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首页> 外文期刊>American journal of medical genetics, Part A >Long-term weight control in adults with Prader-Willi syndrome living in residential hostels
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Long-term weight control in adults with Prader-Willi syndrome living in residential hostels

机译：在住宿宿舍的Prader-Willi综合征的成年人长期体重控制

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Hyperphagia leading to severe obesity with increased morbidity and mortality is the major manifestation of Prader-Willi syndrome. Caring for these individuals in a home environment is challenging and stressful for caregivers and families. Residential hostels specifically for PWS adults offer programs of diet, exercise, and vocational opportunities, but long-term effects of PWS hostel living have not been reported. We studied long-term changes in body mass index (BMI) for PWS adults living in residential hostels compared with age-matched controls living with families at home. The study included all 34 individuals (18 men) aged >17 years with genetically confirmed PWS living in residential hostels. BMI was recorded at the time of yearly clinic visits and compared to 23 PWS adults (10 men) living at home. BMI on entering the hostel was 36.3 +/- 11.0 kg/m(2) and decreased to 27.0 +/- 5.6 kg/m(2) (p < 0.001) after 6.9 +/- 3.9 years. For 21 residents, a slight rise of BMI to 28.8 kg/m(2) was observed 5.1 +/- 2.5 years after the lowest value was achieved. BMI of 23 PWS adults at home was 36.8 +/- 12.7 kg/m(2) versus 27.9 +/- 7.1 kg/m(2) for hostel residents in the same age range (p = 0.008). From 2008 to 2019, there were five deaths among PWS individuals aged 18-40 years living at home, compared with one death (a 43-year-old man) among hostel residents. Adults with PWS living in hostels lose weight, maintain BMI values in a normal to mildly overweight range, and have lower mortality in contrast to individuals in a family home environment.
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