USMLE题库Endocrinology11-20.pptx
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Endocrinology题库Q11A16-year-oldCaucasiangirlwhoaspirestobeafashionmodelpresentsforaroutineexamination.Shewishestomodifyherdietandexerciseplanssothatshecanloseanadditional20lbs(9kg)becauseshebelievessheiscurrentlytoofat”toparticipateinabeautypageantlaterthisyear.Sheisfrustratedthatshecantseemtodroptheseextrapounds11inspiteofstrictdietarylimitationsandvigorousexercisefortwohourseachday.Hermenstrualhistoryissignificantforamenorrheaforthepastfourmonths.Herheightis(165cm)andherweightis100lbs(45.3kg),resultinginaBMIof16.6Kg/m2.Onphysicalexamination,sheispaleandemaciated.Finehairisnotedonhertrunk.Whichofthefollowingisthemostlikelymechanismofheramenorrhea?
A.PrimaryovarianfailureB.PrimarypituitarydysfunctionC.HypothyroidismD.HyperprolactinemiaE.HypothalamicdysfunctionA11Correctanswer:
EAnorexianervosaisaneatingdisordercharacterizedbyanintensefearofeating,significantlossofbodyweight(tolessthan85%ofexpectedweightorBMIlessthan17.5kg/m2),inaccurateperceptionofonesownbodyshapeandsize,andamenorrhea.TheamenorrhearesultsfromlossofpulsatileGnRHreleasefromthehypothalamus,whichoccurswhenthelevelofbodyfatfallsbelowacertaincriticallevel.LossofthiscyclicgonadotropinreleaseleadstoadecreaseinLHandFSHsecretionfromthepituitary,whichinturncausescirculatingestrogenlevelstobelow.AdministrationofpulsatileGnRHinindividualswithanorexianervosaallowsforthenormalizationofestrogenlevelsandsubsequentovulation.Amenorrheaalsooftenoccursinseriousathleteswithverylowbodyfat(eg.longdistancerunners,balletdancers)aswellasinindividualswithprolongedcachecticillnesses,viathesamemechanism.Ifleftuntreated,thelowestrogenlevelsintheseamenorrheicindividualscouldleadtoosteoporosis.(ChoicesAandB)TheamenorrheainpatientswithanorexianervosastemsfromadeficiencyinhypothalamicGnRHrelease,ratherthanaproblemwiththepituitaryorovaries.(ChoiceC)Whilethedecreasedcaloricintakeassociatedwithanorexianervosacancausethyroiddysfunction,thesethyroidabnormalitiesplaynoroleinthedevelopmentofamenorrhea.Patientswithanorexianervosacandeveloptheabnormalityofthyroidfunctioningtermed,fsickeuthyroidsyndrome/orlowT3syndrome.Earlyinthecourseofanorexia,T3levelsarelow,andT4andTSHlevelsarenormal.However,inprolongedseveredisease,bothT3andT4levelsarelow.(ChoiceD)Whilehyperprolactinemiaisaknowncauseofamenorrhea(andgalactorrhea,aswell),patientswithanorexianervosadonottypicallyhavehyperprolactinemia.Q12A43-year-oldnursecomestothephysiciancomplainingofepisodicconfusion,blurredvision,tremors,andsweating.Theseepisodeshaveincreasedinintensityoverthepastyear.Herothermedicalproblemsincludemajordepression.Serumlaboratoryresultsobtainedduringanepisodeareasfollows:
Glucose35mg/dLInsulin18pU/L(N:
9pU/L)Proinsulin10pU/L(N:
20%oftotalinsulin)C-peptide4.01ng/mL(N:
0.8-1.9ng/mL)Whichofthefollowingisthemostlikelycauseofherhypoglycemia?
A.Alpha-celltumorB.InsulinadministrationC.SomatostatinomaD.SulfonylureauseE.VIP-secretingtumoA12Correctanswer:
DThispatientishypoglycemicduetoexcessiveendogenousinsulinsecretion.Theproductionofinsulinbeginswiththesynthesisofproinsulinintheendoplasmicreticulum,whereitisfoldedanditsdisulfidebondsareoxidized.ItissubsequentlytransportedtotheGolgiapparatus,whereitispackagedintosecretoryvesiclesandfurtherprocessedbyaseriesofproteasestoformmatureinsulinandc-peptide.Insulinsecretagoguessuchassulfonylureasandmeglitinidescauseincreasedinsulinproductionandsecretion.ThesemedicationsbindtoATP-dependentK+channelsonthecellmembraneofpancreaticbetacells,inhibitingthehyperpolarizingpotassiumcurrentandcausingdepolarization.Thisopensvoltage-gatedCa2+channels,resultinginincreasedexocytosisofsecretorygranulesfilledwithmatureinsulinandc-peptide(aswellasasmallamountofproinsulinthatescapescleavage).Consequently,thereisariseinseruminsulin,c-peptide,andproinsulinlevels.Insulinsecretagoguesandinsulinomacancausesimilarchangesininsulin,c-peptide,andproinsulinlevels.Theonlywaytodistinguishbetweeninsulinomaandsulfonylureaormeglitinideabuseisbytestingtheurineorbloodforhypoglycemicagents.Factitioushypoglycemiaoccursmostcommonlyindiabeticpatientsandnon-diabeticswithhealth-relatedoccupations.Itisassociatedwithdepressionandpersonalitydisorders.(ChoiceA)Glucagonomaisararetumorofpancreaticalphacellsthatsecretesexcessiveamountsofglucagon.Thisresultsinhyperglycemia,anemia,andnecrolyticmigratoryerythema(araisederythematousrashtypicallyaffectingtheface,groin,andextremities).(ChoiceB)Exogenousins