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Research
CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCDStructural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients
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Elastase Exocytosis by Airway Neutrophils Associates with Early Lung Damage in Cystic Fibrosis ChildrenProtease elastase exocytosis by airway neutrophils occurs in all cystic fibrosis children, and its cellular measure correlates with early lung damage
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Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosisOur data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target
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Use of a primary epithelial cell screening tool to investigate phage therapy in cystic fibrosisThis study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates
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CrossTalk opposing view: Mucosal acidification does not drive early progressive lung disease in cystic fibrosisWhether airway mucosal acidification drives early progressive lung disease is controversial
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The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosisDe novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF
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Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool childrenWe characterized the lower airways microbiome using BAL samples obtained from clinically stable CF young children who underwent bronchoscopy and chest CT.
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Persistent activation of interlinked type 2 airway epithelial gene networks in sputum-derived cells from aeroallergen-sensitized symptomatic asthmaticsOur findings provide new insight into the molecular mechanisms operative at baseline in the airway mucosa in atopic asthmatic with natural aeroallergen exposure
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Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosisBackground: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.
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Air trapping in early cystic fibrosis lung disease-Does CT tell the full story?Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"