title: Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It?
abstract: The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis (CF). Pulmonary inflammation, infection, and structural lung damage manifest very early in life and are prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer-term clinical trajectory for individuals with CF. This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for future clinical practice and research.
authors.affiliation: 1 Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Victoria, Australia.|2 Infection and Immunity, Murdoch Children's Research Institute, Melbourne, Victoria, Australia.|3 Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Sarath C
authors.initials: SC
authors.lastname: Ranganathan
authors.affiliation: 4 Telethon Kids Institute, Perth, Western Australia, Australia.|5 Centre for Child Health Research, University of Western Australia, Perth, Western Australia, Australia.|6 School of Physiotherapy and Exercise Science, Curtin University, Perth, Western Australia, Australia.
authors.forename: Graham L
authors.initials: GL
authors.lastname: Hall
authors.affiliation: 7 Department of Respiratory and Sleep Medicine, Children's Health Queensland, South Brisbane, Queensland, Australia.|8 Child Health Research Centre, University of Queensland, Brisbane, Queensland, Australia; and.
authors.forename: Peter D
authors.initials: PD
authors.lastname: Sly
authors.affiliation: 4 Telethon Kids Institute, Perth, Western Australia, Australia.|5 Centre for Child Health Research, University of Western Australia, Perth, Western Australia, Australia.|9 Department of Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Western Australia, Australia.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Stick
authors.affiliation: 4 Telethon Kids Institute, Perth, Western Australia, Australia.|7 Department of Respiratory and Sleep Medicine, Children's Health Queensland, South Brisbane, Queensland, Australia.
authors.forename: Tonia A
authors.initials: TA
authors.lastname: Douglas
mesh_terms: D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology* / Q000628:therapy*; D006801:Humans; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D008171:Lung Diseases / Q000150:complications / Q000503:physiopathology
keywords: cystic fibrosis; early intervention; early lung disease; newborn screening