Pulm review

Question 1

Development stages

Answer 1

from lung bud (distal divirticulum)
Every Pulmonologist Can See Alveoli.
Embryonic stage, Pseudoglandular, Canalicular, Saccular, Alveolar

Question 2

Embryonic stage

Answer 2

Embryonic stage (wk4-7): lung bud-> trachea-> bronchial buds-> main stem bronchi-> secondary Lobar bronchi-> tertiary (segmental bronchi)
Bronchi have hyaline cartilage
Broncioles have no cartilage, Terminal–> respiratory
Alveoli (capillaries and gas exchange

If theresa mistake–> transesophageal fistula

Question 3

Pseudoglandular stage

Answer 3

Wk 5-17
Lung resembles gland, endodermal tubules–> terminal bronchioles surrounded by capillaries

Respiratory bronchioles and alveoli are NOT present, not compatible with life

Fetal respiration- fetus breathes in utero takes up amnion–>stimulates lung development and growth of respiratory muscles, important to growth in pseudoglandular phase

Oligohydroamnios- pulmonary hypoplasia, potters sequence, fetal kidney abnormalities

Question 4

Canalicular phase

Answer 4

wk 16-25, terminal bronchioles divide–> respiratory bronchioles–> alveolar ducts
Respiration capable at the end of the canalicular phase airway diameter increases, pneumocytes start to develop

Type 1 are for respiration
Type 2 secrete surfactant to lower the surface tesnsion and keeps the alveoli open

Question 5

Saccular phase

Answer 5

phase wk 26 to birth
terminal sacs (primitive alveoli) form, capillaries multiply to prep for gas exchange

Question 6

Alveolar period

Answer 6

Week36 to 8 years old
At birth, only 1/3 of alveoli are present , following birth theres an increase in the number of respiratory bronchioles and alveoli
Alveolarization and airspaces subdivide, new walls form septa

Question 7

Bronchopulmonary dysplasia

Answer 7

premature babies need surfactant and O2 with mechanincal ventilation (they dont have surfactant and stong enough muscles to breath)

Ventilation and O2 can cause toxicity, alveolarization doesnt progress normally but during childhood they can do better

Question 8

Pulmonary hypoplasia

Answer 8

oligohydramnios (potters sequence). congenital diaphragmatic hernia- defective formation of pleuroperitoneal membrane (leads to a hole in diaphragm, abdominal organs herniate into chest –> pulmonary hypoplasia Fatal

Question 9

Broncho genic cysts

Answer 9

abnormal budding of foregut and dilitation of terminal/ terminal large, discrete, round fluid filled destension CXR asymptomatic
usually in mediastinum, contain clear fluid –> air when infected No communication of lungs, columnar ciliated

Pulmonary vascular resistance in utero is high, hypoxemia–> vasoconstriction at birth PVR goes down

Question 10

Upper respiriatory tract and lower respiratory tract

Answer 10

Upper: nasal cavity, pharynx and larynx

Lower: trachea, brocni and lungs

Question 11

Conducting zone

Answer 11

NO GAS EXCHANGE, large airways: nose, pharynx, trachea and bronchi
Filters, warms humidifies the air, anatomic dead space

cartilage and goblet cells–> bronchi, pseudostratified ciliary epithelium–> terminal bronchioles “mucociliary escalator”–> cuboid cells
Smooth muscles: sympthetic activation (beta 2) activation–> bronchodilation
Parasympathetic activation M3 –> bronchoconstriction

Question 12

Respiratory zone

Answer 12

GAS EXCHANGE, respiratory bronchioles, alveolar ducts and alveoli
CUBOID in bronchioles–> simple squamous in alveoli
NO cilia, Alveolar macrophages clear debris and immune response

Question 13

Difference between bronchi and bronchioles

Answer 13

Bronchi has cartilage: left and right primary, secondary/tertiary aka lobar or segental,

