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目的:分析低血清铜蓝蛋白氧化酶活性在41例Wilson病(Wilson’sdisease,WD)和52例非Wilson病(non-Wilson’sdisease,NWD)患者之间的差异,协助临床尽早确诊或排除WD。方法:用分光光度计测定血清铜蓝蛋白(ceruloplasmin,CP)催化邻联大茴香胺产生的反应产物的吸光值,计算出酶活力。结果:WD组的CP活性为(11.17±13.80)IU·L-1,NWD组为(39.42±9.77)IU·L-1,两组差异有统计学意义P<0.001。部分NWD患者经治疗后,CP活性可显著升高,而WD组治疗前后未见明显变化。结论:极度降低的CP活性结合临床表现可确诊WD;治疗后CP活性显著升高,可作为排除WD的一个参考依据。
OBJECTIVE: To analyze the differences of the activity of low-grade ceruloplasmin oxidase between 41 Wilson’s disease patients (WD) and 52 non-Wilson’s disease patients (NWD) WD. Methods: The absorbance of the reaction products produced by adjuvant cydanilate was measured by spectrophotometer and the activity of enzyme was calculated. Results: The CP activity in WD group was (11.17 ± 13.80) IU · L-1 and that in NWD group was (39.42 ± 9.77) IU · L-1. The difference between the two groups was statistically significant (P <0.001). Some patients with NWD after treatment, CP activity was significantly increased, while no significant changes in the WD group before and after treatment. Conclusion: The extremely decreased activity of CP combined with clinical manifestations can confirm the diagnosis of WD. After treatment, the CP activity is significantly elevated, which can be used as a reference for excluding WD.