四医大本科泌尿系统疾病

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,四医大本科泌尿系统疾病,内容,肾小球肾炎,肾小管-肾间质性肾炎,肾盂肾炎,药物引起的肾小管间质性肾炎,肾和膀胱常见肿瘤,正常肾脏结构复习,肾脏的生理单位,滤过屏障,a normal adult kidney,a normal glomerulus,肾单位,肾小体,肾小管,肾小球,肾小囊,近端肾小管,远端肾小管,细 段,（血管球）,（脏层、囊腔、壁层）,曲部,（近曲小管）,直部,（降支粗部）,降支细部,升支细部,直部,曲部,（远曲小管）,（升支粗部）,髓袢降支,髓袢升支,髓 袢,泌尿系统疾病的分类,1 炎症：变态反应性炎、感染,2 代谢性 3 血管性,4 中毒性 5 尿路阻塞,6 先天性畸形 6 遗传病,7 肿瘤,第一节,肾 小 球 肾 炎,(glomerulonephritis),一 病因和发病机制：,(一)引起肾小球肾炎的抗原,1 内源性抗原：,(1)肾小球本身成分：,基底膜成分(层连蛋白、Goodpasture抗原)、毛细血管上皮Heymann抗原、内皮细胞膜抗原、系膜细胞抗原等,(2)非肾小球抗原：,核抗原、DNA、免疫球蛋白、甲状腺球蛋白、肿瘤抗原、免疫复合物等,2 外源性抗原：,(1) 感染的产物：,细菌：链球菌、葡萄球菌等；,病毒：乙肝病毒、EB病毒；,霉菌：白色念珠菌；,寄生虫：丝虫、疟疾。,(2) 药物：金、汞、青霉胺等,(3),异种血清、类毒素等,1 循环免疫复合物沉积,非肾小球源性抗原，可分为：,外源性：感染产物、药物、异种蛋白,内源性：,DNA,、,肿瘤抗原等,*免疫荧光：颗粒状荧光,(二)肾小球肾炎的免疫发病机制,2.肾小球原位免疫复合物形成,(1)抗体与肾小球内固有的不溶性肾小球抗原结合；,(2)抗体与植入到肾小球内的非肾小球抗原结合。,*免疫荧光：线性或颗粒状荧光,3 影响抗原、抗体、免疫复合物在肾小球内沉积和沉积部位的因素：,(1)抗原抗体免疫复合物的大小；,(2)抗原、抗体的比例；,(3)抗原结合价、抗体亲和力；,(4)与循环免疫复合物的电荷有关：,阳离子：易通过基底膜（上皮下）,阴离子：不易通过基底膜（内皮下、吞噬清除）,中性复合物：系膜区,需要分清的几个概念,弥漫性病变（,50%,）,局灶性病变（,50%的肾小球有大新月体形成。,新月体：,肾小球壁层上皮细胞增生，单核细胞渗出，堆积成层，在肾球囊内毛细血管丛周围呈新月形或环状，故称新月体或环状体。,新月体分类,I 按新月体的大小,小新月体：体积50%，又称环状体或盘状体。,II 按新月体的成份,细胞性新月体,纤维细胞性新月体纤维性新月体,细胞性新月体成份：增生的肾小球上皮细胞（壁层上皮细胞）+渗出的单核细胞+红细胞+中性粒细胞+纤维素性渗出。,(3)大体：肾小球体积增大，色苍白，皮质内可见散在的点状出血。,(4)电镜：肾小囊内可见纤维素性沉积，细胞增生和浸润，进而基质增多，胶原纤维形,成。,(5)荧光：随病因的不同而不同，IgG、C3线状或颗粒状沉积于毛细血管壁或系膜区。,the glomeruli are crescents composed,of proliferating epithelial cells.,glomerulus with epithelial crescents squashing,the glomerular tufts from all sides.,This immunofluorescence micrograph of a glomerulus,demonstrates positivity with antibody to fibrinogen.,3.临床病理联系：,急进性肾炎综合征_ 血尿、蛋白尿、水肿、高血压，少尿、无尿，氮质血症，尿毒症。,预后急差。,另：肺出血肾炎综合征,（Goodpasture syndrome）,(1)临床表现：,多见于青壮年男性。,起病急、进展快，,咳血和进行性肾衰。,(2)病因及病理变化：,抗肾小球基底膜抗体,新月体性肾小球肾炎,与肺泡基底膜有交叉反应肺出血,positivity with antibody to IgG and has a smooth, diffuse,linear pattern that is characteristic for deposition of glomerular,basement membrane antibody with Goodpasture syndrome.,(三)肾病综合征及相关的肾炎类型,1.膜性肾小球肾炎,(membranous glomerulonephritis),2.轻微病变性肾小球肾炎,(minimal change glomerulonephritis),3. 局灶性节段性肾小球肾炎,(focal segmental glomerulonephritis ),4.膜增生性肾小球肾炎,(membranoproliferative glomerulonephritis),5.