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Updated: May 15, 2026

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
08:23

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Published on: November 5, 2019

Developmental function in toddlers with sickle cell anemia.

F Daniel Armstrong1, T David Elkin, R Clark Brown

  • 1Department of Pediatrics (D-820), PO Box 016820, Miami, FL 33101, USA. darmstrong@miami.edu

Pediatrics
|January 9, 2013
PubMed
Summary
This summary is machine-generated.

In infants with sickle cell disease, neurodevelopment is generally average, but behavioral and adaptive functions decline with age. Further research is needed to understand these developmental changes.

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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Developmental Psychology

Background:

  • Neurocognitive impairment is recognized in children and adults with sickle cell anemia.
  • Limited data exists on neurodevelopmental outcomes in very young children with sickle cell disease.

Purpose of the Study:

  • To assess the neurodevelopmental status of infants in the Pediatric Hydroxyurea Phase III Clinical Trial (Baby Hug).
  • To investigate the relationships between neurodevelopment, age, cerebral blood flow velocity, and hemoglobin concentration in infants with sickle cell disease.

Main Methods:

  • 193 infants (hemoglobin SS or S-β(0) thalassemia) aged 7–18 months underwent standardized neurodevelopmental assessments.
  • Evaluated associations between neurodevelopmental scores and age, income, education, hemoglobin levels, and transcranial Doppler velocity.

Main Results:

  • Mean neurodevelopmental functioning was within the average range.
  • No infants had impaired mental development scores (<70); however, 22 had clinically significant scores (11 psychomotor, 11 behavioral).
  • Poorer performance correlated with older age at baseline; behavior ratings decreased by 2.82 percentile points per month of age. Parent-reported functional abilities and hemoglobin levels showed a negative association with higher transcranial Doppler velocities.

Conclusions:

  • Overall neurodevelopmental functioning in infants with sickle cell disease is within the normal range.
  • Behavioral and adaptive functioning showed a decline with increasing age, even in this young cohort.
  • Further research is required to elucidate the mechanisms underlying the association between poorer developmental function and older age in this population.