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題名 | Metabolic Disorders Mimicking Reye's Syndrome=擬似雷氏症候群的代謝異常 |
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作者 | 張碧峰; 黃秀芬; 胡務亮; 侯家瑋; 倪衍玄; 張美惠; Chang, Pi-feng; Huang, Shu-feng; Hwu, Wuh-liang; Hou, Jia-woei; Ni, Yen-hsuan; Chang, Mei-hwei; |
期刊 | 臺灣醫學會雜誌 |
出版日期 | 20000400 |
卷期 | 99:4 2000.04[民89.04] |
頁次 | 頁295-299 |
分類號 | 417.509 |
語文 | eng |
關鍵詞 | 擬似雷氏症候群; 代謝異常; Reye's-like syndrome; Metabolic disorder; |
英文摘要 | Background: Several metabolic disorders such as encephalopathy and hepatic dysfunction have been described as 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 metabolic etiologies of Reye's-like syndrome in patients treated at National Taiwan University Hospital. Methods: From January 1991 to June 1998, 19 children with a syndrome fitting the Reye's-like syndrome description were identified for study. Urine organic acid analysis, plasma amino acid analysis, liver pathology, and skin fibroblast enzyme assays were studied during the acute stage of illness. Results: The etiologies of patients' syndromes included urea cycle disorders (n = 7), glycogen storage disease type Ia ( 4), primary carnitine deficiency (2), hereditary fructose intolerance (1), methylmalonic acidemia (2), and 3-hydroxy-3-methylglutaric acidemia ( 1). Fatty acid oxidation defects were suspected in the remaining two cases. Conclusions: A significant number of patients who present with Reye's-like syndrome have an underlying inherited metabolic disorder. In patients with Reye's-like syndrome, an accurate diagnosis is essential to ensure normal growth and development and to prevent recurrence of the condition. |
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