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[先天性和获得性出血性疾病]

[Congenital and acquired bleeding disorders].

作者信息

Salaj Peter

出版信息

Vnitr Lek. 2018 Summer;64(5):547-558.

Abstract

Hemostasis can be characterized as an array of physiological mechanisms providing both blood fluidity in the intact blood vessels and hemostasis in the event of impaired continuity of the blood vessel wall. The impaired hemo-static balance may lead on the one hand to an increased tendency to bleed, either spontaneously or only in response to an external stimulus. At the opposite end of bleeding are thrombophilic conditions characterized by an increased tendency to blood coagulation and thereby to the development of venous or arterial thrombosis. The hemostatic balance is the result of normal functioning of the blood vessel wall, platelets and plasma agents which include the coagulation and fibrinolytic systems and their inhibitors. By coagulation we understand the process leading to the formation of fibrin networks including the controlled interaction of coagulation factors. It is a physiological process as opposed to thrombosis which can be defined as increased coagulation under pathological conditions. Key words: coagulation - coagulation factors - coagulopathy - hemophilia - inhibitors of coagulation factors deficiencies - purpura - thrombocytopathy - thrombocytopenia.

摘要

止血可被描述为一系列生理机制,这些机制既维持完整血管内血液的流动性,又在血管壁连续性受损时实现止血。止血平衡受损一方面可能导致出血倾向增加,这种增加既可能是自发性的,也可能仅在外部刺激下出现。与出血相反的是血栓形成倾向状态,其特征是血液凝固倾向增加,进而导致静脉或动脉血栓形成。止血平衡是血管壁、血小板和血浆成分正常功能的结果,血浆成分包括凝血和纤维蛋白溶解系统及其抑制剂。我们所说的凝血是指导致纤维蛋白网络形成的过程,包括凝血因子的受控相互作用。这是一个生理过程,与血栓形成相反,血栓形成可定义为病理条件下的凝血增加。关键词:凝血 - 凝血因子 - 凝血病 - 血友病 - 凝血因子缺乏抑制剂 - 紫癜 - 血小板病 - 血小板减少症。

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