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In the current study, we examined adaptive skills and trajectories over time in 257 individuals with Angelman syndrome (AS) using the Vineland Adaptive Behavior Scales, 2nd Edition. Multilevel linear models were used to examine differences between molecular subtypes over time, from one year to 13 years of age, in the adaptive domains of communication, daily living skills, socialization and motor skills. Individuals with non-deletion subtypes typically demonstrated a higher level of adaptive skills compared to those with deletion subtypes. Statistically significant growth was observed in all adaptive domains through at least early adolescence. Individuals with AS should continue to receive developmental services and educational supports through adolescence and into adulthood given the slow rates of growth being observed across adaptive domains.

BACKGROUND: Angelman syndrome (AS) is a neurodevelopmental disorder associated with severe global developmental delay. However, the ages at which different developmental skills are achieved in these individuals remain unclear. We seek to determine the probability and the age of acquisition of specific developmental milestones and daily living skills in individuals with AS across the different molecular subtypes, viz. class I deletion, class II deletion, uniparental disomy, imprinting defect, and UBE3A variants. METHODS: Caregivers participating in a longitudinal multicenter Angelman Syndrome Natural History Study completed a questionnaire regarding the age at which their children achieved specific developmental milestones and daily living skills. The Cox Proportional Hazard model was applied to analyze differences in the probability of achievement of skills at various ages among five molecular subtypes of AS. RESULTS: Almost all individuals, regardless of molecular subtype, were able to walk with support by five years of age. By age 15, those with a deletion had at least a 50% probability of acquiring 17 out of 30 skills compared to 25 out of 30 skills among those without a deletion. Overall, fine and gross motor skills such as holding and reaching for small objects, sitting, and walking with support were achieved within a fairly narrow range of ages, while toileting, feeding, and hygiene skills tend to have greater variability in the ages at which these skills were achieved. Those without a deletion had a higher probability (25-92%) of achieving daily living skills such as independently toileting and dressing compared to those with a deletion (0-13%). Across all molecular subtypes, there was a low probability of achieving independence in bathing and brushing teeth. CONCLUSION: Individuals with AS without a deletion are more likely to achieve developmental milestones and daily living skills at an earlier age than those with a deletion. Many individuals with AS are unable to achieve daily living skills necessary for independent self-care.

Author(s): Nidhisha Sadhwani [1]; Ninad Sawant [1]; Deepak Gupta (corresponding author) [1] Key Message: Cranio-orbital deformities, especially hypotelorism and lateral orbital retrusion, can be effectively managed by fronto-orbital advancement. Anterolateral [...]

This study explores the relationship between procrastination and declining healthy habits among adolescents, a topic lacking systematic reviews in the existing literature. The primary purpose is to lay the groundwork for promoting mental health and preventing procrastination as risky behavior. This systematic review examined five areas related to procrastination and its influence on healthy lifestyle habits in adolescents: technology and procrastination; sleep and procrastination; academic procrastination; and the COVID-19 pandemic. The findings highlight that technology misuse is linked with procrastination; adolescents tend to procrastinate when going to sleep; academic procrastination negatively impacts long-term educational achievements, and the COVID-19 pandemic has exacerbated this phenomenon. Ultimately, it is concluded that procrastination is related to all these aspects and has detrimental effects on adolescents’ physical and psychological development.

Kikuchi’s disease is also known as histiocytic necrotizing lymphadenitis. It is a rare, idiopathic, self-limiting disease presenting with fever, malaise, and generalized lymphadenopathy, mostly in young females, although it has been occasionally reported in the pediatric population. Cutaneous manifestations are visible in around 16%–40% of cases, ranging from nonspecific papules, plaques, nodules, and ulcers favoring the face, upper extremities, and trunk. We report here a case of an 11-year-old child who presented with fever, leukopenia, lymphadenopathy, and cutaneous eruption. A final diagnosis of Kikuchi’s disease was established after a lymph node biopsy report.

