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

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.
 ...
PMID:[A case report of a 63-year-old patient with malignant hyperthermia]. 161 62

Malignant hyperthermia (MH) may occur, when a genetically predisposed individual or pig (MHS) is exposed to triggering agents. The increase in free, ionized sarcoplasmic calcium inducing the vicious circle of MH is believed to result from calcium-induced release with volatile anaesthetics, and from depolarization-induced calcium release with succinylcholine (SCH). The administration of SCH to susceptible humans or pigs frequently produces an increase in masticatory muscle tone. This hitherto ill-defined phenomenon is referred to as "masseter spasm" (MS). We have attempted to elucidate the pathophysiology of MS in a porcine model. METHODS. After the protocol had been approved by the state authorities, 6 MHS pigs were investigated. The pigs were mixed breeds (German Landrace and Dutch Pietrain) and were 9 +/- 1 weeks old with an average body weight of 25.5 kg. Premedication consisted of intramuscular injection of azaperone, 7.5 mg.kg-1. Anaesthesia was induced with piritramide, 1.2 mg.kg-1, administered via a cannulated ear vein. Subsequent to laryngoscopic endotracheal intubation, neuromuscular blockade was achieved with 4 mg pancuronium. Ventilation was set at 12 breaths per minute and adjusted to maintain an end-tidal CO2 concentration of 4.7% by adapting the tidal volume (PhysioFlex). Anaesthesia was maintained with piritramide, 2.25 mg.kg-1.h-1, pancuronium, 0.4 mg.kg-1.h-1, and N2O (60% in O2). Instrumentation included an arterial line, a central venous line, and a fiberoptic pulmonary artery catheter (Oximetrix). Masticatory muscle tone (MMT) was assessed with an intermolar balloon, connected to a pressure transducer and calibrated to zero prior to SCH administration. As a reference variable for effects produced by SCH, intraocular pressure (IOP) was measured manometrically in the anterior chamber. After stabilization of haemodynamic variables, the neuromuscular blockade was allowed to wear off. After recovery of the evoked masseter electromyogram, a paralyzing dose of pancuronium was administered (0.5 mg.kg-1). When paralysis was complete, SCH was administered (1.5 mg.kg-1), followed a few minutes later by dantrolene infusion (5 mg.kg-1 over 10 min). RESULTS. The administration of SCH was followed by clinically unequivocal MH episodes in all pigs, indicated by an increase in oxygen uptake (VO2; PhysioFlex; Fig. 1) and end-tidal CO2 concentration and a decrease in oxygen saturation of mixed venous blood (svO2; Fig. 2). Despite complete neuromuscular blockade (monitored with EMG), SCH produced an increase in MMT in all pigs which was reversed by dantrolene (Fig. 3). The time course of MMT paralleled that of IOP, suggesting a similar underlying mechanism. DISCUSSION. Succinylcholine is a trigger of MH in susceptible individuals; onset of the syndrome may be associated with "masseter spasm". SCH increases extraocular muscle tone, probably by means of stimulating multiply innervated fibers; the resulting IOP increase is not prevented by competitive neuromuscular blockade. The existence of multiple innervated fibers has also been shown in muscle spindles in the deep layers of the masseter, with their stimulation resulting in elevation of the jaw. We speculate that the increases in MMT and IOP observed in this study reflect the same process, i.e. a motor response, initiated by SCH-induced stimulation of the intramyocellular contractile system of multiply innervated muscle fibers, that is independent of neuromuscular transmission. Triggering of MH with SCH despite complete neuromuscular blockage suggests a mechanism other than depolarization-induced calcium increase. And, for the semantics, according to neurological terminology MS should be referred to as contracture not as spasm.
 ...
PMID:[The effect of muscle relaxants on masseter tone. An experimental study in an MH-susceptible swine model]. 161 14

The Authors report a case of biopsy-proven malignant hyperthermia in a pediatric patient who underwent general anesthesia with halothane and succinylcholine for foot surgery, in whom the presenting symptom was isolated postoperative myoglobinuria. The above syndrome, in the absence of a positive family history, may present itself with a set of minor and atypical symptoms, thus being often underestimate. The need for further investigating all the cases of postoperative myoglobinuria is stressed by the Authors, which also consider muscular biopsy for inclusion among routine investigations. In the case reported here, six months later, a new surgical operation was done safely with spinal anesthesia using hyperbaric bupivacaine.
 ...
PMID:[Isolated postoperative myoglobinuria as the only sign of malignant hyperthermia. Value of spinal anesthesia with bupivacaine in a posterior surgical intervention]. 162 Apr 19

