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Molecule to Malady > Lecture 10 - Respiratory System and CF > Flashcards

Lecture 10 - Respiratory System and CF Flashcards

1
Q 
Order of branches in the lungs
1)
2)
3)
4)
5)
6)
7)
8)
A

1) Upper airways
2) Trachaea
3) Bronchi
4) Bronchioles
5) Terminal bronchioles
6) Respiratory bronchioles
7) Alveolar ducts
8) Alveoli

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2
Q 
What are acini? (singular: acinus)
1)
2)
3)
4)
A

1) Respiratory bronchioles
2) Alveolar ducts
3) Alveolar sacs
4) Alveoli

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3
Q 
Stages of lung development
1)
2)
3)
4)
5)
A

1) Embryonic
2) Pseudoglandular
3) Canalicular
4) Sacular/Alveolar
5) Postnatal

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4
Q

Which lung development period is between 0 and 6 weeks of gestation?

A

Embryonic

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5
Q

Which time is the embryonic period of lung development?

A

0 to 6 weeks gestation

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6
Q

Which time is the pseudoglandular period of lung development?

A

6 to 16 weeks gestation

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7
Q

Which stage of lung development is between 6 and 16 weeks development?

A

Pseudoglandular

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8
Q

Which time is the canalicular period of lung development?

A

16 to 26 weeks gestation

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9
Q

Which stage of lung development is between 16 and 26 weeks of gestation?

A

Canalicular

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10
Q

Which time is the sacular/alveolar period of lung development?

A

26 to 36 weeks gestation

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11
Q

Which stage of lung development is between 26 and 36 weeks of gestation?

A

Sacular/alveolar

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12
Q

Which stage of lung development is between birth and teenagehood?

A

Postnatal

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13
Q

When do lung buds first appear?

A

Day 20-26 (embryonic period)

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14
Q

Which germ layer do the lungs originate from?

A

Endoderm

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15
Q

What happens in the embryonic period?
1)
2)
3)

A

1) Lung buds form
2) Ventral outpouching of primitive foregut
3) Endoderm pushes out proximally into surrounding mesoderm

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16
Q

What happens in the pseudoglandualr period?
1)
2)

A

1) Airways branch until terminal bronchioles

2) This entails 16 divisions of airways

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17
Q

What are terminal bronchioles?

A

Pre-acinar bronchi

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18
Q

What happens in the canalicular period?
1)
2)
3)

A

1) Acini form
2) Epithelium thins
3) Pneumocytes form (type I and type II)

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19
Q 
What happens in the sacular/alveolar period?
1)
2)
3)
4)
A

1) Sacules form into alveolar ducts and alveoli
2) Interstitial tissue decreases markedly
3) Septation of alveoli makes more alveoli
4) Walls of sacules become thin

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20
Q

Number of alveoli at birth

A

10^8

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21
Q

Number of alveoli in adulthood

A

3 x 10^8

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22
Q

Lung surface area at birth

A

4M squared

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23
Q

Lung surface in adulthood

A

10M squared

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24
Q 
Functions of the lungs
1)
2)
3)
4)
5)
6)
7)
A

1) Gas exchange
2) Defence
3) Acid-base balance
4) Heat exchange
5) Water balance
6) Phonation
7) Metabolic

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25
Q 
Lung defence mechanisms
1)
2)
3)
4)
5)
A

1) Upper airway filter
2) Reflexes (sneeze, cough)
3) Mucociliary elevator
4) Alveolar macrophages
5) Surfactant

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26
Q

Airways surface liquid layers
1)
2)

A

1) Periciliary layer

2) Mucus layer

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27
Q

What coats the mucociliary elevator?

A

Airways surface liquid

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28
Q

Rate of mucus movement in mucociliary elevator

A

~1mm/minute

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29
Q

What secrete mucus in airways?

A

Goblet cells

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30
Q

Function of type I pneumocytes

A

Make wall of alveoli

Across these O2 is exchanged with alveolar capillaries

31
Q

Function of type II pneumocytes

A

Surfactant secretion

32
Q

How can O2 more easily cross between alveoli and blood?

A

Fused basal laminae of type I pneumocyte and endothelial wall of alveolar capillary

33
Q

Lung volume used at rest

A

Tidal volume

34
Q

Amount of air that you can breathe in over tidal volume

A

Inspiratory reserve

35
Q

Amount of air you can breathe out under tidal volume

A

Expiratory reserve

36
Q

What is inspiratory capacity?

A

Inspiratory reserve + Tidal volume

37
Q

What is vital capacity?

A

Inspiratory reserve + Expiratory reserve + Tidal volume

38
Q

What is residual volume?

A

Amount of air in lungs that can not be exhaled

39
Q

What is functional residual capacity?

A

Expiratory capacity + Residual volume

40
Q

What is the air that can not be breathed out of the lungs?

A

Residual volume

41
Q

What is inspiratory reserve + tidal volume?

A

Inspiratory capacity

42
Q

What is inspiratory reserve + expiratory reserve + tidal volume?

