European Journal of Pediatrics

, Volume 157, Issue 5, pp 368–377

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

  • U. Eiholzer
  • R. Gisin
  • C. Weinmann
  • S. Kriemler
  • H. Steinert
  • T. Torresani
  • M. Zachmann
  • A. Prader
Endocrinology

DOI: 10.1007/s004310050832

Cite this article as:
Eiholzer, U., Gisin, R., Weinmann, C. et al. Eur J Pediatr (1998) 157: 368. doi:10.1007/s004310050832

Abstract

Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m2/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 SD, while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SD 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 were 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.

Key words

Body compositionGrowth hormone treatmentPhysical performancePrader-Willi syndromeQuality of life

Abbreviations

BMI

body mass index

DEXA

dual X-ray energy absorptiometry

FFM

fat free mass

GHD

growth hormone deficiency

hGH

human growth hormone

LBM

lean body mass

PWS

Prader-Labhart-Willi syndrome

IGF

insulin-like growth factor

W AnT

Wingate anaerobic test

Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • U. Eiholzer
    • 1
  • R. Gisin
    • 1
  • C. Weinmann
    • 1
  • S. Kriemler
    • 2
  • H. Steinert
    • 3
  • T. Torresani
    • 4
  • M. Zachmann
    • 4
  • A. Prader
    • 4
  1. 1.Centre for Adolescent Medicine and Foundation Growth Puberty AdolescenceZurichSwitzerland
  2. 2.Children’s Exercise and Nutrition CentreMcMaster UniversityHamiltonCanada
  3. 3.Department of Medical Radiology, Nuclear MedicineZurich UniversityZurichSwitzerland
  4. 4.Department of PaediatricsUniversity of ZurichZurichSwitzerland