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呼吸系统疾病 The Respiratory System,第四章,快速气体交换 Rapid gas exchange Ventilation Perfusion Diffusion 清洁空气,肺的结构与功能,Mucosa,Submucosa,Cartilage,Muscles,Bronchus,Bronchial epithelium,Kulchitsky cells,Bronchial Submucosal Glands,Alveoli,The respiratory membrane,病原体 Pathogen 空气 contaminated air exposure 鼻咽部菌群 nasopharyngeal flora aspiration 宿主防御功能下降 Compromised defense mechanisms 肺部疾病 other common lung disease 免疫缺陷 Immunodeficiency 生活方式 Life style,肺部感染 Pulmonary Infections,分类 Classification,根据解剖和影像学 Anatomy and radiography 根据病因 Etiology 根据在哪获得 Setting in which they arise Community-Acquired Hospital-acquired (nosocomial ),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),小叶性肺炎,大叶性肺炎,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,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。,大叶性肺炎,大叶性肺炎,大叶性肺炎,In the era before antibiotics,充血水肿期 Hyperemia and edema 红肝期 Red hepatization 灰肝期 Gray hepatization 溶解消散期 Resolution (hopefully),Hyperemia and edema,12-24 hours,Red hepatization 2-3 days,Gray hepatization,3-4 days,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,小叶性肺炎,小叶性肺炎,Filled with exudate,Aerated lung,小叶性肺炎,小叶性肺炎,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,Organization,Abscess formation,Abscess formation,Purulent pleuritis,Hemorrhagic Pneumonia by Pseudomonas aeruginosa,Pseudomonas pneumonia,Klebsiella pneumonia,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,Viral pneumonia,Atypical pneumonia,Severe Acute Respiratory Syndrome (SARS),Pneumonia in the Immunocompromised Host,机会致病原 opportunistic agents 常为多重感染 bacteria (P. aeruginosa, Mycobacterium spp., L. pneumophila, and Listeria monocytogenes) viruses (cytomegalovirus and herpesvirus) fungi (P. jiroveci, Candida spp., Aspergillus spp., and Cryptococcus neoformans),CMV infections,in situ hybridization,Sputum cytology from an AIDS patient,(Papanicolaou),(Giemsa),Pneumocystis Pneumonia,(silver stain),Pneumocystis Pneumonia,(silver stain),Pneumocystis Pneumonia,Pneumocystis carinii in bronchial lavage from an AIDS patient (Grocotts silver),(Papanicolaou),(Immunostaining),慢性阻塞性肺病 (COPD) Chronic Obstructive Pulmonary Disease,limitation of airflow usually resulting from an increase in resistance caused by partial or complete obstruction at any level 肺气肿 Emphysema 慢性支气管炎 Chronic bronchitis 支气管扩张 Bronchiectasis 哮喘 Asthma,FEV1 / FVC,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。,慢性支气管炎 Chronic bronchitis,A persistent cough productive of sputum for at least 3 months, in at least 2 consecutive years 单纯型 Simple 喘息型 Asthmatic 阻塞型 obstructive,病 因 etiology,吸烟和空气污染 Cigarette smoking and air pollutants 感染 Microbial infection Secondary role 使炎症持续,加重症状 maintaining the inflammation and exacerbating symptoms,发病机制 Pathogenesis,多痰粘液分泌亢进 Hypersecretion of mucus 大气道病变 large bronchial involvement 粘液腺增生,杯状细胞分泌亢进 Hypertrophy of mucous glands and a marked increase in mucin- secreting goblet cells,气道阻塞 Airflow obstruction 小气道炎症和管壁纤维化 inflammation, bronchiolar wall fibrosis 杯状细胞化生,细支气管痰栓形成 goblet cell metaplasia with mucus plugging of the bronchiolar lumen 并发肺气肿 coexistent emphysema,发病机制 Pathogenesis,inner perichondrium,basal lamina,Chronic bronchitis,Normal bronchus,Chronic bronchitis,Chronic bronchitis,Chronic bronchiolitis,luminal and mucus,chronic inflammation,肺气肿 Emphysema,Abnormal permanent enlargement of the airspaces distal to the terminal bronchioles, accompanied by destruction of their walls without