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Published in: Journal of Medical Case Reports 1/2016

Open Access 01-12-2016 | Case report

Physiological adaptation after a 12-week physical activity program for patients with Prader–Willi syndrome: two case reports

Authors: Alexandre Slowetzky Amaro, Maria Cristina Triguero Veloz Teixeira, Maria Luiza Guedes de Mesquita, Graciele Massoli Rodrigues, Daniela Andrea Rubin, Luiz Renato Rodrigues Carreiro

Published in: Journal of Medical Case Reports | Issue 1/2016

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Abstract

Background

Physical activity programs are a powerful tool against several diseases including obesity and their comorbidities. Prader–Willi syndrome is the most common genetic disease associated with obesity, and brings with it behavioral and emotional problems that need complex management. Research into the effect of physical activity programs on Prader–Willi syndrome is limited and it is frequently argued that if a physical activity program is too complex, the participants are more likely to drop out. Therefore, in this study, we assessed the physiological adaptation effect of a physical activity program with increasing complexity and load, in a boy and a girl with Prader–Willi syndrome by assessing changes in lipid profile, body composition, and physical fitness parameters.

Case presentation

Case 1 was an 11-year-old girl, mixed race (brown), with an intelligence quotient of 68, 52.0 % body fat, and a body mass index of 45.3 kg/m2. The Prader–Willi syndrome diagnosis was made when she was 5-years old and was found to be due to an imprinting genomic defect. Case 2 was a 14-year-old boy, mixed race (brown), with an intelligence quotient of 74, 48.8 % body fat, and a body mass index of 37.3 kg/m2. The diagnosis was made when he was 10-years old and was found to be caused by gene deletion. Both participants presented physical characteristics and behavior problems typical of Prader–Willi syndrome. Case 2 presented high blood pressure, high cholesterol and sleep apnea and had to use continuous positive airway pressure to sleep. Both participants were assessed for 12 weeks (three times a week) using a physical activity program designed to improve strength and muscle hypertrophy. The work load was progressively adjusted as necessary and new exercises were added to the program. Prior to the program, the participants’ parents received instructions about managing problem behavior and advice about nutrition.

