Bovet P, Reymond M J, Rey F, Gomez F
Département de Médecine Interne, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
J Endocrinol Invest. 1988 Mar;11(3):201-4. doi: 10.1007/BF03350135.
Congenital adrenal hypoplasia (AH) is a rare condition, known to be associated with isolated hypogonadotropic hypogonadism (IHH). Three studies have reported attempts to stimulate gonadotropin secretion with pulsatile gonadotropin-releasing hormone (GnRH) in a total of 4 patients presenting such a syndrome, with conflicting results. In the present study, one patient with idiopathic IHH and AH was treated with pulsatile sc GnRH--doses ranging from 2.5 to 10.0 micrograms/pulse, every 90 min--during 8 weeks in an attempt to induce puberty. The prepubertal basal plasma levels of LH, FSH and testosterone, and saliva testosterone levels remained unaltered throughout treatment, at all doses of GnRH tested. The gonadotropin response to an acute iv GnRH administration (0.1 mg) also remained at the prepubertal level after pulsatile GnRH treatment. No circulating anti-GnRH antibodies were detected. The absence of gonadotropic response to exogenous pulsatile GnRH suggests that the IHH of patients with AH is due to an abnormal pituitary function rather than to a lack of endogenous GnRH.
先天性肾上腺发育不全（AH）是一种罕见病症，已知与孤立性低促性腺激素性性腺功能减退症（IHH）相关。三项研究报告了对总共4例患有此类综合征的患者尝试用脉冲式促性腺激素释放激素（GnRH）刺激促性腺激素分泌的情况，结果相互矛盾。在本研究中，一名患有特发性IHH和AH的患者接受了皮下脉冲式GnRH治疗——剂量为每脉冲2.5至10.0微克，每90分钟一次——为期8周，试图诱导青春期。在整个治疗期间，在所有测试的GnRH剂量下，青春期前基础血浆促黄体生成素（LH）、促卵泡生成素（FSH）和睾酮水平以及唾液睾酮水平均未改变。在脉冲式GnRH治疗后，对急性静脉注射GnRH（0.1毫克）的促性腺激素反应也保持在青春期前水平。未检测到循环抗GnRH抗体。对外源性脉冲式GnRH缺乏促性腺激素反应表明，AH患者的IHH是由于垂体功能异常而非内源性GnRH缺乏所致。
