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Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection

This study aimed to determine how pulmonary inflammation & infection impacts on ventilation distribution throughout early life in people with cystic fibrosis.

Airway epithelial repair in health and disease: Orchestrator or simply a player?

This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells

Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosis

Early detection of the cyanobacterium Pseudomonas aeruginosa in the lungs of young children with cystic fibrosis (CF) is considered the key to delaying...

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes.

Cystic fibrosis research a Eureka Prize finalist

A world-leading cystic fibrosis research program, based at The Kids Research Institute Australia, is a finalist in the 2015 Australian Museum Eureka Prizes.

New study recommends changes to cystic fibrosis monitoring in young children

A new Australian study that looked at the long term impacts of early lung infections in young kids with cystic fibrosis has recommended changes to monitoring

Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

This study assessed an alternative approach, using a small scale halide assay that can be adapted for a personalized high throughput setting to analyze CFTR function of pAEC.

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

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

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.