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

Purpose: A sensitive inverse relationship between plasma homocysteine concentration and folate status has been demonstrated. Although children with sickle cell anemia (SCA) are at potential risk for folate deficiency, plasma homocysteine levels have not been reported in such patients. Therefore, a study was designed to assess plasma homocysteine levels as a marker of folate status. Design: Plasma homocysteine concentrations were measured in 120 children with SCA (102 in steady state and 18 during an acute complication) who had never received supplemental folic acid. Folate status was directly assessed in 34 of these patients. Results: Plasma homocysteine levels in the patients with SCA and control subjects were similar. The mean value ± 1 SD was 5.8 ± 2.5 μmol/L (range, 1.6 to 14.1 μmol/L) in the patients with SCA and 6.1 ± 2.7 μmol/L (range, 1.7 to 15.3 μmol/L) in 73 pediatric control subjects. In a subpopulation of the study group (34 children), simultaneous serum folate, red cell 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 concentration with various clinical or laboratory measures or with red cell folate concentration. Conclusion: Folate stores in children with SCA not receiving folic acid supplements are adequate despite an underlying hemolytic anemia.