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Su: Sim Lab 2 > 2. Malignant Hyperthermia > Flashcards

2. Malignant Hyperthermia Flashcards

1
Q

MH genetics mutations (2)

A

mutation in either:
- RyR1
- CaV1.1

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2
Q

RYR1 receptor

A

Ca2+ release channel
on sarcoplasmic reticulum

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3
Q

RYR1 known mutaions

A

110

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4
Q

CAV1.1 receptor

A

DHP receptor
L-type Ca2+ channel
in T-tubule

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5
Q

CAV1.1 known mutations

A

2

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6
Q

MH chromosome locations

A

1
15
19

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7
Q

MH genetic pattern

A

autosomal dominant
w/ variable penetrance

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8
Q

MH genetic prevalance

A

1:3000

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9
Q

MH genetic concentration

A

scandinavian descent
higher inicidence of occurrence in wisconsin and upper midwest

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10
Q

_____% of pts who develop MG have have previous uneventful anesthetics with triggering medications

A

50%

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11
Q

krebs cycle

A

glucose —>
ATP + CO2 + heat

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12
Q

normal physiology responses

A

transient hyponatremia
transient hyperkalemia
ATP hydrolysis (incr heat)
SERCA activation (incr heat)

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13
Q

how does MH occur

A

sustained RyR activation
= sustained Ca2+ release (need more ATP)
Mg2+ inhibition of Ca2+ release is blocked
– Ca2+ release continues

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14
Q

MH most potent trigger

A

halothane

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15
Q

MH triggers

A

volatile agents (iso, sevo, des)
sux

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16
Q

MH awake triggers

A

high intensity exercise
heat
anoxia
apprehension

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17
Q

exercise ____ min prior to trigger _____ severity and _____ onset

A

exercise <60 min prior to trigger increased severity and hastened onset

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18
Q

MH general clinical signs

A

incr metabolism
incr sympathetics
muscle damage
hyperthermia

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19
Q

incr metabolism signs 7

A

incr CO2 production
incr O2 consumption
decr MVO2 tension
metabolic acidosis
respiratory acidosis
cyanosis
mottling

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20
Q

incr sympathetics

A

tachycardia
hypertension
arrhythmias

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21
Q

MH arrythmia progression

A

PVCs
bigeminy
VTACH
VFIB
asystole

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22
Q

3 ways to correct hyperkalemia

A

membrane stabilization
transfer of K+ INTO cells
incr K+ excretion

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23
Q

correct hyperkalemia: membrane stabilization

A

hyperventilation
give CaCl (500-1000mg) or Ca gluconate (1000mg)

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24
Q

correct hyperkalemia: transfer of extracellular K+ INTO cells

A

give 5-10 units insulin w/25-50g glucose
(10 units insulin w.50 mL of 50% dextrose)

give beta agonist (albuterol)

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25
Q

when do you just give insulin in MH treatment

A

if glucose levels are > 250

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26
Q

correct hyperkalemia: incr K+ excretion

A

give loop /thiazide diuretic (furosemide 20-40mg)
give GI cation exchange resins (kayexalate)
start hemodialysis
give sodium bicarb

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27
Q

what do GI cation exchange resins do

A

bind K+ in GI tract

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28
Q

MH clinical signs: muscle damage (7)

A

masseter spasm
rigidity
incr creatine kinase (CK)
hyperkalemia
hyperphosphatemia
myoglobinemia
myoglobinuria

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29
Q

what indicates muscle breakdown

A

myoglobin release

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30
Q

clinical signs: hyperthermia

A

fever (1 degree in 5-15 mins)
sweating

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31
Q

hyperthermia is the first sign of MH in _____ of cases

A

4%

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32
Q

hyperthermia is one of the first signs of MH in ____ of cases

A

8%

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33
Q

is there a dependable early sign in MH presentation

A

not really

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34
Q

common early MH signs (4)

A

hypercarbia
tachycardia
generalized muscle rigidity
MMR

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35
Q

what should be an immediate assumption of MH

A

MMR after exposure to trigger

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36
Q

above ____ degrees C causes:

A

above 41.5C causes:
- organ dysfunction
- DIC

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37
Q

late MH signs

A

rhabdo
myoglobinuria

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38
Q

MH arterial blood gas

A

elevated base deficit

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39
Q

MH BMP

A

hyperkalemia

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40
Q

MH Mag

A

hypermagnesemia

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41
Q

MH venous blood gas

A

decr MVO2

42
Q

MH serum myoglobin

A

incr myoglobin

43
Q

MH serum CK

A

incr CK

44
Q

MH serum lactate dehydrogenase

A

incr lactic dehydrogenase

45
Q

when does CK peak after acute MH

A

14 hours

46
Q

differential diagnosis

A

list of all potential diagnoses based on
- signs
- symptoms
- lab results

47
Q

MH DDX (10)

A

neuroleptic maliignant syndrome
thyroid storm
pheochromocytoma
serotonin syndrome
iatrogenic hyperhtermia
brainstem/hypothalamic injury
transfusion reaction
sepsis
anaphylaxis
drug abuse
baclofen withdrawal

