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Pediatric Imaging |
1 From the Departments of Diagnostic Imaging (R.G.S., G.M.H., K.B., J.W.L., K.J.H.), Epidemiology and Biostatistics (X.X.), and Hematology (W.C.W.), St Jude Childrens Research Hospital, 332 N Lauderdale St, Memphis, TN 38105-2794; and Departments of Pediatrics (R.G.S., W.C.W.) and Radiology (R.G.S, J.W.L., K.J.H.), University of Tennessee School of Medicine, Memphis. Received May 20, 2002; revision requested July 16; final revision received October 9; accepted November 5. Supported by the American Lebanese Syrian Associated Charities (ALSAC). R.G.S. supported by grant HL 60022 from the National Institutes of Health. Address correspondence to R.G.S. (e-mail: grant.steen@stjude.org).
PURPOSE: To determine whether sickle cell trait (hemoglobin AS) is associated with abnormalities in the brain of asymptomatic children.
MATERIALS AND METHODS: Magnetic resonance (MR) imaging and MR angiography were performed prospectively in 26 siblings (eight girls, 18 boys; mean age, 10.5 years) of patients with sickle cell disease. Two neuroradiologists, blinded as to whether a child had hemoglobin AS or AA, reviewed images obtained in siblings. With MR imaging, lacunae, loss of white matter volume, encephalomalacia, or leukoencephalopathy was identified. With MR angiography, arterial stenosis, occlusion, or tortuosity was identified. Images with definite or possible abnormalities were mixed with randomly selected images and were referred to a third neuroradiologist for a completely blinded review. In cases in which all neuroradiologists concurred, a score was assigned that indicated the sibling had an abnormality. MR angiographic findings were assigned a score for tortuosity with a new quantitative scale.
RESULTS: Among 26 siblings screened, 21 children had sickle cell trait. Among these 21 children, two had mild abnormalities at MR imaging (sample prevalence rate, 10% [95% CI: 1%, 29%]), and four had arterial tortuosity (sample prevalence rate, 19% [95% CI: 5%, 42%]). When children with sickle cell trait were compared with 31 control subjects without the trait, arterial tortuosity was significantly more common in children with sickle cell trait (P = .014). Among children with sickle cell trait, percentage of hemoglobin S was significantly greater in children who had tortuosity than percentage of hemoglobin S in children who had normal blood vessels at MR angiography (P < .03).
CONCLUSION: Findings suggest that greater percentage of hemoglobin S is associated with mild vasculopathy. This vasculopathy may explain some of the excess risk of stroke among African Americans.
© RSNA, 2003
Index terms: Brain, MR, 17.121411 Cerebral angiography, 17.12142 Children, central nervous system Magnetic resonance (MR), vascular studies, 17.12142 Sickle cell disease (SS, SC), 10.651, 17.651
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