Editorial
Prader-Willi syndrome: Making progress, one step at a time
Síndrome de Prader-Willi: avanzando paso a paso
Assumpta Caixàs
Servicio de Endocrinología y Nutrición, Hospital Universitari Parc Taulí, Institut d’Investigació i Innovació Parc Taulí (I3PT), Departament Medicina Universitat Autònoma de Barcelona, Sabadell, Spain
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Cl<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>Chile, Col<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>Colombia.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Felipe Caamaño-Navarrete, Iris Paola Guzmán-Guzmán, Constanza Palomino-Devia, Felipe Augusto Reyes-Oyola, Rocío Bustos-Barahona, Daniel Jerez-Mayorga, Pedro Delgado-Floody" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Felipe" "apellidos" => "Caamaño-Navarrete" ] 1 => array:2 [ "nombre" => "Iris Paola" "apellidos" => "Guzmán-Guzmán" ] 2 => array:2 [ "nombre" => "Constanza" "apellidos" => "Palomino-Devia" ] 3 => array:2 [ "nombre" => "Felipe Augusto" "apellidos" => "Reyes-Oyola" ] 4 => array:2 [ "nombre" => "Rocío" "apellidos" => "Bustos-Barahona" ] 5 => array:2 [ "nombre" => "Daniel" "apellidos" => "Jerez-Mayorga" ] 6 => array:2 [ "nombre" => "Pedro" "apellidos" => "Delgado-Floody" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2530018022000130?idApp=UINPBA00004N" "url" => "/25300180/0000006900000001/v1_202202280604/S2530018022000130/v1_202202280604/en/main.assets" ] "en" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Editorial</span>" "titulo" => "Prader-Willi syndrome: Making progress, one step at a time" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "1" "paginaFinal" => "3" ] ] "autores" => array:1 [ 0 => array:3 [ "autoresLista" => "Assumpta Caixàs" "autores" => array:1 [ 0 => array:3 [ "nombre" => "Assumpta" "apellidos" => "Caixàs" "email" => array:1 [ 0 => "acaixas@gmail.com" ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Endocrinología y Nutrición, Hospital Universitari Parc Taulí, Institut d’Investigació i Innovació Parc Taulí (I3PT), Departament Medicina Universitat Autònoma de Barcelona, Sabadell, Spain" "identificador" => "aff0005" ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Prader-Willi: avanzando paso a paso" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Prader-Willi syndrome (PWS) is a genetic neurodevelopmental disorder. It has an estimated prevalence of between 1:10,000 and 1:30,000 live births and is caused by a lack of expression of genes of paternal origin in the 15q11–q13 chromosomal region. Genetic subtypes can be paternal microdeletion (70%–75% of cases), maternal disomy (20%–25%), imprinting defect (1%–3%) or chromosomal translocations (less than 1%).<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The clinical manifestations of PWS include short stature due to lack of growth hormone (GH), kyphoscoliosis, hypopigmentation, hypogonadotropic hypogonadism, small hands and feet, narrow bifrontal diameter, inverted U-shaped mouth with thin upper lip, and eye abnormalities (myopia, strabismus or almond-shaped palpebral fissures). There is usually decreased foetal movement during pregnancy, neonatal hypotonia with suction difficulties in the perinatal stage, and infant lethargy, which improves with age. Additionally, exaggerated hyperphagia and lack of satiety are very characteristic, which leads sufferers to desperately search for food and eat anything (even if is in poor condition) and, over time, to morbid obesity with multiple associated comorbidities, with these being the main cause of mortality in this population.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In recent decades, interest in understanding the syndrome and developing drugs that can reduce this exaggerated hyperphagia has led some pharmaceutical companies to focus their attention on including this type of patient in their clinical trials. Unfortunately, some drugs indicated for PWS failed to reach the market due to suspension of the trial because of side effects. One due to thrombosis and death from pulmonary thromboembolism (in the case of beloranib, an inhibitor of the enzyme methionine aminopeptidase-2<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>); others due to ineffectiveness (livoletide, a deacylated ghrelin analogue,<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and carbetocin, an oxytocin analogue<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a>). Others are still under development, such as controlled-release diazoxide choline,<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> intranasal oxytocin,<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,8</span></a> and drugs that inhibit the endocannabinoid CB1 receptor,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> and once again there is a glimmer of hope.