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Early lung surveillance of cystic fibrosis: what have we learnt?Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier
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Distribution of Early Structural Lung Changes due to Cystic Fibrosis Detected with Chest Computed TomographyTo examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed...
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Airway epithelial repair in health and disease: Orchestrator or simply a player?This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells
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Virome assembly reveals draft genomes of native Pseudomonas phages isolated from a paediatric bronchoalveolar lavage sampleWe 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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Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomographyOur data suggest that FRC PC-CTs are less sensitive than TLC PC-CTs and that FB-CTs have similar sensitivity to PC-CTs in detecting lung disease
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Oxidative stress and abnormal bioactive lipids in early cystic fibrosis lung diseaseSeveral lipid biomarkers of early cystic fibrosis lung disease were identified, which point toward potential disease monitoring and therapeutic approaches
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Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiencesSensitive measures of early lung disease are being integrated into therapeutic trials and clinical practice in cystic fibrosis (CF). The impact of early disease surveillance (EDS) using these novel and often intensive techniques on young children and their families is not well researched.
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Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosisMucus hyperconcentration in cystic fibrosis (CF) lung disease is marked by increases in both mucin and DNA concentration. Additionally, it has been shown that half of the mucins present in bronchial alveolar lavage fluid from preschool-aged CF patients are present in as non-swellable mucus flakes.
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The longitudinal microbial and metabolic landscape of infant cystic fibrosis: the gut-lung axisIn cystic fibrosis, gastrointestinal dysfunction and lower airway infection occur early and are independently associated with poorer outcomes in childhood. This study aimed to define the relationship between the microbiota at each niche during the first 2 years of life, its association with growth and airway inflammation, and explanatory features in the metabolome.
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Azithromycin reduces bronchial wall thickening in infants with cystic fibrosisCOMBAT-CF showed that children aged 0-3 years treated with azithromycin did clinically better than placebo but there was no effect on CT-scores. We reanalysed CTs using an automatic bronchus-artery (BA) analysis.