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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cellsThis study assessed an alternative approach, using a small scale halide assay that can be adapted for a personalized high throughput setting to analyze CFTR function of pAEC.
Research
Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infectionThis study aimed to determine how pulmonary inflammation & infection impacts on ventilation distribution throughout early life in people with cystic fibrosis.
Research
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...
Research
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,...
Research
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.
Research
Interpretation of lung function in infants and young children with cystic fibrosisThis paper is a review of lung function tests best suited to use for early detection of lung disease in Cystic Fibrosis.
Research
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.
Research
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
News & Events
$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’).