Clinical Observations, Plasma Retinol Concentrations, And In Vitro Lymphocyte Functions In Children With Sickle Cell Disease

Background: Children with sickle cell disease (SCD) often have infections, growth deficits, and impaired immunity, problems that also are observed in individuals with a vitamin A deficiency (plasma retinol concentration <20 mu g/dL). The goal of this study was to investigate the association between vitamin A, health status, and the in vitro immune function of children with SCD.Methods: Fifty-nine children (40 SS, 11 SC, and 8 S beta(thalassemia )[S beta(thal)] hemoglobin genotypes) 9 months to 18 years old were investigated for plasma levels of retinol, retinol binding protein, C-reactive protein, alpha-1-acid glycoprotein, lymphocyte proliferation, and interleukin (IL)-2 activity in supernatant of phytohemagglutinin-treated lymphocytes.Results: The plasma retinol concentrations of children with SCD (mean 57.6 mu g/dL, range 4.6-116 mu g/dL) were not different from those of 21 normal individuals (mean 62 mu g/dL, range 28.7-162 mu g/dL). Plasma retinol concentrations did not vary by hemoglobin genotype but were lower in boys than in girls (P<0.05) and were also lower in children with inflammation (P=0.1). Seven children (11.9%) (6 HbSS, 1 HbS beta(Othal) ) were vitamin A-deficient, and 9 children (15.3%) had suboptimal vitamin A status (plasma retinol concentration of 20-29 mu g/dL). Children with vitamin A deficiency had slightly lower height (P=0.09) and weight mean percentiles, lymphocyte proliferative responses, and IL-2 activity (P>0.1), but higher means of C-reactive protein (P=0.05), pain crisis episodes and inflammation (P=0.1), and health scores (P>0.1) than children who were not vitamin A-deficient. Lymphocyte proliferative responses negatively correlated with health score, pain crisis episodes, and blood units received, but positively correlated with retinol binding protein (P<0.05 to P=0.1).Conclusion: Identification and correction of suboptimal vitamin A status in children with SCD may improve immunity and attenuate certain health complications associated with this disease.