Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality
Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality
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DOI：
10.1007/s00408-016-9881-4
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发表时间：
2016-08-01
期刊：
影响因子：
5
通讯作者：
Kiyan, Esen
中科院分区：
文献类型：
--
作者：
Aslan, Goksen Kuran;Huseyinsinoglu, Burcu Ersoz;Kiyan, Esen
晚发性庞贝氏症（LOPD）的特征是进行性骨骼肌和呼吸肌无力。关于吸气肌训练（IMT）对LOPD受试者肺功能的影响知之甚少。本研究的目的是调查8例接受酶替代治疗（ERT）的LOPD患者接受8周IMT治疗对肺功能、生活质量和睡眠质量的影响。在IMT治疗前后，分别测量坐位和仰卧位的肺功能，最大吸气压和呼气压，最大咳嗽流量，生活质量（使用诺丁汉健康概况评估）和睡眠质量最大吸气压（cmH 2 O和%预测值）显著增加，（中位数[四分位距]：30.0 cmH（2）O [21.5-48] vs 39 cmH（2）O [31.2-56.5]和38.3%[28.1-48.4] vs 50.5%[37.7-54.9]）（p = 0.01）。其他肺功能测量值无显著变化。除了社交孤立分项评分（p = 0.02）外，生活质量分项评分在IMT后没有变化（p > 0.05）。IMT治疗前后睡眠质量总分和总分无显著性差异，提示IMT对LOPD患者ERT治疗后的最大吸气压有积极影响。
Late-onset Pompe disease (LOPD) is characterized by progressive skeletal and respiratory muscle weakness. Little is known about the effect of inspiratory muscle training (IMT) on pulmonary function in subjects with LOPD. The aim of the present study was to investigate the effect of an 8-week IMT program on pulmonary function tests, quality of life, and sleep quality in eight patients with LOPD who were receiving enzyme replacement therapy (ERT).Before and after the IMT program, spirometric measurements in sitting and supine positions, and measurements of maximum inspiratory and expiratory pressures, peak cough flow, quality of life (assessed using the Nottingham Health Profile), and sleep quality (assessed using the Pittsburgh sleep quality index) were performed.A significant increase in maximum inspiratory pressure (cmH(2)O and % predicted) (median [interquartile range]: 30.0 cmH(2)O [21.5-48] versus 39 cmH(2)O [31.2-56.5] and 38.3 % [28.1-48.4] versus 50.5 % [37.7-54.9]) was observed after training (p = 0.01). There were no significant changes in the other pulmonary function measurements. With the exception of the social isolation subscore (p = 0.02), quality of life subscores did not change after IMT (p > 0.05). Sleep quality subscores and total scores were similar before and after IMT.These results suggest that IMT has a positive effect on maximum inspiratory pressure in subjects with LOPD who are under ERT.