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Assessment of early bronchiectasis in young children with cystic fibrosis is dependent on lung volumeThe aim of this study was to determine whether assessment of early CT scan-detected bronchiectasis in young children with cystic fibrosis (CF) depends on...
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Determinants of culture success in an airway epithelium sampling program of young children with cystic fibrosisDeterminants of culture success through retrospective analysis of a program of routinely brushing children with Cystic Fibrosis airway disease
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Novel end points for clinical trials in young children with cystic fibrosisCystic fibrosis (CF) lung disease commences early in the disease progression and is the most common cause of mortality.
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Detection of biofilm in bronchoalveolar lavage from children with non-cystic fibrosis bronchiectasisThe presence of Pseudomonas aeruginosa biofilms in lower airway specimens from cystic fibrosis (CF) patients is well established.
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Quantitative assessment of airway dimensions in young children with cystic fibrosis lung disease using chest computed tomographyTo evaluate lung disease progression using AA dimensions on chest CT over 2-years in young CF patients longitudinally and compare to disease controls.
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The detection of bile acids in the lungs of paediatric cystic fibrosis patients is associated with altered inflammatory patternsOur work has identified a clinically relevant host-derived factor that may participate in shaping early events in the aetiology of CF respiratory disease
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Early detection of lung function abnormalities in young children with cystic fibrosisIn this review, we have examined the role of lung function testing in infants and preschool children with CF.
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Cystic Fibrosis Early Surveillance ProgramResearchers are able to track the progress of lung disease through a comprehensive longitudinal set of biological samples, images and data archives.
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Survival of people with cystic fibrosis in AustraliaSurvival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival.
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SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair ModulationCystic fibrosis (CF), due to pathogenic variants in CFTR gene, is associated with chronic infection/inflammation responsible for airway epithelium alteration and lung function decline. Modifier genes induce phenotype variability between people with CF (pwCF) carrying the same CFTR variants. Among these, the gene encoding for the amino acid transporter SLC6A14 has been associated with lung disease severity and age of primary airway infection by the bacteria Pseudomonas aeruginosa.