Respiratory Physiology

Question 1

4 Symptoms of CF

Answer 1

Persistent productive cough
Airway colonization by pathogens (S aureus, Pseudomonas)
Abnormalities in chest X-ray
Airway obstruction

Question 2

4 General treatments for CF

Answer 2

Aerosol treatment to loosen congestion in the lungs
Antibiotics for infections
Anti-inflammatory drugs
Chest physiotherapy

Question 3

CFTR gene stands for…

Answer 3

Cystic Fibrosis Transmembrane conductance Regulator gene

Question 4

What are the defects from Class 1-6 mutations?

Answer 4

1. No synthesis of protein
2. No maturation of protein
3. Defective regulation
4. Decreased conductance
5. Decreased abundance
6. Defective regulation

Question 5

The combination of which 2 drugs is used to treat CF?

Answer 5

Kalydeco (potentiator - helps the channel work better)

Lumacaftor (helps protein folding so it can travel to the Golgi)

Question 6

CFTR is a ___ channel and regulates what 3 other things

Answer 6

Cl channel
Regulates ENaC (sodium channel), K channels, and the HCO3-/Cl exchanger
Also permeable to small molecules like glutathione and bicarb

Question 7

Functions of resp system

Answer 7

Provide oxygen and remove CO2
Regulate pH
Phonation
Defend against microbes
Influence arterial concentration of chemicals by removing some from pulmonary blood and adding others
Trap and dissolve blood clots

Question 8

4 major functions of the respiratory epithelium

Answer 8

Physical barrier
Antimicrobial function
Regulatory function
Pro-inflammatory function

Question 9

4 types of cells in large airways

Answer 9

Ciliated
Undifferentiated columnar
Secretory
Basal

Question 10

4 types of cells in small airways

Answer 10

Ciliated
Undifferentiated columnar
Clara
Basal

Question 11

Airway surface liquid plays an important role in… (3)

Answer 11

Airway hydration
Innate immunity
Antimicrobial defense

Question 12

2 layers of the ASL

Answer 12

Pericilia layer

Mucus layer

Question 13

ASL contains

Answer 13

Ions
Glycoproteins (mucins)
Proteins involved in airway defence
Water
Electrolytes

Question 14

2 types of submucosal glands

Answer 14

Serous

Mucus

Question 15

What two neuropeptides stimulate submucosal glands

Answer 15

Acetylcholine

VIP

Question 16

Intrinsic neural network

Answer 16

Parasympathetic NS

Innervate SM glands

Question 17

Housekeeping control of low rate gland secretion

Answer 17

Primarily VIP mediated and CFTR dependent

Involves mostly the intrinsic neuronal network

Question 18

Emergency control of high rate of gland secretion

Answer 18

Primarily ACh mediated and CFTR independent

Involves the vagal and central neuronal network

Question 19

Physical defence mechanisms in the upper conducting zone

Answer 19

Turbulent air flow
Coughing
Cilia beating
Epithelial cells shedding

Question 20

Chemical defence mechanisms in the upper conducting zone

Answer 20

Mucus
NO
Increased viscosity

Question 21

Cellular defence mechanisms in the upper conducting zone

Answer 21

Phagocytosis by macrophages

Question 22

2 functions of surfactant

Answer 22

Helps opsonization of micro organisms and antigens

Prevents alveoli from collapsing

Question 23

Surfactant is mostly made up of what phospholipid

Answer 23

Dipalmitoyl phosphatidlycholine

Question 24

VIP function

Answer 24

Vasoactive Intestinal Peptide

Suppression of airway inflammation and airway hyper reactivity