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...

development of inhibitor to factor viii is the most serious complication of hemophilia therapy. to determine the prevalence of inhibitors in iran hemophilia a patients exposed to blood products, 1280 hemophilia a patients (age range 9 months-84 years) were evaluated. all patients received several blood products such as fresh frozen plasma (ffp), cryoprecipitate, and factor viii. 635 of 1280 pat...

After the increasing rate of deaths observed during the 1980s due to human immunodeficiency virus (HIV) infection, the health-related quality of life and life expectancy of persons with hemophilia have improved, mainly due to the progresses of replacement therapy and antiviral drugs and to the improvement of the global comprehensive care provided by specialized centers. As a consequence, an inc...

Of the several clinical issues surrounding therapeutic choices for hemophilia patients, prophylactic therapy stands out as the most positive. The first study comparing prophylactic and on-demand treatment , which involved 22 years of follow-up, found that the primarily prophylactic treatment strategy led to better outcome at equal treatment costs in young adults with severe hemophilia. 1 In con...

A wide range of factor VIII and IX levels is observed in heterozygous carriers of hemophilia as well as in noncarriers. In female carriers, extreme lyonization may lead to low clotting factor levels. We studied the effect of heterozygous hemophilia carriership on the occurrence of bleeding symptoms. A postal survey was performed among most of the women who were tested for carriership of hemophi...

Background: Improper management of bleeding disorders in the dental setting can lead to complications that endanger lives such patients. Objective: This study aimed assess knowledge and awareness hemophilia among undergraduate students a Malaysian university. Methodology: A cross-sectional survey was conducted year 2018-2019 collect data. 23-item questionnaire developed validated by authors use...

Low bone density is a growing concern in aging men with hemophilia and may result in high-morbidity fragility fractures. Using high-resolution peripheral quantitative computed tomography (HR-pQCT), we demonstrate low trabecular and cortical bone density contributing to lower volumetric bone mineral density (BMD) at both distal radius and tibia in patients with hemophilia compared with age- and ...

BACKGROUND Hemophilia is a potentially disabling condition as hemophilic arthropathy develops early in life and is progressive, especially in patients treated in an on-demand regime. OBJECTIVE This study aimed to describe the structural joint status and the functional independence score of hemophiliac adults and correlate structural damage with the functional deficits found in these patients....

Background: Considering the increasing number of patients with hemophilia and infrastructure requirements for a comprehensive approach, development of a recombinant factor has become a milestone. The objective of this study was to assess the safety, efficacy and non inferiority of Safacto (Recombinant factor VIII) compared with plasma-derived factor in the treatment of hemophilia A. Methods: 10 ...