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速度和预测成人身高、身体成分、静息能量消耗和骨密度、肌肉力量和自尊指标有积极影响。普瑞德-威利综合征现在被认为与父亲种系遗传的15q小缺失或母亲染色体失调有关。患有普瑞德-威利综合征的婴儿手脚短小，面部特征明显。在最初无法茁壮成长之后，由于贪婪和不受控制的食欲，它们的体重迅速增加。Prader-Willi患者有显著的身体肌肉量减少、虚弱、成年期严重骨质疏松、线性生长缓慢和变异性性腺功能减退。如果他们保持在瘦的状态，他们的高度速度进一步降低，但仍有增加的体脂量。先前的非受控研究表明，生长激素治疗可以改善患有普瑞德-威利综合征的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.