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Development aspects of lung disease Flashcards

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1
Q

What are the 5 stages of lung development and at what gestational age do they occur?

A

Embryonic = 3-8 weeks
Pseudoglandular = 5-17 weeks
Canalicular = 16-26 weeks
Saccular = 24-38 weeks
Alveolar = 36 weeks - 2/3 years

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2
Q

What happens during embryonic stage?

A

Lung bud develops off of foregut
- Respiratory diverticulum from foregut
- Lung bus then divides into lobar buds
- Cells derived from endoderm

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3
Q

What happens during pseudoglandular phase?

A

Rapid branching of airways
- Eventually 16-25 primitive segmental bronchi
Development of specialised cells such as cilia and mucous glands.

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4
Q

What happens during canalicular stage?

A

Lung develop distal architecture
- Terminal bronchioles, alveolar sacs, capillaries, blood vessels all from gas exchange units
- Type 1 and 2 pneumocytes appear

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5
Q

When is the limit of viability of life?

A

24 weeks
At canicular stage

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6
Q

What do type 1 pneumocytes do?

A

Allow for gas exchange

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7
Q

What do type 2 pneumocytes do?

A

Produce and store surfactant which stops alveolar from collapsing

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8
Q

What happens during the saccular stage? (6)

A

Alveolar sacs grow and become well formed
More surfactant is produced
Bronchioles elongate
Interstitial tissue between sacs reduces
Alveolar walls become thinner, improving gas exchange

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9
Q

What happens in alveolar stage?

A

Lungs can independently sustain breathing without support
Cells well differentiated
Pulmonary vasculature is well developed and forming the final alveolar structure.

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10
Q

What happens with lung development post-natal?

A

Alveolar development continues
Prior to birth in healthy foetus 20-60 million alveolar air sacs growing to 200-300 million sacs at 3-8 years old.

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11
Q

What are congenital abnormalities?

A

Those present at birth

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12
Q

Give some examples of upper congenital abnormalities

A

Laryngomalacia
Tracheomalacia
Tracheooesophageal fistula

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13
Q

What are some lower congenital abnormalities?

A

Congenital pulmonary airway malformation (CPAM)
Congenital diaphragmatic hernia

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14
Q

When are congenital abnormalities picked up?

A

Antenatal screening
New-born
Childhood
Incidental

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15
Q

How is antenatal screening done?

A

Ultrasound
MRI

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16
Q

What are the signs of congenital abnormality in newborns?

A

Tachypnoea
Resp distress
Feeding issues

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17
Q

What are the signs of congenital abnormality in childhood?

A

Stridor/wheeze
Recurrent pneumonia
Cough
Feeding issue

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18
Q

What is laryngomalcia?

A

Dynamic abnormal collapse of the larynx due to floppiness

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19
Q

When is laryngomalacia picked up?

A

Seen in infants from 6 weeks onwards

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20
Q

How does laryngomalacia present?

A

Stridor
- Worse with feeding or when upset/excited

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21
Q

How does laryngomalacia progress?

A

Will improve within the first year as airways become bigger

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22
Q

When does laryngomalacia become a concern?

A

If affecting feeding or growth (weight gain)
OR causes apnoea

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23
Q

What is tracheomalacia?

A

A floppiness of the trachea

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24
Q

What can cause tracheomalacia?

A

Genetic conditions
External compression (e.g. vessels or tumour)

