Respiratory Flashcards
1
Q
What embryologic structure forms the respiratory tract?
A
Endoderm (foregut)
Ventral esophagus–> Lungs
Mesodermal mesenchyme–> branching
2
Q
When does airway branching complete?
A
12-14 weeks
3
Q
What part of the central great vessels forms the pulmonary vasculature?
A
Sixth aortic arch branches
Pulmonary arteries supply the intrapulmonary structures
4
Q
What structures does the bronchial artery system supply?
A
Conducting airways
Visceral pleura
Connective tissue
Pulmonary arteries
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
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
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
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
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
10
Q
How do small pulmonary arteries change in 4-6 week infants?
A
Medial smooth muscle layer involutes
Muscular wall thickness decreases
11
Q
During what phase do bronchioles and alveoli increase?
A
Alveolar phase
12
Q
When do alveoli develop?
A
Late gestation–> 3-8 years
13
Q
How many alveoli does the term infant have?
A
50-150 million alveoli
14
Q
How many alveoli does the adult have?
A
200-600 million
15
Q
What factors delay alveolization?
A
Antenatal steroids
Supplemental oxygen
Nutritional deficiencies
Mechanical ventilation
16
Q
What are the embryonic stages of lung development?
A
Embryonic pseudoglingular canalicular saccular alveolar
17
Q
When does embryonic lung development occur?
A
0-5 weeks
18
Q
What two key structures develop during the embryonic phase of lung development?
A
Trachea
bronchi
19
Q
What pulmonary anomalies develop during the embryonic phase of lung development?
A
Laryngeal cleft
Tracheal stenosis
Tracheoesophageal fistula
20
Q
When does the pseudo glandular phase of lung development occur?
A
5 to 15 weeks
21
Q
What key structures develop during the pseudoglanular phase of lung development?
A
Non-respiratory bronchioles
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
23
Q
When do the lungs begin to develop amniotic fluid?
A
During the pseudo-glandular phase
24
Q
When does the canalicular phase of lung development occur?
A
15-25 weeks
25
What structures develop during the canalicular phase?
Respiratory bronchioles
26
What lung anomaly occurs during canalicular development?
Pulmonary hypoplasia
27
When during lung development do pneumocytes begin to differentiate from type 2 to type 1?
Canalicular phase, 15-25 weeks
28
When does the saccular phase of lung developing occur?
25-35 weeks
29
What structures develop during the saccular phase of lung development?
Alveolar ducts
30
What lung anomaly occurs during the saccular phase of lung development?
Pulmonary hypoplasia
31
When does the alveolar phase of lung development occur?
36+ weeks
32
What structures develop during the alveolar phase of lung development?
Alveoli
33
```
What does
Each
Pulmonary part
Comes
Through
Age
represent?
```
```
Stages of lung development
Embryonic
Pseudo-glandular
Canalicular
Terminal sac (Saccular)
Alveolar
```
34
Describe type 1 pneumocytes
```
Fried egg
Tight junctions
90% surface area
Smaller number
Gas exchange
Formed from type 2 pneumocytes
```
35
Describe type 2 pneumocytes
```
Cuboid
10% of surface area
Greater number
Surfactant metabolism and secretion
Form type 1 cells
```
36
Fetal lung fluid production prior to term is equivalent to
Functional residual capacity
| 20 to 30 ml/kg
37
At term fetal fluid production decreases to
4-5 ml/kg/hour
38
Chloride being _______ transported into airspaces _______ volume of fetal lung fluid
Actively
| Increases
39
Sodium secretion into air spaces
Causes of reabsorption of fetal lung fluid
40
Prenatal factors that contribute to fetal fluid reabsorption (35%) are
Increased sodium secretion
Decreased chloride secretion
Increased lymphatic noncotic pressure
Low fetal alveolar protein
41
Fetal lung fluid is cleared during labor by
```
Mechanical compression (fetal lung compression)
Catecholamine surge (increased Na transport)
Higher cortisol and thyroid hormone concentrations (increased Na transport)
```
42
Fetal lung fluid is cleared postnatally by
Lung distention
Pulmonary lymphatic absorption by increased oncotic pressure/low fetal alveolar protein
Increased intrathoracic pressure from crying pushes fluid to capillaries and lymphatics
43
How is oxygenation index calculated?
