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Background
Nephroticsyndromeiskidneydiseasewithproteinuria,hypoalbuminemia,and
edema.Nephroticrangeproteinuriais3gramsperdayormore.Onasinglespot
urinecollection,itis2gofproteinpergramofurinecreatinine.
Therearemanyspecificcausesofnephroticsyndrome.Theseincludekidney
diseasessuchasminimalchangenephropathy,focalglomerulosclerosis,and
membranousnephropathy.Nephroticsyndromecanalsoresultfromsystemic
diseasesthataffectotherorgansinadditiontothekidneys,suchasdiabetes,
amyloidosis,andlupuserythematosus.
Nephroticsyndromemayaffectadultsandchildren,ofbothsexesandofanyrace.
Itmayoccurintypicalform,orinassociationwithnephriticsyndrome.Thelatter
connotesglomerularinflammation,withhematuriaandimpairedkidneyfunction.

Classification
Nephroticsyndromecanbeprimary,beingadiseasespecifictothekidneys,orit
canbesecondary,beingarenalmanifestationofasystemicgeneralillness.Inall
cases,injurytoglomeruliisanessentialfeature.
Primarycausesofnephroticsyndromeincludethefollowing,inapproximateorderof
frequency:
Minimalchangenephropathy
Focalglomerulosclerosis
Membranousnephropathy
Hereditarynephropathies
Secondarycausesincludethefollowing,againinorderofapproximatefrequency:
Diabetesmellitus
Lupuserythematosus
Amyloidosisandparaproteinemias
Viralinfections(eg,hepatitisB,hepatitisC,humanimmunodeficiencyvirus[
HIV])
Preeclampsia
Nephroticrangeproteinuriamayoccurinotherkidneydiseases,suchasIgA
nephropathy.Inthatcommonglomerulardisease,onethirdofpatientsmayhave
nephroticrangeproteinuria. [1]
Nephroticsyndromemayoccurinpersonswithsicklecelldiseaseandevolveto
renalfailure.Membranousnephropathymaycomplicatebonemarrow
transplantation,inassociationwithgraftversushostdisease.
Kidneydiseasesthataffecttubulesandinterstitium,suchasinterstitialnephritis,
willnotcausenephroticsyndrome.
Theabovecausesofnephroticsyndromearelargelythoseforadults,andthis
articlewillconcentrateprimarilyonadultnephroticsyndrome.However,nephrotic
syndromeininfancyandchildhoodisanimportantentity.Fordiscussionofthis
topic,seetheMedscapeReferencearticlePediatricNephroticSyndrome.
Fromatherapeuticperspective,nephroticsyndromemaybeclassifiedassteroid
sensitive,steroidresistant,steroiddependent,orfrequentlyrelapsing.
Corticosteroids(prednisone),cyclophosphamide,andcyclosporineareusedto
induceremissioninnephroticsyndrome.Diureticsareusedtoreduceedema.
Angiotensinconvertingenzyme(ACE)inhibitorsandangiotensinIIreceptorblockers
areadministeredtoreduceproteinuria.(SeeTreatmentandMedication.)

Pathophysiology
Inahealthyindividual,lessthan0.1%ofplasmaalbuminmaytraversethe
glomerularfiltrationbarrier. [2]Controversyexistsregardingthesievingofalbumin
acrosstheglomerularpermeabilitybarrier.Onthebasisofstudiesinexperimental
animals,ithasbeenproposedthatthereisongoingalbuminpassageintotheurine,
inmanygramsperday,withequivalentsubstantialtubularuptakeofalbumin,the
resultbeingthattheurinehas80mgperdayorlessofdailyalbumin. [3]
However,studiesofhumanswithtubulartransportdefectssuggestthatthe
glomerularurinaryspacealbuminconcentrationis3.5mg/L. [4]Withthis
concentration,andanormaldailyglomerularfiltrationrate(GFR)of150liters,one
wouldexpectnomorethan525mgperdayofalbumininthefinalurine.Amounts
abovethatlevelpointtoglomerulardisease.
Theglomerularcapillariesarelinedbyafenestratedendotheliumthatsitsonthe
glomerularbasementmembrane,whichinturniscoveredbyglomerularepithelium,
orpodocytes,whichenvelopsthecapillarieswithcellularextensionscalledfoot
processes.Inbetweenthefootprocessesarethefiltrationslits.These3structures

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thefenestratedendothelium,glomerularbasementmembrane,andglomerular
epitheliumaretheglomerularfiltrationbarrier.Aschematicdrawingofthe
glomerularbarrierisprovidedintheimagebelow.

