[Reye's syndrome: the death of a syndrome? (Or death by a syndrome?)].
Reye syndrome is characterized by acute encephalopathy and fatty degeneration of the liver almost exclusively in children. The onset is heralded by profuse vomiting and varying neurologic impairment from irritability to coma, decerebration and death. The encephalopathy must be associated with a greater increase in the levels of ammonia, or alanine amino-transferase and aspartate amino-transferase in serum; and with a fatty metamorphosis of the liver diagnosed by biopsy or at autopsy. The only characteristic universally accepted as diagnostic are the specific mithocondrial changes in the liver-biopsy specimen. Larger studies confirmed the association of aspirin with RS. The CDC of Atlanta cautioned physician and parents and a dramatic decline in case began at that time. Classic Reye syndrome is now so rare in the USA that when an apparent case is encountered in a child who has not taken aspirin, other diagnoses should be considered. After a brief survey of RS relative lack of specificity of case definition and of the polyhedric etiopathogenetic moments, the A. on the personal experience, point: a) the biological unicity of the man and the necessary coexistence of "constitutional" factors (metabolic and/or endocrine, and/or immunitary factors, the later almost never investigated), toxic, and infectious factors for the syndrome's deflagration; b) some aspects of the continued existence of therapeutic and diagnostic problems: the aspirin and/or salicilate use and the pharmacogenetic; the continued existence of other, generally similar conditions, such the drug and other known and unknown toxic mithocondrial factors that provoke this unusual response to common infections; and the inborn errors of metabolism; c) some practical aspects of diagnostic and therapeutic approach.
Calvani
M
M
Divisione Pediatrica, Ospedale San Camillo de Lellis, Roma.
D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects; D001241:Aspirin / Q000009:adverse effects; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D012202:Reye Syndrome / Q000175:diagnosis / Q000209:etiology
D000894:Anti-Inflammatory Agents, Non-Steroidal; D001241:Aspirin
Sindrome di Reye: morte di una sindrome? (O morire per una sindrome?)