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Silent Infarcts in Children With Sickle Cell Anemia and Abnormal Cerebral Artery Velocity
Charles H. Pegelow, MD;
Winfred Wang, MD;
Suzanne Granger, MS;
Lewis L. Hsu, MD;
Elliott Vichinsky, MD;
Franklin G. Moser, MD;
Jacqueline Bello, MD;
Robert A. Zimmerman, MD;
Robert J. Adams, MD;
Donald Brambilla, PhD;
for the STOP Trial
Arch Neurol. 2001;58:2017-2021.
Background A substantial minority of neurologically normal children with sickle cell disease have lesions consistent with cerebral infarction as seen on magnetic resonance imaging (MRI).
Objectives To determine if transfusion therapy affects the rate at which silent infarcts develop and to evaluate the contribution of MRI of the brain to stroke prediction by transcranial Doppler (TCD) ultrasonography.
Study Design Children with elevated TCD ultrasonographic velocity were randomized to receive long-term transfusion therapy or standard care. Magnetic resonance imaging of the brain was obtained at randomization, annually, and with clinical neurologic events. The risk for new silent lesions and/or stroke was compared for each treatment arm.
Results Among the 37% of subjects with silent infarcts, those receiving standard care were significantly more likely to develop new silent lesions or stroke than were those who received transfusion therapy. For subjects receiving standard care, those with lesions at baseline were significantly more likely to develop stroke or new silent lesions than those whose MRI studies showed no abnormality.
Conclusions Transfusion therapy lowers the risk for new silent infarct or stroke for children having both abnormal TCD ultrasonographic velocity and silent infarct. However, those with both abnormalities who are not provided transfusion therapy are at higher risk for developing a new silent infarct or stroke than are those whose initial MRI showed no abnormality. The finding of a silent infarct reinforces the need for TCD ultrasonographic screening and consideration of transfusion therapy if the abnormalities are seen. Similarly, elevated TCD ultrasonographic velocity warrants MRI of the brain because children with both abnormalities seem to be at increased risk for developing new silent infarct or stroke.
From the Department of Pediatrics, University of Miami, Miami, Fla (Dr Pegelow); St Jude Children's Research Hospital, Memphis, Tenn (Dr Wang); New England Research Institutes, Watertown, Mass (Ms Granger and Dr Brambilla); Emory University, Atlanta, Ga (Dr Hsu); Children's Hospital, Oakland, Calif (Dr Vichinsky); Cedars-Sinai Medical Center, Los Angeles, Calif (Dr Moser); Montefiore Medical Center, Bronx, NY (Dr Bello); Children's Hospital of Philadelphia, Philadelphia, Pa (Dr Zimmerman); and Medical College of Georgia, Augusta (Dr Adams).
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