Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.
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Emiralioğlu Nagehan, Çakır Banu, Sertçelik Ahmet, Yalçın Ebru, Kiper Nural, Şen Velat, Altıntaş Derya Ufuk, Serbes Mahir, Çokuğraş Haluk, Kılınç Ayşe Ayzıt, Başkan Azer Kılıç, Hepkaya Evrim, Yazan Hakan, Türel Özden, Kafi Hale Molla, Yılmaz Aslı İmran, Ünal Gökçen, Çağlar Tuğçe, Damadoğlu Ebru, Irmak İlim, Demir Esen, Öztürk Gökçen, Bingöl Ayşen, Başaran Erdem, Sapan Nihat, Aslan Ayşe Tana, Asfuroğlu Pelin, Harmancı Koray, Köse Mehmet, Hangül Melih, Özdemir Ali, Tuğcu Gökçen, Polat Sanem Eryılmaz, Özcan Gizem, Gayretli Zeynep Gökçe, Keskin Özlem, Bilgiç Sevgi, Yüksel Hasan, Özdoğan Şebnem, Topal Erdem, Çaltepe Gönül, Can Demet, Ekren Pervin Korkmaz, Kılıç Mehmet, Süleyman Ayşe, Eyüboğlu Tuğba Şişmanlar, Cinel Güzin, Pekcan Sevgi, Çobanoğlu Nazan, Çakır Erkan, Özçelik Uğur, Doğru Deniz
Аннотация
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.