Week 7 (Genetic, Malignant, and Environmental Lung Disease) Flashcards

Question

Treatment for non-small cell lung cancer

Answer 1

**Stage I and II: surgery** **Stage IIIA**: surgery on select cases; chemo (adjuvant or **neoadjuvant**); **cisplatin** or **carboplatin** based **Stage IIIB** and **IV**: **palliative** (radiation, stereotactic body radiation therapy (SBRT), chemo, thoracentesis/pleurodesis, laser therapy; bronchial stents)

Answer 2

Epidermal growth factor receptor-tyrosine kinase inhibitor Binding of **TK domain** produces a signal transduction cascade --\> **tumor growth and proliferation** **Monoclonal antibodies compete** with binding and therefore **block** signals for tumor proliferation, invasion, metastasis, angiogenesis, inhibition of apoptosis **I**matinib (Gleevac); gefitinib (Iressa), cetuximab (Erbitux), erlotinib (**Tarceva**) Used alone or in conjunction with **other chemotherapy** Greater response and survival with **EGFR mutations** treated with **gefitinib** and **erlotinib** (exon 18, 19, 21), worse (20) Greater response in **females, Asian, adenocarcinoma**

Answer 3

Cell membrane protein involved in central and peripheral **nervous systems** Present in childhood **neuroblastoma**, **lymphoma**, **non-small cell lung cancer** Translocation results in **fusion** protein (**ELM4-ALK**) with activity in **tyrosine kinase domain** In 3-7% of non-small cell lung cancer but in 13% of **light or non-smokers** No overlap in patients with EGFR mutations Typically **adenocarcinoma, younger, male, light or non-smoker** Target therapy with **crizotinib** (Xalkori) effective in 60%

Answer 4

**Limited** (30% at presentation): primary tumor confined to hemithorax and/or mediastinum; disease confined to single radiation port; equivalent to stages I-IIIA of non-small cell carcinoma (pleural and pericardial disease not considered limited disease) **Extensive** (70% at presentation): metastases to contralateral lung; distant metastases (brain, bone marrow, liver, adrenals); usually with some response to chemo and/or radiation New staging to change to stage I-III classification

Answer 5

Surgery: usually not recommended because likely micrometastatic disease and rapid growth; rare solitary lesion amenable to resection **Chemo**: always; 15-20 agents; etoposide and cisplatin most active **Radiation**: improves local control from 10-60%; improved survival; prophylactic cranial radiation after chemo response

Answer 6

**Untreated: 6-17 weeks** Treated: 40-70 weeks; \<1% at 5 years **Limited disease: 14-20 months; 20-40% at 3 years** Extensive disease: 8-13 months; \<5% at 3 years

Answer 7

**Middle aged** (55-74 yo) **\>/= 30 pack year current smokers** or have quit within the **past 15 years** Current smokers encouraged on smoking cessation General good health without signs or symptoms of lung cancer

Answer 8

Lymphatic stomata insert directly into parietal pleura Subpleural lymphatics adjacent to visceral pleura **Fluid enters** pleural space through **parietal pleura** via Starling's forces and **drained** through **visceral pleura** **Proteins, particles, cells drained** via lymphatic stomata in **parietal pleura** 0.5-1L absorbed and reabsorbed from pleural space each day

Answer 9

**pH \> 7.6** WBC \< 1500 nucleated cells/mm3 (75% macrophages, 23% lymphocytes, 1% mesothelial cells, no eosinophils) Protein is 15% of plasma levels (1-1.5 g/dL) Glucose = plasma levels

Answer 10

**Transudate** (**bland** fluid): increase in hydrostatic pressure = **CHF**; decrease in colloid oncotic pressure = **anasarca** albumin or **nephrotic** syndrome; increased negative pressure in pleural space = **atelectasis**; increased transport of peritoneal fluid through diaphragm = **ascites, peritoneal dialysis** **Exudate** (**turbid** fluid): results from **pleural inflammation, lung inflammation** (increased capillary permeability = inflammation), impaired lymphatic drainage = **malignancy** Most common causes of **transudate**: CHF, cirrhosis, PE Most common causes of **exudate**: pneumonia, cancer, PE

Answer 11

**Breach** of pleural space (trauma to parietal or visceral pleura) **Air** collects within pleural cavity Pleural pressure **equilibrates** with atmospheric pressure Air slowly **resorbs** through **vasculature** **Tension pneumothorax:** one way air valve is created so on inspiration, air **enters** one hemithorax from tear in lung or hole in chest wall and **does not exit** on expiration; pleural pressure exceeds atmospheric pressure during entire respiratory cycle

Answer 12

**Blunting** of costophrenic angle **Meniscus** sign **Contralateral** **displacement** of trachea and mediastinum **Free flowing** vs. **loculated**

Answer 13

**Loss of lung markings** Pleural reflection **Deep sulcus sign** in supine patient Tension PTX has **contralateral** displacement of trachea and mediastinum

Answer 14

**Tap** effusion when significant amount of fluid (**\>10mm** on decubitus film or **significant** fluid on **US**) AND **unknown diagnosis**; OR when **respiratory compromise** Insert thoracentesis needle **above** the **rib** because artery, nerve and vein run below the rib Removal of more than 1-1.5L at a time may lead to **re-expansion edema**

Answer 15

Pleural/serum **protein** \>0.5 Pleural/serum **LDH** \>0.6 Pleural LDH \> 2/3 of institution normal Need to fulfill only **1 criteria**

Answer 16

**CHF: bilateral** R \>\> L **Cirrhosis** and **peritoneal dialysis: R side** **Nephrotic syndrome, hypoalbuminemic states: bilateral** with diffuse edema

Answer 17

Pleural/serum **protein \> 0.5** Pleural/serum LDH \< 0.6 Pleural LDH \< 2/3 of institution normal **Transudate** if: **serum-pleural albumin ratio \> 1.2**

Answer 18

**Hypothyroidism** **Heart disease, ascites** **PE** 23% transudative due to **atelectasis** **Malignancy** Early **lymphatic** **obstruction**, concomitant **CHF** **Sarcoidosis**

Answer 19

**Infectious** **Malignant** **Inflammatory**: connective tissue disease **PE** **Pancreatitis** **Postcardiotomy syndrome** **Drug induced** **Esophageal rupture** **Subdiaphragmatic abscess** **Hypothyroidism**

Answer 20

Cell count and diff pH Glucose Culture and sensitivity Cytology Hct Amylase Adenosine deaminase Triglycerides

Answer 21

**Neutrophilic "acute process"**: pneumonia, PE, pancreatitis **Lymphocytic**: malignant, TB, fungal, chylothorax **Eosinophilic**: blood, air, drug reaction (dantrolene, bromocriptine, nitrofurantoin), asbestos exposure, paragonimiasis, Churg-Strauss syndrome **Bloody**: Hct 1-20% (cancer, PE, trauma), Hct \> 50% of peripheral Hct (hemothorax)

Answer 22

TG \>/= **110** **Chylothorax**: milky white fluid, chylomicrons, due to **disruption of thoracic duct** (iatrogenic, lymphoma), **unilateral**

Answer 23

**Empyema**: pH 5.5-7.29, **glucose \<40** **Esophageal** rupture: pH 5.5-7, glucose \<60 **Rheumatoid** **pleuritis**: pH 7, **glucose 0-30** **Malignancy**: pH 6.95-7.29, glucose 30-59 **TB**: pH 7-7.29, glucose 30-59 **Lupus** **pleuritis**: pH 7-7.29, glucose 30-59

Answer 24

**Pancreatis**/pancreatic pseudocyst: **pancreatic** amylase Carcinoma **l**ung: salivary amylase Adenocarcinoma ovary: salivary amylase Lymphoma: macroamylase/salivary amylase Esophageal rupture: salivary amylase Pneumonia: salivary amylase Ruptured ectopic pregnancy: prob salivary amylase

Answer 25

Elevated levels in **TB pleurisy, empyema, rheumatoid pleurisy, malignancy** **Normal** level is highly specific for **absence of TB**

Answer 26

**Simple**: 40-57% of patients with pneumonia will develop a simple effusion; **pH \> 7.2; LDH \< 1000; Glucose \>/= 0.5 of plasma level** **Complicated**: 10-15% of patients with pneumonia will develop a complicated effusion; **pH \< 7.2; LDH \> 1000; \>1/2 hemithorax; glucose \<0.5 of serum level; loculations** **Empyema**: 5% of patients with pneumonia will develop an empyema; gram stain or **culture** **positive**; frank **pus**

Answer 27

**Antibiotics** **Simple**: no drainage, re-tap if patient is not doing well **Complicated**: complete **drainage** (thoracentesis; chest tube +/- fibrinolytics via chest tube; VATS/decortication) **Empyema**: **chest** **tube** +/- **fibrinolytics** via chest tube; VATS/decortication

Answer 28

Approximately 60% of malignant effusions can be diagnosed by **cytology** Increased diagnostic yield with **repeat** **thoracentesis**: 65% diagnosed with first cytology; another 27% diagnosed with second cytology (however ONLY another 5% diagnosed with third cytology, so don't do 3) If suspect malignant effusion, do **closed** **pleural biopsy** (dx additional 7% of malignant disease when used in addition to cytology); video assisted thoracoscopic surgery (**VATS**) has 90% overall diagnosis rate Treatment: **observation**, therapeutic **thoracentesis**, **chest** **tube** insertion with intrapleural **sclerosant**, thoracoscopy with **talc** poudrage (put talc in pleural space), long term indwelling catheter drainage, pleuroperitoneal shunt, pleurectomy Note: **sclerosis** causes **inflammation** of the pleural tissue and **adherence** of the **visceral pleura to the parietal pleura**; need to re-expand lung and have apposition of pleural surfaces prior to sclerosis

Answer 29

**Spontaneous** **primary**: no clinically evident lung disease, no trauma or iatrogenic cause, usually subpleural apical blebs **Spontaneous** **secondary**: due to underlying lung **disease** **Iatrogenic**: direct trauma to visceral or parietal pleura **Traumatic**

Answer 30

**Young thin male 10-30 years old** **Smoking** increases risk 20 fold Chest imaging with **subpleural blebs** R/o connective tissue disease (Marfans) Risk of **recurrence** 30% after first incidence, 60% after second, 90% after third

Answer 31

**Airways** **disease**: COPD, CF, status asthmaticus **Interstitial lung disease**: eosinophilic granulomatosis, sarcoidosis, LAM, tuberous sclerosis, rheumatoid lung disease, IPF, radiation fibrosis **Infectious** disease: PCP PNA, staphylococcal PNA, necrotizing gram negative PNA Other: **ARDS barotrauma, Marfans, EDS**

Answer 32

**Central** **line** **insertion**: neck/subclavian **Transbronchial lung biopsy** **Transthoracic lung biopsy** **Mechanical ventilation**

Answer 33

First occurence: **Stable** patient with **small** PTX (\<3cm apex to cupola) --\> **observation** in ED 3-6 hours; if no change d/c with f/u in 12-48 hours **Stable** patient with **large** PTX (\>/= 3cm apex to cupola) --\> **re-expand lung**, **hospitalization** preferable but can d/c home with **Heimlich valve** **Unstable** patient with large PTX --\> **re-expand lung** and **hospitalize** patient Consider **sclerosis** if activities include high risk if PTX recurred (pilot, scuba diver), persistent air leak **Second** occurrence: **sclerosis** recommended **VATS** preferred intervention for sclerosis: 95-100% success via VATS compared with 78-91% success with chest tube instillation; **VATS bullectomy** to remove apical bullae

Answer 34

Stable patient with small PTX (\<3cm apex to cupola) --\> hospitalize +/- re-expansion of lung Stable patient with large PTX (\>/= 3cm apex to cupola) --\> re-expand lung and hospitalize patient Unstable patient with large PTX --\> re-expand lung and hospitalize patient Proceed with **VATS** **pleurodesis** +/- **bullectomy** as needed

Answer 35

Inflammation of the **lung parenchyma** (pneumonitis) secondary to **infection** Most common cause of **infectious** **death** in US Overall mortality rate for community-acquired bacterial pneumonia 14%

Answer 36

**Mucocilliary covering** in conducting airways **Immunoglobulin A** in mucosal secretions **Macrophages** in respiratory parenchyma can phagocytize microorganisms and if overwhelmed **recruit neutrophils from capillaries**

Answer 37

Filling of **alveoli** with **neutrophil**-rich **exudate**

Answer 38

**Bronchopneumonia**: pattern of consolidation typically involves **more than one lobe** with **bronchiolocentric** distribution (**patchy**) **Lobar** **pneumonia**: uncommon due to availability of antibiotics

Answer 39

**Cough** in more than 90% of patients **Dyspnea** in 66% of patients Sputum production in 66% of patients Pleuritic chest pain in 50% of patients Fever Malaise **Elderly** patients may have **few symptoms**

Answer 40

**Inspiratory** **crackles** is most sensitive finding with any type of pneumonia **Increased tactile fremitus** over consolidated lobe **Dull percussion** over consolidated lobe **Bronchovesicular, egophony, whispered pectoriloquy** over consolidated lobe

Answer 41

All definitions of pneumonia include visualizing an **infiltrate** on a radiograph Limited value in predicting the pathogen Great value in determining **extent** of pneumonia and detecting **complications** like **parapneumonic effusions** or **lung abscesses**

Answer 42

1) **Community** acquired acute pneumonia 2) **Community** acquired **atypical** pneumonia 3) **Healthcare** **associated** pneumonia 4) **Aspiration** pneumonia 5) **Chronic** pneumonia 6) **Necrotizing** pneumonia and **lung abscess** 7) Pneumonia in the **immunocompromised host**

Answer 43

Infectious pneumonia that develops in a patient who is **not hospitalized** or who has been hospitalized for **less than 48 hours** and has not been admitted to a hospital or extended health care facility in the past 90 days Common pathogens: **Strep pneumo, H influenzae, Moraxella** catarrhalis**, Staph aureus, Leigonella** pneumophilia**, Klebsiella** pneumoniae

Answer 44

**Strep pneumo** most **common** cause of community-acquired pneumonia (15-25% of cases) Most common cause of **lobar** pneumonia Paired **diplococci** seen on sputum gram stain Can cause **"rust colored sputum"** **Encapsulated** organism: may disseminate quickly in **asplenic** patients

Answer 45

Typically causes **bronchopneumonia** with multiple abscesses Risk factors are recent **influenza** infection, **IVDU**

Answer 46

Most common **gram** **negative** bacillus to cause bacterial pneumonia Can cause **bronchopneumonia**, **lobar** pneumonia and/or **lung abscess** Abrupt onset of symptoms Cough productive of gelatinous **currant jelly sputum** Risk factors are **alcoholism, debilitation** (cause **aspiration**), **malnourishment**

Answer 47

**Gram negative** bacillus, lives in **water** Responsible for 5% of community acquired pneumonias Fatal in 15% of cases Usually causes **bronchopneumonia** Presents with high fever, GI symptoms (diarrhea, vomiting) Patients commonly **lethargic** with **headache** Lab findings: **hyponatremia** is common as are hematuria and proteinuria and indicators of hepatic dysfunction; sputum gram stain with many **neutrophils** but **few if any microorganisms**; positive **urinary** legionella antigen Risk factors: **old** age, organ **transplantation**, chronic **lung, heart, kidney disease** and hematologic disease

Answer 48

Atypical symptoms: **nonproductive** cough, **headache**, **myalgias**, upper respiratory symptoms, **walking pneumonia** Atypical physical exam, lab and radiographic findings: **unremarkable** physical exam, minimal leukocytosis, lack of lobar consolidation on CXR Atypical (not pneumococcus) pathogens: bacteria that **do not have a cell wall** or **intracellular** **bacteria**; **viruses** Atypical pathogens: **mycoplasma** pneumoniae, **legionella**, **chlamydia** **pneumoniae**, **chlamydia psittaci**, **coxiella** burnetti, (MLC) **influenza** A and B, **parainfluenza** virus (children), **metapneumovirus**, **adenovirus**, **SARS** virus

Answer 49

**Lacks cell wall** so not visible on gram stain Fewer than 10% of people infected develop lower respiratory tract symptoms Most common in **younger** patients (school-aged children and college students) Upper airway symptoms (rhinitis) Notable for **nonpulmonary** features: **erythema nodosum, erythema multiforme, neurologic abnormalities** (meningitis, encephalitis, transverse myelitis, cranial nerve palsies, cerebellar ataxia), **cold agglutinins occasionally** with hemolysis

Answer 50

Upper airway symptoms are **cough, rhinorrhea** and **sore throat** (viral damage to airways) Systemic symptoms abrupt onset of **fever** and **myalgia** (caused by interferon production) May cause lower airway infection (**pneumonia**) on its own: much less common than upper airway infection Damage to lung epithelial layer also leads to risk of **secondary infections** (commonly **pneumococcus** and **Staph aureus**)

Answer 51

**Outpatient**: **strep** **pneumo**, **mycoplasma** pneumoniae, **H influenzae**, respiratory **viruses** **Inpatient (non-ICU)**: **strep** **pneumo**, **mycoplasma** pneumoniae, **chlamydia** pneumoniae, **H influenzae**, **Legionella**, **aspiration** (of mixed oropharyngeal flora), respiratory **viruses** **Inpatient (ICU)**: **strep** **pneumo**, **Legionella**, **staph aureus**, gram negative bacilli, **H influenzae**

Answer 52

PA and lateral **CXR** **Blood cultures prior to antibiotics** (3-14% will be positive in setting of CAP): quality measure tracked by medicare Possible **urine antigen** testing for **Legionella** and **Strep** **pneumo** Sputum culture: optional given that its useful only 14% of the time **CBC** and **complete metabolic panel** **Thoracentesis** should be done if **pleural effusion** present

Answer 53

1 point for: **Confusion** **BUN \> 19** **RR \> 30** **SBP \< 90 or DBP \< 60** **Age 65 or above**

Answer 54

**Macrolide** antibiotic preferred if patient does not have significant comorbidities (**azithromycin**, **clarithromycin**) Respiratory **fluoroquinolone** if patient has comorbidities (**levofloxacin**, **moxifloxacin**)

Answer 55

By convention most physicians treat for **7-10 days** One study shows **levofloxacin x 5 days** is equivalent Certain organisms (**Staph aureus, Legionella**) may need **prolonged** treatment

Answer 56

If patient not improving after 48 hours of treatment, consider pathogen **reisstant** to chosen antibiotics or **spread** of infection beyond the alveoli (**complicated parapneumonic effusion, empyema, suppurative pericarditis**)

Answer 57

Pneumococcal polysaccharide vaccine (**Pneumovax** **23**): given once to adults **\>65** decreases risk of bacteremia and death in patients who get a pneumococcal pneumonia **Influenza**: recommended yearly for everybody **more than 6 years old**

Answer 58

Should be addressed before patient with CAP discharged from hospital Medicare and medicaid quality measure **Quitting** smoking **reduces** risk of pneumococcal disease by **14% each year** thereafter

Answer 59

**PPIs** may be emerging risk factor for **community-acquired bacterial pneumonia** **NSAIDs** are associated with **blunted inflammatory response** which can lead to delay in presentation and higher risk for pleuorpulmonary complications in patients with pneumonia

Answer 60

Includes **hospital-acquired pneumonia** (HAP or nosocomial pneumonia) and **ventilator-associated pneumonia** (VAP), and includes patients who have resided in extended care facility or hospital in **previous 90 days** and those receiving outpatient **hemodialysis**, **chemo** and **wound care**

Answer 61

Develops **more than 48 hours** after **hospitalization**

Answer 62

Develops **more than 48 hours** after **intubation**

Answer 63

**Avoid endotracheal intubation** (consider BiPAP in right settings) Keep **head of bed at 30-45 degrees** Maintain patient's **oral hygiene** **Hand washing** by all medical staff

Answer 64

**Antimicrobial** **therapy** in last 90 days **Current** **hospitalization** for longer than 5 days High-frequency of **antibiotic resistance in the hospital** or the community **Immunosuppressive** **disease** or therapy Residence in **nursing facility** **Family member** with multidrug-resistant pathogen

Answer 65

**Standard** pathogens: **Strep pneumo, H influenzae, E coli, Proteus, serratia marcescens, klebsiella pneumonia, legionella pneumophilia** **Multidrug** **resistant** pathogens: **pseudomonas** aeruginosa, **MRSA**, klebsiella with extended-spectrum beta lactamase (**ESBL**) activity, **acinetobacter**

Answer 66

Anti-psuedomonal cephalosporin (**cefepime** or **ceftazidime**) or anti-psuedomonal carbapenem (**imipenem** or meropenem) or beta lactam + beta lactamase inhibitor (**piperacillin/tazobactam**) PLUS Anti-pseudomonal fluoroquinolone (**ciprofloxacin**) or aminoglycoside (**amikacin, gentamicin**, tobramycin) PLUS **Linezolid** or **vancomycin**

Answer 67

Can change broad-spectrum antibiotic therapy to more tailored regimen based on identification of responsible pathogen and deliniation of drug sensitivities Treat common pathogens for at least **7 days** and multidrug-resistant pathogens for at least **14 days**

Answer 68

If patient **upright**: usually **basal** segment of **lower lobe** (all the way down) If patient **supine**: usually **posterior** segment of **upper lobe** or **apical** segment of **lower lobe** (not all the way down)

Answer 69

**Lowest** hypothalamic set point for temperature is **late** at night Body **wants to be cool** in the middle of the night but you're **warm** because of infection so **get really sweaty** to try to cool down more!

Answer 70

Caused by inhaling **spores** of coccidioies immitis a **dimorphic** fungus found in the **southwestern** and far western US **Granulomatous** pneumonia Lung lesions, **cough**, pleuritic chest pain, **fever**, **erythema** **nodosum** or **erythema** **multiforme** = **San Joaquin Valley Fever Complex** **Disseminated** disease can involve **skin, bone and meninges**

Answer 71

**Dimorphic** fungus endemic to the **Mississippi River Valley** Lives in soils enriched by **bird** or **bat droppings** Like M tuberculosis an **intracellular** parasite of **macrophages** Presentation can mimic that of TB

Answer 72

Blastomyces dermatitidis: soil-inhabiting dimorphic fungus Pulmonary blastomycosis can present as an abrupt illness with productive cough, fever and upper lung involvement on CXR Macrophages have limited ability to kill B dermatitidis, thus **neutrophils** are recruited (**suppurative** **granulomatous** inflammation) Can cause **epithelial** **hyperplasia** that can be mistaken for cancer **Midwest and east coast** **Broad based budding yeast**

Answer 73

**Gram** **positive**, thin **branching** **filamentous** bacterium **Anaerobic**, **not acid fast** (NO and NO!) Colonies **yellow** and smell bad and look like **sulfur** **granules** Risk factor is **COPD** Causes **chronic** **pneumonia**

Answer 74

**Anaerobic** bacteria **Staph aureus** **Klebsiella pneumoniae** **Strep pyogenes** **Strep pneumo type 3** (only type of strep pneumo that cavitates!)

Answer 75

10-15% of abscesses are related to **carcinoma obstructing an airway** (so look for proximal obstructive tumor) **Right-sided bacterial endocarditis** can result in lung abscess (usually see multiple abscesses) Treat with **antibiotics** until radiographic resolution (do NOT stick needle into abscess to drain it)

Answer 76

Now is called **pneumocystis jiroveci** but used to be pneumocystis carinii (**PCP**) Classified as **fungus** (but similar to protozoa) AIDS, occurs when **CD4 count \<200** Presents with **non-specific symptoms**: subtle onset progressive dyspnea, nonproductive cough, **low-grade fever** PE has **tachypnea** and **tachycardia** but normal lung sounds Elevated serum LDH is non-specific (released by injured lung) **Bilateral perihilar interstitial infiltrates** that becomes increasingly homogeneous and diffuse as disease progresses; less often see nodules, pneumatoceles or normal CXR Diagnose with direct fluorescent antibody (DFA) testing of sputum induced with hypertonic saline Treatment: **TMP-SMX** and **corticosteroids** at the same time (esp in patients with PaO2 \<70 on room air or A-a gradient \>35)! Want to reduce **inflammation that is caused by treating PCP**

Answer 77

**Angioinvasive** **molds** that often induce **thrombosis** and **infarction** with subsequent suppuration **Hematogenous** spread common with involvement of **heart valves** and **brain** Often seen in **transplant** patients on corticosteroids or patients with **hematologic** **malignancies** Often diagnosed with **aspergillus EIA** Treated with **voriconazole** and by decreasing immunosuppressant medications **Halo sign**: angioinvasive causes bleeding during invasion **Aspergilloma** will **NOT** invade, but see **air crescent sign** on CXR

Answer 78

Ubiquitous mycobacteria found in **water** and **soil** Opportunistic pathogens in patients with cell-mediated immunodeficiency or underlying structural bronchopulmonary disease **M avium** and **M intracellulare** grouped together as **M avium complex (MAC)** HIV infected patients can get both **pulmonary** disease and **disseminated** disease **Multiple cavitary** or **nodular opacities** on imaging

Answer 79

**Gram** **positive**, **branching** **filaments** **Aerobic and partially acid fast** (YES and YES!) Causes **indolent** **bronchopneumonia** with **abscess** formation 1/5 of infections involve **CNS** Most infected patients have defect in T cell-mediated immunity (prolonged **steroid** use, **HIV**, **diabetes**) Treated with prolonged course of **TMP-SMX**

Answer 80

Rust colored sputum = pneumococcus Currant jelly sputum = klebsiella Alcoholic = klebsiella, aspiration pneumonitis Seizure = aspiration pneumonitis Lung and brain infection (indolent) = nocardia Lung and brain infection (fulminant) = aspergillus Putrid sputum = lung abscess (anaerobes) Sulfur granules = actinomyces Acid fast = M. tuberculosis, atypical mycobacterium, nocardia Pneumonia with bullous myringitis = mycoplasma Walking pneumonia = mycoplasma Pneumonia with unusually high LDH = PCP pneumonia Night sweats = TB Pneumonia after earthquake = coccidiodomycosis Poor dentition = anaerobes Pneumonia on ventilator = MRSA, pseudomonas Foreign born = TB (remember, BCG called "FOB vaccine") Cerebrovascular accident = aspiration pneumonia Dementia = aspiration pneumonia Diplococci = pneumococcus Dysphagia = aspiration pneumonia Coughing with eating = aspiration pneumonia Sickle cell disease = functional asplenia, risk of sepsis with encapsulated organisms (SHiNE SKiS: **strep pneumo, H influenza B**, Neisseria meningitidis, E coli, Salmonella, Klebsiella, group B Strep) Pneumonia with high fever, hyponatremia, and hematuria = legionella Bird or bat droppings = histoplasmosis

Answer 81

Occupational lung diseases: any disorder of the lungs that results from extrinsic etiology related to the **workplace** Lung must eliminate particles without excessive inflammation (but appropriately respond to infectious particles) Often found incidentally on chest imaging and **radiographic findings** may be **out of proportion to symptoms** May **exacerbate** **pre-existing** lung disease (increased deposition and decreased clearance of fibers) **Occupational asthma** and **asbestos-related diseases** are most common in US

Answer 82

**"Dust diseases"** due to accumulation of **inorganic** dusts within the lungs and the tissue reaction to their presence Can be **fibrotic** or **non-fibrotic** Examples**: coal worker's** pneumoconiosis, **asbestos**, **silicosis** **Primary** **prevention** is key because no treatment other than **symptomatic** (eliminate hazard, use ventilator, respirator) Clues: clusters, young age, slower progression of ILD Easily missed: 25% of "IPF" biopsies had mineral deposits and 6% of "sarcoidosis" patients had + beryllium lymphocyte proliferation test!

Answer 83

**Parenchymal/interstitial** lung disease: **pneumoconioses, hypersensitivity pneumonitis**, drug-induced lung disease **Airway**: occupational **asthma, COPD, bronchiolitis** obliterans Physiology: high altitude/diving Smoking, radiation, etc --\> malignancies

Answer 84

**Fibrogenic**: **silica** (silicon dioxide), silicates (asbestos is lower and talc and kaolin are upper), **coal dust, aluminum** **Low-fibrogenic**: deposits of **iron, tin, barium, titanium, glass wool**; **granulomatous** is **beryllium**

Answer 85

Inhalation of **crystalline** **silica** (**quartz**) found in **sand** and **rock** Seen in **hard rock/coal mining**, construction, quarrying, masonry, **sandblasting**, foundry, glass workers, ceramics, granite, stonework Silica generates **O2 radicals** and injures **alveolar macrophages** Note: coal workers can get **CWP and silicosis**!

Answer 86

**Chronic simple silicosis**: latency **10-30 years**, low exposure with variable course; usually see **upper** lung zone **nodular** opacities, can be **calcified**, have **enlarged LN** with **eggshell** calcifications **Accelerated silicosis**: develops within **10 years** of initial exposure, associated with **high-level exposure**, more **severe** and often progressive, **progressive massive fibrosis (PMF)** refers to progressive coalescence of silicotic nodules leading to **respiratory impairment** **Acute silicoproteinosis** or **acute** **silicosis**: exposure to **high concentrations** of silica, symptoms occur within **a few weeks** to a few **years** after initial exposure, **poor prognosis** (dyspnea, cyanosis, cor pulmonale, respiratory failure and death within 4 years); see **bilateral** **alveolar** **filling** process in both **lower lobes**

Answer 87

Complicated silicosis or **PMF** Increased risk of **TB** and **non-TB mycobacterial disease**, **connective tissue disease, lung cancer, chronic bronchitis** **Caplan's syndrome** is when you have RA and silicosis Treatment is **supportive**, very rarely lung transplant

Answer 88

Inhalation of **coal dust** More prevalent in underground workers (higher concentration) **Alveolar macrophages** engulf coal dust and release **toxic** **proteases** and **cytokines** leading to **fibrosis** and **emphysema** **Black lung** Also ask about loading ships with coal, manufacture of carbon electrodes and graphite mining Simple, complicated and p**rogressive massive fibrosis** **Caplan's syndrome** is also RA plus CWP

Answer 89

**Highest** exposure risk is cutting machine operator that **cuts coal directly** **Roof bolters** drill through rock (exposed to silica too); continuous mine operator, loading machine operator, shot firer **Train operators** drop sand onto tracks for traction and may develop silicosis **Motormen, brakemen, drivers, shuttle car operators** have less dust exposure because coal already been cut at this point **Mechanics, electricians** and **maintenance men** have **least** amount of dust exposure

Answer 90

**Upper zone** predominant nodules Can develop **progressive massive fibrosis** and **emphysema** In **complicated** CWP, micronodules have **coalesced** to form PMF (person will die soon)

Answer 91

**Simple pneumoconiosis**: usually no functional deficits **Progressive massive fibrosis (PMF):** larger (\>1cm) opacities, significant functional deficits Workers with simple pneumoconiosis have much greater risk of getting PMF

Answer 92

Asbestos is a type of silicate ("magic mineral") that is cheap, easy to mine and multiple applications Two forms are **serpentine** (chrysotile/white \>90%) and **amphibole** (crocidolite/blue, tremolite, etc) Astestos fiber is critical in disease pathogenesis: released during **mining, milling, fabrication, installation** (\<3 microns in diameter)

Answer 93

**Shipbuilding**, repair or refitting (WWII vets) **Building** fireproofing, maintenance, or demolition **Brake work** **Construction** **Thermal insulation** Plumbers, electricians, welders, present in many rocks Additionally: launderers of asbestos workers' clothes, living or working in buildings constructed in early post-WWII period, houses built between 1930-50 (in inuslation), textured paint and patching in ceiling/walls before 1977, hot water and steam pipe blankets and insulation in older homes, some vinyl floor tiles/backing

Answer 94

Asbestos **plaques** **Diffuse pleural thickening** Asbestosis: pulmonary **parenchymal fibrosis** **Bronchogenic carcinoma** **Malignant mesothelioma** Benign **exudative** or **bloody** **pleural** **effusion** Anxiety

Answer 95

Detectable on **CXR** Very common in asbestos-exposed workers (shipyard workers) Plaques usually develop **first** **Does not predispose** to lung cancer?

Answer 96

**Pulmonary parenchymal fibrosis** Clear dose response relationship **Latency** period ranges **15-40 years** **Dyspnea, cough, crackles, clubbing** Restrictive lung disease with **diffusion impairment** Biospies not required for diagnosis 20-40% progress Higher subsequent risk of **lung cancer** **Progression** is slower (**15-20 years**) compared to IPF (2-3 years)

Answer 97

**Bilateral reticular/linear opacities** at **lung bases** **Honeycombing** (cysts pulled taut) Usually have associated **pleural disease/plaques** Usually **basilar** and **subpleural** distribution If see **pleural** **plaques** with lung **fibrosis**, call it **asbestosis**!

Answer 98

Rare malignancy of **pleura** or **peritoneum** very closely associated with **asbestos** and **not affected by smoking** Occurs even with low levels of exposure especially with amphibole Presents with **large pleural effusion** and **dyspnea** Prognosis poor with limited treatment options (only live 1-1.5 years after this)

Answer 99

Strong **synergy** between **asbestos** and **tobacco** (up to 40-60x increase in exposed smokers) **Asbestosis** markedly increases risk of lung cancer and all histologic subtypes seen Also linked to other malignancies

Answer 100

Work-related asthma: **occupational** **asthma** (with latency/sensitization, without latency/RADS) and **work-exacerbated asthma** **COPD** **Bronchiolitis obliterans**

Answer 101

**Spirometry pre and post-bronchodilator** **Methacholine** challenge testing **Skin testing** **Specific agent** inhalation challenges **Antibody tests**

Answer 102

Results from **allergic** **sensitization** to a specific workplace chemical Once sensitized a **very low dose** can cause an asthma attack Health care workers and **latex** **Isocyanate** in **auto spray paints**, polyurethane foams, adhesives, surface coatings, elastomers, or in manufacture of **plastics** or **rubber** **Carpenters** and **wood dust** Risk factors: **atopy, smoking, genetics** \>50% of patients have **persistent** symptoms/asthma despite **cessation of the exposure** Workers who remain exposed have worse prognosis so early diagnosis matters **Treated** similarly to **non-occupational asthma**

Answer 103

**Reactive airways dysfunction syndrome** (**RADS**) Results from **single high-level exposure** to highly toxic material Symptoms occur **within 24 hours** **Paper mill** explosions (SO2), **chlorine**, **mustard gas** **Direct mucosal injury** --\> airway hyperresponsiveness Can be fatal acutely and treated with oral/inhaled **corticosteroids** May not improve with time

Answer 104

Not just tobacco! **Coal** **Hard rock mining** **Concrete products** **Dust fumes, gas, diesel exhaust** **Wood stoves**

Answer 105

Duration of symptoms: **acute** vs. **chronic** Characteristics: sputum production; **daytime** vs. **nighttime** symptoms (nighttime = asthma) **Associated** **symptoms**: fever, conjunctivitis, runny nose, ear pain, sore throat, nausea, vomiting, abdominal pain, rash Factors/tx that makes it better or worse Additional medical history (birth history)

Answer 106

**Viral URI** **Otitis media** **Sinusitis** **Pharyngitis**: strep, peritonsillar/retropharyngeal abscess, epiglottitis **Croup** **Foreign body aspiration**

Answer 107

**Bronchiolitis** **Asthma** **Pneumonia**

Answer 108

Pathogenesis: **rhinoviruses** (50%), **RSV**, influenza, parainfluenza, coronavirus, adenovirus, enterovirus, immune response to the virus Symptoms: **fever** at onset (typically 24-48 hours), **rhinorrhea**, **earache**, sore **throat**, cough, malaise, headache, duration **10-14 days** Treatment: **no OTC** cough/cold meds for kids less than 2 yo and lack of efficacy and potential toxicity of OTC meds for kids less than 6 years old too; **supportive** **care** (anti-pyretics (Tylenol, Advil \> 6mo?), cool mist humidifier, nasal bulb suction)

Answer 109

Pathogenesis: antecedent URI (AOM is a bacterial superinfection on top of viral URI?), colonization with bacterial otopathogen (**strep pneumo, H influenzae, moraxella catarrhalis**) **Acute** otitis media (**suppurative** infection) Otitis media with **effusion** (inflammation with effusion; can happen with **any viral URI**; is fluid in back of ear that makes it pop) Diagnosis: recent, abrupt onset of signs/symptoms (fever, irritability, URI symptoms); presence of middle ear effusion (bulging TM, limited mobility of TM, air fluid level behind TM, otorrhea); signs or sx of middle ear inflammation (erythema of TM, otalgia) Treatment: 6mo-2yr antibiotics if otorrhea w/AOM, uni/bilateral AOM with severe sx, bilateral AOM w/o otorrhea, but if unilateral AOM without otorrhea can do observation; \>2yr antibiotics if otorrhea w/AOM, uni/bilateral AOM with severe sx, but if uni/bilateral AOM without otorrhea can do observation; use **amoxicillin** (**cefdnir**, **azithromycin**)

Answer 110

**Viral**: acute, self-limited rhinosinusitis, usually less than 14 days **Bacterial**: superinfection with otopathogen (**Strep pneumo, nontypeable H influenzae, Moraxella catarrhalis**) Timing of sinus development: ethmoidal and maxillary at birth (maxillary sinuses are pneumatized by 4yrs); sphenoidal 5yrs; frontal 8-12yrs Diagnosis: **antecedent URI**, including purulent nasal discharge, fever and cough; symptoms persisting for greater than 10-14 days without improvement Treatment: antibiotic (**amoxicillin** (bactrim, azithromycin))

Answer 111

**Viral**: adenoviruses, coronaviruses, enteroviruses, rhinoviruses, RSV, HIV, **EBV** (infectious mononucleosis) **Bacterial**: group A beta-hemolytic streptococcus, corynebacterium **diphtheriae**, mycoplasma pneumoniae, neisseria gonorrhoeae, H influenzae, strep pneumo

Answer 112

Rapid onset fever and sore throat **No cough** Headache and abdominal pain, nausea or vomiting Tonsillar enlargement, white **exudates**, **petechiae** on palate **Painful cervical lymphadenopathy** Symptoms of **scarlet fever:** circumoral pallor and strawberry tongue, red papular sandpaper rash

Answer 113

**Reduce** clinical **symptoms** of fever and pain **Reduce** suppurative **complications** (peritonsillar or retropharyngeal abscess) Reduce non-suppurative complications (acute rheumatic fever, glomerulonephritis), however **can still get glomerulonephritis even if you treat strep throat** Treatment: **penicillin** (amoxicillin), cephalosporins, macrolides

Answer 114

Pathogenesis: H influenza B (**Hib**), but not much anymore because we vaccinate Symptoms: rapid onset of fever, sore throat, dyspnea, **drooling, tripod-ing** Diagnosis: lateral neck radiographs (**thumb** **sign**), direct visualization Management and treatment: establish airway (nasotracheal intubation, tracheostomy), calming measures (avoid unnecessary exams, blood draws, etc), antibiotics (third gen **cephalosporin**, **vancomycin** if MRSA is concern)

Answer 115

Pathogenesis: viral infection of glottis and subglottis (**parainfluenza**, influenza, RSV, adenovirus, enterovirus) Symptoms: rhinorrhea, cough, pharyngitis, fever, **"barking"** cough, hoarseness, inspiratory stridor (high fever at night, cough and stridor **worse at night**) Treatment: supportive care (anti-pyretics, fluids, cool mist, oral dexamethasone, **racemic epi**) Clinical course: improves within **5-7 days**, nighttime symptoms gradually improve

Answer 116

Symptoms: initially sudden onset coughing, gagging, possible **airway** **obstruction**; in asymptomatic period object becomes **lodged**, reflexes fatigue out; complications are obstruction, erosion, infection (fever, hemoptysis, pneumonia, new-onset wheezing) History is key! Location: **right bronchus** \>50% of cases, trachea approximately 10% of cases High-risk objects: **toys**, **food** (nuts, globular objects (hot dogs, grapes)) Treatment: endoscopic **removal**

Answer 117

Inflammation (**bronchiolitis**): viral infections **Asthma** Anatomic abnormalities Mucociliary clearance disorders (**CF**) **Aspiration** Non-pulmonary conditions: heart failure, foreign body aspiration

Answer 118

Pathogenesis: acute **inflammation** with edema, mucus and cellular **debris in the bronchioles**, predominantly viral etiology (**RSV** \>50% of cases, parainfluenza, adenovirus, mycoplasma) 90% of children infected with RSV by age 2 (40% of those will have bronchiolitis) Risk factors for severe disease: age less than 12 wks, history of **prematurity**, congenital heart disease, chronic pulmonary disease, history of **CF**, **immunodeficiency**, environmental risk factors (older siblings, childcare attendance) History: birth hx, NICU hx, prior episodes of wheezing, fam hx of wheezing, sick contacts/daycare exposure PE: vitals (oxygenation and RR; don't be fooled by absence of wheezing), **accessory muscle use** Diagnosis: primarily **clinical**, chest radiograph, viral testing (RSV, influenza, parainfluenza, pertussis) Treatment: **supportive** care, trial of **bronchodilators**, humidified air, supplemental **oxygen**, **Palivizumab** only for **high risk kids** and is very expensive

Answer 119

Treatment for **bronchiolitis** Monoclonal antibody designed to prevent serious lower respiratory tract disease caused by RSV in **high-risk children only** Dose and schedule: IM injection, given monthly Nov-March, **$900 per dose**

Answer 120

Pathogenesis: **airway inflammation**, bronchial **hyperreactivity** and **reversibility** of obstruction Most **diagnosed before 6yo**, most who wheeze in childhood are NOT later diagnosed with asthma, **AA** have more ER visits, hospitalizations, deaths Risk factors: fam hx of parental asthma, allergic history (**eczema, allergic rhinitis, food allergy**), bronchiolitis requiring hospitalization, penumonia, male, environmental tobacco smoke exposure, low birthweight Symptoms: **dry** **cough**, chest **tightness** (persistent **nighttime cough**, associated with exercise), **expiratory** wheezing Triggers: physical activity, airway irritants, **cold or dry air**, infection Evaluation: **FEV1/FVC \<0.8**, low FEV1, **bronchodilator response** (improve FEV1 \>12%), exercise challenge (worsening FEV1 \>15%); peak flow monitoring (daily variation \>20%) Management: assessment and **monitoring** of disease activity, **education** of patient and family, ID triggers and co-morbid conditions, appropriate meds, treat co-morbidity (allergic rhinitis, GERD)

Answer 121

**Mild** **intermittent**: daytime sx \<2 days/wk **Mild persistent**: daytime sx \>2 days/wk but not daily **Moderate** **persistent**: daily sx **Severe** **persistent**: daily sx throughout the day

Answer 122

**Well-controleld**: sx **\<2 days/wk** and once per day Not well-controlled: sx \>2 days/wk or more than twice per day on 2 days/wk Very poorly controlled: sx throughout the day

Answer 123

**Beta agonists**: SABA. LABA **Inhaled corticosteroids**: low- or medium- or high-dose inhaled corticosteroid Oral corticosteroids Leukotriene receptor antagonist Cromolyn (prevents mast cell degranulation), montelukast (leukotriene receptor blocker) Step-wise approach to management

Answer 124

Leading cause of **under-5 deaths** However, abx and vaccinations (Hib and pneumococcal conjugate) caused 97% decrease since 1939! Treatment: **empiric antibiotics** based on age and risk factors, consider hospitalization (age \<6mo, immunocompromised, toxic appearance, moderate-severe respiratory distress, hypoxia, vomiting/dehydration, failure of oral antibiotic therapy, social factors)

