L King1, M Reid, T E Forrester. 1. Sickle Cell Unit, Tropical Medicine Research Institute, The University of the West Indies, Kingston, Jamaica.
Abstract
OBJECTIVE: The aim of this study was to determine, using a combination of measures, the prevalence of iron deficiency anaemia (IDA) in children under five years-of-age who have sickle cell disease (SCD) and attend the Sickle Cell Clinic (SCU) of the Tropical Medicine Research Institute. MATERIALS AND METHODS: Children with homozygous sickle cell anaemia (Hb SS) or doubly heterozygous for Hb S and Hb C (Hb SC) disease who had not received a blood transfusion within three months prior to the iron measurements, were enrolled. The diagnosis of IDA was made if transferrin saturation was less than 16% with serum iron less than 10.7 micromol/l and a low mean corpuscular volume (MCV) for age. RESULTS: Twelve children (8.5%), seven with Hb SS and five with Hb SC had IDA. Adjusting for genotype, children with IDA had significantly higher red blood cell (RBC) counts (4.3 x10(9)/l vs 3.0 x 10(9)/l, p < 0.001) and total iron binding capacity (TIBC) (65.6 micromol/l vs 55.2 micromol/l, p < 0.004) but significantly lower reticulocyte (retic) counts (7.8 % vs 12.2%, p = 0.001) than children without IDA. CONCLUSION: Iron deficiency anaemia is a clinical problem which affects children with SCD in Jamaica. The higher RBC counts in the IDA group may be due to decreased haemolysis and increased red cell survival whilst the lower reticulocyte counts may be due to impaired erythropoiesis. These observations need to be extended by clinical studies to establish improved diagnostic measures for IDA in SCD. Additionally, clinical trials are needed to determine whether treatment of IDA in children with SCD reduces morbidity and is associated with clinical benefits such as improvements in neurocognitive function.
OBJECTIVE: The aim of this study was to determine, using a combination of measures, the prevalence of iron deficiency anaemia (IDA) in children under five years-of-age who have sickle cell disease (SCD) and attend the Sickle Cell Clinic (SCU) of the Tropical Medicine Research Institute. MATERIALS AND METHODS:Children with homozygous sickle cell anaemia (Hb SS) or doubly heterozygous for Hb S and Hb C (Hb SC) disease who had not received a blood transfusion within three months prior to the iron measurements, were enrolled. The diagnosis of IDA was made if transferrin saturation was less than 16% with serum iron less than 10.7 micromol/l and a low mean corpuscular volume (MCV) for age. RESULTS: Twelve children (8.5%), seven with Hb SS and five with Hb SC had IDA. Adjusting for genotype, children with IDA had significantly higher red blood cell (RBC) counts (4.3 x10(9)/l vs 3.0 x 10(9)/l, p < 0.001) and total iron binding capacity (TIBC) (65.6 micromol/l vs 55.2 micromol/l, p < 0.004) but significantly lower reticulocyte (retic) counts (7.8 % vs 12.2%, p = 0.001) than children without IDA. CONCLUSION:Iron deficiency anaemia is a clinical problem which affects children with SCD in Jamaica. The higher RBC counts in the IDA group may be due to decreased haemolysis and increased red cell survival whilst the lower reticulocyte counts may be due to impaired erythropoiesis. These observations need to be extended by clinical studies to establish improved diagnostic measures for IDA in SCD. Additionally, clinical trials are needed to determine whether treatment of IDA in children with SCD reduces morbidity and is associated with clinical benefits such as improvements in neurocognitive function.