📋 MOCK OSCE · FCPS, MCPS, MD PAEDIATRICS ⏱ 10 min · CHRONIC LIVER DISEASE

Chronic Liver Disease · Short Case

Candidate task: perform focused clinical examination (GPE + abdomen) on a child with suspected chronic liver disease.
Then discuss differential diagnosis, investigations, management & follow‑up.
Pre‑exam Protocol
⏤ · Wash, Warm, Introduce, Position, Expose, Approach ▾

Standard pre‑examination protocol – must be demonstrated:

🖐 Wash hands with sterilizing solution.
🔥 Warm hands and stethoscope.
👋 Introduce yourself to child & parent.
🧍 Position child: standing → sitting → supine.
👕 Exposure — shirt off, trousers rolled.
➡️ Approach from the right side.
✔ CPSP marker: Pre‑exam Protocol is observed and scored.
1. Clinical Examination (≈6 min)
02 General Look (Inspection from end of bed) ▾

Key observations:

  • Jaundice: scleral icterus, skin yellowing.
  • Nutritional status: muscle wasting, subcutaneous fat loss, edema.
  • Dysmorphic features: Alagille syndrome (triangular facies, deep-set eyes), Zellweger.
  • Abdominal contour: distension (ascites), prominent veins (caput medusae).
  • Skin: bruising, spider naevi, scratch marks (pruritus), xanthomas.
  • Mental state: encephalopathy (drowsy, confused, asterixis).
👁 Red flags: scleral icterus + spider naevi + bruising → chronic liver disease.
03 General Physical Exam (Hands → Face → Chest → Limbs) ▾

Systematic examination:

  • Hands: clubbing, leuconychia, palmar erythema, xanthomas, asterixis.
  • Face: Cushingoid facies (steroids), Kayser-Fleischer rings (Wilson disease), conjunctival pallor (anaemia).
  • Chest: sternal deformity (rickets), rib rosary (vitamin D deficiency), Harrison’s sulcus.
  • Abdomen: see next step.
  • Lower limbs: ankle oedema, erythema nodosum, joint swelling (IBD).
📏 Anthropometry: weight, height, BMI, head circumference – plot growth.
04 Abdominal Examination ▾

Systematic approach:

  • Inspection: distension, visible veins (caput medusae), scars (Kasai, liver transplant), stoma.
  • Auscultation: bowel sounds (normal/hyperactive), bruits (hepatic – hepatoma), venous hum.
  • Palpation: hepatomegaly (firm/hard), splenomegaly (portal hypertension), ascites (fluid thrill).
  • Percussion: liver span, shifting dullness (ascites).
  • Rectal: if indicated – haemorrhoids (portal hypertension).
🔍 Key: hepatomegaly + splenomegaly + ascites → portal hypertension.
05 Developmental Assessment & Associated Signs ▾

Assess:

  • Neurological: developmental delay, hypotonia (Zellweger), ataxia (vitamin E deficiency), peripheral neuropathy.
  • Eyes: Kayser-Fleischer rings (Wilson), cataracts (steroids, galactosaemia), xerophthalmia (vitamin A).
  • Oral: aphthous ulcers (IBD), enamel hypoplasia (celiac).
  • Growth: short stature, delayed puberty (chronic liver disease).
🧠 Wilson disease: K-F rings + neuropsychiatric symptoms + liver disease.

📋 Case Presentation – (fill in during exam)

This is a _____-year-old _____ child, brought with _____. On examination, the child appears _____ (well/unwell), with _____ (jaundice / pallor / oedema). There is _____ (hepatomegaly / splenomegaly / ascites). Growth parameters: weight _____ percentile, height _____ percentile. Additional findings: _____ (spider naevi / clubbing / K-F rings).

