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

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          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.

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          Author and article information

          Journal
          Isr. J. Med. Sci.
          Israel journal of medical sciences
          0021-2180
          0021-2180
          Dec 1993
          : 29
          : 12
          Affiliations
          [1 ] Endocrinology and Diabetes Research Unit, Children's Medical Center of Israel, Petah Tikva.
          Article
          8300382

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