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Neo Boards > Respiratory > Flashcards

Respiratory Flashcards

1
Q

What embryologic structure forms the respiratory tract?

A

Endoderm (foregut)
Ventral esophagus–> Lungs

Mesodermal mesenchyme–> branching

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2
Q

When does airway branching complete?

A

12-14 weeks

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3
Q

What part of the central great vessels forms the pulmonary vasculature?

A

Sixth aortic arch branches

Pulmonary arteries supply the intrapulmonary structures

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4
Q

What structures does the bronchial artery system supply?

A

Conducting airways
Visceral pleura
Connective tissue
Pulmonary arteries

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5
Q

Where are the preacinar arteries and how do they develop?

When is their development complete?

A

Next to the terminal bronchioles (non-respiratory)
Angiogenesis
16 weeks

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6
Q

Where are the intra-acinar arteries and how do they develop?

A

Next to the respiratory bronchioles and alveolar ducts, within alveolar walls
Vasculogenesis
Develop until 8-10 years

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7
Q

How does fetal vessel wall thickness change throughout gestation?

A

Fetal vessel wall thickness is increased compared to an adult
During the second half of gestation, wall thickness and vessel diameter remain proportional

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8
Q

What small pulmonary artery development occurs in the fetus?

A

Small pulmonary arteries:

  • Move along airways toward alveoli
  • Have an encircling medial smooth muscle cell layer->
  • layer later changes to incomplete muscularization in a spiral or helix->
  • smooth muscle layer disappears (non muscularized vessels)
  • -> vascular smooth muscle cells through preacinar arteries
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9
Q

What changes occur in the small pulmonary arteries in the near-term infant?

A

Half bronchiolar vessels are muscularized or partially muscularized

Vessels NEXT to alveoli are NON muscularized

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10
Q

How do small pulmonary arteries change in 4-6 week infants?

A

Medial smooth muscle layer involutes

Muscular wall thickness decreases

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11
Q

During what phase do bronchioles and alveoli increase?

A

Alveolar phase

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12
Q

When do alveoli develop?

A

Late gestation–> 3-8 years

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13
Q

How many alveoli does the term infant have?

A

50-150 million alveoli

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14
Q

How many alveoli does the adult have?

A

200-600 million

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15
Q

What factors delay alveolization?

A

Antenatal steroids
Supplemental oxygen
Nutritional deficiencies
Mechanical ventilation

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16
Q

What are the embryonic stages of lung development?

A 
Embryonic 
pseudoglingular 
canalicular 
saccular 
alveolar
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17
Q

When does embryonic lung development occur?

A

0-5 weeks

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18
Q

What two key structures develop during the embryonic phase of lung development?

A

Trachea

bronchi

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19
Q

What pulmonary anomalies develop during the embryonic phase of lung development?

A

Laryngeal cleft
Tracheal stenosis
Tracheoesophageal fistula

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20
Q

When does the pseudo glandular phase of lung development occur?

A

5 to 15 weeks

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21
Q

What key structures develop during the pseudoglanular phase of lung development?

A

Non-respiratory bronchioles

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22
Q

What airway anomalies occur during the pseudo-glandular phase of lung development?

A 
Branching abnormalities 
Bronchogenic cysts 
Congenital diaphragmatic hernia 
ongenital lobar emphysema 
Cystic adenomatoid malformation
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23
Q

When do the lungs begin to develop amniotic fluid?

A

During the pseudo-glandular phase

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24
Q

When does the canalicular phase of lung development occur?

A

15-25 weeks

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25
Q

What structures develop during the canalicular phase?

A

Respiratory bronchioles

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26
Q

What lung anomaly occurs during canalicular development?

A

Pulmonary hypoplasia

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27
Q

When during lung development do pneumocytes begin to differentiate from type 2 to type 1?

A

Canalicular phase, 15-25 weeks

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28
Q

When does the saccular phase of lung developing occur?

A

25-35 weeks

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29
Q

What structures develop during the saccular phase of lung development?

A

Alveolar ducts

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30
Q

What lung anomaly occurs during the saccular phase of lung development?

A

Pulmonary hypoplasia

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31
Q

When does the alveolar phase of lung development occur?

A

36+ weeks

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32
Q

What structures develop during the alveolar phase of lung development?

