Q-1. Fluid of choice in infant diarrhea
a) ORS
b) Salt water
c) Sugar water
d) Dextrose
Answer: ORS
Explanation:
ORS is fluid of choice in most patients with mild to moderate dehydration.
Oral zinc should be given to speedy recovery and decrease the risk of future diarrheal episodes.
Q-2. Dose of dexamethasone for threatened preterm labour
a) 12 mg 12 hourly 4 doses
b) 12 mg 12 hourly 2 doses
c) 6 mg 12 hourly 4 doses
d) 6 mg 12 hourly 2 doses
Answer: 6 mg 12 hourly 4 doses
Explanation:
Antenatal steroids decrease the risk of RDS, grade III and IV intra-ventricular hemorrhage, peri-ventricular leukomalacia and necrotizing enterocolitis and death in the neonate.
Dose of dexamethasone and betamethasone for threatened preterm labour:
Dexamethasone 6 mg 12 hourly 4 doses
Betamethasone 12 mg 24 hourly 2 doses
Q-3. A child with edema and decreased urine output, on evaluation, serum albumin is 2.5 g/dL, S. Creatinine is 0.5 mg/dL, and urine protein is 3+ with no RBC casts. Pathological change expected is
a) Minimal change disease
b) Interstitial nephritis
c) IgA nephropathy
d) FSGS
Answer: Minimal change disease
Explanation:
Minimal change disease:
Minimal-change disease is the most common single form of nephrotic syndrome in children.
A disorder of T cells, which release a cytokine that injures the glomerular epithelial foot processes leading to a decreased synthesis of polyanions. It causes leakage of albumin.
Dependent edema is the most prominent sign.
Diagnosis:
Critical level for diagnosis is proteinuria of more than 40 mg/h/m2.
A random albumin-to-creatinine concentration ratio is in excess of 5.
Serum albumin of less than 2.5 g/dl
Hyperlipidemia
Q-4. One month old child presents with conjugated hyper-bilirubinemia and intra-hepatic cholestasis. On liver biopsy and staining with PAS, red colored granules were seen inside the hepatocytes. Probable diagnosis is
a) Alpha 1-antitrypsin deficiency
b) Congenital hepatic fibrosis
c) Extra-hepatic biliary atresia
d) Malformation of duct
Answer: Alpha 1-antitrypsin deficiency
Explanation:
Alpha 1-antitrypsin deficiency:
A genetic inherited autosomal co-dominant condition
Alpha1-antitrypsin is the prototype member of the serine protease inhibitor (serpin) super-family of proteins.
Caused by mutations in the SERPINA1 gene located in the long arm of chromosome 14
This genetic defect alters the configuration of the alpha1-antitrypsin molecule and prevents its release from hepatocytes.
The alpha1-antitrypsin molecule normally would serve as protection against proteases such neutrophil elastase.
Investigations:
Serum alpha 1-antitrypsin level
Liver biopsy and staining with PAS- Red colored granules inside the hepatocytes
Q-5. An infant at 7 months with history of vomiting and failure to thrive improved with IV glucose. After one month, infant returns with same complaints. On evaluation found to have high glutamine and uracil. Which is the likely enzyme defect?
a) CPS1
b) Ornithine trans-carbamylase
c) Arginase
d) Arginosuccinase lyase
Answer: Ornithine trans-carbamylase
Explanation:
Defective ornithine trans-carbamylase:
Ornithine transcarbamylase (OTC) deficiency is an X-linked genetic disorder of the urea cycle that leads to elevated levels of ammonia in the blood.
Levels of glutamine are elevated in blood, CSF and urine, probably as result of enhanced glutamine synthesis in response to elevated levels of tissue ammonia.
Immediate temporary discontinuation of protein intake
Compensatory increases in dietary carbohydrates and lipids