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KMID : 0985520150200020105
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Korean Journal of Pancreas and Biliary Tract
2015 Volume.20 No. 2 p.105 ~ p.110
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A Case of Combined Congenital Hepatic Fibrosis and Biliary Hamartoma Presented with Recurrent Acute Cholangitis
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Kim Ki-Bae
Ji Myoung-Jin
Yang Ji-Youl
Han Joung-Ho
Chae Hee-Bok
Park Seon-Mee
Kang Min-Ho
Sung Ro-Hyun
Youn Sei-Jin
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Abstract
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Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.
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KEYWORD
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Biliary hamartoma, Congenital hepatic fibrosis, Recurrent acute cholangitis
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