Hypothalamic obesity in children

Obesity Reviews

Volumn 13, Issue 9, 2012, Pages 780-798

  Bereket, A ^a   Kiess, W ^b   Lustig, R H ^c   Muller, H L ^d   Goldstone, A P ^e   Weiss, R ^f   Yavuz, Y ^g   Hochberg, Z ^h  

^a MARMARA UNIVERSITY   (Turkey)

^b UNIVERSITY OF LEIPZIG   (Germany)

^c UNIVERSITY OF CALIFORNIA   (United States)

^d KLINIKUM OLDENBURG   (Germany)

^e HAMMERSMITH HOSPITAL   (United Kingdom)

^f HEBREW UNIVERSITY   (Israel)

^g ISTANBUL BILIM UNIVERSITY   (Turkey)

^h TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

Author keywords

Craniopharyngioma; Hypothalamic; Obesity; Prader Willi syndrome

Indexed keywords

ADIPONECTIN; GHRELIN; INSULIN; LEPTIN; METHYLPHENIDATE; MODAFINIL; OCTREOTIDE; SIRTUIN; VASPIN;

ARTICLE; BARIATRIC SURGERY; BILIOPANCREATIC BYPASS; BRAIN METABOLISM; CANCER SURGERY; CHILDHOOD CANCER; CRANIOPHARYNGIOMA; ENDOCRINE DISEASE; ENERGY METABOLISM; GASTRIC BANDING; HUMAN; HYPOTHALAMIC OBESITY; HYPOTHALAMUS DISEASE; INSULIN RELEASE; LEPTIN DEFICIENCY; NEUROPATHOLOGY; OBESITY; PATIENT CARE; POSTOPERATIVE COMPLICATION; PRADER WILLI SYNDROME; SIGNAL TRANSDUCTION; SLEEVE GASTRECTOMY; STARVATION; STOMACH BYPASS; WEIGHT REDUCTION;

AUTONOMIC NERVOUS SYSTEM; CHILD; CONGRESSES AS TOPIC; CRANIOPHARYNGIOMA; ENERGY METABOLISM; HUMANS; HYPOTHALAMIC DISEASES; HYPOTHALAMIC NEOPLASMS; OBESITY; PITUITARY NEOPLASMS; PRADER-WILLI SYNDROME; WEIGHT GAIN;

EID: 84865556763     PISSN: 14677881     EISSN: 1467789X     Source Type: Journal    
DOI: 10.1111/j.1467-789X.2012.01004.x     Document Type: Article

References (181)

