Restrictive Lung Disease Flashcards
(216 cards)
1
Q
How does restrictive lung disease effect the lungs?
A
Effects lung expansion and compliance
2
Q
What is the hallmark for restrictive lung disease?
A
Inability to increase lung volumes proportionate to increase in alveolar pressure
3
Q
What issues are usually connected to restrictive lung disease?
A
- Connective tissue diseases
- Environmental factors
- Pulmonary fibrosis
- Increased alveolar or interstitial fluid
- Limitation in chest/diaphragm excursion
4
Q
What happens to the lungs in regards to gas exchange in pts with restrictive lung disease?
A
Leads to reduced surface are for gas diffusion, V/Q mismatching, and hypoxia
All lung volumes and reduced
5
Q
What is FEV1/FVC ratio expected to be in a patient with restrictive lung disease?
A
Normal or increased since all lung volumes are low
6
Q
What lung capacity is used to classify restrictive lung disease as mild, moderate, or severe?
A
Total lung capacity (will be reduced in restrictive disease)
7
Q
What decreases in TLC quantify mild, moderate, and severe restrictive lung disease?
A
- Mild: TLC 65-80% of predicted value
- Moderate: TLC 50-65% predicted value
- Severe: TLC <50% of predicted value
8
Q
Extensive list for causes of restrictive lung disease
A
9
Q
What causes pulmonary edema?
A
Increased capillary pressure or capillary permeability leading to fluid leakage into the interstitial and alveolar space
10
Q
Pulmonary edema caused by increased capillary permeability is associated with a high concentration of _________ in the edema fluid
A
Protein
11
Q
What can increased capillary permeability with pulmonary edema cause?
A
Diffuse alveolar damage (associated with ARDS)
12
Q
How does pulmonary edema present on CXR?
A
Bilateral symmetric perihilar opacities
13
Q
What is a newer method to diagnose pulmonary edema?
A
Lung ultrasound
14
Q
When is cardiogenic pulmonoary edema present and what are the symptoms?
A
- Seen with acute decompensated HF
- S/S: Dyspnea, tachypnea, elevated cardiac pressures, SNS activation
more severe SNS activation compared to increased-permeability pulmonary edema
15
Q
How is the heart affected by cardiogenic pulmonary edema?
A
Decreased systolic or diastolic cardiac function
16
Q
What disease processes can worsen cardiogenic pulmonary edema?
A
- Any increases in preload
- Aortic regurg
- Mitral regurg
17
Q
What can increase the risk of developing cardiogenic pulmonary edema?
A
- Any increases afterload or SVR
- HTN
- LC outflow obstruction
- Mitral stenosis
18
Q
What is another term for negative pressure pulmonary edema?
A
Post-obstructive pulmonary edema
19
Q
When does post-obstruction pulmonary edema occur?
A
After the relief of an upper airway obstruction like laryngospasm, epiglottitis, tumors, or OSA
20
Q
What is the MOA of negative pressure pulmonary edema?
A
- Attempted spontaneous ventilation during obstruction creates negative pressure which draws fluid from the alveolar capillaries
- Negative intrapleural pressure decreases the interstitial hydrostatic pressure, increases venous return, and increases left ventricular afterload
Intense SNS activation, HTN, and central displacement of blood volume
21
Q
What is the onset time for post-obstructive pulmonary edema?
A
Onset ranges from a few minutes - 2-3 hours after relief of obstruction
22
Q
What are symptoms of post-obstructive pulmonary edema?
A
- Tachypnea
- Cough
- Desaturation
23
Q
What is the treatment for negative pressure pulmonary edema?
A
- O2
- Maintain patent airway
- Mechanical vent
- Radiographic evidence of NPPE resolves within 12-24 hrs
24
Q
Which patients are at risk for neurogenic pulmonary edema?
A
- Small fraction of acute brain injury pts
- Minutes to hours after injury
25
What is the MOA of neurogenic pulmonary edema?
Massive outpouring of SNS impulses from injured CNS causes gen vasoconstriction and blood volume shift into pulmonary circulation→ increases pulmonary capillary pressure can fluid transfer into interstitium and alveoli
26
What fluid status can lead to injury in pulmonary blood vessels?
Hypervolemia
27
Why does the speed of re-expansion of a collapsed lung matter?
Rapid re-expansion can lead to Re-expansion pulmonary edema
28
What factors effect the risk of re-expansion pulmonary edema after relief of PTX or effusion?
