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 language | English |
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Pages (from-to) | 370-375 |
Number of pages | 6 |
Journal | Journal of Pediatrics |
Volume | 99 |
Issue number | 3 |
DOIs | |
Publication status | Published - 1 Jan 1981 |