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On a quest for kids with cystic fibrosis

PRAGMA-CF, a new way of measuring early lung disease in young kids with cystic fibrosis is changing the way we detect and treat CF.

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...

Use of a primary epithelial cell screening tool to investigate phage therapy in cystic fibrosis

This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates

Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis

Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target

MRCF launches Perth-based biotech developing new treatment for Cystic Fibrosis

A The Kids Research Institute Australia spin-off company has received $20 million from the Medical Research Commercialisation Fund to develop a promising new therapy for the treatment of Cystic Fibrosis.

Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis

Background: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.

Chest imaging in cystic fibrosis studies: What counts, and can be counted?

The aim of this study is to characterize the role of currently available CT and MRI markers in clinical studies, and to discuss challenges with CF studies.

Air trapping in early cystic fibrosis lung disease-Does CT tell the full story?

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

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...

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.