- Amount of air/liquid in pleural space (>L increases risk)
- Duration of collapse (>24 hrs increases risk)
- Speed of re-expansion
29
What is the treatment for re-expansion pulmonary edema?
Supportive care
30
What drugs have high risk of causing drug induced pulmonary edema?
- Opioids (heroin)
- Cocaine
31
If there is high protein concentration in the pulmonary edema fluid what does that suggest?
High permeability pulmonary edema (Protein leaking out)
32
What are the effects of cocaine on the pulmonary system?
- Vasoconstriction
- Acute myocardial ischemia
- Myocardial infarction
33
Can opioid induced pulmonary edema be reversed?
No, Naloxone does not reverse opioid induced pulm edema
34
What is the treatment for drug-induced pulmonary edema?
- Supportive care
- Potential intubation/ vent
35
What is another condition that has similar symptoms as drug induced pulmonary edema?
Diffuse alveolar hemorrhage (DAH) → If pulmonary edema doesnt respond to diuretics DAH is likely
36
What is a right to left intrapulmonary shunt?
Perfusion of nonventilated alveoli
37
What is the onset of high altitude pulmonary edema? What are the causes?
- Onset: gradual→ typically occur within 48-72hrs at high altitude
- Causes: Hypoxic pulmonary vasoconstriction→ increases pulmonary vascular pressure
38
What is the treatment for high-altitude pulmonary edema?
- O2
- Quick descent from high altitude
- inhalation of NO may improve oxygenation
39
What is the net effect of an intrapulmonary shunt?
A decrease in PaO2
40
What does the shunt fraction provide an assessment of?
Assessment of V/Q matching and an estimate of the response to treatments
41
Should elective cases proceed if pt has pulmonary edema?
no, delay case and make effort to optimize cardiorespiratory function before surgery
42
A physiologic shunt is ___% of cardiac output
2-5%
43
Calculation of the shunt fraction obtained when the patient breathes 100% O2 eliminates the contribution of ____?
V/Q mismatching
44
What are considerations to take when determining if the patient can tolerate extubation?
- Pt is alert and cooperative
- Can tolerate spontaneous ventilation without tachypnea, tachycardia or respiratory distress
45
Guidelines for extubation:
- Vital capacity >15 mL/kg
- Alveolar-arterial oxygen difference <350 cmH2O on 100% O2
- PaO2 of >60 mmHg on FiO2 of <0.5
- Negative inspiratory pressure of more than -20 cmH2O
- Normal pHa
- RR <20
- VD:VT of <0.6
46
What are some beneficial vent settings when a patient has pulmonary edema?
- Low VT
- RR 14-18
- End inspiratory pressures <30 cmH2O
- PEEP and inspiratory pausing can optimize lung compliance
47
What are two things that signify an inability to tolerate extubation?
Rapid RR with low TV
48
When a patient appears ready for a trial of vent withdrawal, what 3 options are considered?
1. SIMV, which allows progressively fewer mandatory breaths until patient breathing on their own
2. Intermittent trials of total removal of mechanical support
3. Use of decreasing levels of pressure support ventilation
49
What should the PaO2 and PaCO2 remain when vent weaning?
- PaO2 should remain >60 mmHg on FiO2 <0.5
- PaCO2 should remain <50 mmHg, and the pHa >7.30
50
What is additional criteria for vent weaning to extubation?
- PEEP <5 cmH2O, RR <20 and VC>15 mL/kg
- Pt should be alert, with active laryngeal reflexes and the ability to cough and clear secretions
51
Why is supplemental O2 often needed after extubation?
V/Q mismatching
52
Acute Respiratory Distress Syndrome is caused by ____ and manifests as ______?
Caused by inflammation;
Acute hypoxemic respiratory failure
53
What is associated with the highest risk of ARDS?
Sepsis
54
Symptoms of ARDS:
- Rapid-onset respiratory failure
- Hypoxemia
- CXR findings similar to cardiogenic pulmonary edema
55
In ARDS, proinflammatory cytokines increase alveolar ____ and alveolar ____
Capillary membrane permeability;
Edema
56
Acute ARDS usually resolves, but may progress to what 2 things?
Fibrosing alveolitis with persistent arterial hypoxemia and decreased pulmonary compliance
57
What does breathing typically look like in patient with restrictive lung disease?
