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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant hyperpyrexia is a highly lethal complication of general anesthesia. It occurs with sufficient frequency, particularly in children and young adults, to justify use of continuous temperature monitoring during adminnstration of general anesthesia. Fever may be a late manifestation of the malignant hyperpyrexia syndrome, and it is important to be aware of the early signs. Prompt recognition and aggressive management should lower the high mortality known to occur with this complication. Family members should be screened for CPK elevations to determine possible susceptibility to malignant hyperpyrexia. Two case reports illustrate these points of diagnosis and management.
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PMID:Malignant hyperpyrexia during anesthesia in childhood. 126 56

We have experienced a case of fulminant malignant hyperthermia who was a 63-year-old female weighing 44 kg. There was no particular past history nor family history. She underwent right mastoidectomy because of chronic otitis media. Her preoperative physical status was ASA I. She was premedicated with diazepam 10 mg and loxatigine 75 mg P.O. The induction was done with thiamylal 200 mg IV and fentanyl 0.1 mg IV followed by vecuronium 6 mg IV for endotracheal intubation. Intubation was easy and uneventful. Anesthesia was maintained with nitrous oxide 3 l.min-1, oxygen 3 l.min-1 and enflurane 2.0%. Seventy min after the induction of anesthesia, arterial blood gas analysis showed severe respiratory acidosis (PCO2: 63.2 mmHg, pH: 7.27) and it was improved with manual hyperventilation at that time. Pulse rate increased from 80 to 115 b.p.m. 20 minutes later. Then, the patient was ventilated with 100% oxygen, and anesthetic circuits and machine were exchanged for new units. Surgery was postponed. Muscle stiffness of upper extremities was observed and her temperature increased to a maximum of 38.9 degrees C. Surface cooling was started and dantrolene sodium 60 mg and furosemide 20 mg were given intravenously. The patient was transferred to the intensive care unit, and clinical signs improved gradually within one hour. Serum enzymes; CPK, LDH, GOT and GPT increased on the first postoperative day. On the 11 th postoperative day skeletal muscle biopsy was done under local anesthesia with 1% procaine and Ca-induced Ca-release rate test revealed positive for enflurane. This is the oldest patient of malignant hyperthermia reported in Japan.
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PMID:[A case report of a 63-year-old patient with malignant hyperthermia]. 161 62

A 5-year-old boy with acute lymphatic leukemia in remission developed signs of malignant hyperthermia (MH) during general anesthesia for removal of a central venous access port. The anesthetic procedure for implantation of the port 17 months before had been uneventful despite use of the same triggering agents, halothane and succinylcholine. Meanwhile, the patient had received chemotherapy (COALL-03-85). The first sign of MH was masseter spasm following succinylcholine; then tachycardia, acidosis, myoglobinuria, and CPK elevation (8953 IU) appeared. There was only moderate temperature elevation to 37.8 degree C. Rapid improvement and complete recovery occurred after dantrolene i.v. The patient's father was found to have undiagnosed muscle pain and an elevated CPK level. An in vitro contracture test with halothane and caffeine revealed susceptibility to MH and supported the patient's diagnosis and genetic predisposition. Referring to several other cases in the literature concerning MH in patients with lymphomas and leukemias, a possible correlation between the two diseases is discussed. As the MH crisis in our patient was most probably genetic in origin, a common acquired cause such as a viral infection seems less probable. We do not believe the chemotherapy our patient received between the two anesthetics was the cause since about one-half of the patients in the literature had not had chemotherapeutic pretreatment at the time of the MH crisis. We believe that a common genetic predisposition is the most likely link between the two diseases. In any case, patients with leukemias and lymphomas should be monitored very carefully for symptoms of MH.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Malignant hyperthermia in a child with acute lymphatic leukemia]. 186 72

The ideal screening test for malignant hyperthermia susceptibility (MHS) has yet to be discovered. It should be simple noninvasive, yet totally specific and sensitive. Until such an ideal test becomes available, allowing simple routine preoperative screening, tests should only be used in certain specific situations. These include: patients in whom a clinical crisis was suspected; the members of the family of a subject labeled MHS because of a fatal, or otherwise, crisis, or in whom tests were positive; patients with other pathological conditions which could be linked to malignant hyperthermia (MH) (some myopathies, effort or stress MH, neuroleptic malignant syndrome). The various tests proposed in the literature aim at revealing MHN subjects, using or not a triggering agent, halothane most often. However, detecting these abnormalities sometimes gives greater insight into the physiopathology of MH than in the detection of an individual patient's susceptibility. The tests have been classified as in vivo, electrophysiological, blood, and in vitro muscle biochemical, morphological, and pharmacological tests. The discovery of new tests gives renewed hope: CPK levels, platelet tests, calcium sarcoplasmic reticular reuptake, lymphocyte Quin 2 test, nuclear magnetic resonance spectroscopy. However, experts worldwide agree that the only reference test to this day remains the in vitro halothane caffeine contracture tests. These tests have shown their reliability; they must be performed on muscle strips obtained from surgically removed muscle biopsies, by laboratories used to this technique and who have at their disposal a sufficiently large group of MHS subjects with a clear-cut clinical crisis, as well as controls. The patients must therefore travel to these laboratories. The design of common protocols for European laboratories on one hand, and the North American laboratories on the other, is a good guarantee of the reliability of these tests.
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PMID:[Screening tests for malignant hyperthermia susceptibility]. 256 Jun 12

