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KMID : 0358419770200010041
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Korean Journal of Obstetrics and Gynecology
1977 Volume.20 No. 1 p.41 ~ p.45
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Familial Adreogenital Syndrome
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최영중/Choi YJ
최영균/이영혜/한의정/Choi YK/Lee YH/Han EJ
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Abstract
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Patient. Kim. S.R, was examined together with her younger sister (kim. J.R) because of enlargement of clitoris, fusion of labioscrotal folds and musculine configuration. The child has been brought up as a girl. The patient developed in a way similar to her younger sister. Sex chromatin in oral mucosa smear: positive. Chromosome study: cytogenenetically normal 46/XY, female. Baseline urinary 17 KS was 37.3㎎/24hours. After three day`s treatment with dexamethasone, the output oh 17KS was 4. 55㎎/24 hours. Retroperitoneal pneumogram including tomography revealed to marked enlargement bilateral adrenal gland. Vaginogram showed uterus measured 3×2 ㎝ and distended vagina 6㎝ in length. Two case of female pseudohermaphroditism with virilization delveloped in sisters is presented a brief review of the concerned literatures.
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KEYWORD
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