Factors in childhood associated with lung function decline to adolescence in cystic fibrosis

•Loss of lung function during late childhood remains a problem for patients with cystic fibrosis.•Respiratory exacerbations requiring hospitalization in early life appear to me drivers of loss of lung function later in childhood.•In this study we confirm that respiratory exacerbations requiring hosp...

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Published in:Journal of cystic fibrosis 2022-11, Vol.21 (6), p.977-983
Main Authors: Begum, Nelufa, Byrnes, Catherine A, Cheney, Joyce, Cooper, Peter J, Fantino, Emmanuelle, Gailer, Nicholas, Grimwood, Keith, GutierrezCardenas, Diana, Massie, John, Robertson, Colin F, Sly, Peter D, Tiddens, Harm AWM, Wainwright, Claire E, Ware, Robert S
Format: Article
Language:English
Subjects:
ACFBAL study
Adolescent
Adult
Aspergillus
CF-FAB study
Child
Child, Preschool
Cystic Fibrosis - complications
Forced Expiratory Volume
Hospitalisation
Humans
Longitudinal Studies
Lung
Neutrophilic inflammation
Respiratory exacerbations
Spirometry
Vital Capacity
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Summary:•Loss of lung function during late childhood remains a problem for patients with cystic fibrosis.•Respiratory exacerbations requiring hospitalization in early life appear to me drivers of loss of lung function later in childhood.•In this study we confirm that respiratory exacerbations requiring hospitalization in the first 5 years of life, together with markers of neutrophilic inflammation drive loss of lung function from childhood to adolescence. Despite improvements in general health and life expectancy in people with cystic fibrosis (CF), lung function decline continues unabated during adolescence and early adult life. We examined factors present at age 5-years that predicted lung function decline from childhood to adolescence in a longitudinal study of Australasian children with CF followed from 1999 to 2017. Lung function trajectories were calculated for 119 children with CF from childhood (median 5.0 [25%-75%=5.0–5.1]) years) to early adolescence (median 12.5 [25%-75%=11.4–13.8] years). Lung function fell progressively, with mean (standard deviation) annual change -0.105 (0.049) for forced vital capacity (FVC) Z-score (p
ISSN:1569-1993
1873-5010
DOI:10.1016/j.jcf.2022.03.008