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Wilson disease — RCPSC IM MCQ

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HardGastroenterology/HepatologyWilson diseaseRCPSC IM

A 25-year-old with Wilson disease has decompensated cirrhosis, coagulopathy, hemolysis and rapidly worsening encephalopathy despite medical therapy. What is the best next step?

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Correct answer: DArrange emergency liver-transplant assessment while providing intensive supportive care

The best answer is “Arrange emergency liver-transplant assessment while providing intensive supportive care”. Acute or rapidly decompensating Wilson-related liver failure can be fatal without urgent transplantation. Chelation is important in stable disease but should not delay transplant assessment in fulminant deterioration. A prognostic index, trajectory and extrahepatic complications inform urgency, but referral should occur before irreversible multiorgan failure develops.

Reference: Canadian Association for the Study of the Liver: https://hepatology.ca/