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Journal of Intellectual Disability Research: JIDR

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https://read.qxmd.com/read/30767315/molecularly-confirmed-kabuki-niikawa-kuroki-syndrome-patients-demonstrate-a-specific-cognitive-profile-with-extensive-visuospatial-abnormalities
#1
J Harris, E M Mahone, H T Bjornsson
BACKGROUND: Kabuki (Niikawa-Kuroki) syndrome (KS) is caused by disease-causing variants in either of two components (KMT2D and KDM6A) of the histone methylation machinery. Nearly all individuals with KS have cognitive difficulties, and most have intellectual disability. Recent studies on a mouse model of KS suggest disruption of normal adult neurogenesis in the granule cell layer of the dentate gyrus of the hippocampus. These mutant mice also demonstrate hippocampal memory defects compared with littermates, but this phenotype is rescued postnatally with agents that target the epigenetic machinery...
February 14, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30767307/relationship-of-functional-reach-test-scores-and-falls-in-special-olympics-athletes
#2
J Thomas, S O'Neal
BACKGROUND: The Functional Reach Test is used to assess balance of Special Olympic athletes as part of the FUNfitness screens held at Special Olympics games. However, it is unknown if the Functional Reach Test is an accurate assessment of balance in this population. The objective of this study was to explore the relationship between Functional Reach Test scores and falls in Special Olympic athletes. METHODS: Data were collected as a part of the FUNfitness screens during Arizona Special Olympics games...
February 14, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30747463/fragile-x-syndrome-clinical-trials-exploring-parental-decision-making
#3
C S D'Amanda, H L Peay, A C Wheeler, E Turbitt, B B Biesecker
BACKGROUND: The objective of this research was to understand parental proxy decision-making for drug trial participation for children with Fragile X syndrome (FXS). Specifically, we aimed to capture preferences, motivations, influencing factors and barriers related to trial involvement among trial joiners and decliners and describe ease of trial decision-making and decisional regret. METHODS: Interviews were conducted with parents from two groups: those who chose to enrol their child with FXS in a trial (N = 16; Joiners) and those who declined trial participation (N = 15; Decliners)...
February 12, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30747460/learning-new-relational-categories-by-children-with-autism-spectrum-disorders-children-with-typical-development-and-children-with-intellectual-disabilities-effects-of-comparison-and-familiarity-on-systematicity
#4
O E Hetzroni, M Hessler, K Shalahevich
BACKGROUND: Systematicity principle, used during analogical reasoning, enables building up deeper abstract concepts as part of structure mapping. The purpose of this study was to investigate structure mapping processes that occur during acquisition of new relational categories and to identify the learning patterns and systematicity of children with autism spectrum disorder (ASD) compared with intellectual and developmental disabilities (IDD) and typical development (TD). Comparison effect and level of familiarity were used to investigate structural mapping processes...
February 12, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30734976/hospital-readmissions-among-older-people-with-intellectual-disability-in-comparison-with-the-general-population
#5
A Axmon, M Björkman, G Ahlström
BACKGROUND: Older people with intellectual disability have high multimorbidity and poor physical and mental health compared with the general population. Consequently, they have a greater need for health care. Hospital readmissions may be an indicator of the quality of health care. However, so far, only a few studies have investigated this outcome in populations of people with intellectual disability. None has focused on older people. METHOD: We identified a cohort of people with intellectual disability aged 55+ years and alive at the end of 2012 (n = 7936)...
February 8, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30734385/exploring-the-approximate-number-system-in-sotos-syndrome-insights-from-a-dot-comparison-task
#6
C Lane, J Van Herwegen, M Freeth
BACKGROUND: Sotos syndrome is a congenital overgrowth condition associated with intellectual disability and an uneven cognitive profile. Previous research has established that individuals with Sotos syndrome have relatively poor mathematical ability, but domain-specific numeracy skills have not been explored within this population. This study investigated the approximate number system (ANS) in Sotos syndrome. METHOD: A dot comparison task was administered to 20 participants with Sotos syndrome (mean age in years = 18...
February 7, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30724417/exploring-the-cognitive-phenotype-of-kabuki-niikawa-kuroki-syndrome
#7
L C M van Dongen, P A M Wingbermühle, W M van der Veld, C Stumpel, T Kleefstra, J I M Egger
BACKGROUND: Kabuki syndrome (KS) is a Mendelian disorder, characterised by short stature, facial dysmorphisms and developmental delay and/or intellectual disability. Clarification of the neurocognitive profile in KS may provide directions for education and treatment interventions for KS. Previous studies on cognitive functioning in KS are scarce and have mainly focused on the general level of intelligence. The few more extensive studies suggested weaknesses in language skills, visuoconstruction, perceptual reasoning and speed of information processing...
