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 and curative care of regional Pediatric
conditions.
The book serves as a good guide and 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 .................... 4
PHYSICAL EXAMINATION ........ 25
IMMUNIZATON AND VACCINATION 34
PEDIATRIC GENETIC DISORDERS 34
NEUROLOGICAL CONDITIONS 34
RESPIRATORY CONDITIONS ... 39
CARDIOVASCULAR CONDITIONS67
HEMATOLOGY ............................... 85
GASTROENTEROLOGY .............. 94
GENITOURINARY TRACT........ 132
DERMATOLOGY........................... 135
INFECTIOUS DISEASES ............ 140
NEONATOLOGY ........................... 165
MISCELLANEOUS........................ 208
END OF BOOK REVISION.......... 216
INDEX ............................................... 267

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MOSES KAZEVU

CHAPTER 1: INTRODUCTION

INTRODUCTION TO PAEDIATRICS AND CHILD

HEALTH

 Paediatrics (from the Greek word habits, values and lifestyles that
pedo pais; “a child” iatros; would make children responsible
“healer”) deals with the care of adults and citizens.
children and adolescents.  It is important to remember that a
 Paediatrics covers the age group child is not a miniature adult.
less than 18 years of age.  The principles of adult
 Postnatal period medicine cannot be directly
o 0-28 days= Neonate adapted to children.
o 0-1 year= Infant  Pediatric biology is unique and
o 1-3 years= Toddlers risk factors of pediatric disease are
o 3-6 years= Preschool child distinct.
o 6-12 years= School going  Clinical manifestations of
child childhood disease may be different
 Adolescence- 10-20 years from adults.
o 10-13 years= Early  Drug dosages in children are
o 14-16 years= Middle specific and not a mathematical
o 17-20 years= Late derivation of the adult doses.
 Note: 13-19 years= teenager  Nutrition is a critical necessity for
 A paediatrician specializes in children not only to sustain life,
health care of children and but to ensure their growth and
adolescents. development.
 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
developing. It is time to acquire

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MOSES KAZEVU

CHAPTER 2: GROWTH & DEVELOPMENT

NORMAL GROWTH AND DEVELOPMENT

 Growth is an essential feature that GROWTH

distinguishes a child from an adult.  There are specific periods in a
 The process of growth begins from child’s life when the rate of growth
the time of conception and is steady, accelerates or
continues until the child grows into decelerates.
a fully mature adult.  The fetus grows fast in the first
 Growth denotes a net increase in half of gestation.
the size or mass of tissue.  Thereafter, the rate of growth is
 It is largely attributed to slowed down until the baby is
multiplication of cells and born.
increase in the intracellular  In the early postnatal period the
substance. velocity of growth is high,
 Hypertrophy or expansion of especially in the first few months.
cell size contributes to a lesser
 Thereafter, there is slower but
extent to the process of growth. steady rate of growth during mid-
 Development specifies maturation childhood.
of functions.  A second phase of accelerated
 It is related to the maturation growth occurs at puberty.
and myelination of the nervous
 Growth decelerates thereafter for
system and indicates
some time and then ceases
acquisition of a variety of skills
altogether.
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.

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

 The general body growth is rapid Figure 2.1: Rates of growth of different tissues and organs
(Adapted from Ghai Essential Pediatrics 8th Ed)
during fetal life, first one or two
years of postnatal life and also ASSESSMENT OF
during puberty. PHYSICAL GROWTH
 In the intervening years of mid-  Includes assessment of
childhood, the somatic growth (Anthropometric measurements):
velocity is relatively slowed down.  Weight
 Order of growth is cephalocaudal  Height
and distal to proximal.  Head circumference/ Occipital
 During fetal life, growth of frontal circumference (OFC)
head occurs before that of neck,  Chest circumference
and arms grow before legs.  Mid upper arm circumference
 Distal parts of the body such as (MUAC)
hands increase in size before  It is difficult to precisely define the
upper arms. normal pattern of growth.
 In the postnatal life, growth of  Generally, it implies an average of
head slows down but limbs readings obtained in a group of
continue to grow rapidly. 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
Figure 2.1: Beam scale for accurate measurement of
subtracted from the infant’s weight. The child should be nude or in minimal light
clothing. (Image courtesy of Ghai Essential Pediatrics 8th
chronological age. This correction, Ed)
however is not required after 2
years of age.  The average birthweight of
neonates is about 3kg (2.5 – 3.5
kg).
WEIGHT  During the first few days after
 The weight of a child in the nude birth, the newborn loses
or minimal light clothing is extracellular fluid equivalent to
recorded accurately on a lever or about 10% of the body weight.
electronic type of weighing scale.  Most infants regain their
 Spring balances are less accurate. birthweight by the age of 10
 The weighing scale should have a days.
minimum unit of 100g.  Subsequently, they gain weight at
 It is important that the child is a rate of approximately 30g per
placed in the middle of the day for the first 3 months of life.
weighing pan.  Thereafter, they gain 20g per day
 The weighing scale should be between 3 and 6 months.
corrected for any zero error before  Between 6 and 12 months they
measurement. gain 10g/day.
 Serial measurements should be  The birthweight after 1 year can be
done on the same weighing scale. calculated using this formula:
 Approximate weight= 8 + (2 x
years)
 So at 2 years:
 Weight= 8 + (2 x 2)
 Weight= 8+ 4
 Weight= 12kg

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TABLE 2.2: WEIGHT  The head is held firmly in position
Age Formula Expected against a fixed upright head board
weight by one person.
At birth - 3 kg  Legs are straightened, keeping feet
0-3 30 g per 5.7 kg at right angles to legs, with toes
months day gain (at 3 months) pointing upward.
3- 6 20g / day 7.5 kg
 The free foot board is brought into
months gain (at 6 months)
firm contact with the child’s heels.
6- 12 10g/ day 9.3 kg  Length of the baby is measure
months gain (at 9 months) from a scale which is set in the
measuring table.
1 years 8 + (2 x 1) 10 kg  Measurement of length of a child
2 years 8 + (2 x 2) 12 kg lying on a mattress and/or using
3 years 8 + (2 x 3) 14 kg cloth tapes is inaccurate and not
3-7 8+ (2 x 4) At 4 years= 16 recommended.
years kg
8+ (2 x 7) At 7 years= 22
kg
7 years- 3 kg per At 8= 24kg
puberty year gain
10- 14 32- 50 kg
years
14 years More than 50
Figure 2.2: Measurement of length on an infantometer.
kg Note how the knees are gently straightened while the head
(Averages were used to calculated and feet are aligned (Image adapted from Ghai Essential
Pediatrics 8th Ed)
expected weight)
 Standing height:
HEIGHT/ LENGTH  For the standing height, the
 Length/height is recorded for child stands upright.
children under 2 years of age.  Heels are slightly separated and
 Hairpins are removed and braids the weight is borne evenly on
undone. both feet.
 Bulky diapers should be removed.  Heels, buttocks, shoulder
 The child is placed supine on a blades and back of the head are
rigid measuring table or an brought in contact with a
infantometer. vertical surface as wall, height

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 measuring rod or a  At 1 year= 74cm (50cm + 24/1)
standiometer,  At 2 years= 86cm (74cm +
 The head is so positioned that 24/2)
the child looks directly  At 3 years= 94cm (86cm +
forwards with Frankfort plane 24/3)
(the line joining floor of  At 4 years= 100cm (94cm +
external auditory meatus to the 24/4)
lower margin of orbit) and the  After 4 years add about 5cm for
biauricular plane being each year until they are 12 years.
horizontal.  After this, increments in height
 The head piece is kept firmly vary according to the age at the
over the head to compress their onset of puberty. There is a
hair. marked acceleration of the growth
during puberty.
TABLE 2.3: HEIGHT/LENGTH
Age Expected
Formula length
(cm)
At birth - 50
At 1 year 50+ 24/1 74
At 2 years 74 + 86
24/2
At 3 years 86+ 24/3 94
At 4 years 94 + 100
24/4
4-12 years Add 5cm
every
year

Figure 2.3: Method of recording height: Note the erect HEAD CIRCUMFERENCE
posture and the bare feet placed flat on the ground. The
back of heels, buttocks, shoulders and occiput are touching  Hair ornaments are removed and
the wall. (Adapted from Ghai Essential Pediatrics 8th Ed)
braids undone.
 The infant measures approximately  Using a non-stretchable tape, the
50 cm at birth. maximum circumference of the
 At 3month= 60cm head from the occipital
 At 6 months= 70cm

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 protuberance to the supraorbital  6-12 month it increases by
ridges on the forehead is recorded. 0.5cm per month
 The crossed tape method, using  At 12 years it reaches about 55cm
firm pressure to compress the hair (10cm increase)
is the preferred way to measure
head circumference (as shown
TABLE 2.4 HEAD CIRCUMFERENCE
below).
Age Formula Expected
circumference
(cm)
At birth - 35
At 3 2cm increase 40
months per month
At 6 1 cm increase 43
months per month
At 1 0.5cm 45
year increase per
month (10cm
increase from
birth)
At 12 10cm increase 55
years from 1 year
 Note: the anterior fontanelle is
Figure 2.4: Method of recording head circumference. Note
the crossed tape method (Adapted from Ghai Essential
diamond in shape while the
Pediatrics 8th Ed) posterior fontanelle is deltoid.
 The anterior fontanelle closes by
 Head growth is rapid, especially in
18 months.
the first half of infancy.
 The posterior fontanelle closes by
 It reflects the brain growth during
3 months.
this period.
 The head growth slows CHEST CIRCUMFERENCE
considerably thereafter.  The chest circumference is
 At birth the head circumference is measured at the level of the
35cm (34- 37cm). nipples, midway between
 At the first year it increases by inspiration and expiration.
10cm to about 45cm (44-47cm).  The crossed tape method is
 First 3 months it increases by recommended.
2cm per month
 3-6 months it increases by 1cm

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 as well as avoid compression of
soft tissue.

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 Figure 2.6: Measurement of mid upper arm circumference.
Note how the anatomical landmarks are first located
chest are almost equal by the age (Arrows) to accurately meaasure the circumference
of 1 year (45cm). (Adapted from Ghai Essential Pediatrics 8th Ed)

 Thereafter the chest circumference BODY MASS INDEX (BMI)

exceeds the head circumference.  The formula to calculate BMI is
MID UPPER ARM weight (kg)/ height (meter)2.
CIRCUMFERENCE  BMI is primarily used to assess
 To measure this first mark a point obesity.
midway between the tip of  BMI at or above the 95th centile for
acromial process of scapula and age or more than 30kg/m2 is
the olecranon of ulna while the obesity.
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

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TABLE 2.5 Approximate anthropometric  Developmental milestones are
values by age important, easily identifiable
Age Weight Height OFC events during the continuous
(kg) (cm) (cm) process of development e.g.
Birth 3 50 35 turning over, sitting, reaching for
6 months 6 65 43 object, and pointing to objects.
1 year 10 74 45
 Increasingly complex skills are
2 years 12 86 48
learnt, matching the formation of
3 years 14 94 49
new synapses in the brain.
4 years 16 100 50
 While development is a global
process reflected in new motor
 Weight, height, head abilities and language, social and
circumference (until the age of 5) cognitive skills, intelligence
and sexual maturity are routinely pertains to the part of the
monitored during under-5 clinic to development dealing with
assess for adequacy of growth and cognitive or adaptive behavior.
development.  Intelligence is the ability to apply
 Standardized growth curves knowledge to manipulate one’s
represent normal values for age for environment or to think abstractly
95% of children and are used to and refers to the aggregate or
plot weight, height, body mass global capacity of the individual to
index and head circumference. act purposefully, think rationally
 Special growth curves exist for and to deal effectively with the
children with particular genetic environment.
conditions (e.g. Down syndrome,
achondroplasia). RULES OF DEVELOPMENT
 Development is a continuous
DEVELOPMENT process, starting in utero and
 Development refers to maturation progressing in an orderly manner
of functions and acquisition of until maturity.
various skills for optimal  The child has to go through
functioning of an individual. many developmental stages
 The maturation and myelination of before a milestone is achieved.
the nervous system is reflected in  Development represents the
the sequential attainment of functional maturation of the
developmental milestones. nervous system.

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 The sequence of attainment of factors (e.g. personalities of family
milestones is the same in all members, economic status,
children. E.g. all infants babble depression or mental illness in
before they speak in words and sit caregivers, availability of learning
before they stand. Variations may experiences in the environment,
exist in the time and manner of cultural setting into which the
their attainment. child is born).
 The process of development
DOMAINS OF
processes in a cephalocaudal
direction. DEVELOPMENT
 Head control precedes trunk  Developmental milestones provide
control, which precedes ability a systematic way to assess an
to use lower limbs. infant’s progress.
 The control of limbs proceeds  The domains of development
in a proximal to distal manner include:
such that hand use is learnt  Motor development
before control over fingers. o Gross motor development
 Certain primitive reflexes have to o Fine motor skill
be lost before relevant milestones development
are attained. For example, palmar  Cognitive
grasp is lost before voluntary grasp  Social and emotional
is attained and the asymmetric development
tonic neck reflex has to disappear  Hearing, speech and language
to allow the child to turnover.  Attainment of a particular skill
 The initial disorganized mass depends on the achievement of
activity is gradually replaced by earlier skill.
specific willful actions.  Delays in one developmental
 When shown a bright toy, a 3-4 domain may impair development
month old squeals loudly and in another domain e.g. it may be
excitedly moves all limbs, difficult to assess problem-solving
whereas a 3-4 year old may just skills in a child with cerebral palsy
smile and ask for it. who understands the concept of
 Development may be influenced matching geometric forms but
by both intrinsic factors (e.g. lacks the physical ability to
child’s physical characteristics, demonstrate that knowledge.
state of health, temperament and  Information about motor
genetic attributes) and extrinsic milestones should be obtained

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 from the history as well as from  Infants with CNS damage have
observation during the physical delayed development of
examination. postural reactions.
 At birth the infant is able to turn
the head side to side.
GROSS MOTOR FUNCTION
 By 2-3 months the infant:
 Motor development progresses in a
 Lifts their head when lying
cephalocaudal direction with
prone
suppression of primitive reflexes
 Has head lag when pulled from
and development of postural tone
supine position
and secondary protective reflexes.
 By 4 months the infant is able to
 The primitive reflexes include the
roll over and there is no head lag
Moro, grasp, stepping and
when pulled from supine position.
asymmetric tonic neck reflexes
The infant also pushes chest up
must have disappeared by 3-6
with arms.
months of age before head control
 By 6 months the infant is able to
(4 months) and independent sitting
sit alone and leads with head when
at 6-8 months can occur.
pulled from supine position.
 Each primitive reflex requires a
 By 9 months the infant pulls to
specific sensory stimulus to
stand and cruises.
generate the stereotypical motor
 An infant stands holding on
response.
furniture.
 Infants with CNS injuries show
stronger and more-sustained  By 12 months the infant is able to
primitive reflexes. walk.
 Postural reactions such as  A delay in walking beyond 18
parachute reaction are not months of age is a warning sign in
present at birth (i.e. they are children who have been crawling
required). These reactions as the early locomotor pattern.
which help facilitate the  At 18 months of age, a child can
orientation of the body in climb onto a chair and walk up and
space, require complex down stairs two feet per step by 24
interplay of cerebral and months of age.
cerebellar cortical adjustments  By two and half years of age a
to proprioceptive, visual and child should be able to stand on
vestibular input. tip-toes, jump on both feet and
kick a ball.

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

FINE MOTOR SKILLS become more available for thee

 Involve the use of the small manipulation of objects.
muscles of the hands.  As control over distal muscles
 An infant’s fine motor skills improves reaching and
progress from control over manipulative skills are enhanced.
proximal muscles to control over  During the second year of life the
distal muscles. infant learns to use objects as tools
 During the first year of life, as (e.g. building blocks).
balance in sitting and ambulatory  At birth the infant keeps the hands
positions improves the hands tightly fisted.

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 By 3-4 months the infant can bring  Differential diagnosis of motor
the hands together to the midline delay:
and then to the mouth.  CNS injury
 By 4-5 months they can reach for  Spinal cord dysfunction
objects.  Peripheral nerve pathology
 By 6-7 months they can rake  Motor endplate dysfunction
objects with the whole hand as  Muscular disorder
well as transfer object from hand  Metabolic disorders
to hand.  Neurodegenerative conditions
 By 9 months they can use the LANGUAGE SKILLS
immature pincer (ability to hold
 Delays in language development
small objects between the thumb
are more common than delays in
and index finger)
other domains.
 By 12 months they can use the
 Receptive language is always more
mature pincer.
advanced than expressive language
RED FLAGS IN MOTOR (i.e. a child can usually understand
DEVELOPMENT 10 times as many words as he or
she can speak).
 Persistent fisting beyond 3 months
 Language and speech are not
of age is often the earliest sign of
synonymous. Language refers to
neuromotor problems.
the ability to communicate with
 Early rolling over, early pulling to
symbols i.e. sign language,
stand instead of sitting and
gestures, writing and body
persistent toe walking may all
language. Speech is the vocal
indicate spasticity.
expression of language.
 Spontaneous postures such as
 A window of opportunity for
scissoring in a child with spasticity
optimal language acquisition
or a frog-leg position in a
occurs during the first 2 years of
hypotonic infant are important
life.
visual clues to motor
 At birth the infant attunes to
abnormalities.
human voice and is able to
 Early hand dominance (before 18
differentially recognize parents’
months of age) may be a sign of
voices.
weakness of the opposite upper
 By 2-3 months: cooing (runs of
extremity associated with a
vowels), musical sounds (e.g. ooh-
hemiparesis.
ooh, aah-aah)

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 By 6 months: babbling (mixing  Infant intelligence can be
vowels and consonant together) estimated by evaluating problem
(e.g. ba-ba-ba, da-da-da) solving and language milestones.
 By 9-12 months: Jargoning (e.g.  Language is the single best
babbling with mixed consonants, indicator of intellectual potential.
inflection and cadence). They also  Gross motor skills correlate poorly
begin using mama and dada (non- with cognitive potential.
specific).  By 3 months the infant can move
 By 12 months: 1-3 words, mama objects.
and dada (specific).  By 6 months the infant can imitate
 By 18 months: 20-50 words, facial expression and recognize
beginning to use 2 word phrases. strangers.
 By 2 years: 2-word telegraphic  By 9 months they can imitate
sentences e.g. mommy come, 25- certain gestures.
50% of child’s speech should be  By 12 months they can understand
intelligible. simple commands.
 By 3 years: 3 word sentences,  By 24 months they can identify
more than 75% of the child’s shapes, colors and count.
speech should be intelligible.
RED FLAGS IN COGNITIVE
 Differential diagnosis of speech
DEVELOPMENT
or language delay:
 Global developmental delay or  Language development estimates
mental retardation verbal intelligence, whereas
 Hearing impairment problem-solving skills estimate
 Environmental deprivation nonverbal intelligence.
 Pervasive developmental  If skills are delayed significantly in
disorders including autism both language and problem solving
spectrum disorders. domains, mental retardation should
be considered.
COGNITIVE DEVELOPMENT
 If only language skills are delayed,
 Involves skills in thinking,
hearing impairment or a
memory, learning and problem
communication disorder should be
solving.
considered.
 Intellectual development depends
 If only problem-solving skills are
on attention, information
delayed, visual or fine motor
processing and memory.

15
 problems that interfere with  Attachment: Bonding with a
manipulative tasks may be present. primary caregiver begins at
 If there is a significant discrepancy birth. Developing empathy is
between language and problem- critical during the first 3 years
solving skills, the child is at high of life.
risk for learning disabilities.  A sense of self and
independence: The process of
separation and individuation
SOCIAL SKILLS begins at about 15 months of
 Social skills are the ability to age.
interact with other people and the  Social play: Toddlers exhibit
environment. parallel play during the first 2
 Development of social skills is years of life. They learn to play
dependent on cultural and together and share at about 3
environmental factors. years of age
 Milestones, in order include:

16
 3

Table 1.7: Domains of development

 CHAPTER 3: PEDIATRIC HISTORY

PEDIATRIC HISTORY
 Good doctors will continue to be  The older child should be given an
admired for their ability to distil opportunity to talk, to present their
the important information from the symptoms and to tell how they
history, for their clinical skills, for interfere with school and play
their attitude towards patients and activities.
for their knowledge of disease,  The simple act of offering a toy,
disorders and behavior problems. picture book or pen-torch is often
 Always greet the child and parents an effective step toward
by name, irrespective of age as this establishing rapport.
will convey an attitude of concern  The format generally of taking a
and interest. Introduce yourself. history in a pediatric patient is as
 Considerable tact and discretion follows:
are required when taking the  Demographics
history especially in the presence  Presenting complaint
of the child: questioning on  History of presenting complaint
sensitive subjects should best be  Review of system
reserved for a time when the  Past medical history
parents can be interviewed alone.  Drug history
 It may therefore be necessary to  Birth history (pregnancy and
separate the parent and child- delivery)
patient when taking the history  Growth and development
especially when the problems are history
related to behavior, school o Neonatal period and
difficulties and socioeconomic infancy
disturbances in the home o Subsequent developmental
environment. milestones
 Information taken in the history is  Immunizations
usually obtained from the mother  Nutrition and feeding history
however useful information can be  Family history
obtained by observing the infant  Social-economic history
and young child during the history-  Summary
taking.  Differential diagnosis

4
DEMOGRAPHIC  Onset: the order of onset of
symptoms is important. If there
DATA/IDENTITY is doubt about the date of onset
 This includes identifying data such of the disease, the patient
as: should be asked when he/she
 Patient’s name, last felt quite well and why
 Age, he/she first consulted the
 Sex, doctor.
 Tribe,
 This must be recorded in the
 Religion
informant’s words and never
 Region of residence,
interpreted by the doctor into
 Informant (parent or patient),
medical terminology.
 Date of history/examination
 When the patient’s own
and
phraseology is used, the words
 Language of interview
should be written in inverted
 Indication of whether it is a
commas, e.g. “giddiness”, “wind”,
hospital referral or self-referral
“palpitation” and an attempt
 Key point: observe the child at
should be made to find out
play in the waiting area and
precisely what they mean to the
observe their appearance, behavior
patient.
and gait as they come into the
clinic room. The continued HISTORY OF
observation of the child during the
PRESENTING
whole interview may provide
important clues to the diagnosis COMPLAINT
and management.  Describe the course of the patient’s
illness, including when and how it
PRESENTING began, character of symptoms,
COMPLAINT aggravating or alleviating factors,
 Reason that the child is seeking pertinent positives and negatives,
medical care and duration of past diagnostic testing.
symptoms.  Days of the week should not be
 What prompted referral to the written in history since they don’t
doctor? indicate duration of disease.
 What do the parents think or  Let the parent give the history in
fear is the problem? their own way and then ask
specific questions.

20
 Ask how severe are the symptoms,  Speech disturbance: duration,
have they changed during the past onset, nature, problems in reading
days, weeks or months, has there or understanding.
been any change recently in the  Memory: independent opinion of
child’s appetite, energy or relative or friend must be sought.
activities, has the child been absent
from school, has anyone who cares RESPIRATORY SYSTEM
for the child been ill, has the child  Cough: character, frequency,
been thriving or losing weight, duration, causing pain and timing,
what change in behavior has there productive
been, has there been a change in  Sputum: Quantity, color, nature
appetite, in micturition or bowel (frothy, stringy and sticky), odor,
habit. timing (when most profuse),
hemoptysis (blood), is the blood
REVIEW OF SYSTEMS red (frank) or brown (digested)? Is
 This systematic enquiry runs from it pure blood or ‘specks’?
the “head to toes”. And these  Dyspnea: on exertion or at rest.
questions should be asked in order Expiratory difficulties,
to find out any other associated precipitating factors, cough, fog,
symptoms of the disease. emotion, change in environment
and wheezing.
CENTRAL NERVOUS
 Pain in chest: location, character,
SYSTEM affected by respiration, positions
 Loss of consciousness: sudden, that increase or decrease pain
warning, injuries, passage of urine,  Hoarseness: with or without pain
duration and after effect. (involvement or recurrent
Precipitating factors and laryngeal nerve)
witnesses?  Throat: soreness, tonsillitis, ulcers,
 Dizziness and vertigo infection
 Numbness  Nasal discharge or obstruction
 Seizures, headaches and  Epistaxis
behavioral changes  Night sweats
 Visual disturbance: seeing double  Wheezing: asthma, chest infection,
(diplopia), dimness, zigzag figures relieving factor, COPD
(fortification spectra)
 Deafness or tinnitus: Discharge
from ears, pain and hearing loss.

21
CARDIOVASCULAR Pain, regurgitation. Progression
SYSTEM from solid food to liquid.
 Dyspnea: on exertion (degree), at  Diarrhea (frequency and looseness
rest, time especially if wakes at of motions) and constipation
night, position (orthopnea), (recent or longstanding and
relieving factors, gradual or severity)
sudden onset, change, duration  Nature of stool: black (melena),
(paroxysmal nocturnal dyspnea), clay/pale (obstructive jaundice),
precipitating factors, number of bulky and fatty (steatorrhea), pile
pillows used.  Tenesmus: painful sensation and
 Chest pain: site, on exertion or at urgent need to defecate (rectal
rest, character, radiation, duration, pathology)
relief by drugs. Etc. accompanying  Flatulence
sensations e.g. breathlessness,
GENITOURINARY
vomiting, cold sweat, pallor,
 Micturition: frequency, retention,
frequency, other relieving factors
dribbling and amount of urine
 Palpitation
passed, incontinence.
 Dizziness and faints
 Urine: color, amount, smell, blood
 Swelling of ankles: time of day (hematuria), frothy.
 Intermittent claudication: at rest or  Dysuria and flank pain
exertion
 Menstruation
 Coldness of feet: Raynaud’s  Age of onset (menarche)
phenomenon  Periods:
GASTROINTESTINAL o Last normal menstrual
 Abdominal pain or discomfort: period (LMP)
site, character (constant or o Regularity: regular or
colicky), radiation, relationship to irregular
food and bowel actions. o Menstrual cycle: number of
 Nausea and vomiting: frequency days or interval e.g. 4/28
and relationship to food, amount of o Amount of loss: Increase or
vomitus, contents, color, blood decrease in flow
(hematemesis) o Inter-menstrual discharge-
character, blood or
 Appetite and loss of weight
otherwise
 Dysphagia: food hard or soft,
fluids, level at which food “sticks”.

22
  Vaginal discharge: quantity,  Maternal obstetric problems,
color (normally clear), smell delivery, prolonged rupture of
and irritation. membranes, maternal pyrexia.
 Visits to antenatal care
MUSCULOSKELETAL AND (normally should have four
SKIN visits and should have receive,
 Joint swelling hematinics, fancidar-malaria
 Joint stiffness prophylaxis, if mother is
 Skin rash: type, duration, any exposed and on septrin there is
treatment, painful and itching no need for fancidar).
(psoriasis)  Gestational age at birth and
length of gestation (preterm,
PAST MEDICAL term or post-term).
HISTORY  Perinatal history:
 Medical problems,  Method of delivery:
hospitalizations, history of similar Spontaneous vertex delivery
illness, operations, accidents or (SVD), forceps, caesarean
injuries.  Birth weight
 Diabetes, Epilepsy, Asthma, TB,  Apgar scores
HIV, hypertension, rheumatic  Birth complications (e.g.
fever, heart disease, cystic fibrosis, infection, jaundice),
spina bifida should be enquired  Postnatal history:
appropriately.  Length of hospital stay and
 Any history of any pediatric method of feeding.
deaths- enquire about the cause of
death. GROWTH AND
DEVELOPMENTAL
DRUG HISTORY
HISTORY
 Past and present medications-
 Age at attainment of important
including name and dosage.
milestones (Walking, talking, self-
 Allergies.
care, smile, head support, sit,
BIRTH HISTORY stand).
 Include: Prenatal, Perinatal and  Relationships with siblings, peers,
Postnatal history adults.
 Prenatal history:  School grade and performance,
behavioral problems.

23
 Monitoring growth curves (Look  Occupation of parents and parental
at under-five card). level of education
 Relationship of parent
IMMUNIZATIONS  Water source and toilet
 Are they up to date? (check under  Alcohol, smoking, drugs, sexual
5 card) activity.
NUTRITION  Safety: child car seats, smoke
 Type of diet, amount taken each detectors, bicycle helmets.
feed, change in feeding habits,  A history of recent travel abroad,
breast feeding (duration and particularly in tropical areas is
weaning) important as the child may have a
disorder uncommon in his/her own
FAMILY HISTORY country but having been contracted
 Are parents alive? in another country where disease
 Siblings and their ages may be endemic.
 Any pediatric deaths (including
SUMMARY
miscarriages, stillbirths,
 Short and brief highlighting the
terminations)
important positives and negatives.
 Medical problems in family
including diabetes, seizures,
asthma, allergies, cancer, cardiac,
renal or GI disease, tuberculosis, DIFFERENTIAL
smoking. DIAGNOSIS
 Listed in order from most-likely to
SOCIO-ECONOMIC least likely in relation to the
HISTORY history.
 Family situation

24
 CHAPTER 4: PHYSICAL EXAMINATION

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

25
 o General inspection-general chromosomal disorders or in
condition, nutritional status, mucopolysaccharidoses.
pallor, jaundice, cyanosis  Level of consciousness: Alert,
o Vital signs hyper-alert, lethargic, stupor or
o Anthropometric comatose?
measurements- height,  Is the child cyanosed, pale or
weight, and head jaundiced (in carotinaemia the
circumference sclerae are not yellow)
 Skin  Infant should be pink with
 Head exception of the periphery,
 Face which may be slightly blue.
 Eyes  Congenital heart disease is only
 Ears and nose suggested if the baby has
 Mouth and pharynx central cyanosis.
 Neck o The best areas to look for
 Thorax and lungs central cyanosis are the
 Cardiovascular system tongue and buccal mucosa
 Breasts not the limbs and the nails.
 Abdomen  A pale baby maybe anemic or
 Genitalia and rectum ill and requires carful
 Musculoskeletal system investigation to find the cause.
 Nervous system  A blue baby may have either a
 Otoscopic examination of the cardiac anomaly or respiratory
ears problem and rarely
methaemoglobinaemia.
GENERAL  Normal physiological jaundice
EXAMINATION appears 2-3 days after birth of
GENERAL INSPECTION the infant peaking about the 5th
 General condition of the child: Is day and subsequently
the child unwell (stable), disappearing within one week.
breathless or distressed?  Check for capillary refill time and
 Does the child look like the rest of skin turgor (skin pinch).
the family?  Posture and movements
 The face may be characteristic  A term baby lies supine for the
in Down’s syndrome and other first day or two and has
vigorous, often asymmetric
movements of all limbs.

26
  In contrast a sick baby adopts  Temperature: Rectal preferred.
the frog position with legs o Average rectal temperature
abducted, externally rotated and is higher in infancy than in
is inactive. adulthood usually not
 Older infants and children falling below 37.2 degrees
should be observed for Celsius until after 3 years.
abnormal movements, posture
and gait. SKIN
 Examine for texture and
ANTHROPOMETRIC appearance.
MEASUREMENTS  In infants it is soft and smooth
 Height, weight, head because it is thinner than in
circumference (Occipito-frontal older children.
circumference: OFC) and mid-  The presence of any skin rash, its
upper arm circumference should color and whether there are present
be plotted on a percentile chart. macules, papules, vesicles, bullae,
 For details of each refer to Chapter petechiae or pustules should be
2. recorded.
 The skin texture, elasticity, tone
VITAL SIGNS and subcutaneous thickness
 Parameters measured are should be assessed by picking
 Pulse up the skin between the fingers.
o Palpate the femoral artery  Pigmented naevi, strawberry
or brachial artery (in cubital naevi, haemangiomata or
fossa) or auscultate the lymphangiomata may be
heart. present and may vary in size
 Respiratory rate and number.
o Normal: 30- 60 breaths per  They may be absent or small at
minute birth and grow in subsequent
 Blood pressure: measured days or weeks.
routinely in children above the
age of 2.

27
 0

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

 Palpate the skin and roll it to check  In the infant the skull should be
for skin turgor (dehydration) palpated to determine the size and
tension in the fontanelles an assess
the skull sutures.
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
Figure 4.2: Top of head anterior fontanelle and cranial
above the mean) is due to sutures (Adapted from Hutchinson's Pediatrics)
macrocephaly, which may be
due to hydrocephalus, subdural  Premature fusion of sutures
hematoma or inherited suggests craniostenosis.
syndromes.  In neonates the posterior fontanelle
 Familial macrocephaly may be very small and
(autosomal dominant) is a subsequently closes by 3 months
benign familial condition with but the anterior fontanelle is larger,
normal brain growth. only closing at around 18 months
(1 year 8 months).
 A tense and bulging fontanelle results in the eyes opening so they
suggests raised intracranial can be inspected.
pressure and a deeply sunken one  Squint is a condition in which
suggests dehydration. early diagnosis and treatment is
 Large fontanelles, separation of important.
sutures, delayed closure of the
fontanelles may be associated with EARS AND NOSE
raised intracranial pressure, or MOUTH AND PHARYNX
other systemic disorders such as  Any discharges: basal skull
hypothyroidism and rickets. fracture (CSF)
 Hair: alopecia, seborrhea of the  Nasal congestion
scalp.  Any foreign objects
 Check for tonsils, teething, oral
FACE
thrush
 Abnormalities of facial
development are usually obvious NECK AND THROAT
and an example is the infant with  Short, webbed (Turner syndrome),
cleft lip. torticollis
 Associated with this there may be  Thyroid: enlarged, bruit
a cleft palate, but full visual  Swellings
examination of the palate  Midline: thyroglossal cyst, goiter
including the uvula is necessary to
 Lateral: lymph nodes, branchial
ensure that the palate is intact and
cyst, cystic hygroma,
there is not a sub-mucous and soft
sternomastoid tumor.
palate clefts cannot be felt on
palpation.
EXAMINATION OF THE
EYES
 Inspect for: RESPIRATORY SYSTEM
 Subconjuctival hemorrhages INSPECTION
 Cataracts  Scars of past surgery (look at the
 Papilloedema front and the back of the chest).
 Congenital abnormalities e.g.  Shape: normal and symmetrical,
colobomata. pectus carinatum (undue
 ‘Rocking’ the baby from the prominence of the sternum-pigeon
supine to vertical position often chest), Pectus excavatum (funnel
chest), Harrison’s sulci,

29
 hyperinflation (increased EXAMINATION OF THE
anteroposterior diameter).
CARDIOVASCULAR
 Use of accessory muscles of
respiration. SYSTEM
 Flaring of the nares.  The examination of the CVS of
 Intercostal recession, any stridor, infants and children is carried out
audible wheeze or grunt. in a similar manner to that of
 Count the respiratory rate. adults.
 The examiner should always feel
PALPATION for femoral pulses and ascertain
 Chest wall movements: is it whether there is any radiofemoral
symmetrical? or brachiofemoral delay as this
 Feel the trachea: central or would suggest the possibility of
deviated coarctation of the aorta.
 Palpate for any fractured ribs or  The most important factor in
clavicle. recording the blood pressure of
 Tactile vocal fremitus (over 5 children is to use a cuff of the
years of age- ask the child to say correct size.
99).  The cuff should cover at least two-
thirds of the upper arm.
PERCUSSION  If the cuff size is less than this a
 Percuss all areas: normal, resonant, falsely high blood pressure
hyper-resonant, dull (collapse, reading may be obtained.
consolidation), stony dull (pleural  In small infants relatively
effusion) accurate systolic and diastolic
AUSCULTATION pressures as well as mean
 Air entry, vesicular (normal), arterial pressure can be
absent breath sounds (pleural obtained by use of the Doppler
effusion) and bronchial method.
(consolidation)  The apex should be visible and
 Added sounds: Rhonchi, stridor, palpable and the position noted.
inspiratory or expiratory crackles  The precordial areas should be
(fine versus coarse), pleural palpated for the presence of thrills.
friction rub  If the apex beat is not obvious look
 Vocal resonance. for it on the right side of the chest
as there could be dextrocardia or a
left-sided congenital

30
 diaphragmatic hernia with the
heart pushed to the right or  Note: Percussion of the heart is not
collapse of the right lung. normally undertaken in children.
 All areas should be auscultated
while the baby is quiet systolic
AUSCULTATION
murmurs may be very harsh and  Listen to all 4 valve area (apex,
can be confused with breath lower L sternal edge, upper L
sounds. sternal edge and upper R sternal
edge)
INSPECTION  Quality of heart sounds.
 Are there features of Down’s  Additional sounds i.e. clicks,
(Atria septal defect-ASD, murmur (timing of the murmur).
Ventricular septal defect- VSD),  Blood pressure-use a cuff that
Turner’s (coarctation of the aorta) covers at least 2/3 of the upper arm
or Marfan’s (aortic incompetence). or use Doppler.
 Cyanosis: peripheral and central
 Hands: clubbing (Cystic fibrosis)
and splinter hemorrhages EXAMINATION OF THE
(endocarditis)
ABDOMEN
 Edema: precordium, ankles and
 In the infant the abdomen and
sacrum
umbilicus are inspected and
 Precordium for scars of past
attention should be paid to the
surgery.
presence of either a scaphoid
PALPATION abdomen (in neonates may
 Pulses: Radial/brachial/femoral- indicate diaphragmatic hernia or
radiofemoral delay, rate. duodenal atresia) or distended
 Character of pulse- collapsing, abdomen which suggests intestinal
volume obstruction especially if visible
 Heart rate- rhythm peristalsis can be seen.
 Apex beat- position (normal  Peristalsis from left to right
position in children 4th-5th left suggests a high intestinal
intercostal space in the mid- obstruction whereas one from
clavicular line), beware of the right to left would be more
dextrocardia. in keeping with low intestinal
obstruction.
 Palpate for a parasternal heave and
for precordial thrills.

31
 Any asymmetry of the abdomen intestinalis (intramural gas in the
may indicate the presence of an wall of the bowel).
underlying mass.  Auscultation of the abdomen in the
 Abdominal movement should be younger patients gives rather
assessed and abdominal palpation different signs than in the adults.
should be performed with warm The infant even in the presence of
hands. peritonitis may have some bowel
 The edge of the liver can sounds present.
normally be felt in the new  However, in the presence of ileus
born baby or peritonitis breath sounds
 The spleen can only be felt if it become conducted down over the
is pathological abdomen to the suprapubic area
 The kidneys can be felt in the and in even more severe disorders
first 24 hours with the fingers the heart sounds similarly can be
and thumb palpating in the heard extending down over the
renal angle and abdomen on abdomen to the suprapubic area.
each side.
INSPECTION
 The lower abdomen should be
palpated for the bladder and an  General distention.
enlargement can be confirmed by  Superficial veins- direction of
percussion from a resonant zone, flow, striae, umbilicus.
progressing to a dull zone.  Masses, scars, visible peristalsis.
 In a baby with abdominal PALPATION AND
distention where there is suspicion PERCUSION
of perforation and free gas in the
 First lightly palpate the entire
abdomen the loss of superficial
abdomen, keep looking at the
liver dullness on percussion may
child’s face all the time.
be the only physical sign present
 Localized tenderness, rebound
early on.
tenderness and rigidity
 Areas of tenderness can be elicited
 Masses
by watching the baby’s reaction to
 Ascites-percuss for the shifting
gentle palpation of the abdomen.
dullness
 There may be areas of erythema,
 Spleen, liver and kidneys
cellulitis and edema of the
abdominal wall and on deeper  Hernial orifices
palpation crepitus can occasionally  Genitalia (testes) and anus (site)
be felt from pneumatosis

32
AUSCULTATION  All older children should be
 Bowel sounds: absence implies observed for gait to detect
ileus abnormal coordination and
balance.
EXAMINATION OF  Older children may be tested for
GENITALIA AND sense.
RECTUM

STOOL AND URINE
EXAMINATION
 Examination of a stool which
preferably fresh is often
EXAMINATION OF THE informative.
NERVOUS SYSTEM  The color, consistency and smell
 The neurological examination of are noted as well as the presence of
the young infant and child is blood or mucus.
different from that routinely  Urine examination is also
carried out in the adult. important in children since
 Muscle tone and strength are symptoms related to the urinary
important parts of the examination. tract may be non-specicific
 In infants muscle tone may be (Urinalysis).
influenced by child’s state of  After a complete examination:
relaxation.  Document findings and
 Assess child’s developmental summarize.
levels relating to gross motor, finer  Provide a diagnosis with
motor, vision, hearing, speech and differentials.
social skills.  List appropriate investigations
along with management plan.

