User:Arijit Biswas
From Proteopedia
The Factor XIII homepage
Coagulation factor XIII (FXIII), is the last enzyme to be activated in the blood coagulation pathway and functions mostly to cross-link alpha- and gamma-fibrin chains, resulting in a stronger clot with an increased resistance to fibrinolysis. Acquired FXIII deficiency in most cases is associated with sepsis, oncohaematological and liver disorders, inflammatory bowel disease, disseminated intravascular coagulation (DIC) syndrome, major surgery, and very rarely because of FXIII inhibitors. On the other hand Inherited FXIII deficiency is rare phenomenon affecting only one out of 2.000.000 individuals. The prevalence of inherited FXIII deficiency is higher in countries where the consanguineous marriages are common. Patients suffering from severe FXIII deficiency are characterized by lifeālong spontaneous bleeding diathesis, impaired wound healing and high rate of spontaneous abortions.