cystic fibrosis

Question 1

What is the name of the integral membrane proteins affected impatience with cystic fibrosis?

Answer 1

cystic fibrosis trans membrane conductance regulator (CFTR)
- It is and an ION channel that regulates the amount of composition of secretions in epithelial tissue

Question 2

Fact: the airway and a patient with cystic fibrosis is characterized by an aggressive, unrelenting, neutrophilic inflammatory response with release of proteases and oxidants leading to array, remodeling and bronchiectasis

Answer 2

Fact: cystic fibrosis is an autosomal recessive disease where the CF transmembrane conductance regulator leads to increase production and thickness of secretions

Question 3

how and when is cystic fibrosis diagnosed?

Answer 3

Diagnosed by DNA testing for the presence of the CFTR mutation during newborn screening

Question 4

what are the two types of COPD?

Answer 4

Emphysema and bronchitis

Question 5

What is the difference between emphysema and chronic bronchitis?

Answer 5

• emphysema: characterized by destruction of alveoli with airspace enlargement
• Chronic bronchitis, chronic cough and phlegm with small airway disease, small bronchi and narrow and reducing number

Question 6

What does alpha-1 antitrypsin do?

Answer 6

Inhibits neutrophil elastase activity in the lungs.

Question 7

What is neutrophil elastase?

Answer 7

• released by neutrophils during inflammation
• destroys gram neg bacteria
• can destroy lung tissue