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原位肝移植治愈甲型血友病。

Cure of hemophilia A by orthotopic liver transplantation.

作者信息

Gibas A, Dienstag J L, Schafer A I, Delmonico F, Bynum T E, Schooley R, Rubin R H, Cosimi A B

机构信息

Gastrointestinal Unit, Massachusetts General Hospital, Boston.

出版信息

Gastroenterology. 1988 Jul;95(1):192-4. doi: 10.1016/0016-5085(88)90310-1.

Abstract

A patient with hemophilia A and transfusion-associated end-stage chronic liver disease underwent orthotopic liver transplantation. He had no requirement for exogenous factor VIII replacement during the 27 mo he survived. Although his hemophilia was cured, he had antibodies to the human immunodeficiency virus; ultimately he died of complications arising from acquired immunodeficiency syndrome. Liver transplantation for cirrhotic hemophiliacs can free them of the need for antihemophilic-factor therapy; however, application of this approach may be limited by the high prevalence of human immunodeficiency virus infection in multitransfused hemophiliacs.

摘要

一名患有甲型血友病和输血相关终末期慢性肝病的患者接受了原位肝移植。在其存活的27个月里,他无需外源性凝血因子VIII替代治疗。尽管他的血友病得到了治愈,但他感染了人类免疫缺陷病毒;最终,他死于获得性免疫缺陷综合征引发的并发症。对肝硬化血友病患者进行肝移植可使其无需接受抗血友病因子治疗;然而,由于多次输血的血友病患者中人类免疫缺陷病毒感染的高患病率,这种方法的应用可能会受到限制。

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