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Roux-en-Y Gastric Bypass in an Adolescent Patient with Bardet–Biedl Syndrome, a Monogenic Obesity Disorder

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Abstract

Bardet–Biedl syndrome (BBS) is a rare genetic disorder characterized by a wide range of phenotypic variability and associated with the development of life-threatening obesity. Birth weight tends to be normal, but rapid weight gain begins after the first year, probably due to polyphagia rather than abnormalities in energy metabolism. A morbidly obese 16-year-old male patient with BBS was referred to our institution, after nonsurgical methods of weight control had failed, for surgical treatment of his obesity. His preoperative body mass index (BMI) was 52.28 kg/m2 (height, 1.84 m; weight, 177 kg) and was above the 99th centile for age and gender. The patient underwent laparoscopic Roux-en-Y gastric bypass (RYGBP). The postoperative period was uneventful. Three and a half years after the operation, the patient's weight has decreased to 118 kg (BMI, 34.85 kg/m2), while significant improvement in his hypertension, hyperuricemia, and mobility has been noted. In our BBS patient, RYGBP proved to be safe and effective; nevertheless, longer follow-up is required to evaluate the weight loss durability and to assess the lasting beneficial effect of surgical intervention on genetically determined co-morbidities.

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References

  1. Kiess W, Reich A, Müller G, et al. Clinical aspects of obesity in childhood and adolescence—diagnosis, treatment and prevention. Int J Obes Relat Metab Disord. 2001;25(Suppl 1):S75–9.

    Article  PubMed  Google Scholar 

  2. Marinari GM, Camerini G, Novelli GB, et al. Outcome of biliopancreatic diversion in subjects with Prader–Willi syndrome. Obes Surg. 2001;11:491–5.

    Article  CAS  PubMed  Google Scholar 

  3. de Almeida MQ, Cercato C, Rascovski A, et al. Results of biliopancreatic diversion in two patients with Prader–Willi syndrome. Obes Surg. 2005;15:901–4.

    Article  PubMed  Google Scholar 

  4. Till H, Blüher S, Hirsch W, et al. Efficacy of laparoscopic sleeve gastrectomy (LSG) as a stand-alone technique for children with morbid obesity. Obes Surg. 2008;18:1047–9.

    Article  CAS  PubMed  Google Scholar 

  5. Scheimann AO, Butler MG, Gourash L, et al. Critical analysis of bariatric procedures in Prader–Willi syndrome. J Pediatr Gastroenterol Nutr. 2008;46:80–3.

    Article  CAS  PubMed  Google Scholar 

  6. Beales PL, Elcioglu N, Woolf AS, et al. New criteria for improved diagnosis of Bardet–Biedl syndrome: results of a population survey. J Med Genet. 1999;36:437–46.

    CAS  PubMed  Google Scholar 

  7. Tobin JL, Beales PL. Bardet–Biedl syndrome: beyond the cilium. Pediatr Nephrol. 2007;22:926–36.

    Article  PubMed  Google Scholar 

  8. Stefan M, Nicholls RD. What have rare genetic syndromes taught us about the pathophysiology of the common forms of obesity? Curr Diab Rep. 2004;4:143–50.

    Article  PubMed  Google Scholar 

  9. Croft JB, Morrell D, Chase CL, et al. Obesity in heterozygous carriers of the gene for the Bardet–Biedl syndrome. Am J Med Genet. 1995;55:12–5.

    Article  CAS  PubMed  Google Scholar 

  10. Grace C, Beales P, Summerbell C, et al. Energy metabolism in Bardet–Biedl syndrome. Int J Obes Relat Metab Disord. 2003;27:1319–24.

    Article  CAS  PubMed  Google Scholar 

  11. Moore SJ, Green JS, Fan Y, et al. Clinical and genetic epidemiology of Bardet-Biedl syndrome in Newfoundland: a 22-year prospective, population-based, cohort study. Am J Med Genet. 2005;132:352–60.

    Article  PubMed  Google Scholar 

  12. Blacque OE, Leroux MR. Bardet-Biedl syndrome: an emerging pathomechanism of intracellular transport. Cell Mol Life Sci. 2006;63:2145–61.

    Article  CAS  PubMed  Google Scholar 

  13. Marshall WF, Nonaka S. Cilia: tuning in to the cell's antenna. Curr Biol. 2006;16:R604–14.

    Article  CAS  PubMed  Google Scholar 

  14. Katsanis N, Lupski JR, Beales PL. Exploring the molecular basis of Bardet–Biedl syndrome. Hum Mol Genet. 2001;10:2293–9.

    Article  CAS  PubMed  Google Scholar 

  15. Rahmouni K, Fath MA, Seo S, et al. Leptin resistance contributes to obesity and hypertension in mouse models of Bardet–Biedl syndrome. J Clin Invest. 2008;118:1458–67.

    Article  CAS  PubMed  Google Scholar 

  16. Seo S, Guo DF, Bugge K, et al. Requirement of Bardet–Biedl syndrome proteins for leptin receptor signaling. Hum Mol Genet. 2009;18:1323–31.

    Article  CAS  PubMed  Google Scholar 

  17. Korenkov M, Kohlschmidt N, Zechner U, et al. Adipositaschirurgie bei monogenetisch bedingter Adipositas. Zentralbl Chir. 2007;132:256–9.

    Article  CAS  PubMed  Google Scholar 

  18. GarciaVF DeMaria EJ. Adolescent bariatric surgery: treatment delayed, treatment denied, a crisis invited. Obes Surg. 2006;16:1–4.

    Article  Google Scholar 

  19. Fried M. Bariatric surgery in paediatrics—when and how? Int J Pediatr Obes. 2008;3(suppl 2):9–15.

    Google Scholar 

  20. Silberhumer GR, Miller K, Kriwanek S, et al. Laparoscopic adjustable gastric banding in adolescents: the Austrian experience. Obes Surg. 2006;16:1062–67.

    Article  PubMed  Google Scholar 

  21. Barnett SJ, Stanley C, Hanlon M, et al. Long-term follow-up and the role of surgery in adolescents with morbid obesity. Surg Obes Relat Dis. 2005;1:394–8.

    Article  PubMed  Google Scholar 

  22. Papadia FS, Adami GF, Marinari GM, et al. Bariatric surgery in adolescents: a long-term follow-up study. Surg Obes Relat Dis. 2007;3:465–8.

    Article  PubMed  Google Scholar 

  23. Treadwell JR, Sun F, Schoelles K. Systematic review and meta-analysis of bariatric surgery for pediatric obesity. Ann Surg. 2008;248:763–76.

    Article  PubMed  Google Scholar 

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Correspondence to Rudolf A. Weiner.

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The authors disclose no commercial interest in the subject of study

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Daskalakis, M., Till, H., Kiess, W. et al. Roux-en-Y Gastric Bypass in an Adolescent Patient with Bardet–Biedl Syndrome, a Monogenic Obesity Disorder. OBES SURG 20, 121–125 (2010). https://doi.org/10.1007/s11695-009-9915-6

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  • DOI: https://doi.org/10.1007/s11695-009-9915-6

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