假肥大型肌营养不良患者血清肌酸激酶变化规律

Changes of serum creatine kinase levels in children with Duchenne muscular dystrophy

目的假肥大型肌营养不良患者血清肌酸激酶随病程进展而不断变化。该研究旨在分析假肥大型肌营养不良患者血清肌酸激酶的变化规律。方法收集临床已确诊的假肥大型肌营养不良患者的血清，其中男性39例，女性1例，通过连续监测法测定血清肌酸激酶。结果假肥大型肌营养不良患者血清肌酸激酶在3—5岁达到峰值，以后随病程进展和年龄增加逐年下降，年均下降率为8．7％。结论假肥大型肌营养不良患者的血清肌酸激酶在3—5岁达到峰值，以后随年龄增加而逐年下降，这种变化规律可以反映肌纤维坏死速率和病程进展速度，评估治疗效果。

Objective Duchenne muscular dystrophy （DMD） usually occurs prior to 3 years old. The value of serum creatine kinase changes with clinical progression and age in patients with DMD. This study aimed to investigate the regularity in the changes of serum creatine kinase activities in children with DMD. Methods Peripheral blood samples were obtained from 40 children with DMD （ranged from 3-14 years）. Serum creatine kinase levels were assayed by kinetic UV test. Results Serum creatine kinase level in the 40 DMD patients （ ranged from 2 595- 45 495 U/L） was remarkably higher than the reference value （35-174 U/L）. The highest serum creatine kinase level （average： 27 750-31 173 U/L） was found in 3-5 years old patients. Mterwards, serum creatine kinase level decreased with clinical progression and age, with a yearly average rate of decline was 8.7%. Conclnsions Serum creatine kinase level reaches a peak between 3 and 5 years old and then reduces with increasing age in children with DMD. The characteristic changes of serum creatine kinase are suspected to reflect the rate of muscle decay.