Stoupa Athanasia, González-Briceño Laura, Pinto Graziella, Samara-Boustani Dinane, Thalassinos Caroline, Flechtner Isabelle, Beltrand Jacques, Bidet Maud, Simon Albane, Piketty Marie, Laborde Kathleen, Morel Yves, Bellanné-Chantelot Christine, Touraine Philippe, Polak Michel
Service d'Endocrinologie, Gynécologie et Diabétologie Pédiatriques, Hôpital Universitaire Necker-Enfants Malades, Paris, France.
Horm Res Paediatr. 2015;83(4):262-7. doi: 10.1159/000369901. Epub 2015 Feb 7.
To describe cortisol response to tetracosactide and to review the literature on adrenal function in non-classic congenital adrenal hyperplasia (NCCAH) patients.
We compared cortisol responses to tetracosactide (250 μg) between NCCAH patients and a comparison group (CG) of patients with premature pubarche and normal tetracosactide test. An adequate cortisol response was defined as a peak ≥18 μg/dl.
We included 35 NCCAH patients (26 girls, 9 boys), whose mean age at testing was 7.0 years (0.8-15.6), and 47 patients in the CG (39 girls, 8 boys), whose mean age was 7.2 years (0.5-9.9). Baseline cortisol was significantly higher in the NCCAH group than in the CG [12.9 (4.3-22.2) vs. 9.7 (4.2-16.2) μg/dl, respectively; p = 0.0006]. NCCAH patients had lower cortisol peak response compared to the CG [18.2 (6.3-40) vs. 24.9 (12-30.3) μg/dl, respectively; p < 0.0001]. Peak cortisol was <18 μg/dl in 21/35 (60%) NCCAH patients versus 1/47 (2.1%) in the CG. No NCCAH patients had acute adrenal insufficiency, but 2 reported severe fatigue that improved with hydrocortisone.
The cortisol response to tetracosactide was inadequate (<18 μg/dl) in 60% of patients with NCCAH. Hydrocortisone therapy may deserve consideration when major stress (surgery, trauma, childbirth) or objectively documented fatigue occurs in NCCAH patients with inadequate cortisol response.
描述皮质醇对二十四肽促肾上腺皮质激素的反应，并综述非经典型先天性肾上腺皮质增生症（NCCAH）患者肾上腺功能的相关文献。
我们比较了NCCAH患者与青春期早熟且二十四肽促肾上腺皮质激素试验正常的对照组（CG）患者对二十四肽促肾上腺皮质激素（250μg）的皮质醇反应。皮质醇反应充分定义为峰值≥18μg/dl。
我们纳入了35例NCCAH患者（26例女孩，9例男孩），检测时的平均年龄为7.0岁（0.8 - 15.6岁），以及47例CG患者（39例女孩，8例男孩），平均年龄为7.2岁（0.5 - 9.9岁）。NCCAH组的基线皮质醇显著高于CG组[分别为12.9（4.3 - 22.2）μg/dl和9.7（4.2 - 16.2）μg/dl；p = 0.0006]。与CG组相比，NCCAH患者的皮质醇峰值反应较低[分别为18.2（6.3 - 40）μg/dl和24.9（12 - 30.3）μg/dl；p < 0.0001]。21/35（60%）的NCCAH患者皮质醇峰值<18μg/dl，而CG组为1/47（2.1%）。没有NCCAH患者出现急性肾上腺功能不全，但有2例报告严重疲劳，氢化可的松治疗后有所改善。
60%的NCCAH患者对二十四肽促肾上腺皮质激素的皮质醇反应不足（<18μg/dl）。当NCCAH患者出现严重应激（手术、创伤、分娩）或客观记录的疲劳且皮质醇反应不足时，可能值得考虑氢化可的松治疗。
