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Research

CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosis

Whether airway mucosal acidification drives early progressive lung disease is controversial

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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

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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...

Research

LPS binding protein and activation signatures are upregulated during asthma exacerbations in children

Asthma exacerbations in children are associated with respiratory viral infection and atopy, resulting in systemic immune activation and infiltration of immune cells into the airways. The gene networks driving the immune activation and subsequent migration of immune cells into the airways remains incompletely understood. Cellular and molecular profiling of PBMC was employed on paired samples obtained from atopic asthmatic children during acute virus-associated exacerbations and later during convalescence.

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The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial

In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7% hypertonic saline improved the lung clearance index in children aged 3-6 years with cystic fibrosis, but it remained unclear whether improvement is also seen in structural lung disease. We aimed to assess the effect of inhaled hypertonic saline on chest CT imaging in children aged 3-6 years with cystic fibrosis.

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Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial

Primary ciliary dyskinesia (PCD) is a rare, progressive, inherited ciliopathic disorder, which is incurable and frequently complicated by the development of bronchiectasis. There are few randomised controlled trials (RCTs) involving children and adults with PCD and thus evidence of efficacy for interventions are usually extrapolated from people with cystic fibrosis.

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Ultra low dose CT screen-detected non-malignant incidental findings in the Western Australian Asbestos Review Programme

The prevalence of LDCT-detected indeterminate lung nodules in 906 individuals with significant asbestos exposure was 8.5%, lower than many other CT studies

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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

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Exposure to bile leads to the emergence of adaptive signaling variants in the opportunistic pathogen pseudomonas aeruginosa

Adaptive variants of P. aeruginosa that arise following long term bile exposure enables the emergence of ecologically competitive sub-populations

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Normative values for lung, bronchial sizes, and bronchus-artery ratios in chest CT scans: from infancy into young adulthood

To estimate the developmental trends of quantitative parameters obtained from chest computed tomography (CT) and to provide normative values on dimensions of bronchi and arteries, as well as bronchus-artery (BA) ratios from preschool age to young adulthood.