{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T05:42:12Z","timestamp":1790487732383,"version":"4.1.0"},"reference-count":29,"publisher":"The Endocrine Society","issue":"7","license":[{"start":{"date-parts":[[2020,3,31]],"date-time":"2020-03-31T00:00:00Z","timestamp":1585612800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Centraal Ziekenfonds"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Context<\/jats:title><jats:p>Prader\u2013Willi syndrome (PWS) is associated with several hypothalamic-pituitary hormone deficiencies. There is no agreement on the prevalence of central adrenal insufficiency (CAI) in adults with PWS. In some countries, it is general practice to prescribe stress-dose hydrocortisone during physical or psychological stress in patients with PWS. Side effects of frequent hydrocortisone use are weight gain, osteoporosis, diabetes mellitus, and hypertension\u2014already major problems in adults with PWS. However, undertreatment of CAI can cause significant morbidity\u2014or even mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To prevent both over- and undertreatment with hydrocortisone, we assessed the prevalence of CAI in a large international cohort of adults with PWS. As the synacthen test shows variable results in PWS, we only use the metyrapone test (MTP) and insulin tolerance test (ITT).<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>Metyrapone test or ITT in adults with PWS (N\u2005=\u200582) and review of medical files for symptoms of hypocortisolism related to surgery (N\u2005=\u2005645).<\/jats:p><\/jats:sec><jats:sec><jats:title>Setting<\/jats:title><jats:p>Outpatient clinic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients or Other Participants<\/jats:title><jats:p>Eighty-two adults with genetically confirmed PWS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Main Outcome Measure<\/jats:title><jats:p>For MTP, 11-deoxycortisol\u2005&amp;gt;\u2005230 nmol\/L was considered sufficient. For ITT, cortisol\u2005&amp;gt;\u2005500 nmol\/L (Dutch, French, and Swedish patients) or\u2005&amp;gt;\u2005450 nmol\/L (British patients) was considered sufficient.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Central adrenal insufficiency was excluded in 81 of 82 patients. Among the 645 patients whose medical files were reviewed, 200 had undergone surgery without perioperative hydrocortisone treatment. None of them had displayed any features of hypocortisolism.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Central adrenal insufficiency is rare (1.2%) in adults with PWS. Based on these results, we recommend against routinely prescribing hydrocortisone stress-doses in adults with PWS.<\/jats:p><\/jats:sec>","DOI":"10.1210\/clinem\/dgaa168","type":"journal-article","created":{"date-parts":[[2020,3,31]],"date-time":"2020-03-31T08:55:08Z","timestamp":1585644908000},"page":"e2563-e2571","source":"Crossref","is-referenced-by-count":38,"title":["Central Adrenal Insufficiency Is Rare in Adults With Prader\u2013Willi Syndrome"],"prefix":"10.1210","volume":"105","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8333-1512","authenticated-orcid":false,"given":"Anna G W","family":"Rosenberg","sequence":"first","affiliation":[{"name":"Internal Medicine, Division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karlijn","family":"Pellikaan","sequence":"additional","affiliation":[{"name":"Internal Medicine, Division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Poitou","sequence":"additional","affiliation":[{"name":"Assistance Publique-Hopitaux de Paris, Nutrition Department, Institute of Cardiometabolism and Nutrition, Pitie-Salpetriere Hospital, Sorbonne Universite, Paris, France"},{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"European Reference Network on Rare Endocrine Conditions"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony P","family":"Goldstone","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"PsychoNeuroEndocrinology Research Group, Neuropsychopharmacology Unit, Division of Psychiatry, Computational, Cognitive and Clinical Neuroimaging Laboratory, Department of Brain Sciences, Faculty of Medicine, Imperial College London, Hammersmith Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"H\u00f8ybye","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"European Reference Network on Rare Endocrine Conditions"},{"name":"Department of Molecular Medicine and Surgery, Patient Area Endocrinology and Nephrology, Inflammation and Infection Theme, Karolinska Institute and Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania","family":"Markovic","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"Metabolism & Obesity Services, Royal Prince Alfred Hospital, Camperdown, Australia"},{"name":"Boden Collaboration, University of Sydney, Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graziano","family":"Grugni","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"European Reference Network on Rare Endocrine Conditions"},{"name":"Divison of Auxology, Italian Auxological Institute, IRCCS, Piancavallo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonino","family":"Crin\u00f2","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"Reference Center for Prader\u2013Willi Syndrome, Bambino Gesu Hospital, Research Institute, Palidoro (Rome), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Assumpta","family":"Caix\u00e0s","sequence":"additional","affiliation":[{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"Department of Endocrinology and Nutrition, Hospital Universitari Parc Taul\u00ed (UAB), Institut d\u2019Investigacio i Innovacio Parc Taul\u00ed (I3PT), Sabadell, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muriel","family":"Coupaye","sequence":"additional","affiliation":[{"name":"Assistance Publique-Hopitaux de Paris, Nutrition Department, Institute of Cardiometabolism and Nutrition, Pitie-Salpetriere Hospital, Sorbonne Universite, Paris, France"},{"name":"International Network for Research, Management & Education on Adults with PWS"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sjoerd A A","family":"Van Den Berg","sequence":"additional","affiliation":[{"name":"Internal Medicine, Division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"},{"name":"Department of Clinical Chemistry, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aart Jan","family":"Van Der Lely","sequence":"additional","affiliation":[{"name":"Internal Medicine, Division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"},{"name":"European Reference Network on Rare Endocrine Conditions"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0295-7063","authenticated-orcid":false,"given":"Laura C G","family":"De Graaff","sequence":"additional","affiliation":[{"name":"Internal Medicine, Division of Endocrinology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"},{"name":"International Network for Research, Management & Education on Adults with PWS"},{"name":"European Reference Network on Rare Endocrine Conditions"},{"name":"Academic Center for Growth, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The 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