Department of Pediatrics, University of Chieti, Chieti, Italy.
Center of Advanced Studies and Technology - CAST (ex CesSI-MeT), University of Chieti, Chieti, Italy.
Front Endocrinol (Lausanne). 2023 Mar 15;14:1141039. doi: 10.3389/fendo.2023.1141039. eCollection 2023.
Linear growth during childhood is the result of the synergic contribution of different factors. The best growth determinant system during each period of life is represented by the growth hormone-insulin-like growth factor axis (GH-IGF), even if several other factors are involved in normal growth. Within the broad spectrum of growth disorders, an increased importance has been placed on growth hormone insensitivity (GHI). GHI was reported for the first time by Laron as a syndrome characterized by short stature due to GH receptor (GHR) mutation. To date, it is recognized that GHI represents a wide diagnostic category, including a broad spectrum of defects. The peculiar characteristic of GHI is the low IGF-1 levels associated with normal or elevated GH levels and the lack of IGF-1 response after GH administration. Recombinant IGF-1 preparations may be used in the treatment of these patients.
儿童期线性生长是多种因素协同作用的结果。在生命的每个阶段，最好的生长决定因素系统是由生长激素-胰岛素样生长因子轴（GH-IGF）代表的，尽管还有其他一些因素参与正常生长。在广泛的生长障碍中，生长激素不敏感（GHI）的重要性越来越大。Laron 首次报道了 GHI，其特征是由于 GH 受体（GHR）突变导致身材矮小。迄今为止，人们已经认识到 GHI 代表了一个广泛的诊断类别，包括广泛的缺陷。GHI 的独特特征是与正常或升高的 GH 水平相关的低 IGF-1 水平，以及 GH 给药后 IGF-1 反应缺乏。重组 IGF-1 制剂可用于这些患者的治疗。
