Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Center for Child Development, Exercise and Physical Literacy, Children's Hospital of the University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Haemophilia. 2020 Nov;26(6):1072-1080. doi: 10.1111/hae.14180. Epub 2020 Oct 15.
The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption.
This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion.
A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%).
Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction.
Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHS , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.
血友病关节健康评分（HJHS）旨在检测患有血友病的儿童和青少年关节健康的早期变化。HJHS 被一些人认为在临床使用上过于耗时，这可能限制了其广泛应用。
本研究旨在通过结合实际数据和专家意见，开发一种更短或更方便的 HJHS 版本，用于测量血友病儿童和青少年的关节功能，从而作为开发 HJHS 的第一步。
对使用 HJHS（0-124，最佳得分为 0）评估血友病 A/B 患者（4-30 岁）的已发表研究的汇总数据进行了一项横断面多中心二次分析。确定了最不重要的项目、评分选项和/或关节。一个由 19 名国际多学科专家组成的专家组在一次结构化会议上评估了结果，并对适应性建议进行了投票（共识设定为≥80%）。
对来自 7 项研究的 499 名血友病患者的原始数据进行了评估。中位年龄为 15.0 岁（范围 4.0-29.9），83.2%为重度血友病，61.5%接受了预防治疗。HJHS 总分为 6.0（1.0-17.0）。“肿胀持续时间”和“弹响”这两个项目被认为在临床上信息较少，被选为减少项目的候选。
对 499 名儿童和青少年血友病患者的分析表明，HJHS 能够区分儿童和成人以及不同的治疗方案。减少“肿胀持续时间”和“弹响”这两个项目后，HJHS 仍具有相同的区分能力。需要采取额外的步骤来实现更短的 HJHS 评估。
