Metabolic disorders mimicking Reye's syndrome.

摘要: Background Several metabolic disorders such as encephalopathy and hepatic dysfunction have been described Reye's-like syndrome because they present with similar clinical manifestations that mimic Reye's syndrome. We performed a retrospective study to explore the underlying etiologies of in patients treated at National Taiwan University Hospital. Methods From January 1991 June 1998, 19 children fitting description were identified for study. Urine organic acid analysis, plasma amino liver pathology, skin fibroblast enzyme assays studied during acute stage illness. Results The patients' syndromes included urea cycle (n = 7), glycogen storage disease type Ia (4), primary carnitine deficiency (2), hereditary fructose intolerance (1), methylmalonic acidemia 3-hydroxy-3-methylglutaric (1). Fatty oxidation defects suspected remaining two cases. Conclusions A significant number who an inherited disorder. In syndrome, accurate diagnosis is essential ensure normal growth development prevent recurrence condition.