A child with primary sclerosing cholangitis successfully treated by liver transplantation.
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.
Chen
Chien-Han
CH
Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
Ho
Ming-Chih
MC
Lee
Po-Huang
PH
Chang
Mei-Hwei
MH
Jeng
Yung-Ming
YM
Ni
Yen-Hsuan
YH
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