Tamir Miloh and Pinar Bulut, Clinical Liver Disease Special Issue: Liver Disease in Children
Volume 2, Issue 5, pages 215–218, October 2013
Primary sclerosing cholangitis (PSC) is an idiopathic, chronic hepatobiliary disorder characterized by inflammation with progressive obliterative fibrosis and focal dilatation of the intrahepatic and/or extrahepatic bile ducts which leads to biliary cirrhosis and end-stage liver disease.[1] The clinical features of PSC in children and adolescents are summarized in Table 2. PSC is more common in Caucasian male patients and has a strong association with inflammatory bowel disease (IBD), especially ulcerative colitis. In children, secondary causes of sclerosing cholangitis include Langerhans cell histiocytosis, immunodeficiencies, and cystic fibrosis. The incidence of PSC in children is 0.23 cases per 100,000 person-years compared with 1.11/100,000 in adults.[2] . . . Please click here to access the full text of this article. There is also a video presentation and an interview with the authors