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.