EFFECT OF GH IN GROWTH, BODY COMP, BONE DENS & MUSCLE STRENTH IN PRADI-WILLI SYN

这项研究将确定对患有Prader-Willi综合征的青春期前儿童进行生长激素治疗是否会对线身高速度和预测的成人身高、身体成分、静息能量消耗和骨密度、肌肉力量和自尊指数产生积极影响。Prader-Willi综合征现在被认为与从父亲的生殖系遗传的一个小的15q缺失或与母亲的同体有关。患有Prader-Willi综合征的婴儿手脚较小，面部特征明显。在最初未能茁壮成长之后，由于贪婪和不受控制的食欲，它们的体重迅速增加。Prader-Willi患者的身体肌肉质量显著减少，身体虚弱，成年后出现严重的骨质疏松症，线性生长缓慢，可变的性腺激素减退症。如果他们保持在变薄的状态，他们会进一步降低身高速度，但仍然会增加乳房脂肪质量。以前的非对照研究表明，格罗斯激素治疗改善了Prader-Willi综合征儿童的身体肌肉质量和线性生长。

This study will determine whether growth hormone therapy in prepubertal children with Prader-Willi syndrome positively affects linear height velocity and predicted adult height, body composition, resting energy expenditure and bone density, muscle strength, and indices of self esteem. Prader-Willi Syndrome is now recognized to be associated with a small 15q deletion inherited from the paternal germline or with maternal isdisomy. Infants with Prader-Willi syndrome have small hands and feet and a characteristic facial appearance. After initially failing to thrive, they gain weight rapidly as a result of voracious and uncontrolled appetite. Prader-Willi patients have remarkably decreased body muscle mass, weakness, severe osteoporosis in adulthood, slow linear growth and variable hypogonadtropic hypogonadism. If they are maintained in the thinned state, they have further diminished height velocity and yet still have increased budy fat mass. Previous uncontrolled studies have suggested that treatment with grwoth hormone improves body muscle mass as well as linear growth in children with Prader-Willi syndrome.