Neurobehavioral impact of sickle cell disease in early childhood

Abstract

The physical effects of sickle cell disease (SCD) begin in infancy or early childhood, yet most behavioral studies have focused on school-age children. We evaluated the impact of higher versus lower neurologic risk on language, motor abilities, executive functions, and temperament in toddlers and early preschoolers with SCD. Thirty-nine children with higher risk SCD were compared to 22 children with lower risk SCD. Language and motor abilities were lower in older compared with younger children but were unrelated to sickle cell subgroups. Executive functions, particularly working memory, were poorer in children with higher risk SCD regardless of age. Parent-reported activity level was also lower in children with higher risk. Specific behavioral influences of SCD are evident early in childhood and include working memory decrements. Executive function deficits in SCD can emerge early in life and may be an important context for other areas of cognitive and behavioral development.

Language Processing Deficits in Sickle Cell Disease in Young School-Age Children

“…In toddlers with SCD, we did not find evidence for a relationship between language development and neurologic risk (Schatz & Roberts, 2007). In school-age children (6-16 years old) with SCD, we have found associations between verbal ability and anemia severity (i.e., hematocrit).…”

“…naire that we have used previously (Schatz & Roberts, 2007) was administered that includes 7 items assessing parental warmth and involvement (Strayhorn & Weidman, 1988) as well as 5 items assessing parenting self-efficacy (Dumka, Stoerzinger, Jackson, & Roosa, 1996). The internal consistency for this parenting measure in the present sample was á = .71.…”

“…The contrast between children with SCD at low neurologic risk to a well-matched comparison group without a chronic health condition allows for an inference about the role of non-neurologic health problems on cognitive development in SCD. These data may be important for interpreting prior studies of cognitive development in young children with SCD, which have not typically included a comparison group without a chronic health condition (e.g., Schatz & Roberts, 2007;Thompson, Gustafson, Bonner, & Ware, 2002). We hypothesized that low-risk SCD would show no more than small effect size differences from the non-SCD group across measures (using the effect size labels of Cohen, 1988).…”

“…Syntactic processing involves both short-term memory and complex working memory, whereby incoming information must be stored and manipulated while abstract syntactic rules are abstracted (Gathercole, 1999;Laws, 2004). Working memory deficits have been found in children with SCD at high neurologic risk in early childhood (Schatz & Roberts, 2007) and appear to be particularly robust for verbally-mediated working memory tasks later in childhood (Schatz, 2005). Prior research has suggested phonological processing may also be affected in children with SCD (Schatz & Roberts, 2005;Steen, Elliot, Miles, Jones, & Wang, 2002), although phonological processing has been much less study than vocabulary and general verbal ability.…”

Resolución temporal visual en pacientes con anemia drepanocítica

“…Otras formas son: SC, SB 0 y SB + . Los subtipos SS y SB 0 están asociados con una elevada ocurrencia de problemas neurológicos, incluidos infartos cerebrales clínicos o silentes (Ballas et al, 2012) y pobre funcionamiento cognitivo desde la temprana infancia (Schatz & Roberts, 2007). Los accidentes cerebrovasculares se presentan como una de las complicaciones más importantes de la drepanocitosis, que limita el desarrollo potencial pleno de niños y adultos (Detaum, Armstrong et al, 2012) La disfunción neurocognitiva está bien documentada en niños con drepanocitosis (Schatz, Finke, Kellett, & Kramer, 2002) y aunque puede ser una de las consecuencias más importantes de esta enfermedad no ha sido muy estudiada en adultos (Vichinsky et al, 2010).…”