Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024591 (malignant hyperthermia)
2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
...
PMID:Malignant hyperpyrexia. 77 64

The intra- and postoperative course of 30 general and 3 regional anesthetics in 27 MH-carriers verified by in vitro contracture tests is reported. None of the patients received dantrolene prophylactically. Disposable tubings were used for ventilation, vaporizers and soda lime were removed. ECG, esophageal temperature, blood pressure, oxygen saturation, and end tidal pCO2 were monitored. Minor tranquilizers were offered for premedication. Fentanyl, thiopentone, nitrous oxide, non depolarizing relaxants, neuromuscular antagonists and naloxone were used. In three patients, surgery was performed during epidural or spinal anesthesia with the use of amide local anesthetics. Neither MH-related changes in perioperative heart rates, body temperatures, and CK levels nor any other symptoms of MH were observed in any patient. The anesthetic techniques used seem to be safe and reliable; the anesthetic management of known MHS patients is discussed in detail.
...
PMID:Anesthesia in malignant hyperthermia susceptible patients. 197 53

Malignant hyperthermia (MH) is a rare genetic myopathy whose hallmark is rise in body temperature. This hypermetabolic state is triggered by inhalational anesthetics and/or depolarizing muscle relaxants such as succinylcholine. Even the use of dantrolene may not be protective against the hypermetabolic crisis. Eight patients at risk for MH undergoing tonsillectomy, adenoidectomy, and/or myringotomy with ventilation tube insertion were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers, and nondepolarizing muscle relaxants without dantrolene and without complication. Cardiac monitoring and rectal temperatures were followed. In order to provide additional evidence, all eight patients had vastus lateralis muscle biopsies with subsequent caffeine-halothane contracture studies performed. The contracture study showed positive results in seven of eight patients studied, indicating MH-susceptible muscle. No anesthetic or operative complications were encountered. This study demonstrates that patients at risk of developing MH crisis can have otolaryngologic procedures performed relatively safely while undergoing appropriately selected anesthesia.
...
PMID:Malignant hyperthermia in the otolaryngologic patient: prospective anesthetic and surgical management of eight children. 232 99

Malignant hyperthermia (MH) is a seemingly rare genetic myopathy. Hypermetabolic crisis accompanied by a rise in body temperature to as high as 44 degrees C, is its hallmark. Malignant hyperthermia is usually triggered by potent inhalation anesthetics and/or depolarizing muscle relaxants. Because of the extraordinary incidence of death in patients who are at risk, pediatric surgeons may be reluctant to operate on these patients. Eight such patients were referred to the Pediatric Surgery Service and the UCLA Malignant Hyperthermia Center following pediatric surgical procedures aborted for first episodes of malignant hyperthermia (five) or for a strong family history of malignant hyperthermia (three). They were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers, and nondepolarizing muscle relaxants. The patients were not treated prophylactically with dantrolene. Cardiac monitoring, end-tidal PCO2, and rectal temperatures were monitored. After completion of their pediatric surgical procedures, all eight patients had a vastus lateralis muscle biopsy performed and subsequent caffeine/halothane contracture studies completed. The contracture study result was positive in all patients studied. No anesthetic or surgical complications were encountered. This study shows that patients at risk for developing MH crisis can have pediatric surgical procedures performed safely with appropriately selected general anesthesia.
...
PMID:Malignant hyperthermia: experience in the prospective management of eight children. 272 6

Malignant hyperthermia is a seemingly rare genetic myopathy. Hypermetabolic crisis accompanied by a rise in body temperature to as high as 44 degrees C is its hallmark. Malignant hyperthermia is usually triggered by potent inhalated anesthetics and/or depolarizing muscle relaxants. Because of the extraordinary risk of death in patients who are at risk, plastic surgeons may be reluctant to operate on these patients. Five such patients were referred to the Plastic Surgery Service and the UCLA Malignant Hyperthermia Center for anesthetic and surgical management following plastic surgical procedures aborted for first episodes of malignant hyperthermia. They were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers, and nondepolarizing muscle relaxants. The patients were not treated prophylactically with dantrolene. Cardiac monitoring, end-tidal pCO2, and rectal temperatures were followed. After completion of their plastic surgical procedures, all five patients had a vastus lateralis muscle biopsy performed and subsequent caffeine/halothane contracture studies completed. The contracture study was positive in all patients studied. No anesthetic or surgical complications were encountered. This study demonstrates that patients at risk of developing malignant hyperthermia crisis can have plastic surgical procedures performed safely while undergoing appropriately selected general anesthesia.
...
PMID:Malignant hyperthermia in plastic surgery. 317 76

