NEURO-musculoskeletal dz Flashcards
(235 cards)
1
Q
What is the pathology of myasthenia gravis
A
Autoimmune dz
IgG antibodies destroy post-junctional nicotinic, ACh receptors at the NMJ
2
Q
What is deficient in patients with myasthenia gravis
A
Nicotinic receptors
There is plenty of ACh
3
Q
What is a key feature of myasthenia gravis
A
Skeletal muscle weakness that becomes WORSE LATER in the day or with exercise
Periods of rest allow skeletal muscle function recovery
4
Q
Which organ plays a key role in myasthenia gravis
A
Thymus gland
5
Q
What are 5 symptoms of myasthenia gravis
A
- Diplopia
- Ptosis
- Bulbar muscle weakness (dysphagia, dysarthria)
- Dyspnea on exertion
- Proximal muscle weakness
6
Q
What are 5 situations that exacerbate myasthenia gravis symptoms
A
- Pregnancy
- Infection
- Electrolyte abnormalities
- Surgical and psychological stress
- Aminoglycoside abx
7
Q
How does myasthenia gravis affect a neonate
A
Anti-AchR IgG antibodies cross the placenta and cause weakness in up to 20% of neonates for 2 - 4 weeks
8
Q
Considerations for neonates of parturients with myasthenia gravis
A
Neonate may require airway mgmt
9
Q
What is the first line medication used for myasthenia gravis
A
Anticholinesterases
-Pyridostigmine
10
Q
How can the cause of acute weakness for a patient with myasthenia gravis who is taking pyridostigmine be determined
A
Give edrophonium 1 - 2 mg IV
- If weakness worsens, pt has cholinergic crisis
- I muscle strength improves, the patient has myasthenic crisis
11
Q
A patient has increased weakness when edrophonium is given to determine the cause of myasthenia weakness while taking pyridostigmine. Why is weakness worsening and what should be given
A
Patient has anticholinergic crisis caused by increased ACh at the NMJ in the presence of an anticholinesterase
Patient should be given an anticholinergic medication
12
Q
What medications are used for immunosuppression in myasthenia gravis
A
corticosteroids
cyclosporine
azathioprine
mycophenolate
13
Q
Surgical procedure indicated for pts with myasthenia gravis
A
Thymectomy = reduces circulating anti-AchR IgG
14
Q
Additional treatment for myasthenia gravis
A
Plasmapheresis can provide relief during myasthenic crisis before thymectomy
15
Q
What test is used to distinguish between cholinergic crisis and myasthenic crisis
A
Tensilon test (edrophonium 1-2 mg IV)
16
Q
How are patients with myasthenia gravis affected by nondepolarizing and depolarizing NMBD
A
Nondepolarizer = INCREASED sensitivity Depolarizers = DECREASED sensitivity (resistant)
17
Q
Why do patients with myasthenia gravis have altered responses to NMB drugs
A
D/t fewer nicotinic receptors (type-m) at the NMJ
- resistant to succinylcholine
- increased sensitivity to nondepolarizers
18
Q
How should the dose for nondepolarizing NMBD be adjusted in patients with myasthenia gravis
A
Potency is increased
Reduce dose by 1/2 or 1/3 and monitor response with nerve stimulator
19
Q
How should the dose for succinylcholine be adjusted in patients with myasthenia gravis
A
Potency decreased
If RSI is required, increase dose to 1.5 - 2.0 mg/kg
20
Q
How does pyridostigmine use in patients with myasthenia gravis affect succinylcholine metabolism
A
Pseudocholinesterase efficacy is impaired
Succs duration is prolonged
21
Q
In myasthenia gravis pts, what is a primary postoperative concern
A
If NMB drugs was used, there’s a higher risk of residual neuromuscular blockade
22
Q
What are postoperative patients with myasthenia gravis at increased risk for
A
Pulmonary aspiration d/t bulbar muscle weakness
23
Q
What are 5 factors that increase the risk of postoperative mechanical ventilation in myasthenia gravis pts
A
- Dz duration >6 years
- Daily pyridostigmine >750 mg/day
- Vital capacity <2.9 L
- COPD
- Surgical procedure: median sternotomy > transcervical thymectomy
24
Q
What is Eaton-Lambert syndrome
A
Disorder of NMJ resulting in skeletal muscle weakness
25
Other names for Eaton-Lambert syndrome
1. Myasthenic syndrome
| 2. Lamber-eaton myasthenic syndrome (LEMS)
26
Causes of Eaton-Lamber Syndrome
IgG-mediated destruction of presynaptic voltage-gated
27
Pathophysiology of Eaton-Lambert Syndrome