Abstract Background Angelman syndrome (AS) is a neurodevelopmental disorder associated with severe global developmental delay. However, the ages at which different developmental skills are achieved in these individuals remain unclear. We seek to determine the probability and the age of acquisition of specific developmental milestones and daily living skills in individuals with AS across the different molecular subtypes, viz. class I deletion, class II deletion, uniparental disomy, imprinting defect, and UBE3A variants. Methods Caregivers participating in a longitudinal multicenter Angelman Syndrome Natural History Study completed a questionnaire regarding the age at which their children achieved specific developmental milestones and daily living skills. The Cox Proportional Hazard model was applied to analyze differences in the probability of achievement of skills at various ages among five molecular subtypes of AS. Results Almost all individuals, regardless of molecular subtype, were able to walk with support by five years of age. By age 15, those with a deletion had at least a 50% probability of acquiring 17 out of 30 skills compared to 25 out of 30 skills among those without a deletion. Overall, fine and gross motor skills such as holding and reaching for small objects, sitting, and walking with support were achieved within a fairly narrow range of ages, while toileting, feeding, and hygiene skills tend to have greater variability in the ages at which these skills were achieved. Those without a deletion had a higher probability (25–92%) of achieving daily living skills such as independently toileting and dressing compared to those with a deletion (0–13%). Across all molecular subtypes, there was a low probability of achieving independence in bathing and brushing teeth. Conclusion Individuals with AS without a deletion are more likely to achieve developmental milestones and daily living skills at an earlier age than those with a deletion. Many individuals with AS are unable to achieve daily living skills necessary for independent self-care.

BACKGROUND: Angelman syndrome (AS) is a rare neurodevelopmental disorder characterized by the absence of a functional UBE3A gene, which causes developmental, behavioral, and medical challenges. While currently untreatable, comprehensive data could help identify appropriate endpoints assessing meaningful improvements in clinical trials. Herein are reported the results from the FREESIAS study assessing the feasibility and utility of in-clinic and at-home measures of key AS symptoms. METHODS: Fifty-five individuals with AS (aged

While hip arthroscopy (HA) has increased in recent years, limited data exists regarding utilization and outcomes among racial groups. The National Surgical Quality Improvement Program (NSQIP) database was queried for patients who underwent HA from 2006 to 2017. Patients were stratified into 6 self-reported racial/ethnic categories: White, African American, Hispanic, Asian and Pacific Islander, Native American, and Unknown. Major and minor complications in the 30-day post-operative period were identified. Data were available for 2230 patients who underwent HA. There were significant differences in the proportions of HA procedures when examining by race. White patients comprised 69% of the patient sample, African American patients 5.6%, Hispanic patients 3.9%, Asian patients 2.5%, Native American patients 0.7% and Unknown race/ethnicity patients 18.3% ( P

ObjectiveCOVID-19 has markedly impacted the provision of neurodevelopmental care. In response, the Cardiac Neurodevelopmental Outcome Collaborative established a Task Force to assess the telehealth practices of cardiac neurodevelopmental programmes during COVID-19, including adaptation of services, test protocols and interventions, and perceived obstacles, disparities, successes, and training needs.Study designA 47-item online survey was sent to 42 Cardiac Neurodevelopmental Outcome Collaborative member sites across North America within a 3-week timeframe (22 July to 11 August 2020) to collect cross-sectional data on practices.ResultsOf the 30 participating sites (71.4% response rate), all were providing at least some clinical services at the time of the survey and 24 sites (80%) reported using telehealth. All but one of these sites were offering new telehealth services in response to COVID-19, with the most striking change being the capacity to offer new intervention services for children and their caregivers. Only a third of sites were able to carry out standardised, performance-based, neurodevelopmental testing with children and adolescents using telehealth, and none had completed comparable testing with infants and toddlers. Barriers associated with language, child ability, and access to technology were identified as contributing to disparities in telehealth access.ConclusionsTelehealth has enabled continuation of at least some cardiac neurodevelopmental services during COVID-19, despite the challenges experienced by providers, children, families, and health systems. The Cardiac Neurodevelopmental Outcome Collaborative provides a unique platform for sharing challenges and successes across sites, as we continue to shape an evidence-based, efficient, and consistent approach to the care of individuals with CHD.

