A 30-year-old woman presents with acute hepatitis, neuropsychiatric symptoms, and a golden-brown ring around the corneal limbus on slit-lamp examination. Serum caeruloplasmin is 6 mg/dL (normal 20-40). What is the MOST appropriate long-term treatment?

• A Zinc supplementation alone
• B Liver transplantation immediately
• C Deferasirox
• D D-penicillamine or trientine (chelation therapy) ✓

Explanation

Wilson's disease presents with hepatic, neuropsychiatric, and Kayser-Fleischer ring features with low caeruloplasmin. First-line long-term treatment is copper chelation with D-penicillamine (monitoring for nephrotoxicity and lupus-like syndrome) or trientine (better tolerated). Zinc alone is used for maintenance phase or pre-symptomatic patients but not for initial treatment of symptomatic disease. Liver transplant is reserved for acute liver failure from Wilson's or end-stage disease. Deferasirox is an iron chelator, not copper.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.