Cystic Fibrosis Flashcards
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?
Pancreatic insufficiency
Recurrent bronchopulmonary infection
Name the 2 common respiratory pathogens involved in the early years of someone with cystic fibrosis?
Staph aureus
Haemophilus influenza
Who is cystic fibrosis more common in, adults or children?
Adults
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?
Bronchiectasis
Once developed, what does pseudomonas aeruginosa form in alginate film?
Microcolonies
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) Large doses
What does the drug Ivacaftor do in CF?
Opens up chloride channels, and reverses the effects of cystic fibrosis
