Lane H, Siddiqi A-E-A, Ingram-Rich R, Tobase P, Scott Ward R
Intermountain Hemophilia and Thrombosis Center, Primary Children's Hospital, Salt Lake City, UT, USA.
Haemophilia. 2014 Sep;20(5):709-15. doi: 10.1111/hae.12398. Epub 2014 Mar 16.
In persons with haemophilia (PWH), repeated ankle haemarthroses lead to pain, loss of joint range of motion (ROM), and limitations in activity and participation in society. PWH are offered ankle arthrodesis (AA) to eliminate pain. In our experience, PWH are hesitant to proceed to AA due to concerns regarding gait anomalies, functional decline and complete loss of ROM. The aim of this study was to report outcomes in ROM, assistive device (AD)/wheelchair use, activity scale and work/school absenteeism for participants in the CDC's Universal Data Collection surveillance project (UDC) pre- and post- AA. Males with haemophilia enrolled in the UDC with first report of AA (1998-2010) were selected. Descriptive statistics were calculated using data from the annual study visit pre-AA and the follow-up visit (~12-24 months) post-AA. The 68 subjects who fulfilled the criteria were: mean age 36.9 years (SD = 12.9); 85.3% white; 85.3% haemophilia A; 72% severe, 20.6% moderate; and 10.3% with inhibitor once during the study period. Mean loss in total arc of ankle motion was 17.02° (SD = 21.8, P ≤ 0.01) pre- compared to post-AA. For 61.8%, there was no change in use of AD for ambulation/mobility. For 85.3%, there was no change in use of a wheelchair. On a self-reported activity scale, 11.8% improved, 8.8% worsened and 79.4% did not change. Work/school absenteeism averaged 2.7 (SD = 6.4) pre- and 1.5 (SD = 6.4, P = 0.26) days per year post-AA. While ankle ROM was significantly reduced post-AA, for most subjects, there was no change in use of AD/wheelchair for ambulation/mobility. Physical activity was maintained and work/school absenteeism remained stable.
在血友病患者（PWH）中，反复的踝关节血肿会导致疼痛、关节活动范围（ROM）丧失以及活动和社会参与受限。为消除疼痛，PWH会接受踝关节融合术（AA）。根据我们的经验，由于担心步态异常、功能下降和ROM完全丧失，PWH对进行AA手术犹豫不决。本研究的目的是报告疾病控制与预防中心通用数据收集监测项目（UDC）中参与者在AA手术前后的ROM、辅助装置（AD）/轮椅使用情况、活动量表以及工作/学校缺勤情况。选取了参加UDC且首次报告进行AA手术（1998 - 2010年）的男性血友病患者。使用AA手术前年度研究访视和术后随访（约12 - 24个月）的数据进行描述性统计。符合标准的68名受试者：平均年龄36.9岁（标准差 = 12.9）；85.3%为白人；85.3%为A型血友病；72%为重度，20.6%为中度；10.3%在研究期间曾有过一次抑制剂。与AA手术后相比，术前踝关节总活动弧平均损失17.02°（标准差 = 21.8，P≤0.01）。61.8%的患者在行走/移动时AD使用情况无变化。85.3%的患者轮椅使用情况无变化。在自我报告的活动量表上，11.8%有所改善，8.8%恶化，79.4%无变化。AA手术前每年工作/学校缺勤平均为2.7天（标准差 = 6.4），术后为1.5天（标准差 = 6.4，P = 0.26）。虽然AA手术后踝关节ROM显著降低，但对于大多数受试者，行走/移动时AD/轮椅的使用情况没有变化。身体活动得以维持，工作/学校缺勤情况保持稳定。
