Department of Anesthesiology, Intensive Care and Pain Medicine, Clinical Hospital Center Rijeka, Rijeka, Croatia.
Department of Anesthesiology, Resuscitation, Emergency and Intensive Care Medicine, Faculty of Medicine, University of Rijeka, Rijeka, Croatia.
Am J Case Rep. 2020 Mar 18;21:e920685. doi: 10.12659/AJCR.920685.
BACKGROUND Congenital factor X deficiency is a rare inherited coagulopathy. Pregnancies in women with this disorder are often associated with adverse outcomes, including miscarriage, premature labor, and hemorrhage during pregnancy and in the peripartum period. The literature on this disorder is sparse and shows a limited number of successful pregnancies in women with factor X deficiency. CASE REPORT In this report, we present the case of a successful pregnancy and term delivery by elective cesarean section in a 39-year-old primigravida with congenital factor X deficiency. Medical management followed the recommendations of an interdisciplinary team comprising specialists in obstetrics, anesthesia, transfusion medicine, hematology, and neonatology. This high-risk pregnancy was successfully brought to term, and a healthy male neonate was delivered by elective cesarean section at 39 weeks' gestation. The patient's factor X deficiency (0.19 kIU/L) was treated using 4 units of solvent-detergent-treated fresh frozen plasma (SD-FFP) 1 h before the cesarean section, leading to hemostatic levels of factor X and an uneventful intraoperative course. Postoperatively, the patient's factor X levels were controlled daily and corrected using SD-FFP as needed, with no clinically significant blood loss. CONCLUSIONS SD-FFP can be used to manage congenital factor X deficiency in the peripartum period and maintain perioperative blood loss within normal limits.
先天性因子 X 缺乏症是一种罕见的遗传性凝血障碍。患有这种疾病的女性妊娠常伴有不良结局,包括流产、早产以及妊娠和围产期出血。关于这种疾病的文献很少,表明因子 X 缺乏症女性成功妊娠的案例有限。
本报告介绍了 1 例成功妊娠并经选择性剖宫产足月分娩的病例。患者为 39 岁初产妇,患有先天性因子 X 缺乏症。医疗管理遵循由产科、麻醉、输血医学、血液学和新生儿科专家组成的跨学科团队的建议。这例高危妊娠成功足月,健康男婴于 39 孕周行选择性剖宫产分娩。患者因子 X 缺乏症(0.19 kIU/L)于剖宫产前 1 小时接受了 4 单位溶剂/去污处理的新鲜冷冻血浆(SD-FFP)治疗,使因子 X 达到止血水平,术中过程顺利。术后,患者每日监测因子 X 水平,并根据需要使用 SD-FFP 进行校正,无明显临床意义的失血。
SD-FFP 可用于围产期管理先天性因子 X 缺乏症,并维持围手术期失血量在正常范围内。