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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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Draft Genome Sequences of Three Pseudomonas fluorescens Strains Isolated from Marine Sponges Harvested off the West Coast of IrelandThree Pseudomonas sp. strains isolated from marine sponges have shown potential quorum sensing inhibition activity
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Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool childrenWe characterized the lower airways microbiome using BAL samples obtained from clinically stable CF young children who underwent bronchoscopy and chest CT.
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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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Exposure to bile leads to the emergence of adaptive signaling variants in the opportunistic pathogen pseudomonas aeruginosaAdaptive variants of P. aeruginosa that arise following long term bile exposure enables the emergence of ecologically competitive sub-populations
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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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Ultra low dose CT screen-detected non-malignant incidental findings in the Western Australian Asbestos Review ProgrammeThe 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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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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Infection, inflammation,and lung function decline in infants with cystic fibrosisBetter understanding of evolution of lung function in infants with cystic fibrosis...
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Defective function at the epithelial junction: A novel therapeutic frontier in asthma?The airway epithelium forms a highly regulated physical barrier that normally prevents invasion of inhaled pathogens and allergens from the airway lumen.