Bronchioles have NO cartilage: loular /large, terminal respiratory feed alveoli

Question 14

Airway cells

Answer 14

• Goblet cells: secrete mucus (moslty glycoproteins and water) protects against particles and infections
• Ciliated epithelial cells: beating cilia moves mucus to epiglotis, so you can swallow it
• Club cells in bronchioles: non ciliated epithelial cells, secrete protective proteins, detoxify P450
• Trachea and bronchi cells: ciliated pseudostratified columnar cells and GOBLET cells
• Bronchiole cells: epithelium transitions to ciliated simple squamous cuboidal epithelial, and club cells

Question 15

Resistance to air flow

Answer 15

UPPER airways (nose, mouth, pharynx): 50% Airway resistance
LOWER airways- highest in medium bronchi (turbulent flow); lowest in terminal bronchioles- slow turbulent flow

Question 16

ALVEOLI histology

Answer 16

small sacs, separated by septa, simple squamous Pneumocytes, gas exchange, surrounded by capillaries

Type 1 pneumocytes- vast majority of cells in alveoli, thin for gas exchange
Type 2 pneumocytes- produce surfactant, proliferate to form other cell types, key for regeneration after injury

Alveolar Macrophages- phagocytose foreign material, release cytokines and proteases

Surfactant when you exhale, alveoli shrink and want to collapse–> atelectasis, decreases efficiency for gas exchange. Surfactant prevents collapse: mix of lecithins–> DipalmitolPTcholine

Question 17

Neonatal respiratory distress syndrome

Answer 17

Fetal lung maturity: lungs mature when adequate surfactant is present 35 WEEKS
Lecithin-sphingomyein L:S ratio both are 1:1 until 35 weeks, when the ratio is >2:1 its considered mature

NRDS: is a surfactant deficiency, increased surface tension–> alveolar collapse–> atelectasis, ground glass look, hypoxemia an increased CO2 due to poor ventilation, poorly responsive to O2 (lungs are collapsed, intrapulmonary shunting- no gas exchange)

Risk factors: prematurity, maternal diabetes (high insulin decrease surfactant) C section (decreases cortisol, decreases surfactant)
Complications: bronchopulmonary dysplasia (O2 toxicity, no alveolarization) patent ductus arteriosus– hypoxia keeps shunt open, Retinopathy of prematurity (O2 –> free radicals, neovascularization in retina, retinal detachment –> blindness

Treatments: BETAMETHASONE (Corticosteroid given to mom), direct surfactant administration

Question 18

Foreign body aspiration

Answer 18

Commonly with peanuts and kids, Right lung is more common. Site of aspiration is the RIGHT lung (wide, less of angle, more verticle), right 60%, in main bronchus, sometimes in right lower lobe, Left 23% main bronchus small number in left lower,

Question 19

Adequacy of effort and diffusing capability of membrane

Answer 19

Adequacy of effort- the volume of inspired air should be >90% of the largest Vital capacity
Diffusing capacity of membrane: volume of gas that diffuses per minute per mmHg 21 ml/min/mmHg norm

Question 20

Lung volumes:
Tidal volume
Inspiratory volume
Expiratory volume
Residual volume
Total lung capacity
Inspiratory capacity
Vital capacity
Functional residual capacity

Answer 20

Capacity= is multiple volume
Tidal volume: air that moves into lung with each quiet inspiration 500 mL
Inspiratory volume: air that’s still breathed in after tidal inspiration
Expiratory volume: air thats expired out after tidal expiration
Residual volume: air after maximum expiration (expiratory volume) cant be measured by spirometry

Total Lung capacity: all air in lungs at maximum inspiration, Inspiratory reserve volume+ Tidal volume + expiratory volume + residual volume
Inspiratory capacity: air that can be inspired after tidal expiration, IRV + TV
Vital capacity: air that can be expired after maximum inspiration
Functional residual capacity: residual volume after quiet expiration (RV + ERV) volume when system is relaxed

Question 21

Lung pressures

atmospheric pressure, alveolar pressure, intrapleural pressure, transpulmonary pressure

