Apex- Musculoskeletal diseases Flashcards
1
Q
Select the statement that BEST describes myasthenia gravis
A. Pregnancy reduces the severity of symptoms
B. Neonates of affected mothers are at risk for msucle weakenss
C. Edrophonium impairs muscle strength during symptom exacerbation
D. Acetylcholine production is decreased
A
B. Neonates of affected moms are at risk for muscle weakness
A - pregnancy exacerbates the sx of MG
B- yes - anti- AchR IgG antibodies cross placenta
C- doesnt fix the non-functioning post synaptic receptors
D- Ach is present in sufficient quantiy - post synaptic issue
2
Q
Myasthenia gravis- are symptoms worse in the morning or worse as the day goes on
A
worse as the day goes on
3
Q
Early signs of Myasthenia gravis (2)
A
diplopia and ptosis
bublbar muscle weakenss - dysphagia, dysarthia, drooling
DOE
proximal muscle weakness (jsut think oral muscles affected, they are proximal)
4
Q
Thoughts about mom with myasthenia gravis
A
Her: preganancy and stress exacerbate sx
baby: 15-20% of neonates come out with weakness
Anti-AchR IgG antibodies cross placenta - can persist for up to 2-4 weeks
5
Q
What’s the Tensilon test and when would it be used? How much?
A
Edrophonium 1-2mg IV to somone with MG to differentiate between cholineric crisis from too much pyridostigmine or exacerbation of MG (myasthenic crisis)
Edrophonium = anticholinesterase
-if muscle strength improves, the pt has myasthenic crisis
> increasing the concnetration of ACH at the NMJ improved the pts muscle strengtrh
> if it doesnt > cholinergic crisis - give anticholinergic
6
Q
Patient with MG will be (select 2):
-Sensitive to Vec
-Resistant to Roc
-Sensitive to Sux
-Resistant to Sux
A
Sensitive to Vec
Resistant to Roc
Sensitive to NDMR - less functioning receptors = less that need to be blocked
Resistant to SUX- sux requires functioning receptors to exert it’s effects
-neurotransmission is imparied, takes more sux (acts like ach) to depolarize the membrane
7
Q
If sux is indicated in a myasthenia gravis patient for RSI, how should you adjust the dose?
A
increase to 1.5-2mg/kg
(resistance to sux)
8
Q
identify the characteristics comon to Eaton-Lambert syndrome (select 3):
-destruction of voltage-gated calcium channels
-bulbar muscle weakness
-diagnosed with tensilon test
-destruction of the nicotinic recpetor
-increased sensitivity to sux
-small cell lung ca
A
-destruction of voltage-gated calcium channels
-bulbar muscle weakness
-diagnosed with tensilon test
-destruction of the nicotinic recpetor
-increased sensitivity to sux
-small cell lung ca
the other 3 are myasthenia gravis
9
Q
How are eaton lambert and MG similar and how are they different
A
they both are disorders of the NMJ resulting in muscle weakness mediated by IgG destruction
Eaton Lambert = destruction of presynaptic voltage-gated calcium channels at the presynaptic nerve terminal
Myasthenia gravis = estruction of the post-synaptic nicotinic ach receptors on the motor endplate
10
Q
What is myasthenic syndrome
A
Eaton-Lambert Syndrome
IgG mediated destruction of the presynaptic voltage gated calcium channels that result in muscle weakness
AKA: Lambert- Eaton Myasthenic syndrome (LEMS)
11
Q
Common comorbidies with Eaton-Lambert Syndrome vs MG
A
EL = small cell lung ca (oat-cell)
MG = thymoma
12
Q
Muscle weakness in EA vs MG
proximal/distal muscles
which one better in the morning and which one worse
A
both proximal muscles
EA= worse in the AM and gets better throughout day
MG= best in the AM and gets worse throughout day
13
Q
T/F: Anticholinestereases can be helpful with eaton-lambert syndrome
A
false
tensilon test dont do shit either
14
Q
What is 3,4-diaminopyridine (DAP) used to tx?
what does it do?
A
Eaton-Lambert Syndrome
increases Ach release from the presynaptic nerve terminal and improves strength of contraction
15
Q
which disease begins with an influenza-like illness that is followed by ascending paralysis?
