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罕见病因所致肉眼血尿:胡桃夹综合征

Gross hematuria of uncommon origin: the nutcracker syndrome.

作者信息

Russo D, Minutolo R, Iaccarino V, Andreucci M, Capuano A, Savino F A

机构信息

Istituto di Scienze Radiologiche, School of Medicine, University "Federico II,", Naples, Italy.

出版信息

Am J Kidney Dis. 1998 Sep;32(3):E3. doi: 10.1053/ajkd.1998.v32.pm10074588.

DOI:10.1053/ajkd.1998.v32.pm10074588
PMID:10074588
Abstract

Left renal vein hypertension, also called "nutcracker phenomenon" or "nutcracker syndrome," is a rare vascular abnormality responsible for gross hematuria. The phenomenon is attributable to the idiopathic decrease in the angle between the aorta and the superior mesenteric artery with consequent compression of the left renal vein. The entrapment of the left renal vein is not easily detectable by ordinary diagnostic procedures. We report two cases of gross hematuria (persistent in one patient and recurrent in the other) caused by "nutcracker phenomenon." In both cases, no remarkable findings were obtained from medical history, urinary red blood cells morphology, repeated urinalysis, pyelography, cystoscopy, or ureteroscopy. Left renal vein dilation in one case was found with a computed tomography (CT) scan performed on the venous tree of left kidney. The diagnosis of "nutcracker phenomenon" was confirmed by renal venography with measurement of pressure gradient between left renal vein and inferior vena cava in both cases. In one case, the diagnosis was complicated by the presence of Mycobacterium tuberculosis in urine. The "nutcracker phenomenon" is probably more common than thought. Early diagnosis is important to avoid unnecessary diagnostic procedures and complications such as the thrombosis of the left renal vein. Many procedures are available to correct the compression of the left renal vein entrapped between the aorta and the superior mesenteric artery: Gortex graft vein interposition, nephropexy, stenting, and kidney autotransplantation. After surgery, gross hematuria ceases in almost all patients.

摘要

左肾静脉高压,也称为“胡桃夹现象”或“胡桃夹综合征”,是一种导致肉眼血尿的罕见血管异常。该现象归因于主动脉与肠系膜上动脉之间夹角的特发性减小,进而导致左肾静脉受压。普通诊断程序不易检测到左肾静脉受压情况。我们报告了两例由“胡桃夹现象”引起的肉眼血尿病例(一例持续存在,另一例反复出现)。在这两例中,病史、尿红细胞形态、多次尿液分析、肾盂造影、膀胱镜检查或输尿管镜检查均未发现明显异常。其中一例通过对左肾静脉树进行计算机断层扫描(CT)发现左肾静脉扩张。两例均通过肾静脉造影并测量左肾静脉与下腔静脉之间的压力梯度确诊为“胡桃夹现象”。在一例中,由于尿液中存在结核分枝杆菌,使诊断变得复杂。“胡桃夹现象”可能比想象的更为常见。早期诊断对于避免不必要的诊断程序和诸如左肾静脉血栓形成等并发症很重要。有多种方法可用于纠正夹在主动脉和肠系膜上动脉之间的左肾静脉受压情况:使用戈尔特斯移植静脉搭桥、肾固定术、支架置入术和自体肾移植术。手术后,几乎所有患者的肉眼血尿都会停止。

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Gross hematuria of uncommon origin: the nutcracker syndrome.罕见病因所致肉眼血尿:胡桃夹综合征
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