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 X deficiency (also known as Stuart-Prower factor deficiency) is an extremely rare hereditary hematologic disorder, affecting 1 person in 2 million. The gene causing this condition is autosomal recessive; thus, only those inheriting from both parents exhibit clinical symptoms, such as moderate bleeding, easy bruising and subcutaneous bleeding from mucous membranes. Patients with marked de...

Background and Aims: Human T-lymphotropic virus (HTLV) is a human retrovirus which has been known to cause adult T-cell leukemia/lymphoma and some other inflammatory disorders. Patients with hereditary bleeding diseases are at high risk for these viruses. In this study, we evaluated serological HTLV-I/II infection among these patients in west Azerbaijan of Iran. Material and Methods: We stud...

Factor XI deficiency (Hemophilia C) is a very rare autosomal recessive bleeding disorder. Patients with factor XI deficiency do not typically show any spontaneous bleeding or specific symptoms. Sometimes those who have this disorder are identified during special situations such as trauma or surgery. Orthognathic surgery is particularly associated with a high bleeding risk. Therefore, great care...

Fibrinogen, a hexameric glycoprotein encoded by three genes - FGA, FGB, FGG - clustered on chromosome 4q is involved in the final steps of coagulation as a precursor of fibrin monomers required for the formation of the haemostatic plug. Inherited disorders of fibrinogen abnormalities are rare and not as well clinically characterized as some other inherited bleeding disorders. To characterize th...

Whereas many healthy people consider their bleeding and bruising excessive, patients with underlying von Willebrand disease, the most common hereditary bleeding disorder, often fail to identify their bleeding symptoms. Therefore, it is necessary to ask for specific information from patients about bleeding and bruising (Is the patient easily bruised? What is the size of the bruises? If the patie...

Introduction: Hereditary Gingival Enlargement (HGF), a rare entity, is also known as familial elephantiasis, elephantiasis gingivae, diffuse fibromatosis. It benign, non-haemorrhagic fibrous enlargement of gingival tissue. frequently component feature many syndromes. Jones syndrome one such syndrome, characterized by overgrowth and progressive deafness.
 Case report: A 27-year-old male pat...