Masking by enzyme inhibitor of raised serum glutamate dehydrogenase activity in Reye's syndrome.
Serum glutamate dehydrogenase (GDH) activity was greatly raised (up to 830 times the upper limit of normal) in 16 patients with Reye's syndrome. The serum activity was masked by an inhibitor, and the rises were observed only after dialysis or sample dilution. Serum GDH values from 38 paediatric patients, including 10 with hyperammonaemia due to other causes, showed no such rise after dialysis. Only 1 of 13 adult patients with liver disease had high GDH activity, but this level was not increased after dialysis. Serum ornithine carbamyl transferase activity was also raised in patients with Reye's syndrome, but levels were not increased after dialysis. The ratio of dialysed/undialysed GDH activity clearly distinguished all Reye's patients from controls. The inhibition of a mitochondrial enzyme which regulates ammonia metabolism may contribute to the hyperammonaemia of Reye's syndrome. Serum GDH levels before and after dialysis would seem to be a useful diagnostic aid in Reye's disease.
D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000175:diagnosis; D000641:Ammonia / Q000097:blood; D002648:Child; D004796:Clinical Enzyme Tests; D003956:Dialysis; D005260:Female; D005969:Glutamate Dehydrogenase / Q000037:antagonists & inhibitors / Q000097:blood*; D006801:Humans; D007223:Infant; D008297:Male; D008930:Mitochondria, Liver / Q000201:enzymology; D009954:Ornithine Carbamoyltransferase / Q000097:blood; D012202:Reye Syndrome / Q000175:diagnosis*
D000641:Ammonia; D005969:Glutamate Dehydrogenase; D009954:Ornithine Carbamoyltransferase