A

Alveoli

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33
Q 
What does
Each
Pulmonary part
Comes 
Through
Age
represent?
A 
Stages of lung development
Embryonic
Pseudo-glandular
Canalicular
Terminal sac (Saccular)
Alveolar
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34
Q

Describe type 1 pneumocytes

A 
Fried egg
Tight junctions
90% surface area
Smaller number
Gas exchange
Formed from type 2 pneumocytes
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35
Q

Describe type 2 pneumocytes

A 
Cuboid
10% of surface area
Greater number
Surfactant metabolism and secretion
Form type 1 cells
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36
Q

Fetal lung fluid production prior to term is equivalent to

A

Functional residual capacity

20 to 30 ml/kg

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37
Q

At term fetal fluid production decreases to

A

4-5 ml/kg/hour

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38
Q

Chloride being _______ transported into airspaces _______ volume of fetal lung fluid

A

Actively

Increases

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39
Q

Sodium secretion into air spaces

A

Causes of reabsorption of fetal lung fluid

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40
Q

Prenatal factors that contribute to fetal fluid reabsorption (35%) are

A

Increased sodium secretion
Decreased chloride secretion
Increased lymphatic noncotic pressure
Low fetal alveolar protein

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41
Q

Fetal lung fluid is cleared during labor by

A 
Mechanical compression (fetal lung compression)
Catecholamine surge (increased Na transport)
Higher cortisol and thyroid hormone concentrations (increased Na transport)
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42
Q

Fetal lung fluid is cleared postnatally by

A

Lung distention
Pulmonary lymphatic absorption by increased oncotic pressure/low fetal alveolar protein
Increased intrathoracic pressure from crying pushes fluid to capillaries and lymphatics

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43
Q

How is oxygenation index calculated?

A

FiO2 x MAP/ PaO2

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44
Q

How is oxygen content calculated?

A

(1.34xHgB)x(HgB) x O2 sat) + (0.003(paO2))

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45
Q

Alveolar-arterial gradient is calculated by:

A

PaCO2/R - paO2

R= 0.8

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46
Q

Surfactant consists primarily of what component?

A

Phosphatidylcholine-disaturated (50%)

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47
Q

What two surfactant proteins are in artificial surfactants?

A

SP-B, SP-C

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48
Q

Which surfactant protein is least clinically relevant?

A

SP-D

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49
Q

Which surfactant protein is most impacted by antenatal steroids?

A

SP-A

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50
Q

Chromosome 10 encodes SP___ and ____

A

A and D

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51
Q

SP-B is encoded on chromosome ____

A

2

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52
Q

SP-C is encoded on chromosome ____

A

8

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53
Q

Chronic interstitial lung diseases is associated with deficiency of surfactant protein _____

A

SP-B

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54
Q

Surfactant is produced by

A

Type II pneumocytes

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55
Q

Surfactant proteins nice in T2 pneumocytes from ______ to ___________ to join SP-A to create tubular myelin to reduce surface tension

A

Multivesicular bodies to

Lamellar bodies

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56
Q

Remaining surfactant in the alveolar surface are recycled through _________ to the _______ cell.

Remaining remnants are cleared by _______

A

Endocytosis
Type II pneumocytes

Macrophages

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57
Q

Lung maturity can be increased by

A

Inflammation, relative hypoxia, poor growth:

Hypertension/PIH, CV disease, infarction, IUGR, PROM, incompetent cervix, hemoglobinopathies, chorioamnionitis

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58
Q

Substances that showed lung maturity:

A

cAMP, growth factors, sex hormones (prolactin, estrogen), thyroid hormones, steroids, methylxanthines, B-agonists

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59
Q

Components that promote surfactant secretion and fetal lung fluid are

A

Purines
Prostaglandins
Beta agonist
Lung distension and fetal breathing

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60
Q

The amniotic fluid component that does indicate fetal lung maturity is

A

Lecithin

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61
Q

The pattern of phosphatidylinositol in amniotic fluid during lung maturation is

A

Early rise around 28 weeks and decrease around 35 weeks

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62
Q

Sphingomyelin is or is not related to lung maturity?

A

It is not related to lung maturity

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63
Q

A lecithin / sphingomyelin ratio that is greater than ____ indicates lung maturity

A

2

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64
Q

The pattern of phosphatidylglycerol and amniotic fluid during lung maturation is

A

Increase after 34 to 35 weeks

Correlated with lung maturity, infants with RDS will not have phosphatidylchloride in amniotic fluid

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65
Q

A lecithin / sphingomyelin ratio of ____ indicates 100% risk for RDS

A

Less than 1

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66
Q

Independent of the lecithin/sphingomyelin ratio the lack of ______ or the presence of ____ increases the risk of RDS

A

Phosphatidylglycerol

Diabetes or Rh isoimmunization

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67
Q

The presence of lamellar bodies in amniotic fluid suggests

A

Mature lung tissue

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68
Q

Natural surfactants contain which surfactant proteins?

