Two Cases of Congenital Hepatic Fibrosis with Polycystic Kidney Disease.
- Author:
Dong Hyun LEE
1
;
Ok Nyu KONG
;
Ji Young KIM
;
Chan Won PARK
;
Jae Hyeon MOON
;
Dae Gun NAM
;
Hyung Jun CHU
;
Dae Hwan KANG
;
Geun Am SONG
;
Mong CHO
;
Ung Suk YANG
Author Information
1. Department of Internal Medicine, College of Medicine, Pusan National University, Busan, Korea. word-prayer@hanmail.net
- Publication Type:Case Report
- Keywords:
Congenital hepatic fibrosis;
Portal hypertension;
Polycystic kidney disease;
Achacnoid cyst
- MeSH:
Bile Ducts;
Biopsy;
Brain;
Diagnosis;
Esophageal and Gastric Varices;
Fibrosis*;
Hematemesis;
Humans;
Hypertension, Portal;
Kidney;
Ligation;
Liver;
Magnetic Resonance Imaging;
Needles;
Polycystic Kidney Diseases*;
Polycystic Kidney, Autosomal Recessive;
Splenomegaly;
Splenorenal Shunt, Surgical;
Tomography, X-Ray Computed;
Varicose Veins;
Young Adult
- From:The Korean Journal of Hepatology
2001;7(4):485-490
- CountryRepublic of Korea
- Language:Korean
-
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.
