Cushing综合症VIP免费

CushingsyndromeCushing综合症DefinitionCushingsyndrome,theconstellationofclinicalsignsandsymptomsresultingformchronicglucocorticoidexcess,wasprobablyfirstdescribedin1899定义为各种病因造成肾上腺分泌过多的糖皮质激素（主要是皮质醇）所致病症的总称主要临床表现为满月脸，多血质外貌，向心性肥胖、痤疮、皮肤紫纹高血压和骨质疏松等AetiologyClinicalpresentationlaboratoryexaminationDiagnosisanddifferentialdiagnosisTreatment(principle)Prognosis病因分类临床表现实验室检查诊断及鉴别诊断治疗(原则)预后Aetiology病因分类Hypothalamus下丘脑（CRH）pituitarygland垂体（ACTH）adrenalcortex肾上腺皮质zonaglomerulosazonafasciculatazonareticularis（Aldosterone）（cortisol）（gonadalhormone）++--AetiologyACTH-dependentCushingsyndromeCushingdiseaseEctopicACTHsyndromeACTH-independentCushingsyndromeAdrenocorticaladenomaAdrenocorticaladenocarcinomaMicronodularadrenaldiseaseMassivemacronodularadrenaldisease病因分类依赖ACTH的cushing综合症cushing病异位ACTH综合症非依赖ACTH的cushing综合症肾上腺皮质腺瘤肾上腺皮质癌原发性色素结节性肾上腺病大结节性巨大肾上腺病Clinicalfeature各型临床特征CushingDiseaseItisthemostcommontypeofCushingsyndrome,andaccountsforabout70%ofthesyndromePathogenesisMostpatientshaveACTH-secretinganteriorpituitarycorticortropemicroadenomas(＜10mmindiameter),butasmallnumberhavediffusecorticotropehyperplasiaandpituitarymacroadenomaCushing病最常见，约占cushing综合症的70%,多见于成人，女性多于男性，是由于垂体分泌过多的ACTH所致病因垂体微腺瘤－80%垂体大腺瘤－10％：压迫、侵袭等症状下丘脑功能紊乱EctopicACTHsyndromeThemajorityofpatientswithectopicACTHsyndromehaveoutsidepituitarytumorOthersitesarepulmonarycarcinosis,carcinoidofbronchus,thymiccarcinomapancreaticcancer,chromaffintumor,medullarycarcinomaofthyroidandsoon异位ACTH综合症继发于垂体以外的恶性肿瘤小细胞肺癌、支气管类癌、胸腺癌、胰腺癌、嗜铬细胞瘤、神经母细胞瘤等等临床上分两型缓慢进展型迅速进展型AdrenocorticaladenomaPatientswithadrenaladenomausuallyhavegradualonsetofsignofhypercortisolism,accountsforabout15-20%ofthesyndromeAdenomaisroundorellipse,diameteris3-4cm,weightisabout40g肾上腺皮质腺瘤多见于成人，男性多于女性－15%-20%腺瘤呈圆形或椭圆形，直径约3-4cm，重40g左右，包膜完整其病缓慢，病情中等，多毛及雄激素增多少见AdrenocorticaladenocarcinomaItoccursinlowerthan5%patientsofcushingsyndromePatientswithadenocarcinomatendtohaveamoreacuteandprogressivecourseandvirilizingeffectsmaypredominateThebulkoftumorislarger肾上腺皮质腺癌占cushing综合症的5％以下瘤体积大（直径5-6cm)，肿瘤可浸润包膜病情重，进展快，重度cushing综合症的表现(低钾性碱中毒、女性男性化）MicronodularadrenaldiseaseThediseaseisalsoknownasMeador’ssyndromeAbouthalfofpatientswiththisdisorderhavenodistinctiveclinicalpresentationotherthanbeingyoung-alwaysyoungerthan30yearsofage,halfyoungerthan15yearsandsomeinfants原发性色素结节性肾上腺病此病又称这为Meador综合症或原发性色素性结节性肾上腺疾病患者多为儿童和青少年临床表现：一部分同一般的临床表现一部分为家族性，呈显性遗传(Carney综合症）Massivemacronodularadrenaldisease大结节性巨大肾上腺病Clinicalpresentation临床表现典型病例重型cushing综合症早期病例以并发症为主者周期性或间歇性Typicalcases典型病例Clinicalpresentation1Progressiveobesityisthemostcommonsign.Itisusuallycentral,involvingtheface,necktrunkandabdomenAlloverthebodyandnervoussystem:weakness,changeofemotionandsoonTheskinisatrophic,thestratumcorneumisthinned,andthereislossofsubcutaneousfatallowingsubcutan...