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)

A case of pregnancy complicated by malignant hyperthermia susceptibility is reported. Serum CPK and electrolyte concentrations were measured during pregnancy and labour. Labour and delivery were managed successfully under epidural analgesia using plain bupivacaine 0.5%.
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PMID:Malignant hyperthermia susceptibility. Management during pregnancy and labour. 42 40

Malignant hyperthermia syndrome developed during epidural analgesia in 25-year-old female to be operated on for haemorrhoidal varices. After premedication with diazepam and atropine epidural analgesia was started with lidocaine 300 mg and bupivacaine 50 mg. Signs and symptoms of malignant hyperthermia syndrome appeared 30 min. later, with muscle rigidity, hyperpyrexia 41.5degrees C, and loss of consciousness. Treatment alleviating the syndrome was applied as indicated in this complication. Full recovery was obtained.
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PMID:A case of malignant hyperthermia during epidural analgesia. 97 Jun 24

The purpose of this retrospective study was to estimate the frequency and severity of anaesthetic complications in patients with Duchenne's muscular dystrophy (DMD). Forty-four boys with DMD were exposed to anaesthesia and surgery 84 times during a period of 22 years (1965-86). The procedures took place at 15 different hospitals. Retrospective examination of the case records showed: 19 cases with local analgesia without any complications, and 18 of 65 general anaesthetics with minor or more serious complications. In ten cases an increase in body temperature above 37.5 degrees C was seen, five had abdominal pain and dark-coloured urine after surgery, and three had a critical perioperative course with a resemblance to malignant hyperthermia. The complications were almost exclusively related to the use of succinylcholine. The use of succinylcholine was dispersed through all ages. Three out of the eight patients with severe complications occurred 1.5, 2.5 and 4 years before the neuromuscular disease was diagnosed. Thus an unusual course of anaesthesia in male children calls for further investigation. Although it has been stated before that succinylcholine is contraindicated in patients with Duchenne's muscular dystrophy, the drug continues to be used.
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PMID:Complications during anaesthesia in patients with Duchenne's muscular dystrophy (a retrospective study) 231 Nov 64

An overall management plan for malignant hyperthermia susceptible (MHS) parturients is presented based on the experience of managing 14 of these patients. A summary of the pertinent features of their deliveries and care plus a case report of one of these parturients is described. Discussion centres around the problems of diagnosis of malignant hyperthermia susceptibility in pregnancy, the differential diagnosis and management of fever and tachycardia in a MHS parturient during labour and the use of dantrolene prophylaxis. Management of the MHS parturient in labour includes temperature and heart-rate monitoring, provision for cooling, and ready availability of a vapour-free anaesthetic machine. A large-bore intravenous infusion for hydration and for potential therapy of a MH crisis is essential. Epidural analgesia, using 2-chloroprocaine, is recommended. Emergency and elective Caesarean section anaesthesia are discussed. The importance of being prepared for a potential crisis is stressed with particular emphasis on early diagnosis by monitoring of temperature at two sites, of heart rate and rhythm with a continuous ECG and of end-tidal carbon dioxide, should a general anaesthetic be required. Recommendations are made for appropriate anaesthetic agents for both regional and general anaesthesia. Treatment of a MH crisis is outlined, with emphasis on availability of appropriate resuscitative drugs.
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PMID:The anaesthetic management of the malignant hyperthermia susceptible parturient. 371 39

A 3-in-1 lumbar plexus block with the aid of a nerve stimulator was performed in 32 patients and a psoas compartment block was performed in five patients for muscle biopsy of the upper leg for diagnosis of malignant hyperthermia (MH) susceptibility. Twenty-two patients were found to be MH susceptible by the in vitro contracture test. Twenty patients received 40 ml prilocaine 1.5% with epinephrine 1:200,000 and two received 40 ml bupivacaine 0.5% with epinephrine 1:200,000 without any untoward reaction. The 3-in-1 block provides a high success rate and excellent analgesia for muscle biopsy of the upper leg. Amide local anaesthetics are safe in MH-susceptible patients.
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PMID:3-in-1 lumbar plexus block for muscle biopsy in malignant hyperthermia patients. Amide local anaesthetics may be used safely. 381 2

