表现为肾病综合征的成人节段性膜性肾病1例报告及文献复习

姜毅

doi:10.12182/20230560308

表现为肾病综合征的成人节段性膜性肾病1例报告及文献复习

doi: 10.12182/20230560308

姜毅

温州医科大学附属衢州医院（衢州市人民医院）肾内科（衢州 324000）

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- 被引次数: 0
出版历程
- 收稿日期: 2022-07-06
- 修回日期: 2023-05-06
- 网络出版日期: 2023-05-20
- 刊出日期: 2023-05-20

Adult Segmental Membranous Nephropathy Presenting As Nephrotic Syndrome: A Rare Case Report and Literature Review

JIANG Yi

Department of Nephrology, Quzhou Affiliated Hospital of Wenzhou Medical University, Quzhou People's Hospital, Quzhou 324000, China

摘要

摘要: 节段性膜性肾病（segmental membranous nephropathy, sMN）是一类罕见的肾脏病理类型，表现为IgG沿肾小球上皮下呈节段性颗粒样沉积。该病的临床表现多样，可表现为蛋白尿、肾病综合征或肾功能不全，也可继发于狼疮、肿瘤等多种疾病。本文报告了1例病理明确诊断为sMN而临床表现为肾病综合征的成人病例，并总结了国内外报道的总计87例成人sMN患者临床特点：成人sMN患者可单独出现或与其他肾小球或肾小管间质疾病同时出现，经常叠加在另一种主要疾病过程中，临床需首先排除继发性肾损害。
- 节段性膜性肾病 /
- 膜性肾病 /
- 1型神经表皮生长因子样蛋白
Abstract: Segmental membranous nephropathy (sMN) is a rare pathological variant of membranous nephropathy (MN). Patients with sMN present with segmental subepithelial granular deposits of IgG in glomeruli. Patients with sMN have diverse clinical manifestations, including proteinuria, nephrotic syndrome, and renal insufficiency. Patients may have sMN secondary to a variety of diseases, such as lupus and tumors. We, herein, reported the case of an adult whose diagnosis of sMN had been confirmed by pathology analysis, but who had clinical manifestations of nephrotic syndrome. In addition, we reviewed the clinical characteristics of 87 adult sMN cases reported in China and other countries and found that sMN in adults might occur separately or the patients may present with other glomerular or tubulointerstitial diseases in conjunction, often overlaying with another major disease process, which suggests that clincians must first rule out secondary renal injury.
- Segmental membranous nephropathy /
- Membranous nephropathy /
- Neural epidermal growth factor-like 1 protein

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图 1 患者肾脏病理光镜下可见基底膜节段性增厚（H&E ×400）

Figure 1. Segmental thickening of the basement membrane of the patient's kidney tissue can be seen under the light microscope (H&E ×400)

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图 2 患者肾脏病理免疫荧光示IgG2（A）、IgG3（B）节段性沿毛细血管襻呈细颗粒状沉积（×400）

Figure 2. Immunofluorescence of the patient's kidney tissue shows segmental deposition of IgG2 (A) and IgG3 (B) along the capillary loop in the form of fine granules (×400)

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图 3 患者肾脏病理电镜示肾小球基底膜节段性增厚（箭头a），节段性上皮下电子致密物沉积（箭头b）

Figure 3. Electron microscopy of the patient's kidney tissue shows segmental thickening of the glomerular basement membrane (indicated by the arrow a) and segmental deposition of subepithelial electron dense matter (arrow b)

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参考文献(17)

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