Professor Stephen Stick has been awarded the prestigious Richard C. Talamo Distinguished Clinical Achievement Award from the US Cystic Fibrosis Foundation. ...

• 1. Telethon Kids Institute
• News & Events
• News & Events at Telethon Kids
• 2015
• October

Pictured: Connor Barrett #00b2a9 Diagnosed with cystic fibrosis (CF) at three weeks of age, Connor Barrett’s daily routine involves 25 separate doses ...

• 1. Telethon Kids Institute
• Our research
• Research Impact
• 2023
• Paradigm shift

Pulmonary exacerbations are associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no consensus about which outcomes should be evaluated in studies of pulmonary exacerbations or how these outcomes should be measured. ...

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

Myeloperoxidase is released by neutrophils in inflamed tissues. MPO oxidizes chloride, bromide, and thiocyanate to produce hypochlorous acid, hypobromous acid, and hypothiocyanous acid, respectively. These oxidants are toxic to pathogens, but may also react with host cells to elicit biological activity and potential toxicity. In cystic fibrosis and related diseases, increased neutrophil inflammation leads to increased airway MPO and airway epithelial cell exposure to its oxidants. ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2023
• Jun 2023

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

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

This review reports on methods used to evaluate airway clearance techniques (ACT) in adults with CF and examined data for evidence of any effect. Sixty-eight studies described ACT in adequate detail and were included in this review. ...

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

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