Article

A daily comprehensive muscle training programme increases lean mass and spontaneous activity in children with Prader-Willi syndrome after 6 months.

Institute Growth Puberty Adolescence, Zürich, Switzerland.
Journal of pediatric endocrinology & metabolism: JPEM (Impact Factor: 0.71). 02/2006; 19(1):65-74. DOI: 10.1515/JPEM.2006.19.1.65
Source: PubMed

ABSTRACT The reduction of spontaneous physical activity (PA) and of muscle tissue are thought to be major causes of fat accretion and metabolic deterioration in Prader-Labhart-Willi syndrome (PWS). We investigated whether a generalized physical training programme in a home setting improves these parameters. The prospective study included 11 prepubertal children (mean age 8.7 years, range 5.9-11.8) with documented PWS and under continuous growth hormone treatment for at least 2.8 years. Seven children were enrolled in a training programme for several muscle groups during 4-10 minutes daily. Twelve matched children with PWS served as controls (average age 8.8 years, 6.1-11.3). Before and after training, at 6 months, PA was assessed by measuring walking distance by pedometer registration and by an activity score, and body composition by DEXA expressed as standard deviation scores (SDS) related to height. After training, lean mass (LM) increased from -1.83 to -1.48 SDS, p <0.05, whereas the controls showed no change. In the training group, walking distance and PA increased from 4.2 to 4.7 km/d and from 255 to 266 points, respectively, and these rises significantly exceeded those observed in controls. CONCLUSION: Children with PWS can be motivated by their families to follow a short daily training, which has general effects on PA and does increase, but not normalize LM.

0 Bookmarks
 · 
68 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Compared to other children, those with disability have additional challenges to being physically active. Prader-Willi Syndrome is a genetic form of childhood obesity that is characterized by hypotonia, growth hormone deficiency, behavioral, and cognitive disability. In children, the low prevalence of this syndrome (1 in 10,000 to 15,000 live births) makes group-based physical activity interventions difficult. In contrast, the home environment presents a natural venue to establish a physical activity routine for this population. This manuscript describes the design of a parent-led physical activity intervention incorporating playground and interactive console-based games to increase physical activity participation in youth with and without Prader-Willi Syndrome. The study participants will be 115 youth ages 8-15 y (45 with the syndrome and 70 without the syndrome but categorized as obese). The study will use a parallel design with the control group receiving the intervention after serving as control. Participants will be expected to complete a physical activity curriculum 4 days a week for six months including playground games 2 days a week and interactive console games 2 days a week. Parents will be trained at baseline and then provided with a curriculum and equipment to guide their implementation of the program. Tips related to scheduling and coping with barriers to daily program implementation will be provided. Throughout, parents will be contacted by phone once a week (weeks 1-4) and then every other week to receive support in between visits. Measurements of children and parents will be obtained at baseline, 12 weeks, and at the end (week 24) of the intervention. Children main outcomes include physical activity (accelerometry), body composition (dual x-ray absorptiometry), motor proficiency (Bruininks-Oseretsky Test of Motor Proficiency), quality of life and physical activity self-efficacy (questionnaires). Intervention compliance will be monitored using mail-in daily self-report checklists. This parent-guided physical activity intervention aims to increase physical activity by using a curriculum that builds physical activity related self-confidence through the development and/or enhancement of motor skill competency. Ultimately, helping children develop these skills as well as joy in being physically active will translate into sustained behavior change.Trial Registration: Current Controlled Trial: NCT02058342.
    BMC Pediatrics 02/2014; 14(1):41. DOI:10.1186/1471-2431-14-41 · 1.92 Impact Factor
    This article is viewable in ResearchGate's enriched format
  • [Show abstract] [Hide abstract]
    ABSTRACT: Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13. In infancy it is characterized by hypotonia with poor suck resulting in failure to thrive. As the child ages, other manifestations such as developmental delay, cognitive disability, and behavior problems become evident. Hypothalamic dysfunction has been implicated in many manifestations of this syndrome including hyperphagia, temperature instability, high pain threshold, sleep disordered breathing, and multiple endocrine abnormalities. These include growth hormone deficiency, central adrenal insufficiency, hypogonadism, hypothyroidism, and complications of obesity such as type 2 diabetes mellitus. This review summarizes the recent literature investigating optimal screening and treatment of endocrine abnormalities associated with PWS, and provides an update on nutrition and food-related behavioral intervention. The standard of care regarding growth hormone therapy and surveillance for potential side effects, the potential for central adrenal insufficiency, evaluation for and treatment of hypogonadism in males and females, and the prevalence and screening recommendations for hypothyroidism and diabetes are covered in detail. PWS is a genetic syndrome in which early diagnosis and careful attention to detail regarding all the potential endocrine and behavioral manifestations can lead to a significant improvement in health and developmental outcomes. Thus, the important role of the provider caring for the child with PWS cannot be overstated.
    International Journal of Pediatric Endocrinology 08/2013; 2013(1):14. DOI:10.1186/1687-9856-2013-14
    This article is viewable in ResearchGate's enriched format
  • [Show abstract] [Hide abstract]
    ABSTRACT: Children classified as overweight or obese and those with disabilities are at a greater risk of not meeting the minimum recommendation of 60min a day of moderate to vigorous physical activity (PA). Youth with Prader-Willi Syndrome (PWS) appear to participate in less PA compared to nonsyndromal children, likely due to syndrome-related factors. However, description of PA patterns in youth with PWS is lacking. The purpose of this study was to characterize PA in youth with PWS and to compare it to PA in children with nonsyndromal obesity. Twenty-four youth with PWS (ages 8-16 years) and 40 obese children without PWS (OB) (ages 8-11 years) wore accelerometers for eight consecutive days. Data were screened for compliance and classified into PA intensities: sedentary behavior (SED), light (LPA), moderate (MPA), vigorous (VPA) and moderate plus vigorous (MVPA). Youth with PWS spent 19.4% less time in weekly LPA (p=0.007) and 29.8% less time in weekly VPA compared to OB controls (p=0.036). All other intensities were similar between groups. In addition, PWS participated in less LPA and VPA during the weekends compared to OB, and less LPA on weekdays when compared to OB. There was also a trend towards PWS participating in less MVPA during the weekends and less VPA during the weekends than OB controls. There was a trend towards PWS participating in less VPA on weekends compared to weekdays, while OB participated similarly in VPA on weekdays and weekend days. On average, neither PWS nor OB children met minimum MVPA recommendations. The results suggest there is a need to design exercise programs for PWS youth that focus on integrating vigorous intensity activities, especially during the weekends when structured PA may not be available.
    Research in Developmental Disabilities 08/2014; 35(11):3081-3088. DOI:10.1016/j.ridd.2014.07.035 · 3.40 Impact Factor