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The ultrafiltrate, which is cell free, contains all of the substances in plasma
(electrolytes, glucose, phosphate, urea, creatinine, peptides, low molecular
weight proteins) except proteins having a molecular weight of ≥68 kd
(such as albumin and globulins).
The filtrate is collected in Bowman's space and enters the tubules, where
its composition is modified by tightly regulated secretion and absorption of
solute and fluid, until it leaves the kidney as urine.
Effect of APGSN on glomerular filtration:
APSGN is an antibody-antigen disease that occurs as a result of certain
strains of the Group A ß-hemolytic streptococcal infection (type 12 and 49)
and is most commonly seen in children of the 5-12 years age-group.
The exact mechanism of the pathophysiology for APSGN is not certain. It is
believed that immune complexes develop and become trapped in the
glomerular capillary loop at the basement membrane.
This produces swelling and occlusion of the capillary lumen and results in
alterations in the glomerular filtration rate.
Morphological alteration in PSGN as seen in different techniques:
Method Description
Light microscopy a. Proliferation of all 3 layers of renal microstructure
(epithelium, mesangium and endothelium)
b. Hypercellularity (owing to infiltration of glomeruli
by PMN cells and proliferation of cells).
Immunofluorescence Granular immune complex deposits (mainly IgG and C3),
in the mesangium and along the GBM.
Electron microscopy Subepithelial electron dense deposits, with appearance
of “humps”.
Note that: Swelling of these humps usually disappear
after 6 weeks of onset.
Hypercellularity and
Compression of Reduction in GFR
proliferation of all
afferent and efferent and resultant
the 3 layers of renal
renal vessels oliguria
microstructure
Activation of Renin-
Retention of salt and Edema associated
Angiotensin-
water with hypertension
Aldosterone system
Hypothermia Since, they are chronically undernourished in utero, they also lack
adequate brown fat stores. This predisposes them to hypothermia.
Hypoglycemia Insufficient energy store predisposes SFD babies to hypoglycemia.
Polycythemia Placental insufficiency + Intrauterine hypoxia
↓ ↓
Feed intolerance Stimulation of erythropoiesis→ Polycythemia
↓ ↓
Poor weight gain Jitteriness, feeding intolerance, hypoglycemia, hypocalcemia,
hyperbilirubinemia, respiratory distress, cardiac failure.
History:
Points important in history Points to which etiology of CH?
Family history of hypothyroidism Dyshormonogenesis
Recurrent transient hypothyroidism Transplacental TRBAb
Residence in iodine deficient area Iodine deficiency
Maternal intake of anti-thyroid drugs/ CH due to fetal iodine exposure
anti-arrhythmic drugs with ↑I2 content*
*The most common example is Amiodarone.
Congenital hypothyroidism
Thyroid scan
Dyshormonogenesis/
USG
Iodine deficiency
Ectopic thyroid
No thyroid gland Normal thyroid gland
+Ve -Ve
[TRBAb: Thyrotropin receptor blocking antibody, TR: Thyrotropin receptor, TPO: Thyroid peroxidase.]
Treatment:
Levothyroxine (T4) given orally is the treatment of choice. As 80% of
circulating T3 is formed by mono-deiodination of T4, serum levels of T4 and
T3 in treated infants return to normal.
Starting dose of T4 is 10-15 μg/kg/day.
T4 and TSH level is expected to come to normal within 1 week and 1 month,
respectively with this treatment.
Lifelong thyroid replacement is required in most cases. But it should be
stopped for 1 month at the age of 3 years in suspected transient CH.
In case of rare central hypothyroidism (thyroid hormone deficiency due to a
disorder of the hypothalamic-pituitary axis), cortisol replacement should be
done first, followed by thyroid replaceme.
3.c. Clinical features of pyogenic meningitis in children:
Symptoms
Classical:
a. Fever,
b. Headache,
c. Photophobia.
Others:
a. Irritability,
b. Lethargy,
c. Mental confusion,
d. Nausea and vomiting,
e. Altered consciousness.
Signs
Classical:
a. Neck rigidity,
b. Kernig sign,
c. Brudzinski sign.
Kernig sign
This maneuver is usually performed with the patient supine with hips and
knees in flexion.
Extension of the knees is attempted.
Inability to extend the patient’s knees beyond 135⁰ without causing pain
constitutes a positive test for Kernig’s sign.
Brudzinski sign
With the patient supine, the physician places one hand behind the patient’s
head and places the other hand on the patient’s chest.
The physician then raises the patient’s head (with the hand behind the
head) while the hand on the chest restrains the patient and prevents the
patient from rising.
Flexion of the patient’s hips and knees constitutes a positive sign.
Brudzinski’s neck sign has more sensitivity than Kernig’s sign.
Others:
Skin
a. Erythematous maculopapular rash -> purpura and petechiae (Neisseria)
b. Tache Cerebrale: Stroke skin with a blunt instrument -> 30-60 sec -> raised red
rash.
Central Nervous System
a. Seizures: Focal/ generalized; tonic/ clonic/ tonic-clonic.
b. Respiratory distress,
c. SIADH (Syndrome of inappropriate ADH secretion),
d. Focal neurologic signs:
Cranial nerve palsies (most common: CN6).
Hemiparesis/ quadriparesis,
Visual signs: Papilledema/ visual field defects (due to cortical venous or
arterial thrombosis)
Auditory signs: Ataxia & hearing loss (due to labyrinthitis)
Altered state of consciousness: Obtunded/ semicomatose/ comatose.
Systemic Conditions
Signs of cellulitis, septic arthritis, otitis media, pneumonia etc. which may act as
the source of infection.
Surgical management
Surgical treatment in VSD is indicated if there is associated PS, PAH or AR.
Operative treatment consists of closure of VSD with the use of a patch.
Group D
4. A 4 year old boy was presented at the emergency room with acute onset of
cough and respiratory distress. He has no fever. His father also suffers from
recurrent episodes of similar problem. The child was admitted 3 times with similar
complications in the preceding 1 year.
a. What is the most probable diagnosis?
b. How will you manage the condition? (1+7)
Group B
2.a. Causes of failure of breastfeeding:
There are 10 steps of successful breastfeeding, failure to adopt any of which may cause
failure of breastfeeding:
6. Give newborn infants no food or drink other than breast milk, unless medically
indicated.
7. Practice rooming-in - that is, allow mothers and infants to remain together - 24
hours a day.
8. Encourage breastfeeding on demand.
9. Give no artificial teats or pacifiers (also called dummies or soothers) to
breastfeeding infants.
10.Foster the establishment of breastfeeding support groups and refer mothers to
them on discharge from the hospital or clinic.
Umbilical cord clamping must be delayed for 1-2 minutes to allow transfer of
additional amount of blood from placenta to the infant
The umbilical cord should be clamped at 2-3 cm away from the abdomen using a
commercially available clamp
The stump should be away from genitals to avoid contamination
The cord should be inspected every 15-30 min during initial few hours after birth
for early detection of any oozing.
Cleaning of the baby:
The baby should be dried and cleaned at birth with a clean and sterile cloth
The cleaning should be gentle as only the blood and meconium to be wipe out
Wiping must not be vigorous (as it will wash vernix caseosa, the greasy material
on the skin of infant that protect the skin and maintain temperature).
Recording of weight:
A sterile pre-heated sheet should be placed on weighing machine with 10 gm
sensitivity.
Head to toe examination:
Examine midline structures (cleft lip, neck masses, chest abnormality,
omphalocele, meningocele, cloacal abnormality)
Identify and document the patency of anal opening
Examine the baby for any birth injury
Note the axial temperature of the baby.
Initiation of breastfeeding:
Start breastfeeding at the earliest point, must within 1 hour
Assist the mother to put the baby on the breast, irrespective of the mode of
delivery.
Administration of Vitamin K:
<1 kg baby: 0.5 mg
>1 kg baby: 1 mg
Preferable solution to administer: Vitamin K1.
Group C
3.a. Rheumatic chorea:
Introduction:
It is one of the major criteria of modified Jones’ criteria to diagnose acute rheumatic
fever (ARF).
Also known as:
1. Sydenham’s chorea
2. Chorea minor
3. St. Vitus Dance.
Description:
It is found in 15% of the patients with ARF
It occurs more often in prepubertal girls (8-12 years)
The changes seen are (serially):
1. Emotional ability and personality changes
2. Loss of motor coordination (deterioration of handwriting)
3. Characteristic spontaneous purposeless movements (usually affecting all 4
limbs)
4. Development of motor weakness (hypotonia).
Prognosis:
V. Special tests:
X-PERT TB/ RIF assay:
Detects M.tuberculosis genome + Rifampicin resistance (which is a very
reliable indicator for MDR-TB).
Adenosine deaminase (ADA):
ADA levels may be high in tubercular ascites. However, it is a
nonspecific marker and results should be interpreted very cautiously.
10. Endoscopy:
If ascites is suspected to be due to cirrhosis/ portal hypertension, then look for GI
varices (endoscopy).
11. Series of other tests may be required to diagnose the underlying cause.
3.c. Tuberculin test:
Introduction:
A tuberculin skin test is done to see if someone has ever been exposed to tuberculosis
(TB). It is also popularly known as Mantoux test.
Preparation:
Tuberculin is a glycerol extract of the tubercle bacillus.
Whereas, Purified protein derivative (PPD) tuberculin is a precipitate of species-
nonspecific molecules obtained from filtrates of sterilized, concentrated cultures.
Interpretation of results:
Pathology:
Skin smear shows: Vasculitis of dermal capillaries and postcapillary venules
Inflammatory exudate contains: Neutrophils and monocytes
Renal histology: Endocapillary proliferative glomerulonephritis.
Diagnosis:
2 of the following criteria must be present:
1. Palpable purpura
2. Age at onset ≤20 yr
3. Bowel angina (postprandial abdominal pain, bloody diarrhea)
4. Biopsy demonstrating intramural granulocytes in small arterioles +/ venules.
Treatment:
• Mostly supportive: Adequate hydration, nutrition and analgesia
• Empiric use of prednisone (1 mg/kg/day for 1-2 week, followed by taper):
It reduces abdominal and joint pain but does not alter overall prognosis/ prevent
renal disease.
Group D
4. A 4 year old child has been brought to the emergency with convulsions persisting
for more than 30 minutes. How will you diagnose the case? Briefly narrate the
management of the case.
Diagnosis of the case:
It is a case of status epilepticus (SE) because SE is defined as prolonged single seizure/
multiple episodes of seizures lasting >30 minutes without regaining consciousness in
between the episodes.
It should be emphasized that no investigation is necessary to start immediate
management of SE and only when SE in controlled, investigations are to be done to rule
out any provocable cause of seizure.
History:
Description of the event
Associated symptoms
Duration of the post-ictal period (characterized by a state of disorientation)
Prior history of seizures
Detailed history of anti-epileptic drug use
History of neurological development.
Investigations:
Routine investigations:
1. Blood: Hb, TC, DC ESR
2. Renal function: Na+ K+ Urea Creatinine
3. Liver function test.
Special investigations;
1. Serum glucose
2. Serum calcium
3. Malarial parasite
4. Culture (if fever present)
5. Lumber puncture (in case of suspected CNS infections).
Other tests:
1. CE-CT brain (in case of a suspected structural lesion in brain)
2. EEG (in case of suspected non-convulsive SE).
Management of the case:
A. Airway:
Must be secured. If required apply oropharyngeal suction and intubation
Prevention of aspiration.
B. Breathing:
Free flow oxygen
Ventilation, if required.
C. Circulation:
Maintain circulatory volume by IV fluid
Control of body temperature.
D. Drugs:
To reduce brain edema: IV dextrose, mannitol
Definitive drugs:
WBUHS Paediatrics (including Neonatology) 2010 Paper
Group A
1. Outline the metabolism of iron in the body. Compare the laboratory findings of
iron deficiency anemia and thalassemia. (4+6)
Iron metabolism:
Absorption:
Most of the iron in the diet is in the ferric (Fe3+) form, whereas it is the ferrous
(Fe2+) form that is absorbed.
Some amount of Fe3+ to Fe2+ conversion occurs within the stomach. Gastric
secretions dissolve the iron and permit it to form soluble complexes with vitamin
C and other substances that aid its reduction to the Fe2+ form.
Rest of the conversion occurs within the duodenum. Fe3+ reductase activity is
associated with the iron transporter in the brush borders of enterocytes.
Almost all iron absorption occurs in the duodenum. Transport of Fe2+ into the
enterocytes occurs via divalent metal transporter 1 (DMT1).
Within enterocytes:
Some Fe2+ is again converted to Fe3+ and stored within the enterocytes as
ferritin-Fe3+ complex.
The rest binds to the basolateral Fe2+ transporter ferroportin (FP) and is
transported to blood. This transport is aided by a protein named hephaestin (Hp).
Within blood:
In the plasma, Fe2+ is converted to Fe3+ and bound to the iron transport protein
transferrin.
Note:
Heme binds to an apical transport protein in enterocytes and is carried into the
cytoplasm. In the cytoplasm, HO2, a subtype of heme oxygenase, removes Fe2+ from
the porphyrin ring of heme and adds it to the intracellular Fe2+ pool.
Storage of iron:
70% of the iron in the body is in hemoglobin, 3% in myoglobin, and the rest in ferritin,
which is present not only in enterocytes, but also in many other cells.
Regulation of iron absorption:
Iron absorption is regulated by hepcidin, a small circulating peptide that is
synthesized and released from the liver in response to increases in intrahepatic
iron levels.
Hepcidin inhibits iron transfer from the enterocyte to plasma by binding to
ferroportin and causing it to be endocytosed and degraded.
Thus, when the body is replete with iron, high hepcidin levels inhibit its
absorption into the blood. Conversely, with low body stores of iron, hepcidin
synthesis falls and this in turn facilitates iron absorption.
Comparison between laboratory findings of iron deficiency anemia & thalassemia:
Parameters Iron deficiency anemia Thalassemia
Hb level ↓ ↓
RBC level ↓ Normal/ ↑
RBC indices
Mean cell volume (MCV) ↓ ↓↓
Mean cell hemoglobin ↓ ↓↓
(MCH)
Red cell distribution width ↑↑ Normal
(RDW)*
Iron status
Serum iron ↓ ↑ (∝ iron overload)
Serum ferritin ↓ ↑ (∝ iron overload)
Transferrin saturation ↓ ↑
Total iron binding capacity ↑ ↓
(TIBC)
*RDW is the coefficient of variation of red cell volume distribution. RDW is the objective
documentation of subjective anisocytosis.
A simplified way to differentiate between these 2 entities:
Microcytic anemia
RDW
Normal Elevated
Favors iron
Favors thalassemia
deficiency anemia
Group B
2.a. Breast milk versus cow’s milk:
2.b. Developmental milestones of 1 year old child
Please see 2012 Paper Group C Question no. 3.d.
2.c. Urinary findings of acute post-streptococcal glomerulonephritis
Please see 2011 Paper Group C Question no. 3.a.
Group C
3.a. Transient tachypnea of newborn.
Introduction:
Liquid that fills the lung lumen during normal fetal development must be
absorbed into the vascular system soon after birth to permit successful
pulmonary gas exchange.
This transition occurs rapidly in most infants, but sometimes the process is
delayed, producing the clinical and radiographic features of a condition that is
known as transient tachypnea of the newborn (also called the syndrome of
retained fetal lung liquid).
Risk factors:
1. Term babies born by cesarean section
2. Preterm infant
3. Maternal asthma
4. Maternal diabetes.
Clinical features:
The disorder typically begins soon after birth with a rapid respiratory rate,
ranging from 60-100 per minute.
The baby remains alert and active and maintains good color.
