory collapse or shock, systolic blood pressure <70mmHg in adults
and <50mmHg in children
(5) Severe normocytic anemia Hb<5g/dl, Packed cell volume <15%
(6) Hyperpyrexia
(7) Hypoglycemia- <2.2 mmol/L (<40 mg/dl), Failure to feed
(8) Metabolic acidosis- blood pH< 7.25, bicarbonate <15mEq/L, Deep breathing,
respiratory distress (acidotic breathing)
(9) Acute renal failure (serum creatinine >265 micromol/l), blackwater fever
(Hemoglobinuria)
➢ Black water fever is a severe form of falciparum malaria with hemolysis,
hemoglobinuria and severe anemia.
(10) Abnormal spontaneous bleeding and coagulopathy (DIC)
(11) Hyperparasitemia: >2% or 100 000/microliter in low intensity transmission
areas or >5% or 250 000/microliter in areas of high stable malaria transmission
intensity.
(12) Clinical jaundice plus evidence of other vital organ dysfunction

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DIAGNOSIS
• Rapid diagnostic test: malarial antigens (PfHRP2/PMA/pLDH) from asexual
and/or sexual forms of the parasite as color changes on antibody coated line on
the strips. Although sensitivity and specificity are a problem.
➢ RDT is an immunochromatographic test that detects specific parasite
antigens in blood mainly:
o Histidine Rich protein 2 (HRP 2)
o Plasmodium lactate dehydrogenase (pLDH)
➢ Some RDT detect only one species (P. falciparum) while other detect
multiple (P. vivax, P. malariae and P. ovale) that is why it is possible to be
RDT negative and Malarial parasite slide (MPS) positive.
• Peripheral blood smear (Giemsa Stain): thin and thick smear. This is the gold
standard for diagnosis of malaria.
➢ Thick smear: diagnosis can be made quickly,
used for screening. Thick smear has a
sensitivity of detecting 5-10
parasites/microlite.
➢ Thin smear: identifies the species and
staging (determination of the level of
parasitemia). Thin smear has a lower
sensitivity of 200 parasites/microliter of
blood.
➢ Microscopy also provides information about
the parasite load (number of infected
RBC/total RBC), prognosis (mature
schizonts and pigmented neutrophils
indicating a poor prognosis) and tracks Figure 35: Gametocyte of Plasmodium falciparum
response to therapy.
o >2% or 100 000/microliter in low intensity transmission areas or >5% or
250 000/microliter in areas of high stable malaria transmission intensity
➢ Note: sometimes peripheral smears may be negative due to partial
antimalarial treatment or sequestration of parasitized cells in deep vascular
beds. Repeating smears every 6-8 hours at least 3 times is recommended if
the clinical suspicion for malaria is high and the initial smear is negative.
• PCR: Highly sensitive and specific for detecting all species of malaria even in
low level parasitemia. This is not available commercially and so its use is
limited.

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• Quantitative buffy coat (QBC) test: this is a newer method for identifying the
malarial parasite in the peripheral blood. It involves staining of the centrifuged
and compressed red cell layer with acridine orange and its examination under
UV light source. It is fast, easy and claimed to be more sensitive than the
traditional thick smear. Disadvantage is that it is expensive and needs special
equipment. Additionally, false positives may be present due to staining artifacts
and inability to speciate the parasite.
• Other investigations for management and assessment of severity include:
➢ Full blood count
➢ PT, PTT
➢ Blood glucose
➢ Urea, creatinine and electrolytes (bicarbonate, chloride)
➢ Serum lactate
➢ Arterial blood gasses: pH
➢ Chest X-ray for respiratory distress syndrome
➢ Serum bilirubin
➢ Serum transaminases
➢ Lumbar puncture: for a patient presenting with acute febrile encephalopathy.
DIFFERENTIAL DIAGNOSIS
• Common clinical differentials of malarial include:
➢ Typhoid fever
➢ Leptospirsosis
➢ Dengue
➢ Viral hepatitis
• Cerebral malaria should be differentiated from other causes of acute febrile
encephalopathy like meningitis and encephalitis. Patients with algid malaria
(those in shock) mimic meningococcemia and gram-negative shock
COMPLICATIONS
• Cerebral malaria:
➢ Pathogenesis can be explained by cytoadherence. P. falciparum infected
RBCs with matures stages of the parasite bear Histidine rich protein II which
adheres to Intracellular adhesion molecule 1 (ICAM-1)
➢ Note: cytoadherence also causes stickiness of RBCS, “sludging”, “stasis” of
blood, and “blockage” cause lesions, local anoxia, ischemia thus pain e.g.
abdominal pain with occlusion of the small sized vasculature.

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• Cardiovascular and hematology:
➢ Severe anemia: hemolytic, normochromic normocytic
➢ High output cardiac failure secondary to anemia
➢ Disseminated intravascular coagulation (DIC)
➢ Thrombocytopenia with bleeding tendencies
➢ Shock
• Respiratory:
➢ Acute respiratory distress syndrome and respiratory failure
➢ Pulmonary edema
• Gastrointestinal:
➢ Jaundice
➢ Splenomegaly (Tropical splenomegaly syndrome) and Splenic rupture
(which can also lead to shock)
➢ Hepatomegaly
➢ Ulceration and ischemia of GIT (due to occlusion of blood vessels)
• Genitourinary
➢ Renal failure: due to hemoglobinuria and tubular damage.
➢ Black water fever: hemoglobinuria
➢ Uremia
➢ Nephrotic syndrome (antibody-antigen complexes cause lesions)
• Metabolic:
➢ Hypoglycemia (<2.2 mmol/l in children)
➢ Metabolic acidosis (blood pH< 7.25)
TREATMENT
• Choice of antimalarial therapy is based on resistance patterns, species type and
severity of illness.
• Establish lab diagnosis before empirical therapy. This is to prevent irrational
therapy and consequent drug resistance and also to avoid missing other causes
of febrile illness.
UNCOMPLICATED MALARIA
• Treat with a first-line anti-malarial agent, as in the national guidelines.
• Uncomplicated P. falciparum malaria:
➢ Treat for 3 days with the recommended Artemisin-based combination
therapy

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 ➢ Artemether 20mg-lumefantrine 120mg is given with food (preferably
containing fatty as foods) as follows: stat, after 8 hours, then 12 hourly on
day 2 and 3.
• Artemether-lumefantrine (Coartem) (20mg artemether and 120mg lumefantrine)
➢ Child weighing 5-14kg (2months-3 years): one tablet twice a day for 3 days
➢ Child weighing 15-24 kg (3-8 years): 2 tablets twice a day for 3 days
➢ Child 25-35kg (9-12 years): 3 tablets twice a day for 3 days
➢ Child >35kg (>12 years): 4 tables twice a day for 4 days
COMPLICATED MALARIA
• Artesunate 2.4 mg/kg IV or IM on admission 0h, then at 12 h and 24h, then daily
until the child can take oral medication (2mg/kg IV artesunate) but for a
minimum of 24h even if the child can tolerate oral medication earlier.
• Once patient regains consciousness, discontinue parenteral therapy and
commence full course of Artemether-Lumefantrine. There should be an interval
of at least 8 hours between last dose of artesunate and first dose of artemether
lumefantrine.
• Second line: Quinine IV
➢ Loading dose 20mg/kg body weight (maximum 1200mg) diluted in 10ml/kg
(or equivalent volume with Quinine) in 5% or 10% dextrose over 4 hours
then after 8 hours give maintenance dose 10mg/kg in 10ml/kg of 10%
dextrose over 4 hours
➢ Treatment of hypoglycemia with 5ml/kg 10% dextrose IV infusion
➢ Maintenance fluids/feeds and supportive oxygen by mask
➢ If weak pulse and CRT>3sec, give 20ml/kg normal saline until pulse is
restored (use blood for resuscitation if Hb<5g/dl at 20ml/kg whole blood)
➢ Treat convulsions with phenobarbitone
➢ Once patient is able to swallow, oral quinine at 10mg/kg body weight every
8 hours to complete a 7-day course of treatment.
• Supportive therapy:
➢ Admit to intensive care if available
➢ Rapid clinical assessment with respect to level of consciousness, blood
pressure, pallor, rate and depth of respiration, hydration status. Fundus
should be examined and patient weighed.
➢ Relevant investigations are sent

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➢ Parenteral antimalarial therapy should be started empirically awaiting
confirmation of diagnosis. Doses should be calculated as mg/kg body
weight.
➢ If blood glucose cannot be determined or if hypoglycemia documented,
glucose should be given.
➢ Good nursing care especially if patient is unconscious
➢ Oxygen therapy and other respiratory support if required
➢ Appropriate fluid therapy to avoid under/overhydration. One of the most
important points is not to ascribe deep/rapid breathing in a child with severe
malaria and anemia to congestive heart failure. In most of these children it
has been demonstrated that rapid breathing is due to metabolic acidosis and
respond well to fluid expansion/blood transfusion and not diuretic therapy.
➢ Monitor pulse, blood pressure, respiratory rate, core body temperature,
pallor, level of consciousness at least 4 hourly to detect complications early.
➢ Monitor urine output by indwelling catheter and watch for hemoglobinuria.
➢ Central venous pressure and arterial blood pressure if present should be
monitored.
➢ Monitor blood glucose in children with coma every hour, then 2-4 hourly or
if clinical deterioration occurs.
➢ Blood counts, electrolytes and creatinine should be monitored on a regular
basis. Monitor parasite count 6 hourly for first 48 hours.
➢ Broad spectrum antibiotics (3rd generation cephalosporins and
aminoglycoside, beta lactam-beta lactamase inhibitor combinations) should
be given in:
o Any child with localized signs suggesting bacterial infection
o Any child in shock
o Any child with severe respiratory distress in whom deep breathing
does not rapidly improve with correction of dehydration or anemia
➢ Avoid harmful treatments such as corticosteroids and osmotic diuretics

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Complication Management
Hyperpyrexia Paracetamol to induce defervescence
Avoid aspiring and NSAIDs due to risk of gastrointestinal bleeding
Cerebral malaria • Nurse in lateral position
• 2 hourly turning
• Continuous NG aspiration, maintain airway, irrigate and patch eyes
• If convulsions occur exclude hypoglycemia, hyperthermia, manage seizures
with slow IV push of diazepam (0.3mg/kg IV or 0.5mg/kg rectal), if seizure
recurs load with phenytoin 15-20mg/kg
• Consider CT scan for intracranial bleed, cerebral edema or herniation if
consciousness deteriorate or new neurological abnormalities appear
• For raised ICP nurse with head propped up and in midline position, control
seizures, hypoglycemia, hyperventilate the patient. Use of mannitol is
controversial and corticosteroids do not have a role.
Anemia Transfuse packed cells if hemoglobin <5g/dl or in presence of impaired
consciousness, hyperparasitemia, respiratory distress of metabolic acidosis, use
diuretics (Furosemide 1mg/kg stat) and slow transfusion if fluid overload present,
perform exchange transfusion in patients with overt congestive cardiac failure
Hypoglycemia 5ml/kg of 10% glucose given slowly IV followed by infusion of 10% dextrose,
frequent monitoring of blood sugar. May use octreotide and glucagon if IV fluids
restricted.
Metabolic Correct hypovolemia, hypoglycemia and anemia. Administer oxygen and sodium
acidosis bicarbonate slowly if pH <7
DIC Vitamin K, fresh frozen plasma and/or cryoprecipitate, exchange transfusion in
presence of fluid overload.
Renal failure Careful fluid challenge with 20ml/kg of normal saline, followed by furosemide
1mg/kg (max 5mg/kg) if no response noted, restrict fluid intake, dialysis indicated
in presence of refractory hyperkalemia or metabolic acidosis, fluid overload and
rapid rise of serum creatinine.
Hemolysis Give Artemisin derivative antimalarials, transfusion with pack cells to maintain
hematocrit, monitor urine output and renal function for need of dialytic support

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Acute respiratory Occurs due to pulmonary capillary leak and less commonly fluid overload.
distress Administer oxygen, nurse in propped up position, restrict fluid intake, loop
syndrome diuretics may help, severe cases need mechanical ventilation with high peak and
expiratory pressure
Shock Send blood culture and administer broad spectrum antibiotics e.g. cefotaxime and
amikacin/gentamicin, monitor central venous pressure, replace fluids, administer
vasopressors and respiratory support as required
Hyperparasitemia Consider exchange transfusion
• Note:
➢ An optimal response to therapy is defined as a count which on day 1 is less
than day 0, a count on day 3 which is <25% of count on day 0, no parasites
in peripheral blood 72 hours after starting therapy and up to 28 days and no
fever after 72 hours.
➢ Patients with parasitologically confirmed malaria who continue to have
fever 72 hours after starting antimalarials are occasionally encountered in
clinical practice. If they are smear negative causes could be IV
thrombophlebitis, secondary bacterial infections, coinfections such as
typhoid or rarely immune phenomena. If the smear is positive then this is
early treatment failure caused by either wrong choice of drug, substandard
drug, wrong dose, poor compliance or drug resistance.
➢ If drug resistance is suspected treatment should be changed to alternative
Artemisin based combination or quinine.
➢ Reappearance of asexual parasites within 28 days of treatment is defined as
recrudescence/late treatment failure due to either wrong choice of drug,
wrong dose, poor compliance or drug resistance. Recrudescence is fairly
common if Artemisin monotherapy is used. Treatment of recrudescence
includes optimizing drug therapy or change to an alternative regime.
PREVENTION
• Avoid mosquito bites by:
➢ Application of repellants
➢ Sleeping under treat mosquito nets
➢ Wearing protective clothing
• Control of the Anopheles mosquito especial in endemic areas:
➢ Clear grass and water paddle around the house
➢ Spray areas around the house and the house
• Chemoprophylaxis:

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 ➢ Pyrimethamine Sulfadoxine administered twice during pregnancy reduces
the prevalence of maternal anemia and low birth weight.
➢ For travelers from non-endemic areas to endemic areas drugs commonly
used are doxycycline, atovaquone-proguanil (Expensive but safest), and
mefloquine
➢ Prophylaxis should be started at least 1-2 weeks before departure and
continued for 4 weeks after return (except atovaquone-proguanil where it
can be started on the day of departure).

HUMAN IMMUNODEFICIENCY VIRUS

• HIV is an Enveloped Positive sense RNA virus of genus lentivirus belonging to
the family Retroviridae.
• There are 2 types of HIV
➢ HIV type 1: cause of pediatric acquired immunodeficiency syndrome
(AIDS)
➢ HIV type 2: rare cause of infection in children
• More than 1 million children have AIDs and as many as 10 times this number
are infected with HIV worldwide.
• Structure:
➢ Enveloped with projecting knob like spikes on the surface (gp 120) and the
anchoring transmembrane (gp 41)
o GP120 protein contains the binding site for the CD4 molecule, the
most common T-lymphocyte surface receptor for HIV.
o Most HIV strains have a specific tropism for one of the chemokines:
the fusion-inducing molecule, CXCR-4 which has been shown to act
as a co-receptor for HIV attachment to lymphocytes and CCR-5, a
beta chemokine receptor that facilitates HIV entry into macrophages.
o Following viral attachment, gp 120 and the CD4 molecule undergo
conformational changes, allowing gp41 to interact with the fusion
receptor on the cell surface.
o Viral fusion with the cell membrane allows entry of viral RNA into
the cell cytoplasm for replication of the virus.
➢ Genome consists of 2 identical copies of the positive sense single stranded
RNA genome (retroviruses are diploid). It has 3 major sections:

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 o Gag region: encodes viral core
proteins (p24, p17, p9 and p6
which are derived from the
precursor p55)
o Pol region: encodes the viral
enzymes (Reverse transcriptase
p51, Integrase p32, and proteas
p10 and endonuclease)
o Env region: encode the viral
envelope proteins (gp120 and gp41)

• Transmission:
➢ Perinatal transmission (mother-to-child transmission- MTCT) currently
accounts for >95% of pediatric HIV cases.
o Transmission rates range from 50% because of the common use of
antiretroviral therapy during pregnant.
o Vertical transmission occurs in utero, intrapartum or postpartum
(through breastfeeding).
o Up to 30% of infected newborns are infected in utero.
o The highest percentage of HIV-infected children acquire the virus
intrapartum.
o Breastfeeding is an important route of transmission, especially in the
developing countries.
o Risk factors for vertical transmission include:
o Fetal factors:
▪ Preterm delivery (<34 weeks gestation)-Prematurity
▪ Birthweight <2500g
o Maternal factors:
▪ Primary maternal HIV infection
▪ Advanced maternal HIV disease
▪ High maternal viral load
▪ Low maternal antenatal CD4 count
▪ Use of illicit drugs during pregnancy
▪ >4 hours duration of ruptured membranes, PROM
▪ Concomitant maternal genital infections including
chorioamnionitis
o Factors reducing vertical transmission:

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 o Undetectable maternal viral load
o Cesarean section
o Adherence to maternal ARV therapy and infant post-exposure
prophylaxis
➢ Other modes of transmission:
o Sexual contact: important mode of infection in adolescents
o Blood product transmission which is now rare because of mandatory
blood product screening
o Sharing of intravenous and tattoo needles
PATHOGENESIS
• The first cells to be infected through mucosal entry of HIV are the dendritic cells.
• These cells transport the virus to the lymphatic tissue where it selectively
invades the CD4 lymphocytes, monocytes and macrophages.
• HIV attaches via gp120 to the CD4 surface receptor and undergoes a
conformational change and expresses 2 more co-receptors:
➢ On macrophages, monocytes, microglial, dendritic cells and Langerhans
cells Chemokine co-receptor 5 (CCR5) is expressed.
➢ T-helper cells express CXCR-4
• Once gp120 and co-receptors bind, gp 41 causes fusion of the HIV with the host
(gp 41 is a fusion molecule)
• After infecting CD4 cells there is progressive viral replication followed by a
viremia phase 3-6 weeks after infection.
➢ RNA is reverse transcribed into circular DNA (in the presence of reverse
transcriptase) that is incorporated into the host genome (in the presence of
integrase) forming a provirus that can remain dormant for extended periods.
➢ Because the HIV-1 reverse transcriptase is error-prone, many mutations
arise creating wide genetic variation in HIV-1 isolates even within an
individual patient.
➢ HIV-1 transcription is followed by translation. A capsid polyprotein is
cleaved to produce, among other products, the virus specific proteas (p10).
This enzyme is critical for HIV-1 assembly. The RNA genome is then
incorporated into the newly formed viral capsid. As the new virus is formed,
it buds through the cell membrane and is released.
➢ HIV-2 is a rare cause of infection in children. It is prevalent in Western and
Southern Africa. If HIV-2 is suspected a specific test that detects antibody
to HIV-2 peptides should be used.

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• Subsequently there is a decline in the viremia due to the normal immune
response of the body.
• CD8 cells are helpful in containing the initial infection.
• A variable period of clinical latency follows but during this phase viral
multiplication continues. The cytokines play an important role in sustaining viral
load during this phase.
• There is also B-cell activation leading to an increase antibody production and
resultant hypergammaglobulinaemia.
NATURAL HISTORY
• Before the introduction antiretroviral therapy 3 distinct patterns of disease were
described:
➢ Rapid progressors: approximately 10-20% of HIV infected newborns in
developed countries presented with a rapid disease course, with onset of
AIDS and symptoms during the first few months of life and if untreated
death from AIDS-related complications by 4 years of age. In developing
countries >85% of HIV infected newborns may have such a rapidly
progressing disease.
o If intrauterine infection coincides with the period of rapid expansion
of CD4 cells in the fetus, it could effectively infect the majority of the
body’s immunocompetent cells.
o Most children in this group have a positive HIV-1 culture and/or
detectable virus in the plasma in the first 48 hours of life. This early
evidence of viral presence suggests that the newborn was infected in
utero. In contrast to the viral load in adults, the viral load in infants
stays high for at least the first 2 years of life.
➢ Slow progressors: majority of perinatally infected newborns (60-80%) have
a slower progression of the disease with a median survival time of 6 years.
Many patients have a negative viral culture or PCR in the 1 st week of life
and therefore considered to be infected intrapartum.
o In typical patients, the viral load rapidly increases by 2-3 months of
age (median 100,000 copies/ml) and then slowly declines over a
period of 24 months.
o This observation can be explained partially by the immaturity of the
immune system in newborns and infants.
➢ Long-term survivors: occurs in a small percentage (<5%) of perinatally
infected children who have minimal or no progression of disease with

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 relatively normal CD4 counts and very low viral loads for longer than 8
years.
• HIV infected children have changes in the immune system similar to those seen
in HIV infected adults.
➢ CD4 cell depletion may be less dramatic because infants normally have
relative lymphocytosis e.g. a value of 1500 CD4 cells/mm3 in children <1
year of age is indicative of severe CD4 depletion as opposed to <200 CD4
cells/mm3 in adults.
➢ Lymphopenia is relatively rare in perinatally infected children and is usually
seen in older children or those with end-stage disease.
➢ B-cell activation occurs in most children in the infection as evidenced by
hypergammaglobulinemia associated with high levels of anti-HV-1
antibody. This response may reflect both dysregulation of T-cell suppression
of B-cell antibody synthesis and active CD4 enhancement of B-lymphocyte
humoral responses.
• CD4 depletion and inadequate antibody responses lead to increase susceptibility
to various infections and various clinical manifestations
CLINICAL FEATURES
• Most infants with perinatally acquired HIV infection are asymptomatic for the
first year of life.
• Some HIV infected infants progress rapidly to symptomatic disease and onset of
AIDS in the first year of life.
• Subtle and nonspecific presentations such as Parotitis, lymphadenopathy,
hepatosplenomegaly, thrombocytopenia, failure to thrive, chronic or recurrent
diarrhea, Recurrent infections such as otitis media, interstitial pneumonia and
sinusitis, or oral thrush may persistently be present.
• Some children may present with loss of developmental milestones and severe
varicella infection or zoster
• Systemic and pulmonary findings are common in the US and Europe while
chronic diarrhea, wasting and severe malnutrition are common in Africa.
• Symptoms found more commonly in children than adults are: recurrent bacterial
infections, chronic parotid swelling, lymphocytic interstitial pneumonitis and
early onset of progressive neurologic deterioration.
• Clinical presentation varies with the degree of immunosuppression.
➢ Mild suppression: lymphadenopathy or parotitis

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 ➢ Moderate: recurrent bacterial infections, candidiasis, chronic diarrhea and
lymphocytic interstitial pneumonitis (LIP)- caused by a response to HIV
infection or related to EBV infection.
➢ Severe AIDS diagnoses include opportunistic infections e.g. pneumocystis
jiroveci (carinii) pneumonia PCP, severe failure to thrive, encephalopathy
and malignancy, although this is rare in children.
• More than one clinical feature is often present.
• An unusual constellation of symptoms, especially if infectious should, alert one
of HIV infection.
• NOTE: children with persistent lymphadenopathy, hepatosplenomegaly,
recurrent fever, parotid swelling, thrombocytopenia, or any suggestion of SPUR
(Serious, Persistent, Unusual, Recurrent) infections should be tested for HIV.
HIV STAGING
• Staging is by:
➢ Clinical status (WHO staging)
➢ Degree of immunologic impairment
WHO CLINICAL STAGING OF HIV/AIDS IN CHILDREN WITH
CONFIRMED INFECTION
CLINICAL STAGE FINDINGS
CLINICAL STAGE 1 • Asymptomatic
• Persistent generalized lymphadenopathy
CLINICAL STAGE 2 ➢ Unexplained persistent hepatosplenomegaly
➢ Pruritic papular eruptions
➢ Skin and Oral Mucocutaneous manifestations
➢ Angular cheilitis
➢ Recurrent oral ulceration
➢ Lineal gingival erytherma
➢ Extensive wart virus infection
➢ Extensive molluscum contagiosum
➢ Herpes zoster
• Fungal nail infections
• Unexplained persistent parotid enlargement
• Recurrent or chronic upper respiratory tract infections (otitis
media, otorrhea, sinusitis, tonsillitis)
CLINICAL STAGE 3 • Unexplained moderate malnutrition or wasting not
adequately responding to standard therapy

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 • Unexplained persistent diarrhea (14 days or more)
• Unexplained persistent fever (above 37.5oC intermittent or
constant, for longer than one month)
• Persistent oral candidiasis (after the first 6-8 weeks of life)
• Oral hairy leukoplakia
• Acute necrotizing ulcerative gingivitis/periodontitis
• Lymph node TB
• Pulmonary TB
• Severe recurrent bacterial pneumonia
• Symptomatic lymphoid interstitial pneumonitis
• Chronic HIV-associated lung disease including
bronchiectasis
• Unexplained anemia (<8g/dl), neutropenia (<0.5 x 109/L) or
chronic thrombocytopenia (<50 x 109/L)
CLINICAL STAGE 4 • Unexplained severe wasting, stunting or severe malnutrition
not responding to standard therapy
• Pneumocystis jiroverci pneumonia
• Recurrent severe bacterial infections (e.g. empyema,
pyomyositis, bone or joint infection, meningitis but
excluding pneumonia)
• Chronic herpes simplex infection (orolabial or cutaneous of
more than 1 month’s duration or visceral at any site)
• Esophageal, tracheal, bronchial or lung candidiasis
• Extrapulmonary/disseminated TB
• Extrapulmonary cryptococcosis (including meningitis)
• HIV encephalopathy
• Disseminated endemic mycosis (extrapulmonary
histoplasmosis, coccidioidomycosis)
• Disseminated non-tuberculosis mycobacterial infection
• Chronic cryptosporidiosis (with diarrhea)
• Chronic isosporiasis
• Cerebral or B-cell non-Hodgkin lymphoma
• Progressive multifocal leukoencephalopathy
• Symptomatic HIV-associated nephropathy or HIV
associated cardiomyopathy

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DEGREE OF IMMUNOLOGIC IMPAIRMENT
HIV-ASSOCIATED AGE-RELATED CD4 CELL VALUES
IMMUNODEFICIENCY
<1 month 12-35 months 36-59 months >5 years
Not significant (normal) >35% >20% 25% >500
cells/mm3
Mild 30-35% 25-30% 20-25% 350-499
cells/mm3
Advanced 25-30% 20-25% 15-20% 200-349
cells/mm3
Severe <25% or <20% or <15% or <200
cells/mm3 or
<1500 <750 <350 <15%
3 3 3
cells/mm cells/mm cells/mm

DIAGNOSIS
• In children over 18 months old, HIV infection is diagnosed by detecting
antibodies to the virus.
➢ Children less than 18 months of age who are born to infected mothers will
have transplacental maternal IgG HIV antibodies and at this age, a positive
test confirms HIV exposure but not HIV infection.
➢ Children born to HIV mothers have maternal IgG HIV antibodies which may
persist for as long as 18-24 months.
➢ Demonstration of IgG antibody to HIV by a repeatedly reactive enzyme
immunoassay (EIA) and confirmatory test (Western blot or
immunofluorescence assay) can establish the diagnosis of HIV infection.
• The most sensitive test in diagnosing HIV before 18 months of age is HIV DNA
PCR.

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 ➢ All infants born to HIV infected mothers should be tested for HIV infection,
whether or not they are symptomatic.
➢ Speficic viral diagnostic assays such as HIV DNA or RNA PCR, HIV
culture or HIV p24 immune dissociated p24 (ICD-p24) are essential for
diagnosis of young infants born to HIV infected mothers.
➢ HIV specific DNA PCR is performed at birth and monthly until 4 months of
age to detect infants who are infected perinatally.
➢ By 6 months of age the HIV culture and/or PCR identifies all infected infants
who are not having any continued exposure due to breast feeding.
➢ HIV DNA PCR is preferred in developed countries. Plasma HIV RNA
assays are more sensitive than DNA PCR for early diagnosis however data
to support this is limited.
➢ HIV culture has similar sensitivity to HIV DNA PCR however it is more
technically complex and expensive and results are available in 2-4 weeks as
compared to 2-3 days with PCR.
➢ The p24 antigen assay is less sensitive than other virologic tests.
➢ 2 negative HIV DNA PCRs within the first 3 months of life at least 2 weeks
after completion of postnatal antiretroviral therapy indicate the infant is not
infected although this is confirmed by loss of maternal HIV antibodies from
the infant’s circulation after 18 months of age.
➢ Negative HIV specific DNA PCR at 4 months is consistent with an infant
who has not been infected. If the DNA PCR is negative for HIV, infants are
followed until they lose their transplacentally acquired maternal antibody
(by age 18-24 months).
➢ Early infant diagnosis now uses HIV DNA PCR test on dried blood spot
(DBS) samples, the positive tests need confirmation using a HIV DNA PCR
on a whole blood sample.
• When testing children note:
➢ Testing history: all known HIV positive children should not be tested for
HIV again
➢ HIV negative children with a documented result with no known HIV risk
should not have an HIV test done
➢ All children who have never had HIV test before and have risk for HIV
acquisition must have an HIV test. Risk for HIV in children include:
o Children whose biological mother is HIV positive
o HIV stats unknown both parents are deceased
o History of sexual assault or exposure to HIV infected body fluids

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 ➢ An age appropriate HIV test must be done for children
➢ A clinician can override the HIV screening tool based on clinical
presentation of the patient.
EARLY INFANT DIAGNOSIS
• For children <24 months who are breast-feeding, the mother should be tested
first. If she is HIV positive perform a nuclei acid test (NAT) which can be done
on the HIV exposed infant regardless of age.
• NAT can be performed on either a Dried blood spot (DBS) which is sent to the
laboratory or fresh blood sample using a point-of-care machine (POC).
• Infants who have HIV detectable by NAT at birth are likely to have been infected
in-utero. These infants will progress to disease rapidly and in the absence of
treatment will experience high mortality in the first few months of life.
• Infants infected at or around delivery may not have the virus detectable by NAT
for several days to weeks.
• The ability of NAT to detect the virus in the blood may be affected by ARV
drugs taken by the mother or infant for postnatal prophylaxis, resulting in false-
negative results. This includes drugs present in breast milk as a result of maternal
ART during breastfeeding.
• The rationale behind this recommendation is that infants who are first identified
as HIV-exposed postpartum have a high cumulative risk of already having
acquired HIV by the time prophylaxis is initiated, thus NAT should be
performed around the time of initiating prophylaxis which would be at birth.
This will help to minimize the risk of development of resistance because of
extended prophylaxis in infected infants and help to promote linkage to timely
initiation of ART.
• Note:
➢ All mothers of breastfeeding children <24 months old should be tested every
3 months. If she tests HIV positive, a NAT should be performed on the HIV
exposed infant. A Positive NAT is confirmed with a confirmatory NAT test
to rule out false positives.
➢ ART is started immediately without waiting for second test result
➢ Where NAT is negative in a never breastfed HIV exposed infant, a repeat
test should be done at 6 weeks
➢ Where NAT is negative and HIV exposed infant is still breastfeeding, NAT
retest should be done at 6 weeks, 6 months and 9 months then do serological
at 12 months, 18 and 24 months.