Answer 125

Neonates: **Group B strep, E coli,** Strep pneumo, H influenzae 3 wks-3mos: **RSV**, influenza/parainfluenza/adenovirus, strep pneumo, H influenzae, **C trachomatis** 4mo-4yrs: **RSV**, influenza/parainfluenza/adenovirus, strep pneumo, H influenzae, **C trachomatis**, group A strep \>5yrs: M pneumoniae, **Strep pneumo,** **Clamydophila pneumoniae,** H influenzae, influenza and other viruses, legionella **Runts may cough chunky sputum:** RSV, Mycoplasma, chlamydia trachomatis, C pneumoniae, strep pneumo

Answer 126

**Autosomal recessive** CF gene is CFTR protein which is expressed on **epithelial cells of airways, GI tract, sweat glands, GU system** Failure to clear mucous secretions, **paucity of water in mucous secretions**, **elevated salt content in sweat**, **chronic respiratory infections**

Answer 127

Inability to secrete salt and water Excessive reabsorption of salt and water **Insufficient** water on airway surface **to hydrate secretions** Altered microenvironment (more acidic) **aggravates mucociliary clearance** Airway colonization with **staph aureus, pseudomonas,** burkholderia cepacia

Answer 128

Lungs develop **bronchiolitis** --\> bronchitis --\> bronchiolectasis --\> bronchiectasis --\> **bronchiolar obliteration** Paranasal sinuses contain inflammatory products **Pancreas** is usually **small and fibrotic** **Focal biliary cirrhosis** develops with time **Uterine cervix** glands are often distended with **mucus** Epididymis, vas deferens, seminal vesicles are **obliterated** or atretic in most cases

Answer 129

Respiratory: **cough with purulent sputum**, recurrent wheezing/bronchiolitis, prolonged **infections**, often requiring hospitalization GI: **meconium ileus** (abdominal distention, emesis, failure to pass stool within 24-48 hours of life), frequent greasy stools (due to exocrine pancreatic insufficiency), **failure to thrive** (eat but don't gain weight)

Answer 130

**Positive quantitative sweat test** AND Chronic obstructive pulmonary disease Documented exocrine pancreatic insufficiency Positive fam hx Can do genetic testing and newborn screening

Answer 131

**CFTR** **modulators**: Ivacaftor (designed for patients with G551D mutation in at least one CFTR gnee) Antibiotics: **azithromycin**, nebulized **tobramycin** directed at pseudomonas **Bronchodilators** Agents to promote airway secretion clearance (**inhaled DNAse I** cleaves long strands of denatured DNA, hypertonic saline) **Chest physiotherapy** **Vaccination** (influenza and pneumococcal) **Nutritional** treatment: pancreatic enzyme replacement, vitamin supplementation (K, A, D, E), parenteral supplenemtation Lung transplantation: but non-pulmonary manifestations are unchanged **Multi-disciplinary** approach is critical to success (primary care, pulmonology, respiratory therapy, gastroenterology, nutrition, social work, etc) Transition from pediatric to adult providers of care is critical to success (physical, social, economic changes and challenges need to be anticipated for success)

Answer 132

High compliance **Low resistance** Therefore, usually low pressures Normal pressures: **Systolic: 15-30 mmHg** **Diastolic: 3-12 mmHg** **Mean: \<20 mmHg** Mean PA pressure **\>25 mmHg = pulmonary HTN**

Answer 133

Left atrial pressure (**LAP**) Pulmonary vascular resistance (**PVR**) Pulmonary blood flow (**PBF**)

Answer 134

**Precapillary** (increased **PVR**): **primary** PH, **secondary** collagen vascular disease, liver disease, HIV, thromboembolic disease, **hypoxemic** PH (hypoxic pulmonary vasoconstriction, lung disease) **Postcapillary** (increased **LA pressure**): LV systolic or diastolic **heart failure**, aortic and mitral **valve disease** **Mixed**: **chronic** LV failure, chronic aortic and mitral valvular disease Increased **pulmonary blood flow**: congenital heart disease (**ASD, VSD, PDA**), **high output failure, liver disease, anemia**

Answer 135

Based on different treatment options **Pulmonary arterial HTN:** problems in vasculature of lungs **Pulmonary venous HTN:** cardiac conditions **Hypoxic induced:** affects circulation of lungs **Thrombotic emboli:** obstruct proximal portion of pulmonary circulation **Miscellaneous**: tumor obstructing circulation, etc

Answer 136

More **female** than male Etiology **unclear** Genetic: familial form, **AD**, mutation in **BMPR2** Coagulation defects, hormonal (association with **pregnancy**)

Answer 137

Collagen vascular disease (**scleroderma** 20% (especially **CREST** 50%), mixed connective tissue disease 30-40%, **SLE 5**%) **Portal HTN** (2-5% with cirrhosis, 8% at liver transplant) Drugs (**cocaine**, **amphetamines**, **anorexins**) Hemoglobinopathies (**sickle cell anemia, chronic hemolytic anemia**) **HIV** Congenital heart disease (**L to R shunts**) Other: **schistosomiasis**, **Gaucher's** disease, **hereditary hemorrhagic telangiectasia**, persistent pulmonary hypertension of the **newborn** and **thyroid** disorders

Answer 138

**Vasoconstriction**: impaired synthesis of **NO** and **prostacyclin**; enhanced production of endothelin and thromboxane **Vascular wall remodeling**: concentric **medial hypertrophy** due to smooth muscle and endothelial cell proliferation; **BMPR2** genetic defect (normally inhibits vascular smooth muscle proliferation and favors apoptosis) **Thrombosis**: injury to **endothelium**, abnormal **fibrinolysis**, enhanced **procoagulant** activity, platelet abnormalities

Answer 139

Normal Mild to moderate PH: **intimal hyperplasia** and **medial hypertrophy** Severe PH: **plexiform** **lesion** (abnormal **angiogenesis**, **aneurysmal** formation)

Answer 140

Exertional **dyspnea** (60%) Decreased exercise tolerance Dyspnea at rest Anginal chest pain Syncope

Answer 141

**No limitation** of usual physical activity **Mild** **limitation** of **physical** **activity**. No discomfort at rest. Normal physical activity causes increased dyspnea and fatigue. (This is because decreased CO which is fine at rest but you feel it when you start exercising) **Marked** **limitation** in **physical activity**. No discomfort at rest. **Unable to perform any physical activity**. Dyspnea and/or fatigue may be present **at rest.**

Answer 142

Accentuated **pulmonary** component of **2nd heart sound** in \>90% (**louder** and **delayed**) **Early systolic ejection click** (RVH) **RV S4 gallop** (RVH) **Prominent jugular "a" wave** (RVH) Diastolic murmur of **pulmonary** **regurg**, or holosystolic murmur of **tricuspid regurg** **Jugular "v" waves** with **pulsatile liver** Peripheral **edema** and **ascites**

Answer 143

**R axis deviation** **R atrial enlargement** **Q wave** in V1 **ST-T inversion** in V1-6 Of course RVH because pressing against high pressure of pulmonary circulation

Answer 144

**Enlarged main pulmonary artery shadows** **Pruning**: attenuation of peripheral vascular markings

Answer 145

**Endothelial** **cells have** prostacyclin synthetase and make prostacyclin (**PGI2**), causing **vasodilation** and platelet **aggregation inhibition** **Platelets** contain thromboxane synthase and make thromboxane (**TxA2**) causing **vasoconstriction** and platelet adhesion and **aggregation**

