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For those born with cystic fibrosis (CF), hyper-concentrated mucus with a dysfunctional structure significantly impacts CF airways, providing a perfect environment for bacterial colonization and subsequent chronic infection. Early treatment with antibiotics limits the prevalence of bacterial pathogens but permanently alters the CF airway microenvironment, resulting in antibiotic resistance and other long-term consequences.
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).
Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target
This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates
Whether airway mucosal acidification drives early progressive lung disease is controversial
De novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF
We characterized the lower airways microbiome using BAL samples obtained from clinically stable CF young children who underwent bronchoscopy and chest CT.
Our findings provide new insight into the molecular mechanisms operative at baseline in the airway mucosa in atopic asthmatic with natural aeroallergen exposure
Background: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.
Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"