Swyer Syndrome: A Case Report.

Jung, S K; Jung, Y D; Lee, E S; Lee, J G; Lee, Y H; Rha, J Y

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Case Report

Korean Journal of Obstetrics & Gynecology 1999;42(5):1142-1145.
Published online January 1, 2001.

Swyer Syndrome: A Case Report.

S K Jung, Y D Jung, E S Lee, J G Lee, Y H Lee, J Y Rha

Abstract

A 18-year-old female was referred on January, 1998 for primary amenorrhea. Physical examination revealed eunuchoid body proportions, no breast development and no pubic and axillary hair. No somatic abnormalities could be detected. Peripheral blood karyotype was 46, XY. Exploratory laparotomy revealed a hypoplastic uterus, bilateral tubes and bilateral streak gonads. It is well known that patients with gonadal dysgenesis in whom a Y chromosome component exists are at risk for developing gonadoblastoma or dysgerminoma. So, bilateral gonadectomy was performed and hormone replacement therapy was instituted. We report a case with review of the literature.

Key Words: pure gonadal dysgenesis, Swyer syndrome

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