Rehabilitation Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD, USA.
Department of Radiology, University of Pittsburgh, Pittsburgh, PA, USA.
Bone. 2014 Mar;60:41-7. doi: 10.1016/j.bone.2013.11.022. Epub 2013 Dec 4.
The purpose of this study was to determine the association between measures of disease severity, impairment, and ambulation ability in persons with polyostotic fibrous dysplasia (PFD). A cross-sectional sample of 81 patients (ages 5-57) with polyostotic fibrous dysplasia was evaluated as part of an ongoing study. Subjects were scored on the Skeletal Disease Burden Score (SDBS), completed a 9-minute walk test (9MW), manual muscle testing (MMT), and measurements of range of motion (ROM). Correlations between continuous variables were calculated using the Pearson correlation coefficient and ordinal variables by Spearman correlation coefficient. It was found that subjects with more severe disease walked slower than those with less skeletal disease, with the exception of the youngest subjects. Walking velocity was faster in subjects with better hip strength and range of motion and slower in those with bilateral coxa vara. Those subjects with more severe disease had less range of motion, were weaker at the hips, and more likely to have leg length discrepancy. Skeletal disease severity was associated with hip weakness, leg length discrepancy, and loss of range of motion. In most cases, findings did not differ in the presence or absence of associated endocrinopathies. Skeletal disease severity, MMT and ROM each has an impact on walking efficiency in persons with PFD. These findings suggest that treatment focused on strategies to improve or, at least, maintain hip strength and range of motion, correct leg length discrepancies and hip malalignment may help preserve ambulation ability in persons with PFD and that treatment should begin at a young age.
本研究旨在确定多骨性纤维结构不良（PFD）患者的疾病严重程度、功能障碍和步行能力评估指标之间的关系。 作为一项正在进行的研究的一部分，评估了 81 名多骨性纤维结构不良患者（年龄 5-57 岁）的横断面样本。 受试者根据骨骼疾病负担评分（SDBS）进行评分，完成 9 分钟步行测试（9MW）、手动肌肉测试（MMT）和运动范围（ROM）测量。 使用 Pearson 相关系数计算连续变量之间的相关性，使用 Spearman 相关系数计算有序变量之间的相关性。 结果发现，骨骼疾病更严重的患者比骨骼疾病较轻的患者步行速度慢，但最年轻的患者除外。 髋部力量和 ROM 较好的患者步行速度较快，双侧髋内翻患者步行速度较慢。 骨骼疾病更严重的患者运动范围更小，髋部力量更弱，且更容易出现肢体长度差异。 骨骼疾病的严重程度与髋部无力、肢体长度差异和运动范围丧失有关。 在大多数情况下，存在或不存在相关内分泌疾病时，结果没有差异。 骨骼疾病的严重程度、MMT 和 ROM 均会影响 PFD 患者的步行效率。 这些发现表明，针对改善或至少维持髋部力量和运动范围、纠正肢体长度差异和髋部错位的治疗策略可能有助于维持 PFD 患者的步行能力，并且治疗应在年轻时开始。
