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Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study
STUDY OBJECTIVES: Compared to typically developing children and young adults (CYA-TD), those living with Sickle Cell Disease (CYA-SCD) experience more cognitive difficulties, particularly with executive function. Few studies have examined the relative importance of silent cerebral infarction (SCI),...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10408298/ https://www.ncbi.nlm.nih.gov/pubmed/37560456 http://dx.doi.org/10.3389/fneur.2023.1087054 |
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author | Koelbel, Melanie Hamdule, Shifa Kirkham, Fenella J. Stotesbury, Hanne Hood, Anna Marie Dimitriou, Dagmara |
author_facet | Koelbel, Melanie Hamdule, Shifa Kirkham, Fenella J. Stotesbury, Hanne Hood, Anna Marie Dimitriou, Dagmara |
author_sort | Koelbel, Melanie |
collection | PubMed |
description | STUDY OBJECTIVES: Compared to typically developing children and young adults (CYA-TD), those living with Sickle Cell Disease (CYA-SCD) experience more cognitive difficulties, particularly with executive function. Few studies have examined the relative importance of silent cerebral infarction (SCI), haemoglobin and arterial oxygen content on age-related cognitive changes using cross-sectional or longitudinal (developmental trajectory) data. This study presents cohort data from a single timepoint to inform studies with multiple timepoints. METHODS: We compared cross-sectional raw and scaled scores as age-related changes in cognition (trajectories) in CYA-SCD and age-and ethnicity-matched CYA-TD. We also compared cross-sectional age-related changes in cognition (trajectories) in CYA-SCD with and without SCI to CYA-TD. General cognitive abilities were assessed using Wechsler Intelligence Scales, including the Verbal Comprehension Index (VCI) and Perceptual Reasoning Index (PRI) underpinning IQ. Executive function was evaluated using the Delis-Kaplan Executive Function System (D-KEFS) Tower subtest and the Behaviour Rating Inventory of Executive Function (BRIEF) questionnaire. SCI were identified from contemporaneous 3 T MRI; participants with overt stroke were excluded. Recent haemoglobin was available and oxygen saturation (SpO(2)) was measured on the day of the MRI. RESULTS: Data were available for 120 CYA-SCD [62 male; age = 16.78 ± 4.79 years; 42 (35%) with SCI] and 53 CYA-TD (23 male; age = 17.36 ± 5.16). Compared with CYA-TD, CYA-SCD experienced a delayed onset in VCI and slower rate of development for BRIEF Global Executive Composite, Metacognition Index (MI), and Behaviour Regulation Index. The rate of executive function development for the BRIEF MI differed significantly between CYA-TD and CYA-SCD, with those with SCI showing a 26% delay compared with CYA-TD. For CYA-SCD with SCI, arterial oxygen content explained 22% of the variance in VCI and 37% in PRI, while haemoglobin explained 29% of the variance in PRI. CONCLUSION: Age-related cognitive trajectories of CYA-SCD may not be impaired but may progress more slowly. Longitudinal studies are required, using tests unaffected by practice. In addition to initiation of medical treatment, including measures to improve arterial oxygen content, early cognitive intervention, educational support, and delivery of extracurricular activities could support cognitive development for CYA-SCD. |
format | Online Article Text |
id | pubmed-10408298 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-104082982023-08-09 Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study Koelbel, Melanie Hamdule, Shifa Kirkham, Fenella J. Stotesbury, Hanne Hood, Anna Marie Dimitriou, Dagmara Front Neurol Neurology STUDY OBJECTIVES: Compared to typically developing children and young adults (CYA-TD), those living with Sickle Cell Disease (CYA-SCD) experience more cognitive difficulties, particularly with executive function. Few studies have examined the relative importance of silent cerebral infarction (SCI), haemoglobin and arterial oxygen content on age-related cognitive changes using cross-sectional or longitudinal (developmental trajectory) data. This study presents cohort data from a single timepoint to inform studies with multiple timepoints. METHODS: We compared cross-sectional raw and scaled scores as age-related changes in cognition (trajectories) in CYA-SCD and age-and ethnicity-matched CYA-TD. We also compared cross-sectional age-related changes in cognition (trajectories) in CYA-SCD with and without SCI to CYA-TD. General cognitive abilities were assessed using Wechsler Intelligence Scales, including the Verbal Comprehension Index (VCI) and Perceptual Reasoning Index (PRI) underpinning IQ. Executive function was evaluated using the Delis-Kaplan Executive Function System (D-KEFS) Tower subtest and the Behaviour Rating Inventory of Executive Function (BRIEF) questionnaire. SCI were identified from contemporaneous 3 T MRI; participants with overt stroke were excluded. Recent haemoglobin was available and oxygen saturation (SpO(2)) was measured on the day of the MRI. RESULTS: Data were available for 120 CYA-SCD [62 male; age = 16.78 ± 4.79 years; 42 (35%) with SCI] and 53 CYA-TD (23 male; age = 17.36 ± 5.16). Compared with CYA-TD, CYA-SCD experienced a delayed onset in VCI and slower rate of development for BRIEF Global Executive Composite, Metacognition Index (MI), and Behaviour Regulation Index. The rate of executive function development for the BRIEF MI differed significantly between CYA-TD and CYA-SCD, with those with SCI showing a 26% delay compared with CYA-TD. For CYA-SCD with SCI, arterial oxygen content explained 22% of the variance in VCI and 37% in PRI, while haemoglobin explained 29% of the variance in PRI. CONCLUSION: Age-related cognitive trajectories of CYA-SCD may not be impaired but may progress more slowly. Longitudinal studies are required, using tests unaffected by practice. In addition to initiation of medical treatment, including measures to improve arterial oxygen content, early cognitive intervention, educational support, and delivery of extracurricular activities could support cognitive development for CYA-SCD. Frontiers Media S.A. 2023-07-25 /pmc/articles/PMC10408298/ /pubmed/37560456 http://dx.doi.org/10.3389/fneur.2023.1087054 Text en Copyright © 2023 Koelbel, Hamdule, Kirkham, Stotesbury, Hood and Dimitriou. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neurology Koelbel, Melanie Hamdule, Shifa Kirkham, Fenella J. Stotesbury, Hanne Hood, Anna Marie Dimitriou, Dagmara Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title | Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title_full | Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title_fullStr | Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title_full_unstemmed | Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title_short | Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
title_sort | mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study |
topic | Neurology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10408298/ https://www.ncbi.nlm.nih.gov/pubmed/37560456 http://dx.doi.org/10.3389/fneur.2023.1087054 |
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