Congenital Hepatic Fibrosis with Caroli's Disease
Korean Journal of Pathology
;
: 275-279, 1997.
Article
in Korean
| WPRIM
| ID: wpr-84712
ABSTRACT
Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Bile
/
Bile Ducts
/
Bile Ducts, Intrahepatic
/
Fibrosis
/
Esophageal and Gastric Varices
/
Tomography, X-Ray Computed
/
Liver Transplantation
/
Caroli Disease
/
Fever
/
Headache
Limits:
Female
/
Humans
Language:
Korean
Journal:
Korean Journal of Pathology
Year:
1997
Type:
Article
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