SGL

Question 1

What is the prevalence of CFTR

Answer 1

1/2500 with CF, 1/20 carriers

Question 2

What is the main function of the CFTR protein in the sweat duct

Answer 2

To reabsorb luminal CL and and augment sodium reabsorption through epithelial sodium channels eNAC CFTR mutations result in loss or reduction of chloride secretion into the lumen Active luminal sodium absorption through ENaCs also is increased, and both of these ion changes increase passive water reabsorption from the lumen, lowering the water content of the surface fluid layer coating mucosal cells.

Question 3

what deficiency would you see in fibrotic pancreas?

Answer 3

low fat absorption, low vit A

Question 4

What are the 3 most common lung absesses

Answer 4

Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa

Question 5

Common causes of death in CF patients

Answer 5

cardiopulm

translplant complications

liver