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PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic FibrosisChest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease. However, there are no standardized outcome...
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Biomarkers in paediatric Cystic Fibrosis lung diseaseBiomarkers in cystic fibrosis are used for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis,...
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What did we learn from two decades of chest computed tomography in cystic fibrosis?Despite our current treatment, many cystic fibrosis (CF) patients still show progressive bronchiectasis and small airways disease.
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Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?Clinical trials for the treatment of cystic fibrosis (CF) lung disease are important to test and optimise new therapeutic interventions.
The Advancing Innovation in Respiratory (AIR) Health Team is a multi-disciplinary group with skills in clinical medicine, physiology, psychology, and in cellular and molecular biology, that are committed to improving the lives of children with respiratory diseases and their families.
Research
COMBAT CF: A phase 3 multi-centre randomized placebo-controlled study of azithromycin in the primary prevention of radiologically-defined bronchiectasis in infants with cystic fibrosis.A phase 3 multi-centre randomized placebo-controlled study of azithromycin in the primary prevention of radiologically-defined bronchiectasis in infants with cystic fibrosis
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The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosisDe novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF
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Multiple-breath washout outcomes are sensitive to inflammation and infection in children with cystic fibrosisThe lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection
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Early lung disease in infants and preschool children with cystic fibrosis: What have we learned and what should we do about it?This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research
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Reduced transforming growth factor β1 (TGF-β1) in the repair of airway epithelial cells of children with asthmaEvidence into the role of TGF-β1 in airway epithelial repair in asthma is still controversial. This study tested the hypothesis that the reduced TGF-β1 levels previously observed in paediatric asthmatic airway epithelial cells directly contribute to the dysregulated repair seen in these cells.