初诊苯丙酮尿症患者微量元素测定

目的探讨初诊PKU患者血清内微量元素铁、铜、锌的变化及与高苯丙氨酸的关系。方法采用分光光度法测定27例PKU患儿及42例正常儿童血清内的铁、铜、锌含量，用HPLC法测定血清内的苯丙氨酸浓度并对两者进行分析比较。结果PKU患儿血清铁、锌明显低于正常时照，血清铜无显著性差异。血清锌的水平与患者体内苯丙氨酸浓度有关。结论PKU患儿体内存在微量元素的缺乏，有必要在治疗时适当的补充微量元素以达到更好的治疗效果。

Determination of trace element in first visit patient with phenylketonuria

Objective: To investigate the change of concentration of trace element in children with phenylketonuria and the relationship of content between phenylalanine and trace element.Methods: A total of 27 first visit PKU cases were recruited in this study.A group of 42 healthy children were included in this study as a normal control.Their serum samples were analyzed for phenylalanine by HPLC,for iron,copper and zinc by spectro-photometer.Results:The concentration of serum iron and zinc in patients with PKU were significantly lower than normal control,there were no significant differences in serum copper between two groups.There was also a weak negative correlation between serum Phe concentrations and serum zinc levels.Conclusions:All patients with PKU have zinc and iron deficency.Serum trance elements should be detected and supplemented to patients with PKU in time to ensure a good therapy result.