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一例患有IX因子缺乏症孕妇的罕见并发症:病例报告及文献复习

An unusual complication in a gravida with factor IX deficiency: case report with review of the literature.

作者信息

Guy G P, Baxi L V, Hurlet-Jensen A, Chao C R

机构信息

Department of Obstetrics and Gynecology, Columbia Presbyterian Medical Center, College of Physicians and Surgeons, Columbia University, New York, New York.

出版信息

Obstet Gynecol. 1992 Sep;80(3 Pt 2):502-5.

PMID:1495722
Abstract

Factor IX deficiency (hemophilia B, Christmas disease) is an X-linked recessive coagulation disorder. It occurs in one out of every 25,000-30,000 male births and requires even rarer genetic circumstances for phenotypic expression in females. We report the occurrence of a large, late-trimester subchorionic hematoma in a gravida with factor IX deficiency and with laboratory evidence of consumptive coagulopathy during treatment. The patient was managed conservatively and had a successful outcome at term. The only four reported cases of antepartum management of factor IX deficiency in the English literature are reviewed.

摘要

因子IX缺乏症(血友病B,克里斯马斯病)是一种X连锁隐性凝血障碍。每25000 - 30000例男性出生中就有1例发生该病,而女性出现表型表达所需的遗传情况更为罕见。我们报告了1例患有因子IX缺乏症的孕妇在妊娠晚期出现大面积绒毛膜下血肿,并在治疗期间有消耗性凝血病的实验室证据。该患者接受了保守治疗,足月时获得了成功的结局。我们还回顾了英文文献中仅有的4例因子IX缺乏症产前管理的报道病例。

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