Presynaptic VG Ca++ channels action is limited, reducing the amount of Ach that is released into the synaptic cleft
28
What is the difference between myasthenia gravis and Eaton-Lamber syndrome
MG = deficient post-synaptic nicotinic receptors d/t destruction. Normal Ach quantity
ELS = deficient Ach d/t presynaptic VG Ca++ channel destruction. Normal nicotinic Ach receptor quantity
29
How does Eaton-Lambert weakness present
Proximal muscle weakness
| Generally worse in the morning, better throughout the day as Ach concentration increases
30
Pulmonary consequences of Eaton-Lambert Syndrome
Respiratory musculature and diaphragm become weak
31
3 Additional Eaton-Lambert Syndrome symptoms, aside from weakness
1. Autonomic nervous system dysfunction causes orthostatic HoTN
2. Slowed gastric motility
3. Urinary retention
32
What is the primary treatment for Eaton-Lambert Syndrome
| MOA
3,4-diaminopyridine (DAP)
| MOA = increases Ach release from presynaptic nerve terminal and improves contraction strength
33
Are anticholinesterases used for Eaton-Lamber Syndrome? Why or why not?
NO they are not helpful
| because it does not increase Ach release
34
How are pts with Eaton-Lamber Syndrome affected by NMBD
They are sensitive to BOTH succinylcholine and nondepolarizers
35
How should doses for neuromuscular blockers be adjusted in patients with Eaton-Lambert Syndrome
Doses should be reduced for both succinylcholine and nondepolarizers
36
How does Eton-Lamber Syndrome affect the dosing of NMB reversal
Reversal with anticholinesterases may be inadequate despite proper dosing
37
What condition is associated with Eaton-Lambert Syndrome
60% of ELS pts have small-cell carcinoma of the lung (oat-cell carcinoma)
38
A patient with lung cancer is undergoing mediastinoscopy. What associated condition could they possibly have that affects the anesthetic
Eaton-Lambert syndrome
39
What is Guillain-Barre Syndrome
An acute idiopathic polyneuritis
| Characterized by an assault on myelin in the peripheral nerves
40
What is the pathophysiology of Guillain-Barre Syndrome
The action potential can't be conducted so the motor endplate never receives the incoming signal
41
What is the clinical presentation of Guillain-Barre Syndrome
Preceded by flu-like illness by 1-3 weeks
GBS persists for ~2 weeks
Full recovery in ~4 wks
42
What are the 3 most common etiologies of GBS
1. Campylobacter jejuni bacteria
2. Epstein-Barr Virus
3. Cytomegalovirus
43
How does weakness progress in patients with Guillain-Barre Sundrome
1. Flaccid paralysis begins in the distal extremities
| 2. Ascends bilaterally towards the proximal extremities, trunk, and face
44
How are the upper airway and ventilation affected by Guillain-Barre Syndrome
Intercostal muscle weakness impairs ventilation
Facial and pharyngeal weakness causes difficulty swallowing
45
What additional sensory deficits accompany Guillain-Barre Syndrome
Paresthesias
Numbness
Pain
46
How is autonomic function affected by Guillain-Barre Syndrome
Dysfunction is common
- Tachy or brady
- HTN or HoTN
- Diaphoresis or anhidrosis
47
What is the treatment for Guillain-Barre Syndrome
Plasmapheresis and IV IgG
48
Which 2 medication classes have no improvement on Guillain-Barre
1. Steroids
| 2. Interferon
49
What are the 3 major anesthetic concerns in the patient with Guillain-Barre
1. Skeletal muscle denervation
2. Impaired ventilation
3. Autonomic dysfunction
50
Which NMB drug should be avoided in Guillain-Barre and why
Succinylcholine
| Risk for hyperkalemia from proliferation of extrajunctional Ach receptors
51
How are patients with Guillain-Barre affected by depolarizing NMB drugs
Increased sensitivity
52
Why is aspiration risk increased in Guillain-Barre
Facial and pharyngeal muscle weakness causes difficulty swallowing and protecting airway, increasing the risk of aspiration
53
Postoperative considerations in the Guillain-Barre patient
May require mechanical ventilation
54
What type of monitoring may be necessary in patients with Guillain-Barre and why
Arterial-line BP
| Pts with autonomic dysfunction are at risk for hemodynamic instability
55
Describe the response a patient with Guillain-Barre can have to indirect-acting sympathomimetics