1. Babinski J. Tumeur du corps pituitaire sans acromégalie et avec arrêt de développement des organes génitaux. Rev Neurol 1900; 8: 531-533.
2. Frohlich A. Ein fall von tumor der hypophysis cerebri ohne akromegalie. Wiener Klin Rdsch 1901; 15: 883-886.
3. Bruch H. The Frohlich syndrome: report of the original case. Am J Dis Child 1939; 58: 1281-1289.
4. Smith P. The disabilities caused by hypophysectomy and their repair. The tuberal (hypothalamic) syndrome in the rat. JAMA 1927; 88: 159-161.
5. Hetherington A, Ranson S. Hypothalamic lesions and adiposity in the rat. Anat Rec 1940; 78: 149-172.
6. Han PW, Frohman LA. Hyperinsulinemia in tube-fed hypophysectomized rats bearing hypothalamic lesions. Am J Physiol 1970; 219: 1632-1636.
7. Frohman LA, Bernardis LL, Schnatz JD, Burek L. Plasma insulin and triglyceride levels after hypothalamic lesions in weanling rats. Am J Physiol 1969; 216: 1496-1501.
8. Bray GA, York DA. Hypothalamic and genetic obesity in experimental animals: an autonomic and endocrine hypothesis. Physiol Rev 1979; 59: 719-809.
9. King BM. The rise, fall, and resurrection of the ventromedial hypothalamus in the regulation of feeding behavior and body weight. Physiol Behav 2006; 87: 221-244.
10. Zhang Y, Proenca R, Maffei M, Barone M, Leopold L, Friedman JM. Positional cloning of the mouse obese gene and its human homologue. Nature 1994; 372: 425-432.
11. Hochberg I, Hochberg Z. Expanding the definition of hypothalamic obesity. Obes Rev 2010; 11: 709-721.
12. Hochberg I, Hochberg Z. Hypothalamic obesity. Endocr Dev 2010; 17: 185-196.
13. Bray GA, Gallagher TF, Jr. Manifestations of hypothalamic obesity in man: a comprehensive investigation of eight patients and a review of the literature. Medicine (Baltimore) 1975; 54: 301-330.
14. Swaab DF, Gooren LJ, Hofman MA. The human hypothalamus in relation to gender and sexual orientation. Prog Brain Res 1992; 93: 205-217.
15. Kreier F, Fliers E, Voshol PJ etal. Selective parasympathetic innervation of subcutaneous and intra-abdominal fat - functional implications. J Clin Invest 2002; 110: 1243-1250.
16. Coll AP, Farooqi IS, Challis BG, Yeo GS, O'Rahilly S. Proopiomelanocortin and energy balance: insights from human and murine genetics. J Clin Endocrinol Metab 2004; 89: 2557-2562.
17. Snow A, Gozal E, Malhotra A etal. Severe hypersomnolence after pituitary/hypothalamic surgery in adolescents: clinical characteristics and potential mechanisms. Pediatrics 2002; 110: e74.
18. Clement K, Vaisse C, Lahlou N etal. A mutation in the human leptin receptor gene causes obesity and pituitary dysfunction. Nature 1998; 392: 398-401.
19. Zimmermann U, Kraus T, Himmerich H, Schuld A, Pollmacher T. Epidemiology, implications and mechanisms underlying drug-induced weight gain in psychiatric patients. J Psychiatr Res 2003; 37: 193-220.
20. Tong Q, Ye CP, Jones JE, Elmquist JK, Lowell BB. Synaptic release of GABA by AgRP neurons is required for normal regulation of energy balance. Nat Neurosci 2008; 11: 998-1000.
21. Abizaid A, Liu ZW, Andrews ZB etal. Ghrelin modulates the activity and synaptic input organization of midbrain dopamine neurons while promoting appetite. J Clin Invest 2006; 116: 3229-3239.
22. Heisler LK, Jobst EE, Sutton GM etal. Serotonin reciprocally regulates melanocortin neurons to modulate food intake. Neuron 2006; 51: 239-249.
23. Masaki T, Chiba S, Yasuda T etal. Involvement of hypothalamic histamine H1 receptor in the regulation of feeding rhythm and obesity. Diabetes 2004; 53: 2250-2260.
24. Kim SF, Huang AS, Snowman AM, Teuscher C, Snyder SH. From the cover: antipsychotic drug-induced weight gain mediated by histamine H1 receptor-linked activation of hypothalamic AMP-kinase. Proc Natl Acad Sci U S A 2007; 104: 3456-3459.
25. Tung YC, Yeo GS. From GWAS to biology: lessons from FTO. Ann N Y Acad Sci 2011; 1220: 162-171.
26. Moraes JC, Coope A, Morari J etal. High-fat diet induces apoptosis of hypothalamic neurons. PLoS ONE 2009; 4: e5045.
27. Körner A, Kratzsch J, Kiess W. Adipocytokines: leptin - the classical, resistin - the controversical, adiponectin - the promising, and more to come. Best Pract Res Clin Endocrinol Metab 2005; 19: 525-546.
28. Körner A, Kratzsch J, Gausche R, Schaab M, Erbs S, Kiess W. New predictors of the metabolic syndrome in children - role of adipocytokines. Pediatr Res 2007; 61: 640-645.