FiO2 x MAP/ PaO2
44
How is oxygen content calculated?
(1.34xHgB)x(HgB) x O2 sat) + (0.003(paO2))
45
Alveolar-arterial gradient is calculated by:
PaCO2/R - paO2
R= 0.8
46
Surfactant consists primarily of what component?
Phosphatidylcholine-disaturated (50%)
47
What two surfactant proteins are in artificial surfactants?
SP-B, SP-C
48
Which surfactant protein is least clinically relevant?
SP-D
49
Which surfactant protein is most impacted by antenatal steroids?
SP-A
50
Chromosome 10 encodes SP___ and ____
A and D
51
SP-B is encoded on chromosome ____
2
52
SP-C is encoded on chromosome ____
8
53
Chronic interstitial lung diseases is associated with deficiency of surfactant protein _____
SP-B
54
Surfactant is produced by
Type II pneumocytes
55
Surfactant proteins nice in T2 pneumocytes from ______ to ___________ to join SP-A to create tubular myelin to reduce surface tension
Multivesicular bodies to
| Lamellar bodies
56
Remaining surfactant in the alveolar surface are recycled through _________ to the _______ cell.
Remaining remnants are cleared by _______
Endocytosis
Type II pneumocytes
Macrophages
57
Lung maturity can be increased by
Inflammation, relative hypoxia, poor growth:
Hypertension/PIH, CV disease, infarction, IUGR, PROM, incompetent cervix, hemoglobinopathies, chorioamnionitis
58
Substances that showed lung maturity:
cAMP, growth factors, sex hormones (prolactin, estrogen), thyroid hormones, steroids, methylxanthines, B-agonists
59
Components that promote surfactant secretion and fetal lung fluid are
Purines
Prostaglandins
Beta agonist
Lung distension and fetal breathing
60
The amniotic fluid component that does indicate fetal lung maturity is
Lecithin
61
The pattern of phosphatidylinositol in amniotic fluid during lung maturation is
Early rise around 28 weeks and decrease around 35 weeks
62
Sphingomyelin is or is not related to lung maturity?
It is not related to lung maturity
63
A lecithin / sphingomyelin ratio that is greater than ____ indicates lung maturity
2
64
The pattern of phosphatidylglycerol and amniotic fluid during lung maturation is
Increase after 34 to 35 weeks
| Correlated with lung maturity, infants with RDS will not have phosphatidylchloride in amniotic fluid
65
A lecithin / sphingomyelin ratio of ____ indicates 100% risk for RDS
Less than 1
66
Independent of the lecithin/sphingomyelin ratio the lack of ______ or the presence of ____ increases the risk of RDS
Phosphatidylglycerol
Diabetes or Rh isoimmunization
67
The presence of lamellar bodies in amniotic fluid suggests
Mature lung tissue
68
Natural surfactants contain which surfactant proteins?
SP/A and SP/B
69
Laplace's law
The smaller the alveolar radius the greater the pressure needed to maintain distention against surface tension
P = 2T / r
70
Vasodilators that contribute to decrease in pulmonary vascular resistance at delivery are
Nitric oxide
| Endothelin-1
71
Compounds that contribute to delayed decrease in pulmonary vascular resistance are
Defective prostaglandin or nitric oxide synthesis
Indomethacin
Prostaglandin synthesis inhibitors aka aspirin
Inflammatory response molecules: leukotrienes, thromboxane, platelet activating factor
72
Neural input to the medulla results in vagal nerve mediated limited inspiratory duration via the ______ reflex
Hering breuer inflationary
73
The herringbrew deflation reflex causes
Increase in ventilatory rate related to abrupt deflation of the lungs
Think of pneumothorax or periodic breathing
Maintains infants FRC
74
A pronounced increase in diaphragmatic contraction during inflation describes the
Paradoxical reflex of head
75
Chemoreceptors for CO2 and pH changes are located on
Central, Ventrolateral medulla
76
Changes in tidal volume are sensed by mechanoreceptors located
In the airway smooth muscle
77
Chemoreceptors for O2 changes are located on
Peripheral chemoreceptors in the carotid bodies in aortic bodies.
