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目的:研究限制典型半乳糖血症患者从水果和蔬菜等少量摄取外源性半乳糖的相关因素。方法:笔者进行一个为期6周的研究。对3名Q188R突变的纯合子青少年患者添加严格限制的半乳糖饮食,并不断增加口服半乳糖的量,直到给予600mg/d的最大量。每周进行包括全面的眼科检查和实验室研究在内的体格检查。结果:在这些研究中没有发现有明显的临床或生化指标的改变。结论:这些研究进一步表明试图将微量半乳糖排除在饮食之外是不正确的。一旦给予自由饮食,就有必要进行长期的随访研究。
OBJECTIVE: To study the factors that limit the uptake of exogenous galactose from fruits and vegetables in patients with typical galactosemia. Methods: The author conducted a 6-week study. Three homozygous adolescent patients with the Q188R mutation were given a strictly limited galactose diet and the amount of oral galactose was continuously increased until the maximum amount of 600 mg / d was given. Weekly physical exams including comprehensive eye exams and laboratory studies. RESULTS: No significant clinical or biochemical changes were found in these studies. Conclusion: These studies further suggest that trying to exclude micro-galactose from the diet is incorrect. Once given a free diet, there is a need for long-term follow-up studies.