A

SP/A and SP/B

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69
Q

Laplace’s law

A

The smaller the alveolar radius the greater the pressure needed to maintain distention against surface tension

P = 2T / r

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70
Q

Vasodilators that contribute to decrease in pulmonary vascular resistance at delivery are

A

Nitric oxide

Endothelin-1

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71
Q

Compounds that contribute to delayed decrease in pulmonary vascular resistance are

A

Defective prostaglandin or nitric oxide synthesis
Indomethacin
Prostaglandin synthesis inhibitors aka aspirin
Inflammatory response molecules: leukotrienes, thromboxane, platelet activating factor

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72
Q

Neural input to the medulla results in vagal nerve mediated limited inspiratory duration via the ______ reflex

A

Hering breuer inflationary

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73
Q

The herringbrew deflation reflex causes

A

Increase in ventilatory rate related to abrupt deflation of the lungs

Think of pneumothorax or periodic breathing
Maintains infants FRC

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74
Q

A pronounced increase in diaphragmatic contraction during inflation describes the

A

Paradoxical reflex of head

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75
Q

Chemoreceptors for CO2 and pH changes are located on

A

Central, Ventrolateral medulla

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76
Q

Changes in tidal volume are sensed by mechanoreceptors located

A

In the airway smooth muscle

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77
Q

Chemoreceptors for O2 changes are located on

A

Peripheral chemoreceptors in the carotid bodies in aortic bodies.

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78
Q

Response to hypoxemia by preterm and term infants differs in that term infants will have ________ and preterm infants will have ______

A

Increased respiratory rate

Apnea

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79
Q

Suprasternal retractions indicate

A

Upper airway obstruction

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80
Q

Unilateral subcostal retractions indicate

A

Decreased movement of the opposite diaphragm

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81
Q

The neonatal lung functions as zone

A

III

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82
Q

Air trapping or alveolar distention causes lung function to shift to zone

A

I or II

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83
Q

Increased extravascular fluid causes the lungs to shift to zone

A

IV

Increased pulmonary vascular resistance, decreased blood flow

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84
Q

Alveolar pressure rank correlates with lung perfusion zones

A

In zone I alveolar pressure is most prominent
In zone 2 alveolar pressure is second most prominent
In zone 3 alveolar pressure is third most prominent

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85
Q

In lung perfusion zones 2, 3 and 4, the prominent perfusion pressure is due to

A

Pulmonary arterial pressure

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86
Q

Optimal alveolar ventilation perfusion ratio should be

A

Close to 1

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87
Q

Have you ever ventilation is calculated by

A

(Tidal volume - dead space volume) x respiratory rate

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88
Q

High PCO2s are associated with a ____ alveolar ventilation and _____ perfusion

A

Low

Normal

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89
Q

An anatomic shunt will give a VQ ratio of

A

0

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90
Q

VQ ratio in the setting of a dead space will create a ratio of _____

A

> 1 to infinity

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91
Q

A VQ ratio greater than 1 suggests a _____ PaO2 and a ____ PaCO2.
O2 and CO2 content are ______

A

High

Low

Normal

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92
Q

A VQ ratio consistent with dead space demonstrates _____ ventilation and _______ perfusion.

A

Normal

Decreased

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93
Q

The most significant effect from VQ mismatching will be on oxygenation or ventilation?

A

Oxygenation

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94
Q

Two normal lung anatomical shunts exist in the

A

Coronary things draining to the thespian veins to the left ventricle

Bronchial circulation draining to the pulmonary veins

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95
Q

To determine the percentage of an interpulmonary shunt use the calculation

A

02 content pulmonary capillary - 02 content systemic arterial /
02 content pulmonary capillary - 02 content mixed venous

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96
Q

To estimate interpulmonary shunt % you can also use the calculation for ______, which does not take into consideration oxygenation as an increase of FIO2

A

A-a gradient

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97
Q

Bronchodilation ________ anatomic dead space

A

Increases

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98
Q

Fowler’s method estimates

A

Anatomic dead space using volume and nitrogen concentration of expired air

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99
Q

Physiologic dead spaces always greater than / less than an atomic dead space

A

Greater than

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100
Q

Physiologic dead space can be estimated using the _____ equation

A

Bohr

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101
Q

Bohr equation measures _______ through the following calculation:

A

Physiologic dead space

TV x (arterial CO2 - expired CO2) / arterial CO2

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102
Q

Partial pressure of gases within the alveoli tend to shift between _____ and _____ with relatively constant levels of _____ and _____

A

Oxygen and nitrogen

CO2 and H2O

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103
Q

Airway resistance is proportional to changes in ______

and inversely proportional to changes in _______

A

Pressure

Flow

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104
Q 
Respiratory system resistant is broken down by:
Airway (\_\_\_%)
Chest wall (\_\_\_\_%)
Lung tissue (\_\_\_\_%)
A

55%

25%

20%

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105
Q

In the newborn 50% of the airway resistance is due to

A

Nasal passages

106
Q

Increased airway resistance with laminar flow are due to

A

Increased airway length

Increased gas viscosity

107
Q

Poiseuille’s law describes

A

Laminar airflow resistance

108
Q

Laminar flow is calculated with the equation

A

Poiseuille’s law:

Change in pressure x pi x radius^4 /
8 (length x viscosity)

109
Q

Turbulent flow resistance ________ with increased airway length, ________ with increased radius, and _______ with increased gas density

A

Increases

Decreases

Increases

110
Q

Turbulent flow resistance is calculated by

A

(length x density)/

Radius^5

111
Q

Both turbulent and laminar flow resistance ______ with increased airway length and _______ with increasing radius.

A

Increase

Decrease

112
Q

Laminar flow resistance is inversely proportional to _______ while turbulent flow resistance is proportional to _______

A

Viscosity

Density

113
Q

Total and capacity equ

A

Vital capacity + residual volume

Or

Inspiratory capacity + functional residual capacity

114
Q

Functional residual capacity is

A

Expiratory reserve volume
+
residual volume

115
Q

Inspiratory capacity is

A

Inspiratory reserve volume
+
Title volume

116
Q

Inspiratory capacity is approximately equal to

A

Functional residual capacity

117
Q

Dead space is approximately equal to

A

1/3 Tidal volume

118
Q

Infants with RDS have decreased ________ and increased _______

A

Lung volume capacities

Dead space

119
Q

Compliance equals

A

Change in volume /

Change in pressure

120
Q

Elastance equals

A

Change in pressure /

Change in volume

121
Q

Hypoinflation lung disease such as atelectasis, RDS, and low volumes have findings of

A

Low FRC

Low compliance

122
Q

Hyperinflation lung disease such as MAS, chronic lung disease

A

Low compliance

High FRC

123
Q

Time constants for respiratory mechanics are dependent on

A

Resistance and compliance

124
Q

The stiffer the long tissue, the lower the compliance, the ________ The time to for inspiration and the _______ The time for exhalation

A

Longer

Faster

125
Q

One time constant is defined as

A

Time required for 63% of alveoli to discharge air volume

126
Q

Compared to an adult neonatal lungs have decreased:

A

Tidal volume
Total capacity
Inspiratory capacity
Vital capacity

127
Q

Pao2 measures the

A

Free 02 molecules dissolved in plasma in arterial blood

128
Q

True or false: pao2 does include the amount of oxygen bound to hemoglobin.

A

False

129
Q

Equation for oxygen content is

A

1.34 ml/g hgb x (hgb x o2 sat) + (0.003 * pao2)

130
Q

Which parameter is most significantly impacted by changes in hemoglobin concentration?

Oxygen content
Oxygen saturation
Pao2

A

Oxygen content

131
Q

Both high altitude and severe VQ mismatch have decreased

A

PAO2 and oxygen saturations

132
Q

Pao2 and oxygen saturations in the setting of severe anemia are

A

Unchanged

133
Q

Carbon monoxide poisoning causes PAO2, oxygen saturations, or both to be decreased?

A

Only oxygen saturations are decreased

Pao2 is unchanged

134
Q

Increased a a gradients are associated with

A

VQ mismatch

Shunting

135
Q

Oxygen delivery is determined by

A

Cardiac output * 02 content

136
Q

Pacific principle

A

Oxygen consumption

Difference between oxygen delivered to tissues and oxygen returning from the tissues

137
Q

Oxygen consumption is calculated by

A

CO x 1.34 ml/g hgb x hgb x (arterial o2 sat - venous o2 sat)

138
Q

Increased oxygen consumption results in an increase in

A

Cardiac output

139
Q

Five common sense areas in which oxygen is consumption is increased

A 
Increased caloric intake
Decreased body temperature
Neonate versus adult
Term versus preterm
AGA versus SGA infant
140
Q

Shift in barometric pressure and associated FIO2 requirements are calculated by

A

(pB1 - pH2O) x FiO2 = (pB2 - pH2O) x FiO2

141
Q

The LEFT oxyhemoglobin dissociation curve happens with:

A

LOW

  • acid
  • paCO2
  • 2,3 DPG
  • temp
  • P50
  • adult hgb (more fetal hgb)

The left curve is the REST curve- higher O2 affinity wouldn’t work in high oxygen demand states

142
Q

The RIGHT oxyhemoglobin dissociation curve happens with

A

RISING

  • acid
  • pCO2
  • 2,3 DPG
  • hydrogen
  • temp
  • p50
  • Adult hgb

The right curve is the WORK curve- lower O2 affinity answers increased oxygen demand states

143
Q

What inside catalyzes the conversion of CO2 to bicarbonate?

A

Carbonic anhydrase

144
Q

70% of CO2 in the blood is transported as

A

Bicarbonate ions

145
Q

Three forms of carbon dioxide in the body are

A 
Dissolved CO2 (10%)
Bicarbonate ion (70%)
Carbamino compounds (20%)
146
Q

How is CO2 solubility different than 02 and what is that advantage?

A

CO2 is 20 times more soluble in blood than oxygen

Increase solubility allows for easier transport and extraction with smaller changes in blood CO2 levels

147
Q

Bohr effect:

A

Increased oxygen release at lower pH or higher PCO2 (think increased oxygen need while running)

Curve shifts right

O2 and CO2 exchange at tissue-blood interface

PaCO2 decreases as it’s carried to alveoli. Lower paCO2/increased O2

Curve shifts back to left

148
Q

Haldane effect

A

High oxygen concentrations in the long facilitates carbon dioxide unloading into the alveoli

149
Q

Bohr effect

A

High CO2 in the tissues facilitate 02 unloading

OR

Low CO2 in the lungs facilitates 02 loading

150
Q

Henderson hasselbach equation

A

Hydrogen ions are adjusted by the kidneys and lungs to regulate pH by clearing CO2 or increasing bicarbonate

151
Q

To calculate hydrogen concentration by the Henderson Hasselbach equation:

A

24 x PCO2 / HCO3

152
Q

Carboxyhemoglobinemia is a result of

A

Excess carbon monoxide from tobacco smoke, fires, motor vehicle exhaust

153
Q

In paradoxygen carry capacity results from carboxyhemoglobinemia due to

A

Carbon monoxide binding to hemoglobin at a higher affinity than oxygen and competing with oxygen for hemoglobin binding sites

Oxyhemoglobin curve shifts to the left causing decreased o2 delivery to the tissues

154
Q

True or false: carbon monoxide can across the placenta and bind with you fetal hemoglobin

A

True

155
Q

Treatment of carbon monoxide poisoning is

A

Providing 100% oxygen to displace carbon monoxide from hemoglobin

156
Q

Excess nitrates, nitrites, exogenous nitric oxide, maternal prilocaine, aniline dyes, and hemoglobin M can cause

A

Methemoglobinemia

157
Q

What enzyme if defective can cause methemoglobinemia

A

Cytochrome B5 reductase

158
Q

How does methemoglobinemia alter iron and impact oxygen binding capacity of hemoglobin?

A

Changes iron from ferrous to ferric state

Decreases hemoglobin-oxygen binding abilities

159
Q

Clinically methemoglobinemia will have

A

Desaturations with normal PaO2

Brown appearance of blood following exposure to oxygen

160
Q

Mean airway pressure is calculated using

A

K (PIP - PEEP) X (ITIME/ITIME + ETIME) + PEEP

161
Q

Mechanisms involved in high frequency ventilation include

A 
Bulk convection
Pendulluft
Asymmetric velocity
Taylor dispersion
Molecular diffusion
162
Q

Pendelluft effect is

A

Gas moving between alveoli due to different time constants

Think pendulum

163
Q

Asymmetric velocity of high frequency ventilation is

A

Gas velocities varying during inspiration versus expiration

Think high inspiratory rates with passive, somewhat continuous exhalation

164
Q

Parabolic movement of inspired gas in which the highest velocity moves within the middle is called

A

Taylor dispersion

Tall Taylor runs through the middle

165
Q

Molecular diffusion

A

Transported gases across the alveoli taking advantage of a diffusion gradient

166
Q

Indications for ECMO

A

Fi02 100%
PAO2 less than 40
A-a gradient greater than 600
OI greater than 40

167
Q

Ekmo contraindications

A

Prematurity less than 34 weeks
Severe IVH
Significant coagulopathy
Irreversible pulmonary disease or neurologic abnormalities
Congenital anomalies or otherwise poor long-term outcome

168
Q

Nitric oxide is formed from

A

L arginine and nitric oxide synthase

169
Q

Nitric oxide relaxes vascular smooth muscle by

A

Guanylyl cyclase activation and increased cGMP

170
Q

The inactive form of nitric oxide is

A

NO2/NO3 (oxidized)

171
Q

How does nitric oxide selectively decrease pulmonary vascular resistance without causing hypotension?

A

Nitric oxide combined with hemoglobin becomes oxidized which is then an inactive form of nitric oxide

172
Q

How does inhaled nitric oxide improve ventilation perfusion mismatch?

A

Inhale nitric oxide selectively dilates ventilated blood vessels as it is unable to reach those that are not ventilated, and therefore not perfused

173
Q

In what clinical condition is inhaled nitric oxide contradicated?

A

Critical congenital heart disease with dependent right to left shunting

174
Q

Potential adverse effect of inhaled naturic oxide therapy is

A

Methemoglobinemia

175
Q

The most common microorganisms responsible for early congenital pneumonia are

A

GBS
E coli
Klebsiella
Listeria

176
Q

The microorganisms must commonly responsible for late congenital pneumonia are

A

Staph aureus
Pseudomonas
Fungus
Chlamydia

177
Q

Fever associated with congenital pneumonia is most commonly due to an infection with

A

Herpes or enterovirus

178
Q

Vital sign changes associated with tension pneumothorax are

A

Decrease in blood pressure
Decrease in heart rate
Decrease in respiratory rate

179
Q

Significant pneumothorax can be associated with these two comorbidities

A

Interventricular hemorrhage

SIADH

180
Q

What is a Spinnaker sail sign?

A

X-ray finding with pneumomediastinum

Elevated, well visualized thymus and hyperlucency

181
Q

Describe Wilson Mikity syndrome

A

Variant of BPD with minimal early disease progressing to significant BPD and slow recovery

182
Q

What microorganism is associated with the increased risk of BPD?

A

Ureaplasma urealyticum

183
Q

On x-ray stage 4 BPD has

A

Distortion of architecture, large cystic areas, interstitial fibrosis, atelectasis, hyperinflation

184
Q

What is the mortality rate of severe chronic lung disease?

A

20-40%

185
Q

Why are antibiotics given in the setting of meconium aspiration syndrome?

A

Meconium increases bacterial
Clinical findings are indistinguishable from congenital pneumonia
Sepsis may be a cause for aspiration

186
Q

What cardiac findings are present in the setting of pulmonary hypertension?

A

Single or narrowly split loud S2

Possible ST changes due to subendocardial ischemia

187
Q

What is the mechanism of action of sildenafil?

A

Phosphodiesterase 5 inhibitor

188
Q

Inspiratory stridor is due to what type of airway obstruction?

A

Supraglottic
Nose, nasopharynx, oropharynx, hypopharynx

Differential includes Pierre-Robin, treacher-collins, macroglossia, Beckwith-Wiedeman syndrome, hypothyroidism, glycogen storage diseases, trisomy 21, choanal atresia, thyroglossal duct cyst

189
Q

An obstruction with a fixed size during inspiration and expiration will cause what kind of stridor?

A

Biphasic

Due to laryngeal obstruction

190
Q

Most common cause of biphasic strider is

A

Laryngomalacia

191
Q

Expiratory stridor can be often due to

A

Tracheomalacia
Tracheal stenosis
External compression from vascular ring or mediastinal mass

192
Q

The most concerning type of stridor is

A

Expiratory

193
Q

Most common type of vascular ring is ______ due to a ______

A

Complete vascular ring
Double aortic arch (persistent right and left fourth branchial arches)
40%

194
Q

An alternative cause of a complete vascular ring and second most common is

A

A rate aortic arch with ligamentum arteriosum/PDA

30%

195
Q

Of the three causes of incomplete vascular rings, in order of most two least common are

A

Abberant right subclavian artery, 20%
Anomalous innominate artery, 10%
Abberant left pulmonary artery, rare

196
Q

Choanal atresia was commonly occurs where and in what patient population?

