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Pharm Exam 1 > MH Lecture > Flashcards

MH Lecture Flashcards

1
Q

Which chromosomes have the gene responsible for MH?

A

1, 3, 7, 17, 19

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2
Q

What chromosome most commonly causes MH?

A

19

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3
Q

What conditions our patients at increased risk for MH?

A

myopathies associated with RYR1 mutations
exertional Rhabdomyolysis
severe statin induced myopathy
Duchenne & Becker Muscular Dystrophy (avoid succinylcholine)

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4
Q

What would happen if you gave succinylcholine to an individual with Muscular dystrophy?

A

Rhabdomyolysis and hyperkalemia

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5
Q

What’s the MH hotline?

A

1-800-644-9737

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6
Q

Is MH more common in males or females?

A

Males

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7
Q

What animal can also experience MH?

A

Pigs

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8
Q

Malignant Hyperthermia is a direct result of a defect in the _______ Receptor?

A

Ryanodine

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9
Q

Physiologically what is happening during and MH event?

A

uncontrolled release of Calcium which causes massive muscle contraction leading to a hyper metabolic state

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10
Q

What two drug classes cause MH?

A

Halogenated anesthetics
depolarizing neuromuscular blockers

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11
Q

which 3 co-existing diseases are definitely associated with MH?

A

King-Denborough syndrome
Central core disease
Multiminicore disease

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12
Q

What occurs when the T-tubule is depolarized?

A

Calcium enters myocyte

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13
Q

Muscle disease NOT associated with MH:

A

Osteogenesis imperfecta
Noonan syndrome
Arthrogryposis multiplex congenita
Myotonias
Neuroleptic Malignant syndrome

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14
Q

What three factors cause one to be high risk for MH?

A

Midwest location
children/young adult age
male

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15
Q

Conditions that mimic MH:

A

contract dye
diabetic coma
drug toxicity overdose
heat stroke
anesthesia machine malfunction (high co2)
thyroid storm
intercranial free blood
pheochromocytoma
sepsis

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16
Q

Dantrolene dosage:

A

2.5mg/kg Q5-10mins

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17
Q

What score on the MH grading scale indicates a need for testing?

A

greater than 20

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18
Q

s/s of MH:

A

tachycardia
tachypnea
arterial hypoxemia
respiratory/metabolic acidosis
hyperkalemia
cardiac dysrhythmia
hypotension
skeletal muscle rigidity
hyperthermia
mottled skin

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19
Q

What is the primary intracellular ion affected by MH?

A

Calcium

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20
Q

What are the three earliest signs of MH?

A

tachycardia, increased EtCO2, masseter spasm

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21
Q

how long after exposure to triggering agent can MH event occur

A

6 hours

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22
Q

What is Trismus?

A

a tight jaw that can still be opened. normal response to succinylcholine

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23
Q

What happens if Trismus is prolonged and becomes exaggerated?

A

“jaws of steel” MH risk increases greatly

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24
Q

What is Masseter muscle rigidity?

