What is factor XIII and its importance in blood clotting process:
Factor XIII is a blood clotting factor which is a fibrin stabilizing factor in blood clotting process or system , it is an enzyme transglutaminases which plays an important role blood clotting . After it is acted up on by thrombin which is released when ever there is tissue rupture or injury and acts on fibrinogen and leads to the formation of fibrin which in turn forms a insoluble clot of fibrin fibers with the help of Factor XIII. As Factor XIII is an enzyme it helps in formation of γ-glutamyl-Є-lysyl amide cross links in peptides of fibrin fibers resulting in to insoluble blood clot, which helps in blocking the wounds and cuts there by preventing blood loss.
Insufficient amount or deficiency of factor XIII in ones blood is due to congenital defect and result in to faulty blood clotting , where the clot formed is not able to stop bleeding, which poses a risk of excessive bleeding. Congenital deficiency of Factor XIII is rare and affect 1 person out of 3000000. The deficiency results in soft tissue bruising, mucosal bleeding and some times intracranial bleeding which may become life threatening if not treated. In newborn children’s deficiency of Factor XIII result in umbilical cord bleeding which may become life threatening.
The US FDA on 17 Feb 2011 approved a product to treat congenital defect of blood clotting factor xiii .The name of product approved is Corifact, which is manufactured from pooled plasma from healthy donors. US FDA granted approval for corifact as an orphan drug as the disease where it will be used to treat is a rare disease. It was approved by US FDA in their accelerated approval regulations requiring further study to demonstrate its safety and efficacy.US FDA approved corifact on clinical study on 14 patients , which included children’s with congenital deficiency of factor Xiii. Side effects observed were increase in liver (hepatic) enzymes , elevated thrombin-antithrombin levels, hypersensitivity reactions (rash, pruritus, allergy, erythema), arthralgia, headache, chills, fever.
Patients after administration of corifact may produce antibodies against Factor XIII which may result in nullification or activity of product rendering it less or complete ineffective . In higer dosages it may cause abnormal clotting resulting in to adverse events because of abnormal clotting .
Corifact is manufactured by Germany Based CSL Behring of Marburg.
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