摘要
本文报道1例老年男性患者,表现为肾病综合征伴血清肌酐升高,血清免疫固定电泳检出IgM-λ型单克隆条带。肾活检病理示肾小球系膜增生性病变,刚果红染色阴性。石蜡切片免疫荧光染色示IgG及各亚型均阴性;C3阴性、IgM+。电镜下见肾小球系膜区及肾小球基膜内大量排列紊乱纤维样物质沉积,直径10~30 nm。肾组织免疫组化染色DNAJ热休克蛋白家族成员B9阳性,诊断为纤维样肾小球病伴单克隆丙种球蛋白血症。治疗上予以控制血压、保护肾功能等慢性肾脏病综合管理。
The patient was a 63-year-old male.The clinical features included nephrotic syndrome with elevated serum creatinine and IgM lambda monoclonal band.Renal biopsy revealed mesangial proliferative glomerulonephritis with negative Congo red staining.Immunofluorescence on paraffin-embedded sections of renal tissues was negative for IgG subtype,C3(-),IgM(+).Electron microscopy revealed abundant fibrillary deposits with a randomly arranged pattern in the mesangial area and glomerular basement membrane,measuring approximately 10-30 nm in diameter.Immunohistochemical staining for DNAJ heat shock protein family member B9(DNAJB9)was positive,confirming the diagnosis of fibrillary glomerulonephritis associated with monoclonal gammopathy(IgM-LAMBDA).Management focused on blood pressure control and comprehensive care of chronic kidney disease aimed at preserving renal function.
作者
张丽
李佳莹
董伟
杨艳
李锐钊
梁馨苓
ZHANG Li;LI Jiaying;DONG Wei;YANG Yan;LI Ruizhao;LIANG Xinling(Department of Nephrology,Guangdong Provincial People's Hospital,Guangdong Academy of Medical Sciences,Southern Medical University,Guangzhou 510080,China)
出处
《肾脏病与透析肾移植杂志》
2025年第4期391-394,共4页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
西藏自治区自然科学基金组团式医学援藏项目[XZ2023ZR-ZY63(Z)]
国家自然科学基金(81970625)
广东省自然科学基金面上项目(2022A1515012282)。
