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News & Events
Healthy kids help medical researchThat'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 researcherProfessor Stephen Stick has been awarded the prestigious Richard C. Talamo Distinguished Clinical Achievement Award from the US Cystic Fibrosis Foundation.

COMBAT CF is one of two long-standing international trials which have resulted in new early intervention options helping to reduce progressive lung damage in kids living with CF.
Research
Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosisPulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcomes should be measured.
Research
Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infectionA population of neutrophils recruited into cystic fibrosis (CF) airways is associated with proteolytic lung damage, exhibiting high expression of primary granule exocytosis marker CD63 and reduced phagocytic receptor CD16. Causative factors for this population are unknown, limiting intervention. Here we present a laboratory model to characterize responses of differentiated airway epithelium and neutrophils following respiratory infection.
Research
Substrate-dependent metabolomic signatures of myeloperoxidase activity in airway epithelial cells: Implications for early cystic fibrosis lung diseaseMyeloperoxidase is released by neutrophils in inflamed tissues. MPO oxidizes chloride, bromide, and thiocyanate to produce hypochlorous acid, hypobromous acid, and hypothiocyanous acid, respectively. These oxidants are toxic to pathogens, but may also react with host cells to elicit biological activity and potential toxicity. In cystic fibrosis and related diseases, increased neutrophil inflammation leads to increased airway MPO and airway epithelial cell exposure to its oxidants.
Research
Multiple-breath washout outcomes are sensitive to inflammation and infection in children with cystic fibrosisThe lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection
Research
Early lung disease in infants and preschool children with cystic fibrosis: What have we learned and what should we do about it?This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research
Research
Feasibility of parental collected nasal swabs for virus detection in young children with cystic fibrosisNo evidence to demonstrate relationships with symptoms and viruses, prolonged symptoms, prolonged shedding or patterns of virus infections in CF
Research
Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosisThe aim of this study was to assess if MMP activation positively correlates with neutrophil elastase activity, disease severity and bronchiectasis in young...