EC Gynaecology

Congenital dysfibrinogenemia (CD) is a rare coagulation disorder caused by monoallelic mutations in one of the fibrinogen genes (FGA, FGB, or FGG) inherited in autosomal dominant pattern. Most individuals with CD are asymptomatic at the time of diagnosis; however, the condition may lead to bleeding or thrombosis, which can pose life-threatening risks during pregnancy, childbirth, and the early postpartum period. As such, early diagnosis and the management of blood loss are crucial. This article discusses five of our recent cases to highlight their diagnosis and treatment, with the goal of presenting various clinical scenarios involving pregnant women with congenital dysfibrinogenemia, emphasizing multidisciplinary management during antenatal, intrapartum and postpartum periods to improve obstetric and neonatal outcomes.

 Keywords: Congenital Dysfibrinogenemia (CD); Fibrinogen Replacement Therapy (FRT)

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