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Research

Lentiviral vector gene therapy and CFTR modulators show comparable effectiveness in cystic fibrosis rat airway models

Mutation-agnostic treatments such as airway gene therapy have the potential to treat any individual with cystic fibrosis (CF), irrespective of their CF transmembrane conductance regulator (CFTR) gene variants. The aim of this study was to employ two CF rat models, Phe508del and CFTR knockout to assess the comparative effectiveness of CFTR modulators and lentiviral vector-mediated gene therapy. 

Research

Transcriptomic analysis of primary nasal epithelial cells reveals altered interferon signalling in preterm birth survivors at one year of age

Many survivors of preterm birth (<37 weeks gestation) have lifelong respiratory deficits, the drivers of which remain unknown. Influencers of pathophysiological outcomes are often detectable at the gene level and pinpointing these differences can help guide targeted research and interventions. This study provides the first transcriptomic analysis of primary nasal airway epithelial cells in survivors of preterm birth at approximately 1 year of age.

Research

Respiratory and chest wall mechanics in very preterm infants

Data on static compliance of the chest wall (Ccw) in preterm infants are scarce. We characterized the static compliance of the lung and Ccw to determine their relative contribution to static compliance of the respiratory system in very preterm infants at 36 wk postmenstrual age. We also aimed to investigate how these compliances were influenced by the presence of bronchopulmonary dysplasia and impacted breathing variables.

Research

The role of exome sequencing in childhood interstitial or diffuse lung disease

Children’s interstitial and diffuse lung disease (chILD) is a complex heterogeneous group of lung disorders. Gene panel approaches have a reported diagnostic yield of ~ 12%. No data currently exist using trio exome sequencing as the standard diagnostic modality.

Research

Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infection

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.

Expertise and technologies

The Respiratory Physiology Platform at the Wal-yan Respiratory Research Centre offers access to specialist equipment housed within the outpatient research department at Perth Children’s Hospital, dedicated for research use.

News & Events

Researchers one step closer to preventing asthma in children

Wal-yan Respiratory Research Centre PhD student Niamh Troy has found how OM85 helps babies fight off severe lung infections.

News & Events

Thumbs up from first preterm study participant to use new lung function testing equipment

Natasha, who is a participant in the West Australian Lung Health in Prematurity (WALHIP) study, this week became the first person to receive a lung health assessment using new state-of-the-art lung function testing equipment at Perth Children’s Hospital.

News & Events

Wal-yan Centre welcomes new PhD scholarship awardee

The Wal-yan Respiratory Research Centre this month welcomed new PhD scholarship awardee Yaqin Alziyadat, whose exciting research work will support the Centre’s vision to ensure all children have healthy lungs for life.

News & Events

Perth campaign aims to raise awareness of dangers of a chronic wet cough in Aboriginal children

An intensive health promotion campaign which aims to raise awareness of the dangers of a chronic wet cough in Aboriginal children launched this month in Perth.