A 25-year-old woman presents with acute liver failure, haemolytic anaemia, and psychiatric symptoms. Kayser-Fleischer rings are absent on slit-lamp examination. Serum ceruloplasmin is 8 mg/dL (normal 20–60). The MOST appropriate first-line treatment for this presentation is:
- A D-penicillamine
- B Zinc acetate alone
- C Trientine (triethylenetetramine) with or without liver transplantation evaluation ✓
- D BAL (dimercaprol) chelation therapy
Explanation
Wilson's disease presenting with acute liver failure, Coombs-negative haemolytic anaemia, and psychiatric features is an emergency. Note that KF rings may be absent in 50% of hepatic presentations. Trientine is preferred over D-penicillamine for acute presentation due to better tolerability and rapid copper chelation; liver transplantation is often indicated for fulminant hepatic failure from Wilson's disease. Zinc prevents copper absorption and is used for maintenance/presymptomatic patients but is too slow for acute presentation. BAL is used for arsenic/mercury poisoning.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.