Conclusions

After physical activity program several health markers assessed by biological tests and parental report had improved in both participants. The participants positively accepted the adaptations made to the physical activity program during the study. More studies are necessary to assess the benefits of physical activity in the Prader–Willi syndrome population.
Literature
1.
go back to reference Vogels A, De Hert M, Descheemaeker MJ, Govers V, Devriendt K, Legius E, et al. Psychotic disorders in Prader–Willi syndrome. Am J Med Genet. 2004;127:238–43.CrossRef Vogels A, De Hert M, Descheemaeker MJ, Govers V, Devriendt K, Legius E, et al. Psychotic disorders in Prader–Willi syndrome. Am J Med Genet. 2004;127:238–43.CrossRef
2.
go back to reference Emerick JE, Vogt KS. Endocrine manifestations and management of Prader-Willi syndrome. Int J Pediatr Endocrinol. 2013;14:2–10. Emerick JE, Vogt KS. Endocrine manifestations and management of Prader-Willi syndrome. Int J Pediatr Endocrinol. 2013;14:2–10.
3.
go back to reference Lukoshe A, Hokken-Koelega AC, van der Lugt A, White T. Reduced cortical complexity in children with Prader-Willi syndrome and its association with cognitive impairment and developmental delay. PLoS One. 2014;9(9):e107320.CrossRefPubMedPubMedCentral Lukoshe A, Hokken-Koelega AC, van der Lugt A, White T. Reduced cortical complexity in children with Prader-Willi syndrome and its association with cognitive impairment and developmental delay. PLoS One. 2014;9(9):e107320.CrossRefPubMedPubMedCentral
4.
go back to reference Whittington J, Holland A. Neurobehavioral phenotype in Prader-Willi syndrome. Am J Med Genet. 2010;154C:438–47.CrossRefPubMed Whittington J, Holland A. Neurobehavioral phenotype in Prader-Willi syndrome. Am J Med Genet. 2010;154C:438–47.CrossRefPubMed
5.
go back to reference Sinnema M, Einfeld SL, Schrander-Stumpel CTRM, Maaskant MA, Boer H, Curfs LMG. Behavioral phenotype in adults with Prader-Willi syndrome. Res Dev Disabil. 2011;32:604–12.CrossRefPubMed Sinnema M, Einfeld SL, Schrander-Stumpel CTRM, Maaskant MA, Boer H, Curfs LMG. Behavioral phenotype in adults with Prader-Willi syndrome. Res Dev Disabil. 2011;32:604–12.CrossRefPubMed
6.
go back to reference Butler MG, Theodoro MF, Bittel DC, Donnelly JE. Energy expenditure and physical activity in Prader–Willi syndrome: comparison with obese subjects. Am J Hum Genet. 2007;143A:449–59. Butler MG, Theodoro MF, Bittel DC, Donnelly JE. Energy expenditure and physical activity in Prader–Willi syndrome: comparison with obese subjects. Am J Hum Genet. 2007;143A:449–59.
7.
go back to reference Capodaglio P, Cimolin V, Vismara L, Grugni G, Parisio C, Sibilia O, et al. Postural adaptations to long-term training in Prader-Willi patients. J Neuroeng Rehabil. 2011;8:26–32.CrossRefPubMedPubMedCentral Capodaglio P, Cimolin V, Vismara L, Grugni G, Parisio C, Sibilia O, et al. Postural adaptations to long-term training in Prader-Willi patients. J Neuroeng Rehabil. 2011;8:26–32.CrossRefPubMedPubMedCentral
8.
go back to reference 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;31:1373–9.CrossRefPubMed 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;31:1373–9.CrossRefPubMed
9.
go back to reference Schlumpf M, Eiholzer U, Gygax M, Schmid S, van der Sluis I, L’Allemand D. A daily comprehensive muscle training programme increases lean mass and spontaneous activity in children with Prader-Willi syndrome after 6 months. J Pediatr Endocrinol Metab. 2006;19:65–74.CrossRefPubMed Schlumpf M, Eiholzer U, Gygax M, Schmid S, van der Sluis I, L’Allemand D. A daily comprehensive muscle training programme increases lean mass and spontaneous activity in children with Prader-Willi syndrome after 6 months. J Pediatr Endocrinol Metab. 2006;19:65–74.CrossRefPubMed
10.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
11.
go back to reference Cheraghi F, Shamsaei F, Mortazavi SZ. The effect of family-centered care on management of blood glucose levels in adolescents with diabetes. Int J Community Based Nurs Midwifery. 2015;3(3):177–86.PubMedPubMedCentral Cheraghi F, Shamsaei F, Mortazavi SZ. The effect of family-centered care on management of blood glucose levels in adolescents with diabetes. Int J Community Based Nurs Midwifery. 2015;3(3):177–86.PubMedPubMedCentral
12.
go back to reference Kovacs R, Baggish AL. Cardiovascular adaptation in athletes. Trends Cardiovasc Med Elsevier. 2016;26(1):46–52.CrossRef Kovacs R, Baggish AL. Cardiovascular adaptation in athletes. Trends Cardiovasc Med Elsevier. 2016;26(1):46–52.CrossRef
13.
go back to reference Baar K. Nutrition and the adaptation to endurance training. Sport Med. 2014;44 Suppl 1:S5–12.CrossRef Baar K. Nutrition and the adaptation to endurance training. Sport Med. 2014;44 Suppl 1:S5–12.CrossRef
14.
go back to reference Weschler D. Wisc III: Escala de inteligência Wechsler para crianças: Manual. 3a Edição. Casa do Psicólogo. São Paulo; 2002. Weschler D. Wisc III: Escala de inteligência Wechsler para crianças: Manual. 3a Edição. Casa do Psicólogo. São Paulo; 2002.
15.
go back to reference Achenback TM, Escorla LA. Manual for the ASEBA School-Age Forms & Profiles. Burlington: Research Center for Children, Youth & Families; 2001. Achenback TM, Escorla LA. Manual for the ASEBA School-Age Forms & Profiles. Burlington: Research Center for Children, Youth & Families; 2001.
16.
go back to reference de Onis M, Onyango A, Borghi E, Siyam A, Blössner M, Lutter C. Worldwide implementation of the WHO Child Growth Standards. Public Health Nutr. 2012;15(09):1603–10.CrossRefPubMed de Onis M, Onyango A, Borghi E, Siyam A, Blössner M, Lutter C. Worldwide implementation of the WHO Child Growth Standards. Public Health Nutr. 2012;15(09):1603–10.CrossRefPubMed
17.
go back to reference Theodoro MF, Talebizadeh Z, Butler MG. Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity. Obesity. 2006;14(10):1685–90.CrossRefPubMed Theodoro MF, Talebizadeh Z, Butler MG. Body composition and fatness patterns in Prader-Willi syndrome: comparison with simple obesity. Obesity. 2006;14(10):1685–90.CrossRefPubMed
18.
go back to reference Marques AC. O Perfil do Estilo de Vida de Pessoas com Síndrome de Down e Normas para Avaliação da Aptidão Física. Porto Alegre: Universidade Federal do Rio Grande do Sul; 2008. Marques AC. O Perfil do Estilo de Vida de Pessoas com Síndrome de Down e Normas para Avaliação da Aptidão Física. Porto Alegre: Universidade Federal do Rio Grande do Sul; 2008.
19.
go back to reference Holm VA, Cassidy SB, Butler MG, Hanchett JM, Louise R, Holm A, et al. Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91(2):398–403.PubMed Holm VA, Cassidy SB, Butler MG, Hanchett JM, Louise R, Holm A, et al. Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91(2):398–403.PubMed
20.
go back to reference Duran A, Castner D, Tucker J, Rubin D. Ambulatory moderate plus vigorous physical activity is associated with greater bone mineral density in the hip in children with Prader-Willi syndrome. J Fed Am Soc Exp Biol. 2014;28(Supplement 1):1166. Duran A, Castner D, Tucker J, Rubin D. Ambulatory moderate plus vigorous physical activity is associated with greater bone mineral density in the hip in children with Prader-Willi syndrome. J Fed Am Soc Exp Biol. 2014;28(Supplement 1):1166.
21.
go back to reference Eiholzer U, Meinhardt U, Petrò R, Witassek F, Gutzwiller F, Gasser T. High-intensity training increases spontaneous physical activity in children: a randomized controlled study. J Pediatr Mosby, Inc. 2010;156(2):242–6.CrossRef Eiholzer U, Meinhardt U, Petrò R, Witassek F, Gutzwiller F, Gasser T. High-intensity training increases spontaneous physical activity in children: a randomized controlled study. J Pediatr Mosby, Inc. 2010;156(2):242–6.CrossRef
Metadata
Title
Physiological adaptation after a 12-week physical activity program for patients with Prader–Willi syndrome: two case reports
Authors
Alexandre Slowetzky Amaro
Maria Cristina Triguero Veloz Teixeira
Maria Luiza Guedes de Mesquita
Graciele Massoli Rodrigues
Daniela Andrea Rubin
Luiz Renato Rodrigues Carreiro
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2016
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-016-0966-8

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