阿伯内西畸形中肝脏病变从良性到恶性的转变
Benign to Malignant Hepatic Lesion Transformation in Abernethy Malformation.
作者信息
Tom Chloe K, Ter-Oganesyan Ramon, Shefali Chopra, Kaur Navpreet, Pace Jordan L, Rastegarpour Ali, Genyk Yuri, Kahn Jeffrey A
机构信息
Division of Gastrointestinal and Liver Diseases, University of Southern California, Los Angeles, CA, USA.
Division of Vascular and Interventional Radiology, Department of Radiology, University of Southern California, Los Angeles, CA, USA.
出版信息
ACG Case Rep J. 2024 Apr 5;11(4):e01307. doi: 10.14309/crj.0000000000001307. eCollection 2024 Apr.
Abernethy malformation or congenital extrahepatic portosystemic shunt is an extremely rare condition whereby the portomesenteric blood drains into a systemic vein and bypasses the liver through a complete or partial shunt. Severe complications include hyperammonemia and encephalopathy, benign and malignant liver tumors, and hepatopulmonary syndrome. We describe a case where a female adult diagnosed with congenital extrahepatic portosystemic shunt subsequently developed focal nodular hyperplasia and then hepatocellular carcinoma.
阿伯内西畸形或先天性肝外门体分流是一种极其罕见的病症，即门静脉肠系膜血液通过完全或部分分流流入体静脉并绕过肝脏。严重并发症包括高氨血症和脑病、良性和恶性肝脏肿瘤以及肝肺综合征。我们描述了一例成年女性病例，该患者被诊断为先天性肝外门体分流，随后发展为局灶性结节性增生，进而发展为肝细胞癌。
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