Answer 21

atmospheric pressure: 760 mmHg
Alveolar pressure (PA) pressure in the alveoli
Intrapleural pressure: pressure in pleural space
Transpulmonary pressure: Alveolar pressure-intrapulmonary pressure (need it to keep alveoli OPEN): Negative during normal quiet breathing, alveoli and lungs tend to collapse in on themselves, pull inward /recoild and need an outward force to keep the walls open. Chest wall tends to expand, creates a NEGATIVE pressure in pleural space–> you need to suck the alveoli open

PNEUMOTHORAX: TPP=PA-Pp in pneumothorax Pp goes from -5 to 0 the lung collapses in on itself

Question 22

AIRFLOW and pressure changes (Quiet Breathing)

Answer 22

Inhalation: intrapleural pressure becomes more negative, alveolar pressure becomes negative airflows into the lungs

Exhalation: intrapleural pressure becomes less negative, alveolar pressure becomes positive, airflow out of lungs

Question 23

Lung compliance

Decreased compliance issues, increased lung compliance issues

Answer 23

for a given pressure how much volume changes

Compliance: small amount of diaphragm effort, generates small pressure change across lungs, large volume change, easy to move air in and out
= change of Volume/ change of pressure

A non compliant lung: large amount of diaphragm effort to get a big pressure change across lung and only a small amount of volume change, harder to move air in/out-> decreases Functional reserve capacity

Decreased lung compliance: decrease FRC: Pneumonia, pulmonary edema, pulmonary fibrosis

Increased lung compliance: increased FRC: Emphysema (floppy lungs), Aging, surfactant

Question 24

Emphysema

Answer 24

floppy chest, Increased FRC/lung compliance, increased volume in chest–> Barrel chest

Question 25

Forced exhalation

Answer 25

when pleural pressure becomes positive, compresses airway pressure on alveoli-> positive pressure in airway pushes air out--> air flows from airways

Question 26

Equal pressure point

Answer 26

why forced exhalation does not cause collapse/atelectasis Pleural pressure= airway pressure, beyond the point the airway would collapse, but at that point there is cartilage preventing it from collapsing Diseased lungs: equal pressure point, moves toward alveoli. Obstruction (bronchitis) more pressure drop, tmphysema leads to a loss of elastic recoli, collapses

Question 27

COPD

Answer 27

Slow exhalation, prevents large rise in pleural pressure, forceful exhaaltion would increase pleural pressure, pursed lips--> increased airway and alveolar pressure, prevents collapse

Question 28

Hemoglobin | Dissolved o2 equation

Answer 28

O2 transport- Dissolved O2 (determined by Henry's law: PaO2 x solubility= Dissolved o2 Very small amount (2%) of total blood O2) Bound O2= hemoglobin 98%, positive cooperativity Right Curve shift: unloading of O2, releases R=Release, Things that rise metabolic activity (increased CO2, decreased pH, increased temp, increased 23BPG_ Left curve shift: Latches on the O2, low metabolic activity, decreased CO2, increased pH, decreased temp and decreased 23BPG

Question 29

Chronic hypoxia, CO poisoning, Methemoglobinemia

Answer 29

Chronic hypoxia: increased 23 BPG, COPD, High altitudes, anemia CO poisoning: standard pulse ox, cherry red lips, normal < 3% smokers usually 10-15%, poisoning>15 Methemogglobinemia: oxidized iron Fe cant bind O2, --> hypoxia, anesthetics, Nitric oxide, Dapsone, Methylene blue Chocolate brown blood

Question 30

pulmonary circulation pressure and O2 levels

Answer 30

low pressure system: systemic (120/80), pulmonary artery (24/12), walls of pulmonary artery are very thin, little smooth muscle, low resistance to flow, very distensible Blood O2 levels: system (decrease PaO2)--> vasodilation to increase blood flow in pulmonary: PaO2--> vasoconstriction decrease blood flow to non ventilated areas "hypoxic vasoconstriction", shunts blood away from poorly ventilated areas, more blood to well ventilated areas, key for fetal circulation, low O2 constricts pulmonary arteries in womb--> dilate at birth

