原发性肾小球肾炎.ppt

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GN),机制,：,体内生成抗,GBM,抗体,/,免疫复合物沉积导致,的过于强烈的炎症反应,/,坏死性血管炎,/,特发,性,/,毛细血管壁严重损伤,LM,：,弥漫性小球毛细血管壁断裂,，,血液流入肾,小囊，凝固,，,巨噬细胞浸润和肾小囊上皮,细胞增生,新月体形成,IM,：,IgG,和,C3,沿,GBM,线状沉积,/IgG,和,C3,颗粒,或团块状沉积,/,阴性,EM,：,GBM,断裂,纤维素沉积,/,电子致密物沉积,临床,：,青壮年发病,急进性肾炎综合征,新月体性肾小球肾炎，弥漫性新月体形成,(C),。,新月体性肾小球肾炎，,GBM,和肾小囊基底膜均断裂，新月体形成,。,新月体性肾小球肾炎，纤维素沉积于肾小囊,(F),。,新月体性肾小球肾炎，肾小囊内大量纤维素沉积,(F),。,新月体性肾小球肾炎，细胞性新月体形成,(C),。,新月体性肾小球肾炎，新月体内可见溶菌酶阳性的单核巨噬细胞,(),。,新月体性肾小球肾炎，细胞纤维性新月体形成,。,新月体性肾小球肾炎，纤维性新月体形成，伴再沟通现象,(),。,I,型新月体性肾小球肾炎，,IgG,细线状沿,GBM,沉积，无明显的细胞增生性反应,。,新月体性肾小球肾炎，,GBM,断裂,(),，肾小囊细胞增生，纤维素沉积,(F),。,型新月体性肾小球肾炎，,IgA,团块状和颗粒状沉积于系膜区及毛细血管壁，即新月体性,IgA,肾病,。,3),弥漫性系膜增生性肾小球肾炎,(diffuse mesangioproliferative GN),机制,：短时间，少量，,大分子免疫复合物沉积,/,其他类型,GN,演变而来,LM,：,系膜细胞和基质弥漫性轻、中、重度增生,嗜复红,蛋白沉积,IM,：,IgG,和,C3,沿系膜区团块状沉积,EM,：,系膜增生伴电子致密物沉积,临床,：各年龄均可发病,隐匿性肾炎、蛋白尿、血尿、肾,病综合征、慢性肾炎,中度系膜增生性肾小球肾炎,重度系膜增生性肾小球肾炎,系膜增生性肾小球肾炎，,IgG,在系膜区团块状沉积,。,系膜增生性肾小球肾炎，电子致密物,(D),沉积于系膜区,。,4),弥漫性膜增生性肾小球肾炎,(diffuse membranoproliferative GN),机制,：,长期慢性,大量，,大分子免疫复合物沉积（,I,，,III,型）；补体代谢障碍（,II,型）,LM,：,系膜细胞和基质弥漫性重度增生,，广泛插入，,GBM,弥漫增厚，双层或多层化，血管腔闭塞,IM,：,I,和,III,型：,IgG,和,C3,沿系膜区及毛细血管壁团块状和颗粒状沉积。,II,型：,C3,沿系膜区及毛细血管壁沉积。,EM,：,I,和,III,型：系膜增生，,插入，,伴内皮下和上皮下电子致密物沉积。,II,型：基底膜内带状电子致密物沉积。,临床,：青壮年发病,，,肾病综合征、慢性肾炎,5),肾小球微小病变,(minimal change nephopathy),机 制,：,不清楚，可能与细胞免疫有关,LM,：无病变或轻微病变,IM,：阴性,EM,：上皮细胞足突广泛融合,临 床,：,儿童发病，肾病综合征,6),膜性肾小球肾炎,(membranous GN),机 制,：,长期慢性,小分子量免疫复合物沉积,/,原,位免疫复合物沉积,LM,：,GBM,弥漫性增厚,GBM,外侧颗粒状嗜复,红蛋白沉积,GBM,钉突形成,IM,：,IgG,和,C3,沿毛细血管壁细颗粒状沉积,EM,：,GBM,外侧多数电子致密物沉积,临 床,：,中老年发病,肾病综合征,7),局灶性肾小球肾炎,(focal GN),机 制,：,短时间,少量免疫复合物沉积,LM,：,部分肾小球,(,少于,50%),或部分毛细血管,襻坏死、增生或硬化,IM,：,IgG,和,C3,沿系膜区和毛细血管壁团块状,和颗粒状沉积,EM,：,系膜增生伴电子致密物沉积,临 床,：,各年龄均可发病,隐匿性肾炎,蛋白尿,血尿,局灶性肾小球肾炎的特殊类型,局灶节段性肾小球硬化症,(Focal Segmental Glomerulosclerosis),病因发病机制：,原发性,FSGS,：,1.,各种原因导致的肾小球上皮细胞损伤和剥脱；,2.,超负荷蛋白导致的肾小球上皮细胞损伤和剥脱；,3.,高脂血症；,4.,肾小球血液动力学改变；,5.,血内通透因子的出现。,继发性,FSGS,：,各种,GN,演变而来。,LM,：,局灶节段性肾小球硬化球性硬化多数肾小球硬化,IM,：,IgM,和,C3局灶节段性沉积,EM,：,系膜基质增多，电子致密物沉积，上皮细胞足突融合，剥脱,临床表现：,大量蛋白尿，肾病综合征,8),硬化性肾小球肾炎,(sclerosing GN),机 制,：,各种肾小球肾炎或肾小球病的终末阶段,LM,：,大部分肾小球系膜基质增生,硬化,(,50%,的肾小球,),IM,和,EM,：,无意义,临 床,：,青壮年和老年发病,慢性肾炎,肾功能衰竭,5.,临床病理联系,尿量,：,少尿或无尿,:,内皮或系膜细胞大量增生,毛细血管堵塞,(,毛细血管内增生性,GN,，,膜增生性,GN),；,大量新月体形成,(,新月体,性,GN),；,大量肾单位荒废,(,硬化性,GN),多尿、夜尿和等比重尿：大量肾单位荒,废,(,硬化性,GN),尿质,：,蛋白尿,：,肾小球毛细血管通透性增加,(,各型,GN);,电荷屏障破坏,(,微小病变性肾小球病,),血 尿,：,肾小球毛细血管壁严重损伤和断裂,(,除,微小病变性肾小球病和膜性,GN,外的肾小球肾炎,),肾病综合征,(renal syndrome):,长期大量蛋白尿,(,大于,3.5g/24h),导致低蛋白血症,导致严重水肿，肝脏代偿性合成脂蛋白增强导致高脂,血症。,尿毒症,(uremia):,因肾功能衰竭,代谢废物体内潴留,水和电解质紊,乱,，,自身中毒。