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与血栓形成相关的血液蛋白质缺陷。实验室评估。

Blood protein defects associated with thrombosis. Laboratory assessment.

作者信息

Bick R L, Ancypa D

机构信息

Presbyterian Hospital of Dallas, University of Texas, Southwestern Medical Center, Dallas, USA.

出版信息

Clin Lab Med. 1995 Mar;15(1):125-63.

PMID:7781275
Abstract

This review has stressed the common hereditary and acquired blood protein defects associated with thrombosis. The most common of the hereditary defects appear to be antithrombin, protein C, and protein S deficiency, and the most common acquired defects are anticardiolipin antibodies and the lupus anticoagulant. Therefore, these are the defects which should first be searched for in an individual with unexplained thrombosis. If these more common defects are not found, the rarer defects, including HC-II, plasminogen, or TPA deficiency, dysfibrinogenemia, elevated PAI-1, or heterozygous homocystinemia should be looked for. The incidence of activated protein C co-factor deficiency (APC resistance) is not yet clear but may also represent a common defect. PAI-1 defects may, with time, be shown to be common. Finding these defects has important implications for therapy for the individual patient and for the institution of family studies to identify, inform, and possibly treat others at risk. It is expected that as knowledge of hemostasis expands, more hereditary and acquired defects, such as elevated lipoprotein(a) or defects of extrinsic (tissue factor) pathway inhibitor (EPI, TFPI), may be associated with enhanced risks for thrombosis.

摘要

本综述着重介绍了与血栓形成相关的常见遗传性和获得性血液蛋白质缺陷。最常见的遗传性缺陷似乎是抗凝血酶、蛋白C和蛋白S缺乏,而最常见的获得性缺陷是抗心磷脂抗体和狼疮抗凝物。因此,对于不明原因血栓形成的个体,应首先排查这些缺陷。如果未发现这些较常见的缺陷,则应寻找更罕见的缺陷,包括HC-II、纤溶酶原或TPA缺乏、异常纤维蛋白原血症、PAI-1升高或杂合性高胱氨酸血症。活化蛋白C辅因子缺乏(APC抵抗)的发生率尚不清楚,但也可能是一种常见缺陷。随着时间的推移,PAI-1缺陷可能会被证明很常见。发现这些缺陷对于个体患者的治疗以及开展家系研究以识别、告知并可能治疗其他有风险的人具有重要意义。预计随着对止血认识的扩展,更多的遗传性和获得性缺陷,如脂蛋白(a)升高或外源性(组织因子)途径抑制剂(EPI、TFPI)缺陷,可能与血栓形成风险增加有关。

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