Question 31

Gas exchange, perfusion and diffusion limited gas exchange

Answer 31

Inspired air in trachea- PO2=150 mmHg, PCO2 0 mmHg Alveoli air in alveoli: PAO2= 100 mmHg, PACO2 40mmHg Venous blood: Vein PO2= 40 mmHg, PVCO2 46 mmHg Arterial blood PaO2= 90mmHg, PaCO2 40 mmHg Perfusion limited gas exchange: gas transport limited by perfusion (blood flow) more blood flow--> more uptake of gas Diffusion limited gas exchange: gas transport limited by diffusion

Question 32

Diffusing capacity of CO (DLCO) test

Answer 32

measures the ability of lungs to transfer gas, essentially the patient takes up CO (diffusion limited gas). Machine measures the CO exhaled, Normal 75%- 140% predicted , severe disease if <40% Emphysema: destruction of alveoli, decreases surface area Fibrosis/pulmonary edema: thickness increases and distance

Question 33

Resistance to blood flow, vessels in pulmonary vasculature

Answer 33

As blood moves thru pulmonary vasculature, 2 types of vessels: alveolar (capillaries), extra Alveolar (arteries and veins) Increased lung volumes: crushes alveolar vessels--> high resistance, pulls extra-alveolar vessels open

Question 34

Pulmonary hypertension

Answer 34

normal pulmonary Artery P=24/12, Males 10-14 mmHg, considered Pulmonary HTN when mean Pulmonary HTN> 25 mmHg--> Loud P2 at upper left Dyspnea, untreated --> COR pulmonale (Chronic high pressure in right ventricles, right ventricle hypertrophies, eventually dialates and fails, JVVD, lower extremity edema, hepatomegaly, death from heart failure, or arrythmia Diagnosis made via right heart catheterization and non invasively by ECG and ultrasound

Question 35

Pulmonary arteriosclerosis: PpA equations

Answer 35

thickened artery walls, proliferation of smooth muscle cells, thickened media, and narrowing of lumen PpA= CO x PVR + PLA Causes: high left atrial pressure "pulmonary venous HTN", heart failure, valve disease High pulmonary vascular resistance- pulmonary arterial HTN, hypoxemia--> vasoconstriction, COPD, sleep apnea, high altitiudes Chronic pulmonary emboli, scleroderma, cocaid and idiopathic in females Plexiform lesions: pathopneumonic of idiopathic pulmonary arterial hypertension, endothelial proliferation forms multiple tumors BMPR mutations

Question 36

Ventilation/perfusion | Ventilation equation

Answer 36

ventilation= volume x frequency respiratory rate 500cc per breath x 20 breaths a minute = 10000 breaths of cc per minute Alveolar ventilation= useful for gas exchange Dead space ventilation: wasteful ventilation Dead space: anatomy- volume of conducting portions of respiratory tract, nose, trachea Physiologic- anatomic plus volume in alveoli that dont participate in gas exchange, includes functional dead space, insufficient perfusion leads to increased dead space, apex is largest contributor, increases in disease Increased dead space increases CO2

Question 37

Bohrs Dead space equation VD

Answer 37

Vd/ VT= (PaCO2- PeCO2)/ PaCO2 Vt= tidal volume PaCO2= arteria; blood gas PeCO2= exhaled air

Question 38

Alveolar Ventilation equation

Answer 38

``` predicts alveolar CO2 Total Ventilation: TV= volume/min Volume IN is Slighly > Volume OUT due to O2 uptake Minute ventilation, alveolar ventilation= TV - dead space VA= Alveolar ventilation VCO2= rate of CO2 production PACO2= Alveolar CO2 TV= total ventilation Vds= Dead space ventilation K is a constant ``` PACO2= (VCO2 x K)/ VA = (VCO2 x K) / (TV -Vds) 3 major causes of increased CO2= increased Co2, decreased VA, incresed Vds

Question 39

Alveolar Gas equation

Answer 39

``` Predicts Alveolar O2, PAO2= alveolar O2 PIO2= inspired O2, R= rate of exhalation CO2 production/ O2 consumption varies with diet, metabolic rate ``` PAO2= PIO2- PAO2/R

