Obstet Gynecol Sci.
2017 Jul;60(4):378-382. 10.5468/ogs.2017.60.4.378.
Female with 46, XY karyotype
- Affiliations
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- 1Department of Obstetrics and Gynecology, Inje University Busan Paik Hospital, Busan, Korea. 107526@paik.ac.kr
- 2Paik Institute for Clinical Research, Inje University Busan Paik Hospital, Busan, Korea.
- 3Department of Pathology, Inje University Busan Paik Hospital, Busan, Korea.
- KMID: 2386281
- DOI: http://doi.org/10.5468/ogs.2017.60.4.378
Abstract
- 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.
