A 30-year-old man has decompensated liver disease with Kayser-Fleischer rings and serum ceruloplasmin 8 mg/dL (normal 20-60). He has haemolytic anaemia and psychiatric symptoms. He is diagnosed with Wilson's disease. MELD score is 28. Which statement about treatment is MOST accurate?

• A D-penicillamine is the first-line treatment and should be started immediately at full dose
• B Trientine is preferred over D-penicillamine in acute decompensation due to better tolerability
• C Liver transplantation is the treatment of choice for Wilson's disease with acute liver failure (ALF) or MELD >25 ✓
• D Zinc acetate is equally effective to chelation therapy in acute decompensation

Explanation

Wilson's disease presenting as acute liver failure (particularly with haemolysis, low ALP, and high bilirubin) or with MELD >25 has extremely poor prognosis with medical management. Liver transplantation is curative and indicated for ALF or severe decompensation unresponsive to medical therapy. D-penicillamine, while first-line for stable/early disease, carries risk of initial paradoxical worsening due to copper mobilisation and neurological deterioration. Trientine is preferred in acute decompensation (better tolerated), but neither chelator is adequate for ALF. Zinc blocks copper absorption but has a slow onset (months) — not suitable for acute scenarios. Wilson's Index (combining bilirubin, INR, AST, WBC, albumin) predicts need for transplant.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.