Congenital hepatic fibrosis in Jamaican children.

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Abstract
Clinical and pathological data are presented on 8 children with the syndrome described as congenital hepatic fibrosis. The karyotypes were normal in the 3 children in whom this examination was undertaken. Four also had polycystic disease of the kidneys. These studies emphasize the presence of recurrent fevers and infections and severe anemia as important features when the disease occurs in early childhood. Clinical portal hypertension was not seen. As in previous studies, a strong familial tendency was encountered. The exact etiology remains obscure.