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Flashcards in cystic fibrosis Deck (5)
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1

what are the consequences of cystic fibrosis?

- sweaty sweat
- intestinal blockage
- fibrotic pancreas
- recurrent bacterial lung infections
- filled sinuses
gallbladder and liver disease

2

what is CFTR?

- Cystic Fibrosis Trans-membrane conductance Regulator (CFTR)
- there is a CFTR channel defect so lose inhibition of sodium channel so cells fills with Na+ and increase in H2O so this leads to dryness at airway.
- Gene located on long arm of chromosome 7.

3

what is the most common cause of CF?

Del508 inherited by one of each parent

4

what is the celtic gene?

G551D (Celtic gene = 5% of cases) mutation causes non-functioning channel, helped by Ivacaftor BUT is 200,00 dollars a year.

5

what is the exacerbation management of CF?

ABs , Physiotherapy, Adequate hydration, Increased Dietary input