内科学 内分泌总论-高鑫

1,高鑫GaoXinDepartmentofEndocrinologyZhongshanHospital,FudanUniversityGao.xinzs-,PrinciplesofEndocrinology,2,Threestagesofendocrinologydevelopment内分泌学发展三阶段,腺体内分泌学OrganicEndocrinology组织内分泌学HistologicalEndocrinology分子内分泌学MoleculerEndocrinology,3,PrinciplesofEndocrinology,Basicofendocrinology_StructureandfunctionofEndocrinologySystem1.Endocrineglandsandcells2.Hormonesecretioncell3.Definitionofhormone:classificationSynthesisandEliminationDiurnalrhythmsVariation4.Correlationwithneuralandimmunesystem5.RegulationofendocrinesystemsAxisragulationRegulationbetweenhormones6.MechanismsofHormoneActionHormoneswithCellSurfaceReceptors:4typesHormoneswithIntracellularReceptorsDownstreamSignalingPathways7.NutrimentmetabolismClinicalendocrinologyCharacteristicofendocrinesystems:PrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatment,4,PrinciplesofEndocrinology,一、内分泌学基础-内分泌系统结构与功能一、内分泌腺和激素分泌细胞二、激素分泌细胞的结构特点三、激素定义：分类激素的合成与贮存激素分泌方式激素转运激素降解与转换激素的分泌节律血浆激素组分的不均一性激素合成与分泌的调节途径激素与神经系统及免疫系统的相互联系四、内分泌调节轴与调节系统内分泌调节轴激素间的相互调节与影响五、激素作用机制主要作用于核转录因子的激素主要与靶细胞表面受体结合的激素：激素-膜受体相互作用；膜受体结构：一般将膜受体分为四类激素信号的受体后转导通路六、营养素代谢二、临床内分泌内分泌系统疾病特点：诊断原则：诊断包括功能诊断、定位诊断诊断和病因诊断（解剖诊断）治疗原则治原则：,5,PrinciplesofEndocrinology,Basicofendocrinology_StructureandfunctionofEndocrinologySystem1.Endocrineglandsandcells2.Hormonesecretioncell3.Definitionofhormone:classificationSynthesisandEliminationDiurnalrhythmsVariation4.Correlationwithneuralandimmunesystem5.RegulationofendocrinesystemsAxisragulationRegulationbetweenhormones6.MechanismsofHormoneActionHormoneswithCellSurfaceReceptors:4typesHormoneswithIntracellularReceptorsDownstreamSignalingPathways7.NutrimentmetabolismClinicalendocrinologyCharacteristicofendocrinesystems:PrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatment,6,EndocrineSystem,Endocrineglands,Hormonesandtheirregulationsystem,MetabolicProcess代谢过程,OrgansFunction器官功能,Growthanddevelopment生长发育,Reproductionandaging生殖衰老,DiffusednerveandendocrineCellsinothertissue,NervousSystem,7,EndocrineSystem,Endocrineglands,Hormonesandtheirregulationsystem,MetabolicProcess代谢过程,OrgansFunction器官功能,Growthanddevelopment生长发育,Reproductionandaging生殖衰老,DiffusednerveandendocrineCellsinothertissue,NervousSystem,8,PrinciplesofEndocrinology,Endocrineglands,9,EndocrineSystem,Endocrineglands,Hormonesandtheirregulationsystem,MetabolicProcess代谢过程,OrgansFunction器官功能,Growthanddevelopment生长发育,Reproductionandaging生殖衰老,DiffusednerveandendocrineCellsinothertissue,NervousSystem,10,DiffusednerveandendocrineCellsinothertissue,PrinciplesofEndocrinology,11,PrinciplesofEndocrinology,BasicofEndocrinologyisthestudyof:(1)hormones(2)theirreceptors(3)andtheintracellularsignallingpathwaysClinicalendocrinologyisthestudyofendocrinediseases(1)hormoneoverproduction;(2)hormoneunderproduction;(3)alteredtissueresponsestohormones;(4)tumorsofendocrineglands.,12,PrinciplesofEndocrinology,Definitionofhormone,Hormonesarechemicalmessengerssecretedintobloodorextracellularfluidbyonecellthataffectthefunctioningofothercells.,Whatexactlyarehormones?,13,14,15,PrinciplesofEndocrinology,16,PrinciplesofEndocrinology,17,PrinciplesofEndocrinology,18,PrinciplesofEndocrinology,Hormonesclassification,Mostcommonly,hormonesarecategorizedintofourstructuralgroups:Peptidesandproteins:ACTH,LH,FSH,PHT,TSH,Insulin,Glucagon,IGFsSteroids:cortisone,aldosterone,1,25(OH)2D3,androgen,estrogenAminoacidderivatives:catecholamine,noradrenalin,adrenalinFattyacidderivatives:prostaglandings,Retinol,19,Regulationofendocrinesystems,20,Regulationofendocrinesystems,21,22,AnteriorPituitaryHormones,23,MechanismsofHormonesActionandintracellularsignallingpathways,与膜受体结合G蛋白偶联发挥生物效应,（肽类激素、生物胺、前列腺素）,与膜受体结合受体自身磷酸化发挥生物学效应（酪氨酸激酶）（生长因子家族、Insulin,IGFs),与核受体结合与DNA特异序列结合功能蛋白转录（甾体类激素）,24,MechanismsofHormonesActionandintracellularsignallingpathways,25,G-proteincoupled7membranespanningreceptor,26,G-proteincoupled7membranespanningreceptor,27,MechanismsofInsulinActionandintracellularsignallingpathways,28,MechanismsofNuclearReceptorAction,29,30,Threestagesofendocrinologydevelopment内分泌学发展三阶段,腺体内分泌学OrganicEndocrinology组织内分泌学HistologicalEndocrinology分子内分泌学MoleculerEndocrinology,31,PrinciplesofEndocrinology,TedRyder-oneofthefirstpatientsoftype1diabetestreatedbyinsulinin1922,32,PrinciplesofEndocrinology,J.J.R.Macleod(1876-1935),FrederickG.Banting(1891-1941),JamesB.Collip(1892-1965),CharlesHBest(1899-1978),TheNobelprizein1923toBantingFGforhiscontributiontodiscoveryofinsulin,33,Coris:发现了磷酸化酶的可逆磷酸化（无活性的磷酸化酶b/有活性的磷酸化酶a之间的互变）获得1951年诺贝尔奖。Sutherland:成功分离和确定的腺苷酸环化酶和磷酸二酯酶（cAMP合成与分解的两个关键酶）提出了激素作用的第二信使学说获得1971年诺贝尔生理医学奖。Krebs&Fisher:于60年代末发现蛋白激酶A(PKA)（依赖cAMP,刺激多种底物蛋白磷酸化)阐明了PKA启动的磷酸化和去磷酸化途径。获得1992年诺贝尔生理医学奖。,MechanismsofHormonesActionandintracellularsignallingpathways,34,PrinciplesofEndocrinology,Basicofendocrinology_StructureandfunctionofEndocrinologySystem1.Endocrineglandsandcells2.Hormonesecretioncell3.Definitionofhormone:classificationSynthesisandEliminationDiurnalrhythmsVariation4.Correlationwithneuralandimmunesystem5.RegulationofendocrinesystemsAxisragulationRegulationbetweenhormones6.MechanismsofHormoneActionHormoneswithCellSurfaceReceptors:4typesHormoneswithIntracellularReceptorsDownstreamSignalingPathways7.NutrimentmetabolismClinicalendocrinologyCharacteristicofendocrinediseases:PrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatmentExpectation,35,CharacteristicsofendocrineDiseasesPrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatment,Clinicalendocrinology,36,CharacteristicsofendocrineDiseasesPrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatment,Clinicalendocrinology,37,Classificationofendocrindiseases,按病变器官分类按功能分类：亢进、减退、正常；按病变部位分类：原发性、继发性、三发性,激素分泌缺陷受体基因缺陷-激素抵抗,38,Acromegaly肢端肥大症,Dwarfism侏儒症,Characteristicsofendocrinediseases,39,HyperthyroidismGravesDiaease,HyperthyroidismGravesDiaease,Characteristicsofendocrinediseases,40,Characteristicsofendocrinediseases,41,“Pear”obesity,“Appleobesity”,Fatdistrophy,Characteristicsofendocrinediseases,42,CharacteristicsofendocrineDiseasesPrinciplesofDiagnosis:Function,Localization,PathologicaldiagnosisPrinciplesofpreventionandtreatment,Clinicalendocrinology,43,内分泌疾病的诊断原则,三大原则：功能诊断定位诊断病因/病理诊断,44,功能诊断,症状、体征实验室资料：代谢紊乱的证据；激素分泌异常的证据；内分泌功能试验：兴奋试验