A

Unilateral (2/3), right (2:1)

Females (2:1)

197
Q

The syndrome most commonly associated with choanal atresia is

A 
CHARGE
C- coloboma
H- heart disease
A- choanal atresia
R- mental deficiency
G- genital hypoplasia
E- ear anomalies
198
Q

Most common location of vocal cord paralysis injury is

A

Unilateral, left (left recurrent laryngeal nerve)

199
Q

Appropriate management of vocal cord paralysis includes

A

Imaging, x-ray and MRI, to determine side of pathology
Airway productive strategies: if bilateral, tracheostomy. If unilateral or mild may need observation in hopes of spontaneous resolution in 2-9 months
For persistent injury, arytenoidectomy or vocal cord lateralization techniques

200
Q

Pathophysiology of tracheomalacia is

A

Collapse of cartilaginous rings supporting the trachea

201
Q

Tracheomalacia presents with

A

Expiratory strider

202
Q

Most common cause of congenital tracheal narrowing is

A

Tracheomalacia

203
Q

Diagnosis of tracheomalacia is made with

A

Bronchoscopy

204
Q

Treatment and outcome of tracheomalacia include

A

Supportive respiratory strategies including CPAP or tracheostomy
Resolution by 6 to 12 months of age

205
Q

Pathophysiology of laryngeomalacia is

A

Epiglottis or arytenoid cartilage collapse with prolapse into the glottis

206
Q

The most common cause of congenital stridor is

A

Laryngomalacia

207
Q

Presentation and patient population associated with laryngomalacia is

A

Onset in the first month of life, male predominance (2:1)

208
Q

Diagnosis of laryngeomalacia

A

Laryngoscopy

209
Q

Treatment and expected outcome of laryngomalacia is

A

Conservative/supportive treatment

Spontaneous resolution by 2 years of age

210
Q

Most congenital diaphragmatic hernias are on the _____ side

A

Left (85%)

211
Q

Syndromes that maybe associated with a CDHR

A 
Fryns syndrome
Denys-Drash
Cornelia-de-Lange
Marfan
Spondylocostal dysostosis
Craniofrontonasal
212
Q

Most common location of a CDH is

A

Left posterior lateral region, foramen of bochdalek, 90%

213
Q

An infectious cause that has been associated with a right side CDH is

A

GBS infection

214
Q

In CDH, herniation of the _____ and _____ often occur; prognosis is worse if _____ and _____ are also involved.

A

Intestine and spleen

Liver and stomach

215
Q

Survival rate for infants with CDH is

A

60%

216
Q

Diaphragmatic paralysis most commonly involves the

A

Right side, unilateral (9:1)

217
Q

Right-sided diaphragmatic paralysis is most commonly associated with

A

Phrenic nerve injury due to birth trauma or cardiothoracic surgery

218
Q

Physical exam in unilateral diaphragmatic paralysis will show

A

Affected side with decreased retractions,

Increased collapse during inspiration

219
Q

Bilateral diaphragmatic paralysis can be due to

A

Furniture of injury due to birth trauma or cardiothoracic surgery
Neuromuscular disorder

220
Q

Presentation of bilateral diaphragmatic paralysis is

A

Severe respiratory failure often requiring intubation

221
Q

Outcome of unilateral versus bilateral diaphragmatic paralysis is

A

Spontaneous resolution by one year if unilateral, often fatal if bilateral

222
Q

Pleural effusion due to congestive heart failure, hypoproteinemia, not immune hydrops, or iatrogenic has a _______ fluid

A

Transudative

Increased pH
Decreased WBC, protein, specific gravity, LDH

223
Q

Pleural effusion due to inflammation or infection has a _______ fluid

A

Exudative

Decreased pH
Increased WBC, protein, specific gravity, LDH
Low glucose

224
Q

Pathophysiology of chylothorax

A

Intrauterine thoracic duct obstruction

—>. Thoracic duct and plural space fistula

225
Q

Laterality of a chylothorax is most often

A

Right side» left side&raquo_space; bilateral

226
Q

Fluid diagnosis of chylothorax shows

A

Xanthochromia
Lymphocytosis (70%)
Increased protein and triglycerides

227
Q

Optimal feeding preference for patients with chylothorax is

A

Formula with medium chain triglycerides

228
Q

Cartilaginous deficiency causing disruption of bronchopulmonary development and subsequent ball valve effect with over distention is called

A

Congenital lobar emphysema

229
Q

Congenital lobar emphysema most commonly affects what patient population and what pulmonary location?

A

Males&raquo_space;> females

Left upper lobe, 45%
Right middle lobe 30%
Right upper lobe 20%

230
Q

What other anomaly can be associated with congenital lobar emphysema?

A

Congenital heart disease

231
Q

What is the typical presentation of congenital lobar emphysema?

