Cystic Fibrosis

Question 1

Describe the etiology of cystic fibrosis

Answer 1

Question 2

Describe the common pulmonary presentation of cystic fibrosis

Answer 2

Question 3

Describe the presentation of moderate-advanced and acute exacerbated CF

Answer 3

Question 4

Describe the systemic/multisystem effects of CF

Answer 4

Question 5

Describe the diagnostic testing for CF

Answer 5

Question 6

Describe the diagnostic criteria for CF

Answer 6

Question 7

Describe the treatment for cystic fibrosis

Answer 7

Question 8

Describe the new treatment for CF

Answer 8

Question 9

What is the genetic transmission pattern for CF?

Answer 9

autosomal recessive

Question 10

What population is CF most common in

Answer 10

caucasian

Question 11

Why are secretions thickened/viscous in CF?

Answer 11

abnormal transport of sodium and chloride across secretory epithelia

Question 12

What is the mainstay lab testing for CF?

Answer 12

Sweat chloride testing

Question 13

Describe the class 1 mutation in CF

Answer 13

no functional CFTR protein is made (second most common mutation)

Question 14

Describe a class 2 mutation in CF

Answer 14

CFTR protein is made but misfolded and doesn’t go to the cell surface (MC mutation)

Question 15

When should CF be suspected in older patients?

Answer 15

• recurrent sinopulmonary infections
• frequent pancreatitis
• GI tract abnormalities
• male infertility
• refractory, uncontrolled asthma
• unexplained bronchiectasis