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KMID : 0358420070500040684
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Korean Journal of Obstetrics and Gynecology
2007 Volume.50 No. 4 p.684 ~ p.688
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A case of primary amenorrhea with hypertension due to 17α-hydroxylase deficiency
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Park Dae-Joong
Kim Chung-Hoon
Lee Sa-Ra
Lee Hee-Young
Shim Seung-Hyuk
Kim Sung-Hoon
Chae Hee-Dong
Kang Byung-Moon
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Abstract
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17α- hydroxylase deficiency is a rare form of congenital adrenal hyperplasia and characterized by the coexistance of hypertension caused by the hyperproduction of mineralocorticoid precursors and sexual abnormalities, such as female pseudohermaphroditism and sexually infantile female with 46,XX karyotype or male pseudohermaphroditism with 46, XY karyotype, due to impaired production of sex hormone. We experienced a case of 17α- hydroxylase deficiency (46,XX) presented with primary amenorrhea, sexual infantilism, and hypertension. We report this case with a brief review of the concerned literatures.
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KEYWORD
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17α-Hydroxylase Deficiency, Hypertension, Primary Amenorrhea
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