29. Friebe D, Neef M, Erbs S etal. Re tinol binding protein 4 (RBP4) is primarily associated with adipose tissue mass in children. Int J Pediatr Obes 2011; 6: e345-e352. Epub 2010 Oct 15.
30. Friebe D, Neef M, Kratzsch J etal. Leucocytes are a major source of circulating nicotinamide phosphoribosyltransferase (NAMPT)/pre-B cell colony (PBEF)/visfatin linking obesity and inflammation in humans. Diabetologia 2011; 54: 1200-1211.
31. Körner A, Neef M, Friebe D etal. Vaspin is related to gender, puberty and deteriorating insulin sensitivity in children. Int J Obes (Lond) 2011; 35: 578-586.
32. Landgraf K, Friebe D, Ullrich T etal. Chemerin as a mediator between obesity and vascular inflammation in children. J Clin Endocrinol Metab 2012; 97: E556-E564.
33. Joo EY, Hong SB, Sohn YB etal. Plasma adiponectin level and sleep structures in children with Prader-Willi syndrome. J Sleep Res 2010; 19: 248-254.
34. Guran T, Turan S, Bereket A etal. The role of leptin, soluble leptin receptor, resistin, and insulin secretory dynamics in the pathogenesis of hypothalamic obesity in children. Eur J Pediatr 2009; 168: 1043-1048.
35. Feigerlova E, Diene G, Conte-Auriol F etal. Hyperghrelinemia precedes obesity in Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 2800-2805.
36. Farooqi IS, Jebb SA, Langmack G etal. Effects of recombinant leptin therapy in a child with congenital leptin deficiency. N Engl J Med 1999; 341: 913-915.
37. Farooqi IS, O'Rahilly S. Genetics of obesity in humans. Endocr Rev 2006; 27: 710-718.
38. Farooqi S. Obesity genes-its all about parents. Cell Metab 2009; 9: 487-488.
39. Lustig RH. Childhood obesity: behavioral aberration or biochemical drive? Reinterpreting the First Law of Thermodynamics. Nat Clin Pract Endol Metab 2006; 2: 447-458.
40. Heymsfield SB, Greenberg AS, Fujioka K etal. Recombinant leptin for weight loss in obese and lean adults: a randomized, controlled, dose-escalation trial. JAMA 1999; 282: 1568-1575.
41. Thaler JP, Yi CX, Schur EA etal. Obesity is associated with hypothalamic injury in rodents and humans. J Clin Invest 2012; 122: 153-162.
42. Lustig RH, Sen S, Soberman JE, Velasquez-Mieyer PA. Obesity, leptin resistance, and the effects of insulin suppression. Int J Obes 2004; 28: 1344-1348.
43. Leibel RL, Rosenbaum M, Hirsch J. Changes in energy expenditure resulting from altered body weight. N Engl J Med 1995; 332: 621-628.
44. Lustig RH. The 'skinny' on childhood obesity: how our Western environment starves kids' brains. Pediatr Ann 2006; 35: 898-907.
45. Schofl C, Schleth A, Berger D, Terkamp C, Von Zur Muhlen A, Brabant G. Sympathoadrenal counterregulation in patients with hypothalamic craniopharyngioma. J Clin Endocrinol Metab 2002; 87: 624-629.
46. Shaikh MG, Grundy RG, Kirk JM. Reductions in basal metabolic rate and physical activity contribute to hypothalamic obesity. J Clin Endocrinol Metab 2008; 93: 2588-2593.
47. Leibel RL. The role of leptin in the control of body weight. Nutr Rev 2002; 60: S15-SS9.
48. Keim NL, Stern JS, Havel PJ. Relation between circulating leptin concentrations and appetite during a prolonged, moderate energy deficit in women. Am J Clin Nutr 1998; 68: 794-801.
49. Rosenbaum M, Goldsmith R, Bloomfield D etal. Low-dose leptin reverses skeletal muscle, autonomic, and neuroendocrine adaptations to maintenance of reduced weight. J Clin Invest 2005; 115: 3579-3586.
50. Rosenbaum M, Murphy EM, Heymsfield SB, Matthews DE, Leibel RL. Low dose leptin administration reverses effects of sustained weight reduction on energy expenditure and circulating concentrations of thyroid hormones. J Clin Endocrinol Metabol 2002; 87: 2391-2394.
51. Lustig RH, Rose SR, Burghen GA etal. Hypothalamic obesity in children caused by cranial insult: altered glucose and insulin dynamics, and reversal by a somatostatin agonist. J Pediatr 1999; 135: 162-168.
52. Lustig RH, Hinds PS, Ringwald-Smith K etal. Octreotide therapy of pediatric hypothalamic obesity: a double-blind, placebo-controlled trial. J Clin Endocrinol Metabol 2003; 88: 2586-2592.
53. Velasquez-Mieyer PA, Cowan PA, Buffington CK etal. Suppression of insulin secretion promotes weight loss and alters macronutrient preference in a subset of obese adults. Int J Obes 2003; 27: 219-226.
54. Lustig RH, Greenway F, Velasquez-Mieyer P etal. A multicenter, randomized, double-blind, placebo-controlled, dose-finding trial of a long-acting formulation of octreotide in promoting weight loss in obese adults with insulin hypersecretion. Int J Obes 2006; 30: 331-341.
