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性早熟的诊断与管理

Diagnosis and management of precocious puberty.

作者信息

Wheeler M D, Styne D M

机构信息

Department of Pediatrics, University of California, School of Medicine, Davis.

出版信息

Pediatr Clin North Am. 1990 Dec;37(6):1255-71. doi: 10.1016/s0031-3955(16)37010-9.

Abstract

The onset of pubertal development before the age of 8 years in girls or 9 years in boys constitutes precocious puberty. There are numerous causes of precocious puberty, which can be classified as central or peripheral precocious puberty. Central precocious puberty results from premature activation of the hypothalamic-pituitary-gonadal axis and thus presents with physical and hormonal findings similar to those found in normal puberty. Peripheral precocious puberty results from extrapituitary gonadotropin secretion or secretion of sex steroids independent of pituitary gonadotropins. All types of precocious puberty are characterized by rapid growth and advancement of skeletal age, leading to the paradox of the tall child becoming a short adult as a result of premature epiphyseal fusion. Long-acting GnRH agonists afford effective, selective, and reversible therapy of central precocious puberty without significant toxicity. GnRH agonists are not effective in managing the premature sexual maturation associated with peripheral precocious puberty, but a number of other agents have been used with some success. These agents include testolactone, ketoconazole, and medroxyprogesterone acetate. GnRH agonist treatment leads to an increase in predicted final height. To determine the true benefit of any of these agents in increasing ultimate height, there is a need for continuing studies in treated cohorts to follow growth patterns until adult stature is achieved.

摘要

女孩在8岁前或男孩在9岁前出现青春期发育即构成性早熟。性早熟有多种病因,可分为中枢性或外周性性早熟。中枢性性早熟是由下丘脑 - 垂体 - 性腺轴过早激活引起的,因此其身体和激素表现与正常青春期相似。外周性性早熟是由垂体外促性腺激素分泌或独立于垂体促性腺激素的性类固醇分泌引起的。所有类型的性早熟都以生长迅速和骨龄提前为特征,由于骨骺过早融合,导致出现高个子儿童成年后变矮的矛盾现象。长效促性腺激素释放激素(GnRH)激动剂可为中枢性性早熟提供有效、选择性和可逆的治疗,且无明显毒性。GnRH激动剂对治疗与外周性性早熟相关的过早性成熟无效,但已使用一些其他药物并取得了一定成功。这些药物包括睾内酯、酮康唑和醋酸甲羟孕酮。GnRH激动剂治疗可使预测的最终身高增加。为了确定这些药物中任何一种在增加最终身高方面的真正益处,需要对接受治疗的队列进行持续研究,以跟踪生长模式直至达到成人身高。

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