[Malignant hyperthermia as a complication of anesthesia: predisposition is hereditary].
The frequency of malignant hyperthermia in the Netherlands is about 1 in 200,000 anaesthesias. Five times a year, an anaesthetic procedure will be complicated by a malignant hyperthermic metabolic disturbance, which can cause death if treatment is not instituted rapidly, by the administration of dantrolene. Suxamethonium and all the anaesthetic vapours can trigger such a reaction. Malignant hyperthermia patients are healthy patients who have a mutation of the ryanodine receptor gene RYR. Predisposition to malignant hyperthermia is inherited as an autosomal dominant condition. So far a genetic malignant hyperthermia test is not available because of genetic heterogeneity. The in-vitro contracture test in skeletal muscle is currently used as a diagnostic test for malignant hyperthermia. Patients who are likely to be at risk based on a clinical grading score, and family members with at least a 25% chance of inheriting malignant hyperthermia, are eligible for this test.
Snoeck
M M
MM
Afd. Anesthesiologie, Academisch Ziekenhuis, Nijmegen.
Gielen
M J
MJ
Sengers
R C
RC
Padberg
G W
GW
Iles
D E
DE
Booij
L H
LH
D015220:Calcium Channels / Q000235:genetics; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D005799:Genes, Dominant; D020022:Genetic Predisposition to Disease; D006801:Humans; D008305:Malignant Hyperthermia / Q000175:diagnosis / Q000235:genetics*; D009124:Muscle Proteins / Q000235:genetics; D017354:Point Mutation; D019837:Ryanodine Receptor Calcium Release Channel
D015220:Calcium Channels; D002148:Calmodulin-Binding Proteins; D009124:Muscle Proteins; D019837:Ryanodine Receptor Calcium Release Channel