Krysiak Robert, Marek Bogdan, Okopień Bogusław
Klinia Chorób Wewnetrznych i Farmakologii Klinicznej Katedry Farmakologii, Slqski Uniwersytet Medyczny, Katowice.
Endokrynol Pol. 2009 Nov-Dec;60(6):503-14.
The term precocious puberty is defined as the appearance of secondary sex characteristics before the age of 8 in girls and 9 in boys. There are two major forms of premature sexual maturation: gonadotrophin-dependent (central, or 'true' precocious puberty) and gonadotrophin- independent precocious puberty. The latter, also called peripheral precocious puberty, is characterized by increased production of sex steroids, causing the typical physical changes of puberty, in the absence of reactivation of the hypothalamic-pituitary axis. It may result from several different disorders including testotoxicosis, McCune-Albright syndrome, congenital adrenal hyperplasia, adrenal and gonadal tumours. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities have led to earlier intervention in patients with peripheral precocious puberty to prevent the decline in their psychosocial wellbeing, adult height and quality of life. We review the ethiopathogenesis, clinical picture, diagnosis and treatment of various disorders causing peripheral precocious puberty and provide the reader with current recommendations concerning approach to the patient with this health problem.
性早熟这一术语的定义为女孩在8岁前、男孩在9岁前出现第二性征。性早熟主要有两种类型:促性腺激素依赖性(中枢性,或“真性”性早熟)和促性腺激素非依赖性性早熟。后者也称为外周性性早熟,其特征是在不存在下丘脑-垂体轴重新激活的情况下,性类固醇生成增加,导致青春期典型的身体变化。它可能由多种不同疾病引起,包括睾丸中毒症、McCune-Albright综合征、先天性肾上腺皮质增生症、肾上腺和性腺肿瘤。关于症状发病机制的知识积累以及安全、有效治疗方法的发展,已促使对外周性早熟患者进行更早干预,以防止其心理社会幸福感、成人身高和生活质量下降。我们回顾了导致外周性早熟的各种疾病的病因发病机制、临床表现、诊断和治疗,并为读者提供了针对患有此健康问题患者的当前处理建议。