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抗磷脂综合征与心脏瓣膜手术

The antiphospholipid syndrome and heart valve surgery.

作者信息

Gorki Hagen, Malinovski Vitali, Stanbridge Rex D L

机构信息

Department of Cardiothoracic Surgery, St. Mary's Hospital, Praed Street, London W2 1NY, United Kingdom.

出版信息

Eur J Cardiothorac Surg. 2008 Feb;33(2):168-81. doi: 10.1016/j.ejcts.2007.11.004. Epub 2007 Dec 21.

Abstract

The antiphospholipid syndrome (APLS) is a complex autoimmune disease often connected to systemic lupus erythematodes. Main features are thromboses, fetal loss and specific antibodies. The involved autoantibodies are directed against plasma proteins such as beta2glycoprotein1 (beta2GPI) or prothrombin which depend on negatively charged phospholipids. Direct antibodies against phospholipids are of no importance for APLS. Clotting tests such as activated partial thromboplastin time or diluted Russell's viper venom test (dRVVT) can show a prolonged time for coagulation despite a prothrombotic state in vivo but the investigator needs awareness about disturbing phospholipid sources and other influential factors. Enzyme linked immuno sorbent assay tests for antibodies against cardiolipin, beta2GPI and prothrombin are valuable solid phase tests with different specificity. Antiphospholipid, anticardiolipin or lupus anticoagulant are misnomers in connection with APLS. They are preserved as a reminiscence of the pioneering work on the way to the still not exactly revealed basics of APLS. Valve operations in APLS patients seem to be rare; a meta-analysis of 57 cases proves that the perioperative management is, at the moment, an empirical approach with high morbidity and mortality in these young patients.

摘要

抗磷脂综合征(APLS)是一种复杂的自身免疫性疾病,常与系统性红斑狼疮相关。主要特征为血栓形成、胎儿丢失和特定抗体。所涉及的自身抗体针对的是诸如β2糖蛋白1(β2GPI)或凝血酶原等血浆蛋白,这些蛋白依赖于带负电荷的磷脂。直接针对磷脂的抗体对APLS并不重要。诸如活化部分凝血活酶时间或稀释蝰蛇毒时间(dRVVT)等凝血试验,尽管体内处于促血栓形成状态,但可能显示凝血时间延长,不过研究者需要了解干扰磷脂来源及其他影响因素。针对心磷脂、β2GPI和凝血酶原的抗体的酶联免疫吸附测定试验是具有不同特异性的有价值的固相试验。抗磷脂、抗心磷脂或狼疮抗凝物与APLS相关的这些名称并不准确。它们作为在尚未完全揭示的APLS基础研究道路上开创性工作的一种纪念而保留下来。APLS患者进行瓣膜手术似乎很少见;对57例病例的荟萃分析证明,目前围手术期管理是一种经验性方法,在这些年轻患者中具有高发病率和死亡率。

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