HEMOGLOBIN-A1 IN GALACTOSEMIA, A POSSIBLE ROLE IN MONITORING DIETARY COMPLIANCE
HEMOGLOBIN-A1 IN GALACTOSEMIA, A POSSIBLE ROLE IN MONITORING DIETARY COMPLIANCE
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DOI：
10.1111/j.1651-2227.1981.tb05770.x
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发表时间：
1981-01-01
期刊：
影响因子：
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通讯作者：
MARTIN, JM
中科院分区：
文献类型：
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作者：
HOWARD, NJ;MONAGHAN, H;MARTIN, JM
发现 2 名半乳糖血症婴儿（分别为 24.1% 和 23.8%（正常为 6.3-8.2%））的 HbA1 水平升高，从而评估了饮食管理对半乳糖-1-磷酸尿苷基转移酶缺乏症和半乳糖激酶缺乏症的影响。在 2 个命题中排除膳食半乳糖和乳糖，导致 1 个命题中的 HbA1 水平降至正常，另一个命题中的 HbA1 水平降至正常。对 12 名转移酶缺乏症治疗受试者、7 名携带者和 1 名半乳糖激酶缺乏症儿童进行了 HbA1 测量。 5 名转移酶缺陷儿童的 HbA1 水平升高，其中 4 名同意在 6 周内严格排除半乳糖和乳糖的饮食。该试验导致所有受试者的 HbA1 水平下降（平均下降 12.6% 至平均 9.2%）。 HbA1 水平对于评估和确保半乳糖血症的饮食依从性可能有价值。
The finding of elevated HbA1 levels in 2 galactosemic infants of 24.1% and 23.8% (normal 6.3-8.2%) led to an assessment of the effects of dietary management in galactose-1-phosphate uridyl transferase deficiency and galactokinase deficiency. Exclusion of dietary galactose and lactose in the 2 propositi resulted in a reduction of HbA1 levels to normal in 1 and towards normal in the other. HbA1 was measured in 12 treated subjects with transferase deficiency, 7 carriers and 1 child with galactokinase deficiency. Five transferase deficient children had elevated HbA1 levels, and 4 of these agreed to a 6 wk strict dietary exclusion of galactose and lactose. This trial resulted in a fall in HbA1 levels in all subjects (mean 12.6% to mean 9.2%). HbA1 levels may be of value in assessing and ensuring dietary compliance in galactosemia.