Moreira A C, Elias L L
Department of Medicine, Faculty of Medicine, Ribeirão Preto, SP, Brazil.
J Clin Endocrinol Metab. 1992 Jan;74(1):198-203. doi: 10.1210/jcem.74.1.1309366.
21-Hydroxylase congenital adrenal hyperplasia (21-OHCAH) involves a primary defect of the adrenal gland and a secondary involvement of ACTH secretion. The responses of the pituitary-adrenal axis to ovine CRH (oCRH, 1 micrograms/kg) were examined in subjects with different degrees of 21-OH deficiency. We studied 43 subjects: 7 classical and 6 nonclassical (NC) 21-OHCAH patients, 15 heterozygotes (HT) and 15 control subjects. Baseline plasma ACTH levels were higher in classical CAH than in NC-CAH, HT, and control subjects (mean +/- SEM, 66 +/- 14, 6 +/- 1.6, 4 +/- 0.5, and 5 +/- 0.5 pmol/L, respectively). The mean plasma ACTH response to oCRH in NC-CAH (17 +/- 3 pmol/L) was higher than in controls and HT (9 +/- 0.8 and 11 +/- 1.5 pmol/L). The highest ACTH responses to oCRH were obtained for classical CAH patients (126 +/- 29 pmol/L). Plasma cortisol rose after oCRH in control, HT, and NC-CAH patients but did not change in classical CAH. After oCRH, plasma 17-hydroxyprogesterone (17-OHP) were 4 +/- 0.5, 8 +/- 1.6, 93 +/- 28, and 359 +/- 110 nmol/L for controls, HT, NC-CAH, and classical patients, respectively. There was a significant correlation (r = 0.70) between 17-OHP and the ACTH responses to oCRH. The 17-OHP responses to oCRH were also correlated (r = 0.94) with the 17-OHP responses to the synthetic ACTH test. We conclude that the release of endogenous ACTH by oCRH result in graded 17-OHP responses on 21-OH deficiency. The present study also suggests that different degrees of adrenal biosynthetic defect may result in graded ACTH responses to oCRH.
21-羟化酶先天性肾上腺增生（21-OHCAH）涉及肾上腺的原发性缺陷以及促肾上腺皮质激素（ACTH）分泌的继发性改变。我们在不同程度21-羟化酶缺乏的受试者中检测了垂体-肾上腺轴对羊促肾上腺皮质激素释放激素（oCRH，1微克/千克）的反应。我们研究了43名受试者：7名典型21-OHCAH患者、6名非典型（NC）21-OHCAH患者、15名杂合子（HT）以及15名对照受试者。典型先天性肾上腺增生（CAH）患者的基础血浆ACTH水平高于非典型CAH、杂合子及对照受试者（均值±标准误分别为66±14、6±1.6、4±0.5和5±0.5皮摩尔/升）。非典型CAH患者对oCRH的血浆ACTH平均反应（17±3皮摩尔/升）高于对照和杂合子（9±0.8和11±1.5皮摩尔/升）。典型CAH患者对oCRH的ACTH反应最高（126±29皮摩尔/升）。对照、杂合子及非典型CAH患者在注射oCRH后血浆皮质醇升高，但典型CAH患者无变化。注射oCRH后，对照、杂合子、非典型CAH及典型患者的血浆17-羟孕酮（17-OHP）分别为4±0.5、8±1.6、93±28和359±110纳摩尔/升。17-OHP与对oCRH的ACTH反应之间存在显著相关性（r = 0.70）。17-OHP对oCRH的反应也与对合成ACTH试验的17-OHP反应相关（r = 0.94）。我们得出结论，oCRH释放内源性ACTH会导致在21-羟化酶缺乏时出现分级的17-OHP反应。本研究还表明，不同程度的肾上腺生物合成缺陷可能导致对oCRH的ACTH反应分级。
