Cystic Fibrosis DSA

Question 1

cystic fibrosis

Answer 1

autosomal recessive
mutation in CFTR

affects lung, pancreas, intestines, liver, sweat glands, sinuses, vas deferens

life - to 37yo

Question 2

CF lungs

Answer 2

decreased Cl secretion
-impaired mucociliary clearing

infections, bronchiectasis, fibrosis, mucous plugging

Question 3

CF sinuses

Answer 3

nasal and sinus polyps

Question 4

CF pancreas

Answer 4

acinar dilation, cystic and fibrotic pancreas, exocrine insufficiency

Question 5

CF intestine

Answer 5

meconium ileus secondary to severe obstruction

Question 6

CF sweat gland

Answer 6

failure of Cl absorption

elevation of Cl and Na in sweat

Question 7

CF vas deferens

Answer 7

95% men infertile

Question 8

vitamin absorption in CF

Answer 8

can’t absorb fat soluble

-ADEK

Question 9

CF MSK

Answer 9

decreased bone density secondary to decreased vit D absorption

Question 10

CF kidney

Answer 10

nephrolithiasis

Question 11

CF diagnosis

Answer 11

immunoreactive trypsinogen marker for pancreatic injury

genetic mutation analysis

confirmation - sweat test**

Question 12

sweat test

Answer 12

confirm Dx of CF

pilocarpine iontophoresis - stimulates sweat

Question 13

CXR CF

Answer 13

hyperinflation

Question 14

CXR PFT

Answer 14

obstruction

Question 15

progressive irreversible airflow obstruction

Answer 15

COPD

20-30yr after exposure

Question 16

leading cause of COPD

Answer 16

smoking

40mL per year decrease in FEV1

also work dusts, mining, cotton mills, grain-handling

Question 17

alpha1 antitrypsin

Answer 17

deficiency - risk fx for COPD

neutrophil elastase

Question 18

emphysema

Answer 18

enlarged air spaces distal to terminal bronchiole with destruction of alveolar wall

Question 19

centriacinar

Answer 19

emphysema
-distal to terminal bronchiole

smoking

Question 20

panacinar

Answer 20

emphysema
-alveolar ducts and adjacent alveoli

alpha1 antitrypsin

Question 21

enlargement of bronchial mucous glands and increased epithelial goblet cell production leads to cough and mucous production

Answer 21

chronic bronchitis and bronchiolitis

Question 22

hypoxemia leads to vasoconstriction and increased pulmonary vascular resistance in small pulmonary arteries

Answer 22

pulmonary HTN

vascular remodeling including medial smooth m enlargement and intimal fibrosis

Question 23

lung mechanics in COPD

Answer 23

elastic recoil decreased in COPD

and airway resistance increased in COPD

distal airways less than 2mm diamter**

Question 24

hypoxemia

Answer 24

mild in early COPD

hypercapnea - severe COPD

Question 25

blue bloaters

Answer 25

chronic bronchitis COPD

Question 26

pink puffers

Answer 26

emphysema COPD

Question 27

mild COPD

Answer 27

FEV1/FVC < 70 FEV1 > 80%

Question 28

moderate COPD

Answer 28

FEV1/FVC < 70 FEV1 50-80%

Question 29

severe COPD

Answer 29

FEV1/FVC < 70 FEV1 30-50%

Question 30

very severe COP

Answer 30

FEV1/FVC < 70 < 50%

Question 31

CXR COPD

Answer 31

hyperinflation, flat diaphragms, bullae

Question 32

SABAs

Answer 32

albuterol | levalbuterol

Question 33

LABAs

Answer 33

salmeterol | formoterol

Question 34

anticholinergics short acting

Answer 34

ipatroprium bromide

Question 35

anticholinergic long acting

Answer 35

tiotroprium

Question 36

methylxanthines

Answer 36

theophylline antagonizes adenosine recepors -increased cAMP, weak bronchodilator, requires close monitoring

Question 37

roflumilast

Answer 37

PDE4 inhibitor | -increase FEV1 by 50 mL and reduce exacerbations in moderate to severe exacerbations of COPD

Question 38

prednisone

Answer 38

systemic corticosteroid

Question 39

corticosteroid in COPD

Answer 39

inhaled - reduced exacerbations

Question 40

COPD immunizations

Answer 40

influenza and pneumococcus

Question 41

lung transplant COPD

Answer 41

survival 5 years for severe incapacitation

Question 42

COPD exacerbation infections

Answer 42

h flu, streptococcus, moraxella, pseudomonas (hospitalized), rhinovirus, influenza, parainfluenza, RSV

Question 43

50% patients FEV1 at 40%

Answer 43

will survive 5 years