Faculty of Medicine, University of Geneva, Geneva, Switzerland.
Unité de Gynécologie Endocrinienne, Hôpital Port-Royal, Université Paris Descartes, Equipe EPOPE, Inserm, Paris, France.
Haemophilia. 2019 Sep;25(5):747-754. doi: 10.1111/hae.13825. Epub 2019 Jul 31.
Hereditary fibrinogen disorders (HFD) are rare quantitative or qualitative fibrinogen anomalies, including afibrinogenaemia (A), hypofibrinogenaemia (H), dysfibrinogenaemia (D) and hypodysfibrinogenaemia (HD). As fibrinogen plays an essential role in pregnancy, we addressed the issue of obstetrical and postpartum complications in women with HFD.
A systematic literature review, restricted to English manuscripts, was conducted according to the PRISMA guidelines. We searched through the MEDLINE database for English articles, published from January 1985 until November 2018, focusing on pregnancy in A, H, D and HD. A total of 198 articles were identified, 15 articles were added from other sources. Overall, 213 articles were screened and 54 were included in the final analysis.
A total of 188 pregnancies from 70 women were analysed. About half of pregnancies resulted in miscarriage; more specifically in 15 (42.9%), 36 (46.8%), 27 (42.9%) and 4 (30.8%) of A, H, D and HD patients, respectively. Preterm complications were also frequent (33.5%). Metrorrhagia, mainly in the first trimester, was observed in 21.7% of the pregnancies. Placenta abruption was reported in 5 (14.3%), 4 (5.2%), 5 (7.9%) and 1 (7.7%) of A, H, D and HD, respectively. A total of 24 (12.7%) deliveries were complicated by postpartum thrombotic events (3.2%) or postpartum haemorrhage (9.6%). A fibrinogen replacement therapy was introduced in 30% of pregnancies, as prophylaxis (81.1%) or on demand (18.9%).
These results suggest that women with HFD are at high risk of obstetrical and postpartum complications. Prospective international registries may allow to identify more precisely the incidence of obstetrical and postpartum adverse outcomes and their management.
遗传性纤维蛋白原紊乱（Hereditary fibrinogen disorders，HFD）是罕见的纤维蛋白原定量或定性异常，包括无纤维蛋白血症（afibrinogenaemia，A）、低纤维蛋白血症（hypofibrinogenaemia，H）、异常纤维蛋白血症（dysfibrinogenaemia，D）和低异常纤维蛋白血症（hypodysfibrinogenaemia，HD）。由于纤维蛋白原在妊娠中起着重要作用，我们研究了纤维蛋白原异常的女性在产科和产后的并发症问题。
根据 PRISMA 指南，我们进行了一项系统的文献综述，仅限于英文文献。我们在 MEDLINE 数据库中搜索了 1985 年 1 月至 2018 年 11 月期间的英文文章，重点关注 A、H、D 和 HD 患者的妊娠问题。共确定了 198 篇文章，另外还从其他来源添加了 15 篇文章。总共筛选了 213 篇文章，最终有 54 篇纳入了分析。
共分析了来自 70 名女性的 188 例妊娠。大约一半的妊娠以流产告终；具体来说，A、H、D 和 HD 患者的流产率分别为 15（42.9%）、36（46.8%）、27（42.9%）和 4（30.8%）。早产并发症也很常见（33.5%）。月经过多，主要发生在妊娠早期，占 21.7%。胎盘早剥分别发生在 5（14.3%）、4（5.2%）、5（7.9%）和 1（7.7%）例 A、H、D 和 HD 患者中。产后血栓事件（3.2%）或产后出血（9.6%）共导致 24（12.7%）例分娩复杂化。30%的妊娠采用纤维蛋白原替代疗法进行预防（81.1%）或按需治疗（18.9%）。
这些结果表明，纤维蛋白原异常的女性在产科和产后并发症方面风险较高。前瞻性国际登记可能更准确地确定产科和产后不良结局的发生率及其处理方法。
