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Systems biology and bile acid signalling in microbiome-host interactions in the cystic fibrosis lungThe study of the respiratory microbiota has revealed that the lungs of healthy and diseased individuals harbour distinct microbial communities. Imbalances in these communities can contribute to the pathogenesis of lung disease. How these imbalances occur and establish is largely unknown. This review is focused on the genetically inherited condition of Cystic Fibrosis.
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Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomographyOur data suggest that FRC PC-CTs are less sensitive than TLC PC-CTs and that FB-CTs have similar sensitivity to PC-CTs in detecting lung disease
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BAL Inflammatory Markers Can Predict Pulmonary Exacerbations in Children With Cystic FibrosisPulmonary exacerbations in cystic fibrosis are characterized by airway inflammation and may cause irreversible lung damage. Early identification of such exacerbations may facilitate early initiation of treatment, thereby potentially reducing long-term morbidity. Research question: Is it possible to predict pulmonary exacerbations in children with cystic fibrosis, using inflammatory markers obtained from BAL fluid?
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Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiencesSensitive measures of early lung disease are being integrated into therapeutic trials and clinical practice in cystic fibrosis (CF). The impact of early disease surveillance (EDS) using these novel and often intensive techniques on young children and their families is not well researched.
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Lung inflammation and simulated airway resistance in infants with cystic fibrosisCystic fibrosis (CF) is characterized by small airway disease; but central airways may also be affected. We hypothesized that airway resistance estimated from computational fluid dynamic (CFD) methodology in infants with CF was higher than controls and that early airway inflammation in infants with CF is associated with airway resistance.
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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
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The feasibility of using electrical impedance tomography to guide positive pressure airway clearance in children with cystic fibrosis and tracheobronchomalaciaPositive expiratory pressure devices are frequently used for airway clearance in children with cystic fibrosis and tracheobronchomalacia. This study aimed to establish if electrical impedance tomography is a feasible measure to titrate pressures in non-sedated children.
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The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesiaStructural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.
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Feasibility of unsedated lung MRI in young children with cystic fibrosisKathryn Ramsey BSc (Hons), PhD Co-Head, Foundations of Lung Disease kathryn.ramsey@thekids.org.au Co-Head, Foundations of Lung Disease Associate
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Early nasal microbiota and subsequent respiratory tract infections in infants with cystic fibrosisRespiratory tract infections (RTIs) drive lung function decline in children with cystic fibrosis (CF). While the respiratory microbiota is clearly associated with RTI pathogenesis in infants without CF, data on infants with CF is scarce. We compared nasal microbiota development between infants with CF and controls and assessed associations between early-life nasal microbiota, RTIs, and antibiotic treatment in infants with CF.