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Abstract #2891

Kidney MR Fingerprinting and Arterial Spin Labeling (ASL) Assessments of Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Christina J. MacAskill1, Madison E. Kretzler2, Ashlee Parsons3, Victoria Gange3, Jenna Hach3, Stephanie Larson3, Yuran Zhu1, Jacob Perino2, Susan Farr2, Michael Markley2, Nicole Pritts2, Mireia Perera-Gonzalez4, Heather Clark5, Bernd Kuehn6, Ke Cheng Liu6, Xin Yu1,2,7, Dan Ma1,2, Yong Chen1,2, Katherine M. Dell3,8, and Chris A. Flask1,2,8
1Biomedical Engineering, Case Western Reserve University, Cleveland, OH, United States, 2Radiology, University Hospitals- Cleveland Medical Center, Cleveland, OH, United States, 3Center for Pediatric Nephrology, Cleveland Clinic Children’s, Cleveland, OH, United States, 4Bioengineering, Northeastern University, Boston, MA, United States, 5School of Biological and Health Systems Engineering, Arizona State University, Tempe, AZ, United States, 6Siemens Healthineers, Erlangen, Germany, 7Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, United States, 8Pediatrics, Case Western Reserve University, Cleveland, OH, United States

Synopsis

Keywords: Kidney, Genetic Diseases, ARPKD, Pediatric Genetic Disease

Motivation: There are no clinically available therapies for ARPKD.

Goal(s): We evaluated the ability of T1 and T2 from MR Fingerprinting (MRF) to assess kidney cystic burden and Arterial Spin Labeling (ASL) to assess kidney perfusion to detect and stage ARPKD kidney disease.

Approach: 12 ARPKD patients and 10 healthy adult volunteers were scanned on a Siemens 3T with kidney MRF and ASL MRI methods to generate kidney cortical perfusion maps.

Results: Significant increases in kidney T1 and T2, and reduced perfusion were observed between ARPKD patients and healthy volunteers (p<0.001) and between ARPKD patients with early and mild-to-moderate CKD based on eGFR (p<0.03).

Impact: The multi-modal MRI biomarkers in combination evaluated were able to distinctly stratify all three cohorts, including the two ARPKD patient cohorts, demonstrating the utility of a multi-modal MRI approach during future clinical trials aimed at limiting ARPKD kidney disease progression.

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Abstract

Keywords