A 32-year-old man with cirrhosis presents with confusion, asterixis, and jaundice. Serum ceruloplasmin is 9 mg/dL (normal 20–40). Kayser-Fleischer rings are present. 24-hour urine copper is 680 µg/day (normal <100). The treatment of choice for this acute presentation of Wilson disease with neurological involvement is:
- A D-penicillamine
- B Zinc acetate monotherapy
- C Liver transplantation
- D Trientine plus zinc ✓
Explanation
Trientine (triethylene tetramine) is preferred over D-penicillamine in neurological Wilson disease because D-penicillamine causes acute worsening of neurological symptoms in up to 50% of cases (paradoxical neurological deterioration) due to rapid copper mobilization. Trientine combined with zinc (to reduce copper absorption) is the recommended combination for neurological Wilson disease. Zinc monotherapy is adequate for pre-symptomatic cases or maintenance. Liver transplantation is reserved for acute liver failure or decompensated cirrhosis unresponsive to medical therapy.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.