저생식샘자극호르몬 생식샘저하증과 부분 성장호르몬 부분결핍에 동반된 심한 자궁 저형성 1례
A Case of Extreme Uterine Hypotrophy in Hypogonadotropic Hypogonadism with Partial GH Deficiency
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초록
사춘기 지연이 있는 여자 환자에서 영상검사에서 자궁이 관찰되지 않을 때, 자궁의 발육부전인지 뮐러관 무발생인지를감별하는 것은 쉽지 않다. 이러한 경우에는 진단을 하기 전 호르몬 검사, 염색체 검사 등의 결과를 함께 평가하여야 하며, 에스트로겐 투여 후 자궁의 변화를 관찰해보는 것도 방법중의 방법 중의 하나이다. 저자들은 초기에 뮐러관 무발생을 의심하였다가 최종적으로 저생식샘자극호르몬 생식샘저하증으로 진단하고 에스트로겐 투여 후 자기공명영상을 통하여 자궁의 발육을 확인하였던 심한 자궁 저형성 증례를 경험하였기에 보고하는 바이다.
Abstract
Congenital hypogonadotropic hypogonadism is one of the causes of pubertal failure and primary amenorrhea, it is related to uterine hypotrophy. If the uterus is extremely hypotrophied, it is difficult to identify in imaging studies and can be misdiagnosed as a structural anomaly of internal genitalia. We report a case of extreme uterine hypotrophy in 18-year-old girl with primary amenorrhea that was finally diagnosed as hypogonadotrophic hypogonadism. The patient was initially suspected of Müllerian agenesis owing to the invisible uterus. After 4 months of treatment with estrogen, she showed significant growth of the uterus.
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