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The New England Journal of Medicine Aug 1983
Review
Topics: Animals; Dantrolene; Disease Models, Animal; Halothane; Humans; Malignant Hyperthermia; Muscles; Swine; Swine Diseases
PubMed: 6348539
DOI: 10.1056/NEJM198308183090706 -
Comprehensive Therapy Dec 1975Malignant hyperpyrexia is a dangerous complication of general anesthesia occurring in individuals with an underlying disease of muscle. The essential clinical features... (Review)
Review
Malignant hyperpyrexia is a dangerous complication of general anesthesia occurring in individuals with an underlying disease of muscle. The essential clinical features of the syndrome are a drastic and sustained rise in body temperature, metabolic acidosis, and widespread muscular rigidity. The results of experiments on susceptible pigs and in vitro studies of human muscle have shown that all the clinical features of the syndrome can be explained by a raised level of calcium ions in the myoplasm. This is caused by a massive and sudden release of calcium into the myoplasm from the calcium-storing membranes in the muscle cell when exposed to general anesthetic agents. Two myopathies predisposing to malignant hyperpyrexia have been identified. One is usually subclinical, dominantly inherited, and manifested only by raised serum CPK levels. The other occurs in young boys with a number of physical abnormalities, whose relatives are unaffected. The serum CPK is a useful screening test in families in which malignant hyperpyrexia has occurred. Unfortunately, though, the serum CPK is not a specific test, and in doubtful cases the only unequivocal method of establishing susceptibility to malignant hyperpyrexia is to carry out an in vitro muscle test in which the muscle is exposed to caffeine, halothane, succinylcholine, and potassium chloride. Susceptible individuals should be given local, regional, or spinal anesthesia if an operation is needed. If this form of anesthesia is unsuitable, barbiturates such as thiopentone, tranquilizers such as diazepam, narcotics such as Pantopon, and neuroanaleptics such as fentanyl, nitrous oxide, d-tubocurarine, and althesin appear to be safe. By far the most important aspect of treatment is prophylaxis. Early diagnosis and immediate cessation of the offending anesthetic agents are the most important factors in trying to reduce the very high mortality of the syndrome.
Topics: Adenosine Triphosphate; Anesthesia, General; Animals; Calcium; Cold Temperature; Creatine Kinase; Humans; Lactates; Malignant Hyperthermia; Muscle Contraction; Muscular Diseases; Procaine
PubMed: 770064
DOI: No ID Found -
Annual Review of Medicine 1977Malignant hyperpyrexia is a genetically related syndrome that cannot be predicted reliably in advance of administration of anesthesia except by a strong family history.... (Review)
Review
Malignant hyperpyrexia is a genetically related syndrome that cannot be predicted reliably in advance of administration of anesthesia except by a strong family history. The definitive etiology is unknown, although triggering agents that release calcium from the calcium-storing sarcoplasmic membrane of the muscle cell are highly suspect. As soon as the syndrome is diagnosed, therapy must be prompt, vigorous, and carried out with the same urgency as a cardiac arrest. Specific therapy with dantrolene sodium may prove to be an answer to this serious problem in anesthetic practice.
Topics: Acute Kidney Injury; Adenosine Triphosphate; Anesthetics; Bicarbonates; Body Temperature; Calcium; Creatine Kinase; Humans; Malignant Hyperthermia; Muscle Contraction; Procainamide; Procaine; Sarcoplasmic Reticulum
PubMed: 324354
DOI: 10.1146/annurev.me.28.020177.001101 -
British Journal of Anaesthesia Mar 1973
Review
Topics: Adenosine Triphosphate; Anesthesia, General; Animals; Bicarbonates; Caffeine; Creatine Kinase; Glycolysis; Humans; Magnesium; Malignant Hyperthermia; Muscle Contraction; Muscular Diseases; Procaine; Prognosis; Swine
PubMed: 4581791
DOI: 10.1093/bja/45.3.269 -
Neurocritical Care 2009The parkinsonism-hyperpyrexia syndrome (PHS) is a rare but potentially fatal complication seen in Parkinson's disease (PD) patients, most commonly following reduction or... (Review)
Review
The parkinsonism-hyperpyrexia syndrome (PHS) is a rare but potentially fatal complication seen in Parkinson's disease (PD) patients, most commonly following reduction or cessation of antiparkinson medications. Clinically it resembles neuroleptic malignant syndrome with rigidity, pyrexia, and reduced conscious level. There may be features of autonomic instability, and serum creatine kinase (CK) may be elevated. Complications of PHS include acute renal failure, aspiration pneumonia, deep venous thrombosis/pulmonary embolism, and disseminated intravascular coagulation (DIC). Management consists of dopaminergic drug replacement, supportive measures, and treatment of complications. The prognosis is improved with early recognition and management. Mortality of up to 4% has been reported, but an additional one-third of patients have permanent sequelae. Patients and physicians should be warned against sudden reduction in antiparkinson medications. PHS should always be considered in a patient with parkinsonism who presents with an acute deterioration in symptoms.
