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呼吸系统疾病TheRespiratorySystem,第四章,1,2,快速气体交换RapidgasexchangeVentilationPerfusionDiffusion清洁空气,肺的结构与功能,3,Mucosa,Submucosa,Cartilage,Muscles,Bronchus,4,Bronchialepithelium,5,Kulchitskycells,6,BronchialSubmucosalGlands,7,Alveoli,8,Therespiratorymembrane,9,病原体Pathogen空气contaminatedairexposure鼻咽部菌群nasopharyngealfloraaspiration宿主防御功能下降Compromiseddefensemechanisms肺部疾病othercommonlungdisease免疫缺陷Immunodeficiency生活方式Lifestyle,肺部感染PulmonaryInfections,10,11,分类Classification,根据解剖和影像学Anatomyandradiography根据病因Etiology根据在哪获得SettinginwhichtheyariseCommunity-AcquiredHospital-acquired(nosocomial),12,一、细菌性肺炎,大叶性肺炎(95%肺炎链球菌)小叶性肺炎军团菌性肺炎(肺炎+全身毒血症状),13,小叶性肺炎,大叶性肺炎,14,患者杨某,男,20岁,学生。酗酒后遭雨淋,于当天晚上突然起病,寒颤、高热、呼吸困难、胸痛,继而咳嗽,咳铁锈色痰,其家属急送当地医院就诊。听诊,左肺下叶有大量湿性啰音;触诊语颤增强;血常规:WBC:17X109/L;X线检查,左肺下叶有大片致密阴影。,典型病例1,15,入院经抗生素治疗,病情好转,各种症状逐渐消失;X线检查,左肺下叶的大片致密阴影缩小2/3面积。病人于入院后第7天自感无症状出院。冬季征兵体检,X线检查左肺下叶有约3cmX2cm大小不规则阴影,周围边界不清,怀疑为“支气管肺癌”。在当地医院即做左肺下叶切除术。病理检查,肺部肿块肉眼为红褐色肉样,镜下为肉芽组织。,16,大叶性肺炎,17,大叶性肺炎,18,大叶性肺炎,19,Intheerabeforeantibiotics,充血水肿期Hyperemiaandedema红肝期Redhepatization灰肝期Grayhepatization溶解消散期Resolution(hopefully),20,Hyperemiaandedema,12-24hours,21,Redhepatization2-3days,22,23,24,25,Grayhepatization,3-4days,26,27,28,并发症complications,肺脓肿,脓胸,脓气胸败血症,脓毒败血症,感染性休克肺肉质变,29,小叶性肺炎,30,小叶性肺炎,31,Filledwithexudate,Aeratedlung,小叶性肺炎,32,小叶性肺炎,33,并发症Complications,心力衰竭、呼吸衰竭肺脓肿、脓胸、脓毒败血症支气管扩张,34,Organization,35,Abscessformation,36,Abscessformation,37,Purulentpleuritis,38,HemorrhagicPneumoniabyPseudomonasaeruginosa,39,Pseudomonaspneumonia,40,Klebsiellapneumonia,41,二、病毒性肺炎/三、支原体性肺炎,轻者为急性间质性肺炎,旧称不典型性肺炎atypical缺乏实变体征WBC轻度升高重者为急性呼吸窘迫症ARDS肺泡弥漫性损伤透明膜形成,42,Viralpneumonia,43,Atypicalpneumonia,44,SevereAcuteRespiratorySyndrome(SARS),45,PneumoniaintheImmunocompromisedHost,机会致病原opportunisticagents常为多重感染bacteria(P.aeruginosa,Mycobacteriumspp.,L.pneumophila,andListeriamonocytogenes)viruses(cytomegalovirusandherpesvirus)fungi(P.jiroveci,Candidaspp.,Aspergillusspp.,andCryptococcusneoformans),46,CMVinfections,47,48,49,insituhybridization,50,SputumcytologyfromanAIDSpatient,(Papanicolaou),(Giemsa),51,PneumocystisPneumonia,52,53,(silverstain),PneumocystisPneumonia,54,(silverstain),PneumocystisPneumonia,55,PneumocystiscariniiinbronchiallavagefromanAIDSpatient(Grocottssilver),56,(Papanicolaou),(Immunostaining),57,慢性阻塞性肺病(COPD)ChronicObstructivePulmonaryDisease,limitationofairflowusuallyresultingfromanincreaseinresistancecausedbypartialorcompleteobstructionatanylevel肺气肿Emphysema慢性支气管炎Chronicbronchitis支气管扩张Bronchiectasis哮喘Asthma,58,FEV1/FVC,59,60,我国国家“十五”课题最新统计数据(2005年公布)显示40岁以上人口COPD患病率为8%。