遗传性纤维蛋白原紊乱的产科并发症：纤维蛋白原治疗研究。
Obstetrical complications in hereditary fibrinogen disorders: the Fibrinogest study.
机构信息
Gynecology Department, Gynecological Endocrinology Unit, Hospital Saint Joseph, INSERM UMR 1153, EPOPé Group, Paris, France; University Hospitals of Geneva and the Faculty of Medicine of the Geneva University, DFEA-Ob/Gyn-reproductive medicine, Geneva, Switzerland.
Gynecology Department, Bigorre Hospital Center, Tarbes, France.
出版信息
J Thromb Haemost. 2023 Aug;21(8):2126-2136. doi: 10.1016/j.jtha.2023.04.035. Epub 2023 May 11.
BACKGROUND
Women with hereditary fibrinogen disorders (HFDs) seem to be at an increased risk of adverse obstetrical outcomes, but epidemiologic data are limited.
OBJECTIVES
We aimed to determine the prevalence of pregnancy complications; the modalities and management of delivery; and the postpartum events in women with hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia.
METHODS
We conducted a retrospective and prospective multicentric international study.
RESULTS
A total of 425 pregnancies were investigated from 159 women (49, 95, and 15 cases of hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia, respectively). Overall, only 55 (12.9%) pregnancies resulted in an early miscarriage, 3 (0.7%) resulted in a late miscarriage, and 4 (0.9%) resulted in an intrauterine fetal death. The prevalence of live birth was similar among the types of HFDs (P = .31). Obstetrical complications were observed in 54 (17.3%) live birth pregnancies, including vaginal bleeding (14, 4.4%), retroplacental hematoma (13, 4.1%), and thrombosis (4, 1.3%). Most deliveries were spontaneous (218, 74.1%) with a vaginal noninstrumental delivery (195, 63.3%). A neuraxial anesthesia was performed in 116 (40.4%) pregnancies, whereas general or no anesthesia was performed in 71 (16.6%) and 129 (44.9%) pregnancies, respectively. A fibrinogen infusion was administered in 28 (8.9%) deliveries. Postpartum hemorrhages were observed in 62 (19.9%) pregnancies. Postpartum venous thrombotic events occurred in 5 (1.6%) pregnancies. Women with hypofibrinogenemia were at an increased risk of bleeding during the pregnancy (P = .04).
CONCLUSION
Compared with European epidemiologic data, we did not observe a greater frequency of miscarriage, while retroplacental hematoma, postpartum hemorrhage, and thrombosis were more frequent. Delivery was often performed without locoregional anesthesia. Our findings highlight the urgent need for guidance on the management of pregnancy in HFDs.
背景
遗传性纤维蛋白原异常（HFD）的女性似乎有更高的不良产科结局风险，但流行病学数据有限。
目的
本研究旨在确定低纤维蛋白原血症、异常纤维蛋白原血症和低纤维蛋白血症女性的妊娠并发症、分娩方式和产后事件的发生率。
方法
我们进行了一项回顾性和前瞻性的国际多中心研究。
结果
共纳入 159 例女性的 425 例妊娠，其中低纤维蛋白原血症、异常纤维蛋白原血症和低纤维蛋白血症患者分别为 49、95 和 15 例。总的来说，只有 55 例（12.9%）妊娠发生早期流产，3 例（0.7%）发生晚期流产，4 例（0.9%）发生宫内胎儿死亡。不同类型 HFD 的活产率相似（P=0.31）。54 例（17.3%）活产妊娠出现产科并发症，包括阴道出血（14 例，4.4%）、胎盘后血肿（13 例，4.1%）和血栓形成（4 例，1.3%）。大多数分娩为自发性（218 例，74.1%），阴道非器械分娩 195 例（63.3%）。116 例（40.4%）妊娠行椎管内麻醉，71 例（16.6%）和 129 例（44.9%）妊娠分别行全身麻醉和未行麻醉。28 例（8.9%）分娩时输注纤维蛋白原。62 例（19.9%）妊娠发生产后出血。5 例（1.6%）妊娠发生产后静脉血栓事件。低纤维蛋白原血症患者妊娠出血风险增加（P=0.04）。
结论
与欧洲的流行病学数据相比，本研究未观察到流产发生率更高，而胎盘后血肿、产后出血和血栓形成更为常见。分娩时通常不使用局部麻醉。我们的研究结果强调迫切需要制定 HFD 妊娠管理指南。
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