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)

Propofol is an intravenous (IV) drug recently introduced into the United States for induction and maintenance of anesthesia. In spite of extensive laboratory evaluation, it is not possible to predict all the potential side effects that might be associated with a new drug. Because malignant hyperthermia (MH) remains a serious and potentially life-threatening complication of anesthesia, all new anesthetic drugs should be considered potential triggering drugs until proven otherwise. We report the use of IV propofol for the induction and maintenance of general anesthesia in an MH patient and review the literature on this subject.
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PMID:Propofol in patients susceptible to malignant hyperthermia: a case report and review of the literature. 135 35

1. Malignant hyperpyrexia (MH) is an inherited muscle abnormality that presents clinically as a syndrome of life-threatening complications during general anaesthesia. 2. Propofol is a new sedative hypnotic used for the induction and maintenance of anaesthesia. 3. Propofol did not induce MH in five susceptible pigs. Propofol did not induce contracture in isolated MH susceptible muscle but did modify halothane, caffeine and KCl contractures.
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PMID:Propofol anaesthesia in malignant hyperpyrexia susceptible swine. 152 53

In this study we investigated in vitro and in vivo effects of propofol in malignant hyperthermia susceptible (MHS) patients in order to assess the safety of propofol infusion as a non-triggering anaesthetic technique for diagnostic and therapeutic procedures. In vitro, human MHS muscle samples were exposed to propofol and changes in (a) baseline tension and (b) contracture tension on exposure to halothane and caffeine were measured. In vivo, (a) anaesthesia was induced in ten muscle biopsy positive MHS patients with propofol 2.5 mg/kg and (b) anaesthesia was produced in five muscle biopsy positive MHS patients with infusions of propofol up to 10 mg/kg/hr. In vitro, human MHS muscle did not develop contractures with propofol alone. Propofol had no significant effect on contracture development in response to halothane and caffeine. In vivo, no evidence of an MH response was detected following induction or maintenance of anaesthesia with propofol. Our results and literature review are in agreement that propofol is a 'safe' induction and maintenance agent in MHS patients. Propofol can be used for muscle biopsy anaesthesia because it does not alter the sensitivity of diagnostic muscle biopsy testing.
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PMID:Propofol is a 'safe' anaesthetic agent in malignant hyperthermia susceptible patients. 159 50

Propofol may be a useful anesthetic in the management of malignant hyperthermia patients. It appears not to trigger malignant hyperthermia while providing stress-free conditions. This case report, along with a small number of others, documents the safe use of propofol for this patient population.
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PMID:Propofol for anesthesia in a patient susceptible to malignant hyperthermia. 183 46

The anesthetic technique chosen for a malignant hyperthermia (MH) susceptible patient should include drugs that do not trigger MH, while providing stress-free conditions. This case report describes a MH susceptible patient who was successfully induced and maintained with propofol for third molar extractions while under general anesthesia. Based on this case report, and the other relative few in the literature, it appears unlikely that propofol will trigger an episode of MH. Propofol provides the anesthetist with an alternative for inducing MH susceptible patients, but continued experience is necessary to document its safety and efficacy in these patients.
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PMID:Propofol anesthesia in the malignant hyperthermia susceptible patient. 202 58

A review of the pharmacology of propofol, a new IV anesthetic agent, is presented. Solubilized in a soybean emulsion, propofol is one of a series of sterically hindered phenols that exhibit anesthetic activity. Induction of anesthesia with propofol may be associated with pain on injection, apnea, and a reduction in arterial blood pressure (BP) and cardiac output. Caution should be ascribed to its use in patients with coronary artery disease, where these effects may have the potential for producing myocardial ischemia. The hemodynamic responses to laryngoscopy and intubation are attenuated. The pharmacokinetic profile suggests suitability as an infusion for either maintenance of anesthesia or sedation. Use of propofol as an infusion during surgery may result in a further reduction in cardiac output, particularly with the concomitant administration of adjuvant increments of fentanyl. The ventilatory response to CO2 is depressed during such an infusion. The high clearance of propofol suggests that even after a prolonged infusion, recovery should be rapid. This finding has been confirmed in a series of studies establishing propofol as an ideal agent for use in a total IV anesthetic technique. Both the quality and speed of recovery, together with the absence of emetic sequelae, support the use of propofol in an outpatient setting. Propofol appears to have no long-term effect on adrenocortical function and appears safe for use in patients with acute intermittent porphyria and susceptibility to malignant hyperpyrexia.
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PMID:The pharmacology of propofol. 269 45

Neuroleptic malignant syndrome (NMS) is the most serious side effect produced by the administration of antipsychotic drugs. NMS shares many clinical similarities with malignant hyperthermia (MH), but the etiology of NMS and the relation between NMS and MH remain unknown. Anesthetic regimens for patients with NMS are not well established. We gave repeated anesthesia to a patient with a history of NMS undergoing electroconvulsive therapy for the treatment of depression. Propofol and vecuronium were used in twelve consecutive ECT sessions without complications. In this case report, we describe the safe and satisfactory repeated use of propofol in a patient with a history of NMS, and outline NMS and its questionable relation to MH.
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PMID:Repeated propofol anesthesia for a patient with a history of neuroleptic malignant syndrome. 1046 42

