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局灶节段性肾小球硬化中的膜内微球结构

Intramembranous microspherical structures in focal segmental glomerulosclerosis.

作者信息

Inoue Yoshimoto, Kanda Yoko, Kinoshita Chiharu, Kanda Chiaki, Joh Kensuke

机构信息

Kyoto Min-iren Chuo Hospital, Kyoto, Japan.

出版信息

Clin Exp Nephrol. 2008 Dec;12(6):504-8. doi: 10.1007/s10157-008-0101-2. Epub 2008 Nov 13.

DOI:10.1007/s10157-008-0101-2
PMID:19002748
Abstract

A 45-year-old male had proteinuria for 3 years. For persistent proteinuria over 2 g/day, he underwent renal biopsy. Light microscopy revealed focal segmental glomerulosclerosis together with diffuse capillary wall thickening. Periodic acid methenamine silver (PAM) staining showed a prominent bubbly appearance without spike formation could be found in almost all capillary walls. Electron microscopy revealed many microspheres measuring 50-70 nm in diameter distributed in diffuse and global fashion together with the thickened glomerular basement membrane. A few cytoplasmic processes of the podocytes showed infolding to the GBM. The patient exhibited no symptoms and no physical and serological findings suggesting autoimmune disease, such as systemic lupus erythematosus or Sjögren's syndrome. Therefore, the present case is important, because the peculiar microstructure in the GBM was noted in focal segmental glomerulosclerosis, which has never been reported in the literature.

摘要

一名45岁男性蛋白尿3年。因持续蛋白尿超过2 g/天,他接受了肾活检。光镜检查显示局灶节段性肾小球硬化伴弥漫性毛细血管壁增厚。高碘酸甲苯胺银(PAM)染色显示几乎所有毛细血管壁均可见明显的泡沫状外观,无钉突形成。电镜检查显示许多直径为50 - 70 nm的微球以弥漫性和全球性方式分布,同时伴有肾小球基底膜增厚。足细胞的一些胞质突起向肾小球基底膜折叠。该患者无任何症状,也无提示自身免疫性疾病(如系统性红斑狼疮或干燥综合征)的体格检查和血清学发现。因此,本病例很重要,因为在局灶节段性肾小球硬化中发现了肾小球基底膜中独特的微观结构,这在文献中从未有过报道。

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引用本文的文献

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A case of podocytic infolding glomerulopathy with focal segmental glomerulosclerosis.一例伴局灶节段性肾小球硬化的足细胞内褶性肾小球病
Case Rep Nephrol Urol. 2013 Aug 8;3(2):110-6. doi: 10.1159/000354591. eCollection 2013.
2
Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan.提出足细胞内褶性肾小球病作为一种新的疾病实体:对日本全国性研究中25例病例的回顾
Clin Exp Nephrol. 2008 Dec;12(6):421-31. doi: 10.1007/s10157-008-0104-z. Epub 2008 Nov 18.

本文引用的文献

1
Membranous glomerulopathy with spherules: an uncommon variant with obscure pathogenesis.伴有小球的膜性肾小球病:一种发病机制不明的罕见变异型。
Am J Kidney Dis. 2006 Jun;47(6):983-92. doi: 10.1053/j.ajkd.2006.03.004.
2
Clinical and morphological prognostic factors in membranous nephropathy: significance of focal segmental glomerulosclerosis.膜性肾病的临床和形态学预后因素:局灶节段性肾小球硬化的意义
Am J Kidney Dis. 2003 Jan;41(1):38-48. doi: 10.1053/ajkd.2003.50015.
3
A case of lupus nephritis with alteration of the glomerular basement membrane associated with Takayasu's arteritis.
一例狼疮性肾炎合并肾小球基底膜改变并与高安动脉炎相关。
Clin Nephrol. 2002 Aug;58(2):161-5. doi: 10.5414/cnp58161.
4
Extracellular clusters of spherical microparticles in glomeruli in human renal glomerular diseases.人类肾小球疾病中肾小球内球形微粒的细胞外簇。
Lab Invest. 1973 Apr;28(4):415-25.
5
Intramembranous fine deposit disease associated with collagen disorders: a new morphological entity?与胶原紊乱相关的膜内微小沉积物病:一种新的形态学实体?
Virchows Arch A Pathol Anat Histopathol. 1992;420(5):447-51. doi: 10.1007/BF01600517.