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遗传性出血性毛细血管扩张症与难治性腹水

Hereditary Hemorrhagic Telangiectasia and Refractory Ascites.

作者信息

Thandassery Ragesh B, Patel Rahul S, Grewal Priya

机构信息

Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York City, NY.

Interventional Radiology, Icahn School of Medicine at Mount Sinai, New York City, NY.

出版信息

ACG Case Rep J. 2020 Oct 14;7(10):e00458. doi: 10.14309/crj.0000000000000458. eCollection 2020 Oct.

Abstract

Hereditary hemorrhagic telangiectasia is a rare autosomal dominant disease that can involve the liver. The presence of arteriohepatic venous shunts can lead to high output cardiac failure and biliary ischemia, whereas arterioportal venous shunts can result in portal hypertension. Cirrhosis and nodular regenerative hyperplasia are also reported in these patients. Management of these patients in the setting of symptomatic liver disease is challenging. Transarterial embolization and hepatic artery ligation are usually considered palliative options. In selected cases, orthotopic liver transplantation can cure both liver disease and heart failure.

摘要

遗传性出血性毛细血管扩张症是一种罕见的常染色体显性疾病,可累及肝脏。肝动静脉分流的存在可导致高输出量心力衰竭和胆汁性缺血,而肝门动静脉分流可导致门静脉高压。这些患者中也有肝硬化和结节性再生性增生的报道。对有症状性肝病的这些患者进行管理具有挑战性。经动脉栓塞和肝动脉结扎通常被认为是姑息性选择。在某些特定病例中,原位肝移植可治愈肝病和心力衰竭。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c654/7556362/bab727d682cd/ac9-7-e00458-g001.jpg

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