ABSTRACT
PURPOSE: The goal of the study was to assess development in young children with sickle cell disease as part of the Cooperative Study of Sickle Cell Disease (CSSCD). PATIENTS AND METHODS: The Denver Developmental Screening Test (DDST) was administered to children younger than 6 years at 12 participating institutions of the CSSCD. Trained examiners administered tests to 344 children. RESULTS: Tests were scored as normal in 90.4%, questionable in 6.4%, and abnormal in 1.5%; 1.7% of children were considered untestable. There was no relationship between DDST results and sickle cell genotype. Questionable and abnormal (Q/A) scores were more common in children ages 3-5 years than in younger children (12.6% versus 3.8%; P = 0.002). CONCLUSIONS: Because the DDST is a screening test, it should be interpreted cautiously. However, the more numerous Q/A scores in our "older" group agree with the findings of recent reports of neuropsychological impairment in school-age children with sickle cell disease. Our data suggest that development is relatively normal before age 3 years; deficits seen in older children may reflect subsequent ischemic insults.
Subject(s)
Anemia, Sickle Cell/psychology , Developmental Disabilities/etiology , Mass Screening , Psychomotor Performance , Age Factors , Anemia, Sickle Cell/genetics , Brain Ischemia/etiology , Brain Ischemia/psychology , Child, Preschool , Female , Genotype , Humans , Infant , Intelligence , Male , Neuropsychological Tests , Sickle Cell Trait/genetics , Sickle Cell Trait/psychology , Thalassemia/genetics , Thalassemia/psychologyABSTRACT
The Denver Prescreening Developmental Questionnaire (PDQ), a parent-answered questionnaire, has been revised to extend the age of children who can be screened to those from 6 years down to birth, to make the test items more challenging for children and more informative for parents, and to make it easier for professionals to compare a child's performance with Denver Developmental Screening Test (DDST) norms. This Revised PDQ (R-PDQ) identified 84% of nonnormal DDST results. Test-retest reliability over 1 week was 94.1%, and parent-teacher agreement was 83%. A field test of the R-PDQ, conducted in a variety of settings with 1434 children, revealed that suspect scores on first-stage screening varied with the setting, from 15.6% in private pediatric practices to 50.5% in Head Start and urban day care centers. Parents found the R-PDQ interesting and easy to complete; health professionals found it economical and easy to interpret. We recommend that the R-PDQ be coupled with a brief developmental examination, and that the R-PDQ be used in busy office settings and community mass screening programs.
