Studies on the Diagnosis and the Treatment of Malignant Hyperthermia

1)恶性高热是一种仅在麻醉过程中观察到的罕见综合征。通过期刊、问卷调查、人际交往等方式收集病例资料，对本病进行回顾性调查，并进行统计分析。恶性高热的死亡率逐年下降，部分原因是麻醉科医生对这种疾病有了更好的认识。2)对体征和症状的分析表明，一些因素对死亡率有影响。死亡病例的平均年龄和危象期间的最高体温均高于存活病例。以低血压、心动过缓、心律失常为体温升高前体征的死亡者发生率较高。然而，死亡病例中咬肌痉挛的发生率较低。丹曲林是一种治疗这种综合征的特效药物，它的应用降低了死亡率。没有Sta…死亡者与存活者在性别、使用琥珀胆碱氯化物、心动过速和危象期间高血压、肌红蛋白尿的发现以及除咬肌痉挛以外的全身肌肉僵硬方面的组织差异更大。3)由于这种综合征被认为是遗传性和遗传性的，人类白细胞抗原(人类白细胞抗原)的配型被寄予很高的期望来发现这种综合征的携带者。虽然已经观察到DR基因NJ28的发生率较高，但最近的分析表明，该抗原经常在该综合征患者中发现，但在没有体温过高的肌红蛋白尿患者中也经常发现。因此，这项研究应该扩大到包括恶性高热在内的更广泛的肌肉疾病。4)这种综合征的机制尚未很好地阐明。研究了膜的反常功能的可能性。用自旋标记法测定本综合征患者红细胞膜的流动性。在危机发生后的两周内，BE温度的流动性变化恢复正常。5)作为本综合征的诊断试验和触发机制，已证实本综合征患者肌浆网钙诱导钙释放速率(CICR)加快。以豚鼠皮肤纤维为材料，研究了多种麻醉剂和局麻药对CICR的增强作用。在其他药物中，临床浓度的氟烷被证实能强烈地增强CICR。然而，临床浓度的局麻药并不影响CECR机制。利用这种去皮纤维技术，发现与恶性高热有关的恶性综合征患者肌肉的CICR是完整的。较少

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