Abstract

A 15-yr-old girl presented with complaints of right upper quadrant pain and jaundice. Elevation of serum alkaline phosphatase, signs of protal hypertension, and computed tomographic scan findings suggested a diagnosis of primary sclerosing cholangitis. However, cultures of the bile and of the common bile duct specimen obtained during a surgical procedure grew Cryptococcus neoformans. Treatment with amphotericin B was begun. An episode of upper gastrointestinal bleeding, however, led to the hepatorenal syndrome, and the patient died before antifungal therapy was completed. At autopsy, active sclerosing cholangitis associated with cryptococci involved the common bile duct. We suggest that opportunistic infection of the biliary tree should be considered in pediatric patients with presumed primary sclerosing cholangitis.

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