Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia
Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia
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DOI：
10.1210/jc.2006-1757
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发表时间：
2007-04-01
影响因子：
5.8
通讯作者：
Bougneres, P.
中科院分区：
文献类型：
--
作者：
Gastaud, F.;Bouvattier, C.;Bougneres, P.
目的：本研究的目的有两个方面：1)详细描述出生时不同表型严重程度的先天性肾上腺增生（CAH）成年妇女的性和生殖结局；2) CAH亚型之间以及CAH女性与非CAH对照女性之间这些结果的比较。设计：这是一项横断面研究，采用面对面访谈、书面问卷、女性性功能指数和妇科检查。患者：患者包括35名患有CAH的女性，代表出生时的Prader期I-V，年龄18-43岁，从出生到青春期在同一儿科内分泌诊所接受治疗。从医院工作人员家庭中选择69名非cah健康对照妇女。结果：没有CAH妇女对自己的性别分配表示怀疑。20%（35人中有7人）有同性恋倾向；23%（35人中有8人）已婚；其中三人报告完全缺乏性活动；37%的人（35人中有13人）说他们从未有过阴道插入的异性性行为。通过女性性功能指数评估的性功能在CAH女性中比对照组低得多，在高水平的CAH女性中最低。81%（22人中有18人）在阴道插入时感到疼痛。只有8名妇女怀孕，17%（35人中有6人）有了孩子。结论：尽管医生对这种罕见疾病进行了专业的医疗和手术治疗，但患有CAH的女性在性功能和生殖生活方面仍然受到重大限制。
Objectives: The objectives of the study were 2-fold: 1) a detailed description of sexual and reproductive outcomes in adult women with congenital adrenal hyperplasia (CAH) of different phenotypic severity at birth; and 2) comparisons of these outcomes among CAH subtypes and between CAH women and non-CAH control women.Design: This was a cross-sectional study using a face-to-face interview, a written questionnaire, the Female Sexual Function Index, and a gynecological examination.Patients: Patients included 35 women with CAH, representing Prader stages I-V at birth, aged 18-43 yr, who had been treated from birth to adolescence in the same pediatric endocrine clinics. Sixty-nine non-CAH healthy control women were selected from hospital-staff families.Results: None of the CAH women expressed doubts about their gender assignment. Twenty percent (seven of 35) had homosexual inclinations; 23% (eight of 35) were married; three reported a complete lack of sexual activity; and 37% (13 of 35) said they never had heterosexual intercourse with vaginal penetration. Sexual functioning as assessed by the Female Sexual Function Index was much lower in CAH women than controls and lowest in CAH women with high Prader stages. Eighty- one percent (18 of 22) experienced pain during vaginal penetration. Only eight women became pregnant, and 17% (six of 35) had children.Conclusions: Despite expert medical and surgical care by physicians dedicated to this rare disease, women with CAH still suffer major limitations in their sexual function and reproductive life.