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Published in: Current Diabetes Reports 9/2020

Open Access 01-09-2020 | Obesity | Genetics (AP Morris, Section Editor)

Bariatric Surgery for Monogenic Non-syndromic and Syndromic Obesity Disorders

Authors: Niels Vos, Sabrina M. Oussaada, Mellody I. Cooiman, Lotte Kleinendorst, Kasper W. ter Horst, Eric J. Hazebroek, Johannes A. Romijn, Mireille J. Serlie, Marcel M. A. M. Mannens, Mieke M. van Haelst

Published in: Current Diabetes Reports | Issue 9/2020

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Abstract

Purpose of Review

The global prevalence of obesity has increased rapidly over the last decades, posing a severe threat to human health. Currently, bariatric surgery is the most effective therapy for patients with morbid obesity. It is unknown whether this treatment is also suitable for patients with obesity due to a confirmed genetic defect (genetic obesity disorders). Therefore, this review aims to elucidate the role of bariatric surgery in the treatment of genetic obesity.

Recent Findings

In monogenic non-syndromic obesity, an underlying genetic defect seems to be the most important factor determining the efficacy of bariatric surgery. In syndromic obesity, bariatric surgery result data are scarce, and even though some promising follow-up results have been reported, caution is required as patients with more severe behavioral and developmental disorders might have poorer outcomes.

Summary

There is limited evidence in support of bariatric surgery as a treatment option for genetic obesity disorders; hence, no strong statements can be made regarding the efficacy and safety of these procedures for these patients. However, considering that patients with genetic obesity often present with life-threatening obesity-related comorbidities, we believe that bariatric surgery could be considered a last-resort treatment option in selected patients.
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Literature
1.
go back to reference (WHO) WHO. The top 10 causes of death. 2018. (WHO) WHO. The top 10 causes of death. 2018.
2.
3.
go back to reference Oussaada SM, van Galen KA, Cooiman MI, Kleinendorst L, Hazebroek EJ, van Haelst MM, et al. The pathogenesis of obesity. Metabolism. 2019;92:26–36. Oussaada SM, van Galen KA, Cooiman MI, Kleinendorst L, Hazebroek EJ, van Haelst MM, et al. The pathogenesis of obesity. Metabolism. 2019;92:26–36.
4.
go back to reference Bray GA, Kim KK, Wilding JPH. Obesity: a chronic relapsing progressive disease process. A position statement of the World Obesity Federation. Obes Rev. 2017;18(7):715–23.PubMed Bray GA, Kim KK, Wilding JPH. Obesity: a chronic relapsing progressive disease process. A position statement of the World Obesity Federation. Obes Rev. 2017;18(7):715–23.PubMed
5.
go back to reference Kleinendorst L, Massink MPG, Cooiman MI, Savas M, van der Baan-Slootweg OH, Roelants RJ, et al. Genetic obesity: next-generation sequencing results of 1230 patients with obesity. J Med Genet. 2018;55(9):578–86. Kleinendorst L, Massink MPG, Cooiman MI, Savas M, van der Baan-Slootweg OH, Roelants RJ, et al. Genetic obesity: next-generation sequencing results of 1230 patients with obesity. J Med Genet. 2018;55(9):578–86.
6.
go back to reference Bell CG, Walley AJ, Froguel P. The genetics of human obesity. Nat Rev Genet. 2005;6(3):221–34.PubMed Bell CG, Walley AJ, Froguel P. The genetics of human obesity. Nat Rev Genet. 2005;6(3):221–34.PubMed
7.
go back to reference Nordang GBN, Busk OL, Tveten K, Hanevik HI, Fell AKM, Hjelmesaeth J, et al. Next-generation sequencing of the monogenic obesity genes LEP, LEPR, MC4R, PCSK1 and POMC in a Norwegian cohort of patients with morbid obesity and normal weight controls. Mol Genet Metab. 2017;121(1):51–6.PubMed Nordang GBN, Busk OL, Tveten K, Hanevik HI, Fell AKM, Hjelmesaeth J, et al. Next-generation sequencing of the monogenic obesity genes LEP, LEPR, MC4R, PCSK1 and POMC in a Norwegian cohort of patients with morbid obesity and normal weight controls. Mol Genet Metab. 2017;121(1):51–6.PubMed
8.
go back to reference Saeed S, Bonnefond A, Manzoor J, Shabbir F, Ayesha H, Philippe J, et al. Genetic variants in LEP, LEPR, and MC4R explain 30% of severe obesity in children from a consanguineous population. Obesity (Silver Spring, Md). 2015;23(8):1687–95. Saeed S, Bonnefond A, Manzoor J, Shabbir F, Ayesha H, Philippe J, et al. Genetic variants in LEP, LEPR, and MC4R explain 30% of severe obesity in children from a consanguineous population. Obesity (Silver Spring, Md). 2015;23(8):1687–95.
