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Dysregulated repair following epithelial injury is a key forerunner of disease in many organs, and the acquisition of a mesenchymal phenotype by the injured...
The clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented.
Cross-sectional studies implicate neutrophilic inflammation and pulmonary infection as risk factors for early structural lung disease in infants and young...
Recently, an established "small macrophage" phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the...
There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations in people with cystic fibrosis (CF). Outcomes used for evaluation should be meaningful; that is, they should capture how people feel, function or survive and be acknowledged as important to people with CF, or should be reliable surrogates of those outcomes. We aimed to summarise the outcomes and corresponding endpoints which have been reported in studies of pulmonary exacerbations, and to identify those which are most likely to be meaningful.
Chest CT identifies children at an early age who have adverse long-term outcomes
Here we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity
Aspergillus species and P. aeruginosa are commonly present in the lower airways from infancy
The aim of this study is to identify and quantify the preferences of patients with cystic fibrosis regarding treatment outcomes
Acinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT