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Respiratory System

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Congenital anomalies of the respiratory system   agenesis, hypoplasia, cong pulmonary airway malform, tracheal/bronchial atresia/stenosis/fistula, vascular anomalies, foregut cysts, congen lobar overinflation, pulm sequestrations  
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Lung Hypoplasia   defective development of both lungs; dec wt/vol/acini; 10% of neonatal autopsies; mc d/t space occupying lesions in uterus, oligo or impaired fetal respiratory movements  
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Congenital pulmonary airway malformation (CPAM)   hamartomatous lesion; 1/5000 births; 5 types rated by cyst size; Type 1: mc w/large cysts & good Px; Type 2: medium cysts w/poor Px; (types 3-5 are very rare)  
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Pulmonary sequestration   lung tissue w/o connection to airway; blood supplied from aorta (not pulm aa); can be either extralobar or intralobar  
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Extralobar lung sequestration   external to lung; may be a/w other congenital anomalies; more common in male babies  
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Intralobar lung sequestration   in the lung substance; a/w infection and bronchiectasis  
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Atelectasis   **a REVERSIBLE condition**; incomplete expansion or collapse of previously infated lung; 3 Types: (Obstructive/resorption, Compression, Contraction)  
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Obstructive/Reabsorption Atelectasis   d/t resorption of air distal to a complete obstruction by foreign body, mucous plug, COPD, post-op; **Trachea shifts TOWARDS AFFECTED LUNG;** No blood flow problems  
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Compression Atelectasis   d/t fluid, air, blood or tumor in pleural space (hemo/pneumothoraces); **shift of trachea AWAY from AFFECTED LUNG b/c there is filling of space**  
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Contraction Atelectasis   d/t generalized fibrosis and scarring of lung; probably shifts towards affected lung  
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Pulmonary Edema   Hemodynamic Edema (vol overload, hypoalbuminemia, nephrotic syndrome, liver dx), Microvascular Injury (infxn, gases, aspiration/near drowning, shock, radiation, drugs/chemicals), Unknown Origin (altitude, CNS trauma); Tx directed at cause (ex CHF d/t LHF)  
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Acute Respiratory Distress Syndrome (ARDS)   aka: diffuse alveolar damage, shock lung, acute alveolar/lung injury; diffuse damage of alveolar epithelium/capillaries w/hyaline membranes (edema+debris), progressive respiratory failure, cyanosis, arterial hypoxemia that is unresponsive to O2 therapy  
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Conditions a/w ARDS   infection, chemical injury (heroin, meth OD, asprin), physical injury, hematologic conditions (disseminated intravascular coagulation), inhaled irritants, pancreatitis, uremia, cardiopulmonary bypass, hypersensitivity rxns  
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Hyaline membrane formation in ARDS   activated PMNs (neutrophils) release cytokines that induce local tissue damage; interstitial and intra-alveolar edema and cellular debris form membrane; interstitial inflammation, loss of type I pneumocytes  
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Obstructive Pulmonary Diseases   emphysema, chronic bronchitis, asthma, bronchiectasis  
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Obstructive lung disease   1. tumors/foreign bodies; 2. Lung diseases; characterized by inc resistance to air flow d/t partial or complete obstruction at any level; on PFT the TLC is nml to increased w/dec expiratory flow rate;  
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Chronic Obstructive Pulmonary Disease (COPD)   persistant and irreversible air flow obstruction including: Chronic Bronchitis and Emphysema  
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Emphysema   destruction of alveolar septa resulting in enlarged air spaces and loss of elastic recoil distal to terminal bronchioles; proteases (incl elastase) from PMNs/MQs stimulated by smoke/pollution; antiproteases incl: a-1-antitrypsin; 95% is CENTRILOBAR  
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Centrilobar/centriacinar Emphysema   involves blebs/bullae in central (proximal) parts of acini (respiratory bronchioles) in upper/APICAL lobes; black pigment around emphysematous space; d/t smoking & pollution; mc in males; resembles 'Coal Worker's Pneumoconiosis'  
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Panacinar (panlobular) emphysema   acini are uniformly enlarged; worse in LOWER lobes; d/t alpha-1-antitrypsin deficiency  
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Distal acinar (paraseptal) emphysema   distal acinus involved (proximal is nml); adjacent to pleura in paraseptal CT at lobular margins next to fibrosis/scars/atelectasis; usu limited to UPPER HALF of lung; can form bullae and spontaneous pneumos in young adults  
