MH is
a life-threatening clinical hypermetabolic syndrome involving skeletal muscle
MH is triggered by
T/F MH is an allergy
False!
it is an inherited disorder
__ genetic defects are associated with MH
(PPT)
80
on average a patient requires how many anesthetics before a triggering event occurs?
3
but this crisis may delevlop at first exposure to anesthesia
MH complicates 1 in how many surgical procedures in adults? and 1 in how many surgeries of children?
100,000
30,000
(pg. 841)
children under what age approximate 52% of all reactions
15 years of age
MH is more sustible in males or females?
males
3 co-existing disease are defineitly associated with MH
factors that increase risk
what is the most common myopathy that triggers MH
Evans myopthay
What other genes are linked with MH suceptibility ?
CACNA1S
STAC3
all myopathies have what gene dysfunction?
RYR1
MH-associated genetic variants are found within what gene?
RYR1
RYR1
encodes the ryanodine receptor, the major calcium release channel of the SR
STAC3
gene variants are manifested most as native american myopathy in the Lumbee Native American tribe of North Cariolina
CACNA1S
encodes a subunit of a dihydropyridine calcium channel located in the skeletal muscle T tubule
the elevated IC calcium results in
muscle contraction and abnormal muscle metabolism
energy-dependent reuptake mechanisms attempt to remove excess calcium from the myoplasm, increasing muscle metabolism how many folds?
2-3
this accelerated cellular process does what?
what lab values markedly increase in the EC?
what 3 things cause saroplama destruction?
Patients with DMD have an absence of __ which stabilizes the sarcolemma during muscle contraction & increases membrane permeability
dystrophin
what happens at the cellular level with DMD