Children's Hospital of Philadelphia, Endocrinology and Diabetes, Philadelphia, PA 19104, USA.
J Clin Endocrinol Metab. 2023 Jul 14;108(8):2115-2123. doi: 10.1210/clinem/dgad081.
Central precocious puberty (CPP) classically refers to premature activation of the hypothalamic-pituitary-gonadal axis with onset of sexual development before the age of 8 years in girls and 9 years in boys. A decrease in the age of thelarche has been reported over the past several decades; however, the tempo of pubertal progression can be slower and adult height may not be adversely affected in many of the girls who experience thelarche at 6-8 years. Outside of this secular trend in the development itself, the past several decades have also brought about advances in diagnosis and management. This includes the widespread use of an ultrasensitive luteinizing hormone assay, decreasing the need for stimulation testing and a better understanding of the genetics that govern the onset of puberty. Additionally, management of CPP using gonadotropin-releasing hormone analogs (GnRHas) has changed with the advent of new longer-acting formulations. Emerging long-term outcomes of GnRHa administration with regards to obesity, cardiovascular risk factors and fertility are reassuring. Despite these advancements, clinical care in CPP is hampered by the lack of well-designed controlled studies, and management decisions are frequently not supported by clear practice guidelines. Data in boys with CPP are limited and this article focuses on the diagnosis and management of CPP in girls, particularly, in those who present with thelarche at the age of 6-8 years.
中枢性性早熟(CPP)通常是指下丘脑-垂体-性腺轴过早激活,女孩在 8 岁前、男孩在 9 岁前出现性发育。过去几十年,性成熟年龄提前的报道有所增加;然而,在许多 6-8 岁出现性早熟的女孩中,青春期进展的速度可能较慢,成年身高可能不会受到不利影响。除了这种发育本身的季节性趋势外,过去几十年在诊断和治疗方面也取得了进展。这包括广泛使用超敏黄体生成素测定,减少了对刺激试验的需求,以及对控制青春期开始的遗传因素的更好理解。此外,随着新型长效制剂的出现,使用促性腺激素释放激素类似物(GnRHa)治疗 CPP 也发生了变化。GnRHa 给药对肥胖、心血管危险因素和生育能力的新兴长期影响令人安心。尽管取得了这些进展,但 CPP 的临床治疗受到缺乏精心设计的对照研究的阻碍,治疗决策通常得不到明确的临床实践指南的支持。CPP 男孩的数据有限,本文重点介绍女孩 CPP 的诊断和治疗,特别是那些在 6-8 岁出现性早熟的女孩。