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

本研究将确定生长激素治疗是否对青春期前Prader-Willi综合征儿童的线性身高增长速度和预测的成年身高、身体成分、静息能量消耗和骨密度、肌肉力量和自尊指数有积极影响。Prader-Willi综合征现在被认为与从父系生殖系遗传的小15 q缺失或与母系同二体性有关。 患有Prader-Willi综合征的婴儿具有小手和脚以及特征性的面部外观。 在最初未能茁壮成长之后，由于贪婪和不受控制的食欲，他们的体重迅速增加。Prader-Willi患者的身体肌肉量显著减少，虚弱，成年后严重骨质疏松，线性生长缓慢和可变的低促性腺激素性性腺功能减退症。 如果他们保持在变瘦的状态，他们的身高增长速度会进一步降低，但布迪量仍会增加。 以前的非对照研究表明，生长激素治疗可改善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.