33
CHAPTER 5: IMMUNIZATION AND VACCINATION

IMMUNIZATON AND VACCINATION

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

34
SPECIFIC  Non-live or inactivated (IPV-
IMMUNIZATIONS SALK), administered
subcutaneously or
BCG
intramuscularly, has the
 Rationale for Vaccine: protection advantage of no vaccine-related
against TB polio but the disadvantage of
 Timing of vaccination (in not inducing secondary
Zambia): immunity.
 At birth  Timing of vaccination (in
 If no scar repeat dose at 12 Zambia):
weeks (3 months) unless in  At birth to 13 days: OPV0
symptomatic HIV  At 6 weeks: OPV 1
ORAL AND INACTIVATED  After at least 4 weeks of OPV1:
POLIO VACCINES (OPV/IPV) OPV2
 After at least 4 weeks of OPV
 Rational for vaccine: Poliovirus is
2: OPV3
an enterovirus with propensity for
 OPV 4 is given at 9 months
the CNS causing transient or
permanent paresis of the only if OPV 0 was not given
extremities and PNEUMOCOCCAL VACCINES
meningoencephalitis. (PCV)
 There are 2 types of vaccines  Rational for vaccine:
 Live attenuated (OPV-SABIN), Pneumococcus (Streptococcus
administered orally pneumoniae) is the most common
o Advantages: induction of cause of acute otitis media and
both host immunity and invasive bacterial infections in
secondary immunity children younger than 3 years of
because it is excreted in the age.
stool of the recipient and
 There are 2 types of vaccines:
may infect and thus  Pneumovax: composed of
immunize, close contacts polysaccharide capsular
(i.e. herd immunity) antigens from 23 pneumococcal
o Disadvantages: possibility serotypes.
of vaccine related polio in o Advantages: vaccine
host as well as contains antigens from
unimmunized contacts. pneumococcal strains
causing almost all cases of

34
 bacteremia and meningitis  Timing of vaccination (in
during childhood. Zambia):
o Disadvantages: little  At 6 weeks: PCV 1
immunogenicity in children  After 4 weeks of PCV 1: PCV
younger than 2 years. 2
o Indications: older children  After 4 weeks of PCV 2: PCV
and adults at high risk for 3
pneumococcal disease (e.g.
patients with sickle cell DIPTHERIA, PERTUSSIS,
anemia who are TETANUS, HEPATITIS B AND
functionally asplenic, HEMOPHILUS INFLUENZAE
immunodeficiency, chronic TYPE B (DPT-HepB-HiB)
liver disease and nephrotic  Rationale for vaccine:
syndrome, and patients  Diphtheria, tetanus and
with anatomic asplenia) pertussis all may cause serious
 Prevnar: composed of 7 disease, especially in young
pneumococcal serotypes infants.
o Advantages:  Hepatitis B infects 300 million
immunogenicity and worldwide.
efficacy in preventing  H. influenza type B was a
meningitis, pneumonia, serious cause of invasive
bacteremia and otitis media bacterial infection, including
from the most common meningitis, epiglottitis and
pneumococcal strains in sepsis before vaccine licensure
children younger than 2 in 1985. Since licensure it has
years of age. become a rare cause of such
o Disadvantages: does not infection.
confer as broad coverage  Vaccine is inactivated.
against pneumococcal  Timing of vaccination (in
strains as Pneumovax. Zambia):
o Indication: all children  At 6 weeks: DPT-HepB-HiB 1
younger than 2 years of age  After 4 weeks of DPT-HepB-
and selected children older HiB 1: DPT-HepB-HiB 2
than 2 years of age who are  After 4 weeks of DPT-HepB-
at high risk for HiB 2: DPT-HepB-HiB 3
pneumococcal disease.

35
MEASLES, MUMPS AND  Type of vaccine: live attenuated
RUBELLA vaccine
 Rational for vaccine: immunizes  Timing of vaccination (in
against 3 viral diseases: Zambia):
 Measles: a severe illness with  At 9 months or soon after
complications that include unless if symptomatic HIV
pneumonia associated with  At 18 months or soon after
significant mortality. unless if symptomatic HIV
 Mumps: most commonly ROTA VACCINE
associate with parotitis but may
 Rationale for vaccine: protection
also cause meningoencephalitis
against viral diarrhea causes by
and orchitis
Rota virus.
 Rubella: causes a mild viral
 Timing of vaccination (in
syndrome in children but may
Zambia):
cause severe birth defects in
 At 6 weeks: ROTA 1
offspring of susceptible women
 After 4 weeks of ROTA 1:
infected during pregnancy
ROTA 2

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 HIVDate .……………….
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-
Date ………………….. HepB-HiB 1)
Date …………………..
OPV 3 DPT-HepB-HiB 3

36
 (at least 4 weeks after OPV2) (at least 4 weeks after DPT-
Date ………………….. HepB-HiB 2)
Date …………………..
OPV 4 Measles
(at 9 months, only if OPV 0 Rubella
was not given) (at 9 months or soon after
Date ………………….. unless symptomatic HIV)
Date …………………..
PCV 1 Measles
(at 6 weeks) Rubella (at 18 months or soon
Date ………………….. after unless symptomatic HIV)
Date …………………..
PCV 2 ROTA VACCINE 1 (at 6
(at least 4 weeks after PCV 1) weeks)
Date ………………….. Date …………………..
PCV 3 ROTA VACCINE 2
(at least 4 weeks after PCV 2) (at least 4 weeks after ROTA 1)
Date ………………….. Date …………………..

ADVERSE EFFECTS OF CONTRAINDICATIONS

IMMUNIZATION AND PRECAUTIONS TO
 Most vaccine side effects are mild IMMUNIZATION
to moderate in severity and occur CONTRAINDICATIONS
within the first 24 hours after 1. Anaphylaxis to a vaccine or its
administration e.g. local constituents
inflammation and low grade fever. 2. Encephalopathy within 7 days
 Because MMR and varicella after DTaP vaccine
vaccines are live attenuated 3. Patients with progressive
vaccines fever and rash may occur neurologic disorders including
1-2 weeks after immunization (i.e. uncontrolled epilepsy should not
after the incubation period of the receive the DTaP vaccine until
virus) neurologic status is stabilized.
 Serious side effects that may result 4. Immunodeficient patients should
in permanent disability or be life- not receive OPV, MMR and
threatening are rare (e.g. vaccine Varicella vaccine.
related polio after OPV)

34
  Household contacts of  Collapse or shocklike state
immunodeficient patients within 48hours after prior
should not receive OPV as it is vaccination
shed in stool  Seizures within 3 days after
5. Pregnant patients should not prior vaccination
receive live vaccines.  Persistent, inconsolable crying
lasting more than 3 hours
PRECAUTIONS occurring within 48 hours after
 For all vaccines, moderate to prior vaccination
severe illness (with or without  MMR and Varicella vaccines
fever) are not contraindication to  Immunoglobulin (IVIG)
immunization. administration within the
 DTaP vaccine: preceding 3-11 months which
 Temperature of 40.5OC within might interfere with the
48hours after prior vaccination patient’s immune response to
these vaccines.

34
 CHAPTER 6: PEDIATRIC GENETIC DISORDERS

PEDIATRIC GENETIC DISORDERS

DOWN SYNDROME CYTOGENETICS
 The extra-chromosome 21 may
(TRISOMY 21)
result from
 This is the most common
 Meiotic non-disjunction (94%)
autosomal trisomy and the most
 Translocation (5%)
common genetic cause of severe
 Mosaicism (1%)
learning difficulties.
 Karyotype of the parent is only
 The incidence (without antenatal
required if the affected child has
screening) in live-born infants is
translocation underlying Down
about 1 in 1000.
syndrome.
 The syndrome was named after
Langdon Down, who first coined MEIOTIC NON-DISJUNCTION
the term monoglism because of the  Accounts for about 94% of the
mongoloid facial appearance of cases.
patients with the syndrome.  Most cases result from an error at
Nowadays the term mongolism is meiosis.
obsolete.  The pair of chromosome 21s fails
 Chromosome 21 is present in to separate, so that one gamete has
triplicate, the origin of the extra 2 chromosome 21s and one has
chromosome being either paternal none.
or maternal.  Fertilization of the gamete with
 Down’s syndrome occurs more two chromosome 21s gives rise to
commonly in mothers conceiving a zygote with trisomy 21.
at older age. This is attributed to  Parental chromosomes do not need
the exposure of the maternal to be examined.
oocyte to harmful environmental  The incidence of trisomy 21 due to
influences for a longer period since non-disjunction is related to
Graafian follicles are present in the maternal age.
fetal life and exist through female
reproductive life.
 The sperm has a short lifespan and
therefore has less chances of
injurious exposure.

34
 Meiotic non-disjunction can occur  Parental chromosomal analysis is
in spermatogenesis so that the recommended since one of the
extra 21 can be of paternal origin. parents may well carry the
translocation in balanced form
TRANSLOCATION (25% of cases)- rearranged genetic
 Accounts for 5% of all the cases. material (in mother or father) but
 Occurs when the extra no extra-chromosomes.
chromosome 21 is jointed onto  Balanced carriers have no signs
another chromosome (usually or symptoms of Down
chromosome 14 –Robertsonian syndrome but can pass the
translocation, but occasionally translocation to the children.
chromosome 15, 22 or 21).  In translocation Down syndrome:
 This may be present in a  Risk of recurrence is 10-15% if
phenotypically normal carrier with the mother is the translocation
45 chromosomes (2 being ‘joined carrier and about 2.5% if the
together’) or in someone with father is the carrier.
Down syndrome and a set of 46  If a parent carries the rare 21:21
chromosomes but 3 copies of translocation all the offspring
chromosome 21 material. will have down syndrome.
 If neither parents carry a
translocation (75% of cases) the
risk of recurrence is <1%.

34
MOSAICISM o Upslanting palpebral
 Accounts for 1%. fissures (Mongolian slant to
 Some cells are normal and some eyes)
have trisomy 21. o Brushfield spots in iris
 This usually arises after the (speckled irides)
formation of chromosomally o Flat nasal bridge
normal zygote by non-disjunction o Folded or dysplastic ears
at mitosis but can arise by later o Open mouth (macroglossia-
mitotic disjunction in a trisomy 21 protruding tongue,
conception. microstonia- small mouth)
 The phenotype is sometimes o Short neck
milder in Down syndrome o Excessive skin at the nape
mosaicism. of the neck
o Characteristic grimace
RISK FACTORS when crying
1. Advancing age  Extremities:
o 35 years 1 in 350 o Hypotonia
o 40 years 1 in 100 o Short broad hands
o 45 years 1 in 30 o Single palmar creases
2. Having had a child with down (simian crease)
syndrome (translocation) o Short, Incurved fifth finger
3. Carriers of genetic translocation due to Clinodactlyly
for down syndrome (hypoplasia of middle
phalanx of 5th finger)
CLINICAL FEATURES o Wide ‘sandal’ gap between
 Most affected infants are the big and second toe.
hypotonic o Hyperflexible joints
 Clinical signs include:  Other: duodenal atresia,
 Craniofacial features: congenital heart defects (40%)
o Brachycephaly (skull is and Hirschsprung disease
shorter than usual)  Neonatal features:
o Flat occiput and third o Flat facial profile
fontanelle o Round face
o Epicanthal folds (a fold of o Fine soft and sparse hair
skin running across the o Small dysplastic ears that
inner edge of the palpebral are low set
fissure) o Poor Moro reflex

35
 o Excessive skin at the nape o It ranges from mild to
of 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
should be preset for making a years, reaches a plateau at
diagnosis clinically. adolescence and continues
 Mental retardation through out adulthood.
o Almost all have mental
retardation

36
DIAGNOSIS  The results may take 1-2 days
 Diagnosis can be difficult to make using rapid FISH (fluorescent in
when relying on clinical signs situ hydridisation) techniques.
alone and a suspected diagnosis  Diagnosis is confirmed by
should be confirmed by a senior karyotype.
paediatrician.  Parents need information about the
 History (usually from a third short and long term implications of
party): the diagnosis.
o Determine patient’s age and  It is difficult to give a precise long
obtain presenting complaint term prognosis in the neonatal
o In History of presenting period, as there is individual
complaint include parental variation in the degree of learning
age, previous eyeglass difficulty and the development of
prescription, occlusion complications.
therapy, onset of strabismus  Important steps after suspecting
and/or nystagmus, previous down syndrome in a neonate:
external infections and  Establish diagnosis
treatment modalities,  Thorough clinical examination
tearing and photophobia. to observe for congenital heart
o Review pulmonary, CVS disease or gastrointestinal
(including any previous malformation
cardiovascular surgery),  Inform the parents in a
gastrointestinal and supportive way
neurological systems.  Laboratory orders:
o Ask for history of any other o Chromosomal karyotype
siblings with down o Thyroxine
syndrome. o TSH
 Examination: 8 or more o Full blood count with
characteristic clinical findings differential
lead to a definite diagnosis. In o Platelet count
doubtful cases, chromosomal o Echocardiography
analysis may be necessary. o Hearing screen
 Before blood is sent for analysis, o Fundocscopy
parents should be informed that a  Discuss diagnosis and
test for Down syndrome is being management plan with parents
performed.

34
  Refer parents to an infant CONGENITAL HEART
intervention program and to a DISEASE
support group  50% have endocardial cushion
 Conduct a follow-up meeting defects ranging from isolated
with family septal defects (ASD, VSD) to total
COMPLICATIONS AND absence of septum.
ASSOCIATED  Murmur on auscultation is due
to turbulent flow of blood
ABNORMALITIES
through defect.
CENTRAL NERVOUS SYSTEM  Other defects: PDA, Tetralogy of
 Delayed motor milestones fallot, Mitral valve prolapse,
 Moderate to severe learning Aortic and mitral regurgitation.
difficulties  Presence of heart disease is the
 Early onset Alzheimer’s disease most significant factor in
 Epilepsy determining survival.
 Alerting signs are poor feeding,
EYE PROBLEMS
easy fatigability, dyspnea,
 Increased risk of cataract,
diaphoresis, cyanosis and a cardiac
nystagmus, squint and
murmur.
abnormalities of visual acuity.
 Routine evaluation is performed in HEMATOLOGICAL
infancy and then yearly.  Patients with Down syndrome are
at increased risk of development of
HEARING DEFECT
lymphoproliferative disorders,
 May be uni-/bilateral, conductive
including acute lymphoblastic
or sensorineural or mixed.
leukemia, acute myeloid leukemia,
 40-60% have conductive hearing myelodysplasia and transient
loss and are prone to serious otitis lymphoploriferative syndrome.
media (most commonly during the
 Other disorders include
first year).
polycythemia, thrombocytopenia
 Routine evaluation before 6 and erythroblastosis fetalis.
months of age and then every year
is advisable GASTROINTESTINAL
MALFORMATION
 Duodenal atresia present in 5% of
cases.

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

36
 Cardiac disease screening  Patients with Down syndrome
 Hearing- every 3 months until 3 have a shortened life expectancy.
years and then annually. About 1/3 die within the 1st year,
 Eye disorder screening and ½ die as they reach 4 years.
 Thyroid function test The remainder of patients have
 Celiac disease screening reduced life expectancy as
 Hematology- FBC compared to the general
 Atlanto-axial X-ray population.

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

34
 valve prolapse, aortic valve  Renal malformations like
stenosis, and hypoplastic left heart horseshoe kidney, reduplication of
are common. the renal pelvis and agenesis may
 Hypothyroidism and diabetes also be present.
mellitus may occur.

DIAGNOSIS  Echocardiography and

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

34
 view of high risk of
gonadoblastoma.

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 (<9th
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
KLINEFELTER  Occasionally, hypospadias or
 Klinefelter is the most common cryptorchidism is present.
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.
 Gynaecomastia (40%)
DIAGNOSIS
 Variable intelligence
 Based on chromosomal analysis
 Behavioral findings include
 The diagnosis should be
antisocial behavior and excessive
considered in all boys with mental
shyness or aggression. These
retardation, as well as in children
findings may be noted before the
with psychosocial, learning
appearance of the physical
disability or school adjustment
findings.
problems.

34
MANAGEMENT  Testosterone therapy should be
 Behavioral and psychosocial started in middle to late
rehabilitation. 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 91st 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

HYDROCEPHALUS atrophy. It is not true

 This is increased cerebrospinal hydrocephalus.
fluid (CSF) under pressure within ETIOLOGY
the ventricles of the brain.
CONGENITAL CAUSES
 It results from blockage of CSF
 Arnold Chiari type II
flow, decreased CSF absorption or
malformation: characterized by
rarely increased CSF production.
downward displacement of the
TYPES OF cerebellum and medulla through
HYDROCEPHALUS the foramen magnum, blocking
 They include: CSF flow. This malformation is
 Non-communicating often associated with a
hydrocephalus: enlarged lumbosacral myelomeningocele.
ventricles caused by  Dandy-Walker malformation: a
obstruction of CSF flow combination of an absent or
through the ventricular system hypoplastic cerebellar vermis and
(e.g. stenosis of the aqueduct of cystic enlargement of the fourth
sylvius) ventricle which blocks the flow of
 Communicating hydrocephalus: CSF.
enlarged ventricles as a result  Congenital aqueductal stenosis
of increased production of CSF (Some cases of aqueductal stenosis
(e.g. tumors) or decreased are inherited as an X-linked trait
absorption of CSF (e.g. and these patients may have thumb
bacterial meningitis) abnormalities and other CNS
 Hydrocephalus ex vacuo: this is anomalies such as spina bifida)
enlargement of ventricular
system due to compensatory
response to severe cortical

34
ACQUIRED CAUSES
 Causes include intraventricular
hemorrhage (most common in
preterm infants), bacterial
meningitis and brain tumors.
CLINICAL FEATURES
 History
 Did the child have any fever
before the head started getting
big?  Older children with closed cranial
 Any history of convulsions? sutures have the symptoms and
 What was the baby’s head signs of increased intracranial
circumference at birth and was pressure:
the baby born at term or  Headache
preterm?  Nausea and vomiting
 Did the child cry immediately  Unilateral sixth nerve palsy:
after birth? convergent strabismus or
 At what age did the caregiver esotropia (one or both eyes turn
notice the head getting bigger? inward) of which the primary
 Any abnormality of the spine? symptom is diplopia (double
 Any similar illness in the vision)
siblings?  Papilledema
 What treatment was given and  Brisk deep tendon reflexes but
for how long? with a usually downward
 Increasing head circumference that plantar response.
crosses percentile lines or head
EVALIATION
circumference >97% for age.
 Increasing head circumference and
 Infants with open cranial sutures
signs or symptoms of increased
have the following clinical signs:
intracranial pressure mandate an
 Large anterior and posterior
urgent CT scan.
fontanelles and split sutures.
 Sunset sign, a tonic downward MANAGEMENT
deviation of both eyes caused  Requires the surgical placement of
by pressure from the enlarged a ventriculoperitoneal shunt to
third ventricle on the upward divert the flow of CSF.
gaze center in the midbrain.  Other shunts:

35
 o Ventricular-lumbar shunt  Patients with aqueductal stenosis
o Ventricular-atrial shunt have the best cognitive outcome.
o Ventricular-pleural shunt  Patients with Chiari type II
o Ventricular-spinal shunt malformation may have low-
o Ventricular-osseus shunt normal intelligence and language
 Complications of disorders.
ventriculoperitoneal shunts include  Patients with X-linked
shunt infection and shunt hydrocephalus may have severe
obstruction. mental retardation.
 Acute: infection, slit ventricular
syndrome (small ventricles CEREBRAL PALSY
formed in shunt).  Cerebral palsy is a termed used for
 Long term: shunt becoming static/ non-progressive disorders of
short as the child is growing, the brain affecting the
blocked shunt, dislodged shunt development of movement,
and shunt nephritis. posture and coordination acquired
early in brain development.
 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
Figure 7: Hydrocephalus. Axial CT scan shows marked
dilatation of both lateral ventricles 2 years. Beyond this age it is more
appropriate to use acquired brain
PROGNOSIS injury as the diagnosis.
 Outcome depends on the cause  Although the underlying cause is
static the resulting motor disorder

36
 may evolve, giving the impression  Hypoxia
of deterioration.
 The diagnosis for each child
PERINATAL
should formulate:  Prematurity <37 weeks
 The distribution of the motor (approximately half the cases of
disorder CP are associated with preterm
 The movement type delivery & low birth weight).
 The cause  Periventricular leukomalacia
 Any associated impairment  Intracerebral hemorrhage
 In neonates the diagnosis may be  Birth asphyxia
suspected if a baby has difficulty  HIE in full term: affects thalami,
sucking, irritability, convulsions or basal ganglia, cortex or sub-
an abnormal neurological cortical white matter
examination.  Birth trauma
 Prolonged, precipitous delivery
CAUSES
 Around 80% of cerebral palsy is POSTNATAL (BEFORE 2
antenatal (prenatal) in origin due to YEARS)
vascular occlusion, cortical  Hypoglycemia
migration disorders or structural  Hyperbilirubinemia
maldevelopment of the brain  Meningitis/encephalitis
during gestation.  Subdural hematoma
 Maternal risk factors include:  Acute infantile hemiplegia
Multiple gestation, Preterm labor.  Head trauma
 The causes of Cerebral palsy can  Cardiopulmonary arrest
generally be classified as prenatal,
perinatal and postnatal. CLINICAL PRESENTATION
 Numerous children who develop
PRENATAL CP will have been identified as
 Genetic factors being at risk in the neonatal period.
 Cerebral malformations  Diagnosis is usually made in the
 Intrauterine infection during first year when the early features
pregnancy (congenital infection- emerge.
TORCH infections)  Early features of CP are:
 Radiation  Abnormal limb and/or trunk
 Cerebral infarcation posture and tone in infancy:
 Metabolic defects initially the tone may be

37
 reduced but eventually SPASTIC CEREBRAL PALSY
spasticity develops.  There is damage to the upper
 Delayed motor milestones: motor neuron (pyramidal or
delays in sitting and rolling corticospinal tract) pathway.
over, accompanied by slowing  Limb tone is persistently increased
of head growth. (spasticity) with associated brisk
 Abnormal patterns deep tendon reflexes and extensor
 Feeding difficulties, with plantar responses.
oromotor incoordination, slow  Tone in spasticity is velocity
feeding, gagging and vomiting. dependent, so the faster the muscle
 Abnormal gait once walking is is stretched the greater the
achieved resistance it will have. This elicits
 Asymmetric hand function a dynamic catch which is the
before 12 months of age hallmark of spasticity.
 Persistence of primitive  The increased limb tone may
reflexes: such as the moro, suddenly yield under pressure in a
grasp and asymmetric tonic ‘clasp knife’ fashion.
neck reflex.
 Limb involvement is increasingly
 In CP primitive reflexes which described as unilateral or bilateral
facilitate the emergence of normal to acknowledge asymmetrical
patterns of movement and which signs.
need to disappear for motor
 Spasticity tends to present early
development to progress may
and may even be seen in the
persist and become obligatory.
neonatal period.
 The diagnosis is made by findings
 Sometimes there is initial
of abnormalities of tone, delays in
hypotonia, particularly of the head
motor development, abnormal
and trunk.
movement patterns and persistent
 There are 3 main types of spastic
primitive reflexes. Diagnosis may
cerebral palsy:
be suspected in neonates but can
 Hemiplegia: characterized by
only be made months later.
unilateral spastic motor
 There are 4 main clinical subtypes:
weakness.
 Spastic (90%)
o Unilateral involvement of
 Dyskinetic (6%)
the arm and leg.
 Ataxic (4%)
 Mixed

38
o The arm is usually affected
more than the leg with the
face spared.
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  Quadriplegia: characterized by
(toe-heel gait) on the motor involvement of head,
affected side may become neck and all 4 limbs
evident. o All four limbs are affected
o Past medical history may severely.
be normal, with an o The trunk is involved with
unremarkable birth history a tendency to opisothonus
with no evidence of HIE. (extensor posturing), poor
o In some, the condition is head control and low
caused by neonatal stroke. central tone.
Larger brain lesions o This more severe cerebral
(Strokes) may cause palsy is often associated
hemianopia (loss of half of with seizures, microcephaly
visual field) of the same and moderate or severe
side as the affected limbs. intellectual impairment.
o Most implicated risk o There may have been a
factors: perinatal vascular history of perinatal HIE,
insults, postnatal trauma, CNS infections, trauma,
CNS malformations. malformations.
o This is also associated with
seizures, scoliosis,
weakness of face and
pharyngeal muscles,
dysphagia,

39
 gastroesophageal reflux or o All 4 limbs but the legs are
aspiration pneumonia, affected to a much greater
failure to thrive, speech degree than the arms so that
problems and sensory hand function may appear
impairments. 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.
 Diplegia: involves the lower
o There may be history of
extremities more than the upper
early rolling over.
extremities or face
DYSKINETIC CEREBRAL  Chorea- irregular, sudden and
PALSY brief non-repetitive movements.
 This refers to movements which  Athetosis- slow writhing
are involuntary, uncontrolled, movements occurring more
occasionally stereotyped and often distally such as fanning of the
more evident with active fingers.
movement or stress.  Dystonia- simultaneous
 Arms are usually more affected contraction of agonist and
than legs. antagonist muscles of the trunk
and proximal muscles often
 Muscle tone is variable and
giving a twisting appearance.
primitive motor reflex patterns
predominate.  Intellect may be relatively
unimpaired.
 May be described as:

40
 Affected children often present  The signs are due to damage or
with floppiness, poor trunk control dysfunction in the basal ganglia or
and delayed motor development in their associated pathways
infancy. (extrapyramidal).
 Abnormal movements may only  The commonest cause was
appear towards the end of the first hyperbilirubinemia (kernicterus)
year of life. due to rhesus disease of the
newborn but it is now HIE at term.
ATAXIC (HYPOTONIC)  In-coordinate movements,
CEREBRAL PALSY intention tremor and an ataxic gait
 Most are genetically determined. may be evident later.
 When due to acquired brain injury
(cerebellum or its connection), the
signs occur on the same side as the MIXED TYPE
lesion but are usually relatively  A proportion of the patients have
symmetrical. features of diffuse neurological
 There is early trunk and limb involvement of the mixed type.
hypotonia, poor balance and
delayed motor development.

35
DIAGNOSIS significant symptoms. Lab
 Diagnosis is made on clinical investigations are necessary.
grounds, with repeated  Hydrocephalus and subdural
examinations often required to effusion: head size is large,
establish the diagnosis. fontanel may bulge and sutures
 Diagnosis should be suspected in a may separate.
child with low birthweight and  Brain tumors or space occupying
perinatal insult, clinically has an lesions: features of increased ICP.
increased tone, feeding difficulties  Muscle disorders: Congenital
and global development delay. myopathies and muscular
 Abnormalities of tone posture, dystrophies can mimic cerebral
involuntary movements and palsy. Distribution of muscle
neurological deficits should be weakness and other features is
recorded. characteristic, hypotonia is
 CT or MRI scan may be useful in associated with diminished
demonstrating cerebral reflexes.
malformations, delineating their  Ataxia-telangiectasia: ataxic may
extent and ruling out very rare appear before the ocular
progressive or treatable causes telangiectasia are evident
such as tumors.  Spinal cord injury
 In born errors of metabolism
MANAGEMENT
should be excluded by screening of
 Parents should be given details of
the plasma amino acids and urine
the diagnosis as early as possible,
organic acid, reducing substance.
but prognosis is difficult during
DIFFERENTIAL DIAGNOSIS infancy until the severity and
 Neurodegenerative disorders: there pattern of evolving signs and the
is progressively increasing child’s developmental progress
symptoms, familial pattern, have become clearer over several
consanguinity, specific months or years of life.
constellation of signs and  Management plan should be
symptoms are clues for holistic and involve the family.
neurometabolic disorders. Failure  Children with cerebral palsy are
to thrive, vomiting, seizures are likely to have a wide range of
associated medical, psychological

37
 and social problems, making it cerebral palsy can cope at
essential to adopt to a mainstream school provided minor
multidisciplinary approach to learning difficulties and physical
assessment and management. access are addressed otherwise
 Physiotherapy: to advise on those with severe CP need special
handling and mobilization. To to be in special schools for a
minimize effects of spasticity disabled.
and prevent contractures.  Prognosis depends on the degree
 Occupational therapy: to advise and type of cerebral palsy, level of
on equipment such as learning disability and presence of
wheelchairs and seats and on other associated problems.
play materials and activities  The degree of independent living
that best encourage the child’s relates to:
hand function  Type and extent of cerebral
 Speech therapy palsy
 Pediatric management: monitor  Degree of learning disability
foe developmental progress,  Presence of associated
medical problems, development problems e.g. visual
of contractures or dislocations, impairment, epilepsy
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.
 Children with CP need a lot of
help in everyday tasks such as
dressing, bathing, feeding.
Children with milder forms of

37
MENINGITIS
 This is inflammation of the meninges.
 It is classified as:
 Bacterial
 Aseptic
BACTERIAL MENINGITIS
 The highest incidence of bacterial meningitis is during the first month of life.
ETIOLOGY
 Causes of infection are based on the age of the child:
 0-1 month: Group B streptococcus, Escherichia coli, Listeria
monocytogenes
 1-3 months: Group B streptococcus, Streptococcus pneumonia, Listeria
monocytogenes
 3months- 3 years: Streptococcus pneumoniae, Haemophilus influenza type
B, Neisseria meningitidis
 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
 Young age
 Immunodeficiency (e.g. asplenia, humoral-mediated immunodeficiency and
terminal complement deficiency)
 Anatomic defects (e.g. basilar skull fracture, ventriculoperitoneal shunt)
CLINICAL FEATURES
 Infants and young children often have minimal and nonspecific signs and
symptoms
 Poor feeding, irritability (high pitched cry), lethargy, respiratory distress.
 Fever may be absent or minimal.
 A bulging fontanelle may be present on physical examination
 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)
 Seizures
 Photophobia
 Emesis
 Headache
DIAGNOSIS
 Index of suspicion for bacterial meningitis should be especially high in febrile,
irritable infants.
 Evaluation should include:
 Lumbar puncture, shows:
o Pleocytosis 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
o Increased protein
o Positive Gram stain and culture
o Bacterial antigens may be tested but have a low sensitivity and are not
recommended on a routine basis.

37
 o Pretreatment with antibiotics may sterilize the CSF culture but should
not alter the CSF cellular and biochemical profile above.
 Blood culture: positive in the majority of cases of bacterial meningitis
 CT scan with contrast to evaluate for brain abscess is often recommended,
especially for patients with focal neurologic findings.
MANAGEMENT
 Early empiric treatment of bacterial meningitis is critical.
 Antibiotics: third generation cephalosporin
 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.
 Supportive care:
 Fluid rehydration with monitoring of urine output
COMPLICATIONS
 Complications are highest with Gram-negative organisms followed by S.
pneumoniae, H. influenza B and finally N. meningitidis.
 Hearing loss is the most common complication occurring in up to 25%of
patients.
 Global brain injury occurs in 5-10% of patients.
 Other complications:
 SIADH
 Seizures
 Hydrocephalus
 Brain abscess
 Cranial nerve palsy
 Learning disability
 Focal neurologic deficits
ASEPTIC MENINGITIS

38
 36

SEIZURE DISORDERS  Extent of brain involvement

 Whether consciousness is
OF CHILDHOOD impaired or not
 A seizure is an abnormal  Nature of the movements
paroxysmal excessive firing of the  Generally, they can be classified
cerebral neurons that may involve into 2 broad categories:
transient, involuntary alteration of  Febrile seizures: common but
consciousness, behavior, motor benign seizures associated with
activity, sensation or autonomic fever. Include:
function. o Simple
 Epilepsy is the occurrence of 2 or o Complex
more spontaneous seizures without  Afebrile seizure: Either
an obvious precipitating cause. classified as generalized or
 Status epilepticus is a seizure that partial depending on whether
lasts more than 5 minutes or when both sides or one side of the
seizures occur close together i.e. 2 brain are involved as well as
or more seizures within a 5-minute whether consciousness is
period without the person impaired or not
returning to normal between them. o Generalized seizures:
 Previous it was described as a caused by discharge from a
seizure that lasts more than 30 group of neurons in both
minutes. cerebral hemispheres.
 The seizure discharge is caused by Include
an imbalance between excitatory  Tonic-clonic/ Grand-
and inhibitory input within the mal (common): this is
brain or abnormalities in the the most common type
membrane properties of individual of generalized seizure.
neurons. They are characterized
 In some children the cause of by increased thoracic
seizures is known however in 60- and abdominal muscle
70% of cases the cause is tone. Followed by clonic
unknown. movements of the arms
and legs, eyes, rolling
CLASSIFICATION OF
upward, incontinence,
SEUZIRES decreased consciousness
 Criteria for classification are: and a postictal state of
 Presence or absence of fever 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. FEBRILE SEIZURES
The seizure lasts <15  A febrile seizure is any seizure that
seconds and there is no is accompanied by a fever (Temp>
postictal state. 38.5oC) and is not caused by any
 Atonic CNS infection such as meningitis
o Partial (focal) seizures are in neurologically normal patients
caused by the discharge from 6months to 6 years of age.
from a group of neurons in
 The pathophysiologic mechanism
one hemisphere. The
is unknown.
seizure symptoms may
 Febrile seizures can be inherited
have predominately motor,
and several gene mutations have
sensory or psychomotor
been implicated.
features. There are 2 types:
 Febrile seizures occur in about 3%
 Simple: consciousness is
of children.
not impaired.
 Complex partial CLASSIFICATION
seizures: consciousness  Simple/benign febrile seizure:
is decreased. 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

37
  Complex/ atypical febrile seizure:  First time or occasional febrile
lasts longer than 15 minutes, has seizures are not treated with
focal features or recurs within 24 anticonvulsants.
hours.  Aggressive antipyretic treatment of
o These are associated with subsequent febrile illnesses may
post-ictal focal deficit. help prevent febrile seizures.
o Complex febrile seizures  Tepid sponging is no longer
which are focal, prolonged recommended.
or repeated in the same  Frequent recurrent febrile seizures
illness have an increased do pose a risk and may require
risk of 4-12% of subsequent additional treatment including:
epilepsy.  Daily anticonvulsant
DIAGNOSIS prophylaxis with valproic acid
or phenobarbital
 Based on history, a normal
 Abortive treatment with rectal
neurologic examination and the
diazepam
exclusion of any CNS infection.
 Infection of midazolam or
 Examination should focus on the
diazepam (0.2-0.3 mg/kg/dose)
cause of the fever, which is usually
is given for control of seizures
a viral illness, but a bacterial
 The family should be taught the
infection including meningitis
first aid management of seizures.
should always be considered.
If there is a history of prolonged
 A lumbar puncture is necessary
seizures (>5 min), rescue therapy
only if meningitis is suspected.
with rectal diazepam or buccal
 If the child is unconscious or
midazolam can be supplied.
has cardiovascular/respiratory
 Prognosis: approximately 30% of
instability, lumbar puncture is
patients with 1 febrile seizure will
contraindicated and antibiotics
have a recurrence. Recurrence risk
should be started immediately.
decreases with increasing patient
 Neither neuroimaging nor EEG is
age. The risk of epilepsy is low
needed unless the neurologic
(2%).
examination is abnormal.
CHAPTER 8: RESPIRATORY CONDITIONS
MANAGEMENT
 Maintenance of airway, breathing
and circulation.

38
 RESPIRATORY CONDITIONS
RELEVANT ANATOMY  The first and last stages i.e.
embryonic and alveolar stages are
AND PHYSIOLOGY OF almost 5 weeks in duration (but the
THE RESPIRATORY alveolar stage continues after birth
SYSTEM to childhood) and the middle 3
stages i.e. pseudoglandular,
DEVELOPMENT
canalicular and terminal saccular
 By 16 weeks gestation, the
stages are almost about 10 weeks
bronchial tree has developed.
in duration. This is an easy way to
 By 26-28 weeks gestation
remember the stages of lung
sufficient air sacs and pulmonary
development in fetus.
vasculature have developed so that
the fetus is able to survive. EMBRYONIC PHASE
 90% of alveolar development  This phase is around 3-8 weeks of
occurs after birth and alveoli the gestational age.
increase in number until 8 years of  Two lung buds branch off forming
age. the right and left lung.
 During the nascent stage of  There is formation of
development, the lungs develop respiratory diverticulum (from
and gradually become more foregut endoderm in 4th week)
efficient and adapt to respiration. to formation of major
 Fetal lung development has 5 bronchopulmonary segments.
distinct stages:  The larynx and trachea develop
 Embryonic phase from the foregut.
 Pseudoglandular phase
 Canalicular phase PSEUDO-GLANDULAR PHASE
 Terminal saccular phase  The pseudo-gandular phase is
 Alveolar phase between the 5th-16th week of
 To remember these stages, recall gestation.
the mnemonic “Every Premature  There is formation of all the
Child Takes Air”. conducting airways (up to terminal
bronchioles-acinus).

39
 16 airway generations in humans  Growth of bronchioles and alveoli.
are completed by 16 weeks  Gas carrying tissue in the lungs
 No respiratory bronchioles or expands and becomes more
alveoli are present so respiration is efficient for carrying air during the
not possible. alveolar phase.
CANALICULAR PHASE ANATOMY
 Occurs between the 16-26th week  The right lung has 3 lobes while
of gestation. the left has 2 with a lingula.
 Respiratory bronchioles and  Infants are at a higher risk for
alveolar ducts form. respiratory insufficiency than older
 A few terminal sacs form towards children and adults because infants
the end of the stage- may rarely have anatomically smaller air
survive with intensive care. passages, less compliant (Stiffer)
 A barrier between air and blood lungs with more compliant chest
forms which enables oxygen to wall, and less efficient pulmonary
supply blood to respiratory mechanics.
capillary and carbon dioxide to  Congenital malformation of the
depart from the respiratory respiratory tract may be associated
capillaries in the lungs. with other congenital anomalies
especially of the cardiovascular
TERMINAL SACCULAR PHASE system.
 This occurs between the 26 to 37th
week of development (birth). PHYSIOLOGY
 Terminal sacs/ primitive saccule  Pulmonary vascular resistance
(alveoli) form. They are separated decreases after birth when the fetal
from each other by primary septa. pulmonary and systemic
 The production of surfactant starts. circulations separate and the lungs
 Surfactant is crucial during ventilate for the first time.
delivery since it allows the  The primary function of the lungs
amniotic fluid in the lungs to drain is gas exchange.
away and fills the lungs with air  Lung pathology may be classified
appropriately. as obstructive or restrictive:
 Obstructive defects: secondary
ALVEOLAR PHASE to decreased airflow through
 Lasts until early childhood (8 years narrowed airways. Examples
of age).

37
 include asthma, bronchiolitis  Family history: should include
and foreign body aspiration assessment for genetic diseases
 Restrictive defects: secondary e.g. cystic fibrosis, asthma.
to pulmonary processes that  Environmental history: is
decrease lung volume (the extremely important because
amount of air filling the fumes, strong odors, tobacco
alveoli). Examples include smoke, allergens, animals and day
pulmonary edema, scoliosis, care attendance may cause or
pulmonary fibrosis and exacerbate pulmonary disease.
respiratory muscle weakness.

CLINICAL ASSESSMENT PHYSICAL EXAMINTION

OF PULMONARY  Should emphasize the chest and
DISEASE respiratory system
HISTORY  In general assessment, assess for
 History is most important in the evidence of increased work of
diagnosis. breathing such as tachypnea
 Antenatal, prenatal and neonatal (Normal respiration rate in new
histories are very important borns is 30-50 and infants + the
because complications of rest=20-30 B/min), nasal flaring,
pregnancy, fetal or postnatal expiratory grunting and chest wall
tobacco exposure, prematurity and retractions.
airway instrumentation can cause  Evaluate ears, nose, and throat for
pulmonary problems. signs of obstruction, atopy or
 Review of systems: should include infection.
documentation of atopy (Asthma),  Perform a chest examination:
failure to thrive or steatorrhea  Inspiratory stridor suggests
(cystic fibrosis), choking extrathoracic obstruction e.g.
(aspiration) or recurrent infections croup and laryngomalacia
(immunodeficiency). (softening and weakness of
 Past medical history: should laryngeal cartilage that
include previous respiratory collapses into the airway,
problems including frequent especially when in the supine
respiratory tract infections, cough, position).
wheeze, stridor, snoring and
exercise intolerance.

38
  Expiratory wheezing suggests for laboratory analysis. Common
intrathoracic obstruction, as in indications include persistent
asthma and bronchiolitis. pneumonia, cough, stridor or
 Crackles/rales/crepitations wheezing.
suggests parenchymal disease
such as pneumonia and INFECTIOUS DISEASES
pulmonary edema. (UPPER RESPIRATORY
 Assess for related findings in other TRACT)
organs: e.g. heart murmurs,  The term upper respiratory tract
increased second heart sound infection embraces a number of
(elevated pulmonary pressure), different conditions including:
eczema, and digital clubbing.  Common cold (coryza)
 Sore throat (pharyngitis,
including tonsillitis)
INVESTIGATIONS  Acute otitis media
 Pulse oximetry: measures oxygen  Sinusitis (relatively
saturation. uncommon)
 Arterial blood gases (ABG): gold  The commonest presentation is a
standard to measure oxygenation child with a combination of nasal
(PO2) and ventilation (PCO2). discharge and blockage, fever,
 Imaging studies: painful throat and earache. Cough
 Chest X-ray (CXR) may be troublesome.
 Computed tomography (CT)  Upper respiratory tract infections
scan may cause:
 Magnetic resonance imaging  Difficulty in feeding in infants
(MRI) as their noses are blocked and
 Nuclear studies e.g. ventilation- this obstructs breathing.
perfusion scans  Febrile convulsions
 Pulmonary function tests: measure  Acute exacerbations of asthma.
airflow as a function of time  Admission may be required in to
(spirometry) and lung volumes. exclude a more serious infection, if
 Laryngoscopy and bronchoscopy feeding is inadequate or for
are performed in some conditions parental reassurance.
to visualize the upper or lower
airways or to obtain
bronchoalveolar lavage specimens

39
CORYZA (COMMON COLD/  Narrowing of the airway and
NASOPHARYNGITIS) pharyngeal irritation causes dry
 This is the commonest infection of hacking cough.
childhood.  Sore throat.
 The commonest pathogens are  Health education to advise parents
viruses- rhinovirus (over 100 that colds are self-limiting (usually
different serotypes), coronavirus, lasting 3-4 days) and have no
RSV, parainfluenza and specific curative treatment may
adenovirus. reduce anxiety and save
unnecessary visits to doctors.
 Transmission is by droplet
infection.  Persistent symptoms for more than
10 days or fever should prompt the
 Predisposing factors: chilling,
sudden exposure to cold air and clinician to evaluate for bacterial
overcrowding, rhinitis could also superinfection (e.g. sinusitis, acute
be due to allergy. otitis media)

CLINICAL FEATURES DIAGNOSIS

 Classical features:  Clinical.
 Fever (low grade), irritability  Viral agent is rarely identified.
 Clear or mucopurulent nasal DIFFERENTIAL DIAGNOSIS
discharge (rhinorrhea)  Differential diagnosis:
o Mucopurulent discharge  Foreign body in nose (presents
does not always indicate with unilateral serosanguineous
secondary infection it can or purulent discharge from a
sometimes result from nostril)
shedding of epithelial and  Snuffles of congenital syphilis:
inflammatory cells this is severe rhinitis with
resulting from the viral bilateral serosanguineous
infection. discharge commonly
 Nasal blockage (which can excoriating the upper lip and
cause respiratory distress in leaving fine scars. Nasal
young infants because they are strictures may ulcerate leaving
obligate nose breathers) a flat nasal bridge
 Eustachian tube openings may
be blocked leading to serious MANAGEMENT
otitis media and congestion of  Relieve nasal congestion:
tympanic membrane.

