OBJECTIVE: To assess the frequency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City.
BACKGROUND: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficult. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts.
METHODS: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specific findings of splenoportography and histologic changes in liver biopsy.
RESULTS: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenic, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotherapy. At the time of this report all children have shown a good clinical course.