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Embryology - Development of the Respiratory System
Cole 3/1 lecture - 1 hr
74
Medical
Undergraduate 4
02/23/2011

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Cards

Term
posterior
Definition
the foregut(esophagus) is posterior/anterior to the heart?
Term
B = early
A = late
Definition
which is early phase of development, which is late?

A)mechanism of bronchial branching and cytodifferentiation

B)positioning of lung primordium and primary lung bud formation
Term
TBX4 from T-box transcription factor gene family (beta?)
Definition
determines location of the outgrowth of the foregut, also induces formation, growth and continued differnetiation of the lungs
Term
retinoic acid
Definition
what's produced in increase by adjacent mesoderm in the early phase of lung development that is also important in limb patterning?
Term
endoderm surrounding the foregut
Definition
where are TBX4 genes expressed in the early phase of lung development?
Term
Retinoic acid
Definition
what is in accuatane and rogaine that is bad for fetuses in excess?
Term
week four, formation of the laryngeotracheal(respiratory) diverticulum
Definition
when does lung development begin, and what structure forms first?
Term
splanchnic mesoderm
Definition
outgrowth of the foregut(future esophagus) into the surrounding ____ _____ begins lung development
Term
laryngeal opening
Definition
what is the connection between the gut and respiratory tract?
Term
the laryngeal orifice, supporting cartilages of the larynx
Definition
what does the original opening (of the diverticulum)? become, what does the surrounding mesenchyme become?
Term
distally, the tracheoesophageal septum
Definition
which direction does the laryngeotracheal diverticulum grow? what separates it from the esophagus?
Term
tracheoesophageal fistula
Definition
most common malfmormation of the trachea
Term
tracheoesophageal fistula
Definition
abnormal communication between trachea and esophagus
Term
improper formation of tracheoesophageal septum
Definition
what causes tracheoesophageal fistula?
Term
a blind ending of the esophagus
Definition
what is esophageal atresia?
Term
EA with distal TEF

(followed by Isolated EA 8%, isolated TEF4%, EA with proximal TEF 1%, and then EA with double TEF1%)
Definition
what is the most common combo of esophageal atresia/ tracheoesopogeal fistula?
Term
polyhydramnios

assoc with CNS abnormalities & esophageal atresia (including TEF)

this happens because the baby normally swallows amniotic fluid in development and pees it back out, but babies with a blind esophagus(atresia)- cannot swallow, so that can cause fluid to buildup
Definition
term for high volume of amniotic fluid, what is it associated with?
Term
diagnosed: 1) baby presents with excessive drooling (Salivation) frequently accompanied by choking, coughing, and sneezing.

2)When fed the baby swallows normally, but will cough and struggle as the fluid returns through the nose and mouth

3) the infant may become cyanotic from laryngeospasm, and sto pbreathing as fluid spills from the blind pouch and is aspirated into the trachea and lungs
Definition
how are EA and TEF diagnosed?
Term
remove the chunk of esophagus with the fistula then rejoin the esophagus
Definition
how do you treat EA/TEF?
Term
type 1: supraglottic interarytenoid cleft
type 2: partial cricoid cleft
type 3: total cricoid cleft
type 4: laryngoesophageal cleft (tricky to remove)
Definition
what kind of clefts can you get in a laryngotrachealesophageal cleft abnormality?
Term
1)generalized hypOplasia (overall narrow)
2)funnel-like narrowing
3)segmental stenosis

baloon dilation and tracheal stents
Definition
what kinds of tracheal stenosis can you get? how is this treated?
Term
week 5
Definition
when do the lung buds divide into 2 bronchial buds?
Term
week 6, endoderm
Definition
when do the lobes begin development, and what tissue are they derrived from?
Term
visceral -the lungs grow into msplanchnic mesoderm
parietal = somatic mesoderm
Definition
what is visceral pleura derrived from?
what is parietal pleura derrived from?
Term
smooth muscle, nerves, blood vessels, and visceral pleura

endoderm = the respiratory epithelium
Definition
what does splanchnic mesoderm become in the lungs? what forms from endoderm?
Term
a single pulmonary vein from the posterior left atrium anasomoses with veins forming in mesoderm around developing bronchioles

(this is weird because they form separately the join together-says Cole)
Definition
what do the pulmonary veins start as?
Term
also surrounding mesenchyme
Definition
what regulates the pattern of branching of lung endoderm
Term
max surface area/gas exchange with min resistance
Definition
human lungs are optimized for maximum _____ and the pattern of segmentation is optimized for minimal _____
Term
3x
Definition
airway segment length is never ____ it's diameter
Term
diameter
Definition
increased alveolar area (more branches) increases resistance with decreased ____
Term
1) Embryonic
2) (psudo) glandular
3) canalicular
4) terminal sac
5) postnatal (alveolar)
Definition
List the stages of lung development in order
Term
initial formation of resp. diverticulum up to formation of segmental bronchi

lungs grow into pleural cavities

differentiation of pleura

(no gas exchange)
Definition
what happens in the embryonic stage?
Term
major formation and growth of duct systems withing bronchopulmonary segments

(no gas exchange)

