Q3
What is the role of liver function tests in evaluating cirrhosis?
✅ Model Answer:
• Synthetic function:
- Albumin: Low (due to decreased synthesis, normal 3.5-5.0 g/dL)
- INR/PT: Elevated (prolonged clotting time due to decreased synthesis of clotting factors)
- Total protein: Low (decreased synthesis)
• Hepatocellular injury:
- AST/ALT: Elevated (hepatocyte damage), may be normal in end-stage cirrhosis
- GGT: Elevated (cholestasis)
• Cholestasis:
- Total and Direct Bilirubin: Elevated (impaired excretion)
- Alkaline phosphatase: Elevated (bile duct obstruction)
• Severity scoring: Child-Pugh and MELD scores use albumin, INR, bilirubin, ascites, encephalopathy
Q4
What is the management of ascites in a child with cirrhosis?
✅ Model Answer:
• Dietary management: Sodium restriction (<2 mEq/kg/day, or <2 g/day)
• Fluid restriction: If hyponatremia (Na <125 mEq/L)
• Diuretics:
- Spironolactone: First-line (1-3 mg/kg/day) – potassium-sparing diuretic
- Furosemide: Add if inadequate response (1-2 mg/kg/dose)
- Monitor: Weight, urine output, electrolytes (K+, Na+)
• Paracentesis: For tense ascites causing respiratory distress
• IV Albumin: If severe hypoalbuminemia (albumin <2.0 g/dL) after large-volume paracentesis
• Treat underlying cause: Liver transplantation if refractory ascites
Q5
What are the complications of cirrhosis in children?
✅ Model Answer:
• Portal hypertension: Variceal bleeding (esophageal/gastric varices), splenomegaly, hypersplenism
• Ascites: Fluid accumulation in peritoneal cavity
• Spontaneous Bacterial Peritonitis (SBP): Infection of ascitic fluid (fever, abdominal pain, leukocytosis)
• Hepatic encephalopathy: Confusion, asterixis, somnolence (due to ammonia accumulation)
• Coagulopathy: Bleeding tendency (elevated INR)
• Malnutrition: Poor weight gain, muscle wasting (due to poor intake, malabsorption)
• Jaundice: Hyperbilirubinemia
• Hepatopulmonary syndrome: Hypoxemia due to intrapulmonary shunting
• Portopulmonary hypertension: Pulmonary hypertension
• Hepatocellular carcinoma (HCC): Rare in children but possible
Q6
What is the role of liver biopsy in pediatric liver disease?
Q7
What is the management of variceal bleeding in children with cirrhosis?
✅ Model Answer:
• Acute bleeding management:
- ABCs: Airway, breathing, circulation, IV access
- Fluid resuscitation: Blood products (PRBCs, platelets, FFP), IV fluids
- Somatostatin/Octreotide: Reduce portal pressure (bolus + infusion)
- Proton pump inhibitors (PPI): To reduce gastric acid
- Endoscopy: Diagnostic and therapeutic (band ligation or sclerotherapy)
- Antibiotics: Prophylactic (ceftriaxone) to prevent SBP
• Secondary prophylaxis: Non-selective beta-blockers (propranolol), repeated variceal banding
• Refractory bleeding: TIPS (transjugular intrahepatic portosystemic shunt) or liver transplantation
Q8
What is the prognosis and role of liver transplantation in pediatric cirrhosis?
✅ Model Answer:
• Prognosis: Depends on underlying cause and complications
- Biliary atresia: 80-90% survival at 5 years with successful Kasai procedure; otherwise liver transplant
- Autoimmune hepatitis: Good response to immunosuppression
- Wilson disease: Good with chelation therapy
- Alpha-1 antitrypsin: Variable, often requires transplant
• Indications for liver transplantation:
- End-stage liver disease (Child-Pugh class C, MELD score >15)
- Refractory ascites, variceal bleeding
- Hepatic encephalopathy
- Hepatopulmonary syndrome
- Hepatocellular carcinoma (within Milan criteria)
- Metabolic liver disease with systemic effects
• Prognosis after transplant: 85-90% 5-year survival in children
• Post-transplant: Immunosuppression, regular follow-up, monitoring for rejection and complications
⚠️ Key Concept: Cirrhosis in Children
• Clinical triad: Edema + Ascites + Jaundice → chronic liver failure.
• Common causes: Biliary atresia, metabolic diseases (Wilson, α1-AT), autoimmune hepatitis.
• Diagnosis: Low albumin, elevated INR, elevated bilirubin, nodular liver on US.
• Management: Spironolactone + furosemide for ascites, Vitamin K for coagulopathy.
• Complications: Variceal bleeding, SBP, hepatic encephalopathy.
• Transplantation: 85-90% 5-year survival.