Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, affecting approximately 70,000 people worldwide. CF leads to progressive obstructive lung disease, which is the primary cause of disease morbidity and mortality in this patient population. Tools to monitor CF lung disease and therapeutic responses are currently limited in young patients with particularly sensitivity to subclinical or regional disease. With ultrashort echo time (UTE) MRI, we were able to identify CF-specific lung structural abnormalities caused by bronchiectasis, bronchial wall thickening, mucus plugging, ground glass opacity, and consolidation. UTE MRI involves no ionizing radiation and its continuing development places this technique as a potential longitudinal imaging surrogate for CT.
Through the combination of UTE MRI and hyperpolarized 129Xe MRI we are able to identify structure-function relationships within cystic fibrosis patients.
- Thomen RP, Walkup LL, Roach DJ, Cleveland ZI, Clancy JP, Woods JC. Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients. J Cyst Fibros. 2017 Mar;16(2):275-282. doi: 10.1016/j.jcf.2016.07.008.
- Roach DJ, Crémillieux Y, Fleck RJ, Brody AS, Serai SD, Szczesniak RD, Kerlakian S, Clancy JP, Woods JC. Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease. Ann Am Thorac Soc. 2016 Nov;13(11):1923-1931.