Developmental function in toddlers with sickle cell anemia

F. Daniel Armstrong, T. David Elkin, R. Clark Brown, Penny Glass, Sohail Rana, James F. Casella, Ram V. Kalpatthi, Steven Pavlakis, Zhibao Mi, Winfred C. Wang

Research output: Contribution to journalArticlepeer-review

28 Scopus citations


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-b0 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.

Original languageEnglish (US)
Pages (from-to)e406-e414
Issue number2
StatePublished - Feb 2013


  • Bayley Scales
  • Cognitive development
  • Sickle cell disease
  • Toddlers
  • Transcranial Doppler

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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