Exaggerated response d/t upregulation of postjunctional adrenergic receptor
56
What vascular complication are patients with GBS at risk for
DVT
57
Are steroids used in GBS
No, steroids aren't useful
58
What is another name of Guillain-Barre Syndrome
Acute idiopathic polyneuritis
59
What is familial periodic paralysis
Paralysis characterized by acute episode of skeletal muscle weakness accompanied by changes in serum potassium concentration
60
What are the 2 variants of familial periodic paralysis
Hypokalemia
| Hyperkalemia
61
How does familial periodic paralysis differ from other neuromuscular diseases
1. It's a disorder of the skeletal muscle membrane (reduced excitability)
2. It's not a dz of the NMJ
62
What abnormality is hypokalemic periodic paralysis associated with
Ca++ channelopathy
63
What ion channel abnormality is hyperkalemic periodic paralysis associated with
Na+ channelopathy
64
How is hypokalemic periodic paralysis diagnosed
Skeletal muscle weakness following glucose-insulin infusion which decreases serum K+
65
how is hyperkalemic periodic paralysis diagnosed
Skeletal muscle weakness following oral K+ administration which increases serum K+
66
What is the treatment for familial periodic paralysis
Acetazolamide for both forms of the disease
67
What is the mechanism of acetazolamide action when treating familial period paralysis
It creates a non-anion gap acidosis, which protects against hypokalemia
Facilitates K+ excretion, which guards against hyperkalemia
68
What are intraoperative anesthetic considerations for patients with familial period paralysis
1. Avoid hypothermia at all cost (even during CPB)
| 2. Monitor serum K+
69
What 4 medications should be avoided in hypokalemic period paralysis
1. Glucose containing solutions
2. Potassium wasting diuretics
3. Beta-2 agonist
4. Succinylcholine
70
What 2 medications should be avoided in hyperkalemic periodic paralysis
Succinylcholine
| K+ containing solutions (LR)
71
Which neuromuscular blocking drugs are safe for use in hypokalemia and hyperkalemic periodic paralysis
Succinylcholine should be avoided, especially in hyperkalemic periodic paralysis
nondepolarizing drugs are safe, in lower doses
72
What are 2 classes of drugs that induce malignant hyperthermia
1. Halogenated anesthetics
| 2. Depolarizing NMBD
73
What is malignant hyperthermia
Disease of skeletal muscle, characterized by disordered calcium homeostasis
74
What is the pathophysiology of malignant hyperthermia
1. Ca++ Enters the cell and activates the defective ryanodine receptor
2. The sarcoplasmic reticulum releases too much Ca++ into the cell
3. Increased Ca++ engages with contractile elements
4. Increased Ca++ needs to be returned to SR via SERCA2 pumps
5. This process uses a lot of ATP and O2
6. Increased CO2 production plus K+ and myoglobin waste is released
75
What are 7 consequences of increased intracellular Ca++ in the myocyte
1. Rigidity from sustained contraction
2. Accelerated metabolic rate and depletion of ATP
3. Increased O2 consumption
4. Increased CO2 and heat production
5. Mixed respiratory and lactic acidosis
6. Sarcolemma breaks down
7. Potassium and myoglobin leak into the systemic circulation
76
What are 3 co-existing diseases associated with MH
1. King-Denborough syndrome
2. Central core disease
3. Multiminicore disease
77
Are patients with Duchenne muscular dystrophy at risk for MH
No
An MH-like syndrome is associated with Duchenne MD but it is due to rhabdomyolysis
MD pts have normal RyR1 receptors
Dantrolene does not treat the condition
78
What are risk factors of MH
1. Geography
2. Male sex
3. Youth
79
What is the most sensitive indicator of MH
EtCO2 that rises out of proportion to minute ventilation
80
When does MH occur
Up to 6 hours after exposure to a triggering agent
81
What are 5 early signs of MH
1. Tachycardia
2. Tachypnea
3. Masseter spasm
4. Warm soda lime
5. Irregular heart rhythm
82
What are 5 late signs of MH
1. Muscle rigidity
2. Cola-colored urine
3. Coagulopathy
4. Irregular heart rhythm
5. Overt hyperthermia
83
What is the definition of masseter muscle rigidity
A tight jaw that cannot be opened
