Rosa R M, Bierer B E, Bunn H F, Epstein F H
Blood Cells. 1982;8(2):329-35.
Since the rate of polymerization of sickle hemoglobin is exquisitely dependent on its concentration, a small reduction in intracellular hemoglobin concentration should cause a significant inhibition of sickling. In three patients with homozygous sickle cell anemia, sustained hyponatremia was induced by a program consisting of a high fluid intake, a low salt diet and a vasopressin analog, DDAVP. During periods of hyponatremia, mean corpuscular hemoglobin concentration (MCHC) fell 13% and in vitro sickling was reduced as assessed by morphology and oxygen affinity. The frequency and duration of sickle cell crises appeared to be decreased during periods when patients were hyponatremic. These preliminary results indicate that reduction in intracellular hemoglobin concentration is an effective approach to the treatment of sickle cell anemia.
由于镰状血红蛋白的聚合速率对其浓度极为敏感，细胞内血红蛋白浓度的小幅降低应会显著抑制镰变。在三名纯合镰状细胞贫血患者中，通过高液体摄入量、低盐饮食和一种加压素类似物去氨加压素（DDAVP）组成的方案诱导出持续性低钠血症。在低钠血症期间，平均红细胞血红蛋白浓度（MCHC）下降了13%，并且通过形态学和氧亲和力评估发现体外镰变减少。在患者处于低钠血症的时期，镰状细胞危象的频率和持续时间似乎有所降低。这些初步结果表明，降低细胞内血红蛋白浓度是治疗镰状细胞贫血的一种有效方法。
