CF

Question 1

most common CFTR mutation

Answer 1

DF508 homozygous

Question 2

respiratory manifestations of CF

Answer 2

infection, inflammation, mucus, airway obstruction

Question 3

GI problems in CF

Answer 3

meconium ilieus (distal small intestine obstructed by meconium), distal intestinal obstruction syndrome, constipation, gallstones, liver failure, GERD

Question 4

sweat glands in CF

Answer 4

excess salt production in sweat

Question 5

liver/pancreas in CF

Answer 5

small bile ducts get plugged, exocrine failure of pancreas, CF related diabetes, decreased digestive enzymes, fat soluble vitamin deficiency (ADEK)

Question 6

repro in CF

Answer 6

infertility especially in males

Question 7

bones in CF

Answer 7

osteopenia/osteoporosis

Question 8

tx of pancreatic insufficiency of CF

Answer 8

pancreatic enzyme replacement, fat soluble vitamins, insulin if diabetes is present

Question 9

associations of CF diabetes

Answer 9

weight loss, lung fn decline, mortality

beta cell dysfn, islet destrcution

Question 10

leading mortality/morbidity in CF

Answer 10

BE/ lung disease

Question 11

characteristics of airway disease in CF

Answer 11

infection, inflammation, mucus accumulates, airway remodeling/ BE

Question 12

sx of lung disese in CF

Answer 12

dypsnea, cough, sputum, infectious exacerbations

Question 13

tx of lung disease in CF

Answer 13

antibiotics, airway clearance, CFTR modulators