肾炎肾病幻灯课件

Nicetomeetyou AcuteGlomerulonephritis BeijingChildren sHospitalaffiliatedtoCapitalUniversityofMedicalSciencesMengQun AcuteGlomerulonephritis DefinitionEtiologyandPathogenesisPathologyClinicalfeaturesLaboratoryfindingsDiagnosisanddifferentialdiagnosisTreatment Definition acuteonsetpostinfectiousglomerulonephritis pharyngitisorpyodermaoftenbelongtoacutepoststreptococcalglomerulonephritis APSGN resultofinflammatoryglomerularinjury Definition hematuriaproteinuriaedemahypertensionrenalinsufficiency commonage 5 14y 2y raremale female 2 1self limitedprognosis finemorbidity onthedecline Etiology bacteria viruses mycoplasma groupA hemolyticstreptococcitype12 pharyngitistypes2 49 50 55 60 pyodermatype49 pharyngitisorpyoderma Pathogenesis secondarytoadirecttoxiceffectontheglomerulusofastreptococcalproteinthestreptococcalproductinduceanimmunecomplex mediatedinjury1 introducinganantigentotheglomrulus plantedantigen2 depositionofcirculatingimmunecomplexes3 alteringanormalrenalantigentoaself antigeninducinganautoimmuneresponsetotheself antigen streptococcalantigen CIC insituIC triggerautoimmunity complementactivited inflammatoryglomerularinjury GBMdamaged hematuriaproteinuria proliferativemesangialandendothelialcells GFR oliguria edema hypertention Pathology diffuseglobalhypercellularityproliferativemesangialandendothelialcellscrescentformationsubepithelialdepositioninterstitialedemainterstitialinfiltrationofleukocyte Clinicalfeatures presentfromasymptomatictooliguricacuterenalfailurelatentperiod afterpharyngitis 6 12daysafterskininfection 14 28days Nonspecificsymptomsandsignstirednessheadacheappetiteslightlyfevervomiting thesignofinfection Classicalmanifestation 1 hematuriagrosshematuria 50 70 patientscoffee coloredortea coloredturntomicrohematuria after1 2w Classicalmanifestation 2 edema 70 patientsreason sodiumandfluidretentionpresents faceperiorbitalupperextremities Classicalmanifestation 3 hypertention 30 80 patientsmildtomoderateschoolagepatients 130 90mmHgpre schoolagepatients 120 80mmHg Classicalmanifestation 4 proteinuria theextentisvariousmost 3g dnephrotic rangeproteinuria 20 patientsurinevolume Severecase 1 severehypervolemia oftenoccurs 1weekoftheonsetofAGNcausedbysodiumandfluidretentionmanifestations dyspneacoughsevereedemaenlargedheartandliver Severecase 2 hypertensiveencephalopathy intheearlyonsetofAGNBP 150 160 100 110mmHgreason CNSvasculitismanifestations headachevomitingconfusionsomnolenceconvulsioncoma Severecase 3 acuterenalfailure last3 5d 10dmesangialandendothelialcellsproliferationbloodvolumeofcapillaryglomerularfiltrationrate GFR Non Classicalmanifestation 1 asymptomaticAGN microhematuriawithoutotherclinicalmanifestationcontactingAGNpatientsorwhenstreptococciisepidemic Non Classicalmanifestation 2 extrarenalAGN edemahypertentionC3ASOurinetest nomalorslightlyabnomal Non Classicalmanifestation 3 nephroticsyndromelikeAGN severeedemahematuriaproteinuriahypoalbuminimiahypercolesteralimia Laboratoryfindings 1 urine dysmorphicredbloodcellredbloodcellcastscastsofleukocytesrenaltubuleepithelialcellshyalineandgranularcastsproteinuria erythrocytesedimentationrate antistreptolysinO ASO pharyngitis10 14daysofphasereachthehightesttiterin3 5weeks returntonomalin3 6monthantideoxyribonucleaseB pyoderma Laboratoryfindings 2 complement returntonormal 8weekscirculationgimmunecomplexes BUN Cr correlatewithdiseaseactivity Laboratoryfindings 3 Diagnosis acuteonset abnomalurinetestoredema oliguria hypertentionAPSGN above streptococcalinfectialdisease increasedASO decreasedC3 Differentialdiagnosis 1 