外周性早熟的治疗。

Melissa J Schoelwer, E. Eugster
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引用次数: 35

摘要

周围性性早熟(PPP)的病因很多,表现多样,暴露于雄激素、雌激素或两者兼而有之。临床表现取决于潜在的过程,可能是急性或渐进的。治疗的主要目标是停止青春期的发育,使性类固醇恢复到青春期前的水平。衰减线性生长速度和骨骼成熟率,以最大限度地提高身高潜力是许多患者的额外考虑因素。mcune - albright综合征(MAS)和家族性男性性早熟(FMPP)分别由GNAS1和LH受体基因激活突变引起,是PPP的罕见病因。针对这两种情况,已经研究了几种不同的治疗方法,取得了不同的成功。迄今为止的经验表明,女孩继发于MAS的性早熟的理想治疗方法仍然难以捉摸。相比之下,虽然接受治疗的患者数量仍然很少,但有几种成功的FMPP治疗方案可供选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Treatment of Peripheral Precocious Puberty.
There are many etiologies of peripheral precocious puberty (PPP) with diverse manifestations resulting from exposure to androgens, estrogens, or both. The clinical presentation depends on the underlying process and may be acute or gradual. The primary goals of therapy are to halt pubertal development and restore sex steroids to prepubertal values. Attenuation of linear growth velocity and rate of skeletal maturation in order to maximize height potential are additional considerations for many patients. McCune-Albright syndrome (MAS) and familial male-limited precocious puberty (FMPP) represent rare causes of PPP that arise from activating mutations in GNAS1 and the LH receptor gene, respectively. Several different therapeutic approaches have been investigated for both conditions with variable success. Experience to date suggests that the ideal therapy for precocious puberty secondary to MAS in girls remains elusive. In contrast, while the number of treated patients remains small, several successful therapeutic options for FMPP are available.
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