[Reye syndrome--a status review of current concepts of its pathogenesis].
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.
D000348:Aflatoxins / Q000009:adverse effects; D000641:Ammonia / Q000097:blood; D001241:Aspirin / Q000009:adverse effects; D002222:Carbamoyl-Phosphate Synthase (Ammonia) / Q000172:deficiency; D002648:Child; D005230:Fatty Acids, Nonesterified / Q000097:blood; D006801:Humans; D007005:Hypoglycins / Q000009:adverse effects; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D008099:Liver / Q000201:enzymology; D008930:Mitochondria, Liver / Q000201:enzymology; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D011379:Prognosis; D012202:Reye Syndrome / Q000139:chemically induced / Q000209:etiology*; D014777:Virus Diseases / Q000150:complications
D000348:Aflatoxins; D005230:Fatty Acids, Nonesterified; D007005:Hypoglycins; D007773:Lactates; D019344:Lactic Acid; D000641:Ammonia; D002222:Carbamoyl-Phosphate Synthase (Ammonia); D001241:Aspirin
Reye-Syndrom--Eine Bestandsaufnahme derzeitiger Vorstellungen zur Pathogenese.