Malignant hyperthermia (MH) is a seemingly rare genetic myopathy. Hypermetabolic crises accompanied by a rise in body temperature to as high as 44 degrees C are its hallmark. These are usually triggered by potent inhalation anesthetics or depolarizing muscle relaxants. Because of the extraordinary risk of death in patients who are at risk, otolaryngologists may be reluctant to operate on these patients. Ten such patients undergoing tonsillectomy and adenoidectomy, myringotomy with ventilation tube insertion, and nasal polypectomy were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers and non-depolarizing muscle relaxants without complication. The patients were not treated prophylactically with dantrolene. Cardiac monitoring and rectal temperatures were followed. All ten patients had vastus lateralis muscle biopsy performed and subsequent caffeine/halothane contracture studies completed. The contracture study was positive in eight out of the patients studied. No anesthetic or surgical complications were encountered. This study demonstrates that patients at risk of developing MH crisis can have otolaryngologic procedures performed safely while undergoing appropriately selected general anesthesia.
...
PMID:Prospective management of malignant hyperthermia in the otolaryngological patient. 339 52

Malignant hyperthermia is a seemingly rare genetic myopathy. Hypermetabolic crisis accompanied by a rise in body temperature to as high as 44 degrees C is its hallmark. Malignant hyperthermia is usually triggered by potent inhaled anesthetics or depolarizing muscle relaxants. Because of the extraordinary risk of death in patients who are susceptible, neurosurgeons may be reluctant to operate on these patients. Three such patients were referred to the Neurosurgery Service and the UCLA Malignant Hyperthermia Center after neurosurgical procedures aborted for first episodes of malignant hyperthermia. They were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers, and nondepolarizing muscle relaxants. The patients were not treated prophylactically with dantrolene. Cardiac monitoring, end-tidal pCO2, and rectal temperatures were followed. After completion of the neurosurgical procedures, all three patients had a vastus lateralis muscle biopsy and subsequent caffeine/halothane contracture studies. The contracture study was positive in all patients. No anesthetic or surgical complication was encountered. This study demonstrates that neurosurgical procedures can be performed safely in patients at risk of developing malignant hyperthermia while they undergo appropriately selected general anesthesia.
...
PMID:Neurosurgery in the malignant hyperthermia-susceptible patient. 341 64

Seven episodes of rhabdomyolysis with acute renal failure (ARF) have been observed in 6 patients treated with various short-acting tranquilizers and antidepressants. Clinical features usually included severe hyperthermia, diffuse hypertonicity with or without coma, circulatory failure or unstable blood pressure, and often acute respiratory failure. Serum CPK were always elevated. The type of ARF was prerenal failure without oliguria in 5/7 episodes, and acute tubular necrosis in 2/7 episodes, requiring hemodialyses in one patient. Three patients died. In any case, the tranquilizers and antidepressants responsible for this syndrome were stopped, and electrolyte disorders and acidosis were corrected. Associated acute circulatory failure, septicemia and/or acute hepatic failure required prompt therapy, and artificial ventilation was required in 4 instances. The further use of phenothiazines, butyrophenones, sulpiride and their derivatives should be avoided in any patient having developed such an accident, whose pathophysiology is similar to that described in malignant hyperthermia of various origin.
...
PMID:[Rhabdomyolysis with acute renal failure and malignant neuroleptic syndrome]. 613 93

Malignant hyperthermia is a seemingly rare genetic myopathy. Hypermetabolic crisis accompanied by a rise in body temperature to as high as 44 degrees C is its hallmark. Malignant hyperthermia is usually triggered by potent inhalational anesthetics and/or depolarizing muscle relaxants. Because of the extraordinary risk of death in patients who are at risk, otologists may be reluctant to operate on these patients. Seven such patients were referred to the Section of Otology, Neurotology, and Skull Base Surgery and the UCLA Malignant Hyperthermia Center Following first episodes of malignant hyperthermia or with a strong family history of malignant hyperthermia for anesthetic and surgical management. They were anesthetized with nitrous oxide, barbiturates, opiates, tranquilizers, and nondepolarizing muscle relaxants. The patients were not treated prophylactically with dantrolene. Cardiac monitoring, end-tidal PC02, and rectal temperatures were followed. All seven patients had a vastus lateralis muscle biopsy performed and subsequent caffeine/halothane contracture studies completed. The contracture study was positive in six of the seven patients studied. No anesthetic or surgical complications were encountered. This study demonstrates that patients at risk of developing malignant hyperthermia crisis can have otologic surgical procedures performed safely while undergoing appropriately selected general anesthesia.
...
PMID:Malignant hyperthermia in the otology patient: the UCLA experience. 857 42