Topics: Antiparkinson Agents; Fatal Outcome; Humans; Male; Malignant Hyperthermia; Middle Aged; Parkinsonian Disorders; Substance Withdrawal Syndrome; Syndrome
PubMed: 18712508
DOI: 10.1007/s12028-008-9125-4 -
Orphanet Journal of Rare Diseases Aug 2015Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as... (Review)
Review
Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane, isoflurane and the depolarizing muscle relaxant succinylcholine, and rarely, in humans, to stressors such as vigorous exercise and heat. The incidence of MH reactions ranges from 1:10,000 to 1: 250,000 anesthetics. However, the prevalence of the genetic abnormalities may be as great as one in 400 individuals. MH affects humans, certain pig breeds, dogs and horses. The classic signs of MH include hyperthermia, tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, hyperkalaemia, muscle rigidity, and rhabdomyolysis, all related to a hypermetabolic response. The syndrome is likely to be fatal if untreated. An increase in end-tidal carbon dioxide despite increased minute ventilation provides an early diagnostic clue. In humans the syndrome is inherited in an autosomal dominant pattern, while in pigs it is autosomal recessive. Uncontrolled rise of myoplasmic calcium, which activates biochemical processes related to muscle activation leads to the pathophysiologic changes. In most cases, the syndrome is caused by a defect in the ryanodine receptor. Over 400 variants have been identified in the RYR1 gene located on chromosome 19q13.1, and at least 34 are causal for MH. Less than 1 % of variants have been found in CACNA1S but not all of these are causal. Diagnostic testing involves the in vitro contracture response of biopsied muscle to halothane, caffeine, and in some centres ryanodine and 4-chloro-m-cresol. Elucidation of the genetic changes has led to the introduction of DNA testing for susceptibility to MH. Dantrolene sodium is a specific antagonist and should be available wherever general anesthesia is administered. Increased understanding of the clinical manifestation and pathophysiology of the syndrome, has lead to the mortality decreasing from 80 % thirty years ago to <5 % in 2006.
Topics: Genetic Counseling; Humans; Malignant Hyperthermia
PubMed: 26238698
DOI: 10.1186/s13023-015-0310-1 -
Advanced Emergency Nursing JournalMalignant hyperthermia (MH) is caused by a genetic disorder of the skeletal muscle that induces a hypermetabolic response when patients are exposed to a triggering agent... (Review)
Review
Malignant hyperthermia (MH) is caused by a genetic disorder of the skeletal muscle that induces a hypermetabolic response when patients are exposed to a triggering agent such as volatile inhaled anesthetics or depolarizing neuromuscular blockers. Symptoms of MH include increased carbon dioxide production, hyperthermia, muscle rigidity, tachypnea, tachycardia, acidosis, hyperkalemia, and rhabdomyolysis. Common scenarios for triggering agents are those used are during surgery and rapid sequence intubation. Hypermetabolic symptoms have a rapid onset; hence, prompt recognition and treatment are vital to prevent morbidity and mortality. The first-line treatment agent for an MH response is dantrolene. Further treatment includes managing complications related to a hypermetabolic response such as hyperkalemia and arrhythmias. This review is focused on the recognition and treatment considerations of MH in the emergency department to optimize therapy and improve patient morbidity and mortality.
Topics: Dantrolene; Diagnosis, Differential; Emergency Service, Hospital; Humans; Malignant Hyperthermia; Muscle Relaxants, Central; Risk Factors
PubMed: 33915557
DOI: 10.1097/TME.0000000000000344 -
Neurologia Mar 2018
Topics: Humans; Male; Malignant Hyperthermia; Middle Aged; Parkinsonian Disorders; Syndrome
PubMed: 26968824
DOI: 10.1016/j.nrl.2016.01.004 -
Anaesthesia and Intensive Care Aug 1977
Topics: Animals; Dantrolene; Humans; Male; Malignant Hyperthermia; Swine
PubMed: 900458
DOI: 10.1177/0310057X7700500301 -
The Medical Journal of Australia Dec 1977
Topics: Adult; Anesthesia, General; Animals; Child; Dantrolene; Humans; Male; Malignant Hyperthermia; Swine
PubMed: 611371
DOI: 10.5694/j.1326-5377.1977.tb99272.x