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Case Report
A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst
Bong Seok Choi, Sang Nam Bae, Yong Tak Im, Jae Hong Park, Chang Hoon Lee, Jun Woo Lee
Clin Exp Pediatr. 2002;45(7):923-927.   Published online July 15, 2002
Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis...
Two Cases of Congenital Hepatic Fibrosis
Cue Jung Hwang, Young Hun Kim, Dae Kyun Koh, Byung Churl Lee
Clin Exp Pediatr. 1992;35(4):569-574.   Published online April 15, 1992
The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly,...
Caroli's Disease.
Jong Hoon Park, In Hyun Cho, Sun Ja Lee, Dong Hyuk Kum, Soo Dong Pai
Clin Exp Pediatr. 1981;24(4):401-406.   Published online April 15, 1981
Caroli*s disease, a rare syndrome characterized by congenital, segmental saccular dilatation of intrahepatic bile ducts, is first described by Caroli & associaties in 1958. It is though by many to be part of spectrum of congenital hepatic fibrosis. The disease usually presents with bile stasis and stone formation with further complication arising from recurrent cholangitis and liver abscess. The preoperative diagnosis...