Early structural lung changes in young children with cystic fibrosis: a call to action.

Authors

Aline da Silva Amoras, Tália Andrea Soria Muñoz, Leticia Cole de Melo, Silvia Onoda Tomikawa, Miriam Cardoso Neves Eller, Cleyde Myriam Aversa Nakaie, Joaquim Carlos Rodrigues, Luiz Vicente Ribeiro Ferreira da Silva-Filho

Published in

Respiratory research & clinical practice. Volume 52. Issue 2. Pages e20250402. Epub May 22, 2026.

Abstract

Cystic fibrosis (CF) lung disease begins in early childhood, despite close follow-up and monitoring. Although imaging methods such as chest CT may detect structural changes before significant clinical manifestations, their role in preschool children remains debated. Evidence from low- and middle-income countries is limited. We sought to investigate the age at first chest CT, as well as imaging findings and their impact on treatment decisions.
We conducted a retrospective cross-sectional study including children with CF attending a referral center in the city of São Paulo, Brazil, between 2009 and 2019. Clinical data were obtained from patient medical records and the Brazilian CF Registry database.
A total of 90 children were included, of whom 56.7% were male and 71.1% carried at least one F508del variant. The median age at CF diagnosis was 2.4 months (IQR, 1.5-4.5). The first chest CT was performed at a median age of 36.7 months (IQR, 28.1-47.8). Structural lung abnormalities were common, including air trapping (in 86.6% of the children), mucoid impaction (in 75.5%), bronchiectasis (in 45.5%), and atelectasis (in 35.5%), with only 4.4% of the scans being considered normal. Prescription of dornase alfa was based on CT findings in 74% of the cases, with initiation at a median age of 36.9 months (IQR, 28.9-57.6). A strong correlation was observed between age at first chest CT and age at initiation of dornase alfa therapy (r = 0.658; p < 0.001).
Early chest CT revealed a high prevalence of structural lung disease in Brazilian infants and preschool children with CF and strongly influenced the initiation of dornase alfa.

PMID:
42780783
Bibliographic data and abstract were imported from PubMed on 25 Sep 2026.

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