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[葡萄糖-6-磷酸脱氢酶缺乏症及其他红细胞酶异常]

[Glucose-6-phosphate dehydrogenase deficiency and other erythrocyte enzyme abnormalities].

作者信息

Pohl A, Blechschmidt E, Moser K

出版信息

Wien Klin Wochenschr. 1984 Jul 6;96(14):542-8.

PMID:6089445
Abstract

Normal values of glucose-6-phosphate dehydrogenase (G6PD), 6-phosphogluconate dehydrogenase (6PGD), glutathione reductase (GR), glucosephosphate isomerase (GPI), pyruvate kinase (PK) and pyrimidine 5'-nucleotidase (P5N) have been determined in normocytes, reticulocytes, newborn cord erythrocytes, and leucocytes. Metabolic and clinical aspects of G6PD and the classification of its genetic variants are reviewed. Enzyme determinations and their variation in drug-induced haemolysis are critically presented. Extensive tables are published listing the drugs and compounds that can cause haemolysis in G6PD-deficient patients, as well as those preparations which may, probably, be administered safely. Clinical and biochemical data in patients with the inherited enzyme defects GR, GPI, PK, and P5N, as well as acquired deficiency of the last-mentioned in chronic lead intoxication, are reviewed in the light of our personal experience in this field.

摘要

已测定了正常红细胞、网织红细胞、新生儿脐带血红细胞和白细胞中葡萄糖-6-磷酸脱氢酶(G6PD)、6-磷酸葡萄糖酸脱氢酶(6PGD)、谷胱甘肽还原酶(GR)、葡萄糖磷酸异构酶(GPI)、丙酮酸激酶(PK)和嘧啶5'-核苷酸酶(P5N)的正常值。综述了G6PD的代谢和临床方面及其遗传变异的分类。批判性地介绍了酶的测定及其在药物诱导溶血中的变化。已发表了大量表格,列出了可导致G6PD缺乏患者溶血的药物和化合物,以及可能安全使用的制剂。根据我们在该领域的个人经验,对患有遗传性酶缺陷GR、GPI、PK和P5N的患者的临床和生化数据,以及慢性铅中毒中最后提到的酶的获得性缺乏进行了综述。

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