Cystic Fibrosis Flashcards Preview

PA30245 Infection and Immunity > Cystic Fibrosis > Flashcards

Flashcards in Cystic Fibrosis Deck (26)
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1
Q

What is the most common lethal genetic disorder?

A

Cystic Fibrosis

2
Q

What number are carriers of CF in western populations?

A

1 in 25

3
Q

What are the most common causes of morbidity in CF?

A

Lung dysfunction

Infection

4
Q

Is CF autosomal recessive or dominant?

A

Recessive

5
Q

The disease severity of CF is related to what?

A

Mutation

6
Q

What are the 3 forms of CF screening?

A

Immunoreactive trypsinogen (IRT)-Guthrie Test.
Genetic screening.
Sweat test

7
Q

What Cl- level indiates CF?

A

Abov 60mM

8
Q

What Cl- level makes CF unlikely?

A

Below mM

9
Q

What structural changes occur in CF?

A

Bronchial wall thickening
Bronchiectasis
Pneumothorax

10
Q

What causes the decline in lung function in CF?

A

Massive neutrophil infiltration-Frustrated phagocytosis-Failure to clear bac and dying neuts- Inflammatory damage to airway tissue

11
Q

What transfers Cl- out?

A

CFTR

12
Q

What makes mucus difficult to clear in CF?

A

Low volume which leads to sticky viscous mucus

13
Q

What does viscous mucus provide?

A

Growth environment for bacteria

14
Q

How can we partially correct viscous mucus in CF?

A

Hypertonic saline

15
Q

What organisms cause lung infections in CF?

A

Pseudomonas aeruginosa
Staph aureus
Haemophilus influenza
Burkholderia cepacia

16
Q

Why are lung infections difficult to treat in CF?

A

Biofilms grow

17
Q

What prophylactic antibiotics are given to infants with CF for s.aureus?

A

Fluoxacillin

18
Q

What can be inhaled in CF to prevent pathogens?

A

Antibiotics- Tobramycin

19
Q

What are B-agonists used for in CF?

A

Activation of residual CFTR activity

20
Q

What osmotic agents are used in CF?

A

Hypertonic saline

Mannitol

21
Q

Why is Recombinant DNAse used in CF?

A

Breaks down viscous mucus (contains large amounts of DNA)

22
Q

Which defect is most common in CFTR in western europeans?

A

ΔF508

23
Q

What does ataluren target?

A

Class I (G542X) protein synthesis. Forces read through of premature termination codon.

24
Q

What does Lumacaftor target?

A

Class II chanel processing (ΔF508 )

25
Q

What does Ivacaftor target?

A

Class III channel regulation (G551D)

26
Q

What does emiloride do?

A

Blockes sodium resorption by blocking ENaC