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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。结累: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徽管状结构。病情发展快,预后差,多数肾功能持续恶化。
Objective: To investigate the etiology, clinicopathological features, diagnosis, differential diagnosis, treatment and prognosis of immunostimuliform-like glomerulopathy. METHODS: Two rare cases of immunostimulatory glomerulopathy were reported and reviewed in combination with the literature. Tired: 2 cases were young men, clinical manifestations of proteinuria, nephrotic syndrome and microscopic hematuria, 1 case of hypertension, 1 case of renal insufficiency. Two pathological types under light microscope were proliferative glomerulonephritis, IgG, IgM, C3 and / or IgA immune complex deposition in the mesangial and basement membrane, but Congo red staining was negative. Under the electron microscope, the mesangial area and the basilar membrane expanded subcutaneously, and the electron-dense material was deposited. The hollow microtubule-like structure with a diameter of 30-50 nm and no branch was seen. Conclusion: ITG has the typical morphological features, and the diagnosis depends on the characteristic electron microscope tubular structure. Rapid development of the disease, the prognosis is poor, most of the renal function continued to deteriorate.