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• Note:
➢ Where there is no Point of care NAT a DBS should be sent for HIV DNA
PCR. Where NAT is positive, a repeat test should be done to rule out false-
positive results.
➢ ART should be initiated without waiting for the receipt of the second test
result because of the high risk of mortality with in-utero infection. If the
second test is negative, a third NAT should be performed before interrupting
ART.
➢ Although plasma remains the gold standard sample for NAT.
➢ DBS will be the preferred mode of sample transportation for both DNA and
RNA testing.

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 ➢ HIV testing for those <24 months, NAT is gold standard. Although plasma
remains the gold standard sample for NAT, DBS will be the preferred mode
of sample transportation for both DNA and RNA testing.
MANAGEMENT
• Principles
➢ Antiretroviral therapy
➢ Prophylaxis
➢ Treatment of opportunistic infections and common infections
➢ Adequate nutrition and immunization
REDUCTION OF VERTICAL TRANSMISSION
• Use of maternal antenatal, perinatal and postnatal antiretroviral drugs to achieve
an undetectable maternal viral load at the time of delivery.
• Avoidance of breast-feeding
➢ Avoidance of breast-feeding is not safe in many parts of the world, where
use of formula-feeding increases the risk of gastroenteritis and malnutrition.
It may be safer for babies in this environment to breastfeed and antiretroviral
drugs may be given to the breast-feeding baby or mother to reduce the
ongoing risk of MTCT.
• Active management of labor and delivery to avoid prolonged rupture of
membranes or unnecessary instrumentation.
• Pre-labor caesarean section if the mother’s viral load is detectable close to the
time of delivery.
COMPLICATIONS

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CHAPTER 15: NEONATOLOGY

NEONATOLOGY

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NICU ORIENTATION

NICU ORIENTATION
HOW TO CONDUCT/PREPARE FOR A WARD ROUND
• Remove all jewelry, bracelets/rings (except wedding ring) and white lab coats.
(Keep your valuables with you)
• Roll up sleeves
• Begin each day with a “3 minute” hand to elbow wash.
• Look at previous notes in patient’s files.
• Look at all flow charts in notes (Feeds, antibiotics, IV fluids etc.)
• Record last weight, noting change in grams from previous weight.
• Record vital signs: Temperature range (axillary), heart rate, Capillary refill time.
• Medications: please list names, number of days for antibiotics, frequency and
doses.
• Lab: record all results (electrolytes, bilirubin, FBC etc.)
• Follow up all cultures, X-rays, U/S, CT.
• Examine baby- Wash your hands before and after touching a baby.
• Clean with disinfectant your stethoscope, tape measure, thermometer, phone and
ultrasound probe before and after use.

ADMISSION CRITERIA TO NICU

• Weight less than 1.8kg
• In respiratory distress
• Temperature greater than 38OC
• Hypoglycemia unresponsive to feeds
• Macrosomic baby above 4kg
• All infants of diabetic mothers
• Infants with meconium aspiration
• Infants with congenital anomalies
• Asphyxiated babies
• Babies with convulsions
• Babies with jaundice
• Persistent vomiting
• All infants with a septic risk (PROM >18 hours, maternal UTI etc.)
• Hypothermia (Temperature <36oC) unresponsive to warming by radiant warmer
or kangaroo mother care
• Newborn/infant in unstable condition
• Prematurity at less than 36 weeks.
• Persistent pulmonary hypertension of the newborn
• Sepsis or suspected sepsis
• Hemolytic disease of the newborn
• Hematologic disorders
• Congenital infections
• Any other newborn/infant requiring further evaluation as determined by the
neonatology resident or the attending consultant.

DISCHARGE CRITERIA FROM NICU

• Low birth weight neonate or any infant with one of the above diagnoses whose
condition has stabilized as determined by attending physician.
• Must be at least 24 hours old on the day of discharge or as ordered by the
physician.

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• Must demonstrate the ability of maintaining an adequate nutrition as evidenced
by sufficient caloric fluid intakes as determined by the physician.
• Must have adequate urinary output as determined by the physician and should
have passed meconium.
• Must maintain appropriate body temperature outside of artificial environments.
• Must meet physical assessment criteria deemed by the physician as “stable”
and/or not requiring further hospitalization.
• Infants with a weight of <1.7kg will be seen weekly until a target weight of 1.7kg
is reached when they will then be seen in outpatient clinic as necessary.
• Must be without episode of bradycardia as required by the discharging physician
or can be monitored appropriately at home.
• Any infant not meeting the above discharge criteria may be discharged with prior
approval of the attending physician.
• Before discharge ensure:
➢ Baby receives intramuscular vitamin K 1mg.
➢ Baby is discharged without any clinical problems
➢ Breastfeeding technique of the mother is correct and that parents understand
how to care for the umbilical stump
➢ Discharge summary form is filled in
➢ The mother collects the birth record (mothers whose babies were born alive
but died before discharge should receive both the birth record and death
certificate on discharge
• Tell the parents to visit the nearest local clinic when the baby is 3 days old and
then at 7 days old (ensure that parents understand follow-up plans, they have all
the dates and necessary referral letters).
• For neonates discharged within 6 hours:
➢ Advise parent to seek medical attention if there is a delay in passing urine
or meconium beyond 24 hours.
➢ Give BCG and OPV 0 if available at the hospital. If not, advise the parents
to take the baby to the nearest local clinic within 13 days (also applies for
babies born premature).
OTHER IMPORTANT CONSIDERATIONS
• Mothers:
➢ Must wash hands and breasts before and after breastfeeding or expressing
milk
➢ Must wash hands before and after handling baby

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 ➢ Promote exclusive breastfeeding/expressed breast milk to protect baby
against infection
➢ Should have unrestricted visits
• Staff
➢ Minimal handling of babies to avoid unnecessary cross-transfer of infection
➢ Consider teaching mothers to feed as this is effective in reducing cross
infection
• Public
➢ Restricted entry for public- father, grandparent and family are allowed
controlled scheduled visits
➢ Adults who are infectious are restricted from entering nursery e.g. flu, snotty
nose, body rashes, infectious hepatitis
➢ Restricted entry for people coming from areas of infectious disease
outbreak.
• IV access lines
➢ Use umbilical venous catheter (UVC) only where absolutely necessary and
peripheral IV drip not possible.
➢ Remove UVC within 7 days or as soon as no longer needed
➢ IV drips must be removed as early as possible once not needed
➢ Close monitoring of drip site for signs of infection
➢ Central venous pressure (CVP) lines to be removed within 7-10 days of
insertion, monitor closely for signs of infection
➢ Use aseptic technique when doing invasive procedures and inserting IV
lines.
PREMAJOR ROUND PREPARATION
• The resident doctor should make certain that the problem list is complete and
formulate a preliminary plan of care.
• All files should be well summarized.

HOW TO PRESENT BABIES DURING MAJOR ROUND

• Sample problem list:
➢ Day 4
o RDS
o Suspected sepsis, investigations- FBC was normal, culture status- blood,
CSF chemistry, Gram stain

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 o Anemia-hematocrit
o Hyperbilirubinemia, state cause: ABO, Rh & Coombs status, Rx:
phototherapy
• Current plan
➢ Mention physical findings if they are pertinent or show a change from the
norm.
➢ Do not repeat non-active problems, keep your presentation concise.
➢ After completing your presentation, give a brief summary of your plans for
the day, or over the next couple of hours, if necessary.
➢ Write these down on the bottom of your progress notes and make a daily
checklist. The progress notes must have clear plans at the end of the
problems.
• After rounds: Enter new orders in the patient’s files and communicate with
nurses.

FEEDS AND FLUIDS

FLUID REQUIREMENTS
• Water is the primary need in the first 24-48 hours due to physiologic diuresis.
• NOTE: NEVER GIVE A CHILD BELOW THE AGE OF 6 MONTHS PLAIN
WATER.
• The expected early postnatal weight loss is higher in preterm infants. Preterm
infants may lose up to 15% of their body weight.
• Hyaline membrane disease may be associated with delayed postnatal diuresis.
Transdermal water losses are high in very preterm infants.
• All infants <30 weeks GA/ <1200g must be placed into a plastic bag at delivery
without drying and the face exposed. The weight should be monitored daily.
• Cover all infants <30 weeks/ <1200g and under a radiant heater with a clear
plastic.
• Fluids to be started with include 5% DW (extremely low birth weight) and 10%
dextrose.
Birth weight (g) Fluid of choice Initial total fluid intake (day Glucose
1) mg/kg/min
<1000 5% DW 90 ml/kg/day 3.2
1000g-1199g 10% dextrose 80ml/kg/day 5.6
1200-1499g 10% dextrose 70ml/kg/day 4.2

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>1500g 10% dextrose 60ml/kg/day 4.2

• General goals:
➢ Keep glucose at 2.6- 7 mmol/L: If ELBW infant becomes hypoglycemic
give D10% 3ml/kg then change fluids to 10% Neonatolyte (NNL)/10%
dextrose
➢ Increase total daily increment to 10-20ml/kg/day dependent on the urine
output, wight and serum sodium. Do not forget to subtract breastfeeding
volume from total fluid volume.
➢ After 36-48 hours, change 5%DW to ¼ strength Darrow’s solution in 10%
dextrose (or 5% pediatric maintenance solution or 10% Neonatalyte)
depending on the blood glucose
➢ Avoid oliguria <0.5ml/kg/day. Generally, >6 week nappies is a good urinary
output, aim for 0.5-1 ml/kg/hour.
➢ Keep daily urine output ≥1ml/kg/h.
➢ Keep Na+ at 135-145mmol/L.
➢ Keep K+ at 4-5mmol/L.
➢ Keep Cl- at 98-108mmol/L.
➢ Monitor weight: excessive changes in weight may reflect rapid changes in
fluid status. Weight loss >10% of birth weight is of concern
• Note: 5% DW is suitable for ELBW infants because it has low solute load which
can easily be handled by the immature kidneys.
• Thermal energy requirement:
➢ Full term infant: 60-80kcal/kg/day in first week of like and thereafter 80-
120kcal/kg/day
➢ Preterm: 50-90kcal/kg/day in the first week of like and thereafter 100-
150kcal/kg/day
ENTERAL FEEDING
• Breast milk feeding must be encouraged for all infants.
• Donor breast milk is preferable to formula for preterm infants unable to access
mother’s own breast milk.
• Fluids and enteral intake should be individualized for sick infants and infants
with risk factors or feed intolerance.
• Infants ≥1500g and ≥32 weeks
➢ In the absence of mother’s own milk, consider preterm formula.

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 ➢ Start on bolus feeds 2-3 hourly at 60ml/kg on day 1.
➢ Increase to 75, 100, 125, 150ml/kg/day from Day 2 to Day 5 as feeds are
tolerated.
• Infants <1500 or 32 weeks
➢ Start with bolus tube feeds (expressed breast milk)
➢ Orogastric tubes are preferable to nasogastric tube.
➢ Start milk on D1 at 12-24ml/kg/day
➢ Increase feeds daily by 24 ml/kg/day
➢ Consider continuous feeds if prolonged significant feeding intolerance-
discuss with senior clinician
➢ Syringes should be changed every 4 hours for feeds given via a syringe
driver
➢ Continue until a rate of 10ml/hr or 1200g is achieved before challenging 2
hourly bolus feeds.
➢ Stop supplemental IV fluids when an enteral intake of 150ml/kg/day is
achieved (consider individual baby tolerance)
➢ Increase enteral volume incrementally to 180-200ml/kg/day.
➢ Add supplements and breast milk fortifier according to guideline
➢ The feeding tube must be changed weekly and the administration set 3 times
a week
➢ Initiate TPN for confirmed prolonged feeding intolerance >3 days and for
confirmed cases of NEC.
• Criteria for nil per oral
➢ Tense abdominal distention
➢ Erythema of the abdominal wall
➢ Decreased bowel sounds
➢ Gross or occult blood in the stools
➢ Abdominal tenderness
➢ Heavy bile stained nasogastric aspirate
➢ Pneumatosis intestinalis on AXR
➢ Vomits after 2 consecutive feeds with the feeding tube in the correct position

INCUBATOR CARE

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CARING FOR A BABY IN AN INCUBATOR
• An incubator is a glass-covered, fully enclosed neonatal bed connected to a
power source, providing an environment where heat and humidity can be
controlled to suit the needs of the neonate requiring the facility.
• Uses of an incubators include:
➢ Provision of a thermo-neutral environment.
➢ Provision of a clear view of observation of a very ill neonate.
➢ Provision of stress free environment for a very ill neonate where minimal
handling can be instituted.
• Indications for an incubator include:
➢ Grossly premature babies or Very low birth weight (<1.2kg).
➢ Hypothermic neonates with temperature <36.5oC. especially premature
babies.
➢ Neonates having repeated or prolonged apneic spells.
➢ Neonate having convulsions.
➢ Very ill neonates to relieve stress.
➢ Neonates who are ventilated.
ADVANTAGES OF AN INCUBATOR
• Provides a thermo-neutral environment thereby reducing heat loss of the
neonate.
• Makes observation of a neonate easy with minimal handling.
• Provides an easy access to the neonate for all nursing procedures whilst
maintaining baby’s temperature.
• Offers facilities for administration of controlled percentage of oxygen and
humidified air.
• Provides an environment which allows for connection to ventilators and other
devices without stressing the neonate
DISADVANTAGES OF AN INCUBATOR
• Can be a source of infection as the warm and humidified environment is a good
media for growth of micro-organisms hence if distilled water is not changed and
the incubator is not properly cleaned, this can be a danger.
• Can cause dryness of the skin and mucus membrane if the air is not humidified
and this can lead to cracking of the neonate’s skin and even bleeding.

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INCUBATOR CARE
• Daily cleaning can be conducted through portholes without removing the
neonate, however, total dismantling and cleaning can be done when the
incubator is badly soiled or when a baby graduates from the incubator or dies.
• Weekly cleaning can be done on empty incubators and these incubators are
dismantled, decontaminated, cleaned and sterilized. Allow for 24 hours for this
process.
• Empty incubators should be kept warm at an average incubator temperature of
34oC and adjusting according to the neonate’s skin temperature. Always
compare knob set temperature with the incubator temperature inside to ascertain
accuracy.
• All incubators should be humidified with distilled water put in reservoir tanks
and the water should be changed on daily basis.
• Humidity should be kept 40-60% for term neonates and 60-80% for premature
and ventilated babies.
• Incubator temperature should be set to provide a neutral thermal environment
according to the neonate’s body temperature and in response to the set
temperatures.
• All opening on the incubator should be kept closed at all times and procedures
should be conducted through portholes unless impossible e.g. resuscitation.
OBSERVATIONS AND NURSING CARE OF AN INCUBATOR
BABY
• Frequency of observation will depend on the neonate’s condition.
• Temperature:
➢ Should be checked and if the infant is hypothermic or hyperthermic, regulate
the incubator temperature by either increasing or reducing by 1 oC at a time
and rechecking the Axillay temperature of the neonate every 15 minutes.
• Respirations
➢ Breathing can be checked on looking at chest rise. If apnea, see if more than
10 seconds and stimulate if prolonged or repeated-use of bag and mask.
➢ Insert a nasogastric tube to expel air from the stomach to avoid splinting. An
apnea monitor can be used. Inform medical officer for further management.
• Heart rate
➢ Can be checked on breathing and color of the neonate.

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 ➢ A cardiac monitor can be used. If HR <100, resuscitate and inform-medical
officer/senior doctor.
• Weight: can be done if necessary especially if incubator scale is not available
• Feeding- preferably nasogastric tube feeding as per calculated amount per kg
body weight/24 hours.
• Eliminations
➢ Urine: can be observed in terms of color, odors and can be measured using
urine collectors, counting nappies or measuring nappy weight pre and post
urination.
➢ Stool- can be observed for consistency and color, whether there is diarrhea,
melena or meconium also note the frequency.
• Other nursing care can be done as required.

EXAMINATION OF THE NEWBORN

• All newborns must be fully examined within 24 hours of birth and at discharge.
• Before examining:
➢ Introduce yourself and ask the mother/parents if you may examine their baby
➢ Explain the purpose of the examination
➢ Check the maternal notes for:

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 o Antenatal details
o Booking blood results (HIV, RPR, HBsAg, blood group, blood
glucose)
o Antenatal ultrasound results
o Antitetanus toxoid injections
o Maternal medical or social problems.
o Maternal drug history
o Family history
o Details of labor
o Risk for infection (PROM, fever, UTI, GBS, chorioamnionitis,
infections of note)
➢ Check neonatal records for:
o Resuscitation details
o Birth weight and gestational age
o Condition of baby since birth (feeding, passed urine and meconium)
o Previous management and follow up plans made
• Note: certain observations are made while talking to the mother and while
undressing the baby. Completely undress the baby, ensure the baby does not
become hypothermic
• Examination follows:
➢ General appearance
➢ Color of baby
➢ Skin lesions
➢ Heart sounds and murmurs
➢ Palate
➢ Observe and palpate the abdomen
➢ Genitalia
➢ Hips
➢ Plot anthropometry (weight, length and head circumference)
➢ Spontaneous activity of infant
➢ Palpate all pulses
➢ Patency of the upper airway
➢ Signs of respiratory distress
➢ Hernia sites
➢ Anus (position/patency)
➢ Spine
➢ Red reflex in both eyes

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 ➢ Neurological (do a Ballard score if premature)
o Head control by pulling to sit
o Moro, grasp and suck reflex
o Truncal control in ventral position
o Palpate anterior fontanelle

SIZE AT BIRTH
• Recall average birth weight= 3kg (2.5 – 3.5 kg)
➢ Babies born weighing between 1500-2500g= low birth weight (LBW).
➢ Babies born weighing between 1000-1500g= very low birth weight (VLBW).
➢ Babies less than 1000g= Extremely low birth weight (ELBW).
• Babies with a birthweight below the 10th centile for gestational age are called
small for gestational age or small-for-dates.
• The majority of these infants are normal but small.
• The incidence of congenital abnormalities and neonatal problems is higher in
those whose birthweight falls below 2 SD below the mean.
• An infant’s birthweight may also be low because of preterm birth, or because
the infant is both preterm and small for gestational age.
• Small-for-gestation age infants may have grown normally but are small or they
may have experienced intrauterine growth restriction (IUGR) i.e. they have
failed to reach their full genetically determined growth potential and appear thin
and malnourished.
• Babies with a birthweight above the 10th centile may also be malnourished e.g.
a fetus growing along the 80th centile who develops growth failure and whose
weight falls to the 20th centile.
PATTERNS OF GROWTH RESTRICTION
• Has been classified as:
➢ Asymmetrical growth restriction (common): weight or abdominal
circumference lies on a lower centile than that of the head. This occurs when
the placenta fails to provide adequate nutrition late in pregnancy but brain
growth is relatively spared at the expense of liver glycogen and skin fat.
o Associated with: utero-placental dysfunction secondary to maternal pre-
eclampsia, multiple pregnancy, maternal smoking or it may be
idiopathic.
o These infants rapidly put on weight after birth.

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 ➢ Symmetrical growth restriction: head circumference is equally reduced. It
suggests a prolonged period of poor intrauterine growth starting in early
pregnancy (or that the gestational age is incorrect). It is usually due to a small
but normal fetus, but may be due to a fetal chromosomal disorders or
syndromes, a congenital infection, maternal drug and or alcohol abuse or a
chronic medical condition or malnutrition.
o Infants are more likely to remain small permanently
THE GROWTH RESTRICTED INFANT
• After birth, these infants are liable to:
➢ Hypothermia because of their relatively large surface area
➢ Hypoglycemia from poor fat and glycogen stores
➢ Hypocalcemia
➢ Polycythemia (venous hematocrit > 0.65)
LARGE FOR GESTATIONAL AGE INFANTS
• Large for gestational age infants are those above the 90th weight centile for their
gestation.
• Macrosomia is a features of infants of mothers with either permanent or
gestational diabetes, or a baby with a congenital syndrome (e.g. Beck-
Wiedemann syndrome).
• The problems associated with being large for gestational age are:
➢ Birth asphyxia from a difficult delivery.
➢ Breathing difficulty from an enlarged tongue in Beckwith-Wiedemann
syndrome
➢ Birth trauma, especially from shoulder dystocia at delivery (difficult delivery
the shoulders form impaction behind maternal symphysis pubis)
➢ Hypoglycemia due to hyperinsulinism
➢ Polycythemia

PREMATURITY

315
PREMATURITY AND
BIRTH WEIGHT
• A baby born alive before 37 weeks
(259 days) of gestation is
considered premature.
➢ Late preterm (34- 37 weeks)
➢ Moderate preterm (32-34
weeks)
➢ Very preterm (28-32 weeks)
➢ Extreme preterm (less than 28)
• Terms that refer to premature
babies (Prematurity) are preterm
and preemie.
• Preterm babies are at risk for a
number of complications related to
the fact that their organs may not be
mature at the time of birth.
• The earlier the baby is born the
higher the risk of complications.
• Recall average birth weight= 3kg
(2.5 – 3.5 kg)
➢ Babies born weighing between
1500-2500g= low birth weight
(LBW).
➢ Babies born weighing between
1000-1500g= very low birth
weight (VLBW).
➢ Babies less than 1000g=
Extremely low birth weight
(ELBW).
• Most babies that are considered to
have low birth weight are
premature however, other
conditions can cause LBW in a
baby born after a full-term
316
pregnancy, such as smoking during
pregnancy.
• Babies with LBW who are full term
(but underweight) and “premature
babies” with weights less than
expected are termed small for
gestational age (SGA).
• Causes for intrauterine growth
restriction include:
➢ Infections of the fetus before
delivery
➢ Chromosome or gene
abnormalities (S-SGA)
➢ Insufficient nutrition provided
by placenta (A-SGA).
➢ Poor nutrition in the mother,
other problems such as chronic
disease or smoking (A-SGA)
CAUSES OF PREMATURITY
• Many factors are linked to
premature birth. Some factors
directly cause early labor and birth
while others can make the mother
or baby sick and require early
delivery.
• Maternal factors include:
➢ Pre-eclampsia (toxemia or high
blood pressure of pregnancy
occurring after 20 weeks of
pregnancy)
➢ Chronic medical illness (such
as heart or kidney disease,
SCD)
➢ Infection (such as group B
streptococcus, urinary tract
infections, vaginal infections,
 infections of the fetal/placental • Every premature infant is different
tissues) however features of prematurity
➢ Drug use (such as cocaine) include:
➢ Abnormal structure of the ➢ Very small size (at 24 weeks-
uterus male 700g, female 620g)
➢ Cervical incompetence ➢ Very thin fragile skin with
(inability of the cervix to stay veins visible underneath (Dark
closed during pregnancy) red color all over the body)
➢ Previous preterm birth ➢ Soft hair on the body: lanugo
• Factors involving the pregnancy ➢ Ears: Pinna soft, no recoil,
➢ Abnormal or decreased startles to loud noise
function of the placenta ➢ Eyes: fused eyelids, infrequent
➢ Placenta previa (low-lying eye movements.
position of the placenta) ➢ Faint cry
➢ Placental abruption (early ➢ Breast tissue: no breast tissue
detachment of the placenta palpable
from the uterus) ➢ Genitalia:
➢ Infection of the placenta o Male: Scrotum lacks rugae
➢ Premature rupture of (smooth), no testes in
membranes (amniotic sac) scrotum (cryptorchidism)
➢ Polyhydramnios (too much o Female: prominent
amniotic fluid) clitoris, labia majora
• Factors involving the fetus widely separated, labia
➢ When the fetal behavior minora protruding
indicates the intrauterine ➢ Little activity, weak cry
environment is not healthy ➢ Feeding problems: baby can’t
➢ Multiple gestation (twins, suck or swallow normally
triplets etc.) ➢ Breathing problems: apnea of
➢ Congenital anomalies prematurity
➢ Limbs extended. Jerky
FEATURES AND movements
COMPLICATIONS OF
PREMATURITY
FEATURES OF PREMATURITY

317
 318

COMPLICATIONS OF blood vessels due to hypoxic or

PREMATURITY
CENTRAL NERVOUS SYSTEM
• Intraventricular hemorrhage with
hydrocephalus.
➢ Intraventricular hemorrhage is
rupture of germinal matrix
hypotensive injury.
➢ Predisposing factors:
prematurity, RDS, hypo or
hypervolemia and shock.
➢ Signs and symptoms: most
asymptomatic, lethargy, poor
suck, high-pitched cry and
bulging fontanelle.
 ➢ Diagnosis: cranial ultrasound
(through anterior fontanelle)
➢ Treatment: directed toward
correction of underlying
condition (RDS, shock etc.) In
cases of associated
hydrocephalus, placement of
ventriculoperitoneal shunt may
be required.
• Hemorrhagic and periventricular
white matter brain injury
(leukomalacia)
• Cerebral palsy (difficult muscle
control, stiffness)
• Learning disability/ Mental
retardation
• Deafness
• Retinopathy of prematurity: this is
a proliferative retinopathy
➢ Caused by proliferation of
immature retinal vessels due to
excessive use of oxygen.
➢ Can lead to retinal detachment
and blindness in severe cases.
➢ Diagnosis: all very low birth
weight infants should be
screened for ROP with an
ophthalmoscopic exam.
➢ Treatment: Laser surgery may
be needed in severe cases.
• Blindness
RESPIRATORY SYSTEM
• Respiratory distress syndrome
(hyaline membrane disease): the
lungs are immature and do not
produce surfactant (which is
319
produced from the pneumocyte
type II cells as early as 20 weeks
but peaks at 35 weeks of gestation).
➢ Incidence: infants <32 weeks
gestation
➢ Signs and symptoms: seen
within first 3 hours of birth,
tachypnea, grunting and
cyanosis.
➢ Surfactant contains both
Lecithin and sphingomyelin.
➢ The Lecithin-syphingomyelin
ratio is a determining factor in
lung maturity.
➢ A L-S ratio greater than 2.0 to
2.5 is indicative of lung fetal
lung maturity.
• In addition, the alveoli are small,
inflate with difficulty and do not
remain gas filled between
inspirations. The rib cage is weak
and compliant.
• There is high surface tension and
propensity for alveolar collapse.
➢ Alveolar collapse results in
progressive atelectasis,
intrapulmonary shunting,
hypoxemia and cyanosis.
• Apnea of prematurity: due to
immaturity of the breathing center
in the brain
➢ Apnea: cessation of breathing
for more than 20 seconds
associated with bradycardia
• Chronic lung disease/
bronchopulmonary dysplasia
 ➢ Need for supplemental oxygen CARDIOVASCULAR SYSTEM
beyond 28 days of life. • Persistent/patent ductus arteriosus
➢ Characterized by squamous • Anemia of prematurity
metaplasia and hypertrophy of • Hypotension
small airways. • Atrial septal defects
➢ Infants with
• Ventricular septal defects
bronchopulmonary dysplasia
can be wheezing remember, GASTROINTESTINAL SYSTEM
“not all that wheezes is • Necrotizing enterocolitis
asthma!” • Neonatal jaundice
➢ Treatment: supplemental • Poor feeding
oxygen, oral steroids,
bronchodilators. GENITOURINARY SYSTEM
• Pneumothorax • Inguinal hernias
• Note in extremely premature babies METABOLIC
(23-25 weeks) the lung may be
• Hypoglycemia
incompletely developed (in
• Hypothermia
addition to being immature) these
• Hypocalcemia
rarely survive outside the womb.
• Electrolyte and fluid imbalance
• Recall the five stages of lung
• Osteopenia of prematurity
development include:
➢ Embryonic stage: 3-8 weeks
➢ Pseudoglandular stage: 5-16
• Rickets of prematurity
weeks
➢ Canalicular stage-16- 26 weeks • Infections: neonatal sepsis
➢ Terminal saccular-26-37 week • Perinatal asphyxia
➢ Alveolar stage- birth until early
childhood (8 years)

320
321
DIAGNOSIS to provide an estimate of an infant’s
• History: gestational age (Ballard score).
➢ Risk factors: Young maternal
age, multiple pregnancy,
infection, maternal illness (e.g.
pregnancy induced
hypertension), cervical
incompetence, antepartum
hemorrhage
➢ Full obstetric history
➢ Condition at birth: APGAR
score, resuscitation required
➢ Gestation: must be known to
give accurate prognosis.
Calculate from menstrual
period, by early dating
ultrasound scan or by
assessment of gestation after
birth
➢ Associated problems such as
twin pregnancy (much higher
risk of poor neurological
outcome), congenital
abnormalities or infection
(chorioamnionitis may have
been a trigger for preterm
labor)
➢ Antenatal steroids: if given,
these reduce the incidence of
respiratory distress syndrome
and intraventricular
hemorrhage
• Examination: features of
prematurity
• The external appearance and
neurological findings can be scored

322
323
MANAGEMENT
• Admit to neonatal intensive care
unit (NICU) for special care and
monitoring.
• Temperature control: maintain
temperature in an incubator at ideal.
➢ Parents are encouraged to
participate in as much care of
their baby as they are
comfortable providing. This
may involve holding, Kangaroo
care, diaper changes, giving
baths, feeding.
• Feeding
➢ Total parenteral nutrition or
intravenous feeding
➢ Gavage feeding: Preterms
before the 34th week of
gestation need to be fed
through a nasogastric tube or
nasojejunal tube.
➢ Breast feeding often has to be
delayed. Expressed breast milk
can be given in a bottle.
➢ Decisions about feeding
depend on the infant’s
particular circumstances.
• Prevention of the infection: hand
washing and aseptic techniques
• Treatment of complications.
• In anticipation of RDS secondary to
surfactant deficiency, doses of
324
dexamethasone (steroid) can be
given to the mother 48 hours before
delivery- this is assuming that the
lungs are already at the terminal
saccular stage thus they can be
stimulated to produce surfactant.
TRANSIENT TACYPNEA
OF THE NEWBORN
• This is the commonest cause of
respiratory distress in term infants.
• Infants with transient tachypnea of
the newborn (TTN) within the first
few hours of birth presents with
tachypnea, increased oxygen
requirement, and occasional
hypoxia noted on arterial blood
gases without concomitant carbon
dioxide retention.
• Causes include:
➢ Cesarean section
➢ Maternal asthma and smoking
➢ Prolonged labor
PATHOPHYSIOLOGY
• It is caused by delay in the
resorption of lung liquid and is
more common after birth by
caesarean section.
➢ It was initially thought to be a
problem of relative surfactant
 deficiency but it is now that both TTN and RDS have
characterized by an airspace defective amiloride sensitive
fluid burden secondary to sodium transport (ENaC)
inability to absorb fetal lung
fluid.
CLINICAL FEATURES
➢ An infant born by cesarean HISTORY
delivery is at risk of having • Signs of RD (tachypnea, nasal
excessive pulmonary fluid as a flaring, grunting, retractions,
result of not experienced all the cyanosis in extreme case) becomes
stages of labor and subsequent evident shortly after birth.
low release of counter- • This disorder is transient with
regulatory hormones at the time resolution occurring by age 72
of delivery. hours.
• In vivo experiments demonstrated
PHYSICAL EXAMINATION
that lung epithelium secretes Cl-
• Findings include:
and fluid throughout gestation but
➢ Tachypnea with variable
only develops the ability to actively
grunting
reabsorb Na+ during late gestation.
➢ Flaring
• At birth the mature lung switches
➢ Retracting
from active Cl- (fluid) secretion to
➢ Cyanosis (extreme cases)
active sodium (fluid) absorption in
response to circulating WORKUP
catecholamines. • Arterial blood gas
• Changes in oxygen tension • Pulse oximetry
augment the sodium transporting • The Chest X-ray (diagnostic
capacity of the epithelium and standard) may show fluid in the
increase gene expression for the horizontal fissure.
epithelial amiloride sensitive ➢ Prominent perihilar streaking
sodium channel (ENaC). (markings) which correlates
• The inability of the immature fetal with the engorgement of the
lung to switch from fluid secretion lymphatic system with retained
to fluid absorption results at least in lung fluid and fluid in the
large part, from an immaturity in fissures.
the expression of ENaC which can ➢ Patchy infiltrates have also
be upregulated by glucocorticoids. been described
• Bioelectrical studies of human
infants nasal epithelia demonstrate

325
 airways obstruction e.g.
choanal atresia,
pulmonary hemorrhage
➢ Extrapulmonary:
o Congenital heart disease
o Intracranial birth
trauma/encephalopathy
o Severe anemia
• Additional ambient oxygen may be o Metabolic acidosis
required.
MANAGEMENT
• The condition usually settles within
• Medical care is supportive. As the
the first day of life but can take
retained lung fluid is absorbed by
several days to resolve completely.
infant’s lymphatic system the
• This is a diagnosis made after
pulmonary status improves.
consideration and exclusion of
• Supportive care include:
other causes.
➢ Intravenous fluids
• When managing an infant with
➢ Gavage feeding (until the
TTN it is important to observe for
respiratory rate has decreased
signs for clinical deterioration that
[<60bpm] enough to allow oral
may suggest other diagnoses and to
feedings)
observe closely for the
➢ Supplemental oxygen to
development of fatigue
maintain adequate arterial
• Causes of Respiratory distress in oxygen saturation
term infants: ➢ Maintenance of
➢ Pulmonary thermoneutrality
o Common: transient ➢ Environment of minimal
tachypnea of the newborn stimulation
o Less common: meconium
• As TTN resolves the infant’s
aspiration, pneumonia,
tachypnea improves, oxygen
RDS, Pneumothorax,
requirements decrease and the CXR
persistent pulmonary
shows resolution of perihilar
hypertension of the
streaking.
newborn, milk aspiration
• If condition worsens continuous
o Rare: diaphragmatic
positive airway pressure (CPAP) by
hernia, tracheo-
nasal prongs or endotracheal tube
esophageal fistula (TOF),
pulmonary hypoplasia,

326
 or mechanical ventilation can be ➢ Seen within the first 3 hours of
done. birth.
• The use of medications for TTN is ➢ Tachypnea (>60breath/min)
minimal ➢ Laboured breathing with chest
• Aside from use of antibiotics for a wall recession (particularly
period of 36- 48 hours after birth sternal and subcostal
until sepsis has been ruled out, no indrawing) and nasal flaring.
further pharmacotherapy generally ➢ Expiratory Grunting
is prescribed. ➢ Cyanosis if severe
• Diuretics have not been shown to
be beneficial.

