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镰状红细胞内聚合物形成的不均匀性:与溶血性贫血严重程度的可能关联。

Non-uniformity of intracellular polymer formation in sickle erythrocytes: possible correlation with severity of hemolytic anemia.

作者信息

Noguchi C T, Schechter A N

出版信息

Am J Pediatr Hematol Oncol. 1984 Spring;6(1):46-50.

PMID:6711762
Abstract

Intact sickle erythrocytes were fractionated to obtain a more uniform population in mean corpuscular hemoglobin concentration (MCHC). Polymer fraction at complete deoxygenation varied with increasing MCHC and, at varying oxygen saturation, was in good agreement with theoretical predictions based on solubility and the non-ideal behavior of concentrated hemoglobin solution and of water. These data and the theoretical analysis indicate that, in uniform sickle erythrocytes at 34 g/dl, polymer is only detected below 84% oxygen saturation. Non-uniformity of unfractionated sickle erythrocytes causes this critical oxygen saturation to shift to higher values (over 90%). The implication is that polymer may exist in arterial blood, a possibility which must be considered in therapeutic strategies. Recently, we showed that polymer formation correlated with the severity of hemolysis for 12 sickle syndromes varying from sickle trait to hemoglobin SS (African) disease.

摘要

对完整的镰状红细胞进行分级分离,以获得平均红细胞血红蛋白浓度(MCHC)更均匀的群体。完全脱氧时的聚合物级分随MCHC的增加而变化,并且在不同的氧饱和度下,与基于溶解度以及浓缩血红蛋白溶液和水的非理想行为的理论预测高度一致。这些数据和理论分析表明,在34 g/dl的均匀镰状红细胞中,仅在氧饱和度低于84%时才能检测到聚合物。未分级的镰状红细胞的不均匀性导致这个临界氧饱和度向更高值(超过90%)移动。这意味着聚合物可能存在于动脉血中,这是治疗策略中必须考虑的一种可能性。最近,我们表明,对于从镰状性状到血红蛋白SS(非洲人)病的12种镰状综合征,聚合物形成与溶血严重程度相关。

相似文献

1
Non-uniformity of intracellular polymer formation in sickle erythrocytes: possible correlation with severity of hemolytic anemia.镰状红细胞内聚合物形成的不均匀性:与溶血性贫血严重程度的可能关联。
Am J Pediatr Hematol Oncol. 1984 Spring;6(1):46-50.
2
Intracellular polymerization of sickle hemoglobin: disease severity and therapeutic goals.镰状血红蛋白的细胞内聚合:疾病严重程度与治疗目标
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Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.镰状血红蛋白的细胞内聚合。细胞异质性的影响。
J Clin Invest. 1983 Sep;72(3):846-52. doi: 10.1172/JCI111055.
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Determination of sickle hemoglobin polymer in SS and AS erythrocytes.SS型和AS型红细胞中镰状血红蛋白聚合物的测定。
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Rate of deoxygenation modulates rheologic behavior of sickle red blood cells at a given mean corpuscular hemoglobin concentration.在给定的平均红细胞血红蛋白浓度下,脱氧速率调节镰状红细胞的流变行为。
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Polymerization of sickle cell hemoglobin at arterial oxygen saturation impairs erythrocyte deformability.在动脉血氧饱和度下镰状细胞血红蛋白的聚合会损害红细胞的可变形性。
J Clin Invest. 1988 Jun;81(6):1669-74. doi: 10.1172/JCI113504.
7
Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes.血红蛋白S聚合:镰状细胞综合征溶血及临床严重程度的主要决定因素。
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Electron microscopic studies of the intracellular polymerization of sickle hemoglobin.镰状血红蛋白细胞内聚合的电子显微镜研究。
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Effects of carbon dioxide and pH variations in vitro on blood respiratory functions, red blood cell volume, transmembrane pH gradients, and sickling in sickle cell anemia.体外二氧化碳和pH值变化对镰状细胞贫血患者血液呼吸功能、红细胞体积、跨膜pH梯度及镰变的影响。
J Lab Clin Med. 1984 Aug;104(2):146-59.

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2
Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry.镰状细胞病成人死亡率:镰状细胞病实施联合会(SCDIC)登记处的结果。
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Oxygen-dependent flow of sickle trait blood as an in vitro therapeutic benchmark for sickle cell disease treatments.
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Am J Hematol. 2018 Oct;93(10):1227-1235. doi: 10.1002/ajh.25227. Epub 2018 Aug 21.
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Steady state hemoglobin concentration and packed cell volume in homozygous sickle cell disease patients in Lagos, Nigeria.尼日利亚拉各斯纯合子镰状细胞病患者的稳态血红蛋白浓度和红细胞压积
Caspian J Intern Med. 2012 Spring;3(2):405-9.
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Haematological values in homozygous sickle cell disease in steady state and haemoglobin phenotypes AA controls in Lagos, Nigeria.尼日利亚拉各斯稳态纯合子镰状细胞病及血红蛋白表型为AA的对照者的血液学值。
BMC Res Notes. 2012 Aug 1;5:396. doi: 10.1186/1756-0500-5-396.