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PE23.1-21 | Gastrointestinal and Hepatobiliary System — Graded Quiz
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A 5-day-old neonate is brought with failure to pass meconium, abdominal distension, and bilious vomiting. Digital rectal examination precipitates an explosive discharge of stool and gas. Which investigation will confirm the diagnosis?
Rectal suction biopsy with absent ganglion cells (and hypertrophied nerve fibres on acetylcholinesterase staining) is the gold standard for confirming Hirschsprung disease. Barium enema demonstrates the transition zone but suction biopsy is the confirmatory test.
Hirschsprung disease gold standard diagnosis: rectal suction biopsy (absent ganglion cells on H&E + AChE staining). Failure to pass meconium within 48 hours of birth should prompt evaluation. Barium enema transition zone is supportive but not confirmatory.
Barium meal is for upper GI evaluation. A normal RAIR (recto-anal inhibitory reflex) would EXCLUDE Hirschsprung — in HD the RAIR is absent, which supports diagnosis but suction biopsy remains the gold standard. Full-thickness biopsy is not the first-line — suction biopsy (submucosa) is sufficient in experienced hands.
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A severely malnourished 18-month-old presents with profuse watery diarrhoea and clinical signs of severe dehydration (lethargic, sunken eyes, skin pinch returns very slowly). He is unable to drink. Which is the correct initial fluid management (WHO Plan C)?
WHO Plan C for severe dehydration: IV Ringer's lactate (or normal saline if RL not available) — for infants: 30 mL/kg IV over 30 min, then 70 mL/kg IV over 2.5 hours. Reassess after each stage. If IV line cannot be established, use NG route with ORS. 5% dextrose alone is inappropriate (no electrolytes).
WHO Plan C (severe dehydration): IV RL 30 mL/kg over 30 min + 70 mL/kg over 2.5 hours in infants; reassess after each phase. Use NG ORS only if IV unavailable. 5% dextrose alone is NEVER used for gastroenteritis rehydration.
20 mL/kg rapid saline bolus is used in septic shock (but evidence in paediatric diarrhoeal disease favours WHO Plan C protocol). NG ORS is a fallback if IV access fails. 5% dextrose has no electrolytes and should not be used alone for rehydration.
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A child with bloody diarrhoea develops haemolytic uraemic syndrome (HUS: microangiopathic haemolytic anaemia + thrombocytopenia + acute renal failure). His stool culture grows an E. coli strain producing Shiga toxin (O157:H7). What is the correct statement about antibiotic use in this child?
Antibiotics are CONTRAINDICATED in EHEC (enterohaemorrhagic E. coli, Shiga toxin-producing) diarrhoea/HUS because they lyse bacteria and promote Shiga toxin release, worsening thrombotic microangiopathy and HUS. Management of HUS is supportive: fluid management, dialysis if needed, transfusion.
EHEC (O157:H7) dysentery leading to HUS: antibiotics are CONTRAINDICATED — they worsen HUS by releasing Shiga toxin. Management is supportive. Contrast with Shigella dysentery, where antibiotics are RECOMMENDED. Know this distinction — it is a high-stakes clinical decision.
No antibiotic (azithromycin, ceftriaxone, metronidazole, fluoroquinolone) should be given for EHEC infection. The bacterial lysis from antibiotics releases more preformed Shiga toxin, worsening HUS. Shigella dysentery, in contrast, REQUIRES antibiotics.
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A 3-year-old presents with recurrent episodes of colicky abdominal pain with drawing up of legs, followed by passing 'redcurrant jelly' stool. The abdomen shows a sausage-shaped mass in the right upper quadrant. What is the most appropriate initial treatment if the child is haemodynamically stable and no evidence of peritonitis?
The presentation is classic for intussusception (colicky pain, redcurrant jelly stool, sausage-shaped mass). In a haemodynamically stable child without peritonitis, air (pneumatic) enema under fluoroscopic guidance is the first-line non-surgical treatment with success rates of 75–90%.
Intussusception: colicky pain + redcurrant jelly stool + sausage-shaped mass. Dx: USG (target sign). Treatment: air enema (first-line in stable child); surgery if enema fails or peritonitis. Most common type: ileocolic.
Surgery is reserved for failed enema reduction or peritonitis. Corticosteroids have no role. Manual reduction under GA is NOT the standard approach — enema reduction is the initial treatment of choice when there are no contraindications.
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A 9-year-old child is evaluated for chronic diarrhoea, failure to thrive, and recurrent chest infections. Sweat chloride test on two separate occasions shows 75 mEq/L. Stool shows fatty globules. Which complication of the underlying condition directly explains the malabsorption?
Sweat chloride >60 mEq/L on two occasions = cystic fibrosis. In CF, defective CFTR causes thick mucus plugging of pancreatic ducts → acinar destruction → exocrine pancreatic insufficiency → fat, protein, fat-soluble vitamin malabsorption (steatorrhoea). Treatment: pancreatic enzyme replacement therapy.
CF diagnosis: sweat chloride >60 mEq/L on two occasions (or CFTR gene mutation). Malabsorption mechanism: exocrine pancreatic insufficiency. Treatment: PERT + fat-soluble vitamin supplementation + pulmonary physiotherapy. Coeliac: anti-tTG IgA + biopsy.
Villous atrophy is from coeliac disease (gluten), not CF. Bile acid malabsorption occurs with terminal ileal disease (Crohn's). Lactase deficiency causes osmotic diarrhoea without steatorrhoea or chest infections.
