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

Aslan G. K., Huseyinsinoglu B. E., Oflazer P., Gurses N., Kiyan E.

LUNG, vol.194, no.4, pp.555-561, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 194 Issue: 4
  • Publication Date: 2016
  • Doi Number: 10.1007/s00408-016-9881-4
  • Journal Name: LUNG
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.555-561
  • Keywords: Pompe diseases, Pulmonary functions, Inspiratory muscle training, Diaphragm weakness, Sleep quality, Quality of life, RESPIRATORY-FAILURE, NATURAL COURSE, CHILDREN, GLYCOGENOSIS, MANAGEMENT, DIAGNOSIS, THERAPY, VALUES, FALL, RMT
  • Bezmialem Vakıf University Affiliated: Yes


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).