9.
go back to reference Akinci A, Turkkahraman D, Tekedereli I, Ozer L, Evren B, Sahin I, et al. Novel mutations in obesity-related genes in Turkish children with non-syndromic early onset severe obesity: a multicentre study. J Clin Res Pediatr Endocrinol. 2019;11(4):341–9.PubMedPubMedCentral Akinci A, Turkkahraman D, Tekedereli I, Ozer L, Evren B, Sahin I, et al. Novel mutations in obesity-related genes in Turkish children with non-syndromic early onset severe obesity: a multicentre study. J Clin Res Pediatr Endocrinol. 2019;11(4):341–9.PubMedPubMedCentral
11.
go back to reference Rohde K, Keller M, la Cour PL, Bluher M, Kovacs P, Bottcher Y. Genetics and epigenetics in obesity. Metabolism. 2019;92:37–50.PubMed Rohde K, Keller M, la Cour PL, Bluher M, Kovacs P, Bottcher Y. Genetics and epigenetics in obesity. Metabolism. 2019;92:37–50.PubMed
12.
go back to reference Poirier P, Cornier MA, Mazzone T, Stiles S, Cummings S, Klein S, et al. Bariatric surgery and cardiovascular risk factors: a scientific statement from the American Heart Association. Circulation. 2011;123(15):1683–701. Poirier P, Cornier MA, Mazzone T, Stiles S, Cummings S, Klein S, et al. Bariatric surgery and cardiovascular risk factors: a scientific statement from the American Heart Association. Circulation. 2011;123(15):1683–701.
13.
go back to reference Aarts EO, Dogan K, Koehestanie P, Aufenacker TJ, Janssen IM, Berends FJ. Long-term results after laparoscopic adjustable gastric banding: a mean fourteen year follow-up study. Surg Obes Relat Dis. 2014;10(4):633–40.PubMed Aarts EO, Dogan K, Koehestanie P, Aufenacker TJ, Janssen IM, Berends FJ. Long-term results after laparoscopic adjustable gastric banding: a mean fourteen year follow-up study. Surg Obes Relat Dis. 2014;10(4):633–40.PubMed
14.
go back to reference Peterli R, Steinert RE, Woelnerhanssen B, Peters T, Christoffel-Courtin C, Gass M, et al. Metabolic and hormonal changes after laparoscopic Roux-en-Y gastric bypass and sleeve gastrectomy: a randomized, prospective trial. Obes Surg. 2012;22(5):740–8. Peterli R, Steinert RE, Woelnerhanssen B, Peters T, Christoffel-Courtin C, Gass M, et al. Metabolic and hormonal changes after laparoscopic Roux-en-Y gastric bypass and sleeve gastrectomy: a randomized, prospective trial. Obes Surg. 2012;22(5):740–8.
15.
go back to reference Ramon JM, Salvans S, Crous X, Puig S, Goday A, Benaiges D, et al. Effect of Roux-en-Y gastric bypass vs sleeve gastrectomy on glucose and gut hormones: a prospective randomised trial. J Gastrointest Surg. 2012;16(6):1116–22.PubMed Ramon JM, Salvans S, Crous X, Puig S, Goday A, Benaiges D, et al. Effect of Roux-en-Y gastric bypass vs sleeve gastrectomy on glucose and gut hormones: a prospective randomised trial. J Gastrointest Surg. 2012;16(6):1116–22.PubMed
16.
go back to reference Yousseif A, Emmanuel J, Karra E, Millet Q, Elkalaawy M, Jenkinson AD, et al. Differential effects of laparoscopic sleeve gastrectomy and laparoscopic gastric bypass on appetite, circulating acyl-ghrelin, peptide YY3-36 and active GLP-1 levels in non-diabetic humans. Obes Surg. 2014;24(2):241–52. Yousseif A, Emmanuel J, Karra E, Millet Q, Elkalaawy M, Jenkinson AD, et al. Differential effects of laparoscopic sleeve gastrectomy and laparoscopic gastric bypass on appetite, circulating acyl-ghrelin, peptide YY3-36 and active GLP-1 levels in non-diabetic humans. Obes Surg. 2014;24(2):241–52.
17.
go back to reference Inge TH, Coley RY, Bazzano LA, Xanthakos SA, McTigue K, Arterburn D, et al. Comparative effectiveness of bariatric procedures among adolescents: the PCORnet bariatric study. Surg Obes Relat Dis. 2018;14(9):1374–86. Inge TH, Coley RY, Bazzano LA, Xanthakos SA, McTigue K, Arterburn D, et al. Comparative effectiveness of bariatric procedures among adolescents: the PCORnet bariatric study. Surg Obes Relat Dis. 2018;14(9):1374–86.
18.
go back to reference Crowley VE. Overview of human obesity and central mechanisms regulating energy homeostasis. Ann Clin Biochem. 2008;45(Pt 3):245–55.PubMed Crowley VE. Overview of human obesity and central mechanisms regulating energy homeostasis. Ann Clin Biochem. 2008;45(Pt 3):245–55.PubMed
19.
go back to reference Farooqi IS, O'Rahilly S. Mutations in ligands and receptors of the leptin-melanocortin pathway that lead to obesity. Nat Clin Pract Endocrinol Metab. 2008;4(10):569–77.PubMed Farooqi IS, O'Rahilly S. Mutations in ligands and receptors of the leptin-melanocortin pathway that lead to obesity. Nat Clin Pract Endocrinol Metab. 2008;4(10):569–77.PubMed
20.
go back to reference Farooqi S, O'Rahilly S. Genetics of obesity in humans. Endocr Rev. 2006;27(7):710–8.PubMed Farooqi S, O'Rahilly S. Genetics of obesity in humans. Endocr Rev. 2006;27(7):710–8.PubMed
22.
go back to reference Kleinendorst L Chapter 5 - Molecular basis of obesity disorders. Clinical molecular medicine: principles and practice. In: Van Haelst MM, editor.: Elsevier; 2020. p. 80. Kleinendorst L Chapter 5 - Molecular basis of obesity disorders. Clinical molecular medicine: principles and practice. In: Van Haelst MM, editor.: Elsevier; 2020. p. 80.
23.
go back to reference Doulla M, McIntyre AD, Hegele RA, Gallego PH. A novel MC4R mutation associated with childhood-onset obesity: a case report. Paediatr Child Health. 2014;19(10):515–8.PubMedPubMedCentral Doulla M, McIntyre AD, Hegele RA, Gallego PH. A novel MC4R mutation associated with childhood-onset obesity: a case report. Paediatr Child Health. 2014;19(10):515–8.PubMedPubMedCentral
24.
go back to reference Farooqi IS, Yeo GS, Keogh JM, Aminian S, Jebb SA, Butler G, et al. Dominant and recessive inheritance of morbid obesity associated with melanocortin 4 receptor deficiency. J Clin Invest. 2000;106(2):271–9.PubMedPubMedCentral Farooqi IS, Yeo GS, Keogh JM, Aminian S, Jebb SA, Butler G, et al. Dominant and recessive inheritance of morbid obesity associated with melanocortin 4 receptor deficiency. J Clin Invest. 2000;106(2):271–9.PubMedPubMedCentral
25.
go back to reference Farooqi IS, Keogh JM, Yeo GS, Lank EJ, Cheetham T, O'Rahilly S. Clinical spectrum of obesity and mutations in the melanocortin 4 receptor gene. N Engl J Med. 2003;348(12):1085–95.PubMed Farooqi IS, Keogh JM, Yeo GS, Lank EJ, Cheetham T, O'Rahilly S. Clinical spectrum of obesity and mutations in the melanocortin 4 receptor gene. N Engl J Med. 2003;348(12):1085–95.PubMed
26.
go back to reference Potoczna N, Branson R, Kral JG, Piec G, Steffen R, Ricklin T, et al. Gene variants and binge eating as predictors of comorbidity and outcome of treatment in severe obesity. J Gastrointest Surg. 2004;8(8):971–81 discussion 81-2.PubMed Potoczna N, Branson R, Kral JG, Piec G, Steffen R, Ricklin T, et al. Gene variants and binge eating as predictors of comorbidity and outcome of treatment in severe obesity. J Gastrointest Surg. 2004;8(8):971–81 discussion 81-2.PubMed
27.
go back to reference Valette M, Poitou C, Le Beyec J, Bouillot JL, Clement K, Czernichow S. Melanocortin-4 receptor mutations and polymorphisms do not affect weight loss after bariatric surgery. PLoS One. 2012;7(11):e48221.PubMedPubMedCentral Valette M, Poitou C, Le Beyec J, Bouillot JL, Clement K, Czernichow S. Melanocortin-4 receptor mutations and polymorphisms do not affect weight loss after bariatric surgery. PLoS One. 2012;7(11):e48221.PubMedPubMedCentral
28.
go back to reference Aslan IR, Campos GM, Calton MA, Evans DS, Merriman RB, Vaisse C. Weight loss after Roux-en-Y gastric bypass in obese patients heterozygous for MC4R mutations. Obes Surg. 2011;21(7):930–4.PubMed Aslan IR, Campos GM, Calton MA, Evans DS, Merriman RB, Vaisse C. Weight loss after Roux-en-Y gastric bypass in obese patients heterozygous for MC4R mutations. Obes Surg. 2011;21(7):930–4.PubMed
29.
go back to reference Hatoum IJ, Stylopoulos N, Vanhoose AM, Boyd KL, Yin DP, Ellacott KL, et al. Melanocortin-4 receptor signaling is required for weight loss after gastric bypass surgery. J Clin Endocrinol Metab. 2012;97(6):E1023–31.PubMedPubMedCentral Hatoum IJ, Stylopoulos N, Vanhoose AM, Boyd KL, Yin DP, Ellacott KL, et al. Melanocortin-4 receptor signaling is required for weight loss after gastric bypass surgery. J Clin Endocrinol Metab. 2012;97(6):E1023–31.PubMedPubMedCentral
30.
