The occurrence of 2-hydroxyisovaleric acid in patients with lactic acidosis and ketoacidosis.

Examination of urine samples by capillary column gas chromatography and mass spectrometry showed the presence of 2-hydroxyisovaleric acid together with trace amounts of the three branched-chain 2-keto acids in five patients with lactic and ketoacidosis. None of these compounds was detected in urine from healthy controls.