Secteur des Sciences de la Santé, Institut de Recherche Expérimentale et Clinique, Neuromusculoskeletal Lab (NMSK), Université catholique de Louvain, Brussels, Belgium.
Service d'hématologie, Cliniques universitaires Saint-Luc, Brussels, Belgium.
Haemophilia. 2017 Sep;23(5):e409-e418. doi: 10.1111/hae.13274. Epub 2017 Jul 9.
Adequate management of haemophilia patients requires early detection of joint impairment in relatively asymptomatic patients. This study sought to quantify the impact of the ankle's structural impairment on muscle strength in children, adolescent and young adults with haemophilia (CAAwH).
Twenty-three CAAwH underwent bilateral magnetic resonance imaging (MRI) assessing the anatomical status of tibiotalar joint (TTJ) and subtalar joint (STJ) using the International Prophylaxis Study Group MRI scale. An isokinetic dynamometer enabled a detailed evaluation of muscle strength at slow and fast speed. In parallel, 10 typically developing healthy boys (TDB) participated in a 1-week interval test-retest assessment to assess the test's reliability.
Forty-six MRI ankle scores were obtained, with 11 patients unilaterally affected and one bilaterally. Of the 13 affected feet, nine showed abnormalities at TTJ, three at the posterior STJ and the remaining one at both joints. Muscle strength was not reduced in CAAwH exhibiting TTJ and/or STJ arthropathy, as compared to healthy TDB, nor was there any difference between the CAAwH's affected or unaffected sides.
Contrarily to adult patients, CAAwH with repeated ankle bleeding may be less impaired than current structural evaluations imply, with possibly a latency between the occurrence of structural and functional damage.
为了实现血友病患者的充分管理，需要在相对无症状的患者中尽早发现关节损伤。本研究旨在定量评估踝关节结构损伤对儿童、青少年和青年血友病患者（CAAwH）肌肉力量的影响。
23 名 CAAwH 患者接受了双侧磁共振成像（MRI）检查，使用国际预防研究组 MRI 量表评估距下关节（TTJ）和跗骨间关节（STJ）的解剖结构。使用等速测力计可详细评估慢速和快速运动下的肌肉力量。同时，10 名典型发育健康男孩（TDB）参加了为期 1 周的测试-再测试间隔评估，以评估测试的可靠性。
共获得 46 个 MRI 踝关节评分，11 名患者单侧受累，1 名双侧受累。在 13 只受累的足部中，9 只 TTJ 出现异常，3 只后 STJ 出现异常，其余 1 只在两个关节均出现异常。与健康 TDB 相比，在 TTJ 和/或 STJ 关节炎的 CAAwH 中，肌肉力量并未降低，并且在 CAAwH 的受累侧和未受累侧之间也没有差异。
与成年患者不同，反复踝关节出血的 CAAwH 可能受损程度低于当前的结构评估结果，可能存在结构和功能损伤之间的潜伏期。
