|
 Previous Article | Table of Contents | Next Article 
Blood, 15 March 2003, Vol. 101, No. 6, pp. 2401-2404
RED CELLS
Stroke risk in siblings with sickle cell anemia
M. Catherine Driscoll,
Anne Hurlet,
Lori Styles,
Virgil McKie,
Beatrice Files,
Nancy Olivieri,
Charles Pegelow,
Brian Berman,
Richard Drachtman,
Kantilal Patel, and
Donald Brambilla
From the Department of Pediatrics, The George
Washington University School of Medicine, Washington, DC; Children's
National Medical Center, Department of Hematology-Oncology,
Washington, DC; Department of Pediatrics, Columbia University College
of Physicians and Surgeons, Division of Hematology, New York, NY;
Children's Hospital Oakland, Department of Hematology-Oncology,
Oakland, CA; Department of Pediatrics, Medical College of Georgia,
Division of Hematology-Oncology, Augusta, GA; Department of Pediatrics,
East Carolina University School of Medicine, Division of
Hematology-Oncology, Greenville, NC; Department of Medicine, University
of Toronto, Hospital for Sick Children, Toronto, ON,
Canada; Department of Pediatrics, University of Miami,
Division of Hematology-Oncology, Miami, FL; Department of Pediatrics,
Rainbow Babies and Children's Hospital, Case Western School of
Medicine, Cleveland, OH; Department of Pediatrics, University of
Medicine and Dentistry of New Jersey, Division of Hematolgy-Oncology,
New Brunswick, NJ; New England Research Institutes, Watertown, MA.
Cerebrovascular disease is a common cause of morbidity in sickle
cell anemia (HbSS): approximately 10% of patients have a clinical stroke before 20 years of age, and another 22% have silent infarction on magnetic resonance imaging. The phenotypic
variation among patients with HbSS suggests a role for modifier genes
and/or environmental influences. To assess the familial component of clinical stroke in HbSS, we estimated the prevalence of clinical stroke
among all patients and among HbSS sibling pairs at 9 pediatric centers. The sample included 3425 patients with sickle cell
disease who were younger than 21 years, including 2353 patients with
HbSS. The stroke prevalence was 4.9% for all genotypes; 7.1% for
patients with HbSS; 1.1% for patients with HbS o
thalassemia; 0.6% for patients with S+ thalassemia; and
0% for patients with HbSC. In 207 sibships, more than 1 child
had HbSS. There were 42 sibships in which at least 1 sibling
had a stroke, and in 10 of the 42, 2 siblings had a stroke. A
permutation test indicated that the number of families in which 2 children had strokes was larger than the number expected if strokes
were randomly distributed among children in sibships (P = .0012). There was no difference in stroke prevalence based on sex, nor
was the mean age at stroke presentation significantly different between
singletons and sibships with stroke. We conclude that there is a
familial predisposition to stroke in HbSS. Attempts to identify genetic
modifiers should be initiated with family-based studies.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
L. Chang Milbauer, P. Wei, J. Enenstein, A. Jiang, C. A. Hillery, J. P. Scott, S. C. Nelson, V. Bodempudi, J. N. Topper, R.-B. Yang, et al.
Genetic endothelial systems biology of sickle stroke risk
Blood,
April 1, 2008;
111(7):
3872 - 3879.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Hoppe, W. Klitz, K. D'Harlingue, S. Cheng, M. Grow, L. Steiner, J. Noble, R. Adams, L. Styles, and for the Stroke Prevention Trial in Sickle Cell Ane
Confirmation of an Association Between the TNF( 308) Promoter Polymorphism and Stroke Risk in Children With Sickle Cell Anemia
Stroke,
August 1, 2007;
38(8):
2241 - 2246.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. A. Manci, C. A. Hillery, C. A. Bodian, Z. G. Zhang, G. A. Lutty, and B. S. Coller
Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease
Blood,
February 15, 2006;
107(4):
1651 - 1658.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. D. Hurn, S. J. Vannucci, and H. Hagberg
Adult or Perinatal Brain Injury: Does Sex Matter?
Stroke,
February 1, 2005;
36(2):
193 - 195.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. J. Adams, D. J. Brambilla, S. Granger, D. Gallagher, E. Vichinsky, M. R. Abboud, C. H. Pegelow, G. Woods, E. M. Rohde, F. T. Nichols, et al.
Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study
Blood,
May 15, 2004;
103(10):
3689 - 3694.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Hoppe, W. Klitz, S. Cheng, R. Apple, L. Steiner, L. Robles, T. Girard, E. Vichinsky, and L. Styles
Gene interactions and stroke risk in children with sickle cell anemia
Blood,
March 15, 2004;
103(6):
2391 - 2396.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. R. Buchanan, M. R. DeBaun, C. T. Quinn, and M. H. Steinberg
Sickle Cell Disease
Hematology,
January 1, 2004;
2004(1):
35 - 47.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
