Stikkelbroeck Nike M M L, Hermus Ad R M M, Braat Didi D M, Otten Barto J
Department of Pediatric Endocrinology, University Medical Center Nijmegen, Nijmegen, The Netherlands.
Obstet Gynecol Surv. 2003 Apr;58(4):275-84. doi: 10.1097/01.OGX.0000062966.93819.5B.
Fertility in women with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency appears to be reduced. The purpose of this review is to summarize the reported evidence about subfertility in women with CAH and to review the causes of reduced fertility. Compared with a non-CAH female population, pregnancy and live-birth rates are severely reduced in salt-wasting patients, mildly reduced in simple virilizing patients, and normal in nonclassical patients. Several factors have been suggested to contribute to the impaired fertility in CAH females: adrenal overproduction of androgens and progestins (17-hydroxyprogesterone and progesterone), ovarian hyperandrogenism, polycystic ovary syndrome, ovarian adrenal rest tumors, neuroendocrine factors, genital surgery, and psychological factors such as delayed psychosexual development, reduced sexual activity and low maternal feelings. It is obvious that these factors are interrelated. Improving endocrine, surgical, and psychological management could contribute to improving fertility chances in these patients.
Obstetricians & Gynecologists, Family Physicians.
After completion of this article, the reader will be able to define the various types of CAH, to describe the fertility issues in the CAH patients, to outline the various other causes of hyperandrogenism, and to list the treatment options for the patient with CAH.
由于21-羟化酶缺乏导致的先天性肾上腺皮质增生症（CAH）女性的生育能力似乎有所下降。本综述的目的是总结已报道的关于CAH女性生育力低下的证据，并探讨生育力下降的原因。与非CAH女性人群相比，失盐型患者的妊娠和活产率严重降低，单纯男性化型患者轻度降低，非经典型患者正常。已有多种因素被认为与CAH女性生育力受损有关：肾上腺雄激素和孕激素（17-羟孕酮和孕酮）过度分泌、卵巢雄激素过多、多囊卵巢综合征、卵巢肾上腺残余瘤、神经内分泌因素、生殖器手术以及心理因素，如性心理发育延迟、性活动减少和母性情感淡漠。显然，这些因素相互关联。改善内分泌、手术和心理管理有助于提高这些患者的生育几率。
妇产科医生、家庭医生。
阅读本文后，读者将能够定义CAH的各种类型，描述CAH患者的生育问题，概述雄激素过多的其他各种原因，并列出CAH患者的治疗选择。