55. Kellerer M, Lammers R, Fritsche A etal. Insulin inhibits leptin receptor signalling in HEK293 cells at the level of janus kinase-2: a potential mechanism for hyperinsulinaemia-associated leptin resistance. Diabetologia 2001; 44: 1125-1132.
56. Niswender KD, Schwartz MW. Insulin and leptin revisited: adiposity signals with overlapping physiological and intracellular signaling capabilities. Front Neuroendocrinol 2003; 24: 1-10.
57. Hill JW, Williams KW, Ye C etal. Acute effects of leptin require PI3K signaling in hypothalamic proopiomelanocortin neurons in mice. J Clin Invest 2008; 118: 1796-1805.
58. Xu AW, Kaelin CB, Takeda K, Akira S, Schwartz MW, Barsh GS. PI3K integrates the action of insulin and leptin on hypothalamic neurons. J Clin Invest 2005; 115: 951-958.
59. Klöckener T, Hess S, Belgardt BF etal. High-fat feeding promotes obesity via insulin receptor/PI3K-dependent inhibition of SF-1 VMH neurons. Nat Neurosci 2011; 14: 911-918.
60. Müller HL. Childhood craniopharyngioma - current concepts in diagnosis, therapy and follow-up. Nat Rev Endocrinol 2010; 6: 609-618.
61. Rushing EJ, Giangaspero F, Paulus W, Burger PC. Craniopharyngioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavanee WK (eds). WHO Classification of Tumours of the Central Nervous System. IARC Press: Lyon, 2007, pp. 238-240.
62. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998; 89: 547-551.
63. Nielsen EH, Feldt-Rasmussen U, Poulsgaard L etal. Incidence of craniopharyngioma in Denmark (n=189) and estimated world incidence of craniopharyngioma in children and adults. J Neurooncol 2011; 104: 755-763.
64. Müller HL. Consequences after craniopharyngioma surgery in children. J Clin Endocrinol Metab 2011; 96: 1981-1991.
65. Müller HL. Childhood craniopharyngioma: current controversies on management in diagnostics, treatment and follow-up. Expert Rev Neurother 2010; 10: 515-524.
66. Müller HL. Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm Res 2008; 69: 193-202.
67. Müller HL, Emser A, Faldum A etal. Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma. J Clin Endocrinol Metab 2004; 89: 3298-3305.
68. Choux M, Lena G, Genitori L. Craniopharyngioma in children. Neurochirurgie 1991; 37 (Suppl. 1): 1-174.
69. Becker G, Kortmann RD, Skalej M, Bamberg M. The role of radiotherapy in the treatment of craniopharyngeoma - indications, results, side effects. In: Wiegel T, Hinkelbein T, Brock M, Hoell T (eds). Controversies in Neuro-Oncology. Front Radiat Ther Oncol. Karger: Basel, 1999, 33: pp. 100-113.
70. Einhaus SL, Sanford RA. Craniopharyngiomas. In: Albright AL, Pollack IF, Adelson PD (eds). Principles and Practice of Pediatric Neurosurgery. Thieme: New York, 1999, pp. 545-562.
71. Müller HL, Gebhardt U, Schröder S etal. Analyses of treatment variables for patients with childhood craniopharyngioma - results of the multicenter prospective study trial KRANIOPHARYNGEOM 2000 after three years of follow up. Horm Res Paediatr 2010; 73: 175-180.
72. Gupta N, Du R, Lustig RH, Fisch B, McDermott MW. Craniopharyngioma. In: Gupta N, Banerjee A, Haas-Kogan D (eds). Pediatric CNS Tumors, 1st edn. Chapter 7, Springer: Berlin, 2004, pp. 123-142.
73. Riva D, Pantaleoni C, Devoti M, Saletti V, Nichelli F, Giorgi C. Late neuropsychological and behavioural outcome of children surgically treated for craniopharyngeoma. Childs Nerv Syst 1998; 14: 179-184.
74. Pierre-Kahn A, Recassens C, Pinto G etal. Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series. Childs Nerv Syst 2005; 21: 817-824.
75. Müller HL, Bruhnken G, Emser A etal. Longitudinal study on quality of life in 102 survivors of childhood craniopharyngioma. Childs Nerv Syst 2005; 21: 975-980.
76. Müller HL, Gebhardt U, Faldum A etal. Functional capacity and body mass index in patients with sellar masses - cross-sectional study on 403 patients diagnosed during childhood and adolescence. Childs Nerv Syst 2005; 21: 539-545.
77. Fisher PG, Jenab J, Goldthwaite PT etal. Outcomes and failure patterns in childhood craniopharyngiomas. Childs Nerv Syst 1998; 14: 558-563.
78. DeVile CJ, Grant DB, Hayward RD, Stanhope R. Growth and endocrine sequelae of craniopharyngioma. Arch Dis Child 1996; 75: 108-114.
79. Merchant TE, Goloubeva O, Pritchard DL etal. Radiation dose-volume effects on growth hormone secretion. Int J Radiat Oncol Biol Phys 2002; 52: 1264-1270.
80. Honegger J, Buchfelder M, Fahlbusch R. Surgical resection of craniopharyngiomas: endocrinological results. J Neurosurg 1999; 90: 251-257.
