2. Malignant Hyperthermia Flashcards

(101 cards)

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
when do you just give insulin in MH treatment
if glucose levels are > 250
26
correct hyperkalemia: incr K+ excretion
give loop /thiazide diuretic (furosemide 20-40mg) give GI cation exchange resins (kayexalate) start hemodialysis give sodium bicarb
27
what do GI cation exchange resins do
bind K+ in GI tract
28
MH clinical signs: muscle damage (7)
masseter spasm rigidity incr creatine kinase (CK) hyperkalemia hyperphosphatemia myoglobinemia myoglobinuria
29
what indicates muscle breakdown
myoglobin release
30
clinical signs: hyperthermia
fever (1 degree in 5-15 mins) sweating
31
hyperthermia is the first sign of MH in _____ of cases
4%
32
hyperthermia is one of the first signs of MH in ____ of cases
8%
33
is there a dependable early sign in MH presentation
not really
34
common early MH signs (4)
hypercarbia tachycardia generalized muscle rigidity MMR
35
what should be an immediate assumption of MH
MMR after exposure to trigger
36
above ____ degrees C causes:
above 41.5C causes: - organ dysfunction - DIC
37
late MH signs
rhabdo myoglobinuria
38
MH arterial blood gas
elevated base deficit
39
MH BMP
hyperkalemia
40
MH Mag
hypermagnesemia
41
MH venous blood gas
decr MVO2
42
MH serum myoglobin
incr myoglobin
43
MH serum CK
incr CK
44
MH serum lactate dehydrogenase
incr lactic dehydrogenase
45
when does CK peak after acute MH
14 hours
46
differential diagnosis
list of all potential diagnoses based on - signs - symptoms - lab results
47
MH DDX (10)
neuroleptic maliignant syndrome thyroid storm pheochromocytoma serotonin syndrome iatrogenic hyperhtermia brainstem/hypothalamic injury transfusion reaction sepsis anaphylaxis drug abuse baclofen withdrawal
48
MH iatrogenic DDx
inadequate anesthesia/analgesia hypoventilation/CO2 rebreathing incr CO2 absorption
49
NMS differing from MH
history is defining feature trigger medication
50
thyroid storm difference from MH
no muscle rigidity
51
pheochromocytoma difference from MH
no muscle rigidity
52
serotonin syndrome difference from MH
clonus involvement present (not tonic which is found in MH)
53
transfusion reaction difference from MH
blood products are given no muscle rigidity
54
sepsis difference from MH
no muscle rigidity
55
anaphylaxis difference from MH
no muscle rigidity
56
baclofen withdrawal difference from MH
lack of acidosis
57
common features within MH differential diagnosis
hypertension tachycardia tachypnea (non-paralyzed pts) hypercarbia fever
58
differentiating features within MH differential diagnosis
muscle rigidity masseter spasm rhabdo hyperkalemia metabolic acidosis
59
MH associated syndrome: strong
hypokalemic periodic paralysis central core disease multi-mini core myopathy
60
MH associated syndome: mild
muscular dystrophy king-deborough syndrome recent/frequent rhabdo recent/frequent heat stroke
61
central core disease
myotonic dystrophy deficiency of mitochondrial enzymes
62
central core disease SE
repiratory failure cardiomyopathy
63
multiminicore myopathy
RyR gene cause weakness SEPN gene causes stiffness
64
multiminicore SE
axial muscle weakness rib/spine stiffness arthogyroposis
65
king denborough
RyR mutation
66
king denborough phenotype
short stature dysmorphic facial features
67
MH precautions
no Sux no volatile agents TIVA remove vaporizer tape off sux flush anesthesia machine use charcoal scrubbers
68
anesthesia machine flush MH
10L/min flow 20 mins
69
minimum flow needed for charcoal scrubbers
3L/min
70
how long do charcoal scrubbers last
12 hrs
71
MH treatment: oxygenation/ventilation
place ETT incr flow >10L/min incr MV
72
do you need to change out anesthesia machines when treating MH
no - just change out circuit
73
dantrolene MH dose
2.5 mg/kg
74
dantrolene trade names
dantrium renovo
75
dantrolene reconstitution
20 mg powder into 60 ml sterile water
76
difference between dantrolene and ryanodex
ryanodex does not contain mannitol
77
how often do you dose dantrolene for MH
every 5 mins until symptoms cease
78
dantrolene max does
10 mg/kg
79
what labs should you draw off arterial line for MH case
BMPs -- Red or Green tube blood gases -- ABG syringe creatine kinase -- green tube coag/TEG -- blue tube
80
BMP tests for
K+ levels
81
blood gases test for
acid base status
82
CK tests for
degree of muscle breakdown
83
Coag/TEG tests for
possible DIC (functionality of cloitting factors)
84
when do you treat hyperkalemia with MH
with EKG abnormalities or K+ >6 mEq/L
85
hyperkalemia treatment for MH
10% CaCl - 5 mL insulin/D50 - 10 units insulin / 30-50 dextrose sodium bicarb - 1-2 mEq/kg
86
when do you treat metabolic acidosis in MH
base deficit > 8mEq/L
87
50 mEq of NaHCO3 produces ___ of CO2
50 mEq of NaHCO3 produces 1L of CO2
88
what cardiovascular drugs are contraindicated with MH pts
verapamil diltiazem
89
when do you discontinue cooling measures
once pt is 38 degrees
90
cooling measures
cool saline expose pt ice packs - axilla, neck, groin intraperitoneal lavage
91
urine output
1-2 mL/kg/hr
92
continued dantrolene dosing after acute epidose ends
1 mg/kg every 4-6 hrs for up to 24 hrs 0.25 mg/kg/hr for up to 24 hrs
93
DIC is associated in _____ of MH cases
7%
94
pts with rhabdo should be monitored for
compartment syndorme
95
rhabdo treatment
hydration diuresis
96
dantrolene drug categoy
paralytic (requires amnesia - background propofol)
97
why is mannitol in dantrolen
facilitate diuresis and renal protection
98
what IV should you be giving dantrolen through
14-16ga IV
99
MH testing
halothane-caffeine contracture test -- muscle biopsy from quads or rectus abdominus
100
what cells express MH mutations
WBC
101
caffeine
lowers threathold for spontaneous Ca2+ release promotes contractions