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Bullous Emphysema   any emphysema with blebs or bullae (usu sub-pleural) prone to rupture causing spontaneous pneumothorax; spaces >1cm diameter; typical presentation in kid is 16-18 skinny complaining of problems breathing  
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Alpha-1-antitrypsin deficiency (AAT)   nml enzyme present in serum/MQs (inhibits proteases/elastases secreted by PMNs); chrom14 PiZZ = marked dec in 0.12% of pop; inc tendency for emphysema, elastic tissue destruction b/c lack of protective enzyme; double wammy if they smoke! develop dx early  
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Clinical Course of Emphysema   insidous progression (dyspnea when 1/3 of parenchyma is lost)! "pink puffer" - barrel chested, cachectic, accessory mm, hyperventilation, dyspnea, blood gases OK; Px = death by **pulmonary failure; Complications = less common (pulm HT, cor pulmonale)  
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Chronic Bronchitis   persistent productive cough for >3 consecutive mo in at least 2 consecutive yrs; usu co-exists w/emphysema  
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Chronic Bronchitis Pathophysiology   d/t cigarette smoke or pollutants; sulfur or nitrogen dioxides; hypertrophy & hypersecretion of bronchial mucosal glands; goblet cell metaplasia, thick bronchial squamous wall/inflam/fibrosis, infection  
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Clinical Course of Chronic Bronchitis   presents w/productive cough, may progress to COPD (5-15% of smokers); "blue bloater" - obese, edematous male in CHF, hypoxic and cyanotic w/inc CO2, poor respiratory drive; Cause of death = **cardiac failure (cor pulmonale), recurrent infxn  
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Emphysema vs (Bronchitis)   50-75(40-45); bad early dyspea (mild late); pink puff (blue bloat); late cough (early); no sputum (copious); few infxns (many); terminal resp insuff (repeat); cor pulm rare/term (common); nml air resist (inc); dec elastic (nml); sm heart (lg); flat diaphr  
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Asthma   hyperreactive airways resulting in episodic bronchospasm when triggered by certain stimuli; sudden onset severe dyspnea; wheezing (not all); common (5% of adults, 7-10% kids)  
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Extrinsit Asthma (type I hypersensitivity reaction)   IgE rxn to extrinsic antigen; 3 Types: Atopic (allergic d/t genetics); Occupational; Allergic bronchopulmonary asperigillosis  
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Atopic Asthma   mc; triggered by environ allergens; onset in 1st 2 decades; fam Hx of atopy; a/w allergic rhinitis, urticaria, eczema; IgE elevated; eosinophilia common; type 1 wheal and flare rxn to allergen  
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Intrinsic Asthma   non-immune triggering mechanism (aspirin sensitivity, viral pulmonary infxn, cold, stress, exercise, inhaled irritants); *people w/extrinsic asthma are also susceptible to intrinsic triggers  
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Acute of Immediate Phase of Asthma   30-60min after exposure; Ag-x-links IgE on mast cell & primary mediators released (*histamine: eosinophil/neutrophil chemotactic factors - leukotriene B4); Ag enter mucosa activate mast/eosinophils (2* mediators released: Leutriene C4, D4, E, PG D2, PAF)  
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Late Phase of Asthma   4-8hrs after exposure; mediated by recruited cells (basophils, neutrophils, eosinophils); eosinophils relsease PAF, leukotriene C4, major basic ptn, eosinophil cationic ptn (ECP); *MBP & ECP damage epithelial cells*  
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Asthma histological findings   hyperplasia of mucous glands and goblet cells, hypertrophy of bronchial smooth muscle, edema/inflam w/eosinophils, thickened BM, patchy necrosis of epithelium, intraluminal mucus plugs (w/Curshmann's spirals & Carcot-Levden crystals)  
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Asthma: clinical course   acute attacks may be fatal; status asthmaticus is when a pt doesn't come out of an attack; there is an inc risk of COPD in adults; when a pt wheezes it indicates that air is moving at least (if you don't hear wheezes, pt may be tiring out)  
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Bronchiectasis   permanent dilation of bronchi and bronchiles d/t destruction of muscle & elastic supporting tissue; a/w chronic necrotizing infxns; 2* to many etiologies; occurs at any age, but esp in children  
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Causes of Bronchiectasis   obstruction (tumor, foreign body); Congen/hereditary (CF, immotile cilia, Kartagener's syndrome, immunodef: RA, SLE, IBD); necrotizing/suppurative pneumonia or post-infectious (measles, whooping cough, influenza, TB, staph, pseudomonas); post transplant  