Question 40

Lung perfusion

Answer 40

In upright position= blood flow distribution is uneven, caused by gravity at apex= lowest blood flow, base highest, same with ventilation but less change than perfusion

Question 41

Ventilation/Perfusion V/Q ratio

Answer 41

V/Q ratio: alveolar ventilation/ pulmonary blood flow NORMAL= .8 lowest at base, highest at apex at apex highest V/Q--> increased PaO2 and PaCO2, Tb likes O2 where it lives With exercise only venous blood changes

Question 42

hypoxia

Answer 42

``` O2 delivery to tissues: depends on CO2 and O2 content, need both to be normal O2 content (mlO2)= (O2 binding capacity) (% saturation) + (dissolved O2) ``` Hypoxemia= decrease O2 content of blood Hypxia= decreased O2 delivery to tissue Ischemia= no blood flow hypoxia w/o hypoxemia: HF, anemia, CO HF (cant pump oxygenated blood to tissue), Anemia blood is oxygenated carrying capacity decreases, All Hb is staurated but theres not much Hb CO= takes up Hb sites

Question 43

Hypoxemia

Answer 43

defect in oxygenating blood, categorized by A-a gradient norma A-a O2 gradient= 10-15 PAO2 from alveolar gas equation and Pa from blood gas

Question 44

hypoxemia w/ normal A-a gradient

Answer 44

ALWAYS due to low Alveolar O2 content (low PA), decreased Oxygen content of air- high altitude, PIO2 at sea level 150 mmHg, in colorodo - 100 mmHg Hypoventilation: decreased respiratory rate, reduced tidal volume, causes increased PACO2--> decreased PAO@ narcotics, NM weakness, obesity, improves with O2

Question 45

Hypoxemia with increased A-a gradient

Answer 45

LOW arterial O2 content (PaO2), most primary lung diseases--> pneumonia, pulmonary edema Diffusion defects, shunt, V/Q mismatch Diffusion defects: decreased area (emphysema), increased thickness (fibrosis, and edema) Shunting: no ventilation, venous --> arterial bypasses lung, not really improved with O2, Mismathc, gets better with O2

Question 46

Obstructive lung diseases

Answer 46

Air trapping, slow flow out, less air out Decreased forced expiration (FEV1) , slow flow out in 1st second, decreased FVC (forced vital capacity, less air out) Low FEV1/ FVC RATIO HALLMARK (ratio is less than 70%) Chronic bronchitis, emphysema, COPDs, asthma bronchiectasis, alpha 1 antitrypsin deficiency, primary ciliary dyskinesia, kartageners syndrome, allergic bronchopulmonary aspergillosis

Question 47

Chronic bronchitis

Answer 47

BLUE BLOATERS- enlarged heart and is placed horizontal Chronic cough, productive of sputum, at least 3 months over 2 years No other cause of cough present SMOKERS Hypertrophy of mucosecretory glands, REID index (thickness of gland/ total wall)> 50% Can lead to mucous plug, increased risk of infection poor ventilation of lung--> increased CO2 and decreased O2, hypoxic vasoconstriction, pulmonary HTN Right heart failure--> cor pulmonale Clinical presentation: Cough, wheezing, crackles, dyspnea, cyanosis, Shunting --> hypoxemia O2 doesnt help

Question 48

Emphysema

Answer 48

PINK PUFFERS- ventricle heart, depressed diaphragm SMOKERS, too many proteases are created, overwhelm the anti proteases, upper lung damage alpha 1 anti trypsin deficiency- ineffective- ineffective anti proteases, lower lobe damage Destruction of alveoli: smoke activates Macrophage, recruitment of PMNS, release of proteases Loss of elastic recoil, small airways collapse on exhalation, air is trapped in the lungs Clinical presentation: Dyspnea, cough, hyperventilaiton weight loss, cor pulmonale, BARREL chest, upper lobe, SMOKERS get centriacinar damage (the bronchiole portion of aciner), alpha 1 trypsin deficiency (PAN ACINAR damage the bronchiole and the alveoli)

Question 49

COPD

Answer 49