A
Guillain-Barre
16
Q
What describes a group of diseases characterized by intermittent attacks of skeletal muscle weakness that are associated with hypo- or hyperkalemia
A
Familial periodic paralysis
17
Q
What describes a group of diseases where the hallmark is prolonged muscle contracture (myotonia) after a voluntary skeletal muscle movement
A
Myotonic dystrophy
18
Q
What is myotonia
what is it associated with
A
prolonged muscle contracture
myotonic dystrophy
occuring after a voluntary skeletal muscle movement
19
Q
What is Charcot-Marie-Tooth disease?
A
an inherited peripheral neutopathy presenting as skeletal muscle weakness and wasting
-usually confined to the lower third of the legs but can also affect the quads, hands and forearms
20
Q
What is the most common cause of acute, generalized paralysis
A
GBS
21
Q
Which diseae is characterized by an immunologic assault on the myelin in the peripheral nerves
A
GBS
22
Q
GBS time frames:
flu like illness:
symptoms persists for:
full recovery within:
A
1-3 weeks prior to symptoms
2 weeks
4 weeks
23
Q
Describe the paralysis associated with GBS
A
flaccid paralysis beginning distally and ascending proximally twoards the trunk and face
24
Q
Treatment for GBS (2)
A
- IV IgG
- Plasmapheresis
25
T/F: steroids are useful in treatment of GBS
FALSE
26
SUX vs NDMR for GBS
NO SUX!!
-reduce dose of NDMR
27
Can you use regional anesthesia for GBS?
controversial - inconclusive data
28
The peripheral nervous system includes all the nerves of the body where
outside of the brain and spinal cord
29
What is acute idiopathic polyneuritis?
GBS
## Footnote
immunolgic attack on myelin in the peripheral nerves
30
3 most common viruses associated with GBS
## Footnote
3 other things assoicated with it
1. Campylobacter jejuni bateria
2. Ebstein-Barr Virus
3. Cytomegalovirus
## Footnote
1. vaccines!
2. surgery!
3. lymphomatous diseaise
31
Which NMD will ephedrine result in an exaggerated response and why
GBS
## Footnote
peripheral desruction of mylin, impaired synaptic transmission, upregulation of postjuncitonal reeptors - acetycholine and adrenergic
32
What is plasmapheresis and when would it be used?
it's when blood is withdrawn from the body, blood and plasma are seperated into plasma and cells, and the cells are transfused back
-goal is to remove plasma which has antibodies that are causing issues in autoimmune diseases
## Footnote
like dialsysis but specifically removes antibodies from the plasma portion of hte blood
33
T/F: Interferon has shown to improve outcomes in GBS
False -MS
34
T/F: Regional anesthesia is preferrred over GA in GBS
False
## Footnote
there is controversy with regional and GBS
35
Which agents are safe to administer to the patietn with hypokalemic periodic paralysis? (select 2):
-Roc
-terbutaline
-D5LR
-Acetazolamide
ROC & Acetazolamine
## Footnote
*acetazolamide is the treatment for both hyperand hypokalemic variants
-hypokalemia periodic paralysis can follow a glucose-insulin infusion. pt becomes weak when serum k decreases
36
Hypokalemic vs Hyperkalemic period paralysis
-which results in muscle weakenss following a glucose-insulin infusion vs potassium administration
hypokalmeic - glucose-insulin infusion
hyperkalemia- oral potassium administration
## Footnote
both result in skeletal mujscle weakenss
37
Treatment for Familial period paralysis - hypo vs hyperkalemic forms
Acetazolamide for both
## Footnote
-creates a non-anion gap acidosis (loss of bicarb)- protects agaisnt hypokalemia
+facilitates renal K excretion > guards agisnt hyperkalemia
38
Familial periodic paralysis - which is more important to avoid- hypo or hyperthermia
avoid hypothermia
39
Sux considerations with familial periodic paralysis: hyperkalemic vs hypokalemic
hyperkalemic- contraindicated for obvious reasons
hypokalemic- contraindicated bc it's associated with MH
40
T/F: Familial Periodic paralysis is a disease of the NMJ
FALSE- disorder of the skeletal muscle membrane
## Footnote
hypoklemic PP Is associated with a calcium channelopathy
hyperkalemic PP is associated with a sodium channelopathy
41
Which NMD is acetazolamide used for and why
Familial periodic paralysis- both varients hypo or hyperkalemia
## Footnote
it results in non-anion gap acidosis, protecting agaisnt hypokalemia
and facilitates renal potassium exretion - protecting agaisnt hyperkalemia
*FPP = skeletal muscle weakness resulting from either hypo or hyperkalemia
42
T/F: LR has potassium in it
True
## Footnote
avoid in hyperkalemic periodic paralysis
43
MH isi primarily associated with the genetic mutation of the:
A. SERCA2 pump
B. ryanodine receptor
C. dihydropyridine receptor
D. actin myofilamint
B.