78
Response to hypoxemia by preterm and term infants differs in that term infants will have ________ and preterm infants will have ______
Increased respiratory rate
Apnea
79
Suprasternal retractions indicate
Upper airway obstruction
80
Unilateral subcostal retractions indicate
Decreased movement of the opposite diaphragm
81
The neonatal lung functions as zone
III
82
Air trapping or alveolar distention causes lung function to shift to zone
I or II
83
Increased extravascular fluid causes the lungs to shift to zone
IV
| Increased pulmonary vascular resistance, decreased blood flow
84
Alveolar pressure rank correlates with lung perfusion zones
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
85
In lung perfusion zones 2, 3 and 4, the prominent perfusion pressure is due to
Pulmonary arterial pressure
86
Optimal alveolar ventilation perfusion ratio should be
Close to 1
87
Have you ever ventilation is calculated by
(Tidal volume - dead space volume) x respiratory rate
88
High PCO2s are associated with a ____ alveolar ventilation and _____ perfusion
Low
Normal
89
An anatomic shunt will give a VQ ratio of
0
90
VQ ratio in the setting of a dead space will create a ratio of _____
>1 to infinity
91
A VQ ratio greater than 1 suggests a _____ PaO2 and a ____ PaCO2.
O2 and CO2 content are ______
High
Low
Normal
92
A VQ ratio consistent with dead space demonstrates _____ ventilation and _______ perfusion.
Normal
Decreased
93
The most significant effect from VQ mismatching will be on oxygenation or ventilation?
Oxygenation
94
Two normal lung anatomical shunts exist in the
Coronary things draining to the thespian veins to the left ventricle
Bronchial circulation draining to the pulmonary veins
95
To determine the percentage of an interpulmonary shunt use the calculation
02 content pulmonary capillary - 02 content systemic arterial /
02 content pulmonary capillary - 02 content mixed venous
96
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 gradient
97
Bronchodilation ________ anatomic dead space
Increases
98
Fowler's method estimates
Anatomic dead space using volume and nitrogen concentration of expired air
99
Physiologic dead spaces always greater than / less than an atomic dead space
Greater than
100
Physiologic dead space can be estimated using the _____ equation
Bohr
101
Bohr equation measures _______ through the following calculation:
Physiologic dead space
TV x (arterial CO2 - expired CO2) / arterial CO2
102
Partial pressure of gases within the alveoli tend to shift between _____ and _____ with relatively constant levels of _____ and _____
Oxygen and nitrogen
CO2 and H2O
103
Airway resistance is proportional to changes in ______
| and inversely proportional to changes in _______
Pressure
Flow
104
```
Respiratory system resistant is broken down by:
Airway (___%)
Chest wall (____%)
Lung tissue (____%)
```
55%
25%
20%
105
In the newborn 50% of the airway resistance is due to
Nasal passages
106
Increased airway resistance with laminar flow are due to
Increased airway length
| Increased gas viscosity
107
Poiseuille's law describes
Laminar airflow resistance
108
Laminar flow is calculated with the equation
Poiseuille's law:
Change in pressure x pi x radius^4 /
8 (length x viscosity)
109
Turbulent flow resistance ________ with increased airway length, ________ with increased radius, and _______ with increased gas density
Increases
Decreases
Increases
110
Turbulent flow resistance is calculated by
(length x density)/
| Radius^5
111
Both turbulent and laminar flow resistance ______ with increased airway length and _______ with increasing radius.
Increase
Decrease
112
Laminar flow resistance is inversely proportional to _______ while turbulent flow resistance is proportional to _______
Viscosity
Density
113
Total and capacity equ
Vital capacity + residual volume
Or
Inspiratory capacity + functional residual capacity
114
Functional residual capacity is
Expiratory reserve volume
+
residual volume
115
Inspiratory capacity is
Inspiratory reserve volume
+
Title volume
116
Inspiratory capacity is approximately equal to
Functional residual capacity
117
Dead space is approximately equal to
1/3 Tidal volume
118
Infants with RDS have decreased ________ and increased _______
Lung volume capacities
Dead space
119
Compliance equals
Change in volume /
| Change in pressure
120
Elastance equals
Change in pressure /
| Change in volume
121
Hypoinflation lung disease such as atelectasis, RDS, and low volumes have findings of
Low FRC
| Low compliance
122
Hyperinflation lung disease such as MAS, chronic lung disease
Low compliance
| High FRC
123
Time constants for respiratory mechanics are dependent on
Resistance and compliance
124
The stiffer the long tissue, the lower the compliance, the ________ The time to for inspiration and the _______ The time for exhalation
Longer
Faster
125
One time constant is defined as
Time required for 63% of alveoli to discharge air volume
126
Compared to an adult neonatal lungs have decreased:
Tidal volume
Total capacity
Inspiratory capacity
Vital capacity
127
Pao2 measures the
Free 02 molecules dissolved in plasma in arterial blood
128
True or false: pao2 does include the amount of oxygen bound to hemoglobin.
False
129
Equation for oxygen content is
1.34 ml/g hgb x (hgb x o2 sat) + (0.003 * pao2)
130
Which parameter is most significantly impacted by changes in hemoglobin concentration?
Oxygen content
Oxygen saturation
Pao2
Oxygen content
131
Both high altitude and severe VQ mismatch have decreased
PAO2 and oxygen saturations
132
Pao2 and oxygen saturations in the setting of severe anemia are
Unchanged
133
Carbon monoxide poisoning causes PAO2, oxygen saturations, or both to be decreased?
Only oxygen saturations are decreased
| Pao2 is unchanged
134
Increased a a gradients are associated with
VQ mismatch
| Shunting
135
Oxygen delivery is determined by
Cardiac output * 02 content
136
Pacific principle
Oxygen consumption
| Difference between oxygen delivered to tissues and oxygen returning from the tissues
137
Oxygen consumption is calculated by
CO x 1.34 ml/g hgb x hgb x (arterial o2 sat - venous o2 sat)
138
Increased oxygen consumption results in an increase in
Cardiac output
139
Five common sense areas in which oxygen is consumption is increased
```
Increased caloric intake
Decreased body temperature
Neonate versus adult
Term versus preterm
AGA versus SGA infant
```
140
Shift in barometric pressure and associated FIO2 requirements are calculated by
(pB1 - pH2O) x FiO2 = (pB2 - pH2O) x FiO2
141
The LEFT oxyhemoglobin dissociation curve happens with:
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
The RIGHT oxyhemoglobin dissociation curve happens with
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
What inside catalyzes the conversion of CO2 to bicarbonate?
Carbonic anhydrase
144
70% of CO2 in the blood is transported as
Bicarbonate ions
145
Three forms of carbon dioxide in the body are
```
Dissolved CO2 (10%)
Bicarbonate ion (70%)
Carbamino compounds (20%)
```
146
How is CO2 solubility different than 02 and what is that advantage?
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
Bohr effect:
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
Haldane effect
High oxygen concentrations in the long facilitates carbon dioxide unloading into the alveoli
149
Bohr effect
High CO2 in the tissues facilitate 02 unloading
OR
Low CO2 in the lungs facilitates 02 loading
150
Henderson hasselbach equation
Hydrogen ions are adjusted by the kidneys and lungs to regulate pH by clearing CO2 or increasing bicarbonate
151
To calculate hydrogen concentration by the Henderson Hasselbach equation:
24 x PCO2 / HCO3
152
Carboxyhemoglobinemia is a result of
Excess carbon monoxide from tobacco smoke, fires, motor vehicle exhaust
153
In paradoxygen carry capacity results from carboxyhemoglobinemia due to
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
True or false: carbon monoxide can across the placenta and bind with you fetal hemoglobin
True
155
Treatment of carbon monoxide poisoning is
Providing 100% oxygen to displace carbon monoxide from hemoglobin
156
Excess nitrates, nitrites, exogenous nitric oxide, maternal prilocaine, aniline dyes, and hemoglobin M can cause
Methemoglobinemia
157
What enzyme if defective can cause methemoglobinemia
Cytochrome B5 reductase
158
How does methemoglobinemia alter iron and impact oxygen binding capacity of hemoglobin?
