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葡萄糖-6-磷酸脱氢酶缺乏症患者的红细胞流变学良好(作者译)

[Favourable erythrocyte rheology in patients with glucose-6-phosphate dehydrogenase deficiency (author's transl)].

作者信息

Tillman W, Labitxke N, Schröter W

出版信息

Klin Wochenschr. 1977 Apr 15;55(8):385-91. doi: 10.1007/BF01488624.

DOI:10.1007/BF01488624
PMID:870749
Abstract

The rheological properties of erythrocytes of 6 patients with glucose-6-phosphate dehydrogenase deficiency were studied. A method of filtration and measurements of viscosity of erythrocyte suspensions with a hematocrit of 80% were used. The erythrocytes of all patients were more flexible than the erythrocytes of healthy controls. The erythrocytes of the patients passed the polycarbonate filters with a pore diameter of 5 micron more rapidly and showed a lower viscosity of erythrocyte suspensions. The increased flexibility of erythrocytes was due to a lower hemoglobin content which was measured in isolated ghosts. After incubation of the cells with acetylphenylhydrazine, Heinz bodies formed in the erythrocytes of controls. The flexibility of these cells decreased markedly. Though more Heinz bodies were found in the erythrocytes of the patients when the cells were incubated under identical conditions, even these erythrocytes became less rigid than the erythrocytes of controls. From these results we condlude that the more flexible erythrocytes of the patients could pass the splenic sinus even when they contain Heinz bodies. The rheological properties of the erythrocytes explain why splenectomy is not efficient in glucose-6-phosphate dehydrogenase deficiency.

摘要

对6例葡萄糖-6-磷酸脱氢酶缺乏症患者的红细胞流变学特性进行了研究。采用了一种过滤方法,并对血细胞比容为80%的红细胞悬液的粘度进行了测量。所有患者的红细胞比健康对照者的红细胞更具柔韧性。患者的红细胞能更快地通过孔径为5微米的聚碳酸酯滤器,且红细胞悬液的粘度较低。红细胞柔韧性增加是由于在分离的空壳中测得的血红蛋白含量较低。用乙酰苯肼孵育细胞后,对照组红细胞中形成了海因茨小体。这些细胞的柔韧性明显降低。尽管在相同条件下孵育时,患者红细胞中发现了更多的海因茨小体,但即使是这些红细胞也比对照组红细胞的刚性小。从这些结果我们得出结论,患者红细胞更具柔韧性,即使含有海因茨小体也能通过脾窦。红细胞的流变学特性解释了为什么脾切除术对葡萄糖-6-磷酸脱氢酶缺乏症无效。

相似文献

1
[Favourable erythrocyte rheology in patients with glucose-6-phosphate dehydrogenase deficiency (author's transl)].葡萄糖-6-磷酸脱氢酶缺乏症患者的红细胞流变学良好(作者译)
Klin Wochenschr. 1977 Apr 15;55(8):385-91. doi: 10.1007/BF01488624.
2
[Decreased flexibility of newborn infant erythrocytes].[新生儿红细胞柔韧性降低]
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Protection by ascorbate against acetylphenylhydrazine-induced Heinz body formation in glucose-6-phosphate dehydrogenase deficient erythrocytes.抗坏血酸对葡萄糖-6-磷酸脱氢酶缺乏的红细胞中乙酰苯肼诱导的海因茨小体形成的保护作用。
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引用本文的文献

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The fatty acid composition of red cells deficient in glucose-6-phosphate dehydrogenase and their susceptibility to lipid peroxidation.葡萄糖-6-磷酸脱氢酶缺乏的红细胞的脂肪酸组成及其对脂质过氧化的敏感性。
Klin Wochenschr. 1985 Jul 1;63(13):578-82. doi: 10.1007/BF01733008.
2
[Porphyria erythropoietica congenita Günther and chloroquine (author's transl)].先天性红细胞生成性卟啉病(京特氏病)与氯喹(作者译)
Klin Wochenschr. 1978 Jun 15;56(12):623-4. doi: 10.1007/BF01477011.

本文引用的文献

1
HEINZ BODY ANEMIA--AN ULTRASTRUCTURAL STUDY. I. HEINZ BODY FORMATION.海因茨小体贫血——一项超微结构研究。I. 海因茨小体的形成
Blood. 1965 Jun;25:885-96.
2
The preparation and chemical characteristics of hemoglobin-free ghosts of human erythrocytes.人红细胞无血红蛋白空泡的制备及其化学特性
Arch Biochem Biophys. 1963 Jan;100:119-30. doi: 10.1016/0003-9861(63)90042-0.
3
[Erythrocyte isolation from blood with cotton].[用棉花从血液中分离红细胞]
Klin Wochenschr. 1966 Aug 15;44(16):983-4. doi: 10.1007/BF01711475.
4
Heinz body anemia: an ultrastructural study. II. Red cell sequestration and destruction.海因茨小体贫血:超微结构研究。II. 红细胞的扣押与破坏。
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Influence of deformability of human red cells upon blood viscosity.人类红细胞变形能力对血液黏度的影响。
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Fluid drop-like transition of erythrocytes under shear.红细胞在剪切力作用下的液滴状转变。
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7
Biochemical variants of glucose-6-phosphate dehydrogenase giving rise to congenital nonspherocytic hemolytic disease.导致先天性非球形红细胞溶血性贫血的葡萄糖-6-磷酸脱氢酶的生化变异体。
Blood. 1968 Feb;31(2):131-50.
8
In vivo lability of glucose-6-phosphate dehydrogenase in GdA- and GdMediterranean deficiency.葡萄糖-6-磷酸脱氢酶在GdA和地中海型Gd缺乏症中的体内不稳定性。
J Clin Invest. 1968 Apr;47(4):940-8. doi: 10.1172/JCI105786.
9
Glucose-6-phosphate dehydrogenase (G-6-PD) Hamburg, a new variant with chronic nonspherocytic haemolytic anaemia.
Eur J Clin Invest. 1974 Jun;4(3):187-91. doi: 10.1111/j.1365-2362.1974.tb00390.x.
10
A simple method for measuring red cell deformability in models of the microcirculation.一种在微循环模型中测量红细胞变形性的简单方法。
Blut. 1973 Jun;26(6):369-79. doi: 10.1007/BF01632746.