PRADER WİLLİ SENDROMU OLAN BİREYLERDE OBEZİTE KONTROLÜNDE EGZERSİZİN ROLÜ

Yıl 2021, Cilt: 7 Sayı: 1, 16 - 29, 30.04.2021

Ayşegül Atlı

Öz

Prader willi sendromu (PWS); beslenme bozuklukları, osteoporoz, fiziksel inaktivite, hipotoni, obezite, mental retardasyon, skolyoz ve gelişimsel gerilikler ile bireyi olumsuz etkileyen genetik bir bozukluktur Sendrom ilk olarak 1956'da Dr. Prader, Labhart ve Willi tarafından tanımlanmıştır. Paternal olarak türetilen kromozom 15'in uzun kolunda 11-13 segmentinin olmamasından kaynaklanır. Birbirini tetikleyen hipotoni, fiziksel inaktivite ve obezite için uygun egzersiz reçetelerine yönelik çalışmalar yapılmaktadır. Bebeklik döneminde zayıf emme beslenme eksikliğine neden olurken ilerleyen dönemlerde aşırı yeme davranışı obezite neden olur. Her iki durumda da bireyin gelişimine olumsuz etki eder. PWS’ye bağlı obezitenin tipik özelliği normal obez olan bireylere göre gövde ve bacaklarda daha fazla yağ kütlesi artışı olmasıdır. Cinsiyet farklılığına bakıldığında erkek bireylerde kadınlardan daha fazla yağ kütlesi artışına neden olur. Yiyecek isteme davranışı, istifleme yapmak veya yiyecek aramak, yiyecek almak için yiyecek veya para çalmak yaygındır. Hipotoni ve obezite arasında bağlantı vardır. Azalmış fiziksel aktivite ve yeme davranışındaki artma obeziteye zemin hazırlar. Hem Aerobik hem de anaerobik aktiviteler ile enerji tüketimini artırarak kilo kaybını sağlamak ve korumak için çok önemlidir. PWS’li bireyde ince ve kaba motor becerilerin geliştirilmesi, solunum kapasitesinin artırılması, osteoporozun önlenmesi orta ve hafif şiddetdeki egzersizler ile sağlanabilir. Hem bireysel eğitimin hem de grup içi egzersizlerin sosyal yönden bireyin gelişime etkisi büyüktür. Bu çalışmanın amacı genetik bir bozukluk olan PWS‘li bireylerin gelişimde obezite için egzersizin önemini vurgulamaktır.

