Fanta Michael, Cibula David, Vrbíková Jana
Dept. of Gynecology and Obstetrics, 1st Medical Faculty of Charles University and General Faculty Hospital, Praha 2, Czech Republic.
Gynecol Endocrinol. 2008 Mar;24(3):154-7. doi: 10.1080/09513590801911992.
The clinical symptoms of nonclassic adrenal hyperplasia (NCAH) are identical with polycystic ovary syndrome (PCOS). The aim of our study was to determine the prevalence of nonclassic adrenal hyperplasia (21-hydroxylase-deficiency) in hyperandrogenic women, its biochemical, endocrine and clinical characteristics and to compare them with parameters of patients with ovarian hyperandrogenism.
Since 1999, 298 patients with elevation of at least one androgen and manifestation of one of the clinical androgenic symptoms (oligo/amenorrhea, hirsutism or acne) have been identified in our database. A diagnosis of NCAH was considered when the basal or stimulated 17-hydroxyprogesterone was elevated.
Only eight patients were identified as having 21- hydroxylase deficient NCAH in the whole group of 298 hyperandrogenic women. Hirsutism and acne were found only in three, two patients, five of them had oligo/amenorrhea. Seven patients had both elevated basal and stimulated 17-hydroxyprogesterone, while in one case only elevation of stimulated level was found. All of the NCAH patients had elevated concentrations of testosterone, six DHEA, lower SHBG was found in four patients. Surprisingly, none of the NCAH patients had increased DHEAS.
In our study, the prevalence of NCAH in hyperandrogenic women was 2.68%. Their leading symptom was oligomenorrhea, skin androgenic disorders were a minor clinical problem. None of the NCAH patients had an elevated DHEAS, the androgen dominantly produced by the adrenal glands.
非经典型肾上腺增生(NCAH)的临床症状与多囊卵巢综合征(PCOS)相同。我们研究的目的是确定高雄激素血症女性中非经典型肾上腺增生(21-羟化酶缺乏)的患病率、其生化、内分泌和临床特征,并将其与卵巢高雄激素血症患者的参数进行比较。
自1999年以来,我们在数据库中识别出298例至少一种雄激素水平升高且有临床雄激素症状(少经/闭经、多毛或痤疮)之一的患者。当基础或刺激后的17-羟孕酮升高时,考虑诊断为NCAH。
在298例高雄激素血症女性的整个队列中,仅8例被确定为患有21-羟化酶缺乏的NCAH。多毛和痤疮仅分别在3例、2例患者中出现,其中5例有少经/闭经。7例患者基础和刺激后的17-羟孕酮均升高,而1例仅刺激水平升高。所有NCAH患者的睾酮浓度均升高,6例硫酸脱氢表雄酮升高,4例患者的性激素结合球蛋白降低。令人惊讶的是,所有NCAH患者的硫酸脱氢表雄酮均未升高。
在我们的研究中,高雄激素血症女性中NCAH的患病率为2.68%。她们的主要症状是月经过少,皮肤雄激素紊乱是一个较小的临床问题。所有NCAH患者的肾上腺主要产生的雄激素硫酸脱氢表雄酮均未升高。