先天性子宫内膜缺如（MRKH综合征）1例
Journal: Basic Medical Theory Research DOI: 10.12238/bmtr.v7i6.17068
Abstract
原发性闭经是指14岁以上女性未出现第二性征及初潮；或16岁以上女性虽有第二性征发育但未见初潮。原发性闭经最常见的病因是卵巢功能不全及生殖道异常。其中生殖道异常以穆勒管发育不全（MRKHS）最为常见。MRKH综合征可分为两型：MRKHS I型指仅有单纯穆勒管发育异常；II型指至少伴有一种先天性畸形（如肾、骨骼、心脏异常或听力缺陷等）的穆勒管发育不全。学者研究表明MRKH综合征是原发性闭经最常见的病因，该综合征表现为女性外生殖器及第二性征发育正常，但子宫及阴道上2/3发育异常。这是一种罕见疾病，发病率约1/5000。该病例报告了一例MRKH综合征，因原发性闭经确诊。
Keywords
先天性子宫内膜缺如；原发性闭经；MRKH综合征
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