go back to reference Censani M, Conroy R, Deng L, Oberfield SE, McMahon DJ, Zitsman JL, et al. Weight loss after bariatric surgery in morbidly obese adolescents with MC4R mutations. Obesity (Silver Spring, Md). 2014;22(1):225–31. Censani M, Conroy R, Deng L, Oberfield SE, McMahon DJ, Zitsman JL, et al. Weight loss after bariatric surgery in morbidly obese adolescents with MC4R mutations. Obesity (Silver Spring, Md). 2014;22(1):225–31.
31.
go back to reference Mul JD, Begg DP, Alsters SI, van Haaften G, Duran KJ, D'Alessio DA, et al. Effect of vertical sleeve gastrectomy in melanocortin receptor 4-deficient rats. Am J Physiol Endocrinol Metab. 2012;303(1):E103–10.PubMedPubMedCentral Mul JD, Begg DP, Alsters SI, van Haaften G, Duran KJ, D'Alessio DA, et al. Effect of vertical sleeve gastrectomy in melanocortin receptor 4-deficient rats. Am J Physiol Endocrinol Metab. 2012;303(1):E103–10.PubMedPubMedCentral
32.
go back to reference Moore BS, Mirshahi UL, Yost EA, Stepanchick AN, Bedrin MD, Styer AM, et al. Long-term weight-loss in gastric bypass patients carrying melanocortin 4 receptor variants. PLoS One. 2014;9(4):e93629. Moore BS, Mirshahi UL, Yost EA, Stepanchick AN, Bedrin MD, Styer AM, et al. Long-term weight-loss in gastric bypass patients carrying melanocortin 4 receptor variants. PLoS One. 2014;9(4):e93629.
33.
go back to reference Elkhenini HF, New JP, Syed AA. Five-year outcome of bariatric surgery in a patient with melanocortin-4 receptor mutation. Clin Obes. 2014;4(2):121–4.PubMed Elkhenini HF, New JP, Syed AA. Five-year outcome of bariatric surgery in a patient with melanocortin-4 receptor mutation. Clin Obes. 2014;4(2):121–4.PubMed
34.
go back to reference • Jelin EB, Daggag H, Speer AL, Hameed N, Lessan N, Barakat M, et al. Melanocortin-4 receptor signaling is not required for short-term weight loss after sleeve gastrectomy in pediatric patients. Int J Obes. 2016;40(3):550–3. Paediatric patients with homozygous. MC4R. mutations show initial short-term weight loss. At long term, there was weight regain in one patient. • Jelin EB, Daggag H, Speer AL, Hameed N, Lessan N, Barakat M, et al. Melanocortin-4 receptor signaling is not required for short-term weight loss after sleeve gastrectomy in pediatric patients. Int J Obes. 2016;40(3):550–3. Paediatric patients with homozygous. MC4R. mutations show initial short-term weight loss. At long term, there was weight regain in one patient.
35.
go back to reference • Bonnefond A, Keller R, Meyre D, Stutzmann F, Thuillier D, Stefanov DG, et al. Eating behavior, low-frequency functional mutations in the melanocortin-4 receptor (MC4R) gene, and outcomes of bariatric operations: a 6-year prospective study. Diabetes Care. 2016;39(8):1384–92. Carriers of. MC4R. variants had poorer outcomes after bariatric surgery. The described variants are not all pathogenic. It is therefore hard to draw firm conclusions.PubMed • Bonnefond A, Keller R, Meyre D, Stutzmann F, Thuillier D, Stefanov DG, et al. Eating behavior, low-frequency functional mutations in the melanocortin-4 receptor (MC4R) gene, and outcomes of bariatric operations: a 6-year prospective study. Diabetes Care. 2016;39(8):1384–92. Carriers of. MC4R. variants had poorer outcomes after bariatric surgery. The described variants are not all pathogenic. It is therefore hard to draw firm conclusions.PubMed
36.
go back to reference •• Cooiman MI, Kleinendorst L, Aarts EO, Janssen IMC, van Amstel HKP, Blakemore AI, et al. Genetic obesity and bariatric surgery outcome in 1014 patients with morbid obesity. Obes Surg. 2020;30(2):470–7. Various genetic defects are described. RYGB is more effective than SG in patients with an. MC4R. mutation. No significant differences in bariatric surgery outcomes between patients with. PCSK1, POMC, and. SIM1. mutations and non-carriers.PubMed •• Cooiman MI, Kleinendorst L, Aarts EO, Janssen IMC, van Amstel HKP, Blakemore AI, et al. Genetic obesity and bariatric surgery outcome in 1014 patients with morbid obesity. Obes Surg. 2020;30(2):470–7. Various genetic defects are described. RYGB is more effective than SG in patients with an. MC4R. mutation. No significant differences in bariatric surgery outcomes between patients with. PCSK1, POMC, and. SIM1. mutations and non-carriers.PubMed
37.
