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1、库欣综合征库欣综合征肾上腺皮质肾上腺皮质球状带球状带-盐皮质激素（醛固酮）盐皮质激素（醛固酮）束状带束状带-糖皮质激素糖皮质激素网状带网状带-性激素性激素肾上腺髓质肾上腺髓质儿茶酚胺儿茶酚胺髓质素髓质素肾上腺分泌的激素肾上腺分泌的激素肾上腺疾病肾上腺疾病o皮质皮质功能亢进功能亢进功能减退功能减退先天性肾上腺皮质增生先天性肾上腺皮质增生肾上腺意外瘤肾上腺意外瘤o髓质髓质嗜铬细胞瘤嗜铬细胞瘤肾上腺髓质增生肾上腺髓质增生神经母细胞瘤及神经节细胞瘤神经母细胞瘤及神经节细胞瘤糖代谢糖代谢脂肪代谢脂肪代谢蛋白质代谢蛋白质代谢水和电解质平衡水和电解质平衡心血管系统心血管系统胃肠道系统胃肠道系统中枢神经系统中
2、枢神经系统钙磷和骨代谢钙磷和骨代谢免疫和炎症反应免疫和炎症反应应激反应应激反应内分泌系统内分泌系统血液系统血液系统糖皮质激素的生理作用糖皮质激素的生理作用库欣综合征库欣综合征Cushingssyndrome皮质醇增多症皮质醇增多症19321932年年Harvey CushingHarvey Cushing首先报道首先报道分分类类ACTHdependent84%1.Pituitary(Cushingsdisease)79%2.Ectopic14%3.ACTHuncertainsource6%4.Adrenalnodularhyperplasia1%(partiallyACTHdependent)
3、5.EctopicCRHorrelatedpeptidesrareNon-ACTHdependent16%1.Adrenaladenoma58%2.Adrenalcarcinoma42%3.non-ACTHdependentmicronodularhyperplasiaordysplasiaIatrogenic1.Glucocorticoidtherapy2.ACTHtherapyPseudo-Cushings1.Alcohol2.DepressionSite of secretionFemaleMaleBronchial carcinoid tumor112Small cell lung c
4、arcinoma15Medullary thyroid carcinoma3Pancreatic carcinoid tumor12Thymic carcinoid tumor1Disseminated carcinoid tumor1Mesothelioma1Pancreatic carcinoma1Colonic carcinoma1Phaechromocytoma1Gall bladder carcinoma1Total1616Etiology of the ectopic ACTH syndrome in patients seen at St.Bartholomews Hospita
5、l 1969-2001一、皮质醇过多引起的表现一、皮质醇过多引起的表现1、脂肪代谢紊乱和脂肪重新分布、脂肪代谢紊乱和脂肪重新分布2、蛋白分解加速，、蛋白分解加速，合成减少合成减少3、葡萄糖利用减少，糖原异生作用增加、葡萄糖利用减少，糖原异生作用增加4、电解质紊乱、电解质紊乱5、GH分泌减少，作用受抑制分泌减少，作用受抑制6、性腺功能受抑制、性腺功能受抑制7、精神异常、精神异常8、抵抗力下降、抵抗力下降二、二、其他激素分泌过多的临床表现其他激素分泌过多的临床表现1、雄激素过多、雄激素过多2、盐皮质激素过多、盐皮质激素过多3、ACTH过多过多三、三、肿瘤本身引起的临床表现肿瘤本身引起的临床表现1、
6、腹块、腹块2、蝶鞍扩大、蝶鞍扩大3、异源性、异源性ACTH综合症引起的局部症状综合症引起的局部症状(肺癌、支气管类癌、胸腺癌等肺癌、支气管类癌、胸腺癌等)临床表现临床表现SymptomsSignsWeight gain Truncal obesityMenstrual irregularity PlethoraHirsuitism in women Moon faceHeadache HypertensionThirst BruisingBack pain StriaeMuscle weakness Buffalo humpAbdominal pain AcneLethargy/depress
7、ion Osteoporosis皮质醇增多的临床表现皮质醇增多的临床表现库欣综合症的主要临床表现库欣综合症的主要临床表现满月脸（满月脸（90%90%）向心性肥胖（向心性肥胖（85%85%）糖耐量异常（糖耐量异常（85%85%）高血压高血压(80%)(80%)性功能紊乱性功能紊乱(75%)(75%)骨质疏松骨质疏松(65%)(65%)紫纹紫纹(60%)(60%)四肢无力四肢无力(65%)(65%)多毛多毛(70%)(70%)水肿水肿(55%)(55%)水牛背水牛背(55%)(55%)痤疮痤疮(55%)(55%)精神异常精神异常(45%)(45%)The features of the ectop
8、ic ACTH syndromeShorthistoryWeightlossSeveremyopathyPigmentationHypokalemicalkalosisVeryhighplasmaACTHOvertneoplasm,usuallysmallcelllungcarcinoma辅助检查辅助检查一、激素测定一、激素测定皮质醇及其周期皮质醇及其周期ACTH及其周期及其周期尿尿/唾液游离皮质醇测定唾液游离皮质醇测定二、动态试验二、动态试验1、抑制试验、抑制试验a.午夜地塞米松抑制试验午夜地塞米松抑制试验b.小剂量地塞米松抑制试验小剂量地塞米松抑制试验c.大剂量地塞米松抑制试验大剂量地塞米
9、松抑制试验d.metyraponetest查查17-OH和和plasmadeoxycortisol2、兴奋试验、兴奋试验a.CRHtestb.ACTHtestc.hypoglycemiatest三、影像学检查三、影像学检查1、pituitaryimaging2、meta-iodobenylquanidinescanning3、simultaneousbilateralinferiorpetrosalsinussampling诊断步骤诊断步骤明确高皮质醇血症明确高皮质醇血症血皮质醇；血皮质醇；24h尿游离皮质醇；尿游离皮质醇；小剂量地塞米松抑制试验；小剂量地塞米松抑制试验；胰岛素低血糖试验；胰岛
