CONGENITAL HEPATIC FIBROSIS WITH POLYCYSTIC KIDNEY DISEASE PRESENTED AS PORTAL HYPERTENSION IN A 7 YEAR OLD FEMALE CHILD – A RARE CASE REPORT

Abstract

INTRODUCTION
Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive
disorder, clinically characterized by hepatic fibrosis and portal
hypertension. CHF results from ductal plate malformation (DPM) of
the intrahepatic bile ducts. Four clinical forms can be observed: portal
hypertensive, cholangitic, mixed and latent. CHF is one of the
“fibropolycystic diseases” which also include several conditions with
a variety of intrahepatic bile duct dilatation and associated periportal
fibrosis such as Caroli disease, autosomal recessive and dom-inant
polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune,
Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes. Most of
them are accompanied by progressive cystic degeneration of the
kidneys. We present the case of a 7-year-old female patient with CHF
with POLYCYSTIC KIDNEY DISEASE and a review of the
literature.