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Growth Hormone Therapy in Adults with Prader-Willi Syndrome
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 Title & Authors
Growth Hormone Therapy in Adults with Prader-Willi Syndrome
Cho, Sung Yoon;
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 Abstract
Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. Many features of PWS indicate a deficiency in growth hormone (GH) production, and these findings provide a rationale for GH therapy in PWS. It is possible that rhGH therapy could have beneficial effects in adults with PWS, similar to those in adults with GH deficiency (GHD) of non-syndromic cause. However, there is a paucity of data on the use of GH in adults with PWS. Here, the previous studies about efficacy and safety of rhGH therapy in PWS adults are summarized. Briefly, rhGH therapy in PWS adults may improve body composition, leading to increased lean body mass and decreased fat mass, as well as decreased subcutaneous and visceral adiposity without overall changes in body mass index. There may be at least transient deterioration in glucose homoeostasis in some PWS patients on rhGH therapy, which requires further study. In addition, clinical care guidelines for rhGH therapy in adults with PWS were suggested.
 Keywords
Prader-Willi syndrome;Growth hormone deficiency;Growth hormone therapy;
 Language
English
 Cited by
 References
1.
Smith A, Egan J, Ridley G, Haan E, Montgomery P, Williams K, et al. Birth prevalence of Prader-Willi syndrome in Australia. Arch Dis Child 2003;88:263-4. crossref(new window)

2.
Vogels A, Van Den Ende J, Keymolen K, Mortier G, Devriendt K, Legius E, et al. Minimum prevalence, birth incidence and cause of death for Prader-Willi syndrome in Flanders. Eur J Hum Genet 2004;12:238-40. crossref(new window)

3.
Cassidy SB, Dykens E, Williams CA. Prader-Willi and Angelman syndromes: sister imprinted disorders. Am J Med Genet 2000;97:136-46. crossref(new window)

4.
Eiholzer U, Bachmann S, l'Allemand D. Is there growth hormone deficiency in prader-willi Syndrome? Six arguments to support the presence of hypothalamic growth hormone deficiency in Prader-Willi syndrome. Horm Res 2000;53 Suppl 3:44-52. crossref(new window)

5.
Burman P, Ritzen EM, Lindgren AC. Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH. Endocr Rev 2001;22:787-99. crossref(new window)

6.
Ledermann SE, Scanes ME, Fernando ON, Duffy PG, Madden SJ, Trompeter RS. Long-term outcome of peritoneal dialysis in infants. J Pediatr 2000;136:24-9. crossref(new window)

7.
Haqq AM, Stadler DD, Jackson RH, Rosenfeld RG, Purnell JQ, LaFranchi SH. Effects of growth hormone on pulmonary function, sleep quality, behavior, cognition, growth velocity, body composition, and resting energy expenditure in Prader-Willi syndrome. J Clin Endocrinol Metab 2003;88:2206-12. crossref(new window)

8.
Eiholzer U, Gisin R, Weinmann C, Kriemler S, Steinert H, Torresani T, et al. Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance. Eur J Pediatr 1998;157:368-77. crossref(new window)

9.
Carrel AL, Myers SE, Whitman BY, Allen DB. Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: A controlled study. J Pediatr 1999;134:215-21. crossref(new window)

10.
Carrel AL, Moerchen V, Myers SE, Bekx MT, Whitman BY, Allen DB. Growth hormone improves mobility and body composition in infants and toddlers with Prader-Willi syndrome. J Pediatr 2004;145:744-9. crossref(new window)

11.
Lindgren AC, Lindberg A. Growth hormone treatment completely normalizes adult height and improves body composition in Prader-Willi syndrome: experience from KIGS (Pfizer International Growth Database). Horm Res 2008;70:182-7. crossref(new window)

12.
Jin DK. Endocrine problems in children with Prader-Willi syndrome: special review on associated genetic aspects and early growth hormone treatment. Korean J Pediatr 2012;55:224-31. crossref(new window)

13.
Miller J, Silverstein J, Shuster J, Driscoll DJ, Wagner M. Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome. J Clin Endocrinol Metab 2006;91:413-7. crossref(new window)

14.
Nixon GM, Brouillette RT. Sleep and breathing in Prader- Willi syndrome. Pediatr Pulmonol 2002;34:209-17. crossref(new window)

15.
Sanchez-Ortiga R, Klibanski A, Tritos NA. Effects of recombinant human growth hormone therapy in adults with Prader-Willi syndrome: a meta-analysis. Clin Endocrinol (Oxf) 2012;77:86-93. crossref(new window)

16.
Hoybye C. Five-years growth hormone (GH) treatment in adults with Prader-Willi syndrome. Acta Paediatr 2007;96:410-3. crossref(new window)

17.
Sode-Carlsen R, Farholt S, Rabben KF, Bollerslev J, Schreiner T, Jurik AG, et al. One year of growth hormone treatment in adults with Prader-Willi syndrome improves body composition: results from a randomized, placebo-controlled study. J Clin Endocrinol Metab 2010;95:4943-50. crossref(new window)

18.
Sode-Carlsen R, Farholt S, Rabben KF, Bollerslev J, Schreiner T, Jurik AG, et al. Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome. Growth Horm IGF Res 2011;21:185-90. crossref(new window)

19.
Hoybye C, Hilding A, Jacobsson H, Thoren M. Growth hormone treatment improves body composition in adults with Prader-Willi syndrome. Clin Endocrinol (Oxf) 2003;58:653-61. crossref(new window)

20.
Mogul HR, Lee PD, Whitman BY, Zipf WB, Frey M, Myers S, et al. Growth hormone treatment of adults with Prader-Willi syndrome and growth hormone deficiency improves lean body mass, fractional body fat, and serum triiodothyronine without glucose impairment: results from the United States multicenter trial. J Clin Endocrinol Metab 2008;93:1238-45. crossref(new window)

21.
Gondoni LA, Vismara L, Marzullo P, Vettor R, Liuzzi A, Grugni G. Growth hormone therapy improves exercise capacity in adult patients with Prader-Willi syndrome. J Endocrinol Invest 2008;31:765-72. crossref(new window)

22.
Hoybye C, Thoren M, Bohm B. Cognitive, emotional, physical and social effects of growth hormone treatment in adults with Prader-Willi syndrome. J Intellect Disabil Res 2005;49:245-52. crossref(new window)

23.
Bertella L, Mori I, Grugni G, Pignatti R, Ceriani F, Molinari E, et al. Quality of life and psychological well-being in GHtreated, adult PWS patients: a longitudinal study. J Intellect Disabil Res 2007;51:302-11. crossref(new window)

24.
Marzullo P, Marcassa C, Campini R, Eleuteri E, Minocci A, Sartorio A, et al. Conditional cardiovascular response to growth hormone therapy in adult patients with Prader-Willi syndrome. J Clin Endocrinol Metab 2007;92:1364-71. crossref(new window)

25.
Tauber M, Diene G, Molinas C, Hebert M. Review of 64 cases of death in children with Prader-Willi syndrome (PWS). Am J Med Genet A 2008;146A:881-7. crossref(new window)

26.
Gerard JM, Garibaldi L, Myers SE, Aceto T, Jr., Kotagal S, Gibbons VP, et al. Sleep apnea in patients receiving growth hormone. Clin Pediatr (Phila) 1997;36:321-6. crossref(new window)

27.
Nixon GM, Rodda CP, Davey MJ. Longitudinal association between growth hormone therapy and obstructive sleep apnea in a child with Prader-Willi syndrome. J Clin Endocrinol Metab 2011;96:29-33. crossref(new window)