Background: Primary ciliary dyskinesia (PCD) is generally likened to cystic fibrosis (CF) due to similarities in impaired mucociliary clearance and some other symptoms. The aim of our study was to investigate pulmonary and extrapulmonary characteristics of children with CF and PCD since no studies have addressed respiratory muscle strength in children with PCD and to compare the results to those obtained from healthy age-matched controls. Methods: Pulmonary and extrapulmonary characteristics were assessed by 6-min walk test, spirometry, maximum inspiratory and expiratory pressure measurements, and knee extensor strength test in the children with CF, PCD, and healthy controls. Results: Children with PCD and CF had similar PFT results, except forced expiratory flow between 25% and 75% of vital capacity (FEF 25-75) which was lower in PCD (p = .04). Maximum inspiratory pressure (MIP) value was lower in the children with CF compared with the healthy controls (p = .016), MEP value of the children with PCD was worse than those with CF and healthy controls (p = .013 and p = .013), respectively. 6-min walk test (6MWT) distance of the children with CF was lower than their healthy counterparts (p = .003). Knee extensor muscle strength differed among the children with PCD, CF, and healthy control groups, but post hoc test failed to show statistical significance (p = .010). Conclusion: Children with CF and PCD had some impairments in pulmonary functions, respiratory muscle strength, functional capacity, and peripheral muscle strength compared with healthy children. However, the unique characteristics of each disease should be considered during physiotherapy assessment and treatment. The clinicians may especially focus on the respiratory and peripheral muscle strength of the children with PCD.

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Background: Video game-based systems have been proposed to improve effectiveness and compliance with exercise training in children and adolescents with noncystic fibrosis bronchiectasis (NCFB). This study aimed to investigate the effects of aerobic and breathing video game-based exercises (VGE) on pulmonary function, respiratory and peripheral muscle strength, functional capacity, and balance in children and adolescents with NCFB. Method: Thirty-nine children and adolescents aged between 8 and 18 years with NCFB were randomly allocated into three groups as "home-based chest physiotherapy group" (CP), "aerobic VGE given in addition to home-based chest physiotherapy group" (CP + aerobic VGE), and "breathing VGE given in addition to home-based chest physiotherapy group" (CP + breathing VGE). All three groups performed chest physiotherapy program twice a day for 7 days per week for 8 weeks. Pulmonary function, respiratory and peripheral muscle strength, functional capacity, and balance were assessed at baseline and after 8 weeks of training.Results: The improvement in maximum expiratory pressure and balance scores were significantly higher in both CP + aerobic and CP + breathing VGE groups.The significant improvement in maximum inspiratory pressure was greater in the CP + breathing VGE group. The changes in peripheral muscle strength and functional capacity were significantly higher in the CP + aerobic VGE group. Conclusions:The present study showed that aerobic VGE provides additional benefits in improving peripheral muscle strength and functional capacity, while breathing VGE provides further increase in improving respiratory muscle strength. In addition, both aerobic and breathing VGE were effective in improving balance, but they were not superior to each other.

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