Elevated insulin-like growth factor-I values in children with Prader-Willi syndrome compared with growth hormone (GH) deficiency children over two years of GH treatment

J Clin Endocrinol Metab. 2010 Oct;95(10):4600-8. doi: 10.1210/jc.2009-1831.

Abstract

Background: Children with Prader-Willi syndrome (PWS) are routinely treated with GH and have a response comparable with that observed in children with GH deficiency (GHD).

Objective: The objective of the study was to compare changes in serum IGF-I, IGF binding protein 3 (IGFBP-3), IGF-I to IGFBP-3 molar ratio, and growth velocity during the first 2 yr of GH therapy in PWS and GHD children.

Subjects and methods: Thirty-three children with PWS (14 boys, 4.9 ± 3.8 yr) and 591 with GHD (351 boys, 9.6 ± 3.6 yr), all naive to GH treatment, were included in this study. Serum IGF-I and IGFBP-3 were measured at 0, 6, 12, and 24 months of GH therapy. The mean initial dose of GH was 0.9 and 1 mg/m(2) · d in the PWS and GHD groups, respectively.

Results: Mean ± SD IGF-I sdscore (SDS) and IGFBP-3 SDS were significantly higher in PWS compared with GHD. The IGF-I to IGFBP-3 molar ratio was significantly lower at baseline and subsequently not different. Despite significantly lower GH doses in PWS children at 6, 12, and 24 months (P = 0.021, P = 0.021, P = 0.001), IGF-I reached 2.8 ± 1.2 SDS at 24 months (72% of values > 2 SDS), and remained at 0.7 ± 1.6 SDS in GHD children (17% of values > 2 SDS). IGFBP-3 did not exceed 2 SDS in either group. There was no significant change in the IGF-I to IGFBP-3 molar ratio.

Conclusions: IGF-I SDS increases to a greater extent in PWS than GHD. Bioavailable IGF-I is apparently not different, suggesting that any possible safety issues related to elevated IGF-I are similar in both groups.

Publication types

  • Comparative Study
  • Evaluation Study

MeSH terms

  • Body Mass Index
  • Child
  • Child, Preschool
  • Cohort Studies
  • Dwarfism, Pituitary / blood
  • Dwarfism, Pituitary / drug therapy*
  • Dwarfism, Pituitary / metabolism
  • Female
  • Growth Charts
  • Growth Disorders / blood*
  • Growth Disorders / complications
  • Growth Disorders / drug therapy*
  • Growth Disorders / metabolism
  • Hormone Replacement Therapy
  • Human Growth Hormone / deficiency
  • Human Growth Hormone / therapeutic use*
  • Humans
  • Infant
  • Insulin-Like Growth Factor Binding Protein 3 / blood
  • Insulin-Like Growth Factor I / metabolism*
  • Male
  • Prader-Willi Syndrome / blood*
  • Prader-Willi Syndrome / complications
  • Prader-Willi Syndrome / drug therapy*
  • Prader-Willi Syndrome / metabolism
  • Up-Regulation

Substances

  • Insulin-Like Growth Factor Binding Protein 3
  • Human Growth Hormone
  • Insulin-Like Growth Factor I