Year : 2013 | Volume : 4 | Issue : 2 | Page : 58--64
Evaluation of clinical severity of sickle cell anemia in Nigerian children
Samuel Ademola Adegoke, Bankole Peter Kuti
Department of Paediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria
Sickle cell anemia (SCA) in children exhibits wide clinical diversity. SCA«SQ»s variable and unpredictable phenotypic expressions pose significant management challenges to caregivers and physicians alike. We therefore introduced a scoring system to evaluate the clinical severity of SCA in Nigerian children using simple clinico-laboratory parameters, and related the severity to their sociodemographic, hematological, and biochemical profiles. The total severity score of the 115 children ranged from 1 to 24, with mean ± standard deviation of 9.85 ± 5.22. Approximately one-third, 33.9% had mild disease, 55.7% moderate disease, and 10.4% severe disease, according to our severity score grading system. Age was positively correlated with disease severity score (r = 0.61; 95% confidence interval [CI] = 2.1-3.7; P = 0.001). Also, presence of dactylitis/hand-foot syndrome at first presentation, total white blood cell count, absolute neutrophil count, platelet count, and serum bilirubin concentration were significantly higher among those with severe disease. Conversely, hemoglobin F (HbF) percentage was significantly higher in those with mild disease (5.4 ± 3.2% vs. 3.1 ± 1.3%; P = 0.001). On multivariate analysis, dactylitis/hand-foot syndrome as first manifestation (odd ratio [OR] = 0.4; 95% CI = 1.7-3.5; P = 0.043 and HbF% (OR = 0.6; 95% CI = 0.2-0.7; P = 0.013) were the only independent predictors of severe disease. Routine evaluation of disease severity in children with SCA will help to prospectively identify children at higher risk for a turbulent clinical course who may need more active management and monitoring.