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Increased rate of lung function decline in Australian adolescents with cystic fibrosis
Author(s) -
Welsh Liam,
Robertson Colin F.,
Ranganathan Sarath C.
Publication year - 2014
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.22946
Subject(s) - medicine , cystic fibrosis , spirometry , cohort , body mass index , pediatrics , pulmonary function testing , cystic fibrosis related diabetes , vital capacity , lung , lung function , obesity , diffusing capacity , asthma , insulin resistance , impaired glucose tolerance
Summary Introduction Though baseline lung function as measured by spirometry in children with cystic fibrosis (CF) has improved, the annual rate of decline has not changed significantly during the critical period of adolescence. The aim of this study was to describe factors associated with longitudinal decline in lung function in a contemporary cohort of children with CF. Methods Best annual lung function data from children attending the CF service of the Royal Children's Hospital Melbourne were reviewed to determine rate of decline in FEV 1 up until time of transfer to an adult center. Mixed models were used to determine the influence of age, sex, genotype, newborn screening, respiratory hospitalization, CF related diabetes mellitus (CFRD), pancreatic insufficiency, Pseudomonas aeruginosa ( PsA ) infection, and body mass index (BMI) on lung function decline. Results Longitudinal lung function data (range 5–20 years) were obtained for 98 patients with CF (55 male). Overall, the annual rate of decline in FEV 1 % predicted for the entire cohort was 1.4% per annum though the greatest rate of FEV 1 decline was seen during adolescence (2.6%). Increasing age, homozygous ΔF508 genotype, CFRD, mucoid PsA infection, pancreatic insufficiency and respiratory hospitalizations were all significant predictors of FEV 1 decline. Conclusion FEV 1 declines at its sharpest rate during adolescence even in the presence of newborn screening. Genotype, increasing age, CFRD, PsA infection, pancreatic insufficiency and a greater number of respiratory hospitalizations are all associated with an increased rate of lung function decline in Australian children and adolescents with cystic fibrosis. Pediatr Pulmonol. 2014; 49:873–877. © 2013 Wiley Periodicals, Inc.