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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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Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosisTreatment for pulmonary exacerbations of cystic fibrosis (CF) can produce a range of positive and negative outcomes. Understanding which of these outcomes are achievable and desirable to people affected by disease is critical to agreeing to goals of therapy and determining endpoints for trials.
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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.
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Bullying and mental health amongst Australian children and young people with cystic fibrosisThis study describes the peer bullying experiences of young people with CF, and examines associations between school bullying and the psychological well-being of these young people
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Airway surface liquid pH is not acidic in children with cystic fibrosisModulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.
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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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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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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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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.