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International Journal of Nutrology
Case Report
DOI: 10.54448/ijn23214
Submitted: 05-04-2023
Published: 05-04-2023

Management of weight loss in a patient with Prader-Willi syndrome with satietogenics: a case report

Adriano Jorge Hospital Foundation – FHAJ, Internal Medicine, ManausAmazonas, Brazil
Adriano Jorge Hospital Foundation – FHAJ, Internal Medicine, ManausAmazonas, Brazil
University of the State of Amazonas, Medicine Course, Amazonas, Brazil
Nutrology Service of the General Surgery Unit, University Hospital Complex Professor Edgard Santos (HUPES), Federal University of Bahia (UFBA), Salvador, Bahia, Brazil
University of the State of Amazonas, Medicine Course, Amazonas, Brazil / Adriano Jorge Hospital Foundation – FHAJ and University of the State of Amazonas, nutrology, Manaus, Amazonas, Brazil

Abstract

Objective: To report the evolution and therapeutic approach in a patient with PraderWilli Syndrome (PWS) associated with literature data assessment with a focus on the pharmacological treatment of obesity in patients with this pathology, since there are currently not many studies that contribute or name a strategy with the use of satietogenics. Methods: The present study was elaborated according to the rules of CARE case report. Due to the cognitive impairment present in the syndrome, the patient’s mother was first contacted about the writing of this paper and was given consent through the signing of the Free and Informed Assent Term for this paper. The patient was treated at a hospital-adjacent Nutrology outpatient clinic in 2019. Case Description: YSP, 20 years old, diagnosed with PWS and Grade III obesity (initial BMI of 55.4kg/m², 140kg) attended the Nutrology outpatient clinic of Adriano Jorge Hospital Foundation – FHAJ in 2019 under referral from a third party, and his companion (relative) reporting hyperphagia and poor school performance, initially without a nutrition plan, during follow-up, nutritional guidance and multi-professional care have been initiated. Results: During the follow-up of the years 2019-2021, resulting from the SARS-CoV-2 pandemic in Brazil and the consequent confinement, the patient presented worsening anxiety and hyperphagia, evolving with increased binge eating and discontinuation of physical activities and diet adherence. During the follow-up in the same period, there were 03 returns denoting weight maintenance, later (2021) the use of on-label (Sibutramine), off-label (Fluoxetine), and Metformin medications were associated with the therapeutic regimen for obesity, due to evidence of insulin resistance. Final considerations: The patient evolved with progressive weight loss and better adherence to the food plan and physical activities.

