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葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症女性杂合子中伯氨喹所致溶血性贫血。

Primaquine-induced haemolysis in females heterozygous for G6PD deficiency.

机构信息

Shoklo Malaria Research Unit, Faculty of Tropical Medicine, Mahidol-Oxford Tropical Medicine Research Unit, Mahidol University, Mae Sot, Thailand.

Centre for Tropical Medicine and Global Health, Nuffield Department of Medicine, University of Oxford, Oxford, UK.

出版信息

Malar J. 2018 Mar 2;17(1):101. doi: 10.1186/s12936-018-2248-y.

DOI:10.1186/s12936-018-2248-y
PMID:29499733
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5833093/
Abstract

Oxidative agents can cause acute haemolytic anaemia in persons with G6PD deficiency. Understanding the relationship between G6PD genotype and the phenotypic expression of the enzyme deficiency is necessary so that severe haemolysis can be avoided. The patterns of oxidative haemolysis have been well described in G6PD deficient hemizygous males and homozygous females; and haemolysis in the proportionally more numerous heterozygous females has been documented mainly following consumption of fava beans and more recently dapsone. It has long been known that 8-aminoquinolines, notably primaquine and tafenoquine, cause acute haemolysis in G6PD deficiency. To support wider use of primaquine in Plasmodium vivax elimination, more data are needed on the haemolytic consequences of 8-aminoquinolines in G6PD heterozygous females. Two recent studies (in 2017) have provided precisely such data; and the need has emerged for the development of point of care quantitative testing of G6PD activity. Another priority is exploring alternative 8-aminoquinoline dosing regimens that are practical and improve safety in G6PD deficient individuals.

摘要

氧化剂可导致 G6PD 缺乏症患者发生急性溶血性贫血。了解 G6PD 基因型与酶缺乏表型表达之间的关系是必要的,以便避免严重溶血。在 G6PD 缺乏的半合子男性和纯合子女性中,氧化溶血模式已得到很好的描述;而在比例更高的杂合子女性中,溶血主要发生在食用蚕豆后,最近又发生在使用氨苯砜后。人们早就知道 8-氨基喹啉类药物,特别是伯氨喹和他非诺喹,可导致 G6PD 缺乏症患者发生急性溶血。为了支持在消除间日疟原虫中更广泛地使用伯氨喹,需要更多关于 G6PD 杂合子女性中 8-氨基喹啉类药物溶血后果的数据。最近(2017 年)的两项研究提供了正是这样的数据;并且需要开发即时检测 G6PD 活性的定量检测方法。另一个优先事项是探索替代的 8-氨基喹啉给药方案,这些方案在 G6PD 缺乏个体中具有实用性并提高安全性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/8d966299d8e7/12936_2018_2248_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/c562bd78be58/12936_2018_2248_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/f54fde8b96d3/12936_2018_2248_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/8d966299d8e7/12936_2018_2248_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/c562bd78be58/12936_2018_2248_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/f54fde8b96d3/12936_2018_2248_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acf9/5833093/8d966299d8e7/12936_2018_2248_Fig3_HTML.jpg

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