[HTML][HTML] Biochemical, molecular and clinical aspects of coagulation factor VII and its role in hemostasis and thrombosis

F Bernardi, G Mariani - haematologica, 2021 - ncbi.nlm.nih.gov
Activated factor VII (FVIIa), the first protease of clotting, expresses its physiological
procoagulant potential only after complexing with tissue factor (TF) exposed to blood. Deep …

Factor VII deficiency: clinical phenotype, genotype and therapy

M Napolitano, S Siragusa, G Mariani - Journal of clinical medicine, 2017 - mdpi.com
Factor VII deficiency is the most common among rare inherited autosomal recessive
bleeding disorders, and is a chameleon disease due to the lack of a direct correlation …

Management of rare inherited bleeding disorders: proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

M Trossaert, V Chamouard… - European Journal of …, 2023 - Wiley Online Library
Introduction The rare coagulation disorders may present significant difficulties in diagnosis
and management. In addition, considerable inter‐individual variation in bleeding phenotype …

Health issues in women and girls affected by haemophilia with a focus on nomenclature, heavy menstrual bleeding, and musculoskeletal issues

AC Weyand, RF Sidonio Jr, M Sholzberg - Haemophilia, 2022 - Wiley Online Library
Introduction Women and girls affected by haemophilia, including haemophilia carriers
(WGH) are at risk of bleeding symptoms that may go unrecognized, including heavy …

The use of prophylaxis in the treatment of rare bleeding disorders

A Shapiro - Thrombosis Research, 2020 - Elsevier
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation factor
deficiencies that include fibrinogen, prothrombin, α 2-antiplasmin, plasminogen activator …

An overview of inherited factor VII deficiency

KS Robinson - Transfusion and Apheresis Science, 2019 - Elsevier
Factor VII (FVII) deficiency is the most common of the Rare Inherited Coagulation Disorders.
The inheritance is autosomal recessive but there is variable penetrance. Overall there is …

[HTML][HTML] Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review

R Abdul-Kadir, K Gomez - Journal of Thrombosis and Haemostasis, 2022 - Elsevier
Abstract Background Congenital factor VII (FVII) deficiency is an inherited bleeding disorder,
with heterogenous bleeding symptoms. Women with FVII deficiency face hemostatic …

[HTML][HTML] Factor VII gene defects: review of functional studies and their clinical implications

S Shahbazi, R Mahdian - Iranian Biomedical Journal, 2019 - ncbi.nlm.nih.gov
Coagulation factors belong to a family of plasma glycosylated proteins that should be
activated for appropriate blood coagulation. Congenital deficiencies of these factors cause …

Management of pregnancy in women with factor VII deficiency: a case series

EJ Lee, L Burey, S Abramovitz, MT Desancho - Haemophilia, 2020 - Wiley Online Library
Introduction Inherited factor VII deficiency is the most common autosomal recessive inherited
bleeding disorder, with an estimated incidence of one per 500 000 cases in the general …

The clotting trigger is an important determinant for the coagulation pathway in vivo or in vitro—inference from data review

S He, H Cao, C Thålin, J Svensson… - … in Thrombosis and …, 2021 - thieme-connect.com
Blood coagulation comprises a series of enzymatic reactions leading to thrombin generation
and fibrin formation. This process is commonly illustrated in a waterfall-like manner, referred …