Developmental Aspects of Lung Disease Flashcards

1
Q

What are the 5 stages of structural lung development and when do they occur?

A
  1. embryonic 3-8 weeks
  2. Pseudo-glandular 5-17 weeks
  3. Canalicular 16-26 weeks
  4. Saccular 24-38 weeks
  5. 24-38 weeks
  6. Alveolar 36 weeks-2/3 years
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2
Q

What embryonic structure does the lung bud originate from?

A

Foetal foregut

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3
Q

What embryonic structure do the blood vessels and connective tissues surrounding he lungs originate from?

A

Mesoderm

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4
Q

In what stage of foetal lung development does rapid branching occur?

A

Pseudoglandular

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5
Q

In what stage of embryonic lung development do specialised cells such as cilia and mucous glands develop?

A

Pseudoglandular

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6
Q

In which stage of embryonic lung development do terminal bronchioles, alveolar sacs and capillary blood vessels form gas exchange units.

A

Canalicular

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7
Q

In which stage of embryonic lung development do type 1 and type 2 pneumocytes appear.

A

Canalicular

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8
Q

What happens in the saccular phase of lung development?

A

alveolar sacs grow in size and become well formed

More surfactant is produced in this stage & the bronchioles will continue to elongate.

The interstitial tissue between the sacs begins to reduce and the alveolar walls become thinner

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9
Q

During which stage are the lungs able to independently sustain breathing?

A

Alveolar phase

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10
Q

How many alveolar air sacs does an infant have at birth?

A

20-60 million

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11
Q

How many alveolar air sacs does a child have by the time the they reach school age?

A

200-300 million

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12
Q

What is Laryngomalacia?

A

Congenital abnormal collapse of the larynx or voice box

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13
Q

How does Laryngomalacia present?

A

stridor or inspiratory squeak which worse when the baby is active, emotional or feeding

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14
Q

How is Laryngomalacia managed?

A

It generally improves so watch and wait. Intervention is only when the baby is struggling to feed, breath or is failing to thrive

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15
Q

What is tracheomalacia?

A

dynamic abnormal collapse of tracheal walls

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16
Q

How does tracheomalacia present?

A
  • Barking cough
  • Recurrent “croup”
  • Breathless on exertion
  • Stridor/wheeze
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17
Q

What are the common causes of tracheomalacia?

A

genetic conditions

external compression (e.g. from a tumour)

18
Q

How is tracheomalacia managed?

A

physio and antibiotics when unwell (the condition will remove with time)

19
Q

What is a Tracheo-oesphageal fistula

A

an abnormal connection between the trachea and the oesophagus

20
Q

How does a Tracheo-oesphageal fistula present in a newborn?

A

A choking colour change (due to the inability to clear secretions), coughing with feeding and an inability to pass a nasogastric tube into the stomach.

21
Q

How is a Tracheo-oesphageal fistula managed?

A

It must be surgically repaired

22
Q

What is a Congenital Pulmonary Airway Malformation?

A

abnormal non-functioning lung tissue

23
Q

What % of Congenital Pulmonary Airway Malformation cases are detected antenatally?

A

80%

24
Q

How should Congenital Pulmonary Airway Malformation be managed?

A

Conservative management should be applied if CPAM is asymptomatic

Surgical intervention may be required

25
Q

What is the risk associated with Congenital Pulmonary Airway Malformation?

A

malignant change

26
Q

At what stage of gestation does the diaphragm begin to form?

A

7 weeks gestation

27
Q

At what stage of gestation does the diaphragm close?

A

by 18 weeks

28
Q

How many live births are affected by congenital diaphragmatic hernia?

A

1 in 2500 births

29
Q

What is the most common type of congenital diaphragmatic hernia?

A

Bochdalek

30
Q

What side of the diaphragm is the most common for a congenital diaphragmatic hernia?

A

Left side

31
Q

Is a left or right sided congenital diaphragmatic hernia associated with worse outcomes?

A

Right sided is associated with worse outcomes

32
Q

What is the prognosis of congenital diaphragmatic hernia associated with?

A

Prognosis depends on degree of lung hypoplasia

33
Q

How is congenital diaphragmatic hernia managed?

A

surgically

34
Q

What is Transient Tachypnoea of the Newborn?

A

The amniotic fluid in the lungs does not move off as fast as it should have

35
Q

What is Transient Tachypnoea of the Newborn associated with and why?

A

Caesarian section.

This occurs because the child has not passed through the birth canal and the first breath was not vigorous.

36
Q

How is Transient Tachypnoea of the Newborn managed?

A

generally improves within 1-2 days but occasionally will require a degree of respiratory support or oxygen

37
Q

What causes respiratory distress syndrome?

A

surfactant deficiency

38
Q

What does respiratory distress syndrome look like on an x-ray?

A

Ground glass appearance

39
Q

What are the three things that should be given in a case of respiratory distress syndrome?

A
  • Antenatal steroids
  • Surfactant replacement
  • Appropriate ventilation and nutrition
40
Q

What is chronic lung disease?

A

Chronic Lung Disease is associated with prematurity where ongoing oxygen support is required at term

41
Q

What is lung remodelling?

A

Remodelling is the alteration of airway structure following external influences

42
Q

Name three things that can cause remodelling

A
  • Environmental exposures
  • Chronic diseases of childhood
  • Infection