http://repub.eur.nl/res/aut/42871/ List of Publications en http://repub.eur.nl/static-eur/img/logo.png http://repub.eur.nl http://repub.eur.nl/res/pub/31953/ 2012-01-01T00:00:00Z Summary. Background: Hemophilia A patients have a lower cardiovascular mortality rate than the general population. Whether this protection is caused by hypocoagulability or decreased atherogenesis is unclear. Objectives: To evaluate atherosclerosis and endothelial function in hemophilia A patients with and without obesity as well as in matched, unaffected controls. Methods: Fifty-one obese (body mass index [BMI]≥30kgm-2) and 47 non-obese (BMI≤25kgm-2) hemophilia A patients, and 42 obese and 50 matched non-obese male controls were included. Carotid and femoral intima-media thickness [IMT] and brachial flow-mediated dilatation (FMD) were measured as markers of atherogenesis and endothelial function. Results: The overall population age was 50±13 years. Carotid IMT was increased in obese subjects (0.77±0.22mm) as compared with non-obese subjects (0.69±0.16mm) [mean difference 0.07mm (95% confidence interval [CI] 0.02-0.13, P=0.008)]. No differences in mean carotid and femoral IMT between obese hemophilic patients and obese controls were found (mean difference of 0.02mm [95% CI -0.07-0.11, P=0.67], and mean difference of 0.06mm [95% CI -0.13-0.25, P=0.55], respectively). Thirty-five per cent of the obese hemophilic patients and 29% of the obese controls had an atherosclerotic plaque (P=0.49), irrespective of the severity of hemophilia. Brachial FMD was comparable between obese hemophilic patients and obese controls (4.84%±3.24% and 5.32%±2.37%, P=0.45). Conclusion: Hemophilia A patients with obesity develop atherosclerosis to a similar extent as the general male population. Detection and treatment of cardiovascular risk factors in hemophilic patients is equally necessary. http://repub.eur.nl/res/pub/29058/ 2008-02-15T00:00:00Z The introduction of replacement therapy in the 1960s has improved medical and social circumstances gradually. The availability of prophylactic treatment has further increased the possibilities of a "normal" life for patients with hemophilia. We examined whether social participation and health-related quality of life (HRQol) of today's hemophilia patients differs from the general male population. There were a total of 721 participants in the Hemophilia in the Netherlands 5 study (HiN-5 study) ages 16 to 64 years. Patients with severe hemophilia participated less in full-time work compared with the general population. Occupational disability was reported by 35% of patients with severe hemophilia between ages 31 and 64 years, compared with 9% in the general population. HRQol of patients with severe hemophilia between ages 31 and 64 years was lower than of the general population. The differences with the general population in HRQol were least pronounced for patients between ages 16 and 30 years. Despite major improvements in treatment during the last decades, patients with hemophilia are still less involved in full-time paid work and suffer more from occupational disability than men from the general population. After the introduction of prophylactic treatment, the number of patients who are occupationally disabled is reduced.