导致实质脏器水肿,;,化学性炎,;,多系统,因中毒而功能遭抑制,因电解质紊乱而代谢异常,CONCLUSION,The,glomerulinephritis(GN),is a nonsuppurative inflammation,is caused by allergic reaction or hypersensitive reaction.Glomerulus is major injury site.Its clinical manifestation include:proteinuria,hematuria,renal hypertension,renal edema and various degree influence on renal function.CONCLUSION,antigen and atibody complex or immune complex,：,circulating immue complex,(non-renal antigen),immune complex in situ,(anti-GBM antigen,anti-epithelial antigen:gp330,anti-mesangium antigen:Thy1,anti-endothelial antigen:ACE)(anti-planted antigen).,CONCLUSION,Immune complex deposit in,varing site of glomerui,.,The molecular size,carring charge,quantity and deposited speed etc.of immune complex are key factors for its deposited site.,The immune complex activate,inflammatory mediators,including active complement,capillary active material and cytokin,etc.,Pathologic characteristic of glomerulonepfritis,正常,内皮细胞增生,系膜细胞增生,上皮下驼峰状,电子致密物沉积,Endocapillary proliferative GN,:,large number,small size immune complex suddenly deposit on subepithelial site.Endothelial cells and mesangial cells diffusely markedly proliferate,with leukocyte infiltration.,Membranous GN,:,small number and small size immune complex,，,immune complex in situ or planted antigen,，,slowly deposit or form on subepithelial site.GBM becomes diffuse thickening.,正常,Mesangioproliferative GN,:,small number and large size immune complex deposit on mesangial site.Mesangial cells and matrix become diffuse proliferation.,Membranoproliferative GN,:,Type I,large number and large size immune complex deposit on mesangial site.Mesangial cells and matrix become diffuse proliferation,interposition and thick GBM.,Type II,C3 nephritic factor(anti-C3bBb antibody)is form.Mesangial cells and matrix become diffuse proliferation,large number electron dense deposit appear in GBM.,单核巨噬细胞浸润，,新月体形成,正常,上,皮细胞增生,GBM,断裂，,纤维蛋白漏出,Crescentic GN,:,strong allergic reaction by anti-GBM antibody or other immune complex deposit.Glomerular capillary wall suffer severe damege and large number crescent formed.,Minimal change GN,:is from,negative charge decrease in filtrated barrier or the effect of cellular immune reaction.Diffuse effacement or fusion of foot processes of glomerular epithelial cell.,Focal GN,:,small number immune complex deposit in glomeruli,but show focal distribubution.Glomerular lesions show focal and/or segmental necrosis,proliferation or sclerosis.,Sclerosing GN or end-stage glomerular disease,:,various type GN will become sclerosing GN,if they are can not recover in a long time.The hyaline sclerosing glomeruli are over 75%.,资料可以编辑修改使用,学习愉快！,课件仅供参考哦，,实际情况要实际分析哈！,感谢您的观看,