Paediatric Haemophilia Centre, Dr. von Hauner Children's Hospital, LMU Munich, Munich, Germany.
Department of Paediatrics, Paediatric Haemophilia Centre, Goethe University, University Hospital Frankfurt, Frankfurt, Germany.
Hamostaseologie. 2019 Nov;39(4):355-359. doi: 10.1055/s-0039-1677874. Epub 2019 Feb 5.
Similar to the general population, overweight and obesity have increasingly become a medical and economic burden also in patients with haemophilia in industrialized nations. In this study in seven German haemophilia centres, we identified a prevalence of overweight and obesity of 25.2% among 254 young patients <30 years (median: 13 years; range: 0-30 years) with severe haemophilia A and without a history of inhibitors. The median FVIII dosage based on bodyweight was significantly higher in normal weight compared with overweight or obese patients (96.9 vs. 72.9 IU/kg/week, respectively; < 0.0001). This suggests that an individualized dosing regime which might be based on FVIII pharmacokinetics, physical activity and pre-existing haemophilic arthropathy is applied rather than dosing by bodyweight only. The bleeding rates observed in obese (median: 1; range: 0-17) versus normal weight patients (median: 2; range: 0-28) did not differ significantly ( = 0.057). Lower bleeding rates might be due to reduced activity or expected higher FVIII plasma levels in overweight patients. Due to the increasing prevalence of overweight/obesity in patients with haemophilia an interdisciplinary approach for individualized haemophilia treatment and weight loss programmes might be helpful for optimal and economical treatment for this group of patients.
类似于普通人群,超重和肥胖也越来越成为工业化国家血友病患者的医疗和经济负担。在这项对七个德国血友病中心的研究中,我们发现 254 名年龄<30 岁(中位数:13 岁;范围:0-30 岁)、无抑制剂史的重度血友病 A 年轻患者中,超重和肥胖的患病率为 25.2%。与超重或肥胖患者相比(分别为 96.9 和 72.9 IU/kg/周;<0.0001),正常体重患者的基于体重的 FVIII 剂量中位数显著更高。这表明,应用了基于 FVIII 药代动力学、体力活动和已存在的血友病性关节炎的个体化给药方案,而不仅仅是根据体重给药。肥胖患者(中位数:1;范围:0-17)和正常体重患者(中位数:2;范围:0-28)的出血率无显著差异( = 0.057)。较低的出血率可能是由于超重患者活动减少或预期 FVIII 血浆水平升高所致。由于血友病患者超重/肥胖的患病率不断增加,针对该人群的个体化血友病治疗和减肥计划的跨学科方法可能有助于为该组患者提供最佳和经济的治疗。