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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 trialPrimary 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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Protocol for a study of the psychosocial determinants of health in early childhood among children with cystic fibrosisThis protocol outlines the study aims to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcomes in...
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Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapiesIn this review, we consider recent evidence regarding hypoxia and sterile inflammation in cystic fibrosis airways
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The effect of 100% oxygen on tidal breathing parameters in preschool childrenThis study aimed to determine whether breathing 100% oxygen altered tidal breathing parameters in healthy young children and young children with CF.
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Alpha-1 antitrypsin mitigates the inhibition of airway epithelial cell repair by neutrophil elastaseFree 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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CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosisWhether airway mucosal acidification drives early progressive lung disease is controversial
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Using syringe infusion pumps Springfusors for the administration of intravenous antibiotics for children with CF at PCH (SIPS-CF)André Schultz MBChB, PhD, FRACP Head, BREATH Team Head, BREATH Team Prof André Schultz is the Head, BREATH Team at The Kids Research Institute
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$3.4mill to improve treatment management of cystic fibrosisThe Kids researchers have been awarded over $3.4 million for a new trial to pioneer improved ways for managing cystic fibrosis (‘CF’).
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Randomised controlled trials in cystic fibrosis: What, when and how?The major morbidity and mortality from cystic fibrosis (CF) comes from progressive lung disease with bronchiectasis leading to respiratory failure
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Airway macrophages display decreased expression of receptors mediating and regulating scavenging in early cystic fibrosis lung diseaseCystic fibrosis (CF) airway disease is characterized by chronic inflammation, featuring neutrophil influx to the lumen. Airway macrophages (AMs) can promote both inflammation and resolution, and are thus critical to maintaining and restoring homeostasis. CF AM functions, specifically scavenging activity and resolution of inflammation, have been shown to be impaired, yet underlying processes remain unknown.