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True hermaphrodite presenting as primary amenorrhea

机译:真正的雌雄同体表现为原发性闭经

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True hermaphrodite is one of the rare variety of disorders of sex development. Most of them are genotypically females (46 XX) and present as under virilized males. Features of hyperandrogenism are present in those reared as females. The commonest histological variety of gonad found in them is ovotestis, which is at risk for malignancy. We report a girl with 46 XY true hermaphrodite without ovotestis presenting as primary amenorrhea and isolated clitoromegaly in the absence of any other features of virilization.Keywords: Disorders of sex development, true hermaphrodite, ovotestis
机译:真正的雌雄同体是性发育障碍的稀有品种之一。他们中的大多数是基因型雌性(46 XX),男性化为男性。高雄激素血症的特征存在于那些以雌性饲养的人群中。其中发现的最常见的性腺组织学变异是卵睾丸,存在恶性肿瘤的风险。我们报道了一个有46个XY真实雌雄同体但没有卵睾丸的女孩表现为原发性闭经和孤立的阴蒂肿大,而没有任何其他男性化特征。

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