A 32-year-old woman presents with acute liver failure, haemolytic anaemia with Coombs-negative haemolysis, and low serum caeruloplasmin. AST/ALT ratio is >4, alkaline phosphatase is very low. Slit-lamp examination shows Kayser-Fleischer rings. What is the most appropriate management?
- A Emergency liver transplantation evaluation as D-penicillamine is contraindicated in acute presentation ✓
- B D-penicillamine 1 g/day plus high-dose vitamin C
- C Trientine plus zinc acetate as outpatient therapy
- D Plasmapheresis followed by oral zinc acetate
Explanation
Acute liver failure in Wilson's disease (Wilson's hepatic crisis) carries a very high mortality and does not respond to chelation therapy in the acute phase — D-penicillamine can actually worsen the acute copper release. The New Wilson Index (score based on bilirubin, INR, AST, WBC, albumin) ≥11 predicts need for liver transplantation with high sensitivity. Acute presentation with fulminant hepatic failure, Coombs-negative haemolysis, very high bilirubin-to-ALP ratio, and normal/low ALP is characteristic of Wilson's hepatic crisis and requires emergency liver transplant evaluation.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.