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Effectiveness of a 6-month home-based training program in Prader-Willi patients
Authors:Luca Vismara  Veronica Cimolin  Graziano Grugni  Manuela Galli  Cinzia Parisio  Olivia Sibilia  Paolo Capodaglio
Affiliation:1. Institute of Exercise Physiology and Wellness, University of Central Florida, 12494 University Boulevard, Orlando, FL 32816, United States;2. Department of Exercise and Sport Science, University of North Carolina, Chapel-Hill, Chapel Hill, NC, United States;3. Department of Health and Kinesiology, Georgia Southern University, Statesboro, GA, United States;4. Sports Science Center Research Institute, MusclePharm, Inc., Denver, CO, United States;5. Department of Sports Exercise Science, United States Sports Academy, Daphne, AL, United States;6. Institute of Exercise Physiology and Wellness, University of Central Florida, Orlando, FL, United States;1. University of Miami, Laboratory of Neuromuscular Research and Active Aging, Department of Kinesiology and Sports Sciences, Coral Gables, FL, United States;2. Miller School of Medicine, Center on Aging, University of Miami, Miami, FL, United States;1. Division of Urology, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT;2. Cancer Control and Population Sciences Program, Huntsman Cancer Institute, Salt Lake City, UT;3. Department of Health, Kinesiology, and Recreation, The University of Utah, Salt Lake City, UT;4. Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York, NY
Abstract:In addition to hypotonia and relative sarcopenia, patients with Prader-Willi syndrome (PWS) show reduced spontaneous physical activity and gait disorders. Scant evidence exists that daily muscle training increases their lean mass and physical activity levels. Whether adequate long-term physical training is feasible and effective in improving muscle function and gait in PWS is still unknown.Eleven adult PWS patients (mean age: 33.8 ± 4.3 years; mean BMI: 43.3 ± 5.9 kg/m2) admitted to our hospital were enrolled in this study. During their hospital stay they attended a 2-week rehabilitation program which included supervised exercise sessions. At discharge, Group 1 (6 patients) continued the same exercises at home for 6 months, while Group 2 (5 patients) did not continue home-based training. They were assessed at admission (PRE), at 2 weeks (POST1) and at 6 months (POST2). The assessment consisted of a clinical examination, 3D gait analysis and muscle strength measurement with an isokinetic dynamometer.After 2 weeks of supervised training (POST1), no significant changes in spatial-temporal gait parameters were observed, although significant improvements in ankle dorsal flexion during stance and swing and knee flexor strength were evidenced by 3D gait analysis and dynamometry in all patients.Following 6 months of home training (POST2), Group 1 had showed significant improvements in cadence and reduced knee hyperextension in mid-stance. Ankle plantar and dorsal flexors isokinetic strength had improved significantly at 120° s?1, whereas Group 2 showed no changes in their spatial-temporal and kinematic parameters.The present study reinforces the idea that even in participants with PWS who present with a distinctive psychological profile, long-term group interventions are feasible and effective in improving their overall physical functioning. Providing an effective and simple home-based training program represents a continuum of the rehabilitation process outside the hospital, which is a crucial issue in chronic conditions.
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