肾病综合征(英文)-儿科课件PPT课件

NephroticSyndromeDepartmentofPrdiatrics,TongjiHospitalNephroticSyndrome•Definition•Etiology•Pathology•Pathophysiology•ClinicalManifestation•LaboratoryData•Diagnosis•TherapyandPrognosisMale4yearsand6monthsoldComplaintofedemaandoliguriaDefinition:NephroticCriteria•Massiveproteinuria•ISKDC:40mg/m2/hr•CAN:+++trice/2wor50-100mg/kg/24hr•Mendoza:UrineProtein/Cr≥2.0•Hypoalbuminemia:30g/L•Hyperlipidemia:Cholesterol5.72mmol/L•EdemaDefinition:NephriticCriteria•Hematuria:RBC++(10/HP),trice/2w•Hypertension:•130/90mmHginchildrenover7y•120/80mmHgin3-6ychildren•110/70mmHgin3ychildren•Azotemia:BUN6.4mmol/L,Cr133umol/L•Hypocomplementemia:C30.8/LSecondaryNS:DIAMOND•Infection:APSGN,HBV,HIV,shuntnephropathy,refluxnephropathy,leprosy,syphilis,schistosomiasis,hydatiddisease•Drug,Toxic,Allegy:mercury,snakevenom,vaccine,pellicillamine,Heroin,gold,NSAID,captopril,probenecid,volatilehydrocarbons•Neoplasma:Hodgkin’sdisease,carcinoma(renalcell,lung,neuroblastoma,breast,andetc)•Autoimmuneorcollagen-vasculardiseases:SLE,Hashimoto’sthyroiditis,EMC,HSP,Vasculitis•GeneticDisease:Alportsyn.,Fabrysyn.,Nail-patellasyn.,Sicklecelldisease,Amyloidosis,Congenitalnephropathy•Metabolicdisease:Diabetesmellitus•Others:Chronictransplantrejection,congenitalnephrosclerosisPathology•PrimaryNS:–MinimalChangeNephropathy(MCN):80%–Mesangialproliferativeglomerulonephritis(MsPGN):10-12%–Focalsegmentalglomerulosclerosis(FSGS):5%–Others:MembranousNephropathy(MN)―mostcommoninadultsMembraneproliferativeglomerulonephritis(MPGN):2-5%Cresentglomerulonephritis:rare,Crescentglomeruli50%•SecondaryNS:dependentoncauses,APSGN,MN,FSGS,MsPGNorMPGNMinimalChangeNephropathy(MCN)•Littleornomicroscopicabnormality•Absenceofimmunecomplexes•Effacementofepithelialfootprocess•OccasionallymesangialhypercellurityMCN:normalinLMMCN:effacementofepithelialfootprocessinEMMesangialproliferativeglomerulonephritis(MsPGN)•Mesangialproliferationandexpansion•IgG,C3andsometimesIgA,IgMdepositsinmesangialstalk•Electron-densedepositsinmesangialorparamesangialareasMsPGN:MesangialproliferationandexpansionIgGandC3depositsinmesangialMesangialproliferativeglomerulonephritis(MsPGN)•Mesangialproliferationandexpansion•IgG,C3andsometimesIgA,IgMdepositsinmesangialstalk•Electron-densedepositsinmesangialorparamesangialareasFocalsegmentalglomerulosclerosis(FSGS)•Focalandsegmentalcapillarycollapseandmesangialsclerosis•DepositsofIgMorC3intheglomeruli•Lossofvisceralepithelialcellpodocytes,duplicationofthebasalGBMlamina,separationofepithelialcellfromGBMMembranousNephropathy(MN)•DiffuseGBMthickening,characteristicGBMspikes•SubepithelialdepositofIgGandC3Membraneproliferativeglomerulonephritis(MPGN)•Mesangialproliferationandexpansion•Subendothelialmesangialinterposition,tramtrackapperance•MesangialandsubendothelialdepositsofIgGandC3PathophysiologyofnephroticsyndromeMCN:Involvementofimmunesystem•NoIgorcomplementdeposit•AssociationbetweenallegyandidiopathicNS•Abnormalitiesofhumuralandcellularimmunity:IgG↓,IgA↓,CD4/CD8↓•RelapseofNStriggeredbyavarietyofminorinfections•Autologousremissionaftermeasles•InductionofremissionbycorticosteroidsandakylatingagentsMCN:pathogenesisofproteinuria•Lymphacyte→29kdpeptide→glomerularpolyanion↓→proteinuria•ConA→lymphacytes→60-160kdGPF→proteinuria•lymphacytes→13-18kdSIRS→proteinuriaGPF:glomerularpermeabilityfactorSIRS:solubleimmuneresponsesuppressorMCN:pathogenesisofedema•FFNa↓,CH2O↓Edema•ProteinuriaNareabsorptionindistalrenaltubulesNaandwaterretentionEdema•Hypoalbuminemiaintravascularoncoticpressure↓(25mmHg→6-8mmHg)FluidextravasationhypovolemiaADHandaldosteroneWaterandsaltretensionEdemaMCN:pathogenesisofhypoalbuminemia•Lossofproteinfromultrafiltration•Increasedcatabolismofproteininrenaltubules•Lossfromintestineprovedby51Chromium-albumintracingMCN:pathogenesisofhyperlipidemiaHypoalbuminemia→hypaticsynthesisoflipid↑→hyperlipidemiaClinicalManifestation•Simplenephrosis:2--7y,massiveedemainfaceandparaorbitalareas,ascites,pleuraleffusion,lossofappetite,nauseaandvomiting,inertiaandlethargy•Nephriticnephrosis:7y,moderateedema,grosshematuria,hypertension•Complications:Complications(1)•Infection:URI,peritonitis,cellulitisandetc–IgG,IgA,Complement–WBCfunction–LackofZnandothertraceelements•Hypercoagulablestateandthrombosis–HigherconcentrationofⅤ,Ⅶ,Ⅷ,Ⅹ–Lowerlevelofanticoagulantsubstance:antithrombinⅢ，proteinS,proteinC–Overvigorousdiuresis–Higherbloodviscosity,increasedplateletaggregation–RoleofcorticosteroidsComplications(2)•Electrolyteimbalance:hyponatrimia,hypokalemia,hypercalcemia–Salt-depleteddiet–Overvigorousdiuresis–Extrarenalloss–Protein-boundcalciumlossfromurine–Steroidsinducedhypocalcemia•ARF:pre-renal•Hypovolemicshock•Others:growthfailureLaboratoryData(1)•ESR:simplenephrosis100mm/h,nephriticnephrosis100mmHg•Serumpreoteinelectrophoresis:2,,insimplenephrosisbutinnephriticNephrosis•Immunoglobulin:IgG,IgA,IgM–IgAIgM,C3nephriticnephrosis–IgMIgA,normalC3simplenephrosisLaboratoryData(2)•Renalfunction:usuallynormal•Urineproteinpattern:–simplenephrosisalbu