Caregiver-reported barriers to care for children and adults with Williams Syndrome.

Individuals with Williams syndrome (WS) may experience a variety of medical, behavioral, and educational concerns. The primary objective of this study was to assess barriers to health care for patients with WS, primarily using the Barriers to Care Questionnaire (BCQ), and to assess whether various demographic factors are correlated with these barriers. A REDCap survey was distributed using the Williams Syndrome Association Research Registry. 319 caregivers of individuals with WS in the United States completed the BCQ. On the BCQ, lower scores indicate more barriers to care. Younger age was associated with lower scores for both the pragmatics and the skills subscales while lower income levels and increased distances to providers knowledgeable about WS were consistently associated with lower total BCQ scores.

Impact of an Asynchronous Training for the Early Intervention and Childcare Workforce Addressing the Developmental Impact of the Opioid Crisis on Young Children.

OBJECTIVES: The US opioid epidemic contributes to a growing population of children experiencing neonatal abstinence syndrome (NAS) and adverse childhood experiences (ACEs). A review of the developmental impacts of the opioid crisis highlights that both prenatal exposure to teratogens and ACEs can result in developmental delay and disabilities. Training for the early intervention/early childhood (EI) systems is needed to enable them to meet the needs of this growing population. METHODS: To address this, an IRB-approved online training on best practices for NAS, developmental monitoring and referral, and trauma-informed care was created for Ohio EI providers who provided informed consent to participate. The feasibility of utilizing an online training was assessed. Knowledge on opioid addiction, NAS, ACEs, and early intervention provider characteristics were collected for 2973 participants. RESULTS: Within 6 months, the training reached providers in all Ohio counties and seventeen other states. 57% of providers reported caring for one or more children with a caregiver who has confirmed opioid use. 31% reported these children had experienced four or more ACEs. Providers' ACEs awareness was moderately associated with their experiences with prenatally-exposed youth. There was a significant increase in knowledge following training. Differences in post-training knowledge differed only by county-level opioid death rates, where those providers with low-medium opioid death rates reported more awareness of children with prenatal opioid exposure compared to participants who lived in a county with medium and medium-high opioid death rates. CONCLUSIONS: Online-training is feasible for closing gaps in the early intervention system.

Supporting the transition to senior pediatric resident: an interactive online resource.

Transitions to roles with increasing clinical responsibility and independence can be anxiety provoking for many trainees. To support our pediatric residents preparing to transition from the role of junior to senior resident, we developed a novel asynchronous online interactive case-based resource. Our experience suggests that this resource was voluntarily utilized and can effectively increase trainee confidence and reduce anxiety about this key transitional stage. We suggest that interactive case-based resources can be utilized as supplemental support for residents preparing for residency transitions in other programs and settings.

La transition vers des rôles comportant des responsabilités cliniques et une autonomie plus importante peut provoquer de l'anxiété pour de nombreux stagiaires. Pour soutenir nos résidents en pédiatrie qui se préparent à passer du rôle de résident junior à celui de résident senior, nous avons créé une nouvelle ressource interactive en ligne, asynchrone, qui est basée sur une étude de cas. Notre expérience suggère que cette ressource a été utilisée sur une base volontaire et qu'elle peut effectivement accroître la confiance des stagiaires et réduire l'anxiété liée à cette étape clé de leur parcours. Nous estimons que les ressources interactives basées sur des cas peuvent être utilisées à titre complémentaire pour aider les résidents à préparer leurs transitions dans d'autres programmes et contextes de résidence.

Impact of social pediatrics rotation on residents' understanding of social determinants of health.

Social Pediatrics is the newest mandatory rotation in the General Pediatrics residency program at the University of Alberta. Evaluation of the residents include a written reflective assignment, asking them to identify assets and disparities that have influenced the health of a child encountered on the rotation. While there are many published papers on reflective writing , few papers are found in the area of how social determinants of health (SDoH) impact an individual's overall health. This study examines the question: how has exploring SDoH during the Social Pediatrics rotation led to changes in residents' awareness of their own practice of pediatrics? Grounded theory was used to analyse 35 reflections from residents who had submitted them as a mandatory assignment at the end of their rotation. In addition, 10 semi-structured telephone interviews were conducted to further understand residents' perceptions. Interviews were transcribed verbatim and analysis of the reflections and interviews was guided by grounded theory using open, axial, and selective coding. Analyses of written reflections revealed the following categories: 1) judgment/bias, 2) systemic challenges, 3) advocacy, and 4) a sense that everyone is doing their best. Interview data reinforced overlapping categories of bias, systemic challenges and advocacy in addition to two new categories: 1) increased exposure and knowledge of specific disadvantaged populations, and 2) understanding impact of SDoH on overall health.Categories that were generated highlight the importance of residents' education regarding the role of SDoH on overall health and management plans. They became aware of structural determinants of health working with health-care professionals who were advocates for the communities they worked with. Analysis of residents' written reflection assignments and follow-up interviews revealed the value of reflective practice in physician development and reinforced the benefit of fostering experiences not typically encountered in traditional clinical learning environments.