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The scientific community and clinical experts are working constantly to better understand the pathophysiology of this syndrome and to be able to give more precise action guidelines to improve the health and quality of life of these patients and their caregivers.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Our group is a reference in PWS both at a clinical and research level and has contributed to the knowledge of different aspects of the syndrome. Regarding hyperphagia, we have studied the role of hunger and satiety peptides, such as ghrelin, polypeptide YY (PYY)<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> and brain-derived neurotrophic factor (BDNF).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> However, we have recently observed that, when analysing nine peptides in pre- and postprandial state in three groups of adult subjects (patients with PWS, patients with obesity and healthy controls) by means of cluster analysis, fasting peptides discriminate better than postprandial peptides, a hormonal pattern characteristic of this syndrome. This pattern consists of elevated levels of ghrelin, leptin, PYY, GLP1, GIP and suggests not only an excess of the hunger hormone (ghrelin), but also some resistance to the action of satiating hormones. Therefore, new drugs should be aimed not only at increasing these satiating hormones but also at modifying them to allow them to overcome this resistance.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">By investigating brain function in PWS patients using functional magnetic resonance imaging in relation to different aspects of the syndrome, in terms of food, we were able to observe that, while viewing images of spoiled food, cortical brain activity in areas related to visually evoked disgust (anterior insula, frontal operculum) was decreased compared to healthy controls. Moreover, there was little activity at the subcortical level in the limbic structures (hypothalamus, amygdala, hippocampus and periaqueductal grey matter), which are areas related to the instinct to react in this situation. This implies that patients with PWS can come to discern that the food is theoretically inappropriate, but they eat it anyway because their instinct does not lead them to avoid it.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Regarding motor aspects, we observed a dysfunction at the level of the cerebellum to carry out complicated motor tasks that involve coordination,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> as well as an alteration in the ability to imitate gestures with a relatively preserved visuospatial praxis, which did not correlate with any specific anatomical alteration at the level of the brain. Rather, it seems more related to diffuse cerebral dysfunction.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The behavioural aspects have been widely described in a recent consensus.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> The best known is obsessive-compulsive behaviour,<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> with an obsession with food and the repetition of certain acts such as scratching of the skin or anus. The alterations in functional brain connectivity observed that may explain this behaviour are broader and more subcortical than those of patients with classic obsessive-compulsive disorder.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">A novel aspect is evaluating these patients’ awareness of the disease. Our group has validated a test for its evaluation<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">19</span></a> and has been able to observe that these patients are quite aware of the disease and the effects of the medication, but not of its social consequences. These aspects may be relevant for adherence to treatment and for their relationships with their companions and caregivers.