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References

  1. Alves C, Franco RR. Prader-Willi syndrome: endocrine manifestations and management. Arch. Endocrinol. Metab. 2020;64(3):223-34. doi: 10.20945/2359-3997000000248.
  2. Damiani D. Growth hormone usage in Prader-Willi syndrome. Arq Bras Endocrinol Metab 2008;52(5):833-8.
  3. Muscogiuri G, Barrea L, Faggiano F, et al. Obesity in Prader-Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches. J Endocrinol Invest. 2021;44(10):2057-2070.doi:10.1007/s40618-021-01574- 9.
  4. Einfeld SL, Kavanagh SJ, Smith A, Evans EJ, Tonge BJ, Taffe J. Mortality in Prader-Willi syndrome. Am J Ment Retard. 2006;111(3):193-198. doi:10.1352/0895-8017(2006)111[193: MIPS]2.0.CO;2.
  5. Carvalho, D. F., Cercato, C., Almeida, M. Q., Mancini, M. C., & Halpern, A. (2007). Therapeutic approach to obesity in Prader–Willi syndrome. Brazilian Archives of Endocrinology and Metabology, 51(6), 913–919.
  6. Angelo DelParigi MT, Mark L. Heiman, Arline D. Salbe, Barbora Vozarova, Susan M. Sell, Joy C. Bunt, P. Antonio Tataranni, High Circulating Ghrelin: A Potential Cause for Hyperphagia and Obesity in Prader-Willi Syndrome, The Journal of Clinical Endocrinology & Metabolism, Volume 87, Issue 12, 1 December 2002, Pages 5461–5464, https://doi.org/10.1210/jc.2002-020871
  7. Vilar L et al. Hypopituitarism - Etiology and Diagnosis. in. Clinical endocrinology / Lucio Vilar. [etal]- 6. ed- Rio de Janeiro: Guanabara Koogan, 2016.
  8. Yerevanian A, Soukas AA. Metformin: Mechanisms in Human Obesity and Weight Loss. Curr Obes Rep. 2019 Jun;8(2):156-164. doi: 10.1007/s13679- 019-00335-3.
  9. Silverthorn, Dee Unglaub. Human Physiology [electronic resource] : an integrated approach / Dee Unglaub Silverthorn; translation Ivana Beatrice Mânica da Cruz. [et al.]- 5. ed. - Porto Alegre: Artmed, 2010.
  10. Miller JL, Lynn CH, Driscoll DC, Goldstone AP, Gold JA, Kimonis V, Dykens E, Butler MG, Shuster JJ, Driscoll DJ. Nutritional phases in Prader-Willi syndrome. Am J Med Genet A. 2011 May;155A(5):1040-9. doi: 10.1002/ajmg.a.33951.
  11. Han JC, Muehlbauer MJ,Cui HN, et al. Lower brain-derived neurotrophic factor in patients with prader- willi syndrome compared to obese and lean control subjects. J Clin Endocrinol Metab 2010; 95:3532.
  12. Zipf WB, O'Dorisio TM, Cataland S, Sotos J. Blunted pancreatic polypeptide responses in children withobesity of Prader-Willi syndrome. J Clin Endocrinol Metab 1981; 52:1264.
  13. Butler MG, Bittel DC, Talebizadeh Z. Plasma peptide YY and ghrelin levels in infants and children with Prader-Willi syndrome. J Pediatr Endocrinol Metab 2004; 17:1177.
  14. de Almeida MQ, Cercato C, Rascovski A, Goldbaum TS, de Figueiredo DA, Matsuda M, Zilberstein B, Villares SM, Halpern A. Results of biliopancreatic diversion in two patients with Prader-Willi syndrome.Obes Surg. 2005 JunJul;15(6):901-4. doi: 10.1381/0960892054222885.
  15. Anthony P, Goldstone E, Louise Thomas AE. Brynes, Gabriela Castroman, RayEdwards, Mohammad A. Ghatei, Gary Frost, Anthony J. Holland, Ashley B. Grossman, Má rtaKorbonits, Stephen R. Bloom, Jimmy D. Bell, Elevated Fasting Plasma Ghrelin in Prader-Willi Syndrome Adults Is Not Solely Explained by Their Reduced Visceral Adiposity and Insulin Resistance, The Journal of Clinical Endocrinology & Metabolism, Volume 89, Issue 4, 1 April 2004, Pages 1718–1726, https://doi.org/10.1210/jc.2003-031118
  16. Charlotte Höybye, Endocrine and metabolic aspects of adult Prader–Willi syndrome with special emphasis on the effect of growth hormone treatment, Growth Hormone & IGF Research, Volume 14, Issue 1, 2004, Pages 1-15, ISSN 1096-6374, https://doi.org/10.1016/j.ghir.2003.09.003.
  17. Haqq AM, Grambow SC, Muehlbauer M, Newgard CB, Svetkey LP, Carrel AL, Yanovski JA, Purnell JQ, Freemark M. Ghrelin concentrations in PraderWilli syndrome (PWS) infants and children: changes during development. Clin Endocrinol (Oxf). 2008 Dec;69(6):911-20. doi: 10.1111/j.1365- 2265.2008.03385.x.
  18. Holsen LM, Zarcone JR, Brooks WM, Butler MG, Thompson TI, Ahluwalia JS, Nollen NL, Savage CR. Neural mechanisms underlying hyperphagia in Prader-Willi syndrome. Obesity (Silver Spring). 2006 Jun;14(6):1028-37. doi: 10.1038/oby.2006.118.
  19. Shapira NA, Lessig MC, He AG, James GA, Driscoll DJ, Liu Y. Satiety dysfunction in Prader-Willi syndrome demonstrated by fMRI. J Neurol Neurosurg Psychiatry. 2005;76:260–2.
  20. Cacciari E, Zucchini S, Carla G, et al. Endocrine function and morphological findings in patients with disorders of the hypothalamo-pituitary area: a study with magnetic resonance. Arch Dis Child. 1990;65:1199–202.
  21. Gutiérrez A, Saracíbar G, Casis L, Echevarría E, Rodríguez VM, Macarulla MT, et al. Effects of fluoxetine administration on neuropeptide Y and orexins in obese Zucker rat hypothalamus. Obesity Research 2002;10(6):532-40.

How to Cite

Pina Neto, J. M., Madureira, L. E. N., Palhares, F. D. P. M., Pimentel, R. F. W., & Maduro, I. P. de N. N. (2023). Management of weight loss in a patient with Prader-Willi syndrome with satietogenics: a case report. International Journal of Nutrology, 16(2). https://doi.org/10.54448/ijn23214