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Blood, 15 November 2008, Vol. 112, No. 10, pp. 4314-4317. Prepublished online as a Blood First Edition Paper on September 4, 2008; DOI 10.1182/blood-2008-03-143891. This Article Full Text (PDF) Supplemental Appendix All Versions of this Article: blood-2008-03-143891v1 112/10/4314    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Bernaudin, F. Articles by Delacourt, C. Search for Related Content PubMed PubMed Citation Articles by Bernaudin, F. Articles by Delacourt, C. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted March 6, 2008 Accepted August 12, 2008 G6PD deficiency, absence of alpha-thalassemia and hemolytic rate at baseline are significant independent risk factors for abnormally high cerebral velocities in patients with sickle cell anemia Francoise Bernaudin*, Suzanne Verlhac, Sylvie Chevret, Martine Torres, Lena Coic, Cecile Arnaud, Annie Kamdem, Isabelle Hau, Maria Grazia Neonato, and Christophe Delacourt Reference Center for Sickle Cell Disease, Department of Pediatrics, Intercommunal Creteil Hospital, Creteil, France Medical Imagerie, Department of Radiology, Intercommunal Creteil Hospital, Creteil, France Department of Statistics, Saint-Louis Hospital, Paris, France Pediatrics, University of Southern California, Los Angeles, United States Hematology, Molecular Biology, Tenon Hospital, Paris, France * Corresponding author; email: francoise.bernaudin{at}chicreteil.fr. Stroke is predicted by abnormally high cerebral velocities by transcranial doppler (TCD). This study aimed at defining predictive factors for abnormally high velocities ( 2m/sec) based on the Creteil pediatric sickle cell anemia cohort composed of 373 stroke-free SS children. Alpha genes and beta-globin haplotypes were determined. Biological parameters were obtained at baseline. Alpha-thalassemia was present in 155/325 and G6PD deficiency in 36/325 evaluated patients. TCD was abnormal in 62 of the 373 patients. Multivariate logistic regression analysis showed that G6PD deficiency [OR=3.36, 95%CI (1.10-10.33)], (p=0.034); absence of alpha-thalassemia [OR= 6.45, 95%CI (2.21-18.87)], (p=0.001); hemoglobin [OR per g/dl = 0.63, 95%CI (0.41-0.97)], (p=0.038); and LDH levels [OR per IU/l= 1.001, 95%CI (1.000-1.002)], (p=0.047) were independent risk factors for abnormally high velocities. This study confirms the protective effect of alpha-thalassemia, and shows for the first time that G6PD deficiency and hemolysis independently increase the risk of cerebral vasculopathy CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia David C. Rees, Christopher Lambert, Elaine Cooper, Jack Bartram, David Goss, Colin Deane, and Swee Lay Thein Blood 2009 114: 742-743. [Full Text] [PDF] This article has been cited by other articles: D. C. Rees, C. Lambert, E. Cooper, J. Bartram, D. Goss, C. Deane, and S. L. Thein Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia Blood, July 16, 2009; 114(3): 742 - 743. [Full Text] [PDF]

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