44
What is an inheritied disease of skeletal muscle that's characterized by disordered calcium homeostasis?
MH
## Footnote
exposure to halogenated anesthetics or sux activates the defective ryanodine recpetor (RYR1) > stimulates the sarcoplasmic retriculum to release way to much calcium into the cell.
45
What happens with MH when the defective ryanodine receptor (RYR1) is exposed to triggering agents?
it stimulates sacroplasmic reticulum to release way too much calcium into the cell
## Footnote
>sustained skeletal muscle contraction
>>hypermetabolic state
>increased o2 consumption
46
MH triggers -2
halogenated anesthetics and depolarizing NMBs (SUX)
47
3 co-existing diseases that are definitely associated with MH
1. King-Denborough syndrome
2. Central core disease
3. Multiminicore disease
48
Is Duchenne's muscular dystrophy associated with MH?
no
## Footnote
but its assoicated with rhabdo if sux is given
49
T/F: MH is not associated with Beker musuclar dystrophy
True
50
Is myotonia congenitia a risk factor for MH?
no
51
T/F: myotonic dystrophy carries of risk of MH
False
52
T/F: osteogenesis imperfecta is not associated with MH
true
53
RyR1 receptor vs RyR2 receptor
RyR1 is located in skeletal muscle
RyR2 is located in the cardiac muscle
54
Word association game:
King-Denborough syndrome
MH
## Footnote
+central core disease
+multiminicore disease
55
Word association game:
Central core disease
MH
## Footnote
+king-denborough syndrome
+mulitminicore disease
56
Association b/t MH and Duchenne's
halogenated agents and sux can initiate an MH-like syndrome but this is NOT MH, it's due to rhabodmyolysis
## Footnote
lack of dystrophin (a structural protein) destablizes the sarcolemma during muscle contraction and increases membrane permeability
so giving SUX = muscle contraction = destabalized sarcolemma
>CA is free to enter the cell , increasing rate of metabolism
>intracellular K is free to exit > hyperkalemic cardiac arrest
>Myoglbin is free to exit the cell > renal failure
57
T/F: Duchenne's pts have a normal RyR1 receptor and can safely get inhalationals and sux
False - they DO have a normal RyR1 reeptor but cant get inhalational and sux bc they cause a MH-like syndrome caused by rhabdo
58
T/F: ANY pt with duchennes should get TIVA over GA
True
## Footnote
INH can induce rhabdo somehow
59
ANY patient with ANY muscular dystrophy who sustains cardiac arrest on induction should immediately be treated with what
## Footnote
why
Calcium chloride
## Footnote
assume severe hyperkalemia
60
Incidence of MH
1:5,000 to
1:50,000
61
4 states that have a higher risk of MH
Wisconsin
Nebraska
West Virgina
Michigan
| males > females
62
Early vs Late signs of MH (3 each)
Early = masseter spasm, increased etco2, tachy
Late = hyperthermia, cola urine (rhabdo), DIC
63
What is the most sensitive indicator for MH
EtCO2 that rises out of proportion to minute ventilation
64
MH can occur as late as how many hours/days following exposure to a triggering agent
6 hours
65
Core temp can (but does not always) begin to rise within what time frame after exposure to a triggering agent
15 mins
## Footnote
but profound hyperthermia is usually a late sign
66
What are the differences between trismus and masseter muscle rigidity
Trismus = tight jaw that an still be opened (normal response to sux)
Masseter muscle rigidity = tight jaw that cant be opened (NMB will not relax)
67
T/F: true masseter muscle rigidity cannot be relaxed with a NMB
True -yikes then what lol
68
T/F: if a patient experiences masseter muscle rigidity, assume MH until proven otherwise
True
69
4 differential diagnosis's for MH
1. thyroid storm
2. pheo
3. sepsis
4. heatstroke
70
Early/intermediate/late sign of MH:
warm soda lime
Early
71
Early/intermediate/late sign of MH:
Irregular heart rhythm
can occur during any phase
72
Early/Intermediate/late sign of MH:
musle rigidity
late
## Footnote
*masster spasm is early
73
T/F: masseter muscle rigidity can be a normal response to sux
| can you proceed with surgery?
