Darling G, Mathias P, O'Regan M, Naughten E
Children's Hospital, Dublin, Eire.
J Inherit Metab Dis. 1992;15(5):769-73. doi: 10.1007/BF01800019.
Serum selenium levels in 73 patients with phenylketonuria were significantly lower than in controls. The phenylketonuric and hyperphenylalaninaemic individuals taking the non-supplemented amino acid mixture generally had lower levels: 36% were below the normal range as defined by our laboratory, compared with 19% in the supplemented group. The low levels were present even in those on diet, who had a greater phenylalanine tolerance--that is, a tolerance for more than 9 x 50 mg phenylalanine exchanges per day, in other words a higher intake of natural protein. Individuals on long-term synthetic diets may be at risk for selenium deficiency even on selenium supplements. In areas where the soil may be low in selenium, the deficiency may be aggravated. Long-term low levels may impair health but the required amount of selenium supplementation remains uncertain.
73例苯丙酮尿症患者的血清硒水平显著低于对照组。服用未添加硒的氨基酸混合物的苯丙酮尿症和高苯丙氨酸血症患者的硒水平通常较低:36%低于我们实验室定义的正常范围,而补充硒的组中这一比例为19%。即使是那些对苯丙氨酸耐受性较高(即每天能耐受超过9×50mg苯丙氨酸交换量,换句话说能摄入更多天然蛋白质)的饮食控制患者,也存在低硒水平。长期食用合成饮食的患者即使补充了硒,仍可能有硒缺乏的风险。在土壤硒含量可能较低的地区,这种缺乏情况可能会加剧。长期低硒水平可能损害健康,但所需的硒补充量仍不确定。