The Journal of Pediatrics
Volume 149, Issue 2 , Pages 199-204 , August 2006

Ghrelin levels in young children with Prader-Willi syndrome

Received 1 April 2005 ,Revised 11 January 2006 ,Accepted 13 April 2006.

References 

  1. Cassidy SB . Prader-Willi Syndrome . J Med Genet . 1997;34:917–923
  2. Holm VA , Cassidy SB , Butler MG , Hanchett JM , Greenswag LR , Whitman BY , et al.   Prader-Willi syndrome (consensus diagnostic criteria) . Pediatrics . 1993;91:398–402
  3. Cassidy SB . Prader-Willi syndrome . In:  Cassidy SB ,  Allanson JE editor. Management of genetic syndromes . 2nd ed.. New York: Wiley; 2005;p. 429–448
  4. Coplin SS , Hine J , Gormican A . Out-patient dietary management in the Prader-Willi syndrome . J Am Diet Assoc . 1976;68:330–334
  5. Butler JV , Whittington JE , Holland AJ , Boer H , Clarke D , Webb T . Prevalence of, and risk factors for, physical ill-health in people with Prader-Willi syndrome (a population-based study) . Dev Med Child Neurol . 2002;44:248–255
  6. Kojima M , Hosoda H , Date Y , Nakazato M , Matsuo H , Kangawa K . Ghrelin is a growth-hormone-releasing acylated peptide from stomach . Nature . 1999;402:656–660
  7. Tschöp M , Smiley DL , Heiman ML . Ghrelin induces adiposity in rodents . Nature . 2000;407:908–913
  8. Nakazato M , Murakami N , Date Y , Kojima M , Matsuo H , Kangawa K , et al.   A role for ghrelin in the central regulation of feeding . Nature . 2001;409:194–198
  9. Wren AM , Small CJ , Ward HL , Murphy KG , Dakin CL , Taheri S , et al.   The novel hypothalamic peptide ghrelin stimulates food intake and growth hormone secretion . Endocrinology . 2000;141:4325–4328
  10. Wren AM , Seal LJ , Cohen MA , Brynes AE , Frost GS , Murphy KG , et al.   Ghrelin enhances appetite and increases food intake in humans . J Clin Endocrinol Metab . 2001;86:5992–5995
  11. Neary NM , Small CJ , Wren AM , Lee JL , Druce MR , Palmieri C , et al.   Ghrelin increases energy intake in cancer patients with impaired appetite (acute, randomized, placebo-controlled trial) . J Clin Endocrinol Metab . 2004;89:2832–2836
  12. Asakawa A , Inui A , Kaga T , Yuzuriha H , Nagata T , Ueno N , et al.   Ghrelin is an appetite-stimulatory signal from stomach with structural resemblance to motilin . Gastroenterology . 2001;120:337–345
  13. Wren AM , Small CJ , Abbott CR , Dhillo WS , Seal LH , Cohen MA , et al.   Ghrelin causes hyperphagia and obesity in rats . Diabetes . 2001;50:2540–2547
  14. Xie QF , Wu CX , Meng QY , Li N . Ghrelin and truncated ghrelin variant plasmid vectors administration into skeletal muscle augments long-term growth in rats . Domest Anim Endocrinol . 2004;27:155–164
  15. Murakami N , Hayashida T , Kuroiwa T , Nakahara K , Ida T , Mondal MS , et al.   Role for central ghrelin in food intake and secretion profile of stomach ghrelin in rats . J Endocrinol . 2002;174:283–288
  16. Bagnasco M , Tulipano G , Melis MR , Argiolas A , Cocchi D , Muller EE . Endogenous ghrelin is an orexigenic peptide acting in the arcuate nucleus in response to fasting . Regul Pept . 2003;111:161–167
  17. Shuto Y , Shibasaki T , Otagiri A , Kuriyama H , Ohata H , Tamura H , et al.   Hypothalamic growth hormone secretagogue receptor regulates growth hormone secretion, feeding, and adiposity . J Clin Invest . 2002;109:1429–1436
  18. Asakawa A , Inui A , Kaga T , Katsuura G , Fujimiya M , Fujino MA , et al.   Antagonism of ghrelin receptor reduces food intake and body weight gain in mice . Gut . 2003;52:947–952
  19. Shearman LP , Wang SP , Helmling S , Stribling DS , Mazur P , Ge L , et al.   Ghrelin neutralization by an RNA-spiegelmer ameliorates obesity in diet-induced obese mice . Endocrinology . 2006;147:1517–1526
  20. Cummings DE , Purnell JQ , Frayo RS , Schmidova K , Wisse BE , Weigle DS . A preprandial rise in plasma ghrelin levels suggests a role in meal initiation in humans . Diabetes . 2001;50:1714–1719