An Initiative to Increase Residency Program Diversity.

A physician workforce that reflects the patient population is associated with improved patient outcomes and promotes health equity. Notwithstanding, racial and ethnic disparities persist within US medical schools, making some individuals underrepresented in medicine (URM). We sought to increase the percentage of URM residents who matched into our pediatric residency programs from a baseline of 5% to 35% to achieve demographic parity with our patients. We developed a multifaceted approach using multiple iterative tests of change, with the primary strategy being increased visibility of URM trainees and faculty to residency applicants. Strategies included applicant interviews with URM faculty, interview dinners with URM residents, visibility at academic conferences for URM trainees, development of targeted marketing materials, and a visiting student program supported by networking with URM residents. The primary outcome measure was the percentage of matched residents in the categorical pediatrics, child neurology, and medical genetics training programs who identified as URM. The percentage of URM residents increased to 16% (6 of 37) in 2018, 26% (11 of 43) in 2019, 19% (8 of 43) in 2020, and 21% (9 of 43) in 2021 (a four-year average of 22% URM residents; P = .0002). This progress toward a more representative residency program was met by challenges, such as pipeline concerns, the minority tax, and recruitment during a pandemic. We were able to implement small, low-resource strategies that had a large cumulative impact and could be implemented in other residency programs. Specific tactics and challenges encountered are discussed in this special article.

Brief Report: Evaluating the Effectiveness of Music to Obtain More Accurate Blood Pressure Readings in Children with Williams Syndrome.

This study examined if listening to music will improve the accuracy of blood pressure (BP) readings in children with Williams syndrome (WS). Fifty-two participants (7-12 years) were randomly assigned to a music or non-music group. BPs were obtained at two time points. There was a significant decrease in both systolic and diastolic BP from Time 1 to Time 2 for everyone. Participants from the music group had lower systolic BP readings at Time 2 than participants in the non-music group (Cohen's d = 0.33). Systolic BP readings were approximately 3.8 mmHg lower in the music group. Music may be beneficial in obtaining more accurate systolic BP readings in children with WS.

Health Disparities and Their Effects on Children and Their Caregivers During the Coronavirus Disease 2019 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has uncovered long-standing health disparities in marginalized communities, including racial and ethnic minorities and children with underlying medical and social problems. African Americans, Hispanics, and Native Americans have higher rates of COVID-19 infections and deaths than their population percentages in the United States. Unique populations of children, including children with developmental disabilities, children in the foster care system, children with chronic medical problems, and children who are homeless are particularly vulnerable to COVID-19 infection. This article explores how the COVID-19 pandemic superimposed on health disparities directly and indirectly affects children, adolescents, and their caregivers.

The Effect of the Low FODMAP Diet on Gastrointestinal Symptoms, Behavioral Problems and Nutrient Intake in Children with Autism Spectrum Disorder: A Randomized Controlled Pilot Trial.

Some research suggests that GI symptoms seen in children with ASD may relate to behavior problems. The objective of this pilot study was to assess the effect of the low fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAP) diet on GI and behavioral problems in children with ASD. At follow-up, the low FODMAP diet group had significant relief in some GI problems compared with both baseline in the group and control group. At baseline and at follow-up, there were no significant differences in behavioral problems between the low FODMAP diet group and the control group. Randomized controlled studies including larger sample sizes are needed to confirm the effects of low FODMAP diets in children with autism who have gastrointestinal problems.

The Medicago truncatula Vacuolar iron Transporter-Like proteins VTL4 and VTL8 deliver iron to symbiotic bacteria at different stages of the infection process.

