TY - JOUR
T1 - Impact of Modulator Therapy on the Chronic Colonization of Lower Respiratory Tract Pathogens in Children
T2 - Data From Cystic Fibrosis Registry of Turkey
AU - Sunman, Birce
AU - Yalçın, Ebru
AU - Erdal, Meltem Akgül
AU - Şen, Velat
AU - Başkan, Azer Kılıç
AU - Kılınç, Ayşe Ayzıt
AU - Yazan, Hakan
AU - Çakır, Erkan
AU - Demir, Ayşegül Doğan
AU - Ünal, Gökçen
AU - Savaş, Suat
AU - Pekcan, Sevgi
AU - Korkmaz, Merve
AU - Canıtez, Yakup
AU - Öztürk, Gökçen Kartal
AU - Gülen, Figen
AU - Arık, Elif
AU - Keskin, Özlem
AU - Ersoy, Ali
AU - Köse, Mehmet
AU - Serbes, Mahir
AU - Altıntaş, Derya Ufuk
AU - Başaran, Abdurrahman Erdem
AU - Bingöl, Ayşen
AU - Hocoğlu, Zeynep İlkşen
AU - Aslan, Ayşe Tana
AU - Yuluğ, Demet Polat
AU - Özdemir, Ali
AU - Tabakçı, Satı Özkan
AU - Tural, Dilber Ademhan
AU - Harmancı, Koray
AU - Özsezen, Beste
AU - Çobanoğlu, Nazan
AU - Tongal, Sedef Narin
AU - Çaltepe, Gönül
AU - Hangül, Melih
AU - Aydın, Zeynep Gökçe Gayretli
AU - Kılıç, Mehmet
AU - Hızal, Mina
AU - Baş, Nilay
AU - Özcan, Gizem
AU - Eyüboğlu, Tuğba Şişmanlar
AU - Emiralioğlu, Nagehan
AU - Cinel, Güzin
AU - Özçelik, Uğur
AU - Doğru, Deniz
N1 - Publisher Copyright:
© 2025 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.
PY - 2025/6
Y1 - 2025/6
N2 - Background: Modulators have revolutionized cystic fibrosis (CF) management, but their effects on respiratory pathogens remain unclear. This study evaluated changes in lower respiratory tract pathogen detection after modulator therapy in children with CF, registered in the Cystic Fibrosis Registry of Turkey. Method: This retrospective, multicenter cohort study included children receiving modulator therapy between 2020 and 2022. Chronic respiratory tract colonization rates before and after therapy were compared, along with inhaler treatments, oral steroid, azithromycin use, pulmonary function tests, and hospitalizations for pulmonary exacerbations. The cohort was stratified by age, modulator type, and lung disease severity. Changes in microbiologic data over a 1-year period were also analyzed for children not receiving modulator therapy. Results: A total of 101 children (mean age 9.95 ± 4.44 years) were included. Following modulator therapy, respiratory cultures of 57 (56.4%) were negative. Among 32 children with chronic Pseudomonas aeruginosa (P. aeruginosa) colonization, 14 (44%) showed negative respiratory cultures after receiving modulator therapy (p = 0.039). Conversion to culture-negative status was significant for methicillin-sensitive Staphylococcus aureus (MSSA) (p = 0.022) and methicillin-resistant Staphylococcus aureus (MRSA) (p = 0.034), with ETI therapy yielding the highest conversion rates. Inhaled antibiotic use for chronic respiratory pathogens decreased significantly (p = 0.039), and spirometry parameters improved (p < 0.001). Among 1232 children not receiving modulators, 180 (14.6%) had negative respiratory cultures when examined at 1-year interval. In the same group, intermittent/chronic P. aeruginosa colonization was negative in 58 cases, while 85 developed new growth, following modulator therapy, with positivity rates rising from 16.3% to 18.5% (p = 0.030). No significant changes in other pathogen detection were observed. Conclusion: Modulators, particularly ETI, reduced respiratory pathogen detection and improved lung function in children with CF.
AB - Background: Modulators have revolutionized cystic fibrosis (CF) management, but their effects on respiratory pathogens remain unclear. This study evaluated changes in lower respiratory tract pathogen detection after modulator therapy in children with CF, registered in the Cystic Fibrosis Registry of Turkey. Method: This retrospective, multicenter cohort study included children receiving modulator therapy between 2020 and 2022. Chronic respiratory tract colonization rates before and after therapy were compared, along with inhaler treatments, oral steroid, azithromycin use, pulmonary function tests, and hospitalizations for pulmonary exacerbations. The cohort was stratified by age, modulator type, and lung disease severity. Changes in microbiologic data over a 1-year period were also analyzed for children not receiving modulator therapy. Results: A total of 101 children (mean age 9.95 ± 4.44 years) were included. Following modulator therapy, respiratory cultures of 57 (56.4%) were negative. Among 32 children with chronic Pseudomonas aeruginosa (P. aeruginosa) colonization, 14 (44%) showed negative respiratory cultures after receiving modulator therapy (p = 0.039). Conversion to culture-negative status was significant for methicillin-sensitive Staphylococcus aureus (MSSA) (p = 0.022) and methicillin-resistant Staphylococcus aureus (MRSA) (p = 0.034), with ETI therapy yielding the highest conversion rates. Inhaled antibiotic use for chronic respiratory pathogens decreased significantly (p = 0.039), and spirometry parameters improved (p < 0.001). Among 1232 children not receiving modulators, 180 (14.6%) had negative respiratory cultures when examined at 1-year interval. In the same group, intermittent/chronic P. aeruginosa colonization was negative in 58 cases, while 85 developed new growth, following modulator therapy, with positivity rates rising from 16.3% to 18.5% (p = 0.030). No significant changes in other pathogen detection were observed. Conclusion: Modulators, particularly ETI, reduced respiratory pathogen detection and improved lung function in children with CF.
KW - CFTR modulator
KW - Pseudomonas
KW - cystic fibrosis
KW - pathogen
UR - https://www.scopus.com/pages/publications/105007907934
U2 - 10.1002/ppul.71155
DO - 10.1002/ppul.71155
M3 - Article
C2 - 40504038
AN - SCOPUS:105007907934
SN - 8755-6863
VL - 60
JO - Pediatric Pulmonology
JF - Pediatric Pulmonology
IS - 6
M1 - e71155
ER -