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Are you interested in cystic fibrosis research?

We are looking for 6 new members to join our Child and Adolescent Cystic Fibrosis Consumer Reference Group of WA

Research

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.

Research

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...

Research

Infection, inflammation,and lung function decline in infants with cystic fibrosis

Better understanding of evolution of lung function in infants with cystic fibrosis...

Research

Automatic bronchus and artery analysis on chest computed tomography to evaluate the effect of inhaled hypertonic saline in children aged 3-6 years with cystic fibrosis in a randomized clinical trial

SHIP-CT showed that 48-week treatment with inhaled 7% hypertonic saline (HS) reduced airway abnormalities on chest CT using the manual PRAGMA-CF method relative to isotonic saline (IS) in children aged 3-6 years with cystic fibrosis (CF). An algorithm was developed and validated to automatically measure bronchus and artery (BA) dimensions of BA-pairs on chest CT. Aim of the study was to assess the effect of HS on bronchial wall thickening and bronchial widening using the BA-analysis.

Research

Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial

Primary ciliary dyskinesia (PCD) is a rare, progressive, inherited ciliopathic disorder, which is incurable and frequently complicated by the development of bronchiectasis. There are few randomised controlled trials (RCTs) involving children and adults with PCD and thus evidence of efficacy for interventions are usually extrapolated from people with cystic fibrosis.

Research

The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial

In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7% hypertonic saline improved the lung clearance index in children aged 3-6 years with cystic fibrosis, but it remained unclear whether improvement is also seen in structural lung disease. We aimed to assess the effect of inhaled hypertonic saline on chest CT imaging in children aged 3-6 years with cystic fibrosis.

Research

CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosis

Whether airway mucosal acidification drives early progressive lung disease is controversial

Research

The effect of 100% oxygen on tidal breathing parameters in preschool children

This study aimed to determine whether breathing 100% oxygen altered tidal breathing parameters in healthy young children and young children with CF.