泌尿系统整合PBLPPT课件.pptx

1,泌尿系统PBL第二组,2,肾小球正常结构,杨佳妮、,3,4,肾单位（Nephron）,5,6,血管球毛细血管（有孔毛细管）：有孔（70nm左右，最大100nm）无隔膜内皮细胞游离面细胞衣负电荷,7,肾脏基膜（RenalBasementMembrane）连续结构由毛细血管内皮细胞与足细胞共同产生,8,足细胞（podocyte)：突起（process）裂孔（slitpore)裂孔膜(slitmembrane),9,10,GlomerulusFunction,张家旭,11,Glomerularfiltration,12,Glomerularfiltrationbarrier,Neutralsolutes:Solutessmallerthan2nanometersinradiusarefreelyfilteredSolutesgreaterthan4.2nanometersdonotfilterSolutesbetween2and4.2nmarefilteredtovariousdegrees,13,Glomerularfiltrationrate(GFR)isthevolumeoffluidfilteredfromtherenal(kidney)glomerularcapillariesintotheBowmanscapsuleperunittime.,KfisthefiltrationcoefficientaproportionalityconstantPgcistheglomerularcapillaryhydrostaticpressurePbcistheBowmanscapsulehydrostaticpressuregcistheglomerularcapillaryoncoticpressurebcistheBowmanscapsuleoncoticpressure=0,EFR,125ml/min,1.Changesinrenalbloodflow2.ChangesinglomerularcapillaryhydrostaticP-changesinsystemicBP-afferentorefferentarteriolarconstriction3.ChangesinhydrostaticPinBowmanscapsule-ureteralobstruction,renaledema4.Changesinglomerularcapillaryoncoticpressure5.ChangesinKf-Reductionineffectivefiltrationsurfacearea-Changesinglomerularcapillarypermeability,14,TwomechanismscontroltheGFRRenalautoregulationNervousandhumoralregulation,RegulationofGlomerularFiltration,Undernormalconditions(MAP=80-180mmHg)renalautoregulationmaintainsanearlyconstantglomerularfiltrationrateTwomechanismsareinoperationforautoregulation:MyogenicmechanismTubuloglomerularfeedback,15,尿常规,刘逸馨,16,项目,理学检验（physicalexam）：尿量、尿气味、尿外观、比重（SG）化学检验（chemicalexam）pH、蛋白质、葡萄糖、酮体、胆红素、尿胆原、血红蛋白/隐血、亚硝酸盐、白细胞酯酶、维生素C、微量白蛋白显微镜检验（microscopicexam）细胞（RBC、WBC）、管型、结晶、微生物,17,尿量（Vol）,正常：成人6002000ml/24h少尿（oliguria）：尿量2500ml/d，如糖尿病、尿崩症、使用利尿剂、咖啡因和乙醇,18,尿气味（Odor）,正常：芳香味，与摄入食物中挥发酸有关异常：提示病理情况、标本处理或贮存不当,19,外观,尿色(Col)正常：淡黄色至黄褐色（尿胆素）异常：血尿、胆红素尿、血红蛋白尿透明度(Clr)正常：清澈透明无沉淀。放置一段时间后，可出现絮状沉淀，尤其女性尿液；异常：尿液排挤时即浑浊，多由白细胞、上皮细胞、粘液、微生物等引起，需作显微镜检查予以辨别,20,比重（SG）,反映肾小管重吸收肾小球滤过成分、肾功能状态、患者脱水状态。正常：1.0151.025，晨尿最高增高：高热性脱水、急性肾小球肾炎、心功能不全，蛋白尿及糖尿病降低：尿崩症、慢性肾炎等肾脏浓缩功能减退等张尿：牢固在1.010左右，为肾实质严重受损，肾脏浓缩及稀释功能下降所致,21,化学检验（chemicalexam）,22,蛋白质（PRO）,肾功能异常的早期症状。正常：定性（-），定量080mg/24h肾小球性：重度（34g/d），以白蛋白为主，如链球菌感染后AGN，糖尿病肾病。肾小管性：轻度（20%、140mg/24h,39,Classification,BenignproteinuriaPathologicalproteinuriaGlomerularproteinuriaTubularproteinuriaOverflowproteinuria,40,Benignproteinuria,DehydrationFeverInflammatoryprocessIntensiveactivityMostacuteillnessesOrthostatic/Posturalproteinuria,41,Glomerularproteinuria,Mechanisms:Filtrationbarrierinjury(Size/Chargebarrier)Characteristic:HMWproteins70%-80%(IgG,transferrin,albumin)Morethan2g/24hCause:Primary:GN,nephroticsyndromeSecondary:Diabetesmellitus,LupusnephritisDrugs:Heroin,NSAIDs,42,Tubularproteinuria,Mechanisms:LowreabsorptionatproximaltubuleCharacteristic:LMWproteins50%(/-microglobulin)Albumin25%Lessthan1g/24hCause:InterstitialnephritisDrugs:Heavymetals,NSAIDs,antibioticsTransplantation,43,Overflowproteinuria,Mechanisms:IncreasedquantityofproteinsinserumCharacteristic:LMWplasmaproteins(Bence-Jonesprotein,Myoglobin,Hemoglobin)Cause:MonoclonalgammopathyLeukemiaRhabdomyolysisHemolysis,44,Microalbuminuria,MAU,Detectedbyspecialalbumin-specificurinedipsticksDiabetesmellitus,hypertensivenephropathy,Lupusnephritis,45,Selectiveproteinuriaindex,IgG150kDTRF70kD0.10.2Non-selectiveproteinuriaSizeSPIChargeSPI:AMY-S/AMY-P3g/dL)andcell-richfluidB.Swellingoftissue,nopittingedemaduetoviscosityC.Increasedvascularpermeabilityinvenules,associatedwithinflammationD.i.e.Tissueswellingafterabeesting,cellulitis,53,Type3Lymphedema,A.Protein-richfluidB.NopittingedemaduetoviscosityC.LymphaticobstructionD.i.e.Afterradicalmastectomy&radiation,filariasisduetoWuchereriabancrofti,54,Type4Myxedema,A.Increaseinhyaluronicacid(glycosaminoglycan)B.NopittingedemaduetoviscosityC.i.e.Gravesdisease,hypothyroidism,55,AboutMr.Yu,Pittingedemaeliminateexudate,lymphedema,myxedemaNosignsofascites,jaundice,spiderangioma,caputmedusaeeliminatecirrhosisedemaNosignsofjugularretention,hepatomegalyeliminatecardiacedemaNosymptomsofweightloss,vomiting&burn,nohistoryofdrug-takeeliminatemalnutrition&drug-inducededemaHematuria,dysmorphicRBC,renaldysfunction,hypertension,periorbitalpuffinesstoperipheraledemainjust3daysnephrogenicedema,56,肺出血-肾炎综合征GoodpastureSyndrome,方昊昱,57,Definition:,肺出血-肾炎综合征（GoodpastureSyndrome）由抗肾小球基膜(GBM)抗体导致的肾小球和肺泡壁基膜的严重损伤临床表现为肺出血、急进性肾小球肾炎和血清抗肾小球基膜抗体阳性三联征。型RPGN,58,Etiology:,1、感染:呼吸道感染，流感病毒感染HIV患者-卡氏肺囊虫肺炎2、吸入碳氢化合物:汽油蒸汽、羟化物、松节油3、吸入可卡因,59,机体,激活补体,ADCC,调理作用,细胞溶解,刺激,产生,抗体,抗肾小球基底膜抗体抗肺泡毛细血管基底膜抗体,肾小球基底膜、肺泡毛细血管基底膜,Pathogenesis:,60,Pathogenesis:,胶原的3()的NC1结构域，Goodpasture抗原Co14A3，2q352q37GBM、TBM、ABM生理条件-隐匿诱发因素-上皮/内皮/系膜细胞-炎性介质-胶原高级结构解离GBM-有孔毛细血管ABM-完整性破坏后出现病症HLA二类抗原相关的淋巴细胞T细胞细胞因子,61,Pathologicalchanges:,1.肾脏病变LM:细胞性新月体、纤维性新月体血管球萎缩、纤维化肾小管；肾间质EM:GBM断裂，无电子致密物沉积IF:IgG沿基膜线性连续，C3颗粒状沉积,62,Pathologicalchanges:,2.肺部病变LM:RBC、WBC、M含铁血黄素间质水肿、出血、浸润、纤维化EM:ABM断裂、溶解IF:IgG、C3沿ABM线状沉积,63,ClinicalFeatures:,1.肾脏症状血尿、蛋白尿、红细胞管型少尿、无尿、氮质血症急性肾衰、尿毒症,64,ClinicalFeatures:,2.肺部症状呼吸道感染咯血（低氧血症/呼吸困难）胸痛肺部叩诊呈浊音，听诊可闻湿啰音肺,3.其他缺铁性贫血，高血压，肝脾肿大，心脏扩大，眼底异常改变，皮肤紫癜，便血等,65,Goodpasture综合征诊断&治疗,杜佳飞,66,辅助检查,实验室检查,痰液：含铁血黄素细胞、血痰,尿液：血尿、蛋白尿,血液：小细胞低色素性贫血、白细胞高,肾功能：BUN和Scr进行性增高，Ccr降低,特异性检查：血清抗GBM抗体阳性,67,辅助检查,肺部浸润是肺部病变的特征,68,辅助检查,肾小球新月体形成,抗GBM,抗肾小球基底膜抗体沉着,69,诊断,典型患者的诊断完全符合下列三联征(1)肺出血，肺泡基膜IgG呈线样沉积。(2)急进性肾炎综合征肾脏大量新月体形成(毛细血管外增生性肾炎)可伴毛细血管坏死GBM有IgG呈线样沉积(3)血清抗GBM抗体阳性,70,诊断,注意事项（1）表现轻微或不同步发生病变。有时只发生于一脏器。（2）与其他肾小球疾病(主要是膜性肾病)有时可相互转变（3）偶尔自身免疫功能紊乱会产生非特异性基膜抗体，还可引起肺肾以外的器官损害（4）个别情况下如自身免疫高度活动期，大量抗GBM抗体沉积可发生一过性血清抗GBM抗体阴性。,71,治疗,1、一般治疗要加强护理注意保暖，防治感冒，戒除吸烟，减少和避免各种可能的致病诱因，合并感染（抗菌药）2、血浆置换与免疫吸附疗法：可去除抗GBM抗体，前者24L/h，每天或隔天1次，持续24周3、联合应用免疫抑制剂和糖皮质激素疗法，能有效地抑制抗基膜抗