Background: Life expectancy for people with cystic fibrosis (CF) varies considerably both within and between countries. The objective of this study was to compare survival among countries with single-payer healthcare systems while accounting for markers of disease severity.
Methods: This cohort study used data from established national CF registries in Australia, Canada, France and New Zealand from 2015 to 2019.
Background: Newborn screening allows novel treatments for cystic fibrosis (CF) to be trialled in early childhood before irreversible lung injury occurs. As respiratory exacerbations are a potential trial outcome variable, we determined their rate, duration and clinical features in preschool children with CF; and whether they were associated with growth, lung structure and function at age 5 years.
Methods: Respiratory exacerbations were recorded prospectively in Australasian CF Bronchoalveolar Lavage trial subjects from enrolment after newborn screening to age 5 years, when all participants underwent clinical assessment, chest CT scans and spirometry.
Non-cystic fibrosis (CF) bronchiectasis, the abnormal dilatation of bronchial airways, is a heterogeneous condition caused by a variety of lung insults and results in significant morbidity and mortality. Although frequently reported as being an uncommon respiratory disease in the developed world, its impact on the respiratory health of specific populations has recently received increased attention. There are limited data on which to base management strategies.
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