Primary sclerosing cholangitis in a child

Wen Terng Lin, Se John Lin, Yen Hsuan Ni, Huey Ling Chen, Hsiu Po Wang, Jan Show Chu, Mei Hwei Chang

研究成果: 雜誌貢獻文章同行評審


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.

頁(從 - 到)209-213
期刊Journal of the Formosan Medical Association = Taiwan yi zhi
出版狀態已發佈 - 3月 1999

ASJC Scopus subject areas

  • 醫藥 (全部)


深入研究「Primary sclerosing cholangitis in a child」主題。共同形成了獨特的指紋。