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Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis

There is no data exclusively on the relationship between health-related quality-of-life (HRQOL) and lung disease severity in early school-aged children with cystic fibrosis (CF). Using data from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) we assessed the relationships between HRQOL, lung function and structure.

Methods used to evaluate the immediate effects of airway clearance techniques in adults with cystic fibrosis: A systematic review and meta-analysis

This review reports on methods used to evaluate airway clearance techniques (ACT) in adults with CF and examined data for evidence of any effect. Sixty-eight studies described ACT in adequate detail and were included in this review.

Novel method to select meaningful outcomes for evaluation in clinical trials

A standardised framework for selecting outcomes for evaluation in trials has been proposed by the Core Outcome Measures in Effectiveness Trials working group. However, this method does not specify how to ensure that the outcomes that are selected are causally related to the disease and the health intervention being studied. Causal network diagrams may help researchers identify outcomes that are both clinically meaningful and likely to be causally dependent on the intervention, and endpoints that are, in turn, causally dependent on those outcomes.

Parental experiences of early pulmonary surveillance for children with cystic fibrosis: A research proposal for improved family psychosocial outcomes

The proposed study intends to explore parental experiences, including coping, related to their child's involvement in the early surveillance program.

Protocol For Study Of Information Needs Of Parents Of Infants Newly Diagnosed With Cystic Fibrosis

This study aimed to investigate the information needs, priorities, and information-seeking behaviours of parents of infants newly diagnosed with CF.

Risk factors for bronchiectasis in children with cystic fibrosis

We sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic...

Regional Differences in Susceptibiity of Bronchial Epithelium to Mesenchymal Transition and Inhibition by the Macrolide Antibiotic Azithromycin

Dysregulated repair following epithelial injury is a key forerunner of disease in many organs, and the acquisition of a mesenchymal phenotype by the injured...

Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trial

The clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented.

Progression of early structural lung disease in young children with cystic fibrosis assessed using CT

Cross-sectional studies implicate neutrophilic inflammation and pulmonary infection as risk factors for early structural lung disease in infants and young...

Small macrophages are present in early childhood respiratory disease

Recently, an established "small macrophage" phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the...