CF - L3

Question 1

What structures are in the acinus?

Answer 1

respiratory bronchioles
alveolar ducts
alveolar sacs
alveoli

Question 2

What are the stages of lung development? Describe each one

Answer 2

embryonic: out-pouching of foregut, endodermal
pseudoglandular: branch to level of terminal bronchioles
canalicular: acinar region develops
alveolar: sacules form and become thin walled
postnatal

Question 3

What are the features of the mucociliary escalator?

Answer 3

ASL has 2 layers:
periciliary layer
mucus gel layer
mucus secreted by goblet cells
mucus propelled by cilia (12-15bpm)

Question 4

What are the type I & II pneumocytes?

Answer 4

I: lining cells of alveolus
II: regenerative epithelium, produce surfactant

Question 5

What do restrictive and obstructive diseases do to FRC?

Answer 5

lower it and make it higher respectively

Question 6

What measures FEV1?

Answer 6

spirometer

Question 7

What cells is CFTR expressed on?

Answer 7

apical plasma membrane epithelial cells of airway ducts
alveolar type II
alveolar macrophages
neutrophils

Question 8

How does CFTR affect ENaC?

Answer 8

inhibits it so sodium is not absorbed

Question 9

What functions are lost with no functional CFTR?

Answer 9

ENaC not inhibited (increased Na absorption and water follows)
Cl not excreted

Question 10

What are the ASL volume hyperabsorption consequences?

Answer 10

volume depletion of PCL so cilia cant beat
absence of lubrication
promotes chronic infection

Question 11

CFTR additional ion transport?

Answer 11

bicarbonate
regulation of pH
glutathione
thiocyanate

Question 12

How does the anaerobic lung environment affect microbial infection?

Answer 12

ideal environment for some bacteria

others like Pseudomonas convert to anaerobic biofilm mode

Question 13

How does CF affect inflammation?

Answer 13

promotes chronic airway infection
predominately neutrophils
antiproteases cannot overcome the effects of the inflam response
oxygen radicals (bacterial products)

Question 14

What is ceramide and what is its role in CF?

Answer 14

Its a breakdown of products of sphingomyelin found in plasma membrane. In CF it accumulates in the lipid bilayer and promotes pulmonary inflammation (to do with cell stress)

Question 15

What is bronchiectasis?

Answer 15

abnormal widening of the bronchi or their branches, causing a risk of infection

Question 16

What are two common pathogens in CF?

Answer 16

influenza
staph aureus
also ones that you see hardly anywhere else (normal people can kill them)