- Rapid shallow breathing
- Tachypnea should not be used as the sole criterial for delaying extubation is gas exchange and other assessments are normal
58
Supportive care for ARDS:
- Ventilation
- Antibiotics
- DVT prophylaxis
- Early enteral feeding
59
How does prone positioning help with management of ARDS?
- Prone position exploits gravity and repositioning of the heart to recruit lung units and improve V/Q matching
60
What pt position can help reduce risk of aspiration pneumonitis?
Elevate HOB during intubation and extubation
61
When is ECMO considered for management of ARDS?
With severe hypoxemic/hypercapnic respiratory failure
62
What are symptoms of chemical pneumonitis (aspiration pneumonitis)?
- Abrupt dyspnea
- Tachycardia
- Desaturation
63
What are additional supportive therapies of ARDS management?
- Fluid management
- NMBD
- inhaled nitric oxide
- prostacyclins (PGI2)
- recruitment maneuvers
- surfactant replacement
- glucocorticoids
- ketoconazole
64
Interstitial lung disease is a group of lung pathologies leading to what?
Diffuse parenchymal disease
65
What happens in the lungs when gastric content is aspirated?
Destroys surfactant producing cells and damages the pulmonary capillary endothelium→ leads to increased capillary permeable pulmonary edema and atelectasis
66
What are some examples of interstitial lung disease?
- Sarcoidosis
- Hypersensitivity pneumonia
- Pulmonary langerhans cell histiocytosis
- Pulmonary alveolar proteinosis
- Lymphangioleiomyomatosis
67
If a patient aspirates in a supine position where will this likely be visible on CXR?
- Superior segment of RLL
- CXR may not show aspiration for 6-12 hrs
68
How to patients with interstitial lung disease usually present?
Present with dyspnea and nonproductive cough, ultimately leading to chronic restrictive lung disease
69
Progressive pulmonary fibrosis causes loss of pulmonary vasculature, leading to what?
Pulmonary hypertension and cor pulmonale
70
What are some things you should do if you note aspiration?
- Suction oropharynx
- Turn pt to side
- T-burg position (prevent aspiration once gastric contents are in pharynx)
- Abx if pt symptomatic after 48hrs and + cultures
71
What is sarcoidosis?
Systemic granulomatous disorder involving many tissues, mainly the lungs and thoracic lymph nodes
72
How is sarcoidosis often found?
Often asymptomatic, and identified accidentally on CXR
73
Symptoms of sarcoidosis:
Wheezing, dyspnea and cough
74
What lungs issues are associated with E-cigarette vaping associated lung injury?
- Pneumonia
- Diffuse alveolar damage
- Acute fibrinous pneumonitis
- Bronchiolitis
- Interstitial lung disease
75
Myocardial sarcoidosis may cause ____
dysrhythmias
76
Neurologic sarcoidosis often displays what?
Unilateral facial nerve palsy
77
Laryngeal sarcoidosis occurs in __% and may interfere with ___
5%; intubation
78
What is a classic manifestation of sarcoidosis?
Hypercalcemia
79
Why is ACE activity increased with sarcoidosis?
Because ACE is produced by granuloma cells
80
Which additives have been associated with EVALI?
- THC
- CBD
- Vitamin E acetate
- Nicotine
- Other oils
81
What are S/S of e-cig/vape lung injury?
- Dyspnea
- Coughing
- N/V/D
- Abd pain
- Chest pain
*May be febrile, tachycardia, tachypnea, and hypoxia*
82
Other than increased ACE, what are some other markers of sarcoidosis?
- Serum amyloid A
- Adenosine deaminase
- Serum soluble IL2 receptor
83
How is sarcoidosis diagnosed?
- Kvein skin test is used - similar to TB test
- Tissue or lavage fluid for dx may be obtained by mediastinoscopy and bronchoscopy
84
What does CXR show in patient with E-cig lung injury?
Similar findings to diffuse alveolar damage seen in ARDs
85
What med group can be used in sarcoidosis?
Corticosteroids are used to suppress sx of sarcoidosis and treat hypercalcemia
86
What is the treatment for EVALI?
- Abx
- Systemic steroids
- Supportive Care
87
What are the symptoms of covid induced restrictive lung disease?
- Symptoms range from dyspnea to ventilator dependence and pulmonary fibrosis
88
Advanced pulmonary fibrosis may lead to ___ ___?
pulmonary hypertension
89
What is hypersensitivity pneumonitis?
