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1、Diseases of Respiratory System呼吸系统疾病呼吸系统疾病ANATOMY and FUNCTIONUpper respiratory tract nose, pharynx, larynx(鼻、咽、喉鼻、咽、喉)Lower respiratory tract trachea 气管气管 bronchi 支气管支气管 bronchiole 细支气管细支气管 Lobar 大叶大叶, segmental, lobular 小叶小叶Terminal airways and alveoli respiratory bronchiole alveolar sac alveolusn
2、Bronchialtree(通气）通气）15000L/dconductingportionFrommainbronchitoterminalbronchiolesnTerminalairways andalveoli(气体交换气体交换)respiratoryportionFromrespiratorybronchioletoalveoliDefenses MechanismNasalhairandTurbinates MucociliaryapparatusandcoughImmunoglobulin(IgG,IgM,IgA)ComplementproductionCytokines(IL-1
3、,TNF)AlveolarmacrophagesPolymorphonuclearleukocytesCell-mediatedimmunityBronchial tree 1.Mucosapseudostratifiedciliatedcolumncellgobletcell,producingmucusbasalcell-stemcellsmallgranulescellhighlyspecializedbronchialliningcells,containingneurosecretorygranules2.Submucosalglandserousandmucus3.Wallsmoo
4、thmuscle,contractileelasticfibers,provideflexibilitycartilageplate,forsupportBronchioles 细支气管细支气管 1mm1mm 1.MucosaciliatedepithelialcellClaracell(non-ciliatedsecretorycell)2.WallsmoothmusclenoglandnocartilageTerminal airways and alveoli Unitofgasexchangerespiratorybronchioles,alveolarducts,alveoliAlv
5、eolitypeIcells95%ofthesurface,40%oftheliningcellsgaspermeableAlveolitypeIIcells5%ofthesurface,60%oftheliningcellsproducingsurfactant,loweringthesurfacetensionInvolvedintherepairofalveolarepitheliumPulmonary vasculatureDoublebloodsupply,protectingfromischemiaPulmonarycirculation:functionalBronchialsy
6、stem:nutrientPulmonary lymph circulationDeepandsuperficialnetworkdrainingtothehilumlymphnodesNolymphaticsinmostalveolarwallsRmunicatingwithexternalenviromentsusceptibletothediseases2.bloodfrombodybiologicalpathogens/embolus3.relatedtoheartlocation/pulmonarycirculation.4.specificdiseasesdamagetothewa
7、llofbronchialtree,obstructionofbronchiolesdisintegrationofalveolar/capillarymembraneAetiologicalfactorDiseaseGeneticCysticfibrosis1-AntitrypsindeficiencySomeasthmaEnvironmentalSmokinglungcancerAirpollutionChronicbronchitisandemphysemaSusceptibilitytoinfectionOccupationSilicosisAsbestosis,mesotheliom
8、aandlungcancerInfectionInfluenzaMeaslesBacterialpneumoniasTuberculosisAtelectasis(collapse)ObstructivepulmonarydiseaseChronicbronchitisEmphysemaAsthmaBronchiectasisRestrictivepulmonaryAcuterespiratorydistresssyndrome(ARDS)DiffuseinterstitialpneumonaryfibrosisPneumoconioses(尘肺尘肺)PulmonaryinfectionPne
9、umoniaAbscess,TuberculosisTumorCorpulmonaleI.Pneumonia肺肺炎炎PneumoniacanbeverybroadlydefinedasanyinfectioninthelungAcute,fulminantorchronicHistologicspectrumFibrino/purulentalveolarexudateacutebacterialpneumoniasMononuclearinterstitialinfiltratesviralandotheratypicalpneumoniasGranulomaschronicpneumoni
10、asAcute bacterial pneumoniasLobarpneumoniaConsolidationofanentirelobeLobularpneumonia(Bronchopneumonia)ScatteredsolidfociinthesameorseverallobesA.Lobar pneumonia大叶性肺炎大叶性肺炎ConceptionContiguousairspacesofpartorallofalobearehomogeneouslyfilledwithanexudatethatcanbevisualizedonradiographsasalobarorsegme