Sometimes sternal and subcostal retractions of the chest wall, grunting during
expiration and occasionally mild cyanosis that disappears with delivery of
supplemental oxygen may be seen.
Signs and symptoms usually resolve by 3-4 days after birth.
Radiologic appearance:
Findings suggestive of excessive fluid in the lungs:
1. Hyperextended lung fields
2. Prominent pulmonary vascular markings (particularly around the hilum)
3. Thickened interlobar fissure
4. Flattening and depression of diaphragm
5. Fluid may be found in pleural space.
Treatment:
The condition usually resolves spontaneously without any specific therapy within
2-3 days of life.
Some infants may require administration of 40% O2 to maintain good color.
Infants with respiratory distress sometimes benefit from being managed in the
prone, head-up position.
3.b. Clinical presentation and management of acute bronchiolitis.
Introduction:
Acute bronchiolitis is a common serious acute lower respiratory tract infection (LRTI)
caused by respiratory syncytial virus (RSV) in the age group of 1-6 months.
Inflammation of bronchial mucosa leads to edema and mucus plug formation; which
narrows the airway and increases airway resistance both during inspiration and
expiration; which may progress to emphysema and atelectasis. Due to diminished
ventilation and perfusion, hypoxia and retention of CO2 may lead to respiratory failure.
Clinical presentation:
Few days following an upper respiratory tract infection; breathing becomes fast
and respiratory distress develops.
Majority of the infants have only mild symptoms and recovers within 3-7 days.
Those with severe disease, the following features are seen:
Retraction of lower intercostal spaces and suprasternal notch
Dyspnea ± cyanosis
Moderately high fever
Accessory muscles of respiration are working
Expiration is prolonged
Fine crepitations and rhonchi in auscultation
Breath sounds are faint/ absent
Respiratory distress is out of proportion to the physical signs
Hyperinflation results in pushing down of liver and spleen
If chest is overinflated, increased resonance is noted on percussion.
Management:
Treatment of acute bronchiolitis is symptomatic. Infants with mild symptoms can
be cared at home in a humidified atmosphere.
If respiratory distress/ feeding problems occur, the child should be hospitalized
and following treatment are given:
a. Position: The child is kept in a humidified atmosphere in sitting position at an
angle of 30⁰-40⁰ with head and neck elevated.
b. Oxygen:
Moist oxygen is given continuously even in the absence of cyanosis
Infants with severe disease should be given 60% moist oxygen
continuously given through a hood
Pulse oximetry should be done frequently to maintain an oxygen
saturation above 92%.
c. Fluid and electrolyte balance should be maintained.
d. Bronchodilators:
If the child shows improvement with bronchodilators, then it may be
given every 4-6 hourly
The bronchodilator of choice is inhaled salbutamol with ipratropium
and epinephrine.
e. Antiviral agent:
Ribavirin is an antiviral agent which has no role in treating infants with
acute bronchiolitis who were previously healthy.
But it can shorten the course of disease in infants with:
a. Congenital heart disease
b. Chronic lung disease
c. Immunodeficiency.
Ribavirin is delivered through a nebulizer 16 hours a day for 3-5 days.
3.c. OPV versus IPV
Points OPV IPV
Potency Low (needs ≥4 doses) High (needs 2-3 doses)
Intestinal immunity (IgA production) High Low
Secondary (herd) immunization Yes No
Purpose Community protection Individual protection
Role in times of epidemic Definite role No role
Reverts to virulence? No Yes, very rarely
Disease in immunocompromised? No Yes
Risk of escape of wild virus Non-existent Possible (if produce with
wild virus seeds)
Possible combination vaccine No Yes (with DPT and Hib)
Price Low High
Injection safety No issue A possible risk
Vaccine associated paralytic polio 1 case/ 2.5 million doses None
(VAPP)
3.d. Phototherapy
Introduction:
Phototherapy remains the mainstay of treating hyperbilirubinemia in neonates. It is
highly effective and carries an excellent safety track record for over 50 years.
Mechanism:
Phototherapy acts by converting insoluble bilirubin into soluble isomers that is
excreted in urine and feces.
There are 2 main modes of action:
a. Configurational isomerization: This is a reversible reaction where the Z
isomers are converted into E isomers. This is a not a major reaction.
b. Structural isomerization: This is an irreversible reaction where bilirubin is
converted to lumirubin. This is the major reaction.
Wavelength of choice: 460- 490 nm (Blue-green light).
Minimum irradiation level: 30 microwatt/sq.cm./nm.
Types of phototherapy lights:
CFL lights (most commonly used in India)
Blue LED lights
Halogen bulbs
Fibre-optic light sources.
Guidelines of a successful phototherapy:
Irradiance of lights should be periodically measured
Lamps should be changed if they are flickering/ ends are blackened/ irradiance
level falls below the specified level
Expose maximal surface area of the baby
Avoid blocking of lights by any equipment/ coverings of the baby
Ensure good hydration and nutrition of the baby
Make sure that the light falls on the baby perpendicularly if the baby is in
incubator
Minimize interruption of phototherapy during feeding sessions/ procedures.
Administering phototherapy:
Make sure the temperature of the room is 25⁰-28⁰C to prevent hypo/hyper-
thermia of the baby
Remove all clothes of the baby except the diaper
Cover the baby’s eyes with an eye-patch that does not block the baby’s nostrils
Place the naked baby:
o In a cot/ bassinet if the weight is >2kg
o In an incubator/ under radiant warmer if the weight is <2kg
Keep the distance between the baby and the light between 30-45 cm
Ensure optimum breastfeeding and during breastfeeding sessions, remove eye-
patch for better mother-child interaction.
Monitoring and stoppage of phototherapy:
If TSB (total serum bilirubin) ≥25 mg/dL, repeat TSB in 2-3 hr
If TSB 20-25 mg/dL, repeat TSB in 3-4 hr
If TSB <20 mg/dL, repeat TSB in 4-6 hr
If TSB continues to fall, repeat TSB in 8-12 hr
When TSB is <13-14 mg/dL, discontinue phototherapy
If TSB is not decreasing/ is moving closer to level for exchange transfusion/ the
TSB: albumin ratio exceeds recommended limits, consider exchange transfusion.
Group D
4. A 7 year old child with H/O exchange transfusion in neonatal period presents with
hematemesis. Physical examination is unremarkable except for splenomegaly (6 cm).
a. What is your differential diagnosis?
b. Describe the steps in the management of this child. (1+7)
My provisional diagnosis is extra-hepatic portal venous obstruction.
Explanation:
In this question, H/O exchange transfusion is insignificant as exchange
transfusion has many indications other than severe hyperbilirubinemia (severe
anemia, polycythemia, severe electrolyte imbalance etc.).
Concomitant presence of hematemesis and splenomegaly suggest a common
presentation of portal hypertension, the cause of which may be extra-hepatic or
intra-hepatic.
In India, the extra-hepatic portal venous obstruction (EHPVO) is the commonest
cause of portal hypertension (50-75%); followed by intra-hepatic portal venous
obstruction (IHPVO: Cirrhosis: 25-35%).
The common presentations include:
Entity Common clinical presentation
EHPVO Upper GI bleeding (hematemesis/ melena) + Splenomegaly
IHPVO Jaundice + Ascites + Hepatosplenomegaly ± Upper GI bleed
- So, from the clinical presentation, we can conclude that the cause of portal
hypertension in the mentioned patient is extra-hepatic.
Management:
There are 3 parts in the management of a patient with portal hypertension:
1. Emergency management of bleeding
2. Prophylaxis of first episode of bleeding (primary prophylaxis)
3. Prophylaxis of subsequent bleeding episodes (secondary prophylaxis).
Emergency management of bleeding
A. Airway:
Must be protected, particularly if there is risk of aspiration
If required: Oropharyngeal suction.
B. Breathing:
Oxygen
Ventilation.
