Paediatric Booklet by Win Nursing Coaching

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WIN COACHING HALDI GHATI MARG, IN FRONT OF DUCKLING SCHOOL, NEAR JAIPUR GYM, PRATAP NAGAR JAIPUR. 8742073072, 9587958165
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NEONATOLOGY
INTRODUCTION
➢ Neonatal Period
- 1st 28 days of Life
➢ Early Neonatal period
- 1st 7 days of life
- Day of birth - < 7 completed days
➢ Late Neonatal Period
- D7 – 28 days of life
Classification
1. According to their gestational Age (Irrespective of birth weight)
- Term – Born b/w 37 completed weeks to <42 gestational weeks
- Pre Term – Born <37 weeks
- Post Term – born at or beyond 42 weeks of gestation
2. According to birth weight (Irrespective of gestational age)
- LBW (Low birth weight) - < 2500 gms birth weight
- VLBW (Very low birth weight) - <1500 gms birth weight
- ELBW (Extremely low birth weight) - <1000 gms birth weight
3. According to Gestational Age & Birth weight
LFD (large for date) or

LFG (large for gestational age)
AFD (Appropriate for date) or
AFG (Appropriate for gestational age)
SFD (Small for date) or
-
SGA (Small for gestational age)
- SFD or SFG – Birth weight is < 10th Percentile or expected, according to GA.
- AFD or AFG – Birth weight is between 10th – 90th percentile of expected according to gestational
age.
- LFD or LFG – Birth weight is >90th percentile of expected, according to GA.
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Important cause for LFD Neonate

➢ Infant of diabetic mother
➢ Congenital hypothyroidism
➢ Constitutional
➢ Soto’s syndrome / cerebral gigantism
➢ Beckwith widemann syndrome
▪ Hemi hypertrophy
▪ Macroglossia
Normal Term Neonate
➢ Birth weight of an average Indian Baby – 2.8 kg (Ghai – 2.9 kg)
➢ Length 50 cm
➢ US : LS ratio 1.7 – 1.9 : 1
➢ HC 33 – 35 cm
➢ Heart Rate 120 – 140 bpm (110 – 160 bpm)
➢ Respiratory Rate - 40 – 60/min
→ Peripheral cyanosis (Acro cyanosis) Normal
→ Soft systolic murmur
→ Central cyanosis at birth
Abnormal
→ Jaundice at birth / D1
Pre term Neonate

→ Tool used to assess Gestational age of a neonate – Expanded new ballard score (ENBS)
→ Used for 20-44 wks gestation

Characteristics
General Head to TOE
1. Lesser subcutaneous fat 1. Head appears relatively large
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➢ Appears emaciated 2. Anterior fontanelle – large wide OPEN

2. Generalized hypotonia 3. Ear cartilage is poorly formed
➢ Extended posture → soft & deformable ears
3. Skin 4. Breast buds < 5 mm size
➢ Thin, translucent & Friable 5. Genitalia
4. Abdunt lanugo Male – Undescended testes
5. Little vernix caseosa - Poorly formed scrotum
Female – Labia majora widely
- Separated
- Labia minora clearly
- Visible
6. Absent deep creases on the sole
Primitive Neonatal Reflexes
Present at birth (Term) Appears Disappears

Rooting reflex 32 weeks 1 month
Moro’s reflex 28-37 week 5 - 6 month
Palmer reflex 28 weeks 3 month
Grasp reflex 28 week 3 month
ATNR 35 week 5 - 6 month
Present after birth Appears Disappears
STNR 4-6m 8 - 12 m
Parachute Reflex 7-8m Persists throughout life
Landau reflex
Neck righting reflex
✓ ATNR → Asymmetric Tonic Neck Reflex

✓ STNR → Symmetric Tonic Neck Reflex
❖ Q. which reflex helps mother in breast feeding → Rooting reflex
❖ Q. Which reflex is earliest to disappear → Routing reflex
Moro’s Reflex/Embrace Equivalent

Components of a Complete MORO’s reflex
➢ Symmetric abduction & extension of UL along with opening hands followed by flexion & adduction
of UL
• Extension of head & trunk movement of lower limbs crying
➢ Begin to appear 28 wks of gestation
• 1 component to appear
st Opening of hands
➢ MORO’s reflex completely appears 37 wks of gestation.
➢ Disappear at 5 – 6 months (nelson)
[3 – 6 months (op ghai)]
➢ Abnormal persistence beyond 6 months → Cerebral damage
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➢ MORO’s reflex, if once disappears never reappears.

➢ Causes of Absent MORO’s Reflex
a. Stage 3/ severe H/E
b. Down Sx
c. Acute bilirubin Encephalopathy
➢ Causes of Exaggerated MORO’s reflex Stage 1 HIE.
➢ Cause of Asymmetric MORO’s reflex

Neurological causes Skeletal causes
1. Erb’s palsy 1. Fracture clavicle
2. Congenital hemiplegia 2. Shoulder joint dislocation
➢ MC bone to fracture in a neonate ClavicleQ
Important condition in neonates not requiring any specific Rx

Skin & Mucosa
1. MILIA – Colourless papules dlt plugging of sweat ducts
2. Erythema toxicum Neonatorum

• Erythenatuous maculo papualr rash mainly on trunk in 1st week of life.
• dlt immune phenomenon
• Eosinophil filled sterile lesion
3. Mongolian Spots
• Bluish black areas of discolouration
• Mainly on lower back, buttocks
• d/t migration of neural crest cells
4. Strok Bite/Salmon Patch

• Capillary hemangionas
• In between eye brows / nape of neck
5. Epstein Pearls
• Pearl like white lesions
• Hard palate involved
• Epithelial inclusion cysts
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6. Acne Neonatorum – d/t maternal androgens
7. Sub conjunctival hemorrhages
8. Mastitis Neonatorum
• B/L breast engorgement
• In male/female neonates
• D2-3 of life
• d/t effect of maternal hormones
9. Vaginal Bleeding
• Female neonate
• D3-5 of life
• d/t effect of withdrawal of maternal hormones
10. Hymenal Tags – Skin growth near the vaginal opening.
11. Physiological Phimosis

- Phimosis is a condition in which the foreskin of the
penis cannot stretch to allow it to be pulled
12. Physiological weight loss

• Term neonates loose up to 10% of birth weight by 3-5 days, regained by D10 of lifeQ.
• Preterm neonates loose up to 15% of birth weight by 7-10 days, Regained by D15 of lifeQ.
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CEPHAL HEMATOMA CAPUT SUCCEDANEUM

1. Sub periosteal haemorrhage involving 1. D/t edema in the layers of scalp
cranial bones
2. does not cross sutures 2. Can cross sutures
3. May take 24 hrs to appear completely 3. Already present at birth in its maximum size
4. Takes 5-7 weeks to disappear 4. Disappears by 48-72 hrs
5. Predisposes to neonatal jaundice 5. Does not predispose to neonatal jaundice
NEONATAL RESUSCITATION
Neonatal Resuscitation Protocol
• Given by American Academy of pediatrics (October – 2015).
• 10% of neonates need some resuscitation at birth.
• <1% require chest compression & or medications.
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Antenatal counseling
Team Briefing
Protocol Equipment Check
Birth
Term Gestation ? Routine Care

Good Muscle tone ? Yes Provide warmth
Breathing or crying ? Clear airway if required
Baby stays Dry
[60 sec.] with mother Ongoing evaluation
Golden
Minute of Provide worth
Position
life
Clear airway if required
(1st minute Dry
of Life) Stimulate
Heart Rate <100/min or Yes Labored breathing or

Apnea or Gasping persistent cyanosis
Yes
Clear airway
Start PPV Check SPO2
Check SPO2 Considered CPAP
Consider cardiac monitor Supplement O2 if required
HR < 100/min 30 sec

No Post Resuscitation care present
Check chest movement Team debriefing
Take ventilation corrective
steps Yes
May intubate the baby
No Heart Rate <60/min

UVC – Umbilical venous catheterization
Start chest compressions - Vascular axis of choice during
Co-ordinate with PPV
Use 100% O2 neonatal Resuscitation
Use cardiac monitor
Intubate the baby
Consider UVC
Inj. Adrenaline (up to 3 time)
HR < 60/min
Consider
Yes
No impmrovement
➢ Hypovolemia – poor peripheral perfusion, feeble pulse

Rx by Fluids
- Normal saline (Fluid of choice)
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- O Group Rh negative blood

- Ringer lactate no longer recommended
➢ Pneumothorax
Q. Suction of Airways → mouth f/b Nose
➢ Usual size of suction catheter – 12 or 14 F
➢ Usual pressure – 80 mm Hg or 100 cm H2O
➢ Recommended temp. of delivery room 25⁰C
➢ CPAP – Continuous positive airway pressure
➢ PPV - Positive pressure ventilation (Bag & mask ventilation).
➢ Team Debriefing → question the team members how well they followed the protocol so that they do
even batter in next time.
➢ Post Resuscitation Care

o Take the baby in nursing or an ICU
o Score IV fluid
o Monitor condition of babys
o Maintain normal temp ,normal Blood glucose
o & treat according to the condition of baby.
Recommended Target Saturation of O2
AGE TARGET SATURATION

1 minute 60 – 65%
2 minute 65 – 70%
3 minute 70 – 75%
4 minute 75 - 80 %
5 minute 80 – 85%
10 minute 85 – 90%
Positive Pressure Ventilation
- Device used → Self inflating bag & mask (‘AMBU’ BAG)
- Function of Reservoir → ↑O2 delivered to baby

- Oxygen delivery depends on → O2 supply & Reservoir
O2 supply Reservoir Fio2 delivered

- - 21%
+ - 40%
+ + 90 – 100%
➢ Start with room air (Fio2 21%) in term neonates
➢ Babies born at <32 wks of gestation → 21-30% fio2
➢ Rate at which PPV done →40 – 60 breaths/min
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➢ Pressure required to deliver breath to neonate

1st breath 30 – 40 cm H2O
Subsequent breaths 15 – 20 cm H2O.
➢ Absolute C/I to bag & mask ventilation → Congenital diaphragmatic hernia
CXR Findings
- Bowel gas shadows in thorax
- Mediastinal shift
- Pulmonary hypoplasia
➢ Initiation of PPV is the single most important step in neonatal resuscitation.
➢ Most sensitive indicator of effective ventilation - ↑HR
Ventilation Corrective Steps
Ensure:-
- Mask is appropriate size
- Seal between mask & face should be tight (E-C clamp technique).
- Head of baby should be slightly extended
- Mouth kept slightly open
Endotrachial Intubation in NRP
- Laryangoscope (neonatal) = with straight blade
Size→ O Preterm
1 term
Endotrachial tube size
Weight Gestational Age Size of ET (mm)

< 1000 g < 28 wks 2.5 mm
1000-2000 g 28-34 wks 3 mm
> 2000 g > 34 wks 3.5 mm
➢ Ways to confirm whether ET is in trachia
1. BIL visible chest rise with each breath
2. Improvement in vital parameters (HR, colour, SPO2)
3. BIL audible breath sounds in chest
4. Misting of ET tube with each breath
5. ETCO2 determination (End Tidal CO2) or capnography.
Recommend method to know whether ET is in trachea.
→ CXR AP view is not useful for confirming ET tube is in trachea.

Is useful to know the level of ET tube.
Tip of ET tube should be at lower border of body of 2nd thoracic vertebra in children.
Chest compressions
➢ Site - In the midline, on the lower 1/3rd of body of sterum (or).

- In the midline just below the line joining 2 nipples.
➢ Technique - 2 Thumb – Encircling technique is preferred over
- 2 finger technique
Because
→ Higher pressure generated
→ Better perfusion
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→Lesser rescuer fatigability

➢ Depth – 1/3 of AP diameter of chest.
➢ Ratio of chest compression: PPV 3:1 → 90 cc and 30 breathe in min.
In 2 sec → 3 Chest compression+ 1 Breath
Inj. Adrenaline
→ Dose 0.01 mg/kg/dose, upto 3 time.
(0.01 – 0.03 mg/kg)
or
0.1 ml/kg/dose of 1 : 10,000 Adr.
• 1 : 10,000 – Strength / concentration of Adrenaline recommended in NRP
• Preferred Route → Intravenous through umbilical venous catheter.
Can be given intra trachially (0.05 – 0.1 mg/kg/dose).
If not able to secure a vascular access.
Resuscitaion of a baby Born Through Meconium Stained Liquor (MSL)
Previous Recommendations
Baby born through MSL
Assess
Vigorous neonate Non – vigorous neonate
- HR > 100/min - Do not stimulate

- Good muscle tone - Put the body under a warmer
- Strong respiratory effort - Do ET intubation & tracheal suction to remove
Meconium
Proceed as per NRP - Intra partum suction of mouth & nose, no longer
Recommended
Latest Recommendations
→ Routine ET intubation & tracheal suction of all neonates born through MSL is no longer
recommended.
→ Ensure at least 1 person, skilled in ET intubation is available at the time of resuscitation of these
babies.
Condition in which do not resuscitate a Neonate
1. Anencephaly
2. Confirmed case of Trisomy 13 (Patau syndrome)
3. GA <22 wks.
New born corner should be available at all health facilities where childbirth is taking place.
Delayed cord clamping

➢ Wait for at least 30 seconds before clamping umbilical cord.
➢ Recommended for all stable term & preterm neonates.
➢ Advantages
o Lesser chance of anemia.
o Lesser need of blood transfusion
o High BP
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o Lesser risk of NEC (Neonatal entercolitis and NHC (intra ventricular hemorrhage).
➢ Disadvantage - ↑risk of neonatal jaundice.
- A single dose of Inj. VIT K1 1 mg IM for all babies, at birth to prevent hemorrhagic disease of new
born. (If Birth weight <1 kg, then dose of Vit. K – 0.5 mg)
- Early initiation of breast feeding → within 1 hr
- Maintain normal temperature of the baby → 36.5°c – 37.°5c
- Record weight of the baby
Before Discharge
➢ Immunisation
➢ Screening for diseases
➢ Screen for jaundice
➢ Vit. D supplementation (400 H/day)
NEONATAL SEPSIS
Definition:– Any systemic bacterial infection in a neonate.
Classification:-
a. Early onset sepsis
➢ MC seen in 1st 72 hrs.
➢ Organisms responsible are usually derived from maternal genital tract like group B
streptococci, E.coli, Klebseilla.
➢ Risk factors
Mother Delivery Baby
Maternal fever Difficult/prolonged LBW
Foul smelling liquor multiple PV examinations Prematurity
Pre mature rupture of membranes
b. Late onset Sepsis
➢ Organisms acquired from environment
➢ Community acquired Hospital acquired
o Staphylococcus aureus - Acinetobacter
o E. coli - Klebsiella
➢ Meningitis is commonly seen
➢ Risk factors
- Lack of breast feeding
- Multiple needle pricks
- Superficial infection involving skin or umbilical cord stump.
- LBW
- Prematurity
Q. Most effective/Important method to prevent neonatal sepsis?
A. Proper hand washing of caregivers for atleast 2 minutes (6 steps).
MC Organism Responsible for N.Sepsis
In India →Acineobacter >Klebsiella
In hospital in India → Acineobacter >Klebsiella
In hospitals across the world → E. coli
Overall throughout the world → group B Streptococcus
Early onset sepsis (World) → group B Streptococcus
Clinical Features
➢ Poor feeding on alteration in established feeding behaviour (earliest)
➢ Temperature disturbances (hypothermia > fever)
➢ Respiratory – Dyspnea, hypoxia, Tachypnea
➢ CNS – Shrill cry, Irritability, seizures.
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➢ GI - Abdominal distention, feed intolerance

➢ Metabolic - Hypoglycemia, acidosis
➢ Severe sepsis
• Septic shock
• Multiple organ dysfunction
• Sclerema
- Generalized, non-pitting edema seen in neonates with severe sepsis/severe hypothermia.
Diagnosis
Definitive Test – Blood culture
Screening Test – Sepsis screen
Components – any 2 out 5 → +ve sepsis screen
1. TLC (Total laucocyte count) → < 5000/m3
2. ANC (Absolute Neutrophil count) → < 1800
3. I – T Ratio (immature : Total neutrophil) → > 0.2 mm
4. Micro ESR → > 15mm
5. CRP (C-Reactive Protein) → > 1 mg/dl
Has high negative predictive value
Supportive Tests:
• Blood glucose
• CXR
• Lumbar puncture & CSF study
Treatment
1. Supportive Care
✓ Shift to Nursery/ NICU
✓ Start IV fluids
✓ Maintain normal blood glucose
✓ Maintain normal temperature
✓ Monitor
2. Specific Treatment
→ IV Emperical broad spectrum antibiotics
Inj. Ampicillin + Gentamicin

+
Inj. Cefotaxime, if meningitis present or
suspected.
Sepsis screen Blood culture CSF S/O meningitis Duration of Antibiotics
+ - - 1 week
+- + - 2 weeks
+- +- + 3 weeks
Neonatal Hypothermia
→ Definition – Axillary temperature <36.5⁰C in a neonate
→ For how much time should thermometer be kept in axilla, to record temperature accurately –
Minimum 3 minutes.
S
→ Classification Axillary Temperature
Cold stress 36 – 36.4⁰C
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Moderate hypothermia 32 – 35.9⁰C

Severe hypothermia < 32⁰C
→ Cold stress clinical definition – Abdomen is warm but soles are cold to touch.
Q. Hypothermia is more common in neonates, why?
1. Large surface area, as compare of them body Wt. (max. head loss from head)
2. Lesser subcutaenous fat espically preterm/SGA/IUGR
3. Shivering is absent.
4. Vulnerability to get exposed
Ways by which neonates protect themselves against Hypothermia.
1. Non shivering thermogenesis
➢ Most important
➢ d/t the presence of brown fat
✓ Lipid deposits richer in mitochondria
✓ Areas richer in brown fat are
• Axilla
• Groin
• Nape of neck
• Interscapular area
• Around the kidneys & major blood vessels of abdomen.
➢ Mechanism
Baby exposed to cold environment
Sympathetic stimulation
Neither release of nor epinephrine
Uncoupling of β oxidation of fat
Heat generated
2. Cutaneous vasoconstriction when exposed to cold temperature.

