Hepatic lipids in Reye-Johnson syndrome and in acute encephalopathy without fatty liver.
The relationship between Reye-Johnson syndrome and acute encephalopathy without fatty liver was investigated by comparing the lipid composition of liver samples obtained from five patients with Reye-Johnson syndrome, two patients with acute encephalopathy, and five controls. The mean total hepatic triglyceride concentration was increased nearly sevenfold in Reye-Johnson syndrome and slightly decreased in acute encephalopathy when compared with the mean control value. The mean total hepatic free fatty acid concentration was increased nearly threefold in acute encephalopathy when compared with the mean value in Reye-Johnson syndrome. Total phospholipid content was decreased in the liver in Reye-Johnson syndrome, and this difference was caused mainly by a diminution of the hepatic lecithin fraction. The ratio of palmitic acid to oleic acid and hepatic free fatty acids was 2.5 in Reye-Johnson syndrome, 0.7 in acute encephalopathy, and 0.8 in controls. These results suggest that, despite clinical similarities and laboratory evidence of hepatic dysfunction in both Reye-Johnson syndrome and acute encephalopathy, different pathogenic mechanisms may be responsible for the liver abnormalities found in the two syndromes.
D000208:Acute Disease; D000293:Adolescent; D001927:Brain Diseases / Q000378:metabolism*; D002784:Cholesterol / Q000378:metabolism; D005230:Fatty Acids, Nonesterified / Q000378:metabolism; D006801:Humans; D007223:Infant; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism*; D008297:Male; D010743:Phospholipids / Q000378:metabolism; D012202:Reye Syndrome / Q000378:metabolism*; D014280:Triglycerides / Q000378:metabolism
D005230:Fatty Acids, Nonesterified; D010743:Phospholipids; D014280:Triglycerides; D002784:Cholesterol