Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

CSI > CF > Flashcards

CF Flashcards

1
Q

CF inheritance

A

autosomal recessive

1: 3200 whites
1: 25 are carriers!

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

CF is a form of..

A

bronchiolitis (inflamm. process of bronchioles) (

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

gene involved in CF

A

CFTR gene

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

how many mutations involved in CFTR gene

A

move than 1000, 230 known to be clin. relevant

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

mutation that causes 60% of CF

A

D F508

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

CF is caused by..

A

abnormal chloride channel in membrane (CFTR- cystic fibrosis transmembrane conductance regulator)

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

altered CFTR results in alt. transport Cls and therefore H2O flux across…

A

apical surface of epithelial cells

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

the inadeq. hydration of thracheobronchial epi in the resp. tract impairs the..

A

mucociliary function

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

this produces an abnormal amount of mucous–>obstructs glands and ducts–>tissue damage

A

exocrine gland

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

what else increases sputum viscosity besides mucous?

A

high conc. of EC DNA in airway secretions from chronic inflamm. and autolysis of neutrophils

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

CF is characterized by aggressive ??

A

neutrophilic inflammatory response

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

neutrophilic inflammatory response accompanied by release of these which cause ??

A

proteases and oxidants

cause remodeling and broncheictasis

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

pulmonary secretions obstruct..

A

small and medium sized airways

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

complex bac flora in CF

A

staph aureus, H. flu, pseudomonas

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

early colonization of ??? creates environment for ??? by same genetic strain

A

pseudomonas

lifelong pulmonary infection

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

??? can also adopt a ??? which worsens pt prognosis

A

pseudomonas

mucoid phenotype

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

late infection seen with

indicate better or worse outcome?

A

Burkholderia cepacia

worse

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

CF: inc. risk of nonpulm. manifests

A 
GIT malignancies
osteopenia
arthropathies
pancreatitis
infertility
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

pancreatitis in CF

A

tissue destruction–>fibrotic scarring, cyst prolif., loss of acinar tissue, ablation of norm. pancr. architecture

20
Q

like in lung, this can occur in the pancreas from ???

long term can lead to ??

A

obstruction of ducts
from tenacious exocrine secretions
chronic malabsorption, poor growth, fat soluble vit. insuff, loss of pancr. islet cell mass, CF-rel. DM

21
Q

CF can cause infertility due to

A

congenital bilat. absence of vas deferens w. azoospermia (have functioning spermatogenesis, can’t move?)

  • secretory obstruction of vas
  • females: repro tract secretions
22
Q

CF s/s

A

chronic lung disease/bronchiectasis
malnourished
infertile

23
Q

manifests. of CLD/bronch

A

cough/sputum, dec. exercise tolerance
+/- hemoptysis
+/-chronic sinusitis (nasal sec, polyps)
digital clubbing/inc. AP chest diameter

24
Q

why malnourished in CF?

A

steatorrhea (fat in poop from chronic panc.), diarrhea, abd. pain
biliary cirrhosis and gallstones (blocked intrahep. bile ducts–>multilob cirrhosis)

25
Q

lab findings CF:

ABG

A

hypoxia, chronic compensated resp. acidosis (advanced cases)

26
Q

lab findings CF:

PFTs

A

mixed obstructive and restrictive pattern
air trapping ( high RV: TLC)
red. FVC and TLC
red. pulmonary diffusing capacity (DLCO)

27
Q

CF CXR

A

(early) hyperinflation
(prog) peribronch. cuffing, bronchiectasis, mucous plugging, blebs in apices
+/- inc. interstitial markings, sm. rounded peripheral opacities, focal atelectasis

28
Q

CT: more or less specific for bronchiectasis ID?

A

much more - shows mucous impaction
also shows cystic disease
large cysts predisposed for pneumothoracies

29
Q

CF dx

A

clin symps, fam hx, + newborn screening–>sweat electrolyte measurement (2x, 2 diff days)

30
Q

sweat electrolytes followed by..

A

pilocarpine

31
Q

levels of Cl- elevated above ?? in CF

A

> 60

32
Q

sweat chloride test

A

highly specific (good at ruling in + case), but does not exclude dx–>do genotypic typing

33
Q

CFTR genotyping analyzes ?? genetic defects

A

20-80, can do complete DFTR seq. for complicated cases

34
Q

other CF dx tests

A

semen analysis

assessment of pancreatic function

35
Q

CF tx focus on

A 
clearance and reduction of airway secretions
rev. of bronchoconstriction
tx of resp inf
pancr. enzyme replacement
nutritional and psychosoc. support
36
Q

how to clear lower airway secretions?

A

postural drainage
chest percussion/vibration
positive expiratory pressure/ flutter breathing device (acapella valve)

37
Q

CF pts should also receive

A

ppx pneumococcal vaccines and annual influenza vaccinations

38
Q

other way to clear lower airway secretions: ??? which cleaves the ??? in sputum causing viscosity

assoc. with dec. risk of ?? and dec. need for ??

A

inhaled recombinant hum. DNase
cleaves EC DNA

resp. exacerbations
abx

39
Q

other lower airway clearance tx

A

inhale hypertonic saline 2x/day

short-term abx (test sputum)

40
Q

CF have higher incidence of MAI..

recommend..

A

atypical mycobacteria colonization (MTB!)

yearly screening for AFB

41
Q

if positive for ???

this is demonstrated to reduce exacerbations

A

pseudomonas
long-term abx (duration based on prescence in sputum)
-azithromycin, inhal. tobramycin, aztreonam, colistin, levaquin

42
Q

these reverse CF bronchoconstriction if ???

A

inhal. bronchodilaters

if demonstrate improvement after bronchodil. on PFTs

43
Q

if persistent asthma/ABP mycosis, consider

A

inhal. corticosteroids

44
Q

this is an oral med for ??? mutation

it potentiates ??? and results in these improvements

A

ivacaftor
G551D mutation
potentiates CFTR channel (inc. time open)
improves lung function, dec. pulm. exacerb, dec. sweat Cl-

45
Q

the only definitive CF tx
complications: req..
survival rate..

A

lung transplantation
needs double lung or heart-lung transplant
only few centers offer
3 year survival rt is 55%

46
Q

CF prognosis: longevity inc. or dec?

median survival age?

A

increasing (good!)

36 years

47
Q

death in CF pts typ occurs from

A 
pneumonia
pneumothorax
hemoptysis
term. chronic resp. failure
cor pulmonale

CSI (75 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions