5 recherche sur le mot-clé 'Hyperphagia'

Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study / Jessica MACKAY in Journal of Autism and Developmental Disorders, 52-9 (September 2022)  

Public ISBD [article]  inJournal of Autism and Developmental Disorders > 52-9 (September 2022) . - p.3877-3889 Titre : Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study Type de document : texte imprimé Auteurs : Jessica MACKAY, Auteur ; Gillian M. NIXON, Auteur ; Antony R. LAFFERTY, Auteur ; Geoff AMBLER, Auteur ; Nitin KAPUR, Auteur ; Philip B. BERGMAN, Auteur ; Cara SCHOFIELD, Auteur ; Chris SETON, Auteur ; Andrew TAI, Auteur ; Elaine THAM, Auteur ; Komal VORA, Auteur ; Patricia CROCK, Auteur ; Charles VERGE, Auteur ; Yassmin MUSTHAFFA, Auteur ; Greg BLECHER, Auteur ; Daan CAUDRI, Auteur ; Helen LEONARD, Auteur ; Peter JACOBY, Auteur ; Andrew WILSON, Auteur ; Catherine S. CHOONG, Auteur ; Jenny DOWNS, Auteur Article en page(s) : p.3877-3889 Langues : Anglais (eng) Mots-clés : Autism Spectrum Disorder  Caregivers  Child  Humans  Hyperphagia  Prader-Willi Syndrome/genetics  Quality of Life  Sleep  Sleep Wake Disorders  Growth hormone  behaviour  Parental well-being  Prader-Willi syndrome Index. décimale : PER Périodiques Résumé : Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n=50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS. En ligne : http://dx.doi.org/10.1007/s10803-021-05265-5 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=485 [article] Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study [texte imprimé] / Jessica MACKAY, Auteur ; Gillian M. NIXON, Auteur ; Antony R. LAFFERTY, Auteur ; Geoff AMBLER, Auteur ; Nitin KAPUR, Auteur ; Philip B. BERGMAN, Auteur ; Cara SCHOFIELD, Auteur ; Chris SETON, Auteur ; Andrew TAI, Auteur ; Elaine THAM, Auteur ; Komal VORA, Auteur ; Patricia CROCK, Auteur ; Charles VERGE, Auteur ; Yassmin MUSTHAFFA, Auteur ; Greg BLECHER, Auteur ; Daan CAUDRI, Auteur ; Helen LEONARD, Auteur ; Peter JACOBY, Auteur ; Andrew WILSON, Auteur ; Catherine S. CHOONG, Auteur ; Jenny DOWNS, Auteur . - p.3877-3889. Langues : Anglais (eng) in Journal of Autism and Developmental Disorders > 52-9 (September 2022) . - p.3877-3889 Mots-clés : Autism Spectrum Disorder  Caregivers  Child  Humans  Hyperphagia  Prader-Willi Syndrome/genetics  Quality of Life  Sleep  Sleep Wake Disorders  Growth hormone  behaviour  Parental well-being  Prader-Willi syndrome Index. décimale : PER Périodiques Résumé : Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n=50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS. En ligne : http://dx.doi.org/10.1007/s10803-021-05265-5 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=485

Behavioral changes in patients with Prader-Willi syndrome receiving diazoxide choline extended-release tablets compared to the PATH for PWS natural history study / Theresa V. STRONG in Journal of Neurodevelopmental Disorders, 16 (2024)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 16 (2024) Titre : Behavioral changes in patients with Prader-Willi syndrome receiving diazoxide choline extended-release tablets compared to the PATH for PWS natural history study Type de document : texte imprimé Auteurs : Theresa V. STRONG, Auteur ; Jennifer L. MILLER, Auteur ; Shawn E. MCCANDLESS, Auteur ; Evelien GEVERS, Auteur ; Jack A. YANOVSKI, Auteur ; Lisa MATESEVAC, Auteur ; Jessica BOHONOWYCH, Auteur ; Shaila BALLAL, Auteur ; Kristen YEN, Auteur ; Patricia HIRANO, Auteur ; Neil M. COWEN, Auteur ; Anish BHATNAGAR, Auteur Langues : Anglais (eng) Mots-clés : Humans  Prader-Willi Syndrome/complications/drug therapy  Female  Male  Hyperphagia/drug therapy/etiology  Child  Adult  Adolescent  Diazoxide/administration & dosage/pharmacology  Young Adult  Delayed-Action Preparations  Child, Preschool  Cohort Studies  Dccr  Hyperphagia  Natural history  Prader-Willi syndrome  JLM, SEM, EG, and JAY received funding from Soleno to support the conduct of the  clinical trial. EG reports receipt of lecturing and consulting fees from Soleno.  