系膜增生性肾小球肾炎,(mesangioproliferative glomerulonephritis),1.膜性肾小球肾炎,(membranous glomerulonephritis),又称膜性肾病，多见于中老年男性。,特征: 上皮下含免疫复合球蛋白的电子致密物沉积,弥漫性毛细血管壁增厚。,(1)临床表现：起病缓慢，病程长，大量蛋白尿或肾病综合征。,(2)大体：肾脏体积增大，色苍白，皮质明显增,宽，大白肾。,(3)光镜：毛细血管壁弥漫性增厚，但细胞数较正常。特殊染色下可见“钉突”、“链环”等结构。,(4)电镜：毛细血管上皮下有电子致密物，基底膜增厚。,(5)荧光：免疫球蛋白、补体（IgG、C3）沿毛细血管壁呈高亮度细颗粒状沉积。,I期,II期,III期,IV期,膜性肾小球肾炎的分期示意图,the darker electron dense immune deposits,are seen scattered within the thickened basement,membrane. The spikes seen with the silver stain,represent the intervening matrix of basement membr,ane between the deposits.,the capillary loops are thickened and prominent,but the cellularity is not increased.,deposts of mainly IgG and complement collect in,the basement membrane and appear in a diffuse,granular pattern by immunofluorescence, as seen here.,2.轻微病变性肾小球肾炎,(minimal change glomerulonephritis),又称脂性肾病，多见于儿童。,(1)临床表现：肾病综合征。,(2)大体：肾肿大，色苍白，切面可见黄色条纹。,(3)光镜：肾小球无病变或仅见轻微病变，肾小管上皮细胞空泡变性和脂肪变性。,(4)电镜：,肾小球上皮细胞足突广泛融合、消失-特征。,(5)荧光：Ig和补体（-）或弱阳性。,This is minimal change disease (MCD) which,is characterized by effacement of the epithelial,cell (podocyte) foot processes and loss of the,normal charge barrier such that albumin selectively,leaks out and proteinuria ensues.,3 局灶性肾小球肾炎、局灶节段性肾小球硬化症(focal glomerulonephritis； focal segmental glomerulonephritis，FSGS),特点:部分肾小球的部分小叶硬化,脏层上皮细胞损伤,(1)临床表现：无症状血尿、大量蛋白尿和/或肾病综合征。,(2)光镜：肾小球局灶性病变（坏死、增生、硬化）,局灶+节段性硬化=FSGS,(3)电镜：病变部位有电子致密物。,(4)荧光：IgG、C3颗粒状或团块状沉积于病变部位。,An area of collagenous sclerosis runs across the middle of,this glomerulus. In contrast to minimal change disease,patients with FSGS are more likely to have non-selective,proteinuria, hematuria, progression to chronic renal failure,and poor response to corticosteroid therapy.,This trichrome stain of a glomerulus in a patient with,focal segmental glomerulosclerosis (FSGS) demonstrates,blue collagen deposition. FSGS accounts for about a sixth,of cases of nephrotic syndrome in adults and in children.,4. 膜增生性肾小球肾炎,(membranoproliferative glomerulonephritis MPGN),又称系膜毛细血管性肾小球肾炎，多见于青年人和中年人。,特点: 肾小球基膜增厚，系膜细胞和基质增加。,(1)临床表现：肾病综合征、部分为急性肾炎,综合征。,(2)光镜：肾小球明显分叶，系膜细胞和基质,增生，广泛插入到毛细血管基底膜与内,皮细胞之间,毛细血管壁增厚，“双轨”,形成。