obvious fibrosis 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,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 Distal acinar 不规则型 Irregular,obstruction,Spontaneous pneumothorax,asymptomatic,发病机理 Pathogenesis,Centriacinar emphysema,Bullous emphysema,Centriacinar emphysema,Inadequate ventilation Less perfusion Narrowed bronchiole,Conditions Related to Emphysema,间质性肺气肿 Mediastinal (interstitial) emphysema 代偿性肺气肿 Compensatory emphysema 阻塞性过充气 Obstructive overinflation 瘢痕旁肺气肿 Paracicatrical emphysema 老年性肺气肿 Senile emphysema,COPD的临床表现,Respiratory failure,Right heart failure,Pink puffer,桶状胸 Barrel chest,支气管扩张症 Bronchiectasis,Permanent dilation of bronchi and bronchioles caused by destruction of the muscle and elastic supporting tissue resulting from or associated with chronic necrotizing infections 继发于持续性感染或阻塞 Secondary to persisting infection or obstruction 诊断依靠病史和影像学,Predisposing conditions,支气管阻塞 Bronchial obstruction 肿瘤 Tumor 异物 Foreign bodies 先天性或遗传性疾病 Congenital or hereditary conditions Cystic fibrosis Kartagener syndrome 坏死性化脓性肺炎 Necrotizing, or suppurative, pneumonia,Cystic Fibrosis (CF),Cystic Fibrosis (CF),Bronchiectasis,57 year old woman with a long history of recurrent respiratory tract infections and episodes of hemoptysis,Bronchiectasis,Bronchiectasis Due to pneumonia,Bronchiectasis,Bronchiectasis,Purulent exudation in the lumen Destruction of lining epithelium Scarring of the bronchial wall,HRCT scans of bronchiectasis,支气管扩张症的临床表现,咳嗽,咳脓臭痰 Severe, persistent cough with mucopurulent sputum, sometimes fetid 咯血 Flecks of blood in sputum or frank hemoptysis 严重病例及合并症,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,Selected causes of chronic interstitial lung disease Occupational and environmental exposure Asbestosis Silicosis Hypersensitivity pneumonitis Drug or treatment related Chemotherapeutic agents Ionizing irradiation Oxygen Immunologic lung disease Sarcoidosis Wegner granulomatosis Collagen vascular disease Goodpasture syndrome Miscellaneous Post acute respiratory distress syndrome Idiopathic pulmonary fibrosis,杵状指(趾) Clubbing of digits,Honeycomb lung,矽 肺 Silicosis,职业病 Occupational diseases 吸入二氧化硅粉尘引起 Inhalation of crystalline silica 致残和致死 Disabling and fatal 病理:硅结节形成 广泛的肺纤维化,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 Alveolar macrophage is a key cellular element in the initiation and perpetuation of lung injury and fibrosis,Silicosis,a slice of lung from a 61 yo ceramics worker,矽结节 Silicosis,临床表现 Clinical course,早期常为体检发现 usually detected in routine chest radiographs 晚期表现: 呼吸困难 shortness of breath 肺心病 cor pulmonale 合并TB increased susceptibility to TB 可能致癌 carcinogenic (controversial),Silicosis,急性呼吸窘迫征 (ARDS) Acute Respiratory Distress Syndrome,进行性呼吸衰竭 Acute onset of Dyspnea Hypoxemia Bilateral pulmonary infiltrates (X-ray) No evidence of left-sided heart failure 病理:弥漫性肺泡损伤 Diffuse Alveolar Damage (DAD) 常伴多器官衰竭 Multiple Organ Dysfunction Syndrome (MODS),发病机制 Pathogenesis,广泛内皮和肺泡I型、II型上皮损伤 Endothelial and epithelial (I and II) damage 肺水肿 alveolar flooding 气体交换丧失 loss of diffusion capacity 表面活性物质异常 surfactant abnormalities 促炎和抗炎介质的失衡 Imbalance of pro-inflammatory and anti-inflammatory mediators,病理变化 Morphology,急性渗出期 Acute Exudative Stage 水肿 Edema (interstitial and alveolar) 透明膜形成 Hyaline membranes 肺泡塌陷 Many alveoli collapse 增生期 Proliferative Stage II型肺泡上皮增生 肺泡内机化 Intra-alveolar fibrosis,widening of the interstitium ARDS,Hyaline membranes,Hyaline membranes,Hyaline