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肝豆状核变性的肝移植

Liver transplantation for Wilson disease.

作者信息

Ahmad Ahsan, Torrazza-Perez Euriko, Schilsky Michael L

机构信息

Section of Transplantation and Immunology, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of New Mexico, Albuquerque, USA.

出版信息

Handb Clin Neurol. 2017;142:193-204. doi: 10.1016/B978-0-444-63625-6.00016-1.

DOI:10.1016/B978-0-444-63625-6.00016-1
PMID:28433103
Abstract

Liver transplantation (LT) is a life-saving and curative treatment for Wilson disease (WD), providing restoration of function of the liver and mitigation of portal hypertension. Indications for LT in patients with WD include acute liver failure or end-stage liver disease not treatable by medical therapy. LT is also used to treat hepatocellular carcinoma when it develops in patients with WD when tumor resection is not feasible. LT solely for neurologic or psychiatric WD remains controversial. Living liver donation as well as cadaveric orthotopic and auxiliary LT are options for transplantation for WD. Outcomes for LT for WD are excellent, and supportive measures while awaiting transplantation help bridge the patient to a more successful outcome. Future hepatocyte or stem cell transplantation may augment or supplant current LT for WD.

摘要

肝移植(LT)是治疗威尔逊病(WD)的一种挽救生命且具有治愈性的疗法,可恢复肝功能并减轻门静脉高压。WD患者进行LT的适应证包括急性肝衰竭或药物治疗无法治愈的终末期肝病。当WD患者发生肝细胞癌且无法进行肿瘤切除时,也可采用LT进行治疗。单纯为治疗神经或精神性WD而进行的LT仍存在争议。活体肝捐赠以及尸体原位和辅助性LT都是WD移植的选择。WD患者LT的预后良好,等待移植期间的支持性措施有助于患者获得更成功的预后。未来的肝细胞或干细胞移植可能会增强或取代目前用于WD的LT。

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