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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.


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CLINICAL TRIALS AND OBSERVATIONS

Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia

Charles T. Quinn1,3,, Elizabeth P. Shull2,3, Naveed Ahmad3, Nancy J. Lee2,3, Zora R. Rogers1,3, and George R. Buchanan1,3

1 Division of Hematology-Oncology, Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas, Dallas, TX; 2 Southwestern Comprehensive Sickle Cell Center, Dallas, TX; and 3 Children's Medical Center Dallas, Dallas, TX

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-ß0-thalassemia who presented in their first year of life and had 5 years or more of follow-up. We defined 3 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.


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A. Hryniewicz-Jankowska, P. K. Choudhary, L. P. Ammann, C. T. Quinn, and S. R. Goodman
Monocyte Protein Signatures of Disease Severity in Sickle Cell Anemia
Experimental Biology and Medicine, February 1, 2009; 234(2): 210 - 221.
[Abstract] [Full Text] [PDF]


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C. T. Quinn, N. J. Lee, E. P. Shull, N. Ahmad, Z. R. Rogers, and G. R. Buchanan
Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort
Blood, January 15, 2008; 111(2): 544 - 548.
[Abstract] [Full Text] [PDF]



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