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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
complete airway obstruction
compressive atelectasis
pleural space is expanded by fluid or by air i.e. pneumothorax
contraction atelectasis
local or generalized fibrotic changes
not reversible
pulmonary edema
changes in microvascular permeability cause pulmonary edema
caused by increased hydrostatic pressure, decreased oncotic pressure, alveolar injury or idiopathic
Acute Respiratory Distress Syndrome
diffuse alveolar wall damage
initially involves capillary endothelium but eventually epithelium
pathogenesis of ARDS
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
Chronic Bronchitis
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
centriacinar-central or proximal parts of the respiratory unit
distal acinar-distal acinus
irregular emphysema-irregular acinar involvement and is associated with scarring
centriacinar emphysema
central or proximal parts
upper lobes and apices
male smokers
panacinar emphysema
uniform destruction
lower basal zones
alpha1AT deficinecy
distal acinar
distal acinus
near pleura and adjacent to fibrosis or scars
underlying lesion in spontaneous pneumothorax
irregular emphysema
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
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
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?
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?
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?
unknown cause with progressive pulmonary interstitial fibrosis resulting in hypoxemia
repeated cycels of alveolitis causes abnormal wound healing
What is usual interstitial pneumonia (UIP)?
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
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?
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?
manifested by severe fibrosis and scarring in areas of dust accumulation
results in respiratory insufficiency
What is silicosis?
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?
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?
serpentines-flexible chrysotile
amphiboles-brittle straight crocidolite
lead to asbestos bodies
What is the pathogenesis of asbestos related diseases?
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?
may be an incidental discovery with autopsy-bilateral hilar adeonpathy on xray
often diagnosis of exclusion
What is the pathogenesis of sarcoidosis?
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?
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?
slowly progressive
remitting and resloving course
can spntaneously resolve
What are hypersensitivity penumonitis diseases?
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?
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?
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?
goodpasture syndrome
idiopathic pulmonary hemosiderosis
vasculitis associated hemorrhage
What is Goodpasture syndrome?
necrotizing hemorrhagic interstitial pneumonitis ith progressive glomerulonephritis
circulating antibodies vs basement membrane antigens in lungs and kidneys
what is idiopathic pulmonary hemosiderosis
rare, children
intermittent diffuse alveolar hemorrhage
What are the pulmonary defenses
nasal, tracheobronchial, and alveolar mechamisms
filter, neutralize, and clear inhaled organisms and particles
What causes Community Acquired Pneumonia?
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?
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?
involves large portion of an entire lobe
cause by pneumococci
what are the stages of CAP
congestion-1st 24 hours
red hepatization=consolidation
gray hepatization
resolution-normal structure restored
What causes atypical pneumonias
influenca A or B
repsiratory synctial virus
herpes simplex
mycoplasma pneumoniae
what is the morphology of atypical pneumonias?
patchy congestion without consolidation
hyaline membranes
frequently superimposed bacterial infection
Which type of influenza mutate?
type A
B and C don't
what is a lung abscess?
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?
endemic along ohio and ms rivers
granulomas with coagulative necrosis
What s blastomycosis?
central, SE US, mexico, Canada, Middle East, africa, etc
Blastomyces dermatidis-yeast
suppurative granulomas
What is coccidioidomycosis?
coccidioides immitis
SW and W US, Mexico
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?
What is the pathogenesis of lung tumors?
most important is tobacco smoke
What are the three types of precursor lesions in lung tumors?
squamous dysplasia and carcinoma in situ
atypical adenomatous hyperplasia
diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
most common lung cancer
peripheral mass
produces mucin
bronchioloalveolar carcinoma
terminal bronchioloalveolar regions
often mucin producing cells along preserved alveolar septa
not associated with smoking
squamous cell carcinoma
closest correlation with smoking
in or near lung hilus
small cell carcinoma
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:
antidiuretic homrone
parathormone or prostaglandin
metastatic tumors
very common
direct extension, lymphatic, or hematogenic spread
what conditions cause a transudative pleural effusion
heart failure
nephrotic syndrome
what conditions cause and exudative pleural effusion
what is empyema?
pleural space infection leading to pus accumulation
what causes hemorrhagic pleuritis
bleeding disorders
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?
chest px, dyspnea, recurrent pelural effusion
survival <2 yrs

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