中枢性性早熟患儿身高改善的研究进展

Research progress of height improvement in central precocious puberty

中枢性性早熟(central precocious puberty,CPP)是下丘脑-垂体-性腺轴提前启动(女孩8岁前,男孩9岁前)引起的提前出现第二性征的疾病.CPP的发病机制与遗传、种族、环境因素有关.促性腺激素释放激素类似物(gonadotropin-releasing hormone analogues,GnRHa)是治疗CPP的常用药物.GnRHa改善CPP身高的影响因素包括CPP的发病年龄和干预年龄.女孩6岁前确诊并治疗的CPP病例效果最佳,故CPP需早治疗.足够的疗程是保证身高改善的重要因素,至少需要2年.骨龄越小,对于身高改善越有利,芳香化酶抑制剂通过延缓骨骺闭合可改善CPP男孩的身高.KISS1、KISS1R、MKRN3等基因与CPP有关,早期识别可以对早期干预起指导作用.用GnRHa治疗的CPP女孩,身高增长速度小于4 cm/年,联用基因重组人生长激素可有效改善成年身高.适当有氧运动亦可改善身高.该文就CPP身高改善影响因素及策略的研究现状作一综述.

Central precocious puberty (CPP) is a disease that results from the premature activation of hypothalamic-pituitary-gonadal axis(before 8 years in girls and 9 years in boys).The CPP children show abnormally early appearance of secondary sexual characteristics.Genetic factors,ethnicity,environmenal factors have been involved in the pathogenesis of CPP.Gonadotropin-releasing hormone analogues(GnRHa) are the most common drugs for CPP.The factors of GnRHa treatment on height improvement include the age of puberty onset and the age of treatment initiation.The CPP girls diagnosed and treated before the age of 6 have the best effect,so CPP children need early treatment.Adequate treatment duration is an important factor to ensure height improvement,at least 2 years.The CPP children that are younger according to the bone age have more favorable outcomes of height improvement.By delaying epiphyseal closure,aromatase inhibitors can improve the height of CPP boys.KISS1 、KISS1R 、MKRN3 and other genes are related to CPP,early identification of CPP genes can play a guiding role in early intervention.For the CPP girls treated with GnRHa,when the growth rate is less than 4cm/year,recombinant human growth hormone can effectively improve adult height.Appropriate aerobic exercise can also improve height.This article reviews the factors and strategies of height improvement in central precocious puberty.