,(3)电镜：毛细血管基底膜不规则增厚，增生系膜组织插入到基底膜和内皮细胞之间，电子致密物可沉积在基底膜和系膜区的不同部位（内皮下、上皮下、系膜区）。,(4)荧光：IgG、C3呈颗粒状沿毛细血管壁和系膜区沉积。,This is membranoproliferative glomerulonephritis (MPGN).,Those cases that are idiopathic are divided into types I,and II by pathologic findings. As seen here, the glomerulus,has increased overall cellularity, mainly increased mesangial cellularity.,This silver stain demonstrates a double contour to many basement membranes, or the tram-tracking“ that is characteristic of membranoproliferative glomerulonephritis (MPGN) type I that results from basement membrane reduplication.,demonstrates a mesangial cell at the lower left that is interposing,its cytoplasm at the arrow into the basement membrane,leading to splitting and reduplication of basement membrane,that is piled up above the mesangial cytoplasm in this micrograph.,This is MPGN type I.,5. 系膜增生性肾小球肾炎,(mesangioproliferative glomerulonephritis),特点：弥漫性系膜细胞和系膜基质增生。,多见于青少年,。,(1)临床表现：肉眼/镜下血尿、蛋白尿、少数,肾病综合征。,(2)光镜：系膜细胞和/或系膜基质增生，系膜,区增宽。,(3)电镜：系膜区有电子致密物。,(4)免疫荧光：IgG/,或IgM、C3沿系膜区团,块状沉积。,(四) IgA肾病,(IgA nephropathy, Berger disease),(1)临床表现多样。,(2)病理变化多样。,(3)荧光：IgA和C3团块状沉积于系膜区、血管壁。,This is IgA nephropathy (Berger disease). The IgA is deposited,mainly within the mesangium, which then increases mesangial,cellularity as shown at the arrow. Patients with IgA nephropathy,usually present with hematuria.,This immunofluorescence pattern demonstrates positivity,with antibody to IgA. Note that the pattern is that of mesangial,deposition in the glomerulus. This is IgA nephropathy.,(五)慢性肾小球肾炎 (chronic,glomerulonephritis,),硬化性肾小球肾炎(sclerosing glomerulonephritis),(1)临床表现：慢性肾功衰竭。,又称终末期肾病。,(2)大体：颗粒固缩肾（双肾体积减小，表面细颗粒状、质硬，皮质变薄，皮髓质分界不清，小血管壁增厚、变硬，口哆开）。,(3)光镜：,I 50%的肾小球纤维化、玻璃样变，所属的肾小管萎缩、纤维化、消失；,II 残存肾单位代偿性肥大（肾小球体积增大、小管扩张）；,III 间质纤维组织明显增生，有多量淋巴细胞、单核细胞浸润。,IV 小动脉硬化，壁厚、腔小。,临床病理联系：,慢性肾炎综合征：,尿量多尿、夜尿、低比重尿,高血压,贫血,氮质血征、尿毒征,第二节 肾小管-间质性肾炎,一、肾盂肾炎(pyelonephritis),以肾小管、肾盂和肾间质为主的炎症，多见于女性（男：女=1：910）。,临床表现：发热、腰部酸痛、血尿、脓尿等。,一 病因及发病机制：,1 与细菌感染有关，主要是革兰氏阴性菌，也可与霉菌感染有关。,肾组织、尿液培养（+）,2 感染途径：,（1）血源性感染,一般为双侧，以葡萄球菌多见。,（2）上行性感染,下泌尿道炎症（尿道炎、膀胱炎）,肾盂炎症。,一侧或双侧。,问题？,什么情况下会出现上行性感染？,女性的肾盂肾炎发病率为什么高 于男性？