Interstitial granulomatous inflammation after inhalation of fungus/spores particles
*may present as acute, subacute, or chronic
90
What is the most common finding from covid induced restrictive lung disease?
Reduced diffusion capacity (directly related to the severity of the initial disease process)
91
What are long term complications associated with covid induced restrictive lung disease?
- Decreased exercise capacity
- Hypoxia
- Opacities on CT
92
What characterizes acute respiratory failure?
- Abrupt increase in PCO2 and decrease pH
- PaO2 <60mmHg despite O2 supplementation and absence of Right to left intracardiac shunt
- PaCO2 >50 mmHg without respiratory compensated metabolic alkalosis
93
How does chronic respiratory failure differ from acute respiratory failure?
PaCO2 increased but pH is normal in chronic
*Normal pH reflects renal compensation for respiratory acidosis*
94
What are the 3 treatment goals of ARF?
1) Patent airway
2) Hypoxemia correction
3) Removal of excess O2
95
Which devices can be beneficial in mild to moderate V/Q mismatching?
- NC
- Venturi mask
- NRB
- T-piece
*These devices rarely provide O2 concentrations >50%*
96
What is the next step to maintain oxygenation if NC, Venturi mask, NRB do not maintain PaO2 >60mmHg?
Continuous positive airway pressure (CPAP)
97
How does CPAP increase lung volumes?
Opening collapsed alveoli and decreasing right-to-left intrapulmonary shunting
*CPAP mask may increase risk of aspiration*
98
What PaO2 correlates with SpO2 >90%?
PaO2 >60mmHg
99
Symptoms of hypersensitivity pneumonitis:
- Dyspnea and cough 4-6 hours after inhalation
- Followed by leukocytosis, eosinophilia and hypoxemia
100
What is volume cycled ventilation?
- Fixed tidal volume with inflation pressure as dependent variable
- Pressure limit can be set→ when pressure exceeds this value the pressure relief valve prevents further has flow and triggers alarm to alert provider
101
What does CT show with hypersensitivity pneumonitis?
Ground glass opacities in the mid to upper lung zones
102
How is hypersensitivity pneumonitis diagnosed?
Bronchoscopy, trans-tracheal or bronchial biopsy, and cryobiopsy
103
What could elevated PAP indicate?
- Pulmonary edema
- PTX
- Kinked ETT
- Mucous plug
104
What is a disadvantage to volume cycled ventilation?
Inability to compensate for leaks in the delivery system
105
Repeated episodes of hypersensitivity pneumonitis may lead to what?
Pulmonary fibrosis
106
What are the primary modes of volume cycled ventilation?
- Assist/controlled (AC)
- Synchronized intermittent mandatory ventilation (SIMV)
107
Treatment for hypersensitivity pneumonitis:
- Antigen avoidance
- glucocorticoids
- lung transplant
108
What is pulmonary langerhans cell histiocytosis and what is it strongly associated with?
- inflammation around smaller bronchioles, causing destruction of the bronchiolar wall and surrounding parenchyma
- Strong association with smoking tobacco
109
How does AC ventilation work?
- Set resp rate
- If negative pressure is sensed a VT will be delivered
110
What areas of the lung does pulmonary langerhans cell histiocytosis affect?
Upper and middle zones of the lung
111
How does SIMV ventilation work?
- Allows spontaneous ventilation
- Provides a predefined minute ventilation
- Periodic mandatory breaths that are synchronized with the pts inspiratory efforts
112
What does lung biopsy show in pulmonary langerhans cell histiocytosis?
Inflammatory lesions around the bronchioles containing langerhans cells, eosinophils, lymphocytes and neutrophils
113
What are the advantages of SIMV over AC vent modes?
- Use of respiratory muscles
- Lower mean airway pressures
- Prevention of respiratory alkalosis
- Improved pt ventilator coordination
114
What does CT show with pulmonary langerhans cell histiocytosis?
Cysts or honeycombing in upper zones with costophrenic sparing
115
Treatment for pulmonary langerhans cell histiocytosis:
- Smoking cessation
- Systemic glucocorticoids
- Symptomatic support
116
What is Pressure cycled ventilation (PCV)?
- Provides gas flow until a preset airway pressure is reached
- VT varies with changes in compliance and airway resistance
117
What is pulmonary alveolar proteinosis characterized by?