84
How is masseter muscle rigidity affected by NMB drugs
Spasm is due to increased Ca++ in myoplasm
| Since the site of action is distal to the NMJ, a NMB will not relax the jaw
85
What is the gold standard for diagnosing MH
Caffeine-halothane contracture test, which requires a live muscle biopsy sample
86
What are possible differential diagnoses to consider apart from MH
1. Thyroid storm
2. Malignant neuroleptic syndrome
3. Sepsis
4. Pheochromocytoma
5. Serotonergic syndrome
6. Metastatic carcinoid
7. Cocaine intoxication
87
What are preventative measures taken in the OR prior to an MH pt
1. Anesthesia machine must be flushed with high flow O2 for 100 minutes
2. All external parts should be removed and replaced
3. Vaporizers should be removed
88
What 10 interventions are warranted when MH is suspected
1. Discontinue triggering agent
2. Initiate TIVA
3. Call for help and notify surgeon to stop
4. Hyperventilate w/ 100% FiO2 at 10 L/min
5. Apply charcoal filters to inspiratory and expiratory ports
6. Exchange circuit and reservoir bag
7. Give dantrolene
8. Cool patient
9. Treat acidosis
10. Maintain UO 2 mL/kg/hr
89
What is the dose for dantrolene
2.5 mg/kg IV q 5-10 minutes
90
What are the 2 MOAs of Dantrolene
1. Reduces Ca++ release from RyR1 receptor in skeletal myocyte
2. Prevents Ca++ entry INTO myocyte, which reduces the stimulus of Ca-induced Ca++ release
91
How is dantrolene supplied
As 20 mg vial with 3 g of mannitol
| Must be reconstituted with 60 mL of preservative-free H2O NOT saline
92
What drug class is dantrolene
muscle relaxant
93
What are common side effects of dantrolene
Muscle weakness
| Venous irritation
94
What are 11 steps in treating MH
1. DC triggering agent
2. Call for help
3. Hyperventilate with 100% O2 at FGF 10 L/min
4. Apply charcoal filter and new circuit
5. Administer dantrolene or Ryanodex
6. Cool patient
7. Correct lactic acidosis
8. Treat hyperkalemia
9. Protect against dysrhythmias
10. Maintain UO >2 mL/hr
11. Check coagulation status
95
How is anesthesia maintained once MH is identified
Start TIVA and stop volatile anesthetic
96
What are 3 benefits of hyperventilation with 100% O2 at 10 L/min once MH is identified
1. Facilitates CO2 elimination
2. Enhances O2 delivery
3. Drives K+ into cells
97
When is dantrolene d/c'd
Once symptoms of hypermetabolism subside
98
What is the continued dose of dantrolene in the ICU
1 mg/kg q6 hrs
OR
0.1-0.3 mg/kg/hr for 48 - 72 hrs
99
When should the diagnosis of MH be reconsidered?
If >20 mg/kg of dantrolene has been used
100
What are 3 methods to aid in cooling the MH pt
1. Cold IVF
2. Cold fluid lavage of stomach and bladder
3. Ice packs
101
How can lactic acidosis be addressed in the MH pt
Sodium bicarb 1 - 2 mEq/kg
| titrate to ABG base deficit
102
What are 3 methods of treating hyperkalemia in the MH pt
1. CaCl 5 - 10 mg/kg IV
2. Insulin 0.15 units/kg + D50 1 mL/kg
3. Hyperventilation
103
How do you protect against dysrhythmias in a pt with MH
Give class 1 antiarrhythmics
1. Procainamide 15 mg/kg
2. Lidocaine 2 mg/kg
104
What medication should be avoided when administering dantrolene and why
CCBs
| They can precipitate life-threatening hyperkalemia
105
Why should UO be maintained >2 mL/kg/hr
Protect against renal injury from free myoglobin
106
In a pt with MH, what are 3 methods to maintain UO >2 mL/kg/hr
1. IV hydration
2. Mannitol 0.25 g/kg IV
3. Furosemide 1 mg/kg IV
107
What is Duchenne muscular dystrophy
An x-linked, recessive dz that results from the absence of dystrophin protein
108
What is dystrophin
Critical structural component of the cytoskeleton of skeletal and cardiac muscle cells
It helps anchor actin and myosin to the cell membrane
109
What happens in the absence of dystrophin
The sarcolemma is destabilized during muscle contraction and increases membrane permeability
110
What is released from the myocyte when dystrophin is not present
1. creatine kinase
| 2. myoglobin
111
How is Ca++ affected by the absence of dystrophin
Ca++ can freely enter the cell and activate proteases that destroy the contractile elements, causing inflammation, fibrosis, and cell death
112
Should pts with DMD be given succinylcholine?
