Sept29 M1-Developmental anomalies of the respiratory system Flashcards

(38 cards)

1
Q

what allows first bifurcation of two lung buds

A

caudal endoderm of trachea bifurcates in mesenchyme (which will be pleura)

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2
Q

name of mesenchyme surrounding all endoderm of resp and what will provide

A

splanchnic mesenchyme, will form CT and cartilage around trachea + smooth muscle

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3
Q

how trachea develops (2)

A

Overgrowth of cells in lumen

Amniotic fluid flows in there for recanalization and budding

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4
Q

characteristic of lungs budding

A

asymmetric. right lung more aligned with trachea

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5
Q

what azygos vein normally does and what can do (abnormal) when lung develops

A

supposed to go over right bronchus. sometimes indents in right upper lobe (still lsurrounded by parietal pleura)

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6
Q

name of result when azygos vein goes through right upper lobe

A

azygous lobe

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7
Q

how many times one lung bud branches

A

22

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8
Q

how early diaphragm movements contribute to lung formation

A

help amniotic fluid circulation

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9
Q

initial lung buds: mesenchyme in contact, something in particular

A

fibroblast and mesenchyme have receptors for GFs for proliferation at these tips

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10
Q

lung epithelium description at embryo. when branching starts

A

pseudostratified

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11
Q

how lung epithelium changes with budding

A

becomes thinner and eventually cuboidal

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12
Q

lung epithelium closer to mesenchyme: description + what phase

A

end budding phase: squamous and in direct contact with vessels and capillaries in mesenchyme

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13
Q

what proliferation of mesenchyme at initial budding alloes

A

further bifurcations and thinning of mesenchyme, more ducts

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14
Q

2 factors expressed at endodermal tips and role

A

Fibroblast GF and Shh (sonic hedgehog) : proliferation

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15
Q

when production of blood vessels stimulated in lung and how

A

at cuboidal epith phase. VEGF produced and angioblasts (precursors) stimulated

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16
Q

time of end of lung development where gas exchange capability is possible

17
Q

terminal sac phase (26 weeks to birth) what happens in lungs

A
  • ducts bud into resp bronchioles in mesenchyme + angiogenesis
  • endoderm: pneumocyte type 1 (surface) and type 2
18
Q

post alveolar phase (lung dev after birth) what happens

A

terminal sacs further divided by secondary mesenchymal septa (alveoli form)
from 70M at birht to 300M at 8 years

19
Q

first genetic defect possible in lung formation

A

defective gene expression during endodermal-mesenchyme interaction (trachea sep from esophagus + budding)

20
Q

genetic defect possible in lung formation at budding step + consequence (2)

A

defective shh expression. affects tracheo-esophageal septum dev + lung branching

21
Q

ultimate consequence of shh defect

A

lung hypoplasia

22
Q

defective apoptosis consequence on lung dev (gut too)

A

no recanalization of gut and trachea (atresia)

23
Q

how gut atresia can affect lung dev

A

leads to abnormal amniotic fluid turnover, affects branching of lung

24
Q

cause of atresia

A

failure of normal mesenchyme-endodermal interaction that would facilitate apoptosis of lumen endodermal cells

25
atresia excess consequence
endodermal cells degenerate : segment of agenesis
26
how BP affects lung dev
defective vasculature so hypoplasia and maybe atresia of gut: bad amniotic fluid circul to lung
27
consequence of incomplete apoptosis on gut wall
mesenchyme can project in and cause doubling of gut tube
28
mesenchyme overgrowth consequence
smooth muscle hypertrophy: stenosis of lumen
29
stenosis def + 2 causes
narrowing of gut lumen. reduced endodermal cell prolif or excessive mesenchyme prolif
30
esophageal atresia def + consequence
top and bottom of esophagus not linked: vomiting
31
esophago-tracheal (or TO) fistula def +consequence
esophagus links to trachea. most food goes in bronchi
32
trachea atresia def
top and bottom of trachea not linked
33
OT fistula + atresia of esophagus consequence
regurgitation and trachea linked to bottom of esophagus so air in stomach
34
one fatal error in esophagus and tracheal dev
failure of TO septum to separate (fatal)
35
polyhydramnios def
excess amniotic fluid
36
polyhydramnios one cause
gut atresia
37
oligohydramnios def
deficit of amniotic fluid
38
give 1 cause of oligohydramnios + consequence + syndrome name
kidney agenesis (essential to produce amniotic fluid) - hypoplasia of the lung - death at birth - Potters syndrome