Lecture 10 -airways In Children

Question 1

Why is ling development important?

Answer 1

For aerobic cellular respiration

Question 2

What are the goals of lung development?

Answer 2

Large Surface area, vascular system, prevent alveolar collapse and host defence

Question 3

What happens to the lungs 4-7 weeks in the embryonic stage?

Answer 3

Right and left lung bud formation, branching morphogenesis and visceral and parietal pleura formation

Question 4

What happens for lung development 5-17 weeks in the psuedoglandular stage?

Answer 4

Smooth muscle and cartilage formation, development of mucus glands and blood vessel development

Question 5

What % of births are pre term?

Answer 5

7%

Question 6

What is a key problem for babies born preterm?

Answer 6

They wont have the surfactant present in the type 2 alveolar cells

Question 7

What is a significant risk in pre term babies?

Answer 7

Lung disease - the highest risk is at 24-28 weeks, this is just as the airways juxtapose with blood vessels

Question 8

What diseases are you more likely to have if you were born early?

Answer 8

Asthma and COPD

Question 9

What is used for cystic fibrosis?

Answer 9

Modulators - CFTR channel proteins modulators

Question 10

What is the most superior CF drug?

Answer 10

Kaftrio

Question 11

What happens 16-26 weeks in lung development canalicular stage?

Answer 11

Increase in airway size, widening of epithelial cells, early surfactant product by type 2 cells

Question 12

What is moderate to late preterm?

Answer 12

Around 32 to 37 weeks

Question 13

What can happen for lung development in 26-36 weeks in the saccular stage?

Answer 13

Collagen and elastic fibre deposition, end of branching morphogenesis

Question 14

What is extreme preterm?

Answer 14

Less than 28 weeks

Question 15

What is very preterm?

Answer 15

28 to 32 weeks

Question 16

What are the effects of prematurity on the lungs?

Answer 16

Risk of chronic lung disease, the highest risk is 24-28 weeks gestation - as the airways juxtapose with blood vessels

Question 17

What is angiogenesis in chronic lung disease?

Answer 17

Administers surfactant to preterm babies, helps with biochemical and functional issues

Question 18

What is the control of breathing like in children?

Answer 18

Immature - decrease respiratory rate causing apnoea

Question 19

What is the pattern of breathing in children?

Answer 19

It can be irregular and periodic

Question 20

What are the airway sizes like in children?

Answer 20

Small airways so there is a higher resistance to airflow

Question 21

What is the airway compliance and respiratory muscles like in children?

Answer 21

Airway compliance - high and respiratory muscles are inefficient (diaphragms do most of the work)

Question 22

What are the respiratory rates like in children?

Answer 22

Raised respiratory rate and a lower tidal volume

Question 23

What is the total lung capacity and functional residual capacity like in children?

Answer 23

Low - children are more vulnerable to lower rapid drops in oxygen

Question 24

How do you measure lung physiology in children?

Answer 24

Pulse oximetry

Question 25

What does saturation =?

Answer 25

Percentage of available Hb saturated with O2

Question 26

What does Hb absorb?

Answer 26

Different wavelengths depending on degree of oxygenation

Question 27

How is CO2 measured in children?

Answer 27

A small adhesive patch is applied to the skin, it warms the skin to allow CO2 to diffuse

Question 28

What does spirometry measure?

Answer 28

VC, FEV1,FVC

Question 29

What is CF?

Answer 29

It is an autosomal recessive disease, defects in the CFTR gene

Question 30

What happens in the CF airways?

Answer 30

Abnormal mucociliary clearance, ongoing infection and progressive inflammation

Question 31

How is FEV1 a key parameter of lung function testing?

Answer 31

It correlates best with airway all thickness and mucus plugging which are features of large airway obstruction and occur later in disease

Question 32

What are the limitations of FEV1?

Answer 32

Doesn’t pick up the damage to the smaller airways that occurs early in CF

Question 33

How early can lung damage start?

Answer 33

As early as 3 months - changes on lung CTs in 1st year of life

Question 34

What is a useful tool for very young children?

Answer 34

Lung clearance index

Question 35

What is an advantage in lung clearance index (LCI)?

Answer 35

Can be used in young children

Question 36

What is an example of a CF potentiator?

Answer 36

Ivacaftor

Question 37

What is an example of CF corrector?

Answer 37

Lumacaftor - used to correct CFTR protein production