JAY reports grant support from Soleno Therapeutics and from Rhythm  Pharmaceuticals for obesity-related projects, and material support for research  from Hikma Pharmaceuticals and Versanis-Bio and is supported by the Intramural  Research Program of the Eunice Kennedy Shriver National Institute of Child Health  and Human Development, National Institutes of Health, ZIAHD000641. TVS, JB and LM  are employed by FPWR. Index. décimale : PER Périodiques Résumé : BACKGROUND: Prader-Willi syndrome (PWS) is a rare neurobehavioral-metabolic disease caused by the lack of paternally expressed genes in the chromosome 15q11-q13 region, characterized by hypotonia, neurocognitive problems, behavioral difficulties, endocrinopathies, and hyperphagia resulting in severe obesity if energy intake is not controlled. Diazoxide choline extended-release (DCCR) tablets have previously been evaluated for their effects on hyperphagia and other behavioral complications of people with PWS in a Phase 3 placebo-controlled study of participants with PWS, age 4 and older with hyperphagia (C601) and in an open label extension study, C602. METHODS: To better understand the longer-term impact of DCCR, a cohort from PATH for PWS, a natural history study that enrolled participants with PWS age 5 and older, who met the C601 age, weight and baseline hyperphagia inclusion criteria and had 2 hyperphagia assessments ≥ 6 months apart, were compared to the C601/C602 cohort. Hyperphagia was measured using the Hyperphagia Questionnaire for Clinical Trials (HQ-CT, range 0-36). The primary analysis used observed values with no explicit imputation of missing data. A sensitivity analysis was conducted in which all missing HQ-CT assessments in the C601/C602 cohort were assigned the highest possible value (36), representing the worst-case scenario. Other behavioral changes were assessed using the Prader-Willi Syndrome Profile questionnaire (PWSP). RESULTS: Relative to the PATH for PWS natural history study cohort, the DCCR-treated C601/C602 cohort showed significant improvements in HQ-CT score at 26 weeks (LSmean [SE] -8.3 [0.75] vs. -2.5 [0.43], p < 0.001) and 52 weeks (LSmean [SE] -9.2 [0.77] vs. -3.4 [0.47], p < 0.001). The comparison between the cohorts remained significant in the worst-case imputation sensitivity analysis. There were also significant improvements in all domains of the PWSP at 26 weeks (all p < 0.001) and 52 weeks (all p ≤ 0.003) for C601/C602 participants compared to the PATH for PWS participants. CONCLUSION: Long-term administration of DCCR to people with PWS resulted in changes in hyperphagia and other behavioral complications of PWS that are distinct from the natural history of the syndrome as exemplified by the cohort from PATH for PWS. The combined effects of administration of DCCR should reduce the burden of the syndrome on the patient, caregivers and their families, and thereby may benefit people with PWS and their families. TRIAL REGISTRATION: Clinical study C601 was originally registered on ClinicalTrials.gov on February 22, 2018 (NCT03440814). Clinical study C602 was originally registered on ClinicalTrials.gov on October 22, 2018 (NCT03714373). PATH for PWS was originally registered on ClinicalTrials.gov on October 24, 2018 (NCT03718416). En ligne : https://dx.doi.org/10.1186/s11689-024-09536-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575 [article] Behavioral changes in patients with Prader-Willi syndrome receiving diazoxide choline extended-release tablets compared to the PATH for PWS natural history study [texte imprimé] / Theresa V. STRONG, Auteur ; Jennifer L. MILLER, Auteur ; Shawn E. MCCANDLESS, Auteur ; Evelien GEVERS, Auteur ; Jack A. YANOVSKI, Auteur ; Lisa MATESEVAC, Auteur ; Jessica BOHONOWYCH, Auteur ; Shaila BALLAL, Auteur ; Kristen YEN, Auteur ; Patricia HIRANO, Auteur ; Neil M. COWEN, Auteur ; Anish BHATNAGAR, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 16 (2024) Mots-clés : Humans  Prader-Willi Syndrome/complications/drug therapy  Female  Male  Hyperphagia/drug therapy/etiology  Child  Adult  Adolescent  Diazoxide/administration & dosage/pharmacology  Young Adult  Delayed-Action Preparations  Child, Preschool  Cohort Studies  Dccr  Hyperphagia  Natural history  Prader-Willi syndrome  JLM, SEM, EG, and JAY received funding from Soleno to support the conduct of the  clinical trial. EG reports receipt of lecturing and consulting fees from Soleno.  