A Case of Combined Congenital Hepatic Fibrosis and Biliary Hamartoma Presented with Recurrent Acute Cholangitis
| Autor: | Seon Mee Pakr, Myoung Jin Ji, Ki Bae Kim, Ji Youl Yang, Rohyun Sung, Hee Bok Chae, Min Ho Kang, Joung-Ho Han, Sei Jin Youn |
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| Rok vydání: | 2015 |
| Předmět: |
First episode
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Endoscopic retrograde cholangiopancreatography medicine.diagnostic_test business.industry medicine.medical_treatment Hepatosplenomegaly medicine.disease Gastroenterology Ductal Plate Malformation Liver biopsy Internal medicine medicine Hamartoma Congenital hepatic fibrosis Cholecystectomy medicine.symptom business |
| Zdroj: | Korean Journal of Pancreas and Biliary Tract. 20:105-110 |
| ISSN: | 2288-0941 |
| DOI: | 10.15279/kpba.2015.20.2.105 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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