Fulminant Wilson's Disease Requiring Liver Transplantation in One Monozygotic Twin Despite Identical Genetic Mutation

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...

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Veröffentlicht in:	American journal of transplantation 2010-05, Vol.10 (5), p.1325-1329
Hauptverfasser:	Kegley, K. M., Sellers, M. A., Ferber, M. J., Johnson, M. W., Joelson, D. W., Shrestha, R.
Format:	Artikel
Sprache:	eng
Schlagworte:	Acute Disease Adolescent Biological and medical sciences Chromosomes, Human, Pair 13 - metabolism Copper - metabolism Disease Progression End‐stage liver disease Female Gastroenterology. Liver. Pancreas. Abdomen Hepatolenticular Degeneration - genetics Hepatolenticular Degeneration - metabolism Hepatolenticular Degeneration - surgery Humans Liver - metabolism Liver - surgery Liver Diseases - genetics Liver Diseases - metabolism Liver Diseases - surgery liver failure Liver Failure, Acute - genetics Liver Failure, Acute - metabolism Liver Failure, Acute - surgery Liver Transplantation Liver, biliary tract, pancreas, portal circulation, spleen Liver. Biliary tract. Portal circulation. Exocrine pancreas Medical sciences Metabolic diseases Metals (hemochromatosis...) Mutation Other diseases. Semiology Other metabolic disorders Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Surgery of the digestive system twins Twins, Monozygotic - genetics Wilson's disease
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