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Blood, 15 January 2008, Vol. 111, No. 2, pp. 544-548.
Prepublished online as a Blood First Edition Paper on October 1, 2007; DOI 10.1182/blood-2007-07-100719.


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Submitted July 19, 2007
Accepted September 25, 2007

Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort

Charles T Quinn*, Nancy J Lee, Elizabeth P Shull, Naveed Ahmad, Zora R Rogers, and George R Buchanan

Department of Pediatrics, Division of Hematology-Oncology, University of Texas Southwestern Medical Center, Dallas, TX, United States
Southwestern Comprehensive Sickle Cell Center, Dallas, TX, United States
Children's Medical Center Dallas, Dallas, TX, United States

* Corresponding author; email: charles.quinn{at}utsouthwestern.edu.

The Cooperative Study of Sickle Cell Disease (CSSCD) reported that dactylitis, severe anemia, and leukocytosis in very young children with SCD increased the risk of later adverse outcomes, including death, stroke, frequent pain, and recurrent acute chest syndrome (ACS). This model has not been validated in other cohorts. We evaluated its performance in the Dallas Newborn Cohort (DNC), a newborn inception cohort of children with sickle cell disease. We studied 168 children (55% male, 97% SS) with a mean follow-up of 7.1 years who provided 1,188 patient-years of observation. Twenty-three (13.7%) subjects experienced adverse events: 2 (1.2%) died, 14 (8.3%) had a stroke, 4 (2.4%) had frequent pain, and 3 (1.8%) had recurrent ACS. There was no relationship between the early clinical predictors and later adverse outcomes, with the possible exception of leukocyte count. Most subjects who experienced adverse events were predicted to be at low risk for adverse events. No subject who was predicted to be at high risk actually experienced an adverse outcome. The sensitivity of the model did not rise above 20% until specificity fell below 60%. We suggest that this model should not be used as a criterion to initiate early interventions for sickle cell disease.


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