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Respiratory Flashcards

1
Q

Fetal lung fluid plays a critical role in lung devleopment. Prior to birth, ~30% of fetal lung fluid is cleared from he alveoli and airways. Of the following, which anion/cation is actively transported across the pulmonary epithelial cells to induce fetal lung absoprtion prior to delivery?

A

Sodium

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2
Q

At 12 hours of age, a term infant devleops unequal chest wall movements during spontaneous breathing with moderate retractions on the L side and mild retractions on the right side. The R chest appears to collapse more with inspiration than the L side. What is the diagnosis?

A

R-sided diaphragmatic paralysis

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3
Q

An infant is born with a TE fistula. At what stage of lung devleopment did this occur

A

Embryonic; other anomalies that can occur during this phase are laryngeal clefts ,tracheal stenosis, and pulmonary sequestration

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4
Q

An infant is born with a CDH. At what stage of lung devleopment did this occur?

A

Pseudoglandular; other abnormalities include braching abnormalities of the lung, bronchogenic cysts, congenital lobar emphysema, cystic adenomatoid malformations. CDH occurs secondary to failure of the pleuro-peritoneal closure

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5
Q

At what stage in lung devlopment can pulmonary hyoplsia occur?

A

canalicular or saccular

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6
Q

True or false: a self inflating bag cannot reliably delivery free flow 100% O2

A

TRUE

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7
Q

What are the physiologic changes seen in a tension PTX?

A

decrease in PaO2, increase in HR and CVP, decrease in arterial BP and pulse pressure, and decrease in cerebral O2 delivery

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8
Q

What can delay alveolarization?

A

antenatal steroids (despite the increased benefit of increased fetal surface area), supplemental O2 (hyper and hypo O2), nutritional deficiencies, mechanical ventilation, insulin, protein kinase C,

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9
Q

How is NO produced in the lungs?

A

in the enothelial cells lining the pulmonary blood vessels, nitric oxide synthase converts L-arginine into nitric oxide; then NO is used by guanylyl cyclase to convert GTP to cGMP which relaxes smoother muscle

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10
Q

What receptor is involved in fetal lung fluid secretion?

A

NA-K-2Cl transporter and Cl channels (ClC2 and ClCN2); inhibited by Bumetanide, beta adrenergics and vasopressin

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11
Q

What drug inhibits ENaC channels?

A

amiloride

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12
Q

What drug inhibits Na-K-ATPase?

A

Ouabain

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13
Q

lung over inflation leads to cessation of inspiration- name the reflex

A

hering Breur inflation reflex, NB>adults

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14
Q

inhibition of the Hering Breur reflex results in extended inspiraton; causes a paradoxical increase in diaphragmatic contraction during inflation

A

paradoxical reflex of head; important during the first few breaths after birth

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15
Q

increased in ventilation rate with a/w abrupt deflation of the lungs

A

hering bruer deflation reflex. Occurs with PTX and ‘sighs”

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16
Q

What is the hallmark of new BPD?

A

alveolar/ capillary hypoplasia

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17
Q

What is the principle lipid in surfactant?

A

saturated phosphatidyl choline (dipalmitoylphosphatidylcholine) that makes up 50% of surfactant and is responsilbe for the formation of the lipid monolayer and thereby reduction in the surface tension.

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18
Q

What is the primary function of SP-B?

A

promotes absorption and speading of surfactant. (SP-C does this too). critical for surfactant function; type II cell functions

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19
Q

Infant presents with severe respiratory failure soon after birth. What surfactnant protein is deficient? What is the genetic mutation most commonly seen in individuals of Northern European desccent?

