Kulshreshtha Bindu, Marumudi Eunice, Khurana Madan Lal, Kriplani Alka, Kinra Geeta, Gupta Denvender K, Kucheria Kiran, Khadgawat Rajesh, Gupta Nandita, Seith Ashu, Ammini Ariachery C
Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India.
Gynecol Endocrinol. 2008 May;24(5):267-72. doi: 10.1080/09513590801945230.
Androgen excess is believed to be one of the major factors responsible for poor fertility outcomes in females with congenital adrenal hyperplasia (CAH). Some believe that the adverse effect of androgens on fertility could have its origins as early as the antenatal years. To assess the impact of prolonged androgen exposure on fertility in CAH patients, we compiled the data of females with CAH followed in our clinic during the last 25 years who were sexually active and had not been initiated on steroids until age 9 years.
This was an observational case study on seven patients with classical CAH who fulfilled the inclusion criteria. The age at initiation of therapy in these females ranged from 9 years to 29 years.
All patients had varying degrees of genital ambiguity. The most common presenting complaints were genital ambiguity, non-development of secondary sexual characteristics, hirsutism and primary amenorrhea. Genital surgery was performed in all patients at ages ranging from 12 to 29 years, except for one patient who underwent surgery at age 5 years without a diagnosis of CAH being made. Breast development ensued within 2 to 12 months and periods started in all patients within 2-24 months of steroid initiation. There were 13 pregnancies (seven normal vaginal deliveries, two spontaneous abortions and four pregnancies were medically terminated).
Late initiation of steroid therapy did not affect fertility in our cohort of CAH women. Androgen excess in situations of subnormal cortisol may not adversely affect fertility in females with CAH.
雄激素过多被认为是先天性肾上腺皮质增生症（CAH）女性生育结局不佳的主要因素之一。一些人认为雄激素对生育的不良影响可能早在产前就已存在。为评估长期暴露于雄激素对CAH患者生育能力的影响，我们收集了过去25年在我们诊所随访的性活跃且直到9岁才开始使用类固醇的CAH女性的数据。
这是一项针对7例符合纳入标准的经典型CAH患者的观察性病例研究。这些女性开始治疗的年龄从9岁到29岁不等。
所有患者均有不同程度的生殖器模糊。最常见的就诊主诉是生殖器模糊、第二性征未发育、多毛症和原发性闭经。除1例5岁时未诊断出CAH就接受手术的患者外，所有患者均在12至29岁接受了生殖器手术。乳房在2至12个月内开始发育，所有患者在开始使用类固醇后的2至24个月内月经来潮。共有13次妊娠（7例顺产、2例自然流产、4例人工流产）。
在我们的CAH女性队列中，类固醇治疗开始较晚并未影响生育能力。皮质醇水平低于正常情况下的雄激素过多可能不会对CAH女性的生育能力产生不利影响。
