Haemophilia Foundation of Argentina, Buenos Aires, Argentina.
Department of Orthopaedics and Traumatology, Juan A. Fernandez Hospital, Buenos Aires, Argentina.
Haemophilia. 2021 Jul;27(4):641-647. doi: 10.1111/hae.14312. Epub 2021 May 10.
Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration and subsequent disability. The aim of this study is to evaluate the level of agreement between the Haemophilia Joint Health Score and the Haemophilia Early Arthropathy Detection with Ultrasound tools in children with severe haemophilia on primary and secondary prophylaxis.
All patients were followed up regularly at our centre. Elbows, knees and ankles were evaluated by physical examination using the Haemophilia Joint Health Score 2.1 (HJHS 2.1), and by ultrasound with HEAD-US score.
A total of 80 children with haemophilia on prophylaxis were included in this study. Mean age was 10.8 years (range 4-18). We evaluated 480 joints, of which 423 (88.1%) were concordant with both tools, whereas 57 (11.9%) were discordant; 377 (78.5%) joints scored 0 on HJHS, 370 (77%) on HEAD-US and 345 (72%) on both tools. The overall Kappa concordance coefficient was .656. For elbows, knees and ankles the respective values were .783, .522 and .589. For HJHS scores greater than 3, all joints scored ≥1 on HEAD-US.
HJHS and HEAD-US are used to assess joint health in children with haemophilia on prophylaxis. In this study, the level of agreement between both tools was consistent with literature values only for the elbow joint.
原发性预防是目前血友病治疗的金标准，用于预防出血和随之而来的关节损伤。早期发现关节出血，无论是有症状还是亚临床的，最好在儿童时期，有助于预防关节恶化和随后的残疾。本研究的目的是评估在原发性和次级预防中，严重血友病儿童的 Haemophilia Joint Health Score（HJHS）和 Haemophilia Early Arthropathy Detection with Ultrasound（HEAD-US）工具之间的一致性水平。
所有患者均在我们中心接受定期随访。使用 Haemophilia Joint Health Score 2.1（HJHS 2.1）通过体格检查和超声检查 HEAD-US 评分评估肘部、膝关节和踝关节。
本研究共纳入 80 名接受预防治疗的血友病儿童。平均年龄为 10.8 岁（范围 4-18 岁）。我们评估了 480 个关节，其中 423 个（88.1%）与两种工具均一致，而 57 个（11.9%）不一致；377 个（78.5%）关节在 HJHS 中评分为 0，370 个（77%）在 HEAD-US 中评分为 0，345 个（72%）在两种工具中评分为 0。总体 Kappa 一致性系数为.656。对于肘部、膝关节和踝关节，相应的值分别为.783、.522 和.589。对于 HJHS 评分大于 3 的所有关节，在 HEAD-US 中评分均≥1。
HJHS 和 HEAD-US 用于评估接受预防治疗的血友病儿童的关节健康状况。在本研究中，两种工具之间的一致性水平仅与文献值一致，仅限于肘关节。
