Executive Functioning Predicts Adaptive Functioning and Self-Care Independence in Pediatric Sickle Cell Disease.

Objective: This study examined the impact of executive functioning (EF) on adaptive functioning and self-care independence in pre-transition youth with sickle cell disease (SCD).

Methods: Thirty-four youth with SCD (ages 12-18 years) completed a brief neuropsychological battery and their caregivers completed questionnaires assessing adaptive functioning, EF behaviors, and independence in completing self-care tasks, including disease management. Hierarchical linear regression analyses were utilized to investigate if EF predicted significantly more variability in adaptive functioning and self-care independence than what is accounted for by socioeconomic status (SES), disease knowledge, and estimated intellectual functioning.

Hydroxyurea Use Associated with Nonverbal and Executive Skills in Sickle Cell Anemia.

Objective: Hydroxyurea (HU) is used in children with sickle cell disease (SCD) to increase fetal hemoglobin (HF), contributing to a decrease in physical symptoms and potential protection against cerebral microvasculopathy. There has been minimal investigation into the association between HU use and cognition in this population. This study examined the relationship between HU status and cognition in children with SCD.

Executive function "drives" differences in simulated driving performance between young adults with and without autism spectrum disorder.

Individuals with autism spectrum disorder (ASD) may experience greater difficulty learning to drive than peers who do not have ASD, but reasons for those differences are unclear. This study examined how diagnostic symptoms of ASD and commonly co-morbid executive dysfunction relate to differences in simulated driving performance between young, inexperienced drivers with and without ASD. Participants included 98 young adults, ages 16-26 years, half of which were diagnosed with ASD.

Neuropsychological outcome following thalamic stroke in adolescence: an identical twin comparison.

Medial thalamic stroke in adults commonly results in severe learning and memory impairments and executive dysfunction, particularly during the acute phase. However, there is limited research on the cognitive recovery from thalamic stroke in physically healthy adolescents. This study aimed to fill this gap in the literature by utilizing a monozygotic twin control to investigate the neuropsychological outcomes of bilateral thalamic stroke in adolescence.

Driving Comparisons Between Young Adults with Autism Spectrum Disorder and Typical Development.

Objective: Many individuals with autism spectrum disorder (ASD) are reluctant to pursue driving because of concerns about their ability to drive safely. This study aimed to assess differences in simulated driving performance in young adults with ASD and typical development, examining relationships between driving performance and the level of experience (none, driver's permit, licensed) across increasingly difficult driving environments.

Method: Participants included 50 English-speaking young adults (16-26 years old) with ASD matched for sex, age, and licensure with 50 typically-developing (TD) peers.

The Kennedy Krieger Independence Scales-Sickle Cell Disease: Executive components of transition readiness.

Purpose/objective: Youth with sickle cell disease (SCD) are at an increased risk for executive dysfunction and simultaneously have increased self-management needs compared to typical adolescents. This unique combination may contribute, in part, to difficulties during transition to young adulthood. Current measures assessing adaptive skills do not assess the executive components (e.

Predictors of Academic Achievement for School Age Children with Sickle Cell Disease.

Background: Children with sickle cell disease (SCD) are at risk for neurocognitive impairment and poor academic achievement, although there is limited research on factors predicting academic achievement in this population. This study explores the relative contribution to academic achievement of a comprehensive set of factors, such as environmental (socioeconomic status), disease-related (stroke, transfusion therapy, adherence), and psychosocial variables (child behavior, child quality of life (QoL)), controlling for intellectual functioning (IQ).

Methods: Eighty-two children with SCD completed measures assessing IQ and academic achievement, while parents completed questionnaires assessing adherence, child behavior, and child quality of life.

The Kennedy Krieger Independence Scales-Spina Bifida Version: a measure of executive components of self-management.

Purpose/objective: Successful implementation of functional self-care skills depends upon adequate executive functioning; however, many scales assessing adaptive skills do not address the inherent executive burden of these tasks. This omission is especially relevant for individuals with spina bifida, for whom medical self-care tasks impose a significant burden requiring initiation and prospective memory. The Kennedy Krieger Independence Scales-Spina Bifida Version (KKIS-SB) is a caregiver-reported measure designed to address this gap; it assesses skills for managing both typical and spina bifida-related daily self-care demands, with a focus on the timely and independent initiation of adaptive skills.

The role of neuropsychological evaluation in pediatric sickle cell disease.

Neuropsychological impairment occurs in children and adolescents with sickle cell disease. While the neuropsychological sequelae associated with cerebrovascular disease in these children have been identified, a well-informed clinical approach to neuropsychological evaluation for these vulnerable children has not been delineated. Moreover, issues related to standard of care for children and adolescents with sickle cell disease and transition to adulthood remain understudied despite the relevance to long-term medical, psychosocial, and neuropsychological outcomes.

Age-related differences in executive function among children with spina bifida/hydrocephalus based on parent behavior ratings.

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence.

Neuropsychological functioning in preschool-age children with sickle cell disease: the role of illness-related and psychosocial factors.

Cognitive and academic deficits have been identified in school-aged children with sickle cell disease (SCD). However, there have been very few identified studies that examine neuropsychological functioning in preschool-age children with SCD. It is important to understand effects of SCD from a developmental perspective and to consider the contribution of environmental factors in this at-risk population.