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A Case of Female Pseudohermaphroditism due to Congenital Adrenal Hyperplasia and a Case of Male Pseudohermaphroditism.

Journal of the Korean Pediatric Society 1981;24(12):1203-1208.
Published online December 15, 1981.
A Case of Female Pseudohermaphroditism due to Congenital Adrenal Hyperplasia and a Case of Male Pseudohermaphroditism.
Y S Shin, H J Park, H J Chun, M J Shin
Department of Pediatrics, Seoul Red Cross Hospital, Seoul, Korea
男性,女性假性半陰陽 各 1例
申潤植, 朴浩鎭, 全幸助, 辛美子
서울赤十字病院 小兒科
Abstract
Recently we experienced each of female and male pseudohermaphroditisms which were admitted with complaint of ambiguous genitalia nearly at thesame time. Case 1, 12 year 7 month old, had musculine appearance with acne, beard and mustache, broad shoulders and short hair. Her phallus was large penile shape with abundant pubic hair and vaginal opening was hidden behind the fused labioscrotal fold. By vaginogram suggesting uterine cavity was demonstrated and by laparoscope uterine fundus, fallopian tube and suspected ovary were revealed. The blood pressure and electrolytes were normal but the 24hours urinary 17-KS was 19. 7mg. The chromosome analysis was 46, XX with 13% Barr body. There was no history of severe dehydration during postnatal period. She was discharged on. hydrocortisone 60mg daily by circadian therapeutic schedule after total clitoridectomy with some reconstruction of labia. Case 11, 10 year old, had no any musculine or feminine appearance and no pigment-ation or breast enlargement. He had thumb-sized movable mass above right inguinal region suggesting scrotum, his phallus was enlarged like penis without pubic hair and vaginal open- ing was hidden behind the fused labioscrotal fold. Suspected vaginal canal and uterine tract were visible by laparoscope. The chromosome analysis was 46, XY with negative barr body. The 24 hours urinary 17-KS and 17-OHCS were 1.7mg and 7.5mg, respectively. He was discharged after clitoridectomy and gonadectomy with recommendation of estrogen therapy at the age of 13 to 15 years. Herein each of male and female pseudohermaphroditisms were presented with a brief review of the related literatures.
Key Words: Ambiguous external genitalia, Male and Female pseudohermaphroditism


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