81. Sanford RA. Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg 1994; 21 (Suppl. 1): 39-43.
82. Karavitaki N, Warner JT, Marland A etal. GH replacement does not increase the risk of recurrence in patients with craniopharyngioma. Clin Endocrinol (Oxf) 2006; 64: 556-560.
83. Müller HL, Bueb K, Bartels U etal. Obesity after childhood craniopharyngioma - German multicenter study on preoperative risk factors and quality of life. Klin Padiatr 2001; 213: 244-249.
84. Srinivasan S, Ogle GD, Garnett SP, Briody IN, Lee JW, Cowell CT. Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab 2004; 89: 81-86.
85. Müller HL, Schneider P, Etavard-Gorris N, Gebhardt U, Kolb R, Sörensen N. Volumetric bone mineral density (BMD) in patients with childhood craniopharyngioma. Exp Clin Endocrinol Diabetes 2003; 111: 168-173.
86. Müller HL, Heinrich M, Bueb K etal. Perioperative dexamethasone treatment in childhood craniopharyngioma - influence on short-term and long-term weight development. Exp Clin Endocrinol Diabetes 2003; 111: 330-334.
87. DeVile CJ, Grant DB, Kendall BE etal. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg 1996; 85: 73-81.
88. Roth C, Wilken B, Hanefeld F, Schröter W, Leonhardt U. Hyperphagia in children with craniopharyngioma is associated with hyperleptinemia and a failure in the down regulation of appetite. Eur J Endocrinol 1998; 138: 89-91.
89. Harz KJ, Müller HL, Waldeck E, Pudel V, Roth C. Obesity in patients with craniopharyngioma: assessment of physical activity and food intake. J Clin Endocrinol Metab 2003; 88: 5227-5231.
90. Müller HL, Handwerker G, Wollny B, Faldum A, Sörensen N. Melatonin secretion and increased sleepiness in childhood craniopharyngioma. J Clin Endocrinol Metab 2002; 87: 3993-3996.
91. Müller HL, Handwerker G, Gebhardt U etal. Melatonin treatment in obese patients with childhood craniopharyngioma and increased daytime sleepiness. Cancer Causes Control 2006; 17: 583-589.
92. Müller HL. Increased daytime sleepiness in patients with childhood craniopharyngioma and hypothalamic tumor involvement: review of the literature and perspectives. Int J Endocrinol 2010; 2010: 519607.
93. Müller HL, Müller-Stöver S, Gebhardt U, Kolb R, Sörensen N, Handwerker G. Secondary narcolepsy may be an underrated cause of increased daytime sleepiness in obese patients after childhood craniopharyngioma. J Pediatr Endocrinol Metab 2006; 19: 423-429.
94. Mason PW, Krawiecki N, Meacham LR. The use of dextroamphetamine to treat obesity and hyperphagia in children treated for craniopharyngioma. Arch Pediatr Adolesc Med 2002; 156: 887-892.
95. Roth C, Hunneman D, Gebhardt U, Stoffel-Wagner B, Reinehr T, Müller HL. Reduced sympathetic metabolites in urine of obese patients with craniopharyngioma. Pediatr Res 2006; 61: 496-501.
96. Roth C, Gebhardt U, Müller HL. Appetite-regulating hormone changes in patients with craniopharyngioma. Obesity (Silver Spring) 2011; 19: 36-41.
97. Roth CL, Enriori PJ, Gebhardt U etal. Changes of peripheral alpha-melanocyte-stimulating hormone in childhood obesity. Metabolism 2010; 59: 186-194.
98. Müller HL, Gebhardt U, Wessel V etal. First experiences with laparoscopic gastric banding (LAGB) in the treatment of obese patients with childhood craniopharyngioma. Klin Padiatr 2007; 219: 323-325.
99. Müller HL, Gebhardt U, Maroske J, Hanisch E. Long-term follow-up of morbidly obese patients with childhood craniopharyngioma after laparoscopic adjustable gastric banding (LAGB). Klin Padiatr 2011; 223: 372-373.
100. Lustig RH, Post SR, Srivannaboon K etal. Risk factors for the development of obesity in children surviving brain tumours. J Clin Endocrinol Metab 2003; 88: 611-616.
101. Holmer H, Pozarek G, Wirfält E etal. Reduced energy expenditure and impaired feeding-related signals but not high energy intake reinforces hypothalamic obesity in adults with childhood onset craniopharyngioma. J Clin Endocrinol Metab 2010; 95: 5395-5402.
102. Holmer H, Ekman B, Björk J etal. Hypothalamic involvement predicts cardiovascular risk in adults with childhood onset craniopharyngioma on long-term GH therapy. Eur J Endocrinol 2009; 161: 671-679.
103. De Vile CJ, Grant DB, Hayward RD, Kendall BE, Neville BGR, Stanhope R. Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging. J Clin Endocrinol Metab 1996; 81: 2734-2737.
