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Research

Multilocus Sequence Typing Reveals Extensive Genetic Diversity of the Emerging Fungal Pathogen Scedosporium aurantiacum

Scedosporium spp. are the second most prevalent filamentous fungi after Aspergillus spp. recovered from cystic fibrosis (CF) patients in various regions of the world. Although invasive infection is uncommon prior to lung transplantation, fungal colonization may be a risk factor for invasive disease with attendant high mortality post-transplantation. Abundant in the environment, Scedosporium aurantiacum has emerged as an important fungal pathogen in a range of clinical settings.

Research

The effect of CFTR modulators on structural lung disease in cystic fibrosis

Newly developed quantitative chest computed tomography (CT) outcomes designed specifically to assess structural abnormalities related to cystic fibrosis (CF) lung disease are now available. CFTR modulators potentially can reduce some structural lung abnormalities. We aimed to investigate the effect of CFTR modulators on structural lung disease progression using different quantitative CT analysis methods specific for people with CF (PwCF).

Research

Feasibility of unsedated lung MRI in young children with cystic fibrosis

Kathryn Ramsey BSc (Hons), PhD Co-Head, Foundations of Lung Disease kathryn.ramsey@thekids.org.au Co-Head, Foundations of Lung Disease Associate

Research

The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesia

Structural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.

Research

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"

Research

Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trial

The clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented.

Research

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis

In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.

News & Events

New drug therapy provides hope for kids with cystic fibrosis

The family of two girls with cystic fibrosis are hopeful after The Kids Research Institute Australia spin-off company, Respirion, receives $20 million in funding to develop a promising new therapy.

News & Events

Cure4CF Grant a boost for innovative Cystic Fibrosis research

A $350,000 Cure4 Cystic Fibrosis grant is set to propel the Wal-yan Respiratory Research Centre’s Phage WA program forward, supercharging its fight against antimicrobial resistant (AMR) lung infections in people with Cystic Fibrosis (CF) using  cutting-edge phage therapy.

Research

MetaNeb Versus Usual Care During Exacerbations of Cystic Fibrosis: An RCT

During exacerbations, when symptom and treatment burden are increased, individuals with cystic fibrosis (CF) are likely to prefer airway clearance techniques (ACTs) that require minimal effort. Therefore, in adults with CF who were hospitalised with an exacerbation, we sought to compare the effect of the MetaNeb with usual ACTs on respiratory function and expectorated sputum.