| Why or why not
NO
Because the absence of dystrophin allows extrajunctional nicotinic receptor to populate the sarcolemma, predisposing the pts to severe hyperkalemia
113
Why does succinylcholine have a black box warning for children
It can cause cardiac arrest and sudden death d/t hyperkalemia in undiagnosed children with skeletal muscle myopathy
114
What are 5 pulmonary considerations for patients with Duchenne muscular dystrophy
1. Kyphosis causing restrictive lung dz
2. Decreased pulmonary reserve
3. Increased secretions
4. Increased risk of PNA
5. Respiratory muscle weakness
115
What are 3 cardiac considerations for patients with Duchenne muscular dystrophy
1. Degeneration of cardiac muscle
2. S/Sx of cardiomyopathy
3. Thorough preop cardiac workup
116
How does degeneration of the cardiac muscle affect patients with Duchenne muscular distrophy
1. Reduces contractility
2. Leads to papillary muscle dyfxn
3. Mitral regurg
4. Cardiomyopathy
5. CHF
117
What s/sx of cardiomyopathy may be present in patients with Duchenne muscular dystrophy
1. Resting tachycardia
2. JVD
3. S3/S4 gallop
4. Displaced PMI
118
What should be included in a preop work-up in pts with DMD
1. EKG
2. Echo
3. cardiac MRI
119
What EKG changes may be present in pts with DMD
1. Sinus tachycardia
2. Short PR interval
3. Posterobasal cardiac scarring
- -Increased R wave in lead I
- -Deep Q wave in limb leads
120
What gastric abnormalities should be considered in pts with DMD
Increased risk of aspiration d/t:
1. Impaired airway reflexes
2. GI hypomotility
121
What medications should be avoided in pts with MD
1. Succinylcholine
| 2. Volatile anesthetics
122
Define scoliosis
It is a lateral and rotational curvature of the spine and ribcage
123
What is kyphoscoliosis
A posterior curvature of the spinal column
124
What are 5 etiologies of scoliosis
1. Idiopathic (80%)
2. Congenital
3. Myopathic (MD)
4. Neuropathic (CP)
5. Traumatic
125
What is the Cobb angle in scoliosis
It describes the magnitude of the spinal curvature
126
How is the Cobb angle measured
1. The 2 most displaced vertebrae are identified on top and bottom
2. A parallel line is drawn to each
3. A perpendicular line is drawn to each
4. The angle where they intersect is the Cobb angle
127
```
Describe the significance at for each Cobb angle
40-50
60
70
100
```
```
40-50 = surgery indicated
60 = decreased pulm reserve
70 = pulm symptoms present
100 = Gas exchange significantly impaired with high risk of PPC
```
128
What type of pulmonary defect occurs with scoliosis
Restrictive ventilatory defect compresses the lungs
129
What are early signs of altered pulmonary mechanics in pts with scoliosis
1. Decreased FEV1 and FRC
2. Decreased chest wall compliance
3. Normal FEV1/FVC ratio
130
List the volumes and capacities that are decreased during early respiratory complications of scoliosis
1. VC
2. TLC
3. FRC
4. RV
131
What are 7 late respiratory complications of scoliosis
1. V/Q mismatching
2. Hypoxemia
3. Hypercarbia
4. Pulm HTN
5. Reduced response to hypercapnia
6. Cor pulmonale
7. Cardiorespiratory failure
132
What 6 CV changes occur as a result of severe scoliosis
1. RV hypertrophy d/t increased PVR
2. RV strain
3. RA enlargement
4. MV prolapse
5. MV regurg
6. Coarc of aorta
133
What are 2 airway complications r/t the prone position and scoliosis pts
1. Upper airway edema
| 2. ETT kink or migration
134
What is a possible neck complication r/t prone positioning in scoliosis pts
Neck rotation vertebral compression cerebral hypoperfusion
135
What are 3 possible ocular complications r/t prone positioning in scoliosis pts
1. Ischemic optic neuropathy
2. Central retinal artery occlusion
3. Corneal abrasion
136
What are 2 possible upper extremity complications r/t prone positioning in scoliosis pts
Brachial plexus injury
| Ulnar n. injury
137
What are 3 possible lower extremity complications r/t prone positioning in scoliosis pts
1. Pressure on iliac crest = lateral femoral cutaneous n. injury
2. Pressure lateral to fibula = peroneal n. injury
3. Hip flexion = femoral vein occlusion and DVT
138
What are the complications from increased abdominal pressure r/t prone positioning in scoliosis pts
