Sickle cell disease (SCD) is an inherited disorder caused by a variant (rs334) in the β-globin gene encoding hemoglobin. Individuals with SCD are thought to be at risk of vitamin D deficiency. Our aim was to assess serum 25-hydroxyvitamin D (25OHD) concentrations, estimate deficiency prevalence, and investigate factors associated with 25OHD concentrations in children and adolescents with SCD attending BC Children's Hospital in Vancouver, Canada. We conducted a retrospective chart review of SCD patients (2-19 y) from 2012 to 2017. Data were available for n = 45 patients with n = 142 25OHD measurements assessed using a EUROIMMUN analyzer (EUROIMMUN Medizinische Labordiagnostika AG, Lübeck, Germany). Additional data were recorded, including age, sex, and season of blood collection. Linear regression was used to measure associations between 25OHD concentration and predictor variables. Overall, mean ± SD 25OHD concentration was 79 ± 36 nmol/L; prevalence of low 25OHD concentrations (<30, <40, and <75 nmol/L) was 5%, 17% and 50%, respectively. Mean 25OHD concentrations measured during Jul-Sep were higher (28 (95% confidence interval CI: 16-40) nmol/L higher, P < 0.001) compared to Jan-Mar. Vitamin D deficiency rates varied widely by season: Based on 25OHD <30 nmol/L, prevalence was 0% in Oct-Dec and 6% in Jan-Mar; based on <40 nmol/L, prevalence was 0% in Oct-Dec and 26% in Jan-Mar.
Keywords: 25-hydroxyvitamin D; deficiency; hemoglobinopathy; nutrition; pediatrics; sickle cell disease; vitamin D.