Chronic Bronchitis, emphysema and asthma, DO NOT GIVE O2 because will lose the loss of the drive to breath

Question 50

Alpha 1 antitrypsin (AAT) deficiency

Answer 50

inherited, Autosomal co-dominant Decreased dysfunctional AAT, balances naturally occuring proteases Proteases is an elastase in PMNs and alveolar Macrophages that stops it from destroying alveoli LUNGS: Panacinar emphysema, imbalance between PMN and elastase inhibitor (AAT)--> lower lung damage Liver cirrhosis- abnormal alpha 1 builds up in liver , only occurs in phyenotypes with pathologic polymerization of AAT in ER of hepatocytes

Question 51

Asthma

Answer 51

REversible bronchoconstriction, usually due to allergic stimulus, type 1 hypersensitivity RXN AIRWAYS hypersensitive, common in kids, associated with atopic rhinitis, eczema, may have family Hx of allergies Triggers: allergens, stress, URI, exercise, cola, aspirin Aspirin- exacerbated asthma asthma, chronic rhinosinusitis, nasal polyposis Chronic asthma/ rhinusinusitus symptoms, acute exacerbation after ingestion of aspirin or NSAIDs, dysregulation of arachidonic acid metabolism, over production of leukotrienens, Treatment (LT receptor antagonist, monteleukasts, zafirlukast), I/E ratio is lowed, mucous plug CURSHMANNS SPIRALS AND CHRO LEYDANOLUS

Question 52

Bronchiectasis

Answer 52

Results of chronic, recurrent, airway inflammation, airways become permanantly dilatated large airways dilates, small medium airways are thickened, recurrent infections, cough, excessive sputum production smells bad, bronchiectasis leads to hemoptysis, corpulmonale, amyloidosis, TUMOR, SMOKERS, CF, kertagners syndrome, allergic aspergillosis

Question 53

Primary ciliary dyskinesia, kertagners syndrome

Answer 53

immotile cilia syndrome, cilia unable to beat, beat normally or absent, inherited (autosomal recessive), gene mutation in dyenin structure formation, dynein is a motor protein creates flow Kertageners syndrome: chronic sinusitis, bronchiectasis, malinfertility, situs infersus

Question 54

Allergic bronchopulmonary aspergillosis

Answer 54

HS type 1 to aspergillus, lungs become colonized with aspergillus, fumigatus low virulence fungus, only infects immuno copromised Asthma/ Cystic fibrosis pts APDA ptients, increased Th2 CD4, synthesis interleukins, eosinophila IGa Ab, treat with steroids

Question 55

Restrictive lung disease

Answer 55

Ratio is normal of (FEV1/FVC) because both are reduced. Interstitial lung disease, dry cough, cant get air in --> less air out decrease FVC, decrease FEV1) Causes : poor breathing mechanics and interstitial lung disease leads to decreased lung compliance, decreased lung expansion (during inspiration) Poor breathing mechanics: not a pulmonary tissue problems, under ventilation of lungs, alveoli working Aa gradient normal neuromuscular problems (ALS POLIO MG) structural problems, (scoliosis and morbid obesity) Interstitial lung disease (bilateral, diffuse pattern, small irregular, opacities, reticulonodular, honey comb appearance DLCO (diffuces capacity (lung CO, normal= extralveolar, decreases interstitium) Low DLCO conditions: interstitial lung disease, emphysema, abnormal vasculature, pulmonaty HTN, embolism, prior lung resection anemia

Question 56

Idiopathic pulmonary fibrosis

Answer 56

most common idiopathic interstitial pneumonia, slow onset dyspnea, typically affect adults>40

Question 57

Pneumoconioses

Answer 57

Coal minors lung: inhalation of coal dust: CXR small round nodules opacities, UPPER LOBES, simple<1cm, complicates> 1 cm with corpulmonale Silicosis: inhaled silica from quartz, granite, sandstone, most US people, FOUNDRIES, Mines, Macrophages respond to silica-->inflammation/fibrosis, TB, no Macrophage killing, cancer, eggshell, Corpulmonale ASbestosis: goes in bronchioles- inhalaltion of asbestos fibers, ship building/ roofing, plumbing, LOWER LOBES, (I) 3 clinical problems (interstitial lung disease, pleural plaques, lung cancer

