A Clinical Grading Scale to Predict Malignant Hyperthermia Susceptibility

Larach, Marilyn Green; Localio, A. Russell; Allen, Gregory C.; Denborough, M. A.; Ellis, F. R.; Gronert, Gerald A.; Kaplan, Richard F.; Muldoon, Sheila M.; Nelson, Thomas E.; Ørding, Helle; Rosenberg, Henry; Waud, B. E.; Wedel, Denise J.

doi:10.1097/00000542-199404000-00008

Cited by 504 publications

(318 citation statements)

References 0 publications

Supporting

Mentioning

293

Contrasting

Unclassified

Order By: Relevance

“…71 All of the reactions occurred in the immediate postoperative period, in the recovery room. Applying the MH clinical grading scale 72 to the clinical details available in these reports only two of these reactions were "very likely" to be MH; the other six were evenly split between "somewhat less than" or "somewhat more than" likely to be MH. The incidence of MH triggering in MHS patients undergoing a non-triggering anesthetic has been estimated to be less than 1%.…”

Section: Ma Al LI Ig Gn Na An Nt T H Hy Yp Pe Er Rt Th He Er Rm Mi mentioning

confidence: 99%

Patient selection in ambulatory anesthesia — An evidence-based review: part II

et al. 2004

View full text Add to dashboard Cite

P Pu ur rp po os se e: : This is the second of two reviews evaluating the management of patients with selected medical conditions undergoing ambulatory anesthesia and surgery. Conditions highlighted in this review include: diabetes mellitus; morbid obesity; the ex-premature infant; the child with an upper respiratory infection; malignant hyperthermia; and the use of monoamine oxidase inhibitors.S So ou ur rc ce e: : Medline search strategies and the framework for the evaluation of clinical evidence are presented in Part I. P Pr ri in nc ci ip pa al l f fi in nd di in ng gs s: : Diabetes mellitus has not been linked with adverse events following ambulatory surgery. The morbidly obese patient is at an increased risk for minor respiratory complications in the perioperative period but these events do not increase unanticipated admissions. The ex-premature infant may be considered for ambulatory surgery if post-conceptual age is > 60 weeks and hematocrit is > 30%. The child with a recent upper respiratory tract infection is at an increased risk for perioperative respiratory complications, particularly if endotracheal intubation is required. Patients with malignant hyperthermia may undergo outpatient surgery but require four hours of postoperative temperature monitoring. Sporadic cases of drug interactions have been reported when meperidine and indirectacting catecholamines are administered in the presence of monamine oxidase inhibitors. Ambulatory anesthesia and surgery is safe if these combinations of drugs are avoided.C Co on nc cl lu us si io on n: : Ambulatory anesthesia can be performed in, and is being offered to, a variety of patients with significant coexistent disease. In many cases there is little evidence documenting the outcomes expected in such patients. Prospective observational and interventional trials are required to better define perioperative management. Objectif : C'est la seconde revue qui évalue la prise en charge de patients, dont les pathologies médicales ont été ciblées, qui doivent subir une anesthésie en chirurgie ambulatoire. Les situations choisies comprennent : le diabète, l'obésité morbide, l'enfant né prématurément, l'enfant atteint d'infection des voies respiratoires supérieures, l'hyperthermie maligne et l'usage d'inhibiteurs de la monoamine-oxydase. Source : Les stratégies de recherche dans Medline et le cadre de l'é-valuation de la preuve clinique sont présentés dans la partie I. Constatations principales : Le diabète n'a pas été relié à des événements indésirables à la suite d'une opération ambulatoire. Le patient très obèse est plus à risque de complications respiratoires péri-opératoires mineures, ce qui n'augmente pas les admissions hospitalières imprévues. L'enfant prématuré est admis en chirurgie ambulatoire si l'âge post-conception est > 60 semaines et si l'hématocrite est > 30 %. L'enfant qui a une infection récente des voies respiratoires supérieures

show abstract

Section: Ma Al LI Ig Gn Na An Nt T H Hy Yp Pe Er Rt Th He Er Rm Mi mentioning

confidence: 99%

Patient selection in ambulatory anesthesia — An evidence-based review: part II

et al. 2004

View full text Add to dashboard Cite

show abstract

“…Those suffering an MH reaction are deduced to have the MHS phenotype. However, the spectrum of responses to anesthetics is so varied that a clinical grading scale has been developed which classifies potential MH probands into 6 groups, D1 to D6, on the basis of clinical signs and symptoms [43]. D1 almost certainly signifies no MH, while D6 is almost certainly an MH reaction.…”