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Bronchiectasis histological findings   acute/chronic inflam in walls of bronchi & bronchioles; desquamation of lining epithelium w/necrotizing ulcers; squamous metaplasia/dysplasia; necrosis/abscess; fibrosis w/chronicity; acute inflam a/w acute exacerbation  
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Restrictive Lung Diseases   fibrosing, granulomatous, pulmonary eosinophilia, smoking-related, pulmonary alveolar proteinosis  
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Restrictive lung disease: characteristics   reduced expansion of lung parenchyma & dec total lung capacity; Pulm Fxn Tests: dec TLC w/nml expiratory flow rate;  
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Two categories of restructive lung diseases   1. Chest wall disorder w/nml lung (polio, obesity, pleural dx); 2. Interstitial Lung Dxs (ARDS, Pneumoconiosis, Sarcoidosis)  
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Interstitial Lung Disease   grp of diseases characterized by diffuse and chronic involvment of the pulmonary connective tissue; the interstitium gets fibrotic  
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Interstitial Lung Disease: Pathogenesis   regardless of type, the earliest manifestation is Alveolitis (inflam w/in alveolar walls & spaces w/ accumulation of alveolar MQs)  
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Fibrosing Lung Diseases   idiopathic pulm fibrosis d/t repeated cycles of alveolitis (wound healing causes fibroblast proliferation); **early & late lesions are present at same time**; subpleural fibrosis is more pronounced; insidious onset w/gradual dyspnea  
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Fibrosing Lung Diseases: Clinical course   insidious onset w/gradual dyspnea; dry cough, 40-70yo; hypoxemia, cyanosis, clubbing occurs late; mean survival is <3yrs; Tx: lung transplant  
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Honeycomb lung   the end stage of any interstitial lung disease; cystic spaces w/thick bands of fibrous tissue  
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Pulmonary involvement in collagen vascular diseases   lung changes seen in: SLE, RA (30-40  
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Pneumoconiosis   non-neoplastic lung rxn to inhalation of mineral dusts, organic and inorganic particulates, chemical fumes and vapor; most commonly a/w coal dust, silica, asbestos and beryllium from workplace exposure  
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Coal Workers' Pneumoconiosis   range of asymptomatic anthracosis (pigment change w/o cellular rxn) to simple CWP w/MQ accumulation & lettle destruction; to complicated PWC or progressive massive fibrosis (PMF) w/extensive fibrosis & compromised lung fxn  
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Caplan Syndrome   coexistance of RA with a pneumoconiosis; develops a distinct nodular pulmonary lesion w/central necrosis surrounded by palisading fibroblasts, MQs and plasma cells  
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Silicosis   a/w quarry mining, sandblasting, drilling, tunneling, stone cutting; actue, chronic/nodular, complicated/conglomerate forms  
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Asbestosis   linked to parenchymal interstitial fibrosis, bronchogenic carcinoma, pleural effusions, local fibrous plaques or diffuse pleural fibrosis, malignant pleural & pertoneal mesotheliomask, & laryngeal carcinoma; family members of exposed workers also at risk  
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Sarcoidosis   multisystem disorder w/unknown etiology; **formation of non-caseating granulomas**; <40yo; blacks>whites; F>>M; (classicly a young black female)  
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Sites affected by sarcoidosis   lungs (diffuse interstitial alveolitis, permanent respiratory dysfxn in 25% pts); Bilateral hilar adenopathy (potato nodes); skin and/or eye symptoms too  
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Sarcoidosis lab findings   not diagnostic; elevated ACE (produced by granulomas); Hypercalcemia (d/t inc Ca absorption 2* to activated Vit D by MQ in granulomas); Hyperagammaglobuilinemia  
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Sarcoidosis immune findings   cutaneous anergy to common Ag (candida); Dec peripheral T lymph w/absolute lymphopenia; CD4/CD8 ratio is reduced; Lungs (inc activated CD4 cells; CD4:CD8 ratio is 10:1 in lung); Polyclonal hypergammopathy  
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Sarcoidosis Histological Findings   non-caseating granulomas (exclude mycobacteria, fungi & berylliosis), tight clusters of epithelioid cells, multinucleated giant cells, rim of CD4 cells, Schaumann (chonchoid bodies - basophilic concretions of Ca/Fe/ptn); Asteroid bodies (in giant cells)  
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Manifestations of Extrapulmonary Sarcoidosis   skin lesions (nodules, plaques, red macules); Eyes (blindness, iritis, iridocyclitis, uveitis); Mikulicz's Syndrome (salivary glands + uveitis); Spleen & liver (in 75% of cases); Bone marrow (rare: 3-5%)...differentiate from TB  
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Hypersensitivity Pneumonitis (allergic alveolitis)   an immune mediated inflam lung disease that affects alveoli; usu occupational, restrictive lung disease (dec diffusion capacity, dec compliance, dec lung vol); acute symptoms (4-8hrs: fever, cough, dyspnea)  
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Farmers lung   moldy hay exposure; actinomyces asperigillosis  
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Bagassiosis syndrome   moldy sugar cane exposure; thermophilic actinomycetes  
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Maple bark disease   exposure to maple bark; cryptostroma corticale  
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Humidifier lung   exposure to cool humidifiers; thermophilic actinomyces  
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Pigeon breeders' lung   exposure to pigeon droppings; pigeon ptns or cryptococcosis  
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Bat cave/spelunking lung   exposure to histoplasma capsulatum  
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Hypersensitivity Histological Findings   chronic interstitial inflammation; interstitial fibrosis; obliterative bronchiolitis, granuloma formation  
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Pulmonary embolism   usu HUGE; 95% are embolic from DVTs; 50.000 deaths/yr d/t immobilization, hypercoaguable states, instant death  
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Pulmonary infarct   usu d/t smaller emboli; inadequate circulation to lung; may be septic  
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Pulmonary HTN   results in cor pulmonale; a/w COPD, interstitial diseases, heart disease, recurrent PE, AI disorders (ex: scleroderma); primary or idiopathic  
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Pulmonary Hemorrhage Syndromes   Goodpastures syndrome, Idiopathic pulmonary hemosiderosis; Vasculitis associated hemorrhage (hypersensitivity angiitis, Wegener granulomatosis, Lupus erythematosis)  
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Goodpastures Syndrome   rapidly progressive glomerulonephritis and hemorrhagic interstitial pneumonitis d/t autoantibodies to glomerular and pulmonary BM; causes focal necrosis of alveolar walls, intra-alveolar hemorrhage, fibrosis; hemoptysis & bilat fluffy infiltrates on CXR  
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Pulmonary Defenses: Upper airways   gag reflex and mucociliary elevator  
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Pulmonary defenses: Lower airways   alveolar MQs; PMNs, alternate compliment path (opsonization); IgA secretions; serum IgG & IgM in alveolar fluid (activate comp by classic C3b path); Tcells to control viruses/intracellular organisms  
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Lobar pneumonia   entire lobe involved; streptococcus pneumonia (pneumococcus)  
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Bronchopneumonia   common bacterial pneumonia; patchy lobular distribution; spread via airways  
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Interstitial pneumonia   patchy interstitial inflammation; viral or mycoplasma  
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Streptococcus pneumonia   pneumococcus; G+ diplococci; mc community acquired bacterial pneumonia; usu healthy young-mid age adults (elderly and debillitated too)  
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Pneumococcal pneumonia: lobular characteristics   abrupt onset fever; shaking chills; pleuritic chest pain; productive cough; rusty colored sputum; four stages (congestion, red hepatization, gray hepatization, resolution)  
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Bronchopneumonia   multiple foci of infection; bilateral and basal; poorly delineated firm areas (grey, yellow, red coloration)  
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Pneumonia complications   pleuritis (pleural fibrosis, pyothorax, empyema); abscesses; fibrosis, bronchopleural fistula, bronchiectasis, sepsis  
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Pneumonia: Staphylococcus aureus   G+ cocci, complication of viral (influenza) pneumonia; a/w IVDA, debilitated, chronic lung disease; bronchopneumonia w/abscesses; complications (bacterial endocarditis, brain/kidney abscess)  
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Klebsiella pneumonia: Freidlanders Bacillus   mc Gram-negative bacillary pneumonia; a/w elderly, debilitated, malnourished & alcoholic pts; lobar distribution; hemorrhagic necrosis w/red currant jelly sputum; abscesses, bronchopleural fistula, fibrosis, bronchiectasis  
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Legionella pneumonia   G(-) flagella bacillus; a/w standing water; elderly & compromised; 15-25% fatal; Pontiac fever (milder self-limited resp illness w/same organism); bronchopneumonia (mononuclear exudate, necrosis, silver stain/immunoflu); diff culture; retrospective titer  