Changes iron from ferrous to ferric state
Decreases hemoglobin-oxygen binding abilities
159
Clinically methemoglobinemia will have
Desaturations with normal PaO2
| Brown appearance of blood following exposure to oxygen
160
Mean airway pressure is calculated using
K (PIP - PEEP) X (ITIME/ITIME + ETIME) + PEEP
161
Mechanisms involved in high frequency ventilation include
```
Bulk convection
Pendulluft
Asymmetric velocity
Taylor dispersion
Molecular diffusion
```
162
Pendelluft effect is
Gas moving between alveoli due to different time constants
| Think pendulum
163
Asymmetric velocity of high frequency ventilation is
Gas velocities varying during inspiration versus expiration
| Think high inspiratory rates with passive, somewhat continuous exhalation
164
Parabolic movement of inspired gas in which the highest velocity moves within the middle is called
Taylor dispersion
| Tall Taylor runs through the middle
165
Molecular diffusion
Transported gases across the alveoli taking advantage of a diffusion gradient
166
Indications for ECMO
Fi02 100%
PAO2 less than 40
A-a gradient greater than 600
OI greater than 40
167
Ekmo contraindications
Prematurity less than 34 weeks
Severe IVH
Significant coagulopathy
Irreversible pulmonary disease or neurologic abnormalities
Congenital anomalies or otherwise poor long-term outcome
168
Nitric oxide is formed from
L arginine and nitric oxide synthase
169
Nitric oxide relaxes vascular smooth muscle by
Guanylyl cyclase activation and increased cGMP
170
The inactive form of nitric oxide is
NO2/NO3 (oxidized)
171
How does nitric oxide selectively decrease pulmonary vascular resistance without causing hypotension?
Nitric oxide combined with hemoglobin becomes oxidized which is then an inactive form of nitric oxide
172
How does inhaled nitric oxide improve ventilation perfusion mismatch?
Inhale nitric oxide selectively dilates ventilated blood vessels as it is unable to reach those that are not ventilated, and therefore not perfused
173
In what clinical condition is inhaled nitric oxide contradicated?
Critical congenital heart disease with dependent right to left shunting
174
Potential adverse effect of inhaled naturic oxide therapy is
Methemoglobinemia
175
The most common microorganisms responsible for early congenital pneumonia are
GBS
E coli
Klebsiella
Listeria
176
The microorganisms must commonly responsible for late congenital pneumonia are
Staph aureus
Pseudomonas
Fungus
Chlamydia
177
Fever associated with congenital pneumonia is most commonly due to an infection with
Herpes or enterovirus
178
Vital sign changes associated with tension pneumothorax are
Decrease in blood pressure
Decrease in heart rate
Decrease in respiratory rate
179
Significant pneumothorax can be associated with these two comorbidities
Interventricular hemorrhage
| SIADH
180
What is a Spinnaker sail sign?
X-ray finding with pneumomediastinum
| Elevated, well visualized thymus and hyperlucency
181
Describe Wilson Mikity syndrome
Variant of BPD with minimal early disease progressing to significant BPD and slow recovery
182
What microorganism is associated with the increased risk of BPD?
Ureaplasma urealyticum
183
On x-ray stage 4 BPD has
Distortion of architecture, large cystic areas, interstitial fibrosis, atelectasis, hyperinflation
184
What is the mortality rate of severe chronic lung disease?
20-40%
185
Why are antibiotics given in the setting of meconium aspiration syndrome?
Meconium increases bacterial
Clinical findings are indistinguishable from congenital pneumonia
Sepsis may be a cause for aspiration
186
What cardiac findings are present in the setting of pulmonary hypertension?
Single or narrowly split loud S2
| Possible ST changes due to subendocardial ischemia
187
What is the mechanism of action of sildenafil?
Phosphodiesterase 5 inhibitor
188
Inspiratory stridor is due to what type of airway obstruction?
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
An obstruction with a fixed size during inspiration and expiration will cause what kind of stridor?
