Malignant Hyperthermia Flashcards
MH is transmitted by
Autosomal Dominance
Where is the genetic defect located chromosomally
Long arm of chromosome 19
Where is the defect associated with MH
Ryanodine receptors
Ry1 (skeletal) only
True/False
Triggering of MH may not occur with a SINGLE anesthetic exposure
True- may trigger after several exposures
True/False
MH is a malignancy
False
True/False
MH causes hyperthermia
False
What is MH
A fulminant HYPERMETABOLIC state of skeletal muscle induced by VOLATILE inhalational anesthetics, succinylcholine, and perhaps by stress/excercise
** What is the MOA of MH
Release of substantial amounts of calcium into sarcoplasm caused by defective Ryd1 receptors, with reversal of usual gradients into the SR, mitochondria, and/or sarcolemma into extracellular fluid.
As a result, calcium pump requires excessive amounts of ATP leading to heat, acid, and C02 production which alters the energy supply and demand and causes cell breakdown, release of intracellular contents, and ischemia
How does Dantrolene stop MH
by stopping the loss of calcium from SR, but not interfering with calcium uptake. This allows the calcium pumps to catch-up with calcium demands
Name the triggering agents for MH
All halogenated agents including: Methoxyflurane, Cyclopropane, ether, and the rest
Succinylcholine
Decamethonium
List the Main clinical presentations of MH
SNS stimulation secondary to hypermetabolism TACHYCARDIA MASSETER MUSCLE RIGIDITY and generalized muscle rigidity HYPERCAPNIA INCREASED TEMP (1-2 degrees/5 min) hyperKalemia hyperCacemia myoglobinuria CPK > 20K Lactidemia r/t hypermetabolism Ventricular dysrhythmias
MMR occurs more commonly in
children
young adults
Most MMR occurs after which inhalation/muscle blockers
Halothane, Sevoflurane following succs administration
Will repeat doses of succs or NDMR relieve MMR
NO
What will the peripheral nerve stimulator reveal with MMR
flaccid paralysis
List the differential diagnosis for MMR
Myotonic syndrome TMJ dysfunction Underdosing with succs Not allow succs to work Increased resting tone after succ with fever or elevated epi levels
MH
List management strategies with MMR
DC anesthesia and postpone case if possible
continue anesthesia with non-triggering agent
give dantrolene, only if generalized rigidity/hypermetabolism noted
admit for 12-24 hours after episode
serial CKs, at 6,12,24 hours
* contracture test- only definitive diagnosis
Identify other dx’s associated with MH
King-denborough syndrome Duchenne muscular dystrophy Becker dystrophy Myopathies Myotonias Periodic paralysis Osteogenesis imperfecta
List syndromes with clinical resemblances to MH
Pheochromocytoma Thyrotoxicosis Sepis Hypoxic encephalopathy Mitochondrial myopathies NMS Serotonin Syndrome
Differences between pheochromocytoma and MH
Highest BP/ Tachy with pheo
No rigidity with pheo
CO2 less with pheo