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Aspiration Bronchopneumonia   in pts w/dec consciousness or vomiting; alcoholics; gastric contents causes chemical & bacterial pneumonia; mixed aerobic & microerophillic orgs; often fatal; abscesses in survivors  
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Lung abscess   d/t aspiration (infective material or gastric contents); complication of bacterial pneumonia, septic emboli, bronchogenic carcinoma, cryptogenic; mixed anaerobes/aerobes (mc:staph, pseudomonas, klebsiella)  
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Sites of lung abscesses   supapical and axillary R upper lobe; apical R lower lobe; ends of vertical airways; usu multiple; air fluid level on CXR; suppurative/fibrotic periphery on histo; drains into bronchi  
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Clinical features of lung abscess   productive cough (purulent/bloody sputum; positional, resembles bronchiectasis); fever, malaise, wt loss; complications (finger clubbing, 2* amyloidosis, lung infarct, bronchopleural fistula); *rule out underlying carcinoma*  
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Interstitial pneumonia   primary atypical pneumonia (mycoplasma); viral pneumonia  
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Primary atypical pneumonia: mycoplasma   usu kids; sporadic/epidemic; atypical features (non-prod cough, no consolid, no leukocytosis), insidious onset, self-limited, mild, resp distress; interstitial mononuclear infiltrate; type 1 pneumocyte necrosis; hyaline membrane/edema; clear alveoli  
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Viral pneumonia pneumonitis: pathogens   interstitial lung infxn; influenza A, B & C; parainfluenza, respiratory syncitial virus, measles, chickenpox, cytomegalovirus (AIDS_  
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Viral pneumonia pneumonitis: clinical features   fever, HA, malaise, hacking non-prod cough; ill defined patches in lung (no consolidation, marked resp distress); little sputum production; no leukocytosis; Diffuse alveolar damage (type 1 pneumocytes); Necrotizing bronchiolitis w/influenza, measles, CMV  
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Cytomegalovirus: types of infection   herpesvirus; all infected sites show cytopathic change (gigantic cells; huge basophilic intranuclear inclusion, surrounding halo); Cytomegalic inclusion disease; Interstitial Pneumonia (AIDS); Transfusion-induced (mononucleosis-like syndrome)  
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Viral Pneumonia Histopathology   1. type 1 pneumocyte infxn (cytopathic - Adeno, CMV, herpes, measles); Viral inclusions (CMV, herpes) w/necrosis; 2. Interstitial inflam (mononuc cells, edema); 3. Hyaline membrane (diffuse alveolar damage); 4. Necrotiz bronchiolitis (flu, measles, CMV)  
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Anthrax   G+ bacillus; cutaneous infxn (wild/domestic animals); Pasteur's first vaccine; Koch's postulates; pulmonary disease (bioterrorism)  
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Anthrax infection   Sm 1* lung infxn (pneumonia w/sm focus; alveolar MQs carry bacteria to hilar nodes); Bacteria germinate in hilar nodes (hemorrhagic mediastinitis); Hemorrhagic meningitis (cardinal cap); Progress (10days to symptoms; 3 days to death)  
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Anthrax toxins   edema factor (capillary leak w/little cell response); Lethal factor; Protective Antigen (accounts for poor response to antibacterial treatment  
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Deep mycoses: Pathogenic Fungi   dimorphic; cause disease in nml hosts; Histoplasma capsulatum, Coccidioides immitis, Blastomyces dermatitis  
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Deep mycosis: Opportunistic Fungi   ubiquitous; cause disease in compromised hosts; Mucorales (non-septate); Asperigillus (mold-like); Candida sp, Cryptococcus neoformans (yeast-like)  
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Histoplasmosis (histoplasma capsulatum)   mimics TB; Ohio & Central Mississippi River Valley; appalachia; soil, Starling/bat droppings; test w/Histoplasmin skin test  
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Histoplasmosis: Pathology   1* lesion (many periph pulm lesions w/hilar adeonopathy; mimics Ghon complex; progressive fibrosis & calcification); 2* Dx (progressive; upper lobe cavitation); Disseminated Dx (immunocomp/infants; liver, spleen, nodes, meninges, adrenal, upper oral GI)  
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Histoplasmosis Histological Findings   round/oval yeast forms in MQs; visible w/silver not H&E stain; In Lungs (MQ+org in alveoli; *Caseating Granulomas* w/langhan's giant cells, fibrosis, calcification); Disseminated (reticulo-epith cells/MQs+org; no Tcell response = no granulomas)  
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Histoplasmosis: Clinical Findings   similar symptoms to TB; disseminated (fever, hepatosplenomegaly, anemia, leukopenia, thrombocytopenia)  
