Abstract | BACKGROUND: 46XY pure gonadal dysgenesis ( Swyer syndrome) is a rare disorder of sexual development. Patients have a 46XY karyotype, though phenotypically they appear female with normal external genitalia and vagina. Although patients exhibit normal Müllerian structures (uterus, fallopian tubes, and vagina), they possess a pair of bilateral undifferentiated gonad streaks. Delayed puberty and primary amenorrhea are the common presentations. There is an increased risk of developing tumors in the gonads and therefore a bilateral gonadectomy is recommended. CASE: SUMMARY AND CONCLUSION:
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Authors | Aisha Anwar, Muhammad Akhtar, Gail Busby |
Journal | Journal of pediatric and adolescent gynecology
(J Pediatr Adolesc Gynecol)
Vol. 34
Issue 6
Pg. 869-871
(Dec 2021)
ISSN: 1873-4332 [Electronic] United States |
PMID | 33989803
(Publication Type: Case Reports)
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Copyright | Copyright © 2021. Published by Elsevier Inc. |
Topics |
- Adolescent
- Dysgerminoma
(surgery)
- Fallopian Tubes
- Female
- Gonadal Dysgenesis, 46,XY
(complications, diagnosis, surgery)
- Gonadoblastoma
- Humans
- Ovarian Neoplasms
(complications, surgery)
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