Plasma homocysteine levels and folate status in children with sickle cell anemia

Purpose: A sensitive inverse relationship between plasma homocysteine conce ntration and folate status has been demonstrated. Although children with si ckle cell anemia (SCA) are at potential risk for folate deficiency, plasma homocysteine levels have not been reported in such patients. Therefore, a s tudy was designed to assess plasma homocysteine levels as a marker of folat e status. Design: Plasma homocysteine concentrations were measured in 120 children wi th SCA (102 in steady state and 18 during an acute complication) who had ne ver received supplemental folic acid. Folate status was directly assessed i n 34 of these patients. Results: Plasma homocysteine levels in the patients with SCA and control su bjects were similar. The mean value +/- 1 SD was 5.8 +/- 2.5 mu mol/L (rang e, 1.6 to 14.1 mu mol/L) in the patients with SCA and 6.1 +/- 2.7 mu mol/L (range, 1.7 to 15.3 mu mol/L) in 73 pediatric control subjects. In a subpop ulation of the study group (34 children), simultaneous serum folate, red ce ll folate, and total homocysteine concentrations were also measured. Their serum folate and red cell folate concentrations were normal: 12.4 +/- 10.0 nmol/L (range, 1 to 42 nmol/L) and 604 +/- 374.7 nmol/L (range, 205 to 1741 nmol/L), respectively. There was no correlation of plasma homocysteine con centration with various clinical or laboratory measures or with red cell fo late concentration. Conclusion: Folate stores in children with SCA not receiving folic acid sup plements are adequate despite an underlying hemolytic anemia.