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Wilson Disease Fulminant Hepatic Failure — RACP Adult Medicine MCQ

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HardGastroenterology & HepatologyWilson Disease Fulminant Hepatic FailureRACP Adult Medicine

A 35-year-old man presents with acute liver failure (INR 5.2, bilirubin 450 µmol/L, encephalopathy grade III). He was well 5 days ago. Hepatitis A IgM, hepatitis B surface antigen, HBV DNA, hepatitis C, and hepatitis E IgM are all negative. Paracetamol level is undetectable. Autoimmune markers are negative. Caeruloplasmin is very low at 0.05 g/L and Kayser-Fleischer rings are present. What is the definitive management?

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Correct answer: D — Urgent liver transplantation

Wilson disease presenting as fulminant hepatic failure has near-100% mortality without liver transplantation. Medical chelation therapy (penicillamine, trientine) is too slow to be effective in the acute setting. The combination of very low caeruloplasmin, Kayser-Fleischer rings, Coombs-negative haemolytic anaemia (often present), and acute liver failure in a young person is diagnostic. The New Wilson Index (NWI) score >11 predicts death without transplant with high sensitivity. Albumin dialysis (MARS/Prometheus) or plasma exchange may be used as a bridge to transplant.

Reference: eTG – 2025 – Gastrointestinal; EASL – 2012 – Wilson Disease Guidelines