钼缺乏

定义

钼在人体内作为有限数量酶的辅助因子发挥作用：亚硫酸盐氧化脢、黄嘌呤氧化酶和醛氧化酶。一种罕见的严重代谢缺陷导致钼辅助因子缺乏，并阻止这些酶的合成。有这种缺陷的婴儿很少能在生命的最初几天存活下来，存活下来的婴儿有严重的神经系统异常。尽管与饮食缺乏有关的钼缺乏在人类中极为罕见，但长期全肠外营养中已将其描述为亚硫酸盐给药后的继发性钼缺乏。症状包括：心动过速、头痛、夜盲、易怒和昏迷。生化变化可包括血浆和蛋氨酸浓度升高、血清尿酸浓度降低、尿硫代硫酸盐升高和尿中尿酸和硫酸盐水平降低。

Molybdenum functions as a cofactor for a limited number of enzymes in humans: sulphite oxidase, xanthine oxidase and aldehyde oxidase. A rare severe metabolic defect causing molybdenum cofactor deficiency and preventing these enzymes from being synthesized has been described. Few infants with such defects survive the first days of life, and those who survive have severe neurological abnormalities. Although molybdenum deficiency related to a dietary deficiency is extremely rare in humans, it has been described in long-term total parenteral nutrition as being secondary to the administration of sulphite. Symptoms include: tachycardia, headache, night blindness, irritability and coma. Biochemical changes can consist of elevated plasma and methionine concentration, low serum uric acid concentration, high urinary thiosulfate and low urinary uric acid and sulphate levels.

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• 钼缺乏