Development aspects of lung disease Flashcards

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
Q

How does tracheomalacia present?

A

Barking cough
Recurrent croup
Breathless on exertion
Stridor/wheeze

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26
Q

What is the management of tracheomalacia?

A

Usually no management, child will grow out of it as airways grow and firm up
Physio and antibiotics
Natural history resolution with time

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27
Q

What is a tracheooesophageal fistula?

A

Abnormal connection between trachea and oesophagus

28
Q

What are tracheooesophageal fistulas associated with?

A

Oesophageal atresia
Genetic conditions

29
Q

When are tracheooesophageal fistulas diagnosed?

A

Antenatally or post-natal

30
Q

What does tracheooesophageal fistula present as?

A

Chocking
Colour change
Cough with feeding
Unable to pass NG into stomach

31
Q

How is tracheooesophageal fistulas treated?

A

Surgical repair

32
Q

What are some complications of tracheooesophageal fistulas?

A

Tracheomalacia
Strictures
Leak
Reflux

33
Q

What is congenital pulmonary airway malformation?

A

Abnormal non-functioning lung tissue

34
Q

When can congenital pulmonary airway malformation be detected?

A

80% detected antenatally

35
Q

How can congenital pulmonary airway malformation occur?

A

Sporadically and many resolve in utero

36
Q

What are the management options for congenital pulmonary airway malformation?

A

Watchful waiting - conservative management if asymptomatic
Surgical intervention - if severe or symptomatic

37
Q

What is a possible risk of congenital pulmonary airway malformation?

A

Malignant changes but is uncommon

38
Q

What is a congenital diaphragmatic hernia (CDH)?

A

Where diaphragm doesn’t close properly during development

39
Q

When does the diaphragm develop?

A

Week 7 to Week 18

40
Q

How common is CDH?

A

1 in 2500 births

41
Q

What is the most common type of CDH?

A

Bochdalek (90%)

42
Q

What side of the diaphragm is most likely impacted?

A

Usually the left side more than the right
(if the right side then usually worse outcomes)

43
Q

When is CDH diagnosed?

A

Antenatally
Some later

44
Q

What are the complications of CDH?

A

Underdevelopment of the lungs leading to significant morbidity and mortality.

45
Q

What is the management options and prognosis of CDH?

A

Surgical repair
Prognosis depends on degree of lung hypoplasia

46
Q

What changes occur after birth (neonatal)?

A

Lungs inflate and fluid is absorbed

47
Q

What is transient tachypnoea?

A

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
Q

What is the prognosis of transient tachypnoea?

A

Improves/self resolves within 1-2 days

49
Q

What is infant respiratory distress syndrome and who does it occur in?

A

Due to a surfactant deficiency - alveolar collapse when no air is in them so makes inspiration much harder work

50
Q

What is the treatment for IRDS?

A

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
Q

What is Chronic lung disease?

A

Inflammation and infection due to the significant resp insult that occurs from being born premature.

52
Q

What is chronic lung disease associated with?

A

Being born preterm
Or when ongoing oxygen is required at term

53
Q

What are the complications of chronic lung disease?

A

Increased childhood respiratory morbidity
- Tendency to infection
- Increased O2 requirements
- Hospital admissions

Leads to chronic obstructive disorders in later life

54
Q

What are the 4 main contributing factors to lung function later in life?

A

Individual factors
Early life events
Environment and lifestyle
Allergic diseases

55
Q

What early life events can effect lung function later in life? (10)

A

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
Q

What environmental and lifestyle factors can effect lung function? (5)

A

Short/long term air pollution
Indoor second hand smoke exposure
Active smoking
Serum vitamin D concentration
BMI

57
Q

What are some allergic diseases that could affect lung function in later life? (4)

A

Asthma
Rhinitis
Aero/food allergen
Sensitization

58
Q

What individual factors affect lung function in later life? (3)

A

Age
Sex
Height

59
Q

What can a low lung function growth trajectory be attributed to? (5)

A

Genetics
Preterm birth
Early life environment exposures
LRTI
Childhood persistent asthma

60
Q

What can a potential lung function catch up be attributed to? (6)

A

Genetics and host factors
Exposure avoidance
Diet
Physical activity
Supplementation of beneficial factors
Prevention and/or treatment via precision medicine

61
Q

What can poor lung function lead to in later life?

A

Adult obstructive diseases (e.g. COPD)

62
Q

What can improve lung function? (4)

A

Diet
Exercise
Exposure to irritants
Medication

63
Q

What is remodelling of the airways?

A

Alteration of airway structure following external influence

64
Q

What external influence leads to remodelling?

A

Environmental exposures
Chronic disease of childhood
Infection

65
Q

What is the effect of airway remodelling on the airways? (7)

A

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
Q

What are the signs of chronic inflammation?

A

Increased bronchial responsiveness
Increased mucus secretion
Airway oedema
Airway narrowing