3. Flexed posture
4. Higher heart rate in neonates more heat generation.
C/F
Acidosis
Hypoglycemia Multiorgan
Hypoxia Dysfunction
Prevention / Treatment
1. Warm Chain to prevent N. hypothermia
➢ Thermal care in delivery room (25⁰C)

➢ Warm resuscitation
➢ Immediate drying of baby
➢ Skin to skin contact
➢ Early initiation of breast feeding
➢ Bathing postponed
➢ Clothing & bedding
➢ Rooming in
➢ Warm transportation (Weakest link)
➢ Training & awareness
2. Devices Used
Radiant Warmer Incubator
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Most important mechanism by which baby gets heated

Radiation Convection
Most important mechanism by which heat loss occurs
Convection Radiation
3. Kangaroo Mother Care
➢ Skin to skin contact between the baby & mother/care giver
➢ Indication → for all stable LBW neonates
➢ Components → Skin to skin contact (kangaroo position)
→ Exclusive breast feeding
→ Early discharge from hospital.
Advantage:-
✓ Lesser risk of nosocomial infections
✓ Lesser risk of neonatal mortality
✓ ↑ breast milk output
✓ Higher exclusive breast feeding rates
✓ Shortens length of hospital stay
✓ Maternal child bonding
✓ Lesser risk of hypothermia
➢ Normal temperature of neonate 36.5 – 37.5⁰C
➢ Delivery room temperature 25⁰C
➢ Nursery / NICU temperature 22 - 26⁰C
➢ MCC of neonatal mortality in India Prematurity
➢ Temperature of non-asphyxiated neonates is a strong predictor of neonatal mortality at all
gestational ages.
Thermoneutral Environment
➢ Range of temperature in which a neonate has minimum BMR, least O2 requirement & still the body
is able to maintain a normal body temperature.
Neonatal Hypoglycemia
Definition – Blood glucose <40mg/dl or plasma glucose < 45 mg/dl.
High Risk neonates for hypoglycemia
1. Large for date neonates
2. Small for date preterm
3. IUGR
4. Infant of diabetic mother
5. N. sepsis
6. N. Hypothermia
➢ Regular blood glucose monitoring is recommended in high risk neonates at regular intervals (2 hrs,
6 hrs, 12 hrs, 24 hrs, 48 hrs, 72 hrs, of life)
C/F
➢ Jitteriness > tremors  most common
➢ N. Seizures
➢ Cyanosis
➢ Apnea
➢ Lethargy
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➢ Poor feeding
➢ ↑ sweating
Treatment
Symptomatic
IV 10% dextrose at 2 ml/kg stat. bolus
Continuous IV fluids (GIR @ 6 mg/kg /min)
Monitor blood glucose titrate IVF according to Blood Glucose
Asymptomatic
1. <20 mg/dl → Rx as symptomatic case

2. 20-40 mg/dl
Offer a feed to baby & recheck Blood Glucose after ½ hr – 1 hr
➢ Case 1 – Blood Glucose still low – Rx as symptomatic case
➢ Case 2 – BG is normal – Continue frequent feeding Blood Glucose
monitoring.
Persistent Hypoglycemia
Important Causes during Infancy
Endocrine Causes
1. Congenital hypopituitarism
2. Congenital adrenal insufficiency
3. Congenital Hyperinsulinemia (or)
Nesidioblastosis (or)
PHHI (Persistent Hyperinsulinemic
Hypoglycemia of infancy)
➢ MCC of persistent hypoglycemia during infancy
➢ Drugs used in Rx
• Diazoxide
• Octreotide
• Glucagon
• Nifedipine
➢ Surgery in focal cases
Metabolic causes
1. Glycogen storage disease
2. Galactosemia
3. Herediatry fructose intolerance
4. Mitochondrial disorder
5. Fatty acid oxidation defect
Infant of Diabetic Mother

➢ Patho Physiology
➢ Pederson’s maternal hyperglycemia /fetal hyperinsulinemia Hypothesis
Maternal hyperglycemia
Fetal hyperglycemia
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Fetal hyperinsulinemia Neonatal hypoglycemia
Insulin acts as a growth factor for fetus
Macrosomia /LFD ↑ed Extra medullary hematopoiesis

All organs↑ in size in IDM Polycythemia
Except Brain Neonatal Hyperbilirubinemia
➢ Hairy pinna +nt in IDM
Problems in IDM
1. Macrosomia / LFD Baby
➢ Difficult / prolonged labour
➢ Perinatal asphyxia
➢ ↑ed chances of birth trauma
2. Metabolic
➢ Hypoglycemia
➢ Hypocalemia
➢ Hypomagnesemia
➢ Polycythemia
➢ Neonatal jaundice
3. Respiratory system
➢ More chances of RDS d/t delayed maturation of surfactant.
4. CVS
➢ MC congenital abnormality in IDM – CHD
➢ MC congenital Heart disease in IDM – VSD
➢ Most specific congenital heart disease in IDM – TGA
5. CNS
➢ MC congenital neurologic abnormality in IDM – Neutral tube defect.
➢ Most specific neurologic abnormality in IDM – Sacral agenesis of caudal regression syndrome.
→ Overall most specific congenital abnormality in IDM.
➢ Sacral agenesis or caudal regression syndrome
6. Renal
➢ Renal agenesis
➢ Duplication of ureter
➢ Renal vein thrombosis
7. GI – Duodenal atresia
➢ Lazy (small) left colon syndrome
8. Long term problems (“BOND”)
➢ Blindness
➢ Obesity
➢ Non ketotic hypoglycemia
➢ Diabetic mellitus
Perinatal Asphyxia (Birth Asphyxia)

➢ Inability to initiate or sustain breathing
Hypoxia
Hypercapnia
Acidosis
Multiorgan dysfunction
WIN COACHINGEsply CNSMARG,
HALDI GHATI (HIE chypoxic
IN FRONT OF ischemic encephalopathy)
DUCKLING SCHOOL, NEAR JAIPUR GYM, PRATAP NAGAR JAIPUR. 8742073072, 9587958165
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HIE (Hypoxic Ischemic Encephalopathy)

Part of brain mc involved in HIE in:-
→ Term neonates – para sagittal area – Spastic quadruplegia (Type of cerebral palsy)
→ Pre term neonates – Periventricualar area – Periventricular Leuco-Malacia (PLM)
Spastic Diplegia
Diagnostic Criteria For severe birth Asphyxia
→ APGAR Score 0-3 for >5 minutes
→ Severe acidosis (Cord blood PH <7.0)
→ Any clinical evidence of CNS dysfunction
Tone abnormalities, seizures etc.
→ Any Evidence of dysfunction of at least 1 organ other than CNS.
→ Eg: Renal – Acute tubular necrosis
Heart – Myocardial dysfunction
Staging of HIE
Stage 1 Stage 2 Stage 3
Level of conciousness Hyperalert Normal / Depressed Comatosed
Tone Normal Mild hypotonia Severe hypotonia
Moro’s Reflex Exaggreated Normal / depressed Absent
Seizures Absent Present Not seen
Autonomic Generalized sympathetic Generalized Both systems are
involvement overactivity mydriasis Parasympathetic depressed pupil mid
↑Heart rate overactivity miosis dilated variable HR
Brady cardia
Outcome 99% Normal 80% normal 50% die
50% severe neurologic
Sequelae
Treatment of HIE
1. Supportive Rx
➢ NICU
➢ IVF
➢ Normal Blood glucose & temperature
➢ Monitor
Tools used for bedside monitoring of neonates with HIE – AEEG
(Amplitude integrated Electro encephalogram)
2. Latest Rx modality for moderate to severe HIE in neonates
Therapeutic Hypothermia
Temp. Maintained is → 33.5⁰C – 34.5⁰C
3. Neonatal seizures
➢ Doc – Phenobarbitone
➢ MC type – subtle seizures
➢ MC cause – Hypoxia
➢ Type with best prognosis – Focal clonic seizures
➢ Type with worst outcome – Myoclonic seizures
➢ Preferred initial CNS imaging – Transcranial ultrasound
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Important Scores in Neonates

APGAR score
Components 0 1 2
Appearance Completely blue or pale
Body pink but Completely pink
extremities blue
Pulse Rate Absent <100 min > 100/min
Grimace No responses Grimaces only Coughs/sneezes
Activity Limp/Flaccid Some flexion Actively moving baby
Respiratory effort None Slow & irregular Normal / strong
Grimace → Response to stimulation of Oropharynx by a catheter
➢ Maximum score → 10
➢ Minimum score →0
➢ > 7 Score → Normal
➢ 0-3 score → Severe birth asphyxia
➢ Apgar score documented at 1 minute & 5 minutes of life.
➢ His no role in neonatal resuscitation
➢ It has prognostic importance
Scores use to Assess Respiratory Distress in a :→
Preterm neonate Silverman score
Term neonate Downe’s Score
Silverman Score
Components 0 1 2
Upper chest retractions Chest & abdomen Chest wall lags behind Chest wall & abdomen move in
rise together abdomen opposite direction see saw
Lower chest retractions Absent Minimal Marked
Xiphisternal retractions Absent Minimal Marked
Nasal flare Absent Minimal Marked
Grunt None Audible only with Audible without stethoscope
stethoscope
➢ Max score – 10
➢ Min Score – 0
➢ 0 – 3 Score – Normal
➢ >7 score → Severe respiratory distress
DOWNE’S SCORE
Components (Scores → 0,1,2)
1. Cyanosis
2. Air entery
3. RR
4. Gurnt
5. Retractions
RESPIRATORY DISORDERS
Respiratory Distress Syndrome/ Hyaline membrane Disease
MC cause of Respiratory distress in a preterm neonate.
Basic defect → Deficiency of mature surfactant.
Type II →10% Surfactant
Surfactant
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Composition
DPPC (Di palmitoyl phosphatidyl choline) or lecithin (most imp.)
Phosphatidyl glycerol
Cholesterol
L:S →2:1
Surfactant proteins
A, B, C, D
B – Most important surfactant protein
Synthesis
➢ Begins in fetal lungs 20 weeks gestation
➢ Begins to appear in amniotic fluid 28-32 wks of gestation
➢ Mature surfactant in adequate amount >35 wks of gestation
Functions:
➢ To ↓surface tension of alveoli cor)
➢ To prevent alvedi from collapsing during expiration.
Pathophysiology of RDS
Deficiency of mature surfactant
Alveolar collapse Eosinophilic

Diffuse alveolar damage Hyaline membrane
Interstitial edema Appearance on
Fibrin deposition Lung Biopsy
C/F →
A preterm neonate born at <35 wks of gestation presenting with respiratory distress soon after the birth
with typical CXR findings.
CXR Findings in RDS
➢ Ground glass haziness of lungs
➢ Presence of air bronchogram
➢ Reticulo granular or reticulo nodular appearance
➢ Features of lung collapse
Diagnosis
1. C/F
2. CXR findings
3. Ways to detect adequacy of surfactant in amniotic fluid.
→ L:S Ratio >2:1 →mature surfactant
→Complete ring of bubbles on surface → mature surfanct (on ‘shake test’)
→ Nile Blue Sulfatase test → to detect lung maturity.
→ Shake test
➢ > 1 Complete ring of bubbles on surface.
➢ Ethyl alcohol + Amniotic Fluid Mature surfactant
Treatment of RDS
1. Supportive care
2. Specific treatment
A. Mild RDS – CPAP (Continous positive airway pressure)
B. Moderate – Severe RDS
→ Intra tracheal surfactant
+
Respiratory support (CPAP/mechanical ventilation + O2)
Prevention of RDS
Antenatal corticosteroids
Indication
21
➢ To all pregnant ladies who are expected to deliver between 24-34 wks.
Steroid to choose
Inj. Betamethasone 12 mg IM; 2 doses, 24 hrs apart
Inj. Dexamethasone 6 mg IM; 4 does, 12 hrs apart
Inj. Betamethasone has slightly neuroprotective effect.
→ Steroid of choice
Recommended by Indian government → Inj. Dexamethasone
→ Cheaper & easily available, equally efficacious
Beneficial Effects
➢ ↓ RDS risk
↓ JVH
↓ NEC
↓ Neonatal mortality
➢ Does not decrease the risk of neonatal jaundice
Neonatal Pulmonary Alveolar Proteinosis
Basic Defect → d/t deficiency of surfactant protein B
→ Function of surfactant protein B – Forms a thin layer of surfactant in the inner layer
of alveoli.
CLINICAL FEATURES
➢ A Term neonate presenting with severe respiratory distress soon after birth with CXR showing
ground glass haziness.
➢ No improvement with surfactant therapy.
➢ H/o similar illness in a previous sibling who died (family history).
Meconium Aspiration syndrome
Meconium
➢ 1st stool passed by a neonate; greenish black in colour; sterile
➢ Comprises of
o Amniotic fluid
o Bile
o Mucus
o Lanugo
o Denuded interstitial epithelial cells
o Water
Pathophysiology
1. Obstructive emphysema (MC & most important)
2. Chemical pneumonitis
3. Segmental collapse or atelectasis.
Clinical features
➢ A term SGA/IUGR baby, born through meconium stained liquour, presents with respiratory distress
soon after birth.
➢ O/E – AP diameter of chest increased
-Typical CXR findings
CXR in MAS
1. Hyper inflated lungs → ↑ed radiolucency of lungs
→ Flattening of domes of diaphragm
2. Pulmonary infiltrates
3. Segmental collapse
Treatment
➢ Mainly supportive including respiratory support (O2 +- mechanical ventilation)
22
➢ In severe Cases
• inhaled nitric oxide may be used
• intractracheal surfactant
• High freq. ventilation.
• ECMO( Extracorporal membrane oxygenation may have to be used)
Complications
1. Pneumothorax
2. PPHN (Persistent pulmonary HTN of new born)
TTNB (Treatment tachypnea of new born) / delayed adaptation

MCC of respiratory distress in a term neonate
Basic Defect Delayed clearance of lung fluids
Risk factor Delivery by caesarean section
Clinical features
➢ Term / post term neonates
➢ Born by caesarean section
➢ Presents with mild respiratory distress, soon after birth that improves.
➢ Spontaneously in 72 hrs.
➢ With typical CXR findings
➢ CXR Findings
o Fluid in interlobar fissure
o Pleural effusion
o Perihilar streating (d/t prominent bronchovascular markings)
Treatment
➢ Mild & self limiting illness
➢ Usually no Rx required
➢ Distress usually resolves spontaneously in 48 – 72 hrs)
Neonatal Apnea
Definition – Cessation of breathing of at least 20 sec or for any
Duration, in the presence of bradycardia or cyanosis
Important causes
1. Neonatal sepsis
2. Neonatal Hypothermia
3. Neonatal Hypocalcemia
4. Neonatal Hypoglycemia
5. Polycythemia
6. Neonatal jaundice
7. NEC
8. Apnea of prematurity
a. More preterm the neonate → more chances of apnea of prematurity
b. It is a diagnosis of exclusion
Treatment
1. Respiratory support (CPAP or mechanical ventilation)
2. Look for the cause & Rx it.
a. IV antibiotics for neonatal sepsis
23
b. Warm up for N. hypothermia

c. IV ca gluconate for N. hypocalcemia
d. IV 10% dextrose for N. Hypoglycemia
e. Partial exchange transfusion with normal saline (Rxoc) for polycythemia.
f. Inj. Caffeine citrate (doc)
For apnea of prematurity
Inj. Aminophyline
Neonatal hypocalcemia
Total Ca Ionised Ca
✓ In a term neonate < 8 mg/dl < 1.2 m.mol/L

✓ In a preterm neonate <7 mg/dl < 1 m.mol/L
Polycythemia
✓ Hb > 22 g/dl (or).
✓ Hematocrit > 65% in neonate
Broncho pulmonary Dysplasia / Chronic lung disease (CLD)

Mc affects babies born at < 28 wks of gestation or with birth weight <1000 gms.
D/t atelectotrauma, volutrauma, free radicals.
Definition
➢ BPD is defined for babies born at < 32 wks gestation, who require O2 for 1st 28 days of their life as-
➢ Assessed at 36 wks of PMA (post menstrual age)
o Mild BPD if no supplement O2 req
o Moderate BPD O2 req <30%
o Severe BPD O2 req >30% OR CPAP or mechanical ventilation
Congenital diaphragmatic hernia

What is it:-
➢ A diaphragmatic defect through which abdominal contents may herniated into thorax.
➢ Pulmonary hypoplasia
o Intestinal malrotation
Associated with
➢ Esophageal atresia
➢ Congenital heart diseases
➢ Omphalocele
➢ Trisomy 13, 18
MC type → Postero lateral or Bochdalek variety
MC on→ Left side
MC in → Females
Clinical features
At birth
TRIAD:
▪ Respiratory distress
24
▪ Scaphoid abdomen
▪ Mediastinal shift
Later in life Intestinal obstruction
Diagnosis
1. C/F
2. Antenatal USG (Between 16-24 wks)
3. CXR
✓ Bowal gas shadows in thorax
✓ Mediastinal shift
✓ Pulmonary hypolasia
Treatment
Medical Management
Baby with known / suspected CDH
Severe Respiratory distress
Yes No
Do endotracheal intubation
Suspition of esophageal ET in trachea
Intubation (max. cardiac Insert a NG tube , CXR to confirm ,
Impulse shifted to right) Dx surgery
1. Confirm position of ET (Not by CXR)

2. Remove ET & reintubate
❖ Bag & mask ventilation is absolutely C/I
Predictors of poor outcome in CDH
1. Severe pulmonary hypoplasia
2. Lung head Ratio (LHR) < 1
3. Any malformation associated
4. Symptoms in 1st 24 hrs.
5. Liver into thorax
6. ECMO need
MC cause of mortality in CDH – Pulmonary complication/hypoplasia
Necrotising Enterocolitis
➢ Acute intestinal necrosis of unknown etiology
➢ Risk Factors
• Pre maturity (single greatest risk factor)
▪ Mean gestational age 30-32 wks
▪ 10% cases occur in term neonates
• Aggressive use of formula feeding
• Lack of breast feeding
25
• Fetal hypoxia
• Maternal coccive abuse
• Absent or reversed end diastolic flow in the umbilical artery on antenatal USG.
➢ Part of intestine Mc involved
• Terminal ileum & ascending colon
Modified Bell’s staging of NEC

Stage I (NEC suspect)
General features
• Temp. disturbance
• Apnea
• Lethragy
Abdominal features
• Feed intolerance
Investigations
1. Abdominal X ray
Normal (or)
Mild distention
2. Stool examination
Ia → Occult blood in stool
Ib → Fresh blood in stool
Treatment
➢ NPO
➢ IV fluids 48 – 72 hrs & reassess
➢ IV antibiotics
Stage II (Definite NEC)

General features
• Same as above
Abdominal features
• Absent bowel sounds
• Mild abdominal distention
Investigation
1. Abdominal x-ray
a. IIa – Pneumatosis intestinalis
(air in the wall of intestine)
b. IIb – Portal vein gas
Treatment
➢ NPO
➢ IVF + TPN 7-10 days & reassess
➢ IV antibiotics
Stage III (Advanced NEC)
General Features
• Shock
26
• Bleeding
• Recurrent
• Life threatening apnea
Abdominal features
• Abdomen hugely distended & tender
• Abdominal wall cellulitis
Investigations
1. Abdominal X-ray
• IIIa– Peritonitis
• IIIb– Pneumopeuconeam – free
o Gas under diaphagram
2. Blood Examination
• Severe acidosis
• Hyponatremia
• Refractory thrombocytopenia
Treatment
➢ Same as stage II
➢ IV fluid boluses
➢ Inotropes
➢ Blood products
➢ Mechanical ventilation
+
➢ Surgery in IIIb
Prognosis → 10 - 30% risk of mortality despite best supportive care.
NEONATAL JAUNDICE
➢ Clinical jaundice in neonates is seen at billirubin level ≥ 5 mg/dl
➢ 60% of term neonates & 80% of preterm neonates have clinical jaundice in 1 st week of life.
PHYSIOLOGICAL JAUNDICE PATHOLOGICAL JAUNDICE
1. Icterus / clinical jaundice never appear in 1st 24 1. May appear in 1st 24hrs of life
hrs of life
2. Always unconjugated; urine does not stain 2. May be conj or unconjugated so high
diapers & no pale stools colored urine or pale stool may be seen
3. Palms & soles never stained yellow 3. Palms & soles may be stained yellow
4. Clinical jaundice does not persist beyond 2 wks 4. May persist beyond 3 weeks
in term neonates & 3 wks in preterm neonates
Physiological jaundice
Reasons HdL > 50 mg/dL
1. Higher production of billirubin LDL < 100 mg/dL
➢ Higher Hb level in neonates Total Billirubin 0.3 – 1.0 mg/dL
Indirect Billirubin 0.2 – 0.8 mg/dL
➢ Shorter life span of RBCs (90 days v/s 120 days)Q Direct Billirubin 0.1 – 0.3 mg/dL
➢ More ineffective erythropoiesis Cholesterol < 200 mg/dL
2. Ineffective carrier medicated uptake of billirubin by liver Triglyceride < 150 mg/dL
3. Immature UDP glucuronyl transferase enzyme activity
4. ↑sed enterohepatic circulation in neonates
27
Breast feeding jaundice Breast milk jaundice

➢ d/t inadequate breast feeding → d/t substances present in breast milk like
Dehydration pregnanediol & free fatty Acids, that interfere
Relative polycythemia with the conjugation of billirubin
Higher billirubin level
➢ Rx: Frequent breast feeding → Rx: Continue breast feeding,
unless the billirubin Level is > 20 mg/dl,
when breast feeding may only be temporarily
withheld.
IMPORTANT CAUSES OF UNCONJUGATED HYPERBILLIRUBINEMIA

↑ Production of billirubin ↓ Conjugation of billirubin
1. Hemolytic disorders 1. Crigler Najjar syndrome
✓ Erythroblastosis fetalis Deficiency of UDP Glucuronyl
(Hemolytic disease of newborn) Transferase enzyme
- Mc cause of neonatal jaundice in 1st 24 → Type I - Complete
hrs of life Deficiency
✓ Hereditary spherocytosis → Type II – Partial
✓ G6PD deficiency Deficiency
2. Polycythemia 2. Gilbert syndrome
3. Delayed cord clamping 3. Down syndrome
4. Cephalhematoma 4. Congenital hypothyroidism
Conjugated Hyperbillirubinemia
Conjugated Billirubin → > 2 mg/dl (or) > 20% of total billirubin
Important causes
Non obstructive causes
1. Infections
➢ Viral →EBV, CMV, Hepatitis
➢ Bacterial →Congenital TB (Ghon focus seen in liver), syphilis
➢ Parasitic →Toxoplasmosis
2. Toxins Sepsis, UTI, total
3. Metabolic Alpha1 antitrypsin deficiency
- Cystic fibrosis
- Tyrosenima
- Galactosemia
- Herediatry Fructose intolerance
4. Idiopathic neonatal hepatitis
- MC cause of conjugated hyperbillirubinemia in neonates
Obstructive causes
S. NO. Intra hepatic causes Extra Hepatic causes
1. Congenital heptic fibrosis 1. Extra hepatic biliary Atresia (EHBA)
2. Caroli’s disease 2. Choledochal cyst
3. Progressive familial intra 3. Stone
Hepatic cholestasis (PFIC)
4. Alagille syndrome 4. Stricture
28
✓ Triangular facies
✓ Pulmonary stenosis
✓ Butterfly vertebrae
5. Dubin Johnson syndrome 5.Mass
➢ Pigmented liver (Dark liver)
6. Rotor syndrome
• Screening test for EHBA – HIDA scan (or) Hepatic scintigraphy
• Surgery for EHBA – Kasai procedure
• EHBA is the mc indication for liver transplantation in children
Clinical features of neonatal jaundice
Icterus in neonates has a cephalocaudal progression
➢ Billirubin also measured by
✓ Transcutaneous bilirubinometer
✓ Blood sample → serum billirubin level
NEUROLOGICAL MANIFESTATIONS
➢ Mc involved part of brain in neonatal jaundice → Basal ganglia
➢ Type of cerebral palsy seen → Extra pyramidal type
➢ KERNICTERUS → Yellow staining of basal ganglia (previously used term)
➢ Acute bilirubin Encephalopathy
Early features (mild) → Hypotonia, poor feeding, loss of moro’s reflex
Fever, irritability, seizures
Features of → Hypertonia
Advanced disease Opisthotonic posturing
(Severe) Coma,
Death
➢ Chronic Bilirubin Encephalopathy:→
✓ Sensorineural hearing loss
✓ Athetosis -
✓ Mental retardation
✓ Dental dysplasia -
✓ Upward gaze, limitation
Treatment of Neonatal Jaundice
I – Phototherapy
II – Exchange Transfusion
III – Drugs
Exchange Transfusion
➢ Used in very severe cases especially erythroblastosis fetalis
➢ Double volume exchange transfusion done.
Drugs
✓ IV Ig (Intra venous immunoglobulin)
• Used in Erythroblastosis fetalis
• Occupies the receptors for FC segment of Ig in reticule endothelial system & prevents
further production of Ig.
Severe neonatal jaundice Due to erythroblastosis fetalis – Treatment (order)
1. Start Phototherapy
2. Do Exchange Transfusion 3. IV Ig
Phototherapy
29
➢ Most effective wave length of light used → 450-470 nm

