Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance

被引:77
作者
Eiholzer, U
Gisin, R
Weinmann, C
Kriemler, S
Steinert, H
Torresani, T
Zachmann, M
Prader, A
机构
[1] Ctr Adolescent Med & Fdn Growth Puberty Adolescen, CH-8006 Zurich, Switzerland
[2] McMaster Univ, Childrens Exercise & Nutr Ctr, Hamilton, ON, Canada
[3] Univ Zurich, Dept Med Radiol Nucl Med, CH-8091 Zurich, Switzerland
[4] Univ Zurich, Dept Paediat, CH-8032 Zurich, Switzerland
关键词
body composition; growth hormone treatment; physical performance; Prader-Willi syndrome; quality of life;
D O I
10.1007/s004310050832
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m(2)/week) during 1 year. The children were divided into three groups: group 1. overweight and prepubertal (n = 6, age 3.8-7.0 years); group 2. underweight and prepubertal (n = 3, age 0.6-4.1 years); group 3: pubertal (n = 3, age 9.2-14.6 years). In group 1, height increased from -1.7 SD to -0.6 SDI while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SE to 1.2 SD. Hand length increased from -1.5 SD to -0.4 SD and foot length from -2.5 SD to -1.4 SD. Body fat, measured by dual X-ray energy absorptiometry, dropped by a third, whereas muscle mass increased by a fourth. Physical capability (Wingate test) improved considerably. The children n ere reported to be much more active and capable. In group 2, similar changes were seen, but weight for height increased, probably because muscle mass increase exceeded fat mass decrease. Changes in group 3 were similar as in group 1, even though far less distinct. Conclusion Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight children.
引用
收藏
页码:368 / 377
页数:10
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