Assessment of Executive Functions in Preschool Children With Sickle Cell Anemia

Downes, Michelle; Kirkham, Fenella J.; Telfer, Paul; Haan, Michelle de

doi:10.1017/s1355617718000255

Cited by 12 publications

(18 citation statements)

References 21 publications

(38 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Moreover, the dlPFC is a core region in the executive control network that is involved in working memory, cognitive flexibility, selective attention and response inhibition (Elliott, 2003). One might speculate that the observed alterations in dlPFC_LC functional connectivity might underlie executive function deficits and associated cortical changes reported in SCD (Chen et al, 2017; Coloigner et al, 2017; Downes et al, 2019, Downes et al, 2018; Schatz and Buzan, 2006; Scott Mackin et al, 2014; Swift et al, 1989; Vichinsky et al, 2010). High levels of noradrenaline via projections from the LC to the prefrontal cortex have been shown to impair prefrontal inhibitory functions critical for executive function (Xing et al, 2016).…”

Section: Discussionmentioning

confidence: 99%

Patients with sickle-cell disease exhibit greater functional connectivity and centrality in the locus coeruleus compared to anemic controls

Bhatt

Zeltzer

Coloigner

et al. 2019

NeuroImage: Clinical

View full text Add to dashboard Cite

HighlightsPatients with sickle-cell disease (SCD) have greater resting-state functional connectivity between the locus coeruleus (LC) and dorsolateral prefrontal cortex (dlPFC).Patients with SCD have greater resting state centrality of the LCSCD patients with chronic pain exhibited even greater functional connectivity between the LC and dlPFC.This study supports hyper-connectivity between the LC and PFC is a potential chronic pain generator.

show abstract

Section: Discussionmentioning

confidence: 99%

Patients with sickle-cell disease exhibit greater functional connectivity and centrality in the locus coeruleus compared to anemic controls

Bhatt

Zeltzer

Coloigner

et al. 2019

NeuroImage: Clinical

View full text Add to dashboard Cite

show abstract

“…Visuomotor and executive functioning skills are known to be reduced in older patients with SCD, 51,52 but these deficits were not yet apparent in our sample. Notably, we relied on parent report of executive functioning skills, which may not be sensitive to some of the early executive deficits observed in other studies 20 . Weaknesses in school readiness skills were strongly associated with parent education and occupation ( r = .42).…”

Section: Discussionmentioning

confidence: 99%

“…Three studies with small samples ( n < 30) have assessed neurocognitive performance in preschool‐aged patients with SCD 20–22 . Findings documented deficits in language, visuospatial, and motor domains with medium to large effect sizes compared to normative expectations 22 .…”

Section: Introductionmentioning

confidence: 99%

Neurocognitive functioning in preschool children with sickle cell disease

Heitzer

Cohen

Okhomina

et al. 2021

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Background Children with sickle cell disease (SCD) experience neurodevelopmental delays; however, there is limited research with preschool‐age children. This study examined neurocognitive risk and protective factors in preschoolers with SCD. Procedure Sixty‐two patients with SCD (60% HbSS/HbSβ0‐thalassemia; 40% HbSC/HbSβ+‐thalassemia) between the ages of 3 and 6 years (mean = 4.77 years) received a neuropsychological evaluation as routine systematic surveillance. Patients were not selected for disease severity, prior central nervous system findings, or existing cognitive concerns. Thirty‐four patients (82% HbSS/HbSβ0‐thalassemia) were prescribed hydroxyurea (HU) at the time of their neuropsychological evaluation. On average, these patients had been prescribed HU at 2.15 (standard deviation = 1.45) years of age. The average dose was 28.8 mg/kg/day. Besides genotype, there were no group differences in medical or demographic factors based on HU treatment status. Results Patients with HbSS/HbSβ0‐thalassemia scored below normative expectations on measures of intelligence, verbal comprehension, and school readiness (false discovery rate‐adjusted p‐value [pFDR] < .05). Age, sickle genotype, and HU treatment exposure were not associated with measured neurocognitive outcomes (pFDR > .05). Greater social vulnerability at the community level was associated with poorer performance on measures of intellectual functioning, verbal comprehension, visuomotor control, and school readiness, as well as parent report of executive dysfunction (pFDR < .05). Greater household socioeconomic status was positively associated with academic readiness. Conclusions Preschoolers with severe SCD (HbSS/HbSβ0‐thalassemia) perform below age expectations on measures of intelligence and academic readiness. Sociodemographic factors were stronger drivers of neurocognitive performance than disease severity or disease‐modifying treatment. Neurodevelopmental interventions targeting the home and broader community environment are needed.

show abstract

“…70 Executive function is a term for a collection of skills that is used to control everyday behavior. 71 Basic executive function skills such as attention control tend to emerge first and later progress into more complex skills such as cognitive flexibility. 72 Findings of Downes et al revealed that children with SCA perform poorer than their matched peers on multistep assessment.…”

Section: Cognitive Function In Children Less Than Five Years Of Agementioning

confidence: 99%

Neurologic complications in children under five years with sickle cell disease

Galadanci

DeBaun

Galadanci

2019

Neuroscience Letters

View full text Add to dashboard Cite

Introduction: Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world affecting every organ. The major challenge in the medical care of children with SCD is preventing end-organ dysfunction, particularly the brain. Major neurologic complications in children less than five years with SCD include, but are not limited to, Silent cerebral infarct, cerebral sinus thrombosis, epilepsy, reversible encephalopathy syndrome, and ischemic and hemorrhagic stroke. Recurrent headaches and migraine are not rare in children under five years with SCD. This review will focus on the neurologic complications and the description of the modifiable risk factors in children less than 5 years of age with emphasis on differences between high and low resource settings.Areas covered: Neurologic complications of children under 5 years of age and the modifiable risk factors. The PUBMED database was searched using medical subject headings (MeSH) and keywords for articles regarding neurologic complications in children under 5 years of age. Conclusion:Neurologic complications in children under five years of age with SCD may be more frequent than currently reported, among which Silent cerebral infarct and cognitive impairment are the most common.

show abstract

Assessment of Executive Functions in Preschool Children With Sickle Cell Anemia

Cited by 12 publications

References 21 publications

Patients with sickle-cell disease exhibit greater functional connectivity and centrality in the locus coeruleus compared to anemic controls

Patients with sickle-cell disease exhibit greater functional connectivity and centrality in the locus coeruleus compared to anemic controls

Neurocognitive functioning in preschool children with sickle cell disease

Neurologic complications in children under five years with sickle cell disease

Contact Info

Product

Resources

About