Factor XIII deficiency: a review of literature

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 freque...

factor xiii deficiency, review of the literature and report of a case

conclusions the case was fully investigated and diagnosed as a case with factor xiii deficiency causing such uncontrolled bleeding after extraction of a tooth. introduction factor xiii deficiency is a relatively rare hereditary bleeding disorder, which is usually found during infancy or early childhood. this condition may involve both genders within different races in an equal manner. its incid...

Acquired factor XIII deficiency due to an inhibitor: a case report and review of the literature.

Current understanding in diagnosis and management of factor XIII deficiency

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 resis...

Factor XIII deficiency.

Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder that can present with umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding and life-threatening intracranial haemorrhage. FXIII deficiency has also been associated with poor wound healing and recurrent miscarriages. FXIII plays an integral role in haemostasis by catalysing the cross-lin...

Factor XIII deficiency in Pakistan.

Patients with undiagnosed haemostatic defects seen at The Aga Khan Hospital and Fatimid Blood Transfusion Centre during the period of 7 years (1985-1992) were screened with routine tests including bleeding time (BT), whole blood clotting time (CT), platelet count, activated partial thromboplastin time (APTT), prothrombin time (PT) and 5 molar urea test. Nine patients had a positive 5 molar urea...