Related Experiment Videos
[Congenital hepatic fibrosis. Study of 26 cases]
J A Ramírez Mayans1, N Mata Rivera, M A Mora Tiscareño
1Servicio de Gastroenterología, Instituto Nacional de Pediatría, Distrito Federal, México.
Insights
Congenital hepatic fibrosis in children often presents with hematemesis and liver enlargement. Portal hypertension is common, and liver biopsy is key for diagnosis.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Medical Genetics
Abstract:
We studied 26 children with congenital hepatic fibrosis during the period 1971-1993. About half of the children were about the 6 years old. Only two had brothers with same disease. The chief clinical manifestation was hematemesis associated or not with liver enlargement, predominantly of left lobe. Only one case showed fever and cholangitis. Liver function tests were usually normal. Twenty-two children had portal hypertension. Liver biopsy was of definitive for diagnosis. Seven children died.