Schematicdrawingoftheglomerularbarrier.Podo=podocytesGBM=glomerularbasement
membraneEndo=fenestratedendothelialcellsESL=endothelialcellsurfacelayer(often
referredtoastheglycocalyx).Primaryurineisformedthroughthefiltrationofplasmafluid
acrosstheglomerularbarrier(arrows)inhumans,theglomerularfiltrationrate(GFR)is125
mL/min.Theplasmaflowrate(Qp)iscloseto700mL/min,withthefiltrationfractionbeing20%.
Theconcentrationofalbumininserumis40g/L,whiletheestimatedconcentrationofalbuminin
primaryurineis4mg/L,or0.1%ofitsconcentrationinplasma.ReproducedfromHaraldssonet
al,PhysiolRev88:451487,2008,andbypermissionoftheAmericanPhysiologicalSociety
(www.theaps.org).

Filtrationofplasmawaterandsolutesisextracellularandoccursthroughthe
endothelialfenestraeandfiltrationslits.Theimportanceofthepodocytesandthe
filtrationslitsisshownbygeneticdiseases.Thus,incongenitalnephroticsyndrome
oftheFinnishtype,thegenefornephrin,aproteinofthefiltrationslit,ismutated,
leadingtonephroticsyndromeininfancy.Similarly,podocin,aproteinofthe
podocytes,maybeabnormalinanumberofchildrenwithsteroidresistantfocal
glomerulosclerosis.
Theglomerularstructuralchangesthatmaycauseproteinuriaaredamagetothe
endothelialsurface,theglomerularbasementmembrane,orthepodocytes.Oneor
moreofthesemechanismsmaybeseeninanyonetypeofnephroticsyndrome.
Albuminuriaalonemayoccur,or,withgreaterinjury,leakageofallplasmaproteins,
(ie,proteinuria)maytakeplace.
Proteinuriathatismorethan85%albuminisselectiveproteinuria.Albuminhasa
netnegativecharge,anditisproposedthatlossofglomerularmembranenegative
chargescouldbeimportantincausingalbuminuria.Nonselectiveproteinuria,being
aglomerularleakageofallplasmaproteins,wouldnotinvolvechangesin
glomerularnetchargebutratherageneralizeddefectinpermeability.Thisconstruct
doesnotpermitclearcutseparationofcausesofproteinuria,exceptinminimal
changenephropathy,inwhichproteinuriaisselective.

Pathogenesisofedema
Anincreaseinglomerularpermeabilityleadstoalbuminuriaandeventuallyto
hypoalbuminemia.Inturn,hypoalbuminemialowerstheplasmacolloidosmotic
pressure,causinggreatertranscapillaryfiltrationofwaterthroughoutthebodyand
thusthedevelopmentofedema.
Capillaryhydrostaticpressureandthegradientofplasmatointerstitialfluidoncotic
pressuredeterminethemovementoffluidfromthevascularcompartmenttothe
interstitium.Theoncoticpressureismainlydeterminedbytheproteincontent.The
fluxofwateracrossthecapillarywallcanbeexpressedbythefollowingformula:
Qw=K([P cP i][pppi]
Inthisformula,Qwisnetfluxofwater,Kisthecapillaryfiltrationcoefficient,P cis
capillaryhydrostaticpressure,andP iistheinterstitialfluidhydrostaticpressure,
whileppistheplasmaoncoticpressure,andpiistheinterstitialfluidoncotic
pressure.
Withahighenoughcapillaryhydrostaticpressureoralowenoughintravascular
oncoticpressure,theamountoffluidfilteredexceedsthemaximallymphaticflow,
andedemaoccurs.Inpatientswithnephroticsyndrome,thiscausesareductionin
plasmavolume,withasecondaryincreaseofsodiumandwaterretentionbythe
kidneys.
Analternativehypothesisisthataconditionofrenalsodiumretentionoccurs
becauseoftheproteinuria,butthisisnotrelatedtointravascularvolumeorto
serumproteinconcentration.Theevidencesupportingthissocalledoverfill
hypothesisincludesthefactsthat(1)sodiumretentionisobservedevenbeforethe
serumalbuminlevelstartsfallingand(2)intravascularvolumeisnormaloreven
increasedinmostpatientswithnephroticsyndrome.Thiscouldoccurifintraluminal
proteindirectlystimulatedrenalepithelialsodiumreabsorption. [5]
Athirdpossiblemechanismisanenhancedperipheralcapillarypermeabilityto
albumin,asshownbyradioisotopictechniqueinhumanstudiesof60patientswith
nephroticsyndrome. [6]Thiswouldthenleadtoincreasedtissueoncoticpressure

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andfluidretentionintheperipheraltissues.

Metabolicconsequencesofproteinuria
Metabolicconsequencesofthenephroticsyndromeincludethefollowing:

NephroticSyndrome

Infection
Hyperlipidemiaandatherosclerosis
Author:EricPCohen,MDChiefEditor:VecihiBatuman,MD,FACP,FASNmore...
Hypocalcemiaandboneabnormalities
Hypercoagulability
Updated:Dec09,2014
Hypovolemia
Acuterenalfailuremayindicateanunderlyingglomerulonephritisbutismoreoften
precipitatedbyhypovolemiaorsepsis.Edemaofthekidneysthatcausesa
pressuremediatedreductionintheGFRhasalsobeenhypothesized.
Hypertensionrelatedtofluidretentionandreducedkidneyfunctionmayoccur.
Failuretothrivemaydevelopinpatientswithchronicedema,includingascitesand
pleuraleffusion.Failuretothrivemaybecausedbyanorexia,hypoproteinemia,
increasedproteincatabolism,orfrequentinfectiouscomplications.Edemaofthe
gutmaycausedefectiveabsorption,leadingtochronicmalnutrition.
Infection
Infectionisamajorconcerninnephroticsyndromepatientshaveanincreased
susceptibilitytoinfectionwithStreptococcuspneumoniae,Haemophilusinfluenzae,
Escherichiacoli,andothergramnegativeorganisms.Varicellainfectionisalso
common.Themostcommoninfectiouscomplicationsarebacterialsepsis,cellulitis,
pneumonia,andperitonitis.
Proposedexplanationsincludethefollowing:
Urinaryimmunoglobulinlosses
Edemafluidactingasaculturemedium
Proteindeficiency
Decreasedbactericidalactivityoftheleukocytes
Immunosuppressivetherapy
Decreasedperfusionofthespleencausedbyhypovolemia
Urinarylossofacomplementfactor(properdinfactorB)thatopsonizes
certainbacteria
Hyperlipidemiaandatherosclerosis
Hyperlipidemiamaybeconsideredatypicalfeatureofthenephroticsyndrome,
ratherthanamerecomplication.Itisrelatedtothehypoproteinemiaandlowserum
oncoticpressureofnephroticsyndrome,whichthenleadstoreactivehepaticprotein
synthesis,includingoflipoproteins. [7]Inaddition,reducedplasmalevelsof
lipoproteinlipaseresultsindiminutionoflipidcatabolism.Someoftheelevated
serumlipoproteinsarefilteredattheglomerulus,leadingtolipiduriaandthe
classicalfindingsofovalfatbodiesandfattycastsintheurinesediment.
Atheroscleroticvasculardiseaseappearstooccuringreaterfrequencyinpersons
withnephroticsyndromethaninhealthypersonsofthesameage.Curryand
Robertsshowedthatthefrequencyandextentofcoronaryarterydiseasestenoses
weregreaterinpatientswithnephroticsyndromethaninnonnephroticcontrol
subjects. [8]
Whentheirstudywaspublished,in1977,lipidloweringtreatmentswerelesswidely
usedthantheyaretoday.Accordingly,theaveragehighestserumtotalcholesterol
inthisserieswasover400mg/dL.Thatisintherangeofserumcholesterolseenin
familialhypercholesterolemia,adiseasethatpredisposesindividualstomyocardial
infarction.
Hypocalcemia
Hypocalcemiaiscommoninthenephroticsyndrome,butratherthanbeingatrue
hypocalcemia,itisusuallycausedbyalowserumalbuminlevel.Nonetheless,low
bonedensityandabnormalbonehistologyarereportedinassociationwithnephrotic
syndrome.ThiscouldbecausedbyurinarylossesofvitaminDbindingproteins,
withconsequenthypovitaminosisDand,asaresult,reducedintestinalcalcium
absorption. [9]
TessitoreetalreportedthatwhentheGFRwasnormal,personswiththenephrotic
syndromehadnoconsistentcalciumorbonyabnormalities. [10]Yetinthatsame
study,whentheGFRwasreduced,bonemineralizationdefectswerefoundby
biopsy.Alaterstudyfoundosteomalaciaonbonebiopsyinoverhalfofadultswho
hadlongstandingnephroticsyndromebutwhoseGFRwaspreserved. [9]
Afurthercomplicationderivesfromtherapies,especiallyprednisoneuse.Lowbone
massmaybefoundinrelationtocumulativesteroiddose. [11]Thissubjectremains
controversialasreportedbyLeonardetal,intermittentcorticosteroidtreatmentof
childhoodsteroidsensitivenephroticsyndromedoesnotappeartobeassociated
withbonemineraldeficits. [12]Itispossiblethatlongdurationofeitherthenephrotic
syndromeortreatmentsforitaretheimportantriskfactorsforbonediseasein
thesepatients.
Hypercoagulability

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Venousthrombosisandpulmonaryembolismarewellknowncomplicationsofthe
nephroticsyndrome.Hypercoagulabilityinthesecasesappearstoderivefrom
urinarylossofanticoagulantproteins,suchasantithrombinIIIandplasminogen,
alongwiththesimultaneousincreaseinclottingfactors,especiallyfactorsI,VII,
VIII,andX.
AstudybyMahmoodietalofalmost300patientswithnephroticsyndrome
confirmedthatvenousthromboembolism(VTE)wasalmost10timeshigherin
thesepersonsthaninthenormalpopulation(1%vs0.10.2%). [13]Moreover,that
riskappearedespeciallyelevatedduringthefirst6monthsofnephroticsyndrome,
beingatalmost10%.Thishighincidencemayjustifytheroutineuseofpreventive
anticoagulationtreatmentduringthefirst6monthsofapersistentnephrotic
syndrome.
Mahmoodi'sstudyalsoshowedanincreasedriskofarterialthromboticevents,
includingcoronaryandcerebrovascularones,innephroticsyndrome.Unliketherisk
ofVTE,whichwasrelatedtoproteinuria,thisarterialriskwasrelatedtousualrisk
factorsforarterialdisease,suchashypertension,diabetes,smoking,andreduced
GFR.
Hypovolemia
Hypovolemiaoccurswhenhypoalbuminemiadecreasestheplasmaoncotic
pressure,resultinginalossofplasmawaterintotheinterstitiumandcausinga
decreaseincirculatingbloodvolume.Hypovolemiaisgenerallyobservedonlywhen
thepatient'sserumalbuminlevelislessthan1.5g/dL.Symptomsincludevomiting,
abdominalpain,anddiarrhea.Thesignsincludecoldhandsandfeet,delayed
capillaryfilling,oliguria,andtachycardia.Hypotensionisalatefeature.

