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Research

Phage therapy to treat cystic fibrosis Burkholderia cepacia complex lung infections: perspectives and challengesge

Burkholderia cepacia complex is a cause of serious lung infections in people with cystic fibrosis, exhibiting extremely high levels of antimicrobial resistance. These infections are difficult to treat and are associated with high morbidity and mortality. 

Research

Lentiviral vector gene therapy and CFTR modulators show comparable effectiveness in cystic fibrosis rat airway models

Mutation-agnostic treatments such as airway gene therapy have the potential to treat any individual with cystic fibrosis (CF), irrespective of their CF transmembrane conductance regulator (CFTR) gene variants. The aim of this study was to employ two CF rat models, Phe508del and CFTR knockout to assess the comparative effectiveness of CFTR modulators and lentiviral vector-mediated gene therapy. 

News & Events

Born with cystic fibrosis, now research is her life’s work

When Ingrid Laing was born, the outlook for kids with cystic fibrosis was bleak. Her parents were told she might make it to 20 if she was lucky.

News & Events

Healthy kids help medical research

That's why Melissa has signed up her four year old healthy son Odin for a study at Perth's The Kids Research Institute Australia that will help kids with cystic fibrosis.

News & Events

Prestigious award for cystic fibrosis researcher

Professor Stephen Stick has been awarded the prestigious Richard C. Talamo Distinguished Clinical Achievement Award from the US Cystic Fibrosis Foundation.

Research

Progression of early structural lung disease in young children with cystic fibrosis assessed using CT

Cross-sectional studies implicate neutrophilic inflammation and pulmonary infection as risk factors for early structural lung disease in infants and young...

Research

Small macrophages are present in early childhood respiratory disease

Recently, an established "small macrophage" phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the...

Giving kids with CF a chance at a longer, healthier life

COMBAT CF is one of two long-standing international trials which have resulted in new early intervention options helping to reduce progressive lung damage in kids living with CF.

Research

Methods used to evaluate the immediate effects of airway clearance techniques in adults with cystic fibrosis: A systematic review and meta-analysis

This review reports on methods used to evaluate airway clearance techniques (ACT) in adults with CF and examined data for evidence of any effect. Sixty-eight studies described ACT in adequate detail and were included in this review.

Research

Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis

There is no data exclusively on the relationship between health-related quality-of-life (HRQOL) and lung disease severity in early school-aged children with cystic fibrosis (CF). Using data from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) we assessed the relationships between HRQOL, lung function and structure.