CHU Nantes, Physical Medicine and Rehabilitation Center, Nantes, France.
Inserm, UMR 1229, RMeS, Regenerative Medicine and Skeleton, Université de Nantes, ONIRIS, Nantes, France.
Haemophilia. 2018 Sep;24(5):703-710. doi: 10.1111/hae.13563. Epub 2018 Jul 10.
Haemophilia is characterized by a congenital deficiency of clotting factor VIII or IX. One of the consequences of haemophilia is joint bleedings. Repetitive haemathroses induce cartilage damage and chronic synovitis leading to joint deterioration, and to definitive haemophilic arthropathy which is source of walking disability. Three-dimension gait analysis (3DGA) appears particularly relevant in the case of haemophilia because it allows an evaluation of several joints in weight-bearing situations. The purpose of this study was to review the interest and the contribution of 3DGA in the management of patients with haemophilia. The greatest interest of gait analysis would be to detect early walking changes with a non-invasive and well-tolerated examination, especially in paediatric population. In adulthood, this technic may be also useful to help detect walking worsening in patients known to have already arthropathy. However, it takes time to realize and needs expensive equipment, which limits its possibility of routine use. Although generalizations of these results remain difficult, especially to compare patients with haemophilia to normal population. Indeed, in the studies, patient groups are small and usually heterogeneous in terms of age and target joints. It certainly results of the rarity of the disease. So, it could be interesting to perform a study with a larger cohort in order to allow subgroup analysis, helping to define clearly the place of 3DGA in the strategy of haemophilia evaluation.
血友病的特征是凝血因子 VIII 或 IX 的先天性缺乏。血友病的后果之一是关节出血。反复的关节积血会导致软骨损伤和慢性滑膜炎，从而导致关节恶化，并最终导致血友病性关节炎，这是导致行走障碍的根源。三维步态分析（3DGA）在血友病的情况下显得尤为相关，因为它可以在负重情况下评估多个关节。本研究旨在回顾 3DGA 在血友病患者管理中的作用和贡献。步态分析的最大意义在于通过非侵入性和耐受性良好的检查来早期发现行走变化，尤其是在儿科人群中。在成年期，这种技术也可能有助于帮助检测已经有关节病的患者行走恶化的情况。然而，该技术需要时间来实现，并且需要昂贵的设备，这限制了其常规使用的可能性。尽管这些结果难以推广，尤其是将血友病患者与正常人群进行比较。实际上，在这些研究中，患者群体较小，而且通常在年龄和目标关节方面存在异质性。这肯定是由于该疾病的罕见性所致。因此，进行一项具有更大队列的研究可能会很有趣，以便进行亚组分析，有助于明确 3DGA 在血友病评估策略中的地位。
