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New drug offers hope for people living with cystic fibrosis

A promising new treatment pioneered in Western Australia for people with cystic fibrosis has commenced testing in a clinical trial in the United States and Australia.

International clinical trial reduced lung inflammation in young kids with cystic fibrosis

Promising results from an Australian-led clinical trial could drastically change the way we care for young children with cystic fibrosis (CF).

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

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

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

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 cystic fibrosis

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

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

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