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Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis

Aspergillus species and P. aeruginosa are commonly present in the lower airways from infancy

Exposure to bile leads to the emergence of adaptive signaling variants in the opportunistic pathogen pseudomonas aeruginosa

Adaptive variants of P. aeruginosa that arise following long term bile exposure enables the emergence of ecologically competitive sub-populations

Single-breath washout and association with structural lung disease in children with cystic fibrosis

Acinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT

CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCD

Structural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients

Elastase Exocytosis by Airway Neutrophils Associates with Early Lung Damage in Cystic Fibrosis Children

Protease elastase exocytosis by airway neutrophils occurs in all cystic fibrosis children, and its cellular measure correlates with early lung damage

Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis

Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target

Use of a primary epithelial cell screening tool to investigate phage therapy in cystic fibrosis

This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates

CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosis

Whether airway mucosal acidification drives early progressive lung disease is controversial

The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis

De novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF

Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children

We characterized the lower airways microbiome using BAL samples obtained from clinically stable CF young children who underwent bronchoscopy and chest CT.