A

SP-B homozygote deficient (AR); SP-B is encoded by the SFTPB gene on chromosome 2. The most common mutation in Northern Europeans is the framshift mutation (121in22). It accounts for 60-70% of all sFTPB allele mutants identified to date

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20
Q

Name the hypdrophobic surfactant proteins

A

SP-B and SP-C

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21
Q

name the hydrophilic surfactant proteins

A

SP-A and SP-D

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22
Q

This surfactant protein helps regulate surfactant expression and uptake and is also a collectin and is part of the innate immume system; tubular myelin. The most abundant surfactant protein

A

SP-A

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23
Q

This surfactant protein regulates surfactant reuptake and recycling, is a collectin, and is part of the innate immune system; antioxidant

A

SP-D

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24
Q

Name the malformations that can happen in the lung during the embryonic phase

A

atresias (Laryngeal, esophageal, tracheal); bronchogenic cysts, TEF, pulmonary agenesis/aplasia, pulmonary sequestration

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25
Malformations that can occur in the pseudoglandular phase
renal agenesis --> pulmonary hypoplasia, CCAM, pulmonary lymphangiectasia, CDH, tracheomalacia/bronchomalacia
26
malformations that can occur in the cannalicuklar phase
renal dysplasia and pulmonary hypoplasia, alveolar capillary dysplasia, surfacant deficiency (RDS)
27
malformations that can form during the saccular phase
oligohydramnios and pulmonary hypoplasia, alveolar capillary dysplasia, surfactant defiency (RDS)
28
malformations that can happen in the alvelolar phase
lobar emphysema, pulmonary HTN, surfactant deficiency (RDS)
29
mass of abnormal pulmonary tissue that is not connected to the tracheobronchial tree, blood supply from the aorta, does NOT participate in gas exchange,
bronchopulmonary sequestration; intralobar are usually in the LLL and present with recurrent pulmonary infections, extralobar are a/w CDH
30
abnormal budding and braching of the tracheobronchial tree, mostly found in the mediastinal area, can have a check valve so they can expand rapidly causing cardiopulmonary compromise
bronchogenic cyst
31
very rare, presents in 1/3 with resp distress at birth, and 50% with resp distress by 1 monthusually in the L upper/middle lobe of the lung becomes over inflated and causes compression of the other lobes and mediastinum
congenital lobar emphysema; CXR at birth may demonstrate opacification of the affected lobe due to retained FLF but with fluid resoprtion and air entry, hyperinflation manifests; MRI helpful
32
increase risk in patients with Turners or Noonan's syndrome; extremely rare, dilated pulmonary lymphatics or chylothorax,
congenital pulmonry lymphangiectasis; males 2:1; primary form is a/w syndromes, secondary form is a/w CHD (HLHS, cor triatriatum, thoracic duct agenesis, infections like TORCH).
33
CXR: unilateral uniformly dense lung mass, oftne triangular or oval, mostly on the lower lobes
bronchogenic cyst
34
heterogeneous lung disease with cystic changes, areas of atelectasis and possibly PIE
CLD
35
single lesion with one or a few large cysts that may be filled with air or fluid; cysts > 2 cm; accounts for 70% of cases
Type 1 CPAM
36
homogenous patterm of multiple cysts; cyst size > 2 cm; accounts for 20% of cases
type 2 CPAM
37
large, solid homogenos mass usually with shift of mediastinal contents; microcystic adenomatoid solid without cystc elements; can be a/w other anomalies and carries a worse prognosis
Type 3 CPAM
38
CXR: hyperinflated lobe, mediastinal shift, compression of contralateral lung with lung markings to the periphery (in contrast to PTX),
congential lobar emphysema
39
CXR: hyperinflated lungs, diffuse granular densities representing dilated lymphatic vessels, increase risk of pleural effusions
congenital lymphangiectasis
40
CXR : diffuse, patchy, intraparenchymal densities, areas of overdistension.
MAS
41
CXR: obscured diaphragmatic border, lateral decub film with suspected side of effusion down to demonstrate layering
pleural effusions
42
CXR: Spinnaker sail sign because of elevated, well visualied thymus, hyperluceny in the superior retrosternal space
pneumomediastinum
43
CXR: hyperlucency around the entire heart
pneumopericardium
44
CXR: unilateral lung mass, often triangular or oval, can have a cystic component
pulmonary sequestration
45
equation for compliance
change in vol/ change in pressure
46
