Hemoglobin sickle cell disease: Experience of the Yalgado Ouedraogo University Hospital of Ouagadougou, Burkina Faso

Objectives. To evaluate the clinical features of children with hemoglobin sickle cell disease (HbSC) and compare them to children with sickle cell anemia (HbSS). Population and methods. This was a descriptive and retrospective study. New patients with sickle cell disease who consulted at the Yalgado Ouedraogo University Hospital's Pediatric Center in Ouagadougou, Burkina Faso, between May 2005 and June 2006, were included. They were free of any major disease unrelated to sickle cell disease. Clinical and laboratory results reported for these children were based on their health book and medical records. Results. Sixty-one children were included in the study, 38 and 23 children were positive for HbSC and HbSS, respectively; there was no significant difference between the 2 groups in terms of sex ratio or mean age at inclusion. Mean age at diagnosis was 5 years and 2 years for HbSC and HbSS children, respectively. The first clinical event. appeared at a significantly later age for HbSC than HbSS children (4 years versus 2 years). Painful episodes were equivalent in mean number per year and mean length per episode between the 2 groups; the median hemoglobin (Hb) level at inclusion was significantly higher for HbSC than for HbSS children, i.e., 95 g/l versus 70 g/l. Conclusion. At the Yalgado Ouedraogo University Hospital Pediatric Center, children with HbSC disease presented clinical and biological features very similar to those with HbSS. (c) 2009 Elsevier Masson SAS. All rights reserved.