Search
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
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
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
Cystic fibrosis clinical isolates of aspergillus fumigatus induce similar muco-inflammatory responses in primary airway epithelial cellsAspergillus is increasingly associated with lung inflammation and mucus plugging in early cystic fibrosis disease during which conidia burden is low and strains appear to be highly diverse. It is unknown whether clinical Aspergillus strains vary in their capacity to induce epithelial inflammation and mucus production.
Research
Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse ModelSeverity and disease progression in people with Cystic Fibrosis is typically dependent on their genotype. One potential therapeutic strategy for people with specific mutations is exon skipping with antisense oligonucleotides. CFTR exon 9 is an in-frame exon and hence the exclusion of this exon would excise only 31 amino acids but not alter the reading frame of the remaining mRNA.
Research
Rhinovirus Infection Is Associated With Airway Epithelial Cell Necrosis and Inflammation via Interleukin-1 in Young Children With Cystic FibrosisThis study found rhinovirus infection drives necrotic cell death in cystic fibrosis airway epithelial cells
Research
Variation in treatment preferences of pulmonary exacerbations among Australian and New Zealand cystic fibrosis physiciansDespite advances in cystic fibrosis (CF) management and survival, the optimal treatment of pulmonary exacerbations remains unclear. Understanding the variability in treatment approaches among physicians might help prioritise clinical uncertainties to address through clinical trials.
News & Events
Cystic fibrosis research a Eureka Prize finalistA world-leading cystic fibrosis research program, based at The Kids Research Institute Australia, is a finalist in the 2015 Australian Museum Eureka Prizes.