➢ Mechanisms by which phototherapy acts.
1. Photo Isomerisation
a. Bilirubin → Polar compound Excreted through kidney without conjugation
b. Slow & reversible
2. Structural Isomeristion
a. Bilirubin → Lumirubin excreted through kidney without conjugation
b. Faster & irreversible
c. Most important mechanism by which phototherapy acts.
3. Photo oxidation (least important)
➢ Effectiveness of phototherapy depends upon
1. Exposed surface area of baby
2. Distance b/w baby & photo therapy unit (30-45cm)Q
3. Type of lamp used; LED lamps > CFL lamps
Does not depend on skin pigmentation of baby
Irradiance should be at least → 30 uw/Cm2/nm
→ Measured using flux meter
➢ Adverse Effects of Photo therapy
• Bronze baby syndrome
• Watery diarrhoea
• Dehydration
• Hypocalcemia
• Retinal toxicity
• Gonadal toxicity or mutations
• Impaired maternal child bonding
➢ In otherwise healthy term neonates,
AGE PHOTOTHERAPY CUT OFF EXCHANGE TRANSFUSION CUT OFF
24 – 48 hrs > 15 mg/dl >20 mg/dl
48 – 72 hrs > 18 mg/dl > 25 mg/dl
> 72 hrs >20 mg/dl > 25 mg/dl
➢ In preterm neonates
o Phototherapy cut off 1 % of Birth wt. (in gram)
o Exchange transfusion cut off Phototherapy cut off +5 (mg/dl)
Phenobarbitone
➢ Enzyme inducer
➢ ↑ activity UDP Glucuronyl transferase enzyme
➢ Useful in crigler najjar syndrome type II
Indication of exchange transfusion in a Baby with RH incompatibility
➢ Cord blood bilirubin → > 5 mg/dl
➢ Cord blood Hb → > 10 mg/dl
GROWTH
GROWTH CHARTS
Growth → Increase in physical size
EMBRYO 1st 8 weeks

FETUS Rest of IUL (From 9 weeks)
NEONATE 1st 28 days of life
INFANT 1st year of life
30
TODDLER 1 – 3 year
PRE SCHOOL 3 – 6 year
ADOLESCENT 10 – 19 year
Growth Assessment
Growth assessed by anthropometric parameter like:-
1. Weight
2. Height
3. Head circumference
4. Mid arm circumference
5. Skin fold thickness
6. Chest circumference
7. BMI
MID ARM CIRCUMFERENCE
➢ Device used by health workers to measure MAC – SHAKIR’S TAPE
Red→ <11.5 cm (Severe)
Orange→ 11.6 cm-12.4 cm (Malnutrition)
Yellow→ 12.5 cm – 13.5 cm
Green→>13.5 cm (Normal)
✓ First year of life → 11--16 cm
✓ 1 to 5 year → 16--17 cm
Skin fold thickness
→ Device used to measure → Harpenden calipers → Subcutaneous fat → Triceps skin fold → Check upto
16 yr Age:-
✓ < 6mm Severe
✓ > 10mm Normal
→ Areas to be measured
• Supra Scapular
• Sub scapular
• Biceps
• Triceps
→Tanner’s charts used Previously

• WHO charts are used now
Chest Circumference (CC)
- At birth, HC > CC
- By 9m – 1yr → HC = CC
- >1yr → CC > HC
BODY MASS INDEX (BMI)

Body Mass Index (BMI) = wt (Kg)
Ht (m)2
GROWTH CHARTS
→ Graphical representation of the anthropometric parameters
→ NCHS Growth charts (1977)
→ CDC Growth Charts (2000)
→ WHO Growth Charts (2006)
NCHS – National center for health statistics
CDC – Center for disease control & Prevention
31
WHO Growth Charts

➢ Preferred growth charts for under – 5 children all over the world
➢ Based on MGRS (Multicenter Growth Reference study)
✓ Conducted in 6 countries across the world including India
✓ Countries (BONGUI)
Brazil Ghana
Oman US
Norway India (New Delhi)
➢ Enrolled those babies who were exclusively breast fed in 1st few month of life.
➢ Exclued factor like maternal smoking, alcohol.
WHO Growth charts available for
1. Weight for age
2. Weight for height
3. Height for age
4. HC for age
5. MAC for age
6. Skin fold thickness for age
7. BMI for age
8. Major motor milestones
India growth Charts (Preferred beyond 5 yrs of age)
1. IAP (Indian Academy of pediatrics) charts
2. K.N. Agarwal Charts
3. Khadilkar Charts
WEIGHT
Birth weight of an average Indian baby → 2.8 to 2.9 kg
BIRTH WEIGHT W
At 5 months → 2w
At 1 year → 3w
At 2 year → 4w
At 3 year → 5w
At 5 year → 6w
At 7 year → 7w
At 10 year → 10 w
➢ Birth weight doubles at 5 monthsQ
➢ Birth weight Triples at 1 yearQ
➢ Birth weight Quadruples at 2 yearQ
EXPECTED WEIGHT OF CHILD

X+9
< 1 year → X – Age in months
2
1 – 6 years → 2x + 8 X – age in years
7X - 5
7 – 12 yrs → X – Age in Years
2
32
HEIGHT
➢ Length – 2 yrs of life
1st
• Device used to measure length – InfantometerQ
➢ Height – 2 years of life
• Device used to measure height – StadiometerQ
LENGTH/HEIGHT OF CHILD
At birth 50 cm
By 3 months 60 cm
By 9 months 70 cm
By 1 year 75 cm
At 2 year 90 cm
At 4 – 4.5 years 100 cm
➢ Maximum growth takes place during 1st year > puberty

➢ Length of the child increased by 50% in 1st year
➢ Height of child doubles itself of increased by 100% 4 - 4.5 years
➢ EXPECTED HEIGHT OF A CHILD = (6x + 77) cm x → Age in year.
➢ UPPER SEGMENT : LOWER SEGMENT RATIO
• UPPER SEGMENT – Above symphysis pubis
• LOWER SEGEMENT – Below symphysis pubis
AGE US : LS RATIO
At birth 1.7 – 1.9 : 1
At 3 years 1.3 : 1
At 7 – 10 yrs 1 : 1
HEAD CIRCUMFERENCE/OCCIPITO FRONTAL CIRCUMFERENCE

➢ Measured using non Stretchable measuring tape with mm markings.
➢ At birth, HC 33 to 35 cm
AGE Rate of Increase In HC
1 st 3 months 2 cm/month
Next 6 months 1 cm/month
Next 6 months 0.5 cm/month

Next 2 years 0.2 cm/month
Q. If HC at birth is 35 cm. When it will become 43 cm.
A. At birth → 35 cm
1 m → 37 cm
2 m → 39 cm
3 m → 41 cm
4 m → 42 cm
5 m → 43 cm
Brain Development
Size of Brain
o At 1 month → 36% of adult size
o At 1 yrs of age → 72% of adult size
o At 2 yrs of age → 85% of adult size
33
MICROCEPHALY / Small head

➢ Definition – HC of a child < - 35 D or Z score of expected according to age & sex of child
➢ Important Causes
Primary/Genetic causes
1. Cri-Du-Chat syndrome (5p) Can not
2. Smith Lemli opitz syndrome See
3. Patau syndrome (Trisomy 13) P
4. Edward Syndrome (Trisomy 18) E
5. Familial F
6. Rubinstein Taybi syndrome R in
7. Cornelia De Lange Syndrome Child
Secondary causes:-
Maternal Causes Other Causes
1. Infections (Torch) 1. Severe malnutrition’s
2. Smoking 2. CNS infections During Infancy (Meningo encephalitis)
3. Alcohol Intake (Fetal alcohol syndrome) 3. Acquired microcephaly
4. Radiation Exposure - Rett syndrome (x linked dominant)
5. Phenylketonuria - Angelman syndrome
6. Phenytoin Intake - Seckel syndrome
MACROCEPHALY / LARGE HEAD

Definition → HC > + 25D of expected according to age & sex of child.
Important Causes
1. ↑Thickness of Cranial Bones
➢ Chronic hemolytic anemia Eg. thalessmia
➢ Rickets
➢ In osteogenesis imperfect
2. Sub dural fluid collection
➢ Present as effusion or empyema
➢ As a sequelae of meningitis
3. Megalencephaly (↑ size of Brain)
◼ Lysosomal storage disorders
➢ Mucopolysaccharidosis
➢ Tay sach disease
➢ GM, Gangliosidosis
4. Hydrocephalus (increase in size of ventricles)
Normal Dentition
Primary Dentition Secondary Dentition
Milk/Temporary Teeth Permanent Tooth
Begins at 6-7 months 6 years
1st tooth to erupt Lower central incisor 1st molar
Last tooth Second molar 3rd molar (or) wisdom tooth
Completes at 2.5 – 3 yrs 12 yrs (except the 3rd molar)
3rd molar (18-25 yrs)
Total no. of teeth 20 28 - 32
34
Abnormalities of Dentition
Delayed Dentition
• Definition – When no tooth erupts by the age of 13 months.
Important causes (“FRIED Chop”)
1. Familial
2. Rickets
3. Idiopathic
4. Endocrine
▪ Hypo pituitarism
▪ Hypo thyroidism
▪ Hypo parathyroidism
5. Down syndrome
Natal Teeth
1. Pierre Robin sequence
2. Ellis van crerald syndrome
3. Epidermolysis bullosa (lethal acantholytic variety)
4. Soto’s syndrome
Hutchinson’s Teeth
➢ Notched incisors
➢ Seen in congenital syphilis
NORMAL DEVELOPMENT
Development
35
➢ Attainment of maturity of functions

➢ Rules of development
o Continuous process, starting in utero
o Sequence of attainment of milestones remains same
o Depend on neurological status of child
o Cephalo caudal direction
o Certain primitive reflex must be lost before score milestone are attained.
Domains of development
❖ Gross motor
❖ Fine motor
❖ Social
❖ Language
Gross motor
1 month In ventral suspension, head in below the plane of rest of body; No neck control
2 months In ventral suspension, head in the plane of rest of body
3 months In ventral suspension, head goes
Above the part of part of body
- Neck holding +
- In prone position – can lift hand and shoulder above the ground level & can bear it
forearm.
4 months Partial wt bearing when made to stand
5 months Feet to mouth, complete neck control
6 months Supports his weight on extended arms in prone position prone to supine.
Sitting with support / sitting in tripod position.
7 months Supine to prone
8 months sitting without support; crawls
9 months standing with support
10 months creeping
10-11 months Pivotting
Cruising
12m – 1 year Stand without support
Can walk on hands & feet like a bear
Walk without one hand held
walk with support
15 months Creep upstairs
Walks without support
18 months Goes upstairs & down stairs holding the side railing
Pulls a toy.
Run
2 years Goes upstairs & down stairs 2 feet per step.
Kicks a ball
Walks backwards
3 years Goes upstairs with alternating feet & down stairs 2 feet/sleep
Rides a tricycle
4 years Goes upstairs & down stairs with alternating feet.
Hops
5 years Skips
Can stand on 1 leg for >10 sec.
36
FINE MOTOR
1 month Hands kept closed, Plamar grasp reflex present
3 months Hands kept open, Palmar grasp reflex lost, Holds on object when placed in hand
‘Hand regard’ Appears disappear at 20th wk
4 months Tries to reach an palmar grasp
5 months Bidextrous grasp
6 months Unidextrous or palmar grasp, Can feed self a biscuit
7 months Transfer objects from 1 hand to another
9 months Immature/assisted pincer grasp
12 months Mature/unassisted pincer grasp
‘CASTLING’
15 months Scribbles spontaneously, Feeds self with a cup, Tower of 2 cubes
18 months Feeds self with a spoon, Tower of 3 cubes, Turn 2-3 pages at a time
2 years Tower of 6 cubes, Turns pages singly, Undress self, Copies a horizontal or vertical line
Turns a door knob or unscrews a lid
3 year Tower of a 9 cubs, Can dress or undress self except button
Copies a circle, handedness get established
4 years Can button & unbutton, Copies a rectangle or a plus sign or cross, Makes a bridge
with cubes, Catches a ball reliably
5 years Can tie shoe laces, Copies a triangle a multiplication sign or tilted cross.
Makes a gate
SOCIAL
1 month Looks at the mother intently, when talked to
2 months Social smile
3 months Recognizes mother
6 months Mirror play appears
7 months Stranger anxiety appears
8 months Object permanence
37
9 months Wave bye bye

10 months Plays peek a boo
12 months Kisses on request
Plays a simple ball game
15 months Points to object
Indicates wet pants
18 months Domestic mimicary
Dry during day time
2 years Parallel play
3 years Join in play
Know his name, age, gender
Dry at right
4 years goes to toilet alone
Asks questions
5 years starts helping in simple household taskes
Distinguishes morning from evening
Compare 2 weights
Can follow 3 step commands
LANGUAGE
1 month Quitens when a bell is rung
2 months Vocalises
3 months cooing
4 months laughs aloud
5 months razzing
6 months Speaks monosyllables like ma, ba, da (Babbling)
9 months Speaks bisyllables like mama, papa, without meaning
Bisyllable babbling
1 year Speaks 2-3 words without meaning
Imitates simple sounds
15 months Jargon speech
18 months Known 10 words with meaning
2 years Speak short sentence (2 word sentence)
Use pronuncitae like I, me, you
Vocabulary of 50, 100 words
3 years Uses pleurals & past tense
Repeats 3 digits, 3 word sentences
4 years Tells a story/poem, sing a song
5 years Repeats 4 digits
Names 4 colours
Reflexes
The
infent lies bed with high pelvis and Ventral suspension; unable to
38
knees deawn up at 2 weeks hold neck in the line with Infant fixates on her mother as
trunck at 4 weeks she talks to her at 1 months
The Ventral suspension, head in the

infent lies with flat pelvis and Sitting; back rounded but able line with trunck at 8 – 10
extended hips at 6 weeks to hold head at 8 weeks weeks
Ventral suspension; head in Grasping ‘with the eye’ at 3

In prone: face lifted to above 450 at 8
line with the trunk at 12 months
weeks
weeks
The child brings the hand in

midline as he plays at 3 to 4 Sitting; back much straiger at 4
In prone: face, head and chest off the
months of age months
couch at 3 months
Infant lifts head from the

Hand regard (between 12 and supine position when above to
Bidextrose grasp approach to a
20 weeks) be pulled at 5 months
dangling ring at 4 months
Pull to sit; flexes the head on to chest Pull to sit; complete head lag A child mouthing an object at 6
at 5 months in a newborn months of age
Bears almost entire weight at 6 Pivoting; turns around to pick Sitting with support of hands at
months up an oject at 11 months 6 months
39
Immature grasp at 6 months (palmar A child smiles at himself in the

In prone: weight on hands mirror at 6 months of age
grasp) with exteded arms at 6
months
Diagonal localization of the

Sitting withoud support at 8 months Creep position at 10 months source of sound at 10 months
of age (abdomen off ground
and weight on hands and knee
Mature grasp at 1 yr of age, note the Child walking with one hand – Stands well at 12 months
use of thumb and index finger held at 12 – 13 months
Pincer grasp approach to small

objects (index finger and thumb) Scribbles spontaneously at 15 Pull to sit; complete head lag in
months a newborn
At month, the baby showing

Social smile
intent regards of his mother’s
face as she talks to him A child makes tower of 5 – 6
cubes
40
The child is able to walk upstairs and

downstairs one foot per step at 4 yr
Drawing skills at various ages
Block skills at various ages
41
DEVELOPMENTAL THEORIES OF PAEDIATRICS

Freud’s Psychosexual stage of development
Birth to 1 year Infant Oral Feeding, mouthing – Id
1 year to 3 years Toddler Anal Bowel /bladder
3 years to 6 years Pre – Schooler’s Phallic Gender identity
6 years to 12 years Schooler Latency -
12 years to beyond Adolescent Genital Sexual orientation
- Oral fixation seen in Addiction.
- Anal fixation seen in OCD.
- Phallic phase
a. Electra complex – Female – Penis envy. (Female child attract to father)
b. Oedipus complex – Male – Castration anxiety. (Male child attract to mother.)
Structural vs topographical theory of mind

Sigmund freud
- Structural
1. Id -
✓ Birth
✓ Instinctive, Impulsive
✓ Immediate gratification
✓ Pleasure principle
2. Ego
✓ 4 – 6 months onwards
✓ Reality principle
3. Super ego
✓ 3 – 4 y onwards
✓ Morality principle
- Topographical
1. Conscious
2. Preconscious
3. Unconscious
Erick – Erikson stages of Development psychosocial
Age Psychosocial stage Purpose
Infancy Birth – 18 months Trust v/s Mistrust Hope
Early childhood 18 – 3 years Autonomy v/s shame and doubt Will
Late childhood 3 – 6 years Initiative v/s Guilt Purpose
School age 6 – 12 years Industry v/s Inferiority Competence
Adolescence 12 – 20 years Identify v/s Role confusion Fidelity
Early adulthood 20 – 35 years Intimacy v/s isolation Love
Middle adulthood 35 – 65 years Generatively v/s stagnation Care
Late adulthood 65 – Death Integrity v/s despair Wisdom
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❖ Play
Solitary Infant
Parallel Toddler
Imaginery Cooperative/ Associative → Pre- schoolar
Competitive Schoolar
Athletic >12 years
❖ Cognitive development theory – Jean piaget

•Stage of cognitive development.
1. Sensorimotor stage – (0 to 2 yrs.) learned by sense organ object permanent.
2. Pre-operational stage –(2 – 7 years)
- Reasoning & think to solve the problem.
- Learn to think in past, present, future.
3. Concrete operational – (7 to 11 Adulthood)
- Solving concrete problem.
4. Formal operations – (11 yrs to Adulthood)
- Abstract thinking develop.
❖ Lawrence Kohlberg – Moral development.
1. Level one → Pre – conventional mortality.
• Stage 0 – (Birth – 2years) → Egocentric Judgement.

• Stage 1 (2 – 4 years) →Punishment - obedience orientation.
• Stage 2 (4 – 7 yrs.) → Instrument Relativist orientation.
2. Level Two→ Conventional mortality.
• Stage – 3 (7 – 10 yrs.) → Good boy or nice girl orientation.

• Stage – 4 (10 – 12 yrs.) → Law and order orientation.
3. Level Three → Post – conventional mortality.
• Stage – 5 → Social contract and legalistic orientation.

• Stage – 6 → Universal Ethical principles orientation.
❖ Unconscious → Governed by pleasure principle.
❖ Conscious → Regulated by reality principle.
43
DEVELOPMENTAL & BEHAVIOURAL DISORDER

Abnormalities of Development
Developmental Delay
• Performance in 1 or more domains significantly below average.
• If developmental delay involves 2 or more domains – Global developmental delay
Dissociation
• Substantial difference in rate of development between 2 or more domains.
• Ex:- Isolated speech delay.
Deviancy
• Developmental milestones occurring out of sequence.
• Ex: Crawling comes before sitting
Developmental Red Flags (upper limit)

Gross motor Fine motor
Sitting with support 9 months Pincer grasp 12 months
Standing with support 12 months Scribbling 24 months
Walking with Support 15 months
Walking without support 18 months
LANGUAGE SOCIAL
Babbling 12 months Social smile 6 months
Single words 16 months Waving bye 12 months
Important causes of development Delay(“CDGP PIC”)

1. Chromosomal Trisomy 21, 13, 18
2. Development brain abnormalities (lissencephaly, myelomeningocoele)
3. Genetic (Fragile x syndrome, Red syndrome, prader willi syndrome)
4. Perinatal – asphyxia, HIE
5. Postnatal – Trauma, abuse, infections
6. Cong. Infection – Torch
Development Assessment (DQ)
Developmental Age
DQ = X 100
Chronological Age
Screening Test for Developmental Assessments

1. Phatak’s Baroda screening tests
2. Ages & stages questionnaire
3. Revised Denver Development screening test
4. Trivandrum development screening chart
Definitive tests for intellectual & developmental Assessment:-
Name of test Age group
Bayley scale for infant development II 1 month – 3.5 years
Wechsler intelligence scale for children IV 6 yrs – 17 yrs
th
Stanford binet intelligence scale, 5 ed 2 yrs – 65 yrs
Vineland adaptive behavior scale II Birth to 89 yrs
44
Intellectual disability (ID) /Mental Retardation (old name)

Mental Age
IQ (intelligence quotient) = X 100
Chronological Age
Degree IQ level
Mild Id 51 – 70
Moderate ID 36 – 50
Severe ID 21 – 35
Profound ID 0 – 20
BEHAVIOURAL DISORDERS IN CHILDREN
Nocturnal Enuresis
Definition – Involuntary passage of urine at night beyond 5 years of age.

