Defective Activation of Clotting, Fibrinolytic, and Permeability‐Enhancing Systems in Human Fletcher Trait Plasma
Defective Activation of Clotting, Fibrinolytic, and Permeability‐Enhancing Systems in Human Fletcher Trait Plasma
复制标题
人类弗莱彻性状血浆中凝血、纤溶和通透性增强系统的激活缺陷
DOI：
10.1161/01.res.34.5.641
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发表时间：
1974
影响因子：
20.1
通讯作者：
O. Ratnoff
中科院分区：
文献类型：
--
作者：
Hidehiko Salto;O. Ratnoff
当正常血浆暴露于异物表面时，Hageman因子（HF, factor XII）被激活；在适当的情况下，它会引发导致凝血、纤维蛋白溶解、血管通透性增加、酯溶活性和激肽形成的反应。然而，具有Fletcher特征的个体血浆中的凝血、纤维蛋白溶解和血管通透性增加受到损害，尽管在功能和免疫上与正常血浆中的HF浓度没有区别。这种缺陷凝血可以通过少量正常血浆、HF缺陷血浆、PTA缺陷血浆或活化PTA完全修复，但只能通过活化HF部分修复。通过添加少量正常血浆、HF缺陷血浆、pta缺陷血浆或活化HF，可以部分纠正纤维蛋白溶解缺陷和通透性增强活性。部分纯化血浆钾激肽的制备在很大程度上修复了高岭土存在下弗莱彻性状血浆中的凝血和纤维蛋白溶解缺陷。在免疫扩散研究中，弗莱彻特征血浆和单特异性抗钾激肽血清之间没有形成沉淀线。弗莱彻特征血浆似乎缺乏血浆前钾likrein，这很可能参与活化HF的功能。这些研究强调凝血、纤溶和血管通透性增强之间的密切关系。
When normal plasma is exposed to foreign surfaces, Hageman factor (HF, factor XII) is activated; under appropriate circumstances, it then initiates reactions leading to coagulation, fibrinolysis, increased vascular permeability, esterolytic activity, and kinin formation. However, coagulation, fibrinolysis, and increased vascular permeability were impaired in plasma from an individual with Fletcher trait, despite a normal concentration of HF that is functionally and immunologically indistinguishable from that in normal plasma. This defective clotting is completely repaired by the addition of small amounts of normal plasma, HF-deficient plasma, plasma thrombo-plastin antecedent (PTA)-deficient plasma, or activated PTA but only partially repaired by the addition of activated HF. Defective fibrinolysis and permeability-enhancing activity were partially corrected by the addition of small amounts of normal plasma, HF-deficient plasma, PTA-deficient plasma, or activated HF. A preparation of partially purified plasma kallikrein largely repaired defective coagulation and fibrinolysis in Fletcher trait plasma in the presence of kaolin. In immunodif fusion studies, no precipitin line formed between Fletcher trait plasma and monospecific antikallikrein serum. Fletcher trait plasma appeared to be deficient in a plasma prekallikrein, which most probably participates in the functioning of activated HF. These studies emphasize the intimate relationships among clotting, fibrinolysis, and enhancement of vascular permeability.