February 6, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30697858/the-association-of-life-events-and-mental-ill-health-in-older-adults-with-intellectual-disability-results-of-the-wave-3-intellectual-disability-supplement-to-the-irish-longitudinal-study-on-ageing
#8
L Bond, R Carroll, N Mulryan, M O'Dwyer, J O'Connell, R Monaghan, F Sheerin, P McCallion, M McCarron
BACKGROUND: Exposures to life events are associated with emotional, psychological and behavioural problems in those with intellectual disability (ID). Older adults with ID may experience different life events given differences in living circumstances, cognitive decline, greater dependency on others and less autonomy. This study examines the relationship of life events and mental ill health in an older ID population in Ireland. METHODS: The study was part of 'The Intellectual Disability Supplement to The Irish Longitudinal Study on Ageing'...
January 30, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30687997/dual-task-effects-on-postural-sway-during-sit-to-stand-movement-in-children-with-down-syndrome
#9
G M Pena, S L Pavão, M F P Oliveira, D Godoi, A C de Campos, N A C F Rocha
BACKGROUND: Cognitive and postural tasks require common cognitive mechanisms, resulting in conflicts when both tasks are simultaneously performed. The presence of neuromotor dysfunctions, such as Down syndrome, may impair coordination processes required to perform dual-tasks. The objective of this study was to investigate the dual-task effects on postural sway during sit-to-stand movements in typical children and children with Down syndrome in a cross-sectional study. METHODS: Twenty six typical children (10...
January 28, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30687982/effects-of-mindfulness-coping-styles-and-resilience-on-job-retention-and-burnout-in-caregivers-supporting-aggressive-adults-with-developmental-disabilities
#10
R E Nevill, S M Havercamp
BACKGROUND: Considering the growing body of studies investigating the effects of mindfulness-based interventions on caregivers supporting people with developmental disabilities, the current study aimed to explore the role that the cognitive processes of mindfulness, coping style and resilience played in predicting caregiver retention and burnout among a sample of direct support professionals working with aggressive adults with developmental disabilities. METHODS: Ninety-seven direct support professionals were surveyed to determine level of mindfulness, coping styles, resilience and burnout and were interviewed 3 months later to determine if they were still working with the aggressive adult...
January 27, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30675741/effects-of-the-neuromuscular-bandage-as-rehabilitative-treatment-of-patients-with-drooling-and-intellectual-disability-an-interventional-study
#11
M Lorca Larrosa, J A Ruiz Roca, M I Ruiz Roca, P López-Jornet
BACKGROUND: The aim of this work was to assess the effect of neurological bandages (Kinesio Taping) for managing saliva flow in patients with drooling and intellectual disability. METHODS: Quasi-experimental study included 30 patients (20 male and 10 female participants) mean age of 15 years with intellectual disability and drooling [Public Special Education Centre in Cartagena (Murcia, Spain)]. Treatment consisted of the application of a strip of neuromuscular bandage applied in the suprahyoid area for a 3-month period...
January 23, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30663148/development-and-psychometric-properties-of-the-attitudes-toward-intellectual-disability-questionnaire-short-form
#12
D Morin, P Valois, A G Crocker, C Robitaille
No abstract text is available yet for this article.
January 20, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30637858/using-z-and-age-equivalent-scores-to-address-wisc-iv-floor-effects-for-children-with-intellectual-disability
#13
E Toffalini, S Buono, T Zagaria, A Calcagnì, C Cornoldi
BACKGROUND: The Wechsler Intelligence Scale for Children - Fourth Edition often produces floor effects in individuals with intellectual disability. Calculating respondents' Z or age-equivalent scores has been claimed to remedy this problem. METHOD: The present study applied these methods to the Wechsler Intelligence Scale for Children - Fourth Edition scores of 198 individuals diagnosed with intellectual disability. Confirmatory factor analysis and profile analysis were conducted using a Bayesian approach...
January 13, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30628132/adults-with-down-syndrome-a-comprehensive-approach-to-manage-complexity
#14
A Carfì, D L Vetrano, D Mascia, E Meloni, E R Villani, N Acampora, V Brandi, B E Fries, L Martin, R Bernabei, G Onder
BACKGROUND: Down syndrome (DS) is characterised by premature ageing that affects selected organ systems, and persons with this condition can present patterns of co-morbidities and deficits often observed in the older population without DS. However, information on the characteristics of adult persons with DS is limited. The objective of the study is to describe characteristics of adults with DS collected with a standardised, comprehensive assessment instrument. METHODS: Cross-sectional study...