RESPIRATORY DISTRESS
SYNDROME

RESPIRATORY
DISTRESS SYNDROME
• Respiratory distress syndrome
(RDS) occurs secondary to
insufficiency of lung surfactant due
to immaturity of surfactant
producing type 2 alveolar cells.
• Alveoli are small, inflate with
difficulty and do not remain gas
filled between inspirations.
• Ribcage is weak and compliant.
• High surface tension and
propensity for alveolar collapse is
high.
• Alveolar collapse results in
progressive atelectasis, Figure 36: Etiology of respiratory
intrapulmonary shunting, distress in term infants
hypoxemia and cyanosis.
• Diagnosis:
• This is usually seen in infants <32
➢ Chest X-ray- fine, diffuse
weeks’ gestational age.
reticulogranular or “ground
• Signs and symptoms:

327
 glass” pattern and air
bronchograms.
• Treatment:
➢ Aggressive respiratory support
including
o Oxygen
o Continuous positive
airway pressure (CPAP)
o Intubation
o Mechanical ventilation
➢ To decrease barotrauma, novel
methods of ventilation are
sometimes used- high
frequency oscillation, jet
ventilation and liquid
ventilation.
➢ Exogenous surfactant
replacement (instillation via
endotracheal tube) has
dramatically reduced mortality
in infants with RDS.

328
 HYPOXIC ISCHEMIC ENCEPHALOPATHY

HYPOXIC ISCHEMIC ENCEPHALOPATHY

• This is acute or subacute brain injury due ischemia (decreased blood flow) and
hypoxia (decreased oxygen delivery).
• HIE is characterized by abnormal level of consciousness, abnormal tone and
abnormal primitive reflexes. Abnormal breathing and seizures may occur.
• A baby who is acutely asphyxiated will go through a period of primary apnea into
terminal apnea, if the hypoxia insult is not terminated early.
• Hypoxic ischemic encephalopathy (HIE) has no predilection for race or sex.
• The condition is seen in the newborn period.
• Most neonates are term at birth. In most cases, the condition manifests at birth or
within a few hours after birth.
• Intraparrtum hypoxia may be suggested by the presence of one or more of the
following things:
➢ An acute intrapartum event
➢ Fetal bradycardia
➢ Reduced variability
➢ Meconium stained liquor
➢ Prolonged second stage
➢ Need for resuscitation at birth for 5 minutes or longer
➢ A 5-minute APGAR score <7
➢ Acidosis on cord or neonatal blood in the first hour of life (defined as pH <7
or base excess <-10)
CAUSES OF HYPOXIC ISCHEMIC ENCEPHALOPATHY
• Causes of hypoxic ischemic encephalopathy are many. The main cause is birth
asphyxia.
➢ Perinatal asphyxia, gas exchange, either placental or pulmonary is
compromised or ceases altogether resulting in cardiorespiratory depression.
Hypoxia, hypercapnia and metabolic acidosis follow. Compromised cardiac
output diminishes tissue perfusion.
• A lot of risk factors are implicated and these can be divided into:
➢ Prepartum:
o Maternal diabetes

329
 o Vascular disease affecting circulation to placenta
o Pre-eclampsia
o Cardiac disease: both hypo and hypertension
o Congenital infections
o Drugs and alcohol abuse
o Severe fetal anemia
o Intrauterine growth restriction
o Fetal Lung malformations
➢ Intrapartum:
o Excessive placental bleeding
o Very low maternal blood pressure
o Umbilical cord accidents
o Prolonged stages of labor (excessive or prolonged uterine contractions)
o Abnormal fetal positions
o Cord compression: cord around the neck, shoulder dystocia (difficult
child birth), cord prolapse
o Rupture of placenta/membrane/uterus
➢ Postpartum:
o Severe prematurity
o Cardiac disease
o Pulmonary disease
o Infections: sepsis
o Low neonatal blood pressure
o Brain/skull trauma
o Congenital brain malformations
PATHOPHYSIOLOGY
• Brain hypoxia and ischemia from systemic hypoxia with reduced cerebral blood
flow are the primary triggering events for HIE.
• In this regard, HIE is similar to stroke syndromes in adults, except that in
neonates the pathology is more generalized and the causes are different.
➢ Initial compensatory adjustments which include hypoxia and hypercapnia
(increased carbon dioxide partial pressure) are powerful stimuli, increasing
cerebral blood flow and thus oxygen delivery.
• During the early phase of shock, the cardiac output is redistributed (dive reflex)
and systemic blood pressure increases (due to increased epinephrine release) to
maintain Cerebral blood flow.

330
• Cerebral autoregulation mechanism maintains brain perfusion for a while in spite
of an initial drop in the mean blood pressure. The range of blood pressure in the
cerebral blood flow is set at lower limits than adult range (i.e. 60 to 100mmHg)
➢ CPP= MAP-ICP
2 1
➢ MAP= (𝐷𝑃) + (𝑆𝑃)
3 3
➢ MAP= 40-50mmHg
➢ Normal ICP
o <1.5-6mmHg in term infants
o <3-7mmHg in young children
o <10-15mmHg in adults
o ICP>20-25mmHg is considered raised ICP
• With prolonged asphyxia the early compensatory adjustments fail and cerebral
blood flow may become “pressure passive” i.e. dependent on systemic blood
pressure.
• As systemic blood pressure falls, cerebral blood flow falls below critical levels
and brain hypoxia occurs this results in intracellular energy failure (due to
anaerobic respiration and lack of aerobic respiration)
• During the early phase of injury, the brain temperature drops and local release of
the neurotransmitter GABA (Gamma amino butyric acid) increases resulting in
changes that reduce cerebral oxygen demand, transiently minimizing the impact
of asphyxia.
• Neuronal cellular injury is dependent on the extent of the initial injury.
➢ Hypoxia and ischemia increases release of excitatory amino acids (glutamate
and aspartate) in cerebral cortex and basal ganglia. These amino acids cause
cell death by increasing intracellular calcium (apoptosis).
➢ These excitatory amino acids also cause nitric oxide to be released and this
may exacerbate the neuronal damage although its mechanisms are unclear.
➢ Energy failure also causes the inactivation of the sodium potassium ATPase
pump and this leads to retention of intracellular sodium accompanied by
influx of water into the cell causing swelling and death.
➢ There is also destruction of ion pumps by lipid peroxidation of cell
membranes. This also causes influx of water, swelling and death.
• Following the initial phase of energy failure from asphyxia injury, cerebral
metabolism may recover only to deteriorate in the second phase.
• Reperfusion injury is a second determinant to the extent of brain damage. By 6-
24 hours after initial injury a new phase of neuronal destruction sets in

331
 characterized by apoptosis. This is also known as “delayed injury” and it may
continue for days to weeks. The severity of brain injury in this phase correlates
well with the severity of long term adverse neurodevelopmental outcome in
infants.
• Reperfusion injury is often associated with inflammatory reactions and may
result in hyperkalemia.
CLASSIFICATION (SIGNS AND SYMPTOMS)
• The symptoms depend on whether the hypoxic-ischemic encephalopathy is:
➢ Mild (Stage 1- HIE-1)-‘Sympathetic stimulation’
o The infant is irritable, responds excessively to stimulation (hyperalert
with predominant sympathetic activity), may have starring of the eyes
and hyperventilation and has impaired feeding.
o Muscle tone may be slightly increased/normal and deep tendon reflexes
may be brisk in the first few days
o Transient behavioral behaviors such as: poor feeding, irritability or
excessive crying.
o It is also associated with tachypnea and tachycardia.
o APGAR score is between 6 and 7.
o There are no seizures.
o Typically resolves in 24hours
➢ Moderate (Stage 2- HIE II)- ‘Parasympathetic stimulation’
o In stage to the baby is lethargic, develops seizures and may be hypotonic
with parasympathetic over-activity.
o Seizures may occur within the first 24 hours of life.
o The infant shows marked abnormalities of tone and movement, cannot
feed.
o Reflexes are sluggish or absent.
o There is occasional apnea with bradycardia.
o APGAR score is between 4-5.
o Recovery is between 1-2 weeks and is associated with better long-term
outcome.
o An initial period of well-being may be followed by sudden deterioration,
suggesting reperfusion injury during this period seizure intensity might
increase.
➢ Severe (Stage 3- HIE III)

332
 o There are no normal spontaneous movements or response to pain (coma/
Stupor).
o Limbs are flaccid (hypotonic)
o Breathing is irregular and infant often requires ventilation support.
o Reflexes are absent and there is severe apnea.
o Seizures are prolonged and often refractory to treatment.
o Multi-organ failure is present.
o APGAR score is between 0-3.
o Babies usually deteriorate and die or survive with multiple
complications such as cerebral palsy.
ASSESSMENT OF SEVERITY OF ASPHYXIA
HIE-1 HIE-2 HIE-3
APGAR 6-7 4-5 0-3
Conscious level Irritable/ hyperalert Lethargic Comatose/ stupor
Tone Normal Hypotonic Hypotonia/ flaccid
Primitive reflex Exaggerated Depressed or Absent
(moro, grasp Absent
reflexes)
Brain stem reflex Normal Normal Impaired
(corneal, gag
reflexes)
Pupils Mydriasis Miosis Variable often
unequal, poor light
reflex
Seizures Absent Present (focal or Present/absent not
multifocal) responding to
conventional
measures
Respiration Tachypnea Occasional apnea Severe apnea
Heart Tachycardia Bradycardia Variable
Recovery < 24 hours 1-2 weeks -

333
THE THOMPSON HIE SCORE
• The Thompson HIE score should be performed on admission, before 5 hours of
age and daily until normal or age 7 days (up to 10 days if cooled).
• The maximum score, based on the clinical signs in the previous 24 hours, is
recorded in each category and then totaled for the day.
• A score of <5 in the first 5 hours of life is associated with a normal aEEG at 6
and 24 hours.
• If infants are not cooled:
➢ A peak score of 10 or less during the first 6 days with a score of 0 by day 7
predicts a normal outcome.
➢ A score of 11-15 at any time or score above 0 on day 7 predicts an abnormal
outcome in 65%.
➢ A score of above 15 predicts an abnormal outcome in 92%.

334
DIAGNOSIS AND INVESTIGATIONS
• History and Examination: Many clinical features of neonatal HIE are nonspecific,
as such the diagnosis is made with caution and only after a careful assessment of
history, physical examination and laboratory studies.
• History:
➢ Maternal and family history: health, consanguinity, congenital abnormalities,
perinatal death
➢ Previous pregnancies: live/still born or miscarriage or neonatal deaths
➢ Current pregnancy: gestation, medication, events, booking investigations and
scans.
➢ Labor and delivery: augmentation, fetal heart monitoring, medication, sepsis,
intrapartum events, length 2nd stage, presentation, method, and indication for
delivery
➢ Resuscitation and evidence for intrapartum hypoxia:
o Blood gas on cord
o APGAR scores at 1, 5 and 10 minutes
o Need for intubation and details
o Time of first gasp and onset of heart >100bpm
o Time onset of regular (non-gasping) respirations
o Drugs/fluid administered to infant
➢ Placental information: record appearance and weight- send for histology if
there is diagnostic uncertainty.
• Investigations:
➢ Full blood count and blood culture
➢ Serum electrolytes: sodium, potassium, calcium, magnesium
➢ Urea and Creatinine
➢ Group and Save, Cross match
➢ For sepsis, perform septic screen including a lumbar puncture
➢ Clotting profile/panel- prothrombin time, Partial thromboplastin time and
fibrinogen levels
➢ Cardiac and liver enzymes
➢ Serum bilirubin
➢ Arterial blood gases
➢ Imaging:
o Cranial ultrasonography: perform at least on day 1 and again on day 7-
14. Cranial US shows gross anatomy, established damage at birth and
evolving focal or global injury.

335
 o MRI brain: If available, MRI is the
most reliable guide to prognosis
and diagnosis available. It is best
done at 7-14 days for optimal
prognostic information.
o Echocardiography
o Amplitude-integrated
Electroencephalography (aEEG):
cerebral function monitor
▪ If available, apply aEEG to all
infants with signs of HIE as
soon as possible Figure 37: MRI of the brain at term. Left: HIE showing
▪ Monitor for at least 6 hours abnormal (white) signal in the basal ganglia and thalami and
if not cooled (preferably absence of signal in the internal capsule bilaterally. Right:
normal scan showing grey basal ganglia and a white signal
24h). If cooled, continue from myelin in the posterior limb of the internal capsule.
until rewarm is complete.
▪ Background patten should only be used to prognosticate in the
“crosshead” mode
▪ Record in the notes, seizure activity at any time and background
pattern at age 6 hours and then every 24 hours.
▪ A severely suppressed background persisting for 6 hours predicts a
poor outcome in over 80% and if this persists beyond 48 hours,
despite cooling, the prognosis is almost invariably poor
▪ A normal voltage pattern at 48 hours in cooled infants associated
with a good outcome in most infants
➢ Hearing test

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MANAGEMENT
IMMEDIATE MANAGEMENT ON ADMISSION
• Initial resuscitation, stabilization and treatment is largely supportive and should
focus on:
Immediate management
➢ Admit of NICU
➢ Perform ABCs: Suction if still having blocked • Admit to NICU
airways. If baby not breathing, ambu bag and • ABCs
intubate. If no spontaneous respirations, put on • Put on oxygen
ventilator on intermittent positive pressure • Check vital
ventilation (IPPV). • Give glucose at 3ml/kg
➢ Adequate ventilation: put on oxygen once • Document Thompson HIE
breathing is spontaneous. score
➢ Check vitals: Record level of consciousness,
• For signs of
respirations, temperature, heart rate, blood
encephalopathy:
pressure, capillary refill time, oxygen saturation,
➢ Nil per oral
tone, urine output and blood glucose.
➢ Start IV fluids at
➢ Give glucose at 3ml/kg
40ml/kg/day
➢ Document the Thompson HIE score
➢ Apply aEEG as soon as
➢ If there are signs of encephalopathy
possible
o Keep nil by mouth
• Consider cooling protocol
o Commence IV fluids at 40ml/kg/day
for moderate to severe HIE
o Apply the aEEG as soon as possible if
available
➢ If there are clinical or aEEG signs of moderate to severe HIE, using cooling
protocol- discuss all cases with senior.
CLINICAL MANAGEMENT
• Principles:
➢ Respiratory support
➢ Recording of amplitude-integrated electroencephalogram (aEEG, cerebral
function monitor) to detect abnormal background activity to confirm early
encephalopathy or identify seizures
➢ Treatment of clinical seizures with anticonvulsants
➢ Fluid restriction because of transient renal impairment
➢ Treatment of hypotension by volume and inotrope support
➢ Monitoring and treatment of hypoglycemia and electrolyte imbalance
especially hypocalcemia

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• Recent randomized trials have shown that mild hypothermia (cooling to a rectal
temperature of 33-34oC for 72h by wrapping the infant in a cooling blanket)
reduces brain damage if started within 6 hours of birth.
TEMPERATURE AND COOLING
• Avoid overheating infants at any stage.
• All cooled and borderline cases should be discussed with a senior clinician.
• It is not necessary to obtain the aEEG result before cooling if infants have obvious
moderate-severe encephalopathy on clinical grounds.
• Criteria for cooling:
➢ All of the following
o ≥35-weeks gestation
o Birth weight >1800g
o Able to begin cooling by age 6 hours
o Absence of: severe congenital anomaly, or uncontrolled bleeding,
systemic hypotension and/or pulmonary hypertension (FiO 2>0.8) not
responding to treatment
And
➢ Suspected intrapartum hypoxia based on the presence of at least one of the
following:
o First hour blood gas (cord/infant/arterial/capillary/venous): pH ≤7 or
base deficit ≥10 mmol/l
o 5-minutes AGPAR<7 or assisted ventilation at birth continued for ≥10
minutes
And
➢ Moderate-severe neonatal encephalopathy base on one of the following:
o Abnormal amplitude-integrated EEG (aEEG) occurring at any time
during the first 6 hour defined by: moderately abnormal background,
suppressed background, discontinuous normal voltage, burst
suppression, low voltage, flat trace or seizures.
o If an aEEG is not available, moderate-severe encephalopathy can be
defined by the presence of at least one finding in at least three categories
as indicated in the table below or definite seizures.

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• Note: if the aEEG is normal but cooling has already commenced based on
appropriated clinical criteria alone, cooling should be continued for 72 hours.
Cooling should not be delayed by aEEG application in severe clinical HIE.
Cooled infants should be entered in a cooling register.
RESPIRATORY SUPPORT AND VENTILATION
• Aim for normal PaCO2 and oxygen saturation.
• If oxygen is needed and respiratory effort is good, nasal CPAP or nasal cannulae
are often adequate.
• Ventilate if apnea or respiratory acidosis with pH <7.25 and inform senior
clinician.
• If pCO2<3.3kPa wean ventilation rapidly and recheck within 30 minutes-
consider extubation.
• Wean FiO2 and peak inspiratory pressure at the bedside observing saturation and
chest wall movement.
• If ventilated do blood gases hourly or more often if abnormal- continues until
normal.
BLOOD PRESSURE, HEMOGLOBIN AND COAGULATION
MANAGEMENT
• Monitor blood pressure and keep it in the normal range (MAP=40-50mmHg).
• Give fluid bolus of normal saline at 20ml/kg if suspected hypovolemia.
• Metabolic acidosis alone, is not an indication for a fluid bolus.
• Transfuse if significant anemia (PCV <30%/Hb<10), consider occult bleeding,
consider Coombs test.

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• Check INR/PTT/Fibrinogen in severely anemic or bleeding infants e.g.
subaponeurotic hemorrhage.
• Treat active bleeding:
➢ Vitamin K 1-2mg/kg IV
➢ FFP 15ml/kg over 30 minutes (consider cryoprecipitate if poor response or
low fibrinogen)
• Treat normovolemic hypotension with inotropes usually dobutamine starting at
5mcg/kg/min as first line. If not available give Dopamine at 5mcg/kg/min.
• An echocardiogram to assess cardiac function can inform fluid and inotrope
management (if ECHO not available use capillary refill time, urine output and
blood pressure where possible as a proxy)
• Central access via umbilical vein catheter or other central line is preferable for
sick infants requiring inotropes.
FLUID BALANCE, ACIDOSIS AND METABOLIC MANAGEMENT
• Intrinsic renal failure and SIADH commonly occur.
• Initially fluid restrict to 40ml/kg/24 hours.
• Initially use potassium-free 10% dextrose solution containing 1/5-1/2 normal
saline. Ensure that maximum sodium is adhered to with the fluids available.
• Potassium containing fluids should only be used if urine output and serum
potassium are normal
• Monitor urine output, electrolyte, blood glucose and blood gases.
• Remember to palpate for bladder and massage if distended. If not distended and
oliguric (<1ml/kg/hr) or continues to retain fluid after massage, then catheterize.
• Catheterize if urine retention or oliguria (<1ml/kg/hr).
• Hypocalcemia and hypomagnesia should be anticipated and treated.
• Treat hyponatremia <130mml/l with fluid restriction if oliguric- if not oliguric
consider increasing fluids and using 0.9% saline in maintenance fluids.
• There is no proven benefit of sodium bicarbonate to treat lactic acidosis.
• Monitor plasma glucose initially at least 4 hourly:
➢ Increase IV glucose concentration if plasma glucose <2.6mmol/L
➢ Adjust according to further monitoring
➢ If hypoglycemia occurs, use 12 or 15% glucose as an infusion solution.

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FEEDING
• Beware of necrotizing enterocolitis.
• Preferably feed with breast milk.
• Introduce feeds slowly, particularly moderate-severe HIE usually nil by mouth
on admission then commence 3 hourly feeds after 12-24 hours:
➢ Increment by 12ml/kg/day during the first 3 days and by up to 30m/kg/day
thereafter if abdomen exam normal
➢ Infants with mild encephalopathy who are not cooled can be fed with higher
volume
SEPSIS
• Teat with first line antibiotics as per hospital protocol.
➢ Cefotaxime
• Discontinue antibiotics at 36 hours if sepsis ruled out.
SEIZURES
• Assess sensorium, tone and seizures.
• If seizure lasting more than 3 min or more than 3 in one hour:
➢ Start Phenobarbitone 20mg/kg IV stat.
➢ If still seizing after 15 minutes repeat loading dose phenobarbitone 10m/kg
IV stat and put on maintenance phenobarbitone 5mg/kg/day OD.
➢ If seizures are refractory to phenobarbitone, clonazepam may be used with a
loading dose of 100-200 mcg/kg followed by infusion at 10-30 mcg/kg/hour
if seizures continue.
➢ If clonazepam is not available diazepam 1mg/dose 3 hourly can be started in
addition to the maintenance phenobarbitone.
➢ If still having seizures increase the dose to 1.5mg/dose.
➢ If still having seizures reduce the frequency to 2 hourly then hourly if seizures
continue.
COMMUNICATION WITH PARENTS
• Explain the clinical condition and potential for other causes
• Explain management
• Document the parent’s version of events
• Prepare them for a potential poor outcome if investigations and signs are
suggestive

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PROGNOSIS
• Mild HIE has a good prognosis, complete recovery can be expected.
• Moderate HIE also shows full recovery on clinical neurological examination.
Infants feed normally by 2 weeks of age however if clinical abnormalities persist
beyond that time, full recovery is unlikely.
• Severe HIE has a mortality rate of 30-40% and over 80% who severe have
neurodevelopmental disabilities particularly cerebral palsy.
• If MRI at day 4-14 days in a term infant shows significant abnormalities (bilateral
abnormalities in the basal ganglia and thalamus and lack of myelin in the
posterior limb of the internal capsule), there is a very high risk of later cerebral
palsy.
• Note: Hypoxic-ischemic injury even though most commonly occurs antenatally
or during labor or delivery, it can occur postnatally or by neonatal conditions e.g.
inborn errors of metabolism and kernicterus.
• Clinical features of HIE should only be considered to have birth asphyxia if there
is:
➢ Evidence of severe hypoxia antenatally or during labor or at delivery
➢ Resuscitation needed at birth
➢ Features of encephalopathy
➢ Evidence of hypoxic damage to other organs such as liver, kidney, or heart
➢ No other prenatal or postnatal cause identified
➢ Characteristic findings on MRI neuroimaging
SYSTEMIC EFFECTS AND COMPLICATIONS
• Complications of HIE are multi-systemic and affect all systems of the body:
• CNS
➢ Cerebral infarcation
➢ Intracranial/ intraventricular hemorrhage
➢ Cerebral edema
➢ Hyper/hypotonia
• CVS
➢ Myocardial ischemia: poor contractility
➢ Tricuspid insufficiency
➢ Cardiac stunt
➢ Hypotension

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• Pulmonary
➢ Pulmonary hypertension
➢ Pulmonary hemorrhage
➢ Respiratory distress syndrome
• Gastrointestinal
➢ Necrotizing enterocolitis
➢ Perforation
➢ Hepatic dysfunction
• Adrenals: Adrenal hemorrhage
• Renal:
➢ Acute tubular or cortical necrosis
• Metabolic
➢ Inappropriate secretion of ADH (SIADH)
➢ Hyponatraemia
➢ Hypocalcemia
➢ Hypoglycemia
➢ Myoglobinuria
• Integument: subcutaneous fat necrosis
• Hematology: Disseminated intravascular coagulation

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 NECROTIZING SEIZURES

NEONATAL SEIZURES
• R
MANAGEMENT
• Treat electrolyte and glucose abnormalities and sepsis.
• Ensure adequate ventilation and perfusion. Commence CFM/Brainz monitor
• Treat seizures (clinical and/or electrical) with anticonvulsants if they are
recurrent or last more than 3 minutes.
➢ Give Phenobarbitone 20mg/kg IV infusion over 10 minutes
➢ If seizure persists repeat phenobarbitone dose
➢ If seizure still persists midazolam is given
➢ If seizure still persists phenytoin can be given

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 NECROTIZING ENTEROCOLITIS

NECROTIZING ENTEROCOLITIS (NEC)

• NEC is a life-threating condition that primarily affects preterm or SGA babies.
• The condition usually involves the terminal ileum and the colon but may involve
any area from the stomach to the rectum.
• Patients are severity ill, with an acute abdomen.
• NEC should be considered in any preterm infant with abdominal distension
ETIOLOGY AND RISK FACTORS
• Multifactorial
• Prematurity (biggest risk factor)
• Infection and sepsis
• Hypoxia
• Too rapid feeding protocols, feeding regimens and type of feed (hyperosmolar
formula)
• Hypertonic milk
• Excessive fluids
• Polycythemia
• Perinatal asphyxia and hypoxic ischemic encephalopathy
• PDA and indomethacin
PATHOGENESIS
• It is a rare complication due to impaired blood flow to the bowel.
• Mucosal ischemia allows gut microorganisms to penetrate the bowel wall causing
a severe hemorrhagic colitis.
➢ Hypovolemia and hypoxia result in damage within the mucosa cells
(intestinal sloughing) initiating the NEC.
➢ It is often multifactorial in origin resulting in loss of integrity of the gut
mucosal barrier with passage of bacteria into the wall of the bowel and
inflammation that may lead to gangrene and perforation.
➢ Bacterial infection has been implicated in NEC.
➢ Clostridial infections have been associated with some outbreaks of NEC but
the etiology remains unclear.
• It is one of the most common surgical conditions in neonates.

