Journal of Applied Hematology

ORIGINAL ARTICLE
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

Correspondence Address:
Samuel Ademola Adegoke
Department of Paediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Osun State
Nigeria

Abstract

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.



How to cite this article:
Adegoke SA, Kuti BP. Evaluation of clinical severity of sickle cell anemia in Nigerian children.J Appl Hematol 2013;4:58-64


How to cite this URL:
Adegoke SA, Kuti BP. Evaluation of clinical severity of sickle cell anemia in Nigerian children. J Appl Hematol [serial online] 2013 [cited 2019 Sep 18 ];4:58-64
Available from: http://www.jahjournal.org/text.asp?2013/4/2/58/134928


Full Text

.