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EVC-EVC2 complex stability and ciliary targeting are regulated by modification with ubiquitin and SUMOEllis van Creveld syndrome and Weyers acrofacial dysostosis are two rare genetic diseases affecting skeletal development. They are both ciliopathies, as they are due to malfunction of primary cilia, microtubule-based plasma membrane protrusions that function as cellular antennae and are required for Hedgehog signaling, a key pathway during skeletal morphogenesis.
Research
Insights into the associations between the gut microbiome, its metabolites and heart failureHeart failure (HF) is the end stage of most cardiovascular diseases and remains a significant health problem globally. We aimed to assess whether patients with left ventricular ejection fraction ≤45% had alterations in both the gut microbiome profile and production of associated metabolites when compared to a healthy cohort.
Research
Defining the pediatric response to SARS-CoV-2 variantsThe global population has been severely affected by the coronavirus disease 2019 (COVID-19) pandemic, however, with older age identified as a risk factor, children have been underprioritized. This article discusses the factors contributing to the less severe response observed in children following infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), including, differing viral entry receptor expression and immune responses.
Research
Evaluation of the implementation and clinical effects of an intervention to improve medical follow-up and health outcomes for Aboriginal children hospitalised with chest infectionsAboriginal children hospitalised with acute lower respiratory infections (ALRIs) are at-risk of developing bronchiectasis, which can progress from untreated protracted bacterial bronchitis, often evidenced by a chronic (>4 weeks) wet cough following discharge. We aimed to facilitate follow-up for Aboriginal children hospitalised with ALRIs to provide optimal management and improve their respiratory health outcomes.
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Thoracic Society of Australia and New Zealand (TSANZ) position statement on chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New ZealandThis position statement, updated from the 2015 guidelines for managing Australian and New Zealand children/adolescents and adults with chronic suppurative lung disease (CSLD) and bronchiectasis, resulted from systematic literature searches by a multi-disciplinary team that included consumers.
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A complete genome of an obligately lytic Pseudomonas aeruginosa bacteriophage, Minga-mokiny 4We report the isolation of a bacteriophage with obligately lytic activity against Pseudomonas aeruginosa from wastewater. The reported phage, Minga-mokiny 4, appears to belong to the Schitoviridae family, is of the Litunavirus genus, and has a 72,362-bp genome. No known genes associated with lysogeny, bacterial resistance, or virulence were predicted.
Research
Oscillometry and spirometry are not interchangeable when assessing the bronchodilator response in children and young adults born pretermThe European Respiratory Society Oscillometry Taskforce identified that clinical correlates of bronchodilator responses are needed to advance oscillometry in clinical practice. The understanding of bronchodilator-induced oscillometry changes in preterm lung disease is poor. Here we describe a comparison of bronchodilator assessments performed using oscillometry and spirometry in a population born very preterm and explore the relationship between bronchodilator-induced changes in respiratory function and clinical outcomes.

The Wal-yan Respiratory Research Centre offers a broad range of outpatient and portable tests for those affected by chronic respiratory conditions.

Neuromuscular disorders include many rare conditions, such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy, that adversely impact respiratory health.

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Five innovative research projects supported by Inspiration Awards 2022Valued at a total of $440,000, the Wal-yan Respiratory Research Centre Inspiration Awards 2022 will support five cutting-edge research projects.