Lung function outcomes of cystic fibrosis patients after early-life pulmonary exacerbations: National registry analysis

Abstract

Background: Pulmonary exacerbations (PEs) are associated with a subsequentdecline in lung function. We aim to evaluate lung function in cystic fibrosis(CF) patients with frequent PEs in their first 2 years of age using spirometry atage 6.Methods: This retrospective cohort study included CF patients who were 6 yearsold from the CF registry of Turkey in 2019. According to the number of PEs,patients were classified: those who had two or fewer PEs in the first 2 years of agewere defined as Group 1 and those who had more than two PEs were defined asGroup 2. The patients' demographics and clinical characteristics were comparedbetween Group 1 and Group 2.Results: The study included 88 patients who had data on PE from their first2 years and completed their sixth year by 2019. Fifty-nine patients were includedin Group 1 and 29 in Group 2. The mean percent-predictive FEV1 (ppFEV1),percent-predictive FVC (ppFVC) values, and the mean age at first PE were lowerin Group 2 than in Group 1 (p = 0.019, p = 0.017, p < 0.001). The patients with chronicPseudomonas aeruginosa (PA) colonization had lower mean ppFEV1 and ppFVCvalues than those without (p = 0.001, p = 0.001). Patients with PA in respiratory-sample culture during their first PE had lower ppFEV1 and ppFVC values thanthose with SA (p = 0.046; 0.018). Conclusions: This study showed that more frequent PEs in the first 2 years of ageand chronic PA colonization were associated with poorer FEV1, FVC, and BMIvalues in CF patients.