病理学――泌尿系统课件.ppt

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1、泌尿系统疾病TheKidneyanditscollectingsystem第十二章肾脏功能排泄代谢产物内分泌功能调节体液平衡维持酸碱平衡Normaladultkidney150grams1millionnephrons14calycesNormaladultkidney4basicmorphologiccomponents肾小球Glomeruli肾小管Tubules肾间质Interstitium血管Bloodvessels流行病学美国40万终末肾病人HypertensionDiabetesChronicinfectionCysticdi

2、sease透析和肾移植$47,000peryearforperitonealdialysis$76,000peryearforhemodialysis$52,000perprocedure70,000peopleawaiting血透腹透肾小球疾病Glomerulardiseases原发性肾小球肾炎PrimaryGlomerulonephritis继发性肾小球疾病GlomerulopathiesSecondarytoSystemicDiseases遗传性肾小球疾病HereditarydiseaseVisceralepithelialcel

3、ls("podocytes")scanningelectronmicrograph水和离子自由通过，蛋白不能通过size-dependent(3.5nm)charge-dependent(thepolyanions）滤过膜肾小球损伤的机制大部分与免疫复合物沉积有关抗体在滤过膜与固有/植入抗体反应循环免疫复合物沉积Mediatedbycomplementactivation,polys,perhapsalsomacrophages,coagulationsystem,etc.肾穿刺RenalpunctureElectronmicrosco

4、pyNormal电子致密物沉积Granular,characteristicofcirculatingandinsituimmunecomplexdepositionImmunofluorescencemicroscopyLinear,characteristicofclassicanti-GBMantibodyGNNormalglomerulusstainedwithHENormalglomerulusstainedwithPAShowdoesglomerulardamageensue?1Podocyteinjury急性弥漫增生性肾小

5、球肾炎毛细血管内增生性肾小球肾炎(链球菌)感染后性肾小球肾炎(一)（五）Acutepost-streptococcalGN2weekslaterAcutediffuseproliferativeGNNormalAcutediffuseproliferativeGNEMImmunofluorescence(Igandcomplement)内皮细胞和系膜细胞弥漫性肿胀，增生Chokeofftheirbloodsupply毛细血管损伤血尿蛋白尿高血压水肿肾炎综合征Pathology－Clinicalfeatureoliguriaazotem

6、iaGFR↓预后儿童病例多数康复，少数进展为快速进行型GN或慢性肾病15%-25%成人病例发展为终末肾GoodpasturesGN,antiIgGCrescenticGN(PASstain)OliguriaAnuricAzotemiaNephriticsyndrome预后肾小球受累少于80%者略好于超过80%者部分病人血浆置换有效(Goodpasturesyndrome)膜性肾小球肾炎/膜性肾病30y-50y好发,缓慢进展Heymannnephritis(动物模型)AginsituActionofC5b-C9onpodocytes病理

7、变化：GBM弥漫性增厚足突消失(三-1)（三）NormalMembranousglomerulopathy(Silverstain)Membranousglomerulopathy(EM)Membranousglomerulopathy(EM)Immunofluorescence(Igandcomplement)GBMdamagedNephrinlost低蛋白血症大量蛋白尿高脂血症和脂尿高度水肿肾病综合征Pathology－Clinicalfeature预后差异大，起病和进展隐匿60%病例持续蛋白尿40%2-20年进展为终末肾10-

8、30%有部分或完全蛋白尿缓解轻微肾小球病变/脂性肾病光镜下肾小球正常1-7岁儿童DisordersofT-cell肾病综合症(三-2)（一）临床表现及预后选择性蛋白尿>90%对短程激素疗法有效，但其中2/3