Natta C L, Reynolds R D
Am J Clin Nutr. 1984 Aug;40(2):235-9. doi: 10.1093/ajcn/40.2.235.
In 16 patients with sickle cell anemia, plasma pyridoxal phosphate (PLP) concentrations were significantly lower than in 16 controls (p less than 0.0001) whereas sickle cell anemia erythrocyte PLP concentrations were significantly elevated (p less than 0.0005), possibly reflecting a greater affinity of PLP to the sickle Hb beta chain compared to the normal beta chain. Oral supplementation of five patients with 50 mg pyridoxine twice daily for 2 months resulted in increased plasma and erythrocyte PLP levels and a slight, but not significant, increase in erythrocyte cell number, Hb, concentration, and hematocrit. In one subject there was also a reduction in the frequency and duration of painful crises and a virtual elimination of hospitalizations for the treatment of the painful crises. Since pyridoxal and PLP have been shown to have antisickling properties in vitro, these studies suggest that pyridoxine supplementation may also be of therapeutic benefit in vivo in sickle cell anemia.
在16例镰状细胞贫血患者中，血浆磷酸吡哆醛（PLP）浓度显著低于16名对照者（p<0.0001），而镰状细胞贫血患者红细胞中的PLP浓度显著升高（p<0.0005），这可能反映出与正常β链相比，PLP对镰状血红蛋白β链具有更高的亲和力。对5例患者每日口服两次50mg吡哆醇，持续2个月，结果血浆和红细胞中的PLP水平升高，红细胞数量、血红蛋白、浓度和血细胞比容略有增加，但不显著。有1例患者疼痛性危象的发作频率和持续时间也有所降低，并且几乎无需因疼痛性危象住院治疗。由于已证明吡哆醛和PLP在体外具有抗镰变特性，这些研究表明，补充吡哆醇在体内对镰状细胞贫血也可能具有治疗益处。
