Ⅲ型胶原肾小球病的形态学观察  

Collagen type Ⅲ glomerulopathy: a morphologic study

作　　者：李玲[1] 邹万忠[1] 王素霞[1] 王盛兰[1] 王薇[1] 韩志惠[1] 杜娟[1] 薄莉[2] 

机构地区：[1]北京大学医学部病理学系,100083 [2]贵阳医学院附属医院病理科

出　　处：《中华病理学杂志》2005年第7期385-388,共4页Chinese Journal of Pathology

摘　　要：目的了解Ⅲ型胶原肾小球病的形态学改变,并对Ⅲ型胶原可能的细胞来源进行初步探讨。方法对3例肾活检组织进行光镜、免疫荧光、电镜和Ⅰ、Ⅲ、Ⅳ型胶原及α平滑肌肌动蛋白(α-SMA)的免疫组织化学染色(SP法)观察。结果2例患者临床表现为肾病综合征,其中1例伴高血压,第3例表现为肾功能不全和肾性高血压。3例均无肾病家族史。光镜检查可见肾小球基膜内和系膜区弥漫性过碘酸-希夫反应阳性物质沉积,系膜细胞无明显增生。电镜检查在基膜内疏松层和系膜区可见大量胶原纤维沉积,系膜细胞胞膜下平行排列的束状微丝明显增加。免疫组织化学显示这些胶原纤维为Ⅲ型胶原,Ⅰ型和Ⅳ型胶原阴性,同时系膜区多数系膜细胞α-SMA阳性。结论Ⅲ型胶原肾小球病光镜、电镜及免疫组织化学上都有其特殊的病理改变。肾小球内激活的系膜细胞可能是Ⅲ型胶原的来源。Objective To study the morphologic changes of collagen type Ⅲ glomerulopathy and to investigate the possible cellular origin for collagen Ⅲ production. Methods Light microscopy, immunofluorescent staining, immunohistochemistry (for collagen I, Ⅲ and Ⅳ and alpha-SMA) and electron microscopy studies on 3 renal biopsy cases of collagen type Ⅲ glomerulopathy were performed. Results Two cases presented with nephrotic syndrome, one of which was associated with systemic hypertension. The third case showed renal impairment and renal hypertension. None had any known family history of renal diseases. Light microscopy showed diffuse thickened glomerular basement membrane and expanded mesangium with deposition of weakly PAS-positive homogeneous material not associated with mesangial cell proliferation. Electron microscopy revealed massive collagen fiber deposits in the subendothelial spaces and mesangium. The mesangial cells also contained bundles of microfilaments in the subplasmalemmal regions. Immunohistochemically, the diffuse positivity for type Ⅲ collagen corresponded to the homogeneous material seen under light microscopy. The staining for type Ⅰ and Ⅳ collagens was negative. Alpha-SMA was expressed in many mesangial cells. Conclusions The diagnosis of collagen type Ⅲ glomerulopathy can be made on the basis of detailed morphologic examination and ancillary investigations. It is possible that activated mesangial cells may be the cellular origin of collagen Ⅲ.

关 键 词：Ⅲ型胶原 肾小球病 形态学观察 免疫组织化学染色 平滑肌肌动蛋白 系膜细胞 Ⅳ型胶原 胶原纤维 形态学改变 肾活检组织 肾病综合征 肾性高血压 肾功能不全 肾小球基膜 α-SMA 系膜区 初步探讨 细胞来源 免疫荧光 临床表现 

分 类 号：R692.6[医药卫生—泌尿科学]