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Blood, 1 January 2007, Vol. 109, No. 1, pp. 40-45. Prepublished online as a Blood First Edition Paper on August 29, 2006; DOI 10.1182/blood-2006-02-005082.
Submitted February 27, 2006
UT Southwestern Medical Center at Dallas, Dallas, TX USA * Corresponding author; email: charles.quinn{at}utsouthwestern.edu.
Sickle cell anemia (SS) is highly phenotypically variable, and early predictors of outcome could guide clinical care. To determine whether early vaso-occlusive complications predicted subsequent adverse outcomes in the Dallas Newborn Cohort, we studied all members with SS or sickle-
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| Copyright © 2006 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
0-thalassemia who presented in their first year of life and had 
5 years of follow-up. We defined three potential early predictors: hospitalizations in the first 3 years of life for (1) painful events other than dactylitis, (2) dactylitis, and (3) acute chest syndrome (ACS). We studied the associations of these predictors with the following late adverse outcomes (occurring after the third birthday): death; first overt stroke; use of disease-modifying therapy; and hospitalizations for pain events and ACS. None of the early events predicted death or stroke. Early pain and ACS both predicted a modest, temporary increase in the number of later painful episodes, but early ACS strongly increased the odds of more frequent ACS throughout childhood. Dactylitis had limited utility as a predictor. Although we still lack a useful prognostic framework for young children with SS, those who experience early ACS might be candidates for higher risk interventions to mitigate or cure their disease.