11、ntalconsolidation.Adiseaseofacuteexudativeinflammation急性渗出性炎症急性渗出性炎症PathogenesisHealthyadultHostdefensesdepressedNormalinhabitantsoftheoropharynxandnasopharynxPneumococcus(I I、IIII、IIIIII型型) )Pathology and clinical features肺泡腔内急性渗出肺泡腔内急性渗出纤维蛋白、中性粒细胞纤维蛋白、中性粒细胞 Aratherclearcut4stagedbattleintheaffecte
12、dlunginaperiodabout78daysFourstages:CongestionRedhepatizationGrayhepatizationResolution Congestion (12d) GrossHeavy,red,boggyLMVascularcongestionProteinaceousfluidcontainingnumerouspneumococcifillingthealveoliScatteredneutrophilsClinicalfeatureAbruptonsetofhighfever,shakingchillspinkfrothysputumMois
13、traleChestradiograph:dim,uniformshadow Red hepatization (34d)GrossAliver-likeconsistency,granularAfibrinousorfibrinopurulentexudateofpleuraLMneutrophils,redcells,andfibrinpackingwithinalveolarspacesNumeruspneumococcidetectedClinicalfeatureRustysputumDyspnea,cyanosis,chestpainChestradiograph:asolidap
14、pearanceextendingtoentirelobesorsegmentsPulmonaryconsolidation(实化实化)Redhepatization Gray hepatization (46d) “Aturningpoint”GrossDry,gray,firm,granularLMRedcellslysedFibrinousexudateandneutraphilewithinalveoliNopneumococcidetectedClinicalfeaturesameasredhepatizationpurulentsputumhypoxiaimprovedLobarp
15、neumoniaGrayhepatizationGreyhepatization Resolution（after 1 week）GrossPleuralresolvedororganizedfibrousthickeningorpermanentadhesionsLMExudateswithinalveolienzymaticallydigestedEitherresorbedorexpectoratedClinicalfeaturesRecoveryComplicationsNotcommonCarnification(肺肉化肺肉化,organizingpneumonia）Pulmonar
16、yabscessandpyothoraxSepticemiaToxicpneumoniaCarnification肺肉化肺肉化B. Lobular pneumonia (Bronchopneumonia)initialinfectionofthebronchiandbronchiolesadjacentalveoliApurulentinflammationApatchydistributionofinflammationthatgenerallyinvolvesmorethanonelobeMostfrequentlybilateralandbasalPathogenesisOrganism
17、s:RelativelyavirulentPneumococcus,staphylococcus,andstreptococcus,etc.“Opportunisticinfection”OftenasecondarydiseaseTerminallyillpatients,infantsandtakingimmunosuppressivedrugs,etc.AspirationpneumoniaAcommoncauseofdeath“terminalpneumonia”Pathology and clinical featuresGrossInthelowerandposteriorport
18、ions1cm/34cm,graytoyellowConfluenceoffocitheappearanceofalobarconsolidationHyperemicandedematousScatteredirregularfociofpneumoniaarecenteredonterminalbronchiolesandrespiratorybronchiolesLobularpneumoniaLMFocalsuppurativeexudatefillingthebrochi,bronchiolesandadjacentalveolarspacesClinicalfeaturesOnse
19、tdelitescenceCoughMucosalfluidsputumBubbleX-ray:fociofsolidComplicationsCommonPoorinprognosis“terminalpneumonia” RespiratoryinsufficientCardiacinsufficientPulmonaryabscessandpyothoraxSolidfibroustissueMeningitis,arthritis,andinfectiveendocarditisComparison between lobar and lobular pneumonialobarlob
20、ularOnset PrimarySecondaryAgeHealthyadultsOfteninfantsandtheelderlyBactMostlypneumococcusOftencommensalsProgAcompleterecovery Poorinprognosis,“terminalpneumonia”PathLMFibrinou-purulentPurulentGross LowerormiddlelobeBilateralandbasalLiver-likePachydistributionC. Interstitial pneumonia Mononuclearinfl
21、ammatoryinfiltrationinpulmonaryinterstitiumAtypicalpneumoniaModestsputumproductionNophysicalfindingsofconsolidationWhitecellcountmoderatelyelevatedPathogenesis MycoplasmathemostcommoncauseViruses,chlamydiaeandrickettsiae,etc.Aprimarilyupperrespiratorytractinfectionwithcoryza,pharyngitis,laryngitisan
