title: Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection.
abstract: Measures of ventilation distribution are promising for monitoring early lung disease in cystic fibrosis (CF). This study describes the cross-sectional and longitudinal impacts of pulmonary inflammation and infection on ventilation homogeneity in infants with CF.Infants diagnosed with CF underwent multiple breath washout (MBW) testing and bronchoalveolar lavage at three time points during the first 2 years of life.Measures were obtained for 108 infants on 156 occasions. Infants with a significant pulmonary infection at the time of MBW showed increases in lung clearance index (LCI) of 0.400 units (95% CI 0.150-0.648; p=0.002). The impact was long lasting, with previous pulmonary infection leading to increased ventilation inhomogeneity over time compared to those who remained free of infection (p<0.05). Infection with Haemophilus influenzae was particularly detrimental to the longitudinal lung function in young children with CF where LCI was increased by 1.069 units for each year of life (95% CI 0.484-1.612; p<0.001).Pulmonary infection during the first year of life is detrimental to later lung function. Therefore, strategies aimed at prevention, surveillance and eradication of pulmonary pathogens are paramount to preserve lung function in infants with CF.
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Shannon J
authors.initials: SJ
authors.lastname: Simpson
authors.affiliation: Murdoch Children's Research Institute, Melbourne, Australia Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia Department of Paediatrics, University of Melbourne, Melbourne, Australia.
authors.forename: Sarath
authors.initials: S
authors.lastname: Ranganathan
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Judy
authors.initials: J
authors.lastname: Park
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Lidija
authors.initials: L
authors.lastname: Turkovic
authors.affiliation: Department of Microbiology and Immunology, University of Melbourne, Melbourne, Australia.
authors.forename: Roy M
authors.initials: RM
authors.lastname: Robins-Browne
authors.affiliation: Murdoch Children's Research Institute, Melbourne, Australia Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Billy
authors.initials: B
authors.lastname: Skoric
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Kathryn A
authors.initials: KA
authors.lastname: Ramsey
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Tim
authors.identifier: http://orcid.org/0000-0003-3727-5131
authors.initials: T
authors.lastname: Rosenow
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Georgia L
authors.initials: GL
authors.lastname: Banton
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia.
authors.forename: Luke
authors.initials: L
authors.lastname: Berry
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia Department of Respiratory and Sleep Medicine, Princess Margaret Hospital for Children, Perth, Australia School of Paediatrics and Child Health, University of Western Australia, Perth, Australia.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Stick
authors.affiliation: Telethon Kids Institute, University of Western Australia, Subiaco, Australia graham.hall@telethonkids.org.au.
authors.forename: Graham L
authors.initials: GL
authors.lastname: Hall
mesh_terms: D001944:Breath Tests; D018893:Bronchoalveolar Lavage; D001992:Bronchoalveolar Lavage Fluid / Q000276:immunology; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000276:immunology / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D006192:Haemophilus Infections / Q000276:immunology / Q000503:physiopathology*; D006193:Haemophilus influenzae; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016209:Interleukin-8 / Q000276:immunology; D008137:Longitudinal Studies; D008297:Male; D018410:Pneumonia, Bacterial / Q000276:immunology / Q000503:physiopathology*; D011552:Pseudomonas Infections / Q000276:immunology / Q000503:physiopathology*; D011550:Pseudomonas aeruginosa; D055732:Pulmonary Aspergillosis / Q000276:immunology / Q000503:physiopathology*; D012123:Pulmonary Ventilation; D013203:Staphylococcal Infections / Q000276:immunology / Q000503:physiopathology*; D013211:Staphylococcus aureus
chemical_list: C508606:CXCL8 protein, human; D016209:Interleukin-8