• Objective: To determine causes of death and mortality rates in patients with hemophilia over a period of 20 years, to assess changes in mortality, and to distinguish between hemophilia-related death and recent death induced by viral infections. • Design: Cohort study of 919 patients followed from January 1986 to June 1992. Results were compared with outcomes of previous follow-up from 1973 to...

Introduction. Hemophilia is an X-linked hereditary blood clotting disorder caused by insufficiency of factor VIII or IX that affects mainly men. In extremely rare cases, the disease can be observed in women, which most often associated with asymmetric inactivation X chromosome. The severity hemophilia women does not differ from Aim – to present a clinical observation surgical treatment stage 4 ...

Hemophilia B is a recessive X-linked bleeding disorder characterized by deficiency of the coagulation factor IX (FIX). In hemophilia B patients the severity of the bleeding phenotype is related to the degree of the FIX defect. Hemophilia B treatment has improved greatly in the last 20 years with the introduction first of plasma-derived and then of recombinant FIX concentrates. Replacement thera...

Since the 1970s, mortality in the hemophilia population has been dominated by human immunodeficiency virus (HIV) and few reports have described mortality in uninfected individuals. This study presents mortality in 6018 people with hemophilia A or B in the United Kingdom during 1977 to 1998 who were not infected with HIV, with follow-up until January 1, 2000. Given disease severity and factor in...

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...

Chronic joint disease from repeated bleeding into joints is a serious complication of hemophilia. To measure the extent of and to identify risk factors for deviations from normal in joint range of motion (ROM), we used crosssectional data collected from 4343 males with hemophilia aged 2 to 19 years who received care at 136 US hemophilia treatment centers (HTCs). Factors examined included age, r...

BACKGROUND Studies on health-related quality of life are based on the increasingly evident need for medical care not to be limited to preventing death, but to focus instead on the value of health. OBJECTIVE This study aimed to measure the health-related quality of life in hemophilia, using the Hemophilia- Specific Quality of Life (Haem-A-QoL) questionnaire and describe the socioeconomic chara...

Chronic joint disease from repeated bleeding into joints is a serious complication of hemophilia. To measure the extent of and to identify risk factors for deviations from normal in joint range of motion (ROM), we used cross-sectional data collected from 4343 males with hemophilia aged 2 to 19 years who received care at 136 US hemophilia treatment centers (HTCs). Factors examined included age, ...

bypassing agents are the most commonly used medicines for the treatment of hemophilia patients with inhibitors. the aim of this study is to identify the cost components of management of bleeding vents in hemophilia patients with inhibitors in iran. this study is a cross-sectional study using a bottom-up approach to determine the cost components of treatment of hemophilia patients with inhibitor...

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...