Flashcards in Cystic Fibrosis Deck (21):
What is the likelihood of someone carrying the CFTR gene?
1 in 25
Where does the cystic fibrosis protein sit?
Sits in the cell membrane
What can the cystic fibrosis protein act as in terms of chloride ions?
An active transport channel
What does cystic fibrosis do to the cilia of an individual?
It collapses the cilia
What are the 2 main cardinal features of cystic fibrosis?
Recurrent bronchopulmonary infection
Name the 2 common respiratory pathogens involved in the early years of someone with cystic fibrosis?
Who is cystic fibrosis more common in, adults or children?
What are the 3 main problems with a CFTR abnormality?
Decreased mucociliary clearance
Increased bacterial adherence
Decreased endocytosis of bacteria
What is the haemoptysis present in cystic fibrosis due to?
Once developed, what does pseudomonas aeruginosa form in alginate film?
What life expectancy decline would you expect to see with pseudomonas aeruginosa in cystic fibrosis?
10 year decline
What does the pseudomonas aeruginosa produce?
Toxins which cause tissue damage
What kills the patient with Burkholderia cepacia infection?
The immune system goes into overdrive trying to get rid of it
How would you want to treat recurrent respiratory tract infections?
Early and aggressively
2 antibiotics (synergistic effect)
What does the drug Ivacaftor do in CF?
Opens up chloride channels, and reverses the effects of cystic fibrosis
What are the drawbacks of Ivacaftor?
It is expensive (£180k pa)
Only works in 5-10% of patients
What fraction of CF patients will go on to develop CF related diabetes?
What effect does CF have on puberty in males?
It occurs later (2 years)
What percentage of men with cystic fibrosis are infertile?
What does fertility in women with CF depend on?
Their nutritional status