Influence of severity of anemia on clinical findings in infants with sickle cell anemia: Analyses from the BABY HUG study

• Type of Material: Article
• Authors: Jeffrey D. Lebensburger, University of Alabama Birmingham; Scott T. Miller, King's County Hospital Center; Thomas H. Howard, University of Alabama Birmingham; James F. Casella, Johns Hopkins University; Robert Brown, Emory University; Ming Lu, Clinical Trials & Surveys Corp.; Rathi V. Iyer, University of Mississippi; Sharada Sarnaik, Children's Hospital of Michigan; Zora R. Rogers, UT Southwestern Medical Center; Winfred C. Wang, St. Jude Children's Research Hospital
• Language: English
• Date: 2012-10-01
• Publisher: Wiley: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2011 Wiley Periodicals, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1002/pbc.24037
• Title of Journal or Parent Work: Pediatric Blood and Cancer
• ISSN: 1545-5009
• Volume: 59
• Issue: 4
• Start Page: 675
• End Page: 678
• Grant/Funding Information: We acknowledge the efforts of the BABY HUG subjects and their families, the contributions of all who participated in BABY HUG (http://www.ctasc.com/cms/StudySites/babyhug.htm); and the support of the National Heart, Lung, and Blood Institute/ National Institutes of Health Contracts N01-HB-07150 to N01-HB-07160; with partial support of the Best Pharmaceuticals for Children Act; and the National Institute of Child Health and Human Development.
• Keywords: transcranial doppler; PREDICTION; COHORT; Hematology; Life Sciences & Biomedicine; Pediatrics; RISK-FACTORS; Science & Technology; YOUNG-CHILDREN; sickle cell anemia; DISEASE; HYDROXYUREA; Oncology; renal; ADVERSE OUTCOMES; MORTALITY; pain; ABNORMALITIES; HEMOLYSIS; spleen; acute chest syndrome; hydroxyurea
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

Background: Clinical complications of sickle cell anemia begin in infancy. BABY HUG (ClinicalTrials.gov, NCT00006400) was a NHLBI-NICHD supported randomized phase III placebo-controlled trial of hydroxyurea (HU) in infants (recruited at 9-18 months) unselected for clinical severity with sickle cell anemia. This secondary analysis of data from BABY HUG examines the influence of anemia on the incidence of sickle cell related complications, and the impact of hydroxyurea therapy in altering these events by comparing children with lower (<25th percentile) and higher (>75th percentile) hemoglobin concentrations at study entry. Procedure: Infants were categorized by: (1) age-adjusted hemoglobin quartiles as determined by higher (Hi) and lower (Lo) hemoglobin concentrations at study entry (9-12 months old: <8.0 and >10.0gm/dL; 12-18 months old: <8.1 and >9.9gm/dL) and (2) treatment arm (hydroxyurea or placebo). Four subgroups were created: placebo (PL) LoHb (n=25), PL HiHb (n=27), hydroxyurea (HU) LoHb (n=21), and HU HiHb (n=18). The primary and secondary endpoints of BABY HUG were analyzed by subgroup. Results: Infants with lower hemoglobin at baseline were more likely to have a higher incidence of clinical events (acute chest syndrome, pain crisis, fever) as well as higher TCD velocities and lower neuropsychological scores at study exit. Hydroxyurea reduced the incidence of these findings. Conclusion: Infants with more severe anemia are at risk for increased clinical events that may be prevented by early initiation of hydroxyurea.