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Pediatrics international : official journal of the Japan Pediatric Society · 2026

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

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Özkan Tabakçı Satı, Uytun Salih, Eryılmaz Polat Sanem, Cinel Güzin, Yalçın Ebru, Kiper Nural, Akgül Erdal Meltem, Şen Velat, Savaş Suat, Çelebi Özcan, Ufuk Altıntaş Derya, Serbes Mahir, Ayzıt Kılınç Ayşe, Çokuğraş Haluk, Arslan Hüseyin, Yazan Hakan, Molla Kafi Hale, Çollak Abdülhamit, Ünal Gökçen, Yılmaz Aslı Imran, Çağlar Hanife Tuğçe, Irmak Ilim, Damadoğlu Ebru, Kartal Öztürk Gökçen, Demir Esen, Başaran A Erdem, Bingöl Ayşen, Canıtez Yakup, Çekiç Şükrü, Asfuroğlu Pelin, Tana Aslan Ayşe, Harmancı Koray, Kılıç Gonca, Köse Mehmet, Ersoy Ali, Hangül Melih, Özdemir Ali, Özcan Gizem, Çobanoğlu Nazan, Gayretli Aydın Zeynep Gökçe, Keskin Özlem, Yüksel Hasan, Özdoğan Şebnem, Topal Erdem, Çaltepe Gönül, Can Demet, Korkmaz Ekren Pervin, Kılıç Mehmet, Şişmanlar Eyüboğlu Tuğba, Pekcan Sevgi, Çakır Erkan, Emiralioğlu Nagehan, Özçelik Uğur, Doğru Ersöz Deniz

Аннотация

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.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты