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Cushing's syndrome
Cushingssyndromeisacollectionofhormonaldisorderscharacterisedbyhighlevelsofthe hormonecortisol.AnothernameforCushingssyndromeishypercortisolism.Somepeoplehave Cushingssyndromesymptomswhentheytakeglucocorticoidhormonestotreatinflammatory conditionssuchasasthma,lupusorrheumatoidarthritis. Othercausesincludetumoursofthepituitaryandadrenalglands,andtumoursinotherareasof thebody.Aroundonein50,000peopleareaffectedbyCushingssyndrome,withfemalesmore susceptibletosomeformsthanmales.Withouttreatment,thedisordercanbefatal. ManyoverweightpeoplewithhypertensionordiabeteshavesomefeaturesofCushingssyndrome. Testscanbedonetoruleoutthispossibility.

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Symptoms of Cushings syndrome

Someofthesymptomsinclude: Weightgainaroundtheabdomen,andobesity Wastingofthelimbs Abuffalohumpoffathighontheback Round,redandpuffy-lookingface(moonface) Thinskin,easilybruised,slowhealingandulcers Muscularweakness Thirst Frequenturination Headaches Highbloodpressure Highwhitebloodcellcount,lowserumpotassium Highbloodsugar(in80percentofpatients) Moodswings,irritability,anxiety,depression Impotence Irregularmenstrualperiodsornomenstrualperiods Increasedfacialhairinwomen Weakenedbones,susceptibilitytobonefractures(especiallyinribsandspine), osteoporosisandbackache SusceptibilitytopneumoniaandTB.

The hormone cortisol

Cortisolismadebytheadrenalglands.Whencortisollevelsaretoolow,thepituitarysecretesthe stimulatinghormoneadrenocorticotropin(ACTH).Highlevelsofcortisolpromptthepituitaryto decreaseACTH,whichslowscortisolproduction. Cortisolisessentialtolife.Itsfunctionsinclude: Helpingthebodymanagestress Helpingtomaintainconstantbloodsugarlevels Inhibitinginflammation Contributingtothemaintenanceofconstantbloodpressure Contributingtotheworkingsoftheimmunesystem.

Causes of Cushings syndrome

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ThesymptomsofCushingssyndromearecausedbydamagetobodytissuesduetohighlevelsof thehormonecortisolinthebloodoveralongtime.Thedisordersthattriggerhighcortisollevels include: Tumourofthepituitarygland Tumouroftheadrenalgland ACTH-producingtumours(ectopic)elsewhereinthebody Multipleendocrineneoplasia1(MEN1) Glucocorticoidhormonetherapy.

Tumour of the pituitary gland

ThisformisknownasCushingsdiseaseandaccountsforaboutsevenin10casesofCushings syndrome.OnetypeofpituitaryglandtumoursecretesadditionalACTH,whichforcestheadrenal glandstomaketoomuchcortisol. Thesetumoursareusuallybenignnon-cancerousadenomasandtheyoccurmoreofteninwomen thanmen(3to1).Complicationsincludediabetes,kidneystonesandmentaldisturbances,suchas psychosis.

Tumour of the adrenal gland

Atumourononeoftheadrenalglandstriggerstheproductionofhighcortisollevels.Thetumours areusuallynon-cancerous.ThepituitaryrespondsbydroppingitslevelofACTHhormone,which causesthehealthyadrenalglandtoshrink.Onsetofsymptomscanbequiterapid.

ACTH-producing tumours
Inrarecases,othertumoursinthebodycanproduceACTH.Approximately17percentof Cushingssyndromedevelopsthisway.Fiftypercentofcasesresultfromlungtumours.Other causescomefromtumoursinthethymus,pancreasandthyroidgland.ThistypeofCushings syndromeissometimesreferredtoasectopic.

Multiple endocrine neoplasia 1 (MEN1)

Thepituitary,pancreas,adrenals,thyroidandparathyroidglandsbelongtotheendocrinesystem. MEN1isaninheritedconditioncharacterisedbytumoursonatleasttwooftheseglands.Ifthe pituitaryoradrenalsareaffected,Cushingssyndromecanresult. Primarypigmentedmicronodularadrenaldiseaseoccursinchildrenandyoungadults,wherethere isanincreaseinthenumberofcortisol-producingadrenaltumours.