AGNcausedbyotheretiologiesandpathogenicmechanisms suchasviruses Differentialdiagnosis 2 IgAnephropathylatent 24 48hnoedema hypertentionC3 normal Differentialdiagnosis 3 ChronicGNwithacuteepisode latent short 1 2days patients malnutritionanemiagrowthretardedrenalfunction usuallyabnormalproteinuria severegravityofurine Differentialdiagnosis 4 Idiopathicnephroticsyndrome ASO normalPathology Differentialdiagnosis 5 rapidlyprogressiveglomerulonephritis crescenticglomerulonephritis oliguriaoranuriarapidlossofrenalfuntionsecondaryGN LupusnephritisPurpuranephritisHBV associatedglomerulonephritis Treatment nospecifictreatmentsupportivecare Generaltreatment stayinbed 2 3weekssaltintake 60mg Kg dlimitproteinintake 0 5g Kg d azotemiaperiod Appropriateantibiotictherapy penicillinG 10 14daysotherantibiotics accordingtosensitivitytest Othertreatment Edema controlliquidandsaltdiureticsHypertention rest controlliquidandsaltNifedipineCaptopril Treatmentofseverecases 1 Severehypervolemia restricttheintakeofliquidandsaltloopdiuretics furosemidesodiumnitroprussidedialyticsupport Hypertensiveencephalopathy sodiumnitroprussidecontrolconvulsionAcuterenalfailure comprehensivetherapy Treatmentofseverecases 2 Prognosisandprevention recovery 95 severecasesmayprogressedtochronicGNandCRFprevention preventinfection NephroticSyndrome Definition NS massiveproteinuriahypoproteinemiaedemahyperlipidemia mayoccurasaresultofanyformofglomerulardiseasemaybeassociatedwithavarietyofextrarenalconditionsaccordingtoetiology primaryNS simpleandnephritictypeNSsecondaryNScongenitalNS usuallyaffectspre schoolchildrenthemostcommonage 3 5yearsmale female 3 7 1mostcommonformofnephropathy racialandgenomicandenviromentalbackground nephrin NPHS1 podocin steroidresponsiveNS associatewithHLA DR7frequentlyrelapse correlatewithHLA DR9steroidresistantNS NPHS2 EtiologyandPathogenesis unclearthechangeofformationandelectronofcapillaryglomerularpermeabilitytoprotein minimalchangeNS noimmunecomplexdepositionnegativechargeofbarrier injury cell mediatedimmunity non minimalchangeNS Igand orCdepositatglomerrulidamagethefiltratedbarrier Physiopathology Proteinuria themostbasicclinicalcharacterthefusionoffootprocessesofthevisceralepitheliumofGBMthenegativechargeglomrularpermeabilitytoprotein Hypoproteinemia reason thelossofalbumininurinesynthesisofalbuminnoproperintakingproteinserumalbuminisnegativelycorrelatedwiththeseverityofproteinuria Edema themostcommonsymptoms 1 hypoproteinemiaplasmaoncoticpressurehypovolemia2 serumalbumin 25g Lfluidintotheinterstitialspace 15g Lasites3 tubularsodiumreabsorption4 thedefectofNaarrangement5 serumADH Hyperlipidemia hypercholesterolemiahypertriglyceridemiasynthesislow densitylipoproteinscatabolismverylowdensitylipoproteinsdamagevasculatureinduceglomerulosclerosiscorrelatedwithhypoalbuminemiaproteinuria Pathology minimalchangeNS 76 4 membranoproliferativeGN 7 5 focalsegmentalglomerulosclerosis 6 9 mesangialproliferativeGN 2 3 focalglomerulosclerosis 1 7 membranousnephropathy 1 5 MCNS LightMicroscopy noglomerularlesionsminimalfocalsegmentalmesangialprominencethematrix noexpandedtotheextentthatcapillarylumensarecompromisedcapillarywalls thinandcapillarylumenspatentproteinandlipidresorptiondropletsintubularepithelialcellinterstitialedema rare MCNS ElectronMicroscopy theeffacementofvisceralepithelialcellfootprocessesmicrovilloustransformationglomerularandproximaltubularepithelialcellshaveincreasedclearanddensecytoplasmicdroplets MCNS ImmunofluoresensesMicroscopy noremarkablefindingslow levelmesangialstainingforIgM membranoproliferativeGN mesangialproliferativeGN