A 28-year-old woman presents with progressive jaundice, hepatosplenomegaly, and low serum ceruloplasmin (8 mg/dL, normal 20–40). Kayser-Fleischer rings are present on slit-lamp examination. Liver biopsy shows copper content 420 μg/g dry weight (normal <50). She has neuropsychiatric symptoms including dysarthria and dysphagia. The most appropriate long-term treatment that is ALSO recommended for neurologically symptomatic Wilson's disease as first-line is:
- A D-penicillamine
- B Zinc acetate
- C Liver transplantation immediately
- D Trientine ✓
Explanation
Wilson's disease management depends on clinical phenotype. For neurologically symptomatic Wilson's disease, trientine is currently preferred over D-penicillamine because D-penicillamine can paradoxically worsen neurological symptoms in up to 30% of patients due to acute copper mobilization, and this deterioration can be irreversible. Trientine is a copper chelator with fewer side effects and lower risk of neurological worsening. Zinc acetate interferes with copper absorption and is used for maintenance or pre-symptomatic disease but acts too slowly for active disease. Transplantation is reserved for acute liver failure.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.