C3肾小球病临床表现和病理改变的异质性·综述·.PDF

　 军 杂 2014年12月1 日　第39卷　第12期 998 解放 医学 志　 ·综　　述· C3 肾小球病临床表现和病理改变的异质性 王金泉 [摘要]　C3 肾小球病是由于补体旁路途径异常活化介导的疾病。由于致病机制的多样性和多重性，C3 肾小球病的 临床表现与病理改变具有异质性，临床可以表现为尿检异常、高血压、肉眼血尿、肾病综合征、肾炎综合征、肾功能 不全等，病理改变可以表现为膜增生性肾小球肾炎、系膜增生、新月体形成、局灶节段性坏死、弥漫增生和渗出病变 等，预后也不均一。本文对C3肾小球病的临床表现及病理特征进行综述。 [关键词]　C3肾小球病；临床表现；病理学，临床 [中图分类号]　R692.6　　　　[文献标志码]　A　　　　[文章编号]　0577-7402(2014)12-0998-06 [DOI]　10.11855/j.issn.0577-7402.2014.12.15 Heterogeneity of the clinical manifestations and pathology features in C3 glomerulopathy WANG Ji n-quan National Clinical Research Center of Kidney Diseases, J inling Hospital, Nanjing University School of Medicine, Nanjing 210002, China [Abstract]　C3 glomerulopathy is a kind of glomerular diseases mediated by abnormal activation of alternative complement pathway. As diversity and multiplicity of pathogenic mechanism, heterogeneity exists in the clinical manifestation and pathological features of C3 glomerulopathy. The clinical manifestation of the disease may be shown as abnormality in urine, hypertension, hematuria, nephrotic syndrome, nephritic syndrome, renal insufficiency, etc. Membranoproliferative glomerulonephritis, mesangial proliferation, crescent formation, focal segmental necrosis, diffuse hyperplasia and exudative lesions, etc may be found in renal biopsies. Also, the prognosis of C3 glomerulopathy is not uniform. The clinical manifestations and pathological features of C3 glomerulopathy were reviewed in the present paper. [Key words]　C3 glomerulopathy; clinical manifestation; pathology, clinical C3 肾小球病(C3 glomerulopathy ，C3G)是由 白血症、副球蛋白血症、自身免疫性疾病和感染 补体旁路途径(alternative pathways of complement 的MPGN定义为继发性MPGN ，其余所谓的原发性 [1] activation ，AP) 异常活化介导的一组疾病 。目前 MPGN依据电子致密物在肾小球沉积的部位分为3 已明确由于不同个体致病机制不同，C3G 的临床表 型。①Ⅰ型：电子致密物在肾小球系膜区及GBM 现、病理改变和预后在不同亚类的C3G之间、不同