Pediatric Drugs

, Volume 13, Issue 2, pp 87–95

Current Management of Primary Sclerosing Cholangitis in Pediatric Patients

Review Article

DOI: 10.2165/11586500-000000000-00000

Cite this article as:
Ibrahim, S.H. & Lindor, K.D. Pediatr-Drugs (2011) 13: 87. doi:10.2165/11586500-000000000-00000


Primary sclerosing cholangitis (PSC) is a chronic cholestatic condition affecting the extrahepatic and intrahepatic biliary tree. The incidence is seemingly increasing in children as a result of increased use of cholangiographic screening techniques in children with inflammatory bowel disease. The clinical presentation of PSC in childhood is variable and frequently without obvious cholestatic features, and cholangiography is essential for the diagnosis of this disorder. Histologic findings may help to exclude autoimmune sclerosing cholangitis. The underlying pathogenesis remains poorly understood and, as a result, therapeutic agents that halt disease progression and improve prognosis are lacking. PSC treatment is mainly supportive and directed at controlling cholestatic symptoms and preventing complications. Ursodeoxycholic acid is helpful in inducing biochemical improvement; long-term pediatric studies to determine a benefit of this agent in young patients are lacking, although results from adult studies have not been promising. Some agents such as antibiotics are under investigation with some promising results. Liver transplantation is required for children who progress to end-stage liver disease. Prospective multicenter trials in children with PSC are needed.

Copyright information

© Adis Data Information BV 2011

Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  2. 2.Department of Medicine, Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  3. 3.Mayo Clinic College of MedicineRochesterUSA

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