Sippell W G
Department of Paediatrics, Universitäts-Kinderklinik, Kiel, FRG.
Horm Res. 1994;41 Suppl 2:14-5. doi: 10.1159/000183952.
Central precocious puberty is characterized by premature activation of the hypothalamic gonadotrophin-releasing hormone (GnRH) pulse generator, resulting in accelerated growth and bone maturation with clinical signs of gonadarche before the age of 8 years in girls and 9 years in boys. In rapidly progressing central precocious puberty, the plasma luteinizing hormone (LH) response to exogenous GnRH exceeds the reference range for sex and stage of puberty, and the ratio of LH:follicle-stimulating hormone after GnRH is usually above 1. Short adult stature, with unfavourable body proportions, i.e. an increased upper:lower segment ratio, is probably the only handicap of idiopathic central precocious puberty that persists into adulthood. This cannot be avoided by treatment with either medroxyprogesterone or cyproterone acetate. Following the first report of pituitary-gonadal suppression in a 2-year-old girl with central precocious puberty using daily injections of the gonadotrophin-releasing hormone (GnRH) agonist deslorelin, various short-acting GnRH agonists have been studied in children with central precocious puberty. Some of the older patients treated for shorter periods have now reached adult or near-final height. By 1985, slow-release (depot) GnRH agonists, such as triptorelin and later leuprorelin, were available. These are injected only once every 4 weeks instead of daily or given by nasal spray six times daily, making long-term treatment much more acceptable to patients and their parents. Moreover, suppression of gonadarche is more complete with long-acting (e.g. triptorelin) compared with short-acting (e.g. buserelin) agonists, resulting in significantly slower bone age progression and, thus, greater improvement in predicted, and probably also in achieved, final height.(ABSTRACT TRUNCATED AT 250 WORDS)
中枢性性早熟的特征是下丘脑促性腺激素释放激素(GnRH)脉冲发生器过早激活,导致生长加速和骨骼成熟,并在女孩8岁、男孩9岁之前出现性腺发育的临床体征。在快速进展的中枢性性早熟中,血浆促黄体生成素(LH)对外源性GnRH的反应超过了青春期性别和阶段的参考范围,GnRH刺激后LH与促卵泡生成素的比值通常高于1。身材矮小且身体比例不佳,即上下身比例增加,可能是特发性中枢性性早熟唯一持续到成年期的不利因素。用甲羟孕酮或醋酸环丙孕酮治疗无法避免这种情况。在首例使用促性腺激素释放激素(GnRH)激动剂地洛瑞林每日注射治疗一名2岁中枢性性早熟女孩的垂体-性腺抑制报告之后,各种短效GnRH激动剂已在中枢性性早熟儿童中进行了研究。一些接受较短疗程治疗的年长患者现已达到成人身高或接近最终身高。到1985年,长效(缓释)GnRH激动剂,如曲普瑞林和后来的亮丙瑞林问世。这些药物只需每4周注射一次,而不是每日注射或每日经鼻喷雾给药6次,这使得长期治疗对患者及其父母来说更容易接受。此外,与短效激动剂(如布舍瑞林)相比,长效激动剂(如曲普瑞林)对性腺发育的抑制更完全,导致骨龄进展明显更慢,因此预测的最终身高,可能还有实际达到的最终身高,都有更大改善。(摘要截选至250词)