104. Müller HL, Gebhardt U, Teske C etal. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma - results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after three year follow-up. Eur J Endocrinol 2011; 165: 17-24.
105. Puget S, Garnett M, Wray A etal. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg 2007; 106 (Suppl. 1): 3-12.
106. Müller HL. More or less? Treatment strategies in childhood craniopharyngioma. Childs Nerv Syst 2006; 22: 156-157.
107. Müller HL, Albanese A, Calaminus G etal. Consensus and perspectives on treatment strategies in childhood craniopharyngioma - results of a meeting of the Craniopharyngioma Study Group (SIOP), Genova 2004. J Pediatr Endocrinol Metab 2006; 19 (Suppl.1): 453-454.
108. Goldstone AP. Prader-Willi syndrome: advances in its genetics, pathophysiology and treatment. Trends Endocrinol Metab 2004; 15: 12-20.
109. Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 4183-4197.
110. Cassidy SB, Driscoll DJ. Prader-Willi syndrome. Eur J Hum Genet 2009; 17: 3-13.
111. Chamberlain SJ, Lalande M. Neurodevelopmental disorders involving genomic imprinting at human chromosome 15q11-q13. Neurobiol Dis 2010; 39: 13-20.
112. Sahoo T, Del GD, German JR etal. Prader-Willi phenotype caused by paternal deficiency for the HBII-85 C/D box small nucleolar RNA cluster. Nat. Gene 2008; 40: 719-721.
113. de Smith AJ, Purmann C, Walters RG etal. A deletion of the HBII-85 class of small nucleolar RNAs (snoRNAs) is associated with hyperphagia, obesity and hypogonadism. Hum Mol Genet 2009; 18: 3257-3265.
114. Butler JV, Whittington JE, Holland AJ, McAllister CJ, Goldstone AP. The transition between the phenotypes of Prader-Willi syndrome during infancy and early childhood. Dev Med Child Neurol 2009; 52: e88-e93.
115. Miller JL, Driscoll DC, Lynn CH etal. Nutritional phases in Prader-Willi syndrome. Am J Med Genet 2011; 155A: 1040-1049.
116. Farooqi IS. Monogenic human obesity. Front Horm Res 2008; 36: 1-11.
117. Stevenson DA, Heinemann J, Angulo M etal. Deaths due to choking in Prader-Willi syndrome. Am J Med Genet 2007; 143: 484-487.
118. Stevenson DA, Heinemann J, Angulo M etal. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr 2007; 45: 272-274.
119. Swaab DF, Purba JS, Hofman MA. Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases. J Clin Endocrinol Metab 1995; 80: 573-579.
120. Choe YH, Song S, Paik Y etal. Increased density of ghrelin expressing cells in the gastric fundus and body in Prader-Willi syndrome. J Clin Endocrinol Metab 2005; 909: 5441-5445.
121. Cummings DE, Clement K, Purnell JQ etal. Elevated plasma ghrelin levels in Prader Willi syndrome. Nat Med 2002; 8: 643-644.
122. Delparigi A, Tschop M, Heiman ML etal. High circulating ghrelin: a potential cause for hyperphagia and obesity in Prader-Willi syndrome. JCEM 2002; 87: 5461-5464.
123. Erdie-Lalena CR, Holm VA, Kelly PC, Frayo RS, Cummings DE. Ghrelin levels in young children with Prader-Willi syndrome. J Pediatr 2006; 149: 199-204.
124. Gimenez-Palop O, Gimenez-Perez G, Mauricio D etal. A lesser postprandial suppression of plasma ghrelin in Prader-Willi syndrome is associated with low fasting and a blunted postprandial PYY response. Clin Endocrinol (Oxf) 2007; 66: 198-204.
125. Goldstone AP, Patterson M, Kalingag N etal. Fasting and post-prandial hyperghrelinemia in Prader-Willi syndrome is partially explained by hypoinsulinemia, and is not due to peptide YY 3-36 deficiency or seen in hypothalamic obesity due to craniopharyngioma. JCEM 2005; 90: 2681-2690.
126. Goldstone AP, Thomas EL, Brynes AE etal. Elevated fasting plasma ghrelin in Prader-Willi syndrome adults is not solely explained by their reduced visceral adiposity and insulin resistance. JCEM 2004; 89: 1718-1726.
127. Haqq AM, Farooqi IS, O'Rahilly S etal. 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. JCEM 2003; 88: 174-178.
128. Haqq AM, Grambow SC, Muehlbauer M etal. Ghrelin concentrations in Prader-Willi syndrome (PWS) infants and children: changes during development. Clin Endocrinol (Oxf) 2008; 69: 911-920.
129. Kanumakala S, Greaves R, Pedreira C, Donath S, Warne G, Zacharin M etal. Fasting ghrelin levels are not elevated in children with hypothalamic obesity. J Clin Endocrinol Metab 2005; 90: 2691-2695.
130. Paik KH, Choe YH, Park WH etal. Suppression of acylated ghrelin during oral glucose tolerance test is correlated with whole-body insulin sensitivity in children with Prader-Willi syndrome. J Clin Endocrinol Metab 2006; 91: 1876-1881.
