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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.
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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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
Research
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.
Research
The detection of bile acids in the lungs of paediatric cystic fibrosis patients is associated with altered inflammatory patternsOur work has identified a clinically relevant host-derived factor that may participate in shaping early events in the aetiology of CF respiratory disease
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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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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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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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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.
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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