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Newly 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).
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.
Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV1) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV1 decline in adolescence and adulthood have not slowed. This suggests that interventions aimed at preventing lung disease should be targeted to mild disease and commence in early life.
The aim of this study was to assess the relationship between dust levels and health in Indigenous children in Western Australia
Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target
Whether airway mucosal acidification drives early progressive lung disease is controversial
Aspergillus Infections and Progression of Structural Lung Disease in Children with Cystic Fibrosis Rationale: Recent data show that Aspergillus
Of Pigs, Mice, and Men: Understanding Early Triggers of Cystic Fibrosis Lung Disease Of Pigs, Mice, and Men: Understanding Early Triggers of Cystic
CrossTalk opposing view: mucosal acidification does not drive early progressive lung disease in cystic fibrosis CrossTalk opposing view: mucosal
The cumulative effect of inflammation and infection on structural lung disease in early cystic fibrosis Lung inflammation and infection are common