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去铁胺B对葡萄糖-6-磷酸脱氢酶缺乏症中溶血的影响。

Effect of desferrioxamine B on hemolysis in glucose-6-phosphate dehydrogenase deficiency.

作者信息

Khalifa A S, el-Alfy M S, Mokhtar G, Fakeir A A, Khazbak M A, el-Baz F, el-Kholy M

机构信息

Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

出版信息

Acta Haematol. 1989;82(3):113-6. doi: 10.1159/000205320.

Abstract

Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrioxamine B 500 mg intravenously followed by packed red cell transfusion, while the remaining 10 cases were only transfused. Sequential estimation of hemoglobin level, reticulocytic count and hemoglobinuria was done before treatment, 3, 24, 48 and 72 h thereafter. The hemoglobin level was higher in the desferrioxamine B group. The degree of increase was statistically significant at 48 and 72 h (p less than 0.01). Hemoglobinuria stopped in 78.5% in the first group and only in 30% of the second group at 72 h. It was concluded that desferrioxamine B is helpful in shortening the course of hemolytic crisis in G6PD-deficient patients. It could be used as an adjuvant to packed red cell transfusion.

摘要

本研究纳入了24名在溶血危象期间患有葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症的婴幼儿。他们的年龄在3至36个月之间,中位数为10个月。其中22名是男性,2名是女性。他们中有14人静脉注射了单次大剂量500毫克去铁胺B,随后进行了浓缩红细胞输血,而其余10例仅接受了输血。在治疗前、治疗后3小时、24小时、48小时和72小时对血红蛋白水平、网织红细胞计数和血红蛋白尿进行了连续评估。去铁胺B组的血红蛋白水平较高。在48小时和72小时时,升高程度具有统计学意义(p小于0.01)。在72小时时,第一组中78.5%的患者血红蛋白尿停止,而第二组中只有30%的患者血红蛋白尿停止。得出的结论是,去铁胺B有助于缩短G6PD缺乏症患者溶血危象的病程。它可用作浓缩红细胞输血的辅助药物。

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