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1、NephroticSyndromeDepartmentofPrdiatrics,TongjiHospitalNephroticSyndromeDefinitionEtiologyPathologyPathophysiologyClinicalManifestationLaboratoryDataDiagnosisTherapyandPrognosisMale4yearsand6monthsoldComplaintofedemaandoliguriaDefinition:NephroticCriteriaMassiveproteinuriaISK
2、DC:>40mg/m2/hrCAN:>+++trice/2wor>50-100mg/kg/24hrMendoza:UrineProtein/Cr≥2.0Hypoalbuminemia:<30g/LHyperlipidemia:Cholesterol>5.72mmol/LEdemaDefinition:NephriticCriteriaHematuria:RBC++(>10/HP),trice/2wHypertension:>130/90mmHginchildrenover7y>120/80mmHgin3-6ychildren>110/70mmH
3、gin<3ychildrenAzotemia:BUN>6.4mmol/L,Cr>133umol/LHypocomplementemia:C3<0.8/LDefinitionNephriticNephrosis:nephroticcriteriawithatleastonenephriticcriteriaSimplenephrosis:nephroticcriteriawithoutnephriticcriteriaEtiologyCongenitalNS:rareIdiopathicNS:majoritySecondaryNS:manycau
4、sesespeciallyin<3yor>13ypatientscauses―DIAMONDSecondaryNS:DIAMONDInfection:APSGN,HBV,HIV,shuntnephropathy,refluxnephropathy,leprosy,syphilis,schistosomiasis,hydatiddiseaseDrug,Toxic,Allegy:mercury,snakevenom,vaccine,pellicillamine,Heroin,gold,NSAID,captopril,probenecid,volat
5、ilehydrocarbonsNeoplasma:Hodgkin’sdisease,carcinoma(renalcell,lung,neuroblastoma,breast,andetc)Autoimmuneorcollagen-vasculardiseases:SLE,Hashimoto’sthyroiditis,EMC,HSP,VasculitisGeneticDisease:Alportsyn.,Fabrysyn.,Nail-patellasyn.,Sicklecelldisease,Amyloidosis,Congenitalneph
6、ropathyMetabolicdisease:DiabetesmellitusOthers:Chronictransplantrejection,congenitalnephrosclerosisPathologyPrimaryNS:MinimalChangeNephropathy(MCN):80%Mesangialproliferativeglomerulonephritis(MsPGN):10-12%Focalsegmentalglomerulosclerosis(FSGS):5%Others:MembranousNephropathy(
7、MN)―mostcommoninadultsMembraneproliferativeglomerulonephritis(MPGN):2-5%Cresentglomerulonephritis:rare,Crescentglomeruli>50%SecondaryNS:dependentoncauses,APSGN,MN,FSGS,MsPGNorMPGNMinimalChangeNephropathy(MCN)LittleornomicroscopicabnormalityAbsenceofimmunecomplexesEffacemento
8、fepithelialfootprocessOccasionallymesangialhypercellurityMCN:normalinLMMCN:
