Pharm 21- Malignant Hyperthermia Flashcards

1
Q

What is malignant hyperthermia?

A

Autosomally dominant disorder of skeletal muscles
Calcium that is stored in skeletal muscle is released inappropriately causing muscles to contract and stiffen simultaneously

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2
Q

Rhabdomyolysis

A

Skeletal muscle breakdown

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3
Q

Faulty metabolism causes what?

A
Hyperthermia 
Rhabdomyolysis
Run-away hypermetabolism
Acidosis
Flushed skin
Myoglobinuria
Hypotension
Death
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4
Q

Hematuria

A

Cloudy whole blood

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5
Q

Hemoglobinura

A

Only the hemoglobin gets filtered out through kidneys; urine bright red but no cells there

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6
Q

How soon do symptoms appear?

A

Within 1 hour (but can be delayed up to 12 hours)

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7
Q

What patient population has the most cases?

A

Children and young adults < 30 years old

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8
Q

What are some known chemical MH triggers?

A

Volatile Gas and Sux

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9
Q

What are not MH triggers?

A
Intravenous anesthetics (including propofol)
Opiates
Non-depolarizating muscle relaxants
Ketamine
Sedatives and tranquilizers
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10
Q

How many anesthetic procedures have MH?

A

1:5000 - 1:100,000 cases

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11
Q

What races does it occur?

A

All races and ethnicities

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12
Q

International Mortality data on MH ranges from what? What is this based on?

A

1.4% - 20.0% which appears to be based entirely on the speed of MH diagnosis and therapy initiation

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13
Q

Mean age of cases in US deaths

A

20

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14
Q

What has a high predictive value for increased morbidity and mortality?

A

“Muscular Build”

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15
Q

What is a major positive prognositic indicator?

A

Preparedness by the medical team

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16
Q

Specific Clinical Signs of MH

A

Muscle Rigidity
Dramatically increase co2 production
Rhabdomyolysis
Profound hyperthermia

17
Q

Non-specific Clinical Signs of MH

A

Tachycardia
Tachypnea
Acidosis (respiratory and metabolic)
Hyperkalemia

18
Q

MH Clinical Presentations

A

Fulminant MH
Masseter Muscle Rigidity (MMR)
Late onset MH

19
Q

Fulminant MH

A

Most common; muscular rigidity extreme hyperthermia, tachycardia. Typically occurs shortly after anesthetic induction

20
Q

Masseter Muscle Rigidity (MMR)

A

profound jaw muscle rigidity after succinylcholine administration; may be an early sign of MH

21
Q

Late Onset MH

A

Uncommon; occurs within the first hour of anesthetic terminiaton

22
Q

Patients with a history of MMR post-anesthetic induction have what percent change of having Fulminant MH during their next anesthetic?

23
Q

What are other prognositc indicators?

A

Patients with muscular disorders: muscular dystrophy, myotonias, Duchennes Disease, becker’s disease, are at much greater risk of FUlminant MH and shouldn’t receive SUX

24
Q

Why does MH occur?

A

A mutation in the ryanodine receptor gene (RYR1) in skeletal muscles affects the muscles ability to properly utilize calcium

25
Treatment of Acute MH ***
Immediately discontinue volatile gas anesthetics and succinylcholine administraiotn Hyperventilate with 100% oxygen (+/- change out gas tubing) Give 1-2 mg/kg Bicarb Dantrolene: 2.5 mg/kg IV push PRN Cool patient Tx arrhythmias but don't give Na channel blockers Monitor coag and electrolytes
26
Dantrolene (Dantrium)
Blocks release of calcium from skeletal muscle's sarcoplasmic reticulum which prevents the pathologically massive, prolonged release of calcium through the RYR1 channels which is the etiology of Fulminant MH