A 28-year-old woman presents with hemolytic anemia, acute liver failure, Kayser-Fleischer rings on slit-lamp examination, and serum ceruloplasmin of 8 mg/dL (normal 20–60). Urine copper is 450 µg/24h. She is in acute hepatic failure with encephalopathy. The treatment approach for this acute severe Wilson's disease presentation is:
- A D-penicillamine 1g/day plus pyridoxine
- B Zinc acetate 50 mg thrice daily as monotherapy
- C Trientine (triethylenetetramine) plus liver transplantation evaluation ✓
- D Dimercaprol (BAL) intramuscularly for copper chelation
Explanation
Acute fulminant Wilson's disease with hepatic failure and hemolytic anemia requires urgent liver transplantation evaluation, as acute liver failure from Wilson's disease has very high mortality without transplantation. Trientine is preferred over D-penicillamine in acute presentations because it is better tolerated and less likely to cause initial clinical worsening (D-penicillamine can precipitate neurological deterioration). The Wilson Disease Severity Index (Nazer score) or MELD score guides transplantation urgency. Zinc acetate has no role in acute presentation (it prevents absorption but cannot rapidly chelate tissue copper). Dimercaprol is used for acute heavy metal poisoning but not specifically for Wilson's disease management.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.