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

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 NICE CG100 Seo 2006. Regular monitoring of liver function tests and copper levels is essential to assess treatment efficacy and adjust therapy accordingly NICE CG100. In cases of acute liver failure or decompensated cirrhosis, referral for liver transplantation should be considered as a definitive treatment option NICE CG100 Seo 2006. Supportive care addressing complications such as coagulopathy, encephalopathy, and portal hypertension should be provided in line with standard hepatic management protocols NICE CG100. Nutritional advice to avoid copper-rich foods complements pharmacological treatment NICE CG100. 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 NICE CG100 Seo 2006.