Primary sclerosing cholangitis in a child

Primary sclerosing cholangitis (PSC) is a rare disease in Taiwan and has no t been described in Taiwanese children previously. We report a 4-year-old g irl who presented with prolonged fever, eosinophilia (11%), hepatomegaly, a nd markedly elevated serum levels of alkaline phosphatase (3,318 IU/L) and gamma-glutamyl transpeptidase (475 IU/L). Subsequent investigations includi ng endoscopic retrograde cholangiopancreatography and liver histology confi rmed the diagnosis fu PSC. Treatment with a low dose of prednisolone for 2 months and ursodeoxycholic acid during 32 months of follow-up resulted in c linical remission and halted disease progression, A high index of suspicion is necessary for physicians to diagnose this disorder in children with chr onic 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.