Respiratory Flashcards by Amelia Sybenga

Q

When is the the embryonal period in lung development and what is it?

A

26 days - 6 weeks

Development of proximal airways (R&L bronchi & 10 segmental bronchi)

Pulmonary arteries from from 6th arch at end of 6 weeks

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

When is the the pseudoglandular period in lung development and what is it?

A

6-16 weeks

Development of terminal bronchioles, division of lobules & acini; pseudostratified columnar epithelium

Bonus answer:

8 weeks: lymphatics into lung (week 8)

10-13 weeks: cartilage, SmM, cilia, goblet cells

15-16 weeks: tubular submucosal glands

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

When is the the acinar/cannalicular period in lung development and what is it?

A

16-28 weeks

Vascularizaiton w/growth of cannaliculi (respiratory bronchioles) and alveolar ducts (eventually alveolar saccules) from terminal bronchioles

Bonus Answer:

Type II alveolar cells –> surfactant synthesis

Type I alveolar cells differentiate from Type II

Capillaries proliferate under Type I cells

Cartilage extension to distal bronchi (week 24)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

When is the the saccular period in lung development and what is it?

A

28-34 weeks

Subdivision saccules, respiratory bronchioli, interstitial tissue shrinks, fibroblasts differentiate, production of collagen, ECM & elastin, Double capillary network, early gas exchange (32 weeks)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

When is the the alveolar period in lung development and what is it?

A

34 weeks-2 years post natal

Lymphatics extend through interlobular septae to pleura

Double to single capillary bed (air-blood barrier = 0.2 microns)

Induction of surfactant (from 32 weeks, maternal glucocort)

Type II pneumocytes increase

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Embryonal

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Pseudoglandular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Pseudoglandular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Pseudoglandular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Cannalicular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Saccular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Saccular

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What period of lung development is this?

A

Alveolar

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What signals induce surfactant production from Type II pneumocytes

A

Exercise, hyperinflation, steroids

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What gene is required for production/expression of SP-A, B, C and ABCA3

A

TTF-1

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What are the 5 primary lipoproteins comprising surfactant?

A

Large: SP-A & SP-D

Small: SP-B & SP-C

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

When is steroid administration recommended to prevent surfactant deficient IRDS in prematurity?

A

28-34 weeks

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Lung biopsy: what disease?

Study These Flashcards

A

Surfactant SP-B deficient

Q

Lung biopsy: what disease?

Study These Flashcards

A

Surfactant ABCA3 deficient

Q

Lung biopsy: neonate in ARDS

What is this?

Study These Flashcards

A

Early surfactant deficiency

Q

Lung biopsy: neonate in ARDS

What is this?

BQ: What is differential?

Study These Flashcards

A

Surfactant deficiency

DDx: congenital alveolar proteinosis

Q

What is the mechanism of congenital pulmonary alveolar proteinosis?

Study These Flashcards

A

Failure of surfactant degradation/clearance due to mutations in CSF2RA or CSF2RB (GC-CSF)

*can also be cause by receptor autoantibody

Q

What is choanal atresia?

What syndrome is associated with it?

Study These Flashcards

A

Occlusion of airway between nasal passage & nasopharynx.

Ass’d w/CHARGE syndrome

Q

What 2 syndromes are frequently associated with laryngomalacia?

Study These Flashcards

A

Pierre Robin, cri-du-chat

What syndrome is associated with laryngeal stenosis/atresia?

VATER/VACTERL

What is Mounier-Kuhn syndrome?

Tracheobronchomegaly with chronic lung infections

What is Kartagener syndrome?

Immotile cilia syndrome Associated w/situs inversus, otitis/rhinitis, bronchiectasis & male infertility EM shows "radial spoke" defect

What is this?

Williams-Campbell Syndrome: congenital bronchiectasis 2\* deficiency of cartilage distal to main bronchus

What are the characteristics of CPAM Type 0?

Absence of alveoli/pulmonary alveolar agenesis/hypoplasia Tracheobronchial origin; lethal

What are the characteristics of CPAM Type 1?

Most common, bronchial origin Single or multiloculated cyst ~10cm lined by bronchial epithelium; mucinous metaplasia --\> mucinous adenocarcinoma; KRAS mutations

What are the characteristics of CPAM Type II

Small-medium cysts up to 2cm; bronchial origin; 10-15% Bronchiolar type structures w/rhabdomyomatous dysplasia; looks like extralobar sequestration

What are the characteristics of CPAM Type 3?

Solid appearing with small cysts ~0.2 cm, alveolar duct origin Large, poor px

What is risk to neonate on ventilation without surfactant supplementation?

Hyalin membrane disease (DAD/barotrauma)

What is the most common respiratory pathogen of childhood?

RSV

What are the histologic features of a respiratory adenovirus infection?

Necrotizing bronchiolitis & smudged intranuclear inclusions in bronchiolar & alveolar epithelial cells

What are the associations with congenital diaphragmatic hernia?

Trisomy 18 & 21, TOF, extralobar sequestrations, ectopia cordis, tracheoesophageal fistula

What genetic alteration is associated with alveolar capillary dysplasia with misalignment of pulmonary veins?

Mutations resulting in *FOXF1* _deletions_/silencing (Chr 16)

What is diaphragmatic eventration and what are most common syndromic associations w/congenital diaphragmatic hernia (CDH)?

1. Replacement of all or part of diaphragm with fibroelastic tissue - functions as a type of CDH--\> pulmonary hypoplasia sequence 2. Aneuploidys, Pallister-Killian (tetrasomy 12q) syndrome, microduplications/deletions

4 mo infant presents w/tachypnia, hypoxia and failure to thrive. Lung biopsy shows this (serotonin stain):

Neuroendocrine hyperplasia of infancy

What is the most common malignant tumor of the lung in children?

Metastases

A female child presents with chrondromatous hamartoma of the lung. What other syndrome/ other tumors might you look for in this patient?

Carney triad: Extra-adrenal paraganglioma GIST

What is this lung lesion? Stains?

Sclerosing pneumocytoma (hemangioma) TTF-1, EMA

What is the most common primary lung malignancy in children?

Bronchial carcinoid

What is the translocation in NUT midline carcinoma?

t(15;19)(q14;p13.1)