10、素低血糖试验；明确病因分类明确病因分类大剂量地塞米松抑制试验；大剂量地塞米松抑制试验；血血ACTH；甲吡酮试验；甲吡酮试验；CRH兴奋试验；兴奋试验；静脉导管分段取血测静脉导管分段取血测ACTH；影像学检查；影像学检查；高皮质醇血症的诊断高皮质醇血症的诊断o疑有疑有CushingsCushingsa.典型临床表现典型临床表现b.24小时小时UFC或或17-OH升高升高c.血浆皮质醇分泌失去正常节律血浆皮质醇分泌失去正常节律d.午夜地塞米松抑制试验阳性午夜地塞米松抑制试验阳性o确诊确诊CushingsCushingsa.小剂量地塞米松抑制试验阳性小剂量地塞米松抑制试验阳性b.血浆皮质醇对胰岛素诱
11、发的低血糖无反应血浆皮质醇对胰岛素诱发的低血糖无反应Identification of anatomical site of lesionSerum ACTH Low in adrenal disease,high in pituitary and ectopic production CRH test Increased ACTH following CRH in pituitary disease No increase in ACTH following CRH in ectopic production High-dose dexamethasone suppression test(
12、2 mg qds for 2 days)Serum cortisol reduced by high-dose in pituitary disease Suppression of urinary free cortisol to less than 10%of baseline Identifying the pathological lesionPituitary CT has a sensitivity of about 50%for identifying microadenomas MRI has increased sensitivity but is not 100%predi
13、ctive If diagnostic doubt need bilateral inferior petrosal sinus sampling for ACTH Abdominal CT will allow identification of adrenal pathology Somatostatin scintigraphy to identify sites of ectopic hormone production Causes of hypercortisolism Physiological statesPregnancy Stress Chronic excessive e
14、xercise Malnutrition Pathological states Cushings syndrome Diabetes mellitus Hyperthyroidism Severe chronic disease Glucocorticoid resistance Psychological states Anorexia nervosa Panic disorder Melancholic depression Obsessive-compulsive disorder 不同病因库欣综合征的鉴别Transphenoidalsurgery:success rate：appro
15、ximately 90%Large tumours occasional require open surgery via the anterior fossa Post-operative radiotherapy occasionally required If pituitary surgery fails need to consider bilateral adrenalectomy 25%patients develop Nelsons syndrome after bilateral adrenalectomy Adrenal adenomas require adrenalec
16、tomy Performed either laparoscopically or via open surgery Open surgery can be performed via a transabdominal or retroperitoneal approachGeneralconsiderationsMedical ManagementlAdrenolyticTherapyMetyrapone、Ketoconazole、Aminoglutethimide、Mitotane(o.pDDD)、Etomidate.lNeuromodulatoryagents5-HTAntagonist
17、s：Cyproheptadine、Ritanserin；DopamineAgonists：Bromocriptine；SomatostatinAnalogues：Octreotide；GABAAgonists：SodiumValproate.lReceptorblockadeGlucocorticoidAntagonists：Mifepristone(RU486).Future Strategies for medical agentso Specific receptor antagonistso Cholinergic pathwayo Anti-muscarinic agents o R
18、etinoic acidSurgical ManagementTransphenoidalAdrenalectomySurgeryforectopicACTHsyndrome术前12小时及2小时各肌注醋酸可的松50mg,术后肌注50mg q6h,术后第三日起减量至q8h，第五日减量为q12h术中静滴氢可200mg,术后再滴注100mg围手术期的特殊处理围手术期的特殊处理术后替代治疗术后替代治疗o氢化可的松氢化可的松2030mg或可的松或可的松2537.5mgo根据肾上腺皮质功能调整替代治疗剂量根据肾上腺皮质功能调整替代治疗剂量o一般一般612个月内停止替代治疗个月内停止替代治疗 自愈率低 未经治疗死亡率为50%伴动脉硬化或骨质疏松者致残率高预预后后