Macedo Delanie B, Cukier Priscilla, Mendonca Berenice B, Latronico Ana Claudia, Brito Vinicius Nahime
Laboratório de Hormônios e Genética Molecular LIM/42, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Arq Bras Endocrinol Metabol. 2014 Mar;58(2):108-17. doi: 10.1590/0004-2730000002931.
The onset of puberty is first detected as an increase in the amplitude and frequency of pulses of gonadotropin-releasing hormone (GnRH) after a quiescent period during childhood. The reemergence of pulsatile GnRH secretion leads to increases in the secretion of the gonadotropins, luteinizing hormone (LH), and follicle-stimulating hormone (FSH) by the pituitary gland, and the consequent activation of gonadal function. Early activation of the hypothalamic-pituitary-gonadal axis results in gonadotropin-dependent precocious puberty, also known as central precocious puberty (CPP), which is clinically defined by the development of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys. Pubertal timing is influenced by complex interactions among genetic, nutritional, environmental, and socioeconomic factors. CPP is diagnosed on the basis of clinical signs of progressive pubertal development before the age of 8 years in girls and 9 years in boys, pubertal basal and/or GnRH-stimulated LH levels, and advanced bone age. Magnetic resonance imaging of the central nervous system is essential for establishing the CPP form as organic or idiopathic. Depot GnRH-analogues represent the first-line of therapy in CPP. Very recently, the genetic component of CPP was demonstrated by the evidence that the deficiency of the MKRN3 gene, located on long arm of chromosome 15, causes familial CPP in humans. In this current review, clinical and therapeutic aspects of the CPP will be discussed, contributing to adequate diagnosis and criterious approach of this relevant condition of pediatric endocrinology.
青春期的开始首先表现为在童年静止期后促性腺激素释放激素（GnRH）脉冲的幅度和频率增加。GnRH脉冲式分泌的重新出现导致垂体分泌促性腺激素、黄体生成素（LH）和卵泡刺激素（FSH）增加，从而激活性腺功能。下丘脑-垂体-性腺轴的过早激活导致促性腺激素依赖性性早熟，也称为中枢性性早熟（CPP），临床上定义为女孩在8岁前、男孩在9岁前出现第二性征发育。青春期时间受遗传、营养、环境和社会经济因素之间复杂相互作用的影响。CPP的诊断基于女孩8岁前、男孩9岁前青春期进行性发育的临床体征、青春期基础和/或GnRH刺激后的LH水平以及骨龄提前。中枢神经系统的磁共振成像对于确定CPP是器质性还是特发性至关重要。长效GnRH类似物是CPP治疗的一线药物。最近，位于15号染色体长臂上的MKRN3基因缺陷导致人类家族性CPP这一证据证明了CPP的遗传成分。在本综述中，将讨论CPP的临床和治疗方面，有助于对小儿内分泌学这一相关病症进行充分诊断和谨慎处理。
