Body fat excess and stimulated growth hormone levels in adult patients with prader-willi syndrome

American Journal of Medical Genetics, Part A

Volumn 149, Issue 4, 2009, Pages 726-731

  Grugni, Graziano ^a   Crinò, Antonino ^b   Bertocco, Paride ^a   Marzullo, Paolo ^a  

^a ISTITUTO AUXOLOGICO ITALIANO   (Italy)

^b BAMBINO GESÙ CHILDREN S HOSPITAL   (Italy)

Author keywords

Fat mass; GH; Obesity; Prader Willi syndrome

Indexed keywords

ARGININE; GROWTH HORMONE; GROWTH HORMONE RECEPTOR;

ADULT; AREA UNDER THE CURVE; ARTICLE; BODY FAT; BODY MASS; CLINICAL ARTICLE; DISEASE ASSOCIATION; DUAL ENERGY X RAY ABSORPTIOMETRY; ENZYME REGULATION; FEMALE; GENETIC ANALYSIS; GROWTH HORMONE BLOOD LEVEL; HUMAN; OBESITY; PRADER WILLI SYNDROME; PRIORITY JOURNAL;

ADIPOSE TISSUE; ADULT; ARGININE; BODY MASS INDEX; CASE-CONTROL STUDIES; FEMALE; GONADOTROPIN-RELEASING HORMONE; HUMAN GROWTH HORMONE; HUMANS; MALE; OBESITY; PRADER-WILLI SYNDROME; YOUNG ADULT;

EID: 63749102425     PISSN: 15524825     EISSN: 15524833     Source Type: Journal    
DOI: 10.1002/ajmg.a.32713     Document Type: Article

References (33)

