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NephroticSyndromeDefinitionGlomerularpermeability↑Clinicalfeatures

MassiveproteinuriaHypoproteinemia

HyperlipidemiaEdemaEpidemiologyMale:female=3.7:1PreschoolClassificationPrimary/Idiopathic(90%)SecondarySLEHBVAnaphylactoidpurpuraCongenitalPathogenesisGlomerularpermeabilityProteinuriaHypoproteinemiaEdema

HyperlipidemiaApertureBarrierEndotheliumslitdiaphragmCharge-SelectiveBarrierEndotheliumEtiology肾小球毛细血管壁结构或电荷的变化可导致蛋白尿。高选择性蛋白尿低选择性蛋白尿病因及发病机制目前尚不明确局部免疫病理过程可损伤滤过膜正常屏障作用T淋巴细胞异常具有遗传基础PathophysiologyHypoproteinemiaHyperlipidemiaEdemaOthersMinimalchangedisease(MCD)(76.4%)Mesangialproliferativeglomerulonephritis(MsPGN)Focalsegmentalglomerulosclerosis(FSGS)Membranousnephrosis(MN)Membranoproliferativeglomerulonephritis(MPGN)PathologicalChangesDamageofGBMGenaralsituationEdema、Ascites、PleuraleffusionHematuriaBloodpressureRenalfunctionClinicalManifestationEdema、ascitesComplications

InfectionElectrolytedisorderandhypovolemiaHypercoagulabilityandthrombosisAcuterenalfailureRenaltubularfunctiondisorderInfectionManifestationsURISpontaneousperitonitis(自发性腹膜炎)TuberculosisCellulitis(蜂窝织炎)UrinarytractinfectionInfectionCausesImmunoglobulinandcomplementfactor↓ProteinmalnutritionEdemaImmunosuppressivetherapyManagementandProphylaxisHeightenourvigilancePromptevaluationEarlytherapyManifestationsHyponatremiaHypokalemiaHypocalcemiaHypovolemicshockElectrolytesDisorderandHypovolemiaCausesSaltintakerestrictionDiuretictreatmentVomit,Diarrhea,intestinalreabsorbtionLossofbindingproteinProphylaxisAvoidingaggressivediuretictherapyAppropriatesaltintakeElectrolytesDisorderandHypovolemiaHypercoagulabilityandThrombosisManifestationsBrainSkinLungKidneyScrotum(阴囊)

ExtremitiesHypercoagulabilityandThrombosisCausesCoagulationfactors↑Plateletaggregation↑AntithrombinⅢ↓HyperlipidemiaDiuretictherapySteroidtherapyHypercoagulabilityandThrombosisProphylaxisAvoidingpunctureofdeepveinsProphylacticanticoagulationdrugsManifestationsOliguriaoranuriaHypertensionElevatedserumCrandBUNlevelsAcuteRenalFailureCausesBloodvolume↓CrescentformationAcuteinterstitialnephritisDrugsProphylaxisAvoidinguseofrenaltoxicdrugsAvoidingaggressivediuretictherapyAcuteRenalFailureRenalTubularFunctionDisorderManifestationsRenalglycosuriaAminoaciduriaFanconisyndromUrineUrinalysis,24hurinaryprotein,UrinaryPro/CrSerumAlbumin,cholesterol,triglycerideIgG,IgA,IgMSodium,potassium,calciumCoagulationfunctionBUN,CrLaboratoryTestsC3ASO乙肝两对半系统性疾病的血清学检查UltrasonographyRenalbiopsyLaboratoryTestsDiagnosis24hurinaryprotein>50mg/(kg.d)Serumalbumin<30g/LSerumcholesterol>5.7mmol/LEdemaSimpletypeNephritictype

Classification

NephritictypeHematuriaHypertensionRenalfunctioninsufficientHypocomplementemiaClassificationDifferentialDiagnosisIsedemacausedbyrenaldiseases?Nephroticsyndrome?Simpletypeornephritictype?Primary,secondaryorcongenital?TreatmentGeneraltreatmentRestDiet:sodium,protein,calciumandvitaminDPreventandcureinfectionDiuresisEducationTreatmentSteroidPrednisonePrednisoloneImmunosupressivedrugsCTXCsAMMFFK506TreatmentAnticoagulanttherapyOthersACEIImmunologicregulatorsChinesemedicineTreatmentSideeffectsofcorticosteroidMetabolicdisturbanceHypertensionInfectionPepticulcerEuphoria,iunacy,induceepilepsy,insomniaOsteoporosis,growthretardationCataract(白内障)AdrenalinsufficiencyTreatmentIndicationsforcytotoxicdrugsFrequentrelapseSteroiddependentSteroidresistantUnabletotoleratesteroidtreatmentSideeffectsofCT

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