肾小球疾病

DepartmentofNephrology FirstAffiliatedHospitalSunYat sunUniversity Glomerulardisease outline AnatomyandfunctionGeneralpathogenesisClinicalsyndromesandpresentationSpecificglomerulardiseases Anatomyandfunction Anatomy Anatomy RenalFunction RemovewastesMaintainhomeostasisSecreteEPO Anatomy nephron GlomerularAnatomy CapillaryLumen Endothelialcell Glomerularbasementmembrane Epithelialcell Podocytes Structureofnormalglomerularcapillary 系膜细胞 系膜基质 内皮细胞 上皮细胞足突 Generalpathogenesis Whatcausesglomerulardisease Mostareofimmunologicorigin andcausedbyimmunecomplexes AntibodymediatedGN CirculatingImmunecomplex Location Mesangialandsub endothelial AntibodymediatedGN In situImmunecomplex Location GBMsub epithelial AntibodymediatedGN In situImmunecomplex trappedAg Location GBMsub epithelial pathogenesis GlomerularFiltrationBarrier GlomerularFiltrationBarrierInjury Fusionoffootprocess ProteinuriaGBMInjury Hematuria Proteinuria ClassificationofGlomerularDisease Etiology Pathology ClinicalFeatures Clinicalsyndromesandpresentation CharacteristicsofGlomerularDiseases Parameter Glomerular Tubulointerstitial ProteinuriaMWofProteinRenalmorphologyRBCMorphology Massive 1 5 2 0g dLarge Medium SmallSymmetrydysmorphic Smallamount 2 1 0g dSmallAsymmetrynormal Clinicalsyndromesandpresentation LatentGN Nephroticsyndrome AcuteGN RPGN ChronicGN 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 急进的肾功能恶化血尿 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 Howareglomurulardiseasesdiagnosed Usuallybyhistory physicalfindings Urinalysisandotherlaboratorydata Occasionallyarenalbiopsymustbeperformed RenalBiopsyProcessing RenalBiopsyProcessing RenalBiopsyProcessing RenalBiopsyProcessing Pathology PASMASSONH EPASM 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 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crecenticGN PathologicalclassificationofGN Clinicalsyndromesofglomerulardiseases Asymptomatichematuria orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis Asymptomatichematuria orproteinuriaLatentnephritis MildchronicGNorrecoveryphaseofacuteGNIsolatedmicroscopichematuria DysmorphicRBC IsolatedproteinuriaNoHBP edemaorAzotemiaTreatmentissimilartochronicGN CASEI 11year oldmaleHistory Intermittenthematuriax1yearHxofrecurrentpharyngitisPhysical tonsillitisUrinalysis 15RBC HPF1 proteinRBCcastsLabData dysmorphicRBC ThePatientHasIgAnephropathy IgANephropathy MostcommonGNMesangioproliferativeorotherpathologicaltypesPredominentmesangialIgAdepositPersistentorepisodeofhematuriaExacerbateoninfection1 3haveelevatedserumIgA Clinicalsyndromesofglomerulardiseases Asymptomatichematuria orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis NephroticSyndrome InsidiousonsetManifestationsProteinuria 3 5g dHypoalbuminemiaalb 30g lEdemaHyperlipidemia Nephroticsyndrom etiology 1 PrimaryNephroticsyndromminimalchangediseaseFSGSmembranousnephropathymembranoproliferativeGNIgAN Nephroticsyndrom etiology 2 SecondaryNephroticsyndromAutoimmunity SLE Infection HepatitisBorC HIVTumor solidcarcinoma lymphomaMetabolic DM AmyloidosisDrugs NSAIDS Nephroticsyndrom epidemiology ChildrenYongpeopleOldpeoplePrimaryMCDFSGS MsGNMNMPGNSecondaryHSPSLEDNHepatitisBHSPTumorInheritedNSHepatitisBMM AL 14year old male high schoolstudentHistory NosignificantmedicalhistoryFatiguex3weeksEdemax1weekPhysical MildgeneralizededemaUrinalysis 4 