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Flashcards in CF Deck (47):
1

CF inheritance

autosomal recessive
1:3200 whites
1:25 are carriers!

2

CF is a form of..

bronchiolitis (inflamm. process of bronchioles) (

3

gene involved in CF

CFTR gene

4

how many mutations involved in CFTR gene

move than 1000, 230 known to be clin. relevant

5

mutation that causes 60% of CF

D F508

6

CF is caused by..

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

7

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

apical surface of epithelial cells

8

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

mucociliary function

9

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

exocrine gland

10

what else increases sputum viscosity besides mucous?

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

11

CF is characterized by aggressive ??

neutrophilic inflammatory response

12

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

proteases and oxidants
cause remodeling and broncheictasis

13

pulmonary secretions obstruct..

small and medium sized airways

14

complex bac flora in CF

staph aureus, H. flu, pseudomonas

15

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

pseudomonas
lifelong pulmonary infection

16

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

pseudomonas
mucoid phenotype

17

late infection seen with
indicate better or worse outcome?

Burkholderia cepacia
worse

18

CF: inc. risk of nonpulm. manifests

GIT malignancies
osteopenia
arthropathies
pancreatitis
infertility

19

pancreatitis in CF

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

20

like in lung, this can occur in the pancreas from ???
long term can lead to ??

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

CF can cause infertility due to

congenital bilat. absence of vas deferens w. azoospermia (have functioning spermatogenesis, can't move?)
-secretory obstruction of vas
-females: repro tract secretions

22

CF s/s

chronic lung disease/bronchiectasis
malnourished
infertile

23

manifests. of CLD/bronch

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

24

why malnourished in CF?

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

25

lab findings CF:
ABG

hypoxia, chronic compensated resp. acidosis (advanced cases)

26

lab findings CF:
PFTs

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

27

CF CXR

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

28

CT: more or less specific for bronchiectasis ID?

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

29

CF dx

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

30

sweat electrolytes followed by..

pilocarpine

31

levels of Cl- elevated above ?? in CF

>60

32

sweat chloride test

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

33

CFTR genotyping analyzes ?? genetic defects

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

34

other CF dx tests

semen analysis
assessment of pancreatic function

35

CF tx focus on

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

36

how to clear lower airway secretions?

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

37

CF pts should also receive

ppx pneumococcal vaccines and annual influenza vaccinations

38

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

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

inhaled recombinant hum. DNase
cleaves EC DNA

resp. exacerbations
abx

39

other lower airway clearance tx

inhale hypertonic saline 2x/day
short-term abx (test sputum)

40

CF have higher incidence of MAI..
recommend..

atypical mycobacteria colonization (MTB!)
yearly screening for AFB

41

if positive for ???
this is demonstrated to reduce exacerbations

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

42

these reverse CF bronchoconstriction if ???

inhal. bronchodilaters
if demonstrate improvement after bronchodil. on PFTs

43

if persistent asthma/ABP mycosis, consider

inhal. corticosteroids

44

this is an oral med for ??? mutation
it potentiates ??? and results in these improvements

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

45

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

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

46

CF prognosis: longevity inc. or dec?
median survival age?

increasing (good!)
36 years

47

death in CF pts typ occurs from

pneumonia
pneumothorax
hemoptysis
term. chronic resp. failure
cor pulmonale