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先天性肾上腺皮质增生症中女性性功能的多维度研究:一项病例对照研究。

Multidimensional Aspects of Female Sexual Function in Congenital Adrenal Hyperplasia: A Case-Control Study.

作者信息

Dwiggins Maggie, Brookner Brittany, Fowler Kylie, Veeraraghavan Padmasree, Gomez-Lobo Veronica, Merke Deborah P

机构信息

Medstar Washington Hospital Center and Children's National Medical Center, Washington, District of Columbia.

Norton Children's Medical Group, Louisville, Kentucky.

出版信息

J Endocr Soc. 2020 Sep 4;4(11):bvaa131. doi: 10.1210/jendso/bvaa131. eCollection 2020 Nov 1.

Abstract

CONTEXT

46,XX patients with classic congenital adrenal hyperplasia (CAH) are exposed to elevated androgens causing varying levels of virilization. The majority undergo feminizing genitoplasty early in life, with potential impact on sexual function and health-related quality of life (HRQoL).

OBJECTIVE

We aimed to determine how sexual and lower urinary tract function, body image, and global HRQoL differs between patients with classic CAH and controls and to characterize how gynecologic anatomy contributes to outcomes.

METHODS

36 patients with classic CAH and 27 control women who were matched for age, race, and marital status underwent standardized gynecological examination and validated questionnaires. The responses were analyzed in relation to gynecological measurements, genotype, and disease status.

RESULTS

Compared with controls, patients with CAH were more likely to have sexual dysfunction ( = 0.009), dyspareunia ( = 0.007), and other pelvic pain ( = 0.007); were less likely to be heterosexual ( = 0.013) or ever have been sexually active ( = 0.003); had poorer body image independent of body mass index ( < 0.001); and had worse HRQoL in the areas of general health ( = 0.03) and pain ( = 0.009). The patients with CAH had smaller vaginal calibers and perineal body lengths and larger clitoral indexes when compared with controls ( < 0.001). A larger vaginal caliber in CAH patients was associated with better overall sexual function ( = 0.024), increased sexual satisfaction ( = 0.017), less pain ( < 0.001), and greater number of sexual partners ( = 0.02).

CONCLUSIONS

46,XX patients with CAH have increased rates of sexual dysfunction, poor body image, and poor HRQoL, which is mitigated by having a larger vaginal caliber. Management aimed at optimizing vaginal caliber might improve sexual function.

摘要

背景

患有经典型先天性肾上腺皮质增生症(CAH)的46,XX患者会接触到升高的雄激素,导致不同程度的男性化。大多数患者在幼年时接受女性化生殖器整形手术,这可能会对性功能和健康相关生活质量(HRQoL)产生影响。

目的

我们旨在确定经典型CAH患者与对照组在性和下尿路功能、身体形象以及总体HRQoL方面有何不同,并描述妇科解剖结构如何影响结果。

方法

36例经典型CAH患者和27例年龄、种族及婚姻状况相匹配的对照女性接受了标准化妇科检查和经过验证的问卷调查。对问卷回答与妇科测量、基因型和疾病状态进行了分析。

结果

与对照组相比,CAH患者更易出现性功能障碍(P = 0.009)、性交困难(P = 0.007)和其他盆腔疼痛(P = 0.007);异性恋的可能性较小(P = 0.013)或从未有过性活动(P = 0.003);与体重指数无关,身体形象较差(P < 0.001);在总体健康(P = 0.03)和疼痛(P = 0.009)方面的HRQoL较差。与对照组相比,CAH患者的阴道口径较小、会阴体长度较短且阴蒂指数较大(P < 0.001)。CAH患者较大的阴道口径与更好的总体性功能(P = 0.024)、更高的性满意度(P = 0.017)、更少的疼痛(P < 0.001)以及更多的性伴侣数量(P = 0.02)相关。

结论

46,XX型CAH患者性功能障碍、身体形象差和HRQoL差的发生率增加,而较大的阴道口径可减轻这些情况。旨在优化阴道口径的治疗可能会改善性功能。

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