Wilson Disease — RACP Adult Medicine MCQ

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HardGeneral Internal MedicineWilson DiseaseRACP Adult Medicine

A 48-year-old man with known Wilson disease on penicillamine presents with worsening neurological symptoms. His 24-hour urine copper is very elevated. What alternative treatment should be considered?

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Correct answer: E — Trientine

In Wilson disease, if penicillamine causes worsening neurological symptoms (which occurs in 10-50% of neurological Wilson disease patients), trientine (triethylene tetramine) is the preferred alternative chelator as it has a lower risk of neurological deterioration. Zinc is used for maintenance therapy and in presymptomatic patients. Tetrathiomolybdate is investigational. Liver transplantation is reserved for fulminant hepatic failure or decompensated cirrhosis unresponsive to medical therapy.

Reference: eTG – 2025 – Gastrointestinal; EASL – 2012 – Wilson Disease Guidelines