估计全国有2500万人罹患此病,每年因COPD死亡的人数达100万,致残人数达5001000万,COPD居我国疾病负担的首位。,61,慢性支气管炎Chronicbronchitis,Apersistentcoughproductiveofsputumforatleast3months,inatleast2consecutiveyears单纯型Simple喘息型Asthmatic阻塞型obstructive,62,病因etiology,吸烟和空气污染Cigarettesmokingandairpollutants感染MicrobialinfectionSecondaryrole使炎症持续,加重症状maintainingtheinflammationandexacerbatingsymptoms,63,发病机制Pathogenesis,多痰粘液分泌亢进Hypersecretionofmucus大气道病变largebronchialinvolvement粘液腺增生,杯状细胞分泌亢进Hypertrophyofmucousglandsandamarkedincreaseinmucin-secretinggobletcells,64,气道阻塞Airflowobstruction小气道炎症和管壁纤维化inflammation,bronchiolarwallfibrosis杯状细胞化生,细支气管痰栓形成gobletcellmetaplasiawithmucuspluggingofthebronchiolarlumen并发肺气肿coexistentemphysema,发病机制Pathogenesis,65,innerperichondrium,basallamina,Chronicbronchitis,Normalbronchus,66,Chronicbronchitis,67,Chronicbronchitis,68,Chronicbronchiolitis,luminalandmucus,chronicinflammation,69,肺气肿Emphysema,Abnormalpermanentenlargementoftheairspacesdistaltotheterminalbronchioles,accompaniedbydestructionoftheirwallswithoutobviousfibrosis指呼吸性细支气管、肺泡管、肺泡囊、肺泡因组织弹性减弱而过度充气伴有肺泡间隔破坏,致使肺容积增大的病理状态,70,病理类型,腺泡(小叶)中央型Centriacinar全腺泡(小叶)型Panacinar腺泡(小叶)周围型Distalacinar不规则型Irregular,obstruction,Spontaneouspneumothorax,asymptomatic,71,72,发病机理Pathogenesis,73,Centriacinaremphysema,74,75,Bullousemphysema,76,Centriacinaremphysema,77,78,InadequateventilationLessperfusionNarrowedbronchiole,79,ConditionsRelatedtoEmphysema,间质性肺气肿Mediastinal(interstitial)emphysema代偿性肺气肿Compensatoryemphysema阻塞性过充气Obstructiveoverinflation瘢痕旁肺气肿Paracicatricalemphysema老年性肺气肿Senileemphysema,80,81,COPD的临床表现,Respiratoryfailure,Rightheartfailure,82,Pinkpuffer,桶状胸Barrelchest,83,支气管扩张症Bronchiectasis,Permanentdilationofbronchiandbronchiolescausedbydestructionofthemuscleandelasticsupportingtissueresultingfromorassociatedwithchronicnecrotizinginfections继发于持续性感染或阻塞Secondarytopersistinginfectionorobstruction诊断依靠病史和影像学,84,Predisposingconditions,支气管阻塞Bronchialobstruction肿瘤Tumor异物Foreignbodies先天性或遗传性疾病CongenitalorhereditaryconditionsCysticfibrosisKartagenersyndrome坏死性化脓性肺炎Necrotizing,orsuppurative,pneumonia,85,CysticFibrosis(CF),86,CysticFibrosis(CF),87,Bronchiectasis,57yearoldwomanwithalonghistoryofrecurrentrespiratorytractinfectionsandepisodesofhemoptysis,88,Bronchiectasis,89,BronchiectasisDuetopneumonia,90,Bronchiectasis,91,Bronchiectasis,PurulentexudationinthelumenDestructionofliningepitheliumScarringofthebronchialwall,92,HRCTscansofbronchiectasis,93,支气管扩张症的临床表现,咳嗽,咳脓臭痰Severe,persistentcoughwithmucopurulentsputum,sometimesfetid咯血Flecksofbloodinsputumorfrankhemoptysis严重病例及合并症,94,肺间质疾病ILD,肺弥漫性纤维化肺顺应性减弱,容量减小已知病因和特发性间质性肺炎,95,SelectedcausesofchronicinterstitiallungdiseaseOccupationalandenvironmentalexposureAsbestosisSilicosisHypersensitivitypneumonitisDrugortreatmentrelatedChemotherapeuticagentsIonizingirradiationOxygenImmunologiclungdiseaseSarcoidosisWegnergranulomatosisCollagenvasculardiseaseGoodpasturesyndromeMiscellaneousPostacuterespiratorydistresssyndromeIdiopathicpulmonaryfibrosis,96,杵状指(趾)Clubbingofdigits,97,Honeycomblung,98,99,矽肺Silicosis,职业病Occupationaldiseases吸入二氧化硅粉尘引起Inhalationofcrystallinesilica致残和致死Disablingandfatal病理:硅结节形成广泛的肺纤维化,100,101,发病机制Pathogenesis,直径5m的硅尘沉积于肺间质肺泡巨噬细胞引发和持续肺损伤和纤维化Alveolarmacrophageisakeycellularelementintheinitiationandperpetuationoflunginjuryandfibrosis,102,Silicosis,asliceoflungfroma61yoceramicsworker,103,矽结节Silicosis,104,105,临床表现Clinicalcourse,早期常为体检发现usuallydetectedinroutinechestradiographs晚期表现:呼吸困难shortnessofbreath肺心病corpulmonale合并TBincreasedsusceptibilitytoTB可能致癌carcinogenic(controversial),106,Silicosis,107,急性呼吸窘迫征(ARDS)AcuteRespiratoryDistressSyndrome,进行性呼吸衰竭AcuteonsetofDyspneaHypoxemiaBilateralpulmonaryinfiltrates(X-ray)Noevidenceofleft-sidedheartfailure病理:弥漫性肺泡损伤DiffuseAlveolarDamage(DAD)常伴多器官衰竭MultipleOrganDysfunctionSyndrome(MODS),108,109,发病机制Pathogenesis,广泛内皮和肺泡I型、II型上皮损伤Endothelialandepithelial(IandII)damage肺水肿alveolarflooding气体交换丧失lossofdiffusioncapacity表面活性物质异常surfactantabnormalities促炎和抗炎介质的失衡Imbalanceofpro-inflammatoryandanti-inflammatorymediators,110,111,病理变化Morphology,急性渗出期AcuteExudativeStage水肿Edema(interstitialandalveolar)透明膜形成Hyalinemembranes肺泡塌陷Manyalveolicollapse增生期ProliferativeStageII型肺泡上皮增生肺泡内机化Intra-alveolarfibrosis,112,wideningoftheinterstitiumARDS,113,Hyalinemembranes,114,Hyalinemembranes,115,Hyalinemembranes,116,Healingstage,117,Healingstage,118,suvivingpatientARDS,119,临床表现Clinicalfeatures,肺顺应性差Poorpulmonarycompliance难治性低氧血症Poorresponsetooxygen血管床进行性减少Pulmonaryvascularbedisprogressivelyobliterated易感染Pronetobacteriainfection,120,预后Prognosis,Dependssuccessfullytreatementsbeforeextensivefibrosis40-70%mortalityHigh-dosesteroidsfailedConservativetherapyhelpsConsiderableinterestinthequalityofsurvivals,121,LUNGTUMORS,MetastatictumorsPrimarytumorsBRONCHOGENICCARCINOMAOthersbronchialcarcinoidsmesenchymalmalignanciesLymphomasafewbenignlesions,95%,5%,122,慢性肺动脉高压症和肺心病(自学),概念病因发病机制病理变化临床病理联系,思考题:试述慢性支气管炎并发肺心病的发病机制。,123,肺癌,癌症死因第一位causeofcancer-relateddeaths发病率随年龄和烟龄增长pack-yearssmoked.