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Published on: November 20, 2015
Neurodevelopmental Outcomes in Preterm Children with Sickle Cell Disease
Sarah E Bills1, Jeffrey Schatz1, Erin Hunt1
1Department of Psychology, University of South Carolina, Columbia, SC, USA.
Insights
Pediatric sickle cell disease (SCD) and preterm birth combined increase language deficits and abnormal cognitive screenings in children. This highlights a higher neurodevelopmental risk for affected individuals.
Area of Science:
- Neuroscience
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder impacting various organs.
- Preterm birth is associated with developmental challenges, including cognitive deficits.
- The combined effects of SCD and preterm birth on neurodevelopment are not fully understood.
Purpose of the Study:
- To investigate the synergistic impact of pediatric sickle cell disease (SCD) and preterm birth on cognitive functioning in children aged 6-8.
- To identify specific cognitive domains affected by the combined risk factors.
Main Methods:
- Cognitive assessments were administered to children with high-risk SCD genotypes (born preterm and full-term) and lower-risk/no SCD (born preterm and full-term).
- Statistical analyses, including factorial ANOVAs and log linear analyses, examined the relationships between SCD risk, preterm birth, and cognitive outcomes.
- Children were classified as having abnormal cognitive screening if scores fell 1.5 standard deviations below the population mean.
Main Results:
- Children with high-risk SCD and preterm birth exhibited poorer performance on expressive language tests compared to other groups.
- No significant differences were observed in processing speed and working memory tests across groups.
- A significant three-way interaction indicated that children with high-risk SCD born preterm were more likely to have abnormal cognitive screening results.
Conclusions:
- The combination of SCD and preterm birth may elevate the risk for language impairments and abnormal cognitive screenings.
- Neurodevelopmental risks associated with comorbid SCD and preterm birth can present as heterogeneous cognitive deficits.
- Further research is necessary to pinpoint cognitive domains most vulnerable to the combined effects of SCD and preterm birth.
Objectives:
To explore the combined effect of pediatric sickle cell disease (SCD) and preterm birth on cognitive functioning.
Methods:
Cognitive functioning was examined in children ages 6-8 with high risk SCD genotypes born preterm (n = 20) and full-term (n = 59) and lower risk SCD genotypes/no SCD born preterm (n = 11) and full-term (n = 99) using tests previously shown to be sensitive to SCD-related neurocognitive deficits. Factorial ANOVAs and log linear analyses were conducted to examine the relationship between SCD risk, preterm birth status, and cognitive outcomes. Continuous scores were examined for specific tests. Children were categorized as having an abnormal screening outcome if at least one cognitive score was ≥1.5 standard deviations below the population mean.
Results:
Children with elevated risk due to high risk SCD and preterm birth performed worse than other groups on a test of expressive language but not on tests that emphasize processing speed and working memory. There was a three-way interaction between preterm status, SCD risk, and abnormal screening outcome, which was largely driven by the increased likelihood of abnormal cognitive scores for children with high risk SCD born preterm.
Conclusions:
The combination of SCD and preterm birth may confer increased risk for language deficits and elevated rates of abnormal cognitive screenings. This suggests that neurodevelopmental risk imparted by comorbid SCD and preterm birth may manifest as heterogenous, rather than specific, patterns of cognitive deficits. Future studies are needed to clarify the domains of cognitive functioning most susceptible to disease-related effects of comorbid SCD and preterm birth.