1. Pressure transmitted to veins that drain the spine increase bleeding
2. Reduced pulmonary compliance
139
When can you assume a pt with scoliosis will need postop mechanical ventilation (respiratory value)
When VC<40% predicted
140
What 3 pulmonary measure should be assessed preoperatively in the scoliosis pt
1. Exercise tolerance
2. ABG
3. VC
141
How is intubation complicated in scoliosis pts
If a pt has cervical scoliosis, intubation can be difficult
142
Is the use of N2O recommended
N2O increased PVR which is usually already high in scoliosis pts
Avoid N2O
143
What airway alteration may need to occur with scoliosis thoracic corrections higher than T8
1. May require OLV
| 2. Possible DLT or bronchial-blocker
144
Scoliosis surgery considerations r/t blood loss
Prepare for significant blood loss
1. large bore IV access or CVC
2. Type and x-match
3. cell-saver
4. autologous donation
145
Hypotension to reduce blood loss during scoliosis surgery can carry what risk
MAP < 60 mmHg increases risk of cerebral hypoperfusion and ION
146
How is end-organ perfusion monitored during scoliosis surgery
1. ABG (d/t risk of metabolic acidosis)
| 2. UO
147
What warming measures are taken during scoliosis surgery
1. Active rewarming with forced air warmer
| 2. Fluid warmers
148
What venous risks are possible in patients undergoing scoliosis surgery
How can it be identified
Venous air embolism
Dead space is increased and observed as a reduction in EtCO2 (with and increased PaCO2 - EtCO2 gradient)
149
What are 6 risks of the wake-up test during scoliosis surgery
1. Vascular access removal
2. Tracheal extubation
3. Awareness
4. Pain
5. Air embolism
6. Damage to surgical instrumentation
150
What pathway and vessels does SSEP monitor
```
Pathway = Posterior spinal cord (dorsal column pathway)
Vessels = posterior spinal arteries
```
151
Does NMB drugs interfere with SSEP or MEP
```
SSEP = no
MEP = yes
```
152
What pathway and vessel does MEP monitor
```
Pathway = anterior spinal cord
Vessel = anterior spinal artery
```
153
What 3 ways does rheumatoid arthritis impact the airway
1. TMJ = limited mouth opening
2. Cricoarytenoid joints = decreased glottic opening
3. Cervical spine = AC sublux w/ flesion; ltd extension
154
What symptoms may be present with cricoarytenoid arthritis
| What can these symptoms result in
1. Hoarseness
2. Stridor
3. Dyspnea
Result = airway obstruction
155
What airway management adjustments should be made in the pt with rheumatoid arthritis
Smaller endotracheal tube
156
What is the cause of AO subluxation in RA pts
D/t weakness of transverse axial ligament, which allows the odontoid process to directly compress the spinal cord at the level of the foramen magnum
157
What are RA pts at risk for with AO subluxation
Quadriparesis or paralysis
158
How is AO subluxation assessed and diagnoses
Assessed with lateral xray of c-spine
| AO sublux = distance between anterior arch of atlas and odontoid process is >3 mm
159
What are treatments for c-spine instability in RA pts
1. Surgical correction with odontoid decompression and posterior cervical fusion
160
What is the most common airway complication of RA
AO subluxation and separation of the AO articulation
161
What is rheumatoid arthritis
An autoimmune dz that targets the synovial joints
162
How does RA affect the entire body
There's widespread systemic involvement d/t infiltration of immune complexes into the small and medium arteries leading to vasculitis
163
What mediator plays a central role in the pathogenesis of RA
Cytokines -- TNF and interleukin-1
164
What are hallmark signs of RA
Morning stiffness that generally improves with activity
| Painful, swollen, and warm joints
165
How are lymph nodes affect in RA
Node enlargement in the cervical, axillary, and epitrochlear regions
166
What joints are typically affected by RA
The proximal interphalangeal and metacarpophalangeal joints in the hands and feet
167
What are possible eye complications r/t RA
Sjorgen's syndrome = dry eyes prone to corneal abrasions
168
What are possible nervous system complications of RA
Peripheral neuropathy d/t nerve entrapment
169
What are possible endocrine complications of RA
