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两名儿童中出现的伴有膜增生性肾小球肾炎样表现的IgA相关性肾小球肾炎。

IgA-associated glomerulonephritis with membranoproliferative glomerulonephritis-like pattern in two children.

作者信息

Iitaka Kikuo, Yamamoto Asako, Ogawa Natsuko, Sekine Toru, Tamai Shinya, Motoyama Osamu

机构信息

Department of Pediatrics, Yamato City Hospital, 8-3-6 Fukaminishi, Yamato, Kanagawa 242-8602, Japan.

出版信息

Clin Exp Nephrol. 2003 Dec;7(4):284-9. doi: 10.1007/s10157-003-0253-z.

Abstract

In this article, we report two patients with IgA-associated glomerulonephritis with a membranoproliferative glomerulonephritis (MPGN) -like pattern. Both patients had nephrotic syndrome at onset. One patient was treated with high-dose alternate-day prednisolone (PSL), and the other with indomethacin and low-dose PSL. One lost the urinary abnormalities 3 years after starting treatment. The other lost the nephrotic state and hematuria over a 5-year period, but proteinuria persisted until the last follow-up. Both patients had diffuse proliferative changes with mesangial interposition and subendothelial deposits, associated with strongly positive deposits of C3 and IgA along the capillary walls of the glomeruli. These two patients showed histological changes compatible with type-I MPGN, but the pattern of IgA deposits was not typical of idiopathic MPGN or IgA nephropathy. We assume this is a rare form of MPGN, not associated with liver disease or other systemic diseases.

摘要

在本文中,我们报告了两名患有IgA相关性肾小球肾炎且具有膜增生性肾小球肾炎(MPGN)样模式的患者。两名患者起病时均患有肾病综合征。一名患者接受大剂量隔日泼尼松龙(PSL)治疗,另一名患者接受吲哚美辛和小剂量PSL治疗。一名患者在开始治疗3年后尿异常消失。另一名患者在5年期间肾病状态和血尿消失,但蛋白尿持续至最后一次随访。两名患者均有弥漫性增殖性改变,伴有系膜插入和内皮下沉积物,肾小球毛细血管壁有强阳性的C3和IgA沉积物。这两名患者的组织学改变与I型MPGN相符,但IgA沉积模式并非特发性MPGN或IgA肾病的典型表现。我们认为这是一种罕见的MPGN形式,与肝脏疾病或其他全身性疾病无关。

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