The symbiotic relationship between legumes and rhizobium bacteria in root nodules has a high demand for iron, and questions remain regarding which transporters are involved. Here, we characterize two nodule-specific Vacuolar iron Transporter-Like (VTL) proteins in Medicago truncatula. Localization of fluorescent fusion proteins and mutant studies were carried out to correlate with existing RNA-seq data showing differential expression of VTL4 and VTL8 during early and late infection, respectively. The vtl4 insertion lines showed decreased nitrogen fixation capacity associated with more immature nodules and less elongated bacteroids. A mutant line lacking the tandemly-arranged VTL4-VTL8 genes, named 13U, was unable to develop functional nodules and failed to fix nitrogen, which was almost fully restored by expression of VTL8 alone. Using a newly developed lux reporter to monitor iron status of the bacteroids, a moderate decrease in luminescence signal was observed in vtl4 mutant nodules and a strong decrease in 13U nodules. Iron transport capability of VTL4 and VTL8 was shown by yeast complementation. These data indicate that VTL8, the closest homologue of SEN1 in Lotus japonicus, is the main route for delivering iron to symbiotic rhizobia. We propose that a failure in iron protein maturation leads to early senescence of the bacteroids.

A Blended Curriculum to Improve Resident Physical Exam Skills for Patients With Neuromuscular Disability.

Introduction: Children with neuromuscular disabilities (NMD) receive care in a wide variety of clinical settings. Residents lack training to develop physical examination skills for evaluating patients with NMD. We devised a curriculum to teach residents how to examine patients with NMD using a systematic and simplified approach. Methods: Creation of this resource was a response to a survey of final-year residents that revealed the need for education focused on developing physical examination skills. The curriculum has four components-multimedia PowerPoint with embedded video, knowledge assessment, clinical exam (CEX) assessment, and module feedback-and was completed by 37 residents over an 8-month period from January to September 2016. We utilized knowledge assessment, direct clinical skills observation using the CEX, and module-feedback responses as part of the evaluation. Results: All 37 residents completed the curriculum, with an overall knowledge score of greater than 80%. Residents demonstrated most of the desired patient care behaviors on the CEX assessment and provided positive feedback on the quality, usefulness, and applicability of the module, in addition to requesting more curricula to develop their physical examination skills. Discussion: The CEX assessment provided a unique opportunity for faculty feedback on residents' physical exam performance. After completing the module, residents achieved high scores in most areas of the standardized CEX and were able to conduct the NMD physical exam in a sensitive manner. The assessment highlighted the need to improve residents' skills of detecting abnormal clinical findings and communicating with the patient during the physical exam.

Ventilator Boot Camp Improves the Knowledge and Skills Associated With Mechanical Ventilator Use During Interfacility Transport of Intubated Pediatric Patients.

BACKGROUND: The American Academy of Pediatrics Section on Transport recommends the use of portable ventilators during the transport of patients with advanced airways. We sought to identify knowledge gaps and evaluate the effectiveness of a transport ventilator competency boot camp. METHODS: Electronic health records of children requiring ventilatory support during air and ground interfacility transport from January 1 through December 31, 2015, were reviewed to determine when manual ventilation was used in lieu of a portable ventilator, and simulations were constructed from commonly occurring scenarios. All registered respiratory therapists trained in air and ground critical-care transports participated. Demographic data were collected. We assessed performance on 3 facilitated simulated scenarios using a ventilator connected to a low-fidelity pediatric mannequin attached to breathing simulator. Scores were based on the participants' ability to correctly perform pre-use checks, select and optimize ventilator settings, set alarms, and complete safety checks. A 60-min interactive education intervention was conducted between the pre- and post-assessments. The pre-assessment, intervention, and post-assessment were conducted 6 weeks apart. De-identified assessments were scored, and results were shared after study completion. Descriptive statistics reported participant demographics. Paired t tests compared before and after assessments. Statistical significance was established at P < .05. RESULTS: A total of 172 electronic health records were reviewed. Manual ventilation was used more frequently in toddlers requiring pressure control ventilation; noninvasive ventilation was rarely used. A total of 17 registered respiratory therapists participated; 18% were male, 41% had 6-9 years of tenure and 5 years of experience with our transport team. Completing ventilator pre-use check and engaging alarms provided the most opportunity for improvement. Improvements were greater with the use of noninvasive ventilation (P = .006) than pressure control ventilation (P = .10) and volume control ventilation use (P = .07). CONCLUSIONS: Quality data were useful in identifying areas requiring knowledge and competency assessment. Re-assessment results validated the need to conduct education and competency assessment at defined intervals.

Learning in a Web-Based World: An Innovative Approach to Teach Physical Examination Skills in Patients with Neurodisability.

Despite increasing numbers of patients with neurodisability, residents lack training to develop physical examination skills. Following a blended educational intervention combining online and bedside teaching, residents demonstrated desired patient-care behaviors on standardized clinical exam assessment.

