There is no data exclusively on the relationship between health-related quality-of-life (HRQOL) and lung disease severity in early school-aged children with cystic fibrosis (CF). Using data from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) we assessed the relationships between HRQOL, lung function and structure. ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2021
• November 2021

A population of neutrophils recruited into cystic fibrosis (CF) airways is associated with proteolytic lung damage, exhibiting high expression of primary granule exocytosis marker CD63 and reduced phagocytic receptor CD16. Causative factors for this population are unknown, limiting intervention. Here we present a laboratory model to characterize responses of differentiated airway epithelium and neutrophils following respiratory infection. ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2022
• May 2022

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

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2019
• March 2019

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

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2019
• August 2019

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

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2019
• April 2019

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

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2018
• December 2018

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

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2019
• April 2019

The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2017
• September 2017

De novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2018
• July 2018

This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2017
• June 2017

There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations in people with cystic fibrosis (CF). Outcomes used for evaluation should be meaningful; that is, they should capture how people feel, function or survive and be acknowledged as important to people with CF, or should be reliable surrogates of those outcomes. We aimed to summarise the outcomes and corresponding endpoints which have been reported in studies of pulmonary exacerbations, and to identify those which are most likely to be meaningful. ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2020
• November 2020

Chest CT identifies children at an early age who have adverse long-term outcomes ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2020
• May 2020