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.
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
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- Male Pseudohermaphroditism Presented with Sudden Cardiac Arrest
- A case of primary amenorrhea with hypertension due to 17alpha-hydroxylase deficiency
- A case of male Pseudohermaphroditism due to 17alpha-Hydroxylase Deficiency
- Untreated Congenital Adrenal Hyperplasia with 17-alpha Hydroxylase/17,20-Lyase Deficiency Presenting as Massive Adrenocortical Tumor
- A case of 17 alpha-hydroxylase deficiency
