A 29-year-old woman presents with acute liver failure, Coombs-negative haemolytic anaemia, neuropsychiatric symptoms, and serum ceruloplasmin 12 mg/dL. Slit-lamp examination shows Kayser-Fleischer rings. The MOST appropriate immediate pharmacological treatment is:
- A D-penicillamine 1 g/day
- B Zinc acetate 50 mg three times daily
- C Trientine 750 mg twice daily ✓
- D Emergency liver transplantation listing only; chelation is contraindicated in acute liver failure
Explanation
Wilson's disease presenting as acute liver failure (ALF) requires urgent listing for liver transplantation as medical management alone has poor prognosis in this setting. However, pharmacological chelation can still be initiated as a bridge. Trientine is preferred over D-penicillamine for acute Wilson's disease because penicillamine can exacerbate neurological symptoms due to rapid copper mobilisation and has significant side effects. In ALF, the New Wilson's Index (scores based on bilirubin, INR, AST, WBC, albumin) predicts need for transplant. Zinc maintenance therapy is not appropriate for acute presentation.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.