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致密物沉积病并非膜增生性肾小球肾炎。

Dense deposit disease is not a membranoproliferative glomerulonephritis.

作者信息

Walker Patrick D, Ferrario Franco, Joh Kensuke, Bonsib Stephen M

机构信息

Nephropathology Associates, Little Rock, AR 72211, USA.

出版信息

Mod Pathol. 2007 Jun;20(6):605-16. doi: 10.1038/modpathol.3800773. Epub 2007 Mar 30.

DOI:10.1038/modpathol.3800773
PMID:17396142
Abstract

Dense deposit disease (first reported in 1962) was classified as subtype II of membranoproliferative glomerulonephritis in the early 1970s. Over the last 30 years, marked differences in etiology and pathogenesis between type I membranoproliferative glomerulonephritis and dense deposit disease have become apparent. The sporadic observation that dense deposit disease can be seen with markedly different light microscopy appearances prompted this study. The goal was to examine a large number of renal biopsies from around the world to characterize the histopathologic features of dense deposit disease. Eighty-one cases of dense deposit disease were received from centers across North America, Europe and Japan. Biopsy reports, light microscopy materials and electron photomicrographs were reviewed and histopathologic features scored. Sixty-nine cases were acceptable for review. Five patterns were seen: (1) membranoproliferative n=17; (2) mesangial proliferative n=30; (3) crescentic n=12; (4) acute proliferative and exudative n=8 and (5) unclassified n=2. The age range was 3-67 years, with 74% in the range of 3-20 years; 15% 21-30 years and 11% over 30 years. Males accounted for 54% and females 46%. All patients with either crescentic dense deposit disease or acute proliferative dense deposit disease were between the ages of 3 and 18 years. The essential diagnostic feature of dense deposit disease is not the membranoproliferative pattern but the presence of electron dense transformation of the glomerular basement membranes. Based upon this study and the extensive data developed over the past 30 years, dense deposit disease is clinically distinct from membranoproliferative glomerulonephritis and is morphologically heterogeneous with only a minority of cases having a membranoproliferative pattern. Therefore, dense deposit disease should no longer be regarded as a subtype of membranoproliferative glomerulonephritis.

摘要

致密物沉积病(首次报道于1962年)在20世纪70年代初被归类为膜增生性肾小球肾炎的II型。在过去30年中,I型膜增生性肾小球肾炎和致密物沉积病在病因和发病机制上的显著差异已变得明显。致密物沉积病在光镜下表现明显不同这一偶发观察结果促使了本研究。目的是检查来自世界各地的大量肾活检样本,以明确致密物沉积病的组织病理学特征。从北美、欧洲和日本的各个中心收集了81例致密物沉积病病例。对活检报告、光镜材料和电子显微镜照片进行了审查,并对组织病理学特征进行了评分。69例病例可接受审查。观察到五种模式:(1)膜增生性,n = 17;(2)系膜增生性,n = 30;(3)新月体性,n = 12;(4)急性增生性和渗出性,n = 8;(5)未分类,n = 2。年龄范围为3至67岁,其中74%在3至20岁之间;15%在21至30岁之间,11%超过30岁。男性占54%,女性占46%。所有新月体性致密物沉积病或急性增生性致密物沉积病患者年龄均在3至18岁之间。致密物沉积病的基本诊断特征不是膜增生性模式,而是肾小球基底膜电子致密物改变的存在。基于本研究以及过去30年积累的大量数据,致密物沉积病在临床上与膜增生性肾小球肾炎不同,并且在形态学上具有异质性,只有少数病例具有膜增生性模式。因此,致密物沉积病不应再被视为膜增生性肾小球肾炎的一个亚型。

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