进行性肌营养不良症及运动神经元疾病的血清CK及其同工酶MB测定的诊断意义

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。

DIAGNOSTIC VALUE OF SERUM CREATINE KINASE AND ITS ISOENZYME MB DETERMINATION IN PROGRESSIVE MUSCULAR DYSTROPHY AND MOTOR NEURON DISEASE

The serum oreatine kinase(CK)and its isoenzyme MB activities were determined in 128 patients including 71 cases of progressive muscular dystrophy (PMD), 35 cases of motor neuron disease (MND) and 22 PMD carriers of pseudohypertrophic type. 235 healthy blood donors served as controls (215 for OK, 20 for MB). Determination of CK total activity was performed by using creatine colour development technique, and fractionation of MB, by using quantitative column chroma tography. total CK activity of all types of PMD or MND were significantly higher than that of controls. CK value of PMD in pseudohypertrophic type was .higher than that of PMD of limb-girdle or fascioscapulohumeral type which was, in turn, higher than that in MND. Our data showed that it was impossible to differentiate between the various types of PMD and MND based upon the total CK activity alone, because of scattered and wide overlapping of their individual values However, MB activity among the various types of PMD or MND did not overlap. It showed most significant increase in PMD of pseudohypertrophio type. The prominent increase in MB value was quite characteristic and diagnostic for this type of PMD. But MB was not helpful in differentiating other types of PMD and MND. Our data also showed that CK remained to be a more sensitive index than MB in detecting PMD carriers. The source of serum MB In patients with pseudohy per trophic type of PMD was discussed.