FALSE - Trismus (jaw tight but can still be opened) can be a normal response to sux
| no for masseter muscle, yes for trismus
## Footnote
prob wise to convert to a non-triggering agent anyway they say
74
Why won't NMB work for masseter muscle rigidity?
bc the spasm is due to increased calcium in the myoplasm which is distal to the NMJ, so blocking it wont fix the problem
75
What is the caffeine-halothane contracture test the gold standard for diagnosing?
| how is it done?
## Footnote
sensitivity/specificity?
MH
| live muscle biopsy sample
## Footnote
high sensitivity and low specificity
(think MH is HIGHLY SENSITIVE to SUX)
76
Which drug is contraindicated in the management of MH?
A. Verapimil
B. Mannitol
C. Calcium chloride
D. Insulin
A. Verapamil
## Footnote
due to life-threatening hyperkalemkia that can resoult when a CCB is co-administered with dantrolene
-mannitol is in each vial of dantroline so rule that out
77
T/F: data suggests dantrolene prophylaxis is MH precaution patients
false
78
MH Precautions: 3 things you want to do
1. Flush with high flow o2 for 20-100minutes dependeing on vent
2. remove and replace all external parts
> CO2 absorbent, circuit, breathing bag
3. Remove vaporizers
79
T/F: if pt doesnt present with s/s MH within the ifrst hour, it's very unlikely that it will occur later
True
80
How do you use charcoal filters
put one on each port (I&E) of anesthesia machine and flush the machine with high FGF > 10LPM for 90 seconds prior to using machine on pt
81
Dantrolene - what are the 2 MOAs
1. reduces calcium release from the RyR1 recpetor in the skeletal myocyte
2. prevents calcium entry into the myocyte , which reduces the stimulus for calcium-induced release
82
Dantrolene vial:
How many mg dantrolene
What else is in it
How much to reconsitute with and what
20mg
3g of manitol
60mL of preservative-free water
83
T/F: you should diute dantrolene with NS
false- introduces additional solute, prolonging the time it takes for it to dissolve
## Footnote
60mls of perservative-free water
84
T/F: dantrolene is classified as a muscle relaxant
true
85
First 3 steps in treating MH
1. Discontinue triggering agent
2. Call for help (notify suregon to terminate)
3-A. hyperventilate with 100% fio2 at a minimum of 10L/min
3-B charcoal filters and new circuit and bag
| *dont waste time changing the soda lime
## Footnote
#4 is giving dantrolene or ryanodex
86
How often do charcol filters need to be replaced?
every hour
87
Dose of Dantrolene
## Footnote
when to stop
2.5mg/kg IV every 5 -10 mins
## Footnote
stop when symptoms of hypermetabolism subside
88
If pt requires more than how much dantrolene should you then reconsider a diganosis of MH
> 20mg/kg - no more than 8 doses
89
Ryanodex vial contains how much dantrolene
| how much to dilute
250mg
| 5mL of sterile water
90
MH pt...your giving dantrolene, cooling them, correcting acidosis, treating hyperkalemia....how should you protect agaisnt dysrhythmias?
Class 1 antidysrhythmics
Procainamide 15mg/kg IV
Lidocaine 2mg/kg IV
91
MH- urine output should be maintained greater than what
| why
## Footnote
how can you facilitae this
>2ml/kg/hr
| protects agaisnt renal injury from free myoglobin
## Footnote
IV hydration
Mannitol 0.25mg/kg IV
Fureosemide 1mg/kg IV
92
MH can reoccur up to how many hours post stabalization
36 hrs
93
MH- when to stop cooling pt
when temp drops below 38
94
Can you give calcium with MH ?
