Johansson E, Lindh U, Westermarck T, Heiskala H, Santavuori P
Department of Radiation Sciences, Division of Physical Biology, Uppsala, Sweden.
J Trace Elem Electrolytes Health Dis. 1990 Sep;4(3):139-42.
The elemental profiles of thrombocytes and mononuclear cells were investigated in five patients with Infantile and eight with Juvenile Neuronal Ceroid Lipofuscinosis. The patients with the infantile form had suffered from the disease for a year and those with the juvenile form for some six years. The thrombocytes exhibited increased concentrations of calcium and magnesium, but the same concentrations of iron and zinc as found in healthy subjects. The mononuclear cells exhibited an increased concentration of iron and a reduced concentration of zinc. The elevated concentrations of magnesium, calcium and iron in the thrombocytes and mononuclear cells may represent the end products of ceroid pigmentation. Five patients with Juvenile and one with Infantile Neuronal Ceroid Lipofuscinosis were treated with antioxidants along with vitamins E, B2 and B6, but this treatment did not affect significantly the concentration of iron in the mononuclear cells. However, selenium was detected in some mononuclear cells in all the patients so treated. This was unexpected since iron (III), being antagonistic to selenium in the form of selenite--which was the antioxidant given--forms a stable complex which cannot be broken down biologically.
对5例婴儿型和8例青少年型神经元蜡样脂褐质沉积症患者的血小板和单核细胞的元素谱进行了研究。婴儿型患者患病1年，青少年型患者患病约6年。血小板中钙和镁的浓度升高，但铁和锌的浓度与健康受试者相同。单核细胞中铁的浓度升高，锌的浓度降低。血小板和单核细胞中镁、钙和铁浓度的升高可能代表蜡样色素沉着的终产物。5例青少年型和1例婴儿型神经元蜡样脂褐质沉积症患者接受了抗氧化剂以及维生素E、B2和B6的治疗，但这种治疗对单核细胞中铁的浓度没有显著影响。然而，在所有接受治疗的患者的一些单核细胞中检测到了硒。这是出乎意料的，因为以亚硒酸盐形式存在的铁（III）与硒具有拮抗作用——亚硒酸盐是所给予的抗氧化剂——会形成一种稳定的复合物，这种复合物不能被生物分解。
