PRADER-WILLI SYNDROME: NEW OPPORTUNITIES IN TREATMENT OF ADOLESCENTS AND ADULTS

Authors

DOI:

https://doi.org/10.21856/j-PEP.2022.1.06

Keywords:

Prader-Willi syndrome, obesity, hyperphagia, diabetes mellitus, bariatric surgery

Abstract

Introduction. Prader–Willi syndrome (PWS) is a genetic disease characterized by development of severe obesity in combination with a number of comorbid conditions. Known therapeutic treatments for obesity in Prader-Willi syndrome have a moderately positive effect, and surgical weight correction methods are associated with a high risk of post-operative complications.

Research objective: analyze the results of the bariatric surgical intervention by the laparoscopic distal gastric bypass with one anastomosis and the subsequent plastic correction of skin-fat flaps and excess tissues in the patient with Prader-Willi syndrome, complicated by morbid super obesity (BMI = 73 kg/m²).

Case report: One patient with a genetic disease — Prader–Willy syndrome, which was admitted to the clinic with morbid superobesity (BMI = 73 kg/m2) and its complications, is provided with a laparoscopic distal bypass with one anastomosis and a subsequent plastic correction. After the operation, the weight of the patient decreased by 80 kg, glucose and AD levels were normalized, at night the patient sleeps without the use of CPAP-machine, moving freely without dyspnea.

Discussion. Evidence for bariatric surgery in PWS patients is assumed to be a body mass index (BMI) ≥ 35 kg/m2 in combination with severe associated comorbid conditions (obstructive apnea syndrome, Pickwick syndrome, type 2 diabetes mellitus, severe form of non-alcoholic steatohepatosis, the diseases of the musculoskeletal system) or BMI ≥ 40 kg/m2 in combination with lighter associated pathologies. The search for an optimal treatment of obesity in PWS patients continues.

Conclusion. A multidisciplinary approach to the treatment of PWS patients is important, with the mandatory involvement of endocrinologists, geneticists, general practitioners and bariatric surgeons. The conduct of modern bariatric operations in combination with medicinal treatment makes it possible to achieve a steady reduction in body mass and positive medical and social rehabilitation in patients suffering from morbid obesity.

References

Aver'yanov AP. Mezhdunar Endokrinol Zhurn 2009;4(22): 90-98.

Pylypenko VN. Ukr Zhurn Dityachoї Endokrinologіi 2012;2: 29-32.

Pylypenko VN. Zbіrnik naukovih prac' spіvrobіtnikіv NMAPO іmenі P. L. Shupika 2010;19(3): 261-268.

Burman P, Ritzen EM, Lindgren AC. Endocrinol Rev 2001;22: 787-799.

Griggs JL, Su XQ, Mathai ML. Am J Med Sci 2015;7(11): 509-516. https://doi.org/10.4103/1947-2714.170611.

Alqahtani AR, Elahmedi MO, Al Qahtani AR, et al. Surg Obes Relat Dis 2016;12(1): 100-110. https://doi.org/10.1016/j.soard.2015.07.014.

Musella M, Milone M, Leongito M, et al. J Invest Surg 2014;27(2): 102-105. https://doi.org/10.3109/08941939.2013.832824

Gibbons E, Casey AF, Brewster KZ. Disabil Health J 2017;10(1): 3-10. https://doi.org/10.1016/j.dhjo.2016.09.005.

Swaab DF Acta Paediat 1997; 423: 50-54.

Walley AJ, Blakemore AI, Froguel P. Hum Mol Genet 2006;15(2): R124-130. https://doi.org/10.1093/hmg/ddl215.

Williams K, Scheimann A, Sutton V, et al. J Clin Sleep Med 2008;4(2): 111-118.

Peterli R, Wölnerhanssen BK, Peters T, et al. JAMA 2018;319(3): 255-265. https://doi.org/10.1001/jama.2017.20897.

Michalik M, Frask A, Lech P, et al. Wideochir Inne Tech Maloinwazyjne 2015;10(2): 324-327. https://doi.org/10.5114/wiitm.2015.49669

Crino A, Fintitni D, Bocchini S, Grugni G. Diab Metab Syndr Obes: Targets Ther 2018;11: 579-593. https://doi.org/10.2147/DMSO.S141352.

Iossa A, De Peppo F, Caccamo R, et al. Eat Weight Disord 2018;23(4): 479-486. https://doi.org/10.1007/s40519-016-0348-7.

Nobili V, Vajro P, Dezsofi A, et al. J Pediatr Gastroenterol Nutr 2015;60(4): 550-561. https://doi.org/10.1097/MPG.0000000000000715.

Shoar S, Mahmoudzadeh H, Naderan M, et al. Obes Surg 2017;27(12): 3110-3117. https://doi.org/10.1007/s11695-017-2738-y.

Cazzo E, Gestic MA, Utrini MP, et al. Sao Paulo Med J 2018;136(1): 84-88.

Fong AK, Wong SK, Lam CC, Ng EK. Obes Surg 2012;22(11): 1742-1745.

Scheimann AO, Butler MG, Gourash L, et al. J Pediatr Gastroenterol Nutr 2008;46(1): 80-83.

Busetto L, Dicker D, Azran C, et al. Obes Surg 2018;28(7): 2117-2121. https://doi.org/10.1007/s11695-018-3283-z.

Scheimann AO, Butler MG, Gourash L, et al. J Pediatr Gastroenterol Nutr 2008;46(1): 80-83.

Tripodi M, Casertano A, Peluso M, et al. Obes Surg 2020;30(11): 4602-4604. https://doi.org/10.1007/s11695-020-04708-9.

Moura D, Oliveira J, de Moura EG, et al. Surg Obes Relat Dis 2016;12(2): 420-429. https://doi.org/10.1016/j.soard.2015.10.077.

De Peppo F, Di Giorgio G, Germani M, et al. Obes Surg 2008;18(11): 1443–1449. https://doi.org/10.1007/s11695-008-9509-8.

Alqahtani AR, Elahmedi M, Alqahtani YA. Semin Pediatr Surg 2014;23(1): 37-42. https://doi.org/10.1053/j.sempedsurg.2013.10.013.

Liu SY, Wong SK, Lam CC, Ng EK. Pediatr Obes 2020;15(1): e12575. https://doi.org/10.1111/ijpo.12575

Vlasov VV. Vvedenie v dokazatel'nuju medicinu, Moskva, 2001: 392 p.

Pilipenko VM. Ukr Zhurn Dityachoї Endokrinologіi 2017;3-4(23-24): 13-21.

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Published

2022-03-15

How to Cite

Лаврик, А., Пилипенко, В., Галич, С., Згонник, А., Дмитренко, О., Дабіжа, О., & Панків, А. (2022). PRADER-WILLI SYNDROME: NEW OPPORTUNITIES IN TREATMENT OF ADOLESCENTS AND ADULTS. Problems of Endocrine Pathology, 79(1), 43-50. https://doi.org/10.21856/j-PEP.2022.1.06

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Section

CLINICAL ENDOCRINOLOGY