Laron syndrome due to a post-receptor defect: Response to IGF-I treatment

Z. Laron, B. Klinger, R. Eshet, H. Kaneti, A. Karasik, A. Silbergeld

Research output: Contribution to journalArticlepeer-review

Abstract

Three siblings with Laron syndrome (LS) and normal serum growth hormone binding protein (GHBP) are described. Basal serum levels of hGH were high and IGF-1 low, and in contradistinction to the classical form of the disease serum GHBP and IGFBP-3 were normal in these patients. After 7 days of human growth hormone administration serum IGFBP-3 levels as well as the number of red blood cell IGF receptor sites increased. After short- and long-term IGF-1 administration the IGF-1 receptor binding capacity as well as the number of IGF receptor sites decreased to levels found in control subjects. One year treatment with IGF-1 increased the growth velocity by 47-96% in the two older children. It is concluded that the findings described are compatible with a normal GH receptor and normal signal transmission for IGFBP-3 synthesis but a defect exists in the post-GH receptor mechanism for the generation of IGF-1. This is the first description of this type of defect leading to a variant of Laron syndrome.

Original languageEnglish
Pages (from-to)757-763
Number of pages7
JournalIsrael Journal of Medical Sciences
Volume29
Issue number12
StatePublished - 1993
Externally publishedYes

Keywords

  • Growth hormone receptor
  • IGF-1 treatment
  • Laron syndrome
  • Post-receptor defect

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