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• It is most frequent in preterm infants with an incidence as high as 8-10% in infants
<30 weeks’ gestation.
• It affects preterm infants in the first few weeks of life.
➢ Usually affects the terminal ileum and colon to a variable extent.
• Preterm infants fed cow’s milk formula are more likely to develop this condition
than if they are fed only breast milk (antibodies in breast milk are protective and
decrease the risk of NEC).
• The picture mimics neonatal sepsis because of the presence of abdominal
distension, apnea, bradycardia, instability of temperature, cyanosis and lethargy.
• Prematurity, respiratory distress syndrome (RDS), hypoxic ischemic
encephalopathy, congenital cardiac malformations, umbilical vessel
Catheterisation, exchange transfusion, hypoglycemia, polycythemia,
postoperative stress and hyperosmolar feeds have all implicated in the etiology.
CLINICAL FEATURES
• Can occur in the first few days of life in term patients, but more commonly from
the second week onward in preterm infants.
• Onset can be insidious but patients with a rapid onset deteriorate quickly.
• Initial symptoms include:
➢ Feeding intolerance
➢ Delayed gastric emptying
➢ Abdominal distension or tenderness or both
➢ Ileus/decreased bowel sounds
➢ Abdominal wall erythema (Advanced stages)
➢ Bloody stools (hematochezia)
➢ Bile stained vomiting and Bilious aspirate
• Systemic signs are non-specific:
➢ Apnea
➢ Lethargy
➢ Decreased peripheral perfusion
➢ Shock (in advanced stages)
➢ Cardiovascular collapse
➢ Bleeding diathesis
• Cardinal signs:
➢ Lethargy
➢ Abdominal distension
➢ Absent bowel sounds

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 ➢ Bloody stools
➢ Vomiting and discolored abdominal wall
• Metabolic acidosis and oliguria may be present and NEC may lead to
thrombocytopenia, disseminated intravascular coagulation and death.
• If the baby has a patent processus vaginalis, free fluid or even gas or meconium
is occasionally seen in the scrotum.
BELL’S CLINICAL STAGING
• Clinical manifestation may be described in 3 stages:
➢ Stage 1 (Suspected NEC): Clinically ill with unstable temperature, apnea,
bradycardia, lethargy, mild abdominal distension, vomiting.
o IA- Infant with suggestive clinical signs but normal abdominal X-ray
o IB- same as IA plus bloody stool (frank or occult)
➢ Stage 2 (Definite NEC): Clinical signs similar to stage 1. Bowel sounds are
diminished with or without abdominal tenderness. Pneumatosis intestinalis
(gas in intestinal wall), portal venous gas and dilation of intestines are seen
on abdominal X-ray.
o IIA- mildly ill infant
o IIB- moderately ill infant
➢ Stage 3 (Advanced NEC): In addition to the above, the infant is severely sick
with hemodynamic instability. There are frank signs of peritonitis with
abdominal wall redness, hypotension, metabolic and respiratory acidosis and
DIC. Pneumoperitoneum may occur due to intestinal perforation. X-ray
findings: pneumatosis intestinalis, portal venous gas or pneumoperitoneum.
o IIIA- critical with impending perforation
o IIIB- critical with proven perforation
DIAGNOSIS
• NEC can progress rapidly from mild abdominal distension to fullness to septic
shock and necrosis of the entire intestine. The management requires high index
of suspicion.
• On examination: palpation may reveal crepitus from the intramural gas, which
can be palpated among the coils of distended loops of bowel and this is an
important sign of NEC.
• Investigations:
➢ Culture of blood, CSF, urine and stool.
➢ U/E & creatinine: low sodium

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 ➢ Full blood count: may show thrombocytopenia, abnormal white count and
low hematocrit
➢ Abdominal X-ray- Anteroposterior and left lateral decubitus.
➢ Blood gases: shows acidosis
• Classic radiological findings:
➢ Distended loops of bowel and thickening of the bowel
wall with intramural gas (Pneumatosis intestinalis).
o Grade I- thickening of the bowel wall +/- dilation of
the gut
o Grade II- bowel wall
o Grade III- gas in the liver and biliary tree
o Grade IV- gas within the peritoneum
➢ Air fluid levels
➢ Venous portal gas
➢ Pneumoperitoneum is suggestive of perforation

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MANAGEMENT
MEDICAL TREATMENT
• Resuscitate: support breathing and intubate if required.
• Assess circulatory status apply fluid and inotropic support judiciously.
➢ Parenteral fluids and nutrition: ¼ Strength Darrows in 10% dextrose.
• Bowel rest, no oral feeds (stop feeds as soon as NEC is suspected)
• Gastric decompression (insert a nasogastric tube and aspirate stomach contents.
Leave it free to drain and monitor output).
• Strict input-output monitoring
• Investigate: AXR, FBC, CRP, blood culture. Consider U&E, Arterial blood
gasses and INR/PT.
• Broad spectrum antibiotics to cover both aerobic and anaerobic organisms,
change antimicrobial treatment according to sensitivity.
➢ Cefotaxime
➢ Metronidazole
• Serial abdominal examinations
• Stage 1:
➢ Reassess abdomen in 24-48 hours.
➢ Restart small volume feeds cautiously if examination is normal
• Stage 2 and 3:
➢ Keep NPO for 5-10 days.
➢ Start total parental nutrition preferably via a deep percutaneous venous line
by 48 hours.
• Patients with advanced disease may require repeat FBC and clotting profile.
• Plasma and platelet transfusion may be necessary to prevent bleeding tendency.
➢ If neonate poorly perfused and HCt>40% give ordinary plasma at 20ml/kg.
➢ If HCT<40% give packed red cells 10-15ml/kg.
• Surgery when indicated.
• DO NOT GIVE INDOMETHACIN TO AN INFANT WITH SUSPECTED OR
DEFINITE NEC.
• Peritoneal drainage can be used in patients who are too unstable for surgery or in
whom ventilation is difficult due to abdominal fluid. Consult with pediatric
surgeon present. Restart feeds slowly (20-30ml/kg/day).
• Continue antibiotics until septic markers normalize.

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• Patients who have had surgery may return to NICU with a stoma. Feeds may be
resumed 5-7 days post-surgery once bowel sounds are present and the stoma is
functioning.
SURGICAL MANAGEMENT
• Indications for surgery:
➢ Increasing peritonitis
➢ Failure to stabilize with medical treatment
➢ Development of an abdominal mass
➢ Persistent loop of free gas on an abdominal X-ray
➢ Persistent metabolic acidosis
• Exploratory laparotomy is indicated for pneumoperitoneum, presence of a fixed
loop on serial radiographs or a positive paracentesis.
• Treatment may include resection of necrotic bowel and possible primary
anastomosis.
• In very low birth weight (under 500g) infants percutaneous drainage has been
used in preference to laparotomy.
➢ This is performed by means of applying a local anesthetic to the right lower
quadrant of the abdominal and then a 10-12 French gauge catheter is inserted
into the abdominal cavity to drain air, meconium and fecal material which
has leaked from perforated bowel.
• The extent of pneumatosis is not proportional to severity of illness and not always
an indication for surgical intervention.
• Portal venous gas is a poor prognostic sign.
PROGNOSIS
• It carries a high mortality especially in more premature infants.
• Patients may present with strictures 3 to 6 weeks post NEC, including those who
have had surgery.
COMPLICATIONS
• Intestinal obstruction (a late complication due to adhesions and strictures)
• Fistulae
• Nutritional deficiencies (e.g. malabsorption, short gut syndrome, growth failure,
malnutrition)
• Cholestasis

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PREVENTIVE MEASURES
• Start small volume feeds to stimulate GIT mucosal development
• Cautious advancement of feeds in small premature infants
• Do not increase feeds if infant has gastric residual of 50% or more
• Fresh breast milk appears protective
DIFFERENTIAL DIAGNOSIS
• Differential diagnosis early in the disease it may be difficult and one should
consider:
➢ Neonatal sepsis
➢ Volvulus neonatorum
➢ Hirschsprung’s enteritis
➢ Infarction of the bowel

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 MECONIUM ASPIRATION SYNDROME

MECONIUM ASPIRATION SYNDROME

• Meconium aspiration syndrome (MAS) is caused by intrauterine/ intrapartum
aspiration of meconium stained amniotic fluid in term or post-term infants.
• Meconium is usually passed 24 hours after delivery in a term baby.
• Aspiration of meconium can occur before, during or after delivery.
• Meconium (first stool) is a material in the fetal gut that consists of:
➢ Water
➢ Mucopolysaccharides
➢ Desquamated skin
➢ Gastrointestinal mucosal epithelial cells, mucus, secretions
➢ Lanugo (hair)
➢ Vernix Caseosa (an oily substance that covers the skin at birth)
➢ Bile salts
➢ Amniotic fluid
• Meconium is sterile and does not contain bacteria which is the primary
differentiating factor from stool.
• Meconium aspiration syndrome is commoner in babies born post-term and in
babies with fetal distress.
• Infants that also become acidotic may inhale thick meconium and develop
meconium aspiration syndrome. (asphyxiated infants may start gasping and
aspirate meconium before delivery)
• Meconium is passed before birth by 8-25% of babies. It is rarely passed by
preterm infants (<37 weeks) and occurs increasingly the greater the gestational
age affecting 20-25% of deliveries by 42 weeks as well as small for gestational
age.
• Acute or chronic hypoxia and/or infection can result in the passage of meconium
in utero. In this setting, gasping by the fetus or newly born infant can cause
aspiration of amniotic fluid contaminated by meconium.
• Factors that facilitate passage of meconium:
➢ Acidosis
➢ Placental insufficiency
➢ Maternal hypertension
➢ Pre-eclampsia
➢ Infection

352
 ➢ Oligohydramnios
➢ Maternal drug abuse (tobacco and cocaine)
• Aspiration of meconium into the tracheobronchial tree causes complete or partial
bronchial obstruction leading to patchy areas of sub-segmental atelectasis and
compensatory areas of hyperinflation.
PATHOPHYSIOLOGY
• In utero meconium passage results from stimulation of a maturing GI tract usually
due to fetal hypoxic stress.
• Meconium is often passed as a consequence of distress (i.e. hypoxia) in the fetus
at term and becomes more frequent after 42 weeks of gestation.
• As the fetus approaches term, the GI tract matures, vagal stimulation from head
or spinal cord compression may cause peristalsis and relaxation of the sphincter
muscles leading to passage of meconium.
• The degree of meconium-stained amniotic fluid varies from slightly green to dark
green and thick consistency (pea-soup).
• The meconium stained amniotic fluid may reach the distal airways and alveoli in
utero if the fetus becomes hypoxic and develops gasping or deep respiratory
movements or may occur at the time of birth with first inspirations.
• Effects of meconium in amniotic fluid:
➢ Meconium directly alters amniotic fluid, reducing antibacterial activity hence
increasing chances of perinatal bacterial infection
➢ Meconium is irritating to fetal skin hence increasing the incidence of
erythema toxicum.
➢ Perinatal aspiration of meconium stained amniotic fluid.
• Aspiration of meconium stained amniotic fluid causes hypoxia via 4 major
mechanisms:
➢ Airway obstruction: complete or partial obstruction. Partial obstruction
causes air trapping and hyperdistention of the alveoli commonly termed the
ball-valve effect. The gas trapped may rupture into pleura (pneumothorax),
mediastinum (pneumomediastinum) or pericardium (pneumopericardium)
➢ Surfactant dysfunction: meconium deactivates and inhibits the production of
surfactant. Constituents of meconium such as free fatty acids have higher
minimal surface tension than surfactant and strip it from alveolar surface
resulting in diffuse atelectasis.

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 ➢ Chemical pneumonitis: enzymes, bile salts and free fatty acids in meconium
irritate the airway and parenchyma. This begins within a few hours of
aspiration
➢ Persistent pulmonary hypertension of the newborn: infants have primary or
secondary persistent pulmonary hypertension of the newborn as a result of
chronic in utero stress and thickening of pulmonary vessels. This causes
hypoxemia and can result in pulmonary infections despite meconium being
sterile.
CLINICAL FEATURES
• Signs and symptoms of mild to moderate respiratory distress in the first few hours
of life that often deteriorates in subsequent 24-48 hours.
➢ Tachypnea
➢ End expiratory grunting
➢ Nasal flaring
➢ Intercostal recessions
➢ Barrel chest
➢ Cyanosis
➢ Auscultation: rales and rhonchi
• Some eventually develop severe respiratory failure with severe hypoxemia and
cyanosis.
• Meconium is a lung irritant that causes chemical pneumonitis.
• Yellow-green staining of fingernails, umbilical cord and skin may be observed.
EVALUATION
• Meconium aspiration syndrome is suggested by a history of meconium noted at
or before delivery and the presence of respiratory distress.
• Diagnosis:
➢ Presence of meconium in amniotic fluid
➢ Neonatal respiratory distress
➢ Characteristic radiological findings
• Chest X-ray shows over-inflated, accompanied by patches of collapse and
consolidation:
➢ Increased lung volume (hyperinflation)
➢ Diffuse asymmetric patchy infiltrates
➢ Areas of consolidation often worse on the right

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 ➢ Pneumothorax (this is due to ball valve effect of meconium depending on the
severity of the clinical picture) or pneumomediastinum may occur in 25% of
patients

Figure 38: Meconium aspiration syndrome. Note hyper-expansion of lungs and heterogenous opacities in
right lung (Image adapted from Essentials of Pediatrics 8th Ed)

MANAGEMENT
• Prevention is of paramount importance.
➢ Initial assessment at delivery complicated by meconium stained amniotic
fluid, determine whether the infant is vigorous as demonstrated by heart rate
>100 b/min, spontaneous respiration and good tone.
➢ If vigorous routine care should be provided regardless of consistency of
meconium.
➢ If respiratory distress develops or depressed infant, tracheal intubation under
direct laryngoscopy and intratracheal suctioning.
• Best approach to meconium stained amniotic fluid is combined obstetric and
pediatric approach and may include:
➢ Minimal handling to prevent agitation (may worsen the condition and
predispose to pulmonary vasoconstriction and PPHN)
➢ Oropharyngeal and nasopharyngeal suctioning on the perineum (though not
effective in preventing MAS)
➢ Direct suctioning of the tracheal via endotracheal intubation
➢ If the baby is born in a good condition and is crying no suction is needed.
However, if the baby is in a poor condition or not making any respiratory
effort the oropharynx and the vocal cords should be suctioned under direct
vision before starting intermittent positive pressure ventilation.

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➢ Manage hypoxemia: Generally, oxygen is required (to prevent persistent
pulmonary hypertension of the newborn) and if the meconium aspiration
syndrome is severe, CPAP, mechanical ventilation or ECMO (extracorporeal
membrane oxygenation) may be necessary.
o Assisted ventilation (CPAP): consider when saturation is less than 90%,
if FiO2 (fraction of inspired oxygen) >0.41 however, CPAP may
sometimes aggravate air trapping and should be instituted with caution
if hyperinflation is apparent clinically or radiologically.
o Mechanical ventilation:
▪ Required if excessive CO2 retention (PaCO2>60mmHg) or
persistent hypoxemia (PaO2<50mmHg).
▪ Infants requiring higher inspiratory pressures (30-35cm H2O), PEEP
(3 to 6 cm H2O) should be selected based on response. Adequate
expiratory time should be permitted to prevent air trapping behind
partially obstructed airway.
▪ Useful starting points: inspiratory time of 0.4 to 0.5 seconds at a rate
of 20 to 25 b/min adjust as required.
▪ HFOV (high frequency oscillatory ventilation) if not coping on
conventional ventilation and in those who develop air-leak
syndromes.
➢ Correct hypoglycemia
➢ Restrict fluids to avoid cerebral and pulmonary edema.
➢ Monitor:
o Oxygen saturation
o Renal function: Electrolytes particularly calcium and monitor pH (likely
metabolic acidosis)
o Blood pressure- may require inotropes
o Blood gasses and pH
o Hemoglobin (maintain Hb >15mg/dl improves oxygenation)
➢ It is acceptable practice to start antibiotic after obtaining blood culture.
o It is difficult to distinguish MAS from bacterial pneumonia. Use broad
spectrum antibiotics (e.g. Penicillin and gentamicin) guided by blood
culture
➢ Surfactant can be given because endogenous surfactant can be inhibited by
meconium. This however should not be routinely used but may benefit
infants that are clinically deteriorating and require escalating support.
➢ Sedatives may be warranted if requiring mechanical ventilation.

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COMPLICATIONS
• Air leak: in 15 to 33%. It is common with mechanical ventilation, especially in
the setting of air trapping.
➢ Pneumothorax
➢ Pneumomediastinum
• Persistent pulmonary hypertension of the newborn (PPHN).
• Pulmonary sequelae:
➢ Chemical pneumonitis which is complicated by bronchopneumonia.
➢ Long term reactive airway disease

NEONATAL JAUNDICE

NEONATAL JAUNDICE
• This is yellowish discoloration of mucous membranes and skin as a result of
increased bilirubin levels.
• It usually occurs during the first week of life and is most frequently caused by
indirect (unconjugated) hyperbilirubinemia that is physiologic in nature.
• Visible jaundice occurs in the neonate when serum bilirubin levels exceed 5mg/dl
(85 micromol/dl).
• Neonatal jaundice is important as:
➢ It may be a sign of another disorder e.g. hemolytic anemia, infection,
metabolic disease, liver disease
➢ Unconjugated bilirubin can deposit in the brain, particularly the basal ganglia
causing kernicterus (bilirubin encephalopathy- choreaoathetoid cerebral
palsy) as well auditory nucleus (deafness).

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CLASSIFICATION OF JAUNDICE
PHYSIOLOGIC JAUNDICE
• This is benign and self-limited indirect hyperbilirubinemia that typically resolves
by the end of the first week of life and requires no treatment.
• Visible jaundice usually appears between 24-72 hours of age.
• It increases in severity until day 4-5 and gradually falls and disappears by day 10.
• Over 50% of all newborn infants become visibly jaundiced because:
➢ There is marked physiological release of hemoglobin from the breakdown of
red blood cells because of high Hb concentration at birth. (high red cell mass
at birth due to the relatively hypoxic environment)
➢ The red cell life span of a newborn infant (70-80 days) is markedly shorter
than that of an adult (120 days)
➢ Hepatic bilirubin metabolism is less efficient in the first few days. (Immature
liver conjugation system- Delayed activity of the hepatic enzyme glucuronyl
transferase, Low concentration of the binding protein ligandin in the
hepatocytes)
➢ Increased enterohepatic circulation due to sterile gut.
• Clinical features:
➢ Jaundice
➢ Elevated indirect bilirubin levels:
o Peak serum concentrations in normal full-term infant reaches 5-16 mg/dl
at around 3-4 days of life.
o In preterm infants, the peak bilirubin is reached after 5-7 days and may
take 10-20 days before decreasing.
• Physiologic jaundice is a diagnosis of exclusion therefore every jaundice baby
should be carefully evaluated to rule out pathological causes.
NONPHYSIOLOGIC JAUNDICE
• This is jaundice that is secondary to a pathophysiologic cause and it may be
classified as:
➢ Indirect hyperbilirubinemia: this is an elevated bilirubin in which the
conjugated or direct component is <20% of the total bilirubin.
➢ Direct hyperbilirubinemia: conjugated or direct bilirubin is >20% of total
bilirubin level. This is always pathologic in neonates.
• There is no clear cut demarcation between pathological and physiological
jaundice.

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• Total serum bilirubin (TSB) have been arbitrarily defined as pathological if it
exceeds 5mg/dl on first day, 10mg/dl on second day or 15mg/dl thereafter in term
babies.
• If the jaundice appears on the first day of life even in preterm babies, it is deemed
pathological and severe enough to require intervention.
• The same applies if it persists beyond the usual period, it needs investigation and
treatment.
• Early jaundice is generally secondary to hemolysis although it may be secondary
to infection, extensive bruising and concealed hemorrhage.
• Hemolysis may be secondary to antibodies, or defects in the red cells structure or
enzymes.
• Early jaundice, secondary to hemolysis from incompatible blood group like Rh
isoimmunization is a major cause of hemolytic jaundice although the incidence
is declining with anti-D prophylaxis in Rh negative mothers.
• The jaundice secondary to ABO isoimmunization is not severe enough to require
exchange blood transfusion. Other minor blood group isoimmunization can also
produce neonatal jaundice.
• The red cell defects such as hereditary spherocytosis, glucose-6-phosphate
dehydrogenase deficiency, pyruvate kinase deficiency are not common but
important particularly when there is no obvious cause to explain the presence of
jaundice or hemolysis.
DIFFERENTIAL DIAGNOSIS
INDIRECT HYPERBILIRUBINEMIA
• Possible diagnosis includes:
➢ Physiologic jaundice
➢ Increased RBC load from bruising: Cephalohematoma, Caput succedaneum,
birth trauma
➢ Excessive bilirubin production from hemolysis: spherocytosis, elliptocytosis,
pyruvate kinase deficiency, glucose 6 phosphate dehydrogenase deficiency,
ABO-Rh incompatibility.
➢ Defective conjugation of bilirubin by the liver
o Crigler-Najar syndrome (Deficiency of glucuronyl transferase)
▪ Crigler-Najar Type 1: very severe and affected individuals can die
in childhood due to kernicterus. This is an absolute deficiency of
glucoronyl transferase

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 ▪ Crigler-Najar Type 2: This is less severe and due to a relative
deficiency of glucoronyl transferase
o Gilbert’s syndrome:
➢ Breastfeeding jaundice
o Typically occurs during the first week of life with increased bilirubin
levels and is usually related to suboptimal milk intake. Poor intake leads
to weight loss, dehydration and decreased passage of stool, with
resultant decreased excretion of bilirubin in the stool.
➢ Breast milk jaundice
o Typically occurs after the first week of life and is likely related to breast
milk’s high levels of beta-glucuronidase and high lipase content.
Elevated bilirubin is highest in the 2nd and 3rd weeks of life and lower
levels of bilirubin may persist until 10 weeks of life.
➢ Inborn errors of metabolism: hypothyroidism
➢ Upper GI obstruction (increased enterohepatic recirculation): pyloric
stenosis, duodenal stenosis, annular pancreas
DIRECT HYPERBILIRUBINEMIA
• Possible diagnosis includes:
➢ Obstruction of the hepatobiliary tree: choledochal cysts
➢ Obstructive jaundice secondary to biliary atresia
➢ Neonatal infection: sepsis
➢ Neonatal hepatitis: HAV, HBV, HCV, EBV, TORCH infections, Varicella,
Herpes, Tuberculosis
o TORCH= Toxoplasmosis, Other (syphilis), rubella, cytomegalovirus,
herpes simplex virus
➢ Cystic fibrosis
➢ Cholestasis associated with parenteral nutrition
➢ Metabolic disorders:
o Galactosemia
o Hereditary fructose intolerance
o Tyrosinemia
o Alpha 1 antitrypsin deficiency

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 361

Figure 39: Differential Diagnosis of Indirect and Direct Hyperbilirubinemia (Image courtesy of BRS
Pediatrics-2005)

METABOLISM OF BILIRUBIN
• Bilirubin is as a result of hemolysis of RBCs.
• Recall, RBCs have the pigment hemoglobin which consists of Heme (with iron)
and the globin part.
• RBCs after 120 days (70 days in infants) are broken down in the
reticuloendothelial system (spleen) by macrophages.
• Hemoglobin is broken down to the heme part and the globin part.
➢ The globin part is broken down to amino acids that are recycled.
➢ The heme part is broken down by heme oxygenase to oxy-heme (a closed
tetrapyrrolic ring with iron)
➢ Oxy-heme is further converted by heme reductase to biliverdin (an open
tetrapyrrolic ring without iron)
➢ The iron removed from the heme binds to transferrin and is transported to
the liver to be stored where it binds to ferritin.
➢ Biliverdin is converted by biliverdin reductase to bilirubin
• Bilirubin is toxic to tissue therefore; it is transported to the liver as it binds to
albumin.
• Bilirubin is taken up by hepatocytes at their sinusoidal surface. This uptake is
very rapid.
• In the hepatocytes bilirubin is bound to cytoplasmic proteins: ligandins and Z
protein.
➢ Ligandins are a group of enzymes that represent 2% of cytosolic proteins
➢ Z proteins bind fatty acids.
➢ The primary function of these proteins is that of avoiding the reflux of free
bilirubin into the blood because the time lapse between uptake of bilirubin
and conjugation is relatively long.
➢ Ligandin concentrations are low at birth but rapidly increase over the first
few weeks of life. Ligandin concentrations may be increased by the
administration of pharmacologic agents such as phenobarbital.
• In the hepatocytes bilirubin is conjugated as it is bound to glucuronic acid.
Conjugated bilirubin is also called Direct bilirubin, while unconjugated bilirubin
is called indirect bilirubin. The enzyme important for glucuronidation is
Glucuronyl transferase.
• Conjugated bilirubin is then secreted into bile which is stored and concentrated
in the gall bladder.
• Bile is then secreted into the first part of the duodenum from the common bile
duct.
• In the intestines bilirubin is deconjugated by bacterial/intestinal enzyme Beta-
glucuronidase to form free bilirubin.
• Bilirubin is converted by bacterial dehydrogenase to urobilinogen (a colorless
substance)
• The bulk of urobilinogen, bilirubin and urobilin is excreted in feces (by
conversion to stercobilinogen and stercobilin which gives feces their
characteristic orange-yellow color).
• Small amounts of bilirubin and urobilinogen are reabsorbed into the blood
stream.
• The reabsorbed urobilinogen is excreted in the urine (about 4mg/day).
• Urobilinogen is converted to urobilin (orange-yellow pigment) by
dehydrogenase
EVALUATION OF HYPERBILIRUBINEMIA
• Jaundice should always be evaluated under the following circumstances:
➢ Jaundice appears at <24 hours of age
➢ Bilirubin rises >5-8mg/dl in a 24-hour period
➢ The rate of rise of bilirubin exceeds 0.5mg/dl per hour (suggestive of
hemolysis)

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KRAMER STAGING
• This is a clinical assessment of jaundice.
• Progression is cephalocaudal.
• The body is divided into 5 zones:
➢ Zone 1: 6mgl/dl
➢ Zone 2: 9mg/dl
➢ Zone 3: 12mg/dl
➢ Zone 4: 15mg/dl
➢ Zone 5: >15mg/dl

• To change mmol/dl multiply by 17.

INVESTIGATIONS
• To evaluate indirect hyperbilirubinemia:
➢ Full blood count
➢ Reticulocyte count
➢ Smear (for hemolysis)
➢ Evaluation of sepsis may be indicated
• To evaluated direct bilirubinemia:
➢ Hepatic ultrasound (to evaluate for choledochal cyst)
➢ Serologies: viral hepatitis
➢ Radioisotope scan of the hepatobiliary tree
➢ Evaluation of sepsis may be indicated.
MANAGEMENT
• Treatment of jaundice is based on the gestational age of the baby and the postnatal
age.

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• Babies who are preterm should be treated at a lower serum bilirubin level than a
baby born at term.
• Several other factors such as well-being of child, severity of hemolysis and
presence of sepsis should be taken into consideration in determining the threshold
for treatment.
• Serum bilirubin assessments, observation and reassurance are appropriate for
physiologic jaundice.
• Phototherapy and exchange transfusion are the mainstay of treatment.
• Phototherapy (with blue light, wave length 450-500 nm): very effective and acts
by:
➢ Photo-isomerisaton: creates water-soluble photoisomers of indirect bilirubin:
o Polar configurational isomers (Z, E- bilirubin)
o Structural isomers (lumirubin)
➢ Photo-oxidation
• During phototherapy ensure the eyes are covered to protect them from exposure
to light
➢ Side effects: photodermatitis, temperature instability, loose stools and
dehydration
• Exchange transfusion is performed for rapidly rising bilirubin levels secondary
to hemolytic disease.
➢ The aim is to remove antibody coated red cells, antibodies in serum, reduce
and correct anemia.
➢ The estimated blood volume of a term neonate is 85ml/kg while that of a
preterm neonate is about 100ml/kg
• Adequate hydration is important as dehydration will increase the serum bilirubin
level.
COMPLICATIONS
• Kernicterus (bilirubin encephalopathy) is the main complications.
• Mainly the basal ganglia and auditory nucleus are affected, but any part of the
brain is at risk.
• Indirect bilirubin at sufficiently high concentrations can pass through the blood-
brain barrier and produce irreversible damage.
• Bilirubin most frequently localizes in the basal ganglia, hippocampus and some
brainstem nuclei.
• This occurs when the level of unconjugated bilirubin exceeds the albumin
binding capacity of bilirubin of the blood.

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• Clinical features include
➢ Lethargy and poor feeding (acute manifestation)
➢ In severe cases there is irritability, increased muscle tone causing the baby to
lie with an arched back (opisthotonos)
➢ Choreoathetoid cerebral palsy: due to damage to the basal ganglia
➢ Hearing loss: sensorineural deafness
➢ Opisthotonus
➢ Seizures
➢ Oculomotor paralysis

Figure 40: Summary of causes of neonatal jaundice (Image adapted from Pediatrics at a Glance)

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Figure 41:Approach to Jaundiced Pediatric patient (Image adapted from Pediatrics at a Glance)

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CONGENITAL INFECTIONS
• Congenital and perinatal infections are often referred to by the acronym TORCH.
➢ T- toxoplasmosis
➢ O- others (syphilis, malaria, tuberculosis, HIV, HCV, HBV, varicella,
enterovirus and parvovirus)
➢ R- rubella
➢ C- cytomegalovirus
➢ H- herpes simplex virus (HSV)
• Fetal and neonatal infections occur only with primary infection in the mother.
• Latent infection or reactivation affects the baby very infrequently with the
exception of syphilis.
• Not all infections in mother are transmitted to the baby due to the placental barrier
and not all infected babies are affected.
• Transmissibility and severity of fetal affection depends on the timing of gestation.
Infection during the first trimester has the most devastating consequences.
• Congenital and prenatal infections can manifest during pregnancy as
ultrasonographic findings soon after birth or later in life.
• Common manifestations of intrauterine infections:
➢ Abortions (Recurrent only with syphilis)
➢ Intrauterine growth retardation
➢ Intrauterine death
➢ Prematurity
➢ Deafness
➢ Chorioretinitis
➢ Aseptic meningitis
➢ Microcephaly and mental retardation
➢ Lymphadenopathy
➢ Hepatosplenomegaly
➢ Neonatal hepatitis
➢ Anemia
➢ Thrombocytopenia
➢ Skeletal abnormalities/bony changes
➢ Skin rash
➢ Jaundice (conjugated)
➢ Petechiae
➢ Hydrops (non-immune)

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• Note: Growth restriction alone with no other signs is not an indication for
investigation for congenital infection.
• Also try to get the placenta sent for histology as this will often assist greatly in
making the diagnosis.
• Tests useful in diagnosis include:
➢ Serology and TORCH screen: IgM and IgG estimation should be done in
both the baby and the mother
➢ Full blood count
➢ Liver function tests
➢ Renal function tests
➢ Skeletal survey
➢ Fundus examination
➢ Hearing evaluation
➢ Imaging of the CNS
• Treatment is possible and rewarding for toxoplasmosis, syphilis and herpes
simplex only partly successful for CMV and not available for rubella.

