Appetite- and weight-regulating neuroendocrine circuitry in hypothalamic obesity
Endocr Rev. 2023 Nov 29:bnad033. doi: 10.1210/endrev/bnad033. Online ahead of print.ABSTRACTSince hypothalamic obesity (HyOb) was first described over 120 years ago by Joseph Babinski and Alfred Fröhlich, advances in molecular genetic laboratory techniques have allowed us to elucidate various components of the intricate neurocircuitry governing appetite and weight regulation connecting the hypothalamus, pituitary gland, brainstem, adipose tissue, pancreas and gastrointestinal tract. On a background of an increasing prevalence of population-level common obesity, the number of survivors of congenital (e.g. septo-optic dyspl...
Source: Endocrine Reviews - November 29, 2023 Category: Endocrinology Authors: Hoong-Wei Gan Manuela Cerbone Mehul Tulsidas Dattani Source Type: research
Appetite- and weight-regulating neuroendocrine circuitry in hypothalamic obesity
Endocr Rev. 2023 Nov 29:bnad033. doi: 10.1210/endrev/bnad033. Online ahead of print.ABSTRACTSince hypothalamic obesity (HyOb) was first described over 120 years ago by Joseph Babinski and Alfred Fröhlich, advances in molecular genetic laboratory techniques have allowed us to elucidate various components of the intricate neurocircuitry governing appetite and weight regulation connecting the hypothalamus, pituitary gland, brainstem, adipose tissue, pancreas and gastrointestinal tract. On a background of an increasing prevalence of population-level common obesity, the number of survivors of congenital (e.g. septo-optic dyspl...
Source: ENDOCR REV - November 29, 2023 Category: Endocrinology Authors: Hoong-Wei Gan Manuela Cerbone Mehul Tulsidas Dattani Source Type: research
Appetite- and weight-regulating neuroendocrine circuitry in hypothalamic obesity
Endocr Rev. 2023 Nov 29:bnad033. doi: 10.1210/endrev/bnad033. Online ahead of print.ABSTRACTSince hypothalamic obesity (HyOb) was first described over 120 years ago by Joseph Babinski and Alfred Fröhlich, advances in molecular genetic laboratory techniques have allowed us to elucidate various components of the intricate neurocircuitry governing appetite and weight regulation connecting the hypothalamus, pituitary gland, brainstem, adipose tissue, pancreas and gastrointestinal tract. On a background of an increasing prevalence of population-level common obesity, the number of survivors of congenital (e.g. septo-optic dyspl...
Source: Endocrine Reviews - November 29, 2023 Category: Endocrinology Authors: Hoong-Wei Gan Manuela Cerbone Mehul Tulsidas Dattani Source Type: research
Appetite- and weight-regulating neuroendocrine circuitry in hypothalamic obesity
Endocr Rev. 2023 Nov 29:bnad033. doi: 10.1210/endrev/bnad033. Online ahead of print.ABSTRACTSince hypothalamic obesity (HyOb) was first described over 120 years ago by Joseph Babinski and Alfred Fröhlich, advances in molecular genetic laboratory techniques have allowed us to elucidate various components of the intricate neurocircuitry governing appetite and weight regulation connecting the hypothalamus, pituitary gland, brainstem, adipose tissue, pancreas and gastrointestinal tract. On a background of an increasing prevalence of population-level common obesity, the number of survivors of congenital (e.g. septo-optic dyspl...
Source: ENDOCR REV - November 29, 2023 Category: Endocrinology Authors: Hoong-Wei Gan Manuela Cerbone Mehul Tulsidas Dattani Source Type: research
Aberrant brain intra- and internetwork functional connectivity in children with Prader-Willi syndrome
ConclusionImpaired intra- and internetwork FC patterns in PWS children are associated with developmental delays, which may result from neural pathway dysfunctions. Intranetwork FC reorganization patterns can discriminate PWS children from HCs.Registration number on the Chinese Clinical Trail RegistryChiCTR2100046551. (Source: Neuroradiology)
Source: Neuroradiology - November 25, 2023 Category: Radiology Source Type: research
Early psychomotor development and growth hormone therapy in children with Prader-Willi syndrome: a review
Conclusion: Early psychomotor development is strongly correlated with the prognosis of patients with PWS; moreover, current studies support that the initiation of interventions at an early age can exert significant beneficial effects on enhancing the cognitive and linguistic development of patients with PWS and allow them to"catch up" with motor development. What is Known:•Prader-Willi syndrome is a rare genetic disorder characterized by multisystem damage, and children with Prader-Willi syndrome are typically characterized by early developmental delays, specifically in the areas of cognitive and motor deve...
