医学
纤维蛋白原
低纤维蛋白原血症
怀孕
无纤维蛋白原血症
因素十三
产科
氨甲环酸
妇科
重症监护医学
儿科
内科学
外科
遗传学
生物
失血
作者
Alessandro Casini,Rezan A. Kadir,Magy Abdelwahab,Marilyn J. Manco‐Johnson,S. Raut,Cecil Ross,Philippe de Moerloose,Cristina Santoro,Suchitra S. Acharya
标识
DOI:10.1016/j.jtha.2024.01.008
摘要
Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and / or qualitative fibrinogen deficiency. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (i.e., afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency to 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 ISTH SSC 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.
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