Successful pregnancy after the treatment of primary amenorrhea in a patient with non-classical congenital adrenal hyperplasia
Article first published online: 4 JUN 2012
© 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology
Journal of Obstetrics and Gynaecology Research
Volume 39, Issue 1, pages 406–409, January 2013
How to Cite
Purwana, I. N., Kanasaki, H., Oride, A. and Miyazaki, K. (2013), Successful pregnancy after the treatment of primary amenorrhea in a patient with non-classical congenital adrenal hyperplasia. Journal of Obstetrics and Gynaecology Research, 39: 406–409. doi: 10.1111/j.1447-0756.2012.01903.x
- Issue published online: 7 JAN 2013
- Article first published online: 4 JUN 2012
- Received: November 28 2011.; Accepted: March 3 2012.
- congenital adrenal hyperplasia;
- polycystic ovary syndrome
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.