PTH6: PATIENTS' PERCEPTIONS OF THE OUTCOMES OF TREATMENT WITH TOPICAL NSAIDS, RUBIFACIANTS AND ORAL PAIN REMEDIES. A PROSPECTIVE STUDY OF MEDICINES AVAILABLE THROUGH COMMUNITY PHARMACIES

Obesity is a major health concern not only in the general population but also in patients with haemophilia. Little is known about the consequences of obesity for haemophilia patients. As obesity is an important risk factor for osteoarthritis, these effects may be even more pronounced in haemophilia patients who are prone to joint damage. The association between obesity and limitations in daily activities as well as the frequency of bleeds and use of factor VIII (FVIII) concentrate in obese and normal weight haemophilia patients was assessed. Fifteen obese (BMI≥30kgm-2) and fifteen normal weight (BMI≤25kgm-2) haemophilia A patients matched for severity and age were analysed. The Hemophilia Activities List (HAL) was used to assess the impairment in daily activities. Compared with the normal weight haemophilia patients, obese haemophiliacs had a significantly lower sum score (88/100 and 98/100, respectively, P=0.02), which was mainly caused by an impaired lower limb function. All other components of the HAL also showed lower scores in the obese patients, but did not reach statistical significance. A higher frequency of bleeds requiring treatment with FVIII concentrate occurred in the obese haemophiliacs (17 bleeds in eight individuals) compared with the controls (three bleeds in three individuals) (P=0.045). Compared with non-obese haemophilia patients, obese haemophiliacs had more joint bleeds and a lower overall HAL score, which was driven by a lower limb function score. Prevention of overweight and weight reduction requires special attention from physicians treating haemophilia patients. © 2011 Blackwell Publishing Ltd

Cardiovascular risk assessment in haemophilia patients

Haemophilia patients have a reduced cardiovascular mortality, which may be the result of a lifelong deficiency of factor VIII or IX. On the other hand, the prevalence of risk factors may differ in these chronically ill patients compared to the general population. The prevalence of risk factors and expected risk of cardiovascular disease was compared in haemophilia patients and healthy controls. In adult haemophilia A and B patients, body mass index, blood pressure, cholesterol levels and fasting glucose levels were measured and compared to healthy age-matched males. The expected risk of mortality due to cardiovascular disease was calculated using a European risk prediction algorithm (SCORE). A total of 100 haemophilia A and B patients and 200 healthy controls were analysed. The mean age of the patients was 47 years (range 18-83). The number of haemophiliacs with hyperglycaemia (24%) and hypertension (51%) was higher than in the controls (p-values 0.001 and 0.03, respectively). The mean low-density lipoprotein (LDL) cholesterol level in cases was lower than the controls (3.02 mM (0.69-6.57) and 3.60 mM (1.68-5.95), respectively, p 10% between cases and controls (12% and 7%, respectively, p = 0.18). The prevalence of risk factors and expected risk of cardiovascular disease in haemophilia patients is comparable to the general population. This strengthens the hypothesis that hypocoagulability may reduce cardiovascular mortality in haemophilia patients

Cardiovascular risk assessment in haemophilia patients

Item does not contain fulltextHaemophilia patients have a reduced cardiovascular mortality, which may be the result of a lifelong deficiency of factor VIII or IX. On the other hand, the prevalence of risk factors may differ in these chronically ill patients compared to the general population. The prevalence of risk factors and expected risk of cardiovascular disease was compared in haemophilia patients and healthy controls. In adult haemophilia A and B patients, body mass index, blood pressure, cholesterol levels and fasting glucose levels were measured and compared to healthy age-matched males. The expected risk of mortality due to cardiovascular disease was calculated using a European risk prediction algorithm (SCORE). A total of 100 haemophilia A and B patients and 200 healthy controls were analysed. The mean age of the patients was 47 years (range 18-83). The number of haemophiliacs with hyperglycaemia (24%) and hypertension (51%) was higher than in the controls (p-values 0.001 and 0.03, respectively). The mean low-density lipoprotein (LDL) cholesterol level in cases was lower than the controls (3.02 mM (0.69-6.57) and 3.60 mM (1.68-5.95), respectively, p 10% between cases and controls (12% and 7%, respectively, p = 0.18). The prevalence of risk factors and expected risk of cardiovascular disease in haemophilia patients is comparable to the general population. This strengthens the hypothesis that hypocoagulability may reduce cardiovascular mortality in haemophilia patients

The effect of haemophilia and von Willebrand disease on arterial thrombosis: A systematic review