40
  Nasal saline drops PRN (when  Sinusitis
necessary)  Bronchiolitis
 Nasal decongestants  Exacerbation of asthma
(ephedrine, xylometozoline)  Bronchopneumonia
may cause rebound congestion
and should not be used
routinely and only used in
refractory cases for limited
EPIGLOTTITIS
duration.
 This is acute inflammation and
 Antihistaminics are also not
edema of the epiglottis, arytenoids
recommended in children
and aryepiglottic folds.
below 6 months.
 The disorder is common in
 Fever and pain:
children 2-7 years of age, with
 Best treated with paracetamol
equal incidence in males and
(coupol).
females.
 Avoid giving cough syrups: if
cough is suppressed in infants  Infection with Hemophilus
and young children mucoid influenzae type B (HIB) was the
secretions may be retained in most common cause before HIB
the bronchi and this may immunization. This is now rare
predispose to spasmodic cough, because of the success of the HIB
wheezing, atelectasis and immunization program.
suppuration.  Group A beta-hemolytic
 Antibiotics are of no benefit as the streptococcus, Streptococcus
common cold is viral in origin. pneumoniae, and Staphylococcus
species may also cause epiglottitis.
 Secondary bacterial infection is
very uncommon but should be CLINICAL FEATURES
treated with antibiotics when  Abrupt onset of rapidly
suspected. progressive upper airway
 There is no evidence supporting obstruction without prodrome.
that large doses of vitamin C are  It sometimes starts with a minor
helpful. upper respiratory tract illness
COMPLICATIONS which progresses rapidly within
the course of a few hours.
 Complications:
 Otitis media  The following signs and symptoms
 Laryngitis may occur:

41
  High fever and toxic
appearance
 Muffled speech and quiet
stridor (softer than
laryngotracheobronchitis).
 Dysphagia with drooling.
 Sitting forward in tripod
position with neck
hyperextension.
 Use of accessory muscles of
respiration
 Marked suprasternal and
Figure 8: Thumb Sign of epiglottitis
subcostal retraction of the
chest. DIFFERENATIAL DIAGNOSIS
 Complete airway obstruction with  Croup
respiratory arrest may occur  Bacterial tracheitis
suddenly.  Retropharyngeal abscess
DIAGNOSIS
 Diagnosis is made by cautious
direct laryngoscopy where there is
visualization of a cherry red
swollen epiglottis when airway is
established.
 Investigations: Lab studies
demonstrate DIFFERENTIATING BETWEEN
 Leukocytosis with left shift. SUPRAGLOTTIC AND
 90% of patients have a positive SUBGLOTTIC DISORDERS
blood culture if the epiglottitis
is secondary to HIB. Feature Supraglottic Subglottic
 Epiglottis appears like a Stridor Quiet Loud
“thumbprint” on a lateral Cough None Barky
radiograph of the neck. Voice Muffled Hoarse
Dysphagia/ Present Absent
drooling
Fever High Low to
moderate
(croup)

42
 High LARYNGOTRACHEOBRON
(tracheitis) CHITIS (CROUP)
Toxicity Present Absent unless
 Croup is inflammation and edema
tracheitis is
of the subglottic larynx, trachea
present
Posture Neck Normal and bronchi.
extended,  There are 2 forms of croup:
tripod  Viral croup
positon  Spasmodic croup
VIRAL CROUP
 This is the most common cause of
stridor.
MANAGEMENT
 It typically occurs in children of
 Epiglottitis is a medical
age 3 years of age in the late fall
emergency.
and winter.
 Controlled nasotracheal intubation
 The male to female ratio is 2:1
should be performed by experience
personnel.  Parainfluenza viruses are the most
common cause.
 Before intubation, minimize
stimulation while offering  Other organisms: respiratory
humidified oxygen. syncytial virus (RSV), rhinovirus,
adenovirus, influenza A and B and
 Avoid causing distress or
Mycoplasma pneumoniae.
examining the throat with a tongue
depressor as this may cause  Bacterial etiology or bacterial
respiratory arrest. superinfection are unusual.
 Antibiotic therapy typically  Clinical features:
includes a second or third  Begins with upper respiratory
generation intravenous infection prodrome for 2-3 days
cephalosporin. If epiglottitis is followed by stridor and cough.
secondary to HIB, rifampin (gradual onset)
prophylaxis is indicated for  Symptoms:
unimmunized household contacts o Mild Inspiratory stridor but
younger than 4 years of age. as obstruction increases the
stridor becomes more
marked and the suprasternal
and sternal recession with

43
 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. Figure 9: Steeple sign/ Wine Bottle sign (Croup)

 Stridor and cough worsen at  Diagnosis should be suspected on

night and with agitation. the basis of clinical features.
 As obstruction worsens, breath  Management:
sounds may become inaudible  Supportive care involving cool
and stridor may apparently mist and fluids. Improvement is
decrease. This may also noted when patients are
unfortunately be misinterpreted exposed to cool night air.
as clinical improvement by an  Children with stridor at rest
unwary physician. benefit from systemic
 Anterior-posterior radiograph of corticosteroids such as
the neck demonstrates the “steeple intramuscular dexamethasone,
sign” (also known as wine bottle nebulized budesonide or oral
sign) of subglottic narrowing. corticosteroids with reduce
 It is tapering of the upper airway edema.
trachea on a frontal chest  Children with respiratory
radiograph reminiscent of a distress benefit from racemic
church steeple. epinephrine aerosols which
vasoconstrict subglottic tissues.
 Beta 2 agonist (e.g. albuterol)
are useful when wheezing is
appreciated on examination.
 Hospitalization is indicated for
children in respiratory distress.

44
SPASMODIC CROUP BACTERIAL TRACHEITIS
 This occurs year round in preshool  This is an uncommon but
age children. reemerging cause of stridor.
 It occurs in children between the  It is acute inflammation of the
age of 1 and 3 years. trachea.
 There is sometimes no preceding  It is caused by Staphylococcus
coryza. aureus (60%), Streptococcus, and
 It is likely secondary to a nontypeable Hemophilus influenza.
hypersensitivity reaction.  Clinical features:
 It characteristically present with  Abrupt onset
acute onset of stridor usually  Toxicity, high fever and
occurring at night. mucous and pus in the trachea
 The child wakes up suddenly with  Management: Appropriate
brassy cough and noisy breathing. antistaphylococcal antibiotics and
 Spasmodic croup typically recurs airway support are indicated.
and resolves without treatment.
INFECTIOUS DISEASES
DIFFERENTIAL DIAGNOSIS (LOWER RESPIRATORY
 The syndromes of croup should be
distinguished from each other and
TRACT)
also from the croup associated
with diphtheria in which a
membrane is seen on laryngoscopy BRONCHIOLITIS
or occasionally with measles.  It is a viral infection of upper and
 Rarely the croup may result from lower respiratory tract (medium
angioneurotic edema. and small airways).
 A retropharyngeal abscess may  It denotes inflammation of the
cause respiratory obstruction bronchioles.
 Aspiration of a foreign body is an  Bronchiolitis is the most common
important cause of obstruction. It lower respiratory tract infection in
may be rarely confused with the first 2 years of life.
wheezing in asthma.  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.

45
 Risk of infection is increased with  Bronchiolar spasm occurs in some
 Day care attendance, cases.
 Multiple siblings,  The bronchial lumen which is
 Exposure to tobacco smoke and already narrow in the infants is
 Lack of breastfeeding (High further reduced.
quantities of secretory IgA  During expiration, the bronchioles
antibodies to RSV are present are partially collapsed and egress
in the colostrum and breast of air from the lungs is severely
feeding reduces the likelihood restricted resulting in trapping of
of an infant being hospitalized air inside the alveoli causing
with acute bronchiolitis). emphysematous changes.
 More significant disease occurs in  When obstruction becomes
patients with chronic lung disease, complete the trapped air in the
congenital heart disease, history of lungs may be absorbed causing
prematurity, immunodeficiency atelectasis.
diseases and in infants younger  Due to diminished ventilation and
than 3 months. diffusion, hypoxemia is produced
 Etiology: in almost all of these infants.
 Respiratory syncytial virus is (ventilation perfusion mismatch)
the most common (80%).  Retention of carbon dioxide leads
 Less common causes include to respiratory acidosis.
parainfluenza, influenza,  The presence of eosinophils in the
adenovirus, human blood and respiratory secretions
metapneumovirus, rhinovirus, suggest that the virus infection
and Mycoplasma pneumoniae initiates the wheezing attack in a
(Rare). child who is already sensitized.
 Dual infection with RSV and
human metapneumovirus is CLINICAL FEATURES
associated with severe  Onset is gradual with upper
bronchiolitis. respiratory symptoms such as
rhinorrhea, nasal congestion, fever
PATHOGENESIS and cough occurring initially.
 The inflammation causes by viral  A few days following an upper
infection of bronchiolar mucosa respiratory tract infection
leads to edema, thickening, breathing becomes fast and
formation of mucus plugs and respiratory distress develops.
cellular debris.

46
 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. INVESTIGATIONS
 Apneic spells may be present and  CXR reveals:
these should be monitored  Hyperinflation with air trapping
especially in young infants and in  Patchy infiltrates
children with a history of apnea of  Focal Atelectasis
prematurity.  Diaphragm is pushed down
 Majority of infants have mild  Lung fields appear abnormally
symptoms and recover in 3-7 days. translucent.
 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.
 Hypoxemia may occur.

Figure 10: Chest X-ray. Bronchiolitis showing

hyperinflation (Image adapted from Essential Pediatrics
8th Ed)

47
  Symptoms of asthma can be
identical to bronchiolitis.
Suspect asthma if:
o Family history of asthma
o Prior episodes
o Responds to
bronchodilators
DIFFERENTIAL DIAGNOSIS
 Bacterial pneumonia
 Signs of obstruction in
pneumonia are less
pronounced, fever is high and
Figure 11: Bronchiolitis with air trapping and right apical adventitious sounds in lungs are
pneumothorax (arrow). Additional findings include
bilateral parahilar infiltrates and atelectasis (Image prominent.
adapted from Hutchinsons Pediatrics)  Foreign bodies in trachea
 Full blood count: Leukocyte count  Diagnosed by the history of
is normal or slightly elevated. aspiration of foreign body,
 Viral antigen/ antibody testing. localized wheeze and signs of
 PCR on nasopharyngeal aspirates. collapse or localized
obstructive emphysema.
DIAGNOSIS  Bronchial asthma
 Made on the basis of clinical  Bronchiolitis is often confused
features (Clinical diagnosis) and with bronchial asthma.
may be confirmed with viral  Bronchial asthma is unusual
antigen/ antibody testing or PCR below the age of 1 year, a
on nasopharyngeal aspirate. family history of asthma is
 Bronchiolitis is generally a self- usually present.
limiting illness.  Congestive heart failure
 Symptoms subside in 3-7 days.  Suggested if there is
 Death may occur in 1% of the cardiomegaly on X-ray,
severely ill patients due to tachycardia, large tender liver,
respiratory failure. raised JVP, edema and rales on
 The relationship between acute auscultation of the lungs.
bronchiolitis to bronchial asthma
later in life is observed in about
25%.

48
MANAGEMENT  Steroids are controversial and may
 Admit (low threshold for be most effective in patients with a
hospitalization for high-risk infant) prior history of wheezing.
 Hospitalization is indicated for  Nebulized racemic epinephrine
respiratory distress, hypoxemia, may be effective in reducing
apnea, dehydration or airway constriction.
underlying cardiopulmonary  Aerosolized ribavirin a nucleoside
disease. analog with in vitro activity
 Primarily supportive with nasal against RSV may be considered
bulb suctioning, hydration and for very ill infants. Evidence of
oxygen as needed. definitive benefit is lacking.
 Careful handwashing to prevent  Antibiotics have no role.
spread of infection is necessary.  RSV monoclonal antibody
 Sitting position at angle 30 to 40 (palivizumab) may be given
degrees with head and neck prophylactically by monthly
elevated. intramuscular injection during
 Moist oxygen is given RSV season to prevent severe
continuously even in the absence disease in infants with a history of
of cyanosis. (keep oxygen prematurity, chronic lung disease
saturation more than 92%). or cyanotic or hemodynamically
 Continuous positive airway significant congenital heart
pressure (CPAP) or assisted disease.
ventilation may be required to
COMPLICATIONS
control respiratory failure.
 Bacteremia
 Extracorporeal membrane
 Superadded bacterial pneumonia
oxygenation is effective, if
respiratory failure is not  Dehydration
controlled by mechanical  Respiratory failure
ventilation.  Pericarditis
 Fluids and electrolyte balance  Cellulitis
should be maintained.  Empyema
 Nebulized bronchodilators are  Meningitis
controversial and may only be  Suppurative arthritis
effective in up to 50%.
 Trial of nebulized albuterol

49
 o Haemophilus influenza type
PNEUMONIA
b
o Mycoplasma pneumoniae
PNEUMONIA o Group B beta-haemolytic
 Pneumonia involves infection and Streptococcus (in
inflammation of lung parenchyma. newborns)
 It is associated with poverty, o Staphylococcus aureus
multiple siblings, exposure to  Fungal
tobacco smoke and prematurity as o Pneumocystis jirovercii
well as urban residence. (PCP)
 Predisposing factors include:
 Congenital anomaly of the CLINICAL FEATURES
bronchi  Acute pneumonia usually presents
 Inhaled foreign body with a short history of fever, cough
 Immunosuppression and signs of respiratory distress
 Recurrent aspiration (e.g. with including tachypnea (most reliable
a trachea-esophageal fistula) sign), dyspnea and intercostal
 Cystic fibrosis recession.
 Causes may be classified based on:  Grunting, fever and feeding
 Etiological agent difficulties are common
 Age findings in infants.
 Productive cough, chest pain is
CLASSIFICATION BY seen in children.
ETIOLOGICAL AGENT  Pneumonia should be suspected
 These are divided into: in all children who present with
 Viral (most common cause of cough or difficulty breathing
pneumonia in all age groups) (shortness of breath)
o Respiratory syncytial virus  Signs include dullness to
o Influenza virus percussion, bronchial breathing
o Para-influenza and crackles reflecting the
o Adenovirus underlying consolidation.
o Coxsackie virus  Clinical signs are often not reliable
 Bacterial (less common but in infants and the diagnosis should
severe) always be confirmed by Chest X-
o Streptococcus pneumoniae ray (which may either indicate
(often causes lobar lobar pneumonia or a more
pneumonia) widespread bronchopneumonia)

50
 The most common causes of congestion and rhinorrhea. Fever,
pneumonia in children under 5 cough, tachypnea and dyspnea
years are: typically follow.
 Viruses  Physical exam may demonstrate
 Pneumococci tachypnea, wheezing, rales or
 Haemophilus influenza respiratory distress.
 Signs of serious illness (in a child  A child tachypnea if
under 5 who is either resting/  RR> 60b/m in children under 2
sleeping) are: months.
 Chest in-drawings  RR>50b/m in children from 2
 RR> 60b/m in children under 2 to 11 months
months  RR> 40b/m in children from 12
 Cyanosis (examine the lips, months to 5 years
buccal membranes and  Diagnosis is suggested by
fingernails) interstitial infiltrates,
 Nasal flaring hyperinflation, perihilar
 The child refuses to drink to infiltration, hilar adenopathy and
breastfeed. atelectasis on CXR and a WBC
 The child is abnormally sleepy count < 20, 000 cells/mm3 with
or difficult to wake lymphocyte predominance.
 Stridor (hoarse noise on  Management is supportive.
inspiration)
BACTERIAL PNEUMONIA
 Grunting (a short repetitive
noise produced by a partial  Symptoms have a more rapid onset
closure of the vocal cords) on and greater severity. Fever, cough
expiration and dyspnea typically occur
 Severe malnutrition without preceding upper
 The most common causes of respiratory symptoms.
pneumonia in children over 5 years  Physical exam may demonstrate
and adults are: rales, tachypnea, decreased breath
 Viruses sounds and evidence of respiratory
 Pneumococci distress.
 Mycoplasma pnemoniae  Diagnosis is suggested by a WBC
VIRAL PNEUMONIA count> 20, 000 cells/mm3 with a
neutrophil predominance and lobar
 Symptoms often begin with upper consolidation on CXR.
respiratory complaints e.g. nasal

51
 Management includes appropriate pharyngitis and malaise. The
antibiotics and supportive care. cough may last 3-4 weeks.
 1 to 3 months old:  Lung examination demonstrates
erythromycin or cefuroxime widespread rales. Examination
 3 months to 5 years old: findings are often worse than
ampicillin or cefuroxime expected by history.
 5 years: erythromycin or  Diagnosis:
cefuroxime  Positive cold agglutinins are
suggestive but nonspecific.
CHLAMYDIA TRACHOMATIS
 CXR findings vary but may
 Chlamydia trachomatis is a show bilateral diffuse infiltrates
common cause of afebrile  Definitive diagnosis is by
pneumonia at 1-3 months of age elevation of serum IgM titers
(pneumonitis syndrome). for Mycoplasma.
 Symptoms: staccato-type cough,  Management includes oral
dyspnea and absence of fever. A erythromycin or azithromycin.
history of conjunctivitis after birth
may be identified in 50% of DIAGNOSIS
patients.  Chest x-ray
 Physical examination may  Viral (hyperinflation, perihilar
demonstrate tachypnea and infiltration, hilar adenopathy
wheezing. and atelectasis)
 Diagnosis is suggested by  Bacterial (alveolar
eosinophilia and CXR with consolidation)
interstitial infiltrates.  Mycoplasma (interstitial
infiltrates)
 Definitive diagnosis is by positive
 Tuberculosis (hilar adenopathy)
culture or direct fluorescent
 Pneumocystis (Reticulonodular
antibody (DFA) staining of cells
infiltrates)
from conjunctiva or nasopharynx.
 Blood cultures (positive in 10-30%
MYCOPLASMA PNEUMONIA of bacterial cases)
 Mycoplasma pneumonia: common  Full blood count
cause of pneumonia in older COMPLICATIONS
children and adolescents.  Pleural effusion
 Symptoms: low-grade fever, chills,  Septicemia
nonproductive cough, headache,
 Bronchiectasis

52
 Empyema thoracis unimmunized or underimmunized
 Lung abscess (following children.
staphylococcal pneumonia)  Infection is highly contagious.
 Placental transfer of antibody does
PERTUSIS not protect young infants
 Pertussis (Latin= “Intense cough”) passively.
is an acute respiratory infection
also known as “Whooping cough”. PATHOGENESIS
 It is caused by the bacterium  B. pertussis produces a pertussis
Bordetella pertussis. toxin as well as few other
 It is a tiny, gram-negative biological active substances.
cocco-bacilli  These are responsible for various
 B. parapertussis causes illness that inflammatory changes with
appears clinically very similar to pertussis toxin playing a central
pertussis. role.
 Transmission is by direct  The mucosal lining of the
inhalation of microdroplets spread respiratory tract is inflamed with
by infected patients. necrosis and desquamation of
 Routine immunization beginning epithelial cells leading to
at 2 months of age has been obstruction, atelectasis and
effective in reducing the overall accumulation of secretion.
incidence of pertussis infection.  The resultant hypoxia can affect
 Universal vaccination is liver and brain also.
recommended at 2, 3 and 4 months
CLINICAL FEATURES
of age.
 Incubation period is typically 7-10
 Combined diphtheria-tetanus-
days.
pertussis vaccine are given
from the age of 6 weeks, 3  Pertussis is characterized by 3
doses of 0.5 ml at 4 weeks stages:
intervals; booster dose 1 year  Catarrhal stage (lasts 1-2
after the 3rd dose. weeks): characterized by upper
respiratory symptoms such as
 Infants younger than 6 months of
rhinorrhea, nasal congestion,
age are most at risk for severe
conjunctival redness and low-
disease.
grade fever
 Adolescents and adults whose
 Paroxysmal stage (lasts 3-4
immunity has waned are the major
weeks)
source for pertussis infection of

53
 o Characterized by fits of  WBC are elevated with a
forceful coughing lymphocytosis.
(“paroxysms”) that are the  Low ESR
hallmark of pertussis.  Diagnosis is confirmed by
o A whoop is an inspiratory identification of organisms on
gasp against a partially culture (gold standard) of
closed glottis heard at the nasopharyngeal secretions plated
very end of a coughing fit on Regan-Lowe or Bordet-Gengou
(the whoop is heard rarely media, or by positive direct
in young infants because fluorescent antibody tests of
they cannot generate nasopharyngeal secretions or PCR
enough pressures in their on nasopharyngeal swab.
respiratory passage).
o The coughing fits are COMPLICATIONS
exhausting and post-tussive  Respiratory system is the site for
vomiting is common. most complication.
o Young infants may have  Secondary bacterial infections
cyanosis, apnea and  Otitis media
choking during the  Emphysema
paroxysms of cough.  Air leaks- pneumothorax or
o Between the fits, children pneumomediastinum
appear well and are afebrile  Subcutaneous emphysema
 Convalescent phase (lasts  During paroxysmal stage serious
weeks to months): recovery CNS complications:
stage in which paroxysmal  Seizures
cough continues but becomes  Encephalopathy
less frequent and less severe  Complications associated with
over time. severe cough:
o The cough can persist for  Subconjunctival hemorrhage
some time and hence the (raised pressure in various
disease has also been called blood vessels)
‘Cough of 100 days’.  Retinal hemorrhage
 Epistaxis
DIAGNOSIS  Intracranial hemorrhage
 Diagnosis is suspected based on  Inguinal hernia (increased intra-
clinical features. abdominal pressure)
 Rectal prolapse

54
  Diaphragmatic rupture (rare)  Maintain nutrition: small, frequent,
 Due to protracted course: easily swallowable and caloric
 Vomiting dense foodstuffs should be given.
 Poor feeding Forced feeding should be avoided.
 Malnutrition Feeds are better given soon after a
 Flaring of underlying bout of coughing.
tuberculosis can also occur  All contacts irrespective of
symptoms, age and immunization
DIFFERENTIAL DIAGNOSIS
status should be given antibiotics
 Viral infection e.g. Adenovirus, for 2 weeks.
influenza, RSV
 For unimmunizaed or incompletely
 Mycoplasma infection immunized contacts, the schedule
 Foreign body aspiration should be completed.
 Endobronchial TB  Those whose have received a
MANAGEMENT vaccine dose >6 months back
 Hospitalization of young infants should receive a booster.
often occurs during the
paroxysmal phase because of
choking, apnea, or cyanosis. NON-INFECTIOUS
 Supportive care and oxygen (if DISEASES
needed) are important therapies. ASTHMA (REACTIVE
 A nebulized mist and/or
AIRWAY DISEASE)
salbutamol can be helpful in some
 This is a chronic inflammatory
patients.
disorder of the airways that causes
 Antibiotics are given to all patients
recurrent persistent episodes of
to prevent the spread of infection
wheezing, cough, dyspnea and
(azithromycin or erythromycin is
chest tightness.
used).
 Symptoms are typically associated
 Antibiotics do not alter the
with widespread, variable airflow
patient’s clinical course unless
obstruction that is at least partially
they are administered during
reversible either spontaneously or
the catarrhal phase or very early
with therapy.
in the paroxysmal phase.
 Inflammation causes airway hyper-
 All patients need to be isolated till
responsiveness to many stimuli.
they have received at least 5 days
 Asthma is the most common
of antibiotics.
chronic pediatric disease.

55
 50% of children have symptoms  Emotions
by 1 year and 90% by 5 years of  Allergens
age.  Gastroesophageal reflux
 30-50% have remission by  Exposure to pollutants
puberty.  Asthma may accompany other
acute or chronic lung diseases such
ETIOLOGY as cystic fibrosis.
 Predisposing factors: atopy, family
history of asthma and exposure to PATHOPHYSIOLOGY
tobacco smoke. Infection, diet and  Mechanisms include smooth
pollution increase susceptibility in muscle bronchoconstriction,
predisposed patients. airway mucosal edema, increased
 Trigger factors: secretions with mucous plugging,
 Respiratory infection e.g. eventual airway remodeling and
rhinovirus infection production of inflammatory
 Exercise mediators.
 Cold air

 Nasal flaring
 Retractions
CLINICAL FEATURES  Multiphonic (multiple pitch)
 Typical features during an wheezing with a prolonged
exacerbation: expiratory phase.
 Tachypnea o This is believed to be due
 Dyspnea to many airways of

56
 different dimensions  Once suspected, the pattern or
vibrating from abnormal phenotype should be further
narrowing. explored by asking:
o Asthma should be  How frequent are the
suspected in any child with symptoms?
wheezing on more than one  What triggers the symptoms?
occasion. Specifically, are sport and
o Although it may be clear to general activities affected by
most clinicians what the asthma?
‘wheezing’ is, patients and  How often is sleep disturbed by
parents do not always mean asthma?
the same thing. It is best to  How severe are the interval
describe the sound to a symptoms between
parent (e.g. ‘a whistling in exacerbations?
the chest when your child  How much school has been
breaths out’) and ask if that missed due to asthma?
fits with the child’s  Examination of the chest is usually
symptoms. normal between attacks. In long-
o The presence of wheeze is standing asthma there may be
confirmed on auscultation hyperinflation of the chest,
by a health professional to generalized polyphonic expiratory
distinguish it from wheeze and a prolonged expiratory
transmitted upper phase.
respiratory noises.  Onset of the disease in early
 Some patients have only chronic or childhood may result in Harrison
recurrent cough. sulci.
 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.

57
 Evidence of eczema should be exacerbations usually precipitated
sought as should examination of by viral respiratory infections) and
the nasal mucosa for allergic usually a therapeutic response to a
rhinitis. bronchodilator trial.
 Growth should be plotted but is  Note: “all that wheezes is not
normal unless the asthma is asthma”.
extremely severe.  Investigations: usually diagnosis
 The presence of a wet cough or from the history and examination
sputum production, finger and no investigations are required
clubbing or poor growth may to confirm the diagnosis.
suggest chronic conditions such as Sometimes specific investigations
cystic fibrosis or bronchiectasis. are required to confirm diagnosis,
 CXR shows: or explore the severity and
 Hyperinflation phenotype in more detail.
 Peribronchial thickening  Skin-prick test for common
 Patchy atelectasis allergens
 PFTs reveal increased lung  CXR
volumes and decreased expiratory  Spirometry: Peak expiratory
flow rates. flow rate (PEFR)

DIAGNOSIS
 Diagnosis is clinical (history of
recurrent wheeze, with

58
 59

intermittent, it is more
effectively controlled by
MANAGEMENT the addition of anti-
 Treatment depends on the severity inflammatory
of illness and trigger factors. medications.
 Self-management with a normal o Cromolyn sodium and
lifestyle is the primary goal. nedocromil sodium
 Control of asthma  Anti-inflammatory
 Assessment and monitoring of prophylaxis is induced
asthma symptoms. by inhibition of
 Control of trigger factors: activation and release of
avoidance of causal allergens, inflammatory mediators.
dust mites, molds, animal  Prevent degranulation of
dander, cockroaches, pollens, mast cells.
smoke, pollution and irritants.  These drugs have no
 Patient and family education effect on acute
 Pharmacologic therapy added symptoms but may help
in a stepwise fashion based on prevent exacerbations.
disease severity o Corticosteroids
o Sympathomimetics  Most effective anti-
 Beta 2 adrenergic inflammatory agents,
agonists may be given  Systemic steroids are
by inhaler or by given 5-10 days for
nebulization moderate to severe
 hort-acting exacerbations.
bronchodilators e.g.  Inhaled steroids are very
albuterol are first line effective in preventing
therapies for exacerbations.
exacerbation. They can o Anticholinergic agents (e.g.
also be used for atropine or ipratropium
prevention of exercised bromide= Atrovent)
induced symptoms.  Second-line
 Long-acting bronchodilators
preparations may be  Decrease airway vagal
used for chronic control tone and block reflex
 If asthma is persistent bronchoconstriction and
i.e. more severe than
 they may be useful in  CFTR is a cyclic AMP-
severe exacerbation dependent chloride channel
o Leukotriene modifiers found in the membrane of cells.
(motelukast, zafirlukast) are  The failure of the chloride
oral anti-inflammatory conductance by epithelial cells
agents for long-term control leads to dehydration of secretions
of mild, persistent asthma that are too viscid and difficult to
o Methylxanthines clear.
(theophylline) play a  Abnormal mucus produced in
controversial role in asthma airways helps create airway
management. They are obstruction (through reduction in
bronchodilators with the airway surface liquid layer and
possible anti-inflammatory consequent impaired ciliary
effects but have a narrow function), inflammation and
toxic-therapeutic ratio and infection (especially with
their use has therefore Staphylococcus aureus and
decreased. Pseudomonas aeruginosa).
 In the intestine, thick viscid
CYSTIC FIBROSIS
meconium is produced leading to
 Cystic fibrosis is a multisystem
meconium ileus in 10-20% of
autosomal recessive disorder that
infants.
results in altered content of
 The pancreatic ducts also become
exocrine gland secretions.
blocked by thick secretions leading
 The condition has equal incidence
to pancreatic enzyme deficiency
in males and females.
and malabsorption.
 Median age of survival is 31-32
 Abnormal function of the sweat
years.
glands results in excessive
PATHOPHYSIOLOGY concentrations of sodium and
 The genetic defect (Long arm of chloride in sweat.
Chromosome 7) produces an
CLINICAL FEATURES
abnormal ion-channel regulator
 Classic hallmarks of CF:
protein known as cystic fibrosis
 Chronic progressive pulmonary
transmembrane conductance
insufficiency
regulator (CFTR) that causes
 Pancreatic insufficiency
sodium and chloride transport
 High sweat electrolytes
dysfunction in epithelial cells.

60
 The features depend on age of sinusitis and nasal polyps.
diagnosis and treatment received. Recurrent pneumonia,
The common clinical presentation bronchiectasis, pulmonary
includes meconium ileus in fibrosis, cor pulmonale and
neonatal period, recurrent respiratory failure eventually
bronchiolitis in infancy and early occur.
childhood, recurrent lower  Pancreatic insufficiency occurs
respiratory tract infections, chronic in 90% of patients with
lung disease, bronchiectasis, malabsorption predisposing to
steatorrhea and with increasing age malnutrition and FTT.
pancreatitis and azoospermia. CHRONIC PROGRESSIVE
Pancreatic insufficiency is present PULMONARY INSUFFICIENCY
in >85% of CF patients.
 Clinical expression is variable.  Persistent colonization or infection
 Meconium ileus at birth is with CF pathogens e.g.
present in 20% Staphylococcus aureus,
o Inspissated meconium Haemophilus influenzae,
causes intestinal Pseudomonas aeruginosa or
obstruction with vomiting, Burkholderia cepacia.
abdominal distension and  The child has a persistent loose
failure to pass meconium in chronic cough, wheeze, purulent
the first few days of life. sputum production
o Initial treatment is with  On examination: hyperinflation of
Gastrografin enemas but the chest due to air trapping,
most cases require surgery. coarse inspiratory crepitations
 Recurrent or chronic respiratory and/or expiratory wheeze.
symptoms, steatorrhea and  Mucus plugging
failure to thrive (FTT) are  Nasal polyps and chronic sinusitis
typical presenting features.  Chest radiography:
 Pneumonia develops as lungs  Bronchiectasis
become colonized first with  Atelectasis
Staphylococcus aureus and  Pulmonary infiltrates
later with Pseudomonas  Hyperinflation
aeruginosa.
 Common pulmonary GASTROINTESTINAL
complications: hemoptysis, ABNORMALITIES
pneumothorax, asthma, chronic

61
 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

62
DIAGNOSIS MANAGEMENT
 Needs:  Treatment requires a team
 One or more phenotypic approach and includes:
features or  Antibiotics for pulmonary
 Positive family history or exacerbations
 Increased immunoreactive  Pulmonary toilet with chest
trypsinogen on newborn screen. percussion and antimucous
 Laboratory evidence of abnormal therapy
CFTR:  Bronchodilators for wheezing
 Sweat test: chloride  Good nutrition, pancreatic
>60mmol/L on at least 2 enzyme replacement and fat
occasions (normal 10- soluble vitamin (Vitamins A,
40mmol/L in normal children) D, E, and K)
o Sweating is stimulated by  Oxygen as needed for
pilocarpine iontroporesis hypoxemia
 Two CF mutations (confirmed  Anti-inflammatory and
diagnosis) immunosuppressive therapy
 Characteristic ion transport  Lung transplantation and
abnormality across the nasal ultimately future gene therapy
epithelium  Psychological support for
patients and families

63
64
65
66
CHAPTER 9: CARDIOVASCULAR CONDITIONS
CARDIOVASCULAR CONDITIONS
CONGENITAL HEART NORMAL DEVELOPMENT OF
DISEASES THE ATRIAL SEPTUM
 These are a group of diseases  To understand Atrial septal defects
involving malformations of the it is essential to understand normal
heart and major blood vessels. interatrial septum development:
 In early development of the
 They account for about 1% of all
heart, the heart is not a 4
births but incidence is higher in
chambered organ but rather a 1
premature babies.
chambered organ around 5th
 There are 3 main categories of
week of intrauterine
congenital heart diseases:
development.
 Left to right shunts
 The single chamber of the heart
 Right to left shunts
develops an atrial septum, a
 Obstructive congenital heart
ventricular septum and a
disease
structure between the atrium
ACYANOTIC and ventricle which is derived
from endocardial cushions
CONGENITAL HEART (Atrioventicular cushions)
DISEASE making it into 4 chambers.
 Left to right shunts:  Throughout prenatal life, blood
 Atrial septal defects is allowed to physiologically
 Ventricular septal defects shunt from the right atrium to
 Patent ductus arteriosus the left atrium.
 The atrial septum starts to
ATRIAL SEPTAL DEFECTS
develop from the top of the
 Initially ASD are left to right
primitive atrium, this crescent-
shunts because the pressure in left
shaped septum is known as the
atrium is greater than the pressure
septum primum. While the
in the right atrium but the shunt
septum primum is descending
can reverse after occurrence of
another septum develops from
pulmonary hypertension.
the apex towards the base (in
the ventricle, which fuses with
the endocardial cushion

67
 forming the interventricular lastly the foramen ovale &
septum). foramen secundum.
 In the initial part of  At birth the septum primum
development of the atrial fuses with the margins of the
septum as the septum primum foramen ovale forming the
descends, it does not fuse with fossa ovale.
the endocardial cushion and so  Later in life the septum primum
a foramen formed between the and septum secundum
free edge of the septum primum anatomically fuse to complete
and AV cushions called the the formation of the atrial
foramen primum. septum.
 The foramen helps shunt blood  Note also that in the initial
from the right to left atrium. stages of development, a part of
 This foramen is closed when the inferior vena cava fuses
the primum fuses with the AV with the interatrial septum to
cushion. When the foramen form the sinus venosus.
primum closes the center of the
septum primum develops
CLASSIFICATION OF ATRIAL
several openings which SEPTAL DEFECTS
coalesce with each other to  There are 3 main variants of ASD.
form the foramen secundum. They include:
 Once the foramen secundum  Ostium primum
has been formed another o This type of ASD is a
septum develops on the right defect in the lower portion
side. This is known as the of the atrial septum
septum secundum. (Septum primum).
 The crescent shaped septum o There is failure of the
secundum descends and covers septum primum to grow
the formen secundum (on the down in order to reach the
septum primum) however the endocardial cushions
septum secundum has a leaving the foramen
foremen known as the foramen primum open.
ovale. o It accounts for about 5% of
 In early development of the the ASDs.
heart blood is shunted through o It is associated with
the foramen primum and then abnormalities in
the foramen secundum and development of AV valves

68
 (mitral anterior leaflet o In sinus venosus, the right
defect- Mitral regurgitation pulmonary veins usually
or tricuspid septal leaflet). drain anomalougly into the
This is because there is right atrium or superior
failure of the fusions of the vena cava instead of into
septum primum with the the left atrium.
endocardial cushions.
o Ostium primum ASD is a
PATHOPHYSIOLOGY
common congenital heart  Blood flows across the septal
lesion in Down syndrome. defect from the left atrium to the
 Ostium secundum right atrium.
o This type of ASD is a  The direction of the blood flow is
defect in the middle portion determined by the compliance of
of the atrial septum. the right and left ventricles
o The septum primum (compliance is determined by
develops completely with systemic and pulmonary vascular
the foramen secundum but resistance).
the septum secundum does  Blood therefore flows from areas
not develop enough to of higher resistance to areas of
cover the foramen lower resistance.
secundum and due to this  Increased blood flow across the
under-development of the ASD leads to an increase in size of
septum secundun the the right atrium and right ventricle
foramen ovale becomes and to increased pulmonary blood
large and the foramen flow.
secundum is uncovered.
CLINICAL FEATURES
o Ostium secundum is the
 Symptoms are minimal, if any
most common type of ASD
except in patients with an ostium
(90%)
primum defect who develop mitral
o It is not associated with
regurgitation that results in CHF.
mitral or tricuspid defects.
 Physical examination:
 Sinus venosus
 Increased right ventricular
o This type of ASD is a
impulse as a result of right
defect high in the septum
ventricular overload
near the junction of the
 Systolic ejection murmur (from
right atrium and superior
excessive pulmonary blood
vena cava.

69
 flow) best heard at the mid and VENTRICULAR SEPTAL
upper left sternal borders. A DEFECTS
mid-diastolic filling rumble  Ventricular septal defects are
representing excessive blood classified by location as:
flow through the tricuspid valve  Inlet
may also be heard.  Trabecular (muscular)
 Fixed split second heart sound  Membranous
because of the excessive  Outlet (supracristal)
pulmonary blood flow, the
normal physiologic variation in PATHOPHYSIOLOGY
timing of the aortic and  After birth as pulmonary vascular
pulmonic valve closure with pressure decreases, blood flow
respiration is absent. across the VSD from the left
ventricle to the right ventricle
DIAGNOSIS owing to the lower resistance
 Usually diagnosed incidentally. within the pulmonary circulation
 Echocardiography is diagnostic. compared with the resistance
 ECG shows: within the systemic circulation.
 Right axis deviation  However, with time the pulmonary
 Right ventricular hypertrophy vessels hypertrophy in response to
 Right atrial enlargement this increased pulmonary flow.
 Chest x-ray shows:  This hypertrophy may lead to
 Right atrial and ventricular increased pulmonary vascular
enlargement resistance (pulmonary
 Increase pulmonary vascular hypertension).
markings
CLINICAL FEATURES
MANAGEMENT  Clinical features and course vary
 Treatment is closure by open heart greatly depending on the
surgery to prevent right sided hear magnitude of the left to right shunt
failure, pulmonary hypertension, across the VSD.
atrial dysrhythmias and  The amount of blood flow directed
paradoxical embolism. from one side of the heart to the
 Some centers close ASDs using other side is determined by both
interventional catheterization the size of the VSD and the degree
procedures. of pulmonary vascular resistance.

70
 For example, the larger the VSD flow) then a diastolic
and the lower the pulmonary murmur of mitral
vascular resistance (PVR) the turbulence (mitral filling
greater the blood flow across the rumble representing the
VSD into the pulmonary vessels. excess blood from the lungs
The greater the pulmonary blood not passing through the
flow, the more symptomatic the mitral valve) may be heard
patient. at the apex.
 Typical clinical presentations  Large VSDs
include: o Often cause signs and
 Small VSDs symptoms of CHF.
o Little to no shunt across o Have less turbulence across
VSD the VSD, so the systolic
o May close spontaneously murmur is shorter and
o On examination, a thrill at lower in pitch.
the lower left sternal border o A mitral filling rumble may
and a grade 4 high-pitched be heard at the apex.
holosystolic murmur may  Pulmonary vascular resistance may
be present, indicative of a eventually become elevated in
very restrictive defect with moderate or larger VSDs in
a high flow velocity across response to chronically high
the VSD. pulmonary flow.
o As the size of the VSD  When PVR becomes elevated the
decreases the intensity of right ventricular impulse is
the murmur increases. noticeably increased and the
 Moderate VSDs second heart sound may be single
o May have a larger shunt and loud. The mitral filling rumble
across the VSD that may disappears because of diminished
result in signs and pulmonary blood flow as a result
symptoms of CHF. of decreased left to right shunting.
o A holosystolic murmur is  If PVR remains elevated,
usually present and its pulmonary hypertension becomes
intensity depends on the irreversible even if the VSD is
size of the shunt. surgically closed. In the extreme
o If excessive blood flows situation in which PVR exceeds
across the VSD (2:1 systemic vascular resistance
pulmonary to systemic shunting changes from left-to-right

71
 to right-to-left a condition termed PATENT DUCTUS
Eisenmenger syndrome. ARTERIOSUS
DIAGNOSIS  In the fetus, the ductus arteriosus
 Confirmed by ECHO connects the pulmonary artery to
 ECG shows: the aorta. It serves as a by-pass
 Normal or mild left ventricular because the lungs are not function
hypertrophy with small VSDs hence pulmonary vascular
 Left ventricular hypertrophy resistance is high.
and right ventricular  After birth as the PaO2 rises the
hypertrophy if pulmonary ductus normally fibroses. If it
hypertension is present in remains open it is termed a PDA.
moderate VSDs  Incidence of PDA is especially
 Chest X-ray findings high in preterm infants.
 Normal in small VSDs  Note: intrauterine the PDA is kept
 Cardiomegaly and increased open by production of PGE2
pulmonary vascular markings PATHOPHYSIOLOGY
in moderate or large VSDs
 In a PDA, blood flow through the
MANAGEMENT ductus from the aorta to the
 Medical: pulmonary artery (left to right
 Medical management of CHF shunt) leading to increased
in a symptomatic child. Large pulmonary blood flow.
shunts are also associated with CLINICAL FEATURES
high incidence of pulmonary
 Signs and symptoms depend on
infections from excessive blood size of PDA and the relationship
flow. between systemic vascular
 Surgical closure, indicated in: resistance and pulmonary vascular
o Heart failure refractory to resistance.
medical interventions
 Small PDAs: usually produce no
o Large VSDs with
symptoms
pulmonary hypertension are
 Moderate or Large PDAs generally
usually surgically closed at
result in signs and symptoms of
3-6 months of age.
CHF due to increased pulmonary
o Small to moderate VSDs
blood flow
are usually closed between
 Physical findings:
2 to 6 years of age.

