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Research
Signal-correction errors in the EasyOne Pro LAB multiple-breath washout device significantly impact outcomes in children and adultsMultiple-breath washout (MBW) is an established technique to assess functional residual capacity (FRC) and ventilation inhomogeneity in the lung. Indirect calculation of nitrogen concentration requires accurate measurement of gas concentrations.
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Role of Tris-CaEDTA as an adjuvant with nebulised tobramycin in cystic fibrosis patients with Pseudomonas aeruginosa lung infections: A randomised controlled trialWe tested if disrupting iron utilisation by P. aeruginosa by adding the Tris-buffered chelating agent CaEDTA to nebulised tobramycin would enhance bacterial clearance and improve lung function in CF patients.
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Changes in airway inflammation with pseudomonas eradication in early cystic fibrosisNeutrophil elastase is a significant risk factor for structural lung disease in cystic fibrosis, and Pseudomonas aeruginosa airway infection is linked with neutrophilic inflammation and substantial respiratory morbidity. We aimed to evaluate how neutrophil elastase (NE) activity changes after P. aeruginosa eradication and influences early disease outcomes. We assessed participants in the AREST CF cohort between 2000 and 2018 who had P. aeruginosa cultured from their routine annual bronchoalveolar lavage (BAL) fluid and who underwent eradication treatment and a post eradication BAL. Factors associated with persistent P. aeruginosa infection, persistent neutrophilic inflammation following eradication and worse structural lung disease one year post-eradication were evaluated.
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Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil ActivityHere we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity
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Single-breath washout and association with structural lung disease in children with cystic fibrosisAcinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT
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Parental experiences of early pulmonary surveillance for children with cystic fibrosis: A research proposal for improved family psychosocial outcomesThe proposed study intends to explore parental experiences, including coping, related to their child's involvement in the early surveillance program.
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Early cystic fibrosis lung diseaseThis paper is about lung disease in patients with cyctic Fibrosis and prevention strategies to slow the onset of lung disease.
News & Events
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
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.