Yes calcium chloride 5-10mg/kg IV for hyperkalemia
| insulin 0.15units/kg + 1ml/kg D50
95
Insulin and D50 dosing for MH hyperkalemia
0.1units/kg + 1ml/kg D50
96
10 Steps in treating MH
1. D/C agent
2. call for help
3. A- hyperentilate 100%/10L
3. B- charcoal filter and change circuit
4. Dantrolene 2.5mg/kg q 5 mins w 60mls water
5. cool pt
6. correct acidosis
7. treat hyperkalemia
8. protect agaisnt dysrhythmias
9. maintain uop > 2ml/kg/hr
10. coags to check for DIC (impending demise)
97
Preop findings in the patient with Duchenne muscular dystrophy include: (select 2):
-mitral stenosis
-pulmonary fibrosis
-increased creat kinase
-deep q waves in limb leads
-increased creat kinase
-deep q waves in limb leads
## Footnote
- dystrohin stablizes cell membrane - DMD has none so sarcolemma becomes unstable during contraction and increases membrane permeability.
-CK leaks through muscle membrane > elevated plasma concentration
-like skeletal muscle, cardiac muscle is also at risk for degeneration, leading to MR (not stenosis, cardiomyopathy, and conduction defects
-scarring of the posterobasal aspect of hte lv manifests as R wave amplitude increase in lead I and deep Q waves in limb leads
98
EKG changes in DMD (2)
deep q waves in limb leads
increased R wave in lead I
## Footnote
from scarring of hte posterobasal aspect of the left ventricle ... great
-found something that says when calcium freely inters the cell, it activates proteases that destroy contactile elements and cause inflammation, fibrosis , and cell death
-while that somewhat suports this scarring - why is it only the posterobasal aspect of the LV?
99
T/F: DMD just affects skeletal muscle
false
## Footnote
cardiac muscle is also at risk for degeneration leading to MR, cardiomyopathy, and conduction defects
100
What is the most common skeletal muscle myopathy?
Duchenne's muscular dystrophy
## Footnote
absence of dystrophin protein
101
T/F: DMD results in a restrictive lung disease
True - from kyophoscoliosis
102
DMD is an x-linked autosomal (recessive/dominate) disease
x-lined recessive
103
What is the critical structural component of the skeletal and cardiac myocytes that helps anchor actin and myosin to the cell membrane
Dystrophin
104
Why does are DMD pts at risk of hyperkalemia following sux?
bc the absence of dystrophin allows extrajunctional reeptors to populate the sarcolemma
105
3 signs of cardiomyopathy
1. resting tachycardia
2. JVD
3. S3/S4 gallop
4. displacement of PMI
106
What angle describes the magnitude of spinal curvature in pts with scoliosis
| what angle is an indication for surgery
Cobb
| > 40-50 degrees
107
Scoliosis surgery 6 considerations
1. VC < 40% predicted correlates with requirement for post-op ventilation
2. cervical scoliosis may cause difficult intubation
3. thoracic correction higher than T8 may require one-lung ventilation
4. prepare for significant blood loss
5. monitor end-organ perfusoin with serial ABGs ( risk of metabolic acidosis) and urine output
6. risk of VAE
108
Pt's undergoing scoliosis correction.... VC < what would indicate need for post op ventilation
< 40%
109
thoracic scolioisis correction higher than what may require one-lung ventilation
> T 8
110
T/F: VAE is a risk with scoliois correction surgery
true
111
5 causes of scoliosis
| which has the highest incidence
1. idopathic*
2. Congential
3. myopathic
4. neuropathic
5. traumatic
| idopathic = 80% of caes
112
2 myopathic caues of scolioisis
muscular dystrophy
amytonia congenita
113
Cobb angle of what is associated with decreased pulmonary reserve
| what angle would pulm symptoms be seen
60
| 70
## Footnote
thats why they suggest you get it corrected when its 40-50
114
Goal of spinal fusion with instrumentation fo scolioisis is what
to stop the progression of hte curvature and prevent further deterioation of cardiopulmonary function
115
cardiac complications associated with scoliosis mostly affect the right or left heart
right - due to increased PVR
## Footnote
RV hypertrophy
RV strain
RA enlargement
but also may have MVP, MR, and coarctation of aorta
116
T/F: MVP is the most common co-exisiting cardiac condition in those with scoliosis
True
## Footnote
MR & coarcaation too
117
Which is superior for the pt in the prone position to reduce IAP and improve pulm compliance... jackson frame or wilson
Jackson better
118
T/F: Nitrous oxide increases PVR
True
119
VAE would result in (select 2):
-increaed etco2
-decreased etco2
-increased PacO2-Etco2 gradient
-decreased Paco2 - etco2 gradient
-decreased etco2
-increased paco2-etco2 gradient
## Footnote
increased dead space (air no blood), VAE
120
Dens
| AKA: Odontoid process
## Footnote
superior boney projection off the axis (C2)
provides a pivot point that faciltates head rotation
121
RA impacts the airway in what 3 places
1. TMJ
2. Cricoarytenoid joint
3. Cervical spine
122
T/F: RA decreases mouth opening and size of the glottic opening
True
## Footnote
*use smaller ETT to minimize laryngeal trauma
*risk of post-extubation airway obstruction
123
atlantoaxial subluxation and seperation of the atlanto-odontoid articulation is the most common airway complication of which disease?
