腎病綜合征 英文課件

NephroticsyndromeFigure1.Nephroticedema.Figure2.Nephroticedema.ThemostcommonsyndromeofkidneydiseaseNephroticsyndromeNephriticsyndromeAsymptomaticurinaryabnormalitiesAcuterenalfailureorRapidlyprogressiverenalfailureChronickidneydisease(Table1)(一)腎病綜合征(二)腎炎綜合征(三)無癥狀性尿檢異常(四)急性及急進性腎衰竭綜合征(五)慢性腎臟病(表1)腎臟疾病常見綜合征Table1.STAGESOFCHRONICKIDNEYDISEASE*STAGEDESCRIPTIONGFR(mL/min/1.73m2)1Kidneydamagewithnormalor↑GFR≥902Kidneydamagewithmildor↓GFR60-893Moderate↓GFR30-594Severe↓GFR15-295Kidneyfailure<15(ordialysis)*ChronickidneydiseaseisdefinedaseitherkidneydamageorGFR<60mL/min/1.73m2for≥3months.Kidneydamageisdefinedaspathologicabnormalitiesormarkersofdamage,includingabnormalitiesinbloodorurinetestsorimagestudies.NephroticsyndromeThisischaracterizedbyproteinuria(Typically>3.5g/24h),hypoalbuminemia(lessthan30g/dL)andedema.Hyperlipidaemiaisalsopresent.PrimaryandsecondarycausesaresummarizedinTable2,3

Inpractice,manycliniciansreferto“nephroticrange”proteinuriaregardlessofwhethertheirpatientshavetheothermanifestationsofthefullsyndromebecausethelatterareconsequencesoftheproteinuria.NEPHROTICSYNDROME

PathophysiologyProteinuriaHypoalbuminemiaEdemaHyperlipidemia

Cause(diagnosisanddifferentialdiagnosis)Systemicrenaldisease

hepatitisBassociatedglomerulonephritis,Henoch-Schonlein

purpura,systemiclupuserythematosus,diatetesmellitus,amyloidosisIdiopathicnephroticsyndrome

ComplicationsInfectionCoagulationdisordersProteinmalnutritionanddyslipidemiaAcuterenalfailure

ProteinuriaProteinuriacanbecausedbysystemicoverproduction,tubulardysfunction,orglomerulardysfunction.Itisimportanttoidentifypatientsinwhomtheproteinuriaisamanifestationofsubstantialglomerulardiseaseasopposedtothosepatientswhohavebenigntransientorpostural(orthostatic)proteinuria.HypoalbuminemiaHypoalbuminemiaisinpartaconsequencesofurinaryproteinloss.Itisalsoduetothecatabolismoffilteredalbuminbytheproximaltubuleaswellastoredistributionofalbuminwithinthebody.Thisinpartaccountsfortheinexactrelationshipbetweenurinaryproteinloss,theleveloftheserumalbumin,andothersecondaryconsequencesofheavyalbuminuria.Insomepatients,however,theintravascularvolumehasbeenmeasuredandfoundtobeincreasedalongwithsuppressionoftheRASaxis.Ananimalmodelofunilateralproteinuriashowsevidenceofprimaryrenalsodiumretentionatadistalnephronsite,perhapsduetoalteredresponsivenesstohormonessuchasatrial

natriureticfactor.Hereonlytheproteinurickidneyretainssodiumandvolumeandatatimewhentheanimalisnotyethypoalbuminemic.Thus,localfactorswithinthekidneymayaccountforthevolumeretentionofthenephroticpatientaswell.EdemaFigure4.HyperlipidemiaMostnephroticpatientshaveelevatedlevelsoftotalandlow-densitylipoprotein(LDL)cholesterolwithlowornormalhigh-densitylipoprotein(HDL)cholesterol.Lipoprotein(a)[Lp(a)]levelsareelevatedaswellandreturntonormalwithremissionofthenephroticsyndrome.Nephroticpatientsoftenhaveahypercoagulablestateandarepredisposedtodeepveinthrombophlebitis,pulmonaryemboli,andrenalveinthrombosis.CauseTable2CAUSESOFTHENEPHROTICSYNDROMETable3bNEPHROTICSYNDROMEASSOCIATEDWITHSPECIFICCAUSES(“SECONDARY”NEPHROTICSYNDROME)PathologypatternsandclinicalpresentationsofidiopathicnephroticsyndomePRIMARYNEPHROTICSYNDROME

MinimalChangeDiseaseFocalSegmentalGlomerulosclerosisMembranousNephropathy

MembranoproliferativeGlomerulonephritis(MPGN)Figure5a.Pathologyofglomerulardisease.Lightmicroscopy.(a)Normalglomerulus;minimalchangedisease.PRIMARYNEPHROTICSYNDROMEMinimalChangeDisease

FocalSegmentalGlomerulosclerosisMembranousNephropathy

MembranoproliferativeGlomerulonephritis(MPGN)Figure5b.Segmentalsclerosis;focalsegmentalglomerulosclerosis.Figure6.Lightmicroscopicappearancesinfocalsegmentalglomerulosclerosis.Segmentalscarswithcapsularadhesionsinotherwisenormalglomeruli.PRIMARYNEPHROTICSYNDROMEMinimalChangeDiseaseFocalSegmentalGlomerulosclerosis

MembranousNephropathy

MembranoproliferativeGlomerulonephritis(MPGN)Figure7a.EarlyMN:aglomerulusfromapatientwithseverenephroticsyndromeandearlyMN,exhibitingnormalarchitectureandperipheralcapillarybasementmembranesofnormalthickness(Silver–methenamine×400).Figure7bmorphologicallyadvancedMNFigure7c.MorphologicallymoreadvancedMN(samepatientasin(b))Table6PRIMARYNEPHROTICSYNDROMEMinimalChangeDiseaseFocalSegmentalGlomerulosclerosisMembranousNephropathy

MembranoproliferativeGlomerulonephritis(MPGN)Figure8.PathologyofmembranoproliferativeglomerulonephritistypeI.(a)Lightmicroscopyshowsahypercellularglomeruluswithaccentuatedlobulararchitectureandasmallcellularcrescent(methenaminesilver).Table7DiagnosisandDifferentialdiagnosisInitialevaluationofthenephroticpatientincludeslaboratoryteststodefinewhetherthepatienthasprimary,idiopathicnephroticsyndromeorasecondarycauserelatedtoasystemicdisease.Commonscreeningtestsincludethefastingbloodsugarandglycosylatedhemoglobintestsfordiabetes,andantinuclearantibodytestforrheumatoiddisease,andtheserumcomplement,whichscreenformanyimmunecomplex-mediateddisease(Table3),Inselectedpatients,cryoglobulins,hepatitisBandCserology,anti-neutrophilcytoplasmicantibodies(ANCAS),antiGBMantibodies,andothertestsmaybeuseful.Oncesecondarycauseshavebeenexcluded,treatingtheadultnephroticpatientoftenrequiresarenalbiopsytodefinethe