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Pediatric Pathology > Respiratory > Flashcards

Respiratory Flashcards

1
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

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2
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

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3
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)

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4
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)

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5
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

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6
Q

What period of lung development is this?

A

Embryonal

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7
Q

What period of lung development is this?

A

Pseudoglandular

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8
Q

What period of lung development is this?

A

Pseudoglandular

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9
Q

What period of lung development is this?

A

Pseudoglandular

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10
Q

What period of lung development is this?

A

Cannalicular

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11
Q

What period of lung development is this?

A

Saccular

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12
Q

What period of lung development is this?

A

Saccular

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13
Q

What period of lung development is this?

A

Alveolar

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14
Q

What signals induce surfactant production from Type II pneumocytes

A

Exercise, hyperinflation, steroids

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15
Q

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

A

TTF-1

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16
Q

What are the 5 primary lipoproteins comprising surfactant?

A

Large: SP-A & SP-D

Small: SP-B & SP-C

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17
Q

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

A

28-34 weeks

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18
Q

Lung biopsy: what disease?

A

Surfactant SP-B deficient

19
Q

Lung biopsy: what disease?

A

Surfactant ABCA3 deficient

20
Q

Lung biopsy: neonate in ARDS

What is this?

A

Early surfactant deficiency

21
Q

Lung biopsy: neonate in ARDS

What is this?

BQ: What is differential?

A

Surfactant deficiency

DDx: congenital alveolar proteinosis

22
Q

What is the mechanism of congenital pulmonary alveolar proteinosis?

A

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

*can also be cause by receptor autoantibody

23
Q

What is choanal atresia?

What syndrome is associated with it?

A

Occlusion of airway between nasal passage & nasopharynx.

Ass’d w/CHARGE syndrome

24
Q

What 2 syndromes are frequently associated with laryngomalacia?

A

Pierre Robin, cri-du-chat

25
Q

What syndrome is associated with laryngeal stenosis/atresia?

A

VATER/VACTERL

26
Q

What is Mounier-Kuhn syndrome?

A

Tracheobronchomegaly with chronic lung infections

27
Q

What is Kartagener syndrome?

A

Immotile cilia syndrome

Associated w/situs inversus, otitis/rhinitis, bronchiectasis & male infertility

EM shows “radial spoke” defect

28
Q

What is this?

A

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

29
Q

What are the characteristics of CPAM Type 0?

A

Absence of alveoli/pulmonary alveolar agenesis/hypoplasia

Tracheobronchial origin; lethal

30
Q

What are the characteristics of CPAM Type 1?

A

Most common, bronchial origin

Single or multiloculated cyst ~10cm lined by bronchial epithelium; mucinous metaplasia –> mucinous adenocarcinoma; KRAS mutations

31
Q

What are the characteristics of CPAM Type II

A

Small-medium cysts up to 2cm; bronchial origin; 10-15%

Bronchiolar type structures w/rhabdomyomatous dysplasia; looks like extralobar sequestration

32
Q

What are the characteristics of CPAM Type 3?

A

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

Large, poor px

33
Q

What is risk to neonate on ventilation without surfactant supplementation?

A

Hyalin membrane disease (DAD/barotrauma)

34
Q

What is the most common respiratory pathogen of childhood?

A

RSV

35
Q

What are the histologic features of a respiratory adenovirus infection?

A

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

36
Q

What are the associations with congenital diaphragmatic hernia?

A

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

37
Q

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

A

Mutations resulting in FOXF1 deletions/silencing

(Chr 16)

38
Q

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

A
  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
39
Q

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

A

Neuroendocrine hyperplasia of infancy

40
Q

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

A

Metastases

41
Q

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

A

Carney triad:

Extra-adrenal paraganglioma

GIST

42
Q

What is this lung lesion? Stains?

A

Sclerosing pneumocytoma (hemangioma)

TTF-1, EMA

43
Q

What is the most common primary lung malignancy in children?

A

Bronchial carcinoid

44
Q

What is the translocation in NUT midline carcinoma?

A

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

Pediatric Pathology (37 decks)

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