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意大利罗马萨皮恩扎大学儿科医院性早熟的临床管理与治疗

Clinical Management and Therapy of Precocious Puberty in the Sapienza University Pediatrics Hospital of Rome, Italy.

作者信息

Micangeli Ginevra, Paparella Roberto, Tarani Francesca, Menghi Michela, Ferraguti Giampiero, Carlomagno Francesco, Spaziani Matteo, Pucarelli Ida, Greco Antonio, Fiore Marco, Tarani Luigi

机构信息

Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.

Department of Experimental Medicine, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.

出版信息

Children (Basel). 2023 Oct 10;10(10):1672. doi: 10.3390/children10101672.

Abstract

Puberty identifies the transition from childhood to adulthood. Precocious puberty is the onset of signs of pubertal development before age eight in girls and before age nine in boys, it has an incidence of 1/5000-1/10,000 with an F:M ratio ranging from 3:1 to 20:1. Precocious puberty can be divided into central, also known as gonadotropin-dependent precocious puberty or true precocious puberty, and peripheral, also recognized as gonadotropin-independent precocious puberty or precocious pseudopuberty. Thus, the main aim of this narrative report is to describe the standard clinical management and therapy of precocious puberty according to the experience and expertise of pediatricians and pediatric endocrinologists at Policlinico Umberto I, Sapienza University of Rome, Italy. In the suspicion of early sexual maturation, it is important to collect information regarding the age of onset, the speed of maturation of secondary sexual features, exposure to exogenous sex steroids and the presence of neurological symptoms. The objective examination, in addition to the evaluation of secondary sexual characteristics, must also include the evaluation of auxological parameters. Initial laboratory investigations should include serum gonadotropin levels (LH and FSH) and serum levels of the sex steroids. Brain MRI should be performed as indicated by the 2009 Consensus Statement in all boys regardless of chronological age and in all girls with onset of pubertal signs before 6 years of age. The gold standard in the treatment of central precocious puberty is represented by GnRH analogs, whereas, as far as peripheral forms are concerned, the triggering cause must be identified and treated. At the moment there are no reliable data establishing the criteria for discontinuation of GnRH analog therapy. However, numerous pieces of evidence suggest that the therapy should be suspended at the physiological age at which puberty occurs.

摘要

青春期标志着从儿童期到成年期的转变。性早熟是指女孩在8岁前、男孩在9岁前出现青春期发育迹象,其发病率为1/5000 - 1/10000,男女比例为3:1至20:1。性早熟可分为中枢性(也称为促性腺激素依赖性性早熟或真性性早熟)和外周性(也称为促性腺激素非依赖性性早熟或假性性早熟)。因此,本叙述性报告的主要目的是根据意大利罗马第一大学附属翁贝托一世综合医院儿科医生和儿科内分泌学家的经验与专业知识,描述性早熟的标准临床管理和治疗方法。在怀疑有性早熟时,收集有关发病年龄、第二性征成熟速度、外源性性类固醇暴露情况以及神经症状的信息非常重要。客观检查除了评估第二性征外,还必须包括对体格发育参数的评估。初步实验室检查应包括血清促性腺激素水平(LH和FSH)以及性类固醇的血清水平。对于所有男孩,无论实际年龄如何,以及所有在6岁前出现青春期体征的女孩,均应按照2009年共识声明的指示进行脑部MRI检查。中枢性性早熟治疗的金标准是促性腺激素释放激素类似物,而对于外周性类型,必须找出并治疗引发原因。目前尚无确定促性腺激素释放激素类似物治疗停药标准的可靠数据。然而,大量证据表明,该治疗应在青春期出现的生理年龄时暂停。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b41/10604951/e7d8d5f38f06/children-10-01672-g001.jpg

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