membranes,Healing stage,Healing stage,suviving patient ARDS,临床表现 Clinical features,肺顺应性差 Poor pulmonary compliance 难治性低氧血症 Poor response to oxygen 血管床进行性减少 Pulmonary vascular bed is progressively obliterated 易感染 Prone to bacteria infection,预后 Prognosis,Depends successfully treatements before extensive fibrosis 40-70% mortality High-dose steroids failed Conservative therapy helps Considerable interest in the quality of survivals,LUNG TUMORS,Metastatic tumors Primary tumors BRONCHOGENIC CARCINOMA Others bronchial carcinoids mesenchymal malignancies Lymphomas a few benign lesions,95%,5%,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。,肺 癌,癌症死因第一位 cause of cancer-related deaths 发病率随年龄和烟龄增长 “pack-years smoked“. 确诊的病人一半以上已有远处转移 distant metastatic disease 5年生存率约为15% 5-year survival,肺癌的临床病理分型,肺小细胞癌 Small cell lung cancer (SCLC) 肺非小细胞癌 Non-small-cell lung cancer (NSCLC) 鳞癌 Squamous cell carcinoma 腺癌 Adenocarcinomas 大细胞癌 Large cellcarcinomas,SCLC,Kulchitsky cell起源 hADH (hyponatremia/ water intoxication) ACTH (Cushings syndrome) 中央型 Centrally located masses 坏死常见 Necrosis is invariably present and may be extensive 燕麦细胞癌 Oat cell carcinoma,SCLC,Oat cell carcinoma,SCLC,Oat cell carcinoma,SCLC,鳞状细胞癌,病人大多有吸烟史 Closely correlated with a smoking history 中央型 Tend to arise centrally in major bronchi 常见空洞 Cavitation is not uncommon 副癌综合征 Preneoplastic lesions,Squamous cell carcinoma,Squamous cell carcinoma,Squamous cell carcinoma,Squamous cell carcinoma,Squamous cell carcinoma,Metaplaisa? Anaplasia? Dysplasia?,腺 癌 Adenocarcinoma,周围型多见 peripherally located 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 arising in relation to peripheral lung scars 生长缓慢但早期转移 支气管肺泡干细胞起源 Bronchioalveolar stem cells origin,Adenocarcinoma,Adenocarcinoma,Adenocarcinoma,细支气管肺泡癌(BAC) Bronchioloalveolar carcinoma,A distinct subtype of adenocarcinoma 弥漫型多见multiple diffuse nodules Growing along preexisting structures and preservation of alveolar architecture 分为粘液型和无粘液型 mucinous/nonmucinous subtypes,BAC,BAC,BAC,Precursor lesions of ADC,AAH,BAC,低分化癌,排除SCLC, SCC or ADC Cells are not-columnar in shape Do not contain mucous Do not show squamous differentiation Do not have neuroendocrine properties or small cell characteristics 可发生与任何部位 arise anywhere in lungs 大部分与吸烟有关,预后差 smoking-related, cures are rare,大细胞癌,Large cell carcinoma,肺癌的临床表现,原发灶引起的症状 Symptoms due to primary tumor 原发灶蔓延引起的症状 Symptoms due to locoregional spread 转移引起的问题 Metastatic disease 副肿瘤综合征 Paraneoplastic syndromes,中央型肺癌 Central tumors cough, dyspnea, 肺不张atelectasis, 阻塞后肺炎,喘息 咯血hemoptysis 周围型肺癌 Peripheral tumors cough, dyspnea 胸水pleural effusion 疼痛severe pain,Due to primary tumor,Due to locoregional spread,上腔静脉综合征 Superior vena cava obstruction 声音嘶哑 Hoarseness 肩膀和上臂疼痛 Horners syndrome 吞咽困难 Dysphagia 心包积液 Pericardial effusion,眼球内陷 a sunken eyeball (enophthalmia) 瞳孔缩小,对光反射异常 上睑下垂 droopy upper eyelid 患侧面部无汗,Horners Syndrome,Normal,Abnormal,Metastatic disease,脑 brain (mental or neurologic changes) 肝 liver (hepatomegaly) 骨 bones (pain),Paraneoplastic syndromes,Hypercalcemia Cushing syndrome Hyponatremia Neuromuscular syndromes Hematologic manifestations Clubbing of the fingers Blindness and dementia,3% to 10% of all patients,Metastatic carcinoma,dilated lymphatic channel,Transesophageal Echocardiogram,6/22, 8 days before death, showed thickening of the leaflets of the PV and one of two pedunculated vegetations, There was no valvar insuff

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