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睾丸女性化综合征的研究进展
引用本文:崔正慧,金杭美. 睾丸女性化综合征的研究进展[J]. 中国妇幼健康研究, 2006, 17(6): 547-549
作者姓名:崔正慧  金杭美
作者单位:浙江大学医学院附属妇产科医院,浙江,杭州,310006
摘    要:睾丸女性化综合征是一种X连锁的隐性遗传病,为男性假两性畸形中最常见的类型.患者染色体核型46,XY,性腺为睾丸,雄激素合成正常,但由于雄激素受体基因的突变,导致功能正常的雄激素受体减少或缺失,最终形成临床上从几乎正常男性到几乎正常女性的广泛表型.因为发育异常的睾丸存在恶变可能,目前普遍认为应该首选睾丸切除术,但在手术时机的选择上还存在着很多争议.该文主要从发病的分子机制以及手术方式,手术时机的选择方面进行综述.

关 键 词:睾丸女性化综合征  雄激素受体  基因突变  睾丸切除术
文章编号:1673-5293(2006)06-0547-03
收稿时间:2006-08-30
修稿时间:2006-08-30

Progress in study on testicular feminization syndrome
CUI Zheng-hui,JIN Hang-mei. Progress in study on testicular feminization syndrome[J]. Chinese Journal of Maternal and Child Health Research, 2006, 17(6): 547-549
Authors:CUI Zheng-hui  JIN Hang-mei
Affiliation:The Affiliated Obstetrical and Gynecological Hospital of Medical College, Zhejiang University, Zhejiang Hangzhou310006, China
Abstract:Testicular feminization syndrome,the most common type of male pseudohermaphroditism,is an X-linked recessive disorder.It is characterized by varied phenotypes of feminization from almost nomal male to almost normal female of male individual with 46,XY karyotype who has testis,and normal or even elevated androgen levels.It is caused by mutations in the androgen receptor gene,resulting in decrease or absence of normal andrognen receptor.For present,it is generally considered that testectomy should be performed because of increased risk of malignant transformation of abnormal testiculus,but the timing of operation is controversial.In this article,we reviewed molecular mechanism of the disease,surgical procedure,timing of operation and so on.
Keywords:testicular feminization syndrome  androgen receptor  gene mutation  testectomy
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