Liver transplantation for Wilson disease.

摘要: The aim of this paper is to review the current status liver transplantation (LT) for Wilson disease (WD), focusing on indications and controversies, especially in patients with neuropsychiatric disease, identification acute failure (ALF) cases related WD. LT remains treatment choice ALF, as initial presentation WD or when anti-copper agents are stopped, chronic progressed cirrhosis, unresponsive chelating medications not timely treated copper agents. indication highly debated progressive neurological deterioration improve appropriate medical treatment. In case Wilsonian early key mortality 100% without emergency LT. As many metabolism parameters believed be less reliable simple biochemical tests have been proposed diagnosis good sensitivity specificity. corrects complications resulting from excellent 1 5 year survival. Living represents an alternative deceased donor long-term survival, recurrence. Future options may include hepatocyte gene therapy. Although both these shown promising results animal models WD, prospective human studies much needed demonstrate their beneficial effects potential replace need therapy