Answer 146

CCBs: diltiazem, nifedipine or amlodipine in large doses; avoid in pts with right heart failure; not commonly used now **Endothelin antagonists**: endothelin causes pulmonary vasoconstriction and vascular smooth muscle cell proliferation; bosentan (**Tracleer**) is oral, nonselective endothelin receptor blocker with hepatic toxicity, **contraindicated in pregnancy** but **first line** treatment despite $$$; ambrisentan **Prostacyclin analogues**: epoprostenol (**Flolan**; potent **vasodilation**, inhibits platelet aggregation, reduces SM proliferation and migration, delivered continuously through **catheter via infusion pump**, $$); **Iloprost** (Ventavis; **inhaled** and can be delivered via nebulizer); Treprostinil (**Remodulin**; subQ **pump** or **IV** administration) **NO**: **inhaled**; potent vasodilator at the area that is being ventilated so has **local** action (passes from alveolus to endothelial cell causing **direct** **relaxation** and **improving V/Q mismatch**, inactivated by binding to Hgb and **no systemic effects** if gets into systemic circulation) **Phosphodiesterase inhibitors**: sildenafil (Viagra), Tadalafil (Levitra), verdenafil (Cialis; inhibit PDE which converts cGMP to GMP, used in ED, contraindicated with nitrates) **Anticoagulation**: pts with PH at high risk for intrapulmonary thrombosis and thromboembolism so use **coumadin** (warfarin)

Answer 147

**Atrial** **septostomy**: increased CO, concomitant loss in arterial saturation, very high mortality rate Lung **transplant**: indicated if pt unresponsive to medications

Answer 148

1) **Hypercoagulability** 2) **Stasis** of blood turbulent flow 3) **Endothelial** **injury** Lines of Zahn confirm thrombus occurred over time

Answer 149

**Immobilization** (air travel) Recent **trauma** and surgery in **past 3 months** Previous **PE or DVT** **Stroke** with limb paresis **Obesity** **Malignancy** Significant history of **cigarette smoking** **Oral contraceptives**, pregnancy, hormone replacement Thrombophilia: **factor V Leiden mutation** (AD point mutation gives resistance to activated protein C)

Answer 150

Most venous thrombus **emboli originate in pelvis** or **deep veins of the leg** Large emboli can lodge at **bifurcation** of **PAs** (**saddle** emboli), more commonly in second, third and fourth branches **Increased PVR** causes PH and acute RH failure **Impaired gas exchange** (increased dead space, R to L shunting, V/Q mismatch secondary to atelectasis, lung infarct) **Alveolar hyperventilation** (reflex) with low PaCO2 **Bronchoconstriction** **Decreased lung compliance** from pulmonary edema and hemorrhage

Answer 151

**All highly non-specific!** **Dyspnea** in 84% **Pleuritic chest pain** in 74% Cough Hemoptysis Tachypnea (\>16) in 92% Tachycardia Accentuated pulmonic component of second heart sound Cardiovascular collapse

Answer 152

**ABG**: hypoxemia, hypocapnia, alkalosis, if circulatory collapse then hypercapnia and acidosis Non-specific **lab** **findings**: leukocytosis, increase ESR, elevated LDH, AST, BNP **EKG**: normal but could have tachycardia, non-specific T wave changes, RH hypertrophy **CXR**: atelectasis, pulmonary parenchymal abnormality and/or pleural effusion (transudate and exudate), but normal CXR in 30%

Answer 153

**Air** CO2 Foreign particles due to IVDA (especially talc) **Amniotic fluid** Bone fragments and marrow **Fat** Cement (from ortho surgery) **FAT BAT**: fat, air, thrombus, bacteria, amniotic fluid, tumor

Answer 154

**Pulmonary HTN** (mean \>25 mmHg) that persists **6 months after diagnosis PE** Occurs in 2-4% pts with PE Presents with all symptoms and findings of pulmonary HTN Treatment is **pulmonary endarterectomy**

Answer 155

Correct diagnosis is important because **anticoagulation** is **life saving** but also can **cause morbidity** **No definitive way** to identify whether pt has PE (clinical eval alone inaccurate and more definitive testing may be inaccurate too) therefore treatment based on both **clinical** **suspicion** and **testing**

Answer 156

**Suspected DVT = 3** **Alternative dx less likely than PE = 3** **HR \> 100 = 1.5** **Immobilization/surgery 4wks = 1.5** **History DVT or PE = 1.5** **Hemoptysis = 1** **Malignancy = 1** 3-6 is moderate (20%) \>6 is high probability (66%)

Answer 157

Assessment for DVT: **ultrasound** **lower extremities**, **D-dimer** (plasmin derived fibrin degradation product that is highly sensitive for DVT) **V/Q scan** **Spiral (helical) CT** angiography scan: excellent with proximal arteries Pulmonary angiogram: gold standard but rarely done today

Answer 158

Hospital mortality 58% **Thrombolysis** **Surgical embolectomy** (requires cardiopulmonary bypass): restricted to pts with absolute contraindication to thrombolysis, **extremely high mortality rate** (only do this if absolute contraindication to thrombolysis)

Answer 159

**Hydration, oxygen, monitoring** **Rapid** **anticoagulation**: unfractionated heparin, low molecular weight heparin (subQ; enoxaparin (Lovenox)), fondaparinux **Long term anticoagulation**: warfarin (coumadin) for at least 3 months to indefinitely, depending on condition

Answer 160

**Compression stockings** or other pneumatic compression devices for lower extremities Pharmacologic prophylaxis: **unfractionated heparin** or **low molecular weight heparin, coumadin,** maybe **aspirin** **IVC filter** (umbrella)

Answer 161

**Whooping cough** **Bordatella pertussis** infection Clinical presentation: **Catarrhal** stage 1-2 weeks (common cold sx); **paroxysmal** stage 4-6 weeks (severe cough with distinctive "whoop", cyanosis, post-tussive emesis); **convalescent** stage 4-6 weeks (gradual improvement) Complications: **apnea, seizures, death** Diagnosis: acute cough lasting **14 days** with at least one: **paroxysms** of cough, **inspiratory** **whoop**, post-tussive **emesis** Lab dx: culture of nasopharyngeal secretions, PCR Treatment: **macrolide** antibiotics, most helpful if started in **catarrhal** stage within 1-2 weeks of coughing, post-exposure prophylaxis Vaccination: **DTaP** for age 6 and younger; **TdaP** for ages 7 and older (just different concentrations but both diphtheria, tetanus and acellular pertussis)

Answer 162

**Large**, free-flowing effusion **Loculated** effusion Effusion with **thickened** **parietal pleura** You culture an **organism** from the effusion fluid **Pus** in effusion (empyema!)

Answer 163

If the **ADA** is normal, it is **NOT TB** If there are **mesothelial** **cells** in fluid, it is **NOT TB** (TB encases pleural space and you don't get mesothelial cells)

Answer 164

Vitals stable for 24 hours Able to take oral antibiotics Able to maintain adequate hydration and nutrition Mental status normal (or at base line level) No other active clinical or psychosocial problems requiring hospitalization

Answer 165

**Silicosis** **Berylium exposure** **Occupational asthma** **Hypersensitivity pneumonitis**

Week 7 (Genetic, Malignant, and Environmental Lung Disease) Flashcards

(189 cards)