Guy G P, Baxi L V, Hurlet-Jensen A, Chao C R
Department of Obstetrics and Gynecology, Columbia Presbyterian Medical Center, College of Physicians and Surgeons, Columbia University, New York, New York.
Obstet Gynecol. 1992 Sep;80(3 Pt 2):502-5.
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.
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。
