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The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesia

Structural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.

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...

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis

In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.

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

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

Exciting new clinical trials in cystic fibrosis: Infants need not apply

The recent announcement of the negative results of the TIGER- 2 phase 3 study of denufosol tetrasodium

Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosis

We investigated whether cyanide in bronchoalveolar lavage (BAL) fluid could be used as an early diagnostic biomarker of infection in kids with cystic fibrosis