Cystic Fibrosis Flashcards
1
Q
What is the cause of cystic fibrosis?
A
It is a genetic condition whereby a mutation causes a defect in the CFTR protein which is a chloride channel
2
Q
Describe the consequence of a CFTR defect
A
The chlorine channel does not open in response to increased cAMP. So chlorine is not excreted into the human and so there is increased sodium absorbance.
3
Q
What is the epidemiology of CF?
A
1 in 25 people carry the gene
4
Q
State eight signs and symptoms of CF
A
- salty sweat
- intestinal blockage
- fibrotic pancreas
- failure to thrive
- recurrent bacterial lung infections
- bilateral absence of vas deferens
- filled sinuses
- gallbladder & liver disease
5
Q
Describe the management of CF
A
Antibiotics
Treatment for pancreatic insufficiency
Regular clinical reviews
Bowel and liver symptomatic treatment if required
6
Q
What type infections do CF patients typically suffer from?
A
Pseudomonas