JAY reports grant support from Soleno Therapeutics and from Rhythm  Pharmaceuticals for obesity-related projects, and material support for research  from Hikma Pharmaceuticals and Versanis-Bio and is supported by the Intramural  Research Program of the Eunice Kennedy Shriver National Institute of Child Health  and Human Development, National Institutes of Health, ZIAHD000641. TVS, JB and LM  are employed by FPWR. Index. décimale : PER Périodiques Résumé : BACKGROUND: Prader-Willi syndrome (PWS) is a rare neurobehavioral-metabolic disease caused by the lack of paternally expressed genes in the chromosome 15q11-q13 region, characterized by hypotonia, neurocognitive problems, behavioral difficulties, endocrinopathies, and hyperphagia resulting in severe obesity if energy intake is not controlled. Diazoxide choline extended-release (DCCR) tablets have previously been evaluated for their effects on hyperphagia and other behavioral complications of people with PWS in a Phase 3 placebo-controlled study of participants with PWS, age 4 and older with hyperphagia (C601) and in an open label extension study, C602. METHODS: To better understand the longer-term impact of DCCR, a cohort from PATH for PWS, a natural history study that enrolled participants with PWS age 5 and older, who met the C601 age, weight and baseline hyperphagia inclusion criteria and had 2 hyperphagia assessments ≥ 6 months apart, were compared to the C601/C602 cohort. Hyperphagia was measured using the Hyperphagia Questionnaire for Clinical Trials (HQ-CT, range 0-36). The primary analysis used observed values with no explicit imputation of missing data. A sensitivity analysis was conducted in which all missing HQ-CT assessments in the C601/C602 cohort were assigned the highest possible value (36), representing the worst-case scenario. Other behavioral changes were assessed using the Prader-Willi Syndrome Profile questionnaire (PWSP). RESULTS: Relative to the PATH for PWS natural history study cohort, the DCCR-treated C601/C602 cohort showed significant improvements in HQ-CT score at 26 weeks (LSmean [SE] -8.3 [0.75] vs. -2.5 [0.43], p < 0.001) and 52 weeks (LSmean [SE] -9.2 [0.77] vs. -3.4 [0.47], p < 0.001). The comparison between the cohorts remained significant in the worst-case imputation sensitivity analysis. There were also significant improvements in all domains of the PWSP at 26 weeks (all p < 0.001) and 52 weeks (all p ≤ 0.003) for C601/C602 participants compared to the PATH for PWS participants. CONCLUSION: Long-term administration of DCCR to people with PWS resulted in changes in hyperphagia and other behavioral complications of PWS that are distinct from the natural history of the syndrome as exemplified by the cohort from PATH for PWS. The combined effects of administration of DCCR should reduce the burden of the syndrome on the patient, caregivers and their families, and thereby may benefit people with PWS and their families. TRIAL REGISTRATION: Clinical study C601 was originally registered on ClinicalTrials.gov on February 22, 2018 (NCT03440814). Clinical study C602 was originally registered on ClinicalTrials.gov on October 22, 2018 (NCT03714373). PATH for PWS was originally registered on ClinicalTrials.gov on October 24, 2018 (NCT03718416). En ligne : https://dx.doi.org/10.1186/s11689-024-09536-x Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575

Validation of the Food Safe Zone questionnaire for families of individuals with Prader-Willi syndrome / Elisabeth M. DYKENS in Journal of Neurodevelopmental Disorders, 17 (2025)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 17 (2025) Titre : Validation of the Food Safe Zone questionnaire for families of individuals with Prader-Willi syndrome Type de document : texte imprimé Auteurs : Elisabeth M. DYKENS, Auteur ; Elizabeth ROOF, Auteur ; Hailee HUNT-HAWKINS, Auteur ; Theresa V. STRONG, Auteur Langues : Anglais (eng) Mots-clés : Humans  Prader-Willi Syndrome/psychology/complications  Female  Male  Child  Psychometrics  Hyperphagia/etiology/psychology/diagnosis  Surveys and Questionnaires  Adult  Adolescent  Parents/psychology  Child, Preschool  Food Safety  Reproducibility of Results  Family/psychology  Feeding Behavior  Clinical trials  Hyperphagia  PWS food safety  Prader-Willi syndrome  this study was obtained by the Vanderbilt University Institutional Review Board,  Integrated Science Committee, under the auspices of the University’s Human  Research Protections Program. Vanderbilt participants provided written, informed  consent using the e-consent function of RedCap, a secure, web-based data  collection platform. After consenting, parents were invited to complete 3  questionnaires on RedCap. Additional study approval was obtained for participants  recruited from the Foundation for Prader-Willi Research (FPWR) Patient Registry.  Prior to collecting data from the Registry, the study was reviewed and approved  by FPWR’s research committee and IRB. All registrants in FPWR’s Patient Registry  gave approval for their de-identified data to be used for research purposes.  