经典型半乳糖血症患者对口服半乳糖的高耐受性：饮食方面的影响
High tolerance for oral galactose in classical galactosaemia: dietary implications.
作者信息
Bosch A M, Bakker H D, Wenniger-Prick L J M de B, Wanders R J A, Wijburg F A
机构信息
Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands.
出版信息
Arch Dis Child. 2004 Nov;89(11):1034-6. doi: 10.1136/adc.2003.037671.
AIM
To study the relevance of restricting the exogenous intake of small amounts of galactose, such as from fruit and vegetables, in patients with classical galactosaemia.
METHODS
For a period of six weeks, increasing doses of oral galactose to a maximum of 600 mg per day, were added to a very strict galactose restricted diet in three adolescent patients homozygous for the Q188R mutation. During the study, physical examination, including an extended ophthalmic examination, and laboratory studies were performed on a weekly basis.
RESULTS
No significant change in any of the studied clinical or biochemical parameters was observed.
CONCLUSIONS
These findings provide further evidence that attempts to exclude trace amounts of galactose from the diet are not justified. Once the diet is made more liberal, a long term follow up study will be necessary.
目的
研究限制经典型半乳糖血症患者从水果和蔬菜等来源少量摄入外源性半乳糖的相关性。
方法
在三名纯合Q188R突变的青少年患者中，在非常严格的半乳糖限制饮食基础上，持续六周每天增加口服半乳糖剂量，最大至600毫克。研究期间，每周进行体格检查，包括全面眼科检查以及实验室检查。
结果
所研究的任何临床或生化参数均未观察到显著变化。
结论
这些发现进一步证明，试图从饮食中排除微量半乳糖是不合理的。一旦饮食更加宽松，有必要进行长期随访研究。
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