Vergnes C, Lorient-Roudaut M F, Haissaguerre M, Roudaut R, Wicker P, Boisseau M R, Dallocchio M
Arch Mal Coeur Vaiss. 1985 Mar;78(3):440-3.
The case of a young man hospitalised for bilateral lower limb deep vein thrombosis is reported. None of the usual causes were found after systematic wide-ranging investigation. The only abnormality on admission was a spontaneous increase in the cephalin-kaolin time to 65 seconds compared to a control time of 40 seconds. Measurements of the clotting factors showed a moderate and isolated deficiency in factor XII (30 p. 100), also present in a brother (50 p. 100) and a sister (42.5 p. 100). Fibrinolytic therapy was administered : an initial course of Streptokinase was followed by extension of a left femoral vein thrombosis and pulmonary embolism. Two courses of Urokinase were given with an eight day interval without significantly improving the venous circulation. This case is an example of thrombogenic disease due to a deficiency of a clotting factor resulting in non-activation of physiological fibrinolysis.
报告了一名因双侧下肢深静脉血栓形成而住院的年轻男子的病例。经过系统的广泛检查,未发现任何常见病因。入院时唯一的异常是白陶土部分凝血活酶时间自发延长至65秒,而对照时间为40秒。凝血因子检测显示,因子XII中度且单独缺乏(30%),其兄弟(50%)和姐妹(42.5%)也存在这种情况。给予了纤溶治疗:先用链激酶初始疗程,随后左股静脉血栓形成和肺栓塞扩展。间隔八天给予两个疗程的尿激酶,但静脉循环未得到明显改善。该病例是由于凝血因子缺乏导致生理性纤溶未激活而引发血栓形成疾病的一个例子。