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

GH 对生长、身体成分、骨密度的影响

• 批准号: 6220142
• 负责人: Lynne L. Levitsky
• 金额: $ 0.06万
• 依托单位: MASSACHUSETTS GENERAL HOSPITAL
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-12-01 至 1999-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6220142
• 关键词: Prader Willi syndrome; bioenergetics; body composition; body height; bone density; child (0-11); clinical research; hormone regulation /control mechanism; hormone therapy; human puberty; human subject; human therapy evaluation; mental disorder chemotherapy; muscle strength; self concept; somatotropin; Prader Willi syndrome; bioenergetics; body composition; body height; bone density; child (0-11); clinical research; hormone regulation /control mechanism; hormone therapy; human puberty; human subject; human therapy evaluation; mental disorder chemotherapy; muscle strength; self concept; somatotropin

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.

这项研究将确定患有Prader-Willi综合征青春期前儿童的生长激素治疗是否会对线性高度速度产生积极影响，并预测成人身高，身体成分，静息能量消耗和骨密度，肌肉力量，肌肉力量，肌肉力量和自尊心索引。现在被认为是与父亲种系或母体静脉曲张遗传的小15Q缺失有关的prader-willi综合征。患有Prader-Willi综合征的婴儿的手和脚很小，外观具有特征性的面部外观。 最初未能蓬勃发展后，由于食欲不足和不受控制的食欲，它们迅速增加了体重。 Prader-Willi患者的体内肌肉质量，无力，骨质疏松症的严重降低，线性生长缓慢和可变的性不良性型性降低。 如果将它们保持在稀薄状态，则其高度速度进一步降低，但仍增加了脂肪质量。 先前的不受控制的研究表明，用GRWOTH激素的治疗​​改善了Prader-Willi综合征儿童的身体肌肉质量以及线性生长。