Late onset adrenocortical hydroxylase deficiencies associated with menstrual dysfunction.

Forty-eight women with menstrual dysfunction and clinical evidence of excess androgen production were subjected to an adrenocorticotropic hormone stimulation test that demonstrated the presence of a late-onset 11- or 21-adrenocortical hydroxylase deficiency. Adrenocortical suppression therapy with prednisone was then instituted. Eighty-three percent of the women improved and either conceived, established normal regular menstrual cycles, or noted a significant increase in menstrual regularity. In patients desiring fertility, there was an overall pregnancy rate of 64% and a corrected pregnancy rate of 93%. Some of these patients were given clomiphene in addition to the prednisone. The present study has demonstrated that an adrenocortical abnormality, characterized by a partial deficiency of either 11-hydroxylase or 21-hydroxylase, plays a significant role in many women with clinical evidence of hyperandrogenism and menstrual abnormalities. In such women, adrenocortical suppression is indicated and often results in a significant improvement of the menstrual disturbance.