Nagehan Emiralioğlu, Banu Çakır, Ahmet Sertçelik, Ebru Yalçın, Nural Kiper, Velat Şen, Derya Ufuk Altıntaş, Mahir Serbes, Haluk Çokuğraş, Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Evrim Hepkaya, Hakan Yazan, Özden Türel, Hale Molla Kafi, Aslı İmran Yılmaz, Gökçen Ünal, Tuğçe Çağlar, Ebru Damadoğlu, İlim Irmak, Esen Demir, Gökçen Öztürk, Ayşen Bingöl, Erdem Başaran, Nihat Sapan, Ayşe Tana Aslan, Pelin Asfuroğlu, Koray Harmancı, Mehmet Köse, Melih Hangül, Ali Özdemir, Gökçen Tuğcu, Sanem Eryılmaz Polat, Gizem Özcan, Zeynep Gökçe Gayretli, Özlem Keskin, Sevgi Bilgiç, Hasan Yüksel, Şebnem Özdoğan, Erdem Topal, Gönül Çaltepe, Demet Can, Pervin Korkmaz Ekren, Mehmet Kılıç, Ayşe Süleyman, Tuğba Şişmanlar Eyüboğlu, Güzin Cinel, Sevgi Pekcan, Nazan Çobanoğlu, Erkan Çakır, Uğur Özçelik, Deniz Doğru
{"title":"Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.","authors":"Nagehan Emiralioğlu, Banu Çakır, Ahmet Sertçelik, Ebru Yalçın, Nural Kiper, Velat Şen, Derya Ufuk Altıntaş, Mahir Serbes, Haluk Çokuğraş, Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Evrim Hepkaya, Hakan Yazan, Özden Türel, Hale Molla Kafi, Aslı İmran Yılmaz, Gökçen Ünal, Tuğçe Çağlar, Ebru Damadoğlu, İlim Irmak, Esen Demir, Gökçen Öztürk, Ayşen Bingöl, Erdem Başaran, Nihat Sapan, Ayşe Tana Aslan, Pelin Asfuroğlu, Koray Harmancı, Mehmet Köse, Melih Hangül, Ali Özdemir, Gökçen Tuğcu, Sanem Eryılmaz Polat, Gizem Özcan, Zeynep Gökçe Gayretli, Özlem Keskin, Sevgi Bilgiç, Hasan Yüksel, Şebnem Özdoğan, Erdem Topal, Gönül Çaltepe, Demet Can, Pervin Korkmaz Ekren, Mehmet Kılıç, Ayşe Süleyman, Tuğba Şişmanlar Eyüboğlu, Güzin Cinel, Sevgi Pekcan, Nazan Çobanoğlu, Erkan Çakır, Uğur Özçelik, Deniz Doğru","doi":"10.1002/ppul.27165","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Pulmonology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/ppul.27165","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/9 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0
Abstract
Background: 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.
期刊介绍：
Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases.
PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.
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