Biphasic
| Due to laryngeal obstruction
190
Most common cause of biphasic strider is
Laryngomalacia
191
Expiratory stridor can be often due to
Tracheomalacia
Tracheal stenosis
External compression from vascular ring or mediastinal mass
192
The most concerning type of stridor is
Expiratory
193
Most common type of vascular ring is ______ due to a ______
Complete vascular ring
Double aortic arch (persistent right and left fourth branchial arches)
40%
194
An alternative cause of a complete vascular ring and second most common is
A rate aortic arch with ligamentum arteriosum/PDA
| 30%
195
Of the three causes of incomplete vascular rings, in order of most two least common are
Abberant right subclavian artery, 20%
Anomalous innominate artery, 10%
Abberant left pulmonary artery, rare
196
Choanal atresia was commonly occurs where and in what patient population?
Unilateral (2/3), right (2:1)
| Females (2:1)
197
The syndrome most commonly associated with choanal atresia is
```
CHARGE
C- coloboma
H- heart disease
A- choanal atresia
R- mental deficiency
G- genital hypoplasia
E- ear anomalies
```
198
Most common location of vocal cord paralysis injury is
Unilateral, left (left recurrent laryngeal nerve)
199
Appropriate management of vocal cord paralysis includes
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
Pathophysiology of tracheomalacia is
Collapse of cartilaginous rings supporting the trachea
201
Tracheomalacia presents with
Expiratory strider
202
Most common cause of congenital tracheal narrowing is
Tracheomalacia
203
Diagnosis of tracheomalacia is made with
Bronchoscopy
204
Treatment and outcome of tracheomalacia include
Supportive respiratory strategies including CPAP or tracheostomy
Resolution by 6 to 12 months of age
205
Pathophysiology of laryngeomalacia is
Epiglottis or arytenoid cartilage collapse with prolapse into the glottis
206
The most common cause of congenital stridor is
Laryngomalacia
207
Presentation and patient population associated with laryngomalacia is
Onset in the first month of life, male predominance (2:1)
208
Diagnosis of laryngeomalacia
Laryngoscopy
209
Treatment and expected outcome of laryngomalacia is
Conservative/supportive treatment
| Spontaneous resolution by 2 years of age
210
Most congenital diaphragmatic hernias are on the _____ side
Left (85%)
211
Syndromes that maybe associated with a CDHR
```
Fryns syndrome
Denys-Drash
Cornelia-de-Lange
Marfan
Spondylocostal dysostosis
Craniofrontonasal
```
212
Most common location of a CDH is
Left posterior lateral region, foramen of bochdalek, 90%
213
An infectious cause that has been associated with a right side CDH is
GBS infection
214
In CDH, herniation of the _____ and _____ often occur; prognosis is worse if _____ and _____ are also involved.
Intestine and spleen
| Liver and stomach
215
Survival rate for infants with CDH is
60%
216
Diaphragmatic paralysis most commonly involves the
Right side, unilateral (9:1)
217
Right-sided diaphragmatic paralysis is most commonly associated with
Phrenic nerve injury due to birth trauma or cardiothoracic surgery
218
Physical exam in unilateral diaphragmatic paralysis will show
Affected side with decreased retractions,
| Increased collapse during inspiration
219
Bilateral diaphragmatic paralysis can be due to
Furniture of injury due to birth trauma or cardiothoracic surgery
Neuromuscular disorder
220
Presentation of bilateral diaphragmatic paralysis is
Severe respiratory failure often requiring intubation
221
Outcome of unilateral versus bilateral diaphragmatic paralysis is
Spontaneous resolution by one year if unilateral, often fatal if bilateral
222
Pleural effusion due to congestive heart failure, hypoproteinemia, not immune hydrops, or iatrogenic has a _______ fluid
Transudative
Increased pH
Decreased WBC, protein, specific gravity, LDH
223
Pleural effusion due to inflammation or infection has a _______ fluid
Exudative
Decreased pH
Increased WBC, protein, specific gravity, LDH
Low glucose
224
Pathophysiology of chylothorax
Intrauterine thoracic duct obstruction
| --->. Thoracic duct and plural space fistula
225
Laterality of a chylothorax is most often
Right side>> left side >> bilateral
226
Fluid diagnosis of chylothorax shows
Xanthochromia
Lymphocytosis (70%)
Increased protein and triglycerides
227
Optimal feeding preference for patients with chylothorax is
Formula with medium chain triglycerides
228
Cartilaginous deficiency causing disruption of bronchopulmonary development and subsequent ball valve effect with over distention is called
Congenital lobar emphysema
229
Congenital lobar emphysema most commonly affects what patient population and what pulmonary location?