A

Vital capacity

43
Q

What is expiratory capacity + residual volume?

A

Functional residual capacity

44
Q

What is FEV1?

A

Volume exhaled in 1 second

45
Q

What is forced vital capacity?

A

Total volume exhaled in spirometry

46
Q

What is the total volume exhaled in spirometry?

A

Forced vital capacity (FVC)

47
Q

How is lung capacity measured?

A

With a spirometer

FEV1

48
Q

Difference between normal flow-volume curve and CF flow-volume curve

A

With CF, expiratory flow drops very quickly, a airways are narrower.
Takes longer to empty, reinflate lunge

49
Q

Main cause of morbidity and mortality in CF

A

Lung infections

50
Q 
Which cell types in the lungs is CFTR expressed on?
1)
2)
3)
4)
5)
A

1) Ciliated epithelial cells
2) Serous cells of submucosal glands
3) Alveolar type II pneumocytes
4) Alveolar macrophages
5) Neutrophils (role on macrophages and neutrophils not well understood)

51
Q

Important CFTR function in normal lung

A

Modulate airway surface liquid
By Cl- secretion, inhibiting Na+ absorption (by inhibiting ENaC)

ASL reabsorption occurs by ENaC actively taking Na+ into epithelial cell, with Cl- moving passively after

When ASL is depleted, CFTR transports Cl- out of epithelial cell and inhibits ENaC from removing Na+ from lungs.

52
Q 
What happens in CF to airway surfactant liquid?
1)
2)
3)
4)
A

1) Cl- can’t exit type I pneumocytes
2) EnAC Na+ reabsorption isn’t inhibited by CFTR.
3) This results in higher osmotic pressure in pneumocyte
4) Water enters pneumocyte, airway surface liquid is dehydrated, decreases in volume

53
Q

Result of decreased airways surfactant liquid volume
1)
2)
3)

A

1) Mucus layer sits on cilia, preventing cilia from beating
2) Mucus isn’t lubricated, so forms plaque on ciliated epithelium
3) Bacteria colonise newly-formed anaerobic environment

54
Q

How can antimicrobial function be impaired in CF airways?
1)
2)
3)

A

1) Defective CFTR transport of bicarbonate, pH regulation
2) Inhibits correct function of immune system in airways
3) CFTR might be involved in binding microbes, drawing them into epithelial cells to be killed

55
Q

Which two important antioxidants does CFTR regulate?

A

Glutathione

Thiocyanate

56
Q

Result of impaired CFTR regulation of glutathione and thiocyanate

A

Glutathione and thiocyanate are important antioxidants

Increases oxidative stress in airways, leading to inflammation

57
Q

How is an anaerobic environment formed in CF airways?
1)
2)

A

1) Mucus plaque formed

2) Ciliated epithelium uses more oxygen, as has to work harder

58
Q

Bacterium which likes to grow in anaerobic parts of CF lungs

A

Pseudomonas aeruginosa

59
Q

Which type of inflammation is predominant in CF?

A

Neutrophilic inflammation

60
Q

Lung defence against neutrophil-released proteaes

A

Antiproteases

Overwhelmed with large neutrophil response in CF

61
Q 
Cause of lung inflammation in CF
1)
2)
3)
4)
5)
A

1) Bacteria grow in anaerobic environments, byproducts include oxygen radicals
2) Oxygen radicals breathed in normally
3) Neutrophils release radical oxygen species, might be overactive in CF
4) Accumulation of abnormally-folded CFTR in cells can lead to apoptosis
5) Abnormal intracellular processes of epithelial cells lead to cell stress, release of cytokines

62
Q

What is ceramide?

A

A secondary messenger, a breakdown product of sphingomyelin, which is found in cell membranes, endosomal walls

Excess ceramide leads to apoptosis

63
Q

What is bronchiectasis?

A

Dilation of bronchi

64
Q

What is dilation of bronchi called?

A

Bronchiectasis

65
Q

Common pathogen in children with CF

A

Staph aureus

66
Q

Common pathogens in adults with CF

A

Pseudomonas
Burkholderia cepacia
Stenotrophomonas maltophillia

67
Q

Rising microbe infecting CF lungs

A

Mycobacteria abcessis

Resistant to many drugs

68
Q

What does 100% lung function mean?

A

Mean lung function for a population

69
Q

Normal range of lung functions

A

80% - 120% lung function

70
Q

When does CF lung function begin to deteriorate?

A

Around teenage years

71
Q

What is a breakdown product of sphingomyelin, involved in apoptosis?

A

Ceramide

72
Q

What information foes FVC give?

A

Total lung capacity

Doesn’t give information about narrowing of airways

73
Q

Proportion of CF mortality and morbidity caused by lung disease

A

90%

74
Q

Airway surface liquid volume regulation

A

1) When ASL levels are too high - ENaC actively pumps Na+ into cell. Cl- follows passively.
2) When ASL levels are too low - CFTR inhibits ENaC. Cl- pumped out of cell.

Molecule to Malady (35 decks)

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