Successful pregnancy after the treatment of primary amenorrhea in a patient with non-classical congenital adrenal hyperplasia.
We describe a case of non-classical congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase deficiency in a 30-year-old Japanese woman who achieved pregnancy after treatment of primary amenorrhea. Hirsutism and clitoromegaly were present. Ultrasound examination showed polycystic appearance of the ovary. Luteinizing hormone-releasing hormone (LH-RH) test resulted in exaggerated LH response, showing a polycystic ovary syndrome (PCOS) pattern. The diagnosis was based on the elevated intial levels of 17-hydroxyprogesterone (55 ng/mL) and dihydroepiandosterone (7780 ng/mL). The first withdrawal bleeding occurred within 6 weeks after treatment with hydrocortisone (20 mg/day) combined with conjugated estrogens (1.25 mg/day) and medroxyprogesterone acetate (10 mg/day), which were continued for five courses. The bleeding remained cyclic every 28 days with maintenance doses of hydrocortisone. Subsequently, ovulation was induced using clomiphene citrate (100 mg/day). Pregnancy was achieved at the second attempt of ovulation induction and was within 10 months after initial presentation. Continuing hydrocortisone, the patient delivered a healthy baby at term.
Department of Obstetrics and Gynecology, Shimane University School of Medicine, Izumo, Japan.
Indri N
IN
Purwana
Haruhiko
H
Kanasaki
Aki
A
Oride
Kohji
K
Miyazaki
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy; D000328:Adult; D000568:Amenorrhea / Q000150:complications / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D004966:Estrogens, Conjugated (USP) / Q000627:therapeutic use*; D005260:Female; D006628:Hirsutism / Q000150:complications / Q000188:drug therapy; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D007231:Infant, Newborn; D017258:Medroxyprogesterone Acetate / Q000627:therapeutic use*; D010062:Ovulation Induction; D011247:Pregnancy; D011256:Pregnancy Outcome; D016896:Treatment Outcome
D004966:Estrogens, Conjugated (USP); D017258:Medroxyprogesterone Acetate; D006854:Hydrocortisone