PEDIATRICS Vol. 80 No. 6 December 1987, pp. 918-923
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Primary Sclerosing Cholangitis in Children: Study of Five Cases and Review of the Literature

Alfred Sisto MD1, Patricia Feldman MD1, Laurent Garel MD1, Ernest Seidman MD1, Pierre Brochu MD1, Claude L. Morin MD1, Andrée M. Weber MD1, and Claude C. Roy MD1

1 From the Departments of Pediatrics, Radiology, and Pathology, Hôpital Ste-Justine and the University of Montreal, Montreal

Primary sclerosing cholangitis in five children is described and 78 cases in the pediatric age group are reviewed. In 24% of the cases, primary sclerosing cholangitis is not associated with an underlying disease and may appear to be prolonged cholestasis of infancy. When an associated condition is present, chronic inflammatory bowel disease, in particular ulcerative colitis, is most common (47%). Histiocytosis X and a variety of immune disorders account for 15% and 10% of cases, respectively. Primary sclerosing cholangitis should be considered in the differential diagnosis of chronic liver disease in the pediatric age group, even in young infants. Results of this survey demonstrate that neither clinical features nor liver function tests are reliable diagnostic predictors, that histologic changes are often nonspecific, and that cholangiography is essential to establish the correct diagnosis.

Key Words: cholangitis • cholestasis • biliary tract • histiocytosis X • cholangiography

Submitted on December 2, 1986
Accepted on April 29, 1987




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