presents as chronic lung disease of infancy or non-specific interstitial lung disease at a few months of life, AD with gene mutation on chrom 8 that alters protein folding
SP-C deficiency, Tx is lung transplant
47
This protein helps with the transport of lipids; deficiency in this protein presents with refractory acute respiratory failure on the NB (30-40%). Inheritance is AR.
ATP binding cassette transporter protein A3 (ABCA3)
48
Electron microscopy shows small abnormal vesicles with electron dense inclusions; lamellar bodies and tubular myelin in surfactant layer are absent;
ABCA3; also see an abnormal accumulation of lamellar bodies in type II cells, inability to transport it to the surface
49
the primary anti-inflammatory effect of glucocorticoids is mediated by?
annexin A1 synthesis. This results on decreased phosphlipid A2 expression and therefore decreased producton of prostaglandins, thromboxanes, prostacuyclins, and luekotrienes
50
True or False: Chorio can induce lung maturation and decrease RDS, but it may also lead to symptoms of diffuse PNA presenting as respiratory distress
TRUE; chorio and subsequent inflammation can present as diffuse PNA that can clincally mimis RDS but responds poorly to exogenous surfactant
51
new synthetic surfactant that contains a synthetic amino acid peptide consisting of succesive repeats of 1 leucine and 4 lysines designed to mimic the properties of SP-B
lucinactant- but has not been shown to be superior to animal derived surf
52
List the benefits of caffeine
improved resp drive, increased min ventilation and CO2 sensitivity, decreased hypoxic ventilator depression, improved diaphragmatic contractility, improved lung compliance, significant decrease in incidenceof BPD as well as the need for PDA treatment (either medical or surgical)
53
this lesion occurs in 1 in 10,000 and represents 40% of all congenital lung malformations; it typically occurs in the lower lobes and does not have a predilection for R or L side
CPAM
54
type of CPAM that is rare and limted to the upper tracheobronchial tree
type 0 CPAM
55
type of CPAM that has hamartomatous malformation of the distal acini with peripheral cysts;
Type 4 (rare)
56
What is the most promsing adjunct treatment with surfactant for the treatment of RDS?
Inositol
57
What are the effects of caffeiene on the preterm infant for the treatment of apnea?
stimulatiion of the medullary respiratory centers, increase CO2 sensitivity, bronchodilation, enhanced diaphragmatic function, increased minute ventilation, reduced hypoxic respiratory depression. Caffeien has also been shown to stimulate the CNS and CV systems, increase catecholamine secretion, enhancce diuresis, and antagonize protaglandin function'
58
at a constant temperature, a given volume of gas varies inversely to the pressure to which it is subjected; gas flow in normal respiration occurs due to this principle
Boyle's law; pressure varies inversely with volume; P1V1= P2V2
59
the principle that gas expands as it is warmed and shrinks as it is cooled
Charle's law
60
the total pressure exerted by a mixture of gases is equal to the sum of the partial pressure of each gas
Dalton's law
61
the transfer of solute by diffusion is directly proportional to the cross sectional area available for diffusion
Fick's law
62
at a constant termperature, any gas physically dissolves in a liquid in proportion to its partial pressure
Henry's law
63
what respiratory mechanics are increased in neonates compared to adults?
RR, residual vol, minute ventilation (RR x TV), and alveolar minute ventilation [ (TV-dead space) x RR ], oxygen consumption
64
What respiratory mechanics are decreased in neonates compared to adults?
TV, inspiratory capacity, vital capacity, and total lung capacity
65
What respiratory mechanics are the same in neonates and adults?
dead space and FRC
66
resitance equation
change in pressure / flow; (Length x viscosity]/ r^ 4
67
if you increase to FiO2 by 1%, how much should the paO2 increase?
7 torr
68
RUE sat 100% and LLE sat 60%- whats on your DDx?
severe coarctation and interrupted aortic arch or severe systemic hypotension
69
describe the Haldane effect
decreased Hgb saturation with O2 increases its ability to carry CO2, this enhances removal of CO2 from oxygen consuming tissues; increased Hgb saturation decreases its ability to carry CO2; promotes dissociation of CO2 from Hgb in the O2 rich capillaries of the lung
70
What is a transudate?
ph>7.4; WBC > 1,000 and LDH < 200. Chylothorax most common transudate; will also show >80% mononuclear cells (ie lymphocytes) and TG> 100 mg/dL if on enteral feeds
71
What is an exudate?
pH< 7.4; WBC >1000; LDH > 200
72
What is the pathophysiology of apnea of prematurity?
