A 24-year-old woman has elevated serum copper (225 μg/dL), low caeruloplasmin (8 mg/dL), 24-hour urine copper 280 μg/day, Kayser-Fleischer rings, and elevated liver enzymes. Liver biopsy shows copper deposition > 250 μg/g dry weight. The diagnosis is Wilson's disease. Which statement about its treatment is MOST accurate?
- A Penicillamine is the preferred first-line agent for all presentations including neurological
- B Trientine (triethylene tetramine) is preferred over penicillamine for neurological Wilson's disease as penicillamine may worsen neurological symptoms initially ✓
- C Zinc salts are the preferred first-line agent for acute liver failure due to Wilson's disease
- D Once KF rings disappear, treatment can be discontinued safely
Explanation
Penicillamine is a copper chelator effective in Wilson's disease but has a significant risk of paradoxical neurological deterioration in patients with neurological presentations (in up to 50% of cases), possibly due to mobilisation of copper from liver to brain. Trientine (triethylene tetramine) is equally effective but associated with significantly lower risk of neurological worsening, making it preferred for neurological Wilson's disease. Zinc acetate is used for maintenance and in presymptomatic patients. Acute liver failure from Wilson's requires liver transplantation, not chelation alone.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.