A

a tight jaw that CANNOT be opened
it is NOT a normal response to succinylcholine

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25
What should be done if a patient experiences masseter muscle rigidity?
assume MH until proven otherwise.
26
What should be done is trismus occurs?
check EtCO2, urine color, blood for CK, acidosis, electrolytes (esp K+)
27
If Jaws of Steel persist more than a few minutes what should be done?
Halt procedure
28
What is the most common cause of death in a patient that experienced MH?
Ventricular Fibrillation
29
Can masseter muscle rigidity be fixed with a non-depolarizing NMB?
No, spasm is due to increased calcium in the myoplasm
30
Which drug is contraindicated in the management of MH?
Verapamil- CCB could lead to hyperkalemia when administered with dantrolene
31
What is the UOP goal while treating MH?
2mL/kg/hr
32
What are dantrolenes 2 mechanisms of action?
reduces calcium release from the RyR1 receptor in skeletal myocyte prevents calcium entry into the myocyte- reducing the stimulus for calcium-induced calcium release
33
dantrolene classification
muscle relaxant
34
Dantrolene side effects:
muscle weakness, venous irritation
35
When should Dantrolene administration STOP?
when hypermetabolic state stops. if pt requires more than 20mg/kg reconsider diagnosis
36
What should dantrolene be reconstituted with?
60 mL sterile water
37
How much dantrolene is in each bottle?
20mg
38
What 2 actions should you take if you suspect MH?
1. stop triggering agent 2. call for help
39
How often should the vapor-clean filter be replaced during MH crisis?
hourly
40
what causes Rhabdomyolysis to occur in a MH crisis?
depletion of ATP- thus, a late aign
41
T/F some people who experience an MH crisis can have normal ryanodine receptors
TRUE
42
Apart from ryanodine receptors, what three abnormalities can also cause MH?
fatty acid phosphatidylinositol and abnormal Na* channels
43
muscle diseases associated with MHS:
exertional rhabdo severe statin myopathies duchennes & Becker MD
44
how should we care for patients with muscular diseases associated with MHS?
avoid succs: could cause rhabdo/hyperkalemia
45
what are the three diseases caused by ryanodine receptor abnormalities?
central core myopathy multiminicore disease king-denborough syndrome native american myopathy
46
condition that mimic MH:
contrast dye diabetic coma drug toxicity-overdose heat stroke anesthesia machine malfunction thyroid storm intercranial free bleed phenocromatoma sepsis
47
considering autosomal-dominant inheritance, first degree family members have a ___% chance of MH
50
48
What are the indications to refer for contracture test?
suspicious history (MH clinical grade > 20) severe masseter muscle rigidity MH-sensitive relative unexplained Rhabdo severe/recurrent rhabdo
49
Why is genetic testing not preferred?
higher false negative rate
50
Ventricular arrhythmias, cola colored urine, and bleeding are all ______ signs of MH
LATE
51
What is the hallmark early sign of MH?
Hypercarbia
52
What are the two lab levels that increase in MH and when do they peak?
CK and Transaminase enzymes/ peak 12-24 hours
53
MH ABG:
arterial hypoxemia hypercarbia respiratory/metabolic acidosis 7.15-6.80 decreased central venous O2 desaturation
54
what are LATE complications of MH?
DIC Pulmonary edema Acute renal failure CNS: blindness, seizures, coma, paralysis
55
how long should the anesthesia machine be flushed out prior to providing support to a MH susceptible individual?
10l/min for 20 minutes
56
What is a nonpharmalogical thing we can do to prevent triggering an episode?
avoid stress
57
receiving ____ triggering agents greatly increases risk of experiencing an episode
2 agents.
58
T/F most individuals with MH susceptibility have most likely had 1-2 safe anesthetic experiences
TRUE
59
Why isn't dantrolene given prophylactically?
adverse reactions
60
T/F if dad is MHS, triggering agents should be avoided in mom to protect fetus
TRUE
61
What is the first step to treating an MH crisis?
RECOGNITION !!!!!!!!
62
How much bicarb should be given to correct metabolic acidosis?
1-2 mEq/kg IV
63
What are some cooling measures?
iced iv fluids gastric and bladder ice saline lavage surface cooling (bair hugger) GOAL = 38°
64
What is the max dantrolene dosage?
10mg/kg
65
What is Ryanodex dosage?
250mg/vial reconstitute with 5ml water
66
what is the half life of dantrolene?
6-8 hours/ metabolized into active form in liver then excreted by kidneys
67
how do we maintain UOP during an MH crisis?
1. iv fluids 2. mannitol 0.25g/kg 3. furosemide 1mg/kg
68
what is calcium dosage for MH?
Ca Cl: 0.5-1g Calcium Gluc: 1.3-5g
69
How long should dantrolene be continued after an MH crisis?
1mg/kg Q4-6H for 24H

Pharm Exam 1 (4 decks)

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