Consent for publication: Not Applicable. Competing interests: The authors declare  no competing interests. Index. décimale : PER Périodiques Résumé : BACKGROUND: Prader-Willi syndrome (PWS), a genetic neurodevelopmental disorder, is characterized by hyperphagia and significant behavioral problems. Hyperphagic individuals with PWS are chronically hungry yet rarely feel sated, and often engage in food-seeking behaviors. To avoid life-threatening obesity in their children, families implement food security strategies (e.g., locking food sources, constant supervision around food, alerting others). Although widely used, these strategies have yet to be systematically examined. We thus developed and analyzed the psychometric properties of a new measure of these diverse strategies, the Food Safe Zone, and evaluated them in relation to hyperphagic symptoms and demographic variables. In doing so, we also shine a light on the extraordinary efforts of families in managing their children's hyperphagia. METHODS: Our team developed 20 FSZ items that were revised for clarity and completeness in an iterative feedback process with stakeholders, including parents, PWS specialists, and individuals with PWS. The FSZ was pilot tested, descriptive findings were reviewed by additional stakeholders, and then administered to 624 parents in a large-scale study. Based on an open-ended question, "Is there anything else you do to ensure food safety?" two additional items were added and evaluated in a follow-up study. RESULTS: Principal component analyses revealed that 21 FSZ items loaded onto 5 factors that were readily interpretable, accounting for 67% of test variance: Alerting Others and Food Supervision in the Community; Locking or Restricting Food Sources; Checking for Food; At Home Supervision and Meals; and Avoiding Food Settings. Internal consistency and test-rest reliability were robust. Convergent validity analyses revealed that parents implemented FSZ strategies in response to the severity of their child's hyperphagia, and not their child's age, gender or PWS genetic subtype. CONCLUSIONS: The psychometrically sound FSZ holds promise for future research, especially on the effects of food safety tactics on family members. In future clinical trials, the FSZ could also be used to help parents think critically about their food safety tactics in relation to their child's hyperphagia, or as an exploratory endpoint; if hyperphagia is lessened, so too may food safety tactics, thereby enhancing familial quality of life. En ligne : https://dx.doi.org/10.1186/s11689-024-09589-y Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=576 [article] Validation of the Food Safe Zone questionnaire for families of individuals with Prader-Willi syndrome [texte imprimé] / Elisabeth M. DYKENS, Auteur ; Elizabeth ROOF, Auteur ; Hailee HUNT-HAWKINS, Auteur ; Theresa V. STRONG, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 17 (2025) Mots-clés : Humans  Prader-Willi Syndrome/psychology/complications  Female  Male  Child  Psychometrics  Hyperphagia/etiology/psychology/diagnosis  Surveys and Questionnaires  Adult  Adolescent  Parents/psychology  Child, Preschool  Food Safety  Reproducibility of Results  Family/psychology  Feeding Behavior  Clinical trials  Hyperphagia  PWS food safety  Prader-Willi syndrome  this study was obtained by the Vanderbilt University Institutional Review Board,  Integrated Science Committee, under the auspices of the University’s Human  Research Protections Program. Vanderbilt participants provided written, informed  consent using the e-consent function of RedCap, a secure, web-based data  collection platform. After consenting, parents were invited to complete 3  questionnaires on RedCap. Additional study approval was obtained for participants  recruited from the Foundation for Prader-Willi Research (FPWR) Patient Registry.  Prior to collecting data from the Registry, the study was reviewed and approved  by FPWR’s research committee and IRB. All registrants in FPWR’s Patient Registry  gave approval for their de-identified data to be used for research purposes.  