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CONGENITAL TOXOPLASMOSIS
• The maternal primary infection is generally subclinical.
• In HIV infected or immunocompromised women, reactivation of latent infection
may also affect the fetus.
• Transmisibility increase but the risk of fetal disease decreases with advancing
pregnancy.
• Clinical manifestation (those mentioned earlier on)
➢ Generalized Intracranial cranial calcification
➢ Hydrocephalus
➢ Chorioretinitis
• Infants asymptomatic at birth may later present with mental retardation and
deafness
• Diagnosis:
➢ Positive toxoplasma IgM in serum of affected child
➢ PCR can also be done
• Treatment (for all affected babies even if asymptomatic):
➢ Pyrimethamine, sulfadiazine and folinic acid (leucovorin) for a period of 1
year.
➢ Prednisone is added if there is CNS or eye involvement
➢ Liaise with infectious disease team if you are considering treatment
• Prevention: advising pregnant women to wash fruits and vegetables carefully,
limit contact with soil and refrain from eating undercooked meat since infection
results from ingestion of food or water contaminated with oocytes or tachyzoites
in infected meat.
CONGENITAL RUBULLA
• Transmisibility is highest in the first trimester and so is the rate of fetal disease
(90% at <11 weeks).
• The fetus is completely spared if infection occurs beyond 16 weeks.
• Clinical manifestations (as mentioned before):
➢ Cataract
➢ Congenital heart disease (PDA)
➢ Deafness
• Delayed manifestations:
➢ Diabetes mellitus
➢ Renal disease

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• Diagnosis: positive rubella IgM in cord or neonatal blood
• No treatment exists. Ophthalmology and hearing should be check.
Neurodevelopmental follow-up is essential.
• An unequivocal diagnosis of rubella in the first trimester of pregnancy is an
indication for maternal termination of pregnancy. (Send urine for rubella PCR)
• Vaccinating all children and particularly all adolescent girls against rubella is
strongly recommended to reduce the burden of congenital rubella.
• Since this is a notifiable disease, fill out form for notifiable disease and sent to
UTH virology laboratory.
CONGENITAL CYTOMEGALOVIRUS (CMV) INFECTION
• This is the most common congenital infection in developed countries, incidence
is unknown in developing countries.
• Most fetal infections occur with primary infection in the mother, transmission
and fetal disease occur throughout pregnancy.
• Overall transmission rate with primary infection is 30% and 10% of all infected
babies are symptomatic.
• CMV remains latent in the body and can reactivate any time, similarly
reinfections can also occur. Transmission risk with reactivation or reinfection is
only 1% and only 5-10% of infected babies are symptomatic.
• Manifestations:
➢ Can affect all organ systems
➢ Characteristic features:
o Periventricular calcifications
o Microcephaly
• CMV transmission can also occur during delivery and breastfeeding and is of
consequence only in the preterm and very low birthweight babies. In this setting
it presents as sepsis like illness with pneumonia and respiratory distress.
• CMV transmission due to blood and blood products can also cause anemia,
thrombocytopenia and hepatosplenomegaly in preterm infants.
• Diagnosis:
➢ Send urine for CMV PCR or shell vial
➢ Confirmed by Positive IgM in cord blood or in the first 2 weeks of life
(sensitivity is low and a negative IgM does not rule out CMV).
• A positive CMV IgM after the first 2 weeks of life can also occur due to postnatal
transmission and is not an accurate method for diagnosis.

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• In babies older than 2 weeks, a positive quantitative CMV PCR is the method of
choice.
• Treatment: Antiviral treatment with ganciclovir
or valganciclovir (indicated only in patients
with progressive disease and deafness).
➢ Side effects: neutropenia
• The optimal length of treatment is under study
but at least 6 weeks should be given.
• Ophthalmology review and audiology should be
done.
• Neurodevelopment follow up is needed.
PERINATAL HERPES SIMPLEX
VIRUS (HSV)
• Transmission of HSV to babies usually occurs Figure 42: Classical periventricular
calcification of CMV
in mothers who develop primary genital herpes
at the time of delivery.
• Reactivation of genital herpes is associated with very low rates of transmission
and fetal affection.
• Infected babies may be asymptomatic or have fulminant disease.
• Disease usually develops 3-10 days after delivery and is more common in the
premature infant.
• 3 forms of disease have been described:
➢ Vesicular eruption limited to skin, eyes, and mouth.
➢ CNS disease presenting as meningitis/encephalitis with seizures and altered
sensorium
➢ Disseminated disease presenting as sepsis-like illness with high mortality
• The latter two may not have associated skin eruption which further complicates
diagnosis.
• Only 50% of infants will have any skin lesions and the infant may look as though
they have bacterial sepsis. Fever and aseptic meningitis should increase your
suspicion of herpes.
• Diagnosis: Tzanck smear of skin lesions, culture or PCR for the virus from
lesions or from CSF.
• HSV serology has no role in making diagnosis.

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• Treatment: High dose intravenous acyclovir (20mg/kg TDS) for 2 weeks (in
vesicular eruption) and 3 weeks in other forms in neonates with suspected or
confirmed infection
• Babies born to mothers with active herpetic lesions during delivery should be
watched carefully for disease.
• Elective C-section should be considered in mothers with active primary genital
herpes and unruptured membranes.
CONGENITAL SYPHILIS (CONGENITAL LOUIS)
• This is the most common congenital infection in our setting, it is probably worth
checking for this first.
• Syphilis is the only maternal infection that is associated with recurrent abortion
hence, the TORCH screen should include VDRL.
• Maternal syphilis can be transmitted throughout pregnancy, more commonly
during later pregnancy as the placenta thins down.
• Even if the mother was RPR/VDRL negative earlier in pregnancy, this does not
exclude congenital syphilis- the test only becomes positive 2 months after
infection and with the rapid test we likely to see more false negative tests.
• Apart from the clinical features mentioned earlier babies have other pathognomic
features like:
➢ Skeletal lesions
➢ Snuffles
➢ Pneumonia alba and bullous skin lesion
➢ Rash on palms and soles
➢ Hepatosplenomegaly
➢ Growth restriction
➢ Delayed features
o Depressed nasal bridge (saddle nose)
o Notched central incisors (Hutchinson teeth)
o Keratitis
o Saber shins
o Frontal bossing
• Diagnosis: quantitative VDRL estimation.
• False positive results (usually low titer <1:16) can occur with EBV, VZV,
hepatitis, measles, TB, SLE, lymphoma, mycoplasma or protozoal infection,
connective tissue disease and pregnancy itself.

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• False negative results in rapid tests can be caused when the antibody titer is very
high (prozone effect). Laboratory testing can include dilution in suspicious cases.
• Specific treponemal tests such as TPHA are of little use when testing
• There is no need for routine lumbar puncture.
• Congenital syphilis is a notifiable disease. Contacts should be treated.
• All fully treated infants should have their VDRL/RPR rechecked at 3 months to
ensure the titer is decreasing.
• Treatment:
➢ Benzyl penicillin 50 000 U/kg/dose BD for 10 days
o It should be continued even when other antibiotics are used. If the course
is interrupted by more than 24 hours it should be restarted from the
beginning.
➢ Ceftriaxone should be used if Benzyl penicillin is not available.

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 CHAPTER 16: MISCELLANEOUS

MISCELLANEOUS
FAILURE TO THRIVE ➢ Organic etiologies suggest
underlying organ system
• This is a term used to describe a
pathology, infection,
growth rate of less than expected
chromosomal disorders or
for a child less than 5 years.
systemic illness.
• Failure to thrive usually refers to
weight below 3rd or 5th centile or a INORGANIC CAUSES
failure to gain weight over a period • Poor formula preparation (feeding
of time or a change in rate of diluted formulae)
growth that has crossed 2 major • Poor feeding techniques
centiles e.g. 75th to 50th over a (misperception or lack of
period of time. knowledge about diet and feeding)
• Failure to thrive is a descriptive • Child abuse and neglect
term rather than diagnosis. • Parental immaturity
• FTT should be distinguished from • Maternal depression
isolated short stature, in which • Alcohol and drug use
height is the most abnormal growth • Marital discord
parameter.
• Mental illness
• In children with FTT, weight is
• Family violence
usually affected before length,
• Poverty
which is usually affected before
• Isolation from support system
head circumference (head
circumference is initially spared in ORGANIC CAUSES
FTT). • Neurological:
ETIOLOGY ➢ Cerebral palsy
➢ Mental retardation
• Causes include both:
➢ Hydrocephalus
➢ Inorganic or psychosocial
➢ Intracranial tumors
etiologies (Most common
➢ Generalized muscle weakness
cause-80%) i.e. a disturbed
➢ Increased or decreased tone
parent-child bond that results in
inadequate caloric intake/ • Cardiac: congenital heart disease
retention or emotional • Pulmonary
deprivation. ➢ Bronchopulmonary dysplasia

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 ➢ Cystic fibrosis ➢ Fanconi syndrome
➢ Asthma • Hematology and oncology
• Gastrointestinal: ➢ Iron-deficiency anemia
➢ Craniofacial problems ➢ Malignancy
➢ Tracheoesophageal fistula • Endocrine: hypothyroidism,
➢ Gastroesophageal reflux diabetes mellitus & rickets
disease • Genetic, congenital and metabolic
➢ GI obstruction (pyloric ➢ Fetal alcohol syndrome
stenosis, malrotation, ➢ Inborn errors of metabolism
Hirschprung’s disease) ➢ Chromosomal abnormalities
➢ Acute and chronic diarrhea • Immunologic- immunodeficiency
➢ Inflammatory bowel disease status
➢ Celiac disease • Infectious:
➢ Cystic fibrosis ➢ Tuberculosis
• Renal ➢ HIV
➢ Chronic renal failure ➢ Hepatitis
➢ Renal tubular acidosis • Toxin: lead poisoning
➢ Recurrent urinary tract
infection

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 376

CLINICAL FEATURES • The degree of FTT is usually

• These children present with poor measured by calculating weight,
growth, often associated with poor height and weight for height as
development and cognitive percentage of the median value for
functioning. age based on appropriate growth
charts
Degree of FTT Weight-for-Age Length/height-for-age Weight-for-height
(% of median) (% of median) (% of median)
Mild 75-90 90-95 81-90
Moderate 60-74 85-89 70-80
Severe <60 <85 <70

EVALUATION
• Evaluation of FTT requires:
➢ Full history with a complete
dietary history
o Food diary over several
days
o Details of exactly what
happens at mealtimes
o Is the child well with lots
of energy or does the child
have other symptoms such
as diarrhea, vomiting,
cough, or lethargy?
o Was the child premature
or had intrauterine growth
restriction at birth or any
significant medical
problems?
o The growth of other
family members and any
illnesses in the family
o Is the child’s development
normal?
o Are there psychosocial
problems at home?
➢ Physical examination
➢ Observation of parent-child
interaction
• Routine screening tests are usually
not useful and laboratory
evaluation should therefore be
focused and directed by clues from
the history and physical
examination.
• For initial evaluation the following
investigations are adequate:
➢ Full blood count with ESR
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➢ Urine and stool microscope and
culture
➢ Renal and liver function test
and serum electrolytes
• Evaluation of potential organic
etiologies will be directed by the
timing or onset of FTT i.e. parental
onset of inadequate weight gain (as
in intrauterine growth retardation)
should be distinguished from
postnatal onset of inadequate
weight gain.
• Weight gain in response to
adequate calorie feeding
establishes the diagnosis of
psychosocial FTT.
MANAGEMENT
• Goals of management:
➢ Nutritional rehabilitation
➢ Treatment of organic causes if
present
➢ Remedial measure of
psychosocial factors.
• Indications for hospitalization:
➢ Severe malnutrition
➢ Diagnostic and laboratory
evaluation needed for organic
cause
➢ Lack of catch up growth during
outpatient treatment
➢ Suspected child abuse or
neglect.
• Management of these patients
depends on the underlying causes.
PROGNOSIS development is variable and less
• If managed early and adequately certain.
prognosis for physical growth • The growth and development of
recovery is good. these children should be monitored
• However, the outlook for cognitive, regularly.
emotional and behavioral

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379
 PEDIATRIC SHOCK

PEDIATRIC SHOCK
• Shock is a clinical state characterized by inadequate delivery of oxygen and
metabolic substrate to meet the metabolic demands of tissue.
• It may present with normal or decreased blood pressure.
CLASSFICIATION
• It may be classified by:
➢ Degree of compensation
➢ Etiology
CLASSIFICATION BY DECOMPENSATION
• Shock may be:
➢ Compensated
➢ Decompensated
➢ Irreversible
COMPENSATED
• Characterized by normal blood pressure and cardiac output with adequate tissue
perfusion but maldistributed blood flow to essential organs.
DECOMPENSATED
• Characterized by hypotension, low cardiac output and inadequate tissue
perfusion.
IRREVERSIBLE SHOCK
• Characterized by cell death and is refractory to medical treatment

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ETIOLOGY
HYPOVOLEMIC SHOCK
• This is the most common cause of shock in children and is caused by any
condition that results in decreased circulating blood such as hemorrhage or
dehydration e.g. from acute gastroenteritis
• The amount of volume loss determines the success of compensatory mechanisms
such as endogenous catecholamines, in maintaining blood pressure and cardiac
output.
• Volume losses greater than 24% result in decompensated shock.
CARDIOGENIC SHOCK
• Occurs when cardiac output is limited because of primary cardiac dysfunction.
• Causes include:
➢ Dysrhythmias e.g. supraventricular tachycardia
➢ Congenital heart disease e.g. any lesion that impairs left ventricular outflow
➢ Cardiac dysfunction after cardiac surgery
• Clinical features are the signs and symptoms of congestive heart failure.
DISTRIBUTIVE SHOCK
• Associated with distal pooling of blood or fluid extravasation and is typically
caused by anaphylactic or neurogenic shock as a result of medications or toxins.
• Anaphylactic shock: characterized by acute angioedema of the upper airway,
bronchospasm, pulmonary edema, urticarial, and hypotension because of
extravasation of intravascular fluid from permeable capillaries.
• Neurogenic shock typically secondary to spinal cord transection or injury is
characterized by a total loss of distal sympathetic cardiovascular tone with
hypotension resulting from pooling of blood within the vascular bed.
SEPTIC SHOCK
• This is secondary to an inflammatory response to invading microorganisms and
their toxins and results in abnormal blood distribution.
• There are 2 clinical stages:
➢ Hyperdynamic stage: characterized by normal or high cardiac output with
bounding pulses, warm extremities and a wide pulse pressure.
➢ Decompensated stage: follows the hyperdynamic stage if aggressive
treatment has not been initiated. It is characterized clinically by impaired
mental status, cool extremities and diminished pulses.

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DIAGNOSIS
• Recognition of shock may be difficult because of the presence of compensatory
mechanisms that may prevent hypotension until 25% of intravascular volume is
lost.
• The index of suspicion for shock must be high
• History:
➢ Severe vomiting and diarrhea
➢ Trauma with hemorrhage
➢ Febrile illness, especially in an immunocompromised patient
➢ Symptoms of CHF
➢ Exposure to a known allergic antigen
➢ Spinal cord injury
• Physical examination:
➢ Blood pressure may be normal in the initial stages of hypovolemic and septic
shock.
➢ Tachycardia almost always accompanies shock and occurs before blood
pressure changes in children.
➢ Tachypnea may be present as a compensatory mechanism for severe
metabolic acidosis.
➢ Mental status changes may indicate poor cerebral perfusion.
➢ Capillary refill may be prolonged, cool and mottled extremities.
➢ Peripheral pulses may be bounding in early septic shock
LABORATORY STUDIES
• Blood investigations
➢ Full blood count: to assess for blood loss and infection.
➢ Electrolytes: to assess electrolyte abnormalities including calcium.
➢ Blood urea nitrogen and creatinine: to evaluate renal function
➢ Glucose: assess for frequently encountered metabolic derangements
➢ Coagulation factors: to evaluate for DIC which may accompany shock
➢ Arterial blood gasses: to evaluate metabolic acidosis
• Toxicology screen: to evaluate for a poisoning which could cause shock.

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MANAGEMENT
• Initial resuscitation involves the ABCs:
➢ Stabilize the airway. Early endotracheal intubation to secure the airway and
decrease the patient’s energy expenditure
➢ Supplemental oxygen provided by mask or nasal catheter
➢ Vascular access with two large bore cannulae. If peripheral access is not
readily available, intra-osseous access should be established and appropriate
fluid resuscitation started.
➢ Disability (neurological examination)
➢ Exposure
• To restore intravascular volume, intravenous crystalloid or colloid solution
should generally be used before administration of inotropic and vasopressor
agents.
• Inotropic and vasopressor medications e.g. dobutamine, dopamine, epinephrine
are indicated if the blood pressure increase in response to fluid is inadequate.
➢ Dopamine 3 to 20 mcg/kg/min
➢ Dobutamine 1 to 20 mcg/kg/min
➢ Epinephrine 0.01 to 1.0 mcg/kg/min
➢ Norepinephrine 0.01 to 1.0 mcg/kg/min
• Metabolic derangements such as metabolic acidosis, hypocalcemia or
hypoglycemia should be treated.
• Other considerations:
➢ Broad-spectrum antibiotics for septic shock
➢ Blood products for hemorrhage
➢ Fresh frozen plasma for DIC
DRUG INFUSION PREPARATION INFUSION RATE
Dopamine Body weight in kg x 6= amount of 1ml/hr= 1mcg/kg per min
Dobutamine drug (mg) to be added to total (example: to deliver 10mcg/kg
volume of 100 ml IV fluid per minute run infusion at
10ml/h)
Epinephrine Body weight in kg x 0.6= amount 1ml/hr= 0.1 mcg/kg/min
Norepinephrine of drug (mg) to be added to total (Example: to deliver 0.3 mcg/kg
Milrinone volume of 100ml IV fluid per minute run infusion at 3
ml/hr)

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TYPE OF SHOCK CLINICAL FEATURES MANAGEMENT
Hypovolemic • Increased HR • 20 ml boluses of 20ml/kg normal saline or
(reduced cardiac • Rapid pulses ringer’s lactate as indicated up to 60ml/kg
output and increase • Delayed CRT in 1 hour in non-malnourished child
systemic vascular • Tachypnea • 15ml/kg half strength Darrows in 5% or
resistance) • Dry skin ringer’s lactate with 5% dextrose dextrose
• Sunken eyes in 1 hour in malnourished
• Oliguria • Blood product as indicated for acute
blood loss
Septic • Increased HR • 20 ml boluses of 20ml/kg normal saline or
(increased cardiac • Normal or reduced BP ringer’s lactate as indicated up to 60ml/kg
output and increased • Reduced pulses in 1 hour in non-malnourished child
systemic vascular • Delayed CRT • For malnourished children repeat
resistance) • Tachypnea 15mk/kg of half strength Darrow’s in 5%
• Mental state changes dextrose or Ringer’s lactate in 5%
• Third spacing dextrose in the next hour. If no
• Edema improvement, give maintenance IV
4ml/kg/hr and start inotropes (dopamine)
• Consider colloid if poor response to
crystalloids
• Broad spectrum antibiotics
Distributive Angioedema, rapid third • Repeat boluses of 20ml/kg crystalloids as
Anaphylaxis spacing of fluids, reduced indicated
(increased cardiac BP, respiratory distress • Pharmacological support of systemic
output, reduced vascular resistance with norepinephrine
systemic vascular or phenylephrine
resistance)
Distributive Reduced BP with normal • Pharmacological support of systemic
Spinal cord injury HR, paralysis with loss of vascular resistance with norepinephrine
(normal cardiac vascular tone or phenylephrine
output, reduced • Fluid resuscitation as indicated by clinical
systemic vascular status and associated injuries
resistance)
Cardiogenic Normal to increased HR, • Pharmacologic support of cardiac output
(reduced cardiac reduced pulses, delayed with dobutamine and dopamine
output, normal to CRT, oliguria, jugular • Judicious fluid replacement as indicated
increased systemic venous distension. BP clinically
vascular resistance) normal until late in course

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HYPOGLYCEMIA IN CHILDREN AND ADOLESCENCE
• Hypoglycemia is a plasma glucose <2.6 mmol/l.
• Note: in preterm neonates in the 1st three days of life, glucose may be as low as
1.1 mmol/l without any underlying abnormality.
• In term neonates, it may be as low as 1.7 mmol/l in the first 3 days and 2.2 mmol/l
in the remainder of the week. Therefore, a glucose of 2.6mmol/l or lower requires
investigations.
CAUSES
• Infectious:
➢ Intrauterine infections
➢ Sepsis due to gram negative organism.
• Non-infectious:
➢ Causes of hypoglycemia in neonates:
o Conditions that increase insulin excess:
▪ Infant of a diabetic mother (transient hypoglycemia)
▪ Persistent hyperinsulinemic hypoglycemia of infancy (results from
insulin producing tumors or islet cell hyperplasia (nesidioblastosis)
o Conditions that diminish glucose production or substrate supply
▪ Intrauterine growth retardation and Prematurity which reduce
hepatic glycogen stores along with poorly developed
gluconeogenesis (transient hypoglycemia)
▪ Perinatal asphyxia (transient hypoglycemia)
▪ Rhesus incompatibility
▪ Inborn errors of metabolism
▪ Amino acids and organic acids
▪ Disorders of carbohydrate metabolism e.g. glycogen storage
disease, fructose intolerance, lactosemia.
▪ Fatty acid oxidation defects
▪ Urea cycle defects
▪ Endocrine causes:
▪ Hypopituitarism
▪ Growth hormone or adrenal insufficiency
▪ Beckwith-Wiedemann syndrome: Patients are LGA and present
with visceromegaly, hemihypertrophy, macroglossia, umbilical
hernias and distinctive ear creases.
➢ Other causes

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 o Drugs: alcohol, aspirin, beta blockers
o Ketotic hypoglycemia

CLINICAL FEATURES
• Hypoglycemia is often accompanied by signs and symptoms of autonomic
(adrenergic) activation and/or neurological dysfunction (neuroglycopenia)
• Autonomic features:
➢ Tremors
➢ Pounding heart
➢ Cold sweatiness
➢ Pallor
• Neurological features:
➢ Difficulty concentration
➢ Blurred vision
➢ Disturbed color vision
➢ Difficulty hearing
➢ Slurred speech
➢ Poor judgement and confusion
• Other features include:
➢ Problems with short term memory
➢ Dizziness and unsteady gait
➢ Loss of consciousness
➢ Seizures
➢ Death
➢ Behavioral changes
o Irritability
o Erratic behavior
o Nightmares
o Inconsolable crying
• Non-specific symptoms (associated with low, high or normal blood glucose):
➢ Hunger
➢ Headache
➢ Nausea
➢ Tiredness

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• Neonates may be asymptomatic or present with diaphoresis, jitteriness,
myoclonic jerks, cyanosis, apnea, feeding problems, tachycardia, hypothermia,
hypotonia, seizures and rarely myocardial infarction.
INVESTIGATIONS
• Blood:
➢ Serum glucose
➢ Hormone panel: insulin, cortisol, growth hormone
➢ Serum lactate
➢ Lipid profile: free fatty acids, cholesterol
➢ Serum ketones (hydroxyl butyrate and acetoacetate) and organic acids
➢ Full blood count
• Urinalysis

• Note: in hypoglycemic states and in the absence of ketones, it is important to look

for free fatty acids. Normal Free fatty acids indicate hyperinsulinism and raised
FFA indicate fatty acid oxidation defect.
• Hypoglycemia in the presence of urinary ketones suggests a counter regulatory
hormone deficiency or an enzyme defect in the glycogenolysis or
gluconeogenesis pathways.
TREATMENT
• Hypoglycemia is treated promptly after obtaining the critical intravenous samples
by intravenous infusion of 5ml/kg of 10% dextrose followed by adequate glucose
infusion to maintain euglycemia.
➢ Give 2.5ml/kg 10% dextrose IV bolus. After bolus is administered an IV
infusion should match normal hepatic glucose production.
➢ Approximately 5-8 mg/kg/min in an infant and about 3-5mg/kg/min IV
infusion in an older child should be continued. Adjust this till plasma level is
more than 3 mmol/l
• Severe hypoglycemia can be reversed by injection of glucagon IM or
subcutaneously:
➢ <12 years give 0.5mg
➢ >12 years give 10-10mcg/kg
• Treat underlying cause

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• In neonate treatment is directed at increasing oral feeding, if possible and if
necessary intravenous glucose.

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POISONING IN CHILDREN
• A poison is any agent of self-injury absorbed into the body through epithelial
surfaces.
• Common causes of poisoning in children are household products including
kerosene oil, drugs (paracetamol, barbiturates), chemicals (corrosive) and
pesticides (organophosphate compounds).
• While children younger than 11 years usually have accidental poisoning, toxic
exposures in adolescents ae primarily intentional (suicide, abuse) or
occupational.
• Clues to poisoning in children
➢ Acute onset of symptoms e.g. encephalopathy in an otherwise healthy child
(especially in children aged 1-3 years)
➢ Unexplained multisystem involvement
➢ Metabolic acidosis
➢ Acute renal failure, acute liver failure
➢ Arrhythmias in a child with no known cardiac illness.
• All children with suspected poisoning should be treated as medical emergencies.
➢ If the child is brough in the pre-toxic phase decontamination is highest
priority and treatment is based on history. The maximum potential toxicity
based on greatest possible exposure should be assumed.
➢ During toxic phase, the time between the onset of poisoning and peak effects,
management is based primarily on clinical and laboratory findings.
Resuscitation and stabilization are the first priority.
➢ During resolution phase, supportive care and monitoring should continue
until clinical and laboratory abnormalities have resolved.
• Indications for admission to ICU
➢ Need for antidote or enhanced elimination therapy
➢ Progressive clinical deterioration
➢ Evidence of severe poisoning, coma, respiratory depression, hypotension,
cardiac conduction abnormalities, arrhythmias, hypothermia or hyperthermia
APPROACH TO A CHILD WITH SUSPECTED POISONING
• History
➢ When did the event happen (Time, route, duration and circumstance-location
and intent of exposure)
➢ The drug/substance ingested (Name and amount of drug, chemical or toxin,
bite/sting involved)

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 ➢ The quantity of drug/substance ingested?
➢ Mode of poisoning e.g. ingestion/inhalation?
➢ Time of onset, nature and severity of symptoms
➢ Has any treatment been given? If yes what is the timing of first aid measures?
➢ Family history of diseases and drug therapy
• Examination:
➢ Vitals: Respiratory rate, heart rate, blood pressure, temperature, capillary
refill time and oxygen saturation
➢ Specific features will depend on the poison or toxin ingested.
• Initial assessment and treatment
➢ A, B, C, D, E- Manage airway, breathing and circulation, disability (quick
neurological exam-AVPU and Pupillary light reflexes), exposure.
o In child with altered mental status, respiratory depression and pupillary
constriction, a trial of naloxone should be given before intubation.
o Bag and mask ventilation is associated with a higher risk of aspiration,
minimize by aspiration by intubation using Sellick maneuver (Rapid
sequence induction) and synchronized mechanical ventilation.
o Adequacy of breathing should be assessed by respiratory effort, chest
movement, air entry and oxygen saturation.
➢ Place in left lateral position
➢ Physical examination including full neurological exam
• Supportive care
➢ Oxygen by nasal catheter/prong (1-2 L/min)
➢ Intravenous fluids
o Treat shock with fluid boluses, repeated if needed. Dopamine is the
vasopressor of choice if shock remains unresponsive, except in
poisoning due to tricyclic antidepressant or monoamine oxidase
inhibitors where its use is avoided.
o Maintenance fluids 2/3 of normal (half-strength darrows in 10%
dextrose)
➢ Do not use emetics
➢ Consider gastric lavage except in paraffin/alkaline/acid poisoning as well as
unconscious children
➢ Give activated charcoal for enhanced elimination of most toxins (1g/kg by
mouth or via nasogastric tube). Activated charcoal is contraindicated in an
unconscious child or in suspected GI hemorrhage or perforation
• Investigations

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 ➢ Urine, vomitus for toxicology
➢ Blood levels
➢ Clotting profile, blood glucose, liver function tests, electrolytes, urea and
creatinine when indicated
ACETAMINOPHEN (PARACETAMOL)
• This is the most common and safest analgesic and antipyretic used in children.
• The toxic dose is usually >200 mg/kg in children below 12-year-old.
• Hepatic damage after paracetamol overdose usually begins at >150 mg/kg and
occurs due to formation of a highly reactive intermediate, N-acetyl-p-
benzoquinoneimine. This is normally detoxified by endogenous glutathione.
• Overdose of paracetamol results in depletion of glutathione, allowing the
intermediate metabolite to damage hepatocytes.
• Stages of paracetamol toxicity are as follows:
➢ Stage I (12-24 hours): Child may appear well however, nausea, vomiting and
cold sweats.
➢ Stage II (24-48 hours): Clinical recovery with biochemical evidence of
hepatorenal injury; elevation of hepatic transminases to above 1000 IU/L is
associated with serious hepatic damage.
➢ Stage III (48-96 hours): Peak hepatotoxicity. Patient may have anorexia,
nausea, vomiting, right subcostal pain. If there is severe liver damage, there
will be repeated vomiting, jaundice, hypoglycemia, coagulopathy,
encephalopathy, ALT >2000 IU/L.
➢ Stage IV (7-8 days): Recovery is heralded by return of consciousness and
improvement in the hepatic function tests. Histological recovery may take up
to 3 months.
• Death may occur within 2-7 days of ingestion usually from cerebral edema and
sepsis.
MANAGEMENT
• Initial management
• If single dose <150mg/kg or cumulative dose <90mg/kg/day then no action
needed.
• Estimate paracetamol levels and take blood for INR, LFTs, U/Es after NAC
regimen.
• Those presenting 8 hours post ingestion should also start treatment immediately.
• Abnormal blood results should prompt a further 16 hour NAC infusion.

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• NAC is given by IV route and should be administered when indicated using the
normograms or situations indicated above.
• Dose: 150mg/kg infused over 60 minutes followed by a 4-hour infusion of
50mg/kg followed by a 16 hour infusion of 100mg/kg
• NAC side effects: bronchospasm, nausea, vomiting, flushing and urticarial rash
which resolve after infusion is stopped and antihistamine is given.
• Note: if bronchospasm occur stope NAC.

Loading dose 140 mg/kg, maintenance dose 70mg/kg four hourly for 17 doses
as oral solution mixed with fruit juice.
• Once hepatic failure occurs, the agent is contraindicated.
• Supportive treatment:
➢ Correction of hypoglycemia
➢ Maintenance of hydration

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 ➢ Electrolyte balance
➢ Treatment of coagulopathy
➢ Hemodialysis for acute renal failure
➢ Management of fulminant hepatic failure

END OF BOOK REVISION

OSCE STATIONS
GENERAL PEDIATRICS
STATION 1
Look at this picture of a child admitted two weeks ago with generalized body
swelling

393
1. What is your differential diagnosis?
2. Urinalysis was done and results as follows: Ketones 1+, Glucose nil, Protein 4+
and Blood 1+, What is your diagnosis?
3. Which initial drug is used in this condition and list five of its side-effects?
4. What are the atypical features of nephrotic syndrome?
5. If this was nephritic syndrome which bed side investigation can you do and what
investigations would you order for?
6. What are the causes of hypertension in children?
STATION 2
1. Look at this patient with history of fever. What Questions would you ask to make
a diagnosis?
STATION 3
1. A bedside urinalysis is done and the following results are taken:
Glucose-3+
Protein- 1+
Ketones- Negative
Nitrites- Negative

394
 Blood- 1+
a) What questions would you ask on history to ascertain your diagnosis?
b) What is the possible diagnosis based on urinalysis?
c) How would you manage this patient had he come in DKA?
d) What are the major life-threatening complications that may arise during the
management of DKA and how will the patient present?

