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The cumulative effect of inflammation and infection on structural lung disease in early cystic fibrosis

Pulmonary inflammation in surveillance bronchoalveolar lavage has a cumulative effect on structural lung disease extent, more so than infection

Early respiratory viral infections in infants with cystic fibrosis

Early viral infections were associated with greater neutrophilic inflammation and bacterial pathogens

Oxidative stress and abnormal bioactive lipids in early cystic fibrosis lung disease

Several lipid biomarkers of early cystic fibrosis lung disease were identified, which point toward potential disease monitoring and therapeutic approaches

Rhinovirus Infection Is Associated With Airway Epithelial Cell Necrosis and Inflammation via Interleukin-1 in Young Children With Cystic Fibrosis

This study found rhinovirus infection drives necrotic cell death in cystic fibrosis airway epithelial cells

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography

Our 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

The detection of bile acids in the lungs of paediatric cystic fibrosis patients is associated with altered inflammatory patterns

Our work has identified a clinically relevant host-derived factor that may participate in shaping early events in the aetiology of CF respiratory disease

Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosis

This study developed and tested a highly usable, and moderately acceptable, smartphone app to improve the psychosocial health of young people living with CF

Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis

Mucus 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.

Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model

Severity and disease progression in people with Cystic Fibrosis is typically dependent on their genotype. One potential therapeutic strategy for people with specific mutations is exon skipping with antisense oligonucleotides. CFTR exon 9 is an in-frame exon and hence the exclusion of this exon would excise only 31 amino acids but not alter the reading frame of the remaining mRNA.

SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation

Cystic fibrosis (CF), due to pathogenic variants in CFTR gene, is associated with chronic infection/inflammation responsible for airway epithelium alteration and lung function decline. Modifier genes induce phenotype variability between people with CF (pwCF) carrying the same CFTR variants. Among these, the gene encoding for the amino acid transporter SLC6A14 has been associated with lung disease severity and age of primary airway infection by the bacteria Pseudomonas aeruginosa.