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OBJECTIVE: To identify behavioral manifestations of executive dysfunction that are associated with cerebrovasculopathy, as measured by transcranial Doppler (TCD) ultrasonography, in children with sickle cell disease (HbSS). METHODS: Participants were 62 children and adolescents with HbSS disease who had no documented history of cerebrovascular accident. Children were classified according to the National Institutes of Health Stroke Prevention Trial in Sickle Cell Anemia (STOP) criteria (i.e., normal, conditional, and abnormal). RESULTS: Although children with abnormal TCD values were rated by their parents as exhibiting greater executive dysfunction in the areas of inhibitory control, problem-solving flexibility, and modulation of emotional responses compared with children in the conditional group, these differences were not clinically significant. In contrast, teachers rated children in the abnormal TCD group as having clinically significant executive dysfunction as manifested in their ability to solve problems in working memory, plan and organize, and self-monitor. CONCLUSIONS: Our findings lend preliminary support for the utility of teacher-completed screening instruments designed to assess everyday behaviors associated with executive dysfunction in children with HbSS disease, especially among those children at greatest risk for neurological impairment as identified by TCD ultrasonography.
Mary C. Kral (Thu,) studied this question.