Current management of primary sclerosing cholangitis in pediatric patients

Samar H. Ibrahim, Keith Lindor

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)87-95
Number of pages9
JournalPediatric Drugs
Volume13
Issue number2
DOIs
StatePublished - 2011
Externally publishedYes

Fingerprint

Sclerosing Cholangitis
Pediatrics
Ursodeoxycholic Acid
End Stage Liver Disease
Cholangiography
Biliary Tract
Inflammatory Bowel Diseases
Liver Transplantation
Multicenter Studies
Disease Progression
Anti-Bacterial Agents
Incidence
Therapeutics

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pharmacology (medical)

Cite this

Current management of primary sclerosing cholangitis in pediatric patients. / Ibrahim, Samar H.; Lindor, Keith.

In: Pediatric Drugs, Vol. 13, No. 2, 2011, p. 87-95.

Research output: Contribution to journalArticle

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