Lung
Terms
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- What is pulmonary hypoplasia?
- defective development resulting in small lungs
- foregut cysts
- abnormal detachment of primitive gut
- congenital pulmonary airway malformation
- hamartomaotous lesion type I-good prognosis
- Pulmonary sequestration
- lung tissue without normal connection to airway syste, with a systemic vascular supply
- resorption atelectasis
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complete airway obstruction
reversible - compressive atelectasis
- pleural space is expanded by fluid or by air i.e. pneumothorax
- contraction atelectasis
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local or generalized fibrotic changes
not reversible - pulmonary edema
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changes in microvascular permeability cause pulmonary edema
caused by increased hydrostatic pressure, decreased oncotic pressure, alveolar injury or idiopathic - Acute Respiratory Distress Syndrome
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diffuse alveolar wall damage
initially involves capillary endothelium but eventually epithelium - pathogenesis of ARDS
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Diffuse alveolar damage (DAD)
1-activated neutrophils aggregate in pulmonary vasculature
2-activated pulmonary macrophages release ROS
3-surfactant is lost or damaged - Obstructive Pulmonary Diseases
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Emphysema
Chronic Bronchitis
Asthma
Bronchiectasis - What is the main morphological feature of emphysema?
- abnormal permanent enlargement of airspaces distal to the terminal bronchioles iwth alveolar wall destruction and minimal fibrosis
- What are the types of Emphysema
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centriacinar-central or proximal parts of the respiratory unit
panacinar-uniform
distal acinar-distal acinus
irregular emphysema-irregular acinar involvement and is associated with scarring - centriacinar emphysema
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central or proximal parts
upper lobes and apices
male smokers - panacinar emphysema
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uniform destruction
lower basal zones
alpha1AT deficinecy - distal acinar
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distal acinus
near pleura and adjacent to fibrosis or scars
underlying lesion in spontaneous pneumothorax - irregular emphysema
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irregular emphysema
associated with scarring
bullous emphysema and interstitial emphysema are related - Pathogenesis of emphysema
- protease-antiprotease theory
- what ist he protease-antiprotease hypotheses
-
destruction of alveolar wall from imbalances between pulmonary proteases and their inhibitors
inactivation of alpha1antitrypsin - Chronic bronchitis
- persistent cough with sputum production for at least 3 months in at least 2 consecutive years
- What is the pathogenesis of chronic bronchitis
-
chronic irritation causes mucus hypersecretion iwth mucus gland hypertrophy
goblet cell metaplasia
bronchiolitis - Asthma
- paroxysmal reversible bronchospasm of tracheobronchial airways due to smooth muscle hyper-reactivity
- what is atopic asthma
-
most common type
family history of atopy
overproduction of Th2 cells with IgE and eosinophil dominated responses - What is nonatopic astham
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often triggered by respiratory tract infections, chemical irritants, or drugs
NO IgE involvement - what is the acute phase of atopic asthma?
- IgE coated Mast cells cause leukotriene and cytokine mediator release
- What is the late phase reaction of atopic asthma
-
recruited leukocytes
persistent bronchospasm and edema-->epithelial damage and loss - What is the morphology of asthma?
- lungs are overinflated with patchy atelectasis and irway occlusion by mucus plugs
- What is bronchiectasis?
- chronic necrotizing infection of bronchi and bronchioles leading to abnormal PERMANENT dilation of airways
- What is associated with bronchiectasis?
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congenital or hereditary conditions
postinfectious conditions
bronchial obstruction
other conditions - What is the morphology of bronchiectasis?
-
lower lobe airways
dilations have different shapes - What is the pathogenesis of diffuse interstitial diseases?
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epithelial or endotheelial injury by inhaled or bloodborne toxins
early acute changes of alveolitis
late stage progresses to interstitial fibrosis - what is idippathic pulmonary fibrosis?
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unknown cause with progressive pulmonary interstitial fibrosis resulting in hypoxemia
repeated cycels of alveolitis causes abnormal wound healing - What is usual interstitial pneumonia (UIP)?
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patchy interstitial fibrosis
lower lobe predominance
fibroblastic foci within bronchiolar walls
ongoing destruction leads to honeycomb lune with dense fibrosis - What is cryptogenic organinzing pneumonia
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cough, dyspnea, and often recent RI-gradual or steroid
loose fibrous plugs -
What are pneumoconioses?
