Female with 46, XY karyotype
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 p...
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Published in | Obstetrics & gynecology science Vol. 60; no. 4; pp. 378 - 382 |
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Main Authors | , , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Korea (South)
Korean Society of Obstetrics and Gynecology; Korean Society of Contraception and Reproductive Health; Korean Society of Gynecologic Endocrinology; Korean Society of Gynecologic Endoscopy and Minimal Invasive Surgery; Korean Society of Maternal Fetal Medicine; Korean Society of Ultrasound in Obstetrics and Gynecology; Korean Urogynecologic Society
01.07.2017
대한산부인과학회 |
Subjects | |
Online Access | Get full text |
ISSN | 2287-8572 2287-8580 |
DOI | 10.5468/ogs.2017.60.4.378 |
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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. |
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AbstractList | 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. KCI Citation Count: 0 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.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. 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. |
Author | Im, Do Hwa Kim, Young Nam Jeong, Dae Hoon An, Hyo Jung Jung, Eun Jung Park, Yong Hee Kim, Ki Tae Byun, Jung Mi Sung, Moon Su Lee, Kyung Bok Jung, Soo Jin |
AuthorAffiliation | 1 Department of Obstetrics and Gynecology, Inje University Busan Paik Hospital, Busan, Korea 2 Paik Institute for Clinical Research, Inje University Busan Paik Hospital, Busan, Korea 3 Department of Pathology, Inje University Busan Paik Hospital, Busan, Korea |
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Cites_doi | 10.1111/j.1464-410X.2005.05639.x 10.1016/j.fertnstert.2008.04.043 10.1016/j.bpobgyn.2009.09.009 10.1016/j.jpag.2007.09.006 10.1210/jc.2005-2554 10.1111/j.1365-2265.2012.04330.x 10.1016/j.fertnstert.2009.02.087 10.1093/humrep/dem074 |
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Keywords | Gonadal dysgenesis Androgen-insensitivity syndrome 46, XY female |
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