Elevated homocysteine levels indicate suboptimal folate status in pediatric sickle cell patients

We investigated whether pediatric patients with sickle cell disease (SCD) (9 +/- 4 years; 27 homozygous SCD [HbSS]; 19 sickle-C disease [HbSC]) have different folate status compared with age-, sex-, and race-matched normal hemoglobin (HbAA) controls (n = 20), and whether their folate status can be improved by folate supplementation. The patients were supplemented with vitamins B-6 and B-12 during one week and with folate during the following week. Circulating folate, homocysteine, vitamin B-6 and vitamin B-12 levels were measured at baseline (patients and controls), after one week and after two weeks (patients). The patients had similar folate, vitamin B-6, and vitamin B-12, but higher homocysteine levels compared with HbAA controls (12.7 +/- 4.5 vs. 10.9 +/- 3.5 mu mol/l; P = 0.04). Vitamin B-6 and B-12 supplementation did not change their homocysteine levels, but folate supplementation caused a 53% reduction (to 5.7 +/- 1.6), We conclude that patients with SCD have adequate vitamin B-6 and B-12 status, but suboptimal folate states, leading to elevated plasma homocysteine levels. They may therefore benefit from folate supplementation to reduce their high risk for endothelial damage, (C) 1998 Wiley-Liss, Inc.