A Case of 17alpha-Hydroxylase Deficiency.
- Author:
Yong Kyu LEE
1
;
Yun Jong KANG
;
Chan Young PAK
;
Soo Young CHOI
;
Moon Soo PARK
Author Information
1. Department of Pediatrics, Han Il Hospital, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
17alpha-hydroxylase deficiency
- MeSH:
Adrenocorticotropic Hormone;
Blood Pressure;
Child;
Corticosterone;
Desoxycorticosterone;
Dexamethasone;
Diagnosis;
Estrogens;
Female;
Humans;
Hydrocortisone;
Hypertension;
Hypokalemia;
Ovary;
Potassium;
Progesterone;
Sexual Development;
Testosterone
- From:Journal of the Korean Pediatric Society
1996;39(4):577-584
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A 12-year-old girl with hypertension, hypokalemia, cystic ovaries and absence of secondary sexual development is presented. Hormonal study revealed very low levels of cortisol, testosterone, estrogen, and high levels of progesterone, deoxycorticosterone, corticosterone, FSH and ACTH. Following treatment with dexamethasone and estrogen, the levels of the latter group remarkably decreased. Serum potassium level and blood pressure also became normal. With all the above hormonal profile and clinical findings, we reached the diagnosis of 17alpha-hydroxylase deficiency and report this case with the review of literature.