72
  Classical murmur which is vasoconstriction as a result of cold
described as a continuous temperature.
“machinery-like” murmur  Central cyanosis especially in the
heard best at the upper left tongue and inner mucus
sternal border. membranes may be attributed to
 If the left to right shunt is large, both cardiac and non-cardiac
there may also be a diastolic causes.
rumble of blood flow across the  Non-cardiac causes: pulmonary
mitral valve at the apex, a disease, sepsis, hypoglycemia,
widened pulse pressure polycythemia and
(>30mmHg) and brisk pulses. neuromuscular disease that
 Risk of pulmonary hypertension impair chest wall movement
caused by excessive pulmonary  Cardiac: Remember them by
blood flow is significant in the 5Ts:
children older than several years of o Tetralogy of Fallot (TOF)
age. o Transposition of great
arteries
MANAGEMENT o Tricuspid atresia
 Indomethacin is used in premature o Truncus arteriosus
infants to close a PDA medically. o Total anomalous
 Indomethacin an NSAID pulmonary venous
inhibits production of PGE2 connection
which is required to keep the
PDA.
 PDAs may also be closed TETRALOGY OF FALLOT
surgically by coil embolzation,  This is the most common cyanotic
video-assisted thoracoscopic congenital cardiac disease in
surgery, and ligation in a children.
thoracotomy.
CLINICAL FEATURES
CONGENITAL  As the name suggests TOF has 4
CYANOTIC HEART anatomical features:
 Subpulmonary stenosis (Right
DISEASES ventricular outflow tract
 Cyanosis may be peripheral, obstruction)
central or both.  Right ventricular hypertrophy
 Peripheral cyanosis is usually
caused by vasomotor instability or

73
  Ove-riding of the aorta with severe hypoxia, and breathlessness
respect to the ventricular and pallor because of tissue
septum (aorta overlies a portion acidosis.
of the ventricular septum this is  To compensate, a child with TOF
due to a defect of the learns to squat. This position
development of the (knee-chest position in an infant)
aorticopulmonary septum) increases venous return to the heart
 Large ventricular septa defect and increases systemic vascular
(VSD) resistance thereby decreasing the
 The severity of right ventricular right to left shunt.
outflow obstruction determines the  On auscultation there is a very
amount of right to left shunting short murmur during a tet spell.
across the VSD.  Signs:
 Because of the right ventricular  Clubbing of the fingers and
outflow obstruction blood flows toes in older children
right to left across the VSD and  A loud harsh ejection systolic
into the overriding aorta, as a murmur at the left sternal edge
result there is diminished blood from day 1 of life with
flow and cyanosis results. increasing right ventricular
 Most are diagnosed: outflow tract obstruction, which
 Antenatally or is predominantly muscular and
 Following the identification of below the pulmonary valve, the
a murmur in first 2 months of murmur will shorten and
life. Cyanosis at this stage may cyanosis will increase.
not be obvious although a few  Note:
present with severe cyanosis in  Actions like increase systemic
the first few days of life. vascular resistance (exercise,
 Hypercyanotic spells (tet spell), vasodilation, volume depletion)
may lead to myocardial infarction, or increase resistance through
cerebrovascular accidents and even the right ventricular outflow
death if left untreated. tract (e.g. crying, tachycardia)
 Symptoms depend on the severity increase right to left shunting
of right ventricular obstruction resulting in cyanosis.
 They are characterized by a rapid  Actions that increase systemic
increase in cyanosis, usually vascular resistance or reduce
associated with irritability or resistance through the right
inconsolable crying because of ventricular outflow obstruction

74
 (e.g. volume infusion, systemic  Right ventricular hypertrophy
hypertension, Valsalva when older
maneuver, bradycardia) reduce  Echocardiography
the right to left shunt through  This will demonstrate the
the VSD and increase systemic cardinal features, but cardiac
arterial saturation. catheterization may be required
 TOF is condition that is PDA to show the detailed anatomy of
dependent. the coronary arteries.
INVESTIGATIONS MANAGEMENT
 Chest X-ray shows:  Initial management is medical with
 Mild to moderate cardiomegaly definitive surgery at around 6
 Uplifted apex secondary to months of age. It involves closing
right ventricular enlargement the VSD and relieving the right
and concavity in the region of ventricular outflow tract
pulmonary artery segment obstruction sometimes with an
giving a boot shaped heart. artificial patch, which extends
 A right sided aortic arch occurs across the pulmonary valve.
in 25% of patients with TOF.  Infants who are very cyanosed in
 Decreased pulmonary vascular the neonatal period require a shunt
markings reflecting reduced to increase pulmonary blood flow.
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 ballon dilatation of
the right ventricular outflow tract.
 Hypercyanotic spells are usually
self-limiting and followed by a
period of sleep.
 Sedation and pain relief
(morphine is excellent)
 Placement in knee-chest
position (mimics squatting
position)
 ECG
 Normal at birth

75
 Intravenous propranolol (or an  Intravenous fluid
alpha adrenoceptor agonist) administration
which probably works both as a  Intravenous Sodium
peripheral vasoconstrictor and bicarbonate to correct acidosis
by relieving the subpulmonary  Muscle paralysis and artificial
muscle obstruction that is the ventilation in order to reduce
cause of reduced pulmonary metabolic oxygen demand.
blood flow as well as reduce  Correction of significant
heart rate, contractility and anemia with transfusion
ventricular outflow obstruction.

76
 more common in girls whereas
aortic valve involvement is more
RHEUMATIC FEVER often seen in boys.
 Rheumatic fever is a delayed non-  The major risk factor is pharyngitis
suppurative autoimmune caused by certain strains of group
complication of upper respiratory A beta-hemolytic streptococcus.
infection with Lancefield group A Especially the M serotypes.
beta-hemolytic streptococcus  The streptococcal strains that
(Streptococcus pyogenes) cause streptococcal skin infection
characterized by inflammation of (i.e. impetigo) do not cause
the connective tissues. rheumatic fever.
 There also seems to be a familial  The cause of rheumatic fever is
tendency to develop the disease unknown but the disease appears
and the expression of certain HLA to be autoimmune in nature.
groups on a host’s B-cells may
make them more susceptible to PATHOGENESIS
rheumatic fever.  Antibodies produced in response
 Rheumatic fever predominantly to a streptococcal throat infection
affects the heart, blood vessels, in a susceptible host react to
joints, CNS and skin. streptococcal M protein as well as
 It is most common in children 5-15 cross react with host connective
years of age, reflecting the age tissues of the body.
group most susceptible to  This phenomenon is attributed to
streptococcal throat infections. molecular mimicry.
 It has no gender predilection but  Rheumatic fever seems to be the
mitral valve disease and chorea is result of the host’s unusual

77
 response at both the cellular and
humoral level to Streptococcus.

associated with rheumatic fever

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

78
 both group A beta hemolytic  Occasionally the child is unable to
streptococcus and basal ganglia. stand or even to sit up (chorea
 Children appear restless. paralytica)
 The face, hands and arms are  Speech is also affected and can be
mainly affected and the jerky or indistinct.
movements appear continuous,  Patients are unable to sustain
quick and random. The chorea protrusion of the tongue
may begin as clumsiness of the (chameleon tongue).
hands.  On gripping the examiner’s
 Incoordination may be marked fingers, patients are unable to
or only obvious when the child maintain the grip (milkmaid’s
is asked to pick a coin off the grip)
floor.  Gait and cognition are not affect
 Involuntary purposeless, non- but emotional lability is common.
repetitive movements of the limbs,  Infrequently the child becomes
face and trunk e.g. grimacing, confused or even maniacal (chorea
wriggling and writhing. The insaniens).
movements can be brought under  The following clinical maneuvers
voluntary control temporarily. are helpful in arriving at the
 The movements are aggravated by diagnosis:
excitement/stress, attention and  When the hand is outstretched
they disappear during sleep. above the head, forearms tend
 The first indication may be that the to pronate (pronator sign)
child begins to drop things or her  When hands are stretched
handwriting suddenly deteriorates forwards, wrist flexes and
or she gets trouble with her elders fingers hyperextend
for making faces. Sometimes the  The child relaxes hand grip on
movements are confined to one and off as if he is milking a
side of the body (hemichorea) cow (Milkmaid grip)
 Hypotonia may result in muscular  The child cannot maintain
weakness. It also causes the tongue protrusion (darting
characteristic posture of the tongue/ chameleon tongue)
outstretched hand in which the  During speech an audible click
wrist is slightly flexed, whereas is heard.
there is hyperextension of the
metacarpophalangeal joints
(choreic hand).

79
  The knee reflex may show a maintained at minimum doses
sustained contraction resulting required for symptom suppression.
in a hung up reflex.
POLYMIGRATORY ARTHRITIS
 There is no single confirmatory
test for syndenham chorea.  This is classically migratory,
 The diagnosis rest on asymmetric and exquisitely
presumptive evidence of painful.
rheumatic fever and exclusion  It occurs in 70% of patients and
of other likely causes of chorea most commonly involves the
 Elevated antistreptolysin O elbows, knees, ankles and wrists.
(ASO) or anti-DNase B (ADB)  Uncommonly smaller joints may
may indicate a recent also be involved.
streptococcal infection.  The affected joints show redness,
 Neuroimaging such as head warmth, swelling, pain and
MRI may show increased limitation of movement.
signal intensity in the caudate  The pain and swelling appear
and putamen on T2-weighted rather quickly last 3 to 7 days and
sequence. subside spontaneously to appear in
 Single-photon emission some other joint.
computed tomography  It does not result in chronic
(SPECT) may demonstrate disease.
increased perfusion to the  Arthritis tends to be commoner in
thalamus and striatum. older patients.
 Symptoms may last from several
ERYTHEMA MARGINATUM
months to 2 years. Generally, all
 This is a non-pruritic/itching rash
patients recover. Relapses or
that starts as pink to red macules
recurrences can occur.
with a pale center which may
 Differential diagnosis:
coalesce and spread centripetally
 Encephalitis
with central clearing over the trunk
 Kernicterus
and proximal limbs.
 Systemic lupus erythematosus
 It is believed that the inability to
 Huntington’s disease
recognize erythema marginatum is
 Wilson’s disease
not because it does not occur but
 Management: treatment using
because of the dark complexion of
haloperidol, valproic acid or
the skin.
phenobarbital. Drugs are

80
SUBCUTANEOUS NODULES severe manifestations include
 Although rarely seen are cardiac dilatation and heart failure.
associated with severe cardiac  Pericarditis and pericardial
involvement. effusions are less common.
 These small, mobile and painless  Pericarditis results in precordial
nodules occur on the bony pain that may be quite severe.
prominences (shins, elbows,  On auscultation a friction rub is
occiput & spine) of the extensor present.
surfaces of the extremities.  ECG shows ST and T changes
 They are non-tender and last from consistent with pericarditis.
a few days to weeks but have been  Rheumatic pericarditis is
known to last for almost a year. associated with only small
effusions and generally does
CARDIAC INVOLVEMENT not result either in tamponade
 This is found in 50% of patients. or constrictive pericarditis.
 It is the hallmark and most  A patient of rheumatic
important complication of pericarditis always has
rheumatic fever. additional mitral or mitral and
 Rheumatic carditis is designated as aortic regurgitation murmurs.
a pancarditis involving the  Carditis is an early manifestation
pericardium, myocardium and of rheumatic fever. In 60-70% it is
endocardium clinically obvious whereas in the
 Endocarditis is the most common remaining the diagnosis is based
cardiac findings and typically on echocardiographic findings
causes insufficiency of the left- labeled as subclinical carditis.
side valves (mitral and aortic). It  Other features of carditis are:
rarely affects the pulmonic or  Cardiac enlargement
tricuspid valves.  Soft first sound
 Endocarditis is represented by a  Protodiastolic (S3) gallop
pansystolic murmur of mitral  Congestive cardiac failure
regurgitation with or without an  Cary Coombs’ murmur: a soft
associated aortic regurgitation delayed diastolic mitral
murmur. murmur heard transiently
 Myocarditis is usually manifested during the course of acute
by tachycardia out of proportion to rheumatic fever possibly as a
the extent of the fever. Other result of flow across the

81
 inflamed and thickened mitral o Elevated C reactive protein
valve. o Elevated WBC
 Serologic markers
DIAGNOSIS o Antistreptolysin-O titers are
 The JONES criteria for rheumatic abnormally elevated in 70-
fever is used in diagnosis of 80% of patients with
rheumatic fever. rheumatic fever.
 Diagnosis requires evidence of o Anti-DNase antibodies
recent streptococcal infection and o Anti-hyaluronidase
either 2 major criteria or 1 major antibodies
plus 2 minor criteria.  Echocardiography typically shows
 Lab findings: evidence of carditis, such as
 Nonspecific inflammatory decreased ventricular function,
markers: valvular insufficiency or
o Elevated ESR pericardial effusion.

82
 83

MANAGEMENT development of migratory

 Eradication of GABHS infection arthritis. Therefore their use is
 Benzathine penicillin recommended only after the
intramuscular infection (one diagnosis of rheumatic fever is
dose) certain.
 Penicillin orally for 10 days  Corticosteroids are often used
 Control of inflammation in patients with severe cardiac
 NSAIDs are useful for control involvement such as CHF and
of joint pain and swelling, but severe valvuar dysfunction.
if given before definitive  Supportive therapy:
diagnosis, they may obscure the  CHF: diuretics, dietary salt
diagnosis by halting the restriction, digoxin and bed rest
  Syndeham’s chorea, if severe immediately or many years after
may be treated with the event.
haloperidol, phenobarbital or  Valvular insufficiency or stenosis
valproic acid. is usually delayed (usually more
 Long term management than 3 years after rheumatic fever)
 Continuous antimicrobial and if severe may require valve
prophylaxis to prevent recurrent replacement or valvuloplasty.
episodes of rheumatic fever.  The severity of eventual rheumatic
Monthly injections of valvular disease relates to the
benzathine penicillin is the number of childhood episodes of
most effective prophylaxis. rheumatic fever.
Alternatively, the penicillin can
be given orally every day but
compliance may be a problem.
HEMATOLOGY
Oral erythromycin can be
substituted in those sensitive to
penicillin.
 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,

84
CHAPTER 10: HEMATOLOGY
HEMATOLOGY
SICKLE CELL DISEASE molecules that aggregate into
larger polymers.
 This is an autosomal recessive
disease that results from the  Sickle red blood cells are less
substitution of glutamic acid deformable and obstruct the
(hydrophilic) for valine microcirculation, resulting in
(hydrophobic) at position 6 of the tissue hypoxia, which further
beta-globin chain. promotes sickling.
 The disorder results from a point  These red blood cells are rapidly
mutation (GAG changing to GTG) hemolyzed and have a life span of
on chromosome 11p15.5 only 10-20 days.
(chromosome 11 position short  The usual onset of the disease is 5-
arm on position 15.5). 6 months of age as HbF (which has
 Patents who are homozygous for 2 alpha chains and 2 gamma
chains) concentration reduces.
HbS gene have sickle cell disease.
 Patients who are heterozygous for  Depending on the type of
HbS gene have sickle trait. hemoglobin chain combinations, 3
 These individuals have both clinical syndromes occur:
HbA (50-60%), HbS (35-45%)  Homozygous HbSS have the
and a small percentage of HbF. most severe disease.
 Combined heterozygosity
 Patients are usually
asymptomatic without anemia (HbSC) for HbS and C who
unless exposed to severe suffer intermediate symptoms.
hypoxemia.  Heterozygous HbAS (sickle
 Some patients have an inability cell trait) have no symptoms.
to concentrate urine PATHOGENESIS
(isosthenuria) or hematuria  Deoxygenated HbS molecules are
(5%) during adolescence. insoluble and polymerize. This
 Carriers are protected against increases viscosity.
Plasmodium falciparum.  Polymer formation at
 Deoxygenation of the heme moiety concentrations exceeding
of sickle hemoglobin leads to 30g/dl.
hydrophobic interactions between
adjacent sickle hemoglobin (HbS)

85
  Polymers form a gel-like  Adhesion proteins on activated
substance containing Hb endothelial cells (VCAM-1) may
crystals called tachoids. play a role particularly in vaso-
 Flexibility of the cells is decreased occlusion when rigid cells are
and they become rigid and take up trapped, facilitating
their characteristic sickle polymerization.
appearance.  HbS releases its oxygen to the
 This process is initially reversible tissues more easily than does
but with repeated sickling the cells normal Hb and patients therefore
eventually lose their membrane feel well despite being anemic
flexibility and become irreversibly (except of course during crises or
sickled. complications).
 This is due to dehydration,
partly caused by potassium
CLINICAL FEATURES
leaving the red cells via  Clinical features are often termed
calcium activated potassium ‘crises’ because they occur
channels called the Gados suddenly.
channel.  They include:
 These irreversibly sickled cells are  Sequestration crisis
dehydrated and dense and will not  Hyper-hemolytic crisis
return to normal when oxygenated.  Aplastic crisis
 Sickling produces:  Vaso-occlusive crisis
 Shortened red cell survival  Mixed
 Impaired passage of cells  Although infection, dehydration,
through the microcirculation, acidosis, cold temperature,
leading to obstruction of exercise, extreme emotions (all of
small vessels and tissue which favor sickling) can act as
infarction triggers, in most cases no
 Precipitating factors for sickling: predisposing cause is identifiable.
 Hypoxia VASO-OCCLUSIVE CRISIS
 Dehydration  This is the most common crisis.
 Cold  This occurs when the
 Acidosis microcirculation is obstructed by
 Infection and fever sickled red blood cells resulting in
 Living at high altitude ischemic injury.
 A crisis may occur without the
presence of these factors.

86
 The major complaint is pain inability to concentrate urine
usually affecting bones such as (isosthenuria).
femur, tibia and lower vertebrae.  Other presentations:
 Typically presents as deep,  Acute chest syndrome
gnawing or throbbing pain  Retinal hemorrhages
lasting 3-7 days.  Priapism: sustained painful,
 The pain is recurrent purposeless erection. Always
 Triggers of vaso-occlusive crisis consider SCD in any patient
include: presenting with priapism.
 Hypoxemia: may be due to  Avascular necrosis of the
acute chest syndrome or femoral head
respiratory complications  Cerebrovascular accidents
 Dehydration: acidosis results (Stroke): Dysathria,
in a shift of the oxygen hemiplegia, may be
dissociation curve asymptomatic.
 Changes in body temperature
ACUTE CHEST SYNDROME
 Vaso-occlusion may present as:
 Acute Dactylitis: painful  This is a type of vaso-occlusive
swelling of digits of the hands crisis that affects the lungs and
and feet presents with chest pain, cough,
o DDx: osteomyelitis tachypnea, dyspnea, hypoxemia,
 Hand and foot syndrome: fever or a new pulmonary
painful and swollen hands infiltrate.
and/or feet)  Causes include infection (e.g.
 Acute abdomen: abdominal viral, Mycoplasma pneumoniae,
pain and distention often Chlamydia pneumoniae,
caused by sickling within Streptococcus pneumoniae),
mesenteric artery. sickling, atelectasis, fat embolism,
o DDx: cholecystitis, painful bone crises involving the
appendicitis, splenic ribs and pulmonary edema from
sequestration fluid overload.
 The spleen may undergo auto-  Management:
infarction and is often not palpable  Admission
beyond 6 years of age (auto-  Hydration: IV fluids
splenectomy).  Pain management: analgesics-
 Involvement of the kidney results NSAIDs (diclofenac/ naproxen)
in papillary necrosis leading to

87
  Oxygen support- ventilation organisms (e.g. Haemophilus
(CPAP may be necessary) influenza, Streptococcus
 Antibiotics (also should cover pneumoniae, Neisseria
for mycoplasma and chlamydia, meningitidis)- thus vaccine should
usually cefuroxime and be given
azithromycin)  They are also at risk of other
 Incentive spirometry common infectious organisms such
 Bronchodilators as Salmonella, Mycoplasma
 Steroids pneumoniae, staphylococcus
 Early use of partial exchange aureus and Escherichia coli).
transfusion in a patient who
does not improve rapidly. APLASTIC CRISIS
 This most commonly occurs
SEQUESTRATION CRISIS following infection with
 This is due to sickled cells that erythrovirus B19 (previously
block splenic outflow, leading to called Parvovirus B19) which
the pooling of peripheral blood in invades proliferating erythroid
the engorged spleen resulting in progenitors.
splenic sequestration.  There is a rapid fall in hemoglobin
 Less common also occurs in the with no reticulocytes in the
liver. peripheral blood because of failure
 This occurs in patients <6 years of of erythropoiesis in the marrow.
age.  Presents with pallor, fatigue,
 Presents with abdominal tachycardia.
distension, abdominal pain,  Children with aplastic crisis may
shortness of breath, tachycardia, present with congestive heart
pallor, fatigue, and shock. failure due to severe anemia.
 Mortality can be high.  Usually only supportive care and
 Labs reveal reticulocytosis. occasionally packed red blood
 There will be severe hemolysis, cells transfusions are required.
rapid splenomegaly and a high
HYPERHEMOLYTIC CRISIS
reticulocyte count.
 Rapid hemolysis.
 Splenectomy is recommended by
some practitioners because  Often occurs in patients with other
recurrence occurs in up to 50%. hemolytic diseases (e.g. G6PD
deficiency)
 Children have increased
susceptibility to encapsulated

88
 Presents with pallor, fatigue, o Reticulocyte count: 5-15%
tachycardia and jaundice. (this may be seen as a
 Remember jaundice also results raised WBC count)
from turn-over of RBCs with o WBC count: 12 000-20 000
life span of 10-20 days. cell/mm3
 Usually only supportive care and o Platelet count: increased,
occasionally packed red blood often >500, 000
cells transfusions are required. platelet/micro litre
 The hyperhemolytic paradigm o Increased RDW. (Normal
proposes that hemolysis in sickle 11-15%)
cell disease leads to increased cell-  Peripheral smear
free plasma Hb which consumes o Shows: Sickle shaped cells,
Nitric oxide (NO) leading to a Target cells and Howell-
state of deficiency, endothelial Jolley bodies
dysfunction and a high prevalence
of pulmonary hypertension.
DIAGNOSIS
 Requires:
 Full history: recurrence of
symptoms (usually pain- joints,
back, abdominal)
 Full examination
 Investigations
INVESTIGATIONS
Figure 12: Sickle Shaped RBCs with normal shaped RBCs
 Hb electrophoresis (diagnostic
gold standard): can differentiate o Howell jolley bodies
between homozygous or indicate that the patient is
heterozygous. (this needs to be functionally asplenic.
checked prior to blood transfusion) o Howell jolley bodies are
 Blood investigations: basophilic nuclear remnant
 FBC: findings include (clusters of DNA) in
o Hb: 6-9g/dl circulating erythrocytes,
o Hematocrit: 18-27% during maturation in the
bone marrow, late
erythroblasts normally

89
 expel their nuclei, but in  Sickling test: if the diagnosis
some cases a small portion has not been made this is done
of DNA remains. to establish the presence of
sickle hemoglobin.
 Blood culture
 Arterial blood gases
 Imaging:
 Chest X-ray
 X-ray of skull
o Shows hair on end
appearance.

Figure 13: Howell-Jolly Bodies

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 14: Target cells

 Liver function tests

 Urea and electrolytes
 Hemoglobin solubility test

90
 splenomegaly and
hepatomegaly
 ECHO for pulmonary
hypertension
 Spirometry
COMPLICATIONS
CENTRAL NERVOUS SYSTEM
 Transient ischemic attacks
 Seizures
 Diminished cognition
 Cerebral infarction: most common
finding is obstruction of a distal
intracranial internal carotid artery
o This is caused by marrow or a proximal middle cerebral
hyperplasia due to chronic artery.
hemolysis so the vertical  Cerebral hemorrhage
trabeculae found between
 Coma
the inner and outer tables of
 Stroke
the diploe bones becomes
 Eyes:
accentuated thus having the
 Ptosis
hair on end appearance.
 Retinal hemorrhages
o This is also seen on CT
 Proliferative retinopathy, retinal
scan and can be also
detachment.
illustrated in thalassemia
and other forms of RESPIRATORY
hemolytic anemias.  Pulmonary emboli
 MRI: for early bone marrow  Fat emboli
changes  Respiratory distress
 CT  Respiratory failure
 Technetium 99 scan to detect
 Pulmonary hypertension: NO
early osteonecrosis
deficiency by increased free
 Transcranial Doppler U/S: to
plasma Hb that binds NO and
detect risk of stroke in children
causes a deficiency.
 Abdominal U/S: cholecystitis,
cholelithiasis, or ectopic CARDIOVASCULAR
pregnancy and to measure  Cardiomegaly

91
 Arrhythmias  There is often delayed sexual
 Dilatation of both ventricles and maturation which may require
left atrium hormone therapy.
 Iron overload cardiomyopathy  Bones:
 Myocardial infarctions  Avascular necrosis of head of
 Congestive heart failure femur
 Cor-pulmonale- from pulmonary  Compression of vertebrae
hypertension  Shortening of bones of hands
 Hemochromatosis and feet.
 Osteomyelitis due mostly to
GASTROINTESTINAL Staphylococcus aureus and
 Cholelithiasis- pigment stones Salmonella.
occur as a result of chronic
hemolysis GYNAE
 Chronic hepatomegaly  Spontaneous abortion: impaired
placental blood flow
 Liver dysfunction
 Intrauterine growth retardation
 Acute Abdomen-Mesenteric artery
blockage  Pre-eclampsia
 Autosplenectomy  Fetal death

GENITOURINARY MANAGEMENT AND

 Chronic tubulointerstitial nephritis PROGNOSIS
 Isosthenurea: kidneys lose  Avoid and treat precipitating
concentration capacity. factors quickly.
 Priapism  Rehydration
 Impotence  Pain control: opioids (morphine),
NSAIDs
DERMATOLOGY  Oxygen and antibiotics are only
 Leg ulcers over the medial or given if specifically indicated:
lateral malleoli.  Antibiotics: cefuroxime,
 Poor wound healing amoxicillin/calvulanate,
penicillin V, Ceftriaxone,
MUSCULOSKELETAL
azithromycin, cefaclor
 Growth and developmental delay:
 Prophylaxis with penicillin 500mg
young children are short but regain
daily and vaccination with
their height by adulthood. They
polyvalent pneumococcal and
however remain normal weight.

92
 Haemophilus influenza type b
vaccine.
 Folic acid is given in all patients
with hemolysis.
 Transfusion: for sudden, severe
anemia due to acute splenic
sequestration, aplastic crisis or
hyper-hemolytic crisis.
 Bone marrow transplantation is the
only cure.
 Hydroxyurea has be given to
patients with SCD, 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).
 15mg/kg/day increased by
2.5mg over 12 weeks
depending on response.
 Median life expectancy is in the
mid-40s.

93
CHAPTER 11: GASTROENTEROLOGY

GASTROENTEROLOGY
MALNUTRITION TYPES OF MALNUTRITION
 Nutrition is the provision of  These include:
adequate energy and nutrients to  Undernutrition:
meet the metabolic demands of the o Protein-energy malnutrition
body. (PEM)
 Essential nutrients cannot be o Micronutrient deficiencies
synthesized by the body and must  Over-nutrition:
be derived from the diet. They o Overweight and obesity
include certain vitamins, minerals,  Co-existence of under and
amino acids, fatty acids and a over-nutrition “double burden
carbohydrate source. of malnutrition”
 Nonessential nutrients can be o Obesity and PEM
synthesized from other compounds o Obesity and micronutrient
or may be derived from the diet. deficiencies in the same
individual
 Macronutrients supply energy and
 Acute and chronic malnutrition
essential nutrients needed for
growth, development, disease UNDERNUTRITION
prevention and activity. They  There is inadequate consumption,
include carbohydrates, proteins, poor absorption or excessive loss
fats and minerals. of nutrients.
 Malnutrition is defined as the  The terms protein energy
cellular imbalance between supply malnutrition (PEM) and
of nutrients and energy and the malnutrition are used
body’s demand for them to ensure interchangeably with
growth, maintenance and specific undernutrition.
function. (WHO)  Malnourished children may suffer
 Malnutrition is the result of a lack from numerous associated
or an excess in the provision of complications.
energy and/or nutrients to the body  They are susceptible to infections,
(undernutrition or especially sepsis, pneumonia and
overweight/obesity). gastroenteritis.

94
 Vitamin deficiencies and  Nutritional status is also expressed
deficiencies of minerals and trace in terms of standard deviation (SD)
elements can also be seen. or Z score of an anthropometric
 Malnutrition in young children is index such as WH. This score
conventionally determined through indicates deviation of a child’s
measurement of height, weight, nutritional status from median
skinfold thickness (or reference values.
subcutaneous fat) and age.  Malnutrition is defined as less than
 Weight-for-age (WA), height-for- 3 SD from the median (<3 Z
age (HA) and weight-for-height scores.
(WH) are the 3 athropometric  The welcome classification is a
indices used in assessing clinical classification for children
nutritional status. admitted to a hospital or a nutrition
 Classification by WA is known unit.
as Gomez classification  It is based upon WA (Gomez
o Over 90% is normal classification).
compared to mean WA  A child with a WA less than
o 76-90% is mild 60% and no edema is said to
malnutrition (First degree) have marasmus.
o 61-75% is moderate  A child with a WA greater than
malnutrition (Second 60% with edema has
degree) kwashiorkor.
o Less than 60% is severe
Weight/Age With edema Without
malnutrition (Third degree)
edema
 Classification by WH or by HA 60-80% of Kwashiorhor Undernutrition
is also called Waterlow expected
classification weight
o 80-90% below median WH <60% of Marasmic Marasmus
is considered mild expected kwashiorkor
malnutrition weight
o 70-80% below median WH
is considered moderate
malnutrition
CLASSIFICATION OF UNDER-
o <70% below median WH is NUTRITION
considered severe  Wasting: child is thin, he/she has
malnutrition lost fat and muscle mass so they
have a low weight for height.

95
  Usually due to a recent lack of  Presence of bipedal edema
food or illness (infections) that  Mid upper arm circumference <
prevents the child from eating 11.5cm.
or absorbing nutrients of foods.  No distinction is made between the
 Stunting: child is short in stature, clinical conditions of kwashiorkor
She/he did not grow in or severe wasting because their
length/height as he/she should treatment is similar.
have (Low height for age)  Children who are <-3SD weight-
 This is a long term process, for-age may be stunted (short
often starting in utero which is stature) but not severely wasted.
due to mother’s malnutrition,  Stunted children who are not
food intake lacking quality severely wasted do not require
(insufficient intake of essential hospital admission unless they
micronutrients) and the have a serious illness.
repetition of episodes of  The causes of malnutrition can be
common infections. viewed as immediate, underlying
 Underweight: child weighs less and basic:
than they should (low weight for  Immediate determinants: these
age) are immediate determinants of
a child’s nutritional status, they
include low birthweight,
infection (e.g. diarrhea and
pneumonia) and inadequate
dietary intake
 Underlying determinants:
include food (insufficient
quality, quantity and variety),
 A child can be both wasted and health (Access to curative and
stunted. preventive health services) and
care (Availability of food)
SEVERE ACUTE
 Basic determinants: social
MALNUTRITION economic status, education
 This is defined either by: level of the family, woman’s
 Weight-for height below-3 empowerment, cultural taboos
standard deviation of median regarding food and health,
WHO growth reference access to water and sanitation
 Visible wasting etc.

96
CLINICAL FEATURES OF o Head circumference and
MALNUTRITION brain growth retardation
o Changes in dendritic
MILD MALNUTRITION
arborization and
 It is most common between 9 morphology of dendritic
months and 2 years. spines.
 Main features: o Cerebral atrophy on
 Growth failure: manifested by CT/MRI
slow or cessation of linear o Abnormalities in auditory
growth, static or decline in brainstem potentials and
weight, decrease in mid-arm visual evoked potentials
circumference, delayed bone  CVS:
maturation, normal or o Changes in cardiac volume,
diminished weight for height Z muscle mass and electrical
scores and normal or properties of the
diminished skin fold thickness. myocardium
 Infection: e.g. gastroenteritis, o Systolic function affected
pneumonia and tuberculosis more than diastolic function
 Anemia: may be mild to  GIT
moderate and any o Upper GIT: mucosa shine
morphological type may be and atrophic, papillae of
seen tongue flattened
 Activity: diminished o Small and large intestines:
 Skin and hair changes (rarely mucosa and villi atrophic,
occur) brush border enzymes
reduced, hypotonic, rectal
MODERATE TO SEVERE
prolapse
MALNUTRITION
o Liver: fatty liver,
 Usually associated with one of deposition of triglycerides
classical syndromes, namely o Pancreas: exocrine
marasmus, kwashiorkor or with secretion depressed,
manifestations of both. endocrine function less
severely affected, glucagon
PATHOLOGICAL FEATURES
production reduced, insulin
 Malnutrition is multi-systemic and levels low, atrophy and
can affect many organs. degranulation or
 CNS: hypertrophy of islets seen.

97
  ENDOCRINE responsiveness, anxiety and
o Elevated growth hormone, attention deficits
thyroid involution and  Clinical signs and symptoms of
fibrosis, adrenal glands micronutrient deficiency:
atrophic and cortex thinned,  Iron: fatigue, anemia, decreased
increased cortisol, cognitive function, headache,
catecholamine activity glossitis and koilonychias
unaltered.  Iodine- goiter, developmental
 LYMPORETICULAR delay and mental retardation
o Thymus involuted, loss of  Vitamin D- poor growth,
distinction between cortex rickets and hypocalcemia
and medulla, depletion of  Vitamin A- night blindness,
lymphocytes, paracortical xerophthalmia, poor growth
areas of lymph nodes and hair changes.
depleted of lymphocytes,  Folate- glossitis, anemia
germinal centers smaller (megaloblastic), neural tube
and fewer. defects (in fetuses of women
without folate supplementation)
ASSESSMENT AND DIAGNOSIS
 Zinc- anemia, dwarfism,
OF MALNUTRITION hepatosplenomegaly,
 Malnutrition must be recognized hyperpigmentation and
and accurately defined for rational hypogonadism, acrodermatitis,
decisions to be made about enteropathica, diminished
refeeding. immune response, poor wound
 Evaluation is divided into: healing.
 Assessment of past and present  History of presenting complaint
dietary intake (History)  Usual diet before current
 Anthropometry (Examination) episode of illness
 Laboratory assessments  Breastfeeding history
HISTORY  Food and fluid intake taken in
past few days
 Clinical signs and symptoms of  Recent sinking of eyes
malnutrition:  Time when urine was last
 Poor weight gain passed
 Slowing of linear growth  History of body hotness or
 Behavioral changes- irritability, febrile illness
apathy, decrease social  Review of systems:

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  CNS: irritability, apathy,  This gives a guide to food
decreased social intake.
responsiveness, anxiety and  Breast feeding
attention deficits.  Family history: any recent death of
 CVS: easy fatigability, sibling, history of TB and measles.
palpitation, ankle or leg  Social economic history
swelling, dyspnea and  Employment status of parents
palpitations  Education level of parents
 Respiratory system: Cough >12  Situation at home (who takes
weeks care and feeds child)
 GIT:
EXAMINATION
o Vomiting & diarrhea
 Duration and frequency  On examination look for:
 Type of diarrhea  Shock: lethargic or
(watery/ bloody) unconscious, with cold hands,
 Loss of appetite slow capillary refill (>3s) or
 GUT: Time when urine was weak (low volume), rapid pulse
last passed and low blood pressure
 Musculoskeletal and skin: poor  Signs of dehydration
growth, pathological fractures,  Severe palmar pallor
rickets, dry peeling skin with  Bilateral pitting edema
raw exposed areas, and  Eye signs of vitamin A
hyperpigmented plaques over deficiency:
areas of trauma o Dry conjunctiva or cornea,
 Past medical history: Bitot spots (these are
 Contact with TB greyish foamy patches on
 Recent contact with measles the exposed bulbar
 Known or suspected HIV conjunctiva they are due to
infection/exposure build-up of keratin in
 Birth history: birth weight Vitamin A deficiency)
 Developmental history: milestones o Corneal ulceration
reached o Keratomalacia: softening of
 Immunization history the cornea, followed by
 Diet and nutritional history: perforation of the eyeball
 Parents are asked to record the and permanent blindness
food the child eats during (extreme care must be
several days. taken during ophthalmic

99
 examination at this stage,  Mid upper arm circumference
due to risk of rupturing <11.5cm
cornea)  Edema of both feet
 Localizing signs of infection, (kwashiorkor with or without
including ear and throat severe wasting)
infections, skin infection or
PHYSICAL FINDINGS
pneumonia.
 Signs of HIV infection  Decreased subcutaneous tissue:
 Fever (temp >37.5oC) or legs, arms, buttocks and face
hypothermia (rectal  Edema: distal extremities and
temperature <35.5oC) anasaraca (generalized edema)
 Mouth  Oral changes: cheilosis, angular
 Skin changes of kwashiorkor stomatitis, papillar atrophy
o Hypo-or hyperpigmentation  Abdominal findings: abdominal
o Desquamation distension secondary to poor
o Ulceration (spreading over abdominal musculature,
limbs, thighs, genitalia, hepatomegaly secondary to fatty
groin and behind the ears) infiltration
o Exudative lesions  Skin: dry peeling skin with raw
(resembling severe burn) exposed areas, hyperpigmented
often with secondary plaques over areas of trauma
infection (including  Nail changes: nails become
Candida) fissured or ridged.
 Conduct an appetite test:  Hair changes: hair is thin, sparse,
o Check if the child has brittle, easily pulled out (flag-post
appetite by providing ready hair), and turns a dull brown or
to use therapeutic food. reddish color.
ANTHROPOMETRY LABORATORY
 In addition to weight and height, INVESTIGATIONS
skinfold thickness of the triceps  Essential for detection of early
reflects (mid-arm circumference) physiological adaptation to
subcutaneous fat stores and can be malnutrition.
assessed by measuring it.  Clinical history, examination and
 Weight for length/height <-3SD anthropometry are of greater value
(wasted) than any single biochemical or
immunological measurement.

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 Lab investigations: malnutrition and should be
 Serum albumin admitted for inpatient care.
 Assay of specific minerals and  Children who have a good appetite
vitamins and no medical complications can
 Full blood count: low be managed as outpatients.
lymphocyte count, impaired
cell-mediated immunity
PROTEIN ENERGY
 Urea and electrolytes, MALNUTRITION
creatinine  Kwashiorkor and marasmus are 2
 Liver enzymes forms of PEM described.
 Clotting profile  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
 Kwashiorkor has fair to normal
calorie intake and 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
 Children with severe acute dysadaptation to starvation.
malnutrition should first be  In addition to PEM children may
assessed with a full clinical be affected by micronutrient
examination to confirm whether deficiencies that can have
they have any general danger sign, detrimental effects on growth and
medical complications and an development.
appetite.  The most common and clinically
 Children with severe acute significant micronutrient
malnutrition with loss of appetite deficiencies in children and
or any medical complication have childbearing women worldwide
complicated severe acute are iron, iodine, zinc and vitamin
A.

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 Deficiencies in Vitamins C, B and nutrition causing anorexia,
D have improved although still decreased nutrient absorption,
remain a challenge in developing increased metabolic needs and
countries. direct nutrient losses.
 Pathophysiology:  See “systemic effects of
 Malnutrition affects virtually malnutrition” above.
every organ system. Dietary
GENERAL MANAGEMENT
protein is needed to provide
amino acids for synthesis of SEVERE ACUTE
body proteins and other MALNUTRITION
compounds that have a variety  Principles of management involves
of functional roles. correction and prevention of:
 Energy is essential for all 1. Hypoglycemia
biochemical and physiological 2. Hypothermia
functions of the body. 3. Dehydration
 Micronutrients are essential in 4. Electrolytes
many metabolic functions in 5. Infections
the body as components and 6. Micronutrients
cofactors in enzymatic 7. Initiate feeding
processes. 8. Catch-up feeding
 Immunosuppression is seen in 9. Sensory stimulation
malnutrition and changes 10.Prepare for follow up
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

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HYPOGLYCEMIA  Give the first feed of F-75
therapeutic milk, if it is quickly
 All severely malnourished children
available and then continue
are at risk of hypoglycemia and
with feeds every 2h for 24h,
immediately on admission, should
then continue feeds every 2h or
be given a feed or 10% glucose or
3, day and night.
sucrose. Frequent 2 hourly feeding
 If the child is unconscious treat
is important.
with IV 10% glucose at 5ml/kg
 Diagnosis:
or if IV access cannot be
 Blood glucose <3mmol/litre
quickly established, then give
(<54mg/dl)
10% glucose or sucrose
 If blood glucose cannot be
solution by NGT.
measured it should be assumed
 If IV glucose is not available,
that all children with SAM are
give one teaspoon of sugar
hypoglycemia and given
moistened with 1 or 2 drops of
treatment.
water sublingually and repeat
 Treatment: every 20 min to prevent
 Give 50ml of 10% dextrose relapse. Children should be
(one rounded teaspoon of sugar monitored for early swallowing
in 3 tablespoons of water) which leads to delayed
orally or by NGT followed by absorption, in this case another
the first feed as soon as dose of sugar should be given.
possible.

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 Continue with 2h oral or NGT  When a low-reading
feeds to prevent recurrence thermometer is available take
 Start on appropriate IV or IM rectal temperature (<35.5oC) to
antibiotics. confirm hypothermia
 Monitoring  Treatment:
 Repeat glucose measurement  All children with hypothermia
after 30min. should be treated routinely for
 If blood glucose <3mmol/l (<54 hypoglycemia and infection.
mg/dl) repeat the 10% glucose  Feed the child immediately and
or oral sugar solution. then every 2h unless they have
 If the rectal temperature falls abdominal distention, if
<35.5oC or if level of dehydrated, rehydrate first.
consciousness deteriorates  Re-warm child: ensure child is
repeat glucose measurement clothed (especially the head),
and treat accordingly. cover with a warmed blanket
 Prevention and place a heater (no pointing
 Feed every 2h starting directly at the child) or lamp
immediately or when nearby, or put the child on the
dehydrated, rehydrate first. mother’s bare chest or abdomen
Continue feeding throughout (skin-to-skin) and cover them
the night. with a warmed blanket and/or
 Encourage mothers to watch for warm clothing.
any deterioration, help feed and  Keep the child away from
keep the child warm. draughts.
 Check on abdominal distention.  Give appropriate IV or IM
antibiotics
HYPOTHERMIA
 Monitoring
 This is very common in  Rectal temperature 2 hourly
malnourished children and often until it rises to >36.5oC. Take it
indicates coexisting hypoglycemia every 30 min if a heater is
or serious infection. being used.
 Diagnosis  Ensure child is covered at all
 Axillary <35oC or does not times, especially at night. Keep
register on a normal the head covered, preferably
thermometer, assume with a warm bonnet, to reduce
hypothermia. heat loss.

