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Prader–Willi syndrome
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== Treatment == While PWS has no cure, several treatments are available to lessen the condition's symptoms. During infancy, subjects should undergo therapies to improve muscle strength. Speech and occupational therapy are also indicated. During the school years, children benefit from a highly structured learning environment and extra help. The largest problem associated with the syndrome is severe obesity. Access to food must be strictly supervised and limited, usually by installing locks on all food-storage places, including refrigerators.<ref name="treatments" /> <!-- why? How are these related? Because hypotonia can be a symptom of PWS, it is vital to provide proper nutrition during infancy. -->Physical activity in individuals with PWS for all ages is needed to optimize strength and promote a healthy lifestyle.<ref name=Cas2008 /> An intranasal formulation of [[betahistine]] dihydrochloride received [[orphan drug]] designation from the US [[Food and Drug Administration]] (FDA) for the treatment of obesity associated with Prader–Willi syndrome.<ref name="FDA orphan">{{cite web |title=Orphan Drug Designations and Approvals-Betahistine dihydrochloride |website=US Food and Drug Administration |url=https://www.accessdata.fda.gov/scripts/opdlisting/oopd/detailedIndex.cfm?cfgridkey=247407 |access-date=31 December 2024}}</ref><ref name="Melão 2018">{{cite web |last=Melão |first=Alice |title=FDA Grants Orphan Drug Status to Auris' Intranasal Betahistine for Treatment of PWS-linked Obesity|website=Prader-Willi Syndrome News |date=17 December 2018 |url=https://praderwillinews.com/news/intranasal-betahistine-granted-fda-orphan-drug-status/ |access-date=31 December 2024}}</ref><ref name="Timmins 2018">{{cite journal |vauthors=Timmins P |title=Industry Update: the Latest Developments in the Field of Therapeutic delivery, 1–31 December 2018 |journal=Therapeutic Delivery |volume=10 |issue=4 |date=2019 |issn=2041-5990 |doi=10.4155/tde-2019-0003 |pages=215–226}}</ref> In March 2025, the FDA approved a drug, to be called Vykat XR, that treats the acute hunger that is a symptom of PWS.<ref>[https://www.reuters.com/business/healthcare-pharmaceuticals/us-fda-greenlights-first-ever-treatment-rare-metabolic-disorder-2025-03-26/ US FDA approves first treatment for rare genetic disorder Prader-Willi syndrome]. [[Reuters]]. March 26, 2025</ref> Prescription of daily recombinant growth hormone injections is indicated for children with PWS. GH supports linear growth and increased muscle mass, and may lessen food preoccupation and weight gain.<ref>{{cite journal |vauthors=Davies PS, Evans S, Broomhead S, Clough H, Day JM, Laidlaw A, Barnes ND | title = Effect of growth hormone on height, weight, and body composition in Prader-Willi syndrome | journal = Arch. Dis. Child. | volume = 78 | issue = 5 | pages = 474–6 | date = May 1998 | pmid = 9659098 | pmc = 1717576 | doi = 10.1136/adc.78.5.474 }}</ref><ref>{{cite journal |vauthors=Carrel AL, Myers SE, Whitman BY, Allen DB | title = Benefits of long-term GH therapy in Prader-Willi syndrome: a 4-year study | journal = J. Clin. Endocrinol. Metab. | volume = 87 | issue = 4 | pages = 1581–5 | date = April 2002 | pmid = 11932286 | doi = 10.1210/jcem.87.4.8414 | doi-access = free }}</ref><ref>{{cite journal |vauthors=Höybye C, Hilding A, Jacobsson H, Thorén M | title = Growth hormone treatment improves body composition in adults with Prader-Willi syndrome | journal = Clin. Endocrinol. | volume = 58 | issue = 5 | pages = 653–61 | date = May 2003 | pmid = 12699450 | doi = 10.1046/j.1365-2265.2003.01769.x | s2cid = 6941937 }}</ref> Because of severe obesity, obstructive [[sleep apnea]] is a common [[sequelae|sequela]], and a [[positive airway pressure]] machine is often needed. A person who has been diagnosed with PWS may have to undergo surgical procedures. One surgery that has proven to be unsuccessful for treating obesity is the [[gastric bypass]] procedure.<ref>{{cite journal |last1=Scheimann |first1=AO |last2=Butler |first2=MG |last3=Gourash |first3=L |last4=Cuffari |first4=C |last5=Klish |first5=W |title=Critical analysis of bariatric procedures in Prader-Willi syndrome. |journal=Journal of Pediatric Gastroenterology and Nutrition |date=January 2008 |volume=46 |issue=1 |pages=80–3 |doi=10.1097/01.mpg.0000304458.30294.31 |pmid=18162838|pmc=6815229 }}</ref> Behavior and psychiatric problems should be detected early for the best results. These issues are best treated with parental education and training. Sometimes medication is introduced as well. [[Serotonin]] agonists have been most effective in lessening temper tantrums and improving compulsivity.<ref name=Cas2008 />
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