A child with primary sclerosing cholangitis successfully treated by liver transplantation
Journal
Acta Paediatrica Taiwanica
Journal Volume
45
Journal Issue
4
Pages
239-241
Date Issued
2004
Author(s)
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 posttransplantation course was uneventful during the 12-month follow-up. She experienced neither additional episode of cholangitis nor recurrence of liver cirrhosis after OLT.
SDGs
Other Subjects
azathioprine; colchicine; cyclosporin A; methotrexate; pentoxifylline; steroid; tacrolimus; ursodeoxycholic acid; abdominal pain; article; ascites; bile duct; case report; cholangitis; disease course; endoscopic retrograde cholangiopancreatography; esophagus varices; fatigue; female; fever; follow up; human; human tissue; hypoalbuminemia; jaundice; liver biopsy; liver cirrhosis; liver failure; liver fibrosis; liver transplantation; living donor; primary sclerosing cholangitis; rare disease; school child; splenomegaly; Abdominal Pain; Bile Ducts; Child; Cholangiopancreatography, Endoscopic Retrograde; Cholangitis, Sclerosing; Female; Fever; Humans; Liver Transplantation; Treatment Outcome
Type
journal article