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The effect of CFTR modulators on structural lung disease in cystic fibrosisNewly 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).
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BANK CF: The Respiratory Centre BIOBANKThe Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) program has grown from an early surveillance program initiated in Perth in 1999, which performed bronchoalveolar lavage (BAL) to evaluate pulmonary infection and inflammation, as well as infant lung function testing.
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SHIP CT: A Phase 3 randomised, double-blind, controlled trial of inhaled 7% hypertonic saline versus 0.9% isotonic saline for 48 weeks in patients with Cystic Fibrosis at 3-6 years of age in parallel with the North American SHIP clinical trial.SHIP-CT, led by Professor Stephen Stick, Director of the Wal-yan Respiratory Research Centre at The Kids, is a unique study in preschool-aged children (from 3-6 years of age) with CF using images of the lung from chest CT scans as the main outcome measure.
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Real world effectiveness of early ensitrelvir treatment in patients with SARS-CoV-2, a retrospective case seriesEnsitrelvir, a 3C-like protease inhibitor, received emergency approval in Japan in November 2022 for treating non-hospitalized patients with mild-to-moderate COVID-19. However, confirmation of its real-world clinical effectiveness is limited.
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tesG expression as a potential clinical biomarker for chronic Pseudomonas aeruginosa pulmonary biofilm infectionsPseudomonas aeruginosa infections in the lungs affect millions of children and adults worldwide. To our knowledge, no clinically validated prognostic biomarkers for chronic pulmonary P. aeruginosa infections exist. Therefore, this study aims to identify potential prognostic markers for chronic P. aeruginosa biofilm lung infections.
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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.
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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.
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The airway epithelium is a direct source of matrix degrading enzymes in bronchiolitis obliterans syndromeLong-term survival after lung transplantation is hindered by the development of bronchiolitis obliterans syndrome (BOS).
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Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosisEarly detection of the cyanobacterium Pseudomonas aeruginosa in the lungs of young children with cystic fibrosis (CF) is considered the key to delaying...