A

Respiratory distress in the first month of life
Gradual or rapid progression
Dimish breath sounds or wheezing
Maybe diagnosed on prenatal ultrasound, and can regress prior to birth
Chest x-ray shows opacification of affected lobe

232
Q

A branching abnormality of the lung with communication to the tracheobronchial tree is a

A

Congenital cystic adenomatoid malformation

233
Q

Perfusion and location of CCAMs are typically

A

Perfused by pulmonary circulation

Unilateral (80-90%)l

234
Q

The most common type of CCAM is a type

A

Type 1, 50-70%

235
Q

The most rare type of CCAM are type

A

Type zero and type four

236
Q

Timing of defect of type 1 2 and 3 CCAM is

A

Type 1: 7-10 weeks

Type 2: 3 weeks

Type 3: 4 weeks

237
Q

The CCAM most likely to cause a mass effect is a type

A

Type 3

Can also see compression with type 1 and type 4

238
Q

CCAMs involving ciliated epithelium are

A

Type 0, type 1, type 2

239
Q

Nonciliated epithelium are found in CCAMs type

A

Type 3 and 4

240
Q

Mucus cells and cartilage are found only in CCAM type

A

Type 0 and 1

241
Q

What is the typical progression of CCAMs during gestation?

A

Occur by the 10th week of gestation, many regress with peak size at 25 weeks gestation

242
Q

Foresee cams that persist after birth, what is the difficult presentation?

A

50% asymptomatic at birth
Large lesions will cause respiratory distress after delivery
30% will present with recurrent pneumonia in childhood

243
Q

What is the pre and postnatal management of CCAM?

A

Monitoring for hydrops, fetal surgery if indicated
Supportive care for respiratory distress
Complete resection

244
Q

Which CCAM types have the best prognosis?

A

Type 1 and 4

245
Q

Which CCAM types have the worst prognosis? And why?

A

Type 0 and 2, due to associated anomalies

Type 3 due to mass effect and associated pulmonary hypoplasia and hypertension

246
Q

An airway cystic mass filled that is air filled or solid derived from the foregut is a

A

Bronchogenic cyst

247
Q

Bronchogenic cysts tend to be located

A

Posterior to trachea
Can be intraparenchymal
May communicate with GI tract

248
Q

Presentation of bronchogenic cysts

A

Most are asymptomatic until second decade of life, can present with coughing wheezing and recurrent pneumonia
Cysts that connect to the airway may enlarge rapidly after birth and cause respiratory distress

249
Q

Why is surgical excision indicated for bronchogenic cysts even if asymptomatic?

A

Due to risk of complications including malignant changes

250
Q

Congenital lymphangiectasia most commonly affects

A

Males, 2:1

Patients with noonan syndrome and trisomy 21

251
Q

Congenital lymphangiectasia is caused by

A

Abnormal development or lymphatic obstruction that causes dilation of the lymphatic vessels in the lung

252
Q

Clinical signs of congenital lymphangiectasia include

A

Respiratory distress after birth
Plural effusions
Hyperinflated lungs and diffuse granular densities on x-ray

253
Q

Non-functional lung tissue that doesn’t communicate with the tracheobronchial tree and is perfused by systemic circulation is a

A

Bronchopulmonary sequestration

254
Q

Most bronchopulmonary sequestrations are located

A

Intralobar, 75%
Lower lobes
Tend to occur more on left side

255
Q

Are intralobar or extralobar sequestrations more likely to have associated anomalies?

A

Extralobar, 40 to 60%

256
Q

A bronchopulmonary sequestration that presents in the neonatal period is more likely to be located in a _____ space.

A

Extralobar

257
Q

Clinical course of bronchopulmonary sequestration is typically

A

Incidental finding during prenatal imaging
Mini regress in the prenatal period, some may develop hydrops
Extralobar type may present with respiratory distress in the neonatal.
Intralobar type may present with recurrent pneumonia in childhood

258
Q

Additional anomalies that maybe associated with bronchopulmonary sequestration is

A

Congenital heart disease,

Congenital diaphragmatic hernia

259
Q

Mechanism of action of acetazolamide is

A

Carbonic anhydrase inhibitor, proximal tubule

260
Q

Mechanism of action of furosemide is

A

Blocks active chloride transport, ascending loop of Henle

261
Q

Mechanism of action of spironolactone is

A

Aldosterone antagonist, collecting system

262
Q

Mechanism of action of chlorothiazide is

A

Inhibition of sodium chloride reabsorption, distal tubule

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