22、dtracheobronchitisMycoplasma:Children&youngadultsSporadicallyoraslocalepidemicViralinfectionsatanyagePathologyGrossPatchy,wholelobesbilaterallyorunilaterallyRed-yellow,congestedandsubcrepitant(捻发音捻发音)LMSeptawidenedandedematousAmononuclearinflammatoryinfiltrateFreeofcellularexudateDiffusealveolardama
23、gewithhyalinemembranesinseverecasesAmixedhistologicpicturewithsecondaryinfectionCytomegalovirusInfectionsClinical courseExtremelyvariedAsevereupperrespiratorytractinfectionChestradiographsTransient,ill-definedpatchesmainlyinthelowerlobesPhysicalfindingscharacteristicallyminimalPrognosis Good,Complet
24、erecoveryMostseriousinfectionscomplicatedbybacterialsuperinfection,PoorinprognosisD. SARS(Severe acute respiratory syndrome)FirstidentifiedinNovember2002inChinaSARScoronavirusLaboratorydiagnosticcriteriaSerologicaltestofanti-SARSCoVClinicalfeaturesFulminant,fever,contagiousRapidlyprogressingtosevere
25、respiratorysyndromePathologicalfeatureSevereatypicalpneumoniacoronavirusalveolardamage. Atelectasis/Collapse 肺不张肺不张/肺萎陷肺萎陷Loss of lung volume caused by inadequate expansion of airspace. 含气肺组织容积减少，甚至消失。 Complicate many primary lung disordersCategories:nResorption atelectasis 吸收性肺不张nCompression atelec
26、tasis 压迫性肺不张nMicroatelectasis 微小肺不张nConstraction atelectasis 缩窄性肺不张ObstructivepulmonarydiseaseAsthmaChronicbronchitisEmphysemaBronchiectasisRestrictivepulmonaryAcuterespiratorydistresssyndrome(ARDS)DiffuseinterstitialpneumonaryfibrosisPneumoconioses(尘肺尘肺)Diffuse pulmonary diseases:. Obstructive pulm
27、onary diseases 阻塞性肺部疾病阻塞性肺部疾病 resistancetorespiratoryairflowForced vital capacity用力肺活量 FVCForced expiratory volume at 1 second FEV1一秒钟肺呼气容积 FEV1/ FVCIncludingtumor,foreignbodyCOPDAsthmaChronicbronchitisEmphysemaBronchiectasisAirflowcanbereducedintwoways: resistancetoairflowNarrowedairwaysasthma(smal
28、lairway)chronicbronchitis/Bronchiectasis outflowpressureLossofelasticrecoilemphysema“Smallairwaydisease”Obstructionofsmallbronchioles2mmNarrowchannelThinwallLackofcartilageLessciliatedcellsA. Asthma 哮喘哮喘Characterized by episodic, reversible bronchospasm resulting from an exaggerated bronchoconstrict
29、or response to various stimuliClinical : episodic dyspnea, cough, wheezing5% adults 7-10% childrenPathogenesis bronchial inflammationExtrinsicasthma:ExtrinsicantigentypeIhypersensitivityreactionIntrinsicasthma:nonimmuneAspirin,Infections,Cold,psychologystress,exercise,SO2A. Asthma 哮喘哮喘Chronic obstru
30、ctive pulmonary diseases (COPD) 慢性阻塞性肺部疾病慢性阻塞性肺部疾病ChronicbronchitisEmphysema10% US adult and 4th death causeAnatomic distribution of pure chronic bronchitis and pure emphysema B. Chronic bronchitis Chronic inflammation of trachea and bronchiDefined clinically as the presence of a persistent producti
31、ve cough without a discernible cause for at least 3 consecutive months in at least 2 consecutive yearsPathogenesisPhysicalandchemicalfactorsCold,humidCigarettesmoking,airpollution(SO2,NO2,Cl2,etc.)Infections:Bacteria,virusInflammationvMucousepitheliuminjuredandmetaplasiaciliamovementvInflammationext