2. Viva Discussion (≈4 min)
06 Viva · Differential, Investigations, Management, Follow‑up ▾
🔹 Differential Diagnosis

• Biliary atresia (neonatal cholestasis)
• α₁-Antitrypsin deficiency (PiZZ)
• Alagille syndrome (paucity of bile ducts)
• Wilson disease (copper accumulation)
• Autoimmune hepatitis (type 1/2)
• Chronic viral hepatitis (B, C)
• NASH / NAFLD (obesity, metabolic syndrome)
• Progressive familial intrahepatic cholestasis (PFIC)
• Cystic fibrosis liver disease
• Glycogen storage disease (types I, III, IV)

🔹 Investigations – Diagnosis

• LFTs: AST, ALT, ALP, GGT, bilirubin (conj/unconj)
• Synthetic function: albumin, PT/INR
• Viral serology: HAV IgM, HBsAg, anti-HCV
• Autoimmune: ANA, SMA, anti-LKM-1, IgG
• Metabolic: ceruloplasmin (Wilson), α₁-AT phenotype
• Imaging: abdominal ultrasound, MRCP, liver stiffness (FibroScan)

🔹 Investigations – Aetiology

• Wilson: serum copper, 24h urinary copper, K-F rings
• α₁-AT: Pi phenotype (PiZZ)
• PFIC: serum bile acids, GGT (low in PFIC 1 & 2)
• Alagille: JAG1 mutation, butterfly vertebrae
• NASH: fasting glucose, lipid profile, BMI
• Biliary atresia: intraoperative cholangiogram

🔹 Investigations – Exclude Others

• Infections: CMV, EBV, HSV, HIV, toxoplasmosis
• Drug-induced: acetaminophen level, toxicology screen
• Metabolic: galactosaemia, tyrosinaemia, HFI (urine reducing substances)
• Haematologic: CBC, Coombs test (haemolysis)
• Malignancy: AFP (hepatoblastoma), imaging

🔹 Investigations – Rule Out Complications

• Portal hypertension: oesophagogastroduodenoscopy (varices)
• Ascites: diagnostic paracentesis (SAAG, culture)
• Encephalopathy: ammonia, EEG
• Coagulopathy: PT/INR, vitamin K response
• Hepatocellular carcinoma: AFP, ultrasound/MRI
• Renal: creatinine, urea (hepatorenal syndrome)

🔹 Management – Across Organ Systems

Nutrition

High-calorie (120–150% RDA), MCT oil, fat-soluble vitamins (A, D, E, K), branched-chain amino acids, nocturnal NG feeds.

Ascites

Salt restriction (<2 mmol/kg/day), spironolactone, furosemide, albumin, therapeutic paracentesis if tense.

Encephalopathy

Lactulose, rifaximin, protein restriction (2 g/kg), branch-chain amino acids, treat precipitating factors (infection, bleeding).

Portal hypertension / Varices

Propranolol (non-selective β-blocker), endoscopic band ligation/sclerotherapy, TIPSS if refractory.

Coagulopathy

Vitamin K (IV/IM), FFP/cryoprecipitate for active bleeding, platelet transfusion if hypersplenism.

Pruritus

Ursodeoxycholic acid (10–15 mg/kg/day), rifampicin, cholestyramine, ondansetron, naltrexone.

Infection

Prophylactic antibiotics (cholangitis), spontaneous bacterial peritonitis (SBP) – 3rd gen cephalosporin + albumin.

Liver Transplantation

Consider for end-stage liver disease, intractable pruritus, metabolic disease (Wilson, tyrosinaemia), HCC.

📈 Prognosis

  • Biliary atresia: 5-year native liver survival ~30–50% after Kasai; post-transplant ~80–90%.
  • Wilson disease: excellent if chelation started early; liver failure requires transplantation.
  • α₁-AT: 10–15% develop cirrhosis; transplantation curative.
  • Autoimmune hepatitis: good response to steroids; ~20% progress to cirrhosis.
  • PFIC: progressive; many require biliary diversion or transplantation.

📋 Follow‑up Schedule

  • Monthly: weight, LFTs, PT/INR, albumin (in unstable patients).
  • 3–6 monthly: growth, fat-soluble vitamins, renal function.
  • Annually: ultrasound + AFP (HCC surveillance), bone density (if on steroids).
  • Endoscopy: variceal screening at diagnosis and every 1–2 years.
💡 Examiner expectation: logical differential, systematic investigation (diagnosis → aetiology → exclude others → complications), and a management plan covering all systems. Know the prognosis and follow-up schedule for common aetiologies.
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Mock OSCE · Chronic Liver Disease · Based on Wyne‑Harris, Nelson & Pediatric Clinical Advisor