Emergent Living Donor Liver Transplantation for Fulminant Hepatic Failure Secondary to Wilson’s Disease

Emergent living donor liver transplantation in adults with fulminant hepatic failure secondary to Wilson's disease is rarely performed.( )We report a case of decompensated Wilson's disease presenting with fulminant hepatic failure treated with an emergent living donor liver transplant. A 25-year-old female presented with fulminant hepatic failure and underwent an emergent living donor liver transplant using a left-lobe graft from her brother. Explant revealed a nodular, cirrhotic liver with numerous yellow-green nodules on the cut surface, and histopathology revealed confluent necrosis and cholestasis with positive copper immunostain. Quantitative hepatic copper was 2119 mcg/g (range: 10−35 mcg/g). Recipient genetic testing revealed c.2930C>T p. (Thr977Met) homozygous variant in the ATP7B gene. The donor was heterozygous for the mutation. The recipient continues to do well three years later with normal ceruloplasmin and urinary copper excretion.