| what does this increase the risk for (2)
RA
| quadriparesis or paralysis
124
You see your pt has RA... so you appropriately downsize your tube, your preceptor asks why
bc they have RA which affects the cricoarytenoid joints and they can have decreased glottic opening diameter
125
RA pts are at risk for quadriparesis or paalysis due to c-spine immobility, is flexion or extension worse?
flexion
## Footnote
RA= no flex ZONE
126
If you see pt has hx RA- try and pay attention to their voice quiality- why
bc if they are hoarse, stridorus, or dyspneic, theres an increased risk of airway obstruction (cricoarytenoid arthritis)
127
T/F: the thoracolumbar spine is generally not affected by RA
true
128
Anesthetic considerations for the patient with RA include (select 3):
-Aortic regurg
-obstructive ventilatory pattern
-hypercoagulability
-hypoglycemia
-anemia
-pulmonary effusion
Anemia
Aortic regurgitation
Pulmonary effusion
## Footnote
aortic regurg from dilation of hte aortic root
-restritive pulm defect due to intersistal fibrosis, costrochondral involevment and pleural effusions
-NSADS = first line tx of RA; asa inhibits plaetet functio nand increases risk of bleeding
-glucocortioids are used to reduce inflammation. this ncreases (not decreases blood sugar)
129
What is the most common hematologic complication of RA?
Anemia
130
T/F: pleural effusion is hte most common pulmonary complication of RA
True
131
RA patients - increased or decreased risk of bleeding
increased - NSAIDS = first line tx
132
RA patients - increased or decreased blood sugar
increased due to glucocorticoids to reduce inflmmation
133
First line tx for RA
NSAIDS
134
What is RA?
an autoimmune disease targeting synovial joints
## Footnote
there is also widespread systemic involvement leading to vasculitis
135
Hallmark of RA
morning stiffness that generally improves with activity
## Footnote
pain, swollen, warm joints
weakness, fatigue, anorexia
136
T/F: RA typicallly affects proximal interphalangeal and metacarpophalngeal jointsi n the heads and feet and not weight-bearing joints
True
| OA affects weight bearing joints
137
medical mangement of RA aims at reducing inflammation by which 3 methods
1. antirheumatics
2. glucocorticoids
3. nsaids
## Footnote
antirheumatics = methotrexate, cyclosporine, and etarercept
*immune suppression (autoimmune disease) - increases risk of infection and cancer
138
3 antirheumatic drugs used for RA
| risk?
1 Methotrexate
2. cyclosporine
3. Etanercept
| infecftion and cancer
## Footnote
* Methotrexate associated with liver dysfunction and bone marrow suppresion
* Cyclosporine prolongs duration of sux
139
T/F: RA is more common in men
False - women (2-3x more common)
140
What is Sjorgen's syndrome associated with (2)
RA and corenal abrasion
141
What is Rheumatoid factor ?
it's an anti-immunoglobin antibody that is increased in 90% of pts with RA
142
T/F: CRP and sed rate are both increased in RA pts
True
143
How do antirheumatic drugs work
They inhibit:
Tumor necrosis factor (TNF)
Interleukin-1 & 6
T cells and B lymphocytes
144
Risk with methotrexate
liver dysfunction and bone marrow supression
145
Which antirheumatic drug can prolong the duration of sux
Cyclosporine
146
Durgs that are MOST likely to induce systemic lupus rythematosus include: (select 2):
-cyclophosphamide
-hydralazine
-isoniazid
-hydrocortisone
Hydralazine and isoniazid
## Footnote
cylophosphamide and hydrocortisone are treatements for SLE
147
What is Lupus?
