Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Charitéplatz 1, 10117 Berlin, Germany.
Int J Endocrinol. 2014;2014:469289. doi: 10.1155/2014/469289. Epub 2014 Feb 10.
Introduction. Men with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency show impaired fecundity due to testicular adrenal rest tumors and/or suppression of the gonadal axis. Sexual well-being might be an additional factor; however, no data exists. Patients and Methods. Prospective longitudinal monocentric study included 20 male CAH patients (14 salt wasting, 6 simple virilizing; age 18-49 yr). Clinical assessment, testicular ultrasound, biochemical and hormonal parameters, three validated self-assessment questionnaires (SF-36, GBB-24, and HADS), and male Brief Sexual Function Inventory (BSFI) were analyzed at baseline and after two years. Results. Basal LH and testosterone levels suggested normal testicular function. LH and FSH responses to GnRH were more pronounced in patients with a good therapy control according to androstenedione/testosterone ratio < 0.2. This group had significant higher percentage of patients on dexamethasone medication. GBB-24, HADS, and SF-36 showed impaired z-scores and no changes at follow-up. BSFI revealed impairments in dimensions "sexual drive," "erections," and "ejaculations," whereas "problem assessment" and "overall satisfaction" revealed normal z-scores. Androstenedione levels correlated (P = 0.036) inversely with z-scores for "sexual drive" with higher levels associated with impaired "sexual drive." Conclusion. Male CAH patients showed a partly impaired sexual well-being which might be an additional factor for reduced fecundity.
简介。由于 21-羟化酶缺乏,患有先天性肾上腺增生症(CAH)的男性由于睾丸肾上腺残余肿瘤和/或性腺轴抑制而表现出生育能力受损。性健康可能是另一个因素;然而,目前尚无相关数据。
患者和方法。前瞻性纵向单中心研究纳入了 20 名男性 CAH 患者(14 名盐耗竭型,6 名单纯男性化型;年龄 18-49 岁)。在基线和两年后,进行临床评估、睾丸超声、生化和激素参数、三个经过验证的自我评估问卷(SF-36、GBB-24 和 HADS)以及男性简短性功能问卷(BSFI)分析。
结果。基础 LH 和睾酮水平提示睾丸功能正常。根据雄烯二酮/睾酮比值<0.2,对 GnRH 的 LH 和 FSH 反应在治疗控制良好的患者中更为明显。该组有更高比例的患者使用地塞米松治疗。GBB-24、HADS 和 SF-36 显示 Z 评分受损,随访时无变化。BSFI 显示“性欲”、“勃起”和“射精”维度受损,而“问题评估”和“总体满意度”则显示正常 Z 评分。雄烯二酮水平与“性欲”的 Z 评分呈负相关(P = 0.036),水平较高与“性欲”受损相关。
结论。男性 CAH 患者的性健康状况部分受损,这可能是生育能力降低的另一个因素。