  21. Cummings DE , Weigle DS , Frayo RS , Breen PA , Ma MK , Dellinger EP , et al.   Plasma ghrelin levels after diet-induced weight loss or gastric bypass surgery . N Engl J Med . 2002;346:1623–1630
  22. Cummings DE , Foster-Schubert KE , Overduin J . Ghrelin and energy balance (focus on current controversies) . Curr Drug Targets . 2005;6:153–169
  23. van der Lely AJ , Tschöp M , Heiman ML , Ghigo E . Biological, physiological, pathophysiological, and pharmacological aspects of ghrelin . Endocr Rev . 2004;25:426–457
  24. Barreiro ML , Tena-Sempere M . Ghrelin and reproduction (a novel signal linking energy status and fertility?) . Mol Cell Endocrinol . 2004;226:1–9
  25. Cummings DE , Clement K , Purnell JQ , Vaisse C , Foster KE , Frayo RS , et al.   Elevated plasma ghrelin levels in Prader Willi syndrome . Nat Med . 2002;8:643–644
  26. Tschöp M , Weyer C , Tataranni PA , Devanarayan V , Ravussin E , Heiman ML . Circulating ghrelin levels are decreased in human obesity . Diabetes . 2001;50:707–709
  27. DelParigi A , Tschop M , Heiman ML , Salbe AD , Vozarova B , Sell SM , et al.   High circulating ghrelin (a potential cause for hyperphagia and obesity in Prader-Willi syndrome) . J Clin Endocrinol Metab . 2002;87:5461–5464
  28. Goldstone AP , Thomas EL , Brynes AE , Castroman G , Edwards R , Ghatei MA , et al.   Elevated fasting plasma ghrelin in Prader-Willi syndrome adults is not solely explained by their reduced visceral adiposity and insulin resistance . J Clin Endocrinol Metab . 2004;89:1718–1726
  29. Hoybye C . Endocrine and metabolic aspects of adult Prader-Willi syndrome with special emphasis on the effect of growth hormone treatment . Growth Horm IGF Res . 2004;14:1–15
  30. Tauber M , Conte Auriol F , Moulin P , Molinas C , Delagnes V , et al.   Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption (a pathophysiological hypothesis) . Horm Res . 2004;62:49–54
  31. Cummings DE , Frayo RS , Marmonier C , Aubert R , Chapelot D . Plasma ghrelin levels and hunger scores in humans initiating meals voluntarily without time- and food-related cues . Am J Physiol Endocrinol Metab . 2004;287:E297–E304
  32. Haqq AM , Farooqi IS , O’Rahilly S , Stadler DD , Rosenfeld RG , Pratt KL , et al.   Serum ghrelin levels are inversely correlated with body mass index, age, and insulin concentrations in normal children and are markedly increased in Prader-Willi syndrome . J Clin Endocrinol Metab . 2003;88:174–178
  33. Paik KH , Jin DK , Song SY , Lee JE , Ko SH , Song SM , et al.   Correlation between fasting plasma ghrelin levels and age, body mass index (BMI), BMI percentiles, and 24-hour plasma ghrelin profiles in Prader-Willi syndrome . J Clin Endocrinol Metab . 2004;89:3885–3889
  34. Butler MG , Bittel DC , Talebizaceh Z . Plasma peptide YY and ghrelin levels in infants and children with Prader-Willi syndrome . J Clin Endocrinol Metab . 2004;17:1177–1184
  35. Groschl M , Uhr M , Kraus T . Evaluation of the comparability of commercial ghrelin assays . Clin Chem . 2004;50:457–458
  36. Soriano-Guillen L , Barrios V , Chowen JA , Sanchez I , Vila S , Quero J , et al.   Ghrelin levels from fetal life through early adulthood (relationship with endocrine and metabolic and anthropometric measures) . J Pediatr . 2004;144:30–35
  37. Barkan AL , Dimaraki EV , Jessup SK , Symons KV , Ermolenko M , Jaffe CA . Ghrelin secretion in humans is sexually dimorphic, suppressed by somatostatin, and not affected by the ambient growth hormone levels . J Clin Endocrinol Metab . 2003;88:2180–2184
  38. Foster-Schubert KE , McTiernan A , Frayo RS , Schwartz RS , Tworoger SS , Cummings DE . Human plasma ghrelin levels in crease during a one-year exercise program . J Clin Endocrinol Metab . 2005;90:820–825
