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Wilson Disease ALF Diagnosis — RACP Adult Medicine MCQ

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HardGastroenterology & HepatologyWilson Disease ALF DiagnosisRACP Adult Medicine

A 55-year-old man with known Wilson disease presents with acute liver failure (INR 6.0, bilirubin 400 µmol/L). His Coombs-negative haemolytic anaemia has Hb 65 g/L. Serum copper is paradoxically elevated (released from necrotic hepatocytes). What ratio helps distinguish Wilson's from other causes of acute liver failure?

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Correct answer: EINR:platelet ratio

In acute liver failure, a disproportionately elevated bilirubin relative to a low/normal ALP (bilirubin [µmol/L] ÷ ALP [U/L] ratio >2.0 when ALP is <600 U/L) is characteristic of Wilson disease. The low ALP is thought to reflect zinc-mediated inhibition of ALP activity. Additionally, the AST:ALT ratio is typically >2.2 in acute Wilsonian hepatitis. The New Wilson Index (NWI) incorporating bilirubin, INR, AST, WCC, and albumin predicts need for transplant (score >11 = transplant needed). Coombs-negative haemolytic anaemia from copper-mediated erythrocyte damage is another diagnostic clue.

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