[Congenital liver fibrosis].
In two siblings-girls who died as a result of infectious complications after previous protracted hypoxic brain damage at the age of weeks and 5 months postmortem examination revealed congenital fibrosis of the liver. Morphological examination of hepatic tissue of the younger infant revealed in the small porto-biliary spaces malformation of the ductal trabeculae and in the periphery of the major and large and unequal hyperplasia of the minor interlobular bile ducts which partly penetrated from the periportal area into the lobules which were not structurally altered. In the older infant there was a more marked ductal hyperplasia associated with connective tissue proliferation and the course of the disease was complicated by abscedent cholangitis.
Chlumská
A
A
II. patologicko-anatomický ústav 1. LF UK, Praha.
Vítková
I
I
D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000151:congenital* / Q000235:genetics / Q000473:pathology
Kongenitálni fibróza jater.