纤维连接蛋白肾小球病——临床及病理分析  

Fibronectin glomerulopathy:clinical and morphological features

作　　者：陈惠萍[1] 曾彩虹[1] 朱小东[1] 朱茂艳[1] 张明超[1] 梁少珊[1] 黄倩[1] 徐峰[1] 郑春霞[1] 刘志红[1] 

机构地区：[1]南京军区南京总医院全军肾脏病研究所,南京210002

出　　处：《肾脏病与透析肾移植杂志》2011年第5期425-431,共7页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：目的:分析纤维连接蛋白肾小球病(fibronectin glomerulopathy,GFND)患者的临床表现、实验室检查和肾活检病理特征,旨在提高对此病的诊断及鉴别诊断。方法:收集10例经肾活检明确GFND患者的临床病理资料。结果:(1)一般情况:10例患者中男性6例,女性4例,年龄19～46岁,其中<30岁者6例;仅1例追溯到明确肾脏疾病家族史;(2)临床特点:肾病范围的蛋白尿最常见(80%),无肉眼血尿发作者,镜下血尿的发生率仅40%,4例病初即存在高血压,全部患者均有脂代谢异常,肾功能受损者5例(50%);本组患者常见肾小管间质受损的实验室检查证据;(3)病理特征:全部患者肾小球体积增大,6例组织学改变类似膜增生性肾炎I型(分叶状),5例见肾小球系膜溶解,全部患者Masson三色染色均见肾小球毛细血管外周袢内皮下嗜复红物沉积,7例系膜区嗜复红物沉积;10例患者免疫荧光染色免疫球蛋白和补体均阳性,Fibrin阳性者6例,纤维连接蛋白染色肾小球阳性者100%;电镜观察均见肾小球毛细血管袢内皮下及系膜区见不均质、含脂质的电子致密物,经免疫电镜证实这些电子致密物为纤维连接蛋白。结论:GFND的诊断需依靠肾活检病理。Objective： To analyze the clinical and histopathological features of patients with fibronectin glomerulopathy （GFND）. Methodology：Ten patients with renal biopsy-proven GFND were retrospectively studied. The clinical and laboratory tests, and renal biopsy data （histopathological, immunofluorescence and electron microscopy） were collected. Results：They were 6 males and 4 females with an average age of （28.8±5.8） years old （ranged from 16 to 46）. Only one patient had the family history of renal disease. The initial manifestations were proteinuria and nephritic syndrome in 80%, and micro-hematuria in 40% patients. None of them showed gross hematuria. Hypertension was found in 4 and elevated serum creatinine in 5 cases at onset of the disease. All patients had abnormal lipid metabolism, and most of patients had increased urine enzymes. The light microscopy examination revealed marked hypertrophy with numerous periodic acid-Schiff （PAS） and Masson-trichrom-positive deposits in the subendothelial space of all cases and in the mesangium of 7 cases. Six patients （ 60.0% ） presented membrane proliferative glomerulonephritis （ MPGN ） with endothelial and mesangial proliferation, and with mesangiolysis in 5 patients. The immunofluorescence determination showed all positive both in immunoglobulins and complements. The fibronectin was positively stained in the mesangium and capillary loop. The electron microscopy revealed numerous heterogeneous deposits included lipids in the mesangium and subendothelial space. Those deposits were fibronectin proved by the immuno-electron microscopy. Conclusion： The diagnosis of GFND must be depend on renal biopsy.

关 键 词：纤维连接蛋白肾小球病 肾活检 蛋白尿 高血压 

分 类 号：R692.6[医药卫生—泌尿科学]