Urinary and Serum Galactitol in Galactosemic Patients
Urinary and Serum Galactitol in Galactosemic Patients
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半乳糖血症患者的尿液和血清半乳糖醇
DOI：
10.1111/j.1442-200x.1991.tb01521.x
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发表时间：
1991
影响因子：
1.4
通讯作者：
M. Hayashi
中科院分区：
文献类型：
--
作者：
Takeshi Yamazaki;M. Mino;M. Hayashi
在两个典型的半乳糖血症患者的血清和尿半乳糖醇水平进行了检查。即使在严格的饮食治疗下，半乳糖醇水平也远高于健康对照组。在一名6天大的患者中，他在诊断前吃了大量的半乳糖，尿半乳糖醇需要两周多的时间才能降低到稳定值。在一例8岁病例中，在1.25 g/kg半乳糖负荷后，尿半乳糖醇需要超过10天才能达到基线水平。在这两名患者中，尿半乳糖水平迅速下降。在后一患者中，在加载少量半乳糖（5g，相当于200 ml牛奶）后，与半乳糖相比，尿和血清半乳糖醇的较高水平维持较长时间，但它们在24小时后恢复到基线水平。根据这些结果，我们讨论了使用尿半乳糖醇作为检查半乳糖摄入量的指标。
Serum and urinary galactitol levels were examined in two patients with classical galactosemia. Even under strict dietary therapy, galactitol levels were much higher than those of healthy controls. In a 6‐day‐old patient who had eaten large amounts of galactose before diagnosis, it took more than two weeks for urinary galactitol to decrease to the stable value. In an 8‐year‐old case, more than ten days were required for urinary galactitol to reach the base line level after a galactose load of 1.25 g/kg. In both patients, the urinary galactose levels decreased rapidly. After loading with a small amount of galactose (5 g, equivalent to 200 ml of milk) in the latter patient, higher levels of urinary and serum galactitol were maintained for a long time, as compared with galactose, but they returned to baseline level after 24 hours. From these results, we discussed the use of urinary galactitol as an index to check galactose intake.