abstract background the purpose of this study was to assess the prevalence of major depressive and anxiety disorders in hemophilic and major beta thalassemic patients related to education of their mothers as a family’s agent. materials and methods a case-control study was performed on 34 major beta thalassemic patients. for each patient the control group was selected and matched (with age and s...

INTRODUCTION New longer-acting factor products will potentially allow for less frequent infusion in prophylactic treatment of hemophilia. However, the role of administration frequency relative to other treatment attributes in determining preferences for prophylactic hemophilia treatment regimens is not well understood. AIM To identify the relative importance of frequency of administration, ef...

The ability of factor VIIa to initiate thrombin generation and clot formation in blood from healthy donors, blood from patients with hemophilia A, and in anti-factor IX antibody-induced ("acquired") hemophilia B blood was investigated. In normal blood, both factor VIIa-tissue factor (TF) complex and factor VIIa alone initiated thrombin generation. The efficiency of factor VIIa was about 0.0001 ...

background: hemophilia b is an x-linked recessive coagulation disorder caused by factor ix deficiency.  analysis of factor ix gene polymorphisms is considered the best approach for prenatal diagnosis and carrier detection of hemophilia b where the identification of gene mutation is not easily possible.   objective: to study the frequency of three factor ix-linked restriction fragment length pol...

The ability of factor VIIa to initiate thrombin generation and clot formation in blood from healthy donors, blood from patients with hemophilia A, and in anti–factor IX antibody–induced (“acquired”) hemophilia B blood was investigated. In normal blood, both factor VIIa–tissue factor (TF) complex and factor VIIa alone initiated thrombin generation. The efficiency of factor VIIa was about 0.0001 ...

background bone marrow transplantation (bmt)is nowadays used in various hematological disorders including leukemias. hemophilia a & b are sex linked bleeding disorders in which there are various genetic abnormalities in factor viii & ix genes. among various hematological disorders, bleeding disorders mainly hemophilia in now widely treated using plasma derived and recombinant factor viii & ix c...

Heterogeneous loss of function mutations at F8 and F9 genes causes X-linked recessive bleeding disorders, hemophilia A (HA) and hemophilia B (HB), respectively. HA is clinically indistinguishable from HB and accounts for more than 80% of hemophilia cases; the former affects 1/5000 and the latter 1/25000 male births worldwide. In Iran, it is estimated that around 4300 HA and 900 HB patients are ...

Result: Thirty-one patients (48.4%) were diagnosed with hemophilia A,eighteen patients (28.1%) had von Willebrand disease, eleven patients (17.9%) had Hemophilia B and four patients had other factor deficiencies. Elevenpatients with Hemophilia A and one patient with Hemophilia B had severe disease. The prevalence of hepatitis C virus infection was 5%. All patients were negative for hepatitis B ...

BACKGROUND Although hemophilia has a potentially high economic impact, there are no published estimates of healthcare costs for this disease in Portugal. The aim of this study was to evaluate costs of treatment and hospital utilization among patients with hemophilia A and B, with and without inhibitors, over a 3-year period in a Portuguese Comprehensive Care Hemophilia Centre. This is the first...

Hemophilia is an X-linked recessive bleeding disorder caused by deficiency of FVIII (hemophilia A) or FIX (hemophilia B). Mutations in the FVIII or FIX genes, both located on the long arm of the X chromosome, are detectable in the majority of cases of hemophilia A or B, respectively. During normal hemostasis, FVIII and FIX form an enzymatic complex; thus, deficiency of either protein leads to a...