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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.
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Azithromycin reduces bronchial wall thickening in infants with cystic fibrosisCOMBAT-CF showed that children aged 0-3 years treated with azithromycin did clinically better than placebo but there was no effect on CT-scores. We reanalysed CTs using an automatic bronchus-artery (BA) analysis.
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Virome assembly reveals draft genomes of native Pseudomonas phages isolated from a paediatric bronchoalveolar lavage sampleWe present lung virome data recovered through shotgun metagenomics in bronchoalveolar lavage fluid from an infant with cystic fibrosis, who tested positive for Stenotrophomonas maltophilia infection. Using a bioinformatic pipeline for virus characterization in shotgun metagenomic data, we identified five viral contigs representing Pseudomonas phages classified as Caudoviricetes.
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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
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Distribution of Early Structural Lung Changes due to Cystic Fibrosis Detected with Chest Computed TomographyTo examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed...
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.
Research
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.
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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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Early nasal microbiota and subsequent respiratory tract infections in infants with cystic fibrosisRespiratory tract infections (RTIs) drive lung function decline in children with cystic fibrosis (CF). While the respiratory microbiota is clearly associated with RTI pathogenesis in infants without CF, data on infants with CF is scarce. We compared nasal microbiota development between infants with CF and controls and assessed associations between early-life nasal microbiota, RTIs, and antibiotic treatment in infants with CF.
Research
The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesiaStructural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.