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Korean Journal of Obstetrics & Gynecology 1997;40(9):2097-2104.
Published online January 1, 2001.
A Case of Male-Pseudohermaphroditism due to 17-beta-hydroxysteroid Dehydrogenase Deficiency with Multiple Osteoporosis.
Sae Chang Bang, Jae Ho Ha, Jin Ho Kim, So Jeung Kim, Kyung Rok Seung, Jong Hyun Choi
Department of Obstetrics and Gynecology, College of Medicine, Konkuk University, Korea.
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.
Key Words: Male pseudohermaphroditism, 17beta-hydroxysteroid dehydrogenase deficiency


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