131. Paik KH, Jin DK, Song SY etal. 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.
132. Scerif M, Goldstone AP, Korbonits M. Ghrelin in obesity and endocrine diseases. Mol Cell Endocrinol 2011; 340: 15-25.
133. Tauber M, Conte-Auriol F, Moulin P, Molinas C, Delagnes V, Salles JP. Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption: a pathophysiological hypothesis. Horm Res 2004; 62: 49-54.
134. Feigerlova E, Diene G, Conte-Auriol F etal. Hyperghrelinemia precedes obesity in Prader-Willi syndrome. J Clin Endocrinol Metab 2008; 93: 2800-2805.
135. Goldstone AP, Holland AJ, Butler JV, Whittington JE. Appetite hormones and the transition to hyperphagia in children with Prader-Willi syndrome. Int J Obes 2012; doi: 10.1038/ijo.2011.274.
136. Ding F, Prints Y, Dhar MS etal. Lack of Pwcr1/MBII-85 snoRNA is critical for neonatal lethality in Prader-Willi syndrome mouse models. Mamm Genome 2005; 16: 424-431.
137. Relkovic D, Doe CM, Humby T etal. Behavioural and cognitive abnormalities in an imprinting centre deletion mouse model for Prader-Willi syndrome. Eur J Neurosci 2010; 31: 156-164.
138. Motaghedi R, Lipman EG, Hogg JE, Christos PJ, Vogiatzi MG, Angulo MA. Psychiatric adverse effects of rimonobant in adults with Prader Willi syndrome. Eur J Med Genet 2011; 54: 14-18.
139. Tan TM, Vanderpump M, Khoo B, Patterson M, Ghatei MA, Goldstone AP. Somatostatin infusion lowers plasma ghrelin without reducing appetite in adults with Prader-Willi syndrome. J Clin Endocrinol Metab 2004; 89: 4162-4165.
140. De WK, Ishkanian SL, Bogarin R etal. Long-acting octreotide treatment causes a sustained decrease in ghrelin concentrations but does not affect weight, behaviour and appetite in subjects with Prader-Willi syndrome. Eur J Endocrinol 2008; 159: 381-388.
141. Sze L, Purtell L, Jenkins A etal. Effects of a single dose of exenatide on appetite, gut hormones, and glucose homeostasis in adults with Prader-Willi syndrome. J Clin Endocrinol Metab 2011; 96: E1314-E1319.
142. Seetho IW, Jones G, Thomson GA, Fernando DJ. Treating diabetes mellitus in Prader-Willi syndrome with exenatide. Diabetes Res Clin Pract 2011; 92: e1-e2.
143. Paisey RB, Bower L, Rosindale S, Lawrence C. Succesful treatment of obesity and diabetes and incretin analogue over four years in an adults with Prader-Willi syndrome. Pract Diabetes 2011; 28: 306-307.
144. Choe YH, Jin DK, Kim SE etal. Hyperghrelinemia does not accelerate gastric emptying in Prader-Willi syndrome patients. J Clin Endocrinol Metab 2005; 90: 3367-3370.
145. Wharton R, Wang T, GraemeCook F, Briggs S, Cole R. Acute idiopathic gastric dilatation with gastric necrosis in individuals with Prader-Willi syndrome. Am J Med Genet 1997; 73: 437-441.
146. DePeppo F, DiGiorgio G, Germani M etal. BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits. Obes Surg 2008; 18: 1443-1449.
147. Scheimann A, Butler MG, Stevenson D, Miller JL, Cuffari C, Klish WJ. 'Efficacy of laparoscopic sleeve gastrectomy as a stand-alone technique for children with morbid obesity' and 'BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits'. Obes Surg 2009; 19: 671-672.
148. Scheimann AO, Butler MG, Gourash L, Cuffari C, Klish W. Critical analysis of bariatric procedures in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr 2008; 46: 80-83.
149. Coutant R, Maurey H, Rouleau S etal. Defect in epinephrine production in children with craniopharyngioma: functional or organic origin? J Clin Endocrinol Metab 2003; 88: 5969-5975.
150. Mason PW, Krawiecki N, Meacham LR. The use of dextroamphetamine to treat obesity and hyperphagia in children treated for craniopharyngioma. Arch Pediatr Adolesc Med 2002; 156: 887-892.
151. Ismail D, O'Connell MA, Zacharin MR. Dexamphetamine use for management of obesity and hypersomnolence following hypothalamic injury. J Pediatr Endocrinol Metab 2006; 19: 129-134.
152. Greenway FL, Bray GA. Treatment of hypothalamic obesity with caffeine and ephedrine. Endocr Pract 2008; 14: 697-703.
153. Danielsson P, Janson A, Norgren S, Marcus C. Impact of sibutramine therapy in children with hypothalamic obesity or obesity with aggravating syndromes. J Clin Endocrinol Metab 2007; 92: 4101-4106.
154. Hainerová IA, Zamrazilová H, Sedláčková D, Hainer V. Hypogonadotropic hypogonadism in a homozygous MC4R mutation carrier and the effect of sibutramine treatment on body weight and obesity-related health risks. Obes Facts 2011; 4: 324-328.
