摘要
目的:本文旨在探讨临床上发现的一类伴特殊电镜表现的非典型膜性肾病的临床病理特征。方法:回顾性分析解放军南京总医院肾脏科2012年1月至2016年8月间经肾活检光镜表现为肾小球病变轻,电镜下以基膜上皮侧少量或散在的规则圆形致密颗粒沉积为主,诊断为非典型膜性肾病的18例患者,观察其临床、实验室指标及病理特点。结果:(1)女性14例,男性4例,平均年龄为37. 17±15. 22岁,临床主要表现为水肿、不同程度的尿蛋白(4.38±3.78)g/24h,3例患者伴镜下血尿(18～140万/ml,多形性),无肉眼血尿,16例患者尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG酶)升高,8例患者尿视黄醇结合蛋白(RBP)升高,所有患者抗磷脂酶A2受体(PLA_2R)抗体阴性;(2) 10例患者有美白化妆品使用史或染发史,余无明确诱因;(3)肾组织学特点:光镜下肾小球轻度系膜细胞增生和系膜基质增多,6例患者可见肾小管间质急性病变。免疫荧光示IgG或C3沿肾小球毛细血管袢呈颗粒样沉积,IgG亚型以IgG1沉积为主。电镜下均见肾小球基膜上皮侧少量或散在的中高密度电子致密物沉积,致密物呈类圆形或圆形,有不同程度的足突融合;(4)给予泼尼松或联合雷公藤、血管紧张素Ⅱ受体拮抗剂(ARB)等治疗,随访过程中11例患者尿检转阴。结论:临床上发现光镜表现为肾小球病变轻微,结合免疫荧光和电镜特点最后诊断为非典型膜性肾病,大多数有美白化妆品使用史及染发史,临床上预后较好。
Objective：To explore the clinical pathologic features of atypical membranous nephropathy （AMN） with special manifest of electron microscopy. Methodology： Eighteen patients of biopsy-proven AMN with minimal glomerular change by light microscopy and few or scattered circular electron dense deposits on the epithelium of the GBM by electron microscopy were retrospectively evaluated. The clinical manifestation and pathologic features we analyzed. Results：A total of 14 women and 4 man with 12 to 64 years old were enrolled in the study.All patients had edema and proteinuria; 3 had microscopic hematuria; 16 had the high level of urinary N-acetyl-B-D-glucosaminidase to creatinine （NAG） and 8 cases had the high level of urinary retinol-binding protein（ RBP ）.No patients had the antibody against the PLA2R. l0 patients were skinf-lightenting creams users or had the history of hairdye. Light microscopy revealed mildly proliferative mesangial cells and mesangial matrix. Acute tubularinterstitial injury were found in six patients. The immunofluorescence findings showed granular deposits of IgG or C3 along the glomerular capillary wall.IgG1 deposits were observed along the glomerular capillary loops. The electron microscopy showed few or scattered circular electron dense deposits on the epithelium of the GBM. 11 patients reached complete remission in fallow-up after immunosuppressive therapy. Conclusion： Clinicians should improve the realization of atypical membranous nephropathy, which associated with minimal glomerular change by light microscopy and few or scattered circular dense deposits on the epithelium of the GBM by electron microscopy.
作者
范芸
徐峰
张明超
朱晓东
徐效如
芮昊辰
刘志红
曾彩虹
FAN Yun;XU Feng;ZHANG Mingchao;ZHU Xiaodong;XU Xiaoru;RUI Haochen;LIU Zhihong;ZENG Caihong(National Clinical Research Center of Kidney Disease,Jinling Hospital,Nanjing University School of Medicine,Nanjing 21006,China)
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2018年第5期412-417,439,共7页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
江苏省医学重点人才项目(ZDRCA2016098)
江苏省临床医学研究中心项目(YXZX2016003)
关键词
司非典型膜性肾病
临床病理
预后
atypical membranous nephropathy
clinicopathological
prognosis
