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KMID : 0358419920350081242
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Korean Journal of Obstetrics and Gynecology
1992 Volume.35 No. 8 p.1242 ~ p.1252
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4 Cases of 46 , XY Pure Gonadal Dysgenesis
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김완영/Kim WY
심상덕/최두석/황도영/김종훈/이진용/Shim SD/Hwang DY/Kim JH/Lee JY
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Abstract
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Four cases of 46,XY pure gonadal dysgenesis,which were confirmed by the findings of gonads at laparoscopy, are described. The first three cases are siblings. All the patients and a normal male (46, XY) karyotype in the cytogenetic studies. Patient 1, aged 22, had a hypoplastic uterus, normal tubes, and bilateral streak gonads. Patient 2, aged 29, had a hypoplastic uterus, normal tubes, right streak gonad harboring gonadoblastoma and left gonadoblastoma overgrown dysgerminoma. Patient 3, aged 19, had a cord-like uterus, hypoplastic tubes, and right streak gonad and left goand harboring gonadoblastoma. Patient 4, aged 26, had a cord-like uterus, normal tubes, and bilateral streak goands. Gonadal streaks were extirpated in all the patients and total abdominal hysterectomy was also performed due to the malignant potential of the gonadoblastoma in patient 2.
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