32、endinghypersecretionofmucousglandssmoothmuscleandelasticfibersinjuredvMucuspluggingthickenbronchiolesairwaynarrow/obstructionemphysemavIncapabilityofIgAsynthesisorphagocytosisForms:Simplechronicbronchitis:mucoid sputum, no airflow obstructionChronicmucopurulentbronchitispus sputum (secondary infecti
33、on)Chronicasthmaticbronchitishyperresponsive airway, asthmaChronicobstructivebronchitischronic outflow obstructionPathology GrossMucousmembraneduskyred(hyperemic)andswollenbyedemafluidLumenfilledwithmucusandpusDilatedbronchialglandducts“Pits”onthesurfaceofthebronchialepitheliumLMEpithelialdamageAlte
34、rnationofciliaEpithelialcell-degenerativeIncreasednumberofgobletcellsSquamousmetaplasiaHypertrophyofmucinousglands“TheReidindex”increasesizeofmucousglandsInflammationinthewallInfiltrationofLCandmacrophageSmoothmusclehypertrophy,hyperplasiaDestructionofelasticfibersFibrosisDestructionofcartilageAtrop
35、hy,degenerationExpiratoryoutflowobstructionWhereasthedefiningfeatureofchronicbronchitisisprimarilyareflectionoflarge bronchialinvolvement,themorphologicbasisofairflowobstructioninchronicbronchitisismoreperipheralandresultsfrominflammation,fibrosis,andresultantnarrowingofbronchioles(“smallairwaydisea
36、se”)andcoexistentemphysema.Clinical course AprominentcoughandtheproductionofsputumMoresevereinthewintermonthsFromhibernaltoperennialAccompaniedbyhypercapnia,hypoxemia,cyanosisandemphysemaComplicatedbypulmonaryhypertension&cardiacfailure(corpulmonale)Recurrentinfectionsandrespiratoryfailure Complicat
37、ionvInvolvesmallairwaybronchiolitis,lobularpneumoniaemphysema,atelectasis,bronchiectasisvVasculitis,periangiitisvCorpulmonale C. Emphysema （肺气肿）（肺气肿）DefinedasastateofpathologicallyincreasedinflationofterminallungunitPermanentenlargementoftheacinuswithdestructionoftheirwallsbutwithoutfibrosisOverinfl
38、ationwithoutdestructionofwallAclearassociationbetweenheavycigarettesmokingandemphysemapathologypathologyCentriacinarPanacinarDistalacinar Centriacinar type CentralorproximalpartsoftheaciniaffectedwhiledistalalveolisparedMorecommonandsevereintheupperlobes,particularlyintheapicalsegmentsSeverecases:Di
39、stalacinusalsoinvolvedAsequenceofcigarettesmokingCentrilobularemphysema Panacinar typeEntireAcinienlargedfromtherespiratorybronchioletotheterminalblindalveoliMorecommonlyinthelowerlungzonesPaleandvoluminouslungsoftenobscuringtheheartinautopsy1-antitrypsindeficiencyPanacinar emphysema Distal acinar (
40、paraseptal) typeDistributionalongthelobularseptaAtthemarginsofthelobulesandadjacenttothepleuraAdjacenttoareasoffibrosis,scarring,oratelectasisMoresevereintheupperhalfofthelungsSometimesformingcystlikestructures(bullae)BullaeLM肺泡壁变薄和断裂肺泡弹力纤维破坏肺泡道、囊和肺泡扩张肺泡壁毛细血管床受压气管和支气管炎症PathogenesisIncompleteobstruct
41、ionornarrowofbronchioleChronicbronchiolitisObstructionofthelumen resistancetotheexpiratoryairflowInjuryofalveolarwall1-antitrypsindeficiencyPathogenesisClinical featuresDyspnea(呼吸困难呼吸困难):usualfirstsymptomCyanotic(紫绀紫绀),headache,heartrateSomnolence(嗜睡嗜睡),clubbedfinger(杵状指杵状指)hyperventilationBarrel-ch
