Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease

Blood - Tập 117 - Trang 772-779 - 2011
Monica L. Hulbert1, Robert C. McKinstry2,3, JoAnne L. Lacey2, Christopher J. Moran2, Julie A. Panepinto4, Alexis A. Thompson5, Sharada A. Sarnaik6, Gerald M. Woods7, James F. Casella8, Baba Inusa9, Jo Howard9, Fenella J. Kirkham10, Kofi A. Anie11, Jonathan E. Mullin12, Rebecca Ichord13, Michael Noetzel3,14, Yan Yan3, Mark Rodeghier15, Michael R. DeBaun16
1Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN
2Department of Radiology, Washington University School of Medicine, St Louis, MO
3Department of Pediatrics, Washington University School of Medicine, St. Louis, MO
4Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
5Department of Pediatrics, Northwestern University School of Medicine, Chicago, IL;
6Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI
7Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, MO
8Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD
9Guy's and St Thomas's National Health Service Foundation Trust, London, United Kingdom;
10University College Institute of Child Health, London, United Kingdom;
11Imperial College School of Medicine, Central Middlesex Hospital, London, United Kingdom;
12Case Western Reserve University School of Medicine, Cleveland, OH
13Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA
14Department of Neurology, Washington University School of Medicine, St. Louis, MO
15Statistical Collaborator, Chicago, IL; and
16Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN

Tóm tắt

Abstract

Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.


Tài liệu tham khảo

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