Abstract #3444

Liver and muscle energy metabolism in patients with organic acidemias using 31P and 1H MR spectroscopy

Alessandra Bierwagen^1,2, Daria Caspari³, Maik Rothe^1,2, Klaus Straßburger^2,4, Jong-Hee Hwang^1,2, Michael Roden^1,2,5, and Regina Ensenauer³

¹Institute for Clinical Diabetology, German Diabetes Center, Leibniz Institute for Diabetes Research, Heinrich Heine University, Duesseldorf, Germany, ²German Center for Diabetes Research (DZD e.V.), Partner Düsseldorf, Duesseldorf, Germany, ³Experimental Pediatrics and Metabolism, University Children’s Hospital, Heinrich Heine University, Duesseldorf, Germany, ⁴Institute for Biometrics and Epidemiology, German Diabetes Center, Leibniz Institute for Diabetes Research, Heinrich Heine University, Duesseldorf, Germany, ⁵5Department of Endocrinology and Diabetology, Medical Faculty, Heinrich Heine University, Duesseldorf, Germany

This study assessed energy and fat metabolism in a well phenotyped cohort of patients with organic acidemias using non-invasive MR spectroscopy. ³¹P and ¹H spectra of the liver as well as ¹H spectra of the muscle were acquired in 27 patients and 30 healthy controls (age-, sex-, BMI-matched). We found higher liver fat content in patients with propionic acidemia. The level of creatine in the soleus muscle was higher in patients with methylmalonic acidemia and propionic acidemia. In conclusion, the results provide evidence for previously unknown abnormalities of liver fat and muscle energy metabolism in patients with organic acidemias.

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