Epidemiology
➢ 60% are boys
➢ Family history is positive in 50%
• If 1 parent had NE, each child has 44% risk of NE
• If both parents had NE, each child has 77% risk of NE
Types
• Primary NE – Never attained urinary continence at night; more common.
• Secondary NE – Previously normal at night & now develop NE.
Management
• 1st Line – Diet & life style changes + motivational therapy (Star chart)
• 2nd Line – Bed ard alarm technique
• 3rd Line – imipramine
Oral desmopressin – for short term
Oxybutynin
✓ Combination of Drugs and Bed & alarm technique – Lowest relapse rates
PICA
➢ Persistent eating of non – nutritive, non – foodQ

Substances over a period of at least 1 month.
➢ More common in intellectual disability & autism spectrum disorders.
➢ Rx by behavioural therapy
Thumb sucking
➢ Self soothing behaviour

➢ Common in infancy & seen in 25% of children aged 2 years.
➢ Thumb sucking beyond 5 yrs, may be a/w sequelae (Paronychia, anterior open bite)
➢ Rx by behavioural therapy
45
Bruxism (Teeth Grinding)
➢ Seen in 5-30% of children

➢ Begin in the 1st 5 years of life
➢ A/w day time anxiety
➢ Persistent bruxism can manifest as
• Muscular on temporomandibular joint pain/Dental malocclusion.
Breath Holding Spells
➢ Results from immaturity of ANS

➢ Begins around 6-18 months
➢ Triggers : Injury, anger, frustration
➢ Starts with a cry & progress to apnea syncope, tonic posturing.
➢
TICS & Stereotypes
TICS STEREOTYPIES
Sudden, non- rhythmic, rapid, recurrent, Stereotyped, rhythmic, repetitive movement
motor movements or vocalizations seen in or patterns of speech, with lack of variation
Tourette syndrome over time.
Autistic spectrum disorders
➢ Persistent impairment in reciprocal social communication & interaction & restricted, repititive
pattern of behaviour or interest.
Risk Factors
➢ Closer spacing or pregnancies.
➢ Extremely pre-term birth (<26 wks)
➢ Family members with learning / psychological problems
➢ Antenatal exposure to thalidomide, valproate, organophosphate.
➢ Antenatal rubella exposure.
Screening Tool M - Chat
(Modified checklist for autism in toddlers) for 16-30 months.
Treatment
➢ Cognitive Behaviour therapy
➢ Rx of co morbidities like atomoxetine for hyperactivity.
➢ Intra nasal oxytocin (upcoming therapy)
Attention Deficit Hyperactivity Disorder (ADHD)
➢ Persistent inattention &/or Hyperactivity – Impulsivity, that interferes with functioning or

development, present for at least 6 months, in 2 or more settings, beginning before 12 yrs of age;
must not be secondary to another disorder.
46
➢ Risk Factors
- Maternal smoking, alcohol, lead or mercury
- Genetic component – DAT 1 & DRD 4 genes
➢ Mc neuro behavioural disorder of childhood.
➢ Co - Occurs with other emotional, behavioral, language & learning disorders
➢ Approx 2% of adults have ADHD
➢ 60 – 80% of children with ADHD continue to have it in adolescence & upto 60% of adolescents
exhibit ADHD symptoms into adulthood.
➢ Treatment
- Drugs
o Methylphenidate
o Amphetamine
o Atomoxetine
RETT SYNDROME
➢ X linked dominant inheritance
➢ Common in girls
➢ Most common gene involved MECP2 gene
➢ HC is normal at birth
➢ Normal Develop for 1st 6 month of life.
Deceleration of head growth
Acquired microcephaly Delayed development
Loss of purposeful hand movements
Development of sterotypic hand wringing movement.
Gait/ posture apraxia.
➢ Associated with: Speech problems, Seizures, Breathing irregularities, Intellectual disability
47
PUBERTY & ADOLESCENCE
Adolescent Age Group

➢ Α state of transition from childhood to adulthood
➢ Puberty refers to the physical aspect of adolescence.
➢ Who definition of Adolescence → 10-19 yrs.
• Early Adolescence 10-13 yr
• Mid Adolescence 14-16 yr
• Late Adolescence 17-19 yr
Sequence of changes in puberty in females
Thelarche (Breast development) (1st visible sign)
2 – 2.5 Yrs Pubarche
Growth spurt (peak ↑in growth velocity) (earliest)
Menarche
Sequence of Changes in puberty in Males

Testicular enlargement (1st sign)
Penile enlargement
Pubic hairs Deeping or Ripening of voice
Growth spurts
Axillary hairs
Facial Hairs
How to assess pubertal development ?

Tanner’s staging or sexual maturity Rating (SMR)
➢ Stage 1 to 5
• Stage 1 Pre pubertal stage
• Stage 5 Mature adult
➢ Parameters
• In females – Breast, pubic hairs
• In males – Testis, penis, pubic hairs
48
Tanner’s staging in females and Males
Growth Spurt
➢ Occurs in which stage
• In girls – Tanner’s Stage 3
• In boys – Tanner’s Stage 4
➢ Growth spurt occurs later & lasts longer in boys.
➢ Height increased by
• Boys 20-30 cm
• Girls 16-28 cm
Problems in adolescent’s age group
➢ Nutrition & eating disorders
➢ Mental health problems
• Adjustment & anxiety disorder
• Depression
• Delinquent behavior
• Poor body image
➢ Sleep Disturbances
➢ Substance abuse – Tobacco, Alcohol
➢ Infections:-
• STD
• T.B
• HIV
• Skin
• Parasitic infection
➢ Accident MCC of mortalility
49
NUTRITION & MALNUTRITION

BREAST MILK & BREAST FEEDING
Breast feeding should be initiated → As soon as possible (or)
→ Within 1 hr of child birth
Exclusive Breast feeding
✓ Child should be fed only breast milk, nothing else, not even sips of water unless medically
indicated.
✓ Any form of prelacteal feeding is absolutely C/I
✓ Recommended for 6 months exclusively
What should be initiated at 6 months → Complementary feeding
• Semisolid energy dense food given in addition to → Breast feeding
• Should be AFASS→
- A = Acceptable
- F = Feasible
- A = Affordable
- S = Sustainble
- S = Safe
Breast milk output is maximum at :-
➢ 5-6 months of lactation
➢ 730 ml/day
For how long can expressed breast milk be stored:-
At room temperature 8-10 hrs
In a refrigerator 24 hrs
In a deep freezer (-20⁰C) 3 months
Reflexes Helping in Breast Feeding
Mother
1. Milk Secretion reflex (by Prolactin)
2. Milk ejection reflex (by Oxytocin)
Baby
1. Rooting reflex
2. Sucking reflex
Signs of good Positioning while Breast feeding
1. Body of the baby should be well supported.
2. Occiput, shoulder, & buttocks should be in a straight line.
3. Baby should be turned towards the mother.
4. Abdomen of baby should touch abdomen of mother.
Signs of Good Attachment while breast feeding
1. Mouth of the baby should be wide open.
2. Entire areola should be in baby’s mouth except a small upper part that may be visible.
3. Lower lip of baby should be everted.
4. Chin of baby should touch the mother’s breast.
C/I to Breast Feeding
Related to baby
1. Galactosemia
2. Lactose intolerance
Related to mother
Absolute C/I
• Mother on chemotherapy or radiotherapy.
Relative C/I
• Maternal HIV
50
• Maternal active untreated T.B

• Active herpes simplex
• Breast Abscess
• Maternal active varicella
Advantages of breast feeding for baby
I. Composition
1. Carbohydrates
- BM is richer in lactose (7g/dl) as compared to cm (4.5 g/dl).
- BM give more energy as carbohydrates to baby.
2. Proteins
- BM contains lesser protein (1g/dl) as compared to cm (3.5 gm/dl).
- BM is richer in whey proteins (Lactalbumin) which are more easily digestible, as compared to
casein in cm.
- BM contains adequate amount of amino acids like cysteine, taurine, methionine which are
adequate for CNS development of baby.
3. Lipids
- BM is richer in PUFA (poly unsaturated fatly acids) – beneficial for baby
- BM contains adequate amounts of DHA (Docosa hexaenoic acid) – CNS development of baby.
4. Minerals
- Ca : phosphate ratio in BM is such that it favours ca absorption (Cm richer in phosphate –
predominantly cm fed baby – more chances of hypocalcemia)
- Fe present in BM is more easily absorbable
5. Vitamins
- BM contains adequate amount of all vitamins except vitamin D 1 , Vitamin K & Vitamin B12 (in
strictly vegan mother)
- Vit K inj. At birth prevents hemorrhagic disease of newborn.
II – BM contains Anti infective Substances like
➢ TGF β (Transforming growth factor)
➢ Phagocytic macrophages
➢ PABA (PARA Amino Benzoic acid)
➢ Lactoferrin
➢ Lysozyme
➢ Antibodies especially IgA
➢ Anti staphylococcal factor
➢ Bifidus factor
➢ Bile stimulated lipase
III – BM protects Against Diseases like

➢ Neonatal period – NEC, Neonatal sepsis
➢ Later in life – Obesity, HTN, Diabetes, Allergies, dental caries
IV – BM fed babies have higher IQ

V – Helps in maternal child bonding
VI – Easily available, even in resource limited settings & is free from risk of contamination
Variations in the composition of BM

I – Depending on time after birth
1. Colostrum
a. 1st 72 hrs of birth
51
b. Thick and yellowish

c. Rich in immunoglobin, macrophages protein
d. 1st imunization of baby
e. Lactose is less
2. Transitional milk
a. Next 2 weeks
b. Composition is between colostrum & mature milk
3. Mature milk
a. Thin & watery
b. Richer in lactose & poorer in proteins
II – Depending on gestational age
Preterm BM Richer In
➢ Sodium
➢ Iron
➢ Proteins
➢ Fat
➢ Ig
➢ Calories
III – Depending of each feeding session
1. FORE MILK
a. At beginning of a feed
b. More thin & watery
c. Satisfies mainly the thirst of the baby
2. HIND MILK
a. At end of feed
b. Richer in fat
c. Thicker
d. Satisfy hunger of baby
MALNUTRITION
➢ Best indicator of Acute malnutrition ↓in wt for height (wasting)

➢ Best indicator of Chronic malnutrition ↓in Ht for age (stunting)
Classifications
I – IAP Classification
➢ Based on weight for age & edema
➢ N - >80% of expected
➢ Grades
o 71 – 80% expected
o <50%
➢ Add ‘K’ if edema is present
II – Gomez Classification
➢ Based on weight for age
➢ N – Wt for age >90% of expected
➢ Grades
- (mild) 75 – 89% of expected
- (moderate) 60 – 74% of expected
- (Severe) <60% of expected
52
III – WHO classification

Based on wt for height, ht for age & edema
Wt for height Ht for age
B/w - 2 to – 3 Z score or B/w – 2 to – 3 Z – score or
70-79% of expected WASTING 85 – 89% of expected
STUNTING
<- 3 z-score or < - 3 Z-score or
< 70% of expected < 85% of expected
SEVERE WASTING SEVERE STUNTING
➢ If edema is present, add ‘Edematous’ to the category
IV – WELLCOME Trust classification
Based on wt for age & edema
Wt for age Edema Category

60 – 80 % of expected - Under nutrition
60 – 80 % of expected + Kwashiorkor
<60 % of expected - Marasmus
<60 % of expected + Marasmic kwashiorkor
KWASHIORKOR MARASMUS
Edema Present Absent
Appetite Poor Voracious appetite
CNS involvement Apathy, listless Active child
Hepatomegaly Seen Usually not seen
Skin & Hair changes More common Less common
IN SEVERE MALNUTRITION
Skin Changes → Flaky paint dermatosis (or)
Crazy permanent dermatosis
Hair Changes → Easy pluckability
Flag sign
Flag sign Flaky paint dermatosis

SEVERE ACUTE MALNUTRITION (SAM)
Definition – In a child b/w 6 months to 5 yrs age, as presence of any 1 or more of:-
Wt for Ht < - 3 Z-Score or <70% of expected or
Mid arm circumference < 11.5 cm or
Symmetric bipedal edema of nutritional origin
Complications
S → Sugar deficiency (hypoglycemia) → BG <54 mg/dl
H → Hypothermia → Rectal temperature < 35.5⁰C
I → Infections
E
L Electrolyte imbalance (mc is Hypokalemia)
Bitot’s spot SHAKIR’S TAPE
D (Vit.A deficiency)
Dehydration
E
h
53
D→ Deficiency of micronutrients
Treatment
1. Initial hospitalization especially with poor appetite or complications
2. Look for complications & Rx
➢ Hypoglycemia 10% dextrose
➢ Hypothermia dry & warm up
➢ Infections Antibiotics
➢ Electrolyte Imbalance Supplement K+, Mg2+
➢ Dehydration WHO ORS or ReSoMal (Rehydration solution for
▪ Malnourished child)
➢ Deficiency of micronutrients Supplement multivitamins & minerals
o Fe started Later
3. Nutritional Rehabilitation
➢ Start with 70-80 K.cal /kg/day & 0.7 g/kg/day proteins,
gradually over 1-2 week (prevents refeeding / Nutritional recovery syndrome) 150 – 200 k
cal/kg/day & 4-5 g/kg /day proteins given.
CRITERIA FOR DISCHARGE FROM HOSPITAL
➢ Child should have lost edema & started gaining weight
➢ All infections & micro nutrient deficiencies should have been taken care of.
➢ Childs appetite should have improved & he should be accepting well orally.
➢ Mother/ care giver should be confident of taking care of child at home
Traffic light Diet
Category Characteristics Examples Recommended intake
Red High in calories, sugar & fat Fatty meats, sugar, sweat Reserved for infrequent
beverages, fried food treats
Yellow Nutrient dense, but higher in Lean meats, dairy, starches In moderation
calories & fat grains
Green Low-calorie, high fiber, low Fruits & vegetables Without any limitations
fat, nutrient-dense
Recommended Daily Energy Requirement

Body weight Recommended daily energy requirement
< 10 kg 100 Kilo calories/Kilogram
10 – 20 kg 1000 Kilo calories + 50 Kilocalories/Kilogram Each Kg above 10 KG
> 20 kg 1500 Kilo calories + 20 Kilocalories/Kilogram Each Kg above 20 KG
Recommended daily protein requirement

Group Age Protein requirement
(g/day)
Infants 0 – 6 months 1.2 g/kg/day
6 – 12 months 1.7 g/kg/day
Children 1 – 3 yrs 17
4 – 6 yrs 20
7 – 9 yrs 30
10 – 12 yrs 40
Boys 13 – 15 yrs 54
Girls 13 – 15 yrs 52
Boys 16 – 17 yrs 62
Girls 16 – 17 yrs 56
54
FLUID & ELECTROLYTE DISTURBANCES

BODY COMPOSITION
Total body water (TBW)
➢ TBW constitutes
- 90% of body cut in early fetal life
- 75% of body cut in early at birth
- 60% of body cut in by end of 1 year and remain till puberty
➢ Premature infants have higher TBW than tern Infants
➢ Divided Into:-
- ECF Volume
- ICF volume
ECG & ICF Volume
➢ In fetus & new born, ECF > ICF volume
➢ Normal post natal diuresis – immediate ↓in ECF volume
➢ Cellular growth – continued expansion of ICF volume
➢ By 1 yr age, ICF : ECF volume approaches adult levels
➢ ICF volume is 30 – 40% of body weight & ECF volume is 20 – 25%
Disorders of Acid Base Balance
Metabolic Acidosis in children
➢ MC causes of metabolic acidosis in children – Diarrhoea
➢ Amount of bicarbonate lost, depends on the volume of diarrhea & the bicarbonate concentration
of stool
➢ Metabolic acidosis with normal anion gap
• Post hypocapnea
• Urinary tract diversions
• Renal tubular acidosis
• Diarrhoea
• Ammonium chloride intake
➢ Increased Anion Gap metabolic Acidosis
• Keto acidosis, kidney failure
• Lactic acidosis, liver failure
• Maliganancy, medications
• Issue hypoxia
• Inborn errors of metabolism
• Poisoning Ex- Ethylene glycol
Metabolic alkalosis in children

MC secondary to emesis or diuretic use
Important causes of metabolic Alkalosis
Chloride Responsive (urinary chloride < 15 meq/l)
➢ Gastric losses
➢ Emesis
Volume repletion is necessary
➢ Naso gastric suction
for correction of alkalosis
➢ Diuretics (loop or thiazide)
➢ Elevated CO2 (post – hypercapnia)
➢ Raised sweat chloride (cystic fibrosis)
Chloride – Resistant (Urinary chloride > 20 meq/L)
High Blood pressure
➢ Adrenal adenoma or hyperplasia
➢ Gluco corticoid – remediable aldosteronism
55
➢ Renovascular disease
➢ Renin – secreting tumor
➢ Cushing syndrome
Glucocorticoid – Remediable Aldosteronism (GRA)
➢ Autosomal Dominant
➢ Excess production of aldosterone owing to presence of an aldosterone synthase gene that is
regulated by ACTH
➢ Glucocorticoids – Inhibit ACTH production by pituitary – Down regulate the inappropriate
aldosterone production
Liddle syndrome/ Pseunopyperaldosteronism
➢ Autosomal dominant disorder
➢ d/t an activating mutation of Na Channel in distal nephron
➢ Up regulation of this Na channel is continuously open, features of hyperaldo-steronism →
Hypertension, hypokalemia & metabolic alkalosis, but low serum levels of aldosterone seen.
Bartter & Gitelmen syndrome

Bartter syndrome Gitelman syndrome
Hypokalemic metabolic alkalosis with hyper Hypokalemic metabolic alkalosis, with
calciuria & salt wasting hypocalciuria & hypomagnesemia
Polyuria, polydipsia, vomiting, constipation, FTT, Recurrent muscle cramps & spasms
BP : low normal
Mutations in genes that encodes :- Na+, K+, 2Cl- Mutations in sodium chloride co-transporter
transporter (NKCC2), Luminal K channel (ROMK), (NCCT), present in the distal convoluted tubule
or Cl, Channel (CLC – Ka, CLC – Kb)
Rx:- K supplementation & Indomethacin Rx:- K & Mg supplementation
DISORDERS OF SODIUM
Important causes of hypernatremia
Excessive Sodium Water Deficit Water & Na Defictis
Improper feeds Diabetes insipidus GI loss – Diarrhoea ,vomiting
Excess Na Bicarbonate Increased insensible Cutaneous losses – burns
IV – hypertonic saline Losses (pre mature) Infants, Renal losses – ATN (polyuric
Warmers phase), CKD
Hyperaldosteronism Inadequate intake (Ineffective Post obstructive diuresis osmotic
breast feeding, adipsia) diuretics Diabetes
Important causes of Hyponatremia
Hypovolemic Euvolemic Hypervolemic
GIT/ skin losses:- SIADH Heart failure
Use of diuretics hypothyrodism cirrhosis
Obstructive uropathy Glucocorticoid deficiency Nephrotic syndrome
Desmopressin Renal failure
Water intoxication Hypoalbuminemia
Pseudohyonatremia can be seen in hyperglycemia, mannitol, sucrose intake
56
Diagnostic criteria for SIADH

