A 22-year-old woman presents with jaundice, hemolytic anemia (Coombs negative), and behavioral changes. Slit-lamp examination reveals golden-brown rings at the corneal periphery. Serum ceruloplasmin is 6 mg/dL (normal 20–40). The FIRST-LINE treatment for symptomatic Wilson's disease is:
- A Zinc acetate 50 mg three times daily
- B Tetrathiomolybdate
- C D-penicillamine ✓
- D Liver transplantation
Explanation
D-penicillamine is the first-line chelation therapy for symptomatic Wilson's disease, promoting urinary copper excretion. It is taken on an empty stomach and pyridoxine supplementation is required. Zinc acetate blocks intestinal copper absorption and is used for maintenance therapy or asymptomatic patients. Tetrathiomolybdate is used experimentally for neurological Wilson's disease. Liver transplantation is reserved for acute liver failure or decompensated cirrhosis not responding 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.