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">20</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The International Network for Research, Management and Education on adults with PWS (INfoRMEd-PWS), created in 2019, has contributed with several publications on different endocrinological aspects of PWS, such as central adrenal insufficiency,<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">21</span></a> hyponatraemia,<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">22</span></a> hypogonadism<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">23</span></a> or treatment with GH in adults.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">24</span></a> In all of them an attempt has been made to provide a series of guidelines for the diagnosis and treatment of each of these anomalies.</p><p id="par0055" class="elsevierStylePara elsevierViewall">A registry of patients with PWS in Spain has recently been started by the Spanish societies of Endocrinology and Nutrition (SEEN), Obesity (SEEDO) and Paediatric Endocrinology (SEEP), which we hope will be useful for future studies and clinical trials aimed at improving the quality of life of these patients.</p><p id="par0060" class="elsevierStylePara elsevierViewall">To conclude, we would like to highlight the great work carried out by patient associations at the local level (Catalan, Valencian, Andalusian and Spanish) and the International Prader-Willi Syndrome Organisation (IPWSO), in supporting families and caregivers while at the same time always being willing to collaborate with the scientific community. Without this symbiosis it would be impossible to advance step by step in the treatment of this complex syndrome.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Caixàs A. Síndrome de Prader-Willi: avanzando paso a paso. Endocrinol Diabetes Nutr. 2022;69:1–3.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:24 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "S.B. Cassidy" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/jmg.34.11.917" "Revista" => array:6 [ "tituloSerie" => "J Med Genet" "fecha" => "1997" "volumen" => "34" "paginaInicial" => "917" "paginaFinal" => "923" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9391886" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Survival trends from the Prader-Willi Syndrome Association (USA) 40-year mortality survey" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "A.M. Manzardo" 1 => "J. Loker" 2 => "J. Heinemann" 3 => "C. Loker" 4 => "M.G. Butler" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1038/gim.2017.92" "Revista" => array:6 [ "tituloSerie" => "Genet Med" "fecha" => "2018" "volumen" => "20" "paginaInicial" => "24" "paginaFinal" => "30" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28682308" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Effects of MetAP2 inhibition on hyperphagia and body weight in Prader-Willi syndrome: a randomized, double-blind, placebo-controlled trial" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S.E. McCandless" 1 => "J.A. Yanovski" 2 => "J. Miller" 3 => "C. Fu" 4 => "L.M. Bird" 5 => "P. Salehi" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/dom.13021" "Revista" => array:6 [ "tituloSerie" => "Diabetes Obes Metab" "fecha" => "2017" "volumen" => "19" "paginaInicial" => "1751" "paginaFinal" => "1761" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28556449" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "AZP-531, an unacylated ghrelin analog, improves food-related behavior in patients with Prader-Willi syndrome: a randomized placebo-controlled trial" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S. Allas" 1 => "A. Caixàs" 2 => "C. Poitou" 3 => "M. Coupaye" 4 => "D. Thuilleaux" 5 => "F. Lorenzini" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1371/journal.pone.0190849" "Revista" => array:5 [ "tituloSerie" => "PLoS One" "fecha" => "2018" "volumen" => "13" "paginaInicial" => "e0190849" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29320575" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Intranasal carbetocin reduces hyperphagia in individuals with Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "E.M. Dykens" 1 => "J. Miller" 2 => "M. Angulo" 3 => "E. Roof" 4 => "M. Reidy" 5 => "H.T. Hatoum" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "JCI Insight" "fecha" => "2018" "volumen" => "3" "paginaInicial" => "e98333" ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0030" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A randomized pilot efficacy and