A 22-year-old student presents with acute hepatitis (bilirubin 12 mg/dL, ALT 980 U/L), haemolytic anaemia (Hb 7.8 g/dL, negative Coombs test), kayser-Fleischer rings on slit lamp, serum caeruloplasmin 8 mg/dL, and 24-hour urinary copper 980 µg/day. Liver biopsy shows massive hepatic copper accumulation. The MOST dangerous pharmacological interaction to avoid in the acute management of Wilson's disease is:
- A D-penicillamine + pyridoxine — pyridoxine reverses the cupriuretic effect
- B D-penicillamine in acute Wilson's liver failure — can worsen neurological deterioration via rapid copper mobilisation ✓
- C Trientine + vitamin C — ascorbic acid oxidises trientine rendering it ineffective
- D Zinc acetate + lactulose — zinc competes with colonic pH change needed for chelation
Explanation
In acute Wilson's hepatic crisis (acute liver failure presentation), D-penicillamine is avoided because rapid cupriuresis can paradoxically worsen neurological status through mobilisation of copper to the brain. Standard AASLD/EASL guidance for acute Wilson's disease decompensation is trientine (safer chelator with less neurological side effects) or zinc acetate as a maintenance adjunct, with liver transplantation considered early for Wilson's acute liver failure (MELD/King's criteria apply). Pyridoxine supplementation is required with penicillamine (not to reverse its effect but because penicillamine depletes pyridoxine).
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.