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Lung structural and functional impairments in young children with cystic fibrosis diagnosed following newborn screening – A nationwide observational studyNon-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV1, functional and structural lung magnetic resonance imaging (MRI) outcomes in Swiss children with CF diagnosed following newborn screening.
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Psychosocial characteristics and predictors of health-care use in families of young children with cystic fibrosis in Western AustraliaThis study aimed to identify psychosocial predictors in cystic fibrosis that may inform intervention strategies.
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Scoping review of the literature about family-centred care with caregivers of children with cystic fibrosisNeed for research into family-centred caring as a way of caring for children and families where one or more of the children have cystic fibrosis
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Multiple-breath washout as a lung function test in cystic fibrosis: A cystic fibrosis foundation workshop reportRecent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians...
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Early pulmonary inflammation and lung damage in children with cystic fibrosisAirway inflammation and infection are present from early in life, often before children are symptomatic.
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Early surveillance of infants and preschool children with cystic fibrosisSensitive and non-invasive surveillance tools are needed for the clinical management of infants and preschool children with cystic fibrosis (CF). The lung clearance index from the multiple breath washout and functional and morphological outcomes from magnetic resonance imaging provide promising alternatives to current gold standard techniques. Early detection and treatment of lung disease during this important period offers the opportunity to improve the quality of life for individuals with CF.
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Identifying pediatric lung disease: A comparison of forced oscillation technique outcomesThese findings suggest the utility of specific FOT outcomes is dependent on the respiratory disease being assessed
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Early respiratory viral infections in infants with cystic fibrosisEarly viral infections were associated with greater neutrophilic inflammation and bacterial pathogens
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Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosisThis study developed and tested a highly usable, and moderately acceptable, smartphone app to improve the psychosocial health of young people living with CF
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CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosisCFTR-dependent imbalance of macrophage phenotypes and functions could contribute to the exaggerated inflammatory response seen in CF lung disease