Cystic Fibrosis

Question 1

What is cystic fibrosis

Answer 1

Inherited life threatening disease that increases mucus production and thickens sweat mucus digestive fluids causing mucus plugs, plugging up the ducts

Question 2

Pathologic structure changes

Answer 2

Thickens mucus, total airway obstruction lead to atelectasis, and partial airway obstruction lead to hyperinflation

Question 3

Anatomic alterations to lungs

Answer 3

Atelectasis and hyperinflation, mucus plugs, obstructed bronchi and bronchioles, excessive mucus production

Question 4

Etiology

Answer 4

An autosomal recessive gene disorder caused by mutations in a pair genes located on chromosomes 7: both parents must have this gene. Transports chloride ions

Question 5

Abnormal expression of CF

Answer 5

Abnormal transport of sodium and chloride inhibits digestion of proteins and leads to vitamin deficiency

Question 6

What is the gold standard for diagnosing CF

Answer 6

Sweat test aka sweat chlorine test

Question 7

Symptoms

Answer 7

Salty sweat, sinus problems, nasal polyps, enlarged heart, trouble digesting

Question 8

Management

Answer 8

Bronchopulmonary hygiene therapy, meds, antibiotics, lung or heart transplant