The primary purpose of this study is to see if MRI scans are better at detecting liver and pancreas disorders in children (ages 5 – 17) diagnosed with Cystic Fibrosis (CF). This study will compare a variety of MRI imaging techniques to the currently-used liver tests such as blood tests and ultrasound imaging. The secondary aims of this study are 1) to obtain initial estimates of the prevalence of sub-clinical Cystic Fibrosis Liver Disease (CFLD) detectable by MRI in order to perform poser calculations for future larger studies, 2) to correlate MRI-based metrics of liver function with serum liver enzymes, 3) to compare the repeatability of magnetic resonance elastography to ultrasound elastography in the evaluation of liver stiffness, 4) to identify morphologic metrics of exocrine pancreas function from MRI (e.g., volumetry and fat fraction) that correlate with fecal elastase as the reference standard, 5) to correlate the CFLD findings with the severity of the lung disease using the most recent FEV1, and 6) to compare repeatability of USE acquired with two different MRI systems. A total of 20 CF patients will be enrolled. All subjects will undergo two study visits, separated by 1-2 weeks.
November 2016 to September 2018
This project led by: Scott K Nagle, MD, PhD