title: A child with primary sclerosing cholangitis successfully treated by liver transplantation.
abstract: Primary sclerosing cholangitis (PSC) happens mainly in middle-aged men and is seldom diagnosed in children. Childhood PSC undergoing liver transplantation is rarely reported. Here we present a 12-year-old girl who was admitted with a 6-day history of fever, abdominal pain, fatigue, jaundice, and splenomegaly. Liver histological examination revealed the destroyed bile ducts and bridging fibrosis. Endoscopic retrograde cholangiopancreatography (ERCP) showed beaded appearance of right intrahepatic ducts and absent left intrahepatic ducts. PSC was diagnosed. Because of decompensated liver function, she received a living-related orthotopic liver transplantation (OLT). The post-transplantation course was uneventful during the 12-month follow-up. She experienced neither additional episode of cholangitis nor recurrence of liver cirrhosis after OLT.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
authors.forename: Chien-Han
authors.initials: CH
authors.lastname: Chen
authors.forename: Ming-Chih
authors.initials: MC
authors.lastname: Ho
authors.forename: Po-Huang
authors.initials: PH
authors.lastname: Lee
authors.forename: Mei-Hwei
authors.initials: MH
authors.lastname: Chang
authors.forename: Yung-Ming
authors.initials: YM
authors.lastname: Jeng
authors.forename: Yen-Hsuan
authors.initials: YH
authors.lastname: Ni
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D001652:Bile Ducts / Q000473:pathology / Q000601:surgery; D002648:Child; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D005334:Fever / Q000209:etiology; D006801:Humans; D016031:Liver Transplantation; D016896:Treatment Outcome