title: Cognitive deficits in children with sickle cell disease.
abstract: We tested a hypothesis that children with sickle cell disease who are completely normal by magnetic resonance imaging can still be cognitively impaired, as predicted by a model of diffuse brain injury. Fifty-four patients with hemoglobin SS (average age 10.9 years +/- 2.9 years SD) were examined with the Wechsler Intelligence Scale for Children-III (WISC-III) and were randomly matched by age, race, and gender with healthy children from the Wechsler normative database. Patients were also imaged at 1.5 Tesla with standard imaging sequences. Among 30 patients who were normal by magnetic resonance imaging, there were substantial deficits in Wechsler Full-Scale IQ, Verbal IQ, and Performance IQ (all P < .01) compared with African-American controls. The patient Wechsler Full-Scale IQ was 12.9 points lower than that of controls and decreased as a function of age (probability = .014). The findings suggest that there is diffuse brain injury in patients and that patient deficits increase with age.
authors.affiliation: Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, TN, USA. grant_steen@med.unc.edu
authors.forename: R Grant
authors.initials: RG
authors.lastname: Steen
authors.forename: Charlotte
authors.initials: C
authors.lastname: Fineberg-Buchner
authors.forename: Gisele
authors.initials: G
authors.lastname: Hankins
authors.forename: Larry
authors.initials: L
authors.lastname: Weiss
authors.forename: Aurelio
authors.initials: A
authors.lastname: Prifitera
authors.forename: Raymond K
authors.initials: RK
authors.lastname: Mulhern
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology; D001921:Brain / Q000473:pathology; D016022:Case-Control Studies; D002648:Child; D003072:Cognition Disorders / Q000209:etiology* / Q000473:pathology; D004522:Educational Status; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010290:Parents; D011446:Prospective Studies; D014888:Wechsler Scales