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[下肢静脉血栓形成儿童的生理性凝血抑制剂缺乏症。6例前瞻性研究]

[Deficiencies of physiological coagulation inhibitors in children with venous thromboses of the lower limbs. Prospective study of 6 cases].

作者信息

Khaldi F, Toumi N H, Ben Jaballah N, Zriba S, Ben Naceur B

机构信息

Hôpital d'Enfants de Tunis, Bab-Saadoun, Tunisíe.

出版信息

Ann Pediatr (Paris). 1993 Jun;40(6):348-52.

PMID:8352495
Abstract

Six patients aged 3 to 14 years with lower limb vein thrombosis were included in a prospective study of deficiencies in physiological coagulation inhibitors. The laboratory evaluation included standard hemostasis tests, tests for circulating anticoagulants, immunological and functional assays of protein C, protein S, and antithrombin III, and a study of fibrinolysis. A qualitative protein S deficiency with decreased fibrinolysis and protein C deficiency were found. The family study detected asymptomatic heterozygotes in both families investigated. No antithrombin III deficiency or circulating anticoagulants were found.

摘要

6名年龄在3至14岁的下肢静脉血栓形成患者被纳入一项关于生理性凝血抑制剂缺乏的前瞻性研究。实验室评估包括标准止血试验、循环抗凝剂检测、蛋白C、蛋白S和抗凝血酶III的免疫学及功能测定,以及纤维蛋白溶解研究。发现了纤维蛋白溶解降低的定性蛋白S缺乏和蛋白C缺乏。家系研究在两个被调查的家族中均检测到无症状杂合子。未发现抗凝血酶III缺乏或循环抗凝剂。

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