Cystic fibrosis (CF) is the most common genetic disorder in the human population. It is characterised by inflammation and recurrent and chronic infections of the lungs causing progressive loss in pulmonary function and respiratory failure, which account for 90% of the fatalities. Frequent monitoring of disease status and treatment response in infants and pre-school age children is important in postponing or preventing irreversible lung damage and increasing the life expectancy of CF patients (approximately 40 years).

Computed Tomography (CT) is currently the state-of-the art non-invasive lung imaging modality for CF, however CT involves exposing patients to long term risks from ionizing radiation. In recent years new magnetic resonance imaging (MRI) ultra-short echo time sequences have become available which provide opportunities to obtain the same clinical information as CT (eg. identify air-trapping, bronchial wall thickening and bronchiectasis) in children with CF without radiation risks, thus enabling longitudinal intra-patient scanning.

ultra-short echo time 3D VIBE MRI acquisition from a teenager with cystic fibrosis

Figure 1
Prototype ultra-short echo time 3D VIBE MRI acquisition from a teenager with cystic fibrosis.
From left to right: coronal, axial and sagittal view.

This pilot study aims to assess the feasibility of acquiring these scans at the Herston Imaging Research Facility, RBWH, Brisbane, and providing an MRI based radiographic scoring system for paediatric CF patients. The project presents an extraordinary opportunity to combine CSIRO’s globally competitive medical image analysis expertise with cutting edge imaging equipment and technology through our relationship with Siemens Healthineers. The lung specific MRI acquisition was performed using a prototype ultra-short echo time 3D VIBE sequence. This technique is still under development and not commercially available. The research is performed in close collaboration with Prof Claire Wainwright, an internationally recognised paediatric respiratory physician and leading cystic fibrosis expert based at the Lady Cilento Children’s Hospital in Brisbane.

Team and Contact

Dr Anthony Paproki: anthony.paproki@csiro.au

Associate Professor Jason Dowling: jason.dowling@csiro.au