IMAGING
STATION 1
Examine this chest X-ray of a 6-year-old who has come to the admission room

1. Describe your findings.

2. What are the possible signs and symptoms you would see in this patient?
3. How would you treat this child?
STATION 2
This is the follow-up chest X-ray of the same patient as in station 1 after 9 months
of TB treatment

395
 1. Give 2 possible diagnoses
2. What information would you want from the caregiver?
3. How would you manage this patient?
STATION 3
This is a chest X-ray from an 18-month old toddler, with cough, fever and difficulty
breathing.

396
1. Describe the main abnormality seen.
2. What is your tentative diagnosis?
3. What is the likely causative organism?
4. What antibiotic will you use to treat this condition?

397
STATION 4

1. What abnormality can you see on the CXR?

2. What is your diagnosis?
3. What are the causes?
4. What is the immediate management?

398
STATION 5

1. What abnormalities can you see on the CXR?

2. What are the differentials?
3. What drugs would you give for PCP?
4. What antifungal drugs?
STATION 6
1. Look at this X-ray and write down the most striking feature you see

399
2. What is your most likely diagnosis?
3. Give 2 differential diagnoses?
4. Give the complications
5. What 2 investigations would you request to reach the most likely diagnosis?

400
INSTRUMENTS
STATION 1

1. What is the above equipment called?

2. How is the patient supposed to use the above equipment?
STATION 2

1. What is this equipment called?

2. What are the indications?
3. What are the sites where you do the procedure from?

401
STATION 3

1. What is the equipment below?

2. What are the indications for the use of the equipment?
3. What mechanisms does it use?
4. What precautions are you going to take when using the equipment?
5. What are the complications?
STATION 4
1. What is the use of these equipment in the long term follow up the patient with
DM?

2. Which is the best method to determine how well controlled the blood glucose in
a diabetic patient?

402
STATION 5
1. What is this equipment?

2. What would you use it for?

STATION 6
1. What is this equipment?

2. What are the indications for its use?

403
STATION 7
1. What is this equipment and explain its use?

2. How would a child with an illness where this equipment is used present?
3. How would you manage such a child?
STATION 8
1. What are these pieces of equipment and what are they used for in labor ward?

2. List 2 acute complications that may occur in the brain and GIT of survivors if
there is failure to use this equipment efficiently?
3. Write 1 long term complication that can occur?

404
STATION 9

1. What is the above equipment called?

2. What are the indications?
3. What could have happened in the pic below?

405
4. Label the female genitalia below?

406
ESSAYS
1. A 3kg infant was born to a 16-year-old Para 1 mother. Labor was rather prolonged
lasting 14 hours and there was prolonged rupture of membranes for 24 hours.
APGAR score was 9/10. 2 days after birth the child developed failure to feed,
lethargy, jaundice, and hypotonia.
a) What is the most likely diagnosis?
b) List four investigations that must be done in this child to help you confirm the
diagnosis?
c) What is your management plan?

2. Emmanuel, a month-old infant, born at term, is admitted for jaundice that

appeared on day 6 after birth and has persisted. He is alert, sucks well and has no
fever. His hemoglobin is 15g/dl with total serum bilirubin of 250 micromol/l.
a) Write down 2 important questions you would ask the mother with regards to
the jaundice
b) Write down 2 differential diagnoses
c) Write down your management

3. You walk into a mall with your 4-year-old toddler and she picks a pack of food
with the following nutritional information per 100g:
Protein: 10g, Maize 40g, Wheat flour: 50g, Millet flour: 25g, Olive oil: 4g, Palm
oil: 6g, Zinc: 11mg, Potassium 110mg, Vitamin A: 800mcg and folate: 230mcg
a) Work out the energy content of this pack
b) Considering that your child’s daily caloric requirement is about 1600kcal,
how many packs would she require to cover this need?

4. A 13 months old toddler is admitted with acute diarrhea with severe dehydration.
He weighs 10kg.
a) Write down 5 clinical features you are likely to elicit
b) What is the average percentage of his body weight loss?
c) Outline the management of this patient

5. George, a 6-month-old infant is admitted with pneumonia (first occurrence) and

is able to feed well. His mother tested positive for HIV. Write down 5 questions
you would ask the mother to assess the risk of HIV infection in George.

407
6. A 14-year-old girl, Nana Maliki presents to your hospital with a history of
breathlessness and swelling of her legs for the past one week. Some of the
physical findings include orthopnea and a pansystolic murmur heard best at the
apical area and radiating to the left axilla.
a) What is the complete diagnosis of this medical conditions in this girl?
b) Mention two other signs that could be found in this patient consistent with
the above diagnosis?
c) Indicate 3 investigations you are going to do that will help you confirm your
diagnosis.

7. An 8-month-old male baby presents to hospital for a third episode in 3 months,

of swelling of the hands and crying uncontrollably. Physical examination reveals
an irritable baby with bilateral swollen hands. Further examination revealed a
tinge of jaundice and splenomegaly.
a) What is your provisional diagnosis in this baby?
b) What 2 investigations would you request to confirm the diagnosis?
c) What is the most likely way the child acquired this condition?
d) Write 5 complications that can occur in this condition?

8. A 2-year-old boy presents to 1st level referral hospital where you are working
with history of swelling of the feet for the last one week. There is also history of
diarrhea for 3 weeks prior to admission and a peeling rash on the feet of the child.
The child is the first born. Currently mum is 8 months pregnant. Preliminary
investigation reveals the following:
➢ Potassium: 3.1mmol/l
➢ Sodium 134mmol/l
➢ Chloride 104mmol/l
➢ Blood sugar 1.8mmol/l
a) What is the most likely diagnosis?
b) List 4 diagnostic features that you will look for as you examine the child that
will help you make a more conclusive diagnosis of this condition
c) Indicate any abnormalities in the above given investigation result
d) What 5 things affecting this child would likely cause mortality within 24
hours of admission unless taken care on admission?
e) What 5 steps are you going to take immediately to save the life of this child?

408
9. A 14-year-old girl called DJ is referred from a health center 10km from the
hospital where you are working with history of east fatiguability, fever and
bleeding from the nose for one month. On examination, you find that the girl is
very pale, has purplish bumps in the mucus membrane of the oral cavity and is
toxic looking. The temperature is 39OC.
a) What 4 critical investigations are you going to do to help you make a definitive
diagnosis on this child?
b) What are 2 most likely differential diagnosis that would explain the patient’s
condition
c) List 6 other signs that you would look for on examination of the patient that
would help you diagnose the patient’s illness.

10.A 16-month-old toddler (girl) is brought to the children’s clinic, because the
mother is concerned that the child’s growth and development is suboptimal
compared to her older siblings. Upon further questioning she agrees to the child
having recurrent upper respiratory infections, occasional bouts of loose stool and
fevers that have been treated as “malaria” by the private doctor. On examination
she weighed 7.5kg, her height was 80cm, she had slight pallor with generalized
lymphadenopathy, sores (white patches) in the mouth, CVS was normal and per
abdomen there was hepatosplenomegaly with no pedal edema.
a) Give 4 possible most likely differentials for the described condition.
b) List 4 investigations that you would do to come up with a definitive diagnosis
of the condition of this child?
c) If you are to stage this condition, what stage of disease is the child in.

11.A 2-year-old girl presents to your hospital and after investigations the CSF
revealed the following results
Appearance: cloudy
WBC: 120cells/mm3- polymorphs 80% and lymphocytes 20%
RBC: 50cells/mm3
Gram stain: gram negative coccobacilli
Ziehl Neelsen stain: no organism
Protein: 0.75g/l
Sugar: 1.6mmols/l

409
 a) List 4 cardinal abnormalities that you see in this CSF result
b) What is the causative organism?
c) What specific measure would you have taken to prevent this illness in the
child?
d) List 4 long term complications associated with this condition?

12.This is a Hemogram of a patient you have just seen at your hospital:

WBC: 5.7 x 109/L
RBC: 2.8 x 1012/L
Hb: 5.3g/dl
MCV: 49fl
MCH: 18.3pg
Platelets: 272 x 109/L
a) List 4 abnormalities that you see in this Hemogram
b) What is the diagnosis based on the Hemogram?
c) Give 3 possible causes of this condition.

13.An 8 weeks old infant presents to your admission ward with history of cough for
4 days and difficult breathing. On examination, the infant is well nourished and
weighs 6kg. However, the infant is dyspneic, tachypneic with subcostal
recession. Respiratory rate is 70/min. Temperature is 37.8oC. There is some
cyanosis, examination of the chest reveals a bilateral ronchi best heard at the end
of expiration and a few crepitations.
a) What is the most likely diagnosis?
b) List 3 likely causative organisms
c) Describe in one or two sentences the pathophysiological process of this
condition
d) What is your management?

14.A 2 days old baby comes into the neonatal unit with history of developing
jaundice since a few hours after birth. On examination, you notice that the child
is weak, hypotonic, deeply jaundiced and pale. He is the 2nd born, the first sibling
did not suffer from this condition.
a) What is the most likely full diagnosis of this illness in the neonate?

410
 b) List 4 investigations that you are going to do in order to confirm the diagnosis

15.An 11 months old infant presents to your admission ward convulsing. There is
history of 2 former such episodes of convulsing. According to the mother, the
convulsions involve the whole body and last about 3 minutes. The child has
temperature 39oC.
a) What is the most likely diagnosis?
b) List 3 disease condition that would possibly have caused this condition
c) Indicate 2 cardinal investigations that would help you manage this child.

16.Joanna a 3-year-old toddler, is brought to AMEU with history of diarrhea and

vomiting for the past 3 days. On examination, she is lethargic, too weak to take
fluid orally and passed scanty urine all day. Her weight is 14kg
a) What plan are you going to apply in rehydrating Joanna?
b) What fluid are you going to use in treating this little girl?
c) Give details of the plan you are going to use in rehydrating Joanna

17.Hamaundu a 4-year-old-boy, is brought to the OPD with edema of both hands

and feet and sores in the mouth. He weighs 8.5kg.
a) Assign the nutritional status to Hamaundu based on welcome classification
b) How many milliliters of F75 would you need to cover his caloric requirement
estimated at 1500kcal/24 hours?

18.Kasongo a 10-year-old boy of Kasisi, presents with fever of 3 weeks duration.

He was treated for malaria a week ago despite a negative malaria thick smear. He
has remarkably lost weight and is complaining of abdominal pain associated with
constipation. The mother says that he loses his memory at times and would be
incoherent in his speech.
a) Write down your differential diagnosis
b) What is your most likely diagnosis?
c) List investigations you would carry out

19.A 7-year-old girl is brought to LMGH with a history of recent bedwetting and
unexplained weight loss. No history of tuberculosis contact. Her rapid test for
HIV is negative
a) What is your differential diagnosis?
b) Write down 2 more symptoms that may be associated with this condition

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 c) List down 3 investigations to ascertain your diagnosis

20. Martha, a 4-year-old girl has had a history of bony pains on and off for 2 weeks.
On examination, she is febrile, underweight, pale and in respiratory distress. She
has cervical and axillary lymphadenopathy.
a) What further questions would you ask to help determine the diagnosis?
b) Give 2 differential diagnoses?
c) What investigations would you do to help ascertain your diagnosis?

21.Mwila is referred from Kasama for a huge spleen. She has had few episodes of
abdominal pain which the mother attributed to worm infestation. On examination
she is underweight with a tinge of jaundice. The night of her admission, she bled
profusely from the nose and required nasal packing.
a) Write down 3 possible diagnoses?
b) List down specific investigations to ascertain your diagnosis

22.These are pictures of the same girl in question 20.

a) Write down your findings

b) What is your diagnosis?
c) Choose the most likely karyotype
(1) XY
(2) XO
(3) XYY
(4) 47, XX, +t (14q21q)

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23.A 24-month-old toddler, well nourished, is admitted with acute diarrhea and
severe dehydration. He weighs 9.6kg
a) What is his expected weight?
b) What is his body weight percentage loss?
c) Work out the fluid amount required to cover his deficit?

24.You are called to review a newly diagnosed insulin dependent diabetes mellitus
case. The patient is receiving insulin intravenously and his morning urine analysis
is as follows:
SpGr: 1.025, Protein: +, pH: 6.0, Blood: negative, Ketones: ++++, Glucose:
Negative
a) Explain in few words what may have led to the presence of ketones and the
absence of glucose in urine.
b) In your opinion, what would be the blood sugar levels in this patient? Low,
normal, or high?
c) How would you manage this patient?

25.A 24-month-old girl presents to hospital with history of diarrhea and vomiting
for 4 days and anorexia for 2 weeks. The mother has recently fallen pregnant but
she has recently separated from her husband. On examination the child is
miserable, apathetic and has bilateral pedal edema and dermatosis. Her weight is
7.2kg. Her muscles are wasted.
a) What is the diagnosis?
b) List 5 critical conditions that you must manage in order to save the life of this
child within the first 48 hours
c) What is the expected weight for age for this child?
d) Give differentials that need to be ruled out every time you have such a case.

26.A boy is brought into hospital with difficulties of breathing and swelling of the
legs. The patient indicates that he is unable to sleep supine on the bed as he runs
out of breath. He also indicates that he develops breathlessness after walking only
a few steps. On examination, the boy has a pansystolic murmur in the mitral area
radiating to the left axilla. He also has anemia and fever as well as splenomegaly.
a) Give your differential diagnosis
b) Line up investigations based on your differential diagnosis
c) Which organisms are usually associated with this condition?
d) Which one is the most affected age group and why?

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 e) How would you manage this child?

27.A 13-year-old girl is brought into the emergency room with history of difficulty
breathing since the previous night. She is a known sickle cell disease patient who
was lost to follow up. On examination the girl is in severe respiratory distress
with a respiratory rate of 70/min, with sub-costal and intercostal recession. Her
temperature is 38.7oC. Her hemoglobin is 7g/dl and her oxygen saturation using
a pulse oximeter is 76.
a) What are the 2 key differential diagnoses for the condition that the patient is
presenting with?
b) List 3 investigations you would do in relation with this condition.
c) How would you manage this patient?

28.A3 and a half year old female child presents with noisy breathing and fever of 6
hours duration. Prior to this she had been perfectly well. She was born at LMGH
at full term with a birth weight of 3.9kg. There were no neonatal problems. Her
routine vaccinations are up to date and weight gain has been adequate. There is
no family history of note. On examination the patient looked very ill with
temperature 39.7oC, pulse 110/min, BP 110/78, dribbling of saliva from one
corner of the mouth, intercostal and subcostal indrawing with a clear chest. Blood
investigations reveal
Hb- 12g/dl
WBC- 20 x 109/L (polymorphs-84% and lymphocytes-12%)
Sodium 135mmol/L
Potassium 3.5 mmol/L
Urea 4.6 mmol/L

Urinalysis was normal

a) What is the most likely diagnosis?
b) What is the most likely causative organism?
c) Name 3 management measures you are going to institute in this child

29.Mutinta a 10-year-old girl presents to hospital with history of cough and swelling
of legs for 4 days. On examination she is dyspneic at rest, has bilateral pedal
edema and hepatomegaly. Examination of the heart reveals a pan-systolic
murmur, radiating to the axilla and mid-diastolic murmur in the mitral area. She
also has a diastolic murmur in the aortic area.

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 a) What is the full diagnosis of this child’s condition?
b) List the valve structural problems that this child has
c) Indicate the four measures you will take in order to manage the acute phase
of this child
d) What long term treatment would you institute in order to prevent further
deterioration of this child’s condition?

30.Nalishebo is a 3-year-old girl who presents with edema and is underweight. The
following are her lab results:
Urea 2.5 mmol/L
Sodium 128 mmol/L
Potassium 3.5 mmol/L
Serum albumin 28g/L
Serum cholesterol 2mmol/L
Urinalysis:
Protein 2+
Blood 1+
Leucocytes: Numerous
a) What 2 other important physical features should be present in this child?
b) What is the most likely diagnosis?
c) What other diagnoses should be considered and treated?

31.A 10-year-old boy, Judge Siachinji, whose parents just moved to live in Lusaka
from Siavonga presented at LMGH with terminal hematuria
a) What is the likely diagnosis for this boy?
b) What investigations would you carry out to confirm the diagnosis in this boy
and what would be the finding consistent with the diagnosis?
c) Write down 2 complications from this condition that the boy may develop if
left untreated?
d) How would you treat this child?

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32.Zambia ranks one of the countries in Sub-Saharan Africa with the worst infant
and childhood mortality rates. One of the ways to avert this problem is to
reinforce the aims and objectives of the under-five clinics
a) State the major aims of under-five clinics in Zambia as captured on the under-
five card?
b) List the preventable disease from which a Zambian child is immunized on the
current EPI?

33.An 8-year-old girl presented at LMGH with restlessness, heart palpitations and
was rather dazed in general appearance. Investigations showed an impaired
glucose tolerance test with:
Total serum thyroxin 186nmol/L (normal range 10-30)
Free T4 12.5pmol/L (normal range 9.4-24.5)
Free T3 11.8pmol/L (normal range 2.9-8.9)
Serum TSH <0.1Mu/L (normal range 0.7-2.5)
a) What is the most likely clinical diagnosis for this patient?
b) What is the pathophysiological basis of the heart palpitations and restlessness
in this child?
c) Write down 2 other (physical findings) consistent with this condition you
would likely elicit on physical examination of this child?

34.A 9-year-old-boy is referred to LMGH from a mission Hospital for further

investigation. He had developed progressive abdominal distension over a period
of 7 months, and had reduced exercise tolerance compared to his peers.
Examination revealed a frightened but co-operative child who could only
understand Lozi. He was pale, anicteric and weighed 38 kg. Congestion of the
jugular veins was evident on deep inspiration. The pulse varied between 90-97
beats per minute and the heart sounds were normal. Auscultation of the chest
revealed a few coarse crepitations in the bases. The abdomen was uniformly
distended and invariably impossible to palpate for organomegaly. Shifting
dullness was easy to elicit and there was pitting oedema above both ankles.
a) What is your provisional diagnosis?
b) What 3 investigations would you ask for to support this diagnosis?
c) How would you mange this child?

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35.Mwanja a 35-week-old infant, is admitted with some respiratory distress and
commenced on oxygen 3L/min via nasal prongs and the saturation is stubbornly
stuck at 85% despite increasing the oxygen flow. Mwanja is HIV negative and
his lungs are clear on auscultation. His hematocrit is around 65%
a) Give your differential diagnosis
b) Justify the hematocrit reading
c) Line up the principles of management of this child

36.A 4-month boy is brought to OPD for not gaining weight. He is exclusively
breastfed and mother tells that he sweats profusely while taking feed. He was
treated for bronchopneumonia at 2 months of age. On examination his weight is
4kg irritable child with a respiratory rate of 45/min and heart rate 140/min
a) What is the diagnosis?
b) How will you investigate?
c) List 3 complications of this condition

37.Mweemba, 10-year-old girl is brought into the emergency room with history of
difficulty breathing since the previous night. She is a known sickle cells disease
patient who has been receiving monthly blood transfusions due to cerebral
vascular accident. She is in severe respiratory distress with a respiratory rate of
70/min, associated with subcostal and intercostal recession. Her temperature is
38.7oC, her hemoglobin is 7g/dl and her oxygen saturation is 76%.
a) What are the 2 key differential diagnosis for the condition that the patient is
presenting with?
b) Besides blood, list 2 further investigations you would carry out
c) Comment on her hemogram: WBC 22,300 cells/mm3, MCV: 65.7fl, RDW-
CV 25%
d) How would you mange this child?

38.Chola a 10-year-old, is admitted with a month-long history of headaches, low

grade fever on and off, he was kept in hospital for few days. 3 weeks ago and
treated for meningoencephalitis and showed some improvement. This time, he is
not able to talk coherently and displays squint. His appetite is poor and he lost
some weight.
a) Give 3 differential diagnoses
b) How would you investigate this boy?
c) How would you mange chola?

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39.Sakala, a 9-year-old is brought to AMEU with continuous generalized tonic
clonic seizures for the past half an hour. On examination, he is febrile with not
signs of meningeal irritation. He has scars of sore he developed as a result of
thorny bushes while setting rat traps. Other systems are unyielding
a) What is your differential diagnosis?
b) Give 2 bedside assessments that would help your diagnosis?
c) What is the most likely diagnosis?
d) List down your management principles of this condition

40.A 3-day old neonatal with jaundice from the first day from the umbilicus to the
knee presents with lethargy, pallor and is irritable. You suspect Kernicterus.
a) What is the Kramer staging of this child?
b) Grade his kernicterus
c) A coombs direct test was performed. Whose blood was it performed on?
d) When phototherapy is instituted list 4 measures to take before and during the
procedure.
e) List 2 general complications of phototherapy

41.A 10-year-old known sickle cell patient presents with a history of difficulty
breathing that occurred since the last night as well as a history of several vaso-
occlusive crises over the past month.
a) List 2 ways to manage this patient other than IV fluids and analgesia
During one painful crisis the child presents with a fever and respiratory
embarrassment
b) List 2 differentials
c) List 2 possible organisms
d) List 2 groups of antibiotics for your differentials

42.A 3-week-old neonate that has been thriving is brought to OPD, the mother
complains that the neonate often throws up after feeding then continues to eagerly
feed, a preliminary full blood count is normal aside mild anemia and the child is
not opening bowels as frequent anymore.
a) What is your differential diagnosis?
b) How would you confirm your diagnosis clinically?
c) List 3 biochemical derangements in this condition
d) What gender does this condition occur mostly in?

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43.A 5-year-old presents with a mandibular mass and dental malalignment. A biopsy
from the mass taken for histopathology shows a starry sky appearance.
a) What is the most likely diagnosis?
b) What investigations should be carried out prior to chemotherapy?
c) What 2 crucial measure should be taken prior to chemotherapy?
d) What is the most common complication related to chemotherapy?
e) What biochemical derangements are associated with complication?

44.A 4-month old child not gaining weight presents with a grade 3 pansystolic
murmur at the left lower sternal border with a loud second heart sound at the
pulmonary area. Echo shows tetralogy of Fallot.
a) List 4 clinical features in this condition
b) List 2 hematological and 2 radiological features of the condition
c) Write down 2 complications
d) What 4 drugs are used to manage this condition and reasons why?

45. A 3-year-old with a 5-day history of a febrile illness and headache. He is treated
with Pen V at a local clinic and showed some improvement however he fitted
once before admission.
a) Write down 3 differentials
b) List 4 investigations
c) How would you manage this child?

46.A 2-year-old wakes up failing to walk and was fine the previous day. Physical
examination reveals loss of pain in the lower limbs up to the knees.
a) What is your working diagnosis?
b) What are your differentials?
c) What important questions will you ask for in your history?
d) What’s your worry about this child?

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47.An 11-year-old presents with a nagging itchy skin lesion on the medial malleolus
for 2 months that opened up and keeps growing. Preliminary investigations
reveal:
WBC 13, 000/mm3
Hb g/dl
MCV 75fL
RBS 9.6 mmol/l

a) Give 2 differentials
b) What is the most likely diagnosis?
c) List one conclusive test you will do to confirm your diagnosis
d) List 4 bullets on management

48.A new born to an HIV mother is brought to your pediatric department. The
mother started ART at 8 months gestation and is exclusively breasting the infant.
a) What prophylaxis should the neonate be put on?
b) What do you need to ensure/follow up on with that prophylaxis?
c) What systemic tests have to be done as the child grows and when would you
do them?
d) NAT reveals that the baby is positive, what regimen do you put the child on?

49.A family moved into a new house and their 4-year-old child presents with
difficulty breathing and foaming at the mouth without a no fever or any fits.
a) Write down 4 initial management steps
b) What is the most likely diagnosis?
c) List 4 clinical signs to look for
d) Give 3 fundamental drugs used to treat

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ANSWERS TO OSCE STATIONS
GENERAL PEDIATRICS
STATION 1
Look at this picture of a child admitted two weeks ago with generalized body
swelling

1. What is your differential diagnosis?

Answer:
➢ Nephrotic syndrome
➢ Severe malnutrition
➢ Acute nephritis
➢ Congestive heart failure
➢ Protein losing enteropathy
➢ Chronic liver disease

2. Urinalysis was done and results as follows: Ketones 1+, Glucose nil, Protein 4+
and Blood 1+, What is your diagnosis?
Answer: Nephrotic syndrome

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3. Which initial drug is used in this condition and list five of its side-effects?
Answer
Prednisolone

Side effects:
➢ Mood changes
➢ Cataracts
➢ Moon face
➢ Osteoporosis
➢ Abdominal striae
➢ Light skin
➢ Easy bruising
➢ Peptic ulceration
➢ Weight gain
➢ Growth retardation
➢ Aseptic necrosis of the femoral head
➢ Hypertension
➢ Immunosuppression

4. What are the atypical features of nephrotic syndrome?

Answer: hypertension, hematuria and impaired renal function

5. If this was nephritic syndrome which bed side investigation can you do and what
investigations would you order for?
Answer:
Bedside investigation: blood pressure measurement
Investigations
➢ Urine microscopy
➢ Full blood count
➢ Urea, electrolytes and creatinine
➢ ESR
➢ Throat swab culture
➢ Others: Anti-streptolysin-O-titers (ASOT), CXR, renal U/S, antinuclear
antibody, antiDNA antibodies and complement levels (C3 and C4)
6. What are the causes of hypertension in children?
Answer:

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 ➢ Renal causes: chronic glomerulonephritis, reflux or obstructive
nephropathy, polycystic or dysplastic renal disease, bilateral renal
stenosis
➢ Cardiovascular causes: coarctation of the aorta and takayasu aortoarteritis
➢ Endocrine: hyperthyroidism, hyperparathyroidism, congenital adrenal
hyperplasia, cushing syndrome, primary aldosteronism,
Pheochromocytoma and neuroblastoma
STATION 2
1. Look at this patient with history of fever. What Questions would you ask to make
a diagnosis?
Answer:
➢ Any history of poor feeding
➢ Convulsions/twitching
➢ Excessive crying
➢ Abdominal distension
➢ Yellow discoloration of skin
➢ Vomiting
➢ Diarrhea
➢ Difficulty in breathing
➢ Reduced activity
2. Examiner asks candidate ‘How do you manage such a condition’?
Answer:
➢ Investigations
a) Blood culture
b) Lumbar puncture
c) Urine microscopy/culture and sensitivity
d) CXR
e) Swab any infected sites for MCS
f) Full blood count
g) ESR
h) CRP
➢ Drugs: X-pen and gentamicin or cefotaxime/cloxacillin IV 7 to 10 days.
3. Examiner asks candidate ‘why do a Lumbar puncture’?
Answer:
➢ Child may have meningitis
4. Examiner asks candidate ‘What are the most likely bacterial causes’?

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 Answer:
➢ Escherichia coli
➢ Listeria monocytogenes
➢ Group B streptococcus
➢ Staphylococcus aureus
➢ Streptococcus pneumoniae
STATION 3
1. A bedside urinalysis is done and the following results are taken:
Glucose-3+
Protein- 1+
Ketones- Negative
Nitrites- Negative
Blood- 1+
a) What questions would you ask on history to ascertain your diagnosis?
Answer:
➢ Demographics: Age and gender
➢ Drinking too much water?
➢ Passing a lot of urine? Even at night (or bedwetting)?
➢ History of weight loss?
➢ Eating too much?
➢ Recurrent infections?
➢ Family history of DM?

b) What is the possible diagnosis based on urinalysis?

Answer: Diabetes Mellitus

c) How would you manage this patient had he come in DKA?

Answer:
➢ IV fluids
➢ Soluble insulin
➢ Potassium
➢ Treat any infection

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d) What are the major life-threatening complications that may arise during the
management of DKA and how will the patient present?
Answer:
➢ Headache
➢ Alteration and deterioration in level of consciousness
➢ Delirious outbursts
➢ Bradycardia
➢ Vomiting
➢ Diminished responsiveness to painful stimuli
➢ Diminished reflexes
➢ Dilated or unequal pupils
➢ Hypertension
➢ Convulsions

IMAGING
STATION 1
Examine this chest X-ray of a 6-year-old who has come to the admission room

1. Describe your findings.

425
 Answer: non-homogenous opacities in both lung fields (bilateral pulmonary
infiltrates)

2. What are the possible signs and symptoms you would see in this patient?
Answer:
➢ Symptoms: fever, chronic cough, weight loss, night sweats, difficult
breathing
➢ Signs: Finger clubbing, tachypnea, hepatosplenomegaly, wheeze,
subcostal recession, bilateral crepitations

3. How would you treat this child?

Answer:
➢ Antituberculosis treatment
a) Rifampicin, isoniazide, pyrazinamide and ethambutol for 2 months (intensive
phase)
b) Rifampicin and isoniazide for 4 to 6 months (continuation phase)
STATION 2
This is the follow-up chest X-ray of the same patient as in station 1 after 9 months
of TB treatment

1. Give 2 possible diagnoses

Answer:

426
 ➢ Multidrug resistant pulmonary tuberculosis
➢ Lymphocytic interstitial pneumonitis
➢ Poor compliance with anti-tuberculosis treatment

2. What information would you want from the caregiver?

Answer:
➢ Has patient been taking medication consistently?
➢ Any recurrent chest infections?
➢ Any TB contact?
➢ Persistent fever?
➢ Night sweats?
➢ Weight loss?
➢ What is the HIV status?

3. How would you manage this patient?

Answer:
➢ Investigations
a) Full blood count with differential count, Erythrocyte sedimentation rate
b) Sputum/gastric lavage for Acid fast bacilli + culture + sensitivity
c) HIV test
d) LFT/ U&Es
e) CD4 count
➢ Treatment: Antituberculosis treatment and antiretroviral drugs (AZT/D4T
+ 3TC + NVP/EFV)
STATION 3
This is a chest X-ray from an 18-month old toddler, with cough, fever and difficulty
breathing.

