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Article
December 2, 1988

Inborn Errors of Metabolism in Children Referred With Reye's SyndromeA Changing Pattern

Author Affiliations

From the Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore.; Dr Rowe is now with the Department of Pediatrics, Children's Hospital of Eastern Canada, Ottawa, Ontario.

From the Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore.; Dr Rowe is now with the Department of Pediatrics, Children's Hospital of Eastern Canada, Ottawa, Ontario.

JAMA. 1988;260(21):3167-3170. doi:10.1001/jama.1988.03410210079041
Abstract

Genetic disorders were identified infrequently among children presenting with Reye's syndrome in the past. During a two-year period, we evaluated four consecutive patients referred for intensive care of Reye's syndrome. A standard investigation for inborn errors of metabolism revealed that two patients had enzymatic defects of fatty acid oxidation, and the other two had partial deficiencies of ornithine transcarbamoylase. None had experienced a previous episode of Reye's syndrome, and three of the four had been entirely healthy in the past. Our experience suggests that as the incidence of Reye's syndrome has decreased, patients with its clinical features are now more likely to have manageable inborn errors of metabolism (eg, disorders of ureagenesis, ketogenesis, and branched-chain amino acids).

(JAMA 1988;260:3167-3170)

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