go back to reference Kleinendorst L, Abawi O, van der Kamp HJ, Alders M, Meijers-Heijboer HEJ, van Rossum EFC, et al. Leptin receptor deficiency: a systematic literature review and prevalence estimation based on population genetics. Eur J Endocrinol. 2020;182(1):47–56. Kleinendorst L, Abawi O, van der Kamp HJ, Alders M, Meijers-Heijboer HEJ, van Rossum EFC, et al. Leptin receptor deficiency: a systematic literature review and prevalence estimation based on population genetics. Eur J Endocrinol. 2020;182(1):47–56.
38.
go back to reference Nizard J, Dommergues M, Clément K. Pregnancy in a woman with a leptin-receptor mutation. N Engl J Med. 2012;366(11):1064–5.PubMed Nizard J, Dommergues M, Clément K. Pregnancy in a woman with a leptin-receptor mutation. N Engl J Med. 2012;366(11):1064–5.PubMed
39.
go back to reference Huvenne H, Le Beyec J, Pepin D, Alili R, Kherchiche PP, Jeannic E, et al. Seven novel deleterious LEPR mutations found in early-onset obesity: a DeltaExon6-8 shared by subjects from Reunion Island, France, suggests a founder effect. J Clin Endocrinol Metab. 2015;100(5):E757–66.PubMed Huvenne H, Le Beyec J, Pepin D, Alili R, Kherchiche PP, Jeannic E, et al. Seven novel deleterious LEPR mutations found in early-onset obesity: a DeltaExon6-8 shared by subjects from Reunion Island, France, suggests a founder effect. J Clin Endocrinol Metab. 2015;100(5):E757–66.PubMed
40.
go back to reference •• Nunziata A, Funcke JB, Borck G, von Schnurbein J, Brandt S, Lennerz B, et al. Functional and phenotypic characteristics of human leptin receptor mutations. J Endocr Soc. 2019;3(1):27–41. This review provides an overview of what is known of the outcomes of bariatric surgery in patients with biallelic. LEPR. mutations. Gender-related differences are seen, with long-lasting weight loss in males (n=3), but not in females (n=3).PubMed •• Nunziata A, Funcke JB, Borck G, von Schnurbein J, Brandt S, Lennerz B, et al. Functional and phenotypic characteristics of human leptin receptor mutations. J Endocr Soc. 2019;3(1):27–41. This review provides an overview of what is known of the outcomes of bariatric surgery in patients with biallelic. LEPR. mutations. Gender-related differences are seen, with long-lasting weight loss in males (n=3), but not in females (n=3).PubMed
41.
go back to reference Le Beyec J, Cugnet-Anceau C, Pepin D, Alili R, Cotillard A, Lacorte JM, et al. Homozygous leptin receptor mutation due to uniparental disomy of chromosome 1: response to bariatric surgery. J Clin Endocrinol Metab. 2013;98(2):E397–402.PubMed Le Beyec J, Cugnet-Anceau C, Pepin D, Alili R, Cotillard A, Lacorte JM, et al. Homozygous leptin receptor mutation due to uniparental disomy of chromosome 1: response to bariatric surgery. J Clin Endocrinol Metab. 2013;98(2):E397–402.PubMed
42.
go back to reference Dehghani M, Mehrjardi M, Dilaver N, Tajamolian M, Enayati S, Ebrahimi P, et al. Potential role of gender specific effect of leptin receptor deficiency in an extended consanguineous family with severe early-onset obesity. Eur J Med Genet. 2018;61:465–7.PubMed Dehghani M, Mehrjardi M, Dilaver N, Tajamolian M, Enayati S, Ebrahimi P, et al. Potential role of gender specific effect of leptin receptor deficiency in an extended consanguineous family with severe early-onset obesity. Eur J Med Genet. 2018;61:465–7.PubMed
43.
go back to reference Krude H, Biebermann H, Luck W, Horn R, Brabant G, Gruters A. Severe early-onset obesity, adrenal insufficiency and red hair pigmentation caused by POMC mutations in humans. Nat Genet. 1998;19(2):155–7.PubMed Krude H, Biebermann H, Luck W, Horn R, Brabant G, Gruters A. Severe early-onset obesity, adrenal insufficiency and red hair pigmentation caused by POMC mutations in humans. Nat Genet. 1998;19(2):155–7.PubMed
44.
go back to reference Millington GW. Proopiomelanocortin (POMC): the cutaneous roles of its melanocortin products and receptors. Clin Exp Dermatol. 2006;31(3):407–12.PubMed Millington GW. Proopiomelanocortin (POMC): the cutaneous roles of its melanocortin products and receptors. Clin Exp Dermatol. 2006;31(3):407–12.PubMed
45.
go back to reference Mountjoy KG, Caron A, Hubbard K, Shome A, Grey AC, Sun B, et al. Desacetyl-alpha-melanocyte stimulating hormone and alpha-melanocyte stimulating hormone are required to regulate energy balance. Mol Metab. 2018;9:207–16. Mountjoy KG, Caron A, Hubbard K, Shome A, Grey AC, Sun B, et al. Desacetyl-alpha-melanocyte stimulating hormone and alpha-melanocyte stimulating hormone are required to regulate energy balance. Mol Metab. 2018;9:207–16.