Presence of Absence of
Urine osmolality > 100 mosm/kg Renal, adrenal, thyroid insufficiency
Urine sodium > 30 mEq/L Heart failure, nephrotic syndrome of
Serum osmolality < 280 mosm/ kg Cirrhosis
Serum sodium <135 mEq/L Diuretic ingestion
Correction with water restriction Dehydration
Disorders of Potassium
Hypokalemia (Serum K+ < 3.5 mEq/L)
Causes
➢ ↑ losses → Diarrhea
➢ ↓ stores → Malnutrition
➢ Shift into intra cellular compartment – Alkalosis
➢ Renal → renal tubular acidosis, cystic kidneys
➢ Endocrine → Cushing syndrome, Hyper aldosteronism
Clinical features
➢ Muscle weakness
➢ Hypotonia
➢ Paralytic ileus
➢ Constipation
➢ Polyuria & Polydipsia
Management
➢ Deficit of K+ should be corrected over a 24 hr period
➢ IV correction
• If serum K+ 2.3 mEq
• ECG changes
o Cardiac rhythm disturbances
o Unable to take orally
➢ Oral Dose :→ 2-4 mEq/kg/day
➢ IV dose :→ 0.5 – 1 mEq/kg, usually given over 1 hr.
➢ Infusion fluid should ideally not contain > 40 mEq/L of K
Hyperkalemia (K+ > 5.5 mEq/L) – ETIOLOGY
Decreased Excretion Transcellular shifts
Renal failure Acidosis
Addision disease Rhabdomyolysis / hemolysis
21 – Hydrosylase deficiency Tumor lysis syndrome
Hyporeninemic hypoaldosteronism Drugs :- Succinyl choline
Urinary tract obstruction Digitalis, β blockers
Sickle cell disease Malignant hyperthermia
Pseudohypoaldosteronism I & II Hyperkalemia periodic paralysis
Medications : Ach inhibitors,
Angiotensin blockers, k- Sparing diuretics
ETIOLOGY
Spurious Laboratory value Increased intake
Hemolysis Intravenous or oral
Tissue ischemia during blood drawing Blood transfusions
Thrombocytosis / Leukocytosis
57
Management
1. MILD (5.5 – 6 mEq/L) → Stop K intake & offending drugs
2. Moderate (6 – 8 mEq/L) → Sodium bicarbonate / glucose insulin infusion
3. Severe (> 8 mEq/L) → IV calcium gluconate (10%)
→ Nebulized salbutamol
→ IV calcium Gluconate reverses the cardiac effects immediately
→ Nebulized salbutamol also rapidly lowers serum K+
4. Refractory hyperkalemia → Hemodialysis
5. Long term management → K+ binding resins are used.
IV Fluids in children
Indications
➢ Maintenance IV fluids → A child who can not fed enterally
➢ Replacement fluids → Continued excessive losses, such as
• drainage from a NG tube (or)
• High urine output because of nephrogenic diabetes insipidus
➢ Deficit Replacement → If dehydration present
Q. 24 hr normal fluid requirement for a 2 yr old child weighing 12 kg is.
A. 1000 ml
B. 1100 ml
C. 1200 ml
D. 1800 ml
Ans:- B
Calculation of maintenance fluid requirement in children (Per day)
• For 1st 10 kg → 100 ml/kg
• For next 20 kg → 50 ml/kg
• Beyond 20 kg → 20 ml/kg
• Eg: 18 kg (10 x 100) + (8 x 50) = 1400 ml
Hourly maintainance fluid rate

Body Weight Maintenance fluid rate
0 – 10 kg → 4 ml / kg / hr
10 – 20 kg → 40 ml / hr + 2 ml/kg/hr x (wt – 10 kg)
> 20 kg → 60 ml /hr + 1 ml/kg/hr x (wt – 20 kg)
Eg: 18 kg → (10 x 4) + (40 + (8 x 2)) → 40 + 16 = 56 ml/hr.
Composition of IV fluids
Fluid Na+ Cl- K+ Ca2+ Lactate
Normal saline (0.9 % Nacl) 154 154 - - -
Half normal saline (0.45% Nacl) 77 77 - - -
Ringer lactate 130 109 4 3 28
➢ Isotonic fluids without glucose are used for acute correction of Intra vascular volume depletion.
➢ D5 + ½ NS + 20 mEq/L KCL is recommended in a child who is NPO (Nil per oral) & does not have
volume depletion (Usual maintenance fluid recommended in children).
Intravenous fluids in Neonates

Day fluid requirements during 1st week of life (ml/kg/day)
Birth wt Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 ≥ Day 7
< 1500 g 80 95 110 120 130 140 150
≥1500 g 60 75 90 105 120 135 150
➢ Initial fluids → 10% dextrose with no electrolytes
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➢ After 48 hrs = Na and K+ added

Shock
➢ An acute syndrome characterized by inability to deliver adequate O2 to meet metabolic demands
of vital organs & tissues.
➢ Types
o Hypovolemic
o Cardiogenic
o Distributive
o Obstructive
o Septic
Septic shock is a combination of:-
1. Hypovolemia from intra vascular fluid losses, through capillary leak
2. Cardiogenic shock from myocardial depressant effects of sepsis
3. Distributive shock from decreased systemic vascular resistance
Primary initiating factor in septic shock is cytokine release
Hypovolemic shock
➢ MC cause of shock in children worldwide.
➢ →Compensatory mechanisms are seen in early phases of shock.
➢ ↑HR
To maintain BP & tissue perfusion
➢ Stroke volume & Vascular smooth muscle tone
2 most important corner stones in Mx of children with sepsis
✓ Early identification
✓ RX with appropriate antibiotic
Management:
Child with shock
0 min → Begin high flow O2 & establish IV/ID access
5 min → Push boluses of 20 cc/kg isotonic saline up to & over 60 cc/kg until perfusion improves are
unless rales or hepatomegaly develop correct hypoglycemia & hypocalcemia. Begin
antibiotics.
Shock not reversed

15 min → Fluid refractory shock :→ Begin inotrope (Dopamine) obtain central access & airway if needed.
Reverse cold shock by titrating dopamine or, if resistant, titrate epinephrine, reverse warm shock
by titrating nor epinephrine
Shock not reversed
60 min → Catecholamine resistant shock: Begin hydrocortisone
Monitor CVP is PICU: Attain normal MAP – CVP & SCVO2 > 70%, maintain Hb > 10g/dl.
Cold Shock with Normal BP Cold Shock with low BP Warm Shock with Low BP
Titrate Epinephrine Titrate Epinephrine Titrate Nor – epinephrine
If SCVO2 still <70% add Consider nor epinephrine If still hypotensive consider
vasodilator with volume loading vasopressin, teripresssin or
nitroso vasodilators, milrinone, angiotensin
consider levosimendan
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If SCVO2 still <70% Consider If SCVO2 still <70% consider low

dobutamine, milrinone, dose epinephrine
enoximone, or levosimendan
Shock not reversed
Persistent catecholamine resistant shock : Rule out & correct pericardial effusion, pneumothorax
Shock not reversed
Use ECMB ( Extra corporal membrane oxygenation)

What is new in septic shock Management
➢ Eliminated protocolized approach
➢ It must be induvidualized to suit the patient physiology & setting.
➢ Crystalloids whether balanced or not, are the most preferred.
➢ Restricting maintainance fluids after initial fluids resuscitation & use of diuretics for fluid removal
termed as “de-resuscitation” is a/w more ventilator free days & shorter length of ICU stay.
Monitoring of Fluid Replacement Responsiveness:-

Poorly predictive
➢ Clinical sings (HR & SBP)
➢ Static variables (CVP & preload estimates) form thermodilution
Consistent Predictor
➢ Among dynamic values, respiratory variation in aortic blood flow peak velocity.
Good Predictor
➢ At bed side, hemodynamic changes induced during passive leg raising test.
GENETICS (Genetic Disorders)

Types of Genetic Disorders
Classification
Type of Disorder Description
Single gene Single genes are altered
Follow mendelian mode of inheritance
Eg: Hemophilia
Chromosomal Entire chromosome or segments of them are:
Disorders - Missing.
- Duplicated or altered
Can affect chromosomal number or structure
Eg:- Down’s Syndrome
Non – Mendelian 1. Trinucleotide repeats imprinting
Disorders 2. Mitochondrial Inheritnace mosaicism
3. Genomic
4. Gonadal
Multifactorial Results from a combination of multiple genetic & environmental causes
Disorders Eg:- Cleft palate
Neural tube defects
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Mendelian Disorders
Autosomal Dominant Disorders
➢ Manifest even if only one of the alleles of the abnormal gene is affected
➢ Examples:-
• Hyper cholesterolemia, hereditary spherocytosis, HNPCC
• Ehler danlos syndrome (Except type VI)
• Adenomatous polyposis coli
• Von Willebrand disease
• Pseudohypoparathyroidism
• Oxystrophia myotonica
• Osteogenesis imperfecta
• Marfan syndrome
• Intermittent porphyria
• Neurofibromatosis 1 and 2
• Achondroplasia, adult polycystic kidney disease
• Noonan’s syndrome
• Tuberous sclerosis
Autosomal Recessive Disorders
➢ Manifest only if both the alleles of the abnormal gene is affected
➢ Examples:-
• Albinism, Alkaptonuria, Ataxia telangiectasia
• Beta (Thalassemia, sickle cell anemia)
• Cystic fibrosis, Congenital adrenal hyperplasia
• Deafness (sensorineural)
• Emphysema (α – 1 antitrypsin deficiency)
• Friedrich’s ataxia
• Gaucher disease, Galactosemia
• Homocystinuria,H emochromatosis
• Inborn errors of metabolism
X Linked Recessive disorders
➢ Males are mc affected
➢ Affected males have carrier daughters & unaffected sons because they pass their X chromosome
to their daughters & Y chromosome to sons.
➢ Male – to – male transmission excludes X – linkage
➢ Examples:
• G6PD deficiency
• Duchenne muscular dystrophy
• Color blindness
• Fragile – x – syndrome, fabry disease
• Chronic granulomatous disease
• Hemophilia A & B hunter disease
• Albinism
• Lesch – Nyhan syndrome, lowe syndrome
X Linked Dominant Disorders
➢ All daughters, but no sons of an affected man have the disease
➢ Examples:-
• X – linked hypophosphatemic rickets
• Rett syndrome
• Fragile x syndrome
• Alport syndromes
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• Charcott – Marie – tooth disease

How to interpret?
Pattern Inheritance
All or most children of a mother are affected Mitochondrial
If at least one of the parents always have the disorder Dominant
If neither parent has the disorder because they are heterozygous Recessive
If both males and females are affected, with almost equal frequency Autosomal
Father to son transmission of trait does not occur X – linked
More males affected; affected sons usually born to unaffected mother X – linked recessive
More females affected; affected sons must have an affected mother X – linked dominant
It is passed from father to all sons Y – linked dominant
Mitochondrial disorders
➢ Mitochondrial DNA present in cytoplasm
o Exclusively derived from maternal side (ovum),
o As only head of sperm (form father) contributes in zygote formation
➢ Both male & female children born to a affected mother inherit the disease.
o As all offsprings receive their mitochondrial DNA from their mother only.
➢ Proportion of mutated mitochondrial DNA (multation load) determines phenotypic expression
called “Threshold of expression”
➢ Example:-
CPEO (Chronic progressive External Opthalmoplegia)
• B/L ptosis
• Ophthalmoplegia
• Proximal muscle weakness
Pearson syndrome
• Pancreatic insufficiency
• Pancytopenia
• Lactic Acidosis
Down syndrome
➢ MC chromosomal abnormality
➢ Incidence → 1 : 800 – 1 : 1000 live births
• 95% - maternal non disjunction
• 3% - Translocation
• 1-2% Mosaicism
Clinical features
CVS
• Endocardial cushion defects (mc CHD)
• VSD, ASD, PDA
CNS
• Delayed development / intellectual disability
GTI
• TEF
• Duodenal atresia (mc cause of intestinal obstruction)
• Annular pancreas
• Hirshsprung disease
• Impeforate anus
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Important features
• Incurved 5th finger (Clinodactyly)/ Intellectual disability
• Congenital heart disease / Congenital hypothyroidism
• Acute leukemia / Alzheimer’s disease / Atlantoaxial instability
• Protruding tongue
• Round face
• Occipit flat/ open, wide fontanellae
• Brushfield spots in iris/ Brachycephaly
• Low (depressed) nasal bridge, low tone (hypotonia)
• Epicanthic fold/ Ears low set & dysplastic
• Mongoloid slant (Oblique palpebral fissure)
• Sandle gap/ Simian palmar crease
Risk of Down syndrome (Maternal Age)

Maternal Age Incidence of Down syndrome
20 years 1 in 1500
30 years 1 in 900
40 years 1 in 100
45 years 1 in 30
First Trimester Screening

Screening Method Detection Rate
Maternal age + Biochemical markers (β – hcG, PAPPA) 70%
Maternal age + Radiology (USG – NT > 3 mm) 80 – 83%
Combined Test 82 – 87%
Maternal age + Radiology + Biochemical markers
Second Trimester Screening

Screening Method Detection Rate
Triple test (β HCG + AFP + U estriol) 67%
Quadruple test (Triple test + inhibin A) 77%
Integrated test (Best test) 94 – 96%
Maternal age + T1 – (NT > 3 mm + PAPPA) + T2 – (Quadruple test)
Confirmatory test for prenatal diagnosis of Down syndrome

Method Gestation Remarks
Chrionic villi Sampling (CVS) 11 – 13 week ↑ risk of abortions & fetal limb defects
Amniocentesis 14 – 16 week Complications
→ Pregnancy loss
→ Amniotic fluid leakage
→ Vaginal bleeding
Cordocentesis (or) percutaneous 18 – 20 week Fetal blood can be analyzed directly by HPLC
umbilical cord blood sampling after ensuring that there is no maternal
(PUBS) contamination
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TURNER SYNDROME
➢ 45 XO
➢ Female sex
➢ Infertile
➢ Short stature & webbed neck
➢ No intellectual disability
➢ Bicuspid aortic valve (50%)
➢ Coarctation of aorta (30%)
➢ Aortic stenosis
➢ Mitral valve prolapse
➢ Short 4th Metacarpal
Important Features
Short stature, sensorineural hearing loss, short 4th metacarpal
Amenorrhea (primary)
Barrbody absent
Cardiac anomalies
Ovaries streaky
Webbed neck
Nipple widely placed
OTHER GENETIC SYNDROMES

Noonan’s Syndrome
• Autosomal dominant, normal karyotype
• PTPN 11 gene (mc), Fertile
• Short stature, webbed neck
• Cubitus valgus
• Clinodactyly, cryptorchidism
• Supra valular PS (mc), HOCM, ASD
• Intellectual disability present
Trisomy 18 (Edwards syndrome)
Rocker bottom foot
Overlapping fingers
Cardiac defects
Kidney malformations
Micocephaly
Mental Retardation
Trisomy 13 (Patau syndrome)

More severe craniofacial & midline defects than trisomy 18 or 21.
Important features
Cutis aplasia
Microphthalmia, microcephaly
Congenital heart disease
Deafness
Rib abnormalities
Developmental delay
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INBORN ERRORS OF METABOLISM

• Disorders of carbohydrate metabolism
• High index of suspicion is a must: if missed may prove fatal
• In neonates & infants
➢ Deterioration after a period of apparent normally
➢ Rapidly progressive encephalopathy & seizures
➢ Sepsis like presentation with negative sepsis screen
➢ Persistent / recurrent vomiting, pecullar body / unine odour
➢ Parental consanguinity, significant family history
➢ Severe metabolic acidosis, ketosis & hypoglycemia
Clinical Pointers for specific IEMS

Clinical finding Disorder
Coarse facies Lysosomal disorders (MPS)
Cataract Galactosemia, Wilson, DM
Retinitis pigmentosa Mitochondrial disorders
Cherry red spot GM 1 gangliosidosis, NPD, Tay Sachs
Eczema/ alopecia Biotinidase deficiency, MCD
Abnormal kinky hair Menke disease
↓ Pigmentation Phenyl ketonuria, albinism
MPS → mucopolysacchride
NPD → Nieman pick
MCD →Multiple carboxylase deficiency.
Disorder of carbohydrate metabolism
• Glycogen storage diseases
• Galactosemia
• Heriditary fructose intolerance
Glycogen Storage diseases (GSDs)
Q. Child with recurrent hypoglycemic attacks & hepatosplenomegaly is likely to have?

A. VON GIERKE disease
VON GIERKE Disease

➢ Most common GSD in children
➢ Autosomal recessive
➢ Type I a → Glucose & phosphatase deficiency
➢ Type I b → Translocase deficiency
➢ Clinical features
• Fasting hypoglycemia +/- Seizures
• Hepatomegaly
• Doll like facies
➢ Investigations
• Hypoglycemia Hyperuricemia
• Lactic acidosis Hyperlipidemia
➢ Treatment
• Corn starch diet
• Symptomatic Rx
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Important glycogen storage Diseases

Liver glycogenosis Enzyme Deficiency
I- VON Gierke Disease Glucose & phosphatase
II – Cori Diesease Debranching enzyme
III – Anderson disease Branching enzyme
IV – Hers disease Liver phosphorylase
Muscle glycogenoses Enzyme deficiency
II – Pompe disease Acid maltose
V – MC Ardle disease Muscle phosphorylase
VII – Tarui disease Phosphofructokinase
Differences between type I and Type III GSD
Type 1 GSD Type III GSD
Kidneys Enlarged, spleen normal Normal, splenomegaly seen
Muscles Not involved May be involved
CK level Normal May be elevated
LFT Usually normal Transaminitis & fasting ketosis +
Lactate Elevated Usually normal
Effect of glucagon No rise in blood glucose, but lactate level 2 hrs after meal → ↑in blood glucose
rises After fast → no increase
Galactosemia
➢ Deficiency of
• GALT (Galactose 1 PO4 uridyl transferase) (mc)
• Galactokinase
• Epimerase
➢ Sepsis with E.coli is common
➢ Breast milk is Contraindicated
Clinical features:-
• Jaundice
• Hepatomegaly
• Seizures +/- MR
• Cataract
• Diarrhoea / vomiting
• Failure to thrive
➢ Treatment → Avoid milk and milk product so lactose free formula use
Hereditary fructose intolerance
➢ Aldolase B deficiency
➢ Symptoms appear on intake of fructose
➢ Aversion to sweet food
➢ Clinical features:-
• Hypoglycemia
• Jaundice
• Hepatomegaly
• Vomiting
➢ Reducing substance in urine Positive – Benedicit’s Test
➢ Treatment – avoid fructose (sweet food)
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Disorders of Amino Acid Metabolism

Diseases of Phenyl Alanine Pathway
➢ Phenylketonuria
➢ Alkaptonuria
➢ Tyrosinemia
HERTNUP Disorder
HOMOCYSTINURIA
Phenylalanine Metabolism
Phenylketonuria (PKU)
➢ Deficiency of phenylalanine hydroxylase
➢ Phenylalanine accumulate in body & will convert into phenylactate
➢ Children born to mothers with PKU have:-
• Microcephaly
• Mental retardation
• Growth retardation
• Congenital heart disease
Clinical features of PKU
• Blonde hair, blue iris, fair skin
• Musty/ mousy body odour
• Microcephaly
• Intellectual disability
• Growth retardation
• Dental enamel changes
• Neurological features:- Seizures, irritability,hypotonia.
Diagnosis
→ FeCl3 Test with urine Green colour
Detects phenyl alanine in urine
➢ Guthrie’s test → Detects phenylalanine in serum
➢ ↑ Blood phenylalanine level
Treatment
➢ Low phenyl alanine diet as soon as possible

➢ Adequate intake of tyrosine should be ensured
Alkaptonuria
• Oochronosis (dark spot on sclera/ear cartilage)
• Arthritis
• Darking of urine on standing
• Cardiac involvement → Mitral or aortic valvitis or calcification
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Tyrosinemia
Typosinemia Type I
➢ Severe disease of kidney, liver & peripheral nerves
➢ Deficiency of fumaryl acetoacetate hydrolose (FAH)
➢ ↑ serum AFP & Succinyl acetone in serum & urine
➢ Treatment – Nitisinone (inhibits tyrosine degradation at 4 – HPPD)
Tyrosinemia Type II
➢ Deficency of tyrosine aminotransferase
➢ Plamar / plantar hyperkeratosis
➢ Corneal ulcers & intellectual disability seen
Tyrosinemia Type III
➢ Deficiency of 4 hydroxy phenyl pyruvate deoxygenase (4 HPPD)
Hartnup Disorder
➢ Defect in transport of monoamino – mono carboxylic aminoacide by intestinal mucosa & renal
tubules
➢ Auto somal recessive
➢ Defect in SLC6A1q gene on chr 5p15
➢ Most children remain asymptomatic
➢ Cutaneous photo sensitivity & pellagra like rash.
➢ Amino aciduria restricted to neutral amino acids (eg. Valine, leucine, phenyl alanine, tyrosine,
tryptophan).
➢ Rx – Nicotinic Acid or Nicotinamide or high protein diet.
Homocystinuria
➢ Caused by cystathionine β synthase deficiency
• Failure to thrive
• Developmental delay
• Seizures
• Behavioral problems
Skeletal abnormalities resembling marfan syndrome seen like
➢ Tall stature
➢ Arachnodactyly
➢ Scoliosis
➢ Pectus excavatum etc.
Complications
➢ Stroke
➢ Spontenous pneumothorax
➢ Acute Pancreatitis
Diagnosis
➢ ↑ methionine & homocysteine in body fluids
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➢ Enzyme assay in liver biopsy / fibroblasts (or)

➢ DNA analysis
Treatment
➢ High doses of vit. B6 & folic acid
➢ Restriction of methionine
➢ Assestive supplementation
LYSOSOMAL STORAGE DISEASES

Disease Enzyme Deficiency Clinical Features
GM1 Gangliosidosis β galactosidase Cherry red spot +
Visceromegaly +
Skeletal lesions +
Gaucher Disease Glucocerebrosidases Visceromegaly +
Sketelal lesions +
Neiman pick disease Sphingomyelinase Cherry red spot +
Visceromegaly +
No Sketelal lesions +
Tay sachs disease Hexosaminidase A Cherry red spot +
No visceromegaly
No skeletal lesions
➢ All mucopolysaccharidoses are autosomal recessive
✓ Except hunter Disease (X linked inheritance)
Niemann pick Disease (NPD)