Twenty-seven patients, four of whom had presented with a crisis of malignant hyperthermia and the 23 other being close relatives of such patients, underwent a muscle biopsy so as to determine their susceptibility to malignant hyperthermia. Halothane-caffeine contracture tests, interpreted in accordance with the criteria of the European Group on Malignant Hyperthermia, yielded the following results: 13 positive (MHS), 10 negative (MHN), 4 equivocal (MHE). The history, clinical examination, CPK level, histoenzymatic morphology and electron microscopic study did not provide information sensitive enough to use for the detection of susceptibility to malignant hyperthermia. This confirmed the literature: the halothane-caffeine contracture test remains the only reliable diagnostic test to detect this susceptibility, despite the search for non invasive tests. If the mechanism of triggering a contracture to increasing doses of caffeine is well known in normal muscle, it is the smaller concentrations required which suggests malignant hyperthermia abnormality. The halothane effect is less well understood. A concentration less than or equal to 2 vol % yields a contracture only in MHS muscle. Differences in protocols used by American authors emphasize the importance of standardization as used by the European Group, which is the only way of collecting and comparing results on well over a thousand patients. This confrontation should reduce the number of equivocal diagnoses and allow a correct classification of patients at risk or their relatives as MHS or MHN.
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PMID:[Tests of contracture and sensitivity to malignant hyperthermia in 27 patients]. 336 12

The history, clinical presentation, and management of malignant hyperpyrexia are presented. The aetiology seems to be associated with some inherited abnormality which affects the movement and binding of calcium ions in the sarcoplasmic reticulum, sarcoplasm, and mitochondria. Whether this is a primary muscular defect or secondary to some trophic neural influence is yet to be established. The subjects carrying the abnormal trait show evidence of a myopathy which is subclinical in most instances and revealed only by estimation of serum CPK or biopsy. In some families where the myopathy is clinically obvious there may be, in addition, a variety of musculoskeletal abnormalities. A plea is made for routine monitoring of temperature during anaesthesia and for procainamide or procaine to be readily available in all operating theatres. A history of anaesthetic deaths in a family calls for special care, and, if the serum CPK is elevated, suxamethonium and halothane are to be avoided. Families with orthopaedic and muscular abnormalities are at increased risk and should have estimation of serum CPK before surgery. As a bonus of this study it is suggested that serum CPK estimations be used to screen pigs for selective breeding and so eliminate the disease, which causes soft exudative pork.
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PMID:Malignant hyperpyrexia. Further muscle studies in asymptomatic carriers identified by creatinine phosphokinase screening. 470 57

A 51-year-old male patient with no history of musculo-skeletal or myopathic abnormalities, but suffering from manic-depressive psychosis, attempted suicide with an overdose of dolpersin hydrochloride (Mydocalm), dipenzepine hydrochloride (Noveril), meprobamate (Mepronox) and nitrazepam (Mogadon). He developed high fever, muscle rigidity, tachycardia, arrhythmias, hypotension and mottled cyanosis, symptoms well-known in persons with malignant hyperthermia, an autosomally inherited disease of skeletal muscle. There is also discussed the manifestation and the symptoms of an acute rhabdomyolysis. The diagnosis was confirmed by chemical pathological laboratory findings, including respiratory and metabolic acidosis, myoglobinaemia accompanied by myoglobin diuresis, and elevated creatine phosphokinase (CPK values up to 2790 U/l). Electron microscopic examination of muscle tissue revealed signs of myolysis and mitochondrial reactions with pleoconic hyperplasia. No inhalation anaesthetics or skeletal muscle relaxants, such as succinyl choline, were used in this case. Therefore, malignant hyperthermia might have been induced by a combination of drugs which were not known to induce this abnormal muscular reaction. However, the muscle relaxant effect of dolpersin hydrochloride may have acted as a possible inducer of the attack.
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PMID:[Possible malignant hyperthermia as reaction to an overdose of myotonolytic, antidepressive and sedative drugs (author's transl)]. 611 87

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.
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PMID:[Rhabdomyolysis with acute renal failure and malignant neuroleptic syndrome]. 613 93

We report a patient with Duchenne muscular dystrophy who developed malignant hyperpyrexia during general anaesthesia. During anaesthesia bradycardia was followed by ventricular fibrillation, on which ventricular flutter supervened and a body temperature rise of 0.6 degrees C for 15 minutes, myoglobinuria and elevation of CPK level were observed. The caffeine sensitivity test of biopsied muscle fibers revealed an increase in sensitivity, although there was no sign of muscle rigidity during or after anaesthesia. Diagnosis of Duchenne muscular dystrophy was first established after the development of malignant hyperpyrexia in the present case as well as in previously reported cases. Determination of serum CPK is very important before general anaesthesia.
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PMID:Malignant hyperpyrexia and Duchenne muscular dystrophy: A case report. 621 75

Muscle biopsies were carried out in 41 patients with malignant hyperthermia, or supposed at-risk, as well as in some of their relatives. In 20 patients no changes were detected, and in 2 patients only acute rhabdomyolysis was seen (53.7%); in 19 patients (that is 46.3%) myopathic aspects were found in form of minimal changes, but also of syndrome-like tissue alterations (e.g. core disease, muscle dystrophy). These results confirm the existence of a morphologically detectable "subclinical myopathy" in about half of our patients. Four cases apt to demonstrate the variability, and the large morphological spectrum of these "myopathies" within the same family are described. In children, morphological changes are often missing. Carriers of such "subclinical myopathies" are occurring probably more frequently than supposed. Their identification in time is difficult, the only reliable methods being pharmacological fiber contraction-tests which can be performed in special laboratories only. A raw screening of patients-at-risk may be, however, achieved by exact anamnestic investigations and preoperative CPK-controls. Individuals suffering from paroxysmal myoglobinuria at any rate have to be considered patients at-risk.
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PMID:[Muscle biopsy studies in malignant hyperthermia]. 684 53


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