Membranoproliferative Glomerulonephritis with Striated Ultrastructural Deposits with Significantly Elevated Fibrinogen and Fibronectin on Mass Spectrometry Analysis: A Case Report and Literature Review.

IF 1.8 4区 医学 Q2 UROLOGY & NEPHROLOGY
Nephron Pub Date : 2025-01-01 Epub Date: 2025-02-13 DOI:10.1159/000544709
Manna Ishida, Shinya Yamamoto, Yohei Iwashige, Shuma Miyazawa, Hirosuke Nakata, Koichi Seta, Kensei Yahata, Sachiko Minamiguchi, Yoko Endo, Akiko Mii, Akira Shimizu, Motoko Yanagita
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引用次数: 0

Abstract

Glomerular diseases with organized deposits can be classified into various etiologies. A diagnostic algorithm based on clinical and pathological findings has been proposed. However, some cases cannot be diagnosed using existing algorithms. Here, we report the case of a 77-year-old man diagnosed with membranoproliferative glomerulonephritis (MPGN) with striated ultrastructural deposits, micro-filament-like substructures with straight bands arranged in parallel in the subendothelial space by two sequential renal biopsies. His examinations and clinical findings were incompatible with known glomerular diseases with organized deposits. Dialysis was initiated 10 months after the second biopsy procedure. Furthermore, we report the first mass spectrometry analysis of laser micro-dissected glomeruli with striated ultrastructural deposits, which revealed significant levels of fibrinogen and fibronectin. Immunostaining was positive for fibrinogen, fibrin, and fibronectin in the subendothelial space. These findings suggest that the deposits were composed of a fibrin-fibronectin complex and that accumulation of these fibrin-fibronectin complexes possibly induced endothelial injury, leading to MPGN. We also reviewed the literature on the clinical and pathological characteristics of the four cases with striated ultrastructural deposits. Our investigation showed that all patients had the MPGN pattern and striated ultrastructural deposits in the subendothelial space, and all underwent hemodialysis within 3 years of renal biopsy. Clinicians should be aware of the findings of glomerulonephritis with striated ultrastructural deposits since this disease may be a new entity and has a poor prognosis.

膜增生性肾小球肾炎伴条纹状超微结构沉积,质谱分析纤维蛋白原和纤维连接蛋白显著升高:1例报告并文献复习。
有组织沉积的肾小球疾病可分为多种病因。提出了一种基于临床和病理结果的诊断算法。然而,有些病例无法使用现有算法进行诊断。在此,我们报告一位77岁的男性患者,通过两次连续的肾脏活检,诊断为膜增生性肾小球肾炎(MPGN),其超微结构呈条纹状沉积,微丝样亚结构在内皮下空间平行排列。他的检查和临床表现与已知的有组织沉积的肾小球疾病不相符。第二次活检术后10个月开始透析。此外,我们首次报道了激光显微解剖肾小球的质谱分析,其中有条纹状超微结构沉积物,显示纤维蛋白原和纤维连接蛋白的显著水平。免疫染色显示内皮下间隙纤维蛋白原、纤维蛋白和纤维连接蛋白阳性。这些发现表明,沉积物由纤维蛋白-纤维连接蛋白复合物组成,这些纤维蛋白-纤维连接蛋白复合物的积累可能诱导内皮损伤,导致MPGN。我们还回顾了4例条纹状超微结构沉积的临床和病理特征。我们的研究显示,所有患者都有MPGN模式和内皮下空间的条纹状超微结构沉积物,并且所有患者在肾活检后3年内进行了血液透析。临床医生应注意具有条纹状超微结构沉积物的肾小球肾炎的表现,因为这种疾病可能是一种新的疾病,预后较差。。
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来源期刊
Nephron
Nephron UROLOGY & NEPHROLOGY-
CiteScore
5.00
自引率
0.00%
发文量
80
期刊介绍: ''Nephron'' comprises three sections, which are each under the editorship of internationally recognized leaders and served by specialized Associate Editors. Apart from high-quality original research, ''Nephron'' publishes invited reviews/minireviews on up-to-date topics. Papers undergo an innovative and transparent peer review process encompassing a Presentation Report which assesses and summarizes the presentation of the paper in an unbiased and standardized way.
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