155. Roubert P, Dubern B, Plas P etal. Novel pharmacological MC4R agonists can efficiently activate mutated MC4R from obese patient with impaired endogenous agonist response. J Endocrinol 2010; 207: 177-183.
156. Lustig RH. Hypothalamic obesity: causes, consequences, treatment. Pediatr Endocrinol Rev 2008; 6: 220-227.
157. Weiss R. [WWW document]. URL http://clinicaltrials.gov/ct2/show/NCT00076362 (accessed January 2012).
158. Fernandes JK, Klein MJ, Ater JL, Kuttesch JF, Vassilopoulou-Sellin R. Triiodothyronine supplementation for hypothalamic obesity. Metabolism 2002; 51: 1381-1383.
159. Tiosano D, Eisentein I, Militianu D, Chrousos GP, Hochberg Z. 11 beta-Hydroxysteroid dehydrogenase activity in hypothalamic obesity. J Clin Endocrinol Metab 2003; 88: 379-384.
160. Geffner M, Lundberg M, Koltowska-Häggström M etal. Changes in height, weight, and body mass index in children with craniopharyngioma after three years of growth hormone therapy: analysis of KIGS (Pfizer International Growth Database). J Clin Endocrinol Metab 2004; 89: 5435-5440.
161. Fried M, Hainer V, Basdevant A etal. Bariatric scientific collaborative group expert panel interdisciplinary European guidelines for surgery for severe (morbid) obesity. Obes Surg 2007; 17: 260-270.
162. Inge TH, Krebs NF, Garcia VF etal. Bariatric surgery for severely overweight adolescents: concerns and recommendations. Pediatrics 2004; 114: 217-223.
163. Aslan IR, Ranadive SA, Ersoy BA, Rogers SJ, Lustig RH, Vaisse C. Bariatric surgery in a patient with complete MC4R deficiency. Int J Obes 2011; 35: 457-461.
164. Lanthaler M, Aigner F, Kinzl J, Sieb M, Cakar-Beck F, Nehoda H. Long-term results and complications following adjustable gastric banding. Obes Surg 2010; 20: 1078-1085.
165. Milone L, Strong V, Gagner M. Laparoscopic sleeve gastrectomy is superior to endoscopic intragastric balloon as a first stage procedure for the super-obese patient (BMI>50). Obes Surg 2005; 15: 612-617.
166. Himpens J, Dapri G, Cadiere GB. A prospective randomized study between laparoscopic gastric banding and laparoscopic isolated sleeve gastrectomy: results after 1 and 3 years. Obes Surg 2006; 16: 1450-1456.
167. Baltasar A, Serra C, Bou R, Bengochea M, Andreo L. Sleeve gastrectomy in a 10-year-old Child. Obes Surg 2008; 18: 733-736.
168. Dan D, Harnanan D, Seetahal S, Naraynsingh V, Teelucksingh S. Bariatric surgery in the management of childhood obesity: should there be an age limit? Obes Surg 2010; 20: 114-117.
169. Ramos A, Neto MG, Galvao M, Evangelista LF, Campos JM, Ferraz A. Laparoscopic greater curvature plication: initial results of an alternative restrictive bariatric procedure. Obes Surg 2010; 20: 913-918.
170. Sugerman HJ, Sugerman EL, DeMaria EJ etal. Bariatric surgery for severely obese adolescents. J Gastrointest Surg 2003; 7: 102-107.
171. Daskalakis M, Till H, Kiess W, Weiner RA. Roux-en-Y gastric bypass in an adolescent patient with Bardet-Biedl syndrome, a monogenic obesity disorder. Obes Surg 2010; 20: 121-125.
172. Marceau P, Marceau S, Biron S etal. Long-term experience with duodenal switch in adolescents. Obes Surg 2010; 20: 1609-1616.
173. Papadia FS, Adami GF, Marinari GM, Camerini G, Scopinaro N. Bariatric surgery in adolescents: a long-term follow-up study. Surg Obes Relat Dis 2007; 3: 465-468.
174. Leslie DB, Kellogg TA, Ikramuddin S. The surgical approach to management of pediatric obesity: when to refer and what to expect. Rev Endocr Metab Disord 2009; 10: 215-229.
175. 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.
176. Maccarinelli G, Sibilia V, Torsello A etal. Ghrelin regulates proliferation and differen- tiation of osteoblastic cells. J Endocrinol 2005; 184: 249-256.
177. Kral JG. Vagotomy as a treatment for morbid obesity. Surg Clin North Am 1979; 59: 1131-1138.
178. Kral JG, Görtz L, Hermansson G, Wallin GS. Gastroplasty for obesity: long-term weight loss improved by vagotomy. World J Surg 1998; 17: 75-78.
179. German J, Kim F, Schwartz GJ etal. Hypothalamic leptin signaling regulates hepatic insulin sensitivity via a neurocircuit involving the vagus nerve. Endocrinology 2009; 150: 4502-4511.
180. Williams DL, Grill HJ, Cummings DE, Kaplan JM. Vagotomy dissociates short- and long-term controls of circulating ghrelin. Endocrinology 2003; 144: 5184-5187.
181. Balbo SL, Grassiolli S, Ribeiro RA etal. Fat storage is partially dependent on vagal activity and insulin secretion of hypothalamic obese rat. Endocrine 2007; 31: 142-148.