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Research

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

These findings indicate that early CF lung disease is characterized by an increased mucus burden and inflammatory markers without infection or structural lung disease

Research

Posaconazole-induced hypertension in children with cystic fibrosis

Posaconazole is a triazole antifungal with a broad spectrum of activity against moulds including Aspergillus spp. Emerging data suggest posaconazole may be effective in the treatment of allergic bronchopulmonary aspergillosis complicating cystic fibrosis.

Research

Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosis

Treatment for pulmonary exacerbations of cystic fibrosis (CF) can produce a range of positive and negative outcomes. Understanding which of these outcomes are achievable and desirable to people affected by disease is critical to agreeing to goals of therapy and determining endpoints for trials.

Research

Airway surface liquid pH is not acidic in children with cystic fibrosis

Modulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.

Research

Quantitative assessment of airway dimensions in young children with cystic fibrosis lung disease using chest computed tomography

To evaluate lung disease progression using AA dimensions on chest CT over 2-years in young CF patients longitudinally and compare to disease controls.

Research

Determinants of culture success in an airway epithelium sampling program of young children with cystic fibrosis

Determinants of culture success through retrospective analysis of a program of routinely brushing children with Cystic Fibrosis airway disease

Research

Detection of biofilm in bronchoalveolar lavage from children with non-cystic fibrosis bronchiectasis

The presence of Pseudomonas aeruginosa biofilms in lower airway specimens from cystic fibrosis (CF) patients is well established.

Research

Assessment of early bronchiectasis in young children with cystic fibrosis is dependent on lung volume

The aim of this study was to determine whether assessment of early CT scan-detected bronchiectasis in young children with cystic fibrosis (CF) depends on...

Research

Elucidating the interaction of CF airway epithelial cells and rhinovirus: Using the host-pathogen relationship to identify future therapeutic strategies

A better understanding of the innate immune responses by CF airway epithelial cells is needed to identify why viral infections are more severe in CF