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KMID : 0358420170600040378
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Korean Journal of Obstetrics and Gynecology
2017 Volume.60 No. 4 p.378 ~ p.382
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Female with 46, XY karyotype
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Jung Eun-Jung
Im Do-Hwa
Park Yong-Hee
Byun Jung-Mi
Kim Young-Nam
Jeong Dae-Hoon
Sung Moon-Su
Kim Ki-Tae
An Hyo-Jung
Jung Soo-Jin
Lee Kyung-Bok
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Abstract
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Disorders of sex development (DSD) are congenital conditions characterized by atypical development of chromosomal, gonadal, and phenotypic sex. 46, XY DSD can result from disorders of testicular development or disorders of androgen synthesis/action. Prophylactic gonadectomy should be considered in patients with 46, XY DSD because of the increased risk of gonadal malignancy. We report two rare cases of 46, XY DSD, including XY pure gonadal dysgenesis and complete androgen insensitivity syndrome, who underwent a prophylactic gonadectomy.
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KEYWORD
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46, XY female, Androgen-insensitivity syndrome, Gonadal dysgenesis
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