A Case of Male-Pseudohermaphroditism due to 17-beta-hydroxysteroid Dehydrogenase Deficiency with Multiple Osteoporosis / 대한산부인과학회잡지
Korean Journal of Obstetrics and Gynecology
; : 2097-2104, 1997.
Article
en Ko
| WPRIM
| ID: wpr-14915
Biblioteca responsable:
WPRO
ABSTRACT
A 37-year-old woman was admitted for chief complaints about primary amenorrhea and multiple bone pains. She was raised phenotypically female but her chromosomal study was no-rmal male karyotype(46 XY). On pelvic examination, she showed relatively normal female ex-ternal genitalia except short blind-ending vagina. There were also no uterus and tubes in operation field. Even though rare disorder, she was diagnosed male pseudohermaphroditism due to 17-beta -hydroxysteroid dehydrogenase deficiency. This disorder is the most common enzyme defect in biosynthesis of testosterone and involves the last step. The serum level of sex steroid: test osterone, estradiol were decreased and FSH, LH were increased. Androstenedione was increased and we could obtain that Androstenedione / Testosterone ratio was increased. Because of 17-beta-HSD deficiency, estrogen was not converted to estradiol, and so estradiol was markedly decreased. Same as above, we experienced a case of male pseudohermaphroditism due to 17-beta-hydroxysteroid dehydrogenase deficiency with multiple osteoporosis. So we report this case with a brief review of literatures.
Palabras clave
Texto completo:
1
Banco de datos:
WPRIM
Asunto principal:
Osteoporosis
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Oxidorreductasas
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Testosterona
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Útero
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Vagina
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Estradiol
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Estrógenos
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Trastorno del Desarrollo Sexual 46,XY
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Examen Ginecologíco
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Amenorrea
Límite:
Adult
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Female
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Humans
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Male
Idioma:
Ko
Año:
1997
Tipo del documento:
Article