an autoimmune disease affecting nearly every organ system
## Footnote
sx result from antibody-induced vasculitis and tissue destruction
148
2 most common problems with lupus
polyarthritis and dermatitis
149
T/F- 75% of lupus pts develop the classic butterfly rash
False
## Footnote
30-50%
150
4 drug groups for tx of SLE
1. NSAIDS
2. corticosteroids
3. immunosupressants (methotrexate, cyclophosphamide)
4. antimalarials (hydroxychloroquine)
151
T/F- pts with SLE are at higher risk for hypercoagulability and thrombosis
True- if they develop antiphospholipid antibodies
152
Is SLE a disease that targets older or younger women
younger
153
Drug induced lupus generally persists for how long
## Footnote
symptoms? (4)
several weeks to months
## Footnote
mild symptoms of arthralgia, anemia, leukopenia, fever
154
What is PISSED CHIMP a mneumonic for what what does it stand for ?
Things that can exacerbate SLE symptoms
Pregnancy
Infection
Surgery
Stress
Enalapril
D-penicillamine
Captopril
Hydralazine
Isosonazid
Methylodpa
Procainamide
155
Match each disease with its defining characterisitic:
-Osteogenesis imperfecta
-Ehlerhs-Danlos syndrome
-Marfan syndrome
Aortic insufficinecy, bleeding into joints, blue sclera
-Osteogenesis imperfecta + Blue sclera
-Ehlerhs-Danlos syndrome + Bleeding into joints
-Marfan syndrome + aortic insufficency
156
Connective tissue disorder associated with AI, aortic dissection, pectus excavatum, kyphoscoliosis and hyperflexible joints
Marfans
## Footnote
*careful with positioning
157
What is an inherited isorder of collagen and is associated with arterial aneurysm and increased bleeding tendency
## Footnote
what 2 things should be avoided
Ehlers-Danlos syndrome
## Footnote
avoid regional and IM injections
158
What disorder is characteristized by "brittle bones" iand is a connective tissue disorder with a high risk of fractures with airway managment and BP cuff
osteogenesis imperfecta
159
which disorder is associated with spontaenous bleeding into joints
| what else is a big association with it
Ehlers-Danlos Syndrome
| Bleeding into joints + AAA
## Footnote
*increased bleeding risk - avoid regional and IM injections
160
T/F: Increased bleeding tendency with ehlers-Danlos syndrome is a result of coagulopathy
False - poor vessel integrity
| *collegen disorder
161
Which disease can result in fractures due to fasiculations from sux?
Osteogenesis imperfecta
| brittle bones
## Footnote
high risk for fractures
-careful airway mgmt - cspine
162
Blue sclera is unique to which disease
osteogenesis imperfecta
| brittle bones
## Footnote
sclera can actually fracture
163
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
Spontaneous ptx
Marfans
## Footnote
keep pips low
164
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
AI
Marfans
| AI & AAA
165
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
Frequent fractures
OI
166
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
increased bleeding tendency
EDS
| bleeding into jints and AAA
## Footnote
avoid IM injections and regional
167
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
Tall stature
Marfans
## Footnote
Abe lincoln
168
B/t- marfan, ehlers Danlos syndrome (EDS) and osterogensis imperfecta (OI) which one is associated with:
Increased serum thyroxine
OI
169
170
What is Multiple sclerosis?
a demylinating diesase of the CNS (brain and spinal cord)
171
Do we need to avoid hyperthermia or hypothermia in Ms pts
avoid hyperthermia
## Footnote
increases risk of demylination
172
Are MS pts at risk for aspiration?
yes- bulbar muscle dysfunction
173
MS patients - which is safe vs questionable - epidural vs spinal
epidural = safe
spinal = questionable - m ay exacerbate sx
174
T/F: pts with myotonic dystrophy may safely receive a halogenated anesthetic
True
## Footnote
no risk of MH
may be more sensitive to them though
175
3 things that increase the risk of contractures with myotonic dystrophy
1. Sux
2. anticholinesterase (use sugammadex)
3. Hypothermia (shivering > contractions
176
symptoms pts may have with MS
-so it's demylination of brain and spinal cord so:
-senosry and motor deficits and autonomic instability
-aspiration risk due to cranial nerve involvement
177
3 things used to treat MS
corticosteroids
interferon
azathioprine
178
MS can be exacerbated by stress and increased body temp as small as what
1 degree C
179
MS - can you give sux?
