Congenital adrenal hyperplasia and luteal dysfunction.
A case of 21-hydroxylase deficiency diagnosed and first treated at the age of 3 years is presented. Although pubertal development was delayed, full reproductive function was attained, with menses occurring at 14-33-day intervals. Infertility evaluation at age 20 revealed elevated 17-hydroxyprogesterone (17Po) and androgen concentrations and reduced luteal phase progesterone levels consistent with inadequate luteal function. This was corrected with additional corticosteroid replacement. Normal pregnancy and delivery followed.
Villanueva
A L
AL
Rebar
R W
RW
D019326:17-alpha-Hydroxyprogesterone; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D000328:Adult; D000728:Androgens / Q000097:blood; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D006908:Hydroxyprogesterones / Q000097:blood; D007247:Infertility, Female / Q000209:etiology; D008183:Luteal Phase; D011247:Pregnancy; D013250:Steroid Hydroxylases / Q000172:deficiency*
D000728:Androgens; D006908:Hydroxyprogesterones; D019326:17-alpha-Hydroxyprogesterone; D013250:Steroid Hydroxylases; D006854:Hydrocortisone