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1、,库欣综合征,肾上腺皮质 球状带-盐皮质激素（醛固酮）束状带-糖皮质激素 网状带-性激素肾上腺髓质 儿茶酚胺 髓质素,肾上腺分泌的激素,肾上腺疾病,皮质 功能亢进 功能减退 先天性肾上腺皮质增生 肾上腺意外瘤髓质 嗜铬细胞瘤 肾上腺髓质增生 神经母细胞瘤及神经节细胞瘤,糖代谢脂肪代谢蛋白质代谢水和电解质平衡心血管系统胃肠道系统,中枢神经系统钙磷和骨代谢免疫和炎症反应应激反应内分泌系统血液系统,糖皮质激素的生理作用,库欣综合征Cushings syndrome,皮质醇增多症1932年Harvey Cushing首先报道,分 类,ACTH dependent 84%1.Pituitary(Cush
2、ings disease)79%2.Ectopic 14%3.ACTH uncertain source 6%4.Adrenal nodular hyperplasia 1%(partially ACTH dependent)5.Ectopic CRH or related peptides rareNon-ACTH dependent 16%1.Adrenal adenoma 58%2.Adrenal carcinoma 42%3.non-ACTH dependent micronodular hyperplasia or dysplasiaIatrogenic 1.Glucocortico
3、id therapy 2.ACTH therapyPseudo-Cushings 1.Alcohol 2.Depression,Etiology of the ectopic ACTH syndrome in patients seen at St.Bartholomews Hospital 1969-2001,一、皮质醇过多引起的表现 1、脂肪代谢紊乱和脂肪重新分布 2、蛋白分解加速，合成减少 3、葡萄糖利用减少，糖原异生作用增加 4、电解质紊乱 5、GH分泌减少，作用受抑制 6、性腺功能受抑制 7、精神异常 8、抵抗力下降 二、其他激素分泌过多的临床表现 1、雄激素过多 2、盐皮质激素过多
4、 3、ACTH过多 三、肿瘤本身引起的临床表现 1、腹块 2、蝶鞍扩大 3、异源性ACTH综合症引起的局部症状(肺癌、支气管类癌、胸腺癌等),临床表现,Symptoms Signs Weight gain Truncal obesityMenstrual irregularity PlethoraHirsuitism in women Moon faceHeadache HypertensionThirst BruisingBack pain StriaeMuscle weakness Buffalo humpAbdominal pain AcneLethargy/depression Ost
5、eoporosis,皮质醇增多的临床表现,库欣综合症的主要临床表现,满月脸（90%）向心性肥胖（85%）糖耐量异常（85%）高血压(80%)性功能紊乱(75%)骨质疏松(65%)紫纹(60%)四肢无力(65%)多毛(70%)水肿(55%)水牛背(55%)痤疮(55%)精神异常(45%),The features of the ectopic ACTH syndrome,Short historyWeight lossSevere myopathyPigmentationHypokalemic alkalosisVery high plasma ACTHOvert neoplasm,usuall
6、y small cell lung carcinoma,辅助检查,一、激素测定 皮质醇及其周期 ACTH及其周期 尿/唾液游离皮质醇测定二、动态试验 1、抑制试验 a.午夜地塞米松抑制试验 b.小剂量地塞米松抑制试验 c.大剂量地塞米松抑制试验 d.metyrapone test 查17-OH 和plasma deoxycortisol 2、兴奋试验 a.CRH test b.ACTH test c.hypoglycemia test三、影像学检查 1、pituitary imaging 2、meta-iodobenyl quanidine scanning 3、simultaneous bi
7、lateral inferior petrosal sinus sampling,诊断步骤,明确高皮质醇血症 血皮质醇；24h尿游离皮质醇；小剂量地塞米松抑制试验；胰岛素低血糖试验；明确病因分类 大剂量地塞米松抑制试验；血ACTH；甲吡酮试验；CRH兴奋试验；静脉导管分段取血测ACTH；影像学检查；,高皮质醇血症的诊断,疑有Cushings a.典型临床表现 b.24小时UFC或17-OH升高 c.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确诊Cushings a.小剂量地塞米松抑制试验阳性 b.血浆皮质醇对胰岛素诱发的低血糖无反应,Identification of anato
8、mical site of lesion,Serum 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(2 mg qds for 2 days)Serum cortisol reduced by hi
9、gh-dose in pituitary disease Suppression of urinary free cortisol to less than 10%of baseline,Identifying the pathological lesion,Pituitary CT has a sensitivity of about 50%for identifying microadenomas MRI has increased sensitivity but is not 100%predictive If diagnostic doubt need bilateral inferi
10、or 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 exercise Malnutrition Pathological states Cushin
11、gs 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,Transphenoidal surgery:success rate：approximately 90%Large tumours occasional require
12、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 adrenalectomy Performed either laparoscopically or via
13、 open surgery Open surgery can be performed via a transabdominal or retroperitoneal approach,General considerations,Medical Management,Adrenolytic Therapy Metyrapone、Ketoconazole、Aminoglutethimide、Mitotane(o.pDDD)、Etomidate.Neuromodulatory agents 5-HT Antagonists：Cyproheptadine、Ritanserin；Dopamine A
14、gonists：Bromocriptine；Somatostatin Analogues：Octreotide；GABA Agonists：Sodium Valproate.Receptor blockade Glucocorticoid Antagonists：Mifepristone(RU 486).,Future Strategies for medical agents,Specific receptor antagonists Cholinergic pathway Anti-muscarinic agents Retinoic acid,Surgical Management,Transphenoidal Adrenalectomy Surgery for ectopic ACTH syndrome,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,术后替代治疗,氢化可的松2030mg或可的松2537.5mg 根据肾上腺皮质功能调整替代治疗剂量 一般612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 后,