Section: T H E M O L E C U L a R B A S I S F O R M A L I G N A N T H mentioning

confidence: 99%

Ca²⁺ signalling and muscle disease

MacLennan

2000

European Journal of Biochemistry

218

155

View full text Add to dashboard Cite

Transient elevations of intracellular Ca2+ play a signalling role in such complex cellular functions as contraction, secretion, fertilization, proliferation, metabolism, heartbeat and memory. However, prolonged elevation of Ca2+ above about 10 µm is deleterious to a cell and can activate apoptosis. In muscle, there is a narrow window of Ca2+ dysregulation in which abnormalities in Ca2+ regulatory proteins can lead to disease, rather than apoptosis. Key proteins in the regulation of muscle Ca2+ are the voltage‐dependent, dihydropyridine‐sensitive, l‐type Ca2+ channels located in the transverse tubule and Ca2+ release channels in the junctional terminal cisternae of the sarcoplasmic reticulum. Abnormalities in these proteins play a key role in malignant hyperthermia (MH), a toxic response to anesthetics, and in central core disease (CCD), a muscle myopathy. Sarco(endo)plasmic reticulum Ca2+ ATPases (SERCAs) return sarcoplasmic Ca2+ to the lumen of the sarcoplasmic reticulum. Loss of SERCA1a Ca2+ pump function is one cause of exercise‐induced impairment of the relaxation of skeletal muscle, in Brody disease. Phospholamban expressed in cardiac muscle and sarcolipin expressed in skeletal muscle regulate SERCA activity. Studies with knockout and transgenic mice show that gain of inhibitory function of phospholamban alters cardiac contractility and could be a causal feature in some cardiomyopathies. Calsequestrin, calreticulin, and a series of other acidic, lumenal, Ca2+ binding proteins provide a buffer for Ca2+ stored in the sarcoplasmic reticulum. Overexpression of cardiac calsequestrin leads to cardiomyopathy and ablation of calreticulin alters cardiac development.

show abstract

“…The clinical signs of an impending MH reaction are highly variable and are caused by a hypermetabolic state with muscle rigidity, metabolic acidosis, rhabdomyolysis, tachycardia, and/or an increase in body temperature (5). In some individuals MH reactions appear to be triggered by physical exercise or emotional stress.…”

Section: Malignant Hyperthermia (Mh)mentioning

confidence: 99%

B-lymphocytes from Malignant Hyperthermia-susceptible Patients Have an Increased Sensitivity to Skeletal Muscle Ryanodine Receptor Activators

Girard

Cavagna

Padovan

et al. 2001

Journal of Biological Chemistry

View full text Add to dashboard Cite

Malignant hyperthemia (MH) is a pharmacogenetic disease triggered by volatile anesthetics and succinylcholine in genetically predisposed individuals. The underlying feature of MH is a hypersensitivity of the calcium release machinery of the sarcoplasmic reticulum, and in many cases this is a result of point mutations in the skeletal muscle ryanodine receptor calcium release channel (RYR1). RYR1 is mainly expressed in skeletal muscle, but a recent report demonstrated the existence of this isoform in human B-lymphocytes. As B-cells can produce a number of cytokines, including endogenous pyrogens, we investigated whether some of the symptoms seen during MH could be related to the involvement of the immune system. Our results show that (i) Epstein-Barr virus-immortalized B-cells from MH-susceptible individuals carrying the V2168M RYR1 gene mutation were more sensitive to the RYR activator 4-chloro-m-cresol and (ii) their peripheral blood leukocytes produce more interleukin (IL)-1␤ after treatment with the RYR activators caffeine and 4-chloro-m-cresol, compared with cells from healthy controls. Our result demonstrate that RYR1-mediated calcium signaling is involved in release of IL-1␤ from B-lymphocytes and suggest that some of the symptoms seen during an MH episode may be due to IL-1␤ production. Malignant hyperthermia (MH)1 is a pharmacogenetic disease triggered by volatile anesthetics and the depolarizing muscle relaxant succinylcholine in predisposed individuals (1-4). The clinical signs of an impending MH reaction are highly variable and are caused by a hypermetabolic state with muscle rigidity, metabolic acidosis, rhabdomyolysis, tachycardia, and/or an increase in body temperature (5). In some individuals MH reactions appear to be triggered by physical exercise or emotional stress. The latter observation has led to the suggestion that MH, heat stroke, and exercise-induced rhabdomyolysis might have a common denominator (2, 6, 7). The underlying causes of MH are abnormalities in the skeletal muscle calcium metabolism (8, 9) and molecular genetic studies have mapped the primary locus of MH to chromosome 19q, the gene encoding the ryanodine receptor calcium release channel (RYR1) (2, 4, 10). Approximately 50% of MH families have mutations in the RYR1 gene, and mutations have been reported in other loci (for recent reviews, see Refs. 11 and 12).The ryanodine receptors are large tetrameric oligomers that function as intracellular calcium release channels. Three different isoforms have been identified at the molecular level: type 1 (RYR1), which is preferentially expressed in skeletal muscle; type 2, which is in the heart and cerebellum; and type 3, which is in the central nervous system as well as in a variety of other tissues (13-16). RYR1 can be pharmacologically activated by a number of compounds, among which are caffeine, halothane, thymol, 4-chloro-m-cresol, E218, bastadin, polylysine, and calcium (17-21). Activation causes the channel to open and thus to a transient calcium flow from the sarcoplasmic reticulum,...

show abstract

A Clinical Grading Scale to Predict Malignant Hyperthermia Susceptibility

Cited by 504 publications

References 0 publications

Patient selection in ambulatory anesthesia — An evidence-based review: part II

Patient selection in ambulatory anesthesia — An evidence-based review: part II

Ca²⁺ signalling and muscle disease

B-lymphocytes from Malignant Hyperthermia-susceptible Patients Have an Increased Sensitivity to Skeletal Muscle Ryanodine Receptor Activators

Contact Info

Product

Resources

About

A Clinical Grading Scale to Predict Malignant Hyperthermia Susceptibility

Cited by 504 publications

References 0 publications

Patient selection in ambulatory anesthesia — An evidence-based review: part II

Patient selection in ambulatory anesthesia — An evidence-based review: part II

Ca2+ signalling and muscle disease

B-lymphocytes from Malignant Hyperthermia-susceptible Patients Have an Increased Sensitivity to Skeletal Muscle Ryanodine Receptor Activators

Contact Info

Product

Resources

About

Ca²⁺ signalling and muscle disease