Cystic Fibrosis DSA Flashcards Preview

RESP II Exam 2 > Cystic Fibrosis DSA > Flashcards

Flashcards in Cystic Fibrosis DSA Deck (43):
1

cystic fibrosis

autosomal recessive
mutation in CFTR

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

life - to 37yo

2

CF lungs

decreased Cl secretion
-impaired mucociliary clearing

infections, bronchiectasis, fibrosis, mucous plugging

3

CF sinuses

nasal and sinus polyps

4

CF pancreas

acinar dilation, cystic and fibrotic pancreas, exocrine insufficiency

5

CF intestine

meconium ileus secondary to severe obstruction

6

CF sweat gland

failure of Cl absorption

elevation of Cl and Na in sweat

7

CF vas deferens

95% men infertile

8

vitamin absorption in CF

can't absorb fat soluble
-ADEK

9

CF MSK

decreased bone density secondary to decreased vit D absorption

10

CF kidney

nephrolithiasis

11

CF diagnosis

immunoreactive trypsinogen marker for pancreatic injury

genetic mutation analysis

confirmation - sweat test**

12

sweat test

confirm Dx of CF

pilocarpine iontophoresis - stimulates sweat

13

CXR CF

hyperinflation

14

CXR PFT

obstruction

15

progressive irreversible airflow obstruction

COPD

20-30yr after exposure

16

leading cause of COPD

smoking

40mL per year decrease in FEV1

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

17

alpha1 antitrypsin

deficiency - risk fx for COPD

neutrophil elastase

18

emphysema

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

19

centriacinar

emphysema
-distal to terminal bronchiole

smoking

20

panacinar

emphysema
-alveolar ducts and adjacent alveoli

alpha1 antitrypsin

21

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

chronic bronchitis and bronchiolitis

22

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

pulmonary HTN

vascular remodeling including medial smooth m enlargement and intimal fibrosis

23

lung mechanics in COPD

elastic recoil decreased in COPD

and airway resistance increased in COPD

distal airways less than 2mm diamter**

24

hypoxemia

mild in early COPD

hypercapnea - severe COPD

25

blue bloaters

chronic bronchitis COPD

26

pink puffers

emphysema COPD

27

mild COPD

FEV1/FVC < 70

FEV1 > 80%

28

moderate COPD

FEV1/FVC < 70

FEV1 50-80%

29

severe COPD

FEV1/FVC < 70

FEV1 30-50%

30

very severe COP

FEV1/FVC < 70

< 50%

31

CXR COPD

hyperinflation, flat diaphragms, bullae

32

SABAs

albuterol
levalbuterol

33

LABAs

salmeterol
formoterol

34

anticholinergics short acting

ipatroprium bromide

35

anticholinergic long acting

tiotroprium

36

methylxanthines

theophylline

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

37

roflumilast

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

38

prednisone

systemic corticosteroid

39

corticosteroid in COPD

inhaled - reduced exacerbations

40

COPD immunizations

influenza and pneumococcus

41

lung transplant COPD

survival 5 years

for severe incapacitation

42

COPD exacerbation infections

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

43

50% patients FEV1 at 40%

will survive 5 years