We report the negative response of MHS swine to i.v. infusion of lignocaine and bupivacaine yielding plasma concentrations which equal or exceed those reported in humans during extradural analgesia. It is concluded that local anaesthetic techniques using the amide-linked local anaesthetics administered in conventional dosage are safe to use in patients known to be genetically susceptible to malignant hyperthermia.
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PMID:Response of mhs swine to i.v. infusion of lignocaine and bupivacaine. 737 38

Induction, emergence and recovery characteristics were compared during sevoflurane or halothane anaesthetic in a large (428) multicentre, international study of children undergoing elective inpatient surgical procedures. Two hundred and fourteen children in each group underwent inhalation induction with nitrous oxide/oxygen and sevoflurane or halothane. Incremental doses of either study drug were added until loss of eyelash reflex was achieved. Steady state concentrations of anaesthesia were maintained until the end of surgery when anaesthetic agents were terminated simultaneously. Time variables were recorded for induction, emergence and the first need for analgesia in the recovery room. In addition, in 86 of the children in both groups, venous blood samples were drawn for plasma fluoride levels during and after surgery. There was a trend toward smoother induction (induction of anaesthesia without coughing, breath holding, excitement laryngospasm, bronchospasm, increased secretion, and vomiting) in the sevoflurane group with faster induction (2.1 min vs 2.9 min, P = 0.037) and rapid emergence times (10.3 min vs 13.9 min, P = 0.003). Among the children given sevoflurane, 2% developed bradycardia compared with 11% in the halothane group. Postoperatively, 46% of the children in the halothane group developed nausea and or vomiting versus 31% in the sevoflurane group (P = 0.002). Two children in the halothane group developed cardiac dysrhythmia and were dropped from the study. In addition, a child in the halothane group developed malignant hyperthermia, received dantrolene, and had an uneventful recovery. Mean maximum inorganic fluoride concentration was 18.3 microM.l-1. The fluoride concentrations peaked within one h of termination of sevoflurane anaesthetic and returned rapidly to baseline within 48 h. This study suggests that sevoflurane may be the drug of choice for the anaesthetic management of children.
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PMID:A comparison of sevoflurance to halothane in paediatric surgical patients: results of a multicentre international study. 882 44

Case report on a 2.5-year old girl suffering from arthrogryposis multiplex congenita (AMC) who was admitted for an extensive orthopaedic operation of equinovarus. The patient showed typical AMC-related problems such as skin and subcutaneous tissue abnormalities, lack of veins, contractural deformities of all four limbs and microgenia. Problems associated with anaesthesia in this patient were difficult intubation and venipuncture and a potential risk of developing malignant hyperthermia when using volatile anaesthetics. For preoperative blood chemistry sampling and intravenous induction of general anaesthesia, the patient received a central venous catheter under local and N2O/O2 anaesthesia on the day before surgery. Following intravenous induction of trigger-free anaesthesia using fentanyl, thiopental and vecuronium, the child was intubated and ventilated with 30% O2 in N2O the next day. A caudal catheter was inserted for intraoperative reduction of anaesthetics and postoperative pain relief. Intraoperatively, caudal anaesthesia was performed with 2 ml of 2% mepivacaine every 90 min. No inadvertent reactions were seen during a 7 h operation. In the recovery room, the patient received 4 ml of plain 0.25% bupivacaine per 4 h via the caudal catheter and had excellent analgesia during 24 postoperative hours. The following course was uneventful and the child was discharged from hospital two weeks later. AMC-related problems concerning the management of anaesthesia are discussed.
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PMID:[Arthrogryposis multiplex congenita: special anesthesiological aspects]. 886 36

The mitochondrial myopathies are a rare group of conditions affecting the respiratory chain and oxidative phosphorylation. The anesthetic management of a 6-year-old girl with complex I respiratory chain deficiency requiring surgery for a fractured hip is presented and discussed. Potential problems were masseter spasm, tendency to develop lactate acidosis, and malignant hyperthermia susceptibility. These problems were avoided by the use of a laryngeal mask airway, allowing the patient to spontaneously ventilate; caudal analgesia; and maintenance of anesthesia with a proprofol infusion.
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PMID:Anesthesia for a child with complex I respiratory chain enzyme deficiency. 979 22

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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