  39. Morgan CR , Lazarow A . Immunoassay of insulin (two-antibody systems) . Diabetes . 1963;12:115–126
  40. Janssen JA , van der Toorn FM , Hofland LJ , van Koetsveld P , Broglio F , Ghigo E , et al.   Systemic ghrelin levels in subjects with growth hormone deficiency are not modified by one year of growth hormone replacement therapy . Eur J Endocrinol . 2001;145:711–716
  41. Malik IA , English PJ , Ghatei MA , Bloom SR , MacFarlane IA , Wilding JP . The relationship of ghrelin to biochemical and anthropometric markers of adult growth hormone deficiency . Clin Endocrinol . 2004;60:137–141
  42. Hoybye C , Barkeling B , Espelund U , Petersson M , Thoren M . Peptides associated with hyperphagia in adults with Prader-Willi syndrome before and during GH treatment . Growth Horm IGF Res . 2003;13:322–327
  43. Ariyasu H , Takaya K , Hosoda H , Iwakura H , Ebihara K , Mori K , et al.   Delayed short-term secretory regulation of ghrelin in obese animals (evidenced by a specific RIA for the active form of ghrelin) . Endocrinology . 2002;143:3341–3350
  44. Nakai Y , Hosoda H , Nin K , Ooya C , Hayashi H , Akamizu T , et al.   Plasma levels of active form of ghrelin during oral glucose tolerance test in patients with anorexia nervosa . Eur J Endocrinol . 2003;149:R1–R3
  45. Kennedy L , Bittel DC , Kibiryeva N , Kalra SP , Torto R , Butler MG . Circulating adiponectin levels, body composition, and obesity-related variable in Prader-Willi syndrome (comparison with obese subjects) . Int J Obes . 2006;30:382–387
  46. Marzullo P , Marcassa C , Campini R , Eleuteri E , Minocci A , Priano L , et al.   The impact of growth hormone/insulin-like growth factor-I axis and nocturnal breathing disorders on cardiovascular features of adult patients with Prader-Willi syndrome . J Clin Endo Metab . 2005;90:5639–5646
  47. Talebizadeh Z , Butler MG . Insulin resistance and obesity-related factors in Prader-Willi syndrome (comparison with obese subjects) . Clin Genet . 2005;67:230–239
  48. McLaughlin T , Abbasi F , Lamendola C , Frayo RS , Cummings DE . Plasma ghrelin concentrations are decreased in insulin-resistant obese adults relative to equally obese insulin-sensitive controls . J Clin Endocrinol Metab . 2004;89:1630–1635
  49. Roof E , Stone W , MacLean W , Feurer ID , Thompson T , Butler MG . Intellectual characteristics of Prader-Willi syndrome (comparison of genetic subtypes) . J Intellect Disabil Res . 2000;44:25–30
  50. Cassidy SB , Forsythe M , Heeger S , Nicholls RD , Schork N , Benn P , et al.   Comparison of phenotype between patients with Prader-Willi syndrome due to deletion 15q and uniparental disomy 15 . Am J Med Genet . 1997;68:433–440
  51. Dykens EM , Cassidy SB , King BH . Maladaptive behavior differences in Prader-Willi syndrome due to paternal deletion versus maternal uniparental disomy . AJMR . 1999;104:67–77
  52. Butler MG , Bittel DC , Kibiryeva N , Talebizadeh Z , Thompson T . Behavioral differences among subjects with Prader-Willi syndrome and type I and type II deletion and maternal disomy . Pediatrics . 2004;113;:565–573
  53. Mitchell J , Schinzel A , Langlois S , Gillessen-Kaesbach G , Schuffenhauer S , Michaelis R , et al.   Comparison of phenotype in uniparental disomy and deletion Prader-Willi syndrome (sex specific differences) . Am J Med Genet . 1996;65:133–136
  54. Stefan M , Ji H , Simmons RA , Cummings DE , Ahima RS , Friedman MI , et al.   Hormonal and metabolic defects in a Prader-Willi syndrome mouse model with neonatal failure to thrive . Endocrinology . 2005;146:4377–4385

 Supported by NIH grants RO1 DK61516 and PO1 DK68384 (to D.E.C.).

PII: S0022-3476(06)00306-4

doi: 10.1016/j.jpeds.2006.04.011

The Journal of Pediatrics
Volume 149, Issue 2 , Pages 199-204 , August 2006

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