| 单纯性XY性腺发育不全 |
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| 引用本文： | 于传鑫,於亢笛,葛汝珍,郑怀美.单纯性XY性腺发育不全[J].复旦学报(医学版),1986(5). |
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| 作者姓名： | 于传鑫 於亢笛 葛汝珍 郑怀美 |
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| 作者单位： | 上海医科大学妇产科医院
(于传鑫，於亢笛，葛汝珍)，上海医科大学妇产科医院(郑怀美) |
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| 摘 要： | 本文报道5例单纯性性晚发育不全(Swyer综合征)。基因型为46,XY,表现型为女性伴性腺发育不全。3例为家族性,2例为散发性。临床特征为女性表现型,原发闭经,无青春期变化。所有患者均有幼稚子宫、正常输卵管、条索状性腺和女性外生殖器。2例有性母细胞瘤。讨论了发病机理、性腺肿瘤和处理等问题。
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| 关 键 词： | 性腺发育不全 基因型 表现型 性腺 性分化 卵巢恶性肿瘤 |
PURE XY GONADAL DYSGENESIS |
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| YTI CHUANXIN,YU KANGDI,GE KUZHEN,ZHENG HUAIMEI.PURE XY GONADAL DYSGENESIS[J].Fudan University Journal of Medical Sciences,1986(5). |
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| Authors: | YTI CHUANXIN YU KANGDI GE KUZHEN ZHENG HUAIMEI |
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| Abstract: | Five patients with pure gonadal dysgenesis (Swyer Syndrome) are reported. Three of them were siblings, two cases were sporadic. Their genotypes were all 46, XY and phenotypes, all female with gonadal dysgenesis primary amenorrhea, Sexual infantilism, hypoplastic uteri, bilateral tubes, streak gonads and ambiguous female genitalia. Gonadoblastomas were found in the two sporadic patients. The pathogenesis, potential risk of gonadal tumor and the treatment are discussed. |
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| Keywords: | gonadal dysgenesis genotype phenotype gonads sex differentiation ovarian malignant tumor |
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