病理学——泌尿系统

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–Hypertension–Diabetes–Chronicinfection–Cysticdisease•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HereditarydiseaseVisceralepithelialcells(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RenalpunctureElectronmicroscopyNormal电子致密物沉积Granular,characteristicofcirculatingandinsituimmunecomplexdepositionImmunofluorescencemicroscopyLinear,characteristicofclassicanti-GBMantibodyGNNormalglomerulusstainedwithHENormalglomerulusstainedwithPAShowdoesglomerulardamageensue?1Podocyteinjury急性弥漫增生性肾小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－ClinicalfeatureoliguriaazotemiaGFR↓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)•Aginsitu•ActionofC5b-C9onpodocytes•病理变化：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–10-30%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蛋白尿复发•204cases10yearsfollow-up–Completeremission71%–Persistproteinuria10%–Chronicrenalfaliure2%膜性增生性肾小球肾炎C3,IgGC1q,C4C3(三-4)（六）发病机制•TypeI–循环免疫复合物•TypeII–致密物沉积病–C3Nef(低补体血症)(PASstain)(Silverstain)(Silverstain)NPGN,TypeII临床表现及预后•肾病综合征•起病可为急性肾炎或轻微蛋白尿•预后差，II型更差–60years20yearsfollow-upCompleteremission0End-stagerenalfaliure40%Renalinsufficiency30%Persistentnephroticsyndrome30%IgA肾病Bergerdisease•青年和儿童多见•反复发作的镜下和肉眼血尿•系膜区IgA沉积(四)（八）发病机制IncreasedproductionReducedclearanceactivationofthealternativecomplementpathwayIgAdeposition临床表现及预后•50%肉眼血尿，30-40%镜下血尿，伴或不伴蛋白尿•5-10%发展为典型的急性肾炎综合征•25-50%病例20年缓慢进展为终末肾慢性肾小球肾炎•Unfortunateoutcomesofglomerulardiseases•30-50%病人需要透析或换肾•20%无肾病史•大量肾小球玻璃样变和硬化严重的不可逆的瘢痕形成(五)（九）HyalineobliterationofglomeruliTubulesarelost,vesselwallsarethickened(Massontrichromestain)临床病理联系•部分有明确肾病史，部分隐匿起病•慢性肾炎综合征–蛋白尿、高血压–多尿、夜尿、低比重尿–氮质血症、尿毒症•预后均很差Clinicalmanifestationsofrenaldisease•Azotemiaanduremia•Renalsyndromes–Acutenephriticsyndrome–Nephroticsyndrome–Asymptomatichematuriaorproteinuria–Rapidlyprogressiveglomerulonephritis–Acuterenalfailure–Chronicrenalfailure–Urinarytractinfection–Nephrolithiasis(renalstones)急性肾盂肾炎Acutepyelonephritis•化脓菌感染引起的肾盂、肾小管和肾间质的急性化脓性炎症–血源性感染Hematogenousinfection–上行性感染AscendinginfectionPredisposingconditions•插管和膀胱镜catheterizationandcystoscopy•女性Female，妊娠Pregnancy•尿储留Urinestasis•糖尿病Diabetesmellitus•膀胱输尿管返流Vesicoureteralreflux膀胱输尿管返流急性肾盂肾炎急性肾盂肾炎急性肾盂肾炎急性肾盂肾炎肾乳头坏死临床表现•症状和体征–脊肋角疼痛–全身症状–脓尿和菌尿–膀胱尿道刺激症(尿频尿急尿痛)•预后–自限性–有危险因素者可转为慢性–肾乳头坏死者预后差慢性肾盂肾炎Chronicpyelonephritis•Amorphologicentityinwhichpredominantlyinterstitialinflammationandscarringoftherenalparenchymaisassociatedwithgrosslyvisiblescarringanddeformityofthepelvicalycealsystem–慢性阻塞性肾盂肾炎–慢性返流性肾盂肾炎甲状腺样变临床表现•反复发作的急性肾盂肾炎•进行性出现肾功能不全和高血压•超声示肾脏变形