Studies on the Diagnosis and the Treatment of Malignant Hyperthermia

1)恶性高热是一种罕见的仅在麻醉期间观察到的综合征。作为对该综合征的回顾性调查，我们对通过期刊、问卷调查和个人交流收集的病例进行了统计分析。恶性高热的死亡率逐年下降，部分原因是麻醉医师对该病的认识不断提高。2）通过对临床症状和体征的分析，揭示了恶性高热死亡的相关因素。死亡病例的平均年龄高于存活病例，危象期间的最高体温高于存活病例。在死亡病例中观察到体温升高前的低血压、心动过缓和心律失常的发生率在统计学上较高。但死亡病例中咬肌痉挛的发生率较低。丹曲林是一种对该综合征特别有效的药物，其给药可降低死亡率。没有舞台 ...更多信息 死亡病例和存活病例在性别、使用氯化琥珀胆碱、危象时心动过速和高血压、肌红蛋白尿和除咬肌痉挛外的全身肌肉强直方面的统计学差异。3）由于该综合征被认为是遗传性的，因此高度期望HLA（人类白细胞抗原）分型来检测该综合征的携带者。虽然已经观察到DR位点中NJ28的较高发生率，但最近的分析表明，该抗原经常在患有该综合征的患者中发现，而且在没有高热的肌红蛋白尿患者中也发现。因此，这项研究应该扩展到包括恶性高血压在内的更广泛的肌肉疾病。4）这种综合征的机制尚未得到很好的阐明。研究了膜功能异常的可能性。用自旋标记法测定了本病患者红细胞膜流动性。危象发生后两周内体温变化基本恢复正常。为了证实这种变化是该综合征的一般特征，应分析更多的该综合征样本。5）作为该综合征的诊断试验和触发机制，已证实该综合征患者骨骼肌肌浆网钙诱导钙释放（CICR）速率加快。使用豚鼠的皮肤纤维，研究了各种挥发性麻醉剂和局部麻醉剂对CICR的增强作用。临床浓度的氟烷被证实在其他药物中强烈增强CICR。然而，临床浓度的局麻药不影响CECR机制。通过使用这种皮肤纤维技术，发现与恶性高热有关的恶性综合征病例的肌肉CICR是完整的。少

1) Malignant hyperthermia is a kind of rare syndrome only observed during anesthesia. As a retrospective survey of this syndrome, a statistical analysis was made on the basis of cases which we collected through journals, questionnaire surveys, and personal communications. The mortality rate of malignant hyperthermia has declined year by year partly because of the better understanding of this desease by anesthesiologist.2) Analysis of the signs and symptoms has revealed that some factors contribute to mortality. The mean age was higher and the maximum body temperature during the crisis was higher in the deceased cases than in the surviving cases. Hypotension, Bradycardia and arrhtyhmia as signs before elevation of body temperature were observed in deceased cases in a statistically higher rate. However, the incidence of masseter spasm was lower in deceased cases. Administration of dantrolene which is a specifically effective agent for this syndrome reduced the mortality. There was no sta … More tistical difference between the deceased and survived cases by sex, usage of succinylcholine chloride, tachycardia and hypertension during the crisis, finding of myoglobinuria, and general muscle rigidity other than masseter spasm.3) Since this syndrome is thought to be hereditary and genetic, typing of HLA( Human Leukocyte Antigen ) is highly expected to detect carriers of this syndrome. Although a higher incidence of NJ28 in DR locus has been observed, recent analysis has shown that this antigen is frequently found in patients with this syndrome but also in patient myoglobinuria without hyperthermia. Therefore, this study should be extended to cover a broader range of muscle diseases including malignant hyperthermia.4) the mechanism of this syndrome has not yet been well elucidated. The possibility of abnormal function of the memberane has been studied. The fluidity of erythrocyte membrane taken from patients of this syndrome was measured by using the spin-labeled method. Shift of fluidity be temperature was restored to normal in two weeks after the crisis. To confirm this change as a general character of this syndrome, more samples of this syndrome should be analysis.5) As a diagnosis test and triggering mechanism of this syndrome, acceleration of the rate of calcium-induced calcium release (CICR) from the sarcoplasmic reticulum of the skeletal muscle has been confirmed in patients with this syndrome. Potentiation of CICR was studied with a variety of volatile anesthetic agents and local anesthetic agents using the skinned fiber of the guinea pig. Halothane in clinical concentration was confirmed to potentiate strongly CICR among the other agents. However, local anesthetics in clinical concentration did not affect CECR mechanism. By using this skinned fiber technique, CICR of muscle of cases with malignant syndrome which is said to be related to malignant hyperthermia was found to be intact. Less