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Understanding early triggers of Cystic Fibrosis Lung Disease

Genetic factors in airway epithelial cells that are functionally associated with asthma pathogenesis

Wall teichoic acid may be important for protecting S. aureus against exposure to bile

Bile acid signalling is a leading trigger for the development of chronic phenotypes underlying the pathophysiology of chronic respiratory disease

The Advancing Innovation in Respiratory (AIR) Health Team is a multi-disciplinary group with skills in clinical medicine, physiology, psychology, and in cellular and molecular biology, that are committed to improving the lives of children with respiratory diseases and their families.

There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations in people with cystic fibrosis (CF). Outcomes used for evaluation should be meaningful; that is, they should capture how people feel, function or survive and be acknowledged as important to people with CF, or should be reliable surrogates of those outcomes. We aimed to summarise the outcomes and corresponding endpoints which have been reported in studies of pulmonary exacerbations, and to identify those which are most likely to be meaningful.

Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"

The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection

Research to further improve outcomes for people with CF is dependent upon well characterised, archived and accessible clinical specimens.

This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research