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Histoplasmosis: Diagnosis   culture; ID org w/special stain; Ag in fluids; Histoplasmin skin test (like TB test, indicates exposure only*); serology 4-6wks  
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Coccidiodomycosis (coccidi   endemic SW USA; San Joaquin Valley Fever; spread by dust; 3 forms: 1. Asymptomatic pulm infxn (60% of exposed); 2. Progressive Pulm Dx; 3. Miliary Dx (systemic spread in darker skin; also w/AIDS)  
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Coccidiodomycosis: Pathology   initial infxn (sm focus w/consolidation in mid/lower lung w/hilar adenopathy); *thick walled non-bud spherules filled w/endospores); *Caseating granulomas; heal w/fibrosis & calcification; progressive/miliary (suppurative inflam d/t loss of cell immunity)  
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Coccidiodomycosis: Clinical findings   Dx by identifying org in sputum/Bx; rising IgM titers; Coccidiodin skin test (indicates exposure only); Prognosis is favorable (worse w/ systemic, bone or CNS involvement)  
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Blastomycosis (blastomyces dermatitis)   3 Forms: 1. Asymptomatic 1* Pulm Infxn; 2. Progressive Pulmn Dx; 3. Disseminated Miliary Dx (rare); endemic areas in central/western US (chicago)  
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Blastomycosis: Pathology   **broad based budding**; suppurative inflam; skin involvement (pseudoepitheliomatous hyperplasia mimics squamous cell carcinoma)  
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Blastomycosis: Clinical Course   non-specific symptoms (like TB); skin papules/fungating ulcers; self-limited requiring no therapy while progressive responds poorly to therapy  
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Blastomycosis: Diagnosis   **NO RELIABLE SKIN or SEROLOGIC TESTS**; diagnosis by direct ID of organism or culture  
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Candidiasis (moniliasis; Candida albicans)   mc fungal disease; normal flora of mouth, GI tract, vagina; causes disease when host defenses are low; Yeast-like cells with **budding hyphae & pseudohyphae (elongated chains w/constriction at attachment); Dx w/culture or Bx  
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Candida clinical disease patterns; Minor infxn of mucosa or skin   vagina, oral cavity (thrush), diaper rash; occurs in diabetics, AIDS pts & nml pts after broad spec Abx  
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Candida clinical disease patterns; Erosive Mucocutaneous candidiasis   usu esophageal involvement; terminally ill; AIDS pts and other immunocomp  
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Candida clinical disease patterns; Systemic Infxn (hematogenous)   immunocomp pts, neonates, nidus of infxn (ex: IV catheter); removal of nidus may be curative  
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Candida pathology: Thrush   grey-white membranes over hyperemic mucosa; superficial mass of matted orgs & inflam debris; intact mucosa w/superficial inflam  
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Candida pathology: Erosive esophageal disease   ulceration, necrosis, acute inflam; underlaying granulation tissue; org in superficial detritus  
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Candida pathology: Systemic candidiasis   abscesses in kidneys, liver, lung; lesions are often hemorrhagic d/t vascular invasion  
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Actinomycosis and Nocardiosis   filamentous, G+ slow growing bacteria (former fungi);  
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Actinomyces   anaerobic normal oral flora; pathogenic w/devitalization of tissue and dec O2 tension; infects jaw, neck, thorax (bronchopneumonia w/abscesses), and abdominal/pelvic areas; **sulfur granules of yellow colonies visible to naked eye on histo**  
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Nocardia   acid fast aerobes; NOT normal flora; cause acute bronchopneumonia w/abscess formation; **branching filaments w/o colonies**  
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Actinomyces & Nocardia Pathology   similar organisms; intense suppuration w/abscess formation; **sinus tracts and fistulae**;  
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Actinomyces & Nocardia Histological Findings   colonies are tangled masses of threads surrounded by radiating/terminal clubbed organisms;  
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Actinomyces & Nocardia Clinical Findings   protracted clinical course; chronic infxn common; fibroblastic rxn (scarring) w/fistulous sinus tracts; Dx by morphology or culture; rarely diagnosed by gross appearance of sulfur granules in sinus drainage (actino)  
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Asperigillosis   2nd mc fungal infxn; spores = asthma/allergic alveolitis typeIII/IV rxns; Colonize (fungus balls in existant lung cavities; no invasion); Invasive Opportunistic Infxn (immunocomp, necro pneumonia, "target lesions"/vascula=sepsis; Flavotoxin (liver cancer)  
