Two Cases of Congenital Hepatic Fibrosis with Polycystic Kidney Disease / 대한간학회지
The Korean Journal of Hepatology
;
: 485-490, 2001.
Article
in Korean
| WPRIM
| ID: wpr-146380
ABSTRACT
Congenital hepatic fibrosis (CHF) is a rare developemental abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all, cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The pateint also had cystic dilation of subarchnoid space in the basal cistern and posterior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. He had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Splenomegaly
/
Varicose Veins
/
Bile Ducts
/
Biopsy
/
Fibrosis
/
Brain
/
Magnetic Resonance Imaging
/
Splenorenal Shunt, Surgical
/
Esophageal and Gastric Varices
/
Tomography, X-Ray Computed
Type of study:
Diagnostic study
Limits:
Humans
Language:
Korean
Journal:
The Korean Journal of Hepatology
Year:
2001
Type:
Article
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