title: Early pulmonary inflammation and lung damage in children with cystic fibrosis.
abstract: Individuals with cystic fibrosis (CF) suffer progressive airway inflammation, infection and lung damage. Airway inflammation and infection are present from early in life, often before children are symptomatic. CF gene mutations cause changes in the CF transmembrane regulator protein that result in an aberrant airway microenvironment including airway surface liquid (ASL) dehydration, reduced ASL acidity, altered airway mucin and a dysregulated inflammatory response. This review discusses how an altered microenvironment drives CF lung disease before overt airway infection, the response of the CF airway to early infection, and methods to prevent inflammation and early lung disease.
authors.affiliation: Department of Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Western Australia, Australia; School of Paediatric and Child Health, University of Western Australia, Perth, Western Australia, Australia; Telethon Kids Institute, Perth, Western Australia, Australia.
authors.forename: André
authors.initials: A
authors.lastname: Schultz
authors.forename: Stephen
authors.initials: S
authors.lastname: Stick
mesh_terms: D001826:Body Fluids / Q000737:chemistry; D002648:Child; D003550:Cystic Fibrosis / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007249:Inflammation / Q000276:immunology*; D008168:Lung / Q000737:chemistry / Q000276:immunology* / Q000378:metabolism; D055370:Lung Injury / Q000276:immunology*; D009077:Mucins / Q000737:chemistry / Q000276:immunology* / Q000378:metabolism; D011014:Pneumonia / Q000276:immunology*
chemical_list: D009077:Mucins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: biochemistry; cell biology; cystic fibrosis; infection and inflammation; paediatric lung disease