46.
go back to reference Dubern B, Lubrano-Berthelier C, Mencarelli M, Ersoy B, Frelut ML, Bougle D, et al. Mutational analysis of the pro-opiomelanocortin gene in French obese children led to the identification of a novel deleterious heterozygous mutation located in the alpha-melanocyte stimulating hormone domain. Pediatr Res. 2008;63(2):211–6.PubMed Dubern B, Lubrano-Berthelier C, Mencarelli M, Ersoy B, Frelut ML, Bougle D, et al. Mutational analysis of the pro-opiomelanocortin gene in French obese children led to the identification of a novel deleterious heterozygous mutation located in the alpha-melanocyte stimulating hormone domain. Pediatr Res. 2008;63(2):211–6.PubMed
47.
go back to reference Kublaoui BM, Holder JL Jr, Gemelli T, Zinn AR. Sim1 haploinsufficiency impairs melanocortin-mediated anorexia and activation of paraventricular nucleus neurons. Mol Endocrinol. 2006;20(10):2483–92.PubMed Kublaoui BM, Holder JL Jr, Gemelli T, Zinn AR. Sim1 haploinsufficiency impairs melanocortin-mediated anorexia and activation of paraventricular nucleus neurons. Mol Endocrinol. 2006;20(10):2483–92.PubMed
48.
go back to reference Blackburn PR, Sullivan AE, Gerassimou AG, Kleinendorst L, Bersten DC, Cooiman M, et al. Functional analysis of the SIM1 variant p.G715V in 2 patients with obesity. J Clin Endocrinol Metab. 2020;105(1). Blackburn PR, Sullivan AE, Gerassimou AG, Kleinendorst L, Bersten DC, Cooiman M, et al. Functional analysis of the SIM1 variant p.G715V in 2 patients with obesity. J Clin Endocrinol Metab. 2020;105(1).
49.
50.
go back to reference Stijnen P, Ramos-Molina B, O'Rahilly S, Creemers JW. PCSK1 mutations and human endocrinopathies: from obesity to gastrointestinal disorders. Endocr Rev. 2016;37(4):347–71.PubMed Stijnen P, Ramos-Molina B, O'Rahilly S, Creemers JW. PCSK1 mutations and human endocrinopathies: from obesity to gastrointestinal disorders. Endocr Rev. 2016;37(4):347–71.PubMed
51.
go back to reference Kaur Y, de Souza RJ, Gibson WT, Meyre D. A systematic review of genetic syndromes with obesity. Obes Rev. 2017;18(6):603–34.PubMed Kaur Y, de Souza RJ, Gibson WT, Meyre D. A systematic review of genetic syndromes with obesity. Obes Rev. 2017;18(6):603–34.PubMed
52.
53.
go back to reference Chen C, Visootsak J, Dills S, Graham JM Jr. Prader-Willi syndrome: an update and review for the primary pediatrician. Clin Pediatr (Phila). 2007;46(7):580–91. Chen C, Visootsak J, Dills S, Graham JM Jr. Prader-Willi syndrome: an update and review for the primary pediatrician. Clin Pediatr (Phila). 2007;46(7):580–91.
54.
go back to reference Miller JL, Lynn CH, Driscoll DC, Goldstone AP, Gold JA, Kimonis V, et al. Nutritional phases in Prader-Willi syndrome. Am J Med Genet A. 2011;155a(5):1040–9.PubMedPubMedCentral Miller JL, Lynn CH, Driscoll DC, Goldstone AP, Gold JA, Kimonis V, et al. Nutritional phases in Prader-Willi syndrome. Am J Med Genet A. 2011;155a(5):1040–9.PubMedPubMedCentral
55.
go back to reference Driscoll DJ, Miller JL, Schwartz S, Cassidy SB. Prader-Willi syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews(®). Seattle (WA): University of Washington, Seattle Copyright © 1993–2020, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.; 1993 Driscoll DJ, Miller JL, Schwartz S, Cassidy SB. Prader-Willi syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews(®). Seattle (WA): University of Washington, Seattle Copyright © 1993–2020, University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle. All rights reserved.; 1993
56.
go back to reference 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(7):881–7.PubMed 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(7):881–7.PubMed
58.
