Perceptual-Motor Dysfunction in Children with Sickle Cell Trait

Flick, Grad L.; Duncan, Christian A.

doi:10.2466/pms.1973.36.1.234

Cited by 10 publications

(3 citation statements)

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“…26 Children with the sickle cell trait have a significantly higher prevalence of vasculopathy and brain injury compared with children with normal hemoglobin, 26 and it is possible that the sickle cell trait could be associated with some degree of cognitive impairment. 27 Additional research is needed to determine if children with the sickle cell trait show cognitive impairment compared with African-American children with normal AA hemoglobin.…”

Section: Discussionmentioning

confidence: 99%

“…Recent findings also suggest that siblings of patients with sickle cell disease might not be an optimal control group; siblings often have the sickle cell trait and can have brain injury related to their heterozygous condition, 26 and it is not known if the sickle cell trait has an impact on cognitive ability. 27 Mechanisms that underlie cognitive impairment in patients with sickle cell disease are poorly understood. 18 It is not known whether cognitive deficits in patients with sickle cell disease occur only in the context of a stroke or "silent infarction" (brain injury that is clinically asymptomatic but visible by magnetic resonance imaging [MRI]) or whether impairment can also occur among children who have no evidence of focal brain injury.…”

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Cognitive Deficits in Children With Sickle Cell Disease

et al. 2005

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We tested a hypothesis that children with sickle cell disease who are completely normal by magnetic resonance imaging can still be cognitively impaired, as predicted by a model of diffuse brain injury. Fifty-four patients with hemoglobin SS (average age 10.9 years +/- 2.9 years SD) were examined with the Wechsler Intelligence Scale for Children-III (WISC-III) and were randomly matched by age, race, and gender with healthy children from the Wechsler normative database. Patients were also imaged at 1.5 Tesla with standard imaging sequences. Among 30 patients who were normal by magnetic resonance imaging, there were substantial deficits in Wechsler Full-Scale IQ, Verbal IQ, and Performance IQ (all P < .01) compared with African-American controls. The patient Wechsler Full-Scale IQ was 12.9 points lower than that of controls and decreased as a function of age (probability = .014). The findings suggest that there is diffuse brain injury in patients and that patient deficits increase with age.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Cognitive Deficits in Children With Sickle Cell Disease

et al. 2005

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“…Swift, Cohen, Hynd, Wisenbaker, McKie, Makari & McKie, 1989; Wasserman, Wilimas, Fairclough, Mulhern & Wang, 1991), although they have a mild anaemia, and a slightly increased risk of vasculopathy and brain damage (Steen et al ., 2003). While there were sporadic early reports of lower scores on neuropsychological tests in children with trait compared to children with normal haemoglobin status (Flick & Duncan, 1973; McCormack, Scarr‐Salapatek, Polesky, Thompson, Katz & Barker, 1975), the findings have not been consistently replicated (e.g. Ashcroft, Desai & Richardson, 1976; Kramer, Rooks & Pearson, 1978), and more recent studies have typically included children with and without trait as one control group (e.g.…”

Section: Sickle Cell Diseasementioning

confidence: 99%

Physiological correlates of intellectual function in children with sickle cell disease: hypoxaemia, hyperaemia and brain infarction

Hogan

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Vargha‐Khadem

et al. 2006

Developmental Science

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Lowered intelligence relative to controls is evident by mid-childhood in children with sickle cell disease. There is consensus that brain infarct contributes to this deficit, but the subtle lowering of IQ in children with normal MRI scans might be accounted for by chronic systemic complications leading to insufficient oxygen delivery to the brain. We investigated the relationship between daytime oxyhaemoglobin saturation (SpO2), cerebral blood flow velocity (CBFV) and intellectual function (IQ) using path-analysis in 30 adolescents with sickle cell disease (mean age 17.4 years, SD 4.2). Initial analyses revealed that the association between SpO2 and Full Scale IQ (FSIQ) was fully mediated by increased CBFV, whereby SpO2 was negatively correlated with CBFV and CBFV was negatively correlated with FSIQ, i.e. decreases in oxygen saturation are associated with increases in velocity, and increased velocity is associated with lowered IQ scores. The mediated relationship suggests that lowered IQ may be a function of abnormal oxygen delivery to the brain. Further analyses showed that the association between CBFV and IQ was significant for verbal but not for performance IQ. The pathophysiology characteristic of SCD can interfere with brain function and constrain intellectual development, even in the absence of an infarct. This supports the hypothesis that lowered intellectual function is partly explained by chronic hypoxia, and has wider implications for our understanding of SCD pathophysiology.

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Cognitive Effects of Acute and Chronic Pediatric Medical Conditions

Lehr

1984

Psychological and Behavioral Assessment

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Perceptual-Motor Dysfunction in Children with Sickle Cell Trait

Cited by 10 publications

References 1 publication

Cognitive Deficits in Children With Sickle Cell Disease

Cognitive Deficits in Children With Sickle Cell Disease

Physiological correlates of intellectual function in children with sickle cell disease: hypoxaemia, hyperaemia and brain infarction

Cognitive Effects of Acute and Chronic Pediatric Medical Conditions

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