Malignant Hyperthermia

Question 1

MH variety of inheritance patterns, commonly?

Answer 1

Autosomal Dominant

Question 2

MH Pathophysiology:
***issue with ?
~MH inappropriate release of calcium - excessive releases of calcium! and now cell has to deal with it. Takes energy to release calcium and to collect the calcium back up into the sarcoplasmic reticulum. Even muscle relaxation with regards to calcium is energy expensive. Extreme use of __ and extreme use of __ to the point that you reach __.

Answer 2

***Calcium release
ATP
oxygen
oxidative failure

Question 3

~__ also signals cell death, muscle cell death.
~Upon cell death… ___ released can get ___.
~___ released can get ___ this can cause major issues in the kidney.

Answer 3

Calcium 
potassium
hyperkalemia 
Myoglobin
myoglobinemia

Question 4

Heat regulation centers?
~Gain center (vasoconstriction to conserve heat) = ?
~Loss center (vasodilation to lose heat) = ?

Most common occurrence under anesthesia?

Answer 4

Hypothalamus
~Posterior Hypothalamus
~Anterior Hypothalamus

Heat loss

Question 5

Malignant Hyperthermia S/S - the 4 Earliest:

1) Earliest sign?

Answer 5

1) Hypercarbia
2) Tachycardia
3) Tachypnea
4) Masseter spasm

Question 6

MH S/S:

• Earliest sign? Will see increased? ___ stimulation.
• Hyperthermia increase in ___ every ___.
• Skeletal muscle rigidity = ___ seen after ___ in children. Confirmed with?

Answer 6

Hypercarbia
ETCO2
SNS
1-2 degrees celsius 
5 minutes
Masseter spasm 
succinylcholine 
CPK > 20,000

Question 7

Anytime we are using all this energy we are increasing our metabolic rate thus ___!
Essentially producing heat, but using a ton of oxygen, producing a ton of CO2. Expect ___ (___ from anaerobic metabolism and from CO2 production from aerobic metabolism). Also expect ___ potentially from using so much oxygen. Would also see ___ starting to go up.

Answer 7

temperature increases
acidosis 
lactic acidosis 
hypoxemia 
potassium

Question 8

Testing for Susceptibility to MH:

• 11 centers in North America (complete list at ?)
• Muscle Biopsy centers in US, Canada and Europe have standardized the ___ and determined its sensitivity and specificity.

Answer 8

• MHAUS

- contracture test

Question 9

• Molecular Genetic Testing for MH Growing Slowly
• Multiple mutations identified as being causal for MH, but mutations are not always found in MH patients
• ___ may be used for determining susceptibility in some individuals
• 3 labs currently perform in the US (send sample by mail)
• Insurance coverage of test is inconsistent
• MHAUS has program to help cover cost in patients with limited financial means and a ___.

Answer 9

• DNA

- positive contracture test

Question 10

MH treatment?
~very expensive to keep and expires relatively quickly
~lots of surgery centers try to cut costs by not supplying it
~refuse to work at place without Sux or Dantrolene

Answer 10

Dantrolene

Question 11

When used with ___ dantrolene sodium for infection may produce life threatening hyperkalemia and myocardial depression.

After MH episode resolves, patient may be switched to ?

Answer 11

Calcium Channel Blockers

oral dantrolene for several days