Menorrhagia is a common presentation of bleeding disorders, especially vWD in women. The frequency of these disorders in women with menstruation problems is determined in this study. Total of 273 patients in reproductive age with menorrhagia were investigated for bleeding disorders in two steps. Step one includes CBC, PT, PTT and BT tests which were performed for all patients; patients diagnose...

Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal ...

Factor X deficiency is a severe rare hemorrhagic condition inherited as an autosomal recessive trait. It is one of the most severe recessive inherited coagulation disorders. We analyzed the clinical manifestations, laboratory phenotype and genotype in 10 patients with severe Factor X deficiency and in their heterozygous relatives. The most frequent bleeding episodes were hematomas (70%) and gum...

During the transitioning through puberty, adolescents present with varied gynaecological issues, among which puberty menorrhagia is a significant complaint. The most common underlying cause of puberty menorrhagia is anovulation, other causes being endocrine dysfunction, PCOS and bleeding disorders. Congenital prothrombin deficiency is an extremely rare inherited coagulopathy, affecting one in t...

Massive hemoperitoneum secondary to ruptured corpus luteum is a rare but serious and life-threatening complication for women with congenital bleeding disorders and may lead to surgical interventions and even oophorectomy. Congenital afibrinogenemia is a rare inherited coagulation disorder. As it can be asymptomatic, its clinical manifestations vary from minimal tendency of bleeding to life-thre...

Background: Recombinant activated factor VII induces hemostasis in patients with coagulopathy disorders. AryoSeven™ as a safe Iranian Recombinant activated factor VII has been available on our market. This study was performed to establish the safety of AryoSeven on patients with coagulopathy disorder. Methods: This single-center, descriptive,...

Adults with bleeding disorders may present to their family physician with minor bleeding symptoms or hematologic laboratory abnormalities discovered during evaluation for surgery or another purpose. Identifying the small proportion of adults who have an underlying bleeding disorder as the cause for such signs or symptoms may be challenging. In cases of asymptomatic hematologic laboratory abnorm...

BACKGROUND Though rare in occurrence, patients with rare bleeding disorders (RBDs) are highly heterogeneous and may manifest with severe bleeding diathesis. Due to the high rate of consanguinity in many caste groups, these autosomal recessive bleeding disorders which are of rare occurrence in populations across the world, may not be as rare in India. OBJECTIVES To comprehensively analyze the ...

Rare bleeding disorders account for about 3–5% of all inherited disorders. Due to the rarity and complexity diagnosing these disorders, their prevalence estimates vary greatly. There is currently no national registry rare across country has not been studied yet. Aim: estimate coagulation among Russian children. For this multicenter study, we used retrospective anonymous patient data collected d...

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...