Lung function outcomes of cystic fibrosis patients after early-life pulmonary exacerbations: National registry analysis
Küçük Resim Yok
Tarih
2026
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Wiley
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Background Pulmonary exacerbations (PEs) are associated with a subsequent decline 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 at age 6. Methods This retrospective cohort study included CF patients who were 6 years old 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 age were defined as Group 1 and those who had more than two PEs were defined as Group 2. The patients' demographics and clinical characteristics were compared between Group 1 and Group 2. Results The study included 88 patients who had data on PE from their first 2 years and completed their sixth year by 2019. Fifty-nine patients were included in 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 lower in Group 2 than in Group 1 (p = 0.019, p = 0.017, p < 0.001). The patients with chronic Pseudomonas aeruginosa (PA) colonization had lower mean ppFEV1 and ppFVC values 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 than those with SA (p = 0.046; 0.018). Conclusions This study showed that more frequent PEs in the first 2 years of age and chronic PA colonization were associated with poorer FEV1, FVC, and BMI values in CF patients.
Açıklama
Anahtar Kelimeler
chronic colonization, cystic fibrosis, pulmonary exacerbation, respiratory function
Kaynak
Pediatrics International
WoS Q Değeri
Q3
Scopus Q Değeri
Q3
Cilt
68
Sayı
1
