title: Late onset adrenocortical hydroxylase deficiencies associated with menstrual dysfunction.
abstract: 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.
mesh_terms: D000293:Adolescent; D000302:Adrenal Cortex / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology; D000312:Adrenal Hyperplasia, Congenital / Q000201:enzymology / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone; D000328:Adult; D000728:Androgens / Q000378:metabolism; D002996:Clomiphene / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008599:Menstruation Disturbances / Q000188:drug therapy / Q000201:enzymology / Q000503:physiopathology*; D011241:Prednisone / Q000627:therapeutic use; D011247:Pregnancy; D013250:Steroid Hydroxylases / Q000172:deficiency*
chemical_list: D000728:Androgens; D002996:Clomiphene; D000324:Adrenocorticotropic Hormone; D013250:Steroid Hydroxylases; D011241:Prednisone