非淀粉样变原纤维性肾小球病超微结构观察  

Observations on the ultrastructure of non-amyloidotic fibrillary glomerulopathy

作　　者：邹万忠[1] 王素霞[1] 章友康[1] 王海燕[1] 

机构地区：[1]北京医科大学病理学系,北京医科大学肾病研究所

出　　处：《中华病理学杂志》1995年第3期146-148,共3页Chinese Journal of Pathology

摘　　要：报道５例成年男性非淀粉样变原纤维性肾小球病。无系统性疾病，临床表现为大量蛋白尿、镜下血尿。其中２例已发展为肾功能不全。病理类型为膜增生性、膜性及系膜增生性肾小球肾炎。刚果红染色均为阴性。免疫荧光检查及超微结构与淀粉样变性肾小球病相似，但纤维直径较粗，约为淀粉样纤维的２倍。结果提示，本病的发生可能为异常球蛋白沉积所致。ive patients with glomerulopathy and glomerular fibrillary protien deposits similar to those of amy-loid but lacking the Congo red tinctorial affinity characterizing amyloid were studied. Clinically,thesepatients had severe proteinuria and microscopic hematuria,in addition,2 patients had hypertension andrenal function impairment. 5 renal biopsies were evaluated by light microscopy ( LM ),immunofluo-rescence(IF),immunoperoxidase(IP ) and electron microscopy(EM).LM :mesangioproliferative,membranous(late stage)and membranoproliferative(late stage)patterns were seen in 2,1 and 2 pa-tients respectively,IF&IP:granular deposits,mainly of IgG, C_3 and K,λlight chains were found in allcases, either in mesangium and/or capillary walls。 EM :randomly distributed fibrils with a diameter ap-proximately 2Inm (the diameter of amyloid fibrils is about 9.5nm)were found in gIomerular basementmembrane and/or mesangium。 The pathologic findings suggested a new clinicopathological entity differ-entiated from amyloidosis.The relatively homogeneous nature of the immunoglobulin in the immune de-pOsits is the basis for the fibril forn1ation.

关 键 词：肾小球疾病 超微结构 非淀粉样变性 电子显微镜 

分 类 号：R692.602[医药卫生—泌尿科学]