go back to reference Lima VP, Emerich DR, Mesquita ML, Paternez AC, Carreiro LR, Pina Neto JM, et al. Nutritional intervention with hypocaloric diet for weight control in children and adolescents with Prader-Willi syndrome. Eat Behav. 2016;21:189–92.PubMed Lima VP, Emerich DR, Mesquita ML, Paternez AC, Carreiro LR, Pina Neto JM, et al. Nutritional intervention with hypocaloric diet for weight control in children and adolescents with Prader-Willi syndrome. Eat Behav. 2016;21:189–92.PubMed
59.
go back to reference le Roux CW, Heneghan HM. Bariatric surgery for obesity. Med Clin N Am. 2018;102(1):165–82.PubMed le Roux CW, Heneghan HM. Bariatric surgery for obesity. Med Clin N Am. 2018;102(1):165–82.PubMed
60.
go back to reference 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(1):80–3.PubMedPubMedCentral 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(1):80–3.PubMedPubMedCentral
61.
go back to reference • Alqahtani AR, Elahmedi MO, Al Qahtani AR, Lee J, Butler MG. Laparoscopic sleeve gastrectomy in children and adolescents with Prader-Willi syndrome: a matched-control study. Surg Obes Relat Dis. 2016;12(1):100–10. This study describes the effect of SG in children with PWS and shows similar BMI loss up to 3 years after surgery, compared with controls.PubMed • Alqahtani AR, Elahmedi MO, Al Qahtani AR, Lee J, Butler MG. Laparoscopic sleeve gastrectomy in children and adolescents with Prader-Willi syndrome: a matched-control study. Surg Obes Relat Dis. 2016;12(1):100–10. This study describes the effect of SG in children with PWS and shows similar BMI loss up to 3 years after surgery, compared with controls.PubMed
62.
go back to reference •• Cazzo E, Gestic MA, Utrini MP, Chaim FDM, Candido EC, Jarolavsky L, et al. Bariatric surgery in individuals with severe cognitive impairment: report of two cases. Sao Paulo Med J. 2018;136(1):84–8. One child with PWS responded well to BPD (55% excess weight loss after 1 year).PubMed •• Cazzo E, Gestic MA, Utrini MP, Chaim FDM, Candido EC, Jarolavsky L, et al. Bariatric surgery in individuals with severe cognitive impairment: report of two cases. Sao Paulo Med J. 2018;136(1):84–8. One child with PWS responded well to BPD (55% excess weight loss after 1 year).PubMed
63.
go back to reference •• Martinelli V, Chiappedi M, Pellegrino E, Zugnoni M, Caccialanza R, Muggia C, et al. Laparoscopic sleeve gastrectomy in an adolescent with Prader-Willi syndrome: psychosocial implications. Nutrition. 2019;61:67–9. One child with PWS responded well to SG (29.2% excess weight loss after 6 months).PubMed •• Martinelli V, Chiappedi M, Pellegrino E, Zugnoni M, Caccialanza R, Muggia C, et al. Laparoscopic sleeve gastrectomy in an adolescent with Prader-Willi syndrome: psychosocial implications. Nutrition. 2019;61:67–9. One child with PWS responded well to SG (29.2% excess weight loss after 6 months).PubMed
64.
go back to reference • Michalik M, Frask A, Lech P, Zdrojewski M, Doboszynska A. The usefulness of biliopancreatic diversion/Scopinaro operation in treatment of patients with Prader-Willi syndrome. Wideochir Inne Tech Maloinwazyjne. 2015;10(2):324–7. Two patients with PWS responded well to BPD (25 to 43% excess weight loss after 18 and 6 months respectively).PubMedPubMedCentral • Michalik M, Frask A, Lech P, Zdrojewski M, Doboszynska A. The usefulness of biliopancreatic diversion/Scopinaro operation in treatment of patients with Prader-Willi syndrome. Wideochir Inne Tech Maloinwazyjne. 2015;10(2):324–7. Two patients with PWS responded well to BPD (25 to 43% excess weight loss after 18 and 6 months respectively).PubMedPubMedCentral
65.
go back to reference Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N, et al. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr. 2007;45(2):272–4. Stevenson DA, Heinemann J, Angulo M, Butler MG, Loker J, Rupe N, et al. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenterol Nutr. 2007;45(2):272–4.
66.
go back to reference Forsythe E, Beales PL. Bardet-Biedl Syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews((R)). Seattle (WA)1993. Forsythe E, Beales PL. Bardet-Biedl Syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews((R)). Seattle (WA)1993.
67.
go back to reference Forsythe E, Kenny J, Bacchelli C, Beales PL. Managing Bardet-Biedl syndrome-now and in the future. Front Pediatr. 2018;6:23.PubMedPubMedCentral Forsythe E, Kenny J, Bacchelli C, Beales PL. Managing Bardet-Biedl syndrome-now and in the future. Front Pediatr. 2018;6:23.PubMedPubMedCentral
68.
go back to reference 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(1):121–5.PubMed 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(1):121–5.PubMed
69.
go back to reference Uday S, Ali M, Azam W, Barrett T. Effect of dietetic management on weight in children with Bardet-Biedl syndrome. Endocrine Abstracts 2015. Uday S, Ali M, Azam W, Barrett T. Effect of dietetic management on weight in children with Bardet-Biedl syndrome. Endocrine Abstracts 2015.
