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一例成人多囊肾病患者下腔静脉受压的疑难病例。病例报告。

A Challenging Case of Inferior Vena Cava Compression in an Adult Polycystic Kidney Disease Patient. A Case Report.

作者信息

Matarneh Ahmad, Akkari Abdelraouf, Sardar Sundus, Miller Ronald, Verma Navin, Ghahramani Nasrollah, Farooq Umar

机构信息

Department of Nephrology Penn State Milton S Hershey Medical Center Hershey Pennsylvania USA.

出版信息

Clin Case Rep. 2025 Mar 2;13(3):e70283. doi: 10.1002/ccr3.70283. eCollection 2025 Mar.

Abstract

Adult polycystic kidney disease (ADPKD) is a multi-system genetic disorder characterized by the development and progressive enlargement of fluid-filled cysts in both kidneys, along with other organs. As one of the main causes of kidney failure, ADPKD can progress to end-stage renal disease (ESRD), with over 50% of affected individuals progressing to ESRD by age 50. The symptoms in ADPKD are variable, with some patients experiencing nonspecific signs, while others present with symptoms related to the mass effect of enlarged kidneys on surrounding structures. This case report highlights an unusual presentation of ADPKD in a patient who developed symptoms of inferior vena cava (IVC) compression. Remarkably, these symptoms improved after bilateral nephrectomies, suggesting that bilateral nephrectomy would provide help in these situations.

摘要

成人多囊肾病(ADPKD)是一种多系统遗传性疾病,其特征是双肾以及其他器官中出现充满液体的囊肿并逐渐增大。作为肾衰竭的主要原因之一,ADPKD可发展为终末期肾病(ESRD),超过50%的患者在50岁时会发展为ESRD。ADPKD的症状各不相同,一些患者出现非特异性体征,而另一些患者则表现出与增大的肾脏对周围结构的占位效应相关的症状。本病例报告强调了ADPKD在一名出现下腔静脉(IVC)压迫症状患者中的不寻常表现。值得注意的是,双侧肾切除术后这些症状有所改善,这表明双侧肾切除术在这些情况下可能会有所帮助。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68b4/11873366/15e1d2c3f844/CCR3-13-e70283-g004.jpg

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