University of Southern California, CA 90033, USA.
Haemophilia. 2012 Sep;18(5):699-707. doi: 10.1111/j.1365-2516.2012.02791.x. Epub 2012 Apr 17.
This study describes health-related quality of life (HRQoL) of persons with haemophilia A in the United States (US) and determines associations between self-reported joint pain, motion limitation and clinically evaluated joint range of motion (ROM), and between HRQoL and ROM. As part of a 2-year cohort study, we collected baseline HRQoL using the SF-12 (adults) and PedsQL (children), along with self-ratings of joint pain and motion limitation, in persons with factor VIII deficiency recruited from six Haemophilia Treatment Centres (HTCs) in geographically diverse regions of the US. Clinically measured joint ROM measurements were collected from medical charts of a subset of participants. Adults (N = 156, mean age: 33.5 ± 12.6 years) had mean physical and mental component scores of 43.4 ± 10.7 and 50.9 ± 10.1, respectively. Children (N = 164, mean age: 9.7 ± 4.5 years) had mean total PedsQL, physical functioning, and psychosocial health scores of 85.9 ± 13.8, 89.5 ± 15.2, and 84.1 ± 15.3, respectively. Persons with more severe haemophilia and higher self-reported joint pain and motion limitation had poorer scores, particularly in the physical aspects of HRQoL. In adults, significant correlations (P < 0.01) were found between ROM measures and both self-reported measures. Except among those with severe disease, children and adults with haemophilia have HRQoL scores comparable with those of the healthy US population. The physical aspects of HRQoL in both adults and children with haemophilia A in the US decrease with increasing severity of illness. However, scores for mental aspects of HRQoL do not differ between severity groups. These findings are comparable with those from studies in European and Canadian haemophilia populations.
本研究描述了美国（美国）血友病 A 患者的健康相关生活质量（HRQoL），并确定了自我报告的关节疼痛、运动受限与临床评估的关节活动范围（ROM）之间的关联，以及 HRQoL 与 ROM 之间的关联。作为一项为期两年的队列研究的一部分，我们从美国地理上不同地区的六个血友病治疗中心（HTC）招募了因子 VIII 缺乏症患者，收集了基线 HRQoL 数据，使用 SF-12（成人）和 PedsQL（儿童），以及自我报告的关节疼痛和运动受限程度。从参与者的病历中收集了临床测量的关节 ROM 测量值。成年人（N = 156，平均年龄：33.5 ± 12.6 岁）的身体和心理成分得分分别为 43.4 ± 10.7 和 50.9 ± 10.1。儿童（N = 164，平均年龄：9.7 ± 4.5 岁）的总 PedsQL、身体功能和心理社会健康得分分别为 85.9 ± 13.8、89.5 ± 15.2 和 84.1 ± 15.3。患有更严重血友病、自我报告关节疼痛和运动受限程度较高的患者得分较差，尤其是在 HRQoL 的身体方面。在成年人中，ROM 测量值与自我报告的测量值之间存在显著相关性（P <0.01）。除了病情严重的患者外，患有血友病的儿童和成年人的 HRQoL 评分与美国健康人群相当。美国血友病 A 患者的身体方面的 HRQoL 随着疾病严重程度的增加而降低。然而，在严重程度组之间，心理方面的 HRQoL 评分没有差异。这些发现与欧洲和加拿大血友病人群的研究结果相似。
