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葡萄糖-6-磷酸脱氢酶缺乏的红细胞受者体内还原型谷胱甘肽的含量及其后果。

Content of reduced glutathione and consequences in recipients of glucose-6-phosphate dehydrogenase deficient red blood cells.

作者信息

Huang C S, Sung Y C, Huang M J, Yang C S, Shei W S, Tang T K

机构信息

Department of Laboratory Medicine, Cathay General Hospital, Taipei, Taiwan, Republic of China.

出版信息

Am J Hematol. 1998 Mar;57(3):187-92. doi: 10.1002/(sici)1096-8652(199803)57:3<187::aid-ajh1>3.0.co;2-r.

Abstract

The red blood cell glucose-6-phosphate dehydrogenase (G6PD) activity of every donor was examined with automatic enzyme-coupled method. The technique of molecular biology was applied to determine the DNA mutations for the 97 donors with undetectable G6PD activity. The concentration of reduced glutathione (GSH) in the stored RBC of the 97 G6PD-deficient donors and 124 normal donors was determined with the technique of high performance liquid chromatography. Routine blood counts, bilirubin and haptoglobin levels were used to evaluate posttransfusional hemolysis for the 48 adult patients transfused with 1 U G6PD deficient and 1 U normal RBC. Most (88, 90.7%) of the 97 donors were confirmed to be G6PD deficient at the DNA level. At each age interval of storage, the GSH concentration of G6PD-deficient RBC was significantly different from that of normal RBC. The total average value of GSH (pmol/gHb) was 2.52 +/- 0.95 (mean +/- 1 standard deviation) vs. 3.74 +/- 1.43 (P < 0.001). Hemoglobin, hematocrit, bilirubin, and haptoglobin levels in the patients receiving G6PD-deficient RBC were not statistically different from those in the recipients of normal RBC; even though the age of stored blood was 26-35 days. Within the same group of patients, the results of bilirubin and haptoglobin were not significantly changed before and after transfusion. The results of this study show that the GSH concentration in the stored blood of G6PD deficient donors was 67% of that in the normal donors. However, hemolysis does not occur in adult patients transfused with 1 U G6PD-deficient RBC. It seems unnecessary to screen G6PD activity for donors of adult recipients in Taiwan.

摘要

采用自动酶联法检测每位献血者的红细胞葡萄糖-6-磷酸脱氢酶(G6PD)活性。应用分子生物学技术对97例G6PD活性检测不到的献血者进行DNA突变检测。采用高效液相色谱技术测定97例G6PD缺乏献血者和124例正常献血者储存红细胞中还原型谷胱甘肽(GSH)的浓度。对48例输注1单位G6PD缺乏红细胞和1单位正常红细胞的成年患者,采用常规血细胞计数、胆红素和触珠蛋白水平评估输血后溶血情况。97例献血者中,大多数(88例,90.7%)在DNA水平被确认为G6PD缺乏。在每个储存年龄区间,G6PD缺乏红细胞的GSH浓度与正常红细胞有显著差异。GSH的总平均值(pmol/gHb)为2.52±0.95(平均值±1标准差),而正常红细胞为3.74±1.43(P<0.001)。接受G6PD缺乏红细胞的患者血红蛋白、血细胞比容、胆红素和触珠蛋白水平与接受正常红细胞的受者相比无统计学差异;即使储存血液的年龄为26 - 35天。在同一组患者中,输血前后胆红素和触珠蛋白结果无显著变化。本研究结果表明,G6PD缺乏献血者储存血液中的GSH浓度是正常献血者的67%。然而,输注1单位G6PD缺乏红细胞的成年患者未发生溶血。在台湾,似乎没有必要对成年受血者的献血者进行G6PD活性筛查。

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