RECURRENT VENOUS THROMBOEMBOLISM IN PATIENTS WITH A PARTIAL DEFICIENCY OF PROTEIN-S

RECURRENT VENOUS THROMBOEMBOLISM IN PATIENTS WITH A PARTIAL DEFICIENCY OF PROTEIN-S
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DOI:
10.1056/nejm198412133112401
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发表时间:
1984-01-01
影响因子:
158.5
通讯作者:
ESMON, CT
ESMON, CT
中科院分区:
医学1区
文献类型:
--
作者:
COMP, PC;ESMON, CT

文献摘要

被引文献

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蛋白S是一种抗血栓血浆蛋白,作为另一种血浆蛋白活化蛋白C的辅助因子。蛋白S是活化蛋白C抗凝作用的必需蛋白,在凝血级联中,活化蛋白C的抗凝作用在V和VIII因子水平上抑制血液凝固。假设蛋白S缺乏的患者对凝血级联的调节控制不足,并且与先天性蛋白c缺乏的患者相似,容易发生血栓形成性疾病。为了确定蛋白S缺乏是否与复发性血栓形成有关,开发了血浆蛋白的功能测定。正常人的蛋白S活性在63-160%之间。使用该试验,鉴定了6例无血缘关系的严重复发性静脉血栓患者,他们在未接受华法林治疗时蛋白S缺乏,水平在15-37%之间。数据表明,蛋白S水平的测定将有助于评估复发性血栓患者。
Protein S is an antithrombotic plasma protein that serves as a cofactor for another plasma protein, activated protein C. Protein S is required for the expression of the anticoagulant effect of activated protein C, which inhibits blood clotting at the levels of factors V and VIII in the blood-clotting cascade. It was postulated that patients deficient in protein S would have inadequate regulatory control of the clotting cascade and would be prone to thrombotic disease in a manner similar to that of patients congenitally deficient in protein C. To determine whether protein S deficiency is associated with recurrent thrombosis, a functional assay for the plasma protein was developed. With this assay, the protein S activity of normal persons ranges from 63-160%. Using this test, 6 unrelated persons with severe recurrent venous thrombosis who were deficient in protein S were identified, with levels between 15-37% while they were not receiving warfarin therapy. Data suggest that the determination of protein S levels will be useful in the evaluation of patients with recurrent thrombosis.