Adrenal insufficiency and infection d/t chronic steroid use
170
What are possible renal complications of RA
Renal insufficiency d/t vasculitis and NSAIDs
171
What are 2 possible pulmonary complications of RA
1. Pleural effusion
| 2. Restrictive pulmonary issues
172
Why are pts with RA prone to restrictive pulmonary issues
1. Diffuse interstitial fibrosis
| 2. Costochondral involvement limiting chest wall expansion
173
What are 5 possible cardiac complications of RA
1. Pericardial effusion or tamponade
2. Restrictive pericarditis
3. Aortic regurg
4. Valvular fibrosis
5. Coronary artery arteritis
174
What are possible GI complications of RA
Chronic NSAID and steroid use effect on GI tract
175
What are 2 hematologic complications of RA
1. Anemia
| 2. Plt dysfxn d/t NSAID use
176
What 3 laboratory tests are altered in pts with RA
| Are they increased or decreased
```
Rheumatoid factor (anti-Ig antibody) = INCREASED
C-reactive protein = INCREASED
Erythrocyte sedimentation rate = INCREASED
```
177
What is the medication management of RA
1. Antirheumatics
2. Glucocorticoids
3. NSAIDs
178
How do antirheumatic drugs improve RA symptoms
They inhibit TNF interleukin-1 and -6. They also inhibit T cells and B lymphocytes
179
What risks are carried with taking antirheumatic drugs
Increased risk for infection and cancer d/t suppression of the immune system
180
What are 3 examples of antirheumatic drugs used for RA
Methotrexate, cyclosporine, etanercept
181
If a pt is taking cyclosporine for RA, how does this affect depolarizing NMB
Succinylcholine duration is prolonged
182
What methotrexate effects should be considered in RA pts
Liver dysfunction
| Bone marrow suppression
183
What is systemic lupus erthematosus
An autoimmune dz characterized by the proliferation of antinuclear antibodies
184
What are the consequences of SLE
consequences occur in nearly every organ d/t antibody-induced vasculitis and tissue destruction
185
What are the 2 most common problem associated with SLE
1. Polyarthritis
| 2. Dermatitis
186
What are 3 possible airway complications r/t SLE
Cricoarytenoiditis causing
- Hoarseness
- Stridor
- Airway obstruction
187
What are 3 possible nervous system complications associated with SLE
1. Stroke
2. Psychosis/dementia
3. Peripheral neuropathy
188
What is a renal complication associated with SLE
Nephritis with proteinuria
189
What are 5 pulmonary complications associated with SLE
1. Restrictive ventilatory defect
2. Pulmonary HTN
3. Interstitial lung dz w/ impaired diffusion capacity
4. Pleural effusion
5. Recurrent pulmonary emboli
190
What are 5 cardiovascular complications associated with SLE
1. Pericarditis
2. Raynaud's phenomenon
3. HTN
4. Conduction defects
5. Endocarditis
191
What are 5 hematologic complications associated with SLE
1. Antiphospholipid antibodies
2. Hypercoagulability
3. Anemia
4. Thrombocytopenia
5. Leukopenia
192
What 2 events can exacerbate SLE symptoms
1. Stress
| 2. Drug exposure
193
What are symptoms of drug-induced SLE
1. arthralgia
2. Anemia
3. Leukopenia
4. Fever
194
What are the 11 most common factors and drugs that illicit SLE
```
P = pregnancy
I = infection
S = surgery
S = stress
E = Enalapril
D = D-PCN
```
```
C = Captopril
H = Hydralazine
I = Isoniazid
M = Methyldopa
P = Procainamide
```
195
What is the medical treatment for SLE
1. Corticosteroids
2. NSAIDs
3. Immunosuppressants
4. Antimalarials
196
What immunosuppressants are commonly used in SLE
1. Cyclophosphamide
2. Azathioprine
3. Methotrexate
4. Mycophenolate mofetil
197
What 2 antimalarials are used in SLE
1. Hydroxychloroquine
| 2. Quinacrine
198
What anesthetic considerations should be made with SLE pts that have cricoarytenoid arthritis
1. Use smaller ETT
2. Prepare for post-extubation laryngeal swelling and airway obstruction
3. Give steroids for airway edema
199
What anesthetic precautions should be considered in SLE pts taking cyclophosphamide
It inhibits plasma cholinesterase and increases succinylcholine duration
200
What cardiac problems are associated with Marfan's syndrome and why
Aortic dissection and aortic insufficiency
| This is d/t aortic root dilation
201