42、est(桶状胸桶状胸)Corpulmonalesecondarypulmonaryhypertensionspontaneouspneumothorax(自发性气胸自发性气胸)clubbedfinger(杵状指杵状指)D. Bronchiectasis（支气管扩张症）（支气管扩张症）DefinitionThepermanent dilationofbronchiandbronchiolescausedbydestructionofthemuscleandelasticsupportingtissue,resultingfromorassociatedwithchronicnecrotizing
43、infections.secondarytopersistinginfectionorobstructionclinical:coughcopiousamountsofpurulentsputumCommoninolderchildrenandyoungadultsPathogenesis secondarytopersistinginfectionorobstructionBronchialobstructionTumors,foreignbodies,impactionofmucusLocalizedtotheobstructedlungsegmentCongenitalorheredit
44、aryconditionsCysticfibrosis,immunoglobulindeficienciesKartagenersydromeNecrotizingorsuppurativepneumoniaChildhoodpneumoniasthatcomplicatedmeasles,whoopingcoughandinfluenza PathologyGrossUsuallythelowerlobesbilaterallyDiffuseorsegmentalAirwaysdilatedextendingalmosttothepleuraDilationcylindricaltypesa
45、cculartypeBronchialmembraneroughed,redmucopusFociofcollapse,fibrosisoremphysema LMEpitheliumAtrophic,proliferativeorsquamousmetaplasiaDesquamation,necrosisandulcerationinacutephaseSubmucosaInfiltrationofmononuclearcellsWallDestructionofelasticfibers,musclesandevencartilageFibrosis,peribronchiolarfib
46、rosisArteriolitis(小动脉炎小动脉炎)andtrichodophlebitis(小静脉炎小静脉炎)Clinico-pathological correlationcoughwithexpectorationofmucopurulentHemoptysis(咯血咯血) Dyspnea,Cyanotic,clubbedfingerInfectionandintoxication(fever,weightlossandmalaise)ComplicatonAbscess(lungandbrain)EmpyemaofthechestCorpulmonale.Restrictivelun
47、gdiseasesReduced compliance (more pressure is required to expand the lungs because they are stiff)Forced vital capacity用力肺活量 FVC Forced expiratory volume at 1 second FEV1一秒钟肺呼气容积 FEV1/ FVC normal.RestrictivelungdiseasesA.AdultRespiratoryDistressSyndrome(ARDS)Brief introductionDiffusealveolardamageIn
48、juryoftherespiratorymembraneDevelopingrapidlyprogressiverespiratoryfailureAccompaniedbyDecreasedlungcomplianceHypoxemia(cyanosis)ExtensiveradiologicalopacitiesinbothlungsPathogenesis DirectLungInjuryIndirectLungInjuryCommonCausesPneumoniaSepsisAspirationofSeveretraumawithshockgastriccontentsUncommon
49、CausesPulmonarycontusionCardiopulmonarybypassFatembolismAcutepancreatitisNear-drowningDrugoverdoseInhalationalinjuryTransfusionofbloodproductsReperfusioninjuryUremiaafterlungtransplantationPathogenesis ActivationofthecomplementsystemSequestrationofneutrophilsandmacrophageOxygenradicalsHydrolyticenzy
50、mesCytokinesDamagetothecapillaryendotheliumDamagetotheepithelialjunctionsExudationoffluidandproteinsfromtheinterstitiumintothealveolarspacesPathology Exudativephase(17d)Hyperemia,Edema,hemorrhageAccumulationofinflammatorycellsHyalinemembranesNecrosisoftypeIcellsDenudedbasementmembranesFibrinthrombii
51、ncapillariesandarteriolesGross(湿肺湿肺)Wine,liver-like,large,heavyCutsurfaceconsolidation,dimfluid Hyaline membranes Eosinophilic, glassyProliferativephase(310d) ProliferationoftypeIIpneumocytesproliferationoffibroblastsandorganizationAlveolarseptathickenedGrossDiffusegray,lustering（carnification)Fibro
52、ticphaseDiffuseinterstitialfibrosisGrossHoneycomblung(蜂窝肺蜂窝肺)Multiplecyst-likespacesthroughoutthelungRemodelingofthelungarchitectureClinical feature Tachypnea(气促气促),dyspnea,cyanotic,etc.Radiologicallybilateraldiffuseshadow Prognosis Poor,progressive,50%diedinacutephaseScarredlungs,respiratorydysfunc
