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Kidney Week

Abstract: PO1565

Rapid, Quantitative Measures of ARPKD Kidney Disease with Novel Magnetic Resonance Fingerprinting

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Macaskill, Christina J., Case Western Reserve University, Cleveland, Ohio, United States
  • Ma, Dan, Case Western Reserve University, Cleveland, Ohio, United States
  • Chen, Yong, Case Western Reserve University, Cleveland, Ohio, United States
  • Flask, Chris, Case Western Reserve University, Cleveland, Ohio, United States
  • Dell, Katherine MacRae, Case Western Reserve University, Cleveland, Ohio, United States
Background

Autosomal recessive polycystic kidney disease (ARPKD) is a rare but potentially lethal genetic disorder characterized by diffuse collecting tubule microcysts.There are currently no disease-specific treatments, although several therapies have shown promise in ARPKD animal models. Clinical trials for ARPKD patients have not been possible due to the lack of sensitive measures of ARPKD kidney disease progression. We previously identified renal T1 and T2 relaxometry as potential imaging biomarkers of ARPKD. The goal of this study was to evaluate a novel, rapid magnetic resonance imaging (MRI) technique, magnetic resonance fingerprinting (MRF), in kidneys of healthy volunteers and pediatric ARPKD patients.

Methods

MRF is a quantitative MRI technique that simultaneously generates maps of multiple MRI parameters (e.g., T1,T2), while also showing resistance to motion artifact, allowing for more rapid and comprehensive assessment of tissue composition and pathology. We developed a kidney MRF acquisition protocol to generate T1 and T2 maps in a single breath-hold (~15 seconds/slice). This MRF method was first validated in vitro using standardized T1 and T2 phantoms. In vivo kidney T1 and T2 maps were then obtained from 10 healthy volunteers and 3 ARPKD patients.

Results

MRF-based T1 and T2 maps demonstrated good agreement with reference values in standardized phantoms. MRF experiments in healthy volunteers further showed repeatable assessments of the renal cortex (T1:1318±91 ms; T2:71±6 ms) and medulla (T1:1592±63 ms; T2:73±5 ms), consistent with literature values. Repeated kidney MRF scans for 3 ARPKD patients (age 7-13 yrs, estimated glomerular filtration rates 52-97 ml/min/1.73m2) on 2 successive days demonstrated good reproducibility (< 3% differences for T1 and T2). Significant differences were seen between the volunteer and ARPKD patient populations for both mean kidney T1 (p<0.007) and T2 (p<0.04).

Conclusion

This novel kidney MRF acquisition provides fast, accurate, and repeatable kidney T1 and T2 maps in pediatric ARPKD patients. The short acquisition times, coupled with resistance to motion artifact, suggest that MRF could allow rapid, quantifiable imaging assessments of ARPKD kidney disease even in younger children, which could be used to identify high risk patients and/or to assess therapeutic efficacy in clinical trials.

Funding

  • NIDDK Support