91 results match your criteria: "Kennedy Krieger Institute and Johns Hopkins University School of Medicine[Affiliation]"

Aim: To examine neurobehavioral findings in three genetic syndromes (PTEN hamartoma tumor syndrome, Malan syndrome [mutations in the NFIX gene], and SYNGAP1-related disorder), a mixed group of other neurodevelopmental genetic syndromes (NDGS), idiopathic neurodevelopmental disorder, and neurotypical control participants.

Method: Using a longitudinal case-control design, caregivers reported neurobehavioral information for 498 participants (PTEN hamartoma tumor syndrome n = 112, Malan syndrome n = 24, SYNGAP1-related disorder n = 47, other NDGS n = 72, idiopathic neurodevelopmental disorder n = 54, neurotypical siblings n = 74, and unrelated neurotypical control participants n = 115) at three timepoints (baseline, and 1-month and 4-month follow-ups) using the online-administered Neurobehavioral Evaluation Tool (NET).

Results: NET scales had good scale and test-retest reliability.

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Temperamental characteristics and emerging cognitive control are meaningful predictors of children's development of adaptive and maladaptive social behaviors during the preschool period. However, knowledge of the interplay of these pathways, when examined concurrently to highlight their individual contributions, is limited. Using a cross-sectional sample of 3-year-old children, we examined parent-reported discrete traits of negative (anger, fear, sadness, and shyness) and positive (low- and high-intensity pleasure) temperamental reactivity as predictors of children's prosociality and physical aggression.

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We discuss the use of two empirically validated behavior-change methods-checklists and goal setting-and designed a checklist to assist behavior analysts in improving their behavioral services to be more culturally responsive and trauma informed. We also present pilot data evaluating the use of the checklist and goal setting on the inclusion of culturally responsive and trauma-informed practices in behavior support plans designed for students in a public school. The training package was effective for both participants, and the participants' weekly goals corresponded to the observed changes in their behavior plans.

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The effects of question previewing on response accuracy and text processing: An eye-movement study.

J Sch Psychol

June 2024

Department of Psychology, Mount Holyoke College, 50 College St., South Hadley, MA 01075, USA. Electronic address:

The present study investigated the impact of manipulating reading strategies (i.e., reading the questions first [QF] or reading the passage first [PF]) during a reading comprehension test where we explored how reading strategy was related to student characteristics (i.

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Increasing Access to Cardiac Rehabilitation Services Among Disadvantaged Populations.

J Public Health Manag Pract

July 2024

Prince George's County Health Department, Largo, Maryland (Mss Murphy and Stokes and Dr Amuta); Prince George's County Health Department, Largo, and Kennedy Krieger Institute and Johns Hopkins University School of Medicine, Baltimore, Maryland (Dr Carter); Independent Consultant, Washington, District of Columbia (Dr Thomas); Totally Linking Care in Maryland, Leonardtown, Maryland (Ms Thomas); Health Quality Innovators, Richmond, Virginia (Ms Lichty-Hess); and Johns Hopkins Center for Health Disparities Solutions, Baltimore, Maryland (Drs Zare and Gaskin).

The Prince George's County Health Department encountered several challenges to increasing access to cardiac rehabilitation (CR) services among disadvantaged populations. They include excessive patient out-of-pocket costs; requirements that CR orders must be signed by a physician; provider reluctance to refer patients to CR, with most primary care providers preferring to refer clients to cardiologists for the latter to determine whether the patient needs CR referral; limited availability of CR programs; and difficulty identifying patients eligible for CR services. Discussions with other local health departments and public health practitioners indicate that these challenges are not unique to Maryland but are indicative of policy and system barriers that prevent the optimal delivery of cardiovascular health services.

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Objective: Dramatic changes in state-level cannabis laws (CL) over the past 25 years have shifted societal beliefs throughout the United States, with unknown implications for youth. In the present study, we conducted an updated systematic review and meta-analysis examining estimated effects of medical cannabis legalization (MCL) and recreational cannabis legalization (RCL) on past-month cannabis use among US youth.

Method: A systematic review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, followed by a subsequent meta-analysis investigating the associations between state-level cannabis laws (ie, MCL vs non-MCL, and RCL vs non-RCL) and past-month cannabis use prevalence among US adolescents and young adults.

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Unlabelled: The Performance Diagnostic Checklist-Human Services (PDC-HS) is a performance analysis tool used to identify barriers to performance in human-service settings. Multiple published studies have used the PDC-HS to determine effective interventions (Wilder et al. (2), 1170-1176, 2020).

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"Toward Equity in Research on Intellectual and Developmental Disabilities" (IDD) is a timely and comprehensive article highlighting gaps in the "dominant culture" approach to current research strategies designed to address IDD. Recentering systems involved in the research enterprise are recommended. This commentary provides additional guidance from a social justice, equity, and inclusion lens, including a clinical anthropology approach to research.

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This study focused on the development and initial psychometric evaluation of a set of online, webcam-collected, and artificial intelligence-derived patient performance measures for neurodevelopmental genetic syndromes (NDGS). Initial testing and qualitative input was used to develop four stimulus paradigms capturing social and cognitive processes, including social attention, receptive vocabulary, processing speed, and single-word reading. The paradigms were administered to a sample of 375 participants, including 163 with NDGS, 56 with idiopathic neurodevelopmental disability (NDD), and 156 neurotypical controls.

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The Neurobehavioral Programs at Kennedy Krieger Institute constitute a comprehensive continuum of care designed to serve individuals with intellectual and developmental disabilities with co-occurring problem behavior. This continuum includes inpatient, intensive outpatient, outpatient, consultation, and follow-up services. The mission of these programs is to fully integrate patient care, research, training, and advocacy to achieve the best possible outcomes with patients, and to benefit the broader community of individuals with severe behavioral dysfunction.