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Asperigillosis pathology   **acute angle-branching septate hyphae (40* angle)**; filaments w/uniform widths; fruiting body (holy water sprinkler; rare)  
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Asperigillosis Clinical Findings   invasive disease limited to immunocomp hosts; Dx by organism on Bx or culture usu in tissue, rarely in sputum or blood  
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Cryptococcus neoformans   opportunistic infxn in AIDS, leukemia/lymphoma, Hodkins disease; found in soil and bird droppings; pigeons excrete it w/o having disease; 2 sites of infxn: Pulmonary (mild/asypmtomatic) and CNS (lethal meningitis w/HA and fever)  
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Cryptococcus neoformans: Pathology   **budded yeast w/thick mucus capsule**; capsule is mucicarmine+; **India Ink reveals Halo around capsule**; little/no host response b/c naked organisms  
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Cryptococcus neoformans: Diagnosis   ID organism in sputum, Bx or CSF; Latex Cryptococcal Agglutination Test (LCAT) detects capsular antigen in body fluids (95% sensitive; confirm w/culture); India ink prep (fast/cheap, but insensitive)  
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Pneumocystis Pneumonia (P. jiroveci)   opportunistic; formerly P. carinii; disease limited to AIDS, sick elderly, malnourished infants; genetics like fungi but life cycle like protozoa; latent infxn d/t benign exposure during infancy; host response is interstitial (but bugs are in alveoli)  
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Pneumocystis Pathology   lungs resemble ARDS (heavy, red, firm, diffuse, patchy pneumonia; alveoli filled w/pink foamy proteinaceous exudate; septae thick edematous w/mononuclear infiltrate); detected w/silver stain  
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Pneumocystis Clinical Findings   Dx made by visualizing organism in sputum, BAL or Bx; effective Abx available, but prognosis depends on underlying condition  
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Carcinoma of the lung   mc cause of death worldwide; 85% occur in smokers most linked to *squamous and small cell*; **non-smokers a/w adenocarcinoma**  
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Ras mutations a/w lung carcinoma   Ras mutations (25% adenocarcinoma dec Px; 20% large cell; 5% squamous); very rare in small cell  
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Myc oncogene overexpression and lung cancer   a/w 10-40% of small cell cancers  
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p53 mutations and lung cancer   found in >80% of small cell and 50% of non-small cell  
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Rb mutation and lung cancer   found in 80% of small cell and 25% of non-small cell  
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Genetic mutation found in all lung cancer types   deletion in short arm of chromosome 3 (3p)  
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Types of lung cancer   small cell (20-25%; responds to chemo) and non-small cell (squamous, large cell, adenocarcinoma; doesn't respond to chem)  
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Lung Cancer Prognosis   Grading (according to worst component); Staging is the most important predictor of prognosis (5yr survival: Bronchioalveolar (operable) >> adenocarcinoma > squamous > large cell > small cell (non-operable))  
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Signs and Symptoms of all types of lung cancer   cough, SOB, hemoptysis, CP, pleural effusion, pneumonia  
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Paraneoplastic Syndromes a/w Lung Cancer   Acanthosis nigricans (a/w diabetics); Dermato/polymyositis; Clubbing of fingers; Myasthenic Syndromes (Eaton-Lambert A.I. in *small cell; Progressive multi-focal encephalopathy); Endocrine (Cushings, extra ADH (*small cell), Hypercalcemia (*squamous))  
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Pancoast Tumor   mc a/w squamous cell lung cancer; growth in apex may involve C8, T1, T2 nerves; severe shoulder pain radiating down arm; may paralyze cervical sympathetics; can cause Horner's syndrome  
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Horners Syndrome from Pancoast Tumor   enophthalmos (sunken eyeball); Ptosis of upper eyelid (drooping); Miosis (constriction of pupil); Anhidrosis (lack of sweat)  
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Pathogenesis of lung cancer   **unlike other cancers, the major environmental insult that inflicts damage is smoking**; injury, regeneration of basal layer, metaplasia, dysplasia, CIS, invasive tumor  
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Lung cancer and smoking   87% in smokers or recent quitters; depends on daily amount, tendency to inhale, duration of habit; 10-60x greater risk than non-smokers (also a/w cancers of mouth, pharynx, larynx, esophagus, pancreas, cervix, kidney, bladder); squamous and sm cell mc  