The proceedings of the 15th professional conference on Williams Syndrome.

Williams Syndrome (WS) is a contiguous gene deletion disorder, caused by the deletion of approximately 26-28 genes from chromosome 7 (7q11.23). Individuals with WS have complex medical, developmental, and behavioral features, requiring multidisciplinary and interdisciplinary collaboration. Guidelines detailing the identification, evaluation, and monitoring of individuals with WS need clarification, especially for primary care providers who are first-line in their management. This report summarizes the proceedings of the 2016 Professional Conference on WS in Columbus, OH. Presentations were directed towards primary care providers and subspecialists, emphasizing evidence-based practices for treating the prevalent medical and behavioral features of WS. Included in this report are findings from a panel of cardiovascular experts discussing three case studies on treatment of hypertension and the use of sedation or anesthesia for non-cardiac procedures. Abstracts from individual expert presenters are included, covering various medical and behavioral topics, and providing updates in management of WS individuals. The following topics were discussed: differences in phenotypes of 7q11.23 deletion versus duplication, growth parameters, endocrine concerns, sleep difficulties, behaviors to monitor, and pharmacological options, the neurodevelopmental profile of WS individuals, and the importance of monitoring medical and behavioral concerns as WS individuals transition to adulthood.

What's on YOUR Facebook profile? Evaluation of an educational intervention to promote appropriate use of privacy settings by medical students on social networking sites.

BACKGROUND: The rise of social media has led to growing concerns about the potential implications of 'unprofessional' postings by physicians and medical students on individuals, institutions, and the medical profession. Relevant and effective guidelines have been difficult to develop and enforce, and there is a need for students and physicians to consider how their online activities may be perceived in the context of their professional roles. The purpose of this project was to examine the Internet presence of a graduating Canadian medical school class by scanning students' public profiles on the social media site Facebook, incorporate this information into an educational activity addressing professionalism and social media, and evaluate the impact of this activity on student behavior. METHODS: A systematic search for public Facebook profiles of each member of the class was conducted, and data were collected on the types of publicly visible material. These were presented as part of an educational session on social media and professionalism. One month later, the Facebook search was repeated. RESULTS: Of 152 students in the class, profiles were found for 121 (79.8%). The majority of students used appropriately restrictive privacy settings; however, a significant minority had publicly visible information, including comments, photographs, location, and status as a medical student. The educational innovation was well received with more than 90% of students agreeing that this topic was important and well addressed. A follow-up search found that many students had altered their privacy settings to make less information publicly available. CONCLUSIONS: A small but significant proportion of students share potentially unprofessional content on social media. An interactive educational intervention, which includes specific disclosure of how participants appear to others on social media, resulted in a significant change in student behavior.

Bronchiolitis: Recommendations for diagnosis, monitoring and management of children one to 24 months of age.

Bronchiolitis is the most common reason for admission to hospital in the first year of life. There is tremendous variation in the clinical management of this condition across Canada and around the world, including significant use of unnecessary tests and ineffective therapies. This statement pertains to generally healthy children ≤2 years of age with bronchiolitis. The diagnosis of bronchiolitis is based primarily on the history of illness and physical examination findings. Laboratory investigations are generally unhelpful. Bronchiolitis is a self-limiting disease, usually managed with supportive care at home. Groups at high risk for severe disease are described and guidelines for admission to hospital are presented. Evidence for the efficacy of various therapies is discussed and recommendations are made for management. Monitoring requirements and discharge readiness from hospital are also discussed.

La bronchiolite est la principale cause d'hospitalisation avant l'âge de un an. La prise en charge clinique de cette maladie varie considérablement selon les régions du Canada et du monde, y compris une grande utilisation de tests inutiles et de thérapies inefficaces. Le présent document de principes porte sur des enfants en santé de deux ans ou moins qui sont atteints d'une bronchiolite. Le diagnostic de bronchiolite repose d'abord sur l'anamnèse de la maladie et sur les résultats de l'examen physique. En général, les examens de laboratoire sont inutiles. La bronchiolite est une maladie spontanément résolutive, qui est généralement prise en charge par des soins de soutien à domicile. Par ailleurs, les groupes très vulnérables à une bronchiolite grave sont décrits, et les indications d'admission à l'hôpital sont présentées. Les données probantes sur l'efficacité des diverses thérapies et les recommandations de prise en charge sont exposées. La surveillance requise et le moment du congé de l'hôpital sont également abordés.