Consent for publication: Not Applicable. Competing interests: The authors declare  no competing interests. Index. décimale : PER Périodiques Résumé : BACKGROUND: Prader-Willi syndrome (PWS), a genetic neurodevelopmental disorder, is characterized by hyperphagia and significant behavioral problems. Hyperphagic individuals with PWS are chronically hungry yet rarely feel sated, and often engage in food-seeking behaviors. To avoid life-threatening obesity in their children, families implement food security strategies (e.g., locking food sources, constant supervision around food, alerting others). Although widely used, these strategies have yet to be systematically examined. We thus developed and analyzed the psychometric properties of a new measure of these diverse strategies, the Food Safe Zone, and evaluated them in relation to hyperphagic symptoms and demographic variables. In doing so, we also shine a light on the extraordinary efforts of families in managing their children's hyperphagia. METHODS: Our team developed 20 FSZ items that were revised for clarity and completeness in an iterative feedback process with stakeholders, including parents, PWS specialists, and individuals with PWS. The FSZ was pilot tested, descriptive findings were reviewed by additional stakeholders, and then administered to 624 parents in a large-scale study. Based on an open-ended question, "Is there anything else you do to ensure food safety?" two additional items were added and evaluated in a follow-up study. RESULTS: Principal component analyses revealed that 21 FSZ items loaded onto 5 factors that were readily interpretable, accounting for 67% of test variance: Alerting Others and Food Supervision in the Community; Locking or Restricting Food Sources; Checking for Food; At Home Supervision and Meals; and Avoiding Food Settings. Internal consistency and test-rest reliability were robust. Convergent validity analyses revealed that parents implemented FSZ strategies in response to the severity of their child's hyperphagia, and not their child's age, gender or PWS genetic subtype. CONCLUSIONS: The psychometrically sound FSZ holds promise for future research, especially on the effects of food safety tactics on family members. In future clinical trials, the FSZ could also be used to help parents think critically about their food safety tactics in relation to their child's hyperphagia, or as an exploratory endpoint; if hyperphagia is lessened, so too may food safety tactics, thereby enhancing familial quality of life. En ligne : https://dx.doi.org/10.1186/s11689-024-09589-y Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=576

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium / Lauren SCHWARTZ in Journal of Neurodevelopmental Disorders, 13 (2021)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 13 (2021) Titre : Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium Type de document : texte imprimé Auteurs : Lauren SCHWARTZ, Auteur ; Assumpta CAIXÀS, Auteur ; Anastasia DIMITROPOULOS, Auteur ; Elisabeth DYKENS, Auteur ; Jessica DUIS, Auteur ; Stewart EINFELD, Auteur ; Louise GALLAGHER, Auteur ; Anthony HOLLAND, Auteur ; Lauren RICE, Auteur ; Elizabeth ROOF, Auteur ; Parisa SALEHI, Auteur ; Theresa STRONG, Auteur ; Bonnie TAYLOR, Auteur ; Kate WOODCOCK, Auteur Langues : Anglais (eng) Mots-clés : Anxiety  Consensus  Humans  Prader-Willi Syndrome/therapy  Quality of Life  Behavior  Hyperphagia  Obsessive–compulsive  Patient vignettes  Prader-Willi syndrome  Rigidity  Social cognition  Temper outbursts  Willi Research AC–no competing interests AD–no competing interests ED–no  competing interests JD–no competing interests SE–no competing interests AH–no  competing interests LR–no competing interests ER–no competing interest PS is  involved in clinical research funded by Soleno Therapeutics, Inc. & Millendo  Therapeutics, Inc. TS is an employee of Foundation for Prader Willi Research  (FPWR) and Director of Research Programs at FPWR BT–no competing interests KW–no  competing interests Index. décimale : PER Périodiques Résumé : Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org ) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC "Behavior Outcomes Working Group" sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive-compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS. En ligne : https://dx.doi.org/10.1186/s11689-021-09373-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=574 [article] Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium [texte imprimé] / Lauren SCHWARTZ, Auteur ; Assumpta CAIXÀS, Auteur ; Anastasia DIMITROPOULOS, Auteur ; Elisabeth DYKENS, Auteur ; Jessica DUIS, Auteur ; Stewart EINFELD, Auteur ; Louise GALLAGHER, Auteur ; Anthony HOLLAND, Auteur ; Lauren RICE, Auteur ; Elizabeth ROOF, Auteur ; Parisa SALEHI, Auteur ; Theresa STRONG, Auteur ; Bonnie TAYLOR, Auteur ; Kate