title: Primary sclerosing cholangitis in Arab children: report of four cases and literature review.
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.
authors.affiliation: Department of Pediatrics, King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia.
authors.forename: A F
authors.initials: AF
authors.lastname: Kagalwalla
authors.forename: I
authors.initials: I
authors.lastname: Altraif
authors.forename: L
authors.initials: L
authors.lastname: Shamsan
authors.forename: M
authors.initials: M
authors.lastname: Omojola
authors.forename: H
authors.initials: H
authors.lastname: Khan
authors.forename: Y A
authors.initials: YA
authors.lastname: Kagalwalla
mesh_terms: D001653:Bile Ducts, Intrahepatic / Q000000981:diagnostic imaging*; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology*; D009336:Necrosis; D012189:Retrospective Studies; D012529:Saudi Arabia