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Toward an Understanding of Data Collection Integrity.

Behav Anal Pract

December 2022

Department of Psychology, Salve Regina University, 100 Ochre Point Ave, Newport, RI 02840 USA.

Unlabelled: Data collection is an integral part of the practice of behavior analysis because behavior analysts rely on data to inform their clinical decisions. Data collection integrity (DCI) is the degree to which data are collected as planned, and issues with DCI can lead to misinformed clinical decisions. The current study aims to add to the limited research on DCI by evaluating risk factors and interventions that target DCI.

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In an effort to address some of the criticisms of Behavioral Parent Training programs (BPT; high attrition, reliance on caregiver report measures), the current study examined the feasibility, acceptability, and outcome of an intensive behavior treatment program (120-minute sessions for 5 days/week over the course of 2 weeks). Using a changing criterion single case experimental design, 12 children ( child age = 4.9 years) and their primary caregivers completed the 2-week function-based intervention procedure designed to increase children's frustration tolerance via a wait training procedure based on the principles of applied behavior analysis.

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Accumulating evidence from clinical and neuropathological study has identified a number of seemingly disparate associations carrying a predisposition for cerebral palsy (CP). We narratively reviewed clinical studies reporting associations between prenatal and perinatal environmental factors and the risk of developing CP. As expected, some processes with direct central nervous system involvement (e.

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Significant advances have been made in recent years regarding the developmental trajectories of brain circuits and networks, revealing links between brain structure and function. Emerging evidence highlights the importance of developmental trajectories in determining early psychiatric outcomes. However, efforts to encourage crosstalk between basic developmental neuroscience and clinical practice are limited.

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In this study, we propose a two-stage procedure for hypothesis testing, where the first stage is conventional hypothesis testing and the second is an equivalence testing procedure using an introduced empirical equivalence bound (EEB). In 2016, the American Statistical Association released a policy statement on P-values to clarify the proper use and interpretation in response to the criticism of reproducibility and replicability in scientific findings. A recent solution to improve reproducibility and transparency in statistical hypothesis testing is to integrate P-values (or confidence intervals) with practical or scientific significance.

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De novo variants in POLR3B cause ataxia, spasticity, and demyelinating neuropathy.

Am J Hum Genet

January 2021

Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada; Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, M5G 1X8, Canada. Electronic address:

POLR3B encodes the second-largest catalytic subunit of RNA polymerase III, an enzyme involved in transcription. Bi-allelic pathogenic variants in POLR3B are a well-established cause of hypomyelinating leukodystrophy. We describe six unrelated individuals with de novo missense variants in POLR3B and a clinical presentation substantially different from POLR3-related leukodystrophy.

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The coronavirus (SARS-CoV-2) pandemic has had a global impact on lives everywhere and has led to the disruption of, and interference with, virtually every aspect of life. In response, leading experts, political leaders, doctors, and scientists have released guidelines that attempt to prevent and mitigate the exponential rate of infection. The response to these safety recommendations has produced tremendous behavior variability as a society.

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Family involvement in routines that exacerbate and maintain child anxiety is referred to as family accommodation. Family involvement is also related to maintenance of severe problem behavior (SPB) demonstrated by children with intellectual and developmental disabilities (IDD). Family members may deliver attention, tangible items, or remove aversive task demands that produce temporary cessation, or avoidance of SPB, but long-term maintenance.

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The potential applicability of concepts and methods of the paradigm of precision medicine to the field of applied behavior analysis is only beginning to be explored. Both precision medicine and applied behavior analysis seek to understand and classify clinical problems through identification of their causal pathways. Both aim to develop treatments directly targeting those causal pathways, which also requires an understanding of the mechanisms by which treatments produce change (treatment-action pathways).

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Competing stimulus assessments (CSAs) are designed to identify stimuli that, when made freely available, reduce problem behavior. Although CSAs have demonstrated utility, identifying competing stimuli can be difficult for some individuals. The current study describes outcomes from an augmented CSA (A-CSA) for 6 consecutively encountered cases with treatment-resistant subtypes of automatically maintained problem behavior.

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The American Academy of Pediatrics (AAP) recommends that infants spend supervised time in the prone (tummy) position to foster motor development and prevent cranial deformities. However, infants may not tolerate the position, and consequently, caregivers may avoid placing their infants in the prone position. The AAP recommends that caregivers provide toys or interaction during tummy time.

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Article Synopsis
  • The extra copy of chromosome 21 in Down syndrome (DS) affects brain and immune system function, and understanding these changes could help in developing treatments.
  • Recent advancements in genetic and epigenetic research, including the use of new mouse models, are shedding light on how specific genes and chromosomal regions contribute to DS phenotypes.
  • Combining findings from human studies with mouse models helps identify critical genes and reveals how the extra chromosome impacts overall gene regulation, opening avenues for potential therapies.
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White-Sutton syndrome (WHSUS) is a recently-identified genetic disorder resulting from de novo heterozygous pathogenic variants in POGZ. Thus far, over 50 individuals have been reported worldwide, however phenotypic characterization and data regarding the natural history are still incomplete. Here we report the clinical features of 22 individuals with 21 unique loss of function POGZ variants.

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Self-injurious behavior (SIB) is inherently problematic because it can lead to injuries, including those that are quite severe and may result in loss of function or permanent disfigurement. The current study replicated and extended Rooker et al. (2018) by classifying the physical characteristics of injuries across groups of individuals with automatically maintained SIB (ASIB Subtypes 2 and 3) and socially maintained SIB.

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