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There is no consensus on how best to measure responses to interventions among children and adults with cystic fibrosis (CF). We have systematically reviewed and summarised the characteristics and measurement properties of tests and tools that have been used to capture outcomes in studies among people with CF, including their reliability, validity and responsiveness. This review is intended to guide researchers when selecting tests or tools for measuring treatment effects in CF trials. A consensus set of these tests and tools could improve consistency in how outcomes are captured and thereby facilitate comparisons and synthesis of evidence across studies.

Cystic fibrosis (CF) is the most common chronic, life-shortening genetic condition affecting young Australians. There is no cure but researchers are working to prevent the onset of lung disease.

Tracheobronchomalacia (TBM) is characterised by abnormal collapsibility of the trachea and bronchi, often seen in children with cystic fibrosis (CF). This study aims to determine the impact of TBM on hospital admissions in young children with CF.

Pulmonary exacerbations pose a significant clinical burden on people with cystic fibrosis (pwCF). Whether management of exacerbations should change in the context of modulator therapy is unclear. We describe the characteristics, treatment and lung function outcomes of pulmonary exacerbations requiring intravenous antibiotic therapy (PERITs) in a contemporary Australian cohort of pwCF, in an era of rapidly broadening access to modulator therapy.

Positive expiratory pressure devices are frequently used for airway clearance in children with cystic fibrosis and tracheobronchomalacia. This study aimed to establish if electrical impedance tomography is a feasible measure to titrate pressures in non-sedated children.

The Airway Epithelial Research Team is investigating the role of the epithelium in the development of airway diseases including asthma, cystic fibrosis and lung transplant rejection.

The Kids Research Institute Australia spin-off company, Respirion, received $20 million in funding to develop a promising new therapy.

The family of two girls with cystic fibrosis are hopeful after The Kids Research Institute Australia spin-off company, Respirion, receives $20 million in funding to develop a promising new therapy.

A The Kids Research Institute Australia spin-off company has received $20 million from the Medical Research Commercialisation Fund to develop a promising new therapy for the treatment of Cystic Fibrosis.

The Kids researchers are pioneering an exciting new approach to clinical trials, which aims to fast-track the best treatments for people with rare and complex diseases.