Mauritz W, Hackl W, Sporn P, Graf M, Sluga E, Steinbereithner K
Ludwig Boltzmann-Institut für experimentelle Anaesthesiologie, Universität Wien.
Anaesthesist. 1988 Jul;37(7):425-31.
During the last 4 years different diagnostic procedures for the detection of malignant hyperthermia (MH) susceptibility have been used at the authors' clinical unit; this study was designed to compare the results of these tests. PATIENTS AND METHODS. Since March 1983, 158 patients have been referred for the following reasons: group A: probands (n = 17) who had had symptoms of MH during anesthesia; group B: patients of probands (n = 48) if the latter were not tested because of age (n = 24) or death (n = 2); group C: relatives from MH families (n = 86); group D: patients (n = 5) who developed fever during stress and/or physical activity (n = 3), had myotonia (n = 1), or developed rhabdomyolysis during intensive care (n = 1); group E: controls (n = 2). Two static halothane and two static caffeine tests according to the European protocol were performed in all patients (n = 158). Histological examinations of skeletal muscle (fixed in glutaraldehyde, stained with hematoxylin-eosin, Gieson, and toluidine blue) were done in the first 100 patients; all specimens were scored by the same investigator (E.S.). Score 0: normal; 1: increased number of sarcolemma cores; 2: 1+cores forming groups; 3: 1+2+fiber degeneration; 4: specific changes-myopathies. Plasma levels of creatine kinase (CK) were determined in the first 50 patients. Complete neurological examinations, including electromyography (EMG), were done in ten patients who had increased CK levels as well as histological scores of 3 or 4 (Table 1).(ABSTRACT TRUNCATED AT 250 WORDS)
在过去4年里,作者所在临床科室采用了不同的诊断程序来检测恶性高热(MH)易感性;本研究旨在比较这些检测结果。患者与方法。自1983年3月以来,158例患者因以下原因前来就诊:A组:麻醉期间出现MH症状的先证者(n = 17);B组:先证者的患者(n = 48),其中因年龄(n = 24)或死亡(n = 2)未对先证者进行检测;C组:MH家族的亲属(n = 86);D组:在应激和/或体力活动期间发热的患者(n = 3)、患有肌强直的患者(n = 1)或在重症监护期间发生横纹肌溶解的患者(n = 1);E组:对照组(n = 2)。对所有患者(n = 158)均按照欧洲方案进行了两次静态氟烷试验和两次静态咖啡因试验。对前100例患者的骨骼肌进行了组织学检查(用戊二醛固定,苏木精 - 伊红、吉氏、甲苯胺蓝染色);所有标本均由同一位研究者(E.S.)评分。评分0:正常;1:肌膜核数量增加;2:1个以上核形成群组;3:1 + 2 + 纤维变性;4:特异性改变 - 肌病。对前50例患者测定了血浆肌酸激酶(CK)水平。对10例CK水平升高且组织学评分为3或4的患者进行了包括肌电图(EMG)在内的全面神经学检查(表1)。(摘要截选至250字)