European Journal of Pediatrics, vol.181, no.8, pp.2961-2970, 2022 (SCI-Expanded)
© 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.Bronchiectasis is a form of airway damage as a consequence of endobronchial infection and inflammation and may be present in different diseases. The underlying aetiologies include both cystic fibrosis (CF) and a group of non-cystic fibrosis diseases (NCFB) such as immunodeficiency, primary ciliary dyskinesia, or severe pulmonary infection. Although children with CF and non-cystic fibrosis bronchiectasis (NCFB) have many similar clinical features, their responses to exercise may be different. The aim of this study was to compare the efficacy of a comprehensive respiratory physiotherapy (CRP) home-program in children with CF and NCFB. Thirty children with CF and thirty children with NCFB were included in the study. Both groups performed the CRP home-program twice daily for 8 weeks. Pulmonary function, exercise capacity, and respiratory and peripheral muscle strength were assessed at baseline and after 8 weeks of training. Both groups experienced significant improvements in pulmonary function, exercise capacity, and respiratory and peripheral muscle strength (p < 0.001). Maximum expiratory pressure, exercise capacity, and peripheral muscle strength were further improved in NCFB group compared to CF (p < 0.05); however, there was a great variability in the improvements for each variable. Conclusion: CRP is beneficial both for children with CF and NCFB and adherence to the program was high in both groups.What is Known:• Different physiotherapy approaches in the management of non-cystic fibrosis bronchiectasis have been based on the experience gained from the research studies performed in cystic fibrosis.• Although having similar pathophysiology, these two diseases show variation in some pulmonary and extrapulmonary features.What is New:• The respiratory muscle strength and the efficacy of comprehensive respiratory physiotherapy have been compared for the first time in children with cystic fibrosis and non-cystic fibrosis bronchiectasis.• Comprehensive respiratory physiotherapy provides higher increases in children with non-cystic fibrosis bronchiectasis in exercise capacity and expiratory and peripheral muscle strength; however, there was a great variability in these improvements. Nevertheless, it can be concluded that both groups significantly benefited from the CRP program.