OBJECTIVE: To assess the frequency and clinical picture of Hepatoportal
Sclerosis in a population of Mexican children of the Instituto Nacional
de Pediatria, Mexico 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 Pediatria, Mexico 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.
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