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Congenital Adrenogenital Syndrome and Successful Pregnancy

Report of a Case

From the Department of Obstetrics and Gynecology, Kumamoto University Medical School, Kumamoto, Japan

Abstract

The case history of a woman born with congenital adrenogenital syndrome to whom a normal female infant was born after unilateral adrenalectomy and dexamethasone therapy is described. This 27-year-old patient, first evaluated at the age of 18, had a masculine figure, abnormal external genitalia, and primary amenorrhea. Clitoridectomy was performed. The urinary 17-ketosteroid excretion was elevated and pneumoretroperitoneography revealed an enlarged left adrenal gland, which weighed 30 g after extirpation and showed adrenocortical hyperplasia. Menses appeared and after a year of marriage, she conceived and discontinued dexamethasone treatment. Under the preoperative supplement of dexamethasone, she was delivered a healthy female infant by cesarean section because of cephalopelvic disproportion and an abnormal birth canal.

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