Haemophilia Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Haemnet, London, UK.
Haemophilia. 2017 Nov;23(6):918-925. doi: 10.1111/hae.13323. Epub 2017 Aug 14.
Contemporary haemophilia care demands Patient-Reported Outcomes. SO-FIT is a UK multi-centre study, assessing self-reported function, health-related quality of life (HRQoL) and joint health in boys with severe haemophilia.
Subjective physical function (PedHAL, HEP-Test-Q) and HRQoL (Haemo-QoL Short Form [SF]) were assessed alongside joint health using the objective Haemophilia Joint Health Score (HJHS v2.1). Demographic and clinical data were collected.
Data from 127 boys mean age 12.38 ± 2.5 (range 8-17) treated at 16 sites were analysed. One-hundred-and-thirteen had haemophilia A, 25/9 past/current inhibitor, 124 were treated prophylactically (46.8% primary) and three on-demand. In the preceding 6 months, boys reported median 0 joint bleeds (range 0-8) with a median HJHS score of 1 (range 0-30). Boys reported good physical functioning; HEP-Test-Q (M = 80.32 ± 16.1) showed the highest impairments in the domain "endurance" (72.53 ± 19.1), in PedHAL (M = 85.44 ± 18.9) highest impairments were in the domains "leisure activities & sports" (M = 82.43 ± 23.4) and "lying/sitting/kneeling/standing" (M = 83.22 ± 20.3). Boys reported generally good HRQoL in Haemo-QoL SF SF (M = 22.81 ± 15.0) with highest impairments in the domains "friends" (M = 28.81 ± 30.5) and "sports & school" (M = 26.14 ± 25.1). HJHS revealed low correlations with the Haemo-QoL SF (r = .251, P < .006), the PedHAL (r = -.397, P < .0001) and the HEP-Test-Q (r = -.323, P < .0001). A moderate correlation was seen between HEP-Test-Q and Haemo-QoL SF of r = -.575 (P < .0001) and between PedHAL and Haemo-QoL SFr = -.561 (P < .0001) implying that good perceived physical function is related to good HRQoL.
The SO-FIT study has demonstrated that children with severe haemophilia in the UK report good HRQoL and have good joint health as reflected in low HJHS scores.
当代血友病护理需要患者报告的结果。SO-FIT 是一项英国多中心研究，评估了严重血友病男孩的自我报告功能、健康相关生活质量（HRQoL）和关节健康。
主观身体功能（PedHAL、HEP-Test-Q）和 HRQoL（Haemo-QoL 短表 [SF]）与关节健康一起使用客观的血友病关节健康评分（HJHS v2.1）进行评估。收集人口统计学和临床数据。
对 16 个地点治疗的 127 名平均年龄为 12.38±2.5（范围为 8-17）的男孩的数据进行了分析。113 人患有血友病 A，25/9 人过去/现在有抑制剂，124 人预防性治疗（46.8%为原发性），3 人按需治疗。在过去 6 个月中，男孩报告中位数为 0 关节出血（范围为 0-8），中位数 HJHS 评分为 1（范围为 0-30）。男孩报告身体功能良好；HEP-Test-Q（M=80.32±16.1）在“耐力”领域表现出最高的损伤（M=72.53±19.1），在 PedHAL（M=85.44±18.9）中，最高的损伤是在“休闲活动和运动”领域（M=82.43±23.4）和“躺着/坐着/跪着/站立”领域（M=83.22±20.3）。男孩在 Haemo-QoL SF SF 中报告了一般良好的 HRQoL（M=22.81±15.0），在“朋友”领域（M=28.81±30.5）和“运动和学校”领域（M=26.14±25.1）的损伤最高。HJHS 与 Haemo-QoL SF（r=.251，P<.006）、PedHAL（r=-.397，P<.0001）和 HEP-Test-Q（r=-.323，P<.0001）的相关性较低。HEP-Test-Q 与 Haemo-QoL SF 的相关性中等，r=-.575（P<.0001），PedHAL 与 Haemo-QoL SF 的相关性中等，r=-.561（P<.0001），这表明良好的感知身体功能与良好的 HRQoL 相关。
SO-FIT 研究表明，英国患有严重血友病的儿童报告了良好的 HRQoL，并且关节健康状况良好，这反映在低 HJHS 评分上。
