Hu Qin, Chen Xiaoyong, Luo Shaojie, Wang Yanhong, Fan Peicong, Wu Xiaona, Zhou Hui, Xu Huayan, Li Na, Cai Xiaotang
Department of Rehabilitation Medicine, West China Second University Hospital of Sichuan University (WCSUH- SCU), Chengdu, 610041, China.
Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education, Sichuan University, Chengdu, 610041, China.
BMC Pediatr. 2025 Jul 2;25(1):488. doi: 10.1186/s12887-025-05805-3.
Duchenne muscular dystrophy (DMD) is a recessive muscle degeneration disorder that involves the gradual loss of functional muscle mass. The Phase Angle (PhA) can indicate changes in cell membrane integrity and intercellular space. It has been recognized as a valuable tool for assessing disease severity and predicting patient outcomes. However, there is limited research on the application of PhA in children with neuromuscular diseases, including DMD.
More biological indicators are needed to reflect clinical progress in DMD children. We specifically examined the correlation between segmental PhAs and motor function and evaluated the effectiveness of segmental PhAs as a measure for assessing motor function in DMD children.
A retrospective longitudinal cohort study of 399 boys with DMD in West China Second University Hospital (2016-2023) was performed. The correlation between PhA and motor function were analyzed through spearman correlation analysis. The predictive value of PhA and PhA combining age for lower limb motor function loss was analyzed by ROC curve.
The time of peak of PhAs are earlier than NSAA score (peak at 6-7years old). The Trunk Phase Angle (TR-PhA) reaches its peak at 6 years old, while other PhAs reaches its peak at 5-6 years old and subsequently declines over time. There was a significant correlation between PhAs and the NSAA score, with the strongest correlation observed in leg PhA (r = 0.753, P < 0.001). PhAs in legs demonstrate the strongest correlation and highest predictive value for lower limb motor function loss(AUC from 0.725 to 0.863). Additionally, PhAs combined with age had more excellent predictive ability for lower limb function loss than PhAs((AUC from 0.929 to 0.951).
杜氏肌营养不良症（DMD）是一种隐性肌肉退化疾病，会导致功能性肌肉质量逐渐丧失。相位角（PhA）可表明细胞膜完整性和细胞间隙的变化。它已被公认为评估疾病严重程度和预测患者预后的有价值工具。然而，关于PhA在包括DMD在内的神经肌肉疾病儿童中的应用研究有限。
需要更多生物学指标来反映DMD患儿的临床进展。我们特别研究了节段性PhA与运动功能之间的相关性，并评估了节段性PhA作为评估DMD患儿运动功能指标的有效性。
对中国西部第二大学医院399名患有DMD的男孩进行了一项回顾性纵向队列研究（2016 - 2023年）。通过Spearman相关性分析来分析PhA与运动功能之间的相关性。采用ROC曲线分析PhA及PhA联合年龄对下肢运动功能丧失的预测价值。
PhA峰值出现时间早于NSAA评分（在6 - 7岁达到峰值）。躯干相位角（TR - PhA）在6岁时达到峰值，而其他PhA在5 - 6岁时达到峰值，随后随时间下降。PhA与NSAA评分之间存在显著相关性，其中腿部PhA的相关性最强（r = 0.753，P < 0.001）。腿部PhA与下肢运动功能丧失的相关性最强且预测价值最高（AUC为0.725至0.863）。此外，PhA联合年龄对下肢功能丧失的预测能力优于单独的PhA（AUC为0.929至0.951）。
1）随着DMD疾病进展，相位角（PhA）先升高后降低；相位角（PhA）的变化早于运动功能，且呈现良好相关性，是预测DMD疾病进展的敏感指标。PhA可作为预测DMD患儿下肢运动功能丧失的简单、快速且非侵入性的工具。
