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Background: The treatment of bleeding disorders improved in the last decades. However, the effect of growing up with bleeding disorders on developmental, emotional, and social aspects is understudied. Therefore, this study assesses HRQOL, developmental milestones, and self-esteem in Dutch young adults (YA) with bleeding disorders compared to peers.Methods: Ninety-five YA (18-30 years) with bleeding disorders (78 men; mean 24.7 years, SD 3.5) and 17 women (mean 25.1 years, SD 3.8) participated and completed the Pediatric Quality of Life Inventory Young Adult version, the Course of Life Questionnaire, and the Rosenberg Self-Esteem Scale. Differences between patients with bleeding disorders and their peers, and between hemophilia severity groups, were tested using Mann-Whitney U tests.Results: YA men with bleeding disorders report a slightly lower HRQOL on the total scale, physical functioning, and school/work functioning in comparison to healthy peers (small effect sizes). YA men with severe hemophilia report more problems on the physical functioning scale than non-severe hemophilia. YA men with bleeding disorders achieved more psychosexual developmental milestones than peers, but show a delay in 'paid jobs, during middle and/or high school.' A somewhat lower self-esteem was found in YA men with bleeding disorders in comparison to peers (small effect size). For YA women with bleeding disorders, no differences were found on any of the outcomes in comparison to peers.Conclusion: This study demonstrates some impairments in HRQOL and self-esteem in YA men with bleeding disorders. By monitoring HRQOL, problems can be identified early, especially with regard to their physical and professional/school functioning. [ABSTRACT FROM AUTHOR]

The article focuses on findings of a study on use of sensor-based gait analysis as a simple tool to measure gait in hemophilia patients. It states that the method is portable, inexpensive and fast and can be performed during regular outpatient visits, and mentions that gait analysis was performed with six inertial measurement units (IMU) with Velcro straps. It notes that gait parameters included pelvis, hip, femur, knee and tibia ROM in the sagittal plane.

Introduction: Desmopressin is frequently used in patients with bleeding disorders because of its prohaemostatic effects. In recent years desmopressin use increased due to reported high incidence of inhibitors in mild haemophilia after clotting factor infusion and the rising costs of clotting factor concentrates. The safety and frequency of side effects have hardly been assessed in well-designed studies. Aim: We therefore prospectively evaluated side effects of desmopressin in a large unselected cohort of bleeding disorder patients, who received a desmopressin test dose. Methods: Blood was drawn prior to, one, three, six and 24 h after desmopressin. Primary outcome was change in serum sodium, haematocrit, serum- and urine osmolality, body weight and vital signs. Self-reported side effects were evaluated as secondary outcome. Results: In total, 108 patients were included, median age 30 years, the majority of whom had von Willebrand disease type 1 (76%). A significant change in water balance parameters was observed. Four patients (4%) had hyponatraemia (≤135 mmol L−1) after 24 h but no severe hyponatraemia occurred (≤125 mmol L−1). After infusion, 41 (38%) patients were hypotensive (≤90 mmHg SBP and/or ≤60 mmHg DBP) and 10 (9%) presented with tachycardia (>100 min−1). However, none of these effects sustained at 24 h. Infusion was discontinued in one patient because of tachycardia, nausea and malaise. Self-reported side effects included: headache, fatigue, flush and dizziness. Conclusion: Observed side effects correspond with the known antidiuretic and vasomotor effects of desmopressin. Changes in parameters were temporary and not clinically relevant. In conclusion, our study supports desmopressin use as a safe treatment option in patients with various bleeding disorders. [ABSTRACT FROM AUTHOR]

The prevalence of obesity in patients with haemophilia ( PWH) is increasing. We investigated the effect of obesity on bleeding frequency and clotting factor concentrate ( CFC) usage in PWH and assessed whether prothrombotic changes observed in obesity differ between controls and PWH. Number of bleeds and CFC usage were compared between obese ( N = 51) and non-obese ( N = 46) haemophilia A patients. Markers of haemostasis and fibrinolysis were compared between PWH, and gender-, age- and body mass index ( BMI)-matched non-haemophilic controls ( N = 91). Median number of bleeds/patient-month was comparable between obese and non-obese patients with severe haemophilia ( P = 0.791). Obese patients with severe haemophilia used 1.4 times more CFC/patient-month than non-obese patients ( P = 0.036). When adjusting for weight this difference disappeared ( P = 0.451). von Willebrand factor plasma concentration ( VWF:Ag), factor VIII activity and endogenous thrombin potential were higher in obese than in non-obese controls. Obesity did not influence these markers in PWH. Plasminogen activator inhibitor type 1 levels were higher in obese vs. non-obese PWH ( P < 0.001), whereas levels were comparable between PWH and controls ( P = 0.912). Plasmin-α2-antiplasmin complex ( PAP) levels appeared to be lower in obese vs. non-obese subjects, both within controls ( P = 0.011) and PWH ( P = 0.008). However, in PWH, PAP levels were higher than in controls ( P < 0.001). Obesity is associated with an increase in net CFC usage in PWH, but has no effect on bleeding frequency. In addition, obesity attenuates hyperfibrinolysis in PWH. Future research investigating whether obese PWH need CFC treatment dosed on weight or whether a lower dosage would suffice to prevent and treat bleedings is needed. [ABSTRACT FROM AUTHOR]