Etiology
Commonprimarycausesofnephroticsyndromeincludekidneydiseasessuchas
minimalchangenephropathy,membranousnephropathy,andfocal
glomerulosclerosis.Secondarycausesincludesystemicdiseasessuchasdiabetes
mellitus,lupuserythematosus,andamyloidosis.Congenitalandhereditaryfocal
glomerulosclerosismayresultfrommutationsofgenesthatcodeforpodocyte
proteins,includingnephrin,podocin,orthecationchannel6protein.Nephrotic
syndromecanresultfromdrugsofabuse,suchasheroin.
Nephroticrangeproteinuriaoccurringinthethirdtrimesterofpregnancyisthe
classicalfindingofpreeclampsia.Inthatcondition,alsoknownastoxemia,
hypertensiondevelopsaswell.Itmayoccurdenovooritmaybesuperimposedon
anotherchronickidneydisease.Inthelattercase,therewillhavebeenpreexisting
proteinuriathatwillhaveworsenedduringpregnancy.
Medicationcancausenephroticsyndrome.Thisincludestheveryinfrequent
occurrenceofminimalchangenephropathywithNSAIDuse,andtheoccurrenceof
membranousnephropathywiththeadministrationofgoldandpenicillamine,which
areolderdrugsusedforrheumaticdiseasestherehavealsobeenreportsoffocal
glomerulosclerosisinassociationwithintravenousbisphosphonates.Lithiumand
interferontherapyalsoareimplicatedinfocalglomerulosclerosisofthecollapsing
type.
Nephroticrangeproteinuriacouldoccurwiththeuseofanticanceragents,suchas
bevacizumab,thatinhibitvascularendothelialgrowthfactor(VEGF). [14]However,
theclinicalpictureofthiscomplicationisofathromboticmicroangiopathyrather
thanofnephroticsyndromeperse.
Theassociationofmembranousnephropathywithcancerisaclinicaldilemma.This
associationpresumablyresultsfromimmunecomplexinjurytotheglomerulus
causedbycancerantigens.
Whilethereareabout6000newcasesofmembranousnephropathyperyearinthe
UnitedStates,thereare1.5millionnewcasesofnonskincancer.Therefore,from
theoncologistsstandpoint,theproblemofparaneoplasticmembranous
nephropathyistrivial.
Nonetheless,acarefullyperformedanalysisfromFrancesuggestedthatthecancer
rateinpersonswithmembranousnephropathyisapproximately10foldhigherthan
itisinthegeneralpopulation,especiallyinindividualsoverage65years. [15]Inthat
study,50%ofmembranousnephropathycaseswerediagnosedbeforethediagnosis
ofcancer.Thus,insomepatientswithmembranousnephropathy,oneshould
considerthepossibilityofanundiagnosedcancer.

Epidemiology
UnitedStatesstatistics
Thefigurebelowshowstheincidencepermillionpopulationofimportantcausesof
nephroticsyndrome.Diabeticnephropathywithnephroticsyndromeismost
common,atanestimatedrateofatleast50casespermillionpopulation.Thatisan
underestimation,however,sincetherateofendstagerenaldiseasefromdiabetes
hasreached100casespermillionpopulationinsomeWesterncountries.In
children,nephroticsyndromemayoccuratarateof20casespermillionchildren.
[16]

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Incidenceofimportantcausesofnephroticsyndrome,innumberpermillionpopulation.Theleft
panelshowssystemiccauses,andtherightpanellistsprimaryrenaldiseasesthatcancause
nephroticsyndrome.fgs=focalglomerulosclerosis,MN=membranousnephropathy,min
change=minimalchangenephropathy.DataareinpartfromSwaminathanetalandBergesioet
al.

Internationalstatistics
Biopsystudiesinchildrenwithnephroticsyndromehaveshownsimilartypesof
histologyinIndiaandTurkey,comparedwithwhatonewouldexpectinWestern
countries. [17,18]InPakistaniadultswithnephroticsyndrome,thespectrumof
histologiesofkidneybiopsieshasbeenfoundtobesimilartothatseeninwestern
countries. [19]
InpartsofAfricaandtheMiddleEast(eg,Egypt),glomerulardiseasemaybe
associatedwithurogenitalschistosomalinfection. [20]However,socalled"tropical
nephroticsyndrome"(eg,fromparasiticdiseasessuchasschistosomiasisor
malaria)maynotbeatrueentity.
DoeetalreviewedcausesofnephroticsyndromeinAfricanchildrenandfoundno
evidenceforadominatingroleofsteroidresistanttropicalglomerulopathiesrather,
kidneybiopsymostoftenshowedtypicalhistologicfindings(focalandsegmental
glomerulosclerosisandminimalchangedisease). [21]
Theconnectionofnephroticsyndrometoquartanmalariaisnotwellestablished.
Indeed,PakasaandSumailicallattentiontotheapparentdeclineofparasite
associatednephroticsyndromeintheCongo. [22,23]Itispossiblethattheperceived
associationbetweennephroticsyndromeandparasiticinfectionswascoincidental,
assupportedbytheongoingandprobablyincreasingoccurrenceofchronickidney
diseaseintheCongo. [23]

