mayer-rokitansky-kÜster-hauser综合征合并平滑肌瘤:诊断困难

L. Markin, Lidia Segedii
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引用次数: 0

摘要

每4000-5000名新生女孩中就有1人被诊断为迈耶-罗基坦斯基-库斯特-豪泽综合征(MRKH)。该综合征的特征是女性表型,正常核型46,XX,先天性缺失阴道或其上部,宫颈和子宫。MRKH综合征的病因尚不清楚,可能是由于其异质性。本文重点综述了MRKH综合征发生的可能病因、勒氏杆菌发育不全患者生殖功能实现的可能性等方面的文献。一个罕见的病例平滑肌瘤患者MRKH,可能性和诊断的困难是在文章中描述。结果表明,MRKH综合征的存在并不排除不同定位的乳头状瘤的可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
LEIOMYOMA IN CASE OF MAYER-ROKITANSKY-KÜSTER-HAUSER SYNDROME: DIFFICULTIES IN DIAGNOSIS
Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is diagnosed in one in 4000-5000 newborn girls. This syndrome is characterized by the female phenotype, normal karyotype 46, XX, congenital absence of the vagina or its upper part, cervix and uterus. The etiology of MRKH syndrome is still unknown, probably due to its heterogeneity. The article highlights and summarizes the literature on the probable etiological factors of MRKH syndrome development, the possibility of the reproductive function realization of patients with Müllerian agenesis. A rare case of leiomyoma in patients with MRKH, possibilities and diffi culties of diagnosis are described in the article. It is shown that the presence of MRKH syndrome does not exclude the possibility of fi broids of diff erent localization.
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