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  Check for hypoglycemia  It is important to note that poor
whenever hypothermia is found circulatory volume or perfusion
 Prevention can co-exist with edema.
 Feed immediately and then
every 2-3h, day and night. CALCULATION OF
 Place the bed in a warm, CALORIES AND FEEDS
draught-free part of the ward
and keep the child covered.
 Use the kangaroo technique for
infants cover with a blanket and GASTROENTERITIS
let the mother sleep with child  Gastroenteritis also known as
to keep the child warm. infectious diarrhea is inflammation
 Avoid exposing the child to of the gastrointestinal tract.
cold (e.g. after bathing or  Symptoms may include diarrhea,
during medical examination) vomiting and abdominal pain.
 Change wet nappies, clothes
 Fever, lack of energy and
and bedding to keep the child
dehydration may also occur. This
and the bed dry. Dry carefully
typically lasts less than 2 weeks.
after bathing bit do not bathe if
 It is not related to influenza though
very ill.
it has sometimes been called
 Use a heater or incandescent
“stomach flu”.
lamp with caution.
 Gastroenteritis is caused by viruses
 Do not use a hot water bottle or
however bacteria, parasites and
fluorescent lamp.
fungus can also cause
DEHYDRATION gastroenteritis
 Dehydration tends to be over- ETIOLOGY
diagnosed and its severity  Viral (particularly Rotavirus in
overestimated in children with children) and bacteria are the
SAM because it is difficult to primary causes of gastroenteritis.
determine accurately from clinical  Non-infectious causes are seen on
signs alone. occasion but they are less likely
 Assume that all children with than a viral or bacteria cause.
watery diarrhea or reduced urine  Risk of infection is higher in
output have some dehydration. children due to their lack of
immunity.

105
 Children are also at higher risk PATHOPHYSIOLOGY
because they are less likely to  Gastroenteritis is defined as
practice good hygiene habits. vomiting or diarrhea due to
inflammation of the small or large
VIRAL
bowel often due to infection.
 Rotavirus, norovirus, adenovirus
 The changes in the small bowel
and astovirus are known to cause
typically noninflammtory while
viral gastroenteritis.
the ones in the large bowel are
 Rotavirus being most common
inflammatory.
BACTERIAL  The number of pathogens required
 Include Escherichia coli, to cause infection varies from as
Salmonella, Shigella, few as one (for cryptosporidium)
campylobacter, Vibrio cholera, to as many as 108 (for V. cholera).
Clostridium difficile, and
SIGNS AND SYMPTOMS
Staphylococcus aureus
 Gastroenteritis usually involves
PARASITIC both diarrhea and vomiting.
 These include Giardia lamblia,  This is accompanied by abdominal
Entamoeba histolytica. cramps.
 Cryptosporidium spps have also  Signs and symptoms usually begin
been implicated. 12-72 hours after contracting the
 In immunocompromised: infectious agent.
Cryptosporidium, Cyclospora,  If it is due to virus the condition
Isospora belli, microsporidium usually resolves in one week.
Some viral infections also involve
NON-INFECTIOUS fever, fatigue, headache and
 Medications: NSAIDs muscle pain.
 Certain foods: lactose (lactose  If stool is bloody (dysentery) the
intolerance, Gluten (celiac disease) cause is less likely to be viral and
 Crohn’s disease more likely to be bacterial.
 Tetrodotoxin from consumption of  Some bacterial infections cause
puffer fish severe abdominal pain and may
 Botulism due to improper persist for several weeks.
preservation of food.
DIAGNOSIS
 Diagnosis is clinical based on a
person’s signs and symptoms.

106
 Determining the exact cause is  For children at risk of dehydration
usually not needed as it does not from vomiting taking a single dose
alter management of the condition. of the anti-emetics such as
 Stool culture should be performed metoclopramide or ondansetron
in those with blood in the stool, may be helpful.
those who might have been  Analgesics for the abdominal pain.
exposed to food poisoning.  The main primary treatment of
 Diagnostic testing may also be gastroenteritis is rehydration.
done for surveillance.  Antibiotics are used if symptoms
 As hypoglycemia occurs in are particularly severe or if a
approximately 10% of infants and susceptible bacterial cause is
young children measuring glucose isolated or suspected.
is recommended.  Macrolides (e.g. azithromycin) is
 Electrolytes and kidney function preferred over a fluoroquinolone
should also be checked when there due to rates of resistance to the
is a concern about severe latter.
dehydration.  Metronidazole is also used.
DIFFERENTIAL DIAGNOSIS PREVENTION
 Inflammatory bowel disease  Hand washing
 Malabsorption syndrome  Drinking clean chlorinated water
 Lactose intolerance  Proper disposal of human waste
 Appendicitis  Breastfeeding
 Volvulus  Vaccination
 Urinary tract infection
COMPLICATIONS
 Whipple’s disease
 Dehydration is the main
 Celiac disease
complication.
 Diabetes mellitus
MANAGEMENT
 Gastroenteritis is usually an acute DIARRHEA
and self-limiting disease that does  Diarrhea is defined as change in
not require medication. consistency and frequency of
 The preferred treatment in those stools i.e. liquid or watery stools,
with mild to moderate dehydration that occur >3 times a day.
is oral rehydration therapy.  If there is associated blood in stool
it is termed dysentery.

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 Diarrhea occurring within 14 days  Non-infectious (inorganic)
is termed acute diarrhea.
INFECTIOUS CAUSES
 Diarrhea that starts off as acute
and lasts more than 14 days is  These can be classified as:
termed persistent diarrhea.  Viral
 Diarrhea that has an insidious o Rotavirus, Norovirus spp
onset and lasts longer than 14 days (Norwalk virus),
is termed chronic diarrhea. cytomegalovirus, Enteric
 The most important consequences adenovirus, Astrovirus,
of diarrhea in children are Coronaviruses,
malnutrition and dehydration. Picornavirus, human
 Malnutrition and diarrhea form calicivirus, HIV
a vicious cycle since  Bacterial
malnutrition increases the risk o Escherichia coli:
and severity of diarrhea. o Shigella
 Impaired absorption, loss of o Vibrio cholerae
nutrients, increased catabolism o Salmonella
and improper feeding in o Campylobacter spp (e.g. C.
diarrhea aggravate the severity jejuni)
of malnutrition. o Clostridium perfringes,
Clostridium difficile,
ACUTE DIARRHEA Staphylococcus aureus,
 This is the abrupt onset of 3 or Plesiomonas shigelloides,
more loose stools per day. Aeromona spp.
 The increased water content in  Parasitic
stool (above the normal value of o Giadia lamblia,
approximately 10ml/kg/d in the Cryptosporidium parvum,
infant and young child or 200g/d Entamoeba histolytica,
in the teenager and adult) is due to Cyclspora cayetanensis,
an imbalance in the physiology of Isospora belli, Balantidium
the small and large intestines coli, Blastocystis hominis,
involved in the absorption of ions, Trichuris trichiura
organic substrates and thus water.  Note ‘*’ denotes causative agents
that can cause dysentery
CAUSES
 Acute diarrhea can be divided into: NONINFECTIOUS CAUSES
 Infectious (organic)
 Include:

108
  Drugs: antibiotics vomitus, is the vomiting
(erythromycin) projectile (pyloric stenosis)?
 Food allergy  Is the child passing less urine
 Surgical conditions: than normal? (Ask when was
Appendicitis or Hirschsprung the last wet nappy)
disease  Presence of fever, cough, rash,
 Inflammatory bowel disease abdominal pain or other
 Hyperthyroidism significant symptoms (e.g.
 Malabsorption syndromes: convulsions, recent measles,
celiac disease, lactose attacks of crying with pallor in
intolerance an infant)
 Is the child able to drink or
feed?
 The majority of cases of acute  If so, is he/she thirstier than
diarrhea in children are of normal, does he/she drink
infectious origin. eagerly?
 Past medical history:
PATHOPHYSIOLOGY
 History of recent antibiotic use
(e.g. may be as a result of C.
CLINICAL PRESENTATION difficile infection)
 Does the child have cystic
fibrosis or diabetes?
ASSESSMENT OF A CHILD  Drug and immunization history
WITH ACUTE DIARRHEA  Drugs or other local remedies
HISTORY taken (including opioids or
 The history of presenting anti-motility drugs e.g.
complaint should contain loperamide that may cause
information on: abdominal distention)
 Onset of diarrhea, association  Immunization history
with meals  Nutrition and feeding history:
 Duration and number of stools  Type and amount of fluids
per day (including breast milk) and
 Nature: Blood or mucus in food taken during the illness
stools and just before the illness
 Number of episodes of  Unusual foods or recent
vomiting, amount, color of restaurant meal

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 Social economic: day care o Cough
attendance or travel o High grade fever
o Tachypnea and/or chest in-
PHYSICAL EXAMINATION
drawing suggesting
 A detailed physical examination pneumonia
should focus on assessment of o High grade fever with
hydration, particularly in young splenomegaly suggests
children. malaria
 Look for: o Fungal infections (oral
 Signs of dehydration thrush or perianal satellite
o General condition: lesions)
Lethargy/unconsciousness  Abdominal mass
, Restlessness or  Abdominal distention
irritability  Note: there is no need for routine
o Depressed anterior stool microscopy or culture in
fontanelle (in infants children with non-bloody diarrhea.
below 18 months)
INVESTIGATIONS
o Sunken eyes
o Lack of tears when crying  Patients can usually be managed
o Dry mucous membranes even without investigations.
o Unable to drink or drinks  Stool microscopy, culture and
poorly/ drinks eagerly, sensitivity (MCS)
thirsty  Stool microscopy is not helpful
o Skin pinch (turgor): in management except in
 Flatten immediately selected cases e.g. cholera and
 Goes back slowly giardiasis.
 Goes back very slowly  Stool culture is of little value in
(>2s) routine management of acute
o Tachycardia diarrhea.
 Blood in stool  Sensitivity is important in
 Signs of severe malnutrition deciding on antibiotic therapy
o Anthropometric e.g. in patients with Shigella
measurements dysentery and do not respond to
o Wasting initial empiric antibiotics.
o Edema  Full blood count: ruling out any
o Signs of vitamin deficiency underlying anemias, systemic
 Signs of infection: infections, coagulopathies

110
 Urea, Creatinine and electrolytes:  Skin pinch goes back quickly.
for electrolyte imbalance and renal  Use treatment plan A.
function
MANAGEMENT
DEHYDRATION
 The 3 essential elements in the
 For all children with diarrhea, their management of all children with
hydration status should be diarrhea are:
classified.  Rehydration therapy
 The degree of dehydration is  Zinc supplementation
graded according to symptoms and  Counselling for continued
signs that reflect the amount of feeding and prevention
fluid lost, they include:
 No dehydration (usually <5% REHYDRATION
loss of body weight) NO DEHYDRATION (PLAN A)
 Some dehydration (usually 5-
10%)  Usually do not need admission if
 Severe dehydration (usually there are no other co-morbidities.
>10%)  Educate the mother/caregivers on
 This classification makes the danger signs of dehydration.
management of acute diarrhea  Continuing diarrhea beyond 3
easy. days
 Increased volume/frequency of
NO DEHYDRATION stools
 There are not enough signs to  Repeated vomiting
classify as some or severe  Increasing thirst, refusal to
dehydration. feed, fever or blood in stool
 Child is well alert; eyes are normal  Encourage the mother to continue
(not sunken), tears are present breast feeding (longer and more
when crying, mouth and tongue frequently).
are moist.  Give ORS (10ml/kg per loose
 In some infants and children, the stool)
eyes normally appear somewhat  If the child is exclusively
sunken. It is helpful to ask the breastfed, give ORS or clean
mother if the child’s eyes are water in addition.
normal or more sunken than usual.  If child is not exclusively
 Child drinks normally and is not breastfed, give one or more of
thirsty. ORS, food based fluids (such as

111
 soup, rice water, yoghurt) or  For >6 months: one tablet
clean water. (20mg) per day for 10-14 days.
 Teach mother how to mix and  For infants dissolve the tablet
give ORS. Give mother 2 in a small amount of clean
packets of ORS to use at home. water, expressed milk or ORS
Also educate mother on how to in a small cup or spoon.
make ORS  Older children can chew the
 For additional fluids given tablet or drink it dissolved in a
(ORS): small amount of clean water in
o <2 years: 50-100 ml after a cup of spoon
each loose stool  Advise mother to return to
 Per day give 500ml clinic/hospital if condition worsens
o > 2 years: 100-200 ml after or does not improve.
each loose stool  Give antibiotics: ciprobid or
 Per day give 1000ml cefotaxime, only give
 If ORS packs are not present metronidazole if there is bloody
oral rehydration salts can be diarrhea
made using:  Deworm- mebendazole
o 1 level teaspoon (5ml) of
salt (do not use too much SOME DEHYDRATION
salt. If solution has too  There are 2 or more of the
much salt the child may following signs:
refuse to drink it).  Restlessness, irritability
o 8 level teaspoons of sugar  Sunken anterior fontanelle and
(preferably brown sugar eyes
which contains more  Tears are absent when crying
potassium than white sugar)  Mouth and tongue are dry
o 1 liter of drinking water  Eager drinking, thirsty
 Tell the mother to give frequent  Skin pinch goes back slowly
small sips from a cup, if the  Weigh the patient, if possible and
child vomits wait 10 minutes. use treatment plan B.
Then continue but more slowly.
Continue giving extra fluid MANAGEMENT
until the diarrhea stops.  The 3 essential elements in the
 Zinc sulphate management of all children with
 For <6 months: half tablet diarrhea are:
(10mg) per day for 10-14 days.  Rehydration therapy

112
  Zinc supplementation  Give antibiotics: ciprobid or
 Counselling for continued cefotaxime, only give
feeding and prevention metronidazole if there is bloody
diarrhea
REHYDRATION
 Deworm- mebendazole
SOME DEHYDRATION (PLAN B)
SEVERE DEHYDRATION
 All cases with obvious signs of
dehydration need to be treated in  There are 2 or more of the
health centers or hospitals. following signs:
 Lethargy or unconsciousness
 Give 75ml/kg in 4 hours of ORS
 Depressed (severely sunken)
(if fluids can be tolerated) and
anterior fontanelle and eyes
reassess after 4 hours. If there is no
dehydration transition to plan A  When child cries there are no
(10ml/kg per loose stool). tears
 Tell the mother to give frequent  Mouth and tongue are dry
 Unable to drink or drinks
small sips from a cup, if the
child vomits wait 10 minutes. poorly
Then continue but more slowly.  Skin pinch goes back very
Continue giving extra fluid slowly (>2s)
until the diarrhea stops.  Weigh the patient and use
 If fluids are not tolerated orally treatment plan C urgently.
give IV ringers lactate. MANAGEMENT
 Zinc sulphate
 The 3 essential elements in the
 For <6 months: half tablet
management of all children with
(10mg) per day for 10-14 days.
diarrhea are:
 For >6 months: one tablet
 Rehydration therapy
(20mg) per day for 10-14 days.
 Zinc supplementation
 For infants dissolve the tablet
 Counselling for continued
in a small amount of clean
feeding and prevention
water, expressed milk or ORS
in a small cup or spoon. REHYDRATION
 Older children can chew the
SEVERE DEHYDRATION (PLAN
tablet or drink it dissolved in a
C)
small amount of clean water in
a cup of spoon  Start IV fluid immediately. If the
child can drink, give ORS by

113
 mouth while the drip is being set water, expressed milk or ORS
up. in a small cup or spoon.
 Give 100ml/kg Ringer’s lactate  Older children can chew the
solution (or if not available normal tablet or drink it dissolved in a
saline) small amount of clean water in
 For children less than 1 year (give a cup of spoon
fluids over 6 hours)  Give antibiotics: ciprobid or
 First 30ml/kg: give in 1 hour cefotaxime, only give
 Last 70ml/kg: give in 5 hours metronidazole if there is bloody
 For children above 1year to 5 diarrhea
years (give 100ml/kg in 3 hours)  Deworm- mebendazole
 First 30ml/kg: give in 30
DIFFERENTIAL DIAGNOSIS
minutes
 Last 70ml/kg: give in 2hours 30  Acute (watery) diarrheal disease-
minutes more than 3 loose stools per day,
no blood in stool.
 Reassess the child every 15-10
min. If hydration status is not  Cholera: profuse watery diarrhea
improving give IV drip more with severe dehydration during
rapidly. Also watch for over- cholera outbreak, positive stool
hydration. culture for Vibrio cholera 01 or
0139.
 Also give ORS (about 5ml/kg per
h) as soon as the child can drink:  Dysentery- blood mixed with the
usually after 3-4 h (infants) and 1- stools (seen or reported)
2 h (children).  Persistent diarrhea: diarrhea lasting
 Reassess an infant after 6 hours more than 14 days
and a child after 3 hours. Classify  Diarrhea with severe malnutrition:
dehydration. Then choose the any diarrhea with signs of severe
appropriate plan (A, B or C) to acute malnutrition.
continue treatment.  Diarrhea associated with recent
 Zinc sulphate antibiotic use: recent course of
 For <6 months: half tablet broad-spectrum oral antibiotics
(10mg) per day for 10-14 days.  Intussusception: blood and mucus
 For >6 months: one tablet in stools, sausage shaped
(20mg) per day for 10-14 days. abdominal mass, attacks of crying
 For infants dissolve the tablet with pallor in infant or young
in a small amount of clean child.

114
ENTERIC FEVER disease- fever and abdominal pain
 Enteric fever is an acute systemic are variable.
illness characterized by fever,  Incubation period is 3 to 21 days.
headache and abdominal  After ingestion, infection with
discomfort caused by Salmonella salmonellae is characterized by
typhi (and less commonly attachment of the bacteria by
Salmonella paratyphi A, B, or C). fimbriae/pili to cells lining the
 Man is the only known host for S. intestinal lumen.
typhi.  Gastric acid is protective so for
 The organism enters the body via infection to happen its either
the gastrointestinal tract and gains there is low gastric acid
access to the bloodstream via the secretion, immunocompromise
lymphatics. or a large inoculum.
 The disease was initially called  The cells specifically infect the M
typhoid fever because of its cells of the peyers patches (in the
clinical similarity to typhus. terminal part of the ileum).
 The bacteria are internalized by
receptor mediated endocytosis and
STRUCTURE OF transported with phagosomes to
SALMONELLA lamina propria where they are
 Salmonella is a member of the released.
family Enterobacteriaecae.  Once in the lamina propria to
 It is an encapsulated facultative salmonella induces an influx of
anaerobic non-spore forming gram macrophages (typhoidal strains) or
negative bacilli. neutrophils (non-typhoidal strains),
 Like other organisms in the family  The Vi antigen is important in
the bacterium has: preventing antibody mediated
 Somatic antigen (O antigen) opsonization and complement-
 Flagella antigen (H antigen) mediated lysis.
 Capsular antigen: Vi (K)  The S. typhi organisms through
induction of cytokine release
PATHOGENESIS spread through the
 Transmission is via fecal oral reticuloendothelial system to the
route. regional lymph nodes (Especially
 Enteric fever is a misnomer, in that the mesenteric lymph nodes).
the hallmark features of this

115
 Upon further multiplication insomnia, anorexia, epistaxis and
bacteremia results and there is abdominal pain.
spread to the phagocytes of the  Patients can present with either
liver, gallbladder and spleen. diarrhea or constipation (due to
 The usual site of carriage is the hypertrophy of Peyers patches)
gallbladder (chronic carrier is  Diarrhea is more common
associated with gall stones). among patients with AIDS and
 In some areas endemic for among children <1 year of age.
urinary schistosomiasis chronic  Diarrhea occurs early but
carriage is in the urinary usually disappears by the time
bladder. fever and bacteremia occur.
 The pathogen can be excreted into  After 1 week as bacteremia
bile and feces further transmitting becomes sustained high fever,
the disease. delirium, tender abdomen and
enlarged spleen occur.
CLINICAL FEATURES  Rose spots (rose colored macules)
 A pro-drome of nonspecific on the abdomen may be seen but
symptoms often precedes fever rarely occur.
and include:  Leukopenia and anemia are often
 Chills seen.
 Headache
 Physical findings:
 Anorexia
 Rose spots
 Cough
 Abdominal tenderness
 Weakness,
 Lymphadenopathy
 Sore throat
 Hepatosplenomegaly
 Dizziness
 Within the 3 weeks without
 Muscle pains
treatment serious complications
 Gastrointestinal symptoms are
can arise such as:
quite variable.
 Meningitis
 The onset of illness is insidious  Encephalitis
and non-specific with persistent  Coma
step ladder fever (lasting more  Lobar pneumonia
than one week, caused by  Myocarditis
endotoxins), headache, asthenia  Osteomyelitis
(someone is weak and does not  Intestinal perforation
have any strength to walk),  Peritonitis
 Intestinal hemorrhage

116
 The fourth week of illness is  Sensitivity and specificity is
characterized by gradual poor.
improvement but 30% of those
DIFFERENATIAL
infected will die and 10% of
untreated will relapse. DIAGNOSIS
 After clinical recovery 5-10% of  Malaria
patients will continue to excrete  Urinary infections
the pathogen severe months  Lower respiratory tract infection
(convalescent carriers)  Dengue fever in endemic areas.
 1-4% will carry organisms for MANAGEMENT AND
more than 1 year (Chronic
PREVENTION
carriage)
 Carriage in the gall bladder and  Isolate the patient.
urinary bladder.  Keep under close surveillance,
hydrate, treat fever.
DIAGNOSIS  Quinolones are the drug of choice.
 Definitive diagnosis: culture of S. (oral route is more effective than
typhi or S. paratyphi from patient parenteral, if patient cannot take
 Blood culture is positive in oral treatment start by injectable
most cases in first 2 weeks. route and change to oral route as
 Culture from intestinal soon as possible).
secretions and urine is also  Ciprofloxacin PO for 5 to 7
used. days
 Bone marrow culture is more  Children: 30mg/kg/day in 2
sensitive than blood culture but divided doses (usually not
is rarely required in patients on recommended in children under
antibiotics. 15 years however, the life-
 Remember “BUS” threatening risks of typhoid
 You find the parasite in blood outweighs the risk of adverse
in first week, urine in second effects)
week and Stool in third week  Alternative: Cefixime PO for 7
 FBC: leukopenia is common but days in children under 15. For
non-specific. those over 6 months: 15 to
 Serological test as Widal antigen 20mg/kg/day in 2 divided
test are of little practical value. doses.
 It is easily misinterpreted  Adults: 1g/day in 2 divided
doses

117
 In patients presenting severe  Other sources of infection include
typhoid with toxic confusional contaminated foodstuffs, utensils
state (hallucinations, altered and houseflies.
consciousness) or intestinal
hemorrhage:
PATHOGENESIS
 Dexamethasone: IV loading  Cholera has one of the shortest
dose 3mg/kg and then 1mg/kg incubation period of 6 hours to 5
every 6 hours for 2 days. days.
 Prevention:  After ingestion, the organisms
 Disinfection of feces with 2% have to pass through the acid
chlorine solution barrier of the stomach.
 Individual (hand washing) and  In the stomach they inactivate
collective hygiene (safe water almost all their biological
supply, sanitation) processes
 The possibility of vaccination  Once they survive the stomach
must be considered: it can be acid they colonize the upper small
useful in some situations (high- intestines.
risk-age group, hyper-endemic  For colonization, a relatively large
zone), but its effectiveness inoculum of V. cholera is required.
remains controversial as both  The organisms produce an
injectable inactivated or oral enterotoxin, cholera toxin which
live attenuated vaccines only causes symptoms.
give partial protection.  The cholera toxin has 2
subunits i.e. an A and B
CHOLERA subunit.
 Cholera is caused by Vibrio  The B unit has affinity for
cholerae. intestinal epithelial mucosa
 Vibrio cholerae is a gram negative,  The A unit enters the
motile, comma shaped organism enterocytes and activates
with a flagellum. adenylyl cyclase which
 Two pathogenic strains V. increases the intracellular
cholerae 01 and 0139 are known. concentration of cAMP.
 The route of transmission is feco-  This results in the excretion of
oral. chloride into the lumen leading
 Contaminated water serves as a to decreased absorption of
reservoir and frequently the source sodium from villous cells.
of infection.

118
  As sodium absorption is  There are generally no fecal
impaired, water is poured out leucocytes.
from intestinal epithelium.  Stool culture confirms the
 The outpouring of fluid and diagnosis as well as helps in
electrolytes produces the identifying the type V. cholerae.
watery diarrhea and related  It is best cultured on thiosulphate
changes. citrate bile sucrose media (TCBS)
CLINICAL FEATURES  Estimation of serum electrolytes
and blood sugar levels is useful for
 Infection can be mild self-limiting
appropriate management of sick
or even asymptomatic.
children.
 Severe infection leads to profuse
watery diarrhea accompanied by TREAMENT
vomiting.  Fluid and electrolyte replacement.
 In young children there can be  If the child can take orally then
significant fever. ORS should be given ad libitum.
 The stools are watery with a fishy  In case oral intake is not possible
odour and the mucus flakes give it or inadequate, intravenous
the typical rice water appearance. rehydration is essential.
 The fluid and electrolyte loss can  Hyponatraemia, hypokalaemia and
be massive leading to symptoms acidosis need appropriate
and signs of severe dehydration attention.
and even circulatory collapse and  Antibiotics can help in shortening
acute renal failure. the duration of illness and possibly
 The outpouring of watery diarrhea the carrier rate but are generally
can continue for 5-7 days. not used.
 Drug of choice is oral
DIAGNOSIS
tetracyclin for 3 days.
 History of massive watery diarrhea
 In younger children,
(rice water) in a patient returning
trimethroprim-
from or residing in an endemic
sulphamethoxazole (septrin)
area.
combination can be used.
 Examination of fresh stool sample
 Prevention is by practicing good
as a hanging drop preparation
hygiene and health measures.
under the microscope can show the
 There are 2 oral vaccines, 1 killed
darting motile V. cholerae.
subunit and another live attenuated
present but protection only lasts

119
 for 6 months and they do not  Gestational diabetes
protect against 0139 strain.  Almost all children have type 1
diabetes requiring insulin from the
COMPLICATIONS
outset.
 Electrolyte imbalance
 Type 2 diabetes due to insulin
 Shock and dehydration resistance is starting to occur in
childhood, as severe obesity
becomes more common.
 Impaired glucose tolerance (IGT)
DIABETES MELLITUS indicates blood glucose levels
 Diabetes mellitus is a metabolic between normal and diabetic cut
disorder characterized by off points during glucose tolerance
hyperglycemia and glycosuria. test. (between 7.8- 11.0mmol/l 2
 Random blood sugar >11.1 hours after glucose load)
mmol/l (>200mg/dl) TYPE 1 DIABETES
 Fasting blood sugar >7 mmol/l
MELLITUS
(>126 mg/dl)
 In type 1 DM etiology is
 This is the second most common
multifactorial with genetic,
chronic disease of childhood,
environmental and autoimmune
affecting 1 of 500 children.
factors:
 It affects boys more than it does
 Genetic factors:
girls (2:1).
o There are strong genetic
 The factors that contribute to influences but inheritance
hyperglycemia include decreased has not been found to fit
insulin secretion, decreased insulin into the Mendelian patterns.
action and increased glucose o About 95% of patients have
production. HLA-DR3 or DR4.
 Based on etiology the types of o Monozygotic twins have a
diabetes include 50% concordance rate,
 Type 1: formerly known as whereas dizygotic twins
insulin dependent (idiopathic or have only have a 30%
autoimmune B-cell destruction) concordance rate.
 Type 2: formerly known as  Environmental triggers:
non-insulin dependent (Defects o Viral infections have been
in insulin secretion or action) implicated including
 Other specific types enteroviruses (coxsackie),

120
 mumps, reovirsues and and against glutamic acid
rubella. decarboxylase (GAD)
o Whether the early  Molecular mimicry probably
introduction of cow’s milk occurs between an environmental
(protein) might trigger DM trigger and an antigen on the
is controversial. surface of the B-cells of the
 Autoimmune factors: the pancreas resulting in an
autoimmune process begins autoimmune process (especially in
with lymphocytic infiltration of genetically predisposed
the pancreas. individuals) which damages the
o Islet cells antibodies pancreatic B-cells and leading to
present in 85% of patients. insulin deficiency.
o These antibodies may be  Note: counter –regulatory
detected in asymptomatic hormones to insulin include:
patients 10 years before the glucagon, cortisol, catecholamines,
clinical symptoms. thyroxin, GH & somatostatin and
o Other markers include: Sex hormones
antibodies against insulin

121
CLINICAL FEATURES sugar above 200mg/dl (>11.l
 There are 2 peaks of presentation mmol/L) with symptoms (polyuria,
of type 1 DM: preschool and polydipsia, weight loss or
teenagers. nocturia), glycosuria and
 Classical presentation: ketonuria.
 Polyuria  Where there is doubt a fasting
 Polydipsia blood glucose above 7mmol/L
 Nocturia and occasional (126mg/dl) or a raised
enuresis. glycosylated hemoglobin (HbA1C)
 As symptoms progress, weight may be helpful.
loss, polyphagia, fatigue, vomiting  A diagnostic glucose tolerance test
and dehydration occur. is rarely required in children.
 Diabetic ketoacidosis (DKA) may  2 hour postprandial plasma
be the initial presentation in 50% glucose ≥ 11.1 mmol/L
of patients. The younger the (200mg/dl) after a glucose load
patient, the shorter the course of of 75g (during oral glucose
symptoms before DKA occurs. tolerance test)
 DKA may be misdiagnosed if the MANAGEMENT
hyperventilation is mistaken for  The goals of therapy include:
pneumonia or the abdominal pain  Eliminate symptoms related to
for appendicitis or constipation. hyperglycemia.
 Adolescents may present with type  Reduce and delay the
1 DM during their pubertal growth complications.
spurt with hormones that are  Achieve normal lifestyle and
antagonistic to insulin action normal emotional and social
(specifically growth hormone and development.
sex steroids)  Achieve normal physical
 Remember: acute infection in growth and development
young non-diabetic children can  Detect associated disease early.
cause hyperglycemia without  Insulin:
ketoacidosis.  Types include: short-acting,
DIAGNOSIS intermediate-acting, long-acting
 Patients must have hyperglycemia and very long-acting.
documented by a random blood o Short acting (neutral,
soluble, regular): peak 2-3

122
 hours and duration up to 8 o Example if daily
hours requirements is 30IU/day
o Intermediate acting:  Morning= 2/3 x 30IU=
 Isophane (peak 6-8h and 20IU (2/3 is lente and
duration 16-24h) 1/3 is soluble insulin)
 Biphasic (peak 4-6h and  Evening= 1/3 x 30IU=
duration 12-20h) 10IU (2/3 is lente and
 Semilente (peak 5-7h 1/3 is soluble insulin)
and duration 12-18h)
o Long acting (lente,
ultralente and PZI): peak 8-
14h & duration.
 Insulin pumps are now being
used in children to achieve
better glucose control and to  Monitoring
improve lifestyles  Daily blood glucose: before all
 Daily insulin requirements 0.5- meals and at bedtime
1 unit/kg/day for prepubertal  Glycosylated hemoglobin level
and 2IU/kg/day for adolescents. reflecting diabetic control for
o Current regimen uses the past 2-3 months, should be
soluble insulin and lente check every 3 months.
o 2 subcutaneous injections  Watch for hypoglycemia: all
are given before breakfast patients should have parenteral
and at dinner. glucagon available in case of
o 2/3 of total insulin seizure or coma secondary to
requirement is given in the low blood sugar.
morning  Watch for “honeymoon”
 2/3 of this being lente period.
 1/3 of this being soluble o Within a few weeks after
insulin initial diagnosis, 75% of
o 1/3 of total insulin patients exhibit a temporary
requirement is given at progressive reduction in
dinner their daily insulin
 2/3 of this being lente requirements.
 1/3 of this being soluble o This is because of a
insulin transient recovery of
residual islet cell function,

123
 resulting in endogenous in the morning (3am to
release of insulin in 8am) but for reasons that
response to carbohydrate differ.
exposure. o It results from a rise in
o This honeymoon period early morning blood sugar
may last anywhere from levels which are triggered
months to 1-2 years. by declining levels of
o Can confuse patients and insulin and an increase in
parents if not educated growth hormone.
about it early. o Testing blood sugar levels
 Watch for somogyi at 3am and again in the
phenomenon (rebound effect): morning can help
o A low blood glucose in the distinguish between the
late evening causes a somogyi and dawn
rebound effect in the body phenomenon. Blood sugar
leading to hyperglycemia in that is low at 3am indicates
the early morning. somogyi effect while high
o This occur when the or normal blood sugar at
evening dose of insulin is that time suggests the dawn
too high, causing phenomenon.
hypoglycemia in the early  Diet: follow the American diabetic
morning hours, resulting in association (ADA) diet.
the release of counter-  Education and close follow-up
regulatory hormones every 3 months
(epinephrine and glucagon)
to counteract this insulin- COMPLICATIONS
induced hypoglycemia.  Short term: DKA, Hypoglycemia
o The patient then has high  Long term:
blood glucose and ketones  Microvascular complications:
in the morning. diabetic retinopathy,
o The treatment is to actually nephropathy and neuropathy
lower the bedtime insulin  Macrovascular complications:
dose and not to raise it. usually seen in adulthood and
 Dawn effect: include atherosclerotic disease,
o It is similar to the somogyi hypertension, heart disease and
effect in that people stroke
experience hyperglycemia

124
TYPE 2 DIABETES children with signs of insulin
MELLITUS resistance (Acanthosis nigricans)
 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.
 Etiology:
 Very strong hereditary
component (stronger for type 2
than type 1)
 The cause is likely a
combination of peripheral
tissue resistance to insulin and
progressive decline in insulin
secretion, both of which result
in a hyperglycemic state.
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
because type 2 DM is more of an for those patients who have high
insulin resistance rather than a blood sugar or who fail oral
deficiency. agents.
 Obesity
DIABETIC KETOACIDOSIS
 Acanthosis nigricans (velvety and
 This is hyperglycemia usually
hyperpigmented skin of the neck
greater than 300mg/dl (>16
and axillary folds) is common.
mmol/l) with ketonuria and a
DIAGNOSIS serum bicarbonate level
 Type 2 diabetes should be <15mmol/l or serum pH <7.30.
suspected if there is a family  It may be triggered by infection or
history, and in severely obese poor compliance.

125
PATHOPHYSIOLOGY epinephrine, cortisol and growth
 Insulin deficiency creates a state of hormone) are released and
diminished glucose substrate at the contribute to fat breakdown
cellular level, despite the high (lipolysis).
serum levels of glucose.  Glucagon stimulates conversion of
 The body’s need for substrate to free fatty acids into ketone bodies
make energy therefore results in (acetone, acetoacetate and beta-
gluconeogenesis. hydroxybutyric acid)
 Hyperglycemia resulting from  The counter-regulatory stress
insulin deficiency leads to an hormones in the face of insulin
osmotic diuresis with polyuria and deficiency lead to fat lipolysis and
eventual dehydration. ketone formation and eventually
 Counter-regulatory stress DKA.
hormones (i.e. glucagon,

126
CLINICAL FEATURES  Hyperkalaemia caused by
 Patients with mild DKA may metabolic acidosis (potassium
present with vomiting, thirst, moves out of the cells in the face
polyuria, polydipsia and mild to of acidosis as hydrogen is taken
moderate dehydration. into the cells) or normokalemia
 Patients with severe DKA may MANAGEMENT
present with severe dehydration,
 Fluid and electrolyte therapy
severe abdominal pain that may
 Assess clinically for
mimic appendicitis, rapid and deep
dehydration.
(kussmaul) respirations and coma.
 If in shock initial resuscitation
 In the presence of ketones that is with normal saline.
gives the patient with DKA “fruity Dehydration should be
breath”. Ketones also contribute to corrected gradually over 48-
coma in severe DKA.
72h. Rapid rehydration should
LABORATORY FINDINGS be avoided as it may lead to
 Investigations: cerebral edema.
 Blood glucose  Begin with a 10-20ml/kg of NS
 Serum ketone over 1 hour. Repeat as
 Urinalysis: glucose, ketones necessary up to 80ml/kg (max 4
 Urea and electrolytes, boluses). if still in shock
creatinine (dehydration) contemplate vasopressors after
 Blood gases analysis (Severe a reevaluation to assess the
metabolic acidosis) cause.
 Blood and urine culture (for  Replacement fluids: calculate
evidence of precipitating cause) fluid based on 10%
 Cardiac monitor for T-wave dehydration, not exceeding
changes of hypokalemia 4000ml/m2/day (normal saline)
 Weight  Monitor:
 Anion gap metabolic acidosis o Fluid input and output
 Hyperglycemia (>11.1mmol/L) o Electrolytes, creatinine and
and glucosuria. acid-base status regularly
o Neurological state
 Ketonemia (>3.0 mmol/L) and
 Insert central venous line
ketonuria.
(CVP) and urinary catheter if
shocked. A nasogastric tube is

127
 passed for acute gastric dilation ketoacidosis and able to tolerate
if there is vomiting or oral feeding.
depressed consciousness.  Potassium repletion (40mmol/l of
Gastric contents should be fluid)
emptied as there is gastric  Although the initial potassium
paralysis in DKA. may be high, it will fall
 Do not stop the intravenous following insulin and
insulin infusion until 1h after rehydration.
subcutaneous insulin has been  Potassium replacement must be
given (reestablish oral fluids, instituted as soon as urine is
subcutaneous insulin and diet) passed.
 Regular insulin:  Continuous cardiac monitoring
 Insulin infusion (0.05-0.1 IU/kg and regular plasma potassium
per h) is stated after 1 hour, measurements are indicated
titrating the dose according to until the plasma potassium is
the blood glucose. stable.
 Do not give a bolus  Antibiotics
 Monitor blood glucose  Blood sugar, neurological status,
regularly (1 hourly). Aim for fluid balance should be done
gradual reduction of blood hourly and serum electrolytes,
glucose of about 2mmol/h, as a acid-base status should be done 2
rapid reduction is dangerous. hourly.
 Change to 5-10%
dextrose/0.18% saline (half
normal saline) after 24h when
the blood glucose has fallen to
15mmol/L to avoid
hypoglycemia or when it is
greater than 5 mmol/L after
insulin infusion.
 Treatment can be switched to
subcutaneous insulin once the
patient is fully conscious, out of

128
129
130
COMPLICATIONS OF DKA  Hypocalcemia due to either
 Cerebral edema excessive use of potassium
 Usually occurs 6-12 hours into phosphate or osmotic losses.
therapy and rarely after 24
hours.
 Risk factors include younger
than 5 years of age, initial
drops in serum glucose levels
faster than 100mg/dl per hour
and fluid administration greater
than 4L/m2 per 24hours.
 Mortality as high as 70%
 Aspiration
 Severe hypokalemia, Arrhythmias

131
 CHAPTER 12: GENITOURINARY TRACT  Nephrotic syndrome from other
primary glomerular disease
including:
GENITOURINARY o IgA nephropathy,
TRACT o Membranoproleferative
glomerulonephritic and
NEPHROTIC o PSGN
SYNDROME  Nephrotic syndrome that results
 This is a condition characterized from systemic disease:
by heavy proteinuria (> o Systemic lupus
50mg/kg/24 hours), erythematous
hypoalbuminemia, o HSP
hypercholesterolemia and edema.
PATHOPHYSIOLOGY
 2/3 of cases present before 5 years
of age.  The basic physiologic defect is a
loss of the normal charge and size
 In young children the ratio of boys
selective glomerular barrier to the
to girls is 2:1.
filtration of plasma proteins.
 By late adolescence, both sexes are
 Excessive urinary protein losses
equally affected.
lead to hypoproteinemia of
CLASSIFICATION nephrotic syndrome.
 There are 3 categories of nephrotic  Due to hypoproteinemia,
syndrome: hypercholesterolemia results
 Primary nephrotic syndrome  Reduced plasma oncotic
o These are not a pressure induces increased
consequence of systemic hepatic production of plasma
disease. proteins, including lipoproteins.
o Account for 90% of all  Plasma lipid clearance is
childhood cases of reduced because of reduced
nephrotic syndrome. activity of lipoprotein lipase in
o The most common cause is adipose tissue.
minimal change disease
CLINICAL FEATURES
(MCD)-90% in young
children and 50% in older  Edema: ranges from mild
children and adolescents periorbital edema (particularly on
waking) to scrotal or labial edema

132
 to widespread edema (leg, ankle resulting from
edema and ascites). hypoproteinemia)
 The edema often follows an upper o Thrombocytosis
respiratory infection (URI). Pleural  Urea and Electrolytes,
effusions (causing breathlessness) Creatinine and lipid studies:
and hypotension may also occur. o Metabolic acidosis which
 Patients may rarely be may be caused by renal
asymptomatic at time of diagnosis. tubular acidosis
 Patients are predisposed to o Hypoalbuminemia and
thrombosis secondary to elevated serum cholesterol
hypercoagulability (loss of anti- o BUN and creatinine should
thrombin III). Patients may present be measure to assess for
with stroke or other thrombotic renal impairment
events such as renal vein  C3, ANA and antistreptococcal
thrombosis and sagittal sinus antibodies are indicated to rule
thrombosis. out causes other than MCD.
 Patients are also at an increased  Renal ultrasound: may show
risk for infection with enlarged kidneys
encapsulated organisms, such as S.  Renal biopsy: rarely indicated
pneumoniae and therefore may in children with typical
present with spontaneous bacterial nephrotic syndrome unless the
peritonitis, pneumonia or creatinine clearance is impaired
overwhelming sepsis. or initial management with
corticosteroids is ineffective.
DIAGNOSIS
 Based on clinical features and the MANAGEMENT
following studies:  Treatment is dictated by the
 Urinalysis underlying cause.
o 3+ to 4+ proteins  Supportive therapy is universally
o Microscopic hematuria indicated.
o Elevated TP/CR  Hospitalization
o Presence of RBC casts  IV infusion of 25% albumin in
indicate a cause other than children with widespread
MCD edema, scrotal or labial edema,
 Full blood count may show hypotension or symptomatic
o Elevated hematocrit (due to pleural effusions.
hemoconcentration  Salt restriction

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 Most patients with MCD respond
to therapy with corticosteroids:
steroid-dependent or steroid
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’
o Caused by measles virus
 Scarlet fever/ ‘Second disease’
o Caused by Streptococcus
CHAPTER 13: DERMATOLOGY
pyogenes

135
  Rubella/German measles/ ‘third sense enveloped virus of the genus
disease’ Morbillivirus within the family
o Caused by rubella virus paramyxoviridae).
 Erythema infectiosum/’Fifth  The virus is highly contagious and
disease’ is transmitted through droplets
o Caused by Parvovirus B19 from the secretions of the nose and
 Roseola infantum: caused by throat.
HHV-6 and HHV-7  The virus can live up to 2 hours in
 Note “fourth disease” a condition the airspace or nearby surface.
whose existence is not widely  Humans are the only known hosts
accepted today was described in of the virus.
1900 and is postulated to be  Risk factors for infection:
related to staphylococcus aureus.  Immunodeficiency (HIV/AIDs)
 Many other common viruses apart  Immunosuppression after organ
from the above mentioned can also transplant
present with an exanthema as part  Drugs: Alkylating agents/
of their presentation although not corticosteroid therapy
classically considered as part of  Travel to areas with measles
the classic numbered list, these  Loss of passive inherited
include: antibodies before age of routine
 Varicella zoster (chicken pox or immunization
shingles)
 Mumps PATHOPHYSIOLOGY
 Rhinovirus (common cold)  Once the measles virus gets onto
 Unilateral laterothoracic the mucosa it infects the epithelial
exanthema of childhood cells of trachea/bronchi.
 Some types of hemorrhagic  It attaches to its target receptor
fevers with the surface protein
 Tick borne diseases e.g. Rocky hemagglutinin (H protein).
Mountain spotted fever  Once bound the fusion/ F protein
helps the virus ultimately get
MEASLES inside the cell.
 Measles/morbilli/ rubeola/ red  The virus is a single stranded RNA
measles/ English measles is a virus and is negative sense so it
highly contagious infectious has to be transcribed by RNA
disease caused by the measles polymerase into a positive sense
virus (a single stranded negative mRNA strand.

