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先天性低纤维蛋白原血症与复发性胎盘早剥。

Congenital hypofibrinogenemia and recurrent placental abruption.

作者信息

Ness P M, Budzynski A Z, Olexa S A, Rodvien R

出版信息

Obstet Gynecol. 1983 Apr;61(4):519-23.

PMID:6828283
Abstract

A 32-year-old white woman with a history of 2 episodes of abruptio placentae was found to have congenital hypofibrinogenemia. She had no bleeding difficulties except when pregnant. The patient's sisters and her mother also had reduced fibrinogen levels. Results of fibrinogen measurement by clotting assays and immunologic studies were similar. Immunoelectrophoresis, molecular weight of fibrinogen chains, cross-linking by factor XIII, carbohydrate staining, and sialic acid quantitation were all normal, suggesting the diagnosis of hypofibrinogenemia rather than dysfibrinogenemia. Fibrinolysis did not account for the reduced fibrinogen level. This case demonstrates that congenital low fibrinogen levels may be associated with placental abruption and that an abnormal fibrinogen molecule is not necessary.

摘要

一名有2次胎盘早剥病史的32岁白人女性被发现患有先天性低纤维蛋白原血症。除怀孕时外,她没有出血困难。患者的姐妹和母亲的纤维蛋白原水平也降低。通过凝血试验和免疫学研究进行的纤维蛋白原测量结果相似。免疫电泳、纤维蛋白原链的分子量、因子XIII交联、碳水化合物染色和唾液酸定量均正常,提示诊断为低纤维蛋白原血症而非异常纤维蛋白原血症。纤维蛋白溶解不能解释纤维蛋白原水平降低的原因。该病例表明,先天性低纤维蛋白原水平可能与胎盘早剥有关,且不一定存在异常纤维蛋白原分子。

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