J Korean Pediatr Soc.
1996 Apr;39(4):577-584.
A Case of 17alpha-Hydroxylase Deficiency
- Affiliations
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- 1Department of Pediatrics, Han Il Hospital, Seoul, Korea.
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.
Keyword
MeSH Terms
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Adrenocorticotropic Hormone
Blood Pressure
Child
Corticosterone
Desoxycorticosterone
Dexamethasone
Diagnosis
Estrogens
Female
Humans
Hydrocortisone
Hypertension
Hypokalemia
Ovary
Potassium
Progesterone
Sexual Development
Testosterone
Adrenocorticotropic Hormone
Corticosterone
Desoxycorticosterone
Dexamethasone
Estrogens
Hydrocortisone
Potassium
Progesterone
Testosterone
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