title: Sclerosing cholangitis in children with inflammatory bowel disease.
abstract: BACKGROUND
  Primary sclerosing cholangitis (PSC) with inflammatory bowel disease (IBD) has been rarely reported in children.
  
  
  OBJECTIVE
  To describe the clinical presentation, sequential liver function test abnormalities, radiological bile duct anomalies and liver histology in four children with PSC and IBD.
  
  
  METHODS
  Over a period of 18 years, four of 130 patients with IBD developed abnormal liver function tests. Three of the four patients had ulcerative colitis and the other Crohn's disease. All four patients had baseline and follow-up liver function tests, percutaneous transhepatic cholangiography and a needle biopsy of the liver.
  
  
  RESULTS
  The four patients at presentation had minimal symptoms or signs of liver disease. All had elevation of serum transaminases, gamma glutamyl transferase and/or alkaline phosphatase. Three had the typical onion skin fibrosis of bile ducts. Percutaneous transhepatic cholangiography demonstrated irregularity and beading of the hepatic and common bile ducts in three patients. The other with normal cholangiography had fibrosing cholangitis on liver biopsy and was considered to have small duct disease.
  
  
  CONCLUSIONS
  We conclude that yearly biochemical assessment of liver function should be performed on all children with IBD, and if abnormal should raise the suspicion of PSC. The latter diagnosis can be confirmed by liver biopsy and cholangiography.
authors.affiliation: Department of Gastroenterology, Royal Alexandra Hospital for Children, Sydney, NSW.
authors.forename: J C
authors.initials: JC
authors.lastname: Ong
authors.forename: E V
authors.initials: EV
authors.lastname: O'Loughlin
authors.forename: K R
authors.initials: KR
authors.lastname: Kamath
authors.forename: S F
authors.initials: SF
authors.lastname: Dorney
authors.forename: M
authors.initials: M
authors.lastname: de Silva
authors.forename: K J
authors.initials: KJ
authors.lastname: Gaskin
mesh_terms: D001707:Biopsy, Needle; D002648:Child; D002675:Child, Preschool; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000601:surgery; D003093:Colitis, Ulcerative / Q000150:complications*; D003424:Crohn Disease / Q000150:complications*; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology*; D008111:Liver Function Tests; D008297:Male