Hague W M, Honour J W, Adams J, Vecsei P, Jacobs H S
Endocrine Unit, Middlesex Hospital, London, UK.
Clin Endocrinol (Oxf). 1989 Apr;30(4):355-65. doi: 10.1111/j.1365-2265.1989.tb00433.x.
Sixty-three women with ultrasonically detected polycystic ovaries (PCO) were investigated for a disorder of adrenal steroid biosynthesis. Serum was obtained before, and at 30 and 60 min after, the administration of 250 micrograms tetracosactrin, and assayed for 17 alpha-OH-progesterone, 21-deoxycortisol, 17 alpha-OH-pregnenolone and dehydroepiandrosterone by radioimmunoassay following paper chromatography. Results were compared with those in 11 women with normal ovaries, seven adult females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD), and 15 women heterozygous for this defect. Although the basal-peak steroid concentration differences were significantly greater when ACTH tests were conducted between 1400 and 1700 h than between 0900 and 1000 h, absolute peak steroid concentrations were not different at either time of day. Four of 63 (6.4%) women with PCO had responses to ACTH characteristic of non-classical (late onset) 21OHD CAH, and about half the remainder had responses characteristic of 21OHD heterozygotes. There was no clear cut evidence for a deficiency in 3 beta-hydroxysteroid dehydrogenase activity in women with PCO.
对63名经超声检查发现有多囊卵巢(PCO)的女性进行了肾上腺类固醇生物合成紊乱的研究。在静脉注射250微克二十四肽促皮质素前、注射后30分钟和60分钟采集血清,经纸层析后采用放射免疫分析法检测血清中17α-羟孕酮、21-脱氧皮质醇、17α-羟孕烯醇酮和脱氢表雄酮的含量。将结果与11名卵巢正常的女性、7名因21-羟化酶缺乏(21OHD)导致先天性肾上腺皮质增生(CAH)的成年女性以及15名该缺陷的杂合子女性的结果进行比较。尽管在1400至1700时进行促肾上腺皮质激素(ACTH)试验时,基础-峰值类固醇浓度差异明显大于在0900至1000时进行试验时,但一天中任何一个时间点的绝对峰值类固醇浓度并无差异。63名患有PCO的女性中有4名(6.4%)对ACTH的反应具有非经典型(迟发型)21OHD CAH的特征,其余约一半女性的反应具有21OHD杂合子的特征。没有明确证据表明患有PCO的女性存在3β-羟类固醇脱氢酶活性缺陷。