Growth hormone normalises height, prediction of final height and hand length in children with Prader-Willi syndrome after 4 years of therapy

Horm Res. 2000;53(4):185-92. doi: 10.1159/000023565.

Abstract

Background: Based on the reported favourable effects of growth hormone (GH) treatment on growth and body composition in Prader-Labhart-Willi syndrome, we studied age dependency and the long-term effects on growth dynamics to elucidate the assumed hypothalamic GH deficiency.

Methods: We examined 23 children treated with hGH (24 U/m(2)/week) during a median of 4 (range 1.5-5.5) years; group 1: 10 young underweight (age 0.3-4.1 years), group 2: 8 prepubertal overweight (age 3.7-9.5 years) and group 3: 5 pubertal overweight children (age 9.0-14.6 years).

Results: After 4 years of therapy, height gain amounted to 1.8 SD; height (0.0 SD) and hand length (-0.2 SD) were normalised in the 2 prepubertal groups; in children above 6 years, height prediction approached parental target height. Weight for height rose in group 1 (to 0.64 SD) and decreased in group 2 (to 0.71 SD) to normal levels. Bone maturation of the pubertal children was too advanced to show a clear growth response to GH (height gain 0.42 SD). Even in this group, weight for height was reduced, but remained supernormal.

Conclusion: Under exogenous GH, growth and body proportions are normalised in prepubertal children. With early institution of treatment, final height prediction reaches the parental target height range after 3 years. Such a growth-promoting effect of exogenous GH has so far only been described in children with GH deficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blood Glucose / analysis
  • Body Height*
  • Body Weight
  • Child
  • Child, Preschool
  • Glycated Hemoglobin / analysis
  • Hand / anatomy & histology*
  • Human Growth Hormone / administration & dosage
  • Human Growth Hormone / therapeutic use*
  • Humans
  • Infant
  • Prader-Willi Syndrome / drug therapy*
  • Prader-Willi Syndrome / physiopathology
  • Time Factors

Substances

  • Blood Glucose
  • Glycated Hemoglobin A
  • Human Growth Hormone