Clinical conditions associated with urinary excretion of 2-hydroxybutyric acid.
Urine and blood samples from patients with different clinical disorders were examined for 2-hydroxybutyric acid (2-HB) by gas chromatography and mass spectrometry. All patients with combined lactic acidosis and ketoacidosis excreted 2-HB in the urine, in amounts up to 2.3 mmol/mmol creatinine. In blood from these patients, 2-HB was present only in trace amounts. Hard physical exercise resulted in lactic acidosis and accumulation of 2-HB. The lactate to pyruvate ratio in blood and urine was markedly increased in the patients excreting 2-HB but was normal in two patients with lactic acidosis who did not excrete 2-HB. It is concluded that an increased NADH2/NAD ratio is the most important factor for the production of 2-HB.
D000138:Acidosis / Q000097:blood / Q000652:urine*; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D005260:Female; D006801:Humans; D006885:Hydroxybutyrates / Q000097:blood / Q000652:urine*; D007223:Infant; D007231:Infant, Newborn; D007662:Ketosis / Q000097:blood / Q000652:urine*; D007773:Lactates / Q000097:blood / Q000652:urine; D008297:Male; D009243:NAD / Q000378:metabolism; D011773:Pyruvates / Q000097:blood / Q000652:urine; D013997:Time Factors
D006885:Hydroxybutyrates; D007773:Lactates; D011773:Pyruvates; D009243:NAD; D003404:Creatinine