Gordon D, Cohen H N, Beastall G H, Hay I D, Thomson J A
Clin Endocrinol (Oxf). 1984 Dec;21(6):597-603. doi: 10.1111/j.1365-2265.1984.tb01401.x.
A patient with congenital adrenal hypoplasia (AH) and hypogonadotrophic hypogonadism was treated with pulsatile subcutaneous GnRH therapy for 16 weeks in an attempt to induce puberty. No rise in serum LH or FSH concentrations occurred despite increasing doses of GnRH (2.8 micrograms/pulse-22.4 micrograms/pulse). In contrast a similar programme of therapy successfully initiated the biochemical changes of puberty in a patient with Kallmann's syndrome. Both patients before therapy had low basal serum LH and FSH concentrations with blunted LH and FSH responses to GnRH stimulation. After 1 week, serum LH and FSH rose into the normal adult range in the patient with Kallmann's syndrome. This study fails to confirm a previous report which suggested that intermittent low dose GnRH therapy may be of value in inducing puberty in AH. The reasons for the difference of pituitary responsiveness to GnRH in AH and Kallmann's syndrome are unclear at present.
一名患有先天性肾上腺发育不全（AH）和低促性腺激素性性腺功能减退的患者接受了皮下脉冲式GnRH治疗16周，试图诱导青春期发育。尽管GnRH剂量增加（2.8微克/脉冲 - 22.4微克/脉冲），血清LH或FSH浓度并未升高。相比之下，类似的治疗方案成功地在一名卡尔曼综合征患者中引发了青春期的生化变化。两名患者在治疗前基础血清LH和FSH浓度均较低，对GnRH刺激的LH和FSH反应迟钝。1周后，卡尔曼综合征患者的血清LH和FSH升至正常成人范围。本研究未能证实先前的一份报告，该报告表明间歇性低剂量GnRH治疗可能对诱导AH患者青春期发育有价值。目前尚不清楚AH和卡尔曼综合征中垂体对GnRH反应性差异的原因。