136
 After that it is ready to be enanthem (rash on mucous
translated into viral proteins, membranes) and a characteristic
wrapped in the cell’s lipid exanthema (rash on the skin)
envelope and sent out of the cell as  Initial symptoms include:
newly made virus.  Fever (>40 degress)
 Primary viremia occurs resulting  Cough: dry hacking
in infection of the  Runny nose
reticuloendothelial system  Inflamed eyes
 Within days the measles virus  The classic symptoms include a 4-
spreads through local tissue and day fever (the 4D’s) and the 3 C’s:
is picked up by dendritic cells  Cough
and alveolar macrophages that  Coryza (head cold, fever, and
carry it from the local tissue in sneezing)
the lungs through blood to the  Conjunctivitis (red eyes)
local lymph nodes.  Small white spots known ‘Koplip’
 Secondary viremia results in spots may form inside the mouth 2
systemic symptoms or 3 days after the start of
 From the local lymph nodes it symptoms.
continues to spread and reaches  They are commonly seen inside
more lung tissue as well as of the cheeks opposite the
organs like the intestine and the lower second molars.
brain.  They are diagnostic of measles.
 The incubation period is about 10  The rash usually starts on the face
days. and then spreads to the rest of the
 The disease is most common in body typically begins 3 to 5 days
preschool children, infants are after the start of symptoms.
protected by transplacental  The rash coincides with the rise in
antibodies which generally decay fever.
by 9 months (hence the rationale  The rash is described as
for vaccination at this age). generalized red maculopapular
rash.
CLINICAL FEATURES
 It starts on the back of the ears and
 Symptoms usually develop 10-12
after a few hours, spreads to the
days post exposure and last 7-10
head and neck before spreading to
days.
cover most of the body.
 Clinical features include a classic
prodrome, followed by a transient

137
 It covers the lower extremities by absent and the rash rapidly
the second day, becomes confluent becomes petechial.
by the third day and lasts 4-7 days.  Drug rashes have history of
 The rash is said to “stain” antecedent drug intake.
changing color from red to dark  In Kawasaki disease, glossitis,
brown before disappearing. cervical adenopathy, fissuring
of lips, extreme irritability,
DIAGNOSIS edema of hands and scaling are
 The basis of diagnosis is clinical distinguishing clinical features.
features (the 3C’s, koplik’s spots).
 Confirmation of measles infection MANAGEMENT
is by serologic testing:  Supportive care is most important.
 Measles IgM antibodies  Antipyretics
(present 3 days after rash and  Maintenance of hygiene
persist 1 month) or  Ensuring adequate fluid
 Isolation of Measles virus RNA  Caloric intake and
from respiratory specimens humidification
 If blood can’t be drawn salivary  Vitamin A has shown to improve
IgA specific to measles can be outcome.
tested.  Immunoglobulin can be used for
 Measles need to be differentiated postexposure prophylaxis in high-
from other childhood risk individuals (e.g. children with
exanthematous illnesses. HIV and other immunodeficiency
 The rash is milder and fever states) who are exposed to
less prominent in rubella, measles.
enteroviral and adenoviral  Complications should be managed
infections appropriately.
 In roseola infantum, the rash  Measles is a preventable and
appears once fever disappears potentially eradicable disease
while in measles the fever through universal immunization
increases with rash. (MMR vaccine)
 In rickettsial infection, the face
is spared which is always
COMPLICATIONS
involved in measles.  Complications are more frequent
 In meningococcemia, the upper in the very young, malnourished
respiratory symptoms are and the immunocompromised.

138
 Bacterial pneumonia is the most SCARLET FEVER
common complication and the  Scarlet fever is a toxin-mediated
most common cause of mortality. bacterial illness that results in a
 Usual bacterial pathogens: characteristic skin rash.
pneumococcus, Staphylococcus  The cause of infection is strains of
aureus and sometimes gram- group A beta hemolytic
negative bacteria streptococcus (GABHS) that
 Otitis media is also common produce erythrogenic toxin.
 Laryngotracheitis.  The peak incidence is in winter
 Giant cell pneumonia and spring.
 Bronchiectasis  Transmission is by large
 Flaring up of latent M. respiratory droplets or by infected
tuberculosis infection nasal secretions.
 Encephalomyelitis (inflammation
of both brain and spinal cord)
CLINICAL FEATURES
 The exanthema may develop
 Subacute sclerosing
during any GABHS infection (e.g.
panencephalitis is a rare late
impetigo, cellulitis, pharyngitis).
complication.
 Before or during the exanthema,
 GIT complications:
fever, chills, malaise and often an
 Diarrhea
exudative pharyngitis may occur.
 Appendicitis
 Hepatitis  The exanthema has the following
 Ileocolitis characteristic features:
 Precipitated malnutrition and  Begins on the trunk and mover
cause noma or gangrene of the peripherally
cheeks  Skin is erythematous with tiny
skin-colored papules
(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.

139
DIAGNOSIS  Rheumatic fever
 The basis of diagnosis is clinical  Post-streptococcal arthritis:
features and a positive throat characterized by joint symptoms
culture for S. pyogenes (gold (without other features of
standard) or positive rapid rheumatic fever) that may last for
streptococcal tests that detect weeks. Antibiotic therapy does not
GABHS antigen. prevent this complication.
 Pediatric autoimmune
MANAGEMENT neuropsychiatric disorders
 The goal is to prevent development associated with streptococcal
of rheumatic fever. infection is a phenomenon in
 Appropriate antibiotics include which patients develop the acute
oral penicillin VK, intramuscular onset obsessive compulsive
benzathine penicillin or for CHAPTERsymptoms
14: INFECTIOUS DISEASES
or a tic disorder after
penicillin-allergic patients streptococcal infection. Antibiotic
erythromycin or macrolides. therapy prevents this complication.
COMPLICATIONS OF GABHS
INFECTION
RUBELLA
 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.

INFECTIOUS DISEASES
 It was previously referred to as
“sepsis neonatorum”
 Neonatal sepsis also denotes
NEONATAL SEPSIS systemic bacterial infections
 Neonatal sepsis is a type of incorporating septicemia,
neonatal infection and specifically pneumonia and meningitis.
refers to the presence of a bacterial  Septicemia: is a condition in
blood stream infection in a which bacteria/toxins are
newborn baby.

140
 present in blood, multiplying  Early onset sepsis manifests as
rapidly and destroying tissues. pneumonia and less commonly
 Sepsis: Presence of bacteria and as septicemia or meningitis.
their toxins in the body which  Late-onset sepsis presents after 7
can kill tissue and produce pus days (or 72 hours, depending on
usually following the infection the system used).
of a wound.  The infections are caused by
 The implicated organisms include: organisms thriving in the
 Group B streptococci external environment of the
 Escherichia coli home or the hospital.
 Listeria monocytogenes  Infection is often transmitted
 Klebsiella sp through the hands of the care-
 Coagulase negative providers.
staphylococci  Presentation is that of
 Acinetobacter septicemia, pneumonia or
 Pseudomonas meningitis.
 Neonatal sepsis is divided into 2  Predisposing factors include:
categories: Early-onset sepsis and low birth weight, lack of
Late-onset sepsis. breastfeeding, poor cord care,
 Early onset sepsis presents in the superficial infections
first 7 days of life (although some (pyoderma, umbilical sepsis),
literature refer to it as within 72 aspiration of feeds and
hours) disruption of skin integrity with
 Infections are caused by needle pricks and use of IV
organisms prevalent in the fluids.
maternal genital tract or in the  Neonatal sepsis is the single most
delivery area important cause of death in
 Predisposing factors include: hospital as well as community in
low birth weight, prolonged developing countries.
rupture of membranes (>18
CLINICAL FEATURES
hours), foul smelling liquor,
multiple per vaginal  Signs and symptoms are non-
examinations, maternal fever, specific and require high index of
difficult or prolonged labor and suspicion for early diagnosis.
aspiration of meconium.  An early but non-specific
manifestation is alteration in the
established feeding behavior.

141
  The baby who had been active congestive heart failure and even
and sucking normally refuses to simple conditions like nasal block
suck and becomes lethargic, or may mimic sepsis.
unresponsive.  A careful clinical examination and
 Poor cry, hypothermia, relevant investigations are
hypoglycemia, abdominal necessary to differentiate these
distension, vomiting, apneic spells, conditions from neonatal sepsis.
swollen body and jaundice are  The current practice in newborns
other common manifestations. less than 30 days is to perform a
 Diarrhea is uncommon. complete workup including full
 A heart rate above 160 can also be blood count with differential,
an indicator of sepsis, this blood & CSF culture, urinalysis
tachycardia can present up to 24 and CSF studies, admit the
hours before the onset of other newborn to the hospital and treat
signs. empirically for serious bacterial
 Fast breathing, chest retractions infection for at least 48 hours until
and grunt indicate pneumonia. culture demonstrates no growth.
 Most cases of meningitis do not INVESTIGATIONS
have any distinct clinical picture
 No investigation is required to start
per se, making it mandatory to
treatment in a sick baby who has
suspect meningitis in all cases
high probability of sepsis.
suspected of sepsis.
 Although culturing for
 The presence of excessive or
microorganisms from a sample of
high pitched crying, fever,
CSF, blood or urine may yield
seizures, blank look, neck
false negatives due to the low
retraction or bulging anterior
sensitivity of the culture methods
fontanel are suggestive of
and concomitant treatment with
meningitis.
antibiotic it still remains the gold
 Shock, bleeding, and renal failure
standard in the definitive diagnosis
are indicators of overwhelming
of neonatal sepsis.
sepsis.
 A sepsis screen should be
DIAGNOSIS performed in equivocal
 Diagnosis is difficult as a host of (confusing) cases.
conditions such as hypothermia,  The screen consists of:
hyperthermia, hypoglycemia,  Leukocyte count (TLC
hypoxia, late metabolic acidosis, <5000/mm3)

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  Absolute neutrophil count TREATMENT AND
(ANC <1800/ mm3) PREVENTION
o Differential leukocyte  Note that diagnosis of neonatal
count showing increased sepsis is difficult and so if there is
number of polymorphs any remote suspicion of sepsis
o Differential leukocyte treat with antibiotics empirically
count (band cells> 20%) until cultures are sufficiently prove
 Immature to total neutrophil to be negative.
ratio (I/T ratio, more than 20%)
 Good supportive care should be
 Increased haptoglobin provided to the infant:
 Gastric aspirate showing >5  Provide warmth, ensure normal
polymorphs per high power temperature (36.5 to 37.5
field degrees)
 Newborn CSF screen showing  Start oxygen by hood or mask,
increased cells and proteins if the baby is cyanosed or
 CRP (more than 1mg/dl) grunting. Provide bag and mask
 Micro ESR (15mm or more in
ventilation if breathing is
the first hour) inadequate. Instilling normal
 Suggestive history of saline drops in nostrils may
chorioamnionitis, PROM help clear the nasal block.
(premature rupture of  Assess peripheral perfusion by
membrane) etc. palpating peripheral pulses,
 Sepsis screen is considered capillary refill time (normally
positive if 2 of these parameters <2-3 seconds) and skin color.
are positive.  Infuse normal saline or Ringer
 Value of sepsis screen is more for lactate 10ml/kg over 5-10
exclusion of diagnosis of neonatal minutes, if perfusion is poor.
sepsis. Repeat the same 1-2 times over
 Lumbar puncture should be the next 30-45 minutes, if
performed in all cases suspected of perfusion continues to be poor.
neonatal sepsis except in Dopamine and dobutamine may
asymptomatic babies being be required to maintain normal
investigated for maternal risk perfusion.
factors.  Insert IV line. If hypoglycemia
is suspected infuse glucose

143
 (10%) 2ml/kg stat. Do not use perforation is a concern)
glucose boluses routinely. Clindamycin is often added.
 Provide a maintenance fluid  Antimicrobial therapy can be made
and electrolytes and glucose (4- specific once a positive culture and
6 mg/kg/min) add potassium to sensitivity report is available.
IV fluids once normal flow of  Intensive care and monitoring is a
urine has been documented. key determinant of improved
 In addition to fluid resuscitation survival of neonates.
and supportive care a common  Outcome depends upon weight and
antibiotic regimen in infants maturity of the infant, type of
suspected with sepsis is beta- etiologic agent, its antibiotic
lactam antibiotic (usually sensitivity pattern and adequacy of
ampicillin) in combination with an specific and supportive therapy.
aminoglycoside (usually  Early onset sepsis carries a
gentamicin) or a third generation higher risk of adverse
cephalosporin (usually outcomes.
cefotaxime… Ceftriaxone is
generally avoided in neonates due DIFFERENTIAL DIAGNOSIS
to the theoretical risk of  Meconium aspiration syndrome
kernicterus.)  Necrotizing enterocolitis
 Organisms targeted are those  Pericarditis (bacterial)
found in the female GUT to which  Pulmonary hypoplasia
neonates are especially vulnerable  Respiratory distress syndrome
especially Group B streptococcus,  Bowel obstruction in the newborn
Escherichia coli and Listeria  Congenital diaphragmatic hernia
monocytogenes (the main rationale  Congenital pneumonia
behind the use of ampicillin versus  Heart failure (congestive)
other beta lactams).  Hemolytic disease of newborn
 Neonates are also vulnerable to
other common pathogens that
cause meningitis and bacteremia TUBERCULOSIS
such as streptococcus pneumoniae
and Neisseria meningitidis.
 Although uncommon, if anaerobic TUBERCULOSIS
species are suspected (such as in  This is a chronic disease caused by
cases where necrotizing Mycobacterium tuberculosis.
enterocolitis or intestinal

144
 Other species of Mycobacterium  In the pediatric group few
include: M. bovis, M. avium infections may also occur by
complex and M. leprae. transplacental route (congenital
 All cases of pulmonary tuberculosis)
tuberculosis and most cases of  Other possible route of infection is
extrapulmonary disease are caused through gastrointestinal system if
by human type strain M. the bovine strain M. bovis is
tuberculosis. ingested.
 A few cases of extrapulmonary  Exposure is the term used to
illness particularly the tubercular describe an individual who has
lymphadenitis may be due to the been in recent contact with an
bovine strain. individual with contagious
 The bacterium has the following pulmonary TB.
characteristics:  Physical examination,
 Pleomorphic tuberculin skin test and chest
 Weakly gram-positive radiograph are all normal.
 Non motile and non-sporing  Latent tuberculosis infection is the
 Resistant to acid discoloration term used to describe an
after staining due to the asymptomatic individual with
presence of mycolic acid in the positive tuberculin skin test,
cell wall. normal physical examination and a
 Slow growing (generation time chest radiograph that either is
is 12-24 hours and culture negative or shows only pulmonary
require at least 3-6 weeks). granulomas or calcifications with
 The infection is spread by the or without regional lymph nodes.
tuberculous patient who discharges  Those at high risk include:
tubercle bacilli in his sputum or immigrants from highly endemic
nasopharyngeal secretion during regions of the world, health care
bouts of coughing or sneezing etc. personnel, homeless individuals,
This is the usual mode of infection. residents of institutions or
 Transmission is usually from the correctional facilities (prison) and
so called “sputum positive” adults, individuals with
children are rarely contagious as immunodeficiency conditions (e.g.
they rarely expectorate infected HIV, chronic disease,
sputum. immunosuppressive medications).

145
PATHOGENESIS  In primary tuberculosis the
 Infection is by respiratory droplets. caseated area may heal completely
 The damage caused to the lungs is and may become calcified.
due to the delayed hypersensitivity  Some of these calcified nodules
reaction (Type IV)-T-cell mediated contain bacteria which are
 Once inhaled into the lung, contained by the immune system
alveolar macrophages ingest the and are capable of lying dormant
bacteria. for years. This initial focus is
 The bacilli then proliferate inside termed the ‘Ghon focus’
the macrophages and cause the  On X-ray the Ghon focus is
release of neutrophil chromo- evident as a small calcified
attractants and cytokines resulting nodule often within the upper
in an inflammatory cell infiltrate parts of the lower lobes or the
reaching the lung and draining lower parts of the upper lobes,
hilar lymph nodes. seen in the mid zone.
 Macrophages present the antigen  When there is associated hilar
to the T lymphocytes with the lymphadenopathy the lesion is
development of a cellular immune termed a ‘Ghon complex’
response. (Primary complex of Ranke)
 A delated hypersensitivity type  In children especially those with
reaction occurs resulting in tissue risks e.g. immunocompromised or
necrosis (caseous necrosis) and other co-morbidity or <2 years
formation of the granuloma. primary infection often progresses
 The granuloma consists of to an active disease.
 A central area of necrotic  If the immune system contains the
material called caseation infection and the patient develops
 Surrounding epithelioid cells cell-mediated immune memory the
 Langhans’ giant cells with to the bacteria this is termed latent
multiple nuclei tuberculosis.
 Lymphocytes  Later in life after initial exposure
 Varying degrees of fibrosis TB, the latent infection can
 Note: epithelioid cells and reactive and cause active disease
Langhans’ giant cells are both cell when immunity decreases.
being derived from the  Sometimes primary infection can
macrophage. end in hematogenous spread
causing miliary tuberculosis.

146
 The TB bacilli seed various organs effusion, consolidation and
through blood borne or lymphatic bronchopneumonia.
spread. If the number of bacilli are  In miliary type of pulmonary TB
more and the host immunity there may be high grade fever,
inadequate disseminated TB toxic look and splenomegaly.
occurs.  The disease is most common in
infants and young children.
CLINICAL FEATURES  The onset of illness is often
 Can manifest as: sudden.
 Pulmonary TB
 Extrapulmonary TB EXTRAPULMONARY
 Mixed TUBERCULOSIS
 In children approximately 25-30%  TB can affect any of the body
cases are of extrapulmonary TB. system and different organs.
 They include:
PULMONARY TUBERCULOSIS
 CNS
 The symptomatology of
o TB-meningitis
pulmonary TB is almost uniform
o TB-encephalitis
across various types of
o TB-spine
involvement in the lungs.
 TB-abdomen (ileitis)
 Children tend to have more non-
 TB of the skin, joints and
specific symptoms:
skeletal disease (Pott’s disease)
 Cough (inconsistent and may
 Disseminated or miliary disease
be absent in advanced disease)
 In children CNS TB is a frequent
 Low grade fever
occurrence.
 Loss of appetite
 Lethargy CENTRAL NERVOUS SYSTEM
 Weight loss DISEASE
 Night sweats
 This is the most serious
 Failure to thrive (commonest
complication of TB in children.
presentations)
 It arises from the formation of a
 The additional clinical signs of the
caseous lesion in the cerebral
disease depend upon the type and
cortex or meninges that results
extent of the involvement.
from occult lymphohematogenous
 There may be no clinical signs or spread.
range from findings of pleural

147
 These lesions can be single or  The diagnosis should be
multiple and enlarge presenting as considered for any patient with
intracranial tumors. basilar meningitis, hydrocephalus
 TB may also be confined to the or cranial nerve involvement that
spinal cord. has no other apparent cause.
 TB meningitis is commonest in  A lumbar puncture is needed to
children between 6 months to 5 demonstrate AFB in CSF.
years of age.  CSF findings are as follows:
 In infants and young children, the  Color: clear or opaque
disease runs a rapid course, it may  Opening pressure: increased
present with:  WBC= 50 – 500 cells/mm3
 Rapid progression to (initially polymorphonuclear
hydrocephalus, bulging anterior neutrophils then lymphocytes
fontanelle predominate)
 Lethargy  Proteins: very high
 Anorexia, headache, vomiting  Glucose: low to very low
 Impaired level of consciousness  Culture: AFB smear and culture
 Seizures rarely positive, PCR may be
 Raised intracranial pressure positive
 Irritability
TUBERCULOUS PERITONITIS
 High pitched cry, drowsiness,
coma  Uncommon in children but can
 In older children signs and occur due to either hematogenous
symptoms progress over the course spread or local extension from
of several weeks beginning with abdominal lymph nodes or
fever headache, irritability and intestines.
drowsiness.  Presents with: low grade fever,
 The disease advances with pain, ascites, weight loss.
symptoms of lethargy, vomiting, TB ABDOMEN
nuchal rigidity, seizures,
hypertonia and focal signs.  There are 2 major clinical
 The final stage of disease is presentations:
marked by coma, hypertension,  Tuberculous peritonitis
decerebrate and decorticate  Tuberculous enteritis
posturing and death.  Tuberculous peritonitis is
uncommon in children but can
occur due to either hematogenous

148
 spread or local extensions from progression to hydronephrosis or
abdominal lymph nodes or urethral strictures may be seen.
intestines.
TB OF BONES/JOINTS
 Low grade fever and pain,
ascites, weight loss are the  Skeletal TB is a late complication.
typical features.  The commonest involvement is
 Tuberculous infection of the vertebra leading to classical Pott’s
intestines occur either secondary to spine with formation of gibbus and
hematogeous spread or from kyphosis.
ingestion of TB bacilli from  Dactylitis of metacarpals is seen in
sputum. Small intestines and children.
appendix are the usual sites of the  Tuberculous arthritis of any of the
involvement. joints can occur.
 Tuberculous ulcers or later
strictures can cause the clinical
features: TB ADENITIS
 Low grade fever, weight loss,
 Cervical lymphadenitis (Scrofula),
diarrhea or constipation or
is the most common form of
features of subacute intestinal
extrapulmonary TB in children.
obstruction can be the
 The nodes draining the lungs fields
presenting features.
are usually involved.
 The common group of nodes
GENITOURINARY TB involved are cervical and axillary
but other groups can also be
 Renal TB has a long incubation involved especially secondary to
period and hence is generally seen drainage form an infected organ.
in older children or adolescents.  The node involvement usually
 Kidneys as well as other parts of occurs 6-9 months after primary
the urinary system can be infection.
involved.  The affected nodes are firm, non-
 Renal involvement is unilateral tender, fixed and often matted due
and initially present as sterile to periadenitis.
pyuria and microscopic hematuria.  There may be accompanying low
 Later abdominal pain and mass, grade fever. In case a node breaks
dysuria and flank hematuria with down it leads to sinus formation.

149
 On biopsy they show caseating  If the infection is through
granuolmas inhalation or the hematogenous
spread occurs further to lungs then
DISSEMINATED/ MILIARY TB
respiratory signs are
 Two forms of disseminated spread predominantly present.
are seen:  The neonate can have acute onset
 Hematogenous spread: there is respiratory distress, fever, poor
usally fever, weight gain, lymphadenopathy and
hepatospenomegaly and hepatosplenomegaly.
lymphadenopathy  Occasionally meningitis can also
 In the other more serious occur.
disseminated form there is large  The clinical signs and symptoms
hematogenous spread and in a can be similar to other infections.
patient with inadequate  A maternal history or contact with
response there is miliary TB. an AFB positive person should
o Miliary TB may start with raise the suspicion.
an insidious fever, malaise
and loss of appetite DIAGNOSIS
o The fever rises and there is  Diagnosis of TB in children is not
lymphadenopathy with simple.
hepatosplenomegaly.  A high index of suspicion in the
endemic area is important.
CONGENITAL TB
 The modalities most frequently
 Perinatal transmission of TB can used for diagnosis are
occur as a hematogenous spread  Mantoux test
through the placenta in mother  Radiological examination
with active disease.  Gastric aspirate/sputum AFB
 Inhalation/ingestion of infected smear and culture
amniotic fluid or exposure after  Polymerase chain reaction
birth to a positive contact can also (PCR)
cause infection in a neonate.  Diagnosis in children is usually
 The transplacental infection based on clinical signs and
presents with a primary complex symptoms, an individual’s risk
like manifestation in the abdomen. factor for infection (chronic
 The liver has the focus with nodes disease, contact), tuberculin skin
in porta hepatis also involved. test findings, chest radiographic
findings and culture.

150
  A contact is defined as any are rinsed out of the stomach
child who lives in a household with saline before food.
with an adult taking ATT or has  Microscopic stain used is
taken such therapy in past 2 modified Ziehl-neelson stain
years. (ZN stain)
 Demonstration of mycobacterium  Urine, lymph node excision
in various clinical specimens (showing caseating granulomas),
remains gold standard. CSF should be performed where
appropriate.
CULTURE, MICRSCOPE AND
 Although it is difficult to culture
SENSITIVITY TB from children the presence of
 Sputum samples are generally multidrug resistance strains makes
unobtainable from children under it important to try to grow the
about 8 years, unless specialist organism so that antibiotic
induction techniques are used. sensitivity can be assessed.
 For example, following an
overnight fast, the patient RADIOLOGICAL
receives salbutamol by  Findings include:
nebulized followed by  Hilar or mediastinal
hypertonic (3% or 5%) lymphadenopathy
inhalation by nebulizer.  Ghon complex- small
 Older children may provide parenchymal infiltrate with
expectoration at end of enlarged hilar lymph nodes
procedure. In young children  Lobar involvement, pleural
including infants a effusion, or cavitary disease
nasopharyngeal aspirate is which typically affects the
collected and processed like upper lung segments.
sputum for smear and culture.  Miliary picture
 Children usually swallow sputum
POLYMERASE CHAIN
so gastric washing (lavage) on 3
consecutive mornings are required
REACTION
to visualize or culture acid-fast  PCR is used to detect DNA from
bacilli (AFM) originating from the clinical samples that are negative
lungs. by microscopy.
 To obtain these, a nasogastric  It has high sensitivity and
tube is passed and secretions specificity when compared with

151
 traditional microbiological  A history of BCG immunization
methods. therefore needs to be taken into
 It is suitable in diagnosis of TB in account when interpreting the test.
children especially when diagnosis  Patients who are
is difficult or needed urgently. immunocompromised,
 The possibility of false positive malnourished or received live
results must be considered vaccines may have false negative
especially when the clinical reactions.
symptoms and history of exposure  The test is positive when:
of the child make the diagnosis  Induration >5mm in children
improbable. with close contact who has
 GenXpert has been developed for clinical or radiological findings
identification of M. tuberculosis in consistent with TB disease or
sputum of adult patients. immunocompromise.
 This method gives results  > 10mm if the child is younger
within 2 hours, in addition it than 4 years, has a chronic
provides results about medical condition, lives in an
sensitivity to rifampicin. area endemic for TB or has not
 The test is under evaluation for received BCG
diagnosis of TB in children.  >15 mm where BCG has been
given, there are no other risk
MANTOUX TEST factors or in children older than
 If TB is suspected a mantoux test 4 years
is performed.  Heaf tests are no longer used for
 2 units of purified protein screening TB.
derivative (PPD) of tuberculin (2  A new generation of diagnostic
TU SSI, 0.1ml intradermal tests is the interferon-gamma
injection read after 48-72 hours as release assays (IGRA)
induration not erythema in mm  These are blood tests that
across the forearm). assess the response of T cells
 Because PPD is a mixture of to stimulation in vitro with a
proteins some of which are small number of antigen found
common to TB and BCG, the test in TB but not in BCG
may be positive because of past  Positive results therefore
vaccination rather than TB indicate TB infection rather
infection. than BCG vaccination.

152
  Sensitivity and specificity of TB) and disseminated TB are
these tests in different settings treated for longer durations of 12
is being evaluated but its months or more.
routine use in clinical practice  Patients with latent tuberculosis
is increasing. are treated with isoniazid for 9
 Co-infection with HIV makes months. Older adolescents,
the diagnosis even more pregnant adolescents, and adults
difficult because with are also given daily pyridoxine
advances immune suppression (Vitamin B6) to prevent
both skin tests and IGRA are neurologic complications of
unreactive. isoniazide treatment. (peripheral
 The IGRA or Mantoux test cannot neuropathy)
distinguish between TB infection  Patients with TB disease are
and TB disease so correlation with treated on the basis of the location
clinical signs and symptoms is of the TB disease and the
required. susceptibility pattern of the
 Various scoring systems have been organism.
developed for diagnosis of TB. In  Treatment includes
these scoring systems more weight  2 months (intensive phase) of
is given to Lab tests i.e. Rifampicin, isoniazide,
demonstration of AFB, tubercules pyrazinamide and ethambutol
on histology, suggestive radiology (now given to children
and tuberculin test >10mm according to newer guidelines
induration. These scoring system despite its side effect of optic
may be used as screening tools but neuritis)
not for starting treatment.  4 months (continuation phase)
of rifampicin and isoniazide
TREATMENT OF
 Close follow-up is needed and
TUBERCULOSIS frequently children are put on
 The principles of therapy in Direct Observed therapy (DOTS)
children with TB are similar to where nursing staff observe the
those of adults. therapy being given 3 times a
 Treatment for a duration of 6 week.
months has become the standard  During the intensive phase the
practice. health worker or trained
 Extrapulmonary TB person watches as the patient
(Osteoarticular TB, neurological

153
 swallows the drugs in his  Drug resistant TB denotes
presence resistance to one of the first line
 During the continuation phase TB drugs (being Rifampicin or
the patient is issued medicines isoniazid)
for one week in a multi-blister  Multidrug resistant (MDR) TB
combi-pack of which the first denotes resistance to at least 2
dose is swallowed by the main first line TB drugs i.e.
patient in the presence of the rifampicin and isoniazide.
health worker or trained  Extreme drug resistant (XDR) TB
person. denotes MDR-TB + resistance to
 The consumption of medicines any fluoroquinolone and at least
in the continuation phase is one of the 3 injectable second-line
also checked by return of the drugs (amikacin, kanamycin or
empty multi-blister combi- capreomycin)
pack when the patient comes  The second line drugs include:
to collect medicine for the next  Oral: fluoroquinolones
week. o Ciprofloxacin
 Side effects of the drugs: o Levofloxacin
 Rifampicin (bactericidal): o Movifloxacin
Discoloration of body fluids o Gatifloxacin
(to orange-pink),  Injectables
hepatotoxicity o Amikacin
 Isoniazid (bactericidal): o Kanamycin
peripheral neuropathy (give o Capreomycin
vitamin B6), hepatotoxicity o Streptomycin
 Streptomycin (bactericidal):
ototoxicity and nephrotoxicity COMPLICATIONS OF
 Pyrizinamide (bacteriostatic): TUBERCULOSIS
hepatotoxicity, hyperuricemia  Pulmonary complications:
(gout)  Pleurisy
 Ethambutol (bacteriostatic):  Pleural effusion
Optic neuritis and color  Empyema
blindness  Pneumothorax
 Drug resistant TB accounts for  Aspergillosis
possibly only 2% of the million  Endobronchitis
cases of TB in the world.  Bronchiectasis
 Laryngitis

154
  Cor pulmonale infective sporozoites migrating to
 Ca bronchus the insect’s salivary glands.
 Miliary TB  The sporozoites are inoculated
into a new human host and those
which are not destroyed by the
MALARIA immune response are rapidly
 Human malaria is usually caused taken up by the liver.
by one of four species of the  In the liver they multiply inside
genus Plasmodium: P. hepatocytes as merozoites; this is
falciparum, P. vivax, P. ovale, P. pre-erythrocytic (or hepatic)
malariae, sporogeny.
 P. knowlesi is another species  After a few days, the infected
seen to cause malaria especially in hepatocytes rupture, releasing
primates. merozoites into the blood from
 Malaria is transmitted by the bite where they are rapidly taken up
of female anopheles mosquitoes. by erythrocytes.
 It also be transmitted in  In the case of P. vivax and
contaminated blood transfusions, P. ovale, a few parasites
or transplacentally from mother to remain dormant in the liver
the fetus (congenital malaria). as hypnozoites. These may
 P. falciparum and P. vivax are reactivate at any time
more commonly seen in sub- subsequently causing
Saharan Africa. relapsing infection.
 P. ovale is rare and seen  Inside the red cells, some
mainly in Africa. parasites again multiply changing
 P. malariae is the rarest. from merozoite to early and late
trophozoite to schizont and finally
LIFE CYCLE appearing as 8-24 new
 The female mosquito becomes merozoites.
infected after taking a blood meal  The erythrocyte ruptures releasing
containing gametocytes, the the merozoites to infect further
sexual form of the malarial cells.
parasite.  Each cycle takes about
 The developmental cycle in the 48hours in P. falciparum, P.
mid-gut of the mosquito usually vivax and P. ovale and about
takes 7-20 days (Depending on 72hours in P. malariae.
temperature), culminating in

155
  P. vivax and P. ovale mainly released from the red cells until
attack reticulocytes and taken up by a feeding mosquito to
young erythrocytes, while P. complete the life cycle.
malariae tends to attack  In the midgut of the mosquito the
older cells. gametocytes fuse to form a zygote
 P. falciparum will parasitize which develops into an ookinete
any stage of erythrocyte. and then an oocyst which ruptures
 A few merozoites do not develop to give sporozoites which migrate
into trophozoites but into to the salivary glands awaiting to
gametocytes (microgametes and be injected into a person in which
macrogametes). These are not it bites for a blood meal.

156
 157

CLINICAL FEATURES  The gastrointestinal and

 The onset of the disease is marked respiratory symptoms are seen
by a sudden rise of fever which more often in young children and
may be periodic. infants.
 Erythrocyte ruptures releasing  After a few days of fever the
the merozoites to infect patient begins to appear pale and
further cells. may even have jaundice.
 Each cycle takes about 48  In adults there is a definite
hours in P. falciparum, P. periodicity of fever, which is
vivax and P. ovale and about generally not seen in children.
72 hours in P. malariae.  The clinical presentation in some
 P. vivax and P. ovale mainly children may be different with
attack reticulocytes and low-grade fever,
young erythrocytes, while P. hepatosplenomegaly, anemia and
malariae tends to attack thrombocytopenia.
older cells.  Congenital malaria is considered
 P. falciparum will parasitize in a neonate whose mother was
any stage of erythrocyte. symptomatic during later
 The initial findings include vague pregnancy. The neonate may or
flu-like symptoms that typically may not have any fever along
include headache, malaise, with poor, feeding, lethargy and
anorexia. vomiting.
 Cyclic fevers follow the flu-like  Unexplained anemia and severe
prodrome and occur 48-72 hours jaundice (indirect hyper-
in correlation with RBC rupture bilirubinaemia) are additional
and parasitemia. features.
 Rigors, chills, sweating,
headache, abdominal pain, nausea
and vomiting may also FEATURES OF SEVERE
accompany the fever. MALARIA
 Other features include hemolytic (1) Cerebral malaria
anemia, splenomegaly, jaundice  Multiple convulsions- more
and hypoglycemia. Cerebral than 2 episodes in 24 hours.
malaria, renal failure, shock and  Impaired consciousness or
respiratory failure all may occur. unrousable coma
(2) Acute pulmonary edema and acute sensitivity and specificity are a
respiratory distress problem.
(3) Shock (‘Algid malaria’)  RDT is an
 Algid malaria is a severe immunochromatographic test
infection with P. falciparum. that detects specific parasite
There is hypotension, shock, antigens in blood mainly:
shallow respiration and pallor a) Histidine Rich protein 2 (HRP
with a rapid fatality. Gram 2)
negative sepsis is often b) Plasmodium lactate
associated. dehydrogenase (pLDH)
 Circulatory collapse or shock,  Some RDT detect only one
systolic blood pressure species (P. falciparum) while
<70mmHg in adults and other detect multiple (P.
<50mmHg in children vivax, P. malariae and P.
(4) Severe anemia <4g/dl ovale) that is why it is
(5) Hyperpyrexia possible to be RDT negative
(6) Hypoglycemia- <2mmol/L, and Malarial parasite slide
Failure to feed (MPS) positive.
(7) Metabolic acidosis- blood pH<  Peripheral blood smear (Giemsa
7.25, bicarbonate <15mEq/L, Deep Stain): thin and thick smear.
breathing, respiratory distress  Thick smear: diagnosis can be
(acidotic breathing) made quickly, used for
(8) Acute renal failure, blackwater screening.
fever  Thin smear: identifies the
 Black water fever is a severe species and staging
form of falciparum malaria (determination of the level of
with hemolysis, parasitemia).
hemoglobinuria and severe  PCR
anemia.
(9) Spontaneous bleeding and DIFFERENTIAL DIAGNOSIS
coagulopathy  Common clinical differentials of
(10) Hyperparasitemia malarial include:
 Typhoid fever
DIAGNOSIS  Leptospirsosis
 Rapid diagnostic test: tests  Dengue
malarial antigens although  Viral hepatitis

158
 Cerebral malaria should be  Respiratory:
differentiated from other causes  Acute respiratory distress
of acute febrile encephalopathy syndrome and respiratory
like meningitis and encephalitis. failure
Patients with algid malaria (those  Pulmonary edema
in shock) mimic  Gastrointestinal:
meningococcemia and gram-  Jaundice
negative shock  Splenomegaly (Tropical
splenomegaly syndrome) and
COMPLICATIONS
Splenic rupture (which can
 Cerebral malaria: also lead to shock)
 Pathogenesis can be explained  Hepatomegaly
by cytoadherence. P.  Ulceration and ischemia of
falciparum infected RBCs GIT (due to occlusion of
with matures stages of the blood vessels)
parasite bear Histidine rich
 Genitourinary
protein II which adheres to
 Renal failure: due to
Intracellular adhesion
hemoglobinuria and tubular
molecule 1 (ICAM-1)
damage.
 Note: cytoadherence also
 Black water fever:
causes stickiness of RBCS,
hemoglobinuria
“sludging”, “stasis” of blood,
 Uremia
and “blockage” cause lesions,
 Nephrotic syndrome
local anoxia, ischemia thus
(antibody-antigen complexes
pain e.g. abdominal pain with
cause lesions)
occlusion of the small sized
 Metabolic:
vasculature.
 Hypoglycemia (<2mmol/l in
 Cardiovascular and hematology:
children)
 Severe anemia: hemolytic,
 Metabolic acidosis (blood
normochromic normocytic
pH< 7.25)
 High output cardiac failure
secondary to anemia TREATMENT
 Disseminated intravascular  Choice of antimalarial therapy is
coagulation (DIC) based on resistance patterns,
 Thrombocytopenia with species type and severity of
bleeding tendencies illness.
 Shock

159
UNCOMPLICATED MALARIA  Clear grass and water paddle
 Treat with a first-line anti- around the house
malarial agent, as in the national  Spray areas around the house
guidelines. and the house
 Uncomplicated P. falciparum  Chemoprophylaxis
malaria:
 Treat for 3 days with the
recommended artemisin-
based combination therapy
 Artemether-lumefantrine HUMAN
(Coartem) (20mg artemether and
120mg lumefantrine) IMMUNODEFICIENCY
 Child weighing 5-<15kg: one VIRUS
tablet twice a day for 3 days  HIV is an Enveloped Positive
 Child weighing 15-24 kg: 2 sense RNA virus of genus
tablets twice a day for 3 days lentivirus belonging to the family
 Child >25kg: 3 tablets twice a Retroviridae.
day for 3 days  There are 2 types of HIV
 HIV type 1: cause of pediatric
COMPLICATED MALARIA
acquired immunodeficiency
 Artesunate 2.4 mg/kg IV or IM on
syndrome (AIDS)
admission 0h, then at 12 h and
 HIV type 2: rare cause of
24h, then daily until the child can
infection in children
take oral medication (2mg/kg) but
 More than 1 million children have
for a minimum of 24h even if the
AIDs and as many as 10 times
child can tolerate oral medication
this number are infected with HIV
earlier.
worldwide.
PREVENTION  Structure:
 Avoid mosquito bites by:  Enveloped with projecting
 Application of repellants knob like spikes on the
 Sleeping under treat mosquito surface (gp 120) and the
nets anchoring transmembrane (gp
 Wearing protective clothing 41)
 Control of the Anopheles  Genome consists of 2
mosquito especial in endemic identical copies of the
areas: positive sense single stranded

160
 RNA genome (retroviruses mandatory blood product
are diploid) screening
 Enzymes: coded for by pol c) Sharing of intravenous and
gene tattoo needles
a) Reverse Transcriptase  Factors that increase the risk of
b) Integrase transmission:
c) Protease  High maternal viral load
d) Endonuclease  Advanced maternal HIV
disease
 Primary maternal HIV
infection
 Cocomitant maternal genital
infection including
chorioamionitis
 Premature birth
 Prolonged rupture of
 Transmission:
membranes
 Perinatal transmission
 Factors that decrease risk of
(mother-to-child
transmission:
transmission- MTCT)
 Undetectable maternal viral
currently accounts for >95%
load
of pediatric HIV cases.
 Cesarean section
 In utero, intrapartum or
 Adherence to maternal ARV
postpartum (through
therapy and infant post-
breastfeeding) transmission of
exposure prophylaxis.
HIV from an infected mother
to her infant may occur. PATHOGENESIS
Transmission rates range from  The first cells to be infected
50% because of the common through mucosal entry of HIV are
use of antiretroviral therapy the dendritic cells.
during pregnant.  These cells transport the virus to
 Other modes of transmission: the lymphatic tissue where it
a) Sexual contact: important mode selectively invades the CD4
of infection in adolescents lymphocytes, monocytes and
b) Blood product transmission macrophages.
which is now rare because of  HIV attaches via gp120 to the
CD4 surface receptor and

161
 undergoes a conformational important role in sustaining viral
change and expresses 2 more co- load during this phase.
receptors:  There is also B-cell activation
 On macrophages, monocytes, leading to an increase antibody
microglial, dendritic cells and production and resultant
Langerhan’s cells Chemokine hypergammaglobulinaemia.
co-receptor 5 (CCR5) is
expressed. CLINICAL FEATURES
 T-helper cells express CXCR-  Most infants with perinatally
4 acquired HIV infection are
 Once gp120 and co-receptors asymptomatic for the first year of
bind, gp 41 causes fusion of the life.
HIV with the host (gp 41 is a  Some HIV infected infants
fusion molecule) progress rapidly to symptomatic
 After infecting CD4 cells there is disease and onset of AIDS in the
progressive viral replication first year of life.
followed by a viremia phase 3-6  Clinical presentation varies with
weeks after infection. the degree of immunosuppression.
 RNA is reverse transcribed  Mild suppression:
into DNA (in the presence of lymphadenopathy or parotitis
reverse transcriptase) that is  Moderate: recurrent bacterial
incorporated into the host infections, candidiasis,
genome (in the presence of chronic diarrhea and
integrase) lymphocytic interstitial
 HIV then produces its own pneumonitis (LIP)- caused by
viral proteins that are a response to HIV infection or
assembled (in the presence of related to EBV infection.
protease).  Severe AIDS diagnoses
 Subsequently there is a decline in include opportunistic
the viremia due to the normal infections e.g. pneumocystis
immune response of the body. jiroveci (carinii) pneumonia
 CD8 cells are helpful in PCP, severe failure to thrive,
containing the initial infection. encephalopathy and
malignancy, although this is
 A variable period of clinical
rare in children.
latency follows but during this
phase viral multiplication  Early symptoms of HIV infection
continues. The cytokines play an  Failure to thrive

162
  Thrombocytopenia infected mothers will have
 Reccurrent infections such as transplacental maternal IgG
otitis media, pneumonia and HIV antibodies and at this
sinusitis age, a positive test confirms
 Lymphadenopathy HIV exposure but not HIV
 Parotitis infection.
 Recurrent, difficult-to-treat  Children born to HIV mothers
thrush have maternal IgG HIV
 Loss of developmental antibodies which may persist
milestones for as long as 18-24 months
 Severe varicella infection or  The most sensitive test in
zoster diagnosing HIV before 18 months
 More than one clinical feature is of age is HIV DNA PCR.
often present.  All infants born to HIV
 An unusual constellation of infected mothers should be
symptoms, especially if infectious tested for HIV infection,
should, alert one of HIV infection. whether or not they are
 NOTE: children with persistent symptomatic.
lymphadenopathy,  HIV specific DNA PCR is
hepatosplenomegaly, recurrent performed at birth and
fever, parotid swelling, monthly until 4 months of age
thrombocytopenia, or any to detect infants who are
suggestion of SPUR (Serious, infected perinatally.
Persistent, Unusual, Recurrent)  2 negative HIV DNA PCRs
infections should be tested for within the first 3 months of
HIV. life at least 2 weeks after
completion of postnatal
HIV WHO PEDIATRIC antiretroviral therapy indicate
STAGING the infant is not infected
although this is confirmed by
loss of maternal HIV
DIAGNOSIS
antibodies from the infant’s
 In children over 18 months old,
circulation after 18 months of
HIV infection is diagnosed by
age.
detecting antibodies to the virus.
 Negative HIV specific DNA
 Children less than 18 months
PCR at 4 months is consistent
of age who are born to
with an infant who has not

163
 been infected. If the DNA  Pre-labor caesarean section if the
PCR is negative for HIV, mother’s viral load is detectable
infants are followed until they close to the time of delivery.
lose their transplacentally
acquired maternal antibody COMPLICATIONS
(by age 18-24 months).

