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PWS is characterized by a complex lifelong trajectory involving neurodevelopmental, nutritional, endocrine, metabolic, and behavioral changes. The major symptoms are hypotonia, short stature, hypogonadism, and eating disorders ranging from anorexia in infancy to hyperphagia, a deficit of satiety, and a high risk of severe obesity. The patients display intellectual disability comprising cognitive deficit, delayed motor and language development, learning deficits, impaired social skills, and emotional regulation. Behavioral features including temper outbursts, anxiety, obsessive\u2013compulsive symptoms and rigidity are common and become more apparent with increasing age. Almost all have hypogonadism and growth hormone deficiency. Central adrenal insufficiency is rare whereas central hypothyroidism occurs in up to 30% of children with PWS. The prevalence of obesity increases with age from almost none in early childhood to more than 90% in adulthood. Up to 25% of adults with obesity have type 2 diabetes. Obesity and its complications are the major causes of comorbidity and mortality in PWS. As there is no specific treatment, care consists of comprehensive management of feeding disorders, a restricted, controlled diet, regular exercise, hormone substitution, and screening and treatment of comorbidities. Here we present the course of PWS from birth to adulthood in 2 patients and discuss their symptoms in relation to the literature.<\/jats:p>","DOI":"10.1210\/clinem\/dgac082","type":"journal-article","created":{"date-parts":[[2022,2,12]],"date-time":"2022-02-12T18:37:25Z","timestamp":1644691045000},"page":"1698-1705","source":"Crossref","is-referenced-by-count":37,"title":["Approach to the Patient With Prader\u2013Willi Syndrome"],"prefix":"10.1210","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3980-1927","authenticated-orcid":false,"given":"Charlotte","family":"H\u00f6ybye","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Karolinska University Hospital , Stockholm Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institute , Stockholm , Sweden"},{"name":"The Clinical and Scientific Advisory Board of the International Organization for Prader-Willi Syndrome, IPWSO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0363-7862","authenticated-orcid":false,"given":"Maith\u00e9","family":"Tauber","sequence":"additional","affiliation":[{"name":"The Clinical and Scientific Advisory Board of the International Organization for Prader-Willi Syndrome, IPWSO"},{"name":"Centre de R\u00e9f\u00e9rence Maladies Rares PRADORT (syndrome de PRADer-Willi et autres Ob\u00e9sit\u00e9s Rares avec Troubles du comportement alimentaire), H\u00f4pital des Enfants, CHU Toulouse, Universit\u00e9 Toulouse III , Toulouse , France"},{"name":"Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity) INSERM UMR1291\u2014CNRS UMR5051\u2014Universit\u00e9 Toulouse III, Toulouse , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2022,2,12]]},"reference":[{"issue":"4","key":"2022110412035830600_CIT0001","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/S2213-8587(21)00002-4","article-title":"Endocrine disorders in Prader-Willi syndrome: a model to understand and treat hypothalamic dysfunction","volume":"9","author":"Tauber","year":"2021","journal-title":"Lancet Diabetes Endocrinol."},{"issue":"2","key":"2022110412035830600_CIT0002","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1002\/ajmg.a.36845","article-title":"A population-based profile of 160 Australians with Prader-Willi syndrome: trends in diagnosis, birth prevalence and birth characteristics","volume":"167A","author":"Lionti","year":"2015","journal-title":"Am J Med Genet A."},{"issue":"1","key":"2022110412035830600_CIT0003","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1186\/s13023-017-0673-6","article-title":"Early diagnosis and care is achieved but should be improved in infants with Prader-Willi syndrome","volume":"12","author":"Bar","year":"2017","journal-title":"Orphanet J Rare Dis."},{"key":"2022110412035830600_CIT0004","first-page":"1993","article-title":"Prader-Willi syndrome. 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