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Nash, Rebecca; Riley, Catharine; Paramsothy, Pangaja; Gilbertson, Kendra; Raspa, Melissa; Wheeler, Anne; Dziuban, Eric J.; Peacock, Georgina – American Journal on Intellectual and Developmental Disabilities, 2019
Children with fragile X syndrome (FXS) display wide-ranging intellectual and behavioral abilities that affect daily life. We describe the educational setting of students with FXS and assess the relationships between school setting, co-occurring conditions, and functional ability using a national survey sample (n = 982). The majority of students…
Descriptors: Genetic Disorders, Intellectual Disability, Behavior Problems, Individualized Education Programs
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Hamrick, Lisa R.; Tonnsen, Bridgette L. – American Journal on Intellectual and Developmental Disabilities, 2019
Although social communication skills are commonly delayed in children with neurogenetic syndromes (NGS), skill profiles in very young children are largely under characterized, in part due to the lack of validated assessment measures appropriate for these populations. We addressed this gap by validating and applying a popular early social…
Descriptors: Rating Scales, Genetic Disorders, Test Validity, Interpersonal Communication
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Joyce, Anna; Hill, Catherine M.; Karmiloff-Smith, Annette; Dimitriou, Dagmara – American Journal on Intellectual and Developmental Disabilities, 2019
Sleep plays a key role in the consolidation of newly acquired information and skills into long term memory. Children with Down syndrome (DS) and Williams syndrome (WS) frequently experience sleep problems, abnormal sleep architecture, and difficulties with learning; thus, we predicted that children from these clinical populations would demonstrate…
Descriptors: Sleep, Cognitive Processes, Down Syndrome, Genetic Disorders
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Adams, Dawn; Clarke, Samantha; Griffith, Gemma; Howlin, Pat; Moss, Jo; Petty, Jane; Tunnicliffe, Penny; Oliver, Chris – American Journal on Intellectual and Developmental Disabilities, 2018
It is well documented that mothers of children with challenging behavior (CB) experience elevated levels of stress and that this persists over time, but less is known about the experience of mothers of children with rare genetic syndromes. This article describes 2 studies, 1 cross-sectional and 1 longitudinal, comparing well-being in mothers of…
Descriptors: Mothers, Stress Variables, Genetic Disorders, Depression (Psychology)
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Adlof, Suzanne M.; Klusek, Jessica; Hoffmann, Anne; Chitwood, Kerrie L.; Brazendale, Allison; Riley, Karen; Abbeduto, Leonard J.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2018
Individuals with fragile X syndrome (FXS) present with significant deficits in reading skills, but scant research exists to understand the characteristics of the reading delays or best practices for reading instruction with this population. Study 1 examined the relationship between phonological awareness and reading skills in individuals with FXS.…
Descriptors: Child Rearing, Genetic Disorders, Reading Difficulties, Teaching Methods
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Hodnett, Jennifer; Scheithauer, Mindy; Call, Nathan A.; Mevers, Joanne Lomas; Miller, Sarah J. – American Journal on Intellectual and Developmental Disabilities, 2018
Smith-Magenis syndrome (SMS) is a genetic disorder, commonly caused by a 17p11.2 deletion, affecting the Retinoic Acid Induced 1 gene. It affects approximately 1 in 25,000 individuals, with over 90% engaging in challenging behaviors. Function-based treatments, using the principles of applied behavior analysis, have consistently been shown to…
Descriptors: Functional Behavioral Assessment, Behavior Problems, Genetic Disorders, Developmental Delays
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Zyga, Olena; Russ, Sandra W.; Dimitropoulos, Anastasia – American Journal on Intellectual and Developmental Disabilities, 2018
Research has shown that children with Prader-Willi syndrome (PWS) have social-cognitive challenges and decreased quality parent-child interactions. However, given the low prevalence rate, developing interventions for children with PWS is faced with the significant challenge of enrolling enough participants for local studies. To better understand…
Descriptors: Genetic Disorders, Intellectual Disability, Interpersonal Competence, Parent Child Relationship
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Robinson, Marissa; Klusek, Jessica; Poe, Michele D.; Hatton, Deborah D.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2018
