Laron Zvi
Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Sackler Faculty of Medicine, Tel Aviv University, Israel.
Pediatr Endocrinol Rev. 2008 Mar;5(3):766-71.
Laron syndrome (LS, congenital primary GH insensitivity) is caused by deletions or mutations in the GH receptor gene, resulting in an inability to generate insulin-like growth factor-I (IGF-I). If untreated, the deficiency of IGF-I results in severe dwarfism, as well as skeletal and muscular underdevelopment. The only treatment is the daily administration of recombinant IGF-I. This review summarizes the present experience by several groups worldwide. The main conclusions are: A. The one or two injections regimen result in the same growth velocity; B. The growth velocity obtained with IGF-I administration is smaller than that observed with hGH in children with congenital isolated GH deficiency; C. Overdosage of IGF-I causes a series of adverse effects which can be avoided by carefully monitoring the serum IGF-I and GH levels.
拉龙综合征（LS，先天性原发性生长激素不敏感）由生长激素受体基因的缺失或突变引起，导致无法生成胰岛素样生长因子-I（IGF-I）。若不治疗，IGF-I缺乏会导致严重侏儒症以及骨骼和肌肉发育不全。唯一的治疗方法是每日注射重组IGF-I。本综述总结了全球多个研究小组的现有经验。主要结论如下：A. 每日一次或两次注射方案产生的生长速度相同；B. 先天性单纯生长激素缺乏儿童使用IGF-I治疗所获得的生长速度低于使用人生长激素（hGH）观察到的生长速度；C. IGF-I过量会引起一系列不良反应，通过仔细监测血清IGF-I和生长激素水平可避免这些不良反应。
