Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study

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dc.authoridTugcu, Gokcen Dilsa/0000-0002-9804-1200
dc.authoridKartal Ozturk, Gokcen/0000-0002-0793-9710
dc.authoridSertcelik, Ahmet/0000-0003-4301-0586
dc.authoridKilinc, Ayse Ayzit/0000-0002-2879-8910
dc.authoridSerbes, Mahir/0000-0001-6422-2639
dc.authoridCakir, Erkan/0000-0002-1438-7854
dc.contributor.authorEmiralioglu, Nagehan
dc.contributor.authorCakir, Banu
dc.contributor.authorSertcelik, Ahmet
dc.contributor.authorYalcin, Ebru
dc.contributor.authorKiper, Nural
dc.contributor.authorSen, Velat
dc.contributor.authorAltintas, Derya Ufuk
dc.date.accessioned2025-02-22T14:09:09Z
dc.date.available2025-02-22T14:09:09Z
dc.date.issued2024
dc.departmentDicle Üniversitesien_US
dc.description.abstractBackground: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. Methods: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. Results: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 >= 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 >= 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. Conclusions: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.en_US
dc.description.sponsorshipThe authors have no funding to report.en_US
dc.identifier.doi10.1002/ppul.27165
dc.identifier.endpage2966en_US
dc.identifier.issn8755-6863
dc.identifier.issn1099-0496
dc.identifier.issue11en_US
dc.identifier.pmid38980199en_US
dc.identifier.scopus2-s2.0-85197756695en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage2956en_US
dc.identifier.urihttps://doi.org/10.1002/ppul.27165
dc.identifier.urihttps://hdl.handle.net/11468/29812
dc.identifier.volume59en_US
dc.identifier.wosWOS:001268137200001en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.ispartofPediatric Pulmonologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.snmzKA_WOS_20250222
dc.subjectcystic fibrosisen_US
dc.subjectFEV1en_US
dc.subjectpulmonary function testen_US
dc.subjectPseudomonas aeruginosaen_US
dc.titleFactors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort studyen_US
dc.typeArticleen_US

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