C. Circulation:
1 wide bore cannula in each hand
IV fluid resuscitation (Preferred fluid of choice: Normal saline)
In case of severe bleeding: Blood transfusion (maintain a Hb level of 7-9 gm %)
Treat any co-existing coagulopathy (platelet, vitamin K, fresh frozen plasma).
D. Drugs:
Reduction of bleeding by splanchnic (and also systemic) vasoconstriction:
o Vasopressin
o Terlipressin
o Glypressin.
Safer and selective splanchnic vasoconstrictors (with fewer side effects):
o Somatostatin
o Octreotide.
IV antibiotic therapy in all patients (to reduce the risk of potentially life-
threatening infections).
E. Endoscopy:
Endoscopy confirms the presence of varices and subsequently they can be
treated endoscopically by the 2 following techniques, the mechanism of both of
which is stoppage of bleeding by variceal thrombosis:
Variceal ligation (banding)
Injection sclerotherapy:
Intra and para-variceal administration of sclerosing agents:
o Ethanolamine oleate
o Sodium tetradecyl sulfate.
F. Failure of endoscopy/ not feasible:
Balloon tamponade on varices by Sengstaken-Blackmore tube (SSBT): It is a last
resort and highly effective in controlling variceal bleeding; but it is associated
with significant complications (high incidence of re-bleeding and aspiration
pneumonia after removal of the tube).
Prophylaxis of first episode of bleeding (primary prophylaxis)
Nonselective β blocker:
Propranolol reduces portal pressure by causing splanchnic vasoconstriction and
reducing cardiac output.
Oral nitrates:
Nitrates reduces portal pressure by reducing hepatic vascular resistance.
Prophylaxis of subsequent bleeding episodes (secondary prophylaxis)
Various options are:
Variceal ligation
Sclerotherapy
Surgical portosystemic shunting
Transjugular intrahepatic portosystemic shunting (TIPS)
Liver transplantation.
WBUHS Pediatrics (including Neonatology) 2011
Group A
1. Describe formation, circulation, absorption and composition of CSF. Give an outline
of management of tubercular meningitis of 2 year old child. (1.5×4)+4
Formation of CSF:
Site of formation:
The CSF is formed by the choroid plexus (50%) and directly from the walls of the
ventricles (50%). The choroid plexus is a venous plexus contained within the four
ventricles of the brain.
Physiology:
The continuous formation of CSF by the choroid plexus occurs in 2 stages:
First, plasma is passively filtered across the choroidal capillary endothelium.
Next, secretion of water and ions across the choroidal epithelium provides for
active control of CSF composition and quantity.
HCO3-, Cl- and K+ ions enter the CSF via channels in the epithelial cell apical
membranes.
Aquaporins provide for water movement to balance osmotic gradients.
Circulation of CSF:
The majority of CSF is produced from within the two lateral ventricles.
Lateral ventricles
Interventricular
foramina of Monro
3rd ventricle
Cerebral aqueduct of
Sylvius
4th ventricle
Foramen of 2 Foramens of
Magendie Luschka
Absorption of CSF:
In adults, the main route for reabsorption of CSF is via the arachnoid
granulations, from where it returns to the vascular system by entering the dural
venous sinuses.
However, in neonates, the arachnoid granulations are not well developed and
sparsely distributed. So, in neonates, lymphatic drainage plays an important role
in CSF absorption. CSF flow along the cranial nerves and spinal nerve roots allow
it to drain into the lymphatic channels.
Composition of CSF:
The reference range for CSF analysis is as follows:
Parameter Value
Appearance and color* Clear, colorless
Opening pressure* 90-180 mm H2O (with patient lying in lateral position)
Glucose 50-80 mg/dL (or > 2/3rd of blood glucose)
Total protein 15-60 mg/dL
Chloride 110-125 mEq/L
Lactate (newborn) 10-40 mg/dL
Lactate (older children) 10-25 mg/dL
pH 7.28 - 7.32
WBC count 0-5
RBC count 0
[*Not needed in this question.]
Interpretation:
If ≥2 parameters are abnormal, it should be considered as a positive sepsis screen
and it is reasonable to start antibiotic therapy.
If a septic screen is negative in the presence of strong clinical suspicion, it should
be repeated within 12 hours.
If the screen is still negative, sepsis can be excluded with reasonable certainty.
Special note:
For early onset sepsis (occurring within first 72 hours of life), documentation of
polymorphs in the neonatal gastric aspirate at birth serves as a marker of
chorioamnionitis and it may be taken as one parameter of sepsis screen.
Group C
3.a. Laboratory diagnosis of acute glomerulonephritis
1. Urine analysis
2. Renal function test
3. Serology
4. Blood
5. Chest X Ray
6. USG abdomen.
Investigation Findings
Urine analysis Urine protein: Mild to moderate proteinuria
Microscopic examination:
Presence of dysmorphic/ crenated RBCs and RBC casts are diagnostic
WBCs, hyaline casts and granular casts are present
Note: Minimal urine findings may be present in children with severe clinical
features.
Renal function Blood urea-creatinine: Normal/ elevated (due to renal impairment)
tests Serum Na+: Hyponatremia may be present (due to impairment of free
water excretion)
Serum K+: Hyperkalemia may be present (due to decreased GFR)
Total serum protein: May be elevated due to increased γ-globulins.
Serology Following serological tests may be done:
ASO titre (high in pharyngitis)
Anti-DNase B (high in streptococcal skin infections)
- These titres decrease to low levels within 4-6 weeks.
Blood Hemoglobin: May be low due to dilution
Occasionally, coagulation abnormalities are identified
Complement: C3 is low in 90% of patients but normalizes within 8-12
weeks. Persistently low C3 levels indicate other forms of GN.
Chest X Ray It shows pleural fluid and mild cardiomegaly (due to pericardial effusion)
Gross cardiomegaly may be seen in severe volume overload and is an
impending sign of cardiac failure (a complication of acute GN)
Consolidation may be seen in presence of streptococcal pneumonia.
USG abdomen It shows bilaterally enlarged kidneys with mild parenchymal changes
Free fluid in the abdomen and pleural fluid are other common findings.
Renal biopsy Renal biopsy is not routine done and only done in following indications:
1. Renal function is severely impaired beyond 7-10 days
2. Serum C3 remains depressed beyond 6-8 weeks.
Microcephaly is defined as head circumference, >3SD below the mean for the age
and sex.
Macrocephaly is defined as a head circumference >2SD above the mean for age
and sex.
Group D
4. A 2 year old child has presented with fever for 20 days. Examination revealed severe
pallor, hepatosplenomegaly and purpuric spots all over the body.
a. Write the differential diagnosis
b. Suggest investigations to reach the final diagnosis. (5+3)
Differential diagnosis:
Malignancy:
1. Acute lymphoblastic leukemia (ALL)
2. Acute myeloid leukemia (AML)
3. Non-Hodgkin lymphoma (NHL).
Infections:
1. Chronic malaria
2. Chronic kala-azar
3. Disseminated (miliary) tuberculosis.
Discussion about the differentials (points in favor of diagnosis):
1. ALL:
Age of presentation: 2-5 years
Common clinical features include:
a. Pallor and fatigue
b. Petechiae/ purpura
c. Infections/ fever
d. Lymphadenopathy
e. Hepatosplenomegaly.
2. AML:
Age of presentation: It can occur at any age but more incidence is seen in
adolescence and during first 4 weeks of life
Clinically, AML presents similar to the ALL but lymphadenopathy and
hepatosplenomegaly are less common in AML.
3. NHL:
Age of presentation of NHL in children: Usually >3 years of age
Children with NHL typically present with extranodular disease involving
mediastinum/ abdomen/ head-neck region and may present with various
features like:
a. Mediastinum: Superior mediastinal syndrome/ Superior vena caval
syndrome/ Pleural or pericardial effusion
b. Abdomen: Abdominal pain/ ascites/ hepatosplenomegaly/ palpable mass/
intestinal obstruction/ intussusception
c. Head-neck region: Cervical adenopathy/ jaw swelling etc.
d. Note that, constitutional symptoms are uncommon in NHL, except in
patients with anaplastic large cell lymphoma (LCL). Many of these patients
have low-grade fever, malaise, anorexia ± weight loss.
