Cystic Fibrosis Flashcards Preview

Respiratory Block > Cystic Fibrosis > Flashcards

Flashcards in Cystic Fibrosis Deck (21):
1

What is the likelihood of someone carrying the CFTR gene?

1 in 25

2

Where does the cystic fibrosis protein sit?

Sits in the cell membrane

3

What can the cystic fibrosis protein act as in terms of chloride ions?

An active transport channel

4

What does cystic fibrosis do to the cilia of an individual?

It collapses the cilia

5

What are the 2 main cardinal features of cystic fibrosis?

Pancreatic insufficiency
Recurrent bronchopulmonary infection

6

Name the 2 common respiratory pathogens involved in the early years of someone with cystic fibrosis?

Staph aureus
Haemophilus influenza

7

Who is cystic fibrosis more common in, adults or children?

Adults

8

What are the 3 main problems with a CFTR abnormality?

Decreased mucociliary clearance
Increased bacterial adherence
Decreased endocytosis of bacteria

9

What is the haemoptysis present in cystic fibrosis due to?

Bronchiectasis

10

Once developed, what does pseudomonas aeruginosa form in alginate film?

Microcolonies

11

What life expectancy decline would you expect to see with pseudomonas aeruginosa in cystic fibrosis?

10 year decline

12

What does the pseudomonas aeruginosa produce?

Toxins which cause tissue damage

13

What kills the patient with Burkholderia cepacia infection?

The immune system goes into overdrive trying to get rid of it

14

How would you want to treat recurrent respiratory tract infections?

Early and aggressively
2 antibiotics (synergistic effect)
Large doses

15

What does the drug Ivacaftor do in CF?

Opens up chloride channels, and reverses the effects of cystic fibrosis

16

What are the drawbacks of Ivacaftor?

It is expensive (£180k pa)
Only works in 5-10% of patients

17

What fraction of CF patients will go on to develop CF related diabetes?

1/3

18

What effect does CF have on puberty in males?

It occurs later (2 years)

19

What percentage of men with cystic fibrosis are infertile?

95%

20

What does fertility in women with CF depend on?

Their nutritional status

21

What is the approximate success rate for ICSI?

25-30%