https://scholars.lib.ntu.edu.tw/handle/123456789/537169
標題: | Primary sclerosing cholangitis in a child | 作者: | Lin W.-T. Lin S.-J. YEN-HSUAN NI HUEY-LING CHEN HSIU-PO WANG Chu J.-S. MEI-HWEI CHANG |
公開日期: | 1999 | 卷: | 98 | 期: | 3 | 起(迄)頁: | 209-213 | 來源出版物: | Journal of the Formosan Medical Association | 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 levels 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 of 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. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-0032984837&partnerID=40&md5=b254db89c3946dee45d3b794fb5a62ee https://scholars.lib.ntu.edu.tw/handle/123456789/537169 |
ISSN: | 0929-6646 | SDG/關鍵字: | prednisolone; ursodeoxycholic acid; article; case report; chronic liver disease; clinical feature; disease course; endoscopic retrograde cholangiopancreatography; eosinophilia; female; human; preschool child; remission; sclerosing cholangitis; Taiwan; Age of Onset; Child, Preschool; Cholagogues and Choleretics; Cholangiopancreatography, Endoscopic Retrograde; Cholangitis, Sclerosing; Eosinophilia; Female; Humans; Liver; Taiwan; Ursodeoxycholic Acid |
顯示於: | 醫學系 |
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