4. Chronic malaria:
Any cases of long term fever should be investigated for malaria as it may
involve any system of the body and present with a very wide variety of clinical
signs and symptoms ranging from its characteristic paroxysm to neurological
manifestations as in cerebral malaria.
5. Chronic kala-azar:
Age of presentation: Although all age groups are affected, children aged 1-4
years are more susceptible.
Clinical features:
High grade fever Pallor Hyperpigmentation of
Weight loss Bleeding skin in late stages (face,
Hepatosplenomegaly manifestations (in hands, upper trunk)
Abdominal discomfort form of petechiae, Cough
Lymphadenopathy (<5%) epistaxis, gum Diarrhoea
bleeding etc.) Secondary infections
6. Disseminated tuberculosis:
Age of presentation: <3 years of age
Introduction:
The majority of children with TB infection develop no signs and symptoms at
any time. Occasionally, the initiation of the infection is marked by several days
of low grade fever and mild cough. Most children who develop tuberculosis
disease experience pulmonary manifestations, but 25-35% of children have an
extrapulmonary presentation.
Other clinical features:
Hepatomegaly Fever lasting several weeks with daily
Splenomegaly spikes in morning temperatures
Pancreatitis Hypercalcemia
Multiple organ dysfunction with Cutaneous lesions
adrenal insufficiency Choroidal tubercles
Investigations to reach the final diagnosis
1. Routine investigations:
a. Blood: Hb, TC, DC, Platelet count, CRP/ESR
b. Renal function test: Na+ K+ Urea Creatinine
c. Liver function test: Bilirubin (direct and indirect), albumin, ALT, AST, ALP.
d. Coagulation profile: BT, CT, PT, aPTT
e. Urine analysis: Urinary protein; note any presence of cast, RBC, WBC etc.
f. Chest X Ray
2. Special investigations:
a. Peripheral blood smear examination
b. Bone marrow aspiration and examination
c. Bone marrow biopsy (in selected cases)
d. Lumber puncture and examination of CSF cytology
e. CT and MRI (in selected cases).
3. Specific investigations:
These are specific investigations for suspected specific diseases.
Ex.:
Peripheral thick and thin blood smears for malaria
Smears of tissue aspirate for kala-azar
Sputum smear/ culture/ tissue biopsy in disseminated TB etc.
Cretinism:
Clinical features:
Cretinism is the most serious consequence of iodine deficiency. It occurs only
in geographic association with endemic goiter.
There are 2 types of cretinism: neurological and myxedematous. There are
some common clinical features and some differentiating clinical features and
laboratory findings, which are as follows: [Reference: Nelson’s Paediatrics]
Neurological cretinism Myxedematous cretinism
Common features
Mental retardation
Deaf-mutism
Neurological symptoms (Ex.: Clonus of the foot, Babinski sign, Patellar
hyperreflexia).
Differentiating features
Normal growth and pubertal Delayed growth and sexual
development development
Affected persons are goitrous but Absence of goiter (USG shows
euthyroid thyroid atrophy)
No myxedema Myxedema (due to fluid retention)
Little or no impaired thyroid function Serum T4 levels are low and TSH
levels are markedly elevated
Disturbance in stance and gait Not common
Treatment:
Cretinism is best to prevent than to treat because, once the neuromotor
deficiency and mental retardation ensues; it is irreversible.
Iodized salt and iodized oil are highly efficacious in preventing iodine
deficiency.
Recommended daily iodine intake:
Age group RDI (in µg)
Children upto the age of 10 years 40-120
Older children and adults 150
Pregnancy 175
Lactation 200
Method of assessment:
Blood pressure cuff is applied over arm, cuff pressure is increased
till brachial pulse is obliterated and then cuff pressure is released
and brachial artery is auscultated (as done while measuring BP).
At one point, the first Korotkov sound appears and it is noted.
While releasing the cuff pressure, at another point, the Korotkov
sound becomes regular and almost double. This point is noted.
The difference between these two points is the degree of pulsus
paradoxus.
Interpretation:
Degree of pulsus paradoxus Interpretation
<10 mm Hg Normal
10-20 mm Hg Pericardial effusion/ Constrictive pericarditis
>20 mm Hg Cardiac tamponade (Severe pericardial
effusion causing impairment of cardiac filling)
Group D
4. A 4 years old child presented with pallor, fever, gum bleeding and 1.5 cm
palpable spleen. Mention the diagnostic possibility and investigations to
confirm diagnosis. (3+5)
Diagnostic possibility:
The differential diagnoses should include:
Malignancy:
1. Acute lymphoblastic leukemia (ALL)
2. Acute myeloid leukemia (AML)
Infections:
1. Chronic malaria
2. Chronic kala-azar
3. Disseminated (miliary) tuberculosis.
Please see the 2011 Group D Question no. 4 for rest of the answer.
Paediatrics including neonatology
WBUHS 2013
Group A
1. Pathophysiology of large VSD and the future changes in hemodynamics if remain
untreated. Mention the complications of VSD. Outline the treatment of VSD with
CCF. (5+3+2)
Pathophysiology of large VSD:
Introduction:
VSD is a characterized by a defect in the membranous part of ventricular septum with
variable extension into the muscular septum; causing an abnormal communication between
left and right ventricle; allowing blood to flow from LV to RV, i.e. along the pressure gradient
throughout the entire period of systole.
Magnitude of the shunt:
Just after birth, the pulmonary vascular resistance remains elevated for some period,
limiting the size of shunt.
Few weeks after birth, the media of small pulmonary arterioles begins to involute; so
the pulmonary vascular resistance begins to fail and size of shunt starts to increase.
Eventually, a large left-to-right shunt develops, and clinical symptoms become
apparent.
Reversal of the shunt: (Eisenmenger syndrome)
In most cases during early infancy, the major contribution to pulmonary arterial
hypertension (PAH) is the large communication between ventricles allowing
exposure of the pulmonary circulation to systemic pressure; resulting in large
pulmonary blood flow.
With continued exposure of the pulmonary vascular bed to high systolic pressure
and high flow, pulmonary vascular obstructive disease eventually develops.
When the ratio of (pulmonary: systemic) resistance approaches 1:1, the shunt
reverses, signs of heart failure abate, and the patient begins to show signs of
cyanosis. This event is known as “Eisenmenger syndrome”.
Heart sounds:
Shunt murmur: Pansystolic murmur along lower left sternal border (4th intercostal
space), which may become palpable (then it is called a thrill).
Flow murmur:
Pulmonary valve: A mid systolic murmur may be heard over pulmonary area
due to functional pulmonic stenosis.
Mitral valve: A mid diastolic murmur may be heard over mitral area due to
functional mitral stenosis.
In clinical practice, these murmurs are very hard to differentiate from the shunt
murmur (pansystolic) as it often masks all other sounds.
Complications of VSD:
1. Pulmonic stenosis.
2. PAH.
3. Aortic regurgitation.
4. Infective endocarditis.
Discussion of complications of VSD
Group B
Write briefly on the following: (5 marks each)
2.a. Kangaroo mother care (KMC):
Introduction:
KMC refers to care of preterm/ low birth weight (LBW) babies by placing the infant in skin-
to-skin contact with the mother/ any other caregiver.
Components:
1. Kangaroo position:
This consists of skin-to-skin contact between mother and infant; keeping the
infant in a vertical position between the mother’s breasts and under her
cloths.
Mother should keep herself in a semi-reclining position to avoid gastric
reflux of the infant.
Head of the baby should be turned to one side and slightly extended to keep
the airway open.
Hips should be flexed and abducted (frog position).
Arms should be flexed.
Baby’s abdomen should at the level of mother’s epigastrium.
