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.
"Considering the abilities, needs, constraints, and preferences of children with PWS and their parents as well as previous intervention approaches that proved successful in this population [18,19] we developed a home-based PA curriculum called Active Play @ Home. This well-rounded and varied PA curriculum includes all the essential exercise components recommended in the national guidelines (progressive games and exercises targeting cardiovascular fitness, muscular strength and endurance) while also targeting motor skill competencies. "
[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.
"A diet with carbohydrate content as low as 45% of total calories may have more favorable effects on body composition and fat utilization . Prescribed daily physical activity is also important and contributes to improved body composition and REE . "
[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
"From a functional perspective, to date there are only few papers providing quantitative data of lower limb muscle strength in non-syndromal obese subjects [15-17]. Physical strength and functional capacities of PWS subjects has been previously performed by manual non-instrumental testing  and with a modified Bruininks-Oseretsky test . "
[Show abstract][Hide abstract] ABSTRACT: despite evidence of an obesity-related disability, there is a lack of objective muscle functional data in overweight subjects. Only few studies provide instrumental strength measurements in non-syndromal obesity, whereas no data about Prader-Willi syndrome (PWS) are reported. The aim of our study was to characterize the lower limb muscle function of patients affected by PWS as compared to non-syndromal obesity and normal-weight subjects.
We enrolled 20 obese (O) females (age: 29.1 +/- 6.5 years; BMI: 38.1 +/- 3.1), 6 PWS females (age: 27.2 +/- 4.9 years; BMI: 45.8 +/- 4.4) and 14 healthy normal-weight (H) females (age: 30.1 +/- 4.7 years; BMI: 21 +/- 1.6). Isokinetic strength during knee flexion and extension in both lower limbs at the fixed angular velocities of 60 degrees /s, 180 degrees /s, 240 degrees /s was measured with a Cybex Norm dynamometer.
the H, O and PWS populations appear to be clearly stratified with regard to muscle strength.: PWS showed the lowest absolute peak torque (PT) for knee flexor and extensor muscles as compared to O (-55%) and H (-47%) (P = 0.00001). O showed significantly higher strength values than H as regard to knee extension only (P = 0.0014). When strength data were normalised by body weight, PWS showed a 50% and a 70% reduction in PT as compared to O and H, respectively. Knee flexors strength values were on average half of those reported for extension in all of the three populations.
the novel aspect of our study is the determination of objective measures of muscle strength in PWS and the comparison with O and H patients. The objective characterization of muscle function performed in this study provides baseline and outcome measures that may quantify specific strength deficits amendable with tailored rehabilitation programs and monitor effectiveness of treatments.
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