Hypercortisolism皮质醇增多症

Hypercortisolism

皮质醇增多症Effectsofglucocorticoid

糖皮质激素作用Effectsonmetabolism对代谢旳影响Effectsonimmunologicfunctionandinflammatory免疫与炎症Effectsonmusculoskeletalandconnectivetissues肌肉骨骼与结缔组织Effectsonfluidandelectrolytehomeostasis水电解质平衡Neuropsychiatricandbehavioraleffects神经心理与行为效应Gastrointestinaleffects胃肠道效应Developmentaleffects对发育旳影响Thehypothalamic-pituitary-adrenalaxis下丘脑-垂体-肾上腺轴NyctohemeralrhythmNeuralstimuliCRHACTHCortisolCortisol(CBGbound)(free)PhysiologicalandMetaboliceffectsNegativefeedbackPlasmaHypothalamusAnteriorpituitaryAdrenalcortexInactivationbyreductionandconjugationLiverUnchangedcortisol(measuredasUFC)TetrahydrocortisolTetrahydrocortisoneCortols,Cortolones(measurdasurinary17-OHCS)Urine1．ACTH依赖性①Cushingdisease库欣病

垂体多有微腺瘤少数为大腺瘤②Ectopiccorticotropinsyndrome异位ACTH综合征2．非ACTH依赖性①Adrenalneoplasia

肾上腺皮质腺瘤②肾上腺皮质癌；③不依赖ACTH旳双侧性肾上腺小结节性增生；④不依赖ACTH性双侧肾上腺大结节性增生。

3.假性库欣综合征：医源性类库欣综合征酒精性类库欣综合征Pathogenesis病因Clinicalfeatures

临床特征Obesity(Theincreasedfatdistributionisnotgeneralized)向心性肥胖Moon-shapedfaceandplethoric满月脸Purplestriae紫纹Hypertension高血压IGT/Diabetes糖耐量异常/糖尿病Osteoporosis骨质疏松Hypokalemicalkalosis低钾性碱中毒ObesityMoon-shapedface7个月，8450g，114/52mmHgPurplestriaePurplestriae痤疮满月脸面部/身体体毛增多体重增长紫纹伤口愈合慢头发细多血质貌水牛背锁骨上脂肪垫肌肉萎缩皮肤菲薄DiagnosisandDifferentialDiagnosis

诊疗与鉴别SuspectedCushing’ssynd.PlasmacortisolUrinaryfreecortisolUrinary17-OHCSLowdosedexamethasonesuppressiontest1.0mg/day2days>50%reducefrombasal<50%reducefrombasalNORMALCushing’sSynd.Highdosedexamethasonesuppressiontest8.0mg/day2days<50%reducefrombasal>50%reducefrombasalProbableCushing’sDiseaseAdrenalDiseaseEctopicACTHSynd.SuspectedCushing’ssynd.PlasmaACTHAdrenalDiseaseEctopicACTHSyndromePlasmaACTHIncreasedLowProbableEctopicACTHSynd.ProbableAdrenalDiseaseTumorSearchCT/MRIScanofAdrenalspositivenegativeECTOPICACTHSYNDROMEnormaladrenalmassConsiderAdrenonodularHyperplasia,OtherDisordersurinary17-KSPlasmaDHEA17-KSDHEAADRENALCARCINOMADHEA17-KS

ADRENALADENOMA诊断流程TREATMENT

治疗CShypothalamuspituitaryadrenalDrugs:CyproheptadineMetergolineBromocryptineAdrenalectomySurgery:TranssphenoidalmicrosurgeryRadiation:

60Co

linearacceleratorDrug:o,p’DDD(mitotane)MetyraponeAninoglutethmideketokonazol