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Abstract #4339

CEST pH Imaging as a Diagnostic for Chronic Kidney Disease in Methylmalonic Acidemia (MMA)

KowsalyaDevi Pavuluri1, Irini Manoli2, Alexandra Pass2, Yuguo Li1,3, Hilary J. Vernon4, Charles P. Venditti2, and Michael T McMahon1,3

1The Russell H. Morgan Department of Radiology and Radiological Science, Division of MR Research, The Johns Hopkins University School of Medicine, Baltimore, MD, United States, 2Medical Genomics and Metabolic Genetics Branch, National Institutes of Health (NIH), Bethesda, MD, United States, 3F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, United States, 4McKusick-Nathans Institute of Genetic Medicine, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, United States

Chronic Kidney Disease (CKD) is a serious complication of methylmalonic acidemia (MMA), manifests with proximal tubular dysfunction, tubulointerstitial nephritis, and eventually decreased glomerular filtration. Routine diagnostic tests, like serum creatinine, leave much to be desired. In this study, we designed an alternative MRI strategy to measure renal perfusion and create pH maps after administering iopamidol, a pH sensitive contrast agent. We detected robust differences in perfusion fraction and pH maps between severe, mild and no renal disease in MMA mouse model. These results demonstrate that MRI may facilitate early detection of kidney disease.

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