Studies on abnormal metabolic function in Reye's syndrome

Conclusions

(1) The presence of medium chain dicarboxylic acylcarnitines and 3-hydroxydicarboxylic acids in the urine of a patient with Reye's syndrome suggests that β-oxidation of dicarboxylic acids to succinyl CoA is occurring but also being inhibited at several stages. The cellular organelle responsible for this oxidation is not apparent.

(2) Administration of carnitine in Reye's syndrome appears to increase the production of acetylcarnitine. This would seem to indicate stimulation of β-oxidation of fatty acids to acetyl CoA by the large amount of carnitine present.