Identification of 2-hydroxy-2-methyllevulinic acid in urine and serum of diabetic patients with ketoacidosis.

A new organic acid, 2-hydroxy-2-methyllevulinic acid, was identified in the urine of four diabetic patients with ketoacidosis using gas chromatography-mass spectrometry. The compound was also detected in two serum samples of the four patients. The compound became undetectable in the urine of the patients after insulin therapy and was not detected in urine and serum of healthy subjects or diabetic patients without ketosis. 2-Hydroxy-2-methyllevulinic acid was also detectable in the urine of a child with elevated blood lactate and pyruvate, and ketosis. This finding suggests that the occurrence of 2-hydroxy-2-methyllevulinic acid is not specific to "diabetic" ketosis but is correlated to ketosis itself.