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一名常染色体显性遗传性多囊肾病患者的夹层动脉瘤

Dissecting aneurysm in a patient with autosomal dominant polycystic kidney disease.

作者信息

Fukunaga Naoto, Yuzaki Mitsuru, Nasu Michihiro, Okada Yukikatsu

机构信息

Department of Cardiovascular surgery, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.

出版信息

Ann Thorac Cardiovasc Surg. 2012;18(4):375-8. doi: 10.5761/atcs.cr.11.01756. Epub 2012 Jan 31.

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is primarily associated with renal failure, but it also causes systemic diseases, including cysts of other systemic organs and cerebral or visceral aneurysm. To make matters worse, life-threatening aortic diseases are associated with ADPKD in some cases. However, only a few reports of ADPKD-associated with thoracic aortic dissection have been published. Herein, we present a case of dissecting aneurysm in a patient with hypertension and ADPKD. He had been followed up for type B aortic dissection for six years. Preoperative creatinine level ranged from 2.1 to 2.4 mg/dl. We performed replacement of the thoracic aorta with prosthetic graft successfully, and postoperatively, dialysis was not required.It is very important for us to recognize the relationship between ADPKD and thoracic aortic dissection, which can cause high mortality and morbidity rates.

摘要

常染色体显性多囊肾病(ADPKD)主要与肾衰竭相关,但它也会引发全身性疾病,包括其他全身器官的囊肿以及脑动脉瘤或内脏动脉瘤。更糟糕的是,在某些情况下,危及生命的主动脉疾病与ADPKD有关。然而,仅有少数关于ADPKD合并胸主动脉夹层的报道发表。在此,我们报告一例患有高血压和ADPKD的患者发生夹层动脉瘤的病例。他因B型主动脉夹层已接受了六年的随访。术前肌酐水平在2.1至2.4mg/dl之间。我们成功地用人工血管置换了胸主动脉,术后无需透析。认识到ADPKD与胸主动脉夹层之间的关系对我们非常重要,因为这可能导致高死亡率和高发病率。

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