1. Aimaretti G, Cornell G, Razzore P, Bellone S, Baffoni C, Arvat E, Camanni F, Ghigo E. 1998. Comparison between insulin-induced hypoglycemia and growth hormone (GH)-releasing hormone + arginine as provocative tests for the diagnosis of GH deficiency in adults. J Clin Endocrinol Metab 83:1615-1618.
2. Beccaria L, Benzi F, Sanzari A, Bosio L, Brambilla P, Chiumello G. 1996. Impairment of growth hormone responsiveness to growth hormone releasing hormone and pyridostigmine in patients affected by Prader-Labhardt-Willi syndrome. J Endocrinol Invest 19:687-692.
3. Bertella L, Mori I, Grugni G, Pignatti R, Ceriani F, Molinari E, Ceccarelli A, Sartorio A, Vettor R, Semenza C. 2007. Quality of life and psychological well-being in GH-treated, adult PWS patients: A longitudinal study. J Intellect Disabil Res 51:302-311.
4. Bittel DC, Butler MG. 2005. Prader-Willi syndrome. Clinical genetics, cytogenetics, and molecular biology. Expert Rev Mol Med 7:1-20.
5. Brambilla P, Bosio L, Manzoni P, Pietrobelli A, Beccaria L, Chiumello G. 1997. Peculiar body composition in patients with Prader-Labhart-Willi. Am J Clin Nutr 65:1369-1374.
6. Burman P, Ritzen EM, Lindgren AC. 2001. Endocrine dysfunction in Prader-Willi syndrome: A review with special reference to GH. Endocr Rev 22:787-799.
7. Butler MG, Hanchett JM, Thompson T. 2006. Clinical findings and natural history of Prader-Willi syndrome. In: Butler MG, Lee PDK, Whitman BY, editors. Management of Prader-Willi syndrome. 3rd edition. New York: Springer. pp 3-48.
8. Carrel AL, Lee PDK, Mogul HR. 2006. Growth hormone in Prader-Willi syndrome. In: Butler MG, Lee PDK, Whitman BY, editors. Management of Prader-Willi syndrome. 3rd edition. New York: Springer. pp 201-241.
9. Clemmons DR. 2007. Value of insulin-growth factor system markers in the assessment ofgrowth hormone status. Endocrinol Metab Clin North Am 36:109-129.
10. Corneli G, Di Somma C, Baldelli R, Rovere S, Gasco V, Croce CG, Grottoli S, Maccario M, Colao A, Lombardi G, Ghigo E, Camanni F, Aimaretti G. 2005. The cut-off limits of the GH response to GH-releasing hormonearginine test related to body mass index. Eur J Endocrinol 153:257-264.
11. Eiholzer U, Lee PDK. 2006. Medical considerations in Prader-Willi syndrome. In: Butler MG, Lee PDK, Whitman BY, editors. Management of Prader-Willi syndrome. 3rd edition. New York: Springer. pp 97-152.
12. Ellis KJ. 2000. Human body composition: In vivo methods. Physiol Rev 80:649-680.
13. Ellis KJ. 2003. Assessment ofbody composition in children with Prader-Willi syndrome or simple obesity. In: Eiholzer U, l'Allemand D, Zipf UB, editors. Prader-Willi syndrome as a model for obesity. Basel (Switzerland): Karger. pp 49-60.
14. Goldstone AP. 2004. Prader-Willi syndrome: Advances in genetics, patho-physiology, and treatment. Trends Endocrinol Metab 15:12-20.
15. Goldstone AP, Brynes AE, Thomas EL, Bell JD, Frost G, Holland A, Ghatei M, Bloom SR. 2002. Resting metabolic rate, plasma leptin concentrations, leptin receptor expression, and adipose tissue measured by whole-body magnetic resonance imaging in women with Prader-Willi syndrome. Am J Clin Nutr 75:468-475.
16. Grugni G, Guzzaloni G, Morabito F. 2001. Impairment of GH responsiveness to GH-releasing hexapeptide (GHRP-6) in Prader-Willi syndrome. J Endocrinol Invest 24:340-348.
17. Grugni G, Marzullo P, Ragusa L, Sartorio A, Trifirò G, Liuzzi A, Crinò A. 2006. Impairment of GH responsiveness to combined GH-releasing hormone and arginine administration in adult patients with Prader-Willi syndrome. Clin Endocrinol 65:492-499.
18. Gunnay-Aygun M, Heeger S, Schwartz S, Cassidy SB. 1997. Delayed diagnosis in patients with Prader-Willi syndrome due to maternal uniparental disomy. Am J Med Genet 71:106-110.
19. Hartman ML. 2000. Physiological regulators ofgrowth hormone secretion. In: Juul A, Jorgensen JOL, editors. Growth hormone in adults. 2nd edition. Cambridge: Cambridge University Press. pp 3-53.
20. Holm VA, Cassidy SB, Butler MG, Hanchett JM, Greenswag LR, Whitman BY, Greenberg F. 1993. Prader-Willi syndrome: Consensus diagnostic criteria. Pediatrics 91:398-402.
21. Hoybye C. 2007. Five-years growth hormone (GH) treatment in adults with Prader-Willi syndrome. Acta Paediatr 96:410-413.
22. Hoybye C, Hilding A, Jacobsson H, Thorén M. 2002. Metabolic profile and body composition in adults with Prader-Willi syndrome and severe obesity. J Clin Endocrinol Metab 87:3590-3597.
23. Kennedy L, Bittel DC, Kibiryeva N, Kalra SP, Torto R, Butler MG. 2006. Circulating adiponectin levels, body composition, and obesity-related variables in Prader-Willi syndrome: Comparison with obese subjects. Int J Obes 30:382-387.
24. Kokkoris P, Pi-Sunyer FX. 2003. Obesity and endocrine disease. Endocrinol Metab Clin North Am 32:895-914.
25. Lee PDK, Allen DB, Angulo MA, Cappa M, Carrel AL, Castro-Magana M, Chiumello G, Davies PSW, Eiholzer U, Grugni G, Hauffa BP, Hintz RL, Lammer C, Mogul HR, Myers SE, Partsch C-J, Pescovitz OH, Ritzen EM, Rosenfeld RG, Sipila I, Wilson DM. 2000. Consensus Statement-Prader-Willi syndrome: Growth hormone (GH)/insulin-like growth factor axis deficiency and GH treatment. Endocrinologist 10:71S-74S.
26. Marzullo P, Marcassa C, Campini R, Eleuteri E, Minocci A, Priano L, Temporelli P, Sartorio A, Vettor R, Liuzzi A, Grugni G. 2005. The impact of growth hormone/insulin-like growth factor-1 and nocturnal breathing disorders on cardiovascular features of adult patients with Prader-Willi syndrome. J Clin Endocrinol Metab 90:5639-5646.
27. Marzullo P, Marcassa C, Campini R, Eleuteri E, Minocci A, Sartorio A, Vettor R, Liuzzi A, Grugni G. 2007. Conditional cardiovascular response to GH therapy in adult patients with Prader-Willi syndrome. J Clin Endocrinol Metab 92:1364-1371.
28. Partsch C-J, Lammer C, Gillessen-Kaesbach G, Pankau R. 2000. Adult patients with Prader-Willi syndrome: Clinical characteristics, life circumstances, and growth hormone secretion. Growth Horm IGF Res 10:S81-S85.
29. Tanner JM, Whitehouse RH, Takaishi M. 1966. Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. Arch Dis Child 41:613-635.
30. Theodoro MF, Talebizadeh Z, Butler MG. 2006. Body composition and fatness patterns in Prader-Willi syndrome: Comparison with simple obesity. Obesity 14:1685-1690.
31. Veldhuis JD, Patrie JT, Brill KT, Weltman JY, Mueller EE, Bowers CY, Weltman A. 2004. Contributions of gender and systemic estradiol and testosterone concentrations to maximal secretagogue drive ofburst-like growth hormone secretion in healthy middle-aged and older adults. J Clin Endocrinol Metab 89:6291-6296.
32. Wesby-van Swaay E, van den Ouweland AMW, Halley DJJ, van der Est MN, Tijmensen TSLN, Niermeijer MF. 1995. Uniparental disomy in Prader-Willi syndrome: Implications for the phenotype. 2nd Prader-Willi Syndrome International Scientific Workshop and Conference, Somarka Oslo, p 45 (Abstract).
33. ZipfWB. 2004. Prader-Willi syndrome: The care and treatment of infants, children, and adults. Adv Pediatr 51:409-434.