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Specific treatment of Prader-Willi syndrome through cyclical rehabilitation programmes
Emanuele Grolla1, Gilberto Andrighetto, Pietro Parmigiani
1M. Baschirotto Institute for Rare Diseases, Centro Diagnostico-riabilitativo per la Diagnosi Terapia e Cura delle Malattie Rare, Costozza di Longare-Vicenza, Italy.
Purpose:
To evaluate retrospectively the efficiency of our rehabilitation programme for patients with Prader-Willi Syndrome. In total, 49 patients were examined, 21 female and 28 male, the youngest in their late teens. Prader-Willi syndrome is generally characterised by cognitive impairment, behavioural abnormalities, and hyperphagia. Patients are usually considerably adverse to any form of physical exercise, and despite hormonal therapy, weight control in adult patients can be difficult.
Methods:
Four times a year, disease-specific residential programmes were organised, each lasting 4 weeks. The patients were restricted to a 1500 Kcal diet. In addition, they were required to do 6.5 h of physical exercise daily, stamina being built up by using music therapy, psychomotor therapy, education and entertainment activities.
Results:
BMI decreased by 2.1 average points in every residential session. For three patients who attended our treatments regularly, a reduction of 8.9 points over 6 years was recorded. An attendance of at least three sessions per year seemed to be necessary to substantially reduce weight.
Conclusions:
A multidisciplinary approach and a daily calorie-counted diet can lead to significant weight loss in teenage and adult PWS patients. This approach would also be suitable in treating patients with other obesity syndromes with mental retardation.
