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Minimal structural lung disease in early life represents significant pathology

In this cohort, as compared with the AREST CF cohort, the authors highlight the limited correlation between infection and inflammation with lung function and structural impairment, and that this was mainly explained by the mild changes identified in lung function and on chest CT scan.

Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review

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 imaging in cystic fibrosis studies: What counts, and can be counted?

The aim of this study is to characterize the role of currently available CT and MRI markers in clinical studies, and to discuss challenges with CF studies.

The effect of 100% oxygen on tidal breathing parameters in preschool children

This study aimed to determine whether breathing 100% oxygen altered tidal breathing parameters in healthy young children and young children with CF.

Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies

In this review, we consider recent evidence regarding hypoxia and sterile inflammation in cystic fibrosis airways

Alpha-1 antitrypsin mitigates the inhibition of airway epithelial cell repair by neutrophil elastase

Free NE activity is deleterious for epithelial homeostasis and support the hypothesis that proteases in the airway contribute to CF structural lung disease

Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis

Lung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with cystic fibrosis

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

The present study investigated whether lung function measured by forced oscillation technique would be impaired in the presence of infection,...

Protocol for a study of the psychosocial determinants of health in early childhood among children with cystic fibrosis

This protocol outlines the study aims to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcomes in...