期刊
PEDIATRICS
卷 131, 期 2, 页码 E406-E414出版社
AMER ACAD PEDIATRICS
DOI: 10.1542/peds.2012-0283
关键词
sickle cell disease; cognitive development; transcranial Doppler; Bayley Scales; toddlers
类别
资金
- National Heart, Blood, and Lung Institute (NHLBI) [N01-HB-07150, N01-HB-07151, N01-HB-07152, N01-HB-07153, N01-HB-07154, N01-HB-07155, N01-HB-07156, N01-HB-07157, N01-HB-07158, N01-HB-07159, N01-HB-07160]
- NHLBI [1-U54-HL-090569]
- National Institutes of Health (NIH)
- Children's National Medical Center
- Duke University Medical Center
- Howard University College of Medicine
- Johns Hopkins University School of Medicine
- Sinai Hospital, Baltimore
- Medical University of South Carolina
- St Jude Children's Research Hospital
- SUNY Downstate Medical Center/Kings County Hospital Center
- University of Miami
- University of Mississippi Medical Center
- University of Texas Southwestern Medical Center, Dallas
- University of Alabama at Birmingham
- Drexel University
- Emory University School of Medicine
- Children's Hospital of Michigan
- Clinical Trials & Surveys Corporation
- National Heart, Lung, and Blood Institute
- National Institute of Child Health and Human Development
- National Heart, Lung, and Blood Institute/National Institutes of Health [N01-HB-07150, N01-HB07160]
- Best Pharmaceuticals for Children Act
BACKGROUND: Neurocognitive impairment occurs in children and adults with sickle cell anemia, but little is known about neurodevelopment in very young children. We examined the neurodevelopmental status of infants participating in the Pediatric Hydroxyurea Phase III Clinical Trial (Baby Hug) to determine relationships with age, cerebral blood flow velocity, and hemoglobin concentration. METHODS: Standardized measures of infant neurodevelopment were administered to 193 infants with hemoglobin SS or hemoglobin S-beta(0) thalassemia between 7 and 18 months of age at the time of their baseline evaluation. Associations between neurodevelopmental scores and age, family income, parent education, hemoglobin concentration, and transcranial Doppler velocity were examined. RESULTS: Mean functioning on the baseline neurodevelopment scales was in the average range. There were no mental development scores <70 (impaired); 22 children had scores in the clinically significant range, 11 with impaired psychomotor scores and 11 with problematic behavior rating scores. Significantly poorer performance was observed with older age at baseline. Behavior rating scores were an average of 2.82 percentile points lower per month of age, with similar patterns observed with parent report using adaptive behavior scales. Parent-reported functional abilities and hemoglobin were negatively associated with higher transcranial Doppler velocities. CONCLUSIONS: Whereas overall functioning was in the normal range, behavioral and adaptive function was poorer with older age, even in this very young group of children. Explanatory mechanisms for this association between poorer developmental function and older age need to be identified. Pediatrics 2013;131:e406-e414
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