Department of Paediatrics, Center for Lysosomal and Metabolic Diseases, and Department of Rehabilitation Medicine & Physical Therapy, Erasmus MC University Medical Center, Rotterdam, the Netherlands.
Department of Paediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus MC University Medical Center.
Phys Ther. 2018 Nov 1;98(11):925-931. doi: 10.1093/ptj/pzy090.
The loss of the ability to walk is among the most prominent signs of Pompe disease. The associations with muscle strength have not been described.
The objective of this study was to estimate the associations of walking performance with muscle strength in 4 specific lower extremity muscle groups along with other factors in adult patients with Pompe disease.
This was a single-center, cross-sectional study.
Muscle strength (hand-held dynamometry of hip flexion and abduction and knee extension and flexion) and walking performance (unable to walk, able with aids, walking without aids but with a waddling gait, or walking without aids and with a normal gait) were assessed in 107 patients at their first visit. Relationships between walking performance and muscle strength were studied through multivariate analyses and regression modeling. Age, sex, body mass index (BMI), disease duration, and use of ventilator support were taken into account as potential confounders. The results were transformed into a nomogram to allow the probability of a patient having a certain level of walking performance to be calculated based on the values of the independent variables.
Walking performance declined significantly with decreasing muscle strength of hip flexion and abduction and knee extension and flexion. The final selected model, including strength of the hip abductor and knee extensor, BMI, age, sex, and use of ventilation, predicted 66% of the cases accurately.
These results are based on cross-sectional data and do not predict future changes.
In adult people with Pompe disease, walking performance can be explained by muscle strength, BMI, age, sex, and ventilation use. The proposed model gives insight into how an individual is expected to walk based on his or her risk factors and serves as a starting point to unraveling factors associated with walking performance and ultimately to developing a prognostic model.
丧失行走能力是庞贝病最显著的标志之一。目前尚未描述其与肌肉力量的关系。
本研究旨在评估成人庞贝病患者的下肢 4 个特定肌肉群的肌肉力量与行走能力的关系，并分析其他因素。
这是一项单中心、横断面研究。
在首次就诊时，评估 107 例患者的肌肉力量（髋关节屈伸和膝关节屈伸的手持测力计测量）和行走能力（无法行走、借助辅助器具行走、无辅助器具但步态蹒跚行走、无辅助器具且步态正常行走）。通过多变量分析和回归建模研究行走能力与肌肉力量之间的关系。年龄、性别、体重指数（BMI）、疾病持续时间和使用呼吸机支持被视为潜在的混杂因素。结果被转化为一个诺模图，以便根据独立变量的值计算患者具有特定行走能力的概率。
行走能力随髋关节屈伸和膝关节屈伸的肌肉力量下降而显著下降。包括髋外展肌和膝关节伸肌力量、BMI、年龄、性别和使用呼吸机在内的最终选择模型准确预测了 66%的病例。
这些结果基于横断面数据，不能预测未来的变化。
在成人庞贝病患者中，行走能力可以通过肌肉力量、BMI、年龄、性别和呼吸机使用来解释。所提出的模型深入了解了根据个体的危险因素，他或她的行走预期情况，并为揭示与行走能力相关的因素和最终开发预后模型提供了起点。
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