TREATMENT WITH HUMAN GROWTH-HORMONE IN PATIENTS WITH PRADER-LABHART-WILLI-SYNDROME REDUCES BODY-FAT AND INCREASES MUSCLE MASS AND PHYSICAL PERFORMANCE

Twelve children with documented Prader-Labhart-Willi syndrome were tre ated with human growth hormone (24 U/m(2)/week) during 1 year. The chi ldren were divided into three groups: group 1. overweight and prepuber tal (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 year s). 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 h eight 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 du al X-ray energy absorptiometry, dropped by a third, whereas muscle mas s increased by a fourth. Physical capability (Wingate test) improved c onsiderably. The children n ere reported to be much more active and ca pable. In group 2, similar changes were seen, but weight for height in creased, probably because muscle mass increase exceeded fat mass decre ase. Changes in group 3 were similar as in group 1, even though far le ss distinct. Conclusion Growth hormone treatment in Prader-Labhart-Wil li syndrome led to dramatic changes: distinct increase in growth veloc ity, height and muscle mass, as well as an improvement in physical per formance. Fat mass and weight for height decreased in the initially ov erweight children, and weight for height increased in underweight chil dren.