safety trial of diazoxide choline controlled-release in patients with Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "V. Kimonis" 1 => "A. Surampalli" 2 => "M. Wencel" 3 => "J.A. Gold" 4 => "N.M. Cowen" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1371/journal.pone.0221615" "Revista" => array:5 [ "tituloSerie" => "PLoS One" "fecha" => "2019" "volumen" => "14" "paginaInicial" => "e0221615" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31545799" "web" => "Medline" ] ] ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0035" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Intranasal oxytocin versus placebo for hyperphagia and repetitive behaviors in children with Prader-Willi syndrome: a randomized controlled pilot trial" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "E. Hollander" 1 => "K.G. Levine" 2 => "C.J. Ferretti" 3 => "K. Freeman" 4 => "E. Doernberg" 5 => "N. Desilva" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jpsychires.2020.11.006" "Revista" => array:6 [ "tituloSerie" => "J Psychiatr Res" "fecha" => "2021" "volumen" => "137" "paginaInicial" => "643" "paginaFinal" => "651" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/33190843" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0040" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Oxytocin in young children with Prader-Willi syndrome: results of a randomized, double-blind, placebo-controlled, crossover trial investigating 3 months of oxytocin" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Damen" 1 => "L.N. Grootjen" 2 => "A.F. Juriaans" 3 => "S.H. Donze" 4 => "T.M. Huisman" 5 => "J.A. Visser" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Clin Endocrinol (Oxf)" "fecha" => "2021" "volumen" => "94" "paginaInicial" => "774" "paginaFinal" => "785" ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0045" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Targeting the endocannabinoid/CB1 receptor system for treating obesity in Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "I. Knani" 1 => "B.J. Earley" 2 => "S. Udi" 3 => "A. Nemirovski" 4 => "R. Hadar" 5 => "A. Gammal" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.molmet.2016.10.004" "Revista" => array:6 [ "tituloSerie" => "Mol Metab" "fecha" => "2016" "volumen" => "5" "paginaInicial" => "1187" "paginaFinal" => "1199" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27900261" "web" => "Medline" ] ] ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0050" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A lesser postprandial suppression of plasma ghrelin in Prader-Willi syndrome is associated with low fasting and a blunted postprandial PYY response" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "O. Giménez-Palop" 1 => "G. Giménez-Pérez" 2 => "D. Mauricio" 3 => "J.M. González-Clemente" 4 => "N. Potau" 5 => "E. Berlanga" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Clin Endocrinol (Oxf)" "fecha" => "2007" "volumen" => "66" "paginaInicial" => "198" "paginaFinal" => "204" ] ] ] ] ] ] 10 => array:3 [ "identificador" => "bib0055" "etiqueta" => "11" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lack of postprandial peak in brain-derived neurotrophic factor in adults with Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Bueno" 1 => "S. Esteba-Castillo" 2 => "R. Novell" 3 => "O. Giménez-Palop" 4 => "R. Coronas" 5 => "E. Gabau" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1371/journal.pone.0163468" "Revista" => array:5 [ "tituloSerie" => "PLoS One" "fecha" => "2016" "volumen" => "11" "paginaInicial" => "e0163468" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27685845" "web" => "Medline" ] ] ] ] ] ] ] ] 11 => array:3 [ "identificador" => "bib0060" "etiqueta" => "12" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hunger and satiety peptides: is there a pattern to classify patients with Prader-Willi syndrome?" