THE ROLE OF EXECUTIVE DYSFUNCTIONS IN THE VERBAL LANGUAGE DEFICITS OF CHILDREN WITH SICKLE CELL DISEASE
(Abstract release date: 05/18/17)
EHA Library. Colombatti R. 05/18/17; 182858; PB2145
Dr. R Colombatti
Contributions
Contributions
Abstract
Abstract: PB2145
Type: Publication Only
Background
Children with Sickle Cell Disease (SCD) frequently present impairment of general and specific neurocognitive functions, even in the absence of clear neurological damage at brain neuroimaging. Verbal language deficits are also common, but the etiology of poor performance in the verbal domain is still not clear. The ability to speak and communicate verbally relies on a complex interaction of cognitive and linguistic functions as well as on environmental factors, like bilingualism or second language (L2) learning.
The majority of children with SCD in Italy are of immigrant families whose first language is not Italian. These children thus perform poorly in tests assessing the verbal domain with adverse impact on school performances.
Aims
To evaluate if verbal language deficits in bilingual children with SCD are mainly due to linguistic and environmental issues or to impairment of executive functions
Methods
In this study a cohort of bilingual children with SCD and social-demographically matched healthy controls recruited from elementary schools of the same city, performed an extensive battery of tests to assess naming skills, phonological and semantic fluency, attention and Executive Functions (inhibition and planning skills) and visuo-spatial skills (Boston naming test, phonological and semantic fluency tests, Flanker task, Elithorn test, PMA spatial relations subscale). All tests were administered in Italian. A composite index considering parental education and employment was used to match socially, demographically and economically children at best.
Analyses of variance (ANOVAs) were performed to test differences between the two groups in verbal language, attention and executive functions. Hierarchical regressions explored the contribution of linguistic knowledge and executive functions (i.e. inhibition) to the verbal language deficit of children with SCD.
Results
Thirty-two children with HbSS SCD aged 6 to 12 years (mean age= 9.03) and 35 controls (mean age= 9.14) were enrolled. Patients and controls were matched for gender (F 53 vs 61%), ethnicity (African 30 vs 29), % of children born in Italy (81 vs 80%), number of years lived in Italy (8.09 vs 8.31) and Socio-Demographic Index (5.15 vs 4.59).
Children’s Performance (in Z scores) at Visuo-Spatial, Boston Naming, Phonological Fluency and Semantic Fluency Tests are shown in Figure 1.
The results overall showed significant differences between patients and controls in inhibition and planning (p= 0.001 and 0.001 respectively) and in verbal tasks that involved Executive Functions more (i.e. phonological fluency) (p=.004). The poorer verbal performance of children with SCD was not associated to visible lesions to Broca’s area; in fact only 9 patients presented Silent Infarcts that were all in the white matter, in watershed areas. Regression analyses showed that in children with SCD inhibition skills explained unique variance in phonological fluency, suggesting that poor executive control was a factor of the lower performance in this task.
Figure 1. Children’s Performance (in Z scores) at Visuo-Spatial, Boston Naming, Phonological Fluency and Semantic Fluency Tests. P-values: Visuo-spatial intelligence: not significant(ns); Boston naming: ns; Phonol-Fluency: 0.004; Semantic fluency: ns
Conclusion
Selective language problems may occur in children with SCD in the absence of clear neurological damage to language areas. These problems are explained by the executive dysfunction of patients with SCD and not by environmental factors like bilingualism. Cognitive rehabilitation or extra tuition may aid in overcoming these difficulties.
Session topic: 25. Sickle cell disease
Keyword(s): sickle cell disease, Children
Abstract: PB2145
Type: Publication Only
Background
Children with Sickle Cell Disease (SCD) frequently present impairment of general and specific neurocognitive functions, even in the absence of clear neurological damage at brain neuroimaging. Verbal language deficits are also common, but the etiology of poor performance in the verbal domain is still not clear. The ability to speak and communicate verbally relies on a complex interaction of cognitive and linguistic functions as well as on environmental factors, like bilingualism or second language (L2) learning.
The majority of children with SCD in Italy are of immigrant families whose first language is not Italian. These children thus perform poorly in tests assessing the verbal domain with adverse impact on school performances.
Aims
To evaluate if verbal language deficits in bilingual children with SCD are mainly due to linguistic and environmental issues or to impairment of executive functions
Methods
In this study a cohort of bilingual children with SCD and social-demographically matched healthy controls recruited from elementary schools of the same city, performed an extensive battery of tests to assess naming skills, phonological and semantic fluency, attention and Executive Functions (inhibition and planning skills) and visuo-spatial skills (Boston naming test, phonological and semantic fluency tests, Flanker task, Elithorn test, PMA spatial relations subscale). All tests were administered in Italian. A composite index considering parental education and employment was used to match socially, demographically and economically children at best.
Analyses of variance (ANOVAs) were performed to test differences between the two groups in verbal language, attention and executive functions. Hierarchical regressions explored the contribution of linguistic knowledge and executive functions (i.e. inhibition) to the verbal language deficit of children with SCD.
Results
Thirty-two children with HbSS SCD aged 6 to 12 years (mean age= 9.03) and 35 controls (mean age= 9.14) were enrolled. Patients and controls were matched for gender (F 53 vs 61%), ethnicity (African 30 vs 29), % of children born in Italy (81 vs 80%), number of years lived in Italy (8.09 vs 8.31) and Socio-Demographic Index (5.15 vs 4.59).
Children’s Performance (in Z scores) at Visuo-Spatial, Boston Naming, Phonological Fluency and Semantic Fluency Tests are shown in Figure 1.
The results overall showed significant differences between patients and controls in inhibition and planning (p= 0.001 and 0.001 respectively) and in verbal tasks that involved Executive Functions more (i.e. phonological fluency) (p=.004). The poorer verbal performance of children with SCD was not associated to visible lesions to Broca’s area; in fact only 9 patients presented Silent Infarcts that were all in the white matter, in watershed areas. Regression analyses showed that in children with SCD inhibition skills explained unique variance in phonological fluency, suggesting that poor executive control was a factor of the lower performance in this task.
Figure 1. Children’s Performance (in Z scores) at Visuo-Spatial, Boston Naming, Phonological Fluency and Semantic Fluency Tests. P-values: Visuo-spatial intelligence: not significant(ns); Boston naming: ns; Phonol-Fluency: 0.004; Semantic fluency: ns
Conclusion
Selective language problems may occur in children with SCD in the absence of clear neurological damage to language areas. These problems are explained by the executive dysfunction of patients with SCD and not by environmental factors like bilingualism. Cognitive rehabilitation or extra tuition may aid in overcoming these difficulties.
Session topic: 25. Sickle cell disease
Keyword(s): sickle cell disease, Children
{{ help_message }}
{{filter}}