A Case of Male Pseudohermaphroditism due to 17alpha-Hydroxylase Deficiency.
- Author:
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
- Publication Type:Case Report
- Keywords:
17alpha-hydroxylase deficiency;
Male pseudohermaphroditism;
Congenital adrenal hyperplasia
- MeSH:
46, XY Disorders of Sex Development*;
Adrenal Hyperplasia, Congenital;
Adrenocorticotropic Hormone;
Adult;
Amenorrhea;
Antihypertensive Agents;
Chymosin;
Desoxycorticosterone;
Estradiol;
Estrogens;
Female;
Humans;
Hydrocortisone;
Hypertension;
Hypokalemia;
Male*;
Phenotype;
Renin;
Testosterone
- From:Korean Journal of Fertility and Sterility
2006;33(2):133-138
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