确诊的病人一半以上已有远处转移distantmetastaticdisease5年生存率约为15%5-yearsurvival,124,125,126,127,肺癌的临床病理分型,肺小细胞癌Smallcelllungcancer(SCLC)肺非小细胞癌Non-small-celllungcancer(NSCLC)鳞癌Squamouscellcarcinoma腺癌Adenocarcinomas大细胞癌Largecellcarcinomas,128,SCLC,Kulchitskycell起源hADH(hyponatremia/waterintoxication)ACTH(Cushingssyndrome)中央型Centrallylocatedmasses坏死常见Necrosisisinvariablypresentandmaybeextensive燕麦细胞癌Oatcellcarcinoma,129,SCLC,130,Oatcellcarcinoma,SCLC,131,Oatcellcarcinoma,SCLC,132,鳞状细胞癌,病人大多有吸烟史Closelycorrelatedwithasmokinghistory中央型Tendtoarisecentrallyinmajorbronchi常见空洞Cavitationisnotuncommon副癌综合征Preneoplasticlesions,133,Squamouscellcarcinoma,134,Squamouscellcarcinoma,135,136,Squamouscellcarcinoma,137,Squamouscellcarcinoma,138,Squamouscellcarcinoma,139,140,141,142,143,144,Metaplaisa?Anaplasia?Dysplasia?,145,腺癌Adenocarcinoma,周围型多见peripherallylocated是女性和非吸烟者常见的肺癌类型常发生自肺周边部疤痕处arisinginrelationtoperipherallungscars生长缓慢但早期转移支气管肺泡干细胞起源Bronchioalveolarstemcellsorigin,146,Adenocarcinoma,147,Adenocarcinoma,148,Adenocarcinoma,149,细支气管肺泡癌(BAC)Bronchioloalveolarcarcinoma,Adistinctsubtypeofadenocarcinoma弥漫型多见multiplediffusenodulesGrowingalongpreexistingstructuresandpreservationofalveolararchitecture分为粘液型和无粘液型mucinous/nonmucinoussubtypes,150,BAC,151,BAC,152,BAC,153,PrecursorlesionsofADC,AAH,BAC,154,低分化癌,排除SCLC,SCCorADCCellsarenot-columnarinshapeDonotcontainmucousDonotshowsquamousdifferentiationDonothaveneuroendocrinepropertiesorsmallcellcharacteristics可发生与任何部位ariseanywhereinlungs大部分与吸烟有关,预后差smoking-related,curesarerare,大细胞癌,155,Largecellcarcinoma,156,肺癌的临床表现,原发灶引起的症状Symptomsduetoprimarytumor原发灶蔓延引起的症状Symptomsduetolocoregionalspread转移引起的问题Metastaticdisease副肿瘤综合征Paraneoplasticsyndromes,157,中央型肺癌Centraltumorscough,dyspnea,肺不张atelectasis,阻塞后肺炎,喘息咯血hemoptysis周围型肺癌Peripheraltumorscough,dyspnea胸水pleuraleffusion疼痛severepain,Duetoprimarytumor,158,Duetolocoregionalspread,上腔静脉综合征Superiorvenacavaobstruction声音嘶哑Hoarseness肩膀和上臂疼痛Hornerssyndrome吞咽困难Dysphagia心包积液Pericardialeffusion,159,160,眼球内陷asunkeneyeball(enophthalmia)瞳孔缩小,对光反射异常上睑下垂droopyuppereyelid患侧面部无汗,HornersSyndrome,Normal,Abnormal,161,Metastaticdisease,脑brain(mentalorneurologicchanges)肝liver(hepatomegaly)骨bones(pain),162,Paraneoplasticsyndromes,HypercalcemiaCushingsyndromeHyponatremiaNeuromuscularsyndromesHematologicmanifestationsClubbingofthefingersBlindnessanddementia,3%to10%ofallpatients,163,Metastaticcarcinoma,164,dilatedlymphaticchannel,165,TransesophagealEchocardiogram,6/22,8daysbeforedeath,showedthickeningoftheleafletsofthePVandone

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