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)

We studied 27 Duchenne muscular dystrophy patients having spinal fusion for scoliosis. One patient died intraoperatively of cardiac arrest; all others have done well with no instances of malignant hyperthermia, postoperative ventilatory system dependence, pneumothorax, persisting infection, neurologic damage, nonunion, or pain. The anesthetic management included primarily intravenous general anesthetics with minimal myocardial depressant effects, avoiding succinylcholine and inhalation agents. Preoperative cardiac studies aided anesthetic management intra-operatively. There was an almost universal sinus tachycardia. Holter monitoring defined 4 of 16 with ventricular premature beats, 4 of 16 with atrial premature beats, and no ventricular tachycardia or atrial flutter or fibrillation. Echocardiogram demonstrated mitral prolapse in 2 of 22, frequent abnormal systolic performance with abnormal shortening fraction less than 28% in 7 of 16, and reduced rate-corrected velocity of fiber shortening in 9 of 15. Afterload was elevated in 7 of 15. The mean forced vital capacity (FVC) preoperatively was 45.3 +/- 15.9% with continuing diminution to 28.7 +/- 14.9% at 3.3 +/- 2.2 years after surgery. The main benefit of surgical stabilization is the relative ease and comfort of wheelchair seating compared with those nonoperated patients who develop progressive deformity. We have not seen lasting improvement or stabilization in FVC following surgery as decreasing function is related primarily to muscle weakness.
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PMID:Spinal fusion in Duchenne muscular dystrophy: a multidisciplinary approach. 158 53

Narcotic analgesics, although frequently used in adult patients, are at present relatively minor drugs in pediatric anesthesia. This review discusses indications, clinical applications, and side effects of opiates for pre-medication, induction and maintenance of anesthesia, and postoperative pain therapy in infants and children. Opiates do not represent the agents at first choice for preoperative anxiolysis or amnesia. With the exception of certain disease states (cardiac risk, elevated intracranial pressure, malignant hyperthermia) where intravenous anesthesia including opiates is clearly indicated, inhalational anesthetics are commonly preferred to narcotics. It has been shown, however, that opiate-supplemented general anesthesia can be used for pediatric surgery in an equally effective and safe manner. Finally, there is an essential need for more narcotic analgesics in the treatment of early postoperative pain, when antipyretic-antiphlogistic analgesics alone prove ineffective. It thus seems that in pediatric anesthesia today opiates are prescribed at the wrong time and withheld when they are most urgently needed.
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PMID:[Opiates in pediatric anesthesia]. 197 Nov 59

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

Tonsillectomy in adults and older children is typically accompanied by 7 to 14 days of pain. On the basis of clinical observations of patients treated perioperatively with dantrolene sodium for malignant hyperthermia, we hypothesized that pharyngeal muscle spasms are a major factor in tonsillectomy pain. We entered 113 patients, 11 years of age and older, into a double-blind, placebo-controlled study to evaluate the effectiveness of dantrolene sodium in reduction of tonsillectomy pain. Patients were randomly assigned either dantrolene (1.5 mg/kg per day) or placebo orally four times a day for 5 days postoperatively. On a standardized questionnaire, the patient recorded pain, diet, activity level, analgesics, and side effects, daily for 2 weeks. Also, alkaline phosphatase (alk phos) and serum aspartate aminotransferase (SGOT) levels were determined before the operation and 2 weeks after. Patients who received dantrolene had no significant differences in subjective pain, diet, or activity level scores from those of patients who received placebo. Dantrolene patients did, however, require significantly less analgesic use than placebo patients (p = 0.034, 0.015, and 0.005 for postoperative days 2, 3, and 4, respectively). There was no significant difference in side effects or changes in liver enzyme between the dantrolene and placebo groups. We conclude that dantrolene sodium, given in the dosage noted, is effective in reduction of analgesic requirements after tonsillectomy.
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PMID:Oral dantrolene sodium for tonsillectomy pain: a double-blind study. 312 47

Care of patients with osteoarthritis is compared for three different types of health service: fee-for-service, prepayment (Kaiser Health Plan), and an experimental service (Midpeninsula Health Service--MHS). While charging fees-for-service, MHS modifies medical practice in defined ways and seeks to involve patients through supervised self-care. Volunteer panels of patients provide the data at 6-month intervals through self-administered questionnaires. This report covers the first 18 months. Health status has not changed. Annualized utilization data, adjusted for age, income, education, pain, disability, disease duration, and prescription drug use, indicate that MHS members have fewer physician visits, fewer diagnostic tests, and lower antiinflammatory drug use than the other two services. MHS members express satisfaction equal to or greater than other participants. The results suggest that, in addition to modified financial incentives, there is another strategy that is relevant to achieving effective, efficient, and satisfying health care.
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PMID:Chronic disease and health system performance. Care of osteoarthritis across three health services. 398 7

