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How should I manage a patient diagnosed with Wilson's Disease who presents with hepatic symptoms?

Answer

Guideline-Aligned (High Confidence)
Generated by iatroX. Developer: Dr Kola Tytler MBBS CertHE MBA MRCGP (General Practitioner).
Last reviewed: 16 August 2025

Management of a patient with Wilson's disease presenting with hepatic symptoms involves prompt initiation of chelation therapy to reduce copper accumulation, which is the underlying cause of liver damage. First-line treatment typically includes agents such as penicillamine or trientine, which bind copper and promote its excretion, thereby improving hepatic function and preventing progression to cirrhosis or liver failure 1 (Seo, 2006). Regular monitoring of liver function tests and copper levels is essential to assess treatment efficacy and adjust therapy accordingly 1. In cases of acute liver failure or decompensated cirrhosis, referral for liver transplantation should be considered as a definitive treatment option 1 (Seo, 2006). Supportive care addressing complications such as coagulopathy, encephalopathy, and portal hypertension should be provided in line with standard hepatic management protocols 1. Nutritional advice to avoid copper-rich foods complements pharmacological treatment 1. Recent literature emphasizes the importance of early diagnosis and individualized treatment plans to improve long-term outcomes, highlighting that newer chelators and zinc therapy may be beneficial in certain patient subsets, although penicillamine remains the mainstay [Seo, 2006]. Overall, management requires a multidisciplinary approach involving hepatologists, neurologists, and dietitians to optimize care 1 (Seo, 2006).

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This content was generated by iatroX. Always verify information and use clinical judgment.