➢ Type A & B results from deficient acid sphingomyelianase, ancoded by a gene on chr 11
(autosomal recessive)
➢ Type A – Rapidly progressive neurodegenerative disorder
• Hepatosplenomegaly
• Lymphadenopathy
• Death by 3yr or age
➢ Type B – Non neuronopathic form in children & adults
• Hepatosplenomegaly
• Cherry red spot
• Pulmonary involvement (diffuse reticular or finely nodular infiltration on CXR)
➢ Type C
• Neuronopathic form that result from defective cholesterol transport
• Often present with prolonged neonatal jaundice
Gaucher’s Disease
➢ MC lysosomal storage disease
➢ Accumulation of glucocerebroside inside cells
➢ Wrinkled paper apperance
➢ Splenohepatomegaly (spleen> hepatic)
➢ Bone pain pathological fracture of bone d/t infiltration
➢ Pancytopenia
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• Fatigbility d/t anemia

• Leukopenia recurrent infection
• Thrombocytopenia → Bleed
➢ Diagnosis
o Deficeincy of glucocerebrrosidase in leukocyutes / fibroblasts
o Gaucher cells in bone marrow
(Wrinkled paper appearance ofcytoplasm)
o X-ray long bones – Erlenmeyer Flask deformity
➢ Treatment
o Enzyme replacement therapy
o Stem cell transplantation
Metabolic diseases for which enzyme replacement therapy (ERT) is available
1. Gaucher Disease
2. Pomple disease (GSD type II)
Fabry Disease
➢ Deficiency of α galactosidase
➢ X – linked recessive
➢ Angiokeratomas, hypohidrosis, corneal & lenticular opacities & acroparesthesias
➢ Angiokeratomas are most dense between umbilicus & knees in ‘bathing trunk area’.
➢ Pain is the most debiliating symptom
➢ Vascular diseases of kidney heart & brain develop
Lesch Nyhan disease
➢ Hypoxanthine guanine phosphoribosyl transferase deficiency
➢ X linked inheritance
➢ Asymptomatic at birth
➢ Development delay
➢ Neurological→ Dystonia, Dysarthria, Spasticity
➢ Definitive Dx – analysis of HPRT enzyme
➢ Rx – High fluid intake, alkalinization & allopurinol
Reye’s syndrome / Jamshedpur fever
➢ Acute metabolic disorder resulting in generalized mitochondrial dysfunction d/t inhibition of fatty
acid oxidation.
➢ Characterised by
• Fatty liver
• Encephalopathy seen d/t hepatic impaired or encephalopathy.
• Parcipitated by drugs,toxins,virus.
• Administration of aspirin and aspirin-containing products is not recommended for
children with a febrile illness or children with varicella or influenza because of its
association with Reye’s syndrome
➢ Jaundice is infrequent in Reye’s Syndrome
➢ Seizures occur in > 80% patients
➢ Drugs toxins, virus, IEM can precipitate
➢ Viruses Influenza A & B, Varicella, Adeno, coxackie A.
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Not caused by RSV

➢ Encephalopathy (liver damage & cerebral edema)
➢ Prognosis is poor (25 – 70% mortality)
Nursing Interventions
1. Provide rest and decrease stimulation in the environment.
2. Assess neurological status.
3. Monitor for altered level of consciousness and signs of increased ICP.
4. Monitor for signs of altered hepatic function and results of liver function studies.
5. Monitor intake and output
Bacterial Infections
Scarlet Fever
Group A B – Hemolytic streptococci.Q
Rheumatic fever, glomerulonephritis
C/F:-
1. Pharyngitis / Tonsillitis
Rarelymembrane
➢ Petechiae over plate
➢ Tender cervical lymphadenopathy
2. Rash
➢ Day 2 of fever
➢ Face downwards
➢ Sand paper appearance
➢ Flexures – PASTIA lines
3. Strawberry tongue
❖ Dick test is done for →Scarlet fever
Rx.--> Penicillin
Diphtheria
Corynebacterium diphtheriaeQ
C/F:→
1. Pharyngeal infection – pseudomembrane ( necrosis & exudates)
Adherent
2. Bull neck appearance – Cervical lymphadenopathy
Wall edema
3. Laryngitis – Cough, Stridor
4. Rarely, nasal – unilateral serosanguinous purulent discharge
Rx:- Antitoxin, antibiotics
DIPHTHERIA VS STREPTOCOCCUS
Diphtheria Streptococcus
1. High grade fever 1. Low grade fever
2. Toxic ++ 2. Less toxic
3. Extension membrane 3. No extension
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Complications of Diphtheria
2nd week
1. Airway obstruction
2. Myocarditis
3. Polyneuritis
Pertussis
Bordetella
C/F:
➢ 1st 2 weeks – catarrhal phase → Most infectious
➢ 2-6 weeks – cough → ‘whoop’Q
▪ Post tissue vomiting
➢ >6 weeks → Convalescence
In young infants (<6 months)
➢ Maternal antibodies – not protective
➢ Whoop – not seen
➢ ↑risk of apnea
➢ ↑risk of mortality
Diagnosis:- Cough plate culture
Medium - BORDET GENGOU
Rx:- Macrolides
ENTERIC FEVER
Salmonella fever
Faeco – oral transmission
C/F:- 1st week
➢ Fever – step ladderQ
➢ Relative Bradycardia
➢ Coated tongue
➢ Bloated abdomen
Diagnosis:-
• Blood culture
2nd week
➢ Rose spots
➢ Splenomegaly
➢ Complications: → Intestinal perforation
→ Encephalopathy – status typhosus
• Widal test
rd
3 week
→ Convalescence
• Stool culture
th
4 week – Urine culture
Gold standard for diagnosis in children → Bone marrow culture
Rx:- Chloramphenicol
Amoxicillin Resistance – Multi drug resistant S. typhi.
Cotrimoxazole
Fluroquinolones – resistance →Nalidixic acid resistant S. typhi
Drug of choice:→ 3rd generation cephalosporins.
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Uncomplicated – Cefixime (oral)

Complicated – Ceftriaxone
Parenteral
Cefotaxime
Tetanus
Clostridium tetani spores
Toxin:→ Tetanospasmin
Tetanolysin
C/F:
Spasm of muscles : Face →below
1. Lock jaw – masseter
2. Grimace – Risus sardonicus
3. Ophisthotonus
Newborn – Feeding difficulty
➢ Classical tetanus
➢ Neonatal tetanus
➢ Cephalic tetanus
• Head injury; chronic otitis mdia
• ↑mortality
• Bulbar palsy
Rx:- Antitoxin, antibiotics, Diazepam, active immunization (after recovery)
Prophylaxis:
Tetanus toxoids, Tetanus immunoglobulin
Clean (minor) All other wounds
History TT TIG TT TIG
<3 doses; Yes No Yes Yes
unknown;
immunodeficient
>3 does TT No NO No No
Yes → If >10 Yes → If >5 yrs
yrs from last from last dose
dose
VIRAL INFECTIONS
Exanthematous Fever
Fever + Rash
Rash – Day of fever 1st - Varicella, rubella
2nd - Scarlet fever
3rd - Small pox
4th - Measles, Exanthema subitum
5th - Typhus
6th - Dengue
7th - Typhoid
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Measles
Measles virus
C/F: →1st 3 days – fever, cough coryza, conjuctivitis
Days 2 – KOPLIK spots → disappear by day 3.
Day 4 – Rash
• Maculopapular rash
• Face (behind ears) →downwards
• Disquemation
Complications:
Acute Chronic
• Otitis media – most common Subacute sclerosing Panencephalitis
• Pneumonia (Severe)
- 2⁰ bacterial
- Virus (giant cell / Hecht pneumonia)
Rubella
Rash – Day 1
NO desquamation
Posterior cervical lymphadenopathy
Varicella
Characteristic – vesicles
➔ Dew drops on rose petal
➔ Day 1 of fever
➔ Pleiomorphic
Complications :- 2° bacterial infection – staphylococcus.
MUMPS
Incubation Period → 2 to 3 wks
Route of Spread → Droplet
C/F:-
Parotid swelling → Bilateral (75%)
• Angle of jaw obscured
• Ear lobe is lifted up.
Complication: -
1. Meningoencephalitis → most common
2. Orchitis → postpubertal males
3. Oophritis
4. Pancreatitis
Infectious Mononucleosis
EBV
Adolescents → Kissing, sexual contact.
<4 yrs of age→ mild / asymptomatic.
C/F: -
Sore throat
74
Rash:→ Ampicillin
➔ Generalized lymphadenopathy
➔ High grade fever
➔ Splenomegaly – soft, enlarged → traumatic rupture
Diagnosis:→
Screening:-
1. PAUL – BUNNELL test – Heterophill antibody
2. Atypical lymphocytes – DOWNEY cells > 20%
Diagnostic:- IgM viral capsid antigen
EBV
Benign Malignant
Oral hairy leukoplakia Nasopharyngeal Carcinoma
Burkitt’s lymphoma
Hodgkin’s
DUNCAN’s disease
Dengue
C/F:→ Thrombocytopenia – bleeding
Fluid accumulation + Hypovolemic shock
NOTE:→ Best marker for diagnosis of dengue infection – NS1 antigen (Febrile Phase)
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HIV
Staging
1. Stage 1 - Asymptomatic
- Persistent generalized lymphadenopathy
2. Stage 2 - Mild
- Unexplained hepatosplenomegaly, unexplained parotid enlargement.
- Herpes zoster
- Molluscum
3. Stage 3 - Moderate
- Long duration
- Diarrhoea (>14 days)
- Fever (>1 month)
- Unexplained weight loss
- Candidiasis
- Oral hairy leukoplakia
- Lymphoid interstitial pneumonitis
4. Stage 4 - Severe immunodeficiency
- CMV
- Extrapulmonary TB
- Toxoplasmosis
- Pneumocystis carini
Diagnosis:→ Western blot / ELISA – Antibodies

Treatment Guidelines for HIV
HARRT – 2 NRTI + 1 NNRTI 2 Protease inhibitors
1. Zidovudine Efavirenz Lopinavir / Ritonavirs

Or Age > 3 yrs Age <3 yrs
Abacavir
(Hb <9g)
2. Lamivudine
Mother to child Transmission of HIV
Perinatal: →15 – 30%
3%
➢ HAART
➢ C – Section
➢ Avoid breast feeding
Diagnosis:-
HIV PCR – Best
P24 antigen assay
Viral culture Insensitive
Prophylaxis : Drug
➢ Nevirapine
➢ Nevirapine + Zidovudine → high risk, if mother
• Did not take HAART/ <4 wks duration
• Diagnosed first time during pregnancy.
• High Viral load >1000 copies/ml.
Other drugs: -
➢ Cotrimoxagohl
➢ Isoniayid
76
Feeding
➢ Breast feed – indicated in India →Mother – HAART, Baby – prophylaxis
➢ Formula feed – Best
➢ Mixed feeding – Not indicated
CONGENITAL INFECTION
General features of Congenital Infection
1. IUGR
2. Microcephaly – Seizures
3. Hepatosplenomegaly
4. Jaundice
5. Petechiae
TORCH
Toxoplasmosis
Others – Varicella, syphilis
Rubella
CMV – Most common
Herpes
General rule:→
1. Earlier More severe
(1st trimester)
Exception:- Varicella – Starts 2nd trimester
2. More chance of transmission 1st trimester
rd
Exception:- CMV, toxoplasmosis – 3 trimester
Rubella – 1st trimester & term gestation.
CMV
MC non genetic cause of deafness.
C/F: →
➢ Asymptmatic
➢ Chorioretinitis
➢ Deafness
➢ Microcephaly
➢ Intellectual disability
➢ Periventricular calcification
Diagnosis:
PCR → Urine – Best
→ Blood
→ CSF
→ Saliva – Screening
Rx:- Ganciclovir
Toxoplasmosis
C/F:- Chorioretinitis – most common
Diffuse calcification
Hydrocephalus
Diagnosis:- IgM/IgA – ISAGA (Immunosorbent agglutination assay)
Rx:- Sulfadoxime – Pyrimethamine
Prevention of intrauterine transmission → SPIRAMYCIN
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Congenital Rubella syndrome

Most severe
Triad: -
1. Cataract
2. Deafness (Sensorineural)
3. Patent ductus arterorus
MC eye manifestation:→ Salt & pepper retinoapathy (does not affect vision)
Other heart defects:- Pulmonary stenosis
VSD
Extended Rubella syndrome:-
➔ Progressive rubella panencephalitis
➔ Diabetes
➔ Hypothyroidism
Diagnosis:- IgMAC → IgM antigen capture assay.
Varicella
Varicella
Congenital Perinatal - MC
C/F: hypoplasia of limbs - If mother develops infection within 5 days before
Scarring of skin – CICATRIX delivery or 2 days after delivery.
C/F:- Vesicles
Rx: - Acyclovir
Prophylaxis:- Varicella zoster immunoglobulin.
Diagnosis: DFA from Scraping of lesions.
Baby – isolation
Herpes Simplex
Perinatal, HSV – 2
C/F:
• Skin – mouth – eye disease vesicles

• Disseminated
• Encephalitis
Prevention:- C-Section (minimize risk)
78
Syphilis
Manifestations
Early (<2yrs of life) Late (>2 yrs of life)

• Rash – palm, soles Triad (Hutchinson)
• Rhinitis – snuffles (infectious) - Interstitial keratitis
• Osteitis; Periostitis - Sensorineural deafness
- Hutchinson’s teeth
Pseudoparalysis - Mulberry molar
➢ RHAGADES – cracks in angle of mouth
➢ Bowing tibia – SABER shin
➢ Swelling knee – CLUTTON’s joint
FETAL CIRCULATION
At Birth:-
1. ↑ Systemic vascular resistance
2. ↓Pulmonary vascular resistance
3. ↑ Pressure in LV and LA and there is ↓ pressure in RA →Closure of Foramen Ovale
4. Ductus arteriosus closes due to ↓ pulmonary vascular resistance
Congenital Heart Defects

Associations of congenital heart Disease
Genetic Associations:
1. Trisomy 21 (Down syndrome):→ Cushion Defect TOF (mc detect)
2. Trisomy 18 (Edward syndrome):→ VSD, ASD
3. Monosomy: 45 x 0 (turner Syndrome):→ Bicuspid Aortic valve (MC) Coarctation of Aorta
4. Noonan syndrome:→ Pulmonary stenosis, Hypertrophic cardiomyopathy
Microdeletion:-
1. DiGeorge syndrome:- Conotruncal Defect
79
(22 q 11.1)
2. William’s syndrome: -Supravalvular aortic stenosis
(7 q11.23)
Associated with skeletal anomalies
1. Holt-oram syndrome:-
- Autosomal dominant
- ASD, VSD
- Radial ray anomaly (absent radius & thumb)
2. TAR Syndrome:-
- Thrombocytopenia + Absent radius
- Autosomal Recessive
- ASD, TOF
- Absent radius, Normal thumb
Prenatal
1. Infant of diabetic mother:→ MC is VSD
: Due to Excess insulin, child develops Asymmetrical septal
hypertrophy (Transient Condition).
2. Maternal phenylketonuria:→ ↑ risk of Septal defect
3. Fetal Alcohol syndrome: → ↑ risk of Septal defect
4. Fetal hydantion syndrome:→ ↑ risk of TGA, VSD, TOF
5. Congenital Rubella syndrome:→ PDA (MC), PS, VSD
6. Lithium Intake:→ Ebstein’s Anamoly
Acyanotic Congenital Heart Disease

VSD
➢ MC acyanotic congenital heart disease
➢ Type of VSD
→ Inlet VSD
→ Outlet VSD
→ Perimembranous VSD (MC type of VSD)
→ Muscular VSD
➢ Findings in VSD
→ Pansystolic murmur at lower left sterna border
Pathophysiology
1. Pulmonary Congestion:-
- ↑ risk for recurrent respiratory infection
- Congestive cardiac failure
2. Infective endocarditis
3. Eisenmenger syndrome:-
→ Due to Left to right shunt, there is increased pulmonary blood flow which cause vascular
remodeling
Increase in PVR
Inverted Shunt:- Right to left
Cyanosis (Eisenmenger Syndrome)

Indication for surgery in VSD
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A. General indication:- Child develop CCF

(Echo → Qp : Qs > 2 : 1
B. Specific indication:- In case of outlet VSD
(Never Close spontaneously)
ASD
It is associated with long survival period
Types:
1. Sinus venosus defect
2. Coronary sinus defect
3. Ostium secundum defect:→ MC type of ASD
4. Ostium primum defect:→ Occur as a cushion defect
Feature: Split S2 ,Wide and fixed
Complications:- Due to small difference in pressures of left atrium and right atrium, complications occur
very late.
Indication for surgery in ASD
A. General indication: →Child develop CCF
ECHO show Qp , Qs > 2 : 1
B. Specific indication:→ ASD < 3mm – close spontaneously
ASD > 8 mm – Never close spontaneously
PDA
→ Associated with Congenital Rubella syndrome
→ Continous Machinery murmur
Management of PDA depends on
1. Term baby: - Surgery
2. Pre-term baby:- NSAID, Indomethacin, Ibuprofen
PDA + Eisenmenger’s syndrome:→ Cyanosis in lower limb but pink in upper limb (No cyanosis in upper
limb). This is called Differential Cyanosis.
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CYANOTIC CONGENITAL HEART DISEASE

Type
Tetralogy of Fallot
1. Subpulmonary stenosis (↓ PBF – cyanosis)
2. VSD (R → L shunt)
3. Right Ventricular Hypertrophy
4. Overriding of the aorta (Single S2)
Murmur:- Ejection systolic murmur due to subpulmonany stenosis.

→ Pansystolic murmur of VSD is not heard because VSD is large and it is non restrictive VSD.
Cyanotic spell / Tet spell

Exertion Hypercyanotic Spell
Cyanotic/ spell
More severe stenosis Hypoxia acidosis
‘TET’↑↑ cyanosis
SPASM of Infundibulum Stimulate Resp. centre
↑ workload ↑ Venous return Hyper ventilation
Management of Cyanotic spell
1. Oxygen
2. Sodium Bicarbonate
3. Morphine
4. β - Blocker
5. α – agonists
During the process of squatting
➢ Femoral vein is compressed (which leads to ↓Venous Return)

➢ Fermoral Artery compression leads to ↑SVR and minimize he hypoxia
➢ So Sqatting is helpful in a child with cyanotic spell
➢ Squatting eqvalent position is knee chest position
Chest x-ray in TOF
Boot shaped heart

(Coer-en-Sabot)
Treatment of TOF
A. Palliative Procedure
Creation of shunt
1. Blalock – tausing shunt (between subclavian artery and pulmonary artery)
2. Modified Blalock-tausing shunt (Gore tex) → MC used shunting procedure in TOF
3. Waterston shunt: Ascending aorta →pulmonary artery
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4. Pott’s shunt: Descending aorta → Pulmonary artery
B. Definitive Procedure: Surgery (multiple stages)
Tricuspid Atresia
Tricuspid Atresia TOF
1. Cyanosis present at birth or shortly after 1. Cyanosis not present at birth.
birth -It develops after 6 months
2. ECG: →Left Axis deviation 2. ECG:→ Right Axis deviation
3. Chest x-ray:→ Box shaped 3. Chest x-ray:→ Boot shaped heart
Transposition of great Arteries

→ In TGA, there is transposition of aorta and pulmonary artery.
→ Usually TGA is associated with VSD

→ Isolated TGA is very rare. There is intense cyanosis at birth and the baby immediately dies.
❖ In TGA + VSD
➢ Congestive Cardiac failure (CCF)
➢ Cyanosis
➢ Pan-systolic murmur at VSD
➢ Chest x-ray:→ Egg on string appearance
➢ Treatment: → Arterial switch procedure (Jatene’s Procedure)
Total Anomalous Pulmonary Venous Communication

➢ Types: Supracardiac TAPVC
Cardiac TAPVC
Intracardiac TAPVC
83
Features
- Congestive cardiac failure

- Cyanosis
- Associated with ASD- split S
On Chest x-ray:-
- Figure of 8 Appearance (or)

- Snowman Appearance
In infracardiac TAPVC
• Intense cyanosis as the blood does not enter the heart.