We gave propofol anesthesia to a patient with limb-girdle type of progressive muscular dystrophy. A 42 year-old male was to have skin graft for third degree burn. His respiratory function test showed %VC of 73.6% and %FEV1.0 of 107.6%. Arterial blood gas data were within normal ranges. He was anesthetized with propofol, fentanyl, vecuronium and nitrous oxide. During position change, Wenckebach type of second degree AV block occurred. AV block returned to sinus rhythm easily by injection of ephedrine hydrochloride and atropine sulfate, and reduction of propofol infusion rate. There were no perioperative respiratory complications and no clinical manifestations of malignant hyperthermia. Propofol anesthesia is suitable for limb-girdle type of progressive muscular dystrophy, because of very little possibility of triggering malignant hyperthermia, rapid awaking, minimal residual effects of the respiratory system, and easiness in controlling anesthetic depth.
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PMID:[Propofol anesthesia for a patient with progressive muscular dystrophy]. 1188 93

A 12-year-old boy with Duchenne muscular dystrophy underwent posterior spinal fusion for progressive scoliosis. Preoperative evaluation was focused on respiratory function as well as cardiac function, which revealed markedly reduced respiratory reserve (FVC 0.77 l, %FVC 25.9%, FEV1.0 0.48 l, %FEV1.0 62%) and well-preserved biventricular function. A possible association between malignant hyperthermia and Duchenne muscular dystrophy has been documented. Thus anesthesia was administered without triggering agents. Propofol and fentanyl were used for induction and maintenance of anesthesia, and the patient was ventilated with O2-nitrous oxide mixture. The anesthesia machine, prepared by using a disposable circuit and fresh CO2-absorbent and disconnecting the vaporizers, was flushed with O2 at a rate of 10 l.min-1 for 20 minutes before use. A small dose of vecuronium was administered while monitoring the train-of-four ratio. The bispectral index (BIS) was utilized to optimize the depth of anesthesia so that the wake-up test could be performed promptly on surgeon's request while avoiding the intraoperative awareness. The BIS was helpful in continuously assessing the wakening process. BIS increased from 40's to 80's in 15 minutes after discontinuation of propofol and nitrous oxide during the test. The patient was kept under close observation postoperatively without any sign of malignant hyperthermia.
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PMID:[Application of bispectral index (BIS) monitoring to anesthetic management of posterior spinal fusion in a patient with Duchenne muscular dystrophy]. 1216 84

Since venous cannulation in children has become easier and extensive experience has been gained with total intravenous anaesthesia (TIVA) in adults, the interest in TIVA for children has recently increased. An intensified sensitivity of the operating room atmosphere to contamination with volatile anaesthetic agents is another important reason to choose intravenous techniques for paediatric anaesthesia. One of the most interesting agents for TIVA in paediatric anaesthesia is propofol. The pharmacokinetic and pharmacodynamic data for modern intravenous drugs is poor. Because the interpatient variability is relatively large, pharmacokinetic data can only provide guidelines for the dosage of propofol. Propofol has a rapid and smooth onset of action and is as easy to titrate in children as in adults. Propofol can be excellently controlled. Severe haemodynamic side-effects are missing in healthy children and plasma is cleared rapidly of propofol by redistribution and metabolism. There is no evidence of significant accumulation, not even after prolonged infusion times. Because propofol has no analgetic properties it must be combined with analgetics or a regional block for all painful procedures. The combination with the ultra-short acting remifentanil is a major advantage, but requires effective analgetic concepts for painful procedures. In comparison the combination of propofol with long acting opioids abolishes some of the favourable properties of propofol. Further studies of the kinetics and dynamics of propofol and other intravenous agents are needed in paediatrics which should focus on age, maturity and severity of illness. The whole importance of the propofol-infusion syndrome has to be cleared up urgently. TIVA has an important significance in paediatric anaesthesia for diagnostic and therapeutic procedures, especially where these have to be repeated. In day-case anaesthesia TIVA has advantages for all short procedures and for ENT and ophthalmic surgery: even after prolonged infusion children have an short recovery time. There is no evidence of agitation or other behavioural disorders after TIVA with propofol in paediatric anaesthesia. Propofol has anti-emetic properties. TIVA with propofol can be combined with regional anaesthesia advantageously to provide long-lasting analgesia after surgery. TIVA with propofol has been used successfully for sedation of spontaneously breathing children for MRI and CT and other procedures with open airways like bronchoscopy or endoscopy. Propofol facilitates endotracheal intubation without the use of muscle relaxants. Of course, in malignant hyperthermia TIVA will continue to be the technique of choice. Nothing is known about awareness under TIVA in paediatric patients. TIVA must be considered by comparison with the volatile agents. The use of ultra-short acting agents may cause problems such as awareness, vagal response, involuntary movements and in some cases slow recovery after prolonged infusion of propofol. But it is not known exactly how often this happens during paediatric anaesthesia. With TIVA an effective postoperative analgesia must be provided. Newer administration techniques such as the target-controlled infusions or closed-loop control systems are under development and will help to minimise the potential risk of overdosage with TIVA in paediatrics. At the present TIVA is an interesting and practicable alternative to volatile anaesthesia for pre-school and school children. TIVA with propofol in infants younger than 1 year old requires extensive experience with TIVA in older children and with the handling of this special age group and should be undertaken with maximum precautionary measures.
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PMID:[Total intravenous anesthesia. On the way to standard practice in pediatrics]. 1450 2


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