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25
How does tracheomalacia present?
Barking cough Recurrent croup Breathless on exertion Stridor/wheeze
26
What is the management of tracheomalacia?
Usually no management, child will grow out of it as airways grow and firm up Physio and antibiotics Natural history resolution with time
27
What is a tracheooesophageal fistula?
Abnormal connection between trachea and oesophagus
28
What are tracheooesophageal fistulas associated with?
Oesophageal atresia Genetic conditions
29
When are tracheooesophageal fistulas diagnosed?
Antenatally or post-natal
30
What does tracheooesophageal fistula present as?
Chocking Colour change Cough with feeding Unable to pass NG into stomach
31
How is tracheooesophageal fistulas treated?
Surgical repair
32
What are some complications of tracheooesophageal fistulas?
Tracheomalacia Strictures Leak Reflux
33
What is congenital pulmonary airway malformation?
Abnormal non-functioning lung tissue
34
When can congenital pulmonary airway malformation be detected?
80% detected antenatally
35
How can congenital pulmonary airway malformation occur?
Sporadically and many resolve in utero
36
What are the management options for congenital pulmonary airway malformation?
Watchful waiting - conservative management if asymptomatic Surgical intervention - if severe or symptomatic
37
What is a possible risk of congenital pulmonary airway malformation?
Malignant changes but is uncommon
38
What is a congenital diaphragmatic hernia (CDH)?
Where diaphragm doesn't close properly during development
39
When does the diaphragm develop?
Week 7 to Week 18
40
How common is CDH?
1 in 2500 births
41
What is the most common type of CDH?
Bochdalek (90%)
42
What side of the diaphragm is most likely impacted?
Usually the left side more than the right (if the right side then usually worse outcomes)
43
When is CDH diagnosed?
Antenatally Some later
44
What are the complications of CDH?
Underdevelopment of the lungs leading to significant morbidity and mortality.
45
What is the management options and prognosis of CDH?
Surgical repair Prognosis depends on degree of lung hypoplasia
46
What changes occur after birth (neonatal)?
Lungs inflate and fluid is absorbed
47
What is transient tachypnoea?
When there is a delay in clearing foetal lung fluid after birth - Usually child has vigorous first breath and this forces the fluid out
48
What is the prognosis of transient tachypnoea?
Improves/self resolves within 1-2 days
49
What is infant respiratory distress syndrome and who does it occur in?
Due to a surfactant deficiency - alveolar collapse when no air is in them so makes inspiration much harder work
50
What is the treatment for IRDS?
Antenatal steroids - Promote lung development prior to delivery if child is expected to be preterm Surfactant replacement - Given directly into the lungs via a tube Appropriate ventilation and nutrition - important for lung development
51
What is Chronic lung disease?
Inflammation and infection due to the significant resp insult that occurs from being born premature.
52
What is chronic lung disease associated with?
Being born preterm Or when ongoing oxygen is required at term
53
What are the complications of chronic lung disease?
Increased childhood respiratory morbidity - Tendency to infection - Increased O2 requirements - Hospital admissions Leads to chronic obstructive disorders in later life
54
What are the 4 main contributing factors to lung function later in life?
Individual factors Early life events Environment and lifestyle Allergic diseases
55
What early life events can effect lung function later in life? (10)
Parental atopy Parental education Maternal age at delivery Maternal smoking during pregnancy Second-hand smoke exposure at home Season of birth Birth weight Breastfeeding Peak weight/height velocity Lung infection
56
What environmental and lifestyle factors can effect lung function? (5)
Short/long term air pollution Indoor second hand smoke exposure Active smoking Serum vitamin D concentration BMI
57
What are some allergic diseases that could affect lung function in later life? (4)
Asthma Rhinitis Aero/food allergen Sensitization
58
What individual factors affect lung function in later life? (3)
Age Sex Height
59
What can a low lung function growth trajectory be attributed to? (5)
Genetics Preterm birth Early life environment exposures LRTI Childhood persistent asthma
60
What can a potential lung function catch up be attributed to? (6)
Genetics and host factors Exposure avoidance Diet Physical activity Supplementation of beneficial factors Prevention and/or treatment via precision medicine
61
What can poor lung function lead to in later life?
Adult obstructive diseases (e.g. COPD)
62
What can improve lung function? (4)
Diet Exercise Exposure to irritants Medication
63
What is remodelling of the airways?
Alteration of airway structure following external influence
64
What external influence leads to remodelling?
Environmental exposures Chronic disease of childhood Infection
65
What is the effect of airway remodelling on the airways? (7)
Narrowing of lumen Increased mucous plugging Changes to cell of the airway with tendency to increase mucous production Changes to the interstitial Hypertrophy of muscles Increase in number of inflammatory cells Changes to vasculature of the airway
66
What are the signs of chronic inflammation?
Increased bronchial responsiveness Increased mucus secretion Airway oedema Airway narrowing

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