Race,sex,andagerelateddemographics
Becausediabetesismajorcauseofnephroticsyndrome,AmericanIndians,
Hispanics,andAfricanAmericanshaveahigherincidenceofnephroticsyndrome
thandowhitepersons.HIVnephropathyisacomplicationofHIVinfectionthatis
unusualinwhitesitisseenwithgreaterfrequencyinAfricanAmericans. [24]Focal
glomerulosclerosisappearstobeoverrepresentedinAfricanAmericanchildren,as
comparedwithwhitechildren,asacauseofnephroticsyndrome. [25]
Thereisamalepredominanceintheoccurrenceofnephroticsyndrome,asthereis
forchronickidneydiseaseingeneral.Thismaleoverrepresentationisalsoseenin
paraneoplasticmembranousnephropathy. [15]However,lupusnephritisaffects
mostlywomen.
Theimagebelowshowstypicalagesatwhichagivencauseofnephroticsyndrome
mayoccur.Itdoesnotshoweverypossiblecauseofnephroticsyndrome,suchas
lupusnephritis,whichtypicallyaffectsyoungblackwomen.Theagesshownare
averages.

Aschemaoftheaveragepatientagesassociatedwithvariouscommonformsofnephrotic
syndrome.

Prognosis
Inthepreantibioticera,infectionwasamajorfactorinthemortalityrateamong
patientswithnephroticsyndrome. [26]Treatmentsfornephroticsyndromeandits
complicationsappeartohavereducedthemorbidityandmortalityonceassociated
withthesyndrome.Currently,theprognosisforpatientswithprimarynephrotic
syndromedependsonitscause.

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Infantswithcongenitalnephroticsyndromehaveadismalprognosis:survival
beyondseveralmonthsispossibleonlywithdialysisandkidneytransplantation.
Onlyapproximately20%ofpatientswithfocalglomerulosclerosisundergoremission
ofproteinuriaanadditional10%improvebutremainproteinuric.Manypatients
experiencefrequentrelapses,becomesteroiddependent,orbecomesteroid
resistant.Endstagerenaldiseasedevelopsin2530%ofpatientswithfocal
segmentalglomerulosclerosisby5yearsandin3040%ofthesepatientsby10
years.
Theprognosisforpatientswithminimalchangenephropathyisverygood.Most
childrenrespondtosteroidtherapystill,about50%ofchildrenhave1or2relapses
within5yearsandapproximately20%ofthemcontinuetorelapse10yearsafter
diagnosis.Only30%ofchildrenneverhavearelapseaftertheinitialepisode.
Approximately3%ofpatientswhoinitiallyrespondtosteroidsbecomesteroid
resistant.
Adultswithminimalchangenephropathyhaveaburdenofrelapsesimilartothatof
children.However,thelongtermprognosisforkidneyfunctioninpatientswiththis
diseaseisexcellent,withlittleriskofrenalfailure.
Poorpatientresponsetosteroidtherapymaypredictapooroutcome.Childrenwho
presentwithhematuriaandhypertensionaremorelikelytobesteroidresistantand
haveapoorerprognosisthanarethosewhodonotpresentwiththeseconditions.
AstudybyDonadioetalof140patientswithidiopathicmembranousnephropathy,
89ofwhomreceivednotreatmentwithcorticosteroidsorimmunosuppressivedrugs
and51ofwhomweretreatedprimarilywithshorttermcoursesofprednisonealone,
foundthatsurvivalratesinthesepatientswerethesameasthoseexpectedforthe
generalpopulation. [27]
Theprognosismayworsenbecauseof(1)anincreasedincidenceofrenalfailure
andthecomplicationssecondarytonephroticsyndrome,includingthrombotic
episodesandinfection,or(2)treatmentrelatedconditions,suchasinfectious
complicationsofimmunosuppressivetreatments.
Insecondarynephroticsyndromes,morbidityandmortalityarerelatedtothe
primarydiseaseprocess(eg,diabetes,lupus,amyloidosis).Indiabeticnephropathy,
however,themagnitudeofproteinuriaitselfrelatesdirectlytomortality. [28]
Indiabeticnephropathywithnephroticsyndrome,thereisusuallyagoodresponse
toangiotensinblockade,withreductionofproteinuriatolow,subnephroticlevels.
Trueremissionisuncommon,however.Cardiovascularmorbidityandmortality
increaseaskidneyfunctiondeclines,andsomepatientswilleventuallyneeddialysis
orakidneytransplant.
Inprimaryamyloidosis,prognosisisnotgood,evenwithintensivechemotherapy.In
secondaryamyloidosis,remissionoftheunderlyingcause,suchasrheumatoid
arthritis,isfollowedbyremissionoftheamyloidosisanditsassociatednephrotic
syndrome.

