Maesaka H, Suwa S, Tachibana K, Katsumata N
Pediatr Res. 1985 Dec;19(12):1258-62. doi: 10.1203/00006450-198512000-00007.
We observed prolonged genital bleeding during the first 2-3 months after treatment in five of 13 female patients with salt-losing 21-hydroxylase deficiency. Their relatively low concentrations of serum follicle-stimulating hormone and luteinizing hormone before therapy increased rapidly to high levels which were maintained for 1-3 wk and then decreased. The duration of these relatively high levels after therapy was longer in the patients with genital bleeding than those without. Before therapy, there was no release of serum follicle-stimulating hormone and luteinizing hormone following the administration of synthetic luteinizing hormone-releasing hormone in two patients; 1 month after therapy, the response to luteinizing hormone-releasing hormone increased significantly. Serum estradiol increased above 300 pg/ml in four patients with genital bleeding but was less than 175 pg/ml in three patients without bleeding. The etiology of genital bleeding in these female patients may be more prolonged activation of the hypothalamo-pituitary-ovarian axis and a greater increase in the responsiveness of internal genitalia to gonadotropins and sex hormones, perhaps induced by prolonged exposure to excessive adrenal steroids starting before birth.
在13例失盐型21-羟化酶缺乏症女性患者中，我们观察到5例在治疗后的最初2 - 3个月出现持续性生殖器出血。治疗前她们血清促卵泡生成素和促黄体生成素浓度相对较低，治疗后迅速升至高水平并维持1 - 3周，随后下降。生殖器出血患者治疗后这些相对高水平持续的时间比未出血患者更长。治疗前，2例患者在注射合成促黄体生成素释放激素后血清促卵泡生成素和促黄体生成素无释放；治疗1个月后，对促黄体生成素释放激素的反应显著增强。4例生殖器出血患者血清雌二醇升至300 pg/ml以上，而3例未出血患者血清雌二醇低于175 pg/ml。这些女性患者生殖器出血的病因可能是下丘脑-垂体-卵巢轴的激活时间延长，以及内生殖器对促性腺激素和性激素的反应性增加幅度更大，这可能是由于从出生前开始长期暴露于过量肾上腺类固醇所致。
