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M2 Renal/Respiratory > Development of the Lung > Flashcards

Development of the Lung Flashcards

1
Q

What tissues of the respiratory tract arise the endoderm? What tissues arise from the (splanchnic) mesoderm?

A

Endoderm: epithelial linings of the larynx, trachea, bronchi, and lungs Mesoderm Cartilage, Smooth Muscle, Elastic and Fibrous Connective tissue

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2
Q

What is the laryngotracheal tube? What structures does it turn into?

A

A combined tube that will turn into the esophagus and the trachea when separated by the tracheoesophageal septum

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3
Q

What is the first tracheal division? How many divisions are there? What is the second tracheal division? How many divisions are there?

A

Primary Bronchi; 1 on the left, 1 on the right Lobar Bronchi; 3 on the right, 2 on the left

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4
Q

What can occur from abnormal or insufficient development of the tracheoesophageal septum?

A

tracheoesophageal fistulas various types, all have weird connection between stomach, trachea, and esophagus

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5
Q

How are tracheoesophageal defects evaluated?

A

X-ray (look for gas in bowels) Contrast study Bronchoscopy

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6
Q

What are some anomalies associated with abnormal tracheoesophageal septum formation? Which TE septum abnormalities are most likely to have associated abnormalities?

A

Cardiac abnormalities; renal agenesis, dysplasia, or horseshoe; hemi-vertebrae; intestinal atresia, malformation Most common with esophageal atresia and least common with transesophageal fistulas.

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7
Q

What is the biggest determiner of survival for patients with TEF disorders?

A

Birth weight (and presence of cardiac defect)

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8
Q

What occurs during the pseudoglandular phase? When does this take place?

A

Airway branching continues, capillaries begin to form week 6-16

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9
Q

What occurs during the canalicular phase? When does this take place?

A

Capillary density increases, pneumocytes differentiate week 16-26 (The end of this phase is the beginning of extrauterine viability)

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10
Q

What occurs during the terminal sac phase? When does this take place?

A

Distal airways dilate, terminal sacs form, elastic fibers form, epithelium thins week 26-Birth

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11
Q

What is Congenital Cystic Adenomatoid Malformation (CCAM)?

A

Overgrowth of bronchial tissue compared to surrounding mesenchyme; this limits the lung development as the CCAM will never be functional lung

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12
Q

What are the three classifications of CCAM?

A

Type I- large macroscopic cysts Type II- small macroscopic cysts Type III-microscopic cysts

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13
Q

What are potential symptoms of CCAM?

A
  • Respiratory distress
  • Recurrent lung infections (pneumonia w/ abcess formation is a major concern)
  • pulmonary hypoplasia
  • pulmonary hypertension
  • mediastinum distortion
  • imparied venous returns
  • hydrops fetalis
  • Asymptomatic (ultrasound abnormalities)
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14
Q
  1. How much of the lung is affected in CCAM?
  2. How is CCAM treated?
A
  1. Usually affects only a single lobe
  2. Treatment:
    • Intubation to improve hypoxemia
    • Thoracotomy & Lobectomy
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15
Q

What is hydrops fetalis / prenatal hydrops?

How is prenatal hydrops treated?

A

Prenatal hydrops is edema within at least two compartments in a fetus.

Options:

  • Thoracocentesis
  • Thoracoamniotic shunt
  • Fetal lobectomy
  • Induce delivery & perform resection
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16
Q

Why is it recommended that CCAMs are almost always resected within the first 3-6 months of life (3 reasons)?

A
  • CCAMs can cause recurrent infections; resection of symptomatic lesions are more difficult
  • There is always diagnostic uncertainty before a pathologic specimen is available
  • Cancers can develop in CCAMs at as little as 1 year of age
17
Q
  1. When is the alveolar phase of lung development?
  2. What occurs during this phase?
  3. When do most alveoli form?
A
  1. 32 weeks - 8 years
  2. Alveolar formation occurs via subdivision of terminal sacs aka septation. The alveolar septa (walls) become thinner.
  3. Most alveoli form after birth
18
Q
  • Are phases of lung development distinct or overlapping?
  • What is the direction of lung development?
  • When does functional lung tissue form in comparison to funcitonal tissues in many other organs?
A
  • Overlapping
  • Proximal to distal (aka centrifugal)
  • Functional lung tissue develops relatively late (we don’t use ‘em much before we’re born!)
19
Q

The prenatal lung contains fluid. What happens when the prenatal lung produces an insufficient amount of fluid? Why?

A

Hypoplastic Lung

The fetus has shallow and episodic respiratory movements during development wherein the fluid helps to stretch the lungs out. Less fluid = less stretching = small lungs.

20
Q
  1. What is wet lung?
  2. How does it present? When?
  3. How is it treated?
A
  1. Wet lung is a problem with fluid removal from the lungs after birth.
  2. Transient tacypnea. Presents immediatelly or within hours of birth, lasts a few hours to two days
  3. Typically a transient condition. Supportive care if needed.
21
Q
  1. What produces pulmonary surfactant?
  2. When does this production begin?
  3. Why is this important in terms of preterm babies?
A
  1. Type II pneumocytes
  2. Begins toward end of the canalicular period, with significant increase in production at 35 weeks
  3. Prior to 35 weeks, usually insufficient surfactant to support extrauterine life
22
Q

What are the three major components of surfactant?

A
  1. Phospholipid
    • Dipalmitoylphosphatidylcholine
    • Dipalmitoylphosphatidylglycerol
  2. Protein
  3. Antioxidant
23
Q

Name 7 clinical signs of respiratory distress.

A
  1. Rapid breathing (>60/min in infant)
  2. Flaring nostrils
  3. Chest retractions
  4. Expiratory grunt
  5. Often cyanotic
  6. Tachycardia
  7. Anxious expression
24
Q
  1. In what context is Respiratory Distress Syndrome typically seen?
  2. What is its other name?
  3. What is the central problem in RDS?
  4. What pathologic changes to the lungs occur in RDS?
A
  1. Usually premature with immature lungs
  2. aka Hyaline membrane disease
  3. inability to keep alveoli filled with air
  4. Pathologic changes:
    • Poor lung tissue compliance
    • Alveolar collapse causes atelectasias
    • Engorgement of pumonary capillaries
    • Formation of Hyaline Membranes
25
Q
  1. What is a hyaline membrane is why is it bad?
  2. What causes the formation of a hyaline membrane?
  3. What is a hyaline membrane composed of?
  4. How does it appear on light microscopy?
A
  1. Waxy appearing layers of membrane that line collapsed alveoli in RDS. The presence of the membrane fills the alveoli and prevents gas exchange, exacerbating problems of RDS.
  2. Destruction of Type I pneumocytes → breakdown of the air/capillary barrier → leak of serum into the alveoli
  3. (Wiki) Fibrin, cellular derbris, RBCs, and some inflammatory cells
  4. Eosinophillic, somewhat amorphus mass lining the inside of an alveolus
26
Q
  1. What are the odds of an infant having RDS if they are born at:
    • <28 weeks?
    • 28-34 weeks?
    • >34 weeks?
  2. Does RDS show a gender bias?
  3. Name 4 maternal factors that increase the odds of an infant being born with RDS.
A
  1. Odds of RDS:
    • 28 weeks = 100%
    • 28-34 weeks = 33%
    • >34 weeks = 5%
  2. Yes - male predominance
  3. Maternal factors:
    • Perinatal depression
    • Diabetes
    • C-section
    • Multiple birth
27
Q

What x-ray findings are seen in RDS?

What lab findings are found in RDS?

A

X-ray:

  • Low lung volumes
  • Diffuse atelectasias (partial lung collapses)
    • “Ground glass opacities”
  • Air bronchograms
    • the outlines of air-filled bronchi (dark) can be seen due to the opacification of the alveoli (gray/white), such as due to alveolar collapse and fluid accumulaiton
  • N.B. can be difficult to distinguish from pneumonia

Lab:

  • Moderate hypoxia
  • Respiratory acidosis
  • Delayed metabolic acidosis (not sure why)
28
Q

Name two possible ways of preventing RDS.

A
  1. Antenatal bethamethasone
    • Corticosteroid given to the pregnant mother expecting preterm delivery in order to bolster lung development
  2. Arrest of preterm labor
29
Q

Name five treatments for RDS.

A
  • Treatments:
    1. Supplemental O2
    2. Assisted ventilation
      • nCPAP
      • mechanical
    3. Exogenous surfactant replacement (aerosol)
    4. Fluid restriction
    5. Post-natal steroids
      • Use caution: reduces mortality but increases risk of cerebral palsy
30
Q
  1. How long does the severe phase of RDS last?
  2. What coincides with the start of recovery?
  3. What can severe RDS cases result in?
A
  1. Peak severity 1-3 days
  2. Diuresis (at ~72 hrs)
  3. Bronchopulmonary dysplasia
    • chronic lung disease
    • prolonged mechanical ventilation
    • sepsis
31
Q

Meconium Aspiration Syndrome

  1. What is meconium?
  2. How common is MAS?
  3. What problems does aspiration of meconium cause?
  4. How does MAS appear on x-ray?
  5. How is MAS treated?
A

Meconium Aspiration Syndrome

  1. Earliest stool of an infant, composed of what the infant ingests during its time in the uterus.
  2. 1.5-2% of term or post-term infants
  3. Problems:
    • Irritative to lungs
    • Obstructive to lungs
      • Causes significant respiratory distress
    • Medium for bacterial culture
  4. X-ray: Patchy atelectasias (collapses) or consolidations (fluid-filled areas)
  5. Treat the infant as for RDS. Consider antibiotics.
32
Q
  1. What ratio can indicate the amount of surfactant present within the lungs of a fetus?
  2. Where is the test sample drawn from?
  3. How is the sample developed?
  4. What tests results are normal? Abnormal?
A
  1. Ratio of lecithin : sphingomyelin
  2. Drawn from amniotic fluid (amniocentesis)
  3. Seperate the components using Thin-Layer Chromatography (TLC)
  4. If the L:S ratio is:
    • >2.0 = 98% chance OK
    • 1.5-1.9 = 50% chance RDS
    • <1.5 = 73% chance RDS
33
Q

Presence of what compound in the amniotic fluid is an even more accurate marker of lung development than the L:S ratio?

A

Phosphatidylglycerol (PG)

Amniocentesis → Thin Layer Chromatography

34
Q

Aside from fluid exiting the lungs and circulatory system changes, changes in what other system also help the infant to begin breathing?

A

Neural changes, notably sympathetic stimulation

35
Q

Name three functions of surfactant.

A
  1. Decrease surface tension within the alveoli
  2. Stabilize alveolar surface
  3. Decrease fluid transfer (between intraalveolar space and circulation?)

M2 Renal/Respiratory (30 decks)

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