ELEVATED HOMOCYSTEINE LEVELS INDICATE SUBOPTIMAL FOLATE STATUS IN PEDIATRIC SICKLE-CELL PATIENTS

We investigated whether pediatric patients with sickle cell disease (S CD) (9 +/- 4 years; 27 homozygous SCD [HbSS]; 19 sickle-C disease [HbS C]) have different folate status compared with age-, sex-, and race-ma tched normal hemoglobin (HbAA) controls (n = 20), and whether their fo late status can be improved by folate supplementation. The patients we re supplemented with vitamins B-6 and B-12 during one week and with fo late during the following week. Circulating folate, homocysteine, vita min B-6 and vitamin B-12 levels were measured at baseline (patients an d controls), after one week and after two weeks (patients). The patien ts had similar folate, vitamin B-6, and vitamin B-12, but higher homoc ysteine 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 hav e adequate vitamin B-6 and B-12 status, but suboptimal folate states, leading to elevated plasma homocysteine levels. They may therefore ben efit from folate supplementation to reduce their high risk for endothe lial damage, (C) 1998 Wiley-Liss, Inc.