Sclerosing cholangitis: Pediatric perspective

Nanda Kerkar, Tamir Miloh

Research output: Contribution to journalReview articlepeer-review

31 Scopus citations

Abstract

Sclerosing cholangitis is a rare progressive cholestatic liver disease affecting the biliary tract. It may be associated with other diseases including autoimmune hepatitis, immunodeficiencies, cystic fibrosis, and sickle cell disease. Sclerosing cholangitis not associated with other diseases is termed"primary sclerosing cholangitis," which has a strong association with male gender, Caucasian race, and inflammatory bowel disease. Diagnosis is based on typical biochemical, radiologic, and histologic features. Medical management is directed mainly at managing complications (pruritus, cholangitis, strictures, and nutritional deficiencies). Administration of ursodeoxycholic acid results in biochemical improvement, but has not been proven to prolong transplant-free survival. Patients with autoimmune overlap respond to immunosuppression. The disease is typically progressive and evolves to biliary cirrhosis and possibly cholangiocarcinoma. Orthotopic liver transplantation remains the only life-extending alternative for patients with sclerosing cholangitis, with good longterm patient and graft survival, and recurrent graft primary sclerosing cholangitis in about 10% of children.

Original languageEnglish (US)
Pages (from-to)195-202
Number of pages8
JournalCurrent gastroenterology reports
Volume12
Issue number3
DOIs
StatePublished - Jun 2010
Externally publishedYes

Keywords

  • Autoimmune overlap
  • Biliary cirrhosis
  • Children
  • Cholangitis
  • Cholestasis
  • Liver transplantation
  • Outcome
  • Portal hypertension

ASJC Scopus subject areas

  • Gastroenterology

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