治疗对苯丙酮尿症患儿锌、铜和钙状况的影响。

The effect of treatment on zinc, copper and calcium status in children with phenylketonuria.

作者信息

Taylor C J, Moore G, Davidson D C

出版信息

J Inherit Metab Dis. 1984;7(4):160-4. doi: 10.1007/BF01805600.

Abstract

The trace element status of 25 children receiving a low phenylalanine diet for a treatment of phenylketonuria was studied using hair and serum samples. Hair trace element and calcium concentrations were assayed using X-ray microanalysis and compared with samples from sibling controls. Plasma, zinc and copper levels were compared with unselected hospital attenders. PKU patients were found to have significantly lower hair zinc (P less than 0.001) and calcium (P less than 0.01) concentrations and significantly hair copper (P less than 0.001) concentrations compared with controls. Plasma zinc levels were below the lower limit of normal (9.6 mumol l-1) in 8 of 19 patients (42%) in whom it was measured. The relative zinc deficiency in PKU patients may result from a competitive inhibition of absorption by copper or other metals supplemented in the diet.

摘要

采用头发和血清样本，对25名接受低苯丙氨酸饮食治疗苯丙酮尿症的儿童的微量元素状况进行了研究。使用X射线微量分析法测定头发微量元素和钙浓度，并与同胞对照样本进行比较。将血浆、锌和铜水平与未经过挑选的就诊患儿进行比较。结果发现，与对照组相比，苯丙酮尿症患者的头发锌浓度（P<0.001）和钙浓度（P<0.01）显著降低，头发铜浓度也显著降低（P<0.001）。在19名接受检测的患者中，有8名（42%）的血浆锌水平低于正常下限（9.6 μmol l-1）。苯丙酮尿症患者相对锌缺乏可能是由于饮食中补充的铜或其他金属对锌吸收的竞争性抑制所致。