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Single-breath washout and association with structural lung disease in children with cystic fibrosis

Acinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

These findings indicate that early CF lung disease is characterized by an increased mucus burden and inflammatory markers without infection or struct…

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

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

New drug therapy provides hope for kids with cystic fibrosis

The family of two girls with cystic fibrosis are hopeful after The Kids Research Institute Australia spin-off company, Respirion, receives $20 millio…

Trial refresh: A case for an adaptive platform trial for pulmonary exacerbations of cystic fibrosis

This review will discuss the necessary steps required for a Bayesian adaptive platform trial to optimize treatment of pulmonary exacerbations of cyst…

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 promi…

Human Primary Epithelial Cell Models: Promising Tools in the Era of Cystic Fibrosis Personalized Medicine

In this Review, we consolidate the latest updates in the development of primary epithelial cellular model systems relevant for cystic fibrosis

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

Elucidating the interaction of CF airway epithelial cells and rhinovirus: Using the host-pathogen relationship to identify future therapeutic strategies

A better understanding of the innate immune responses by CF airway epithelial cells is needed to identify why viral infections are more severe in CF