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Early pulmonary inflammation and lung damage in children with cystic fibrosis
Author(s) -
Schultz André,
Stick Stephen
Publication year - 2015
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.12521
Subject(s) - cystic fibrosis , medicine , inflammation , airway , lung , immunology , cystic fibrosis transmembrane conductance regulator , mucin , pathology , surgery
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.