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对进行性肌营养不良症(PMD)71例、运动神经元病(MND)35例及假肥大型PMD基因携带者22例共128例进行了血清CK及其同工酶MB活性的测定。以正常人215名作为CK对照组,另20名作为MB活性对照组。CK用肌酸显色法测定,MB活性用定量柱层析法测定。不论何型PMD或MND之CK含量均显著高于对照组。CK含量之增多以假肥大型PMD最甚,肢带型及面肩肱型次之,MND再次之。但各型PMD或MND之CK个体值离散程度大,交叉重叠多,故单凭CK值不能区分PMD或AND的各种类型。各型PMD或MND的MB个体值离散程度小,尤其假肥大型PMD的MB值增多最显著,特异性极强,为诊断假肥大型PMD最具特征性的指标,其他两型PMD及两型MND则否,故无鉴别意义。对22例假肥大型PMD携带者CK与MB测定的结果表明似仍以CK值较为灵敏。对假肥大型PMD患者血清中MB的来源作了讨论。
Serum CK and its isoenzyme MB activity were measured in 71 cases of progressive muscular dystrophy (PMD), 35 cases of motor neuron disease (MND), and 22 cases of dutiable PMD gene in 22 cases. 215 normal subjects were used as CK control group and the other 20 as MB active control group. CK was determined by creatinine colorimetry and MB activity was determined by quantitative column chromatography. No matter what type of PMD or MND of CK were significantly higher than the control group. The increase of CK content is the largest in dull hypertrophic PMD, followed by brachiocephalic and facial and shoulder humerus, followed by MND. However, each individual PMD or MND CK discrete values of individual large, overlapping and more, so alone CK value can not distinguish between the various types of PMD or AND. The PM value of each type of PMD or MND is less discrete. In particular, the MB value of the dummy hypertrophic PMD increases most significantly and has extremely strong specificity, which is the most characteristic index for diagnosing the dull PMD. The other two types of PMD and two types MND is not, so no distinction. The results of CK and MB determination of 22 cases of fake PMD carriers showed that the value of CK was still more sensitive. The origin of MB in the serum of duchenme PMD patients is discussed.