70 - Novel Magnetic Resonance Fingerprinting of Congenital Hepatic Fibrosis (CHF) in ARPKD Patients

Poster Number: 70
Publication Number: 70.341

Chris A. Flask, Case Western Reserve University, Cleveland, OH, United States; Elise Keshock, Case Western Reserve University, Cleveland, OH, United States; Susan Farr, Case Western Reserve University School of Medicine, Cleveland, OH, United States; Christina MacAskill, Case Western Reserve University School of Medicine, Department of Radiology, Cleveland, OH, United States; Jenna M. Hach, Cleveland Clinic Foundation, Cleveland, OH, United States; Katherine M. Dell, Cleveland Clinic Children's and Case Western Reserve University, Cleveland, OH, United States

Background: Autosomal Recessive Polycystic Kidney Disease (ARPKD) affects 1/20,000 children and is characterized by both cystic kidney disease and congenital hepatic fibrosis (CHF). CHF can be associated with significant mortality and morbidity and is becoming increasingly prevalent as more ARPKD children survive to adulthood. Despite promising studies in animal models, there are still no clinically-available therapies for CHF, primarily due to the lack of non-invasive biomarkers for progressive CHF that could be used in clinical trials. Our previous published work in the PCK rat model of ARPKD/CHF identified mean T1-MRI as a non-invasive biomarker for progressive CHF. More recently, we have optimized and applied a novel MRI technology, MR Fingerprinting (MRF), to the study of human ARPKD. MRF rapidly and simultaneously generates quantitative maps of multiple MRI parameters (e.g. T1, T2) and is inherently resistant to motion artifacts.

Objective: To evaluate rapid T1-MRF methodology to assess CHF in pediatric and young adult ARPKD patients with a spectrum of liver disease.

Design/Methods: ARPKD patients ages 6-25 years (n=8) with no history of kidney or liver transplantation and healthy volunteers (n=10) were recruited. Subjects were scanned on a Siemens Vida 3T MRI scanner without sedation or intravenous contrast using a rapid MRF acquisition (Radiology 2021;300:380-87).  Repeat scans were performed 1 day later to assess repeatability. The MRF data was reconstructed offline to generate quantitative T1 maps for each imaging slice. A region of interest (ROI) method was used to determine the mean liver T1 value for each subject. Mean liver T1 values for ARPKD and healthy subjects were compared using two-tailed Student's t-test (significance, p< 0.05).  Mean liver T1 was also compared in ARPKD subjects with (n=3) and without (n=5) overt signs of portal hypertension (indicative of moderate-severe CHF).

Results: ARPKD patients had significantly increased mean liver T1 values compared to healthy volunteers (969±48 vs. 798±62 ms, p< 0.001).  Within the ARPKD patients, mean liver T1 was also significantly higher in ARPKD subjects with vs. without clinical evidence of portal hypertension (1018±7 vs. 943±39 ms, p=0.017). Liver T1-MRF in ARPKD subjects had excellent repeatability (interscan variation avg= 2.2%, range 0.3-4.3%).Conclusion(s): These preliminary data show that mean liver T1, assessed by novel MRF, can distinguish CHF across a spectrum of disease severity, suggesting it could be a potential non-invasive biomarker for use in clinical trials.