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The Korean Journal of Pathology 1982;16(4): 878-883.
선천성 감섬유증 1예
Congenital Hepatic Fibrosis -A case report-
ABSTRACT
Congenital hepatic fibrosis is an unusual congenital disorder of the liver, manifestating a heredofamilial tendency and presenting within the first two decades of life. Congenital hepatic fibrosis is characterized by a generalized portal and interlobular fibrosis of the liver accompanied by bile duct hyperplsia. The intrahepatic portal involvement results in presinusoidal block with signs of portal hypertension and bleeding from esophageal varices. Although the lobular architecture of the liter may appear distorter, the hepatocytes are not affected. Liver function is normal, except in cases in which superimposed liver pathology may causes minor functional abnormalities. In some autosomal recessive manner and it was particularly in these cases that they found evidence of polycystic disease of the kidney. In a study of the pyelographic findings Kerr et al. drew attention to the similarities to medullary sponge kidney. Death has occured in 50 percent of the patients, mainly from uremia or massive variceal bleeding. The survivors have no or minor symptoms. The patients with portal hypertension due to congenital hepatic fibrosis are ideal candidates for portosystemic stunt. We have recently experienced a case of congenital hepatic fibrosis in 14 year old Korean male child. The patient's chief complaint was repeated hematemesis from esophageal varicies in recent years. There are marked hepato-splenomegaly and mild anemia but routine CBC and liver function test were within normal limits. Hassab precedure (castro-esophageal decongestion with splenectomy) and liver wedge biopsy were performed. The spleen weighed 720 ems and showed marked congestion of red pulp. The liver was extremely firm and enlasged. The microscopic findings are characterized by wide and generalized portal and interlobular fibrosis with bile duct hyperplasia, but no evidence of previousliver cell necrosis was observed. The patient was discharged in improved condition and has no recurrent hematemesis.
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