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布加综合征(BCS):不容忽视的具有挑战性的诊断——单中心报告及影像学研究。

Budd-Chiari syndrome (BCS): a challenging diagnosis not to be overlooked-single center report and pictorial essay.

机构信息

Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

University Clinic for Visceral Surgery and Medicine, INSELSPITAL, University Hospital of Bern, Bern, Switzerland.

出版信息

J Ultrasound. 2023 Mar;26(1):249-254. doi: 10.1007/s40477-022-00723-x. Epub 2022 Sep 30.

Abstract

Budd-Chiari syndrome (BCS) is a rare disease with a variable clinical presentation and often late diagnosis. Doppler ultrasonography (DUS) permits to determine the site of the obstructed venous tracts, the thrombotic or non-thrombotic nature of the obstruction with its morphologic features and the flow-pattern alterations. Other non-specific findings, which are seen in most of the other liver diseases, include ascites, hepatosplenomegaly and caudate hypertrophy. The aim of this study is to show our experience in BCS reporting retrospectively 15 cases referred to our hepatology center between 2017 and 2021. Four selected cases depict the extreme heterogeneous behaviour of BCS and highlight the importance of DUS as a diagnostic tool when there is a clinical suspicion. In patients, mainly young, who present with ascites and abdominal pain, BCS has to be considered and DUS is the first imaging technique to be performed to rule it out.

摘要

布加综合征(BCS)是一种罕见疾病,临床表现多样,常诊断较晚。多普勒超声检查(DUS)可确定受阻静脉的部位、梗阻的血栓或非血栓性质及其形态特征和血流模式改变。其他非特异性发现,在大多数其他肝脏疾病中都可见到,包括腹水、肝脾肿大和尾状叶肥大。本研究旨在回顾性展示我们在 2017 年至 2021 年间向我们的肝脏病学中心转诊的 15 例 BCS 报告的经验。四个精选病例描绘了 BCS 的极端异质性行为,并强调了在有临床怀疑时 DUS 作为诊断工具的重要性。在出现腹水和腹痛的主要为年轻患者中,应考虑 BCS,DUS 是首先要进行的以排除该病的影像学技术。

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