Laron Z
Endocrinology and Diabetes Research Unit, WHO Collaborating Center for the Study of Diabetes in Youth, Schneider Children's Medical Center, Tel Aviv University, 14 Kaplan Street, Petah Tikva 49202, Tel Aviv, Israel.
Mol Pathol. 2001 Oct;54(5):311-6. doi: 10.1136/mp.54.5.311.
To contribute to the debate about whether growth hormone (GH) and insulin-like growth factor 1 (IGF-1) act independently on the growth process.
To describe growth in human and animal models of isolated IGF-1 deficiency (IGHD), such as in Laron syndrome (LS; primary IGF-1 deficiency and GH resistance) and IGF-1 gene or GH receptor gene knockout (KO) mice.
Since the description of LS in 1966, 51 patients were followed, many since infancy. Newborns with LS are shorter (42-47 cm) than healthy babies (49-52 cm), suggesting that IGF-1 has some influence on intrauterine growth. Newborn mice with IGF-1 gene KO are 30% smaller. The postnatal growth rate of patients with LS is very slow, the distance from the lowest normal centile increasing progressively. If untreated, the final height is 100-136 cm for female and 109-138 cm for male patients. They have acromicia, organomicria including the brain, heart, gonads, genitalia, and retardation of skeletal maturation. The availability of biosynthetic IGF-1 since 1988 has enabled it to be administered to children with LS. It accelerated linear growth rates to 8-9 cm in the first year of treatment, compared with 10-12 cm/year during GH treatment of IGHD. The growth rate in following years was 5-6.5 cm/year.
IGF-1 is an important growth hormone, mediating the protein anabolic and linear growth promoting effect of pituitary GH. It has a GH independent growth stimulating effect, which with respect to cartilage cells is possibly optimised by the synergistic action with GH.
参与关于生长激素（GH）和胰岛素样生长因子1（IGF-1）是否独立作用于生长过程的争论。
描述孤立性IGF-1缺乏（IGHD）的人类和动物模型中的生长情况，如拉伦综合征（LS；原发性IGF-1缺乏和GH抵抗）以及IGF-1基因或GH受体基因敲除（KO）小鼠。
自1966年描述LS以来，对51例患者进行了随访，许多患者从婴儿期就开始跟踪。患有LS的新生儿比健康婴儿（49 - 52厘米）更矮（42 - 47厘米），这表明IGF-1对子宫内生长有一定影响。IGF-1基因敲除的新生小鼠体型小30%。LS患者的出生后生长速度非常缓慢，与最低正常百分位数的差距逐渐增大。如果不进行治疗，女性患者的最终身高为100 - 136厘米，男性患者为109 - 138厘米。他们有小肢畸形、包括脑、心脏、性腺、生殖器在内的器官过小，以及骨骼成熟延迟。自1988年以来生物合成IGF-1的可得性使其能够用于治疗LS患儿。在治疗的第一年，它使线性生长速度加快到8 - 9厘米，而IGHD患者接受GH治疗时的生长速度为每年10 - 12厘米。随后几年的生长速度为每年5 - 6.5厘米。
IGF-1是一种重要的生长激素，介导垂体GH的蛋白质合成代谢和促进线性生长的作用。它具有独立于GH的生长刺激作用，就软骨细胞而言，可能通过与GH的协同作用而得到优化。
