Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke

Douglas J. Scothorn, Cynthia Price, Daniel Schwartz, Cindy Terrill, George R. Buchanan, Wanda Shurney, Ingrid Sarniak, Robert Fallon, Jen Yih Chu, Charles H. Pegelow, Winfred Wang, James F. Casella, Linda S. Resar, Brian Berman, Thomas Adamkiewicz, Lewis L. Hsu, Kwaku Ohene-Frempong, Kim Smith-Whitley, Donald Mahoney, J. Paul ScottGerald M. Woods, Masayo Watanabe, Michael R. DeBaun

Research output: Contribution to journalArticle

172 Scopus citations


Objective: To test the hypothesis that children with sickle cell disease (SCD) who have an initial stroke temporally unrelated to another medical event are at higher risk for recurrent stroke than are children who had strokes temporally related to medical events. Methods: A retrospective cohort study of children with SCD and stroke who received regularly scheduled blood transfusions for a minimum of 5 years was conducted. Medical records were examined for the documentation of antecedent or concurrent medical events (hypertension, acute chest syndrome, aplastic crisis, fever associated with infection, exchange transfusion) associated with physician contact within 14 days before the initial stroke. Results: A total of 137 pediatric patients from 14 centers were studied. Mean age at first stroke was 6.3 years (1.4 to 14.0 years) with mean follow-up of 10.1 years (5 to 24 years). Thirty-one (22%) patients had a second stroke (2.2 per 100 patient years); 26 patients had an identified medical or concurrent event associated with their initial stroke. None of these patients had recurrent stroke 2 or more years after the initial event. The remaining 111 patients had an ongoing risk of recurrent stroke (1.9 per 100 patient-years) despite long-term transfusions (P = .038). Conclusions: The absence of an antecedent or concurrent medical event associated with an initial stroke is a major risk factor for subsequent stroke while receiving regular transfusions.

Original languageEnglish (US)
Pages (from-to)348-354
Number of pages7
JournalJournal of Pediatrics
Issue number3
StatePublished - Jan 1 2002


ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Scothorn, D. J., Price, C., Schwartz, D., Terrill, C., Buchanan, G. R., Shurney, W., Sarniak, I., Fallon, R., Chu, J. Y., Pegelow, C. H., Wang, W., Casella, J. F., Resar, L. S., Berman, B., Adamkiewicz, T., Hsu, L. L., Ohene-Frempong, K., Smith-Whitley, K., Mahoney, D., ... DeBaun, M. R. (2002). Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. Journal of Pediatrics, 140(3), 348-354. https://doi.org/10.1067/mpd.2002.122498