Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier ...

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

This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2016
• January 2016

To examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed... ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2013
• July 2013

Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes. ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2011
• May 2011

erology, predicting, Pseudomonas aeruginosa infection, young children, cystic fibrosis ...

• 1. Telethon Kids Institute
• Our research
• Reports and Findings
• 2010
• November 2010

Newly developed quantitative chest computed tomography (CT) outcomes designed specifically to assess structural abnormalities related to cystic fibrosis (CF) lung disease are now available. CFTR modulators potentially can reduce some structural lung abnormalities. We aimed to investigate the effect of CFTR modulators on structural lung disease progression using different quantitative CT analysis methods specific for people with CF (PwCF). ...

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

Structural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system. ...

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

Kathryn Ramsey KR BSc (Hons), PhD Co-Head, Children's Lung Health kathryn.ramsey@telethonkids.org.au Co-Head, Children's Lung Health Associate Professor Kathryn Ramsey is a National Health and Medical Research Council (NHMRC) Emerging Leader (EL2) ...

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

Scedosporium spp. are the second most prevalent filamentous fungi after Aspergillus spp. recovered from cystic fibrosis (CF) patients in various regions of the world. Although invasive infection is uncommon prior to lung transplantation, fungal colonization may be a risk factor for invasive disease with attendant high mortality post-transplantation. Abundant in the environment, Scedosporium aurantiacum has emerged as an important fungal pathogen in a range of clinical settings. ...

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

The marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments. We aimed to predict the progression of bronchiectasis in preschool children with CF. ...

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

In this cohort, as compared with the AREST CF cohort, the authors highlight the limited correlation between infection and inflammation with lung function and structural impairment, and that this was mainly explained by the mild changes identified in lung function and on chest CT scan. ...

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

There is no consensus on how best to measure responses to interventions among children and adults with cystic fibrosis (CF). We have systematically reviewed and summarised the characteristics and measurement properties of tests and tools that have been used to capture outcomes in studies among people with CF, including their reliability, validity and responsiveness. This review is intended to guide researchers when selecting tests or tools for measuring treatment effects in CF trials. A consensus set of these tests and tools could improve consistency in how outcomes are captured and thereby facilitate comparisons and synthesis of evidence across studies. ...

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