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Flashcards in CF Deck (9)
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0
Q

Median age of CF survival

A

~41 yrs old

1
Q

Describe pulmonary and extrapulmonary manifestations of CF

A

Multi organ dz

2
Q

CFTR

A

Chloride channel that helps maintain airway surface liquid necessary for lung clearance

3
Q

Mutations

A

.

4
Q

Common features of CF

A
CF PANCREAS
C hronic cough
F ailure to thrive
P ancreatic insufficiency
A lkalosis
N eonatal intestinal obstruction / nasal polyps
C lubbing
R ectal prolapse
E lectrolyte abnormalities 
A bsence of vas deferens
S putum / sinus disease
5
Q

Common CF pathogens

A

Pseudo aeruginosa
Burkholderia cepacia
MRSA
Aspergillus

6
Q

When do we treat CF with positive cultures?

A

When pt exhibits signs of pulmonary exacerbation

7
Q

Symptoms of pulmonary exacerbation

A
Increase cough frequency
Increase SOB
Increase sputum production
Decreased exercise tolerance 
Decreased appetite
Congested
8
Q

Signs of pulmonary exacerbation

A
Increase respiratory rate
Use of accessory muscles for breathing
Decline in PFTs
Fever and leukocytosis
Weight loss
New infiltrate on chest radiograph