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Research
SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair ModulationCystic fibrosis (CF), due to pathogenic variants in CFTR gene, is associated with chronic infection/inflammation responsible for airway epithelium alteration and lung function decline. Modifier genes induce phenotype variability between people with CF (pwCF) carrying the same CFTR variants. Among these, the gene encoding for the amino acid transporter SLC6A14 has been associated with lung disease severity and age of primary airway infection by the bacteria Pseudomonas aeruginosa.
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Survival of people with cystic fibrosis in AustraliaSurvival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival.
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Cystic Fibrosis Early Surveillance ProgramResearchers are able to track the progress of lung disease through a comprehensive longitudinal set of biological samples, images and data archives.
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Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosisTreatment for pulmonary exacerbations of cystic fibrosis (CF) can produce a range of positive and negative outcomes. Understanding which of these outcomes are achievable and desirable to people affected by disease is critical to agreeing to goals of therapy and determining endpoints for trials.
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Posaconazole-induced hypertension in children with cystic fibrosisPosaconazole is a triazole antifungal with a broad spectrum of activity against moulds including Aspergillus spp. Emerging data suggest posaconazole may be effective in the treatment of allergic bronchopulmonary aspergillosis complicating cystic fibrosis.
News & Events
New study recommends changes to cystic fibrosis monitoring in young childrenA new Australian study that looked at the long term impacts of early lung infections in young kids with cystic fibrosis has recommended changes to monitoring
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Early detection of lung function abnormalities in young children with cystic fibrosisIn this review, we have examined the role of lung function testing in infants and preschool children with CF.
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Stability of interleukin 8 and neutrophil elastase in bronchoalveolar lavage fluid following long-term storageInterleukin-8 (IL-8) and neutrophil elastase (NE) are commonly measured markers of inflammation in bronchoalveolar lavage (BAL) fluid from patients with cystic
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Quantitative assessment of airway dimensions in young children with cystic fibrosis lung disease using chest computed tomographyTo evaluate lung disease progression using AA dimensions on chest CT over 2-years in young CF patients longitudinally and compare to disease controls.
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The cumulative effect of inflammation and infection on structural lung disease in early cystic fibrosisPulmonary inflammation in surveillance bronchoalveolar lavage has a cumulative effect on structural lung disease extent, more so than infection