no- life threatening hyperkalemia
## Footnote
-demylination of CNS , upregulation of receptors bc they arent getting as much ACH as they should be (i think)
180
What does scleroderma cause?
excessive fibrosis in the skin and organs, espeically in the microvasculature
## Footnote
Airway - skin fibrosis limits mouth opening and mandibular mobility
lungs - pulmonary fibrosis and pulmonary htn
heart- dysrhythmias and CHF
vesels - decreased compliance > HTN
kidneys- renal failure and RAS > HTN
peripheral and cranial nerves - entrapment by tight connective tissue > neuroapthy
eyes - dryness - risk of corneal abrasion
181
Which disease is associated with Telangiectasia
| what is it
## Footnote
problem?
those with CREST syndrome - a type of sclerodoma
| spider veins
## Footnote
increase risk of mucosal bleeding which can become problematic during airway manipulation (particularly during nasal intubation); concerning bc pts with scleroderma often have liited mouth oppening which may necesitate nasal fiberoptic intubation
182
What disease is associated with excess osteoblastic and osteoclastic activity that causes abnormally thick, but weak, bone deposits
| what 2 things can cause it
Paget's disease
| excessive PTH or decreased calcitonin
183
2 most common porblems with paget's disease
pain and fractures
| neuropathy can occur too
184
T/F- there is no vascular involvement with paget's disease
True
## Footnote
just excess osteoblasts and osteoclasts activity > big weak bones
>pain and fractures
>>nereve entrapment peripherally - neuropathy
185
Which IVF is MOST likely to exacerbate skeletal muscle weakness in a patient with hypokalemic periodic paralysis?
A. D5W
B. LR
C. NS
D. Albumin
A. D5W
## Footnote
glucose load from D5W spikes insulin which shifts K into the cells
186
SUX is MOST likely to impede airway management in a patient with which condition:
A. Guillain-Barre
B. Osteogenesis imperfecta
C. Myasthenia gravis
D. Myotonic dystrophy
D. Myotonic dystrophy
## Footnote
its characterized by a prolonged contracture after a voluntary contraction from dysfunctional calcium sequestration by the SR
-contractions can be so severe that they interfere with ventilation and intubation
-sux with GB - hyperkalemia
-sux with OI = fracture risk
-sux with MG - n/a - dose is increasesd
187
FDA black box warning , sux to kids may cause :
MH or Rhabdo
Rhabdo
188
What statements are true regarding acute idiopathic polyneuritis ? (select 3):
-exaggerated response to ephedrine
-sux should be avodied
-epstein bar is common etiology
-steroids improve symptoms
-no risk of aspiration
-paralysis starts with bulbar muscles
**-exaggerated response to ephedrine
-sux should be avodied
-epstein bar is common etiology**
-steroids improve symptoms
-no risk of aspiration
-paralysis starts with bulbar muscles
| * Guillian barre*
189
Airway complications of RA include all of the following except:
A. limited TMJ mobiity
B. AO subluxation
C. RLN palsy
D. Cricoarytenoid joint dislocation
C.
## Footnote
RLN palsy is a complication of SLE
190
T/F: RLN palsy is a complication of SLE
True
191
Which diseases are assoicated with vasculitis? (select 2)
-paget's
-RA
-SLE
-OA
RA and SLE
192
T/F: marfans syndrome is an acquired disease
false- autosomal dominant trait
193
A pt with Marfans presents with AI and acute dissection of hte ascendign aorta; select the MOST accurate statement :
A. Spontaneous ptx is a rate complication
B. increased risk of cardiac tamponade
C. Esmolol infusion should be initiated
D. Marfan syndrome is an aquired disease
B. increased risk of tamponade
## Footnote
first line therapy for aortic dissection is an esmolol infusion to decrease wall stress, however, a slower HR increases the regurgitant fraction in the pt with AI
dissection of the ascending aorta can extend into the periocardium and this increases the risk of tamponade (JVD, hypotension, mujffled heart tones = becks triad)
*high risk for spontaenous PTX
*autosomal dominant trait (not acquired)