Males >>> females
Left upper lobe, 45%
Right middle lobe 30%
Right upper lobe 20%
230
What other anomaly can be associated with congenital lobar emphysema?
Congenital heart disease
231
What is the typical presentation of congenital lobar emphysema?
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
A branching abnormality of the lung with communication to the tracheobronchial tree is a
Congenital cystic adenomatoid malformation
233
Perfusion and location of CCAMs are typically
Perfused by pulmonary circulation
| Unilateral (80-90%)l
234
The most common type of CCAM is a type
Type 1, 50-70%
235
The most rare type of CCAM are type
Type zero and type four
236
Timing of defect of type 1 2 and 3 CCAM is
Type 1: 7-10 weeks
Type 2: 3 weeks
Type 3: 4 weeks
237
The CCAM most likely to cause a mass effect is a type
Type 3
Can also see compression with type 1 and type 4
238
CCAMs involving ciliated epithelium are
Type 0, type 1, type 2
239
Nonciliated epithelium are found in CCAMs type
Type 3 and 4
240
Mucus cells and cartilage are found only in CCAM type
Type 0 and 1
241
What is the typical progression of CCAMs during gestation?
Occur by the 10th week of gestation, many regress with peak size at 25 weeks gestation
242
Foresee cams that persist after birth, what is the difficult presentation?
50% asymptomatic at birth
Large lesions will cause respiratory distress after delivery
30% will present with recurrent pneumonia in childhood
243
What is the pre and postnatal management of CCAM?
Monitoring for hydrops, fetal surgery if indicated
Supportive care for respiratory distress
Complete resection
244
Which CCAM types have the best prognosis?
Type 1 and 4
245
Which CCAM types have the worst prognosis? And why?
Type 0 and 2, due to associated anomalies
| Type 3 due to mass effect and associated pulmonary hypoplasia and hypertension
246
An airway cystic mass filled that is air filled or solid derived from the foregut is a
Bronchogenic cyst
247
Bronchogenic cysts tend to be located
Posterior to trachea
Can be intraparenchymal
May communicate with GI tract
248
Presentation of bronchogenic cysts
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
Why is surgical excision indicated for bronchogenic cysts even if asymptomatic?
Due to risk of complications including malignant changes
250
Congenital lymphangiectasia most commonly affects
Males, 2:1
| Patients with noonan syndrome and trisomy 21
251
Congenital lymphangiectasia is caused by
Abnormal development or lymphatic obstruction that causes dilation of the lymphatic vessels in the lung
252
Clinical signs of congenital lymphangiectasia include
Respiratory distress after birth
Plural effusions
Hyperinflated lungs and diffuse granular densities on x-ray
253
Non-functional lung tissue that doesn't communicate with the tracheobronchial tree and is perfused by systemic circulation is a
Bronchopulmonary sequestration
254
Most bronchopulmonary sequestrations are located
Intralobar, 75%
Lower lobes
Tend to occur more on left side
255
Are intralobar or extralobar sequestrations more likely to have associated anomalies?
Extralobar, 40 to 60%
256
A bronchopulmonary sequestration that presents in the neonatal period is more likely to be located in a _____ space.
Extralobar
257
Clinical course of bronchopulmonary sequestration is typically
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
Additional anomalies that maybe associated with bronchopulmonary sequestration is
Congenital heart disease,
| Congenital diaphragmatic hernia
259
Mechanism of action of acetazolamide is
Carbonic anhydrase inhibitor, proximal tubule
260
Mechanism of action of furosemide is
Blocks active chloride transport, ascending loop of Henle
261
Mechanism of action of spironolactone is
Aldosterone antagonist, collecting system
262
Mechanism of action of chlorothiazide is
Inhibition of sodium chloride reabsorption, distal tubule
