Successful pregnancy after the treatment of primary amenorrhea in a patient with non-classical congenital adrenal hyperplasia

Authors

  • Indri N. Purwana,

    1. Department of Obstetrics and Gynecology, Shimane University School of Medicine, Izumo, Japan
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  • Haruhiko Kanasaki,

    Corresponding author
    1. Department of Obstetrics and Gynecology, Shimane University School of Medicine, Izumo, Japan
      Dr Haruhiko Kanasaki, Department of Obstetrics and Gynecology, Shimane University School of Medicine, 89-1 Enya Cho, Izumo, Shimane 693-8501, Japan. Email: kanasaki@med.shimane-u.ac.jp
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  • Aki Oride,

    1. Department of Obstetrics and Gynecology, Shimane University School of Medicine, Izumo, Japan
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  • Kohji Miyazaki

    1. Department of Obstetrics and Gynecology, Shimane University School of Medicine, Izumo, Japan
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Dr Haruhiko Kanasaki, Department of Obstetrics and Gynecology, Shimane University School of Medicine, 89-1 Enya Cho, Izumo, Shimane 693-8501, Japan. Email: kanasaki@med.shimane-u.ac.jp

Abstract

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.

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