WOODCOCK, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 13 (2021) Mots-clés : Anxiety  Consensus  Humans  Prader-Willi Syndrome/therapy  Quality of Life  Behavior  Hyperphagia  Obsessive–compulsive  Patient vignettes  Prader-Willi syndrome  Rigidity  Social cognition  Temper outbursts  Willi Research AC–no competing interests AD–no competing interests ED–no  competing interests JD–no competing interests SE–no competing interests AH–no  competing interests LR–no competing interests ER–no competing interest PS is  involved in clinical research funded by Soleno Therapeutics, Inc. & Millendo  Therapeutics, Inc. TS is an employee of Foundation for Prader Willi Research  (FPWR) and Director of Research Programs at FPWR BT–no competing interests KW–no  competing interests Index. décimale : PER Périodiques Résumé : Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org ) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC "Behavior Outcomes Working Group" sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive-compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS. En ligne : https://dx.doi.org/10.1186/s11689-021-09373-2 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=574

The Combination of Food Approach and Food Avoidant Behaviors in Children with Autism Spectrum Disorder: "Selective Overeating" / Monica, V. NADEAU in Journal of Autism and Developmental Disorders, 52-3 (March 2022)  

Public ISBD [article]  inJournal of Autism and Developmental Disorders > 52-3 (March 2022) . - p.987-994 Titre : The Combination of Food Approach and Food Avoidant Behaviors in Children with Autism Spectrum Disorder: "Selective Overeating" Type de document : texte imprimé Auteurs : Monica, V. NADEAU, Auteur ; Emily RICHARD, Auteur ; Gregory L. WALLACE, Auteur Article en page(s) : p.987-994 Langues : Anglais (eng) Mots-clés : Autism Spectrum Disorder  Child  Diet  Feeding Behavior  Food Preferences  Humans  Hyperphagia  Autism  Eating  Flexibility  Health  Picky  Selective Index. décimale : PER Périodiques Résumé : Based on parent questionnaires, we demonstrate the presence of an eating pattern among children with autism spectrum disorder (ASD) that is characterized by both food selectivity and eating in the absence of hunger. Children with ASD were categorized as engaging in eating patterns of selective overeating, selective eating only, overeating only, or typical eating. Group differences were found in the areas of diet composition, body mass index, and behavioral flexibility. Both the selective overeating group and selective eating only group were prone to favor calorie-dense, nutrient-deficient diets as compared to other groups. Eating groups also presented with differing profiles of everyday behavioral flexibility. These results suggest that selective overeating in ASD may present unique challenges and require tailored interventions. En ligne : http://dx.doi.org/10.1007/s10803-021-04945-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=455 [article] The Combination of Food Approach and Food Avoidant Behaviors in Children with Autism Spectrum Disorder: "Selective Overeating" [texte imprimé] / Monica, V. NADEAU, Auteur ; Emily RICHARD, Auteur ; Gregory L. WALLACE, Auteur . - p.987-994. Langues : Anglais (eng) in Journal of Autism and Developmental Disorders > 52-3 (March 2022) . - p.987-994 Mots-clés : Autism Spectrum Disorder  Child  Diet  Feeding Behavior  Food Preferences  Humans  Hyperphagia  Autism  Eating  Flexibility  Health  Picky  Selective Index. décimale : PER Périodiques Résumé : Based on parent questionnaires, we demonstrate the presence of an eating pattern among children with autism spectrum disorder (ASD) that is characterized by both food selectivity and eating in the absence of hunger. Children with ASD were categorized as engaging in eating patterns of selective overeating, selective eating only, overeating only, or typical eating. Group differences were found in the areas of diet composition, body mass index, and behavioral flexibility. Both the selective overeating group and selective eating only group were prone to favor calorie-dense, nutrient-deficient diets as compared to other groups. Eating groups also presented with differing profiles of everyday behavioral flexibility. These results suggest that selective overeating in ASD may present unique challenges and require tailored interventions. En ligne : http://dx.doi.org/10.1007/s10803-021-04945-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=455