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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 manifestations, laboratory tests and pathological features of renal biopsy in patients with fibronectin glomerulopathy (GFND) to improve the diagnosis and differential diagnosis of this disease. Methods: The clinical data of 10 patients with GFND confirmed by renal biopsy were collected. Results: (1) The general situation: There were 6 males and 4 females in 10 cases, ranging in age from 19 to 46 years, of whom 6 were <30 years old; only 1 case was traced back to a clear family history of renal disease; 2) Clinical features Nephrotic proteinuria is the most common (80%), no gross hematocele, microscopic hematuria incidence of only 40%, 4 cases of early existence of hypertension, all patients have abnormal lipid metabolism, impaired renal function 5 cases (50%); the group of patients common tubulointerstitial damage laboratory evidence; (3) pathological features: all patients increased glomerular volume, 6 cases of histological changes similar to membranoproliferative nephritis I Type (lobulated), 5 cases of mesangial lysis, all patients with Masson trichrome staining were seen glomerular capillary perirenal subretinal erythrocyte sedimentation, mesangial area 7 cases of red blood deposition ; Immunofluorescence staining of 10 patients with immunoglobulin and complement were positive, Fibrin-positive in 6 cases, fibronectin staining of glomerulus-positive 100%; electron microscopy were found in glomerular capillary loop and mesangial see Inhomogeneous, lipid-containing electron-dense materials confirmed by immunoelectron microscopy of these electron-dense connexin. Conclusion: The diagnosis of GFND depends on the renal biopsy pathology.