Abstract：
Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and/or qualitative fibrinogen deficiencies. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (ie, afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency for miscarriages, bleeding, and thrombosis. Even though it is well established that management of such pregnancies requires a multidisciplinary approach involving specialists (hematologists and maternal/fetal medicine experts with expertise in the management of inherited bleeding disorders), specific guidelines are lacking. In this International Society on Thrombosis and Haemostasis (ISTH) Scientific and Standardization Committee communication, we aim to propose an expert consensus opinion with literature evidence where available on the strategy for management of pregnancy, delivery, and puerperium in CFDs.
摘要：
先天性纤维蛋白原疾病（CFDs）是一组罕见的先天性定量和/或定性纤维蛋白原缺乏症。分子异常的范围很广,导致纤维蛋白原紊乱的几种亚型(即，纤维蛋白原血症，低纤维蛋白原血症，纤维蛋白原异常血症，低纤维蛋白原血症）。患有CFDs的女性怀孕是一种高风险的临床情况，随着流产趋势的增加，出血,和血栓形成。尽管众所周知，此类妊娠的管理需要涉及专家（血液学家和具有遗传性出血性疾病管理专业知识的母体/胎儿医学专家）的多学科方法，缺乏具体的指导方针。在此ISTHSSC通信中，我们的目标是提出专家共识意见，并提供有关妊娠管理策略的文献证据，delivery,和CFDs的产褥期。
