van der Net J, Vos R C, Engelbert R H H, van den Berg M H, Helders P J M, Takken T
Department of Paediatric Physiotherapy and Paediatric Exercise Physiology, University Children's Hospital, UMC Utrecht, The Netherlands.
Haemophilia. 2006 Sep;12(5):494-9. doi: 10.1111/j.1365-2516.2006.01307.x.
In the Netherlands comparable levels of sports-participation between persons with haemophilia and healthy controls have been reported. This raises the question if children with haemophilia under the currently available prophylaxis do reach comparable levels of physical fitness and health-related quality of life (HRQoL) as their healthy peers. The aim of this study was to investigate the level of physical fitness, functional ability and quality of life and to determine the feasibility to safely test the exercise capacity of boys with severe haemophilia A. Thirteen subjects participated in this study. Physical fitness was determined using the measurement of maximal oxygen uptake (VO2peak) attained during a graded maximal exercise test to volitional exhaustion. Joint health, physical activity levels and health-related quality of life (Haemo-Qol) were also measured. Mean VO2peak was 1.86+/-0.77 L min-1 (Z-score: -0.39+/-1.61) which was not significantly different from reference values. Relative VO2peak was 47.42+/-8.29 mL min-1 kg-1 (Z-score: -0.52+/-1.43), which did not differ significantly from reference values either. One boy suffered a joint bleeding one day after the test. Haemo-Qol scores in parents and children ranged from 3.2% to 36.7% (100% reflects poor outcome). Relationship between the child or parent reports of Haemo-QoL and both absolute and relative VO2peak ranged from R=0.00 and R=0.4. Exercise testing in children with severe haemophilia A was a safe procedure. Patients with severe haemophilia A with good joint health and no limitations of activities have comparable physical fitness and physical active lifestyle with healthy peers and good HRQoL.
在荷兰，有报告称血友病患者与健康对照者的体育参与水平相当。这就引发了一个问题：在目前可用的预防措施下，血友病患儿的身体素质和与健康相关的生活质量（HRQoL）是否能达到与其健康同龄人相当的水平。本研究的目的是调查身体素质、功能能力和生活质量水平，并确定安全测试重度甲型血友病男孩运动能力的可行性。13名受试者参与了本研究。通过在分级最大运动试验中测量达到自愿疲劳时的最大摄氧量（VO2peak）来确定身体素质。还测量了关节健康状况、身体活动水平和与健康相关的生活质量（Haemo-Qol）。平均VO2peak为1.86±0.77 L min-1（Z分数：-0.39±1.61），与参考值无显著差异。相对VO2peak为47.42±8.29 mL min-1 kg-1（Z分数：-0.52±1.43），也与参考值无显著差异。一名男孩在测试后一天发生了关节出血。父母和孩子的Haemo-Qol评分在3.2%至36.7%之间（100%表示不良结果）。儿童或父母报告的Haemo-QoL与绝对和相对VO2peak之间的关系范围为R = 0.00至R = 0.4。对重度甲型血友病患儿进行运动测试是一种安全的程序。关节健康良好且活动无限制的重度甲型血友病患者与健康同龄人具有相当的身体素质和积极的生活方式，以及良好的HRQoL。
