Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).
Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).
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菲茨杰拉德特征：缺乏迄今为止未被识别的试剂，菲茨杰拉德因子，参与凝血、纤维蛋白溶解、激肽生成的表面介导反应，以及稀释血浆增强血管通透性的特性（PF/Dil）。
DOI：
10.1172/jci108009
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发表时间：
1975
期刊：
影响因子：
--
通讯作者：
Joseph P. Abraham
中科院分区：
文献类型：
--
作者：
Hidehiko Saito;O. Ratnoff;Robert Waldmann;Joseph P. Abraham
在一名71岁无症状男性的血浆中观察到部分凝血活酶时间延长，这与一种迄今为止尚未认识到的因子缺乏有关。患者的血浆还表现出受损的表面介导的纤维蛋白溶解和酯溶解活性，以及受损的激肽生成和增强血管通透性的特性（称为PF/Dil）。患者血浆中含有正常量的所有已知凝血因子，除了弗莱彻因子（血浆前激肽释放酶），其浓度为合并正常血浆的10-15%。然而，弗莱彻性状血浆中含有正常量的患者血浆中缺失的药物，并纠正了凝血、纤维蛋白溶解和血管通透性的缺陷。弗莱彻性状血浆在纠正激肽生成和酯酶活性方面效果较差，可能是因为患者的前激肽释放酶部分缺乏。该因子在患者血浆中缺乏的作用部位似乎是在Hageman因子和血浆前激肽释放酶激活之后。一部分正常血浆，缺乏其他凝血因子，纠正了患者血浆中凝血的缺陷;患者血浆的类似部分没有纠正这种异常。目前还没有证据表明病人的缺陷具有家族性。然而，病人的病症可以用他的姓氏来称呼为菲茨杰拉德特征，而他血浆中明显缺乏的因子可以称为菲茨杰拉德因子。
The prolonged partial thromboplastin time observed in the plasma of a 71-yr-old asymptomatic man was related to the deficiency of a hitherto unrecognized agent. The patient's plasma also exhibited impaired surface-mediated fibrinolysis and esterolytic activity and impaired generation of kinins and of the property enhancing vascular permeability designated PF/Dil. The patient's plasma contained normal amounts of all known clotting factors except Fletcher factor (a plasma prekallikrein) which was present at a concentration of 10-15% of pooled normal plasma. Fletcher trait plasma, however, contained normal amounts of the agent missing from the patient's plasma and corrected the defects in clotting, fibrinolysis, and vascular permeability. Fletcher trait plasma was less effective in correcting generation of kinins and esterolytic activity, presumably because of the patient's partial deficiency of prekallikrein. The site of action of the factor deficient in the patient's plasma appeared to be subsequent to the activation of Hageman factor and plasma prekallikrein. A fraction of normal plasma, devoid of other clotting factors, corrected the defect in clotting in the patient's plasma; a similar fraction of the patient's plasma did not correct this abnormality. No evidence yet exists pointing to the familial nature of the patient's defect. Tentatively, the patient's disorder may be referred to by his surname as Fitzgerald trait, and the agent apparently deficient in his plasma as Fitzgerald factor.