We describe the development of 236 children with Angelman syndrome (AS) using the Bayley Scales of Infant and Toddler Development, Third Edition. Multilevel linear mixed modeling approaches were used to explore differences between molecular subtypes and over time. Individuals with AS continue to make slow gains in development through at least age 12 years of age at about 1-2 months/year based on age equivalent score and 1-16 growth score points/year depending on molecular subtype and domain. Children with a deletion have lower scores at baseline and slower rate of gaining skills while children with UBE3A variant subtype demonstrated higher scores as well as greater rates of skill attainment in all domains. The developmental profiles of UPD and ImpD were similar.

Introduction: Nonmetric dental traits have a crucial role in ethnic classification of populations that help in forensic racial identification. Many studies have demonstrated the differences in the expression and frequency of dental traits between various ethnic groups for ancestry determination in the context of forensic dental anthropology. The present study is an attempt to assess the frequency and variation in nonmetric traits for establishing ethnicity in the Vidarbha subpopulation. Aim: The aim of the study was to assess the frequency and variation of nonmetric traits in permanent teeth for establishing ethnicity in the Vidarbha subpopulation. Materials and Methods: Participants of the Vidarbha subpopulation were selected by random sampling method. Molars, premolars, and incisors were evaluated for frequency of nonmetric traits, and variations were graded according to Dentoanthropological System of State University of Arizona criteria. Descriptive statistics, Chi-square test, and Student's t-test were used for analysis of data. Results: The most common occlusal morphology in mandibular first and second molar was 5 cusp and 4 cusp, respectively, with the “+” groove pattern. Cusp 3 and cusp 4 were observed frequently in the maxillary molars. One lingual cusp was most commonly seen in mandibular premolars show while incisors showed faint shoveling. Conclusion: Our study concludes that nonmetric traits were present and showed variation in permanent maxillary and mandibular teeth among the Vidarbha subpopulation. Cusp 5 and Cusp 4 in first and second mandibular molars, respectively, groove pattern “+” and grade 1 protostylid were the most frequent grades observed in permanent mandibular molars. One lingual cusp was most commonly seen in mandibular premolars and faint shoveling was a notable feature in incisors of this population.

While the Internet of Things (IoT) paradigm has transformed connectivity, it has also brought with it previously unheard-of security risks. The categorization of IoT attacks using several machine learning techniques and a deep learning method is the main emphasis of this research. In addition to proposing a binary and multiclass classification framework with Machine Learning (ML) algorithms like Random Forest (RF), Decision tree (DT), Extra Tree Classifier (ETC), Support Vector Machine (SVM), and k-Nearest Neighbor (KNN) and Deep Learning (DL) architectures like Deep Neural Network (DNN), the study assesses a wide range of attack types in IoT environments. Benchmark datasets with real-world IoT attack scenarios, such as Edge-IIoTset, are used for experimentation. Preprocessing is done on the dataset using Principal Componenet Analysis (PCA) for feature selection, Synthetic Minority Oversampling Technique to handle class imbalance and Standard Scaling for feature scaling. These approaches’ comparative performance and efficacy are examined. The outcomes indicate how successful the DL model in managing intricate attack patterns and the generalization capabilities of ML algorithms across various attack classes. The DNN model yields the best results, with 100% accuracy for binary classification, 96.15% accuracy for 6-class classification, and 94.68% accuracy for 15-class classification. Further, 10-fold cross validation has been applied to make sure that the model does not overfit. This work contributes to the improvement of IoT security mechanisms by offering insights into the selection of appropriate approaches for binary and multiclass classification of threats.

