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PWS Smart-Start is a behavioral caregiver training program developed specifically for caregivers of children with Prader-Willi syndrome (PWS) ages 3 to 14. The purpose of the current study was to evaluate the acceptability and preliminary efficacy of the program. Thirty-four caregivers of children with PWS received the PWS Smart-Start training using online live video-coaching across a 10-week period. The impact of the training on a variety of variables including caregiver and child behavior, caregiver stress and burnout, and family functioning were evaluated using a quasi-experimental pretest posttest design. Results indicated statistically significant decreases across child behavior challenges and parental stress and burnout following the training. Statistically significant improvements were also seen in parenting practices, the quality of the parent–child relationship, and family quality of life. Results of social validity measures indicated caregiver satisfaction with the program. These preliminary results indicate that the PWS Smart-Start program has the potential to not only support children with PWS through behavioral challenges but also reduce caregiver stress and burnout and improve a variety of aspects of family life. Future research is needed to replicate and further validate these results and to extend the exploration of other behavior analytic interventions for this population. [ABSTRACT FROM AUTHOR]

Background: Prader–Willi syndrome (PWS) is a genetic disorder characterised by hyperphagia, intellectual disability and increased propensity to a range of medical disorders. To better characterise the clinical presentation of PWS across the lifespan, this study reports on the demographics and clinical diagnosis of individuals with PWS hospitalised in the United States. Methods: The National Inpatient Sample, an all‐payor administrative claims database of hospitalisations in the United States, was queried for patients with a coded diagnosis of PWS from October 2019 through December 2021. Hospitalisations for patients with PWS were matched to five non‐PWS hospitalisations based on age, sex, year and hospital characteristics. Results: There were 4400 (95% CI: 3,885 to 4,915) PWS hospitalisations, with a median age of 24. Compared to controls, PWS hospitalisations had longer hospital stays (median 5 vs. 3 days) and higher in‐hospital mortality (2.2% vs. 1.3%). Infectious (19.0%) and respiratory (16.2%) diagnoses were most common for PWS patients. Codes for overweight or obesity were present in 38.1% of PWS hospitalisations, with Hispanic ethnicity was associated with a higher odds of overweight/obesity in PWS patients (aOR 1.73; 95% CI: 1.11–2.71). Conclusions: PWS hospitalisations are characterised by higher healthcare utilisation and complexity compared to matched controls. The high prevalence of obesity and significant rates of infectious and respiratory conditions highlight specific health challenges for PWS patients. Validation of the Q87.11 administrative claims code is an essential step for ongoing health services research in this condition. [ABSTRACT FROM AUTHOR]

Background: Prader–Willi syndrome (PWS) is a congenital disease caused by a rare and generally non‐inherited genetic disorder. The inability to recognise facial expressions of emotion is an apparent social cognition deficit in people diagnosed with PWS. The main objective of the present study is to compare the ability to recognise emotional facial expression, in both non‐contextualised and contextualised scenarios, among the main subtypes of PWS and a control group. Methods: The sample consisted of 46 children divided into three groups: deletion (n = 10), maternal uniparental disomy (mUPD) (n = 13) and control (n = 23). The protocol included the Facially Expressed Emotion Labeling and the Deusto‐e‐Motion 1.0. Results: The control group recognised facial emotions more accurately and quickly in both non‐contextualised and contextualised scenarios than children with PWS, regardless of genetic subtype. Despite no differences being detected between PWS subtypes when non‐contextualised scenarios were analysed, in contextualised situations, a longer reaction time was observed in children with the mUPD subtype. Conclusions: This is the first study to assess the ability to recognise emotional facial expressions in contextualised situations among PWS subtypes and a control group. The findings suggest that some of the social cognitive deficits evidenced in children with mUPD PWS may be similar to those in autism spectrum disorder. [ABSTRACT FROM AUTHOR]

Prader–Willi syndrome (PWS) is a rare genetic disorder caused by the loss of paternal genes on chromosome 15. The Aberrant Behavior Checklist (ABC) is a standardized rating scale for assessing problematic behaviors in persons with developmental disabilities. Our study aims to describe ABC scores in youth with PWS and track their change over time. The analysis included 69 patients. Mean ABC scores were compared in four age groups (5–8, 9–12, 13–16, and 17–22 years). A statistically significant difference was found only in the Irritability subscale, with lower scores in the 5–8 age group compared to the 9–12 age group. For change over time, scores for Irritability, Lethargy, Stereotypic Behavior, Hyperactivity subscales, and Total score were likely to decrease after age 12. Irritability subscale scores of males were predicted to increase more than those of females between ages of 5 and 12. The Lethargy score in the nondeletion group had a greater reduction than the deletion group in the 12–20 year range. This study highlights the need for systematic collection and characterization of behavioral data given the burden of maladaptive behaviors that often persist for a lifetime. [ABSTRACT FROM AUTHOR]

Prader-Willi syndrome (PWS) is a genetic disorder associated with baseline respiratory impairment caused by multiple contributing etiologies. While this may be expected to increase the risk of severe COVID-19 infections in PWS patients, survey studies have suggested paradoxically low disease severity. To better characterize the course of COVID-19 infection in patients with PWS, this study analyses the outcomes of hospitalizations for COVID-19 among patients with and without PWS. The National Inpatient Sample, an all-payors administrative claims database of hospitalizations in the United States, was queried for patients with a coded diagnosis COVID-19 in 2020 and 2021. Hospitalizations for patients with PWS compared to those for patients without PWS using Augmented Inverse Propensity Weighting (AIPW). There were 295 (95% CI: 228-362) COVID-19 hospitalizations for individuals with PWS and 4,112,400 (95% CI: 4,051,497-4,173,303) for individuals without PWS. PWS patients had a median age of 33 years compared to 63 for those without PWS. Individuals with PWS had higher baseline rates of obesity (47.5% vs. 28.4%). AIPW models show that PWS diagnosis is associated with increased hospital length of stay by 7.43 days, hospital charges by $80,126, and the odds of mechanical ventilation and in-hospital death (odds ratios of 1.79 and 1.67, respectively). PWS patients hospitalized with COVID-19 experienced longer hospital stays, higher charges, and increased risk of mechanical ventilation and death. These results suggest that PWS should be considered a risk factor for severe COVID-19, warranting continued protective measures and vaccination efforts. Further research is needed to validate coding for PWS and assess the impact of evolving COVID-19 variants and population immunity on this vulnerable population., (© 2025 Wiley Periodicals LLC.)

Introduction: Histamine H 3 receptor antagonists/inverse agonists, since the discovery of histamine H 3 receptor (H 3 R), are important ligands in the search for new potential drugs. The most interesting are CNS diseases as these receptors are mainly there present., Areas Covered: The current review covers patent applications/patents that were published during the last 6 years (October 2017 - December 2023). Documents were found in two free available patent databases: Espacenet and PatentScope and divided into three basic categories such as methods, compounds, and therapeutic indications. It provides an overview of 51 patent applications/patents. Many pharmaceutical compositions with H 3 R antagonists/inverse agonists have been claimed. Furthermore, PubMed, Scopus, and ClinicalTrials databases were searched for literature to prepare this review., Expert Opinion: Interest in the H 3 R field is still high and has remained almost unchanged over the last 10 years in the number of publications, but the type of publications has changed (fewer new ligands, more pharmacological studies). Currently, the search for new H 3 R ligands is focused on multi-target compounds. The first crystal structure of H 3 R with a ligand appeared. New therapeutic indications, such as autism, fatigue, and Prader-Willi syndrome, are verified in clinical trials.