Laron Zvi, Ginsberg Shira, Lilos Pnina, Arbiv Mira, Vaisman Nahum
Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, 14 Kaplan Street, 49202 Petah Tikva, Israel.
Growth Horm IGF Res. 2006 Feb;16(1):61-4. doi: 10.1016/j.ghir.2005.12.001. Epub 2006 Jan 27.
Laron syndrome (LS) is an autosomal recessive disease caused by deletions or mutations in the GH receptor gene leading to an inability of insulin-like growth factor I (IGF-I) generation. Among the major resulting body changes are dwarfism and obesity. The only effective treatment is daily administration of biosynthetic IGF-I. Body composition determination by DEXA (dual energy X-ray absorptiometry) of three girls with LS treated by IGF-I for 1, 3 and 11 1/2 years, respectively, revealed that concomitantly with the increase in growth there was a significant increase in body adipose tissue to double or triple the normal values. Due to the underdevelopment of the muscular and skeletal systems body mass index (BMI) did not accurately reflect the degree of obesity. In conclusion, IGF-I similar to insulin, exerts an adipogenic effect.
拉龙综合征(LS)是一种常染色体隐性疾病,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)。由此产生的主要身体变化包括侏儒症和肥胖症。唯一有效的治疗方法是每日注射生物合成IGF-I。对分别接受IGF-I治疗1年、3年和11.5年的三名拉龙综合征女孩进行双能X线吸收法(DEXA)身体成分测定,结果显示,随着生长加速,身体脂肪组织显著增加,达到正常值的两倍或三倍。由于肌肉和骨骼系统发育不全,体重指数(BMI)不能准确反映肥胖程度。总之,与胰岛素类似,IGF-I具有促脂肪生成作用。