Fertility in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Abstract

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.

Target audience: Obstetricians & Gynecologists, Family Physicians.

Learning objectives: 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.