膜性肾病作为孤立性免疫球蛋白a沉积的节段性模式:1例报告。

IF 0.7 Q4 UROLOGY & NEPHROLOGY
Shinya Yokote, Saeko Hatanaka, Akihiro Shimizu, Masahiro Okabe, Kotaro Haruhara, Takaya Sasaki, Hiroyuki Ueda, Kensuke Joh, Nobuo Tsuboi, Takashi Yokoo
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引用次数: 0

摘要

67岁男性,有高血压和血脂异常病史,表现为水肿和大量蛋白尿。肾活检的光镜分析显示弥漫性节段性膜质特征。免疫荧光染色显示沿肾小球毛细血管壁的IgA、半乳糖缺陷IgA1、κ和λ轻链和C3呈节段性阳性,但IgG、IgM或C1q呈阴性。电镜显示节段性肾小球毛细血管壁上皮下和膜内电子致密沉积(EDD),相应区域有足突消失。系膜区或系膜旁区未见EDD。患者的组织病理学显示膜性肾病伴孤立的IgA沉积。没有临床发现提示膜性肾病的继发原因。糖皮质激素和环孢素联合治疗导致蛋白尿缓解。据我们所知,这是第一例报道的膜性肾病与孤立IgA和半乳糖缺乏IgA1沉积相关的节段性模式。需要进一步的病例报告和研究来阐明膜性肾病的发病机制,这显示了这些独特的组织病理学特征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Membranous nephropathy as a segmental pattern with solitary immunoglobulin A deposition: a case report.

A 67-year-old man with a history of hypertension and dyslipidemia presented with edema and heavy proteinuria. Light microscopic analysis of kidney biopsy revealed a diffuse segmental membranous feature. Immunofluorescence stain was segmentally positive for IgA, galactose-deficient IgA1, both κ and λ light chains, and C3 along the glomerular capillary walls, but negative for IgG, IgM, or C1q. Electron microscopy showed subepithelial and intramembranous electron-dense deposits (EDD) in the segmental glomerular capillary walls, along with foot process effacement in the corresponding areas. No EDD was observed in the mesangial or para-mesangial areas. The patient's histopathology revealed membranous nephropathy with a solitary IgA deposition. No clinical findings suggested a secondary cause of membranous nephropathy. Combination therapy with corticosteroids and cyclosporine resulted in proteinuria remission. To our knowledge, this is the first reported case of membranous nephropathy with a segmental pattern associated with solitary IgA and galactose-deficient IgA1 deposition. Further case reports and studies are required to elucidate the pathogenesis of membranous nephropathy, which shows these unique histopathological features.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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