title: Primary sclerosing cholangitis in a child.
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 gamma-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.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: W T
authors.initials: WT
authors.lastname: Lin
authors.forename: S J
authors.initials: SJ
authors.lastname: Lin
authors.forename: Y H
authors.initials: YH
authors.lastname: Ni
authors.forename: H L
authors.initials: HL
authors.lastname: Chen
authors.forename: H P
authors.initials: HP
authors.lastname: Wang
authors.forename: J S
authors.initials: JS
authors.lastname: Chu
authors.forename: M H
authors.initials: MH
authors.lastname: Chang
mesh_terms: D017668:Age of Onset; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D004802:Eosinophilia / Q000209:etiology; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D013624:Taiwan; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid