{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T04:05:32Z","timestamp":1792037132386,"version":"4.4.0"},"reference-count":61,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T00:00:00Z","timestamp":1743724800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Obesity Reviews"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Bardet\u2013Biedl syndrome (BBS) is a rare, genetically heterogeneous, and highly pleiotropic autosomal recessive ciliopathy. Patients typically present with early loss of vision, hyperphagia, severe obesity, learning difficulties, and renal dysfunction. In patients with BBS, dysfunction of the immotile primary cilia in the hypothalamic melanocortin\u20104 receptor (MC4R) pathway responsible for controlling energy balance, hunger, and satiety results in severe hyperphagia manifesting in food\u2010seeking behaviors that drive the development of obesity early in childhood. These behaviors have negative impacts on many areas of the lives of patients with BBS and their families\/caregivers, including sleep, mood, school\/work, and social\/family relationships. Additionally, many patients feel stigmatized due to their hyperphagia\u2010associated food\u2010seeking behaviors and the resulting obesity, which exacerbates the impacts of hyperphagia on quality of life. Early identification and management of hyperphagia in patients with BBS is key: mitigating food\u2010seeking and weight gain can improve quality of life and reduce the risk of metabolic and cardiovascular diseases that is increased in patients with BBS. Until recently, the only treatment strategies available were lifestyle and diet modifications. However, targeted treatment with the novel MC4R agonist setmelanotide now offers an effective management option to reduce hyperphagia and weight in patients with BBS, improving overall health and quality of life.<\/jats:p>","DOI":"10.1111\/obr.13915","type":"journal-article","created":{"date-parts":[[2025,4,5]],"date-time":"2025-04-05T18:55:21Z","timestamp":1743879321000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Hyperphagia in Bardet\u2013Biedl syndrome: Pathophysiology, burden, and management"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9164-9782","authenticated-orcid":false,"given":"Philip L.","family":"Beales","sequence":"first","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health  London 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