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01-08-2016

Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality

Authors: Goksen Kuran Aslan, Burcu Ersoz Huseyinsinoglu, Piraye Oflazer, Nilgun Gurses, Esen Kiyan

Published in: Lung | Issue 4/2016

Abstract

Introduction

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).

Methods

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.

Results

A significant increase in maximum inspiratory pressure (cmH₂O and % predicted) (median [interquartile range]: 30.0 cmH₂O [21.5–48] versus 39 cmH₂O [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.

Conclusion

These results suggest that IMT has a positive effect on maximum inspiratory pressure in subjects with LOPD who are under ERT.

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Title: Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality
Authors: Goksen Kuran Aslan
Burcu Ersoz Huseyinsinoglu
Piraye Oflazer
Nilgun Gurses
Esen Kiyan
Publication date: 01-08-2016
Publisher: Springer US
Published in: Lung / Issue 4/2016
Print ISSN: 0341-2040
Electronic ISSN: 1432-1750
DOI: https://doi.org/10.1007/s00408-016-9881-4

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Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality

Abstract

Introduction

Methods

Results

Conclusion

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Abstract

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Results

Conclusion

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