Lipid-rich protein material in the alveoli
118
What are complications of mechanical ventilation?
- Infection
- Barotrauma
- Atelectasis
119
When does pulmonary alveolar proteinosis usually present? With what symptoms?
In 40-50s with symptoms of dyspnea and hypoxemia
120
What can PAP be associated with?
Chemotherapy, AIDS or inhaled dust
121
_________ is the most important predisposing factor for developing nosocomial pna (VAP)
Intubation
122
How can barotrauma present?
- SQ emphysema
- Pnuemomediastinum
- Pneumoperitoneum
- Pneumopericardium
- Pulmonary interstitial emphysema
- Arterial gas embolism
- Tension pneumothorax
*Reflect passage of air from ruptures alveoli*
123
___________ increases the risk of barotrauma by weakening the pulmonary tissue
Infection
124
How can you tell if pt has atelectasis without CXR?
Hypoxemia from atelectasis will not be responsive to an increase in FiO2
125
What are other differential dx that cause sudden hypoxemia?
- Tension PTX
- PE
*These usually have hypotension as well*
126
How can atelectasis be indentified?
Lung ultrasound (LUS) by bronchogram showing static air
127
Which gradient is useful for evaluating gas exchange, lung function, and distinguishing the cause of arterial hypoxemia?
PAO2- PaO2 gradient
128
What are mechanisms of arterial hypoxemia?
129
When does desaturation of arterial blood occur?
PaO2 <60mmHg
130
What are the 3 main causes of arterial hypoxemia?
- V/Q mismatch
- Right to left pulmonary shunting
- Hypoventilation
131
Increasing FiO2 improves PaO2 for V/Q mismatch and hypoventilation but does not help _____________
Significant Right to left pulmonary shunt
132
What stimulates compensatory responses?
- Acute decrease in PaO2 <60mmHg
- Chronic hypoxemia responses occur when PaO2 <50 mmHg
133
What are the compensatory responses to hypoxemia?
1. Carotid body–induced increase in alveolar ventilation
2. Hypoxic pulmonary vasoconstriction to divert blood flow away from hypoxic alveoli
3. Increased SNS activity to increase cardiac output and enhance tissue oxygen delivery
134
How does chronic hypoxemia improve O2 carrying capacity?
An increase in RBC mass improves O2 carrying capacity
135
What is the Dead space: VT ratio (VD:VT)?
- Reflects the efficacy of CO2 transfer across alveolar capillary membranes
- Indicates areas in the lungs that receive adequate ventilation but inadequate blood flow
- Ventilation to these alveoli is describe as wasted or dead space
136
What is the normal VD:VT?
<0.3 may increase in ≥0.6 when there is an increase in dead space ventilation
137
What causes increased VD:VT?
- ARF
- Decreases in cardiac output
- Pulmonary embolism
138
What does chest xray show with PAP?
Batwing alveolar opacities in middle and lower lung zones
139
Treatment for severe cases of PAP:
Requires lung lavage under GA to remove alveolar material and improve macrophage function
140
Airway management during anesthesia for lung lavage includes:
Double Lumen Tube to lavage each lung separately and optimizing oxygenation during the procedure
141
What is lymphangioleiomyomatosis?
Rare multisystem disease causing proliferation of smooth muscle of the airways, lymphatics and blood vessels
142
What population does lymphangioleiomyomatosis mostly occur?
Women of reproductive age
143
What do PFTs show with lymphangioleiomyomatosis?
Restrictive and obstructive disease with decreased diffusing capacity
144
Symptoms of lymphangioleiomyomatosis:
- Dyspnea
- Hemoptysis
- Recurrent pneumothorax
- Pleural effusions
145
Treatment for lymphangioleiomyomatosis:
Sirolimus (immunosuppressive)
146
What is aging-related restrictive physiology associated with?
What does this lead to?
Decreased chest wall compliance and elastic recoil
Increased RV and decreased VC
147
Geriatric patients breathe at ____ lung volumes with increased ___
higher lung volumes with increased FRC
148
What two things in geriatric patients decrease the efficiency of the diaphragm?
Kyphosis and anteriorposterior diameter of the chest increase
149
How does FEV1 and FVC change with age?
They both decline
150
What are some thoracic extrapulmonary causes of chronic extrinsic restrictive lung disease?
