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	Full Text PDF Editorial by Cohen, A. R. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited Related Article by Sarnaik, S. A. PubMed Citation

ABSTRACT

Background Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions to prevent a first stroke.

Methods To enter the study, children with sickle cell anemia and no history of stroke had to have undergone two transcranial Doppler studies that showed that the time-averaged mean blood-flow velocity in the internal carotid or middle cerebral artery was 200 cm per second or higher. The patients were randomly assigned to receive standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of the total hemoglobin concentration. The incidence of stroke (cerebral infarction or intracranial hemorrhage) was compared between the two groups.

Results A total of 130 children (mean [±SD] age, 8.3±3.3 years) were enrolled; 63 were randomly assigned to receive transfusions, and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group — a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial.

Conclusions Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.

Source Information

From the Departments of Neurology (R.J.A., F.T.N.), Pediatric Hematology and Oncology (V.C.M.), and Medicine (A.K.), Medical College of Georgia, Augusta; the Sickle Cell Center, Emory University School of Medicine, Atlanta (L.H.); the Department of Pediatric Hematology and Oncology, East Carolina University School of Medicine, Greenville, N.C. (B.F.); the Department of Hematology and Oncology, Children's Hospital Oakland, Oakland, Calif. (E.V.); the Sickle Cell Center, University of Miami School of Medicine, Miami (C.P.); the Pediatric Sickle Cell Program, Medical University of South Carolina, Charleston (M.A.); the New England Research Institutes, Watertown, Mass. (D.G., D.B.); and the National Heart, Lung, and Blood Institute, Bethesda, Md. (D.R.B.). Other authors were Gerald Woods, M.D. (Department of Hematology and Oncology, Children's Mercy Hospital, Kansas City, Mo.), Nancy Olivieri, M.D. (Department of Hematology and Oncology, Hospital for Sick Children, Toronto), Catherine Driscoll, M.D. (Department of Hematology and Oncology, Children's National Medical Center, Washington, D.C.), Scott Miller (Pediatric Sickle Cell Program, State University of New York Health Science Center at Brooklyn, Brooklyn), Winfred Wang, M.D. (Department of Hematology and Oncology, St. Jude Children's Research Hospital, Memphis, Tenn.), Anne Hurlett, M.D. (Department of Pediatric Hematology, Columbia–Presbyterian Medical Center, New York), Charles Scher, M.D. (Department of Pediatric Hematology and Oncology, Tulane University Medical School, New Orleans), Brian Berman, M.D. (Department of Pediatric Hematology and Oncology, Rainbow Babies and Children's Hospital, Cleveland), Elizabeth Carl, B.A., and Anne M. Jones, R.N. (Department of Neurology, Medical College of Georgia, Augusta), E. Steve Roach, M.D. (Department of Pediatric Neurology, University of Texas Southwestern Medical Center, Dallas), Elizabeth Wright, Ph.D. (New England Research Institutes, Watertown, Mass.), Robert A. Zimmerman, M.D. (Department of Neuroradiology, Children's Hospital of Philadelphia, Philadelphia), and Myron Waclawiw, Ph.D. (National Heart, Lung, and Blood Institute, Bethesda, Md.).

Address reprint requests to Dr. Adams at the Department of Neurology, Medical College of Georgia, 1467 Harper St., HB-2060, Augusta, GA 30912-3200.

Full Text of this Article

Related Letters:

Prevention of Stroke by Transfusions in Children with Sickle Cell Anemia
Sarnaik S. A., Hsu L., Files B., Adams R. J.
Extract | Full Text  
N Engl J Med 1998; 339:1477-1478, Nov 12, 1998. Correspondence

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• Fullerton, H.J., Chetkovich, D.M., Wu, Y.W., Smith, W.S., Johnston, S.C. (2002). Deaths from stroke in US children, 1979 to 1998. Neurology 59: 34-39 [Abstract] [Full Text]  
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• Walters, M. C., Nienhuis, A. W., Vichinsky, E. (2002). Novel Therapeutic Approaches in Sickle Cell Disease. ASH Education Book 2002: 10-34 [Abstract] [Full Text]  
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• Pegelow, C. H., Wang, W., Granger, S., Hsu, L. L., Vichinsky, E., Moser, F. G., Bello, J., Zimmerman, R. A., Adams, R. J., Brambilla, D., for the STOP Trial, (2001). Silent Infarcts in Children With Sickle Cell Anemia and Abnormal Cerebral Artery Velocity. Arch Neurol 58: 2017-2021 [Abstract] [Full Text]  
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