腎病綜合征-英文PPT

NephroticsyndromeFigure1.Nephroticedema.Figure2.Nephroticedema.ClinicalSyndrome腎臟及泌尿系疾病經(jīng)常會(huì)引起一些臨床癥狀、體征和實(shí)驗(yàn)室表現(xiàn)相似的綜合征。識(shí)別患者屬于哪一種綜合征對(duì)診斷很有幫助，因?yàn)閷?dǎo)致每個(gè)綜合征的病因較之其包含的個(gè)別臨床癥狀和體征的致病原因要少，故識(shí)別患者屬于哪一種綜合征對(duì)診斷有幫助。ThemostcommonsyndromeofkidneydiseaseNephroticsyndromeNephriticsyndromeAsymptomaticurinaryabnormalitiesAcuterenalfailureorRapidlyprogressiverenalfailureChronickidneydisease(Table1)

(一)腎病綜合征(二)腎炎綜合征(三)無(wú)癥狀性尿檢異常(四)急性及急進(jìn)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.Nephroticsyndrome

Thisischaracterizedbyproteinuria(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

PathophysiologyProteinuriaProteinuriacanbecausedbysystemicoverproduction,tubulardysfunction,orglomerulardysfunction.Itisimportanttoidentifypatientsinwhomtheproteinuriaisamanifestationofsubstantialglomerulardiseaseasopposedtothosepatientswhohavebenigntransientorpostural(orthostatic)proteinuria.Heavyproteinuria(albuminuria)Figure3.HypoalbuminemiaHypoalbuminemiaisinpartaconsequencesofurinaryproteinloss.Itisalsoduetothecatabolismoffilteredalbuminbytheproximaltubuleaswellastoredistributionofalbuminwithinthebody.Thisinpartaccountsfortheinexactrelationshipbetweenurinaryproteinloss,theleveloftheserumalbumin,andothersecondaryconsequencesofheavyalbuminuria.

ThesaltandvolumeretentionintheNSmayoccurthroughatleasttwodifferentmajormechanisms.Intheclassictheory,proteinurialeadstohypoalbuminemia,alowplasmaoncoticpressure,andintravascularvolumedepletion.Subequent

underperfusionofthekidneystimulatestheprimingofsodium-retentivehormonalsystemssuchastheRASaxis,causingincreasedrenalsodiumandvolumeretention,Intheperipheralcapillarieswithnormalhydrostaticpressuresanddecreasedoncoticpressure,theStarlingforcesleadtotranscapillaryfluidleakageandedema.EdemaInsomepatients,however,theintravascularvolumehasbeenmeasuredandfoundtobeincreasedalongwithsuppressionoftheRASaxis.Ananimalmodelofunilateralproteinuriashowsevidenceofprimaryrenalsodiumretentionatadistalnephronsite,perhapsduetoalteredresponsivenesstohormonessuchasatrial

natriureticfactor.Hereonlytheproteinurickidneyretainssodiumandvolumeandatatimewhentheanimalisnotyethypoalbuminemic.Thus,localfactorswithinthekidneymayaccountforthevolumeretentionofthenephroticpatientaswell.EdemaFigure4.HyperlipidemiaMostnephroticpatientshaveelevatedlevelsoftotalandlow-densitylipoprotein(LDL)cholesterolwithlowornormalhigh-densitylipoprotein(HDL)cholesterol.Lipoprotein(a)[Lp(a)]levelsareelevatedaswellandreturntonormalwithremissionofthenephroticsyndrome.Nephroticpatientsoftenhaveahypercoagulablestateandarepredisposedtodeepveinthrombophlebitis,pulmonaryemboli,andrenalveinthrombosis.CauseTable2CAUSESOFTHENEPHROTICSYNDROMETable3aNEPHROTICSYNDROMEASSOCIATEDWITHSPECIFICCAUSES(“SECONDARY”NEPHROTICSYNDROME)Table3bNEPHROTICSYNDROMEASSOCIATEDWITHSPECIFICCAUSES(“SECONDARY”NEPHROTICSYNDROME)

Pathologypatternsandclinicalpresentationsofidiopathicnephrotic

syndomeInadults,thenephroticsyndromeisacommonconditionleadingtorenalbiopsy.Inmanystudies,patientswithheavyproteinuriaandthenephroticsyndromeshavebeenagrouphighlylikelytobenefitfromrenalbiopsyintermsofachangeinspecificdiagnosis,prognosis,andtherapy.Selectedadultnephroticpatientssuchastheelderlyhaveaslightlydifferentspectrumofdisease,butagaintherenalbiopsyisthebestguidetotreatmentandprognosis(Table2,3).RenalbiopsyPRIMARYNEPHROTICSYNDROME

MinimalChangeDiseaseFocalSegmentalGlomerulosclerosisMembranousNephropathy

Membranoproliferative

Glomerulonephritis(MPGN)Figure5a.Pathologyofglomerulardisease.Lightmicroscopy.(a)Normalglomerulus;minimalchangedisease.Table4PRIMARYNEPHROTICSYNDROME

MinimalChangeDisease

FocalSegmentalGlomerulosclerosisMembranousNephropathy

Membranoproliferative

Glomerulonephritis(MPGN)Figure5b.Segmentalsclerosis;focalsegmentalglomerulosclerosis.Figure6.Lightmicroscopicappearancesinfocalsegmentalglomerulosclerosis.Segmentalscarswithcapsularadhesionsinotherwisenormalglomeruli.Table5PRIMARYNEPHROTICSYNDROME

MinimalChangeDiseaseFocalSegmentalGlomerulosclerosis

MembranousNephropathy

Membranoproliferative

Glomerulonephritis(MPGN)Figure7a.EarlyMN:aglomerulusfromapatientwithseverenephroticsyndromeandearlyMN,exhibitingnormalarchitectureandperipheralcapillarybasementmembranesofnormalthickness(Silver–methenamine×400).Figure7bmorphologicallyadvancedMNFigure7c.MorphologicallymoreadvancedMN(samepatientasin(b))Table6PRIMARYNEPHROTICSYNDROME

MinimalChangeDiseaseFocalSegmentalGlomerulosclerosisMembranousNephropathy

Membranoproliferative

Glomerulonephritis(MPGN)Figure8.Pathologyofmembranoproliferative

glomerulonephritistypeI.(a)Lightmicroscopyshowsahypercellular

glomeruluswithaccentuatedlobulararchitectureandasmallcellularcrescent(methenaminesilver).Table7DiagnosisandDifferentialdiagnosis

Initialevaluationofthenephroticpatientincludeslaboratoryteststodefinewhetherthepatienthasprimary,idiopathicnephroticsyndromeorasecondarycauserelatedtoasystemicdisease.Commonscreeningtestsincludethefastingbloodsugarandglycosylatedhemoglobintestsfordiabetes,andantinuclearantibodytestforrheumatoiddisease,andtheserumcomplement,whichscreenformanyimmunecomplex-mediateddisease(Table3),Inselectedpatients,cryoglobulins,hepatitisBandCserology,anti-neutrophil

cytoplasmicantibodies(ANCAS),antiGBMantibodies,andothertest