نتایج جستجو برای: combined factor v viii deficiency

تعداد نتایج: 1585920  

Journal: :Journal of thrombosis and haemostasis : JTH 2004
B Zhang D Ginsburg

Combined deficiency of factor (F)V and FVIII (F5F8D) and combined deficiency of vitamin K-dependent clotting factors (VKCFD) comprise the vast majority of reported cases of familial multiple coagulation factor deficiencies. Recently, significant progress has been made in understanding the molecular mechanisms underlying these disorders. F5F8D is caused by mutations in two different genes (LMAN1...

Journal: :Journal of thrombosis and haemostasis : JTH 2006
U Seligsohn D Ginsburg

In 1954 Oeri et al. [1] described two young siblings with a lifelong history of a bleeding tendency related to a deficiency of both factor (F) V and FVIII. Reports of additional families in the late 1950s and 1960s [2–6] suggested that the common occurrence of FV and FVIII deficiencies was not a mere coincidence of parahemophilia and hemophilia A. This was further supported by the observation t...

2014
Ahmed Ghassan El Adib Farah Majdi Mohamed Othmane Dilai Hamid Asmouki Ahlam Bassir Karam Harou Abderraouf Soumani Said Younous Lahoucine Mahmal

Inherited combined factor V and factor VIII deficiency (F5F8D) is autosomal recessive transmission disorder. Epistaxis, postsurgical bleeding, and menorrhagia are the most common symptoms. The risk of miscarriage and placental abruption is consequent. We report a case of successful pregnancy in a patient with F5F8D. 20-year-old woman, born of consanguineous parents, third gestate, first parity,...

Journal: :Blood 2008
Bin Zhang Marta Spreafico Chunlei Zheng Angela Yang Petra Platzer Michael U Callaghan Zekai Avci Namik Ozbek Johnny Mahlangu Tabitha Haw Randal J Kaufman Kandice Marchant Edward G D Tuddenham Uri Seligsohn Flora Peyvandi David Ginsburg

Combined deficiency of factor V and factor VIII (F5F8D) is caused by mutations in one of 2 genes, either LMAN1 or MCFD2. Here we report the identification of mutations for 11 additional F5F8D families, including 4 novel mutations, 2 in MCFD2 and 2 in LMAN1. We show that a novel MCFD2 missense mutation identified here (D81Y) and 2 previously reported mutations (D89A and D122V) abolish MCFD2 bind...

Journal: :Seminars in Thrombosis and Hemostasis 2013

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