53、tion,pulmonaryhypertension,etc.Recoveringnormalfunction46monthslaterB. Idiopathic pulmonary fibrosis IPF 特发性肺纤维化特发性肺纤维化C. Silicosis 肺硅沉着病（硅肺）肺硅沉着病（硅肺）Pneumoconiosis(尘肺尘肺):non-neoplasticlungreactiontoinhalationofmineraldusts(coaldust,silica,asbestos)Occupationdisease(职业病职业病)云南十余返乡民工相继死亡云南十余返乡民工相继死亡n云
54、南省水富县向家坝镇自2003年起先后有77人到安徽凤阳县石英干粉厂务工。不少民工返乡后出现了一种症状相同的“怪病”咳嗽、全身浮肿等，陆续有12人死亡。n卫生部对这些返乡民工进行职业病诊断，结果如下：接受体检的63名存活村民，诊断为矽肺30例;死亡的12例中，有矽肺2例，高度怀疑矽肺合并肺结核1例，肺部感染1例，肺结核2例，其余6例死亡人员没有影像学资料，无法诊断。n患者李正友曾到凤阳石英干粉厂务工。“工厂到处弥漫着灰尘，在一个工作间作业的人互相都看不清。”刘树祥也曾到凤阳的石英厂打工，他说：“每天下班后，喉咙、鼻孔里都是粉尘。”Brief introductionInhalationofcry
55、stallinesilicaPathologically:numerousminutesilicoticfibroticnodules+diffusefibrosisinthelungrespiratoryinsufficiencynPersonsworkinginmine,sandblastingandstonecuttingwithoutprotectionnmorethan10y,usually2030ytodevelopsilicosis.nAslowprogressivecoursenthedurationtoexposuretheamountoftheinhaledparticle
56、sPathogenesis5mreachingtheterminalairways1mretainedandcausefibrosismacrophageengulfparticlesIncorporationofphagocytosedparticlewithlysosomeandformationofphagolysosomeDisruptionofphagolysosomewithreleaseoflysosomeenzymeDeathofmacrophagebylysisandsilicaparticlesreleaseFreeparticlere-ingestedbyanotherm
57、acrophageMacrophagemigratestothelymphaticandaccumulatesinrespiratorybronchioleandlymphnodeCellularaggregationofthemacrophageformedandlatelybecomescollgenousnodulesSilic acidLysozyme Mechanical stimulation Oxygen free radical and protease Chemokine Fibrogenesis factor derived from macrophage Immunolo
58、gic mechanism PathologyTwocharacteristicfeaturesSilicoticnoduleGrossTiny,barelypalpable,pale-to-blackenednodulesLMConcentricallyarrangedcollagenfibershyalinizedandsurroudingacentralbloodvesselWeaklybirefringentsilicaparticlesprimarilyinthecenterbypolarizedmicroscopicallyExpansionandcoalescence,Hard,
59、collagenousscarsSilicotic nodule (maturing) exhibits virtually acellular, hyalinized collagen and peripheral dust laden macrophages.DiffusefibrosisPulmonarymassivefibrosisFibrosisalsointhehilarlymphnodesandpleuraCalcificationStages and clinico-pathological correlationsStageISmallnodulesscatteringint
60、hehilarlymphnodesandcentralzonesofmiddleandlowerlobesLessfibrosis,withoutsymptomStageIILargernodules,coalescing,scatteringmiddleandlowerlobes(2cm),centralareanecrotic,calcifiedorwithcavityformationPulmonarymassivefibrosis(1/2lung)PleurathickeningandadhesionIrregularemphysemaandbullaeformationPlain c
61、hest radiograph of a patient with simple (uncomplicated) silicosis, manifested by micronodular polygonal interstitial pattern and eggshell calcifications in mediastinal and hilar lymph nodes.A patient with complicated silicosis associated with tuberculosis who was employed as a facade worker for 30
62、years. The radiograph shows fibrous retractive changes of hilipetal distribution, which leaves the periphery bullous or “empty.” Disseminated polygonal granulomas are seen as well as eggshell calcifications of mediastinal and hilar lymph nodes.Accelerated silicosis associated with rheumatoid arthrit
