Laron Z, Lilos P, Klinger B
Institute of Pediatric and Adolescent Endocrinology Children's Medical Center, Beilinson Campus, Sackler Faculty of Medicine, Petah Tikva, Israel.
Arch Dis Child. 1993 Jun;68(6):768-70. doi: 10.1136/adc.68.6.768.
Growth curves for children with Laron syndrome were constructed on the basis of repeated measurements made throughout infancy, childhood, and puberty in 24 (10 boys, 14 girls) of the 41 patients with this syndrome investigated in our clinic. Growth retardation was already noted at birth, the birth length ranging from 42 to 46 cm in the 12/20 available measurements. The postnatal growth curves deviated sharply from the normal from infancy on. Both sexes showed no clear pubertal spurt. Girls completed their growth between the age of 16-19 years to a final mean (SD) height of 119 (8.5) cm whereas the boys continued growing beyond the age of 20 years, achieving a final height of 124 (8.5) cm. At all ages the upper to lower body segment ratio was more than 2 SD above the normal mean. These growth curves constitute a model not only for primary, hereditary insulin-like growth factor-I (IGF-I) deficiency (Laron syndrome) but also for untreated secondary IGF-I deficiencies such as growth hormone gene deletion and idiopathic congenital isolated growth hormone deficiency. They should also be useful in the follow up of children with Laron syndrome treated with biosynthetic recombinant IGF-I.
我们诊所对41例拉伦综合征患者中的24例(10名男孩,14名女孩)进行了研究,根据在婴儿期、儿童期和青春期进行的多次测量绘制了儿童生长曲线。出生时即已发现生长迟缓,在12/20次可获得的测量中,出生身长在42至46厘米之间。从婴儿期开始,出生后的生长曲线就与正常情况有很大偏差。两性均未出现明显的青春期生长突增。女孩在16至19岁之间完成生长,最终平均(标准差)身高为119(8.5)厘米,而男孩在20岁以后仍继续生长,最终身高为124(8.5)厘米。在所有年龄段,上身与下身的比例均比正常平均值高出2个标准差以上。这些生长曲线不仅构成了原发性遗传性胰岛素样生长因子-I(IGF-I)缺乏症(拉伦综合征)的模型,也构成了未治疗的继发性IGF-I缺乏症的模型,如生长激素基因缺失和特发性先天性孤立性生长激素缺乏症。它们对于接受生物合成重组IGF-I治疗的拉伦综合征儿童的随访也应是有用的。