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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
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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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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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Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway diseaseMacrophages are the major resident immune cells in human airways coordinating responses to infection and injury. In cystic fibrosis, neutrophils are recruited to the airways shortly after birth, and actively exocytose damaging enzymes prior to chronic infection, suggesting a potential defect in macrophage immunomodulatory function.
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A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CFDiagnosis of chronic disease in a child can result in unresolved grief (UG) in parents. This study aimed to evaluate the efficacy of psychological insight-oriented therapy (IOT) as a treatment for UG compared to disease related education in parents of children with cystic fibrosis. Sequence of delivery, first IOT then disease related education (or vice versa) was also examined, to let all participants experience both interventions.
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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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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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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