Cystic Fibrosis DSA Flashcards

1
Q

cystic fibrosis

A

autosomal recessive
mutation in CFTR

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

life - to 37yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

CF lungs

A

decreased Cl secretion
-impaired mucociliary clearing

infections, bronchiectasis, fibrosis, mucous plugging

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

CF sinuses

A

nasal and sinus polyps

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

CF pancreas

A

acinar dilation, cystic and fibrotic pancreas, exocrine insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

CF intestine

A

meconium ileus secondary to severe obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

CF sweat gland

A

failure of Cl absorption

elevation of Cl and Na in sweat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

CF vas deferens

A

95% men infertile

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

vitamin absorption in CF

A

can’t absorb fat soluble

-ADEK

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

CF MSK

A

decreased bone density secondary to decreased vit D absorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

CF kidney

A

nephrolithiasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

CF diagnosis

A

immunoreactive trypsinogen marker for pancreatic injury

genetic mutation analysis

confirmation - sweat test**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

sweat test

A

confirm Dx of CF

pilocarpine iontophoresis - stimulates sweat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

CXR CF

A

hyperinflation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

CXR PFT

A

obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

progressive irreversible airflow obstruction

A

COPD

20-30yr after exposure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

leading cause of COPD

A

smoking

40mL per year decrease in FEV1

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

alpha1 antitrypsin

A

deficiency - risk fx for COPD

neutrophil elastase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

emphysema

A

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

19
Q

centriacinar

A

emphysema
-distal to terminal bronchiole

smoking

20
Q

panacinar

A

emphysema
-alveolar ducts and adjacent alveoli

alpha1 antitrypsin

21
Q

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

A

chronic bronchitis and bronchiolitis

22
Q

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

A

pulmonary HTN

vascular remodeling including medial smooth m enlargement and intimal fibrosis

23
Q

lung mechanics in COPD

A

elastic recoil decreased in COPD

and airway resistance increased in COPD

distal airways less than 2mm diamter**

24
Q

hypoxemia

A

mild in early COPD

hypercapnea - severe COPD

25
Q

blue bloaters

A

chronic bronchitis COPD

26
Q

pink puffers

A

emphysema COPD

27
Q

mild COPD

A

FEV1/FVC < 70

FEV1 > 80%

28
Q

moderate COPD

A

FEV1/FVC < 70

FEV1 50-80%

29
Q

severe COPD

A

FEV1/FVC < 70

FEV1 30-50%

30
Q

very severe COP

A

FEV1/FVC < 70

< 50%

31
Q

CXR COPD

A

hyperinflation, flat diaphragms, bullae

32
Q

SABAs

A

albuterol

levalbuterol

33
Q

LABAs

A

salmeterol

formoterol

34
Q

anticholinergics short acting

A

ipatroprium bromide

35
Q

anticholinergic long acting

A

tiotroprium

36
Q

methylxanthines

A

theophylline

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

37
Q

roflumilast

A

PDE4 inhibitor

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

38
Q

prednisone

A

systemic corticosteroid

39
Q

corticosteroid in COPD

A

inhaled - reduced exacerbations

40
Q

COPD immunizations

A

influenza and pneumococcus

41
Q

lung transplant COPD

A

survival 5 years

for severe incapacitation

42
Q

COPD exacerbation infections

A

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

43
Q

50% patients FEV1 at 40%

A

will survive 5 years