以色列阿拉伯人中伴有高红细胞2,3-二磷酸甘油酸的良性镰状细胞贫血。

Benign sickle cell anemia in Israeli-Arabs with high red cell 2,3 diphosphoglycerate.

作者信息

Roth E F, Rachmilewitz E H, Schifter A, Nagel R L

出版信息

Acta Haematol. 1978;59(4):237-45. doi: 10.1159/000207767.

DOI:10.1159/000207767

Abstract

Arabs living near the Sea of Galilee were found to be homozygous for hemoglobin S. Studies of solubility, mechanical precipitability, electrophoretic mobility on starch-gel and citrate agar media, minimum gelling concentration, and peptide mapping of the hemoglobin beta-chain confirmed complete identity of the hemoglobin with that found in Afro-American hemoglobin S homozygotes. A comparison of Arab Hb S homozygotes with Afro-American Hb S patients showed no significant differences in hemoglobin levels, red cell indices or morphology. Hb F averaged 4.4% in Arab patients. The 2,3 diphosphoglycerate levels were increased approximately twofold in Arabs, whereas in Afro-Americans, it was increased by only 7% in females and 20% in males.

摘要

居住在加利利海附近的阿拉伯人被发现血红蛋白S呈纯合状态。对其溶解度、机械沉淀性、在淀粉凝胶和柠檬酸盐琼脂培养基上的电泳迁移率、最低凝胶浓度以及血红蛋白β链的肽图谱分析，证实该血红蛋白与非裔美国血红蛋白S纯合子中的血红蛋白完全相同。将阿拉伯血红蛋白S纯合子与非裔美国血红蛋白S患者进行比较，发现血红蛋白水平、红细胞指数或形态上无显著差异。阿拉伯患者的血红蛋白F平均为4.4%。阿拉伯人的2,3 - 二磷酸甘油酸水平大约增加了两倍，而非裔美国人中，女性仅增加7%，男性增加20%。