proteinManyhyalinecastsFewgranularcastsNoRBCsorRBCcastsLabData proteinuria4g d alb20g l normalrenalfunction Hepatitis Auto immunityAb Renalbiopsy CASEII ThepatienthasMinimalchangedisease ElectronMicroscopy effacementoffootprocesses Introduction Incidence Etiology ClinicalFeatures ClinicalCourse Lossofnetnegativechargeoncapillarybasementmembrane Nephroticsyndrome Prominentproteinuria edemaNohypertension Sensitivetosteroid relapsemayoccur 80 ofnephroticsyndromeinchildren MinimalChangeDisease CASEIII 65year old male Smokefor40yearsHistory Fatiguex3monthsCoughandchestpainx2monthsFacialedemax1weekPhysical edema Urinalysis protein LabData proteinuria8g d alb24g l normalrenalfunction Hepatitis Auto immunityAb WhyisathoroughClinicalevaluationimportantinpatientswiththenephroticsyndrome Manysuchpatientshaveanoccultmalignancy CASEIII LungCarcinoma CASEIII LM PASM spikes alongtheGBM CASEIII IF IgGdepositionalongGBM CASEIII EM subepithelialelectrondensematerial It sClearlyacaseOfcarcinomarelatedMembranousnephropathy CASEII MN Commonlyoccurredinmiddle old agedpeopleEtiology PrimarySecondary Tumor related HepatitisBrelated Drugs relatedPresentation HT Renalfailure Thrombosis Introduction MembranousNephropathy Incidence Etiology Path ClinicalCourse Immunecomplexdisease Mayassociatedwithcarcinomas infections drugs andheavymetals SomeadultsdevelopESRD Diffuse uniformbasementmembranethickeningwithsubepithelialprojections spikes Commonlyoccurredinmiddle old agedpeople HowtotreattheNephroticSyndrome Pathogenesis Complication TreatmentofNS Liver Edema Hypoalbuminemia Glominflammation Proteinuria Permeability LipoproteinSynthesis BloodLipid Primary SecondaryCauses TreatmentofComplication Bloodvolume Thrombosis CVdisease ARF Infection Antithrombotic Anti infection Diuretics Dialysis Statins 0 25mg kg d Slowthespeedoftapering 1mg kg d 8w 0 5mg kg d Taper5mgperweek Howtouseglucosteroids 1 Maintenancefor1year 0 25mg kg d Slowthespeedoftapering 1mg kg d 8w 0 5mg kg d Taper5mgperweek Howtouseglucosteroids 2 Maintenancefor1year Sufficientinitialdose 0 25mg kg d Slowthespeedoftapering 1mg kg d 8w 0 5mg kg d Taper5mgperweek Howtouseglucosteroids 3 Maintenancefor1year Sufficientinitialdose Slowtapering 0 25mg kg d Slowthespeedoftapering 1mg kg d 8w 0 5mg kg d Taper5mgperweek Howtouseglucosteroids Maintenancefor1year Sufficientinitialdose Slowtapering Longmaintenance Clinicalsyndromesofglomerulardiseases Asymptomatichematuria orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis 急性肾小球肾炎AcuteGN Historyofstreptococcusinfection2weeksagoAcuteonsetProminenthematuriaandRBCcastsARF HTLowC3 for8weeksESRincreased Anti DNAseB 急性肾小球肾炎AcuteGN 急性肾小球肾炎AcuteGN Clinicalsyndromesofglomerulardiseases Asymptomatichematuria orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis RapidprogressiveGN SimilartoacuteGNononsetRapiddeteriorationofrenalfunctionCr OliguriaandobviousmacroscopichematuriaCrescentformation 50 Needaggressivetherapy Largedoseofsteroidpulsetherapy CTX Plasmaphoresis RapidprogressiveGN RapidprogressiveGN Clinicalsyndromesofglomerulardiseases Asymptomatichematuria orproteinuriaNephroticsyndromeAcuteglomerularnephritisRapidlyprogressiveglomerularnephritisChronicglomerularnephritis Chronicglomerulonephritis Proteinuria 3 5g d HematuriaHypertensionEdemaAzotema BUN Cr ChronicGN NS TreatmentofChronicGN ReduceproteinuriaControlhypertensio