427
1. Describe the main abnormality seen.
Answer: homogenous opacity of the whole right lung, consolidation or lobar
pneumonia

2. What is your tentative diagnosis?

Answer: Right lung lobar pneumonia

3. What is the likely causative organism?

Answer: Steptococcus pneumoniae or Haemophilus influenza or Staphylococcus
aureus

4. What antibiotic will you use to treat this condition?

Answer: Antibiotics (chloramphenicol, crystalline penicillin or cephalosporin,
gentamycin)

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STATION 4

1. What abnormality can you see on the CXR?

Answer: the costophrenic angle extends more inferiorly than usual because of air
(deep sulcus sign) and the liver appears to be more radiolucent
2. What is your diagnosis?
Answer: pneumothorax
3. What are the causes?
Answer: Trauma, bronchial asthma, cystic fibrosis, TB, spontaneous
4. What is the immediate management?
Answer: aspiration

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STATION 5

5. What abnormalities can you see on the CXR?

Answer: pneumoceles
6. What are the differentials?
Answer:
➢ Pneumocystis jiroverci pneumonia
➢ Tuberculosis
➢ Bacterial pneumonia
➢ Karposi’s sarcoma
7. What drugs would you give for PCP?
Answer: Trimethoprim-sulfamethoxazole (TMP-SMX) (15-20mg TMP and 75-
100 mg SMX/ kg/day in 4 divided doses) administered intravenously or orally
8. What antifungal drugs?

STATION 6
1. Look at this X-ray and write down the most striking feature you see

430
 Answer: Hair on end appearance
2. What is your most likely diagnosis?
Answer:
➢ Sickle cell disease
➢ Hemolytic anemia
3. Give 2 differential diagnoses?
Answer:
➢ Meningioma
➢ Hemangioma
➢ Cyanotic heart disease

4. Give the complications

Answer:
➢ Cerebrovascular accident
➢ Subarachnoid hemorrhage
➢ Retinopathy

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 ➢ Adeno-tonsillar hypertrophy
➢ Acute chest syndrome
➢ Splenic sequestration
➢ Autosplenectomy with recurrent infections with encapsulated organisms
e.g. Streptococcus pneumoniae
➢ Gall stones
➢ Avascular necrosis of femoral head
➢ Nephrotic syndrome
➢ Isosthenuria
➢ Priapism and impotence

5. What 2 investigations would you request to reach the most likely diagnosis?
Answer:
➢ Hemoglobin electrophoresis
➢ Sickling/solubility test
➢ PCR
➢ Full blood count and peripheral smear

INSTRUMENTS
STATION 1

1. What is the above equipment called?

Answer: Spacer and inhaler

432
2. How is the patient supposed to use the above equipment?
Answer:
➢ Shake the inhaler well before use (3-4 shakes)
➢ Remove the cap from the inhaler and from the spacer, it has one
➢ Put the inhaler into the spacer.
➢ Breathe out, away from the spacer.
➢ Bring the spacer to your mouth
➢ Press the top of the inhaler once
➢ Press the top of the inhaler once
➢ Breathe in very slowly until you have taken a full breath
➢ Hold your breath for 10 seconds, then breath out
STATION 2

1. What is this equipment called?

Answer: Bone marrow aspiration needle
2. What are the indications?
Answer: can be diagnostic (in pure aplastic anemia, bone marrow culture)
3. What are the sites where you do the procedure from?
Answer: Sternum, iliac crest

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STATION 3

1. What is the equipment below?

Answer: Phototherapy machine
2. What are the indications for the use of the equipment?
Answer: unconjugated hyperbilirubinemia (jaundice)
3. What mechanisms does it use?
Answer:
a. Photo-oxidation
b. Photo-isomerization
c. Structural-isomerization
4. What precautions are you going to take when using the equipment?
Answer:
It should be 20 to 45cm from the infant, the child should be naked except for the
eyes and testes for the male child which should be shielded.

5. What are the complications?

Answer:
➢ Hyperthermia and dehydration due to insensible water loss
➢ Watery diarrhea
➢ Hypocalcemia
➢ Retinal damage
➢ Erythema
➢ Bronze baby syndrome if used in infant with direct hyperbilirubinemia

434
 ➢ Potential genetic damage and mutations
➢ Disturbed of maternal-infant interaction
STATION 4
1. What is the use of these equipment in the long term follow up the patient with
DM?

Answer:
➢ Opthalmoscope for eye examination as patient may develop cataracts and
diabetic retinopathy.
➢ Multistix for monitoring glycosuria and proteinuria which are signs of
diabetic nephropathy
2. Which is the best method to determine how well controlled the blood glucose in
a diabetic patient?
Answer: glycosylated hemoglobin (HbA1C)
STATION 5
1. What is this equipment?

Answer: diagnostic set

435
2. What would you use it for?
Answer:
➢ To check for red reflex
➢ To do fundoscopy (Examine eyes)
➢ To examine ears
➢ To examine the nose
➢ To examine the mouth/throat
STATION 6
1. What is this equipment?

Answer: Nasogastric tube

2. What are the indications for its use?
Answer:
➢ Feeding
➢ Rehydration
➢ Emptying the stomach
➢ Gastric lavage
➢ Administration of medication

436
STATION 7
1. What is this equipment and explain its use?

Answer: Otoscope, used for the examination of the ear

2. How would a child with an illness where this equipment is used present?
Answer:
➢ Fever
➢ Pain in the ear
➢ Rubbing the ear
➢ Ear discharge
3. How would you manage such a child?
Answer:
➢ Investigations: ear swab
➢ Treatment: Oral antibiotics
STATION 8
1. What are these pieces of equipment and what are they used for in labor ward?

Answer:

437
 ➢ Endo-tracheal tube
➢ Laryngoscope
➢ Ambu-bag
➢ These are used for intubation and resuscitation with birth asphyxia

2. List 2 acute complications that may occur in the brain and GIT of survivors if
there is failure to use this equipment efficiently?
Answer:
➢ Hypoxic-ischemic encephalopathy
➢ Necrotizing enterocolitis and paralytic ileus

3. Write 1 long term complication that can occur?

Answer: Cerebral palsy

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STATION 9

1. What is the above equipment called?

Answer: Colposcopy machine
2. What are the indications?
Answer:
➢ As part of sexual assault for examination
➢ Further investigations if there is a cytological abnormality on pap smear
3. What could have happened in the pic below?

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 Answer: Sexual assault
4. Label the female genitalia below?
Answer:

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ANSWERS TO ESSAYS
1. A 3kg infant was born to a 16-year-old Para 1 mother. Labor was rather prolonged
lasting 14 hours and there was prolonged rupture of membranes for 24 hours.
APGAR score was 9/10. 2 days after birth the child developed failure to feed,
lethargy, jaundice, and hypotonia.
a) What is the most likely diagnosis?
Answer: Early onset Neonatal sepsis

b) List four investigations that must be done in this child to help you confirm the
diagnosis?
Answer:
➢ Full blood count with differential
➢ Blood culture
➢ Lumbar puncture for CSF biochemistry, microscopy, culture and sensitivity
➢ Urine microscopy, culture and sensitivity

c) What is your management plan?

Answer:
➢ Admit child
➢ Supportive care
o Insert IV line.
o Provide warmth (maintain temperature 36.5 to 37.5oC).
o Start oxygen by hood or mask 2-3 liters.
o Assess peripheral perfusion (pulses, capillary refill time and skin
color) and Infusion with normal saline or ringer’s lactate 10ml/kg over
5-10 minutes. Repeat the same 1-2 times over the next 30-45 minutes,
if perfusion remains poor start dopamine.
o Treat hypoglycemia by infusing 2ml/kg of 10% dextrose.
o Initial enteral feeds if no abdominal distension and when baby is
hemodynamically stable. Feed with mother’s milk. Consider
parenteral nutrition if baby is not expected to receive enteral feeds for
prolonged period.
o Administer vitamin K 1mg IM if not given.
o Transfuse packed cells if hematocrit (<35-45%).
➢ Antibiotics: Benzyl penicillin (50 000 U/Kg QID IV)/ampicillin (100-
200/kg/day in 4 divided doses) and gentamicin (7.5 mg/kg IV OD or in two

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 divided doses) for 7 to 10 days as culture results are being waited for,
antibiotics should be tailored to culture and sensitivity reports

2. Emmanuel, a month-old infant, born at term, is admitted for jaundice that

appeared on day 6 after birth and has persisted. He is alert, sucks well and has no
fever. His hemoglobin is 15g/dl with total serum bilirubin of 250 micromol/l.
a) Write down 2 important questions you would ask the mother with regards to
the jaundice
Answer:
➢ Any similar illness in the previous pregnancies
➢ Is the child passing pale stool and dark urine?

b) Write down 2 differential diagnoses

Answer:
➢ Breast milk jaundice
➢ Obstructive jaundice secondary to biliary atresia

c) Write down your management

Answer:
➢ Management is dependent on the cause
➢ Admit patient
➢ Continue breastfeeding
➢ Investigate: Blood typing/grouping, FBC, Coombs test, direct bilirubin,
abdominal ultrasound
➢ Hydrate with IV fluids
➢ Phototherapy with blue light (430-490nm) if unconjugated
hyperbilirubinemia
o Monitor temperature and ensure adequate fluid intake
o Cover eyes and genitalia with gauze pad/aluminum foil and place
infant naked under lights
o Place machine 45cm from infant
o Remove eye pads during feeds and observe for conjunctivitis
o Turn infant every 2-3 hours
o In severe jaundice check the Total serum bilirubin 3 hourly
➢ Exchange transfusion for rapidly rising bilirubin levels
➢ Consult surgeons if cause is obstructive

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3. You walk into a mall with your 4-year-old toddler and she picks a pack of food
with the following nutritional information per 100g:
Protein: 10g, Maize 40g, Wheat flour: 50g, Millet flour: 25g, Olive oil: 4g, Palm
oil: 6g, Zinc: 11mg, Potassium 110mg, Vitamin A: 800mcg and folate: 230mcg
a) Work out the energy content of this pack
Answer:
1g protein/carbohydrates= 4kcal
1g fat= 9Kcal

Total protein= 10g

Total carbohydrates= 40g + 50g + 25g= 115g
Total fats= 4g +6g= 10g

Total calories from proteins= 10 x 4= 40kCal

Total calories from carbohydrates= 115 x 4= 460kCal
Total calories from fat= 10 x 9= 90kCal

Total calories in 1 pack= 40 + 460 + 90= 590kCal

Total energy content (in KJ) = 590 x 4.2= 2478Kj

b) Considering that your child’s daily caloric requirement is about 1600kcal,

how many packs would she require to cover this need?
Answer:
If 1 pack gives 590kCal
1600
Then for 1600kCal, number of packs= = 2.711
590
Therefore 3 packs are needed

4. A 13 months old toddler is admitted with acute diarrhea with severe dehydration.
He weighs 10kg.
a) Write down 5 clinical features you are likely to elicit
Answer:
➢ Alteration in consciousness: Lethargic or comatose
➢ Sunken anterior fontanelle
➢ Sunken eyes
➢ Dry mucus membranes
➢ Skin pinch goes back very slowly (>2 sec)

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 ➢ Tachycardia

b) What is the average percentage of his body weight loss?

Answer: more than 10% with severe dehydration

c) Outline the management of this patient

Answer:
➢ Rehydration: WHO plan C
o 100ml/kg Ringer’s lactate or ½ strength Darrows IV
▪ 300ml in the first 30 minutes
▪ 700ml in next 2hrs 30 minutes
o Monitor every 15 minutes to watch out for overhydration (check for
edema, heart rate and respiratory rate) and reassess after 3 hours and
reclassify hydration status
➢ Continue breast feeding if child still breast feeding
➢ Zinc 20mg/day for 2 weeks
➢ Educate mother on signs of overhydration and dehydration

5. George, a 6-month-old infant is admitted with pneumonia (first occurrence) and

is able to feed well. His mother tested positive for HIV. Write down 5 questions
you would ask the mother to assess the risk of HIV infection in George.
Answer:
➢ What was the mother’s viral load and CD4 count prenatally, intrapartum and
postnatally?
➢ When did she start antiretroviral treatment and is mother currently on
treatment?
➢ What was the mode of delivery: vaginal or cesarean section?
➢ Is George currently still exclusively breastfed, mixed fed or not breastfed?
➢ Is George on any prophylactic antiretroviral drugs?
➢ Was Nucleic acid test done on George at birth?

6. A 14-year-old girl, Nana Maliki presents to your hospital with a history of

breathlessness and swelling of her legs for the past one week. Some of the
physical findings include orthopnea and a pansystolic murmur heard best at the
apical area and radiating to the left axilla.
a) What is the complete diagnosis of this medical conditions in this girl?
Answer: Biventricular heart failure secondary to acute mitral regurgitation

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 b) Mention two other signs that could be found in this patient consistent with
the above diagnosis?
Answer:
➢ S3 heard sound
➢ Displaced apex heart beat
➢ Bibasal crepitations

c) Indicate 3 investigations you are going to do that will help you confirm your
diagnosis.
Answer:
➢ Chest X-ray
➢ Echocardiography
➢ Electrocardiography

7. An 8-month-old male baby presents to hospital for a third episode in 3 months,

of swelling of the hands and crying uncontrollably. Physical examination reveals
an irritable baby with bilateral swollen hands. Further examination revealed a
tinge of jaundice and splenomegaly.
a) What is your provisional diagnosis in this baby?
Answer: Acute dactylitis in a possible Sickle cell anemia patient

b) What 2 investigations would you request to confirm the diagnosis?

Answer:
➢ Sickling test
➢ Hemoglobin electrophoresis

c) What is the most likely way the child acquired this condition?
Answer: The condition is inherited and the inheritance pattern is autosomal
recessive meaning one can only get the condition if both parents are either
carriers or sufferers of the condition

d) Write 5 complications that can occur in this condition?

Answer:
➢ Cerebrovascular accident: Transient ischemic attacks and stroke
➢ Neurosensory hearing loss
➢ Retinal hemorrhage

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 ➢ Retinal detachment and blindness
➢ Acute chest syndrome
➢ Recurrent pneumonias
➢ Respiratory failure
➢ Pulmonary infarction
➢ Pulmonary hypertension
➢ Myocardial infarction
➢ Arrhythmias
➢ Heart failure and cor-pulmonale
➢ Cholelithiasis
➢ Isosthenuria
➢ Priapism and impotence
➢ Menstrual irregularities, infertility and recurrent abortions
➢ Delayed puberty
➢ Osteomyelitis
➢ Avascular necrosis of head of femur and humerus
➢ Recurrent leg ulcers
➢ Delayed growth development and musculoskeletal deformities

8. A 2-year-old boy presents to 1st level referral hospital where you are working
with history of swelling of the feet for the last one week. There is also history of
diarrhea for 3 weeks prior to admission and a peeling rash on the feet of the child.
The child is the first born. Currently mum is 8 months pregnant. Preliminary
investigation reveals the following:
➢ Potassium: 3.1mmol/l
➢ Sodium 134mmol/l
➢ Chloride 104mmol/l
➢ Blood sugar 1.8mmol/l
a) What is the most likely diagnosis?
Answer: Severe acute malnutrition

b) List 4 diagnostic features that you will look for as you examine the child that
will help you make a more conclusive diagnosis of this condition
Answer:
➢ Decreased mid-upper arm circumference (<11.5 cm)
➢ Weight/height <-3 Standard deviation

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➢ Muscle wasting and decreased subcutaneous tissue: prominent outline of ribs
and sternum, baggy pants appearance
➢ Bilateral pedal edema and signs of dehydration (increased capillary refill time
>3 sec, alterations in level of consciousness-drowsiness, lethargy, apathy,
cold peripheries, weak thready rapid pulse, hypotension)
➢ Hair changes: thin, sparse, brittle, brown/reddish discoloration, flag sign
➢ Eye signs: bitot spots, dry conjunctiva, corneal clouding, corneal
ulceration/keratomalacia
➢ Oral changes: cheilosis, angular stomatitis, papilla atrophy on tongue,
abdominal distension, hepatomegaly
➢ Skin changes: desquamation, hypo- and hyperpigmentation, ulceration,
exudative lesions, dryness of skin, crazy pavement and flaky paint dermatosis
➢ Nail changes: brittle spoon shaped

c) Indicate any abnormalities in the above given investigation result

Answer:
➢ Decreased potassium (normal= 3.5 to 5.5 mmol/L)
➢ Low glucose level (hypoglycemia <3 mmol/L)

d) What 5 things affecting this child would likely cause mortality within 24
hours of admission unless taken care on admission?
Answer:
➢ Hypoglycemia
➢ Hypothermia
➢ Dehydration
➢ Electrolyte imbalance
➢ Infection

e) What 5 steps are you going to take immediately to save the life of this child?
Answer:
➢ Manage hypoglycemia
o 5ml/kg of 10% dextrose IV
o Start 2 hourly feeds with F75 (11ml/kg/feed if edema not severe and
8-9 ml/kg/feed if severe)
o Check blood glucose every 30 minutes until glucose level is normal
and stabilizes, if hypoglycemic repeat 5ml/kg 10% dextrose

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➢ Manage hypothermia (Axillary temp <35oC or rectal temp <35.5oC, if <32oC
it is considered severe hypothermia)
o Keep the child warm (cloth child, provide heat using overhead warmer,
skin contact and heat convectors)
o Start 2 hourly feeds with F75 (Warm feeds)
o For severe hypothermia
▪ Give humidified oxygen
▪ Give 5ml/kg of 10% dextrose IV
▪ Rewarm child slowly
▪ Start feeds with F-75
o Monitor temperature 30 mins if using a heater otherwise monitor
hourly
➢ Manage dehydration: rehydrate over 12 hours
o If child in shock give 15ml/kg of half-strength Darrows with 5%
dextrose or Ringer’s lactate with 5% over 1 hour and monitor
hydration status (pulse should decrease, respiratory rate should
decrease, capillary refill time should improve and urine should be
passed)
o If still in shock repeat 15ml/kg in next 1 hour.
o After infusion or if child not in shock give 5ml/kg every 30 minutes of
ReSoMal orally or by Nasogastric tube for first 2 hours and then for
next 10 hours alternate ReSoMal with F-75 hourly (same amount
5ml/kg)
➢ Manage electrolyte imbalance (hypokalemia)
o ECG monitoring
o If diarrhea present 600mg OD slow K
o Start potassium chloride infusion at 0.3-0.5 mEq/kg/hour
o Give potassium 4mmol/kg/day for 2 weeks
➢ Treat infection/antibiotics (7 days)
o Uncomplicated: amoxicillin (50-100mg TDS PO)
o Septicemia: IV ampicillin (100-200/kg/day QID) or X-pen (100mg-
200mg/kg/day QID) + Gentamycin (7.5mg/kg OD or in 2 divided
doses)
o If no improvement occurs within 2 days change to IV cefotaxime
(100mg/kg/day TDS) or ceftriaxone (50mg/kg/day BD)

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9. A 14-year-old girl called DJ is referred from a health center 10km from the
hospital where you are working with history of east fatiguability, fever and
bleeding from the nose for one month. On examination, you find that the girl is
very pale, has purplish bumps in the mucus membrane of the oral cavity and is
toxic looking. The temperature is 39OC.
a) What 4 critical investigations are you going to do to help you make a definitive
diagnosis on this child?
Answer:
➢ Full blood count with differential: pancytopenia, thrombocytopenia
➢ Peripheral smear to look for blast cells
➢ Clotting profile: aPTT, Bleeding time, prothrombin time for DIC
➢ Chest X-ray to check for any mediastinal masses as well as CT scan
➢ Bone marrow aspirate
➢ Blood culture

b) What are 2 most likely differential diagnosis that would explain the patient’s
condition
Answer:
➢ Idiopathic thrombocytopenic purpura
➢ Leukemia

c) List 6 other signs that you would look for on examination of the patient that
would help you diagnose the patient’s illness.
Answer:
➢ Hepatomegaly
➢ Splenomegaly
➢ Generalized lymphadenopathy
➢ Bone pain
➢ Jaundice, Gum hypertrophy
➢ Cranial nerve palsy, papilledema, meningism

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10.A 16-month-old toddler (girl) is brought to the children’s clinic, because the
mother is concerned that the child’s growth and development is suboptimal
compared to her older siblings. Upon further questioning she agrees to the child
having recurrent upper respiratory infections, occasional bouts of loose stool and
fevers that have been treated as “malaria” by the private doctor. On examination
she weighed 7.5kg, her height was 80cm, she had slight pallor with generalized
lymphadenopathy, sores (white patches) in the mouth, CVS was normal and per
abdomen there was hepatosplenomegaly with no pedal edema.
a) Give 4 possible most likely differentials for the described condition.
Answer
➢ HIV infection with TB co-infection
➢ Severe acute malnutrition
➢ Infectious mononucleosis
➢ Enteric fever

b) List 4 investigations that you would do to come up with a definitive diagnosis

of the condition of this child?
Answer:
➢ HIV test
➢ Full blood count with differential
➢ Gastric lavage or sputum for geneXpert
➢ Peripheral smear

c) If you are to stage this condition, what stage of disease is the child in.
Answer: HIV stage 3

11.A 2-year-old girl presents to your hospital and after investigations the CSF
revealed the following results
Appearance: cloudy
WBC: 120cells/mm3- polymorphs 80% and lymphocytes 20%
RBC: 50cells/mm3
Gram stain: gram negative coccobacilli
Ziehl Neelsen stain: no organism

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 Protein: 0.75g/l
Sugar: 1.6mmols/l
a) List 4 cardinal abnormalities that you see in this CSF result
Answer:
➢ Pleocytosis with predominance of neutrophils but also presence of
lymphocytes is also an abnormality
➢ Presence of gram-negative coccobacilli
➢ Increased proteins
➢ Low blood sugar

b) What is the causative organism?

Answer: Hemophilus influenza type B

c) What specific measure would you have taken to prevent this illness in the
child?
Answer: immunization against Hemophilus infleunza at 6 weeks, 10 weeks
and 14 weeks

d) List 4 long term complications associated with this condition?

Answer:
➢ Hearing loss
➢ Learning disability/mental retardation
➢ Cranial nerve palsy
➢ Focal neurological deficits

12.This is a Hemogram of a patient you have just seen at your hospital:

WBC: 5.7 x 109/L
RBC: 2.8 x 1012/L
Hb: 5.3g/dl
MCV: 49fl
MCH: 18.3pg
Platelets: 272 x 109/L
a) List 4 abnormalities that you see in this Hemogram

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 Answer:
➢ Low red blood cell count
➢ Low hemoglobin
➢ Low mean corpuscular volume
➢ Low mean hemoglobin concentration

b) What is the diagnosis based on the Hemogram?

Answer: Microcytic hypochromic anemia

c) Give 3 possible causes of this condition.

Answer:
➢ Iron deficiency anemia
➢ Sideroblastic anemia
➢ Thalassemia

13.An 8 weeks old infant presents to your admission ward with history of cough for
4 days and difficult breathing. On examination, the infant is well nourished and
weighs 6kg. However, the infant is dyspneic, tachypneic with subcostal
recession. Respiratory rate is 70/min. Temperature is 37.8oC. There is some
cyanosis, examination of the chest reveals a bilateral ronchi best heard at the end
of expiration and a few crepitations.
a) What is the most likely diagnosis?
Answer: Bronchiolitis

b) List 3 likely causative organisms

Answer:
➢ Respiratory syncytial virus
➢ Parainfluenza virus
➢ Adenovirus

c) Describe in one or two sentences the pathophysiological process of this

condition
Answer: Viral infection causes inflammatory obstruction (edema and mucus)
of bronchioles. There may be bronchiolar spasms which also further increases
the obstruction. During expiration airways collapse and there is air trapping.
Obstruction affects expiration more than inspiration.

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 d) What is your management?
Answer:
➢ Admit patient
➢ Provide moist oxygen (keep oxygen saturation more than 92%), continuous
positive airway pressure (CPAP) or assisted ventilation if there is respiratory
failure
➢ Nasal bulb suction
➢ Hydration with IV fluids
➢ Position at 30-40o
➢ Nebulized bronchodilators (albuterol and ipratropium) and steroids may help
some patients
➢ Aerosolized ribavirin if available

14.A 2 days old baby comes into the neonatal unit with history of developing
jaundice since a few hours after birth. On examination, you notice that the child
is weak, hypotonic, deeply jaundiced and pale. He is the 2nd born, the first sibling
did not suffer from this condition.
a) What is the most likely full diagnosis of this illness in the neonate?
Answer: Hemolytic disease of the newborn

b) List 4 investigations that you are going to do in order to confirm the diagnosis
Answer:
➢ Direct Coomb’s test
➢ Full blood count
➢ Peripheral smear
➢ Serum bilirubin

15.An 11 months old infant presents to your admission ward convulsing. There is
history of 2 former such episodes of convulsing. According to the mother, the
convulsions involve the whole body and last about 3 minutes. The child has
temperature 39oC.
a) What is the most likely diagnosis?
Answer: Febrile convulsion

b) List 3 disease condition that would possibly have caused this condition
Answer:
➢ Severe Malaria

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 ➢ Sepsis
➢ Pneumonia

c) Indicate 2 cardinal investigations that would help you manage this child.
Answer:
➢ Malaria parasite slide
➢ Full blood count with differential

16.Joanna a 3-year-old toddler, is brought to AMEU with history of diarrhea and

vomiting for the past 3 days. On examination, she is lethargic, too weak to take
fluid orally and passed scanty urine all day. Her weight is 14kg
a) What plan are you going to apply in rehydrating Joanna?
Answer: WHO PLAN C

b) What fluid are you going to use in treating this little girl?
Answer: Ringer’s lactate or ½ strength Darrows

c) Give details of the plan you are going to use in rehydrating Joanna
Answer:
➢ Give 100ml/kg Ringer’s lactate IV in 3 hours (1400ml in 3 hours)
- Give 420ml in 30 minutes then
- Give 980ml in 2hours 30 minutes
➢ Reassess every 15 minutes if hydration status not improving increase speed
of infusion.
➢ Reassess after 3 hours and re-classify hydration status, if hydrated switch to
appropriate plan.

17.Hamaundu a 4-year-old-boy, is brought to the OPD with edema of both hands

and feet and sores in the mouth. He weighs 8.5kg.
a) Assign the nutritional status to Hamaundu based on welcome classification
Answer:
Expected weight for a 4-year-old child = 2 x (4 + 4) = 16kg
Hamaundu’s weight= 8.5 kg
8.5
W/A percentage= × 100= 53.1% of expected weight
16
Hamaundu has Marasmic Kwashiorkor

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 b) How many milliliters of F75 would you need to cover his caloric requirement
estimated at 1500kcal/24 hours?
Answer:
Each 100ml of F-75 has 75kCal
If 100ml F-75 gives 75Kcal, volume needed for 1500kcal= (1500 x 100)/75=
2000ml
Therefore, 2000ml per day of F75 is needed

18.Kasongo a 10-year-old boy of Kasisi, presents with fever of 3 weeks duration.

He was treated for malaria a week ago despite a negative malaria thick smear. He
has remarkably lost weight and is complaining of abdominal pain associated with
constipation. The mother says that he loses his memory at times and would be
incoherent in his speech.
a) Write down your differential diagnosis
Answer:
➢ Enteric fever
➢ Viral hepatitis
➢ Abdominal Tuberculosis
➢ Leukemia
➢ Lymphoma

b) What is your most likely diagnosis?

Answer: Enteric fever

c) List investigations you would carry out

Answer:
➢ Full blood count with differential
➢ Peripheral smear
➢ Blood culture
➢ Urine microscopy/culture and sensitivity
➢ Widal antigen test
➢ Hepatitis profile
➢ Liver function tests and enzymes
➢ Abdominal x-ray
➢ Abdominal ultrasound
➢ Urea, electrolytes, creatinine
➢ Bone marrow culture

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19.A 7-year-old girl is brought to LMGH with a history of recent bedwetting and
unexplained weight loss. No history of tuberculosis contact. Her rapid test for
HIV is negative
a) What is your differential diagnosis?
Answer:
➢ Type 1 Diabetes mellitus
➢ Diabetes insipidus
➢ Urinary tract infection
➢ Chronic renal

b) Write down 2 more symptoms that may be associated with this condition
Answer:
➢ Polyphagia
➢ Polydipsia

c) List down 3 investigations to ascertain your diagnosis

Answer:
➢ Random blood sugar
➢ Urinalysis, Urea, Electrolytes and creatinine
➢ Ultrasound of abdomen

20. Martha, a 4-year-old girl has had a history of bony pains on and off for 2 weeks.
On examination, she is febrile, underweight, pale and in respiratory distress. She
has cervical and axillary lymphadenopathy.
a) What further questions would you ask to help determine the diagnosis?
Answer:
➢ Any recurrent abdominal pain?
➢ Any history of yellowing of eyes?
➢ Any personal or family history of sickle cell disease?
➢ Any joint swelling?
➢ Any recurrent respiratory infections? Pulmonary TB?
➢ Any bleeding?
➢ Any TB contact?
➢ HIV status

b) Give 2 differential diagnoses?

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 Answer:
➢ Acute leukemia
➢ Acute chest syndrome in Sickle cell disease

c) What investigations would you do to help ascertain your diagnosis?

Answer:
➢ Sickling test
➢ Full blood count with differential
➢ Peripheral smear
➢ Chest X-ray
➢ Lymph node biopsy

21.Mwila is referred from Kasama for a huge spleen. She has had few episodes of
abdominal pain which the mother attributed to worm infestation. On examination
she is underweight with a tinge of jaundice. The night of her admission, she bled
profusely from the nose and required nasal packing.
a) Write down 3 possible diagnoses?
Answer:
➢ Hyperreactive malarial splenomegaly (Tropical splenomegaly syndrome)
➢ Acute leukemia
➢ Viral hepatitis
b) List down specific investigations to ascertain your diagnosis
Answer:
➢ Blood investigations
- Rapid diagnostic test for malaria
- Peripheral smear to rule out severe malaria, acute leukemia
- Hepatitis viral panel
- Full blood count and ESR
➢ Bone marrow aspirate

22.These are pictures of the same girl in question 20.

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 a) Write down your findings
Answer:
➢ Webbed neck
➢ Low posterior hair line
➢ Low set ears

a) What is your diagnosis?

Answer: Turner syndrome (monosomy X)

c) Choose the most likely karyotype

(1) XY
(2) XO
(3) XYY
(4) 47, XX, +t (14q21q)

23.A 24-month-old toddler, well nourished, is admitted with acute diarrhea and
severe dehydration. He weighs 9.6kg
a) What is his expected weight?
Answer:
Expected weight= 2 x (age + 4)
Expected weight= 2 x (2 + 4)
Expected weight= 2 x 6
Expected weight= 12kg

b) What is his body weight percentage loss?

Answer:

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 12−9.6
Body weight percentage loss= × 100= 20%
12

c) Work out the fluid amount required to cover his deficit?

