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    Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy children?
    (Wiley, 2020) Denizoğlu Kulli, Hilal; Gürses, Hülya Nilgün; Zeren, Melih; Üçgün, Hikmet; Çakır, Erkan
    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 (FEF25-75) 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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    Does the effect of comprehensive respiratory physiotherapy home-program differ in children with cystic fibrosis and non-cystic fibrosis bronchiectasis?
    (Springer, 2022) Gürses, Hülya Nilgün; Üçgün, Hikmet; Zeren, Melih; Denizoğlu Külli, Hilal; Çakır, Erkan
    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.
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    Sit-to-stand test in children with bronchiectasis: Does it measure functional exercise capacity?
    (Mosby-Elsevier, 2020) Zeren, Melih; Gürses, Hülya Nilgün; Kulli, Hilal Denizoğlu; Üçgün, Hikmet; Çakır, Erkan
    Background: Similar to six-minute walk test (6MWT), sit-to-stand test (STST) is a self-paced test which elicits sub-maximal effort; therefore, it is suggested as an alternative measurement for functional exercise capacity in various pulmonary conditions including COPD and cystic fibrosis. We aimed to investigate the association between 30-second STST (30s-STST) and 6MWT in both children with bronchiectasis (BE) and their healthy counterparts, as well as exploring cardiorespiratory burden and discriminative properties of both tests. Methods: Sixty children (6 to 18-year-old) diagnosed with non-cystic fibrosis BE and 20 age-matched healthy controls were included. Both groups performed 30s-STST and 6MWT. Test results, and heart rate, SpO(2) and dyspnea responses to tests were recorded. Results: Univariate analysis revealed that 30s-STST was able to explain 52% of variance in 6MWT (r = 0.718, p<0.001) in BE group, whereas 20% of variance in healthy controls (r = 0.453, p = 0.045). 6MWT elicited higher changes in heart rate and dyspnea level compared to 30s-STST, indicating it was more physically demanding. Both 30s-STST (21.65 +/- 5.28 vs 26.55 +/- 3.56 repetitions) and 6MWT (538 +/- 85 vs 596 +/- 54 m) were significantly lower in BE group compared to healthy controls (p<0.01). Receiver operating characteristic (ROC) curve analysis revealed an area under the ROC curve (UAC) of 0.765 for 30s-STST and 0.693 for 6MWT in identifying the individuals with or without BE (p<0.05). Comparison between AUCs of 30s-STST and 6MWT yielded no significant difference (p = 0.466), indicating both tests had similar discriminative properties. Conclusions: 30s-STST is found to be a valid alternative measurement for functional exercise capacity in chil-dren with BE. (c) 2020 Elsevier Inc. All rights reserved.