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一个存在血管纤溶酶原激活物释放缺陷及血浆因子VIII/血管性血友病因子升高的家族中的静脉血栓形成。

Venous thrombosis in a family with defective release of vascular plasminogen activator and elevated plasma factor VIII/von Willebrand's factor.

作者信息

Stead N W, Bauer K A, Kinney T R, Lewis J G, Campbell E E, Shifman M A, Rosenberg R D, Pizzo S V

出版信息

Am J Med. 1983 Jan;74(1):33-9. doi: 10.1016/0002-9343(83)91115-4.

DOI:10.1016/0002-9343(83)91115-4
PMID:6401391
Abstract

A family is described in which venous thrombosis developed in five members as early as 14 years of age. Routine coagulation studies, plasma antithrombin III, factor V, plasminogen, beta-thromboglobulin, fibrinopeptide A, prothrombin fragment F1+2, and thrombin-antithrombin III complex were all within normal limits. However, defective release of vascular plasminogen activator was observed on several occasions in all five subjects as compared with a control population of 125 persons (0.04 Committee on Thrombolytic Agents [CTA] units/ml plasma as compared with 0.21 CTS units/ml). In addition, levels of factor VII/von Willebrand's factor were significantly elevated above the normal range in this pedigree.

摘要

有一个家族,其中五名成员早在14岁时就出现了静脉血栓形成。常规凝血研究、血浆抗凝血酶III、因子V、纤溶酶原、β-血小板球蛋白、纤维蛋白肽A、凝血酶原片段F1+2以及凝血酶-抗凝血酶III复合物均在正常范围内。然而,与125人的对照人群相比,在所有五名受试者中均多次观察到血管纤溶酶原激活剂释放存在缺陷(血浆中为0.04溶栓剂委员会[CTA]单位/毫升,而对照为0.21 CTS单位/毫升)。此外,在这个家系中,因子VII/血管性血友病因子水平显著高于正常范围。

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1
Venous thrombosis in a family with defective release of vascular plasminogen activator and elevated plasma factor VIII/von Willebrand's factor.一个存在血管纤溶酶原激活物释放缺陷及血浆因子VIII/血管性血友病因子升高的家族中的静脉血栓形成。
Am J Med. 1983 Jan;74(1):33-9. doi: 10.1016/0002-9343(83)91115-4.
2
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Eur J Med. 1993 May;2(5):287-8.
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[Biological diagnosis of von Willebrand's disease (author's transl)].血管性血友病的生物学诊断(作者译)
Sangre (Barc). 1978;23(5B):728-45.
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Electrophoretic heterogeneity of normal factor VIII/Von Willebrand protein, and abnormal electrophoretic mobility in patients with Von Willebrand's disease.正常因子VIII/血管性血友病因子蛋白的电泳异质性,以及血管性血友病患者的异常电泳迁移率。
J Lab Clin Med. 1976 Feb;87(2):185-94.
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[Sugar-chain of factor VIII/von Willebrand factor in von Willebrand's disease. Analysis by immuno-affinoelectrophoresis using lectin].[血管性血友病中凝血因子VIII/血管性血友病因子的糖链。使用凝集素的免疫亲和电泳分析]
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Diagnosis of von Willebrand's disease. A comparative study of diagnostic tests on nine families with von Willebrand's disease and its differential diagnosis from hemophilia and thrombocytopathy.血管性血友病的诊断。对九个血管性血友病家族的诊断试验及其与血友病和血小板病的鉴别诊断的比较研究。
Am J Med. 1976 Mar;60(3):344-56. doi: 10.1016/0002-9343(76)90750-6.

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