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结节性硬化症:介入放射科医生在管理中的关键作用。

Tuberous sclerosis complex: The critical role of the interventional radiologist in management.

作者信息

Garg Puneet, Sharma Anuradha, Rajani Heena, Choudhary Apratim R, Meena Rajkumar

机构信息

Department of Radiology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

出版信息

SA J Radiol. 2021 Mar 30;25(1):2034. doi: 10.4102/sajr.v25i1.2034. eCollection 2021.

Abstract

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome that is characterised by hamartomas in multiple organs, the characteristic imaging features of which are illustrated in this case report. Angiomyolipoma (AML) is the most common renal manifestation of TSC, which may present with life-threatening haemorrhage at the time of diagnosis. Interventional management with selective renal embolisation is currently the treatment of choice for the safe and effective management of ruptured renal AML.

摘要

结节性硬化症(TSC)是一种常染色体显性神经皮肤综合征,其特征为多个器官出现错构瘤,本病例报告展示了其特征性影像学表现。肾血管平滑肌脂肪瘤(AML)是TSC最常见的肾脏表现,在诊断时可能出现危及生命的出血。选择性肾动脉栓塞介入治疗目前是破裂性肾AML安全有效管理的首选治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e532/8063773/2f06919e414b/SAJR-25-2034-g001.jpg

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