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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...

Treat early to protect lungs

Early intervention is being touted as the key to preventing lung damage in children with cystic fibrosis.

Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies

In this review, we consider recent evidence regarding hypoxia and sterile inflammation in cystic fibrosis airways

Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil Activity

Here we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity

Elastase Exocytosis by Airway Neutrophils Associates with Early Lung Damage in Cystic Fibrosis Children

Protease elastase exocytosis by airway neutrophils occurs in all cystic fibrosis children, and its cellular measure correlates with early lung damage

Discrete choice experiment to evaluate preferences of patients with cystic fibrosis among alternative treatment-related health outcomes: A protocol

The aim of this study is to identify and quantify the preferences of patients with cystic fibrosis regarding treatment outcomes

CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCD

Structural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients