BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth ...BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult.Recombinant factor VIIa is the most common replacement therapy for FVIID.However,no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID.Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening.She successfully delivered a live infant without any complications,such as postpartum hemorrhage,neonatal abnormalities,and so on.CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.展开更多
To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prol...To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prolongation of the prothrombin time(PT)with a normal activated partial thromboplastin time(aPTT).Factor assay and genotyping for FVII can be done to confirm the diagnosis.1,2 Patients may present with menorrhagia,and bleeding tendencies may vary from slight gingival bleeding,epistaxis,and ecchymosis,to severe bleeding,such as gastrointestinal and intracranial bleeding.展开更多
基金Supported by Natural Science Foundation of Guangxi,No.2018JJB140171Medical Excellence Award Funded by the Creative Research Development Grant from the First Affiliated Hospital of Guangxi Medical University(2020)+1 种基金Development and Application of Appropriate Medical and Health Technologies in Guangxi,No.S2018111Self-funded Scientific Research Project of Guangxi Health Commission,No.Z20190512.
文摘BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult.Recombinant factor VIIa is the most common replacement therapy for FVIID.However,no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID.Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening.She successfully delivered a live infant without any complications,such as postpartum hemorrhage,neonatal abnormalities,and so on.CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.
文摘To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prolongation of the prothrombin time(PT)with a normal activated partial thromboplastin time(aPTT).Factor assay and genotyping for FVII can be done to confirm the diagnosis.1,2 Patients may present with menorrhagia,and bleeding tendencies may vary from slight gingival bleeding,epistaxis,and ecchymosis,to severe bleeding,such as gastrointestinal and intracranial bleeding.
