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A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosisThe marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments. We aimed to predict the progression of bronchiectasis in preschool children with CF.
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The measurement properties of tests and tools used in cystic fibrosis studies: a systematic reviewThere 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.
Research
Minimal structural lung disease in early life represents significant pathologyIn this cohort, as compared with the AREST CF cohort, the authors highlight the limited correlation between infection and inflammation with lung function and structural impairment, and that this was mainly explained by the mild changes identified in lung function and on chest CT scan.
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Air trapping in early cystic fibrosis lung disease-Does CT tell the full story?Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"
Research
Chest imaging in cystic fibrosis studies: What counts, and can be counted?The aim of this study is to characterize the role of currently available CT and MRI markers in clinical studies, and to discuss challenges with CF studies.
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The AREST CF experience in biobanking - More than just tissues, tubes and time.Research to further improve outcomes for people with CF is dependent upon well characterised, archived and accessible clinical specimens.
Research
Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trialThe clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented.
News & Events
New drug therapy provides hope for kids with cystic fibrosisThe 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.
News & Events
Cure4CF Grant a boost for innovative Cystic Fibrosis researchA $350,000 Cure4 Cystic Fibrosis grant is set to propel the Wal-yan Respiratory Research Centre’s Phage WA program forward, supercharging its fight against antimicrobial resistant (AMR) lung infections in people with Cystic Fibrosis (CF) using cutting-edge phage therapy.
Research
Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosisWe hypothesized that the inflammatory response in the lungs of children with cystic fibrosis (CF) would vary with the type of infecting organism, being...