What determines the outcome? -
caused by aerosols
1-amount of dust retained
2-size, shape, and bouyancy of particles
3-physiochemical reactivity and solubility of the particles - What is coal workers' pneumoconiosis?
- carbon dust caused
- What are the different effects that are part of the coal workers' pneumoconiosis?
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anthracosis
simple coal worker's pneumoconiosis
progressive massive fibrosis - What is antrhacosis
- small harmless accumulations in the lungs of urban dwellers and smokers
- what is simple coal workers' pneumoconiosis?
-
numerous aggregates of caol dust laden macrophages forming coal macules
No significant dysfunction - What is progressive massive fibrosis?
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manifested by severe fibrosis and scarring in areas of dust accumulation
results in respiratory insufficiency
BLACK LUNG DISEASE - What is silicosis?
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prolonged inhalation of silica particles
produces chronic nodular dense pulmonary fibrosis - What is the pathogenesis of silicosis?
- macrophage ingestion of silica leads to activation-->release of oxidants, cytokines, and growth factors-->fibrosis and collagen deposition
- What is the morphology of silicosis?
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collagenous nodules start as small lesions in upper lung becoming larger and more diffuse
birefringent silica particles with polarized light - What causes serpentines and amphiboles?
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asbestos
serpentines-flexible chrysotile
amphiboles-brittle straight crocidolite
lead to asbestos bodies - What is the pathogenesis of asbestos related diseases?
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amphiboles reach deep lung more than serpentine fibers
fibers inhaled reach alveoli ingested by alveolar macrophages release C5a most cleared
some coated by hemosiderin and glycoproteins form dumbbell shaped asbestos bodies - What is sarcoidosis
-
releative common disease
unknown etiology
noncaseating granulomas in any tissue
f>m, blacks 10x whites - How do you diagnose sarcoidosis?
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ONLY BY BIOPSY
may be an incidental discovery with autopsy-bilateral hilar adeonpathy on xray
often diagnosis of exclusion - What is the pathogenesis of sarcoidosis?
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genetic factors
numerous activated CD4 with increased Th1 cytokine production
cutaneous anergy
polyclonal hypergammaglobinemia
activated t cells
elevated gamma delta t cells - What is the morphology of sarcoidosis?
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organomegaly
multinucleated giant cells
rare necrosis
shaumann bodies (laminated calcified proteinaceous concretions)
asteroid bodies (stellate inclusions in giant cells - What are the clinical features of sarcoidosis?
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slowly progressive
remitting and resloving course
can spntaneously resolve - What are hypersensitivity penumonitis diseases?
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farmer's lung-thermophilic actinomycetes in hay
pegeon breeder's lung-proteins from birds or excreta
humidifier or ac lung-thermophilic bacteria - How do you prevent progression of hypersensitivity pneumonitis?
- early cessation of exposure to the agent
- What is pulmonary eosinophilia?
- diverse clinicopathologic conditions characterized by eosinophil infiltrates in pulmonary interstitial or alveolar spaces
- What is desquamative interstitial pneumonia?
-
smoking related interstitial disease
dusty brown smoker's macrophages
steroid therapy of stop smoking - What are the three forms of pulmonary alveolar proteinosis?
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acquired PAP-90% autoimmune GM-CSF
congenital PAP-newborns, rapidly fatal
secondary PAP-epxosure to irratint, immunosuppressed - How is PAP characterized clinically, radiologically, and histologically?
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C-respiratory difficulty, cough, and sputum with gelatinous material
X-diffuse pulmonary opacification
H-dense amorphous PAS+ lipid laden material in intra-alveolar spaces - What are in situ thromboses?
- rare but with DAD, pulmonary hypertension, and pulmonary atherosclerosis
- What is pulmonary hypertension?
-
elevated pulmonary artery pressure caused by increased pulmonary vascular resistance
usually 2 to chronic obstructive or interstitial lung disease
L heart failure
recurrent PE
autoimmune disorders - What is the pathogenesis of pulmonary hypertension?
- endothelial dysfunction and injury->persistnet vasoconstriction with intimal and medial phyerptrophy and increased resistance
- what are plexogenic arteriopathies?
- tufts within capillary channels creating a vascular plexus
- What are the pulmonary hemorrhage syndromes?
