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Research

Respiratory tract exacerbations revisited: Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatment

For cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in...

Research

Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis

Lung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with cystic fibrosis

Research

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

The present study investigated whether lung function measured by forced oscillation technique would be impaired in the presence of infection,...

Research

Changes in airway inflammation with pseudomonas eradication in early cystic fibrosis

Neutrophil elastase is a significant risk factor for structural lung disease in cystic fibrosis, and Pseudomonas aeruginosa airway infection is linked with neutrophilic inflammation and substantial respiratory morbidity. We aimed to evaluate how neutrophil elastase (NE) activity changes after P. aeruginosa eradication and influences early disease outcomes. We assessed participants in the AREST CF cohort between 2000 and 2018 who had P. aeruginosa cultured from their routine annual bronchoalveolar lavage (BAL) fluid and who underwent eradication treatment and a post eradication BAL. Factors associated with persistent P. aeruginosa infection, persistent neutrophilic inflammation following eradication and worse structural lung disease one year post-eradication were evaluated.

Research

Early cystic fibrosis lung disease

This paper is about lung disease in patients with cyctic Fibrosis and prevention strategies to slow the onset of lung disease.

Research

Parental experiences of early pulmonary surveillance for children with cystic fibrosis: A research proposal for improved family psychosocial outcomes

The proposed study intends to explore parental experiences, including coping, related to their child's involvement in the early surveillance program.

Research

Lung function testing in preschool-aged children with cystic fibrosis in the clinical setting

This study investigated the nature and prevalence of atypical pain responses in Rett syndrome and their relationships with specific MECP2 mutations.

Research

Virome assembly reveals draft genomes of native Pseudomonas phages isolated from a paediatric bronchoalveolar lavage sample

We present lung virome data recovered through shotgun metagenomics in bronchoalveolar lavage fluid from an infant with cystic fibrosis, who tested positive for Stenotrophomonas maltophilia infection. Using a bioinformatic pipeline for virus characterization in shotgun metagenomic data, we identified five viral contigs representing Pseudomonas phages classified as Caudoviricetes.

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Telethon Institute awarded two national Centres of Research Excellence

Telethon Institute awarded two national Centres of Research Excellence

Research

SYNERGY CF: Getting the best start to life - preventing early cystic fibrosis lung disease by solving the host-inflammation infection conundrum

Cystic fibrosis related progressive lung disease characterised by inflammation and infection commences soon after birth.