目的 为加强对胶原Ⅲ肾小球病的认识，首次报道国内两例胶原Ⅲ肾小球病。方法 对肾活检组织进行光镜、电镜、免疫荧光及免疫组化观察。结果 两例均表现肾病综合征、贫血和高血压，其中1例有轻度肾功能减退，无肾脏病家族史，临床资料除外指甲髌骨综合征。肾脏病理光镜表现类似非典型膜性肾病，电镜和免疫组化检查证实大量Ⅲ型胶原纤维在肾小球内沉积。结论 经病理学确诊为胶原Ⅲ肾小球病。同时，就本病的临床表现、病理特征、诊断、治疗和发病机理等方面对文献进行复习。

    Collagen Ⅲ glomerulopathy: two cases report Zhou Fude, Zou Wanzhong, Huang Zhaoxing, et al. Institute of Nephrology, The First Hospital of Beijing Medical University, Beijing 100034

    Objective Two cases of collagen Ⅲ glomerulopathy are reported first time in China to recognize this kind of glomerulopathy. Methods Light, electron microscopy, immunofluorescence and immunohistochemistry were performed in renal tissue from biopsy. Results Both patients presented with nephrotic syndrome, anemia and hypertension, and one of them with mild impaired renal function. A family history of these two cases had not been found and the similar glomerulopathy such as nail-petella syndrome etc, had been excluded. Diagnostic renal biopsy by light microscopy showed a picture similar to atypical membranous nephropathy with irregular thickened GBM and without obvious cell proliferation. By electron microscopy, it was found that in laminal rara interna of GBM and mesangium there were abundant collagen fibers which were identified as collagen type Ⅲ by immunohistochemistry method. Conclusion Both cases are diagnosed as collagen Ⅲ glomerulopathy by pathology. A brief review of the literature was made, including its clinical manifestations, pathologic features, diagnosis, treatment and pathogenesis.

     ......