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Successful Pregnancy in a Patient with Combined Deficiency of Factor V and Factor VIII

机译:因子V和因子VIII联合缺乏症患者的成功妊娠

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Inherited combined factor V and factor VIII deficiency (F5F8D) is autosomal recessive transmission disorder. Epistaxis, postsurgical bleeding, and menorrhagia are the most common symptoms. The risk of miscarriage and placental abruption is consequent. We report a case of successful pregnancy in a patient with F5F8D. 20-year-old woman, born of consanguineous parents, third gestate, first parity, two miscarriages, admitted for child birth of a spontaneous pregnancy estimated at 38 weeks and was diagnosed with F5F8D. At admission, patient was hemodynamically stable, with good obstetric conditions. The biologic results showed low levels of PT (52%), factor V (7%), and factor VIII (5%), and the activated partial thromboplastin time was prolonged (68,6%). Parturient was admitted in intensive care unit, maternal and fetal monitoring was performed. Fresh frozen plasma (FFP) and factor VIII concentrates were perfused at the induction of labor. Analgesia used fentanyl titration. The delivery gave birth to a newborn male, with Apgar 10/10 and 3000 g. The puerperium was simple without any important bleeding. Laboratory tests for the newborn were acceptable. Little literature is available on this subject and there are no guidelines available concerning pregnancy; we chose to prescribe a combination of factor VIII concentrate and FFP in pre-, per- and postpartum. The same protocol was successfully used in a patient before dental extraction and prostatectomy. Vaginal delivery is possible, as our case. Management by multidisciplinary team is recommended.
机译:遗传的联合因子V和因子VIII缺乏症(F5F8D)是常染色体隐性传播疾病。鼻出血,术后出血和月经过多是最常见的症状。因此有流产和胎盘早剥的风险。我们报告了一名F5F8D患者成功怀孕的案例。一名近亲父母,第三胎,第一胎,两胎流产的20岁妇女,因自发妊娠而出生,估计在38周时出生,被诊断出患有F5F8D。入院时患者血流动力学稳定,产科条件良好。生物学结果显示低水平的PT(52%),V因子(7%)和VIII因子(5%),活化的部分凝血活酶时间延长(68.6%)。产妇进入重症监护病房,进行母婴监测。在引产时灌注新鲜的冷冻血浆(FFP)和VIII因子浓缩液。镇痛采用芬太尼滴定法。分娩产下了一名新生男性,Apgar 10/10和3000μg。产褥很简单,没有任何重要的出血。新生儿的实验室检查是可以接受的。关于该主题的文献很少,也没有关于怀孕的指南。我们选择在产前,产后和产后开处方VIII因子浓缩液和FFP。在拔牙和前列腺切除术之前,该协议已成功用于患者。根据我们的情况,可以进行阴道分娩。建议由多学科团队进行管理。

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