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Other risk factors for lung cancer   industrial hazards (radiation, uranium, asbestos), air pollution (radon), molecular genetics, precursor lesions (squamous dysplasia & Carcinoma In Situ; atypical adenomatous hyperplasia; neuroendocrine cell hyperplasia)  
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Squamous cell carcinoma   closely a/w smoking; mc in men; **central; 90% bronchi**; firm gray lesions extend into parenchyma; well differentiated **pearls & bridges**; highest freq of p53 mutations  
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Adenocarcinoma   mc in women & non-smokers; epithelial neoplasm w/*glandular differentiation w/mucin production*; **peripherally located w/pleural fibrosis**; 4 subtypes (acinar, papillary, solid w/mucin, bronchioalveolar); Size: 2.5cm-->replacing entire lobe  
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Bronchioalveolar   1-5% of all; grows along walls of alveoli **birds on a wire**; 1/3 a/w copious mucin (2/3 no mucin); Stage 1 is good Px; **not linked to smoking**  
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Small cell carcinoma; aka: Oat Cell   *highly malignant*; neuroendocrine features; strongly a/w smoking; **perihilar mass**; grows & mets FAST (70% present at advanced stage); Paraneoplastic syndromes (diabetes insipidus, ectopic ACTH, Eaton-Lambert Syndrome block Ca chan dec ACh weak muscle)  
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Small cell carcinoma histological findings   **chemo responsive**sheets of small cells (round/oval); scant cytoplasm; absent nucleoli; inc mitotic rate  
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Large Cell Carcinoma   10% of all; this is diagnosed by exclusion; poorly differentiated tumors w/no features of squamous, glands, or small cells; large cells w/lots of cytoplasm, prominent nucleoli and vesicular chromatin  
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Carcinoid Tumors   neuroendocrine (serotonin) neoplasms derived from basal layer of resp epithelium; no clear a/w smoking; 50% asymptomatic when found on CXR for something else; hemoptysis, SOB, 55yo  
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Other pulmonary tumors   inflammatory pseudo tumors, pulmonary epithelioid hemangioendotheliomas, carcinomas pulmonary blastoma  
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Pulmonary metastases   1/3 of all fatal cancers; found at autopsy; multiple & circumscribed "cannon ball lesions"; histology resembles a primary tumor  
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Pneumothorax   air in pleural cavity; traumatic (penetrating injury, scuba diving), spontaneous (bleb in young adult), Iatrogenic (lung Bx, positive pressure ventilation)  
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Tension pneumothorax   when pneumothroax is intense enough to shift the mediastinum to the opposite side; must be reduced immediately  
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Hydrothorax   pleural effusion; resembles water (elsewhere in body regarded as edema); heart failure d/t hydrostatic pressure; dec oncotic pressure (nephrotic syndrome, cirrhosis, **severe starvation**)  
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Pyothorax   turbid fluid containing PMNs; d/t infxn (stab wounds, extension of bacterial pneumonia)  
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Empyema   thick pustular pleural effusion; flocculation and fibrosis (tries to wall itself off)  
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Hemothorax   blood in pleural space (ruptured vessel; dissecting aneurysm, stab wound, gun shot wound); fluid may be blood stained, serosanguinous (TB, cancer, pneumonias)  
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Chylothorax   pleural effusion of milky fluid; lipid rich (chyle - separates on standing w/fat layer on top); due to lymphatic obstruction  
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Pleuritis   inflammtion of pleura d/t extension of any pulmonary infxn (visceral pleura, bacterial, viral, collagen vascular disease, pulmonary infarct); sharp stabbing chest pain on inspiration  
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Tumors of the Pleura - solitary fibrous tumor   uncommon localized neoplasm a/w pleura; usu benign (1/3 malignant); 80% arise on visceral pleura; can arise on any mesothelial suface; on a stalk...**not linked to asbestos**  
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Malignant Mesothelioma   neoplasm of mesothelial cells of pleura; **80% a/w asbestos exposure**; no inc risk w/smoking, but definate inc risk of other lung cancers in asbestos exposed smokers; latency period of 12-60yrs; encases lung into fissures/septa; limited to periphery  
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Mesothelioma histological findings   biphasic pattern of epithelial and sarcomatous elements; often difficult to distinguish from adenocarcinoma (use IHC to differentiate: Meso is CEA/Leu-M1 negative and Calretinin/WT-1 positive)  
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