最新库欣综合征简化1课件

库欣综合征简化1库欣综合征简化1最新库欣综合征简化1课件2最新库欣综合征简化1课件3最新库欣综合征简化1课件4最新库欣综合征简化1课件5最新库欣综合征简化1课件6最新库欣综合征简化1课件7最新库欣综合征简化1课件8Siteofsecretion

Female

MaleBronchialcarcinoidtumor

11

2Smallcelllungcarcinoma

1

5Medullarythyroidcarcinoma

3Pancreaticcarcinoidtumor

1

2Thymiccarcinoidtumor

1Disseminatedcarcinoidtumor

1Mesothelioma

1Pancreaticcarcinoma

1Coloniccarcinoma

1Phaechromocytoma

1Gallbladdercarcinoma

1Total

16

16EtiologyoftheectopicACTHsyndromeinpatientsseenatSt.Bartholomew'sHospital1969-2001Siteofsecretion

Female

MaleB9

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

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

临床表现10SymptomsSigns

WeightgainTruncalobesityMenstrualirregularityPlethoraHirsuitisminwomen'Moon'faceHeadacheHypertensionThirstBruisingBackpainStriaeMuscleweaknessBuffalohumpAbdominalpainAcneLethargy/depressionOsteoporosis皮质醇增多的临床表现Symptoms11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%)库欣综合症的主要临床表现满月脸（90%）12ThefeaturesoftheectopicACTHsyndromeShorthistoryWeightlossSeveremyopathyPigmentationHypokalemicalkalosisVeryhighplasmaACTHOvertneoplasm,usuallysmallcelllungcarcinoma

ThefeaturesoftheectopicAC13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.metyraponetest查17-OH和plasmadeoxycortisol2、兴奋试验

a.CRHtestb.ACTHtestc.hypoglycemiatest三、影像学检查

1、pituitaryimaging2、meta-iodobenylquanidinescanning3、simultaneousbilateralinferiorpetrosalsinussampling辅助检查

一、激素测定14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；诊断步骤明确高皮质醇血症15高皮质醇血症的诊断疑有Cushing'sa.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Cushing'sa.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高皮质醇血症的诊断疑有Cushing's16IdentificationofanatomicalsiteoflesionSerumACTH

Lowinadrenaldisease,highinpituitaryandectopicproductionCRHtestIncreasedACTHfollowingCRHinpituitarydiseaseNoincreaseinACTHfollowingCRHinectopicproductionHigh-dosedexamethasonesuppressiontest(2mgqdsfor2days)Serumcortisolreducedbyhigh-dose

inpituitarydiseaseSuppressionofurinaryfreecortisoltolessthan10%ofbaselineIdentificationofanatomicals17IdentifyingthepathologicallesionPituitaryCThasasensitivityofabout50%foridentifyingmicroadenomasMRIhasincreasedsensitivitybutisnot100%predictiveIfdiagnosticdoubtneedbilateralinferiorpetrosalsinussamplingforACTHAbdominalCTwillallowidentificationofadrenalpathologySomatostatinscintigraphytoidentifysitesofectopichormoneproductionIdentifyingthepathologicall18Causesofhypercortisolism

Physiologicalstates

Pregnancy

Stress

Chronicexcessiveexercise

Malnutrition

Pathologicalstates

Cushing'ssyndrome

Diabetesmellitus

Hyperthyroidism

Severechronicdisease

Glucocorticoidresistance

Psychologicalstates

Anorexianervosa

Panicdisorder

Melancholicdepression

Obsessive-compulsivedisorderCausesofhypercortisolism19不同病因库欣综合征的鉴别不同病因库欣综合征的鉴别20Transphenoidalsurgery:successrate：approximately90%LargetumoursoccasionalrequireopensurgeryviatheanteriorfossaPost-operativeradiotherapyoccasionallyrequiredIfpituitarysurgeryfailsneedtoconsiderbilateraladrenalectomy25%patientsdevelopNelson'ssyndromeafterbilateraladrenalectomyAdrenaladenomasrequireadrenalectomyPerformedeitherlaparoscopicallyorviaopensurgeryOpensurgerycanbeperformedviaatransabdominalorretroperitonealapproachGeneralconsiderationsTransphenoidalsurgery:succes21MedicalManagementAdrenolyticTherapy

Metyrapone、Ketoconazole、Aminoglutethimide、Mitotane(o.p'DDD)、Etomidate.

Neuromodulatoryagents

5-HTAntagonists：Cyproheptadine、Ritanserin；

DopamineAgonists：Bromocriptine；

SomatostatinAnalogues：Octreotide；

GABAAgonists：SodiumValproate.ReceptorblockadeGlucocorticoidAntagonists：Mifepristone(RU486).

