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[无嘌呤饮食对莱施-奈恩综合征患者红细胞中嘌呤磷酸核糖基转移酶活性的影响]

[Effect of a purine-free diet on the purine phosphoribosyltransferase activity of erythrocytes in patients with Lesch-Nyhan syndrome].

作者信息

Semenova I A, Pen'kovskaia N P

出版信息

Vopr Med Khim. 1984 Jul-Aug;30(4):94-7.

PMID:6506590
Abstract

Activity of purine phosphoribosyl transferases was studied in 11 patients with Lesch-Nyhan syndrome before and within 1 or 3 months after beginning of the purine-free diet treatment. The enzymatic activity tended to normalization within 1 month after the dietary treatment but the ratio of the enzymes activity was deteriorated within the subsequent 3 months of treatment. A positive clinical effect of the purine-free diet was noted.

摘要

在11例莱施-奈恩综合征患者开始无嘌呤饮食治疗前以及治疗开始后1个月或3个月内,对嘌呤磷酸核糖基转移酶的活性进行了研究。饮食治疗后1个月内酶活性趋于正常化,但在随后的3个月治疗中酶活性比例恶化。观察到无嘌呤饮食有积极的临床效果。

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1
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Dietary-induced variation of hypoxanthine-guanine phosphoribosyl transferase activity in patients with the Lesch-Nyhan syndrome.莱施-奈恩综合征患者饮食诱导的次黄嘌呤-鸟嘌呤磷酸核糖基转移酶活性变化
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Hypoxanthine-guanine phosphoribosyltransferase: characteristics of the mutant enzyme in erythrocytes from patients with the Lesch-Nyhan syndrome.次黄嘌呤-鸟嘌呤磷酸核糖转移酶:莱施-奈恩综合征患者红细胞中突变酶的特征
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