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June 1981

Nodular Regenerative Hyperplasia of the Liver: An Unusual Cause of Portal Hypertension in Childhood

Am J Dis Child. 1981;135(6):572-574. doi:10.1001/archpedi.1981.02130300070026

Nodular regenerative hyperplasia of the liver is a disease usually of adults, characterized by portal hypertension, normal or mildly elevated serum concentrations of alkaline phosphatase and transaminase, and diffuse regenerative nodules throughout the liver with absent or minimal fibrosis. Because the youngest patient described thus far with nodular regenerative hyperplasia of the liver has been 17 years of age, this entity is seldom considered in the differential diagnosis of portal hypertension in childhood. In this report we describe a 14-year-old boy with portal hypertension secondary to nodular regenerative hyperplasia of the liver in whom the diagnosis was achieved only by laparotomy and a wedge liver biopsy.

Report of a Case.—A 14-year-old boy was admitted to the hospital for evaluation of splenomegaly and epistaxis. Otherwise he was asymptomatic, and there was no family history of bleeding, liver disease, parasites, or contact with Schistosoma mansoni.

The patient was well nourished, with