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Research

BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosis

Cystic fibrosis (CF) is a rare, inherited, life-limiting condition predominantly affecting the lungs, for which there is no cure. The disease is characterized by recurrent pulmonary exacerbations (PEx), which are thought to drive progressive lung damage. Management of these episodes is complex and generally involves multiple interventions targeting different aspects of disease. The emergence of innovative trials and use of Bayesian statistical methods has created renewed opportunities for studying heterogeneous populations in rare diseases.

Research

Pseudomonas aeruginosa Resistance to Bacteriophages and Its Prevention by Strategic Therapeutic Cocktail Formulation

Antimicrobial resistance poses a significant threat to modern healthcare as it limits treatment options for bacterial infections, particularly impacting those with chronic conditions such as cystic fibrosis (CF). Viscous mucus accumulation in the lungs of individuals genetically predisposed to CF leads to recurrent bacterial infections, necessitating prolonged antimicrobial chemotherapy. Pseudomonas aeruginosa infections are the predominant driver of CF lung disease, and airway isolates are frequently resistant to multiple antimicrobials.

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

Cystic fibrosis clinical isolates of aspergillus fumigatus induce similar muco-inflammatory responses in primary airway epithelial cells

Aspergillus 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

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 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

Born with cystic fibrosis, now research is her life’s work

When Ingrid Laing was born, the outlook for kids with cystic fibrosis was bleak. Her parents were told she might make it to 20 if she was lucky.

News & Events

Healthy kids help medical research

That's why Melissa has signed up her four year old healthy son Odin for a study at Perth's The Kids Research Institute Australia that will help kids with cystic fibrosis.

News & Events

Prestigious award for cystic fibrosis researcher

Professor Stephen Stick has been awarded the prestigious Richard C. Talamo Distinguished Clinical Achievement Award from the US Cystic Fibrosis Foundation.