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Research
Airway epithelial repair in health and disease: Orchestrator or simply a player?This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells
Research
Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosisImproved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes.
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The longitudinal microbial and metabolic landscape of infant cystic fibrosis: the gut-lung axisIn cystic fibrosis, gastrointestinal dysfunction and lower airway infection occur early and are independently associated with poorer outcomes in childhood. This study aimed to define the relationship between the microbiota at each niche during the first 2 years of life, its association with growth and airway inflammation, and explanatory features in the metabolome.
News & Events
New drug therapy provides hope for kids with cystic fibrosisThe Kids Research Institute Australia spin-off company, Respirion, received $20 million in funding to develop a promising new therapy.
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.
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.
Research
Exciting new clinical trials in cystic fibrosis: Infants need not applyThe recent announcement of the negative results of the TIGER- 2 phase 3 study of denufosol tetrasodium
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Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic FibrosisIn school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.
Research
Changing Prevalence of Lower Airway Infections in Young Children with Cystic FibrosisAspergillus species and P. aeruginosa are commonly present in the lower airways from infancy
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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.