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題 名 | Primary Sclerosing Cholangitis in a Child=兒童的原發性硬化膽管炎 |
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作 者 | 林文騰; 林世強; 倪衍玄; 陳慧玲; 王秀伯; 朱娟秀; 張美惠; | 書刊名 | 臺灣醫學會雜誌 |
卷 期 | 98:3 1999.03[民88.03] |
頁 次 | 頁209-213 |
分類號 | 417.5456 |
關鍵詞 | 兒童; 原發性硬化膽管炎; Primary sclerosing cholangitis; Children; Eosinophilia; Ursodeoxycholic acid; Endoscopic retrograde cholangiopancreatography; Bile duct; |
語 文 | 英文(English) |
英文摘要 | Primary sclerosing cholangitis (PSC) is a rare disease in Taiwan and has not been described in Taiwanese children previously. We report a 4-year-old girl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, and markedly elevated serum level of alkaline phosphatase (3,318 IU/L) and γ-glutamyl transpeptidase (475 IU/L). Subsequent investigations including endoscopic retrograde cholangiopancreatography and liver histology confirmed the diagnosis fo PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in clinical remission and halted disease progression. A high index of suspicion is necessary for physicians to diagnose this disorder in children with chronic liver disease. Our experience in this case indicates that therapy with prednisolone and ursodeoxycholic acid may be helpful for the treatment of PSC in children, and suggests the need for more trials of combined therapy. |
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