• Pulmonary venous congestion
• On chest x-ray: ‘White out’ lung or
Ground glass appearance
ACQUIRED HEART DISEASE

Rheumatic Fever
➢ School going children
➢ Etiology:- Group A B- haemolytic streptococci
→ Can occur as pharyngitis or pyoderma
➢ After a period of 2-3 weeks (throat infection) or 4-6 weeks (skin), rheumatic fever occurs.
➢ Mechanism:- Molecular mimicry Streptococcal Ab’s wrongly rocognise the body tissue as
streptococcal Ag and start attacking the body tissue.
Jones Criteria
A) Major Criteria
1. Joints: Polyarthritis - Large Joints (Knee/ankle)
- Migratory
- Most common
- Occurs early
- Resolves without any sequele
2. Carditis: Pancarditis - Sequele present once the rheumatic fever subsides
3. Nodules: - Subcutaneos Nodule (Elbow)
- Late Manifestation
- Non tender
- It is associated with occurance of carditis.
4. Erythema Marginatum:- Early finding
5. Sydenham’s chorea: - late manifestation
- Involunatary movements involving the extremities
- Semi purposive
- Emotional lability, hypotonia
- Milk maid grap
Darting tongue sign
84
B) Minor criteria
Pyrexia (fever)
Prolonged PR interval
Polyarthralgia
Persistently high ESR/CRP
C) Essential Criteria
Evidence of streptococcal infection - ↑ASO titre
For skin infection: Anti DNAase B Ab is elevated.
➢ To diagnose rheumatic fever, 2 major or

I major + 2 minor or +
3 minor (Recurrence)
With essential criteria
➢ Revised Jones Criteria (2015)

→ Low risk area:- RF <2/lakh, RHD - <1/1000
→ Moderate high risk area:- RF <2/lakh, RHD - <1/1000
• In high risk area:- Polyarthritis, monoarthritis, polyarthralgia can also be taken as major
criteria and in minor criteria – monoarthralgia.
• Subclinical or clinical carditis can be taken as a major criteria in both low risk an high risk
area.
• Fever:→ >38⁰C in high risk and >38.5⁰c in low risk
• ESR:→ > 30 mm/hour in high risk area and >60mm/hour in low risk area.
Infective Endocarditis
• Vegetations develop over the valves. It can cause.
1. Vascular phenomenon:
1. Infarct
2. Vasculitis:- Splinter Haemorrhage
Osler’s node: Finger
Roth spots: Retina
2. Immunological Phenomenon
1. Glomerulonephritis
2. Splenomegaly
3. Janeway lesions
➢ Fever >5days will be present in infective Endocarditis
Duke’s Criteria
Major Criteria
1) Blood Culture:-
Atleast 2 positive culture in HACEK/Coxiella → 1 positive culture
2) ECHO:- vegetarians, abscess, Valvular regurgitation
Minor criteria
1) Predisposing factor (eg. RHD)

2) Fever >5 days
3) Vascular phenomenon
4) Immunological phenomenon
85
5) 1 positive blood culture

➢ To Diagnose infective endocarditis:-
Two major (or)
1 major +3 minor (or)
5 minor
Organisms associated with IE

1. Streptococcus vividans (α-haemolytic) : Affects already damaged valves: SABE
2. Staphylococcus aureus: Affects naive valve: Acute bacterial endocarditis
3. Enterococcus
Special situations:
➢ IV drug abuse: Pseudomonas

➢ Excessive use of antibiotic or – candida
In open heart surgery
Duration of treatment
3rd generation cephalosporin (or) vancomycin.

+
Aminoglycoside
For 4-6 weeks
Guidelines for prophylaxis
1. Damaged heart valves (RHD)

2. Unrepaired CHD (Especially Cyanotic)
3. RepaiRed CHD – Prosthetic material is used residual defect following repair first 6 months after
repair.
4. Past history of IE
5. Cardiac transplant
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DISORDERS OF UPPER AIRWAY

Upper airway → Nasopharynx – Glottis
BILATERAL CHOANAL ATRESIA
Due to failure of resorption of Buccopharyngeal septum
(Normally Resorption at 5th/6th weeks)
Leads to septum in posterior part of nostril
Types:-
B/L Choanal Atresia
Bony (30%) Mixed (Mc) Membranous
Clinical Features:-
Bilateral Choanal Atresia
➢ Cyanosis on feeding; Relieved on crying (Cyclical Cyanosis)
➢ Respiratory distress in neonakes (Neonates – obligate Nasal Breathess)
➢ Associated with:-
➢ Coloboma →Vertebral Defects
➢ Heart defects →Imperforate anus
➢ Choanal alresia →Tracheo - esophageal fistula
➢ Growth retardation →Renal anamolies
➢ GI abnormalities
➢ Ear anamolies
Unilateral Choanal Atresia

Do not present in neonatal Period; present only in later, life as Nasal obstruction.
Diagnosis:
➢ Inability to pass 8 F Catheter via nostril.
➢ Anterior Rhinoscopy (Nasal endoscopy)
➢ CT (Best) – Anatomy defined well (must before operating)
Management:
➢ Temporarily to maintain airway,
• Guedel’s oropharyngeal airway
• Mc Govern’s Nipple
➢ Definitive Surgery
• Transnasal endoscopic resection of septum.
• Transpalatal endoscopic reaction of septum.
87
Disorders of Larynx:
Disorder of Larynx
Congenital Acquired
1. Laryngomalacia (most common) 1. Acute epiglottitis
2. Subglottic Stenosis 2. Laryngeal papilloma
3. Vocal cord paralysis 3. Vocal nodule
4. Vascular ring
5. Subglottic hemangioma 1. Hallmark of laryngeal disorder – Stridor
6. Laryngeal web/cyst (least common) 2. Vocal cord only affected – Hoarseness of
voice
Laryngomalacia
➢ Most common
➢ MC stridor in newborn
➢ Due to hypotonia of laryngeal musculature.
➢ Otherwise normal baby
Supine position immediately after feeding
Inspiratory stridor (only complaint)

➢ Omega shaped epiglottitis
➢ Prone position → stridor disappears.
➢ Bronchoscopy must be done to diagnosis →Other congenital anamolies.
Treatment
➢ Resolves spontaneously (so wait till 18 months of age)
➢ If associated with failure to thrive, progressive respiratory failure – supraglottoplasty must be done.
Subglottic Stenosis
➢ 2nd Most common
➢ Subglottis → Normal Narrowest part of upper airway in children. (Narrowing have – severe stridor)
➢ Extends from udersurface of vocal cord to lower part of cricoids.
Vocal Cord paralysis:
➢ 3rd most common
➢ Associated with Arnold chiari malformation (hydrocephalus + Meningocele)
➢ Iatrogenic in surgery tracheo-esophageal Fistula; Esophageal atresia
Vascular Rings
➢ Stridor + dysphagia
➢ Arch of aorta → Innominate artery
Form a ring and causes compression of 1. Larynx 2. Esophagus
Subglottic Hemangioma:
➢ Associated with Prominent stridor.
➢ Clues – Cutaneous hemangioma
Treatment
1. CO2 laser excision
2. Intralesional steroid
3. Oral propanalol.
88
Acquired – Acute Inflammatory upper Airway Disorders

Acute Epiglottitis
→Most dangerous upper airway obstruction
Associated with:-
1. High grade fever

2. Wide open mouth
3. Drooling of saliva
4. Tripod posture(To overcome obstruction)
5. Toxic child
6. Respiratory distress
7. Stridor (late finding)→ near total obstruction happened
➢ Etiology – S.Pneumonia
X-ray – “thumbprint sign”
Rx:- Antibiotic of choice – 3rd Gen cephalosporin (ceftaxime / ceftriaxone)
Laryngeal Papilloma
➢ Aka →Multiple laryngeal papilloma (MLP);
→Recurrent laryngeal papilloma (RLP)
→Juvenile laryngeal papilloma
➢ Hallmark – Recurrence
➢ Wart like lesion
➢ Due to human papilloma virus (HPV)
• Type 6 & 11
➢ Mother → Active Condylomata
• High chance of papilloma in child

➢ Characterised by hoarseness of voice
Treatment
1. Excision – Microdebrider (Recurrent)

2. Antiviral drugs - 1. Ribavirin
2. Interferon
3. Photodynamic therapy
4. Diet (Indole – 3 – Carbinol / diet)
Cruciferous vegetables life 1. Broccoli, 2. Cauliflower
Vocal Nodule
➢ Aka singer’s nodule
➢ Symmetrical
➢ At the junction of anterior 1/3rd, Middle 1/3rd (most common)
➢ Due to misuse / Abuse of vocal cord
➢ Rx – Voice rest
89
Foreign Body in Airway

Why children are at risk?
➢ 6 months – 5 years/(By 6 months of age, mouthing (a developmental milestone) develops.

➢ Diameter of airway is small.
➢ Force of air during cough in child is not enough.
➢ Lots of mucus/secretions in airway.
Site:
➢ More common in Right bronchus.

• Large diameter
• Almost in line with trachea
• Airflow through R bronchus is greater.
Most common FB:
➢ Nuts (Peanuts)
Clinical Features
1. Initial Response
• Coughing (‘gag reflex)
• Choking
2. Asymptomatic period
• Foreign body is retained
• Deceptive period
• History is essential for diagnosis
3. Complications
• Erosion
• Obstruction
• Hyperinflation of lungs
Diagnosis:-
History
Universal sign of choking
Positive Negative
↓ ↓
Rigid X-ray (only 25% FB are Radio opaque)
Bronchoscopy CT
(Diagnostic & Therapeutic) ↓
Suggestive of FB.
↓
Rigid bronchoscopy
Heimleich maneuver:-
➢ Just below sternum
➢ Backward & upward
C/I:-
Fracture rib
Chest injury
Flail chest
90
Disorders of lower Airway

Clinical sounds of problems in lower airway
Wheezing Grunting
(Continuous musical sound due to vibration ( Expiration against partially closed glottis)
Of air which passes via partially obstructed pathway) Reserved and most specific for pneumonia (To
prevent the complete collapse of pus filled
alveoli in expiration Grunting occurs)
Polyphonic Monophonic
Obstruction at Multiple site obstruction at one site
Eg: Asthma Eg: Foreign body in bronchi/trachea
Why children more Prone for wheeze:
1. Normally Airway resistance
In children <5 years radius is low and so the airway resistance is very high.
2. Compliance (Normally in expiration there is inward pressure which causes intrathoracic airways to
collapse)
→In children, the above effect is magnified.
3. Hyperactive smooth muscle in trachea / bronchi.
Acute Bronchitis
➢ First episode of wheezing (following URI) in a child less than 2 year.
Risk Factors
1. Boys
2. Winter season
3. Not breastfed
(IgA antibodies in breastmilk affords protection to RSV)
4. Overcrowding
Etiology
1. Respiratory syncytial Virus (RSV) – Most common
2. Influenza
3. Adenovirus
4. Corona virus
Emerging Pathogens →1. Human BOCA virus
2. Human Metapneumo Virus
Inflammation of Bronchiole
Airway edema + mucosal plugs
Some air goes in (20-30% O2) – Hypoxia
Air exchange occurs
Expiration – More obstruction
CO2 trapping Hypercarbia
Respiratory acidosis
91
Chest x-ray:-
1. Hyperinflation
2. Peribronchial cuffing
Treatment
1. 100% O2 (Hyperbaric oxygen) through Nasal Prongs (Aim →SPO2 >92%) [Humidified oxygen]
2. CPAP (Continuous positive Airway pressure)
Prevents collapse of airway
3. To relieve wheezing → 1. Salbutamol 2. Epinephrine
4. To relieve edema → 3% saline (Hypertonic)
5. Anti-viral drugs
• RSV → Self limiting
(No need for anti-viral drugs)
- But if there is associated co-morbidities, (Chronic lung disease, cardiac disorder)
Ribavirin (aerosol can be given)

Prophylaxis:→ Palivizumab
Bronchiectasis
➢ Ectasis → dilation
➢ Chronic suppurative disease
➢ End stage of some other lung pathology
Pathogenesis:-
Infection/Inflammation
Bronchus wall damaged
Repaired
Again damaged
Repaired
Repeated cycle of above
Irreversible dilation
Bronchiectasis
Predisposing Factors:-
1. Airway narrowing
a. Foreign body
b. Enlarged lymph node
c. Tumours (Rare)
2. Airway injury
Infections: 1. Measles
2. Pertussis
3. TB
3. Altered host defences:
• Kartagener syndrome (sinusitis, dextrocardia)
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(Problem in dyenin arm of cilia which lead to stasis of secretions which causes infection)
• Cystic fibrosis (Thick mucus → infection)
4. Altered Immune status
(1⁰ > 2⁰ immunodeficiency)
Special Entities
1. Williams – Camphell syndrome
a. Defective bronchial cartilage by birth.
2. Marnier – kuhn syndrome
a. Congenital tracheobronchomalacia.
b. Elasticity of trachea and bronchi is high predisposing to infections.
3. Right middle lobe syndrome
a. Idiopathic
b. Non-obstructive → repeated infection of middle lobe
4. Yellow nail syndrome
a. Bronchiectasis
b. Lymphedema
c. Pleural effusion
Clinical Features:-
➢ Chronic productive cough
➢ Growth retardation
➢ Clubbing
Inability to
clear
secretions
Distortion of
Recurrent
bronchial Bronchiectasis infection
anatomy
Inflammation
Investigation
➢ HRCT (Best modality)
➢ X-ray chest
Treatment
➢ Treatment of primary condition
➢ Postural physiotherapy (for drainage of pus)
➢ Directed antibiotics (Inhalational route)
Aztreonam
Colistin
Tobramycin
➢ Definitive treatment →Lung transplantation
93
Childhood Asthma
➢ Diagnosis of asthma is made only in age group >5 yrs, because in <5 yrs old children hyperactive
airway is normal.
➢ Type –I Hypersensitivity Reaction (IgE mediated)
Triad:
1. Airway Hyperreactivity Partially/completely Reversible
2. Airway inflammation spontaneously or by B2 agonist.
3. Airway obstruction due to bronchospasm
Asthma improves with age (child outgrows asthma)
Etiopathogenesis:-
Diagnosis
➢ > 3 episodes of Reversible wheezing
Triggers
Allergens Other
Animal dander Exercise / Emotion
Dust mites Viral infection / episodic wheezer
Pollen Smoking (Passive)
Fungi Changes in temperature
Drugs (NSAID’, B-blocker)
➢ Episodic wheezer (common cold rival infection – wheezing)
➢ Multitrigger wheezer (multiple trigger+)
Risk Factors
Major Minor
1. Allergic Rhinitis
1. Parentral asthma 2. Eosinophilia(>9%)
2. Eczema/Atopic dermatitis 3. Food allergen sensitization
3. Inhalant allergen sensitization 4. Wheezing apart from cold (may be
excluded)
Asthma Predictive Index (API)
➢ > 3 episodes of Reversible wheezing + 1 major risk factor & 2 minor risk factor.
Classification
1. Transient Wheezer
(From early childhood → Settles by 6 year of age)
Low risk of asthma
2. Persistent wheezer
(>6yrs of age) High risk of asthma
3. Late onset wheezer
(wheeze start from 3 yrs of age and then persists)
Wheezing:
➢ Continuous, Musical sound produced by transmission of vibration produced by obstructed airway.
➢ Polyphonic (multiple level of obstruction)
X-ray →Hyperinflation of lung field.
94
Diagnosis
➢ Mainly clinical; CBC – Eosinophilic (>9%)
Pulmonary Function Test (PFT)
➢ Gold standard for diagnosis
➢ Flow volume curve → obstructive pattern
• ↓ PEFR
• ↓FVC
• ↓ FEV1
• ↓ FEF 25-75 (Most sensitive parameter for pulmonary asthma)
Treatment
Management
Relievers Controllers
(To treat Bornchospasm and relieve acute attacks) (To prevent further attackes)
1. β – agonist 1. Inhaled cortico steroids – DOC
β2 Specific → Salbutamol, Terbutaline - Beclamethasone
Non – Specific →Adrenaline - Budesonide
- Fluticasoe
2. Mast cell stabilizer
- Cromolyn sodium
- Nedocromyl cromoglycate
3. Leukotriene - modifier
- Zileutron
- Monteleukast
4. Immunomodulator
- Omalizumab (anti – IgE)
Delivery Methods:→
Metered dose Inhaler (MDI)
➢ Used in children >12 yrs of age

➢ In between 5-12 yrs (MDI + Spacer needed)
➢ If <9 yrs (face mask + spacer + MDI needed)
Rotohaler
➢ Used in >5 yrs of age

➢ Rotocaps (capsules) are used
➢ Crushed in chamber and Inhaled by forceful inspiration via mouth piece.
Childhood Pneumonia
Inflammation of lung paranalyma
Pneumonia
Infectious Non-infectious
Caused by microbes Pneumonia like picture
95
Pneumonitis
Definitions of Pneumonia
Community Acquired Pneumonia (CAP)
➢ Infection acquired outside hospital (at least 14 days before)
➢ Commonly caused by Viral / Atypical bacteria
Hospital Acquired Pneumonia (HAP)
➢ Infection acquired within 48-72 hours of hospitalization
➢ Commonly caused by Bacteria
Recurrent Pneumonia
➢ 2 or more episodes in a year or > 3 epidsodes in any timeframe
➢ Caused by
• Immunodeficiency (1⁰ & 2⁰)
• GERD
• Congenital heart disease (L→R shunt)
• Cystic fibrosis
Etiology:→
Pneumonia
<3 months Till 5 years >5 years

➢ Group B shreptococci RSV(MC overall) Myloplasma
➢ E.coli Streptoccocus pneumonia Pneumonia
(MC Bacterial especially b/w 3 month – 3 year) (Atypical pneumonia)
ARI Control Program
Age Clinical Signs No Pneumonia Pneumonia Severe Pneumonia

0-2 months Respiratory rate/min <60 -NA >60
Chest indrawing - - NA And/or present
2-12 months Respiratory rate/min <50 >50 -NA

Chest in drawing - - Present
12-60 months Respiratory rate/min <40 > 40 -NA

Chest indrawing - - Present
Management Plan Home care Oral antibiotic Urgent referral to

+ Home care hospital after 1st
dose of antibiotic
Pneumococcal Pneumonia
➢ Lobar consolidation present
➢ Stage of pneumonia thypically followed
o (Congestion – Red hepatization
grey hepatization
➢ Thick rusty sputum

➢ Rx: penicillin
o If penicillase Producing Pneumococci (PPP) → 3rd Gen caphalosporin (Ceftriaxone and
Cefotaxime
96
Staphylococcal Pneumonia
➢ MC age <1 year

➢ Characterised by:
1.Confluent suppurative lesion
2. Pneumatocele
• MC Staphylococcus
• Klebsiella/E.coli.
➢ Pneumothorax present
➢ Pyothorax present (Empyema)
➢ Rapid progression (Most severe)
➢ Associated with malnutrition, Cystic fibrosis, Diabetes
➢ Rx:- cloxacilin
• If MRSA – Vancomycin
Interstitial Pneumonia
➢ aka- bronchopneumonia
➢ Infection speeifically involing alvelolar septa (intesstitium)
➢ Etiology:- <5yrs – RSV
▪ >5 yrs – mycoplasma , chalmydia a/w conjctivitis)
➢ Rx: Macrolides
Cystic Fibrosis
➢ Multisystem disorder
➢ aka Mucoviscidosis
➢ Single gene defect
➢ MC in white population
➢ MC life threatening genetic disorder.
➢ Chromosome (7q)
Cystic fibrosis Transmembrane Regulator (CFTR)
Protein defect
(Transmembrane chloride iron regulator)
Physiology in cystic fibrosis
Thick mucus
Infections (Repeated)
Bronchiectasis
Two Cardinal Features:
1. Gastrointestinal (diarrhea with steatorrhea)
→Due to exocrine Pancreatic insufficiency (leading cystic fibrosis)
Mucocystic / fibrocystic disease of pancreases

2. Recurrent Respiratory tract infection
→Older children (>5 years)
97
Clues to cystic Fibrosis:
GI Manifestations:→
1. Failure to pass meconium (>48 hrs – delayed)
CFTR defect
↓↓ Fluid in lumen of small intestine
Thick meconium
Stays of small intestine itself
Meconium ileus
X-ray (Barium contrast)
1. Microcolon
2. Filling defects Ileocolic junction
3. Air fluid levels in small intestine
→ ↑↑ Air fluid levels in small intestine alos denotes
Meconium peritonitis (also charactrised by scrotal Calification; peritoneal calcification)
→ Meconium plug is also a feature of Cystic fibrosis (less specific)
2. Bulley, greasy shorts
3. Rectal prolapse
Respiratory Manifestations
➢ Recurrent respiratory tract infections
➢ Earliest finding → Bronchiolitis
➢ Most important cause of mortality
➢ MC organism → Staphyloccocus aureus
Also Mucoid colonies of pseudomonas
Burlcholdenia capacia (most of mortality)
➢ Long term → Bronchiectasis (Copious sputum; clubbing)
➢ B/L Nasal Polyposis
Genitourinary System:
1. Males → Almost 100% - infertile (Azoospermia)
Due to B/L absence of vas deferens
2. Females → high chances of infertility
Sweat Gland:
CFTR Defect
Na+& Cl- don’t go into sweet gland
“Salty sweat”
Diagnostic Methods:
1. Pilocarpine electrophoresis
(↑↑ chloride levels in sweat [>60 mEq/L])
2. Identifying CF Mufation (MC – 50%)
3. Transepithelial (Nasal) Membrane potential difference.
98
Treatment
1. Airway clearance therapy (ACT)
a. Postural drainage
b. Salbutamol / hypertonic saline
c. Inhaled mucolytic (Recombinant DNAse)
d. Inhaled Tobramycin
2. Systemic antibiotics
a. Staphylococcus – Cloxacillin
b. MRSA – Vancomycin, linezolid, teicoplanin
c. Pseudomonas – Tobramycin, Meropenam.
3. Nutritional
a. Fat soluble vitamin supplementation
b. Replacement of pancreatic enzymes
4. Emerging Therapies – IVACAFTOR (CFTR Potentiator)
99
Gastrointestinal disorders
Foreign body in GIT
➢ Very common in age group <2 years.
➢ 80-90% pass spontaneously
➢ MC → Coin
➢ Features →Abdominal Pain/Nausea, Recurrent cause of unknown etiology
Management:-
➢ Based on location
Foreign Body
General Specific
Eesophagus – Removed within 24 hours Button battery →causes thermal electric injury
(Endoscopic) Esophagus – Removed
Stomach – Pass spontaneously Stomach – obsarration except in
Large (>5cm) Age (<5years)
Size (>2cm)
Removed
Removed Sharp object
- Single sided (pin)
Don’t causes injury → observe
- Double sided (Toothpick, fish bone)
Erode GIT
Sinus / Fistula – must be removed