MANAGEMENT

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.

164
 CHAPTER 15: NEONATOLOGY

NEONATOLOGY
 Look at all flow charts in notes
(Feeds, antibiotics, IV fluids etc.)
NICU ORIENTATION
 Record last weight, noting change
in grams from previous weight.
NICU ORIENTATION  Record vital signs: Temperature
HOW TO range (axillary), heart rate,
CONDUCT/PREPARE FOR A Capillary refill time.
WARD ROUND  Medications: please list names,
 Remove all jewelry and white lab number of days for antibiotics,
coats. frequency and doses.
 Begin each day with a “3 minute”  Lab: record all results
hand to elbow wash. (electrolytes, bilirubin, FBC etc.)
 Look at previous notes in  Follow up all cultures, X-rays,
patient’s files. U/S, CT.

165
 Examine baby- Wash your HOW TO PRESENT BABIES
hands before and after touching DURING MAJOR ROUND
a baby.  Sample problem list:
FLUID REQUIREMENTS  Day 4
 Water is the primary need in the a) RDS
first 24-48 hours due to b) Suspected sepsis,
physiologic diuresis. investigations- FBC was
normal, culture status- blood,
 Start with 10% dextrose.
CSF chemistry, Gram stain
Birth Initial total fluid Insensible c) Anemia-hematocrit
weight intake water loses d) Hyperbilirubinemia, state
(g) cause: ABO, Rh & Coombs
Term 50-60ml/kg/day ~26ml/kg/day status, Rx: phototherapy
~1500 60-70 ~46  Current plan
~1000 70-80 ~56
 Mention physical findings if
~500 80-100 ~82
they are pertinent or show a
change from the norm.
 General goals:  Do not repeat non-active
 Avoid oliguria problems, keep your
<0.5ml/kg/day. presentation concise.
 Keep daily urine output  After completing your
≥1ml/kg/h. presentation, give a brief
 Keep Na at 140-150mmol/L.
+
summary of your plans for the
 Keep K at 4-5mmol/L.
+
day, or over the next couple
 Keep Cl at 98-108mmol/L.
-
of hours, if necessary.
 Write these down on the
PREMAJOR ROUND bottom of your progress notes
PREPARATION and make a daily checklist.
 The resident doctor should make The progress notes must have
certain that the problem list is clear plans at the end of the
complete and formulate a problems.
preliminary plan of care.  After rounds
 All files should be well  Enter new orders in the
summarized. patient’s files and
communicate with nurses.

166
CRITERIA FOR ADMISSION  Must demonstrate the ability of
TO NICU maintaining an adequate nutrition
 Newborn/infant in unstable as evidenced by sufficient caloric
condition fluid intakes as determined by the
 Prematurity at less than 36 weeks. physician.
 Respiratory distress  Must have adequate urinary
output as determined by the
 Hypoglycemia
physician and should have passed
 Persistent pulmonary
meconium.
hypertension of the newborn
 Must maintain appropriate body
 Congenital heart disease
temperature outside of artificial
 Sepsis or suspected sepsis
environments.
 Congenital anomalies
 Must meet physical assessment
 Hemolytic disease of the newborn
criteria deemed by the physician
 Hematologic disorders as “stable” and/or not requiring
 Seizure disorders further hospitalization.
 Meconium aspiration  Infants with a weight of <1.7kg
 Perinatal asphyxia will be seen weekly until a target
 Metabolic disorders including weight of 1.7kg is reached when
infant of a diabetic mother they will then be seen in
 Congenital infections outpatient clinic as necessary.
 Any other newborn/infant  Must be without episode of
requiring further evaluation as bradycardia as required by the
determined by the neonatology discharging physician or can be
resident or the attending monitored appropriately at home.
consultant.  Any infant not meeting the above
DISCHARGE CRITERIA discharge criteria may be
discharged with prior approval of
FROM NICU the attending physician.
 Low birth weight neonate or any
infant with one of the above INCUBATOR CARE
diagnoses whose condition has
stabilized as determined by CARING FOR A BABY IN
attending physician.
AN INCUBATOR
 Must be at least 24 hours old on
the day of discharge or as ordered  An incubator is a glass-covered,
by the physician. fully enclosed neonatal bed

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

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 Weekly cleaning can be done on OBSERVATIONS AND
empty incubators and these NURSING CARE OF AN
incubators are dismantled, INCUBATOR BABY
decontaminated, cleaned and
 Frequency of observation will
sterilized. Allow for 24 hours for
depend on the neonate’s
this process.
condition.
 Empty incubators should be kept
 Temperature:
warm at an average incubator
 Should be checked and if the
temperature of 34oC and adjusting
infant is hypothermic or
according to the neonate’s skin
hyperthermic, regulate the
temperature. Always compare
incubator temperature by
knob set temperature with the
either increasing or reducing
incubator temperature inside to
by 1oC at a time and
ascertain accuracy.
rechecking the Axillay
 All incubators should be temperature of the neonate
humidified with distilled water every 15 minutes.
put in reservoir tanks and the
 Respirations
water should be changed on daily
 Breathing can be checked on
basis.
looking at chest rise. If apnea,
 Humidity should be kept 40-60% see if more than 10 seconds
for term neonates and 60-80% for and stimulate if prolonged or
premature and ventilated babies. repeated-use of bag and mask.
 Incubator temperature should be  Insert a nasogastric tube to
set to provide a neutral thermal expel air from the stomach to
environment according to the avoid splinting. An apnea
neonate’s body temperature and monitor can be used. Inform
in response to the set medical officer for further
temperatures. management.
 All opening on the incubator  Heart rate
should be kept closed at all times  Can be checked on breathing
and procedures should be and color of the neonate.
conducted through portholes  A cardiac monitor can be
unless impossible e.g. used. If HR <100, resuscitate
resuscitation. and inform-medical
officer/senior doctor.

169
 Weight: can be done if necessary are called small for gestational
especially if incubator scale is not age or small-for-dates.
available  The majority of these infants are
 Feeding- preferably nasogastric normal but small.
tube feeding as per calculated  The incidence of congenital
amount per kg body weight/24 abnormalities and neonatal
hours. problems is higher in those whose
 Eliminations birthweight falls below 2 SD
 Urine: can be observed in below the mean.
terms of color, odors and can  An infant’s birthweight may also
be measured using urine be low because of preterm birth,
collectors, counting nappies or because the infant is both
or measuring nappy weight preterm and small for gestational
pre and post urination. age.
 Stool- can be observed for  Small-for-gestation age infants
consistency and color, may have grown normally but are
whether there is diarrhea, small or they may have
melena or meconium also experienced intrauterine growth
note the frequency. restriction (IUGR) i.e. they have
 Other nursing care can be done as failed to reach their full
required. genetically determined growth
potential and appear thin and
SIZE AT BIRTH malnourished.
 Recall average birth weight= 3kg  Babies with a birthweight above
(2.5 – 3.5 kg) the 10th centile may also be
 Babies born weighing between malnourished e.g. a fetus growing
1500-2500g= low birth weight along the 80th centile who
(LBW). develops growth failure and
 Babies born weighing between whose weight falls to the 20th
1000-1500g= very low birth centile.
weight (VLBW).
 Babies less than 1000g= PATTERNS OF GROWTH
Extremely low birth weight RESTRICTION
(ELBW).  Has been classified as:
 Babies with a birthweight below  Asymmetrical growth
the 10th centile for gestational age restriction (common): weight
or abdominal circumference

170
 lies on a lower centile than that THE GROWTH RESTRICTED
of the head. This occurs when INFANT
the placenta fails to provide  After birth, these infants are liable
adequate nutrition late in to:
pregnancy but brain growth is  Hypothermia because of their
relatively spared at the relatively large surface area
expense of liver glycogen and  Hypoglycemia from poor fat
skin fat. and glycogen stores
o Associated with: utero-  Hypocalcemia
placental dysfunction  Polycythemia (venous
secondary to maternal hematocrit > 0.65)
pre-eclampsia, multiple
pregnancy, maternal LARGE FOR
smoking or it may be GESTATIONAL AGE
idiopathic. INFANTS
o These infants rapidly put  Large for gestational age infants
on weight after birth. are those above the 90th weight
 Symmetrical growth centile for their gestation.
restriction: head circumference  Macrosomia is a features of
is equally reduced. It suggests infants of mothers with either
a prolonged period of poor permanent or gestational diabetes,
intrauterine growth starting in or a baby with a congenital
early pregnancy (or that the syndrome (e.g. Beck-Wiedemann
gestational age is incorrect). It syndrome).
is usually due to a small but
 The problems associated with
normal fetus, but may be due
being large for gestational age
to a fetal chromosomal
are:
disorders or syndromes, a
 Birth asphyxia from a difficult
congenital infection, maternal
delivery.
drug and or alcohol abuse or a
 Breathing difficulty from an
chronic medical condition or
enlarged tongue in Beckwith-
malnutrition.
Wiedemann syndrome
o Infants are more likely to
 Birth trauma, especially from
remain small permanently shoulder dystocia at delivery
(difficult delivery the
shoulders form impaction

171
 behind maternal symphysis  Babies less than 1000g=
pubis) Extremely low birth weight
 Hypoglycemia due to (ELBW).
hyperinsulinism  Most babies that are considered to
 Polycythemia have low birth weight are
premature however, other
PREMATURITY conditions can cause LBW in a
baby born after a full-term
PREMATURITY AND pregnancy, such as smoking during
BIRTH WEIGHT pregnancy.
 A baby born alive before 37 weeks  Babies with LBW who are full
(259 days) of gestation is term (but underweight) and
considered premature. “premature babies” with weights
 Late preterm (34- 37 weeks) less than expected are termed
 Moderate preterm (32-34 small for gestational age (SGA).
weeks)  Causes for intrauterine growth
 Very preterm (28-32 weeks) restriction include:
 Extreme preterm (less than 28)  Infections of the fetus before
 Terms that refer to premature delivery
babies (Prematurity) are preterm  Chromosome or gene
and preemie. abnormalities (S-SGA)
 Preterm babies are at risk for a  Insufficient nutrition provided
number of complications related to by placenta (A-SGA).
the fact that their organs may not  Poor nutrition in the mother,
be mature at the time of birth. other problems such as chronic
 The earlier the baby is born the disease or smoking (A-SGA)
higher the risk of complications.
CAUSES OF PREMATURITY
 Recall average birth weight= 3kg
 Many factors are linked to
(2.5 – 3.5 kg)
premature birth. Some factors
 Babies born weighing between
directly cause early labor and birth
1500-2500g= low birth weight
while others can make the mother
(LBW).
or baby sick and require early
 Babies born weighing between
delivery.
1000-1500g= very low birth
 Maternal factors include:
weight (VLBW).
 Pre-eclampsia (toxemia or
high blood pressure of

172
 pregnancy occurring after 20 FEATURES AND
weeks of pregnancy) COMPLICATIONS OF
 Chronic medical illness (such
PREMATURITY
as heart or kidney disease,
FEATURES OF PREMATURITY
SCD)
 Infection (such as group B  Every premature infant is different
streptococcus, urinary tract however features of prematurity
infections, vaginal infections, include:
infections of the fetal/placental  Very small size (at 24 weeks-
tissues) male 700g, female 620g)
 Drug use (such as cocaine)  Very thin fragile skin with
 Abnormal structure of the veins visible underneath (Dark
uterus red color all over the body)
 Cervical incompetence  Soft hair on the body: lanugo
(inability of the cervix to stay  Ears: Pinna soft, no recoil,
closed during pregnancy) startles to loud noise
 Previous preterm birth  Eyes: fused eyelids, infrequent
 Factors involving the pregnancy eye movements.
 Abnormal or decreased  Faint cry
function of the placenta  Breast tissue: no breast tissue
 Placenta previa (low-lying palpable
position of the placenta)  Genitalia:
 Placental abruption (early o Male: Scrotum lacks
detachment of the placenta rugae (smooth), no testes
from the uterus) in scrotum
 Infection of the placenta (cryptorchidism)
 Premature rupture of o Female: prominent
membranes (amniotic sac) clitoris, labia majora
 Polyhydramnios (too much widely separated, labia
amniotic fluid) minora protruding
 Factors involving the fetus  Little activity, weak cry
 When the fetal behavior  Feeding problems: baby can’t
indicates the intrauterine suck or swallow normally
environment is not healthy  Breathing problems: apnea of
 Multiple gestation (twins, prematurity
triplets etc.)  Limbs extended. Jerky
 Congenital anomalies movements

173
COMPLICATIONS OF blood vessels due to hypoxic
PREMATURITY or hypotensive injury.
 Predisposing factors:
CENTRAL NERVOUS SYSTEM prematurity, RDS, hypo or
 Intraventricular hemorrhage with hypervolemia and shock.
hydrocephalus.  Signs and symptoms: most
 Intraventricular hemorrhage is asymptomatic, lethargy, poor
rupture of germinal matrix

174
 suck, high-pitched cry and lungs are immature and do not
bulging fontanelle. produce surfactant (which is
 Diagnosis: cranial ultrasound produced from the pneumocyte
(through anterior fontanelle) type II cells as early as 20 weeks
 Treatment: directed toward but peaks at 35 weeks of
correction of underlying gestation).
condition (RDS, shock etc.) In  Incidence: infants <32 weeks
cases of associated gestation
hydrocephalus, placement of  Signs and symptoms: seen
ventriculoperitoneal shunt may within first 3 hours of birth,
be required. tachypnea, grunting and
 Hemorrhagic and periventricular cyanosis.
white matter brain injury  Surfactant contains both
(leukomalacia) Lecithin and sphingomyelin.
 Cerebral palsy (difficult muscle  The Lecithin-syphingomyelin
control, stiffness) ratio is a determining factor in
 Learning disability/ Mental lung maturity.
retardation  A L-S ratio greater than 2.0 to
 Deafness 2.5 is indicative of lung fetal
 Retinopathy of prematurity: this is lung maturity.
a proliferative retinopathy  In addition, the alveoli are small,
 Caused by proliferation of inflate with difficulty and do not
immature retinal vessels due to remain gas filled between
excessive use of oxygen. inspirations. The rib cage is weak
 Can lead to retinal detachment and compliant.
and blindness in severe cases.  There is high surface tension and
 Diagnosis: all very low birth propensity for alveolar collapse.
weight infants should be  Alveolar collapse results in
screened for ROP with an progressive atelectasis,
ophthalmoscopic exam. intrapulmonary shunting,
 Treatment: Laser surgery may hypoxemia and cyanosis.
be needed in severe cases.  Apnea of prematurity: due to
 Blindness immaturity of the breathing center
in the brain
RESPIRATORY SYSTEM  Apnea: cessation of breathing
 Respiratory distress syndrome for more than 20 seconds
(hyaline membrane disease): the associated with bradycardia

175
 Chronic lung disease/  Alveolar stage- birth until
bronchopulmonary dysplasia early childhood (8 years)
 Need for supplemental oxygen
CARDIOVASCULAR SYSTEM
beyond 28 days of life.
 Characterized by squamous  Persistent/patent ductus arteriosus
metaplasia and hypertrophy of  Anemia of prematurity
small airways.  Hypotension
 Infants with  Atrial septal defects
bronchopulmonary dysplasia  Ventricular septal defects
can be wheezing remember,
GASTROINTESTINAL SYSTEM
“not all that wheezes is
 Necrotizing enterocolitis
asthma!”
 Neonatal jaundice
 Treatment: supplemental
oxygen, oral steroids,  Poor feeding
bronchodilators. GENITOURINARY SYSTEM
 Pneumothorax  Inguinal hernias
 Note in extremely premature
babies (23-25 weeks) the lung may METABOLIC
be incompletely developed (in  Hypoglycemia
addition to being immature) these  Hypothermia
rarely survive outside the womb.  Hypocalcemia
 Recall the five stages of lung  Electrolyte and fluid imbalance
development include:  Osteopenia of prematurity
 Embryonic stage: 3-8 weeks
 Pseudoglandular stage: 5-16
weeks  Rickets of prematurity
 Canalicular stage-16- 26  Infections: neonatal sepsis
weeks  Perinatal asphyxia
 Terminal saccular-26-37 week

176
177
DIAGNOSIS infant’s gestational age (Ballard
 History: 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 to provide an estimate of an

178
179
MANAGEMENT  In anticipation of RDS secondary
 Admit to neonatal intensive care to surfactant deficiency, doses of
unit (NICU) for special care and dexamethasone (steroid) can be
monitoring. given to the mother 48 hours
 Temperature control: maintain before delivery- this is assuming
temperature in an incubator at that the lungs are already at the
ideal. terminal saccular stage thus they
 Parents are encouraged to can be stimulated to produce
participate in as much care of surfactant.
their baby as they are
comfortable providing. This
may involve holding, TRANSIENT TACYPNEA
Kangaroo care, diaper OF THE NEWBORN
changes, giving baths, feeding.  This is the commonest cause of
 Feeding respiratory distress in term infants.
 Total parenteral nutrition or  Infants with transient tachypnea of
intravenous feeding the newborn (TTN) within the first
 Gavage feeding: Preterms few hours of birth presents with
before the 34th week of tachypnea, increased oxygen
gestation need to be fed requirement, and occasional
through a nasogastric tube or hypoxia noted on arterial blood
nasojejunal tube. gases without concomitant carbon
 Breast feeding often has to be dioxide retention.
delayed. Expressed breast milk
 Causes include:
can be given in a bottle.
 Cesarean section
 Decisions about feeding  Maternal asthma and smoking
depend on the infant’s
 Prolonged labor
particular circumstances.
 Prevention of the infection: hand PATHOPHYSIOLOGY
washing and aseptic techniques  It is caused by delay in the
 Treatment of complications. resorption of lung liquid and is

180
 more common after birth by in large part, from an immaturity
caesarean section. in the expression of ENaC which
 It was initially thought to be a can be upregulated by
problem of relative surfactant glucocorticoids.
deficiency but it is now  Bioelectrical studies of human
characterized by an airspace infants nasal epithelia demonstrate
fluid burden secondary to that both TTN and RDS have
inability to absorb fetal lung defective amiloride sensitive
fluid. sodium transport (ENaC)
 An infant born by cesarean
delivery is at risk of having CLINICAL FEATURES
excessive pulmonary fluid as a HISTORY
result of not experienced all  Signs of RD (tachypnea, nasal
the stages of labor and flaring, grunting, retractions,
subsequent low release of cyanosis in extreme case) becomes
counter-regulatory hormones evident shortly after birth.
at the time of delivery.  This disorder is transient with
 In vivo experiments demonstrated resolution occurring by age 72
that lung epithelium secretes Cl- hours.
and fluid throughout gestation but
only develops the ability to
PHYSICAL EXAMINATION
actively reabsorb Na+ during late  Findings include:
gestation.  Tachypnea with variable
grunting
 At birth the mature lung switches
 Flaring
from active Cl- (fluid) secretion to
 Retracting
active sodium (fluid) absorption in
 Cyanosis (extreme cases)
response to circulating
catecholamines. WORKUP
 Changes in oxygen tension  Arterial blood gas
augment the sodium transporting  Pulse oximetry
capacity of the epithelium and  The Chest X-ray (diagnostic
increase gene expression for the standard) may show fluid in the
epithelial amiloride sensitive horizontal fissure.
sodium channel (ENaC).  Prominent perihilar streaking
 The inability of the immature fetal (markings) which correlates
lung to switch from fluid secretion with the engorgement of the
to fluid absorption results at least

181
 lymphatic system with retained persistent pulmonary
lung fluid and fluid in the hypertension of the
fissures. newborn, milk aspiration
 Patchy infiltrates have also o Rare: diaphragmatic
been described hernia, tracheo-
esophageal fistula (TOF),
pulmonary hypoplasia,
airways obstruction e.g.
choanal atresia,
pulmonary hemorrhage
 Extrapulmonary:
o Congenital heart disease
o Intracranial birth
trauma/encephalopathy
 Additional ambient oxygen may be
o Severe anemia
required.
o Metabolic acidosis
 The condition usually settles
within the first day of life but can MANAGEMENT
take several days to resolve  Medical care is supportive. As the
completely. retained lung fluid is absorbed by
 This is a diagnosis made after infant’s lymphatic system the
consideration and exclusion of pulmonary status improves.
other causes.  Supportive care include:
 When managing an infant with  Intravenous fluids
TTN it is important to observe for  Gavage feeding (until the
signs for clinical deterioration that respiratory rate has decreased
may suggest other diagnoses and [<60bpm] enough to allow oral
to observe closely for the feedings)
development of fatigue  Supplemental oxygen to
 Causes of Respiratory distress in maintain adequate arterial
term infants: oxygen saturation
 Pulmonary  Maintenance of
o Common: transient thermoneutrality
tachypnea of the newborn  Environment of minimal
o Less common: meconium stimulation
aspiration, pneumonia,  As TTN resolves the infant’s
RDS, Pneumothorax, tachypnea improves, oxygen

182
 requirements decrease and the  Alveolar collapse results in
CXR shows resolution of perihilar progressive atelectasis,
streaking. intrapulmonary shunting,
 If condition worsens continuous hypoxemia and cyanosis.
positive airway pressure (CPAP)  This is usually seen in infants <32
by nasal prongs or endotracheal weeks’ gestational age.
tube or mechanical ventilation can  Signs and symptoms:
be done.  Seen within the first 3 hours of
 The use of medications for TTN is birth.
minimal  Tachypnea (>60breath/min)
 Aside from use of antibiotics for a  Laboured breathing with chest
period of 36- 48 hours after birth wall recession (particularly
until sepsis has been ruled out, no sternal and subcostal
further pharmacotherapy generally indrawing) and nasal flaring.
is prescribed.  Expiratory Grunting
 Diuretics have not been shown to  Cyanosis if severe
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.

183
  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.

HYPOXIC ISCHEMIC
ENCEPHALOPATHY

HYPOXIC ISCHEMIC
ENCEPHALOPATHY
 This is acute or subacute brain
injury due ischemia and hypoxia.
 A baby who is acutely asphyxiated
will go through a period of
Figure 15: Etiology of respiratory primary apnea into terminal apnea,
distress in term infants if the hypoxia insult is not
 Diagnosis: terminated early.
 Chest X-ray- fine, diffuse  Hypoxic ischemic encephalopathy
reticulogranular or “ground (HIE) has no predilection for race
glass” pattern and air or sex.
bronchograms.  The condition is seen in the
 Treatment: newborn period.
 Aggressive respiratory support  Most neonates are term at birth. In
including most cases, the condition manifests
o Oxygen at birth or within a few hours after
o Continuous positive birth.
airway pressure (CPAP)
o Intubation
o Mechanical ventilation

184
CAUSES OF HYPOXIC  Postpartum:
ISCHEMIC o Severe prematurity
o Cardiac disease
ENCEPHALOPATHY
o Pulmonary disease
 Causes of hypoxic ischemic
o Infections: sepsis
encephalopathy are many.
o Low neonatal blood
 A lot of risk factors are implicated
pressure
and these can be divided into:
o Brain/skull trauma
 Prepartum:
o Congenital brain
o Maternal diabetes
malformations
o Vascular disease affecting
circulation to placenta PATHOPHYSIOLOGY
o Pre-eclampsia  Brain hypoxia and ischemia from
o Cardiac disease: both systemic hypoxia with reduced
hypo and hypertension cerebral blood flow are the
o Congenital infections primary triggering events for HIE.
o Drugs and alcohol abuse  In this regard, HIE is similar to
o Severe fetal anemia stroke syndromes in adults, except
o Intrauterine growth that in neonates the pathology is
restriction more generalized and the causes
o Fetal Lung malformations are different.
 Intrapartum:  Initial compensatory
o Excessive placental adjustments which include
bleeding hypoxia and hypercapnia
o Very low maternal blood (increased carbon dioxide
pressure partial pressure) are powerful
o Umbilical cord accidents stimuli, increasing cerebral
o Prolonged stages of labor blood flow and thus oxygen
(excessive or prolonged delivery.
uterine contractions)  During the early phase of shock
o Abnormal fetal positions the cardiac output is redistributed
o Cord compression: cord (dive reflex) and systemic blood
around the neck, shoulder pressure increases (due to
dystocia (difficult child increased epinephrine release) to
birth), cord prolapse maintain Cerebral blood flow.
o Rupture of  Cerebral autoregulation
placenta/membrane/uterus mechanism maintains brain

185
 perfusion for a while in spite of an by increasing intracellular
initial drop in the mean blood calcium (apoptosis).
pressure. The range of blood  These excitatory amino acids
pressure in the cerebral blood flow also cause nitric oxide to be
is set at lower limits than adult released and this may
range (i.e. 60 to 100mmHg) exacerbate the neuronal
 With prolonged asphyxia the early damage although its
compensatory adjustments fail and mechanisms are unclear.
cerebral blood flow may become  Energy failure also causes the
“pressure passive” i.e. dependent inactivation of the sodium
on systemic blood pressure. potassium ATPase pump and
 As systemic blood pressure falls, this leads to retention of
cerebral blood flow falls below intracellular sodium
critical levels and brain hypoxia accompanied by influx of
occurs this results in intracellular water into the cell causing
energy failure (due to anaerobic swelling and death.
respiration and lack of aerobic  There is also destruction of ion
respiration) pumps by lipid peroxidation of
 During the early phase of injury, cell membranes. This also
the brain temperature drops and causes influx of water,
local release of the swelling and death.
neurotransmitter GABA (Gamma  Following the initial phase of
amino butyric acid) increases energy failure from asphyxia
resulting in changes that reduce injury cerebral metabolism may
cerebral oxygen demand, recover only to deteriorate in the
transiently minimizing the impact second phase.
of asphyxia.  Reperfusion injury is a second
 Neuronal cellular injury is determinant to the extent of brain
dependent on the extent of the damage. By 6-24 hours after initial
initial injury. injury a new phase of neuronal
 Hypoxia and ischemia destruction sets in characterized by
increases release of excitatory apoptosis. This is also known as
amino acids (glutamate and “delayed injury” and it may
aspartate) in cerebral cortex continue for days to weeks. The
and basal ganglia. These severity of brain injury in this
amino acids cause cell death phase correlates well with the
severity of long term adverse

186
 neurodevelopmental outcome in o Typically resolves in
infants. 24hours
 Reperfusion injury is often  Moderate (Stage 2- HIE II)
associated with inflammatory o In stage to the baby is
reactions and may result in lethargic, develops
hyperkalemia. seizures and may be
hypotonic with
CLASSIFICATION (SIGNS parasympathetic over-
AND SYMPTOMS) activity.
 The symptoms depend on whether o Seizures may occur
the hypoxic-ischemic within the first 24 hours
encephalopathy is: of life.
 Mild (Stage 1- HIE-1) o The infant shows marked
o The infant is irritable, abnormalities of tone and
responds excessively to movement, cannot feed.
stimulation (hyperalert o Reflexes are sluggish or
with predominant absent.
sympathetic activity), o There is occasional apnea
may have starring of the with bradycardia.
eyes and hyperventilation o APGAR score is between
and has impaired feeding. 4-5.
o Muscle tone may be o Recovery is between 1-2
slightly increased/normal weeks and is associated
and deep tendon reflexes with better long-term
may be brisk in the first outcome.
few days o An initial period of well-
o Transient behavioral being may be followed by
behaviors such as: poor sudden deterioration,
feeding, irritability or suggesting reperfusion
excessive crying. injury during this period
o It is also associated with seizure intensity might
tachypnea and increase.
tachycardia.  Severe (Stage 3- HIE III)
o APGAR score is between o There are no normal
6 and 7. spontaneous movements
o There are no seizures. or response to pain
(coma/ Stupor).

187
 o Limbs are flaccid o Multi-organ failure is
(hypotonic) present.
o Breathing is irregular and o APGAR score is between
infant often requires 0-3.
ventilation support. o Babies usually deteriorate
o Reflexes are absent and and die or survive with
there is severe apnea. multiple complications
o Seizures are prolonged such as cerebral palsy.
and often refractory to
treatment.

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 -

188
DIAGNOSIS AND respirations, put on ventilator
INVESTIGATIONS on IPPV.
 History and Examination: Many  Adequate ventilation: put on
clinical features of neonatal HIE oxygen once breathing is
are nonspecific, as such the spontaneous.
diagnosis is made with caution and  Check vitals: Record level of
only after a careful assessment of consciousness, respirations,
history, physical examination and temperature, heart rate, blood
laboratory studies. pressure, capillary refill time,
oxygen saturation, tone, urine
 Investigations:
 Serum electrolytes: U &E output and blood glucose.
 Creatinine  Perfusion and blood pressure
 Group and Save, Cross match management
 Cardiac and liver enzymes  Careful fluid management:
 Clotting profile/panel- o Initial management: If
prothrombin time, Partial capillary refill time is >2
seconds consider volume
thromboplastin time and
fibrinogen levels expansion with normal
 Full blood count saline (or Ringers lactate)
 Serum bilirubin 10ml/kg over 5-10min
 Arterial blood gases while awaiting plasma
 Imaging: (Max 2 boluses).
o MRI brain o Give 1mg vitamin K,
o Cranial ultrasonography Cefotaxime and 10%
o Echocardiography dextrose for day 1.
o Electroencephalography  Avoidance of hypo or
 Hearing test hyperglycemia
 Avoidance of hypothermia:
MANAGEMENT this has been shown to
 Initial resuscitation, stabilization increase the risk of adverse
and treatment is largely supportive outcomes in neonates with
and should focus on: HIE II and HIE III.
 Admit of NICU  Therapeutic hypothermia (33-
 Perform ABCs: Suction if still 33.5 degrees for 72 hours)
having blocked airways. If followed by slow and
baby not breathing, ambubag controlled rewarming for
and intubate. If no spontaneous

189
 infants with HIE II and HIE  If still having seizures
III. increase the dose to
 Treatment of seizures: 1.5mg/dose.
o Assess sensorium, tone  If still having seizures
and seizures. reduce the frequency
o If seizure lasting more to 2 hourly then
than 3 min or more than 3 hourly if seizures
in one hour: continue.
 Start Phenobarbitone
20mg/kg IV stat.
SYSTEMIC EFFECTS AND
 If still seizing after 15 COMPLICATIONS
minutes repeat  Complications of HIE are multi-
loading dose systemic and affect all systems of
phenobarbitone the body:
10m/kg IV stat and  CNS
put on maintenance  Cerebral infarcation
phenobarbitone  Intracranial/ intraventricular
5mg/kg/day OD. hemorrhage
 If seizures are  Cerebral edema
refractory to  Hyper/hypotonia
phenobarbitone,  CVS
clonazepam may be  Myocardial ischemia: poor
used with a loading contractility
dose of 100-200  Tricuspid insufficiency
mcg/kg followed by  Cardiac stunt
infusion at 10-30  Hypotension
mcg/kg/hour if  Pulmonary
seizures continue.  Pulmonary hypertension
 If clonazepam is not  Pulmonary hemorrhage
available diazepam  Respiratory distress syndrome
1mg/dose 3 hourly  Gastrointestinal
can be started in  Necrotizing enterocolitis
addition to the  Perforation
maintenance  Hepatic dysfunction
phenobarbitone.  Adrenals: Adrenal hemorrhage
 Renal:

190
  Acute tubular or cortical  It is often multifactorial in
necrosis origin resulting in loss of
 Metabolic integrity of the gut mucosal
 Inappropriate secretion of barrier with passage of
ADH (SIADH) bacteria into the wall of the
 Hyponatraemia bowel.
 Hypocalcemia  Bacterial infection has been
 Hypoglycemia implicated in NEC.
 Myoglobinuria  Clostridial infections have
 Integument: subcutaneous fat been associated with some
necrosis outbreaks of NEC but the
 Hematology: Disseminated etiology remains unclear.
intravascular coagulation  It is one of the most common
surgical conditions in neonates.
NECROTIZING ENTEROCOLITIS  It is most frequent in preterm
infants with an incidence as high
NECROTIZING as 8-10% in infants <30 weeks’
ENTEROCOLITIS (NEC) gestation.
 NEC is a serious condition that  It affects preterm infants in the
primarily affects preterm or SGA first few weeks of life.
babies.  Usually affects the terminal
 The condition usually involves the ileum and colon to a variable
terminal ileum and the colon but extent.
may involve any area from the  Preterm infants fed cow’s milk
stomach to the rectum. formula are more likely to develop
this condition than if they are fed
PATHOGENESIS only breast milk (antibodies in
 It is a rare complication due to breast milk are protective and
impaired blood flow to the bowel. decrease the risk of NEC).
 Mucosal ischemia allows gut  The picture mimics neonatal sepsis
microorganisms to penetrate the because of the presence of
bowel wall causing a severe abdominal distension, apnea,
hemorrhagic colitis. bradycardia, instability of
 Hypovolemia and hypoxia temperature, cyanosis and
result in damage within the lethargy.
mucosa cells initiating the  Prematurity, respiratory distress
NEC. syndrome (RDS), hypoxic

191
 ischemic encephalopathy,  Metabolic acidosis and oliguria
congenital cardiac malformations, may be present and NEC may lead
umbilical vessel Catheterisation, to thrombocytopenia, disseminated
exchange transfusion, intravascular coagulation and
hypoglycemia, polycythemia, death.
postoperative stress and  If the baby has a patent processus
hyperosmolar feeds have all vaginalis, free fluid or even gas or
implicated in the etiology. meconium is occasionally seen in
the scrotum.
RISK FACTORS
 Polycythemia BELL’S CLINICAL
 Hypertonic milk STAGING
 Too rapid feeding protocols  Clinical manifestation may be
 Prematurity described in 3 stages:
 Perinatal asphyxia  Stage 1 (Suspected NEC):
 Sepsis unstable temperature, apnea,
 Excessive fluids bradycardia, lethargy, mild
abdominal distension,
CLINICAL FEATURES vomiting.
 Initial symptoms include: o IA- Infant with suggestive
 Feeding intolerance clinical signs but X-ray
 Delated gastric emptying non-diagnostic
 Abdominal distension or o IB- same as IA plus
tenderness or both bloody stool (frank or
 Illeus/decreased bowel sounds occult)
 Abdominal wall erythema  Stage 2 (Definite NEC):
(Advanced stages) Clinical signs similar to stage
 Bloody stools (hematochezia) 1. Bowel sounds are
 Bile stained vomiting and diminished with or without
Bilious aspirate abdominal tenderness.
 Systemic signs are non-specific: Pneumatosis intestinalis (gas
 Apnea in intestinal wall) and dilation
 Lethargy of intestines are seen on
 Decreased peripheral perfusion abdominal X-ray.
 Shock (in advanced stages) o IIA- mildly ill infant
 Cardiovascular collapse o IIB- moderately ill infant
 Bleeding diathesis

192
  Stage 3 (Advanced NEC): In  Investigations:
addition to the above, the  Culture of blood, CSF, urine
infant is severely sick with and stool.
hemodynamic instability.  U/E & creatinine
There are frank signs of  Full blood count
peritonitis with abdominal wall  Abdominal X-ray- left lateral
redness. Pneumoperitoneum decubitus.
may occur due to intestinal  Blood gases
perforation.  Classic radiological findings:
o IIIA- critical with  Distended loops of bowel and
impending perforation thickening of the bowel wall
o IIIB- critical with proven with intramural gas
perforation (Pneumatosis intestinalis).
o Grade I- thickening of the
DIAGNOSIS
bowel wall +/- dilation of
 NEC can progress rapidly from
the gut
mild abdominal distension to
o Grade II- bowel wall
fullness to septic shock and
o Grade III- gas in the liver
necrosis of the entire intestine. The
and biliary tree
management requires high index
o Grade IV- gas within the
of suspicion.
peritoneum
 On examination: palpation may  Air fluid levels
reveal crepitus from the intramural  Venous portal gas
gas, which can be palpated among  Pneumoperitoneum is
the coils of distended loops of suggestive of perforation
bowel and this is an important sign
of NEC.