PatientEducation
Pediatricnephroticsyndromeisachronicillnesscharacterizedbyrelapsesand
remissions,whichcanextendthroughoutchildhood.Therewillbeillnessfromthe
diseaseandfromitstreatment.Parentsmaymonitortheirchild'surineandrecord
theresultsinadiary.Thediarycanalsobeusedtowritedownanagreeduponplan
forthemanagementofrelapses.Informationbookletsshouldbegiventothefamily.
Peersupportandpsychologicalcounselingmaybehelpfulforsomefamilies.
Progressiontorenalfailurewillrequirepreparationfordialysisand/orkidney
transplantation.Similarly,adultswithformsofnephroticsyndromethatcannotbe
curedmayprogresstorenalfailureandtheneedfordialysisorkidney
transplantation.
ClinicalPresentation

ContributorInformationandDisclosures
Author
EricPCohen,MDProfessor,DepartmentofMedicine,DivisionofNephrology,MedicalCollegeofWisconsin
NephrologySectionChief,ZablockiVeteransAffairsHospital
EricPCohen,MDisamemberofthefollowingmedicalsocieties:AmericanSocietyofNephrology,Radiation
ResearchSociety,CentralSocietyforClinicalandTranslationalResearch,InternationalSocietyofNephrology
Disclosure:Nothingtodisclose.
Coauthor(s)
RamapriyaSinnakirouchenan,MDAssistantProfessor,DivisionofNephrology,MedicalCollegeofWisconsin
RamapriyaSinnakirouchenan,MDisamemberofthefollowingmedicalsocieties:AmericanSocietyof
Nephrology,IndianMedicalAssociation
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedicalCenterCollege

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ofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:ReceivedsalaryfromMedscapeforemployment.for:Medscape.
EleanorLederer,MDProfessorofMedicine,Chief,NephrologyDivision,Director,NephrologyTrainingProgram,
Director,MetabolicStoneClinic,KidneyDiseaseProgram,UniversityofLouisvilleSchoolofMedicineConsulting
Staff,LouisvilleVeteransAffairsHospital
EleanorLederer,MDisamemberofthefollowingmedicalsocieties:AmericanAssociationfortheAdvancement
ofScience,InternationalSocietyofNephrology,AmericanSocietyforBiochemistryandMolecularBiology,
AmericanFederationforMedicalResearch,AmericanSocietyforBoneandMineralResearch,AmericanSociety
ofNephrology,AmericanSocietyofTransplantation,KentuckyMedicalAssociation,NationalKidneyFoundation,
PhiBetaKappa
Disclosure:Receivedgrant/researchfundsfromDeptofVeteransAffairsforresearchReceivedsalaryfrom
AmericanSocietyofNephrologyforasncouncilpositionReceivedsalaryfromUniversityofLouisvillefor
employmentReceivedsalaryfromUniversityofLouisvillePhysiciansforemploymentReceivedcontract
paymentfromAmericanPhysicianInstituteforAdvancedProfessionalStudies,LLCforindependentcontractor
ReceivedcontractpaymentfromHealthcareQualityStrategies,Incforindependentcont.
ChiefEditor
VecihiBatuman,MD,FACP,FASNHuberwaldProfessorofMedicine,SectionofNephrologyHypertension,
TulaneUniversitySchoolofMedicineChief,RenalSection,SoutheastLouisianaVeteransHealthCareSystem
VecihiBatuman,MD,FACP,FASNisamemberofthefollowingmedicalsocieties:AmericanCollegeof
Physicians,AmericanSocietyofHypertension,AmericanSocietyofNephrology,InternationalSocietyof
Nephrology
Disclosure:Nothingtodisclose.
AdditionalContributors
LauraLyngbyMulloy,DO,FACPProfessorofMedicine,Chief,SectionofNephrology,Hypertension,and
TransplantationMedicine,Glover/MealingEminentScholarChairinImmunology,MedicalCollegeofGeorgia,
GeorgiaRegentsUniversity
Disclosure:Nothingtodisclose.

References
1. NeelakantappaK,GalloGR,BaldwinDS.ProteinuriainIgAnephropathy.KidneyInt.1988Mar.33(3):716
21.[Medline].
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