肾小球疾病八年制

肾小球疾病八年制第1页outlineAnatomyandfunctionGeneralpathogenesisClinicalsyndromesandpresentationSpecificglomerulardiseases第2页Anatomyandfunction

第3页Anatomy第4页AnatomyRenalFunctionRemovewastesMaintainhomeostasisSecreteEPO第5页Anatomy-nephron第6页第7页第8页第9页GlomerularAnatomyCapillaryLumenEndothelialcellGlomerularbasementmembraneEpithelialcellPodocytes第10页Structureofnormalglomerularcapillary系膜细胞系膜基质内皮细胞上皮细胞足突第11页第12页第13页Generalpathogenesis第14页Whatcausesglomerulardisease?Mostareofimmunologicorigin,andcausedbyimmunecomplexes!第15页AntibodymediatedGN-

CirculatingImmunecomplexLocation:Mesangialandsub-endothelial第16页AntibodymediatedGN-

In-situImmunecomplex

Location:GBMsub-epithelial第17页AntibodymediatedGN-

In-situImmunecomplex

(trappedAg)Location:GBMsub-epithelial第18页pathogenesis原位免疫复合物形成循环免疫复合物激活T淋巴细胞补体激活细胞因子C5b-9C5a,C3a上皮,系膜,内皮细胞巨噬细胞多种核细胞,血小板系膜细胞氧化应激,蛋白酶,促凝GlomerularDisease第19页GlomerularFiltrationBarrier第20页GlomerularFiltrationBarrierInjuryFusionoffootprocess:ProteinuriaGBMInjury:Hematuria/Proteinuria第21页ClassificationofGlomerularDiseaseEtiologyPathologyClinicalFeatures第22页Clinicalsyndromesandpresentation

第23页CharacteristicsofGlomerularDiseasesParameterGlomerularTubulointerstitialProteinuriaMWofProteinRenalmorphologyRBCMorphologyMassive>++>1.5~2.0g/dLarge/Medium/SmallSymmetrydysmorphicSmallamount<2+<1.0g/dSmallAsymmetrynormal第24页ClinicalsyndromesandpresentationLatentGNNephroticsyndromeAcuteGNRPGNChronicGN镜下或肉眼血尿蛋白尿畸形红细胞,棘型红细胞蛋白尿>3.5g/d低蛋白血症高脂血症水肿血尿蛋白尿(1-3g/d)ARF水肿高血压红细胞管型急进旳肾功能恶化血尿,蛋白尿进行性少尿,无尿红细胞管型有或无系统症状血尿蛋白尿高血压肾功能减退第25页Howareglomurulardiseasesdiagnosed?Usuallybyhistory,physicalfindings,Urinalysisandotherlaboratorydata.Occasionallyarenalbiopsymustbeperformed!第26页RenalBiopsyProcessing第27页RenalBiopsyProcessing第28页RenalBiopsyProcessing第29页RenalBiopsyProcessing第30页PathologyPASMASSONH&EPASM第31页PathologicalclassificationofGN轻微病变性肾小球肾炎minimalchangeglomerulonephritis局灶节段性病变focalsegmentallesions弥漫性肾小球肾炎diffuseglomerulonephritis膜性肾病membranousnephropathy增生性肾炎proliferativeglomerulone-phritis硬化性肾炎sclerosingglomerulonephritis未分类性肾小球肾炎unclassifiedglomerulo-nephritis第32页增生性肾炎proliferativeglomerulone-phritis

系膜增生性肾小球肾炎mesangialproliferativeGN毛细血管内增生性肾小球肾炎endocapillaryproliferativeGN系膜毛细血管内增生性肾小球肾炎mesan-gialcapillaryGN新月体肾炎crecenticGNPathologicalclassificationofGN第33页ClinicalsyndromesofglomerulardiseasesAsymptomatichematuria/orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis第34页第35页第36页第37页Asymptomatichematuria/orproteinuria

LatentnephritisMildchronicGNorrecoveryphaseofacuteGNIsolatedmicroscopichematuria(DysmorphicRBC)IsolatedproteinuriaNoHBP,edemaorAzotemiaTreatmentissimilartochronicGN第38页CASEI

11year-oldmaleHistory: Intermittenthematuriax1year HxofrecurrentpharyngitisPhysical:tonsillitis

Urinalysis: 15RBC/HPF

1protein RBCcastsLabData:dysmorphicRBC

第39页H&E第40页PAS第41页IgA第42页第43页ThePatientHasIgAnephropathy!第44页IgANephropathyMostcommonGNMesangioproliferativeorotherpathologicaltypesPredominentmesangialIgAdepositPersistentorepisodeofhematuriaExacerbateoninfection1/3haveelevatedserumIgA第45页ClinicalsyndromesofglomerulardiseasesAsymptomatichematuria/orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis第46页NephroticSyndromeInsidiousonsetManifestationsProteinuria>3.5g/dHypoalbuminemiaalb

<30g/l

EdemaHyperlipidemia第47页Nephroticsyndrom-etiology(1)PrimaryNephroticsyndrom

minimalchangedisease FSGSmembranousnephropathymembranoproliferativeGNIgAN

第48页Nephroticsyndrom-etiology(2)SecondaryNephroticsyndrom

Autoimmunity:SLE,Infection:HepatitisBorC,HIVTumor:solidcarcinoma,lymphomaMetabolic:DM,AmyloidosisDrugs:NSAIDS

