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肝脏遗传性出血性毛细血管扩张症:病理生理学及放射学在诊断和治疗中的作用

Hereditary hemorrhagic telangiectasia of liver: Pathophysiology with role of radiology in diagnosis and treatment.

作者信息

Viyannan Maheswaran, Balalakshmoji Devanand, Leelakrishnan Venkatakrishnan

机构信息

Department of Radiology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore, Tamil Nadu, India.

Department of Med. Gastro, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore, Tamil Nadu, India.

出版信息

Indian J Radiol Imaging. 2020 Jan-Mar;30(1):98-101. doi: 10.4103/ijri.IJRI_367_19. Epub 2020 Mar 30.

Abstract

Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is a rare condition which can result in significant abnormalities. Liver involvement in HHT consists primarily of the consequence of various intrahepatic shunts. Even though these vascular shunts are present in the majority of patients with HHT, symptoms occur only in with clear predilection to . The symptoms and imaging findings of liver vascular malformations can be easily overlooked or misdiagnosed which can result in delay in treatment or potentially harmful vascular interventions. In this case report, we discuss the pathophysiology of HHT in liver involvement, role of imaging in diagnosis, and the possible role of interventional radiologist in the treatment.

摘要

遗传性出血性毛细血管扩张症(HHT)或奥斯勒-韦伯-伦杜综合征是一种罕见疾病,可导致严重异常。HHT累及肝脏主要是各种肝内分流的结果。尽管大多数HHT患者存在这些血管分流,但症状仅在具有明显倾向的患者中出现。肝脏血管畸形的症状和影像学表现很容易被忽视或误诊,这可能导致治疗延迟或潜在有害的血管介入。在本病例报告中,我们讨论了HHT累及肝脏的病理生理学、影像学在诊断中的作用以及介入放射科医生在治疗中的可能作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd20/7240902/e37aa8108b3d/IJRI-30-98-g001.jpg

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