Serum Ghrelin and Glucagon-like Peptide 1 Levels in Children with Prader-Willi and Bardet-Biedl Syndromes
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Original Article

Serum Ghrelin and Glucagon-like Peptide 1 Levels in Children with Prader-Willi and Bardet-Biedl Syndromes

J Clin Res Pediatr Endocrinol 2024;16(2):146-150 DOI: 10.4274/jcrpe.galenos.2023.2023-7-7
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Abstract

Objective

Prader-Willi syndrome (PWS) and Bardet-Biedl syndrome (BBS) are causes of pediatric syndromic obesity. We aimed to investigate a possible role for ghrelin and glucagon-like peptide-1 (GLP-1) in the pathophysiology of PWS and BBS.

Methods

The study included 12 children with PWS, 12 children with BBS, 13 pediatric obese controls (OC) and 12 pediatric lean controls (LC). Fasting serum ghrelin and GLP-1 levels were measured by ELISA.

Results

In the PWS group, no significant difference was detected for median ghrelin levels when compared with OC and LC, which were 0.96 (0.69-1.15), 0.92 (0.72-1.20) and 1.13 (0.84-1.29) ng/mL, respectively. Similarly, there was no difference in PWS median GLP-1 levels when compared with OC and LC; 1.86 (1.5-2.94), 2.24 (1.62-2.78) and 2.06 (1.8-3.41) ng/mL, respectively. In the BBS group, there was no difference in median ghrelin levels when compared with OC and LC; 1.05 (0.87-1.51), 0.92 (0.72-1.20) and 1.13 (0.84-1.29) ng/mL, respectively. Neither was there a significant difference in median GLP-1 levels; 2.46 (1.91-4.17), 2.24 (1.62-2.78) and 2.06 (1.8-3.41) ng/mL for BBS, OC and LC, respectively.

Conclusion

There were no differences in median fasting ghrelin or GLP-1 levels when comparing patients with PWS and BBS with obese or lean peers. However, similar studies with larger series are needed.

Keywords: BBS, PWS, ghrelin, GLP-1

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References

1

Muscogiuri G, Barrea L, Faggiano F, Maiorino MI, Parrillo M, Pugliese G, Ruggeri RM, Scarano E, Savastano S, Colao A; RESTARE. Obesity in Prader-Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches. J Endocrinol Invest 2021;44: 2057-2070. Epub 2021 Apr 23

2

Butler MG. Prader-Willi Syndrome: Obesity due to Genomic Imprinting. Curr Genomics. 2011;12:204-215.

3

Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest 2015;38:12491263. Epub 2015 Jun 11

4

Best S, Lord J, Roche M, Watson CM, Poulter JA, Bevers RPJ, Stuckey A, Szymanska K, Ellingford JM, Carmichael J, Brittain H, Toomes C, Inglehearn C, Johnson CA, Wheway G; Genomics England Research Consortium. Molecular diagnoses in the congenital malformations caused by ciliopathies cohort of the 100,000 Genomes Project. J Med Genet 2021;59:1791-747. Epub 2021 Oct 29

5

Cummings DE, Foster-Schubert KE, Overduin J. Ghrelin and energy balance: focus on current controversies. Curr Drug Targets 2005;6:153-169.

6

Cummings DE, Clement K, Purnell JQ, Vaisse C, Foster KE, Frayo RS, Schwartz MW, Basdevant A, Weigle DS. Elevated plasma ghrelin levels in Prader Willi syndrome. Nat Med 2002;8:643-644.

7

Büscher AK, Cetiner M, Büscher R, Wingen AM, Hauffa BP, Hoyer PF. Obesity in patients with Bardet-Biedl syndrome: influence of appetite-regulating hormones. Pediatr Nephrol 2012;27:2065-2071. Epub 2012 Jun 5

8

Kanoski SE, Fortin SM, Arnold M, Grill HJ, Hayes MR. Peripheral and central GLP-1 receptor populations mediate the anorectic effects of peripherally administered GLP-1 receptor agonists, liraglutide and exendin-4. Endocrinology 2011;152:3103-3112. Epub 2011 Jun 21

9

Ahmed RH, Huri HZ, Muniandy S, Al-Hamodi Z, Al-Absi B, Alsalahi A, Razif MF. Altered circulating concentrations of active glucagon-like peptide (GLP-1) and dipeptidyl peptidase 4 (DPP4) in obese subjects and their association with insulin resistance. Clin Biochem 2017;50:746-749. Epub 2017 Mar 11

10

Purtell L, Sze L, Loughnan G, Smith E, Herzog H, Sainsbury A, Steinbeck K, Campbell LV, Viardot A. In adults with Prader-Willi syndrome, elevated ghrelin levels are more consistent with hyperphagia than high PYY and GLP-1 levels. Neuropeptides 2011;45:301-307. Epub 2011 Jul 1

11

Bueno M, Boixadera-Planas E, Blanco-Hinojo L, Esteba-Castillo S, Giménez-Palop O, Torrents-Rodas D, Pujol J, Corripio R, Deus J, Caixàs A. Hunger and Satiety Peptides: Is There a Pattern to Classify Patients with Prader-Willi Syndrome? J Clin Med 2021;10:5170.

12

Beales PL, Elcioglu N, Woolf AS, Parker D, Flinter FA. New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey. J Med Genet 1999;36:437-446.

13

Neyzi O, Bundak R, Gökçay G, Günöz H, Furman A, Darendeliler F, Baş F. Reference Values for Weight, Height, Head Circumference, and Body Mass Index in Turkish Children. J Clin Res Pediatr Endocrinol 2015;7:280-293.

14

Marshall WA, Tanner JM. Variations in pattern of pubertal changes in girls. Arch Dis Child 1969;44:291-303.

15

Kuppens RJ, Diène G, Bakker NE, Molinas C, Faye S, Nicolino M, Bernoux D, Delhanty PJ, van der Lely AJ, Allas S, Julien M, Delale T, Tauber M, Hokken-Koelega AC. Elevated ratio of acylated to unacylated ghrelin in children and young adults with Prader-Willi syndrome. Endocrine 2015;50:633-642. Epub 2015 May 20

16

Tauber M, Coupaye M, Diene G, Molinas C, Valette M, Beauloye V. Prader-Willi syndrome: A model for understanding the ghrelin system. J Neuroendocrinol 2019;31:e12728. Epub 2019 May 23

17

Haqq AM, Grambow SC, Muehlbauer M, Newgard CB, Svetkey LP, Carrel AL, Yanovski JA, Purnell JQ, Freemark M. Ghrelin concentrations in Prader-Willi syndrome (PWS) infants and children: changes during development. Clin Endocrinol (Oxf) 2008;69:911-920. Epub 2008 Aug 15

18

Butler MG, Bittel DC. Plasma obestatin and ghrelin levels in subjects with Prader-Willi syndrome. Am J Med Genet A 2007;143:415-421.

19

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.

20

Choe YH, Song SY, Paik KH, Oh YJ, Chu SH, Yeo SH, Kwon EK, Kim EM, Rha MY, Jin DK. Increased density of ghrelin-expressing cells in the gastric fundus and body in Prader-Willi syndrome. J Clin Endocrinol Metab 2005;90:5441-5445. Epub 2005 Jun 14

21

Turkkahraman D, Sirazi EC, Aykal G. Serum alpha-melanocyte-stimulating hormone (a-MSH), brain-derived neurotrophic factor (BDNF), and agouti-related protein (AGRP) levels in children with Prader-Willi or Bardet-Biedl syndromes. J Endocrinol Invest 2022;45:1031-1037. Epub 2022 Jan 31

22

Guo DF, Rahmouni K. Molecular basis of the obesity associated with Bardet-Biedl syndrome. Trends Endocrinol Metab 2011;22:286-293. Epub 2011 Apr 21

23

Guo DF, Lin Z, Wu Y, Searby C, Thedens DR, Richerson GB, Usachev YM, Grobe JL, Sheffield VC, Rahmouni K. The BBSome in POMC and AgRP Neurons Is Necessary for Body Weight Regulation and Sorting of Metabolic Receptors. Diabetes 2019;68:1591-1603. Epub 2019 May 24