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1、库欣综合征曾正陪北京协和医院内分泌科In1912,aphysicianbythenameofHarveyCushingdescribedeightpatientswithcentralbodyobesity,glucoseintolerance,hypertension,excesshairgrowth,osteoporosis,kidneystones,menstrualirregularity,andemotionalliability.Itisnowknownthatthesesymptomsaretheresultofexcessproductionofcortisol
2、bytheadrenalglands.库欣综合征（Cushing’ssyndrome）又称皮质醇增多症，或柯兴综合征，是由于肾上腺皮质长期分泌过量皮质醇引起的症候群，这称为自发性库欣综合症。长期应用外源性糖皮质激素或饮用酒精饮料也可引起类似库欣综合征临床表现，称为类库欣综合征。库欣综合征的发现肾上腺的结构球状带束状带网状带髓质ACTH依赖性ACTHDependent(80%)垂体或垂体以外的某些肿瘤组织分泌过量ACTH，使双侧肾上腺皮质增生并分泌过量皮质醇，皮质醇的分泌过多是继发的。ACTH非依赖性ACTHIndependent(20%)肾上腺皮质自主地分泌过量皮质醇，其原因可
3、以是肾上腺皮质肿瘤，也可以是大结节增生。库欣综合征的病因从激素分泌分类库欣综合征的病因从激素分泌分类库欣综合征(Cushingsyndrome,皮质醇增多症)Sincecortisolproductionbytheadrenalglandsisnormallyunderthecontrolofthepituitary(likethethyroidgland),overproductioncanbecausedbyatumorinthepituitaryorwithintheadrenalglandsthemselves.因垂体或肾上腺肿瘤分泌过多皮质醇所致的一组症候群库欣病(C
4、ushingdisease)WhenapituitarytumorsecretestoomuchACTH(AdrenalCorticalTropicHormone),itsimplycausestheotherwisenormaladrenalglandstoproducetoomuchcortisol.ThistypeofCushingssyndromeistermed"CushingsDisease“.Inthiscase,serumcortisolwillbeelevated,and,serumACTHwillbeelevatedatthesametime.因垂体瘤分泌过
5、多促肾上腺皮质激素(ACTH),刺激肾上腺分泌过多皮质醇所致的库欣综合征库欣综合征的病因从病变部位分类垂体瘤（PituitaryAdenomas）PituitaryadenomascausemostcasesofCushing'ssyndrome.Theyarebenign,ornon-cancerous,tumorsofthepituitaryglandwhichsecreteincreasedamountsofACTH.Mostpatientshaveasingleadenoma.Thisformofthesyndrome,knownas"Cushing'sdisease,
6、"affectswomenfivetimesmorefrequentlythanmen.异位ACTH综合征(EctopicACTHSyndrome)Somebenignormalignant(cancerous)tumorsthatariseoutsidethepituitarycanproduceACTH.ThisconditionisknownasectopicACTHsyndrome.Lungtumorscauseover50percentofthesecases.Menareaffected3timesmorefrequentlythanwomen.Themostcom
7、monformsofACTH-producingtumorsareoatcell,orsmallcelllungcancer,whichaccountsforabout25percentofalllungcancercases,andcarcinoidtumors.OtherlesscommontypesoftumorsthatcanproduceACTHarethymomas,pancreaticisletcelltumors,andmedullarycarcinomasofthethyr
