EVALUATION OF GH EFFECTS ON REE AND BODY COMPOSITION OF CHILDREN WITH PWS

我们寻求支持，以调查生长激素（GH）的合成代谢影响的身体组成与遗传形式的肥胖，普瑞德-威利综合征（PWS）的儿童。我们已经开始研究生长激素对身体组成的代谢影响，包括线性生长率、骨密度、营养摄入和心血管危险因素，包括静息能量消耗、瘦体重和体脂百分比、肌肉力量和敏捷性、肺功能以及碳水化合物和脂质代谢。这个患者群体提供了一个已知的遗传肥胖风险因素的模型，以及生长激素缺乏的代谢后果。最近的研究表明，生长激素的合成代谢作用可以改善身体成分，同时减少肥胖和其他心血管危险因素。我们已经获得了一项全面的对照研究的同行评审资助，这为从事多学科临床研究提供了一个独特的机会。然而，自从这项临床拨款授予以来，我们为这项研究增加了一个额外的方面：静息能量消耗（REE）的测量。已经获得了足够的资金来完成大部分的研究目标，但没有用于稀土元素的测量。利用呼吸商测量REE对研究方案具有重要的科学价值，并将直接适用于未来GH和其他合成代谢激素对身体成分的代谢影响的研究。这项评估将为PWS中导致体成分异常的因素提供重要信息，同时进一步为研究人员开展生长激素对体成分和肥胖的代谢作用的重要研究做准备。

We seek support to investigate the anabolic effects of growth hormone (GH) on body composition in children with a genetic form of obesity, Prader-Willi Syndrome (PWS). We have begun to study the metabolic effects of GH on body composition including linear growth rate, bone density, nutrient intake, and cardiovascular risk factors including resting energy expenditure, lean body mass and percent body fat, muscle strength and agility, pulmonary function, as well as carbohydrate and lipid metabolism. This patient population provides a model with known risk factors for genetic obesity, and metabolic consequences of growth hormone deficiency. Recent investigations have shown anabolic effects of GH improving body composition, while decreasing obesity and other cardiovascular risk factors. We have been awarded peer reviewed funding for a comprehensive controlled study, which provides a unique opportunity to engage in multi-disciplinary clinical research. However, since the time of this clinical grant award, we have added an additional facet to the study: the measurement of resting energy expenditure (REE). Sufficient funds have been obtained to accomplish the majority of study objectives, but not for REE measurements. The measurement of REE utilizing respiratory quotient measurements contributes substantial scientific value to the study protocol, and will be directly applicable to future investigations of metabolic effects of GH and other anabolic hormones on body composition. This assessment will provide important information about factors leading to abnormal body composition in PWS, while further preparing the fellow to conduct important future investigations of the metabolic effects of GH on body composition and obesity.