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

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)209-213
Number of pages5
JournalJournal of the Formosan Medical Association = Taiwan yi zhi
Volume98
Issue number3
Publication statusPublished - Mar 1999
Externally publishedYes

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Keywords

  • Bile duct
  • Children
  • Endoscopic retrograde cholangiopancreatography
  • Eosinophilia
  • Primary sclerosing cholangitis
  • Ursodeoxycholic acid

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Lin, W. T., Lin, S. J., Ni, Y. H., Chen, H. L., Wang, H. P., Chu, J. S., & Chang, M. H. (1999). Primary sclerosing cholangitis in a child. Journal of the Formosan Medical Association = Taiwan yi zhi, 98(3), 209-213.