第49页Nephroticsyndrom-epidemiology

Children

Yongpeople

OldpeoplePrimary

MCDFSGS,MsGNMNMPGNSecondaryHSPSLEDNHepatitisBHSPTumorInheritedNSHepatitisBMM,AL

第50页14year-old,male,high-schoolstudentHistory: Nosignificantmedicalhistory Fatiguex3weeks Edemax1weekPhysical: MildgeneralizededemaUrinalysis: 4protein Manyhyalinecasts Fewgranularcasts

NoRBCsorRBCcastsLabData: proteinuria4g/d,alb20g/l,normalrenalfunction,Hepatitis(-),Auto-immunityAb(-)RenalbiopsyCASEII第51页The

patienthasMinimalchangedisease!第52页第53页ElectronMicroscopy:effacementoffootprocesses第54页Incidence:Etiology:ClinicalFeatures:ClinicalCourse:Lossofnetnegativechargeoncapillarybasementmembrane.Nephroticsyndrome.Prominentproteinuria&edemaNohypertensionSensitivetosteroid,relapsemayoccur.80%ofnephroticsyndromeinchildrenMinimalChangeDiseaseIntroduction第55页CASEIII65year-old,male,Smokefor40yearsHistory: Fatiguex3months

Coughandchestpainx2months

Facialedemax1weekPhysical: edema,Urinalysis: protein++++

LabData:

proteinuria8g/d,alb24g/l,normalrenalfunction,

Hepatitis(-),Auto-immunityAb(-)第56页WhyisathoroughClinicalevaluationimportantinpatientswiththenephroticsyndrome!Manysuchpatientshaveanoccultmalignancy!第57页CASEIIILungCarcinoma第58页SilverPAS第59页CASEIIILM-PASM:”spikes”alongtheGBM第60页CASEIIIIF:IgGdepositionalongGBM第61页CASEIIIEM:subepithelialelectrondensematerial第62页It’sClearlyacaseOfcarcinomarelatedMembranousnephropathy!第63页CASEII-MNCommonlyoccurredinmiddle&old-agedpeopleEtiology：Primary

Secondary：Tumor-related,HepatitisBrelated,Drugs-related

Presentation:HT&Renalfailure,Thrombosis

第64页MembranousNephropathyIncidence:Etiology:Path:ClinicalCourse:Immunecomplexdisease.Mayassociatedwithcarcinomas,infections,drugs,andheavymetals.SomeadultsdevelopESRD.Diffuse,uniformbasementmembranethickeningwithsubepithelialprojections(“spikes”).

Commonlyoccurredinmiddle&old-agedpeopleIntroduction第65页HowtotreattheNephroticSyndrome?第66页Pathogenesis/ComplicationLiverEdemaHypoalbuminemiaGlominflammation

Proteinuria↑Permeability↑LipoproteinSynthesis↑BloodLipid↑PrimarySecondaryCausesBloodvolume↓ThrombosisCVdisease↑ARFInfectionDevelopment↓第67页TreatmentofNSLiverEdemaHypoalbuminemiaGlominflammation

Proteinuria↑Permeability↑LipoproteinSynthesis↑BloodLipid↑PrimarySecondaryCausesCausativeTherapySteroid/CTX/CsAACEI/ARBAlbumininfusion?Diuretics第68页LiverEdemaHypoalbuminemiaGlominflammationProteinuria↑Permeability↑LipoproteinSynthesis↑BloodLipid↑PrimarySecondaryCausesTreatmentofComplicationBloodvolume↓ThrombosisCVdisease↑ARFInfectionAntithromboticAnti-infectionDiuretics/DialysisStatins第69页0.25mg/kg.dSlowthespeedoftapering1mg/kg.d×8w0.5mg/kg.dTaper5mgperweekHowtouseglucosteroids(1)Maintenancefor1year第70页0.25mg/kg.dSlowthespeedoftapering1mg/kg.d×8w0.5mg/kg.dTaper5mgperweekHowtouseglucosteroids(2)Maintenancefor1yearSufficientinitialdose第71页0.25mg/kg.dSlowthespeedoftapering1mg/kg.d×8w0.5mg/kg.dTaper5mgperweekHowtouseglucosteroids(3)Maintenancefor1yearSufficientinitialdoseSlowtapering第72页0.25mg/kg.dSlowthespeedoftapering1mg/kg.d×8w0.5mg/kg.dTaper5mgperweekHowtouseglucosteroidsMaintenancefor1yearSufficientinitialdoseSlowtaperingLongmaintenance第73页ClinicalsyndromesofglomerulardiseasesAsymptomatichematuria/orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis第74页急性肾小球肾炎AcuteGNHistoryofstreptococcusinfection2weeksagoAcuteonsetProminenthematuriaandRBCcastsARF&HTLowC3，for8weeksESRincreased，Anti-DNAseB（＋）第75页急性肾小球肾炎AcuteGN第76页急性肾小球肾炎AcuteGN第77页ClinicalsyndromesofglomerulardiseasesAsymptomatichematuria/orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritis

Chronicglomerularnephritis第78页RapidprogressiveGNSimilartoacuteGNononsetRapiddeteriorationofrenalfunctionCr↑,OliguriaandobviousmacroscopichematuriaCrescentformation>50%Needaggressivetherapy(Largedoseofsteroidpulsetherapy＋CTX；Plasmaphoresis)第79页RapidprogressiveGN第80页RapidprogressiveGN第81页ClinicalsyndromesofglomerulardiseasesAsymptomatichematuria/orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis

第82页ChronicglomerulonephritisProteinuria(<3.5g/d)HematuriaHypertens