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遗传性出血性毛细血管扩张症的肝脏受累表现为类似局灶性结节性增生。

Hepatic involvement in hereditary hemorrhagic telangiectasia mimicking focal nodular hyperplasia.

作者信息

Alvarenga Fernandes Daniel, de Fátima Ferreira Santana Boin Ilka, Batista Araújo Filho José de Arimatéia, Cristina de Ataíde Elaine, Marchiori Edson, Reis Fabiano

机构信息

Radiology, University of Campinas (UNICAMP), BRAZIL.

Liver Transplantation Unit, Department of Surgery, University of Campinas (UNICAMP), BRAZIL.

出版信息

Rev Esp Enferm Dig. 2023 Mar;115(3):152-154. doi: 10.17235/reed.2022.9069/2022.

Abstract

Accurate diagnosis of hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is important for reducing the risk of complications. Hepatic involvement in HHT is usually asymptomatic, but when present can cause noted morbidity and mortality. A 62-year-old woman presented with moderate upper-abdominal pain and tachycardia. A diagnosis of HHT was made based on the findings of hepatic involvement in a contrast-enhanced abdominal computed tomography (CT) scan, the presence of arteriovenous malformations (AVMs; a.k.a. telangiectasias) on mucocutaneous surfaces, and a history of recurrent epistaxis. Imaging methods are important diagnostic tools in patients suspected of having HHT.

摘要

准确诊断遗传性出血性毛细血管扩张症(HHT),又称伦杜-奥斯勒-韦伯病,对于降低并发症风险至关重要。HHT的肝脏受累通常无症状,但一旦出现则可导致明显的发病和死亡。一名62岁女性出现中度上腹部疼痛和心动过速。根据腹部增强计算机断层扫描(CT)显示的肝脏受累情况、黏膜皮肤表面存在动静脉畸形(AVM;又称毛细血管扩张症)以及反复鼻出血病史,诊断为HHT。影像学方法是疑似患有HHT患者的重要诊断工具。

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