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Bueno" 1 => "E. Boixadera-Planas" 2 => "L. Blanco-Hinojo" 3 => "S. Esteba-Castillo" 4 => "O. Giménez-Palop" 5 => "Torrents-Rodas" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3390/jcm10215170" "Revista" => array:5 [ "tituloSerie" => "J Clin Med" "fecha" => "2021" "volumen" => "10" "paginaInicial" => "5170" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34768690" "web" => "Medline" ] ] ] ] ] ] ] ] 12 => array:3 [ "identificador" => "bib0065" "etiqueta" => "13" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lack of response to disgusting food in the hypothalamus and related structures in Prader Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Blanco-Hinojo" 1 => "J. Pujol" 2 => "S. Esteba-Castillo" 3 => "G. Martínez-Vilavella" 4 => "O. Giménez-Palop" 5 => "E. Gabau" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.nicl.2019.101662" "Revista" => array:5 [ "tituloSerie" => "Neuroimage Clin" "fecha" => "2019" "volumen" => "21" "paginaInicial" => "101662" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30639180" "web" => "Medline" ] ] ] ] ] ] ] ] 13 => array:3 [ "identificador" => "bib0070" "etiqueta" => "14" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cerebellar dysfunction in adults with prader willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Blanco-Hinojo" 1 => "L. Casamitjana" 2 => "J. Pujol" 3 => "G. Martínez-Vilavella" 4 => "S. Esteba-Castillo" 5 => "O. Giménez-Palop" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3390/jcm10153320" "Revista" => array:5 [ "tituloSerie" => "J Clin Med" "fecha" => "2021" "volumen" => "10" "paginaInicial" => "3320" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34362104" "web" => "Medline" ] ] ] ] ] ] ] ] 14 => array:3 [ "identificador" => "bib0075" "etiqueta" => "15" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Altered gesture imitation and brain anatomy in adult Prader-Willi syndrome patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Caixàs" 1 => "L. Blanco-Hinojo" 2 => "J. Pujol" 3 => "J. Deus" 4 => "O. Giménez-Palop" 5 => "D. Torrents-Rodas" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1017/S1355617721000060" "Revista" => array:6 [ "tituloSerie" => "J Int Neuropsychol Soc" "fecha" => "2021" "volumen" => "27" "paginaInicial" => "1024" "paginaFinal" => "1036" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/33660593" "web" => "Medline" ] ] ] ] ] ] ] ] 15 => array:3 [ "identificador" => "bib0080" "etiqueta" => "16" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Schwartz" 1 => "A. Caixàs" 2 => "A. Dimitropoulos" 3 => "E. Dykens" 4 => "J. Duis" 5 => "S. Einfeld" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/s11689-021-09373-2" "Revista" => array:5 [ "tituloSerie" => "J Neurodev Disord" "fecha" => "2021" "volumen" => "13" "paginaInicial" => "25" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34148559" "web" => "Medline" ] ] ] ] ] ] ] ] 16 => array:3 [ "identificador" => "bib0085" "etiqueta" => "17" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Compulsions in Prader-Willi syndrome: occurrence and severity as a function of genetic subtype" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R. Novell-Alsina" 1 => "S. Esteba-Castillo" 2 => "A. Caixàs" 3 => "E. Gabau" 4 => "O. Giménez-Palop" 5 => "J. Pujol" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Actas Esp Psiquiatr" "fecha" => "2019" "volumen" => "47" "paginaInicial" => "79" "paginaFinal" => "87" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31233206" "web" => "Medline" ] ] ] ] ] ] ] ] 17 => array:3 [ "identificador" => "bib0090" "etiqueta" => "18" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anomalous basal ganglia connectivity and obsessive-compulsive behaviour in patients with Prader Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Pujol" 1 => "L. Blanco-Hinojo" 2 => "S. Esteba-Castillo" 3 => "A. Caixàs" 4 => "B.J. Harrison" 5 => "M. Bueno" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1503/jpn.140338" "Revista" => array:6 [ "tituloSerie" => "J Psychiatry Neurosci" "fecha" => "2016" "volumen" => "41" "paginaInicial" => "261" "paginaFinal" => "271" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26645739" "web" => "Medline" ] ] ] ] ] ] ] ] 18 => array:3 [ "identificador" => "bib0095" "etiqueta" => "19" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An adapted scale to evaluate insight in Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "J. Cobo" 1 => "R. Coronas" 2 => "E. Pousa" 3 => "J.C. Oliva" 4 => "O. Giménez-Palop" 5 => "A. Caixàs" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:2 [ "tituloSerie" => "Med Clin (Barc)" "fecha" => "2021" ] ] ] ] ] ] 19 => array:3 [ "identificador" => "bib0100" "etiqueta" => "20" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Multidimensional evaluation of awareness in Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Cobo" 1 => "R. Coronas" 2 => "E. Pousa" 3 => "J.C. Oliva" 4 => "O. Giménez-Palop" 5 => "S. Esteba-Castillo" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3390/jcm10092007" "Revista" => array:5 [ "tituloSerie" => "J Clin Med" "fecha" => "2021" "volumen" => "10" "paginaInicial" => "2007" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34067179" "web" => "Medline" ] ] ] ] ] ] ] ] 20 => array:3 [ "identificador" => "bib0105" "etiqueta" => "21" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Central adrenal insufficiency is rare in adults with Prader-Willi syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A.G.W. Rosenberg" 1 => "K. Pellikaan" 2 => "C. Poitou" 3 => "A.P. Goldstone" 4 => "C. Høybye" 5 => "T. Markovic" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1210/clinem/dgaa543" "Revista" => array:4 [ "tituloSerie" => "J Clin Endocrinol Metab" "fecha" => "2020" "volumen" => "105" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32810255" "web" => "Medline" ] ] ] ] ] ] ] ] 21 => array:3 [ "identificador" => "bib0110" "etiqueta" => "22" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hyponatremia in children and adults with Prader-Willi syndrome: a survey involving seven countries" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Coupaye" 1 => "K. Pellikaan" 2 => "A.P. Goldstone" 3 => "A. Crinò" 4 => "G. Grugni" 5 => "T.P. Markovic" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3390/jcm10163555" "Revista" => array:5 [ "tituloSerie" => "J Clin Med" "fecha" => "2021" "volumen" => "10" "paginaInicial" => "3555" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34441851" "web" => "Medline" ] ] ] ] ] ] ] ] 22 => array:3 [ "identificador" => "bib0115" "etiqueta" => "23" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Hypogonadism in adult males with Prader-Willi syndrome-clinical recommendations based on a Dutch cohort study, review of the literature and an international expert panel discussion" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "K. Pellikaan" 1 => "Y. Ben Brahim" 2 => "A.G.W. Rosenberg" 3 => "K. Davidse" 4 => "C. Poitou" 5 => "M. Coupaye" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3390/jcm10194361" "Revista" => array:6 [ "tituloSerie" => "J Clin Med" "fecha" => "2021" "volumen" => "10" "numero" => "September" "paginaInicial" => "4361" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34640379" "web" => "Medline" ] ] ] ] ] ] ] ] 23 => array:3 [ "identificador" => "bib0120" "etiqueta" => "24" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Growth hormone treatment for adults with Prader-Willi syndrome: a meta-analysis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A.G.W. Rosenberg" 1 => "C.G.B. Passone" 2 => "K. Pellikaan" 3 => "D. Damiani" 4 => "A.J. van der Lely" 5 => "M. Polak" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1210/clinem/dgab406" "Revista" => array:6 [ "tituloSerie" => "J Clin Endocrinol Metab" "fecha" => "2021" "volumen" => "106" "paginaInicial" => "3068" "paginaFinal" => "3091" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/34105729" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/25300180/0000006900000001/v1_202202280604/S2530018022000051/v1_202202280604/en/main.assets" "Apartado" => array:4 [ "identificador" => "64497" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Editorial" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/25300180/0000006900000001/v1_202202280604/S2530018022000051/v1_202202280604/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2530018022000051?idApp=UINPBA00004N" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 4 | 1 | 5 |
| 2024 October | 31 | 3 | 34 |
| 2024 September | 54 | 3 | 57 |
| 2024 August | 27 | 3 | 30 |
| 2024 July | 19 | 9 | 28 |
| 2024 June | 13 | 0 | 13 |
| 2024 May | 21 | 3 | 24 |
| 2024 April | 25 | 14 | 39 |
| 2024 March | 34 | 6 | 40 |
| 2024 February | 47 | 4 | 51 |
| 2024 January | 48 | 9 | 57 |
| 2023 December | 39 | 6 | 45 |
| 2023 November | 31 | 5 | 36 |
| 2023 October | 70 | 11 | 81 |
| 2023 September | 21 | 3 | 24 |
| 2023 August | 19 | 2 | 21 |
| 2023 July | 9 | 1 | 10 |
| 2023 June | 34 | 7 | 41 |
| 2023 May | 50 | 3 | 53 |
| 2023 April | 43 | 0 | 43 |
| 2023 March | 43 | 5 | 48 |
| 2023 February | 27 | 5 | 32 |
| 2023 January | 10 | 3 | 13 |
| 2022 December | 8 | 8 | 16 |
| 2022 November | 5 | 10 | 15 |
| 2022 October | 12 | 5 | 17 |
| 2022 September | 12 | 13 | 25 |
| 2022 August | 33 | 6 | 39 |
| 2022 July | 24 | 9 | 33 |
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