Question 58

Asbestosis

Answer 58

mainly causes Bronchogenic carcinoma (also mesothelioma is the only risk factor that includes asbstosis), occurs decades after exposure, pleural thickening, pleural effusion, slow onset of symptoms, (dyspnea, cough, chest pain) poor prognosis

Question 59

Hypersensitivity pneumonitis

Answer 59

HS reaction to environmental Ag, agriculture dusts, microorganisms, (fungal bacterial protozoa) Chemicals, mixed type 3 and 4 HS Farmers lung, moldy hay, granier inexposure, also common in bird and pultry handlers Diffese crackles, steroids exposure, chronic granuloma

Question 60

Sarcoidosis

Answer 60

immune mediated granuloma all over the body CD4 TH4 non caseating necrosis in the lungs, skin, eyes and heart Hilar lymphadenopathy Cough dyspnea infiltrates and fibrosis Skin erythema nodosum, uveitis, hypercalcemia, (Vit D) high AC levels Af am females, steroids and immunosuppresants

Question 61

pneumoconioses beryilioses

Answer 61

Beryillium in nuclear and airspace, granulomatous inflammation like TB sarcoidoses, complication corpulmonale and lung cancer

Question 62

Cystic fibrosis

Answer 62

A form of bronchiectasis, inherited genetic diseases, AR both parents carry it, thick sticky mucous in lung and GIT Kids disease Diagnosis via sweat chloride test, pilocarpine, nasal transepithelium, more negative Cystic fibrosis Transmembrane Regulator protein Defect (CFTR), gene encodes, for abnormal protein, ATP ion transporter, epithelial cell function functions ( allows CL ou of ion channel), draws out Na H20, hydrates mucosa and mucus in lungs GIT F508 abnormal folding, CFTR cant get to the membrane Thick mucus in lungs, recurrent pulmonary infection, pseudomonas, S aureus, chronic bronchitis, and bronchiectasis, impaired flow bile and pancreatic secretions, malabsorption of fats Pancreatitis and diabetes (meconum ileus, abdomnial distensionvomiting and clubbing, DNAse is a good treatment, Ivacoftor gets the CFT up there

Question 63

Omalizumab

Answer 63

asthma | IgE binder, prevents mast cell degranulation used for sever asthma despite high dose steroids and dilator

Question 64

Beta 2 adrenergic agonists

Answer 64

asthma RElax airways via smooth muscle dilation SABA: Alburterol immediate reversal of obstruction LABA: salmetorol and formoterol Never use a LABA alone

Question 65

Theophyllines

Answer 65

``` asthma methylxanthine MOA (non selective inhibitor of Phosphodiesterase receptor antagonist of aadenosine ``` SE: headache, tachycardia, arrythmias and seizures

Question 66

Steroids

Answer 66

asthma Prednisone only for asthma thats really bad, ICs 1st line for mild asthma Steroids and Beta 2= better than mutually beneficially

Question 67

Leukotriene modifiers

Answer 67

asthma add on therapy, monteleukast anatgonist of cys L 1 receptor zileuton 5 LO enzyme inhibor

Question 68

Mepolizumab

Answer 68

asthma | anti IL 5

Question 69

COPD treatments

Answer 69

SABAs, anticholinergics (ipratropium), short LABA s can actually be used as a monotherapy, anticholinergics (tiotropium- long acting) ICS (fluticasone and budesonide) Anticholinergics decrease mucus secretion and relaxes airways, SE dry mouth blurred vision, ICSs are better than asthma for COPD

Question 70

Cystic fibrosis treatments

Answer 70

antibiotics and anti inflammatoroy and bronchodilators DNAase alpha- inhaled infiltrating PMNS release thick DNA, dnase breaks it up IVACOFTOR- G551D mutation, opens chloride channel LUM-IVACFOTR: gets the channel up to the membrane