Reye syndrome: rate of oxidation of fatty acids in leukocytes and serum levels of lipid peroxides.
We examined the oxidation of different chain length fatty acids in the leukocytes and the quantity of lipid peroxides in the plasma of two Reye syndrome patients. We have found that the oxidation of [1-14C] octanoic acid in homogenates of leukocytes from one of the Reye syndrome patients was only 38 percent of the control, whereas oxidation of [1-14C] palmitic and [1-14C] lignoceric acid was slightly increased. The level of lipid peroxides in the serum of both of the Reye Syndrome patients was 4.42 and 3.04 times higher than the control level. These results suggest that impaired oxidation of medium chain fatty acids (octanoic acid) and higher levels of lipid peroxides may contribute to the pathogenesis of cellular toxicity in Reye Syndrome. Reye Syndrome (RS) was first described by Reye et. al. in 1963 and is now recognized as an important cause of morbidity and mortality in infants and children. The clinical course in RS consists of an antecedent viral illness with subsequent encephalopathy and hepatic dysfunction. Laboratory findings in RS include hypoglycemia, hyperammonemia, free fatty acidemia, elevated organic acids and amino aciduria. The ultrastructural findings in RS patients include changes in mitochondria, smooth endoplasmic reticulum morphology, and an increase in the number of peroxisomes. The elevation of serum free fatty acids in RS and their decrease in patients who improve clinically suggests a disturbance in fatty acid metabolism. To understand the role of free fatty acids in the pathogenesis of RS, we examined the levels of lipid peroxides in plasma and catabolism of fatty acids of different chain lengths in leukocytes from RS patients.
Department of Pediatrics, Medical University of South Carolina, Charleston 29425.
Y
Y
Yoshida
I
I
Singh
A K
AK
Singh
F W
FW
Tecklenberg
F R
FR
Brown
C P
CP
Darby
D002648:Child; D005227:Fatty Acids / Q000378:metabolism*; D005260:Female; D006801:Humans; D007223:Infant; D007962:Leukocytes / Q000378:metabolism*; D015227:Lipid Peroxidation; D008054:Lipid Peroxides / Q000097:blood*; D008297:Male; D012202:Reye Syndrome / Q000378:metabolism*; D013577:Syndrome
D005227:Fatty Acids; D008054:Lipid Peroxides