a decreased ventilatory response, irritation of the laryngeal mucosa, hypoxic ventilatory depression
73
What is the classic presentation for reverse differential cyanosis?
D-transposition of the great vessels, concomitantly occurring in the setting of a PDA with either a coarctation/arch interruption or PPHN
74
What are components of the pulmonary innate host defense system?
mucociliary clearance in conducting airways, secreted mucins create a direct host defense system at the epithelial barrier, submucosal glands secrete an array of host defense molecules that trap and kill microbes, tubular myelin provides an extracellular reservoir of surfactant lipids and innate defense proteins
75
What is the imaging of choice for interstitial lung disease?
Chest CT
76
What are the most common causes of diffuse lung disease in neonates?
CF, immune deficiency syndromes, pulmonary infections, BPD, ciliary dyskinesia; once these are ruled out, the baby likely has a interstial lung disease syndrome and needs genetic testing
77
What is the most common cause of nasal obstruction in neontes?
choanal atresia; its more commonly unilateral, more common in females, most cases are sporadic; temprary treatment is an oral airway, long term treatment is surgery. Confirm diagnosis with CT
78
What does CHARGE stand for?
coloboma of the eye, heart defects, atresia of the choanae, retardation of growth/development, GU abnormalities, ear anomalies (including deafness)
79
deoxygenation of blood increases it ability to carry CO2- name the effect
Haldane effect
80
increased pCO2 leads to more unloading of O2 from Hgb describes what effect?
Bohr effect
81
What lobes of the lungs does congenital lobar emphysema affect?
upper lobes, and affects the left upper lobe in 40-50% of patients; right middle lobe is the next most common lobe
82
What is the most common location for bronchopulmonary sequestration?
intralobar in 75%o of cases; anomalous systemic arterial blood supply; does NOT communicate with the tracheobronchiole tree, has a pleural covering, and occurs most commonly in the lower lobes, particularly the Left
83
What the most common location for bronchogenic cysts?
mediastinum in 85% of patients; if they occur in the lungs, they are tyically located in the lower lobes with no R or L predilection; they are thin walled cyst with an epithelial lining and do NOT communicate with the bronchial tree
84
This stage of lung development is characterized by braching morphogensis and formation of the conducting airways and terminal bronchioles. Type II pneumocytes are undifferentiated and plueroperitoneal folds close during this stage. Occures between 7-17 weeks
pseudoglandular
85
this stage of lung development is characterized by terminal branching results in the formation of respiratory bronchioles, alveolar ducts, and primitive alveoli. There is also differentialion of type II pneumocytes with emergence of lamellar bodies
canalicular stage; occurs during weeks 16-27
86
This stage of lung development is characterized by enlargement of the peripheral airways, saccule formation with thinning of the alveolar wall and surfactant production in type II pnuemocytes
saccular stage, occurs 28-36 weeks
87
This stage of lung development is characterized by further thinning of the alvelolar wall, secondary septation, and formation of the definitive alveoli resulting in exponential increase in the lung surface area for gas exchange
alveolar stage
88
What percent of surfactant is lipids?
90 % and include phospholipids and neutral lipids
89
What drugs inncrease the elimation of caffeine?
phenobarb and phenytoin; will need to increase to caffeine dose
90
What drugs decrease elimination of caffeine?
cimetidine and ketoconazole
91
What 3 syndromes are a/w congenital pulmonary lymphangiectasia?
T21, Noonan's and Ulrich-Turner
92
What are the radiographic findings for "New BPD"?
hyperinflation and minimal cystic emphysema
93
What is the distinguishing feature of new BPD?
less Pulm HTN
94
what is a/w piriform aperture stenosis?
holoprosencephaly
95
Maternal sedation is a risk factor for what?
TTN
96
What is the Bohr equation?
Volume of CO2 in expired air = volume of CO2 from dead space + volume of CO@ from the alveoli that are both ventilated and perfused
97
Define physiologic dead space
alveolar dead space + anatomic dead space
98
What are the effects of maternal corticosteroid administration on the fetus?
decreased fetal breathing and fetal movements. The fetal heart rate shows less variability, fetal blood flow is not affected. These changes are transient and the fetus returns to baseline within 7 days after corticosteroid administration

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