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Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV1) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV1 decline in adolescence and adulthood have not slowed. This suggests that interventions aimed at preventing lung disease should be targeted to mild disease and commence in early life.
We tested if disrupting iron utilisation by P. aeruginosa by adding the Tris-buffered chelating agent CaEDTA to nebulised tobramycin would enhance bacterial clearance and improve lung function in CF patients.
In this Review, we consolidate the latest updates in the development of primary epithelial cellular model systems relevant for cystic fibrosis
Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target
This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates
Whether airway mucosal acidification drives early progressive lung disease is controversial
De novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF
Background: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.
Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"
The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection