Chronic Kidney Disease (CKD) is a cardinal feature of methylmalonic acidemia (MMA), a prototypic organic acidemia. Impaired growth, low activity, and protein restriction affect muscle mass and lower serum creatinine concentrations, which can delay the diagnosis and management of renal disease in this patient population. We have designed a general alternative strategy for monitoring renal function based on administration of a pH sensitive MRI contrast agents to acquire functional information. We have tested our methods in a mouse model of MMA, and detected robust differences in the perfusion fraction and pH maps we produce between groups with severe, mild, and no renal disease. Our results demonstrate that MRI contrast agents can be used for early detection and monitoring of CKD, particularly in disorders that alter renal pH and perfusion such as MMA.
Speaker: Michael McMahon, John Hopkins University
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