In this paper the Authors describe the anesthetic techniques used for abdominal surgery in a 19 year-old woman with "central-core disease". This uncommon congenital neuromuscular disorder is frequently related to malignant hyperthermia syndrome. Total intravenous anesthesia with the association propofol-fentanyl was well tolerated by the patient. There was no evidence of malignant hyperthermia during the procedure and cardiovascular stability was excellent.
 ...
PMID:[The use of propofol in a female patient predisposed to malignant hyperthermia (central core disease)]. 162 Apr 20

Two 2-year-old males underwent muscle biopsy that established the histopathologic diagnosis of Becker dystrophy in one, and Duchenne dystrophy in the other. Concomitant contracture testing with caffeine or halothane was normal for malignant hyperthermia (MH). The results suggest that acute hypermetabolism or acute rhabdomyolysis during anesthesia, in patients with these disorders, is related to the X-linked myopathy and its associated muscle deterioration, rather than to the autosomal dominant MH.
 ...
PMID:Absence of malignant hyperthermia contractures in Becker-Duchenne dystrophy at age 2. 173 62

Anaesthetic regimens for patients with the poorly understood neuroleptic malignant syndrome (NMS) are not well outlined. In this case report, NMS and its questionable relation to malignant hyperpyrexia are outlined. Satisfactory use of total intravenous anaesthesia, based on propofol, in an NMS-susceptible patient is presented.
 ...
PMID:Propofol safely used in a neuroleptic malignant syndrome patient. 174 6

An acute episode of a malignant hyperthermia-like syndrome is described which occurred after suxamethonium and isoflurane anaesthesia in a 41-year-old healthy male patient undergoing a minor elective hand operation. Dantrolene therapy rapidly reversed the life-threatening signs. Laboratory results appeared to confirm the suspicion of malignant hyperthermia. However, the in vitro contracture test, which was carried out according to the standards of the European Malignant Hyperthermia Group, was equivocal.
Anaesthesia 1991 Nov
PMID:A case of malignant hyperthermia? 161 11

Malignant hyperthermia susceptibility (MHS) is a clinically heterogeneous pharmacogenetic disorder characterized by accelerated metabolism, hyperthermia, and frequently muscle rigidity. MHS is elicited by all commonly used potent inhalation anesthetics and depolarizing neuromuscular blockers and remains an important cause of death due to anesthesia. Recent linkage studies suggest a single genetic locus for this disorder on chromosome 19q13.1. The results of our linkage analyses exclude several loci on 19q13.1 as a site for the gene(s) that produces the MHS phenotype in three unrelated families and clearly establish genetic heterogeneity in this disorder. These results are consistent with the hypothesis that the genetic defect that alters thermoregulation may vary in MHS and that clinical variability in the expression of MHS may be explained by genetic heterogeneity.
 ...
PMID:Evidence for genetic heterogeneity in malignant hyperthermia susceptibility. 177 61

A four year old boy suffered from a prolonged cardiac arrest during anesthesia. Laboratory examination subsequently demonstrated extensive rhabdomyolysis. The possibility of an underlying muscle disease in this family was investigated by muscle biopsy, histological examination and in-vitro contracture testing. The latter test result was consistent with malignant hyperthermia susceptibility. This case history illustrates the differential diagnosis of anesthesia induced rhabdomyolysis.
 ...
PMID:Differential diagnosis of anesthesia induced rhabdomyolysis. A case report. 178 Dec 67

We report on the fulminant crisis of malignant hyperthermia occurring in a 30-year-old female during kidney transplantation. In the past, she had been anaesthetised repeatedly without complications. Anaesthesia was induced with thiopental and vecuronium and continued with isoflurane/N2O/O2. After an initially normal course of anaesthesia, the patient developed symptoms of a fulminant malignant hyperthermia (MH) including excessive increase in end expiratory CO2, hyperkalaemia, tachycardia and hyperpyrexia. The patient was saved by the timely administration of dantrolene. A surgical revision required the next day because of bleeding was done under dantrolene cover and took an uncomplicated course. The patient was extubated 7.5 hours after the second intervention and transferred to a normal ward after 4 days. A subsequently performed in vitro contracture test clearly revealed susceptibility to malignant hyperthermia.
 ...
PMID:[Fulminant malignant hyperthermia during the 6th general anesthesia using volatile anesthetics]. 178 10


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>