Abstract

Clinical presentation and progression of Wilson’s disease can be diverse in different groups of patients. While young children most likely to present with acute or chronic liver failure, older children and adults may exhibit extrahepatic manifestation like neuropsychiatric, cardiac, renal, pancreatic and several others. Diagnosis of Wilson’s disease is based on the combination of specific clinical findings, laboratory and genetic testing. There are different modalities of treatment available for this condition. Liver transplantation has become a acceptable treatment option for acute and chronic liver failure related to Wilson’s disease. It allows for resolution of metabolic disorder and hepatic disease progression. There are no definitive criteria and established outcomes in transplantation of the patients with Wilson’s disease who have different degree of neuropsychiatric presentation. Therefore, it is very important to create specific criteria for selecting of these patients before transplantation and to have short-and long-term follow up system to improve their outcome and survival.

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