摘要
目的:探讨免疫触须样肾小球病的病因、临床病理特征、诊断、鉴别诊断、治疗及预后。方法:报道2例罕见的免疫触须样肾小球病,并结合文献进行复习。结果:2例均为中青年男性,临床表现蛋白尿、肾病综合征及镜下血尿。1例伴有高血压,1例肾功能不全。光镜下2例病理类型均为膜增殖性肾小球肾炎,肾小球系膜区和基底膜有IgG、IgM、C3和(或)IgA免疫复合物沉积,但刚果红染色阴性。电镜下系膜区和基底膜内皮下扩大,电子致密物沉积,其中可见直径30~50nm、无分支的中空微管状结构。结论:ITG具有典型的形态学特征,确诊需依赖电镜特征性微管状结构。病情发展快,预后荠,多数肾功能持续恶化。
Objective: To study the etiology, clinicopathological features, diagnosis and differential diagnosis, therapy and prognosis of immunotactoid glomerulopathy (ITG). Methods:To report two cases of ITG and review the literature. Results :Clinically, the patients were young middle-aged men with proteinuria, nephrotic syndrome and microscopic hematuria, one had hypertension and another had renal insufficiency. Morph-ologically, renal biopsy specimen showed membranoproliferative glomerulonephritis associated with deposition of material that was positive for IgG, IgM, C3,and/or IgA but was not stained by Congo red. Ultrastructurally, the mesangial and subendothelial areas were expanded because of the electron-dense deposits, which were represented by nonbranching hollow microtubule structures (diameter: 30.0-50.0 nm). Conclusions :The diagnosis of ITG is always based on biopsy of the kidney which revealed the characteristic of microtubule structures composed of immune reactants on electron microscopy. State of an illness develops lastly and prognosis is bad. Mostly patients' renal function is to get worse.
出处
《中国误诊学杂志》
CAS
2006年第17期3286-3289,共4页
Chinese Journal of Misdiagnostics
关键词
肾疾病/病理学
肾疾病/诊断
Kidney diseases/pathology
Kidney diseases/diagnosis