,（一） 急性肾盂肾炎,以肾间质和肾小管为主的化脓性炎症。,1 病理变化：,大体、镜下,2 合并症：,（1）急性坏死性乳头炎；,（2）肾盂积脓；,（3）肾周围脓肿。,3 结局：,This is an ascending bacterial infection leading to acute pyelonephritis. Numerous PMNs are seen filling renal tubules across the center and right of this picture.,（二） 慢性肾盂肾炎(chronic pyelonephritis),1 病理改变：,特点：肾小管和间质活动性炎症，有纤维疤痕形成，肾盂、肾盏变形。,大体：双侧肾不对称，体积减小，质硬，有不规则凹陷性瘢痕，间或有小脓肿。,镜下：肾小管、间质浸润较多慢性炎细胞及多少不等的中性粒细胞。肾小管萎缩、间质纤维化、胶样管型，肾小球可硬化。,2 结局：,广泛累及双肾,高血压、肾衰。,*如何与硬化性肾炎区别？,二 、药物引起的肾小管-间质性肾炎,抗生素等诱发免疫反应，引起急性过敏性间质性肾炎。,（一）急性药物性间质性肾炎,间质炎细胞浸润,肾小管变性坏死,（二）镇痛药性肾炎,肾乳头坏死和慢性肾小管间质性炎,第三节 肾和膀胱肿瘤,一、肾细胞癌（renal cell carcinoma）,二、肾母细胞瘤（nephroblastoma）,三、膀胱尿路上皮癌（,urothelial carcinoma),一、肾细胞癌（renal cell carcinoma）,肾脏最常见的恶性肿瘤,男：女=2-3：1,散发性和遗传性两种类型,分类和病理变化,普通型肾细胞癌,乳头状,嫌色细胞癌,集合管癌,未分类,This is a renal cell carcinoma arising in the lower pole,of the kidney. It is fairly circumscribed.,This is the classic histologic appearance of a renal cell carcinoma:,the neoplastic cells have clear cytoplasm and are arranged in nests,with intervening blood vessels. This microscopic appearance is why,they are often called clear cell carcinomas.,临床病理联系,症状：腰痛、肾区肿块和血尿,预后较差，广泛转移，骨和肺转移最常见。,二、肾母细胞瘤(nephroblastoma),又称Wilms，起源于后肾胚基组织；,儿童肾脏最常见的恶性肿瘤；,散发性和家族性；,病理变化,大体：单个、实性肿物，边界清，有假包膜。,组织学特点：不同发育阶段的肾小球和肾小管样结构。,This small kidney from a 4 year old child contains a l,obulated tan-white mass. This is Wilms tumor of the,kidney. Many are now known to be associated with,genetic defects on chromosome 11.,Wilms tumor resembles the fetal nephrogenic zone of the kidney.,The tumor shows attempts to form primitive glomerular and,tubular structures,临床病理联系,儿童肿瘤的特点：,肿瘤发生与先天性畸形多有关系；,组织结构与起源组织胚胎期的结构类似；,临床疗效较好。,三、膀胱尿路上皮癌,泌尿系统最常见的恶性肿瘤；,男：女=2-3：1,发生与接触染料等化学物质有关；,病理变化：,大体：膀胱侧壁和膀胱三角区；,单发或多发；,乳头状或息肉状突起；,In the bladder removed surgically and opened here can be seen a large,urothelial carcinoma. These neoplasms arise from the urothelium.,the urothelial carcinoma does resemble urothelium, but the,thickness is much greater than normal urothelium, and the,neoplastic cells show more pleomorphism,临床病理联系,症状：无痛性血尿；,预后：与组织学分级相关；,转移：主要经淋巴道转移至局部淋巴结。,掌握各型肾小球肾炎的病理变化，临床病理联系；,掌握肾盂肾炎的病理变化；,了解药物引起的肾小管-间质性肾炎的病理变化；,了解肾脏和膀胱常见的肿瘤。,谢谢观赏,