Clothing in KMC:
1. For mother: Any light weight front open dress as per local culture is suitable for KMC.
2. For baby: Baby is dressed with cap, socks, nappy and front open sleeveless shirt.
Monitoring:
Nursing stuff should monitor the baby especially during initial stages:
The baby’s position is neither too flexed nor too extended (clear airway).
Breathing is regular.
Colour of the baby is pink.
Baby is maintaining adequate temperature.
Duration:
Sessions of KMC <1 hour should be avoided because it may be stressful for the baby.
Afterwards, sessions should be gradually prolonged upto 24 hours, interrupted only
for changing diapers.
Stopping of KMC:
Septic screening should be done to support the clinical suspicion of infection before
the institution of specific antibacterial therapy. However, no investigation is required
to start treatment in a sick baby having high probability of sepsis.
The reliable markers of neonatal septicemia include:
a. Total leukocyte count <5000/ cu.mm,
b. Absolute neutrophils count <1800/ cu.mm,
c. (Immature: mature) total neutrophils ratio >20%,
d. Raised micro-ESR (≥15 mm in 1st hour) and
e. Positive C-reactive protein (>8 mg/ ml).
Blood culture and CSF examination (by lumbar puncture) are mandatory before
initiating specific antibacterial therapy.
An X-Ray of the chest should be taken in all cases. It may show scattered or localized
opacities suggestive of patchy consolidation.
Blood should be examined for glucose, bilirubin, urea and electrolytes.
Premature rupture of membrane:
The aim of post exposure prophylaxis is to neutralize the inoculated virus before it can enter
the CNS. Every case of human exposure should be treated as medical emergency.
Post exposure prophylaxis
The type of post exposure prophylaxis depends on the type/ degree of contact, which has
been classified into 3 categories: They are as follows:
The purpose of local treatment is to remove as much virus as possible from the site
of inoculation before it can be absorbed on nerve endings.
It should never be neglected and should be done in every case.
Local treatment comprises of the following processes:
1. Cleansing:
Immediately flush and wash the wound[s], scratches and adjoining areas
with plenty of soap and water, preferable under a running tap, for at least
15 minutes.
If soap is not available, simply flush the wound with plenty of water.
In case of punctured wounds, use catheters to irrigate the wound.
2. Chemical treatment:
The residual virus remaining in the wound should be inactivated by:
Alcohol/
Tincture iodine/
Povidone iodine.
3. Suturing:
Bite wounds should not be immediately sutured to prevent additional
trauma which may help spread the virus into deeper tissues.
If suturing is necessary, it should be done 48-72 hours later, applying
minimum possible stitches under the cover of rabies immunoglobulin
locally.
4. Antibiotic and anti-tetanus measure:
It should follow the local treatment mentioned above when indicated.
Intradermal schedule
WHO recommended the following intradermal regimen and vaccines for use by the
intradermal route:
2-site intradermal method:
The regimen is (2-2-2-0-1-1).
The volume per intradermal site is: 0.1 ml.
Introduction:
It is characterized by hematogenous spread and progressive development of
innumerable small foci throughout the body.
Clinical features:
1. Symptoms:
High grade fever,
Dyspnea,
Rigors,
Alteration of sensorium,
Weight loss.
2. Signs:
Cyanosis,
Lymphadenopathy,
Hepatosplenomegaly,
Fine crepitation and rhonchi,
Choroid tubercles,
Signs of meningitis may be present in 20-30% of cases.
Diagnosis:
1. Demonstration of tubercle bacilli/ its components:
a. ZN stain.
b. Special stain.
c. Culture in LJ medium/ BACTEC assay.
d. PCR.
2. Demonstration of host’s response to tubercle bacilli:
Tuberculin test.
3. Radiological investigations:
Demonstration of following features in X-Ray:
a. Airspace consolidation.
b. Lymphadenopathy.
c. Bronchiectasis.
Demonstration of following features in CT-Scan:
a. Low attenuation of lymph nodes.
b. Lymph node calcification.
c. Branching Centrilobular nodules.
d. Miliary nodules.
Treatment:
{2(HRZE) + 4(HR)}3: 2 months of HRZE and 4 months of HR: thrice weekly DOTS is
recommended under RNTCP.
3.d. Pulse polio immunization.
Introduction:
Pulse polio is an immunization campaign established by the Govt. of India in 1995-96
to eradicate polio in India by vaccinating all children under the age of 5 years against
polio virus.
Objectives:
1. The Pulse Polio Initiative (PPI) aims at covering every individual in the country. It
aspires to reach even children in remote communities through an improved
social mobilisation plan.
2. Not a single child should miss the immunisation, leaving no chance of polio
occurrence.
3. Cases of Acute Flaccid Paralysis (AFP) to be reported in time and stool specimens
of them to be collected within 14 days. Outbreak Response Immunisation (ORI)
to be conducted as early as possible.
4. Maintaining high level of surveillance.
5. Performance of good mop-up operations where polio has disappeared.
Current condition:
On 27 March, 2014 WHO South-East Asia Region (including India) was certified polio-
free by an independent commission under the WHO certification process.
Group D
4. A 4 years old child presented with H/O fever for 7 days and recurrent convulsion for last 2
days and headache. How will you proceed for diagnosis clinically and by laboratory
investigation? (4+4)
Presenting symptoms:
1. Fever.
2. Recurrent convulsion.
3. Headache.
Take history of any other following symptoms:
1. Lethargy/ irritability.
2. Mental confusion/ altered level of consciousness.
3. Nausea/ vomiting.
4. Photophobia.
Look for following signs:
Classical signs:
1. Kernig sign:
This manoeuvre is usually performed with the patient supine with hips and knees
in flexion.
Extension of the knees is attempted.
The inability to extend the patient’s knees beyond 135 degrees without causing
pain constitutes a positive test for Kernig’s sign.
2. Brudzinski sign:
With the patient supine, the physician places one hand behind the patient’s head
and places the other hand on the patient’s chest.
The physician then raises the patient’s head (with the hand behind the head)
while the hand on the chest restrains the patient and prevents the patient from
rising.
Flexion of the patient’s lower extremities (hips and knees) constitutes a positive
sign.
Brudzinski’s neck sign has more sensitivity than Kernig’s sign.
Associated signs:
Serological tests
1. Antibody detection:
For this purpose, 2 types of serum can be used:
a. Acute serum for acute cases (IgM detection).
b. Convalescent serum for chronic cases (IgG detection).
2. In special situations like epidemic, special tests can be applied:
a. NS1 and IgM testing in dengue epidemic.
b. Rapid influenza diagnostic test (RIDT) in influenza epidemic.
Radioimaging
Neuroimaging, especially MRI has become the most sensitive diagnostic technique
for finding damages/ complications already occurred in case of meningitis.
The usual nonspecific findings on MRI, which can be found in meningitis of all
etiologies are:
a. Cerebral edema.
b. Infarct.
c. Hydrocephalus.
Treatment:
Starting of treatment:
Treatment should be started when there is clinical evidence of raised ICP or sudden
increase in ICP, i.e. >20 mm Hg for >3 minutes or 16-20 mm Hg for >30 minutes.
Modes of treatment:
1. Basic therapy
2. Advanced therapy.
Basic therapy
A: Airway
C: Circulation
T: Temperature control
A. Maintain Airway/ Avoid hypoxia:
Monitor oxygenation (target SaO2~100%) and apply supplemental O2 as
required.
If the patient has inadequate respiratory effort/ GCS <8/ rapid clinical
deterioration (signs of herniation, unequal pupils)/ status epilepticus
unresponsive to medications, then intubation should be attempted.
B. Breathing:
Maintain PaO2 >80 mmHg and PaCO2 30-35 mmHg.
Blood pressure:
Maintain mean arterial blood pressure (MAP) in the normal range for age.
C. Circulation:
Keep patient normovolemic, give normal saline. Hypotension, if present should
be treated aggressively.