Retrospective chart review (1978-1993) of 179 children less than age 18 (10.0 +/- 3.8 SD yrs) undergoing muscle biopsy for determination of susceptibility to malignant hyperthermia provided data. One hundred and forty-six patients received femoral and lateral femoral cutaneous nerve blocks as their primary anaesthetic. We examined age, weight, duration of surgery, time to discharge from hospital, choice and dosage of local anaesthetics, choice and dosage of sedation, postoperative pain medications, and complications. All children receiving this form of anaesthesia remained outpatients. Between 1978 and 1985 procaine (10 mg.kg-1) with hyaluronidase or 2-chloroprocaine (12 mg.kg-1) provided nerve blockade; after 1985, lignocaine (6.8 mg.kg-1), or a combination of lignocaine or mepivacaine and 2-chloroprocaine, were the preferred agents. More recently the combination of 2-chloroprocaine and bupivacaine has been popular. Three patients required admission to the recovery room postoperatively, due to heavy sedation. Forty-three children (29%) received pain medication during recovery. Femoral and lateral femoral cutaneous block anaesthesia with light to moderate sedation is well tolerated in children undergoing anterior thigh procedures.
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PMID:Femoral and lateral femoral cutaneous nerve block for muscle biopsies in children. 873 22

Children with developmental disabilities (CWDD) who undergo surgery have special needs. The nurse is required to make accurate assessments of the intellectual, psychosocial, and physical state of the child as well as the emotional functioning of the family. The nurse must be aware of the legal implications and his/her own attitudes regarding CWDD. Parents must be accepted as partners with the health care team and open communication should be established. Special concerns for the health care team are latex allergy and malignant hyperthermia. Nursing Care Plans specific to the child with pain, epilepsy, hydrocephalus, myopathy, Down syndrome, myelomeningocele, and cerebral palsy are provided. The material presented here should serve as a basis for nurses to deliver family-centered compassionate care to children and their families who are living with the burdens and enduring the hardships brought about by developmental disabilities.
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PMID:Nursing care of children with developmental disabilities having surgery. 754 85

Seventy-four responses were received from a questionnaire which had been mailed to 91 bone marrow transplantation institutes throughout Japan to assess the activity of bone marrow transplantation and complications in bone marrow donors. A total of 2329 bone marrow harvests, performed from 1688 adult donors and 641 child donors for allogeneic or syngeneic transplantation up to August 1992, were available for study. Analyses of the responses showed slight diversity regarding the marrow harvesting preparation and methods of the different bone marrow programs. The resulting perioperative complications were principally caused by anesthesia: 73 episodes of hypotension including one death 18 months later, seven of arrhythmia, one of respiratory arrest, three of mental confusion, one of asthma, one of malignant hyperthermia, one tooth injury and one broken aspiration needle. The postoperative complications were chiefly caused by marrow aspiration per se: 731 episodes of transient fever, 26 of long-lasting pain or discomfort, 10 episodes of liver dysfunction including two cases of non-A, non-B hepatitis, four cases of infection, one episode of hypotension, one of dysuria and one case of keloid formation. The study further revealed that the frequency of complications was lower in child donors than in adult donors.
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PMID:[Complications of marrow harvesting for transplantation]. 813 99

Sevoflurane may be an interesting substance for paediatric anaesthesia due to its combination of a very low blood-gas partition coefficient and non-pungency. This review discusses the status of sevoflurane in paediatric anaesthesia on the basis of studies published so far. The blood-gas partition coefficient of sevoflurane in children is 0.66, and hence markedly lower than those of isoflurane (1.25) and halothane (2.26) [15]. Induction of anaesthesia with sevoflurane/N2O is slightly shorter compared to halothane/N2O (Table 1) [4]. During induction of anaesthesia, sevoflurane/O2 is more often associated with excitement (35%) than sevoflurane/N2O (5%) and halothane/N2O (5%) [25]. Seizure-like movements in one case [1] and electrically generalised but clinically silent seizure activity in two cases [12] may raise the question of seizure-inducing effects of sevoflurane. However, up to now there is no clinical evidence of epileptogenic effects of sevoflurane. The MAC50 in neonates and infants 1-6 months of age is 3.3 vol% [14]; in infants 6-12 months and children 1-12 years of age it is 2.5 vol.% [14]. Sixty per cent N2O decreases the MAC50 of sevoflurane and desflurane by only 20%-25% [3, 14]. In contrast, 60% N2O decreases the MAC50 of halothane in children by 60% [16]. Thus, the MAC-reducing effect of N2O in children appears to be attenuated in the presence of less soluble inhalation anaesthetics. Sevoflurane has a similar low incidence of airway irritation as halothane and provides a smooth induction (Fig. 2) [4]. Haemodynamics during sevoflurane anaesthesia may be somewhat more stable compared to halothane. Serum fluoride levels increase rapidly when sevoflurane is administered, but decrease shortly after discontinuation [4]. Mean maximum levels reported are about 20 mumol/l and are of no concern for renal function. A study with mivacurium indicates more pronounced muscle relaxation by sevoflurane compared to halothane [9]. Sevoflurane may induce malignant hyperthermia. Emergence from sevoflurane anaesthesia is significantly more rapid than after halothane anaesthesia (Table 1); however, it is associated with more restlessness and agitation, probably due to the earlier perception of pain [4]. The incidence of postoperative nausea and vomiting after sevoflurane anaesthesia is comparable to that after halothane (Table 2). Sevoflurane may be a user-friendly alternative to halothane and is more preferred by children than halothane [32]. The status of sevoflurane in paediatric anaesthesia will depend on several factors: its own benefit/risk-ratio, a possible re-evaluation of the known risks of halothane and the financial limitations of the hospitals.
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PMID:[Sevoflurane in pediatric anesthesia]. 877 99

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


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