Current understanding in diagnosis and management of factor XIII deficiency
نویسندگان
چکیده مقاله:
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resistant to degradation by the fibrinolytic system that enables the body to stop potential bleeding episodes. In the absence or severe decrease of factor XIII, although the clot is formed, but is rapidly degraded by the fibrinolytic system, and delayed bleeding occurs. Factor XIII deficiency is an extremely rare bleeding disorder with estimated incidence of 1/2-3000, 000 in the general population. Presumptive diagnosis of factor XIII deficiency was by clot solubility test in 5M urea or 1% monochloroacetic acid environments. In patients with abnormal screening clot solubility test, the disease can be confirmed by more specific tests such as quantitative factor XIII activity assay and FXIIIAg assay.After diagnosis of disease all patients with severe factor XIII deficiency(<1 U/dl) should receive prophylactic substitution therapy with fresh frozen plasma (FFP) and cryoprecipitate as traditional choices or purified concentrate of blood coagulation factor XIII (Fibrogammin P) in order to control severe and life-threatening clinical complications of factor XIII deficiency.
منابع مشابه
Factor XIII deficiency: a review of literature
Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...
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عنوان ژورنال
دوره 3 شماره 4
صفحات 164- 172
تاریخ انتشار 2013-12
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