-segmental to terminal bronchi

looks like a gland histologically

start of vasculogenesis
Definition
what happens in the (pesudo) glandular stage?
Term
vascular endothelial growth factor (VEGF)
Definition
induction of vasculogenesis is mediated by ____ which is expressed in the epithelium of terminal buds in the human fetal lung during late pseudoglandular phase
Term
formation of resp. bronchioles and terminal sacs (primitive alveoli)

vascularization increases

capillaries found in walls

-first chance of a viable fetus
Definition
what happens in the canalicular stage that is soooo imporatant?
Term
alveoli (terminal sacs) develop from resp. bronchioles epithelium lining alveoli differentiates into 2 cell types surfactant starts being made
Definition
what happens in the terminal sac phase?
Term
help form the blood-air barrier
Definition
what do type 1 pneumocytes do?
Term
produce surfactant which decreases surface tension and faciliates alveolar expansion
Definition
what do type 2 pneumocytes do?
Term
24-26 = ok
28 wk = good, likely going to live
Definition
how many weeks is there OK sufractant in the baby? GOOD surfactant?
Term
separation of pre-existing alveoli to form 90% of total alveoli a person will have

this happens until age 10
Definition
what happens in the postnatal(alveolar stage?
Term
at least 10%, up to 90% form after
Definition
what percent of alveoli form before birth?
Term
yes!
Definition
do lung development phases overlap?
Term
the dreaded panda cat!
Definition
what creature is pictured on slide 45?
Term
supernumery lobes
Definition
what are typically asymptomatic, relatively common, abnormalities that result from abnormal division and lobation?
Term
tracheal bronchus
Definition
characterized by the presence of the bronchus to the right upper lobe emerging directly from the trachea close to the carina (2% of cases)?
Term
ectopic lung lobes
Definition
believed to form from additional respiratory buds of the foregut, occur along the esophagus or trachea
Term
ectopic endodermal budding
tracheoesophogeal fistula
and ectopic Tbx4 expression
Definition
misexpression of FGF10 causes this
Term
less than 28 = 60%
less than 37 = 5%
Definition
what percent of respiratory distress syndrom cases occur in infants less than 28 weeks gestation? less than 37 (more than 28)?
Term
atelectasis
Definition
incomplete expansion or collapse of parts or a whole lung
Term
collapsed alveoli and alveolar ducts + respiratory bronchi are dilated
Definition
in infant respiratory distress syndrom the histo examination of the lungs has a cahracteristic appearance of ?
Term
collapsed alveoli and alveolar ducts + respiratory bronchi are dilated

-the lining of alveolar ducts is covered with frbrin rich hyaline membranes (damaged cells from collapsed alveoli)

within alveolar spaces, cellular debris, proteinaceous edema fluid and some red blood cells accumulate
Definition
in infant respiratory distress syndrom the histo examination of the lungs has a cahracteristic appearance of ?
Term
hyaline membrane disease
see alveoli filled with debris in a near uniform distribution

0get ground glass appearance on chest x-ray
Definition
what is the old name for infant respiratory distress syndrome? what do you see on the x-ray?
Term
(NOT true emphysema!!!)

alveoli walls are not destroyed


you get over-distention with air of one or more lobes of the lung caused by collapsed bronchi (the cartilage doesn't develop)

bad if bilateral, unilateral can live
Definition
what is congenital neonatal emphysema?
Term
bronchiectasias
Definition
abnormal destruction and dilation of the large airways
Term
congenital bronchial cysts filled with air or fluid

-airways are baggy and enlarged with collections of mucus in pockets which leads to an increased incidence of infection
Definition
what causes honeycomb appearance on radiograph?
Term
pulmonary agenesis
Definition
complete absence of lungs, bronchi and vasculature
Term
bronchial buds do not develop
Definition
what causes pulmonary agenesis?
Term
pulmonary hypoplasia
Definition
characterized poorly deveolped bronchial tree, can be partial or total
Term
pulmonary hypoplasia (also oligohydaminos)
Definition
what condition is bilateral renal agenesis associated with?
Term
pulmonary hypoplasia, it makes it hard for the fetus to practice breathing and inflate the lungs with fluid


can be due to renal issues because the baby cannot recycle the amniotic fluid it swallows (or urethra obstruction)
Definition
what does insufficient amniotic fluid and increased pressure on the fetal thorax cause?
Term
oligohydraminos (too little fluid)
Definition
associated with renal agenesis & obstructive uropathy the complication s of which can be pulmonary hypoplasia and limb defects
Term
pleuropericardial membranes, and the diaphragm
Definition
what membranes divide up the thoracic cavity from other stuff?
Term
the diaphragm(specifically the central tendon), rotates downward with the heart ~85% of diaphragm
Definition
what forms mostly from the septum transversum? where does it come from?
Term
phrenic nerve (C3-5)
Definition
what keeps the diaphragm alive and migrates down with pleurpericardial membranes?
Term
week 4
Definition
when in development does the diaphragm become innervated?
Term
week 8
Definition
when does the diaphragm descend to L1?
Term
part of the diaphragm
Definition
what does the dorsal messentary of the esophagus form?
Term
pleurperitoneal membranes
Definition
sheets of somatic mesoderm from dorsolateral body wall
Term
mesenchyme around the foregut (dorsal mesentary)
Definition
what do the cura develop from?
Term
somatic body wall mesoderm
Definition
what forms the peripheral portions of the diaphragm?
Term
septum transversum -central tendon
somatic mesoderm from body wall-pleuroperitoneal membranes
mesenychyme around the foregut(dorsal mesentery)-crura
somatic body wall mesoderm-peripheral portions
Definition
what structures form the diaphragm (all of them)?
Term
congenital diaphragmatic hernias, most common on left posterolateral side
Definition
what is caused by failure of pleuroperitoneal membranes to fuse with other components? where is this most common?
Term
unusually flat abdomen, breathlessness, cyanosis


(prevent lungs from developing/expanding because the gut is in the thorax)
Definition
what are the clinical findings in congential diaphragmatic hernias?
Term
they tie off the trachea (before birthing, they partially deliver the baby) so the lungs fill with fluid and are forced to inflate

baby birthed by C-section & they don't cut the unbillicus until the trachea is undone
Definition
what is done to fix congenital diaphragmatic hernias?
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