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goodpasture syndrome
idiopathic pulmonary hemosiderosis
vasculitis associated hemorrhage - What is Goodpasture syndrome?
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necrotizing hemorrhagic interstitial pneumonitis ith progressive glomerulonephritis
circulating antibodies vs basement membrane antigens in lungs and kidneys - what is idiopathic pulmonary hemosiderosis
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rare, children
intermittent diffuse alveolar hemorrhage - What are the pulmonary defenses
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nasal, tracheobronchial, and alveolar mechamisms
filter, neutralize, and clear inhaled organisms and particles - What causes Community Acquired Pneumonia?
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streptococcus pneumonia-most common
haemophilus influenzae
moraxella catarrhalis
staph aureus
klebsiella pneumoniae-most common gram- cause
pseudomonas aeruginosa
legionella pneumophilia - What is the morphology of bronchopneumona?
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patchy exudative consolidation of the lung parenchyma, suppurative exudation filling airways and airspaces
restores normal lung structure, but may have scarring - What is the morphology of lobar pneumonia?
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involves large portion of an entire lobe
cause by pneumococci - what are the stages of CAP
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congestion-1st 24 hours
red hepatization=consolidation
gray hepatization
resolution-normal structure restored - What causes atypical pneumonias
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influenca A or B
repsiratory synctial virus
adenovirus
rhinovirus
herpes simplex
cytomegalovirus
mycoplasma pneumoniae - what is the morphology of atypical pneumonias?
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patchy congestion without consolidation
hyaline membranes
frequently superimposed bacterial infection - Which type of influenza mutate?
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type A
B and C don't - what is a lung abscess?
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infection marked by localized suppurative necrosis of lung tissue
staph, strep, gram-, and anaerobes - what characterizes chronic pneumonia?
- localized granulomatous inflammation in immunocompetent patients with or without lymph involvement
- What is histoplasmosis?
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inhalation
endemic along ohio and ms rivers
granulomas with coagulative necrosis
SILVER STAIN - What s blastomycosis?
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central, SE US, mexico, Canada, Middle East, africa, etc
Blastomyces dermatidis-yeast
suppurative granulomas - What is coccidioidomycosis?
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coccidioides immitis
SW and W US, Mexico
SILVER STAIN
endospores - When is lung transplantation performed?
- otherwise healthy for emphysema, idiopathic pulmonary fibrosis, cystic fibrosis, and primary pulmonary hypertension
- What is bronchiolitis obliterans?
- chronic rejection of lung transplant
- What is the most common type of lung tumor?
- carcinoma
- What is the pathogenesis of lung tumors?
- most important is tobacco smoke
- What are the three types of precursor lesions in lung tumors?
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squamous dysplasia and carcinoma in situ
atypical adenomatous hyperplasia
diffuse idiopathic pulmonary neuroendocrine cell hyperplasia - adenocarcinoma
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most common lung cancer
peripheral mass
produces mucin - bronchioloalveolar carcinoma
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uncommon
terminal bronchioloalveolar regions
often mucin producing cells along preserved alveolar septa
not associated with smoking - squamous cell carcinoma
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closest correlation with smoking
in or near lung hilus - small cell carcinoma
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most malignant of lung cancers
central of hilar tumor
oatlike cells with little cytoplasm without squamous or glandular differentiation
often produce paraneoplastic syndromes - large cell carcinoma
- poorly differentiated squamous cell carcinomas or adenocarcinomas
- Paraneoplastic syndromes associated with lung carcinoma release:
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antidiuretic homrone
corticotropin
parathormone or prostaglandin
calcitonin
gonadotropins
serotonin - metastatic tumors
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very common
direct extension, lymphatic, or hematogenic spread - what conditions cause a transudative pleural effusion
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heart failure
nephrotic syndrome
cirrhosis - what conditions cause and exudative pleural effusion
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penumonias
atelectasis
carcinomatosis - what is empyema?
- pleural space infection leading to pus accumulation
- what causes hemorrhagic pleuritis
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bleeding disorders
neoplasms
rickettsial diseases - what causes pleural tumors
- most commonly metastases from lung, breast, ovaries, etc.
- what is a malignant mesothelioma
- uncommon tumor of mesothelial cells-most common in pleura
- what are the clinical features of malignant mesothelioma?
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chest px, dyspnea, recurrent pelural effusion
survival <2 yrs