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A 2-year-old child is found to have hepatomegaly with abnormal LFTs. Serum ceruloplasmin is low (8 mg/dL; normal 20–40). Slit-lamp examination shows Kayser-Fleischer rings. 24-hour urine copper after penicillamine challenge is elevated. What is the first-line treatment for this paediatric patient with pre-symptomatic/stable disease?
Wilson disease: autosomal recessive copper metabolism disorder (ATP7B gene). First-line treatment: D-penicillamine (copper chelator) — with supplemental pyridoxine; or zinc (blocks intestinal copper absorption) for maintenance or as first-line in pre-symptomatic/mild cases. Liver transplant is reserved for acute liver failure or end-stage liver disease.
Wilson disease: low ceruloplasmin + Kayser-Fleischer rings + elevated 24-hour urine copper. Treatment: D-penicillamine + pyridoxine (first-line) or zinc (pre-symptomatic/maintenance). Liver transplant for FHF or end-stage. Autosomal recessive (ATP7B gene mutation).
Liver transplant is reserved for FHF or end-stage disease. Steroids have no role in Wilson disease. UDCA is used in cholestatic conditions (biliary atresia, Alagille, primary sclerosing cholangitis) but is not the treatment for Wilson disease.
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A 12-year-old with known chronic liver disease (Child-Pugh B cirrhosis from chronic HBV) develops sudden haematemesis. Upper GI endoscopy reveals large oesophageal varices. Which of the following statements about portal hypertension management is correct?
Endoscopic variceal ligation (EVL) is the preferred technique for acute oesophageal variceal bleeding (over sclerotherapy, which has more complications). It is also used for secondary prophylaxis. Non-selective beta-blockers (propranolol, carvedilol) are used for PRIMARY prophylaxis and secondary prevention (not acute haemorrhage).
Acute variceal bleed: vasoactive drugs (octreotide/terlipressin) + EVL (preferred over sclerotherapy) + antibiotics (norfloxacin/ceftriaxone). Beta-blockers: primary and secondary prophylaxis only. EHPVO (normal LFTs) vs cirrhotic portal HTN (abnormal LFTs) — distinguish the two.
Propranolol (beta-blocker) reduces portal pressure and is used for primary and secondary prophylaxis — NOT for acute bleeding (it does not arrest acute haemorrhage; vasoactive drugs like terlipressin/octreotide + EVL are used acutely). EHPVO is most common when LFTs are normal. Splenectomy does not treat portal hypertension — the portal venous pressure persists.
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A 6-year-old presents with recurrent jaundice, failure to thrive, abdominal distension, and xanthomas. Liver biopsy shows bile duct paucity. ALAT/ASAT are mildly elevated; GGT is very high; direct bilirubin predominant. Which investigation is most specific for evaluating biliary anatomy?
In a child with chronic cholestatic jaundice and suspected biliary abnormality (paucity, choledochal cyst, primary sclerosing cholangitis), MRCP is the non-invasive gold standard for evaluating biliary anatomy — showing bile duct structure without radiation or contrast injection.
MRCP is the non-invasive gold standard for paediatric biliary anatomy assessment (choledochal cysts, PSC, bile duct paucity). It replaces ERCP in many diagnostic situations. LFT interpretation: GGT elevation = cholestatic/biliary; ALT/AST elevation = hepatocellular.
Anti-SMA (smooth muscle antibody) is a serological marker for autoimmune hepatitis, not biliary anatomy. AFP is elevated in hepatoblastoma and hepatocellular carcinoma — not a biliary anatomy investigation. Stool colour chart is useful for early neonatal cholestasis screening (biliary atresia) but does not characterise anatomy in a 6-year-old.
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A 4-year-old child presents with jaundice, fever, and tender hepatomegaly. Serology reveals anti-HEV IgM positive. His mother is 32 weeks pregnant. She has not been immunized. Which statement about hepatitis E in this context is accurate?
Hepatitis E is a faeco-oral transmitted RNA virus causing acute self-limiting hepatitis. In pregnancy (especially 3rd trimester), HEV has a case-fatality rate of 20–30% due to fulminant hepatic failure — urgent evaluation of the mother is critical. HEV does not cause chronic hepatitis in immunocompetent individuals.
HEV: faeco-oral, acute self-limiting — but HIGHLY DANGEROUS in pregnancy (3rd trimester CFR 20–30%). Both HAV and HEV are faeco-oral. HCV and HBV are blood-borne. HEV vaccine not on India NIS.
HEV does not cause chronic hepatitis in immunocompetent persons (it can in immunocompromised). HEV is faeco-oral (like HAV), NOT blood-borne. HEV vaccine (Hecolin, China) exists but is NOT part of India's NIS.
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A 16-year-old boy presents with a 3-month history of dysphagia, haematemesis, and weight loss. Upper GI endoscopy is indicated. Which finding on endoscopy would be LEAST expected in a child this age with portal hypertension from EHPVO?
In EHPVO, the primary cause of haematemesis is variceal bleeding (oesophageal, gastric, duodenal varices), not H. pylori gastric ulcer. Portal hypertensive gastropathy is also seen. H. pylori gastric ulcer is NOT the expected finding in a child with established EHPVO and haematemesis.
Upper GI endoscopy indications in children: suspected varices/portal HTN, haematemesis, recurrent abdominal pain with suspected peptic disease, foreign body, eosinophilic oesophagitis. In EHPVO, haematemesis = variceal bleeding — endoscopy is both diagnostic and therapeutic (EVL).
Oesophageal varices (especially lower third), portal hypertensive gastropathy, and gastric/duodenal varices are all expected endoscopic findings in EHPVO with portal hypertension. Haematemesis in EHPVO is from variceal bleeding, not peptic ulcer disease as the primary cause.
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