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POEMS 综合征伴反复发作急性肾衰竭一例的连续肾脏活检结果。

Serial renal biopsy findings in a case of POEMS syndrome with recurrent acute renal failure.

机构信息

Department of Nephrology, Kyoto University Hospital, 54 Shogoin-kawaharacho, Sakyo-ku, Kyoto, 606-8507, Japan.

出版信息

Clin Exp Nephrol. 2012 Feb;16(1):173-9. doi: 10.1007/s10157-011-0540-z. Epub 2011 Oct 8.

Abstract

This report describes a patient presenting with recurrent acute renal failure occurring in the course of POEMS syndrome, a multisystem disease associated with plasma cell dyscrasia. Several combined immunosuppression therapies failed to resolve recurrent acute renal failure; autologous peripheral blood stem cell transplantation was therefore applied. A renal biopsy was performed on each of four occasions when he developed renal dysfunction. The renal biopsy showed typical renal histology of POEMS, membranoproliferative glomerulonephritis-like lesions and narrowing of vessel lumina of various sizes caused by endothelial injury, which progressed to glomerulosclerosis and vessel occlusion. Recurrent acute renal failure might be caused by ischemia due to arterial occlusion. Serum levels of vascular epithelial growth factor (VEGF), which is considered to be a causative factor of endothelial lesions in POEMS syndrome, were not elevated throughout the course of this case.

摘要

本报告描述了一例以 POEMS 综合征为表现的复发性急性肾衰竭患者,POEMS 综合征是一种与浆细胞异常相关的多系统疾病。几种联合免疫抑制疗法未能解决复发性急性肾衰竭;因此应用了自体外周血干细胞移植。当他出现肾功能障碍时,进行了四次肾活检。肾活检显示 POEMS 的典型肾脏组织学改变、膜增殖性肾小球肾炎样病变以及内皮损伤引起的各种大小血管腔狭窄,进而发展为肾小球硬化和血管闭塞。复发性急性肾衰竭可能是由于动脉闭塞引起的缺血所致。在本病例过程中,血管内皮生长因子(VEGF)的血清水平并未升高,VEGF 被认为是 POEMS 综合征内皮病变的一个致病因素。

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