Source: European Journal of Pediatrics - November 21, 2023 Category: Pediatrics Source Type: research
Proteome profiling identifies circulating biomarkers associated with hepatic steatosis in subjects with Prader-Willi syndrome
ConclusionThe biomarkers identified in this study offer the potential for improved patient stratification and personalized therapeutic protocols. (Source: Frontiers in Endocrinology)
Source: Frontiers in Endocrinology - November 15, 2023 Category: Endocrinology Source Type: research
Age of diagnosis for children with chromosome 15q syndromes
ConclusionUnderstanding variability in the age of diagnosis for chromosome 15 disorders is an important step in reducing the diagnostic odyssey and improving access to interventions for these populations. Results from this study provide a baseline by which to evaluate efforts to reduce the age of diagnosis for individuals with these conditions. (Source: Journal of Neurodevelopmental Disorders)
Source: Journal of Neurodevelopmental Disorders - November 7, 2023 Category: Neurology Source Type: research
Food cue reward salience does not explain Hyperphagia in adolescents with Prader-Willi syndrome
. (Source: Developmental Neuropsychology)
Source: Developmental Neuropsychology - November 6, 2023 Category: Neurology Authors: Menton M. DeweeseElizabeth RoofAlexandra P. Keya Department of Teaching and Learning, Vanderbilt University, Nashville, Tennessee, USAb Department of Psychology, Vanderbilt University, Nashville, Tennessee, USAc Department of Hearing and Spositive potenti Source Type: research
Diazoxide choline extended-release tablet in people with Prader-Willi syndrome: results from long-term open-label study
CONCLUSIONS: DCCR administration to people with PWS was well-tolerated and associated with broad-ranging improvements in the syndrome. Sustained administration of DCCR has the potential to reduce disease severity and the burden of care for families.PMID:37919617 | DOI:10.1002/oby.23928 (Source: Obesity)
Source: Obesity - November 3, 2023 Category: Eating Disorders & Weight Management Authors: Jennifer L Miller Evelien Gevers Nicola Bridges Jack A Yanovski Parisa Salehi Kathryn S Obrynba Eric I Felner Lynne M Bird Ashley H Shoemaker Moris Angulo Merlin G Butler David Stevenson Anthony P Goldstone John Wilding Melissa Lah M Guftar Shaikh Elizabe Source Type: research
Diazoxide choline extended-release tablet in people with Prader-Willi syndrome: results from long-term open-label study
CONCLUSIONS: DCCR administration to people with PWS was well-tolerated and associated with broad-ranging improvements in the syndrome. Sustained administration of DCCR has the potential to reduce disease severity and the burden of care for families.PMID:37919617 | DOI:10.1002/oby.23928 (Source: Obesity)
Source: Obesity - November 3, 2023 Category: Eating Disorders & Weight Management Authors: Jennifer L Miller Evelien Gevers Nicola Bridges Jack A Yanovski Parisa Salehi Kathryn S Obrynba Eric I Felner Lynne M Bird Ashley H Shoemaker Moris Angulo Merlin G Butler David Stevenson Anthony P Goldstone John Wilding Melissa Lah M Guftar Shaikh Elizabe Source Type: research
Diazoxide choline extended-release tablet in people with Prader-Willi syndrome: results from long-term open-label study
CONCLUSIONS: DCCR administration to people with PWS was well-tolerated and associated with broad-ranging improvements in the syndrome. Sustained administration of DCCR has the potential to reduce disease severity and the burden of care for families.PMID:37919617 | DOI:10.1002/oby.23928 (Source: Obesity)
Source: Obesity - November 3, 2023 Category: Eating Disorders & Weight Management Authors: Jennifer L Miller Evelien Gevers Nicola Bridges Jack A Yanovski Parisa Salehi Kathryn S Obrynba Eric I Felner Lynne M Bird Ashley H Shoemaker Moris Angulo Merlin G Butler David Stevenson Anthony P Goldstone John Wilding Melissa Lah M Guftar Shaikh Elizabe Source Type: research
Disengagement of somatostatin neurons from lateral septum circuitry by oxytocin and vasopressin restores social-fear extinction and suppresses aggression outbursts in Prader-Willi syndrome model
Responding to social signals by expressing the correct behavior is not only challenged in autism, but also in diseases with high prevalence of autism, like Prader-Willi Syndrome (PWS). Clinical evidence suggests aberrant pro-social behavior in patients can be regulated by intranasal oxytocin (OXT) or vasopressin (AVP). However, what neuronal mechanisms underlie impaired behavioral responses in a socially-aversive context, and how can they be corrected, remains largely unknown. (Source: Biological Psychiatry)
Source: Biological Psychiatry - October 27, 2023 Category: Psychiatry Authors: Yann Dromard, Am élie M. Borie, Prabahan Chakraborty, Françoise Muscatelli, Gilles Guillon, Michel G. Desarménien, Freddy Jeanneteau Tags: Archival Report Source Type: research
Hypoventilation in patients with Prader-Willi syndrome across the pediatric age
Conclusion The frequency of alveolar hypoventilation in children and adolescents with PWS is of concern and increases with age. A regular screening by oximetry-capnography would seem to be indicated whatever the sex, the BMI, the rate of obstructive or central apneas, the presence of a GH treatment or not.Key words Prader-Willy syndrome, child, polysomnography, sleep-disordered breathing, alveolar hypoventilation, growth hormone (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 27, 2023 Category: Respiratory Medicine Authors: Chen, C., Ioan, I., Thieux, M., Nicolino, M., Franco, P., Coutier, L., Coutier, L. Tags: Clinical and epidemiological respiratory sleep medicine Source Type: research
Management of Monogenic and Syndromic Obesity
Similar to the general population, lifestyle interventions focused on nutrition and physical activity form the foundation for treating obesity caused by rare genetic disorders. Additional therapies, including metreleptin and setmelanotide, that target defects within the leptin signaling pathway can effectively synergize with lifestyle efforts to treat monogenic disorders of leptin, leptin receptor, proopiomelanocortin (POMC), and proprotein convertase subtilisin/kexin type 1 (PCSK1) and syndromic conditions, such as the ciliopathies Bardet-Biedl and Alstr öm syndromes, whose pathophysiological mechanisms also converge on ...
Source: Gastroenterology Clinics of North America - September 27, 2023 Category: Gastroenterology Authors: Joan C. Han, Marcus C. Rasmussen, Alison R. Forte, Stephanie B. Schrage, Sarah K. Zafar, Andrea M. Haqq Source Type: research