Answer:
Deficit (100ml/kg) = 100 x 9.6
Deficit= 960ml of Ringer lactate (or ½ strength Darrows solution in 5%
dextrose)
288ml to run 30 minutes and 672ml to run in 2 hours 30 minutes

24.You are called to review a newly diagnosed insulin dependent diabetes mellitus
case. The patient is receiving insulin intravenously and his morning urine analysis
is as follows:
SpGr: 1.025, Protein: +, pH: 6.0, Blood: negative, Ketones: ++++, Glucose:
Negative
a) Explain in few words what may have led to the presence of ketones and the
absence of glucose in urine.
Answer: This could probably be caused by a high dosage of insulin causing
hypoglycemia and hunger. The response of the body to hunger leads to
ketogenesis as an alternative source of energy therefore ketones would be
present in urine and glucose absent

b) In your opinion, what would be the blood sugar levels in this patient? Low,
normal, or high?
Answer: The sugar would low

c) How would you manage this patient?

Answer:
a. Check random blood sugar
b. If blood sugar low, give 5ml/kg of 10% dextrose
c. Adjust insulin dosage and monitor glucose 30 minutes to hourly
d. Give intravenous fluids- DNS
e. Monitor for ketones and glucose in urine

25.A 24-month-old girl presents to hospital with history of diarrhea and vomiting
for 4 days and anorexia for 2 weeks. The mother has recently fallen pregnant but
she has recently separated from her husband. On examination the child is

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 miserable, apathetic and has bilateral pedal edema and dermatosis. Her weight is
7.2kg. Her muscles are wasted.
a) What is the diagnosis?
Answer: Severe acute malnutrition

b) List 5 critical conditions that you must manage in order to save the life of this
child within the first 48 hours
Answer:
➢ Hypoglycemia
➢ Hypothermia
➢ Dehydration
➢ Electrolyte imbalance
➢ Infection

c) What is the expected weight for age for this child?

Answer:
Expected= 2 x (4 + 2)
Expected= 12kg

d) Give differentials that need to be ruled out every time you have such a case.
Answer:
HIV infections
Tuberculosis
Pneumocystis Jirovercii pneumonia
Malaria
Vitamin A deficiency
Severe anemia
Candidiasis
Worm infestation

26.A boy is brought into hospital with difficulties of breathing and swelling of the
legs. The patient indicates that he is unable to sleep supine on the bed as he runs
out of breath. He also indicates that he develops breathlessness after walking only
a few steps. On examination, the boy has a pansystolic murmur in the mitral area
radiating to the left axilla. He also has anemia and fever as well as splenomegaly.
a) Give your differential diagnosis
Answer:

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 a. Biventricular heart failure secondary to acute rheumatic heart disease
b. Biventricular heart failure secondary to acute mitral regurgitation New
York heart association class III
c. Nephrotic syndrome
d. Infectious mononucleosis

b) Line up investigations based on your differential diagnosis

Answer:
a. Anti-streptolysin O antibody titers for rheumatic heart disease
b. Throat swabs for culture
c. Chest X-ray may show cardiomegaly, pulmonary edema
d. ECG and ECHO for the heart failure
e. Urinalysis for proteinuria

c) Which organisms are usually associated with this condition?

Answer: Lancefield Group A Beta hemolytic streptococcus

d) Which one is the most affected age group and why?

Answer: children between 5-15 years of age are most affected because this
group is most susceptible to streptococcal throat infections with M serotypes
of group A beta hemolytic streptococcus.

e) How would you manage this child?

Answer:
a. Admit patient
b. Keep child propped up
c. Administer humidified oxygen
d. Sedation with morphine sulfate 0.1 to 0.2 mg/kg/dose SC every 4 hours
as needed or Phenobarbital 2 to 3mg/kg/dose PO or IM
e. Give furosemide 1-2mg/kg/day IV in 2-3 divided doses
f. Give digoxin and captopril/enalapril according to age of the child
g. Monitor input and output chart, daily weighing and salt and fluid
restriction
h. Aspirin 100mg/day in 4 to 6 divided for 2-4 months with Prednisolone
2mg/kg/day in 4 divided doses
i. Cover on antibiotics Benzathine penicillin IM one dose followed by
oral penicillin for 10 days

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27.A 13-year-old girl is brought into the emergency room with history of difficulty
breathing since the previous night. She is a known sickle cell disease patient who
was lost to follow up. On examination the girl is in severe respiratory distress
with a respiratory rate of 70/min, with sub-costal and intercostal recession. Her
temperature is 38.7oC. Her hemoglobin is 7g/dl and her oxygen saturation using
a pulse oximeter is 76.
a) What are the 2 key differential diagnoses for the condition that the patient is
presenting with?
Answer:
a. Acute chest syndrome
b. Congestive heart failure

b) List 3 investigations you would do in relation with this condition.

Answer:
a. Chest X-ray
b. Full blood count with differential count
c. Blood culture

c) How would you manage this patient?

Answer:
a. Admission
b. Hydration: IV fluids (1.5 x maintenance)
c. Oxygen support- ventilation (CPAP may be necessary)
d. Antibiotics
i. Ceftriaxone and Erythromycin-250mg BD for 10 days
e. Transfuse packed cells (15-20 ml/kg) slowly over 4 hours and give
1mg/kg Furosemide stat for anemia
f. Consider partial exchange transfusion

28.A3 and a half year old female child presents with noisy breathing and fever of 6
hours duration. Prior to this she had been perfectly well. She was born at LMGH
at full term with a birth weight of 3.9kg. There were no neonatal problems. Her
routine vaccinations are up to date and weight gain has been adequate. There is
no family history of note. On examination the patient looked very ill with
temperature 39.7oC, pulse 110/min, BP 110/78, dribbling of saliva from one

462
 corner of the mouth, intercostal and subcostal indrawing with a clear chest. Blood
investigations reveal
Hb- 12g/dl
WBC- 20 x 109/L (polymorphs-84% and lymphocytes-12%)
Sodium 135mmol/L
Potassium 3.5 mmol/L
Urea 4.6 mmol/L

Urinalysis was normal

a) What is the most likely diagnosis?
Answer: Acute epiglottitis

b) What is the most likely causative organism?

Answer: Hemophilus influenza type B

c) Name 3 management measures you are going to institute in this child

Answer:
a. Airway and breathing management
ii. Controlled nasotracheal intubation (consult ENT surgeon)
iii. Humidified oxygen by hood
iv. Keep child in sitting position
v. Avoid examination of the throat
b. Steroids: Dexamethasone 0.3-0.5mg/kg repeat 8 hourly for 2-3 days
max
c. Antibiotics (Third generation cephalosporins): Cefotaxime 100-
150mg/kg/day IM in 2 injections for 5 days

29.Mutinta a 10-year-old girl presents to hospital with history of cough and swelling
of legs for 4 days. On examination she is dyspneic at rest, has bilateral pedal
edema and hepatomegaly. Examination of the heart reveals a pan-systolic
murmur, radiating to the axilla and mid-diastolic murmur in the mitral area. She
also has a diastolic murmur in the aortic area.
a) What is the full diagnosis of this child’s condition?
Answer: Biventricular heart failure secondary to mitral regurgitation with
aortic regurgitation New York heart classification class IV

b) List the valve structural problems that this child has

463
 Answer:
Mitral regurgitation
Aortic regurgitation

c) Indicate the four measures you will take in order to manage the acute phase
of this child
Answer:
a. Administer humidified oxygen
b. Sedation with morphine sulfate 0.1 to 0.2 mg/kg/dose SC every 4 hours
as needed or Phenobarbital 2 to 3mg/kg/dose PO or IM
c. Give furosemide 1-2mg/kg/day IV in 2-3 divided doses
d. Give digoxin

d) What long term treatment would you institute in order to prevent further
deterioration of this child’s condition?
Answer: Continuous monthly prophylaxis with injections of benzathine
penicillin 1,200, 000 IU or Pen V 250mg BD PO can be given. For those
allergic to penicillins erythromycin 250mg BD PO is given until the age of 18
or 21.

30.Nalishebo is a 3-year-old girl who presents with edema and is underweight. The
following are her lab results:
Urea 2.5 mmol/L
Sodium 128 mmol/L
Potassium 3.5 mmol/L
Serum albumin 28g/L
Serum cholesterol 2mmol/L
Urinalysis:
Protein 2+
Blood 1+
Leucocytes: Numerous
a) What 2 other important physical features should be present in this child?
Answer:

b) What is the most likely diagnosis?

Answer: Nephrotic syndrome

464
 c) What other diagnoses should be considered and treated?

31.A 10-year-old boy, Judge Siachinji, whose parents just moved to live in Lusaka
from Siavonga presented at LMGH with terminal hematuria
a) What is the likely diagnosis for this boy?
b) What investigations would you carry out to confirm the diagnosis in this boy
and what would be the finding consistent with the diagnosis?
c) Write down 2 complications from this condition that the boy may develop if
left untreated?
d) How would you treat this child?

32.Zambia ranks one of the countries in Sub-Saharan Africa with the worst infant
and childhood mortality rates. One of the ways to avert this problem is to
reinforce the aims and objectives of the under-five clinics
a) State the major aims of under-five clinics in Zambia as captured on the under-
five card?
b) List the preventable disease from which a Zambian child is immunized on the
current EPI?

33.An 8-year-old girl presented at LMGH with restlessness, heart palpitations and
was rather dazed in general appearance. Investigations showed an impaired
glucose tolerance test with:
Total serum thyroxin 186nmol/L (normal range 10-30)
Free T4 12.5pmol/L (normal range 9.4-24.5)
Free T3 11.8pmol/L (normal range 2.9-8.9)
Serum TSH <0.1Mu/L (normal range 0.7-2.5)

a) What is the most likely clinical diagnosis for this patient?

b) What is the pathophysiological basis of the heart palpitations and restlessness
in this child?
c) Write down 2 other (physical findings) consistent with this condition you
would likely elicit on physical examination of this child?

465
34.A 9-year-old-boy is referred to LMGH from a mission Hospital for further
investigation. He had developed progressive abdominal distension over a period
of 7 months, and had reduced exercise tolerance compared to his peers.
Examination revealed a frightened but co-operative child who could only
understand Lozi. He was pale, anicteric and weighed 38 kg. Congestion of the
jugular veins was evident on deep inspiration. The pulse varied between 90-97
beats per minute and the heart sounds were normal. Auscultation of the chest
revealed a few coarse crepitations in the bases. The abdomen was uniformly
distended and invariably impossible to palpate for organomegaly. Shifting
dullness was easy to elicit and there was pitting oedema above both ankles.
a) What is your provisional diagnosis?
b) What 3 investigations would you ask for to support this diagnosis?
c) How would you mange this child?

35.Mwanja a 35-week-old infant, is admitted with some respiratory distress and

commenced on oxygen 3L/min via nasal prongs and the saturation is stubbornly
stuck at 85% despite increasing the oxygen flow. Mwanja is HIV negative and
his lungs are clear on auscultation. His hematocrit is around 65%
a) Give your differential diagnosis
Answer:
a. Tetralogy of fallot
b. Tricuspid atresia
c. Truncus arteriosus

b) Justify the hematocrit reading

Answer: the hypoxia causes a release of erythropoietin which stimulates
synthesis and release of erythrocytes resulting in polycythemia and a high
hematocrit, additionally, respiratory distress may also cause dehydration with
resultant hemoconcentration also contributing to the rise in hematocrit.

c) Line up the principles of management of this child

Answer:
➢ Medical management
• Administer oxygen
• Place child in Knee-chest position
• Sedation and pain relief with morphine

466
 • Increase systemic vascular resistance and reduce infundibular
spasms and right ventricular outflow obstruction:
- Propranolol
- Phenylephrine
• Alternatively, ketamine can be used to increase systemic
vascular resistance and provide sedation
• Correct acidosis with sodium bicarbonate
➢ Definitive management is by surgery at 6 months of age. In some infants
a surgical placement of an artificial tube between the subclavian and
pulmonary artery (Modified Blalock-Taussig shunt) or sometimes by
dilatation of the right ventricular outflow tract

36.A 4-month boy is brought to OPD for not gaining weight. He is exclusively
breastfed and mother tells that he sweats profusely while taking feed. He was
treated for bronchopneumonia at 2 months of age. On examination his weight is
4kg irritable child with a respiratory rate of 45/min and heart rate 140/min
d) What is the diagnosis?
e) How will you investigate?
f) List 3 complications of this condition

37.Mweemba, 10-year-old girl is brought into the emergency room with history of
difficulty breathing since the previous night. She is a known sickle cells disease
patient who has been receiving monthly blood transfusions due to cerebral
vascular accident. She is in severe respiratory distress with a respiratory rate of
70/min, associated with subcostal and intercostal recession. Her temperature is
38.7oC, her hemoglobin is 7g/dl and her oxygen saturation is 76%.
a) What are the 2 key differential diagnosis for the condition that the patient is
presenting with?
Answer:
a. Acute chest syndrome
b. Pneumonia

b) Besides blood, list 2 further investigations you would carry out

Answer:
a. Chest X-ray
b. CT chest

467
 c) Comment on her hemogram: WBC 22,300 cells/mm3, MCV: 65.7fl, RDW-
CV 25%
Answer: This WBC is high due to reticulocytosis and infection, MCV is low
indicated a microcytic anemia and RDW is high indicating a wide variation in
sizes of the RBCs.

d) How would you mange this child?

Answer:
a. Admission
b. Hydration: IV fluids (1.5 x maintenance)
c. Oxygen support- ventilation (CPAP may be necessary)
d. Antibiotics
• Ceftriaxone and Erythromycin-250mg BD for 10 days
e. Transfuse packed cells (15-20 ml/kg) slowly over 4 hours and give
1mg/kg Furosemide stat for anemia
f. Consider partial exchange transfusion

38.Chola a 10-year-old, is admitted with a month-long history of headaches, low

grade fever on and off, he was kept in hospital for few days. 3 weeks ago and
treated for meningoencephalitis and showed some improvement. This time, he is
not able to talk coherently and displays squint. His appetite is poor and he lost
some weight.
a) Give 3 differential diagnoses
Answer:
g. Chronic meningitis

b) How would you investigate this boy?

Answer:

c) How would you mange chola?

468
39.Sakala, a 9-year-old is brought to AMEU with continuous generalized tonic
clonic seizures for the past half an hour. On examination, he is febrile with not
signs of meningeal irritation. He has scars of sore he developed as a result of
thorny bushes while setting rat traps. Other systems are unyielding
a) What is your differential diagnosis?
Answer:
a. Generalized tetanus
b. Sepsis

b) Give 2 bedside assessments that would help your diagnosis?

Answer:
a. Presence of Risus sardonicus and/or Opisthotonos
b. Spatula test: touching the posterior pharyngeal wall with a soft tipped
instrument and observing effect (positive: involuntary contraction of
the jaw, Negative: gag reflex)

c) What is the most likely diagnosis?

Answer: Generalized tetanus

d) List down your management principles of this condition

Answer:
a. Admit to intensive care unit
b. Limit stimulation
c. Airway management
d. Neutralization of free toxin with human tetanus immunoglobulin
e. Antibiotics: crystalline penicillin or metronidazole
f. Relief of spasms with benzodiazepines
g. Supportive care
• Temperature control
• Intravenous fluid
• NG tube feeds with high caloric protein diet
• Control of autonomic instability with IV magnesium sulfate
h. Immunization after recovery

40.A 3-day old neonatal with jaundice from the first day from the umbilicus to the
knee presents with lethargy, pallor and is irritable. You suspect Kernicterus.
a) What is the Kramer staging of this child?

469
 Answer: Kramer 3

b) Grade his kernicterus

Answer: Mild acute kernicterus

c) A coombs direct test was performed. Whose blood was it performed on?
Answer: The test was done on neonate’s blood

d) When phototherapy is instituted list 4 measures to take before and during the
procedure.
Answer:
Before:
➢ Cover the eyes and genitalia with gauze/foil and place infant naked under
lights (nappy untied)
➢ Ensure phototherapy unit is approximately 40cm from infant

During:
➢ Monitor temperature and ensure adequate fluid intake
➢ Turn the infant every 2-3 hours

After:
➢ Remove the eye pads during feeds and observe for conjunctivitis

f) List 2 general complications of phototherapy

Answer:
➢ “Bronzing” of skin especially when phototherapy is used for conjugated
hyperbilirubinemia
➢ Rases
➢ Loose stools
➢ Dehydration
➢ Hypo/hyperthermia

470
41.A 10-year-old known sickle cell patient presents with a history of difficulty
breathing that occurred since the last night as well as a history of several vaso-
occlusive crises over the past month.
a) List 2 ways to manage this patient other than IV fluids and analgesia
Answer:
➢ Oxygen
➢ Hydroxyurea
➢ Folic acid
➢ Blood transfusion
During one painful crisis the child presents with a fever and respiratory
embarrassment
b) List 2 differentials
Answer:
a. Acute chest syndrome
b. Pneumonia

c) List 2 possible organisms

Answer:
a. Mycoplasma pneumoniae
b. Streptococcus pneumoniae

d) List 2 groups of antibiotics for your differentials

Answer:
a. Macrolides e.g. azithromycin
b. Third generation cephalosporins/Penicillins

42.A 3-week-old neonate that has been thriving is brought to OPD, the mother
complains that the neonate often throws up after feeding then continues to eagerly
feed, a preliminary full blood count is normal aside mild anemia and the child is
not opening bowels as frequent anymore.
a) What is your differential diagnosis?
Answer:
Pyloric stenosis
Antral or pyloric web
Tracheosophageal fistula
Duodenal atresia

471
 b) How would you confirm your diagnosis clinically?
Answer: On inspection of abdomen visible peristalsis can be seen and on
palpation an olive-shaped mass is felt in the right upper quadrant

c) List 3 biochemical derangements in this condition

Answer:
c. Hypochloremia
d. Hypokalemia
e. Metabolic alkalosis

d) What gender does this condition occur mostly in?

Answer: Males

43.A 5-year-old presents with a mandibular mass and dental malalignment. A biopsy
from the mass taken for histopathology shows a starry sky appearance.
a) What is the most likely diagnosis?
Answer: Burkitt Lymphoma (small non cleave B-cell lymphoma)

b) What investigations should be carried out prior to chemotherapy?

Answer
a. Urea, Electrolyte (potassium, calcium, phosphorus, magnesium) and
creatinine
b. Serum uric acid
c. Full blood count with differential
d. Clotting profile

d) What 2 crucial measure should be taken prior to chemotherapy?

Answer:
➢ Maintain adequate intravenous fluid hydration 24 hours before
administering chemotherapy
➢ Prophylactic administration of allopurinol and aggressive hydration with
alkalinization of the urine.

e) What is the most common complication related to chemotherapy?

472
 Answer: Tumor lysis syndrome

f) What biochemical derangements are associated with complication?

Answer:
➢ Hypocalcemia
➢ Hyperkalemia
➢ Hyperphosphatemia
➢ Hyperuricemia

44.A 4-month old child not gaining weight presents with a grade 3 pansystolic
murmur at the left lower sternal border with a loud second heart sound at the
pulmonary area. Echo shows tetralogy of Fallot.
a) List 4 clinical features in this condition
Answer:
➢ Poor feeding
➢ Cyanosis: child may present with hypercyanotic “tet” spells characterized
by sudden cyanosis, restlessness, agitation and inconsolable crying. As
child grows, they learn to squat during cyanotic spells to compensate.
➢ Anoxic spells (paroxysmal attacks of dyspnea)
➢ Finger clubbing
➢ In prolonged cyanotic spells cause
• Unconsciousness
• Seizures
• Hemiparesis

b) List 2 hematological and 2 radiological features of the condition

Answer:
➢ Hematological:
• Mild anemia, thrombocytosis
• Polycythemia
➢ Radiological:
• Boot shaped heart on chest X-ray
• Dark lung fields with absent lung markings
• Pulmonary stenosis, right ventricular hypertrophy, ventricular
septal defect and overriding of the aorta on ECHO
• ECG shows right axis deviation

473
 c) Write down 2 complications
Answer:
➢ Heart failure
➢ Bacterial endocarditis
➢ Pulmonary regurgitation
➢ Arrhythmias
➢ Thrombi and Cerebrovascular events

d) What 4 drugs are used to manage this condition and reasons why?
Answer:
➢ Analgesics e.g. Morphine used to relax the pulmonary infundibulum,
reduce ventilatory and for sedation.
➢ Prostaglandins e.g. alprostadil: prostaglandin E is used to keep the ductus
arteriosum open enabling blood to shunt back from the left to the right
➢ Beta blockers e.g. Propranolol are used to prevent cyanotic spells, they
reduce ventricular infundibular spasms
➢ Alpha adrenergic agonists (phenylephrine) to increase systemic vascular
resistance reducing shunting of blood from the right side to the left.

45. A 3-year-old with a 5-day history of a febrile illness and headache. He is treated
with Pen V at a local clinic and showed some improvement however he fitted
once before admission.
a) Write down 3 differentials
Answer:
➢ Meningitis
➢ Febrile convulsion
➢ Severe malaria

b) List 4 investigations
Answer:
➢ Full blood count with differential for infections
➢ Rapid diagnostic test for malaria and Malaria parasite slide
➢ Lumbar puncture for CSF biochemistry, microscopy, culture and
sensitivity
➢ Blood culture

474
 c) How would you manage this child?
Answer:
➢ Admit patient
➢ Check blood glucose and treat hypoglycemia (5ml/kg of 10% dextrose)
➢ Antibiotics: Crystalline penicillin 100,000 IU/Kg QID + IV cefotaxime
100mg/kg QID for 10-14 days as culture results are awaited
➢ Monitor vitals, pupillary reflexes and level of consciousness 4 hourly
➢ Fit chart
➢ IV fluids- 2/3 of fluid requirement
➢ Antipyretic: paracetamol IV
➢ Treat seizures if present: diazepam 0.3 mg/kg/dose IV or 0.5mg/kg/dose
rectal

46.A 2-year-old wakes up failing to walk and was fine the previous day. Physical
examination reveals loss of pain in the lower limbs up to the knees.
a) What is your working diagnosis?
Answer: Acute flaccid paralysis

b) What are your differentials?

Answer:
➢ Guillain Barre syndrome
➢ Poliomyelitis
➢ Transverse myelitis
➢ Tick paralysis

c) What important questions will you ask for in your history?

Answer:
➢ History of respiratory illness, pharyngitis or Gastroenteritis (diarrhea and
vomiting)
➢ Presence of fever, generalized non-throbbing headache, sore throat,
anorexia, nausea, vomiting, muscle aches
➢ Bladder (urinary retention) and bowel complaints: absent or present
➢ Immunization history against polio and any recent vaccines given to the
child

475
 d) What’s your worry about this child?
Answer: Respiratory failure or paralysis of respiratory muscles as paralysis
ascends with disease progression

47.An 11-year-old presents with a nagging itchy skin lesion on the medial malleolus
for 2 months that opened up and keeps growing. Preliminary investigations
reveal:
WBC 13, 000/mm3
Hb g/dl
MCV 75fL
RBS 9.6 mmol/l

a) Give 2 differentials
Answer:
➢ Venous ulcer
➢ Buruli ulcer

b) What is the most likely diagnosis?

Answer: venous ulcer secondary to sickle cell disease

c) List one conclusive test you will do to confirm your diagnosis

Answer: Hemoglobin electrophoresis

d) List 4 bullets on management

Answer:
➢ Pus swab for culture microscopy/culture and sensitivity
➢ IV fluids, crossmatch and transfusion (Hb target >10g/dl)
➢ Antibiotics
➢ Daily wound cleaning

48.A new born to an HIV mother is brought to your pediatric department. The
mother started ART at 8 months gestation and is exclusively breasting the infant.
a) What prophylaxis should the neonate be put on?
Answer: AZT + 3TC + NVP for 12 weeks

b) What do you need to ensure/follow up on with that prophylaxis?

Answer:

476
 ➢ Cotrimoxazole prophylaxis at 6 weeks and as long as child is exposed
➢ Tuberculosis screening
➢ Counsel mother on nutrition and breast feeding
➢ Immunization
➢ Follow up mother’s CD 4 count and viral load every 3 months

c) What systemic tests have to be done as the child grows and when would you
do them?
Answer:
Nucleic acid testing (NAT) at first contact, 6 weeks, 6 months and 9 months
Serological (Antibody) testing at 12 months, if positive follow up with NAT,
if negative, do another serological test at 18 months. If positive at 18 months
follow up with NAT. If negative perform serological test at 24 months old and
if positive follow up with NAT.
Also monitor Full blood count, Urea, electrolytes and creatinine, and Liver
enzymes at first contact and every other contact with the child.

d) NAT reveals that the baby is positive, what regimen do you put the child on?
Answer:
➢ AZT + 3TC + LVP-r or
➢ ABC + 3TC + LVP-r

49.A family moved into a new house and their 4-year-old child presents with
difficulty breathing and foaming at the mouth without a no fever or any fits.
a) Write down 4 initial management steps
Answer:
➢ Admit and Keep airway patent:
- Suction foam and secretions from the mouth
- Consider intubation
- Keep head end of the bed elevated
➢ Administer oxygen via nasal prongs or mask
➢ Gain venous access and send blood for investigations (arterial blood
gasses, U and Es, FBC and Random blood glucose)
➢ Perform a physical examination checking for: oxygen saturation,
respiratory rate, heart rate, blood pressure, level of consciousness.
Perform a neurological, cardiovascular and respiratory examination.

477
c) What is the most likely diagnosis?
Answer: Asthma (acute exacerbation)

d) List 4 clinical signs to look for

Answer:
➢ Use of accessory muscles of respiration or poor respiratory effort
➢ Presence of wheezing
➢ Impaired level of consciousness
➢ Speaking with difficulty or unable to speak
➢ Heart rate >130b/min
➢ Respiratory rate >50b/min

e) Give 3 fundamental drugs used to treat

Answer:
➢ Nebulized Salbutamol 2.5mg
➢ Prednisolone 2mg/kg (max 40mg) PO OD for 3 days

478
INDEX

A
Abdominal Examination · 32
Acute chest syndrome · 113
Acute Dactylitis · 114
Acute diarrheal disease · 186
Acute gastroenteritis · 185
ADD · See Acute diarrheal disease
Adenovirus · 52
Adolescence · 4
AGE · See Acute gastroenteritis
Age range of children · 4
Annular pancreas · 360
Aplastic crisis · 113
Avascular necrosis · 114
B Hyperbilirubinemia · 357
Bacterial Tracheitis · 51
BMI · See Body Mass Index
BODY MASS INDEX · 10
Bronchiolar spasm · 52
Bronchiolitis · 51, 54, 55, 56
D
Dandy-Walker Malformation · 35
Dehydration · 180
Delayed hypersensitivity reaction ·
269
Development · 5, 12, 13, 16
Developmental Milestones · 13
Diarrhea · 185
Direct laryngoscopy · 45
Disseminated tuberculosis · 270
Dive reflex · 330
Domains of Development · See
Developmental Milestones
Down syndrome · 41
Down Syndrome · 41
E Hyperhemolytic Paradigm · 117
Early-onset sepsis · 257
Epiglottitis · 44
Epithelioid cells · 269
Erythrovirus B19 · 116
Growth · 5
H
Hand and foot syndrome · 114
Hb electrophoresis · 117
Head Circumference · 7
Heaf tests · 275
Height · 6
HIE · See Hypoxic ischemic
encephalopathy
Hirschsprung’s enteritis · 351
History · See Pediatric History
Howell-Jolley bodies · 118
Hydrocephalus · 37
Hydrocephalus ex vacuo · 35
Hydroxyurea · 124
Hyperhemolytic crisis · 113
Hypoxic ischemic encephalopathy ·
329
I

C IGRA · See Interferon-gamma

Caput saccedaneum · 359
Cardiovascular System Examination
· 31
Caseous necrosis · 269
Cephalohematoma · 359
Chest Circumference · 8
Chiari type II Malformation · 35
Chronic diarrhea · 200
Codoctytes · See Target Cells
Cognitive Development · 15
Common cold · See Coryza
Communicating Hydrocephalus · 35
Congenital Aqueductal Stenosis · 35
Congenital tuberculosis · 268
Coryza · 40
Crigler-Najar syndrome · 359
Croup · 48
Cystic fibrosis · 80
F release assays
Immunization · 34
Failure to thrive · 374 Infantometer · 6
Fine Motor Skill Development · 13 Interferon-gamma release assays ·
Fine Motor Skills · 15 275
Frankfort plane · 6 Intrauterine growth restriction · 314
FTT · See Failure to thrive Isosthenuria · 111
IUGR · See Intrauterine growth
restriction
G
Gados channel · 112 K
General Examination · 26
GenXpert · 275 Klinefelter · 33
Ghon complex · 269
Ghon focus · 269
Gilbert’s disease · 360 L
Granuloma · 269
Gross Motor Development · 13 Langhans’ giant cells · 269

479
Laryngotracheobronchitis · See Sickle cell disease · 111
P
Croup Snuffles of congenital syphilis · 42
Latent tuberculosis · 269 Spasmodic croup · 48
Paediatrician · 4
Late-onset sepsis · 257 Squestration crisis · 113
Parainfluenza · 52
Length · See Height Standiometer · 6
Parvovirus B19 · Erythrovirus B19
Steeple sign · 49
Pediatric History · 18
Physical Examination · 31
M
Physiologic jaundice · 358
T
Pneumatosis intestinalis · 347
Mantoux test · 273, 275
Pneumonia · 58
MAS · See Meconium aspiration Tachoids · 112
Pneumoperitoneum · 347
syndrome Target cells · 118
Postnatal period · 4
Meconium aspiration syndrome · TB · See Tuberculosis
Prematurity · 316
352 Technetium 99 · 121
Priapism · 114
Mid Upper Arm Circumference · 9 Teenager · 4
Primary complex of Ranke · See
Miliary tuberculosis · 270 Transcranial Doppler Ultrasound ·
Ghon complex
Monoglism · See Down syndrome 121
Primitive reflexes · 13
Motor Development · 13 Transient tachypnea of the newborn
Ptosis · 121
Mycolic acid · 268 · 324
Mycoplasma pneumoniae · 52 Trisomy 21 · See Down syndrome
TTN · See Transient tachypnea of the
R
newborn
N Tuberculosis · 268
RDS · See Respiratory distress
Turner syndrome · 34
syndrome
Nasopharyngitis · See Coryza
Respiratory distress syndrome · 327
NEC · See Necrotizing enterocolitis
Respiratory Syncytial Virus · 52 V
Necrotizing enterocolitis · 262, 345
Respiratory System Examination ·
Neonatal Jaundice · 357
30
Neonatal Sepsis · 257 Vaccination · See Immunization
Rhinovirus · 52
Nervous System Examination · 34 Vaso-occlusive crisis · 113
NICU Orientation · 303 Viral croup · 48
Non-Communicating Hydrocephalus Volvulus neonatorum · 351
S
· 35
Non-Physiologic jaundice · 358
Sepsis · 257
W
Sepsis Neonatorum · See Neonatal
Sepsis
Weight · 5
Sepsis screen · 260
Wine bottle sign · See Steeple sign
Septicemia · 257

480