MH Flashcards

1
Q

MH mutation genes

A

RYR 1 chr 19
CACNA1S chr 1
STAC3 chr 12

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2
Q

Hypermetabolism: ATP needed for what 3 things

A

Ca release, reuptake into SR, and troponin decoupling

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3
Q

MH: succ vs VA causes cell level

A

Succ sustained depolarization at nAChR. VA at ryanodine receptor, ca release from SR

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4
Q

Earliest s/s MH

A

Hypercarbia, tachypnea, tachycardia

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5
Q

Confirmatory lab in kids w masseter spasm after sux

A

> 20,000 CPK

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6
Q

MMR: what is normal, check what, MH occurs how often

A

Mild inc masseter tone after sux w limb flaccidity. Check K and ABG. MH occurs in 20% of cases of MMR

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7
Q

3 symptoms that occur w rhabdo

A

Muscle pain, myoglobinuria, arrhythmias from high K

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8
Q

CPK correlates

A

In >20k well w rhabdo, less well w fever and acidosis

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9
Q

Rapid v slow rhabdo

A

K dangerously high/quick in rapid, in slow its safer, K is redistributed before blood levels increase

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10
Q

Sympathetic response leads to 3 things in heart/vessels

A

Inc catecholamines lead to inc HR, cutaneous vasoconstriction, inc SVR
Inc CO

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11
Q

What to do in isolated MMR

A

D/c volatiles, give propofol, turn up flows, get another IV/give vol/ABG. Observe 24h if other symptoms. Maybe muscle bx

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12
Q

Gold standard MH test

A

Halothane caffeine contracture test. Muscle in halothane, sustained contracture. 80% specificity

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13
Q

How dantrolene works, 1/2 time

A

Reduces muscle tone and metab. Dec release of Ca from SR. Blocks external entry of Ca into sarcoplasm. Inhib ca thru ryanodine receptors. 10-15 e 1/2

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14
Q

Dantrolene: doesnt potentiate effects of what

A

Non depolarizing muscle relaxants or interfere w reversal. Can cause weakness if pre existing NM disease

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15
Q

DONT use dantrolene with what

A

CCB, life threatening hyperkalemia and myo depression

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16
Q

How many vials need to be in facility if sux is used, what else needs to be there

A

36 minimum. Sterile water, 60 ml/vial

17
Q

Dantrolene dosage

A

2.5 mg/kg bolus, then maintenance dose 2 mg.kg q 5 min. Total 10 mg/kg. Then 1 mg/kg q6 for 72 hours

18
Q

Where we want temp. Saline bolus dose.

A

38 c. 15 ml/kg q 10 min

19
Q

Hyperkalemia treatment

A

1 ml/kg d50 glucose and 0.15 u/kg regular insulin and/or CaCl 5-10 mg/kg IV

20
Q

Monitoring how long after, recrudescence how often. Avoid what, measure what

A

36h in icu. 25%. No LR (K). Coag profile for DIC and CKs q6h

21
Q

Lab supplies needed

A

6 abg kits, Ck myoglobin, sma 19, pt/ptt, fibrinogen, fsps, CBC, LA level. Urine myoglobin level x2, urine dipstick hgb

22
Q

Muscle diseases assoc w MH 3

Not assoc w which one

A

Central core disease, king denborough syndrome, and Evans myopathy. Not: MD but can still develop rhabdo so still avoid triggering agents

23
Q

Neuroleptic malignant syndrome presentation. Who is susceptible

A

Fever, muscle rigidity, ANS instab, delirium, elev CPK. Pts on haldol, prolixin, thorazine

24
Q

Sudden cardiac arrest after sux in young males can be what

A

R/t hyperkalemia in undx myopathy. 50% mortality rate, may also have rigidity and hyperthermia

25
Meds/tx for sudden unexpected cardiac arrest
Ca cl, hco3, insulin, glucose, hyperventilation. May need dialysis and CPB
26
Report MH to who
North American mh registry. In CHOP in Pittsburgh. Barbara Brandon