Z Laron1, B Klinger, W F Blum, A Silbergeld, M B Ranke. 1. Institute of Pediatric and Adolescent Endocrinology, Children's Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Israel.
Abstract
OBJECTIVE: The aim of the study was to investigate the serum levels of IGFBP-3, the major IGF-I binding protein, in patients with Laron type dwarfism (LTD) before and after IGF-I treatment. DESIGN AND PATIENTS: Eight Laron type dwarfism patients (four children and four adults) were treated for 7 days by one daily s.c. injection of biosynthetic IGF-I in doses of 120 or 150 micrograms/kg/day. MEASUREMENTS: Blood was sampled in the fasting state before and 1 and 7 days after the last injection. RESULTS: It was found that IGF-I administration significantly reduced plasma hGH levels with recovery after one week of no treatment. Serum IGFBP-3 was abnormally low (0.70 +/- 0.37 mg/l) and decreased significantly further during IGF-I treatment (to 0.48 +/- 0.28 mg/l) (P less than 0.065). CONCLUSIONS: The finding that serum IGFBP-3 is low in Laron type dwarfism, a disease due to molecular defects in the GH receptor, is compatible with the hypothesis that this IGF binding protein is GH-dependent. On the other hand the decrease during IGF-I administration and concomitant suppression of GH secretion may denote either that GH activity is not completely blocked in this syndrome or that there are additional mechanisms regulating IGFBP-3 synthesis.
OBJECTIVE: The aim of the study was to investigate the serum levels of IGFBP-3, the major IGF-I binding protein, in patients with Laron type dwarfism (LTD) before and after IGF-I treatment. DESIGN AND PATIENTS: Eight Laron type dwarfism patients (four children and four adults) were treated for 7 days by one daily s.c. injection of biosynthetic IGF-I in doses of 120 or 150 micrograms/kg/day. MEASUREMENTS: Blood was sampled in the fasting state before and 1 and 7 days after the last injection. RESULTS: It was found that IGF-I administration significantly reduced plasma hGH levels with recovery after one week of no treatment. Serum IGFBP-3 was abnormally low (0.70 +/- 0.37 mg/l) and decreased significantly further during IGF-I treatment (to 0.48 +/- 0.28 mg/l) (P less than 0.065). CONCLUSIONS: The finding that serum IGFBP-3 is low in Laron type dwarfism, a disease due to molecular defects in the GH receptor, is compatible with the hypothesis that this IGF binding protein is GH-dependent. On the other hand the decrease during IGF-I administration and concomitant suppression of GH secretion may denote either that GH activity is not completely blocked in this syndrome or that there are additional mechanisms regulating IGFBP-3 synthesis.
Authors: T Yamaguchi; M Kanatani; M Yamauchi; H Kaji; T Sugishita; D J Baylink; S Mohan; K Chihara; T Sugimoto Journal: Calcif Tissue Int Date: 2006-01-06 Impact factor: 4.333
Authors: Arne Hinrichs; Barbara Kessler; Mayuko Kurome; Andreas Blutke; Elisabeth Kemter; Maren Bernau; Armin M Scholz; Birgit Rathkolb; Simone Renner; Sebastian Bultmann; Heinrich Leonhardt; Martin Hrabĕ de Angelis; Hiroshi Nagashima; Andreas Hoeflich; Werner F Blum; Martin Bidlingmaier; Rüdiger Wanke; Maik Dahlhoff; Eckhard Wolf Journal: Mol Metab Date: 2018-03-15 Impact factor: 7.422