Open Access Articles- Top Results for Factor VII
Journal of Clinical & Experimental Dermatology ResearchA Little Known but Potentially Life-threatening Association of Bullous Pemphigoid and Acquired Hemophilia: Case Report and Review of the Literature
Journal of Anesthesia & Clinical ResearchBloodless Liver Transplantation: ROTEM guided Rational Prophylactic use of Recombinant Activated Factor VII
Journal of Anesthesia & Clinical ResearchRecombinant Activated Factor VII (rFVIIa) Treatment of Refractory Bleeding in Cardiac Surgical Patients
Journal of Genetic Syndromes & Gene therapyLiver-Directed Adeno-Associated Viral Gene Therapy for Hemophilia
Journal of Genetic Syndromes & Gene therapyEngineering Factor Viii for Hemophilia Gene Therapy
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Factor VII (EC 18.104.22.168, blood-coagulation factor VIIa, activated blood coagulation factor VII, formerly known as proconvertin) is one of the proteins that causes blood to clot in the coagulation cascade. It is an enzyme of the serine protease class. A recombinant form of human factor VIIa (eptacog alfa [activated], NovoSeven) has U.S. Food and Drug Administration approval for uncontrolled bleeding in hemophilia patients. It is sometimes used unlicensed in severe uncontrollable bleeding, although there have been safety concerns. A biosimilar form of recombinant activated factor VII (AryoSeven) is also available, but does not play any considerable role in the market.
The main role of factor VII (FVII) is to initiate the process of coagulation in conjunction with tissue factor (TF/factor III). Tissue factor is found on the outside of blood vessels - normally not exposed to the bloodstream. Upon vessel injury, tissue factor is exposed to the blood and circulating factor VII. Once bound to TF, FVII is activated to FVIIa by different proteases, among which are thrombin (factor IIa), factor Xa, IXa, XIIa, and the FVIIa-TF complex itself. The complex of factor VIIa with TF catalyzes the conversion of factor IX and factor X into the active proteases, factor IXa and factor Xa, respectively.
The action of the factor is impeded by tissue factor pathway inhibitor (TFPI), which is released almost immediately after initiation of coagulation. Factor VII is vitamin K dependent; it is produced in the liver. Use of warfarin or similar anticoagulants decreases hepatic synthesis of FVII.
Role in disease
Deficiency is rare (congenital proconvertin deficiency) and inherits recessively. Factor VII deficiency presents as a hemophilia-like bleeding disorder. It is treated with recombinant factor VIIa (NovoSeven or AryoSeven).
Recombinant factor VIIa, marketed under the trade names AryoSeven and NovoSeven, is used for people with hemophilia (with Factor VIII or IX deficiency) who have developed inhibitors against replacement coagulation factor.
It has also been used in the setting of uncontrollable hemorrhage, but its role in this setting is controversial with insufficient evidence to support its use outside of clinical trials. The first report of its use in hemorrhage was in an Israeli soldier with uncontrollable bleeding in 1999. Risks of its use include an increase in arterial thrombosis.
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- Roberts HR, Monroe DM, White GC (2004). "The use of recombinant factor VIIa in the treatment of bleeding disorders". Blood 104 (13): 3858–64. PMID 15328151. doi:10.1182/blood-2004-06-2223.
- Simpson E, Lin Y, Stanworth S, Birchall J, Doree C, Hyde C (Mar 14, 2012). "Recombinant factor VIIa for the prevention and treatment of bleeding in patients without haemophilia.". Cochrane database of systematic reviews (Online) 3: CD005011. PMID 22419303. doi:10.1002/14651858.CD005011.pub4.
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- Versteeg HH, Peppelenbosch MP, Spek CA (2002). "The pleiotropic effects of tissue factor: a possible role for factor VIIa-induced intracellular signalling?". Thromb. Haemost. 86 (6): 1353–9. PMID 11776298.
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