Deformities of the sternum, ribs, vertebrae and costovertebral structures include: ankylosing spondylitis, flail chest, scoliosis and kyphosis
151
Why is work of breathing increased with chronic extrinsic restrictive lung diseases?
D/t abnormal mechanics and increased airway resistance that results from decreased lung volumes
152
Poor ability to cough leads to what?
Recurrent pulmonary infections
153
What are the 2 types of costovertebral skeletal deformities?
Scoliosis and kyphosis
154
When does chronic extrinsic restrictive lung disease d/t costovertebral skeletal deformities start?
Commonly begins in late childhood/early adolescence and may progress during periods of rapid skeletal growth
155
With chronic extrinsic restrictive lung disease d/t skeletal deformities, the severity of respiratory compromise correlates with what?
Degree of spinal curvature
156
What is pectus carinatum?
"pigeon chest" - deformity of sternum characterized by the outward projection of the sternum and ribs
157
What causes pectus carinatum?
Are we concerned about it?
- Cause unknown, runs in families
- Usually more of a cosmetic concer, but may cause respiratory symptoms or asthma
158
What can happen when multiple rib fractures are in a parallel vertical orientation?
Can produce flail chest with paradoxical inward movement of the unstable portion of the thoracic rib cage
159
Symptoms of multiple rib fractures:
- Pain
- Increased work of breathing
- Inability to cough
- Atelectasis
160
Lung contusions reduce what two things?
Chest wall compliance and FRC
161
Treatment for flail chest:
Positive pressure ventilation and stabilization
162
Pleural effusion vs pneumothorax:
Effusion = fluid in the pleural space
Pneumothorax = gas in the pleural space
163
How is pleural effusion diagnosed?
CXR, CT or bedside US (preferred)
164
What causes idiopathic spontaneous pneumothorax?
What population does it occur most in?
- Caused by rupture of apical subpleural blebs
- Occurs in tall, thin men age 20-40
165
What is a tension pneumothorax?
Medical emergency - develops when gas enters the pleural space during inspiration and can't escape during exhalation
166
Symptoms of tension pneumo:
- Respiratory distress
- Tachypnea
- SOB
- Hypoxia
- Pleuritic chest pain
- Tachycardia
- HoTN
167
Which way is the trachea deviated in a tension pneumo?
Away from the pneumothorax
168
How would breath sounds change with a tension pneumo?
Breath sounds are decreased/absent on the side of the pneumo
169
If a patient with a tension pneumo was on a ventilator, what would it show?
Increased airway pressures and decreased Vt
170
Treatment for tension pneumo:
Immediate evacuation through a needle or small-bore catheter placed into the second anterior intercostal space
171
Pleural fibrosis may follow what?
Hemothorax, empyema, surgical pluerodesis
172
What causes acute mediastinitis?
Bacterial contamination after esophageal perforation
173
Symptoms of acute mediastinitis?
Treatment?
- Sx: chest pain and fever
- Tx: broad-spectrum abx and surgical drainage
174
Common anterior mediastinal masses:
- Thymomas (20%)
- germ cell tumors
- lymphomas
- intrathoracic thyroid tissue
- parathyroid lesions
175
Common middle mediastinal masses:
- tracheal masses
- bronchogenic and pericardial cysts
- enlarged lymph nodes
- proximal aortic disease (aneurysm or dissection)
176
Common posterior mediastinal masses:
- Neurogenic tumors and cysts
- Meningoceles
- Lymphomas
- Descending aortic aneurysms
- Esophageal neoplasms
177
Treatment of a mediastinal mass:
Depends on underlying pathology
- may require surgery, radiation, chemo or surveillance
178
Preop considerations for a mediastinal mass:
- Measurement of a flow-volume loop
- Chest imaging
- Assessing for airway compression
179
How is CT useful with mediastinal mass?
Can establish size of the mass and degree of compression
180
How is fiberoptic bronchoscopy useful for a mediastinal mass?
Evaluating the degree of airway obstruction
181
___ is preferred for symptomatic patients with a mediastinal mass requiring a diagnostic tissue biopsy
LA technique
182
What are 3 other thoracic nonpulmonary causes of RLD?
- Asphyxiating thoracic dystrophy
- Fibrodysplasia ossificans
- Poland syndrome
183
What is asphyxiating thoracic dystrophy?
