EFFECTS OF GROWTH HORMONE ON METABOLISM AND SATIATION IN PRADER-WILLI SYNDROME

生长激素对普瑞德威利综合征代谢和饱足感的影响

• 批准号: 7374640
• 负责人: JENNIFER L MILLER
• 金额: $ 7.1万
• 依托单位: UNIVERSITY OF FLORIDA
• 依托单位国家: 美国
• 财政年份: 2005
• 资助国家: 美国
• 起止时间: 2005-12-01 至 2006-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7374640
• 关键词: EFFECTS; GROWTH; HORMONE; METABOLISM; SATIATION

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Prader-Willi Syndrome (PWS) is a complex neurobehavioral syndrome whose clinical features include neonatal failure-to-thrive, a distinct behavioral phenotype, cognitive impairment, hypotonia, hyperphagia and morbid obesity. This syndrome is the most commonly recognized genetic cause of obesity. It has been suggested that the hyperphagia is secondary to altered hypothalamic function, which is a consequence of the genetic alterations that characterize this condition. Children with Prader-Willi syndrome have evidence of a growth hormone (GH) deficiency secondary to hypothalamic dysfunction3. Studies have shown that children with Prader-Willi syndrome treated with GH have a decrease in fat mass and an increase in lean body mass, as well as an increase in growth velocity and in resting energy expenditure26,24. It is currently unknown, however, if treatment of children with Prader-Willi syndrome with GH causes changes in the hypothalamus, or if the effects are solely peripheral1. This study will investigate the beneficial effects of GH on hypothalamic recognition of hunger and satiety, as well as on the basal metabolic rate and body composition of PWS individuals. We will compare the neuroanatomical correlates of hunger and satiation using functional MRI (fMRI) on PWS patients before and one year after GH treatment. In addition, we will use normal weight siblings and non-PWS obese individuals as additional comparison groups. Blood will be drawn to determine various hormonal and neurotransmitter levels at times correlating with hunger and satiety in these individuals. Basal metabolic rate (BMR) and body composition via dual energy X-ray absorptiometry (DEXA) will be assessed for all individuals, and will be reevaluated in PWS subjects one year after treatment with GH.

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。Prader-Willi综合征（PWS）是一种复杂的神经行为综合征，其临床特征包括新生儿发育不良、独特的行为表型、认知障碍、张力低下、嗜食和病态肥胖。这种综合征是公认的最常见的肥胖遗传原因。有人认为，贪食是继发于下丘脑功能的改变，这是这种疾病特征的遗传改变的结果。患有普瑞德-威利综合征的儿童有生长激素（GH）缺乏的证据，继发于下丘脑功能障碍3。研究表明，接受生长激素治疗的Prader-Willi综合征儿童脂肪量减少，瘦体重增加，生长速度和静息能量消耗增加26,24。然而，目前尚不清楚，用生长激素治疗普拉德-威利综合征的儿童是否会引起下丘脑的改变，或者这种影响是否仅仅是外周的。本研究将探讨生长激素对PWS个体下丘脑饥饿和饱腹感识别以及基础代谢率和体成分的有益影响。我们将使用功能性磁共振成像（fMRI）比较PWS患者在GH治疗前和治疗后一年的饥饿感和饱腹感的神经解剖学相关性。此外，我们将使用体重正常的兄弟姐妹和非pws肥胖个体作为额外的对照组。抽血来测定不同的激素和神经递质水平有时与这些人的饥饿和饱腹感有关。将通过双能x线吸收仪（DEXA）评估所有个体的基础代谢率（BMR）和体成分，并在GH治疗一年后对PWS患者进行重新评估。