Department of Pediatric Urology, Jagiellonian University Medical College, Kraków, Poland.
Department of Pediatric Urology, Jagiellonian University Medical College, Kraków, Poland.
J Pediatr Urol. 2020 Aug;16(4):464.e1-464.e6. doi: 10.1016/j.jpurol.2020.05.162. Epub 2020 May 31.
Congenital Adrenal Hyperplasia (CAH) is the most common reason for undifferentiated genital appearance in new-borns. Psychosexual outcome in women with CAH has been rarely evaluated, but it seems to be one of the most important factors determining the indications for the surgical treatment of CAH.
This is to assess sexual function and the health status (HS) in adult females with CAH who had feminizing genitoplasty in childhood.
The protocol was approved by the Ethical Committee, and the hospital database was searched for patients with CAH who had genitoplasty between 1975 and 2000. 57 adult patients were identified, and 9 (18%) patients agreed to participate in the study. Mean age at operation was 5.4 years, and mean follow-up duration was 10.9 years. The Female Sexual Function Index (FSFI) was used to evaluate sexual function, and the 36-item Short Form Health Survey (SF-36) was used to evaluate their health status (HS). A FSFI score < 26,55 was classified as Female Sexual Dysfunction (FSD). The control group consisted of 10 adult female volunteers of comparable age, without any oncological or chronic diseases. Fisher's exact test was used for statistical analysis.
All patients in the CAH group had female gender identity. One was homosexual, and one reported not having any sexual activity. In the control group, all patients had female gender identity. All were heterosexual and one reported not having any sexual activity. The sexual function in five domains and total score were similar in both groups. More pain was reported in the CAH group as compared with the control group, and it was statistically significant. In the CAH group, 5/9 patients had FSD. In the control group, 4/10 patients had FSD. The difference was statistically insignificant (p = 0.66). Mean SF-36 score in the CAH group was 47.1 points, while it was 46.7 points in the control group. The testosterone level in all CAH patients was within the normal range (0.13-1.1 ng/ml). The 17-OH progesterone level was above normal range in 5/9 (55.6%) patients with CAH. All women in the CAH group were hormonally treated. In the control group, all patients had a normal testosterone level (0.15-0.68 ng/ml); the 17-OH progesterone level was in normal range in this group.
We compared our results with the literature data, which used the same questionnaires as in our study.
Health status and sexual function in the traceable CAH patients didn't differ from the control group.
先天性肾上腺增生症（CAH）是新生儿外生殖器未分化的最常见原因。CAH 女性的性心理发展结果很少被评估，但它似乎是决定 CAH 手术治疗指征的最重要因素之一。
本研究旨在评估接受过儿童期女性化生殖器成形术的 CAH 成年女性的性功能和健康状况（HS）。
该方案获得伦理委员会批准，并在医院数据库中搜索 1975 年至 2000 年间接受过生殖器成形术的 CAH 患者。确定了 57 名成年患者，其中 9 名（18%）患者同意参加研究。平均手术年龄为 5.4 岁，平均随访时间为 10.9 年。使用女性性功能指数（FSFI）评估性功能，使用 36 项简短健康调查问卷（SF-36）评估健康状况（HS）。FSFI 评分<26.55 被归类为女性性功能障碍（FSD）。对照组由 10 名年龄相仿、无肿瘤或慢性疾病的成年女性志愿者组成。使用 Fisher 确切检验进行统计学分析。
CAH 组所有患者均为女性性别认同。1 人是同性恋，1 人报告没有任何性行为。对照组所有患者均为女性性别认同。所有均为异性恋，1 人报告没有任何性行为。两组在五个领域和总分的性功能相似。CAH 组报告的疼痛更多，具有统计学意义。CAH 组 5/9 例患者存在 FSD，对照组 4/10 例患者存在 FSD，差异无统计学意义（p=0.66）。CAH 组的平均 SF-36 评分 47.1 分，对照组为 46.7 分。CAH 组所有患者的睾酮水平均在正常范围内（0.13-1.1ng/ml）。5/9 例 CAH 患者的 17-羟孕酮水平高于正常范围。CAH 组所有女性均接受激素治疗。对照组所有患者的睾酮水平均正常（0.15-0.68ng/ml）；该组的 17-羟孕酮水平在正常范围内。
我们将结果与使用与本研究相同问卷的文献数据进行了比较。
可追踪的 CAH 患者的健康状况和性功能与对照组无差异。