Background: Patients with haemophilia and von Willebrand disease (VWD) may have a reduced cardiovascular mortality, due to a hypocoagulable state or decreased atherogenesis. We performed a systematic review to assess the association between haemophilia and VWD, and fatal and nonfatal arterial thrombosis and asymptomatic atherosclerosis. Methods: Medline and PubMed were searched to identify studies that assessed the incidence of cardiovascular mortality and morbidity in haemophilia and VWD, and that measured asymptomatic atherosclerosis with intima media thickness (IMT) of the carotid and femoral arteries, or flow-mediated dilatation (FMD) of the brachial artery. Weighted standardised mortality ratios (SMR) and mean differences (WMD) were calculated and pooled using a random effects model. Results: 15 longitudinal and cross-sectional studies consisting of 19,242 patients were included. Mortality due to arterial thrombosis was nonsignificantly reduced in patients with haemophilia compared with healthy controls (SMR 0.50, 95% CI 0.24 to 1.09). Haemophilia reduced nonfatal coronary events, and severe haemophilia offered better protection, but these results were based on a single study. No results were available for VWD. Although IMT of the carotid and femoral arteries was similar between VWD and haemophilia patients and healthy controls, atherosclerotic plaques of the large arteries were less prevalent in haemophilia patients. Only two studies assessed FMD and the results were inconsistent. Conclusion: Haemophilia may reduce arterial thrombosis, but this association should be further studied in haemophilia patients with a higher prevalence of cardiovascular risk factors

Obesity-related hemostatic changes in hemophilia patients

Introduction and Methods: The prevalence of obesity in patients with hemophilia (PWH) is increasing. We investigated whether hemostatic changes seen in obesity differ between men with and without hemophilia, by comparing coagulation and fibrinolytic markers between 98 hemophilia A patients (50 obese and 48 non-obese) and 92 age- and BMI-matched non-hemophilic men (42 obese and 50 non-obese). Results: Mean age was 50 ± 13 years and 33 PWH had FVIII activity <1%. The distribution of hemophilia severity was comparable between obese and non-obese groups. Mean FVIII activity and ETP were increased in obese controls as compared to non-obese controls (114 vs. 97% (P = 0.016) and 1756 vs. 1518 nM/min (P = 0.001), respectively). Obesity did not change F1 + 2 levels. In PWH, mean ETP and median F1 + 2 levels were lower than in controls (1272 vs. 1625 nM/min (P <0.001) and 120 vs. 153 pM (P <0.001), respectively). Obesity in PWH did not influence FVIII, ETP or F1 + 2 levels. Mean VWF:ag levels were higher in PWH as compared to controls (132 vs. 115%, P = 0.011), with the largest difference between obese PWH and non-obese controls (135 vs. 107%, P = 0.017). Higher median PAI-1 levels were observed in obese subjects than in non-obese (58 vs. 35 ng/mL, P <0.001). In accordance, median PAP levels were lower in obese vs. non-obese subjects (287 vs. 354 μg/L, P <0.001). The obesity-related increase in PAI-1 levels and decrease in PAP levels were comparable between PWH and controls. When comparing PWH with controls, PAI-1 levels were equal and PAP levels were higher in PWH (339 vs. 271 μg/L, P = 0.003). No differences in platelet count or D-dimer levels between any of the groups were found. Conclusion: In PWH a similar obesity-related decrease in fibrinolysis was seen as in non-hemophilic men. The influence of fibrinolytic changes in obese PWH on (athero) thrombosis and bleeding tendency deserves further attention

Hypocoagulability does not protect against atherosclerosis in hemophilia A patients with obesity

Introduction: Hemophilia A patients have a 50% lower cardiovascular mortality than the general population. Whether this is caused by less atherosclerosis due to hypocoagulability is unclear. We assessed whether hemophilia A patients with obesity, a major atherosclerotic risk factor, have a lower prevalence of 'subclinical' atherosclerosis than obese controls. Methods: Fifty obese (body mass index [BMI] > 30 kg/m2) and 48 normal-weight (BMI <25 kg/m2) hemophilia A patients, and 42 obese and 50 normal-weight male controls were included, matched for age, BMI and severity of hemophilia, as appropriate. In all subjects, carotid intima media thickness (IMT) and brachial flow mediated dilatation (FMD) were measured. Results: Mean age of subjects was 50 ± 13 years. IMT was increased in obese (0.76 mm) compared to non-obese subjects (0.69 mm), (D0.07 mm (95%CI 0.03-0.13 mm). IMT and FMD were comparable between obese hemophiliacs and obese controls. 40% of the hemophilia patients and 29% of controls had plaques (IMT > 1.3 mm, P = 0.11), irrespective of the severity of hemophilia. (Figure presented) Conclusion: Hemophilia A patients with obesity have the same degree of subclinical atherosclerosis as obese control subjects, which suggests that the lower cardiovascular mortality is likely caused by a decreased risk of arterial thrombosis

Factor VIII deficiency does not protect against atherosclerosis.

Item does not contain fulltextBACKGROUND: 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] >/= 30 kg m(-2)) and 47 non-obese (BMI </= 25 kg m(-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 +/- 0.22 mm) as compared with non-obese subjects (0.69 +/- 0.16 mm) [mean difference 0.07 mm (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 0.02 mm [95% CI ) 0.07-0.11, P = 0.67], and mean difference of 0.06 mm [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.1 januari 201