Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study.
OBJECTIVE
Population-based surveillance of children aged 3-10 years from metropolitan Atlanta was used to determine if stroke-related neurological damage in children with sickle cell disease (SCD) is associated with developmental disabilities (DD).


METHODS
School and medical records were reviewed annually to identify eligible children. Observed-to-expected ratios, P values, and population attributable fractions were calculated.


RESULTS
Children with SCD had increased risk for DD (O/E = 3.2, P < 0.0001), particularly mental retardation (O/E = 2.7, P = 0.0005) and cerebral palsy (O/E = 10.8, P < 0.0001). This risk was confined to DD associated with stroke (O/E = 130, P < 0.0001; for DD without stroke: O/E = 1.3, P = 0.23).


CONCLUSIONS
Children with SCD have increased risk for DD associated with stroke; thus, aggressive interventions are needed to prevent stroke in these children.
Ashley-Koch
A
A
Office of Genetics and Disease Prevention, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
Murphy
C C
CC
Khoury
M J
MJ
Boyle
C A
CA
D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics; D044383:Black People; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology* / Q000235:genetics; D005260:Female; D005828:Genetics, Population; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology / Q000235:genetics; D008297:Male; D012307:Risk Factors