What spontaneous pulmonary complication are pts with Marfan's at risk for
How can this be minimized
Increased risk for PTX
Careful monitoring of PIP
202
4 Classic physical alterations of Marfan's syndrome
1. Extremely tall stature
2. Pectus excavatum
3. Kyphoscoliosis
4. Hyperflexible joints
203
What is Ehlers-Danlos syndrome
An inherited disorder of procollagen and collagen
204
What complications are associated with Ehlers-Danlos syndrome
Spontaneous bleeding into joints
| AAA
205
What type of anesthesia should be avoided in pts with Ehlers-Danlos syndrome
Avoid regional anesthesia and IM injections d/t increased bleeding risk
206
Why is increased bleeding a problem in Ehlers-Danlos syndrome
Not because of coagulopathy but d/t poor vessel quality
207
What is osteogenesis imperfecta
A connective tissue disorder with autosomal dominant inheritance
"Brittle bone"
208
What anesthetic considerations should be made with positioning and OI pts
Careful positioning because fractures can occur even with BP cuff inflation or fasciculations from meds
209
What airway precautions should be taken in pts with OI
Very careful manipulation d/t risk of c-spine fracture and reduced cervical ROM
210
What 4 pulmonary changes occur with OI and why
1. Reduced chest wall compliance
2. Reduced VC
3. V/Q mismatch
4. Arterial hypoxemia
d/t kyphoscoliosis and pectus excavatum
211
How does increased serum thyroxine affect pts with OI
It increases BMR and VO2 and lead to hyperthermia
212
Why can OI pts have blue sclera
Because the sclera is susceptible to fracture
213
What is multiple sclerosis
A demyelinating disease of the CNS
214
What symptoms do MS pts experience
Sensory and motor deficiencies
| Autonomic instability
215
Why are MS pts at risk for aspiration
Cranial nerve involvement causes bulbar muscle dyfxn
216
What are 3 treatments for MS
1. Corticosteroids
2. Interferon
3. Azathioprine
217
What 2 events can exacerbate MS symptoms
1. Stress
| 2. Increased body temp
218
Which muscle relaxant should be avoided in MS pts and why
Succinylcholine
| It can cause life-threatening hyperkalemia
219
What is myotonic dystrophy
Prolonged contracture after a voluntary contraction
220
What causes myotonic dystrophy
Dysfunctional Ca++ sequestration by the SR
221
What are 3 things that can increase the risk of contractures in pts with myotonic dystrophy
1. Succinylcholine
2. NMB reversal w/ anticholinesterase
3. Hypothermia (shivering)
222
What 4 concerns should the anesthetist have when treating a pt with myotonic dystrophy
1. Aspiration
2. Respiratory muscle weakness
3. Cardiomyopathy and dysrhythmias
4. Sensitivity to anesthetic agents
223
What is scleroderma
A condition causing excessive fibrosis in the skin and organs, especially microvasculature
224
What airway complications are associated with scleroderma
Limited mouth opening d/t skin fibrosis
225
What lung complications are associated with scleroderma
1. Pulmonary fibrosis
| 2. Pulmonary HTN
226
What cardiac complications are associated with scleroderma
1. Dysrhythmias
| 2. CHF
227
What renal complications are associated with scleroderma
1. Renal failure
| 2. Renal artery stenosis leading to HTN
228
What vascular complications are associated with scleroderma
Decreased vessel compliance leading to HTN
229
What peripheral and cranial nerve complications are associated with scleroderma
Nerve entrapment by tight connective tissue leading to neuropathy
230
What ocular complications are associated with scleroderma
Dryness predisposes to corneal abrasions
231
What is CREST syndrome in reference to scleroderma
Calcinosis, Raynaud's phenomenon, Esophageal hypomobility, Sclerodactyly, Telangiectasia
232
How does telangiectasias in pts with scleroderma affect airway management
Spider veins of the mucosa increase the risk of bleeding during airway manipulation, especially with nasal intubation
233
What is Paget's disease
A condition associated with excess osteoblastic and osteoclastic activity that causes abnormally thick, but weak, bone deposits
234
What causes Paget's disease
1. Excess parathyroid hormone
| 2. Calcitonin deficiency
235
What are the most common problems associated with Paget's disease
Pain and fractures
