Laron Z
Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, Tel Aviv University, Israel.
J Pediatr Endocrinol Metab. 2001;14 Suppl 5:1243-8; discussion 1261-2.
The follow-up of a large cohort of patients with Laron syndrome (LS) from infancy to adult age has enabled us to determine the effects of long-term insulin-like growth factor-I (IGF-I) deficiency on auxological, biochemical, physiological and psychological parameters. We found that early and continuous IGF-I deficiency (the anabolic effector of growth hormone) causes dwarfism, acromicria, organomicria, marked obesity, insulin resistance, retardation of skeletal maturation and osteoporosis, as well as muscular and central nervous tissue underdevelopment, and a series of biochemical changes including hypercholesterolemia. These multiple pathologies impair the quality of life of these patients. It is concluded that patients with LS need IGF-I replacement treatment throughout life.
对一大群从婴儿期到成年期的拉伦综合征（LS）患者进行随访，使我们能够确定长期胰岛素样生长因子-I（IGF-I）缺乏对人体测量学、生化、生理和心理参数的影响。我们发现，早期和持续的IGF-I缺乏（生长激素的合成代谢效应因子）会导致侏儒症、肢端过小、器官过小、明显肥胖、胰岛素抵抗、骨骼成熟延迟和骨质疏松，以及肌肉和中枢神经组织发育不全，还有包括高胆固醇血症在内的一系列生化变化。这些多种病理状况损害了这些患者的生活质量。结论是，LS患者一生都需要IGF-I替代治疗。
