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Constitutional delay of puberty versus congenital hypogonadotropic hypogonadism: Genetics, management and updates.
Best Practice & Research Clinical Endocrinology & Metabolism ( IF 6.1 ) Pub Date : 2019-09-05 , DOI: 10.1016/j.beem.2019.101316
Taneli Raivio 1 , Päivi J Miettinen 1
Affiliation  

Delayed puberty (DP) affects approximately 2% of adolescents. In the vast majority of patients in both sexes, it is due to constitutional delay of growth and puberty (CDGP), a self-limited condition in which puberty starts later than usual but progresses normally. However, some CDGP patients may benefit from medical intervention with low-dose sex steroids or peroral aromatase inhibitor letrozole (only for boys). Other causes of DP include permanent hypogonadotropic hypogonadism, functional hypogonadotropic hypogonadism (due to chronic diseases and conditions), and gonadal failure. In this review we discuss these themes along with the latest achievements in the field of puberty research, and include a brief synopsis on the differential diagnosis and management of patients with CDGP and congenital hypogonadotropic hypogonadism.

中文翻译:

青春期与先天性性腺功能低下性腺功能减退的体质延迟:遗传学,管理和更新。

青春期延迟(DP)影响约2%的青少年。在绝大多数男女患者中,这是由于生长和青春期的体质延迟(CDGP)所致,这是一种自我限制的状况,其中青春期比平常开始晚,但正常发展。但是,一些CDGP患者可能会接受低剂量性类固醇或经口芳香化酶抑制剂来曲唑(仅对男孩)的医学干预而受益。DP的其他原因包括永久性性腺功能低下性腺功能减退,功能性性腺功能低下性腺功能减退(由于慢性疾病和状况)和性腺衰竭。在这篇综述中,我们讨论了这些主题以及青春期研究领域的最新成果,并简要概述了CDGP和先天性促性腺激素性性腺功能减退症患者的鉴别诊断和治疗。
更新日期:2019-09-05
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