Mastey Ben-Yehuda Hadassa, Gross-Tsur Varda, Hirsch Harry J, Genstil Larry, Derei Dvorit, Forer Dorit, Benarroch Fortu
Israel National Multidisciplinary Prader-Willi Syndrome Clinic, Department of Neuropediatrics, Shaare Zedek Medical Center, Jerusalem 9103102, Israel.
The Faculty of Medicine, The Hebrew University School of Medicine, Jerusalem 9112002, Israel.
J Clin Med. 2024 Jun 4;13(11):3323. doi: 10.3390/jcm13113323.
: Strict regimens of restricted caloric intake and daily physical exercise are life-saving in Prader-Willi syndrome (PWS) but are extremely challenging in home environments. PWS-specialized hostels (SH) succeed in preventing morbid obesity and in coping with behavioral disorders; however, effects of restricted living environments on quality of life (QOL) have not been described. Evidence on QOL is critical for clinicians involved in placement decisions. : We examined the impact of living in SH versus at home or in non-specialized hostels (H and NSH) on QOL, behavior, and health parameters. All 58 adults (26 males) followed-up in the National Multidisciplinary Clinic for PWS were included: 33 resided in SH, 18 lived at home, and 7 lived in NSH. Questionnaires were administered to primary caregivers to measure QOL, and data were obtained from the medical records. : The H and NSH group were compared with those for adults in SH. Despite strict diet and exercise regimens, QOL was similar for both groups. Eight-year follow-up showed that food-seeking behavior decreased in SH but increased in H and NSH. BMI, cholesterol, and triglyceride levels were lower in SH. : Our results suggest that living in SH is associated with benefits for physical health and behavior without negatively affecting QOL.
严格的热量摄入限制和日常体育锻炼方案对普拉德-威利综合征（PWS）患者来说是救命的，但在家庭环境中实施极具挑战性。PWS专科宿舍（SH）成功地预防了病态肥胖并应对了行为障碍；然而，有限的生活环境对生活质量（QOL）的影响尚未得到描述。生活质量方面的证据对于参与安置决策的临床医生至关重要。我们研究了居住在SH与在家中或非专科宿舍（H和NSH）对生活质量、行为和健康参数的影响。纳入了在国家PWS多学科诊所接受随访的所有58名成年人（26名男性）：33人居住在SH，18人住在家里，7人住在NSH。向主要照顾者发放问卷以测量生活质量，并从医疗记录中获取数据。将H和NSH组与SH中的成年人组进行比较。尽管有严格的饮食和锻炼方案，但两组的生活质量相似。八年的随访表明，SH中的寻食行为减少，而H和NSH中的寻食行为增加。SH中的体重指数、胆固醇和甘油三酯水平较低。我们的结果表明，居住在SH对身体健康和行为有益，且不会对生活质量产生负面影响。