ObjectiveIn the wake of the COVID-19 pandemic, psychologists were pushed to look beyond traditional in-person models of neurodevelopmental assessment to maintain continuity of care. A wealth of data demonstrates that telehealth is efficacious for pediatric behavioral intervention; however, best practices for incorporating telehealth into neurodevelopmental assessment are yet to be developed. In this topical review, we propose a conceptual model to demonstrate how telehealth can be incorporated into various components of neurodevelopmental assessment.MethodsHarnessing existing literature and expertise from a multidisciplinary task force comprised of clinicians, researchers, and patient/parent representatives from the subspecialty of cardiac neurodevelopmental care, a conceptual framework for telehealth neurodevelopmental assessment was developed. Considerations for health equity and access to care are discussed, as well as general guidelines for clinical implementation and gaps in existing literature.ResultsThere are opportunities to integrate telehealth within each stage of neurodevelopmental assessment, from intake to testing, through to follow-up care. Further research is needed to determine whether telehealth mitigates or exacerbates disparities in access to care for vulnerable populations as well as to provide evidence of validity for a wider range of neurodevelopmental measures to be administered via telehealth.ConclusionsWhile many practices are returning to traditional, face-to-face neurodevelopmental assessment services, psychologists have a unique opportunity to harness the momentum for telehealth care initiated during the pandemic to optimize the use of clinical resources, broaden service delivery, and increase access to care for pediatric neurodevelopmental assessment.

Emergency department is the entry point of any multispecialty or tertiary care hospital. This area is core area of any institute where all types of patients are admitted. We decided to study pattern of admission to understand workload of our ER and clue to frequently presenting conditions and readiness of our team to handle any emergency. A study on collection of demographic data of admission in casualty of trauma centre was conducted over a period of 3 months and demographic data were collected along with provisional diagnosis, average casualty stay and severity of admission. These data were collected in retrospective pattern and manually. Collected data were analysed by appropriate statistical software. These data were able to sort out percentage of medical and surgical admissions, also severity of condition leading to presentation to ER was obtained. Major age groups were identified which can be very helpful for allocation of recources. Observation of these study were useful for understanding the readiness and lacunae of a busy trauma center for effective management of patients. Also it gives clue to particular expertise distribution as per common presentation. It can also give insight for planning future studies in better way and to suggest preventive measures at community level for some specific diseases.

Background: Enhanced Recovery After Surgery (ERAS) protocols for bariatric surgery improve clinical outcomes. However, the impact of ERAS protocols on patient satisfaction is unknown. Virtual reality has been implemented as an effective adjunct to standard analgesic regimens. This study seeks to find out if immersive virtual reality in the immediate postoperative period could improve the subjective quality of recovery and further reduce opioid requirements for bariatric surgery patients compared with ERAS care alone. Methods: This is a single-centre, randomised clinical trial of patients recovering from laparoscopic bariatric surgery. Once in the post-anaesthesia care unit (PACU), participants will receive either an immersive virtual reality plus ERAS protocol or ERAS protocol alone. The primary outcome will be the Quality of Recovery-15 (QoR-15) score at PACU discharge. Secondary outcomes include PACU opioid requirements, length of PACU stay, PACU pain scores, QoR-15 score on postoperative day 1, hospital length of stay, opioid requirements, and opioid-related adverse effects until hospital discharge. Conclusions: Positive findings from this study could introduce virtual reality as a non-pharmacological adjunct during PACU care that improves subjective recovery for patients undergoing bariatric surgery. Clinical trial registration: NCT04754165.

Angelman Syndrome (AS) is a neurodevelopmental disorder most commonly caused by the impaired expression of the maternal UBE3A gene on chromosome 15. Though anxiety has been identified as a frequently present characteristic in AS, there are limited studies examining anxiety in this population. Studies of anxiety in other neurodevelopmental disorders have found disorder specific symptoms of anxiety and age specific displays of anxiety symptoms. However, there is a consistent challenge in identifying anxiety in people with neurodevelopmental disorders given the lack of measurement instruments specifically designed for this population. Given the limited information about AS and anxiety, the aims of the current project were to (a) examine symptoms of anxiety in children with AS and (b) determine the correlates of anxiety in children with AS. Participants included 42 adult caregivers of youth with AS in the AS Natural History study who completed the Developmental Behavior Checklist (DBC). The results found that 26% of the sample demonstrated elevated symptoms of anxiety and established a relationship between elevated anxiety in youth with AS and higher levels of irritability, hyperactivity, self-absorbed behaviors, and disruptive/antisocial behaviors. Findings from this research provide a foundation for tailoring evidence-based assessments and treatments for youth with AS and anxiety.

