Article
Fulminant Wilson's disease requiring liver transplantation in one monozygotic twin despite identical genetic mutation.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons - 1 May 2010
Kegley K M, Sellers M A, Ferber M J, Johnson M W, Joelson D W, Shrestha R
Abstract excerpt
Acute decompensated Wilson's disease (WD) that presents as fulminant hepatic failure carries significant mortality without hepatic replacement. The abnormal gene implicated in WD, ATP7B, has been mapped to chromosome 13, and leads to decreased passage of copper from hepatocytes to bile. Excess copper accumulation exceeds hepatocyte storage capacity resulting in intracellular necrosis, apoptosis and cell death in...
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