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.
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,,,
From the * Department of Pediatrics, Duke Children's Hospital,
Duke University Medical Center, Durham, North Carolina; New England
Research Institutes, Inc, Watertown, Massachusetts; § St Jude's
Children's Research Hospital, Department of Hematology/Oncology,
Memphis, Tennessee; 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; 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
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
-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
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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