Abstract Background Angelman syndrome (AS) is a rare neurodevelopmental disorder characterized by the absence of a functional UBE3A gene, which causes developmental, behavioral, and medical challenges. While currently untreatable, comprehensive data could help identify appropriate endpoints assessing meaningful improvements in clinical trials. Herein are reported the results from the FREESIAS study assessing the feasibility and utility of in-clinic and at-home measures of key AS symptoms. Methods Fifty-five individuals with AS (aged

Cat bites are a relatively common emergency department presentation. The most common species isolated from cat bite wounds is Pasteurella multocida . In this case report, we discuss a unique case of an intramuscular abscess caused by Bacteroides pyogenes . Antibiotic resistance in Bacteroides species is a growing concern and thus should be treated with caution. We present an 81-year-old female who presented to the emergency department with left calf pain that began 3 weeks prior to the emergency department presentation. She was initially seen by her primary care provider and was prescribed oral Trimethoprim/Sulfamethoxazole and Amoxicillin/Clavulanate. Despite oral antibiotics, she began to have worsening swelling, erythema, and fevers. In the emergency department, the patient’s left calf was noticeably erythematous and tender to palpation. Lab work showed a C-reactive protein of 12.4 mg/dl, erythrocyte sedimentation rate of 38 mm/h, and white blood cell count of 15.1. Computed tomography imaging showed an irregularly shaped fluid collection at the junction of the calf musculature and upper aspect of the Achilles tendon. The decision was made to perform an operative incision and debridement on the evening of her presentation. On postoperative day 2, the wound cultures grew positive for both Pasturella multocida and Bacteroides pyogenes . She was subsequently discharged with oral Amoxicillin/Clavulanate 875 mg twice daily for 4 weeks. Further studies can be performed to identify the most efficient ways to isolate and treat this species. This case report emphasizes the importance of ordering both aerobic and anaerobic cultures and aiming antimicrobial therapy against both Pasteurella and anaerobic bacteria.

Adult hip dysplasia provides many challenges for joint surgeons. Due to the abnormal bone morphology and altered biomechanics of the hip, surgeons must ensure accurate implant positioning to avoid postoperative complications. We present a 56-year-old female with a history of bilateral Legg-Calve-Perthes disease and subsequent dysplasia who underwent bilateral total hip arthroplasty using robotic navigation. We highlight the utility of robotic navigation in adult hip dysplasia to improve implant positioning and ensure optimal patient outcomes.

Angelman Syndrome (AS) is a severe neurodevelopmental disorder due to impaired expression of UBE3A in neurons. There are several genetic mechanisms that impair UBE3A expression, but they differ in how neighboring genes on chromosome 15 at 15q11-q13 are affected. There is evidence that different genetic subtypes present with different clinical severity, but a systematic quantitative investigation is lacking. Here we analyze natural history data on a large sample of individuals with AS (n = 250, 848 assessments), including clinical scales that quantify development of motor, cognitive, and language skills (Bayley Scales of Infant Development, Third Edition; Preschool Language Scale, Fourth Edition), adaptive behavior (Vineland Adaptive Behavioral Scales, Second Edition), and AS-specific symptoms (AS Clinical Severity Scale). We found that clinical severity, as captured by these scales, differs between genetic subtypes: individuals with UBE3A pathogenic variants and imprinting defects (IPD) are less affected than individuals with uniparental paternal disomy (UPD); of those with UBE3A pathogenic variants, individuals with truncating mutations are more impaired than those with missense mutations. Individuals with a deletion that encompasses UBE3A and other genes are most impaired, but in contrast to previous work, we found little evidence for an influence of deletion length (class I vs. II) on severity of manifestations. The results of this systematic analysis highlight the relevance of genomic regions beyond UBE3A as contributing factors in the AS phenotype, and provide important information for the development of new therapies for AS. More generally, this work exemplifies how increasing genetic irregularities are reflected in clinical severity.