MedicalManagementAdrenolytic22

FutureStrategiesformedicalagentsSpecificreceptorantagonistsCholinergicpathwayAnti-muscarinicagentsRetinoicacid

FutureStrategiesformedica23SurgicalManagementTransphenoidalAdrenalectomySurgeryforectopicACTHsyndromeSurgicalManagement24术前12小时及2小时各肌注醋酸可的松50mg,术后肌注50mgq6h,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前12小时及2小时各肌注醋酸可的松50mg,术后肌注50m25术后替代治疗

氢化可的松20~30mg或可的松25~37.5mg

根据肾上腺皮质功能调整替代治疗剂量一般6~12个月内停止替代治疗术后替代治疗氢化可的松20~30mg或可的松25~37.526

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自愈率低预后27

结束语谢谢大家聆听！！！28

结束语谢谢大家聆听！！！28库欣综合征简化1库欣综合征简化1最新库欣综合征简化1课件30最新库欣综合征简化1课件31最新库欣综合征简化1课件32最新库欣综合征简化1课件33最新库欣综合征简化1课件34最新库欣综合征简化1课件35最新库欣综合征简化1课件36Siteofsecretion

Female

MaleBronchialcarcinoidtumor

11

2Smallcelllungcarcinoma

1

5Medullarythyroidcarcinoma

3Pancreaticcarcinoidtumor

1

2Thymiccarcinoidtumor

1Disseminatedcarcinoidtumor

1Mesothelioma

1Pancreaticcarcinoma

1Coloniccarcinoma

1Phaechromocytoma

1Gallbladdercarcinoma

1Total

16

16EtiologyoftheectopicACTHsyndromeinpatientsseenatSt.Bartholomew'sHospital1969-2001Siteofsecretion

Female

MaleB37

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

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

临床表现38SymptomsSigns

WeightgainTruncalobesityMenstrualirregularityPlethoraHirsuitisminwomen'Moon'faceHeadacheHypertensionThirstBruisingBackpainStriaeMuscleweaknessBuffalohumpAbdominalpainAcneLethargy/depressionOsteoporosis皮质醇增多的临床表现Symptoms39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%)库欣综合症的主要临床表现满月脸（90%）40ThefeaturesoftheectopicACTHsyndromeShorthistoryWeightlossSeveremyopathyPigmentationHypokalemicalkalosisVeryhighplasmaACTHOvertneoplasm,usuallysmallcelllungcarcinoma

ThefeaturesoftheectopicAC41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.metyraponetest查17-OH和plasmadeoxycortisol2、兴奋试验

a.CRHtestb.ACTHtestc.hypoglycemiatest三、影像学检查

1、pituitaryimaging2、meta-iodobenylquanidinescanning3、simultaneousbilateralinferiorpetrosalsinussampling辅助检查

一、激素测定42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；诊断步骤明确高皮质醇血症43高皮质醇血症的诊断疑有Cushing'sa.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Cushing'sa.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高皮质醇血症的诊断疑有Cushing's44IdentificationofanatomicalsiteoflesionSerumACTH

Lowinadrenaldisease,highinpituitaryandectopicproductionCRHtestIncreasedACTHfollowingCRHinpituitarydiseaseNoincreaseinACTHfollowingCRHinectopicproductionHigh-dosedexamethasonesuppressiontest(2mgqdsfor2days)Serumcortisolreducedbyhigh-dose

inpituitarydiseaseSuppressionofurinaryfreecortisoltolessthan10%ofbaselineIdentificationofanatomicals45IdentifyingthepathologicallesionPituitaryCThasasensitivityofabout50%foridentifyingmicroadenomasMRIhasincreasedsensitivitybutisnot100%predictiveIfdiagnosticdoubtneedbilateralinferiorpetrosalsinussamplingforACTHAbdominalCTwillallowidentificationofadrenalpathologySomatostatinscintigraphytoidentifysitesofectopichormoneproductionIdentifyingthepathologicall46Causesofhypercortisolism

Physiologicalstates

Pregnancy

Stress

Chronicexcessiveexercise

Malnutrition

Pathologicalstates

Cushing'ssyndrome

Diabetesmellitus

Hyperthyroidism

Severechronicdisease

Glucocorticoidresistance

Psychologicalstates

Anorexianervosa

Panicdisorder

Melancholicdepression

Obsessive-compulsivedisorderCausesofhypercortisolism47不同病因库欣综合征的鉴别不同病因库欣综合征的鉴别48Transphenoidalsurgery:successrate：approximately90%LargetumoursoccasionalrequireopensurgeryviatheanteriorfossaPost-operativeradiotherapyoccasionallyrequiredIfpituitarysurgeryfailsneedtoconsiderbilateraladrenalectomy25%patientsdevelopNelson'ssyndromeafterbilateraladrenalectomyAdrenaladenomasrequireadrenalectomyPerformedeitherlaparoscopicallyorviaopensurgeryOpensurgerycanbeperformedviaatrans