肾病综合征英文

NephroticSyndrome•DefinitionofNephroticsyndrome•Etiology•Pathogenesis•Clinicalpicture•Diagnosticworkup•Pathologicalpicture•Complication•Managements1.proteinuria(3.5g/day),2.hypoalbuminemia(30g/L)3.edema4.hyperlipidemiaTypesofnephroticsyndrome(1)Idiopathicnephroticsyndrome:Etiologyofthediseaseisunknown,accountingforapproximately90%ofnephrosisinchildhood.(2)Secondarynephrosis:NSresultedfromsystemicdiseasesuchasanaphylactoidpurpura,systemiclupusErythematosus(SLE),andsoon.EtiologyEtiologyEtiologyEtiologyEtiologyInchildrenMinimalchangediseaseispredominantInadultsSystemicdiseaserelated:30%Primaryrenaldisorders:70%MembranousnephropathyFocalglomerulosclerosisMinimalchangediseaseAmyloidosisInelderlyIncreasedincidenceofamyloidosisanddecreasedincidenceofSLEDAMAGEDProteinuria(1)Proteinuria:•Massiveproteinuriaisthemostchiefcharacteristicsofnephrosisresultingfromanincreaseinglomerularcapillarywallpermeabilitytoplasmaprotein.Themechanismmayberelatedto①Molecularbarrierinjury:holesonGBMbecomelarger;②Chargebarrierinjury:lossofnegative•(2)Hypoproteinemia•①Plasmaproteinislostbyurine;•②Proteincatabolism↑,sototalplasmaproteinconcentration↓,especiallyalbumin.(3)HyperlipidemiaAllserumlipid(cholesterol,triglycerides)andlipoproteinlevelsareelevated.•A.Hypoproteinemiastimulatesgeneralizedproteinsynthesisintheliver,includingthelipoprotein;•B.Lipidcatabolismisdiminished.(4)Edema•A.Hypoalbuminemialeadstoadecreaseinplasmaosmoticpressure,whichpermitsthetranslationoffluidfromintravascularcompartmenttointerstitialspace.•B.Theintravascularvolume↓makerenalperfusionpressure↓activatingrennin-angiotensin-aldosteronesystem,whichstimulatesdistaltubularreabsorptionofsodium↑.•C.Reducedintravascularvolumealsostimulatesthereleaseofantidiuretichormone,whichenhancesthereabsorptionofwaterinthecollectingduct.•D.Becauseofplasmaosmoticpressure↓,thesodiumandwaterenterinterstitialspacePathology•Inadults,thenephroticsyndromeisacommonconditionleadingtorenalbiopsy.Inmanystudies,patientswithheavyproteinuriaandthenephroticsyndromeshavebeenagrouphighlylikelytobenefitfromrenalbiopsyintermsofachangeinspecificdiagnosis,prognosis,andtherapy.•Selectedadultnephroticpatientssuchastheelderlyhaveaslightlydifferentspectrumofdisease,butagaintherenalbiopsyisthebestguidetotreatmentandprognosis(1)Minimalchangedisease(78%),theglomeruliappearnormal.Theepithelialcellfootprocessesfused.Morethan95%ofchildrenwithMCD,andbetterrespondingtocorticosteroidtherapy.•LightMicroscopy–Eithernormalorrevealsonlymildmesangialcellproliferation–EM–DiffusefusionoftheepithealialcellfootprocessesPathologyPathologyPathology•(2)FocalSegmentalGlomerulosclerosis(6.7%),sclerosisandhyalinosisinvolvingaportionofglomerulartuft,evenonlyoneoftheglomeruli,accompaniedtubularatrophy.IgMandC3withinscleroticareas.•(3)Mesangialproliferation:Onlymesangialproliferation.Immunoglobulinandcomplementdepositsinthemesangialarea.PathologyPathologySegmentalsclerosis;focalsegmentalglomerulosclerosis.Pathology•(4)Membranenephrosis:GBMthicker,immunecomplexdeposits.•(5)Membranoproliferativeglomerulonephritis:Diffuseproliferationofmesangialcellsandmesangialmatrix.ElectronicdensitydepositsandC3depositinmesangialandGBM.PathologySecondaryto:•DM(theleadingcauseofsecondarynephroticsyndrome)•SLE•Amyloidosis•Infections:HepatitisBandC,HIV,syphilis,post-streptococcal•Malignancy:multiplemyloma,Hodgkinlymphoma,solidtumor•Drugs(NSAIDs,gold,penicillamine,heavymetalsetc).Pathology•GeneralizedOdema-Thepredominantfeature-Theface,particularlytheperiorbitalarea,isswolleninthemorning&lowerextremitiesandgenitalarealaterintheday-Inadvanceddisease:thewholebody(anasarca)shortnessofbreath•Frothyurineandurinedipstickproteinuriavalueof3+•Symptoms&signsforsecondarycauseifpresent•24-hoururinecollection3,5g/day(nephrotic-rangeproteinuria)•ThehistoryandphysicalexaminationSystemicdisease•Serologicstudies(ANA),complement,hepatitisBandhepatitisCserologiesandthemeasurementofcryoglobulins,serumorurineproteinelectrophoresis.•Renalbiopsyrequiredtoestablishthediagnosisinmostoftimes.BUN,creatinine,creatininclearnce.bicarbonates,chlorideserumalbumin,serumproteins,calcium,Lipidprofile,Coagulationtests•AdultsRenalbiopsyismandatoryforeverynephroticpatientexceptthediabeticpatient.Inonestudyofadultswithnephroticrangeproteinuria,knowledgeofthehistologyalteredmanagement40%!•Childrenglucocorticoidtherapyisusuallybegunempiricallyandarenalbiopsyisperformedonlyforglucocorticoid-resistantdisease.-Managementofpatientswithnephroticsyndrome.Swissmedwkly2009;139(29-30):416-422.-Knowledgeofrenalhistologyalterspatientmanagementinover40percentofpatients.NephrolDialTransplant1994;9:1255.•10%ofnephroticsyndromecasesindiabetesareduetootherrenaldiseases*Presenceatypicalfeaturessuchas1-Arapidlyprogressivenephroticsyndrome2-Acuterenalfailure3-Presenceofglomerularhaematuriaand/orabsenceofassociatedmicrovascularlesions(retinopathy,neuropathy)Managementofpatientswithnephroticsyndrome.Swissmedwkly2009;139(29-30):416-422.36HistologicPatternKeyPathologicFeaturesMCD•LMNormal•EMFo