title: Physiological adaptation after a 12-week physical activity program for patients with Prader-Willi syndrome: two case reports.
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.
  
  
  METHODS
  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/m(2). 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/m(2). 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.
authors.affiliation: Programa de Pós-graduação em Distúrbios do Desenvolvimento, Universidade Presbiteriana Mackenzie, Rua da Consolação, 930, São Paulo, SP, CEP - 01302-907, Brasil. aleslowetzky@gmail.com.
authors.forename: Alexandre Slowetzky
authors.initials: AS
authors.lastname: Amaro
authors.affiliation: Programa de Pós-graduação em Distúrbios do Desenvolvimento, Universidade Presbiteriana Mackenzie, Rua da Consolação, 930, São Paulo, SP, CEP - 01302-907, Brasil.
authors.forename: Maria Cristina Triguero Veloz
authors.initials: MC
authors.lastname: Teixeira
authors.affiliation: Programa de Pós-graduação em Distúrbios do Desenvolvimento, Universidade Presbiteriana Mackenzie, Rua da Consolação, 930, São Paulo, SP, CEP - 01302-907, Brasil.
authors.forename: Maria Luiza Guedes
authors.initials: ML
authors.lastname: de Mesquita
authors.affiliation: Escola Superior de Educação Física de Jundiaí, Rua Dr. Rodrigo Soares de Oliveira, s/n - Anhangabau, Jundiaí, SP, CEP - 13208-120, Brasil.|Programa de Pós-Graduação Stricto Sensu em Ciências do Envelhecimento, Universidade São Judas Tadeu, Rua Taquari, 546, São Paulo, SP, CEP 03166-000, Brasil.
authors.forename: Graciele Massoli
authors.initials: GM
authors.lastname: Rodrigues
authors.affiliation: Department of Kinesiology, California State University Fullerton, 800 N. State College Blvd, Fullerton, CA, 92834, USA.
authors.forename: Daniela Andrea
authors.initials: DA
authors.lastname: Rubin
authors.affiliation: Programa de Pós-graduação em Distúrbios do Desenvolvimento, Universidade Presbiteriana Mackenzie, Rua da Consolação, 930, São Paulo, SP, CEP - 01302-907, Brasil.
authors.forename: Luiz Renato Rodrigues
authors.initials: LR
authors.lastname: Carreiro
mesh_terms: D000222:Adaptation, Physiological / Q000502:physiology*; D000293:Adolescent; D002648:Child; D004032:Diet; D037001:Directive Counseling; D015444:Exercise; D005260:Female; D007722:Health Knowledge, Attitudes, Practice; D006801:Humans; D008297:Male; D009042:Motivation; D009752:Nutritional Status; D009765:Obesity / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control*; D010349:Patient Compliance; D011218:Prader-Willi Syndrome / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D015397:Program Evaluation; D012891:Sleep Apnea Syndromes / Q000150:complications / Q000628:therapy; D016896:Treatment Outcome; D015431:Weight Loss
keywords: Physical activity; Physical fitness and body composition; Prader–Willi syndrome