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[Reye syndrome--a status review of current concepts of its pathogenesis]
Abstract:
In Europe Reye's syndrome is a rather rare but often fatal disease affecting children and teenagers. Whereas an association with an antecedent viral illness has been documented, our knowledge concerning etiology and pathogeneseis remains sparse. A significant association between salicylate-medication during prodromal illness and RS has well been documented without demonstration of a causal relationship. The metabolic disturbance appears to stem from an acute mitochondrial lesion following a so far not fully understood viral, toxic or multimodal attack. Similar mitochondrial lesions are encountered in hypoglycin- or aflatoxin-intoxication. Consequently the intramitochondrial steps of the urea-cycle are blocked leading to its breakdown in the scene of a tremendous nitrogen-load flooding the organism with ammonia. A number of suspected pathogens are being discussed of which ammonia seems most capable of inducing the neurologic symptoms of RS probably in conjunction with elevated free fatty acids.