48
Q

MH iatrogenic DDx

A

inadequate anesthesia/analgesia
hypoventilation/CO2 rebreathing
incr CO2 absorption

49
Q

NMS differing from MH

A

history is defining feature
trigger medication

50
Q

thyroid storm difference from MH

A

no muscle rigidity

51
Q

pheochromocytoma difference from MH

A

no muscle rigidity

52
Q

serotonin syndrome difference from MH

A

clonus involvement present (not tonic which is found in MH)

53
Q

transfusion reaction difference from MH

A

blood products are given
no muscle rigidity

54
Q

sepsis difference from MH

A

no muscle rigidity

55
Q

anaphylaxis difference from MH

A

no muscle rigidity

56
Q

baclofen withdrawal difference from MH

A

lack of acidosis

57
Q

common features within MH differential diagnosis

A

hypertension
tachycardia
tachypnea (non-paralyzed pts)
hypercarbia
fever

58
Q

differentiating features within MH differential diagnosis

A

muscle rigidity
masseter spasm
rhabdo
hyperkalemia
metabolic acidosis

59
Q

MH associated syndrome: strong

A

hypokalemic periodic paralysis
central core disease
multi-mini core myopathy

60
Q

MH associated syndome: mild

A

muscular dystrophy
king-deborough syndrome
recent/frequent rhabdo
recent/frequent heat stroke

61
Q

central core disease

A

myotonic dystrophy
deficiency of mitochondrial enzymes

62
Q

central core disease SE

A

repiratory failure
cardiomyopathy

63
Q

multiminicore myopathy

A

RyR gene cause weakness
SEPN gene causes stiffness

64
Q

multiminicore SE

A

axial muscle weakness
rib/spine stiffness
arthogyroposis

65
Q

king denborough

A

RyR mutation

66
Q

king denborough phenotype

A

short stature
dysmorphic facial features

67
Q

MH precautions

A

no Sux
no volatile agents
TIVA
remove vaporizer
tape off sux
flush anesthesia machine
use charcoal scrubbers

68
Q

anesthesia machine flush MH

A

10L/min flow
20 mins

69
Q

minimum flow needed for charcoal scrubbers

A

3L/min

70
Q

how long do charcoal scrubbers last

A

12 hrs

71
Q

MH treatment: oxygenation/ventilation

A

place ETT
incr flow >10L/min
incr MV

72
Q

do you need to change out anesthesia machines when treating MH

A

no - just change out circuit

73
Q

dantrolene MH dose

A

2.5 mg/kg

74
Q

dantrolene trade names

A

dantrium
renovo

75
Q

dantrolene reconstitution

A

20 mg powder into 60 ml sterile water

76
Q

difference between dantrolene and ryanodex

A

ryanodex does not contain mannitol

77
Q

how often do you dose dantrolene for MH

A

every 5 mins until symptoms cease

78
Q

dantrolene max does

A

10 mg/kg

79
Q

what labs should you draw off arterial line for MH case

A

BMPs – Red or Green tube
blood gases – ABG syringe
creatine kinase – green tube
coag/TEG – blue tube

80
Q

BMP tests for

A

K+ levels

81
Q

blood gases test for

A

acid base status

82
Q

CK tests for

A

degree of muscle breakdown

83
Q

Coag/TEG tests for

A

possible DIC
(functionality of cloitting factors)

84
Q

when do you treat hyperkalemia with MH

A

with EKG abnormalities
or
K+ >6 mEq/L

85
Q

hyperkalemia treatment for MH

A

10% CaCl - 5 mL
insulin/D50 - 10 units insulin / 30-50 dextrose
sodium bicarb - 1-2 mEq/kg

86
Q

when do you treat metabolic acidosis in MH

A

base deficit > 8mEq/L

87
Q

50 mEq of NaHCO3 produces ___ of CO2

A

50 mEq of NaHCO3 produces 1L of CO2

88
Q

what cardiovascular drugs are contraindicated with MH pts

A

verapamil
diltiazem

89
Q

when do you discontinue cooling measures

A

once pt is 38 degrees

90
Q

cooling measures

A

cool saline
expose pt
ice packs - axilla, neck, groin
intraperitoneal lavage

91
Q

urine output

A

1-2 mL/kg/hr

92
Q

continued dantrolene dosing after acute epidose ends

A

1 mg/kg every 4-6 hrs for up to 24 hrs
0.25 mg/kg/hr for up to 24 hrs

93
Q

DIC is associated in _____ of MH cases

A

7%

94
Q

pts with rhabdo should be monitored for

A

compartment syndorme

95
Q

rhabdo treatment

A

hydration
diuresis

96
Q

dantrolene drug categoy

A

paralytic
(requires amnesia - background propofol)

97
Q

why is mannitol in dantrolen

A

facilitate diuresis and renal protection

98
Q

what IV should you be giving dantrolen through

A

14-16ga IV

99
Q

MH testing

A

halothane-caffeine contracture test
– muscle biopsy from quads or rectus abdominus

100
Q

what cells express MH mutations

A

WBC

101
Q

caffeine

A

lowers threathold for spontaneous Ca2+ release
promotes contractions

Su: Sim Lab 2 (18 decks)

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