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

Evidence is presented that serum creatinine phosphokinase (CPK) activity is of no direct value in screening patients for susceptibility to malignant hyperpyrexia and does not correlate with halothane-induced muscle contracture or the presence of myopathy. Widely differing CPK values were found at different times in the same people. In most "malignant hyperpyrexia" families the susceptible patients had either normal or inconsistently raised CPK values.
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PMID:Evaluation of creatinine phosphokinase in screening patients for malignant hyperpyrexia. 116 12

Dantrolene, a direct acting muscle relaxant used orally for spasticity, has appeared to be effective in the prevention and treatment of malignant hyperthermia in man and animals when administered intravenously. Its pharmacokinetics following intravenous administration have been studied in dogs. Concentrations of dantrolene and its metabolites in plasma, urine, and bile were determined by high-performance liquid chromatography. Recovery of unchanged drug and reduced metabolites was negligible; of the hydroxy metabolite 2% was found in the urine and about 25% in the bile. The half-life of 5-hydroxydantrolene was shorter than that of the parent drug as demonstrated by administration of the metabolite. The apparent renal clearance of 5-hydroxydantrolene was independent of creatinine clearance, urine flow and pH, and appeared to be reduced in the presence of probenecid. Bile to plasma ratios of the hydroxy metabolite were high with biliary concentrations far exceeding the maximum solubility in water. The results of this pilot study indicate that hydroxylation is primarily responsible for the excretion of the dantrolene molecule from the body.
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PMID:Pharmacokinetics of intravenously administered dantrolene and its 5-hydroxy metabolite in dogs. 207 79

A sixteen-year-old male with sickle cell anemia and congenital strabismus developed malignant hyperthermia a few minutes after the administration of succinylcholine, used as the general anesthetic for corrective eye surgery. The patient's hemoglobin S level was reduced to fifteen percent before the operation. He recovered uneventfully within a few hours. Increased serum creatinine phosphokinase activity and pathological changes observed in the muscle biopsy along with strabismus suggest that the patient had an inherited susceptibility to malignant hyperthermia.
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PMID:Malignant hyperthermia in a patient with sickle cell anemia. 248 91

Dantrolene sodium, a skeletal-muscle relaxant known to be effective for treatment of malignant hyperthermia, was evaluated for efficacy in treatment of heatstroke. Non-exertional heatstroke was induced in 11 dogs by external heating following barbiturate anesthesia. When core temperature reached 43 degrees C (109.4 degrees F) heating was discontinued and control animals (n = 6) were allowed to cool passively in room air. Treatment animals (n = 5) received 5 mg/kg dantrolene sodium intravenously at the start of room-air cooling. Serial temperatures (pulmonary arterial, rectal, cerebral, and subcutaneous), blood chemistry tests (including electrolytes, liver enzymes, and complete blood count), and hemodynamic parameters (including cardiac output, arterial pressure, and urinary output) were followed for 12 hours after induction of heatstroke. Autopsies, including gross and microscopic examination, were performed on all animals. Dantrolene administration did not significantly affect cooling rates, hemodynamic parameters, pathological changes, or clinical outcome. Statistically significant changes in urinary output and serum creatinine observed in the first hours after dantrolene administration can be attributed to the mannitol vehicle in which the drug was delivered. There were no statistically significant differences in these values at 12 hours. Dantrolene sodium does not appear to enhance passive cooling in treatment of non-exertional canine heatstroke.
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PMID:Dantrolene sodium for treatment of heatstroke victims: lack of efficacy in a canine model. 374 58

A young healthy male, who had three consecutive episodes of postoperative hyperthermia was anaesthetized with special precautions to prevent malignant hyperthermia. Despite neuroleptic anaesthesia and dantrolene pretreatment, the patient experienced post-anaesthetic hyperthermia. The patient's clinical picture was almost identical to the symptoms experienced by two of his maternal relatives. All three experienced nausea, vomiting, muscle cramps and high fever which occurred between five to seven hours after general anaesthesia. The serum potassium (K) and creatinine phosphokinase (CPK) levels determined during the hyperthermic episode and on the next day were not elevated. On the basis of the patient's family history, his clinical picture, and his laboratory data, we speculate that this patient might have a form of malignant hyperthermia or a possible new variant.
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PMID:Malignant hyperthermia: a possible new variant. 400 78