D. Drugs:
I. Drugs for seizure control:
Seizure increases cerebral oxygen consumption and diminishes the
capacity to maintain ICP.
Midazolam 0.1- 0.2 mg/kg/IV given every 5 minutes till seizures are
controlled, load the patient with phenytoin.
II. Drugs for acute reduction of ICP:
Lidocaine helps in acute reduction of pressure (1.5 mg/kg). It should also
be instilled locally before endotracheal tube suctioning to avoid coughing.
III. Drugs for osmotherapy:
Osmotic and loop diuretics may be used:
• Mannitol: In low-dose 0.25-0.5 mg/kg of 20% solution given as bolus and
may be repeated 4-6 hourly.
• Furosemide: 0.5-1.0 mg/kg/IV may be given alone or with osmotic
diuretics.
IV. Sedation and paralysis:
Sedation decreases the sympathetic activity thus reduces hypertension
and plays a key role in management. Paralysis is used during controlled
ventilation.
V. Steroids:
Steroids are used in vasogenic edema (tumor, abscess and organized
subdural hematoma). Dexamethasone: Loading dose 1 mg/kg, then 0.25
mg/kg/IV 6 hourly.
E. External stimulus prevention:
Prevent external stimulus, monitor signs of over stimulation and keep
surrounding noise level less than 90 db.
H. Head position:
• Maintain head in midline position at 15⁰-30⁰ to improve cerebral venous
drainage; lower cerebral blood volume (CBV) will lower ICP.
• While turning the patient keep hip joint flexed <30°.
Hyperventilation:
Maintain mild hyperventilation if required, the effect will start within 30 sec and
peak in 8 minutes. This will lead to vasoconstriction and decrease in cerebral
blood flow thus resulting in acute reduction of raised ICP.
T. Temperature control:
Keep normothermic (36-37°C) as ↑Temperature ↑ CBF ↑ CBV ↑ ICP.
Clinical features:
Clinically, hypoglycemia may be asymptomatic/ may show a range of clinical features:
Stupor Lethargy
Tremor Difficulty in feeding
Apathy Eye rolling
Cyanosis Episodes of sweating
Convulsions Sudden pallor
Apneic spells Hypothermia
Tachypnea Cardiac arrest (rare)
Weak and high pitched cry
Management of hypoglycemia:
Hypoglycemia
Asymptomatic Symptomatic
Immediately followed
Trial of oral feeds IV glucose infusion by glucose infusion at
6 mg/kg/min
≥2 consecutive values
Repeat hypoglycemic are >50 mg/dL after
>40 mg/dL <40 mg/dL
episodes 24 hour of parenteral
therapy
Monitoring:
Hypoglycemia is linked to long-term adverse effects. These babies should be evaluated
at 3, 6, 9, 12 and 18 months for growth, neurodevelopment and vision and hearing loss.
3.b. Hemorrhagic diseases of newborn:
Basic physiology:
A moderate decrease in factors II, VII, IX and X normally occurs in all newborn
infants by 48-72 hr after birth, with a gradual return to birth levels by 7-10 days
of age.
This transient deficiency of vitamin K–dependent factors is probably due to lack
of free vitamin K from the mother and absence of the bacterial intestinal flora
normally responsible for the synthesis of vitamin K.
Rarely in term infants and more frequently in premature infants, accentuation
and prolongation of this deficiency between the 2nd and 7th days of life result in
spontaneous and prolonged bleeding.
Types:
There are 3 types of hemorrhagic diseases of newborn:
1. Classical disease
2. Early onset disease
3. Late onset disease.
Features Early onset disease Classical disease Late onset disease
Onset 0-24 hr 2-7 days 1-6 mo
Sites of Cephalohematoma, GI, ENT region, Intracranial, GI,
hemorrhage subgaleal, intracranial, intracranial, circumcision, cutaneous, ENT region,
GI, umbilical, intra- cutaneous, injection sites injection sites, thoracic
abdominal
Risk factors Maternal drugs Vitamin K deficiency Cholestasis:
(phenobarbital, Breast-feeding malabsorption of
phenytoin, warfarin, vitamin K (biliary atresia,
rifampin, isoniazid) that cystic fibrosis, hepatitis)
interfere with vitamin K
metabolism
Inherited
coagulopathy
Prevention Avoid high-risk Prevented by parenteral Prevented by parenteral
medications vitamin K at birth and high-dose oral
Administrations of vitamin K during periods
vitamin K (20 mg) to Oral vitamin K regimens of malabsorption or
infant at birth or to require repeated dosing cholestasis
mother before birth over time
Incidence Very rare ≈2% if infant not given Depends on primary
vitamin K disease
2.c. Enumerate the vaccines that can be given to an unimmunized 2 year old child.
IAP recommended general immunization schedule in case of an unimmunized child:
So, applying this table to a 2 year old child, we get the following:
Visit Suggested vaccines
First visit MMR
DTwP1/ DTaP1
OPV1/ IPV1
Hib1
HepB1
Second visit BCG
(after 1 month of first DTwP2/ DTaP2
visit) OPV2
HepB2
Third visit OPV3/IPV2
(after 1 month of MMR
second visit) Typhoid (as third visit in a 2 year old
child will be at 2 year 2 months)
Fourth visit DTwP3/ DTaP3
(6 months after first OPV4/IPVB1
visit) HepB3
Group C
3.a. Modified Jones’ criteria:
Major manifestations:
1. Carditis:
It occurs in 50% of acute rheumatic fever (ARF) patients. It may be
inflammation of one or more layers of heart, which show signs as follows:
Affected layer Sign
Pericarditis Pericardial friction rub/ effusion/ chest pain/ ECG changes
Myocarditis Tachycardia (out of proportion to the severity of fever)
Endocarditis (valvulitis) A murmur indicating MR/ AR is always present
Severe carditis Clinical signs of CHF (Gallop rhythm/ cardiomegaly)
Pancarditis/ Cardiomegaly on chest radiograph
Pericarditis/ CHF
2. Polyarthritis:
This is the most common manifestation of ARF (70%) and usually involves
large joints (knee, ankles, elbows, wrists).
Often, more than one joint, either simultaneously or in succession is
involved with a characteristic migratory nature of the arthritis.
Swelling, heat, redness, severe pain, tenderness and limitation of motion
are common.
The arthritis responds dramatically to salicylate therapy; if the arthritis
does not respond within 48 hours of the diagnosis of rheumatic fever is
probably incorrect.
3. Chorea:
Sydenham’s chorea is present in 15% of patients with ARF and is common
in pre-pubertal girls (8-12 years).
It begins with emotional lability and personality changes.
Then it progresses through loss of motor coordination, characteristic
spontaneous purposeless movements, followed finally by motor weakness
and hypotonia of muscles; which continue for an average of 7 months
before waning progressively.
4. Erythema marginatum:
It is seen in <10% of cases and is rare in Indian subcontinent.
The characteristic nonpruritic serpiginous/ annular erythematous rashes
are most prominent on the trunk and inner proximal portions of the
extremities, but never seen on face.
The rashes disappear on exposure to cold and reappear when covered with
a warm blanket.
5. Subcutaneous nodules:
Subcutaneous nodules are found in 2-10% of the cases.
They are hard, painless, nonpruritic, freely mobile and <2 cm in diameter.
They are distributed symmetrically, singly or in clusters over the extensor
surfaces of large and small joints, scalp and along the spine.
They last for weeks and are significantly associated with carditis.
Minor manifestations:
1. Arthralgia refers to a joint pain without the objective changes of arthritis.
Note that, if polyarthritis is already taken as a major criteria, then arthralgia
can’t be taken as a minor criteria.
2. Fever (with a temperature usually of at least 39°C) generally is present early in
the course of rheumatic fever.
3. In laboratory findings, acute phase reactants (elevated CRP levels and ESR) are
objective evidences of an inflammatory process.
4. A prolonged P-R interval on ECG is neither specific for acute rheumatic fever
nor an indication of active carditis.