Glucocorticoid hormone therapy

Glucocorticoiddrugsarecommonlyusedtoeasethesymptomsofinflammatoryconditionssuchas asthma,rheumatoidarthritisandlupuserythematosus.Thesedrugscantriggersymptomsof Cushingssyndrome,althoughtheeffectsshouldreverseoncethedrugtherapyisstopped. However,suppressionofapersonsownadrenalfunctionmaypersist,creatingadrenal insufficiency,whichmayrequirereplacementtherapyduringgradualwithdrawalofglucocorticoid drugs.

Diagnosis of Cushings syndrome

DiagnosisofCushingssyndromeinvolvesdiscoveringhighcortisollevelsanduncoveringthe cause.Diagnosismayinvolve: Physicalandvisualexamination,withfullfamilymedicalhistory BloodteststocheckifCushingsisofpituitaryorectopicorigin,andtocompareACTH levelselsewhereinthebody.Acombinationofthesetwotestsgivesgreataccuracyfor diagnosis Urinetests Dexamethasonesuppressiontestusingsteroidstocheckthebodysreactionto glucocorticoiddrugs

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Magneticresonanceimaging(MRI)scansandcomputedtomography(CT)scanstocheck forglandsizeandevidenceoftumours.

Treatment for Cushings syndrome

Treatmentdependsonthecauseandmayinclude: Tumour of the pituitary glandthetumourissurgicallyremoved.Otheroptionsinclude radiationtherapyanddrugtherapytoshrinkthetumourandstopitfromproducing hormones.Varioushormonereplacementsmayberequiredafterpituitarysurgery. Tumour of the adrenal glandthetumourissurgicallyremoved.Replacementhormone therapymaybenecessaryforashortwhile. ACTH-producing tumourstreatmentincludessurgerytoremovethetumour,followed possiblybychemotherapy,immunotherapyandradiationtherapy.Medicationcanreduce theabilityoftheadrenalglandstomakecortisol. MEN1radiationtherapyandsurgeryareusedtoremovethetumoursandassociated glands.Ongoinghormonereplacementtherapyisneededaftersurgery. Glucocorticoid hormone therapy induced Iatrogenic Cushings syndrome symptomswillgraduallyresolveiftreatmentcanbereducedorstopped,whichdependson theactivityofthedisorder.Treatmentshouldneverbestoppedsuddenlybecauseofthe possibilityofadrenalsuppression.

Where to get help

Yourdoctor Endocrinologist TheAustralianAddison'sDiseaseAssociationInc.Tel.(02)66524761;(02)66572571. AustralianPituitaryFoundationTel.1300331807 TheWesternAustralianCushing'sDiseaseAssociationTel.(08)94024394;(08)9390 2132.

Things to remember

Cushingssyndromeisacollectionofhormonaldisordersresultingfromhighlevelsofthe hormonecortisol. ManyoverweightpeoplewithhypertensionordiabeteshavesomefeaturesofCushings syndrome.Testscanbedonetoruleoutthispossibility. Causesincludecertaintumoursandglucocorticoiddrugtherapyforinflammatorydisorders. Withouttreatment,Cushingssyndromecanbefatal.

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AustralianAddisonsDiseaseAssociation
Contentonthiswebsiteisprovidedforeducationandinformationpurposesonly.Information aboutatherapy,service,productortreatmentdoesnotimplyendorsementandisnotintendedto replaceadvicefromyourdoctororotherregisteredhealthprofessional.Contenthasbeen preparedforVictorianresidentsandwiderAustralianaudiences,andwasaccurateatthetimeof publication.Readersshouldnotethat,overtime,currencyandcompletenessoftheinformation maychange.Allusersareurgedtoalwaysseekadvicefromaregisteredhealthcareprofessional fordiagnosisandanswerstotheirmedicalquestions. Forthelatestupdatesandmoreinformation,visitwww.betterhealth.vic.gov.au Copyight 1999/2012StateofVictoria.ReproducedfromtheBetterHealthChannel (www.betterhealth.vic.gov.au)atnocostwithpermissionoftheVictorianMinisterforHealth. Unauthorisedreproductionandotherusescomprisedinthecopyrightareprohibitedwithout permission.

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Cushing'ssyndrome

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