PEDIATRICS Vol. 56 No. 6 December 1975, pp. 1081-1084
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Pathogenesis of Reye's Syndrome: A Working Hypothesis

M. Michael Thaler MD1

1 Department of Pediatrics, University of California, San Francisco, California 94143

The brief history of Reye's syndrome has been one of progressive familiarity with a deepening mystery. It is possible that the syndrome reflects a variety of etiologically unrelated conditions with similar clinicopathological manifestations. In this view, subclinical defects in ammonia metabolism may constitute a distinct, identifiable group among disorders responsible for encephalopathy with fatty liver. Awareness of the possible role of mutant urea cycle enzymes and deficient substrates in the pathogenesis of this important illness should stimulate investigations of enzyme kinetics and intracellular transport mechanisms which may reveal a spectrum of abnonmalities in ammonia metabolism-and a specific therapy for each.




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