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与健康同龄人相比,杜氏肌营养不良症患儿的身体能力、疲劳程度和运动表现有何不同?

How do physical capacity, fatigue and performance differ in children with duchenne muscular dystrophy compared with their healthy peers?

作者信息

Mutlu Akmer, Alkan Halil, Fırat Tuzun, Karaduman Aynur A, Yilmaz Oznur T

机构信息

Department of Physiotherapy and Rehabilitation, Faculty of Health Sciences, Hacettepe University, Ankara, Turkey. E-mail:

出版信息

Neurosciences (Riyadh). 2018 Jan;23(1):39-45. doi: 10.17712/nsj.2018.1.20170242.

Abstract

OBJECTIVE

To compare the fatigue levels and energy expenditure of children with Duchenne Muscular Dystrophy (DMD) at different functional levels with healthy children.

METHODS

The cross-sectional study was carried out in the Unit of Pediatric Neuromuscular Diseases in the Department of Physiotherapy and Rehabilitation, Faculty of Health Science, Hacettepe University between March 2015 and January 2016. Fifty two children diagnosed with DMD in Level I-III according to the Brooke Functional Classification Scale and 17 healthy children were included in the study. The Six Minute Walk Test (6MWT), Northstar Ambulatory Assessment Scale (NSAA), Physiological Cost Index (PCI), and Timed performance tests were used to assess the children.

RESULTS

Comparison in terms of PCI indicated a difference between Levels 2 and 3, and Levels 1 and 3 (p<0.0083). A difference was found in ascending and descending 4 stairs after 6MWT when fatigue after activity was evaluated.

CONCLUSION

The walking distances, fatigue levels and energy expenditure of DMD patients were higher than the healthy peers. This difference was more prominent with decreasing functional level.

摘要

目的

比较不同功能水平的杜氏肌营养不良症(DMD)患儿与健康儿童的疲劳程度和能量消耗。

方法

2015年3月至2016年1月期间,在哈杰泰佩大学健康科学学院物理治疗与康复系小儿神经肌肉疾病科开展了这项横断面研究。根据布鲁克功能分类量表,将52例诊断为I - III级DMD的儿童和17例健康儿童纳入研究。采用六分钟步行试验(6MWT)、北极星动态评估量表(NSAA)、生理成本指数(PCI)和定时性能测试对儿童进行评估。

结果

PCI方面的比较表明,2级与3级之间以及1级与3级之间存在差异(p<0.0083)。在评估活动后的疲劳情况时,6MWT后上、下4级楼梯存在差异。

结论

DMD患者的步行距离、疲劳程度和能量消耗高于健康同龄人。随着功能水平的降低,这种差异更为显著。

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Weakness and fatigue in diverse neuromuscular diseases.多种神经肌肉疾病中的虚弱与疲劳。
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Reliability of the North Star Ambulatory Assessment in a multicentric setting.多中心环境下北极星动态评估的可靠性
Neuromuscul Disord. 2009 Jul;19(7):458-61. doi: 10.1016/j.nmd.2009.06.368. Epub 2009 Jun 23.

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