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Korean Journal of Fertility and Sterility 2006;33(2):133-138.
Published online June 1, 2006.
A Case of Male Pseudohermaphroditism due to 17alpha-Hydroxylase Deficiency.
Keoung Ah Park, Youn Kyung Chung, Jung Ryeol Lee, Young Min Choi, Gyoung Hoon Lee, Hee Seung Kim, Byung Chul Jee, Seung Yup Ku, Chang Suk Suh, Seok Hyun Kim, Jung Gu Kim, Shin Yong Moon, Seong Yeon Kim
1Department of Obstetrics and Gynecology, Seoul National University College of Medicine, Seoul, Korea.
2Institute of Reproductive Medicine and Population, Medical Research Center, Seoul National University College of Medicine, Seoul, Korea.
3Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
Female phenotype of a 46,XY male may originates from male pseudohermaphroditism due to 17alpha-hydroxylase deficiency. Lack of cortisol increases adrenocorticotropic hormone (ACTH) and mineralocorticoid production, leading to low renin hypertention and hypokalemia. A 41-year-old phenotypic female presented primary amenorrhea and hypertension. In the hormonal profile, the levels of serum estradiol, testosterone, rennin, and cortisol were decreased and ACTH and deoxycorticosterone were increased. Laparoscopic bilateral gonadectomy was performed, and corticosteroid, antihypertensive drugs, and estrogen were administered. We report this case with a brief review of the literatures.
Key Words: 17alpha-hydroxylase deficiency; Male pseudohermaphroditism; Congenital adrenal hyperplasia
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