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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
Q

What should be done if a patient experiences masseter muscle rigidity?

A

assume MH until proven otherwise.

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26
Q

What should be done is trismus occurs?

A

check EtCO2, urine color, blood for CK, acidosis, electrolytes (esp K+)

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27
Q

If Jaws of Steel persist more than a few minutes what should be done?

A

Halt procedure

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28
Q

What is the most common cause of death in a patient that experienced MH?

A

Ventricular Fibrillation

29
Q

Can masseter muscle rigidity be fixed with a non-depolarizing NMB?

A

No, spasm is due to increased calcium in the myoplasm

30
Q

Which drug is contraindicated in the management of MH?

A

Verapamil- CCB could lead to hyperkalemia when administered with dantrolene

31
Q

What is the UOP goal while treating MH?

A

2mL/kg/hr

32
Q

What are dantrolenes 2 mechanisms of action?

A

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
Q

dantrolene classification

A

muscle relaxant

34
Q

Dantrolene side effects:

A

muscle weakness, venous irritation

35
Q

When should Dantrolene administration STOP?

A

when hypermetabolic state stops.
if pt requires more than 20mg/kg reconsider diagnosis

36
Q

What should dantrolene be reconstituted with?

A

60 mL sterile water

37
Q

How much dantrolene is in each bottle?

A

20mg

38
Q

What 2 actions should you take if you suspect MH?

A
  1. stop triggering agent
  2. call for help
39
Q

How often should the vapor-clean filter be replaced during MH crisis?

A

hourly

40
Q

what causes Rhabdomyolysis to occur in a MH crisis?

A

depletion of ATP- thus, a late aign

41
Q

T/F some people who experience an MH crisis can have normal ryanodine receptors

A

TRUE

42
Q

Apart from ryanodine receptors, what three abnormalities can also cause MH?

A

fatty acid
phosphatidylinositol
and abnormal Na* channels

43
Q

muscle diseases associated with MHS:

A

exertional rhabdo
severe statin myopathies
duchennes & Becker MD

44
Q

how should we care for patients with muscular diseases associated with MHS?

A

avoid succs:
could cause rhabdo/hyperkalemia

45
Q

what are the three diseases caused by ryanodine receptor abnormalities?

A

central core myopathy
multiminicore disease
king-denborough syndrome
native american myopathy

46
Q

condition that mimic MH:

A

contrast dye
diabetic coma
drug toxicity-overdose
heat stroke
anesthesia machine malfunction
thyroid storm
intercranial free bleed
phenocromatoma
sepsis

47
Q

considering autosomal-dominant inheritance, first degree family members have a ___% chance of MH

A

50

48
Q

What are the indications to refer for contracture test?

A

suspicious history (MH clinical grade > 20)
severe masseter muscle rigidity
MH-sensitive relative
unexplained Rhabdo
severe/recurrent rhabdo

49
Q

Why is genetic testing not preferred?

A

higher false negative rate

50
Q

Ventricular arrhythmias, cola colored urine, and bleeding are all ______ signs of MH

A

LATE

51
Q

What is the hallmark early sign of MH?

A

Hypercarbia

52
Q

What are the two lab levels that increase in MH and when do they peak?

A

CK and Transaminase enzymes/ peak 12-24 hours

53
Q

MH ABG:

A

arterial hypoxemia
hypercarbia
respiratory/metabolic acidosis 7.15-6.80
decreased central venous O2 desaturation

54
Q

what are LATE complications of MH?

A

DIC
Pulmonary edema
Acute renal failure
CNS: blindness, seizures, coma, paralysis

55
Q

how long should the anesthesia machine be flushed out prior to providing support to a MH susceptible individual?

A

10l/min for 20 minutes

56
Q

What is a nonpharmalogical thing we can do to prevent triggering an episode?

A

avoid stress

57
Q

receiving ____ triggering agents greatly increases risk of experiencing an episode

A

2 agents.

58
Q

T/F most individuals with MH susceptibility have most likely had 1-2 safe anesthetic experiences

A

TRUE

59
Q

Why isn’t dantrolene given prophylactically?

A

adverse reactions

60
Q

T/F if dad is MHS, triggering agents should be avoided in mom to protect fetus

A

TRUE

61
Q

What is the first step to treating an MH crisis?

A

RECOGNITION !!!!!!!!

62
Q

How much bicarb should be given to correct metabolic acidosis?

A

1-2 mEq/kg IV

63
Q

What are some cooling measures?

A

iced iv fluids
gastric and bladder ice saline lavage
surface cooling (bair hugger)
GOAL = 38°

64
Q

What is the max dantrolene dosage?

A

10mg/kg

65
Q

What is Ryanodex dosage?

A

250mg/vial reconstitute with 5ml water

66
Q

what is the half life of dantrolene?

A

6-8 hours/ metabolized into active form in liver then excreted by kidneys

67
Q

how do we maintain UOP during an MH crisis?

A
  1. iv fluids
  2. mannitol 0.25g/kg
  3. furosemide 1mg/kg
68
Q

what is calcium dosage for MH?

A

Ca Cl: 0.5-1g
Calcium Gluc: 1.3-5g

69
Q

How long should dantrolene be continued after an MH crisis?

A

1mg/kg Q4-6H for 24H

Pharm Exam 1 (4 decks)

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