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KMID : 1218220090010010070
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Korean Journal of Pediatric Urology
2009 Volume.1 No. 1 p.70 ~ p.72
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Complete Androgen Insensitivity Syndrome in Sisters
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Kim Sun-Ouck
Nam Deok-Hyun
Kwon Dong-Deuk
Ryu Soo-Bang
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Abstract
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Complete androgen insensitivity syndrome, also termed complete testicular feminization syndrome, is the most common form of male pseudohermaphroditism that is characterized by 46, XY karyotype, bilateral testes, absent or hypoplastic wolffian duct, female appearing external genitalia, blind vaginal pouch, and absent or rudimentary mullerian derivatives. Two sisters, 20 and 22 years old, visited our hospital because of primary amenorrhea. These patients were phenotypically women with female external genitalia, blind-ended vagina and absent mullerian derivatives. Their karyotypes are male (46, XY). Bilateral abdominal masses regarded as gonads and gonadectomy was performed. Histologic examination revealed that gonads had seminiferous tubules which were filled with Sertoli cells only, reactive Leydig cell hyperplasia and no malignant cell. Postoperatively, estrogen replacement therapy was started.
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KEYWORD
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Androgen insensitivity syndrome, Male pseudohermaphroditism
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