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Promising results for new Western Australian-pioneered cystic fibrosis drugChildren with cystic fibrosis will be included in the next phase of a clinical trial of a promising new treatment pioneered in Western Australia aimed at boosting their immune responses to infections. Originally developed by researchers at The Kids Research Institute Australia and Perth Children’s Hospital.
Research
SYNERGY CF: Getting the best start to life - preventing early cystic fibrosis lung disease by solving the host-inflammation infection conundrumCystic fibrosis related progressive lung disease characterised by inflammation and infection commences soon after birth.
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Telethon Institute awarded two national Centres of Research ExcellenceTelethon Institute awarded two national Centres of Research Excellence
Research
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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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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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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Early lung surveillance of cystic fibrosis: what have we learnt?Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier
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The potential of phage therapy in cystic fibrosisThis review summarises the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways
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Oxidative stress and abnormal bioactive lipids in early cystic fibrosis lung diseaseSeveral lipid biomarkers of early cystic fibrosis lung disease were identified, which point toward potential disease monitoring and therapeutic approaches