Cystic Fibrosis & Bronciectasis Flashcards Preview

Clin Med I - Pulmonary > Cystic Fibrosis & Bronciectasis > Flashcards

Flashcards in Cystic Fibrosis & Bronciectasis Deck (28):
1

Define cystic fibrosis (CF)

- multisystem, childhood dz
- genetic disorder of the exocrine glands
- fibrotic dz with obstructive features

2

What does CF affect?

- plasma membrane Na/Cl channel of epithelial cells in lungs

3

What is the result of CF's affect?

- excessive, viscous, sticky, mucous plug

4

What other systems are affected in CF?

- GI
- reproductive
- nearly all exocrine ducts

5

What is the MC, lethal, autosomal recessive inheritance in caucasians?

- CF

6

What is the affected gene in CF?

- CFTR

7

What is the pathophysiology of CF?

- aberrant CFTR protein allows too much influx of Na/Cl
- causes inadequate hydration of bronchotraceal epithelium
- impairs mucocilliary fxn
- allows oportunistic bacteria

8

What is the result of the pathophysiology of CF?

- thick, sticky mucous
- decreased mucocilliary movement
- dehydration of mucuous
- biofilm formation from mucous adhereing
- neutrophilic DNA

9

What is the main cause of death in CF?

- respiratory failure

10

What is linked to accelerated lung disease and death in CF?

- poor nutrition

11

What are the complications of CF?

- cysts
- bullae
- bronciectasis
- hemoptysis
- PNA
- PAH
- cor pulmonale
- clubbing

12

What are the non-pulmonary symptoms of CF?

- meconium ileus (intestinal blockage)
- pancreatic insufficiency
- constitutional symptoms

13

What are the lung S&S of CF?

- persistent cough
- persistent wheezing
- excessive sputum
- barrel chest
- recurrent/severe PNA
- digital clubing

14

What is the PNA bug that complicates CF?

- P. aerunginosa

15

How does the PNA bug complicate CF?

- adheres to mucoid biofilm
- highly resistant to abx
- progresses to function pulm decline and bronchiectasis

16

How is CF detected?

- universal screenings at birth: blood test to detect elevated trypsinogen
- Sweat Test

17

What will the sweat test show in CF?

- excessive NaCl

18

What is the main function of the CFTR protein?

- reabsorb Cl- and Na+ ions in sweat glands

19

What does a loss of CFTR proteins lead to in the sweat test?

- decreased reabsorption and NaCl
- hypertonic sweat

20

What diagnostic test are used for CF?

- sweat test
- if sweat test is inconclusive: nasal potential difference measurement or molecular CFTR DNA mutation test
- PFTs
- CXR

21

What do the PFTs show in CF?

- obstructive patter progressing to restrictive

22

What is the goal of tx in children with CF?

- delay P. aeruginosa

23

What is the goal of tx in adults with CF?

- suppression of established P. aeruginosa

24

What is the best tx approach to CF?

- multidiciplinary

25

What are the txs for CF?

- chest PT
- positive expiratory pressure mask

26

How is PNA treated in CF patients?

- need ID consult
- long term abx

27

How are complications of CF prevented?

- clearance of viscous, lower airway secretions via inhaled DNAase or hypertonic saline
- vaccinations

28

What is the definitive tx for CF?

- lung transplant