局灶节段性肾小球硬化的诊断与治疗课件.ppt
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1、局灶节段性肾小球硬化的诊断与治疗 Focal Segmental Glomerulosclerosis(FSGS),简 介,In 1957,Arnold RichIn 1970s,International Study of kidney Disease,FSGSFSGS 被定义为一组临床病理综合症分为原发性(或特发性)与继发性 FSGSIn 2004,根据光镜下的特点分为5种亚型,FSGS的临床特征,无症状蛋白尿,肾病综合征 50-70%高血压 30-50%镜下血尿 25-75%肾功能减退 20-30%,几个重要概念,Sclerosis(硬化):细胞外基质增加并伴有毛细血管闭塞。Focal(
2、局灶):累及部分,但非全部肾小球。Segmental(节段):累及肾小球部分毛细血管袢,而非全部肾小球。透明样变:由血浆蛋白渗出引起的光滑的,玻璃样的改变。肾小球肥大:即肾小球毛细血管球比同龄正常人增大。(肾小球的面积 1.5 倍正常值).,FSGS亚型分类,FSGS,经典型FSGS,门部型FSGS,细胞型FSGS,塌陷型FSGS,顶端型,FSGS,经典型,(FSGS,not otherwise specified)(NOS,Classic FSGS),A normal appearing glomerulus with patent capillary lumina and glomerul
3、ar basement membranes of normal thickness.The afferent arteriole can be seen at the hilus(vascular pole)of the glomerulus.(Periodic Acid Schiff Stain),Glomerulus displaying segmental hyaline insudation and adhesionto Bowmans capsule.(Periodic Acid Schiff Stain),Low power view displaying some glomeru
4、li with focal segmental glomerulosclerosisand others appearing histologically unremarkable.(Silver Stain),Panel showing normal glomerulus on left and glomerulus withlesion of segmental sclerosis and podocyte hypertrophy on the right.,Panel showing intact foot processes of a normal glomerulus onthe l
5、eft and foot process effacement in FSGS on the right.,FSGS,经典型,最常见的类型.透明样变,粘连,泡沫细胞,足细胞增生血管,间质小管的改变与小球病变成比例各种亚型最终均可演变为经典型,FSGS,门部型,(FSGS,Perihilar variant),FSGS,门部型,定义:至少1G出现血管极透明样变,伴或不伴硬化 50%节段性受累的小球有极型硬化和/或透明样变 肾小球肥大、粘连很常见。常见于血流动力学改变或肾小球内高压引起的继发性FSGS。,FSGS,塌陷型,(FSGS,Collapsing Variant),FSGS,塌陷型,定义:
6、至少1G出现毛细血管塌陷,并伴有明显的足细胞肥大增生。足细胞常含有蛋白重吸收颗粒空泡样变。系膜细胞增生、肾小球肥大、玻璃样变不常见。间质小管损害非常明显。,FSGS,塌陷型,临床特点:重度蛋白尿或肾病综合征 肾功能损害明显,进展迅速 70%患者5年内进入尿毒症,Low-power view showing three glomeruli with cellular crescents froma patient with P-ANCA seropositivity.(Silver Stain),FSGS collapsing variant or Crescent?,The collapsin
7、g variant typically lack spindle cell morphology,pericellular matrix,extracapillary fibrin and blood elements,ruptures of BC.Podocyte hypertrophy with PRDs,vacuoles,nuclear enlargement and vesiculation,and prominent nucleoli.There is no continuity with the parietal epithelium until adhesions form.,F
8、SGS,细胞型,(FSGS,Cellular variant),FSGS Cellular variant,定义:至少1G出现毛细血管内细胞增多,至少累及25%毛细血管袢并引起官腔堵塞。毛细血管腔内细胞包括:泡沫细胞,巨噬细胞,内皮细胞白细胞。常见足细胞肥大、增生,但不是必需的特征。,FSGS,顶端型,(FSGS,Tip variant),The variant with tip lesion has a discrete segmental lesion with adhesion to Bowmans capsuleinvolving the periphery of the tuft a
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