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PEDIATRICS Vol. 103 No. 3 March 1999, pp. 640-645

Silent Cerebral Infarcts in Sickle Cell Anemia: A Risk Factor Analysis

Received Apr 6, 1998; accepted Aug 21, 1998.

Thomas R. Kinney*, Lynn A. SleeperDagger , Winfred C. Wang§, Robert A. Zimmermanparallel , Charles H. Pegelow, Kwaku Ohene-Frempong#, Doris L. Wethers**, Jacqueline A. BelloDagger Dagger , Elliott P. Vichinsky§§, Franklin G. Moser||, Dianne M. GallagherDagger , Michael R. DeBaun¶¶, Orah S. Platt##, Scott T. Miller***, and for the Cooperative Study of Sickle Cell Disease

From the * Department of Pediatrics, Duke Children's Hospital, Duke University Medical Center, Durham, North Carolina; Dagger  New England Research Institutes, Inc, Watertown, Massachusetts; § St Jude's Children's Research Hospital, Department of Hematology/Oncology, Memphis, Tennessee; parallel  Department of Neuroradiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania;  Division of Pediatric Hematology, University of Miami, Miami, Florida; # Department of Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; ** Comprehensive Sickle Cell Center, St Luke's/Roosevelt Hospital, New York, New York; Dagger Dagger  Division of Neuroradiology, Montefiore Medical Center, Bronx, New York; §§ Department of Hematology/Oncology, Children's Hospital Medical Center of Northern California, Oakland, California; || Department of Imaging, Cedars-Sinai Medical Center, Los Angeles, California; ¶¶ Department of Pediatric Hematology/Oncology, St Louis Children's Hospital, Missouri; ## Department of Laboratory Medicine, Boston Children's Hospital, Boston, Massachusetts; and *** Pediatric Sickle Cell Program, SUNY Health Science Center at Brooklyn, New York.

Background.  Silent infarcts have been reported in 17% of young patients with sickle cell disease and are associated with impaired performance on standardized psychometric tests. Risk factors for the development of these lesions have not been identified.

Methods.  Investigators in the Cooperative Study of Sickle Cell Disease performed a brain magnetic resonance imaging scan on sickle cell anemia patients age 5.9 years and older who had been followed according to the protocols of the Cooperative Study since birth. Individuals with a known history of cerebrovascular accident were excluded from this analysis. Patients with and without silent infarctions were compared with regard to clinical and laboratory parameters.

Results.  The study sample included 42 patients (18.3%) with silent infarcts. Patients who had silent infarcts were significantly more likely to have a clinical history of seizure and a lower painful event rate. Lower hemoglobin level, increased leukocyte count, elevated pocked red blood cell count, and SEN beta S globin gene haplotype were associated also with the presence of silent infarcts. There was no relationship between silent infarcts and platelet count, fetal hemoglobin level, reticulocyte percentage, serum aspartate aminotransferase level, total bilirubin concentration, blood pressure, growth parameters, or presence of alpha -thalassemia. A multivariate model for silent infarction identified the following as risk factors: low pain event rate, history of seizure, leukocyte count >= 11.8 × 109/L, and the SEN beta S globin gene haplotype.

Conclusions.  Patients with risk factors for silent infarcts should be evaluated for cerebrovascular disease. If evidence of infarction is found, consideration must be given to therapeutic intervention. At present, the appropriate treatment has not been determined.  Key words:  sickle cell disease, cerebral infarction.


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