Anahtar Kelimeler

PWS, egzersiz, obezite

Destekleyen Kurum

destekleyen kurum yoktur

Kaynakça

• 1. Butler MG, Rogan PK. Atypical Clinical Findings in Prader-Willi Syndrome Patients: Analysis of Survey Data. Prader Willi Perspect. 1996;4(3):3-6.
• 2. Drissi Touzani K, Marrakchi M, Chraibi F, Abdellaoui M, Benatiya Andaloussi I, Tahiri H. [The syndrome of Prader-Willi (A case report)]. J Fr Ophtalmol. 2018;41(4):e165-e6.
• 3. Theodoro MF, Talebizadeh Z, Butler MG. Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity. Obesity (Silver Spring, Md). 2006;14(10):1685-90.
• 4. WHO Child Growth Standards based on length/height, weight and age. Acta Paediatr Suppl. 2006;450:76-85.
• 5. Lam MY, Rubin DA, White E, Duran AT, Rose DJ. Test-retest reliability of the Bruininks-Oseretsky Test of Motor Proficiency, Second Edition for youth with Prader-Willi syndrome. Ann Phys Rehabil Med. 2018;61(5):355-7.
• 6. Yang A, Kim J, Cho SY, Jin DK. Prevalence and risk factors for type 2 diabetes mellitus with Prader-Willi syndrome: a single center experience. Orphanet journal of rare diseases. 2017;12(1):146.
• 7. Vijayakumar P, Narayanasamy A, Vellingiri B. Excessive carbohydrate consumption and body mass index: the risk factors for type 2 diabetes mellitus in patients with Prader-Willi syndrome in Tamil Nadu population. The Journal of Basic and Applied Zoology. 2018;79(1):54.
• 8. Mackenzie ML, Triador L, Gill JK, Pakseresht M, Mager D, Field CJ, et al. Dietary intake in youth with prader-willi syndrome. Am J Med Genet A. 2018.
• 9. Woods SG, Knehans A, Arnold S, Dionne C, Hoffman L, Turner P, et al. The associations between diet and physical activity with body composition and walking a timed distance in adults with Prader-Willi syndrome. Food Nutr Res. 2018;62.
• 10. Key AP, Dykens EM. Eye Tracking as a Marker of Hyperphagia in Prader-Willi Syndrome. Dev Neuropsychol. 2018;43(2):152-61.
• 11. Duran AT, Wilson KS, Castner DM, Tucker JM, Rubin DA. Association between physical activity and bone in children with Prader-Willi syndrome. J Pediatr Endocrinol Metab. 2016;29(7):819-26.
• 12. Deal CL, Tony M, Hoybye C, Allen DB, Tauber M, Christiansen JS. GrowthHormone Research Society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013;98(6):E1072-87.
• 13. Irizarry KA, Miller M, Freemark M, Haqq AM. Prader Willi Syndrome: Genetics, Metabolomics, Hormonal Function, and New Approaches to Therapy. Advances in pediatrics. 2016;63(1):47-77.
• 14. Fridman C, Kok F, Koiffmann CP. [Hypotonic infants and the Prader-Willi Syndrome]. J Pediatr (Rio J). 2000;76(3):246-50.
• 15. Krasinska A, Skowronska B. Prader-Willi Syndrome - nutritional management in children, adolescents and adults. Pediatr Endocrinol Diabetes Metab. 2017;23(2):101-6.
• 16. Amaro AS, Teixeira MC, de Mesquita ML, Rodrigues GM, Rubin DA, Carreiro LR. Physiological adaptation after a 12-week physical activity program for patients with Prader-Willi syndrome: two case reports. Journal of medical case reports. 2016;10(1):181.
• 17. Abel F, Tan HL, Negro V, Bridges N, Carlisle T, Chan E, et al. Hypoventilation disproportionate to OSAS severity in children with Prader-Willi syndrome. Arch Dis Child. 2018.
• 18. Viardot A, Purtell L, Nguyen TV, Campbell LV. Relative Contributions of Lean and Fat Mass to Bone Mineral Density: Insight From Prader-Willi Syndrome. Front Endocrinol (Lausanne). 2018;9:480.
• 19. Oore J, Connell B, Yaszay B, Samdani A, Hilaire TS, Flynn T, et al. Growth Friendly Surgery and Serial Cast Correction in the Treatment of Early-onset Scoliosis for Patients With Prader-Willi Syndrome. J Pediatr Orthop. 2018.
• 20. Rubin DA, Clark SJ, Haqq AM, Castner DM, Ng J, Judelson DA. Hormonal and Metabolic Responses to a Single Bout of Resistance Exercise in Prader-Willi Syndrome
Hormone Research in Paediatrics. 2017;87(3):153-61.
• 20. Fuller JT, Hartland MC, Maloney LT, Davison K. Therapeutic effects of aerobic and resistance exercises for cancer survivors: a systematic review of meta-analyses of clinical trials. Br J Sports Med. 2018;52(20):1311.
• 21. Amaro AS, Teixeira MC, de Mesquita ML, Rodrigues GM, Rubin DA, Carreiro LR. Physiological adaptation after a 12-week physical activity program for patients with Prader-Willi syndrome: two case reports. Journal of medical case reports. 2016;10(1):181.
• 22. Eiholzer U, Nordmann Y, l'Allemand D, Schlumpf M, Schmid S, Kromeyer-Hauschild K. Improving body composition and physical activity in Prader-Willi Syndrome. J Pediatr. 2003;142(1):73-8.
• 23. Shields N, Westle A, Bennell KL, Taylor NF. Physiotherapists perceived developing positive rapport facilitates participation in exercise among people with Prader-Willi Syndrome: a qualitative study. Disabil Rehabil. 2019:1-6.
• 24. Vismara L, Cimolin V, Grugni G, Galli M, Parisio C, Sibilia O, et al. Effectiveness of a 6-month home-based training program in Prader-Willi patients. Res Dev Disabil. 2010;31(6):1373-9.
• 25. Viardot A, Purtell L, Nguyen TV, Campbell LV. Relative Contributions of Lean and Fat Mass to Bone Mineral Density: Insight From Prader-Willi Syndrome. Front Endocrinol (Lausanne). 2018;9:480.
• 26. Duran AT, Wilson KS, Castner DM, Tucker JM, Rubin DA. Association between physical activity and bone in children with Prader-Willi syndrome. J Pediatr Endocrinol Metab. 2016;29(7):819-26.
• 27. Abel F, Tan HL, Negro V, Bridges N, Carlisle T, Chan E, et al. Hypoventilation disproportionate to OSAS severity in children with Prader-Willi syndrome. Arch Dis Child. 2018.
• 28. Weiss HR, Moramarco MM, Borysov M, Ng SY, Lee SG, Nan X, et al. Postural Rehabilitation for Adolescent Idiopathic Scoliosis during Growth. Asian Spine J. 2016;10(3):570-81.
• 29. Oore J, Connell B, Yaszay B, Samdani A, Hilaire TS, Flynn T, et al. Growth Friendly Surgery and Serial Cast Correction in the Treatment of Early-onset Scoliosis for Patients With Prader-Willi Syndrome. J Pediatr Orthop. 2018.
• 30. Garber CE, Blissmer B, Deschenes MR, Franklin BA, Lamonte MJ, Lee IM, et al. American College of Sports Medicine position stand. Quantity and quality of exercise for developing and maintaining cardiorespiratory, musculoskeletal, and neuromotor fitness in apparently healthy adults: guidance for prescribing exercise. Med Sci Sports Exerc. 2011;43(7):1334-59.
• 31. Rubin DA, Clark SJ, Haqq AM, Castner DM, Ng J, Judelson DA. Hormonal and Metabolic Responses to a Single Bout of Resistance Exercise in Prader-Willi Syndrome
Hormone Research in Paediatrics. 2017;87(3):153-61.
• 32. Hirsch HJ, Gross-Tsur V, Sabag Y, Nice S, Genstil L, Benarroch F, et al. Myokine levels after resistance exercise in young adults with Prader-Willi syndrome (PWS). Am J Med Genet A. 2020;182(1):115-21.
• 33. Joung HJ, Lim IS. Changes in body composition, blood lipid profile, and growth factor hormone in a patient with Prader-willi syndrome during 24 weeks of complex exercise: a single case study. J Exerc Nutrition Biochem. 2018;22(1):35-40.
• 34. Maas AP, Didden R, Bouts L, Smits MG, Curfs LM. Scatter plot analysis of excessive daytime sleepiness and severe disruptive behavior in adults with Prader-Willi syndrome: a pilot study. Res Dev Disabil. 2009;30(3):529-37.
• 35. Krasinska A, Skowronska B. Prader-Willi Syndrome - nutritional management in children, adolescents and adults. Pediatr Endocrinol Diabetes Metab. 2017;23(2):101-6.