"Jeune syndrome"
- disorder with skeletal dysplasia and multiorgan dysfunction
184
What is asphyxiating thoracic dystrophy associated with?
- Cysts in kidney, pancreas and liver
- Retinal abnormality with short ribs, short limbs, narrow thorax and polydactyly
185
Fibrodysplasia ossificans is a genetic variation in what?
Bone morphogenetic protein
186
What is poland syndrome?
Partial or complete absence of pectoral muscles, commonly affecting one side
187
Why might patients with poland syndrome have paradoxical respiratory motion?
Due to the absence of multiple ribs
188
Neuromuscular disorders that interfere with skeletal respiratory muscles can result in what?
Restrictive lung disease
189
Abnormalities of the spinal cord, peripheral nerves, NMJ or skeletal muscles may result in what?
Restrictive pulmonary defects characterized by an inability to generate normal respiratory pressures
190
In contrast to. thoracic cage disorders, where cough is ____, the expiratory muscle weakness of NM disorders prevents adequate expiratory airflow to provide ___ cough
Preserved; sufficient
191
Patients with severe neuromuscular disorders are dependent on their state of what?
Wakefulness to maintain adequate ventilation
192
During sleep, hypoxemia and hypercapnia may develop and contribute to what?
Development of cor pulmonale
193
What percent of patients with Guillain-Barre syndrome require mechanical ventilation?
How long do they need it for?
- 20-25%
- On average for 2 months
194
Why are muscular dystrophy patients predisposed to pulmonary complications?
- Chronic alveolar hypoventilation occurs d/t inspiratory muscle weakness
- Expiratory muscle weakness impairs cough
- Weakness of swallowing muscles may lead to pulmonary aspiration
195
What devices may be helpful with muscular dystrophy?
Nocturnal ventilation devices
196
Injury above which vertebrae results in diaphragmatic paralysis?
T4
197
What causes diminished Vt in patients with spinal cord injuries?
With diaphragmatic breathing, there is a paradoxical inward motion of upper thorax during inspiration that results in diminished Vt
198
What causes mild bronchial constriction in quadriplegic patients?
What can help with this?
- PNS tone is unopposed by SNS activity from the spinal cord
- Anticholinergic bronchodilators are useful
199
Obesity causes decreases in what lung volumes?
FEV1, FVC, FRC, ERV
200
BMI > ___ leads to a decreased RV and TLC
40 kg/m2
201
With extreme obesity, FRC may exceed ___ ___ and approach RV
Closing volume
202
How does obesity cause restrictive lung disease?
Adipose buildup in the anterior abdominal wall hinders diaphragmatic movement, diminishes basal lung expansion, and causes closure of peripheral lung units
203
What is triggered by obesity-related hypoxemia?
Adipose cells release adipocytokines that play a part in systemic inflammation
204
What 3 things in pregnancy can cause restrictive lung disease?
- Subcostal angle of the rib cage and lower chest wall circumference increase and the diaphragm moves cephalad
- increased levels of relaxin cause stretching of the lower rib cage ligament
- Rib cage circumference expands
205
When do the changes in pregnancy occur that causes RLD?
37th week
206
After pregnancy, when does the chest wall normalize?
What happens to the subcostal angle?
Normalizes about 6 months postpartum, except for the subcostal angle, which remains wider by 20%
207
How much does the enlarging uterus push the diaphragm up by?
4 cm
208
What drugs should be avoided with restrictive lung disease?
Drugs with prolonged respiratory depressant effects
209
What is often needed in patients with impaired pulmonary function?
Postoperative mechanical ventilation
210
RLD also contributes to perioperative ___ ___
Pulmonary complications
211
Pneumothorax occurs in ___% of patients after transbronchial biopsy and ___% after percutaneous needle biopsy
5-10%
10-20%
212
What is the major contraindication to pleural biopsy?
Coagulopathy
213
What is helpful for visualizing the airways and obtaining samples for biopsy and culture?
Fiberoptic bronchoscopy
214
What are the risks of mediastinoscopy?
- Pneumothorax
- mediastinal hemorrhage
- venous air embolism
- RLN injury
215
The mediastinoscope can exert pressure on the right innominate artery, causing what?
Loss of pulses in the right arm and compromise of right carotid artery blood flow
216
With mediastinoscopy, blunt dissection along the pretracheal fascia is performed, and permits biopsy of what?
Paratracheal lymph nodes down to the level of the carina
