Patients with Prader-Willi syndrome (PWS) have a shorter life expectancy than the general population, which is mainly due to complications of hyperphagia and obesity.[5]Proffitt J, Osann K, McManus B, et al. Contributing factors of mortality in Prader-Willi syndrome. Am J Med Genet A. 2019 Feb;179(2):196-205.
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[6]Butler MG, Manzardo AM, Heinemann J, et al. Causes of death in Prader-Willi syndrome: Prader-Willi Syndrome Association (USA) 40-year mortality survey. Genet Med. 2017 Jun;19(6):635-42.
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[7]Lionti T, Reid SM, Rowell MM. Prader-Willi syndrome in Victoria: mortality and causes of death. J Paediatr Child Health. 2012 Jun;48(6):506-11.
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[8]Grugni G, Crinò A, Bosio L, et al. The Italian National Survey for Prader-Willi syndrome: an epidemiologic study. Am J Med Genet A. 2008 Apr 1;146A(7):861-72.
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[9]Schrander-Stumpel CT, Curfs LM, Sastrowijoto P, et al. Prader-Willi syndrome: causes of death in an international series of 27 cases. Am J Med Genet A. 2004 Feb 1;124A(4):333-8.
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[10]Stevenson DA, Anaya TM, Clayton-Smith J, et al. Unexpected death and critical illness in Prader-Willi syndrome: report of ten individuals. Am J Med Genet A. 2004 Jan 15;124A(2):158-64.
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However, with effective weight control management, patients with PWS may live into their seventh decade.[2]McCandless SE, Committee on Genetics. Clinical report - health supervision for children with Prader-Willi syndrome. Pediatrics. 2011 Jan;127(1):195-204.
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Growth hormone treatment may also prevent obesity and high proportion of fat mass.[50]Deal CL, Tony M, Höybye C, et al. Growth Hormone Research Society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013 Jun;98(6):E1072-87.
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Early treatment alters the natural course of PWS, and results in significantly improved cognitive and physical outcomes as well as modifying the typical facial appearance.[24]Grootjen LN, Uyl NEM, van Beijsterveldt IALP, et al. Prenatal and neonatal characteristics of children with Prader-Willi syndrome. J Clin Med. 2022 Jan 28;11(3):679.
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[65]Ayet-Roger A, Joga-Elvira L, Caixàs A, et al. Cognitive and adaptive effects of early growth hormone treatment in Prader-Willi syndrome patients: a cohort study. J Clin Med. 2022 Mar 14;11(6):1592.
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[66]Dykens EM, Roof E, Hunt-Hawkins H. Cognitive and adaptive advantages of growth hormone treatment in children with Prader-Willi syndrome. J Child Psychol Psychiatry. 2017 Jan;58(1):64-74.
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Deaths in children with PWS are primarily due to respiratory illness.[9]Schrander-Stumpel CT, Curfs LM, Sastrowijoto P, et al. Prader-Willi syndrome: causes of death in an international series of 27 cases. Am J Med Genet A. 2004 Feb 1;124A(4):333-8.
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[59]Eiholzer U. Deaths in children with Prader-Willi syndrome. A contribution to the debate about the safety of growth hormone treatment in children with PWS. Horm Res. 2005;63(1):33-9.
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[60]Tauber M, Diene G, Molinas C, et al. Review of 64 cases of death in children with Prader-Willi syndrome (PWS). Am J Med Genet A. 2008 Apr 1;146A(7):881-7.
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