70.
go back to reference •• Boscolo M, Fery F, Cnop M. Beneficial outcomes of sleeve gastrectomy in a morbidly obese patient with Bardet-Biedl Syndrome. J Endocr Soc. 2017;1(4):317–22. One patient with BBS, good response to SG (32.6% total weight loss after 3 years).PubMedPubMedCentral •• Boscolo M, Fery F, Cnop M. Beneficial outcomes of sleeve gastrectomy in a morbidly obese patient with Bardet-Biedl Syndrome. J Endocr Soc. 2017;1(4):317–22. One patient with BBS, good response to SG (32.6% total weight loss after 3 years).PubMedPubMedCentral
71.
go back to reference •• Ates U, Ergun E, Gollu G, Sozduyar S, Can OS, Yagmurlu A. Pediatric laparoscopic sleeve gastrectomy in Turkey: short-term results. Pediatr Int. 2018;60(5):461–6. One patient with BBS, good response to SG (28% total weight loss after 1 year).PubMed •• Ates U, Ergun E, Gollu G, Sozduyar S, Can OS, Yagmurlu A. Pediatric laparoscopic sleeve gastrectomy in Turkey: short-term results. Pediatr Int. 2018;60(5):461–6. One patient with BBS, good response to SG (28% total weight loss after 1 year).PubMed
72.
go back to reference Albright F, Forbes AP, Henneman PH. Pseudo-pseudohypoparathyroidism. Trans Assoc Am Phys. 1952;65:337–50.PubMed Albright F, Forbes AP, Henneman PH. Pseudo-pseudohypoparathyroidism. Trans Assoc Am Phys. 1952;65:337–50.PubMed
73.
go back to reference Patten JL, Johns DR, Valle D, Eil C, Gruppuso PA, Steele G, et al. Mutation in the gene encoding the stimulatory G protein of adenylate cyclase in Albright’s hereditary osteodystrophy. N Engl J Med. 1990;322(20):1412–9. Patten JL, Johns DR, Valle D, Eil C, Gruppuso PA, Steele G, et al. Mutation in the gene encoding the stimulatory G protein of adenylate cyclase in Albright’s hereditary osteodystrophy. N Engl J Med. 1990;322(20):1412–9.
74.
go back to reference Weinstein LS, Yu S, Warner DR, Liu J. Endocrine manifestations of stimulatory G protein alpha-subunit mutations and the role of genomic imprinting. Endocr Rev. 2001;22(5):675–705.PubMed Weinstein LS, Yu S, Warner DR, Liu J. Endocrine manifestations of stimulatory G protein alpha-subunit mutations and the role of genomic imprinting. Endocr Rev. 2001;22(5):675–705.PubMed
75.
go back to reference Ferrario C, Gastaldi G, Portmann L, Giusti V. Bariatric surgery in an obese patient with Albright hereditary osteodystrophy: a case report. J Med Case Rep. 2013;7:111.PubMedPubMedCentral Ferrario C, Gastaldi G, Portmann L, Giusti V. Bariatric surgery in an obese patient with Albright hereditary osteodystrophy: a case report. J Med Case Rep. 2013;7:111.PubMedPubMedCentral
76.
go back to reference Beamish AJ, Reinehr T. Should bariatric surgery be performed in adolescents? Eur J Endocrinol. 2017;176(4):D1–d15.PubMed Beamish AJ, Reinehr T. Should bariatric surgery be performed in adolescents? Eur J Endocrinol. 2017;176(4):D1–d15.PubMed
77.
go back to reference Kumar S, Kelly AS. Review of childhood obesity: from epidemiology, etiology, and comorbidities to clinical assessment and treatment. Mayo Clin Proc. 2017;92(2):251–65.PubMed Kumar S, Kelly AS. Review of childhood obesity: from epidemiology, etiology, and comorbidities to clinical assessment and treatment. Mayo Clin Proc. 2017;92(2):251–65.PubMed
Metadata
Title
Bariatric Surgery for Monogenic Non-syndromic and Syndromic Obesity Disorders
Authors
Niels Vos
Sabrina M. Oussaada
Mellody I. Cooiman
Lotte Kleinendorst
Kasper W. ter Horst
Eric J. Hazebroek
Johannes A. Romijn
Mireille J. Serlie
Marcel M. A. M. Mannens
Mieke M. van Haelst
Publication date
01-09-2020
Publisher
Springer US
Published in
Current Diabetes Reports / Issue 9/2020
Print ISSN: 1534-4827
Electronic ISSN: 1539-0829
DOI
https://doi.org/10.1007/s11892-020-01327-7

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