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 B₆ and B₁₂ during one week and with folate during the following week. Circulating folate, homocysteine, vitamin B₆ and vitamin B₁₂ levels were measured at baseline (patients and controls), after one week and after two weeks (patients). The patients had similar folate, vitamin B₆, and vitamin B₁₂, but higher homocysteine levels compared with HbAA controls (12.7 + 4.5 vs. 10.9 + 3.5 μmol/l; P = 0.04). Vitamin B₆ and B₁₂ 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 B₆ and B₁₂ status, but suboptimal folate status, leading to elevated plasma homocysteine levels. They may therefore benefit from folate supplementation to reduce their high risk for endothelial damage. Am. J. Hematol. 59:192–198, 1998. © 1998 Wiley-Liss, Inc.