Author(s): Alvarez F, Bernard O, Brunelle F, Hadchouel M, Leblanc A, , Alvarez F, Bernard O, Brunelle F, Hadchouel M, Leblanc A,
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Abstract Twenty-seven children with congenital hepatic fibrosis were followed for three months to 12 years. Hepatosplenomegaly, normal liver function tests, and kidney abnormalities were present in most patients, indicating that a correct diagnosis of congenital hepatic fibrosis could be made using simple clinical, biologic, and radiologic criteria. Esophageal endoscopy showed varices in 21 patients. Sixteen children underwent portal-systemic shunt surgery. Follow-up examinations did not show any impairment of liver function or any sign of hepatic encephalopathy. Cholangitis was present in only three children.
This article was published in J Pediatr
and referenced in Journal of Liver