A 28-year-old woman presents with chronic hepatitis, haemolytic anaemia (Coombs-negative), psychiatric symptoms (personality change), and Kayser-Fleischer rings on slit-lamp examination. Serum ceruloplasmin is 8 mg/dL (normal 20–60). The initial treatment of choice for this condition is:

• A D-penicillamine (copper chelation) ✓
• B Zinc sulphate supplementation
• C Trientine plus zinc as combination first-line
• D Liver transplantation as immediate cure

Explanation

Wilson's disease (autosomal recessive ATP7B copper transporter defect) with symptomatic neurological, psychiatric, and hepatic involvement requires initial copper chelation therapy. D-penicillamine is the traditional first-line agent. Trientine is an alternative chelator with fewer side effects and preferred by many centres. Zinc maintenance therapy reduces copper absorption and is used for maintenance or in asymptomatic patients. Liver transplantation is curative but 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.