Effortful control, or the ability to suppress a dominant response to perform a subdominant response, is an early-emerging temperament trait that is linked with positive social-emotional development. Fragile X syndrome (FXS) is a single-gene disorder characterized by hallmark regulatory impairments, suggesting diminished effortful control. This…
Descriptors: Genetic Disorders, Congenital Impairments, Intellectual Disability, Males
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Pérez-García, Débora; Brun-Gasca, Carme; Pérez-Jurado, Luis A.; Mervis, Carolyn B. – American Journal on Intellectual and Developmental Disabilities, 2017
To identify similarities and differences in the behavioral profile of children with Williams syndrome from Spain (n = 53) and the United States (n = 145), we asked parents of 6- to 14-year-olds with Williams syndrome to complete the Child Behavior Checklist 6-18. The distribution of raw scores was significantly higher for the Spanish sample than…
Descriptors: Foreign Countries, Child Behavior, Genetic Disorders, Children
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Chan, Wai; Smith, Leann E.; Greenberg, Jan S.; Hong, Jinkuk; Mailick, Marsha R. – American Journal on Intellectual and Developmental Disabilities, 2017
The present investigation explored long-term relationships of behavioral symptoms of adolescents and adults with developmental disabilities with the mental health of their mothers. Fragile X premutation carrier mothers of an adolescent or adult child with fragile X syndrome (n = 95), and mothers of a grown child with autism (n = 213) were…
Descriptors: Behavior Problems, Adolescents, Adults, Genetic Disorders
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Kidd, Sharon A.; Raspa, Melissa; Clark, Renée; Usrey-Roos, Holly; Wheeler, Anne C.; Liu, Jessica A.; Wylie, Amanda; Sherman, Stephanie L. – American Journal on Intellectual and Developmental Disabilities, 2017
The objectives were to describe the demographic characteristics of children with Fragile X syndrome (FXS) and to determine predictors of attendance at Fragile X (FX) clinics. Findings from the Community Support Network (CSN) and Our Fragile X World (OFXW) samples showed that children who attended FX Clinics were mostly male, high-school aged or…
Descriptors: Genetic Disorders, Predictor Variables, Individual Characteristics, Participation
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Reisinger, Debra L.; Roberts, Jane E. – American Journal on Intellectual and Developmental Disabilities, 2017
Social skills are critical for academic, social, and psychological success of children with both typical and atypical development. Boys with fragile X syndrome (FXS) are at high risk for social skill impairments, given intellectual impairments and secondary conditions. The present study examines the impact of adaptive behavior, autism symptoms,…
Descriptors: Anxiety, Autism, Symptoms (Individual Disorders), Males
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McDuffie, Andrea; Machalicek, Wendy; Bullard, Lauren; Nelson, Sarah; Mello, Melissa; Tempero-Feigles, Robyn; Castignetti, Nancy; Abbeduto, Leonard – American Journal on Intellectual and Developmental Disabilities, 2016
Using a single case design, a parent-mediated spoken-language intervention was delivered to three mothers and their school-aged sons with fragile X syndrome, the leading inherited cause of intellectual disability. The intervention was embedded in the context of shared storytelling using wordless picture books and targeted three empirically derived…
Descriptors: Intervention, Speech Communication, Genetic Disorders, Intellectual Disability
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Robinson, Ashley N.; Roberts, Jane E.; Brady, Nancy C.; McQuillin, Samuel D.; Warren, Steven F. – American Journal on Intellectual and Developmental Disabilities, 2016
The present study examined the relationship between salivary cortisol and maternal responsiveness in mothers of boys with fragile X syndrome (FXS). Maternal responsivity is strongly associated with child outcomes, and children with FXS are at risk for compromised development due to intellectual disability and problem behavior. Increased…
Descriptors: Correlation, Metabolism, Mothers, Responses
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Pitts, C. Holley; Mervis, Carolyn B. – American Journal on Intellectual and Developmental Disabilities, 2016
We describe the performance of 292 4- to 17-year-olds with Williams syndrome (WS) on the Kaufman Brief Intelligence Test-2 (KBIT-2; Kaufman & Kaufman, 2004). Mean IQ Composite, Verbal standard score (SS), and Nonverbal SS were in the borderline range relative to the general population, with variability similar to the general population.…
Descriptors: Intelligence Tests, Genetic Disorders, Intelligence Quotient, Children
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