Taulier-Raybaud C, Morel Y, La Selve H, Tran Minh V, Sempé M, David M
Pediatrie. 1986 Dec;41(8):607-16.
A "transient" precocious puberty with ovarian follicular cysts was observed in four girls. Each girl presented with several successive and transient episodes of development of secondary sex characters: breast development, areolar pigmentation, brown abdominal median line were the clinical signs of oestrogen secretion. At the time of referral, plasma and urinary gonadotropins levels were low in the prepubertal range while plasma oestradiol concentrations were elevated, in the pubertal range. At this time, as well as during each episode of "pubertal" development, the gonadotropins response to GnRH stimulation was blunted. In contrast, a prepubertal or pubertal response was observed when the clinical symptoms of estrogenization had resumed. Ultrasonography showed one or several ovarian follicular cysts. In two cases, the treatment with the GnRH agonist, D-TRP 6 GnRH [Decapeptyl (R)], was successful, suggesting a central dysfunction
在4名女孩中观察到伴有卵巢滤泡囊肿的“暂时性”性早熟。每个女孩都出现了几次继发性征发育的连续且短暂的发作:乳房发育、乳晕色素沉着、腹部正中褐色线是雌激素分泌的临床体征。转诊时,血浆和尿促性腺激素水平处于青春期前范围的低水平,而血浆雌二醇浓度升高,处于青春期范围。此时以及在每一次“青春期”发育发作期间,促性腺激素对GnRH刺激的反应减弱。相比之下,当雌激素化的临床症状恢复时,观察到青春期前或青春期的反应。超声检查显示有一个或几个卵巢滤泡囊肿。在两例病例中,使用GnRH激动剂D-TRP 6 GnRH [曲普瑞林(R)]治疗成功,提示存在中枢功能障碍