The efficiency, sensitivity and specificity of measurements of serum creatinine phosphokinase in the screening of malignant hyperthermia-susceptible patients in routine clinical practice has been examined. Although the overall efficiency of this screening method was high, the predictive value of a positive test in the ambulatory surgical population was poor.
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PMID:Evaluation of creatinine phosphokinase screening as a predictor of malignant hyperthermia. A prospective study. 686 May 21

A case of severe muscular rigidity in a premature male infant born by Caesarean Section under general anaesthesia is described. A probable diagnosis of malignant hyperthermia was supported by the clinical symptoms of muscular rigidity and cyanosis, a creatinine phosphokinase of 24,630 I.U. (Normal 0--100) and a urinary myoglobin of 248 mg/l (normal 6--35). The muscle tone and laboratory values slowly returned to normal over a period of days. Anaesthetic management for a ventriculoperitoneal shunt performed at seven weeks of age included pre and postoperative treatment with dantrolene. No crisis occurred at this time. The parents, who have normal CPK and a negative family history, were advised to treat the child as if he had malignant hyperthermia until such time as a definitive diagnosis can be made.
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PMID:Severe muscular rigidity at birth: malignant hyperthermia syndrome? 737 67

A 25-year-old man admitted with severe upper torso trauma displayed masseter muscle spasm after suxamethonium given during resuscitation. Anaesthesia was initially maintained with intravenous agents during transfer and X-ray angiography. However, during surgery to correct a brachial artery injury, malignant hyperthermia was triggered when isoflurane was given, 2.5 h after the suxamethonium. He responded to treatment, including dantrolene administration. Peak serum and urine myoglobin were 12,947 micrograms.l-1 and 54,571 micrograms.l-1, respectively, while maximum serum creatinine kinase was 17,300 IU. The patient made an uneventful recovery and later proved positive for malignant hyperthermia susceptibility on muscle contracture tests.
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PMID:Suxamethonium, masseter spasm and later malignant hyperthermia. 1002 82

A case of rhabdomyolysis from malignant hyperthermia occurred during renal transplantation surgery is presented. After the completion of vascular and uretherovesical anostomosis, the patient's heart rate began to rise, sweatiness was observed and body temperature increased to 41 degrees C. Additionally, metabolic and respiratory acidosis and hyperkalemia were detected. Serum creatine kinase and lactic dehydrogenase levels were increased significantly. After external cooling and the administration of dantrolene sodium, body temperature and heart rate were decreased. During this period; furosemide, mannitol and sodium bicarbonate were given. Three hours after the completion of surgery, urine output was begun and urine myoglobin was found to be positive. Renal function improved gradually and serum creatinine level decreased to 1.6 mg/dl on the 14th postoperative day. Malignant hyperthermia can lead to severe rhabdomyolysis and delayed graft function in renal transplant recipients. Early diagnosis and intervention is crucial for protecting renal function.
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PMID:Severe rhabdomyolysis due to malignant hyperthermia during renal transplantation procedure can cause delayed graft function. 1191 7

A 52-year-old man experienced progressive tonic-clonic activity soon after undergoing a myelogram accompanied by an intrathecal injection of Omnipaque. The activity progressed to seizures, hyperthermia, and acidosis. He was intubated, cooled, and treated symptomatically. His creatinine kinase rose to 60,000 IU/L. He eventually recovered completely. This distinct set of clinical signs renders the syndrome easily recognizable. Although this syndrome superficially resembles malignant hyperthermia, the pathophysiology is different. Survival depends on prompt recognition and rapid symptomatic treatment. Treatment with dantrolene sodium is not necessary.
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PMID:Ascending tonic-clonic syndrome secondary to intrathecal Omnipaque. 1526 25


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