63、is.Plain chest radiograph showing tumor-like masses consistent with massive fibrosis, coarse reticular retractive changes, and small polygonal patchy opacifications.ComplicationsCorpulmonale(肺源性心脏病）肺源性心脏病）-chronichypoxia-inducedvasoconstrictionandparenchymaldestructionTuberculosismostcommon-adepress
64、ionofcell-mediatedimmunitySilicotuberculosis（硅肺结核病）（硅肺结核病）Spontaneouspneumothorax(自发性气胸自发性气胸). Cor pulmonale 肺源性心脏病肺源性心脏病 DefinitionRight ventricularhypertrophy,dilation,andpotentiallyfailuresecondarytopersistent pulmonary hypertensioncausedbydisordersofthelungs or pulmonary vasculature肺部慢性疾病胸廓运动障碍
65、肺动脉高压、右心肥大肺血管病变Causes and pathogenesisPrimarydiseaseswithinthelungCOPD,Silicosis,TuberculosisPrimarydiseasesofpulmonaryarteriesPrimarydisordersofthoracicskeletonAcute-PulmonaryembolismChronic-PulmonaryhypertensionthemostcommoncauseCOPD 慢支、肺气肿慢支、肺气肿 (84.1%)(84.1%) 肺结核肺结核 (5.90%)(5.90%) 哮喘哮喘 (4.43%)(4
66、.43%) 支气管扩张症支气管扩张症 (2.81%)(2.81%) 胸廓畸形胸廓畸形 (1.73%)(1.73%) 矽肺矽肺 (1.21%)(1.21%) 其他其他 结节性多动脉炎、原发性肺动脉高压症结节性多动脉炎、原发性肺动脉高压症 肺细动脉硬化症、多发性肺小动脉栓塞肺细动脉硬化症、多发性肺小动脉栓塞 胸膜广泛性纤维化胸膜广泛性纤维化Chronicbronchitisbronchiolititsventingairexchangehypoxia,arteriospasm,angiosclerosisarteriolitislumennarrowPulmonaryhypertensionemp
67、hysemacapillarybedPathologyChangesoftheheartIncreaseweightoftheheartHypertrophyofrightheartApexformedbytherightheartisbluntBulgingofcornuspulmonalePulmonaryencephalopathyClinico-pathological correlationsRight-sidedheartfailureTachycardia,cyanosis,swellingoflowerextremityRespiratoryinsufficiencyHeada
68、che,convulsion,coma. Tumors of respiratory system呼吸系统肿瘤呼吸系统肿瘤 A. Nasopharyngeal carcinoma40yearsmenwomensmoking PathogenesisEBvirus Pathology部位部位顶部顶部 外侧壁外侧壁 咽隐窝咽隐窝 前壁前壁 巨体巨体 结节型、菜花型、溃疡型、粘膜下型结节型、菜花型、溃疡型、粘膜下型 组织学分型组织学分型 （参照（参照RobbinsRobbins） 鳞癌鳞癌( (高高/ /低分化低分化) ) 腺癌腺癌( (高高/ /低分化低分化) ) 未分化细胞癌（类似于书上的泡状核
69、细胞癌，未分化细胞癌（类似于书上的泡状核细胞癌，最常见，与最常见，与EBVEBV关系最密切）关系最密切） 浸润及转移浸润及转移 直接浸润：向上直接浸润：向上颅底骨颅底骨( (卵圆孔破坏多见卵圆孔破坏多见) ) 向外向外咽骨管（听力障碍）咽骨管（听力障碍） 向前向前鼻腔（鼻塞）鼻腔（鼻塞） 淋巴道转移：（早期即发生）淋巴道转移：（早期即发生） 咽后咽后LNLN颈上颈上LNLN颈内颈内V V旁旁LNLN（多数发生在同侧，双侧少见）（多数发生在同侧，双侧少见） 血道转移：肝、肺、肾血道转移：肝、肺、肾nonkeratinizingundifferentiatedcarcinoma(lymphoepi
70、theliomas)Clinico-pathological correlationsBacksiphonicnasaldischargetackwithblood(回吸的涕带血回吸的涕带血)最早期最早期Deradenoncus(颈淋巴结肿大颈淋巴结肿大)tinnitus(耳鸣耳鸣),nasalobstruction(鼻塞鼻塞)Treatment Resectionand/orradiotherapy敏感程度：泡状核敏感程度：泡状核 低鳞低鳞 未分化癌未分化癌B. Primary lung cancerBronchogenic carcinomasArisingfromthebronchial
71、epithelium95%theleadingcauseofcancerdeathsPeakincidence5565yearsMale:female=4:1Etiology and pathogenesisCigarettesmoking烟草中致癌剂：苯并芘、亚硝酸、苯酚、砷、尼古丁烟草中致癌剂：苯并芘、亚硝酸、苯酚、砷、尼古丁 支气管上皮：鳞化、不典型增生、原位癌支气管上皮：鳞化、不典型增生、原位癌 主要为鳞形细胞癌、小细胞癌主要为鳞形细胞癌、小细胞癌Environmentalinsults(occupationandaircontamination)3 3，4 4苯并芘苯并芘 二氧化硫二
72、氧化硫铀、石棉、砷、铬、镍、氯甲醚、氧化铁粉铀、石棉、砷、铬、镍、氯甲醚、氧化铁粉Etiology and pathogenesisgeneticabnormalities小细胞癌第小细胞癌第3 3对染色体短臂对染色体短臂 （抑癌基因）的脱失（抑癌基因）的脱失 腺癌常有腺癌常有EGFEGF受体的过度表达受体的过度表达pulmonarydiseases 疤痕、炎症疤痕、炎症 石棉肺合并肺癌石棉肺合并肺癌1515 硅沉着病合并肺癌硅沉着病合并肺癌10102020 结核合并肺癌结核合并肺癌1010goblet-cell hyperplasiasquamous dysplasiabasal cell
