In this cross-sectional study, 30 patients with hemophilia A and B who were registered at the Hemophilia Center of Shiraz, Fars Province, southern Iran, were investigated between March and October of 2013. The data collection form consisted of two parts including demographic data and 22 specific questions regarding assessment of knowledge of the patients regarding the disease and treatment. In ...

Hemophilia B is a genetic disorder that is characterized by a deficiency of clotting factor IX (FIX) and excessive bleeding. Advanced understanding of the pathophysiology of the disease has led to the development of improved treatment strategies that aim to minimize the acute and long-term complications of the disease. Patients with hemophilia B are ideal candidates for gene therapy, mostly bec...

BACKGROUND Electronic health records (EHRs) are an important source of information with regard to diagnosis and treatment of rare health conditions, such as congenital hemophilia, a bleeding disorder characterized by deficiency of factor VIII (FVIII) or factor IX (FIX). OBJECTIVE To identify patients with congenital hemophilia using EHRs. DESIGN An EHR database study. SETTING EHRs were ac...

Hemophilia A is a hereditary disorder caused by various mutations in factor VIII gene resulting in either a severe deficit or total lack of the corresponding activity. Recent success in gene therapy of a related disease, hemophilia B, gives new hope that similar success can be achieved for hemophilia A as well. To develop a gene therapy strategy for the latter, a variety of model systems are ne...

Hemophilia A is an inherited bleeding disorder caused by deficiency of coagulation factor VIII. It transmitted in X-linked recessive pattern from female carriers to male children. We report the observation a severe hemophilia newborn with history siblings who present hemarthrosis left elbow. The diagnosis was suspected and confirmed through biologic investigations imaging. Therefore, patient pu...

Hemophilia A and B are X-chromosome linked recessive bleeding disorders that result from a deficiency in factor VIII (FVIII) and factor IX (FIX) respectively. Though factor substitution therapy has greatly improved the lives of hemophiliac patients, there are still limitations to the current treatment that have triggered interest in alternative treatments by gene therapy. Significant progress h...

Electroconvulsive therapy (ECT) is used for medication-resistant and life-threatening mental disorders, and therefore it occupies an important position in psychiatric treatment. ECT reportedly increases intracranial pressure and is suspected of increasing the risk of intracranial hemorrhage, especially in patients with hemorrhagic diseases such as hemophilia. A decrease in or loss of blood coag...

Hemophilia represent the most severe inherited bleeding disorder (INB) , it’s thought to affect inviduals from all geographical areas in equal frequency. In Egypt which has a population of approximately (80million) consanguineous marriage are frequent, therefore autosomal recessive coagulation disorders reach a higher prevalence than in many other countries. The primary aim of this study was to...

This prospective, Post-Authorization Safety Surveillance (PASS) study was carried out in patients with hemophilia A or B and inhibitors treated with FEIBA for 1 year to collect real-world data on safety and effectiveness of FEIBA. The study followed a cohort design and did not make stipulations on treatment or observation schedule, as it was designed to observe routine medical practices based o...

Approximately 25% of patients with hemophilia A develop inhibitory antibodies after treatment with factor VIII. Most of the inhibitory activity is directed against epitopes in the A2 and C2 domains. Anti-A2 inhibitory antibodies recognize a 25-residue segment bounded by R484-I508. Several antigenic residues in this segment have been identified, including R484, R489, and P492. The immunogenicity...