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Research
Airway surface liquid pH is not acidic in children with cystic fibrosisModulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.
Research
Bullying and mental health amongst Australian children and young people with cystic fibrosisThis study describes the peer bullying experiences of young people with CF, and examines associations between school bullying and the psychological well-being of these young people
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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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Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosisThese findings indicate that early CF lung disease is characterized by an increased mucus burden and inflammatory markers without infection or structural lung disease
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Elucidating the interaction of CF airway epithelial cells and rhinovirus: Using the host-pathogen relationship to identify future therapeutic strategiesA better understanding of the innate immune responses by CF airway epithelial cells is needed to identify why viral infections are more severe in CF
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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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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
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Of pigs, mice, and men: Understanding early triggers of cystic fibrosis lung diseaseUnderstanding early triggers of Cystic Fibrosis Lung Disease
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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.