Liver transplantation for Wilson's disease

ML Schilsky - Annals of the New York Academy of Sciences, 2014 - Wiley Online Library
ML Schilsky
Annals of the New York Academy of Sciences, 2014Wiley Online Library
Although Wilsons's disease (WD) may be treated with copper chelation (to remove copper)
or zinc salts (to prevent absorption) to alleviate or prevent symptom development in most
patients, there are WD patients for whom medical therapy is inadequate and survival would
be unlikely without liver transplantation. Liver transplantation is indicated for the∼ 5% of WD
patients with acute liver failure as the first presentation of disease, most commonly in the
second decade of life, or those who present with end‐stage liver disease and severe hepatic …
Although Wilsons's disease (WD) may be treated with copper chelation (to remove copper) or zinc salts (to prevent absorption) to alleviate or prevent symptom development in most patients, there are WD patients for whom medical therapy is inadequate and survival would be unlikely without liver transplantation. Liver transplantation is indicated for the ∼5% of WD patients with acute liver failure as the first presentation of disease, most commonly in the second decade of life, or those who present with end‐stage liver disease and severe hepatic insufficiency, most commonly in the third and fourth decades. Liver transplantation restores normal biliary copper excretion (thereby preventing disease recurrence) and promotes removal of copper from extrahepatic sites. Outcomes of liver transplantation for WD are excellent, including both cadaveric and living donors.
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