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Published on: November 5, 2019
Cognitive profile of children with sickle cell anemia compared to healthy controls
Isabel Pimenta Spínola Castro1, Marcos Borato Viana2
1Universidade Federal de Minas Gerais (UFMG), Programa de Pós-Graduação em Saúde da Criança e do Adolescente, Belo Horizonte, MG, Brazil.
Insights
Children with sickle cell anemia exhibit severe cognitive impairment, with IQs significantly lower than healthy peers, even after accounting for socioeconomic factors. Early intervention is crucial to mitigate cognitive damage.
Area of Science:
- Pediatric Hematology
- Neuropsychology
- Developmental Pediatrics
Background:
- Sickle cell anemia (SCA) is a genetic blood disorder affecting red blood cells.
- Neonatal screening allows for early diagnosis of SCA.
- Cognitive development in children with chronic illnesses requires thorough evaluation.
Purpose of the Study:
- To assess cognitive abilities in children and adolescents diagnosed with SCA via neonatal screening.
- To compare cognitive performance between SCA patients and healthy controls.
- To adjust cognitive scores for socioeconomic status (SES).
Main Methods:
- Cognitive assessment using the Wechsler Intelligence Scale for Children-III (WISC-III).
- Study included 64 children/adolescents with SCA and 64 matched healthy controls.
- Socioeconomic status was measured using the Criterion Brasil scale.
Main Results:
- Patients with SCA demonstrated significantly lower overall IQ, Verbal IQ, and Performance IQ compared to controls.
- Specific cognitive domains including processing speed, distraction resistance, and perceptual organization were impaired in SCA patients.
- After adjusting for SES, SCA was a strong predictor of lower IQ, with patients scoring 21.2 points lower.
Conclusions:
- Cognitive impairment in children with SCA is severe and independent of socioeconomic status.
- The findings underscore the need for early preventive strategies to address cognitive deficits in SCA.
- Early intervention is recommended to prevent long-term cognitive damage in affected children.
Objective:
To evaluate the cognitive abilities of children and adolescents with sickle cell anemia diagnosed through neonatal screening and to compare them with healthy controls, adjusting the results to their socioeconomic status.
Methods:
Cognitive assessment was performed with the Wechsler WISC-III scale in 64 children and adolescents with sickle cell anemia and in 64 controls matched by gender and age, without the disease and without neurological impairment; socioeconomic status was measured by the Criterion Brasil.
Results:
All cognitive scores were lower in the group of patients. The mean overall IQ, Verbal IQ, and Performance IQ were, respectively, 90.95 for the group of patients and 113.97 for the controls (p<0.001); 91.41 for the group of patients and 112.31 for the controls (p<0.001); 92.34 for the group of patients and 113.38 for the controls (p<0.001). Scores for processing speed, distraction resistance, and perceptual organization were also significantly lower in patients. A direct and significant correlation was detected between socioeconomic status and cognitive scores. In the multivariate analysis, for the same socioeconomic status, a child with sickle cell anemia had an average IQ of 21.2 points lower than the mean IQ observed for the controls (p<0.001), indicating that the disease, adjusted for the socioeconomic effect, is a strong predictor of the overall IQ.
Conclusion:
The cognitive impairment of children with sickle cell anemia is severe and manifests even when the disease effect is adjusted to the socioeconomic status. In the authors' view, such impairment requires an early preventive approach in order to avoid this cognitive damage.
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