Preliminary Investigation of UTE MRI Image Texture Pre and Post Modulator Therapy in Pediatric Cystic Fibrosis Lung Disease

Motivation: Ultrashort echo time (UTE) MRI holds promise for evaluation of lung structure in pediatric cystic fibrosis (CF). However, there is little literature evaluating UTE image texture and response to treatment for this population. Goal(s): To investigate UTE image texture at baseline and 1-month post initiation of elexacaftor/tezacaftor/ivacaftor (ETI) in pediatric CF. Approach: UTE MRI was performed in 14 pediatric CF participants at baseline and 1-month post-initiation of ETI-therapy. Lungs were segmented from the thoracic cavity, and image-wide entropy was calculated from parenchymal signals. Results: UTE image entropy was significantly reduced post-therapy, but did not correlate with changes in spirometry or multiple breath washout. Impact: Automated extraction of UTE entropy was significantly reduced post therapy, potentially indicating sensitivity to resolution of CF pathology.