membranousnephropathy ClinicalManifestation 1 cardinalclinicalfeature abruptonsetofedemaheavyproteinuriahypoalbuminemiahyperlipidemia ClinicalManifestation 2 hematuria unusualhypertention notcommontransientGFR 30 patientscausedbyhypovolemiathefunctionofrenal nomalARF rare Complications InfectionsElectrolitedisturbanceandhypovolemiaHypercoagulabilityandthrombosisAcuterenalfailureTubularfunctionlesionGrowthretarded Laboratoryfindings urineanalysis proteinuria 40mg h m2or 50mg kg dUpro Ucr 3 5microscopichematuria 15 patientshyalineandgranularcasts erythrocytesedimentationrate serumalbuminconcentration totalcholesteral LDL triglyceride serumcomplement usuallynormalothers plasmaviscosityRBCaggregation PLT fibrinogenplasminogenandantithrombinIIIrenalfunction usuallynormal Laboratoryfindings Diagnosis simpletypeNS proteinuria 40mg h m2or 50mg kg dhypoproteinemiaALB 30 Lhyperlipidemia TC 5 72mmol Lor220mg dledema Differentialdiagnosis 1 NephritictypeNSabovepoints atleast asfollowsurinetest dysmorphicredbloodcellRBC 10 HP 3times2weekshypertention schoolagechildren 130 90mmHgpre schoolagechildren 120 80mmHgrenalfunctioninsufficiencypersistentcomplement SecondaryNS APSGNLupusnephritisPurpuranephritisHBV associatedglomerulonephritis Differentialdiagnosis 2 Treatment Generaltreatment restinbeddietary salt1 2g dhighqualityprotein1 5 2g kg dVitD400u d Casevereedemaandhypertension limitliquidandsaltantihypertensiondiureticscontrolandpreventinfectionknowledgeeducation Corticosteroidtherapy Prednisone 2mg kg dmaximum60mg dprinciple enoughdosageslowlytaperlongremainVitD Calcium Newpatients shorttermtherapy 8weeksprednisone2mg kg d 4w1 5mg kg qod 4wmiddle longtermtherapy 6 9monthsprednisone1 5 2mg kg d 4 8wQOD 4wslowlytaper Sideeffectsofsteroid Alteredglucosemetabolism hyperglycemiaandglycosuriaCessationofgrowthCushingoidhabitusElevatedbloodpressureBehaviorandpersonalitychangesHypercoagulatorystate thrombosisAdrenalinsufficiencyInfectionOsteoporosis Someconcepts Steroid responsiveNS pred 8w Upro Steroid resistantNS pred 8w Upro Steroid dependentNS responsetosteroidtaperordiscontinued 1month relapse 2timesRelapse Upro Upro 2wFrequentlyrelapse 6monrelapse 2times 1yearrelapse 3times Frequentlyrelapseorsteroiddependentpatients adjustthesteroiddosageadjustperiodofsteroidchangetoothersteroid dexamethasonemethylprednisolone Immunosuppressivetherapy frequentlyrelapsesteroiddependentsteroidresistantserioussideeffectswithsteroidtherapyCyclophosphamide cyclosporinA Cyclophosphamide 2 2 5mg kg dTidpo 8 12w 200mg kg10 12mg kg div 2d 2w 150mg kg500mg m2iv mon 6 8m 200mg kgSideeffects bonemarrowsuppression WBCPLT liverfunctionhemorrhagiccystitisgonadaldysfunction Anticoagulant SodiumHeparin 1mg kg d2 4weeksivUrikinase 3 60000u d1 2weeksivDipyridamole 5 10mg kg dpoTid 6m Enhanceimmunefunction Levamisole 2 5mg kg dQod 6mImmunogloblin 400mg kg d 5days Angiotensinconvertingenzymeinhibitor ACEI CaptoprilEnalaprilFosinoprilTraditionalherbs Others Criteriaofrecovery clinicalcured stoptreatmentfor3yearstotalremissionnorelapsetotalremission laboratorytestisnomal partremission proteinuria Prognosis closelycorrelatedwithpathologyminimalchangeNS fine ClinicalfeaturesofthevarioustypesofpathologyofNS MCNSFSGSMPGNMN AgeMale femaleHematuriaBPSCrC3SensitivetosteriodPrognosisRecurrenceaftertransplantation 1 6everyage6 161 142 13 21 13 1AfewmanymanymanyAfewsomemanysomeAfewsomemanysome 68 93 25 Goodbadbadnotsobad some DifferencesbetweenchildrenandadultswithNS childrenadults24hUpro 50mg kg 3 5gpathologyMCNSMNetiolgyprimaryNSsecondaryNSresponsetosteroidmostwellsomewell ca