Glauber V, Ben Abraham R, Zweig A, Perel A
Department of Anesthesiology and Intensive Care, Chaim Sheba Medical Center, Tel-Hashomer, Tel Aviv, Israel.
Isr J Med Sci. 1997 Oct;33(10):643-7.
Malignant hyperthermia (MH), a rare pharmacogenetic trait, can be lethal when susceptible individuals are exposed to triggering agents during general anesthesia. We present our experience with the caffeine-halothane in vitro contracture test (IVCT) for the diagnosis of malignant hyperthermia susceptibility. Out of 75 patients that were referred for consultation to the MH diagnostic center over a period of 7 years, we performed muscle biopsies and IVCT in 21 patients. A total of 6 patients were found to be MH-positive. Appropriate recommendations for future anesthetic management, and additionally, for testing the immediate family were made following a positive diagnosis. Improved familiarity with the syndrome of MH, and performance of IVCT when family or clinical history suggest malignant hyperthermia susceptibility, are imperative measures to prevent the potential fatality associated with this syndrome.
恶性高热(MH)是一种罕见的药物遗传性状,当易感个体在全身麻醉期间接触触发剂时可能致命。我们介绍了我们使用咖啡因-氟烷体外挛缩试验(IVCT)诊断恶性高热易感性的经验。在7年期间转诊至MH诊断中心咨询的75例患者中,我们对21例患者进行了肌肉活检和IVCT。共发现6例患者MH检测呈阳性。确诊后,针对未来的麻醉管理以及对直系亲属进行检测给出了适当建议。提高对MH综合征的认识,并在家族史或临床病史提示恶性高热易感性时进行IVCT,是预防与该综合征相关潜在死亡的必要措施。