Primary sclerosing cholangitis in arab children: Report of four cases and literature review

Amir F. Kagalwalla, I. Altraif, L. Shamsan, M. Omojola, H. Khan, Y. A. Kagalwalla*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

9 Scopus citations

Abstract

Background: The prevalence of primary sclerosing cholangitis, a rare progressive liver disorder, is increasing with the advent of endoscopic retrograde cholangiography in the investigation of children with obscure liver disease. The etiology of primary sclerosing cholangitis is not known, clinical presentation is variable, treatment is only of limited success and long-term studies on prognosis in children are incomplete. Primary sclerosing cholangitis has not been described in Arab children. Methods: To describe detailed clinical, laboratory, histologic and radiological features in 4 children with primary sclerosing cholangitis identified over a 2-year period at a tertiary referral center in Riyadh Saudi Arabia. Results: Four children, all females, between the ages of 4 and 11 years with primary sclerosing cholangitis are identified. The diagnosis was suggested by the histology and confirmed by the characteristic cholangiographic findings. Clinical findings were itching in all 4 children, hepatomegaly in 3 and splenomegaly in 4 patients. Three of 4 patients had portal hypertension and 2 of these three variceal hemorrhage. Antinuclear and smooth muscle antibodies were negative in all four patients. None had clinical manifestations of chronic inflammatory bowel disease but microscopic colitis was documented in all 4 patients. Conclusions: Primary sclerosing cholangitis is present in Arab children with a prevalence rate of 5% in our study population.

Original languageEnglish (US)
Pages (from-to)146-152
Number of pages7
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume24
Issue number2
DOIs
StatePublished - Feb 1 1997

Keywords

  • Autoimmune hepatitis
  • ERCP
  • Inflammatory bowel disease
  • Primary sclerosing cholangitis
  • Ursodeoxycholic acid

ASJC Scopus subject areas

  • Gastroenterology
  • Histology
  • Medicine (miscellaneous)
  • Food Science
  • Pediatrics, Perinatology, and Child Health

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