Prevalence of sickle cell disease in a pediatric population suffering from severe infections: a Congolese experience

Neonatal screening for sickle cell anemia is not a common practice in the Democratic Republic of Congo (DRC). Children with sickle cell disease are known to have an increased risk of infections. We conducted a pilot study to determine the prevalence of sickle cell anemia during episodes of severe infection. A prospective study was conducted from July 2009 to July 2011. The study sites included four public hospitals at Kinshasa, DRC. The study population was selected from the source population using three-stage sampling. A total of 247 children with severe infection were consecutively recruited and screened for sickle cell disease. There were 124 boys (50.2%) and 123 girls (49.8%) with a sex-ratio of 1:1. More than two-thirds of patients (66.0%) were children between 1 and 24 months of age. Among these 247 children, 19 (7.7%) were homozygous sickle cell anemia patients (Hb SS). No patient had received Hemophilus influenzae, streptococcus pneumoniae and salmonella sp vaccines. Sepsis was the most common form of severe infection observed in 44.5% of patients. A total of 19 (7.7%) positive blood cultures were recorded. Most cases were reported in sickle cell patients (15.8%) compared to 6.1% in children who were negative for Hb S [β6(A3)Glu→Val; HBB: c.20A>T] (p > 0.05). Of 247 children with severe infection, approximately 8.0% carried unknown sickle cell anemia mutations. Based on the findings in this study, opportunistic testing for sickle cell anemia is possible and worthwhile in children who present with severe infection in DRC until neonatal screening is universal.