Magnets (> 2 → must be removed).
Esophageal disorder
➢ MC – Gastroeosophageal reflux Disorder (GERD)
➢ Gastroesophageal reflux is physiological (Fairly common in all children).
➢ Due to lower eosophageal sphincter incompetence
➢ Injury to esophagus
Erosion Ulceration
• Barret’s esophagus (intestinal Metaplasia)

➢ Aspiration pneumonia
➢ Associated with failure to thrive
➢ Irritation of spinal nerves.
Tonic Posturing
Arching of back (Opisthotonus)
Sandifer’s syndrome (Severe GERD)

Diagnosis:
➢ 24 hours esophageal Ph monitoring

➢ Newer and best method (Multichannel intraluminal impedance monitoring)
100
➢ Esophagus erosions/Barret’s esophagus can be seen b y endoscopy
Management:
GERD
Fails
Non Pharmacological Pharmacological
1. Thickening of feeds PPI >>>H2 Receptor antagonist
2. Frequent, small feeds (↓Secretion of Hcl)
3. Supine Position is avoided
Fails
→Prone positioning is best
Except during sleep “Intractable GERD”
Surgery
(Sudden infant death syndrome) (Nissen fundoplication)
Is common
→During sleep → Reinforce LES Region.
Supine with head and elevation
Esophageal Atresia
➢ MC anomaly of esophagus
➢ MC associated with Tracheo – Oesophageal fistula (TEF)
MC →Type C
➢ Almost half associated with syndrome
➢ MC associated syndrome – VACTERL
• V – Vertebral
• A – Anal
• C – Cardiac
• T – TEF
• E – Esophageal atresia
• R – Renal
• L – Limb Defects
➢ Esophageal atresia →Drooling of frothy saliva – Aspiration
Respiratory Distress
➢ Vomiting is also common
Diagnosis:-
➔ Coiled tube (x-ray) of NG tube in esophagus.
Congenital Hypertrophic Pyloric Stenosis
➢ At birth – only 50% obstruction →Normal at birth
➢ At 2-3 weeks after birth →100% obstruction
➢ Presents with: 1. Projectile non – bilious vomiting
2. Swelling (olive shaped) in epigastric region.
3.After food → Visible gastric peristalsis (VGP)
➢ Hypochloremic, Hypokalemic, Metabolic alkalosis
101
Diagnosis
1. USG abdomen – Pylorus 1. Thickness > 3mm

2. Diameter > 14 mm
3. Length >15 mm
2. Barium Meal Findings:
➢ Only suggestive, not diagnostic
3. Fluoroscopy – Visible peristalsis – “Caterpillar sign”
Treatment
➢ Ramstedt’s pyloromyotomy
Duodenal Atresia
➢ MC intestinal obstruction is neonate
➢ Due to failure of Recanalization
➢ MC obstructed part of duodenum → 3rd part of duodenum &
2nd part after opening of bile duct.
➢ Bilious vomiting
➢ NO abdominal distention
➢ Commonly associated with trisomy 21
X-ray Abdomen
➢ Double bubble sign
Treatment
➢ Duodenoduodenostomy
Hirschsprung disease
➢ MC cause lower intestinal obstruction in neonate.
➢ Normally meconium is passed within 24 hours, in Hirschsprung disease these is delay in passage of
meconium (>48 hours)
➢ aka Aganglionosis
➢ Affected part of colon → Constricted
➢ Disorder of Neuronal migration
➢ Migration of Neurons needs 2 genes.
• RET gene
• EDNRB gene
➢ Problem in above genes leads to Hirschsprung disease.
Investigations
I →Rectal Biopsy: (“Suction Biopsy is needed – Submucosal tissue Seen)
➢ Absence of ganglion is Submucosal plexus and myenteric plexus.
➢ Marker – Acetylcholinesterase staining (in suction biopsy)
➢ New Marker – Calretinin (in suction biopsy)
II – Rectal Manometry:-
→ Normally, when rectum is distended, pressure in internal sphincter decreases.

→ In Hirschprung disease, when rectum is distended, paradoxical increase in pressure in Internal sphincter.
Meckel’s Diverticulum
➢ MC congenital anamoly
➢ Remnant of Omphalomesentric duct.
➢ From antemesentric border of ileum.
102
Rule of ‘2’
➢ 2% population (2/100) (Most – asymptomatic)
➢ 2 inches in length
➢ 2 feet from ileocaecal junction
➢ 2 types of ectopic mucosa (Gastric / pancreatic) → Erosion – Bleeding (MC symptomatic
presentation)
➢ Age at Presentation → 2 years.
Diagnosis:
➢ Radionuclide scan (Meckel scan) – 99 m pertechnetate scan
Malabsorption
Etiopathogenesis
1. Damaged Mucosa 1. Post surgery

2. Chronic infection (giardia)
3. Inflammation (celiac disease)
2. ↓ Enzymes ➢ Disaccharidoses
3. ↓Motility ➢ Intentinal obstruction
(Bact. Overgrowth – Deconjugation of bile ➢ Congenital intestinal lymphangiectasia
→ fat malabsorption.)
4. Impaired lymphatic drianage
5. ↓ Pancreatic Secretion 1. Cystic Fibrosis
2. Diamond shwachmann syndrome/also BM
suppression & skeletal abnormalities
Clinical Features
➢ Non Specific – 1. Failure to thrive
2. Chronic abdominal pain
3. Chronic vomiting
➢ Chronic diarrhea (>2weeks) → Non infection
➢ Greasy / foul smelling / Bulky Stool → Fat malabsorption
Investigations:
Malabsorption
Carbohydrate Fat Protein
1. Sugar malabsorption 1. Microscopy 1. Hypoalbuminemia

→ >6-8 fat/LPF (Non – specific)
Colon 2. Gold standard 2. Stool - alpha1 antitripsin
(24 hour feed fat estimation) (Mol. wt like albumin)
(>20% at excreted)
Stools sugar + Acid + H2 Diffuse into blood
Benedict test (+ve) Stool PH (<5.5) frothy stools Excreted by lungs
H2 Breath test
103
Celiac Disease
➢ aka Gluten sensitive enteropathy
➢ Culprit – “Gliadin” (part of gluten)
➢ Common in North India (Wheat is the stable food)
➢ Associated with “BROW” (Barley, Rye, Oat, Wheat)
Genetic Predisposition:
→ HLA DQ2
→ HLA DQ 8
Risk Factors:
↓IgA, Autoimmune thyroiditis
down syndrome, Type I diabetes mellitus, Turner syndrome
Pathogenesis
Gluten in food
Gliadin – Resist Degradation
Enters Duodenum
Gliadin Combines with IgA (for unknown reason)
Comes out of intestinal mucosa
Acted upon by TTG (Tissue transglutaminase)
Removes amine (Deaminated Gliadin)
Inflammation (Tcell mediated)
Stimulate (B-cells) Mucosa affected
Antibodies Atrophy
1. Anti-Gliadin
2. Anti-TTG
3. Anti-Endomycial
Triad of celiac Disease
1. Failure to thrive
2. Chronic diarrhea
3. Iron deficiency anemia (unresponsive to oral iron therapy)
Extraintestinal Manifestation:
→ Dermatitis Herptiformis
104
Diagnosis :-
Celiac Disease
Invasive Non – Invasive
Biopsy Antibodies
Villous atrophy Intial – anti TTG
Crypt hyperplasia Best – Anti EMA
Intraepithelial lymphocytes
Treatment
➢ Complete elimination of gluten from diet.
Complications
1. Small bowel malignancy
2. Non-hodgkin hymphoma (NHL) [causes of death in gluten disease]
Cow’s milk protein allergy (CMPA)
➢ MC type of food allergy
Types
IgE Mediated Non- IgE Mediated (T-cell)
Less common More common

Within minutes Slow onset
Systematic Only GI symptoms
Symptoms +
Activation of Mast cells
Histamine release
Bronchoconstriction Urticaria (liver) ↑Permeability of blood vessels.
Edema (Swollen Lips)
↓Blood volume
Shock
➢ MC manifestation →Protein induced Colitis
➢ MC presentation → Blood in stools.
Diagnosis:
➢ History of milk added recently (1 OR 2 days)
➢ Confirmed by “Elimination and challange test” (Gold standard)
o Avoid COW’S milk → Symptoms subside →Reintroduce milk→ within 48 hours symptoms
PRESENT.
➢ Sigmodioscopy – Aphthous ulcer.
105
➢ Rectal biopsy – Eosinophilis ++

Treatment
• Avoid cow’s milk
Acute Pancreatitis
Etiology:
➢ Abdominal trauma (most common)
➢ Drugs (Valporate, Asparaginase)
➢ Infection (Mumps)
Features:
➢ Pain (may be Radiate)
➢ Acute severe pancreatitis (Cullen’s sign, Grey turner sign)
Diagnosis
➢ Lipase >>> Amylase
Management
➢ Symptomatic / fluids
➢ Early enteral feeds
➢ No need for prophylactic antibiotics
➢ Antibiotics only when there is pancreatic necrosis.
106
Wilson’s disease
➢ Inherited Metabolic disorder.
➢ Chromosome 13 (Wilson disease → 6+7 = 13)
➢ Defect in ATP 7B gene
Food Containing Cu2+
Enters Hepatocytes
Wilson disease → ATP7B
Ceruloplasmin Vesicles with Cu2+ is pushed into bile
Bound Cu2+ Excreted

(Harmless)
Non functioning ATP 7B gene
Ceruloplasmin ↓excretion into bile
↑ Free CU2+ in plasma
(MC Children) – Liver Eyes Basal ganglia (mc in adult)

Chronic liver - KF ring
Disease - Sun flower A – Ataxia B – Bradykinesia
cataract
C – Chorea D – Dyrathria
Depressive pscychosis
Diagnosis:
➢ Screaning → 1. ↓Ceruloplasmin in blood
2. ↑Excretion of Cu2+ (urine)
➢ Gold standard → liver biopsy
CU2+ Stains - 1. Orcein
2. Rubeanic acid
3. Rhodamine
➢ MRI Brain (T2W – “Face of giant panda” sign)
107
Treatment:-
1. Chelating agent of choice (D-penicillamine)
2. Failed medical management → Liver transplantation
SEIZURES & EPILEPSY

Seizures
Genaralized (Rx: Valproate) Focal (Rx: OX/carbahzepine)
GTCS (MC) Grand mal Simple Focal Focal dyscognitaive

Absence Seizures – Petitmal
Myoclonic Consciousness + Consciousness – X
Conditions Causing Seizures

Common Conditions:- Febrile Seizures
Absence seizures
Myoclonic Seizures
Epileptic Syndromes: West syndrome
Lennoux gastaut syndrome
Rolandic epilepsy
Febrile seizures
➔ 6 months – 5 yrs
➔ Neurologically – N
➔ Characteristics
➔ GTCS
➔ 1 seizure/fever episode
➔ Duration <15 mins
➔ Within 24 hrs of fever
Management
➔ ↓Temperature
➔ Terminate with Benzodiazepine
➔ Prophylaxis
Indications for prophylaxis
➔ Recurrent (> 3ep x 6 months or > 4 ep x 1yr)
➔ Risk factors for recurrence Age at onset (<18 months)
Family history
Multiple episodes
Seizures @ low temp - <40⁰C
➔ Drug = Oral – Diazepam/clobazam
Absence Seizures
➔ School going children
➔ 50-60sess
➔ C/F Recurrent string episodes
Inattention to school activities
➔ No post lctal confusion
108
➔ EEg: 3 spike/sec & wave pattern

➔ Rx. 1st choice = Valproate
2nd Choice = Lamtorigine
Juvenile Myoclonic epilepsy
➔ JANZ syndrome
➔ Adolescent
➔ On awakening from sleep
➔ EEG:- 4-6 /sec spike & wave pattern
➔ Rx:- Valproate
West syndrome:
➔ Starts in infancy
➔ SALAAN Spells (Neck flexion)
➔ Intellectual disability
➔ EEG:- Hyparrythmia High voltage spikes in chaotic back ground
➔ Rx: ACTH – 1st choice
➔ Vigabatrin – 2nd choice (1st choice in tuberous sclerosis)
➔ West syndrome cases Cryptogenic (idiopathic)

Symptomatic (2 to other problems)
Lennoux Gastaut syndrome

Rolanoic Epilepsy
➔ 3-10 yrs of age
➔ Benign (Resolves by adolescence)
➔ Centro temporal spikes
Status Epilepticus
➔ One seizure > 30mins
➔ Multiple seizures – 30 mins (LOC in b/w seizures)
➔ Rx: Benzodiazepine
Phenytoin
Valproate
Lamotrigine
Pheno barbitone
Refractory SE
➔ Sz not responding to BZD + 1 more anticonvulsant
Rx: Continuous infusion of Midazolam

No response
Super Refractory SE
Cerebral Palsy
➔ Insult to developing brain – Encephalopathy – Problem in tone, posture,mvt. (Evole over time)
➔ MC insult; Birth asphyxia
109
➔ MC ass. Problem, visual (strabismus)

Acc. To Tone
Hypotonic CP Spastic CP (mc) Extrapyramidal CP
Rare Early pointers a/w Kernicerus
a/w severe MR Cortical thumb >4 months

Persistant neonatal reflexes
Spastic CP
Quadriplegia Diplegia Hemiplegia

➔ Term baby → Preterm Baby → Term /preterm
➔ All 4 limbs → LL>UL → Vaseular (infaction)/
➔ Most severe → commando crawl anomaly (neuronal
➔ ↑Seizure, ↑MR → scissoring position migration disorder).
➔ Pseudo bulbar palsy → Fevourable prognosis
Aspiration (Frequent) → IQ – N
Causes of mortality:
ANOMALIES, HYDROCEPHALUS, RAISED ICP

Neural Tube Defects
➔ 2 types:- Caudal (mc) & cranial
Caudal neural tube defects
1. Spina bifida occulta – Asymptomatic
➔ Spinal cord & meanings – N
➔ Part of vertebral bone – missing
➔ Skin – intact
➔ Tuft of hair / lipoma - +/-
2. Meningo coele
➔ Spinal cord = N
➔ Meninges out pouching
3. Meningo myelocele
➔ Spinal nerve roots & meninges – outpouching in a cavity
4. Meningo myeloschisis
➔ Splitting of meninges
➔ Meninges & nerve roots are exposed outside.
➔ Most severe
Cranial neural tube defects
1. Anencephaly – skull bone & brain –
➔ Early detection – 10-12 wks.
2. Encephalocoele
➔ Out pouching – content = Brain matter
➔ N.C. occipit.
Risk factors for NTD
110
➔ Multifactorial
➔ Folic acid def.
➔ MTHFR gene mutation
➔ Infant of diabetic mother.
➔ Anticonvulsants (Valproate)
➔ Trisomy 18,13
➔ Neural tube devp. → 3-4 wks gestation
Prevention of NTD
➔ Peiconceptional folic acid
➔ Dose Usual:- 400 mcg
Previous affected child: - 4000 mcg.
Ante natal detection
➔ USG:- 14-16 wks POG
➔ Markers :- Achesterase, AFP
Hydrocephalus
➔ CSF – N = soml (infants); 150 ml (older child & adults)
➔ Pathway of CSF:-
➔ Choroid plexus (LU): 3rd Ventricle 4th ventricle
Sub arachnoid spaces Centrial canal

Etiology
Obstructive/non communicating (MC)
➔ MC – aqueductal stenosis
➔ Dardy walker malformation
➔ Arnold chiari malformation
➔ Vein of golden malformation
Non obstructive / communicating
➔ ↑Production – choroid plexus papilloma
➔ ↓ absorption – Meningitis, SAH
Dandy walker malformation
➔ Cystic expansion of 4th ventricle – Hydrocephalus
➔ Prominent occiput
➔ Trans illumination +
➔ Cerebellar hypoplasia
Arnold chiari malformation
➔ Type II – presents in children
➔ Elongation of 4th ventricle – obsturciotn of CSF flow
➔ Vermins of cerebellum – herniation
➔ Small occiput
➔ Association = Meningo myelocoele.
Vein of Galen malformation
➔ Vein of Galen (high pressure) – obstructs CSF flow – hydrocephalus.
➔ Heart – Features of CCF
➔ B – Embolization
Features of hydrocephalus
111
➔ ↑HC
➔ Signs of ↑ICP – vomiting, headache, papilledema (older child)
➔ Bulging AF
➔ Prominent Scalp veins
➔ Separation of sutures
➔ Sunsent sign
➔ Macewan’s sign
Rx:-
➔ Treat the cause
➔ Ventricuolo peritoneal (VP) shunt / ventriculo atrial shunt
➔ Acetazolamide
Raised ICP
➔ N ICP= <15 mmHg (infant)
<15 mmHg (older)
Causes
➔ Mx of ↑ICP
➔ A, B, C, C-spine (Stabilization)
➔ Head end elevation - 30⁰
➔ Hyperventilation (PaCO2 – 35-40 mmHg)
➔ 20% Mannitol
➔ 3% saline
➔ Sedation (Barbiturates, Morphine)
No Response
1. Decompressive craniectomy
2. Induced hypothermia
3. Induced coma
4. Therapeutic CSF drainage
112
PEDIATRIC IMAGES DO PRACTICE
113
114
Image based questions
115
QUE 1 2 3 4 5 6 7 8 9 10
ANS C B A A A A C C B A
QUE 11 12 13 14 15 16 17 18 19 20
ANS C B B B D A B B B C
116

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1

LFD (large for date) or

Important cause for LFD Neonate

Pre term Neonate

→ Used for 20-44 wks gestation

➢ Appears emaciated 2. Anterior fontanelle – large wide OPEN

Primitive Neonatal Reflexes

Present at birth (Term) Appears Disappears

✓ ATNR → Asymmetric Tonic Neck Reflex

Moro’s Reflex/Embrace Equivalent

➢ Disappear at 5 – 6 months (nelson)

[3 – 6 months (op ghai)]

➢ Abnormal persistence beyond 6 months → Cerebral damage

➢ MORO’s reflex, if once disappears never reappears.

➢ Cause of Asymmetric MORO’s reflex

Important condition in neonates not requiring any specific Rx

2. Erythema toxicum Neonatorum

4. Strok Bite/Salmon Patch

6. Acne Neonatorum – d/t maternal androgens

7. Sub conjunctival hemorrhages

10. Hymenal Tags – Skin growth near the vaginal opening.

11. Physiological Phimosis

12. Physiological weight loss

CEPHAL HEMATOMA CAPUT SUCCEDANEUM

Term Gestation ? Routine Care

Heart Rate <100/min or Yes Labored breathing or

HR < 100/min 30 sec

No Heart Rate <60/min

Inj. Adrenaline (up to 3 time)

➢ Hypovolemia – poor peripheral perfusion, feeble pulse

- O Group Rh negative blood

➢ PPV - Positive pressure ventilation (Bag & mask ventilation).

➢ Post Resuscitation Care

Recommended Target Saturation of O2

AGE TARGET SATURATION

- Device used → Self inflating bag & mask (‘AMBU’ BAG)

- Function of Reservoir → ↑O2 delivered to baby

O2 supply Reservoir Fio2 delivered

➢ Pressure required to deliver breath to neonate

Subsequent breaths 15 – 20 cm H2O.

➢ Absolute C/I to bag & mask ventilation → Congenital diaphragmatic hernia

Weight Gestational Age Size of ET (mm)

→ CXR AP view is not useful for confirming ET tube is in trachea.

➢ Site - In the midline, on the lower 1/3rd of body of sterum (or).

→Lesser rescuer fatigability

Baby born through MSL

Vigorous neonate Non – vigorous neonate

- HR > 100/min - Do not stimulate

Delayed cord clamping

➢ GI - Abdominal distention, feed intolerance

Inj. Ampicillin + Gentamicin

Moderate hypothermia 32 – 35.9⁰C

Baby exposed to cold environment

Neither release of nor epinephrine

Uncoupling of β oxidation of fat

2. Cutaneous vasoconstriction when exposed to cold temperature.

➢ Thermal care in delivery room (25⁰C)

Most important mechanism by which baby gets heated