73、hyperplasia carcinoma-in-situsquamous metaplasiainvasive squamous carcinomaMorphologyGrossIntraluminallesionsTumorsarisingfromlargebronchusCentralzoneoflungTumorsarisingfromsmallerbronchiperipheralregionoflungDiffusetype中央型中央型( (主、叶或段支气管主、叶或段支气管) ) 管内型、管壁浸润型、管外浸润型管内型、管壁浸润型、管外浸润型 周围型周围型( (小、细支气管小、细支气
74、管) ) 近胸膜、孤立肿块、表面凹陷近胸膜、孤立肿块、表面凹陷 弥漫性弥漫性( (细支气管、肺泡细支气管、肺泡) ) 弥漫性、肺炎表现或多结节状弥漫性、肺炎表现或多结节状FourmajorhistologicaltypesNon-smallcellcarcinoma非非小细胞癌小细胞癌Squamouscellcarcinoma鳞状细胞癌鳞状细胞癌Adenocarcinoma腺癌腺癌Largecellcarcinoma大细胞大细胞( (未分化未分化) )癌癌Smallcellcarcinoma小细胞小细胞( (未分化未分化) )癌癌Acombinationofhistologicpatterns
75、Squamouscellcarcinomas（排第二）（排第二）ArisingcentrallyinmajorbronchiDisseminatinglaterthanothertypesGrossCentrallylocatedCentralnecrosis,cavitation,andhemorrhageDistalatelectasisandinfectionLMKeratinpearls,intercellularbridges 电镜：张力原纤维、桥粒电镜：张力原纤维、桥粒 淋巴道转移，预后相对较好淋巴道转移，预后相对较好Adenocarcinomas(排第一排第一)Mostcommo
76、nform女女 男男ArisingfromsmallbronchusMetastasizewidelyatanearlystageGrossPeripherallocatedLMAcinar(glandforming)orpapillary,orsolid 电镜：小腺腔、微绒毛电镜：小腺腔、微绒毛 血道转移较早血道转移较早( (肝、脑、骨等肝、脑、骨等) )原位腺癌原位腺癌（类似于书上的（类似于书上的细支气管肺泡癌）细支气管肺泡癌）DerivingfrombronchioleGrossmultiplediffusenodules,pneumonia-likeconsolidationLMTum
77、orscellsgrowingalongthealveolarsurfaceinsinglelayer,Preservationofaleolararchitecture癌细胞沿肺泡壁生长癌细胞沿肺泡壁生长 电镜：电镜：IIII型肺泡上皮细胞的板层结构型肺泡上皮细胞的板层结构Scarcarcinomas瘢痕癌瘢痕癌 近胸膜结节、表面凹陷近胸膜结节、表面凹陷 光镜：中央纤维疤痕光镜：中央纤维疤痕 腺癌多腺癌多 原发灶小就可全身广泛转移原发灶小就可全身广泛转移Largecellcarcinoma 周围型中央型周围型中央型 Anundifferentiatedtype Large,anaplasti
78、c,largevesicularnucleiwithprominentnucleoliGiantcellcarcinomaSpindlecellcarcinoma透明细胞癌、梭形细胞癌、巨细胞癌透明细胞癌、梭形细胞癌、巨细胞癌TendencytospreadtodistantsitesearlyPoorprognosisSmallcellcarcinoma DerivedfromneuroendocrinecellsEarlymetastasisandhighmalignancySensitivetochemotherapyGrossCentrallylocated,Palegray中央型周围
79、型中央型周围型LM燕麦细胞癌燕麦细胞癌Small,AroundtofusiformshapeScantcytoplasm,finelygranularchromatinNecrosismaybeextensive电镜：神经内分泌颗粒电镜：神经内分泌颗粒其他类型其他类型 腺鳞癌腺鳞癌 小细胞未分化癌小细胞未分化癌 + + 腺癌腺癌/ /鳞癌鳞癌 类癌类癌 (Caicinoid) (Caicinoid) 神经内分泌分化标志神经内分泌分化标志 预后好预后好(5(5年生存率年生存率95%)95%)NSEClinical featuresBloodyphlegm(痰血痰血)Cough(咳嗽咳嗽)Brea
80、thlessness(气急气急)Pneumoniaandabscess(肺部感染）肺部感染）Bloodyhydrothorax(血性胸水血性胸水)Hoarsevoice(声音嘶哑声音嘶哑)Dysphagia(吞咽困难吞咽困难)HornerSyndrome（同侧上眼睑下垂、瞳孔缩小、同侧上眼睑下垂、瞳孔缩小、皮肤无汗皮肤无汗)Diagnosis ClinicalmanifestationClinicalmanifestationX-rayX-rayPathologicalexaminationExfoliativecytologyxfoliativecytologyofsputumBiopsyb
81、ybronchoscope扩散和转移扩散和转移 1 1、直接蔓延、直接蔓延 肺内支气管、肺门、纵隔、心包肺内支气管、肺门、纵隔、心包 胸膜、横膈胸膜、横膈 喉返神经、食管喉返神经、食管 2 2、淋巴道播散、淋巴道播散 肺门、纵隔、隆突下、气管旁肺门、纵隔、隆突下、气管旁LNLN 横膈、腹腔、腹主动脉旁横膈、腹腔、腹主动脉旁LNLN 胸膜淋巴渗透胸膜淋巴渗透扩散和转移扩散和转移3 3、血道转移、血道转移 脑、肾上腺、骨、肝、对侧肺脑、肾上腺、骨、肝、对侧肺鳞癌：直接蔓延、淋巴道转移鳞癌：直接蔓延、淋巴道转移腺癌、未分化癌：血道转移、淋巴道转移腺癌、未分化癌：血道转移、淋巴道转移治疗治疗 非小细胞
82、癌：手术首选非小细胞癌：手术首选 小细胞癌：放、化疗手术放、化疗小细胞癌：放、化疗手术放、化疗组织发生组织发生 鳞癌：基底细胞鳞化不典型增生鳞癌：基底细胞鳞化不典型增生 腺癌：支气管上皮、腺上皮、腺癌：支气管上皮、腺上皮、 ClaraClara细胞、肺泡细胞、肺泡型上皮型上皮干细胞学说（干细胞学说（GazdarGazdar）：）：干细胞分化潜能的保持（大细胞癌）干细胞分化潜能的保持（大细胞癌） 恶性细胞恶性细胞 鳞状分化（鳞癌）鳞状分化（鳞癌） （大细胞）腺状分化（腺癌）（大细胞）腺状分化（腺癌） 神经内分泌分化神经内分泌分化 （小细胞癌）（小细胞癌）（多种成分、小细胞癌鳞化、型间转化、标记）（多种成分、小细胞癌鳞化、型间转化、标记）QuestionsnPlease describe differences among lobar,lobular and interstitial pneumonianPlease describe the morphology of chronic bronchitis. What lesions it can develop to? And describe the morphology characteristics of them.