January 9, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30628126/developing-behavioural-indicators-for-intellectual-functioning-and-adaptive-behaviour-for-icd-11-disorders-of-intellectual-development
#15
M J Tassé, G Balboni, P Navas, R Luckasson, M A Nygren, C Belacchi, S Bonichini, G M Reed, C S Kogan
BACKGROUND: We present the work conducted to arrive at deriving behavioural indicators that could be used to guide clinical judgement in determining the presence and severity of deficits in intellectual functioning and adaptive behaviour for the purpose of making a diagnosis of disorders of intellectual development. METHODS: An interdisciplinary expert panel provided guidance in developing behavioural indicators for intellectual functioning. A national dataset of adaptive behaviour on a sample of individuals with a diagnosis of intellectual disability was used to develop the behavioural indicators for the adaptive behaviour...
January 9, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30628125/interventions-for-sleep-difficulties-in-adults-with-an-intellectual-disability-a-systematic-review
#16
REVIEW
P J Shanahan, S Palod, K J Smith, C Fife-Schaw, N Mirza
BACKGROUND: Current literature highlights higher prevalence rates of sleep difficulties amongst adults with an intellectual disability. However, no synthesis has been conducted to assess the effectiveness of existing interventions in this population. Thus, the aim of this review was to assess the effectiveness of sleep interventions in adults with an intellectual disability (ID). METHOD: Eight databases were searched to identify interventions for sleep difficulties amongst adults with an ID...
January 9, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30628119/prejudices-towards-people-with-intellectual-disabilities-reliability-and-validity-of-the-italian-modern-and-classical-prejudices-scale
#17
R Marcone, A Caputo, S Esposito, V P Senese
BACKGROUND: Prejudices and negative attitudes towards intellectual disabilities (IDs) may hinder social inclusion of ID individuals, limiting their well-being. This study investigated the psychometric characteristics of the Italian Modern and Classical Prejudices Scale (MCPS-IT) towards people with ID and the effects of gender, age and socio-economic status (SES) on prejudices. METHOD: The MCPS-IT was administered to 474 adults (69% women, age range 18-70 years, M = 33...
January 9, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30609165/feasibility-of-a-dance-and-exercise-with-music-programme-on-adults-with-intellectual-disability
#18
D Martínez-Aldao, I Martínez-Lemos, S Bouzas-Rico, C Ayán-Pérez
BACKGROUND: Research regarding the feasibility and effects of dancing and exercise with musical support programmes on the physical fitness of adults with intellectual disability (ID) is scarce. The purpose of this study was to provide scientific evidence regarding the feasibility of a training programme consisting of dancing and exercise with music designed for adults with ID, as well as to assess its impact on their body composition and cardiovascular and muscular fitness. METHODS: A total of 30 adults (mean age 36...
January 4, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30609156/adverse-drug-event-related-hospitalisation-in-persons-with-neurodevelopmental-disorders-a-state-wide-retrospective-cohort-study
#19
M Zhou, W Du, L Salvador-Carulla, N Glasgow
BACKGROUND: Little is known about the sociodemographic and clinical characteristics of adverse drug events (ADEs) in patients with neurodevelopmental disorders (NDD). OBJECTIVE: The objective of this study was to describe and compare the demographic details of people with and without NDD hospitalised due to ADEs. METHODS: The all-inclusive New South Wales Admitted Patient Data Collection from 2001 to 2014 was employed to identify ADE-related hospitalisations in patients with NDD using the International Classification of Diseases 10th revision Australian modification codes...
January 4, 2019: Journal of Intellectual Disability Research: JIDR
https://read.qxmd.com/read/30609152/is-risperidone-effective-in-reducing-challenging-behaviours-in-individuals-with-intellectual-disabilities-after-1-year-or-longer-use-a-placebo-controlled-randomised-double-blind-discontinuation-study
#20
L Ramerman, G de Kuijper, T Scheers, M Vink, P Vrijmoeth, P J Hoekstra
BACKGROUND: Many people with intellectual disabilities use risperidone long term for the management of challenging behaviours, despite its limited proof of effectiveness and its clear association with adverse events. Therefore, this study aimed to investigate the effectiveness of ongoing treatment with risperidone in reducing challenging behaviours versus controlled discontinuation on behaviour and health parameters. METHOD: This was a placebo-controlled, double-blind, randomised discontinuation trial of risperidone...
January 4, 2019: Journal of Intellectual Disability Research: JIDR
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