Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia
Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia
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DOI：
10.1159/000249160
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发表时间：
2009-01-01
期刊：
影响因子：
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通讯作者：
Bas, Firdevs
中科院分区：
文献类型：
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作者：
Tutunculer, Filiz;Saka, Nurcin;Bas, Firdevs
背景/目的：先天性肾上腺皮质增生（CAH）的特征是肾上腺功能不全伴或不伴盐耗。它还伴有肾上腺髓质功能减退。本研究的目的是调查肾上腺髓质功能的CAH患者由于21-羟化酶和11 β-羟化酶缺陷和年龄匹配的正常人。研究方法：我们测量了44例CAH患者的血浆儿茶酚胺（肾上腺素和去甲肾上腺素）和尿代谢产物，其中32例是由于21-羟化酶缺乏（17例为盐耗型，15例为单纯男性化型），12例是由于11 β-羟化酶缺乏，25例为健康对照。结果如下：对照组血浆肾上腺素和尿甲肾上腺素水平显著高于CAH患者（分别为p = 0.02和p < 0.001）。对照组血浆去甲肾上腺素水平显著低于CAH患者（p < 0.001）。有趣的是，与CAH的其他亚组相比，盐耗型患者的去甲肾上腺素水平较低。结论：尽管CAH患者肾上腺素分泌不足，但这些患者有能力增加代偿性去甲肾上腺素。然而，这种增加在盐耗型患者中要低得多。这些发现还需要其他研究来证实。版权所有（C）2009 S. Karger AG，巴塞尔
Background/Aims: Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11 beta-hydroxylase deficiencies and in age-matched normal subjects. Methods: We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11 beta-hydroxylase deficiency, and in 25 healthy controls. Results: Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p < 0.001, respectively). Plasma norepinephrine levels were significantly lower in the controls than in patients with CAH (p < 0.001). Interestingly, patients with the salt-wasting form had lower norepinephrine levels in comparison to the other subgroups of CAH. Conclusion: Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies. Copyright (C) 2009 S. Karger AG, Basel