AP-HP, Department of Endocrinology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance, Pitié-Salpêtrière Hospital (Groupe Hospitalier Pitié-Salpêtrière), Université Pierre et Marie Curie, Site Pitié, 47-83 Boulevard de l'Hôpital, 75013 Paris, France.
Eur J Endocrinol. 2012 Oct;167(4):499-505. doi: 10.1530/EJE-12-0454. Epub 2012 Aug 14.
Women with classical congenital adrenal hyperplasia (CAH) exhibit reduced fertility due to several factors including anovulation. This has been attributed to a disturbed gonadotropic axis as in polycystic ovary syndrome (PCOS), but there is no precise evaluation. Our aim was to evaluate the gonadotropic axis and LH pulsatility patterns and to determine factor(s) that could account for the potential abnormality of LH pulsatility.
Case/control study.
Sixteen CAH women (11 with the salt-wasting form and five with the simple virilizing form), aged from 18 to 40 years, and 16 age-matched women, with regular menstrual cycles (28 ± 3 days), were included. LH pulse patterns over 6 h were determined in patients and controls.
No differences were observed between patients and controls in terms of mean LH levels, LH pulse amplitude, or LH frequency. In CAH patients, LH pulsatility patterns were heterogeneous, leading us to perform a clustering analysis of LH data, resulting in a two-cluster partition. Patients in cluster 1 had similar LH pulsatility patterns to the controls. Patients in cluster 2 had: lower LH pulse amplitude and frequency and presented menstrual cycle disturbances more frequently; higher 17-OH progesterone, testosterone, progesterone, and androstenedione levels; and lower FSH levels.
LH pulsatility may be normal in CAH women well controlled by hormonal treatment. Undertreatment is responsible for hypogonadotropic hypogonadism, with low LH pulse levels and frequency, but not PCOS. Suppression of progesterone and androgen concentrations during the follicular phase of the menstrual cycle should be a major objective in these patients.
患有经典型先天性肾上腺皮质增生症（CAH）的女性由于排卵障碍等多种因素导致生育能力下降。这归因于类似于多囊卵巢综合征（PCOS）的促性腺激素轴紊乱，但尚未进行精确评估。我们的目的是评估促性腺激素轴和 LH 脉冲模式，并确定可能导致 LH 脉冲异常的因素。
病例对照研究。
纳入 16 名 CAH 女性（11 名盐皮质激素过多型，5 名单纯男性化型）和 16 名年龄匹配、月经周期规律（28±3 天）的女性作为对照。在患者和对照组中测定 6 小时内 LH 脉冲模式。
患者和对照组的平均 LH 水平、LH 脉冲幅度或 LH 频率无差异。CAH 患者的 LH 脉冲模式存在异质性，因此我们对 LH 数据进行聚类分析，结果分为两群。群 1 的患者 LH 脉冲模式与对照组相似。群 2 的患者 LH 脉冲幅度和频率较低，月经周期紊乱更为常见；17-羟孕酮、睾酮、孕酮和雄烯二酮水平较高，FSH 水平较低。
经激素治疗良好控制的 CAH 女性的 LH 脉冲可能正常。治疗不足导致促性腺激素低下性性腺功能减退症，表现为 LH 脉冲水平和频率降低，但并非 PCOS。在这些患者中，应将降低卵泡期孕激素和雄激素浓度作为主要目标。
