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Original Article
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Korean J Med. 2007;72(2):266-270.
- 증례 : Mayer-Rokitansky-Küster-Hauser 증후군 1예
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김지은
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- A case of Mayer-Rokitansky-Küster-Hauser syndrome
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고려대학교 의과대학 내과학교실
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- Abstract
- Mayer-Rokitansky-Küster-Hauser syndrome (Mllerian agenesis) is a rare malformative anomaly (1/5000 women) that is associated with the congenital absence of the upper vagina and uterus with normal ovaries. We report a case of a 15-year-old woman who presented with primary amenorrhea and the absence of secondary sexual development. A hormone assay revealed hypogonadotrophic hypogonadism. The karyotype was normal, 46XX. Internal genitalia could not be identified on a pelvic ultrasound. There were no other morphological malformations. Hormone substitution therapy remains
the only therapeutic option.
Hormone substitution is aimed at triggering the development of secondary sexual characteristics and preventing osteoporosis. The problem of infertility remains unsolved.(Korean J Med 72:S266-S270, 2007)
Key Words : Mayer-Rokitansky-Küster-Hauser syndrome, Primary amenorrhea, secondary sexual development
Keywords :Mayer-Rokitansky-Kü, ster-Hauser syndrome, Primary amenorrhea, secondary sexual development