193
 194

MANAGEMENT  Gastric decompression (insert a

MEDICAL TREATMENT nasogastric tube and aspirate
stomach contents).
 Bowel rest, no oral feeds (stop
feeds as soon as NEC is suspected)
 Broad spectrum antibiotics to radiographs or a positive
cover both aerobic and anaerobic paracentesis.
organisms, change antimicrobial  Treatment may include resection
treatment according to sensitivity. of necrotic bowel and possible
 Cefotaxime primary anastomosis.
 Metronidazole  In very low birth weight (under
 Parenteral fluids and nutrition. 500g) infants percutaneous
 ¼ Strength Darrows in 10% drainage has been used in
dextrose. preference to laparotomy.
 Intubate when in respiratory  This is performed by means of
failure or worsening acidosis. applying a local anesthetic to
 If shock is present volume the right lower quadrant of the
infusions and pressors are abdominal and then a 10-12
indicated. French gauge catheter is
 Plasma and platelet transfusion inserted into the abdominal
may be necessary to prevent cavity to drain air, meconium
bleeding tendency. and fecal material which has
 If neonate poorly perfused and leaked from perforated bowel.
HCt>40% give ordinary  The extent of pneumatosis is not
plasma at 20ml/kg. proportional to severity of illness
 If HCT<40% give packed red and not always an indication for
cells 10-15ml/kg. surgical intervention.
 Surgery when indicated.  Portal venous gas is a poor
prognostic sign.
SURGICAL MANAGEMENT
 Indications for surgery: COMPLICATIONS
 Increasing peritonitis  Intestinal obstruction (a late
 Failure to stabilize with complication due to adhesions and
medical treatment strictures)
 Development of an abdominal  Nutritional deficiencies (e.g.
mass malabsorption, short gut
 Persistent loop of free gas on syndrome, growth failure,
an abdominal X-ray malnutrition)
 Exploratory laparotomy is  Cholestasis
indicated for pneumoperitoneum,
presence of a fixed loop on serial

195
DIFFERENTIAL DIAGNOSIS  Vernix Caseosa (an oily
 Differential diagnosis early in the substance that covers the skin
disease it may be difficult and one at birth)
should consider:  Bile salts
 Neonatal sepsis  Amniotic fluid
 Volvulus neonatorum  Meconium is sterile and does not
 Hirschsprung’s enteritis contain bacteria which is the
 Infarction of the bowel primary differentiating factor from
stool.
MECONIUM ASPIRATION  Meconium aspiration syndrome is
SYNDROME commoner in babies born post-
term and in babies with fetal
MECONIUM distress.
ASPIRATION  Infants that also become acidotic
SYNDROME may inhale thick meconium and
develop meconium aspiration
 Meconium aspiration syndrome
syndrome. (asphyxiated infants
(MAS) is caused by intrauterine/
may start gasping and aspirate
intrapartum aspiration of
meconium before delivery)
meconium stained amniotic fluid
in term or post-term infants.  Meconium is passed before birth
by 8-20% of babies. It is rarely
 Meconium is usually passed 24
passed by preterm infants and
hours after delivery in a term baby.
occurs increasingly the greater the
 Aspiration of meconium can occur
gestational age affecting 20-25%
before, during or after delivery.
of deliveries by 42 weeks.
 Meconium (first stool) is a
 Factors that facilitate passage of
material in the fetal gut that
meconium:
consists of:
 Acidosis
 Water
 Placental insufficiency
 Mucopolysaccharides
 Maternal hypertension
 Desquamated skin
 Pre-eclampsia
 Gastrointestinal mucosal
 Infection
epithelial cells, mucus,
 Oligohydramnios
secretions
 Maternal drug abuse (tobacco
 Lanugo (hair)
and cocaine)
 Aspiration of meconium into the
tracheobronchial tree causes

196
 complete or partial bronchial  Meconium directly alters
obstruction leading to patchy areas amniotic fluid, reducing
of sub-segmental atelectasis and antibacterial activity hence
compensatory areas of increasing chances of perinatal
hyperinflation. bacterial infection
 Meconium is irritating to fetal
PATHOPHYSIOLOGY skin hence increasing the
 In utero meconium passage results incidence of erythema
from stimulation of a maturing GI toxicum.
tract usually due to fetal hypoxic  Perinatal aspiration of
stress. meconium stained amniotic
 Meconium is often passed as a fluid.
consequence of distress (i.e.  Aspiration of meconium stained
hypoxia) in the fetus at term and amniotic fluid causes hypoxia via
becomes more frequent after 42 4 major mechanisms:
weeks of gestation.  Airway obstruction: complete
 As the fetus approaches term, the or partial obstruction. Partial
GI tract matures, vagal stimulation obstruction causes air trapping
from head or spinal cord and hyperdistention of the
compression may cause peristalsis alveoli commonly termed the
and relaxation of the sphincter ball-valve effect. The gas
muscles leading to passage of trapped may rupture into
meconium. pleura (pneumothorax),
 The degree of meconium-stained mediastinum
amniotic fluid varies from slightly (pneumomediastinum) or
green to dark green and thick pericardium
consistency (pea-soup). (pneumopericardium)
 The meconium stained amniotic  Surfactant dysfunction:
fluid may reach the distal airways meconium deactivates and
and alveoli in utero if the fetus inhibits the production of
becomes hypoxic and develops surfactant. Constituents of
gasping or deep respiratory meconium such as free fatty
movements or may occur at the acids have higher minimal
time of birth with first inspirations. surface tension than surfactant
 Effects of meconium in amniotic and strip it from alveolar
fluid: surface resulting in diffuse
atelectasis.

197
  Chemical pneumonitis:  Meconium is a lung irritant that
enzymes, bile salts and free causes chemical pneumonitis.
fatty acids in meconium  Yellow-green staining of
irritate the airway and fingernails, umbilical cord and
parenchyma. This begins skin may be observed.
within a few hours of
aspiration EVALUATION
 Persistent pulmonary  Meconium aspiration syndrome is
hypertension of the newborn: suggested by a history of
infants have primary or meconium noted at or before
secondary persistent delivery and the presence of
pulmonary hypertension of the respiratory distress.
newborn as a result of chronic  Diagnosis:
in utero stress and thickening  Presence of meconium in
of pulmonary vessels. This amniotic fluid
causes hypoxemia and can  Neonatal respiratory distress
result in pulmonary infections  Characteristic radiological
despite meconium being findings
sterile.  Chest X-ray shows over-inflated,
accompanied by patches of
CLINICAL FEATURES collapse and consolidation:
 Signs and symptoms of mild to  Increased lung volume
moderate respiratory distress in the  Diffuse patchy areas of
first few hours of life that often atelectasis
deteriorates in subsequent 24-48  Parenchymal infiltrates
hours. alternating with hyperinflation.
 Tachypnea  Pneumothorax (this is due to
 End expiratory grunting ball valve effect of meconium
 Nasal flaring depending on the severity of
 Intercostal recessions the clinical picture) or
 Barrel chest pneumomediastinum may
 Cyanosis occur in 25% of patients
 Auscultation: rales and rhonchi
 Some eventually develop severe
respiratory failure with severe
hypoxemia and cyanosis.

198
 pulmonary hypertension of the
newborn) and if the meconium
aspiration syndrome is severe
mechanical ventilation or
ECMO (extracorporeal
membrane oxygenation) may
be necessary.
 It is acceptable practice to start
antibiotic after obtaining blood
culture.
Figure 16: Meconium aspiration syndrome. Note
hyperexpansion of lungs and heterogenous opacities in COMPLICATIONS
right lung (Image adapted from Essentials of Pediatrics 8th
Ed)  Persistent pulmonary hypertension
of the newborn (PPHN).
MANAGEMENT  Chemical pneumonitis which is
 Prevention is of paramount complicated by
importance. bronchopneumonia.
 Best approach to meconium  Long term reactive airway disease
stained amniotic fluid is combined
obstetric and pediatric approach NEONATAL JAUNDICE
and may include:
 Suctioning on the perineum NEONATAL JAUNDICE
 Direct suctioning of the  This is yellowish discoloration of
tracheal via endotracheal mucous membranes and skin as a
intubation result of increased bilirubin levels.
 If the baby is born in a good  It usually occurs during the first
condition and is crying no week of life and is most frequently
suction is needed. However, if caused by indirect (unconjugated)
the baby is in a poor condition hyperbilirubinemia that is
or not making any respiratory physiologic in nature.
effort the oropharynx and the  Visible jaundice occurs in the
vocal cords should be neonate when serum bilirubin
suctioned under direct vision levels exceed 5mg/dl (85
before starting intermittent micromol/dl).
positive pressure ventilation.  Neonatal jaundice is important as:
 Generally, oxygen is required  It may be a sign of another
(to prevent persistent disorder e.g. hemolytic

199
 anemia, infection, metabolic markedly shorter than that of
disease, liver disease an adult (120 days)
 Unconjugated bilirubin can  Hepatic bilirubin metabolism
deposit in the brain, is less efficient in the first few
particularly the basal ganglia days. (Immature liver
causing kernicterus (bilirubin conjugation system- Delayed
encephalopathy- activity of the hepatic enzyme
choreaoathetoid cerebral glucuronyl transferase, Low
palsy) as well auditory nucleus concentration of the binding
(deafness). protein ligandin in the
hepatocytes)
CLASSIFICATION OF  Increased enterohepatic
JAUNDICE circulation due to sterile gut.
PHYSIOLOGIC JAUNDICE  Clinical features:
 This is benign and self-limited  Jaundice
indirect hyperbilirubinemia that  Elevated indirect bilirubin
typically resolves by the end of the levels:
first week of life and requires no o Peak serum
treatment. concentrations in normal
 Visible jaundice usually appears full-term infant reaches 5-
between 24-72 hours of age. 16 mg/dl at around 3-4
 It increases in severity until day 4- days of life.
5 and gradually falls and o In preterm infants, the
disappears by day 10. peak bilirubin is reached
 Over 50% of all newborn infants after 5-7 days and may
become visibly jaundiced because: take 10-20 days before
 There is marked physiological decreasing.
release of hemoglobin from  Physiologic jaundice is a diagnosis
the breakdown of red blood of exclusion therefore every
cells because of high Hb jaundice baby should be carefully
concentration at birth. (high evaluated to rule out pathological
red cell mass at birth due to the causes.
relatively hypoxic
environment)
 The red cell life span of a
newborn infant (70-80 days) is

200
NONPHYSIOLOGIC extensive bruising and concealed
JAUNDICE hemorrhage.
 This is jaundice that is secondary  Hemolysis may be secondary to
to a pathophysiologic cause and it antibodies, or defects in the red
may be classified as: cells structure or enzymes.
 Indirect hyperbilirubinemia:  Early jaundice, secondary to
this is an elevated bilirubin in hemolysis from incompatible
which the conjugated or direct blood group like Rh
component is <20% of the isoimmunisation is a major cause
total bilirubin. of hemolytic jaundice although the
 Direct hyperbilirubinemia: incidence is declining with anti-D
conjugated or direct bilirubin prophylaxis in Rh negative
is >20% of total bilirubin mothers.
level. This is always  The jaundice secondary to ABO
pathologic in neonates. isoimmunisation is not severe
 There is no clear cut demarcation enough to require exchange blood
between pathological and transfusion. Other minor blood
physiological jaundice. group isoimmunisation can also
 Total serum bilirubin (TSB) have produce neonatal jaundice.
been arbitrarily defined as  The red cell defects such as
pathological if it exceeds 5mg/dl hereditary spherocytosis, glucose-
on first day, 10mg/dl on second 6-phosphate dehydrogenase
day or 15mg/dl thereafter in term deficiency, pyruvate kinase
babies. deficiency are not common but
 If the jaundice appears on the first important particularly when there
day of life even in preterm babies, is no obvious cause to explain the
it is deemed pathological and presence of jaundice or hemolysis.
severe enough to require DIFFERENTIAL DIAGNOSIS
intervention.
INDIRECT
 The same applies if it persists
beyond the usual period, it needs HYPERBILIRUBINEMIA
investigation and treatment.  Possible diagnosis includes:
 Early jaundice is generally  Physiologic jaundice
secondary to hemolysis although it  Increased RBC load from
may be secondary to infection, bruising: Cephalohematoma,
Caput saccedaneum, birth
trauma

201
 Excessive bilirubin production decreased excretion of
from hemolysis: spherocytosis, bilirubin in the stool.
elliptocytosis, pyruvate kinase  Breast milk jaundice
deficiency, glucose 6 o Typically occurs after the
phosphate dehydrogenase first week of life and is
deficiency, ABO-Rh likely related to breast
incompatibility. milk’s high levels of beta-
 Defective conjugation of glucuronidase and high
bilirubin by the liver lipase content. Elevated
o Crigler-Najar syndrome bilirubin is highest in the
(Deficiency of glucuronyl 2nd and 3rd weeks of life
transferase) and lower levels of
 Crigler-Najar Type 1: bilirubin may persist until
very severe and 10 weeks of life.
affected individuals  Inborn errors of metabolism:
can die in childhood hypothyroidism
due to kernicterus.  Upper GI obstruction
This is an absolute (increased enterohepatic
deficiency of recirculation): pyloric stenosis,
glucoronyl transferase duodenal stenosis, annular
 Crigler-Najar Type 2: pancreas
This is less severe and
due to a relative
DIRECT
deficiency of HYPERBILIRUBINEMIA
glucoronyl transferase  Possible diagnosis includes:
o Gilbert’s syndrome:  Obstruction of the
 Breastfeeding jaundice hepatobiliary tree: choledochal
o Typically occurs during cysts
the first week of life with  Obstructive jaundice
increased bilirubin levels secondary to biliary atresia
and is usually related to  Neonatal infection: sepsis
suboptimal milk intake.  Neonatal hepatitis: HAV,
Poor intake leads to HBV, HCV, EBV, TORCH
weight loss, dehydration infections, Varicella, Herpes,
and decreased passage of Tuberculosis
stool, with resultant o TORCH= Toxoplasmosis,
Other (syphilis), rubella,

202
 cytomegalovirus, herpes o Galactosemia
simplex virus o Hereditary fructose
 Cystic fibrosis intolerance
 Cholestasis associated with o Tyrosinemia
parenteral nutrition o Alpha 1 antitrypsin
 Metabolic disorders: deficiency

Figure 17: Differential Diagnosis of Indirect and Direct Hyperbilirubinemia (Image courtesy of BRS Pediatrics-2005)

 Hemoglobin is broken down to

the heme part and the globin part.
METABOLISM OF
 The globin part is broken
BILIRUBIN down to amino acids that are
 Bilirubin is as a result of recycled.
hemolysis of RBCs.  The heme part is broken
 Recall, RBCs have the pigment down by heme oxygenase to
hemoglobin which consists of oxy-heme (a closed
Heme (with iron) and the globin tetrapyrrolic ring with iron)
part.  Oxy-heme is further
 RBCs after 120 days (70 days in converted by heme reductase
infants) are broken down in the to biliverdin (an open
reticuloendothelial system tetrapyrrolic ring without
(spleen) by macrophages. iron)

203
  The iron removed from the pharmacologic agents such
heme binds to transferrin and as phenobarbital.
is transported to the liver to  In the hepatocytes bilirubin is
be stored where it binds to conjugated as it is bound to
ferritin. glucuronic acid. Conjugated
 Biliverdin is converted by bilirubin is also called Direct
biliverdin reductase to bilirubin, while unconjugated
bilirubin bilirubin is called indirect
 Bilirubin is toxic to tissue bilirubin. The enzyme important
therefore; it is transported to the for glucuronidation is Glucuronyl
liver as it binds to albumin. transferase.
 Bilirubin is taken up by  Conjugated bilirubin is then
hepatocytes at their sinusoidal secreted into bile which is stored
surface. This uptake is very rapid. and concentrated in the gall
 In the hepatocytes bilirubin is bladder.
bound to cytoplasmic proteins:  Bile is then secreted into the first
ligandins and Z protein. part of the duodenum from the
 Ligandins are a group of common bile duct.
enzymes that represent 2% of  In the intestines bilirubin is
cytosolic proteins deconjugated by
 Z proteins bind fatty acids. bacterial/intestinal enzyme Beta-
 The primary function of glucuronidase to form free
these proteins is that of bilirubin.
avoiding the reflux of free  Bilirubin is converted by bacterial
bilirubin into the blood dehydrogenase to urobilinogen (a
because the time lapse colorless substance)
between uptake of bilirubin  The bulk of urobilinogen,
and conjugation is relatively bilirubin and urobilin is excreted
long. in feces (by conversion to
 Ligandin concentrations are stercobilinogen and stercobilin
low at birth but rapidly which gives feces their
increase over the first few characteristic orange-yellow
weeks of life. Ligandin color).
concentrations may be  Small amounts of bilirubin and
increased by the urobilinogen are reabsorbed into
administration of the blood stream.

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 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:  To change mmol/dl multiply by
 Jaundice appears at <24 hours 17.
of age
INVESTIGATIONS
 Bilirubin rises >5-8mg/dl in a
 To evaluate indirect
24-hour period
hyperbilirubinemia:
 The rate of rise of bilirubin
 Full blood count
exceeds 0.5mg/dl per hour
 Reticulocyte count
(suggestive of hemolysis)
 Smear (for hemolysis)
KRAMER STAGING  Evaluation of sepsis may be
 This is a clinical assessment of indicated
jaundice.  To evaluated direct bilirubinemia:
 Progression is cephalocaudal.  Hepatic ultrasound (to evaluate
 The body is divided into 5 zones: for choledochal cyst)
 Zone 1: 5mgl/dl  Serologies: viral hepatitis
 Zone 2: 10mg/dl  Radioisotope scan of the
 Zone 3: 12mg/dl hepatobiliary tree
 Zone 4: 15mg/dl  Evaluation of sepsis may be
 Zone 5: >15mg/dl indicated.
MANAGEMENT
 Treatment of jaundice is based on
the gestational age of the baby and
the postnatal age.
 Babies who are preterm should be
treated at a lower serum bilirubin
level than a baby born at term.

205
 Several other factors such as well-  The estimated blood volume of
being of child, severity of a term neonate is 85ml/kg
hemolysis and presence of sepsis while that of a preterm neonate
should be taken into consideration is about 100ml/kg
in determining the threshold for  Adequate hydration is important as
treatment. dehydration will increase the
 Serum bilirubin assessments, serum bilirubin level.
observation and reassurance are
COMPLICATIONS
appropriate for physiologic
jaundice.  Kernicterus (bilirubin
encephalopathy) is the main
 Phototherapy and exchange
complications.
transfusion are the mainstay of
treatment.  Mainly the basal ganglia and
auditory nucleus are affected, but
 Phototherapy (with blue light,
any part of the brain is at risk.
wave length 450-500 nm): very
effective and acts by:  Indirect bilirubin at sufficiently
 Photo-isomerisaton: creates high concentrations can pass
water-soluble photoisomers of through the blood-brain barrier and
indirect bilirubin: produce irreversible damage.
o Polar configurational  Bilirubin most frequently localizes
isomers (Z, E- bilirubin) in the basal ganglia, hippocampus
o Structural isomers and some brainstem nuclei.
(lumirubin)  This occurs when the level of
 Photo-oxidation unconjugated bilirubin exceeds the
 During phototherapy ensure the albumin binding capacity of
eyes are covered to protect them bilirubin of the blood.
from exposure to light  Clinical features include
 Side effects: photodermatitis,  Lethargy and poor feeding
temperature instability, loose (acute manifestation)
stools and dehydration  In severe cases there is
 Exchange transfusion is performed irritability, increased muscle
for rapidly rising bilirubin levels tone causing the baby to lie
secondary to hemolytic disease. with an arched back
 The aim is to remove antibody (opisthotonos)
coated red cells, antibodies in  Choreoathetoid cerebral palsy:
serum, reduce and correct due to damage to the basal
anemia. ganglia

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  Hearing loss: sensorineural  Oculomotor paralysis
deafness
 Opisthotonus
 Seizures

Figure 18: Summary of causes of neonatal jaundice (Image adapted from Pediatrics at a Glance)

207
 208

Figure 19:Approach to Jaundiced Pediatric patient (Image adapted from Pediatrics at a Glance)

CHAPTER 16: MISCELLANEOUS

MISCELLANEOUS
FAILURE TO THRIVE  In children with FTT, weight is
 This is a term used to describe a usually affected before length,
growth rate of less than expected which is usually affected before
for a child less than 5 years. head circumference (head
circumference is initially spared in
 Failure to thrive usually refers to
FTT).
weight below 3rd or 5th centile or a
failure to gain weight over a period ETIOLOGY
of time or a change in rate of  Causes include both:
growth that has crossed 2 major  Inorganic or psychosocial
centiles e.g. 75th to 50th over a etiologies (Most common
period of time. cause-80%) i.e. a disturbed
 Failure to thrive is a descriptive parent-child bond that results
term rather than diagnosis. in inadequate caloric intake/
 FTT should be distinguished from retention or emotional
isolated short stature, in which deprivation.
height is the most abnormal  Organic etiologies suggest
growth parameter. underlying organ system
 pathology, infection,  Craniofacial problems
chromosomal disorders or  Tracheoesophageal fistula
systemic illness.  Gastroesophageal reflux
disease
INORGANIC CAUSES  GI obstruction (pyloric
 Poor formula preparation (feeding stenosis, malrotation,
diluted formulae) Hirschprung’s disease)
 Poor feeding techniques  Acute and chronic diarrhea
(misperception or lack of  Inflammatory bowel disease
knowledge about diet and feeding)  Celiac disease
 Child abuse and neglect  Cystic fibrosis
 Parental immaturity  Renal
 Maternal depression  Chronic renal failure
 Alcohol and drug use  Renal tubular acidosis
 Marital discord  Recurrent urinary tract
 Mental illness infection
 Family violence  Fanconi syndrome
 Poverty  Hematology and oncology
 Isolation from support system  Iron-deficiency anemia
 Malignancy
ORGANIC CAUSES  Endocrine: hypothyroidism,
 Neurological: diabetes mellitus & rickets
 Cerebral palsy  Genetic, congenital and metabolic
 Mental retardation  Fetal alcohol syndrome
 Hydrocephalus  Inborn errors of metabolism
 Intracranial tumors  Chromosomal abnormalities
 Generalized muscle weakness  Immunologic- immunodeficiency
 Increased or decreased tone status
 Cardiac: congenital heart disease  Infectious:
 Pulmonary  Tuberculosis
 Bronchopulmonary dysplasia  HIV
 Cystic fibrosis  Hepatitis
 Asthma  Toxin: lead poisoning
 Gastrointestinal:

209
 210

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
 211

EVALUATION  Full blood count with ESR

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

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  Characterized by hypotension, low
cardiac output and inadequate
PEDIATRIC SHOCK tissue perfusion.
 Shock is a clinical state
characterized by inadequate IRREVERSIBLE SHOCK
delivery of oxygen and metabolic  Characterized by cell death and is
substrate to meet the metabolic refractory to medical treatment
demands of tissue.
 It may present with normal or CLASSIFICATION BY CAUSE
decreased blood pressure. HYPOVOLEMIC SHOCK

CLASSFICIATION  This is the most common cause of

 It may be classified by: shock in children and is caused by
 Degree of compensation any condition that results in
 The cause decreased circulating blood such
as hemorrhage or dehydration e.g.
CLASSIFICATION BY from acute gastroenteritis
DECOMPENSATION  The amount of volume loss
 Shock may be: determines the success of
 Compensated compensatory mechanisms such as
 Decompensated endogenous catecholamines, in
 Irreversible maintaining blood pressure and
cardiac output.
COMPENSATED
 Volume losses greater than 24%
 Characterized by normal blood result in decompensated shock
pressure and cardiac output with
CARDIOGENIC SHOCK
adequate tissue perfusion but
maldistributed blood flow to  Occurs when cardiac output is
essential organs. limited because of primary cardiac
dysfunction.
DECOMPENSATED

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 Causes include:  This is secondary to an
 Dysrhythmias e.g. inflammatory response to invading
supraventricular tachycardia microorganisms and their toxins
 Congenital heart disease e.g. and results in abnormal blood
any lesion that impairs left distribution.
ventricular outflow  There are 2 clinical stages:
 Cardiac dysfunction after  Hyperdynamic stage:
cardiac surgery characterized by normal or
 Clinical features are the signs and high cardiac output with
symptoms of congestive heart bounding pulses, warm
failure. extremities and a wide pulse
pressure.
DISTRIBUTIVE SHOCK
 Decompensated stage: follows
 Associated with distal pooling of the hyperdynamic stage if
blood or fluid extravasation and is aggressive treatment has not
typically caused by anaphylactic or been initiated. It is
neurogenic shock as a result of characterized clinically by
medications or toxins. impaired mental status, cool
 Anaphylactic shock: characterized extremities and diminished
by acute angioedema of the upper pulses.
airway, bronchospasm, pulmonary
edema, urticarial, and hypotension
because of extravasation of DIAGNOSIS
intravascular fluid from permeable  Recognition of shock may be
capillaries. difficult because of the presence of
 Neurogenic shock typically compensatory mechanisms that
secondary to spinal cord may prevent hypotension until
transection or injury is 25% of intravascular volume is
characterized by a total loss of lost.
distal sympathetic cardiovascular  The index of suspicion for shock
tone with hypotension resulting must be high
from pooling of blood within the  History:
vascular bed.  Severe vomiting and diarrhea
 Trauma with hemorrhage
 Febrile illness, especially in an
SEPTIC SHOCK immunocompromised patient
 Symptoms of CHF

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  Exposure to a known allergic  Coagulation factors: to evaluate
antigen for DIC which may accompany
 Spinal cord injury shock
 Physical examination:  Toxicology screen: to evaluate for
 Blood pressure may be normal a poisoning which could cause
in the initial stages of shock.
hypovolemic and septic shock.
 Tachycardia almost always MANAGEMENT
accompanies shock and occurs  Initial resuscitation involves the
before blood pressure changes ABCs:
in children.  Supplemental oxygen
 Tachypnea may be present as a  Early endotracheal intubation
compensatory mechanisms for to secure the airway and
severe metabolic acidosis decrease the patient’s energy
 Mental status changes may expenditure
indicate poor cerebral  Vascular access with
perfusion. appropriate fluid resuscitation.
 Capillary refill may be Initial fluid- 20ml/kg bolus of
prolonged cool and mottled normal saline or Ringer’s
extremities. lactate solution
 Peripheral pulses may be  To restore intravascular volume,
bounding in early septic shock intravenous crystalloid or colloid
solution should generally be used
LABORATORY STUDIES before administration of inotropic
 Full blood count: to assess for and vasopressor agents.
blood loss and infection.  Inotropic and vasopressor
 Electrolytes: to assess for medications e.g. dobutamine,
metabolic acidosis and electrolyte dopamine, epinephrine are
abnormalities. indicated if the blood pressure
 Blood urea nitrogen and increase in response to fluid is
creatinine: to evaluate renal inadequate.
function  Metabolic derangements such as
 Calcium and glucose: assess for metabolic acidosis, hypocalcemia
frequently encountered metabolic or hypoglycemia should be treated.
derangements  Other considerations:
 Broad-spectrum antibiotics for
septic shock

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  Blood products for
hemorrhage
 Fresh frozen plasma for DIC

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

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?

216
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. Look at this patient and describe any abnormality that you are able to see.

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

217
 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.

218
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?

219
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?

220
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

221
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?

222
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?

223
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?

224
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?

225
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?

226
STATION 9

1. What is the above equipment called?

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

227
4. Label the female genitalia below?

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?

228
 b) List four investigations that must be done in this child to help you confirm
the diagnosis?

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?

5. George, a 6 month old infant is admitted with pneumonia (first occurrence) and
is able to feed well. Her mother tested positive for HIV. Write down 5 questions
you would ask the mother to assess the risk of HIV infection in George.

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?

229
 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 wat 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 reveal 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) What 5 things affecting this child would likely cause mortality within 24
hours of admission unless taken care on admission?

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

230
 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
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?

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

231
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.

12.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

13.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?
b) List 4 investigations that you are going to do in order to confirm the
diagnosis

14.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?

232
 b) List 3 disease condition that would possibly have caused this condition
c) Indicate 2 cardinal investigations that would help you manage this child.

15.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
d) What plan are you going to apply in rehydrating Joanna?
e) What fluid are you going to use in treating this little girl?
f) Give details of the plan you are going to use in rehydrating Joanna

16.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?

17.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

18.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
c) List down 3 investigations to ascertain your diagnosis

19. 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?

233
 c) What investigations would you do to help ascertain your diagnosis?

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

234
2. Urinalysis was done and results as follows: Ketones 1+, Glucose nil, Protein 4+
and Blood 1+, What is your diagnosis?
Answer: Nephrotic syndrome
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)

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6. What are the causes of hypertension in children?
Answer:
 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

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4. Examiner asks candidate ‘What are the most likely bacterial causes’?
Answer:
 Escherichia coli
 Listeria monocytogenes
 Group B streptococcus
 Staphylococcus aureus
 Streptococcus pneumoniae
STATION 3
1. Look at this patient and describe any abnormality that you are able to see.
Answer: Finding(s): wasting

2. After candidate has described the finding(s), Examiner shows him/her

urinalysis results and asks candidate “what questions would you ask the
caregiver/patient in order to arrive at a diagnosis?”
Urinalysis results: Glucose 3+, Protein 1+, Ketones- neg, Nitrites-neg, Blood-
1+
Answer:
 Questions
a) Drinking too much water?
b) Passing a lot of urine? Even at night?
c) History of weight loss?
d) Eating too much?
e) Recurrent infections?
f) Family history of DM?
3. Examiner asks candidate “what is the possible diagnosis based on urinalysis?”
Answer: diabetes mellitus
4. Examiner asks candidate “How would you manage his patient if they came in
DKA?”
Answer:
 IV fluids
 Soluble insulin
 Potassium
 Treat any infection
5. Examiner asks candidate “what is the major life-threating complication that
may arise during the management of DKA and how the patient will present?”
Answer:

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

1. Describe your findings.

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)

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 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:
 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?

239
 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.

1. Describe the main abnormality seen.

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 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)
STATION 4

1. What abnormality can you see on the CXR?

241
 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
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?

242
STATION 6
1. Look at this X-ray and write down the most striking feature you see

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

243
 Answer:
 Cerebrovascular accident
 Subarachnoid hemorrhage
 Retinopathy
 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

244
INSTRUMENTS
STATION 1

1. What is the above equipment called?

Answer: Spacer and inhaler
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

245
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

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?

246
 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
 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.

247
  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

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?

248
 Answer: Nasogastric tube
2. What are the indications for its use?
Answer:
 Feeding
 Rehydration
 Emptying the stomach
 Gastric lavage
 Administration of medication
STATION 7
1. What is this equipment and explain its use?

249
 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:
 Endo-tracheal tube
 Laryngoscope
 Ambu-bag

250
  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

STATION 9

251
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?

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: 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 with CSF analysis and culture
 Urinalysis, Urine microscopy, culture and sensitivity

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 previous pregnancies?
 Is Child passing stool, diarrhea, color of stool, color of urine?
 Rhesus and ABO blood group of mother, father and infant?

b) Write down 2 differential diagnoses

Answer:
 Rhesus isoimmunisation
 ABO incompatibility
 Glucose phosphate dehydrogenase deficiency

c) Write down your management

Answer:

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  Management is dependent on cause however:
 Admit
 Investigate: direct and indirect bilirubin, ultrasound
 Hydrate with IV fluids
 Phototherapy (blue light, wave length 450-500nm)
o Ensure the eyes and genitalia are covered
o Phototherapy machine should be about 20-45cm from infant
 Exchange transfusion for rapidly rising bilirubin levels secondary to
hemolytic disease

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 of protein/carbohydrates= 4kcal
1g of fat= 9kcal

Total protein= 10g

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

Total calories of protein= 10 x 4= 40kcal

Total calories of carbohydrates= 115 x 4= 460kcal
Total calories of fat= 10 x 9= 90kcal

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

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 give 590kcal

254
 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:
 Sunken anterior fontanelle
 Sunken eyes
 Dry mucus membranes
 Skin pinch goes back very slowly
 Lethargic/comatose and unable to drink
 No tears on crying
 Tachycardia

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

Answer: more than 10% with severe dehydration

5. George, a 6-month old infant is admitted with pneumonia (first occurrence) and
is able to feed well. Her mother tested positive for HIV. Write down 5 questions
you would ask the mother to assess the risk of HIV infection in George.
Answer:
 When did the mother test positive, before, during or after breast feeding?
 When she was found positive did she start any treatment immediately and
did she take the medication?
 What was the CD count and viral load during pregnancy and just before
birth?
 What was the mode of delivery: vaginal or cesarean section?
 Was the baby put on any prophylactic antivirals?
 Is George breastfeeding and is mother still taking antivirals?

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.

255
 a) What is the complete diagnosis of this medical conditions in this girl?
Answer: acute mitral regurgitation

b) Mention two other signs that could be found in this patient consistent with
the above diagnosis?
Answer:
 Displaced apex beat downward and outward with forcible apex and
hyperkinetic precordium.
 Systolic thrill with an S3 heart sound audible at the apex.

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: Vaso-occlusive crises in a possible sickle cell disease patient

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

Answer:
 Hemoglobin electrophoresis and Sickling test
 FBC and peripheral smear

c) What is the most likely wat 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 wither
carriers or sufferers of the condition).

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

Answer:
 Cerebrovascular accident

256
  Acute chest syndrome
 Respiratory failure
 Myocardial infarction
 Cholelithiasis
 Isosthenuria
 Priapism and impotence
 Chronic osteomyelitis
 Avascular necrosis of head of femur
 Delayed Growth development and musculoskeletal deformities
 Ulcers

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 reveal 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 (<11.5cm)
 Weight/height <-3SD
 Pedal edema and signs of dehydration/shock
 Eye signs: bitot spots/dry conjunctiva, corneal ulceration
 Oral changes: cheilosis, angular stomatitis, papilla atrophy
 Skin changes: desquatation, hypo- or hyperpigmentation, ulceration,
exudative lesions
 Flag-post hair (thin, sparse, brittle and color turns dull brown or reddish)
 Nail changes: brittle, spoon shaped nails
 Decreased subcutaneous tissue (legs, arms, buttocks and face)

257
 c) 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

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 easy fatigability, 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 count
 Clotting profile: aPTT (activated partial thromboplastin time), bleeding
time, prothrombin time
 Peripheral smear
 Bone marrow aspirate
 Von Willebran cofactor assay

b) What are 2 most likely differential diagnosis that would explain the patient’s
condition
Answer:
 Thrombotic thrombocytopenic purpura
 Von Willebrand’s disease

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:
 Altered mental state

258
  Hemiplegia
 Paresthesia
 Visual disturbances
 Apahsia
 Jaundice

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
 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
 Lymph node biopsy
 Peripheral smear
 Serum albumin and liver enzymes

c) If you are to stage this condition, what stage of disease is the child in.
Answer: HIV stage 2
11) A 2 year old girl presents to your hospital and after investigations the CSF
revealed the following results
Appearance: cloudy

259
WBC: 120 cells/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
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 (normal is 15 to 60mg/100 ml)
 Can’t comment on sugar level because serum glucose not given, however
when compared to normal range (2.5 to 4.4 mg/dl) glucose level is low

b) What is the causative organism?

Answer: Haemophilus influenzae type B

c) What specific measure would you have taken to prevent this illness in the
child?
Answer: Immunization against Hemophilus influenza

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

Answer:
 Hearing loss
 Learning disability/ mental retardation
 SIADH
 Seizures
 Hydrocephalus
 Brain abscess
 Cranial nerve palsy
 Focal neurological deficits

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

260
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
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 (most likely secondary to hook worm infestation)
 Sideroblastic anemia
 Thalassemia

12.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:

261
  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 the bronchioles. There may be bronchiolar spasms which also
further increased the obstruction. Obstruction affects expiration more than
inspiration.

13.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:
 Coomb’s test: direct and indirect
 Serum bilirubin: total, conjugated and unconjugated
 Full blood count
 Peripheral smear

14.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: Meningitis

b) List 3 disease condition that would possibly have caused this condition
Answer:
 Acute bacterial meningitis

262
  Cerebral malaria
 Encephalitis

c) Indicate 2 cardinal investigations that would help you manage this child.
Answer:
 Full blood count with differential count
 RDT and malarial parasite slide
 Blood glucose
 Urea and serum electrolytes
 Lumbar puncture for CSF biochemistry, CSF microscopy (Gram stain,
Zehl neelsen and India ink) and CSF culture & sensitivity

15.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

c) Give details of the plan you are going to use in rehydrating Joanna
Answer:
 Give 100ml/kg in 3 hours (1400ml in 3 hours) of Ringer’s lactate
intravenously
- Give 30ml/kg in 30 minutes (420ml in 30 minutes)
- After, give 70ml/kg in 2 hours 30 minutes (1190ml in 2 hours 30
minutes)
 Reassess every 10-15 minutes, if hydration status is not improving give IV
drip more rapidly, also watch out for overhydration. Re-assess after 3
hours and re-classify hydration status, if hydrated switch to appropriate
plan.
 Put on maintenance fluid
- Total fluids= (100 x 10) + (50 x 4)
- Give 1200ml in 24 hours

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16.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:
 Normal weight for a 4 year old child= 8 + (2 x 4)= 16kg
 Hamaundu’s weight= 8.5kg
8.5
 W/A percentage of normal= × 100= 53.125%
16
 Hamaundu has weight/age <60% with edema so he has Marasmic
kwashiorkor

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 of F-75 is needed

17.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 (salmonellosis)
 Abdominal tuberculosis
 Typhus
 Brucellosis
 Leukemia

b) What is your most likely diagnosis?

Answer: Enteric fever (salmonellosis/typhoid fever)

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 c) List investigations you would carry out
Answer:
 Stool microscopy/ culture/ sensitivity
 Full blood count
 Peripheral smear
 Urea, electrolytes, creatinine and liver enzymes
 Widal antigen test
 Abdominal X-ray
 Abdominal Ultrasound
 Bone marrow culture

18.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 failure

b) Write down 2 more symptoms that may be associated with this condition
Answer:
 Polyphagia (excessive eating)
 Polydipsia (excessive thirst and drinking of water)

c) List down 3 investigations to ascertain your diagnosis

Answer:
 Random blood sugar/ Fasting blood sugar
 Urinalysis
 Plain abdominal X-ray
 Ultrasound of kidney and bladder
 Urodynamic studies

19. 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.

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a) What further questions would you ask to help determine the diagnosis?
Answer:
 Any recurrent abdominal pain?
 Any joint swelling especially of the painful swelling of digits of hands and
feet?
 Any history of yellowing of eyes?
 Any recurrent respiratory infection?
 Any personal history of sickle cell disease?
 Any family history of sickle cell disease?
 History of blood transfusion?

b) Give 2 differential diagnoses?

Answer:
 Sickle cell disease
 Osteomyelitis (acute)

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

Answer:
 Sickling test/solubility test
 Hemoglobin electrophoresis
 Full blood count
 Peripheral smear

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INDEX

Delayed hypersensitivity reaction · Head Circumference · 7

A 146 Heaf tests · 152
Development · 5, 10, 11, 16 Height · 6
Developmental Milestones · 11 HIE · See Hypoxic ischemic
Abdominal Examination · 31
Direct laryngoscopy · 42 encephalopathy
Acute chest syndrome · 87
Disseminated tuberculosis · 147 Hirschsprung’s enteritis · 196
Acute Dactylitis · 87
Dive reflex · 185 History · See Pediatric History
Adenovirus · 46
Domains of Development · See Howell-Jolley bodies · 89
Adolescence · 4
Developmental Milestones Hydrocephalus · 34
Age range of children · 4
Down syndrome · 34 Hydrocephalus ex vacuo · 34
Annular pancreas · 202
Down Syndrome · 34 Hydroxyurea · 93
Aplastic crisis · 86
Hyperbilirubinemia · 199
Avascular necrosis · 87
Hyperhemolytic crisis · 86
E Hyperhemolytic Paradigm · 89
Hypoxic ischemic encephalopathy ·
B
Early-onset sepsis · 141 184
Epiglottitis · 41
Bacterial Tracheitis · 45
Epithelioid cells · 146
BMI · See Body Mass Index
Erythrovirus B19 · 88 I
BODY MASS INDEX · 9
Bronchiolar spasm · 46
IGRA · See Interferon-gamma
Bronchiolitis · 45, 47, 48
F release assays
Immunization · 34
Failure to thrive · 208 Infantometer · 6
C
Fine Motor Skill Development · 11 Interferon-gamma release assays ·
Fine Motor Skills · 13 152
Caput saccedaneum · 201
Frankfort plane · 7 Intrauterine growth restriction ·
Cardiovascular System
FTT · See Failure to thrive 170
Examination · 30
Isosthenuria · 85
Caseous necrosis · 146
IUGR · See Intrauterine growth
Cephalohematoma · 201
Chest Circumference · 8
G restriction

Chiari type II Malformation · 34

Codoctytes · See Target Cells Gados channel · 86
General Examination · 26 K
Cognitive Development · 15
Common cold · See Coryza GenXpert · 152
Ghon complex · 146 Klinefelter · 34
Communicating Hydrocephalus · 34
Congenital Aqueductal Stenosis · 34 Ghon focus · 146
Congenital tuberculosis · 145 Gilbert’s disease · 202
Coryza · 40 Granuloma · 146 L
Crigler-Najar syndrome · 202 Gross Motor Development · 11
Croup · 43 Growth · 5 Langhans’ giant cells · 146
Cystic fibrosis · 60 Laryngotracheobronchitis · See
Croup
H Latent tuberculosis · 146
D Late-onset sepsis · 141
Hand and foot syndrome · 87 Length · See Height
Dandy-Walker Malformation · 34 Hb electrophoresis · 89

267
 Physiologic jaundice · 200 Steeple sign · 44
M Pneumatosis intestinalis · 192
Pneumonia · 50
Mantoux test · 150, 152
Pneumoperitoneum · 193 T
MAS · See Meconium aspiration
Postnatal period · 4
syndrome
Prematurity · 172 Tachoids · 86
Meconium aspiration syndrome ·
Priapism · 87 Target cells · 89
196
Primary complex of Ranke · See TB · See Tuberculosis
Mid Upper Arm Circumference · 9
Ghon complex Technetium 99 · 91
Miliary tuberculosis · 146
Primitive reflexes · 12 Teenager · 4
Monoglism · See Down syndrome
Ptosis · 91 Transcranial Doppler Ultrasound ·
Motor Development · 12
91
Mycolic acid · 145
Transient tachypnea of the
Mycoplasma pneumoniae · 46
R newborn · 180
Trisomy 21 · See Down syndrome
RDS · See Respiratory distress TTN · See Transient tachypnea of
N
syndrome the newborn
Respiratory distress syndrome · 183 Tuberculosis · 144
Nasopharyngitis · See Coryza
Respiratory Syncytial Virus · 46 Turner syndrome · 34
NEC · See Necrotizing enterocolitis
Respiratory System Examination ·
Necrotizing enterocolitis · 144, 191
29
Neonatal Jaundice · 199
Rhinovirus · 46 V
Neonatal Sepsis · 140
Nervous System Examination · 33
Vaccination · See Immunization
NICU Orientation · 165
S Vaso-occlusive crisis · 86
Non-Communicating
Ventriculoperitoneal Shunt · 35
Hydrocephalus · 34
Sepsis · 141 Viral croup · 43
Non-Physiologic jaundice · 201
Sepsis Neonatorum · See Neonatal Volvulus neonatorum · 196
Sepsis
Sepsis screen · 142
P W
Septicemia · 140
Sickle cell disease · 85
Paediatrician · 4
Snuffles of congenital syphilis · 40 Weight · 5
Parainfluenza · 46
Spasmodic croup · 43 Wine bottle sign · See Steeple sign
Parvovirus B19 · Erythrovirus B19
Squestration crisis · 86
Pediatric History · 4
Standiometer · 7
Physical Examination · 25

268