EBAUCHE D'UTERUS PLUS OU MOINS PRESENT UTERUS -TROMPE- DE FALLOPE OVAIRES FONCTIONNELS Col de I'Uterus Cupule vaginale Vagin Cavite vaginale plus ou moins d'1 cm VULVE MRKH NON MRKH 26
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Androgen Insensitivity Syndrome Androgen receptor mutation o Stimulation from testosterone and dihydrotestosterone (DHT)does not occur Spectrum of disorders CAIS,PAIS,MAIS Tissue response to estrogen remains,allowing certain signs of feminizafion to occur Antimullerian hormone is present,resulfing in regression of the Mullerian duct. Because of androgen resistance,the male Wollfian duct does not form. Female phenotype,XY 27
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处女膜闭锁征 imperforate hymen 28 28
28 处女膜闭锁征 imperforate hymen 28
阴道横隔 transverse vaginal septum Transverse vaginal septurn 29 29
29 阴道横隔 transverse vaginal septum 29
原发性闭经— 第二性征缺乏 高促性腺激素性腺功 能减退 低促性腺激素性腺功 能减退 ●特纳综合征(Turner's syndrome 生理性或体质性青春期延 ●46XX/46XY单纯性腺发 迟 育不全 ·嗅觉缺失综合征 ●影响性激素合成的酶缺陷 性疾病（17/21-羟化酶 缺失) 30
30 原发性闭经——第二性征缺乏 高促性腺激素性腺功 能减退 ⚫ 特纳综合征（Turner’s syndrome ⚫ 46XX/46XY单纯性腺发 育不全 ⚫ 影响性激素合成的酶缺陷 性疾病（17α/21-羟化酶 缺失） 低促性腺激素性腺功 能减退 ⚫ 生理性或体质性青春期延 迟 ⚫ 嗅觉缺失综合征