Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study
Quality of life and psychological well-being in GH-treated, adult PWS patients: a longitudinal study
复制标题
DOI：
10.1111/j.1365-2788.2006.00878.x
复制
发表时间：
2007-04-01
影响因子：
3.6
通讯作者：
Semenza, C.
中科院分区：
文献类型：
--
作者：
Bertella, L.;Mori, I.;Semenza, C.
研究背景Prader-Willi综合征（PWS）是一种先天性15号染色体异常。其特征为身材矮小、肌肉张力减退、摄食过多、肥胖、行为和情绪障碍、性腺功能减退和部分生长激素（GH）缺乏。本研究的目的是评估生长激素治疗的心理健康和生活质量（QoL）在成人PWS group.Methods的长期影响，共13 PWS患者，他们的诊断证实了基因检测，和他们的父母被招募为这项研究。参与者在GH治疗开始时以及随后的6个月、12个月和24个月间隔时接受36项简明健康调查表（SF-36）和心理总体健康指数（PGWBI）评估QoL和心理健康。修改版本相同的问卷给parents.Results显着改善，相对于基线，在这两个尺度，在身体和心理健康的评价，虽然父母的评价是不太乐观的patients.Conclusion我们的研究结果表明，生活质量和心理状态的改善是持续的患者谁继续GH治疗。
Background Prader-Willi syndrome (PWS) is a congenital alteration of chromosome pair 15. It is characterized by short stature, muscular hypotonia, hyperphagia, obesity, behavioural and emotional disturbances, hypogonadism and partial Growth Hormone (GH) deficiency. The aim of this study was to assess the long-term effect of GH treatment on the psychological well-being and Quality of Life (QoL) in an adult PWS group.Methods A total of 13 PWS patients, their diagnosis confirmed by genetic tests, and their parents were recruited for this study. The participants were administered the 36-Items Short Form Health Survey (SF-36) and the Psychological General Well-Being Index (PGWBI), for the assessment of QoL and psychological well-being, at the beginning of GH treatment, and at following intervals of 6, 12 and 24 months. Modified versions of the same questionnaires were given to the parents.Results Significant improvement with respect to the baseline was found, on both scales, in the evaluation of both physical and psychological well-being, although the parents' evaluation was less optimistic than that of the patients.Conclusion Our findings suggest that the amelioration of QoL and psychological status is sustained in patients who continue GH treatment.