Urinary dicarboxylic acids in Reye syndrome.
Urine from 12 patients with Reye syndrome was examined by gas-liquid chromatography for identification of organic acids. Large amounts of lactic acid, dicarboxylic acids (adipic, suberic, and sebacic), and 3-OH butyric acid were noted. The mean (+/- SD) total dicarboxylic acid concentration was 0.98 +/- 0.24 mg/mg creatinine, compared with 0.006 +/- 0.010 mg/mg creatinine in controls, n = 140; the mean in patients with Reye syndrome was higher (1.40 +/- 0.26 mg/mg creatinine, n = 8) when the samples were obtained prior to initiation of therapy, but declined rapidly after administration of hypertonic glucose, exchange transfusion, and osmotic diuretics. The total urine excretion of dicarboxylic acids plus urine ketones at the time of presentation correlated well with the plasma lactate (r2 = 0.9676) and peak blood ammonia (r2 = 0.9216) levels. Our results document the occurrence of significant dicarboxylic aciduria in Reye syndrome and indicate that fatty acid metabolism is more impaired in this disorder than previously appreciated.
D000641:Ammonia / Q000097:blood; D002648:Child; D002849:Chromatography, Gas; D003404:Creatinine / Q000652:urine; D003998:Dicarboxylic Acids / Q000652:urine*; D006801:Humans; D007657:Ketone Bodies / Q000652:urine; D007773:Lactates / Q000097:blood; D013058:Mass Spectrometry; D012202:Reye Syndrome / Q000097:blood / Q000378:metabolism* / Q000652:urine
D003998:Dicarboxylic Acids; D007657:Ketone Bodies; D007773:Lactates; D000641:Ammonia; D003404:Creatinine