Korean J Fertil Steril.  2006 Jun;33(2):133-138.

A Case of Male Pseudohermaphroditism due to 17alpha-Hydroxylase Deficiency

Affiliations
  • 1Department of Obstetrics and Gynecology, Seoul National University College of Medicine, Seoul, Korea. ymchoi@snu.ac.kr
  • 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.

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.

Keyword

17alpha-hydroxylase deficiency; Male pseudohermaphroditism; Congenital adrenal hyperplasia

MeSH Terms

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
Adrenocorticotropic Hormone
Antihypertensive Agents
Chymosin
Desoxycorticosterone
Estradiol
Estrogens
Hydrocortisone
Renin
Testosterone
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