Evidence of Antecedent Group A Streptococcal Infection:
A history of sore throat is not an adequate evidence of recent group A
streptococcal infection.
Positive throat swab cultures or rapid streptococcal antigen tests for group A
streptococci are less reliable than antibody tests, because they do not distinguish
between recent infection and chronic pharyngeal infection.
Streptococcal antibody tests are the most reliable laboratory evidence of
antecedent streptococcal infection capable of producing rheumatic fever. The
onset of the clinical manifestations of acute rheumatic fever coincides with the
peak of the streptococcal antibody response.
Some of the most commonly used Streptococcal antibody tests are Anti-
streptolysin O (ASO) titre and Streptozyme test.
3.b. Pneumatocele:
Introduction:
Pneumatoceles are intrapulmonary air-filled cystic spaces.
Age group:
Although pneumatoceles are seen in all age groups, they are most frequently
encountered in infancy.
Causes:
Staph.aureus (commonest)
Strep.pneumoniae
E.coli
Causes of pneumatocele
Klebsiella pneumoniae
Blunt trauma
Special causes
Positive pressure ventilation in
preterm neonates
Clinical presentation:
• Children present with typical features of pneumonia, including cough, fever and
respiratory distress. No clinical findings differentiate pneumonia with or without
pneumatocele formation.
• Occasionally pneumatoceles become large enough to compress adjacent lung
and the mediastinum enough to cause respiratory or cardiovascular symptoms.
Radiologic features:
• When mature, pneumatoceles appear as thin walled cystic spaces within the lung
parenchyma, containing air.
• They tend to appear within the 1st week of infection and usually resolve by 6th
week.
Complications:
1. Rupture of a pneumatocele may cause pneumothorax
2. Secondary infection: Secondarily infected pneumatocele.
Treatment:
• Post-pneumonic pneumatoceles tend to spontaneously resolve providing the
infection is adequately treated with antibiotics.
• Surgical intervention is only required if the pneumatocele causes symptoms due
to mass-effect or ruptures into the pleural space resulting in pneumothorax.
Dx:
I. Bronchogenic cyst
II. Lung abscess
III. TB
IV. Cystic adenomatoid malformation
V. Hyper IgE syndrome.
3.c. Common causes and laboratory diagnosis of iron deficiency anemia in children
Causes of iron deficiency anemia in children:
It can be divided into 4 distinct groups as follows:
1. Decreased intake
2. Increased loss
3. Decreased iron store
4. Increased iron demand.
Group Causes
Decreased intake Delayed weaning
Malnutrition and iron poor diet
Malabsorption syndromes
Chronic infection and chronic diarrhea
Increased loss • GI bleeding
• Malaria
• Hookworm infestation
• Peptic ulcer, diverticulitis
• Bleeding diathesis
• Fetomaternal hemorrhage
Decreased iron store • Preterms
• Small-for-dates
• Twins
Increased iron demand • Prematurity
• LBW
• Recovery from PEM
• Adolescence
*RDW is the coefficient of variation of red cell volume distribution. RDW is the objective
documentation of subjective anisocytosis.
3.d. Causes and clinical features of hyponatremia:
Introduction:
Under normal conditions, serum sodium is tightly regulated in between 135-145 mEq/L.
Hyponatremia is defined a serum sodium concentration of <130 mEq/L. But if the
concentration comes to <120 mEq/L, then it is usually associated with serious clinical
symptoms and must be corrected immediately
Causes:
Some common causes of hyponatremia and their short description:
1. Systemic dehydration
2. Decreased effective plasma volume (in CHF, nephrotic syndrome, cirrhosis,
positive pressure mechanical ventilation, severe asthma)
3. Primary polydipsia (increased water ingestion)
4. Decreased free water excretion (in adrenal insufficiency/ thyroid deficiency)
5. Primary salt loss (of renal and non-renal origin): PKD, CRF, Cystic fibrosis
6. Syndrome of inappropriate ADH secretion: SIADH (excessive administration
of vasopressin in the treatment of central diabetes insipidus, encephalitis,
brain tumors)
7. Cerebral salt wasting (Hypersecretion of Atrial natriuretic peptide [ANP] in
brain tumors, head trauma, hydrocephalus)
8. Runner’s hyponatremia (Excess fluid ingestion during long-distance running
can result in severe hyponatremia due to hypovolemia-induced activation of
Arginine vasopressin [AVP] secretion)
9. Pseudohyponatremia (Can result from hypertriglyceridemia, which result in a
relative decrease in serum water content)
10. Factitious hyponatremia (Can result from obtaining a blood sample proximal
to the site of intravenous hypotonic fluid infusion).
Clinical features of hyponatremia:
Mechanism of manifestations:
• Hyponatremia causes a decrease in the osmolality of the extracellular space.
• Because the intracellular space then has a higher osmolality, water moves from
the extracellular space to the intracellular space to maintain osmotic equilibrium.
• The increase in intracellular water causes cells to swell. Although cell swelling is
not problematic in most tissues, it is dangerous for the brain, which is confined
by the skull. Brain cell swelling is responsible for most of the symptoms of
hyponatremia.
• As brain cells swell, there is an increase in intracranial pressure, which impairs
cerebral blood flow.
Manifestations:
• Acute, severe hyponatremia can cause brainstem herniation and apnea;
respiratory support is often necessary.
• Neurologic symptoms of hyponatremia include anorexia, nausea, vomiting,
malaise, lethargy, confusion, agitation, headache, seizures, coma and decreased
reflexes. Patients may have hypothermia and Cheyne-Stokes respirations.
• Hyponatremia can cause muscle cramps and weakness; rhabdomyolysis can
occur with water intoxication.
Group D
4. A 6 year old girl child is admitted with hematuria, moderate edema and headache.
Mention the possible differential diagnosis. How will you evaluate such a case to reach
to a definite diagnosis? (2+6=8)
Although the typical presentation is more suggestive of a picture of acute nephritic
syndrome, the differential diagnoses should include the following:
1. Acute nephritic syndromes
2. Nephrotic syndromes, especially:
a. Membranoproliferative glomerulonephritis (MPGN)
b. Idiopathic Focal segmental glomerulosclerosis (FSGS).
Evaluation of such a case:
Investigation Nephritic syndrome Nephrotic syndrome
Urine analysis Urine protein: Mild to moderate Urine protein: Nephrotic range
proteinuria (1+/2+) proteinuria (3+/4+)
Microscopic examination: Microscopic examination:
Presence of dysmorphic/ Gross hematuria/ persistent
crenated RBCs and RBC casts microscopic hematuria suggests
are diagnostic the likelihood of significant
WBCs, hyaline casts and glomerular lesions
granular casts are present. Hyaline casts and granular cases
are present.
Renal function Blood urea-creatinine: Usually Blood urea-creatinine:
tests normal/ may be elevated (due to In MCNS, blood urea levels are
renal impairment) normal unless edema is massive
with associated oliguria
Serum Na+: Hyponatremia may Persistently elevated urea and
be present (due to impairment of creatinine suggest the presence
free water excretion) of significant renal histologic
Serum K+: Hyperkalemia may be lesions
present (due to decreased GFR) Serum albumin: Hypoalbuminemia
Total serum protein: May be (<2.5 gm/dl)
elevated due to increased Total serum cholesterol:
γ-globulins. Hyperlipidemia (>200 mg/dl).
Blood Hemoglobin: May be low due to Serum IgM: High
hemodilution Serum IgG: Low
Complement: C3 is low in 90% of Complement: C3 level is usually
patients but normalizes within 8- normal; persistently decreased
12 weeks. serum C3 levels indicate MPGN.
Chest X Ray It may show evidence of pericardial Tuberculin test (if positive, a CXR) is
effusion/ gross cardiomegaly (an done to exclude TB (renal TB presents
impending sign of CHF)/ with persistent hematuria and pyuria).
consolidation in strep. pneumonia.
USG To detect free fluid in abdomen and pleura (when undetectable clinically)