Congenital hepatic fibrosis in children

F. Alvarez, O. Bernard, F. Brunelle, M. Hadchouel, A. Leblanc, M. Odièvre, D. Alagille

    Research output: Contribution to journalArticlepeer-review

    115 Citations (Scopus)

    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.

    Original languageEnglish
    Pages (from-to)370-375
    Number of pages6
    JournalJournal of Pediatrics
    Volume99
    Issue number3
    DOIs
    Publication statusPublished - 1 Jan 1981

    Cite this