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Research

Variation in treatment preferences of pulmonary exacerbations among Australian and New Zealand cystic fibrosis physicians

Despite 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.

Research

Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model

Severity 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

Predicting disease progression in cystic fibrosis

Comprehensive scores incorporating clinical, lung function, imaging and laboratory data will become essential in the future for predicting disease progression and for use in clinical trials

News & Events

$3.4mill to improve treatment management of cystic fibrosis

The Kids researchers have been awarded over $3.4 million for a new trial to pioneer improved ways for managing cystic fibrosis (‘CF’).

News & Events

Cystic fibrosis research a Eureka Prize finalist

A world-leading cystic fibrosis research program, based at The Kids Research Institute Australia, is a finalist in the 2015 Australian Museum Eureka Prizes.

News & Events

Healthy lungs, healthy life

The lungs are one of the last organs in the body to develop as a baby grows. They're also one of the most important.

Research

Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

This 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

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.