【摘要】 目的 通过对一例纤维样肾小球病(fibrillary glomerulopathy,FGP)患者的临床和病理资料进行分析,探讨其发病机制、临床表现、诊断、治疗及预后。方法 回顾性分析一例纤维样肾小球病患者的临床表现,并将患者肾穿刺组织进行光镜、免疫荧光、电镜检查,通过以上数据,并结合相关文献系统复习纤维样肾小球病的临床特征。结果 通过其临床表现,肾组织病理学检查,特别是电镜诊断本例为纤维样肾小球病。临床表现多为大量蛋白尿、镜下血尿和高血压等。电镜的检查结果 是诊断纤维样肾小球病的主要依据。其主要特点是纤维样物质呈弥漫性或团块状分布于肾小球系膜区和(或)肾小球基底膜,排列紊乱,无规律,纤维僵直,一般无分支。该病预后差,大约50%的患者2~4年内进展为终末期肾脏疾病。结论 电镜结合刚果红染色是诊断纤维样肾小球病的重要方法,该病发病率较低,病理生理机制仍不清楚,预后不佳,目前缺乏较为有效的治疗措施。更多还原

【Abstract】 Objective To discuss the mechanism,clinical feature,diagnosis,treatment and prognosis of fibrillary glomerulopathy(FGP) by analyzing a patient with FGP.Methods The clinical manifestations of a 21-year old patient diagnosed with FGP were studied.The renopuncture biopsy tissues of this patient were observed by light microscope with staining,fluorescent staining and electron microscope.According to the data we studied above,we summarized the characteristics of FGP,and reviewed the related literatures.Results The diagnosis of FGP depended on the following conditions,clinical manifestations,renal histopathological examination,and above all electron microscope.Most patients presented with nephrotic proteinuria,hematuria and hypertension.Electron miacroscope was the main diagnosis basis for FGP,which showed that glomerular mesangial area and glomerular basement membrane were infiltrated by randomly arranged,stiff,nonbranching fibrils.Owing to poor diagnosis,most patients developed to end-stage renal disease within a few years.Conclusions Electron microscopy combined with Congo red staining plays an important role in the diagnosis of FGP.FGP possesses such features as low incidence of the disease,the ambiguity of the pathophysiological mechanisms and poor prognosis,which makes the disease lack of effective treatment measures.更多还原